COLUMBIA LIBRARIES OFFSITE HEALTH SCIENCES STANDARD HX00016012 .^5-- ^Jii;;-;---- ':?;:^;i::;'-'ii;<':'" l«>.;:;X;;<:':>';.:v.;. • . . sUl>:c;^' ■>-v''-.''/hV .,:2SSS^SSi2S2 in tfje Citp of iOteto fSorfe'^'' V^ COLLEGE OF PHYSICIANS AND SURGEONS Reference Library Given by Digitized by the Internet Archive in 2010 with funding from Open Knowledge Commons http://www.archive.org/details/organicfunctionaOOstar PLATE I The Various Neurones Constituting the Nervous System and their Relations. The association neurones are shown in red. A sensory impression from the skin reaches the spinal cord, where it may excite the motor neurone ; or the Clarke cell, and through it the cerebellum; or the medulla, optic thalamus and cerebral cortex, causing automatic responses and conscious per- ceptions. A sensory impression from the semicircular canals may reach the cerebellum, causing auto- matic acts of equilibrium. Cerebellar impulses may be sent to the motor neurone by way of the neu- rones of Deiters in the medulla, producing acts of balancing, or to the cerebrum by way of the optic thalamus, causing perceptions of position. Cerebral impulses may be sent directly to the motor neu- rone or indirectly through the corpus striatum, producing voluntary or automatic acts. The motor neurone sends its nerve to the muscle. ORGANIC AND FUNCTIONAL NERVOUS DISEASES A TEXT-BOOK OF NEUROLOGY BY M. ALLEN STARR, M.D., Ph.D., LL.D., Sc.D. PROFESSOR OP NEUROLOGY, COLLEGE OF PHYSICIANS AND SURGEONS, THE MEDICAL DEPARTMENT OP COLUMBIA UNIVERSITY IN THE CITY OF NEW YORK; CONSULTING NEUROLOGIST TO THE PRESBYTERIAN HOSPITAL AND TO ST. MARY'S FREE HOSPITAL FOR CHILDREN EX-PRESIDENT OF THE AMERICAN NEUROLOGICAL ASSOCIATION AND OF THE NEW YORK NEURO- LOGICAL SOCIETY ; CORRESPONDING MEMBER OP THE SOCI^T^ DE NEUROLOGIE, AND OF THE SOCIETE DE PSYCHIATRIE DE PARIS ; OF THE NEUROLOGICAL SECTION OF THE EOYAL SOCIETY OF MEDICINE, LONDON, AND OF THE GESELLSCHAFT DEUTSCHER NKRVENARZTE AUTHOR OF "FAMILIAR FORMS OF NERVOUS DISEASE," "BRAIN SURGERY," AND "ATLAS OF NERVE CELLS" FOURTH EDITION, THOROUGHLY REVISED ILLUSTEATED WITH 323 ENGEAVINGS IN THE TEXT AND 30 PLATES IN COLOES AND MONOCHEOME LEA & FEBIGER NEW YOEK AND PHILADELPHIA 1913 Copyright, 1913 By Lea & Febiger Press of The New era Printing Company Lancaster. Pa. PEEFACE TO THE FOUETH EDITION. The demand for another edition of this book has given me an op- portunity for a careful revision of its contents, for many additions, and also for an arrangement which should make it of greater value to the student and practitioner. The general aspects of neurology, the method of examination of a patient, and the principles of diagnosis have been brought together in the first part. The various symptoms presented by nervous affections are there described and analyzed ; the practical anatomy and physi- ology of the nervous system, necessary to their understanding, are con- cisely set forth, and the attempt is made to trace each symptom to its underlying disturbance of function in a definite nerve centre or nerve tract. In this way the student is enabled from the study of symptoms to reach a conclusion as to the nature and location of the lesion pro- ducing it, and thus to make a diagnosis of the disease present. In part second the organic diseases are taken up. Such additions as have been made to our knowledge in the past three years have been incorporated in this part, and several chapters, especially those upon poliomyelitis, and syphilis of the nervous system, have been rewritten. Much new material has been added to the chapter on tumors of the brain ; and pellagra has been discussed. In part third functional diseases are fully presented, the space allotted to this department having been more than doubled. Fuller discussion is given to spasmodic neuroses, especially to the various forms of tic and myoclonus. Some functional paralyses, myatonia congenita, myas- thenia gravis and family periodic paralysis have been presented. Psy- chasthenia is considered apart from neurasthenia, and the subjects of the conscious and subconscious mental activities, of suggestion and of psycho-analysis and of hypnosis are fully discussed. In the chapter on hysteria the various theories of the disease are put before the reader, the hypotheses of Babinski, Janet and Freud being contrasted. Chapters have been added on Headaches, and on Disorders of Sleep. In part fourth the diseases of the sympathetic nervous system are discussed, our present knowledge of vaso-motor affections, their physi- ology and pathology being summarized, and chapters added on sym- metrical gangrene and angio-neurotic oedema. Trophic symptoms occurring in nervous disorders are also presented. While the extensive literature of neurology has been carefully sifted, and references have been given to important articles upon the various subjects considered, it has been my endeavor to utilize my personal observation and experience in the presentation of each sul)jcct ; for the mass of pathological and clinical material that has accunuilatcd in my iv PREFACE. possession in the past thirty years in the practice of this specialty has repaid analysis and has led me to many definite conclusions. Due regard has been given to theories of disease, but attention has been especially directed throughout the book to etiology, diiferential diag- nosis, and treatment. Nervous diseases lie on the borderland between medicine and sur- gery, and in the consideration of treatment, the -surgical measures for the cure of many of them are fully described. For the surgical suc- cesses are among the most brilliant of recent achievements in neurology, and my own experience, working with McBurney, McCosh, Hartley, and Harvey Gushing, has been extensive. It is my hope that in this new form the work will add to its prac- tical service as a text-book for students, and as a guide for physicians in the recognition and treatment of nervous diseases. M. Allen Staer. Five West F^fty-fotjrth Street, New York. /■ CONTENTS PART I. The Structure of the Nervous System and the Diagnosis of Nervous Diseases. CHAPTER I. Introductoey. The General Diagnosis of Nervous Diseases , 17 CHAPTER II. The Structure op the Nervous System 21 CHAPTER III. The Peripheral Nervous System .36 CHAPTER IV. The Spinal Cord 47 CHAPTER V. The Diagnosis and Localization of Brain Diseases . . .82 PART II. Organic Nervous Diseases. SECTION I. Injuries and Diseases of the Nerves. CHAPTER VI. Injuries of Nerves. Neuritis 159 CHAPTER VII. Injuries of the Spinal Nerves and Special Forms op Neuritis. 174 CHAPTER VIII. Multiple Neuritis 197 CHAPTER IX. The Toxic Forms of Multiple Neuritis 212 V vi . CONTENTS. CHAPTER X. The Infectious and Toxemic Forms of Multiple Neuritis . . 235 CHAPTER XL Epidemic Multiple Neuritis 245 CHAPTER XII. Multiple Neuritis Associated with Other Diseased States of THE Body 250 SECTION II. Diseases of the Spinal Cord. CHAPTER XIII. The Spinal Bloodvessels 259 CHAPTER XIV. Poliomyelitis . 262 CHAPTER XV. Chronic Atrophic Paralysis. ........ 284 CHAPTER XVI. Muscular Dystrophies 303 CHAPTER XVII. Syringomyelia or Gliosis Spinalis 320 CHAPTER XVIII. Lateral Sclerosis. Spastic Paraplegia 337 CHAPTER XIX. Locomotor Ataxia. Tabes Dorsalis. Posterior Spinal Sclerosis. 342 CHAPTER XX. Combined Sclerosis 383 CHAPTER XXI. Myelitis and Myelomalacia . . . . . . - . 392 CHAPTER XXII. Injuries of the Spinal Cord. H^ematomyelia 416 CHAPTER XXIII. Compression of the. Spinal Cord 433 CONTENTS. vii CHAPTER XXIV. Landry's Paralysis. Acute Progressive Paralysis. . . . 454 CHAPTER XXV. Spinal Meningitis 457 SECTION III. Diseases of the Brain. CHAPTER XXVI. The Cerebral Circulation 463 CHAPTER XXVII. The Cerebral Diseases of Vascular Origin 467 CHAPTER XXVIII. The Cerebral Atrophies of Childhood 514 CHAPTER XXIX. Encephalitis , 535 CHAPTER XXX. Meningo-Encephalitis. Paresis. Dementia Paralytica . . 540 CHAPTER XXXI. Abscess of the Brain 549 CHAPTER XXXII. Thrombosis of the Venous Sinuses 567 CHAPTER XXXIII. Tumors of the Brain 571 CHAPTER XXXIV. Bulbar Paralysis . . . . . . . . . . . 614 CHAPTER XXXV. The Cranial Nerves and Their Diseases 622 SECTION IV. General Diseases of the Nervous System. CHAPTER XXXVI. Multiple Sclerosis 675 Viii CONTENTS. CHAPTER XXXVII. Syphilis of the Nervous System 686 « CHAPTER XXXVIII. Pellagra 698 . CHAPTER XXXIX. Caisson Disease . 701 CHAPTER XL. Pachymeningitis and Meningeal Hemorrhage .... 705 CHAPTER XLI. Cerebral Meningitis 713 PART III. Functional Diseases. CHAPTER XLII. The Painful Neuroses 729 CHAPTER XLIII. Headache and Migraine 755 CHAPTER XLIV. Functional Paralysis. 766 CHAPTER XLV. The Spasmodic Neuroses. Myoclonia 778 CHAPTER XLVI. The Spasmodic Neuroses 78^ CHAPTER XLVII. Tetany 804 CHAPTER XLVIII. Paralysis Agitans and Tremor 806 CHAPTER XLIX. Epilepsy 813 CHAPTER L. Neurasthenia and the Occupation NEXfROSES 837 CHAPTER LI. Psychasthenia , ... 863 CONTENTS. IX CHAPTER LII. Hysteria. 878 CHAPTER LIII. Vertigo 898 CHAPTER IJV. Disorders of Sleep 903 CHAPTER LV. Reflex Neuroses 909 PART IV. The Sympathetic Nervous System and its Diseases. CHAPTER LVI. Vaso-Motor and Trophic Neuroses ., 915 CHAPTER LVII. Symmetrical Gangrene . 932 CHAPTER LVIII. Angio-Neurotic (Edema 938 CHAPTER LIX. Trophic Neuroses 942 Index 955 PART I. STfilJCTUEE OF THE NERVOUS SYSTEM AND DIAGNOSIS OF NERVOUS DISEASES CHAPTER I. INTEODUCTOEY. THE GENEEAL DIAGNOSIS OF NEEVOUS DISEASES. THE METHOD OF EXAMINATION OF A PATIENT. The multitude and variety of symptoms which are present in ner- vous diseases are frequently bewildering and render it difl&cult for the student to know how to examine a patient suffering from a nervous disease. Some suggestions regarding methods of examination are neces- sary, and when each symptom is elicited by this examination the sig- nificance of each symptom or group of symptoms will be discussed. It is important in every case to obtain a complete history of the patient before proceeding to the physical examination. This history should elicit the hereditary tendencies of the patient, for the family history often affords an important clue to the disease present. Alco- holism or drug addiction, chronic lead or other poisons, such diseases as gout, tuberculosis or anaemia, as well as the various nervous diseases in parents make the liability to nervous affections in the next genera- tion greater, and are often the cause of functional affections, such as epilepsy, neurasthenia, psychasthenia and the tics. There are some diseases which run through several generations in a family, and which seem to imply some defective capacity of development. Thus heredi- tary deafness and blindness ; the dystrophies affecting the muscles ; the forms of ataxia seen in Friedreich's disease and in Marie's cere- bellar atrophy, and juvenile spastic paraplegia are all examples of what Gowers has termed abiotrophy, a congenital tendency for the special organ or part of the nervous system involved to wither and die long before the life of the individual ends. The personal history of the patient should be carefully studied, for the development in early life of convulsions, of headache, of habit spasms, of fainting fits, of mental peculiarities, or mental defects will 2 17 18 THE GENERAL DIAGNOSIS OF NERVOUS DISEASES. show a state of weakness of structure or error of function in the brain which may be a cause predisposing to the occurrence of nervous disease in later life. There are many nervous aifections directly or indirectly traceable to bad habits, to alcoholism, to chronic toxaemia by drugs or metals or poisons, to the infectious diseases and to syphilis. That many nervous aifections appear as a sequel of infections is now well known ; the neurasthenia and psychasthcnia following the grippe being examjiles. Sometimes the occupation of the patient will at once lead to the diagnosis ; as in lead palsy or in writer's or telegrapher's cramps, or in the paraplegia developing in those who work in caissons. There are not a few cases of nervous disease, both organic and functional, which are due to injury or to its attendant mental shock. And the influence of care, anxiety, and long-continued worry and overwork of a mental kind is fully appreciated by all physicians as a cause of nervous disease. The history of the mode of onset of the affection in question is equally important. The sudden appearance of symptoms, trauma being excluded, suggests some primary vascular disease, as in apoplexy ; or some acute infection, as in infantile paralysis or chorea. The gradual onset is characteristic of the more slowly advancing sclerotic changes, in the nervous system which cause so many forms of pro- gressive paralysis ; while the possibility of tumor growth should never be overlooked as a cause in these cases. Lastly it is not to be for- gotten that many nervous symptoms are wholly secondary to and the result of diseases of other organs, as for example the headaches from eyestrain, the reflex neuroses, neurasthenia of pulmonary or gastric or intestinal origin, hysteria from ovarian disease. The most prominent symptoms related by the patient will as a rule indicate whether the affection is functional or organic. The fact of occasional complete intermission in these symptoms is itself suggestive of functional disease, as in neuralgia as contrasted with neuritis, as in epilepsy, or in the spasmodic neuroses, as contrasted with the perma- nence of paralysis or of contracture or of ataxia. The extreme varia- bility in the character and in the intensity of symptoms, such as is seen in neuralgia, iu neurasthenia, and in hysteria, is at once suggestive of a functional disease : for when organic changes have occurred in the brain or in the spinal cord the symptoms are not only permanent but are invariable. And the susceptibility of a patient to various physical or psychical impressions or remedies is also an indication that the symptoms do not depend upon destruction of nerve tissue. Thus the history of the chief complaints and of their mode of appearance and constancy will usually give a clue to the form of disease present. If that disease is clearly organic in nature there is rarely any difficulty in assigning it to the part of the nervous system primarily involved. Lesions of the nerves produce pain, paralysis, anaesthesia and trophic symptoms wholly different in their exact locality and distribution in the body from lesions of the spinal cord, and hence by the grouping of the symjjtoms rather than by differences in their kind a differentiation mn QENEBAL DIAGNOSIS OF NEBVOUS DISEASES. ' 19 between nerve and cord lesions is reached. Lesions of the spinal cord produce" symptoms wholly different in their distribution in the body from lesions in the brain, one being as a rule bilateral, the other uni- lateral ; one being attended by electrical changes in the muscles and a loss of reflex action in the muscles, the other not. Lesions of the brain produce many changes in mental activity, in consciousness, memory and temper, in the powers of thought, expression and action, in the use of the cranial nerves, in the gait and balance never seen in disease of the nerves or of the spinal cord. Thus a cursory examination at once directs the attention of the examiner to the portion of the nervous system chiefly affected. The objective examination to determine the exact character, extent, and location of each symptom ; and to ascertain the actual degree of incapacity of every function is more important than the history. During the progress of such an examination incidentally the character and tem- perament of the patient, his emotional condition as shown in his facial expression, attitude, and manner of conversation, his degree of intelli- gence, his power of understanding, his capacity of speech, of movement, or of balance, can easily be observed without direct questions. Dis- turbances in the state of the mind, in the character and acuteness of the emotional control, in the rapidity of mental response to questions and impressions, and in memory are symptoms which are to be elicited by observation, as. the patient may be unaware of them, and unable to judge of his own condition. Powers of expression, the capacity to weigh and judge matters, the prevailing mood, are all important indi- cations of the mental state ; and can sometimes be learned better from relatives than from the patient. Hence the views of those with whom he lives are always to be obtained. In a case of shaking palsy, or of hemiplegia, or of locomotor ataxia, or of spastic paraplegia, mere inspection as a patient walks into the office is sometimes sufficient to warrant a diagnosis. But it is only after a thorough investigation of the powers of motion, of sensation, of reflex activity, of coordination, of gait, of speech, of mental activity and of self-control that one should feel sure that the entire ground has been covered in the examination. The various symptoms presented by the various forms of nervous disease are fully discussed in the following chapters of this part, each symptom in affections of the nerves, of the spinal cord and of the brain being described, the manner in which these symptoms may be elicited being portrayed, and the significance of each symptom being indicated. The attempt is made to trace each symptom to its under- lying pathological cause, so that the nature and situation of the lesion incidentally appears from the study of the symptoms. But as this necessarily implies a knowledge of the anatomy and physiology of the nervous system tlie essential and important facts of applied anatomy are included in the; chapters upon diagnosis. If in the use of this text-book constant comparison is made between the symptoms described in each djs^ease and the discussioji of the si^-- 20 THE GENEEAL DIAGNOSIS OF NERVOUS DISEASES. nificance of those symptoms in the chapters upon diagnosis, a much more comjjlete view will be gained of the disease in question, and it will become at once apj^arent how a complete study of a symptom in nervous diseases usually leads at once to a definite conclusion as to both the character and the situation of the underlying lesion which causes it. The use of the book will be enhanced by a constant reference to the index where, incidentally, the classification of the causes of the majority of symptoms appears. As a working scheme for such a neurological examination the follow- ing is oifered. Present complaint, with history of its onset and course. Previous diseases, and habits, and injuries. Heredity. Appearance, station, gait, power of motion or ataxia. Investigation of each cranial nerve in order, with particular atten- tion to the special senses and ophthalmoscopic examination and to the motion of eyes, face and tongue. Examination for paralysis ; including state of reflexes of pupils and superficial and deep reflexes. Examination for disturbance of touch, temperature, pain and mus- cular senses ; with particular care in the location of these or of all spontaneous pain or numbness. Condition of speech, and intelligence and emotional state. General physical examination of all organs. CHAPTER II. THE STRUCTURE OF THE NERVOUS SYSTEM. I. The Cerebro-spinal Nervous System. Neurones, Central and Peripheral. Den- drites. Axones. Terminal Tassels. Varieties, of Neorones. Nutrition and Pathology of Neurones. II. The Sympathetic Nervous System. The nervous system is divided into two parts, the cerebro-spinal system and the sympathetic system. I. THE CEREBRO-SPINAL NERVOUS SYSTEM. The cerebro-spinal nervous system consists of two large cen- tral organs, the brain and spinal cord, and of a very extensive and wide-spread peripheral system of nerves which bring these central organs into communication with every part of the body. Inasmuch as every form of functional activity in the organism requires direc- tion in accordance with the needs of each organ and also of the entire body, it is evident that a central regulating station in perfect connec- tion with all the diiferent parts is a necessity. The cerebro-spinal nervous system supplies this need. Its function consists in the recep- tion of impressions and the response to these impressions by actions that are performed in a properly ordered manner adapted to an end. Incidentally impressions and actions are registered, so that recollection is possible and repetition is easy. These acts may be unconscious and automatic, or conscious and voluntary, according to the parts of the nervous system which are called into play by the impression. But, whether conscious or unconscious, the action is always one of control of the lower mechanisms of the body ; of the sensory organs, and of the voluntary and involuntary muscles ; of the circulatory and respi- ratory systems ; of the secretory glands, and of the digestive and re- productive systems. Thus the nervous system may be considered as the governing and controlling system of the entire organism. The central organs of the nervous system are made up of gray and white matter, whose visible difference led older anatomists to attach much importance to their varying arrangement. The gray matter was known to be made up of cells, and the white matter of nerve fibres transmitting impulses to and from the cells. But recent investigation has demonstrated that gray and white matter are merely different parts of elementary bodies, termed neurones, which make up the nervous system, and that the study of these neurones, of their structure, and of their connections, whether they lie singly or in groups, as in the spinal cord, or in masses, as in the basal ganglia of the brain, or spread 21 22 TEE STHUCTUEE OF TUE NEEVOVS SYSTEM. out in layers, as in the cerebral cortex, is a matter of the greatest importance from every point of view. It is found that the white mat- ter and tlie nerves are merely the distal portions of these neurones, and are not to be studied apart from the central part or body which forms the gray matter. It is therefore the chief object of the anatomist to-day to establish the situation of the neurone body and the course and termination of the neurone branches, their mutual relations, and their manner of action and interaction. (See Plate I., frontispiece.) The neurones are held together by neuroglia and connective tissue, and are amply supplied with bloodvessels and lymphatics. Classes of Neurones. — There are two classes of neurones differ- ent in their origin in embryonal life. The first class develops in the central nervous system. The second class develops in the ganglia, which lie upon the nerves. I. The First ot Central Class of Neurone. — The neurone consists of a cell body and its projecting branches. These are of two kinds, den- drites and axones. The cell body has various shapes in different parts of the nervous sys- tem, being spherical, pyramidal, and. polygonal, as shown in Fig, 1. The actual structure of the body is shown in Plate II, A, page 22, which demonstrates a protoplasmic mass unstained, a nucleus and nucleolus and a large number of granular bodies which are more or less regularly arranged in the protoplasm about the nucleus. These bodies are stained by basic aniline dyes and have been termed Nissl bodies, chromophile bodies, stainable substance, or tigroid. Nissl bodies are made up of phospho-albumin, and it is supposed that this exists in solution in the living cell among the other ingredients of the protoplasm, but is pre- cipitated by the fixative and then stained by the dye. The cell body varies greatly in size in different parts of the nervous system ; it is from 4 to 135 microns in diameter. Through the cell body fine fibrils have been shown to pass in many directions from and to the branching processes.^ (Figs. 2 and 3.) The dendrites are branching protoplasmic processes of the cell body and resemble it in structure. They are very numerous in some neu- rones. Near the cell body they are thick, but as they extend away they divide and subdivide, resembling the roots of a tree, until they end in fine fibrils. The dendrites vary in length, some neurones having one very long dendrite and many short ones. These variations are shown in Fig. 1. They pursue a tortuous course and are very irregu- lar in their size, shape, and branching. The surface of the dendrite is rough and often nodular, and appears to be covered with small gran- ules called buds or gemmules. Within the dendrite fine filaments lie 1 The reader is referred to Barker' s Nervous System, Appleton & Co., 1898 ; to Ewing, Studies on Ganglion Cells, Archives of Neurology and Psychopathology, 1898, vol. i., p. 263 ; to Bailey, Morphology of Ganglion Cells, Journal of Experimental Medicine, 1901, vol. V, p. 550 ; to Nissl, Die Neuronenlehre, Jena, 1903, and to Lewandowsky^ Handbuch der Neurologic, Bd. I, 1910. /■ THE CEREBROSPINAL NERVOUS SYSTEM. Fig. 1. 23 Neurones, 1, anterior horn of sjiinal cord; 2, optic thaluiniis ; 3, posterior spinal ganglion (chick embryo); 4, cerebellum ; 5, cerebral cortex, surface layer. 24 THE STRUCTURE OF THE NERVOUS SYSTEM. Fig. 2. Large pluripolar ganglion cell of the ventral paramedian field of the abdominal cord of Lvmbriciis. A primitive fibril is seen on its way to a ganglion cell becoming thinner, owing to the emission of the side fibrils. A very complicated intracellular reticulum of neuro-fibrils is to be made out in the forma- tion of which the primitive fibrils of all the processes take part, kk, nucleolus ; k, nucleus, a and /3 are processes containing one primitive fibril in each, which arriving in the cell body split up into several bifurcating neuro-fibrils. The primitive fibril in a can be followed for a very long distance, being thicker at a distance from the cell. The coarse longitudinal processes y and & contain a large number of primitive fibrils which, as far as they can be followed, do not unite to one fibril. (After S. Apathy, Mitth. aus der zool. St. zu Neapel, 1897, Bd. xii., Ht. 4, Taf. xxvii., Fig. 7.) Fig. 3. Cells of the anterior corpora quadrigemina of the cat, to show neuro-fibrils. A, axone ; B, plexus of neuro-fibrils made up of fibrils entering the cell from the branches ; C, perinucleur plexus of fibrils in a cell which has been divided longitudinally; D, fibrils running from the plexus into the branches. (Ramon y Cajal, Bibliog. Anat, 1904, p. 264.) THE CEBEBEO-SPINAL NEBVOUS SYSTEM. 25 which pass into the cell body, and through it into other dendrites or into the axorie.^ These filaments are called neuro-fibrils. They were first demonstrated by Apathy in worms and have been more fully des- cribed in the hmnau neurone by Ramon y Cajal. (See Fig. 2.) It is supposed that the dendrites collect and transmit nervous impulses to the cell body ; and as they are niunerous and long it is evident that impulses from many sources may reach any one cell body. The axone or axis-cylinder process of the neurone is a single, long Fig. 4. <^' ^^ 1 -xt A, Cell dividpr' ] ".gitudinally ; B, surface of cell showing the "terminal feet " of Held, the manner of termination of the fine neuro-fibrils upon the surface of the cells. These fibrils come from a plexus of fibrils lying about the cells. (Ramon y Cajal, Bibliog. Anat., 1904.) branch, arising from a cone-like projection of the cell body. This cone-like projection, as well as its base within the cell body, con- tains no chromophile substance, but fine fibrils can be found within it by acid stains. It is supposed to transmit nervous impulses from the cell. It is straight and smooth in contour. It undergoes a slight contraction soon after leaving the cell body, but then becomes larger and remains uniform in diameter. Though in its course within the central organs it gives oif very fine collateral filaments at right angles to its direction, it does not branch in the nerve until it reaches its very end, some distance from the cell body. There it divides into a number of little end fibrils resembling a tuft or tassel. (Fig. 7.) In the central organs it is possible that these fibrils enter the dendrites of other neurones. It is possible that they end in free terminations ^ Van Gehuchten. March, 1905. Le Ndvraxe, January, 1904. Cajal, Bibliographic Anatomique, 26 TTtE STBUCTUBE OF THE NEHVOVS SYSTEM. which merely touch these dcDcl rites. It is also possible that they end on the surface of other cell bodies in what are known as the plates of Auerbach or the end feet of Held, but the exact connection is not yet determined. (See Figs. 3 and 4.) The collaterals terminate in the same manner. The termination of the axone in muscles is in the so-called muscle plate. In the other organs it ends in a fine plexus of nerve filaments. The axone is made up of many fine fibrils packed together, which are gathered from the cell body and from its dendrites. Fig. 5. Purkinje neurone of cerebellum. Many dendrites. One axone projecting from the body. Fig. 8 shows this structure. The size of the axone at its exit from the cell body varies in different neurones. Some axones extend long distances, as from the cortex of the brain to the lowest part of the spinal cord. This variety is known as Type I. of Golgi. (Fig. 9.) Some axones terminate near the cell body, dividing and subdividing mto-a sort of network. This variety is known as Type II. of Golgi. (Fig. 10.) Between these extremes all varieties are to be found. When the long axones leave the gray matter they are insulated from each other by receiving a medullary sheath or fatty coating, held in place by a thin membrane, but this they lose at their termination. In Fig. 9 this is shown. Some axones on nearing their termination ap- pear to split in two in a Y-shaped manner and each division has its own end tassel. If the nervous impulse passes out from the cell body THE CE-REBnO-SPINAL NEnrOtJS ST.'^fEM. S7 along the ax(>ne it is evident that it may reach many different destina- tions, because of tlie different places of ending of tlie collaterals and of Fig. 6. Neurones of the cerebral cortex, second layer. The long dendrite extends to the surface layer. Smaller dendrites go out from the body. The axons passes downward from the body, giving off collaterals. 28 TEE STBVCTVBE OF TEE NERFOUS SYSTEM. Fig. 7. Fig. 8. The terminal fibres of the axones ; a, axone bifurcating near its termination ; 6, c, d, terminal brushes. (Eamon y Cajal, Studien iiber die Hirnrinde des Meuschen, I., 55.) the terminal filaments. The terminal filaments of the axones are never continuous with other axones ; but impulses are probably conveyed from one neurone to another by the contact of axones with adjacent dendrites or with other cell bodies. The nutrition of both axones and dendrites is controlled by the cell body, for they atrophy when separated from it. Nerves are the axones of neu- rones collected into bundles and held together by connective tissue. II. The Second or Peripheral Class of Neurone. — This class belongs to the sensory part of the ner- vous system. It develops originally in the poste- rior spinal ganglia that lie outside of the spinal cord and in the homologous ganglia that are in Axone from the sciatic couuection witli the scusory crauial nerves and also nerve of a frog, showing in the ganglia of the sympathetic system. The fibrillary structure and a gensory ueurouc iu au early stage of develop- node of Eanvier. (Ver- .'I'l i i • i n ii ^orn ) ment is bipolar, a branch coming out ol each end of the cell body. (Fig. 1, 3.) Later the two branches appear to be fused together for a short distance, and thus in the adult the sensory neurone is pear-shaped and appears to send out a single axone which divides into two branches, passing in opposite directions. One of these branches finds its way outward to the periph- TBE CEBEBEO-SPINAL NEFvVOUS SYSTEM. 29 ery of the body, forming a sensory nerve, and terminates in a fine brush-like Expansion of filaments in the skin or in the tactile cor- puscles. The other branch finds its way inward through the posterior nerve into the spinal cord or brain axis, where it bifurcates, one Fig. 9. ft^s? Scheme of central motor neurone, f I. type of Golgi. ) The motor cell body, together with all its protoplasmic processes, its axis-cylinder process, side fibrils, or collaterals, and end ramifications, represent parts of a single cell or neurone, a.h., axone-hillock devoid of Nissl bodies, and showing fibrillation; nx., axis cylinder or axone. This process, near the cell body, becomes surrounded by myelin, m., and a cellular sheath, the neurilemma, the latter not being an integral part of the neurone ; c, cytoplasm showing Nissl bodies and lighter ground substance ; d., protoplasmic processes (dendrites) containing Nissl bodies; »., nucleus; n.', nucleolus; n.R., node of Ranvier ; .v./., side fibril; n. of n., nucleus of neurilemma sheath; lei., motor end plate or telodendron; m.', striped muscle fibre; s.L,, segmcntatioD of Lanterraann. (Barker.) 30 TBE STEUCTUEE OF THE NEEVOUS SYSTEM. portion passing downward and the other portion upward in the pos- terior columns of the spinal cord, or in the formatio reticularis of the brain axis. As these main branches pass up and down within the cord they give off at right angles little collaterals, and these collaterals, Golgi's cell of Type II. from the dorsal horn of the gray matter of the spinal cord of the newborn mouse. Even in such a neurone the axone is very easily distinguishable from the dendrites. The lat^ ter are only represented in part in the illustration. (After von Lenhossek.) together with the main branches, terminate in brush-like extremities within the gray matter of the posterior horn, either near their entrance or as far from their entrance as the posterior nuclei of the medulla oblongata, or at various levels in the brain axis. The sensory axone never terminates in a cell in the spinal cord or brain axis. It ends in brush-like terminations or tassels. In this form of neurone the cell body is situated about one-half way between the terminal extremities of its two great branches, and this fact of the interposition of the body in the course of a nerve tract, which, from the nature of the case, must be a continuous tract, sug- TEE CEBEBBO-SPINAL NEEVOUS SYSTEM. 31 gests that the function of the cell body is a trophic one. We have proof also t)iat an axone is not always centrifugal in the direction of its impulses. The structure of the cell body in this class of neurones is not dif- ferent from that in the first class ; but the chromophile granules are arranged about the nucleus in a series of concentric rings. (See Plate II., B., page 22.) Varieties of Neurones. — Neurones have been classified into : 1. Primary neurones, whose cell bodies lie in the central nervous system or ganglia and whose axones extend thence to some peripheral part of the organism, to a muscle, or to a gland, or to the skin, or to some sensory organ, and 2. Secondary neurones, whose cell bodies lie in the central nervous system and whose axones extend to some other part of the central nervous system, to terminate about a primary neurone or about another secondary neurone. Neurones have also been classified according to their function into 1. Centrifugal : (a) motor, (6) secretory, (c) trophic. 2. Centripetal or sensory. 3. Intrinsic or association. The diagram (Plate I., frontispiece) illustrates these various neurones and their relations to one another. Reflex acts may be performed by the primary neurones, but all con- scious sensations or voluntary acts involve the action of both primary and secondary neurones acting together. This action may be illus- trated by the analysis of a simple sensation, like the impact of a particle of dust in the eye, which causes a wink, a flow of tears, a conscious pain, and a voluntary effort to remove the particle. The sensation comes in from the eye along the primary sensory neurone, is trans- mitted (a) to the brain axis, where it reaches the primary motor neurone, setting up the centrifugal reflex acts of a wink and of secretion of tears, and (6) to the secondary sensory neurone, which transmits it upward to the brain. This secondary neurone may terminate about another secondary or association neurone, which then sends the impulse on to a secondary motor neurone, and this in turn sends its impulse down to the primary neurone, which transmits it to the muscle, causing the removal by the hand of the particle. There are many more complex acts, either automatic, like the act of breathing or sneezing ; or voluntary, like the act of walking or talk- ing, which call into play a vast number of neurones — centripetal, intrinsic, centrifugal — which cannot be so easily analyzed. But how- ever complex the act, the physical basis of all nervous and mental activity is this series of neurones. The arrangement of these neurones and their mutual relations will be considered in connection with the studies of diseases of the various parts of tlic nervous system. In that connection also the vascular supply of the nervous system will be described. 32 THE STRUCTURE OF THE NERVOUS SYSTEM. The Nutrition of the Neurone. — In order that these neurones may do their work they must have a normal structure and a normal capacity lor sustaining their nutrition when in activity. When a neu- rone is made to work it undergoes certain manifest changes. Hodge ^ was the first to describe alterations in the form and appearance of the cell and of its nucleus consequent upon its activity. There is a gen- eral diminution in the size of the cell, a lessened power to absorb stain- ing substances, which may be taken as a proof of the using up of its own substance and also changes in the appearance of the nucleus, which is decreased in size, and changes from a smooth and rounded outline to a jagged and irregular one. As the cell becomes changed in its structure by constant work, it becomes more and more exhausted, so that finally there comes a time when it is no longer capable of send- ing out impulses, and requires a period of rest to make up what it has lost of form and to regain a store of energy. Vas " and Mann ^ have described these physiological changes more exactly. Vas showed that mild stimulation of a cell caused a swelling of its body and of the nuclei and a clearing up of the central part of the cell by an apparent movement of the chromatin bodies to its periphery. Mann showed that functional activity of the cell is accompanied by an increase in the size due to imbibition of the lymph lying in the cavity about the cell, so that the cell at work fills up the cavity in which it lies. When activity goes on to the point of fatigue, then a shrivelling of the cell begins, first in the nucleus, then in the body. At the same time changes go on in the chromatin. During the period of activity the chromatin material is used up, so that a fatigued cell does not absorb staining material as does a cell at rest. In an exhausted cell the only stain is in and about the nucleus. In Plate II. these changes are shown. These results have been reached by stimulating cells to work in living animals either by electricity or by keeping up movements, such as running, or by exposing one eye to the light while the other was kept dark, and then contrasting the appearance of the cells made to work with those that were kept at rest. When a stimulated cell is allowed to rest it gradually resumes its original appearance ; but the period of rest must be adequate. During the period of reconstruction the chemical activities going on in the cell are numerous, and its power of assimilation of material furnished to it by the blood must be increased. If, however, anything interferes with this increased nutri- tion, such as an imperfect supply of blood or a deleterious substance of any kind in the blood, then the process of building up fails to take place, and the neurone cannot resume its function. The Pathology of the Neurone. — The fundamental fact at the basis of all forms of nervous disease is some change in the power of the neu- rone to continue its normal functions. This may be of a temporary character, whose nature is as yet not understood, and not attended by * Hodge. American Journal of Psychology, vol. ii. , p. 39. ^Vas. Ueber den Bau des Chromatins in den lymphatischen Ganglien, Archiv, fiir Mikroscopische Anatoraie, 1892, Heft 3, p. 375. 3 Gustav Mann. Journal of Anatomy and Physiology, October, 1894. THE CEREBROSPINAL NERVOUS SYSTEM. 33 visible alterations of form ; and may be succeeded by a resumption of natural action^ This is the hypothetical basis of functional nervous diseases. But the loss of power in the neurone may also be due to damage more or less grave to its structure, visible through the micro- scope, and often incapable of repair. This is the basis of organic dis- eases of the nervous system. The varieties will be carefully considered in connection with the various diseases studied. It is always to be remembered that the pathological changes which go on in the neurone are in no sense distinct from those occurring in the cells of other organs ; but the exact appearances are determined by the structure of the neurone as diiferent from that of other cells. We are at present able to see changes in the size and shape of the cell body and of its nucleus and nucleolus. We can distinguish, by Nissl's method of staining, changes in the chromophile bodies within the cell which con- sist in a gradually progressing disintegration of these bodies until they are resolved into a fine dust, and then entirely disappear, leaving the cell body a homogeneous, unstained mass. The protoplasmic substance in which these chromophile bodies lie also undergoes degenerative changes in disease ; but our staining methods are not adequate to show this. We can see a displacement of the nucleus of the cell body to one side as the chromophile bodies disappear, which indicates either a solution of the substance about the nucleus, and its consequent sinking by gravitation from lack of support, or an active migration. We can see the development of vacuoles within the cell body, indicating an absorption of both protoplasmic and chromophile debris. And we can see a gradual shrivelling of the cell body until its place is taken by a granular scar. The dendrites and the axone share in the pathological processes within the cell body, showing similar evidences of disinte- gration and destruction, or the axone alone may undergo these changes in lighter grades of atrophy. All forms of neurones appear to undergo similar pathological alterations. These may be classed together, no matter what their cause, as parenchymatous inflammatory processes or as parenchymatous degeneration. There are certain chemical changes which attend degeneration in the neurones which have recently been determined by Mott.^ He has ascertained that when nervous tissue degenerates, protagon or lecithin breaks up into choline, glycero-phos- phoric acid, and stearic acid. The protagon is converted into a fat of a different composition, the phosphoretted fat being changed into a non- phosphoretted neutral fat. This is a process of hydration, and is expressed by the following equation : C44H90NPO + SH^O = 2C,8H3A + C3H9PO6 + CsHisNOa (lecithin) (water) (stearic acid) (glycero- (choline) phos. acid) Parenchymatous degeneration may be attended by or may be due to inflammation in the connective tissue or in the neuroglia which every- where surrounds and supports the neurones. Such inflammation is 'Arch, of Neurology of the London County Asylums, 1899, vol. i., p. 185. 3 34 THE STRUCTURE OF THE NERVOUS SYSTEM. termed interstitial, and the mechanical compression exerted upon the neurones by an increase in the supporting substance may cause serious degeneration in the neurone. Again, in those forms of inflanmiation in which we have an emigration of leucocytes, we can often see these bodies invading the neurone body and apparently destroying its tissue. It has also been shown that bacteria may find lodgment within the neurone body or in its branches, causing irritation and destruction ; and it is not unlikely that in some cases the invasion of the neurone by leucocytes is really an attempt to remove these bacteria by phagocytosis. In Plate III. these changes are shown. All these pathological processes will be more fully studied in connec- tion with the various diseases to be considered. II. THE SYMPATHETIC NERVOUS SYSTEM. The sympathetic nervous system is the second division of the ner- vous system. Though closely connected with the cerebro-spinal sys- tem by the nerves, it appears to differ from it in its structure and to be independent of it in its function. It consists of a series of masses of gray matter enclosed in capsules termed ganglia, made up of spheri- cal cell bodies and joined to each other and to the so-called involuntary organs of the body — the heart, bloodvessels, and lymphatics ; the lungs ; the digestive, hematogenetic, secretory, and reproductive sys- tems — by nerves of a peculiar type whose axones lack insulating material or medullary sheaths, and hence are gray instead of white. The sympathetic system is subdivided into : 1. Two great cords containing ganglia, which lie on the sides of the vertebral column and are connected with the spinal cord on one side and with the plexuses on the other. 2. Three prevertebral plexuses, the cardiac, solar, and hypogastric, which are masses of ganglia connected with the viscera. 3. Many peripheral plexuses connected with individual organs. 4. Terminal monocellular ganglia scattered through the viscera. 5. Sympathetic nerve fibres which join these plexuses and ganglia together and also establish their connection with the spinal cord and brain. Many groups of cells lying in the central region of the spinal cord and in the lateral part of the central gray matter receive and give off nerve fibres which pass by way of the nerve roots to the spinal nerves, and thence to the sympathetic cords and ganglia.^ These nerves are both sensory and motor ; hence it is probable that some part of the activity of the sympathetic system is controlled by the spinal cord. The connection of the sympathetic system with the brain is made by the great vagus or pneumogastric nerve. The action of the sympathetic system in regulating the vegetative functions and the acts of the heart, bloodvessels, and lungs is usually ^Onuf and Clollins. Archives of Neurology and Psychopathology, 1900, vol. iii., No. 1. /' ..« ,■ .■■(llllllM-fc.l THE SYMPATHETIC NERVOUS SYSTEM. 35 automatic and unconscious ; but this activity may be reflected upon con- sciousness id" an indefinite manner and cause changes in the emotional state and in the general feeling of comfort. Head, who has made a careful study of this subject/ affirms that exaltation or depression of spirits, hallucinations, a state of suspicion, and a change of character may be induced by the reflected pains of visceral disease. He ascribes many of the symptoms which we term hysterical to such sources. It is also a fact that unusual states of consciousness, expectant attention, and conditions of the mind and brain induced by hypnotic suggestion may in turn produce physical changes in the vascular and vegetative organs. These can be explained only by admitting a control by the brain of these organs through sympathetic channels, but in an uncon- scious and involuntary manner. ^The Gulstonian Lectures for 1901. Brain, 1901, vol. xxiv., p. 345. CHAPTER III. THE PERIPHERAL NERVOUS SYSTEM. The peripheral nervous system is made up of the twelve cranial and thirty-one spinal pairs of nerves which connect every part of the body with the central nervous system ; viz., the cerebral axis and the spinal cord ; and also of the sympathetic nervous system with its various ganglia. The nerves, as already shown, are merely prolonga- tions of the neurones ; motor nerves being the axones of the motor neurones situated in the anterior gray horns of the spinal cord and in the gray matter of the medulla, pons, and crura cerebri ; sensory nerves being the axones of sensory neurones situated in the posterior spinal ganglia and in the ganglia of the cranial nerve roots ; sympa- thetic nerves being the axones of the various sympathetic neurones situated in their ganglia. THE HISTOLOGY OF A NERVE FIBRE. When a nerve trunk is dissected, the connective-tissue sheath or perineurium enclosing its fibres torn away, and the individual fibres set free by tearing the finer connective-tissue strands or endoneurium which bind them together, it is possible to distinguish certain parts by means of appropriate methods of staining. There is, first, the axone or axis cylinder. (Fig. 11.) This is made up of a number of primitive fibrils longitudinally arranged and con- tinuous throughout the length of the nerve. The fibrils are cemented together by a substance which appears finely granular. Each axone comes from a single nerve cell ; the individual fibrils come from the cell body and from its dendrites. The fibrils pass together in the axone to the periphery and there terminate, the individual fibrils branching out in various directions, and joining with other fibrils from other axones to form a fine plexus within the organ to which the nerve as a whole has gone. It has been thought possible to trace individual fibrils of the plexus into individual epithelial cells in various internal organs and in the skin. Other axones can be traced directly to ter- minal organs, such as the terminal plates upon the muscle and the ter- minal bulbs and corpuscles in the skin. In these no division or branching of the fibrillary constituents of the axis cylinder has been discovered. Their termination in each of the organs of special sense is still different. The diameter of an axis cylinder varies from 2.6 /z to 7.5 /;!. It is no longer believed that the longest fibres have the great- est diameter. There is, secondly, the myelin sheath surrounding the axis cylinder. 36 TEE EI8T0L0GY OF A NEBVE FIBRE. 37 (Fig. 11.) ,^This is not a continuous tube, but consists of a series of short tubes or interannular segments placed end to end. These seg- ments vary in length from 0.1 mm. to 1 mm. and are longer on thick fibres. The point of junction of two adjacent segments is indicated by a constriction in the contour of the nerve fibre, the node of Ranvier, and if the fibre be stained with osmic acid the myelin will be seen to be deficient at these nodes. (Fig. 8.) If, by any means, the nerve fibre be broken the myelin in any segment will run out and collect in drops, showing that it is a semifluid substance of fatty nature. Node of Ranvier. '"Neurofibrils ' Cement stibstance "Axis cylinder ^Line of Schmidt- Lantermann ' — Schwann's corpuscle Schwann's sheath~i •Medullary sheath Mauthner's sheath .Schwann's sheath Axis cylinder~-i Diagram of the structure of a medullated nerve fibre, showing two different views Concerning the relations of the sheaths of Mauthner and of Schwann. Compare the right and left sides. (Symonowicz. ) The myelin sheath is not a necessary constituent of all nerve fibres, for the majority of the nerves of the sympathetic system are devoid of such sheaths. Nor does the myelin sheath of the nerves of the cerebro- spinal system extend from end to end of the axone. For the axone first receives its sheath at some little distance from the cell from which it issues ; and at its termination, where it breaks up into branching 38 TEE PEBIPHEEAL NEHVOUS SYSTEM. fibrils, the myelin envelope ceases. Throughout the course of the nerve, however, the myelin sheath is present in the cerebro-spinal nerves, forming a protecting envelope and probably acting as an insu- lating and nutrient substance as well. There is, thirdly, the sheath of Schwann — a connective-tissue mem- brane surrounding the myelin sheath. (Fig. 9.) This, like the last, develops in segments which become cemented to one another at the points of constriction of the fibre ; but finally the continuity between adjacent segments becomes complete, so that in an adult fibre the Fig. 12. Cross-section of a nerve, showing five bundles of nerve fibres. sheath is continuous over the node of Ranvier, and here the sheath of Schwann is the only covering of the axis cylinder. On the inner side of this sheath, and half-way between two nodes, a nucleus is found. The sheath of Schwann extends almost to the terminal filaments of the axone, covering them for a little distance after they have lost the myelin sheath. By appropriate staining fine lines may be shown pass- ing between the outer and inner layers of protoplasm through the myelin sheath ; the so-called incisures of Schmitt-Lantermann. These have been considered little trabeculse of the protoplasm within the seg- ment, in the midst of which the myelin lies. Eecent staining methods seem to indicate that they belong to the sheath of Schwann and are connective tissue. The layer of protoplasm lying against the axis cylinder is the layer which was formerly described as the sheath of Mauthner. Some authorities consider that a layer of connective tissue similar to the sheath of Schwann surrounds the axis cylinder, but this is still uncertain. Two views of its relations are shown in Fig. 11. Thus the nerve fibre consists of a central conducting strand sur- rounded and insulated by a series of cylinders placed end to end and joined to one another. SYMPTOMS OF DISEASES OF THE NEBVES. 89 Nerves. ^ Individual nerve fibres are associated in bundles held together hf fine connective-tissue cells whose nuclei in a carmine or fuchsin-stained preparation can be seen lying always adjacent to, but outside of, the sheath of Schwann. This has been called the endo- neurium, while the connective-tissue sheath surrounding the entire bundle is named the perineurium. The connective tissue holding numerous bundles together is called the epineurium, and this forms the firm, glistening sheath of the nerve. Capillary vessels with free anasto- moses run within the nerve, their walls lying adjacent to the individual fibres, and thus affording a perfect supply of nutriment. Lymph spaces also have been demonstrated within the nerve sheath, but not among the fibres. That the interfibrillary spaces of the endoneurium, however, open into these perivascular lymph spaces is probable from the fact that they do so in other organs. While it is evident that the nutri- ment of the axis cylinder is derived from the circulatory fluids, it is probable that it is only at the nodes of Ranvier that the absorption takes place, since elsewhere the myelin sheath interferes with osmosis. Thus, if the nerve be put in nitrate of silver, it is only opposite the nodes that the axis cylinder becomes stained. There is no histological difference between motor and sensory nerve fibres. SYMPTOMS OF DISEASES OF THE NERVES. Parsesthesia. — A very slight pressure upon a nerve, of short dura- tion, may cause disagreeable tingling and numbness of the skin, which is felt in the distribution of the sensory filaments of the nerve. This is an experience common to everyone when from pressure on the sciatic nerve the foot is said to be asleep. Such sensations of numbness often occur in the arm and hand, or in the foot and leg at night during sleep and wake the patient, who may be alarmed, and from a recurrence of the symptoms, anticipate paralysis, especially if the numb sensation is temporarily attended by awkward movements or by a feeling of weakness. Inasmuch as the relief of the pressure is followed by a rapid recovery from the symptoms such sensations are of no importance. They are due to a slight impairment of nutrition in the nerve probably consequent upon a venous congestion, for a similar set of sensations can be caused by tying a string tightly about the finger or wrist and caus- ing a stasis of venous blood. Some persons seem to be especially sub- ject to such symptoms. Such a condition does not ordinarily amount to a neuritis, but if it is long continued it may produce it, as is seen in cases of musculospiral palsy from sleeping on the arm m a state of intoxication. A slight general numbness of all the fingers in both hands, and even of the hands and wrists af5 well, occasionally occurs in attacks of short duration or may become permanent. This is noticed chiefly in women whose hands are constantly immersed in hot water or who use the hands continuously for hours in any employment. It has been called acroparcesthesia.^ It is due to a slight impairment of nutrition in the ^Dana. New York Medical Kecord, January 20, 1891. Collins, New York Med- ical Record, December 'S, 1901. 40 TEH PEBIPEEEAL NEBVOUS SYSTEM. nerves, and if the motor as well as the sensory nerves are aifected it is attended by a sense of weakness and lack of skill. Disturbances of circulation are sometimes the cause, as in numbness from pressure. These symptoms are variable in duration and may continue if the cause is not removed ; but they never go on to any serious disease, and hence are of little importance. Rest from work and massage are the best remedies. Pain. — The effect of an injury or wound of a nerve or plexus of nerves is immediate pain at the point of injury, pain or disagreeable sen- sations, such as numbness or prickling, referred to the regions from which the sensory filaments in the injured nerve come, anaesthesia in this region to all forms of sensation, of temporary or permanent duration, occasionally attended by trophic disturbances, and paralysis of the muscles to which the motor filaments in the injured nerve pass. The paralysis is soon followed by a loss of faradic contractility in the muscle and by a loss of its mechanical excitability to percussion, and later by a progressive atrophy of the muscle. At the point of injury to the nerve the symptoms vary. They differ when the skin is unbroken, when the nerve is exposed to the air, and when the wound is infected. In the first case, while there may be slight tenderness to pressure there may be no spontaneous pain. Thus in a case of bilateral ulnar paralysis from pressure exerted on the nerves of the arms during a long surgical operation, where the arms hung over the sides of the operating table for an hour and their weight against the sharp edge caused the injury, the patient had no pain at all and no tenderness at the point of injury. In the common form of injury to the musculospiral nerve from pressure on the arm by the head during the heavy sleep of intoxication, the patient wakes with wrist-drop, but has no pain. In facial palsy there is rarely pain behind the ear. Thus where the injury is produced by long-continued slow pressure sufficient to destroy the nutrition of a nerve there may be no pain. On the other hand, pressure on a nerve by a tumor or pressure by a pregnant uterus on the lumbar and sacral plexus may give rise to severe pain, both local and distal. If the nerve is lacer- ated suddenly, as by a strain or a blow or by a fracture of a bone tear- ing it, there is always severe pain at the point of injury, and this pain is usually much increased if the nerve is exposed to the air in an open wound. In these cases also the subsequent connective-tissue growth and thickening about the wounded ends is tender and may cause great pain, the finer filaments becoming entangled in the scar tissue. The pain after injury is often felt not only at the point of injury, but distally in the entire peripheral distribution of the nerve. Thus in a case of ulnar nerve injury occurring with a fracture at the elbow there was pain of an agonizing kmd in the inner condyle of the humerus and also severe shooting pains down the inner side of the forearm and into the hand and little and ring fingers ; these pains grew worse for several days and continued for many weeks, with intense tenderness in the entire ulnar nerve distribution, any touch in this PLATE IV The Distribution of Sensory Nerves in the Skin. (After Flower.) The areas of the skin supplied by the cutaneous nerves are shown in finely dotted outline. The circles on the trunk show areas occasionally anaesthetic in hysteria. The lines across the limbs at ankle, knee and thigh, wrist, elbow and shoulder show the upper linnits of anaesthesia in multiple neuritis of varying degrees of severity. SYMPTOMS OF DISEASES OF THE NEBVES. 41 region causing agony. Ulcerations of the skin of the fingers and hand occurred later and the skin became glossy. This condition remained stationary until by operation at the elbow the nerve was released from a mass of callus, and thus was freed from pressure, when all the symp- toms gradually subsided and recovery ensued. The pain and tenderness caused by an injury of a nerve are not always limited to the distribution of that nerve, as one would expect. Thus in a case where a perforating wound of the sole of the foot had left a small piece of rubber shoe embedded in the flesh the orig- inal injury was slight, the wound healed, and for a long time no inconvenience was felt; but four years later pain and tenderness began again in the scar and extended up the leg and thigh until the entire limb became painful, hypersensitive, and useless from the intense tenderness, and even the lower spine was sensitive and pain- ful. These symptoms all subsided rapidly when, by operation, the piece of rubber was removed. It was found to be surrounded by a little mesh of fine nerve filaments holding it like a basket. The rapid recovery showed that there was no true ascending neuritis, as had been supposed. In such cases the continued local pain causes a hypersen- sitive state of the central organ by a summation of impressions, and this shows itself by a general pain. In many cases of neuritis of one cord of the brachial plexus the pain in the entire arm renders it use- less. The pain in neuritis is increased by pressure upon the affected nerve. It is also intensified by motion of the limb, especially if this causes a stretching of the nerve. Anaesthesia. — The anaesthesia following injury of nerves is more pronounced and extensive immediately after the injury than it is later. There appear to be fine anastomoses of the sensory filaments of nerves in the skin, and thus sensations ordinarily conveyed by one nerve may, if that nerve be injured, pass by adjacent nerves. Such anastomoses seem to be more complete in some individuals than in others, hence in two patients with similar injuries the anaesthesia may be different in extent. Thus after injury of the musculospiral nerve it is very • common to find sensation in the back of the hand near the thumb perfectly restored within a week, while the wrist-drop may last some months. After a division of the median nerve above the wrist I have seen the anaesthesia progressively diminish in the hand long before the union of the ends of the severed nerve had restored the muscular power in the flexors of the fingers. After excision of one branch of the tri- geminal nerve for neuralgia, sensation returns in the face in a consider- able part of the region which is anaesthetic immediately after the oper- ation ; but a small area of anaesthesia is usually left as a permanent result of injury to any sensory nerve, and this includes analgesia and thermo-anaesthesia. A loss of muscular sense rarely, if ever, follows a nerve injury. This is because many different sensations combine to produce this sense, deep and superficial nerves from muscles, liga- ments, and articular surfaces all conveyiiig impressions which are coordinated in tlie spinal cord before being sent up to tlie brain and appreciated as muscular sense. 4^ TEE PEBIPHEEAL NEBYOVS SYSTEM. Head ^ explains this in an interesting manner. He believes that nerves contain different systems of fibres, some transmitting sensations of pain and of extremes of temperature which he terms protopathic sensations, others transmitting sensations of touch and of slight temperature which he terms epicritic sensations. He has shown that after an injury of a nerve the distribution of these different forms of anaesthesia varies somewhat and that during recovery protopathic sensations return before epicritic sensations. The unit of protopathic supply lies in the pos- terior roots, but of epicritic supply in the peripheral nerves. The more nearly a peripheral nerve represents the supply of one posterior nerve root the more definite will be the border of the analgesia; the more the representation of posterior nerve roots in a nerve the less definite will be the protopathic symptoms. He believes that the sensi- bility of deep parts, of joints and muscles is transmitted by the sensory filaments in motor nerves. As regeneration occurs in the injured nerve sensations gradually return in the previously insensitive region, but at first all sensations are attended by an abnormal sense of tingling or numbness which is disagreeable. This tingling or numbness is often perceived during the entire course of the disease. It is then due to irritation in the central end of the injured nerve, the pathological irritation being referred erroneously to the region whence the irritated filaments come. Such numbness may be attended by pain of a sharp, shooting character, and in some sensory nerves, notably the trigeminal and sciatic, this pain may be the most important symptom of neuritis from pressure. Thus in a case of osteosarcoma of the pelvis, sciatic neuralgia, supposedly functional, preceded for seven months all other symptoms. In a case of osteoma of the skull near the optic chiasm trigeminal neuralgia preceded by one year the optic atrophy and other symptoms. When septic infection occurs in the injured nerve this pain is more intense than under other circumstances, and it is then always attended by a state of hyperalgesia in the affected area. Such a state is known as anaesthesia dolorosa, for pain is caused by any touch in the sensitive region, even though the touch itself is not perceived. Trophic Disturbances in the anaesthetic region occur more commonly in septic cases, but they may develop in any case. They are usually produced by neglected injuries of the insensitive region, which become infected, a slight pin prick or a scratch being capable of originating a serious trouble if not disinfected at once. Pain is nature's means of protection in health, giving warning of injuries or diseases and leading to their care ; but from insensitive regions no warnings can come, and no protection is therefore given. But this is not a sufficient explana- tion for the occurrence of such trophic symptoms as arise in neuritis, either traumatic or spontaneous. We see peculiar bullous eruptions, herpes, acne or eczema, extraordinary vasomotor conditions, with alter- nate flushing and pallor of the skin attended by intense heat and cold, a dryness and thinning of the skin, with a peculiar, smooth, shining 1 Brain, Part II., 1905. Also No. 121, 1908. Neck in usclea SYMPTOMS OF DISEASES OF TSE NEBVFS. 4S appearance termed glossy skin, unusual sweating, which may be acid and fetid, or 'hardening and thickening of the skin, or abnormal growth of hair, or falling of the hair ; a defective or irregular growth of the nails, which may be ridged, curved, and marked with white plaques in the region supplied by the affected nerve. Very rarely acute gangrene has been observed, chiefly in old persons. The occurrence of these symptoms has given rise to the hypothesis of trophic nerves, whose function is to regulate nutrition and repair and whose injury results in processes of disease in the derma and its adnexa. Such trophic symp- toms develop only when the nerve injured is a sensory nerve. The trophic disturbances seen in cases of locomotor ataxia and syringo- myelia are evidence that central as well as peripheral lesions may be attended by such symptoms ; but even in these diseases it is the sen- sory portion of the nervous system to which the pathological change is limited. The true explanation of trophic changes is to be found not in the hypothesis of an injury to trophic nerves, but in the fact that ordinary sensory impressions are interrupted or perverted, and nature, lacking its accustomed guide to repair and misled by abnormal impres- sions, produces effects which are needless under the circumstances or fails to produce those which ordinarily would be required. Thus, an increased secretion of sweat may be a natural thing under a certain condition — e. (/., heat — when unnatural it may result from a series of abnormal sensations of pathological origin, being received by the central organs which are incapable of distinguishing natural from arti- ficial impulses. A bed-sore is usually produced by pressure or irrita- tion of the skin, not perceived, and so not removed or repaired. If, by extraneous care, such pressure and irritation are avoided, as in health would occur by change of position and care of the skin, bed- sores may be entirely prevented. In a patient of my own, suffering from transverse myelitis with pronounced tendency to bed-sores, the simple expedient of turning him every half hour day and night and wiping off the skin lightly each time that he was turned prevented for four years the development of any skin abrasion. I have never seen trophic skin affections occur in the course of neuritis when the parts were properly protected from injury, and slight injuries were antisepti- cally treated. It must be stated, however, that other theories than the one here offered have been advanced to account for the trophic symptoms in neuritis. They are ascribed to vasomotor disturbance. Vasomotor Symptoms. — Some authors believe that in the course of neuritis the vasomotor nerves are diseased and that their lesion results in an irregularity of blood supply to the part concerned. This irregularity consists of an active hypersemia, as is proven by experi- mental section of the sympathetic nerve to a rabbit's ear, which causes extreme dilatation of the vessels and a rise of temperature in the part ; but it has never been demonstrated that a mere condi- tion of congestion goes on to a condition of inflammation unless other causes, chiefly septic or microbic, enter as a factor. Further- 44 TEE PEBIPEEBAL NEBVOVS SYSTEM. more, it is well known that the bloodvessels are under the control of vasomotor ganglia in their walls, which regulate their calibre even after division of the fibres which connect these ganglia with the spinal cord. Thus, after division of the sympathetic in the ear of a rabbit, the congestion gradually subsides in the ear and the temperature becomes normal. Another argument against the vasomotor origin of trophic disturbances is derived from the study of cases of erythromelalgia^ This is an affection first described by Weir Mitchell, characterized by a dilatation of the arteries in the extremities resulting in an extreme condition of redness and sensation of heat and pain. It is an affec- tion of the hands and feet and occasionally involves the arms to the elbows, and the legs to the knees. After hours of extreme heat and flushing the extremities may suddenly become cold, pale, or blue and shrivelled up, presenting the appearance that the hands present after long soaking in hot water, and this alternation of distention and con- traction of the bloodvessels constitutes the symptomatology of the dis- ease. It appears to be a pure vasomotor neurosis, but it is rarely, if ever, attended by any trophic disturbances of the skin or of the nails, such as occur in neuritis, and no pathological observations are at hand to establish the hypothesis that there is a true neuritis of the vaso- motor nerves. For this reason the theory of the vasomotor origin of trophic affections appears to be incredible. Head affirms that trophic disturbances occur only when the protopathic system of nerves is in- volved and this accords with the theory I have already advanced. Paralysis, — The paralysis which results from traumatic neuritis is limited to the muscles supplied by the injured nerve, and is total only in those muscles which have no collateral nerve supply. It is a flaccid palsy, and if it results in any stiffness of joints or limbs or deformity soon after the injury, this is due to contraction in the healthy unopposed muscles and not to contracture in the paralyzed ones. After a long- continued paralysis, however, attended by atrophy, a shortening of the weak muscle may occur. Thus, in cases of facial palsy which do not recover, a contracture is not very rare, causing stiffness of the face. In such paralyzed muscles no mechanical or reflex motion is possible. Percussion of the muscle or of its tendon, therefore, fails to produce any response. Electrical Changes. — Electrical changes soon develop in the mus- cles paralyzed, usually within four or five days of the time of injury. These are of several kinds and are known as the i-eaction of degenera- tion, partial or complete. In health a muscle may be made to contract by sending a galvanic or faradic current through its nerve, or by applying either current directly to the muscle. In neuritis the nerve becomes inexcitable to any form of electricity and the muscle shows changes in its excitability. A number of different conditions have been observed. Sometimes there is a simple diminution of excita- bility, and then a very strong faradic or galvanic current is needed to produce contractions. Frequently all faradic excitability is lost, and then the muscles react to a galvanic current only. They may be at Muscles of hacK' -» c; fy SYMPTOMS OF DISEASES OF THE NEBVES. 45 first hypersensitive to galvanic stimuli, but later it may require a very strong galvaiiic current to produce contraction. The contraction is rarely quick, as in health. It is sluggish and vermiform. In some cases the normal polar reaction is found, and the closure of the nega- tive pole applied to the muscle produces stronger contractions than the closure of the positive pole. This is termed a partial reaction of degeneration. In many cases the contraction of the muscle when stimulated with the positive pole is greater than when stimulated with the negative pole. This is termed a complete reaction of degeneration. A loss of faradic irritability and a marked decrease in the galvanic irritability of the muscle are, therefore, important symptoms of neuritis. As the disease goes on to recovery a gradual increase in the galvanic irritability occurs — a fact which is often of much aid in prognosis if careful measurements of the strength of the current used be made by the galvanometer. It is possi- ble to record such measurements upon charts, and thus to obtain an electric curve for each muscle which is paralyzed.^ These curves enable one to judge of the progress of the case very accurately, and when the line is advancing steadily toward the normal point, after a great deflection or after a stationary level, the prognosis is favorable. It is to be remembered, however, that voluntary power always re- turns some time before electric reactions become normal. The extent of the paralysis in any case is determined by the distri- bution of the nerve aifected. Thus in facial palsy all the muscles of the face except the orbicularis oris are paralyzed. In musculospiral paralysis the extensors of the wrist and fingers are totally paralyzed and the supinator longus is partially paralyzed. In peroneal paralysis the muscles which abduct and lift the foot are useless. The muscles supplied by the various nerves of the brachial, lumbar and sacral plexuses are shown in Plates V. and VI., on pages 42 and 44. From an examination of any patient it is easy to determine what muscles are paralyzed. Comparison of the facts elicited with these plates will enable one to reach a conclusion as to which nerve or nerves or plexus is affected. But if the extent of the paralysis cannot be ex- plained by the lesion of one or more nerves, it is probable that the paralysis is of spinal rather than nerve origin. The duration of the paralysis depends wholly on the question of the possibility and time of regeneration of the nerve. In case a nerve is divided and at once reunited this paralysis may disappear in a couple of weeks. In an ordinary traumatic case or after inflammation, as in facial palsy, six weeks may be the limit. In other cases a long time, six or eight months, elapses before the paralysis passes off. The further the injury from the end of the nerve, the longer the time to recovery — a fact which gives support to the hypothesis of Ranvier that the new nerve must grow out from the central end at the point of injury and find its way down the old sheath to the muscle. In musculospiral paralysis from pressure over the humerus seven months is the average * Starr, Journal of Nervous and Mental Disease, February, 1887, vol. xiv. 46 TEE PEEIPHEBAL NERVOUS SYSTEM. duration to recovery. In case, however, some obstruction to repair exists the paralysis is permanent. Hence scar tissue which prevents the new filaments from developing must often be removed in order to effect a cure. Spasm of the muscles supplied by the injured nerve occasionally occurs, but is a rare symptom. It is usually a reflex phenomenon due to sensory irritation and not to any pressure on the motor nerve. In a gunshot injury of the ulnar nerve cramps in the hand and extensors of the wrist have been noticed (Raymond). In the so-called occupa- tion neuroses which some authors ascribe to neuritis, such cramps are common, but in ordinary cases of neuritis they do not occur. While the statements of the symptoms occurring in neuritis thus far made are generally true, it is found that neuritis in different nerves produces diverse symptoms, hence a special consideration of neuritis of the different nerves is necessary. See Chapter VII. Diagnosis. — The diagnosis of neuritis usually presents no diffi- culty. The limitation of the pain, anaesthesia, and paralysis to the distribution of a single nerve, and the tenderness at some point along its course are characteristic of the affection at the onset, and within a week of the beginning the development of the reaction of degeneration in the muscles supplied by the nerve affected makes the diagnosis com- plete. In Plate IV. the area of the skin related to each nerve is shown. If a neuritis is present the pain parsesthesia and anaesthesia are limited to that part of the skin which corresponds to the affected nerve. If Plate IV. be compared with Plate VII. it will be evident that the area of skin related to each nerve is very different from the area of skin related to each spinal segment. If in any case of pain, parsesthesia or anaesthesia the distribution of these in the skin is rather that of spinal than of nerve relation, the diagnosis will be of spinal rather than of nerve disease. On page 55 the grouping of the paralyzed muscles in spinal lesions is given. This may be contrasted with the grouping indicated as characteristic of nerve lesions in Plates V. and VI. on pages 42 and 44. The exact determination of the extent of tlie paralysis will enable one to reach a conclusion as to whether it is due to nerve or to spinal lesion. Thus if all the small muscles of the hand are paralyzed and atrophied it will be much more likely that the lesion is in the first dorsal segment of the cord than in the ulnar and median nerves ; and if the long flexors of the fingers escape it will be evident that the median nerve is not affected ; hence the lesion must be spinal. DESCRIPTION OF PLATE VII. This diagram has been constructed by combining the results of a large number of cases of transverse lesion of the cord at different IcTcls studied or reported during the past twelve years. The dorsal areas are taken from Head. PLATE VII Areas of Anassthesia upon the Body after Lesions in the Various Segments of the Spinal Cord. The segments of the cord are numbered : C 1 to VIII, D I to XII, L i to V, S I to 5, and these numbers are placed on the region of the skin supplied by the sensory nerves of the corresponding segment. CHAPTER IV. THE SPINAL CORD. THE DIAGNOSIS AND LOCALIZATION OF SPINAL-CORD DISEASES. The spinal cord is a long cylindrical organ extending from the medulla oblongata at the base of the brain downward through the vertebrae to the level of the second lumbar spine. It is surrounded by two membranes — the pia mater, which contains its numerous bloodvessels, and the dura mater, which acts as a thick protecting sheath, and which lies against the bony walls without being closely adherent to them. From the sides of the spinal cord the spinal nerves come out in pairs, each nerve having two roots — an anterior root, which issues from the antero-lateral groove, and a posterior root, which enters the postero-lateral groove. On each posterior root lies an oval body — the posterior spinal ganglion — which contains the sensory neurone bodies. The cord has two enlargements, the cervical and lumbar, opposite to the exit of the nerves to the extremities. It is made up of thirty-one segments, each segment consisting of a mass of gray matter connected with a pair of spinal nerves which pass to a definite region of the body ; and each segment is joined to the others and also to the braiu by means of nerve tracts running through the various columns which surround the gray matter of the segment. The spinal cord is an organ that has two distinct functions : namely, (1) the function of controlling directly the various parts of the body with which it is joined by means of its pairs of nerves ; and (2) the function of conducting impulses to and from the brain. In consider- ing, therefore, the functions of the cord and the symptoms that arise from disturbance of these functions, it is necessary to understand not only the function of each spinal segment so far as its motor mechan- isms, its sensory connections, its vasomotor and trophic functions, and the distribution of its particular pair of spinal nerves, are concerned, but also the functions of the columns of the cord that pass through the particular segment concerned, and that transmit motor impulses downward from the brain and sensory impulses upward toward the brain. In this chapter each symptom that may present itself in any case of spinal-cord disease will be considered, and these symptoms, one by one, will be referred to the anatomical structure whose function is im- paired, and thus it will be possible to determine what the pathological import of each symptom may be. This will demonstrate how readily 47 48 THE SPINAL COED. Fig. 13. the localization of spinal affections may be determined, and from this localization and the combination of the symptoms it will be easily pos- sible to arrive at a diagnosis of any form of spinal-cord disease. Symptoms. — The symptoms of spinal-cord disease are paralysis, changes in reflex activity, disturbance in the control of the sphincters, alter- ations of gait and of posture, defects of sensation, ataxia, pain, and trophic disorders. Paralysis is a condition of weak- ness or total loss of power in a muscle. It may be limited to a single muscle, it may affect a group of muscles, or it may affect a limb in its entire muscular apparatus. Paralysis may be due to an interfer- ence with the transmission of volun- tary impulses from the motor centres of the brain to the motor cells (neu- rones) of the spinal cord, which im- j)ulses pass in the motor tracts of the spinal cord — viz., in the lateral pyra- midal and anterior median columns. Paralysis may also be caused by a destruction of the motor neurones situated in the anterior gray horns of the spinal cord, whose axis-cylinder processes (axones) pass directly to the muscles through the anterior nerve roots and the motor nerves of the body. There are, therefore, two forms of spinal paralysis, quite clearly dis- Diagram of the spinal cord, showing the motor mechanisms. P»/, lateral pyramidal column ; A3f, anterior median column transmitting voluntary im- pulses from the right pyramid of the medulla to the motor cells of the anterior horns of the cord, whence motor nerves issue in the motor root {m) ; S, sensory nerve sending its fibres into the posterior horn and into the root zone of the column of Burdach (B), whence fibres pass forward to reach the commissural cells (c) and the association cells (o), and the motor cells (to). These form the motor mechanism of reflex and automatic acts. L, fibres of limiting layer, consist- ing of association neurones between various levels of the cord, being branches of the cells («). PLATE VIII The Spinal Cord, its Nerves and Arteries. ( Adamkiewicz.) fi. Basilar. V. Vortobral. I'r. Vcrtcbro-cerebellar. Vna. Vcrlpbro-spiniilis anterior. Sa. Spinalis anterior. M. Magna-spinalis. Ic. V'lrf.t ccrvifal nerve. Vsp. Vertebro-.spinali.s posterior. Sp. Spinalis posterior, fif/. Fourth ventricle. tbt: diagnosis of spinal-coed diseases. 49 tinct from one another according as the lesion affects the first (cortico- spinal) or th9» second (spinomuscular) parts of the motor mechanism. The diagram (Fig. 13) shows these two parts of the motor mechan- ism. The motor neurones of the brain send their axones downward through the pyramids of the medulla, where a partial decussation takes place, the majority of the axones from one pyramid crossing into the opposite lateral pyramidal tract of the cord (Fig. 13, Py), and the remainder passing directly into the anterior median column. (Fig. 13, AM.) As these axones pass downward they terminate at various levels in the anterior gray horns of the spinal cord, their terminations taking the form of fine brush-like expansions which surround the motor neurones of the cord (see page 24), each filament coming into contact with the protoplasmic processes (dendrites) of those neurones, but not being continuous with those dendrites. As these motor axones pass downward to their termination they give off small fine branches (collaterals) at right angles to their course, and these collaterals ter- minate in the same manner in brushes around the motor neurones of the cord. Thus a voluntary impulse starting from a motor neurone of the brain may reach several motor neurones of the cord. As the motor tract passes downward through the cord it becomes smaller and smaller, but few fibres remaining in the anterior median column below the dorsal region, while some fibres of the lateral pyramidal tract extend to the very last segment of the cord. This motor tract is much more highly developed in those animals which make use of the digits of their extremities. Thus in elephants the pyramidal tract is small, while in man it is most highly developed and large. Hence, it is evident that this tract transmits those volun- tary impulses which are concerned in the finely adjusted movements of volition. Each axone in this tract being a process of a motor neurone of the cerebral cortex, is dependent upon that neurone for its nutrition. If the cortical neurones are destroyed or if the axone is separated at any point in its course from its neurone, it degenerates to its terminal brush. Hence a degeneration of the motor tracts of the cord may be due to disease in the brain, as in hemiplegia, as may be due to any transverse lesion of the cord which separates the axones from their neurones. The following figures (14 to 17) of specimens demonstrate descending degeneration of the motor tracts in the cord. Figs. 14 and 15 show descending degeneration consequent upon hemiplegia of slight and of severe type, the anterior median column on the side of the brain lesion and the lateral pyramidal tract upon the other side being degenerated. Figs. 16 and 17 show descending degeneration in both lateral pyramidal and anterior median columns consequent upon a transverse myelitis located at some distance above the level from which the section is taken. Paralysis of the cortico-spinal type, which is always due to a disease in this first element of the motor mechanism, has the follow- ing characteristics* The muscles paralyzed are partially and not 4 Fig. 14. Section of the siiinal cord, at the sixth cervical segment, showing descendiug degeneration in the left lateral pyramidal and the right anterior median columns, after a small lesion in the motor tract of the right cerebral hemisphere. Fig Section of the spina! cord, at the fifth cervical segment, showing descending degeneration in the left anterior median and right lateral pyramidal tract, after extensive lesions in the left cerebral hemi- sphere. There is slight degeneration in the left pyramidal tract and in the posterior columns. Fig. 16. Section of the spinal cord (somewliat distorted) in lower cervical region, showing bilateral descend- ing degeneration in both anterior median and lateral pyramidal tracts. This section demonstrates the unequal size of corresponding columns on the two sides of the spinal cord. When the anterior median column is large in extent the opposite pyramidal tract is correspondingly small. Integrity of the direct cerebellar columns is evident. Fig. 17. Section of the spinal cord at the third Imnbar segment, sliowiiig descending degeneration in botb lateral pyramidal tracts. A few librcs are dcgonfw.ted in 'be a;,iterior mc(?iau , columns. Atihislevc,! there are no direct cerebellar columns. 52 THE SPINAL COBV. absolutely paralyzed. All the muscles of the limb affected are about equally involved; the limb is stiff, its joints being moved with difficulty and slowly on account of the rigicl condition of the muscles. The muscles are thus in a state of slight tonic contraction and are hypersensitive to mechanical irritation, so that tapping a muscle produces a quick contraction, and tapping its tendon causes a prompt response. The muscles show no tendency to atrophy, though from disuse they may gradually become somewhat thin. They are never flabby; their nutrition is good; there is no change in their electric excitability. In this form of paralysis the circu- lation of the limb is sometimes impaired. There is slight blueness and coldness in the extremity and a tendency to oedema. Sensory disturbances do not necessarily attend this form of paralysis, and, if they are present, are an indication of disease elsewhere than in the motor tract. This type of paralysis is seen after all forms of cerebral disease, and then usually affects the arm and leg upon one side (hemiplegia). It is also seen in both lower limbs after a transverse lesion of the cord, such as may be produced by Pott's disease, by transverse myelitis, by soften- ing from thrombosis, by hemorrhage, or by tumors of the spinal cord. If this transverse lesion is in the cervical region, the arms are also in- volved. It is also seen in primary lateral sclerosis and in syphilitic paraplegia, in which conditions it is limited to the legs. The second or spinomuscular type of paralysis has an entirely dif- ferent set of characteristics. The muscles affected are usually entirely paralyzed at the outset, and if they recover at all, recover slowly and imperfectly. While all the muscles of the limb may be affected, as a rule they are not ; a few muscles here and there upon the limb being greatly paralyzed, while others retain their power ; or if all the mus- cles are paralyzed at first, some will recover to a greater extent than others. The limb is never stiff, but hangs helpless, yielding to the force of gravitation, its joints being relaxed and the articular surfaces no longer being held in close approximation by the tonic contraction of the muscles ; hence these joints are all more freely movable than in health. The muscles are relaxed and flabby. They do not respond to mechanical irritation by tapping with a hammer, and tapping their tendons does not cause contraction ; the so-called tendon reflex is lost. The muscles atrophy very soon after they are first affected, and this atrophy may go on rapidly until but little of the muscle is left. There is an early appearance of the reaction of degeneration in the muscle ; that is, it no longer responds when either faradic or galvanic excitation is passed through its nerve, and it no longer responds when faradism is applied directly to it. The circulation in the limb is always impaired in this form of paralysis. The vessels are relaxed, the blood pressure is decreased, there is a slow capillary circulation, the limb is blue and cold, and all the chemical processes seem to be delayed ; hence the surface temperature is much colder than in the first type of paralysis. Soon after the onset the limb is likely to be covered with ciammy per- PLATE IX XIII The Cervical and Sacral Enlargements of the Spinal Cord in Cross-section. (Starr.) Showing tlie various neurons in the gray matter, the direction of their axons, and the varieties of fibers in the different columns of the cord. Blue, motor neurons ; red, sensory- neurons ; purple, association neurons and axons. I. Ant. median column. II. Anterolateral column. III. Gowers' anterolateral ascending column. IV. Marginal column. V. Lateral pyramidal column. VI. Direct cerebellar column. VII. Lissauer's tract. VIII. Kxt. portion of column of Burdach. IX. Root zone of the column of Burdach. X. Descending comma-shaped bundle of Schultze. XI. Post, co-nmissural tract. XII. Column of GoU, XIII. ScijtomarKinal tract. TEF. DIJ GNOSIS OF SPINAL-COIiD DISEASES. 53 spiration, and later on, when this is no longer present, it is very diffi- cult to heat it up to the temperature of the other side. Sensory dis- turbances do not necessarily attend this form of paralysis. This type of paralysis is due to a lesion of motor cells in the anterior gray horns of the cord (Fig. 13, m) or to an affection of the nerve trunks containing the axones of these motor neurones. It occurs in infantile spinal paralysis or anterior poliomyelitis, acute or chronic ; in amyotrophic lateral sclerosis ; in myelitis, either localized in a few seg- ments or extensive throughout the cord ; in syringomyelia when the lesion invades the anterior horns ; in tumors and hemorrhages within the cord ; and in softening of the cord due to embolism or thrombosis. This type is also present in neuritis. The reason for these characteristics of this second type of paralysis will be better understood if the anatomical structure of the second part of the motor tract is considered. The motor neurones of the cord, as already stated, lie in the anterior part of the gray matter. These motor neurones are not scattered irregularly through its gray matter, Fia. 18. j:^ ■y^4f^'^ , I he groups of cells in the spiml cord at the fifth ccrvic il ■-e^iiK ut but are collected into groups, the larger number of groups lying in the cervical and lumbar enlargements. The number of these groups varies greatly in different segments of the cord, as is shown in Figs. 18 to 20, and the groups have a varying extent longitudinally, so that while some groups are limited to a single segment, others extend through 54 TEJS SPINAL COSD. several segments. Each group of neurones controls a single muscle or group of muscles which act simultaneously and in harmony. The fundamental movements of flexion and extension of the larger muscles of the limbs are represented in the large groups of the median, anterior, and lateral parts of the anterior horn. The accessory movements of the fingers and toes which are peculiar to monkeys and to man are represented in the smaller groups lying nearer the central portion of Fig. 19. The groups of cells in the anterior horn of the spinal cord at the seventh cervical segment. the gray matter. In Figs. 18 to 22 groups of cells at different levels of the cord are shown and it will be seen that they differ in their situ- ation and extent at different levels. A careful study of comparative anatomy and of the lesions occurring in anterior poliomyelitis, in which disease single groups of cells are affected, has enabled us to determine the exact level in the cord of the various groups of cells representing the various muscles of the body. These are shown in Table I., which gives each segment of the cervical, lumbar, and sacral regions, with a list of the muscles represented in each segment. It will be noticed that some muscles are represented in two or even three segments, while other muscles are represented in but one. It is evident, therefore, that if the lesion in the cord is limited to one segment, it will paralyze completely two or three muscles which are represented by motor neu- rones in that segment only, and it will paralyze partially other muscles which aTe represented not only in that segment, but also in adjacent segments. Hence the apparent irregular distribution and degree of the paralysis in various muscles upon the limb in cases of infantile paralysis. TTIT? DIAGNOSIS OF SPINAL-COBD DISEASES. 55 Table I. — Showing the Muscles Represented in Groups of Cells in the Various / Segments of the Spinal Cord. n., HL IV. V. VL VII. VIII. L Cervical. ' Cervical. Cervical. Cervical. Cervical. Cervical. Dorsal. Diaphragm. Diaphragm. Stern 0- Lev. ang. soap. mastoid. Rhomboid. Rhomboid. Trapezius. Supra- and Supra- and Scalenus. infraspin. Deltoid. Supin. long. Biceps. infraspin. Deltoid. Supin. long. Biceps. Supin. brev. Serratus mag. Pect. (clav. ). Teres minor. Biceps. Serratus mag. Pect. (clav.). Pronators. Triceps. Brach. ant. Long exten- sors of wrist. Pronators. Triceps. Brach. ant. Long exten- sors of wrist and fingers. Pect. (costal). Latis. dorsi. Teres major. Long flexors of wrist and fingers. Long flexors of wrist and fingers. Extensor of thumb. Intrinsic muscles of Extensor of thumb. Intrinsic muscles of hands. hands. I. Lumbar. II. Lumbar. III. Lumbar. IV. Lumbar. V. Lumbar. Quadr. iumb. Obliqui. Transversalis. Psoas. Psoas. Iliacus. Iliacus. Sartorius. Quad. ext. cruris. Quad. ext. cruris. Obturator. Adductores. Obturator. Adductores. Glutei. Glutei. Biceps femoris. Semi-tend. Popliteus. I. Sacral. II. Saci-al. III. Sacral. IV. and V. Sacral. Biceps femor. Semi-memb. Ext. long. dig. Gastroc. Tibialis post. Gastroc. Tibialis post. Tibialis auticus. Peronei. Intrinsic muscles of foot. Peronei. Intrinsic muscles of foot. Sphincter ani et vesicae. Perineal muscles. Certain special forms of the second type of paralysis are quite com- monly recognized. Thus we have in anterior poliomyelitis the upper- arm type of paralysis, in which the deltoid, biceps, supinator longus, and muscles about the shoulder-blade are affected together, the muscles moving the wrist and fingers escaping. Reference to the table will show that tliis form is due to a lesion in the upper part of the cervical enlargement. We also have a lower-arm type of paralysis, in which 56 THE SPINAL COED. the muscles wliicli move the fingers and wrist are alone invaded, the supinator lougus, which lies among them, escaping entirely. Refer- ence to tlie table will show that this is due to a lesion of the lower cer- vical enlargement. The same distinction can be made in paralysis of w Fig. 20. The groups of cells in the third liuubar segment of the spinal cord. the leg, there being a thigh type, a leg type, and a foot type, according as the lesion is in the upper lumbar, midlumbar, and sacral segments. By reference to the table, therefore, it will be possible in any case of paralysis of the second type to arrive at a localization of the lesion or at an estimate of its extent in the cord when the muscles paralyzed are exactly determined. The electrical examination will assist in this de- termination, inasmuch as the muscles whose groups of cells are de- stroyed will have lost their faradic excitability, while the muscles whose groups of cells are intact will show no change in electrical reactions. That the motor neurones of the cord have a distinct influence upon the nutrition of the muscle and upon the circulation in it, is shown by the rapid atrophy and the vasomotor disturbances in the muscle which also attend this type of paralysis, and to which allusion has already been made. It has already been stated that this type of paraijciis may be due THE DIAGNOSIS OF SPINAL-COED DISEASES. 57 Fig. 21. t: Sl^^ g f.i \m XII D Mi IV c Diagram of the lon'-"udinal appearance of tho cervical and lumbar enlargements of the spinal cord, showing the groups or c Jumns of cells ; made up from drawings of cross-sections of the cord in a series. The figures show tho segment. (Chr. Jacob, Das Menschonhirn, Fig. 4, p. 7.) 58 TEE SPINAL COED. to a lesion in the axones arising from the motor neurones of the cord. It is to be remembered, however, that after their exit from the cord these axones are divided up into nerves, being distributed in various directions through the branchial and lumbar and sacral plexuses. A diifereutial diagnosis can always be made between lesions of the spinal cord and lesions of the nerve trunks by the distribution of the par- alysis ; for the muscles which are paralyzed together from a lesion of a single nerve are different from the muscles paralyzed together from a lesion of the spinal cord. Thus the deltoid is often paralyzed from a lesion of the circumflex nerve alone, but is never paralyzed alone from a lesion of the cord. Thus the extensors of the wrist, together with the supinator longus, are paralyzed in lesions of the musculo- sjnral nerve ; but, as already stated, these muscles are never paralyzed together in small lesions of the spinal cord, their groups of cells lying far apart. And what is true of the nerves of the brachial plexus is also true of the nerves going to the lower extremity. Hence, while the characteristics of paralysis in lesions of the spinal cord and of the motor nerve trunks are the same, the association of paralyzed muscles with one another in the two conditions differs. A further point of differentiation is also found in the distribution of anaesthesia occurring with lesions of the nerve trunks, anaesthesia being frequently present in lesions of the nerve trunks and not necessarily present in lesions of the motor neurones of the cord. Furthermore, when it is present from a lesion of the cord, as will be shown later, the distribution of the anaesthesia in the skin differs entirely from the distribution of anaesthesia after a lesion of the nerve. It is thus possible, from a study of paralysis and of its essential characteristics and distribution in any case, to reach a diagnosis and to localize the lesion and to differentiate between lesions of the motor tracts of the cord and of the motor neurones of the cord and of the nerve trunks. There are cases, however, of general disease of the cord, such as a general myelitis, involving both the white tracts and the gray matter, in which a third type of paralysis is observed. This presents some of the characteristics of both the former types, and, were the diagnosis to rest upon the examination of the paralysis alone it might present certain difficulties. Fortunately, there are other symptoms always present to guide us in the diagnosis ; for in a general inflammation of the cord the changes in reflex action and in the control of the bladder and rectum, and the tendency to very severe trophic disturbances, are so marked as to leave no doubt regarding the actual condition. In this third type of spinal paralysis the paralysis resembles more closely the second type than it does the first, there being the same total par- alysis, the same atrophy, and the same reaction of degeneration in the paralyzed muscles ; but the distribution of the paralysis is more exten- sive — is, in fact, frequently total in both legs and almost complete in both arms, and, although there is no rigidity of the limbs, there is sometimes an increase in the reflex activity and in the mechanical ex- TEE DIAGNOSIS OF SPINAL-COUD DISEASES. 69 citability of the muscles in the early stage though later they are lost. This is particularly noticed in the disease amyotrophic lateral sclerosis in which both the lateral tracts and the anterior horns are simultane- ously progressively destroyed.^ In this disease the first type of par- alysis is present in the legs for several months, and the second type is present in the arms, associated with an increased mechanical excita- bility of the muscles, but finally this is lost, and the arms present the typical second type, which gradually and finally extends also to the legs. In cases of iujury of the spinal cord by fractures and dislocations of the vertebrae, the spinal cord may be seriously bruised or it may be absolutely disintegrated. The symptoms of paralysis appear to differ somewhat in these two conditions, as has been shown by Thorburn ^ and by Kocher.^ When the spinal cord is injured, but not destroyed, there is total paralysis below the level of the injury, with a condition of rigidity of the limbs and an increase of tendon reflexes. There is also a loss of control of the bladder, which may take the form either of retention of urine or of spontaneous evacuation of the bladder. There is likely to be some distention of the abdomen by gas, due to a paralysis of the intestinal wall. When the spinal cord is absolutely divided or destroyed at any level, there is total paralysis below this level, the limbs being completely re- laxed and not in a state of rigidity. The paralysis is symmetrical upon the two sides. The tendon reflexes are absolutely lost. There is always a retention of urine, which has to be relieved by catheter ; there is tympanites with distention of the abdomen ; there is a paral- ysis of the vasoconstrictors leading to a dilatation of the subcutaneous veins, and consequently to an increased temperature, and to priapism. There is an increase in the genital reflex, obtained by pinching the testicle. There is a loss of sensibility to pain and temperature, and usually to touch also, at a definite level of the surface, as shown in Plate VII. (See page 46.) In any case in which tliese characteristics are absent after an injury of the spine, it is certain that the cord has not been completely destroyed at the level of the lesion. Spasmodic Contractions of the Muscles of an involuntary kind are associated with paralysis ia many forms of spinal-cord disease. Like the paralysis, they can be divided into two categories. When the condition present is spastic paralysis and the muscles are rigid and re- flexes exaggerated, patients often notice a spontaneous trembling of the entire limb, due to alternate contraction of the two sets of opposing muscles. This is usually attended by an extensor spasm, so that the legs are stiffened and shake more or less violently. The condition is occasionally so extreme as to warrant the terra " spinal epilepsy," first ' The view of Lapinsky (Deut. Zeitschr. f. Nervenh., July, 1904), that each group of spinal cells repreHcnts a functional coordinated action and not a special muscle or group of muscles, is contradicted by the phenomena of infantile spinal paralysis. ^ A Contribution to the Surgery of the Spinal Cord, Philadelphia, 1893. ^"Die Verletzungen der Wirbelsaule, zugleich als Beitrag zur Physiologie des Menschlichen Ruckenmarl .^ fheLeinm-' 't) Lemiuxuj ]ied:iuu:L?iis Cerebri l.r/'/r'!SruJ mm - The LirnjuMUi^ Lemniscus Ports IMM *% W/,^ <^i I he Xem/usm > -r T'071S ' -Li'/nriiscus. fliKwulj^ The tract of muscular sense — the lemniscus. The Sensory Tract. — With these facts in mind it is possible to trace the sensory tract upward and to understand the symptoms which Avill present themselves when it is injured. It will then be clear that from LOCAL SYMPTOMS. 115 the distribution of the symptoms of antesthesia it is often possible to locate a lesion in the sensory tract. 1. The Tract of Muscular Sense. — The posterior columns of the cord end in the nuclei gracilis and cuneatus of the medulla (Fig. 54), each axone terminating in a brush about the neurone bodies which form these nuclei. From these neurones axones pass upward, crossing over in the sensory decussation to form the opposite interolivary tract which lies between the olives. The hypoglossal nerves on their way from the nucleus to their exit lie on the outer side of this tract. Ascending in this tract the sensory fibres enter the lemniscus, which lies in the pons Varolii behind the pyramidal tracts and deep transverse fibres. Many axones branch from the lemniscus to end about neurones lying in the gray matter of the pons, both in the floor of the ventricle, in the formatio reticularis, and in the deep gray matter. From these neu- rones axones again enter the lemniscus to ascend. The lemniscus ascends (Fig. 55) through the crus cerebri, where it curves about the lateral surface, sending many of its axones into the corpora quadrigemina posterior and anterior, then sinks into the base of the brain, and, pass- ing through the lower part of the internal capsule, terminates largely in the optic thalamus in its outer nucleus. (Fig. 53.) It is possible that all the lemniscus fibres end there. It is possible that some pass up through the capsule and turn outward to end in the cortex of the posterior central convolution and adjacent parietal region. It is pos- sible that the only sensory fibres entering the cortex come from the optic thalamus, but many authors have traced the lemniscus directly to the cortex, at least in part. In 1884 I showed,^ by an analysis of twenty-six cases of very small local lesions limited to the medulla and pons, "that an interruption in these fibres either (1) in the posterior columns of the cord ; (2) in the interolivary tract; (3) in the lemniscus, or (4) in the internal capsule where the lemniscus passes produces a condition of ataxia and loss of the muscular sense in the limbs of the opposite side. Since that date many similar cases of small lesions in the lemniscus have been reported with the same result. Hence it may be stated that this is the tract of muscular sense and that a lesion of this tract causes ataxia. If, therefore, hemiataxia, not of cortical origin, occurs, the conclu- sion can be drawn that the lemniscus is affected. The position of the lesion in the course of the lemniscus can be determined only by a study of other symptoms coincident with the ataxia. As the medulla, pons, and crura are very small and contain the nuclei of the various cranial nerves at different levels some symptoms referable to an affec- tion of one of these cranial nerves will usually enable one to locate the lesion. Thus, if there is oculomotor paralysis with hemiataxia the lesion is in the crus. If there is trigeminal anaesthesia or facial palsy with hemiataxia the lesion is in the pons. If there is auditory or hypoglossal paralysis the lesion is in the medulla. The figures indicate the course of this tract and its relation to the various cranial nerves. 2. The Tract of Tactile, Pain and Temperature Senses. — The ascend- ' Journal of Nervous and Mental Disease, July, 1884. 116 DIAGNOSIS AND LOCALIZATION OF BEAIN DISEASES. Fig. 56. £^) are stained black. In every bundle a number of such fibres are to be seen, g, artery ;y, fat. (Flatau, Spec. Pathol, u. Therap., Nothnagel, Bd. xi., Taf. iii.) INJURIES OF NERVES. 163 changes. The process of degeneration is com- plete in aboi^ two weeks after the injury. The cut end of the divided nerve becomes swollen into a bulbous extremity by a growth of connective tissue and by the development of fine nerve fibres in process of regeneration. This forms a very sensitive scar. A true union of the divided ends rarely occurs. All surgeons believe that function may be rap- idly resumed after suture of freshly divided nerves, and rapid restoration of sensation, ab- sence of wasting and retention of muscular con- tractility may be taken, according to Bowlby, as certain proof of restoration of continuity of a divided nerve. But experiments upon ani- mals do not confirm this view. It seems prob- able that under different circumstances different processes occur. It is undoubtedly true that in some cases the degenerative process, so graphically pictured by Ranvier goes on, from the beginning segmentation of the myelin down to the final result in the connective-tissue strand, the relic of the empty sheath of Schwann ; while, in other cases of a less serious nature, the de- struction is less complete and there remains a fibre consisting of a sheath of Schwann, con- taining a granular mass which may be either an axis cylinder or a mass capable of develop- ing into an axis cylinder under favorable cir- cumstances. If this is the case we can affirm that brilliant surgical successes, with rapid restoration of nerve function after suture, are possible when only partial degeneration is present, but are impossible when total and extensive destruction of the nerve fibre has occurred. The Process of Regeneration. — The proc- ess of regeneration begins about two weeks after an injury or experimental section. With regard to the method of this process two divergent views are held. Ranvier and his followers affirm that the new nerve is wholly a product of the central end of the injured nerve, grow- ing out from it and making its way along the track of the peripheral end, which takes no active part in the process. The central end of an individual nerve fibre becomes hy- pertrophied, and from this swollen part a sin- FlG. -sc/twz .-.>-vxa -M tr^s Regeneration. Longitu- dinal section of a nerve fibre from a case of lead-neuritis in a guinea pig. Biclschowsky stain, ax, axis cylinder ; ax, newly formed axis cylinder branch dividing into many new axones; r. sohw., nucleus of Schwann sheath ; ms, my- elin ; m.t, degenerated myelin. (Doinikow, Beitragezur histo- path. d. peri ph. nerven.,Nissl'S Arbciteu, IV., 4^15, 1911.) 164 INJUBIES OF NEBVES. NEUBITIS. gle new fibre starts out already medullated and grows onward into the old sheath, which it follows down until it reaches its end. By the aid of the nerve callus or cicatricial tissue, that usually joins the central end with the degenerated peripheral end, the new fibres are directed out- ward toward the periphery. And when they reach the peripheral end of the cut nerve they insinuate themselves into the old remaining sheaths of Schwann or between those sheaths, and grow on and out- ward until at last they reach the termination of the peripheral end, and the regeneration is complete. The terminal plates upon the muscles are renewed by a reproduction of protoplasm in the plate. The process thus described is in accordance with the process of original development of nerve fibres from nerve cells in embryonal life as has been recently proven by Harrison. ^ Neumann and Mayer, and more recently Ballance and Stewart, have affirmed, on the contrary, that the regeneration goes on in the peripheral end of the cut nerve, segment by segment being formed successively or simultaneously, the new nerve being built up by the union of each distal segment with the one lying centrally to it, until the process is complete. They hold that the process of regeneration begins in the granular mass left in the sheath of Schwann. Within it they have seen a narrow band of fine homo- geneous substance appear, which has the structure of a rudimentary axis cylinder. This does not fill the sheath of Schwann and is often pressed aside by the nuclei which lie in that sheath. It is not at first con- tinuous with the end of the old axis cylinder, remaining in the central part of the compressed or divided segment, but as it increases in definite structure it approaches this old axis cylinder, and finally unites with it. At the point of union a ring of Ranvier is formed. As this axis cylinder develops a substance is gradually formed around it, which is stained by osmic acid. This increases in thickness as the protoplasmic mass and the nuclei diminish, until it finally forms a new myelin sheath. The new myelin sheath is never continuous with the old one in the central end of the nerve, since it is sep- arated from that by the ring of Ranvier ; but often at first the old sheath seems to bulge out and encircle the new sheath, though this appearance is never permanent. At the point of union of the new fibre with the old one nuclei are often found, but these, like the others, gradually disappear. Lastly, a new sheath of Schwann is produced around the new myelin sheath and within the old sheath of Schwann. It presses aside the old sheath, together with such masses of protoplasm, drops of myelin and nuclei as may remain, leaving them thus wholly outside of the new-made fibre, so that they coalesce with and make part of the endoneurium. The new sheath of Schwann has but one nucleus in each segment and presents the nodes of Ranvier at regular intervals. Neumann has shown that this process goes on in every individual segment of the nerve sheath, so that in segment by segment, proceed- ing toward the periphery, the regenerative changes occur, and as each segment approaches completion it joins itself to the preceding one, ^ Johns Hopkins Hospital Eeports, January, 1906. INJUBIES OF NEBVES. 165 until finally the nerve is reestablished in its entire length. As the degenerative* process begins in the segment nearest to the point of compression, so does the process of regeneration, and in some nerves the two processes may be seen going on together, the segments near the seat of injury being renewed, while those at the periphery are still in a process of degeneration. The new fibres are at first somewhat smaller in calibre than the old ones, but they gradually attain a normal size, and then the process may be said to be completed. Ballance and Stewart afl&rm that the regeneration originates from the nuclei of the old sheath, or even from connective-tissue cells and neu- rilemma nuclei remaining in the connective-tissue strand. The most important evidence of such regeneration is offered by Bowlby.^ In three cases of division of nerves in which union was attempted by opera- tion some months after the injury, he found regenerating nerves in the peripheral portion. There were new fine fibres much smaller than natural, and in some the myelin sheath was scarcely perceptible. In some, however, the myelin sheath was fully developed, including the nodes of Ranvier. The origin of these new fibres was clearly from nuclei which seemed to be identical with the nuclei of the sheath of Schwann. These had arranged themselves in bundles with their long axis parallel to that of the nerve trunk. Then the nuclei had elon- gated and finally been transformed into fibres around which subsequently a myelin sheath was formed. A similar process has been fully de- scribed by Ballance and Stewart. It is evident, therefore, that the process of regeneration varies in dif- ferent conditions according to the exact stage of degeneration reached before it begins. If the final product of degeneration is a band of simple connective tissue, it seems probable that the nerve fibre will have to grow into it from a central origin, as in its original develop- ment in foetal life. If, however, the connective-tissue cells recently discovered are neuroplastic cells and have the power of producing new nerves, just as cells of periosteum may produce a new bone, and if, when degeneration ceases, there remains a sheath of Schwann contain- ing a granular protoplasmic mass, it is not at all improbable that that mass may be differentiated into an axis cylinder and a medullary sheath and joined to the old nerve fibre — - a process which has its analogy in the meduUation of nerves in the embryonal state. If we admit, with Wolberg, that in some cases the axis cylinder is not destroyed, the formation of new myelin is a rapid matter. That some such process as the one described by Bowlby and Stewart must occur in many cases is certain, when the rapid recovery after minor injuries is considered and when the results of nerve suture are taken into account, for in both these conditions the return of function occurs long before a new nerve fil)re starting out from the old one could have reached the periphery. It is affirmed by Mayer that individual nerve fibres in normal nerves are constantly undergoing these processes of degeneration and regenera- tion, either because the necessary renewal of worn-out tissue takes ^ Injuries of Nerves, page 25. 166 INJUBIES OF NEBrES. NEUBITIS. place in this manner, or beeause slight injuries from pressure or over- strain are sufficient to start up degeneration in single fibres. Such changes are more apparent in old age than in youth, and in cachectic conditions than in healthy states. In all persons dying of infectious diseases they can be found well marked. The process of degeneration in the nerves consequent upon the destruction of the ganglion cells from which they arise — the so-called Wallerian degeneration — which is best seen in cases of anterior polio- myelitis, diifers in no respect from that ensuing upon compression or division. NEURITIS. A nerve may be inflamed in a short portion of its course ^ — localized neuritis ; or it may be aflPected at many different parts — disseminated neuritis ; or it may be diseased in its entire length — general neuritis. The process may be described as ascending or descending, according to the direction in which the disease makes progress. Even when the lesion is a strictly local one, limited to a short portion of a nerve, extensive secondary changes occur from the part aifected outward ; and, as these may involve the entire length of the nerve, regeneration and repair may require a much longer time than is taken by the healing of the original lesion. General constitutional states may produce a simul- taneous neuritis in many nerves — multiple neuritis — and as this con- dition develops usually in the distal parts of the nerves, it is often termed peripheral neuritis. Neuritis may also occur secondarily to inflammatory changes in other parts, as with periostitis or abscesses. Syphilitic deposits in the nerves, tubercles in the nerves, cancer or other neoplasms along the nerves may cause a proliferation of the con- nective-tissue elements or a true diffuse inflammation. After wounds and injuries of the nerves a condition has occasionally been seen which is known as ascending neuritis or migratory neuritis. Tender spots along the course of the nerve above the point of injury and pain in the course of the nerve as high as its root in the plexus have been observed in a few cases. This has been chiefly in patients in whom there has been an open wound at the point of primary injury and where there has been a suspicion of an ascending septic process in the nerve. The neuritis, however, is not always a continuous one from the point of injury upward, but in a few cases tender spots have been found at some distance above, without any change in the intermediate space. Hence the term migratory neuritis. The migratory form is supposed to be characteristic of septic infection, the sepsis extending along the connective-tissue sheaths of the nerve. When there is no open wound to account for this sepsis, a septic condition of internal origin, associated with obliteration or plugging of the bloodvessels and with the production of a gangrenous area, has been recorded as a cause. While cases of ascending neuritis in the continuity of the nerve have been produced experimentally, it has not been possible to produce migratory neuritis when the wound has been kept aseptic. In some NEUBITIS. 167 cases of traumatic neuritis a very extreme condition of tenderness of the limb aboye the point of injury, together with inability to move the joints and a general hypersensitive state with pain in the joints, occa- sionally develops. This condition, however, must be considered as probably hysterical, and usually develops only in hypersensitive or neurasthenic individuals. It is not necessarily attended with a distinct localizable tenderness along the course of the nerve, though this may also be present. The number of cases of ascending neuritis recorded in the literature of the past few years is very few. I have never seen a case of true ascending neuritis, though I have seen many cases of painful aifection of the extremities above the level of the neuritis that were hysterical in their nature. There are some cases which are not hysterical. In these the explanation of the condition is found in the fact, well known to psychologists, that mild irritation long continued produces in a nerve centre a hypersensitive state by what is known as a summation of impulses. In this state slight impressions are believed to be intense and local impressions become generalized and widely referred. This state may be induced by neuritis long continued and has been mistaken for an ascending neuritis. Pathology. — A nerve which is inflamed is red and swollen, is lack- ing in its natural surface lustre, and is no longer firm and smooth to the touch. Its vessels are congested and there may be hemorrhages within its sheath. If the process has been in progress for some time there may be bulbous swellings on the nerve the result of connective- tissue infiltration, or the nerve may be markedly atrophied. Such bulbous thickenings are very common after injuries and always occur after a division of a nerve on the central end. A section of the nerve viewed by the microscope shows a distention of the vessels, an infiltra- tion of endoneurium with small cells, and a thickening of the connec- tive-tissue elements. The microscopic changes are similar to those produced by injuries of the nerves. Yet it is possible to distinguish between cases in which the lesions are chiefly limited to the ax one and medullary sheath — the parenchymatous form of neuritis, and cases in which the lesion affects more especially the connective tissue about the fine fibres and the endo- neurium and perineurium — the interstitial form of neuritis. In the latter the bloodvessels and lymphatics take a more active part in the inflammatory changes. In parenchymatous neuritis at the outset the myelin sheath appears slightly swollen, is less homogeneous, and, from a difference of refrac- tive power, is less translucent. It then becomes split up into segments of different length and form, the segmentation occurring preferably at the incisures of Schmitt, while the incisures at other parts disappear. Between these segments of myelin a finely granular protoplasm is seen in which new nuclei are found. In some fibres the axis cylinder may still be preserved. In others it is broken at the same places as the myelin. At the next stage of the process the changes are more marked. The myelin is now reduced to a series of small globules surrounded every- 168 INJUBIES OF NEBVES. NEUBITIS. where by granular jirotoplasm, and in this protoplasm the nuclei are now very numerous. The axone cannot be distinguished in the mass, as a rule, but occasionally a fine line is seen passing through the mass, which may be a remaining axone. (See Fig. 86.) The succeeding stage presents a different picture. While up to this time the size of the nerve fibre has remained about normal and uni- form, it is now seen to vary. At places the fibre is still wide and filled with a granular mass ; at other places it is narrow, the mass having disappeared, leaving either a collapsed sheath or a sheath con- taining only nuclei here and there. In a few such narrow fibres there seems to be an axone lying directly within the sheath of Schwann, and occasionally separated from it at various places by nuclei ; but, as a rule, no trace of the axone remains. As any single fibre may show constrictions at some places, dilatations at others, the variation in its calibre is the most striking feature of this stage. In the terminal stage the calibre is uniform again, but is now everywhere reduced. The sheath of Schwann is empty or contains only a little granular substance and the nuclei are now less numerous than before. There is, in fact, only an atrophied tube with none of its original contents. These tubes lying side by side are folded and undulating, and appear like a strand of connective tissue. These various stages of parenchymatous inflammation are to be seen in different fibres in the same specimen. They are present not only at the seat of inflammation, but they are present from this point onward to the end of the nerve, constituting the change known as secondary degeneration. Their appearance is identical with that observed in the course of degeneration of a nerve after compression or after destruc- tion of the neurone body in the spinal cord, as in anterior poliomye- litis. This had led such an accurate observer as Erb ^ to advance the hypothesis that in cases of multiple neuritis due to toxic agents some slight changes in cells in the spinal cord, not visible to the microscope, are present primarily, and that the changes in the nerves are secondary, the nutrient power of the cell being incapable of supporting the entire axone, which thus shows changes in its most distal part. It has lately been shown by means of the Nissl stain that after any nerve lesion in the periphery a change occurs in the entire length of the axone and also in the central cell body, of which that nerve is the axone. (See Plate II., G.) This change is a degeneration ; but at the end of a month it becomes stationary, and then gradually the cell body regains its original appearance, even though the axone may remain degener- ated. Such cell change, therefore, is not necessarily the primary con- dition in neuritis. Striimpell urges that parenchymatous neuritis has its parallel in other parenchymatous inflammations, and therefore does not need to be traced to any primary affection in the cells ; and hence Erb's hypothesis has not met with general acceptation. Interstitial neuritis presents a diflPerent appearance. In this condi- tion inspection shows the nerve to be congested, swollen, thicker than ^Neurol. Centralbl., 1883, p. 481. NEURITIS. 169 normal, and lacking in lustre, or to be yellow and irregularly swollen by the accupaulation of pus and serum, or to be reduced to a mere con- nective-tissue strand. Upon teasing the nerve it is at once evident from its brittleness, that individual fibres are lacking in continuity and are changed in structure ; and if it is examined under the microscope the exudation of serum and of inflammatory corpuscles, the great in- crease in the number of connective-tissue nuclei, the distended condi- tion of the vessels, as well as the various appearances characteristic of nerve degeneration, are clearly seen. The thickening and hyperplasia of endoneurium and perineurimn are well marked in the older cases. Here the inflammation is originally an interstitial inflammation, though later it becomes a diffuse one. It is possible that the degenerative processes in the nerves may have been due to the compression by the products of inflammation exuded within the nerve siieath. In one or two cases where the patient died early in the disease the nerve fibres which lay near the vessels were affected to a greater degree than those lying deeper, and from this fact it was concluded that their degenera- tion was secondary. In other cases, however, all the fibres in a bundle were equally involved. It is probable that in some cases the inflam- mation is diffuse from the outset, parenchyma and interstitial tissue being affected simultaneously. One marked feature in these cases is the large amount of fatty deposit found in the altered nerves, as shown by the Marchi stain. This is to be ascribed to the fact that myelin in undergoing degeneration breaks down into globules and undergoes a fatty change. It is itself allied to fat, and making up, as it does, a large part of the bulk of the fibre, it would be noticeable in the product of degeneration if it were not absorbed. Sometimes the absorption seems to be interfered with by the vascular condition, and hence the residual amount of fat is increased. (See Fig. 85.) One additional pathological form must be mentioned, since it has been described by such a careful observer as Gombault.^ It is the so- called segmental periaxillary neuritis. In toxic neuritis from lead and alcohol poisoning, Gombault found that the degenerative process was not uniform in the entire length of the nerve fibre. On the contrary, entirely normal segments alternated with the degenerated segments in the nerve. Mayer has noticed a somewhat similar condition, and it has been described as occurring in senility. The changes already de- scribed take place in the myelin sheath of the affected segment, even to its entire absorption, leaving the axis cylinder in the sheath of Schwann ; but, as the adjacent segments are not involved, regeneration is thought to be more easily accomplished. Pitres and Vaillard ^ have noticed a somewhat similar condition in the neuritis occurring after diphtheria, although in their case the axis cylinder as well as the myelin sheath was totally destroyed in many segments. Occasionally simple atrophy of nerve fibre has been observed ; a mere gradual reduction of all the elements without any degenerative process. ' Archives de Phys., 1873, p. 692 ; also Arch, de Neurol., I., 1. ^De la n^vrite segmentaire, Arch, do Neurol., xi., 337. 170 INJUEIES OF NEBVES. NEVEITIS. While it is, of course, impossible to describe definitely the process of regeneration that goes on after neuritis, there is no reason to sup- pose that it differs in any way from that observed in experimental lesions. The time required for the completion of the process will de- pend upon the severity and extent of the degeneration. When that is slight the recovery may be rapid, cases having been reported where a total restoration of function took place in two months. As a rule, however, it is a slow process. The large majority of the cases require over six months for the complete regeneration, and in not a few cases from ten to sixteen months elapse before the condition of the nerves is proved to be normal by the total disappearance of all symptoms. Symptoms. — The symptoms of neuritis or of injuries of the nerves have been described on pages 39 to 46. The special symptoms de- pending on the nerve which is affected are considered separately in the next chapter. Prognosis. — The prognosis in neuritis, as a rule, is a good one. We have seen that there is a spontaneous tendency to regeneration in a nerve that is injured or that has been affected by inflammation, and while this progress toward recovery is usually slow, yet eventually it becomes complete and all the functions of the nerve are restored. The only factor in preventing a recovery is the impossibility of a union be- tween the severed ends of the nerve, or the interposition of callus, or the development of a connective-tissue scar which prevents a reestab- lishmeut of continuity ; but, after such obstructions are remedied by surgical treatment, regeneration takes place, even though the obstruc- tion may have persisted for many months. Therefore, the eventual prognosis of recovery in neuritis is good. Spontaneous recovery, un- less the nerve is put in a normal condition by being freed from scar tissue, may not occur. I have seen a man, aged fifty-two years, who had suffered since the age of six years from paralysis and atrophy of the muscles controlled by the ulnar nerve. At that time his elbow was fractured and dislocated and the nerve displaced, so that it passed over the olecranon, where it could be felt. Until the age of fifty years he suffered from partial paralysis and ansesthesia, and then, from un- known cause, his symptoms, pain, numbness, and atrophy increased rapidly, and he suffered much. Complete recovery of power and sen- sation followed an operation that repaired the nerve, though so many years had elapsed since the injury. Bruns ^ has recently called attention to the ultimate results in injuries of the nerves and of the plexuses, and has shown that while two-thirds of his cases of nerve injury recovered, only about one-quarter of the cases of injuries of the plexuses were cured. ^ Treatment. — In the treatment of injuries and wounds of nerves or in spontaneous neuritis the first and most important object is to secure the possibility of regeneration by establishing the continuity of the in- jured nerve. If the injury is from a stab wound it is important that 'L. Bruns. Keurol. Cent., November, 1902. "Kennedy. Brit. Med. Joui-., February, 1903. NEURITIS. 171 the severed ends of the nerve should be brought together and carefully united, or if laceration ^has taken place, so that there is a loss of con- tinuity, a flap can be made from both ends and these elongated nerves then united ; or it is possible to insert the ends within a tube of decal- cified bone to serve to direct the regenerating fibres outward toward the distal end. Even those who teach that regeneration occurs in the severed end admit that it is only rapid and perfect when such union is established. If the nerve has been lacerated by a fracture of bone or by a strain it is usually sufficient to set the fracture or to place the strained part in splints, thus securing its immobility for a time and allowing nature to effect the proper repair. Occasionally, however, when the fracture has united, or when the local swelling and pain of the strain have gone down and the bony thickening or the induration about the strained part has disappeared, the nerve will still be found to have lost its function. If after six weeks' treatment by electricity and massage it is still evident that there is no progress toward recovery, it is advisable to expose the point at which the nerve was injured. It is sometimes found that regeneration has been prevented by the growth of a connective-tissue mass, and occasionally it is found that a mass of bony callus has formed about the nerve in such a way as to keep up pressure. Thus, in a case of ulnar neuritis under my observation, pro- duced by fracture at the elbow, the repair of the fracture and the restoration of motion in the joint were not attended by a recovery from the neuritis. It was evident, by palpation, that a bony callus still existed about the nerve at the elbow, and surgical exploration showed the nerve to be completely embedded in this mass. When this was chiselled away and a groove formed in which the nerve could lie freely, and the two ends were approximated by a flap, regeneration and re- covery ensued. In a case of an infant in whom brachial neuritis from pressure above the clavicle during delivery had occurred, and a permanent paralysis of the arm had remained for seven years, surgical exploration discovered a mass of scar tissue lying upon the nerve trunk and about twice its diameter, which had prevented regeneration, and when this was removed and the ends of the nerve approximated re- covery ensued. It is evident, therefore, that the first requisite in the treatment of injuries of nerves is to restore, if possible, the original position of the nerve, so that nature may produce regeneration. In cases of spontaneous neuritis from cold, etc., the only thing nec- essary is to maintain the parts in a perfectly quiet position, and, if possible, to reduce any congestion in the inflamed nerve. This may be done by the use of counter-irritants, of which the actual cautery is probably the best. Light touching with a Paquelin cautery along the course of the inflamed nerve will often give relief to intense pain and will reduce the congestion which attends spontaneous neuritis. Small mustard plasters are also of service. liocal applications of heat are often very grateful in painful condi- tions of neuritis ; in fac!t are much more agrceal)le to the patient than cold, ]icn(;e poultices or packing the affected liml> in cotton-wool cov- 172 1NJVMIE8 OF NE:KVES. NEVmTIS. ered with oiled silk may be of service. In the more severe cases, where pain is frequently intense and keeps the patient awake at night, it is necessary to use medicines. The newer analgesics seem to have the power of diminishing painful sensations in peripheral nerves, and hence pheuacetin, 5 grains; antipyrine, 10 grains; acetanilid, 3 grains; exalgin, 3 grains, or salophen, 10 grains, may be used every two or three hours. It is sometimes possible to obtain a better action by a combination of these, to which a small amount of codeine may be added if the pain is very persistent. The following formula is a favorite and very efficacious : -Phenacetin gr. vj Acetanilid gr. iij Codeine gr. i- Caffeine gr. ij- Sacch. lactis gr. V. Triturate. S ig. — One s uch p owder every three hours. M. Codeine is a useful remedy, but in many cases it is necessary to re- sort to hypodermic injections of morphine. These may be used freely and with less danger of producing a habit in neuritis than in any other affection, inasmuch as the gradual recovery will insure a diminution of the pain and the eventual cessation of the use of the drug. The tenderness of the nerve to any manipulation and the production of pain by any movement are sufficient to induce the patient to keep the part in perfect rest, which is essential to recovery. The anjesthesia in the skin within the domain of the affected nerve can be speedily re- duced and in many cases entirely removed by the application of the faradic brush. One pole of the battery should be placed high up upon the limb that is affected, or upon the trunk, and the brush should be applied in the anaesthetic area. The strokes should be from the adjacent sensitive skin into the ansesthetic region, and the strength of the battery should be moderate, yet just sufficient to produce a dis- tinct sensation of the electric current. In this way it seems as if it were possible to produce an extension of sensibility from the sensitive area into the adjacent insensitive region, and thus to open up anasto- motic paths through the skin. The paralysis following a nerve injury or neuritis requires electrical treatment. In the majority of such cases the faradic contractility in the muscle is lost and it is useless to apply faradism. The galvanic contractility, however, is always preserved, even though it may be diminished. For, by a strong galvanic current, even in the worst cases, a fair contraction can be produced in the paralyzed muscle when the current applied to the muscle is interrupted by means of the inter- ruj)ting electrode held in the hand. The pole to be used over the muscle should be that to which the muscle responds most quickly. If a complete reaction of degeneration is present the positive closure contractility will be greater, and then the positive pole should be placed upon the muscle. If a partial reaction of degeneration is pres- ent the negative closure contractility will be greater, and then the NEUBITIS. . 173 negative pole should be used. The other pole is to be applied to the limb higher up in the jcourse of the nerve. The application should be made daily and should last about five minutes to each muscle. The object of such applications is to exercise the paralyzed muscle. It is not probable that the effect of the electricity is in any way to increase the repair of the nerve or to aid its regeneration, hence there is no object in giving a continuous current to the nerve, as some authors advise. The electrodes should be of sponge and covered with clean gauze, and wet with warm salt water. The paralysis may be also aided by massage and careful manipula- tion of the joint which the paralyzed muscles should move. In cases of spontaneous neuritis where the nerve is very tender, massage may be so painful as to be injurious, and it should always be remembered that massage, to be useful, should be painless. I have seen severe injuries of nerves follow the painful manipulation of unintelligent masseurs and osteopaths, and the statement which such individuals often make, that if their manipulations cause pain they are thereby doing good, is absolutely false. The object of massage in paralysis is to increase the nutrition and circulation in the paralyzed muscles. It should be done gently for about one-half hour daily, and should be followed by a sense of warmth and comfort, and not by fatigue. A secondary result of the paralysis of certain muscles is the contrac- ture of their healthy opponents, producing forced positions in a joint and very often quite serious deformities. The joint may even become stiff or the bones may become displaced,. Thus in wrist-drop which follows musculospiral palsy the intrinsic bones of the hand are not uncommonly displaced, so that a distinct protuberance upon the back of the hand becomes visible. Manipulations of such displaced or stiff- ened joints are very valuable, and should be urged from the very beginning, in order to prevent deformity ©r stiffness. The trophic symptoms which occur in neuritis usually yield at once to a careful disinfection of the skin and protection of the skin from extraneous injury or infection. This can be attained by washing the parts in a solution of bichloride or of carbolic acid, applying carbolized vaseline to the surface, and wrapping in absorbent cotton. It is very important to prevent the occurrence of these trophic symptoms by proper care of the skin from the very beginning, and patients suffering from neuritis or having any ansesthetic regions should be warned against the possibility of unnoticed injuries or burns. CHAPTER VII. INJUEIES OF THE SPINAL NERVES AND SPECIAL FOEMS OF NEURITIS. The affections of the cranial nerves will be considered in connection with diseases of the brain ; see Chapter XXXV. There are thirty-one pairs of spinal nerves which are named after the segments of the spinal cord from which they arise, there being eight cervical, twelve dorsal, five lumbar, five sacral, and one coccygeal. The origin, course, and branching of these nerves are shown in Plates XIII. and XV. It is not needful to describe the anatomy of these nerves as their course and destination is a matter of general anatomical knowledge and is shown in the plates, but it is necessary to consider certain special types of neuritis and the results of injuries to certain nerves which are frequently met with in practice. CERVICO-BRACHIAL NEURITIS. The anatomy of the cervico-brachial plexus is shown in Plate XIII. Etiology. — A neuritis of the cervico-brachial plexus may occur in the adult as the result of injuries to the neck and shoulder, especially after dislocations, as the result of spinal caries, or of tumors, or aneu- risms growing in the neck ; as the result of long-continued pressure of the plexus upon an abnormal cervical rib, as in cases described by Keen,^ where full references to -this subject are given ; as a sequel of any of the infectious diseases, especially grippe, or of toxic conditions ; from rheumatic and gouty states, and also from taking cold. It is more frequently met with in women, and persons above the age of forty years are more liable than young persons. Symptoms. — If the neuritis is limited to the upper four cervical nerves a very intense occipital neuralgia is produced. The pain runs up the back of the neck and over the head as high as the vertex, and is attended by extreme sensitiveness of the great occipital nerve to pressure ; by tingling and numbness, and by difficulty in the movement of the head because of the pain produced by motion. The head is often held stiffly on this account. Occasionally the hair of the scalp comes out. There is sometimes a weakness or paralysis of the deep cervical muscles. If the neuritis is limited to the fifth and sixth cervical nerves or their branches in the plexus, pain is very intense in the neck above the clavicle, and is felt over the shoulder and in the axilla and down the back of the arm, and even into the forearm, and the paralysis affects the deltoid, biceps, coracobrachialis and supinator muscles, iyV, W, Keen, Amer. Jour. Med. Sci,, Feb., 1907, 174 Neclc muscles CEEVICO-BBACHIAL NEUEITIS. 175 If the lower cervical nerves and the first dorsal nerve are the seat of the neuritis the pain ^nd tenderness are very intense over the clav- icle, and the pain is felt down the front of the arm over the entire forearm and in the hand and fingers, and the paralysis aifects the muscles of the forearm and hand. It is to be remembered that the spinal origin of the sympathetic nerve in the neck is in the last cervical and first dorsal segments of the spinal cord, and the anterior nerve roots coming from these segments contain the fibres passing to the cervical sympathetic. These nerve fibres leave the main cervical nerves soon after their exit from the vertebral foramina and ascend, lying deeply beneath the muscles. In the majority of cases of neuritis of the lower part of the brachial plexus they are not involved, but occasionally, if the inflam- mation extends deeply through the spinal nerve roots, or if these nerve roots are compressed by tumor, or are involved in caries of the spine, or are lacerated within the spinal canal, the symptoms of paralysis of the sympathetic appear. These symptoms are a slight retraction of the eyeball and narrowing of the palpebral fissure, the eyelids appearing partly closed and the outer angle of the eye being slightly drooping. The pupil is somewhat contracted and does not dilate fully when the eye is shaded. There is a slight pallor of the side of the face and neck, an unusual dryness of the nostril and mouth on the side of the injury, and a diminution in the secretion of sweat on the neck, arm, and chest of the injured side. If the patient is subjected to heat the injured side does not flush and perspire in the normal manner. The existence of these symptoms of injury of the sympathetic, to which attention was first directed by Klumpke, is sometimes of much importance in diagnosis, as an indication that an injury of the plexus is present. Thus in a patient seen with Hartley at the New York Hospital, who had fallen several stories and sustained a fracture of the right side of the cranium and a manifest strain of the left arm, it was important to determine whether the paralysis of the left arm was due to a laceration of the brachial plexus or to an injury of the brain. The existence of great pain on pressure over the brachial plexus and on motion of the arm, with almost total anaesthesia of the hand and arm below the elbow, and the presence of the symptoms indicative of injury of the sympathetic nerve in the neck, made it evident that the paralysis of the arm was due to a laceration of the nerves of the brachial plexus and not to a cerebral injury. The development of the reaction of degeneration in the muscles in the course of ten days con- firmed this diagnosis. Usually in brachial neuritis several cords of the plexus are involved simultaneously, and then the pain radiates over all the branches of the nerves which arise below this plexus. Plate XIII. shows the formation of this plexus from the nerve roots and the distribution of the cords of the plexus to the various nerves of the arm. Plate XIV. demonstrates the distribution of the cutaneous nerves in the skin of the arm. A comparison of the symptoms developing in any case, namely, thQ 176 INJUBIES OF SPINAL NEBVES AND NEUBITIS. distribution of the pain, of the lines of tenderness, of the areas of ting- ling, numbness, and anaesthesia, with these diagrams will serve to indicate what branches of the brachial plexus are involved in the neu- ritis. As cases differ widely from one another in the extent of impli- cation of the nerves^ it is only by such a comparison of each case with a diagram that a definite diagnosis can be reached. Severe injuries of the brachial plexus may result in a tearing of the nerve roots within the spinal canal, as well as in a direct injury to the plexus itself. Such lacerations of the nerve roots produce symptoms which are difficult to differentiate from the symptoms caused by an injury of the cords of the plexus. The diagnosis can sometimes be reached, however, if all the muscles that are paralyzed be listed, and their nervous supply be exactly determined by a comparison with a diagram such as is shown in Plate XIII. In a case recently under my observation, a comparison of the muscles paralyzed, with this diagram, made it evident that the fifth and sixth nerve roots were implicated rather than the upper cord of the brachial plexus, the long posterior thoracic nerve to the serratus magnus muscle being involved, which would have escaped had the cord alone been injured. In another case, the seventh, eighth and first dorsal nerve roots were implicated rather than the lower cords of the brachial plexus. In both these cases the diagnosis was confirmed at the operation, which was undertaken to explore and repair the plexus in case that was found to be injured. In these cases the distribution of anaesthesia in the skin corresponded to the anaesthesia which would have been produced by a lesion of the segments of the cord, and not to the distribution that would have been produced by a lesion of either the individual nerves making up the plexus, or of the cords of the plexus. In cases where the roots of the nerves are torn, there appears to be no possibility of repair, as a union is not possible between the distal end, outside of the vertebra, and the central, inside of the vertebra. These cases, therefore, have a bad prognosis.^ Brachial neuritis of the ordinary form in the adult, due to exposure to cold, is an extremely common disease, almost as common as sciatica. It is an exceedingly painful affection, coming on sometimes with great suddenness, as in a case described by Klumpke ^ where the autopsy showed an extensive hemorrhage in the sheath of the nerves. Hence it has been called " apoplectic neuritis." Its onset may be gradual through several days, under which circumstances an extreme conges- tion with interstitial neuritis and secondary implications of the nerve fibres are present. A pain in the location of the plexus and pain radi- ating outward are the most distressing symptoms, and are so intense and agonizing as to deprive the patient not only of all use of the limb, but also of sleep. Soon after the onset of the pain any motion be- comes so uncomfortable as to make rest of the limb imperative. The numbness and tingling which attend the pain are extremely disagree- able and are more intense after the disease is well established at the 1 Frailer. Jour. Amen Med. Assoc, Dec. 16, 1911. 2 Rev, de M^d., 1885. PLATE XIV ,4roc'i[ect>- Superficial Perineal of\ Piidic and Inferior I Pudendal of small I Sciatic The Distribution of Sensory Nerves in the Skin. (After Flower.) The areas of the skin supplied by the cutaneous nerves are shown in finely dotted outline. The circles on the trunk show areas occasionally ansesthetic in hysteria. The lines across the limbs at ankle, knee and thigh, wrist, elbow and shoulder show the upper limits of anaesthesia in multiple neuritis of varying degrees of severity. CEBVICO-BBACHIAL NEUBITIS. 177 end of a week. The paralysis of the muscles supplied by the affected nerves develops within three or four days of the onset, and these muscles becdme atrophied as the case goes on, and usually after ten days show a reaction of degeneration. In brachial neuritis trophic disturbances of the skin, glossy skin, herpes, eruptions of various kinds, and interference with the growth of the nails are very common occurrences. The skin is manifestly thin, becomes very shiny and smooth, appears as if tightly stretched over the fingers, hand, or arm, and is exceedingly tender and hypersensitive to touch, to heat, and to cold. Sensations caused by moving the fine hair on the skin are peculiarly disagreeable. The limb is very frequently covered with perspiration, which may be of a fetid odor. Such a neuritis usually persists for several weeks or even months. The average duration in ten such cases under my observation was four months. The symptoms gradually subside, but the weakness often persists for some weeks after the pain has disappeared. In cases where the presence of a cervical rib is suspected an aj-ray will demonstrate its existence. Pathology. — Pathological observations in cases of neuritis of the brachial plexus are comparatively rare, as the patients usually recover. Klumpke's case of hemorrhage within the sheath of the plexus has already been mentioned. Jacobson reports a case following carcinoma of the breast of nine months' duration. A microscopic examination showed an almost total disappearance of the nerves of the brachial plexus, though there was no infiltration with carcinomatous masses. The process of degeneration was present in many bundles, with a manifest disappearance of nerve fibres, and an atrophic condition was found in the peripheral nerves of the arm. The cells of the posterior spinal ganglia in the cervical region showed degenerative changes by the Nissl method of staining, and by the Marchi method there was discovered a manifest degenerative change in the posterior root zone of the spinal cord corresponding to the entrance of the nerve roots from which the brachial plexus was made up. Degeneration ascended in the column of Burdach, and also descended in the comma-shaped bundle of Schultze as far down as the fifth dorsal segment. This observation proves that in some cases when the sensory neurone is involved in one of its branches the cell body and the other branch may also degenerate. Prognosis. — The prognosis is fairly good, as recovery eventually occurs in the majority of the cases. But the progress is slow and is at- tended by very great discomfort. It is to be remembered that nerves in regenerating grow but one centimetre a week, and hence the repair in the affected nerves requires a long time, especially if those nerves are the long ones to the fingers. In the traumatic cases the prognosis is less favorable than in injuries of single nerves. Thus in a case seen with McBurney, a calcareous cicatrix had involved the three cords of the plexus and destroyed the nerves so completely that nothing could be done to induce repair. Treatment. — Treatment consists of absolute rest^ the arm being 12 178 INJURIES OF SPINAL NEBVES AND NEUBITIS. carried in a sling and held to the side by bandages, constant applica- tion of heat, either by packing the shoulder and arm with cotton, which is to be covered with oiled silk, hot bottles being applied outside of the bandage, or by the application of poultices. In my experience cold applications — ice packs, or spraying with ether, or chloride of ethyl, which are recommended by the Germans — produce great discomfort and are voluntarily discarded by the patients in favor of hot applica- tions. The use of the actual cautery in producing counter-irritation over the painful nerve trunks is urged by all authorities, and I have certainly known it to alleviate the pain. It may be done daily if the touch made is very light, so as not to break the skin, merely produc- ing a red line upon it. Electricity may be used, and in some patients appears to give considerable relief. A very mild galvanic current, from four to six milliamp&res only, should be applied with the positive pole over the painful region, and the negative pole over the back of the neck. The current should not be broken and should be allowed to pass for ten minutes through the painful region, the pole being shifted in order to bring all the painful parts within the influence of the current. In applying the electricity to the arm, Remak recom- mends that a current be used diagonally through the painful nerve, the positive pole being over the most painful point and the negative upon the other side of the limb. During the early and painful stage of the disease any electrical application producing a contraction of the muscles is too painful to be endured, but later in the course of the case, when the pain is subsiding, it is well to exercise the muscles by electricity in order to keep up their tone until the nerve has regener- ated. The application of electricity has no effect upon the course of the case or upon the regeneration of the nerve trunk. Massage of the limb is advisable when the parts are not too tender to prevent its use, and as soon as the tenderness subsides sufficiently to allow of it, it should be begun and continued until recovery. Some relief is often obtained by douching the limb with hot water from a spray or by alternate douches of hot and cold water. Patients differ in their susceptibility to cold in this condition. Many patients are hypersensitive to cold in conditions of neuritis, and under these circum- stances cold water should be avoided. The pain in brachial neuritis is so intense as to require medical relief. The various analgesic preparations — acetanilid, antipyrine, phenacetin, alone or combined — should be tried before codeine or opium are resorted to. But in the majority of cases in this condition it is necessary to use hypodermic injections of morphine, and these may be used very freely in sufficient amount and with sufficient fre- quency to secure relief from the intense agony which otherwise exhausts the patient. Care should be taken to keep up the general nutrition of the patient during the course of the disease. Good food, adjuvants to digestion and laxatives which counteract the action of the morphine should be used freely, and it is well to insist upon the use of fatty foods in excess, as these appear to have a beneficial effect in increasing OBSTETEICAL PABALTSIS. 179 the rate of regeneration in the nerves. Alcoholic drinks should be avoided or^sed in great moderation. Strychnine and arsenic are of benefit in the stage of recovery, but are not to be used while the pain is intense. The glycero-phosphates of lime and soda in 1 5-grain doses, three times a day, are of much use. Surgical treatment is demanded in traumatic cases which show no tendency to recover after three months. The plexus should be exposed, cicatricial tissue removed, and a union of the separated nerves secured by a nerve flap, or by inserting the ends in a tube of decalcified bone. OBSTETRICAL PARALYSIS, OR BIRTH PALSY OF DUCHENNE. This form of paralysis is noticed within a few hours or days of the birth of an infant. It is usually limited to the deltoid, biceps, brachi- alis anticus, supraspinatus and infraspinatus, and supinator longus muscles, but occasionally involves the extensor muscles of the wrist and fingers. The arm hangs loose at the side of the body, its position as a whole being governed by gravitation, with the forearm extended and pronated, and the wrist and fingers flexed. (See Fig. 89.) If the arm be lifted and then let go, it falls into this position. The muscles which are paralyzed are relaxed and their opponents are not rigid, so that all joints are freely movable and motion does not give the child pain. The only movements in the paralyzed arm are those of extension of the forearm and motions of the wrist and fingers. Sometimes when the extensors of the wrist are involved there is slight extension of the two distal phalanges only. If the paralyzed muscles are examined electrically the reaction of degeneration can be demonstrated, but this is often difficult in. an infant on account of the undeveloped condition of the muscles and the thick layers of fat. A very strong current is necessary to produce the reaction, and this is, of course, attended by pain ; hence electrical examinations are difficult in infants. If such tests are made the normal arm should be taken as a standard for com- parison. If the sensibility is tested by a needle it will usually be found to be considerably impaired over the area of the -arm corresponding to the cutaneous branches of the circumflex nerve — a heart-shaped area whose point corresponds to the insertion of the deltoid muscle, and sometimes, also, along the outer side of the arm and forearm in the dis- tribution of the musculocutaneous nerve. After this condition has remained for several weeks or months without much change, as it often does, the muscles which are paralyzed become considerably atro- phied and feel unduly soft and yielding. (Fig. 90 ) The actual size of the limb, however, may not be very perceptibly reduced, for the fat in a thoroughly healthy baby is much greater than the substance of muscular tissue, and, therefore, there is not the rapid wasting of the arm and forearm which is noticed in adults suffering from paralysis of the brachial plexus. When the condition has lasted three or four months there sometimes appears a slight stiffiiess of the unparalyzed muscles, so that bending the forearm and wrist or opening the little closed hand is not as easy as at the beginning. A permanent rigidity^ however, 180 INJURIES OF SPINAL NEBVES AND NEUEITIS. rarely if ever develops. Many of these cases go on to spontaneous recovery, which ensues within six months after birth. Others remain longer and do not recover within the first year. The condition may remain for three or four years and pass away only when the child is old enough to be taught systematic gymnastic exercises. I have seen one case where no recovery had ensued after seven years, and then an operation by Dowd demonstrated the existence of a cicatricial mass at the junction of the roots of the fifth and sixth cervical nerves in the Fig. 89. Fig. 90. Boy, aged seven, whose right arm had been par- Atrophy of left shoulder and arm due to birth alyzed since birth. The position of the arm and palsy. (W. N. Bullard.) hand and the atrophy are typical. neck, which had prevented regeneration. (Fig. 91.) This was too extensive to warrant removal. I have seen one girl of fourteen who still had complete disability of movement at the shoulder-joint and in- ability to supiuate the arm, due to the occurrence of birth palsy. The course of the disease and the final condition depend upon the severity of the lesion. The lesion in all cases of birth palsy involves the brachial plexus. obsteTbical pjbalysts. 181 When the upper arm type of paralysis is present the roots of the fifth and sixth c^vical neryes are involved. When the lower arm type is present the seventh and eighth cervical nerves are affected. Duchenne showed that pressure backward upon the side of the neck is liable to compress these two nerves against the lamina of the sixth cervical vertebra. Erb has shown that it is possible by a careful examination to find a spot two centimetres above the clavicle, back of the outer edge of the sternomastoid muscle, corresponding to the point of emergence of the sixth cervical nerve between the scaleni, at which point irritation by the faradic current will produce a contraction in the deltoid, biceps, brachialis anticus, and supinator longus muscles ; and if the irritation be increased the extensors of the wrist will also con- tract. Pressure upon this particular region is often made during delivery either by the clavicle or by forceps or by the fingers of the obstetrician. This is more common when there is a breech presenta- tion and the after-coming head is extracted in the common method. Fig. 91. Thickened cord of brachial plexus at the junction of the 5th and 6th cervical nerves found at operation on boy shown in Fig. 89. (Natural size, from a sketch by Dowd.) The index and middle fingers of the left hand being open like a fork over the shoulders of the child, traction is commonly made upon the shoulders, and the pressure of the obstetrician's finger in the neck often produces injury of the plexus. In some cases injury of the plexus is produced by attempts to bring down the hand or arm in breech presentations or to replace these when the head presents. In these cases it is probable that the roots of the nerves are torn or a portion of the upper cord of the plexus is torn.^ Forceps applications in an awkward position may also produce this injury. Children do not appear to be very liable to injury of the nerves in spite of the many accidents which befall them. I have not seen a case of traumatic neuritis in the plexus of a child below the age of fourteen years, ex- cepting the form of brachial neuritis just described. Treatment. — The arm should be kept in a sling, with the elbow flexed, and should not be allowed to hang down, since its weight often suffices to overstretch the ligaments of the shoulder which are no longer 1 W. N. Bullard. Amer. Jour. Med. Sci., July, 1907. 182 INJURIES OF SPINAL NEilVES AND NEUEITlS. assisted by the action of the deltoid. The entire arm should be rubbed daily, the mother being taught the kneading process of massage. It should be bathed freely night and morning with hot and cold water, so as to increase the general circulation and nutrition. Unintelligent people will be more likely to keep up the necessary rubbing if some simple lotion cr ointment be ordered. As soon as any voluntary motion can be made the child should be encouraged to make it, even if it be necessary to bind the unaffected limb to the side of the body. As the child grows older systematic exercises of a gymnastic kind should be insisted upon daily. Galvanic treatment in accordance with the regular methods described on page 172, should be begun early and used until recovery has occurred. The mildest current which will produce contraction in the muscles should be used, that the treatment may not be too painful, and an intelligent nurse or mother can be instructed to carry out this treatment at home. If there is no im- provement after two years an exploratory operation should be under- taken. The upper part of the brachial plexus should be exposed, the nerves should be freed from cicatricial tissue if possible ; and if not possible the cicatrix should be cut out and the ends of the divided nerves be sutured. The shoulder may be elevated and the head drawn over to the side and held in this position by a stiif plaster-of- Paris cast during the time of union in order to prevent traction upon the united nerves. A number of successful instances of this operation have been recently reported.^ PARALYSIS OF THE CIRCUMFLEX NERVE. Injuries of the shoulder and dislocations of the shoulder sometimes produce a paralysis of the circumflex nerve. The symptoms consist of pain and tenderness in the course of the nerve ; paralysis and atrophy of the deltoid muscle, which prevents abduction of the arm, a relaxation of the ligaments of the shoulder-joint, as the deltoid muscle keeps the humerus in apposition to the socket ; and an area of anaes- thesia, triangular in shape, with the apex of the triangle downward corresponding to the insertion of the deltoid. See Plate XIV. A patient under my care had been in the habit of standing in his office and resting his shoulder against the sharp edge of a mantel. About the first of May he began to suffer from pain in the back of the neck and about the shoulder, and after ten days his deltoid became sud- denly paralyzed. This paralysis remained, in spite of treatment by electricity and massage, until the first of November, though by the middle of July he had recovered power enough to raise the arm above the head. The anaesthesia over the shoulder disappeared within the first month. ^ See British Medical Journal, February 3, 1903. NEUBITIS OF THE SHOULDEB-JOINT. 183 PARALYSIS OF THE SUPRASCAPULAR NERVE. Paralysis juif the suprascapular nerve occasionally occurs after dis- location of the humerus, or may be produced by falls upon the shoulder or on the hand, causing contusion of the shoulder. The symptoms are chiefly referable to paralysis of the supraspinatus and infraspinatus muscles. I have seen this following the removal of deep cervical glands in the neck by accidental division of the nerve trunk. ' The shoulder is very much hampered in its movement, as is also the arm. When the arm is abducted and raised the head of the humerus falls from lack of support by the supraspinatus muscle ; hence the arm can- not be held up. Movements of outward rotation are also impaired by the paralysis of the infraspinatus. The scapula is slightly rotated, its lower angle being moved upward and inward, and its upper angle pro- jecting on the side of the neck. The defective action is somewhat supplemented by the deltoid and the teres minor, which muscles occa- sionally become a little hypertrophied in the course of the case. Some- times a small region of anaesthesia is found over the scapula. NEURITIS OF THE SHOULDER-JOINT. PAINFUL SHOULDER. A condition of extreme pain and stiffness at the shoulder-joint, due to a neuritis of the nerves within the joint, is not an uncommon affec- tion. It occurs, as a rule, in women of middle age, but men are not exempt. It occurs in gouty and in anaemic and cachectic individuals, and is often seen in old age. It sometimes appears to follow an ex- posure to cold, and sometimes it can be traced to a slight injury. It begins rather suddenly with pain in the shoulder-joint, but without any swelling or exudation within the joint and without the appearances of rheumatism or arthritis. Very soon any movement becomes extremely painful, and as a result the muscles about the joint are thrown into a state of tonic spasm. The arm is adducted tightly to the chest, and while slight forward or backward motions are possible, any attempt at ab- duction is followed by an immediate contraction in the pectorals and latissimus dorsi. There are tender points in front and at the back of the joint, and pressure of the head of the humerus into its socket is very painful. The condition remains for many weeks, and as the result of the immobility the deltoid frequently becomes atrophied. Occasionally I have seen an anaesthesia in the circumflex area of the skin and such a rapid atrophy of the deltoid as to suggest a neuritis of the circumflex nerve, but this is not an invariable accompaniment of the affection. When there are no objective signs of neuritis in the shoulder, or of tenderness about the nerves in the neck, the affection may be thought to be hysterical, as neurotic individuals appear to be more subject to it than others. But the lack of remissions in the dis- ease and the fact that the spasm of the muscles does not appear to relax under chloroform — a fact that I observed in a patient who had an operation for cancer of the breast while this affection was in prog- ress — leads me to believe that the spasm is a reflex one from the nerves within the joint and that the origin of the trouble is a neuritis 184 INJURIES OF SPINAL NEBVES AND NEURITJS. of these nerves. As any motion is extremely painful, patients guard the arm by carrying it in a sling and by bandaging it to the chest. They find hot applications and packing in cotton agreeable, and they usually obtain considerable relief from the application of a Paquelin cautery to the front and back of the shoulder. It seems to have less effect when applied over the region of the deltoid muscle. The treat- ment is rest, cautery, and the use of sedatives, including codeine, if necessary, for the pain. When the acute symptoms are over, massage of the muscles may be attempted, but movement of the joint is to be avoided, as it is liable to produce a return of the spasm in the muscles. In all cases that I have seen, recovery has eventually occurred, but from four to six months have elapsed before the full use of the arm has been restored. PARALYSIS OF THE POSTERIOR THORACIC NERVE. Injuries to the side of the neck sometimes bruise the long posterior thoracic nerve which passes to the serratus magnus muscle. I have seen two cases of this kind occurring in porters who had carried a piano upon the shoulder ; and several cases from injuries to the side of the neck. I have seen a case resulting from the effort of swinging a hammer. Falls and blows upon the neck or shoulder which cause extreme extension of the head may also produce this. Pressure on the shoulder in a woman placed in the Trendelenberg position on the oper- ating table caused it in one of my cases. And many cases have been noticed after exposure to cold. The symptoms of the disease are severe pains about the shoulder and neck and total paralysis of the serratus magnus muscle. It will be remembered that the function of this muscle is to hold the posterior edge of the shoulder-blade against the chest and to rotate the shoulder- blade in the act of raising the arm. When it is paralyzed the shoul- der-blade projects from the chest like a wing in a very characteristic manner (Figs. 92, 93, 94); and the arm cannot be lifted much above the horizontal level when the forearm is extended. Motions of the forearm forward and backward are also somewhat impaired. If the arms be abducted, forcible inspiration shows a defective expansion of the chest on the paralyzed side. An electrical examination usually demonstrates a reaction of degeneration in the serratus magnus muscle. The motor points of this muscle are on the side of the chest beneath the axilla. A slow recovery usually ensues, provided the nerve is not entangled in the scar at the point of injury. If recovery fails to occur after several months, a surgical exploration is justifiable. PARALYSIS OF THE MUSCULOSPIRAL NERVE. This nerve is injured more often than any other of the nerves of the arm inasmuch as it is exposed in its passage around the humerus just below the insertion of the deltoid. Pressure of the head lying upon the arm, which frequently occurs in the sleep of intoxication, is sufficient PABALYSIS OF THE MUSCVLOSPIBAL NEHVE. 185 to cause musculospiral palsy, or the compression of nerve in the axilla, if during sleep the arm hangs over a sharp edge of a bench or bed. The pressure of a crutch may also produce this form of paralysis. In Fig. 93. Paralysis of the serratus magnus muscle, causing displacement of the scapula when the arm is held forward. (Icon, de la Salpetri6re. ) Paralysis of the serratus magnus muscle, causing displacement of the scapula when the arm is held forward. Fig. 94. Paralysis of the serratus magnus from injury of the posterior thoracic nerve. Position of the scapula when the arm is abducted. Further elevation of the arm is impossible, as rotation of the scapula is impossible. Germany this is called " police paralysis," because of the method used in securing the arms of a prisoner by placing a bar across his back and beneath his two elbows and tying the forearms firmly to the arms. 186 INJVnitS OF SPINAL NSBVES AND NEVBITIS. Gowers describes three cases which have followed violent contraction of the triceps — one in pulling on a pair of tight boots ; one in throw- ing a stone, and one in grasping a su})port to prevent falling. I have seen cases which have developed from pressure during a long-continued anaesthesia, the patient coming out of the anaesthetic to find his arm paralyzed. The nerve may be injured in fractures of the middle third of the humerus. In one patient the administration of a hypodermic injection in this region was followed by paralysis. The symptoms produced by musculospiral palsy are drop-wrist, with pronation, due to paralysis of the long extensors of the wrist and long Fig. 95. Drop-wrist due to musculosiiiral paralysis. extensors of the fingers and thmnb, and weakness in flexion of the elbow from paralysis of the supinators. If the nerve is injured in the axilla the triceps is also paralyzed. Sometimes in very slight cases the supinator longus is not paralyzed. The position of the hand in drop-wrist is shown in Fig. 95. The action of the flexors of the hand is apparently weak in grasping objects, but if the wrist be extended by the examiner and held firmly in this position, the flexors are found to act in a normal manner. The awkward action of the forearm gives the patient considerable discomfort, especially the loss of power of supination. After the drop-wrist has continued for some days a prominence appears upon the back of the hand as a result of the over- flexion of the wrist, due to a partial dislocation of one of the bones of the hand. But this subsides when recovery has taken place. The muscles paralyzed show the reaction of degeneration, excepting in very light cases. In the early stage of the aflection some tingling and numbness may be felt over the back of the hand at the base of the index finger and thumb, and an impairment of sensibility may often be demonstrated in this region. (See Plate XIV.) This, however, PaMLTsis of the ULNAB nebve. 187 rapidly subsides. Musculospiral paralysis can hardly be mistaken for any other disease, though drop-wrist may develop in any form of mul- tiple neuritis, especially in lead palsy. It is then, however, bilateral, and the supinator longus usually escapes. The prognosis is good for recovery, but this usually requires from three to five months. The treatment is that for ordinary traumatic neuritis. PARALYSIS OF THE MEDIAN NERVE. This may occur from wounds or injuries of the forearm or of the brachial plexus, also from pressure of a crutch. The median nerve supplies the pronators, the flexor carpi radialis, the flexors of the fingers, and the muscles that abduct and flex and oppose the thumb and tlie two radial lumbricales, which flex the first phalanx ; hence this injury produces paralysis in these muscles, resulting in supination of the forearm and inability to flex the fingers firmly or to use the hand. The thumb cannot be abducted or opposed to the tips of the fingers. The anaesthesia produced is shown in Figs. 96 and 97. Pain is sometimes felt in the hand. When the nerve is injured at the wrist the action of the long flexors is not interfered with, hence the paralysis is limited to the fingers alone. There is usually some atrophy of the thenar muscles. Trophic changes on the skin and nails often occur. The prognosis and treatment are in accordance with ordinary trau- matic neuritis. PARALYSIS OF THE ULNAR NERVE. The ulnar nerve is very frequently injured, as its course is an ex- posed one at the elbow and in the forearm. It may be affected by pressure in the axilla by a crutch, but this is rare. Exposure to wet and cold often causes ulnar neuritis. Long-continued pressure in a condition of anaesthesia, or even in the heavy sleep of intoxication, may cause paralysis of the muscles supplied by the ulnar nerve, and after dislocation and fractures of the arm or forearm it has been frequently observed. Callus, after fractures about the elbow, occasionally presses on and involves the nerve. The ulnar nerve supplies the flexor carpi ulnaris, the ulnar half of the deep flexors of the flngers, the muscles of the little finger, the interossei, the inner two lumbricales, and the adductors of the thumb. The result of paralysis of the ulnar flexor of the wrist is not very manifest, as other muscles are capable of doing the work of the flexor carpi ulnaris. A paralysis of the flexor profundus digitorum makes the patient incapable of flexing the first phalanges of the little and ring fingers, hence there is no opposition to their extreme exten- sion, and part of the deformity of the hand resulting from ulnar nerve palsy (Figs. 98 and 99) is due to this cause. The chief disability, however, produced by ulnar nerve lesions is the paralysis of all the intrinsic muscles of the hand excepting the first and 188 INJUmES OF SPINAL NERVES AND NEVmflS. second lumbricales, which are supplied by the median nerve. As a result of this paralysis the use of the thumb and fingers is very much impaired, the thenar and hypothenar eminences become flat and flabby, Fig. 96. Fig. 97. Showing areas of sensory loss in injuries of the median nerve. Horizontal lines show total anaesthesia. Vertical lines show partial anaesthesia. ( Bowlby. ) Fig. 98. Fig. 99. Showing sensory loss and abnormal position after injuries of the ulnar nerve. (Bowlby. ) the interossei of the hand are also atrophied, so that the bones and long tendons of the fingers stand out beneath tlie skin ; and the little and ring fingers are flexed in their second and third phalanges because Muscles of back NEURIT IS OF THE LUMBAB NERVES. 189 of the inability to extend them, due to the complete paralysis of the interossei. Tjais is the second cause of the deformity produced in the hand. The extension of the middle and end phalanges of the other two fingers is also considerably impaired. The adduction and abduc- tion of the fingers is completely abolished and the thumb cannot be brought forward because of overextension of its first phalanx by the extensor longus pollicis. The sensory disturbances produced by ulnar palsy are tingling and numbness and possibly pain in the inner half of the hand, in the little finger and one-half of the ring finger, and these parsesthesia are usually attended by anaesthesia, which may be total in the little finger, but is only partial in the hand and ring fingers. The sensory disturbance is not always a total one, sensations of tem- perature and pain being very often retained when the sensation of touch is lost. Trophic disturbances are very liable to occur in the anaesthetic region of the skin. (See Fig. 104, page 203.) The prognosis and treatment are those of general neuritis. NEURITIS OF THE INTERCOSTAL OR DORSAL NERVES. This occasionally occurs after injuries in which ribs are broken or from pressure by small tumors. In one case such a tumor caused in- termittent pressure as it occasionally got caught between the ribs and then produced excruciating pain. It was successfully removed. The nerve aifected will be indicated by the distribution of the pain or of the ansesthesia, as shown on Plate VII. There is no paralysis caused by intercostal neuritis. NEURITIS OF THE LUMBAR NERVES. The anatomy of the lumbar and sacral plexus is shown in Plate XV. The lumbar plexus supplies the flexors and abductors of the thigh, the extensors of the knee and the cremaster muscle. Its sensory dis- tribution is shown in Plate XIV. The lumbar plexus rarely suifers from neuritis, as it is well pro- tected from cold or from external injury. But tumors within the spinal canal, tumors in the abdomen, cancer of the spine, and psoas abscesses occasionally set up a neuritis or cause pressure on the nerves of the plexus. I have seen several cases following operations for appendicitis where filaments of the nerves were divided. The symptoms may be limited to one or two muscles, such as those supplied by the obturator nerve, or the anterior crural nerve, or may be widespread. In the first case the legs cannot be crossed, as the abductors of the thigh are paralyzed and rotation of the leg is impaired. In the second case the extensors of the knee are weak and atrophied and the knee-jerk is lost. In both cases the inability is marked in walking, and the patients move with difficulty. There is usually pain in the groin and thigh, and sometimes this shoots downw^ird into the genital 190 INJURIES OF SPINAL NEBVES AND NEUBITIS. region. There may be tenderness along the course of the anterior crural nerve. The degree of anaesthesia in the skin is slight, but tingling and numbness are felt in the sensory distribution of the plexus. PARALYSIS OF THE EXTERNAL CUTANEOUS NERVE. The external cutaneous nerve of the thigh supplies the skin over the outer portion of the thigh, from the line of the trochanter downward to the knee. Occasionally this nerve is diseased and then, as a rule, there is a sensation of pain in its distribution, which is attended by parsesthesia and by diminution of sensibility in the skin. The affection runs the ordinary course of a neuritis, and should be treated as a peripheral neuritis. Attention was first called to it by Bernhardt, and hence it has been termed " Bernhardt's Paralysis." NEURITIS OF THE SACRAL PLEXUS. Neuritis of the sacral plexus is a rare affection and is usually due to injury of the sacral plexus during a difficult labor. Every obstetrician has observed a number of cases in which during a delayed or difficult labor, the woman has been seized with sudden severe pains in one leg, usually in the back of the leg, and often below the knee, and has suffered from a flexor spasm of the leg for some hours during labor. A day or two after delivery paralysis of the muscles of the leg below the knee develops, and also a condition of anaesthesia extending down the back of the thigh and around the outer side of the leg and into the foot. In some cases the bruising of the nerve is a slight one and all the symptoms subside within three or four weeks. In other cases the injury is more severe, and months elapse before the paralysis subsides and the patient is able to w^alk. It is to be remem- bered that conditions of paralysis may follow labor from anaemic states of the spinal cord or from an infectious multiple neuritis. These con- ditions, however, produce paralysis in both legs, while the disease under discussion is always unilateral. In sacral plexus paralysis the functions of the bladder and rectum are usually normal, and the symp- toms of neuritis, pain, tenderness, paralysis, reaction of degeneration, are limited to the distribution of the sciatic nerve. Tumors in the pelvis and caries of the sacrum produce the same condition. NEURITIS OF THE SCIATIC NERVE. Sciatica, or neuralgia of the sciatic nerve, is considered in Chapter XLII, but there are many cases of so-called neuralgia which are actually due to neuritis, and there are cases of neuritis of the sciatic nerve which develop without any preceding neuralgia. The disease may be pro- duced by any one of the many causes of neuritis and neuralgia, but seems to be more common as a sequel of the grippe than of any other infectious disease, and it also occurs with great frequency in gouty and anaemic individuals. Symptoms. — The symptoms are pain in the entire domain of the NEUEITIS OF THE SCIATIC NEEFE. 191 sciatic nerve and tenderness along the course of the nerve, which is not to be confounded "vv^ith the existence of tender spots at Valleix's points present in sciatica. The nerve is found to be tender in its entire length and not infrequently localized swellings can be detected by palpation. The pain is usually constant as contrasted with the paroxysmal attacks of neuralgia. The patient is never able to get into an easy position so as to be free from pain. There is great restless- ness and uneasiness in the entire limb, and all movement is extremely distressing. Walking can only be done with difficulty and pain and has to be avoided. Very often an actual paralysis develops in the muscles supplied by the sciatic nerve, namely, those below the knee, and an atrophy with reaction of degeneration may develop. Not infrequently the pain of the neuritis gives rise to reflex spasms in the muscles, and such twitchings of the muscles of the leg are liable to be exceedingly distressing and painful. The leg is frequently hot and the secretion of perspiration may be increased. I have occasionally seen glossy skin upon the outside of the leg and upon the foot and irregular growth of the toe-nails. The disease is very slow in its progress, and often remains for months before recovery follows. Treatment. — Treatment consists of hot applications to the leg, which must be kept at rest, the patient being confined to thebed and the position of the limb changed frequently by the aid of numerous small pillows. It is a mistake, in my opinion, to apply a long splint to the limb, inasmuch as the strained position produces very great disturbance and discomfort. But a tight strapping about the hips by adhesive plaster, holding the pelvic bones and the sacrum together sometimes is useful. The tenderness of the limb secures a sufficient amount of rest to the part without this enforced bandaging. Hot applications may be kept up by poultices or by the use of hot bottles, but great caution is to be used in applying the latter, to avoid blisters or serious burns, inasmuch as the skin may be insensitive below the knee. Some patients experience relief from the use of electricity, the continuous galvanic current being employed, with the positive pole over the painful nerve, the negative pole being placed upon the back. The best method of treatment, however, is by means of the actual cautery, which often gives relief from the pain for several hours, so that the patients are glad to have the application repeated, even though it is painful. The various forms of hydrotherapy, especially a hot douche in a bath, are of service. As in the treatment of brachial neuritis, sedatives may be used freely. Hypodermics of cocaine in the upper part of the nerve are often better than morphine. When the condition is not improved by such treatment, puncture of the nerve by a needle has been known to give relief, probably by allowing fluid to escape from the sheath. Several needles may be passed into the nerve and allowed to remain for an hour. This must be done with great care so as to be aseptic. In chronic cases stretching the nerve has been done with some good results, but too much should not be promised, as the operation is uncertain. The sciatic may be stretched without an opera- 192 INJUBIES OF SPINAL NEBVES AND NEUBITIS. tion, the leg being extended and the thigh forcibly flexed on the trunk. This is usually a very painful procedure, requiring an ansesthetic. It is occasionally of benefit in those cases where an interstitial neuritis has caused adhesions between the nerve and its sheath. PARALYSIS OF THE EXTERNAL POPLITEAL NERVE. The external popliteal branch of the sciatic nerve in its passage around the fibula is exposed to injury either directly from pressure, as by a tight garter, or from external wounds, or from dislocation or fracture of the fibula. The result of such injury is a paralysis of the tibialis anticus, of the long peronei muscles in the leg and of the extensors of the toes, causing a dropping and adduction of the foot and toes and lame- ness in walking. The entire leg is raivsed so that the toes do not trip the walker. Atrophy soon follows with reaction of degeneration. There may be a loss of sensation in the outer side of the leg and on the back of the foot. Prognosis and treatment are the same as in other forms of traumatic neuritis. PARALYSIS OF THE INTERNAL POPLITEAL NERVE. Paral^^sis of the internal popliteal nerve is much more rare, as it is protected by its deep position under the knee. Occasionally it is strained or wounded. Then paralysis of the muscles of the calf of the leg and of the sole of the foot causes much difficulty in walking. The patient cannot rise on the toes or flex the ankle or toes. There may be pain, numbness, or anaesthesia in the outer part of the foot and in the sole. NEURITIS OF THE PLANTAR NERVES. The finer branches of the plantar nerves are not infrequently affected by neuritis. Such neuritis may be traumatic from walking in ill-fitting shoes, (3r upon very rough ground for an unusual length of time. It may be due also to taking cold, as when a person stands for some hours in the wet, or has the feet chilled and frozen. The symptoms are pain along the course of the nerves, between the long bones of the foot, great tenderness to pressure, and inability to endure the pressure of the shoe and inability to wallc on account of the pain. Gowers pointed out the fact that propulsion of the body forward is done by pressure on the toes, and that when they are paralyzed the act of walking is seriously impeded. Not infrequently irregular areas of anaesthesia may be found in the sole, and the patient suffers from prickling and burning pain, as well as from sharp neuralgic pains. The sensory symptoms may be associated with motor symptoms of paralysis of the intrinsic muscles of the foot, so that the toes cannot be moved freely and the foot may become quite thin in consequence of the atrophy. Reaction of degeneration is not infrequently present. The affection must be treated by rest in bed, the patient not being allowed to walk about, by local applications of heat and by the general measures recpm' mended for the treatment of neuritis. TUMOBS OF THE NERVES. 193 MORTON'S TOE. In some persons the second digital branch of the internal plantar nerve in its passage between the enlarged ends of the first and second metatarsal bones is compressed by anything which brings these two bones in close apposition. In others, any one of the digital nerves is similarly liable to be compressed. It seems probable that a con- genitally abnormal position of the nerve is the predisposing cause of this affection. The disease develops in adults, and is usually the consequence of wearing shoes that are too narrow. The patient will be seized with a sudden sharp pain between the great and second toes, or between other toes, which is so agonizing in character as to prevent his taking another step, and which lasts until the shoe is re- moved and by a certain amount of manipulation the bones are separated and the pressure removed. But such attacks are liable to recur with great frequency, and to give rise to such distress and inability to walk as to require treatment. The disease is purely mechanical in its origin, and, therefore, anything which removes the pressure or which prevents the recurrence of the pressure will cure. In some cases it is sufficient to prescribe a very wide shoe. In others it is necessary to insert pads and strap them with adhesive plaster to the foot in such a position as to keep the metatarsal bones separated. Flatfoot may be the cause, and if corrected the pain may be obviated. But in some cases all these mechanical contrivances fail to give relief, and then resort to surgical treatment may be necessary. Morton was the first to operate for this condition, and hence it has been named after him. The opera- tion may consist in a division of the nerve above the point of pressure, by an exsection of a portion of the nerve, but inasmuch as regeneration is sure to occur, this operation is unsatisfactory. The operation de- vised by Morton was exsection of the end of the metatarsal bone caus- ing the pressure, and this has uniformly given relief. TUMORS OF THE NERVES. Neuroma. — Any form of tumor — fibroma, angioma, sarcoma, etc., — may develop within a nerve sheath or upon it and thus produce an apparent tumor of the nerve. In such cases the nerve fibres may pass through the tumor or may be dissected apart by it and pass around it. Such tumors have been termed false neuromata, for they are not made up of nerve cells or fibres. But the fibres may become pressed by the new-growth and undergo degeneration. And the symptoms and course of the case under such conditions will be exactly such as have been already considered under compression of the nerves. Fig. 100 shows such false neuromata, which are very often multiple, as in this case. Neuroma, or a tumor consisting of nerve fibres, first described by Virchow, is a rare form of tumor. The fibres of which it consists may be medullated or non-medullated ; nerve cells are not found in such tumors ; connective tissue exists in greater or less degree, so that 13 194 INJUEIES OF SPINAL NEBVES AND NEURITIS. some tumors are neuro-fibromata. There appears to be a tendency for such tumors to be multiple, and in the large majority of the recorded cases hundreds of minute tumors have developed. Occasionally the tumors have been large, but usually they are the size of a pea. When they appear on the sensory nerves of the skin they are easily felt and are usually tender. Under these circumstances they have been called riG. 100, Multiple neuromata. Nerves of the arm, the lumbar plexus, and the sciatic nerve. Specimen from the Museum of the College of Physicians and Surgeons. " tubercula dolorosa." I have seen a patient with more than a hun- dred such tumors distributed over the entire body. Hoggan has shown that some tumors of this description were adenomata of the sweat glands. That some congenital tendency to the multiplication of nerve fibres is at the basis of this affection is proved by the fact that many of the cases reported have been in children. Thus in the so- called plexiform neuroma of the fifth nerve the disease is usually con- genital and other nerves have been affected later in life. The tumors attain a certain size and then, as a rule, cease to grow ; the condition remains permanent until death from some other cause. In the majority of cases no symptoms are caused by neuromata, as ISCHEMIC PARALYSIS. 195 the nerves on which the tumors develop are not afFected by the growth. The tumors riiay be felt 'and may be tender to pressure, but are not subject to treatment and cannot safely be removed, as they are so numerous and as the nerves may be cut. Occasionally pain, hyperses- thesia, and numbness are caused by neuromata. There is one form of neuroma which, however, requires special men- tion. It is the neuroma which develops on the cut end of a divided nerve, either after an amputation or after an injury without union. Such a neuroma may be extremely painful and tender and may cause pains referred to the peripheral termination of the nerve, and also spasms in the muscles related to these terminations. In such cases excision is imperative, but recurrence is not infrequent. In one patient under my observation, who suffered from great pain referred to the toes, for many years after an amputation of the leg above the knee, excision of the neuroma on the sciatic failed to give relief. A portion of the sciatic nerve in the thigh was then excised, but this also failed to stop the pain. In this case it seemed probable that atrophic changes had occurred in the spinal cord, such as are known to follow amputations, and that these had involved not only the anterior horns, but also the sensory columns. ISCHEMIC PARALYSIS. There is a condition of paralysis which occurs as a result of tight bandaging of a limb (usually after fractures) termed ischsemic paralysis. The bandage compresses the muscles and vessels and nerves and causes very great pain. This pain is ascribed, erroneously, to the fracture, and the bandage may be allowed to remain on the limb for several days in spite of the pain. Then when it is removed the limb is found to be paralyzed, the muscles are hard, indurated, rigid and cannot be moved voluntarily and often any attempted passive motion is found to be very difficult and to cause pain. Thus, if the limb affected be the arm, it may be impossible to flex or to extend the fingers without meeting with resistance, and causing pain in the muscles of the forearm. The mucles are swollen, are hard to the touch, do not pit on pressure but are tense; their mechanical excitability is lost and the electrical excitability is diminished. The limb itself is cold, its circulation is defective. There may be numbness and anaesthesia to all sensations in some cases; in other cases there is no evidence of neuritis in the limb. The skin may be mottled or glossy or rough. Sometimes ulcerations due to the pressure are found. This condition may be ascribed to neuritis in consequence of the fracture, by the surgeon who is responsible for the tight bandaging. But it is easily differentiated from neuritis, for that condition never causes the brawny infiltration and rigidity of the mucles present in ischsemic paralysis; and the anaesthesia is limited to the domain of definite nerves in neuritis while in this condition it is rarely present or if so it is distributed over the entire limb. 196 INJUBIES OF SPINAL NEBFES AND NEUBITIS. The prognosis in ischsemic paraly>sis is grave. Months and even years may elapse before nature restores the circulation and nutrition of the muscles which are primarily affected. The treatment consists in daily massage, in very hot baths twice or three times a day kept up for half an hour and followed by cool spong- ing and in passive movements performed after the hot baths. Surgeons have sometimes cut down upon the tendons of the wrist or ankle when these appear to be matted together and adherent to their sheaths and have broken up adhesions with good results. CHAPTER VIII. MULTIPLE NEUEITIS. Varieties. Etiology. Symptoms. Course. Duration. Diagnosis. Prognosis and Treatment. Multiple neuritis or polyneuritis is a general disseminated inflam- mation or degeneration of the nerves, symmetrical in its distribution upon both sides of the body and generally aifecting all the nerves of the limbs, particularly in their terminal branches. It is hence called peripheral neuritis. The affection rarely, if ever, extends as high as the nerve plexuses. Varieties. — There are a number of forms of multiple neuritis which difi'er from one another somewhat in their pathology and in their symp- tomatology. The following etiological classification of these various forms is of practical use. 1. Toxic cases due to the action of a poison derived from without the body. These poisons are alcohol, carbonic oxide gas, bisulphide of carbon, the coal-tar products, especially sulphonal and trional ; and nitrobenzol ; also arsenic, lead, mercury, copper, phosphorus and silver. 2. Infectious cases due to some agent acquired or developed within the body as an accompaniment or sequel of diphtheria, grippe, typhoid, typhus, malaria, scarlet fever, measles, whooping-cough, smallpox, ery- sipelas, and septicsemic conditions, including gonorrhoea and puerperal fever, epidemic forms of beriberi or kakke, and leprous neuritis. 3. Cases due to general diseased states of the body whose origin is undetermined, such as rheumatism, gout, diabetes, anaemia, marasmus, general malnutrition consequent upon tuberculosis, syphilis, and senility, carcinoma, and local malnutrition produced by arterial sclerosis. 4. Cases due to exposure to cold and developing spontaneously without known cause. Etiology. — Multiple neuritis is more common in males than in females in all varieties. All ages are liable to be aifected. The diph- theritic type is of course more commonly seen in children than in adults, and if these cases are included in a general list it would appear as if children were more subject to the disease than adults. In 154 miscellaneous cases, not diphtheritic, 16 were between the ages of six- teen and twenty years, 34 were between the ages of twenty and thirty years, 54 were between the ages of thirty and forty years, 35 were be- tween the ages of forty and fifty years, 19 were between the ages of fifty and sixty years, and 10 were over sixty years of age. Heredity appears to play little or no part in the causation of multiple neuritis, excepting in so far as the general tendencies to gout, rheumatism, dia- 197 198 MULTIPLE NEUEITIS. betes, carcinoma, arterial sclerosis, and tuberculosis may be said to be hereditary. It seems probable that in many cases a number of different causes combine to start the neuritis. Thus in a person who has used alcohol for some years the disease may not develop until some acute illness, like an attack of the grippe, or of rheumatism, or an exposure to cold occurs, when a typical alcoholic neuritis may manifest itself. The par- ticular elements of causation will be more fully discussed in connec- tion with the various types of the affection. Symptoms. — A general statement of the symptoms occurring in any or all forms of multiple neuritis will be made, and then the special combination of these symptoms, together with the common course of the disease in the different classes of cases, will be presented. Some authors have attempted a symptomatic classification of the forms of neuritis, and distinguish between sensory, motor, and ataxic types of the disease.^ As many cases, however, present a combination of these symptoms such a division does not appear to me to be scientifically justifiable, however convenient from the clinical standpoint. The sensory symptoms of multiple neuritis are the earliest to appear and the last to pass away. In the majority of the cases on record, from whatever cause, numbness, tingling, or formication ushers in the disease. These forms of parsesthesise begin in the feet and hands and extend to the knees and elbows. They may be associated with burn- ing, stretching, boring, or tearing sensations M^hich distress the patient especially during the onset, and such sensations usually increase as the affection reaches its height. Their subsidence, as the case goes on, may be regarded as a favorable symptom, but they are among the last evidences of the disease to disappear. Pain is usually present as well as parsesthesise. It is sharp in character and is usually intermittent. At times it may be lancinating and so severe as to necessitate the use of morphine. It is fully as distressing as in cases of locomotor ataxia. Tenderness in the nerves and muscles is a constant symptom. It may be so extreme that the limbs cannot be moved or handled, and thus it may interfere with the application of electricity and massage. When the tenderness and pain are referred to the joints, as not infrequently occurs in the early stage of the disease and in the spontaneous and in- fectious cases, the case may be mistaken for one of acute articular rheumatism ; and if the joints are swollen or the limbs oedematous the difficulty of diagnosis is greatly increased. In addition to these subjective feelings some demonstrable distur- bance of the various sensations is usually present. Hypersesthesia to touch and also to electricity is not infrequently observed during the first two weeks. It is usually followed by some ansesthesia, although this rarely becomes complete. In some cases the loss of tactile sense is quite evident from the onset, either limited to the cutaneous distribu- tion of some special nerve, in which case oddly-shaped areas of insen- sibility will be found, as in lepra, or, as is most often the case, about ^ Judson Burj. Clifford AUbutt's System of Medicine, vol. vi. SYMPTOMS. 199 uniformly distributed over the distal parts of the extremities and fad- ing off inta normal conditions higher up upon the limb. When the ansesthesia is at its height the patient has difficulty in locating a touch upon the hands and feet, even though he feels it. The distribution of the ansesthesia when it is fully developed corresponds to the parts of the extremities covered by gloves and stockings, and hence has been named the glove-shaped and stocking-shaped area of anaesthesia. (Fig.lOl.) This distribution of anaesthesia, contrasting sharply, as it Fig. 101. N. cut.postr.- N. occip.maj. N. occip.min. N. Auric.magn. NN. supraclavic. R. Dors. N. spin. N. axill. N. cut medialis. N. intercost. N. rad. N. cut.medius. N. tnusccut. NN. cut.clunium ^N.perf lig.sacro-tub. ^ — N rad. N. uln. —N. median. K. cut.lat. N.peron. N. sapfc:- N. suralis ^| N.plant.lat.- N. plant. medr- The distribution of anesthesia in multiple neuritis. The dotted areas are anesthetic. These do not correspond with the distribution of the various nerves. does, with that due to spinal-cord disease, or to that occurring in injuries of single nerves, and being symmetrical upon the two limbs, is quite diagnostic of multiple neuritis. The transmission of pain and temper- ature sensations is sometimes delayed, but these impressions are usually felt quite acutely. The sense of pressure is less commonly unpaired. The sense of vibration as tested with the tuning fork is often lost early. The muscular sense escapes any affection in some cases, but in others is the most profoundly disturbed of all the senses. When it is involved the incoordination and ataxia are well-marked symptoms, and some cases have been mistaken for locomotor ataxia because of the pre- dominance of the disturbance of muscular sense. 200 MULTIPLE NEURITIS. The ataxia is very pronounced in diphtheritic cases, in cases due to arsenical poisoning, and in some cases due to alcoholic poisoning. The French and Germans distinguish a class of cases of neuritis which they term neurotabes peripherique or pseudo-tabes, because of the close resemblance to locomotor ataxia. These sensory symptoms are usually limited to the forearms and hands and to the legs and feet. In no case have they involved the entire extremities or the trunk, and but one case of facial tingling with anaesthesia has been recorded.* The skin reflexes are usually pre- served and are occasionally exaggerated. The special senses are rarely involved in multiple neuritis. It is true that optic neuritis has occurred in a few cases, especially in cases due to alcoholic poisoning, and in some cases hearing as well as sight has been aifected. These cases prove that no nerve can be said to be exempt from implication in this disease, but the liability to affection seems to be slight in the case of the nerves of special sense. In alco- holic cases toxic amblyopia has been found. The motor symptoms are as marked and as important as the sensory. Paralysis, beginning as simple weakness, with a feeling of fatigue on any exertion, gradually increases in severity until at the height of the disease it becomes complete. It usually comes on rapidly, so that within two weeks the patient is helpless ; but it may be less sudden and not deprive him of the power of walking and of using his hands for two or three months. It may be arrested in the slighter cases and not go beyond a condition of general feebleness in the extremities. In a few cases a very acute onset is recorded, all the symptoms develop- ing within three or four hours. The distribution of the paralysis is not uniform at the outset. It may begin in the muscles of the legs and then involve those of the forearms ; it may commence in all four extremities at once. It is always more severe in the muscles which move the joints of the feet and hands and the ankles and wrists. It rarely invades those which move the knees and elbows. The extensors of the wrists and fingers and the peronei and anterior tibial muscles in the legs are the muscles first affected, and it is usual for the paralysis to become more complete and to last longer in these muscles than in the flexors of the wrist or in the posterior tibial muscles of the legs. A marked tremor in the hands is not at all uncommon in the early stage of paralysis, especially in the alcoholic form, and usually the weakness is attended by slight incoordination and unsteadiness of gait before the patient is finally incapacitated from walking. When the disease is fully developed all the muscles below the knees and elbows are much weakened or totally paralyzed. Occasionally those of the thighs and arms are involved also, and the muscles of the trunk and those of respiration may become affected, and then the patient usually dies. In two cases which eventually recovered I have seen a total paralysis of the diaphragm lasting several days. In both these cases ^Lowenfeld. Neurol. Centralbl., 1885, No. 7. SYMPTOMS. 201 the patient's respiration ceased as soon as he fell asleep, and the ex- haustion from the long wakefulness and the voluntary efforts of breath- ing was very great. In some cases of multiple neuritis the cranial motor nerves become involved, those of the eye and of the face being most liable to inva- sion. It is only in fatal cases that the action of deglutition has been Fig. 102. Dropped wrists in multiple neuritis. affected, and when the pneumogastric is invaded and the heart be- comes rapid and irregular the prognosis is always grave, though not absolutely bad. The paralyzed muscles are relaxed, flabby, and atrophied ; they may or may not lose their mechanical irritability, but their normal tone is always lost, and hence their tendon reflexes are abolished. The loss of knee-jerk is an early symptom in the disease, though some cases have been observed in which it has been preserved. These were cases in which the neuritis did not advance up the legs sufficiently high to affect the nerves above the knee. The knee-jerk always disappears early in diphtheritic cases. To the electric current their excitability is very rapidly and mark- edly changed ; but the conditions which have been observed are quite various. Sometimes there is a simple diminution of excitability, and then a very strong faradic or galvanic current is needed to produce contractions. Frequently all faradic excitability is lost, and then the muscles react to a galvanic current only. In this condition it may re- quire a very strong galvanic current to produce contraction, and this fact is quite patli()gnornonic of ncui'itis, for in anterior poliomyelitis, where the muscles respond to galvanism only, it does not require a 202 MULTIPLE NEVniTlS. strong current to cause a motion until some months after the invasion. The action of the different poles is not uniform. In many cases the contraction of the muscle when stimulated with the positive pole is greater than when stimulated with the negative pole, and the contrac- tions may be sluggish. Then the reaction of degeneration is present; but in some cases the normal condition is found, and the negative pole produces stronger contractions than the positive pole. If the muscles which are not paralyzed be tested the same electrical changes may often be discovered in them. A loss of faradic irritability and a Fig. 103. Dropped feet in multiple neuritis. marked decrease in the galvanic irritability of the muscle and nerve are, therefore, important symptoms of multiple neuritis, and, as the disease goes on to recovery, a gradual increase in the galvanic irrita- bility occurs, a fact which is often of much aid in prognosis if care- ful measurements of the strength of current used be made by the galvanometer. As a result and accompaniment of the paralysis abnormal positions are assumed by the limbs. The dropped wrist and dropped foot are quite characteristic of multiple neuritis. (Figs. 102 and 103.) They are in part due to the action of gravitation and in part to the unop- posed action of muscles which are not very weak. But other deformi- ties may be present. In a few cases there have been extreme contrac- tures of all the extremities in flexed position. When the legs are thus flexed the posture is at first voluntarily assumed to relieve the pain, and later the flexor muscles become permanently contractured and shortened. These contractures may be exceedingly painful. In the severe cases a typical claw-hand and talipes equiuus are seen. These deformities usually subside as the power returns, or, if they do not, they can be corrected by proper manipulation and by apparatus. In SYMPTOMS. 203 a few cases it has been necessary to resort to tenotomy, but a perma- nent defornyty has not been recorded. The vasomotor and trophic symptoms are less constant than those already described. In some cases marked oedema has been an early and a permanent symptom. This may develop in the feet and hands, or may appear about the joints. It is usually temporary. The circu- lation is not impaired to any greater degree than is customary in a limb whose muscles are inactive, and coldness and cyanosis are rarely sufficient to attract attention. Sometimes profuse perspiration is a noticeable symptom, being limited to the paralyzed parts. This is often seen in alcoholic cases. It may be oifensive, and by its evapora- tion always causes a complaint of coldness. In other cases glossy skin makes its appearance early and remains until the regeneration of the nerves is complete. Its disappearance in one of my own cases was the first sign of recovery in the lower extremities. A change in the appearance and growth of the nails is very common, and a high ridge across the nail becomes evident as recovery begins, indicating the difference between the normal and abnormal nail formations. This is shown in Fig. 104. Bed-sores never appear. Other forms of trophic Fig. 104. Ridged appearance of the nails in traumatic neuritis. The hand is also atrophied, and the thenar eminence flat. The thumb cannot be opposed to the fingers. The scar over the ukiar nerve is visible. disturbance are rarely met with in multiple neuritis, and this is quite remarkable in view of the fact that it has been the tendency of late to refer such trophic affections as ulcerations, bed-sores, gangrene, pem- phigus, and various eruptions to lesions of the nerves. It is true that inflammations of the joints resembling those appearing in acute rheu- matism sometimes occur at the onset of neuritis ; but as they disappear quickly while other symptoms remain, it is improbable that they are to be traced to the changes in the nerves. They may be due to the infectious agent or to the same obscure cause which sets up the neuritis, or they may be evidence of an attack of acute articular rheumatism, which is in turn followed by neuritis, but they cannot be described as trophic symptoms of the disease, otherwise they would be more constant in their occurrence and more permanent in their duration. A negative symptom of great importance is the absence of any inter- ference with the automatic action of the bladder or rectum. Retention 204 MULTIPLE NEUEiriS. or incontinence of urine does not occur, and impaction within or a re- laxation of the rectum is not observed in the course of the disease. Constipation may ensue upon the long-continued rest in bed that is necessitated by the paralysis ; but it is by no means as obstinate or as difficult to relieve as it is in spinal-cord aflPections. Another negative symptom of some importance is the absence of pain or of anaesthesia upon the trunk. In the various forms of spinal- cord disease which are likely to be confounded with neuritis, zones of anaesthesia upon the body are not at all uncommon. It is well known that in locomotor ataxia bands of anaesthesia about the trunk and in the axillae are almost constant signs of the affection. It is well to rec- ognize the absence of this symptom in those cases of multiple neuritis which simulate locomotor ataxia. There are certain mental symptoms which develop during the course of alcoholic multiple neuritis, but as they do not appear in the other types they will be discussed in connection with the toxic cases. Course. — The course of the disease in multiple neuritis varies so exceedingly that no general statement can be made. In the next chap- ters in which the various forms of the affection are considered the course of the case in each form will be described. General constitu- tional disturbances, however, are not infrequent in the course of mul- tiple neuritis, and may be mentioned here. The onset is often sudden and accompanied by a marked febrile movement with chill and tempera- ture of 103° or 104° F. The fever may persist for several days, but usually subsides spontaneously and does not recur. In a few cases there has been a constant elevation of temperature of about one degree, persisting for several weeks. The general symptoms accompanying fever, viz., malaise, loss of appetite, nausea, disturbances of digestion, constipation, flatulence, occasional diarrhoea, febrile condition of the urine, general pains all over the body, headache, and discomfort usually pass off as this subsides. An enlargement of the spleen and a condition of leucocytosis has been discovered by examination during the onset in some cases, especially in those following an infectious disease and in cases that are not due to poisoning. These facts have led to the theory that neuritis may be, under some circumstances, a primary infectious disease. Increased rapidity of the pulse is very common in all forms of neuritis, and may persist during the entire course of the disease, the pulse ranging from 80 to 100. In some cases it becomes exceedingly rapid, reaching 140 or 160, being feeble and small. In these cases the neuritis has extended to the pneumogastric nerve. If this condi- tion persists for several days oedema of the extremities and finally of the lungs may result, and heart failure may be the primary cause of death. The duration of a condition of multiple neuritis is so different in dif- ferent types of cases that no general statement is warranted. In the diphtheritic cases recovery is quite rapid, and I have seen patients who had been completely paralyzed quite well within two months. In DIAGNOSIS. 205 cases of alcoholic neuritis of a mild type, where no actual paralysis developed, r^overy was usually complete in six months. In the severer cases, where drop-foot and drop-wrist had developed, at least a year, and in several cases two years elapsed before their health and power were restored. Arsenic and lead cases are also slow in their progress, from eight to twelve months being the usual duration. The duration is in exact ratio with the degree of degeneration present at the maxi- mum of the symptoms. Diagnosis. — While the individual symptoms occurring in the course of multiple neuritis are not different in character from those found in spinal-cord diseases, the diagnosis can usually be reached with very little difficulty when their combination, the causation, and the course of the case under examination are considered. There are three com- binations of symptoms in neuritis which resemble very closely, respec- tively, anterior poliomyelitis, locomotor ataxia, and diffuse myelitis, and to these attention must be directed. Atrophic paralysis, with reaction of degeneration and loss of reflexes, is common to anterior poliomyelitis and some cases of multiple neu- ritis. Poliomyelitis attacks healthy children, and is known to be due to an infection in many cases. In neuritis it is often possible to ascer- tain some previous condition of ill health or some infectious disease or constitutional state which has produced the affection. In neuritis, a more gradual onset, preceded and attended by numbness and pain, tenderness in the course of the nerves, tenderness in the muscles, and the persistence of sensory symptoms after the invasion will remove all doubt regarding the diagnosis. When these symptoms are not clearly marked the distribution of the paralysis in symmetrically situated muscles, especially if these muscles are supplied by single nerves, and the further extension to muscles in other nerve domains, rather than the affection simultaneously of muscles which are grouped physiologically {i. e., act together to perform one function) will point to neuritis. In neuritis the paralysis advances more or less gradually, while in acute poliomyelitis there is, after the onset, a subsidence of the paralysis in some of the muscles first involved. Ataxia, which is a common symp- tom in neuritis, never appears in infantile paralysis. Cramps in the muscles are complained of in neuritis, but not in poliomyelitis. In the latter fibrillary twitchings occur in the muscles which are paralyzed, but never in the former. Glossy skin never appears in poliomyelitis. Lastly, as the case goes on, a gradual complete recovery will be far more frequent if it was originally a case of multiple neuritis. Gowers has described a number of cases in which, he believes, the two diseases have occurred together, probably being produced by the same cause. This has been seen also in epidemics of infantile paralysis. Under these circumstances a mingling of the symptoms is to be expected, and no sharp differentiation can be made. Ataxia, loss of knee-jerk, pain, and sensory disturbances, including a loss of muscular sense, Romberg's symptom, that is, swaying when 206 MULTIPLE NEUBITIS. standing with closed eyes, and optic neuritis, are common to locomotor ataxia and to multiple neuritis. This form of neuritis has been called acute polyneuritic ataxia or neurotabes peripherica by Dejerine/ who was the first to point out clearly its resemblance to locomotor ataxia. In neuritis the relatively rapid onset of the ataxia, which follows closely upon tlie sensory symptoms ; the prominence of numbness and anaesthesia, rather than of lightning pains ; the extreme degree of the anaesthesia and analgesia, the tenderness of muscles and nerves, neven found in tabes, the usual occurrence of some degree of actual paresis, with atrophy and reaction of degeneration, and the absence of bladder and sexual symptoms, will point inevitably to the correct diagnosis. Furthermore, the ataxic form of neuritis only occurs after poisoning with alcohol or arsenic, or as a sequel of diphtheria, and the establish- ment of the causation will aid the diagnosis. Here again, the course of the case toward recovery and the return of the knee-jerk will decide in favor of neuritis if the diagnosis has not been reached in an early stage. There are very few symptoms of diffuse myelitis which are not found in cases of neuritis, but cases of diffuse myelitis of the type de- scribed by Duchenne (paralysie generate spinale subdigue ascendante) are very rare, and, indeed, it has been affirmed -by Leyden that the cases described by Duchenne under this name were really cases of multiple neuritis. A differential diagnosis between general myelitis and neuritis is made by a consideration of the following points : In neuritis affections of the functions of micturition and defecation do not occur. Girdle sensation is very rarely mentioned as a symptom. Bed-sores and cystitis have not been observed. In neuritis the advance of the paralysis is not like that in myelitis, namely, a gradual advance from legs to thighs, and thighs to trunk, and trunk to arms and neck ; but, as already stated, the paralysis begins in the legs and forearms simultaneously, and does not usually extend to the thighs and arms and very seldom invades the trunk. If the muscles of the abdomen and the muscles of respiration are involved in neuritis it is only in the rapidly fatal toxic cases, and even in these cases motions of the shoul- ders and hips are preserved until the end. In the type of myelitis described by Duchenne there are few sensory symptoms, whereas these are prominent in neuritis. If in myelitis there are sensory distur- bances and anaesthesia the areas are not glove-shaped and stocking- shaped and there is a well-marked line of demarcation around the trunk. This is particularly evident in transverse myelitis. In neu- ritis, however, the anaesthesia is chiefly observed in the hands and feet, in the forearms and legs, but rarely reaches as high as the arms or thighs. In neuritis there is usually tenderness in the nerves and in the muscles of the extremities which is not present in myelitis. In myelitis there is usually tenderness to pressure and sensitiveness to heat along the spine, a symptom not present in neuritis. In the older text-books multiple neuritis is not mentioned, and cases of it were de- scribed as cases of spinal-cord disease ; but since the knowledge of ^ Arch. phys. norm, et path., 1884, p. 231. TREATMENT. 207 neuritis lias become general the diagnosis of myelitis is more and more rarely made/ and now that disease is regarded as an unusual one. Prognosis. — The prognosis in multiple neuritis is good, provided the exciting cause can be removed. The only patients who form an exception to the rule are those whose constitutions are much impaired by excesses or by other diseases ; those who have so far indulged in alcohol or are so completely soaked with arsenic or lead as to be unable to throw off the poison, and those in whom the disease begins with great suddenness, advances rapidly, and involves the phrenic and pneu- mogastric nerves. These cases die either of respiratory paralysis or of some complication. When a case has reached the stationary period the prognosis is generally favorable, and if the encouraging signs of recovery already mentioned begin to appear a cure may be promised. The possibility of the complication of myelitis must not, however, be overlooked, and if it occurs the prognosis becomes at once unfavorable. Even in serious cases of alcoholism, with gastro-intestinal and cerebral symptoms, if the acute stage be safely passed and all alcohol be re- moved from the patient's diet, recovery from very extensive paralysis will occur. The cases of diphtheritic paralysis and ataxia usually re- cover without treatment. Treatment. — The treatment of multiple neuritis requires patience. As we have already seen, the majority of the patients recover, and it is probable that, if the cause of the affection were removed and the patients placed in favorable circumstances, expectant treatment would alone be sufficient. It is, however, not advisable to let therapeutics play a passive part. The course of the disease can be altered and its duration much shortened by active interference. In the stage of in- vasion the free use of aspirin, salophen, salicin, salicylic acid, or the salicylate of soda seems to have important results. These remedies cannot be said to act as promptly as in cases of acute articular rheu- matism, but the consensus of opinion is that their effect in multiple neuritis is very marked. They should be given, as in acute rheumatic fever, in large doses until noticeable effects are obtained. They should be combined with the bromide of potassium or sodium, partly because these drugs counteract unfavorable symptoms produced by the salicin compounds and partly because in the hypersesthetic irritable condition attendant upon the invasion of the disease they are indicated. This condition may require stronger sedatives and not infrequently morphine must be employed to give relief from the excruciating pains. The pains are often relieved by hot or cold applications to the limbs ; but as the muscles are often exceedingly tender, ordinary applications can- not be made. It is then advisable to use evaporating lotions, prefer- ably those containing chloroform, which may be soaked into light cambric or gauze and gently placed upon the limbs, which lie upon the softest pillows or which may be more comfortable if the patient is put upon a water-bed. Applications of a 5 per cent, solution of carbolic acid and of extract of witch-hazel have also been of use. If cool applications prove intolerable heat may be employed. The limbs may 208 MULTIPLE NEUBITIS. be enveloped with cotton and covered with oiled silk, a light bandage keeping these in place, or they may be frequently bathed in hot water and hot bottles placed against them, some soft substance intervening. One of niy patients found great relief from the parsesthesia by cold douches, while another preferred the use of hot water. It is best to let the patient decide, as long as the application has to be made for the relief of pain. Gentle friction with oil of cocoanut or cocoa-butter often affords comfort. In the chronic stage, as we shall see presently, heat is to be preferred to cold. Cases which are distinctly syphilitic, if such occur, should be treated from the outset with inunctions of mercury and large doses of iodide of potash. I believe that both these drugs should be employed together, even in the tertiary stage of syphilis, and it is my experience that all syphilitic nervous lesions, whether central or peripheral, yield more promptly to their combined use than to the employment of either alone. Malarial cases must be treated with quinine or Warburg's tincture. In non-malarial cases quinine has proved of no avail. In cases which are due to poisoning of any kind the first indica- tion is to eliminate the toxic agent from the system and the second to prevent any further ingestion of the poison. Iodide of potash in small doses, largely diluted, aids in the elimination. The second indication is easily fulfilled when arsenic or lead are the toxic agents, but when the case is due to chronic alcoholism special precautions are needed. Alcoholic cases require from the outset special treatment. The condi- tion at the time of the onset of the paralysis may be one verging upon delirium tremens. If all alcohol is suddenly removed, without due care to supply some other heart stimulant and to secure the perfect nutrition of the patient, serious collapse may ensue. The first neces- sity is therefore to take care of the general condition of the patient. If this will admit of the immediate withdrawal of all alcoholic stimu- lation it should be done ; if not, the alcoholic beverage must be imme- diately reduced in quantity, and as soon as possible wholly cut off. The use of milk diet, or kumyss, or peptonized milk, or, if necessary, rectal alimentation, will be followed by a gradual recovery of the power of assimilation, and as soon as the patient ceases to lose weight all alcohol may in any case be safely stopped ; its elimination by the intestines and kidneys may be hastened by appropriate means, and cerebral symptoms, if they arise, may be treated as in other cases of alcoholic intoxication. But it is in the chronic stage, when the patient is gradually recovering, that the vigilance of the physician is called into play to prevent a renewal of the poisoning. It is amazing that patients who know perfectly the injurious effect of alcohol upon them should insist upon getting it ; but it is done. And when these patients are surrounded, as is often the case, by sympathizing friends or servile domestics or unscrupulous nurses who ,do not appreciate the impor- tance of total abstinence for the patient, they often succeed in baffling all attempts to deprive them of the favorite drink. It is only when they are watched constantly by persons who can be implicitly trusted TREATMENT. 209 and who have sufficient authority to cut off all surreptitious supplies, that the physician can feel sure that his conunands are obeyed; and this precaution is by no means needless, even when it is probable that family servants are trustworthy, for the continued pleading and remon- strance of the patients may corrupt the best of attendants, especially if accompanied by threats of discharge at a future day. It is, therefore, necessary to place these patients under the surveillance of trained nurses from the start or to remove them to an institution where they will be under control. In the chronic stage the drugs which are of greatest service are strychnine and arsenic. Strychnine may be given in doses of ^\ to ^ gr. three times daily, and it is well to combine it with phosphoric acid and the syrup of the hypophosphites, or with the glycerophos- phates of lime and soda. Arsenious acid may be used in tablets or pills containing gV ^*^ w S^' three times daily, or in Fowler's solution, three to five drops, three times daily. Hypodermic injections of the cacodylate of soda are of use. The use of iron will be indicated in the majority of cases where there is attendant anaemia. In alcoholic cases both arsenic and strychnine may increase the mental irritability, but should be continued unless this becomes too great. I have seen benefit from both of these drugs, and think it well to employ then alternately, using each for about two weeks at a time. The glycero-phosphate of soda dissolved in water is of much service in the chronic stage. The dose is 15 grains three times daily. The remedies used in the chronic stage have two objects : one is to increase the rate of repair in the nerves, the other is to keep the nutri- tion of the muscles as good as possible. While the drugs mentioned probably meet the first indication, there are other remedies which meet both. These are massage, warm baths, and electricity. The proper manipulation of the limb increases the circulation in it. The increase of circulation brings fresh supplies of material to the nerve which is undergoing repair ; it also aids the nutrition of the muscle, which would otherwise be decidedly affected by the sluggish flow of venous blood, due to the lack of functional activity. As soon, therefore, as the active progress of the disease is checked and the muscular tender- ness has sufficiently subsided to allow the limbs to be rubbed, this remedy should be employed daily. Allusion has already been made to baths and douches in the early stage, for the purpose of quieting sensory symptoms. In the chronic stage the object is a different one. Like massage, warm baths, packs, and douches stimulate the circulation and aid the nutrition and repara- tive processes in progress. Hence they are to be used daily ; and if the warm bath or pack be given at night it will secure not only a local action, but produce a general sedative effect, insuring quiet rest. In multiple neuritis, as well as in many other nervous affections not due to anaemia, a warm bath or pack at night or a warm douche to the neck and spine, is far preferable to and more efficacious than the majority of hypnotic drugs in causing a good night's sleep. 14 210 MULTIPLE NEUlilTIS. The last agent to be mentioned is electricity. It is, however, of some importance, but it is necessary to know the object sought in its application. There is first the object of increasing 'the progress of nerve regeneration. This is said to be attained by the application of a constant galvanic current to the degenerated nerve, passing the cur- rent through the nerve in either direction, or in both alternately. A mild current should be employed, its strength being measured by a galvanometer. The strength of the current will depend (a) on the size of the sponges placed upon the skin, (6) on the pressure upon the sponges, (c) on the resistance of the skin, (d^ on the number of cells of the battery used. If the sponges are two inches in diameter, six milliamperes are enough, if they are three by five inches in measurement, twenty mil- liamperes should not be exceeded. If no galvanometer is used the strength of the current employed is uncertain ; but it is to be remem- bered that the current grows stronger the longer it passes, since the skin resistance is gradually overcome ; and, therefore, if the strength is measured in cells, the number of cells used should be decreased gradu- ally during the application. As so few practitioners use a galvanome- ter, it may be well to state that with large sponges — i. e., three by five inches — wet with warm water, a freshly filled bichromate of pot- ash battery will give nearly one milliampere of strength for every cell used during the first three minutes, provided the sponges be put on any part of the body except the soles of the feet or the palms of the hands, and pressed firmly upon the skin. After the first three min- utes the body resistance decreases, so that when twenty cells are used to start with, one should be cut oif every half-minute until the num- ber is reduced one-third. The duration of the application should be about ten minutes to each limb. During this time the distal sponge should be passed over various parts, so as to include all the nerve branches in the current ; the central sponge should be put over the nerve trunk high up on the limb. The current should be begun and stopped gradually, and never suddenly broken. Applications may be made daily. The second object to be obtained is to reestablish the conduction of impulses in the regenerated nerve. This is secured by the method just described. It may be attained by the use of faradism, the interrupted secondary current being sent along the nerves by placing one pole over the nerve trunk and passing the other over the skin of the limb. The strength used should be just sufficient to be felt distinctly through the palms of the operator's hands. The third object sought is to maintain the nutrition and function of the muscles by exercising them, and thus to preserve their normal irritability. This cannot be done by a far- adic current as long as they do not contract to it. The galvanic current must, therefore, be employed. But now it is not a steady cur- rent which is needed, for this does not cause any motion. It is only when the steady current is suddenly broken and renewed that the con- traction occurs.. Hence, place one pole over the trunk of the nerve TREATMENT. 211 and the other upon the muscle, and with an interrupting electrode make and break the current at the pole which is on the muscle. The pole which ■^'produces a 'contraction with the least current possible is the one to be applied to the muscle. This is in the reaction of degen- eration, the positive ; in normal conditions, the negative pole. Each muscle should be exercised for three or four minutes every other day. When electrical treatment is thus employed very marked improvement is observed, which can be measured accurately if a galvanometer is used, since every week will show a change of the strength of current needed to produce muscular contractions toward the normal. The treatment must be kept up, in the chronic stage, until recovery is complete. If contractures have occurred in the paralyzed limbs, persistent massage may overcome them. If it does not, they are to be treated on general surgical principles. When the posterior tibial muscles are contractured, the patients cannot stand because the heels cannot be placed on the floor. In this condition shoes with very high rubber heels, and either corset-like ankles or braces to support the ankle, may be fitted, and thus the patient may be enabled to walk with a little help long before he can stand in his bare feet. Sometimes division of the Achilles tendon is necessary to restore the power of walking. CHAPTER IX. THE TOXIC FORMS OF MULTIPLE NEURITIS. Alcoholic Neuritis. Multiple Neuritis from Poisoning by Illuminating Gas. Multiple Neuritis from Poisoning by Sulphide of Copper. Multiple Neuritis from Poisoning by the Coal-tar Products. Arsenical Neuritis. Multiple Neuritis Due to Poisoning by Lead. Multiple Neuritis Due to Poisoning by Phosphorus, Mercury, Silver, and Copper. ALCOHOLIC NEURITIS. Historical. — Alcoholic neuritis is the most common of all the toxic forms of multiple neuritis. The credit of the discovery of alcoholic paralysis must be awarded to James Jackson, of Boston, who, in 1822, gave a most accurate description of the disease and ascribed it to the use of ardent spirits.^ Magnus Huss, in 1852, published an extensive work on chronic alcoholism, in which he gave a very complete account of the nervous symptoms produced by the abuse of liquor, dividing the cases into paralytic, ansesthetic, convulsive, epileptic, and hypersesthetic forms. But he ascribed all the symptoms to lesions of the central nervous system. In 1855 Duchenne de Boulogne, in his epoch-mak- ing book, Electrisation Localisee, recorded many cases which we now recognize as cases of neuritis; but he, too, supposed them to be of spinal origin. Lancereaux added further to the knowledge of alco- holic paralysis in an article on alcoholism in the Dictionnaire Ency- dopcedique des Sciences Medicates in 1864, and in the same year DumesniP published the first case in which a lesion was found in the peripheral nerves. But ten years then elapsed before his observations were confirmed by Eichhorst,^ and it required further evidence fur- nished by JoflFroy,* by Leyden,^ and by others to establish the relation between alcoholic paralysis and neuritis. It is to Lancereaux,^ of Paris, to Moeli/ of Berlin, to Dreschfeld,^ of Manchester, England, to Henry Hun,^ of Albany, N. Y., and to Bernhardt,^'' of Berlin, that we owe our knowledge of the pathology and symptomatology of alcoholic multiple neuritis, though other and subsequent writers have added ^New England Journal of Medicine and Surgery, 1822, vol. xi., p. 351, "On a Peculiar Disease Resulting from the Use of Ardent Spirits." ''Gaz. Heb. de Paris, 1864, p. 203, and 1866, No. 4. »Virchow's Archiv, 1876, Bd. 69, p. 205. *Arch. de phys. norm, et path., 1879, p. 172. ^Charite Annalen, 1880, Zeitschr. f. klin. Med., 1880. *De la Paralysie Alcoolique, Gaz. Heb. de Paris, 1881, p. 120. ' Brain, Nos. 26 and 32. 8Moeli. Charitd Annalen, 1884. ® American Journal of the Medical Sciences, April, 1885. "Zeitschr. f. klin. Med., 1886. 212 PLATE XVI m te liMM Longitudinal Section of Tibial Nerve in a Case of Alcoholic Neuritis. (Osmic acid and carmine stain.) Tlie degenerated nerve fibres are stained black ; the thickened interstitial connective tissue is stained red. (Kla.tau, Spec:. Pathol, u. Therap., Nothnagel, Bd. xi., Taf. vii.') ALCOHOLIC NEURITI8. 213 valuable facts and have made the picture of the condition complete. James Rossf Brissaud,^' Pal/ and Growers* may be mentioned as hav- ing made interesting additions to the general knowledge of the affec- tion, and as having clearly presented its differential diagnosis from other diseases. The most complete monograph on the subject is by Remak and Flatau in Nothnagel's System of Medieiiie, 1900. Pathology. — All forms of neuritis have been found in alcoholic cases. In the majority of cases a parenchymatous neuritis with simple degeneration and atrophy is the only lesion in the nerves. (Plate XII.) In other cases a diffuse inflammation is present, with an in- crease of connective tissue in the endoneurium and perineurium, and a marked congestion of the bloodvessels, as well as a degeneration of the nerve fibres. All the characteristic changes already described as oc- curring in local neuritis are present in alcoholic cases. But it is not possible to distinguish clinically between cases in which the lesion is a simple degeneration, and cases in which there is a diffuse inflammation. The lesion is more marked in the finer branches of the nerves, in the skin and muscles, and becomes less intense the higher up the nerve is examined. In the plexuses no change may be found, when at the same time few normal fibres can be discovered below the wrists and ankles. Certain nerves seem particularly susceptible to the influence of alcohol. The degeneration begins in the peronei, the tibials, then in the radial, median, and ulnar nerves, and is more intense in these than elsewhere. (See Plate XVI.) The sciatic and crural and the musculocutaneous and circumflex are but slightly affected. Lesions have been found in the phrenic, in the pneumogastric, and in the optic nerves. Normal fibres are found here and there in the nerves which are severely affected. A thickening of the bloodvessels in the nerves has been discovered in some cases, with a small-cell infiltration of the walls, and occasional rupture, with capillary hemorrhages. Recent observation establishes the fact that while the principal changes in alcoholic paralysis are found in the peripheral branches of the nerves in all cases, yet in some cases changes in the cells of the central nervous system are present. These have been discovered in the cells of the anterior horns of the spinal cord and in the posterior spinal ganglia.^ It is evident, therefore, that while the poison circulat- ing in the blood produces a destructive process in the delicate fila- ments of nerve fibres in the periphery, yet the central nervous system is by no means free from its action. The cells of the spinal cord are less affected by the poisoning than the more highly organized and de- veloped cells of the brain. The changes in the cord cells consist of a change in the Nissl granules, which become finely granular, and lose, finally, their power of absorbing anilin dye. This change is more marked about the nucleus and in the centre of the cell body which has '.James Eoss and Judson Bury. "On Peripheral Neuritis," 1889. ^ Briasaud. Les Paralysies Toxiques, Paris, 1890. 8Pal. "Ucber Neuritis," Berlin, 1892. *Gower8. Text-hook of Nervous Diseases, 1892, 2d ed. ^Larkin and .Jell i fie. N. Y. Med. Record, .July 8, 1899. 214 TEE TOXIC FORMS OF MULTIPLE NEURITIS. a pale appearance. (See Plate II, g.) There is often a deposit of pig- ment in the cells. As these changes, however, have been found in all the cells of the cord, even in those connected with muscles which were not paralyzed, and as they resemble closely the changes known to occur after division of a nerve, they are not to be regarded as the cause of alcoholic paralysis. Heilbronner ^ has found an ascending degeneration in the posterior columns of the spinal cord in an advanced case of alcoholic neuritis, and is inclined to trace it to extensive degeneration in the posterior spinal neurones, but this is certainly an exceptional case. Berkeley, Andriesen and Van Gieson have demonstrated a uniform pathological condition produced in the large cortical cells of the brain in chronic alcoholism, and these changes have been frequently found in cases of alcoholic neuritis. The dendrites of these cells lose the fine granules which cover them, and become smooth and bare. (See Fig. 6.) Then the dendrites become broken and disintegrated. The cells become swollen, vacuolated, degenerated, and finally atrophied, and their axones share in the destruction. So long as the lesion is limited to the shedding of the granules it is not irreparable, and re- generation may occur. In the cases of alcoholic neuritis which present psychical symptoms, there is every reason to suppose that these patho- logical changes are present in the brain, and the degree of impairment of mental capacity is directly proportionate to their intensity. A gen- eral atrophy of the muscular fibres is always present in the paralyzed muscle, with a loss of their striation, a fatty degeneration and a dis- integration of the muscular substance, and an increase of the connec- tive tissue. The muscular fibres show great irregularity of size, and present both parenchymatous and interstitial changes with the deposit of fat. Etiology. — Males are more commonly affected than females by al- coholic paralysis. Thus in 250 cases which I have collected or ob- served but 89 were in women. When, however, the much greater prevalence of drinking among men is considered, it becomes evident that the disease is relatively far more frequent among women than among men. And this fact has been emphasized by all writers. In men the poison seems more liable to manifest itself by acute cerebral symptoms than by those of disease of the peripheral nerves. Neuritis is especially frequent among those persons in the higher classes whose nervous organism is highly developed, and who lead a comparatively inactive life. It seems not at all improbable that sedentary habits predispose an alcoholic drinker to this disease, and hence active work- ers, male or female, though taking an equally large amount of liquor as the luxurious drinker, escape. All alcoholic drinks are not equally prone to produce paralysis. It is the spirituous liquors — brandy, whiskey, gin, and rum and the liqueurs, absinthe, vermouthe, etc. — which are particularly dangerous. But many cases are known to be due to excessive beer drinking. I * Heilbronner. Monatsschrift f. Psych, u. Neurologie, 1898, p. 246. AlCOHOLIG NEURtTiS. . ^l5 see every year at the clinic cases in drivers of beer wagons who con- sume forty gr fifty glasses of beer daily. I have even seen a case in a child of three years who had been given beer several times a day for some weeks. I have known cases in women to be due to taking col- ogne ; and in Ireland, where ether is used as a drink, many cases are due to this cause. The fumes of alcohol are capable of causing the disease in a mild form. It is steady drinking of small amounts throughout the day, rather than excessive occasional indulgence, which is more likely to cause neuritis. The liability to neuritis from medici- nal uses of alcohol should never be forgotten in those cases where it is used as a heart stimulant. I have seen many cases developing after typhoid fever and pneumonia where it seems very probable that the actual cause was the free use of alcoholic stimulants given to support the heart, little food being given. All ages are liable to be affected, but the majority of cases are between thirty-five and fifty years of age. Symptoms. — It is only after alcohol has been consumed in large amounts and for a considerable length of time that neuritis develops. Its onset, though often apparently gradual, is usually sudden. For months the patient has suffered from chronic gastritis, insomnia, general neuralgic pains, or severe pains in the joints or limbs, and from alco- holic tremor and a certain feebleness in movement, when all at once his legs give way beneath him and after the sudden fall he finds him- self unable to rise. Thus a patient of mine, after a year of such pre- monitory symptoms, was seized with paralysis quite unexpectedly when getting out of bed in the night. The onset in other cases is preceded by symptoms of disturbance in the heart and lungs. The patient has had palpitation and dyspnoea ; has had swelling of the feet, undue ex- haustion on exertion and possibly a bronchial cough ; and when ex- amined is found to have a feeble heart, dilated and flabby, with faint sounds or distinct mitral regurgitant murmur, and the signs of chronic pulmonary congestion. Then, after a period of unusual indulgence in stimulants, paralysis suddenly develops with all the sensory distur- bances of a neuritis. When the paralysis begins it soon becomes complete in the feet and legs to the knee, and may advance up to the thigh, involving the ex- tensors of the legs. It next attacks the hands and forearms, and while in all extremities it is greater in the extensors than in the flexors, in some cases both groups of muscles become entirely helpless. The paralyzed muscles are flabby, and soon become atrophied, they have no excitability to mechanical irritation, and the tendon reflexes are lost. They fail to react to a faradic current in the majority of cases, though occasionally a very strong current may produce a response. When galvanism is applied the reaction of degeneration is found to be present. It is often found that strong galvanic currents have to be used to produce any contraction at all. The paralysis of the muscles may advance rapidly in severe cases, involving the motor cranial nerves, the muscles of the trunk, and, lastly, the diaphragm, thus caus- ing death. More frequently, however, it is arrested when only the ^16 TEE TOXIC FORMS OF MVLTtPLiJ NBVRlTt^. distal parts of the extremities are involved, then, after a stationary- period, which varies from a few weeks to several months, it gradually subsides until recovery is complete. The position assumed by the paralyzed limbs has been thought to be almost characteristic. Dropped-wrist and dropped-foot appear early, due to the paralysis of the extensors, and further deformities of hands and feet follow. When the feet are dropped the first joint of the toes may be hyperextended though the second is flexed, but as time goes on this hyperextension gives place to flexion and then the entire foot is paralyzed and hangs down, the natural concavity of the sole being increased. The weight of the bedclothes may increase the dropping of the feet and flexion of the toes. Later the muscles of the sole and of the calf become permanently shortened and the sole can no longer be placed on the floor. When the patient is able to begin to stand, all the weight comes on the toes, which often turn under. Then the heel of the shoe has to be built up in order to form any sup- port. The deformity of the hands is also different in different stages of the disease. At first there is dropped-wrist, the fingers hanging limp. Later, a claw-hand appears, the first phalanges being hyperex- tended, the second and third flexed. The thumb is also hyperex- tended, and, the metacarpal bone being drawn backward but adducted, the thumb can no longer be apposed to the fingers. (See Fig. 104.) After a time the hyperextension gives way, the intrinsic muscles of the hand being paralyzed and the fingers are straight, adducted, and im- movable, being fixed by contractured, atrophied muscles. In this state ankylosis of the smaller joints often develops, and the use of the hands is very slowly regained after painful massage and passive motions. I have seen bilateral facial paralysis of the peripheral type in a case of alcoholic paralysis ; and several cases in which the ocular muscles were paralyzed. Irregular, rapid pulse, due to an affection of the pneumogastric, is a not uncommon occurrence in alcoholic cases, and is a dangerous symptom. The patient suffers far more from the disturbance of sensation than from the paralysis. In the description of Jackson, the pains were graphically portrayed. They are the cause of terrible agony" are suf- ficient to produce insomnia, and wear seriously upon the endurance of the sufferer. In addition to pain, hyperaesthesia, both of the skin and muscles, is always observed. It is usually quite extensive in the legs, though in cases of poisoning by absinthe it has been limited to the soles of the feet. The muscles, as well as the skin, are sensitive to handling and to pressure, and marked tenderness in the course of the nerves is con- stantly elicited by examination. In many cases soon after the onset, the patients cannot bear to be touched or moved, though perfectly un- able to help themselves. Cramps in the calves or in the extensor sur- face of the thighs are often complained of and give rise to much distress. Parsesthesise are always complained of. Numbness, tingling, and ALCOHOLIC NEURITIS. 217 formication are frequent. In one of my patients the sensation was as if heavy brsteelets were 'around the wrists, and as if very tight drawers were on the legs. At other times she felt as if the limbs were swollen, and as if the skin was about to burst. One patient described the sen- sation as being like iron stockings on the legs. Another insisted that there were needles under the skin of the hands. Such sensations may cease as the case increases in severity, and give place to a total lack of sensation in the parts. They return, however, with advancing recov- ery, and are among the last symptoms to disappear. Abolition of tactile sense, and to some degree of muscular sense, is the rule after the paralysis has developed. Temperature sense and the perception of pain are never wholly lost, but may be delayed in trans- mission. Thus in multiple neuritis the phenomena of dissociated sen- sation may be found, but in the reverse sense to that observed in syringomyelia. The anaesthesia may be limited to irregular areas, and may be found in the cutaneous distribution of one nerve only, but is usually found over the entire distal part of the paralyzed limb. Usually the cutaneous reflexes are preserved. The loss of muscular sense is, in some cases, so marked a symptom, and one of such early occurrence, that Dreschfeld distinguished a class of cases which he terms ataxic rather than paralytic. And this dis- tinction is perfectly justifiable, for in many cases it is the incoordina- tion which first attracts the attention of both the patient and the physician. It is this class of alcoholic cases which may be mistaken for locomotor ataxia, and which have been named by French writers pseudo-tabes alcoolique,^ or neurotabes peripherica by the Germans. But ataxia is not exclusively limited to this class of cases. It may be present in some degree in cases of paralysis, and during recovery from paralysis the deficiency in coordinating power may become evident, and appear to retard the progress of the case. Nor are the cases of ataxia, on the other hand, free from paralysis. The gait in neuritis is quite characteristic. By contrasting the walk of a true ataxic patient with that of an ataxic alcoholic patient, WestphaP and Charcot^ have each pointed out several points of difference. The tabetic patient throws the foot forward with undue violence, the toe lifted high in the air, and brings first the heel down forcibly and then the entire foot. The alcoholic, however, has some weakness in the muscles of extension and cannot raise the toe. He, therefore, lifts the foot high in order to step over the hanging toe and not to trip on it, but the motion is made without undue force. He then throws the foot forward in order to throw the toes up and get them out of the way as he brings the foot down to the floor. The motion is awkward, and has an appearance of one stepping over high obstacles, but it is a voluntary attempt to remedy a deficient power — not the involuntary awkwardness of a man unable to manage strong ^Dejerine. Arch, de phys., 1884. See also Dreschfeld, Brain, Nos. 26 and 32. ^ Westphal. Ueber eino bei chronischen Alcoholisten beobachtete Form von Geh- storungen, Charitd Annalen, 1879. * Charcot. Lejons, Le Progress M^dicale, 1886. 218 TEE TOXIC FOliMS OF MULTIPLE NEVRItIS. muscles. There may be in both patients some tottering and swaying when standing witli '^e eyes closed; this so-called Romberg symptom is common to both locomotor ataxia and alcoholic neuritis. The vasomotor symptoms in alcoholic neuritis are very variable. Sometimes they precede the attack of paralysis for some months. There are irregularities of circulation in the extremities causing cold hands and feet, or burning red hands and feet, or undue sweating. When the paralysis is developed the color of the extremities is pale ; they are cool and often wet with perspiration or occasionally livid and hot. If allowed to hang down both hands and feet get purple and swell. Ross saw one case in which Raynaud's disease developed. As the disease goes on, glossy skin appears and then the dermal tissues become atrophied and the skin seems too tight for the fingers and toes. It is often pigmented and scaly, and, when the tenderness is so great as to prevent washing, dark brown, scaly masses may collect on the fingers and hands and feet. The growth of the nails is always affected. They become ridged transversely, are thick and brittle, and too tender to be cut. As recovery begins new, thin, healthy nail ap- pears above the thick ridge. (See Fig. 104.) The special senses are occasionally affected in cases of alcoholic paralysis. Amblyopia has been observed, and also defective vision from central scotoma. The field of color vision is often contracted even when sight is preserved. There may develop a true optic neuritis, evident to the ophthalmoscope, and this may go on to optic nerve atrophy.^ Inequality of the pupils is frequently seen, as is also a moderate contraction of the pupil. All these eye symptoms, occurring as they may in a case of the ataxic variety, make a differential diag- nosis from locomotor ataxia difficult. The Argyll-Robertson pupil (which contracts in accommodation, but not to light) has rarely been seen in alcoholic cases, while it is an early symptom of tabes. One writer affirms that a condition of the pupil just the reverse of the Argyll- Robertson pupil is present in alcoholic neuritis, a pupil which reacts to light but fails to react in accommodation. I have been unable to confirm this statement. One important feature of alcoholic paralysis remains to be noticed, viz., the mental symptoms. These are hardly ever wanting in severe cases. I called attention to these symptoms in 1887.^ They have been most fully described by Korsakow,^ Soukhanoff * and lately by Jelliffe.^ The name Korsakow's psychosis has been given to these symptoms, but I prefer the term pol}Tieuritic psychosis, or chronic alcoholic delirium. There is at first excitement rising to the degree of active delirium, with illusions and hallucinations of the various senses ; there is insomnia, which soon exhausts the patient if it is not remedied ; there is a loss of memory, especially of recent occurrences ; and a lack of power of attention or concentration which prevents intelligent conversation, and a mild dementia. ^Brissaud. Les Paralysies Toxiques, p. 31, Paris, 1886. 'Middleton Goldsmith Lectures, 1887. Medical News, Mrch, 1887. 3 Arch. f. Psych., 1892, Bd. xxi., 669. "Eaev. de Med., Mav, 1897. ^N. Y. Med." Jour., Oct. 24, 1908. ALCOHOLIC NEURITIS. . 219 The patients are usually greatly depressed aud alarmed at their con- dition, and y^quire hourly reassurance of the probability of recovery, forgetting what has been told them almost as soon as it has been uttered. The depression may lead to crying for hours at a time, but the self-reproaches common in true melancholia are never heard in this state. A marked confusion of ideas is often noticed, the patient being indiiFerent and incapable of connected conversation. The indifference to bodily wants may be so great as to lead to uncleanliness, and since paralysis of the sphinctei- is the rare exception, incontinence, if it occurs, is to be ascribed to the mental state. It is useless to attempt to get any reliable history of their illness from these patients. Their statements are unintelligible or unreliable. They will relate occurrences as having happened recently, with much elaboration of detail, when as a fact the story is entirely a product of their imagination. Thus one patient of my own who had been confined to bed for many days, told me one afternoon that she had been out to see an eminent gynecologist during the morning ; had gone to his office and waited for him several hours ; had seen other patients there, and finally had been told by the doctor's brother that he would not return in time to see her, so she had come home again. And this was all related in apparent good faith, so that I have no doubt that she be- lieved that what she said had occurred. Several patients have de- clared that they had been out to walk or to drive, when they had been unable to leave the bed for some weeks. One patient told me a dif- ferent history of her case every day for a week, and it was only from her friends that the correct account was obtained. It is evident that the statements of these patients cannot be accepted regarding any- thing, especially as to their own history. The defects of memory are permanent and after recovery patients have no clear recollection of their illness. This mental state may last for two or three months, but after a time the mind becomes clearer, the power of attention returns, memory improves, the depression is less constant, and mental health is regained. Even in cases lasting four months I have seen permanent recoveries now lasting ten years. Occasionally a permanent dementia develops. While Korsakow's psychosis is usually due to alcohol, it may appear as a result of long-continued malnutrition, and anaemia in cases of gastric origin, and after childbirth attended by severe hem- rhages.^ Patients suffering from this psychosis are legally insane for the time until they recover. , The course of alcoholic neuritis is quite uniform. After a sudden onset the symptoms advance rapidly to a high degree, which is reached in a week or two from the beginning of the paralysis or ataxia. Then they may increase further, and cause death by respiratory paralysis. Usually they remain stationary for a time, which may be several months in severe types, and then gradually subside, the entire duration being from four months to two years. Individual muscles regain their power, tone, firmness, and electrical reaction slowly, and during recov- ery the tingling and numbness in the hands and feet may be severe. * Serbsky. Arbeit, a. d. Wiener, Neur. Institut, xv, 1907. 220 THE TOXIC FORMS OF MULTIPLE NEURITIS. In a few cases the muscles become contractured, and permanent defor- mities develop, only to be overcome by long-continued massage or by operative measures. These deformities cause great difficulty in regain- ing the power of walking. The feet cannot be placed flat on the ground and the knees are partly flexed, so that it is often months before the patients can stand, and even when by the aid of high heels on the shoes, supporting braces to the ankles, and crutches, the upright position can be attained, it is with difficulty that the balance can be preserved. When the fact is considered that those who recover rapidly rarely fail to resort again at once to the use of stimulants, and thus expose them- selves to the danger of a relapse, the ultimate fate of the chronic cases is hardly more serious than that of those who get well quickly. Treatment. — The treatment of alcoholic neuritis differs in no re- spect from that already described on page 207. The following cases illustrate the various forms of the disease : A young man who had drank whiskey eight or ten times a day for some months without ever being drunk, began to feel a numbness in the legs and then in the hands, which was followed in three weeks by some undue fatigue on walking ; occasional sharp pains in the legs, and a week later by weakness in the extensors of the feet and un- steadiness of gait. He went to the Hot Springs of Virginia, was treated for rheumatism, as he suiFered while there from pains in the legs, but was finally told after two weeks that he had locomotor ataxia and was advised to go home. When I saw him on his return his sub- jective numbness was attended by a slight anaesthesia of the fingers and toes. His calves and forearms were very tender to pressure and the extensors were decidedly weak, so that he could not raise his toes from the floor when standing, but he had no drop-foot or drop-wrist. He was quite ataxic in his gait, swayed with eyes closed, but had no ataxia of the hands. His knee-jerks were absent, but he had no dis- turbance of his bladder, and his pupils reacted to light. His pulse was rapid and feeble, and he was subject to faint feelings and occa- sional sudden fears. He was put to bed, deprived of alcoholic drinks, fed frequently and well, and given two warm baths daily and general massage. Cod-liver oil and glycero-phosphate of soda, 15 grains, three times a day, were given daily, with strychnine, gi^p, three times a day, four days in each week. Within four weeks his symptoms had improved so much that he was allowed to get up. At the end of nine weeks his paralysis had entirely disappeared and he no longer had pains or anaesthesia or any ataxia ; and fourteen weeks from the date of his return from Virginia he was perfectly well, except for a very slight numbness in the sole of one foot. The knee-jerks returned in the tenth week. The entire duration of his illness was nineteen weeks. He has had no recurrence. A man, aged thirty-six years, a hard drinker, after an attack of gastritis became paralyzed suddenly in both legs. A few days after this both hands became useless, so that he was completely helpless. On admission to the hospital, seven weeks after the onset, he com- plained of pain in all four extremities, and of tenderness in the ALCOHOLIC NEURITIS. 221 muscles and joints when these were handled. The upper extremities were almost totally paralyzed below the elbows, the only motion pos- sible being a slight flexion of the fingers. There was drop-wrist on both sides. The lower extremities were totally paralyzed below the knee, and the feet hung down motionless. The muscles of the thighs were also aifected, for although he could pull his legs up in bed, it was with great difficulty that they could be straightened out. All the paralyzed muscles were atrophied,' and showed a reaction of degenera- tion. Tactile sense was considerably impaired in areas corresponding to the region covered by long stockings and gloves, but the senses of pain and temperature and muscular sense seemed to be natural. The knee-jerks were lost. When an attempt was made to have him stand, the feet were pushed out and the knees doubled under him at once. Fibrillary twitchings were very noticeable in the atrophied muscles, and a marked tremor of the tongue was seen. The facial and ocular muscles were not affected, and the special senses were normal. There was no incontinence of urine, and no symptoms of thoracic or visceral disease. His mind was much impaired. He talked in a rambling manner, laughed much, and could not fix his attention, and his memory was so poor that no reliance could be put in his statements. Under treatment by complete cutting off of all stimulants and by iodide of potash he began at once to improve. In two months from the time of admission he was able to walk, and two months later he was dis- charged perfectly well. In this case the duration was six months. A young woman, whose father died of alcoholism, had drank champagne and brandy very freely for two years during a period of mental distress. She was then (August) suddenly seized with paralysis attended by severe pain in arms and legs. She was at the time in Europe, but was brought home, being carried to and from the steamer. When I saw her in November she had a typical alcoholic neuritis, with great tenderness along the nerves in the calves and on the front of the thighs, and drop-feet; cold, clammy hands covered with scales and pigmented, and anaesthesia in the glove and stocking areas. She was suffering intensely from such pains and hypersesthesia in her arms and legs that she could not bear the weight of the bed- clothes. She lay in bed with knees flexed and feet extended; the knee-jerks were absent. Her pulse was feeble and rapid and she was in a state of great mental distress at her condition. She required anodynes to relieve the pain. Under food, cessation of alcohol, which was extremely difficult to secure, as it was the only thing which relieved her pains, and general tonics, especially strychnine, she improved rapidly. In February she was able to walk with the help of high heels and corset shoes, and in April all her symptoms had dis- appeared excepting numb sensations and occasional pains in her feet. Her knee-jerks had returned. She soon resumed drinking in spite of warning, and six months after her recovery she was again paralyzed ; this time the hands as well as the feet being helpless, and drop- wrist developing at the same time as drop-foot. I saw her in December in 222 THE TOXIC FORMS OF MULTIPLE NEURITIS. the second month of this attack. She had a pulse of 150, irregular and intermittent, and was cyanotic. Her suffering from pain was extreme, and contractures existed in all the extremities. Glossy skin was present on legs and arms, and the extremities were hot and perspiring. Her mind was much affected. She had hallucinations of touch and sight, affirmed that her hands and feet were filled with needles, which she spent hours in drawing out of the skin, filling an empty box which she showed me as full of them; declared that scissors and knives were still buried in her flesh and cutting her. She had no memory of the events of the past two months. This con- dition continued for three weeks, her life being despaired of on account of her weak heart. During this time she told me daily some new story of having been to balls and receptions, and to drive in the park. Then (January) her circulation improved, her pains became less severe, she became less restless and was able to sleep, and her mind gradually became clearer. At the end of April she could use her hands a little and had lost the glossy skin and her mind was clear. But it was not until October that she was able to walk, and then she had to wear braces. In January she had recovered. MULTIPLE NEURITIS DUE TO POISONING BY CARBON-MONOXIDE OR ILLUMINATING GAS. A certain number of persons are brought into the hospitals every year suffering from acute poisoning by illuminating gas. Either acci- dentally or with suicidal intention they have turned on the gas in their sleeping rooms, and have been found after several hours in a state of coma. If they die softening of both corpora striata in the brain are found. (See Fig. 223, page 482.) If they survive this condition of acute poisoning they sometimes develop symptoms of multiple neuritis within a week. These symptons consist of tingling and numbness of the extremities, more acutely felt in the distal parts and attended by slight anaesthesia, also a condition of weakness in the muscles which does not go on to a total paralysis but makes them feeble. There are rarely any atrophies or changes in the electrical contractility in the affected muscles, and the sensory symptoms are, as a rule, much more marked than the motor symptoms. For many weeks, or even months, these patients suffer from parsesthesise which are excessively disagree- able. In a few cases I have seen a slight ataxia of movement, both in walking and in the use of the hands. The symptoms gradually subside under general tonic treatment, and the patient finally recovers. The following case is of interest, as it appears to be a case of multiple neuritis following poisoning by natural gas : The patient was a healthy man of good habits who had been very much exposed to the fumes of natural gas in his home in Peru, Ind. He gradually developed symptoms of tingling and numbness in the legs, which soon after extended to the hands and increased in extent until the legs and thighs and the hands and forearms were the constant s^at NEURITIS DUE TO POISONING BY SULPHIDE OF CARBON. 223 of disagreeable numbness. After suffering in this manner for three months he began to notice an uncertainty of gait, which soon became a marked araxia. There were at no time any pains, paralysis, atrophy, fibrillary twitchings, or disturbances of the bladder and rectum. His knee-jerks were normal and his pupils reacted perfectly to light, but his gait was distinctly ataxic ; he swayed with his eyes closed and had a marked condition of anaesthesia to touch, temperature, and pain as high as the middle of the thighs and elbows, this anaesthesia being more intense in the hands and feet than in the upper parts. He had had during the entire illness some gastro-intestinal catarrh with at- tacks of diarrhoea, but careful investigation failed to reveal any pos- sible source of poisoning by lead, arsenic, mercury, or drugs. It was discovered, however, that in both his house and office natural gas was burned for heating and lighting purposes, and that the fixtures were imperfect, resulting in incomplete combustion and leakage so that it was necessary to make special provision for ventilation, and both house and office were often filled with foul and nauseating gases when the draughts through the ventilators were imperfect. On removal from his home to the Clifton Springs Sanitarium, he rapidly improved under general treatment and was able to return home after three months quite recovered. A year later, however, also in the spring, after the con- finement of the winter, the symptoms recurred with equal intensity and remained for eight weeks until he again left home, when, after four weeks, they subsided. In the absence of any other cause (and great care was taken to ascertain some cause for the condition) the neuritis was ascribed to the inhalation of natural gas. A number of cases of neuritis in single nerves have been reported by foreign observers occurring after poisoning by illuminating gas. It is to be remembered that in a state of long-continued coma, pressure upon nerve trunks may occur of sufficient duration to cause traumatic neuritis which has not, under such circumstances, any relation to the cause of the coma. MULTIPLE NEURITIS DUE TO POISONING BY SULPHIDE OF CARBON. Multiple neuritis may develop in workmen who are exposed to poisoning from sulphide of carbon. This substance is present in rubber and the disease has been observed in those who work in rubber fac- tories where ventilation is neglected. The fumes or the powdered dust containing sulphide of carbon are inhaled or get into the mouth and are taken into the stomach. Guillain ^ has reported a case in a man who was employed in manufacturing toy balloons. These balloons are plunged in a bath containing bisulphide of carbon. This patient developed total paralysis without sensory symptoms. The effect of the poison is slowly developed and seems to be widespread.^ In addi- ^ Revue de Neurol., February, 1904. ^ R. Landenhoimer. Die SchwefelkohlenstofT Vergiftungen dcr Gummiarbeiter, Leipzig, 1899, where full references to all published esuies, about forty in number, can be found. 224 TEE TOXIC F0R3IS OF MULTIPLE NEURITIS. tion to a state of general ansemia, with disturbances of digestion, the patients suffer from headache, inability to fix the attention and to re- member, and from many symptoms of functional nervous disorder, which may awaken a suspicion of hysteria ; but as time goes on the symptoms of multiple neuritis appear, and these may be of the paralytic or of the ataxic type, and pursue a course not unlike that observed in alcoholic or arsenical cases. The development of cerebral and of spinal symptoms in some of the recorded cases makes it probable that the poison has an effect upon the central nervous system, and that the lesion is not in all cases limited to the peripheral nerves. Autopsies are wanting to establish an exact pathology. MULTIPLE NEURITIS DUE TO POISONING BY THE COAL-TAR PRODUCTS, ESPECIALLY SULPHONAL AND TRIONAL. Since the general use of the coal-tar products in the treatment of disease, especially of painful affections, and the free use of sulphoual and trional in the treatment of insomnia, a few cases of multiple neu- ritis have been reported, occurring in patients who had abused these drugs. And their chemical resemblance to alcohol lends a certain amount of support to the hypothesis that they are capable of producing multiple neuritis. Remak has reported a case as having occurred subsequent to the use of antipyrine, and Stewart Hart ^ has recorded a case, which I saw with him, probably produced by the constant use of trional. A patient of my own, who took 120 grains of sulphoual in four hours by mistake, slept two days, was markedly cyanotic, with pulse of 120 and shallow respiration, and, for three weeks afterward, suffered from parsesthesise, weakness, and ataxia in the arms below the elbows and in the legs below the knees. The symptoms gradually sub- sided. They resembled so closely those which I had seen in hospital cases of illuminating-gas poisoning, that it seemed justifiable to ascribe them to a mild condition of neuritis. Multiple neuritis has been said by Ross to have been caused by poisoning with roburite (dinitrobenzol) used by miners, and by poison- ing with anilin oil, used by dye manufacturers. In the cases recorded the symptoms in both forms of poisoning were those of multiple periph- eral neuritis, together with general constitutional effects — anaemia, cyanosis, and a blue discoloration of the mucous membranes, due to the action of the poison on the haemoglobin. ARSENICAL NEURITIS. Historical. — Up to the year 1883 the various nervous affections produced by acute or chronic poisoning by arsenic had been ascribed to spinal-cord lesions. Vulpian,^ Virchow and Scolozouboff ^ insisted upon the central origin of arsenical paralysis ; the latter, with Popow,^ ^ American Journal of the Medical Sciences, 1901. ^Lepons sur les Mai. du Syst. Nerv., 1879, p. 157, » Arch, de Phys. Norm, et Path., 1884, p. 325. * yirchow's Arch., 1883, Bd. 93, p. 351- ARSENICAL NEURITIS. 225 basing his assertions upon physiological experiments. Dejerine/ how- ever, in 188^, after his, studies of alcoholic paralysis, already cited, concluded from the close resemblance between alcoholic and arsenical paralysis that the lesion was a multiple neuritis. And subsequent observations by Philadelphia^ and Boston physicians, by Jaesche,^ Naunyn,* Dana,^ Goldflam,'' Jolly,^ and Henschen,^ have established beyond doubt that the lesion in arsenical poisoning lies exclusively in the nerves. This fact has received complete confirmation during the prevalence of an epidemic of arsenical poisoning from beer which occurred in England in 1899.^ Etiology. — Arsenical poisoning may occur from the accidental or suicidal ingestion of any one of the arsenical salts — Paris green being the one usually selected by those attempting suicide. The most common cause, however, is the accidental chronic poisoning which occurs in certain manufacturers, notably in tin mines, and in tin works; in dyeing establishments and in sulphuric acid factories, and in those industries in which sulphuric acid is largely used. This acid is now made chiefly from arsenical pyrites and thus contains consider- able quantities of arsenic. Chronic arsenical poisoning may occur from drinking beer in the brewing of which glucose, or "invert sugar," prepared by the aid of sulphuric acid, enters. Thus in the English epidemic it was ascer- tained that many beers brewed in Salford and Manchester contained from 0.14 to 0.28 grain of arsenious acid to the gallon, and this was shown conclusively to be present only in the glucose or invert sugar used in the brewing, all other constituents of the beer being free from arsenic. The glucose was found to contain arsenic in the pro- portion of 4 parts to 1 0,000. Chronic arsenical poisoning may also arise from the inhalation of particles of arsenic in the air, which are given off from wall-papers and from carpets, hangings and furniture coverings, and from arti- ficial flowers containing arsenical dyestuffs. ^"^ This was the cause in a case of my own. The patient was a woman, who, to avoid mis- carriage, was kept for four months in a room which had been newly papered and furnished with green cretonne subsequently found to con- tain large quantities of arsenic. The frequency of such poisoning has led to the passage of laws in the State of Massachusetts restricting the use of dyes containing arsenic. Many toilet powders contain arsenic, and the inhalation of particles given off from these, or the application ^ Cbmptes-rendus, Tome xcviii., Nr. 17, October, 1883. 2 Proceedings of the College of Physicians of Philadelphia, 1883, ^Inaug. Dis., Breslau, 1883. * Berliner klin. Woch., 1886, p. 555. * Brain, 1887, vol. ix., p. 456. «Zeitsch. f. klin. Med., 1888, p. 399. 'Deutsche med. Wochen., 1893, Nr. 5, and Charity Annalen, 1893. * On Arsenical Paralysis, Transactions of the Eoyal Society of Sciences in Upsala, 9 Kelynack and Kirkby, Arsenical Poisoning in Beer Drinkers, Balliere, Tindall and Cox, London, 1901. Also Lancet, 1900, vol. i., p. 1610. i» J. J. Putnam. Boston Medical and Surgical Journal, 1889, p. 235. 15 226 THE TOXIC FORMS OF MULTIPLE NEURITIS. of arsenic in them to the skin may produce poisoning. The medi- cinal use of arsenic in large doses or in small doses long continued may give rise to arsenical poisoning. This is particularly liable to occur in the treatment of chorea, of pernicious anaemia, and of car- cinoma, in all of which diseases the drug is employed for months at a time. I have seen two cases of arsenical paralysis among 1,400 children treated for chorea in my clinic. The more the danger of chronic arsenical poisoning is appreciated and the greater the use of articles into the manufacture of which it enters, the more frequent are the reports of cases arising in an unexpected manner. It is known that in Steiermark, in Austria, large numbers of the peasants eat arsenic. F. Miiller ^ has found arsenical paralysis very common among this class, in fact, even more common than diphtheritic paralysis. Pathology. — A simple parenchymatous neuritis, without any inter- stitial inflammation, has been found in many cases of arsenical multiple neuritis. In these cases the chief change is limited to a destruction and degeneration of the myelin sheath, the axis cylinder remaining intact. The myelin is broken into fragments or little balls, which are found in all stages of fatty and granular degeneration within the sheath of Schwann. The nuclei within the sheath are increased in number. This lesion has been termed segmental periaxillary neuritis, as the process may be scattered along the nerve and may not be con- tinuous from one segment to the next. In severer cases the axis cyl- inder is destroyed, being found in all stages of degeneration. In these cases many empty sheaths of Schwann are seen. Changes have also been found in arsenical paralysis in the spinal cord. The same chromatolysis in the cells of the anterior horns, which has been described in alcoholic paralysis, has been observed, but this, as already stated, is of little importance. Henschen has found a true degeneration in these cells, and has also noticed an ascending degen- eration in the columns of Goll, which may be due to a primary degeneration in the cells of the posterior spinal ganglia. Symptoms. — The symptoms of arsenical neuritis do not differ very markedly from those of alcoholic neuritis. In both there are two types of case — one characterized by paralysis, and the other by ataxia. There is little or no difference in the mode of onset of the symptoms between cases of acute and chronic poisoning. There are certain general constitutional symptoms which first appear in arsenical poisoning and attract attention. If the poisoning is acute, violent vomiting and diarrhoea occur at once, and sometimes save the life of the patient, the poison being rejected and not absorbed. If the poisoning is chronic, a puffiiness of the eyelids appears, at first most noticeable in the morning ; a tearful appearance of the face, and some catarrh of the nose is observed, and not infrequently gastric and en- teric symptoms develop. There may be pain in the stomach, nausea and inability to retain food ; and fluid, green or slimy movements with 'Wienermed. Presse, 1894, Nr. 15. ARSENICAL NEURITIS. 227 some tenesmus. In some cases a mild fever attends the attack, a rise of 1° ov 2° ^. occurring and persisting for some weeks. In arsenical neuritis the first symptoms complained of are sensory in type. Tingling and numbness in the feet and hands, burning of the surface, tearing and shooting pains, and great soreness in the skin and in the calves of the legs and feet, and soon a feeling of weakness in the legs, great fatigue on walking, difficulty in rising from a seat or in going up stairs, and a staggering gait are noticed. As the general feebleness increases, paralysis develops rapidly in some cases, in others ataxia is more marked. The paralysis appears in the feet and hands, the lower extremities being first affected. There is the same limitation of this paralysis to the small muscles and to the extensor groups of muscles of the extremities that is seen in alcoholic cases. The paralysis is flaccid in type, is soon followed by atrophy, which is first noticed in the interossei of the hands, and then appears in the peronei of the legs and in the extensors of the wrists, and is attended by loss of muscular tone and mechanical excitability. The reaction of degeneration is usually present. The tendon reflexes are diminished or lost. The superficial reflexes are normal or are increased. Drop-wrist and drop- foot develop early and may become extreme. The distribution of the paralysis is always bilateral and symmetrical. In severe cases the patients are confined to bed, and contractures in a flexed position gradually develop and may become permanent. Thus in a case of mine which followed acute poisoning, the hands were fixed in a claw position for many months after all other symptoms had passed away. When the ataxia precedes paralysis the gait becomes unsteady and irregular, the patient sways in standing with eyes closed, he drags his feet and shuffles along and finally shows the " stepping gait " described in alcoholic cases. The dropping of the feet serves to distinguish this gait from that of tabes, though French writers have described these cases as pseudo-tabes arsenicale. Such a gait was well marked in both patients with chorea, who developed arsenical paralysis under my ob- servation. The ataxia is also present in the hands and is accompanied by an impairment or loss of muscular sense. It is always attended by a considerable degree of weakness, and this commonly goes on to paralysis and the patients have to go to bed. In both types of the disease, when the symptoms are fully developed, the pain and tenderness are extreme. Many patients cannot endure the slightest touch, even the bedclothes causing agony ; and the ex- treme tenderness in the muscles below the knees and elbows prevents any manipulation of the extremities. The hypersesthesia is felt more acutely in the distal parts of the extremities aud rarely above the elbows and knees, but when the ataxia and paralysis are fully devel- oped it renders the patient particularly helpless. A marked tremor in the hands is usually an early symptom in both types of case. The ocular muscles and the facial muscles are not affected and the trunk muscles, the intercostals, and the diaphragm also escape. Occasionally erythema, redness of the skin, oedema of the extremities, cyanosis and 228 THE TOXIC FORMS OF MULTIPLE XEVIilTIS. unusual sweating, indicate an implication of vasomotor and trophic nerve fibres. Some observers have reported a condition resembling erythromelalgia. The diagnostic symptom of greatest importance in arsenical neuritis is the appearance presented by the skin. This becomes gradually darker from the deposit of pigment, so that the complexion resembles that of a gypsy, and if extreme the skin may finally turn almost black. The pigment is first deposited about the normally pigmented regions of the body, in the axillae and groins and around the nipples. It soon appears, however, on the eyelids, on the abdomen and thorax, and finally on the surfaces of the arms and legs, the flexor surfaces being last aifected. The pigmentation is diffuse, but here and there little islets of normal skin give a mottled appearance to the surface. The color is rather darker than that seen in Addison's disease, and the mucous membranes are never discolored as in that affection. The pig- mentation may be preceded by erythema and may be followed by various eruptions, papillary, eczematous, or herpetic. Herpes has been observed in many cases, usually on the limbs, sometimes on the body. After a time the skin desquamates in small flakes, but several desqua- mations may be necessary before a normal color is regained. Some- times there is a marked thickening of the skin, especially over the knuckles, on the palms and soles. Changes in the nails are very common, a thickening and brittle state being evident and rough ridges appearing transversely. The appearance of normal nails growing out above the ridges indicates beginning recovery. In the Manchester epidemic there were skin lesions in 97 per cent, of the cases.^ In one case of acute poisoning by Paris green, a reddish eruption appeared on the tenth day upon the hands, wrists, and forearms, and upon the aukles and feet, and in twenty-four hours had spread over the entire body. Four days later this had gradually disappeared, and then numbness was first felt in the hands and feet. This extended rapidly, and in three days had reached elbows and knees. Paralysis began at the same time as the numbness, and in a week all power of movement below the knees and elbows was lost. This remained for two weeks, and then slowly passed off, recovery not being complete for six months. The sphincters are never involved in arsenical paralysis. Mental symptoms have been described by a few observers, consist- ing of hallucinations, mild delusions, stupor, and loss of memory. In the English epidemic these were observed only in individuals who showed other signs of chronic alcoholism, but Jolly has seen them in a non-alcoholic case. And the fact that arsenic has a stimulating action upon the brain is said to be one reason for its use as a food by the Steiermark peasantry. The symptoms all subside slowly and progressively, and recovery of power and of coordination is accompanied by a relief from the hyper- sesthesia and the pain. ^British Medical Journal, 1900, vol. ii., p. 1725. ARSENICAL NEVRiTlS. 229 The duration of arsenical paralysis is somewhat greater than that of arsenical a^xia. Either condition may last several months, but occa- sionally the recovery is complete in a few weeks. Frequently after the power has returned, the patient suffers for months from numbness and tingling in the extremities, which are sufficient, as in a case under my own observation, to interfere with the finer motions, to disturb the sleep, and to cause constant discomfort. According to Gerhardt, 97 per cent, recover wholly. And in the English epidemic, although the number of cases was very large, several thousand having been ob- served in the hospitals and dispensaries, the number of deaths reported was comparatively small. Treatment consists in elimination of the arsenic by the free use of water and in restoring the general health of the patient. It does not differ in any way from the line of treatment for multiple neuritis, already described in detail on page 205. The following case is interesting on account of its causation, and offers a typical history of the disease : Female, aged thirty years, previously in good health, became preg- nant in November, 1894, and until March 10, 1895, suffered exceed- ingly from the vomiting of pregnancy, and became rapidly emaciated from inability to retain food, so that she lost ninety pounds in weight. During this time she was confined to one room, the furniture and wall- paper of which on subsequent examination were found to contain about two grains of arsenic to the square yard. On March 10th she began to suffer from tingling and numbness in the legs and noticed some dif- ficulty in stretching out her legs, and this paralysis increased rapidly, so that by the 15th of March there was almost total paralysis of both legs and very great pain and tenderness in both legs, together with a sense of numbness and tingling in the feet and legs as high as the knees. There was great sensitiveness of the legs to cold and heat, and any movement of the legs was extremely painful, the pain being felt in the muscles. The knees were preferably kept in a position of flexion as extension was too painful to be endured. On the 20th of March an extensive eczema appeared all over the body, but more par- ticularly in the legs and arms, and was attended by a deep brown pig- mentation of the skin and by some desquamation. On April 1st, a total paralysis had developed in the feet and ankles and drop-foot was present on both sides. The muscles below the knees were very weak, but the thighs could be moved normally. The knee-jerks were lost. Any attempt to stand was impossible on account of the paralysis of the feet and ankles, and the extreme pain that any pressure upon the soles of the feet produced. The muscles were flabby and the muscu- lar tone and mechanical excitability were abolished, and faradic con- tractility was lost in the muscles below the knees. There was no affection of the bladder or rectum and no tendency to bed-sores, but the nails of the toes were discolored and rigid, and the nails ceased to grow for three months. There was a constant sensation of tingling and numbness in the fingers, but no evidence of paralysis or anses- 230 TEE TOXIC FORMS OF MULTIPLE NEURITIS. thesia. Her room was changed, thus removing her from the source of the poison. The gastro-iutestinal catarrh, which was doubtless due to the arsenic, soon subsided under treatment and she began to retain her food. In Auffust she was able to walk with a little aid. In Decern- ber she still required some help in walking, as the foot-drop persisted, and braces had to be used for the ankles, with high-heeled shoes, and at this time her legs were still stiff and numb. There were no knee- jerks, and sensations of pain, heat, and cold were much less quickly and keenly perceived below the knees than above, or upon the hands. In April, 1896, she had recovered entirely. MULTIPLE NEURITIS DUE TO POISONING BY LEAD. Etiology. — It is said that the use of lead enters into 111 industries. Poisoning by lead is always a chronic poisoning and usually occurs in painters, in plumbers, in typesetters, or in workers in lead objects such as toys, who come in contact with lead, either in the form of metallic lead or of some of the salts of lead which are used in mixed paints. Painters who work in the open air are less liable to lead poisoning than those who labor in rooms ; and those are most liable who, like the varnishers in carriage factories and furniture establishments, scrape off old paint preparatory to putting on a new coat. Any work which results in the production of dust which may be mixed with lead, or its salts, may be productive of lead poisoning. It is evident that the inhalation of the dust is one method of the entrance of the poison into the body. Laborers who work in factories where china earthenware is produced and where glazes and fretted glass are manufactured are very liable to lead poisoning. In the midland counties of England, where such industries are common, in Limoges, France ; in Dresden, Germany, and in Trenton, New Jersey, many cases are seen annually. The usual method of poisoning, however, is by swallowing the lead, and, as many painters do not properly clean the hands before taking their noonday meal, the poisoning is often acquired in this way. Some individuals, however, appear to be extremely susceptible to lead poisoning. Thus, I have known most serious cases in children of painters who could only have obtained the poison by inhalation of the fumes of paints that were kept in the room. I have known small amounts of lead to produce severe poisoning when taken into the stomach. Beer bottles are frequently cleaned by being shaken with lead shot within them, and I have known lead poisoning to occur from drinking the beer from such bottles. I have known lead poison- ing to occur in servants who drank the water first drawn from the spigot early in the morning, water which had stood in the lead pipes all night and had thus become impregnated with some of the soluble salts of lead. I have known severe cases in typesetters whose fingers were stained by the constant handling of metallic lead. I have also known it in plumbers after long labor in laying lead pipes. Horses are subject to lead palsy. Lead is not frequently used as a medicine, MULTIPLE NEUBITIS DUE TO POISONING BY LEAD. 231 and I have never known a case of poisoning from this source. Alco- holic subjects and those who use tobacco in excess are more liable to develop lead palsy than others. Pathology. — The pathology of lead palsy has been a matter of dispute among authorities for many years. Many writers have found changes in the cells of the anterior horns of the spinal cord, while others, more recently, have maintained that the changes are limited to degeneration of the peripheral nerve trunks. The establishment of the neurone theory has thrown light upon these differences of opinion, and the discovery of more delicate methods of staining by Nissl and Marchi have demonstrated that the entire motor neurone may. be affected in this disease. The most evident changes are a degenerative neuritis with atrophy . in the peripheral nerves, chiefly in the radial nerve but not infrequently in many of the peripheral branches of the brachial plexus. The degree of this degeneration and its extent de- pends upon the severity of the poisoning. There are few, if any, changes in the neurilemma or connective tissue sheath, hence this form of neuritis is purely a parenchymatous neuritis. While the majority of these changes are limited to the terminal filaments of the nerves, there may be slight changes detected in the trunk of the nerves and in the anterior nerve roots. The method of Nissl demonstrates chroma- tolysis in the cells of the spinal cord and in those of the spinal ganglia, but these are not usually of a sufficient degree to produce degeneration or complete atrophy that is irreparable. In the severer cases, how- ever, vacuolization of the cell and final degenerative changes may take place. Changes of a similar kind have also been found in the large cells of the brain cortex. The serious lesions are more commonly found in the peripheral nerves than in the spinal cord. Symptoms. — In 93 per cent, of cases the onset of the paralysis is preceded by a severe attack of lead colic. The patient suffers from intense pain in the region of the umbilicus, attended by obstinate con- stipation, very often by nausea and vomiting. The pain is supposed to be due to an irritation of the visceral nerves by the lead. It may also be due to distention of the intestines from paralysis of these nerves and cessation of peristaltic action. There is a characteristic blue line seen along the gums, not to be mistaken for staining of the teeth be- cause of its location and clear blue color. Anaemia is almost always present in patients who have been poisoned by lead. Another com- mon affection in persons exposed to lead poisoning is chronic articular rheumatism. Thus, Tanqueral found that in 1,217 patients who suf- fered from lead colic, 755 developed painful joints, 107 developed paralysis, and 72 suffered from headache. A few days after the onset of an attack of colic, or sometimes not until two or three weeks after the attack has subsided, the patient is suddenly seized with paralysis of the extensor muscles of the fingers, thumb, and wrist. It is a rule for one hand to be affected several days before the other, and occasionally both hands are unequally affected throughout the disease. It is quite commonly found that the paralysis of the extensors of the index finger or of the thumb 232 TBE TOXIC FORMS OF MULTIPLE NEURITIS. precedes the paralysis of the other muscles. As a consequence of this paralysis the wrists drop and cannot be raised, and from lack of ful- crum power the flexors are apparently weakened. If, however, the wrists be extended by the examiner and held, it will be found that the flexor power in the hand and fingers is not impaired. The supinator longus muscle is rarely, if ever, affected, and hence it is apparent that the lesion is limited to the distribution of the radial branch of the musculospiral nerve below the point where the nerve to this muscle is given ofi; There is very often a tremor in the hands and fingers, which comes on early and remains until the paralysis subsides. There is no affection of sensation as a rule, though sometimes tingling and numbness is felt in the back of the hand or in the fingers. The muscles that are affected rapidly undergo atrophy of an extreme type, so that within a couple of weeks of the onset the back of the forearm presents a very atrophic appearance and the muscles show the reaction of degeneration. As a result of the drop-wrist a deformity of the back of the hand soon appears, due to a displacement backward of the internal bones of the wrist, forming a hard protuberance upon the back of the hand near the base of the metacarpal bones ; but firm contractures or per- manent shortening in the paralyzed muscles, such as occurs in alcoholic or arsenical cases, does not develop in lead palsy. Not infrequently, if the disease is a severe one, the intrinsic muscles of the hands also suffer early and the hands become quite useless, apposition of the thumb to the fingers and any abduction or extension of the thumb and fingers being impossible. Then it is evident that branches of the ulnar and median nerves are also involved. The reaction of degeneration appears early in the muscles, and the faradic reaction does not return until the patient has been well for some months. In the majority of cases the paralysis does not go beyond the mus- cles of the forearms and hands, and after a period of five to six months there is a gradual return of power and complete recovery. The patients often complain of severe cramps in the muscles — a form of pain which may even prevent sleep — but pain is not a common symp- tom in lead palsy. The disease is a very slow one and the patients are rarely able to return to work within six or seven months of the onset. As their livelihood depends upon their labor, it is well that this should be un- derstood from the outset, so that during the period of incapacity they may seek some other occupation which will enable them to support life. Relapses are common when patients return to their work. In more serious cases the paralysis may appear in the muscles of the shoulder, the deltoid being first affected, and then the biceps, brachialis anticus and supinator longus being involved. Occasionally the mus- cles of the legs below the knees become paralyzed, the peroneal group being more commonly invaded, though in severe cases the extensors of the toes and even the anterior tibial group may be equally paralyzed. MULTIPLE NEURITL^ DUE TO POISONING BY LEAD. 233 This only occurs in 4 per cent, of the cases and has been observed chiefly in children. It .causes drop-feet and a stepping gait. In the most severe type the patient is almost totally paralyzed. Fibrillary twitchings are occasionally seen in the muscles, but they are not the rule. A general sensory disturbance is common, the patients suffering from intense pain in the arms and legs, from anaesthesia of the hands and feet, and from very disagreeable sensations of pricking and ting- ling. The sphincters are never involved. Vasomotor and trophic symptoms are usually absent, though the hands and arms may be cold, cyanotic, and may perspire too freely. In the severe cases it is possible to demonstrate a condition of tenderness along the affected nerves, but in slighter cases no tender- ness is felt. A type of lead palsy has been described in which the symptoms appear first or exclusively in the cranial nerves ; the laryn- geal, the pharyngeal, the lingual, the ocular, the facial, and the optic nerves having been affected. In these cases headache, vertigo, and mental apathy indicate that the brain as well as the nerves is invaded by the poison. This type is very rare. It is termed lead encephal- opathy by the German writers. The marked pallor and evident anaemia present in all cases is con- firmed by the examination of the blood ; the hemoglobin is low and a peculiar granular appearance of the red cells is found. Prognosis is good as to recovery, but the duration of the disease, as already stated, even in the lighter type, is six months, and in the more severe type very often a year. The following case illustrates a severe condition of chronic poisoning : Male, aged thirty-eight years, after suffering from an attack of lead colic, began to feel numbness and pain in his legs and feet, which soon extended to his hands and forearms. This steadily increased for two weeks, and to it was added paralysis of the extensors of both hands and both feet, so that at the end of that time he was unable to use his hands or to stand. With the paralysis there was rapid atrophy of the muscles, and a decline in the faradic excitability. The muscles became more and more tender, and the spontaneous pains gradually increased until it was necessary to use opium freely to quiet him. By the end of a mouth a well-marked anaesthesia had developed in the hands and below the knees, and it was noticed that the atrophied muscles were in a constant tremor. There were wrist-drop and foot-drop on both sides, and all reaction to faradism ceased. It required a very strong gal- vanic current to produce contraction and AGO was greater than KCG. The knee-jerk was preserved. The muscular sense was impaired. For five months his condition remained stationary, in spite of treatment by strychnine, iodide of potash, massage, and electricity. During a month of his illness he was delirious at night, was excited in the daytime, and subject to de- lusions and had little memory or mental capacity ; but these symptoms gradually subsided. Then a gradual improvement set in, the pains became less severe, sensation returned, the muscles regained their con- 234 TEE TOXIC FOBMS OF MULTIPLE NEURITIS. tour and strength, and, finally, the electric contractility returned to the normal standard. About a year from the onset of the symptoms the recovery was complete. Treatment. — Treatment of the lead colic is oy means of full doses of opium to quiet the pain, and by laxative saline salts, such as sul- phate of magnesia, to relieve the constipation. Large draughts of water should be given constantly, in order to eliminate the poison, which passes off partly through the kidneys. During the stage of chronic poisoning the patient should be instructed to drink a great deal of water, and five grains of iodide of potash may be given three times a day to assist in the elimination of the lead. I have never been able to see that strychnine, which is usually recommended, has any effect upon the progress of regeneration, though general tonics — cod-liver oil, iron, and quinine — may well be employed, as these patients are uniformly anaemic. Treatment of the paralysis is by massage and electricity, galvanism being the only current which will produce any effect. Multiple neuritis of the ataxic type, due to poisoning by phosphorus, has been recorded by Henschen in one case, and in seven other cases of acute poisoning he found mild nervous symptoms of parsesthesia and tenderness along the nerves. The condition must be a very rare one, as it has not been seen by others. Inquiry among employes in match factories failed to elicit any knowledge of a special disease peculiar to this occupation. Mercurial poisoning has been said to cause multiple neuritis, but no case can be found which is not open to objection. The tremor which is produced by acute mercurial poisoning, and which is observed in workers in looking-glass factories, is accompanied by headache, vertigo, and occasionally by hemiplegia or monoplegia of cerebral type, and is not followed by multiple neuritis. Chronic mercurial poisoning after or during the treatment of syphilis also fails to cause neuritis. Gowers^ has described a case of poisoning by silver, in which par- alytic symptoms developed in the arms with drop-wrists, resembling closely those due to lead poisoning. The characteristic blue coloring of the skin was present. The patient died of cancer and no autopsy was made. This is the only case thus far observed. A few cases have been recorded ^ of multiple neuritis due to poison- ing by copper occurring among brass workers. In these cases the symptoms resembled those of the ataxic type of neuritis seen in alco- holics. They are so rare as to require mention only. When platinum is dissolved in aqua regia red fumes of oxides of nitrogen are evolved which are very toxic. In a workman affected by these fumes a painful condition of multiple neuritis developed which ran a long and severe course but finally recovered. ^Diseases of the Nervous System, German edition, vol. iii., p. ooo. '^ Suckling. British Medical Journal, 1888, vol. ii., p. 1334. Walton and Carter, American Journal of the Medical Sciences, 1892, vol. ii., p. 61. CHAPTER X. THE INFECTIOUS AND TOXEMIC FORMS OF MULTIPLE NEURITIS. ' Multiple Neuritis Subseguent to Diphtheria ; to the Grippe ; to Typhoid, Typhus, and Malarial Fever ; to Scarlet Fever, Measles, Whooping-cough, and Smallpox ; to Erysipelas and Septicaemia ; to Gonorrhoea and Puerperal Fever. Leprous Neuritis. MULTIPLE NEURITIS SUBSEQUENT TO DIPHTHERIA. Diphtheritic paralysis is one of the most common forms of mul- tiple neuritis, but does not occur as frequently as a sequel in cases that are treated by antitoxin as in those which are allowed to run a normal course. Rosenau and Anderson ^ have shown that in guineapigs one unit of antitoxin injected on the first day prevents paralysis while 4000 units injected after forty-eight hours fails to prevent paralysis. After the paralysis has appeared antitoxin fails to affect it in any way. The number of cases of diphtheritic paralysis appearing at my clinic since the use of antitoxin has been much less than in any previous period. Rothe ^ found 64 cases of diphtheritic paralysis in 744 cases of diph- theria treated at the Charite Clinic in Berlin, which gives a frequency of 8.7 per cent, of the cases of diphtheria followed by paralysis. This observation is in accord with that of other observers.^ Bernhardt called attention to the fact that in many cases of diphtheria there is a loss of the knee-jerk, a fact which seems to him to point to a probable susceptibility of the entire nervous system to the diphtheritic poison, but Rothe found a loss of the knee-jerk in but one-half of the cases which were paralyzed. It is certainly a fact that for many weeks after the disappearance of all symptoms of diphtheria, even in cases which show no paralysis, the knee-jerk may be absent. Pathology. — The pathology of diphtheritic paralysis has been a matter of much discussion. Some authors maintain that the lesion is a degenerative neuritis only, while others maintain that .the lesions are central in the spinal cord and brain axis. A parenchymatous neuritis is the chief lesion, but occasionally a diffuse process with involvement of the interstitial tissues has been observed. In the finest nerves of the muscles and in the skin, the destructive process is more fully de- veloped, as a rule, than in the trunks of the nerves or in the larger nerves. The neuritis is more pronounced in the cranial nerves in diphtheritic paralysis than in any other form of multiple neuritis. » P>ulletin No. 38, U. S. Marine-Hospital Service, 1907. 2 Inaug. Dissert., Berlin, 1899. » Wollacott. Lancet, August 20, 1899. 235 236 INFECTIOUS AND TOXEMIC POUUS OF NEURITIS. Lesions of the anterior and posterior nerve roots have been found by Dejeriue, and many authors have described parenchymatous degenera- tion in the anterior horn cells and in the posterior spinal ganglia. Jlecent experimental investigations by Murawjeff^ show that the toxin of diphtheria produces chromatolysis and degeneration in the cell bodies of both motor and sensory neurones. He also finds that it attacks the peripheral nerves as well ; in some cases before, in others after it has attacked the neurone. Symptoms. — In the lighter forms of diphtheritic paralysis the soft palate is the only part that is affected. In 472 cases of diphtheritic paralysis observed by Wollacott the soft palate was affected in 413, the difficulty of swallowing, with regurgitation of fluids through the nose and a thickening and indistinctness of the voice being the only symptoms present. There is a loss of reflex in the throat in these cases and a lowered sensitiveness of the pharynx, so that irritation does not cause acts of swallowing. The limitation of the paralysis to the palate has been explained by supposing that the poison of the dis- ease has a direct action upon the terminal filaments of the nerves which, in this position, are, as it were, dipped constantly in the poison. This theory is supported by a case in which paralysis of the abdominal muscles was associated with a diphtheritic inflammation of the navel in a newborn child. But the more serious cases prove that it is through the blood that the poison is carried to nerves far removed from the diphtheritic inflammation. Next in frequency to paralysis of the palate occurs paralysis of some of the ocular muscles, producing internal squint and double vision. This paralysis may affect any one or many of the muscles moving the eyeball of one or both eyes, though it is less common for the levator palpebrse to be affected than any other muscle. The ex- ternal recti are more often affected than the internal, and it is not common to find all the muscles supplied by the oculomotor nerve affected together. Among Wollacott's 472 cases, 104 had some ocular palsy. The paralysis of accommodation prevents reading. The paralysis may not extend to any other muscles than those already named, and in the lighter cases remains in them for a period of four to ten weeks and then gradually passes off. Not infrequently, however, the disease extends much more widely and after a week or more of local palsy of the throat or eyes, or both, the individual develops within twenty-four hours a widespread paral- ysis of both arms and both legs. This is usually preceded or attended by incoordination of movement in all the finer adjusting actions of the hands and by ataxia in the act of walking. Associated with this ataxia and with the weakness of the muscles there are frequently drop- wrist and drop-foot, so that the patient exhibits the stepping gait already described as occurring in alcoholic neuritis. There are also sensory symptoms consisting of numbness in the ex- tremities, disturbance of sensation both of touch, temperature, and pain and of the muscular sense and delayed sensations of pain. Shooting ^Neurolog. Centralbl., 1898, p. 475. MULTIPLE NEURITIS SUBSEQUENT TO DIPHTHERIA. 237 pains are sometimes felt, but are not very prominent and there is rarely tend^ness along the nerve trunks. If the paralysis is not so extreme as to suspend all movement, a tremor is not uncommonly ob- served in the motions of the fingers. The tendon reflexes are lost and mechanical excitability of the muscles disappears ; a rapid atrophy with reaction of degeneration develops in the paralyzed muscles. Paralysis of both sides of the face may occur. In a still more severe type of case, either soon after the onset or after the paralysis has been present for several weeks, bulbar symp- toms may appear, with great difficulty in swallowing, atrophy, and weakness of the tongue, spasms of coughing (which may be danger- ous), weakness of the face, and paralysis of the vocal cords. Such a complication is extremely grave, as the patient may die of respiratory paralysis, of suffocation, or of pneumonia due to the inhalation of food. This occurs in 10 per cent, of the cases. The onset of the paralysis may occur within a week of the infection or it may be delayed for several weeks. In Rothe's cases the paralysis developed in the major- ity in the second week, but in a few over forty days had elapsed between the initial symptoms and the development of paralysis. Diphtheritic paralysis is not by any means confined to children, but occurs in adults and even in old people. Not infrequently it is impos- sible to ascertain a definite history of diphtheria, and a very light attack of diphtheritic sore throat may be followed by a severe attack of diphtheritic paralysis. The occurrence of paralysis of the throat and eye muscles and a marked ataxic condition of the limbs enable one to make a diagnosis, even in cases where no sore throat has occurred. Prognosis. — The prognosis in diphtheritic paralysis is good, pro- vided the condition is limited to paralysis of the throat, or of the eyes, or to a general diphtheritic ataxia. If, however, a true paralysis of the muscles of the limbs ensues ; if the respiratory muscles are aflFected, or if the pneumogastric nerve is invaded, causing frequency of the pulse, the prognosis is very serious. Treatment. — The treatment of diphtheritic paralysis is that of multiple neuritis. The danger of inhalation of food or of imperfect nutrition from difficulty of swallowing is not to be forgotten, and in the extreme cases it may be well to feed the patient with a stomach tube rather than to allow them to attempt to swallow, if this be productive of fits of choking and coughing. Inasmuch as all effiart seems to in- crease the severity of the symptoms, it is well that these patients should be kept in bed as long as there is any difficulty of motion. Strychnine is of a great deal of service in the treatment of diphtheritic paralysis. It was for a time supposed to be a specific for the disease, but this was at a time when the lesion was thought to be central. It is not to be used in very large doses, -^wo ^^ ^ grain every three hours being sufficient for an adult. It has not seemed in my experience to shorten the duration of the paralysis. Tiie following cases are good examples of the ordinary type of diphtheritic paralysis : 238 INFECTIOUS AND T0X2EMIC FORIIH OF NEURITIS. Male, two and a half years of age, suffered from measles during the month of January, 1894, and on the first of February developed symp- toms of diphtheria. This ran its ordinary course, though it was nec- essary to perform intubation and the tube was retained about one week. He then gradually recovered and after a week appeared to regain his ordinary strength ; his appetite improved, and he could stand aud run about. On the 20th of March he was suddenly seized with paralysis, which within two days had become most extensive. He then had ex- treme strabismus due to a paralysis of both internal recti. There was no paralysis of the face, tongue, or palate, and he could talk well ; but there was complete paralysis of the muscles moving the head and of both arms and both legs, and of the body. He was unable to sit up, to move his head, or to move any of the extremities. There was no knee-jerk. There was no disturbance of the bladder or rectum, there was no pain or tenderness ; but respiration was difficult and his pulse was 140. On the 24th of March he died of respiratory paralysis. A boy aged thirteen years, after an attack of diphtheria developed paralysis of the soft palate. One week after the paralysis of the pal- ate he noticed a disturbance of vision which proved on examination to be due to a total paralysis of all the muscles of the eyeballs. The pupil reflex to light was preserved ; but there was no reaction in ac- commodation. This paralysis of the eyeballs came on within twenty- four hours, and at the same time he developed a very marked degree of ataxia. He swayed in standing with eyes closed, and his gait was exactly like that of a well-marked case of tabes. The motions of the hands were also disturbed, and all the tests demonstrated an extreme degree of inco5rdination. The knee-jerks were lost ; the skin reflexes were present. In addition to the ataxia there was actual weakness of the muscles, which was more evident in the arms and hands than in the lower extremities. He had formication in the legs, but not in the hands, and there were no lightning pains. He had great difficulty in swallowing solids as well as liquids, which seemed to point to some in- volvement of the muscles of deglutition as well as of the soft palate. This condition remained stationary for about three weeks, and then all the symptoms began to pass off gradually. His recovery was not complete until six months after the onset. A girl, aged thirteen years, after suffering for a few days from a slight sore throat, found herself unable to see clearly because of the develop- ment of a paresis of accommodation. There followed a slowly pro- gressing ataxia of the upper, and later of the lower, limbs, with un- certain gait, disturbance of touch, pain, temperature, and muscular senses, with delayed sensation of pain aud loss of tendon reflexes. At first she felt shooting pains in the limbs, but later these ceased. There was at no time tenderness of the nerves to pressure. After three months bulbar symptoms appeared, atrophy with paresis of the tongue, difficulty in swallowing, spasms of coughing, weakness of voice, and paralysis of the palate. The muscles of the hands began to atrophy about the time that the bulbar symptoms commenced and soon were useless. In the atrophic muscles the faradic reaction was absent, the MULTIPLE NEURITIS SUBSEQUENT TO THE GRIPPE. 239 galvanic much reduced and very slow. Nine months after the onset she died of pneumonia. The autopsy showed the brain and spinal cord to be normal. In all the peripheral nerves of the extremities, as well as in the hypo- glossal and recurrent laryngeal nerves, well-marked atrophic degenera- tion was found, with destruction of axis cylinders and medullary sheaths, and with thickening and increase of nuclei in the connective- tissue sheaths.^ MULTIPLE NEURITIS SUBSEQUENT TO THE GRIPPE. Severe attacks of neuralgia accompany or follow the grippe in about 15 per cent, of the cases, and are an evidence of direct action of the poison upon the nerve trunks. Thus trigeminal neuralgia, occipital neuralgia, intercostal neuralgia, and sciatica frequently develop and sometimes run a very severe and long course. They are occasionally bilateral and symmetrical, which is rare under other conditions. Many cases of local neuritis appearing in one or in several nerves in the body at once have been observed after the grippe. Any of the cranial or spinal nerves may be involved. It is not uncommon for such a neuritis to attack symmetrical nerves on both sides of the body. Thus I have seen both perouei affected, both ulnar nerves affected, both median nerves affected, and the brach- ial plexus affected on both sides. In all these cases the neuritis ran a typical course. Draper,^ Remak,^ and Bernhardt ^ recorded cases of multiple neuritis accompanied by acute ataxia following the first epidemic of influenza, 1890. Mills,^ Buzzard,^ Eisenloch,'' Savage,^ and others subsequently reported cases of multiple neuritis with sensory symptoms and with paralysis without ataxia, and various writers, in confirming these obser- vations, have described cases in which tlie cranial nerves of one or both sides, the ocular nerves, the facial nerves of one or both sides, and the palate have been involved. In a few cases oedema in the limbs has been noticed as a prominent symptom. These cases have developed within one or two weeks of the attack of influenza, have reached their height about one month after the attack, and have re- mained for many months. A few cases with rapid recovery within two months have been seen. There are no particular features to dis- tinguish these cases from the ordinary types of neuritis, and there is no special treatment for them. iRast. Deut. Arch. f. klin. Med., 1886, Bd. xl., S. 41. 2 New York Medical Kecord, 1890, p. 239. 3 Berliner klin. Woclien., 1890, p. 181. nbid., p. 643. ^Boston Medical and Surgical Journal, 1892, pp. 349, 405. * Transactions of tlie Philadelphia Medical Society, 1892. ''Neurolog. Centralbl., 1893, p. 380. ^ Journal of the American Medical Association, July 24, 1897. 240 INFECTIOUS AND TOXMMIC FORMS OF NEURITIS. MULTIPLE NEURITIS SUBSEQUENT TO VARIOUS INFECTIONS. It is still somewhat a matter of conjecture whether cases of paralysis following typhoid, typhus, and malarial fevers are due to an affection of the peripheral nerves or of the spinal cord. The researches of Pitres and Vaillard ^ have shown that extensive degeneration of peripheral nerves is to be found in the bodies of patients who have died of typhoid fever, and they have also demonstrated that these fevers are often followed by local neuritis as a sequel. Cases of mul- tiple neuritis with autopsies have not as yet been reported after typhoid or typhus. It is so common in severe cases of these diseases to use alcoholic stimulants as a remedy that it is not impossible that in some of these cases the real cause of the disease was alcoholic poisoning. In several hospital cases which I have seen this has been undoubtedly the cause of the supposed post-typhoid neuritis, the symptoms being iden- tical with those occurring in alcoholic cases and including cerebral symptoms, delusions, and loss of memory. Hence great care should be taken in reaching a conclusion that any post-typhoid case is really due to the infection, although all writers on typhoid emphasize the pos- sibility of multiple neuritis as a sequel. Buzzard recorded ^ two cases of paralysis following malarial fever, in which all the symptoms pointed to an affection of the peripheral nerves. In this country Gibney has described several cases of paral- ysis of the extremities, of sudden onset, rapid course, and prompt recovery under large doses of quinine, which he considered malarial. The following case, seen with Holt, was of this nature : A healthy boy, aged ten years, was suddenly seized with a chill and fever and with pains of a severe kind in both legs, associated with weakness so that he could hardly stand. The pain was referred to the sciatic nerves and was accompanied by numbness and tingling of the feet and legs. There was marked tenderness both in the course of the sciatics and in the muscles of the thigh and leg. There was loss of tendon reflexes, preservation of skin reflexes, and marked paresis in all the muscles of the legs, so that he required help in walking. There was no ataxia and no incontinence of urine. The symptoms lasted about twenty-four hours and then decreased in severity during the following day, but recurred on the third, and, after a remission, on the fifth day, with lessened severity. The periodicity of the affection and the rapid cure under quinine left no doubt as to its malarial nature. During the third day, at the time when I saw him, there was redness along the course of the sciatics and in the region of the right median nerves and extreme tenderness along these nerve trunks, in addition to the symptoms mentioned. The electric condition was, un- fortunately, not tested ; but in WestphaPs case faradic contractility was totally abolished during the attack.^ In this case the active mani- festation of the malarial poisoning was by general neuritis. In Buz- ^Rev. de Med., 1885, p. 980, Des Ndvrites peripheriques. ^Paralysis from Peripheral Neuritis, p. 104, Loudon, 1886. ^Neurolog. Ceutralbl., 1885, p. 187. NEURITIS SUBSEQUENT TO VARIOUS INFECTIONS. 241 zard's cases the malarial attacks preceded the nervous affection. The tenderness iij*.muscles and nerves left no doubt regarding the peripheral nature of the disease. I have recently seen a very severe case of general neuritis with total paralysis in both arms and legs lasting many months, and paralysis of the diaphragm which lasted for two weeks and was attended by rapid pulse and great exhaustion. In this case the only cause ascertainable was an attack of severe dengue fever which immediately preceded the paralysis. The patient was an engineer and contracted the disease in South America and was brought to New York with great difficulty. He had cerebral symptoms for some weeks at the height of the disease. Neuritis following variola is a rare complication, and the following case is the only one on record in which an autopsy showed the lesion to be a multiple neuritis : A young man had varioloid in November, 1881, and while con- valescing, six weeks later, began to suffer from severe pains in his four extremities, especially in the joints of his arms, which were diagnosti- cated as rheumatic, although there was no fever. Soon after there followed a true paresis, with progressive atrophy of the muscles of the forearms and legs. The muscles at the same time became very tender to touch or pressure. The tendon reflexes were much diminished. Reaction of degeneration developed in all the paretic muscles. The pains in the joints and limbs continued, but were less severe than at the outset. The sensibility of the skin was about normal. The nerve trunks were tender to pressure. Profuse, offensive perspiration in all four extremities was a distressing symptom. There was no tendency to bed-sores, but an extensive pemphigus developed in the legs, and then the pains became more severe. The patient died of pneumonia. Autopsy showed the brain oedematous and the cord in a state of hypostatic congestion. The pathological changes of importance were found in the nerves and muscles. The majority of the nerves of all the extremities were found in a state of degeneration and atrophy. There was marked degenerative atrophy and fatty degeneration of the muscles.^ Scarlet fever,^ measles,^ whooping-cough,^ and mumps have been, known to be followed by neuritis, both local and multiple, but the cases are so rare as to require only a mention. Multiple neuritis following erysipelas or of septicsemic origin has been reported by French'' and German^ authorities. I have seen a severe case in which the only cause found was a long-continued suppurating tooth. This patient had a continued fever during the early part of the disease. Bury^ reported a case in which an infected wound of the ip, Grocco, Milano. Centralbl. f. med. Wissen., 1885, p. 693. *M. J. Bassette. Journal of Nervous and Mental Diseases, 1892, p. 461. ^Allyn. Medical News, 1891, p. 617. *Leroux. Allg. Wiener med. Zeitsch., 1898, Nr. 29. * Charcot. Revue neurologique, 1893, Nr. 1 et 2. ^Gerhardt. Deutsche med. Wochen., 1898, p. 14. Kraus, Wiener klin. Wochen., 1897, Nr. 40. ' Eoss and Burj. Peripheral Neuritis, p. 291, 1893, X6 242 INFECTIOUS A^'D TOXEMIC FORMS OF NEURITIS. finger gave rise to secondary abscesses, and then multiple neuritis de- veloped. Both local and multiple neuritis have been known to develop subse- quent to attacks of gonorrhoea. They have no special characteristics. German ^ authors have described many cases of so-called puerperal multiple neuritis ; cases that appeared to be of the infectious type and due to septicsemia following abortion, miscarriage, or delivery. These cases are not to be confounded with the local neuritis of the sacral plexus consequent upon compression of this plexus during labor, nor are they to be confounded with cases of myelitis consequent upon anaemia of the spinal cord subsequent to confinement. The cases re- corded by the Germans have been of general widespread multiple neu- ritis of the septic type. Such cases have not been frequently observed in this country, though I have knowledge of four such cases, and in the large material at the Sloane Maternity Hospital there is no record of multiple neuritis occurring in the course of pregnancy or after labor. Cases of neuritis developing during pregnancy and traced to the exhaus- tion following long-continued vomiting are not unknown,^ but are not of the septic type. LEPROUS NEURITIS. Multiple neuritis occurring in leprosy is a very rare affection in this country, but is of some interest on account of the fact that it is the only form of neuritis in which the bacillus of any disease has been found in nerves. This bacillus locates itself entirely in the interstitial tissue of the nerve trunks within the sheath, in the perineurium, and by its direct irritation produces hyperplasia of connective tissue. This causes a compression of the nerve fibres and a secondary degeneration in them. Here and there along the course of an affected nerve are found small bulbous enlargements where the connective tissue is par- ticularly thick. The changes in the nerves themselves are those of simple degeneration. The lesion is due to the direct effect of the bacilli and is not secondary to any infection or any poisoning conveyed to the nerves through the blood. For this reason it is a localized neu- ritis, but is multiple in its character, inasmuch as very many of the terminal branches of the filaments of the nerves are involved. (See Plate XVII.) Symptoms. — The symptoms of leprous neuritis are the gradual development of irregular areas of anaesthesia, the occurrence of par- alysis and atrophy in the peripheral parts of the limbs, and the pro- duction of trophic disorders. The anaesthesia is extremely irregular in its distribution, depending wholly upon the branches of the nerves which are affected by the disease, irregular plaques of anaesthesia appearing here and there upon the hands and feet or upon the forearms and legs, and on the trunk, and not infrequently upon the face. These ' Moebius. Munchener med. Wochen., 1890, Nr. 14. Mader, Wiener klin. Wochen., 1895, p. 537. Jolly, Arch. f. Psych., 1897, p. 650. ^Stiefel. New York Polyclinic, 1893, March. Stembo, Deutsche med. Wochen., 1895, p. 461. ^ ^ ^ » PLATE XVII '.^Z -^ m :4Mi Cross-section of Sciatic Nerve in a Case of Leprous Neuritis. ( Marchi stain.) The degenerated nerve fibres in the various bundles of nerves are stained black. The Infiltration of the perineurium and epineurium with granulation cells, not shown by this stain, has forced the bundles apart. (Flatau, Spec. Pathol, u. Therap., Nothnagel, Bd. xi., Taf. viii.) LEPROUS NEURITIS. 243 anaesthetic areas do not, as a rule, correspond to the distribution of any one nerjte, nor are they symmetrical, as in ordinary cases of mul- tiple neuritis ; but are extremely irregular. They are attended by numbness and tingling and burning sensations or a pain of slight de- gree. The loss of sensibility may affect touch, temperature, and pain- ful sensations equally, or any one of these may be affected alone. A Fig. 105. Atrophy and trophic changes in hand and fingers in a case of leprous neuritis. loss of pain and temperature senses, while touch is preserved, is not uncommon, hence the disease has been mistaken for syringomyelia. The paralysis is usually found in the very small muscles of the hands and feet, but may extend to the longer muscles of the extremities, and not uncommonly a few of the facial muscles are paralyzed. A pro- gressive atrophy occurs in the paralyzed muscles, with reaction of de- generation. There is a loss of mechanical excitability in the muscles affected. The trophic disturbances present in the disease, viz., whitish colored plaques on the skin, peculiar thickening or atrophy of the skin, the glossy surface, the ulcerations, and the gradual loss of substance — are to be regarded rather as a part of the disease leprosy than as evi- dence of the local affection of the nerves. Fig.105 illustrates the condition occasionally resulting from an ex- treme condition of anaesthetic leprosy, the photograph being taken from a patient seen by me in consultation with Piffard — one of the few cases of the kind seen in New York within the past twenty years. The ends of the thumb and forefinger had been eroded. The disease is a slowly progressive one aud treatment appears to be of no avail. The differential diagnosis between lepra ansesthetica and syringo- myelia is sometimes somewhat difficult, as the trophic disturbances, anaesthetic areas, and nauscular palsies of irregular distribution occur 244 INFECTIOUS AND TOXEMIC FORMS OF NEURITIS. in both diseases ; but the distribution of the ansesthesia is usually greater in syringomyelia and begins in (if it is not entirely limited to) the upper extremities. White plaques are not common upon the skin in syringomyelia. Bulbous swellings upon a nerve trunk are not felt in syringomyelia, while in lepra anaesthetica there is an absence of those symptoms of spastic paraplegia, disturbance of the bladder and rectum, and increased reflex activity of the legs, which are commonly found in syringomyelia. References may be made to the articles of Schultze,^ Dehio,^ Zam- bacho,^ Laehr,* and Blaschko.^ ^ Deutsche Arch, f, klin. Med., 1888, Bd. 43. 2 St. Petersburg Wochen.,1889, No. 42, and 1890_, No. 48. ^ Verharidhingen des Internationale wissenschaftlichen Lepra Conferenzes zu Berlin, October, 1897. *Arch. f. Psych., Bd. xxx., 323. ^ Die Nervenkrankheitserscheinungen der Lepra, Berlin, 1899. CHAPTER XI. EPIDEMIC MULTIPLE NEURITIS. BERIBERI OR KAKKE. In 1882 Professor Scheiibe, of Tokio, Japan, called the atteutiou of European physicians to the existence of a peculiar affection prevailing among the Japanese.^ It was called kakke, from the two Chinese words — kiaku, meaning legs, and ke, meaning disease. It had been known among the Chinese for centuries, being mentioned by name in Chinese medical books written 200 B. C, and fully discussed by an eminent author in 640 A. D. It ceased, however, to prevail in China about two hundred years ago, and of late it is found chiefly in Japan. There its importance is considerable, since it is so prevalent that in 1877, 14 per cent, and in 1878, 38 per cent, of the meu serving in the army suffered from it. It is considered a miasmatic infectious disease by Scheube, although Takaki considered it due in some way to the diet of rice and fish. Braddon has pointed out that every beri- beric in the Orient is a rice-eater ; that persons on rice-free diet do not get beri-beri ; that the importation of machine-milled rice into any part of the world is followed by beri-beri ; that the users of home- milled rice do not get beri-beri ; that if from any cause the home supply is exhausted, beri-beri will break out on the importation of machine-milled rice. He, therefore, concludes that beri-beri is due to a saprophyte in the rice pericarp which invades the dead decorticated rice-grain or aleurone. In the primitive preparation the rice is husked in a mortar and then boiled, causing the pericarp to adhere to the aleurone, but giving the rice a dull appearance. The adhesion of the pericarp prevents the invasion of the aleurone by the toxic agent, and rice so prepared does not cause beri-beri. But, on the other hand, a final step in the machine milling of j-ice is its being passed through leather polishers, thus re- moving the pericarp, whose broken parts mix with the kernels and allow the toxic agent free access to the rice grain, a step which pro- duces a finer-looking grain, but a more dangerous article. It has been found that a toxin called " arsin " exists in the pericarp of rice, possibly of fungoid origin, and this can cause beri-beri. If the rice is properly prepared or " cured " this is removed, but " paddy rice " not so cured contains it and is the cause of the disease among the Chinese. Cooking does not "cure" the rice. Since wheat has been substituted for rice in the diet of some of the barracks and prisons * Deutsche Arch. f. klin. Med. , vols, xxvii. and xxxi. 245 246 RPlDEMtC MULTIPLE NEVRlTlS. in Japan the disease has been less common. It occurs in epidemics, but is always endemic in Japan. It rarely attacks Europeans. While not contagious, the disease is transmitted by human beings, for out- breaks have occurred on islands formerly free after the landing of per- sons who were affected. It aifects females rarely, only 9 per cent of the cases being in women ; and it is the youth of the land, between the ages of sixteen and twenty-five years, who suifer. Exposure to damp and cold in crowded dwellings, such as barracks, increases the liability to the affection. The majority of the cases occur during the hot, damp months, but some are always under observation. This disease is not, however, confined to Japan. It has been observed for many years in the islands of the Pacific Ocean, espe- cially in the Philippines, in India, Ceylon, on the west coast of the Red Sea, in Borneo and New Guinea, in Brazil and Cuba, on the isthmus of Panama, and in the Dutch possessions in the China Sea.^ It is there known under the name of beriberi. It is endemic in these regions, but occasionally occurs as an epidemic. An interest- ing account of such an epidemic, occurring in 1882-83 in Manila, in the Philippine Islands, has been given by Koeniger.^ It appears that in the fall of 1882 an epidemic of cholera occurred in Manila of such severity that 20,000 persons, in a population of 400,000 were affected. As a precaution against this disease the native population lived for several months exclusively upon rice, refusing to eat fruit or fish, which are their other chief articles of diet. As the epidemic was sub- siding a terrible cyclone devastated the city, destroying the light wooden houses, and leaving 60,000 families homeless ; and these poor people were exposed for several weeks to the inclemency of the weather, which at this time of the year was rainy. A few days after the cyclone the epidemic of beriberi began, and, as the disease had never before appeared in Manila, the unknown affection excited great alarm. This was increased by its fearful mortality, 60 per cent, of the early cases proving fatal. Europeans were exempt, with two ex- ceptions, and the Chinese population did not suffer greatly, but among the natives the epidemic was widespread. Thus, in one suburb of Manila, of 25,000 inhabitants, 300 died in the course of eight weeks. Men and women were equally affected and persons of all ages, except young children, were attacked. The disease terminated fatally in from ten days to five weeks after its onset ; but as time went on the propor- tion of recoveries increased, and by the end of March, 1883, it had almost disappeared. The months from October to March are the dry, cool season in the Philippine Islands, although the climate is tropical. Exposure to heat could hardly be considered a cause of this epidemic, but the exposure to cold and damp after an exclusive diet of rice was the probable cause. ^ B. Scheube. Diseases of Warm Countries, p. 190, London, 1903, where a complete literature is to be found. ^Deutsche Arch. f. klin. Med., xsxiv. BERIBERI OR KAKKE. 247 Sporadic cases of beriberi, or kakke, occasionally appear in our hospitals, usually in the persons of Chinese or Malay sailors, or in the persons of travellers from tropical climates who have been exposed to the infection in the place from which they came.^ In 1881 a Brazilian naval vessel entered San Francisco with a large number of the crew affected by the disease. They were sent to the United States Marine Hospital and attended by Dr. Hebersmith, who gave ap interesting account of the circumstances leading to the development of the disease in the United States Marine Hospital Report for 1881. In 1886 a commission was appointed by the Dutch Government to investigate the subject of its nature, and the published report contains the following statements.^ The disease is caused by a micro5rganism resembling the bacillus of splenic fever, though somewhat smaller, which colors with fuchsin and gentian-violet, and can be seen with a power of 560 diameters. These bacilli are found in the blood, lungs, heart, brain, cord, and nerves of the patients, and can be cultivated outside of the body. They are seen in pairs and can be separated from the cerebro-spinal fluid in the acute stage of the disease. The germs infect, wooden dwellings chiefly. They may be conveyed by articles of clothing, and probably enter the body by the lungs. Direct contagion has not been observed. A potent predisposing cause to their reception in the body and to the development of the disease is lack of nutrition, consequent upon exposure to damp and cold, and upon in- sufficient or bad food. This view is supported by the recent studies of Hamilton Wright ^ who has proven that the organism gains entrance to the body through the alimentary canal and multiplies in the duo- denum. Wheate * affirms that the infection is always through a skin abrasion. It is probably a toxin from this microorganism which produces the neuritis, as in cases of diphtheria. Symptoms. — The cases of beriberi are divided into two general classes, according to their severity : There are, first, slight cases, in which the onset is gradual, being usually preceded by a little fever, coryza, and conjunctivitis, which cease when the actual symptoms commence. The patient first notices a loss of appetite, a dull oppressive feeling in the epigastrium, nausea and vomiting, a weak and heavy feeling in the legs, and finds that he tires so easily that he cannot walk as much as usual. The fatigue is soon associated with numbness and pain in the legs, and with a slight cedematous swelling. Then, if not before, palpitation of the heart, and general malaise are felt, and the patient finds it necessary to apply for treatment. An examination then shows some diminution of power in the feet and legs and also in the hands, with loss of tendon reflexes 'Seguin. Medical News, Dec. 11, 1886. Bondurant, Jour. Nerv. and Ment. Dis., Dec, 1900. 2 Deutsche med. Wochen., December 9, 1886. See also Harada, Neurolog. Cen- tralbl, 1885, p. 826. ^ On the Classification and Pathology of Beriberi. John Bale Sons, London, De- cember, 190.3. *N. Y. Med. .Jour., April 6, 1907. 248 EPIDEMIC MULTIPLE NEURITIS. and much tenderness in the muscles, which show a diminished electric excitability. There is never any ataxia, though the patient sways when his eyes are closed. There is discovered a slight degree of anaesthesia of irregular distribution, chiefly in the legs and in the radial nerve region on the forearms. Though the patients look pale, examination of the blood does not show any marked anaemia. The circulation in the extremities is sluggish. The heart is irregular and rather rapid, and the oedema of the extremities indicates a failure of its power. Wallace Taylor^ found that a sphygraographic tracing is characterized by a sudden upstroke in ventricular systole, by a pre- cipitous descent from the apex of the percussion wave, and by dicrotism. Beyond this point these cases, which make up the majority, do not advance. They usually recover in a few days, or at most a month, although a few become chronic and require several months before the cure is complete. There are, secondly, severe cases. These may present three differ- ent types, though Wheate believes that these types are merely stages of the disease. There is, first, the atrophic or dry type, in which, after an onset similar in nature to that in the slight cases, but much more rapid, the weakness develops into a true paralysis associated with marked wasting of the muscles and reaction of degeneration, with great diminution of galvanic excitability. Within a week the patient has to go to bed, and then the paralysis soon spreads from the legs to the arms, and may involve the trunk and even the face. The entire muscular system wastes away until the patient is a mere skeleton. In addition to the motor synrptoms there is great sensory disturbance. The suffering from pain, paveesthesise, and general muscular tenderness is extreme, and the patient lies totally helpless and unable to tolerate the lightest touch. The skin may be glossy. There is usually some anaesthesia, but it is never complete, and it does not involve the trunk. The temperature sense is seldom affected. Pain may be delayed in transmission. There are no gastric symptoms and no oedema. Some cases prove fatal from general exhaustion or intercurrent disease, but the majority recover after a convalescence which lasts a year or more, during which the muscular system is rebuilt. There is, secondly, the hydropic or wet type. In these heart failure appears early and is associated with a marked decrease of arterial ten- sion and much oedema of the entire body, the effusion into the cavities being added to that beneath the integument. The swelling of the oedematous parts conceals the atrophy which is going on in the mus- cles, but this is indicated by the paralysis, which is as severe as in tlie preceding form, and it becomes evident during recovery when the oedema has subsided. There is, thirdly, the acute pernicious type. In this all the symp- toms of the two former types appear in rapid succession, and, in addi- tion, gastro-intestinal symptoms and a suppression of urine combine to make the condition an alarming one. Effusions into the pleura and ^Studies in Japanese Kakke, by Wallace Taylor, M.D. Osaka, 1886. BERIBERI OR KAKKE. 249 pericardium appear early. The pulse becomes small and irregular, and cyano^ indicates the heart failure which precedes death. There are no constant changes in the blood, and leucocytosis has not been found. In this form the disease may run its course in two weeks to a fatal termination. This was the form which chiefly prevailed in Manila, the cases of the atrophic form being the ones which recovered. The severity differs much in different epidemics, the mortality vary- ing from 2 per cent, in Japan to 60 per cent, in Manila. It is usually not above 3 per cent. In all the forms there is some danger of a sudden heart failure, and this is usually the cause of death. Treatment. — As to its treatment, it may be mentioned that qui- nine fails to influence its course and that heart stimulants to combat the dangerous complications, hypnotics to counteract the pain and insomnia, and general tonic treatment have proved of the greatest ser- vice. Change of climate is often attended by recovery. In the stage of recovery electricity and massage have been employed with advantage. CHAPTER XII. MULTIPLE NEURITIS ASSOCIATED WITH OTHER DISEASED STATES OF THE BODY. Multiple Neuritis and Rheumatism, Gout, Diabetes, Tuberculosis, Syphilis, and Carci- noma. Multiple Neuritis of Unknown Origin. Senile Polyneuritis. The occurrence of multiple neuritis in connection with certain other diseases has been observed in so many cases as to have led to the hypothesis that there must be some causal relation between the two conditions. The diseases in which neuritis develops are of a nature to affect the general health, to impair the nutrition, and to produce a constitutional disturbance ; but it becomes evident, from the fact that only a small percentage of the patients suffering from these diseases develop neuritis that some other cause must be active in conjunction with the disease. A congenital or acquired weakness of the nervous system is probably present in certain individuals and predisposes them to this complication, when they fall victims to other affections. The diseases with which neuritis is frequently associated are rheumatism and gout, diabetes, tuberculosis, syphilis, carcinoma, senility, and arterial sclerosis. MULTIPLE NEURITIS AND RHEUMATISM. Multiple neuritis is very rarely to be traced to rheumatism. The so-called " rheumatic " cases of the Germans are cases due to exposure to cold and do not present the characteristic symptoms of rheumatic fever and joint affections. In many cases of neuritis some pain in the joints on motion may be present, but it is very seldom that the red, swollen, oedematous, hot joints of rheumatism are found. A critical review of the cases of multiple neuritis supposed to be rheumatic ^ shows no conclusive evidence of the coexistence of the two diseases. It is not enough to consider pain and tenderness about the rheumatic joint as evidence of neuritis when anaesthesia and paralysis in the domain of these tender nerves fail to appear. Nor is it sufficient evi- dence of rheumatism to find painful joints in cases of neuritis without the constitutional effects of the disease. Hence it seems very ques- tionable whether we are justified in considering rheumatism as a cause of multiple neuritis. It is admitted that local neuritis is not infre- quently produced in the vicinity of a rheumatic joint, as in the cases fully described by Bury.^ Thus, in rheumatism of the elbow, the ulnar ^E. g., the cases cited by Steiner. Deut. Arch. f. klin. Med., Iviii., p. 240. ^ Medical Chronicle, June, 1888. 250 MULTIPLE NEURITIS FOLLOWING GOUT. 251 nerve may become inflamed, swollen, tender to pressure, and paralysis and atrophy of the interossei and thenar eminences may ensue, with anaesthesia in the hand. So, too, peroneal neuritis may result from a rheumatism of the knee. I have seen several cases of this kind. In such cases there is a localized neuritis due to extension of the inflam- matory processes directly from the joint to the nerves passing over it. This cannot be considered, however, as a neuritis due to the state of the blood which produces the rheumatism. Furthermore, an error is often made when the tender, stiif .joints which develop late in the course of neuritis are termed rheumatic. In the later stages of alco- holic neuritis, when the tenderness of the skin and muscles and the pain produced by any motion have led the patient to keep the hands and fingers and toes perfectly fixed and immovable for weeks, an ankylosis of the smaller joints often develops, and this may be accom- panied by some thickening of the articular surfaces and is often attended by thickening of the skin over the knuckles, and by profuse sweating of the hands and feet. This may give rise to a condition not unlike that resulting from chronic rheumatism. It is not, however, rheumatic, but is the natural sequel of inactivity in a joint which is fixed, and is a trophic symptom of neuritis. It is evident, therefore, that it is a mistake to regard neuritis as a frequent result of rheumatism, or to establish any causal relation between the two diseases. In case the two diseases coincide or follow one another, each should be treated separately. In the course of a case of chronic articular rheumatism or of chronic arthritis deformans, multiple neuritis may develop from any cause. I have seen several such cases ; but here, again, there is never any certain evidence of a causal relation between the two affections. MULTIPLE NEURITIS FOLLOWING GOUT. Neuralgia and localized neuritis in single nerves have been fre- quently observed as a complication of gout. Of late, English writers have called attention to the frequency of attacks of multiple neuritis affecting symmetrical nerves on both sides of the body as an occa- sional sequel or accompaniment of this constitutional affection. Sciat- ica and brachial neuritis are frequently traced to this cause. Buzzard ^ in particular, has described several cases in which a sudden attack of pain and numbness in the hands has been followed by weakness and loss of faradic excitability in the muscles of the hands and forearms, and in one case this condition soon appeared in the feet. These patients were gouty persons and acute attacks of gout had preceded or accompanied the nervous symptoms. Ebstein^ and Grube ^ and Remak * have published similar cases, and all agree that a direct relationship ^Buzzard. Harveian Lectures, 1885, Lancet, vol. ii., p. 983. Dyce Duckworth, Gout, London, 1893, p. 247. ^Ebstein. Deutsche med. Wochen., 1898, p. 489. 'Grabe. Miinchcner med. Wochen., 1899, p. 23. ^Keraak. Nothnagel's spec. Path. u. Then, vol. xi., Th. iii., p. 623. 252 MULTIPLE NEURITIS AND OTHER DISEASES. between gout and neuritis is proven. A general peripheral neuritis, such as the form occurring after alcoholic poisoning or after exposure to cold, has not been observed. For this reason the following case, in which both peroneal and anterior tibial nerves were affected and the smaller branches of the nerves in the hands were also involved, may be recorded as of interest : The disease began with a severe itching and burning sensation about the toe-nails and upon the dorsum of the right foot, which soon devel- oped in the left foot also. This itching and burning sensation then extended up both legs to the knee, but was not at first attended by any change in the appearance of the skin, or by actual anaesthesia. After several remissions the symptoms became more intense, and the burn- ing and itching were accompanied by the appearance of a crop of mi- nute vesicles. The feet then became swollen, the skin became glossy, red, and extremely tender to the slightest touch, and the sensation was one of great pain throughout both lower extremities. Any moist dressing upon the surface intensified the pain, but dry dressings or oily applications seemed to give some relief. Large serous blebs formed upon the soles of the feet and about the toes, and there was consider- able eczematous exudation and a scaly appearance of the skin of the entire legs. After this condition had lasted for three months similar itching and burning began in the hands about the fingers with very marked, deep-seated parsesthesia. Fine vesicular pin-point elevations in the skin of the hands also appeared, but these did not go on to the formation of blebs, as in the feet. After two months the skin of the legs had become thickened, cracked, and shiny with crusts all over the surface, and when these had peeled off the surface was red and very sensitive to light pressure. The nails of the toes were thickened, rough, striated, and black. It was said that they had not grown at all during six months. Tactile sensibility was diminished over both feet and both legs, but any touch was attended by great pain. The muscles of the feet and legs had become extremely atrophied and were almost entirely paralyzed ; no electrical examination could be made on account of the extreme sensitiveness. At this time sciatica was com- plained of as high as to the buttocks. The knee-jerks, at first exag- gerated, were subsequently lost. When I saw the patient, a woman of about fifty years of age, seven months after the onset, she was unable to stand or bear her feet upon the floor, but the paralysis had subsided and she could move her ankles ; but could not move her toes. There was very marked tenderness to touch over both legs and upon both feet, and the tenderness of the nerves upon the soles of the feet was extreme. The skin of the feet was dry, scaly, and cracked. The nails were badly discolored, ridged, and dark and rough, excepting near the matrix, where a new growth of nail, about one-half inch in depth, had begun. Any covering ap- plied to the feet caused intense burning and itching, so that she kept the feet entirely uncovered both by day and night. There was noth- ing in the way of local treatment that relieved this itching and burn- MULTIPLE NEURITIS FOLLOWING DIABETES. 253 ing, though every form of local application had been tried. The ball of the foot was red, the rest of the foot white ; but during an attack of pain the feet became scarlet, or sometimes became very much more pallid than usual. It was evident that the condition in the feet was that of erythromelalgia. There was a similar burning sensation and itching felt in the hands from time to time, but there was no apparent malnutrition of the skin. The patient came of a very gouty family and had many indications of the presence of gout, and all these symptoms gradually subsided in the course of a year under treatment directed exclusively to the gouty state, namely, dietetic treatment and alkalies given freely. The coal- tar products appeared to give some little relief to the local symptoms. I have seen the patient several times in the past five years and there has been no return of the aiFection. I have seen several other cases that were quite similar to this in their symptoms and course and have no doubt that they can be classed together as gouty neuritis. MULTIPLE NEURITIS FOLLOWING DIABETES. Neuralgia in the course of diabetes is a very common occurrence, and probably is an indication that the condition of the blood in this disease produces an imperfect nutrition of the nerves. The loss of knee-jerk often observed in this disease (30 per cent, of cases) supports this hypothesis.^ The sciatic nerve seems to be the one more com- monly affected than any other nerve.^ I have seen many cases, and think it important to look for sugar in the urine in every case of sci- atica. Bruns has called attention to the frequency of crural and obtu- rator neuralgia and neuritis in the course of diabetes, and Buzzard ^ has observed several cases of brachial plexus neuritis following dia- betes. In some cases the attacks of neuralgia have been bilateral.^ In some cases they have gone on to neuritis. The nervous symptoms occurring in the course of diabetes may be not only neuralgic pains of a sharp, shooting character, but also various forms of parsesthesia, numbness, and burning sensations. Patients often complain of severe cramps in the legs, especially at night, and these may precede or may accompany sciatica. Multiple symmetrical neuritis of extensive distribution, not unlike that occurring after poisoning by alcohol, both of the paralytic and of the ataxic types, has been ascribed to diabetes, but is exceptional.® Several authors have described cases of so-called neurotabes peripherica diabetica, which have resembled locomotor ataxia, but have gone on to recovery.^ In some of these cases the diagnosis from true tabes was 'Ziemssen. Miinchener med. Wochen., 1885, p. 618. ^Auerbach. Deutsches Arch. f. klin. Med., 1887, p. 484. » Buzzard. British Medical Journal, 1890, vol. i., p. 1421. ^Williamson. Medical Chronicle, 1892, November; Lancet, 1897, vol. ii., p. 138, ^Pryce. Brain, 1893, vol. xvi., p. 416 ; also Pavy, Lancet, July 9, 1904. »Leichtentrett, Inaug. Diss., Berlin,1893 ; PwT, Williamson, Rev, deM^d., 1911, Dec, 254 MULTIPLE NEURITIS AND OTHER DISEASES. difficult, especially as perforating ulcer of the foot occurred/ The tendency to gangrene in diabetes is not traceable to neuritis. The in- tensity of the neuritis, according to Gowers, is not related to the amount of the sugar in the urine. The prognosis is good in all tliese forms of neuritis, as they recover when the sugar disappears from the urine. The treatment will necessarily be directed to the original disease as well as to the complication. MULTIPLE NEURITIS AND TUBERCULOSIS. Cases of multiple neuritis occurring in tuberculous patients require some consideration. If a review of cases of multiple neuritis with au- topsy be made it will be found that quite a number of the patients died of phthisis. There is little evidence to prove that the neuritis was due in these cases to the tuberculous disease. No one has found tubercles in the nerve trunks in these cases, and bacteriological exami- nation has not demonstrated the presence of bacilli in the nerves. For this reason it is not possible to speak of a tuberculous multiple neu- ritis. There is no doubt, however, that the existence of a grave con- stitutional affection may lead to such a general impairment of nutri- tion in the nerves, as well as in other organs, as to predispose such a patient to an attack of neuritis. Hence especial mention must be made of neuritis as a complication of phthisis. It should not be for- gotten, however, that in phthisical patients who have used alcohol freely the neuritis may owe its origin to this poison. Oppenheim ^ has reported cases of multiple neuritis in tuberculous patients which went on to recovery. The following case died : A young girl who was suffering from phthisis took a severe cold and began to have pains in the joints, weakness, and numbness of the lower extremities. The weakness of the legs increased rapidly and was attended by rapid, diffuse atrophy. The same symptoms soon de- veloped in the arms. Four weeks after the onset partial reaction of degeneration was present in all the extremities, and a total paralysis of the legs and nearly complete paralysis of the arms were present. In the course of the disease, which lasted five months, there was little pain, but great tenderness of the muscles and hypersesthesia of the skin. The sensation was diminished in the feet and hands, but this was slight in comparison with the motor symptoms. The paralysis finally attacked the trunk muscles and the diaphragm, while the beginning of rapid heart action was thought to indicate paralysis of the pueumo- gastric nerve. Great prostration, delirium, incontinence, bed-sores, and oedema of extremities, with fever, preceded death. A high degree of degeneration of the peripheral nerves, including the phrenic and pneumogastric, was found at the autopsy, with a mod- erate degree of atrophy of the muscles. Spinal cord and anterior ^Raymond. Lefons sur les malad. du syst. nerv., 1895, vol. ii., p. 331, 22eitscb. f. klin. Med., 1886, p. 230, MULTIPLE NEURITIS AND SYPHILIS. 255 motor roots were normal. The changes in the nerves were similar to those after ^ection, viz., a simple degeneration of the fibres with some increase in the connective tissue, but there were no evidences of changes in the vessel walls, no infiltration with cells. The nerves contained very few normal fibres. The myelin sheaths were found in all stages of destruction even to an entire absence of contents of the sheath of Schwann. Throughout the fibres fatty and granular masses were found. The sheath of Schwann did not, however, show an in- crease of nuclei.^ Such a case as this would have been ascribed, a few years ago, to a tuberculous spinal meningitis. It is not my purpose to dispute in any way the fact that many cases of paralysis occurring in the course of phthisis are due to lesions of the central nervous system and its mem- branes, but I desire to emphasize the fact that a multiple neuritis may produce paralysis in tuberculous individuals, and the importance of appreciating this possibility cannot be too strongly urged, since the treatment, as well as the prognosis, will differ widely, according to the diagnosis made. It has been known for some time that local neuritis may complicate phthisis, and the researches of Pitres and Vaillard have confirmed this fact. They have described (a) a latent neuritis in which lesions in the nerves were found post-mortem, but no symptoms had appeared ; (6) neuritis causing paralysis and atrophy in the muscles, and (c) neuritis with sensory symptoms only. They are inclined to ascribe many of the nervous disturbances arising in the course of consumption to an aflFectiou of the peripheral nerves ; and other writers have confirmed these views and have recorded cases in which all forms of cranial and spinal neuritis have appeared. But here, again, the neuritis must not be termed tuberculous, as it has nothing characteristic of that affection in its pathology. MULTIPLE NEURITIS AND SYPHILIS. There has been much discussion in regard to the existence of a syphilitic multiple neuritis. The admitted frequency of syphilitic exudations in nerve trunks, especially in the cranial nerves and about the roots of the spinal nerves, and the admitted existence of gummy growths in the plexuses and in the nerves, and of syphilitic endarteritis in the vessels accompanying the nerves have made it seem not improb- able that a true syphilitic multiple neuritis might occur. The preva- lence of syphilis has resulted in the development of multiple neuritis in many patients who were syphilitic, and this has also awakened the suspicion that syphilis might be the cause of the neuritis. The syphi- litic poison has been designated by some authors as the cause in these cases ; 1)y others the effects have been ascribed to the post-syphilitic toxin which seems to play such a prominent part in the production of tabes. Cases have been reported in all stages of the disease. It is only in a few of the reported cases, however, that a true syphilitic ^Vierordt. Arch. f. Psych., 1883, vol. xiv., p. 3, 256 MULTIPLE NEURITIS AND OTHER DISEASES. multiple neuritis can be admitted. In the cases of Fordyce/ Spell- mann and Etienne/ and Fry,^ the development of multiple neuritis in the course of the second stage of syphilis, the accompanying numerous syphilitic deposits in the skin and periosteum, and the rapid improve- ment under antisyphilitic treatment give a certain probability to the assertion that these cases were of specific origin. It must be acknowl- edged, however, that such cases are extremely rare. At a discussion at the New York Neurological Society it was found that no one of the members had seen a case of multiple neuritis undoubtedly syphilitic, and a review of the literature has convinced me that many of the cases reported as such were not actually due to the disease. MULTIPLE NEURITIS AND CANCER. Auche * collected from the literature and published a number of cases of neuritis developing in the course of carcinoma, especially of the stomach. Some of these were local neuritis due to a direct extension of the carcinoma into adjacent nerve trunks. Others were general multiple neuritis in the peripheral branches. The lesions were found post-mortem to be of the type of degeneration in the finer nerve fila- ments, not unlike those found by Pitres and Vaillard in tuberculous patients. In some of these cases no symptoms had appeared during life. In others minor sensory symptoms were noticed : parsesthesise, and ansesthesise, and pain. In a few cases the symptoms of a general multiple neuritis, typical sensory and motor paralysis developed. This was seen in cases of Miura and Remak,^ where the autopsy confirmed the diagnosis. In this connection it is to be remembered that proc- esses of degeneration are going on continually in normal nerves in a state of health, that these processes are undoubtedly intensified and regeneration is delayed in conditions of wasting disease, such as tuber- culosis and carcinoma, and also in senile conditions or where the circu- lation and nutrition are interfered with, as in states of extensive arterial sclerosis. Hence, it is not proper to lay too much stress upon lesions of this kind found after death when no symptoms have appeared dur- ing life. And while " latent neuritis " may awaken interest as a pathological curiosity, it is of no importance to the clinician. MULTIPLE NEURITIS OF UNKNOWN ORIGIN. A certain number of cases of multiple neuritis develop after expo- sure to cold. A few develop after unusual exertion, and quite a number occur without any ascertainable cause. It is true that the more closely so-called idiopathic or spontaneous cases are scrutinized, in the light of our knowledge that a great variety ^Boston Medical and Surgical Journal, 1890, p. 39. ^Eevue neurologique, 1897, p. 28. ^Journal of Nervous and Mental Disease, 1898, p. 594. *Kevue de m^., October, 1890. 5 Berliner klin. Wochen., 1891, p. 905, JSTothnagel's spec. Path. ll. Ther., Th, ii., p. 312. MULTIPLE NEURITIS OF UNKNOWN ORIGIN. 257 of causes are competent to produce multiple neuritis, the more likely we are to discover some cause. Thus, many cases which have been reported as idiopathic can now be traced to tuberculous, carcinomatous, gouty, rheumatic, or diabetic conditions known to exist in the patients, but not formerly supposed to have any causal relation to neuritis. There are numerous cases of "idiopathic" neuritis which are preceded by severe gastro-enteritis. Is it not possible that an acute intoxication or ptomaine poisoning capable of causing the gastro-intestinal symptoms may produce a general neuritis? This question, which has occurred to several recent writers on the subject, seems to be answered affirmatively by such observations as are collected by Remak from recent literature. It is possible that in some of these "idiopathic" cases there may be acute infection as the basis of the neuritis, inasmuch as some of the cases develop with an acute febrile attack, a chill, temperature rising to 102° or 104° F., and continuing for several days with the general manifestations of the febrile movement, a rapid pulse, nausea and vomiting, diarrhoea, occasionally jaundice, malaise, catarrhal symptoms in the various mucous membranes, and a general condition of acute sickness, such as is indicative of an infection. Remak ^ has laid great stress upon these spontaneous cases and has argued from the general constitutional disturbance that they are always infectious. He cites cases by Striimpell, Rosenheim, and Putnam, which were attended by a marked swelling of the spleen, in support of this hypothesis of infec- tion. I am inclined to agree with Raymond [Legons, 1897) that very many cases of paralysis hitherto ascribed to spinal-cord disease, but resulting in recovery, are actually cases of this nature, such as the cases recently described by Dana^ as acute ataxia, and many cases of so-called acute poliomyelitis in adults. Symptoms. — The symptoms arising in these cases do not differ in any particular manner from the symptoms occurring in alcoholic neu- ritis, though Schultze is inclined to divide the cases into three categories, according to the prevalence of sensory^ motor, or ataxic symptoms. The general description, therefore, of the symptoms of multiple neu- ritis already given is sufficient for these cases, and if the points that are mentioned under the head of diagnosis (page 201) are carefully considered it will be possible to separate these cases from other affec- tions producing somewhat similar symptoms. The cerebral nerves do not escape in these cases, and, in fact, any of the symptoms which have already been considered under the heads of alcoholic multiple neuritis, arsenical multiple neuritis, and diphtheritic multiple neuritis may develop in the course of these cases. In a few cases optic neuritis has been observed, though, as already stated, this complication is rare. Sometimes painful swelling of the joints has been noticed at the beginning or in the course of the cases of idiopathic polyneuritis, and it has been a matter of discussion whether such joint affection was secondary to the neuritis or an independent rheumatic state. ' Nothnagel'H spec. Pathol, u. Therapie, Bd. xi., Tli. ii. ^Jour. Ncrv. and Mont. Dis., 1901, p. 105. 17 258 MULTIPLE NEURITIS AND OTHER DISEASES. Korsakow^ has affirmed that cerebral symptoms, such as have al- ready been described as occurring in alcoholic neuritis, namely loss of memory, or delusions of memory, or disturbances of memory, or even a state approaching dementia, may develop in the course of these cases. * I have never seen these psychical symptoms, however, in any but the alcoholic types of the disease. SENILE POLYNEURITIS. A form of multiple neuritis occurring in old age and not produced by any of the known causes of neuritis, and not attended by any of the acute symptoms, has been observed. This type of neuritis has been described chiefly by Oppenheim.^ I have seen several cases of this affection. Patients are usually persons over the age of seventy years and are often the subjects of arterial sclerosis. The symptoms develop slowly. There is increasing weakness, with numbness in the lower extremities, and then in the upper extremities ; but the patients do not suflFer from sharp pain or from tenderness along the nerves. There is a progressive condition of paresis, which is much more marked in the hands and forearms and in the feet and legs than in the proximal portion of the extremities, and is rarely attended by any symptoms in the nerves of the head, eyes, or face. In some cases atrophy has at- tended the paresis and there has been a diminution in the contractility of the muscles to both currents. Knee-jerks are lost. There is rarely any ataxia. Very frequently the general weakness leads to a tremor, which, however, may be considered as one of old age. In one case that I observed, where the neuritis occurred in a person who had long suflPered from chronic arthritis, the joints that had previously been stiif and deformed became quite relaxed, so that very abnormal positions could be given to the fingers and toes and to the wrists and ankles, without any perception of pain. In this case there was a complicating gangrene of one leg. It remained for a long time after the symptoms of the neuritis had subsided and the paralysis disappeared. The fixed deformity of the joints returned with the subsidence of the paralysis. In spite of their age, these patients do not always die of the disease, and may recover quite completely. Stein ^ has recently recorded a case in which the muscles of the eyes were paralyzed for a short time. In the treatment of this form of multiple neuritis the importance of good food should be remembered, and the free use of stimulants may be required. iZeitsch. f. Psych., vol. xlvi., p. 475, 1890. Arch. f. Psych., vol. xxi., p. 669. 2 Berliner klin. Wochen., 1893, p. 589. 'Munchener raed. Wochen., 1897, p. 463. SECTION II. DISEASES OF THE SPINAL CORD. CHAPTER XIII. THE SPINAL BLOODVESSELS. The maiu arteries of the spinal cord are three in number. They lie on the anterior and posterior surfaces of the organ along its entire length. The anterior spinal artery is formed by the junction of two vessels which arise from the vertebral arteries and it extends to the lowest part of the spinal cord. The posterior spinal arteries also arise Fig. 106. a. spin, postf a. spin. post. Scheme to show the course and distribution of the terminal branches of the arterial plexus of the pia mater, a. spin. post., posterior spinal arteries; a. spin, ant., anterior spinal arteries ; a. sil., ante- rior median fissure ; rac. ant., anterior root arteries. (After Van Gehuchten.) from the vertebral arteries, but do not often join. They pass down- ward along the surface of the cord on each side near the entry of the posterior spinal nerve roots. (See Plate VIII.) In addition to these main arteries there are a large number of smaller arteries, branches of 259 260 TEE l^PINAL BLOODVESSELS. the intercostal arteries, wliich enter the spinal canal at the sides of the spinal nerves and are distributed to the antero-lateral surfaces of the cord, freely anastomosing with one another. These have been called anterior and posterior radicular arteries. They divide into ascending and descending branches and anastomose freely with the anterior and posterior spinal arteries, making a sort of arterial network about the spinal cord from which numerous small branches penetrate the surface of the organ. The number of these radicular arteries is variable, but they are never present on all the nerves, every second or third nerve having an accompanying vessel. There is a fold of pia mater in the anterior fissure of the spinal cord, and in this fold a large branch of the anterior spinal arteries is usually found running longitudinally. From this branch secondary branches enter the gray matter of the spinal cord and pass down in it, forming the central arteries of the cord. Thus the supply of the gray matter and white matter of the cord is from different branches. Fig. 107 demonstrates this dis- tribution. All of the spinal arteries which enter the cord are terminal arteries like the basal arteries of the brain ; that is, they do not anas- tomose. Hence an embolus in a spinal vessel always leads to an area of softening. The peripheral spinal branches which penetrate the organ from its surface enter with the connective-tissue septa or with Fig. 107 . The distribution of the anterior sjiinal artery and vein to the gray matter of the spinal cord. An injected preparation. (Adamkiewicz.) the nerve roots. They supply the white matter of the cord, and their terminal twigs reach the edge of the gray matter and sometimes enter it, but they do not anastomose with the branches of the central artery. The fine capillaries within the gray matter empty into veins which accompany the central arteries and empty into larger veins which lie in the anterior fissure. The majority of the spinal capillaries, how- ever, empty into fine venous twigs which make their exit on the lateral and posterior surfaces of the cord where a venous network surrounds the cord. This in turn empties into larger veins which make their exit from the spinal canal with the spinal nerves, and thus reach the TEE SPINAL BLOODVESSELS. 261 vena cava. Others ascend to empty into the branches of the jugular vein withi^ the skull. While little attentioO has hitherto been paid to diseases of the spinal bloodvessels and to the results of endarteritis, it is probable that these play a large part in the production of various forms of spinal-cord disease. I have seen a number of cases of injury to the spine in which a sudden development of symptoms indicating a transverse total lesion were followed by a gradual and complete recovery. I have also seen similar cases without injury. I saw a college football player after an injury on the field who was totally paralyzed below the neck. On the third day, when I saw him, he lay with the arms abducted and fore- arms flexed, the deltoids alone not being paralyzed, and he was anaesthetic below the fifth rib. There was retention of urine and priapism. There was no spinal fracture. The prognosis was considered a bad one as the probability of an injury to the cord seemed great. But after a week improvement began, by the end of a month he could move his limbs freely and had recovered sensation and at the end of three months he was quite well. In these cases the only explanation of the symptoms possible was that a hemorrhage either outside or inside of the spinal dura had occurred ; had produced pressure enough to suspend the function of the spinal cord below the lesion ; but had been grad- ually absorbed ; and as the pressure of the clot was removed the spinal functions were resumed. Since the discovery of the characteristic symptoms of syringomyelia it has been possible to diagnosticate hem- orrhages within the gray matter of the cord by the sudden appearance of similar symptoms ; ^ and recent pathological study ^ seems to indicate that many supposed cases of myelitis are really cases of softening in the cord due to thrombosis in diseased bloodvessels, and that some forms of sclerosis may originate in obliterating endarteritis of the per- ipheral vessels that enter the posterior and lateral columns of the spinal cord. It will be necessary in the chapters upon tabes and upon lateral sclerosis to call attention more particularly to endarteritis as a possible cause of some cases of these diseases. 1 Wm. Browning. Medical News, October 7, 1905. ^E/. J. Williamson. Manchester Medical Chronicle, 1895. CHAPTER XIV. POLIOMYELITIS. Poliomyelitis , Infantile spinal paralysis ; Epidemic and sporadic forms . Poliomyelitis, infantile spinal paralysis, acute atrophic paralysis, atrophic spinal paralysis or regressive paralysis is an acute infectious disease, observed chiefly among children but also among adults, char- acterized by sudden complete loss of power in one or more limbs, usually in the legs ; occasionally in the trunk or face ; followed by rapid atrophy of the paralyzed muscles and by an imperfect growth of the limb affected, and attended at the onset by pain -but not by per- manent sensory disorder. Etiology. — The cause of the disease is the presence in the body of a micro5rganism of such minute size as to be invisible under our present microscopes. The microorganism is soluble, and may be obtained from the cerebrospinal fluid or from the gray matter of the spinal cord in inoculated monkeys or in persons suffering from the disease. The disease is infectious and probably contagious, and as the con- tagious material has been found in the mucus of the mouth and nose it is probably disseminated through this mucus and after drying gets into the air and is inhaled. From the nasal mucosa it passes along the filaments of the olfactory nerves through the cribriform plate into the brain. The disease may be carried from place to place by persons not affected. In epidemics it always follows the lines of travel, town after town being invaded. It is supposed that it may also be carried by insects or by animals, as animals of all kinds are susceptible to it. The period of incubation is from 5 to 13 days. It is uncertain how long a person affected may convey the disease to others, but the virus has been found active in the mucus of monkeys 40 days after the active febrile symptoms had ceased and can survive an indefinite time when dried. One attack confers immunity. This is associated with the appearance in the blood of the patient of definite principles which have the power of destroying the activity of the virus ; so that if some of this blood is mixed with the virus obtained from a fatal case and a monkey is inoculated with the mixture it will not develop the disease. This test may determine when a suspected person has had the disease.^ The disease has been frequently observed in connection with other in- fectious diseases. Thus it is an occasional sequel of diphtheria, menin- gitis, measles, pneumonia, scarlet fever, and acute malarial poisoning. It has also appeared during an attack of furunculosis. ' Flexner. N. Y. Med. Kecord, Oct. 28, 1911. 262 MtOLOG^. ^63 Exposure to cold or to a sudden check of perspiration has been sup- posed to be^an exciting cause in certain cases. I have seen several children who were attacked with infantile spinal paralysis subsequent to long-continued bathing in cold water during the sununer. Trauma- tism is frequently assigned as a cause by parents, and a few cases are on record, which I can confirm by my own observations, in which the disease has developed immediately after a severe fall or blow on the back. These contributing causes act by weakening the resistance of the body to the invasion of the germ of the disease. In some cases no cause can be discovered. The frequency with which the disease appears in children who are learning to walk, together with the fact that the symptoms are located much more commonly in the legs than in the arms, has led to the sup- position that a functional hyperemia of the cord in its lumbar region, due to overexertion, going on to a pathological congestion and hemor- rhage, may be an etiological factor. The disease occurs in both sexes with about equal frequency. There is no history of its being inherited. The following table demonstrates that the age of maximum liability is between the first and fourth years, but children at all ages are liable to the disease, and it occurs in adult life : Table V. — Age of Onset. 1st year. M. Sd. hth. 5lh. 6th. 7th. 8th. 9lh. loth. 271 560 436 183 120 67 36 31 17 17 The youngest case on record is mentioned by Collins in a child four days old. The youngest patient in my own records was four weeks old. It has long been noticed (Barlow 1878) that the majority of the cases were seen during the summer months and the following table confirms this. January February March . April , May . June . July . August. September October November December Table VI. — Month of Onset. Barlow. Gowers. Sinkler. 53 Starr. Collins. Lovett. 70 509 202 343 239 Total. 1 14 5 2 8 31 8 2 1 4 16 4 11 9 1 5 31 2 21 4 5 23 4 21 5 1 6 38 5 11 61 10 22 13 122 16 13 109 42 77 36 293 11 13 124 57 109 43 359 4 15 79 41 125 47 311 3 6 45 18 35 39 146 1 2 12 6 10 29 60 2 5 12 3 4 26 1,456 Epidemics of the disease have now been observed in many countries of Europe and in many parts of America. Some years ago such epi- demics were a curiosity and in previous editions of this work a number of these epidemics were fully described. They have however so in- 264 POLIOMYELITIS. creased in frequency and have extended all over the world with such rapidity that description of single epidemics would now require an entire volume.^ They have always occurred in warm weather, usually in the months of July and August, but in Australia they have occurred in the corresponding warm months of January and February. The earliest epidemics of any size were noticed in Sweden and Norway and it has been thought probable that the disease has been spread all over the world by emigrants from Sweden and Norway, though this is not by any means proven. It is only within the last year that the disease has appeared in epidemic form in England. In America these epidem- ics have been wide spread all over the United States, and they have attracted much attention because of the facts that the epidemic type of disease is much more fatal and the cases vary very much in severity, while in the sporadic cases death is rare. The knowledge of the disease has been very much extended by the study of cases appearing in these epidemics. It is probable that many mild sporadic cases occur which are not recognized as poliomyelitis. Pathology. — The lesions that have been described in the earlier text-books were those that are seen in the terminal condition after the disease had been present for some time, and for a long time it was sup- posed that they were limited to the anterior horns of the spinal cord and consisted chiefly in a degeneration of the motor cells in these horns and a replacement of the cells by connective tissue. This was the pathological appearance described by Charcot. Later on the observa- tions of Dreschfeld and others established the fact that the disease began with an acute congestion of the entire gray matter of the cord with hemorrhages from the capillaries and an interstitial inflammation as well as a degeneration of the motor neurones. The study of material secured in the recent epidemics has again modified our ideas of the pathology of the disease and it is now agreed that the microorganism acting as an irritant produces an active conges- tion and inflammation of the meninges and of the spinal cord itself both in the gray and in the white matter, the gray matter being however chiefly affected on account of its greater supply of blood through the branches of the anterior spinal artery. The cerebral axis, the medulla, pons and crura cerebri are often involved as well as the spinal cord. The degree of inflammation in the meninges appears to vary greatly in accordance with the severity of the disease, being so intense in the severer cases as to produce the clinical picture of a meningitis. In the milder cases it is probable that the meninges are merely congested and that the chief pathological changes are limited to the gray matter in the domain of the anterior spinal arteries. In the mildest of all cases, now known as the abortive type, it is probable that the lesion is merely an intense congestion of these vessels, possibly with capillary hemor- rhages which are subsequently absorbed so that no permanent lesion is left. There is every probability that many cases occur of this last type which are never recognized as cases of poliomyelitis. *See Epidemic Infantile Paralysis. Starr, Joum. Amer. Med. Assoc, July 11, 1908. PATHOLOGY. 265 Figures 108 to 111 demonstrate the lesions that are found in cases that succumb soon after the onset. There is a distension of the blood vessels botn in the meninges and in the gray matter of the spinal cord and many minute hemorrhages in the gray matter. There is an infil- tration of the meninges and of the cord with serum and with emigrat- ing leucocytes. The serum fills the lymph spaces about the vessels and about the nerve cells, the leucocytes infiltrate the tissues every- where, cluster about the cells, and make their way into the cells. Throughout the neuroglia there is a great increase of small cells and nuclei which may be so intense as to obscure the other elements. The degenerative processes in the ganglion cells or motor neurones appear early, all varieties of degenerative changes being seen. Fig. 108. Acute poliomyelitis. Death 10 days after onset. Lumbar region. Distension of blood vessels and absence of motor cells is seen. The serious infiltration obscures the outline of gray matter. (Larkin.) The cell may have a cloudy appearance and be slightly swollen, staining more deeply by reagents, the chromophile granules appearing to be larger than in the normal cells, as seen by the Nissl stain, and the nucleus appearing granular. This is shown in Plate III., B (page 34). A further stage of degeneration is shown by the fact that the protoplasm no longer absorbs stains ; the cell is swollen, has lost its sharp outline, has a homogeneous appearance, and the nucleus is faint, as are also the outlines of some of the dendrites. It is probable that in both these stages of degeneration an arrest of the process and a gradual regeneration and a return to the condition of health with re- sumption of the function of the cell is possible. If the process of de- generation proceeds beyond this point, however, no repair is possible, and the function of the cell is forever lost. 266 POLIOMTELlTtS. Then the cell appears to be changed into a swollen, irregular, oi* spherical mass of material ; its protoplasm becomes cloudy and its nucleus is not visible, if stained at all ; the chromophile granules have lost their regular arrangement in concentric rings about the nucleus or in radiating lines toward a neuraxone, and the homogeneous mass is seen to be permeated with vacuoles, the dendrites having dropped off. (See Plate III., c, d.) In the last stage the protoplasm shrinks, the cell body being reduced so as to be no larger than its original nucleus. It stains deeply and has a granular appearance. During the later stages of this degeneration leucocytes may be observed penetrating the Fig. 109. Acute poliomyelitis. The distended vessels, the capillary hemorrhages, the infiltration of the entire gray matter with small cells, and the destruction of motor cells is evident. Contrast this figure with Fig. 20, showing normal appearance at this level. (Larkin.) pericellular spaces and encroaching upon the cell body. Both in the dendrites and in the neuraxone similar degenerative processes may be observed in progress, and they are destroyed before the cell undergoes its final degeneration. (See Plate III., E, r, g, page 34.) While in the majority of cases there is a parallel degree of change in the interstitial tissues and in the ganglion cells, so that there is a shrinkage and progressive destruction of the neuroglia as well as of the nerve elements, there are a few cases in which the cellular degenera- tion is attended by few changes in the interstitial tissues. The degree to which various groups of cells are affected varies greatly at different levels of the cord, and in some groups a larger number of cells may be affected than in others. The extent of the paralysis and the degree of the paralysis in any one muscle will necessarily depend upon the PATSOLOGT. 267 number of groups of cells affected and upon the number of cells de- stroyed in ajAj one group. The result of the atrophy of cells and of the neuroglia is a gradual shrinkage of the entire area of the anterior horn, leading secondarily to a collapse inward of the white columns surrounding the gray matter and of the nervous fibre issuing from the horn through these white columns into the anterior nerve roots. There is also a degenerative Fig. 110. Acute poliomyelitis several years after onset. Upper lumbar region. Diffuse degeneration in both anterior horns and central gray matter with liquefaction of tissue in the left anterior horn. (Larkin.) atrophy of fibres in the anterior nerves. Many of the cells in the anterior horns of the cord send their axones to the antero-lateral column, where they turn upward and downward to pass to other levels and terminate in the anterior horn, thus serving to associate the action of different cells lying at different levels of the cord. These associa- tion cells as well as the motor cells suffer from degeneration, and hence there is a secondary degenerative process in their axones, leading to a shrinkage and slight sclerosis in the antero-lateral column of the cord for a varying distance above and below the seat of the lesion. The extent of the lesion in the gray matter varies in various cases. In a few cases tlie pathological change is strictly limited to the anterior horn, being particularly severe in its peripheral region. In the majority of cases the central gray matter which lies between the anterior and posterior horns is also affected. It is in these hitter cases that the growth of the limbs is intcirfered with in the chronic stage, the central gray matter of the cord having a closer relation to the growth of the tissues tlian other parts. 268 POLIOMYELITIS. Similar lesions have been found in the medulla oblongata, in the pons and crura cerebri, involving all parts, but chieflj affecting the motor nerve nuclei. In addition to the pathological changes in the meninges and cord and brain it has been found that the cerebrospinal fluid undergoes changes. It becomes opalescent and then cloudy. It contains lymphocytes both small and large, and it contains also iDolynucloar leucocytes. It gives a protein reaction when tested by Noguchi's butyric acid test. This condition of the cerebrospinal fluid appears early in the disease and Fig. 111. '^^^jS^^^^^^^^^^^k Poliomyelitis. Higher power, samecase as Fig. 104, showing complete ahsence of cells in the horn; hlood Tessels and scar tissue remaining, and liquefaction of the tissue. The child's thigh and leg were atrophic and totally jiaralyzed. (Larkin.) may be utilized as a means of diagnosis ; the fluid being drawn by lumbar puncture and tested. In a case under Flexner's care the diag- nosis was reached a day before the appearance of paralysis by this test.^ Recent observations ^ have shown that in addition to the nervous lesions in poliomyelitis the general results of any infectious disease in characteristic changes in the liver, spleen, kidneys and other organs are present. These need not be described here. Symptoms. — The disease usually begins, like any acute infectious disease, with fever, sometimes attended by convulsions and delirium, sometimes by considerable pain in the back, body, and limbs ; occa- sionally by a rigidity of the neck and back suggestive of acute menin- gitis ; and by digestive disturbances, vomiting, and diarrhoea ; some- ^Frissell. Jour. Amer. Med. Assoc, July 11, 1911." ^Flexner and Draper. Jour. Anier. Med. Assoc., January 13, 1912. SYMPTOMS. 269 times merely by general malaise. The temperature rises rapidly to 102° or 10^° F., and the patient may have a chill followed by sweat- ing. The temperature remains about 101° or 102° for several days, with slight morning remission, then gradually sinks to normal, the entire febrile movement rarely lasting more than a week. Within two or three days of the onset paralysis sets in. This may be in both legs, or in both arms ; rarely in one limb alone, or in all four extrem- ities. If the child is young and is confined to bed by the fever, the paralysis may not be noticed until the third or fourth day. In older children and adults the paralysis is well developed within twenty- four hours of the onset. It is observed that infants cry a good deal during the period of invasion, and those who are old enough to com- plain say that they suffer from pain in the back and in the affected limbs. This pain may remain for some weeks. There is usually no disturbance of the bladder or rectum, although in a few cases retention of urine has been noticed for a few days. There is no tendency to the development of bed-sores. In a few cases herpes zoster has appeared on the paralyzed limb. There is no complaint of numbness and there is never any loss of sensation, but the limbs are sometimes painful upon any movement, especially in the joints. In some cases the patient is so sick during the first week that ty- phoid fever is suspected, and it is only on the appearance of the paral- ysis that the real condition is recognized. After such an onset the fever and general symptoms subside rapidly, so at the end of a week or at most two weeks the child appears to be well. After the fever with its attendant malaise and digestive disturbances has subsided and the general health has been restored, there remains a paralysis more or less extensive. This paralysis is usually more extensive at the onset than it is destined to be permanently. Thus the child may at first be completely helpless, and later recover power in all but one limb ; or the trunk may be paralyzed at the onset, but not permanently affected. Both legs are commonly affected together, but the final paralysis is usually limited to one limb. Occasionally the neck muscles are distinctly weak, and there may be difficulty in swallowing. This is seen in cases in which the arms are paralyzed, and yet the final paralysis may affect but one arm. The face has been paralyzed with the arms, and the ocular muscles also, but either is a rare occurrence. In a number of cases in which the final paral- ysis has been limited to two or three muscles the original paralysis was widespread, involving all the limbs. These facts should be remem- bered in giving a prognosis in the early stage. Sometimes the onset of the paralysis is not sudden, but there is a gradual increase during a week or ten days, then a stationary period, and then a regression. The subsidence of tlie paralysis begins from a week to two months after tlie onset, and then goes on steadily, but it is not until after three months that it is quite possible to determine what muscles will eventu- ally recover. There is usually a certain amount of permanent paralysis. 270 POLIOMYELITIS. The muscles which are paralyzed undergo atrophy. This is more rapid and complete in those that are to be permanently paralyzed, and there is a change in the size of the limbs which is well marked within a month. The paralyzed muscles are relaxed, never rigid, and show a reaction of degeneration to the electrical tests. The reaction of de- generation consists of a loss of the response in both muscle and nerve to faradic stimulus, and a loss of response in the nerve to galvanic stimulus. The galvanic reaction of the muscle remains, but in such a muscle there is found an alteration of its normal contractility to gal- FiG. 112. Extieme atrophy of the shoulder, arm and forearm in an adult who suflfered from an attack o infantile paralysis at the age of three. vanic currents. For the first few weeks the muscle responds too strongly to galvanism and contracts under the positive pole more quickly than under the negative pole when the current is sent through it. Later the contractility to galvanism is progressively decreased, until in a totally paralyzed muscle it is lost. It may be stated as a prognostic sign that the muscles in which the faradic reaction is pre- served will recover though paralyzed for a time at the onset. Such muscles also preserve their tone, so that they contract when percussed sharply with a hammer. The circulation in the aflPected limb is considerably impaired, and it is cold, blue and flabby, but not oedematous. In some cases the bone is subsequently hampered in its growth, so that the limb is shorter and more slender than its fellow in after life. (See Fig. 112.) While the description just given of an acute onset with fever applies to about three-quarters of the cases of anterior poliomyelitis, there re- SYMPTOMS. 271 mains one-quarter in which there is no febrile onset. Of 166 cases in my clinic, ^0 began with fever and 46 began without fever. Sink- riG. 113. Infantile paralysis, with atrophy and impaired growth of the right leg, and drop-foot; four years after the onset. lor reported 178 with fever, 40 without fever. Collins and Romeiser ^ reported 336 cases with fever and 29 without fever. In these cases the child while in a state of perfect liealth is suddenly paralyzed in one or 'Jour, Ainer, Mod. Aksoc, Ma/ 30, 1908, 272 POLIOMYELITIS. more limbs. It gives no sign of pain, it does not appear to be ill, and the paralysis surprises the mother hy its sudden onset. In these cases the paralysis is soon followed by atrophy and by vaso-motor paralysis. It is not attended by pain or tenderness on motion, and usually de- creases to some extent, leaving the limb, however, in part permanently paralyzed. These two types of onset of the disease are evidently quite distinct from one another. After the onset is over there is a slow progressive improvement up to a certain point, and then the permanent condition of paralysis is found to vary greatly in different cases. The location of the paralysis is usually in the legs, and here two types of the disease may be recognized — the leg type and the thigh type. In the leg type the peronei alone or with the anterior tibial Fig. 114. Infantile paralysis and atrophy of the left arm two years after onset. The partial luxation of the humerus is evident ; and also the main en griffe. muscles are commonly affected, although the posterior tibial group may share in the paralysis or may even be as fully paralyzed as the others. As the paralysis remains, deformities of the ankle and foot will appear, the form of talipes developed depending upon the muscles chiefly para- SYMPTOMS. 273 lyzed. In the thigh type, the psoas and iliacus muscles and the glutei and musclegk about the thigh are those chiefly aifected, the muscles on the inner side of the thigh and the muscles below the knee often escap- ing. In these cases the leg hangs like a flail from the body, and can- not support the weight at all. In some cases nearly all of the muscles of the lower extremity are paralyzed, and the atrophy is uniform throughout the limb. (Fig. 113.) In these severe cases it is not uncommon for the muscles of the back and abdomen to share in the paralysis and atrophy. When the arms are attacked two types of paralysis have been des- cribed, the upper-arm type and the lower-arm type. In the upper-arm type the muscles about the scapula and the deltoid, the biceps, and supinator longus are paralyzed and atrophic, and consequently the motions of the shoulder joint and elbow-joint are seriously hampered. In these cases the shoulder-joint is unduly movable and the head of the humerus falls out of the socket. In the lower-arm type the mus- cles below the elbow are invaded, the flexors or extensors of the wrist and fingers, or both together, are affected, the supinator longus escap- ing. In other cases the interossei and the thenar and hypothenar muscles of the hand are paralyzed, while the long flexors and extensors escape. Occasionally a combination of upper-arm and lower-arm types occurs, in which case the entire extremity is useless. (Fig. 114.) The upper part of the trunk is occasionally involved in the paralysis together with the arms. The muscles of the back and trunk are rarely the only ones permanently paralyzed. In a very few cases the entire muscular system of the body appears to be affected by this disease ; both legs, the trunk, and both arms and even the facial muscles are more or less paralyzed ; but even in these cases a careful examination will show that the degree of the paralysis and atrophy is not the same in all the muscles. The relative frequency of paralysis in different parts of the body is shown in the following table (Table YII.). The paralysis is rarely, if ever, exactly sym- metrical when both legs or both arms are involved. Table VII. — The Distribution of Permanent Paralysis, Duchenne.^ Seeligmuller.^ SinJder. Both legs. Kight leg. Left leg . Right arm Left arm . Both arms All extremities Arm and leg same side Arm and leg opposite sides Trunk . . . Three extremities . 9 25 7 5 5 2 5 11 2 1 14 15 27 9 4 1 2 2 1 107 63 62 5 8 1 35 26 1 22 10 Starr. 54 31 37 11 6 5 9 9 6 4 5 Lovett.''' 130 216 239 5 5 3 15 7 6 2 Total 314 350 372 35 28 9 54 63 17 33 17 1 Archives gdn. de Med., 1864, p. 38. ^Gerhardt's Handlmeh d. Kinderkrankheiten, 1880, vol. v., p. 1. 'Keating's Cyclop, of Children's Diseases, 1890, vol. iv., p. 695, *Lovett. Trans. Amer, Med. Assoc, 1908, p. 1680, 18 274 POLIOMYELITIS. Occasionally the cranial nerve nuclei are invaded by the disease, and as a result strabismus of various types, facial palsy, paralysis of the larynx and tongue, and of the pharynx have been observed. In addition to the paralysis and atrophy, there is a loss of reflex ac- tion at the level of the lesion in every case. The skin reflexes usually return after a time, but the tendon reflexes are absent for a long period, even when a partial recovery of the muscle involved has taken place. Thus the knee-jerk is uniformly absent when the thigh muscles are paralyzed, and the elbow-jerk and wrist-jerk when the arms are affected. Sensation is preserved in almost every case, but I have so frequently observed a permanent hypersensitive condition to painful impressions in the paralyzed limb that I cannot but believe that the lesion in the gray matter aifects the pain-sense tracts in their passage through the cord at the level of their entrance, and has a relation to this symptom. There is marked vasomotor paralysis and lack of vasomotor response in the limb to applications of heat and cold. Deformities of the joints are a common sequel in infantile spinal paralysis. The approximation of articular surfaces is secured, in part, by the normal tension of the muscles, especially at the shoulder, hip, and knee, and hence paralysis of the muscles controlling these joints is attended by relaxation and a greater degree of mobility than is normal. Thus the head of the humerus falls from its socket when the deltoid is paralyzed, and abnormal extension of the knee is often seen in the upper-leg type of paralysis. After some months of paralysis the muscles which are the natural opponents of the paralyzed muscles are apt to become permanently contractured, and this also occasions defor- mities. The action of gravitation on a flaccid part of the limb com- bines with the contracture in the case of the foot to increase the deformity there, and hence all forms of talipes may ensue upon infan- tile paralysis. Deformities of the wrist are also observed, but these are not common. Curvature of the spine from paralysis of the muscles of the back is frequently seen, all varieties having been described. Its most common cause is the shortness of one leg, due to arrest of its growth. Such curvatures differ from those due to bone disease in the fact that they do not persist during suspension of the body by the head and arms. It is one of the most important points in treatment to prevent the development of these deformities. The progress of the disease in any case may be divided into stages. After an acute onset there is a stage of maximum intensity lasting from one to six weeks, and followed by a period of steady improve- ment which may extend from six months to a year. Then follows the permanent chronic condition, in which the normal growth of the child may lead to a slow development of the limb, but not to any change in its power of use. It is very rare for a complete recovery to take place after an attack of infantile paralysis. Even in the lightest cases there is usually some weakness, slight atrophy, and coldness left, and one or two muscles will be particularly feeble. In the majority of SYMPTOMS. 275 cases considerable permanent paralysis remains, requiring the use of apparatus to assist the action of the limb and to prevent deformities. Death occurs during the acute onset, in about 7 per cent, of the cases Fig. 115. Infantile paralysis with atrophy of the right leg. The curvature of the spine is secondary to the shortening of the leg. in epidemics of the disease, but in sporadic cases is very rare, and once this stage is passed there is nothing in the disease to threaten life. It is the chief characteristic of the atrophic paralysis in this disease that it selects certain muscles to the exclusion of others. This selec- tion bears no relation to the arrangement of muscles in the limb or to the conjoint action of muscles in producing any definite movement. It is wholly dependent upon the arrangement of the groups of cells controlling the muscles in the anterior horns of the spinal cord, Jh^ 276 POLIOMYELITIS. exact localization of the disease can be determined by referring the symptoms in any case to Table I. in the chapter on the Diagnosis of Spinal-cord Diseases, page 56. Features of Epidemics. — When the disease occurs in epidemics the symptoms are similar to those occurring in the infectious type of sporadic cases, there being a high fever, 104°, with chill, vomiting, malaise, severe general sweating, severe pains in the back and limbs, sometimes attended by rigidity of the spine and retraction of the head, giving rise to the suspicion of meningitis. Tlie excessive sweating implies an involvement of the vasomotor centres in the spinal cord. Diarrhoea occurs on the second day and continues for two or three days. Delirium is a frequent symptom, occasionally convulsions, and aphasia have been noted. The febrile symptoms last for five or ten days. On the third or fourth day the paralysis appears, usually suddenly and at its maximum extent. When the legs are paralyzed it is not unusual to have retention of urine and loss of control of the bladder lasting several days. When the arms are affected the respiratory muscles may be paralyzed and in the fatal cases death occurs from respiratory paral- ysis. Sometimes the paralysis extends to the neck, throat and face, and even to the eyes, giving rise to the symptoms of bulbar palsy, the polio-encephalitis inferior and superior of Wernicke. If the face is affected all the muscles are involved and the patient cannot close the eye. If the ocular muscles are paralyzed strabismus appears. Pain is a more prominent symptom in the epidemic than in the sporadic cases. The variation in the onset and in the distribution of the paralysis have led writers to distinguish between spinal, bulbar, cerebral, neuritic, meningitic, and abortive forms of the disease. These forms differ some- what in the fact that in each one set of symptoms predominates. They are rarely without common symptoms and do not require separate description. In epidemics the mortality has been from 7 to 10 per cent. ; death occurring from the fourth to the tenth day, usually on the sixth day. In sporadic cases death is very rare. In epidemics abortive cases have been recognized, where the fever has produced great muscular weakness, but has not been followed by permanent paralysis, complete recovery having ensued after two or three weeks. In epidemics there is complete recovery in 25 per cent, of the cases. Various cases of slight indefinite temporary illness with prostration but no paralysis, much like the prostration of gripj)e, have been observed in families and communities where this disease was epi- demic. These are probably cases of very mild type and the diagnosis can be made only by an examination of the cerebrospinal fluid or by an experimental inoculation of the blood already described on page 262. The cases are so insignificant that neither procedure is as a rule justi- fiable, as lumbar puncture is not without danger. Diagnosis. — There is no difficulty in recognizing the disease, and it is hardly likely to be mistaken for anything else. Occasionally a child will be attacked with acute articular rheumatism, and, on SYMPTOMS. 277 account of the pain in the joints, will be unwilling to move the limbs, and thus ipay be thought to be paralyzed. A careful examination should soon demonstrate tlie real condition, for acute rheumatism never causes any atrophy or paralysis, and the local tenderness in the joints, the sweating, and the lack of coldness of the limbs may also aid in the diagnosis. Rachitis, sometimes caused in infants living in healthful and com- fortable surroundings by the use of artificial patent foods containing considerable sugar, may lead to a sudden febrile onset, with much pain and tenderness in the limbs and unwillingness to move. But the child is not really paralyzed, and the tenderness of its bones, the appearance of the gums and the sweating, as well as the lack of limitation of the pain and immobility to one or two limbs, should prevent this disease from being mistaken for infantile paralysis. In some cases of anterior poliomyelitis there is considerable pain felt in the limbs, and some tenderness of the surface and of the mus- cles. The existence of pain during the first two days of the disease occasionally leads to mistakes in diagnosis. Thus Marsh ^ records a case of a child aged five years, who was suddenly attacked with pain in the left leg extending down the thigh to the knee. The limb was flexed, abducted, and rotated outward, and any motion was painful ; hence the case was recorded as acute hip disease, but closer examina- tion showed the hip-joint to be quite freely movable, and after two days, when the pain had passed away, the case was found to be one of infantile paralysis. The fever and general constitutional distur- bances present at the onset had rendered the diagnosis obscure. In painful cases it has been suggested that a neuritis may accom- pany the poliomyelitis. The existence of pain alone is not sufficient to warrant this conclusion, inasmuch as we now know that in the early stages there is a congestion of the meninges and central gray matter of the cord which is sufficient to explain the pain. If, however, the pain continues and tenderness develops in the muscles and nerves, it is probable that a neuritis has developed due to the same infectious agent which has caused the poliomyelitis. It is to be remembered that poly- neuritis is usually a disease affecting the extremities symmetrically and causing drop-wrist and drop-foot ; that the distal parts of the extrem- ities are more severely paralyzed than the proximal parts ; that there is no such selection of muscles paralyzed as in poliomyelitis, and that there are usually sensory disturbances of a permanent nature, anaes- thesia and analgesia, or ataxia, in addition to the pain and tenderness along the nei-ves ; hence in the acute stage of onset a polyneuritis should not be confounded with a poliomyelitis. When polyneuritis accompanies poliomyelitis the clinical picture will be made up of a combination of the symptoms of both affections (see page 205). A condition of weakness of the muscles observed in infancy has been described by Oppcnheim and by Spiller, which they term mya- tonia conr/enita and which resembles slightly infantile paralysis. ^Lancet, January 16, 1897. 278 ■ POLIOMYELITIS. Twenty-six cases have been collected by Haberman.^ The infant makes very few movements from the time of birth, all the muscles seemiug to be weak and lacking in tone. There is no atrophy, but there are no deep reflexes to be obtained. The muscles react to strong electrical currents. The chief symptom is the lack of voluntary move- ment ; the infants lie inert and lielpless, though slight motions are made in response to painful impressions. The children are intelligent, 'the cranial nerves are normal, sensation and the action of the sphincters are normal. Slowly, after many months, improvement begins, the muscles develop and power is gained. In the fatal cases no lesion of the nervous system has been found, but there is an atony of the muscles, which appear pale and poorly developed. As congenital poliomyelitis has not been observed, this disease need not be confounded with in- fantile paralysis. It has a relation to myasthenia gravis and is prob- ably due to some congenital disease of the thymus gland. (Chapter XLIV.) A localized injury of the brachial plexus (Erb's paralysis), causing paralysis of the deltoid, biceps, coracobrachialis and supinator longus is not uncommon in infants, and might be mistaken for infantile palsy. The history of trauma during delivery and the local anaesthesia in the distribution of the circumflex nerve should, however, correct the mistake (see page 180). These possibilities being excluded, and the diagnosis of a poliomye- litis being reached from a study of the symptoms, it is now possible by examination of the spinal fluid obtained by lumbar puncture to make such a diagnosis positive. But further it is also possible by such an examination to reach a diagnosis on the second or third day of the dis- ease when the child shows merely an acute fever without any paralysis. At this time the blood examination shows only a moderate increase in leucocytes such as occurs in any infection. But Flexner has proven that even in this early stage the amount of cerebrospinal fluid is in- creased so that on puncture it flows more freely than normal. It is slightly opalescent and on centrifugalization the sediment' contains a considerable number of small and large lymphocytes and a few poly- nuclear leucocytes. The fluid also gives a protein reaction to the* butyric acid test. Such tests can of course be made only by those who have laboratory facilities. Prognosis. — The prognosis in anterior poliomyelitis is always grave. Patients do not often die of the affection, but they rarely escape a per- manent paralysis in some part of the body. It is true that in the majority of cases the original paralysis subsides, so that there is an apparent improvement of a considerable degree. Thus a patient who has originally been paralyzed in both legs may recover the power in one leg entirely, and may be left with a condition of paralysis in the peronei or in the anterior tibial group of the other leg, so that the terminal condition is very much less severe than that at the onset. ^ Zur DifEerentialdiagnose der poliomyelitis anterior acuta, myatonia congenita und polyneuritis. Berlin, S. Karger, 1908. TMAtmenT. 279 As a rule, the limb that is affected never entirely regains its power, and usually shows some atrophy and shortening ; for the growth of the limbs i^ hampered by the existence of the disease, and hence in a growing child the unaffected limb outgrows the other. It is thought that an electrical examination may afford some ground for a prognosis. It is believed that the muscles which respond to the faradic current three weeks after the onset of the disease will eventually recover, while those that fail to respond to this current at that time will always be somewhat impaired in power. The loss of faradic reaction, how- ever, is not an indication that these muscles will be totally paralyzed, since- the faradic reaction has been known to return in a muscle a year after it has been lost, yet such a muscle never recovers completely its size or power. The prognosis is much better in the cases which begin with fever than in those which do not, and in the epidemic cases after the second week of the disease than in the sporadic cases. In epi- demics, as already stated, from 7 to 10 per cent, are fatal on the sixth day. Treatment. — The treatment of infantile spinal paralysis in the acute stage consists in isolating and keeping the child quiet in bed and applying a mild form of counter-irritation along the spine, which is best done by a paste of mustard 1 part and flour 3 parts, applied in a poultice along the back and removed as soon as the skin is reddened, and then renewed after three hours, so that for at least a week there shall be continued counter-irritation without the discomfort of a blister. The frequent application of dry cups along the spine may be used to produce the same effect. Repeated sponging with alcohol and cold water is indicated in the cases in which the temperature is above 101 ° F., but phenacetin or antipyrine is not to be used unless the temperature reaches 103° F. Dr. Harvey Gushing has recently observed that the administration of urotropin results in the presence of formaldehyde in the cerebrospinal fluid. As the virus of the disease exists in the cerebrospinal fluid, it is well to give a child of three years one grain, of urotropin every six hours during the first ten days. Iodide of potas- sium may be given in 1 -grain dose in the early stage, and moderate doses of salicylate of strontium (2 gr.) or of quinine (^ gr.) may be used for a child of two years. If the child is in much pain or has con- vulsions, bromide of sodium (5 gr.) with or without codeine ( J^ gr.), may be employed as a symptomatic remedy. The general treatment of febrile conditions, a light diet and laxatives, is not to be neglected. In the infectious cases some gastro-intestinal poison may be present, hence free purgation has been urged. The best laxative is castor oil Sss, glycerme 5ij, cinnamon-water TTLx, given with an equal amount of lemon juice sweetened. Rest in a prone position in bed is better than constant lying upon the back. The isolation of the patient should be as complete as in scarlet fever. The nose and throat sliould be sprayed with Dobell's solution every few hours to lessen the chance of contagion to others, and this should be continued for several week§ after the fever has subsided. 280 POLIOMYELITIS. When the acute stage is passed there is little to be done during the second week excepting to nourish the child well and to keep the para- lyzed limb warm. "When the paralysis begius to subside spontaneously it is well to administer strychnine in full dose, -^ gr., three times a day for a child of three years of age. This remedy is best given at intervals, and not continuously, and it is my rule to use it for one week, and then to intermit for three days. The condition of mechanical irritability in unparalyzed muscles, as determined by percussion with a hammer, is a good indication of the degree of effect being produced by the strych- nine, and the drug may be increased until it causes a distinct iucrease in this irritability. It is to be remembered, however, that twitcliing of the limbs or stiffness of the back, usually indicative of an effect of strychnine, is not to be relied upon in infantile paralysis when the muscles are paralyzed. Whether general tonics, such as cod-liver oil, hypophosphites, or arsenic, have any effect of a favorable kind may be left to the judgment of the physician in each individual case. The most important indication during the stage of regression is to preserve the nutrition and function of the paralyzed muscles, and this is to be attained by skilful massage, by hydrotherapy, or by the use of electricity. Massage is of the utmost importance in these cases, and should be given once or twice a day with care, combined with such attempts at active movement as the child is able to make. Among the poorer classes it is well to instruct the mother how to give this, so that it may be given with persistence. The massage should not be of the hardest kind, and yet should be sufficient to stimulate the circu- lation in the limbs and to promote the lymphatic and venous flow. Next to massage mechanical devices which induce the child to make use of the weakened limb are to be employed. A household gym- nasium adapted to each individual case can easily be devised by the physician, and if such exercises are made of the nature of play to the child's imagination, much good will be derived from its own. efforts. Hydrotherapy is also an important aid in treatment. The general circulation in the cold and flabby limb may be aided by warm baths, and it is my rule to have these children play in warm water, tempera- ture 99° F., for half an hour twice daily. This warm bath may be followed by a cooler sponging and brisk rubbing, but cold water should not be employed in the bathing of these children, as the tem- perature of the paralyzed limb is always below that of health, and the vasomotor paralysis prevents the quick reaction which is so bene- ficial in other conditions. Proper protection of these limbs by extra flannel clothing is always advisable. Electricity is a valuable agent in the treatment of infantile paralysis, but a clear statement of its use should be made by the physician to the family. Electricity has no influence whatever upon the course of the disease. It does not affect the lesion in the spinal cord, either to decrease the hypersemia or to increase the nutrition of the nerve cen- tres. Applications, therefore, of galvanism to the spine are absolutely TBEATMENT. 281 useless. But applications to the muscles may be of distinct service in two diifer^t ways — first, by causing their contraction, and thus exer- cising them when voluntary exercise is impossible, and, secondly, by promoting the chemical changes in the muscle that are essential to growth and nutrition. Examination in any case will show a certain number of muscles in the paralyzed limb that respond to faradism. These muscles will eventually recover entirely, yet the tone of the muscle and its strength can be kept up during the period of improvement by means of exer- cise with either the faradic or galvanic current. It is quite well proven that just as exercise of a healthy arm will markedly increase the size of the biceps muscle, so applications of faradism regularly to a muscle that it will contract will increase the size of this muscle ; hence to the weakened muscles which still respond to faradism an ap- plication of the faradic current for about ten minutes once or twice a day will be of service. The majority of the paralyzed muscles do not respond, however, to faradism, and it is time wasted to apply the faradic current to these muscles. They do respond, as a rule, to gal- vanic interrupted currents, the positive pole being placed over the muscle and the negative upon the limb at a short distance above. The interruptions should be made by an electrode held in the hand and provided with a finger-key, and each muscle should be treated for about three minutes daily, fifty to sixty interruptions being made per minute by the finger. The strength used should be the least which will secure contraction in the muscle. When interruptions of the cur- rent do not produce a prompt response alternation of the current may be employed by reversing the current rapidly by means of the pole- changer on the battery. It is to be remembered that in this disease the application of electricity is more painful than in health. It is also to be remembered in applying electricity to children that their confidence must be gained, and that, if they are frightened at the first application, subsequent treatment will result in a continual struggle. It is my custom, therefore, to begin a course of electrical treatment to a child by several applications of the sponges and electrodes while no current is passing, thus accustoming the child to the apparatus and gaining its confidence. After two or three such applications it will be possible to use a weak current, and then day by day to increase its strength until by the end of ten days the necessary strength is being used. In this way a daily struggle, with the result of unsatisfactory and probably useless applications, can be avoided ; and the parents' consent obtained to a course of treatment which they would eventually object to if every application resulted in a struggle. Any intelligent mother or nurse can be taught to give the galvanism or faradism to a child in this manner, and it is best to interest the attendant in the treatment from the beginning, and to instruct her carefully, so that within a week the treatment can be left entirely in her hands. Such an application of electricity is to be made daily or twice a day for two or three years. Spontaneous recovery will have been reached at the 2S2 POLIOMYELITIS. end of the first year, but even after this time these muscles may be brought into a condition of hypertrophy by means of continued exer- cise. When, however, a child is quite able to move voluntarily with some force any paralyzed muscle, it is far better to rely upon voluntary exercises than upon electrical applications. If no eifect is obtained from massage, bathing, and electricity in a muscle at the end of a year there is no use in continuing the treatment of that muscle, as it will never recover, its nerve cells being entirely destroyed. The use of braces plays a great part in the treatment of infantile paralysis in the chronic stage. It is necessary to prevent stretching of all muscles, those which are slightly or not at all paralyzed as well as those which are paralyzed. Hence an eifort must be made to hold the joints in their normal positions from the earliest stage of the disease. It is to be remembered that many weak muscles can do their work only when the limb is placed in an advantageous position or when they are assisted in their action. Many of the muscles have, as part of their function, to keep the joints in place, and this part can be supplied by properly adjusted braces ; hence an appa- ratus may enable the child to use a muscle or to move a joint that it could not do if the joint were unsupported. Again, the result of paralysis of one group of muscles is to allow the joint to be bent by its opponent or to yield to the influence of gravitation, and hence the paralysis is often followed by deformity if a brace is not applied early to correct this tendency. There is no disease in which ortho- pedic apparatus is of more service than in infantile paralysis, and it cannot be applied too early, as it may prevent the development of con- tractures and of deformities. There is no stage in which it is too late to fit a brace, for even if these deformities have occurred tenotomy may be employed to straighten and adjust a joint, and then the limb can be fixed by the brace in a proper position. But every case has to be treated skilfully in accordance with its own condition, and the ready-made braces of the shops are often worse than none. Hence for each case a special apparatus must be fitted under the direction of an orthopedic surgeon, and it is to be remembered that in a growing child such apparatus must be constantly readjusted, its length and size being changed from month to month in accordance with the develop- ment of the limb. In many cases of deformity where there is a strong contracture of a fairly healthy muscle overcoming the weak paralyzed muscle the ques- tion of tenotomy will arise. Such tenotomy will of course result' in a temporary replacement of the deformed joint to its natural position, but unless the joint can be held by a brace in this position, tenotomy alone will be of no permanent service. Hence tenotomy is only to be regarded as a preliminary in some cases to the proper application of apparatus. Apparatus has also been devised (especially in the treat- ment of infantile paralysis of the hands) by means of which weakened muscles may be reinforced by elastic bands so applied as to take the place of the paralyzed muscle. Thus a dropped-wrist or a paralysis TBEATMENT. ■ 283 of the extensors of one side of the wrist can be somewhat relieved by a series ofi" elastic bands attached to finger tips or to rings and to the elbow and running through a bracelet at the wrist. Dropped-foot may also be similarly remedied. Such devices, however, are usually dis- carded after a time, as they are more cumbersome than useful. Ap- paratus is especially applicable to spinal curvature of the paralytic type, and in any case in which the body or back muscles are involved at the onset it is well for the child to wear a corset in order to pre- vent the development of some form of curvature. A thick cork sole will prevent the curvature due to a short leg. It has been proposed to divide longitudinally the tendon of certain healthy muscles and attach one-half to the severed tendon of a par- alyzed muscle about the knee, ankle, wrist, and elbow, in order that the healthy muscle may be made to do the work of the muscle which is paralyzed, and many successful attempts in this direction have been reported. I have seen permanent benefit in a remarkable degree, in a case of paralysis of the peronei, by this method of treatment, in which a part of the posterior tibial tendon was attached to the cut peroneus longus tendon. I have also seen paralysis of the anterior tibial muscle relieved by attaching its tendon to the tendon of the long peroneal muscle. I have seen paralysis of the soleus improved by attaching its tendon to the deep muscles beneath it, and I have seen several cases of great improvement in movements of the fingers and wrists by at- taching the tendons of paralyzed muscles to those which were healthy. This is a method of relief which is rational and deserves wide adoption. Periosteal implantation rather than tendon joining is preferred by some surgeons. It has also been proposed to divide the nerve going to a paralyzed muscle and to graft the peripheral end on to a normal nerve trunk adjacent to it, in the hope that the nerve may regenerate and that the muscle will receive impulses and nutrition through the new nerve. This method is still under observation, though some success has been reported. It deserves a trial in cases where tendon grafting is impos- sible. The effect of the division of the healthy nerve is always to be borne in mind. And if the manifest result of the possible failure of the operation will be to render the patient much more helpless than before it, this operation is not to be advised. Lovett has collected twenty cases, sixteen of which were successful. CHAPTER XV. CHEONIC ATEOPHIC PAEALYSIS. Progressive Muscular Atrophy. Amyotrophic Lateral Sclerosis. History. — A condition of progressive paralysis with atrophy was first described by Sir Charles Bell in 1836 ; it was not referred to a lesion of the spinal cord, however, until 1850, when Aran^ studied it carefully. His statements were added to by Duchenne in 1853. But the form of progressive atrophic paralysis, termed progressive muscular atrophy of Aran-Duchenne, was soon found to be but one of several types of the disease. Duchenne in 1853* described another type, which he named paralysie g6nSrale anUrieure subdigue ascendante, a disease beginning with paralysis and atrophy in the legs, and gradually invading the trunk and the arms, and finally causing death from re- spiratory paralysis. For many years all cases of chronic atrophic paralysis were referred to these two classes, and were supposed to have as a basis an affection of the anterior gray matter of the cord. But between 1860 and 1870 numerous cases of atrophic paralysis were reported in which no spinal lesion was to be found. And grad- ually it became evident that some forms of this disease were dependent entirely upon changes in the muscles. To Friederich ^ and the German school must be given the credit of separating the muscular dystrophies from the forms of spinal paralysis. In 1872 Charcot^ and the French school discovered amyotrophic lateral sclerosis and showed the difference between it and progressive muscular atrophy. And finally in 1882 Dejerine and others brought forward many facts to prove that numerous cases formerly supposed to be due to spinal lesions were really due to multiple neuritis (see page 212). A chronic atrophic paralysis characterized by a slowly progressing weakness, and atrophy beginning in one part of the body and advanc- ing to another part, may occur under the following conditions : First : Chronic anterior poliomyelitis. Second : Amyotrophic lateral sclerosis,- (a) beginning in the spinal ' Tubby. Lancet, March 28, 1903. Assoc, franc, de Chirurgie, 1907, p. 380-574. ^Lovett and Lucas. Jour. Amer. Med. Assoc, 1908, Nov. 14. *Arch. gen. de Mdd., xxiv., 42. *Traite de I'Electrotherapie localis^e. * Ueber progressive Muskelatrophie. Berlin, 1873. ^Lepons sur les maladies du systemenerveux. Paris, 1880, ii., 192. 284 CEBONIC POLIOMYELITIS. 285 cord and ascending to the medulla and pons ; (6) beginning in the medulla and pons as bulbar paralysis, and descending to the spinal cord. Third : Multiple neuritis. Fourth : Muscular dystrophy. In the present chapter attention will be directed to chronic anterior poliomyelitis, and to amyotrophic lateral sclerosis, other conditions causing atrophic paralysis being considered elsewhere. CHRONIC POLIOMYELITIS. Pathology. — The pathological changes present in this disease con- sist of a slowly advancing atrophy in the primary motor neurones of the cord, cell bodies, dendrites, and axones degenerating together. These cells, as already stated (p. 52), lie in groups in the anterior horns and in the central gray matter, and the lesion affects these groups in different degrees. We have several clinical types of chronic anterior poliomyelitis, due to the fact that the lesion may begin in different parts of the spinal cord. In the first type the atrophy begins in the lower groups of cells of the lumbosacral region, and extends to all the groups in the lumbar enlargement. The paralysis begins in the peronei and anterior tibial groups of muscles, then advances to the adductors of the thigh and glutei, and finally invades all the muscles of the legs, but does not ex- tend to the arms. In the second type of the disease (Duchenne's subacute ascending paralysis) there is a progressive atrophy of the cells, beginning in the lumbosacral region and advancing gradually up the cord until all the motor cells are involved. The cells in the sacral region, in which the lesion begins, show a more advanced and complete atrophy than those in the cervical region, though at the end of the disease, in case the patient is not carried off by some intercurrent affection, a large majority of the cells of the anterior horns have disappeared. In the third type (the progressive muscular atrophy of spinal origin of Aran-Duchenne) the atrophic process is limited to certain groups of cells in the cervical region of the cord, especially in the first dorsal, eighth and seventh cervical segments which govern the hands. After it has progressed to a considerable extent in these segments, the groups of cells in the fifth segment, which control the deltoid muscle, and the adjacent groups controlling the biceps, coracobrachialis, and supinator longus muscles, become affected, and then finally all the groups of the cervical enlargement are attacked by the disease. Later the same lesions appear in the lumbar region in the various groups of cells, and tlie paralysis extends to the legs. The lesion in all these types is similar. The difference in the clini- cal symptoms is wholly due to the difference of location, different 286 CEEONIC ATBOPEIC PABALYSIS. groups of cells being first attacked or progressively invaded in the three types of case. Tlie difference between the subacute and chronic cases is only the difference of rapidity in progress, and no hard-and- fast line can be drawn between the two. The exact lesions in these affections consist in changes which are only visible to the microscope. There is no deformity or atrophy of the spinal cord on inspection, and there is no change in the mem- branes of the cord. The anterior nerve roots may appear to be slightly atrophied, and are liable to tear more easily in the process of extraction of the cord. On microscopic examination there is not to be seen any congestion of the cord or any exudation of cells within the substance, but a simple progressive atrophy in various stages in the cells of the anterior horns and of the central gray matter. There is no breaking of the dendrites, no swelling of the axoues, no intracel- lular vacuolization, no hernia of the nucleus, and no diffuse chroma- tolysis. There is no trace of capillary hemorrhages. The cell remains with all its characteristics, but is simply shrunken. In the early stage the body of tbe cell is small and its nucleus is diminished in volume, but the cell retains its polygonal form, merely becoming too small for its lymphatic space. Its chromatophile granules appear small, almost like a mass of dust within the cell, and may be thicker about the nucleus or in the periphery. Collections of pigment in the cell appear in large quantity. Little by little the cell diminishes until the only thing left is a round cellular nucleus with a small nucle- olus or a mass of pigment granules. (See Plate III., F, G.) The lesion attacks not only the large and small cells which give origin to the ante- rior nerve-root fibres, but also the large and small cells which give rise to the association fibres passing into the antero-lateral column of the spinal cord. The cells of the column of Clarke are not affected. As a consequence of this atrophy of the cells there is a correspond- ing atrophy and disappearance of their dendrites and axones. As the dendrites disappear the fine plexus of fibres in the gray matter be- comes less noticeable. As the axones atrophy there is a shrinkage in the antero-lateral tracts of the cord due to a disappearance of the anterior motor root fibres, and also of the association fibres that pass to other levels of the cord. The atrophy of the fibres within this column is diffuse, fibres ' here and there through the entire antero- lateral column being found atrophied ; hence, although no lesion is ap- parent by the Weigert stain, the Marchi method reveals this diffuse degeneration. A picrocarmine stain demonstrates very slight sclerosis of the antero-lateral column adjacent to the anterior horn of. the cord on all its sides. The other tracts are normal. There is marked de- geueration and atrophy in the anterior nerve-root fibres and in the nerves to their termination in the muscles. There is a single atrophy of the muscular fibres in the muscles which are paralyzed. This atrophy consists of a granular disintegration or a disintegration of the ;nuscles, with fine fatty deposits and disappearance of striation and ^ CEEONIC POLIOMYELITIS. 287 simple atrophy of the muscular fibres. Occasionally a single hyper- trophied muscular fibre may be fi^und.^ Symptoms. — The symptoms of chronic anterior poliomyelitis differ in the different types of cases. First, in chronic ascending paralysis we have a slowly advancing paralysis beginning in the peronei muscles, first in one leg, but within two or three months of the onset Fig. 116. The anterior horn in the lumbar region in a case of chronic anterior poliomyelitis. Total disappear- ance of the cells and thinning of the network of fibres within the horn. (Oppenheim.) appearing also in the other leg and causing a sensation of weakness in walking, with a tendency to dropping of the feet. The paralysis corresponds exactly with the degree of atrophy present. After some months the weakness appears in the anterior tibial group of muscles ^ Recent cases with careful pathological observations in this disease have been re- ported by Phillipe and Ceston before the Neurological Section of the International Medical Congress, Paris, 1900 ; also Vjy Raymond and Rickling at the same Congress ; by J. B. Charcot, Tliese de Paris, 1892, and by T. Aoyama, Deut. Zeltschr, f. JSferven* heilk., xxvL, 375, 1904. 288 CHBONIC ATBOPHIC PABALYSIS. and then a fully developed drop-foot appears. The patient walks with difficulty and steps high in order to avoid stumbling over his toes. Any dorsal flexion of the foot is impossible when the patient is lying down, and while standing he cannot lift the toes from the floor. The paralysis and atrophy go on little by little, measurements showing a reduction of perhaps one-quarter of an inch every month in the calf. The mechanical excitability of the muscles is diminished, but not lost, and there is very often a fine fibrillary twitching in the muscles, caus- ing a wave-like movement of the skin, especially when the muscles are exposed to cold or are percussed. Patients may perceive fatigue on slight exertion and may have slight muscular pains in the leg, but they have no . sharp pains and absolutely no sensory disturbances. After several months the paralysis advances to the adductors of the thigh and to the glutei muscles. Then walking becomes even more difficult, going up stairs becomes impossible, and it is with difficulty that the patient rises from the chair. In one case still under my observation two years elapsed before this stage was reached. Then about simultaneously the posterior tibial muscles of the leg and the anterior muscles of the thigh are invaded, and also the psoas and iliacus muscles, and when the paralysis in these muscles is advanced to a moderate degree the patients are no longer able to walk. The measure- ments of the legs are by this time reduced several inches, and not uncommonly the atrophy becomes extreme. The legs feel rather cold to the touch, but there is rarely any oedema, and there is no tendency to bed-sores. The disease may not go beyond this point, and for years the patient may live in a state of paraplegia. There is no tendency to any afi'ection of the bladder or rectum, and the muscles of the back may escape. In the second type — Duchenne's ascending paralysis — there is a tendency to progression upward of the affection. The muscles of the back and trunk become involved, the patient can no longer sit upright in bed or on a chair, and usually the muscles of the shoulders and of the hands become invaded simultaneously, and inability to use the upper extremities gradually develops. The course of the atrophy of these cases may be quite similar to progressive muscular atrophy of the Aran-Ducheune type, and finally bulbar paralysis may ensue. These patients commonly die of some intercurrent disease, pneumonia being the most common cause of death, especially if the respiratory muscles become affected. The third type of chronic anterior poliomyelitis is the type described by Aran and Duchenne. The patient first notices an inability to move the muscles of the thumb of one hand, adduction being imperfect. Almost simultaneously there is a paralysis of the abductor indicis, and consequently the patient cannot separate the forefinger from the middle finger. The paralysis of these small muscles is noticed in the finer movements of writing, playing the piano, buttoning the clothes, or picking up small objects^ and this disability is the first thing which CHRONIC POLIOMYELITIS. 289 attracts the patient's attention. As the disease goes on the paralysis extends to^the other fine muscles of the hand, the interossei, lumbri- cales, and the muscles moving the little finger. And parallel with the paralysis there is an atrophy in all these muscles, causing a flattening of the thenar and hypothenar eminences and a distinct thinning of the hand, producing a sulcus upon its outer side. The thumb can no longer be touched to the tip of the fingers, and as the interossei are flexors of the first phalanx, flexion of the fingers is confined to the second and third phalanges ; the hand, therefore, cannot be closed Fig. 117. Hand and forearm in chronic spinal muscular atrophy, showing especially wasting of tl hypothenar eminences and of the abductor indicis. (Dercum. ) thenar and firmly. A characteristic abnormal position is soon assumed by the hand as a result of this paralysis of the interossei. They no longer aflbrd any opposition to the long flexor and extensor muscles. The long flexors flex the second and third phalanges, the long extensors ex- tend the first phalanx, hence the hand is thrown into a position called main en griff e or claw-hand. (See Fig. 118.) And as the interossei are much atrophied the tendons of the long muscles stand out upon the hand forming distinct cords which may be felt and seen. At the same time a paralysis of the thenar muscles results in a hyper- extension of the first phalanx of the thumb, with some rotation out- ward, so that the ball of the thumb is in a plane parallel with that of the palm. As a rule, one hand is invaded some months before the other, and in several cases I have seen the disease come to a standstill when only one hand was afiected ; but, as a rule, there is a tendency to a gradual progress of the paralysis and atrophy, until both hands are almost use- less. In one case three years elapsed before this condition was reached. But before this stage is reached a progressive atrophy appears in the deltoids, first in one, and then in the other shoulder. In consequence of this paralysis abduction of the arms above the horizontal line is impossible, and when the patient is stripped it is evident that almost all abduction of the arm is produced by rotation of the shoulder-blade by the serratus magnus muscle. The paralysis tlicn extends from the deltoid to the biceps, brachialis 19 290 CHBONIC ATBOPHIC PABALTSIS. anticus, and supinator longus muscles. The supraspinatus and infra- spinatus are also aifected, producing a marked atrophy about the shoulder-blade, and later the teres and subscapularis become affected. In this condition the patient is completely paralyzed in the arms, which hang like flails at the side, and are of very little use, flexion at the elbow being impossible by voluntary effort. As the disease progresses beyond this point the atrophy attacks the muscles of the body about the shoulders. The serratus magnus is paralyzed, and then the shoulder-blade sticks out from the side of the body like a wing. The rhomboids, the levator anguli scapulae, and the lower half of the trapezius become paralyzed, the shoulder-blade cannot be moved, and the muscles of the back become so atrophied that all the bones are visible. Even in the extreme cases, however, certain muscles escape for some unknown reason — namely, the upper part of the trapezius between the occipital bone and the clavicle, the triceps, latissimus dorsi, and the lower half of the pectoralis major. Fig. 118. Atrophy and paralysis with main en griffe in chronic anterior poliomyelitis. As these muscles stand out in contrast to the atrophied muscles about them and contract because of the lack of opposition, deformity of the shoulders and of the neck is quite evident. As the disease extends the muscles of the neck are next invaded, and, as these hold the head in an erect posture, gravitation leads to a falling forward of the head, so that the chin rests upon the chest. This falling forward of the head is usually intensified by the contrac- tion of the sterno-cleido-mastoid muscles, which are not affected by the disease. In the last stage of the case the paralysis and atrophy extend to the intercostal muscles and to the muscles of respiration, so that all respi- ration becomes diaphragmatic. As a rule, patients die from some in- tercurrent disease when the atrophy and paralysis have reached this stage ; but in case they live the paralysis may extend to the legs, cans- CEBONIC POLIOMYELITIS. 291 ing a condition of paraplegia of progressive type such as has been already described in the first class of cases. In a number of cases of both types there has been an extension of the paralysis and atrophy to the muscles of the face, of the tongue, and of the throat, producing a gradual difficulty in articulation, in swallowing, and in all movements of the face, and death by suffocation or choking. The symptoms of this complication are more fully described under the head of bulbar palsy. In all these forms of paralysis with atrophy there are frequent fibril- lary contractions in the paralyzed muscle, which are both spontaneous, and can be caused by percussion or by exposure of the limb to cold. There are also progressive electrical changes in the muscles. A partial reaction of degeneration is the type of change most commonly found. The nerves react to both faradic and galvanic currents, but there is a progressive diminution in the degree of contraction, and hence very strong currents are necessary in order to produce any effect. The muscles respond to the galvanic current very sluggishly, with a so- called "vermiform contraction," and usually react better to the posi- tive than to the negative pole. Finally, all faradic reaction is lost, a complete reaction of degeneration develops, and as the muscle becomes completely atrophied all reaction to galvanism is lost. The knee-jerk is not affected in the disease until the rectus femoris becomes atrophied, when it becomes gradually diminished, and finally is lost. In a few cases the reflex has been increased. As already stated, sensation is normal from beginning to end, and there is no affection of the sphincters. The duration of the disease varies very much in different cases. The most rapid case that I have seen was two years in its duration. But an arrest of the atrophy may occur at any time in the course of the case, and hence the prognosis must be very guarded, as it is unjust to condemn these patients when there is still hope. While the majority of the cases correspond to one of the three types described, it is not to be forgotten that a progressive muscular atrophy may begin in any muscle of the body and advance to any other set of muscles with a most irregular course. Thus Werdnig^ has reported a case in which the paralysis began in the muscles of the back and glutei, then advanced to the neck and throat, and finally to the extremi- ties. Striimpell ^ has recorded a case in which the progress was from the hands to the forearms and then to the shoulder, in distinction from the ordinary progress from the hands to the shoulders. Others have described cases in which both hands and legs have been invaded together. Diagnosis. — As already stated, chronic atrophic paralysis may occur in several diseases. The differentiation of chronic anterior poliomyelitis from amyotrophic lateral sclerosis is as follows : In amyotrophic lateral sclerosis there is an increase in the knee-jerks 1 Arch. f. Psych.,, xxvi., 706. ^Deut. Zeitschr. f'iir Nervenheilk., iii., 6. 292 CEBONIC ATBOPHIC PABALYSIS. early in the disease, an early development of Babinski's reflex — i. e., retraction of the great toe and flexion of the other toes on tickling the sole — and increased mechanical excitability in the muscles that are paralyzed ; a tendency to rigidity in the movement of the legs, and some spastic rigidity of the arms; also, an increase in the tendon re- flex at the elbow and wrist. In other words, the symptoms of lateral sclerosis are added to the symptoms of progressive muscular atrophy, and it is by a discovery of these additional symptoms that the diagnosis is made. The progress of the case is usually more rapid in amyo- trophic lateral sclerosis than in chronic anterior poliomyelitis ; and Fig. 119. Atrophy of the muscles about the shoulder-blades and arms in a case of chronic anterior poliomyelitis. The triceps and lalissimus dorsi have escaped. bulbar symptoms either appear early in the aiFection or precede the paralysis in the limbs. A differential diagnosis from muscular dystrophy is to be made by a consideration of the progress of the case and the succession of im- plication of the various muscles, which is quite characteristic in the different types of muscular dystrophy, and differs from that already described. (See Muscular Dystrophy, Chapter XVI.) In muscular dystrophy there is never any fibrillary contraction in the paralyzed muscles. The atrophy of the muscles is often attended by a deposit of fat, so that there is an appearance of hypertrophy in the muscles that are really atrophied. The tendon reflexes in muscular dystrophy are progressively diminished as the muscles become atrophied. The his- tory of the case, its onset in childhood, its development in a child of a family in which other members ha-ve been known to be affected are CBMNIC POLIOMYELITIS. 293 important facts in connection with the differentiation from progressive muscular atrophy. There is no electrical change in the muscles affected in dystrophy. In the muscular atrophies that are consequent upon neuritis there are usually sensory symptoms and tenderness along the nerve trunks, and there is a history of one of the well-known causes. (See Chap. VIII.) In cases of paralysis of the ulnar nerve the distribution of the pa- ralysis and atrophy to the muscles in the hand may simulate progres- sive muscular atrophy, but it is to be remembered that in ulnar paral- ysis the first and second interossei escape and there is usually some affection of sensibility in the little finger (see page 185). J. R. Hunt^ has described an atrophy of the hands due to a neuritis of the deep palmar branch of the the ulnar nerves occurring in certain workmen, who use the fingers, handling files ; and he considers that this neuritis is the cause of an atrophic paralysis of the hands described by Gessler as an occupation atrophy. The symptoms of progressive muscular atrophy are sometimes the first symptoms to develop in a case of syringomyelia, but the subse- quent appearance of dissociated anaesthesia (a loss of sensibility to pain and temperature, while that to touch is present) and of trophic symp- toms, ulcerations of the skin, etc., together with the usual increase of the knee-jerks, will enable the diagnosis to be made. (See Chapter XVII.) A differentiation between progressive muscular atrophy and the Charcot-Marie ^-Tooth ^ type of atrophic paralysis is somewhat difficult. That disease appears in early youth. It produces a progressive mus- cular atrophy of the peroneal type in the legs and of the Aran-Du- chenne type in the hands, about simultaneously or in rapid succession ; but the paralysis does not extend, as a rule, above the knees or elbows, though occasionally the muscles around the thigh and glutei are in- vaded. The contrast between the atrophied lower parts of the limbs and the well-developed upper portions of the limbs is very marked. Fibrillary tremor and reaction of degeneration are present in both dis- eases, but in the Charcot-Marie-Tooth type sensation is usually dimin- ished in the legs and feet and on the hands. In this disease a few autopsies (Hoffman, Muhlenburg, Dubreihl and Marinesco) have dem- onstrated a peripheral neuritis with some posterior sclerosis, but no affection of the anterior horns. (See Chapter XVI.) Prognosis. — The prognosis in chronic anterior poliomyelitis is a serious one, as the tendency of the disease is to go on to extreme atrophy and death from respiratory or bulbar paralysis. It must be remembered, however, that an arrest at any time in the course of the case is possible. Gowers maintains that the cases in which the disease begins on V)oth sides simultaneously are more liable to a spontaneous arrest. When the disease has once been arrested there is no return of power and no increase in size in the affected muscles, which are per- ^See J. E. Hunt. Occupation Neuritis. Jour. Nerv. and Ment. Dis., Nov., 1908. ''Rev. de MM., February, 1880, p. 97. » Tooth. Dissertation, London, 1886, Neurol. Centralbl., 1887, vi., 16. 294 CEBONIC ATBOPHIC PABALYSIS. manently paralyzed. If the disease starts up a second time after an arrest of its progress the prognosis is most unfavorable, as a rapid course is probable. Death occurs from paralysis of the respiratory muscles, or from bronchitis or pneumonia consequent upon the dis- turbance of respiration, or from bulbar palsy Treatment. — The treatment of chronic anterior poliomyelitis is the same as that of acute anterior poliomyelitis in the chronic stage — namely, generri hygienic measures ; good food, especially of fatty kind ; fresh air, and every means of keeping up the general nutrition of the patient. Exercise, while not prohibited, should be advised in great moderation, and, if the legs are affected, walking should be avoided, so as not to put any strain upon the paralyzed muscles. Any effort which causes fatigue is sure to be followed by an increase in the weak- ness and a more rapid atrophy. Any exhausting disease, such as an intercurrent attack of the grippe, pneumonia, or any infectious fever, or a severe gastro-enteritis is very likely to increase the paralytic symptoms. Thus in one of my patients who had been in a stationary condition for a year an attack of the grippe was followed by a rapid increase in his paralysis. There is no treatment that will arrest the progress of the paralysis. Massage and rubbing are usually prescribed and may possibly aid in keeping up the general strength by increasing the nutrition of the atrophied muscles. I have never seen any benefit from the continued use of electricity, though it is usually prescribed. If it is used it should be with a current only sufficient to produce contraction in the muscles, and very strong or very many long-con- tinued applications are to be avoided on the same principle that exer- cise is to be avoided. The muscles should not be overworked or fatigued. In one case the use of mechanical massage and vibratory treatment at the Zander Institute caused marked improvement and was followed by an arrest in the progress of the disease for a year. General tonics are of distinct service, and I have seen temporary improvement from the employment of strychnine, -^-^ grain, three times a day for four days in the week, alternating with arsenic, gJ^ grain three times a day for the other three days of the week. Some authors recommend the hypodermic use of strychnine, but in a long-continued chronic disease of this kind very little advantage is to be obtained, and the dangers attendant upon septic infection are many Apparatus may prevent deformities and assist weakened joints to bear the weight of the body after walking becomes impossible. AMYOTROPHIC LATERAL SCLEROSIS. This is a chronic progressive form of spinal paralysis characterized by the symptoms of progressive muscular atrophy in the arms and of lateral sclerosis or spastic paraplegia in the legs. It was first described by Charcot in 1872 and was carefully studied by his pupils, Gombault in 1877 and Debove in 1879. It has been called Charcot's disease. But while Charcot believed that it was a disease of the spinal cord AMYOTBOPHIC LATERAL SCLEROSIS. 295 only, it is now known that all the motor elements of the nervous system, from the cortex of the brain to the termination of the nerves in the feet, are inVolved, both the cortico-spinal and spinomuscular elements being simultaneously affected. Pathology. — The pathological condition consists of, first, a gradual progressive atrophy in the motor neurones lying in the anterior horns of the spinal cord, similar to that described as occurring in chronic anterior poliomyelitis. This is sometimes limited to the cervical region, but in long-continued cases the lumbar region is affected and in some cases the entire cord is involved. These changes in the cells are also present in the motor nuclei of the cranial nerves in the medulla and pons. Secondly, in addition to these changes in the spinomuscular element, there are atrophy and degeneration in the cortico-spinal ele- ment which lies in the lateral pyramidal, and anterior median columns of the spinal cord. These changes have been followed upward through the medulla, pons, crus, and internal capsule to the motor cells of the brain. And recent investigation has shown that in the majority of cases of advanced amyotrophic lateral sclerosis there is a degeneration, with atrophy of the larger cells of the second and third layers of the cortex about the fissure of Rolando, which are the neurone bodies of the cortico-spinal elements of the motor system. This degeneration of the cortico-spinal elements begins in the ends of the axones in the spinal cord and advances upward until the entire neurone, of whatever length, becomes affected and atrophied. The sclerosis is a replacement hyper- plasia in the spinal cord, the increased connective tissue taking the place of the atrophied nervous elements. The sclerosis is not wholly limited, however, to the motor tracts in the cord, but is present through- out the antero-lateral columns in the vicinity of the gray horns, as in chronic anterior poliomyelitis. (See Fig. 120.) It is evident, there- fore, that the association tracts belonging to the motor system of the cord are involved as well as the longer voluntary tracts ; hence this disease is one of the most widespread of all the so-called " system diseases " of the spinal cord. The point of beginning of the pathological process varies in different cases. In some cases it is evident that the lesion begins in the cervical region of the spinal cord in the gray matter, and hence the symptoms first appear in the muscular system of the arms. This has been the course in about 60 per cent, of my cases. It was the course in 39 out of 81 cases analyzed by Collins.^ In other cases the lesion commences in the lateral columns of the cord, and then the spastic paralysis of the legs is the first evidence of the disease. This is the second form and has been the history in 30 per cent, of my cases and in 14 of Collins' cases. In either case in the course of the disease both sets of symptoms appear. In the cases that begin in the spinal cord the ten- dency of the disease is to extend to the medulla and pons, so that symptoms of bulbar paralysis ensue and terminate the case. In other *J. Collins, American Journal Medical Sciences, June, 1903. See also Haeuel' Arch. f. Psych., Bd. xxxvii., Th. 1, 1903. 296 CHBONIC ATBOPHIC PABAL7SIS. cases, however, bulbar paralysis is the first disease to appear, and later on, after its symptoms are well marked, the symptoms of spastic para- plegia or of progressive muscular atrophy in the hands indicates that the original disease was not simply bulbar palsy, but amyotrophic lateral sclerosis. This has been the course in 10 per cent, of my cases. It was the course in 25 per cent, of Collins' cases. The lesion Fig. 120. The lesions in amyotrophic lateral sclerosis, lumbar region. A, anterior horn, atrophied and sclerotic, no cells remaining ; B, posterior horn ; C, posterior nerve root ; D, posterior septum ; E, posterior commissure ; F, anterior commissure ; G, anterior fissure ; JI, antero-lateral column, slightly sclerotic ; /, posterior column ; J, lateral pyramidal tract, sclerotic. (Blocq.) is a slowly progressive one, occasionally comes to a standstill for a time, but never has any tendency to recover, regeneration not appear- ing in the degenerated neurones. Etiology. — Very little is known about the causation of this disease. It develops in persons between the ages of thirty-five and fifty years. Cold, overexertion, various forms of poisoning, either by alcohol, arsenic, lead, mercury, or by toxins of infectious diseases, or from auto- intoxication through disturbance of the gastro-intestinal tract, rheuma- tism, gout, and diabetes have all been mentioned as causes without any absolute proof. There is no proof that the affection develops subse- quently to syphilis. Gowers has seen it develop after severe injuries. A probable theory of the affection is that in certain families a con- genital weakness of the motor elements of the central nervous system is present, a fact which receives some support from the occurrence of the disease occasionally in several members of the same family. lu AMYOTBOPHIC LATERAL SCLEROSIS. 297 some cases an extensive endarteritis has been found in the spinal bloodvessels, and this has been supposed to be the cause in these cases. Symptoirfs. — Amyotrophic lateral sclerosis usually begins with stiffness in the muscles and increased reflex excitability, soon followed by atrophy and paralysis in one or both hands, the small muscles of the hands being the parts first affected, as in progressive muscular atrophy of the Aran-Duchenne type. The paralysis then advances to the forearms, though occasionally it appears in the muscles about the shoulders before those of the arms are invaded. Paralysis and atrophy Fig. 121. 'W^M Sclerosis of the spinal cord. The specimen is taken from the border of the lateral pyramidal tract in a case of amyotrophic lateral sclerosis. The upper part of the specimen is normal : the lower part is sclerotic, and in this part few or no fibres remain. go hand-in-hand, and the weakness is proportionate to the size of the muscle, as in progressive muscular atrophy. Fibrillary contractions are present in the muscles from the beginning of the disease, and may be elicited by exposure to cold or by percussion. The order in which the various muscles are invaded corresponds quite closely to that de- scribed in progressive muscular atrophy of the Aran-Duchenne type. When all the symptoms are well-developed in the upper extremities the arms are adducted to the body, the forearms are pronated, and the hands flexed or in the position of majin en grife. There is some resistance offered to passive movements, as the muscles are rigid and spastic even when quite weak. The symptoms appearing in the legs may follow or precede those in the arms ; l)ut, as a rule, a considerable length of time (several months) intervenes between the two. When the disease begins in the legs the early diagnosis is lateral sclerosis. The legs become stiff, and the 298 CEBONIC ATEOPHIC PAEALYSIS. muscles rigid, so that the patient moves them with some difficulty ; the knee-jerks are increased, and ankle clonus and the Babinski reflex can be elicited. The gait becomes a true spastic gait, with short steps, scraping of the foot upon the ground, tendency to overlapping of the knees and feet, and great rigidity in bending of all the joints. The muscles of the legs do not atrophy in the early stage and are not the subject of fibrillary twitches. The symptoms may not begin in both legs at the same time ; in fact, one leg may be quite rigid for several months before the other is attacked. The rigidity gives rise to some aching and discomfort in the muscles, but there are no sharp pains, and there are no sensory disturbances either of the nature of anses- FlG. 122. Extreme atrophy of the thenar, hypothenar, and interossei' muscles of the hands in amyotrophic lateral sclerosis. (Dejerine.) thesia or parsesthesia. The bladder and rectum are not in any way affected. In this condition of partial paralysis the patient may remain for several months, the disease coming to a standstill, or the disease may go on progressively. One patient in my clinic remained in a station- ary state for four years after all these symptoms had developed. If it progresses the spastic rigidity of the legs becomes so intense that walking is impossible, and the patient is confined to the bed. Then the muscles which have hitherto been rigid begin to atrophy, and to show fibrillary twitchings, and the spastic paraplegia is succeeded by a flaccid paralysis quite similar to that which has appeared from the outset in the hands and arms. Sometimes the paralysis extends from the upper extremities to the neck, the head falls forward, the chin resting on the sternum, and the head cannot be raised or turned. AMYOTltOPHiC LATEEAL SCLEROSIS. 299 Some months before complete paralysis has developed in the arms it is common to observe some symptoms of bulbar palsy. A few cases begin with these symptoms, and only develop paralysis of the arms and legs later. The first symptom noticed is a disturbance of speech due to a paralysis of the muscles of the tongue, lips, mouth and palate. The speech is slower, pronunciation being difficult ; the voice becomes monotonous, possibly nasal and indistinct, and finally pronunciation of both Unguals and labials is so imperfect that it is with difficulty that the patient is understood. As this paralysis goes on the tongue becomes thin, is thrown into folds, and shows fibrillary twitchings, and cannot be freely moved and protruded from the mouth. The palate also becomes paralyzed, the uvula is no longer properly elevated, and hence swallowing is imperfect, fluids return through the nose, and attacks of choking are frequent. The face also gradually becomes paralyzed, especially in its lower portions, it is flattened so that there is a mask-like expression, and gradually all expression is obliterated. Whistling, blowing, or kissing is impossible. The mouth is partly open, the saliva runs from the corners, and the act of chewing is in- terfered with on account of the paralysis of the cheeks and on account of the weakness of the muscles of mastication. The muscles of the face may show some fibrillary twitching for some time before they be- come paralyzed. Finally the upper branches of the facial nerve are affected, patients cannot close the eyes, but the ocular muscles do not often share in the paralysis. One or two cases have been reported in which the Argyll-Robertson pupil has been present.^ The inability to swallow may cause death from choking or from respiratory paralysis, or a pneumonia may develop from irritation of the lung by particles of food which are inhaled. One of the characteristic symptoms of the disease is the great in- crease in the muscular irritability in all the muscles that are affected. Percussion, either on the muscle or on its tendon, causes a quick, unusually sharp contraction, and this exaggeration of reflex activity throughout the entire body, both in the muscles that are atrophic and in those that are spastic, is characteristic of the disease, and is not present in progressive muscular atrophy. Percussion of almost any point upon the limbs — on the muscle, tendons, or the periosteum — produces sudden contraction of groups of muscles, and sudden exten- sion of any tendon may be followed by a clonus. Percussion of the facial muscles, especially of the masseter, and of the jaw produces sud- den contractions and even the chin reflex may be so exaggerated as to give rise to a clonus. The atrophic paralysis is usually attended by cramps and also by deformities [main en (/rife or drop-wrist) such as appear in progressive muscular atrophy. If deformities occur in the leg talipes may develop from contracture of the posterior tibial tendons. In the later stages of the disease the muscles of the trunk and ' Schlesinger. Obersteiner's Arbeiten, 1900, vol. vii., p. 154. Ziir Kenntniss atyp- ische Formen der Amyotrophischen Lateralsclerose. S<^<^ CHUOmC ATBOPSIC pa:baltsis. shoulders and neck may become atrophied, so that sitting up or lifting the head is impossible. The electrical examination shows a diminution of excitability both to faradism and galvanism, the muscles that are atrophied requiring a stronger current for the production of contraction. A partial reaction of degeneration may also be found. Tachycardia occasionally develops in the later stage of the disease, as in bulbar palsy, and usually is an exceedingly serious symptom, as it may cause death. The brain is not in any way aifected, excepting in its motor region. Intelligence is preserved, consciousness is not affected, memory is not impaired, and epileptic attacks do not occur. Occasionally, however, as in bulbar palsy, an unusual emotional ex- citability develops, and causeless crying or laughing indicate a weaken- ing of the patient's power of self-control. The duration of the disease varies between two and ten years, de- pending entirely upon the time of onset of the bulbar symptoms, which are the immediate cause of death. If these symptoms appear early or are the first to occur the patient rarely lives more than two years. If the symptoms first appearing are those of lateral sclerosis the case may last for many years. The immediate cause of death is usually dis- turbance of respiration or suffocation from food getting into the larynx, or pneumonia from food being received in the lungs, or from heart failure. The course of the disease when it begins with bulbar palsy is illus- trated by the following case : I. D., aged forty-two years, had been much exposed to wet, to extreme heat, and to cold in his occupation, and had suffered from muscular rheumatism for years. In February, 1889, he noticed double vision, due to a weakness of the left external rectus muscle, and ptosis which first affected the left eye and then the right eye. The ptosis gradually subsided, but the strabismus re- mained. In March, 1889, his speech became thick, and in April he began to have difficulty in swallowing, fluid food coming out of his nose. In May he noticed difficulty in chewing, and his friends saw a change in his facial expression due to a weakness of all the facial muscles. During all this time he felt an increasing weakness in all his movements. His neck had become stiff and his head tended to fall forward and was held with chin projecting beyond the line of the body. On several occasions after March, 1889, he had fainting attacks in which he became pulseless and pale. In June, when I saw him, he was thin and pale, clear in his mind, but feeble in all movements. His eyes were both turned in a little and his left eye looked up. He had corresponding double images, but no nystagmus and his pupils were normal. His optic nerves were normal. There was anosmia on the left side. His sensation in the face was normal, but the muscles of mastication were atrophied, did not react to faradism, and were so weak that chewing was impossible. He moved his jaw with his hand and gave it constant support. His face was expressionless ; he could not whistle ; food collected in his cheeks, but all his facial muscles AMYOTBOPHIC LATERAL SCLEROSIS. 301 could be slightly moved, and reacted to faradism. His palate was paralyzed, ^nd it was to this cause that his defect of speech was due, as the tongue was not paralyzed or atrophied. His neck muscles were weak. There was an atrophic condition of the thenar muscles and interossei in the hands, and marked weakness in all the muscles of the forearm. The deltoid and muscles of the arms were in good condi- tion. His gait was slow and feeble, but not spastic ; but his knee- jerks were exaggerated and anklg clonus was obtained. He con- trolled his sphincters well. During the following year his paralysis increased in his arms and legs, his inability to talk became more ap- parent, and in August, 1890, he choked to death. In the following case the symptoms began in the leg on one side : A woman, aged forty-five years, began to suffer from weakness, fibril- lary twitchings, and atrophy in the right leg, with reaction of degenera- tion in the muscles supplied by the peroneal nerve, in February, 1890. This was supposed to be due to traumatism of the nerve, as she had had an injury. But in April, 1890, an atrophy had begun in the thenar and hypothenar eminences of the right hand. These symptoms increased, and in October, 1890, she was almost completely paralyzed in the entire right side and partially in the left side, there being every- where an atrophic state of the muscles with fibrillary contractions and reaction of degeneration. There was no pain, no sensory disturbance, no bladder or rectal symptoms. The tendon reflexes were increased. During the winter of 1890-1891 the paralysis extended, and she was finally confined to the bed. In April, 1891, the muscles of her throat and face were affected, and in July she died of respiratory paralysis. Diagnosis. — The diagnosis of this affection does not present any difficulties. The muscular dystrophies are not attended by fibrillary twitchings or by increased tendon reflexes or by bulbar symptoms. Bulbar paralysis is not, as a rule, attended by any spinal symptoms or paralysis, or increased rigidity, or increased knee-jerks. If such symp- toms develop in the course of bulbar paralysis it is evident that the disease is a true amyotrophic lateral sclerosis in which the bulbar symp- toms have been the first to appear. Compression of the cervical por- tion of the spinal cord by tumor or by pachymeningitis is usually attended by pain in the shoulders, neck, and arms, by stiffness of the cervical vertebrae, and pain from pressure. Localized compression of the spinal cord, such as occurs in these diseases, if attended by spastic paraplegia is usually attended also by sensory disturbances and by an affection of the bladder and rectum. The diagnosis from syringomyelia is made by the absence of sensory disturbances or trophic affections, and, as is well known, in this disease bulbar symptoms, while occa- sionally developing, are unilateral and do not present the typical fea- tures of a bulbar palsy. Tumors of the spinal cord of the nature of gliomatosis give rise to sepsory symptoms as well as to spastic para- plegia and progressive muscular atrophy. The same is true of chronic myelitis. In any case of ])rimary lateral sclerosis it must be remem- bered that spastic paraplegia often goes on to atrophic paralysis. 302 CEBONIC ATEOPHIC PABALYSI8. Hence many cases of true amyotrophic lateral sclerosis beginning with symptoms of spastic paralysis in the legs are supposed to be primary lateral sclerosis. Time alone can decide between the two affections. In a large number of cases diagnosticated as lateral sclerosis the lesions of amyotrophic lateral scleposis have been found. Treatment. — The only treatment is general tonic treatment. It is well to prevent all strains and exertion and to build up the general health in every way possible. Massage is to be used and electrical applications, but with the same caution against over-fatigue of the muscles by these measures as has been mentioned in connection with the treatment of chronic anterior poliomyelitis. Long-continued warm baths and douches without very great alternations of temperatures are of considerable service in maintaining the nutrition. When the bulbar symptoms appear the patient should be instructed in the art of passing a tube into the stomach, so that as soon as difficulty of swallowing appears nourishment may be carried on in this manner, and thus the danger of suffocation may be avoided. The use of strychnine does not appear to retard the progress of the disease, and, in fact, is objection- able on account of the tendency to spastic paraplegia. CHAPTER XVI. - MUSCULAR DYSTROPHIES. The Classification of Various Forms. Etiology. Pathology. Pseudomuscular Hyper- trophy. Erb's Juvenile Form of Dystrophy. Landouzy-Dejerine Form of Dys- trophy. Muscular Atrophy of the Peroneal Type. Myotonia Congenita, Thomsen's Disease. There are certain forms of paralysis appearing, as a rule, in early- life that are due to muscular and not to nervous disease. These have been named the muscular dystrophies. The muscles appear to be affected primarily by a progressive atrophy. This atrophy involves the fine muscular filaments and proceeds slowly until in the end even the largest muscles may be reduced to a small band of connective tissue. In some cases there occurs a deposit of fat around the atro- phied muscle fibres, filling up the empty spaces within the muscle so that no apparent atrophy occurs. In some cases this deposit of fat is abnormally great, increasing the size of the muscle until it may be two or three times its normal diameter, and producing the appearance of a large muscle where there is really an atrophied muscle. This form is termed pseudomuscular hypertrophy. In other cases there is a true hypertrophy of the muscle fibres. Inasmuch as any form of muscular dystrophy produces at first paresis and then paralysis, it is very natural that these muscular dystrophies should be mistaken for spinal paralyses, though a brief study of their characteristic features will make it clear that no such mistake is warranted. The muscular dystrophies have been classified by Erb,^ who has gathered and analyzed all the facts in regard to this affection, more completely than any other author. Erb distinguished first between cases occurring in infants and those developing in adult life, though this distinction appears to be of little moment, inasmuch as an analysis of a large number of cases demonstrates that the disease may develop at any age. It must be admitted, however, that a very large propor- tion of the cases appear in infancy or childhood, and that when adults are affected the disease usually begins before the twentieth year. Erb further classified these cases, first into those in which there is an hyper- trophy of muscular tissue, either a pseudohypertrophy or a true hyper- trophy of the muscle fibres, and, secondly, those in which there is a progressive atrophy without any deposit of fat. This classification, while of value clinically, appears to rest upon no essential pathological factor, for all grades in the process are possible. In some cases the deposit of fat is excessive, causing pseudohypertrophy ; in other cases iDeut. Zoitschr. f. Nervenhk., i., 13 and 173, 303 304 MUSCULAR DYSTROPHIES. there is a deposit of fat taking the place of atrophied muscle, but without producing any apparent change in the size of the muscle, and in many cases of extreme atrophy of the muscles fatty deposits are found between the muscle fibres ; hence this classification does not rest upon a pathological basis. And we may find all these conditions in different muscles in the same patient. A further classification has been based upon the distribution of the affection in different muscles. Here, unfortunately, the different types have been named after the observers who happen to have first carefully described them. Thus we have the Duchenne type of pseudohyper- trophy, and we have the Erb type of juvenile dystrophy, in which the muscles about the shoulder-blades and arms are chiefly affected. We have the Sachs-Hoffman type, in which the affection begins in the peroneal muscles of the legs. We have the Landouzy-Dejerine type, in which the muscles of the face and arms are affected, and we have the Charcot-Marie-Tooth type, in which legs and forearms and back are affected, thighs and arms and face escaping. It is true that the various cases observed conform in many particulars to one of these different types, but there is no essential difference between them in the causation or in the pathology. And intermediate types have been observed not conforming to any of these. Furthermore, it is found that one type may progress and gradually merge into another. It is evident, therefore, that these attempts at classification in the discus- sion of muscular dystrophies are not wholly satisfactory. Etiology. — The causation of muscular dystrophy is a matter of considerable interest. The disease is undoubtedly a disease of develop- ment, and from its marked tendency to appear in very early life, to attain a certain status, and then to be arrested without any tendency to repair, or else to progress steadily until it causes an entire destruc- tion of the muscular system, it seems probable that it must be traced to an inherent defect of development in the muscles affected. It seems as if these muscles were not endowed with the proper vitality, and, after a short life, wither and die. We have evidence in other organs of the body of this difference between the life-period of a special structure and that of the entire body. Thus the life-period of the first and even of the second teeth is a comparatively short one as related to the life-period of the indi- vidual. The life-period of the hair, of the reproductive system, of the eye and ear, and of the brain may be less than that of the entire individual. We see in the study of optic atrophy and of auditory atrophy that there are some families in which these diseases develop, even in early life, without apparent cause, the only explanation being that these nerves have not the power of survival which other nerves have. We recognize the existence of senile atrophy of the brain in old age, but we must admit that the term " old age " cannot be fixed within definite limitations, for some individuals become old at the age of sixty years, while others are young and vigorous at the age of eighty years. I once had under my observation a lady of the age of PATHOLOGY. 305 ninety-nine years, all of whose visceral functions were as vigorous and active as they were at the age of seventy, but whose brain for ten years had been practically dead, powers of perception in eye and ear being suspended, powers of memory being almost obliterated, and powers of reasoning being reduced to the level of a child of three years. Thus, it is evident that the organism as a whole may outlive any one of several of its organs, provided these organs are not essential to life. And the duration of vitality of any organ varies greatly in different individuals. This fact is particularly evident in regard to the reproductive system both in males and females, and it is equally apparent in regard to the muscular system. For it is a matter of comment that the muscles of old people wither and become feeble long before their death.^ It is quite evident from the consideration of these facts that the explanation of muscular dystrophies is to be found in an inherent inability of survival of certain muscles in certain individuals. In other words, that the disease is one of congenital defective power of evolution. This explains to a certain extent the frequency with which we find that several members of a family are affected by this type of disease. It is true that isolated cases are not uncommonly met with where careful investigation fails to reveal any member of the family or any member of any collateral branch similarly aflTected. But in the majority of cases it is found that if the patient has no brother or sister similarly diseased, or has not inherited it directly from either parent, he will have several cousins who have suffered, possibly several second cousins who have suffered, and it is not uncommon to be able to trace the disease through four or even five generations. Whether extraneous causes, such as undue muscular effort, exposure to cold, the unfavorable effects of the occurrence of infectious diseases, falls, and blows, have anything to do with setting up the disease in those who have an inherent tendency toward weakness of the muscles is a matter which may be open to doubt, though these factors have been mentioned as exerting a causative agency. , _ Pathology. — The changes found in the muscles in this disease are those of a simple progressive atrophy. It will be remembered that the muscle fibre, which may be 5 cm. long and from 15 to 55 mm. broad, has a striated appearance due to the existence of two types of structure within its substance, one of which interrupts the light more completely than the other. Each muscle fibre is surrounded by a sheath of connective tissue (the sarcolemma) within which lie nuclei or long oval cells from which the muscle fibre itself has developed. These muscle fibres, surrounded by the sarcolemma, are really made up of filjrils of very fine structure, each fibril originating from a single cell, and the fibrils are separated from one another by a structure termed the sarcoplasma ; hence, under the microscope a muscle fibre has a double striation, a transverse striation, and the longitudinal fibrillar structure. These fibres are gathered into greater or lesser 20 ^ See Gowers, Abiotrophy, London, 1903, 306 MUSCULAR DYSTROPHIES. bundles, forming the muscle, and they are supplied by nerves whose terminal filaments spread out upon the surface of the fibre in the so- called motor plate. Within the muscle and within the fibres lie oval bodies called the muscle spindles which contain the sensory filaments through which the muscular sense is received. When a muscle undergoes atrophy we may find, first, a simple reduc- tion in the calibre of the individual fibres. (Fig. 123.) There is a Fig. 123. Fig. 124. fi?m-rf^ mjmmmmii W^M'ji i^mV'^fg:'::.::'^/:^ ^mmm^mm W':^ Atrophy of a muscle, with increase of nuclei. X250. (Schmaus.) Atrophy of muscle, with deposit of fat about the atrophied fibres. X 250. (Schmaus.) thinning in the entire fibre, which becomes narrower and shorter, pre- serving until the end its striated appearance, though it becomes pale and sometimes a little pigmented by the deposit of coloring matter. Secondly, in other cases there is a degeneration of the muscle, ^con- sisting of a swelling or oedema, followed by an albuminoid or fatty de- generation of the muscle fibre, resulting in the deposit of small fatty globules and granular masses within the sarcolemma. As the muscle becomes degenerated the individual fibre contracts, obliterating the striated appearance, leaving the muscle with a homogeneous, smooth, or yellow tint. As the process goes on all trace of the muscular sub- stance may vanish, leaving the sarcolemma containing only fatty globules. Sometimes waxy deposits may occur within the muscle, with compression and obliteration of the fibres, but this is not com- mon in the dystrophies. Occasionally as the muscle undergoes progres- SYMPTOMS. 307 sive atrophy its sheath is filled up by an oedematous exudation. But usually there is merely an increase in the cells of the sarcolemma. Under some circumstances there is not only within these empty sarco- lemma sheaths a fatty remainder of the degenerated muscle, but be- tween the sheaths there is an actual deposit of fat of new formation. (See Fig. 124.) Under these circumstances not only is the space formerly occupied by the normal muscle filled out by fat, but the fatty deposit may increase the actual diameter of the muscle ; and if this goes on to any extent pseudohypertrophy of the muscle will be pre- sented. Along with the fatty deposit there is usually an hypertrophy of the connective tissue in the muscle, so that when the process be- comes extreme the muscle is turned into a mass of fat separated by trabecule of connective tissue. If, after the course of time, the fat is reduced in volume and is finally absorbed the muscle will have as its only relic this connective-tissue strand, and as this contracts longi- tudinally it will cause a shortening of the atrophied muscles. Thirdly, along with the atrophy we find occasionally a true hyper- trophy of individual muscle fibres. The fibrils are increased by true formation of muscle tissue from their nuclei within the sarcolemma, and not only increased in number, but also increased in lateral diam- eter, so that giant muscle cells and giant muscle fibres quite similar in structure to a normal fibre are evident. Any of these processes may go on alone or may be combined with others in the process of muscular dystrophy. The connective-tissue changes which were for a time thought to be primary have long been known to be of a secondary nature, a hyperplasia of connective-tissue within the muscle occurring just as a hyperplasia of neuroglia occurs in the spinal cord to take the place of atrophied substance. In the connective-tissue near to the ends of the muscle new formations of thick tendons may be seen. These changes in the muscles are found in all forms of muscular atrophy both spinal and peripheral. It is thought that in the dys- trophies the development of hypertrophy of the muscle fibre in con- nection with the atrophy is a peculiar feature of the disease. It is not often that deposits of fat are found within the muscles in the spinal and peripheral types of atrophy. Symptoms. — From the clinical standpoint we recognize the follow- ing types of the disease, the symptoms of which difier distinctly from one another : 1. Pseudomuscular hypertrophy of Duchenne. 2. Erb's juvenile dystrophy. 3. Landouzy-Dejerine type of dystrophy. 1. Pseudomuscular Hypertrophy. — This disease begins in children between the second and the seventh year, and has often made consid- erable progress before it is brought to the attention of the physician. It begins by a gradual increase in the size of the calves of the legs, which arc at first subjects of pride to the mother, but soon awaken her apprehension because of being out of proportion to the growth of the rest of the limbs and because they appear to be attended by a certain 308 MUSCULAR DYSTROPHIES. clumsiness of gait. The child, in learning to walk or in walking, be- gins to stumble, to go up stairs with some difficulty, to get tired too soon after walking or running, and to walk with a peculiar gait. This gait is characterized by a dragging of the legs, which appear to be raised from the ground with difficulty, and in order to drag the leg the child throws its body from side to side and raises its pelvis in the Fig. 125. Pseudohypertrophic muscular dystrophy. Four brothers, aged twelve, eleven, eight, and seven years. The calves and the anterior surface of the thighs are hypertrophied. The muscles of the back are atrophied. The eldest has so much weakness of the muscles of the neck that he cannot hold up his head. (Curschmann, Klin. Abbildungen.) act of walking. As the disease progresses great difficulty is experi- enced in rising from a chair or in getting up from the floor, and these children soon learn to aid themselves by the use of their hands, which give support where the muscles of the legs are weak. When the dis- ease is well advanced these children rise from a lying to a standing posture in a peculiar manner that is characteristic of weakness in the muscles of the calves, quadriceps femoris, and glutei muscles and muscles of the back (Figs. 126 to 130). This typical method of getting on the feet, together with the peculiar waddling gait, is sufficient to make a diagnosis of the affection. When the disease is fully devel- oped inspection shows an hypertrophy of the muscles of the calves (Figs. 131 and 132), an hypertrophy of the anterior muscles of the thighs (Fig. 125), and an atrophy of the glutei muscles. An atrophy of the muscles of the back produces a condition of forward curvature P8EUD0M UBCULAR H YFEllTKOFHY. 309 of the spine and causes the child to throw his shoulders back in order to preserve iiis centre of gravity. Even in the early stage of the dis- ease, before the muscles about the scapulae are affected, it is found that Fig. 126. Pseudohypertrophic paralysis. The act of rising. The child rolls over on the face. if a plumb-line be dropped from the scapula it will clear the buttocks. This is not possible in a state of health. As the disease advances upward the arms become affected, the iu- fraspinati are the first to be hypertrophied, so that the child appears to Fig. 127. Pseudohypertrophic paralysis. The act of rising. The child raises the trunk on hands and knees This position shows the weakness of the muscles of the neck and the atrophy of the arms. have a very prominent pad on the back of the shoulder-blades. The supraspinatus and deltoid may become hypertrophied also, and some- times the triceps and biceps as well, but in the majority of cases these muscles atrophy without any deposit of fat. While these 810 MVSOVLAR DrsTROPStES. muscles hypertrophy otlier muscles about the shoulder become atro- phied; thus the rhomboids and levator anguli scapulse, and also the serrati become atrophied and paralyzed, and hence the shoulder-blades Fig. 128. Pseudohypertrophic paralysis. The act of rising. The child raises the trunk by bringing the foct forward and extending the legs at the knee. Fig. 129. Pseudohypertrophic paralysis. The act of rising. The child raises the shoulders by supporting the weight on the hand pressed upon the knee. PHEVDOMVHCULAR UYPERTROPEY. 311 stick out from the back like a pair of wings (Fig. 131). Biceps and triceps ma/tlien in turn atrophy, rendering all motions at the shoulder- joint imperfect and making flexion and extension of the elbow very- weak. As a rule, the muscles of the forearms and of the hands are not affected. After the muscles are considerably atrophied, with or without the production of pseudohypertrophy, a contraction occurs, causing de- FiG. 130. Pseudohypertrophic paralysis. The act of rising. formities in the joints. Thus talipes equinus is first produced, then a permanent flexion of the legs upon the thighs and the thighs upon the pelvis. Curvature of the spine, which can no longer be corrected by suspension, develops and often leads to lateral curvature by the un- equal contraction of the muscles of the back upon the two sides. Contractions may occur also in the arms, producing a flexion of the elbow and interfering with passive movements of the shoulder-joint. The electrical contractility of the muscles is gradually reduced as the muscles atrophy, but there are no changes of the nature of reaction of degeneration. There are no fibrillary contractions in the muscles. Sensation is not at all impaired. The reflexes remain normal until the muscle becomes too much atrophied to respond. These facts enable a differential diagnosis from anterior poliomyelitis of the acute or chronic typo to be easily reached. The course of the disease is slow and progressive through four to ten 312 MUSCULAR DYSTROPHIES. years, by the end of which time the child is rendered helpless, cannot stand or sit up in bed, or feed itself. The disease is not a fatal one, but in the enfeebled invalid state intercurrent diseases, especially of the respiratory organs, are the usual cause of death. 2. Erb's Juvenile Form. — This type begins, as a rule, between the ages of twelve and sixteen ; but cases have been observed which did not develop until after the age of twenty. In this form of muscular Fig Fig. 132. ^r-^ Pseudohypertrophic paralysis. The calves are large ; the back is weak and curved forward. Deltoids and triceps are atrophied. Serrati are weak, hence the scapulae protrude. Pseudohypertrophic paralysis. The lordosis is well marked, and the attempt to preserve the balance by throwing the shoulders back is demonstrated. dystrophy the muscles of the shoulder are first affected, the pectorals, trapezii, latissimus dorsi, rhomboids and deltoid muscles become grad- ually hypertrophied, but at the same time are weakened. Peculiar deformities of the chest often develop, the shoulders being thrown for- ward and the scapulae protruding. As the disease progresses the biceps and triceps become atrophied, but the muscles of the forearms and hands ERB'S JUVENILE DYSTROPHY. 313 remain in their normal condition. Later on, if the disease does not come to a standstill, the muscles of the back become atrophied, lordosis appears, with deformities of the spine and difficulty in walking. Later the glutei and anterior muscles of the thigh are affected, either atro- phied or hypertrophied, and finally the calf muscles and peronei become implicated, causing total paralysis with talipes equinus. In the majority of cases hypertrophy is more marked in the deltoid, infraspinatus, sar- FiG. 133. Pseudohypertrophic paralysis ; five years after the onset. Muscles of arms and legs greatly hyper- trophied. Both feet contractured and in a position of talipes. (Curschmann, Klin. Abbildungen. ) torius, and gastrocnemii. It will be seen that in the later stage of the disease the terminal condition is similar to that occurring in pseudo- hypertrophy, a fact which makes it evident that the only difference between these two types of dystrophy is in the direction of progress in the muscles involved. 3. Landouzy-Dejerine Tsrpe. — In this type, which develops usually 314 MUSCULAR DYSTROPniES. Fig. 134 / \ in early childhood, but occasionally in adults, the feature is the appear- ance of the muscular atrophy in the face. It is true that both in pseudomuscular hypertrophy and in Erb's juvenile dystrophy the dis- ease may eventually extend to the face, producing all the symptoms seen in the Landouzy-Dejerine type. The atrophy begins in the orbicularis oris and ex- tends to the risorii, to the levator menti, and to the finer muscles of the face about the mouth. The result is that the lips become weak, cannot be firmly closed, and the mouth is habitually open and the lips are slightly everted, producing a peculiar facial appearance that has been termed the "tapir mouth." This form of paralysis aifects the act of speech. Unguals and labials being defectively pronounced, the ability to whistle, or to purse the lips, or to apply the lips firmly to a glass in drinking being lost. Frequently saliva runs from the patient's mouth, as in facial palsy. The affection is bilateral. The muscles about the eyes, as a rule, escape, so that winking is not interfered with, and the muscles of mastication and of deglutition are not involved. As time goes on in these cases the muscles of the shoulders and body, and finally of the legs, become involved, and the terminal stage of the disease is not unlike that in the two types already described. Fibril- Erb's juvenile dystrophy. The disease , , • , i • . i i began at the age of sixteen ; photograph lary twitchmgs are not present, the elec- made at age of thirty. Supra- and infra- trical reactions are normal, but are pro- spinati, and gastrocnemii hypertrophied. grCSSivcly lost. There is UO disturbance rrapczn, deltoids, biceps, triceps, latissimi o .1 .i. -n/r 1 • i • 1 •!• dorsi, serrati magni, erectores spina, all 01 SCnSlblllty. McchaniCal CXCltabuity muscles of thighs, and glutei are atro- of the musclcs is gradually lost as the phied. (By permission of Dr. G. L. i 1 1 Walton.) muscles atrophy. While these three types of muscular dystrophy are sufficiently different from one another to be easily recog- nized, it must be admitted that many patients suffering from the dis- ease present symptoms common to two or more types. Even Erb admits the occurrence of transitional forms of dystrophy, and shows that the course of the disease may present variations in various members of the same family who are affected. In the terminal stage it is not always easy to ascertain to which type a patient is to be assigned. The diagnosis of this affection from bulbar palsy is not difficult, K. LANDOtlZY-DEJEniyE DYSTROPHY. Sli because in dystrophy the tongue and muscles of swallowing are not involved. ■/■ Prognosis. — The prognosis in muscular dystrophy is unfavorable. In all forms of the disease there is a tendency to slow progress, to an Fig. 135. Progressive muscular dystrophy, atrophic type of Landouzy-Dejerine. Age, nine years ; duration, seven years. Face, arms, and legs extremely atrophied. Face expressionless; pectorals and deltoids wholly atrophic ; thighs atrophied. The forearms and the legs are normal. (Curschmann.) extension of the aifection from muscle to muscle until finally a state of helpless paralysis with extreme emaciation is reached. This, how- ever, is not an invariable occurrence, as I have seen several patients in whom the disease has come to a standstill and who have remained in a stationary condition for six or eight years without being incapacitated from walking. The prognosis as far as life is concerned is good, pro- vided complicating diseases, especially of the respiratory organs, are 316 MUSCULAR DYSTFOPHIES. prevented. These patients are peculiarly liable to develop tuberculosis of the lungs or pneumonia or bronchitis from disability in respiratory movements. Occasionally the diaphragm is involved and the patients die of respiratory paralysis. Treatment. — Treatment of muscular dystrophy must be by general measures to support the health and nutrition of the individual. A country life in the open air, with good food and moderate exercise, is advisable, and the systematic use of massage and gymnastic exercises, not carried to the point of fatigue, will often tend to reduce the rate of progress in the muscular dystrophy and in some cases to cause an arrest of its progress. Thus the patients alluded to in whom the disease has come to a standstill have had most skilful massage for years, daily for about one hour. Electricity has been used for the purpose of exer- cising the atrophied muscles, and when massage is impossible it may give good results. When the contractures have occurred the question may arise of the propriety of tenotomy, but in several patients in whom I have had it done it has not afforded any permanent relief, but, in fact, has been attended by an increase of the disability. The appli- cation of corsets or plaster-of-Paris jackets to correct the deformity in the spine is not to be advised, because the lordosis assists the indi- vidual in preserving his equilibrium, and if the spine is held erect in its natural posture the child falls forward and is unable to walk. In- dividuals suffering from muscular dystrophies are usually such chronic invalids as not to consider the question of marriage, but should this question arise it should be strongly advised against, inasmuch as this disease is so peculiarly hereditary. MUSCULAR ATROPHY OF THE PERONEAL TYPE. Char cot-Marie -Tooth Form of Progressive Muscular Atrophy. Progressive Neural Muscular Atrophy of Hoffman. In 1886 Charcot and Marie ^ in Paris, and Tooth ^ in Cambridge, England, described independently a form of progressive muscular atrophy beginning in the muscles supplied by the peroneal nerves and advancing upward as high as the knees in the legs, and subsequently attacking the muscles of the hands and forearms. This particular type of muscular atrophy has been recognized by many authors, espe- cially by J. Hoffman ^ and B. Sachs.* Although it is an extremely rare form of disease, a sufficient number of cases can now be collected to establish the affection as independent both of the muscular dystrophies on the one hand and of anterior poliomyelitis on the other. Etiology. — In some cases there appears to be an hereditary dispo- sition to the disease. Several members of a family may be affected, and in a few families it has been traceable through two or three genera- » Rev. deMdd., 1886. .^ * Brain, vol. x., p. 243. »Arch. f. Psych., Bd. xx., S. 560, and Deut. Zeitschr. f. Nerven., Bd. i., S. 95, * New York Medical Journal, December, 1888. Brain, 1890. MUSCULAR ATROPHY OF TEE PERONEAL TYPE. 317 tions. In many patients, however, no history of inheritance can be obtained. ^ No etiological factors have been established, and syphilis does not appear to be a cause. In a few cases it has been preceded by some form of infectious disease. The disease uniformly appears in young persons before the age of twenty years. Pathology. — The cases first recorded were supposed to be of the nature of muscular dystrophy, a progressive atrophy of the muscles of the legs and forearms and of the feet and hands being the only Fig. 136. Charcot-Marie-Tooth disease. Atrophy of the legs below the knees and of the arms below the elbows. lesion noticed. But in 1889 Hoffman discovered an atrophic neuritis in the peroneal nerves in addition to the muscular atrophy, and from that date the disease has been ascribed to a degenerative neuritis. Bernhardt/ in 1893, recorded a case in which in addition to the atrophy of the peroneal nerves there were found some changes of a sclerotic character in the posterior columns of the spinal cord ; and 'Virchow's Archiv, Bd, cxxxiii,, S. 259. 818 MUSCULAR DYSTROPHIES. Fjg. 137. Siemerling/ in 1899, after a study of all the cases upon record, reached the conclusion that the lesion of the disease consists in a degeneration both of the muscles, nerves, and posterior columns of the spinal cord, a lesion which involves the entire sensory element both in its periph- eral and central prolongations as well as the motor fibres of the nerves. He has found the posterior spinal ganglion aifected, and he has also found a sclerosis in a portion of the lateral columns of the cord not involving the pyramidal tracts. Siemerling is inclined to abandon the view that the disease begins in the peripheral nerves, and is inclined to ascribe it to a central lesion in the posterior horns of the spinal cord, but the exact pathology is still a matter of uncertainty. Symptoms. — The disease begins with a gradually increasing atrophy and weakness of the intrinsic mus- cles of the feet and of the long peroneal muscles on the outer side of the legs. The weakness and atrophy then appear in the anterior tibial muscles and extensor com- munis digitorum and in the poste- rior tibial muscles, so that after a slow increase during two or three years the patient is almost inca- pacitated from walking. The foot falls in walking, and hence the gait resembles the stepping gait of multiple neuritis. Occasionally the vastus iuternus in the thigh is at- rophied and adduction of the knee becomes somewhat difficult. The affection of this muscle somewhat impairs the gait, allowing the knee to fall outward ; hence the patients place the feet somewhat too far from one another in walking. (See Fig. 136.) The muscles which are Charcot-Marie-Tooth disease. Atrophy of the affccted show fibrillary Contractions, legs and drop-feet, and atrophy of the hands. thcV lose their reflcX activity and mechanical excitability, and there is a progressive diminution of the electrical excitability for both currents. In many cases a complete reaction of degeneration appears in an early stage. As a rule the weakness and atrophy do not extend above the knees, and hence the contrast in the appearance of the thigh and leg lArch. I Psjch., Bd. xxxi., S. 105, MUSCULAR ATROPHY OF THE PERONEAL TYPE. 319 is very marked in these patients. In all cases club-feet finally de- velop, and^bilateral club-feet should always suggest this disease. The symptoms appear in the arms after the legs have been affected for some months or years. Occasionally, however, the hands and arms are affected from the beginning. There is a gradual atrophy and weakness of the intrinsic muscles of the hands, of the thenar and hypothenar groups, and also of the forearms, and as these become weaker ab- normal positions of the fingers are assumed, giving rise to deformities of the nature of claw-hand. The muscles both on the front and back of the forearm are affected, and fibrillary contractures and diminution of electrical contractility with reaction of degeneration are found. The disease may come to a standstill at this point, as in the patient whose photograph is shown in Fig. 136, and there may appear to be no progressive increase in the symptoms for a long time. In other cases, however, the disease makes more rapid progress, the atrophy and paralysis involving the thighs and the arms, the muscles of the body and neck, and even the face, and the patient is gradually reduced to a skeleton, and dies from exhaustion. There are a few sensory disturbances. In some cases a slight numb- ness with diminution in the pain sense along the outer side of the legs is noticed, and in other cases complete anaesthesia in the distribution of the peroneal nerves has been found. The paralyzed limbs are usually cold and have a tendency to cyanosis especially in cold weather. The duration of the disease is uncertain, for many cases appear to be arrested, and the patients live their lives with a condition of atrophy in arms and legs and do not die of the affection. In other cases where the progress of the atrophy is more rapid they die of some intercurrent disease. Prognosis for recovery is always unfavorable. Treatment, — A general course of tonic treatment with baths, mas- sage, and electricity to the atrophied muscles appears to have some effect in lessening the rapidity of the atrophy, but does not appear to arrest the progress of the affection. CHAPTER XVII. SYRINGOMYELIA OR GLIOSIS SPINALIS. History. — Syringomyelia {aupcyq, tube, hollow ; //'J£/oc, marrow) or gliosis spinalis is a disease of the spinal cord characterized by the production of a cavity within the cord of varying length. The name was given by Ollivier in 1824, but the condition was first described by Etienne in 1546 and is mentioned by numerous writers on anatomy from that time onward. Portal (1804) was probably the first to ascribe a form of spinal paralysis to this lesion on the basis of four cases which he observed. From his time, however, until 1860 the condition, though occasionally noted by pathologists, excited no interest. Then with the beginning of pathological study of the nervous system various hypo- theses were proposed to explain the existence of cavities within the cord, and Lockhart Clarke, Vulpian, Hallopeau, Charcot and JoflFroy, Leyden, Schultze,^ and Kahler^ made important contributions to the subject. In the monographs of Roth, Wichmann, and Anna Baumler (1889) over 100 cases with autopsies were collected and analyzed. The study of these cases from a pathological standpoint was soon fol- lowed by their analysis from the clinical side, and in 1887 Schultze^ and Kahler* established the possibility of diagnosticating this condition during life. Their statements have been confirmed by clinical observers all over the world. In a monograph by Schlesinger^ 526 references to published cases or discussions of the subject are given. Dimitrofi"® has recently added to this literature. Pathology. — The post-mortem appearances in a case of syringo- myelia are very characteristic. The spinal meninges are normal. The contour of the cord is sometimes irregular, owing to a bulging at some places or a retraction at other places, or it appears at places flattened ; sometimes it is not altered. Fluctuation may be detected by palpation. Usually a rupture occurs in the process of removal of the cord, and the fluid, a clear serum, runs out, leaving the cord partly collapsed. It is then evident that there is a long cavity within the cord, usually near the central canal, but sometimes so extensive as in a cross-section to leave merely a thin ring or wall of cord tissue. Sections of the cord at various levels will demonstrate that this cavity extends for some dis- tance through the cord, and that it varies in size and shape at different * Schultze, Virchow's Archiv, Bd. Ixxxvii. and civ. ^Kahler, Vierteljahi-schrift fur Pract. Heilk., 1879. Arch. f. Psych., Bd. x, 3 Schultze, Zeitschr. fiir klin. Med., Bd. xiii., 1887. *Kahler, Prager med. Woch., 1888, Nos. 6 and 7. ^ Schlesinger, Die Syringomyelic, 1895, Leipzig. «Arcb, f. Psych., Bd. xxxv., S. 42. 320 PATHOLOGY. 321 levels. The usual situation of the cavity is in the lower cervical and dorsal regies. In some cases it is short, not involving more than five or six segments ; in others it is long, extending through the entire length of the cord and upward into the medulla and pons. Sometimes two or three separate cavities have been found at different levels. All possible variations have been observed in different cases. In some cases a tumor has been found on one side or within the wall of the cavity. When the cord is hardened, cut, stained, and examined microscopi- cally it presents certain characteristic appearances. The cavity may Fig. 138. Syringomyelia. Cavity in the central gray matter of the left half, with numerous diyerticula Walls of cavity .formed hy gliomatous tissue infiltrating the spinal cord. Weigert stain. (W. A. Turner.) be of auy size or of any shape, but lies chiefly near the central canal, behind the anterior commissure, or in the posterior central gray matter, or in a posterior horn, or in both horns of the cord. In some cases it invades the central gray matter and the anterior horn or horns, but it is rarely symmetrical in its invasion of the cord tissue on the two sides. In some cases the gray matter is entirely replaced by the cavity. lu other cases the cavity has invaded the white columns of one or both sides. The posterior columns are more frequently invaded than the lateral or anterior. In the most extreme cases it appears as if all the cord tissue had been destroyed. The cavity is then surrounded by a thin wall forming its sac, and no trace of gray or white substance remains. The wall of the cavity is smooth, but here and ihere papil- lary projections occur upon it. 21 322 SYRINGOMYELIA OR GLIO^If? SPINALIS. The cavity is usually surrounded by a zone of thick neuroglia tissue which stains deeply with carmine and hematoxylin, is unstained in the Weigert hematoxylin stain, is deeply stained by the Weigert neuroglia stain, and by the Golgi stains. The thickness of this neuroglia wall varies in different cases. Its structure is most dense near the cavity ; but it is thinner in the adjacent parts and fades away into tlie normal cord, not having as a rule a sharp boundary. (See Fig. 138.) Under a high power of the microscope it is seen to be made up of fine fibres, of nuclei, and of small and large neuroglia cells, a few of which are seen in some cases to be in a state of vacuolization and progressive Fig. 139. Syringomyelia. The cavity has taken the place of the central gray matter, and has invaded both posterior columns. liquefaction and to be breaking down. A fine filamentous network, containing spider cells and spindle-shaped cells with long processes and many nuclei, extends outward into the nerve tissue — the appear- ance being that of a partial infiltration of the normal cord by glioma- tous elements, the degree of which is greatest near to the wall of the cavity. Such an infiltration of the cord with cells is also found in the segments above and below the limits of the cavity, especially about the central canal. The cavity frequently occupies the place usually taken by the central canal. Sometimes it is seen to communicate with the remains of the central canal, and then some epithelial elements may be found in its wall. In other cases the canal is pushed to one side and lies in the wall of the cavity. In a few sections there may appear to be two cavities side by side ; but a careful examination of sections above or below will show that one of these is really a diverticulum from PATHOLOGY. 323 the main cavity. The cavity itself is never entirely lined by cylin- drical epiUielium ; but in a few cases one side of it may be so lined, and in these there is a manifest absorption of the original central canal into the new cavity, with more or less proliferation of the lining epithe- lium. A few cases have been described in which a true glioma or sarcoma filled the cavity, being an evident outgrowth from its wall.^ Changes in the bloodvessels of the cord are sometimes observed. There are very few capillaries to be seen in the wall of the cavity ; but out- side of it, in the adjacent parts of the cord where the infiltration of small cells and nuclei is seen vessels are more numerous than normal, their calibre is larger, and they are more tortuous than usual. In some cases distinct thickening of their walls has been noticed. Capillary hemorrhages are often found within the gliomatous structure. Various hypotheses have been proposed to explain the conditions described. They may be briefly summarized as follows : 1. It has been supposed that syringomyelia always originates in a congenital defect in the development of the spinal cord. It has been thought that the central canal of the cord may be unduly distended during foetal life and early infancy by fluid, thus remaining as an un- usually large cavity within the cord, around which cavity there subse- quently develops a proliferation of the embryonal epiblastic elements or a thickening of the normal glia tissue, which is known to be more abundant in this situation than elsewhere in the cord. Such a cavity is lined by epithelium and is described by many pathologists under the name " hydromyelus." But some believe that hydromyelus may pass into syringomyelia by the proliferation of glia cells, the invasion of the normal tissue, and its subsequent breaking down. They thus ex- plain the admitted fact that the degree in which the cavity in syrin- gomyelia is lined by cylindrical epithelium varies. Others hold that in the closure of the central canal during embryonal development a portion of it is shut oif from the main canal so as to leave a subsidiary canal in the posterior septum of the cord, lined with epithelium and surrounded, as is the normal central canal, by epiblastic tissue which subsequently proliferates, constituting a neuroglia hyperplasia, and then breaking down. Those who hold this opinion, therefore, ascribe all cases of syringomyelia to a congenital malformation of the cord and believe that the cavity of syringomyelia has a necessary relation to the normal central canal and usually communicates with it. 2. Another view of the disease is that the normal glia structure of the spinal cord, which is thickest about the central canal, undergoes a proliferation from some unknown cause — possibly an irritant poison in the fluid of the central canal ;^ that this gliomatous new structure extends outward into the adjacent tissue, both into the gray and white matter, and subsequently breaks down in its center, the cells becoming liquefied and disintegrated ; thus a cavity originates within a gliomatous mass. This cavity at its origin has no necessary relation to the central ' Van Gieson, Journal of Nervous and Mental Disease, July, 1889. ^lijtbea and Manicatide, Archives des Sci. Med. de Bucharest, May, 1896, 824 SYRINGOMYELIA OR GLIOSIS SPINALIS. Fig. 140. A/lhTlor llullL- A. Ceniru'l Canal Cenlral cavilies CQiised dy breaking (Itm''n ofUssice. Softened area- Sufi en ed cord Stem of Cliomatosis Glioma of the spiual cord, with formation of cavities within it. AR, anterior nerve roots- T, tumor; PR, posterior nerve roots; EC, epithelial lined cavities within the tumor. (Hudson, American Journal of the Medical Sciences, cxvii., 648.) PATHOLOGY. 825 canal ; but inasmuch as the gliomatous proliferation begins, as a rule, near to th«r canal, the cavity usually breaks into the central canal and thus makes a communication with it. The wall of the cavity is de- scribed as consisting of hyperplastic neuroglia with larger and smaller branching neuroglia cells, and small spheroidal cells, and oval cells lying in a network of fibres, at places closely packed together, at places loosely arranged, so that it has a porous meshwork structure. This forms a sort of limiting membrane for the cavity, but beyond it within the nervous tissue there is an infiltration of glia cells. These cells are seen to be in a state of liquefactive degeneration both in the wall of the cavity and elsewhere. In some cells the nucleus is attached to or surrounded by the homogeneous remains of the cell body. Some cells are converted into sacs of fluid. Thus there is a manifest tendency in the glia cells to break down, and the cavity is the result of such disintegration. Those who hold this opinion have named the disease spinal gliosis, believing the glia proliferation to be the essential factor in the pathology. Some consider this an inflammatory process,^ others^ deny anything more than a simple hyperplasia. Investigations of Weigert^ upon the structure of neuroglia seem to establish that new formations of glia may be either cellular in structure or fibrous in structure. If cellular, the new formation is a true glioma, such as is found in tumors of the brain or spinal cord, and in such a glioma fibres are few. Weigert calls attention to the fact that this is not the structure of the glia tissue about the cavity of syrin- gomyelia, but that the neuroglia found around this cavity consists almost exclusively of glia fibres with few cells ; and that these fibres, though extending in all directions, are chiefly vertical in their course. Miura also has shown the sharp contrast between ordinary glioma, even glioma containing a cavity, and the gliomatous condition of the cord in syringomyelia. Weigert holds that the neuroglia is merely a substance produced by nature to take the place of nerve tissue which has been destroyed, and that its proliferation is always a sign that the nerve tissue has primarily disintegrated. Such destruction of nerve tissue would, therefore, according to his view, precede the formation of gliomatous tissue ; hence he wholly discards the hypothesis of syrin- gomyelia to which the name spinal gliosis has been applied. Weigert says: "Many authors believe that the essential lesion in syringomyelia is the formation of a tumor followed by softening and the formation of a cavity. It is admitted that there is a growth of neuroglia of the typical fibre type about the cavity. But this fibre mass, devoid of cells, does not resemble a glioma and there is no reason to believe from the mere presence of neuroglia that the cavity is not a congenital or acquired abnormality of the central canal. There is a thick cluster of neuroglia fibres normally about the canal. By the pressure in this enlarged canal the nervous tissue may be destroyed, and hence a 'Miura, Ziegler's Beitrage zur path. Anat., xi., 91. ^Hchnhze, Zeitschr. fiir klin. Med., xiii. _ ^Weigert, Beitrage zur Kenntniss der normalen menschlichen Neuroglia. Frank- fort, 181J5. 326 SYRINGOMYELIA OR GLIOSIS SPINALIS. growth of neuroglia fostered. If the pressure increases the neuroglia may also be destroyed, and in its place about the cavity a hyaline form- less mass may remain. The gliosis is not the essential feature, it is only a secondary result." Turner,' however, has described a case in which there is a gradual and direction transition between a true glioma and a gliomatous infiltration of the cord with the production of a cavity. 3. Many authors have observed, subsequently to disease of the spinal arteries, the formation of cavities in the cord independent in their situ- ation of the central canal. Thus Miiller and Medin have seen a cavity in the gray matter of the cord with walls of normal nerve tissue with no signs of inflammatory disease or of proliferation in the glia, the size of the cavity corresponding in situation to the degree of endarteritis in the spinal vessels and having no definite relation to the central canal. Wieting has described a cord containing numerous cavities due entirely to the low nutrition of the nerve tissues, and consequent necrosis from disease of the spinal arteries in connection with men- ingo-myelitis. It has been thought by Kronthal that lymph stasis within the cord, and consequent necrosis, produced by transverse com- pression of the cord, may lead to the formation of cavities which may or may not communicate with the central canal ; but this surmise is doubtful, since compression by tumors or after Pott's disease is not found to cause cavities. The supposition that a cavity in the cord may be due to a diseased condition of the bloodvessels seems proven in some cases ; but such cavities do not resemble those of syringomyelia, and the suggestion finds little support in Weigert's hypothesis regard- ing the function of neuroglia. For if this hypothesis be correct a neuroglia growth would occur to replace the disintegrated nerve tissue, and in the attempt of nature to fill up the empty space would be thick- est about the cavity. Necrotic cavities, however, rarely have a well- marked wall. 4. Van Gieson has recently described a condition which he calls hemato-myelo-porus, of perforating hemorrhage in the cord, with the production of a long, narrow cavity. Such a cavity is occasionally surrounded by thickened glia tissue. Van Gieson shows that some cases which have been described as syringomyelia have really been old cases of hemorrhage. Turner and Mackintosh point out that the pres- ence of a fibrin-like material, which they describe in some of the cav- ities in several cases of gliomatosis of the cord, suggests that hemor- rhage assists in the formation of these spaces. P. Bailey^ has shown in his studies of spinal injuries that a symptom complex exactly like that of syringomyelia may develop rather rapidly after internal spinal hemorrhage. The hemorrhage may be single and destroy the central gray matter of the cord for some length, or there may be multiple small hemorrhages at various levels. Begg^ has recently reported such a case. ^ Turner and Mackintosh, Brain, 1896, Pt. Ixxv. ^Accident and Injury. D. Appleton & Co., 1906. 3 Lancet, July 16, 1904, p. 145. ^tMPfOM^. 327 5. Lastly, there is not wanting an hypothesis which would trace it to bacteriajl infection. Prus/ from a careful study of Morvan's disease, reached the conclusion, which several authors had already announced, that Morvan's disease and syringomyelia are identical. He calls atten- tion, however, to the fact that Zambaco maintains that it is identical with lepra ansesthetica, the bacillus of which can be recognized. And he appears to be willing to admit that the three diseases are, in fact, due to the same cause, namely, an infection of the nervous system by a germ which, in the lighter forms, attacks the peripheral nerves only and in the more severe forms attacks the spinal cord. In this view the neuroglia formation is set up by the irritation of the bacillus, and the cavity is due to the disintegration of the gliomatous substance. This hypothesis is strongly combated by Babes, who has observed six cases of lepra in which the bacilli were found in the cells of the cord, but in which there was no lesion resembling that of syringomyelia. It seems evident, therefore, from a review of these various hypoth- eses, that cavities may be formed within the spinal cord under vary- ing circumstances and by various pathological processes. First, from congenital defects of development; secondly, by a disintegration sub- sequent to a neuroglia proliferation either of inflammatory origin or of spontaneous occurrence ; thirdly, as the result of retrograde meta- morphosis of tissue, the nutrition of which is impaired by obstruc- tion to the circulation ; fourthly, by actual destruction of the cord by hemorrhage. The time has not yet come to establish conclusively any one of these views of the origin of syringomyelia. In fact, until cases are observed at the outset of the disease, as well as after a long duration, no basis for a conclusion can be established. When the cavity has existed for some time it is not uncommon to find evidences of ascending and descending degeneration in the columns of the cord, which are secondary to pressure or to the destruction of tissue at its point of maximum extent. Degeneration in the motor nerves and atrophy of the muscle fibres are also parts of the lesion in this disease. The various trophic disturbances in the bones and skin also require mention. Symptoms. — The diagnosis of syringomyelia rests upon the pres- ence of three characteristic symptoms which, in the majority of cases, are present together. The existence of one of these symptoms alone should excite suspicion of the possibility of the disease being present. The presence of any two of them make the diagnosis very probable. These symptoms are, first, a loss of the sensations of pain and of tem- perature in any part of the body, tactile sense being preserved in the analgesic area; secondly, trophic disturbances in the skin, muscles, bones, or joints; thirdly, progressive muscular atrophy attended by paralysis. In addition to these symptoms there may be (a) a spastic paraplegia, or (6) disturbance of tactile sense with pain, or (c) the general symp- »Arch. f. Psych., Bd. xxvii., S. 771. 328 SYRINGOMYELIA OR GLIOSIS SPINALIS. toms of transverse myelitis in case the disease invade respectively the («) lateral, or (/9) posterior columns of the cord, or (j) its entire area. Such an extension is not uncommon, and hence these symptoms must be considered as a frequent complication. The distribution of the characteristic symptoms of the disease will depend entirely upon the extent of the lesion in the cord. As this lesion usually begins in the cervical segments the symptoms almost always appear in the hands. If the lesion be limited to one or two segments of the cord the symptoms will be very limited ; but if it extend throughout the entire length of the cord and upward through the medulla and pons to the crus, the symptoms will be widespread and will involve the cranial nerves. The course of the disease is a very chronic one, the symptoms coming on slowly at any age, and often reaching a certain point and remaining stationary for years, the life of the patient being ended, as a rule, by some intercurrent disease, though occasionally sudden death is caused by the rupture of the cavity. The disturbance of sensation, called by Charcot dissociated anaes- thesia, is the chief characteristic of the disease. It is a symptom which is frequently unknown to the patient until it is demonstrated by the physician, although occasionally among the working classes, who are much exposed to injuries, the patient may have noticed that such injuries, especially burns, were not attended by pain. It is found upon examination of these persons that pricking, or cutting, or burning, or freezing of the aifected area is not attended by sensations of pain, or of heat or cold, though the sense of touch is preserved. The sense of heat may be impaired when that of cold remains, or conversely. The sense of pain is a great protection to the body, giving warning of injury and assuring care and rest of the part ; hence its absence exposes these patients to the risks of serious affections of the skin and joints, the consequence of neglect of small pathological processes at their start. The sense of touch is not often affected at all, although in cases where the cavity progresses to a considerable size and invades the posterior columns of the cord it may become somewhat blunted. The muscular sense appears to be preserved, excepting in this last class of cases. It is from these phenomena of dissociated anaesthesia that the conclusion has been reached that the paths of sensations of pain and temperature differ in their location from those of tactile sense, and that they pass into the central portion of the gray matter of the spinal cord soon after their entrance. It is certain that their entire course from below upward is not in the gray matter, otherwise a limited lesion of this portion in the cervical segments would produce a disturbance of these senses in the entire body below the lesion ; but it appears that these sensations on their way from the surface of the body to the centripetal white columns of the cord (the antero-lateral tracts — see page 74) traverse the gray matter at the level at which they enter ; hence the distribution of this disturbance of sensibility corresponds exactly to the position of the lesion in the spinal cord. As SYMPTOMS. 329 the exact area of the skin related to the individual segments of the cord has been ^determined, it is possible to reach a diagnosis of the exact extent of the spinal lesion by determining the exact extent of the an- algesia.^ The diagram, Plate VII. (page 46) shows this relation so far as it is at present determined. It demonstrates that the various districts of the skin can be assigned to the various segments of the cord. The distribution of the analgesia in syringomyelia is usually irregular, rarely symmetrical on the two sides. Inasmuch as the affection is more com- mon in the cervical region the condition of analgesia is more frequently found in the hands and arms. It is found that small injuries to the fingers are not attended by pain, an abnormality which first directs the patient's attention to the existence of his disease. In the early stages of the disease there is merely a decided blunting of the sensations of pain and an inability to distinguish between slight variations of tem- perature, or certain sensations only are not perceived. Thus Dejerine records a case in which the thermal sense was lost for all temperatures above 68° F. The area of analgesia may not coincide exactly with that of loss of temperature sense. Sensations of cold or of burning, or sharp pains, sometimes precede the loss of sensation. When the dis- ease is fully established the patient cannot distinguish any difference between iced water and boiling water, and a deep incision may be made without the slightest pain. Trophic disturbances are a very frequent symptom in syringomyelia. In the majority of patients it is evident that the origin of these dis- turbances is some injury, wound, or burn that had not been observed on account of the loss of pain sense, and that had therefore been ne- glected, had become infected, and had gone on to ulceration or suppura- tion. In some cases, however, it is impossible to ascribe trophic dis- turbances to this cause, and the hypothesis of the existence of trophic centres in the spinal cord presiding over the general nutrition and the repair of the body receives its chief support from the facts observed in this disease. The skin is the seat of the chief trophic disturbances. These may be of various kinds. There may be localized hypersemia or anaemia of the skin. There may be changes in the perspiration, the part being abnormally covered with sweat or abnormally dry ; and in addition to the acute inflammations of the skin already mentioned as produced by injuries, cases have been observed of serous exudation with desquama- tion, gangrene of the skin and subcutaneous tissue, bullae and peculiar hypertrophies and atrophies of the skin. Another trophic disturbance which has excited much interest is the appearance of painless whitlows and small abscesses upon the fingers. Morvan ^ described a disease occurring in a seaport of France among fishermen, in which felons appeared upon the fingers, producing deep ulcerations and even necrosis of the terminal phalanges. These were associated with other trophic disturbances of the skin and nails, and 'Max Laolir, Arch. f. PHychiatfie, 1896, xxviii., 773. Hiazetto hob. de Med. et de Chir., 1883. 330 SYRINGOMYELIA OR GLlOHlH HPlNAUS. with analgesia. This disease, which was named after Morvan, is now thought to be a variety of syringomyelia, for in all cases examined after Fig. 141. Trophic changes In the hands in syringomyelia in the form known as Morvan's disease. The muscles are atrophied. The hands are much deformed. The skin is atrophied. The nails have fallen. The bones are hypertrophied at some parts, atrophied at others. The tip of one finger is eroded. (Curschmann, Klin. Abbildungen.) death a cavity has been found in the spinal cord, appearance of the hands in Morvan's disease. Fig. 141 shows the SYMPTOMS. 331 The growth of the nails is commonly affected in the disease. They are hypertt-ophied, ridged, and occasionally stained. They become particularly brittle and are irregular in their form. Affections of the joints and bones are very frequently observed in syringomyelia. In fact, there is no nervous disease in which joint affections occur so commonly as a complication. The shoulder, elbow, and wrist are the joints most commonly affected. In this respect the disease offers a contrast to tabes, in which the joint affections most fre- quently occur in the lower extremities. The character of the joint affection, is, as a rule, quite similar to that described by Charcot as occurring in locomotor ataxia — a large effusion within the joint, with great thickening of all the tissues, and later an absorption of the bones with an atrophy of the joint surfaces. Fig. 142 shows the appearance Fig. 142. Arthropathy of the right shoulder in syringomyelia. (Dercuiu.) of a patient suffering from an arthropathy of the right shoulder, and Fig. 143 shows the condition found in the bone after death. Schles- inger has collected sixty-three cases of joint affection occurring in the course of the disease, and he estimates that this complication occurs in more than 10 per cent, of the cases. Alterations in the con- dition of the long bones are observed in syringomyelia, and spontane- ous fractures, due to a spongy and brittle condition of the bones, have been recorded by a number of observers. Both the joint affections and these fractures proceed without pain to the patient, and hence are often neglected for some time after they begin. In a considerable number of cases a marked curvature of the spine, either lateral or forward, and occasionally backward, has been observed. This has been ascribed by some authors to atrophy and weakness of the spinal muscles and by others to actual changes in the bones. Both conditions may occur. The spine is, as a rule, sensitive to pressure. Deformity is more likely to occur in the upper portion of the dorsal region than elsewhere. It is never very extensive. As the records of the disease have increased its incidental association with various diseases — acromegalic, hysteria, paralysis agitans — has been recorded. Such associations have no particular significance. Muscular atrophy attended by paralysis is present in more than one- 332 SYRINGOMYELIA OR GLIOSIS SPINALIS. half of the cases of syringomyelia. It usually begins as a progressive muscular atrophy invading the hands, especially the first lumbricalis muscle, then the thenar and hypothenar eminences, finally producing claw-hands [main en griffe of Duchenne), and then advancing up the limb to the forearm, arm, and shoulder. Occasionally the shoulder muscles are the first to be affected, and then the atrophy appears in the deltoid and scapular muscles, and later invades the biceps and Fig. 143. Humerus and scapula of the right side. The head of the humerus has been destroyed. The glenoid surface of the scapula Is enlarged, and its edges are thickened by bony deposits. The coracoid process presents peripheral accretions. (Dereum.) supinator longus. The muscles of the spine are particularly liable to be invaded by the atrophy and paralysis, and as a consequence curva- ture of the spine is a very frequent symptom in this disease. The legs are less frequently affected (12 per cent, of the cases), but atrophic paralysis of the thighs and of the legs below the knee with consequent contractures has been seen. The exact distribution of the atrophy and paralysis depends upon the extent of the lesion in the various segments of the cord. In the table already given on page 54 the relation between the various muscles of the body and the various segments of the cord is shown. It is therefore evident that from a knowledge of the muscles invaded a conclusion can be reached as to the extent of the lesion. The atrophic paralysis of the muscles is attended by fibrillary contractions and SYMPTOMS. 333 tremors and by a gradual diminution in the mechanical and electrical contractility of the muscle. It is only in the last stage of the disease, when the muscle is extremely atrophied, that it presents the reaction of degeneration. The spinal reflexes may be disturbed in this disease. When the symptoms are located in the arms, elbow and wrist reflexes are lost, while the patella reflex is, as a rule, increased. If, however, the disease invades the lumbar region of the cord the patellar reflex may be lost on the side of the lesion. In a certain number of cases there is an extension of the disease into one or both lateral columns of the cord. When this occurs the lateral pyramidal tracts are afiected and the symptoms of spastic paraplegia (see Chapter XVIII.) are added to those of the disease. An increase of knee jerks, the appearance of ankle clonus and Babinski reflex and the slow development of a spastic gait with incontinence of urine will be evidence of such extension. They follow syringomyelia of the cervical and dorsal regions. In a few cases in which the sacral region of the cord has been diseased a loss of control of the bladder and rectum has occurred in addition to the other symptoms. The spinal centre of the cervical sympathetic nerve lies in the first dorsal segment of the cord, and as this segment is very frequently afiected, symptoms of paralysis of the sympathetic of one or both sides are commonly to be detected. They are a narrowing of the palpebral fissure, a retraction of the eyeball, sluggish pupillary action, with im- perfect dilatation, a flattening of the side of the face, and a defective secretion of sweat. The extension of the disease to the medulla may cause symptoms referable to the implication of the cranial nerves. Atrophy with fibril- lary tremor in the tongue and facial muscles, ocular palsies with nys- tagmus, and dissociated anaesthesia of the face and head have been observed. In a few cases paralysis of the vocal cords, disturbances in the act of swallowing, difficulty of respiration, and irregular heart action have indicated that the vagus centre has been affected. These symptoms are most serious, as sudden death commonly ensues. The term syringo-bulbia has been applied to this group of symptoms. The course of the disease is a chronic one. It advances slowly, and the symptoms are, as a rule, well established before the disease is recognized. The patients remain for months in a stationary condition or the paralysis slowly increases until they are disabled. The symp- toms may finally extend to the entire body, although this is rare. Death occurs either from heart failure, or from cystitis, or bed-sores, or from some intercurrent affection, or rarely very suddenly without apparent cause, really from a rupture of the cord allowing an escape of fluid from the cavity. The following history of a case of syringomyelia under my obser- vation at the Yanderbilt clinic illustrates the usual symptoms and course of the disease : P. S., of healthy parentage, noticed in March, 1896, when he was 334 SYRINGOMYELIA OR GLIOSIS SPINALIS. Fig. 144. sixteen years of age, that he was becoming weak and clumsy in his hands, that he was dropping things unintentionally, and was losing strength in "his arms. These symptoms were noticed in the left arm before they appeared in the right. They were not attended by pain or any noticeable sensory disturbance. It was noticed that his hands and arms became gradually thinner as they became weaker, and the emaci- ation soon extended to his body, especially about the muscles -of the chest, and back, and scapulae. He was not aware of any sensory dis- turbance until the time of his first examination at the clinic in Janu- ary, 1897. He had had no trouble with his bladder or rectum. He had had no symptoms in his legs, excepting a slight weariness on any exertion, but he had noticed that his back had gradually become crooked, the right side of his body appearing to bulge. It was evident from the history that all his symp- toms had made such gradual progress during the year that they had not attracted much attention until his inability compelled him to quit work. Examination in January, 1897, showed a very marked condition of atrophy with corresponding paral- ysis in the muscles of both upper ex- tremities, chest, scapulae, and back, as shown in the picture. (Fig. 144.) No muscle was entirely para- lyzed, but all the muscles were ex- tremely weak, presented fibrillary contractions on exposure to cold or on percussion, but did not show any reaction of degeneration. The atrophy was most extreme about the muscles of the scapulae and in the deltoids and upper part of the arms. The muscles of the thorax and back were markedly atrophied, so that a lordosis was very evident, causing peculiar motions of balancing in the act of walking. The atrophy was about equal on both sides. The biceps was less atrophic than the other muscles of the upper arm. The flexors and extensors of wrist and fingers, the thenar and hypothenar muscles, and the interossei of the hand, were very much atrophied. The lower portion of the pectoralis major on both sides was preservedj but the upper Case of syringomyelia. Atrophy of the muscles of the shoulders and right arm. Cur- vature of the spine forward from atrophy of muscles of the bacli. DIAGNOSIS. 835 part was atrophic. The muscles of the abdomen and legs were not in any way a^ected, but the knee-jerks were very much exaggerated, and there was ankle clonus on both sides. The elbow and wrist reflexes were lost. Face was normal. Sensation to touch was preserved in all parts of the extremities, body, and thorax, but sensations of heat and cold and of pain could not be perceived over the upper part of the thorax or in both upper extremities. The loss of pain sense was somewhat less extensive than the loss of sensation to heat and cold on the back. This patient was observed very carefully in St. Luke's Hospital for six months, but there was little or no change in his condition.^ Diagnosis. — When the three characteristic symptoms already men- tioned are present in any case there is no question regarding the diagnosis of syringomyelia. In the early stages of the disease, how- ever, before all three symptoms appear, the disease may be mistaken for other spinal affections. Thus many cases are regarded for a con- siderable time as cases of progressive muscular atrophy or of chronic anterior poliomyelitis, and it is only on the appearance of the peculiar sensory disorder or of the trophic symptoms in the skin or bones that the first diagnosis becomes questionable. In other cases the early sus- picion may be of tabes, especially if the disease be located in the lower part of the cord, for then the pains, especially the burning sensations, the disturbances in temperature and pain sense, and the paraesthesia, with trophic disturbances in the joints and loss of tendon reflex at the knee may suggest locomotor ataxia, even though the ataxia be not manifest. Then it is only when atrophies of the muscles and paralysis occur that syringomyelia is suspected. The diagnosis from amyotrophic lateral sclerosis may be made from the fact that in that disease there is an increase of mechanical excitability in the paralyzed muscles, an increase of reflex action, the early appearance of a spastic gait, an absence of sensory symptoms, and little tendency to trophic disorders. A general myelitis or a disseminated myelitis may be diagnosticated in cases of syringomyelia when both motor and sensory and trophic disturbances are present ; but the lack of symmetry of the sensory dis- orders and the peculiar preservation of the tactile sense in syringo- myelia should enable the observer to avoid this mistake. It is, how- ever, to be remembered that in some cases the two diseases occur together.^ While it is true that syringomyelia is often due to a tumor of the spinal cord, especially glioma, it is to be remembered that tumors of the cord are usually limited in extent to two or three segments, that they produce more widespread symptoms than syringomyelia, especially in the body below the level of the lesion ; that the symptoms resemble those of a transverse myelitis of rapid onset, and that pain of a severe character is a constant symptom in spinal tumors. The course of the 'For other histories the reader is referred to the American Journal of the Medical Sciences, May, 1888,* and December, 1896. 'Gowers, Diseases of the Nervous System, vol. i., p. 339, 336 SYRINGOMYELIA OB GLIOSIS SPINALIS. disease, steadily progressive in tumor, may aid the diagnosis when symptoms are ambiguous. Pachymeningitis cervicalis may give rise to somewhat similar symptoms in the arms; but the severe pain in the neck, the rigidity and the fixed posture, the absence of dissociated anaesthesia, and the lack of trophic disturbances will prevent any mis- take in diagnosis. Syringomyelia presents some of the features of bulbar palsy when the cavity invades the medulla and pons ; but the cavity is rarely con- fined to the medulla and pons, and hence in syringomyelia the symp- toms are not exclusively bulbar ; thus a point of distinction between the two diseases is afforded. Prognosis. — The prognosis as to recovery is unfavorable ; but inas- much as the disease rarely progresses beyond a certain point it cannot be considered dangerous to life. Treatment. — There is no known remedy that will arrest the patho- logical process. The exposure of the back to the a;-rays or to radium has been tried and deserves further trial, the results being as yet uncer- tain. The symptoms are to be treated as they arise — the paralysis, for instance, as in anterior poliomyelitis. The trophic disturbances may often be prevented by care, and if they occur are to be treated by rest, by mechanical appliances, or by surgical measures. The sensory loss cannot be remedied by faradic applications. It is to be remem- bered that the disease often comes to a spontaneous standstill ; hence remedies of a constitutional kind are not to be implicitly trusted, even though they appear to arrest it. CHAPTER XVIII. LATERAL SCLEROSIS. SPASTIC PARAPLEGIA. History. — A condition of stiffness in the legs that slowly advances to a state of paralysis, with increased reflexes and rigidity, but is not attended by sensory symptoms, though recognized by Turck in 1856, was first described by Charcot in 1865. It was carefully studied in 1873 by Seguin,^ who named it tetanoid paraplegia. In 1875 a com- plete analysis of the clinical symptoms was made by Erb,^ who ascribed them to an ascending sclerosis of the lateral columns of the spinal cord and named the affection spasmodic spinal paralysis. In the following year Charcot differentiated it more clearly from other spinal affections, naming it tabes dorsale spasmodique. Careful observation of cases soon developed the fact that as a symptomatic condition it appeared much more commonly as a secondary affection than as a primary disease. And, as the lesion of lateral sclerosis was ascertained to be in many cases the result of transverse lesions of the cord at a high level, and its identity with secondary descending degeneration in the lateral columns was established, many observers denied the existence of a primary lateral sclerosis and affirmed that every case, if of sufficient duration, would prove to be of a secondary nature. It was shown that the lateral tracts in the spinal cord, the sclerosis in which caused the symptoms, are made up of axones whose neurone bodies lie in the motor area of the cerebral cortex. And it was proven that any disease which affected these neurones, whether in the brain cortex or in the sub- cortical tracts, in the brain axis, or in the spinal cord, unilateral or bilateral, is capable of causing the symptom of spastic paralysis. It was evident, therefore, that as a symptom it might appear in many different diseases and be due to many various lesions. It has been shown that lateral sclerosis results from : 1. Any disease in the brain that affects the motor tracts, such as tumors, softening, hemorrhagic destruction, or inflammation, with its degenerative results. Thus it is the lesion present in infantile cerebral palsy (Little's disease), whether unilateral or bilateral, and also in hydrocephalus. It may also occur in multiple sclerosis. After brain diseases it is usually unilateral. It may be bilateral if the brain axis is destroyed and both motor tracts are affected. 2. Any disease in the spinal cord that involves the lateral columns or cuts them off from their nutrient cells in the cortex of the brain, such as transverse myelitis, hemorrhage in the cord, syringomyelia, combined or disseminated sclerosis, or tumors of the cord. ' New York Medical Journal, 1873. ^ Virchow's Archiv, vol. Ixx., and Berliner klin, Woch., No. 26, 1875, 22 337 338 LATERAL 8CLER08I8. SPASTIC PARAPLEGIA. 3. Any disease of the spiue which compresses the spinal cord, such as caries, tumors of the vertebra, pachymeningitis, or aneurism. There are, however, certain cases in which the symptoms of spastic paraplegia develop that cannot be assigned to any of these- causes, since no symptoms of these diseases appear. And in a few such cases death has occurred and the autopsy has proven that the lesion was a lateral sclerosis.^ Oppenheim, Dejerine and Soltas, Striimpell, and others have published cases. Therefore it is admitted that the disease may occur as a primary one, being a degeneration of the motor neurone, whose body lies in the brain cortex and whose axone lies in the lateral pyramidal tract. This is the cortico-spinal element of the motor tract. It has already been shown that the spinomuscular element of this tract is subject to degeneration in chronic anterior poliomyelitis (page 285). It has been shown that in amyotrophic lateral sclerosis both cortico-spinal and spinomuscular elements are degenerated together (page 295). Hence the argument, from analogy, points to the exist- ence of primary lateral sclerosis. Furthermore, Striimpell has shown that in certain families a tendency exists to an imperfect development of the lateral columns of the cord, and he has established the existence of a family type of lateral sclerosis. Neumark ^ has published the history of a family in which seven children out of nine developed the disease. Therefore the existence of the disease, both congenital and acquired, must be admitted, having been proven by pathological observation. In 1892 Erb differentiated from primary lateral sclerosis a second clinical form due to syphilis, and named it syphilitic spastic spinal paralysis. It usually develops within five years of the initial lesion. It differs from spastic paraplegia in the facts that disturbance in the control of the bladder and rectum occur, and that there may be slight subjective and objective disturbance of sensation. It has a slow onset and a chronic course. An analysis of the cases made by Koch ^ in 1893 and added to by Striimpell^ in 1904 has shown that the lesion is a transverse myelitis of the dorsal region of the cord, more or less incomplete, with secondary degeneration downward in the lateral columns and upward in the posterior columns. Erb is inclined to consider these degenerations as primary and due to the syphilo-toxins, but admits that they may be secondary to the transverse lesion. It will be considered under chronic myelitis, as I do not regard it as a true primary lateral sclerosis. Etiology. — The etiology of spastic paraplegia is obscure. In a few cases long marches and overexertion have been known to precede the development of the disease. Trauma has been thought to be a cause, for some cases have devel- oped after falls or blows on the back. It has been thought to be trace- able to infectious diseases in a few cases. ^ See ten cases cited by Erb, Lancet, October 11, 1902, 2 Amer. Jour. Med. Sci., 1893, p. 432 ; Deut. Zeitschr. Nervenk, 1911, p. 420, »Deut. Zeitschr. f. Nervenheilk., 1893, vol. iii, ^ *Ibid., vol. xxvii., p. 291. PATHOLOGY. 339 Syphilis causes a condition closely allied to lateral sclerosis, and is probably sf cause in some cases. But, as in tabes, the aifection is usu- ally a parasyphilitic disease and does not yield to mercurials and iodide of potassium. Persons in middle life are most liable, the majority of cases devel- oping between the ages of twenty and forty years. Even some of the family cases do not develop until after the age of twenty years. In Neumark's cases the disease developed gradually in some, in others it appeared rapidly after acute infectious diseases. Pathology. — The pathology of the affection consists of a degenera- tion in the cortico-spinal element of the motor system, beginning in its peripheral portion, which lies in the lower part of the lateral pyram- idal columns of the spinal cord. The degeneration appears to be a primary one, not of an inflammatory nature. Little by little the axones atrophy from below upward and disappear, the myelin that surrounds them is absorbed, and a secondary hyperplasia of neuroglia occurs, resulting in a sclerosis accurately limited to the distribution of the long tracts of motor function in the spinal cord. In the cervical region the anterior median columns of the cord have been found sclerosed. The association tracts of the cord appear to escape, and there is no affection of the anterior horns or of the spinomuscular element of the nervous system. The appearance of the spinal cord is not unlike that already shown in Figs. 17 and 18, page 51, which demonstrate secondary lateral sclerosis.;. In Neumark's cases a sclerosis of the columns of Goll was also present, not attended by any sensory symptoms. The lesion in his cases was almost identical with that found in Friedreich's ataxia (q. v.). Symptoms. — The symptoms of spastic paralysis are a very grad- ually increasing stiffness and rigidity of the muscles of the legs attended by an increase in the reflexes and a tendency to cramps and tremor. The disease may begin on one side, but soon becomes bi- lateral. The patient appreciates difficulty in all motions of the legs; he cannot step freely, he cannot go up stairs with comfort on account of great stiffness of the joints and muscles. It requires a great effort to produce slight movements, and passive motion is as difficult as active voluntary motion. The gait is characteristic of the affection. The feet are not lifted from the ground, the toes are dragged, the shoe wears out on its inner surface and toe, the legs cannot be abducted freely, and the knees have a tendency to overlap. The patient shuffles along the ground, his steps becoming short, there being trepidation due to the increase of reflex action, causing a clonus of the foot at every step. Much fatigue is felt on walking, and the muscles often ache. Little by little the stiff- ness increases until the entire lower extremity appears to be moved as a mass without any motion of the ankle or knee-joints, and all efforts, such as crossing the leg, kneeling down, or kicking, are very much hampered and Anally become impossible. The muscles appear to be made of hard^ tense co.rpinal arteries. The shaded areas are the regions which are sclerotic in combined sclerosis, and correspond to the distribution of the peripheral system of arteries. (Marie. ) Pathology. — Combined sclerosis consists of a diffuse sclerotic proc- ess in the spinal cord chiefly limited to the posterior and lateral columns. In cases of moderate duration the lesion affects the column of Goll in its entire length, the column of Burdach in part (the root zone, the peripheral portion and the part adjacent to the commissure often escap- ing), the direct cerebellar tract, and the lateral pyramidal tract. In cases of long duration the lateral limiting layer, the tract of Gowers, and a part of the antero-lateral tract adjacent to the pyramidal tract, as well as the cells of Clarke's column and of the central gray matter, are also degenerated and the sclerosis is more complete in the columns first affected. The extent of the lesion vertically also differs in differ- ent cases, according to their duration. In the majority of cases the dorsal region of the cord is the part first involved, and as the case goes on secondary degeneration as well as the extension of the original lesion increase the vertical extent of the sclerotic process. In some cases the lateral columns are more deeply involved than in others. In some PATHOLOGY. 385 cases the posterior columns are those first and most seriously affected. But if the pStient lives long enough the terminal condition is one of invasion of both columns in their entire length. The varying descrip- FiG. 165. Fig. 166. Fig. 167. Fig. 168. Fig. 169. The situation of the lesion in combined sclerosis of vascular origin. Fig. 165, cervical ; Fig. 166 middle dorsal ; Fig. 167, lower dorsal ; Fig. 168, lumbar ; Fig. 169, sacral regions. In the lumbar and sacral region the lesion is one of descending degeneration only. (Ballet et Minor.) tions presented by different authors of these lesions are largely due to the omission of any reference to the duration of the disease in the dif- ferent cases, Putnam being the only observer to distinguish between symptoms of long and of short standing in the same case. In addition to the sclerosis of the cord the majority of observers have noticed a thickening of the pia mater, especially upon the posterior and lateral surfaces, with adhesion to the cord. Some have seen a state of arterio-capillary fibrosis or a true endarteritis in the spinal vessels. The anterior nerve roots have been found atrophied. The combination of secondary degeneration of the motor and sensory tracts sometimes found in advanced cases of general paresis is not to be classed with this disease. 25 386 COMBINED SCLEROSIS. Etiology. — Little is known of the cause of ataxic paraplegia, but the widespread degeneration in many columns of the cord has given rise to the supposition that it is due to an inherent want of vitality in the nervous system with a tendency to degeneration from general mal- nutrition of the neurones. It is certainly more common in persons of Fig. 170. Section through the lower cervical cord showing combined sclerosis of the lateral and posterior columns. (Larkin.) neurotic tendency. It develops usually in males in early adult life, is not particularly related to syphilis, though it has been traced to it in some cases. In many cases it follows extreme muscular exertion, and hence is more common in workmen and those subject to long marches or heavy labor. It has been known to develop after severe exhaust- ing diseases, such as anaemia, leucocythsemia, and cancer, and especially as a complication of pernicious anaemia. Marie traces it to endarteritis in the spinal vessels. Symptoms. — Patients suffering from combined sclerosis complain first of considerable fatigue in the legs after short walks, a sense of numbness and heaviness in the legs, and of stiffness of the muscles and of an unsteadiness of gait. This unsteadiness of gait is usually worse in the dark, and gradually as months go by the patient becomes dis- tinctly ataxic. But this ataxia is combined with a rather marked stiff- ness in the action of the legs similar to that seen in lateral sclerosis ; the feet are not thrown high as in locomotor ataxia, but are dragged along the ground. There is a marked swaying when the eyes are closed. There is slight diminution of tactile sensibility in the feet, less SYMPTOMS. 387 in the thigh^, and there is no girdle sensation. There is an exaggera- tion of the knee-jerks, and ankle clonus and Babinski's reflex are pres- ent. These patients do not complain of lightning pains, as in locomotor ataxia, and they are not subject to crises. They often suffer from a dull aching sensation in the sacrum. As the case goes on, after three or four years the spastic symptoms appear to surpass the ataxic symptoms in intensity, and spastic para- plegia, together with its characteristic gait, obscures the ataxia. These patients sometimes suffer from disturbance of the action of the bladder and rectum as the disease goes on. When the ataxia reaches the upper extremities the reflexes are increased. The Argyll-Robertson pupil is absent, but nystagmus is not infrequently developed, and this may give rise to the supposition that the disease is really disseminated sclerosis. Optic atrophy has been observed. The course of the case is a very chronic one, but is progressive, in this respect differing from the spontaneous arrest frequently seen in locomotor ataxia. In the course of six or ten years these patients become fully paralyzed, are confined to the bed with limbs drawn up and rigid, and with all the distressing twitchings of the muscles, spasms of the legs and back, and tendency to bed-sores, 'which are present in cases of lateral sclerosis in the later stages. They usually die of some complication. Some cases are more rapid in their progress. This is especially true of those associated with and due to pernicious anaemia or some form of toxaemia. In these cases the final termination is reached within a year, emaciation, diarrhoea, and exhaustion preceding death. Dana and Putnam have described these cases most fully. As to the manner of onset and course of these cases there is a word to be said. The facts presented in the discussion of the pathology would indicate that the symptoms in different cases may be quite different. Oppenheim distinguishes two groups of cases : one in which the symp- toms of spastic paraplegia are more prominent ; the other in which the symptoms of locomotor ataxia are more marked. In the first the spastic paralysis is soon followed by the symptoms of ataxia. In the second the reverse order is seen. In my experience the latter class is more frequent than the former, and it is this class which corresponds to the ataxic paraplegia of Gowers. It is a very rare type of spinal affection. Diagnosis. — The diagnosis of the disease presents no difficulties, as is evident from the description of the symptoms. It cannot be mis- taken eventually for tabes or for lateral sclerosis because symptoms of each disease appear in addition to those of the other. Prognosis. — The disease is a slowly progressive one, and no arrest in its course can be expected. Yet occasionally periods of remission, like those occurring in locomotor ataxia, have been observed. Treatment. — Treatment is the same as that in locomotor ataxia. 388 FRIEDREICH'S ATAXIA. FRIEDREICH'S ATAXIA. A defective development of the spinal cord, with the production of neuroglia tissue taking the place of the defective or degenerated fibres in a number of various tracts of the spinal cord, was first described by Friedreich, of Heidelberg, in 1863 and 1876, and was named Fried- reich's ataxia. Friedreich supposed it to be a juvenile form of loco- motor ataxia, and called attention to its congenital origin and to the frequency with which it appeared in several members of a family. To Schultze must be given the credit of showing, in 1877, that the disease is not related in any way to locomotor ataxia, but is due to a defective development of the spinal cord. There is, apparently, a thickened and sclerotic tissue in the posterior and lateral columns of the spinal cord. Fig. 171. The lesion of Friedreich's hereditary ataxia. Maldevelopment and sclerosis of the lateral and posterior columns. (Schultze, Lehrbk. d. Nervenkr., Taf. iv.) Pathology. — Fig. 171, which is taken from one of Friedreich's original cases, demonstrates the peculiar appearance of the spinal cord in this condition. The spinal cord in all of these cases appears unusu- ally thin and small, and there is usually a thickening of the pia mater about it, especially upon the posterior surface. Microscopic examina- tion shows the presence of extensive degeneration and the remains of a few nerve fibres only in the direct cerebellar tracts, the column of Gow- ers, the lateral pyramidal tracts, and in the columns of Goll and Burdach. The exogenous and endogenous fibres are both involved in the lesion. The column of Goll is degenerated in its entire length and is more com- pletely affected than the column of Burdach. The root zone of the column of Burdach and the column of Lissauer often contain very many normal fibres. The direct cerebellar column is affected as well as the pyramidal tract. In the antero-lateral column, Blocq and Marinesco have noticed a diminution in the normal number of fibres. It is evi- dent, therefore, that a defective development in all the fibres of the SYMPTOMS. 389 spinal cord is present which is more marked in the posterior than in the anterioi?* half. The gray matter of the cord also shows changes. There is an atrophy of the cells in both anterior and posterior horns, fewer cells being present than in a normal cord. The cells of the column of Clarke are notably degenerated and are very few in num- ber. Occasionally around the central canal there is a thickening of the ependyma and a mass of small round cells. Both posterior and anterior nerve roots have been found somewhat atrophied. Wherever the nerve fibres are wanting, a thickening of the glia tissue is evident, but it is secondary and not primary. All authors agree that the dis- ease is due to an arrest of development of the various systems of fibres in the spinal cord. Further investigations are necessary to confirm the statements of Marchi that secondary changes in the cerebellum are always present, Nonne and Menzel have shown, however, that an atrophy due to a defective development of the cerebellum will give rise to symptoms almost identical with those of Friedreich's ataxia. These symptoms have been best described by Marie, and it is often difficult to differentiate Friedreich's ataxia from Marie's form of cerebellar atrophy. Etiology. — The disease appears in childhood, and is sometimes present in several members of the same family; hence it has been termed hereditary In the cases observed by me, however, but one child out of several was affected. It occasionally develops after some one of the infectious diseases of childhood, which infection is supposed to accelerate an inherent tendency to imperfect growth. In Friedreich's cases alcoholism in the parents was supposed to be the cause. Oppen- heim believes that it may be a manifestation of hereditary syphilis. It may be congenital, the children never developing the power to stand or walk. It is usual, however, for the symptoms to make their appearance from the sixth to the eighth year of life, though cases are on record of as late a development as the sixteenth year. Boys are more frequently affected than girls. Symptoms. — A slow onset during childhood of symptoms which are a combination of spastic paraplegia and ataxia is characteristic of this disease. As the child learns to walk it is found that he is unsteady on his feet and awkward in his gait, being liable to fall, and he stag- gers in a manner suggestive of cerebellar disease. He walks slowly with little steps, unsteady, with feet far apart, and some tendency to stamp the foot, but the legs are not thrown about to the degree that is present in tabes. When the child attempts to stand he sways from side to side, the muscles of the legs contract, and the head moves as well as the body in the attempts to preserve the balance. Closing the eyes increases this unsteadiness. As the ataxia increases a certain rigidity of the legs begins to appear, and this is commonly attended by a contracture of the posterior tibial group of muscles, causing a talipes equinus. This deformity of the foot makes walking still more difficult until, little by little, the child becomes incapable of standing or walking alone. Fig. 172 shows the ordinary appearance 390 FRIEDREICH'S ATAXIA. Fig. 172. of one of these children in standing, and Fig. 173 shows the char- acteristic deformity of the foot. When the child is quiet and seated constant oscillation of the body and of the head is observed, a symptom which prevented the photograph. Fig. 172, from being clear in out- line. This is not a chorea-like twitching, because it is slower than the movement of chorea, and as effort to keep still increases this oscillation it reminds one of the intention tremor of disseminated sclerosis. The arms are less completely affected than the legs, but they share in the ataxia and in the intentional tremor, so that the child is soon incapacitated from feeding himself or dressing himself. In the later stages of the affection a true paralysis may develop in the legs of the type of spastic paraplegia, the muscles being so contractured and rigid that they cannot be voluntarily moved. Early in the disease nystagmus develops. It is not present when the eyes are quiet, but becomes visible on any attempt at turn- ing the eyes from side to side. Paralysis of the ocular muscles, however, does not occur, and there is no tendency to optic atrophy. A disturbance of speech is commonly pres- ent in these children. The speech is slow and difficult and indistinct, not unlike the speech of disseminated sclerosis. The tongue can be protruded, but usually shows fibrillary tremors. There is rarely any disturbance of sensi- bility in the affected limbs, and shooting pains are not complained of. Sometimes the rigidity of the limbs gives rise to muscular pain. The patellar reflex is diminished or lost early in the disease, but the pupil reflex remains normal. There is no disturbance of the bladder or rectum. As a rule, the chil- dren remain rather infantile in their cerebral development. It is difficult to give them an education, and even when reaching adult life they appear to be weak-minded. But this is not always the case, though the difficulty of speech and the rather stupid appearance of the face may lead to the supposition that the child is less capable than he really is. The course of the case is a gradually progressive one. It usu- ally appears at the age of six or seven years, rendering the child incapa- ble and paralyzed by the age of fifteen years. It never begins after the Friedreich's liereditary ataxia. The awkward posture and forward bending of the body are noticeable; also the slight flexion of knees and elbows. MA&m&i^. 391 age of sixteen years. It is not iu itself a fatal affection, and therefore, if the children are well cared for they may grow up. They die of some intercurrent aifection after many years of a crippled existence. Diagnosis. — The diagnosis of the affection is not difficult on account of the characteristic appearance of the symptoms in childhood. As children are almost exempt from locomotor ataxia, this is the only affec- tion, exceptmg multiple neuritis, that will produce ataxia in children. Fig. 173. Contrast Ijetween the deformed foot in Friedreich's ataxia and a normal foot. There is no difficulty in differentiating it from multiple neuritis on account of the history of the case. Marie's cerebellar hereditary ataxia presents very similar symptoms, but this develops between the twen- tieth and thirtieth years ; it affects the arms ; the patellar tendon reflex Is preserved. The characteristic contracture and deformity of the foot are not present, while, on the other hand, loss of pupil reflex, paralysis of the ocular muscles, and optic-nerve atrophy are frequently observed. The prognosis is unfavorable, and there is no form of treatment which is known to arrest the progress of the disease. CHAPTER XXI. MYELITIS AND MYELOMALACIA. Hj-penemia and A.iiaemia of the Spinal Cord. Lesions of Pernicious Anremia. Acute Myelitis. Myelomalacia. Caisson Disease. Chronic Myelitis. HYPEU^MIA AND ANEMIA OF THE SPINAL CORD. While hypersemia of the spinal cord may occur as an accompani- ment of a general disease of a febrile character, yet as a primary affec- tion it is questionable whether it exists, and it certainly cannot be recognized. Older writers, it is true, devoted some consideration to it, and ascribed numerous symptoms to a congestion of the spinal cord, but these symptoms are better explained at the present time as being due to neurasthenia, hysteria, or general affections of a toxic or infec- tious nature, and are certainly in no way referable to either arterial or venous congestion of the spinal cord. Anaemia of the spinal cord may be said to be equally difficult of diagnosis, as it produces absolutely no characteristic symptoms. If an anaemia from obstruction in the bloodvessels is sufficiently intense to cut off the circulation in the cord, processes of softening ensue which can be recognized and which are described under the head of myelo- malacia. In conditions of general anaemia, the result of long-con- tinued malnutrition of the spinal cord is to produce degenerations of variable degree and variable extent throughout the organ. When these are sufficiently intense the symptoms caused are those of a chronic myelitis. It is very remarkable, however, that considerable degrees of degeneration have been observed after death in cases of severe anaemia when, during life, very few symptoms referable to the spinal cord have been observed. We may therefore say that both in acute and chronic anaemia of the spinal cord it is impossible to make a diagnosis of the condition ; we can only suspect their presence. PERNICIOUS AN.a:MIA AND ITS EFFECTS ON THE SPINAL CORD. There are some very marked changes produced in the nervous sys- tem by a state of pernicious anaemia,^ and although these changes when they are slight may not produce symptoms, it is as a rule possible to detect in these cases evidence of disease of the spinal cord. Patholog"y. — A diffuse sclerosis has been found in all the cases reported. This is not limited to any one system of fibres, but involves ^ Frank Billings, The Shattuck Lecture, 1902. Putnam and Taylor, Journal of Nervous and Mental Disease, 1901, vol. xxviii, pp. 1 and 74. 392 PERNICIOUS AN.^MIA OF SPINAL CORD. 393 any or all of the white columns. As a rule it is more intense in the posterior golumns, and more marked in the cervical and upper dorsal Fig. 174. Diffuse sclerosis of the cervical region in pernicious ansemia. (Billings.) segments of the cord than elsewhere; but the lateral and anterior columns do not escape, and the entire length of the cord may be affected. Figs. 174 to 176 demonstrate the lesion in cases reported by Billings. Fig. 175. Diffuse sclerosis of the cord in pernicious anaemia. (Billings.) The sclerosis is not always diffuse, but may occur in focal areas not symmetrically distributed. It resembles multiple sclerosis in not caus- ing secondary degenerations in the various tracts. This is due to the fact that in some cases there is a degeneration in the myelin only, the axone being unaffected. The gray matter of the cord is not usually involved. Some pigmentation of the neurone bodies has been seen, but chromatolysis and degeneration have not been found. The pos- terior ganglia also escape degeneration. Similar areas of sclerosis have been found in the cerebral axis, and also in various parts of the cere- bral hemis])heres; leading to bulbar palsies and also to symptoms of dementia. 394 MYELITIS AND MYELOMALACIA. Symptoms. — The general symptoms of pernicious anaemia precede the nervous symptoms for several months. The patients complain first of numbness in the legs and arms, attended by a girdle sensation and a slight loss of sensation. Imperfect control of the bladder and rectum follows, sexual power is impaired, and the limbs become weak, and reflexes are lost. Then a general paralysis develops, with some emacia- tion, but without atrophy or electrical changes in the muscles. Some- FiG. 176. Sclerosis of the posterior columns of the cord in pernicious anaemia. (Billings.) times an ataxic gait precedes the paralysis ; sometimes the gait is spastic rather than ataxic. The course of the disease is slow, with marked remissions in the symptoms. In severe cases the patients become paralyzed before the disease terminates fatally. Mental symptoms, similar to those of senile dementia, develop in the later stages. The diagnosis is to be made from the examination of the blood. Treatment. — The treatment must be directed to the underlying condition of pernicious anaemia, by food, tonics, and the use of bone- marrow and of arsenic. This is not often successful, and the percent- age of recovery from pernicious anaemia is below twenty. The exact nature of the disease is as yet undetermined. MYELITIS. Myelitis is a diffuse inflammation of the spinal cord. It may be acute, subacute, or chronic. It may be limited to one or to two adja- cent segments, and then it is called transverse myelitis. It may be more extensive and effect various levels, and then it is called dissemi- nated myelitis. It may progress either upward or downward from its starting point, and then it is called ascending or descending myelitis. It results in a destruction of the spinal elements. Myelomalacia is a condition of necrosis of the spinal cord due to embolism or thrombosis of the spinal arteries. It, too, results in a de- MYELITIS. ^ 395 structlon of the spinal elements. The symptoms of the two affections so closely resemble each other that they may be studied together. In former years myelitis was supposed to be a common disease and occupied much space in text-books. But as the spinal-cord affections, one after another, have been carefully differentiated, and as the knowledge of multiple neuritis in its various forms has grown, it has become evident that many cases formerly considered myelitis were actually of a different nature. My own experience leads me to regard myelitis as a rare affection. Etiology. — Myelitis is, in the majority of cases, an acute infectious disease. It may be the only manifestation of infection, although its bacillus still remains undiscovered. It is often secondary to some other infectious disease. The site of this infection may be a cutaneous or mucous surface which is open to the admission of germs. It has been known to follow small-pox, tonsilitis, typhoid fever, dysentery, gonorrhoea, pneumonia, malaria, grippe, cystitis and pyelitis, endocarditis, measles, felons and abscesses. All forms of meningitis, both the epidemic cerebrospinal meningitis, tuberculous meningitis, and purulent meningitis, may be followed by myelitis, either localized or general ; and after wounds of the cord or operations upon the cord that are not aseptic, access of microbes directly to the organ may cause myelitis. It is thought that occasionally the access of germs to the cord may be along the track of nerve trunks when a purulent neuritis is followed by an ascending neuritis and myelitis. The poison of rabies may cause an infectious myelitis. Muller ^ has collected thirty-six cases following inoculations against rabies by the Pasteur method. Cases are upon record in which myelitis of an acute type has developed subsequently to an exposure to cold. Thus Schultze has described a case of a young man, otherwise in perfect health, who developed an acute myelitis after travelling in a very cold railway car for several hours, and another case developing in a young man who hunted upon a very cold day for several hours. As already stated elsewhere, it is probable that such exposure to cold results in a lowered resisting power of the organs to the inviasion of bacteria already present within the body and capable of setting up an inflammatory process in any organ whose circulatory condition is materially altered. The same lowering of vitality may account for the successful attack by the bac- teria upon the spinal cord after blows, injuries, or falls. The class of the community most often exposed to the disease is the class of active workers — porters, longshoremen, those who are accus- tomed to lifting heavy weights or doing heavy work, and those who are upon their feet a great deal — also soldiers who are subject to long marches. Overexertion is an admitted cause. A great strain may be followed either by hemorrhage or by acute myelitis. Thus in a case of my own the effort made by a porter in lifting a heavy box resulted in a sudden attack of transverse myelitis attended by hemorrhage, as the autopsy demonstrated. Falls and blows upon the back are said to be responsible for some cases of myelitis. When these produce hemor- » Deut. Arch, fur Nerv., April, 1908. 396 MYELITIS AND MYELOMALACIA. rhage the symptoms resulting may be those of a transverse or dissemi- nated lesion, but an acute inflammatory process is rarely set up by this cause. A low grade of chronic myelitis is known to follow railway injuries and other severe injuries of the spine, but cases of an acute inflammatory myelitis have not been observed. A certain number of cases of acute myelitis develop after confinement. Whether these are due to infection by way of the uterus, or whether they are due to anaemia of the cord consequent upon hemorrhage, or whether they are due to a slowly acting venous congestion due to general enfeeblement, has not been determined. Syphilis is a common cause of acute myelitis. Fig. 177. Acute infectious myelitis with multiple hemorrhages,and hemorrhagic infiltration. Case was fatal in twelve days. Entire cord was affected. (Larkin.) If the cord is not affected by gummy exudations in the membranes or syphilitic deposits within the cord itself, syphilitic changes in the blood- vessels with obliterating endarteritis and consequent thrombosis of the finer vessels of the cord is the method by which the disease attacks this organ. The form of disease described by Erb as syphilitic spastic paralysis has been found to be a chronic myelomalacia of the lower dorsal region due to endarteritis with secondary degeneration.^ In some forms of poisoning, notably by lead, mercury, phosphorus, and 'Lancet, October, 1902. MYELITIS. 397 occasionally alcohol, disseminated foci of myelitis have been discovered, as well as multiple neuritis, and this has led to the supposition that the cord may be directly affected by toxins and toxic agents. That certain agents have a selective action upon the spinal cord is evident from our knowledge of the action of strychnine, which excites its activity, and of the coal-tar products and bromide and chloral, which diminish its activity. It is not impossible that the general feebleness and tremor occasionally seen in chronic bromide poisoning is due to defective nutri- tion of the spinal cord. Fig. 178. Acute tranverse myelitis in dorsal region, due to the presence of bacillus coli and streptococci. The white columns are infiltrated and in a state of acute Inflammation. (Marincsco, International Medical Congress, Paris, 1900.) Males are affected more frequently than females by acute myelitis and by myelomalacia. This is because they are more commonly exposed to the usual causes of the affection. While no age is exempt, the majority of the cases occur between the ages of twenty-five and forty-five years. Pathology. — The pathological changes in acute myelitis have been best described by Mari nesco.^ The location of the lesion may be limited to one or two segments of the cord, under which circumstances one of the symptomatic types of transverse myelitis is produced, or the lesion may be disseminated through the various segments of the cord, normal tissue intervening between the inflammatory foci, when the clinical aspect is that of disseminated myelitis. The lesion may be diffuse with a tendency to extend from one segment to another, either upward or downward, or in both directions simultaneously, in which case the 1 International Congress of Medicine, Par is, 1900. 398 MYELITIS AND MYELOMALACIA. clinical picture is that of general myelitis or of an ascending or de- scending myelitis. The gross appearance of the cord is not altered in these conditions, though palpation may reveal certain portions opposite the lesion which are softened to the touch. On cutting the cord across at these softened regions it swells up at the point of section, presenting a semifluid, creamy consistency, all differentiation between gray and white matter being obliterated, and the mass being either white and yellow from the admixture of pus, or red from the admixture of blood. When the dis- integration has not been so extreme the cross-section shows an irregu- larity of the demarcation between gray and white matter, points of congestion, and irregular plaques of yellowish or reddish color, as evidence of infiltration of the cord with inflammatory corpuscles. In other cases plaques here and there of a whitish-yellow color indicate the formation of connective tissue as the result of a process which has fully run its course. The microscopic appearance of a section of the Fig. 179. y Motor neurone bofly of spinal cord from a case of experimental myelitis produced by streptococci. The cell is invaded by leucocytes, within which the streptococci are visible. (Marinesco, International Medical Congress, Paris, 1900. ) cord in the lesion of myelitis is shown in Figs. 177 to 180. Fig. 177 shows a condition of acute hemorrhagic myelitis, the same appearance shown here being visible at all the segments of the cord. Congestion of the vessels, small hemorrhages, an infiltration of the tis- sues by leucocytes and small cells are found, and the cells of the cord are seen in all stages of degeneration, as are also the nerve fibres within the cord. These changes are shown in Plates XIX. and XX. Small cavities in the cord form as a result of the disintegration of the nerv- ous tissues. The neuroglia cells are increased in number and small nodules of neuroglia occasionally form. Granular bodies are found everywhere about the degenerated tissues and in the neuroglia. PLATE XIX 'f'*) '<£^, <« y «; l^j'.-/-/ I *\^ s^° »"t m^ h, ^:\^ 6^-*-^ ^c£: ■^^■' » ""at' , Acute Myelitis, Infiltrating Form. (Schmaus.) a, aj. Less altered tissue. 6, fc]. Swollen fibres, c, q. Distended spaces in the glia, with loss of fibres, d, d^. Granular cells with their fat removed f. Remains of destroyed nerve fibre, g. Vessel with many red and white cells. To the left of the picture the tissue is infiltrated with cells, especially in t lie iyinpli sheaths. MYELITIS. 399 Many of these cells are supposed to be phagocytes in process of destroying«1;he myelin and the axis cylinders. The tendency of these phagocytes to invade and destroy both neurones and fibres of the nervous tissue has led Marinesco to name them " neuronophages." He believes that many of them come from proliferated neuroglia cells, Various bacteria which have been found in the spinal cord, producing this infectious myelitis, are the streptococcus, the white staphylococcus, the yellow staphylococcus, the pneumococcus, and a special diplococcus. Fig. 180. Acute infectious meningo-myelitis with exudation in the pia arachnoid and hemorrhages in the gray matter chiefly. Death in 10 days. Entire cord involved. (Larkin.) The result of such a disintegration and. inflammation of the spinal cord may be its total destruction, changing it into a softened mass, purulent in character. This is the condition in the rapidly fatal cases. In other cases the destructive process is less complete, the inflammatory conditions gradually subside, and there is left a mass of scar tissue, plaques of neuroglia replacing the nerve elements that have been destroyed. Any true regeneration of tlicse nervous elements in the spinal cord appears to be impossible when the inflammation has gone beyond a certain point. It is true that the cells if not seriously dam- aged may resume their function, but when nerve fibres within the spinal cord have b(?en disintegrated no regeneration appears to be possible, and hence permanent damage to tlie cord is always the result of an acute myelitis. A complicating meningitis is not infrequently found in infectious myelitis. 400- MYELITIS AND MYELOMALACIA. There are many cases of acute myelitis in which careful investigation fails to reveal the presence of microbes. It is possible, first, that the microbes have been present and have set up an inflammatory process but have subsequently disappeared from the foci of inflammation, a condition which is known to occur in other organs ; or, secondly, that the inflammatory process is set up by toxic agents in the blood or toxins and not by the direct presence of bacteria. That the spinal cord can be attacked by toxins is evident from the changes that have been demonstrated in it in cases of alcohol, arsenic, and lead poisoning (see page 212) following diphtheria, after poisoning by ergot, by lathyrus, and by strychnine. Similar changes without bacterial influence result from trauma and are described in the chapter upon Injuries of the Spinal Cord. MYELOMALACIA. Myelomalacia or softening of the spinal cord is a lesion resulting from embolism or thrombosis in the arteries or veins of the spinal cord. Such a softening may be red when accompanied by a transudation of blood cells or by minute hemorrhages, or white when the condition has been one of simple death of the tissue, or it may be yellow if the process is not observed until after a fatty degeneration of the d6bris and of the neuroglia elements has ensued. Microscopic examination shows a mass of debris, cells, and fibres in a state of disintegration, balls of myelin, swollen or small granular bits of axones, drops of fat, and red blood globules. In all of these conditions the neuroglia presents a state of hyper- plasia with nuclear formations which may be either primary or secon- dary. If the patient lives and the products of disintegration are absorbed their place is taken by new connective tissue, leaving in the place of the nervous tissue masses or patches of sclerosis. The initial lesion, as well as the sclerotic patch which is its result, is irregular in outline and very variable in extent, the conditions being such as to prevent any strict limitation of the softening in any direction, as has been particularly shown by Obersteiner and Redlich.^ The result of a diminution in the blood supply of the spinal cord, of a suspension of nutrition consequent either upon ischsemia, or upon inflammatory conditions, is a parenchymatous degeneration of the neurones and of their branches in the region affected ; hence the changes that are characteristic of myelomalacia may be present in the vicinity of foci of acute myelitis. In these conditions the nerve cells are much swollen and disintegrated, the nerve fibres are very much swollen, producing distention of their sheaths and the cribriform appearance in the white matter of the spinal cord already described. This condition should be considered as a necrosis rather than as an acute inflammation, but its result is the production of scar tissue of the nature of neuroglia, and hence the terminal state cannot be differentiated from the terminal state produced by acute myelitis. Sometimes, however, when such a scar is distinctly wedge-shaped with base toward the periphery it will ^Handbuch der prakt. Med., Ebstein and Schwalbe, 1900. ro en CL^ CD ^ D" 3 O CD 5' 3 n ^ ^ CfQ 3 CD o O X' o 3 3 GO C !" ,— ^ 3g. C s- _M CD 3-3 ^ m ;c Xs= n CD &^s P Pg Tl Cfl o '^, ^ ■^ ^ ^ J' '"^^ ^v its' ^^i^ s-^Af ^^. 3 p in o a O D 3 CD C"■;' ._.^v.. v^;..;-' "'^* D.IX /../v z../ -as W^ c k-/// ..„ "«■;;.• z?// A if^ s./// S IV ^'^ Y^iS^-- "^J^.- i? /// XK ^:. m-. L I V.' ■ 1U S9' Ascending (D. VI to C. II) and descending (D. VIII to S. V.) degeneration in the spinal cord after a transverse lesion at D. VII. Marchi stain. (Hoohe, Arch. f. Psych., vol. xxviii., plate ix.) DEGENERATION OF THE SPINAL COED. 403 level of the lesion, but have not yet terminated in the posterior horns. But as nor'Saal posterior nerve roots enter the column of Burdach at levels above the lesion, the area of ascending degeneration in this column becomes more and more limited as higher levels are examined. The column of GoU degenerates upward in its entire length after a transverse lesion of the cord, but the extent of this degeneration in any single segment will depend upon the level of the transverse lesion. It will be remembered that the column of Goll contains all the fibres from the sacral, lumbar, and mid-dorsal segments of the cord that pass upward to the medulla. The higher the level of the lesion in the cord the greater the extent of the degeneration in the column of Goll. Figs. 27 and 28 show the varying extent of secondary degenerations after transverse lesions at different levels. The direct cerebellar column degenerates upward after a transverse lesion of the cord. Such ascending degeneration is not evident, however, if the lesion lies in the lumbar or sacral region, inasmuch as this column begins at the first lumbar segment. The higher the level of the lesion the greater the area of degeneration in this column. The column of Gowers (antero-lateral ascending tract) degenerates upward in its entire length after a transverse lesion of the cord, and the area of this degeneration is greater the higher the level of the transverse lesion. These de- generations are well shown by the Marchi stain in Plate XXI. Secondary degeneration occurs after compression or lesion of the cord at a single segment from whate^v^er cause ; thus, the compression of the cord by tumors, or. by caries of the spinal column, or injuries of the cord by fracture of the spine, or by hemorrhages outside or within the cord, or by wounds of the cord are followed by these secondary degenerations. Symptoms. — The symptoms occurring in payelitis and myeloma- lacia depend chiefly upon the location of the lesion. Two kinds of symptoms develop which can be sharply differentiated from one another. The first class of symptoms may be termed direct symptoms, and are due to destruction of the elements of the spinal cord at the point of lesion. These consist of (a) paralysis, atrophy, and reaction of degen- eration in the muscles which are supplied by the cells of the anterior horns destroyed ; (6) loss of the reflex action controlled by the segment which is affected ; (c) parsesthesia and anaesthesia in the area of skin related to the segment which is destroyed. The region of anaesthesia, as outlined upon the body of the patient when compared with Plate VII., page 46, is the best guide to the exact level of the lesion. There may be a condition of hypersesthesia just above the level of anesthesia corresponding to the two segments of the cord just above the level of the lesion. There is usually pain, and tenderness to pres- sure in the back. Hot applications are painful opposite the segment of the cord which is invaded. This is due to the hypersensitive con- diti(m of the segments just above the lesion. The second class of symptoms may be termed indirect symptoms, and are produced by the cutting off of impulses to and from the parts 404 MYELITIS AND MYELOMALACIA. of the cord below the point of injury. It will be remembered that the cord transmits many impulses upward and downward to and from the brain. In transverse myelitis the tracts conveying these impulses are damaged ; hence a certain number of symptoms develop, due to inter- ruption of the motor tracts to the parts below the lesion and to inter- ruption of the sensory tracts from the parts below the lesion. These indirect symptoms are associated with the secondary degenerations already mentioned as a result of the lesion. Such indirect symptoms are (a) motor, consisting of paralysis of voluntary motion in the legs with an increase of muscular tone in the paralyzed muscles. These become rigid, have a tendency to contraction, and produce spasms and contractures, with deformities in the joints. Such paralyzed muscles are not atrophied and have no reaction of degeneration ; (b) the reflexes are markedly increased in the parts below the lesion, so that an exag- gerated knee-jerk, a crossed adductor reflex (tapping on the knee of one side produces adduction of the opposite thigh), ankle clonus, and Babinski's reflex are present ; (c) there is a loss of the control of the bladder and rectum, attended by retention of urine and involuntary evacuations of the bowel ; (rf) sensations in the parts below the level of the lesion are uniformly impaired in all qualities : touch, tempera- ture, pain, and the muscular sense being affected ; (e) trophic symp- toms, especially bedsores over the sacrum, trochanters, and heels and ankles. These develop early, on account of venous congestion in the skin, intensified by too long-continued pressure, the position not being changed and the skin being irritated by fine particles of dirt or infected through small abrasions ; they often become extensive and deep, secrete an ichorous, offensive discharge, expose the bone, which becomes ne- crotic, and cause a general septic condition which may be fatal ; cystitis is usually to be ascribed to infection from dirty catheters or by an ascending urethritis, and is rarely, if ever, a trophic lesion of the blad- der ; (/) vasomotor disturbances, consisting of blueness and coldness of the skin, distention of the veins, and- increase of sweat. The sweat may be offensive. The onset of these symptoms may be sudden, or they may develop gradually in the course of a week or ten days. In the acute cases, which come on within a few hours, the probable condition is one of myelomalacia or a primary vascular lesion. In the subacute cases, which develop more slowly, an infectious or inflammatory origin is more likely. The occurrence of a rise of temperature and marked febrile symptoms point to the latter condition. The course of the case is a slow one, and the gradual recovery from the severe paralysis to the state of partial incapacity takes several months or years. The patients are said to be in a state of chronic myelitis during their life of invalidism. Occasionally in the lighter cases complete recovery ensues. But the damage to the cord, if serious, cannot be repaired, and hence no complete or permanent recovery is possible. On the other hand, if the causes of death, which are cystitis, inflammation of the rectum, sepsis from bed-sores, and pneumonia, can SYMPTOMS OF MYELOMALACIA. 405 be avoided by care and attention to the patient, the prospect of recovery from the attack is good. A permanent condition of chronic myelitis, the result of secondary degenerations, remains for the rest of the patient's life, and this is manifest by a greater or less degree of spastic paraplegia in which the limbs are stiff, the knees overlap in walking, there is trepidation of the feet when the weight rests on the ball of the foot ; there are spasmodic contractions and cramps in the legs, there is more or less loss of sensibility in the limbs, and an inactivity of the bladder and rectum, so that the catheter has to be used regularly and the rectum washed out regularly. Hence a state of invalidism remains, from which, however, the patients do not die unless some complication arises. The chief danger to which they are subject is an infection by the catheter, consequent cystitis, pyelonephritis, and uraemia. These various symptoms of myelitis occur in varying combinations. The most common type is transverse dorsal myelitis, of which the fol- lowing case is a fair example and demonstrates the course of the disease as well as the various symptoms which appear. Male, aged fifty-six years, of good habits, not syphilitic and per- fectly healthy, after a long walk perceived sensations of numbness and tingling in the feet and legs as high as the knees. Instead of resting, he attempted by further walking to wear off" this numbness, but before he had proceeded one-half a mile was conscious of great weakness in the feet and legs, and within one-half hour was con- siderably paralyzed, so as to be unable to stand. The paralysis was total in the legs below the knees. It was impossible to pull the knees up in bed, though they could be extended and slightly adducted. Within two hours all sensation was lost up to the level of an inch below the umbilicus, and at this point a distinct girdle sensation was perceived. Examination showed loss of sense of touch, temperature, and pain up to this level, and for two inches above this level a con- dition of extreme hypersesthesia to touch, pain, and temperature. The muscular sense was not lost in the legs, and all changes of position in the feet and toes was acutely perceived. Retention of urine was present, and there were no sensations in the rectum and no control over its action. There were no symptoms above the level of the um- bilicus. There was no fever. This condition remained stationary for about four months, during which time, by care in the emptying of the bladder and of the rectum, and by frequent change in the position of the patient in bed, complicating cystitis, proctitis, and bed-sores, which were constantly threatened, were avoided. The patient then began to recover slightly the power in the legs, but was much troubled by twitching of the muscles and sudden spasmodic contractions of the legs, sometimes in the form of flexion of the knees, sometimes in the form of extension of the foot. The paralyzed muscles did not atrophy, and the electrical reaction remained normal. Knee-jerks, which were completely lost during the first week, then returned and gradually became exaggerated, and after two weeks ankle clonus, Babinski's reflex, and crossed reflex of tlie knees appeared and have continued. 406 MYELITIS AND MYELOMALACIA. A gradual progressive recovery of power and of sensation ensued during the following two years, but was attended by an increasing rigidity of the legs. His sexual power returned. A stationary con- dition has remained for four years in which the patient has a spastic rigidity of both legs, and, though able to walk, does so with stiffness and great effort with the aid of two canes, there being tendency of the knees to overlap, a dragging of the feet, and a frequent trepidation of the foot when the weight happens to rest upon the ball of the toe. It is impossible for him to rise from the chair or to sit down without assistance. There has never been a return of the control of the bladder and rectum, there being retention of urine, which is relieved by the catheter. Sensations have returned in the previously anaesthetic area, but are by no means as keen as in the hands and arms. The girdle sensation remained for two years and then subsided. There is no longer a zone of hyperaesthesia. It is evident from this case that there was an acute onset, then a stationary period lasting several months, and then a gradual improve- ment, which progressed for six years, leaving the man at the end of that time in a stationary condition from which no recovery is likely. The level of the lesion was the tenth dorsal segment, as was shown by the level of the anaesthesia. Inasmuch as the functions of the tenth dorsal segment of the cord are very few, the direct symptoms in this case were quite subordinate to the indirect symptoms due to a cutting off of the tracts passing through this segment. I have seen a case in which a rapidly advancing transverse myelitis at the sixth dorsal segment left the patient totally paralyzed and abso- lutely anaesthetic below this level. This condition had been present four years when I saw him, and during all this time bed-sores had been prevented by having him turned in bed every half-hour day and night. The pressure of his body weight caused a redness, followed by cyanosis in the skin, which would surely have gone on to a bed-sore had this change of posture not been made. In this patient, although all sensa- tion was abolished below the level of the eighth intercostal nerve, any irritation of the skin in the anaesthetic region produced a sensation which was referred to the skin in the hyperaesthetic band at the level of the sixth intercostal nerve. Such erroneous location of sensations I have observed in several cases of myelitis, but have not seen it described. Transverse lumbar myelitis is the next most common form of mye- litis, and here the lesion may be limited to the lumbar segments or may involve both lumbar and sacral segments of the spinal cord. As an example, the following case may be cited : Male, aged sixty years, of fairly good habits and without any ascer- tainable cause, was suddenly attacked by a tingling and numbness in the legs as high as the kness and in the gluteal region. This numb- ness was followed in the course of twenty-four hours by a condition of marked weakness in the feet and legs, with drop-foot and with reten- tion of urine and inability to control the rectum. He had considerable SYMPTOMS OF MYELOMALACIA. 407 dull pain in the back over the sacrum and down the back of the thighs and legs. Within four hours of the onset he was completely unable to move his legs in bed in any direction, tliough when the knees were pulled up slight adductor movements of the thighs could be produced voluntarily. An extensive area of anaesthesia was found in the legs and in the back and outer part of the thighs, but a funnel-shaped zone on the front of the thigh was found hypersensitive rather than anaes- thetic, and there was no disturbance of sensation above the level of Poupart's ligament in front. The knee-jerks were lost, the muscles that were paralyzed in the legs were flaccid, soft, and soon began to atrophy, later on showing partial reaction of degeneration. He had a slight fever, the temperature varying from 99.5° to 101° F. for six days, when it became normal. At the end of two weeks all the symp- toms began slowly to improve. In the course of three mouths he had begun to move his limbs, though by this time they were markedly atrophied. He had developed a cystitis due to imperfect catheteriza- tion, and was still unable to control the action of his rectum. The anaesthesia, though present, was markedly diminished in intensity, and there was no point upon the limbs where sensations of touch and pain could not be located. A subjective difference, however, between the sensations of touch, temperature, and pain, as felt in the legs and hands, was present. During the following three months the improvement gradually went on, and seven months after the onset he was able to walk with a cane, but was still unable to go up and down stairs. He could rise from a chair by the aid of his hands, but in walking there was a marked weakness of all the muscles and a tendency to drop- foot. Numbness was present over the legs and back of the thighs, and it was still necessary to use a catheter. This condition has persisted for the past five years, the legs being weak, thin, and somewhat insen- sitive. The knee-jerks have never returned, and the action of the bladder has still to be assisted.. He is impotent. The entire lumbo- sacral enlargement up to the second lumbar segment was evidently diseased, but the lesion was nowhere so completely destructive as to suspend motor and sensory functions. I 'have seen a similar case, developing after a confinement that had been attended by profuse hemorrhage but no apparent sepsis. This patient went on slowly to complete recovery in six months. When the cervical region of the cord is invaded by transverse myelitis the direct symptoms appear in the arms and hands, the hands and arms below the elbow being aflFected if the lesion is in the lower part of the cervical region, and the arms and shoulders if the lesion is as high as the fifth cervical segment. A rapid onset of total paralysis with anaesthesia and reaction of degeneration in the muscles aflFected and loss of their reflex activity occurs, the ansesthesia on the body, however, being often lower down than might be expected in a cervical lesion of the cord. Thus in a case of sudden onset of paralysis aflFect- ing both hands and muscles of the forearms, excepting the supinator longus, and attended by anaesthesia in the inner surface of the arm and 408 MtELiTIS AND 3IYEL0MALAC1A. in the greater part of the forearm, excepting the radial side, the anaesthesia upon the trunk, together with the zone of hypersesthesia above it, was below the level of the nipple. Paralysis of the sympa- thetic in the neck, causing a narrowing of the palpebral fissure, retrac- tion of the eyeball and a loss of dilatation of the pupil on irritation of the neck were present. The indirect symptoms of spastic paralysis in the legs develop early. The knee-jerks are exaggerated, ankle clonus appears, the legs are stiff and rigid, and subject to quick sharp spasms of a painful character, and cramps. There is usually retention of urine, though occasionally the bladder may empty itself suddenly without the control of the patient. There is no control of the rectum, which may act in the same manner as the bladder when full. In these cases it is not uncommon for priapism to occur. These cases are more dangerous than those of transverse myelitis of the dorsal and lumbar region, because of the possibility of an ascending myelitis which may invade the cells govern- ing the phrenic nerve and cause death by respiratory paralysis. In disseminated myelitis the symptoms of paralysis with atrophy and disturbances of sensation are more extensive than in cases of transverse lesion, and symptoms appear both in the legs, body, and arms simul- taneously or in rapid succession. A few muscles in each limb may be picked out by the paralysis, adjacent muscles being weak, but showing no tendency to atrophy or reaction of degeneration. A disturbance of sensibility is usually marked, but does not go on to a total anaesthesia. There is commonly a loss of bladder and rectal control. E. W. Taylor has recorded the interesting fact that the course of labor with successful delivery and firm uterine contractions, was in no way interfered with in a case of lumbar myelitis with destruction of the entire lower cord as shown by autopsy. There was paralysis of the abdominal muscles and no power of aiding the expulsion of the child by them, but the uterus contracted rhythmically during labor. Prognosis. — In the early stage of myelitis a very guarded prognosis must be given. It is impossible to tell to what extent or severity the symptoms may develop, and until the progress is arrested the outlook is always serious. Infectious cases, especially those that* are secondary, have a better chance of recovery than cases due to disease of the bloodvessels, and the slower the onset of the symptoms the better the chance of their arrest. The more absolute the ane&sthesia in any case the worse is the prognosis. The greater the pain in the back, especially if it is attended by rigidity of the spine, or spasms in the legs, the worse the prognosis. After the acute onset the prognosis will depend on the severity of the symptoms. Bed-sores and cystitis make the outlook less hopeful. After very complete paralysis I have seen a slow but progressive improvement and a return of power to walk. It is always well to hold out the hope of some improvement for two years. After that time no change can be expected. The j^ounger the patient the more vigorous his general condition, and the better his habits the better his chances. Symptoms of myelomalacia. 400 Treatment. — Absolute rest in bed must be enforced from the out- set. The4)atient should lie in a prone position, if possible, and great care should be taken to change the position at least every half-hour and to so distribute the weight, by means of pillows or by an air-bed or water-bed, as to prevent pressure of the body coming upon the bony points. Frequent sponging of the skin and careful attention to cleanliness, especially if there is an incontinence of urine or feces, are necessary in order to prevent the development of bed-sores. Under all circumstances the use of the bed-pan or urinal should be enforced, as the effort of sitting up may increase the symptoms. If the skin is very red it should be bathed with an antiseptic solution and with a solution of alum and covered with a powder, and no pressure on it permitted. If a bed-sore forms it is to be dressed like any ulcer and kept as aseptic as possible. Stimulants to healing, such as balsam of Peru or a galvanic current directly applied, have never seemed to me to be effective. The most important thing is to remove all pressure from the ulcerated surface, and thus allow it to heal. The greatest care should be taken in catheterization, all catheters should be kept in antiseptic solutions and carefully washed, and coated with carbolized vaseline before being used. It is too commonly the custom to limit the washing of the catheter to the time succeeding instead of preceding its use. If cystitis develops the bladder should be washed out twice or three times a day with a 10 per cent, solution of boric acid at 98° F., and the patient should be given salol, five grains, or urotropin, ten grains, every four hours by the mouth. The action of the bowels should be regulated by means of laxatives daily, which should be as- sisted by an injection given at a regular time of the day, a pad being constantly applied to the anus to prevent leakage. The spasmodic contractions and cramps in the muscles may be relieved by the use of warm cloths to the back or of warm bottles, care being taken not to burn a blister in the anaesthetic skin. Bromides are sometimes of much service for this symptom. Any active medicinal treatment for the disease during the period of onset or during the period of maximum intensity is usually futile, though salicylate of soda and salicin have been supposed to be of use in infectious cases. When the active proc- ess has come to a standstill and a period of improvement sets in, massage and electrical applications to the muscles, gradually increasing voluntary movements and exercises, tending to reestablish the volun- tary control of the limbs, are to be used. Baths are also of service, either warm and cool sponging of the back, or warm douches under moderate pressure (ten pounds), or tepid sitz baths with sponging of the back. Cold (60° F.) or hot (100° F.) baths are to be avoided. When the patient begins to walk great care should be taken to pre- vent over-fatigue and to prevent falls. Rubber heels or soles help these paralytics. In some cases apparatus may be used to facilitate the act of walking, such as braces to the ankles or knees or a stiff corset to the back. 410 MYELITIS AND MYELOMALACIA. CHRONIC MYELITIS. Etiology. — Chronic myelitis may develop as the result of an acute myelitis when this does not go on to a fatal termination but leaves a certain amount of damage to the spinal cord. A myelitis, however, may be from its onset a slow process, and thus the disease may be chronic from the start. Any of the causes capable of causing acute myelitis are equally capable of producing chronic myelitis. Distur- bances in the circulation of the cord are probably the primary cause in the majority of cases, atheroma of the bloodvessels or syphilitic en- darteritis being among the most common conditions found in this disease. Chronic myelitis may also develop as the result of chronic meningitis, and is usually associated with more or less implication of the spinal meninges. The term chronic myelitis may be applied to any or all of the processes of degeneration developing in the cord in con- nection with other spinal-cord affections. Thus the terminal stage of lateral and combined sclerosis, of amyotrophic lateral sclerosis, of acute or chronic poliomyelitis, or of disseminated sclerosis may be diagnosticated as chronic myelitis in case no exact history of the onset of the disease can be obtained. Chronic myelitis may also occur without any ascertainable cause, a chronic irregular degeneration, both in the white columns and in the gray matter being found after death in cases where the disease has been one of slow and irregular type. Chronic myelitis may develop in the course of very many constitu- tional diseases that produce disturbance of general nutrition, and it may also develop secondarily to many of the acute infectious diseases, being in these cases due rather to the toxic agents produced by the infection than to any direct bacterial action. Any of the various forms of injury of the spinal cord or of its bony covering, or of its meninges, may be followed by a chronic degeneration in the spinal cord. Very great and long-continued physical exercise is also supposed to be a cause. Chronic alcoholism may produce a chronic myelitis as well as a periph- eral neuritis, and in some cases of very severe peripheral neuritis which failed to recover, changes have been found of a degenerative nature in the spinal cord. Thus chronic myelitis may be the result of a state of poisoning which produced first a multiple neuritis. Chronic myelitis develops occasionally as a sequel of gout with or without an attending multiple neuritis, the exact nature of the process being still a matter of uncertainty. Chronic myelitis is in some cases due to syphilis, and then it may be either a diffuse process advancing into the cord from the periphery, attended by degeneration which appears first in the lateral columns of the cord and gives rise to the symptoms of spastic paraplegia (the spinal syphilis of Erb), or it may be a trans- verse process due to syphilitic endarteritis in the vessels at a certain level, or to the appearance of syphilitic indurations with infiltration of round cells in both white and gray matter. Such syphilitic infiltra- tions may be very irregular in their distribution ; may be confined to one segment of the cord, causing a chronic transverse myelitis ; may CEBONIC MYELITIS. 411 be limited to one or more columns of the cord, causing symptoms resembling focomotor ataxia or lateral sclerosis of one or both sides ; may cause a unilateral lesion, producing symptoms of Brown-S6quard paralysis, or may be irregularly disseminated throughout the cord. Fig. 181. Chronic myelitis. Swelling, degeneration, and sclerosis in the cord, a, degenerated tissue ; 6, cell in state of chromatolysis ; c, c', swollen axis cylinders; d, sclerotic meshes of glia; g, vessels with thickened walls ; n, enlarged meshes of glia. (Schmaus-Sacki. ) Chronic myelitis is sometimes due to pernicious anaemia, the changes in the cord which occur in the course of this disease being diffuse in location and slow in development^ (see page 393). Slow poisoning by ergot or lathyrus may cause chronic myelitis. Chronic myelitis is usually a disease of adult life, persons of the ages from twenty-five to forty-five years being more liable to the affection ; it is about equally prevalent in males and females. It is possibly traceable in some cases to an inherited neuropathic tendency. It is, in ' F. Billings, The Shattuck Lecture, 1902. 412 MYELITIS AND MYELOMALACIA. my experience, a very rare form of affection, and it is probable that the descriptions found in the older text-books, which rarely correspond to cases now seen in the hospitals, were written before the time when careful distinction was made between the various forms of spinal-cord disease. As the knowledge of spinal -cord disease has increased it has been possible to separate accurately the different types, and the more this is done the rarer the diagnosis of simple chronic myelitis is made. In fact, some authors have denied that as a special disease it can occur, and affirm that it is always to be regarded as the terminal stage of some one of the diseases of the cord already considered. Pathology. — The pathology of chronic myelitis cannot be traced accurately from the onset, inasmuch as the terminal stage of the disease is the only one which is open to direct observation. In patients dying from chronic myelitis the spinal cord may present slight deformities in its contour, but the changes are only visible in microscopic section. These changes are of two varieties : first, the appearance of sclerotic patches irregularly distributed throughout the white and gray matter of the spinal cord. These sclerotic patches consist of a thickening of the glia and of the connective tissue about the bloodvessels, usually attended by a distention of the bloodvessels and by changes in their walls of the nature of endarteritis — both obliterating or atheromatous. As a rule, this sclerotic tissue is thicker and more dense than in the ordinary forms of secondary degeneration, or in tabes, or in lateral sclerosis. Occasionally in the mass of sclerotic tissue a distended axis cylinder has produced a cavity, and the cord presents the cribriform appearance, or Swiss cheese appearance, which has been described as occurring in acute myelitis. Usually the sclerosis is thicker at the periphery of the cord, and is associated with adhesions of the pia and with chronic meningitis. It has a tendency to be thicker around the bloodvessels that enter the cord from the periphery, and very often wedge-shaped patches of sclerosis are seen with the apex pointing inward. It is evident that the neuroglia of the cord is increased in density and also that the connective tissue in the vessel walls is thickened. All throughout this sclerotic tissue a fine cell infiltration is found, more intense about the bloodvessels, but everywhere present in the cord. If this irregular sclerotic process has gone on chiefly in the surface of the cord the so-called annular sclerosis is found, a ring of sclerotic tissue surrounding the fairly normal fibres of the cord, the gray matter not being greatly affected. This condition is rare. The second pathological change present in chronic myelitis is an advanced degeneration of the nerve fibres and of the nerve cells through- out the cord. This may be very irregular in its distribution ; and its extent at the time of the autopsy is very often much greater than the extent of the initial sclerosis to which it is secondary. Secondary degeneration follows each nerve fibre to the end where it is interrupted at any point in its connection with its proper neurone body. Thus a small patch of sclerosis at one level of the lateral column may be fol- lowed by a long descending degeneration in that column. And a small CHBONIC MYELITIS. 413 patch of scl^osis in the posterior column may give rise to ascending degeneration as high as the medulla. It is not always possible to determine from a microscopic examina- tion whether the primary process in a chronic myelitis is due to a degeneration of the nerve fibres with secondary sclerosis, or whether it is due to a primary sclerosis with compression and secondary degen- eration of the nerve fibres. Both conditions are found in the terminal stage of the disease. In some sections it is evident that the thickening of connective tissue is greater than that which occurs in an ordinary replacement hyperplasia. In other regions, however, the sclerosis is quite comparable to that ordinarily found in secondary degenerations. It is this mingling of the two processes in chronic myelitis which gives rise to the irregular appearance found. Enormous swelling of the axis cylinders, such as is found in acute myelitis, is occasionally observed in chronic myelitis ; and granular corpuscles, the products of degenera- tion, are scattered everywhere through the spinal cord. The distribu- tion of these lesions through the cord varies in different cases, both transverse and disseminated chronic myelitis being found. Symptoms. — The symptoms of chronic myelitis are such as might be expected from a chronic implication of the various columns of the spinal cord and of the various segments of the cord in their gray mat- ter. The process is such a slow one, however, and so diflFerent in dif- ferent cases that no typical course can be described in this disease. A general feeling of weakness in the legs, undue fatigue on exertion, going on gradually to a state of paralysis of greater or less degree, perhaps not sufficient to incapacitate the patient for many years, is the usual history. The paralysis is usually of the spastic type, with rigidity in the muscles, but it may be confined to a few muscles in the limb affected, and these muscles may show atrophy and reaction of degeneration. As a rule, there is in the early stage an increase in the tendon reflexes, with ankle clonus, and this may persist for several years and finally subside, and in the later stages the reflexes may be lost. Occasionally, if the lesion happens to afi'ect the reflex arc, the knee-jerks are lost at the outset. The legs may be the only parts paralyzed for many years, or the paralysis may extend within the first year to the arms, or it may begin in the arms, but usually after several years some symptoms of paralysis are present in all the extremities. The paralysis rarely is as extreme as it is in the forms of muscular atrophy, and therefore rarely gives rise to permanent deformities or contractures. These, however, are occasionally seen. Disturbances in the sensory sphere are very common in myelitis. These usually consist of irregular parsesthesise which may be followed by plaques of ansesthesia and analgesia. Pain is not a common symp- tom, excepting in so far as the general stiffness and weakness give rise to extreme sensations of fatigue or painful affections of the muscles paralyzed, l)ut pain in the back is not uncommon when the myelitis is associated with a chronic meningitis. In these cases also some shoot- ing pains in the limbs and in the body may develop. 414 MYELITIS ANT) MYELOMALACIA. Trophic disturbances are quite common in the course of chronic myelitis. Urticaria, irregular eruptions U})on the skin, vasomotor paralysis, giving rise to flushes or streaks of pallor, irregular growth of the hair and of the nails, trophic disturbances of the joints and of the bones are not uncommonly observed in the course of chronic mye- litis. In the last stage of the disease bed-sores are a distinct danger. But all these symptoms may vary in intensity, and many of them may be recovered from with proper care. In the majority of cases as the result of a long-continued chronic myelitis the patient is reduced to a state of helpless paralysis. He is confined to the bed, the limbs are wasted and very often drawn up in a condition of contracture, extreme flexion of the thighs and legs with adduction of the thighs being present. In these cases the hands, too, become helpless, and the patient is reduced to the most abject state of paralysis. A complicating cystitis not infrequently develops, quite early in the course of chronic myelitis, as the irregular action of the bladder may necessitate the constant use of catheters which convey infection. Cystitis and bed-sores are the common cause of death in this disease, though, if the sclerosis invades the upper portion of the cord, affections of respiration and deglutition may be the active cause of death. Many symptoms referable to an implication of the sympa- thetic nervous system are usually observed in the course of chronic myelitis, crises such as occur in locomotor ataxia having been observed frequently. If the first dorsal segment of the cord happens to be affected irritation or paralysis of the sympathetic in the neck will give rise to pupillary symptoms. Diagnosis. — The diagnosis of chronic myelitis is to be made entirely by exclusion. When all other forms of spinal-cord disease can be eliminated, and yet objective spinal symptoms of irregular distribution are present, chronic myelitis may be diagnosticated. When the typical symptoms of the various spinal-cord affections are considered, it will be evident that they are none of them likely to be confounded with chronic myelitis. The most difficult diagnosis is from disseminated sclerosis. In this disease, however, it is to be noted that nystagmus and other cerebral symptoms appear early, of which the most notice- able are the scanning speech and the intention tremor. The develop- ment, therefore, of cerebral symptoms in the course of the case of sup- posed chronic myelitis will indicate that the original diagnosis should have been disseminated sclerosis. In the early stage of primary lateral sclerosis or of amyotrophic lateral sclerosis, where the only symptoms are spastic paraplegia and where no ostensible cause can be found, the supposition of a transverse chronic myelitis of the dorsal region may be entertained ; but this diagnosis can only be substantiated when the development of sensory symptoms and disturbance in the action of the bladder and rectum make it probable that the lesion is more extensive than one affecting the pyramidal tracts of the spinal cord alone. Prognosis. — The prognosis in chronic myelitis is uniformly unfay^ orable, and the patients are destined to a life of invalidism. SENILE PABAPLEGIA. 415 Treatment. — The treatment of chronic myeUtis is one of care of the general health, the various symptoms of the disease being coun- teracted as they arise, so far as possible, and the patient kept in a state of comfort. All the measures already recommended in connection with the treatment of locomotor ataxia may be carried out in the course of chronic myelitis, and are not infrequently attended by temporary improvement. This is particularly true of hydrotherapeutic treatment and the careful . application of massage. A prevention of any of the complicating diseases which cause death will be a duty in the latter stages. SENILE PARAPLEGIA. People of advanced years, especially those who are rather feeble, are occasionally afflicted by a slowly progressive weakness of the legs, attended by pain and sensations of numbness, undue fatigue upon exer- tion, and possibly with a slight degree of difficulty in the retention of urine. These symptoms give rise to the suspicion that they are about to develop chronic myelitis. They are of very slow progress and pro- duce disturbance of walking which results in a slight dragging of the feet, or rather shuffling gait, difficulty in getting up stairs or stepping up into a carriage, but not attended by any true ataxia of movement. A great sense of weariness is usually experienced in these patients after any exertion, and they may be the subjects of senile tremors of the hands or of the head. Very many patients afflicted in this way are the subjects of chronic arterial disease or of chronic affections of the heart. The condition may develop occasionally quite rapidly, all the symptoms appearing within a few days, and making it difficult for the patient to move about at all. In my own experience the affection is more common in women than in men. It is probably due to some dis- turbance of circulation in the lower part of the spinal cord, either of the nature of an arterial anaemia or of a venous congestion. This seems likely because of the fact that in the majority of cases absolute rest in bed, skilful massage, the use of hot applications to the spine, followed by cool douching, heart stimulants, and general tonics result in a pro- gressive recovery. Autopsies are wanting to establish the pathology, as no fatal cases have been recorded. Disseminated or insular sclerosis of the spinal cord is so commonly associated with similar lesions in the brain that it will be discussed in connection with brain diseases. CHAPTER XXII. INJUEIES OF THE SPINAL COED. H^MATOMYELIA. Injueies and wounds of the spinal cord, producing a laceration of the cord and hemorrhages within it or in the spinal canal, are of fre- quent occurrence. The usual cause of such injuries to the cord are fractures and dislocations of the vertebrse. It is natural from the length of the organ and from its location within a narrow cylindrical bony canal that any injury to the wall surrounding it should have a direct or indirect effect upon it. And it is rare for a fracture or a dis- location of the vertebra to occur without producing immediate damage of the spinal cord. Fig. 182. \ Hemorrhage in the central gray matter on the left side, due to fracture of the spine. First dorsal segment. Pathology. — Such an injury is usually attended by a compression and bruising, or by a laceration of the cord, a disintegration of its substance, and a considerable hemorrhage that perforates the cord and is followed by an inflammatory process that may intensify the disinte- gration or may go on to an attempt at repair by the formation of an extensive cicatrix. Fig. 182 shows the appearance of a spinal cord after a fracture of the sixth cervical vertebra. The eighth cervical segment was crushed and disintegrated, the typical form of the gray matter being obliterated and the tracts in the white matter torn across. 416 PATHOLOGY. 417 A large hemorrhage occurred which penetrated the cord and destroyed the gray master for some distance above and below the eighth cervical segment, being largely confined to the gray matter, especially of the posterior horn. The clot followed the line of least resistance, which is always within the gray matter. (Fig. 183.) lu another case, where the fracture occurred in the dorsal region, the hemorrhage surrounded the cord above and below the destroyed segment, filling the spinal canal within the dura. When the cord is exposed at the autopsy or in a surgical operation for the repair of the fracture, its external appearance may not be changed, and if the pia and sheath be not ruptured there may be Fig. 183. Clot in the posterior horn of the left side, three segments above the preceding figure. Sixth cervical segment. very little evidence of the extreme destruction present. It is only upon section that the lesion is evident. Thus, in the case from which the figures are taken external inspection showed no lesion of the cord. But on cutting the cord the general disintegration and hemorrhage were visible. Bullet wounds and stab wounds of the cord cause a similar disintegration of the spinal elements and a hemorrhage without or within the cord. These are more likely to be followed by purulent in- flammation, the result of septic material brought in with the bullet or knife. This inflammatory condition involves the meninges as well as the cord itself, and may go on to purulent myelitis or to abscess of the cord with total destruction in a transverse direction. Hemorrhages within the cord cause a long, narrow, cylindrical clot usually within the gray matter, sometimes in the posterior columns, very rarely in the 27 418 INJURIES OF THE SPINAL CORD. H^MATOMYELIA. lateral columns. The maximum destruction is limited to one or two segments, but the clot may perforate a number of segments, even to one-half the lengtli of the cord. Sometimes many little clots are found at diflFerent levels. Occasionally the capsule of the cord is broken and the hemorrhage reaches the pia and infiltrates it. If the patient dies at once the clot is found surrounded by broken-down cord tissue filled with small cells and granular corpuscles — pigment and hsematin crystals. If the patient survives a few weeks the clot may be somewhat absorbed and contracted, the tissue about it may be yellowish-red from fatty degeneration of the nerve elements, and the cells and fibres will be found in all stages of disintegration. If the patient lives for some years the clot may be entirely absorbed, leaving either a long scar of connective tissue or a long cavity lined with con- nective tissue — the hemato-myelo-porus of Van Gieson. Hemor- rhage from the vessels of the pia mater, causing compression of the cord by a clot, may occur. Janeway has described such a condition occurring in a patient, the subject of hemophilia. I have seen one case which came on suddenly after unusual exertion. In the patients who survive secondary degenerations develop in the cord, as in cases of transverse myelitis, above and below the injured segments ; secondary degeneration also occurs in the motor nerves from the segment injured, to the muscles, and atrophy of the muscles, as in anterior poliomyelitis. Etiology. — Hemorrhage within the spinal canal or within the cord may be the result of severe blows or falls or of direct concussion. Thus I have seen multiple hemorrhages in the pia with a number of small clots outside the cord following a fall through an elevator shaft down five stories. I have also seen a hemorrhage in the conns ter- minalis due to the patient sitting down where there was no seat and injuring the buttocks. Hemorrhages may also occur as the result of spontaneous rupture of bloodvessels, either after great effort or after long-continued arterial disease. In one case a severe sudden bending forward of the neck without dislocation or fracture was followed by a hemorrhage within the cord which penetrated through the cervical and half the dorsal segments, causing a long tubular clot. In another case the lifting of an unusual weight was immediately followed by a hemor- rhage in the lower lumbar region which destroyed the lumbar and sacral enlargement and infiltrated the cauda equina, the latter being found embedded in the clot at the autopsy. Symptoms. — When a vertebra is dislocated or fractured a displace- ment either of the spinous process or of the body of the vertebra can be felt, causing a double deformity. The muscles about the fractured bone are thrown into a state of rigidity, and a fixation of the back or neck in an abnormal position occurs. Any motion causes great pain both at the point of injury and in the domain of nerves which are compressed or lacerated. The exact character of the injury can only be determined by an examination with the Roentgen rays. There are SYMPTOMS. 419 some cases on record where slight fractures or partial dislocations «jaused no •Spinal symptoms. There are many cases where the spinal symptoms exceed in severity any apparent surgical injury, for disloca- tion may be temporary and spontaneously reduced. The surgical aspect of the case, however, is of less interest than the symptoms of spinal lesion. These depend wholly upon the position of the injury; the higher the fracture the more extensive the symptoms, for the injury usually arrests all voluntary motion and conscious sensation in the parts below the level of the lesion. Hence the level of the symptoms indi- cates the level of the lesion. The facts of the localization of spinal functions already presented in detail need not be reproduced here ; but if carefully studied they will permit an accurate diagnosis of the posi- tion of the lesion to be reached. Particular attention should be paid to the posture involuntarily assumed by the patient in bed (see page 66), to the extent 'of the paralysis (see page 48), and to the distri- bution of the anaesthesia (see page 73). In any case of injury of the cord a state of paraplegia is produced at once. The paralysis is complete, the limbs are relaxed and flaccid, the reflex action is much diminished, and, in cases of transverse lesion with entire division of the cord, is lost ; there is incontinence of urine with retention and a paralysis of the rectum ; there is often a state of priapism ; there are pains in the back at the level of the fracture and in the region of the body to which the- nerves near the fracture go ; these pains are often attended by twitchings of the muscles which cause much discomfort ; there is a condition of anaesthesia more or less com- plete whose upper limit is easily defined and corresponds to the level of the lesion ; there is a zone of hyperaesthesia just above the anaesthetic level. The segment of the cord which is affected in any particular fracture is the one that is opposite the fractured vertebra. The researches of Reid^ have demonstrated that there is not a uniform relation between the vertebrae and the segments of the cord. The diagram (Fig. 184) of Dejerine shows the usual relation between the various segments of the cord -and the spines and bodies of the vertebrae. Chipault has given a practical set of rules for determining the rela- tion of the segments to the spinous processes of the vertebrae. He says : "In the cervical region add one to the number of the vertebra, and this will give the segment opposite to it. In the upper dorsal region add two; from the sixth to the eleventh dorsal vertebra add three. The lower part of the eleventh dorsal spinous process and the space below it are opposite the lower three lumbar segments. The twelfth dorsal spinous process and the space below it are opposite the sacral segments." The spinal cord ends opposite the body of the first lumbar vertebra or opposite the cartilage in the space below it. The lower half of the spinal canal is, therefore; occupied by the cauda equina only. In infants the cord reaches as low down as the body of the 'Journal of Anatomy and Physiology, vol. xxiii., p. 312. 420 INJURIES OF THE SPINAL COBD. IIJi:MATOMYELTA. Fig. 184. N. to rectus lateralis .'^to rectus antic, minor Aiuisloniobis with hypoglossal Anastomosis with pneutnogastric N. to rectus antic.major. N. to mastoid region. .Great auricular n. ■Transverse cervical n. '-.Ei^\N. to Trapezius, Ang. Scap. and Rhomboid . Supra clavicular n. _Supra-acromiaL n. _ Phrenic n. A', to levator ang. scap, N. to rhomboid Subscapular n. Subclavicular n. jV. to peetoralis major. Circumflex n. Blusculo-cutaneous n. Median n. Radial n. Ulnar n. Internal cutaneous «. Small internal cutaneous ». Ilio-hijpogaslric n. .llio-inguinal n. —External cutantous n, Genito-crural ;i. Anterior crural n. ' Obturator n. JV. to levator ani. If. to obturator N. to ophincter ani..^ Coccygeal ?i, Superior gluteal n. JV. to pyriformis lY. to gemellus super. ^ N. to gemellus infer. df. to guadratus .Small sciatic n, -Ssiatic n. The relation of the segments of the spinal cord and their nerve roots to the bodies and spines of the vertebr*. (Dejerine et Thomas, Mai. d. 1. Moelle Epinifere, Paris, 1902.) INJURIES OF THE SPINAL CORD. H^MATOMYELIA. 421 third lumbar vertebra, but by the age of twelve years the lower limit is opposite the body of the second vertebra. Certain special symptoms attend fractures at different levels : I. Fractures of the upper four cervical vertebrae are usually attended by sudden death either from involvement of the vital centres in the adjacent medulla or by a lesion of the centres of the phrenic nerves and respiratory paralysis. In a few patients who have survived for a few days pain has been felt in the great occipital nerves ; the head has been held rigid, and any motion of it has been very painful ; a deformity has been felt in the pharynx ; a total paralysis of the entire body below the neck has occurred, and high or subnormal temperature with very rapid pulse have been present. Death always follows soon. II. Fractures of the lower three cervical and first dorsal vertebrae are quite common, over one-quarter of spinal fractures being in this location. Divers into shallow water, laborers whose heads are bent forward upon their bodies, and persons struck by heavy objects falling Qn the shoulders, or who fall from a height in such a manner that the body is bent upon the head usually fracture one or more of these cervi- cal vertebra. In such fractures either the upper or the lower half of the cervical enlargement of the cord is injured. (a) When the upper part of the cervical enlargement is injured the paralysis of the arms is complete and the arms lie relaxed at the side of the patient ; pain is felt in the neck and shoulders, and spasms of the arm muscles are frequent ; anaesthesia is complete below the deltoid area over the shoulder-joint (Fig. 185) ; there is no reflex activity in the upper extremities, but there may be some reflex activity in the legs after the shock passes off, and this soon becomes exaggerated ; there is usually constant priapism, and retention of urine which must be relieved by catheter ; there is paralysis of the rectum ; there is a marked tendency to bed-sores, respiration is wholly diaphragmatic ; coughing and expiratory efforts are impossible ; a high fever and rapid pulse develop ; unusual sweating of the entire body occurs, and, as a rule, death follows during the first or second week. If the patient survives a state of nearly complete paralysis remains, with atrophic paralysis of the arms and spastic paralysis of the legs. (6) When the lower part of the cervical enlargement is injured the paralysis of the arms is partial, the shoulder muscles and the flexors of the forearms escaping, and sometimes some of the muscles of the forearms also escape. In this form the patient lies with the arms abducted from the sides and the forearms flexed, the hands resting on the chest ; the body and legs are paralyzed and flaccid, pain is felt in the hands and on the inner side of the forearms and often about the body ; the anaesthesia is more marked on the inner side of the arms and forearms and is total in the hands (Figs. 186 to 188) ; it affects the body also, but the upper limit of anaesthesia on the body may be as low as the ni[)plc ; there is a region of hyperaesthesia on the outer side of the arm and about the chest above the nipple ; there may be a temporary suspension of reflex action in the legs, followed later by 422 INJURIES OF THE SPINAL CORD. H^MATOMYELIA. exaggeration of all the reflexes ; there is priapism, retention of urine, paralysis of the bladder and rectum ; there is an oculo-pupillary par- alysis with contraction of the pupil, failure to dilate when the neck is scratched, and a narrowing of the palpebral fissure, and retraction of Fig. 185. Fig. 186. Anaesthesia caused by a lesion of the fifth cervical segment. Anaesthesia caused by a lesion of the sixth cervical segment on the right side, and involving the fifth seg- ment of the left side. the eyeball, these symptoms indicating a lesion of the first dorsal seg- ment, but being frequently present with any injury of the lower cervi- cal enlargement. The paralysis is not always symmetrical in the arms^ and many muscles may escape if the lesion is low down in the en- FiG. 187. Fig. 188. Anaesthesia caused by a lesion of the seventh Anaesthesia caused by a lesion of the first cervical segment, almost symmetrical. Dotted dorsal segment. Dotted area hyperaesthetic. area hyperaesthetic. (Herter.) (Bruns.) largement. (Fig. 189.) If the patient recovers a state of atrophic paralysis in the upper extremities of greater or less extent remains and a condition of spastic paralysis in the legs. III. Fractures of the dorsal vertebrae are rather infrequent. They INJURIES OF THE SPINAL CORD. H^MATOMYELIA. 423 cause a paralysis of the legs and abdominal muscles with loss of con- trol of bladder and rectum. There is a distinct girdle sensation often attended by pain. There is a line of anaesthesia about the trunk above 189. Anaesthesia of the right side due to lesion of the fifth cervical segment ; of the left side due to lesion of the first dorsal segment ; tongue-like extension of ansesthesia in axilla and under the arm. (Wiohmann.) which is a zone of hypersesthesia. This line of anaesthesia is usually the most important guide to the level of the lesion, but it is not on a line with that level. (Figs. 190 and 191.) It is from three to four inches below it, for there is an overlapping or anastomosis of the Fig. 190. Fig. 191. Ansesthesia from injury of the seventh dorsal segment, showing the anjesthesia curve about the body and extending higher behind than in front. (Wichmann.) Anaesthesia due to lesion of the eleventh dorsal segment. (Wichmann.) sensory filaments of nerves, and the intercostal nerves of one segment supply the skin directly related to two or even three adjacent segments, as Sherrington^ has shown. (Fig. 192.) Furthermore, the sensory ^Transactions of the Royal Philosophical Society, London, 1893, vol. clxxxiv. 424 INJURIES OF THE SPINAL CORD. H^MATOMYELIA. fibres entering the cord ascend to the two higher segments before terminating, and hence a transverse lesion of the cord does not cut off all the sensations from the nerve roots at its level. This will be understood by reference to Fig. 22. Thus in a case of fracture of the sixth dorsal vertebra the eighth dorsal segment was injured, and the line of anaesthesia corresponded to the distribution of the tenth dorsal nerve, being three inches below the level of the injury. The zone of hypersesthesia which lies above the level of the ansesthesia is due to the fact that sensations received in segments of the cord just above the lesion which are in a state of inflammatory irritation are felt to an exaggerated degree. The girdle sensation is a consciousness of this hypersensitive skin. If the cord is not severed and is capable of Fig. 192. ^ Tth thoracic sensory spinal SKin-Jield wwww 'II//IIIIIII/I \%\\\\\\\\\\\ mil WW \W\\\\\\\\\\\\\\\\\\\\\ \\w\\\\ LLUlUlLiL w Illrd thoracic Vth thoracic Diagram showing tlie nerve supply of the slcin of the thorax ; every part is supplied Dy two nerves. (Sherrington. ) transmitting some sensations through the lesion, patients often refer to this hypersensitive zone, all sensations coming up from the legs. Thus in one of my patients a pin-prick, or pinch, or touch, or a thermal sensation produced in the paralyzed and anaesthetic legs or trunk, was always felt about the level of the sixth dorsal nerve. If on the back of the body it was referred to the back ; if in front, to the front, and it was always felt on the side which was irritated. This man had a transverse injury of the cord due to fracture at the fifth dorsal vertebra and had been paraplegic four years when examined. I have seen this symptom in many cases, but have not seen it described. It is of service in distinguishing total from partial lesions of the cord. In cases of dorsal injury the initial symptoms may slowly subside in part. The patient remains, however, in a state of spastic paraplegia with all the symptoms of lateral sclerosis due to the descending degeneration in the motor tracts of the cord. The danger of bed-sores and cystitis is directly proportionate to the degree of anaesthesia remaining. Some- times these patients live for many years. It is in cases of dorsal injury that the syndrome due to a lesion of one lateral half of the cord, known as Brown-Sequard paralysis, is most commonly seen, although it may result from a lesion at any level. This combination of symptoms is as follows : 1. On the side of the lesion there is paralysis with rigidity; an INJURIES OF THE SPINAL GOBD. H^MATOMYELIA. 425 Fig. 193. .X increase of tendon reflexes; vasomotor paresis causing a temporary- elevation of temperature ; a diminution in muscular sense and of the sense of position of the leg; a general hypersensitive state to touch, temperature, and pain up to the level of the lesion, which may subside after a time ; there is a narrow zone of anaesthesia about the body to touch, temperature, and pain, the latter two being lost a little lower than touch. 2. On the side opposite the lesion there is no paralysis ; there is a slight increase of tendon reflexes; there is anaesthesia to touch, tem- perature, and pain up to a line passing about the body about an inch lower than the zone of anaesthesia on the side of the lesion. The accompanying diagram (Fig. 193), illustrating the course of motor and sensory fibres through a cord, which is supposed to have a lesion involv- ing one segment on the left side only, demonstrates the manner in which these symptoms are pro- duced. The lesion interrupts the transmission of nervous impulses of various kinds. In a patient of my own a fracture of the seventh dor- sal vertebra caused this syndrome, which was partly relieved by oper- ation. The fractured lamina that compressed the cord was removed, the symptoms gradually subsided, but even at the end of eight years there remains some stiffness of the paralyzed leg and an appreciable difference of sensation on the two sides in the legs. IV. Fractures at the lower two dorsal and upper lumbar vertebrae are the most common fractures of the spine, over one-half of the cases being in this locality. Such frac- tures injure the lumbar enlargement of the cord. (a) When the upper part of the enlargement is destroyed there is total paralysis with flaccid condition in the abdominal muscles and in the muscles of the thighs, with in- creasing atrophy and reaction of de- generation and a loss of reflex action ; SSquard paralysis. there is paralysis without atrophy or change in electric reaction in the muscles of the legs and feet; the legs lie extended in bed and cannot be drawn up; there is loss of AncEsthesiu, Analgesia Diagram showing the eflfect of a unilateral lesion of the spinal cord, causing "Brown- 426 INJURIES OF THE SPINAL CORD. HMMATOMYELIA. voluntary control of the bladder and rectum, with retention of urine ; compression of the testicle does not cause a contraction of the abdomi- nal muscle of the same side (Kocher's reflex) ; there is anaesthesia as high as Poupart's ligament about the body, and the girdle sensation is located here. Sometimes, however, when the upper two lumbar seg- ments escape, the funnel-shaped area on the front of the thigh is hyper- sensitive (Fig. 194), a sense of pain is felt there, and the anaesthesia is Fig. 194. Fig. 195. Area of anaesthesia in a lesion of the lower two lum- bar segments on the left side and entire lumbar enlarge- ment on the right side. (Starr and McBurney.) Ansesthesia after a lesion of the fifth lumbar segment. (Eulenberg.) confined to the area corresponding to the third lumbar segment and the segments below it. If these patients recover in part, as they often do, a great degree of inability remains, as the thighs are paralyzed. (6) When the lower part only of the lumbar enlargement is de- stroyed, the upper portion escaping, the paralysis is limited to the legs below the knees, or even to the peronei and feet, and these muscles are soon atrophied and show a reaction of degeneration ; the thighs are drawn up in bed and the legs are flexed ; the knee-jerks are sometimes present, but there is never any ankle clonus or toe reflex ; there is paralysis of the bladder and rectum, with complete relaxation of both sphincters ; pain is felt in the back and in the feet or legs ; the anses- thesia is confined to the back and outer part of the thigh, and is great- est on the outer part of the leg and foot, some one of the peculiar areas of ansesthesia shown in Figs. 195 and 196 being present. Sensation in the testicles is preserved. In these cases a recovery leaves the patient with some power of getting about on crutches, as apparatus may hold the knees and ankles firm, while the thighs are under vol- untary control. (c) Lesions are sometimes limited to the conus terminalis or lower INJURIES OF THE SPINAL CORD. H^MATOMYELIA. 427 three sacral segments. The most common lesion is a hemorrhage. The symptoms produced are a loss of control of the bladder and rectum, with complete relaxation of the sphincters, a total loss of Fig. 196. Fig. 197. Anaesthesia after a lesion of the first sacral segment. (Starr. ) Fig. 198. t III i „ i Anaesthesia from a lesion of the fourth lumbar segment. (Wichmann.) Fig. 199. Anaesthesia after lesions of the second, third, and fourth sacral segments. (Oppenheim, Huber, and Starr.) sexual power, and an area of anaesthesia either limited to the anal re- gion, mcluding the posterior part of the scrotum or posterior half of the labia and perineum, or extending into a heart-shaped area over the sacral region. (Figs. 198 and 199.) 428 INJURIES OF THE SPINAL CORD. HjEMATOMYELIA. Fig. 200. ^ / Paralysis of the left cervical sympathetic nerve from carcinoma of the spine. Ptosis, retraction of the eyeball, and flattening of the face are seen. SYPHILITIC EXUDATION ABOUT VERTEBRA. 439 secondary to cancer of the breast. This patient for a month never made any I'^ovemeut without holding her head firmly with her hands. In the third case the symptoms were those of subacute myelitis of the lumbar region, with great pain in both sciatic nerves, but there was no local tenderness of the spine, or deformity. Here the autopsy showed multiple carcinomata in various abdominal viscera and a small deposit within the spinal canal involving the cord and membranes, but not eroding the bone. In a case of osteosarcoma of the lower lumbar region there was no external evidence of the disease of the bone, and the diagnosis of a tumor of the cauda equina led to an operation being undertaken. The softened bone was found, also an erosion of the dura, and a mass of sarcomatous tissue surrounding and compressing the cauda equina. In a case of carcinoma of the stomach a secondary cancer of the mid-dorsal region developed and the deformity of the spine became evident two mouths before paraplegia occurred. In this case the pain was referred chiefly to the front and sides of the body until the paralysis became complete. It is thus evident that the local signs of the disease may not precede the nervous symptoms. Treatment. — The only treatment for this condition is palliative, and the use of hypodermic injections of morphine to give the patient relief from the pain is imperative. III. SYPHILITIC EXUDATION ABOUT THE VERTEBRA AND CORD. While gummy exostoses are not uncommon on the long bones, a syphilitic disease of the vertebrae is a rare occurrence. It does occur, however, and requires mention. I have seen it as a manifestation of inherited syphilis both in an infant and in a young man. In both Fig. 205. Gummy exudation about the cord and nerve roots, a, gummy masses ; 6, thickened capsule of tlie cord; c, nerve roots; d, pia; e, ascending degeneration in columns of Goll. (Buttersach, Arch. f. Psych., xvii., 603.) cases a diffuse thickening of the tissues about the spine, in the one case in the dorsal, in the other in the cervical region, occurred, and symp- 440 COMPRESSION OF THE SPINAL CORD. toms of slowly advancing compression of the spinal cord followed. In the latter patient these advanced to well-developed paraplegia, with pains and weakness in the hands and a mild degree of spastic para- plegia in the legs. In both cases inunctions of mercury and large doses of iodide of potassium produced a gradual subsidence of all the symptoms, though in the latter case a permanent increase of knee- jerks and a slight trace of spastic gait remains. A condition has been described of a gummy exudation encasing the spinal cord (Fig. 205), the exudation being within the dura and causing compression of the nerve roots and of the cord itself. This was not limited to one level, and therefore the symptoms produced were those of chronic myelitis. It was due to inherited syphilis. Syphilitic exudations have been observed in the tertiary stage as a result of ac- quired syphilis. The symptoms produced were localized swelling and signs of tumor of the vertebrae without much pain, and symptoms of compression of the spinal cord. The course was a slow one, and recovery followed treatment. IV. TUMORS AFFECTING THE SPINAL CORD. Tumors of the vertebra that press upon the cord are twice as com- mon as tumors of the membranes and of the cord combined. Statistics show that tumors affecting the cord are relatively rare. Schlesinger found only 147 in 35,000 autopsies and 151 in 6,540 tumors. The ratio to tumors of the brain is one to thirteen. Yet an added impor- tance to tumors of the cord has been recently given by their success- ful removal, and hence every fact which aids an early diagnosis is of interest. Table IX. — Table of Spinal Cord Tumors. Intradural. Both. Extradural. Single. Multiple. Variety. Medullary. Meningeal. Meningeal. Not meningeal. Total. Sarcoma, 14 53 9 17 11 80 27 107 Tubercle, 62 2 55 9 64 Echinococcus, 5 39 8 36 44 Fibroma, 20 "i 5 15 18 33 Gumma, "i 4 15 2 19 9 28 Glioma, 20 20 20 Psammoma, 18 18 18 Myxoma, 7 "4 11 11 Lipoma, "i 8 "i 8 "3 11 Cysticercus, 2 5 1 4 4 8 Gliosarcoma, 3 "'4 7 7 Endothelioma, 5 i "4 2 6 Melanosarcoma, i "3 1 3 4 Neuroma, 4 3 1 4 Lymphangioma, i i 1 1 2 Cysts, 1 1 1 1 2 Cholesteatoma, "i 1 1 Uncertain, 13 12 "2 "3 24 "q 30 125 142 35 75 13 273 127 400 Pathology. — The preceding table of Schlesinger shows the varieties of tumor affecting the spinal cord and their relative frequency, their TUMORS AFFECTING TEE SPINAL CORD. 441 situation, the fact that tumors are in many cases i^ultiple, the fact that they often involve both membranes and cord together, and the fact that meningeal tumors are more common than medul- lary tumors. Sarcoma about the cord has a ten- dency to extend rapidly, invading the membranes and the posterior surface of the cord, though more commonly com- pressing than destroying the cord. It is rarely encapsulated, but often sur- rounds the cord and can be stripped from it. But sometimes it is closely adherent, and in other cases infiltrates the cord, following the vessels and compressing and destroying the nervous elements. But the fact that such destruction is the exception rather than the rule increases the chance of success in its removal. Sarcoma is, however, often multiple, and not infrequently sarcoma of the cerebellum or of other organs is associated with sarcoma of the cord. Sarcoma often starts from a nerve root and invades both meninges and cord. (Fig. 207.) Tubercle is more likely to develop within the cord than in the membranes when it occurs as a solitary tumor, though a tuberculous meningitis is rela- tively more frequent than tumor and may develop as a complication of it. Tubercles are occasionally found throughout the cord, as Raymond has shown, but the usual form of tumor is a caseous mass within the cord. In some cases it appears to erode the cord, not increasing its diameter or changing its contour, and it usually begins in the gray matter, showing that the in- fection reaches the cord by the vessels rather than from the meninges. In a few cases, as in one of my own, a tuberculous mass inay lie upon the cord and compress it, and hence be easily removed. But the tendency to recurrence is more marked in tubercle than in other Fig. 206. Sarcoma surrounding the spinal cord and in- vading the nerve roots. (Schulz.) 442 COMPRESSION OF THE SPINAL CORD. forms of tumor, and the difficulty of removal, together with the danger of infection of the meninges, makes an operation much more unfavorable in this form of tumor. In my case the patient developed a general tuberculous spinal meningitis two months after the removal of the tumor, and died. Tubercles grow rapidly ; hence the course of the case is one of fast progress. Echinococcus and cysticercus cysts are much more common in Ger- many than in this country, and hence require little notice here. They Fig. 207. Gliosarcoma of the cord. The cord is invaded and destroyed on the left side. (Specimen furnished by E. W. Taylor and M. Prince.) grow rapidly, causing a progressive compression of the cord, but do not invade it as a rule. Hence the symptoms are those of compression myelitis. Fibroma springs from the meninges, usually from the dura on its inner surface. It lies upon and compresses the cord, but does not invade it. It is encapsulated and single. It grows very slowly, and is the most favorable tumor for operation because both diagnosis and surgical treatment are comparatively simple. (Fig. 208.) Gumma usually originates in the meninges, infiltrates them and also the cord, gluing both together in a soft mass and destroying the cord tissue completely. It is diffuse, not encapsulated, and grows very rapidly. It is often difficult to distinguish gumma from tubercle in the cord on inspection. It is a most unfavorable tumor for operation, but may yield to treatment. The treatment, however, is not followed by restoration of the spinal functions. TUMORS AFFECTING THE SPINAL CORD. 443 Glioma of the cord is not to be confounded with gliomatosis, although it occasionalty complicates this disease. (See Syringomyelia, Chapter XVII.) Glioma is an infiltrating tumor of the cord tissue not sharply defined or encapsulated, but caus- ing a marked enlargement of one or Fig. 208. two segments. It may be more extensive and involve the cord in a great part of its entire length. It does not involve the meninges. It is very vascular, but rarely contains cysts as do gliomata in the brain. It grows very rapidly and is im- possible of removal. It is not in- frequently associated with sarcoma, but gliosarcoma often involves the meninges as well as the cord itself. Psammona is a hard oval tumor originating in the arachnoid or dura and lying upon the cord. It is Fig. 209. Fibroma lying ou the Bpinul cord: removed by McCosh. Fibrosarcoma of the lower half of the spinal cord and Cauda equina. (Bruns. ) 444 COMPRESSION OF THE SPINAL CORD. sharply defined and chalky. It is not improbable that the small chalky plaques that are so common in the arachnoid on the posterior surface of the cord are the beginning of psammoma, but they rarely go on to a size that can compress the cord. A lai'ge tumor, four centimetres long and two centimetres thick, was found by Pal. This had given rise to symptoms for six years. These tumors grow slowly and, like fibroma, are easily diagnosed and removed. Myxoma is usually intradural, sometimes extradural, but never infil- trates the spinal cord. It is single, encapsulated, vascular, and may be cystic, of slow growth, and is easily removed. Lipoma is often multiple, is intradural, more frequently than extra- dural, though occasionally masses of fat are found about the back and between the vertebrae. It is a soft, encapsulated, fatty tumor of slow growth and easily removed. The other forms of tumor given in the table are so rare as to be curiosities of pathology. Endothelioma is a very small tumor, always meningeal and often multiple. Melanosar- coma occurs in the cord as a part of a general aifection only. Neuroma is usually a fibroma located on a nerve root. The diagnosis of the variety of tumor present in any case during life is rarely possible, though the facts already stated in regard to the rapidity of growth and the tendency to the apppearance of tumors in other organs, or of multiple tumors of the cord may afford some hint as to the probable nature of the tumor. Malignant tumors are ten times as frequent as benign tumors. The situation of intramedullary tumors is usually in the cervical or lumbar enlargement of the cord ; that of extramedullary or meningeal tumors is chiefly in the dorsal region. If the two are taken together it appears that no part of the cord is particularly liable to tumor. Hence the situation of a tumor does not throw much light upon either diagnosis or prognosis regarding operation. Tumors are much more commonly situated on the posterior and lateral surfaces of the cord than upon the anterior surface, hence they are not difficult of access to the surgeon. Etiolog'y . — Children are subject to tumors of the cord as well as of the brain. Of one hundred cases thirty occurred under the age of fifteen years, seventy above fifteen years. The oldest case recorded was sixty-six years of age. No age is peculiarly liable, but children are more liable to tuberculous tumors than to any other form. Glioma is next in frequency in childhood. Gumma is a disease of middle life. Other forms are equally frequent at different ages. The sexes are equally liable. Traumatism precedes the development of tumors in so many cases as to be justly regarded as a cause. No other cause is known. Symptoms. — The clinical aspect of intramedullary and extramed- ullary tumors is so similar that no absolute distinction can be made in any particular case. The important thing to determine is that a tumor is affecting the cord. This is first indicated by pain of an intense kind, neuralgic, sharp, shooting, burning, and agonizing, and persistent TUMORS AFFECTING THE SPINAL CORD. 445 in one locality. It is not, as a rule, increased by pressure on the nerve or attended Ky tender spots. There is no other disease which causes such definitely localized recurring and persistent pain as tumor of the spinal cord, and in any case in which pain is continued for over a month in one particular part of the body, there being no local disease to explain it and no evidence of spinal caries or carcinoma, a tumor may be suspected. The situation of the pain will depend on the position of the tumor, it being felt in the part of the body (not in the spine) related to the segment or nerve root first affected. Thus in a tumor of the lower cervical region the pain was felt for weeks in the hand and forearm; in a tumor of the upper cervical region, in the shoulder and neck ; in a tumor of the sixth dorsal segment, in the nipple and chest; in a tumor of the tenth dorsal segment, in the abdomen and groin ; in a tumor of the cauda equina, in the sciatic nerve. The pain becomes bilateral after it has persisted for a time ; but this may not occur until spinal symptoms develop. Such extension to the other side is, however, a very important diagnostic symptom. There is rarely any rigidity of the spine, and bending the back does not increase the pain. The painful area is often hypersesthetic. I have known two cases of spinal tumor which for some months were erroneously diagnosed as intercostal neuralgia, and one case was sup- posed to be angina pectoris. The direct spinal symptoms are those of compression and destruction of the segments on which or in which the tumor lies. They are atrophy and paralysis of the muscles, with reaction of degeneration, loss of reflex activity in their tendons, anaesthesia in the skin, which may be attended by analgesia and thermic ansesthesia, although these affections of sensation may occur independently and without loss of touch ; and herpes zoster and trophic changes in the domain of the affected nerve. The existence of dissociated anaesthesia is not always due to intraspinal tumors, as it has been found in cases of extradural tumor, but in such cases it is usually temporary. The indirect spinal symptoms are those of spastic paraplegia of the legs, due to pressure upon the long tracts traversing the segment affected. They are rigidity and weakness with exaggerated reflexes in the legs, an imperfect control of bladder and rectum, anaesthesia up to the level of the lesion, and a tendency to the development of bed- sores. They do not occur in tumors of the lumbar region, as tumors there cause atrophic flaccid paralysis of the lower extremities. Tumors of the cauda equina cause great pain in the sacral region as well as in all the nerves of the legs ; hence the pain is more widespread in these tumors than in tumors of the cord. The paralysis which follows is an atrophic one and is attended by reaction of degeneration. It may be limited to some one of the lumbar or sacral nerves and is rarely bilateral and symmetrical. Paralysis of the bladder and rectum are early symptoms. The facts presented in the chapters upon the diagnosis of local lesions and myelitis and injuries of the cord may be referred to, as they explain more fully these various symptoms. 446 COMPRESSION OF THE SPINAL CORD. It is to be remembered, however, that in no other form of disease of the cord do the symptoms of transverse myelitis of gradual onset coin- cide with the symptom of persistent pain. When the symptoms are fully developed it is possible in some cases to elicit tenderness on pres- sure upon the spine over the tumor, and the patient is often extremely sensitive also to heat or to electricity in the region of the tumor. Occasionally the symptoms of Brown -Sequard paralysis appear, as in a case of gumma under my care which was gradually absorbed by treatment. Course. — The course is progressive. The duration varies some- what according to the nature of the tumor. It is on an average about sixteen months from onset of symptoms to death. Death occurs from complicating bed-sores or cystitis. Diagnosis. — The diagnosis of a spinal tumor presents no difficulty, and should be made very early; in fact, as soon as spinal symptoms are associated with intense persistent pain in one locality. The first question which arises is whether the tumor is single or multiple. This can be determined by the location of the severe pain, for it will appear in several regions if there are several tumors, and by the development of mixed types of paralysis; atrophic rather than spastic paralysis being the type produced by each tumor in the muscles related to the segment involved. The second question is whether the tumor is intraspinal or extra- spinal. The best summary which can be given at present of the points of differentiation is as follows : ^ In favor of extramedullary tumors are long continued root pains, long continued pain and tenderness in the vertebrae, paralysis of root distribution rather than nerve distri- bution, Brown-Sequard syndrome, and slow development of the symp- toms. In favor of intramedullary tumor are an irregular course with sudden appearance of symptoms due to hemorrhage in the tumor, remissions, an absence of neuralgic pains at the outset, the develop- ment of dissociated anaesthesia as in syringomyelia and the absence of tenderness of the vertebrae. If the growth is very slow in its progress it is most likely to be a fibroma, a psammoma, or a lipoma, and in any of these cases extraspinal. All malignant tumors are more rapid in the development of all symptoms than are benign tumors and are attended by general cachexia and constitutional disturbance, which are absent in benign tumprs. The fact of the previous occurrence of a tumor elsewhere and of an operation for it, which had been overlooked by the patient as having no bearing upon the case, has several times been elicited by me and thrown light upon the nature of the tumor and its secondary occurrence in the spinal cord. I have seen three sarcomata in which this history was elicited. It is evident, then, that the nature and the location of the tumor, whether extraspinal or intraspinal, is always uncertain. The differentiation between a tumor and caries of the spine may be difficult in the early stage of the latter disease ; but, as a rule, stiffness of the spine, great tenderness over the spinous process on pressure^ a iJVIalaise, Deut, Arch. Klin. Med., Bd Ixxx., S. 143, 1908, TUMORS AFFECTING TEE SPINAL CORD. 447 marked increase of pain on motion or in walking, and general consti- tutional evidences of tuberculous disease, swollen glands, febrile move- ment, etc., are present in caries and not in tumor. The appearance of kyphosis decides the question. Peptonuria is present in cases of tuber- culous abscess. The differentiation from carcinoma of the vertebra is more difficult ; in fact in many cases it is impossible until the disease of the vertebra is palpable and deformity is evident, though the previous history of the patient and the rapidly advancing cachexia may point to that dis- ease. I cannot agree with the statement of Gowers that the pain in carcinoma is greater than in spinal tumor, but in carcinoma it is more commonly felt in the spine itself as well as in the nerve distribution. Herpes zoster is rarely seen except in tumor. The examination of the blood gives little information, as leucocytosis may be present in both conditions. But examination by Roentgen rays may be of much service. Treatment. — In the early stage of pain analgesics of all kinds are to be used, but morphine will eventually have to be resorted to, as the new analgesics rarely quiet the pain sufficiently. Rest in bed cannot always be enforced, as patients may find that an upright position gives relief to the pain. Thus in one of my patients, who was found to have fibroma of the dorsal region on the posterior surface of the cord, a sitting posture leaning forward over the back of another chair was the only one in which the pain was relieved. In the majority of cases a series of inunctions of mercury combined with the administration of large doses of iodide of potassium has been tried. In cases of gumma this is curative, as I have seen in three cases, but it is to be remem- bered that gumma is rare (twenty-eight in four hundred cases of Schlesinger), and unless there is a previous history of syphilis no im- provement should be looked for. In two of my cases precious time has been lost by such treatment. An operation is to be attempted as early as possible after the diag- nosis is reached. It should be undertaken with the understanding that it is exploratory, but it should be urged as affording the only hope. It is a bloody operation and one which takes much time and no small amount of skill ; but it is not a dangerous operation if done aseptically, as shock is less frequent than in cerebral operations. The higher the tumor in the cord the greater the danger of cessation of respiration during the operation. This has occurred in two of ray operations for cervical tumors, and caused much trouble, but did not prevent com- pletion of the operation. The most important preliminary to the operation is the determination of the exact level of the tumor in the spine. It may be clear to the examiner that the tumor affects a cer- tain segment of the spinal cord or rather that the symptoms indicate the upper level of the lesion. The level of the pain and of the anaes- thesia are the most reliable indications. But it is to be remembered that the symptoms will he the same from a lesion of a segment or a lesion of its nerve roots at a point somewhat lower down within the spinal canal. Thus a tumor pressing on the roots of the sixth dorsal nerve opposite the eighth dorsal segment, where they issue from the 448 COMPRESSION OF THE SPINAL CORD. cord, will give the same symptoms as a tumor in the sixth dorsal seg- ment an inch higher. It is also to be remembered that the relative position of the segments and the vertebra vary, as already stated, page 420. Hence in any operation after the diagnosis of the level of the segment affected is reached and the probable relation of that segment to the spinous processes is determined it is well to make the initial incision a long one and to expose at least three vertebrae, removing their spines before exposing the cord. In one case of my own a con- genital maldevelopment of one spinous process led to the count of the dorsal vertebrae being erroneous, and the cord was therefore exposed one inch lower than was intended. In the majority of patients here- tofore operated upon the tumor has been found somewhat higher than was expected. It is safe to say that in the dorsal region it is four inches higher than the level of the anaesthesia on the back. When the dura is exposed over a region at least two inches long, if palpation does not reveal the tumor, it is well to lay bare another inch before opening it, and it is better to go higher than lower. The dura should pulsate, but does not do so near a tumor, especially below it. I have seen the contrast between pulsation above and no pulsation below a tumor when the dura was exposed. In all cases, even in those in which an extra- dural tumor is found, it is well to open the dura. In some cases a second tumor lies within. There is usually much oedema of the arach- noid about a tumor. This subsides on incision. When the tumor is found it can sometimes be easily lifted off the cord. In other cases it must be dissected away. In some cases it must be shelled out of the cord or even cut out if it is diffuse. Division of the cord should always be in a longitudinal direction, and it is better to cut wedge-shaped pieces out of the tumor than to tear it out. Intraspinal tumors usually recur. In regard to the technique of the operation, the measures of asepsis, the control of hemorrhage by compresses, the careful division of dura and pia, and ligation of bleeding spinal vessels, the proper means of stitch- ing the dura and closing the wound, reference must be made to surgical text-books. It is an important thing to keep the patient in the prone position or on the side as long as possible after the operation to prevent undue drainage of spinal fluid. The loss of spinal fluid after an opera- tion may be considerable without any permanent ill effect. Recent records^ show that 136 tumors have been successfully re- moved from the spinal canal, 29 of which sprang from the bone. In all of these cases the tumor was found, although in several of them it lay at a level somewhat higher than that at which it had been originally located, and in all but three of these cases it was possible to remove the tumor. Many of these cases were successful, as the patients recovered not only from the operation but were very much improved some months afterward when the cases were reported. In some of these cases operation was partially successful, the patients recovering from the operation but not being materially improved by the removal of the tumor. In all of these cases degenerations had occurred in the spinal cord. These remained after the tumor was taken away. Had the iFlatau. Deut. Zejtspjj. t Nervenh., Oct. 15, 190B. ANEURISM OF THE AORTA. 449 operation been performed at an earlier date in the course of the disease there is no d'dubt that these cases, too, would have been successful. In the remainder of the cases the operation failed to relieve, and the patients died. In several of these cases the death was due to shock, the opera- tion being greatly prolonged and hemorrhage being excessive. With present methods of technique this appears to be avoidable. The chief danger in the operation lies in the hemorrhage from large veins in the muscles and about the bone. Such hemorrhages cannot be arrested by tying the vessels, and must be treated by pressure. If, therefore, in the course of the operation as tissues are divided, pads held in place by retractors are pressed into the wound, such hemorrhage may be to some extent avoided. Another cause of death has been meningitis of a septic nature, and this, too, may be avoided by care in the preservation of perfect asepsis. The terminal result of an operation will depend wholly on the degree to which the cord has been compressed or destroyed. If degeneration has set in no permanent recovery can be expected ; but if the opera- tion is done in an early stage a great degree of benefit is obtained and a slight degree of spastic paraplegia only may remain. In any case a successful operation saves life and arrests the progress of the disease, even if the patient remains a paralytic. In the majority of the suc- cessful and unsuccessful operations hitherto reported the result would have been better had the operation been done sooner. A prompt surgical interference as soon as the diagnosis is made cannot be too strongly urged. In case the tumor cannot be removed a division of the posterior nerve roots is to be made above and at the level of the tumor for the relief of the pain. Carcinoma of the spinal cord is inoperable. In sarcoma the chances are about even that the tumor can be removed. Tubercles can some- times be taken away, even from the anterior surface of the cord, as in a patient operated on for me by McBurney ; but the danger of a gen- eral tuberculous affection of the meninges subsequent to the operation, which occurred in this case, or of a recurrence, or of development of tubercles elsewhere, is to be remembered. Fibroma, psammoma, lipoma, myxoma, osteoma, and cysts are easily removed, and do not leave any damage to the cord if taken away early. The treatment of complicating cystitis and bed-sores is described in the chapter on myelitis. V. ANEURISM OF THE AORTA. An aneurism of the aorta may erode the vertebrse and produce a gradually increasing pressure upon the spinal cord. In some cases the physical signs of such an aneurism are present, and there is no difficulty in the diagnosis. In other cases, however, as in one of my patients, the aneurism lying deep in the mediastinum upon the dia- phragm cannot be detected until the pressure symptoms appear. These resemble the symptoms of spinal tumor, the compression of the nerves and of the cord producing similar pain and paraplegia. There is 29 450 COMPRESSION OF THE SPINAL CORD. sometimes, however, a difficulty of movement, especially of rotation of the spinal column, which does not occur in spinal tumors, and the symptoms may suggest carcinoma of the spine or caries of the spine rather than tumor, although no deformity appears until the body of the vertebra is eroded. The appearance of kyphosis iu such a case is not accompanied by any thickening of the tissues about the spinous process of the vertebra or about its laminae, and there is less tenderness of the spine than in these diseases. In any case, however, in which symp- toms of compression myelitis appear the possibility of an aneurism is to be considered and its physical signs looked for. VI. PACHYMENINGITIS CERVICALIS HYPERTROPHICA. Pathology. — This disease, first described by Charcot, presents all the symptoms of a compression myelitis of the cervical region of the spinal cord of rather rapid progress. The lesion consists of a fibrous thickening of the dura mater with consequent compression of the cord and especially of the nerve roots in their passage through the dura. As a result of this thickening of the meninges there is usually a secondary annular myelitis in the periphery of the cord at the point of implication ; hence the affection is really a meningomyelitis of the cervical enlargement. In chronic fibrous pachymeningitis hemorrhages within the dura or in the newly-formed layers of tissue upon its inner surface, such as occur in pachymeningitis of the brain, are not un- common. The thickened dura not uncommonly becomes adherent to the pia, layers of connective tissue uniting both, and attendant upon these changes there is usually thickening of the bloodvessels. The pressure of the newly-formed tissue is exerted chiefly upon the nerve roots as they pass through the dura, and these are found embedded in the fibrous mass. If the process does not cease with the mere thickening of the dura, connective tissue is produced along the lines of the bloodvessels into the cord. The result of the compression of the spinal cord and of its vessels is a degeneration of its tissue. There is also frequently a for- mation of gliomatous masses and the occurrence of secondary de- generations. In the majority of the cases the disease is of syphilitic origin. Changes similar to those described in compression myelitis follow. Symptoms. — The symptoms of this disease are identical with the symptoms of chronic myelitis of the cervical region of the cord. They differ somewhat in their distribution, according as the upper half or lower half of the cervical enlargement is first involved, and they have been divided by Charcot into several stages : first the stage of pain ; second, the stage of paralysis with contractures; and third, the stage of spastic paraplegia secondary to the compression. The pain in the affection is felt in the back of the neck, possibly iu the back of the head, and is attended by great stiffness of the neck with a tendency to bending forward of the head and difficulty on any PACHYMENINGITIS CEBVICALIS HYPEBTBOPHICA. 451 lateral motiou. Pain is also felt in the hands or in the arms. Such pains are of an intense neuralgic character, and are attended by anaes- thesia and parsesthesia and sometimes by muscular spasms or tremor. . After a period of two to five months symptoms of paralysis appear in the muscles, with atrophy and reaction of degeneration. These may be limited to the ulnar and median distribution if the lower half of the cervical enlargement is invaded, or to the musculo-spiral distribu- tion if the upper half is involved. Charcot describes certain defor- mities of the hand and wrist due to paralysis (main en griffe), but these may be present in any affection producing paralysis of either the ex- tensor or flexor muscles upon the arras and forearms, with consequent contracture of their opponents. Thus drop-wrist or claw-hand may be symptoms appearing in this disease. If the lower part of the cer- FiG. 210. Ossification of the vertebrae in spondylose rhizomelique. Icon, de la Salpetri&re, June, 1911. An ossified mass welding tlie vertebrse together. vical enlargement is affected there may be paralysis of the sympathetic nerve. If the pressure affects the spinal cord to a degree sufficient to cause a degeneration of the tracts passing through it, a spastic para- plegia gradually develops, constituting the third stage of the affection. Such a condition may be of long duration and slow progress, patients having been observed in whom it lasted twenty years. I have seen several patients in whom it seemed probable from the symptoms and course that a hypertrophic pachymeningitis of the lum- bar region was present. In one such case a thick connective tissue mass surrounded the cord over the entire lower third from the sixth dorsal segment to the con us. It was adherent to the cord but was separated at the operation without injury to the cord. The symptoms resemble in character those described as occurring in pachymeningitis 452 COMPSESSION OF THE SPINAL COBD. Fig. 211. of the cervical region, but are manifested in tiie lower extremities, pain, rigidity, and paralysis with talipes developing slowly and being of long duration, and attended by extreme atrophy of the legs. Prognosis. — The prognosis is fairly good if the disease is diagnos- ticated in its early stage. Treatment. — The frequent appli- cation of the actual cautery to the back of the neck, with the use of in- unctions of mercury and large doses of iodide of potash, is not infre- quently followed by a cure. This will always awaken the suspicion that the lesion is syphilitic in origin, though such suspicion may not be confirmed by pathological observa- tion. Applications of tincture of iodine to the neck and back some- times give relief. Antineuralgic remedies will be required, and for the treatment of the paralysis such meas- ures as are described for the treat- ment of lesions of the brachial plexus and of spastic paraplegia may be em- ployed. The impossibility of stripping off the thickened membrane from the cord without producing laceration of the cord and excessive hemorrhage renders surgical treatment impossible. VII. SPONDYLOSE RHIZOMELIQUE. SPINAL ANKYLOSIS. This is a condition of bony growth in the vertebrae which produces a thickening of the bodies of the verte- brae and their final union with each other ; an ossification of the ligaments as well as a thickening of the laminse, causing an encroachment upon the caliber of the foramina, and conse- quent pressure upon the nerves which pass through them. The final state is shown in Fig. 210 which shows the vertebrae firmly welded into a bony mass. It is rare for pressure to be made upon the spinal cord, but this has been observed. The result of such ankylosis is a rigid condition of the back^ which M ^'^^^^l 1'/ fl m 1 9 IH ^^^^^^^^^L^' nHH m_ i^ ^^^^^^^^ Position in Spondylose rhizomelique. Absolute rigidity of the spine and secondary atrophy of the muscles. Icon, de la SalpS- triere, April, 1911. SPONDYLOSE BRIZOMELIQVE. 453 is usually bent somewhat forward as is shown in Fig. 211. Another result is a gradually advancing paralysis and atrophy of all the muscles due to compression of the nerves ; hence the thinness of the limbs shown in the figure. The compression is equally severe upon the sen- sory nerves, hence parsesthesia, severe pain, and finally ansesthesia are present in the limbs. These patients are usually treated for years for chronic rheumatism, but it finally becomes evident that spinal ankylosis is present. Males are more commonly affected than females, and it is a disease of middle age.^ The onset is slow, as a rule. The symptoms are : (1) Pain in the back, in the sacro-lumbar region, or about the shoulders, radiating into the limbs and increased by movement ; (2) a progressive stiffness of the spine, and then of the shoulders and hips, so that the body is moved as a single mass ; (3) a deformity resulting in a bend- ing forward of the entire spine in a regular curve, as shown in the figure, producing a groove across the abdomen just above the umbilicus ; (4) absence of tenderness on pressure on the spine ; (5) gradual dim- inution and final cessation of pain in the back and limbs as the anky- losis becomes complete ; (6) absence of bony deposits on the long bones or in the small joints, which serves to distinguish it from rheumatic arthritis ; (7) progressive atrophy of the muscles of the back and limbs, with rigidity of the muscles about the shoulders and hips, with occasional fascicular twitching, but no reaction of degeneration ; this atrophy is supposed to be due to disuse rather than to degeneration in the nerves. The disease is slowly progressive and incurable. ' For full discussion see N. Eldaroff. Icon, de la SalpetriSre, 1911, April and June. CHAPTER XXIV. LANDRY'S PARALYSIS. ACUTE PROGRESSIVE PARALYSIS. This disease, first described by Landry in 1859, is an acute ascend- ing paralysis attended in many cases by febrile symptoms and consti- tutional disturbances that, in the light of our present knowledge, indi- cate an acute infection. It begins in the form of paralysis of the lower limbs and ascends rapidly to the body, to the arms, and to the respira- tory muscles, causing death within a few days, either from respiratory paralysis, from disturbance of the power of swallowing, or from heart failure. It is very rare for .the disease to come to a sudden standstill and for recovery to ensue. Ever since the original description of Landry observers all over the world have occasionally seen cases corresponding pretty closely to the description given by Landry, excepting that in some cases sensory symptoms of the nature of a rapidly advancing anaesthesia have been present associated with the paralysis. More careful observation of temperature than was customary in Landry's time has demonstrated the fact that in the large majority of cases a febrile movement accom- panies the disease. Such febrile movement was present in a case under my own observation. In this case the general appearance of the patient, the enlargement of liver and spleen, a condition of jaundice, vomiting, diarrhcea, and acute nephritis all suggested strongly a con- dition of infection. The paralysis became complete in the course of a week, when the patient died. Westphal described a case quite similar in its rapid course to that of Landry's original case, but in it the disease was a descending rather than an ascending one in its progress. Hence the ascending character of the affection as described by Landry must not be considered essential to the diagnosis. In a few cases the escape of the bladder and rectum has not been complete, and incontinence of urine and feces has occurred. In a few cases, where the disease has lasted beyond a week, reaction of degeneration has developed in the muscles ; hence the various diag- nostic signs of the disease as laid down by Landry have gradually been eliminated, and we have to speak of Landry's paralysis as in- cluded under acute progressive total paralysis. In the majority of the cases recently recorded the distinctly infectious appearance of the patient, as already mentioned in toy case, has been prominently noticed, and it seems at present to be the general consensus of opinion that in Landry's paralysis we have an acute infectious dis- ease which produces rapidly advancing paralysis. It may be that these cases have been cases of poliomyelitis of a very severe type. Pathology. — The lesions found in various cases of Landry's par- 454 SYMPTOMS. 455 alysis have differed widely. The early observers found no lesions, but their methoifs of observation were very imperfect, and hence their con- clusions cannot be accepted, esj)ecially as later investigators have almost uniformly found some changes in the nervous system. Many pathol- ogists have found changes in the spinal cord consisting practically of a widely disseminated acute myelitis, with vascular and perivascular inflammatory conditions, with acute degenerative processes, and with all the characteristic appearances seen in cases of very acute myelitis.^ Other equally reliable observers have considered Landry's paralysis a multiple neuritis and have described numerous changes in all of the peripheral nerves and in the nerve roots.^ In these cases no changes in the spinal cord have been found. Unfortunately, as yet bacterial examination of this disease has failed to reveal the existence of any special germ, though in recent cases various micro-organisms have been discovered : typhoid bacilli, pneumococcus, staphylococcus, diplococcus, etc.^ The probable theory, however, is that it is a disease of acute infectious origin, the germ or toxin of which affects the entire nervous system both in its periphery and in its central organs. In some cases the lesion is found in the nerves ; in others in the neurone bodies in the spinal cord ; in others, still, in the pons and cerebral axis, the specific action being greater on the motor than on the sensory portion of the nervous system. The cortical neurones escape. This hypothesis would give an explanation for the rare cases of acute bulbar palsy or acute ophthalmoplegia followed by descending paralysis, the majority of which go on to a fatal termination in a very short space of time. The causation of the disease is more likely to be a specific infection, inasmuch as autopsies in later cases have demonstrated changes in the viscera, the liver, the spleen, the kidneys, and the lymph glands such as are commonly found in other acute infectious diseases.* Its causa- tion also finds a certain amount of support in the fact that a number of cases of Landry's paralysis have developed in the course of or sub- sequently to other acute infectious diseases. Symptoms. — The disease begins suddenly and affects, first, the muscles of the lower extremities, producing a rapidly increasing flaccid paralysis with total disability to move the muscles voluntarily. There is no tremor, no contracture, no spasm or cramp in the muscles, and percussion of the muscles does not produce any muscular contraction. There is a loss of tendon reflexes. The electrical reactions are not changed, but this is probably because the duration of the paralysis is not sufficiently long to warrant the appearance of a reaction of degen- eration. There may be numbness in the legs, but there is rarely pain. The paralysis advances rapidly from the legs to the thighs, then to the muscles of the trunk, so that the patient can no longer retract the abdomen or sit up in bed ; it then advances to the muscles of the back and of the thorax and of the arms, until finally all the muscular system ^ Bailey and Ewing, New York Medical Journal, July 4, 1896. "Barth. 1890. ^ See Brain. 1908. Spring number. *T. Diller, Journal of Nervous and Mental Disease, October, 1902. 456 LANDRY'S PARALYSIS. ACUTE PROGRESSIVE PARALYSIS. up to the neck is paralyzed. It then advances to the muscles of deglu- tition and of speech and to the face, so that an absolutely total par- alysis of the entire muscular system of the body, including that of the eyes, may occur before death. When the arms are affected the par- alysis begins in the fingers and creeps up the arm as it did up the leg, and here, too, there is no tendency to contracture or deformity. The limbs are wholly given over to the action of gravitation, muscular tone being abolished. The bladder and rectum usually remain in a normal condition, though in a few cases they have been paralyzed. Sensory disturbances in some cases do not appear at all. In other cases the general sense of muscular fatigue and very intense feelings of numb- ness and formication may be felt, but examination usually fails to reveal any marked anaesthesia. The patient's consciousness remains clear to the very end in the majority of cases. According to Landry's account of the disease, it may run its course in some cases within three days, in others it may be longer, up to two weeks, and a large majority of patients die. In a few cases the disease comes to a standstill ; remissions occur, which may be followed by an exacerbation and death, or by a slow recovery. The prognosis is very grave, and the treatment can only be of a' general character, such as would be used in any acute infection or in acute myelitis. Ergot has been recommended in large doses, but this seems to be an empirical suggestion with no rational basis. Antiseptic treatment by hypodermic injections of carbolic acid or of formalin injections into the veins might be tried. Urotropin 10 grains every three hours by the mouth is to be used. CHAPTER XXY. SPINAL MENINGITIS. Etiology. — Acute meningitis limited to the spinal cord develops after various forms of disease of the spinal column or of the dura mater, especially after tuberculous and malignant disease. It may occur as the result of any form of injury of the spinal column or of the spinal cord, especially if that injury involves a laceration of the surface and opens the way for the ingress of germs. Acute spinal meningitis may also develop as a complication of acute myelitis and myelomalacia from any cause. It may also arise as a complication of bed-sores, the septic agent having direct access to the meninges, either through the vessels or along the nerve trunks. It may develop also by extension of a meningitis of the base of the brain, especially after otitic meningitis. The most common cause of acute meningitis is the presence of some infectious agent in the blood as the result of pneumonia, typhoid fever, rheumatism, furunculosis, tuberculous disease anywhere, or any of the infectious diseases, or septicaemia. Exposure to cold is mentioned as a possible cause, though this is questionable. Epidemic cerebro-spinal meningitis is described in Chapter XLI. Pathology. — On opening the spinal canal an accumulation of fluid is found between the dura and pia, infiltrating the meshes of the arachnoid, and this fluid may be purulent. Here and there on the surface of the cord are collections of pus or of thick fibrin and plastic material and gelatinous masses. Fibrinous exudations upon the sur- face of the cord in the pia are evident at places. Thus the usual proofs of an inflammation of a serous membrane, serum, fibrin, and pus are found irregularly scattered, or filling up all the meshes of the arachnoid and covering the pia. The bloodvessels are distended with blood, and small hemorrhages may be found. There is a close adhesion of the pia to the cord, and frequently also to the dura. Meningitis may extend to the nerve roots at their points of exit through the mem- branes. On microscopic examination an extensive congestion of the finer bloodvessels of the meninges and of the arteries entering the periphery of the cord is evident, and there is an exudation of cells about the bloodvessels both in the pia and in the periphery of the cord. Capillary hemorrhages are frequently observed in the pia and within the cord, together with masses of fibrin and collections of pus. CEde- mat<^)us swelling of the various layers of the pia and the production of connective tissue, with a marked thickening of the pia layers, are generally present. Changes are uniformly observed on the nerve roots 457 458 SPnKlL MENTNGirm. and also in the spinal cord. The nerve roots show swelling of the axis cylinder and destruction of the myelin sheath, with all forms of degeneration in the nerves. There is also a congestion of the vessels of the perineurium and endoneurium and an exudation of leucocytes and small cells. The spinal cord is usually affected to the depth of one-half centimetre from the surface on all sides. Fine, small-cell infiltration is evident along the bloodvessels, and many leucocytes are found within the cord. The vessels are distended and occasionally ruptured. Swelling of the axis cylinders of the white columns, seg- mentation of the myelin, and general degenerative processes in the nerve fibres of the cord are present, and these appear to be more intense near to the bloodvessels, and sometimes can be found at a con- FiG. 212. f >■ Acute meningomyelitis. Section through the anterior border of the spinal cord and meninges, showing infiltration of the tissues by inflammatory products. (Marinesco, International Medical Con- gress, Paris, 1900. ) siderable depth from the surface of the cord, especially along the larger arteries. Thus wedge-shaped regions of degeneration, apex inward, may be discovered in the cord. These changes are usually more in- tense in the posterior and lateral columns than in the anterior columns. There is usually a tendency to an increase in the glia and to a connec- tive-tissue production, and if the condition goes on to a chronic stage small plaques of connective tissue and sclerosis may be produced. Similar changes are observed in the medulla oblongata if the disease extends upward to the brain. If the disease does not prove fatal there may be left, as a result of an acute meningitis, a chronic permanent thickening of the pia mater with adhesions to the dura mater and with sclerosis of the spinal cord and chronic degenerative processes in the nerve roots. In the case of the anterior nerve roots these may extend outward to the peripheral nerves. SYMPfOMS. 459 Spiller^ has described a form of meningitis which he terms circum- scribed serous meningitis, the lesion consisting of a collection of clear fluid in the pia-arachnoid, forming a cyst which presses upon the cord, causing symptoms like those of a tumor. The origin of these cysts is unknown. Symptoms. — Acute meningitis usually develops suddenly with ,fever, chill, and marked constitutional disturbances, with all the phe- nomena of a febrile movement — malaise, nausea, and diarrhoea — and in addition, with local symptoms referable to the spinal cord and especially to irritation of its surface and nerve roots. These symp- toms consist of severe pain in the back and radiating pains about the body and down the limbs, which are greatly increased by movement or by pressure or by any irritation of the surface. They are soon fol- lowed by a condition of stiffness of the back and of the neck, so that the entire body is held rigid, and all attempts at movement are resisted because of the increased pain thereby produced. An actual rigidity of the muscles of the back of the neck can be felt, and this may increase to the point of opisthotonos, especially in children. The pains of meningitis are of a neuralgic character and are very constant and fre- quent. They shoot from the back around to the front of the body, and they shoot up and down the arms and legs. They are attended by hypersesthesia and hyperalgesia of the skin, so that all touch or handling of the surface is unendurable, and the patients very often are unable to bear the weight of the bed-clothing, which has to be sup- ported by a basket arrangement in the bed. This hypersensitiveness is often attended by spasms of the muscles and twitchings of the limbs, and these cramps are often very painful. Kernig has noticed that if these patients are made to sit up in bed the legs are always drawn up, with flexion of the knee. The leg cannot be extended on account of the contracture of the flexor muscles. In the course of a few days, as the result of the inflammation and degenerative changes in the nerve roots, anaesthesia may develop in the parts that have been painful, and in some cases paralysis with atrophy develops in the muscles. This is usually noticed first in the legs, and is subsequently attended by an increase of the knee-jerks and the development of ankle clonus. Later on, if the paralysis becomes extreme, the reflexes may be lost. Within a few days of the onset disturbance in the action of the bladder develops, there being, frequently, retention of urine, requiring the use of a catheter, and obstinate constipation ; later on in- continence may ensue. A tendency to the development of bed-sores is observed in all cases, and the skin must be cared for by constant change of the position and by proper support upon an air-bed or water-bed. Vasomotor disturbances are common in the shape of flushings or sudden pallors of the skin, and at any point upon the body a red line will appear after the skin is irritated by a sharp object, such as a pencil or nail (tdches oer^brales ou spinaled). The course of the case is often a rapid one, and within a week im- lAmer. Jour. Med. Sci., Jan., 1909. 460 SPINAL MENINGITIS. plication of the muscles of respiration or an affection of the pneu- mogastric nerve produces death from respiratory or heart failure. It may be preceded by very rapid, irregular respirations, by Cheyne- Stokes respiration, or by intermittent, irregular pulse. In the majority of the cases the meningitis extends to the cranial cavity, and cerebral symptoms, convulsions, and cranial nerve palsies develop just before death. In other cases the progress of the case is less rapid, and two or three weeks pass before the symptoms become severe enough to give rise to alarm, and the patient may, even after the most severe symp- toms, gradually recover, though frequently a state of chronic meningitis is left in which paralyses, muscular atrophies, irregular plaques of anses- thesia, and disturbance in the action of the bladder and rectum remain for many months. In the circumscribed serous meningitis of Spiller the symptoms were those of a spinal tumor, pain and paraplegia slowly developing — and these cases were successfully treated by operation for the sup- posed tumor ; the cyst being emptied. Diagnosis. — The diagnosis of acute meningitis is much aided by Quincke's lumbar puncture (see page 155). In cases of meningitis the fluid contains leucocytes, and bacteria of various kinds have been found in the serum — tubercular bacilli in tuberculous patients ; the streptococcus, pneumococcus or diplococcus in other cases. The mere withdrawal of the fluid where the intra- spinal pressure is great is followed by great relief of the symptoms, notably the pains and spasms of the back, and Quincke's puncture may be done not merely as a diagnostic but also as a therapeutic measure. Prognosis. — The prognosis in acute meningitis will depend some- what upon the cause, and is more unfavorable in tuberculous cases than in purely septic cases. The meningitis following pneumonia and typhoid fever usually recovers. Treatment. — The treatment of meningitis consists in absolute rest in bed. An air-bed or water-bed should be employed early on account of the tendency to bed-sores. It is preferable for patients to lie upon the sides rather than upon the back. Cold-water applications, or ice- bags, should be applied to the spine. Blisters or the actual cautery may be applied along the back. Cupping also may be used, either wet or dry, according to the general condition of the individual. The general constitutional disturbances must be treated by laxatives and by antipyretics. The pains require sedatives, either bromides in large doses or bromide and chloral together, recourse being had to opium if the analgesics, phenacetin, etc., fail. CHRONIC MENINGITIS. Pathology. — A few cases of chronic meningitis begin in an acute process, but the majority develop as a chronic syphilitic inflammation from the start. In chronic meningitis there is no production of pus CHRONIC MENINGITIS. 461 and very little fibrin, but simply a connective-tissue formation with or without an increase of serum. In all cases the result is a congestion and distention of the bloodvessels, a leucocytosis and round-cell infil- tration of the membranes of the cord and of the nerve trunks, and a thickening of all the tissues involved. A chronic degenerative process is usually set up in the spinal cord, especially in the periphery of the cord, giving rise to a chronic meningomyelitis. In fact, the majority of cases of chronic meningitis are associated with chronic myelitis of low grade and slow course. In chronic meningitis of syphilitic origin gummy exudations or gelatinous masses are found all through the meshes of the pia mater and arachnoid, and very often surround the nerve roots. Thus in the case reported by Buttersack (Fig. 205) the gelatinous mass surrounded both the cord and the nerve roots and was attended by a round-cell infiltration of the cord itself. In syphilitic meningitis the blood- vessels are thickened, both veins, arteries, and capillaries, and the lymphatics show deposits in their walls and an infiltration with small cells. The changes in the bloodvessels accompanying a syphilitic meningitis are those found in all forms of obliterating endarteritis, and very frequently are attended by obliteration of the lumen of the vessels. In -the syphilitic forms of chronic meningitis wedge-shaped plaques of connective tissue are found in the spinal cord, irregular regions of sclerosis, and a round-cell infiltration all through the white matter of the cord, which occasionally penetrates as deeply as the gray matter. In the syphilitic forms of meningitis these lesions, both of the meninges and the cord, may be continuous over a considerable distance, or they may be disseminated irregularly through either enlargement or through the entire cord. Symptoms. — The course of a case of chronic meningitis differs from that of acute meningitis only in the absence of febrile onset and in the slower development of the various symptoms. The same stiffness in the back and limbs, tendency to cramps, shooting pains, hyper- sesthesia, paralyses, anaesthesia, and disturbance in the action of the bladder and rectum, develop slowly, together, or in succession. Diagnosis. — The diagnosis of the condition is usually very easy from the symptoms that are present. Ordinary pains in the back, even when attended by rigidity and by some neuralgic pain and oc- casional cramps, are not sufficient to establish a diagnosis, as they may be due to neurasthenia, may appear in traumatic neurosis, or may follow slight falls and injuries of the back. Under these circum- stances, however, the intensity of the symptoms is much less than in meningitis, and in case of doubt Quincke's puncture will fail to reveal the existence of an increase of cerebro-spinal fluid. Muscular rheuma- tism may produce a severe pain in the back with more or less tender- ness in the muscles and in the spine, but never goes on to develop the other symptoms of meningitis already mentioned. Myelitis, though frequently attended by a complicating meningitis, is not, as a rule, attended by pain of as intense a character;, and there are few neuralgic 462 SPINAL MEXIXaiTIS. pains and no tendency to a si^asm or cramps in the muscles or to rigidity of the back. Treatment. — In the chronic cases a thorough course of inunctions of mercury, followed by iodide of potash in full doses, from twenty to one hundred grains, three times a day, will be of service not only in the syphilitic but also in other cases. Cautery applied to the spine will give relief to many of the pains and to the stiffness. Massage and long-continued warm baths, or spinal douches, or sponging of the spine alternately with hot and cold water, are also of service. The general strength of the patient should be supported, and all forms of good food and tonics should be used freely. SECTION III. DISEASES OF THE BEAIN. CHAPTER XXVL THE CEREBRAL CIRCULATION. The majority of cases of brain disease which are met with in prac- tice are due primarily to disease of the bloodvessels. Endarteritis produces a state of malnutrition in the brain which may show itself by mental failure or by premature senility or even by signs of dementia. It also causes thrombosis and embolism, and, by the final weakening of the vessel wall, rupture and hemorrhage. In the former case necrosis of the brain tissue is produced, with arrest of its functions. In the latter case laceration and destruction of the brain tissue is caused with both irritation and arrest of function. Hence a knowledge of the dis- tribution of the cerebral bloodvessels is a necessary preliminary to a study of the various types of apoplexy. The blood reaches the brain through the vertebral and internal carotid arteries. The vertebral arteries unite to form the basilar, which in its course gives off the arteries of the cerebellum and pons. The basilar divides into the posterior cerebral, which supply the thal- amus, the tegmentum of the crura, the corpora quadrigemina and crura. Fig. 213. Int. Car. Mid. C. Showing distribution of bloodvessels to internal capsule. The artery marked x is the so-called artery of cerebral hemorrhage, and it is readily seen that its rupture destroys the fibres in the internal capsule. (Duret.) and tlie temporo-occipital basilar part of the hemispheres. The pos- terior cerebral send the posterior communicating arteries forward to 463 464 THE CEREBRAL CIRCULATION. join the internal carotid. These too send small vessels into the basal ganglia. The internal carotid divides into the middle and the anterior cerebral, the latter being united by the anterior communicating, which thus completes the circle of Willis. From the arteries composing this circle many little branches perforate the base of the brain supplying its basal ganglia. The middle cerebral artery, passing outward through the fissure of Sylvius, gives off in its course many basal arteries which enter the anterior perforated space. These ascend through and outside the len- ticular nucleus and supply the internal capsule and the anterior part of the optic thalamus. (Fig. 213.) The middle cerebral artery divides as it passes outward into several branches which radiate over the island of Reil, and spread outward over the convexity of the brain supplying the greater part of the lateral surface. (Plate XXII.) The larger branches go to the third frontal convolution, to the anterior and pos- terior central convolutions, and along the fissure of Sylvius. The Sylvian artery gives off numerous branches to the operculum and supramarginal convolution above and to the temporal convolutions below, and finally terminates by spreading out over the parietal lobules and the convolutions joining the temporal and occipital regions. The anterior cerebral artery passes forward, supplying the base of the frontal lobe, then curves upward on its median surface, sending numerous branches over the edge. It supplies the median surface of all the frontal convolutions and a small part of the cortex of the lateral surface near to the median line, excepting the third, which receives its supply from a branch of the middle cerebral. Its terminal branches pass backward as far as the cuneus, anastomosing on the way with the terminals of the middle cerebral artery, and with those of the posterior cerebral artery. The anastomoses of the cortical terminal vessels are most perfect, as shown in Plate XXIII. But many of the small basilar vessels have few or no anastomosis with adjacent branches, and hence have been called terminal arteries. The effect of an embolism in such arteries is to cause an area of necrosis which is permanent. The basal arteries do not anastomose freely with the cortical arteries, and hence the white matter lying abpve the basal ganglia and beneath the cortex is relatively imperfectly supplied with blood. This protects it from the occurrence of hemorrhage, but predisposes it to softening after thrombosis or em- bolism. The cerebellum is supplied by bloodvessels which arise from the vertebral, basilar, and posterior cerebral arteries. The blood supply of the crura cerebri and pons and medulla is derived from branches of the basilar and posterior communicating arteries. It is evident that the effect of an embolism or thrombosis in any of these vessels may be a very small or an extensive necrosis of tissue, according to the size of the vessel plugged. The extent of a hemor- rhage in the brain will also depend on the size of the vessel ruptured. There are certain vessels in which embolism and hemorrhage are more common than iu others. The small vessels, which enter the base PLATE XXII Fia OTa The Vascular Supply of the Cerebral Cortex. (Dejerine.) The regions supplied by different arterial branches are shown in different colors The median surface. — Branches of the anterior cerebral artery. Fia. Anterior frontal. Fim. Middle frontal. Ftp. Posterior frontal. Branches of the posterior cerebral artery. OTa. Temporo- occipital anterior. OTm. Temporo-occipital median. K. Calcarine. The lateral surface. — Branches of the middle. cerebral artery. Fi. Inferior frontal. Fa. Ascend- inK frontal. Pa. Ai^cending parietal. Pi. Inferior parietiil. Pc. Anijular. Ta. Anterior temporal. Tm. Mirldle temi)oral. Tp. Posterior tciriporal. THE CEREBRAL CIRCULATION. 465 through the anterior perforated space come off from the upper surface of the internal carotid artery or the middle cerebral. The current of blood rushes directly into these, as it is forced up from the heart Fig. 214. Superficial veins of the external surface of the left hemisphere. 1. Great anastomotic vein of Froland. 2. Lateral sinus. 3. Longitudinal sinus. (Testut.) Fig. 215. An injfotpd and oorrodnd preparation of the cerebral veins and sinuses. (Huntington.) more forcibly than into other vessels, to reach which it has to pursue a more tortuous course. Hence, these are the vessels more commonly 30 466 THE CEREBRAL CIRCULATION. ruptured under an increase of pressure or plugged by a clot or em- bolus from the heart. The distribution of these vessels is to the basal ganglia and internal capsule. Hence the frequency of capsular lesions. The terminal character of these vessels makes repair im- possible. The free anastomosis of the cortical vessels has already been noticed. A thrombosis or embolus in one of them rarely has a perma- nent effect, as the necrosis at first begun is arrested by the development of a collateral supply of blood. Hence cortical lesions have a more favorable prognosis than capsular lesions. The venous circulation in the brain is shown in Figs. 214 and 215. The veins from the interior of the brain empty into the veins of Galen and then into the straight sinus. Those from the cortex reach the longitudinal, lateral, and cavernous sinuses. Intracranial pressure. — Intracranial pressure produces changes in the pulse and arterial tension which can be accurately measured by the Riva Rocci blood pressure apparatus.^ When the contents of the skull is slowly increased by a small tumor or by a small clot the dis- placement of cerebro-spinal fluid and the narrowing of the veins pre- vent tangible effects. But when a large clot suddenly forms, the pressure causes venous stasis and a slowing of capillary circulation. Nature attempts to correct this by raising the arterial tension, and thus maintains the nutrition of the medullary centres necessary to life. If the pressure however increases, beyond the power of the heart to over- come, even while the arterial system is contracted, then, a weak rapid heart action, a relaxed arterial tension, and an irregular respiration are followed by collapse and death.^ The condition of the arterial tension is therefore a good index of the degree of intracranial pressure. A tension of 220 mm. developed suddenly is dangerous and a tension of 280 is fatal. Trephining is the only means of relieving intracranial pressure and may be resorted to successfully in cases of intracranial hemorrhage when the arterial tension rises to the danger point.^ The subject of decompressive operations for the relief of intiacranial pressure has been widely discussed in the past few years and will be fully described in the chapter on brain tumors. 1 This can be bought of Eimer and Amend, New York, in portable form, for |8. 2 Kocher, Hirndruck, Nothnagel's Spec. Path. u. Ther., IX., 3, 2. ^Harvey Gushing, Amer. Jour, Med. Sci., June, 1903, and September, 1902. PLATE XXIII Fig. 1. The Arterial Supply of the Cortex Cerebri. (Szymonowicz.) Section tliroughi tlie cerebral cortex of a rabbit; bloodvessels injected red. X 40. Fig. 2. The Arterial Supply of the Cortex Cerebelli. (Szymonowicz.) Section through the cerebellar cortex of a guinea-pig: bloodvessels injected red. X 44. CHAPTER XXVII. THE CEREBRAL DISEASES OF VASCULAR ORIGIN. CEREBRAL HYPEREMIA AND ANEMIA. The investigations of Mosso have proven that any mental effort oi emotion is attended by a functional hyperemia of the brain and that sleep is attended by a functional anaemia. Formerly a number of abnormal conditions were ascribed to hyjper- cemia of the brain. It is doubtful, however, if they were dependent upon this cause, and it is improbable that congestion of the brain alone is a condition that can be more than temporary or that can produce serious permanent symptoms. It is admitted that in many constitu- tional states both active and passive cerebral congestion may occur. Thus fever, persistent mental strain, great emotional excitement, sun- stroke, and an excess of alcohol, quinine, and strychnine produce con- gestion of the brain. Obstruction to the flow of blood from the cerebral sinuses may lead to venous congestion. In this condition the symp- toms of the original underlying disease, such as tumor or abscess of the brain, thrombosis of a sinus, or tumors and aneurisms in the neck, chronic cardiac disease, emphysema, etc., will occur, in addition to symptoms due to the hypersemia of the brain. There is doubtless a stage of congestion of the brain in all cases of meningitis and of enceph- alitis, but here again it is not possible to distinguish between the symp- toms due to the two conditions. The symptoms produced by congestion of the brain are headache of a severe character, with feelings of pulsa- tion, vertigo increased by a sudden effort or change of position, tinnitus aurium, sensations of pressure about the eyes and head, insomnia, mental irritability, with sense of incapacity to carry on a train of thought, and restlessness. The convulsions and coma in sunstroke are due to tox- aemia and not to the congestion. It is probable that the majority of the symptoms mentioned may, in some cases, be due to imperfect metab- olism, or to chemical changes in the nerve cells, rather than to mere mechanical pressure. I am always reluctant to ascribe morbid states to congestion of the brain, and I regard such a diagnosis as unsatisfac- tory and tentative. I do not believe in the existence of a chronic con- gestion. The treatment of the symptoms during their temporary duration is by ice to the head and the free use of ergot and bromide of sodium. Such treatment will relieve the headache and sensations of pressure in the course of a few hours, but should not be kept up more than two or three days. It is well to give calomel, followed by saline laxatives, to restrict the diet, to cut off all alcoholic stimulants, and stop the use of tea, coff(!e, and tobacco ', in other words, to elimi- 467 468 CEREBRAL DISEASES OF VASCULAR ORICm. nate from the body all those poisons which may be the cause both of the congestion and of the symptoms. If the cause of venous conges- tion can be ascertained it is to be removed, if possible. If impossible, wet cups behind the ears, hot foot baths, counter-irritation to the neck, and sedatives may relieve the symptoms. Ancemia of the brain may be due to loss of blood or to a general anaemic condition. The latter is not uncommon both in young girls and in women at the menopause as a primary disease. It may also be secondary to other constitutional or organic diseases that produce mal- nutrition. The symptoms of anaemia of the brain due to a loss of blood are faintness, vertigo, nausea, confusion of ideas, and finally a loss of consciousness, with suspension of respiration and great feebleness of the heart. They are usually relieved soon by a recumbent posture, by inhalation of ammonia, and by stimulation with alcohol. In extreme cases transfusion with salt solution may be required. The symptoms of anaemia of the brain due to general anaemia are headache, felt chiefly on the vertex, somnolence, especially after meals, depression of spirits, inability to concentrate the attention, or to con- duct any active mental process for any length of time, and insomnia. The insomnia of anaemia is characteristic. The patient gets to sleep easily on lying down; but sleeps only a short time, and then wakes and is unable to get to sleep again. ARTERIAL SCLEROSIS. Pathology. — Arterial sclerosis is the result of endarteritis. This is a chronic inflammatory process in the intima and muscular coats of the vessels, with a production of new cellular tissue, which may(l) increase until it obliterates the lumen of the vessel, producing throm- bosis ; or, (2) undergo calcareous degeneration, roughening the wall and inducing fibrin deposits, which may occlude the vessel, producing thrombosis, or be washed onward, producing embolism; or,(3) undergo fatty degeneration, thus eroding the intima and weakening the muscular coat, causing aneurisms (miliary or larger) which rupture, producing cerebral hemorrhage. Miliary aneurisms are about 1 mm. or smaller in diameter, and are found on many vessels. While they are usually due to endarteritis, von Monakow believes that they may form in senile arteries by a bulging of a normal wall, the muscular coat of which is merely thinned but not diseased. The causes of endarteritis are syphilis, the abuse of alcohol, chronic poisoning by lead, the development of poisons within the body as the result of imperfect nutrition, or of indigestion due to too much food and too little exercise, gout, nephritis, and old age. The affection is distinctly hereditary, and in many families death from apoplexy is the rule. The use of tobacco, coffee, and tea is said to predispose to endarteritis, but this is not established. Symptoms. — In the conditions of arterial sclerosis it is probable that from time to time a temporary stasis of blood may occur in the ABTERIAL SCLEBOSIS. 469 domain of small arterial branches causing a sudden local anaemia of a small area ctf the brain. It is known that such a stasis in the arteries of the leg gives rise to a sudden paralysis of the leg causing intermittent claudication, in which condition the leg is useless for a few minutes. The frequency with which a sudden sense of inability to talk, to walk, Fig. 216. Endarteritis obliterans. Occlusion of the right posterior communicating artery of the circle of Willis. The vessel has dwindled to a fibrous cord. Foci of atheromatosis are noticeable on the basilar artery. M., aged forty-one years ; case of pseudodementia paralytica urfemica. Reproduced from a photograph. ( Berkeley, Mental Diseases. ) to do any special act, or to think, without any loss of consciousness, occurs in patients suffering from arterial sclerosis leads to the supposi- tion that similar stasis may occur in the brain — not of sufficiently long duration to cause permanent softening. The so-called serous apoplexy, from which patients die, M'ith no apparent lesion in the brain except an cfidema, may also be thus explained. 470 CEREBRAL Diseases op vasculab oeigin. A careful study of two hundred consecutive cases of apoplexy in private practice of which I have records has shown that in 80 per cent, of these cases there have been distinct prodromata of the apoplectic attack. For months, or even years, before the occurrence of the rupture or occlusion of the blood vessel the patients have complained of various cerebral symptoms. These symptoms all point to a dis- turbance of cortical activity. The symptoms are very numerous and variable. Those that are the most commonly observed are a disturb- ance of mental action which the patients complain of as a dullness and hebetude, difficulty of clear thinking, difficulty in remembering events of recent occurrence, a sense of perplexity in regard to matters which should be clear ; a sense of difficulty in the management of aflFairs which should be easy ; and an irritability of temper and lack of control over emotions, that become a source of annoyance both to themselves and to tile members of their family. Other patients complain of tem- porary sensations of numbness or weakness in one limb, or in one side of the body ; of irregular darting pains through the head ; of ringing in the ears, or of sounds in the head ; of slight uncertainties of vision, occasional sudden dimness being not uncommon, and of vertigo and of loss of speech. These symptoms occur with such uniformity as a preliminary to apoplectic attacks that they must be traced to the mal- nutrition of the neurones consequent upon the original arterial disease. As all the processes of nutrition in brain cells and fibres must be carried on primarily by a process of osmosis through the vessel walls, it stands to reason that the earliest evidence of disease in these walls will be imperfect function consequent upon malnutrition. The practical outcome of these observations is to warn the practi- tioner against dismissing a middle-aged person who complains of these symptoms with the mere diagnosis of neurasthenia. In every such case the condition of the blood vessels should be investigated, atten- tion being paid not merely to the nervous manifestations, but also to the condition of the heart, to the possible existence of an accentuated second sound of the heart, to the tension of the arteries, not merely in one wrist but in various parts of the body, especially in the temple, and to the condition of the kidneys. If these factors be recognized and the condition of arterial sclerosis be made the object of treatment, the so-called neurasthenia will be much more likely to yield to treat- ment than if attention be merely concentrated upon remedies directed to the nervous system. The treatment of the symptoms is by stimulation of the heart, but this should be temporary only. The underlying state is the only proper object for treatment. (See page 506.) Temporary states of delirium or of mental confusion in old persons and senile dementia are usually the result of long-continued malnutrition of the brain due to chronic arterial sclerosis. They may develop gradually, or may come on rather suddenly as a sequel of some acute illness, especially with imperfect functional activity of the kidneys. The symptoms are in part those just mentioned as characteristic of AMEMIAL SCLilKOSlS. 471 arterial sclerosis. But other symptoms are observed. One symptom especially fifequent is the marked disturbance of sleep. The patients become excited toward evening, do not get to sleep, or, if they do, wake suddenly early in the night and remain awake. They insist on Fig. 217. H-\ ■■/ \ . « .V-;,: ^■'4 -vj"- .•■":;;'■. ■■.<^?- . <■ i -%■■;■■• *'-'f-&jM-> V''-- Su-'.^- >->-'^i.'m . ■■ 1 \:i...'-f ■■■■. i 'A' ... }.'^' 1^ '"If Section of frontal cortex in senile dementia. Loss of tangential fibres in surface layer and of great distension of ttie capillaries with sclerotie changes. 1 laques of gliosis seen as dark spots scattered widely. The cells show fatty degeneration under higher power. T. Simchowicz. Histol. Studien ueber die Senile Demenz, Nissl's Arbeit., iv., 267, 1911. getting up, are bewildered as to their surroundings, do not know their family or servents, are alarmed at being among strangers, beg to be taken home and become more and more irritable and excited as their wishes are opposed. At times they are violent, wish to go out, attempt 472 CEBEBBAL DISEASES OF VASCULAB OBIGIN. to break the windows or doors, and cannot be persuaded that it is night and that they are at home. Hallucinations of sight and of hear- ing may be present. Toward morning they become exhausted and by dawn sink into a restless sleep which grows deeper and they may sleep till noon. Such patients gradually become confused in their minds in the day- time as well as at night. They show their lack of recognition of their surroundings, think they are not at home, do not recognize the familiar objects about them, but usually know their family. They dwell on the past ; allude to persons who are dead as though they were alive ; telj of things long past as if of recent occurrence ; but have no rec- ollection of recent events. Thus one patient said in January that it was June, that he was still in his country place and had no recollection of having come to town in October ; said that he had been down town within three weeks, though he has not been down town for ten months ; had no recollection of important business aflPairs which had caused him great anxiety last year ; and was wholly indifferent to matters told him which should have excited him. In spite of these evidences of mental failure some of these patients are quite sane at intervals. Their memory returns for a time but goes again in a few hours. In regard to matters which have long been a subject of thought and interest — such as the disposal of property — the marriage of children — their religious convictions — their habits of life — they may show a power of memory and of judgment which is surprisingly accurate, in view of the temporary states of bewilderment and excitement to which they are subject. In one gentleman, who was always excited at night and sleepless, and who had at night hallu- cinations of sight and hearing, and was at times unable to recognize his own sons, the capacity to estimate the value of real estate in various parts of -New York was good up to his death ; and his knowledge of his property was good, though for months before the end he would make a new will every day, some containing most absurd provisions, others quite in accord with a* will made before his mind became weak. Such patients take sudden likes and dislikes to those about them, often preferring an ignorant servant to the members of the family, and often showing active hostility to those formerly dearest to them. Under such prejudices undue influence is easily exerted and their legal capacity may be impaired. Such cases usually terminate by an apoplectic attack or by some intercurrent pneumonia, cystitis, or heart failure. The condition itself is not fatal. The excitement may be quieted by active purgatives or by hyoscine hydrobromate, which must be given with great care. APOPLEXY. The result of chronic endarteritis is usually an attack of apoplexy. This may be due to cerebral hemorrhage, or to cerebral thrombosis or to cerebral embolism. APOPLEXY. 473 Pathology of Cerebral Heraorrhage. — When a cerebral vessel rup- tures in the membranes the blood spreads out on the surface of the brain, filling the fissures and depressing the cortex. The clot which forms is thin and flat. It may become organized and adherent to the pia mater, or it may lie as a clot for weeks. In one case in which McBurney operated for the removal of such a clot from the third frontal convolution, for the relief of aphasia, three months after the hemorrhage the clot was only partly organized and was easily sponged Cerebral hemorrhage. Large clot in the centrum ovale above the basal ganglia. Patient lived three days after the onset in coma. (Larkin.) off from the surface and from the fissures. If the hemorrhage is sub- cortical it tears the surrounding brain, sometimes breaking into the ventricles and filling them with blood. It may be a small clot or it may^ be almost as large as the hemisphere, disintegrating its greater portion. Usually it is about two inches in diameter. The clot is globular or irregular in shape in the brain, or forms a cast of the ven- tricles. It compresses the brain tissue around it, and, as a rule, a nmnber of capillary hemorrhages are found in the adjacent brain. The adjacent brain is always oedematous. The clot gradually shrinks, and if small undergoes absorption, leaving a hematin stain. The dis- integrated brain tissue undergoes fatty degeneration, is liquefied, and is in part absorbed. There remains a mass of softened tissue, mingled with blood, which after a time becomes organized by the formation of connective tissue in and about it. This connective tissue may form a thick layer, or even a firm fibrous plaque, upon the surface of the brain, closely adherent to the cortex, and supplied with numerous blood- vessels. In one case operated upon two years after an injury, with hemorrhage, it was as hard as cartilage. If it is within the brain a firm mass of connective tissue enclosing the softened mass may grow. 474 CEBEBBAL DISEASES OF VASCULAR ORIGIN. Then, as the softened mass within this wall becomes liquefied, the final result is a cyst filled with fluid, either bloody or clear, and serous. In one case I saw a connective-tissue structure resembling a honeycomb, its cavities filled with clear fluid. A clot begins to lose its bloody color from the second to the fifth week, depending on its size ; after the eighth to the twelfth week it is a yellowish mass with hematin stains, and within three to six months the connective tissue formation is well under way. This contracts during a year after its formation, and the process cannot be said to have reached its final stage of scar tissue or cystic formation until the end of the second year. A cyst Fic4. 219. Extensive subarachnoid iiemorrhage over entire base of brain by diapedesis ; no rupture of the ves- sels ; no large clots. (Larkin.) wall may be apparent within a month of the onset in small hemor- rhages, encapsulating the softened mass. The torn brain never unites. It undergoes a fatty degeneration in the vicinity of the clot, and a proc- ess of secondary degeneration begins at once in the course of the tracts which are injured. By the end of the week such secondary degenera- tions can be detected by the Marchi stain at long distances from the APOPLElT. 475 original lesion, and after six weeks a sclerosis or replacement hyper- plasia of connective tissue has begun in the entire length of the tract which is degenerated. These degenerations are very extensive through the centrum ovale, as they affect all the association and commissural fibres which are ruptured. They can also be traced through the projection fibres downward into the spinal cord or upward to the Fig. 220. Cerebral hemorrhage. Large clot within the lateral ventricle. Boy of 14 suflfered from splenic anaemia. The hemorrhage occurred from capillary veins in the ependyiua. No large vessel was found ruptured ; death in three hours. (Larkin.) cortex. Figs. 14 and 15 show such descending degeneration in the spinal cord after hemorrhages of small and of great extent in the internal capsule. The clot exerts considerable pressure upon the brain tissue about it, not only at the time of its formation but for some time after. This pressure is quite sufficient to suspend the function of the compressed brain, and may even be sufficient to set up degenerative processes. The brain tissue which is merely compressed will resume its function when the pressure is removed. Therefore, the immediate and tempor- ary effects of a cerebral hemorrhage are usually much more severe and 476 CEBEBBAL DISEASES OF VASCULAM OSIGIN. extensive than the permanent ones. And hence a gradual and pretty- considerable relief from the initial symptoms is to be looked for in cases of cerebral hemorrhage. Such a relief, due to slow absorption of the clot, is necessarily much slower than the relief which occurs after embolism or thrombosis, where the collateral circulation is established within a week. The situation of a cerebral hemorrhage depends upon the fact that certain arteries of the brain are particularly liable to the formation of miliary aneurisms. According to Durand-Fardel, in 75 per cent, of the cases these aneurisms are found on the small branches of the middle cerebral artery that enter the anterior perforated space, viz., the len- ticulo-striate and lenticulo-thalamic branches. These arteries are fairly large branches ; they are almost in a vertical line above the heart, the blood rushes in from the internal carotid artery under high pressure, and they are terminal arteries with no collateral circulation. Hence they have been termed by Charcot the hemorrhagic arteries of the brain, and as a matter of fact more than 60 per cent, of cerebral hemorrhages are due to their rupture. A clot thus forming destroys the lenticular or striate nucleus, or the optic thalamus, and the internal capsule which lies adjacent to them, or if it forms higher up and nearer the terminal branches it lies in the centrum ovale. In either case if the hemorrhage is large it is liable to break its way into the ventricle. The Sylvian artery and its branches have little support to their walls, as they lie upon and not in the brain, and are also prone to the develop- ment of miliary aneurisms. Cerebral hemorrhage is not uncommon in the Sylvian fissure or on the cortex, being found there in 24 per cent, of the cases. The remaining 16 per cent, of the cases are divided between the brain axis and the cerebellum, the last-named organ being most rarely affected. In about 20 per cent, of the cases the hemor- rhage breaks through into the ventricles, or occurs originally from a vetricular blood vessel. A cerebral hemorrhage may result from a diapedesis of blood cells from numerous capillaries, without rupture of the walls (see Fig. 222). The blood then collects about the vessels and infiltrates the brain com- pressing individual neurones and axones, but not producing large clots or cysts. The condition resulting is described by the French as hem- orrhagic softening {ramollissement hemorrhagiqiiej. It is present in many cells in the brain tissue about a large clot. It is the condition which occurs in purpura, in pernicious anaemia, and in leucocythaemia. Pathology of Cerebral Embolism or Thrombosis. — The artery being occluded by an embolus coming from the heart or larger arteries, or by a clot forming upon the roughened wall, or near a constriction formed by oblitering endarteritis, the brain tissue which is supplied with blood through it is cut off from its nutrition. The first arrest of blood supply is usually extensive, but when collateral circulation is established the final area or region which softens may be small. The sudden stoppage of circulation in the artery and in its corresponding veins suspend the functions in this extensive area, but as the collateral APOPLEXY. 477 circulation is resumed the functions reappear in all but the parts which are perma6ently deprived of blood. In the cortex the collateral supply by anastomosing vessels is extensive. In the ganglia and capsule it is very imperfect, as the arteries are terminal. Hence the permanent effect of occlusion is more serious in lesions of the arteries of the base than in those of the branches in the cortex. If a large vessel in the cortex — e. g., a main branch of a Sylvian artery, or the middle cerebral trunk itself — is plugged, the area of softening may be extensive. When a vessel is occluded a clot forms within it which extends backward to the next large branch. In some cases a second embolism is produced by a portion of this clot being swept off into this branch ; then a second attack follows the first within twenty-four hours. This is the explanation of the recurrence of symptoms which have apparently subsided on the day after the attack in cases of embolism. Thus in one of my patients three attacks occurred on three successive weeks ; the first caused paralysis of the arm, the second of arm and leg, the third of the entire side and face. Fig. 221. Transverse section of the middle cerebral artery from a case of obliterating endarteritis. The proliferation of the inner coat and the reduction of the lumen of the vessel are evident. (Spiller.) A serous infiltration and oedema of the brain occurs in the entire domain of the artery whicli is occluded, within an hour ; but this is to some extent relieved when the collateral circulation is established. Capillary hemorrhage or diapedesis of blood cells is also found in the redematous brain. There are some cases of apoplexy in which the only le-sion found after death is an cedematous state of one hemisphere. These are the cases in which a thrombosis of one internal carotid or middle cerebral has been followed by a ra})idly fatal termination before softening has been pr<;duced. 478 CEEEBEAL DISEASES OF VASCULAB ORIGIN. The pathological process in cases of embolism in which the embo- lus is of bacterial origin, as in ulcerative endocarditis, or in pysemic emboli, is somewhat different, as there is an acute encephalitis of limited extent set up in the necrotic area. In addition to the fatty degenera- tion there is an infiltration with leucocytes and the production of pus in which micro-organisms are found. In such cases there is a tendency of the process to extend beyond the limit of the tissue first deprived of its blood and to the production of an extensive encephalitis. A fatty degeneration and a necrosis of brain tissue follow the occlu- sion of the vessel on the third day. In the softened tissue there are Fig. 222. Obliterating endarteritis. The intima is greatly thickened, and one-half of the lumen is filled by deposit of newly-organized tissue that has undergone fatty degeneration in its outer part. A throm- bus Is seen adherent on one side to the intima. It filled the vessel, but contracted during the process of hardening the specimen. at first extravasations of blood and pigment granules (red softening) ; later, after four weeks, these are absorbed and the tissue in a stage of fatty degeneration is yellow (yellow softening). If there is simple necrosis without extravasation or fatty degeneration in the white mat- ter, the brain has its natural color, but is soft (white softening). Round granule cells are everywhere to be found in this softened tissue, and are mingled with drops of myelin in all stages of fatty degeneration. The perivascular spaces are filled with leucocytes and small cells.' The softened tissue undergoes liquefaction or becomes organized by a connective tissue growth. This is formed partly by hyperplasia of the glia and partly from the connective tissue of the smaller vessels which proliferates. It contracts, leaving a depression, or a cavity, or ^ See Friedmann, Handbk. der path. Anat. des Nervensj^stems, S. 489, 1903, APOPLEXY. 479 a scar. In some cases a cyst with smooth walls and serous contents is formed. Such a small cyst is shown in Fig. 254, in the midst of a diffuse tumor of the optic thalamus. The size of the area of softening and of the subsequent scar may be very small, so that at the autopsy a mere patch of sclerosis is found. On the other hand, the softening may involve almost an entire hemisphere. Occasionally bilateral lesions are found. Secondary degenerations follow softening exactly similar to those following hemorrhage, already described. The situation of an area of softening due to embolism is not wholly a matter of accident. As the majority of emboli come from the heart it is natural that embolism of the left hemisphere should be more fre- quent than that of the right hemisphere ; (the ratio is five to four cases), the course of the blood current from the aorta being more direct into the left carotid artery. Such an embolus if large enters the middle : Fig. 223. Obliterating endarteritis. Thickening of all the coats of the artery. The intima has encroached upon the lumen. The new tissue has undergone fatty degeneration, and a considerable mass of fatty debris in which cholesterin crystals can be seen, lies between it and the media. A thrombus is seen adherent to the intima. cerebral artery and stops it, or one of its branches in the Sylvian fissure, thus causing a necrosis in the central area of the brain cortex. If small it is more likely to go directly up with the blood current into one of the branches of the middle cerebral that enters the perforated space. It is found that 60 per cent, of emboli enter these vessels, 25 per cent, enter one of the branches of the Sylvian artery, and the remaining 15 per cent, enter some of the smaller branches of the brain axis. Softening of the cerebellum from embolism is very rare. The situation of the area of softening due to thrombosis depends on the size and position of the thrombus. It is determined by statistics 480 CEEEBBAL DISEASES OF VASCULAB ORIGIN. that thrombi form in the following cerebral vessels in the order named, viz., middle cerebral or its branches, basilar, vertebral, anterior cere- bral, and posterior cerebral. The pathological changes following thrombosis differ in no way from those due to embolism. The relative frequency of these brain lesions may be gathered from the following statistics. In 1908 consecutive autopsies at the Presby- terian Hospital there were 112 cases of cerebral hemorrhage and 48 cases of cerebral softening. Old scars were found in ten brains, and cysts were found in thirteen brains, all but two in the basal ganglia. In a few cases multiple lesions were found. The situation of the hemorrhages and softening was as follows : Table X. — Situation of Lesions in Apoplexy. Frontal lobe . Parietal lobe. Occipital lobe Temporal lobe Caudate nucleus Lenticular nucleus Optic thalamus Internal capsule Lateral ventricle Pons Cerebellum . Inferior peduncle of cerebellum Under corpora quadrigemina. On floor of fourth ventricle . Central part of cerebral hemisphere Multiple small hemorrhages . Hemorrhage. 20 10 5 9 6 14 10 18 10 11 4 1 4 2 1 4 Softening. 5 10 4 5 2 11 4 6 i 4 Etiology. — Males are more frequently affected by the vascular diseases than females, and hence death from apoplexy occurs oftener in men. Of one hundred consecutive cases of my own eighty were males. No age is exempt from cerebral hemorrhage. It may occur prior to birth, and the infant may be born with a clot in the brain. Rupture of the vessels is the probable cause of the majority of cases of infantile cerebral palsy which follow traumatism during labor or severe convulsions. Hence the lesion may occur in infancy or child- hood. But persons in middle life are most liable, the age from forty to fifty being the age at which the greater number of deaths occur. Table XL — Age of Onset in Apoplexy. Cases of apoplexy, wit 1 autopsy. Without , autopsy. Starr. Total Age. Tooth. Dana. Gintrac. 1 to 20 . 8 4 19 12 43 21 " 30 . 7 6 19 6 38 31 " 40 . 17 10 37 15 79 41 " 50 . 43 11 21 53 128 51 " 60 . 24 7 19 52 102 61 "70 . 20 10 26 41 97 71 " 80 . 5 5 22 14 46 81 " 90 , ... ... ... 5 5 APOPLEXY. 481 When the condition of endarteritis is present any severe effort, physical or mental, any sudden emotional shock, any sudden exposure to cold, a blow on the head, or indulgence in alcohol, or the use of a strong heart stimulant may cause a rupture. Thus, lifting a heavy weight, straining at stool, the effort of coughing violently, or vomiting, and the effort of running fast have been the active causes of an apoplexy in cases of my own. I have seen two cases in children suf- fering from whooping-cough. I have known a sudden grief and sudden fright, and also a long-continued anxiety followed by disap- pointment, and in several cases a fit of rage, to be the cause of a hemiplegic or aphasic attack. I have known many cases to follow a debauch. A cold bath has been known to cause an attack, but this I have seen but once, when it was taken soon after a hearty meal. Grant believes that strychnine and quinine in large doses may cause an attack. I have seen two attacks in a young woman subject to purpura. Oppenheim records a case of hemorrhage in the brain fol- lowing an operation for bleeding piles. The operation was followed by hemorrhage from the lungs and from the bladder before the apoplexy occurred. The sudden arrest of menstruation is said to have caused apoplexy. A hemorrhage sometimes occurs during a severe epileptic fit. This is a common history in children. Cerebral hemor- rhage is the cause of death in many cases of chronic nephritis, because of the existence of hypertrophy of the heart and of disease of the arterial walls, which are constant accompaniments of that affection. The increased intra-arterial pressure produced by the force of the blood thrown up from the hypertrophied heart is responsible for the majority of cases of cerebral hemorrhage occurring during Bright's disease. Cases of hemiplegia occurring with or after diphtheria, scarlet fever, and pneumonia are due to rupture of the arteries. It is possible that the toxins of the disease weaken their walls. The condition of the bloodvessels in purpura may lead to cerebral hemorrhage, also the state of the blood in pernicious anaemia and in leucocythsemia. The causes of embolism are cardiac disease, with the formations of vegetations upon the valves or of small fibrin deposits on the rough endocardium which are washed into the circulation. In ulcerative endocarditis these contain numerous micro-organisms. Similar emboli may come from the interior of the larger arteries, and atheroma of the aorta is occasionally the cause of cerebral embolism. An embolus in a small vessel may arise from a thrombus in a larger vessel. Clots may also come from the lungs and even from the veins of the body by way of the larger veins in the lungs. Thus cerebral embolism is a frequent complication of tuberculous disease of the lungs and an occasional complication of pneumonia. I have known two cases to follow phlebitis of the saphenous vein. In these cases secondary thrombi from the lung were the probable cause. The exciting cause of the embfjlism may be a sudden physical effort or a sudden mental or emotional shock. 31 482 CEBEBBAL DISEASES OF VASCULAR ORIGIN. The cause of thrombosis is any form of endarteritis which narrows the bloodvessel and finally obliterates its lumen, leading to an ob- struction of the blood current and to the formation of a clot. Ob- literating endarteritis of syphilitic origin is the usual condition leading to such a filling up of the vessel, and as it is a very common condition it is the active cause of the majority of cases of apoplexy in young persons. In fact it may be stated without hesitation that the vast majority of cases of apoplexy occurring before the age of forty are due to syphilitic endarteritis. Senile endarteritis is also very common. The exciting cause of the formation of a thrombus is anything which weakens the action of the heart and thus slows the blood current. Cases of apoplexy occurring during sleep are usually cases of throm- bosis. Fig. 224. f \ Bilateral softening of the lenticular nuclei after gas poisoning. (Larkin.) Fig. 224 shows a condition of softening in both corpora striata con- sequent upon thrombosis in a case of gas poisoning. The artery sup- plying the lenticular nucleus turns at a rigiit angle in its course. If the heart is weakened the current of blood is arrested at this turn stasis occurs, a thrombus forms and softening results. This is the cause of death in fatal cases. It is the cause of motor paralysis in those who recover. A general state of weakness, a syncope, the reaction which follows effort, mental excitement, or emotional shock, a fright, or a condition of exhaustion from malnutrition or starvation, may induce such a state of weakness in the heart as to lead to a stasis in the vessels ; and if such a stasis occurs in a vessel whose lumen is contracted or whose wall is rough a clot may form. It is not certain whether pathological states of the blood may lead to the formatioa of clots within the vessels, though this is the theory which accounts for the cases of thrombosis occurring in infectious diseases, in phthisis, in gout, rheumatism, anaemia, chlorosis, leucocythemia, and in the puerperal state. Symptoms. — The symptoms produced are so nearly alike in cases of hemorrhage from a vessel and softening due to obstruction in a vessel APOPLEXY. 483 that they may be described together. It is admitted that In extreme cases, sucli as a large hemorrhage or a small embolism they differ widely, and that then a differentiation can be reached. But it has been my experience, from a study of a large number of such cases during life and a comparison of symptoms with pathological findings, that it is not possible to arrive at a positive accurate diagnosis between a clot and a softening in more than one-half of the cases. Hence these symp- toms are here discussed together. Premonitory Symptoms. — In all cases of endarteritis the patients suffer from symptoms of disturbance of the circulation in the brain for some months or even years before an actual attack of an apoplectic nature occurs. Miliary aneurisms, however, do not usually produce any symptoms, and may be latent until they burst. The cases which uniformly escape prodromata are cases of cerebral embolism due to heart disease or those not arising from endarteritis. These symptoms are peculiar sensations of oppression in the head, an internal pressure, the sensation of a band about the forehead, or of fulness and pulsation of the vessels. These come and go irregularly for months, sometimes after physical or, more commonly, after mental effort or emotional excitement ; sometimes in connection with atmos- pheric changes, to which some persons are particularly susceptible ; sometimes without known cause. The influence of alcoholic stimulants is more quickly felt by persons who have endarteritis, and in such persons disturbances of digestion are likely to cause vertigo and headache. Headache is probably the most common symptom of endarteritis. It is usually a dull frontal or occipital pain, rarely unilateral, and vary- ing in intensity from hour to hour while it lasts. It occurs after mental or physical effort or very often after anything which exhausts the strength or depletes the nutrition. It may be due to congestion of the brain, and is then shown by a redness of the face, with distended veins and red eyes and a feeling of fulness and pulsation. It is equally fre- quent, however, in conditions of cerebral anaemia and malnutrition ; for pale, ill-nourished persons, or those who have had exhausting diseases, or who have a weak heart are just as subject to headache as are the plethoric. In such patients, however, there is no congestion of the face and eyes ; and the pain is often felt in the vertex. Headache is rarely, if ever, a continuous symptom, and the attacks are sometimes few and infrequent, in other cases very common. Few patients escape prior to an apoplectic attack. The headache in syphilitic cases is usually felt in the afternoon and evening. It is remarkable that very severe and persistent headache often disappears at once after an apo- plectic attack. When such an attack is followed by severe or persis- tent headache the prognosis is most unfavorable. Attacks of vertigo are among the common symptoms of endarteritis, coming suddenly after efforts, such as straining at stool, running, or lifting weights, or after excitement, or in persons of weak heart, after a meal. They usually alarm the individual^ being sometimes, but not 484 CEBEBBAL DISEASES OF VASCULAR OEIGIN. often, bad enough to cause staggering or the need of lying down. They are commonly merely of the nature of a sense of swaying, or a sense of uncertain stability, or of a movement of the floor. If a person has a severe attack of vertigo with inability to rise and with attendant faintness and vomiting, it is probably due to the formation of a small thrombus or to a minute hemorrhage in the cerebellum. Such attacks often pass off and leave no permanent trace, but occasionally they are followed soon by more serious symptoms. Insomnia is a symptom of endarteritis. The patients cannot get to sleep on going to bed, and lie and toss and think and worry ; finally they are exhausted and fall into a light sleep in which they twitch and turn and are troubled by distressing dreams, waking in the morning without any sense of rest. This is a condition commonly associated with congestion of the brain. It may be due to too forcible heart action, as in cases of endarteritis associated with nephritis and hyper- trophy of the left ventricle, or in cases where the arteries are so rigid as to have lost their elasticity and power of physiological contraction during sleep. After this kind of insomnia rupture of the artery with the formation of a clot is more common than thrombosis. In other cases the obstruction to the circulation causes insomnia by depriving the brain of blood and nutrition. In this condition the patients are unusually drowsy, doze in the daytime, especially after meals, and fall asleep as soon as they go to bed. They sleep heavily, often snoring when this is not their habit, but after three or four hours they wake, and then cannot get to sleep again. The insomnia, therefore, in such patients is worse in the early morning hours. They watch the day break and are early risers. This type of insomnia is more difficult to treat successfully than the other type. For while the former can be cured by warm baths, by hot drinks, and food, by the synthetic hyp- notics and bromides, the latter often is intractable. Sometimes a light meal taken in the night on waking, or a heart stimulant, is followed by a nap, but hypnotics are useless. These patients often have their apoplectic attacks during the night, and it is usually due to thrombosis rather than to the rupture of a vessel. When syphilis is the cause of endarteritis the patients usually have difficulty in getting to sleep early ; but they sleep heavily and are wakened with difficulty, being dazed for some moments after waking. Mental symptoms are frequent prodromata of an apoplexy, and are often present in a state of endarteritis. These may consist of a sense of difficulty in fixing the attention, a defective memory of names, a slight tendency to use one word in place of another, a confusion of thoughts, or even an inability to think without conscious effort and distress, and a marked emotional state due to a lack of self-control. Thus patients may be irritable and impatient on slight cause, may get enraged at little things, or may laugh or cry with hardly any provoca- tion. It is common for these symptoms to be associated with headache and to be of temporary character, passing away with the pain. Patients may suffer for many months from these mental symptoms, and such symptoms may be the only ones observed even in an attack. AFOPLEXT. 485 Seiisations of numbness in one arm or in one leg or in one entire side are not uncommonly complained of by persons who are the subjects of endarteritis, and are very common prodromata of an apoplexy. In two hundred consecutive cases of apoplexy in my private practice of which I have full histories, a complaint of preceding numbness had been made in eighty-six. This consists of a tingling feeling, or the same sensation as when one's foot is asleep. It lasts a few moments or even a day or two, and then passes off. It may be produced ex- perimentally in one-half of the body by compressing the opposite carotid artery, and hence is due to a disturbance of the circulation producing anaemia. When such an attack of numbness wakens a person at night it may cause much alarm. It is not to be confounded with local pressure on the ulnar nerve, as it is diffused over the entire hand; nor with the numb fingers of acro-parsesthesia, which is bilateral ; nor with venous stasis in the limb from pressure which is attended by congestion and pain. It may remain permanently, under which circumstances, an actUal rupture of a vessel or a stasis of blood in it has occurrred. A nd after an attack of apoplexy, with or with- out paralysis, numbness in the affected side is almost always present and remains. Hence, when such attacks of numbness occur they are an infallible warning of the danger of apoplexy. Yet I now have patients under my care who have had many such attacks for several years, but never have had anything more serious. The numbness is rarely attended by an objective anaesthesia. It is a purely subjective symptom. Sometimes a slight awkwardness is associated with it, but when an actual ataxia develops it is because a lesion has occurred. Transient attacks of aphasia or of unilateral paralysis, usually of one hand or foot, occasionally precede a permanent attack. Ringing in the ears, noises like bells or whistles heard ; flashes oj light before the eyes, or a sudden sense of blindness in one side passing off in a few moments ; a subjective sense of a bad odor or an unusual taste have occurred in different persons as prodromata of apoplexy and as symptoms of endarteritis, but are far less common than the other symptoms mentioned. Hemorrhage from the nose is common in persons with endarteritis, and retinal hemorrhages are sometimes found by ophthalmoscopic examination. The prodromata of an apoplectic attack may occur for many months or years and never be followed by a stroke. They should never be disregarded, however, by patient or physician, and it is far better to take pains to avoid the active causes of a cerebral hemorrhage or thrombosis than to suffer its consequences. Some form of prodroma had been felt in more than one-half of my two hundred cases. The Apoplectic Attack which announces the occurrence of a cerebral hemorrhage, or an embolism, or the formation of a thrombus is always of sudden onset and causes a great mental shock to the patient. Apoplexy, the falling as if struck down, is not always attended by unconsciousness. In fifty-four out of two hundred cases a loss of 486 CEBEBBAL DISEASES OF VASCULAE ORIGIN. consciousness occurred. But in the liospital patients observed the proportion was much greater, and in patients who die, unconsciousness at the onset is the rule. The coma is attended by stertorous breathing, by full, slow, tense pulse, not irregular or intermittent, by marked venous congestion of the face, and by complete relaxation of the limbs, so that it is sometimes difficult to determine whether one side of the body is paralyzed. In some cases the head and eyes are turned toward the lesion. The pupils are usually dilated in cases of hemorrhage, the one on the side of the lesion being the larger, and they fail to react to light. In pons lesions they are contracted. The corneal reflex is lost, so that irritation of the eye does not cause a wink. In cases of hemorrhage distention of the retinal veins has been observed. The temperature usually falls at first, and then begins to rise within a few hours, the paralyzed side being a degree higher than the other. There may be an immediate unconscious evacuation of the bladder and rectum, if these are full. If not, there is usually retention of urine during the period of unconsciousness, and the bladder has to be emptied by catheter to avoid its rupture. The state of coma may continue, the temperature may rise to 104° or 105° F., oedema of the lungs and heart failure may follow, and the patient may die within a few hours or on the following day. Occasionally in persons of good vitality the fatal result may be delayed as long as a week, consciousness never returning. In these cases the greater relaxation of the limbs on one side, a manifest drawing of the face and elevation of the corner of the mouth on the non-paralyzed side, with dribbling of saliva on the other side, the failure to elicit reflex movements by pricking one side, the loss of skin reflexes and loss or exaggeration of tendon reflexes, the occurrence of automatic or instinctive movements on the non- paralyzed side, or a marked contracture of the arm and leg, indicate the existence of a hemiplegia. In one girl who never recovered con- sciousness, and whose lesion was an extensive hemorrhage at the base after fracture, any attempt to uncover the body caused an automatic grasp and effort to draw up the bedclothing on the unparalyzed side. The condition of coma is more likely to occur when the lesion, either a clot or softening, is deep in the hemisphere, is near the lateral ven- tricle, or involves the optic thalamus. It is also an indication of a large hemorrhage or the plugging of a large vessel. Extensive corti- cal clots or thrombi almost always cause coma, but small ones do not. The condition of coma is usually attended by a marked increase of arterial tension, as shown by the Riva Kocci apparatus and by the sounds of the heart. This is the probable cause of the increased secretion of uriue which always follows an attack. The urine if ex- amined is often found to contain albumin and sometimes sugar, which disappear within a week after the attack, and may, therefore, be due to the increased arterial pressure. The attack of apoplexy may be attended by a convulsion. This may be general, and if repeated is an indication that the clot has burst into the ventricles. It is a most unfavorable prognostic symptom. APOPLEXY. 487 The convulsion may also be unilateral. This is an almost infallible sign of a lefeion of the cortex in the central region. If the patient recovers consciousness after the attack the mind is usually very dull, and response to questions is for some hours impos- sible. Then attempts to talk are made, and if there is no aphasia they may be intelligent. Frequently it is some days before the patient recognizes his surroundings. As the mind becomes clearer a definite estimate of the symptoms present becomes possible. In the majority of cases paralysis is found. This was present in one hundred and seventy-seven out of two hundred patients. It occurs in the form of hemiplegia and affects one side of the body only. The face is drawn to the non-paralyzed side, as there is no longer any op- position to the normal tone of the non-paralyzed muscles. This gives an unnatural appearance to the face which sometimes leads the unprac- ticed observer to suppose that the drawn side is the one which is paralyzed. The muscles about the eye which close the lids are not involved, and thus the facial paralysis in hemiplegia differs from that due to an affection of the facial nucleus in the pons or of the facial nerve, in which the eye cannot be closed. The motion of the eyes is not affected except in lesion of the crura or of the corpora quadri- gemina. Deviation toward one side may occur during the coma, but this does not persist when consciousness returns. The tongue is also paralyzed, and when protruded turns toward the paralyzed side, being pushed over by the healthy muscle. Swallowing may be difficult and choking frequent for some days after an attack, and this is a sign of unfavorable prognosis. The head can usually be turned freely, though in severe cases the same tendency to turn both eyes and head away from the paralyzed side present in the coma may persist for some days. It is due to the action of normal muscles whose opponents are paralyzed. The arm and leg are completely paralyzed, are relaxed and offer no resistance at first to passive motions. The paralyzed muscles show a normal electrical reaction, and have no tendency to rapid atrophy or to fibrillary twitching. During the coma and state of shock the tendon reflexes may be lost, but they soon return, and by the end of the second day are exaggerated on the paralyzed side, the knee-jerk being high, and a crossed thigh- jerk being sometimes obtained ; that is, a slight adduction of the well thigh and extension of the well leg follows a tap on the patella tendon of the paralyzed side. Ankle clonus is often obtained, and also a retraction of the great toe on irritating the sole of the foot (Babinski's sign). These symptoms continue even though the paralysis improves. The skin reflexes are lost on the paralyzed side, a sign of great diag- nostic importance in distinguishing a true from a false (hysterical) hemiplegia. A loss of the abdominal reflex occurs in lesions of the frontal region even when there is no paralysis. The body is some- times paralyzed so that the patient cannot turn in bed, or move his body, or sit up. Inspection of the chest usually shows that respiratory movements are more active on the paralyzed side. The control of the CEREBRAL DISEASES OF VASCVLAJR ORIGIN. bladder and rectum are lost, and either involuntary evacuations or retention may continue for many days. As time goes on the hemiplegia may gradually subside. I have known it to pass off in part after three mouths of complete paralysis, so that the patient eventually walked and signed his name. As a rule, some beginning voluntary motion is evident by the end of two or three weeks, the power of drawing up the leg being the first to return, then to stretch out the leg, then the power to move the ankle, and finally some flexion of the elbow and shoulder. The finer motions of the hand and foot are regained last of all, if they return at all. The face appears straighter in the course of two months. Walking can be re- sumed after a light attack within a month, but may be regained after a year of paralysis. Every possible variation in the degree of the original hemiplegia may be observed. In light attacks a sense of weakness only is com- plained of, and these patients are awkward, but do not lose power in any muscle absolutely, and are soon able to use the hand and to walk. Such patients recover entirely, and may have no trace of the attack at the end of the year. After severe attacks, however, in which the motor tract is injured, and in which a descending degeneration occurs, a par- tial hemiplegia remains. The muscles gradually become rigid and slow and stiff in motion, the power of finer movements being chiefly im- paired ; the tendon reflexes are exaggerated, as is also the muscular irritability, so that tapping a muscle causes it to contract. Nevi finds that if a hemiplegic while standing bends the body forward to a right angle with the thighs, the limb on the paralyzed side becomes flexed, while that on the sound side remains straight ; and if, when lying down, the legs are lifted up, the one on the paralyzed side becomes flexed when an angle of 40° is reached, while the sound leg can be lifted to at least 65° without flexion. This is of use in dif- ferentiating an organic from a hysterical hemiplegia.^ The gait in hemiplegia is typical. The entire side of the body seems to move together, the body being stiif, the shoulder falling toward the weaker side, the arm being carried in an adducted flexed position with wrist pronated and fingers flexed, the leg being drawn forward with little bending of the knee and ankle, or being carried about in the arc of a circle, the ball of the foot and its inner side scraping the floor. The foot is sometimes turned in and scraped along the floor on its outer side. When the original lesion has been located in the cortex but one limb may be paralyzed. When it lies in the crus the eye on the side of the lesion cannot be turned inward, upward, or downward; its pupil is dilated, and there is a falling of the upper lid. When it lies in the lower half of the pons a condition of alternating paralysis is produced, the face being paralyzed on the side of the lesion, the arm and leg upon the other side. In this condition the facial palsy involves the upper branch of the facial nerve so that the eye cannot be closed. In a few cases of lesion in the medulla the face has escaped, the tongue has been ^ Nouvelle Icon, de la Salp^triere, Jan. , 1910. APOPLEXY. 489 paralyzed on the side of the lesion, and arm and leg on the opposite side. In ^lemorrhage into the ventricles, and occasionally in small lesions of the pons and medulla in the raphe, a bilateral or a general paralysis of all the limbs is produced. Thus the original or permanent distribution of the paralysis indicates the position of the lesion. In 200 cases of apoplexy right hemiplegia occurred in 96 cases, being permanent in 73 ; left hemiplegia occurred in 55, being permanent in 44, alternating paralysis occurred in 4, a lesion of the cms cerebri in 1, and total paralysis in 2. In some cases hemiancesthesia accompanies the hemiplegia ; in a few cases it is the only symptom produced. In the majority of cases of apoplexy it is absent. The symptom is a sensation of numbness in one side, accompanied by a certain degree of loss of the power of per- ceiving touch, or pain, or sensations of heat and cold. The loss of sensation is never permanently complete in organic lesions. In all cases after the shock has passed away and consciousness is clear some sensation is perceived, especially in the shoulder, or thigh, or foot. One of these senses may be partly lost, the others being preserved. Thus in several of my patients touch was very much impaired, but hot and cold objects and painful pricks were felt keenly. In one case touch was not affected, but pain could not be felt, nor differences of temperature. In two cases the patient had a constant sensation of cold in the left side, could not feel warm objects as such at all, but felt them as cold, and felt touches and painful sensations, but less keenly than on the well side. In one of these cases there was a hemi- plegia as well ; in the other cerebellar symptoms without hemiplegia. In another case the sense of pain and of heat was lost, but cold could be felt and a subjective feeling of cold was present all the time. This patient was slightly hemiansesthetic but had no paralysis. An alter- nating hemiansesthesia may be caused by lesions in the pons or medulla, as in three of my patients, without any paralysis. The degree of the anaesthesia may vary, being very slight in many patients and only detected by careful comparison of the two sides of the body simul- taneously tested. In many cases the hemiansesthesia found immedi- ately after the attack passes off, and in the course of a month sensation is normal. It is only when the sensory tract lying behind the motor tract in the capsule is destroyed, or when the optic thalamus is affected that a permanent blunting of sensation remains. And this is never total, as each hemisphere of the brain receives some impressions from both sides of the body. In 200 cases right hemiansesthesia was found in 21, being permanent in 17 ; left hemiansesthesia was found in 20, being permanent in 19 ; alternating ansesthesia was present in 3 and permanent. A loss of tactile memories is sometimes present and indi- cates a lesion of the cortex in the parietal area. Thus patients who are blindfolded cannot recognize objects placed in the hand. This may occur even when the liand is not ansesthetic. It is termed astereog- nosis. (See page 109.) Ilemiataxia or an impairment of muscular sense and consequent 490 CEREBRAL DISEASES OF VASCULAR ORIGIN. awkwardness of movement is an infrequent accomplishment of hemi- plegia. It may occur alone. It leads to an incoordination of motion, both in the gait and in the use of the hand. In 4 cases out of 200 it was noted, and in all it was permanent, in 3 being at first associated with hemiplegia. In one case it was attended by a complete loss of temperature sense, the sense of pain being preserved. Hemianopsia is a frequent symptom of apoplexy. It is bilateral homonymous hemianopsia, causing blindness in one-half of both eyes. As a rule the patient appreciates the blindness in the eye whose tem- poral field is blind, and is not aware that the other eye is aifected until the physician tests the visual fields. Hemianopsia is usually a per- manent symptom. In only 2 out of 13 cases in the series of 200 cases did it pass away. But a gradual increase in the area of the visual field is common, and I have charts which show a great improvement in vision. In the patients who recover the visual tract has been com- pressed but not destroyed. When the lesion is in the visual tract beneath the cortex the visual fields are more irregular and asymmetrical in outline than when the cortex is affected. Right hemianopsia is more common than left hemianopsia — 9 to 4 in my series of cases. (See page 120.) A loss of visual memories indicates a lesion of the cortex of the occipital lobe and angular gyrus. It is termed mind-blindness or psy- chical blindness, and when limited to the memories of written language is termed word-blindness. I have seen this as the chief and only per- manent local symptom of an apoplexy ; the patient, who could talk and write, and was not paralyzed, was unable to recognize friends and familiar places, and was unable to read at all even sentences he had just written. It is usually associated with right hemianopsia. A loss of color vision alone, hemichromatopsia, in one-half of both eyes has been described, but is very rare. I have never seen this condition. (See page 122). Aphasia is a common symptom in apoplexy, being associated with right hemiplegia in right-handed people and with left hemiplegia in left-handed people. In two of my cases it was attended by left hemi- plegia ; both patients were left-handed. It is usually of the form of motor aphasia, but any of the various forms may, of course, be found. Aphasia may occur as the only symptom of an apoplexy ; but this is rare. In the 200 cases of apoplexy, 118 patients had aphasia at the onset, and in 84 this condition was never entirely recovered from, though a very marked improvement occurred in the majority of patients when they lived over two months. In 1 7 of these cases aphasia occurred alone ; in the others it was associated with hemiplegia. In one case it passed away entirely, though it was the only symptom of the attack, and in another case it subsided wholly after four months. In many cases the aphasia remained after the hemiplegia had entirely passed off. The various forms of aphasia and their significance in determining the location of the lesion have already been considered (page 136). The mental symptoms occurring in apoplexy have not been suf- ficiently emphasized in text-books. Some cases of apoplexy occur in APOPLEXT. 491 which they/ilone are present ; confusion of mind, and inability to think, to remember, or to fix the attention, together with a great emotional excitement and lack of control, being felt. These are cases of organic dementia and frequently present many of the symptoms already de- scribed as characteristic of senile dementia. Other cases cannot be considered as demented, for though some mental symptoms are present, and the mind is never as strong, as active, and as useful as before the attack, the mental symptoms pass away. I have a man under my observation now, perfectly well, who twelve years ago had an attack with loss of consciousness and dementia, with lack of memory lasting a month, and inability to transact business for six months, but with no aphasia or paralysis. In 63 of my 200 cases, marked mental symptoms were present and were permanent. In two of these patients delusional insanity finally developed. In 40 a peculiar lack of emotional control was the marked symptom. One man was in a state of constant uncon- trollable laughter, would laugh aloud whenever he spoke or made any voluntary movement in his left side, which was partly hemiplegic. In two men frequent crying was complained of, and occurred on any excitement, even when the patients were amused. Such emotional acts may be as uncontrollable as a convulsive spasm would be, and are equally without intention. In several cases such emotional attacks, either of laughing or crying, were common on any excitement. In some cases after the patients recover from the shock of the attack there remains for months a distress on mental effort, a state of bewilderment, in which they lose the thread of conversation, cannot read or write for any length of time without becoming confused, and are forgetful. Some patients lose all recollection of their surroundings, and hence im- agine that they are away from home, in a strange place. One such patient, an active, intelligent business man, has been reduced to a piti- able condition, without paralysis, aphasia, or dementia, yet so incapable that in reply to almost any question he answers, " I don't know, I can't remember, I am of no use." These patients distrust their own capa- city, become vacillating and capricious, are sometimes suspicious, some- times easily influenced uncjuly by others. In several of these cases a permanent loss of control of the bladder and rectum has persisted after the attack.^ Seguin and Brissaud have noticed that a loss of emotional control occurs more frequently in lesions of the right frontal lobe, and this I can confirm. In many cases in which the mental symptoms are the chief permanent symptoms there has been a slight facial paralysis or a slight hemiplegia or aphasia with the attack, which has subsequently passed off, thus showing that the lesion was anterior to the central or motor region. These patients rarely if ever have hemiansesthesia or hemianopsia. Cerebellar nymptoms, consisting of violent vertigo, double vision, and a staggering gait when the attack is over, may be the chief features of an apoplectic attack. In such cases consciousness is rarely lost, ' YoT furtlier cjiKes see the Medical Record, November 13, 1897, "On Some Unusual Forms of Apoplectic Attack." Also May 16, 190G, on Cerebellar Apoplexy, 492 CEitESEAL DISEASES OF VASCULAB OBIGIN. but the patient is usually extremely prostrated, feels weak and greatly alarmed, may vomit, and break into a cold sweat, and remain incapable of moving in bed or of sitting up for some days. All attempts at motion are attended by an increase of the vertigo and a renewal of the nausea and vomiting. The pulse is weak, and may be either very rapid and intermittent or slow. In one such case I have seen Cheyne- Stokes respiration. In my collection of 200 cases there were four that presented these symptoms. As patients recover the staggering gait may gradually improve, but there remains a sense of uncertainty and a tendency to fall in one direction. Thus one patient always felt as if about to fall to the right side. She subsequently had a second attack with hemiplegia, and a third attack which left her demented. Nystagmus is associated with the vertigo in these cases and usually persists as a permanent symptom. The head is usually held stiffly and sometimes inclines toward the side of the lesion. In severe cases of hemiplegia with hemiansesthesia there is a very marked tendency to the development of bed-sores upon the buttock and heel of the paralyzed side. The surface becomes red after slight pressure of the body weight, blebs soon appear, and an ulcerated sur- face forms which shows a tendency to slough and break down rapidly, so that within a week a deep sore may be present, even exposing the bone and causing necrosis. The lesion in the brain is certainly cap- able of inducing vasomotor symptoms in the paralyzed side, such as cyanosis and its attendant malnutrition ; but it is my conviction that the development of a bed-sore is always due to imperfect care and lack of cleanliness, and that if the patient is turned every half-hour and the skin properly bathed with warm water containing an antiseptic, then with alum-water or alcohol, such sores may be prevented. This is very difficult when the urine and feces are passed unconsciously, and sometimes, with lack of skilful nursing, bed-sores will appear. They are dangerous, as many patients who would otherwise recover die of the septic infection thus produced. A patient who is paralyzed may have difficulty in swallowing, and attempts at feeding may result in food being inhaled into the lungs. Great care has to be taken to prevent this in patients who remain semiconscious for a week or more after an attack. The result will be to develop a pneumonia of septic origin. Charcot believes that the vasomotor paralysis may involve the lung, and in proof cites the frequency with which pneumonia, either septic or spontaneous, de- velops on the paralyzed side in these cases. Such a pneumonia is a common cause of death after an apoplectic attack, and must be regarded as of serious prognostic import. Patients who are unconscious during the attack have retention of urine and inability to empty the i-ectum. As a rule, the bladder empties itself as soon as it fills up, without their knowledge or control. Sometimes there is a continual dribbling of urine; and for many days after consciousness returns this symptom may continue. It is some- times necessary to catheterize such patients regularly. It is always APOPLEXY. 493 well to have a bed urinal in the bed, or pads of absorbent cotton about the genitals, which can be changed frequently. The danger of a septic cystitis is never to be forgotten, as it is the cause of death in a certain number of cases of apoplexy. Minkowski ^ has shown that the cortical centres of the brain control the detrusor of the bladder, while the subcortical centers control the sphincter of the bladder. If the subcortical centres in the corpora striata and optic thalariius are de- stroyed the patient has incontinence of urine. If the cortical centres or the tracts from them to the spinal centre in the sacral segments of the cord are affected the patient has retention of urine. The rectum may require regular washing out, or, in case of impaction, a digital evacuation. Irregular Types of Apoplexy. — Some very extraordinary forms of attack are occasionally seen. Thus one patient had a sudden attack of vomiting and vertigo, and immediately developed a monospasm in the arm with a right hemiplegia without aphasia. There was no loss of consciousness, but the hemiplegia began with a convulsive motion in the right arm, which continued. Gradually the hemiplegia passed oif, but the convulsive movement continued. And for three months the arm was constantly flung about with violent flail-like movements which could not be stopped by the will. These were more violent than athe- toid motions, and were still continuing at the last report. In another patient I saw a series of convulsive attacks, five occur- ring in the course of seven days, each limited to the left side of the face and arm, each lasting a quatter of an hour, and each causing a temporary paralysis which by the end of three weeks had entirely passed off. The only permanent symptom was a slight degree of mental dul- ness and apathy with incapacity for business. In another patient a series of attacks of a convulsive nature limited to the right leg occurred during ten days ; each attack was very vio- lent, so that the entire body was shaken, and lasted for nearly half an hour, leaving the leg paralyzed. Then the attacks ceased, the condi- tion of paralysis gradually subsided in six months and she has had no return during the past seven years. One man suffered from a sudden distress in his head, followed in an hour by a general convulsion, which left him with right hemiplegia and aphasia. During the following three months the condition of paralysis entirely subsided and he recovered his speech, but never recovered his mathematical faculty. He cannot do the simplest sum. This first attacK occurred in 1887. Since that time until his death in 1905 he has had, every six or twelve months, sudden attacks consist- ing of confusion of mind, complete inability to speak, though he under- stands what is said, a twitching which begins in his fingers and thumb, and gradually involves the whole right arm, then the right side of the face, and then the right leg, attended by a tingling of the entire right side. This convulsion lasts several hours, then ceases slowly and leaves a paralysis which passes off entirely during the following two »Deut. Arch. f. Nervenh., Aug. 21, 1907. 494 CEBEBBAL DISEASES OF VASCULAR OBIGIN. days. I know of over twenty such attacks having occurred during the past fifteen years. In the interval he appeared hearty and vigorous ; though eighty years old, he walked five miles a day. He had some difficulty in talking, found words slowly, but was not aphasic. His memory was poor, he had no cardiac disease, but his arteries were tor- tuous and hard. I have similar histories in three other cases. In all these cases the probable lesion was cortical hemorrhage, or thrombosis, as spasm is considered a sign of cortical irritation. General convulsions do not often occur in an apoplectic attack in adults unless the clot bursts into the lateral ventricle. In children, however, they are very common at the onset, and as we shall see in discussing infantile hemiplegia, convulsions come either with or after the attack in the majority of the cases. In two young girls patients of my own, where the hemiplegia occurred during an attack of whooping- cough, general convulsions accompanied the attack. In adults their occurrence is of bad import, but in children this is not so. In one patient who had a sudden attack of left hemiplegia at the age of sixty-eight years, a violent tremor developed in the left hand soon after the attack. This persisted for five months until his death. The hand and arm soon became cyanotic and oedematous, were covered with cold sweat, and were wholly paralyzed. He became violently insane soon after his attack, had well-marked hallucinations and delusions, did not sleep for two months, and finally died of exhaustion. In several patients a febrile movement has persisted after the apo- plectic attack, and severe headache with insomnia of an obstinate kind, not much relieved by anything excepting large doses of morphine, have appeared. In these patients the hemiplegia has shown no tendency to improve, and in all a fatal result has followed within two months. I look upon this combination of symptoms with much dread, and con- sider that in all such cases an acute inflammation must be set up in the brain about the clot or softened area. In the cases where the clot invades the ventricles, general convul- sions are usual, are frequently repeated, the paralysis affects both sides of the body, sometimes a rigidity of the limbs follows, the pupils are widely dilated, the temperature falls, the pulse is slow, and respiration is labored and stertorous. Such an attack may follow a milder attack after twenty-four or forty-eight hours, the original clot breaking through into the ventricle. Hence the need of keeping patients quiet after even a slight attack. These patients usually remain comatose and die within twenty-four hours, of cyanosis and asphyxia. Yet in one hospital case I have seen a gradual recovery after two weeks of uncon- sciousness attended by all these symptoms, and the final result was an extreme state of hemiplegia which remained six years to my knowledge without recurrence. But one patient in my series of 200 cases showed symptoms of ventricular hemorrhage, and died in the attack. Bulbar paralysis of the apoplectic type is illustrated by the following case : A man previously healthy but alcoholic was suddenly attacked at the age of fifty-three years with weakness of the entire left side, fol- APOPLEXY. 495 lowed in a few minutes by paralysis in the entire right side and attended by difficulty in speech and in swallowing. There was no headache, no convulsion, and no loss of consciousness. His condition had been stationary for five years, when I saw him. His eyes were normal. There was no weakness of his face, but his speech was thick, voice loud and husky, and not under control, and swallowing and articula- tion were very imperfect. His tongue protruded, but was thin and had a marked tremor. He was very emotional, laughed and cried without cause. His power in arms and legs was good, but he had an extreme degree of ataxia in all motions of hands and legs, a very ataxic gait, increased by closing his eyes, but his knee-jerks were normal. His control of his bladder was imperfect. He had no pain, no vertigo, no deafness. The probable lesion was a small hemorrhage in the raph6 of the medulla affecting both interolivary tracts, and his ninth and twelfth nerves. Such cases have been observed and the lesion found by Senator, Meyer, and Spitzka. Attacks of Apopledie Nature without Loss of Consciousness. — In the majority of cases of apoplexy a loss of consciousness does not occur. Under these circumstances, at the time of the attack the patient feels suddenly a sense of pressure in the head, or a dizziness, with headache, or finds it necessary to sit down, or is able to drag himself slowly to a chair, or falls if he cannot reach a seat. He then notices a numbness in one limb which extends to the entire side of the body, and is attended by some weakness in the arm and leg. Or he becomes confused in his mind and cannot find words, and soon develops aphasia. Or his numb- ness increases, his arm becomes ataxic, and he moves his leg with dif- ficulty. Or he suddenly becomes conscious of a blindness in one eye which on examiuation is found to be a hemianopsia. Or he feels be- wildered and confused, and can no longer understand what is said to him. Or he is dizzy and staggers in walking. Any one or any com- bination of these symptoms may come on suddenly, may recede, and then return, to leave the patient finally in a state of incapacity of some kind. As a rule, when the attack is slight and not accompanied by unconsciousness or followed by fever, there is a fairly rapid improve- ment ; and after two weeks the patient is able to sit up and begins to recover his lost power. But a complete recovery does not often follow, and some trace of the original symptoms usually remains. Aphasia is not wholly recovered from, some hesitancy of speech or tendency to misplace words remains, some local symptoms continue, and a trace of hemiplegia is seen in his gait for the rest of his life. If the symptoms pass off entirely, as they did in 16 of my 200 cases, the patient may remain well for some years, but the probability is that a second attack will occur. Attacks of G^radual Onset. — Thus far attacks of a sudden nature have been considered. But in a certain number of cases the onset of the nervous symptoms is gradual. Thus in several of my patients four or five days have elapsed before all the symptoms have appeared. In these cases the mental dulness and apprehension, the sense of something 496 CEBEBBAL DISEASES OF VASCULAB OBIGIN. occurring iu the head, with pain, vertigo and weakness, are present throughout the period of onset. In some cases aphasia first appears, then becomes more complete ; right hemiplegia is added, and finally hemiansesthesia. In other cases the paralysis advances slowly from face to arm, and then to leg, or in the reverse order, and if riglit sided, aphasia may finally develop. Mills ^ has described a slowly progres- sive hemiplegia ascending from the leg, the exact pathology of which is unknown. In some cases hemiansesthesia precedes the hemiplegia. In some hemianopsia first occurs, and then a hemiansesthesia or sensory aphasia, which may subsequently subside. In still other cases the mental symptoms become more and more marked, confusion of thought is evident in speech and action, and a dementia finally develops, often attended by emotional excitement, crying or laughing without cause. In some cases monoplegia, or monospasms, or a loss of sensation in one limb only, is the final result, the initial symptom being more extensive than the final ones. It is in these cases of slow onset that the various types of aphasia are particularly frequent, and psychical blindness or deafness or some form of subcortical aphasia persists. The recurrence of apoplectic attacks is a danger to which all patients are liable. Twenty-two patients among my two hundred cases suffered from more than one attack, one died in his fourth and one in his fifth attack. I have known one person to survive seven attacks, and many to survive the third which according to popular superstition, is uni- formly fatal. The same person may have a recurrence of the same symptoms in each attack, or may have different symptoms each time, depending wholly upon the bloodvessel which is affected. One man, aged seventy years, had an extraordinary series of attacks. The first and second were attacks of right hemiplegia with aphasia, the third was of left hemiplegia. These occurred on successive days and lasted a few hours. Five days later he had a fourth attack. During the following ten days he had three more, one right-sided, two left-sided. All this time he was growing more feeble, but after each attack the paralysis passed off in a few hours. Six days after the seventh attack he had an eighth which left a permanent left hemiplegia from which he died four days later. His arteries were rigid, his heart was weak, but the sounds were clear. The diagnosis made was multiple thrombosis, and he was treated by nitroglycerin and strychnine, the administration of which in each attack seemed to be followed by a recovery from the paralysis. The autopsy showed multiple areas of softening due to thrombosis. In several cases multiple lesions were found at autopsy. There is no rule to determine the interval between attacks. A few months, a year, or even ten years may elapse before the recurrence. The average in twenty-two cases showed that the chance is in favor of a second attack within two years of the first one. But one of my patients did not have his second attack until fifteen years after the first one. The Terminal Condition. — The course of the disease after an attack * Journal of Nervous and Mental Diseases, April, 1900 ; October, 1903, APOPLEXY. 497 of apoplexy is usually one of slow improvement and a greater or less degree of n&covery. The terminal condition may be much more favor- able than the original symptoms would lead one to suppose. Many patients remain in a condition of partial mental enfeeblement with less control of the emotions than before the attack, and some defects of memory, especially of events occurring about the time of and subsequent to the attack. Many patients suffer from a partial hemiplegia. The face recovers almost entirely, a slight flatness on one side, and a little deviation of the tongue only remaining. Often the facial expression of amusement or of grief is perfectly shown when voluntary movement is at fault. Sometimes, as in one of my cases, the voluntary power returns in the face, but the paralysis is evident when emotion is shown. This patient had hemiplegia with hemiansesthesia. The arm is usually permanently paralyzed to a greater degree than the leg, and is more commonly subject to contractures. The flexor muscles are usually contracted and hold the elbow flexed, the wrist flexed and pronated and the fingers closed, and the extensors and supinators being weak cannot overcome them. In one patient the arm, forearm, and fingers were rigidly extended, the fixed position being similar to that assumed in reaching out for an object a foot away from the hip. The conse- quence is a marked deformity of the hand in severe cases and an awkward movement in light cases. A rigidity of the muscles develops within two months of the onset, or sooner, so that even passive motion is difficult. This may sometimes be overcome for the time being by massage, by rapid motions of flexion and extension of a limb by hot applications, or by an Esmarch bandage. As a rule, the rigidity is absent when the patient is asleep, and only returns after waking when he begins to move the limb voluntarily. The limb may be so stiff as to be painful, and pain on motion of the joints is a frequent complaint in hemiplegic patients. The arm is usually held close to the side, and complete abduction or circumduction at the shoulder is rarely possible. The leg is also rigid, is moved as a whole, and is held straight. The flexors of the knee and the extensors of the foot are paralyzed to a greater degree than other muscles, and their opponents are held rigid. Hence the knee is not bent in walking, and the foot often assumes an equino-varus position. In very severe old cases in which the patient is confined to bed, an extreme flexion of hip and knee with adduction of the thigh may develop. The rigid muscles may be hypersensitive to percussion or may be too rigid to contract when tapped. The tendon reflexes are uniformly exaggerated on the paralyzed side, and not infrequently on the healthy side as well to a less degree. Ankle clonus is easily obtained, and in extreme cases of hemiplegia I have obtained a clonus by depressing the patella, by depressing the toes, by extending the wrist or fingers or thumb. In cases which recover entirely from paralysis this increase of tendon reflex may remain. Babinski's reflex is always present. In some cases subsequent to hemiplegia a slow spontaneous but iu- 32 498 CEBEBBAL DISEASES OF VASCULAR ORIGIN. voluntary movement begins in the upper extremity, producing flexion and extension of the fingers and hand and often extensive motions of the elbow and shoulder. These are more rarely seen in the foot and in the face. This is athetosis. It is increased by any voluntary effort of the paralyzed or of the healthy hand, and cannot be arrested. Emo- FiG. 225. Athetoid movements of the fingers. (Curschmann.) tional excitement also increases the motion. This symptom is seen in infantile hemiplegia oftener than in adult cases, but it occurs in adults. I have known it to cause peculiar grimaces in the face and an interrup- tion of speech from contortion of the muscles of the face and tongue. Hammond ascribed it to a lesion of the optic thalamus, but it is now regarded as an evidence of cortical irritation. When such symptoms APOPLEXY. 499 are due to lesions iu the optic thalamus it is probable that the lesion causes an irritation of sensory fibres which is conducted to the cortex, and there gives rise to motor impulses. Thus in several children suf- fering from athetosis no cortical lesion has been found at operation. The most pronounced case in an adult that I have seen developed after an operation for the removal of a brain tumor from the posterior cen- tral convolution, and continued for a year after the operation, subsiding slowly as voluntary power was regained. I ascribed it to pressure from a clot which was gradually absorbed. Hemiathetosis is, there- fore, an occasional sequel of an apoplectic attack. Hemitremor is also occasionally developed, a tremor which may be constant even during rest, or which may only occur on voluntary motion. It occurred in but one of my cases. Rhein ^ has shown that it indicates a lesion of the lenticular nucleus iu some cases. Hemichorea may remain after a hemiplegia has subsided. In one case where it had been a symptom for fifteen years Aufschlager found an old cyst in the optic thalamus. ^ In other cases lesions have been found in the superior cerebellar peduncles or in the red nucleus of the tegmentum. The lack of voluntary exercise usually leads to a slight atrophy of the paralyzed muscles. An extreme and rapidly advancing condition of atrophy without any change in electrical reaction has been occasion- ally observed, and cannot be explained. The joints occasionally become painful, probably from disuse, and require massage and passive motion. A true synovitis with hemor- rhage into the joint sometimes develops, as Charcot pointed out, but this is exceedingly rare. I have never seen a case, and question any causal relation between the apoplexy and its occurrence. A condition of hemiansesthesia, hemiataxia, and hemianopsia occa- sionally remains. The first brings with it a disagreable sense of ting- ling or numbness, and leads to awkward movements even when ataxia is absent. The second leads to marked incoordination, of finer motions, and patients drop objects from their hands when not looking at them. Hemianopsia is rarely recovered from, but patients become accustomed to having but one-half of the field of vision clear, and guard against accidents from objects approaching the blind side. Deafness from brain lesion is so rare as to be a curiosity. Mills has reported a case with bilateral destruction of the temporal lobes. These defects of sensation are much more likely to remain stationary than is the condition of paralysis. Any one of the many forms of aphasia already described may persist after a vascular lesion. In fact it is largely from the study of small local areas of softening of the brain due to embolism or thrombosis that our knowledge of the possible forms of aphasia and apraxia is due. It is not necessary to recount these symptoms here. The peculiar combinations of cranial nerve symptoms with hemiplegia characteristic of pons and medulla lesions need not be described, as they have been considered in the chapter on diagnosis of local lesions. 'Jour. Nerv. and Ment. Dis,, Dec, 1907. 2 5ieitschrift f. klin. Med., Bd. LI., th. 2 u. 3, 1903. 500 CEEEBEAL DISEASES OF VASCULAB OBIGIN. Diagnosis. — An apoplectic attack may be distinguished from an at- tack of syncope by tlie pallor, stoppage of heart, and weak respiration, dilatation of the pupils, preservation of knee-jerks, rapid recovery, and absence of coma, or subsequent local symptoms of a brain lesion in syncope. An epileptic attack, with cry, biting of the tongue and general con- vulsion is followed by a state of coma which may be mistaken for apoplexy when no history of the onset can be obtained. But an epi- leptic's tongue is scarred, and there are usually scars upon his head or extremities as evidenc3 of prior attacks. Epilepsy is a disease of youth, and the epileptic rarely shows signs of the vascular conditions which lead to apoplexy. The coma is not very deep, the patient when aroused shows no paralysis, the pupils are equally dilated, there are often hemor- rhages in the conjunctiva and capillary hemorrhages on the face. And within an hour or two returning consciousness enables a history of prior attacks and the absence of any paralysis to be established. The coma of alcoholic intoxication is not as deep as that of apoplexy, and the drunken man can usually be aroused, and gives evidence of his condition by his breath, his dirty appearance, his maudlin resist- ance, his delirium and restlessness, and his lack of paralysis. The corneal and pupil and tendon reflexes are preserved. It should not be forgotten, however, that some cases of apoplexy occur in a state of intoxication, or from traumatism in this state, and symptoms of com- pression of the brain, unilateral paralysis, or inequality of the pupils, should lead to careful watching in any case of doubt. The signs of alcoholic intoxication will pass off in a few hours, a brisk purgative or a large salt enema hastening recovery. The coma of opium poisoning is attended by very slow respiration, small, rapid pulse, cool skin of blue color, and extreme contraction of the pupils ; it is never as deep as the coma of apoplexy, and never attended by unilateral symptoms or loss of the corneal or tendon reflexes. A ursemic condition leading to convulsions and coma may be mis- taken for apoplexy if a full history of the preceding symptoms of nephritis is not obtainable. But the symptoms of oedema of the tibiae and face, the urinary odor of the breath, the small amount of bloody urine of high specific gravity and loaded with albumin and containing casts will point to the correct diagnosis in cases of acute Bright's dis- ease. Ursemic coma is the culmination of an attack which begins with vomiting, headache, and convulsions. In cases of chronic cirrhotic kid- neys apoplexy is a common occurrence, and hence in such cases the finding of casts and albumin in small amount, with urine of a low specific gravity, does not aid in the diagnosis. And here it is only by the absence of the active local symptoms of apoplexy, especially the lack of unilateral paralysis or difference in reflex actions on the two sides, that a diagnosis is to be reached. It is often wise to watch a case for twenty-four hours before arriving at a conclusion. The same may be said of diabetic coma, though here the exami- blAGNOSIS. 501 nation of ^he urine is of far greater value, as the glycosuria which follows a cerebral hemorrhage is rarely as extreme as that found in diabetes. The odor of acetone is unmistakable, and is not present in' apoplexy unless this is associated with diabetes. In cases of doubt reliance must be placed upon the previous history, as diabetic coma is not of sudden onset, is usually preceded by headache, anxiety, distress, and sensations of suffocation, followed by somnolence. But it is to be remembered that diabetic patients sometimes have apoplectic attacks. An apoplectic attack may be the first symptom of general paresis, or may occur at any time in the course of the disease. The symptom will then be due to a hemorrhage in the meninges, usually a dural hematoma, and will be identical with those of a cortical cerebral hemor- rhage at the time of the attack. The history of mental symptoms of a grandiose kind, of mental failure, and loss of memory ; of attacks of emotional excitement ; of disturbances of speech ; of unusual physical efforts in a person really weak ; of exaggerated knee-jerks, and other signs of paresis, will lead to the diagnosis. And the result will be a much more rapid and complete recovery from the hemiplegia or aphasia produced by the attack than is ever seen in a hemorrhage which destroys the brain. The differentiation between cerebral hemorrhage, thrombosis, and embolism is always difficult and often impossible ; but when the char- acteristic symptoms of any one of these affections are present a prob- able diagnosis can be reached. In hemorrhage, if the person is young, there is a history of whoop- ing-cough, of convulsions, or of purpura ; if old (and the majority of cases occur after the age of forty), the individual is ruddy, muscular, or corpulent, has a history of nephritis or of endarteritis, has no his- tory of syphilis, has no cardiac murmur, but has a strong and possibly hypertrophied heart. There is usually a history of mental excitement or physical exertion preceding the attack, but rarely any history of premonitory vertigo or mental confusion. There is a history of very sudden loss of consciousness, with flushing or cyanosis of the face, a marked pulsation of the bloodvessels, the pulse being full, slow, and irregular, of high tension, a slow stertorous respiration, and deep coma from which these patients cannot be aroused. There is often a differ- ence in the size of the pupils, and they fail to react to light, and the eyes are turned to one side. During the first day there is a fall of temperature to 97.5° F., followed by a slow rise to 102° F. A rigidity of the paralyzed limbs occasionally appears, but unilateral twitching or convulsions are rare. The paralyzed side at first feels cooler, but when the temperature of the body begins to rise it is often found to be a degree higher than the other side. The paralyzed extremities are sometimes slightly cedematous, often cyanotic, and sweat freely. The urine often contains albumin and sugar after a hemorrhage. A rapid increase of leucocytes in the blood follows hemorrhage. When the patient recovers consciousness after twenty-four hours, or not until 502 CEBEBBAL DISEASES OF VASCVLAB OBIGIN. after two or three days, he is much dazed, comes to himself very slowly, complains of pain in the head, is restless, and tries to move ; is sleep- less, but is very dull mentally and incapable of showing an apprecia- tion of his condition for at least a week. Hemiplegia is almost always present ; often associated with hemiansesthesia, and sometimes with hemianopsia, though the two latter symptoms may subside within the first ten days. Aphasia is almost always present if the right side of the body is paralyzed, but is rarely one of the pure special types, such as word-deafness, or word-blindness, or a pure motor aphasia. Bed- sores occur more frequently in cases of hemorrhage than in thrombosis or embolism, and so does pneumonia. During the month following a hemorrhage there is a gradual but steady improvement in both mental capacity and in the local symptoms. Some permanent defect, however, is always left with signs of secondary degeneration in the motor tract in the majority of cases. Retinal hemorrhages occasionally may be seen with the ophthalmoscope. In thrombosis the patient if young has a history or physical signs of syphilis, and if old is the subject of endarteritis, his temporal arteries being tortuous, his radial arteries hard, his pulse irregular, and his second heart sound accentuated. There may be a history of some acute illness, especially of an infectious type, just prior to the attack. There is a history of some premonitory symptoms extending back several months, such as headache, vertigo, or insomnia, even if there have been no slight attacks of numbness or of paralysis. It is particu- larly in these cases that the prodromata which have been described occur. There is no nephritis, and the urine is normal. There may be a history of some shock or fright, of some general sense of faintness or weakness just prior to the attack. There is usually a slow onset of the attack, giving a few moments of alarm and conscious illness ; in fact, the patient may feel his paralysis coming on before consciousness is lost. In the majority of the cases there is no loss of consciousness. The face is pale, the pulse normal, not full or slow, the respiration is regular, and there is no fall of temperature. The pupils are equal and react to light. The coma is not very deep when it occurs, and irrita- tion of the body produces automatic movements which show one side to be paralyzed. Twitching of the limbs is common, convulsions rare. The coma does not last more than twelve to twenty-four hours, and when consciousness returns the mind is clear, at first, though excite- ment and even delirium often follow. There are no signs of increased intracranial pressure. The local symptoms are less extensive than in hemorrhage, and rather more so than in embolism. They show con- siderable variation in the first week. Thus a hemiplegia may pass into a monoplegia, a hemiansesthesia may wholly disappear, and a general aphasia subside into some special type, as word-deafness or word-blind- ness. In other words, the compensatory collateral circulation in the brain being established and the initial oedema subsiding, the functions at first suspended may be resumed. But the area of the brain perma- nently softened does not get back its power, and hence permanent local- DIAGNOSIS. 503 izable symptoms of small extent remain, and these show little tendency to improve. Mental symptoms of the nature of impairment of memory, loss of emotional control and excitement as permanent symptoms are more common in thrombosis than in hemorrhage or embolism, and both precede and follow the attack. And there is a marked tendency to recurrence of attacks, usually without loss of consciousness, with a renewal of symptoms which have in part subsided, or with new symp- toms of a local kind. The ophthalmoscope may show arterial sclerosis in the retina. In embolism the patient is usually young and has an audible heart murmur or a history of phlebitis, endocarditis, rheumatism, or infec- tious disease ; is not the subject of nephritis, and has no signs of endar- teritis. He has no special apoplectic appearance, and is pale rather than ruddy during the attack. The attack occurs without premonition, and not after effort or motion. It is not always attended by a loss of con- sciousness. When coma occurs, and as a rule it does not, it is not very deep, and irritation of the body usually causes some automatic motions, even if there is no semiconscious response. The pulse is not full and not slow, but is such as would be present in the cardiac state found. The respiration is rarely stertorous, and the temperature does not fall. The pupils are not unequal in size and usually react to light. Unilateral spasms, or twitching of the paralyzed limbs, or convulsions beginning in one limb and extending to others finally becoming general, are not infrequent, and during the attack and after consciousness is regained these may recur. There is no difference in the temperature of the two sides, and no oedema or cyanosis. Leucocytosis is less mgirked than in hemorrhage. When the patient recovers consciousness he does so rapidly, does not complain of symptoms of pressure in the brain, but often passes into a state of delirium which may last some days. Ex- tensive local symptoms are much less common than limited ones. Thus some special type of aphasia easily distinguished from other types, monoplegia rather than hemiplegia, or hemianopsia alone are the symp- toms found after the third day. There is often a very surprising im- provement in all the local symptoms on the second day, with a return of them on the third or fourth day, followed then by a very slow improve- ment. Sometimes the local symptoms are permanent. In other cases they may entirely pass away, and no trace of the attack may remain. The ophthalmoscope may show embolism of the central artery of the retina. When in any case the symptoms mentioned are present there is little doubt as to the diagnosis. But in many cases there is an absence of many of the distinguishing symptoms, and a differentiation is impossible. This fact is substantiated also by autopsy records. Thus in twenty cases of supposed embolism where cardiac disease was present and the attack was fairly typical, nine were found after death to have been cases of hemorrhage (Kleber ^). It has been my experience that in few con- ditions are errors of diagnosis more common than in apoplexy. ^ Von Monakow. Gehirnpathologie ; Spec, Path. u. Ther. von Nothnagel, Bd. xi., S. 776. 504 CEBBBMAL DISEASES OF VASCVLAB OMIGIN. Ad heroic method of diagnosis, by boring a hole in the skull and aspirating the brain, thus finding hemorrhages and relieving pressure, has been advocated by Neisser,^ who reports a case of extra-dural hemorrhage in the posterior fossa, in which 180 cc. of blood was removed. Deaths have been reported due to hemorrhage in the brain after such explorations. They may be warranted to ascertain the existence of extra-dural clots but not otherwise. Prognosis. — The prognosis in an apoplectic attack depends some- what upon the cause of the apoplexy. Cerebral hemorrhage is much more likely to be fatal than thrombosis or embolism. The first attack is less likely to be fatal than subsequent attacks. The older the patient the less likelihood of his recovery. Very deep coma, its duration for more than a day, a rapid fall of temperature, or a rapid rise of temperature, the development of Cheyne-Stokes respiration, heavy stertor, the deviation of head and eyes to one side, are all important and very unfavorable symptoms, a sudden extreme degree of arterial tension, over 240 mm. ; or a sudden fall of tension, below 80 mm. is unfavorable. (See page 466.) If convulsions occur with signs of increased intracranial pressure a ventricular hemorrhage has probably occurred and death is sure. Retinal hemorrhages are of bad import, as they show a high degree of pressure in the skull, and hence a large clot. Bilateral paralysis, especially if attended by cranial nerve palsies, shows a lesion of the pons or medulla which is almost surely fatal. It is not to be forgotten that a slight attack may be fol- lowed immediately by another, more severe, and this by a fatal attack. If the patient recovers consciousness after the attack the development of delirium, of high temperature, of insomnia, of great restlessness, of pain in the paralyzed limbs, a tendency to bed-sores, and the failure to control the sphincters make a fatal termination within two weeks probable. If few or none of these symptoms occur, and the temper- ature becomes normal within a week, and some slight improvement in the local symptoms is manifest, life will probably be spared. The greatest caution must be exercised in giving a prognosis on the degree of recovery from the local symptoms. Aphasia which is general at the outset always passes away in part, and has, in my experience, more chance of improvement than any other local symptom. A pure type of aphasia, motor or sensory at the out- set, is usually permanent, though careful education enables a patient in the course of time to regain his speech at least to some degree. Aphasia alone without hemiplegia may be entirely recovered from, or may remain permanent and incurable. In 56 cases of attacks of aphasia 14 recovered the use of speech completely. Hemiplegia, even if total, may not remain. It usually passes oflP in part, and it is safe to predict that the patient will walk again, even in severe cases. In one case under my care the patient walked well at the end of a year, though he had made no voluntary movement what- ever in arm or leg at the end of three months. In another case a year elapsed before the patient could stand alone, yet two years later he 'Deut. Zeitsch. f. Nervenh., Vol. xxxiv., p. 6, PMOGNOSIS. 605 walked without a cane. The arm and hand are always more completely and permanently paralyzed than the leg, and many patients who can walk well never regain the power of writing or of feeding themselves. Yet I have seen complete return of all but the very finest of motions, such as the power to pick up a pin or to button the clothing. The increase of reflex in the knee and ankle is not an unfavorable sign. But if contracture of the limbs develops after the first month a complete recovery never takes place. In the series of 200 cases here collected, of 177 patients with hemiplegia 22 recovered completely, and in every patient who lived beyond the second month a very marked improve- ment occurred. The sooner the return of any motion the more com- plete will be the eventual recovery. The paralysis commonly passes away from the face entirely, so that only on very careful testing is a difference in the two sides to be seen. This is probably because even in bad cases the instinctive movements of facial expression are not affected, excepting when the optic thalamus is destroyed, and the con- trol of the face is bilateral in many persons. The tongue usually re- covers with the face. It is evident, therefore, that a promise of much improvement in the power of motion may almost always be given. Post-hemiplegic athetosis and tremor rarely if ever subside or improve. The prognosis in regard to sensory disturbances is less favorable than in regard to paralysis. The initial hemiansesthesia usually sub- sides. But when it persists more than a month it never wholly leaves the patient, and it is usually attended by a feeling of tingling and numbness which gives rise to much discomfort. This numbness also makes a patient awkward in his movements, even when it is not asso- ciated with paralysis. Hemianopsia when due to shock or to pressure on the visual tract usually passes off within ten days or two weeks. If it persists beyond that time it is due to a lesion of the tract or cortex, and is incurable. I have never seen the symptom of hemianopsia pass away when it had persisted one month. The limits of the visual field may extend a little and the patient may learn in time to disregard and even to forget his blindness, but a perimetric examination usually shows that it is stationary. The irregular motor and sensory and cranial nerve symptoms due to lesions of the crura, pons, and medulla, when they are not followed soon by a fatal ending are usually permanent and have a bad prognosis. They are due to a rupture of small tracts which never unite, and are followed by secondary degenerations which are permanent. The cerebellar symptoms often pass away, but a tendency to vertigo may persist. The mental symptoms attendant upon an apoplexy are never abso- lutely recovered from, and after each attack they are more marked and permanent. When they are due to the underlying endarteritis it is to be expected that they will be even worse after than before the attack. The actual obscuration of ideas following the attack, the inability to comprehend, to think, to judge, and to act slowly subside. The flow 506 CEBEBBAL DISEASES OF VASCULAR ORIGIN. of ideas becomes clearer, and if there is no aphasia it may be possible for a man to make a will within two weeks of the attack. But sub- sequently to an attack the mind may be permanently enfeebled, and when an emotional state develops, and the memory is easily confused, and the eifort of continuous talking or reading causes distress for two months after an attack, it is a question whether it will ever be possible for the man to resume his occupation. Yet it is often amazing to see how accurately a business man can judge of his affairs, and how clearly, even though aphasic, he can indicate his desires in regard to important matters while severely paralyzed. In the series of 200 cases, mental symptoms were permanent in 63. But in several cases where more than two slight attacks occurred it was these symptoms which remained and which rendered the patient incompetent after the aphasia and hemiplegia had entirely subsided. A very rapid improvement in all the local symptoms after an apo- plectic attack should not invariably be regarded with favor, as this is the history in attacks occurring in cases of general paresis. Such a recovery should awaken a suspicion of this affection, which, if found, will greatly change the prognosis. It is always to be remembered that, excepting in children, one attack of apoplexy predisposes to another. Endarteritis is a steadily progressive disease. No prognosis can safely be given as to the length of the interval between attacks, but a second attack is reasonably sure. The prognosis in cases due to syphilitic disease of the arteries is not more favorable than in other cases ; for no amount of antisyphilitic treatment can affect the condition of softening due to a thrombus in an artery the subject of obliterating endarteritis. It is, therefore, a mis- take to give a good prognosis in syphilitic cases or to promise any more from treatment in such cases than in others. In fact these patients are more liable to recurrence than others, and hence the prognosis is worse. An obliterating endarteritis is not to be cured. My experience in this respect is quite the reverse of that of von Monakow.^ The eventual prognosis after the end of the sixth month is much worse in cases of embolism and thrombosis than in hemorrhage. For in the former the symptoms then present show the permanent effect of softening, while in the latter a shrinkage of the clot and its scar or cyst may go on for two years. Treatment. — For a person who has beginning endarteritis it is essential to adopt a manner of life which will arrest the progress of the affection and prevent its most serious result. It is generally agreed that a diet which is simple in character, without rich sauces and strong condiments, and which does not contain an excess of nitrogenous matter, is the one least likely to increase the arterial disease Patients there- fore should not eat a great deal of meat, eggs, or cheese, and many are better for avoiding milk. It is most important to avoid alcohol in any form, and the use of wine, beer, and spirits should be forbidden. Strong 1 Gehlrnpathologie ; Spec. Path. u. Ther. von Nothnagel, Bd. ix., S. 870. TBEATMENT. 607 coffee and tobacco are also supposed to induce endarteritis, and should be used in moderation only. A proper care of the digestion is there- fore imperative. An annual visit to Carlsbad for a mild course of treatment, or to Saratoga for a corresponding cure, is most advantage- ous. The habitual use of water both with and between meals is to be urged. A fair amount of exercise not of an exhausting character is to be commended, golf and horseback riding being advisable as equally good means of out-of-door exercise, and billiards or Indian-club swing- ing with light clubs the best indoor forms. Tennis, or lifting weights, or rowing "are to be avoided, as they put too severe a strain upon the heart for a person with endarteritis. Exercise should cause a mild perspiration, but if followed by exhaustion or palpitation, is objection- able. Many persons live more comfortably without exercise, but if the lack of it induces obesity it must be taken. Tepid or cool baths are preferable to very hot ones, and the sudden shock of cold is to be avoided. A climate should be sought without great extremes of heat or of cold, and chills should be guarded against. As the accurate observations of Carter ^ have proven that iodide of potassium does not decrease pulse tension, I see no reason to believe that its continued use in small doses, which is advised by many authors, can in any way pre- vent arterial disease. But a person who has had syphilis should certainly take a course of mercury every three years and a course of iodide of potassium every six months, to prevent the development of endarteritis. Patients who have any arterial disease should avoid sudden exertion, and this is a good rule for every one over forty-five years to follow, as many cases of apoplexy occur after exertion in apparently healthy per- sons. The existence of miliary aneurisms is often unsuspected until they rupture after a sudden effort. Thus one of my friends, a healthy woman of fifty years, lifted a trunk and immediately had a cerebral hemorrhage which killed her. It is just as imperative in persons with endarteritis to avoid exhausting effort as it is for one who has heart disease. And mental or emotional excitement of an intense kind is equally to be avoided. Contentment, with a moderate degree of success and wealth, is better than fame or riches bought by an apoplexy. These precautions should be particularly urged upon patients who are known to have nephritis. The treatment of an attack of apoplexy depends upon the cause, as hemorrhage, or softening, require exactly opposite measures. If it is apparent that a hemorrhage has occurred the patient should not be carried fer, or jolted in the process, but should be placed in bed with his head somewhat higher than usual, and should lie upon the non-paralyzed side rather than upon the other, or upon his back. Res- piration is always hampered by the accumulation of saliva, by a falling backward of tlie larynx, and by paralysis of the respiratory muscles on one side. If the patient lies on the non-paralyzed side the saliva runs out, the larynx does not become obstructed, and the weak side expands 'American Journal of the Medical Sciences, 1901, vol. cxxii., p. 854. 608 Ct:nEMAL DISEASES OF VASCULAB ORIGIN. better than if it is held down by the weight of the body.^ If gravita- tion acts at all on the clot, it should not favor its rupture into the ven- tricle, hence the side of the brain' which is injured should be against the pillow. The full, beating pulse which tends to increase the bleed- ing is often the result of carbonic acid poisoning from imperfect res- piration. All obstructions to breathing, tight clothing or neck-bands should, of course, be removed. The mouth should be wiped out fre- quently with an alkaline antiseptic solution. All exertion, such as swallowing, and the consequent coughing which inevitable choking produces, are to be avoided, hence no attempts at medication by the mouth are to be tried during the coma. Vomiting is always dangerous to the patient, from the strain and the possibility of inhaling particles. It may be arrested by a mustard leaf on the epigastrium. If the face is red or cyanotic, the pulse full, the coma deep, the temperature low, the respiration labored, venesection is indicated, and from twelve to eighteen ounces of blood should be taken from the arm at once. It will do no good unless done within a few hours of the attack. In hos- pital cases I have seen this cause great relief, and believe I have seen it save life. It should be employed much more freely than it is in cases cerebral hemorrhage. That it is a harmless measure is shown by its general use in the last century for all sorts of diseases. The reaction against its abuse has led to its neglect in cases where it is indicated. The cases in which it does harm are cases of weak heart, small pulse, and general anaemia. Placing a tight bandage around the limbs near the body will prevent venous blood from returning, and thus take some blood from the brain, and this is a means to be used when bleeding cannot be done. Mustard paste to the legs or back of the neck ac- complishes similar results, but serious blisters are too often caused in states of unconsciousness by these means and by hot bottles to make their use advisable. Applications of ice to the head are usually ordered, but it is questionable if they do good. They are, however, agreeable to some patients when consciousness returns and there is pain in the head ; hence it is possible that they relieve congestion. No attempt should be made to arouse the patient from the coma or to feed him, and when he begins to recover, all excitement, the presence of several persons, and attempts to talk, should be prevented ; and quiet in a darkened room should be preserved. When it seems certain that a hemorrhage is occurring the use of adrenalin hypodermically or by the rectum is advisable. It is also well to cause free purgation by giving two drops of croton oil in a little butter. This put on the tongue will be absorbed in the mouth, and is not likely to cause choking if swallowed. Con- vulsions may be controlled by chloroform or by chloral hydrate given by rectum m twenty-grain doses. When the patient recovers consciousness his anxiety should be quieted by reassurance that the danger is over. No attempt at feed- ing should be made until he feels hungry, and then cold milk only should be given for the first three or four days. If there is dilSiculty ' E. L. Bowles, Stertor and Apoplexy. London, 1891. TREATMENT. 509 in swallowing a small tube may be introduced through the nose, a spray of cocaine being first used in the throat, and milk given through this. Water may be given freely if desired, and, in cases of nephritis, should be urged upon the patient. In such cases, if there is not a free excretion of urine, an enema of salt solution will be advisable. Heart stimulants should be avoided, and wine, coffee, or tea are not to be used unless a condition of weakness of the heart becomes very mani- fest. Aconite in small doses frequently repeated is of service, as it quiets the increased activity of the heart. Hypodermic injections of camphor, two grains, dissolved in thirty minims of olive oil is the best heart stimulant if one becomes necessary. If the patient is very rest- less bromide of sodium in thirty-grain doses every six hours may be given, and if sleepless, trional in fifteen-grain doses may be used. Tepid sponge baths with alcohol are very quieting and refreshing. Great care should be taken to keep the body clean. The bladder should be emptied by catheter every three hours to prevent soiling of the bed, and a daily enema should be used to clean out the rectum. Castor oil, or aloes, or cascara may be given to keep the bowels open. Pads of oakum or cotton may be used to prevent pressure, and the position must be changed every little while to relieve restlessness and prevent bed-sores. An air-bed or water-bed is rarely necessary, but may give comfort. It has long been the custom to give five or ten-grain doses of iodide of potassium three times a day to assist the absorption of a clot. Those who believe that it will accomplish any result may continue to use it. If it is apparent that the stroke of apoplexy is due to an embolism, or a thrombosis, and the patient is pale and the heart is weak, he should be laid flat in bed, and heart stimulants with vasodilators should be used at once. A hypodermic injection of nitroglycerin, one one- hundredth grain in whiskey or cognac should be given, and the alco- holic stimulant repeated or given with hot water as an enema. Ammonia may be inhaled and Hoffman's anodyne may be used. Heat should be applied to the body, but the measures recommended in hemorrhage — venesection, purgation, ice to the head, and adrenalin — are con- traindicated. If convulsions occur chloroform may be used or chloral in large dose by enema. Absolute rest should be enforced, no effort allowed as the patient recovers consciousness, and then, when he is able to swallow, supporting food, mild stimulation with coffee, hot drinks, milk, and beef tea should be given. These measures are of great value in the slight prodromal attacks which precede a stroke in cases of endarteritis. I have seen very marked and favorable effects from the use of caffeine citrate or the sodium salicylate of caffeine, three-grain doses every six hours. If the pulse tension is high nitrite of sodium in two- or three-grain doses every four hours may be used, as it is less sudden and more prolonged in effect than nitroglycerin. It may be given with the caffeine. If an inflammatory reaction follows the embolism, and the temperature rises, and restlessness ^nd insomnia 510 CEEEBEAL DISEASES OF VASCULAE OEIGIN. follow, the caffeine must be stopped, uud bromide of sodium or chloral hydrate used. The latter in five-grain doses every four hours is of much service in these cases, as it does not increase the arterial tension and yet quiets the brain. Trional is also of service to cause sleep. A rapid relief from the coma not infrequently follows this treatment in cases of embolism or thrombosis as the collateral circulation is stimu- lated. In the cases where the temperature rises and active encepha- litis occurs, ice to the head, antipyretics, such as phenacetin, acetanilid, cool sponging, and laxatives are indicated, but medication accomplishes little. When it is impossible to make an accurate diagnosis between hemor- rhage and thrombosis or embolism it is not safe to employ active medi- cation directed to either condition, lest the damage to the brain may be increased. In such cases it is better to secure absolute rest ; to aid the respiration by position on one side ; to see that the condition of the heart and pulse is favorable, using either mild sedatives like aconite ; or mild stimulants like camphor and caffeine, as they are indicated ; to apply ice to the head, and if there is much restlessness to use- bromides freely. When consciousness returns quiet should be ensured, a mild light fluid diet ordered, plenty of water given, the skin and bladder kept in order, the bowels evacuated by enemata daily, and sedatives, bromide or trional, used if needed. In cases of mild onset without loss of consciousness the same degree of rest is to be secured as in severe cases, and for at least two or three weeks after the attack, no matter how slight, no physical or mental exertion should be allowed. A light diet, plenty of water, and mild laxatives should be used, and stimulants avoided. The subsequent treatment should depend largely upon the condition which is suspected or determined. As the majority of cases of slight attack are due to thrombosis in diseased arteries rather than to hemorrhage, vascular dilators and caffeine, with or without a small amount of digitalis, or spartein may be cautiously tried, and if found to be of benefit used more freely. If they cause headache or other premonitory symptoms they must be discontinued. In cases where spasm or convulsive move- ments have occurred the use of bromide is indicated. In all cases the treatment of the paralytic condition following an apoplexy is really expectant. It is impossible to hasten the process of repair in the brain, and time alone can bring about a spontaneous recovery. During the two or three weeks after the attack the patient must not be allowed to sit up or to make any effort for any purpose. He must be fed, his bladder emptied by catheter, the bedpan used for his evacua- tions, and he must not turn himself in bed when bathed. Cold appli- cations to the head should be kept up for a week or longer if they are agreeable to the patient. By the end of a week the diet may be in- creased and easily digested solid food may be given. A variety of fluids, such as lemonade and mineral waters, may be given, and weak tea and coffee may be allowed, but no stimulants, unless the case is one TBEATMENT. 511 of thrombosis. The position of the patieut must be made as comfort-i able as po»ssible by the use of cushions supporting the arm and leg, and their position may be changed often. Bathing with warm water and alcohol is very soothing, and may be done twice a day. No medicine is needed during this stage unless it is some sedative at night. Con- stant encouragement is needed, and the hope of recovery must be stimu- lated. At the end of two or three weeks in light cases, and at the end of four or five weeks in medium cases, the patient begins to draw the leg up in bed or to straighten it out when it has been passively flexed. After such motions have been possible for a week, but not sooner, the patient may be lifted to a chair and allowed to make attempts at stand- ing with the aid of two persons or one person and a crutch. It is not well for a patient to exert himself in this way too soon or too long, for fear of a relapse. And it does not hasten his recovery to allow him to try. The paralyzed arm should be supported in a sling. The cases are few where it can be accurately determined that there is a surface hemorrhage which can be removed by surgical interference. When this is the case there should be no hesitation in undertaking the operation. Thus in a case in which McBurney trephined for a trau- matic hemorrhage causing aphasia and right hemiplegia, the subsequent progressive recovery would undoubtedly have been impossible had the clot not been removed.^ In subcortical hemorrhage where the arterial tension is high and increasing to the fatal degree, as shown by the Riva Rocci apparatus, the cranium may be opened and the intracranial tension relieved. This measure is not to be undertaken, however, with- out good reason.^ The treatment of the hemiplegia, which should be begun at the end of the second week, is by massage of the limbs daily or twice a day for fifteen minutes at a time, by passive movements of the limbs, and by the application of faradism to exercise the muscles, especially the extensors of the wrist, the muscles moving the fingers, and the flexors and extensors of the ankle and knee. These muscles should be exer- cised for a minute at a time, and the entire application should take half an hour. The facial muscles do not require electrical treatment, as the instinctive movements exercise them. It should be distinctly under- stood that the effect of electricity is limited to exercise. The use of galvanism to the head has long since been abandoned as useless and possibly harmful. Any device which will enable the patient to make voluntary motions, and thus exercise the muscles by act of will, is far more useful than electricity. Thus as soon as any power appears in any muscle, the patient is to be urged to use it. He can do this if the action of the opposing muscle be performed passively or by a mechani- cal device. Thus grasping a bulb which contains a spring will exer- cise the flexors of the fingers while the extensors are still unable to open the hand ; pressing on the pedal of a sewing machine will exercise weak flexors of the ankle while the extensors are still unable to lift ' Brain Surgery, Case XVIII. ''See Harvey (Jusliiiifr, American Journal of tlie Medical Sciences, June, 1903. 512 CEBEBBAL DISEASES OF VASCULAR ORIGIN. the toes. Such devices not only serve to exercise the muscles but also to occupy the patient's mind, which is usually devoted wholly to the consideration of his state. Muscle beating helps some patients, and may be combined with massage. Warm applications followed by cool affusions sometimes stimulate the circulation in the limb and help the strength ; and as soon as walking is possible it should be allowed, and stepping over obstacles, and careful voluntary motions of the leg should be practiced, and the attention should be directed to the act of walking so as to prevent a hemiplegic gait from being acquired. The same training as has been used by Fraenkel in the treatment of locomotor ataxia is very useful in cases of hemiplegia. (See page 382.) The hemiausesthesia may occasionally be benefited within a week by the application of a faradic current to the limbs, the faradic brush being preferred. If it has no effect, however, within a month of the onset there is no treatment which will restore sensation, and faradism usually increases the subjective numbness which annoys the patient. The hemianopsia cannot be treated in any way, and it is often well not to call the patient's attention to it, as little by little it is disregarded. I have seen a number of patients who had bilateral homonymous hemi- anopsia but were not aware of the fact. The aphasia is open to treatment by education in some cases, but not in all. Practice in reading, in reading 'aloud, in naming objects and in writing is sometimes successful in recalling the lost memories and powers of expression, I have had patients who learned to read again by the use of the letters of the alphabet printed on little cards. I have known patients who acquired a new language before they relearned their own tongue. I have known patients who could sing before they could talk. I have known patients to carry about a little dictionary and to find in it the word they could recall by sight but could not utter, and thus to convey their ideas. If no attempts at reedu- cation are successful the deaf-and-dumb language should be acquired, and it may interest the patient to teach him this from the outset, show- ing him large printed letters and letting him form a new association of ideas between them and the position assumed by the hands. If hemi- plegia attends the aphasia the well hand may be used. The cerebellar symptoms usually subside spontaneously, but obstinate vertigo can some- times be relieved by the use of bromide. Post-hemiplegic tremor or athetosis is not benefited by any form of treatment. The mental symptoms cannot be treated. But the patients can be kept from excitement, from emotional strain, from mental effort, and must have cheerful surroundings and mild forms of amusement. It is better for the general welfare of educated and active persons to allow them some definite mental occupation, and if new interests cannot be started it is better to permit some apparent resumption of professional or business life, and visits of former associates, than to allow them to drift into a state of mental depression over their apparently useless condition. Cheerful companionship, entertaining reading by a good TMEATM.ENT. 513 reader, games, the dictation of correspondence, and the general inter- ests of lif(^ may afford sufficient occupation. And eventually travel may be allowed. It is well for a person who has had one attack to avoid extremes of climate and high altitudes. A winter in a dry, mild climate like that of Bermuda, Nassau, California, the Nile, or the Riviera, and a summer in the mountains or by the sea, not exposed to great heat, will prolong life. 33 CHAPTER XXVIII. THE CEREBRAL ATROPHIES OF CHILDHOOD. Infantile Hemiplegia, Diplegia, Feeble-raindedness, Imbecility, Idiocy, Blindness, Deaf-mutism, Epilepsy. MALDEVELOPMENT OF THE BRAIN. The brain may be injured or diseased in foetal life, or at the time of birth, or during infancy, and in consequence may fail to develop in a normal manner. When this occurs a condition of cerebral atrophy is found after death. The atrophy may be limited in its extent to a few convolutions, or may involve one lobe of the brain, or may affect the entire brain in greater or less degree. The symptoms produced by cerebral atrophy are numerous, but patients are easily assigned to one of three groups of cases, in accordance with the chief feature presented, though in some patients the symptoms of all the groups are found. Clinical Groups. — The first group of cases is characterized by spastic paralysis, which may be hemiplegic or diplegic in type. In the diplegic form both legs may be affected, or both arms as well as both legs. The paralysis is often accompanied by aphasia and by athetosis, and not infrequently by sensory defects, by imbecility, and by epilepsy. The second group of cases is characterized by mental defects of greater or less degree. These may vary from absolute idiocy to feeble-mindedness, or the patient may show merely some peculiarity of conduct, indicating a lack of the highest powers of attention, and of reasoning, and of self-control. Epilepsy may develop in these cases. The third group of cases is characterized by sensory defects : either hemianopsia, or total blindness, or deafness, and consequent mutism. In these cases also imbecility and epilepsy may develop. Frequency. — Cerebral atrophy is not rare. I have records of 400 cases. Of these 274 belonged to the first group, 219 having hemi- plegia, and 55 diplegia. Among these patients 100 were aphasic; 89 had athetosis, and 90 had epilepsy. In 120 some degree of mental defect was apparent. To the second group 111 patients could be assigned, as the mental defects were more marked than other symp- toms. Sixty of the second group were epileptics. To the third group 15 patients could be assigned, 7 being blind, 3 having hemianopsia, and 5 being totally deaf from birth. Six of these patients were epileptics, but none was imbecile. Etiology. — In some cases prenatal influences are responsible for the maldevelopment of the brain. Osier reports a case of hemorrhage 514 PATHOLOGY. 515 found lu ^le brain of a foetus, the mother having died of typhoid fever at the sixth month of pregnancy. Cotard has reported a similar condition, the mother having received an injury. Any severe disease in the mother may affect the development of the foetal brain. Syphilis in either parent may prevent a proper evolution of the child's nervous system. Sachs holds that the frequency with which infants of neurotic, epileptic, alcoholic, and insane parents present cerebral defects is proof that prenatal influences are potent causes of cerebral palsy and idiocy. It has been thought that cerebral thrombosis and encephalitis may occur in the foetus. Many cases date from birth. In these some evidence of traumatism can usually be obtained. Thus in 177 of my 400 cases there was a history of difficult labor, either a long protracted labor, or a malposi- tion requiring manual delivery, or instrumental delivery. In several cases labor was premature, and the child was kept alive with difficulty. In two cases the patient was a twin. In many cases the child was born asphyxiated. In all these cases it is probable that meningeal or cere- bral hemorrhage occurred during delivery, and that the pressure upon the brain or some injury to it prevented a normal development. In some cases the symptoms appear suddenly in a normal infant during the early years of life, the first three years being the period of greatest ability. Among my patients eighty-two developed symptoms during the first year, fifty-eight during the second year, twenty-eight during the third year, the remainder later. Some cases are traumatic. Twenty-two of my patients had suffered from severe falls during in- fancy. Other cases were due to cerebral hemorrhage, embolism, or thrombosis, or to an acute encephalitis. Any of these conditions may occur as a complication of the infectious diseases of childhood, and hence cerebral palsies or idiocy m.ay date from an attack of measles, diphtheria, whooping-cough, cerebro-spinal meningitis, pneumonia, scar- let fever, or typhoid fever. There was a history of one of these dis- eases in twenty of my patients. Meningitis and hydrocephalus are common causes. In a great many cases the occurrence of a convulsion is the cause assigned ; but whether the convulsion is the first symptom of the lesion, or whether in a convulsion some vascular rupture causes a lesion of the brain, is never a matter of certainty. Forty per cent, of my patients had an initial convulsion. There are also cases in which no cause can be found. An arrest of development of the brain may occur at any time during the first twenty years of life, and then produce symptoms. Pathology. — Records of the pathological condition found in all the three groups of cases described have been compiled, collections of cases with autopsies having been made by numerous writers. The lesions found are various in kind, in origin, and in situation, but a careful study elicits two conclusions : First the difference in the clinical types is due to the varying situation of the lesion rather than to its varying nature ; secondly, tliat the various processes of disease have, as a fairly uniform result, a condition of atrophy with sclerosis of the brain, which we may terra sclerotic atrophy. 516 THE CEBEBRAL ATEOPHIES OF CHILDHOOD. In the first clinical type the sclerotic atrophy involves the motor area of the brain — i. e., the central convolutions bordering the fissure of Rolando and the cortex of their immediate vicinity, and involves also the motor tract arising from this part of the cortex, and usually the basal ganglia as well. (See Fig. 226.) Sometimes the lesion is confined to the basal ganglia. In the second type the sclerotic atrophy involves the anterior portion of the brain, and sometimes the entire Fig. 226. Sclerotic atrophy of the cortex supplied by the middle cerebral artery in a case of diplegia, idiocy, and epilepsy. Both hemispheres were affected. (Peterson.) hemisphere to a greater or less extent. (See Fig. 227.) In the third type the sclerotic atrophy involves the posterior and lateral parts of the hemispheres. It is not surprising that the variation in the situation should pro- duce varying symptoms in view of the facts of the localization of brain- functions already stated. That there should be a limitation of the sclerotic atrophy to certain lobes or regions — to the frontal, or central, or occipital, or parieto-temporal regions — in various cases, has led to the conclusion that the origin of the disease lay in some interference with the blood supply of the part affected, since it has often been evi- dent that the atrophy was limited to the region nourished by blood reaching it through one arterial trunk. And this theory has been rightly supposed to explain the pathogenesis of these cases. Whether thrombosis or hemorrhage has occurred is not always easy to determine. It has been stated that the various processes of disease have, as a uniform result, a condition of sclerotic atrophy. This fact is borne out by the following resume of pathological findings in 343 cases which have been gathered from the records of Kundrat, Audry, Wallenberg, Osier, Wilmarth, Fere, Henoch, Hirt, Fowler, Schultze, Sachs, Rich- ardiere, Bourneville, Fisher, and from recent American and foreign journals. The conditions found were as follows : MALDEFELOFMENT OF THE BBAIN. 517 Porencephalus, a localized atrophy or agenesis, leaving a cavity in the cerebi^l hemisphere, which may be deep enough to open into the ventricle, 132 cases. Figs. 228 and 229 show this condition. Fig. 227. Congenital maldevelopment of the right hemisphere of the brain, with sclerotic atrophy. (Bailey.) Sclerotic atrophy, an atrophic condition of the brain with an increase of connective tissue and disappearance of the nervous elements ; affect- ing both hemispheres, or one only, or a part of one only, or limited to small areas in various parts, 97 cases. This is the terminal result of encephalitis (q. v.) and often results from maldevelopment from unknown causes acting on the foetus. Maldevelopment and apparent atrophic condition of the minute structures of the hemisphere, chiefly cortical, the cells resembling those of a newborn child, but with no apparent gross defects in the brain, 32 cases. Atrophy, consequent upon the condition of softening produced by embolism or thrombosis, and limited in extent to certain arterial dis- tricts of the brain, 23 cases. Fig. 226 shows this condition. Meningo- encephalitis, a condition shown by thickening and adhesion between the pia and the brain, with destruction of the cerebral cells and atrophy of the cortex, 21 cases. Cysts lying on the brain and producing atrophy by pressure, or associated with atrophy due to the original lesion of which the cyst 5i8 TEE CEBEBBAL ATROPHIES OF CIIILDHOOD. remains as a trace, 14 cases. Figs. 231 and 232 show this condi- tion. Hemorrhage on or in the brain, as shown by the remains of a clot, or by hseraatin staining of a cyst, of the pia, or of the sclerotic tissue, 18 cases. Fig. 228. PoTcncephalus. (Lloyd.) Fig. 229. Frontal section through a porencephalic brain. The left hemisphere is normal. The right hemi- sphere is atrophied in lo/o, and has a cavity in the motor region which extends downward Into the ventricle. The basal ganglia are atrophied. This condition Is always congenital. (Shattenberg.) Hydrocephalus with extreme dilatation of the ventricles, so that the brain tissue is reduced to a mere wall about the cavity, five cases. MALDEVELOPMENT OF THE BRAIN. 519 Hydrocephalus is an accumulation of serum in large amount in the cranium. If the serum collects in the meninges it compresses the brain and is associated with atrophy. This is termed external hydro- FiG. 230. t/ppercavofy' Fossa Sylvil J-ovrer- pari of Ccuvvfy ^ -Lower part t| ofCcwUy Lower horn, ofyintricie Extensive bilateral porencephalus. The cavity within the brain communicated with the external surface at A. (Richter, Arch. f. Psych., xxxii.) Fig. 231. Superior surface of a brain of a congenital iiuljccile who had hcmii>lcgia and epilepsy. The entire right liemispherc is atroi)hiod. C is the arachnoid, which was thickened and fonucd thq wall of a cystic cavity in the hemisphere. (Fcrraro.) 520 THE CEBEBBAL ATBOPEIES OF CHILDHOOD. cephalus. It is a rare condition, secondary to meningitis or to mal- development of the brain, and not infrequently the fluid is poured out to fill the space left between an atrophic brain and the cranium. The common form of hydrocephalus is inter aal hydrooephalus, that is a dis- tention of the ventricles of the brain by an exudation of serum into them. Internal hydrocephalus may be acute or chronic. The acute form is always due to meningitis, either simple or tuberculous (see Chapter XLI,). The chronic form may be secondary to a mild meningitis or ependymitis, the original disease resulting in an adhesion within the ventricles, which closes one of the foramina and prevents the free Fig. 232. Superior surface of the brain of a congenital imbecile. The arachnoid being removed, the poren- cephalic cavity is displayed. The cortex is wholly defective over the upper frontal and parietal lobes, and the cavity in the hemisphere opens into the lateral ventricle at D, in which the choroid plexus E is seen. (Ferraro.) flow of serum from the lateral ventricles outward through the aqueduct of Sylvius and the fourth ventricle and the foramen of Magendie into the space about the brain. It may also be secondary to any disease in or outside of the brain which compresses the base and obstructs the free exit of serum from the ventricles. Thus tumors of the brain usually cause hydrocephalus. It may also be secondary to syphilitic disease in the brain or meninges, and many congenital cases are due to inherited syphilis, with closure of the aqueduct or foramina. Chronic internal hydrocephalus is in many cases a congenital condition, and is MALDEVELOPMENT OF THE BHAIN. 521 very freqi^gntly associated with rickets. It is considered a primary condition in cases wliere no cause can be ascertained, and it is in these cases that the most extreme distention of the brain is observed. The fluid appears to be secreted by the ependyma of the lateral ventricles, and may vary in amount from a few ounces up to five or six pints. It is identical with the cerebrospinal fluid. It gradually distends the ventricles, dilates the passages between them, and compresses the brain. .The brain becomes anaemic and atrophies, so that finally a mere, thin capsule of brain tissue is left about the ventricles. The gradual com- pression and atrophy of the brain interfere with the function of the tracts passing through it, and hence gives rise to spastic diplegia, to Fig. 233. Congenital absence of one-half of the cerebellum. Atrophy of the opposite olivary body and of the superficial transverse fibres of the pons. (Kindness of Dr. Oliver S. Strong.) sensory defects, to mental deterioration, and imbecility. The disten- tion of the brain causes a distention of the skull, giving rise to the characteristic hydrocephalic skull, in which the sutures are separated, the frontal and parietal bosses bulge outward, and the head is of enor- mous size. The softening of the bones may be the earliest sign of a beginning hydrocephalus. Holt states that the average head at birth measures 14 inches in circumference, and at one year measures 18 to 19 inches. A rapid enlargement beyond these limits during infancy is suggestive of hydrocephalus. The enlargement may progress slowly for several years, or may cease at any time, spontaneous arrest of the 52^ THE cxsi:^nAL ATHovmns OV cmLDSOOB, condition being observed in about six per cent, of the patients. Kinety- four per cent, of these patients die before the age of seven years. Attempts to drain the ventricles do not result in an arrest of the secre- tion, and hence are almost uniformly unsuccessful.' Unilateral hydrocephalus was found in one case in the pathological records. It is evident that the common condition in all these cases was an atrophy of the brain. Table XII. Measurements OF THE Skull. (Peterson.) Average of normal Limits of Physiological male skull. variation. Circumference .... 52.0 cm. 48.5 to 57.4 cm. Volume (rou.2:h approximation) 1500.0 " 1201.0 " 1751.0 " Nasooccipital arc 32.0 " 28.0 " 38.0 " Nasobregmatic arc . 12.5 " 10.9 " 14.9 " Bregmato-Iambdoid arc 12.5 " 9.1 " 14.4 " Binauricular arc 32.0 " 28.4 " 35.0 " Antero-posterior diameter . 17.7 " 16.5 " 19.0 " Greatest transverse diameter 14.6 " 13.0 " 16.5 " Length-breadth index 82.2 " 76.1 " 87.0 " Binauricular diameter 12.4 " 10.9 " 13.9 " Facial length .... 12.37 " 10.5 " 14.4 " The maldevelopment of the brain is usually accompanied by a state of microcephalus, all the measurements of the skull being reduced as compared with a normal head. In many such children great asym- metry of the skull is found. In other cases the head is unusually large. A large head may contain a microcephalic brain, the space about it being filled with fluid. The asymmetry becomes more notice- able as the child grows up. The table on page 518 shows the measure- ments of the average normal skull and the limits of physiological varia- tion in an adult. In cases of cerebral atrophy these limits are passed. 1. Symptoms of Cerebral Spastic Paralysis. — The first group of cases present symptoms of paralysis. This may be unilateral or bilateral. Hemiplegia. — In the cases which develop a unilateral paralysis the characteristic features of hemiplegia as seen in adults are evident. This may date from birth or it may develop later. If it develops in infancy it usually begins with a series of convulsions, which are often general, but may be unilateral, and are attended by high fever, 102° to 105° F., and nausea, vomiting, and headache, with delirium. The onset is followed by a period of unconsciousness of varying duration, from an hour to two or three days, the average being twelve hours. The child may die in such an attack. But there is usually a gradual improvement in the paralysis after the active manifestations of the onset have subsided ; and, finally, a stationary condition remains, in which the face is but slightly affected in its voluntary or automatic movements ; the speech is acquired very slowly, or is slowly regained, if it had been lost ; the arm is quite seriously paralyzed, the fingers being stiff* and awkward, and sometimes being in constant slow involuntary motion which is J See Brain Surgery, Chapter VII. MALDEVELOFMENT OF THE BBAIN. 523 called athetosis ; the leg is weak, and the knee and ankle are held rigid, so that the child limps in walking, and sometimes has a club- foot. A slow improvement is the rule. As time goes on the child learns to help himself in many ways, and it is possible for the gradual Fig. 234. Fig. 235. Hemiplegia with contractures. The patient had suf- fered since the age of two years. (Curschmann.) Spastic hemiplegia from angiolithic sarcoma of the brain, the symptoms dating from five years of age. (Der- cum.) growth of the limbs to be attended by increasing power. In all cases the paralyzed limbs are found to be affected in their growth and de- velopment, so that they are smaller, colder, stiffer, and weaker than the others ; they are often blue and cyanotic. The reflexes are exag- gerated on the paralyzed side. The electrical reactions are not quali- tatively changed. The sensation is normal. This condition remains through life as a permanent defect, and although the division of con- tractured muscles or tendons, and the application of ingenious apparatus, may correct deformities and make the paralyzed parts fairly useful, and although the application of electricity to the muscles chiefly affected 624 THM CEHEBMAL ATMOPEIES OF CHILDHOOD. may increase their nutrition, and thus prevent contractures which come from the unbalanced strain between the various muscles, yet a perfect recovery does not occur. The degree of spontaneous recovery possible in any case cannot be determined for several years. But in many cases while some trace of the hemiplegia remains there is so much improve- ment that the patient is capable of earning his living. I have several such patients who, though unable to use the hand freely, and always obliged to limp in walking, are able to occupy places as clerks or mes- sengers, or have been able to go through college, and even to study a profession. It is only in the cases in which weak-mindedness and epi- lepsy occur that the condition is a hopeless one. But even when the final state is not one of helplessness these patients are extremely liable to other nervous diseases. Thus several such persons under my, • observation have suffered from chronic neurasthenia and hysteria. One of them developed attacks of mania of short duration, but frequent recurrence, not unlike the psychical epileptic equivalent, and, though an educated lawyer, has required asylum care for several years past. ^ Spastic Rigidity; Athetosis. — The especial characteristics of this form of hemiplegia are the spastic rigidity of the affected limbs with marked contracture, and the athetosis. The contractures lead to vari- ous deformities about the ankle and wrist, especially to club-foot. Fig. 234 shows these conditions. Athetosis may occur when the hand or foot are quiet. When the child attempts to move the hand or leg un- expectedly involuntary slow motions occur which prevent the intended result, or the limb is thrown into a sudden state of rigid immobility. Athetoid movements on the paralyzed side are set up by any voluntary act on the well side. These athetoid motions begin soon after the onset as a rule. In one case of birth palsy they did not appear until the fourth year and increased until the seventh year, remaining when I saw the child at the age of twelve years. If the athetoid movements affect the face and tongue, grimaces are constant and speech is interfered with. Athetosis has been present in 30 per cent, of my cases. (See Fig. 225.) In some cases the athetosis is the most marked symptom. Thus in the case of a boy, aged eleven years, who had been born asphyxiated after a face presentation, and was revived with difficulty, a peculiar type of athetosis had been present since the age of ten months. The motion was limited to the left side and was present in the face, in the hand, and in the foot. It was not constant in this case, nor was it started by any effort. But it came in attacks, twelve or more in a day, each lasting several minutes. The motion was a quick, involuntary athetoid movement, which he could not control. It was not attended by a loss of consciousness, or by a sense of alarm, or by any numbness. He had no paralysis whatever, no nystagmus, no disturbance of vision, no headaches, and was bright and active mentally. The attacks were first noted during sleep, but as he grew older became more frequent in the daytime, and no treatment had any effect upon them. In another case, also dating from birth, the athetosis affected the MALDEVELOPMENT OF THE BBAIN. 525 entire body, so that any effort was attended by contortions of the face and by peduliar crowing sounds which interfered with talking and eat- ing ; by constant violent movements of the arms and legs, so that no voluntary act could be performed, and walking was impossible. The boy, at the age often years, was perfectly intelligent and not paralyzed, but was in a constant state of active motion without any volitional power. It was impossible for him' to sit upright in a chair or to stand. He could only lie in bed supported by pillows, and was even fed with difficulty. The athetosis ceased during sleep. I have recently seen a second similar case equally distressing in a young man, aged eighteen years, whose entire life had been spent in bed. Choreic movements more sudden, rapid and violent than athetosis may appear in the paralyzed limbs and remain as a permanent symp- tom. Such a condition was noticed in twenty-one of my two hundred and seventy -four cases. In one of my cases there was a total paralysis of the tongue with right hemiplegia, and the child at the age of twelve years was still unable to articulate. Food was pushed back into the throat by the finger, and then swallowed. This child had learned to read, but was weak mentally and epileptic. Aphasia is a common accompaniment of right hemiplegia. In my 274 patients, 130 had right hemiplegia, and in 100 of these aphasia was present. It is usually of the motor type. The children under- stand what is said to them, can acquire a knowledge of the names of objects, and may learn to read, but cannot be taught to talk, or if they acquire language, do so very slowly and imperfectly. I have known children of the age of twelve years to talk like a child of three years. There is, however, as a rule, gradual recovery of speech, and it seems likely that in these cases the right hemisphere takes the place of the left in developing this function. This is often aided by training the child to write with the left hand. Diplegia — Little's Disease. — In many cases dating from birth a con- dition of bilateral paralysis of arms and legs, or of the legs alone, is found as soon as the child is born. This is termed diplegia. It becomes more apparent after the first few weeks when attempts at voluntary motion are made. It is attended by a rigidity of the muscles which is noticed in the legs, and there is an increase of the reflexes. The infant may have difficulty in sucking and may have to be fed with a spoon. Diplegia may however develop slowly, and not become apparent for some months. In some cases, especially after the infectious diseases, it may not appear till the age of three years. In these cases as the baby grows its body is found to be stiff, especially in its back and legs, the latter tending to be adducted and extended. This is noticed in adjusting the diapers. Later when attempts at walking are made the adduction and inward rotation of the thighs may cause an overlapping of the knees and feet, there may develop a pointing of the toes which . gradually becomes a fixed equino-varus, so that the heels can not be 526 TEE CEBEBRAL ATEOPEIES OF CHILDHOOD. put on the ground, and the inability of the child to balance becomes more noticeable. Any attempt at standing increases the stiffness of the back and legs. Sometimes the back is so rigid that the child can- not sit up on a chair, but has to be kept in a lying position. As the child grows the condition becomes more noticeable, and sometimes the child is bedridden and never learns to walk. In other cases the child Fig. 236. Fig. 237. Marked spastic paraplegia ; walking or stand- ing alone impossible. (Dercum.) Spastic paraplegia ; crossed-legged progression. (Dercum.) acquires a spastic gait and helps itself by its hands, as they are often not affected, and so is able to get about. Others improve slowly but steadily, and finally walk without a cane, but stif&y. In the severe cases the arms are rigidly adducted and flexed, and the hands are coutractured and useless. (See Fig. 236.) In such a condition athetoid movements are universal. The tendon reflexes are always exaggerated, the muscles of the limbs are poorly developed, but there are no bed-sores, and there is no loss of control of the sphincters. There are no sensory symptoms. These children frequently have mental defects, and epilepsy is likely to develop. Strabismus is a not uncommon symptom, and difficulty of articula- tion, or of swallowing may be present. Oppenheim has called attentiou MALDEVELOPMENT OF THE BRAIN. 527 to the timidity of these children and to the fact that sudden noises cause them to ju'fiap or even throw them into convulsions. In a few of my cases the symptoms have been exclusively cerebellar in type. The child has been unable to sit up, to balance itself on its feet, or to learn to walk. In one such case dating from birth, the child was still absolutely helpless at the age of nine years, although intelli- gent and not paralyzed. In another the same condition was present at the age of five years, but was attended by feeble-mindedness. This child died of measles, and Fig. 233 shows the unilateral atrophy of the cerebellum found. In many of the cases difficulty in learning to talk has been noticed, the child acquires words slowly, and sometimes even at the age of six years is unable to frame a sentence or to speak clearly. Epilepsy. — In the majority of cases both of hemiplegia and of diplegia epileptic attacks begin soon after the paralysis ; though some- times the interval is several months or even several years. Two-thirds of the cases eventually develop epilepsy. The fits in organic epilepsy are more frequent and severe than in idiopathic epilepsy. I have one patient who has had as many as twenty convulsions daily for weeks at a time. These fits do not de- stroy life, but of course render life a burden. They are different from the fits of idiopathic epilepsy in being of a character which indicates local irritation of the cerebral cortex. (Seepage 91.) They usually begin in one hand with closure of the fist, or pronation of the hand, attended by a sensation of tingling ; the contraction of the muscles is clonic and the spasm extends up to the elbow, and finally the shoulder- joint is thrown into motion, the entire arm moving in the convulsion. The spasm then extends either to the neck and face, which is drawn to the paralyzed side, or it spreads down the trunk and involves the leg, which shakes or is violently extended with clonic spasms of the toes. Such a spasm may last for a few minutes, and then gradually cease, leaving the muscles paralyzed and the side numb for several hours. Or it may extend to the other side of the body, in which case, as a rule, consciousness is lost for some minutes, the patient falls, bites the tongue, froths at the mouth, and subsequent to the attack sleeps heavily for an hour or more, waking dazed, and often unable to talk or to think for some hours. In many cases the attack begins in the leg and extends to arm and face. In other cases the reverse is the order of extension of the spasm ; the point of greatest irritation, in the cor- tex being indicated by the part of the body first aifected. In some cases head and eyes turn toward the paralyzed side at the beginning of the fit. In other cases the epileptic attack begins with sensory rather than with motor symptoms, the initial numbness and tingling always accom- panies and may precede the local spasm. But sometimes it is a visual aura, a light, or color, or figure seen with which the attack begins. In other cases it is a sound, a noise, or a bell, or a voice which is heard. In other cases it is an odor or a taste which is perceived. In all these 528 TRE CEBEBRAL ATROPHIES OF CHILDHOOD. conditions a local spasm or a general convulsion follows the aura, indi- cating that the irritation of the brain which has started in a sensory areaj the chief seat of disease, has radiated outward and has invaded the motor centres. (See page 91.) Mental aurae are also observed, consisting of a dreamy state, a sort of second consciousness, when the mind seems to watch, its own processes without being able to modify them. The mental aura may be a sudden fear, or fright, or distress, pleasant ideas being rarely present, though not unknown. Attacks of an active maniacal kind or of double consciousness may occur without a convulsion ; these are the so-called " psychical epileptic equivalents " of an attack. All these forms of attack are of very great interest, as they indicate quite faithfully the area of brain in which the disease is located, and form the guide to the surgeon in cases which are open to operation. II. Symptoms of Mental Defects. — The second group of cases presents mental defects rather than physical symptoms. There may be an entire lack of mental development from the outset. This is termed congenital idiocy, and is due to congenital defects of the brain or to some arrest of growth soon after |)irth. There may be an acquired idiocy, from some disease occurring during infancy or childhood, such as injury of the brain from trauma, or after convulsions, or due to some of the infectious diseases. Idiots have been classified as micro- cephalic, hydrocephalic, paralytic, epileptic, meningitic, or amaurotic, according to the predominant cause. A maldevelopment of the thyroid gland leading to cretinism and mental weakness is not attended by brain lesions, and should not be classed with idiocy. It may be cured by thyroid treatment. Idiots are wholly lacking in the power of acquiring ideas or concepts, in the power of fixing the attention and in the capacity of remembering, and cannot be trained to keep themselves clean. The sensory powers are defective in many, but they are in constant motion, a rhythmical swaying being often observed. If the degree of mental defect is less marked the mental state is described as one of imbecility. An imbecile can acquire simple ideas, can recognize persons and objects, can say a few words, but is incapable of acquiring any of the higher conceptions which are the basis of knowl- edge and of conduct. Imbeciles are usually helpless if left alone, and are often ugly in disposition, destructive and filthy. The difference between an idiot and an imbecile is one of degree only, and no sharp line can be drawn between them. There are other patients with mental defects, of a higher type of mind than imbeciles, but nevertheless abnormal. These are classed as weak-minded. The child may be slow in learning to walk ; may seem unable to fix its attention upon anything continuously ; may be exceed- ingly active, in constant motion — the activity being, however, aimless; may throw things about, or tear things up, or put everything into its mouth ; may be very difficult to manage because of its inability to retain and combine impressions with sufficient power to reason upon them. MALDEVELOPMENT OF THE BBAIN. 529 and many therefore be incapable of appreciating the meaning of punish- ment, if th^ be inflicted. Such children may have good powers of perception, may recognize persons and objects, show pleasure at bright colors, or music, or caresses, but fail to show evidence of thought in the sense of reasoning power, judgment, or self-control. Some patients constantly drool at the mouth, cannot be taught cleanly habits, and are manifestly imbecile. Other patients are quite bright in many direc- tions, may even be precocious, show talents in music, or drawing, or fondness for mathematics, designing, and languages, yet are apparently unable to appreciate moral ideas, cannot be taught to tell the truth, are cruel and bad, will not control any of their impulses, and so are the dis- tress and despair of parents and teachers.^ It is those mental qualities which are the product of the highest evolution that have failed to develop in this class of cases. The final result is that they have to be taken care of all their lives, either at home or by attendants, being incapable of supporting themselves or directing their conduct. Many of them have epilepsy. III. Sensory Defects. — The third group of cases show sensory defects, usually blindness or deafness, although a loss of taste and smell is occasionally observed. These symptoms are likely to escape obser- vation until the child reaches the age of two or three years. Patients belonging to this class may present no motor • or mental defects, though they may be the subject of epilepsy. On several occasions I have been consulted by parents who have brought children to me sup- posedly the subjects of epilepsy in whom I have detected one or more of these sensory defects. Thus, in three cases a condition of homony- mous hemianopsia was discovered which was undoubtedly congenital, each child having grown up without any appreciation of the defect of vision. In all of these cases there was a corresponding flattening of the skull over the occipital lobe, and all the patients were suffering from epilepsy. Moeli ^ has described three such cases in which poren- cephalus was found in the occipital lobe, and Henschen ^ and Freund * have also observed such cases. In several patients whom I have seen total blindness has been found. It is not always easy to determine whether this is due to a primary atrophy, or lack of development in the optic nerve, or whether it is due to atrophy of the occipital convo- lutions. Both conditions have been found post-mortem in many cases. If the condition is due to atrophy of the optic nerve an ophthalmo- scopic examination will reveal this condition. There are other cases in which the patients are deaf. If a child has never heard, or lost its hearing before it has learned to talk, it becomes mute. The majority of cases of deaf-mutism are undoubtedly due to lesions in the ears or to lesions of the auditory nerves upon the base of the l^rain. There are a few cases, however, on record in which an atrophy of the temporal convolutions has been attended by deafness. 'See F. Peterson. Idiots Savants. Popular Science Monthly, Dec, 1896. ^Archiv. fiir Psych., xxii., 2. ^ Kensclien, Path, des Gehirns, cases 32 and 35. * Freund, Wien. med. Woch., 1888, No. 32, 34 530 THE CESEBRAL ATROPHIES OF CHILDBOOD. ^ Loss of smell and taste have been produced by atrophic conditions of the uncinate convolutions upon the base of the brain. These are usually traceable to basilar meningitis. They very often escape obser- vation, though in any case where the child is known to put all sorts of objects into the mouth and be willing to eat offensive things, a sus- picion of a loss of taste is awakened. A localized atrophy in the convolutions of the brain may also occur from disuse. Thus, in a number of cases recorded where patients have lost the sense of hearing and of sight in early life, as for example, from basilar meningitis, cerebro-spinal meningitis, or lesions of the ear, or eye, the function of the corresponding portion of the brain has not been awakened, and as a result of absolute disuse an atrophy of these con- volutions has been discovered when the patients have died late in life. This was the condition found in the brain of Laura Bridgman, the temporal and occipital convolutions being rudimentary in size, while the sensorimotor convolutions about the fissure of Rolando were enor- mously developed. This girl lost sight and hearing at the age of two years from a basilar meningitis, but developed remarkable powers of observation through the sense of touch ; and of expression through motor action. In two other cases reported by foreign observers the same condition has been found. Hence the localized atrophy due to failure of development in limited areas of the brain after disuse of the corresponding organs is proven in man as well as established by the experiments of von Gudden. Epilepsy is a common condition in these cases of sensory defect. The attack not infrequently begins by an aura of a sensory nature. It may be limited to an attack of petit mal, or it may go on to an attack of grand mal. If the atrophy of the brain is unilateral the epileptic attack may be of a Jacksonian nature. Prognosis. — The prognosis in cerebral atrophy is most unfavorable. The underlying pathological condition is permanent, even though it is not progressive, and there is no hope of improvement or of recovery. The paralysis, the mental defects, and the sensory defects cannot pos- sibly be relieved. The epilepsy may be held in check to some extent by the use of bromides, the number of fits being reduced, their severity somewhat mitigated. But they cannot be stopped. Treatment. — The treatment of an acute attack of apoplexy in childhood does not differ in any way from that occurring in the adult. The same care must be taken in diagnosis, and the same measures adopted as in the conditions of hemorrhage or thrombosis already considered. The treatment of the condition of infantile paralysis and of idiocy consists in rendering the life of these patients as happy and comfort- able by proper surroundings and agreeable attendants as possible. This can best be done by securing for them a country home away from ex- citement and away from the family. Parents too often expend more care, attention, and money upon these chronic invalids than upon their healthy children who suffer in consequence. It should be made clear to MALDEVELOPMENT OF THE BRAIN. 531 them that in doing so they accomplish little and are unjust to those who will bette/ repay their care. Education by those who are skilled in the training of the weak-minded accomplishes something, and is to be recom- mended if parents can afford the expense. The treatment of the epi- leptic condition is the same as that of ordinary epilepsy by the use of bromides ; but in these cases the effect of the drug soon wears off", and I prefer not to use any medical treatment. Surgical Treatment. — When a patient belonging to one of these clinical types is presented to the neurologist and the question is asked, can surgical treatment benefit him? it is evident that a serious problem is opened. In these cases the disease is at a standstill and does not threaten life, and yet is hopeless from the medical standpoint. Surgi- cal treatment is not free from danger, but if it offers any relief almost any risk is justifiable. The brain is still capable of great develop- ment in infancy and youth. Can such development be aided by an operation? In many cases the epileptic attacks are of such frequent occurrence that any risk might well be taken if they could be surely stopped. Any solution of the problem of operative treatment must be based upon two considerations : first, the pathology of the cases ; secondly, the results of experience when such operations have been done. First, from the records of the pathology already given in 343 cases it is evident that a state of cerebral atrophy was present in the majority which nothing could remove, and it is apparent that many of the con- ditions were of such a nature as to be wholly unaflFected by any opera- tive interference. A porencephalic cavity filled with cerebrospinal fluid is not likely to be benefited by any enlargement of the intra- cranial space or by the abstraction of the fluid. In several cases operated upon the withdrawal of this fluid has been followed by sudden collapse and death. ^ On the other hand, there are conditions, such as maldevelopment of the cortex without gross lesion, in which it is pos- sible that anything which may stimulate latent powers of growth, or may remove those influences which interfere with development, might result in improvement. It is to be remembered that the brain is cap- able of growth and development until the age of fourteen years, if not longer, and, granting that a stimulus to its growth may be given dur- ing childhood, the arrest of development in many cases might perhaps have been prevented. And there are cases of cysts lying upon the cortex whose removal may relieve pressure and allow a proper growth. The study of the pathological conditions, therefore, does not abso- lutely contraindicate operative interference, although it makes it clear that the lesion in the majority of the cases is one that cannot be improved by any means. If we admit that porencephalus, atrophy from vascular lesion, meningo-encephalitis, and hydrocephalus are incurable, and that hemorrhages cannot be diagnosed early enough to ^ Cases of Bullard, Boston Medical and Surgical Journal, February 16, 1888. Ham- mond, New York Medical Journal, August 12, 1890. One of my own. 532 TEE CEREBBAL ATROPHIES OF CHILDHOOB. warrant the removal of the clot before it has caused atrophy from pres- sure, we have 193 cases out of 343 in which operation would have been futile. This leaves 150 cases of sclerotic atrophy, maldevelop- ment of the cortex, and cysts, in which it is barely possible that an operation, if it relieved pressure or stimulated brain growth might have had some effect. It is unfortunate that we cannot make an exact patho- logical diagnosis from the clinical symptoms. Therefore, one cannot affirm that in any particular case a removable cyst is present or a con- dition of pressure which may be relieved. The operation must in all cases, therefore, be exploratory, and this should always be understood by all concerned. There is one further fact which requires a word of explanation. In many of these cases inspection shows a very decided irregularity of the skull. It has been thought by some observers, especially Lannelongue, in France, that an early closure of the sutures and fontanelles in infancy may prevent a proper expansion of the skull, and thus pro- duce a pressure on the brain, preventing its growth. These authors regard the atrophy as secondary to compression. This view I consider erroneous. The skull does not unite as long as there is any pressure within it. It is the cerebral defect due to disease which prevents the brain from developing and arrests its normal growth and internal pressure. The bones close because the natural expansion has ceased. The skull does not cause pressure on the brain. This is evident in cases operated upon, for no signs of compression of the brain have been found in these cases. The theory, therefore, upon which opera- tions of craniectomy have been done is not borne out by the facts. Secondly. The results of experience of surgeons in the treatment of these cases by operation has not been very favorable. The operation of craniectomy has been done by many surgeons during the past few years, and the results have been reported by several of them, notably by Lannelongue, Keen, Bullard, Oppenheim, Frank, Hammond, Horsley, Agnew, and McBurney. Many other operators have reported single cases, and my own experience in connection with McBurney is extensive.^ Lannelongue reported, in 1891, that he had operated on 25 patients, chiefly microcephalic or epileptic children, with but one fatal result, and that there was an improvement in many cases. Lannelongue's operation is to make a long, curved incision through the scalp and skull from the temporal ridge backward for six to ten inches, and crack the side of the skull outward, leaving an interspace of an inch or more in width which fills up with fibrous connective tissue. Thus he relieves the supposed compression of the brain. It is unfortunate that these cases were re- ported soon after the operation, so that, although the surgical result was not fatal, we know nothing of the effects of the operation on their brain development. In a recent article by one of Lannelongue's assistants the intimation is given that little was accomplished. ' For an analysis of these operations see the Medical Record, January 23, 1892 ; Brain Surgery, Chapter III, MALDEVELOPMENT OF THE BRAIN. 533 In my own collection of cases, 50 in number, including 15 in my own experience, the result cannot be said to be very encouraging. Of 50 cases 16 died during or soon after the operation. Death was due in 11 cases to shock from sudden evacuation of fluid from a large cavity in the brain. Thus, in one case operated upon for me by Mc- Burney, in which the child, a girl of eleven years, presented only a slight degree of right hemiplegia, with severe attacks of hemiepilepsy and no imbecility or aphasia, the entire left frontal lobe was deficient, its place being occupied by a cyst. The exposure of this on opening the dura was attended by laceration of its wall, as it was adherent to the dura, and the flow of serous contents was followed by immediate collapse and death. In 10 cases reported by others a similar result occurred. In some cases death has been due to excessive hemorrhage from large brittle veins in the pia or in the walls of a porencephalic cavity. Here ligature was impossible, and compression equally so. In some cases death has been due to exhaustion following the opera- tion, which has necessarily been long. It is evident, therefore, that these operations are much more dangerous than the ordinary operation for trephining, because of the existence of pathological conditions which cannot be foreseen or provided against. In the thirty-four patients who have survived the operation a certain amount of improvement has been reported in twenty-five, and in nine no apparent result was noticed. The improvement has consisted in a relief of the paralysis, but not in its disappearance ; a marked relief from the athetoid movements, but no cessation ; an improvement in the gait ; an improvement in the mental capacity, and a change from imbe- cility to a condition of weak-mindedness, but no complete recovery or normal development of the mind ; a diminution in the frequency and a mitigation in the severity of the epileptic attacks, but no absolute cure. In several of my own cases nothing abnormal has been found in the brain at the operation, and it has been supposed that the lesion was of a microscopic character. In two of these cases the subsequent history has shown considerable improvement, which must have been wholly independent of the operation, and awakens the suspicion that such im- provement of a spontaneous kind may have been erroneously ascribed to the operation by partial observers. In three of my cases a thin organized blood clot containing fine con- nective-tissue filaments adherent to the dura in one case and to the brain and cortex in two cases was discovered. It was possible to sponge and scrape away this clot in all these cases, though some hemorrhage followed from the rupture of the new capillaries in the organized mass. These were all cases of several years' duration, hence the clot was not recent in any. In all these cases there was an apparent marked improvement. In a number of my cases cysts have been found — sometimes lying on the cortex, in the pia, and causing a depression of the cortex ; in other cases within the brain, being in part covered by the cortex; in some cases deep in the white matter, being discovered only by the exploratory needle. It has usually been pos- 534 TBE CEREBRAL ATROPHIES OF CHILDHOOD. sible to remove these cysts, the walls being carefully dissected away without rupture. It was found that if they were merely evacuated the fluid soon returned, and a second operation was necessary. This occurred in two cases. When the cyst lay deep in the white matter and could only be discovered by puncture it has not been always pos- sible to remove the wall, and in these cases they probably refilled, as no improvement followed. The best result has been obtained in the cases where a cyst could be removed as a whole. In one case a cystic mass exactly like honeycomb occupied the entire lower two-thirds of the anterior central convolution, and could not be removed entirely. In some cases thick, firm, connective-tissue scars, or porous bands have been found in the cortex, in one case as firm as cartilage, in another case calcified in part. These were probably the result of hemorrhages or of an area of softening. Their removal was attended by severe hemorrhage, in one case making it necessary to postpone twice a completion of the operation. In neither of these cases were the mental symptoms and epilepsy for which the operation was under- taken at all improved. This experience leads me to be very cautious about urging an operation in this class of cases. The dangers are many. The prospect of relief is very small. Cure cannot be promised. Improvement in a small pro- portion of the cases only is the best result which can be expected. Harvey Gushing has recently^ advocated an operation immediately after the appearance of the symptoms, especially in the cases developing at birth. He has had several successful cases, in which, within a few days of birth or of the appearance of hemiplegia, he has opened the skull by making incisions along the lines of the sutures in the parietal bone, has turned down the parietal bone, exposed the brain, found a large surface clot and has removed it. I heartily approve this operation in a case where the diagnosis of hemorrhage can be made in a positive way, but think it should be done only by a surgeon skilled in cerebral surgery. * American Journal of the Medical Sciences, July, 1905. CHAPTER XXIX. ENCEPHALITIS. Acute Hemorrhagic Cortical Encephalitis. Polio-encephalitis Superior and Inferior. Cerebellar Encephalitis. Etiology. — That an acute inflammation of the brain may occur as an independent affection has been established by Striimpell, Leichten- stein, and Oppenheim. It is probably an acute infectious disease, as it has all the characteristic signs of one. It has been known to occur in the course of scarlet fever, measles, influenza, pneumonia, erysipelas, whooping-cough, mumps, diphtheria, ulcerative endocarditis, otitis media, and septicaemia. It is not necessarily accompanied by purulent meningitis, though it may be. In most of these conditions the char- acteristic organism of the original disease has been found in the foci of inflammation in the brain. Ptomaine poisoning has been ascribed as a cause, also alcoholism and poisoning by carbon dioxide. It is still a matter of dispute whether an injury of the head not attended by frac- ture or meningitis can set up an acute inflammation of the brain. Bruises in soft tissues are rarely followed by inflammation, but may predispose an organ to the invasion of bacteria. I have seen abscess to occur under these conditions, and believe it to have resulted from such an inflammation. The disease occurs chiefly in children and young people, but may develop in adults. Varieties. — The inflammatory process is usually limited in its extent in the brain, and is not diffuse. It is sometimes only to be found by microscopic investigation. It is sometimes wholly within the domain of one arterial branch. In other cases it has been known to affect symmetrical parts of both hemispheres, and in a few cases disseminated foci of inflammation have been found. The inflammation may be found in both gray and white matter, and is not limited to either. It may affect any part of the brain. A number of different forms have been described, depending upon the location. Thus, Striimpell's first cases were chiefly cortical in the motor area, and were called acute infantile cerebral palsy, or acute hemorrhagic cortical encephalitis. Later this condition was found to occur in adults and even in old age as in a case of Mills.^ In the cases collected by Wernicke and named by him polioencepha- litis superior the lesion was located in the gray matter lining the aqueduct of Sylvius. These cases are not different from acute ophthalmoplegia. 'C. K. Mills. Review of Neurology and Psychiatry, Feb., 1907, where full refer- ences may be found. 635 536 E'NCEPEALITIB. There is a form limited to the nuclei of the motor cranial nerves of the tongue and face, termed polio-encephalitis inferior, or acute bul- bar paralysis, and causing all the symptoms of chronic bulbar paralysis (Chapter XXXIV.), but with acute onset and rapid course.^ Such cases have occurred as a complication of acute anterior poliomyelitis, in several epidemics ; see Chapter XIV. Lastly, a form limited to the cerebellum has also been described as a separate disease. Pathology. — The lesion in all these cases is the same, and the dif- ference in the symptoms is dependent entirely upon the different func- tions of the parts of the brain affected. Such a multiplication of diseases is an unfortunate weakness of authors who lack the generaliz- ing faculty. The disease has its exact homologue in acute anterior poliomyelitis, and the changes present are the same. The two diseases have been known to occur together. The changes found in these cases in the acute stage are (1) an acute hypersemia with distention of the bloodvessels, rupture of their walls, capillary hemorrhages in the tis- sues, and an emigration of leucocytes and small cells. (2) Various stages and degrees of degeneration of the neurones, both cell body, axones, and dendrites being affected, with subsequent secondary changes of wide extent. The exact microscopic changes are identical with those already described' in the chapter on anterior poliomyelitis, but are located in the cortex or bulbar nuclei. After an acute stage the hypersemia subsides, the clots or diffusely infiltrated red blood cells may be absorbed, and if the degeneration of neurones has not led to the destruction of a large number controlling any special function a gradual recovery ensues and no trace is left. In this respect the disease resembles anterior poliomyelitis, where recovery in many of the muscles at first paralyzed is the rule. But if a number of neurones in any system are destroyed there are permanent symptoms remaining whose character depends on the function of the neurones affected. Thus, if the frontal lobes are affected, imbecility results. If the motor cortex is destroyed a state of hemiplegia remains. In a case examined by Hochhaus, in which cerebral hemiplegia developed in the course of measles in an infant, a cortical encephalitis with some local- ized meningitis was found. If the ocular motor nuclei are affected strabismus and paralysis of ocular movement results. If the facial and lingual nuclei are paralyzed some defect in speech, swallowing, and facial expression remains. If the cerebellum is affected a permanent defect of co5rdination and of gait ensues. If the cord is affected with the brain, some type of poliomyelitis attends the encephalitis. The permanent result of the pathological process is the formation of small or large areas of sclerotic tissue or of small cysts. The glia cells are multiplied, connective-tissue elements are increased, and a small patch of sclerosis or a state of sclerotic atrophy of an entire lobe of the hemisphere, or any degree between these extremes, may be left as a ^For a review of these rare cases see Judson and Carncross, Amer. Jour. Med. Sci., Dec, 1908. SYMPTOMS. 537 permanent condition. Thus, disseminated encephalitis of infectious origin is tne starting point of many cases of so-called multiple sclerosis. It is not possible that, as time goes on or as other infections occur these sclerotic patches may extend, but, as a rule, they are latent. These pathological changes in the brain when on the surface are often at- tended by the lesions of meningitis in the pia and dura. Hence, in the terminal stage an adhesion of the membranes to the cortex is fre- quently found. In a few fatal cases of recent origin thrombosis of the venous sinuses has been observed The occurrence of leucocytosis during life will distinguish this class of cases from those due to vascu- lar lesions only. Symptoms. — The symptoms of encephalitis may be divided into two classes : first the general symptoms of the infectious disease, and secondly the local symptoms of the particular region of the brain affected. The general symptoms develop acutely. After a day or two of in- definite feelings of malaise, attended by vertigo and headache, and in children by extreme fretfulness, there is a sudden chill, attended some- times by vomiting and by a convulsion, followed by a state of stupor or coma. The temperature rises rapidly to 102° or 104° F., the pulse is rapid but regular, the respiration normal, rarely of the Cheyne- Stokes type. In the state of stupor there is restlessness and delirium. This stupor may deepen into a coma which may never be recovered from. If the patient does not die in the coma he gradually recovers consciousness, but delirium and even acute maniacal excitement are not uncommon for a week or more, and have been known to continue with fever for twenty days. The recovery of consciousness is slow, and the mind is not clear for some days. The fever subsides by remis- sions in the morning and its attendant symptoms of general constitu- tional disturbance gradually pass away. Leucocytosis is observed throughout. A day or two after the onset some local symptoms become evident, and then the diagnosis of hemiplegia, or of ophthalmoplegia, or of bulbar palsy, may be made. The local symptoms in the first type of case are aphasia, or hemi- plegia, or monoplegia, and these are not infrequently combined at the outset. The aphasia, as a rule, passes away, but some degree of hemi- plegia or monoplegia remains. Thus, in one of my patients, while total aphasia and right hemiplegia occurred at the onset, a paralysis with athetosis of the right arm was the only permanent symptom. In another case which developed suddenly during convalescence from cerebro-spinal meningitis, a left hemiplegia with mental weakness remains. In another type of case the local symptoms may be sensory rather than motor, some form of word-blindness or word-deafness being a per- manent result, or hemianopsia, as in a case of Furbringer. It is prob- able that this is the origin of deaf-mutism in some cases. In other cases a state of imbecility develops, and the child naturally born, and bright before the attack, grows up deficient in intelligence and in the higher powers of the mind. 53S EVCSPBALtTlS. In some rare cases, as in one of my own, the local symptoms pomt to the cerebellum as the site of the inflammation. A child of three years, able to walk and feed itself, was left after such an acute attack, which came on after measles, with a great degree of ataxia in the hands and an inability to balance itself on its feet, to stand or to walk or to sit up without the aid of its hands. In one such case of Bethe cere- bellar lesions were found. In all these types of cases epilepsy of the cortical variety is a com- mon sequel, and may be the only permanent symptom remaining. In Mills' case Jacksonian epilepsy was the chief symptom. It is my belief that many cases of so-called idiopathic epilepsy, especially those that are characterized by a uniform sensory or motor aura, originate in acute encephalitis during infancy or childhood. In another type of cases (polio-encephalitis superior of Wernicke) the local symptoms are confined to the eye muscles ; there is ptosis of one or both eyes ; the eyeballs cannot be moved voluntarily together ; there is some form of strabismus; there is nystagmus, and in a few cases optic neuritis has been observed. There is always intense vertigo with this condition, and hence a staggering gait is sometimes noticed. I have seen but one such case. The patient was a physi- cian, and recovered entirely within a year. Weakness, tremor, and ataxia of the limbs, facial paralysis, difficulty in speech and in swal- lowing have occurred in the severer cases. In adults, especially in patients who are alcoholics, this type is more common than the hemi- plegic type. It may develop with few constitutional symptoms and even without fever. In this respect it resembles acute anterior polio- myelitis in adults. In still another group of cases the local symptoms are those of an acute bulbar palsy. Speech, swallowing, and respiration are affected, the various characteristic signs of glosso-labio-laryngeal paralysis appear simultaneously, and in these cases a fatal result is more com- mon than recovery. These have been termed polio-encephalitis inferior. But the lesion is not always limited to the gray matter of the pons and medulla, and when it extends to the tracts passing through them the symptoms may be widespread. Thus various forms of alternating hemiplegia and hemiansesthesia or general paralysis may be the result. The combination of cerebral with spinal symptoms has already been mentioned. The course of the case in all these various types is usually one of gradual improvement, and a final recovery with a few local symptoms remain. Such are monoplegia, sensory defects in the visual field, paralysis of one or two ocular muscles, or epilepsy. In some cases all the local symptoms disappear, and then it is evident that few neurones were actually destroyed by the inflammation. In other cases a most irregular combination of symptoms such as nystagmus, defective articulation, tremor, and some form of paralysis, remains, and the terminal state is identical with that of multiple sclerosis. TBEATMENT. 539 Prognosis. — The prognosis during the acute onset must be guarded, as death sometimes occurs. It will depend upon the severity of the symptoms, especially on the occurrence of convulsions and upon the depth of the coma, the degree of temperature, the condition of the pulse and respiration, and the power of the individual to combat an acute infection. The prognosis after the acute stage has passed is favorable for recovery, and even serious local symptoms may pass away. The prognosis is better than in cerebral hemorrhage, embo- lism or thrombosis, as the focus of disease is usually smaller and there is not such a gross lesion. Recurrence of the disease has been seen in .three cases only (Dinken, Wiener, and Oppenheim). Treatment. — The treatment divides itself into the care of the patient during the acute stage and the treatment of local symptoms later. At the outset purgatives may be given and ice applied to the head, and antipyretics may be used freely, antipyrine, quinine, salicin. Leeches to the mastoid region are to be applied excepting in very anaemic persons, and may be repeated daily during the febrile stage. Hot baths with mustard may be given to children, and hot foot baths to adults. When the stage of onset is over the various local symptoms are to be treated in the same manner as if the case were one of apo- plexy, or of ophthalmoplegia, or of bulbar palsy, or of infantile spinal paralysis. CHAPTEH XXX. MENINGO-ENCEPHALITIS. PAEESIS. DEMENTIA PARALYTICA. Paresis or dementia paralytica is a diffuse degenerative disease of the cerebral cortex characterized by mental, motorj sensory, and vaso- motor symptoms, of progressive course and fatal ending. Etiology. — Males are much more liable to paresis than females.^ Inherited weakness of the nervous system predisposes the individual to the development of paresis. It is a disease of early adult life, the majority of cases developing between the ages of thirty and forty-five years ; but no age is exempt, as cases have been reported both in child- hood and in old age. Mott^ has recently reported 22 cases of juvenile general paresis, and has collected 75 other cases. It is evident, there- fore, that the disease may develop in children. In the majority of these cases hereditary syphilis was found to be the cause. It is a post- syphilitic disease in about 60 per cent, of the cases, though statistics gathered from 54 different authorities by Mott^ gave a varying per- centage from 11 per cent, to 94 per cent. The German statistics show 80 per cent, of the cases to be syphilitic, and recent tests of the spinal fluid and of the blood have demonstrated the presence of syphilis in 90 per cent, of the cases according to Nonne. But, as in locomotor ataxia, it is not due to an active syphilitic process, since the patho- logical lesions are not peculiarly syphilitic, and it is not arrested by antisyphilitic treatment. The interval between the syphilitic infection and the onset of paresis is usually a considerable one — from five to twenty years. Mental strain and anxiety are the chief exciting causes of the disease. The tremendous efforts to attain financial and social success, the enormous responsibilities undertaken by ambitious men, the effort to carry through large projects, and the ceaseless work day and night, with no rest and little sleep, which are characteristic of life at the present time in our large cities, are the chief factors in the rapid increase in the number of cases of paresis. It is admitted by all that it is a disease of the more highly civilized, the more mentally active type of men. Hence, the higher classes are more liable than the lower, and those who lead an agricultural life are almost exempt. Peterson states that in Egypt the disease is very rare, though syphilis is prevalent there. The Mohammedans do not use alcohol. Alco- ^ The statistical statements in this chapter are based on the analysis of 3454 cases in the Berlin Asylum by Junius and Amdt, Arch. f. Psych., Bd. XLR^., 1908. See also Jolly, Arch. f. Psych., Bd. XLIV., p. 259, and upon 3,000 cases analyzed by Clark and Atwood, Jour. Nerv. and Ment. Dis., Sept., 1907. ^Archives of Neurology of London County Asylum, 1899, vol. i., p. 250. ^Loc. cit., p. 169. 540 PATHOLOGY. 541 holism and excess in sexual indulgence are certainly additional factors in its prodijction. Alcoholism is present in a third of the cases. A certain number of cases seem to be traceable to injuries of the head and to sunstroke. Pathology. — The disease begins with changes in the vessels, hypersemia of the pia and small cortical vessels, increase of nuclei in the vessel walls, occasional stasis in the capillaries, and exudation of serum into the lymph spaces. As it advances, a formation of fusi- form dilatations of the vessels and the development of fibrils of con- nective tissue between the vessel walls and the surrounding neuroglia occur. In the neuroglia at the same time a marked increase of the nucleated cells, with numerous branching processes is in progress, numerous spider cells developing throughout the cortex, around the vessels and lymph spaces, and about the nerve cells. This increase of neuroglia goes on rapidly, producing a diflPuse sclerosis of the cortex, which is followed by retraction of the tissue leading to atrophic shrink- ing. The entire brain takes part to a lesser degree in this process. In the cerebral tissue at the same time there is in progress a degeneration of the finest nerve fibrillse and of the branching processes of the nerve cells. This begins in the tangential fibres of the first layer of the cor- tex, but later involves all the layers of cells. A swelling chromatoly- sis, with hyaline or fatty degeneration, vacuolization, pigmentation, and final atrophy of the cell bodies takes place. The formation of cystic cavities throughout both gray and white matter is often observed. Mott,^ whose investigations are most reliable, believes that the start- ing point of paresis is a degeneration in the neurones of the cortex. This is attended by the production of choline (see page 33) which can be found in the cerebro-spinal fluid in paretics. Choline is a toxic substance causing a depression of the heart and a lowering of arterial pressure. Mott believes that its presence causes a venous stasis in the veins of the brain, especially those of the convexity opening into the longitudinal sinus, in which the blood flows contrary to gravity. (See Fig. 214, page 465.) He points out the fact that it is in the domain of these veins that the thickening of the pia arachnoid occurs. The choline accumulates in the perivascular lymph spaces about the veins, causing stasis and inflammation, which are intensified by the action of gravity in the veins that empty into the longitudinal sinus. (Edema of the brain follows, and this is always present in paretic brains. The cerebro-spinal fluid contains leucocytes in much greater number than in health. The final result of these pathological processes in the vessels, neu- roglia, and cerebral substance is a gradual atrophy of the brain, so that it weighs much less than normal, sometimes only two-thirds of the normal, the left hemisphere being more atrophied than the right one ; it appears shrunken, the convolutions being narrow and the sulci open; the brain tissue is hard, pale, friable, pigmented and on the summit of the convolutions adherent to the pia, which is opaque and thickened. ' Aicliivurf of JNeuroiogy of London County Asylums, vol. i., p. 396. 542 MENINGO-ENCEPEAIJTIS. PARESIS. When the pia is stripped oif, portions of the cortex come away with it, The thickness of the cortex is seen to be much reduced. A thickened condition of the ependyma of the ventricles is uniformly found. There is usually an increase of fluid within the ventricles and beneath the pia. As a complication the lesions of pachymeningitis with hsematoma are not infrequently present. All of the changes in the cortex are more marked in the frontal lobes and motor area and about the fissures of Sylvius, but in the later stages of the disease no part of the cortex is normal. In about 10 per cent, of the cases spinal lesions are found in addition to the cerebral lesions. These consist of sclerosis in the pos- terior and lateral columns of the cord. There are some cases in which the posterior sclerosis is the first lesion to appear, and in these the lesions of locomotor ataxia are well marked, as already described, page 339. In other cases they appear after the condition of paresis has developed, and then they are not so complete. The sclerosis in the lateral columns is usually due to secondary degeneration in the motor tracts after changes in the motor cells of the cortex. Symptoms. — Paresis begins, as a rule, very gradually, so that many cases are overlooked and others are considered as neurasthenia or dyspepsia for some months, there being present some of the symp- toms of one or both of these diseases. After a time the friends of the patient begin to notice that he is not acting naturally, that he is unusu- ally irritable or excitable, is offended or delighted at slight provoca- tion, is variable in his moods, and appears at times inattentive, forget- ful, and careless of the proprieties. Soon it is found that his aifairs are in confusion, his accounts not properly kept, and that it is impos- sible to keep his attention concentrated upon important business or upon any train of thought, so that his conversation becomes noticeably fragmentary. He cannot do his work, and yet resents any interference with it, maintaining that he is perfectly well. He will admit some confusion of thought, some cerebral sensations of discomfort, irritability and sleeplessness. His memory for recent occurrences then begins to fail. He neglects engagements ; he disregards obligations, sleeps in company, loses his temper, and uses bad language at home or in public, neglects his family, and, though previously moral in conduct, indulges in alcoholic and sexual excesses. By this time it is noticed that his speech is a little thick, indistinct, and hesitating, and a fine tremor of the tongue and lips and possibly of the hands may be detected. The affection is one of fine co5rdmated movements — a cortical ataxia rather than gross paralysis. The pupils are often unequal, or contracted, or dilated, and react very slowly to the light in the early stage ; and soon fail to react to light at all, though they react in accommodation. The Argyll-Robertson pupil is present in 95 per cent, of cases. During the period of invasion headache and sleeplessness are some- times prominent symptoms, and a gradual loss of weight goes on. From the outset restlessness is noticeable, and after a time it seems impossible for the patient to keep quiet, he wants to take all kinds of exercise, to walk for miles^ to go about even when exhausted by fatigue. SYMPTOMS. 543 His intellectual powers are notably weakened, he is no longer attentive, logical, seif-controlled, cautious, or careful in conduct, and the change of character becomes apparent both to his family and to his acquaint- ances. This period of invasion varies from one to three years and may be interrupted by periods of subsidence of many of the symptoms. It usually goes on to the full development of the disease. The prominent symptoms during the period of invasion differ some- what in different cases, and this has led German authors to distinguish between various types of the disease, (a) They find the demented type the most common, 37 per cent, of their cases belonging to it. In this type there is a slowly advancing mental failure with imperfect power of attention and defects of memory and an indifference and apathy as the more prominent mental symptoms. (6) The expansive type is next most common, 27 per cent, of their cases being thus classified. In this type the patients are constantly boasting of their physical or financial well-being, are confident of their own capacity and undertake all sorts of absurd schemes. Such patients if wealthy are liable to ruin themselves by foolish financial schemes, and if in places of power are able to do much harm to others as well as themselves. The cause of many failures in the business and banking world, and the wrecking of several railway systems can be traced to beginning paresis m the man in power, (c) The depressed type is the third in frequency, 17 per cent, belonging to it. In this type the neurasthenic symptoms are attended by much mental depression ; self-accusation and suicidal impulses being common. The patient is distrustful of his capacity, is depressed in his spirits, takes a dark view of his affairs and is reluctant to undertake responsibility, appreciating his own growing incapacity. He IS worried over his affairs, even without cause, and is apprehensive of his own condition. Many such cases are at first supposed to be cases of simple melancholia and the diagnosis is difficult until the physical signs of paresis appear, {d) The fourth type is the agitated paretic who is restless, uneasy, physically overactive and mentally irritable. Eight per cent, belong to this class. These patients wear themselves out by their constant activity, (e) The remaining 11 per cent, of patients, not included in these types, present a mixture of symptoms which prevent their classification. And however the disease may have begun, it is not uncommon for each type eventually to pre- sent many symptoms prominent in the other types. Clark and Atwood,' in an analysis of 3000 cases, found the grandiose or exalted type in 70 per cent., the demented type in 20 per cent., and the depressed type in 10 per cent. This would indicate a racial dif- ference in the process of the disease in the different countries. When paretic dementia is fully developed numerous symptoms pre- sent themselves — both mental and physical. The power of sensory perception is not impaired, but the patient is often so preoccupied or indifferent that he does not notice fully or remember the subjects to which attention is directed. His mind passes 'Jour. Nerv. and M^nt. Dis., Sept., 1907, 544 MENINGO-ENCEPHALITIS. PARESIS. rapidly from one thing to another, and he does not perceive anything accurately. Thus a patient will forget that he has dined, or neglect to put on some of his clothes, will expose his person, or will commit openly small thefts, will make serious mistakes in his accounts or ap- pointments, merely from a lack of careful perception and consequent impairment of memory. Illusions and hallucinations occur only toward the close of the disease, and are followed by a marked loss of power of sensory perception when the dementia becomes extreme. The power of logical thought is impaired very early. The patient shows impaired judgment from the outset, takes little notice of impor- tant matters, dwells upon trivial things, acts without consideration, and indulges in extravagant schemes, reckless expenditures, and excesses of many kinds which his better judgment would condemn. He may be- come exceedingly immoral, all restraints of society, religion, and mor- ality being wholly neglected. He becomes profane, obscene, and tipsy. He very frequently develops a delusion of grandeur, says that he never was so well in his life, believes that he is the strongest, the brightest, the most powerful, the most wealthy of men. And he acts at times in accordance with his delusion, undertaking the most extravagant and impossible schemes without any regard for his actual circumstances. Many a patient involves himself and others in financial difficulties by undertaking colossal combinations and extensive business organizations far beyond his powers or means, before it is realized that these schemes are the product of an unbalanced mind. Yet the delusions are not carried out fully in his actions, and he mixes ordinary affairs with them in an incongruous manner. The delusions are not as fixed or as systematized as in paranoia. The development of such delusions is a sure sign, in Meynert's opinion, that an atrophy of the brain has begun. His emotional state is unstable. He has little control over its mani- festations, can be moved to tears or to laughter in the course of an ordinary conversation, can be excited or depressed by suggestions. He is sometimes depressed for days, and feels discomfort, but does not blame himself, and in the midst of the depression may become excited. He is usually sanguine and hopeful, and his emotion is usually in accordance with his delusion, one of exaltation. At times or on pro- vocation the excitement may become maniacal, and outbursts of rage or of frenzy are not infrequent in the course of the disease. Hence a paretic dement is never a harmless member of society, but requires to be watched. He rarely shows any tendency to suicide. Voluntary action and conduct are affected from the outset ; indeed it is by change in conduct that the changes in thought and character are chiefly betrayed, and the weak logical power made manifest. In- consistency is evident in speech and conduct. Acts are done without consideration ; thus impulsive acts, such as stealing, forgery, enarmous expenditures, even murder may be performed under sudden excitement or under the influence of a delusion, or in a fit of frenzy. All the ordinary restraints to conduct seem to be removed, and instinct rather than morality is the guide, self-control being greatly weakened. SYMPTOMS. 545 Self-consciousness appears to be very imperfect. The patient does not appreciate the inconsistency between his previous personality and his present acts ; he does not notice the growing anxiety of his family ; he regards with indifference loss of means, or even his confinement in an asylum ; he is usually so engaged in his imaginary undertakings under the influence of his delusions that he is contented wherever he is. His personality may be changed, and he may imagine himself a prince, a millionaire, a deity. Gradually the consciousness becomes obscured, he recollects little regarding his illness, and as the deeper dementia ensues he becomes indifferent, dull, stupid, and finally almost stuporous. In the final condition of dementia all mental action is suspended. The physical symptoms are as marked as the mental symptoms, and in many cases are the first to appear. Motor disturbances appear early. There is restlessness, a tendency to be in constant motion. The patient takes long walks or rides, indulges in unusual exercise, wishes to be going out to theatres every night, to visit friends all the time, and is not content to lead an ordi- nary quiet life, as before his illness. He has a sense of physical power, and is eager to show his strength, though this may really be impaired He is very talkative, discussing subjects of which he knows little, or talk- ing at random. Tremor of the muscles appears early, first in the tongue, then in the face and hands, and finally any motion is attended with some trembling. This tremor is followed by incoordination, which shows itself in thickness and indistinctness of speech, in irregu- larity of handwriting, in clumsiness in handling things, and an awk- ward, unsteady, stumbling gait. The speech is quite characteristic, letters being slurred and words mispronounced, so that any long word or combination of difficult syllables, like " third artillery brigade," cannot be said. In writing the patient will omit letters from words, later may write wholly unintelligible phrases, or even merely make marks on the paper, and yet not appreciate these defects of writing. From the first his handwriting is changed, lines being irregular, letters too small or too large, and badly written. The facial expression be- comes blank and inane — no appearance of thought or interest being manifest, but when he talks an excessive play of the facial muscles is noticeable. Paresis shows itself after a time in the limbs, and pro- gresses until in the final stage there is total paralysis, with increased reflex action and loss of all control over bladder and rectum. Finally speech may become impossible. The knee-jerks are either much increased or are lost from the outset. In 60 cases observed in my clinic the knee-jerks were increased in 53 per cent, and lost in 30 per cent.^ which corresponds exactly with the statistics of the German authors based on 992 cases. In the course of the disease epileptiform attacks, attacks of severe vertigo, attacks of sudden loss of consciousness without convulsions, and attacks of monoplegia or hemiplegia, temporary or permanent, often occur. They may be the initial symptoms of the disease. They 36 ^ E. L, Hunt, New York, Medical Keeord, January, 1905. 546 PARESIS. DEMENTIA PARALYTICA. occur in one half of the patients at some time in the course of the affection. Vasomotor disturbances are also present. The face flushes or pales frequently, there are sudden attacks of vertigo and of headache, and of feeling of fulness in the head ; the pulse is usually slow, large, and of low tension. In the late stages venous congestions in various organs are found, and hsematoma of the ears. Lumbar puncture reveals leucocytosis of the spinal fluid, as many as 100 leucocytes being found in 3 c.c. of fluid. This is an early symp- tom and of importance in diagnosis. Sensory symptoms are less marked than motor symptoms, but as the disease advances anaesthesia or analgesia of the limbs may be found, especially in connection with hemiplegia. In the last stage trophic disturbances occur, for in the stupid, dirty, helpless state of the patient cleanliness is diflicult and the liability to bed-sores and to cystitis is great. Some cases are complicated by the development of spinal sclerosis, either in the form of posterior or of lateral sclerosis, with its attendant symptoms, and in a few cases the paresis follows the spinal disease. It is evident that the mental and physical symptoms are very numer- ous. Many cases show only some of them ; others present all the symptoms during the course. In some cases the mental symptoms appear early and are more prominent throughout than the physical symptoms ; in other cases the reverse is observed. A few cases begin with epileptiform attacks. In other cases the symptoms begin with a hsematoma of the dura, causing an attack of apoplexy with aphasia, or hemiplegia, or hemianopsia. These symptoms subside rapidly, so that in a week or two the patient may appear to have recovered. But later the mental and physical symptoms of paresis appear, and then it is evident that the apoplexy was the first sign of paresis. The course of the disease is slowly progressive, though remissions of one or two years are not uncommon. Its average duration is about three years, though rapid cases may terminate within a year, and some are known to have lasted five or six years. According to the German statistics 19 per cent, die in the first year of the disease ; 26 per cent, in the second year; 27 per cent, in the third year; 12 per cent, in the fourth year ; 6 per cent, in the fifth year and 7 per cent, in the sixth or following years. No cases outlived the tenth year. In private practice the duration of the disease is longer than in asylums ; as great care can be taken of patients and fatal accidents avoided. The patients die of exhaustion or of some complicating disease, such as cystitis, or pneumonia, or obstruction of the bowels. Diagnosis. — The diagnosis of paresis is to be made from the combi- nation of physical signs and mental and physical symptoms. The loss of pupil reflex to light, unequal pupils, tremor of the face and tongue, indistinct speech, tremor of the hands, exaggeration or loss of the knee- jerks and spinal leucocytosis are objective physical signs of importance. The mental irritability, excitement with expansive ideas, defects of memory and of self-control are the most important early mental symp- PROGNOSIS. 547 toms. Attacks of epileptic or apoplectic nature are the most important physical symptoms. Neurasthenia may be present in the early stage of paresis, and any or all of its symptoms may then be apparent. But a neurasthenic patient always notices every symptom minutely, describes it fully, and discusses its significance, while a paretic is usually not aware of the fact that he is ill, and does not care to talk about his health as much as about his projects and success. Defects of memory and of speech are rare in neurasthenia, and the neurasthenic presents none of the physical signs of paresis. In a doubtful case it is on the appearance of these signs only that the diagnosis of paresis can be made. There are some cases of cerebral syphilis which resemble paresis and it is often difficult in the early stage to differentiate these diseases. Cerebral syphilis usually causes pain in the head, worse at night. If it produces disturbance of speech it is rather in the form of true aphasia not the tremulous indistinct speech of paresis. There is never any tremor of face and hands, and hence writing is clear and not impaired unless there is agraphia with aphasia, and this differs from the omis- sion of letters and words in writing present in paresis. Optic neuritis is often present in cerebral syphilis, but very rarely in paresis. The emotional state is usually depressed and the patient anxious in syphilis, while it is one of excitement and elation in paresis. Delusions are much less common in syphilis, and are never as extreme as in paresis. The signs of dementia are more marked in an early stage than they are in paresis ; the lack of memory, lack of power of reasoning, and lack of self-control appearing early in syphilis and late in paresis. In syphilis there are often physical signs of single or multiple localizable lesions, while paresis is a diffuse disease. The course of the case in syphilis is variable, with much improvement under treatment, while in paresis it is usually progressive. Multiple sclerosis can hardly be mistaken for paresis ; as the nystag- mus, tremor of an increasing kind on effort, tremor of the head and trunk, and absence of delusions even when some dementia is present are characteristic of multiple sclerosis. The dementia of old age and the dementia of chronic alcoholism are not attended by delusions of exaltation and grandeur. Prognosis. — The prognosis in paresis is absolutely bad. Krafft- Ebing declares that he has never seen a recovery in 2500 cases. It must, however, be remembered that remissions in the symptoms occur, especially in the early stage. I have known many patients who have had all the symptoms, to recover for a time under quiet rest in the country, and be able to return to business. Such remission may last for a year or two, during which time the only evidence of the disease may be the inactive pupil, and the slight tremor of the hands, and the exaggerated or lost knee-jerk. I have never known these physical signs to subside, and I have never known a remission to last more than two years. Hence, the prognosis as to recovery is bad. The prognosis as to the duration is different in the different types of the disease. In the agitated type it runs a rapid course and death 548 PARESIS. DEMENTIA PARALYTICA. occurs within the second year. The duration is somewhat longer in the depressed type. In the expansive type the chance of life is better than in either of these. The demented paretic lives the longest, often for four or five years after the disease is fully declared. The prognosis is also better in the mixed type than in the three first mentioned. It has been noticed that those who were predisposed to the disease by a bad inheritance lived longer when the disease developed than those whose inheritance was good. Treatment. — There is no form of treatment which will arrest paresis. As the majority of patients are syphilitic a course of treat- ment by salvarsan, or by mercury and iodide of potassium is advisable, this treatment being assisted by the use of daily hot (106° F.) baths for half an hour. In some cases I have seen material benefit as a result, and a remission of some months in the symptoms usually follows such a course. It is absolutely necessary for the patient to stop all business, to rest and to avoid emotional excitement. Hence, it is well to remove him from his home surroundings, and, as he is not fit to travel, it is better to send him to a sanitarium, or to some health resort, or some one of the hot springs, where water treatment can be given. Some forms of hydrotherapy are of service in these cases, but cold batlis and douches are to be avoided. A tub bath, or a hot-air box, followed by a shower not below 80° F., and massage daily appears to exert a favorable effect upon the circulation and to quiet many nervous symptoms. When the patient becomes uncontrollable by his family or nurses it is better to commit him to an asylum where he may improve, so that in a period of remission he may be released and allowed to travel. The quiet routine life of an institution, without excitement or responsibility, seems favorable to the subsidence of the symptoms of irritation. In the later stages of the disease, when dementia is well-marked, care in an asylum, or in a home well secluded and provided with skilled nurses, may prolong life for many months. Some of the symptoms require active treatment. Sleeplessness must be treated by hot baths at night, or massage, or by the use of paralde- hyd in 60-grain dose, or trional 20 grains, or sulphonal 15 grains. It is well to give hypnotics with hot milk, as they are more rapidly absorbed and more efficacious. The motor restlessness leads to over- fatigue and heart failure ; hence very many short walks in a day, each followed by a short rest, are better than the long tramps which these patients are proud of taking. When the mental excitement becomes very intense, and the patients cannot be controlled by argument, active purgatives, such as compound cathartic pills, aloes, or croton oil, which cause a temporary anaemia of the brain, will quiet them better than sedatives. An occasional use of bromide, or bromide and chloral, or even a hypodermic injection of morphine ^, or hydrobromate of hyosciney^-g- grain, may, however, be necessary to allay excitement. A single dose of fluid extract of ergot, one drachm, may have the same effect, but the continued use of ergot is to be avoided. CHAPTER XXXI. ABSCESS OF THE BEAIN. Etiology. — Abscess of the brain occurs as a sequel to injuries of the head, with and occasionally without fracture. In cases of com- pound fracture or of wounds in which a septic infection occurs at the time of the injury such abscess formation is to be expected. This is particularly true if a small bit of bone or a foreign body remains in the brain. In cases where the scalp is only bruised it is difficult to understand the source of infection. Von Bergman declares that a simple contusion of the head is not capable of causing an abscess, but I have seen it in several cases where the scalp was not broken, though the bone was fractured. In some cases the abscess follows soon after the injury; in other cases the symptoms do not develop for many weeks or months ; and cases are on record where the only explanation for the presence of a latent abscess found unexpectedly at the autopsy was an injury received years before. Fig. 238. Large abscess in the inferior parietal region secondary to fracture of the skuU. The thick capsule of the abscess can be seen. In the case of an infant seen with Poore at St. Mary's Hospital, a fall upon the left parietal bone, causing a laceration of the scalp and an indentation of the soft bone, was followed within two weeks by the development of right hemiplegia with hemianopsia. At the autopsy a large cerebral abscess was found beneath the site of the injury within 549 550 ABSCESS OF THE BRAIN. the white matter of the brain, there being no apparent affection of the cortex for one-fourth of an inch above the abscess. The abscess had a thick capsule and was well defined. (See Fig. 238.) An attempt was made to open it, but the operation had to be suspended on account of the collapse of the patient. Fig. 239. Abscess of the temporal lobe after otitis media. (Larkin.) In a second case, seen with McBurney at Roosevelt Hospital, a compound fracture of the superior portion of the left parietal bone, which had healed, had been followed three months later by the gradual development of paralysis of the right leg. At the operation a large abscess was found involving the superior parietal lobule and adjacent portion of the posterior central convolution. In the midst of the abscess a considerable piece of bone was found, which evidently had been driven in at the time of the fracture. The operation was successful. The patient recovered and gradually regained power in the leg. PLATE XXIV Abscess of Cerebellum Secondary to Chronic Suppurative Otitis Media. (Bacon.) The right cerebellar hemisphere has been divided and the lower half removed, exposing the abscess cavity and its wall {a) and the area of hemorrhagic softening posterior to the abscess {b). ETIOLOGY. 551 , In a third^case, also seen with McBuj-ney at Roosevelt Hospital, a similar compound depressed fracture, with spicula of bone in the brain, had led to the development of an abscess within two weeks of the time of fracture. The abscess cavity invaded the middle third of the motor area, and caused hemiplegia with paralysis chiefly marked in the arm, and here, too, at the time of operation, pieces of bone had to be removed as well as the purulent accumulation around them. The operation was successful and the boy recovered. In a fourth case, seen with McCosh at the Presbyterian Hospital, a fracture of the skull over the left occipital region had produced well- marked right homonymous hemianopsia. Trephining resulted in the successful evacuation of the abscess, but the hemianopsia remained permanently. In 22 cases of abscess of the brain observed at the Presbyterian Hospital, 12 have been due to trauma. The second and probably the most frequent cause of brain abscess is chronic otitis media. A very thin layer of bone separates the cavity of the middle ear from the dura and brain. It is easy to conceive that a slight degree of caries in this bone will open a way for the Fig. 240. 1. Primary abscess cavity connected with sinus in tympanic roof. 2. Secondary abscess cavity in temporo-sphenoidal lobe. Both abscess cavities found free from pus at autopsy. (Bacon, ) entrance of microorganisms, and their development will lead to either purulent meningitis or to the formation of an abscess. Such an ab- scess may develop in the temporal lobe of the brain or in the lateral lobe of the cerebellum. Fig. 240 and Plate XXIV. show such abscesses. The first attack of otitis media is rarely followed by a brain abscess. It is in the chronic cases, where as time has gone on the bone has been slowly eroded, that a sudden attack of inflammation of the ear goes on-to the formation of the brain abscess. 552 ABSCESS OF THE BRAIN. Many cases in adult life are traced to infectious otitis media occur- ring in childhood, the frequency of this complication of scarlatina and measles being well known. In all such cases there is a history of a chronic purulent discharge from the ear, which from time to time ceases, but recurs, and then suddenly an acute attack of pain in the ear occurs, which is soon fol- lowed by the cerebral symptoms of abscess. In other cases the acute attack of otitis subsides, but the patient does not feel quite well, and after some weeks the signs of a brain abscess occur. Korner has shown that such abscesses are more common on the right side. Barr in a collection of 76 such abscesses found 55 in the temporal lobe, 13 in the cerebellum, 2 in the pons, and 1 in the crus. Poulsen collected 13 cases of abscess, of which 9 were in the temporal lobe and 4 in the cerebellum. Between 1900 and 1905 82 abscesses secondary to otitis media have been published ; of these 54 were in the temporal lobe, 25 in the cerebellum and 2 in the occipital lobe, and 1 in the third frontal convolution. A third cause of brain abscess is a carious process starting in the nasal cavity or orbit, or an infection after some operation upon these parts. Thus, some years ago, before the importance of aseptic surgery was appreciated, I saw a case of abscess of the frontal lobe, with sudden death, which followed within a week of an operation for the removal of an exostosis of the septum. I have known of several similar cases, and Kahnt and Dreyfuss have collected a number. Abscess of the brain has been known to follow erysipelas of the face and head. Metastatic abscess in the brain after abscess and gangrene of the lung is not very common, but occurs. Nahter has collected 8 such cases in a study of 100 cases of pulmonary gangrene. It may also follow bronchiectatic abscess, empyema and pyaemia, ulcerative endo- carditis, and abscess in any organ. In these cases there are small multiple abscesses in the brain, whose existence is usually overlooked, as the general symptoms of the original disease obscure the cerebral symptoms. It has been supposed that in some cases of unknown origin the cere- bral abscess is the result of some infection, and many forms of infec- tious disease — typhus, typhoid, cerebro-spinal meningitis, measles, influenza, and tuberculosis have been considered the starting point of cerebral abscess in various reported cases. Pathology. — Abscess in the brain is the result of a purulent enceph- alitis. Streptococcus pyogenes, staphylococcus pyogenes aureus, albus and citreus, and pneumococcus have been found in such abscesses. It may present several different appearances. There may be a small, localized, softened condition of the brain tissue, in which on micro- scopic examination leucocytes, pus cells, and microorganisms are found. This is the condition in metastatic abscesses. Von Bergman reports a case in which 100 such little collections of pus were found in one brain in a patient who had pyaemia after gangrene of the leg. SYMPTOMS. 553 There may be a collection of pus within an irregular cavity without distinct wall, but surrounded by a more or less pulpy, broken down, and hemorrhagic area of brain tissue. The pus is green or brown and fetid. Such abscesses extend rapidly and lead to a fatal termination. Even if evacuated and drained they are likely to go on, and very often set up a meningitis. There may be a collection of pus enclosed in a thick connective- tissue capsule lying in the white matter of the brain, and quite distinct from the brain tissue. This form occasionally shows a tendency to rapid progress, but is usually of slow growth. It usually lies in or on the brain like a tumor, causing symptoms of pressure ; or it may cause no symptoms whatever, and surprise the pathologist at the autopsy. This form is probably a terminal result of the second form described, nature forming a protecting capsule to the disintegrated tissue and shutting up the pus in the thick wall ; but such a process may go on with some rapidity, as I have seen a thick wall in a case of abscess of but three weeks' duration. The existence of the wall does not pre- clude the possibility of an extension of the abscess, and in such cases an infiltration of the brain tissue about it with pus, leucocytes and bacteria has been found. The size of such an abscess varies. It is usually about as large as a walnut, but may be much larger. The rupture of such a capsule may cause sudden death, as in one of my patients. It may rupture inward into the ventricle or outward on to the surface. It is not possible for an abscess to be absorbed. In many cases of abscess there occurs a complicating meningitis or thrombosis of the cerebral sinuses just prior to death. Symptoms. — The general symptoms of cerebral abscess are, in the order of their importance : 1 . Headache, general but sometimes located over the seat of the abscess. 2. A change in mental characteristics, of the nature of irritability, alternating with dulness, imperfect attention, slowness of thought, and defects of memory — a semicoma- tose condition and appearance of illness out of proportion to the other symptoms. 3. Abnormal temperature. 4. Changes in the blood. 5. Tenderness of the head to percussion, with change in the percussion note over the site of the abscess. 6. Facial palsy of the peripheral type upon the side effected. 7. Optic neuritis. 8. General febrile symptoms with occasional chills. The development of these symptoms in a patient who had had an injury of the head, or is the subject of chronic otitis media, or who has been exposed to any of the causes already mentioned, should awaken grave anxiety. It will be noticed that none of these symptons are distinctly diag- nostic of brain abscess, for all of them may occur in meningitis or in thrombosis of the lateral sinus, and all of them may vary in severity in any given case. I have seen such a combination of symptoms in patients with otitis media purulenta who, by proper surgical treatment, have gone on to recovery without any subsequent evidences of brain abscess. Thus in two of the cases of traumatic abscess of the brain already mentioned headache was not present. In a case of abscess of the cere- 554 ABSCESS OF THE BRAIK. bellum which I saw with Gorham Bacon/ headache was not a very marked symptom until late in the disease, some time after the begin- ning of the abscess ; and in a case of abscess subsequent to ear disease, seen with McBurney, headache, though present, was never very severe. In other patients, however, I have known it to be intense, so that it formed the chief complaint. It is evident, therefore, that from the degree of headache no conclusion can be arrived at that is of diagnostic value. It is usually stated that the headache in meningitis is more severe and constant than it is in abscess ; but if all degrees of headache may occur in abscess, it is evident that this symptom cannot be relied upon in diagnosis. It is equally difficult to estimate the real importance of mental symp- toms. In the first place, the mental capacity and characteristics of different individuals are so diiFerent that it is necessary to have known the person prior to his illness in order to reach any definite judgment. A condition of excitement and talkativeness in a person who is ordi- narily reserved or stolid is of much more importance than in an excit- able and loquacious individual ; and, vice versa, a condition of apathy and stupor in a man of bright, active intelligence is of much greater diagnostic value than in a dull or stupid person. Anyone who watches a large class going through gymnastic movements under the leadership of an instructor will notice that a certain proportion are quite percep- tibly behind the others in their motions, and even in a class of fifty it is rare to ^nd any two who are moving exactly in time, especially when the exercise performed is a novel one. This fact illustrates the varia- bility of the personal equation — the diiFerent rate that obtains in the thinking process of different individuals — and unless this fact is kept in mind in judging of the mental state, of the slowness of thought, or of the defects of attention in a person who is ill, very erroneous con- clusions may be reached regarding their significance. The effect of illness upon mental processes is not always appreciated, and varies much with the individual, some persons becoming restless, irritable, and more active mentally when ill, others becoming lethargic, morose, and apparently dull. Inasmuch as these variations are observed in all forms of illness, it is obviously erroneous to attach much differential diagnostic value to the mental state in distinguishing between three con- ditions — abscess, meningitis, and sinus thrombosis — so closely allied. The course of the temperature in abscess of the brain is quite variable. The statement has been made by MacEwen,^ and seems to be borne out by his cases, that it is common in abscess of the brain to find a persistently low temperature with little variation. MacEwen states that during the preliminary period the temperature has been slightly above normal. During the period of full development of the abscess the temperature is about normal or slightly subnormal, from 97° to 99° F.; and that in the terminal stage if the abscess bursts the temperature rises within a few hours with a bound to 104° or 105° F., 1 American Journal of the Medical Sciences, August, 1895. ^Pyogenic Infective Diseases of the Brain, Macmillan & Cb., London, 1893. SYMPTOMS. 555 but if the ajbscess is evacuated by operation the temperature rises to 100|° or 101° F., and in a few hours comes down to below 100° F., remaining near the normal until recovery ensues. He contrasts sharply this run of temperature with that found in infective thrombosis of the lateral or cavernous sinuses, in which very high temperatures with very great remissions, resulting in a most irregular curve between 106° and 97 J ° F., have been observed. He also contrasts it with the tempera- ture curve of meningitis, in which the temperature is high, between 103° and 104° F. constantly, without the great remissions occurring in thrombosis. Okada found a marked rise of temperature and a febrile course in 46 of 88 cases of abscess of the cerebellum; in 15 the temperature was normal ; in 15 it was subnormal ; in 8 there was a rise of temperature only at the onset, in 4 only at the very end. A study of the temperatures occurring in three successive cases of cerebral abscess shows that any conclusion from a study of temperature is not reliable. Thus in a case seen with Bacon, ^ the curve was one which suggested thrombosis of the sinuses, there being very high temperatures, followed by sudden remissions two or three times a day for several days prior to the operation. In this case the abscess was successfully evacuated and the patient recovered. In a second case the temperature chart corresponded pretty nearly to that described by MacEwen ; the temperature was not above 98 J ° F. or below 98° F. during the week of illness. In a third case the temperature chart was much more suggestive of meningitis, varying from 101° to 104° F. during the entire illness of eight days. Hence the conclusion must be reached that the course ,of temperature is not to be relied upon as a diagnostic sign. The changes in the blood are important from a diagnostic and prog- nostic standpoint. There is an increase in the relative proportion of polynuclear cells on differential count above 85 per cent, and the higher the proportion the more active the inflammation in the brain. Sondern ^ has shown that this is associated with a greater or lesser degree of leucocytosis ; and the higher the leucocytosis with a given percentage of polynuclear cells, the better ordinarily is the body resis- tance toward the infection at the time the blood examination was made. A slight polynuclear increase with pronounced leucocyte increase indi- cates slight infection and well marked body resistance. A pronounced polynuclear increase associated with a pronounced leucocytosis indi- cates a severe infection and good body resistance. A pronounced polynuclear increase and little or no leucocytosis indicate a severe in- fection and little or no body resistance. An absence of leucocytosis in severe inflammatory lesions is a bad prognostic sign. If a differential count is made the high relative polynuclear percentage will also be found. An increasing polynuclear percentage and stationary or de- creasing leucocytosis indicates an increasing degree of infection and ' New York Medical Journal, August 15, 1896. ''Sondern, Blood Count in Sepsis. N. Y. Med. Jour., June 16, 1906. 556 ABSCESS OF THE BHAin. decreasing body resistance. A decrease both in the polynuclear per- centage and in the leucocytosis denotes improvement. In some cases of brain abscess these changes are not found. When pus is confined by dense pyogenic or fibrous membrane, in other words, when no absorption of toxine occurs, there is no leucocytosis and no polynuclear increase. Hence in some abscesses the blood count does not help us. Sometimes the rapid emaciation of the patient, his cachectic appear- ance, with yellow skin and general evidences of a septic state, are so marked as to suggest a latent abscess, even when the temperature is low. MacEwen was the first to call attention to the diiFerence in the per- cussion note over the side of the head upon which the abscess lies. It is somewhat difficult to elicit this physical sign. The stethoscope is to be placed upon the forehead in the median line, or upon some bald spot upon the head, as any contact with the hair vitiates the test. The head is to be struck with a percussion hammer tipped with rubber. The note elicited varies very much in different skulls, it being very dull and flat in a baby, where the bones are very thin, and of higher pitch and clearer in the adult. The patient's head should not rest upon the pillow while the test is applied. MacEwen believes that as the intracranial pressure increases the percussion note becomes higher in pitch and of greater resonance, and that this resonance increases as the disease advances. In four cases of brain tumor I have been able to confirm this statement, the note elicited being of greater resonance and higher pitch over the side of the tumor than upon the opposite side. In all these cases there was a great distention of the lateral ventricles with fluid, a condition which MacEwen also mentions as affecting the percussion note. In two other cases of brain tumor, and in two cases of cerebral abscess, no essential difference could be determined between the two sides. It is evident, therefore, that this sign is one which requires further study before it can be said to be diagnostic, though it appears to be of considerable value, and should be more widely known and more commonly noticed. Tenderness to percussion or to pressure is a symptom emphasized by Horsley as occurring in brain tumors, and it has been observed in brain abscess. It appears to depend in both cases upon the nearness of the tumor or abscess to the surface or upon a thinning of the cranial bones in the vicinity of the tumor or abscess. Such a thinning of the crani il bones occurs in some cases in a very remarkable manner. Thus in a patient suffering from tumor of the brain, seen at the New York Hos- pital with Peabody, the symptoms pointed to a tumor located in the posterior central convolution at the junction of its upper and middle thirds, there being a paralysis with anaesthesia of arm and leg of the opposite side, and over this region there was great tenderness to per- cussion and to pressure and a marked difference in the percussion note. At the autopsy in this case the bone was so much reduced in thickness over this region as to be translucent, affording a sharp contrast to the skull in other parts. The tumor, however, did not lie in contact with SYPMTOMS. 557 the skull nor upon the cortex, but was at least one inch below the level of the cortex, within the superior parietal lobule and nearer to the falx than to the dura of the convexity. Facial palsy is a symptom which has been observed in abscess of the cerebellum from pressure upon the pons at the exit of the seventh nerve. It is not to be forgotten, however, that in ear disease the facial nerve is frequently afiPected in its passage through the middle ear, and therefore this intracranial symptom must not be overestimated as indi- cating either meningitis or abscess. Optic neuritis is a sign of great value in the diagnosis of cerebral abscess and especially in its differentiation from meningitis. In Okada's cases two-thirds of the patients developed optic neuritis in one or both eyes. It often occurs in thrombosis of the lateral sinus, and therefore is of less value in differentiating abscess from this condition. It is to be remembered, however, that many abscesses of the brain run their course without the development of this sign. It seems evident from these considerations that in the diagnosis of cerebral abscess very little reliance is to be placed upon any one symp- tom. It is the combination of these symptoms, however, and a certain order of their development, which makes the diagnosis clear. It is possible to divide the course of the disease into three distinct periods. These are not sharply separated from one another, but in looking back over a case it is quite evident that they are distinct. The history of the following case will illustrate the course of the disease following otitis media : Male, aged thirty-six years. Had had an attack of scarlet fever at the age of fifteen years, which was attended by an inflammation of the middle ear, leaving a chronic discharge from the right ear which had varied in intensity and severity from time to time during twenty years. This otitis media had not produced any pain and was not attended with any degree of deafness. Occasionally the discharge increased consid- erably and then the ear required some treatment. In March he caught cold, suffered from considerable pain in the ear and from a sudden increase in the discharge of pus. The onset of this acute attack was attended by a rise of temperature, which lasted three or four days and then subsided. The discharge was quite profuse and was treated by antiseptic washings. It lasted about one month and then gradually dried up, so that the specialist under whose care he was discharged him cured of the otitis media, the last week in April. This attack had differed in no respect from numerous preceding attacks, and was not considered of any particular importance by himself or his family, but during this month of treatment he suffered from a constant headache over the right side of the head, the side upon which the ear was affected ; and although this headache was only occasionally intense, yet he was never free from it entirely. During the month he became more and moi-e nervous and irritable, also unduly apprehensive as to his own condition, and at times he acted in a markedly hysterical manner. He 558 ABSCESS OF THE BRAIN. also became gradually more and more depressed and somewhat dull, and on two occasions during the month he vomited suddenly without any nausea or digestive cause. He also suffered occasionally from dizziness, and on several occasions felt faint when the ear was being treated. On April 29th, a few days after the discharge from the ear had ceased, his headache suddenly became intense and he vomited twice. He became very much disturbed in his mind, was nervous, apprehen- sive and irritable, felt faint and dizzy. His temperature was 98° F. and his pulse 60. His tongue was coated thickly, his breath was foul, and he had the appearance of a man suffering from some septic condi- tion, but there was no discharge from the ear, there was no tenderness about the mastoid, there was no tenderness of the head to percussion, the optic disks were clear, the pupils reacted promptly and were equal, the knee-jerks were normal, there was no disturbance of his gait, and although he was complaining he was able to be up and about. This condition continued on April 30th and on May 1st, but on May 2d, after brisk purgatives had acted he seemed to be better, though he was still apprehensive and still suffering from headache, nausea, and som- nolence. His temperature had remained constantly at 98° F. and his pulse had varied from 60 to 80. The only new symptom which had appeared was a dilatation of the right pupil. On May 4th his condition had changed markedly for the worse. He had suffered intensely from headache, the somnolence had increased, and he had become more dull and was aroused with some difficulty, and when aroused was fretful and irritable, and did not talk spontane- ously, but only in response to questions. This somnolent condition alternated with a state of great restlessness, in which he turned con- stantly from side to side, complaining greatly of general discomfort and of pain in the right side of the head. His right pupil was still dilated, and though both pupils reacted to light, the optic disk of the right eye was distinctly congested and slightly swollen ; but careful examination failed to reveal any paralysis, any change in the reflex activity, any disturbance of sensation, and there was still no tenderness over the mastoid and no tenderness to percussion of the head. The temperature was still 98° F. and the pulse 52 to 60, and occasionally irregular. At this time a diagnosis of abscess of the brain was made, and an operation was advised and agreed to. It was thought best, however, to defer it twenty-four hours. On May 5th the general con- dition had become somewhat worse. The temperature was still 98° F., the pulse 52, but varying much in its regularity, though never intermittent. His respirations were regular. He lay in a state of stupor, was aroused with great difficulty, his right pupil was still dilated, the optic disks congested, and the left side of the face was slightly flattened, this being the only evidence of paralysis that could be elicited. The knee-jerks were still normal and equal. He was rest- less in spite of his stupor, and occasionally had an expression of pain jipon his face and would put his hand to the right side of his head as SYMPTOMS. 559 if in pain. In spite of tRe absence of more definite symptoms it was decided to/ make an exploratory trephining over the right temporo- sphenoidal lobe, and preparations were begun for this operation ; but before the operation was begun he suddenly had a spasm of the entire left side of the body, and died. No autopsy was permitted, but in view of the sudden death it seemed probable that an abscess of the brain which had developed subsequent to the ear disease had suddenly ruptured, probably into the lateral ventricle. In reviewing the case it is evident that the first period of the disease coincided with tlie month during which the patient was being treated for otitis media, and during which he had suffered from headache and irregular cerebral symptoms. The second period of the disease, or the period of acute onset, began on April 29 th, and lasted for three days, merging gradually into the third period of termination, the beginning of which was shown by the onset of stupor. A reference to the published histories of abscesses by Bacon ^ and others will show that three periods of similar limit can be distinguished, and therefore it seems evident that in the diagnosis of abscess of the brain the combination of the symptoms in a regular succession of stages rather than the existence of any one symptom is of the greatest aid in diagnosis. The distinguishing characteristics of these periods may be briefly enumerated as follows : The preliminary period, during which slight, irregular, and variable cerebral symptoms, chiefly paroxysmal pain and a dull ache in the head, occasional vomiting, and a lack of power of concentration, with mental fatigue developing easily and unusually, are the chief symptoms. During this period occasional rises of tempera- ture with chilly sensations or even an occasional chill may be noticed, and the patient has the malaise of a slight septic infection. During this period the ear may discharge freely or the discharge may gradually cease. A sudden cessation of the discharge, and a sudden fall of tem- perature to a point below normal tw^o or three days after the discharge has ceased, is a quite common method of termination of this stage ; hence the cessation of a discharge in a chronic otorrhoea, if the discharge has been attended by febrile symptoms for a few weeks or days, and if the cessation is attended by a sudden fall of temperature, the patient continuing to feel ill, with indefinite cerebral sensations, is very sug- gestive of the formation of cerebral abscess. Leucocytosis will be observed early — but in moderate degree not as high a count as in meningitis or thrombosis of the sinus. In the second stage all the symptoms that have been already enum- erated may occur in varying combination and in varying severity : the increasing mental stupor, alternating with irritability and restless- ness ; the general septic appearance of the patient, eitlier with irregu- lar temperature or low temperature and with persistently slow pulse ; more serious disturbance of digestion than can be accounted for by the apparent gastric conditions, and tlic development of objective cerebral 'Handbook of Otology, -OtJi cd. Lea and Febiyer, Philadelphia, 1909. 560 ABSCESS OF THE BRAIN. symptoms ; difference in the size of the pupils ; optic neuritis, facial palsy, increase of reflexes in the limbs of the side opposite to the lesion — all these symptoms are suggestive of cerebral abscess. It is in this stage that operation should be performed if the diagnosis seems reason- ably probable, for if the patient goes on to the terminal state, in which deep stupor is the chief characteristic, the operation will probably be too late. The position of the injury in such cases will indicate the point to be trephined. In abscesses of the convexity of the hemisphere following trauma the local symptoms of lesion will be those already studied as characteristic of cortical lesion, especially unilateral spasm and paralysis or aphasia, hemianopsia, or hemiauBesthesia. There are very few localizing symptoms of cerebral disease in abscesses following otitis media, for the functions of the temporo- sphenoidal lobe, and especially of that part of it which lies next to the petrous portion of the temporal bone, and the functions of the cerebellum, so far as its lateral lobes are concerned, are not fully known. There are apparently few distinct cerebral symptoms that develop uniformly in abscess of these regions; but in abscess of the temporo-spheuoidal lobe of the left hemisphere a certain form of aphasia has recently been observed which deserves attention. It will be remembered that in the first and second temporal convolutions are located the memories of words heard, the relative degree of develop- ment of this function of the cortex varying much in individuals of different degrees of education. It is also to be remembered that in the occipital lobe are located the memories of objects and of words seen. In 1889 Freund ^ described a condition which he named optical aphasia. This consists in an inability to name objects which are seen and recognized and whose use can be described. Sometimes the name can be recollected if other senses — touch, smell, and taste — are called in to aid sight. Freund says : " Granting that the function of naming objects seen — i. e., making an association between the visual pictures of the object and the auditory picture — is performed through the association tracts between the occipital lobe and the speech centres, the explanation of a disturbance of this function is found in a lesion which interferes with the action of the association tract. Such a lesion in right-handed persons must lie in the temporal lobe of the left hemisphere." This localization has been substantiated by Freund by several autopsies. Arnold Pick ^ in a well-observed case of cerebral abscess has described this symptom of optical aphasia fully and made it the basis of a diagnosis of the localization of a cerebral abscess which the operation confirmed. In a case reported by Bacon ^ I had the opportunity of studying this defect of speech. It is alluded to in Bacon's history as verbal amnesia, or an aphasia of conduction. I was unaware of the description of Freund at the time, and the case was 1 Arch. f. Psych., XX., 276. ^Praguermed. Woch., 1896, xxi., 5. ^ New York Medical Journal, August 15, 1896. SYMPTOMS. 561 seen before the appearance of Pick's article. I can confirm fully the description "^f Freund and of Pick, and therefore call attention of English-speaking physicians to this symptom. When the patient is shown objects he knows them, but he cannot name them. He can understand and recognize their names, however. He can talk well and will describe the object, avoiding its name. Thus he will say, when shown a knife, " Yes, I know it ; it is used to cut with," etc. He can repeat the name perfectly. He can often name the object if he handles it or smells it or tastes it, but the name does not come to his mind when he sees the object. I noticed in Bacon's case a further difficulty which this patient, who was very intelligent, also recognized. When the name of an object was given it was not possible for him at once or easily to call to his mind the appearance of the object. In a word, it was as difficult for him to make an association between his auditory and his visual centres as it was to make one between his visual and his auditory centres. This is a feature of the condition not noted by Pick. This symptom of optical aphasia, or intercortical sensory aphasia, as I name it, is due to a suspension of function of an association tract lying in the temporo-occipital lobes and occupying the white matter deep under the cortex. It lies in a position in which it is almost inevitably affected by abscess of the temporal lobe develop- ing after otitis media. This symptom should therefore always be looked for, and may be regarded of highest value in locating the abscess. In left-handed persons it will occur in abscess of the right side. (See Plate XI. and page 136 on Aphasia.) When an abscess in the temporal lobe extends inward it may com- press the internal capsule and thus give rise to symptoms of slowly increasing hemiplegia, hemiansesthesia, and hemianopsia upon the opposite side of the body. In a case of Oppenheim's, operated upon by Jansen, these symptoms were present. There was also a conjugate deviation of the eyes to the side of the abscess. The chief local symptoms of cerebellar abscess are vertigo and a staggering gait. The vertigo is sometimes so severe that the patient cannot raise the head from the pillow, and cannot even attempt to stand. It is often associated with vomiting and sometimes with double vision and nystagmus. The vertigo may lead to a staggering gait, but this may develop without true vertigo. The patient walks like a drunken man, grasps at objects for support, and is unable to walk a line. Sometimes there is a marked tendency to stagger toward one side. This is usually toward the side of the abscess. In cases of cerebellar abscess the pressure exerted upon the side of the pons or medulla may lead to symptoms of cranial nerve involvement upon the side of the abscess. In cerebellar lesions there is sometimes a loss of knee-jerk on the side of the lesion. If there is at the same time a pressure on the pons there will be an exaggeration of knee-jerk on the other side. The a})sence of symptoms of temporal lobe lesion in a case where abscess is probable may lead to an exploration of the cerebellum. 36 562 ABSCESS OF THE BRAIN. Diagnosis. — The diagnosis of cerebral abscess does not appear to be very difficult in ordinary traumatic cases, for there is a history of the original injury, of its exact location, and of the development of a series of cerebral symptoms pointing to a localized disease in the brain. If, then, the location of the wound in the head and the localization of the cerebral disease based upon the nervous symptoms coincide, there is little doubt as to the existence of the abscess. Where there is no history of a cause the diagnosis is much more dif- ficult, as it is sometimes impossible to diiferentiate the condition from acute encephalitis. Thus in a patient seen with James the develop- ment of acute cerebral symptoms during ten days suggested abscess. The patient was a pregnant woman, in the eighth month. No history of any of the causes of abscess could be obtained. She developed exophthalmus of the right eye and a slowly increasing left hemiplegia, with high temperature. The autopsy showed a large abscess on the surface of the right frontal region, but no source of infection could be ascertained. In cases of chronic otitis media four separate conditions may supervene which are often difficult of differentiation. These are cerebral abscess, meningitis, thrombosis of the lateral sinus and acute encephalitis. The relative frequency of the three first of these conditions is about the same. Thus in thirty-six cases, collected by Poulson, of cerebral com- plications of ear disease there were thirteen cases of abscess, twelve cases of thrombosis, and eleven cases of meningitis. In meningitis there is usually a more rapid onset and progress of the symptoms than in brain abscess. In meningitis the headache is asso- ciated with hypersesthesia to sound and to light, and to touch all over the body, symptoms which are absent in abscess. In meningitis the temperature is high, varies between 101° and 104° F., and rarely if ever goes below normal. It does not show the rapid rises and falls which occur in thrombosis of the sinuses. In meningitis the pulse is rapid, irregular, and intermittent, while in abscess it is slow. In men- ingitis there are occasional twitchings or spasm of the limbs, or con- vulsions ; strabismus appears early, trismus is common, and pain and rigidity of the neck are complained of, as the disease advances. These symptoms are wanting in brain abscess. In case of doubt lumbar puncture will reveal the presence of a large number of leucocytes and of microorganisms in the cerebro-spinal fluid in meningitis, but not in brain abscess. The blood examination is of equal value. While any inflammatory state like that present in acute otitis media is likely to increase mod- erately the leucocytes in the blood, a very great and sudden increase in their number is a very valuable indication of a new inflammatory com- plication. Thus, in a case of chronic otitis media we may find 8,000 or 10,000 leucocytes, but if a meningitis or a brain abscess develops, the number rises to 20,000 or even 35,000. Such a rise alone will indicate that we have more than a mere otitis media in our patient. But this is not all. Our data as yet are not very extensive, but I TREATMENT. 563 think we may safely say that a more rapid rise in the number of leuco- cytes and a higher leucocyte count is found in meningitis than in brain abscess. Thus in two cases which I was able to contrast as they pro- gressed simultaneously, and in which the autopsies showed the exact condition, it was observed that in the case of meningitis the leucocyte count on the second day of the disease was 9,000, on the third 11,000, and then rose on the fourth day to 22,000, and remained between 22,000 and 26,000 till death on the fourteenth day. In the case of brain abscess the number rose slowly from 9,500 on the third day of the disease up to 14,000 on the eighth day, but never went up above 15,000 up to death, which occurred one month after the onset. In thrombosis of the lateral sinus high fever with pysemic variations in range and frequent chills are the rule. The temperature in the course of twenty-four hours may twice sink below normal and rise to 105° F. The pulse is very rapid and irregular, but not intermittent. There is tenderness, swelling, and oedema over the mastoid process, and oedema of the neck, and the jugular vein may stand out as a hard blue cord on the side of the neck. Exophthalmus on one side, and even swelling of the conjunctiva, may be seen. There is often a marked venous congestion of the scalp. Choked disk appears early in the course of the case. All of these symptoms serve to distinguish the disease from abscess. The diagnosis of cerebral abscess arising under other conditions is never positive. But when a cause is present and cerebral symptoms such as have been described occur in a definite series of stages the probability of an abscess is very great. Prognosis. — The prognosis will depend wholly upon the possibility of evacuating the abscess and draining it successfully. Treatment. — The only treatment in a brain abscess is surgical. In a collection of 55 cases of abscess of the brain which have been operated upon I found that 28 occurred after injuries, 24 after ear disease, and 3 after typhoid fever. Of these 34 recovered and 21 died. Oppen- heim has recently published more extensive statistics. In 60 cases of traumatic abscess operated upon 38 cases recovered. In 196 cases of otitic abscess 96 were cured. In cases of injury and fracture of the skull trephining should be undertaken early, the local trauma com- bining with the local symptoms to guide the surgeon. Fig. 241 shows the relation of the skull to the convolutions of the temporal lobe and cerebellum, and also the position of the lateral sinus. The rules given by Ballance for trephining in the cases developing after otitis media are given in the explanation of the figure. While the differentiation of abscess of the brain from thrombosis of the lateral sinus and from meningitis is of great interest to the physi- cian, yet it must be clearly stated that it is not absolutely necessary to differentiate these conditions before deciding upon the question of sur- gical treatment. It must be agreed tliat all three conditions are neces- sarily fatal to the patient, and therefore, that any means which may offer a reasonable amount of relief to any or all should be employed at 564 ABSCESS OF THE BRAIN. the earliest possible moment without waiting for absolute diagnosis. If the condition is one of meningitis the patient will die whether operated upon or not, and the operation will not do any harm. If the condition Fig. 241. Lateral aspect of a small adult skull. The illustration shows the relations of the lateral sinus to the outer wall of the cranial cavity and the position of the trephine opening (a), which should be made when it is deemed necessary to expose it. The base line (Reid's) passes through the middle of the external auditory meatus, and touches the lower margin of the orbit ; it is marked out in eighths of an inch, as are also the perpendicular lines drawn from it. The measurements are made along the base line from the middle of the bony meatus. The drawing also shows the convolutions of the temporo- sphenoidal lobe, the Sylvian fissure, and the position of the lower end of the fissure of Rolando {Rol.) X X indicates the site of the tentorium as far as it is in relation to the external boundary of the skull. The anterior x shows the point where the tentorium leaves the side of the skull and is attached to the superior border of the petrous bone, a, trephine opening to expose sinus, five-eighths of an inch in diameter, its centre being one inch behind and a quarter of an inch above the middle of the bony meatus. This opening can easily be enlarged upward and backward and downward and forward (see the dotted lines) by suitable angular cutting bone forceps. It is always well to extend it forward, so as to open up the mastoid antrum (c) and the gutter of the carious bone (if there be one), which leads from the antrum, tympanum, or meatus down to the bony groove. The position of the trephine openings, which must be made for the relief of inflammatory intracranial afiections, secondary to dis- ease of the ear, other than for sinus pyaemia, have been added to the drawing for the sake of contrast and completeness. They are as follows : b, trephine opening to explore the anterior surface of the petrous bone, the roof of the tympanum, and the petro-squamous fissure, half an inch in diameter, its centre being situated a short inch (seven-eighths of an inch) vertically above the middle of the meatus. At the lower margin of this trephine hole a probe can be insinuated between the dura and bone, and made to search the whole of the anterior surface of the petrous, c, trephine opening for exposing the mastoid antrum, a quarter of an inch in diameter, and half an inch behind and a quarter of an inch above the centre of the meatus ; or a quarter of an inch above the centre of the meatus and a quarter of an inch behind its posterior border. The trephine should be directed inward and slightly downward and forward. When a superficial disk of bone has been removed, it is well to complete the operation with the gouge. A larger trephine may with advantage be employed, especially in adults, d, trephine opening for temporo-sphenoidal abscess, half an inch in diameter. Situation recommended by Barker one inch and a quarter behind and one inch and a quarter above centre of meatus. The needle of the aspirator is to be directed at first inward and a little downward and forward. Birmingham prefers one and three-fourths of an inch above, in order to avoid the lateral sinus, e, trephine opening for cere- bellar abscess, half an inch in diameter and one inch and a half behind, and a quarter of an inch below the centre of the meatus. Birmingham prefers two inches behind and one inch below to avoid the occipital artery. The anterior border of the trephine should just be under cover of the posterior border of the mastoid process. The drawing shows that a trephine hole made in this situation is faraway from the lateral sinus, and that the trocar and canula of the aspirator, if directed forward, inward, and upward, would hit an abscess occupying the anterior part of the lateral lobe of the cerebellum, which is the usual site of collections of pus in this part of the braiij, — Fronj Brain Surgery, by Starr, p. 193, (Ballance,) tbjsatment. 566 is oue of abscess or of sinus thrombosis, the operation may give relief, and if one condition is not found the other may be sought for. When it is considered that a few years ago both these conditions were con- sidered necessarily fatal, and when it is known that within the last two years the number of successful operations reported for brain abscess has been large, it is evident that surgeons are appreciating the necessity of this procedure. And when it is further learned that of 419 abscesess collected by me in 1906^ 197 recovered, and of 79 cases of sinus thrombosis 41 recovered, it is evident that surgery has done a great work in the treatment of these conditions. This is not the place to give detailed histories or analyses of these operations. It is evident, how- ever, from their study, that the cases in which success did not attend surgical procedure were those in which the operation had been de- layed too long or had not been thoroughly exploratory. When it is considered that the part of the brain which is usually involved in the abscess, viz., the temporal lobe or the cerebellar hemisphere, may be destroyed without producing any local symptoms, and when it is found that the patients, after the evacuation of abscesses as large as a hen's egg, may recover without the apparent loss of any cerebral function, it seems justifiable at the time of the operation to explore the brain thoroughly in these regions without fear. Large incisions may be made into the base of the temporo-sphenoidal lobe or into the lateral lobe of the cerebellum without doing any harm except from the attend- ant hemorrhage. If the abscess is not found on exploring the temporal lobe it is advisable to explore the cerebellum. Inasmuch as almost all brain abscesses have a thick capsule and contain very thick pus, it is not sufficient in the exploration to merely puncture with a needle or with a small trocar, as the wall may be pushed before these instruments and not punctured. I would strongly urge the use of an instrument devised, I believe, by Horsley for the exploration of the deeper parts of the brain. This instrument re- sembles closely a large ear speculum, but is sharp at the end and is placed upon a scissors-like handle and is split in two parts, so that after its introduction its sides may be separated, allowing a view of the bottom of the cavity into which it is introduced. By pushing such a speculum into the brain tissues gently an incision of the vessels can be avoided, the resistance of the capsule of the abscess more easily de- tected, and the wall can be seen before and during the time of its incision. The question of drainage of these abscesses is also one of great interest. It is to be remembered that the cavity of the abscess in the majority of cases is large and that it will require some time for it to contract or for its walls to approximate, and for the cavity to be obliterated. Unless thorough drainage is maintained during that time the abscess will reappear. The tendency of the brain tissue to collapse is so great that unless the drainage is maintained by a fairly stiff tube it will not be successful ; hence it has seemed to me that a small roll * New York Medical Kecord, March, 1906. 566 ABSCESS OF THE BBAIN. of rubber tissue or a decalcified bone tube is preferable to horse-hair or to gauze. The practical conclusion, therefore, which experience has taught is this : that when a cerebral abscess seems probable from the history of the case and from the symptoms which have developed, and the general progress of the case demonstrates the existence of an increasing and serious focal disease of the brain, it is advisable to operate, even though the symptoms may not be absolutely typical and may present many variations from their usual form. The rapidly increasing percentage of recoveries after operation warrants a degree of boldness, provided the surgeon is careful in the observance of every rule of asepsis, without which precaution such a venturesome operation would be criminal. CHAPTER XXXII. THROMBOSIS OF THE VENOUS SINUSES. The various venous sinuses of the cranium and their communica- tions are shown in Fig. 242. A thrombus may form in any of these sinuses, but it is in the lateral or transverse sinus that it is most com- monly found. Etiology. — Thrombosis is the result of a phlebitis of the wall of the sinus due to an extension to it of an inflammatory process, usually of a septic nature, in the immediate vicinity, viz., from.the dura mater in pachymeningitis, from the bone and veins after otitis media, or chronic nasal disease or orbital disease. Occasionally thrombosis may be secondary to septic processes elsewhere, especially abscesses and carbuncles. In all these cases the thrombus is infected, contains bacteria, and if it breaks up and its particles are carried onward it causes infective emboli in other organs, and pyaemia. In children dying of marasmus, in chronic invalids who have suifered from ex- hausting diseases and have died of heart failure, in a few cases of fatal infectious fevers, and in cases of chlorosis, thrombi have been found in the sinuses without phlebitis, and in these cases they have been ascribed to the state of the blood. The pressure of a brain tumor or of a dural tumor occasionally leads to thrombosis. In a collection of cases by Allport^ 118 out of 128 cases were secondary to otitis media. The majority of cases occur between the ages of fifteen and twenty-five years. Pathology. — The thrombus may be confined to one sinus or even to a portion of it, or it may invade several of the sinuses by gradual extension. It often projects into the veins which lead out of the sinus. It is usually a soft clot adherent to the wall of the sinus. The wall is slightly roughened. If time has permitted, the clot may become hard or even organized. If septic it is foul in odor, broken down, yellowish- green, and purulent, and the adjacent wall is covered with pus. Thus in a patient, operated upon for me by Abbe, with lateral sinus throm- bosis secondary to ear disease, a purulent, fetid, green mass occupied the sinus near the ear, a long clot was extracted from the posterior part of the sinus, another was pulled up from out of the jugular vein, in which a hard thrombus had formed which could be felt in the neck; the wall near the purulent mass was rough, softened, evidently infil- trated with pus. In this case the jugular was tied before the sinus was opened. The patient recovered. ^Journal of the American Medical Association, 1902, p. 690. 567 568 THROMBOSIS OF THE VENOUS SINUSES. In patients who die a congestion of the brain is found in the area whose blood empties into the sinus that is the site of thrombosis, and Fig. 242. an acute hemorrhagic softening with infarction has been found. In these cases also multiple septic emboli are found in other organs. TkEATMEnf. 569 Symptoms. — Thrombosis of the longitudinal sinus is extremely rare and occurs chiefly as the result of septic pachymeningitis or in cases of marasmus. In both conditions its occurrence is rarely diag- nosticated, the only symptom indicative of it being a marked venous stasis in the scalp. When this is present the veins of the entire cranium are distended, including those of the orbit and eye. In a case of Oppenheim's, due to chlorosis, choked disk was seen in both eyes. Nose-bleed has been observed, and bulging of the fontanelle in infants. Thrombosis of the lateral sinus is a frequent complication of otitis media, and its diagnosis rests partly on the prior existence of this dis- ease with its symptoms of earache, purulent discharge, headache, and general malaise. It causes a venous congestion of the mastoid region and oedema over the mastoid, with pain and tenderness ; a swelling and thrombosis in the jugular vein which can be felt as a hard cord in the neck in one-third of the cases, or an unusual emptiness of this vein detected in inspiration (Gerhardt's symptom), a swelling of the cervical glands, pain in the neck on motion of the head and sometimes on swal- lowing, nystagmus and occasionally torticollis, the patient holding the head toward the aifected side to avoid pain. There is a choked disk in over one-half of the cases within the first week. The general con- stitutional symptoms of thrombosis of the lateral sinus are very similar to those of brain abscess, and the reader is referred to that chapter for the few points of distinction which can be made. The disease often begins with a chill and high fever (104°-106° F.), headache, vomit- ing, vertigo, stupor, and delirium, and these are soon followed by local cerebral symptoms, such as hemispasm or paralysis, aphasia, or some affection of the cranial nerves. The fever, as a rule, continues high with frequent intermissions, and, if septic emboli lodge, each gives rise to a chill, a fall of temperature below normal, and a rise to 105° or 106° F. Profuse sweating, rapid, irregular pulse, diarrhoea, jaundice, and local signs in lungs, liver, spleen, and kidneys establish the exist- ence of pyaemia. Death follows in the course of ten days unless sur- gical treatment is efficacious. Thrombosis of the cavernous sinus occurs in connection with nasal and orbital disease. Its local symptoms are oedema and swelling, with venous congestion in the face and about the eye, nasal hemorrhage, exophthalmus, and distention of the retinal veins. Various forms of strabismus invariably appear as the oculomotor and abducens nerves pass through the walls of the sinus. Prognosis. — The prognosis is absolutely fatal excepting in the cases where an operation can be performed in time — viz., in thrombosis of the lateral sinus. Treatment. — The treatment of thrombosis of the lateral sinus is by trephining the bone over the sinus, or by chiselling away the mastoid process and laying bare the sinus, which is then opened freely and cleaned out. It is always well to tie the jugular vein prior to the opening of the sinus. If a clot is found and taken away free flow of blood must be allowed to wash out the remnants of the thrombus, and 570 THROMBOSIS OF THE VENOUS SINUSES. the inner wall of the sinus may be scraped with a small curette. The hemorrhage is easily arrested by pressure, and the spontaneous tendency of the sinus wall to heal is remarkable. The only cases which prove fatal are those in which the thrombus is not entirely evacuated, in which secondary thrombi lead to pyaemia, or in which gangrene of the wall of the sinus leads to a secondary thrombosis. In a collection of 150 cases from recent literature 90 cases were found to have recovered from this operation. For full details of the operation the reader is referred to MacEwen's^ book and to the articles by Koerner^ and Jansen.^ ^Pyogenic Infective Diseases of the Brain, Macmillan Co., 1893. 2 Die otitische Erkrankungen des Gehirns, Jena, 1894. 3 Berliner klin. Wochenschrift, 1891, p. 1160, and 1901, p. 721. CHAPTER XXXIII. TUMOES OF THE BRAIN. Tumors of the brain are not uncommon relatively to other nervous diseases, and are seen both in children and in adults. Collections of cases of brain tumors have been published during the past twenty years by Bernhardt/ Steffan,^ Bramwell,^ Mary Putnam Jacobi/ Mills and Lloyd/ Knapp/ Bruus/ Oppenheim/ and myself.'' It is possible by the study of these collections of cases to make a fair estimate of the relative frequency of different varieties of tumor and of the various situations in the brain in which tumors grow, and also to estimate the probabilities of success in finding and removing a tumor. Without attaching too great importance to statistics on this subject, I have selected as the basis of this chapter, from the collections already named, 600 tumors. These are tabulated in Table XIII., in which tumors in individuals below the age of eighteen years, chiefly children, are sep- arated from those in adults, for the purpose of contrast. In this table no tumors are included which have been removed or in which surgical interference has been attempted. Yet during the past twenty years over 1200 tumors have been operated upon. The percentage of cases open to operation has been variously esti- mated by different authors, and their results are demonstrated in Table XIV. The various authors cited have made collections of cases with post-mortem records, and from a study of the condition found at au- topsy have estimated the probability of success in the removal of the tumor found. In this table I have included 100 cases under my own care, some without autopsies. In 20 of these the tumor was found either at the operation or at the autopsy to be accessible. From this table it is evident that about 9 per cent, of tumors can be removed. Etiology. — Little is known regarding the cause of brain tumor, though in a certain small percentage of cases a history of a blow or fall ^ Beitrage zur Symptomatologie und Diagnostik der Himgeschwiilste, 1881. Also yearly summary in Virchow's Jahresbericht, 1881 to 1909. ^Die Krankheiten des Gehirns im Kindesalter. Gerhardt's Handb. der Kinder- krankheiten, 1880, vol. V. 'Intracranial Tumors, 1888. Edinburgh Medical Journal, December, 1894. * Keference Handbook of Medical Sciences, 1888. * Pepper's System of Medicine, vol. v. ^Intracranial Growths, 1801. ■^ Encyclopadisches .Jahrbuch, 1896. SNothnagel'sallg. Path. u. Therap., 1896. ® Medical News, .Jan. 12, 1889; Intracranial Tumors, Keating's Cyclopaedia of Chil- dren's Diseases, 1890. Brain Surgery, 1893. New York Medical Record, February, 1896. Montreal Medical Journal, Nov., 1897. Journ. Nerv. and Ment. Dis., June, 1903. .Jour. Anier. Med. Assoc, July, 1907. Anier. Jour. Med. Sci., April, 1910. 571 57^ TUMORS OF THE BRAIN. on the head has been obtained. It is known that males are more sub- ject to brain tumor than females, and that persons of all ages are lia- ble to brain tumor. Table XIII. (The first column are children's tumors ; the second column adults' tumors.) Situation. 1 i m s 1 1 CO 9 1 1 O S o o ce 03 Cystic. Carcinomatous. Gummatous. 11 "3 13 6 14 16 19 2 "i 47 34 152 9 2 3 1 11 3 "s 4 41 l' 46 5 7 6 1 3 1 10 "i 15 37 19 11 9 2 "i 2 "s 2 54 "i 5 8 4 5 1 "i "e 25 15 1 "i 1 1 9 2 30 ... 1 ... 1 1 1 t i !!! "2 :::! 1 ...1 3 1 ... 1 2' 10 19 3 2 1 2 "2 "2 7i i ... "i 2 13 1 "3 "3 20 "5 3 11 3 12 4 5 7 "i 1 10 1 41 21 127 35 27 21 38 6 8 5 96 43 300 51 III. Cerebral axis : 1. Basal ganglia and lateral 5 3 5 "i 1 10 3 34 8 2 1 3 1 13 5 86 .34 2. Corpora quadrigemina 14 3. Pons 17 2 9 4 IV. Cerebellum 41 57 300 Table XIV. — Percentage of Brain Tumors Eemovable. Author. No. of Cases. Operable. Reference. 100 100 300 40 53 49 100 29 300 50 23 33 100 10 10 16 2 4 6 3 5 21 3 1 3 20 Pepper's System, 1886, vol. v. Hale White Guy's Hospital Reports, 1888. Starr Medical News, January 12, 1889 (children). Intracranial Growths, 1891. Gray Sajous' Annual, 1891. 1892. Verhand. Deut. Gesell. f. Chir., 1892. Trans. New York Acad. Med., Jan., 1893. Starr , Brain Surgery, 1893 (adults). Edinburgh Med. Journ., June, 1894. Die Geschwultze des Gehirns, 1896. Encyclo. Jahresbericht., 1896. Starr Personal cases to 1903. 1,277 104 Pathology. — The Varieties of Brain Tumor. — Almost every variety of tumor known has been found within the brain. There are certain tumors, however, which are quite common, and others which are very rare. In the first class are tubercular, sarcomatous, gliomatous, gliosar- comatous, cystic, carcinomatous, and gummy tumors. In the second class are fibromatous, angiomatous, myxomatous, osteomatous, and lipomatous tumors, also psammoma, cholesteatoma, and echinococcus cysts. Tubercular tumors of the brain are the most common of all the forms of tumor. They occur with greatest frequency in childhood, being sometimes primary, but usually secondary to tuberculosis of other organs. In 20 per cent, of the cases recorded, tubercular tumors are multiple. This fact should not be forgotten in studying the symptoms PATHOLOGY. 573 of brain tumor and in diagnosticating the location of tlie tumor, for if the tumor is probably tubercular, and if the symptoms are not clearly explicable upon the theory of its location in one part of the brain, it is to be remembered that two or more different tumors in different loca- tions may give rise to a great variety of symptoms. Tubercular tumors vary in size from a small collection of miliary tubercles lying in a mass of thickened pia mater up to a large solid circumscribed mass, with hard, cheesy, or broken-down granular centre and a dis- tinct capsule. Not infrequently the tumor is surrounded by a tuber- cular infiltration both in the brain and its membranes. Sometimes irregularly-shaped deposits of tubercular tissues are found upon the base of the brain, in the meshes of the pia mater, compressing the cranial nerves. (Fig. 243.) Tubercular tumors may be found within the cerebral tissue at some distance from the surface, and no part of the brain can be said to be free from a liability to tubercular deposits. But the large majority of Tubercular tumor in right optic thalamus. Tubercular meningitis of the median surface of the hemisphere. this variety of tumors are found in connection with the meninges and about the large vessels of the brain, so that they are more commonly discovered upon the surface and about the base than lying deep within the organ. When the tumor arises in the meninges meningeal thicken- ing by tubercular deposits is quite common. In a few cases the cranial bones have been eroded in the growth of the tumor. Tubercular tumors are sometimes found in the brain after death when no cerebral symp- toms have been present during life.^ The important facts to be considered in making the diagnosis of ^Edinburgh Hospital Reports, vol. i., p. 420, 574 TUMORS OF THE BRAIN. tubercular tumors are (a) hereditary tendeDcies of the individual to tuberculosis ; {b) exposure to such influences as are known to favor the development of the disease ; (c) the history of symptoms of primary pulmonary, or intestinal, or joint, or glandular affections, and (cZ) the presence of local signs or general evidences of tubercular infection. Fig. 244. Section through cerebrum, showing a large tubercular tumor with thick capsule and granular contents lying beneath the cortex in the centrum ovale of the frontal lobe. When cerebral symptoms develop in an individual with tubercular disease the possibility both of tubercular tumor and of tubercular meningitis should not be overlooked. Sarcomatous tumors are next in frequency to tubercular. They are rarely secondary to sarcoma in other parts of the body, and they are only multiple when they appear in the form of melanotic nodules, which are rare in the brain. Sarcoma is usually encapsulated, round, or oval in shape, hard, approaching fibroma in consistency, and is easily separable from the brain tissue, which it compresses and indents more frequently than it infiltrates. Fig. 244 shows a sarcoma lying on one hemisphere of the cerebellum and Fig. 245 shows a sarcoma lying upon the base between the cerebellum and the pons. The relation of this tumor to the brain and the skull is shown in Fig. 247, which demonstrates the difficulty of reaching tumors of the cerebellum at an operation. ^ ^ American Journal of the Medical Sciences, April, 1893. PATHOLOGY. 575 Round-cell and spindle-cell sarcomata are more frequently met with than myx-^sarcomata or gliosarcomata. These tumors grow rapidly and produce well-marked symptoms which do not vary very greatly in Sarcoma, encapsulated, with pedicle attached to the dura, lying in the posterior cranial fossa, and coiupressing the left hemisphere of the cerebellum. their intensity. They are not vascular, and hence the occurrence of apoplectic attacks during their growth is infrequent. It is not to be forgotten that a sarcomatous deposit in the meninges may occur, pro- FiG. 246. Sarcoma, encapsulated, lying on the base of the brain and compressing the right side of the pons and right hemisphere of the cerebellum. Ihe situation of acoustic nerve tumors. ducing a thick layer of new tissue over an extensive surface of the cortex or base, and in such a case operative treatment is necessarily unsuccessful.' Occasionally a sarcoma has no defined limits, and ' Eskridge, Journal of the American Medical Association, September 30, 1893, 576 TJJMOBS OF THE BBAIN. infiltrates the tissue of the brain. Hence, sarcoma is not uniformly fa voidable for operation. From the study of Table XIII. it is evident that sarcoma may be found in any part of the brain, but it is more frequent in the cortex and in Fig. 247. The situation of a tumor at the base of the cerebellum, shown on a Fraser photograph. This is the situation in which tumors of the acoustic nerve occur. the cerebellum than in any other localities. It is the form of tumor most easily extracted from the skull, and the majority of the successful cases of removal of brain tumor have been sarcomata. PATHOLOGY. 577 Gliomatqus tumors come third in the list. This tumor is a product of the neuroglia and presents the appearance of a connective tissue fibrillary network containing a greater or less number of large branch- ing cells and many small nucleated cells. Glioma of the brain is usually primary, but occasionally may develop secondarily to glioma of the retina. Gliomata vary in density, but are usually softer than sarcomata, and are rarely well defined or separable from the brain tissue, even those which appear to have a definite limit being found, on microscopic study, to be surrounded by a zone of gliomatous infiltration in the brain. The usual consistency of a glioma is about that of the brain substance. There is a tendency in all gliomatous tissues to undergo Fig. 248. Glioma of the upper parietal lobule with infiltration of brain tissue and hemorrhage. (Larkin. ) fatty degeneration, to break down and liquefy, so that cavities filled with clear fluid of a straw color or more sharply-defined cysts are very frequently found in and about a gliomatous tumor, and, in fact, some authors have maintained that all cysts found in the brain, not the relics of previous hemorrhages or of embolic softening, are due to gliomatous formations. A similar tendency to the formation of cavities within gliomatous tissue is manifested in the spinal cord in the disease syringomyelia. Glioma may appear in any part of the brain, but is somewhat more frequent in the white substance of the brain than in the gray matter. As a glif)ma grows it destroys the brain tissue, its branching cells sur- rounding and annihilating both nerve cells and nerve fibres. It doed not compress the brain as a sarcoma does. Glioma is very vascular, and hence the symptoms which it produces 37 578 TUMORS OF THE BRAIN. are somewhat variable in intensity, and, in case the delicate vessels within it rupture, apoplectiform attacks occur. Gliosarcoma is a variety of tumor not very frequent, partaking of the nature both of glioma and of sarcoma, commonly accompanied by Fig. 249. A glioma of the pons and medulla infiltrating the tissues, but not destroying the tracts. The symptoms were chiefly cerebellar. cystic cavities. Like glioma the tumor cells are infiltrated through the brain tissues ; but like sarcoma, its constituents are spindle and round cells, and its density is considerable. It is rarely encapsulated, and, like glioma, is much more difficult of removal than sarcoma. Fig. 250. Gliosarcoma of frontal lobes and corpus callosum. (Francis.) It is questionable whether the origin of a gliosarcoma is the devel- opment of sarcomatous cells infiltrating the brain, and causing an PATHOLOGY. 579 irritation ^nd proliferation of the neuroglia, or whether there is a devel- opment of hard, round connective tissue elements, sarcomatous in character, in the midst of the original glioma. The symptoms are more closely like those "of glioma than of sarcoma, the same variability of intensity being observed. Carcinoma of the brain is usually secondary to carcinoma in other parts of the body, either of the orbit or retina, or of the internal organs or of the breast. Carcinoma is a variety seldom found in children, and usually de- velops in adults above the age of fifty years. In cases in which it has occurred in children it has been from direct extension of the disease from the orbit. It may occur as the primary development of carci- noma in the body, giving rise to secondary infection elsewhere, but this also is rare. Carcinoma grows rapidly in the brain, is not sharply defined, rarely has a capsule, is decidedly vascular, and, of all brain tumors, the one least open to operative treatment. Cystic tumors of the brain may arise either in connection with glioma and gliosarcoma, as already described, or independently, as the result of parasitic infection. Hydatid cysts, echinococcus, and cysticercus are much more frequently recorded in German and Australian medical journals than in this country. Kuchenmeister has made a collection of 88 cases of this variety, in all but 13 of which multiple cysts were found. Of these 49 involved the membranes, 41 were in the cortex, 19 in the white substance, 19 in the cerebellum, 18 in the ventricles, 17 in the basal ganglia, and a few in the corpora quadrigemina, pons, and medulla. These are not included in the tables, as it would give an appearance of undue fre- quency of this form of tumor. These parasitic cysts grow slowly and produce compression of the brain tissue, but do not destroy it. The symptoms which they produce are very rarely so well marked as to enable a diagnosis to be reached, but in cases in which a diagnosis is possible these tumors may be easily removed, as the cyst wall is separ- able from the brain tissue. Several such cysts have been removed in Germany. Gummata of the brain, though very rare in childhood, and never the result of inherited syphilis, are very frequent in adult life, and may develop within a year of the original infection. They may also appear as the only apparent evidence of tertiary syphilis, even twenty years after the disease was acquired. Gumma may occur as a soft gelatinous exudation upon the base of the brain, its favorite position, or anywhere in the membranes, or as a harder and more circumscribed tumor in the meninges, especially of the cortex and cerebellum. It grows rapidly, but also subsides rapidly under specific treatment. In one case under my care, where many symptoms, including (If)uble optic neuritis, were present, the patient was perfectly well in nine months. The occurrence of nocturnal head- ache and insomnia of an obstinate character in any case presenting the 580 TUMORS OF THE BRAIN symptoms of brain tumor will suggest the probability of a gumma, and, in fact, in every case in which the diagnosis of brain tumor is made it is well to subject the patient to a course of treatment by mercury and iodide for a period of at least two months, in order to eliminate the pos- sibility of gumma. Yet it must not be hastily argued that the subsi- dence of symptoms under this treatment is positive evidence that the tumor is syphilitic. In a case under my observation, which autopsy proved to be a small sarcoma with a large cyst, the wall of which was sarcomatous tissue, all symptoms, including optic neuritis, subsided under this treatment, and the patient remained well for a period of five months, and then sudden death in coma occurred unexpectedly. Fig. 251. Malignant chordoma on the base of the brain. Kindness of Dr. Smith Ely JellifiFe. Zeit. f. Neurologie, V. 596, 1911. Chordoma occurs on the base involving the cranial nerves and the bones of the skull. ^ Psammoma is usually the form of tumor occurring in the situation of the pineal gland, and gives rise to symptoms similar to those pro- duced by tumors of the corpora quadrigemina. Other forms of tumor however are occasionally found in this location.^ They produce adiposis and sexual irritation. 8 cases. ' Jelliffe and Larkin. ' Bailej and Jelliffe. Jour. Nerv. and Ment. Dis., Jan., 1912. Arch, of Int. Med., Dec, 1911, 60 cases. SYMPTOMS. 581 Tumors of the pituitary body are not uncommon, and are usually of the natur^ of fibroma or myxoma. In some cases tliey cause acro- megalic, a growth of the bones of the extremities and of the jaw. In other cases they cause a production of fat throughout the body, sexual impotence and infantilism. In both cases bilateral temporal hemi- anopsia and rapidly advancing optic atrophy may be found. ^ Chloroma is a form of tumor recently described. It is a flat greenish colored tumor affecting chiefly the bones of the skull, especially those of the orbit and of the base of the brain, but occasionally appear- ing in various lymphatic glands. It was at first supposed to be a round cell sarcoma of periosteal origin but when its tendency to appear in lymphatic structures anywhere in the body was recognized this view was abandoned. Chloroma is a primary disease of the hematopoetic organs with special tendency to the formation of malignant growths of a green color in various localities but chiefly on the base of the brain. The diagnosis from other tumors affecting the brain is made by the exami- nation of the blood. This has the characteristics of anseniia or of leukaemia in all cases, being lacking in hemoglobin and red cells. The chief peculiarity is the existence in the blood of large lymphocytes, with irregular nuclei which stain with hematoxylin. These cells are four times the size of erythrocytes and are not identical with normal large lymphocytes, being true pathological cells. Myeloblasts and myelocytes and granula are also found in some cases. The symptoms of chloroma begin with the usual symptoms of profound anaemia; pallor, malaise, headaches, general pains and cachexia. Hemorrhages from the mucous membranes are frequent. The pain may first be felt in the eye or ear. Soon exophthalmos appears or a swell- ing in or behind the ear which soon leads to deafness. The skull is enlarged about the orbit or the ear and is very tender. As the tumor increases the eyes bulge extremely or some of the cranial nerves become involved by pressure on their trunks as they pass through the enlarged bone. There are rarely symptoms of pressure directly on the brain. In one case seen by me with Dr. Janeway, both optic nerves, one sixth nerve, both glossopharyngeal nerves and one hypoglossal nerve were paralyzed. Tumors developing in the pharynx or bronchial glands or other glands produce a variety of symptoms. Sometimes the tumor affects the bones of the spine, causing compression of the spinal nerves or even a compression myelitis. In my case extensive atrophy and paralysis of the muscles of one arm were present. Occasionally green warts on the skin appear. The course is a slow one to exhaustion and death in two years from some complication of the ansemia. Other varieties of brain tumor, such as fibroma, angioma, myxoma, psammoma, osteoma, cholesteatoma, lipoma, teratoma, which occur in the brain, are of great rarity. They occur both in children and in adults, and their diagnosis cannot be made from other forms of tumor during life. The appearance of a brain in which a tumor has developed is usually 'Harvey Cusliing. Ilarvey LectureH, 1911, pp. 30-45. 582 TUMOBS OF THE BFAIN. characteristic. The membranes are tense, the convolutions are flat- tened by pressure, the ventricles are distended by serous fluid, the brain tissue is wet, the weight of the brain is increased, and frequently about the tumor there is a zone of softening of greater or less extent, accord- ing to the variety of the tumor. Encapsulated tumors are less likely to be surrounded by a softened tissue. Intracranial aneurisms present the same clinical features as tumor of the brain. They are rarely large in size, they appear upon the larger arteries at the base of the brain and upon the Sylvian arteries. They are usually fusiform, occasionally round. They increase in size more rapidly than ordinary brain tumors, or than aneurisms elsewhere, and usually rupture before attaining any great size. They produce symp- toms by their pressure and are occasionally to be diagnosticated by pulsating headache or the sensation which they produce, or by a dis- tinct murmur audible to the stethoscope on the head. Such a murmur is not, however, pathognomonic, as I have heard such a loud double murmur over the Sylvian region in a case which proved at the opera- tion to be one of extensive softening — no aneurism being found.^ In two cases, one of which is shown in Fig. 252, the rupture of the aneurism caused sudden death, but the aneurism had not produced any symptoms during life. In a case recently seen at St. Luke's Hospital the aneurism developed rapidly after a blow on the head, producing, first, third nerve paralysis, and one month later right hemiplegia. The patient died three months after the injury, having had all the general symptoms of brain tumor. An aneurism of the left internal carotid artery at the point of union with the posterior communicating artery was found, which had ruptured. As the arteries were not diseased, it seemed probable that the bruise of the artery against the bone at the time of the injury had weakened the wall and predisposed to aneurism.^ Symptoms. — While the symptoms of brain tumor are in many cases very clear and characteristic, so that there need be little doubt in regard to the diagnosis, both of the existence of a tumor, of its nature, and of its location, it must be stated that there are other cases in which no characteristic symptoms appear at all. Numerous cases have been recorded in which large tumors have been found unexpectedly after death, even in locations in the brain in which the existence of a tumor would presumably have produced very marked symptoms. Thus, in one case under my observation, a large glioma occupied the entire white substance of the frontal lobe upon the left side, yet the patient had sufl^ered only from occasional attacks of epileptic convulsions, not preceded by an aura, and never unilateral, was as intelligent as ever up to the time of his death, though his family had noticed some irritability of temper at times, and on account of certain peculiarities had occasionally questioned his responsibility. This man was under observation by most competent physicians for ^ Starr, Brain Surgery, Case xix. 2 Beadles, Brain, 1907, p. 285, has analyzed 555 cases of intraci-anial aneurism. SYMPTOMS. 583 several years, and was supposed to have epilepsy. His eyes were ex- amined arfd found to be normal within a few weeks of his sudden death, which took place in a convulsion. He had never had the ordi- nary symptoms of a brain tumor. Schoenthal ^ has recorded a case of supposed hysteria in which the variability of the symptoms was surpris- ing, and in which careful examination failed to reveal any permanent or tangible physical signs. After death a large tumor of the right frontal lobe was found. Mayer ^ has collected several such cases. Fig. 2e52. '/'If i: ' Aneuiisiii of the basilar artery. (Larkin.) Buzzard has recorded similar cases. Bramwell ^ has described an enormous tumor lying in the central region upon the cortex of the riglit side in which there were no symptoms of paralysis whatever. And many tumors have been found in and about the cerebellum which have failed to produce the ordinary symptoms of brain tumor. ' Berliner klin. Woch., 1891, No. 10. See also Williams, Lancet, October 14, 1893. *E. Mayer, Inaug. Dissert., Berlin, 1891. ^ Intracranial Tumors, p. 12. 584 TUMOBS OF THE BRAIN. Tumors upon the base of the brain may also be found involving structures which, during life, appear to have been capable of carrying on their ordinary functions. Thus a child under my observation suffered for five months from occasional convulsions, from headache, from spastic jjaraplegia, but had no difficulty whatever of vision, and no paralysis of the cranial nerves. The autopsy showed a large tumor upon the base in the median line, through which both the optic nerves passed and in which the optic chiasm lay. It must have rested upon all the motor nerves of the eyes and compressed the fifth nerve on both sides. This tumor had grown upward, filling both the lateral ventricles and obliterating the third ventricle entirely.^ (^ig- 253.) I have seen a tumor of the medulla oblongata, an infiltrating glioma, which produced an apparent uniform increase in size of the entire medulla to double its ordinary dimensions, but in which there were absolutely no signs of any disease of either cranial nerves or tracts passing through this important part of the nervous system. The patient had headaches, slight optic neuritis, occasional convulsions, and a slightly staggering gait, which led to the diagnosis of tumor of the cerebellum. (See Fig. 249.) It is thus evident that tumors in the various parts of the brain sub- stance may develop and assume a considerable size without producing characteristic symptoms either of a local or of a general type. Such cases, however, are rare, and the careful observer will usually be able to diagnosticate a brain tumor. The symptoms of brain tumor are very numerous, but for purposes of convenience may be separated into two categories. I. General symptoms due to the existence of a new-growth irre- spective of its position. These are headache, general convulsions, double optic neuritis, and optic atrophy, changes of disposition and of mental power, vomiting, vertigo, insomnia, changes in the pulse rate, attacks of syncope, polyuria, and progressive malnutrition. II. Focal symptoms of the disease in the cortex of the brain or beneath the cortex in the projection tracts which join the cortex to the various subcortical centres. These symptoms are unilateral spasms, monoplegia, or hemiplegia, paraesthesia or anaesthesia in one or more limbs, hemianopsia, and the various forms of aphasia. Affections of the cranial nerves and basal parts of the brain, which occur with tumors in the basal ganglia and cerebral axis, or external to the brain upon the base. These latter symptoms are frequently very complex, as may be supposed, when it is remembered that the twelve cranial nerves have extensive nuclei of origin and a long course, and that all con- nection between the external world and the brain passes through the cerebral axis. In the vast majority of cases of brain tumors we find some of these general and focal symptoms present. And from their combination and the order of their development it is usually possible to arrive at a definite diagnosis. These symptoms must, therefore, be studied with care. ' Transactions Amer. Neurol. Assoc, 1891, Starr, Brain Tumor. SYMPTOMS. 585 I. General symptoms occur irrespective of the location of the tumor and depend upon its rapidity of growth, its vascularity, and its patho- logical character. They vary in severity from time to time probably in accordance with the activity of the pathological process going on in and about the tumor and with the condition of blood supply in the brain. When a tumor is growing rapidly they are very severe ; if it remain stationary they may almost disappear. They are often affected by those agents which produce a temporary cerebral hyper- semia or anaemia, such as hot foot baths or general hot baths. Headache is the most prominent and constant symptom of brain tumor. It varies in severity, but is usually very intense. It may be intermittent, it may recur with periodicity, is usually worse at night Fig. 253. Sarcoma of base separating the crura and surrounding the optic chiasm and greatly stretching the optic nerves. The exit of the optic nerves from the tumor is indicated by the white dots. in syphilitic cases, worse on waking in other cases, is increased by physical effort or mental strain, or emotional excitement. It may be a dull, continuous pain, with paroxysms. It varies in its location, is usually frontal or occipital, and the situation of the pain rarely indi- cates the situation of the tumor. If, however, the pain is constantly located in the occipital region the tumor is probably in the posterior fossa. The pain is often associated with indefinite cerebral sensations described as fulness, pressure, confusion, tightness, as if a band were drawn al:)out the forehead, and these give rise to great discomfort. In infants the existence of headache may be inferred from constant motion of the liead, from movements of the hands grasping the head, or pull- ing the hair, and from sudden outbursts of screaming without other ascertainable cause. Headache is probably less severely felt in cases 586 TUMOBS OF THE BRAIN . of tumor in infants, as the opening of the sutures may prevent the extreme degree of intracranial pressure to wliich the headache is chiefly ascribed. In adults the headache prevents the patient from making any physical or mental exertion, and leads to a "desire for seclusion and quiet. The causes which are supposed to produce the headache are the increase of the intracranial pressure and the consequent stretching of the membranes ; the existence of effusion into the ventricles, which is a frequent accompaniment of a tumor ; the variations in the cerebral circulation produced by the pressure of the tumor, or the direct involve- ment of the sensitive dura mater and branches of the fifth nerve. Such effects of the presence of a tumor are more likely to follow when the new-growth is in the posterior cranial fossa under the tentorium cere- belli, and in such cases headache is most constant and most severe. The headache is often associated with a marked tenderness of the head to percussion, and if this is not due to sensitiveness of a single nerve trunk it is a very valuable sign of the location of a tumor. Such a tenderness to percussion is more commonly found in tumors lying just beneath the bone. Flatness to ausculatory percussion can be de- tected over a tumor in some cases. General Convulsions are the next most frequent symptom of brain tumor. They are particularly liable to occur as an early symptom in children, but may also be the first symptom of tumor in adults. Thus, in a patient of mine from whom McBurney removed a large sarcoma of the frontal lobe, the first symptom was a general convulsion which occurred two months before the headache began, and was never repeated during the three years' course of the disease.^ In another patient under my care, who subsequently developed all the general symptoms of brain tumor, general convulsions occurred at intervals during three months before any other symptom appeared. A case diagnosticated as epilepsy has already been mentioned. It is not uncommon to find convulsions occurring at irregular inter- vals from the onset of the disease to its termination. They may be slight in degree, almost of the nature of petit mal, with a sudden loss of consciousness, or this loss of consciousness may be attended by a little twitching of the face and eyes, with stiffening of the back and extremities, balancing movements of an automatic kind, which prevent falling, and then recovery, or they may have all the general character- istics of a general epileptic seizure followed by coma. Sometimes a peculiar general tremor follows the attack and may last for an hour or more. General convulsions may be a culmination of a local spasm ; hence, wherever a convulsion occurs particular inquiry is to be made as to the manner of its beginning, and the patient is to be carefully observed and instructed to watch for a conscious aura on the next occasion. The significance of local spasms will be discussed in the next section. Convulsions are usually indicative of rapid progress in the new- ^ See American Journal of the Medical Sciences, January, 1893. SYMPTOMS. 587 growth, of effusion into the ventricles, of hemorrhage within the tumor or of a secondary affection of the meninges. They may occur from tumors situated anywhere, and have no significance as a diagnostic symptom of the location. Death not infrequently occurs in convul- sions in brain tumor, and heiice a development or rapid increase of this symptom is a sign of danger. Changes of Disposition and of Mental Power occur in the course of brain tumor with considerable frequency.^ In children this symp- tom is quite noticeable, the child becomes fretful and irritable, refuses to notice its toys or to play, or, if it does so, soon becomes wearied, and requires constant attention. It may become indifferent to things in which it was formerly interested, prefer to lie down and to keep quiet, in a manner unnatural to a healthy child, and may even become som- nolent and lethargic, or it may have apparent attacks of causeless ter- ror. In adults the mental dulness is very noticeable. The patient takes little interest in his ordinary surroundings or business, and is content to sit or lie for hours doing nothing and apparently with a vacant mind. Such a patient is easily aroused and replies intelligently to questions, but cannot be considered in a normal state of mental activity. On the other hand, such a patient is not demented, although late in the course of the disease a condition approaching dementia is quite often, observed, especially in tumors of the frontal lobes. Attacks of maniacal excitement have been recorded, but are very rare. The usual mental state present in brain tumor, after it has existed for a period of six months may be described as one of apathy. These symp- toms are probably referable to an interference with the processes of association upon which all thought depends. Somnolence may be associated with this apathy. In the case of a tumor of the frontal lobes and corpus callosum ^ mental apathy was the chief symptom from beginning to end. (See Fig. 250.) Double Optic Neuritis and Optic Nerve Atrophy are very important diognostic symptoms of intracranial tumor. Neuritis is usually asso- ciated with other signs of increased intracranial pressure, but may occur without such pressure. It is present in 80 per cent, of the cases. A marked degree of optic neuritis may exist without any impairment of vision. When the patient shows impairment of visual power or limitation of the visual field for colors or for light, or is becoming blind, it will be found that optic neuritis or optic atrophy is fully developed. Sudden loss of vision appears to be more commonly noted in histories of children's cases than in those of adults, possible because a gradual loss of sight is not detected. It is true that hydrocephalus may cause choked discs, and hence from this symptom alone a tumor cannot be diagnosticated. But in cases where the diagnosis is difficult no more important objective evidence of brain tumor can be found. A limitation of the visual field for colors and an interlacing; of the blue and red color fields may occur before the neuritis, and always accom- panies it. ' Schuster, P.syclii.sohe Storunpen hei Ilirntnmoren, Stuttgart, 1902. ^ 11. G. Francis, American Journal of the Medical Sciences, June, 1895. 588 TUMORS OF THE BRAIN. Tumors of the cerebellum and corpora quadrigemina and tumors upon the base of the brain and in the basal ganglia produce optic neuritis more constantly and earlier in their course than tumors situated in the cortex or centrum ovale. Tumors within the pons itself are less liable to cause optic neuritis than those outside of the pons on the base. Optic neuritis is usually double, though it always appears first in one eye, and is rarely equally intense in both eyes ; but in a few cases it has been found in one eye only, and then is thought to indicate disease of the nerve in the orbit or in front of the optic chiasm, rather than a distant tumor. For the exact changes in the retina and for the pathological causes of optic neuritis the reader is referred to Chapter XXXV.^ Vomiting is a symptom of brain tumor more frequently observed in children than in adults. It may or may not' be accompanied by nausea. It may occur accidentally, without special relation to the time of meals, or it may be so continuous as to threaten inanition. It occurs not infrequently on any movement of the head after the patient has been confined to bed for some time, and then it is usually associated with vertigo. It also frequently accompanies severe headache. Vertigo is a common symptom. The patient feels dizzy, feels him- self turning or falling, and things about him appear to be in motion. He grasps at near objects for support, covers his eyes with his hands or lies down on the floor and cries out with bewilderment and distress. Like vomiting, vertigo may be excited by changes of position. It occurs at intervals in attacks of short or long duration. It occurs more frequently with tumors in the posterior fossa, in the cerebellum, or pons, or on the base involving the auditory nerve, than with tumors elsewhere. Such attacks of vertigo are not to be confounded with the slight constant vertigo due to double vision, and secondary to paresis of the third and sixth nerves. Insomnia may be due to disturbances of the cerebral circulation or to the intensity of the other general symptoms, and is much moi'e rarely complained of in cases of tumor in children and youth than in adults suffering from syphilitic tumors. Fever and Changes in the Eapidity and Rhythm of the Pulse have been observed in the course of brain tumors. The former is ascribed to inflammatory changes in the brain or meninges as a complication. The latter is regarded as evidence of increased intracranial pressure. Slow and irregular pulse is the form usually noted, but toward the close of life very rapid pulse has been observed. Irregular or Cheyne- Stokes respiration has also been noticed as a terminal symptom. ■ Occasionally attacks of syncope occur in patients with tumor of the posterior fossa, and a general feeling of weakness is not infrequently complained of. Polyuria and glycosuria may develop in the course of brain tumor ^ as a symptom of increased pressure. It has been found 1 See especially Knies, The Eye in General Diseases, William "Wood & Co., 1895. 'Rothmann, Zeitschrift f. klin. Med., 1893, xxiii., p. 339. SYMPTOMS. 589 also in small tumors of the medulla^ as an evidence of irritation of the vagus ffucleus. A rise of temperature in the scalp over the tumor has been observed in a few cases, but is not a uniform or reliable symptom of brain tumor. A unilateral thickening of the cranial bones including the face has been observed in a few cases where the tumor grew from the dura. The combination of several of these general symptoms in any case should lead to a strong suspicion of intracranial tumor. When, in addition, local symptoms are added, the diagnosis should not be diffi- cult. In all cases the onset of the general symptoms is gradual, their intensity increasing as time goes on, and a careful study of their mode of development and progress is a great aid in the diagnosis of the kind of tumor present, of its size, and of the rapidity of its growth. The distress produced by the general symptoms is usually much greater than that caused by the local symptoms, and consequently treat- ment is often required for their alleviation. The Focal Symptoms of brain tumor depend entirely upon the situa- tion of the tumor. A distinction is made between direct and indirect focal symptoms, the first being due to irritation or destruction of a limited area by the tumor, the second being due to interference with the function of an area by disease at a distance from it which impairs its circulation or causes pressure upon it. Thus, a tumor of the cerebellum may cause incoordination and vertigo as direct symptoms, and also cause paralysis of the sixth and seventh cranial nerves as an indirect symptom of dis- placement of the pons Varolii to one side, which stretches these nerves unduly. Tumors usually cause both forms of focal symptoms, and, therefore, much care must be given to the question of their significance in any case. Focal symptoms may further be divided broadly into two classes, those produced by tumors in the cortex and cerebral hemispheres and those produced by tumors upon the base of the brain affecting the cranial nerves. Reference may be made to Chapter V. upon the diagnosis of brain diseases for a special consideration of these vari- ous symptoms. With the anatomy of the brain in mind it is not diffi- cult to understand the various local symptoms that may arise from the existence of brain tumor in different localities. It should be remem- bered, however, that a tumor of very slow growth, which compresses and does not infiltrate the brain may exist in any location without necessarily producing focal symptoms, until it has attained a consider- able size. It is evident that a single tumor rarely, if ever, produces a large number of focal symptoms but that all tumors lying in any given location will produce the same symptoms. It is also evident that tumors will almost always produce unilateral symptoms, inasmuch as they rarely lie exactly in the median line. And even the tumors of the corpus callosum, of which about twenty have been recorded, have ^De Jonge, Arch. f. Psych., xiii., p. 658. 590 TUMOBS OF THE BMAIN. rarely been so exactly median in their situation as to give rise to sym- metrical symptoms upon both sides. The focal symptoms usually commence gradually and are limited in extent, but increase steadily as the tumor grows. The Focal Symptoms of Tumors of the Frontal Cortex may be enumerated as follows : mental inactivity, forgetfulness, lack of judg- ment, decided change in character, irritability of temper and unusual stupidity, an inability to concentrate the attention, to think connectedly and continuously, to learn easily, to exercise self-control, lastly a state approaching mild dementia without delusions, in which the patient may become dirty and disregard all restraints of decency. These symp- toms are especially marked in tumors of the frontal cortex and subjacent white matter.^ They are not present in tumors lying upon the base of the frontal region on the orbital bone. They rarely appear in tumors in other regions of the cortex until the last stage of the disease, when the intracranial pressure is very great. A decided change in character and disposition, a mental apathy, and a tendency to somnolence must be regarded as a local sign of frontal lobe disease. There is no ap- parent way to detect from the mental symptoms in which hemisphere of the brain the tumor lies. It is true that much of our thought is dependent upon the integrity of our speech centres, and hence the lesions of the left hemisphere, if they interfere with the use of language, or even of the recollection of words, appear to give rise to mental confusion, which is not so noticeable in lesions of the right hemisphere. But it is not possible to locate all mental action, as has been done by Phelps,^ in the left frontal lobe. A consideration of the facts known regarding the wide dissemination of memory pictures over the entire hemisphere, and their fundamental importance in all think- ing is sufficient to prove that mental activity is not a function of any one region of the brain. (See page 134.) The diagnosis of tumor of the frontal lobes is, therefore, rarely made from a study of direct focal symptoms. It is to be remembered, however, that a tumor when situated in other regions of the cerebral hemispheres, excepting only the temporo-sphenoidal lobes, will produce other direct focal symp- toms, the absence of which may lead to the suspicion that a tumor is situated in the frontal lobe. Tumors in the frontal lobe frequently produce irritation of the cor- tex. While such irritation is limited to the frontal cortex we have no evidence of its occurrence, but when irritation starts from the frontal region it frequently appears to radiate backward, and thus involve the central region of the brain, and under these circumstances localized spasms, parsesthesia, and paralysis,^ on the opposite side may occur. Stewart has noticed a loss of the superficial abdominal and epigastric reflexes on the side opposite to the tumor, and a marked tremor of the limbs on the same side as the tumor. ^ Ferrier, Lancet, June 4, 1892. 2 American Journal of the Medical Sciences, April, 1902, ^ See a case of mj own, shown in Fig. 239. SYMPTOMS. 591 Bruns ^ has carefully recorded three cases of frontal tumor in which there had been some disturbance in the balancing power and a stagger- ing gait similar to that observed in cerebellar disease ; but this symp- tom has not been uniformly noticed. It is known that the frontal lobes and cerebellar hemispheres have a crossed connection,' and it is possible that this symptom may prove of some diagnostic value if con- firmed. It may have been due to an irritation of the cerebellum con- veyed along these connecting tracts. Tumors situated in the third frontal convolution of the left hemi- sphere in right-handed persons and of the right hemisphere in left- handed persons produce motor aphasia with agraphia, the patient being able to understand language both written and spoken, but being unable to give expression to his ideas. When this symptom develops as the result of brain tumor the disturbance of speech is gradual in its onset, the patient noticing first a hesitancy in speech, a loss of words, and possibly a misuse of words before he loses the power of expression. The aphasia is less complete in brain tumor than in those apoplectic conditions which are the common cause of aphasia. Tumors Involving the Motor Area of the brain give rise to spasms or paralysis. The special functions of the motor area are displayed in Figs. 34 and 35, and the symptoms that are produced by a tumor begin- ning in any portion of this area are easily deduced from reference to these figures. If the tumor is cortical it almost inevitably gives rise to tonic or clonic convulsions, occurring at intervals, either limited to one part of the body, such as the face, or hand, or foot, or extending from the part first invaded to other parts in a definite order of succes- sion, the extension being commonly from face to arm, to body, and then to leg, or in the reverse order. Such attacks, first described by Hugh- lings Jackson, are known as attacks of Jacksonian epilepsy. If it be remembered that the irritation starting from one part of the cortex and radiating outward to other parts produces a spasm beginning in the muscles represented in the centre first irritated, and extending to other muscles represented in adjacent centres, it will be evident that the exact order of the spasm aids one to determine the situation of the tumor. A convulsion beginning with the turning of the head and eyes to one side indicates a tumor situated in the anterior middle portion of the motor zone, and if that irritation extends from this point backward or downward the spasm will extend from head and eyes to arm or face, respectively. The situation of several tumors which produced such local spasms is shown in Figs. 36 to 44, pages 93 to 95. Therefore, the point of beginning of a spasm and its order of ex- tension are the most important localizing symptoms of brain tumor in the motor area. Such a spasm is usually followed by temporary paralysis, and as the tumor increases in size and the spasm increases in frequency and extent, the paralysis may remain permanent between the spasms. Spasms due to cortical irritation are almost invariably attended by numbness and tingling in the part first affected by ^ Deutache medicinische Wochenschrift, 1892^ p. 138. 592 TUMOES OF THE BEAIN. spasms, so that such a tingling is often a valuable indication of the location of irritation in the cortex. This tingling may even precede the spasm, and has been termed by Seguin the " signal symptom " of a cortical irritation. When such a paraesthesia is a permanent symp- tom the tumor is more likely to be situated behind the fissure of Rolando than in front of it. When the irritation produced by a tumor in the motor zone is very intense, the local spasm after extend- ing from one limb to the entire side of the body may even become general, be associated with loss of consciousness, and terminate as a general convulsion. While such an order of extent preceding the general convulsion is of great diagnostic importance, a general convul- sion beginning suddenly, with loss of consciousness and without any local spasm, is not to be regarded as indicative of disease in the motor area. There are cases of brain tumor in which the motor area has been generally compressed and destroyed without the production of these local spasms, but with a progressive and increasing paralysis alone. It is thought that such a paralysis not associated with spasms indicates that the situation of the tumor is in the white matter beneath the cortex and not in the cortex itself, and that the symptoms are due to an invasion of the motor tract in its passage toward the internal capsule. Some ataxia is commonly associated with the paralysis pro- duced by tumors, and a marked inco5rdination or disturbance of the muscular sense, like anaesthesia, points to a situation of the tumor behind the fissure of Rolando. The condition of the deep reflexes is altered in all cases of tumor in the motor area ; a marked increase in the tendon reflexes at the elbow, wrist, knee, and ankle is one of the early signs of paralysis. There is no atrophy in the paralyzed muscles, but merely a slight wasting from disuse, and there is no change in the electric reaction of the muscles. There should be no difficulty in differentiating a cerebral paralysis, even of the monoplegic type, from spinal or nerve trunk paralysis, even in infancy, because of these points of contrast. The careful study of the localizing symptoms of tumors of the motor region has resulted in successful localization of such tumors and in their successful removal from the brain ; in fact, there is no region in the brain in which a tumor is more easily access- ible or more possible of early diagnosis than in the motor area. Tumors of the Parietal Region, including the superior and inferior parietal lobules, are not uniformly attended by distinctive and local symptoms, but in a considerable number of cases disturbances of sen- sation and of muscular sense in the limbs of the opposite side have been observed. Tumors in the posterior central convolution are more liable to produce sensory symptoms than those in the anterior central convolution. Tumors lying in the parietal region cause sensory symptoms by pressure upon the sensory tracts on their way from the posterior portion of the internal capsule to the central region of the brain. If this is so, then ataxia and anaesthesia appearing in con- nection with a tumor of the parietal region would be an indirect local symptom rather than a result of a lesion in the sensory centres them- SYMPTOMS. 593 selves. The question of the existence of a muscular sense as distinct from the sslisorj centres and the motor centres has been discussed on page 109. The symptom of word-blindness is almost uniformly due to a lesion of the inferior parietal lobule in the left hemisphere in right-handed people and in the right hemisphere in left-handed people. A reference to Plate XI., and to the discussion of aphasia on page 136 will show the importance of this symptom. Tumors lying in the lower half of the parietal region are necessarily near to the visual tract which passes outward from the internal capsule to the occipital lobe, and hence, defects of vision of the form of bilateral homonymous hemianopsia have been recorded in connection with tumors in this locality. Tumors of the Occipital Region of the brain give rise to the important local symptom of lateral homonymous hemianopsia. (See page 120.) Henschen's researches show that a lesion at almost any portion of the occipital lobe, if sufficiently deep to compress the white matter of this region, will produce hemianopsia, and it is to be expected that in any lesion of the nature of a tumor in which small areas of tissue are not destroyed, but considerable areas are primarily aifected and ad- jacent regions are strongly compressed, symptoms of hemianopsia will be frequent. The occipital lobe is easily accessible to the surgeon, and therefore it is important that tumors here should be discovered as early as possible. It is not generally appreciated that hemianopsia is a symptom often entirely overlooked by a patient, a case having been recently reported by Bleuler ^ in which, though well-marked hemian- opsia existed, the patient was entirely unaware of any visual defect. It is therefore exceedingly important that the extent of the visual field in both eyes should be carefully tested in every case of suspected brain disease, each eye being tested separately. Lesions of the occipital lobe are capable of producing a disturbance known as blindness of mind, in which the patient, though seeing an object, no longer recognizes it as having been previously known. This is a condition allied to word-blindness, in which the patient is sud- denly deprived of the power of reading. This condition appears to be more frequent in lesions upon the left side of the brain in right-handed persons, and upon the right side of the brain in left-handed persons. It may also occur when a lesion is entirely subcortical, as well as when it is in the cortex of the occipital lobe. This symptom has been discussed in connection with aphasia (pages 122 and 137). Tumors of the Temporal Lobes or tumors lying upon the base of the cerebral hemispheres above the tentorium or upon the petrous portion of the temporal bone give rise to few recognizable symptoms. In a few such tumors disturbance of taste and smell seem to have been present when the aj)ex of the temporal lobe had been invaded by the givnvth, and when tlie uncinate gyrus had been destroyed. There are no known symptoms produced by disturbance of the lingual lobule. 38 ^ Archiv. f. Psychiatric, vol. xxv., p. 39. 594 TUMOLS OF THE BEAIN. Tumors invading the first and second temporal convolution upon the left side in right-handed persons and upon the right side in left-handed persons produce the form of aphasia known as word-deafness, in which the person is unable to recall the names of places or persons, and can- not understand what is said to him. They may also cause inter- cortical-sensory-aphasia. (See page 138.) It is believed that the first and second temporal convolutions are the termination of the auditory tract, and that each hemisphere is related to both ears. Deafness of cerebral origin is, however, rare, and is not often observed in tumors of these convolutions. Tumors Lying Within the Sylvian Fissure and upon the island of Reil produce numerous symptoms on account of their pressure upon adja- cent parts ; thus, by pressing upon the third frontal convolution they cause motor aphasia ; by invading the operculum they produce facial paralysis ; by pressing upon the inferior parietal lobule or the superior temporal convolution they may produce various symptoms of sensory aphasia, and by direct pressure upon the island of Reil and upon the underlying external capsule, they may cause paraphasia. If this pres- sure is transmitted more deeply to the internal capsule symptoms of tumor in this region may also be caused. Tumors Lying in the Median Surface of the brain, upon the gyrus fornicatus, gyrus marginalis, or upon the hippocampal convolution are not easily diagnosticated. Ferrier believes that in the latter convolu- tion are situated the tactile centres. The majority of physiologists (Munk, Horsley, Schaeifer) and of pathologists deny this, and recently a case of glioma of this region without sensory symptoms has been reported by Humphrey.^ Tumors in this region are of rare occur- rence, and no focal symptoms can be assigned to them as yet. Tumors of the Corpus Callosum are not at all common, only twenty cases being so far recorded in literature.^ The symptoms in a majority of these cases have been chiefly general symptoms, viz. : headache, vomiting, optic neuritis, epileptiform attacks, physical inertia, hemiparesis, and disturbance of intelligence, principally in the form of dementia. These were the symptoms in one case under my observation. The last-named symptom is the only one that appears to be of constant occurrence in these cases, and in many of them it has been preceded by symptoms quite comparable to those of hysteria. The majority of cases of tumor of the corpus callosum have therefore resembled tumor of the frontal lobes. When tumors occur in the cor- pus callosum they are more likely to be situated far forward about its knee, rather than in its thinner posterior part. (See Fig. 250.) Our knowledge of the function of the corpus callosum is very im- perfect, though it is evident from its structure that it associates the 1 British Medical Journal, August 27, 1893. 2 Bruns, Berliner klin. Woch., 1886, Nos. 21 and 22. Bristow, Dis. of the Nervous System, p. 271. Lutzenberger, Neurol. Centralb., 1890, p. 251. Berkley, American Journal of the Medical Sciences, June, 1890. Oliver, University Medical Magazine, April, 1891. Francis and Starr, American Journal of the Medical Sciences, June, 1895. * Bruce, Brain, xii., p. 171. SYMPTOMS. 595 action of the two hemispheres. The corpus callosnm has been wholly wanting in the brain in a few cases, and although this is occasionally associated with imbecility it is a condition which has been found in persons who presented no symptoms during life.^ It is evident, there- fore, that tumors in this locality cannot be positively diagnosticated, and the situation of such tumors deep between the hemispheres forbids the possibility of their removal. Tumors of the Basal Ganglia, including the lenticular and caudate divisions of the corpus striatum and the optic thalamus, are not very rare. Our knowledge of the functions of these ganglia is very imper- fect, and the necessary loss of function which must occur when they Fig. 254. Sarcoma of the left optic thalamus and internal capsule. Cyst in the centre of the tumor. are destroyed by softening cannot be detected during life. Tumors in this region almost always produce pressure upon the internal capsule through which pass the most important motor and sensory tracts, and which lies between these ganglia. Hence symptoms of the nature of hemiplegia, hemiataxia, hemiansesthesia, and hemianopsia are observed when the tumor invades one or more of these tracts. (See Fig. 53.) It may then be possible to detect the progressive growth of a tumor here by the succession of symptoms produced, one ' Bruce, Brain, xii., p. 171. 596 TVMOBS OF THE BRAIN. tract after another being invaded by the disease. Motor symptoms are more common in cases of tumors of the corpora striata. Sensory symptoms occur in cases of tumors of the optic thalami. When a tumor invades the posterior part of one thalamus, homonymous hemi- anopsia is an invariable symptom. This will be easily understood by reference to Fig. 58. It has been supposed that lesions in the optic thalamus give rise to disturbances in the recognition of the position of the limbs of the op- posite side, and that in consequence awkward positions are assumed and awkward movements, athetoid in character, are made (Meynert). This I can confirm. In the patient whose tumor of the optic thala- mus is shown in Fig. 254 there was noticed a peculiar forced posture Fig. 255. Teratoma of pineal gland, showing secondary hydrocephalus. Kindness of Dr. Pearee Bailey of the limbs when he was at rest. The leg was thrown back and out with the toe pointed down, and the hand was held up above and in front of the head. This symptom persisted for many weeks until total hemiplegia gradually developed. It has also been affirmed that a tremor quite similar to that of multiple sclerosis may be caused by a tumor of the thalmus.^ It has been noticed that disturbances in facial expression and in the natural play of facial feature in speech and thought have been noticed in patients suffering from disease of the optic thalamus.^ The face is affected on the side opposite to the lesion and does not act in smiling, but can be moved voluntarily. It has been thought that certain inhibitory functions are exercised by the thalamus, and that undue emotional reactions occur when it is diseased. 1 D. H. Cooke, Lancet, :^Iay 28, 1892. ^Kirilzew, Neurol. Centralbl., 1891, p. 310; Nothnagel, Zeitschrift f. klin. Med., 1889 ; Bechterew, Neurol. Centralbl., 1884, p. 102. SYMPTOMS. 597 Disturbances in the body temperature have been observed in a few cases. ^ Cases are on record to support each of these statements, yet it must be admitted that numerous negative cases, that is, cases of tumor of the thalamus in which these symptoms are wanting, throw much doubt on the diagnostic significance of the effects mentioned.^ My own view of the functions of these ganglia is that they preside over many acts which are commonly performed in a purely automatic manner, for example, the posture of the body, the ordinary gait, the facial expres- sion, and probably emotional control ; that these acts can, however, be performed voluntarily as well as automatically, and hence, when the automatic mechanism fails, a volitional act takes its place, which prac- tice soon perfects, leaving the individual quite unconscious of his defect, and hence causing few or no symptoms. It is, therefore — on this theory — clear that tumors of the basal ganglia cause few recognizable symptoms. Tumors of the Corpora Quadrigemina present symptoms of a focal kind which are characteristic.^ These consist of a staggering gait similar to that seen in cerebellar disease and symptoms of paralysis or paresis in the muscles controlled by the ocular nerves. The co5rdi- nation in movements of the eyes, the reflex motions of the eyes in fol- lowing a light or turning toward a sound, and the power of steady gazing at an object are controlled by automatic centres in the corpora quadrigemina. Disease here frequently produces nystagmus and in- terferes with these automatic movements. The symptoms are not referable to a disease of one third nerve alone, for the pupils are often unaffected. They are often bilateral and more nearly resemble the symptoms of ophthalmoplegia externa. Hence, it is possible to distin- guish lesions of the corpora quadrigemina from lesions in the crus cerebri, which cause true third nerve paralysis. The eyeballs are not often equally affected, one being more paralyzed than the other ; but neither eyeball, as a rule, is totally paralyzed. The superior and in- ferior recti are more commonly affected than the lateral muscles. It is the combination of these ocular symptoms with the reeling gait which will lead to the diagnosis of tumor of the corpora quadrigemina when the other (general) symptoms of brain tumor are present. The focal symptoms of tumors at the base of the brain are necessarily very complex. Suffice it to say that tumors lying upon the base of the brain will necessarily compress one or more of the cranial nerves, either upon one side alone or upon both sides, in case the tumor is near the median line. The situation of the tumor can usually be determined by noting the order in which the various cranial nerves are invaded from first to twelfth. (See Figs. 52 to 56, and 77.) The reader is also referred to Cluipter XXXIV., for the consideration of lesions at the base, and to Cliapter XXXV., for lesions of the cranial nerves. Tumor of the Crus Cerebri is characterized by the combination of ' Cowan, Lancet, December, 1892 ; Lloyd, Med. News, January, 1892. 2 Wharton Sinklcr, T'nivcrsity Med. Mafr., October, 1893. "Nothnagel, Brain, xii., 21 ; Hall, Heidelberg Dissert., 1892. 598 TUMOBS OF TEE BBAIN. hemiplegia of one side with third nerve paralysis of the opposite side. The tumor lies on the side on which the third nerve is affected. To this combination bilateral hemianopsia may be added if the tumor com- presses the optic tract. ^ Tumors in the Upper Part of the Pons or in the crus cerebri affect the third and fifth nerves, producing external strabismus, with dila- tation of the pupil and ptosis, and also tingling, pain and ansesthesia of the face with ulceration of the cornea, and sometimes grating of the teeth during sleep. Tumors in or near the lower half of the pons involve the sixth and seventh and eighth nerves, causing internal strabismus with contracted pupil, paralysis of the face including inability to close the eyes, and deafness, with vertigo. Alternating hemiplegia is also produced by a lesion in this region. Tumors in the Medulla may disturb the action of the glossopharyn- geal, pneumogastric, spinal accessory, and hypoglossal nerves, pro- ducing difficulty in deglutition, irregular respiration, irregular pulse, flushing of the skin, with sweating, sometimes unilateral, projectile vomiting, polyuria, or glycosuria, retraction of the head or rolling of the head upon the pillow, and lastly, paralysis of the tongue, with in- ability to articulate distinctly or to swallow. When the tumor lies upon the base of the brain, it not only invades the cranial nerves, but it presses upon the various tracts which traverse the crura, pons, and medulla. Thus it may give rise to hemiplegia or hemiataxia or hemiansesthesia, according to the tract invaded. These symptoms are easily understood by reference to Figs. 52 to 56. Tumors of the pons, producing pressure upon the middle peduncle of the cerebellum, produce a tendency to stagger in walking. The patient usually staggers toward the side opposite the tumor, but this is not an invariable rule. Tumors of the pons quite uniformly pro- duce a loss of the tendon reflexes at the knee. The control of the bladder and rectum is also frequently impaired. Tumors of the Cerehellum are exceedingly frequent both in children and in adults, and present almost all of the characteristic general symptoms of brain tumor. The situation of the cerebellum held down in a small cavity by the tentorium cerebelli is such that a small growth in the posterior fossa is capable of producing an increase of intracranial pressure quite early in the course of the case. As a result of such compression an accumulation of fluid in the ventricles is usual. Hence, symptoms appear earlier in the course of the disease in tumors about the cerebellum than in tumors elsewhere in the brain. Head- ache, occipital or frontal, is usually the first symptom, and optic neur- itis appears very early in the course of the case. The percentage of cases having optic neuritis is much greater in tumors of the cerebellum than in tumors in the cerebral hemispheres. Nystagmus is also an early and constant symptom. The focal symptoms of tumors of the cerebellum are quite char- acteristic. They are staggering in walking, sometimes attended by a 1 Venal, Bullet, de la Soc. Anat., Paris, Jan., 1891. SYMPTOMS. 69S strong tendency to stagger or to fall in a particular direction ; vertigo ; cerebellar Seizures ; abnormal positions of the head and body ; and symptoms referable to a compression or displacement or stretching of the cranial nerves or basal arteries lying in the posterior fossa.^ Tumors in the middle lobe of the cerebellum uniformly produce cere- bellar ataxia or a staggering gait. Tumors in the hemispheres of the cerebellum near to and compressing the middle lobe are attended by the same symptoms. So also are tumors which compress the peduncles of the cerebellum, especially the middle peduncles in their course to the pons, or the superior peduncles on their way toward the corpora quadrigemina. Tumors lying within the hemispheres which do not invade these parts do not always produce a staggering gait. Hence, a diagnosis of a tumor of the cerebellum is easy when the tumor lies near to the median line, but is difficult when it lies near the surface in the lateral portion of the hemispheres. Thus in the tumor shown in Fig. 245 there was no staggering ; while in the tumor shown in Fig. 246 the patient staggered toward the left side. The early ap- pearance of staggering as related to the general symptoms is, there- fore, an important sign in favor of a cerebellar tumor, while the appearance of staggering many months after the development of optic neuritis will merely indicate that a tumor in the hemisphere has finally reached or encroached upon the middle lobe. The characteristics of cerebellar ataxia are a staggering gait with steps of irregular length and position, the body swaying like that of a person intoxicated. The ataxia of the legs and of the body is attended by a decided sense of vertigo, and does not usually persist when the patient lies down. The ataxia of the hand is often attended by a tremor. Stewart and Holmes ^ in a study of the vertigo in forty cases of cerebellar tumor reach the conclusion that in cerebellar tumors the sense of displacement of external objects in front of the patient is from the side of the lesion to the opposite side ; thus if the tumor be on the left side objects seem to move to the right. They found that in intracerebellar tumors the sense of self-rotation is from the side of the lesion to the healthy side. But in extracerebellar tumors the sense of self-rotation is from the healthy side toward the side of the lesion. The sense of self-rotation leads to an attempt to correct the supposed position, hence the patient may roll over in bed or may stagger in one direction. Such a uni- lateral staggering is not a common symptom. These characteristics, together with the fact that the knee-jerks are often exaggerated in cere- bellar tumor, will enable anyone to distinguish this disturbance of gait from that appearing in locomotor ataxia. Occasionally, however, the knee-jerk is lost on the side of the tumor. Cerebellar ataxia is due in part to a disturbance in the mechanism of equilibrium in so far as this depends upon impressions coming to the brain from muscles of the trunk and legs. The direction of staggering is not an indication of the side on which the tumor lies. ^ See Mills and Frazier, New York Medical Journal, February 11 and 18, 1905, 2 Brain, 1904, p. 526. 600 TUMOBS OF TEE BEAIN. Cerebellar staggering is also due to a cessation of outward impulses which hold many of the body and neck muscles in proper tonic con- traction. An abnormal position of the head and body occurs in cere- bellar disease. The head is turned back and down to the side of the tumor and is held very rigid on any change of posture. When standing the foot on the side of the tumor is thrown outward and the shoulder elevated to help balance the body. Batten^ observed that a patient with a tumor of the right lobe of the cerebellum carried his head inclined toward the left shoulder and Mills ^ has seen the same symptom. I have often seen abnormal postures of the head and body with cerebellar tumors but do not think that the direction of the head is a definite sign of the side on which the tumor lies. Sudden relax- ation of the muscles of the legs may occur, leading to falls which have been termed cerebellar attacks. They are often attended by a loud tinnitus, vertigo, sudden blindness or a loss of consciousness, and tonic spasms lasting several minutes. Sometimes the patients fall to one side, or forward.^ Bablnski has named this symptom hemiasynergia. When the middle peduncle of the cerebellum is invaded there are usually cranial nerve symptoms produced, and these are always upon the side on which the tumor lies. Hence the combination of the staggering toward one side with cranial nerve symptoms of the opposite side will indicate inevitably which peduncle of the cerebellum is involved. Thus in the case shown in Fig. 236 the diagnosis was made before death from this combination of symptoms. A deviation of the eyes called " skew deviation " has been seen in some cases, one eye directed down and inwards, the other upwards and outwards. Another symptom of cerebellar origin is an imperfect muscular tone in the muscles of the arm and leg on the side of the tumor. It is uniformly admitted that the cerebellum has a direct tonic influence upon the muscles, and that this influence is homolateral. This lack of tone is attended by an awkwardness of movement not due to any actual weakness, but to an imperfect co5rdination of the motions performed. Thus, rapid movements made by both hands or fingers together often prove that the hand on the side of the lesion is moved less freely, rapidly, and skilfully ; and other tests may show a true ataxia. There may be also an ataxia of the leg, so that to stand on one foot is impossible. Such an ataxia in the leg may lead to stagger- ing toward the side of the tumor. This symptom has been named diadokokinesis. The maintenance of a proper tone in the muscles appears to have some relation to the response of those muscles to irri- tation, and it is probably on this account that lesions of the cerebellar peduncles or of the cerebellum are usually attended by a loss of knee- jerk and of the deep reflexes on the side of the lesion ; yet this symptom of a loss of reflex on the side of the tumor is not always found in the cases of pontine angular growth. It has been observed, however, in too many cases to allow us to overlook it. 1 Brain, Part 101, 1903. 2 New York Medical Journal, February 11, 1905. ' New York Medical Journal, February 11, 1905. SYMPTOMS. 601 Tumors of the Cerebello-pontine Angle. — Tumors of this locality are not un(5ommon. The symptoms which indicate the location of a tumor in the cere- bellar pontine angle or upon the acoustic nerve may be divided into three classes : (1) Those referable to the cranial nerves ; (2) those refer- able to the involvement of the cerebellar peduncles ; and (3) those refer- able to compression of the tracts passing through the pons. 1. Symptoms Referable to the Cranial Nerves : (a) Slight anaesthesia of the face on the side of the tumor, first shown by corneal anaesthesia and an absence of the corneal reflex. (6) Weakness of the external rectus muscle of the eye on the side of the tumor, due to a pressure on the sixth nerve. This may be first noticed by the occurrence of nystagmus on turning the eyes toward the side of the tumor, and later a decided internal strabismus may occur. The eye looks away from the tumor. (c) A weakness of the muscles of the face on the side of the tumor, first noticeable in a slight disturbance of the act of winking and a slight lack of expression, which is later followed by an actual weakness of all the muscles supplied by the seventh nerve. Pressure on this nerve is rarely, if ever, sufficient, however, to cause a reaction of degeneration in these muscles. (d) Tinnitus in the ear on the side of the tumor, soon followed by some deafness in the ear, which may be slight and only detected by tests with the Galton whistle, high notes or low notes, or both, being lost. This deafness may be as marked for bone conduction as for ear conduction. (e) Vertigo, felt by the patient as a sensation of falling toward one side, or as a sensation of rotary movement of the body, both of which symptoms must be ascribed to irritation of the labyrinthine division of the auditory nerve. (/) Difficulty of swallowing and hoarseness of the voice, owing to pressure on the ninth nerve. ( g) Sudden attacks, which may be called vagal attacks, referable to irritation of the tenth nerve, consisting of rapid pulse, sensations of flushing of the body or of the head, sudden faintness without an actual loss of consciousness, attended by more or less vertigo. (Ji) Paralysis of the tongue, shown by thickness of speech or dif- ficulty of articulation, and by protrusion of the tongue toward the side of the tumor. 2. Symptoms Referable to the Involvement of the Cerebellar Peduncles. — A staggering gait and feeling of uncertainty of the position of the body in space when supported upon the legs, which causes swaying when the eyes are closed and staggering when the eyes are open, at- tended by a disagreeable sense of vertigo, which leads the patient to seek support whenever in a. standing posture. The direction of the staggering is, in the majority of instances, away from the tumor, al- though cases have been put on record in which the patient staggered toward the side of the tumor. The direction of the staggering is not B02 TVMOBS OF THE BMAIN. to be taken in itself as a very important symptom, but the fact that the patient staggers uniformly toward one side is an important symptom of an aflfection of the peduncles of the cerebellum as they enter the organ, and therefore the existence of staggering in a definite direction, com- bined with evidences of the implication of the cranial nerves upon one side, is a definite local sign of a tumor in the cerebellar pontine angle. A loss of muscular tone and abnormal positions of the head are present in these cases as in cerebellar tumors. 3. Symptoms Referable to Compression of the Tracts Passing Through the Pons. — A third set of symptoms is to be traced to a com- pression of the motor tracts and sensory tracts passing through the pons and medulla on their way to and from the limbs of the opposite side. Compression of the pons is very much less likely to cause disturbance in the transmission of sensation than in the transmission of motor im- pulses, and therefore weakness of a hemiplegic type in the arm and the leg in the side opposite to the tumor is more commonly observed than is anaesthesia in the corresponding parts. This weakness is usually attended by an exaggeration of the reflex, by an increase in the knee- jerk, ankle clonus, and Babinski reflex, and such exaggeration is doubly evident because of the loss of the knee-jerk upon the side correspond- ing to the tumor. This weakness of one leg may intensify the diffi- culty of walking and may influence very markedly the gait. A patient with a weak leg has something of a tendency to stagger toward the leg that is weak, to use it less freely, and to bend downward toward that side. It has already been remarked that many of these patients stag- ger away from the tumor, and it is not at all impossible that this direc- tion of the staggering is due to the imperfect use of the leg on the side opposite to the tumor. The actual weakness present in the arm or leg may be easily detected by dynamometer tests, and in many cases has amounted to such a marked hemiplegia that any use of the arm or of the leg was impossible. While anaesthesia and analgesia in the limbs of the side opposite to the tumor is much less commonly observ^ed than is paralysis, many patients complain of feelings of numbness, tingling, gooseflesh, or burning in these limbs ; and such parsesthesias may be followed by an actual disturbance of sensation. In the advanced stages of these cases, in which the patients have died of the tumor, a hemi- plegia has developed toward the close of life, not, however, involving the face and it is in these extreme cases that anaesthesia has been ob- served in the paralyzed limbs. ^ Diagnosis. — The review of the symptoms just described will con- vince the reader that in the majority of cases of tumor of the brain there are sufficient evidences of the existence of disease within the cranial cavity, and that the gradual development and progress of the disease will enable the physician to come to the conclusion that it is of the nature of a tumor If in any case the symptoms which are pres- ent are carefully classified, the general symptoms being separated from the local symptoms, and the order of appearance of the local symptoms 'Starr. Amer. Jour. Med. Sci., April, 1910. Six cases. DIAGNOSIS. 603 fully determined, it will usually be possible to reacu a conclusion as to the situation of the tumor. The combination of local symptoms is sometimes as characteristic as their order of appearance and method of extension, as, for example, in cases of tumors of the crus or of the corpora quadrigemina. It is not to be forgotten, however, that tumors of the frontal lobes and tumors of the temporal lobes, especially those situated in the right hemisphere, often fail to cause any local symptoms. The absence of distinct local symptoms, when general symptoms are present, will point to these localities as the probable position of the suspected tumor. It is not to be forgotten that many local symptoms are produced indirectly by pressure upon parts not far from the tumor, but yet not directly invaded in its growth. It is also to be remembered that tumors pressing upon large vessels of the brain may so disturb the circulation as to produce symptoms quite similar to those of thrombosis, and these symptoms may be due to suspension of function of a part lying at some distance from the tumor. Gushing ^ has called attention to the fact that a tumor anywhere in the brain may so displace the pons as to put the basal arteries on the stretch ; and that these arteries may then compress the cranial nerves, especially the sixth to the tenth. This may give rise to a mistaken diagnosis of a tumor on the base. The diagnosis of the variety of the tumor present can be reached only by careful study of the general history of the patient and by a consideration of those facts that have been already mentioned in the discussion of the varieties of brain tumor. It is possible to determine the question of the tubercular nature of the tumor by tuberculin injec- tions or by Calmette's test. It is possible to establish the existence of gumma by lumbar puncture and the discovery of lymphocytes in the cerebro-spinal fluid, or by Wassermann's blood tests. It is therefore evident that in the diagnosis of the disease there are always three questions to be settled : first, the existence of a tumor ; secondly, its situation ; thirdly, its variety. A study of the symptoms will usually enable the physician to reach an answer to the first two questions, but the answer to the last question will always remain uncertain. The symptoms of brain abscess may be the same in kind as those of brain tumor, since both produce an increase of intracranial pressure and a progressive destruction of brain tissue. In their origin, mode of development, progress, and termination, however, there are not infre- quently marked differences. Brain abscess develops most commonly after severe injuries or in conjunction with suppurative affections of the inner ear and of the nasal and orbital cavities, and with caries of any of the cranial V^ones which lie in contact with the membranes. These rarely occur prior to the development of brain tumor. The symptoms common to tumor and absr;ess may develop after a blow on the head, but when the condition is one of abscess the symptoms appear in more » Brain, 1911. 604 TUM0B8 OF THE BEAIN. rapid, succession, with greater severity, and more frequently with fever than wlien the condition is that of tumor. Furthermore, the symp- toms of abscess, after appearing suddenly, often subside, the abscess becoming latent, and all symptoms disappearing for months or years, and then breaking out again with suddenly fatal termination. This course contrasts markedly with that in a case of a tumor where a grad- ual progress with slowly increasing intensity of all the symptoms is found. The constant addition of new symptoms is usual in tumors, and a temporary remission rather than intermission of the symptoms is the rule when the progress is not continuous. There may also be some points of distinction found in the individual symptoms. Thus headache is more severe and paroxysmal with tumor; optic neuritis is much more commonly found with tumor ; mental changes are more gradual and constant with tumor, and local symptoms are slower in onset and more apt to develop with tumor. A complication of tumor not infrequent is cerebral hemorrhage. Meningitis is the usual com- plication of abscess. Lastly, a duration of from one to two years, with symptoms constantly present, points directly to tumor. Tubercular meningitis is under certain circumstances easily mistaken for cerebral tumor. This is not true of the ordinary cases of tubercu- lar meningitis with acute hydrocephalus, which develop marked symp- toms rapidly and terminate fatally within four or six weeks. But there are a number of cases of tubercular meningitis which present a chronic course with gradual progress, and in which the diagnosis from tuber- cular tumor is almost impossible. It is true that the symptoms often develop rapidly in these cases, and yet this is sometimes apparently the case in tumor, for, unless a patient is carefully watched, the early symptoms of tumor may escape notice for some time. The symptoms of chronic tubercular meningitis may be the same as those described as general symptoms of brain tumor, but the headache is more severe in meningitis and more continuous ; there is more likely to be hypersen- sitiveness to light, sound, or touch in meningitis, and optic neuritis develops less frequently, less rapidly, and with less intensity than in tumor. Tubercles upon the choroid are found more frequently in men- ingitis than in tubercular tumor. It is, of course, understood that a localized meningitis may give rise to the same symptoms as a small tumor, and then the differentiation is impossible. This is more common about the base of the brain, in the region of the cranial nerves, than elsewhere. It is also to be remembered that a chronic progressive meningitis may develop in the vicinity of a tumor. Here, again, the diagnosis will be impossible. A gradual subsidence of the symptoms, with recovery, will point to meningitis rather than to tumor. Chronic hydrocephalus, while not infrequently the result of tumor or meningitis, may be due to a ch^'onic inflammation of the ependyma of the ventricles. It then advances slowly. The fluid within the ventricles produces pressure upon the brain, causing atrophy. The course is chronic, and the general symptoms are those of cerebral tumor. The local symptoms, however, differ in some respects from DIAGNOSIS. 605 those of tumor. Spastic paralysis develops with chronic hydrocephalus without lo(!klized spasms, aud is always bilateral ; the lower limbs are affected more intensely than the upper. The child presents the extended, adducted, stiif legs, with overlapping knees, rigid muscles, increased tendon reflexes, and the spastic gait so familiar in infantile diplegia (see Chapter XXVIII.), and, in addition, the hands move without proper coordination. As the disease progresses the crura cerebri and pons may be displaced by the pressure of the fluid, and irregular symp- toms due to stretching of the cranial nerves may appear. These, with the paraplegia, may lead to a suspicion of a tumor of the pons or base of the brain, and only by the order of development of the symptoms can the differentiation be made. The diagnosis from syphilitic meningitis is considered in Chapter XXXVII. The diagnosis between cerebral tumor and cerebral hemorrhage will be necessary only in a few cases in which the onset of the symptoms has been very sudden. There are a few cases of tumor, chiefly glioma, in which the growth has been latent for some time, and has then given rise suddenly, after a blow on the head, or exposure to the sun, or some other accidental influence, to well-marked symptoms. These are usually both general and local symptoms, chiefly the latter. The suspicion of a tumor will be aroused if, after such an apoplectic stroke, the symp- toms persist and increase instead of subsiding, and if headache, con- vulsions, and optic neuritis appear. Hemorrhage alone never gives rise to the last-named symptom. A novel means of establishing the diagnosis of brain tumor and of ascertaining at the same time its nature has been proposed by Neisser and by Pollack ^ and fully described by Pfeifer.^ It has been adopted by F. Krause.^ A very small hole is bored in the skull, the scalp over it being anaesthetized by cocaine, and a hollow needle is thrust into the brain under strict aseptic precautions and some aspiration is employed. The needle is then withdrawn and its contents examined. In this way tumors have been detected and their nature determined. Such punc- tures are not often injurious, but two deaths due to hemorrhage are on record. By their means abscess, cysts, hemorrhages and hydrocephalus have been diagnosticated as well as tumors. The examination of the head by means of the a:;-rays does not often help in the diagnosis of brain tumor. Occasionally cases have been re- ported where the presence of the tumor appeared to be visible. In the majority of cases nothing can be seen, as the consistency of the tumor is about the same as that of the brain. There are a few general diseases which present certain symptoms somewhat similar to those occurring in brain tumor, and these should always be kept in mind. They are extreme anaemia, with defective vision, from myopia or hypermetropia ; chronic lead poisoning and ' Neisser and Pollack. Mittheilungcn aas der Grenzgeb. der Med. u. Cliir. , Bd. xiii., 1906. 'Pfeifer. Arch. f. PHych., Bd. xlii., pp. 451-fi03, 1907. ^F. Krause. Chirurgie des Gehirns, Berlin, 1908. 606 TUMOBS OF THE BJRAIN. chronic diffuse nephritis or contracted kidneys. The knowledge that these diseases may simulate brain tumor will lead the physician to be upon his guard. It is not necessary to mention the numerous points of the differential diagnosis which will enable a definite conclusion to be reached in any case. The Course of the Disease. — The general history of the progress of a patient suffering from brain tumor has to some extent been indi- cated in the discussion of the symptoms. Suffice it to say that a gradual increase in the number and intensity of the various symptoms is usual. In some cases the general symptoms precede the local symp- toms by several months, and optic neuritis does not often appear within three months of the beginning of the symptoms, unless the tumor is in the cerebellum or on the base of the brain. In some cases the local symptoms appear before the general symptoms, especially if the tumor is located in the motor cortex, when the case may be regarded for some time as one of cortical epilepsy until the general symptoms of brain tumor intervene.'^ As the case goes on and both local and general symptoms become more numerous the suffering of the patient becomes more intense. If the case is one of gumma it may be possible to relieve the symptoms and, by a progressive course of treatment to cure the patient entirely. Under these circumstances the symptoms gradually subside and become less in intensity up to recovery. In other cases it is possible to locate the tumor absolutely in a position accessible to the surgeon and to remove it by operation, and under these circumstances the recovery of the patient is gradual but progressive after the operation is over. The brain resumes its functions after the pressure of the tumor is removed. When a portion of the brain has been injured in the removal of the tumor recovery may be imperfect, with some defects of sight, or motion, or sensation remaining. In those cases that are not subject to specific treatment and in which the tum3r cannot be removed the course is progressively downward, the patient suffering more and more intensely as the tumor grows, and finally passing into a state of coma or dying in convulsions. The average duration of the symptoms is said to be three years, but individual cases vary greatly. There are a few cases, however, in which, either spontaneously or under specific treatment, a tumor has ceased to grow and the patient has apparently recovered and remained quite well for some months or even years, the brain apparently resuming its function. Two such cases have come under my observation. In one the recovery lasted four months, sudden death following, due to the rupture of a cyst which lay at the side of a sarcoma. In the other an interval of eight years occurred, after which the symptoms returned and caused death. In the last case optic neuritis, which was present at the first attack, subsided entirely, but recurred at the second attack. The tumor was a sarcoma in the cerebellum. In both cases there was no evidence of 1 Kocher, Zeitsch. f. Chirurgie, June, 1893, PBOGNOSIS. 607 syphilis, yet the treatment, which was apparently successful was by inunctions ■^of mercury and large doses (300 to 400 grains daily) of iodide of potassium. In some cases of tumor of the brain sudden death has occurred unexpectedly. I have witnessed four such deaths in patients about to be subjected to operation. In two of these cases tumor of the cere- bellum near to and pressing upon the medulla was found. In one of these cases the heart continued to beat six hours after voluntary res- piratory movements had ceased. Prognosis. — It is evident from this statement of the course of the disease that the general prognosis in brain tumor is unfavorable. We have seen from a table on page 572 that but 9 per cent, of tumors of the brain are open to operation. It is therefore evident that in the vast majority of the cases we cannot give the patient any hope. If a tumor is syphilitic salvarsan may be given in repeated doses at intervals of two weeks. In some cases its effect is immediately bene- ficial. If it does not relieve the symptoms mercury and iodide are to be tried. I prefer inunctions of mercury, one drachm of blue ointment being rubbed in at night after a hot bath, a different part of the body being selected for each application, and after the application being cov- ered with bandages so that the process of absorption of the ointment remaining upon the skin may occur during the night. At least two ounces of blue ointment should thus be used, and if improvement is evi- dent the inunctions may be repeated after an interval of two weeks. The occurrence of salivation will necessitate the cessation of the use of mercury temporarily. At the same time large doses of iodide of potash should be given, beginning with twenty grains three times a day, and increasing the dose one or more grains daily until 100 grains three times a day have been reached. The iodide may be given in Vichy water or in milk, before or after meals, according to the con- dition of digestion. My preference is to give it before meals. Care in the regulation of the diet, the use of simple and nutritious food, frequent massage to aid digestion, and a daily hot bath at a temperature of 100° F. continued for fifteen to twenty minutes are essential during the taking of these large doses of iodide. When the tumor is of a gummatous nature a decided improvement in all the symptoms, and particularly in insomnia and headache, should be observed within a month. Such improvement may, how- ever, occur in cases of either sarcoma, or glioma, or cystic tumors. It is therefore necessary to keep up this treatment for two months longer. If the improvement continues and the patient gradually recovers, it is probable that a gumma has been absorbed. In a case under my observation in which the symptoms in January, 1903, were intense headache, insomnia, mental apathy, staggering gait, great general weakness, optic neuritis in both eyes, with partial blindness in the right eye and total paralysis of the right third nerve, par- tial anaesthesia of tlie right side of the face, and paresis of the right sixth and seventh nerves, there was by November, 1893, a complete 608 TUMOES OF THE BEAIN. recovery, which still persists, an occasional nocturnal headache being the only symptom remaining. Five courses of inunction have been employed and iodide has been given to the extent of 250 grains a day, the dose being varied from time to time when symptoms of intolerance appeared. Thus in ten months a basal gumma of considerable size has been absorbed by persistent treatment. If the tumor present is a sarcoma or glioma, and an improvement has occurred during the first months of treatment, such improvement will not always persist, and therefore a return of the symptoms during the course of specific treatment is a pretty sure proof that the tumor is not of the nature of a gumma. It is useless to continue specific treat- ment after three months of thorough trial ; it is better to refuse medical treatment or to depend entirely upon palliatives under these circum- stances. It must be stated, however, that some authorities believe that tumors of the brain, not syphilitic in nature, have been cured by the persistent use of large doses of iodide. In every case, therefore, where surgical treatment is impossible it is well to employ this remedy. Treatment. — 1. Medical Treatment. — Headache can be relieved by a free use of phenacetin, antipyrine, or acetanilid. The doses of these druo-s which must be used are larger than those commonly em- ployed, and it is my practice to begin with the ordinary dose and rapidly increase the number of doses given, combining with the drug any heart stimulant, cafieine being the one preferred. If the patient be carefully watched while this is being done it will soon be found possible to give safely twenty grains of antipyrine, fifteen to twenty grains of phena- cetin, or ten grains of acetanilid at a dose, and this dose may be repeated after three hours, provided the headache returns. In a few cases the headache may be benefited by ice-bags to the head, by hot baths, or by ergot. If these remedies are useless resort must be had to mor- phine, but this drug is especially unsatisfactory in the treatment of headache from brain tumors, unless very large doses are given. Vomit- ing and vertigo in brain tumor are best relieved by the use of bromide of sodium or by hydrobromate of hyoscine in ^rw E^- ^^^^ repeated every four hours. The course of optic neuritis cannot be arrested, though it may be somewhat delayed by cupping the temples. While strychnine will at any time, when given hypodermically, improve temporarily the power of vision, yet it does no permanent good, and is thought by some to hasten the progress of the disease. Epileptiform convulsions may be reduced in frequency by the free use of bromides, but cannot be arrested as long as the disease goes on. It is thus evident that the treatment of the general symptoms of brain tumor is exceedingly unsatisfactory. There is no treatment known that will in any way affect the local symptoms, massage and electricity to paralyzed limbs being mere palliatives, capable only of maintaining the nutrition of the muscles. 2. The Surgical Treatment of brain tumors is a subject which has awakened much interest of late, inasmuch as over 1,000 tumors have been operated upon up to the present time. TBEATMENT. 609 A careful review of a large number of cases of brain tumor, exam- ined post mortem, shows that about 10 per cent, of tumors are open to surgical treatment. The most recent statistics of results in such cases have been published by Dr. Philips Coombes Knapp,^ whose table I reproduce below. Of 828 tumors operated on up to July, 1905, it was found that in 471 cases the tumor was found and removed; in 189 cases the tumor was not found, in 104 cases the operation was a palliative one only, and in 64 cases the tumor, though found, could not be safely removed. Further analysis of the cases, 471 in number, in which the tumor was removed, shows that the diagnosis was correct, the removal easy, and the recovery or improve- ment most satisfactory in those cases in which the tumor lay near the Rolandic or Sylvian fissures, while the mortality was greatest in the cerebellar cases. These statistics are quite in accord with my own experience. It is evident then that out of 100 brain tumors we may expect success in surgical treatment in but five only. Yet there is no doubt that even if the number of lives saved is small it is our duty to study every case with our minds fixed on the question of possible surgical relief. Table XV. — Kesults in Operations for tumors of the Brain. Not im- Result not Recovered. Improved. proved. Died. stated. Total. Removed 112 122 78 123 36 471 Not found 4 24 61 87 13 189 Impossible 11 11 40 2 64 Palliative ^ 4^ 36 J5 5 104 Total 116 205 186 265 56 828 Frontal 15 6 3 8 2 34 Central 52 82 43 54 5 236 Parietal 7 5 5 12 29 Temporal 2 4 6 5 1 18 Occipital 3 3 2 2 1 11 Cerebellum 7 16 8 23 1 55 Not stated 26 6 11 19 26 88 It is imperative to consider the possibility of operation in every case of brain tumor. Success in these operations depends (1) on the situation of the tumor ; (2) on its removal early in the course of the disease ; (3) on the variety of tumor found ; (4) on the amount of brain tissue destroyed either by the tumor or in the course of the operation. An analysis of the successful cases shows that they Were chiefly tumors situated about the fissures of Rolando or Sylvius, giving rise to motor or aphasic symptoms easily localized. I have had a number of these cases ; in four perfect recovery has been the result, and the patients are now alive and well. A number of tumors have been successfully removed from the frontal convolutions, from the parietal region, and from the occipital region, but the percentage of failures in these locali- ties, due probably to uncertainty in diagnosis, was greater than in the motor region. I have had successful cases in each of these localities. 39 1 Boston Med, and Surg. Jour., Feb. 1, 1906. 610 TUMOES OF THE BEAIN. The following is an example : In January, 1 905, a healthy business man began to suffer from severe frontal headaches, sometimes attended by vomiting, and it was soon noticed that he misplaced words and had difficulty in expressing his ideas. These symptoms increased steadily during February, and early in March he had a temporary attack of right hemiplegia and aphasia, which, however, passed oif entirely in two days, but left his speech more defective. This was followed by recurring attacks of twitching in the right hand and in the right side of the face, attended and followed by numbness in these parts. By the middle of March his speech defect had become constant and he could no longer write, though he could read and understand everything said to him ; and he had become very dull and drowsy. On March 25 he went into a semi-stuporous state in which I first saw him. I found an extreme choked disc in both eyes, worse in the left ; no paralysis, but a flatness of the right side of the face and in- creased reflexes in the right limbs. There was motor aphasia. I concurred in the diagnosis of brain tumor made by his physician. Dr. Rutz, and located the tumor in the left third frontal convolution, and recommended operation. On March 27 Dr. Hartley operated, making a large bony flap over the left temporal region four inches in diameter. When the dura was opened the tumor was visible, lying in the middle of the third frontal convolution. It was easily shelled out by a spoon, and some small discolored parts of the white matter of the brain about it were also taken out, leaving a clean cavity. His recovery was rapid. The day after the operation he was still speechless, but within a week he had regained his speech, could read aloud, and could write, and had no paralysis. On April 16 he was up and the wound had healed. On May 16 he went back to business in apparently good health. In May, 1906, he had no return of brain symptoms. In any case, where the tumor is cortical, can be localized accurately, and is accessible, the chance of success is good. In some cases tumors beneath the cortex can be removed. It is quite safe to incise the brain to a depth of several inches if the ventricle is not opened. If a tumor is not seen at an operation it may be felt for, the finger being thrust into the brain at the bottom of a sulcus. Thus in a case of deep lying tumor of the parieto-occipital region, removed for me by McCosh, it was only at the second operation and by a deep incision that this was found. It proved to be an encapsulated sarcoma with a pedicle attached to the falx, and its removal was followed by apparent recovery, hemi- anopsia remaining. The tumor, however, recurred some months later, and the patient died. It is to be remembered that deep incisions or lacerations of the brain in the removal lead to permanent symptoms, and the scar is likely to prove the focus of irritation for subsequent epileptic attacks. The cerebellum is very difficult to reach, only a small part of each hemisphex'e being accessible through one half of the occipital bone. TREATMENT. 611 Gushing has however succeeded by removing the entire occipital bone ; and since 1904 73 successful cases with recovery have been recorded/ A few tumors from the cerebello-pontine angle involving the eighth nerve have been successfully removed, but this is a very difficult locality to reach. Gushing has succeeded in reaching and removing tumors of the hypophysis, entering the skull through the mouth above the upper jaw. The second factor in success is the promptness with which the need of an operation is recognized and the procedure undertaken. Many patients could have been saved, who have died, had the tumor been removed at an earlier time in the course of the case. The moment an accessible tumor is diagnosticated an operation should be undertaken. It is a mistake to try medical treatment, even antisyphilitic treatment in syphilitic cases, as the operation even in these affords rapid relief, and in cases where it fails valuable time is lost. The third factor in success is the variety of tumor found. The table here given illustrates this fact. Table XVI. — Table of Variety of Tumor Eemoved, and Eesxjlt. Variety. Recovered. Died. Sarcoma ....... 52 20 Gliosarcoma ...... 10 Cyst 29 11 Angioma .....•• 7 1 Gumma ....... 8 3 Tubercular ...... 19 8 Fibroma ....... 7 2 Endothelioma 5 4 Glioma ....... 15 13 Sarcoma, gliosarcoma, and fibroma are usually hard, encapsulated, easily separable from the brain tissue, and can be removed without laceration. Gysts of the brain are easily emptied when found, but the mere abstraction of the fluid gives no permanent relief, as it reaccumulates. It is necessary, therefore, either to drain the cyst and thus secure its closure from the bottom by the approximation of its walls, or else to remove the whole of the cyst by dissecting it out from the brain. The latter is the best method when the cyst is on the surface, but is a dangerous process, and is sure to be followed by the formation of scar tissue, which in itself is a constant irritant to the brain. Drainage is necessary in deeper lying cysts. If, however, the cyst is a part of a glioma the removal of its wall and adjacent tissue affords less hope. Angioma, if lying on the cortex and if made up, as is usual, of vessels from the pia, can be easily removed if care be taken to ligate all the vessels entering the tumor before any are cut. But such liga- tion may be followed, as in one patient of my own, by extensive softening of the area the veins of which were tied. Gumma, if hard, is easily taken out, but if soft and infiltrating the pia is more difficult of removal. The hai^d type requires surgical 'Starr, Amer. Jour. Med, Sci., April, 1910. 612 TUMOBS OF THE BRAIN. treatment ; hence it should be removed. The soft type will yield to medical treatment, which should in any case follow the operation when a gumma is found. The question of operative interference when a tubercular tumor is diagnosticated has given rise to some discussion, von Bergman n being opposed to such operations and Horsley being in their favor. It must be admitted that a permanent cure by operation is less likely to be achieved in the case of a tubercular tumor than in the case of a non- tubercular tumor, there being not only the danger of recurrence but also the possibility of the existence of undetected tumors elsewhere in the brain and the danger of the development of tubercular meningitis subsequently to the operation. An operation is capable of prolonging life, and hence should not be wholly condemned. The prospect of recovery when an endothelioma or a glioma is found is not good, for it is rarely possible to remove these tumors completely, as they infiltrate the brain, and even if removed they are liable to recur. Carcinoma is particularly unfavorable because it is usually secondary to carcinoma elsewhere. The degree of ultimate recovery after such operations will depend upon the amount of destruction of brain tissue produced by the tumor or by the necessary incisions of the operator. Sometimes this is con- siderable, and the patients are left incapacitated for life. In many cases, however, of which I can record several, a complete recovery has occurred, even when extensive paralysis had preceded the operation. In the table of results the word " recovered " indicates that the patient survived the operation and was improved by it. It is not to be inter- preted as implying a complete restoration to health, which rarely occurs. It is no small result in such cases to save a patient's life and to arrest the progress of a fatal disease. The surgical details of the operation do not require description, being beyond the scope of the physician.^ They are many and complex. The operation is not to be undertaken lightly or without experience, and it is better for the physician to refer such cases to some surgeon who is familiar with the methods of procedure. When an operation for a brain tumor is advised it should be explained that it is an exploratory operation, that it may save life, but that it may leave disabilities which will be permanent ; that it may fail because of the impossibility of removal of the tumor, either because of its deep position or of its nature. At the same time it should be urged as affording a possible cure for an otherwise fatal disease. In cases where it is evident that a brain tumor is present, is causing urgent symptoms, especially blindness, and yet in which the tumor cannot be located, Horsley, Harvey Gushing and others advise a de- compressing operation ; that is, a large opening in the skull, usually over the temporo-parietal region, the dura being opened and cerebro- 1 See Brain Surgery, by M. Allen Starr, M.D., Wm. Wood & Co., 1893. Also, Brain Surgery, by Harvey Gushing, in Keen's Surgery, vol. iii. Also, Krause, Chirurgie des Gehtrns, Berlin, 1908, TBEATMENT. 613 spinal fluid being allowed to drain out into the dressings. It is cer- tain that such an operation relieves the general symptoms and restores the sight if there is no optic atrophy. In a case of my own, operated upon by Harvey Gushing/ a choked disk causing blindness was relieved in a week and vision returned. I can report two similar cases. The course of the disease is in these cases prolonged and the patients may be more comfortable. In a few cases subsequently to the operation a tumor has appeared in the opening and has been removed at a second operation. The hernia cerebri which tends to form after the operation may attain a large size ; the discharge of cerebro-spinal fluid may be enormous (60 oz. daily for two weeks in one of my cases) and there is consequently great danger of infection. If this is avoided, however, the hernia may gradually decrease until after three months it has dis- appeared ; the flow of fluid may cease ; and the scalp may heal, leaving only the bone defect. This has been the result in two of my cases, with gradual subsidence of the symptoms. Whether the relief of pressure, or the discharge of fluid arrested the growth of the tumor, or led to its absorption, must be undecided. Others report similar results. If the operation does not cure it at least relieves some symptoms and rarely hastens the fatal end. Hence in every case of brain tumor it seems a duty to resort to a decompressive operation. Gushing prefers to open the skull under the temporal muscle for cases of cerebral tumor and under the occipital muscles for cerebellar tumor, the muscles being split and then united so as to prevent too great protrusion. In inaccessible tumors with hydrocephalus puncture of the lateral ventricles through the corpus callosum has been done, an opening a quarter of an inch in diameter being made, and left open permanently ; so that drainage of the ventricle into the membranes is obtained. In some cases this has relieved the symptoms for a time. In other cases the relief has been very temporary. ^Amer. Jour. Med. Sci., April, 1910. CHAPTER XXXIV. BULBAR PARALYSIS. THE CEREBRAL AXIS. The cerebral axis consists of the crura cerebri, pons Varolii, and medulla oblongata, which lie upon the base of the skull. (See Fig. 78, page 149.) It contains the nuclei of origin of all the cranial nerves except the first and second. These are arranged in groups, lying upon the floor of the fourth ventricle or near to it. It also contains the great motor and sensory tracts connecting the cerebrum and cerebellum with each other and with the spinal cord. Fig. 256 shows a lateral view of the cerebral axis and Fig. 77 (page 148) shows a transparent dia- gram which indicates the position and relative arrangement of the nuclei within it. Figs. 52 to 57 (pages 111 to 117) show the various tracts passing through the cerebral axis. From a study of these diagrams and from a consideration of the symptoms of lesions in the cerebral axis already discussed, pages 110 and 115, it is evident that gross lesions such as hemorrhages, small areas of softening due to thrombi or emboli, plaques of sclerosis, and tumors of the cerebral axis, can be easily located by their effects upon the cranial nerves and upon the sensory and motor tracts. The char- acteristic features of such lesions are alternating paralysis or anaesthesia when the lesion is unilateral, and paraplegia with cranial nerve symp- toms when it is bilateral. The small size of the cerebral axis and the importance of the tenth nerve centres to life make it evident that lesions which are larger than half an inch in diameter are usually immediately fatal. If, however, a lesion spares the tenth nerve nuclei, the patient may live, and then a diagnosis of bulbar lesion is not difficult. The prognosis is, however, more serious than in tumor, hemorrhage, or softening elsewhere in the brain, as an extension of the lesion may end life at any time, and regeneration in these nuclei and tracts does not occur. BULBAR PARALYSIS. Any affection which involves the cerebral axis may be termed bul- bar paralysis. Thus cases of hemorrhage, which are rare, or of soften- ing, which are common in the crura, pons, or medulla, may be described " as acute apoplectiform bulbar paralysis." But since such cases differ from cases of cerebral apoplexy in no respect excepting in the peculiar combination of their symptoms, due to the location of the lesion, it seems unwise to establish a special category of diseases for these cases. 614 StJLBAn PARALYSIS. 615 The facts already stated in tegard to the pathology of apoplexy (page 473), and m regard to its symptoms, and especially those produced by lesions in this region (page 494) apply to this condition and make it Fig. 256. The cerebral axis. The cranial nerves and their nuclei are numbered III. to XII. BrcJ, superior peduncle of cerebellum ; Cm, corpus mammillare ; Cop, posterior commissure ; DB, decussation of superior peduncle ; DLm, sensory decussation in medulla ; DPi/, motor decussation in medulla ; BV, crossed bundle of V. nerve ; Fai, internal arcuate fibres ; Fcl, fornix ; Fng, funiculus gracilis ; Flp, posterior longitudinal bundle ; Frtf, fasciculus retroflexus ; If, infundibulum ; Leo, locus caeruleus ; I/m, lemniscus ; JVa, anterior nucleus of thalamus ; Nai; nucleus arcuatus ; Ng, nucleus gracilis ; iVo, olive ; Mg, nucleus tegmenti ; 2f, red nucleus ; Oaa, ventral olivary nucleus ; Oae, dorsal olivary nucleus ; Po, pons ; Pos, superficial transverse fibres of pons ; Pp, pes pedunculi ; Pp, pyramid ; Qa, Qp, corpora quadrigemina ; Sn, substantia nigra ; Sim, striae acusticse ; Stz, stratum zonale of the thalamus ; Tho, thalamus ; Ust, lower thalamic bundle ; Va, Vicq d'Azyr's bundle ; Vg anterior column; Vma, valve of Vieussens. (Obersteiner.) Section through the pons Varolii, showing multiple hemorrhages which caused death. (Comte, Des Paralysies Pseudo-bulbaires, Paris, 1900.) unnecessary to do more than mention an affection which, though ex- tremely rare, has received some attention in recent text-books. Fig 616 bVLbab p analysis. 257 shows a diagram of such a lesion, a number of small hemorrhages in the pons Varolii having caused the death of the patient. Acute bulbar paralysis has been already described in the chapter upon ence- phalitis ; it being also known as polio-encephalitis inferior. (See page 538.) By bulbar paralysis a disease of a chronic progressive type is usually indicated, also called glosso-labio-laryngeal paralysis. This disease is exactly homologous to chronic anterior poliomyelitis, the lesion being in the cerebral axis and not in the spinal cord. It is a slowly advanc- ing degeneration in the nuclei of origin of the motor cranial nerves, one after another being involved, usually from below upward, but occa- sionally from above downward. In some cases it is a part of a more widespread aifection, viz., amyotrophic lateral sclerosis, and many cases which present the typical features of bulbar palsy, if they survive, develop the additional symptoms which characterize that disease. (See page 294.) But in many cases the bulbar symptoms are the only ones to present themselves, and produce a very typical picture which is easily recognized. Etiology. — The etiology of bulbar paralysis is obscure. In a few eases it appears to have been a congenital aifection, and as, in these it has occurred in two or more children of a family whose parents were related to one another or in children who showed signs of degenera- tion, it has been considered an hereditary or a family disease and due to defective development of the brain. In one such case hereditary syphilis was the manifest cause. The majority of the cases develop between the thirtieth and fiftieth years of life, though no age is exempt. Falls and blows on the head, emotional strain, and cold have been thought to be causes of the disease. Overwork of the facial muscles, such as occurs in players on wind instruments, has been thought to be a cause. Pathology. — The pathology is a progressive atrophy of the motor neurones in the hypoglossal, glossopharyngeal, spinal accessorv, pneu- mogastric, facial, and motor part of the trigeminal nerves and occasion- ally of the abducens and oculomotor nuclei also. The bodies of the neurones undergo a progressive atrophy, lose their branches, both den- drites and axones, become smaller, and finally are reduced to small nuclei or disappear wholly. (See Plate III., page 34.) The axones degenerate in the same manner and leave an empty sheath. The muscles to which they go atrophy. The pathology of the disease is therefore identical with that of chronic anterior poliomyelitis {q. v.). This lesion may be limited to the gray nuclei of the cerebral axis. But in some cases sclerosis of the pyramidal (motor) tracts in the medulla and pons has been found, and this in a few cases could be traced into the lateral and anterior median columns of the spinal cord. In such cases it is evi- dent that the disease was amyotrophic lateral sclerosis and that the bulbar paralysis was only the first stage of that affection. Symptoms. — The symptoms of bulbar paralysis usually begin in the tongue, causing a slight defect in articulation, words which con- PATHOLOGY. 617 tain the Unguals, d, 1, n, r, s, t, being badly pronounced. As the disease go^s on this difficulty of speech is increased by the develop- ment of paralysis in the pharynx, which makes the speech nasal, and of paralysis of the lips, which prevents the enunciation of b, p, v, w, y, and finally it is almost impossible to understand these patients, the vowel sounds alone being clearly produced. When the larynx itself becomes paralyzed even phonation is indistinct. This symptom has been termed dysarthria to distinguish it from aphasia. The paralysis of the tongue is attended by fibrillary twitching and by atrophy, which throws the mucous membrane into folds. A reaction of de- generation may be found in the muscles of the tongue. While the symptoms are developing disturbance in the act of chew- ing and of swallowing appears. The food cannot be rolled about in the mouth on account of the paralysis of the tongue and cannot be carried backward to the pharynx. The pharyngeal muscles are involved, and hence their automatic action is imperfect ; hence the food is not grasped and propelled into the oesophagus, but may stick in the throat and get into the larynx. The automatic closure of the epiglottis on arrival of food near it is also impaired by paralysis of the laryngeal muscles ; hence fluids trickle into the larynx and fits of choking become frequent. These patients sometimes attempt to aid themselves by pushing the food back into the pharynx with the finger or by lying down when they attempt to swallow fluids. The paralysis of the pharynx and uvula may lead to regurgitation of fluids through the nose. When the paralysis reaches the muscles of mastication chewing is impossible, and semisolid food only can be taken. It is probably one reason for the rapid emaciation of these patients that the difficulty of eating deprives them of a proper amount of nourishment. The difficulty in deglutition forms one of the chief dangers of the disease, for foreign bodies get into the larynx and lungs and choke the patients or set up pneumonia, which is fatal. For this reason feeding by the stomach-tube should be begun early, and the patient should be in- structed how to pass the tube, and thus should avoid all acts of swallowing. A further set of symptoms is referable to the facial muscles. The lower muscles become weak and atrophy, causing a lack of facial expression and a flattening of the face from obliteration of its folds. The muscles about the mouth are relaxed and the mouth cannot be firmly closed, and the lower lip hangs down. Hence the saliva runs out, and the patient holds a handkerchief to catch the drooling. He can no longer whistle or blow. The jaw-jerk may be increased. The muscles closing the eye are very rarely paralyzed. The lower muscles of the face and of the tongue are in a state of constant fibrillary con- traction causing a fine tremulous, wavy movement of the skin and mucous membrane. As the facial muscles atrophy a partial reaction of degeneration can be obtained. The paralysis of the muscles of mastication leads to an inability to close the jaw, which makes it necessary for the patient to support it by the hand. The tongue in its 618 BULBAR PAEALYSIS. final state of paralysis lies on the floor of the mouth, cannot be put out or moved about, the soft j)alate hangs motionless and does not rise in speaking or in swallowing ; hence fluids return through the nose. In some cases the oculomotor nuclei become affected and various forms of strabismus and double ptosis develop. Thus in the patients shown in Figs. 258 and 259 there was not only a paralysis of the facial muscles and of the tongue, but double ptosis with paralysis of all the muscles of the eyeballs. Fig 259. Patient suflfering from bulbar paralysis. Double ptosis, slight external strabismus, facial paralysis with lack of facial expression and inability to close the lips are to be seen. The effort to open the eyes causes a wrinkling of the forehead. Bulbar paralysis. Double ptosis, with ex- ternal strabismus, flattening of the face, inability to close the mouth, and atrophy of the right half of the tongue are to be seen. (Icon, de la Salpetriere.) Finally, the respiratory movements of the larynx are interfered with, a peculiar stridor is heard in respiration, the breathing becomes rapid, and death by asphyxiation occurs. In the early stage a laryn- goscopic examination shows weakness of the adductors, which later on becomes a total paralysis. In a few cases the oculomotor nerves and the abducens have been paralyzed, causing ptosis, and strabismus with all the characteristics of ophthalmoplegia (q. v.). There is never any pain or anaesthesia of the face, but headache in the occiput or pain in the nape of the neck is sometimes complained of. Sometimes an increase of knee-jerks is present. A very constant symptom of bulbar paralysis is an emotional state. The patients cry on very slight provocation, and seem to lack a normal control of their emotions and are extremely depressed at their condition. In a few cases symptoms of spastic paraplegia appear and the disease goes on to amyotrophic lateral sclerosis. In other cases an atrophic paralysis develops in the hands and arms, and it is evident that a chronic anterior poliomyelitis has followed the DIAGNOSIS. 619 bulbar palsY. It has already been stated that in both of these diseases bulbar paralysis may develop as a final result. These facts point to a common origin and pathology in all three affections, which may, there- fore, be regarded as stages or varieties of onset in one disease. The course of bulbar paralysis is slowly progressive. In a few cases there may be for a time an arrest of symptoms, but they soon reappear, and the result is uniformly fatal. The average duration of the disease is two years, but I have seen several rapid cases which terminated within a year of the onset. Death is due to choking, to pneumonia or bronchitis set up by inhaling food, or to starvation. Diagnosis. — The diagnosis of bulbar paralysis presents no difficulty. It is to be remembered, however, that similar symptoms are caused by tumors of the cerebral axis, though these are rarely so located as to produce bilateral symmetrical symptoms. I have seen one case, how- ever, in which the entire absence of general symptoms of brain tumor and the slowly progressive development of bulbar paralysis led to- this error, which was only revealed at the autopsy. The history of the slow progress of the symptoms will serve to distinguish bulbar paralysis from vascular lesions in the cerebral axis. But cases of aneurism of the basilar artery sometimes present very similar symptoms, though in the few cases thus far recorded spastic paraplegia has appeared early. The occurrence of spinal and cortical symptoms will distinguish multiple sclerosis from this disease. In the course of multiple neuritis either of diphtheritic or of toxic origin cranial nerve symptoms some- what like those of bulbar palsy may occur. But the history of the case, its rapid progress, the occurrence of other symptoms referable to the nerves of the extremities, and the rapid recovery leave no doubt as to the diagnosis from bulbar paralysis. There is a condition quite like that of bulbar paralysis which is pro- duced by bilateral lesions in the facial motor area of the cerebral cor- tex lying in the operculum or in the motor tracts from those areas to the nuclei of the cranial nerves, especially in the internal capsule near the lenticular nucleus and in the lenticular loop. This condition is naturally a very rare one, but it has been observed. It has been named pseudo-bulbar paralysis. It may be due to a congenital defect with porencephalus, and then the symptoms appear in infancy. It may be due to multiple sclerosis or to several separate attacks of an apoplectic nature, each causing unilateral symptoms, but the final result being a symptom-complex resembling bulbar palsy. The history of the development of the symptoms should prevent any mistake of diag- nosis. And in these cases the paralysis is not accompanied by rapidly advancing atrophy, fibrillary tremor, and reaction of degeneration, which can be demonstrated in cases of bulbar paralysis. There is usually a weakening of the mind, a dementia, and not uncommonly some form of aphasia. And in these cases evidences of arterial disease aid the diagnosis as well as the non-progressive course of the symptoms. Finally, it is necessary to differentiate bulbar paralysis from as- thenic paralysis or myasthenia gravis. The symptoms in myasthenia 620 BULB AM PAEALYSIS. gravis are much like those of bulbar paralysis, though ptosis, paralysis of the orbicularis palpebrarum, and weakness of the muscles of the neck, which are very rare in bulbar palsy, appear early in myasthenia, Furthermore, in myasthenia a great general muscular lassitude and undue fatigue on exertion is quite constant, so that a few movements exhaust the patient completely, rendering him incapable of walking or of using his hands. The rapid onset of this general muscular weak- ness is sufficient to differentiate the disease even from cases of amy- otrophic lateral sclerosis beginning with bulbar symptoms. The elec- FiG 260. C.T. Section of muscle showing lymphoid infiltrations. The letters C.T. indicate a strand of con- nective tissue surrounded by the infiltrating cells. (Hun.) trical excitability of the muscles diminishes progressively as the current is applied, until no contraction can be produced, yet after a period of rest it is found to have returned. This peculiarity, termed electrical fatigue of a muscle, is characteristic of the disease, as shown by Jolly and Murri. The muscles which are affected show no fibrillary tremor, do not atrophy and show no reaction of degeneration. There are no sensory symptoms. The course of the disease differs from that in bulbar palsy, for while the onset may be similarly slow and the various symptoms may all be alike in bulbar and asthenic paralysis, the disease is not steadily progressive. Periods of improvement or even entire subsidence of the symptoms occur. The patient may recover and be free from all signs of disease for some months, though the recovery is never permanent, as almost all cases die within a few years. And if the symptoms prove fatal no lesion in the nervous system has been found. The only constant lesion in myasthenia gravis is an extensive infiltration of the muscles, and of all of the organs of the body, by small lymphoid cells (shown in Fig. 260). The nature of the disease is discussed on page 768. TREATMENT. 621 Prognosis. — The prognosis in bulbar paralysis is very serious. The patients die within three years of the onset of the disease, and some cases run a more rapid course. The length of life may be prolonged by care in feeding these patients. Treatment. — The treatment should consist in keeping up the nutri- tion and general health of the patient by every possible means. Good food, massage, life in the open air, stimulating water treatment by warm packs or hot baths, followed by cool showers or douches, and all forms of tonics should be advised. Strychnine, arsenic, and phos- phorus in the form of hypophosphites and the glycero-phosphates should be employed freely and kept up, each drug being used a short time, and those selected and continued which seem to agree best. In the early stage the patient should be carefully instructed in the passage of the stomach-tube, and this must be insisted upon, as later in the disease, when difficulty in swallowing begins, the chief danger can be avoided by its use. When solids can no longer be swallowed semi- solid food should be given and supplemented by eggs and milk and broths given through the tube. When nothing can be voluntarily swal- lowed all food has to be given in this way. I have known a patient to be kept alive over a year by this method of feeding. The thirst can be reduced by large enemata of salt solution and by following each feeding by giving water through the tube. In the last stages of the disease hypodermic medication, with morphine to allay the mental dis- tress, with atropia to prevent the undue flow of saliva, and with strychnine to support the vital centres may be employed. CHAPTER XXXV. THE CRANIAL NERVES AND THEIR DISEASES. The number, name, and function of the cranial nerves are as follows : First. Olfactory, sense of smell. Second. Optic, sense of sight. Third. Oculomotor, motion of the eyeball up, down, and in. Fourth. Patheticus, motion of the eyeball in and up. Fifth. Trigeminal, sensory nerve of the face ; probably nerve of taste ; and motor nerve of the muscles of mastication. Sixth. Abducens, motion of the eyeball outward. Seventh. Facial, motor nerve of the face. Eighth. Acoustic ; cochlear division, nerve of hearing ; vestibular division, nerve of equilibrium. Ninth. Glossopharyngeal, nerve of the throat, sensory and motor ; possibly of taste. Tenth. Pneumogastric or vagus nerve, larynx, and vital organs. Eleventh. Spinal accessory, nerve of the larynx and neck. Twelfth. Hypoglossal, nerve of the tongue. Each of these nerves has a peripheral course from the surface of the head, from some organ of sense, or from some group of muscles to its primary nerve centre lying upon the base of the brain. Plate XXV. shows the course and the distribution of the superficial nerves of the head and face. The deeper origin of the cranial nerves is shown in Figs. 53 to 57, 77, 78, 256, and 261. The primary centres or cranial nerve nuclei are closely joined to one another by association fibres, and their interaction is necessary in many reflex acts, such as the sniffing of an odor or a sneeze, the turning of the eyes and head toward an object seen, the winking caused by dust in the eye ; the motions of the face in smiling, frowning, or crying ; the pose of the head in listening, or quick turning toward a sound, or a start with winking at a noise ; the involuntary swallowing of saliva or of tasting food, and the acts of respiration and coughing. Each cranial nerve is also connected with the higher centres of the brain. Some are joined directly to the brain cortex. The majority have an indirect connection, the tract from the primary centre to the cortex being interrupted in the basal ganglia. This interruption is for thie purpose of providing for the numerous unconscious and invol- untary automatic acts of high complexity which involve the coordi- nated action of numerous widely separated centres. In studying the cranial nerves it is evident, then, that a sharp dis- tinction must be made between diseases in the peripheral part of th^ 622 PLATE XXV y^. Showing the distribution of the fifth, seventh and eleventh cranial nerves and the cervical and brachial plexuses ; also the area of the middle meningeal artery in the inner table of the skull, injury to which is sometimes the cause of hemiplegia; also the course of the bloodvessels in the neck and face. (Arnold's Atlas.) TKE CRANIAL NERVES. 623 nerve, diseases in the primary centres of the nerve, diseases in the intra- cerebral tracts of the nerve, and diseases of the secondary or cortical centres of the nerve. In this section the diseases of the peripheral Fig. 261. ti Co. The base of the brain and the crura, pons, and medulla, showing the superficial origin of the crania nerves. / to XII, the cranial nerves ; 2%, optic thalamus ; h, pituitary body ; tc, tuber cinereum ; o, corpora albicantia; P, pes .pedunculi ; i, interior and e exterior geniculate body ; PV, pons Varolii ; pa, anterior pyramid of medulla; o, olive; d, decussation of anterior pyramids ; ca, anterior column of spinal cord ; cl, lateral column of spinal cord ; Ce, cerebellum ; fl, flocculus of cerebellum. (Allen Thompson. ) part and of the primary centres of the cranial nerves are considered. The diseases of the intracerebral tracts and cortical centres have been treated under brain diseases, pages 86 to 150. THE FIRST NERVE : THE OLFACTORY NERVE. The structure and course of the olfactory nerve to the olfactory bulb and from the bulb to the brain is demonstrated in Figs. 68 and 262, The primary neurones and the finer filaments of the nerve endings ar^ 624 THE CEANIAL NEBVES AND THEIB DISEASES. situated in the Schneiderian membrane on the upper and middle turbinated bones and on the septum of the nose, all of which parts are pigmented. It is in the olfactory bulb that the primary sensory neurones terminate in a fine brush about the glomeruli of the nerve. (Fig. 262.) The course from the olfactory bulb to the brain must be regarded as an intracerebral tract. The mitral cells in the bulb are Fig, 262. The olfactory bulb and tract. A, Schneiderian membrane in nose in which lies peripheral olfac- tory neurone ; B, glomerulus of olfactory bulb ; C, mitral cells with dendrites in B and axones in D olfactory lobe ; E, granule cells ; F, cells in olfactory lobe ; H, G, fibres of olfactory tract. (Ramon y Cajal. ) the secondary neurones which receive impulses from their dendrites about the glomeruli and send impulses along their axones in the olfactory tract to the cortex. Fig. 68, page 132, shows the course of this tract to its various terminal stations in the optic thalamus and in the cortex of the uncinate gyrus. The thalamic centre is joined to the cortex by the fornix and fascia dendata. It is from the study of comparative anatomy that these facts are established. In man the olfactory apparatus has been reduced to an almost rudimentary state. Any disease that affects the terminal filaments of the nerve in the mucous membrane of the nose will suspend the sense of smell. This symptom is termed anosmia. Acute or chronic catarrhal affections of the nasal organs, arrest of the nasal secretion, which occurs when the fifth nerve is diseased ; tumors of the nose, or polypi ; caries of the ethmoid bones, or ethmoiditis, or diseases which interrupt the trans- mission of impulses to the olfactory bulb will cause anosmia. Menin- gitis upon the base of the brain, fractures of the base of the skull, and tumors of the bone or of the brain in the anterior fossa are capable of tearing or destroying the fine filaments of the olfactory nerve or of separating them from their connection with the bulb. Any disease which destroys or compresses the olfactory bulb or tract or its terminal station in the cortex will have the same effect. An arrest of activity of this centre occurs in hysteria. The sense of smell has much to do with our appreciation of taste, and when it is lost it is impossible for the patient to perceive very delicate differences of taste in various substances. This may be the TEE SECOND NEBVE : THE OPTIC NERVE. 625 symptom fipst noticed by the patient. Loss of the sense of smell is a symptom which is not often perceived spontaneously as long as the disease is unilateral ; hence it has to be sought for by testing the patient with various fragrant substances. The tests are best made with various perfumed powders or essential oils, for it is to be remem- bered that ammonia, alcohol, and vinegar, which are commonly used for this purpose, produce irritation of the fifth nerve as well as of the first, and should not be used as tests. The symptom is of interest chiefly as an indication of disease in the anterior fossa of the skull, such as tumor or abscess of the brain. A primary isolated neuritis of the first nerve has not yet been recorded. It occurs only as a result of nasal disease. Atrophy of the olfactory nerve has been said to occur as a complication of tabes. It must be the terminal lesion in any destruction of the nerve fibres. Irritation of the olfactory organ or of its cortical centres may cause hallucinations of smell (parosmia). This is seen in epilepsy and in insanity. THE SECOND NERVE: THE OPTIC NERVE. Anatomy. — For the finer anatomy and physiology of the optic apparatus and retina the reader is referred to the text-books on ophthalmology. The anatomy of the optic nerve and its connections with the brain are shown in Fig. 263. Many primary optic neurones are situated in the retina, their dendrites being connected with the rods and cones, and these send their axones to the primary optic centres in the thalamus, corpora geniculata, and corpora quadrigemina. It will be noticed that some cell bodies in these basal ganglia send their axones outward to the retina, where they terminate in the rods. Thus the optic nerve con- tains fibres which pass in both directions. The optic nerves pass backward from the eye to the optic chiasm, where a partial decussation of the fibres occurs, the majority crossing over to the other side to enter the opposite optic tract and the minority turning directly into the optic tract of the same side. The crossing fibres convey impulses from the inner or nasal half of the retina, and hence their injury causes blindness in the outer or temporal portion of the visual field, which is much the larger portion. Fibres from the macula lutea, which convey central visual impulses, pass from each eye to both optic tracts ; hence central or direct vision is never affected by a lesion in the optic tract. (See Fig. 264.) The optic tracts, containing fibres from both eyes, curve about the crura cerebri and terminate in the primary optic centres, as shown in the diagram. A lesion of one optic nerve causes blindness of one eye. A lesion of the optic chiasm causes partial or total blindness, according to the number and position of the optic fibres destroyed. If the lesion involves one lateral part only of the chiasm (Fig. 264, ft), the fibres from the temporal half of the retina, are involved, and a unilateral nasal hemianopsia is caused, 40 626 TBE CRANIAL NEEVES AND THEIR DISEASES. that is, a blindness in the inner portion of the visual field of one eye. If the lesion involves the anterior or posterior part only of the chiasm, the fibres which come from the nasal halves of the retinae are involved, Fig. 263. Diagram of the optic nerves and tracts. On tlie right of the figure the masses of gray matter in the basal ganglia are shown. Palv, pulvinar of the optic thalamus ; Pn, posterior nucleus of the optic thal- amus lying between the corpus geniculatum externum cge, and the corpus geniculatum internum cgi ; IC, internal capsule ; F, fillet ; By, red nucleus of the tegmentum ; FED, peduncle of the cerebrum ; Sn, substantia nigra ; OT, optic tract ; JT, optic chiasm ; //, optic nerve ; 1, 2, fibres from the retina to the pulvinar of the optic thalamus ; 1, centripetal ; 2, centrifugal ; 7, 8, fibres between the optic thal- amus and the occipital cortex ; 3, 4, fibres between the retina and the corpus geniculatum externum ; 9, 10, corresponding fibres to the occipital cortex ; 5, 6, fibres between the retina and the corpus quad- rigeminum anterior ; 11, 12, corresponding fibres to the occipital cortex — for the sake of distinctness the decussating optic fibres only are traced ; 13, cell of the superficial gray matter of the CQA passing to the nucleus of the third nerve (16) ; 14, cell of the deep gray of CQA passing to the third nerve nucleus ; 15, cell of the deep gray of CQA sending fibre into the fillet ; 17, fibre from the red nucleus, terminating about 14 ; 18, fibre from the fillet, terminating about 13 ; OL, occipital lobe of the brain with its cortex, containing both cells and terminal brushes of the visual tract, (Starr, Atlas of Nerve Cells.) THE SECOND NEEVE: THE OPTIC NEBVE. 627 and a bilateral temporal hemianopsia is caused, that is, a blindness in the outer portion of the visual field of both eyes. This is termed heteronymous hemianopsia. If the lesion involves the optic tract after the decussation a blindness in one-half of both eyes is caused, and the patient is deprived of seeing anything to one side of the middle line. This is termed homonymous hemianopsia. The relation in the optic nerves, optic chiasm, and optic tracts of the fibres from the nasal half (/»), temporal half {ft), and central spot (/to) of the retina ; Nil, optic nerve ; £11, optic tract ; xll, optic decussation. The left figure shows the fibres which cross ; the right figure shows those which do not cross; P, pupillary fibres. (Dejerine. ) In none of these forms of hemianopsia is central vision impaired. It is only aifected in lesions of the nerve itself. The acuteness of vision is best tested by Snellen's test types, type No. xx as seen at twenty feet being taken as indicating normal vision ^. The extent of the visual field is to be determined for each eye sepa- rately. This is to be done by having the patient fix each eye in turn on that of the examiner, the other eye being covered, and then notice whether he can see the hand of the examiner or a small white object held by him as it is passed outward from the centre of vision on all sides. The retina is less sensitive in its peripheral parts, and the less the illumination of the test object the less distinctly is it seen. The luminosity of blue, red and green are less than white in the order named ; hence the visual field is greatest for white, next for blue, next for red, and least for green. Contraction of the visual field for white or colors as well as defect in the visual field is an important symptom. The optic nerve contains some fibres which pass to the motor mechanism controlling the action of the iris. (Fig. 264, P.) Hence impressions of light cause a narrowing of the pupil. If one optic nerve only is destroyed the pupil reflex is lost in that eye when it is exposed to light. But as this reflex is bilateral, light in the unaifected eye will cause a contraction of the pupil in the blind eye. The fibres which sul^serve this pupil reflex decussate at the chiasm. Hence if 628 TEE CBANIAL NESVES AND THEIS DISEASES. one optic tract be injured, its function suspended, we have not only a bilateral hemianopsia, but also an interference with the pupil reflex. This is difficult to determine, for, as already stated, the reflex is a bilateral one, and light thrown on the unaffected parts of one of the retinae is capable of causing a prompt narrowing of both pupils. If, however, the patient be examined in a dark room, the eyes be only faintly illuminated, so that the pupils are fairly dilated, and then a ray of light be thrown by a mirror from one side carefully upon the blind side only of the retina, no pupil reflex will ensue. This indi- cates infallibly a lesion of the optic tract, or basal ganglia, as it does not occur in cases where the hemianopsia is due to lesion elsewhere in the visual tract, as, for example, in the occipital lobe. It is termed Wernicke's test for a hemiopic pupillary reflex. In lesions of the optic tract the hemianopsia affects color sense as well as white and object vision. It is only in affections of the visual centres in the cortex of the cerebral hemisphere that hemianopsia for color, with preservation of sight for white and objects, has been observed. This is called hemichromatopsia. (See page 122.) Diseases of the Optic Nerve. Hypersemia and anaemia of the optic nerve accompany correspond- ing conditions of the brain, but unless very extreme, are not easily recognized, even by experts, as the normal tint of the retina and the normal calibre of the bloodvessels differ in different individuals within very wide limits, like the color of the hair and of the skin. Growers states that a state of hypersemia may be admitted when the disk is dull red or brick color and when the edge of the disk cannot be distinguished from the rest of the retina. Such a condition is usually associated with a hypersensitiveness to light and pain on using the eyes. The causes of hypersemia are overaction or strain of the eyes due to refractive errors, or the use of the eyes in intense glare and heat, or inflammation of the conjunctiva, iris, or choroid, or some toxsemia, or some form of cerebral or spinal disease. The condition has been observed in many different types of insanity, but no form of insanity is particularly characterized by this symptom. It is a matter of dispute whether the chronic forms of spinal disease which lead to atrophy produce a preliminary hypersemia or ansemia. I have never observed any indication of either condition in cases which I have observed. The pallor of ansemia is almost as great as that of atrophy, and in the early stage of atrophy they caunot be distinguished. The causes of ansemia of the disk are those of general ansemia. A sudden ansemia due to embolism of the central artery of the retina always causes sud- den blindness in one eye, and the contrast between the appearance of the two eyes serves to establish the existence of unilateral ansemia. The arteries in one retina are seen to be small and empty. A moder- ate degree of ansemia may be caused by disease of the bloodvessels and DISEASES OF THE OPTIC NERVE. 629 may lead to temporary blindness. This was the case in a woman sixty years old, who had aortic obstruction and extensive general endarteritis not specific in origin. She had suffered from unilateral headaches for some years. On November 1st she took a coal-tar remedy for head- ache, which probably depressed the heart, and within a few hours she noticed a great dimness of vision in the right eye. This increased rapidly ; in two days she was nearly blind, and on December 1st the right eye was totally blind. Three days later vision began to return in this eye, and by January 1st was fairly good. But during the latter part of November the left eye began to fail, and by the middle of December, while the right eye was recovering, the left eye was totally blind. In February the left eye also improved, and she finally recov- ered vision in both eyes. During all this time the disks were pale, the arteries small but not obliterated, and the veins were distended. She could see better when lying flat on her back than when standing. The absence of visible neuritis, the marked arterial anaemia, and the exist- ence of general endarteritis with aortic obstruction, and the recovery, prove the symptoms to have been due to circulatory conditions. The treatment, which consisted of full doses of iodide of potassium, 90 grains daily, with strychnine and digitalis, was attended not only by relief of the blindness, but also of the headaches, which were probably due to the same cause. Thrombosis of the Retinal Artery may cause local ansemia of the retina, and may be followed by oedema and hemorrhagic effusion, a condition exactly like that which occurs in the brain. This was the condition found in the following case : A boy, aged thirteen years, had had a continued fever for six weeks with temperature between 99° and 102° F., supposed to be enteric, but probably malarial, as the blood examination showed the presence of two generations of tertian malarial parasites. He then developed dimness of vision in the left eye and then in the right. His optic disks were swollen three diopters, with flame-like appearance about the disk. In the left eye a wedge-shaped area extended up and in from the nasal side of the disk, and was color- less ; the retinal artery on this side was small ; the veins were normal, numerous dots about the macula indicating plastic exudation. This condition slowly improved. Later a marked picture of thrombosis of the retinal veins developed in the right eye, with a narrowing of the arteries and much plastic exudation about the disk, and a few retinal hemorrhages. This too gradually subsided, and after four months the optic nerves presented a fairly normal appearance and his vision had returned. The origin was presumably malarial infection, as nephritis was not present, and there were never any signs of cerebral disease. I have seen thrombosis of retinal arteries associated with symptoms of general cerebral arterial disease which subsequently caused death. It is U) be regarded, therefore, as a condition of prognostic importance as indicating a widespread arterial disease. The treatment must always be directed to a removal of the cause. 630 TEE CBANIAL NEEVES AND THEIR DISEASES. (Edema of the Optic Nerve. " Choked Disk." — The optic nerve at its exit from the eyeball receives a double sheath, one layer of which is continuous with the dura mater and the other with the pia mater. When fluid accumulates under pressure within the cranium it therefore finds its way into the space between these sheaths, distending the dural sheath and compressing the nerve at its exit from the eyeball. Such compression is felt earlier by the lymphatics and veins than by the harder nerve fibres, and the consequent result is a dropsical eifusion into the intraocular portion of the nerve from transudation of fluid from the obstructed vessels. This causes a swelling of the optic disk visible to the ophthalmoscope and causing an appearance shown in Fig. 265. Such a swelling may exist for some time without any impairment Fig. 265. Swollen disk in a case of chronic meningitis. (Liebreich.) of vision, hence the importance of ophthalmoscopic examinations in cases without ocular symptoms. The experiments of Merz^ have demon- strated that this swelling of the nerve may come and go rapidly and may vary greatly in degree, according to the varying amount of intra- cranial presisure. The oedema is always attended by venous congestion, but no inflammatory changes may occur, and it is therefore erroneous to identify choked disk with optic neuritis. It is not infrequently accompanied by hemorrhages in the retina. Choked disk will occur under any condition which obstructs the circulation in the eye or in- creases the intracranial pressure. It is very common in tumors and ^ Merz, Arch, of Ophthalmology, July, 1901. DISEASES OF TEE OPTIC NEEVE. 631 abscesses of the brain, occasionally occurs temporarily after large hemorrhaged, and not infrequently accompanies Bright's disease or those affections in which general oedema is a symptom. The only positive means of diagnosis is by ophthalmoscopic exami- nation. In many cases when the choked disk has lasted for some time vision is impaired and the visual field is diminished in extent. In a few cases it produces no symptoms. In some cases it causes a rapidly advancing blindness. But in any case this blindness may pass away as the oedema of the nerve subsides, provided a true neuritis or atrophy does not ensue. Choked disk is usually bilateral, but may commence in one eye some time before it appears in the other. In some cases it goes on to neuritis and to atrophy. Cases have been recorded of choked disk without other symptoms and without apparent cause. In two such cases, reported by Brudenel Carter,^ unsuspected tumor of the brain and latent abscess of the sphenopalatine fossa were eventually proven to be the cause. Optic Neuritis. Etiolog"y. — Optic neuritis occurs occasionally with- out apparent cause, and must be regarded as an idiopathic affection. It . has been known to be hereditary, and develops about the age of twenty and goes on to blindness. In the majority of cases, however, it is secondary to other conditions. It may be due to general poisons in the blood, such as produce multiple neuritis, namely, alcohol, lead, arsenic, mercury, and tobacco. It is the pathological state which is present in so-called toxic amblyopia and in the affection described by ophthal- mologists as retrobulbar neuritis ; where the fibres passing from the macula lutea are especially involved. It may occur as a sequel of any of the infectious diseases, especially malaria. It also occurs occasion- ally as a symptom of severe chronic anaemia, of pernicious anaemia, leucocythsemia, and diabetes. It may be syphilitic in origin. It is a frequent symptom of nephritis. It is supposed to be caused by rheu- matism, by gout, or by taking cold. Optic neuritis may be secondary to a meningitis, in which case the inflammation extends directly along the sheath of the nerve. Its most common cause, however, is from pressure within the skull exerted either directly by tumor or abscess of the brain, or syphilitic exudations upon the base of the brain, or by the pressure of fluid in the ventricles. Much discussion has been de- voted to the cause of optic neuritis in intracranial disease. It may be due to the increase of pressure within the head, producing oedema of the nerve trunk and a secondary neuritis, as Parinaud affirms. This theory is confirmed by the fact of the rapid subsidence of all the ap- pearances of optic neuritis in cases of brain tumor when the skull is opened and the intracranial pressure relieved. Thus in one case recorded by Harvey Gushing^ a swelling of seven diopters was entirely reduced within twenty-four hours of an operation and within a week the optic neuritis had wholly disappeared. It may be due to a direct infection of the nerve by producits of disease within the brain, as Leber and Deutschmann have long maintained, although the rapid subsidence 1 Clifford Alllnitt'H System of Medicine, vol. vi., p. 842. * Surgery, Gynaicology and Obstetrics, Oct., 1905. 632 TEE CRANIAL NEEVES AND THEIB DISEASES. after relief of pressure throws doubt upon this theory. In all these cases optic neuritis is usually bilateral, though in tumors of the brain, upon the base, with direct pressure upon the nerve, it is possible for the nerve on the side of the tumor to be affected much sooner than the other. Symptoms. — The symptoms of optic neuritis are a sudden or gradual failure of sight, objects becoming blurred, as if a fog were between the Fig. 266. Acute optic neuritis. Note disk much swollen ; estimated at + 7 D. Edge indistinct ; vessels obscured at edge ; large number of hemorrhages around the disk, patches of oedema in the retina, veins very tortuous. (Posey.) eye and the object. Some patients complain of sensitiveness to light and of distress in using the eyes. There is also a diminution of the visual field and a lack of distinctness of color vision, blue being lost first. In some cases irregular blind spots develop in the visual field. It is rare for total blindness to occur as the result of optic neuritis within six months of the onset. The progress of the blindness is con- tinuous unless the cause can be removed. Diagnosis. — A diagnosis can be made only by an ophthalmoscopic examination. The figures on Plate XXVI., show the appearance of a normal retina and of a nerve in the early stage of optic neuritis. (See also Fig. 266.) It will be noticed that the changes are very marked in the bloodvessels of the retina, the arteries being smaller and the veins being distinctly congested. Their point of exit in the disk is often obscured. It will be noticed that the outline of the disk in the early stage is no longer clear and in the later stage is completely obliterated. The peculiar striated appearance about the disk is quite evident in the fiarures, beins: more marked in the later stage of the disease, and this striation is partly yellowish and partly red, in contrast to the uniform PLATE XXVI Fig. 1. Normal Eye-ground (average tint). (Norris and Oliver.) Fig. 2. Ophthalmoscopic Appearances in Early Stage of Optic Neuritis. (Norris and Oliver.) DISEASES OF THE OPTIC NERVE. 633 color of the retina in health. In the later stage hemorrhages in the retina are Evident, being indicated by red or yellowish-white plaques. Small whitish or yellow spots are also seen which are due to exudation of inflammatory products. There is an actual exudation within the nerve trunk, so that the optic disk is actually swollen. Its height can be estimated by the ophthalmoscope (1 diopter equals f mm.), but the first evidence of this swelling is the disappearance of the clear central space known as the physiological cup. If the condition subsides there is a gradual reduction of this swelling and little by little a return to the normal appearance of the disk. While the diagnosis of the exist- ence of optic neuritis is easily made by the aid of the ophthalmoscope, it is by no means easy to determine in every case to what the condi- tion is due. If to the symptoms of neuritis there are added headache, malaise, attacks of vertigo, disorders of digestion, with nausea and vomiting, and some irregularities of circulation, and the patient's health, strength, and nutrition suffer the question at once arises whether these are the symptoms of tumor of the brain, chronic tubercular men- ingitis, general arterial disease, nephritis, or merely of general anaemia. I have seen two patients suffering from these symptoms during several months and not a little distressed mentally lest they might be develop- ing a fatal affection. Both were young women suffering from anaemia and also from very irregular painful menstruation due to endometritis. But in both the severity of the nervous symptoms had awakened the fear of brain tumor. Both eventually recovered under general tonic treatment. In such cases the diagnosis can be reached only after a considerable time, the lack of development of local brain symptoms, especially in the cranial nerves, the existence of normal powers of sen- sation and motion, and of normal reflexes being negative symptoms of great value. On the other hand, the appearance of localizing brain symptoms may confirm the suspicion that the neuritis was the first sign of serious intracerebral disease, especially of tumor or abscess. I have seen this condition as an early symptom of serious arterial disease, which led rapidly to multiple cerebral thrombosis with aphasia and hemiplegia and death. It is well known that a very common cause of optic neu- ritis is Bright's disease, and it follows that an examination of the urine should be made in every case. It is never to be forgotten that abuse of alcohol and tobacco combined may be the cause of optic neuritis'and may also produce other nervous symptoms. I have seen such a con- dition in young women addicted to champagne and cigarettes, as well as in many young men of nervous constitution who were victims of the same habits. It is evident, therefore, that in any case of optic neuritis great caution should be exercised in giving a prognosis, every etiological factor should be investigated, and the course of the case should be watched with extreme care. The lesion in optic neuritis is similar to that already described as a diffuse neuritis (page 164) there being a congestion of the vessels, an effusion of serum and blood cells, of leucocytes, and nuclei in the endoiicurium, as well as a })arenchymut()us (hsgencration of tlie nerve fibres. Prognosis. — The prognosis in the affection will depend entirely upon 634 TEE CBANIAL NEBVES AND THEIB DISEASES. the possibility of removing the cause. Even when the optic neuritis has been quite extreme recovery is still possible. Thus, after absorp- tion or removal of tumors of the brain I have frequently seen recovery of vision when the blindness had been so extreme as to incapacitate the patient from recognizing even the largest letters upon the test card. Treatment. — The treatment for the atfection is the removal of the cause, if possible, and complete rest of the eyes, which should be shaded from the light. The ophthalmologists prescribe strychnine by hypo- dermic injections or by the mouth in large doses, -^q- grain, increased at each injection until in ten days |- grain is given, and this drug will certainly stimulate the nerve to activity, even when in a state of neuritis. Whether such stimulation is desirable may be open to question, and certainly this treatment has no eflfect in other forms of neuritis, excepting a temp3rary effect of stimulation of the functions of the nerve. The use of mercurial inunctions and of full doses of iodide of potassium is recommended both in syphilitic and non-syphi- litic cases. The local depletion by wet cups or leeches to the temple is often beneficial in the early stage. In anssmic cases general tonics are of benefit. Gushing advises that in all cases of optic neuritis or of choked disk, whether from intracranial pressure or from Bright's disease, a relief of pressure be secured either by frequently repeated lumbar punctures or by decompressing operations upon the cranium. In this way sight may be saved. See page 613. Optic Nerve Atrophy. Etiology. — Optic neuritis in many cases goes on to a gradual atrophy of the optic nerve, which is then spoken of as consecutive, and in such cases the atrophy is the terminal result. Increased pressure within the cranium may produce atrophy without any primary neuritis, the nerve appearing to undergo a degeneration from the start, there being no initial process of congestion, such as occurs in neuritis. A secondary atrophy also occurs after lesions of the nerve in the orbit or after affections of the optic chiasm and tract. Optic atrophy may, however, occur as a primary affection without any preceding intracranial pressure or disease. Some cases are congenital or develop very soon after birth from actual congenital defective development of the optic nerve. In these cases other nervous symp- toms usually indicate an imperfect development of the nervous system, such as imbecility and idiocy, or spastic paralysis. These cases have been described most fully by Sachs.^ Vogt has observed the same con- dition developing in children at the age of eight or ten. In many cases primary optic atrophy is a condition associated with other diseases, though it can hardly be called secondary to them. This is notably the case in locomotor ataxia, in disseminated or multiple sclerosis, and in paresis. In all of these diseases the occurrence of optic atrophy is an exceedingly serious complication and probably due to the same cause which produces the original affection. It may be the first lesion pro- duced by this cause. Thus in locomotor ataxia 10 per cent, of the cases ^ Amaurotic Family Idiocy, Journal of Nervous and Mental Disease, 1887, p. 541, and 1903, p. 1. PLATE XXVII Primary Atrophy of Optic Nerve (Spinal Post-papillitic or Consecutive Atrophy of Atrophy). Modified from Haab. the Optic Nerve. Modified from Juler. Embolic Atrophy of the Optic Nerve. (De Schweinitz, in Dercum's Nervous Diseases.) DISEASES OF THE OPTIC NEEVE. 635 begin with optic atrophy. And in multiple sclerosis 52 per cent, of the patients de^i^elop this complication. Optic atrophy may occur as the result of poisoning by tobacco, alcohol, lead, and quinine. It may occur as the result of the various infectious diseases and of diabetes, and in some cases has been ascribed to cold. Symptoms. — The symptoms of optic atrophy are a gradually increas- ing blindness, a diminution of the visual field, progressively increasing from without inward, so that finally only central vision is possible, and very often a defective color vision, the fields for various colors being either progressively reduced in extent or altered in their extent. In some cases irregularly situated blind spots, sometimes central, develop — the so-called central scotoma. Pain in the eyes and discomfort after their use or after exposure to strong light are often felt. The appearances in optic atrophy are demonstrated in Plate XXVII. where both gray and white atrophy are shown. The ophthalmoscopic appearances in optic nerve atrophy are not uniform. When the atrophy is secondary to the optic neuritis there are, first, the ordinary changes observed in the neuritis, and then, as the swelling and congestion sub- side, the disk once more becomes evident, has a clearer and clearer out- line, but an unusually white appearance, until finally it presents a perfectly white spot in the retina. The arteries are noticeably dimin- ished in calibre. When the optic atrophy is primary and is not pre- ceded by a neuritis there is a gradual progressive change in the appear- ance of the normal disk, which becomes more sharply outlined, loses its pink tint, and becomes more and more gray, or even clear white in color, the arteries and veins preserving their normal calibre. After a time the lamina cribrosa at the bottom of the disk may become evident, the ap- pearance presented resembling a fine sieve. The lesion in optic atrophy is a progressive parenchymatous neuritis without any interstitial change. Diagnosis. — The diagnosis of the underlying condition in a case of optic atrophy is of as great importance as in a case of optic neuritis. Toxic amblyopia should be first excluded. Symptoms of organic brain or spinal-cord disease should be looked for. The mental characteristics of the patient should be considered, and changes indicative of begin- ning paresis should not be overlooked. And the case should be watched for a long period before giving a diagnosis of primary optic atrophy. More than one-half of the cases eventually develop some form of spinal sclerosis. Prognosis. — The prognosis in optic nerve atrophy is much more serious than in optic neuritis, for while the latter may go on to recovery, the former is progressive, and treatment, unless directed to the cause, is not successful. Treatment. — The progress of the case may be delayed by the use of tonics, by everything which improves the general health, and by the hypodermic use of strychnine, from ^ up to |- grain daily, the dose being increased slowly, or of the chloride of gold, or nitrate of silver, or of nitroglycerin. Recently hypodermic injections of normal salt solution beneath the conjunctiva havfi been recommended. They have not been useful in my cases. 636 TEE CMANIAL NE:rVES AND THEIH DISEASES. THE THIRD, FOURTH AND SIXTH NERVES : THE MOTOR NERVES OF THE EYEBALL. OPHTHALMOPLEGIA. The eyeball and eyelid are moved by numerous muscles supplied by these three nerves, as follows : The third nerve supplies the ciliary muscle, sphincter iridis, levator palpebrse, superior rectus, internal rectus, inferior rectus, inferior oblique. The fourth nerve supplies the superior oblique. The sixth nerve supplies the external rectus. The branches of the cavernous plexus of sympathetic nerves sup- ply the dilator pupillse. These nerves arise from groups of cells lying upon the floor of the aqueduct of Sylvius and of the fourth ventricle of the brain. (Fig. 267.) The groups are closely joined to Fig. 267. Sagittal section through the cerebral axis, to show the nuclei of the ocular nerves in the floor of the aqueduct of Sylvius and the fourth ventricle, and the course of the nerves to their exit. The various groups of cells from which the third nerve arises are seen. RN, red nucleus of tegmentum ; L, lemniscus (sensory tract) ; CC, motor tract in the crus cerebri seen to traverse the pons and enter the anterior pyramid of the medulla. each other by association fibres, the longest of which, between the third and sixth nerve nuclei, pass in the posterior longitudinal bundle. The location of the nuclei of the oculomotor nerves and the function of each group of cells has been the subject of much discussion, and unanimity of view has not yet been reached, though the subject has been approached from the side of comparative anatomy (Spitzka, Edin- ger), embryology (von Gudden, Edinger), physiological experimenta- tion (Hensen and Volchers, Bernheim), and pathology (Westphal, Kahler, Pick, and Starr), as well as from the histological standpoint (Kolliker, Perlia, Bruce). The third nerve nucleus consists of a long column of cells which begin in the gray matter of the wall of the third ventricle near the MOTOB NEBVES OF THE EYEBALL. OPHTHALMOPLEGIA. 637 posterior commissure on the median surface of the optic thalamus, and extends for almost a centimetre in length under the floor of the aque- duct of Sylvius downward to the level of the corpora quadrigemina posterior. Just posterior to this column lies the group of cells giving origin to the fourth nerve, and about 3 cm. below in the pons is the sixth nerve nucleus. The column of cells making up the third nerve nucleus can be divided into a number of groups. These groups, their connections, and their special functions are shown in Figs. 267 and Fig. 268. Nucl. med. ant. Nucl. Edinger- Westphal Levels of sections frmn which the figures ac- companying Perlia's article were taken. Nucl. lal. ant (Darkschewitsch) Nucl. dors. I {ant.) Nucl. vent. I {ant. Nucl. centralis Nucl. dors. II {post.). [v. Gudden) Nucl. vent. II ( post. ) Nucl. Trochl. Scheme of the nuclei of the oculomotor nerve. (After Perlia, Arch. f. Ophth., Leipzig, Bd. XXV., Abth. iv., S. 297.) 268. That each group controls a single muscle is proven by the fact that small lesions limited to different parts of the column of cells and affecting certain groups or their nerve roots only produce different forms of paralysis of the eyeball. A study of the combination of paralyses observed in various cases enabled the writer (in 1888^) to offer the following scheme of the order in which the muscles are rep- resented in these groups : 1 .Joum, of Nerv, and Ment. Dis., vol. xv.,p. 301, 638 THE CBANIAL NEBVES AND THEIE DISEASES. Table XVII. — Arrangement of III. Nerve Nuclei. Eight Side. Median line. Ciliary muscle. Sphincter iridis. Eectus internus. Levator palpebrae. Rectus superior. Rectus inferior. Obliquus inferior. Obliquus superior. Rectus externus. Subsequent observations have lent support to this localization. Third Nerve Paralysis. The third nerve, after arising from its groups of cells upon the floor of the aqueduct of Sylvius, sends its various roots through the teg- mentum of the crus cerebri, which traverse on their way the red nu- cleus of the tegmentum and make their exit in a large bundle on the inner side of the crus in close apposition to the third nerve of the op- posite side. Lesions of the groups of cells of origin of the third nerve may produce a partial paralysis of the ocular muscles. So, too, may lesions of the roots of the third nerve in their passage through the crus. But lesions of the nerve after its exit cause paralysis of all the muscles which it supplies. The third nerve passes from its superficial origin in the crus forward to the sphenoidal fissure, and thus into the orbit, where it divides up in the various small branches going to the muscles already named and to the ciliary ganglion. Etiology. — Lesions upon the base of the brain lying near the crus or between it and the exit of the nerve into the orbit are particularly liable to produce paralysis of the third nerve. Meningitis, either sim- ple or tubercular, abscess of the brain, and hemorrhages on the base often involve the nerve. In meningitis in infants it is often the nerve first affected. Syphilitic exudations upon the base of the brain and a syphilitic neuritis with infiltration of the sheath of the nerve are the most common causes of third nerve palsy. Tumors of the brain lying in this locality or lying in such position as to displace the brain axis, and thus cause traction upon the nerve, may also produce third nerve paralysis. In both these conditions other symptoms of the disease accompany the ocular palsy and give a clue to the diagnosis. The ocu- lomotor nerve is rarely injured within the orbit, inasmuch as it is well protected, but occasionally in fractures of the base the nerve will be torn, and severe falls and blows upon the head have been known to cause the bruising of the nerve against a sharp edge of the sphenoidal fissure. Thus in a patient under my own observation a fall from a car- riage upon the side of the head produced a sudden paralysis of the third nerve which remained about six months and' then gradually recovered. Tumors within the orbit and anything producing extreme exophthal- mus may by pressure or stretching involve one or more branches of the oculomotor nerve, and thus cause a partial paralysis. Thus in a patient with exophthalmic goitre and very extreme exophthalmus I have seen an inability to turn the eye inward and upward which sub- sided when the exophthalmus disappeared. Oculomotor palsy may TRIBB NEBVE PAEALYSIS. 639 occur as a complication of diphtheria and of typhoid fever, and, in fact, it has bee^ recorded in many different forms of multiple neuritis ; under these circumstances it is usually bilateral. German authorities afl&rm that a temporary oculomotor palsy may be produced by poison- FiG. 269. A section through the cms cerebri, showing the origin, course, and exit of the oculo- motor nerves. (Edinger.) ing with sausage, fish, or meat that is diseased. Lastly, it is supposed that the eifect of cold may produce a sudden oculomotor palsy of tem- porary duration, but this is certainly very rare. Symptoms. — The symptoms of third nerve paralysis are ptosis or falling of the upper eyelid, dilatation of the pupil, and loss of reflex action both to light and to accommodation and a turning outward and slightly downward of the eyeball owing to the unopposed contraction of the external rectus and superior oblique muscles. The strabismus is attended by double vision and often by vertigo and uncertainty of gait. There is an inability to turn the eyeball upward, downward, or inward, owing to the paralysis of the superior, inferior, and internal recti muscles. The paralysis of the inferior oblique muscle is also present, but does not produce a visible deflection of the eye when the superior oblique is capable of acting. Ptosis is complete, so that the patient is unable to open the eye sufiiciently to see, although he usually makes efl'orts in this direction by elevating the eyebrow to the utmost limit ; hence in such patients wrinkling of the forehead is more pro- 640 THE CBANIAL NERVES AND THEIB DISEASES. nounced on the side of the paralysis. Fig. 270 shows this condition. In cases where the cause can be removed by treatment the prognosis is good, and even in cases of injury and disease of the nerve from cold, recovery, as a rule, ensues. Fig. 270. The nerve is inaccessible to any form of electrical treat- ment, therefore it is impos- sible to treat the paralysis. The Pupil and Its Nervous Mechanism. — The action of the pupil to light and to ac- commodation is a reflex act in which the optic, oculo-motor, and sympathetic nerves take part. The contraction of the pupil to light is produced by im- pulses passing from the retina to the corpora quadrigemina anterior ; thence to the ante- rior group of cells in the third nerve nucleus, thence in the third nerve to the ciliary gan- glion, from which the short ciliary nerves go to the iris, which contracts by closure of its sphincter. Any lesion which breaks this reflex arc will arrest the contraction of the pupil to light. The dilatation of the pupil in darkness or in distant vision is produced by impulses passing to the dilator pupillse through the sympathetic nerves from the cilio-spinal centre in the eighth cervical and first dorsal segments of the spinal cord. These spinal centres are connected with the corpora quadrigemina by nerve fibres which pass in the tegmentum of the pons and medulla and in the antero-lateral columns of the upper cord. The sympathetic nerve fibres leave the spinal cord in the first dorsal nerve root, pass to the superior cervical ganglion, thence in the carotid or cavernous plexus to the Gasserian ganglion of the fifth nerve, and thence by way of the first branch of the fifth nerve and the long ciliary nerves to the iris. The cilio-spinal centre in the spinal cord receives impulses from many directions, any one of which is capable of producing a dilatation of the pupil. Thus irritating impulses coming in through the lower cervical and upper dorsal posterior spinal nerve roots from the skin of the neck, or if of sufficient intensity from any region of the body, can cause such a dilatation. It is possible that the total absence of such impulses when the posterior nerve roots in the cervical cord are injured causes the per- manent contraction of the pupil, called spinal myosis. Impulses also reach the cilio-spinal centre from the cortex of the bra,in and from many Ptosis and external strabismus due to paralysis of the right third nerve. (Icon, de la Salpetriere. ) THIBD NEBVE P ABA LYSIS. 641 subcortical centres in the basal ganglia and medulla. Attention directed to a distant'' object, with voluntary fixation of the eyes, or emotional changes, fear or anger, or any irritation of the medulla which excites the respiratory and vasomotor centres may cause dilatation of the pupil. It is the last-named cause which produces a contracted pupil in men- ingitis and cerebral disease. A general lowering of the nervous con- dition such as accompanies neurasthenia, hysteria, epilepsy, and anaemia is usually attended by a dilatation of the pupil. The exact path from the optic centres to the spinal centre is as yet undiscovered. A per- manent loss of power of dilatation of the pupil in darkness is known to occur only when the cilio-spinal centre in the cord or the sympa- thetic nerve in the neck or its branches to the dilator pupillse are destroyed by disease. This occurs in tabes, in paresis, in multiple Fig, 271. Diagram of the visual tract and its connection with the third nerve. JV.o., optic nerve ; ehs., chiasm ; tr. o., optic tract ; gen. Za<., corpus geniculatum externum ; bra., brachum conjunctivum ;cg. a., corpus quadrigeminum anierioT ; pulv., ulvinar ; ^rera. m., corpus geniculatum internum; tor., teg- mentum; a.s., aqueduct of Sylvius. (Jacob.) sclerosis, and in bulbar palsy, and in myelitis of this locality. The hemiopic pupillary reflex of Wernicke has been described on page 628. When a patient presents anomalies in the action of the pupils the pos- sibility of his being under the influence of some drug which afiects their action must be considered before a conclusion is reached that a local or general disease of the nervous system is present. Argyll-Robertson Pupil. — This is a condition of loss of reflex of the pupil t() light, while its action in accommodation is preserved. (See pages 3/3G and 045.) 41 .642 THE CBANJAL NEBVES AND THEIB DISEASES. Paralysis of the Fourth Nerve. Patheticus Paralysis. Paralysis of the fourth nerve is extremely rare aud is ouly to be detected by a careful examination for double images with a red glass over one eye. The upright image is then seen to be upright by the normal eye, but appears to be displaced outward and turned obliquely by the affected eye. The fourth nerve supplies the superior oblique muscle and is of all the nerves the one best protected within the brain. It arises from a small group of cells beneath the aqueduct of Sylvius, and decussates with its fellow in the roof of the aqueduct just beneath the corpora quadrigemina posterior, and makes its exit immediately after its decussation on the dorsal surface of the crus lying upon the superior peduncle of the cerebellum. (Fig. 267.) It then curves around the crus, lying upon the upper border of the pons Varolii, and so passes forward upon the side of the optic tract and enters the orbit through the sphenoidal fissure. Any of the causes which produce paralysis of the third nerve may cause paralysis of the fourth. Snyder ^ reports several cases due to injury, attended by vertigo. Isolated paral- ysis of the fourth nerve without affection of the third or sixth is an important symptom of tumor of the cerebellum or of exudation on the under surface of the anterior lobe of the cerebellum. In a patient seen in Meynert's clinic at Vienna, who presented many symptoms of cerebellar disease, the existence of an isolated fourth nerve paralysis made it probable that the cerebellar lesion lay in the anterior and ven- tral part of the cerebellum, a diagnosis which was confirmed by autopsy. Paralysis of the Sixth Nerve. Abducens Paralysis. The symptom produced by paralysis of the sixth nerve is internal strabismus. This nerve supplies the external rectus, and when it is paralyzed the internal rectus, being unopposed, turns the eye inward, producing strabismus and an inability to turn the eye outward. Double vision always accompanies the strabismus, and the pupil is usually somewhat contracted on account of the position of the eyeball, but, of course, reacts to light. The sixth nerve arises from a group of cells upon the floor of the fourth ventricle, passes ventrally through the pons Varolii, making its exit in the groove between the pons aud medulla, and then passes forward beneath the pons to enter the orbit through the sphenoidal fissure. As it has the longest course of any cranial nerve, disease at any part of the base of the brain is liable to involve it, hence abducens paralysis is a frequent symptom of intra- cranial disease, such as basilar meningitis, syphilis of the base, tumors of the brain, and fractures at the base of the skull. The same causes which produce oculomotor paralysis may cause abducens paralysis, and it is equally inacessible to any treatment. The sixth nerve nucleus and the root of the nerve are often involved in disease of the pons Varolii. (See pages 117 and 615.) lArch, of Ophthalmologj, vol. XXXVI., p. 988. PABALYSIS OF THE SIXTH NERVE. ABDUCENS PARALYSIS. 643 The diagnosis between an affection of the abducens, due to disease in the ner^ trunk or due to disease in the brain, can only be made from the presence of other symptoms. Paralysis of the face on the same side as the ocular palsy, and of the arm and legs upon the oppo- site side, indicates a lesion in the pons or on the base, producing uni- lateral pressure on the pons. In the patient whose face is shown in Fig. 272 the paralysis of the sixth nerve was associated with Fig. 272. paralysis of the seventh and eighth nerves on the same side. This patient had suffered from a fracture of the base of the skull, but had recovered from all the symptoms excepting those present in these three nerves. The Conjugate Motions of the Eyes. — In all acts of vision the eyes move together, either being converged to a near object, diverged in looking at a distance, or turned together in any di- rection. In convergence the internal recti act together and there is a contraction of the pupil. In divergence the external recti act together and there is a dilatation of the pupil. In conjugate lateral motions opposite muscles in the two eyes act together. In motions upward and downward homogeneous muscles act together. All these movements are primarily reflex and automatic, the sensations of objects upon the retina causing the appropriate action. For such acts the interaction of the various nuclei of the optic and motor nerves of the eyeballs is all that is necessary. And the very complete system of association fibres joining these various nuclei to one another provides for such combinations of movement. Lesions which destroy these association fibres, especially lesions in the posterior longitudinal bundle, interfere with conjugate lateral motions. Lesions in the nuclei themselves necessarily suspend consensual motions in any direction, both reflex and voluntary. The connections of these nuclei are shown in Fig. 267. Conjugate movements may also be voluntary in origin. The motor centres in the cerebral cortex for ocular movements are located in the anterior part of the motor zone. (See page 92.) They send their impulses down to the subcortical centres by a tract which passes through the knee of the internal capsule, occupies a position near the median line in the foot of the cms cerebri and decussates in the raph6 before it ends in the oculomotor nuclei. The tract conveying impulses Paralysis of the left sixth, seventh, and eighth nerves from a fracture at the base of the skull. 644 THE CRANIAL NEBVES AND THEIB DISEASES. of conj agate lateral motion passes directly to the sixth nerve nuclei, and thence an impulse is sent back to the opposite third nerve by way of the posterior longitudinal bundle. Fig. 273 shows this tract and demonstrates the fact that lesions at different parts of it cause paralysis of lateral conjugate motion of the two eyes. Irritation of the motor centres in the cortex causes involuntary turn- ing of the eyes away from the side of the lesion. This happens in epilepsy when the focus of irritation which starts the fit is unilateral. Paralysis of the motor centres in the cortex, or destruction of the tract which conveys their impulses will eventually result in conjugate deviation of the eyes toward the side of the lesion, for the eyes being once turned in Fig. 273. Diagram to illustrate the mechanism of conjugate movements of the eyes. P, pons Varolii ; ///, oculomotor nuclei ;. VI, abducens nuclei ; S.i., rectus internus ; R.e., rectus externus. A tract from the cortex passes to VI. A tract connects each F/ ^ ith the opposite ///. C, centre of convergent move- ment of both eyes — a part of ///; A, lesion in the left half of the brain, causing a loss of voluntary turning of both eyes to the right ; B, lesion in the left half of the pons, causing a loss of voluntary turning of both eyes to the left ; C, lesion in the left posterior longitudinal bundle, causing a loss of voluntary conjugate motion of the eyes to the right ; D, lesion in the left VI nucleus, causing a loss of voluntary turning of the eyes to the left. that direction remain fixed, there being no possibility of a voluntary correction of this position. Hence in severe attacks of apoplexy when this tract is destroyed the patient is said to look toward his lesion. The diagnosis of the situation of a lesion producing forced conjugate deviation of the eyes can only be made from the existence of other symptoms of cerebral or pons disease. These symptoms have been more fully discussed in Chapter V. Rheumatic Ocular Palsy. — A paralysis affecting one or more of the ocular muscles must not be too hastily ascribed to a lesion of the nerve. These muscles are subject to rheumatic affections like other OPHTHALMOPLEGIA. 645 muscles, an^ occasionally a supposed ophthalmo])legia turns out to be a rheumatic myositis, and subsides promptly. The external rectus is the muscle most frequently affected. Recurrent Oculomotor Palsy. — A rare condition consisting of combined paralysis of several of the muscles of temporary duration, but commonly recurring several times in the course of a short attack, has been described and named recurrent oculomotor palsy. The oculo- motor nerve is the one usually affected, but the fourth and sixth may also be involved. The attacks of double vision are attended by head- ache, nausea, vomiting, and by a fever, and occasionally by conjunctivitis. They have been termed ophthalmic migraine by Charcot. In one case seen by me the cause was probably malarial infection and the attacks ceased under quinine. Darquier^ and Knapp^ have collected and analyzed about forty cases. The lesion is probably a vascular one, congestion or oedema either in the nuclei or in the nerve trunks. In one case a vascular tumor was found on the trunk of the third nerve. Ophthalmoplegia. Disease in the nuclei of the nerves that supply the muscles of the eyeballs and paralyze them is termed ophthalmoplegia. It has been divided into ophthalmoplegia interna and externa, according to the muscles afPected. In ophthalmoplegia interna the action of the pupil is afPected. In ophthalmoplegia externa the motion of the eyeballs and of the levator palpebrse are impaired. Ophthalmoplegia Interna. — Ophthalmoplegia interna is a very rare condition. It may be unilateral or bilateral. It may be acute or chronic in its onset. There are no cases with autopsy on record and the condition as a symptom is a curiosity. Loss of reflex to light while reflex action in accommodation is pre- served, called the Argyll-Robertson pupil, or reflex iridoplegia, occurs as a symptom in tabes, in paresis, in disseminated sclerosis, in soften- ing or atrophy of the brain, in syphilis of the brain, in hydrocephalus, in tumors of the third ventricle or adjacent basal ganglia, and as a congenital condition. It is supposed to point to a suspension of action in the association neurones between the optic terminals and the oculomo- tor ciliary nuclei. These axones pass through the gray wall of the aque- duct of Sylvius, and in all these diseases except tabes microscopic lesions in this region have been observed. There is as yet no satisfac- tory explanation for the occurrence of this symptom in tabes. The condition is usually eventually bilateral, but may be unilateral at first. Loss of action of the pupil in accommodation is known to occur with- out loss of action to light. This is a rare condition, is usually uni- lateral and due to a lesion in the cervical sympathetic. Ophthalmoplegia Externa. — This term is applied to any form of paralysis of the muscles moving the eyeball, not due to a lesion in the ' Annales d'oculistlque, Oct., 1893, tome ex., p. 258. 2 Boston Med. and >Surg. Jour., Sept., 1894. 646 THE CBANIAL NEUVES AND THE IB DISEASES. periphery of one nerve alone. It is usually bilateral. It is usually partial, a few only of the ocular muscles being involved. It may, however, become total, under which circumstances the eyeballs are mo- tionless. Inasmuch as the muscles affected are numerous, almost every possible combination of paralysis of different muscles has been observed. It is not, therefore, necessary to specify the different position of the eyeballs possible and the different forms of double vision possible in ophthalmoplegia. Etiology. — The symptoms may be produced either by lesions in- volving the roots ol the oculomotor nerves in their passage through Fig. 274. Fig. 275. Normal oculomotor nucleus. 1, ventral nu- cleus ; 2, median nucleus ; 3, lateral nucleus ; 4, posterior longitudinal bundle ; 5, 6, nerve roots ; 7, raphe. Atrophy of oculomotor nucleus. 1, ventral nucleus ; la, Dorsal nucleus ; 2, 3, Edinger- Westphal nucleus. (Siemerling. ) the cerebral axis, or by lesions limited to their nuclei. Lesions involv- ing the roots are very coromon. They are due, as a rule, to hemorrhage from, or thrombosis or embolism in the small arteries, and especially in their terminal branches within the crus cerebri. These arteries enter through the posterior perforated space, and are terminal arteries. Tumors, spots of sclerosis, or syphilitic exudations in this region cause the same effect. The majority of cases on record of ophthal- moplegia externa which have been examined pathologically have been produced in this manner. Such lesions necessarily cause a destruction of other nerve tracts passing through the cerebral axis in addition to OPHTHALMOPLEGIA. 647 the oculomotor roots. It is from the presence of symptoms of such destruction that the pathological diagnosis is to be reached. Thus hemiansesthesia, hemiataxia, or hemiplegia of various types have been observed in association with partial ophthalmoplegia. And such an affection of the sensory and motor tracts in the cerebral axis may be bilateral, causing very extensive and complex groups of symptoms of which the oculomotor palsies may be the least noticeable, though they may be the most important as a guide to the localization of the lesion, (See Figs. 53 to 56.) The condition may also be congenital ; Brad- burne ^ has recorded an hereditary type, examples being seen in five generations in one family. Lesions involving the nuclei of the oculo- motor nerves may be either acute or chronic, and resemble in charac- ter the lesions of anterior poliomyelitis. Pathology. — Acute ophthal- Fig. 276. moplegia externa may occur from hemorrhage in the nuclei, from thrombosis or embolism in ter- minal vessels supplying the nuclei, or from inflammatory processes, probably infectious in origin, in the nuclei. It may also be the result of poisoning by bad food, Wernicke has named this condition polioen- cephalitis acuta. (See page 538.) The lesion found is a congestion of the vessels, exuda- tion from them of small cells and leucocytes and serum, swell- ing and degeneration of all pos- sible degrees in the motor cells, with destruction of their den- drites and axones, and conse- quent degeneration in the nerve roots and nerves. This lesion may be limited to the oculomotor nuclei, or it may be extensive in the cerebral axis, affecting other cranial nerves, in which case the ophthal- moplegia is only a part of an acute bulbar paralysis. A chronic atro- phy of the nuclei has also been described and is shown in Fig. 275. These lesions resemble in every particular the lesions of anterior poliomyelitis. Symptoms. — The onset of acute ophthalmoplegia is usually sudden, with severe cerebral symptoms, vertigo, vomiting, headache and even delirium and coma. The patient is always somnolent for some days, and occasionally develops an optic neuritis as well as the ocular palsy. In a few cases other symptoms of bulbar paralysis have developed, and a fatal result followed. In many the general symptoms subside 'Lancet, Feb. 3, 1912. Patient suffering from chronic ophthalmoplegia externa. The wrinkling of the forehead in the effort to open the eyes is noticeable. The external strabis- mus can be seen. 648 THE CBANIAL NEBFES AND THEIE DISEASES. in the course of two weeks, and the only permanent symptoms are the irregular ocular paralyses. In some cases the symptom is the first one of a beginning myasthenia gravis. (See page 769.) The aifection may be arrested in its progress, but usually leaves a chronic condition of paralysis. Chronic ophthalmoplegia externa may be the terminal result of an acute attack, or it may be a primary condition. In a number of cases it has been a congenital condition. If primary it begins gradually and makes slow but steady progress, one muscle after another being affected. It may vary in intensity, some muscles recovering as others are in- volved. Fig. 276 shows the appearance presented by a patient suf- fering from this disease. In this case the disease has remained stationary for six years. It may even go on to recovery, the mus- cles gradually regaining their power. Such recovery occurs in the minority of the cases. In the cases which recover it is probable that the degeneration is only slight in degree, sufficient to arrest the func- tion of the cells for a time. In this condition also a general chronic bulbar paralysis may eventually develop. It is not uncommon to find some defective action of the orbicularis palpebrarum and frontalis muscles supplied by the facial nerve, associated with ptosis ; or some affection of the muscles of mastication. It is rare for bulbar palsy to advance to the oculomotor nerves, though it is not rare for it to develop subsequently to their paralysis. This is probably because a fatal result is reached early in bulbar palsy. As a curious confirmation of the statement that chronic ophthalmoplegia is quite homologous to chronic anterior poliomyelitis, the case of Kalicher may be cited in which, in the course of six months, the paralysis extended from the eyes to the face, and then to the arms and to the legs until total paralysis was present. The lesion in this case was chiefly vascular, the vessel walls being thickened, showing fatty degeneration and unusual dilatations, and hemorrhagic extravasation being found everywhere in the gray matter. Chronic ophthalmoplegia may be the first symptom of tumor in the corpora quadrigemina or of multiple sclerosis. Prognosis. — The prognosis in partial ophthalmoplegia externa is not as unfavorable as might be supposed. Over one-half of the patients recover. This seems to show that in the majority of cases the lesion is a vascular one and does not cause a degeneration of the nuclei. In the total chronic cases the prognosis is bad. Treatment. — The treatment of acute ophthalmoplegia is by the use of ice to the back of the neck, aconite, and phenacetin, and the admini- stration of hot baths daily to produce free sweating. At the same time brisk laxatives and diuretics are to be used, and if the patient is plethoric leeches to the temples or behind the ears are to be applied. The eyes should be kept in perfect rest. In chronic cases it is best to correct diseases of the bloodvessels by the use of heart stimulants, nitroglycerin, and iodide of potassium, and later to stimulate the nerves by the use of strychnine. In cases where syphilis has been a possible cause a mercurial treatment, followed by the free use of iodide, is to be prescribed. THE FIFTH NEBVE: TBIGEMINAL NEBFE. 649 JTHE FIFTH NERVE: TRIGEMINAL NERVE. Anatomy. — The trigeminal nerve is made up of two parts, a motor and a sensory portion, the latter being much larger than the former. The motor part arises from a group of cells lying in the upper part of the pons Varolii in the lateral portion of the formatio reticularis. (Fig. 277.) It passes outward through the formatio reticularis, joins the sensory root, lies under the Gasserian ganglion, and leaves the skull with the inferior maxillary branch. It passes to the muscles of mas- tication, viz., the masse ters, temporals, zygomatic, digastric, and myelo- hyoid muscles, which close the mouth and move the lower jaw. It also sends a filament to the tensor tympani muscle. The sensory part of the nerve develops from the neurones in the Gasserian ganglion. This large ganglion lies upon the nerve at the side of the pons (Fig. 261) upon the base of the skull. It has an extensive distribution upon the face and in the head, as shown in Plate XXV. (p. 622), and all sensations from these parts come in through the nerve. The various areas of the skin of the face connected with the three great divisions are shown in Plate IV. (page 40). Its terminal central axones enter the pons from the ganglion and terminate in a long column of gray matter which is identical in its structure with the substantia gela- tinosa of the spinal cord, and lies in the lateral part of the for- matio reticularis, from the upper part of the pons to the lowest part of the medulla, where it is continuous with the posterior horn of the spinal cord. As these axones enter the pons a few bifurcate, but the majority turn downward, and some pass all the way down to the lowest level of the medulla, lying at the side of the column of gray matter just mentioned. Hence in cross-sections of the pons and medulla this root of the fifth nerve is cut across and appears as a semilunar-shaped white tract. (Fig. 55.) It should be called the descending root, as sensory impulses coming in pass down through it to reach the gray matter. But as this cranial nerve was formerly described as arising in the medulla and pons, like the motor nerves, it was originally named the ascending root of the fifth. The axones end in bushes within the substantia gelatinosa, and thence new sensory fila- ments arise which pass upward to the cortex. These appear to decus- sate in the upper part of the pons, as unilateral lesions of the pons below the entrance of the fifth nerve produce anaesthesia of the face on the side of the lesion only. They then join the general sensory tract in the lemniscus and formatio reticularis and pass to the cortex in the lower third of the sensory motor area. (See page 106.) There is a long semilunar-shaped group of cells which lies on the lateral border of the gray matter lining the aqueduct of Sylvius, in the substance of the tegmentum, and extends for four millimetres beneath the corpora quadrigemina posterior. These cells resemble the cells of the column of Clarke in the spinal cord and are large and flask-shaped. They send axones downward in a long descending nerve root which is semilunar in cross-section and which enters the fifth nerve root oppo- site its exit at the level of the motor nucleus of the nerve. Some 650 THB CBANIAL NERVES AND THEIR DISEASES. authorities ' ascribe motor functions to this group of cells and root, though they were found to be normal in a case where paralysis of the Fig. 277. Scheme of the neurones making up the fifth or trigeminal nerve. (Edinger.) ^ W. A. Turner, Allbutt's System of Medidne, vol. VI., p. 787. THE FIFTH NEBVE: TBIGEMINAL NEEVE. 651 muscles of jtnastication was present and where the motor nucleus of the nerve was degenerated. Other authorities ascribe trophic functions to this group of cells and root, and affirm that they are diseased in cases of hemiatrophy of the face. This statement is made by Mendel ^ on the basis of one case only. Others still regard them as a part of the sensory root. The sense of taste is a part of the function of the fifth nerve, for complete division of this nerve behind the Gasserian ganglion suspends this sense. The nerves through which this sense is conveyed are two : 1. The anterior two-thirds of the tongue sends sensations of taste through the lingual nerves and chorda tympani, which passes into the Fig. 278. The facial nerve and its connections, within the aqueduct of Fallopius. 1, fifth nerve, with the Gasserian ganglion ; 2, ophthalmic division of the fifth nerve ; 3, superior maxillary division of the fifth nerve ; 4, lingual nerve ; 5, sphenopalatine ganglion ; 6, otic ganglion ; 7, submaxillary ganglion ; 8, facial nerve in the aqueduct of Fallopius ; 9, great superficial petrosal nerve ; 10, small superficial petrosal nerve ; 11, stapedius branch of facial nerve ; 12, branch of communication with pneumogastric nerve ; 13, branch of communication with glossopharyngeal nerve ; 14, chorda tympani. (Dalton. ) Fallopian canal in association with the facial nerve, but leaves it at the geniculate ganglion, thence passes by the Vidian or great super- ficial petrosal nerve to Meckel's ganglion (sphenopalatine), and thence to the superior maxillary nerve, and thus into the second trunk of the trigeminal. 2. The posterior third of the tongue sends sensations of taste through the peripheral branches of the glossopharyngeal nerve, thence through the small superficial petrosal nerve or nerve of Jacob- son to the otic ganglion, and thence by the inferior maxillary nerve into the third trunk of the trigeminal. (See Fig. 278.) The protected situation of all these nerves accounts for the rarity of loss of the sense of taste. I have seen it once, however, in a case of severe chronic trigeminal neuralgia and anaesthesia probably due to destructive lesion of the root of the nerve. I have also observed a tf;tal loss of taste on one side of the tongue and pharynx after division of the roots of the nerve and after excision of the Gasserian ganglion done for the relief of neuralgia in several patients. Davies ^ who has 'Nonrol. f:!entralblatt, vol. VII., p. 14. * The Function of the Trigeminal Nerve. Brain, 1907, p. 219. 652 TBE CBANIAL NERVES AND THEIR DISEASE iS. studied the results of resection of the Gasserian ganglion in 107 eases reports an impairment of taste in 27 and a loss of taste in 34 cases. In 46 cases taste was unaflPected. Paralysis of the Fifth or Trigeminal Nerve. A lesion of the motor portion of the trigeminal nerve causes par- alysis of the muscles of chewing. The lower jaw falls open in such cases, and hence both speech and swallowing are interfered with. The patient usually supports the jaw with the hand, and thus assists both acts. It is to be remembered, however, that these muscles act sym- metrically on both sides and together, and hence the destruction of one motor branch of the nerve does not cause very great disability. A little branch from the motor portion passes to the tensor tympani through the otic ganglion, and hence, when the nerve is paralyzed, the ear drum cannot be stretched, and consequently deafness appears. This may be accompanied by tinnitus aurium. When the muscles are paralyzed a gradual atrophy may occur which leads to a sinking of the temple and a thinness of the face, over the muscles which are paralyzed, and at the same time reaction of degeneration may appear in these muscles. Miiller and Schultze both describe a slight difficulty in swallowing due to a paralysis of the muscles about the hard palate as having occurred in two cases. In both these cases there was a slight deviation of the uvula toward the paralyzed side. These symp- toms were referred to a paralysis of the spheno-stapedius muscle, which receives a branch from the motor portion of the trigeminal nerve, k These symptoms of paralysis of the fifth nerve are of some interest, 'as the nerve is occasionally divided by surgeons in the operation for trigeminal neuralgia, and it is chiefly in such cases that the symptoms hitherto described have appeared. It is needless to add that in such cases the motor branch of the trigeminal nerve should be carefully avoided by the surgeon, so that the paralytic symptoms may be prevented. Trismus or tonic spasm of the muscles of mastication is usually an affection associated with general convulsions or is a symptom of the in- fection of tetanus or lockjaw. It is not to be regarded as a local dis- ease of the motor branch of the fifth nerve. Occasionally a spasm of these muscles upon one side of the body occurs in connection with facial spasm, and may be regarded as a local affection quite similar in its origin to blepharospasm and facial tic. Under these circumstances it is usually a reflex manifestation of some sensory disturbance about^ the jaw or throat, and until the origin of such reflex irritation is ascer- tained and removed treatment will not be successful. Tonic spasm of the masseters has been known to occur after long- continued stretching of the muscles, as in a case reported by Peterson, where it developed after a long dental operation. It has been known to occur after a condition of paralysis in the muscles, as in a case re- ported by McConnell. Occasionally a fixation of the jaw and inability to open the mouth occur as a symptom in hysteria. THE FIFTH NEBVE : TRIGEMINAL NEBVE. 653 Clonic spasms of the muscles of mastication occur, causing a chat- tering of the teeth, as in a malarial chill or in an extreme condition of fear, or a grating of the teeth if the spasm is more slow and forcible. It occurs occasionally in paralysis agitans and in old age. Even in health every one has bitten the tongue or bitten the cheek unintention- ally by an unexpected movement of the jaw. The disease is of no moment excepting in children, when grinding of the teeth at night often causes much annoyance to the parents and sometimes disturbs the child's sleep. This may be due to any disease which produces an irri- tation of the nervous system, such as irritation of the intestine by worms, irritation of the genitals by smegma, irritation of the throat by adenoids, or irritation of the ear by wax. Occasionally grinding of the teeth is an early sign of irritation upon the base of the brain by a beginning basilar or tubercular meningitis, and in many cases of this disease the symptom appears at some time in the progress of the case. It has been known to occur as a symptom of tumor of the base of the brain. The spasm is uniformly bilateral. In the vast majority of children's cases, the symptom is outgrown, and it should not cause any alarm excepting in connection with other symptoms of tetanus or of tubercular meningitis. All these forms of facial spasm are to be treated as in facial tic. (See page 761.) Bernhardt has recorded one case of congenital paralysis of the trigeminal nerve which was accompanied by abducens and facial palsy, and this appears to be a unique case in the literature. Injuries of the fifth nerve upon the base of the brain by tumors, syphilitic or tuber- cular exudations in the meninges, or fractures of the base are very rare. When the Gasserian ganglion is involved herpes zoster upon the face may appear. A neuritis of the trigeminal nerve following cold has not been described, yet it is not at all unlikely that very many cases of trifacial neuralgia (see page 729) are really due to this cause. Hemorrhage or softening in the pons Varolii or medulla at any point in the fifth nerve nucleus, or tumors, or patches of sclerosis in the same locality may cause paralysis of the nerve. Thus the symptoms may be present in any form of gross lesion in the cerebral axis. It also ap- pears as a symptom in some cases of bulbar paralysis. In these cases the existence of other symptoms of bulbar disease will enable a diag- nosis of the site of the lesion to be reached. It has also been observed in syringomyelia when the gliomatous degeneration extended into the pons. The symptoms of paralysis of the sensory portion of the trigeminal nerve are numbness and anaesthesia of the face. In Plate XXV., the exit of the three great branches of the sensory portion upon the face are shown, and in Plate IV., their distribution to the skin is shown. The first branch supplies the tear ducts and glands, and any loss of its function is followed by a dryness of the eye. The second branch con- trols the sensation of the mucous membrane of the nose and mouth, and dryness of these parts accompanies this injury. The second and third 654 THE CBANIAL NEBVES AND THEIB DISEASES. branches contain all the fibres concerned in taste, and hence lesion of these parts results in the loss of this sense. The vasomotor and trophic functions of the fifth nerve are important, and trophic symptoms are exceedingly common in cases of any inj ury of the fifth nerve. Whether these are due to the suspension of sensation may be a matter of discus- They consist of ulcerations of the cornea, falling of the teeth. sion. changes in the hard gums and in the mucous membrane of the mouth Fig. 279. Fig. 280. Hemiatrophy of the face. The condition de- veloped slowly during five years, and then came to a standstill at the point shown. Hemiatrophy of the face, developed in two years. Mailhouse. ) The condition had (Kindness of Dr. and nose, with undue dryness and tendency to ulceration and the forma- tion of crusts, and also the development of herpes zoster, which may occur in the nasal and buccal cavities as well as on the skin. The fifth nerve supplies the tongue with sensation as well as with taste, and un- noticed injuries of the tongue in chewing are not uncommon when the fifth nerve is anaesthetic. A loss of the reflexes of the face occurs in paralysis of the fifth nerve, so that winking does not occur when the eye is irritated ; tears do not flow, sneezing cannot be produced by inhalation of irritating substances or by tickling of the nose. The sense of smell is somewhat impaired, partly on account of the dryness of the mucous membrane of the nose, and partly because many irritat- ing substances like ammonia, alcohol, and substances which irritate the mucous membrane are really perceived through the trigeminal nerve. It is possible that the secretion of saliva and swallowing upon the paralyzed side may be suspended. The common form of disease of the fifth nerve is trigeminal neu- ralgia. This has been described on page 729 et seq. It is there shown that such neuralgia is usually due to a lesion of the Gasserian ganglion. Hemiatrophy of the Face. — A gradually advancing atrophy of one-half of the face has been observed. It is a very rare afiectiou. It TEE SEVEN TE NERVE: TEE FACIAL NERVE. 655 is characterized by a very slowly progressive atrophy affecting the skin, the subcutaneous fat, the muscles, and the bones in about equal degree. As a rule, this condition develops in youth ; it has not been observed before the age of ten years, and is rare after the age of twenty years. For some time it may escape notice, but gradually the asymmetry of the face becomes more and more evident, the skin is manifestly thin, and is often slightly pigmented, the cheek sinks in upon the affected side, the temple also sinks, and when the disease is thoroughly devel- oped, as in the case shown in Fig. 280, palpation reveals the decided atrophy of all the tissues. Sometimes the hair on the affected side falls out, but this is not a uniform result. Sensibility is in no way affected and the muscles are not paralyzed, although as they become thin they are weaker than those of the healthy side. After several years the condition appears to come to a standstill, and the disease does not lead to a fatal termination. Occasionally the disease makes rapid progress, as in the patient shown in Fig. 280. In this man the disease had been in progress only two years. He suffered from spasms of the masseters and much pain in the face, but had no anaesthesia and no other symp- toms of intracranial disease. The pathology of this disease is unknown. There is no known treatment. THE SEVENTH NERVE: THE FACIAL NERVE. The facial nerve is the great motor nerve of the face. It arises from a large nucleus which lies deep in the formatio reticularis of the pons Varolii. (Fig. 77.) From this nucleus the fibres pass backward and inward toward the floor of the ventricle, curve about the nucleus of the sixth nerve, forming an angle known as the knee of the facial, and then turn outward, traverse again the formatio reticularis of the pons, and make their exit in the groove between pons and medulla in close prox- imity to the eighth nerve. It then enters the internal auditory foramen of the j)etrous portion of the temporal bone and, after passing through the bony canal called the aqueduct of Fallopius, issues from the stylo- mastoid foramen upon the face just under the lobe of the ear. It there divides into many branches which supply the various muscles of the face. (Plate XXV.) Just before entering the auditory foramen a ganglion is seen to lie on the nerve. This is the geniculate ganglion. As motor nerves do not possess such ganglia, it is evident that at this point some sensory fibres must be associated with the motor fibres. These are partly sensory fibres belonging to the chorda tympani — a sensory nerve of taste which joins the seventh nerve in the Fallopian canal and leaves it at the ganglion to pass by way of the Vidian or superficial petrosal nerve to the superior maxillary branch of the fifth ; and partly sensory nerves of touch supplying the auditory canal and the skin just in front of the ear, as recently shown by J. R, Hunt.' Occasionally, when inflammatory processes attack the seventh nerve ' Ilcqjetic Inflammations of the Geniculate Ganglion, Jour. Nerv. and Ment. Dis., Feb., 1907, alsoAmer, Jour, Med. Sci., Aug., 1908. 656 THE CEANIAL NEEVES AND THEIE DISEASES. in the canal where this sensory nerve lies beside it, its fibres are involved, and then the sense of taste is lost on the anterior two-thirds of the tongue. When the sensory fibres are involved neuralgic pain may be felt just in front of the ear or in the auditory canal or herpes may appear in these places. A small nerve lies at the side of the facial between the ganglion and the pons and enters it between the seventh and eighth nerves. This is the nerve of Wrisberg. It is probably a sensory nerve. It passes toward the glossopharyngeal nucleus, where it ends. It has been thought to be a vestige of the extensive sensory facial branch found in fishes. Fig. 278 shows the various branches of the nerve in its course. The successive invasion of each branch from without inward by a progressive neuritis enables oile to locate the process of a neuritis in the nerve, as will be indicated in the section on diagnosis. The facial nucleus is closely joined to many other cranial nerve nuclei by association fibres. One distinct branch from the oculomotor nucleus joins the facial nucleus and sends some fila- ments directly into the nerve trunk. Thus an anatomical basis for the many reflex and automatic acts in which the muscles of the head and face take part is secured. Thus in the acts of winking, breathing, in motions attendant upon sensations of sight, and sound, and taste, and smell, as well as in the various bodily sensations, changes of the facial expression are constantly seen which are automatic. Mental states are also reflected in the face unconsciously, and this is secured by an intimate association of the facial nuclei and the centres in the optic thalamus. Any disease in the pons, such as hemorrhage, soften- ing, sclerosis, or tumor will suspend these reflex acts. The facial nu- cleus is also directly joined to the cerebral cortex, receiving impulses from the lower third of the motor area by a tract which traverses the knee of the internal capsule and lies in the median part of the motor tract in the crus cerebri. Its fibres leave the motor tract in the pons, pass backward in the raph6, where they partially decussate with those of the opposite side, and end in the nucleus. Thus each hemisphere is joined to both nuclei. Lesions in the course of this tract arrest voluntary movements of the face, but do not suspend the reflex acts. (See Figs. 48 and 49 and page 104.) Facial Paralysis. Pathology. — As facial paralysis usually recovers, but few pathological observations are available. Markowski^ found in a case of facial palsy due to cold that the nerve was normal from the medulla to the geniculate ganglion, but from the ganglion down- ward into the periphery there was a well-marked degeneration. The neuritis was parenchymatous and not interstitial. The peripheral branches contained many fully degenerated fibres, a few remaining globules of myelin in a state of disintegration, and many little fat cells. Some of the branches contained normal as well as degenerated nerve fibres, and very few degenerated fibres were found in the super- ficial petrosal and stapedius nerves. The entire process was a pure degenerative neuritis without any participation of the neurilemma. A, lArch. f, Psjch., Bd, xxiii., p. 367. TEE SEVEN TK NEBVE : THE FACIAL NEBVE. 657 similar lesion was found by Darkschewitch and Tichonow ^ and by G. Alexander.^ Dejerine and Theohari ^ have examined a nerve after facial palsy and found a well-marked degeneration in all the branches of the nerve, though the lower branch was more affected than the others. There was no evidence of interstitial changes. While the root of the facial nerve showed no degeneration, examination of the nucleus by the Nissl stain demonstrated a disappearance of the fine Nissl bodies and a shining appearance such as is found in cells whose function is suspended. This was also seen by Flatau^ in another case. Etiology. — While facial palsy due to maldevelopment in foetal life has been occasionally described, the majority of congenital cases are due to pressure exerted upon the facial nerve during delivery. This pressure may be produced by forceps applied to the head or by a mis- placed hand or shoulder pressing upon the face during labor. Bern- hardt^ has recorded a number of such cases, and every obstetrician has met with them. In some of these cases the orbicularis oris has not been paralyzed. Children occasionally suffer from facial paralysis fol- lowing a blow on the ear or an attack of the mumps. Tubercular swelling of the glands of the neck, causing pressure on the nerve, is an occasional cause of facial palsy in children, but the most common cause in children is otitis media producing an inflammation of the nerve in the Fallopian canal adjacent to the inflamed ear. This also is a cause of facial palsy in the adult in 7 per cent, of the cases. In- tracranial disease, such as tumors of the brain, or exudations upon the base of the brain, or syphilitic inflammation of the nerve trunk within the cranium compressing the nerve, may produce paralysis. Fracture at the base of the skull and caries of the petrous portion of the tem- poral bone, involving the nerve in its course have also produced facial palsy. The usual cause of facial paralysis is supposed to be exposure to cold. Philip ascribed facial palsy to this cause in 72 per cent, of the cases. Remak ® found this a cause in 45 per cent, of his cases, but Thomas ^ describes it as a cause in only one-quarter of his cases. In many of my cases a history of a draught upon the side of the face has been obtained, and it seems probable that this may produce such con- gestion of the nerve as to lead to facial paralysis. The disease does not occur more frequently in winter than in summer, but Bernhardt* found it more common in women than in men and more common in men who were shaven than in those who wore a beard. The Germans call all cases due to exposure to cold " rheumatic," but there appears to be no distinct relation between facial palsy and rheumatism. In very many cases no ostensible cause can be ascertained. The most plausible explanation of the occurrence of facial palsy is that offered by Philip ^ who believes that a congenital narrowing of 'Neurol. Centralbl., 1893, p. 329. 2 Arch. f.Psych., xxxv., p. 72. 3 J.e Somaine Medicale, 1897, p. 453. * Zeitsch. f. klin. Med., 1897. •■■'Berliner klin. Woch., 1899, No. 31. «Eemak, Neurol. Centralbl., 1899. ' Tliomas, .Jonr. Amer. Med. Assoc, 1898, No. 21. ^Dissert., Bonn, 1890. « Berliner klin. Woch., 1888, No. 7, and 1892, No. 30. 42 658 TEE CEANIAL NEEVES AND THEIB DISEASES. the stylomastoid foramen in certain persons predisposes them to a com- pression of the nerve upon any slight congestion of its trunk. It is probable that an unnaturally large nerve lying in a normal foramen might produce some such tendency. A certain number of cases of facial palsy develop during the night, when pressure upon the pillow, combined with venous stasis in the canal, might produce a pressure in such individuals. One attack of facial palsy predisposes to another, Fig. 281. Fig. 282. Facial paralysis of the right side. Attempt to Facial paralysis of the risbt side. Attempt to raise the eyebrows. close the eyes. and in 7 per cent, of the cases there is a recurrence of the disease. Facial paralysis may accompany any form of multiple neuritis, and is then often bilateral. I have seen such cases in adults suffering from alcoholic multiple neuritis, also from severe lead palsy, and several cases in children after diphtheria. Symptoms. — The symptoms of facial paralysis are very noticeable. There is a total paralysis of all tlie muscles upon one side of the face, including the forehead and the muscles which close the eye. The forehead is smooth, its wrinkles are no longer evident, and the eye- brow is immovable. Attempts at frowning are unsuccessful on the paralyzed side, the eye remaining open, and when the patient is told to close it he merely rolls the eyeball upward, leaving the sclera visible. He is not aware, however, that he has not closed the eye. The reflex act of winking is abolished, and the consequent exposure of the eye- ball often leads to conjunctivitis. As the lower lid is not approxi- mated to the eyeball, tears are no longer directed into the tear duct, and may run down upon the cheek. The nasolabial fold of the cheek is flat, the corner of the mouth hangs down, and there is a total lack of the normal play of facial expression during emotion or in conver- sation. The flat, expressionless countenance gives a mask-like appear- ance to the face and attracts attention at once. In the act of respira- tion the ala of the nose is not dilated and the cheek often flaps. All TEE SEVENTH NEBVE : THE FACIAL NEBVE. 659 voluntary motions about the mouth are suspended, hence whistling, blowing/ pursing of the lips, drinking, or moving the food about in the mouth during the act of chewing are imperfectly performed, and the pronunciation of labials is often indistinct. The patient often bites the cheek in the act of chewing, as the buccinator fails to act. Those who can move the ears lose this power. Occasionally, when the mouth is opened, the tongue appears to protrude toward the healthy side. This deviation is often apparent rather than real, the mouth being opened unevenly. If, however, the deviation of the tongue is real, it is due to a paralysis of the muscles attached to the hyoid bone, allowing this to fall upon the paralyzed side. Occasionally in facial palsy a deviation of the uvula has been noticed. One side of the uvula may be elevated, and it is then turned away from the paralyzed side, or it may fall toward the paralyzed side. This symp- tom is a rare one, and I have seen it only occasionally. While mentioned by Erb and others, it has been denied by Horsley and Beevor, who consider it a mere accident and not a symptom of facial palsy. It is quite certain that the motor innervation of the palate comes from the vago-accessorius. Schultze ^ has found such a deviation in but one case in 55. In some cases of facial palsy the sense of taste is affected upon the anterior two-thirds of the tongue upon the paralyzed side. This may be present, however, without attracting the notice of the patient.^ It can be discovered by applying sweet, salty, or bitter solutions to the tongue with a fine camel's-hair brush or by passing a galvanic current through the tongue, when the galvanic taste is no longer produced. This symptom is only present when the lesion in the nerve is a deep one and involves that part of it within the Fallopian canal, where the chorda tympani joins it. An unusual acuteness of hearing and tinnitus aurium ar^ occasionally complained of. They are due to the tension of the drum membrane consequent upon paralysis of the stapedius muscle. Under these circumstances the lesion is very deep in the Fallopian canal or upon the base of the brain. If the paralysis is an accompaniment of middle-ear disease, or if the auditory nerve is affected upon the base of the brain, where it lies in close apposition to the facial nerve, deafness and vertigo will be associated with paralysis. The patients sometimes complain of pain behind the ear at the time of the onset of the affection, and are often tender to pressure at the exit of the nerve. They sometimes have pain in the side of the neck, and occasionally herpes appears below the ear or on the side of the neck. Facial palsy occasionally develops simultaneously on both sides. This is very rare, but I have seen several cases. In one case the left side was affected three days after the right side. The absence of other bulbar symptoms and the recovery in these cases proved them to be peripheral. After the paralysis has been present for some little time stiffness in ' Lehrbuch der Nervenkrankheiten, Bd. i., p. 5. ''Lermoyez, M., Annales des Mai. de F Oreille, 1899, p. 564, 660 THE CBANIAL NERVES AND THEIB DISEASES. the muscles of the face is often felt, but it is rarelj associated with neuralgic pain. If the case does not go on to recovery the facial muscles atrophy and contract, producing a very distressing sense of stiffness in the side of the face. This contracture of the muscles may restore the original lines of the face and the original facial expression, and may even elevate the corner of the mouth slightly, so that at the first glance the paralysis may be thought to be in the well side, but any attempt at voluntary movement will demonstrate the immobility of the truly paralyzed and contractured muscles. The electrical contractility in the facial muscles is usually altered. In some cases the faradic contractility is preserved and the galvanic contractility is slightly increased, so that the muscles respond to a weaker current than those upon the normal side. In these cases recovery occurs within three or four weeks. In cases of medium severity, however, there is a partial reaction of degeneration. The faradic contractility is very much diminished or even lost, the galvanic contractility is heightened, so that only a weak current is necessary to produce contractility, and the cathode closing contraction remains greater than the anode closing contraction. In the course of six weeks the galvanic contraction becomes normal ; after two months the faradic contractility returns, and these patients recover in between two and three months. In severe and permanent cases there is a total reaction of degeneration, a complete loss of faradic contractility, and a gradual reduction in the galvanic contractility, so that very strong currents are necessary to produce any effect, and here the anode closing contrac- tion is greater than the cathode closing contraction, and the opening contractions may be equivalent to the closure contractions. Such a reaction of degeneration may remain for a whole year, and even then recovery may ensue. In fact, Remak records a case of recovery after three years' continued reaction of degeneration. The electrical reac- tions, therefore, are important not only as symptoms but as prognostic indications in this affection. The course of the case varies in different conditions in accordance with the severity of the initial lesion. In the majority of cases all symptoms develop within a few hours of the onset, and remain prac- tically stationary for from two to three weeks, during which time no voluntary movement at all is possible. In the lighter cases the symp- toms then gradually subside, and recovery results in four to six weeks. In more severe cases such recovery does not occur under six months. In the severer type a year may ensue before any relief is evident, and in a few cases no recovery occurs, the condition becoming permanent, the muscles becoming contractured, and very often being the seat of twitching and spasms which are annoying. It is in these severer cases that conjunctivitis, unless prevented by antiseptic alkaline lotions to the eye, occurs as a complication. Diagnosis. — The diagnosis of facial palsy presents no difficulties as the symptoms are very distinct. But facial paralysis may occur not only from a lesion of the nerve trunk, but from a lesion of the brain TEE SEVENTH NEBVE: THE FACIAL NERVE. 661 at any poipt in the motor tract between the cortex and the facial nerve nucleus. Facial paralysis produced by a cortical lesion or by a lesion in the motor tract above the level of the nucleus of the facial nerve is less complete than facial paralysis from lesion in the nerve, and is not attended by complete paralysis of the act of closing the eye. Winking is always possible, and the muscles of the forehead are less commonly involved. In emotional excitement the facial muscles act normally so that the play of expression occurs even though voluntary movement is suspended. In this form also there are no changes in the electrical contractility in the paralyzed muscles. While it is possible that an isolated facial paralysis from cortical or subcortical disease may occur, such paralysis is usually the accompaniment of hemiplegia, and hence is not likely to be mistaken for paralysis due to a lesion in the course of the trunk of the nerve. Facial paralysis due to a lesion of the facial nucleus in the pons Varolii is almost uniformly attended by other symptoms of pons dis- ease, such as (a) a paralysis of the sixth nerve, which lies adjacent to the seventh nerve nucleus ; or (6) an implication of the motor or sen- sory tract passing to the opposite arm and leg, which pass close by the situation of the facial nucleus, thus producing alternating hemiplegia or hemianaesthesia (Fig. 49), or (c) the symptoms of bulbar paralysis ; or (d) the symptoms of muscular dystrophy. Gowers has reported a case of isolated infantile palsy of the face analogous to infantile spinal paralysis. In this case the orbicularis oris escaped. The character- istics of the facial paralysis when the nucleus is aifected are the same as those when the nerve trunk is diseased, but there is no affection of taste. The diagnosis of disease upon the base of the brain between the exit of the facial nerve from the pons and its entrance into the inter- nal auditory foramen can only be made from the presence of other symptoms of intracranial disease such as are due to pressure upon the side of the pons or to implication of other cranial nerves, notably the sixth or the eighth. The diagnosis of the location of the lesion in the course of the nerve within the Fallopian canal is not difficult. If the nerve is affected near the geniculate ganglion or prior to the giving off of the little branch to the stapedius muscle, tinnitus aurium and acuteness of hearing will be noticeable symptoms. They will not be present, however, if the lesion is nearer to the exit of the nerve than the point from which this branch arises. If the nerve is injured in the Fallopian canal between the point at which it is joined by the chorda tympani and the point at which this nerve is given off, a loss of taste in the anterior two-thirds of the tongue will be present. Such a loss of taste does not occur if the lesion is near to the exit of the nerve at the stylomastoid foramen or is in the face after its exit from this bony canal. (See Fig. 278.) Prognosis. — The prognosis in facial paralysis depends upon the cause and the severity of the attack. In lesions of the nucleus within the brain or of the trunk on the base of the brain, and in lesions of the nerve Q62 TBE CHANIAL NESrES AND THEIB DISEASES. following otitis media, recovery is rare. When the cause is cold or is unknown, the majority of patients get well. The severity can be deter- mined with a fair degree of accuracy by the electrical examination, as already described, and this examination often aids the prognosis. Treatment. — If the patient is seen within two or three days of the onset of the disease it is well to apply a small blister over the exit of the nerve beneath the ear. If the cause of the affection can be ascertained and can be removed, especially if it is otitis media, this should be done, but in the majority of cases it is impossible to ascer- tain a cause, and hence the treatment must be symptomatic. It should consist of massage of the face, done by the patient with the tips of his fingers frequently during the day, and this can be aided by placing one finger in the mouth and so compressing and kneading the muscles between the two fingers. The muscles should be treated with elec- tricity daily, that current being used which produces a contraction. But it is not advisable to continue electrical treatment longer than six months, for in the cases which last beyond this time the electrical stimulation favors the development of contractures. When contrac- tures occur in the chronic cases warm applications of water and mas- sage may alleviate the sensation of stiffness. It is useless to apply the faradic current when the muscles do not respond to it. Iodide of potassium in five-grain dose after meals and small doses of strychnine have been thought by some writers to hasten recovery. In congenital cases which show no tendency in the course of two years to recover, and in chronic cases which have remained in a sta- tionary condition for a year it is possible to resort to surgical treat- ment. The facial nerve is cut at its exit from the stylo-mastoid fora- men ; the hypoglossal nerve is exposed behind the internal jugular vein and above the level of the posterior belly of the digastric muscle and the facial nerve is implanted into a longitudinal slit in the hypo- glossal. The junction of the nerves is wrapped in Cargile membrane so that connective tissue will not interfere with the union of the nerves. The result is that nerve fibres grow from the twelfth into the sheath of the seventh eventually producing a regeneration of that nerve and return of control in the muscles of the face. There is usually a coincident paralysis of the tongue which gives little or no inconvenience. Taylor and Pierce Clark have reported some success with this operation.^ THE EIGHTH NERVE: THE AUDITORY NERVE. The eighth or auditory nerve consists of two distinct portions, the cochlear and vestibular nerves, which, though joined in the trunk, are separate from one another both in their peripheral termination and in their central nuclei and also as to function. I. The cochlear part of the nerve is the nerve of hearing. Like all nerves of special sense, it has a special mechanism in the periphery, the organ of Corti, which is a sort of natural harp, its strings vibrating to ^ See Journal of the American Medical Association, March 24, 1906. TKn niGETE NEBVE: THE AUDlTO^Y I^EW^. 663 different tones, each string being in reality an epithelial cell connected with a ner'^e filament whose neurone body lies within the cochlea and sends a central filament into the auditory nerve. The terminations of this nerve in the nuclei of the medulla and their connections with the brain have already been described. (Page 128.) Deafness. — Deafness is usually due to some disease within the ear affecting the peripheral fibres of the nerve. But affections of the acoustic nerve produce disturbances of hearing. Deafness due to disease of the cochlear fibres of the acoustic nerve is distinguished from deafness in the outer or middle ear by the loss of power of perceiving sound through the bones of the head. In a normal condition the sound of the tuning-fork can be heard both when held to the ear and when applied to the teeth, or forehead, or petrous portion of the temporal bone. The sound of the tuning-fork is nor- mally heard longer when the vibrations are conveyed through the air than when they are conveyed through the bone. If there is disease of the middle ear, producing deafness, the tuning-fork is heard through the bone, but not when held near to the ear. If the deafness is due to disease of the auditory nerve the tuning-fork is no longer heard when applied to the bone, or is heard less distinctly than when held outside of the ear. In auditory nerve deafness the higher pitched sounds are less distinctly heard than low notes. This may be tested by the Galton whistle. In auditory nerve deafness hearing is not increased in a noise as it is when disease lies in the middle ear. In auditory nerve deaf- ness inflation of the ear by Politzer's method does not improve the hearing. In auditory nerve deafness changes to the electrical reactions are also present, which are not found in deafness from otitis media. These tests usually produce vertigo, and hence are difficult of appli- cation, but in the normal state the cathode closure produces a slight sound which is distinctly louder than that caused by the anode closure. If the auditory nerve is diseased its reaction is changed. The anode closure sound is greater than the cathode closure sound, and the sound may be heard when the current is broken. In the majority of cases of auditory nerve deafness the vestibular nerve is also affected and vertigo is associated with the deafness. Deafness from acoustic nerve disease or degeneration occurs occa- sionally primarily. It may be a congenital condition due to some defective development either in the nerve or in the organ of Corti, under which circumstances the child is never able to hear and never learns to speak. This is the common cause of deaf-mutism. It is an interesting fact that such deaf-mutes are not made giddy by ordinary methods and do not suffer from seasickness. In other cases an atrophy of the acoustic nerve appears to be due to a congenital defect in its power of nutrition, and deafness comes on in early adult life. This is a peculiarly hereditary affection, many members of the same family being subject to the disease. Thus I have known of one large family in which it has appeared through four generations, each generation 664 TEE CSANIAL NERVES AND TEEIB DISEASES. having had several members who developed the affection. I know another family in which four of seven sons have inherited the deafness of their mother, who had inherited her deafness from her father. They have several uncles and cousins who are deaf. Such cases must be due to a defective vitality in the nerve trunk, and are to be regarded as primary auditory atrophy, the nerve dying before the rest of the body, just as the teeth, hair, and genital organs die before the rest of the body. Syphilis, either hereditary or acquired, may cause acoustic nerve atrophy. Deafness from acoustic atrophy may develop in the course of loco- motor ataxia, of general paresis, and of disseminated sclerosis. I have also seen deafness from auditory atrophy develop in the course of tumors of the brain lying upon the base in such position as to involve the auditory nerve. In syphilis of the base of the brain deafness occasionally develops. Injuries of the head or ear may cause deafness. This is usually attended by vertigo and is due to hemorrhage in the ear. Such hemorrhages may occur spontaneously in the infectious diseases or in states of anaemia. The use of quinine may cause degeneration of the eighth nerve. A functional deafness occurs in hysteria from suspension of activity in fibres of the auditory nerve or in its centres. This form of deafness comes on more sud- denly and is more complete than any other type. The prognosis in deafness from auditory disease is unfavorable, though occasionally counter-irritation over the petrous portion of the temporal bone, and treatment by strychnine, is accompanied by some improvement, but the nerve is not open to any direct treatment, and electrical excitation is of no benefit. Hypodermic injections of pilocarpine, ^ grain, are said to be of some service. Charcot recom- mended quinine, 15 grains a day, to be kept up some weeks, but the majority of aural surgeons are opposed to this form of treatment. Tinnitus Aurium. — Irritation of the auditory nerve in the organ of Corti, or in the middle ear, or in the course of the nerve, may pro- duce subjective sounds which are appreciated as a ringing or roaring in the ears or head, or a buzzing sound supposed to be outside of the ear. This usually indicates disease of the organ of Corti, either of the nature of disturbances in the circulation or of a beginning auditory atrophy, and if it is followed by progressive deafness the diagnosis of auditory atrophy may usually be made. In at least 80 per cent, of the cases of tinnitus aurium some central deafness can be detected by tests with the Galton whistle or the tuning-fork, even when the patient is not aware that he is deaf. Tinnitus aurium may, however, be due to anaemia. Thus, fainting is frequently preceded by a sound in the ear. Gout is a cause of this symptom. A chronic anaemia with re- duction in the number of red corpuscles may cause tinnitus. In these cases the tinnitus is probably due to vibrations of the blood current in the carotid artery, and can be arrested temporarily by pressure upon the carotid artery. Intracranial aneurism causes a sound similar to this. It is because of this blood origin of tinnitus that the symptom TEE EIGHTH NERVE: THE AUDITORY NERVE. 665 develops in states of cachexia and in states of general malnutrition. Direct irritation of the auditory nerve, such as occurs in all forms of otitis media and otitis externa, may produce tinnitus, and anything which interferes with the free circulation of air in the external auditory canal, such as covering the ear with the hand or applying a shell to the ear, will give rise to a perception of unusual sounds. Some drugs, especially quinine and the salicylates, cause tinnitus, and if too long continued, total deafness. In hemorrhagic affections of the ear (Meniere's disease), where the disease lies in the second or vestibular division of the auditory nerve, both tinnitus and deafness are present. A cracking noise due to contraction of the muscles near or about the Eustachian tube is not the same as tinnitus. The treatment of tinnitus aurium is very unsatisfactory unless the cause, such as anaemia, or gout, or malnutrition, or local disease in the ear, can be removed. But when the tinnitus is the first symptom of an auditory atrophy no cure can be expected. Bromides are of some service, as are also belladonna and cannabis indica. II. The second division of the auditory nerve is called the vestibular nerve. It is the nerve of equilibrium. It arises from the bipolar cells of the ganglion of Scarpa, whose dendrites come from the ampullae of the semilunar canals and utricle of the labyrinth. The axones of these cells pass, with the cochlear division of the nerve, to the side of the pons, where they enter directly the lateral portion of the pons (Fig. 83) and terminate about three groups of cells : the cells of Deiters' nucleus, the cells of Bechterew's nucleus, and the cells of the central or posterior nucleus lying upon the floor of the fourth ventricle. The cells of Deiters' and Bechterew's nuclei have numerous con- nections with other parts of the central nervous system, especially with the cerebellum, with the nuclei of the oculomotor nerves (sixth, fourth and third), with various nuclei in the tegmentum, and with the olivary bodies. Some axones of Deiters' cells pass downward through the formatio reticularis to the motor nuclei of the cervical nerves in the spinal cord, and are probably connected either directly or by means of association fibres with the nuclei of the nerves of the head and back. Its connection with the cerebellum is made by fibres which pass by way of the inferior and middle peduncles of the cerebellum to the flocculus and vermis. Each nucleus is also connected by means of direct and indirect neurones with the cerebellum of the opposite side, which it reaches through the corpus trapezoideus and transverse fibres of the pons. The posterior nucleus of the vestibular nerve is connected by direct tracts through the corpus trapezoideus and fillet and through the formatio reticularis with the upper portion of the brain axis, the corpora quadrigemina, corpora geniculata, and optic thalami. It is also connected with the cerebellum by tracts which pass with those from Deiters' nerve. The existence of a tract to the cortex through the internal capsule has not yet been demonstrated, and no cerebral centre or area controlling equilibrium has been discovered. The weight of evidence in favor of the cerebellum as the chief organ 666 T:S^ CMANTA.T' NlSBfES AND THElB DlSJSAftP..^. of equilibrium is overwhelming, and it seems needless to suppose that a cerebral area will be discovered. It is evident, therefore, that the semilunar canals in which impulses are received whose object is to make us aware of our position in space and to direct the whole system of movements by which our equilibrium is preserved, have a most widespread connection with the central nervous system and an espe- cially close connection with the cerebellum. A moment's consideration of the phenomena of equilibrium will convince us of the great importance and complexity of the nervous mechanism controlling it. The body is constantly held in a state of balance, and no movement of any degree can be made in any part without a compensatory movement to preserve the centre of gravity. The slightest variation in our position which would tend to the loss of balance is immediately perceived and immediately corrected. Further- more, the balance is maintained by an interaction of visual sensations, labyrinthine impressions, and muscular sensations, all of which enter into this function, as can be seen by the disturbance of balance oc- casioned by double vision, or by labyrinthine disease, or by a loss of the muscular sense, as in locomotor ataxia. While consciousness takes cognizance of this complex matter of equilibrium, the primary centres which receive the impressions and regulate the activity that they arouse are located in the cerebellum. This is the great organ of equi- librium, and it therefore receives impressions directly from the eyes, from the semilunar canals, and from the muscles of the limbs. The most important impulses in the preservation of equilibrium, however, are derived from the semilunar canals through the vestibular nerve, and aflFections of this nerve are attended by very marked and very intense symptoms of vertigo. Vertigo may be due, as is usually the case, to affections of the terminal organs in the semilunar canals, or to affections of the vestibular portion of the auditory nerve in its course to the cerebellum, or to lesions of the pons, or to disease of the cere- bellum. Disease of the crura not involving the corpora quadrigemina, or of the superior peduncles of the cerebellum, through which the ocu- lar impulses reach the cerebellum, also produces vertigo. Vertigo is a symptom of uncertainty of position, attended by a sensa- tion as if the body itself were being revolved in any one of all possible directions, or as if objects outside of the body were turning rapidly. It causes balancing movements, oscillation of the eyeballs, and stag- gering in the gait. It is attended by great uncertainty and distress of mind, the patient grasping at adjacent objects for support as he feels himself falling, or throwing himself flat upon the floor and clinging to anything in the vicinity for support. A distinction is sometimes made clinically between the sensations of objects outside of one's self turning, called objective vertigo, or of one's body being in a state of revolution, called subjective vertigo. But this distinction of symptoms cannot be referred to any difference in the lesion in any part of the mechanism. Vertigo may be produced by a rapid revolution of the body or by the passage of a galvanic current through the head. It is thought that t:b:b ninth neeve: the glossophabyngeal Nebve. 667 hemorrhages in or destruction of the superior vertical or sagittal semi- lunar canaf (anterior, of Retzius) produce a sense of revolution in the entire body, as if the head were going down and the feet up, the head going toward the side of the lesion. It is thought that hemorrhages in the inferior or horizontal semilunar canal (external, of Retzius) pro- duces a sensation of rotation of the body around its vertical axis toward the side aifected. It is thought that a hemorrhage in the posterior vertical or frontal semilunar canal (posterior, of Retzius) produces a sensation of falling toward the side of the lesion. But, inasmuch as these three canals open into one another, further investigation is neces- sary in order to establish these assertions. Barany has called attention recently ' to the uniform association of nystagmus with labyrinthine disease and has discovered a caloric test of great value. When in a normal person cold or hot water is thrown by a Politzer syringe into the outer ear without exerting pressure, rotary and horizontal nystagmus is produced ; if the labyrinth is de- stroyed this never occurs. When vertigo is caused by labyrinthine disease nystagmus is always present. Vertigo is usually associated with intense vomiting and great men- tal distress. A rapid action of the heart and general relaxation of the bloodvessels, causing profuse sweating and a feeling of great faintness, commonly attend vertigo. It seems probable that anything which causes an intense irritation of the nuclei of the vestibular nerve pro- duces a sympathetic disturbance of the pneumogastric centre which lies adjacent to it. Conversely, we know that irritation coming from the pneumogastric nerve to the brain, such as occurs in gastric disorders or in heart disease, may produce a secondary vertigo. Gastric vertigo is easily differentiated from vertigo due to vestibular disease by the absence of tinnitus and deafness or pain in the ear. Vertigo due to cerebellar affection is also rarely followed by deafness or by pain in the ear. See also page 898. THE NINTH NERVE: THE GLOSSOPHARYNGEAL NERVE. This is a mixed nerve containing both sensory and motor fibres. Its sensory branches come from the tongue, larynx, and respiratory organs and middle ear. It is the nerve of common sensibility of the pharynx and palate and of the middle ear. It is thought that it conveys the sensations of taste from the posterior third of the tongue, those from the anterior two-thirds passing in through the lingual nerve. But as division of the root of the trigeminal nerve has caused a total loss of taste in some patients, there is some doubt as to this function. (See page 651.) The ganglia of origin are the ganglion jugulare on the glossopharyngeal nerve and the two ganglia on the vagus nerve (Plate XXVIII.). The sensory fibres enter the side of the medulla just outside of the upper part of the olivary body, and terminate partly in the gray matter on the floor of the fourth ventricle and partly in a thin column of gray matter like the substantia gelatinosa, lying along the inside of 1 E. E5,rdny. Phys. u. Path, des BoKenpranKapparatus, Deuticke. Wien. 1907. 668 TBE CHANIAL NEBVES AND TEEIB DISEASES. the solitary bundle in the formatio reticularis. The sensory fibres on entering the medulla form a part of this bundle with other sensory fibres of the vagus nerve and turn downward, passing as low as the upper cervical segment of the spinal cord. The solitary bundle is therefore similar in its structure to the descending root of the fifth nerve. It has also been called the respiratory bundle of Krause, as its division suspends respiratory motions. Its fibres end in tufts which lie in the substantia gelatinosa which borders its inner surface. From the cells of this substance new fibres arise which enter the formatio reticularis and the lemniscus, and so ascend to the brain ; but their termination in the cortex has not yet been ascertained. (See Fig. 56, page 116.) The motor branches of the glossopharyngeal nerve arise from a column of cells known as the nucleus ambiguus, which lies in the lateral part of the formatio reticularis. (Fig. 66.) They curve upward, inward, and backward, then outward, forming a knee like that of the facial nerve. They issue from the side of the medulla posterior to the olivary body and pass out of the skull through the jugular foramen. They go to the muscles of the larynx, oesophagus, and pharynx, and preside over the functions of respiration, swallowing, and phonation. (See also Fig. 77, page 148.) The origin of both the motor and sensory fibres of the glosso- pharyngeal nerve are so intimately mingled with those of the vagus nerve that separation between them is impossible. The following table of Spencer shows the probable functions of these two nerves : Table XVIII. — The Distribution of the Ninth, Tenth, and Eleventh Nerves. Afferent. Afferent. Upper roots, ninth nerve. Kespiratory regulating fibres ; Cricothyroid, stylopharyn- respiratory exciting fibres ; geal, oesophagus, pharyngeal (inspiration) inferior laryn- contractors, geal. Middle roots, tenth nerve. Eespiratory inhibitory fibres ; Gastric branches, bronchial (expiration) bronchial. muscles. Afferent. Efferent. Lower roots, tenth nerve. None. Inferior laryngeal, cardiac in- accessory eleventh nerve. hibitory, levator palati. Spinal accessory eleventh None. Sternomastoid, trapezius, nerve, spinal part. THE TENTH NERVE : THE VAGUS OR PNEUMOGASTRIC NERVE. This, like the ninth nerve, contains both sensory and motor fibres. Its sensory branches come from two ganglia on its trunk, of which the upper ganglion is large, oval, and resembles the posterior spinal ganglia, and the other is long and irregular, the cells being scattered among the fibres of the nerve. (Plate XXVIII.) These sensory fibres enter the side of the medulla outside of the olivary bodies and pass in several directions. Some go inward and backward to terminate in a mass of gray matter which lies on the floor of the ventricle laterad of the twelfth nerve nucleus in the medulla and dorsad of it in the upper PLATE XXVIII GLOSSOPHARYNGEAL XI NERVE- PNEUMOGASTRIC lOOR 15 BUNDLES PARS ACCESSORIA 4 OR 5 BUNDLES AURICULAR BRANCH . PARS SPINALIS RIGHT VAGUS BEHIND — LEFT VAGUS TO HEPATIC PLEXUS RIGHT VAGUS TO CCELIAC, SPLENIC AND LEFT RENAL PLEXUSES Distribution of Right Pneumogastric Nerve. (W. Keiller.) TEE TENTH NEEVE : VAGUS OB PNEUMOGASTBIC NEBVE. 669 cord, a mass which projects upon the floor of the tourth ventricle form- ing the ala cinerea. This is the respiratory and cardiac centre. Others join the ninth nerve fibres and enter the solitary bundle, descending in it and ending in the substantia gelatinosa which borders it on its inner side. From these nuclei new fibres arise which enter the for- matio reticularis and ascend to the brain, but their course and termi- nation is yet undetermined. The motor branches of the vagus arise from the cells of the nucleus ambiguus, turn inward, curve about in the formatio reticularis, and then turn outward and make their exit on the side of the medulla, where they form the great trunk of the vagus nerve. The destination of some of these sensory and motor fibres has already been shown in the table, page 668. There are also fibres which pass to the meningeal branches of the fifth, to the auricular branches of the seventh, and to the carotid and abdominal plexuses of the sympathetic system. While it might be supposed from the anatomical distribution that a lesion of the glossopharyngeal nerve would cause anaesthesia and paralysis of the pharynx and larynx and difficulty in swallowing, there are no cases on record of lesion of this nerve trunk. In bulbar paralysis the nuclei of the nerve are affected, and paralysis of the oesophagus and of the pharynx and larynx are the result ; but here, too, it is not possible to distinguish sharply between symptoms due to lesion of the ninth and tenth nerves. The great length of the vagus nerve exposes it to many injuries and to compression by tumors at any point in its course. Acute neuritis of the vagus from cold or rheumatism is not known to occur. Certain poisons, especially that of diphtheria, and alcohol and certain drugs, atropine, veratrum, aconite, and digitalis, etc., appear to have a special selective action upon the nerve and may produce symptoms referable to it. While the distribution of the nerve to the heart, lungs, and viscera is a very wide one, we know but little regarding the symptoms actually produced by its lesion. The nerve is a bilateral one in its action, and in many cases where it has been divided by stab wounds, or in the course of surgical operations upon the neck, very few symp- toms have ensued, though experiments upon animals would indicate that a division of the left nerve should be attended by a very rapid heart action and by a slowing of respiration. This has occasionally occurred when surgeons have included the vagus in tying the left carotid artery. The branches of the nerve which pass to the larynx and supply all the muscles of the larynx are most important, and their injury gives rise uniformly to very marked symptoms. These symptoms have been carefully studied by Gowers, whose table is so complete that it is here reproduced. It will be remembered that the recurrent laryngeal nerve in its long course around the aorta is frequently compressed by aneurisms, and the result of such compression is usually jxiralysis of the vocal cord on one side, with whispering voice and difficulty of respiration. 670 TEE CBANIAL NERVES AND THEIB DISEASES. The abductors of the glottis being paralyzed, the opening of the glottis is not sufficient during the act of respiration, and hence a whistling sound or wheeze and a sense of suffocation results. In a few cases of injury of the vagus in this part of the neck some anaesthesia of the throat back of the palate has been observed, together with disturbance in the act of swallowing. The reflex act of swallowing from irritation of the pharynx is also suspended in these cases. An occasional paralysis of the vagus nerve occurs as a complication of locomotor ataxia, and spasm of the larynx with suffocation may also occur in this disease. Sym.pioms. No voice ; no cough ; stridor on deep inspiration only. Voice low-pitched and hoarse ; no cough ; stridor absent or slight on deep breathing. Voice little changed ; cough normal ; inspiration difficult and long, with loud stridor. Symptoms inconclusive ; little affection of voice or cough. No voice, perfect cough ; no stridor or dyspnoea. Signs. Both cords moderately abducted and motionless. One cord moderately abducted and motionless ; the other normal or even beyond the middle line in phonation. Both cords near together, and during inspiration not sepa- rated but even drawn nearer together. One cord near the middle line, not moving during inspira- tion ; the other normal. Cords normal in position, and moving normally in respira- tion, but not brought together on an attempt at phonation. Lesions. Total bilateral palsy. Total unilateral palsy. Total abductor - palsy. Unilateral abduc- tor palsy. Adductor palsy. The diagnosis of vagus paralysis can be made when the recurrent branches of the nerve are affected and disturbances in respiration and in the voice develop, or when there is a distinct anaesthesia of the pharynx, with difficulty of swallowing, not due to paralysis of the palate or to paralysis of the tongue. Rapidity of the heart is not a sufficient sign to warrant a diagnosis, and there are no lung, gastric, or visceral symptoms that are characteristic of the disease, the majority of the statements made in the books being purely theoretical and not based upon clinical facts or pathological findings. In the course of bulbar paralysis the nuclei of the ninth and tenth nerves become involved and undergo degeneration. This is indicated clinically by the development of respiratory and laryngeal palsy and by a very rapid heart action. These may lead to a fatal result. The occurrence of hemorrhage or of embolism in the medulla involving the vagus nuclei is the cause of sudden death in some cases of apoplexy. The vagus nucleus when disturbed in its function can no longer secure normal breathing or heart action, hence Cheyne-Stokes respiration and intermittent or very rapid heart action are supposed to be symptoms referable to its functional disturbance. Vomiting is also supposed to be due to its irritation. This form of vomiting is sudden, explosive, THE ELEVENTH NERVE: THE SPINAL ACCESSORY NERVE. 671 not preceded by nausea and is very violent, and is attended oy great prostration^ It occurs occasionally in tumors or abscess of the brain in any location and also in meningitis of the base. The vagus nerve is often involved in the course of multiple neuritis due to any of the numerous causes which produce that disease. The signs of its implication are rapid pulse, general vasomotor paralysis, shown by cyanosis, oedema, profuse sweating, and respiratory paralysis, causing irregular, labored breathing. Under these conditions, which are of a very unfavorable prognostic importance, stimulation by alcohol, strychnine, and digitalis, and by nitroglycerin are imperative. (See page 209.) Spasm of the oesophagus is not to be considered as an evidence of affection of the ninth nerve, but is a functional nervous disease usually hysterical in origin or due to organic disease of the oesophagus. THE ELEVENTH NERVE : THE SPINAL ACCESSORY NERVE. Paralysis of the Accessorius. — The eleventh nerve arises from a long column of cells which lie in the lateral part of the anterior horn in the upper five cervical segments of the spinal cord. The roots issue from the lateral surface of the cord and ascend to the brain, where they receive further fibres from the lateral portion of the medulla, the acces- sory portion, which really is independent of the nerve and belongs to the vagus, and then make their exit from the skull through the jugular foramen. It is rarely injured by disease within the spinal canal or skull, though fractures and injuries may affect it. The usual cause of paralysis of the spinal accessory is cold, injury, or pressure by tumors, swollen glands, or abscess in the neck. I have seen the condition in a young girl due to accidental division of the nerve when deep-lying glands were removed from the neck. Symptoms. — The symptoms of paralysis of the spinal accessory nerve are paralysis of the sterno-cleido-mastoid muscle and of the trapezius muscle. The sterno-cleido-mastoid turns the head and elevates the chin, so that when the right muscle contracts the patient looks over his left shoulder. The trapezius turns the head backward and over toward the shoulder and also elevates the shoulder. Other muscles act in asso- ciation with these two muscles, and hence a lesion of the spinal acces- sory nerve causing a paralysis of these muscles is not apparent until the patient is examined ; then it is found that the motions which they ac- complish are not done with the usual amount of power, and a slight amount of resistance renders the patient incapable of turning the head or of elevating the shoulder. The greatest defect lies in the elevation of the arm above the horizontal line ; the trapezius counteracts the action of the serratus, and if it is paralyzed the serratus rotates the scapula in such a manner as to produce a slight elevation upon the side of the neck by the projection upward of its inner angle. When the patient l^rings his arm forward and outward the edge of the shoulder- blade is no longer held tightly to the chest, and hence projects upon the };uck. 672 TEE CBANIAL NEBVES AND THEIE DISEASES. The diagnosis is made by the discovery of the weakness of the mus- cles, of the imperfect elevation of the arm, of the deformity, and of the existence of reaction of degeneration to the electrical examination. The prognosis will depend upon the possibility of removal of the cause, and the treatment is by massage and electricity. In cases which do not recover it may be possible by appropriate apparatus to pro- duce a fixation of the shoulder-blade in its proper position, and thus aid in the movement of the arm. Torticollis: Wryneck. — Congenital lesions of the eleventh nerve due to defects of development or injuries at the time of birth sometimes result in a defective development of the sterno-cleido-mastoid muscle, which fails to grow in proportion to the other muscles, and hence be- comes permanently shortened. In these cases as the child grows the head is slowly turned away from the affected side, the chin approaching the shoulder, and it cannot be turned straight or to the opposite side on account of the permanent shortening of the affected muscle. In such a condition there is no spasmodic contraction of the muscle and no twitching of the head, but the lesion is a wryneck fixed and permanent. This condition is easily remedied by a division of the tendons of the sternomastoid muscle, when the other muscles secure a proper position and proper movement of the head. Spasmodic torticollis is described on page 772. THE TWELFTH NERVE: THE HYPOGLOSSAL NERVE. Anatomy. — The hypoglossal nerve arises from a long group of cells which lies on the floor of the fourth ventricle in its lowermost part, where its walls approach and where it closes into the central canal of the spinal cord. (Fig. 77.) The nerve cells are large and multipolar. They send their axones ventrally through the medulla, passing at the side of the interolivary tract and pyramid and issuing between the pyramid and the olivary body on the ventral surface of the medulla ; thence they make their exit from the skull through the anterior con- dyloid foramen of the atlas and pass to the muscles of the tongue. (See Fig. 52, page 107, and Fig. 78, page 145.) Paralysis of the Hypoglossal Nerve. — The hypoglossal nerve is rarely involved in lesions upon the base of the brain, though occasion- ally tuberculous or syphilitic masses have implicated it. In such cases the vagus nerve, which lies quite near it, is also involved. It is not affected in multiple neuritis. A few cases of injury of the hypoglossal nerve have been recorded where this nerve has been divided by stab wounds or by gunshot wounds in the neck. Occasional attempts to cut the throat result in a division of the hypoglossal nerve. Cameron ^ described such a case with great care. The autopsy showed that the right hypoglossal nerve had been divided just internal to the bifurcation of the carotid artery. The cut ends were retracted, the central end was bulbous, and the distal end was found in a state of advanced degeneration. In this patient the ^British Medical Journal, 1901. TEE TWELFTH NEBVE: THE HYPOGLOSSAL NERVE. 673 Fig. 283. right side of the tongue was paralyzed, had a flabby appearance, and presented 'ihree transverse wrinkles due to the atrophy ; but the tongue could be moved in all directions, though imperfectly, and with diffi- culty toward the left. It protruded to the right side of the mouth in being put out, but there was no paralysis of taste. The roots of the hypoglossal nerve are occasionally affected in dis- ease located within the medulla. They pass close to the pyramidal tract and to the interolivary tract. These convey, respectively, motor and sensory impulses to and from the opposite side of the body ; hence a form of alternate paralysis or alternate anaesthesia may be caused by a lesion which destroys one of these tracts and the hypoglossal root. Such lesions may be of vascular origin or may be new growths. The symptoms of paralysis of the twelfth nerve are inability to move the muscles of the tongue or to protrude this organ from the mouth. The re- sult is that the tongue lies motionless within the mouth, its muscular fibres very often are in a state of fibrillary tremor and the tongue itself becomes atrophied, and the mucous membrane is thrown into folds with deep grooves between them which run lengthwise. (Fig. 283.) Inasmuch as the mus- cles of the tongue are closely interlaced upon the two sides, a unilateral hypo- glossus paralysis does not entirely paralyze this organ. It can be moved in the mouth, and though the pro- nunciation of some of the lingual letters is interfered with, yet unless the paral- ysis is bilateral this is not very notice- able. In the act of protruding the tongue the healthy muscles elevate its posterior portion, pushing it outward more fully on the nonparalyzed side ; hence the tongue deviates toward the side of the paralysis. Chewing and swallowing are not interfered with by unilateral hypo- glossus paralysis, but are seriously impaired if the paralysis is bilateral. It is difficult to examine the tongue electrically on account of its great sensitiveness, but it may be possible to demonstrate a reaction of degeneration in its muscles. Paralysis of the twelfth nerve from disease of its nucleus may be produced as a part of the lesion of bulbar paralysis. ^ (See p. 616.) When the paralysis is due to an affection of the nuclei of the hypoglossal nerve there is usually some paralysis of the orbicularis oris asso- ciated with the paralysis of the tongue as a few of the fibres from that nucleus pass to this muscle. 43 'Deut. Zeitsch. f. Nervenheilk. , Bd. xiii., 4. Eight hemiatrophy of the tongue. (Scripture.) 674 TEE CBANIAL NEEFES AND TUEIB DISEASES. The cortical centres for the movements of the tongue lie in the lower third of the motor area, and are joined to the nucleus by fibres which pass in the motor tract anterior in the capsule and median in the cms to the tracts for the arm and leg. These fibres decussate in the raphe of the medulla. Partial paralysis of the tongue with deviation toward the paralyzed side is usually an accompaniment of hemiplegia, but in this case there is no fibrillary twitching and no atrophy in the tongue. There is no known treatment for this form of atrophic paralysis. A spasmodic contraction of the muscles of the tongue (aphthongia) has been described by Charcot ^ and must have been known in the middle ages, as the best examples are found copied in stone in some of the gargoyles of the Notre Dame in Paris and other cathedrals in Europe. In this aifection the muscles of the tongue are contracted, and as a result the tongue is forcibly thrust out of the mouth, either straight out if the spasm is bilateral (the common form), or toward one side if it is unilateral. The tongue may also be forcibly rolled in the mouth or thrust against the teeth. The spasm occurs, as a rule, in attacks lasting a short time, but extremely annoying to the patient, and is not usually associated with any disease of the tongue, such as ulceration, smoker's tongue, or cancer. It has been regarded as a purely functional, possibly hysterical affection, but like all spasms may be reflex from some peripheral or central irritation. The prognosis is good. The treatment should consist of nerve sedatives, especially bromides, chloral, valerian, and asafcetida. 1 Icon, de la Salp^triere, vol. i., p. 87. SECTION IV. GENEEAl DISEASES OE THE NEEVOUS SYSTEM. CHAPTER XXXVI. MULTIPLE SCLEROSIS. Multiple, or insular, or disseminated sclerosis is a disease which affects both brain and spinal cord together in varying degree, and is characterized by the production of small plaques or islets of sclerotic tissue scattered irregularly through the central nervous system. Pathology. — The sclerotic patches vary from a millimetre to several centimetres in diameter and are of irregular shape. There may be a hundred or more such plaques. They are rarely located in the cortex of the brain, but are found in the white matter beneath it, in the centrum ovale and in the corpus callosum, and in the gray matter of the basal ganglia ; also in the pons, medulla, and spinal Fig. 284. Multiple sclerosis. Sclerotic patches are seen in both anterior, one lateral, and both posterior col- umns. The left anterior horn is reduced in size and is sclerotic. (Larkin.) cord, and with less frequency in the cerebellum both in its white and gray matter. Sometimes the nerves, both cranial and spinal, appear to be invaded for a short distance from their origin. The lesion differs from that occurring in other forms of isclerosis 675 676 MULTIPLE SCLEBOSIS. as it is not limited to definite tracts and as it does not lead to second- ary degeneration in the various tracts in which it may happen to lie. This is because it spares the axones, though involving the medullary sheath of the neurones. In a fresh state the patches are pinkish-gray and more translucent than normal brain tissue. Some have a gelatin- ous appearance. They replace the brain tissue without increasing its volume, hence are not like small tumors. On section each plaque is sharply defined from the normal tissue about it, though the transition from abnormal to normal fibres is less sudden when a single fibre is followed along its course. It may be soft or hard and may in old cases cut like cartilage. There are no long columns of sclerosis, as in tabes or lateral sclerosis, but each islet is limited in extent. On microscopic examination the sclerotic patch is seen to consist of very fine neuroglia fibres and glia cells, forming a feltwork of interlac- FiG. 285. Multiple sclerosis. A sclerotic area implicates a large portion of the medulla oblongata. (Spiller.) ing fibrils with dense infiltration with nuclei, and there may also be an increase of connective tissue. The fibres seem finer than ordinary neuroglia fibres, and are more abundant in the older cases, the early stage being characterized by the presence of small cells. In the older PATHOLOGY. 677 cases larg^ spider cells are often found. There is usually some thick- ening of the bloodvessels. The neurones themselves are singularly free from degeneration, even in the midst of sclerotic patches ; but oc- casionally cell bodies are found in various stages of degeneration when the plaque invades the gray matter, and fatty globules and granular corpuscles, the result of this degeneration, are then scattered through the plaque. Even by Nissl's method of staining there are found few or no evidences of degeneration in the neurones. In some regions a simple atrophy of cells has been seen. The nerve fibres passing through the islet of sclerosis appear to be squeezed, their calibre being reduced but their continuity preserved. The medullary sheath is re- duced materially, and here and there an axone is broken and degen- erated. The result of the degeneration of the myelin is seen in the presence of fatty globules and waxy granules in the nerve patches. But, as a rule, the axones are preserved and lie naked, adjacent to one another, through the plaque, and appear as if the medullary sheath had Fig. 286. Multiple sclerosis. Longitudinal section through a plaque in the spinal cord ; g, thickened neur- oglia ; I, empty spaces in the plaque ; z, large glia cells ; a, axis cylinder. (Schmaus-Sacki.) been dissolved off and absorbed. This peculiar appearance, first noticed by Charcot, has been confirmed by all subsequent observers and is char- acteristic of insular sclerosis. The preservation of the axone explains the absence of secondary degenerations in the various tracts. In a few recently reported cases, probably of very long duration, reliable ob- servers have reported secondary degenerations in both sensory and motor tracts, but those are certainly the exception. The essential feature of the disease is therefore the production of a 678 MULTIPLE SCLEROSIS. peculiar neuroglia structure which is associated with a disappearance of the myelin sheaths of nerve fibres. There are many theories in regard to this process, no one of which is generally accepted. Striimpell believes that it is a congeuitah affection of the nature of gliosis, not unlike syringomyelia {q. v.), and that the nerve fibres are affected secondarily. He urges as proof the fact that it occurs in childhood. Schmaus suggests that instead of a process of degeneration of the myelin sheath there has been a defective development of myelin origi- nally, and that the naked fibres have never developed a myelin cover- ing, a possibility which is rendered more likely because of the fact that in the development of the nerve fibres the myelin appears later than the axone. Under these conditions it would be expected that the symptoms would appear in infancy instead of developing after the age of ten years or later. Rindfleisch and others, Ribbert, Fiirstner, Marie, Bartsch, and Schmaus believe that the process is vascular in origin, a toxic agent ia the blood affecting first the vessels, then the medullary sheath of ETIOLOGY. 679 the nefves, and then leading to a secondary sclerosis. They affirm that the j^laques lie about the vessels, which others deny. Others affirm that a lymph stasis in the lymph spaces about the bloodvessels is the primary cause ; that these spaces are crowded with epithelioid cells which distend them, hinder the flow of lymph, and thus lead to swelling and degeneration of the glia cells and nerve elements, and finally to a sclerosis. But this theory has met with little favor, as the majority of observers regard this lymph stasis as a result and not as a cause, and when, under other conditions, such lymph stasis occurs it leads to a degeneration of the axis cylinder and not to one of the myelin sheath only. Charcot, Erb, Gowers, Leyden, Wernicke, and Thoma ^ hold that the disease is an interstitial or glia inflammation and that the myelin sheaths are destroyed secondarily. Leyden,^ after a critical review, concludes that multiple sclerosis is the termination of a chronic interstitial inflammation. Redlich holds that it is a parenchymatous disease in the myelin, of toxic origin, and that the glia formation is secondary. Andre Tliomas ^ affirms that it begins in the axis cylinder and affects some but not all of the fibrils of which it is made up. Its toxic origin is admitted by all, but the exact manner in which the toxic agent acts and the reason for the limitation of the lesion and the escape of the distal part of the axones is as yet a matter of mystery. Buzzard* who has called attention particularly to the fact that the symptoms of the disease may be intermittent, recoveries and relapses being not uncommon in his experience, refuses to admit any congenital defect and inclines to the toxic theory of the disease. Etiology. — Multiple sclerosis is a disease of youth, the majority of cases developing between the tenth and thirty-first year. No age, how- ever, is exempt. Unger found nineteen cases in infants. The disease is rarely inherited, but two cases being on record in which a woman with the disease had a child who showed symptoms. It has no rela- tion to syphilis. Cold and trauma were formerly supposed to be the active causes, and many cases have been recorded which render this probable, recent cases of Gaupp ^ and Blencke ^ being undoubtedly trau- matic. Of late, owing to the investigations of Marie, more importance has been assigned to infectious and toxic agents, and now it is gener- ally believed that the disease is usually a sequal of some acute infec- tious disease, typhoid, variola, malaria, scarlet fever, measles, whoop- ing-cough, influenza, pneumonia, acute rheumatism, dysentery, cholera, or diphtheria. It has been known to develop in women after child- birth. I have seen such a case, and Balint'^ has reported a case in which after each of four successive confinements a rapid increase in the symptoms of the disease followed. Oppenheim has seen cases due to 'ZurPath. hist, der Mult. Sclerose, Deut. Zeitschr. f. Nervenlieilk. , 1900, Bd. xvii., p. 265. ^Nothnagel's Spec. Path. u. Therap., Bd. x., p. 459. ^Rcv. NouroI(^f,nque, 1900, vol. viii., p. 490. * The Lancet, .Tuly 16, 1904. ■'' Zur Aetiologie der Mult. Sclerose, Centralbl. f. Nervenheilk., June, 1900. «Monatsschr. f. TJnfallsheilk., December, 1900. ■'Deut. Zeitsclir. f. Nervenheilk,, Bd. xvi., p. 437. 6^0 MULTIPLE SCLEttO^m. poisoning by metallic poisons, especially lead, zinc, tin, and mercury, and Etienne ^ has also reported one by carbonic oxide gas. Striimpell, who believes the disease to be of congenital origin and due to mal- development,' ascribes little importance to these causes and holds that they are merely exciting causes when the defective nervous system is present. Hoffman was not able to find any cause in more than one- half of 100 cases studied.^ Symptoms. — It has been already stated that the lesions of multiple sclerosis affect the brain, brain axis, and spinal cord in different degrees in various cases, and therefore it is not surprising that three types of the disease have been described by writers according as symptoms ref- erable to one of these parts predominate ; but since in all cases which continue for a long time the terminal result is about the same, it is only necessary to mention these types as variations in the mode of on- set. It must also be stated at the outset that great irregularity in the development and course of cases has been observed, and therefore Buz- zard is quite right in holding that no typical picture of multiple sclerosis can be drawn. In fact, in many cases it is only by a process of exclu- sion of other forms of cerebral and spinal disease, and of hysteria in particular, that the diagnosis can be reached. It is also necessary to premise any statement of symptoms by calling attention to a fact ad- mitted by all authors that all cases are characterized by occasional complete intermissions in the symptoms, one or many disappearing entirely for months, but returning later. The ordinary type of case, first described by Charcot, is of very gradual development. The patient has some numbness and weakness in the legs, which slowly increases until he has a spastic or ataxic or even cerebellar gait, and stiffness and paralysis in the legs, with in- creased knee-jerks and ankle clonus and Babinski's symptom. There is usually a slight difficulty in the control of the sphincters which ap- pears early. The skin reflexes are often lost. The gait is usually like that of lateral sclerosis, the feet being drawn along the floor and over- lapping and the legs stiff and adducted. It may be a staggering gait like that of a drunken man, with irregular, uncertain steps. Some- times it is a combination of these two gaits. The entire body sways in walking, which is due to an irregular contraction of the muscles of the trunk, producing what Oppenheim has termed " vascillation." At the same time a tremor, or rather a jerky, irregular action of the hands, appears, which is increased by effort both to hold them still and to perform any fine motion. The rate of the tremor is from five to seven per second. This is termed by the Germans an "intentional tremor." It does not occur when the hands are at rest, but gets worse and worse as they are used, the motions being more and more extensive, until finally the patient makes irregular, jerky motions and cannot attain his end. When he ceases to try the tremor stops. Thus he spills all the water from a glass in attempting to drink, and is unable to adjust ^Kev. Neurologique, 1900, vol. viii., p. 825, ^DqxxL Zeitschr. f. Nervenheilk., 1902, Bd. xxi., p. 1. SYMPTOMS. 681 his clothing or to write. The entire arm and forearm share in this tremor, ©ccasionally this tremor is confined to one side. The arms may become weak and stiff, but this is rare. At the same time a marked defect in speech develops, known as scanning speech. It is a slow, jerky utterance of words, with pauses between words or between syllables, and a forced effort to pronounce, which leads to unusual accentuation and possibly to a clipping of words, so that they are not fully uttered. There may be also a tremor of the face and of the tongue, and tremor of the vocal cords has been seen by the laryngoscope. Sometimes there is a tremor or queer, jerky move- ment of the head which can be produced if the patient looks up or turns his head. This may occur, like a senile nodding, when the patient is at rest, the mere act of supporting the head being enough of a voluntary act to start the tremor. Nystagmus on lateral or upward movement of the eyeballs appears early, and later may occur when no motion is made. This is found in 75 per cent, of the cases. • The pupils are often unequal and may be contracted, but always respond to light. Strabismus, due to a weak- ness of one or more ocular muscles, usually the externi, is an occasional symptom. This may only become evident on conjugate movements of the eyes to one side. Temporary attacks of blindness may occur, which are often followed by a permanent condition due to optic atrophy ; in fact, optic atrophy may be, as in tabes, the earliest symptom, as shown by cases of Bruns and Stolling,^ and is found in a large number of cases (52 per cent., Utthoff). It is usually a partial atrophy and causes a narrowing of the visual field or scotoma rather than total blindness. The outer or the inner halves of the disk are pale, or the entire disk may be white and sharply defined. There is also a change in color vision, red and green being lost before blue and yellow, which is the reverse of the order observed in hysteria. It is a peculiarity of the defects of vision in multiple sclerosis that they vary from day to day, at times disappearing entirely. Vertigo is a common symptom, deafness is rare. Mental disturb- ance is frequently observed. There is a sense of well-being and a boastfulness without delusions which suggests paresis. There is a lack of control of the emotions. At times there is involuntary laughing, which is causeless and which annoys the patient, but cannot be con- trolled. There is imperfect memory and a manifest weakening in the power of reasoning and of judgment. In some cases (25 per cent.) attacks of epilepsy or of apoplexy with subsequent aphasia or hemi- plegia occur. Parsesthesia is occasionally complained* of, but anaes- thesia is rarely observed. Pains sometimes give the patients distress. In some cases the spastic paraplegia with atrophy of the muscles, loss of control of the sphincters, ataxia, trophic disturbances in the joints and skin, and bed-sores make it evident that the spinal cord is the part chiefly involved, and in these cases the other symptoms of bul- bar and cerebral nature may be slow in developing, or may not occur before the patient dies. ^Monatsschr. f. Psych, u. Neurologic, 1900, Bd. vii., p. 89. 682 MULTIPLE SCLEUOSIS. In some cases the cerebral symptoms are the first to appear, espe- cially scanning speech and hemiplegic attacks of temporary duration. It is in these patients that the diagnosis may Ije made of paresis or of cerebral softening, and only after months or years, when the bulbar and spinal symptoms develop, is the right diagnosis reached. The fact that other symptoms of paresis do not aj^pear within a year of the onset and that the physical signs of paresis are absent may aid in the differentiation between the two diseases. In a third type of cases the bulbar symptoms precede all others, and difficulty of swallowing, paralysis of the vocal cords, atrophy of the tongue, polyuria, and glycosuria, and attacks of suffocation may occur. The course of the disease varies greatly. In some cases there is a slow but continuous progress in the symptoms, and after some years the patient becomes helpless, and finally dies of some intercurrent dis- ease. In these cases the typical symptoms are so evident that the diagnosis is simple. In other cases there is a slow onset of the spinal symptoms which increase in intensity, and there are sudden attacks of an epileptic or apoplectic type, or sudden attacks of blindness which may subside. In still other cases there are remissions in all the symp- toms which may be so complete as to lead to the hope of recovery, and recurrence takes place only after some months or even years. Buzzard ^ who has recently called attention to the frequency of an intermittent course of the symptoms reports a case in which the symptoms appeared five times in the same patient, each time following a pregnancy and each time subsiding entirely after several months' duration. In other cases the onset is rapid, many symptoms appearing in the course of a few weeks, subsiding and recurring in a series of attacks. And lastly, there are cases which are said to go on to complete recovery by gradual subsidence of all the symptoms. Patients have died within two years of the onset. Others have lived for twenty years or more. It is thus evident that the multiplicity of the symptoms and their very irregular course makes any attempt to draw a picture of multiple sclerosis quite hazardous. It is also evident that a diagnosis can only be reached in the obscure cases by careful and long observation and by an attempt to eliminate all other organic and also some functional diseases. The interest which unusual cases of disease excites has led to the report of many cases of multiple sclerosis in literature. As a fact, it is a very rare afiection. In my clinic but 109 cases have been seen among 31,502 patients, and in my private practice the ratio is about the same.^ Spiller has confirmed its rarity in this country from a study of autopsy records in Philadelphia as well as from his large clinical observation. It is as common as tabes in Germany and in England. Hence too great importance should not be attached to the space occupied by the disease in foreign literature. In one patient, a male aged thirty-five years, no cause could be as- certained. The first symptom noticed was a staggering gait quite like that of a drunken man, which was not increased by closing the eyes iThe Lancet, July 16, 1904. 2 JellifFe, Journal of Nervous and Mental Disease, July, 1904. SYMPTOMS. e^a and was not accompanied by any change in the knee-jerks. After this had been present for some months the man's friends noticed a change in his speech which soon became typically scanning in character. Then it was noticed that there was a change in his manner, so that he gave the impression of being weak-minded and silly, laughed at everything which was said to him, and did not appear to appreciate his condition. Examination showed lateral nystagmus but no optic atrophy or ocular palsy and no actual paralysis. These symptoms had been coming on for a year at the time I saw him, and had not been intermittent, and were not affected by treatment. In a second patient, male, aged twenty -four years, no cause could be found. The first symptom was vertigo, which was increased by walking, and caused an irregular ataxic gait. Soon after a tremor began in the left hand which soon extended to the left leg and increased his ataxic gait, so that by the end of six months he staggered constantly and could hardly dress himself. This tremor was increased by any mental effort, by emotion, or by any active movement. Ten months after the onset he noticed a disturbance of vision which was not due to optic atrophy, but was produced by a lateral nystagmus, which was constant in looking at any object or on turning his eyes. About the same time typical scanning speech was noticed by his wife. This patient had no sensory disturbance, no paralysis, and no mental symp- toms, but all his symptoms were rapidly increasing in intensity. A third patient, a woman aged twenty-five years, had suffered for three years when first seen. Her symptoms began with tremor, first noticed in her head and neck, then in her hands, and finally in her body and legs, which was followed by the slow development of a spastic gait attended by increased knee-jerks. During the third year lateral nystagmus and scanning speech appeared. Her pupils and op- tic nerves were normal, and she had no mental symptoms, and her condition had remained stationary for some months when last seen. A fourth patient, a woman aged thirty years, had suffered for four years. She had always been delicate, though she came of strong stock and had no illness prior to this one. Her symptoms began with stiff- ness and weakness of the legs, which in six months had developed into a spastic paraplegia with extreme contractures and loss of control of the sphincters. Then severe vomiting and indigestion began, which weakened her greatly, and for some time she had to be nourished by enemata. Then for three months she lay in a state of semiconscious- ness, being fed by rectum, her bowels and bladder being evacuated artificially. Of this period she had no recollection, and it was with difficulty that she was kept alive. This condition and the paralysis were supposed to be hysterical, and an attempt was made by orthopedic apparatus to extend the contractured legs and to get her to walk. During this treatment, which lasted six months, her hands began to tremble, her speech l)ecame scanning, her eyesight became poor, and she saw double. Attempts to stand caused great shaking of the entire body and head, but became more and more successful, so that she was able to walk with the snj)|)ort of two persons, and recover/sd control 6S4 MULTIPLE SCLEROSIS. of her sphincters. After three months of a fair degree of comfort her memory began to fail and her control over her emotions was much weakened. Then the intentional tremor increased rapidly, so that no voluntary movement was possible. There was no paralysis of the limbs when I saw her. There was a loss of knee-jerks, nystagmus, paralysis of the left external rectus, no loss of pupil reflex, vision was perfect, there was marked tremor of the intention type, and typical scanning speech. Her manner was childish and hysterical. Diagnosis. — The diagnosis from locomotor ataxia, spastic para- plegia ataxic paraplegia, and amyotrophic lateral sclerosis is to be made (1) by the appearance of bulbar symptoms and cerebral symp- toms, which do not occur in these diseases — e. g., scanning speech, nystagmus, and lack of mental activity, and an emotional state ; (2) by the lack of many symptoms which are characteristic of these diseases ; (3) by the course of the case with remissions which do not occur in these affections. The diagnosis from cerebral disease, from paresis, multiple softening due to atheroma and brain tumor is to be made by the presence of intentional tremor and a scanning speech which does not resemble the trembling, indistinct pronunciation of the paretic ; the absence of severe dementia ; the absence of delusions ; the absence of headache, vomiting, and choked disks ; the preserva- tion of pupil action ; the intermittence of the symptoms, and the irregular course of the case. Toxic tremors and paralysis agitans never resemble intentional tremor and lack all other symptoms of multiple sclerosis. The diagnosis from hysteria may be at all times difficult, especially as hysterical manifestations frequently occur in the course of multiple sclerosis. Both diseases occur in young persons, though hysteria is more common in women and multiple sclerosis in men. Both diseases may follow an emotional shock, or a trauma, or an infectious disease. Both present a multitude of incongruous symp- toms which come and go suddenly without apparent reason, and manifest great changes in character and course. Scanning speech and intentional tremor have been seen in hysteria. Nystagmus is never an hysterical symptom ; optic atrophy is never hysterical ; the peculiar oscillation of the head and body is not seen in hysteria, and loss of control of the sphincters is never hysterical. In the majority of cases a study of the individual, her heredity, nature, character, and her surroundings, and the discovery of the stigmata of hysteria will enable a diagnosis to be reached, though Buzzard, Oppenheim, and every specialist has been obliged to confess to occasional mistakes in diagnosis which were apparently inevitable. The knowledge of the possibility of such mistakes should intensify the effort to obtain objective proof of the existence of multiple sclerosis. So long as subjective symp- toms only are present or symptoms which can be voluntarily simulated no one can be certain of a diagnosis. There is a condition described by Westphal as " pseudo-sclerosis " which presents symptoms almost identical with multiple sclerosis, yet in which no lesions have been found. In this condition the mental symptoms, delirium and an apathetic state going on to moderate TREATMENT. 685 dementia appear early and are more marked than in multiple sclerosis ; the tremor is a slow one, the movements being but two or three to the second, and may occur during rest ; all movements, even speech, are unusually deliberate ; nystagmus and optic atrophy do not develop. Marie considers this disease of Westphal as a form of hysteria, but Striimpell, who has studied two cases, supports Westphal's contention that it is a neurosis, not hysterical in origin. It is not improbable that new histological methods may reveal a lesion as yet not discovered and as widespread as is multiple sclerosis. The diagnosis of multiple sclerosis from Marie's form of hereditary cerebellar ataxia requires consideration, since both diseases develop in youth, both are attended by ataxia, nystagmus and tremor of the head, body, and limbs, and by mental failure. In multiple sclerosis some spastic paraplegia usually precedes the ataxia; this is not often of cerebellar type, and is attended by exaggerated reflexes. In multiple sclerosis optic atrophy is common, but it has not been observed in Marie's disease. The course of the disease is steadily progressive in Marie's disease, while in multiple sclerosis intermissions and remissions are the rule. The diagnosis of multiple sclerosis from cerebro-spinal syphilis is discussed in the next chapter on page 696. Prognosis. — The prognosis in multiple sclerosis is unfavorable for recovery, but the condition does not make rapid progress, and as remissions in the symptoms occur spontaneously some relief from time to time may be promised. In a few cases the symptoms have subsided and have not returned for several years. The cases which have been reported as cured belong to this form. The earlier its development in life the better is the chance of its arrest. Even blindness has been known to subside, and, as the optic atrophy is never total, some degree of vision may be assured. The appearance of bulbar symp- toms should always give rise to apprehension, as some cases of rapidly fatal termination from paralysis of deglutition or from respiratory failure have been recorded. Treatment. — It is essential that patients should avoid physical exertion, and a rest cure often helps greatly to arrest the disease. Even voluntary movements of the hands and too much talking may be avoided. The nutrition must be kept at the best possible point, fresh air, good food, tepid or alcohol sponge baths, massage, and all kinds of tonics being employed to increase the store of energy. As a rule, very hot baths are to be avoided, also cold shocks. A daily bath at 90° F., followed by rubbing, and salt baths at 95° F. are of much service. These patients do well at Nauheim and in water-cure establishments. General galvanization has been thought to be of use. In regard to the use of drugs, I have seen apparent benefit follow the use of arsenic, quinine, and salicin, and would urge these remedies, which may be used in succession two weeks at a time. Cod-liver oil is also of service. There is little effect to be expected from the use of mercury or of iodide of potassium. Nitrate of silver, once highly praised, has been abandoned. CHAPTER XXXyil. SYPHILIS OF THE NERVOUS SYSTEM. While it must be admitted that syphilis is the cause of many forms of nervous disease ; neuritis, cerebral and spinal affections, and func- tional disturbances, it is also to be born6 in mind that there is nothing pathognomonic about syphilitic nervous diseases. If therefore one at- tempted to describe such diseases as a separate entity, one would be obliged to restate many facts elsewhere presented in this work, view- ing them therefore from one side only. In the consideration of all the organic diseases of the nervous system already studied attention has been directed in the sections on Etiology to syphilis as a cause of the disease in question. And in the section upon symptoms the variations in these symptoms due to the syphilitic element of the disease has been emphasized. The question of the treatment of the syphilitic element has also been fidly discussed in connection with each disease. On the other hand, there are many interesting facts in con- nection with the syphilitic element in these diseases which need to be mentioned. In this chapter some of these facts are considered, refer- ence however being constantly made to other sections of this book in which the symptomatology and diagnosis of the different diseases are more fully set forth. Nervous manifestations develop in about ten per cent, of cases of syphilis, as shown by the study of statistics from large clinics and hospitals. These manifestations may appear within a few months of the infection or they may not develop until many years have elapsed. In some cases the nervous disease is a result of inherited syphilis. It has been noticed by many authorities that nervous symptoms appear more commonly in those who have suffered least at the onset, and a mild attack of syphilis, or a thorough course of treatment in the first and second stages, does not exempt the victim from serious nervous diseases, as a tertiary manifestation. In fact in some cases these nervous diseases develop even during a course of antisyphilitic treatment. Pathology. — The pathological changes which are found in syphilis of the nervous system are of three kinds : I. Syphilitic endarteritis with consequent malnutrition and later with thrombosis and consequent softening ; II. Syphilitic exudations about the bloodvessels in the meninges with the production of gummy tumors, often multiple ; and III. Syphilitic chronic hyperplastic inflammation with cellular infil-. tration of the membranes and of the brain, spinal cord or nerves, 686 PATHOLOGY. 687 These three conditions are very frequently combined. In fact it is the excepti(/n to find at an autopsy one of these alone, and hence it is rarely possible to draw a conclusion as to the pathological condition present from a study of the symptoms. In the majority of cases which are examined after death an extensive endarteritis is present, infiltra- tions and exudations about the vessels and in the nervous substance are found and gummata exist. In addition to these active manifestations of syphilis in the nervous system characterized by objective lesions, we also have to admit the existence of a number of postsyphilitic or parasyphilitic diseases of a most serious type, without any characteristic pathology. These are Fig. 288. Sections through a group of cerebral arteries showing endarteritis syphilitica in various stages. Thrombi are seen in two of the vessels. paresis, tabes, and spastic paraplegia, all of which can in the large majority of instances be ascribed to the disease. They are due to de- generative processes in the neurons, whether as the result of the direct action of the microorganism, or of the toxin, or of the antibodies, or of some byproducts of the micro5rganism, or of some antitoxin produced in the body in its effort to destroy the spirochseta pallida we cannot yet affirm. The determination of the syphilitic element as a factor in any of these various forms of nervous disease can now be made by the dis- covery of lymphocytes or of an increase of globulin^ in the cerebro-spinal 688 SYPHILIS OF THE NEBVOUS SYSTEM. fluid obtained by lumbar puncture, and also by the Wassermann test of the blood. And as therapeutic measures must be guided by this determiuation, as well as the prognosis modified in any one case, the importance of these tests cannot be overestimated. Noguchi has made an interesting comparative study of the Wasser- mann reaction, the butyric acid test and the cytological formula as applied to the cerebro-spinal fluid in various forms of syphilis of the nervous system. Wassennann Butyric Cytological Reaction. Acid Eeaction. Formula. Hereditary syphilis 80% 90% . . . Cerebro-spinal syphilis 50% 100% ... General paresis 73% 90% 91% Tabes 52% 100% 100% Fig. 289. Vein in pia mater with swollen and infiltrated wall. Eosin-methylene-blue stain. Magnification 175. (Barrett.) In tuberculous cases search must be made for the bacillus and the Wasserraann test applied to both the spinal fluid and the blood serum. These are, in brief, the means by which we make our diagnosis in syphilis. I. Syphilitic Endarteritis and its Results on the Nervous System. — Syphilitic endarteritis produces a progressive diminution in the calibre of the bloodvessels, both in the arteries, as Huebner has shown, and in the veins, as Reeder has shown. This has already been described on page 469. The decreased supply of blood consequent upon the ob- struction to its flow causes a state of malnutrition of the part of the nervous system supplied by the vessels affected, and eventually leads PATEOLOGT. 689 to thrombosis in those vessels, followed by a localized softening in the area cut off* from its blood supply. The pathology of a syphilitic soft- ening differs in no way from that produced by embolism or thrombosis of non-syphilitic origin (see page 476 and Figs. 221 to 223). The symptoms of this condition are, in the early stage, those of imperfect function of the brain or of the spinal cord. The patients present at first symptoms of neurasthenia, viz., headache, vertigo, in- somnia, inability to work or to concentrate the attention, defects of memory and irritability of temper. Later they have slight temporary suspensions of function, such as partial aphasia, numbness in one limb or in one half of the body, double vision, or staggering gait. Stimu- lants to the heart relieve these symptoms temporarily, but after a series of such prodromata an apoplectic attack occurs. The various types of apoplectic attack have been already described on page 483. An hemi- plegic or aphasic attack in a person between twenty and thirty-five is usually due to syphilitic endarteritis with thrombosis. It may be less severe than an ordinary attack of apoplexy and there is usually no loss of consciousness. It is not infrequently attended by a general convul- sion. Sometimes a state of somnolence may last for several days and the mind be much disturbed and bewildered. The subsequent course of the disease resembles that in an ordinary apoplexy, and the fact that the origin of the attack is a specific endarteritis does not improve the prog- nosis. Antisyphilitic treatment will not benefit the condition, although it may be indicated to prevent other attacks. There are other cases which do not present any apoplectic attacks, but which show all the evidences of small localized areas of softening in the brain already described in the section on arterial sclerosis (page 468). A condition quite similar to that seen in senile dementia (page 470) may develop in these cases, with a chronic course. If the endarteritis affects the spinal bloodvessels there occurs a pro- dromal period of indefinite spinal pain, sensations of numbness in the legs and about the trunk, occasional difficulty in the control of the sphincters, and a delay or loss of the sensation of pain in the legs. Then a sudden attack of paraplegia ensues with all the symptoms of an acute myelitis (see page 403}. The course of such a case is slow and the prospect of recovery is bad. Here again antisyphilitic treatment has no effect in restoring the function of degenerated nervous tissues. For more complete details of the effects of syphilitic endarteritis, the reader is referred to the chapter on vascular diseases of the brain (page 468) and upon myelomalacia (page 400). II. Syphilitic Exudations. — Syphilitic exudations about the blood- vessels and in the meninges of the brain and spinal cord are very common as a sequel of syphilitic disease, especially as an early mani- festation, cases having been observed within six months of the initial lesion. They may occur as a diffuse glue-like mass over a large area of the surface or base of the brain or cord as shown in Figs. 290 and 291, or they may be more distinctly encapsulated, forming a definite gummy tumor, and such gummy tumors are often multiple. The glue- 44 690 SYPHILIS OF THE NEBVOUS SYSTEM. like substance is deposited rapidly and extensively through the meninges and produces pressure upon the subjacent brain, or cranial nerves, or spinal cord and nerves, and upon the bloodvessels. It therefore causes a suspension of function both of the tissues compressed directly and of regions at a distance when their blood supply is cut off. The usual history of a case of brain syphilis is as follows : After a period of several weeks of indefinite nervousness with inability to work, Fig. 290. Multiple gummata upon the base of the brain and about the cerebellum. (Siemerling ) discomfort and restlessness in the late afternoon, with dull headache worse at night, and sleeplessness, the patient is suddenly seized with very intense headache, is dizzy and sees double. He may have numb- ness in one side of the body and a feeling of incapacity for all thought or eflPort. On examination a third nerve palsy may be discovered, or a beginning optic neuritis and possibly a slight hemiplegia or hemi- ansesthesia. An epileptiform attack occasionally precedes the onset or PATHOLOGY. 691 may occur after other symptoms have appeared. The condition if promptly treated soon begins to improve, the ptosis and strabismus subside, and in four or six weeks the patient has no objective signs of the attack and is free from symptoms. If it is not treated the symptoms of brain tumor upon the base ensue. This is the usual history of an exudation of gummy nature upon the base of the brain about the crura, its point of most common occurrence. The frequency of oculo-motor palsy as an evidence of cerebral syphilis has been noted by all writers. Graefe stated that more than a half of all oculo-motor palsies are of syphilitic nature. The nerves supplying the eyeballs have a long course upon the base of the brain and any exudation there is liable to affect them. Irregularity of the pupils, a loss of reaction to light, ptosis, strabismus from partial paralysis of single eye muscles, or all the symptoms of a third nerve or sixth nerve paralysis may develop. In 259 cases of brain syphilis Uhthoff found the oculo-motor nerve affected in 96 cases. Not infre- FiG. 291. •X* t Gumma lying on the surface of the cerebrum. From an exudation on the under surface of the dura. (Larkin.) quently both third nerves are affected. Double sixth nerve palsy is very rare. Such palsies usually subside rapidly under treatment and leave no permanent effects. Or a patient may suffer from gradually increasing localized headaches or diffuse pain over the entire head, which may last for weeks without further manifestation ; or may be followed by a gradually increasing mental dullness going on even to dementia. Changes in the pupils, inequality, or sluggish action or failure to contract to light are often 692 SYPHILIS OF THE NEEFOUS SYSTEM. found, and sometimes an optic neuritis appears. Occasionally epileptic convulsions occur, either localized Jacksonian attacks or general con- vulsions. These may be the only symptoms of a general syphilitic meningitis of the convexity. If to this are added gummy exudations, the local signs of brain tumor may appear. The pressure of syphilitic exudations upon the bloodvessels of the brain adds to the complexity of the symptoms, often causing pseudo- apoplectic attacks. Fig. 292. Syphilitic meningitis of spinal cord. Section from tlie midthoracic region of the spinal cord, show- ing the thickening of the membranes and their adhesion to the cord, a, a, a, a, a, degeneration of the peripheral parts of the cord. (Spiller.) In syphilitic exudations within the Sylvian fissure the pressure may produce a stasis in the Sylvian artery, causing a sudden hemiplegic and aphasic attack and convulsions. If the basilar artery is compressed, very irregular symptoms of bulbar palsy appear and a fatal termination may follow. Yet even in these cases recovery is possible if under brisk treatment the exudation is absorbed and the pressure removed. I have seen one case of total aphasia and hemiplegia recover entirely, although the pressure symptoms remained a week. I have seen a case presenting all the signs of acute bulbar paralysis with a state of coma lasting ten days followed by complete recovery. I have seen a case with all the symptoms of brain tumor attended by unilateral and gen- eral convulsions, reaching the number of eighty attacks in one day which entirely recovered. Such facts make it evident that a brisk antisyphilitic treatment may cure even in desperate cases. PATHOLOGY. 693 The usual history of a case of syphilis of the cord is as follows : After a period of severe backache the patient notices a difficulty in passing water and an occasional leakage of urine ; a loss of sensation to cold, and an inability to distinguish diifereuces of temperature in the legs and body. Soon he feels a girdle sensation and has sharp shooting pains in the limbs. Then his movements become stiif and weak, motions of the spine increase both the backache and the sharp pains, an increase of reflexes is discovered, and some general anaesthesia in the legs, a loss of vibratory sensation or of sensation of cold, and impo- tence. In the course of a week or two partial paraplegia develops, usually with considerable pain in the back, especially in the dorsal region, the arms being exempt. This history indicates the develop- ment of an acute meningo-myelitis with gummy exudations about the dorsal region of the cord, and a cell infiltration about the periphery of the cord, causing degeneration in various columns. At this point by active treatment the progress may be arrested and a progressive im- provement results. Complete recovery may ensue, but is rare, and patients usually remain stiif and weak in their legs and back, and sub- ject to parsesthesise and often unable to control the bladder and rectum completely. Their gait is spastic. Sometimes the lesion is limited to the meninges and does not afiect the cord ; then pain in the back and pain referred to the limbs are prominent symptoms, and rigidity of the spine develops, giving rise to a picture similar to that seen in cervical or lumbar pachymeningitis (see page 450). Many chronic backaches are due to this condition. When the exudation is about the cauda equina the pains are felt in the legs or feet and there may be inability to control the sphincters. A special type of spinal syphilis has been described by Erb. This is probably due to an endarteritis of those spinal vessels which enter the cord around the periphery, and as these vessels are more numerous on the lateral surfaces the eifect of the obstruction of circulation is seen in the degeneration of the fibres and the development of a secondary sclerosis in these lateral columns. In other cases it is a chronic thickening of the meninges about the dorsal region which leads to pressure on the cord and to secondary lateral sclerosis. In still other cases Erb's spastic paraplegia must be ascribed to a primary degenera- tion in the fibres of the lateral columns often combined with some sclerosis in the posterior columns.^ In all these pathological forms the symptoms are the same, viz., a slowly advancing spastic paraplegia (see page 839). In still other cases the exact localization of the spinal symptoms makes it evident that a single gummy tumor is compressing the cord (see page 444). There is nothing particularly characteristic of gumma as distinct from other tumors of the brain or cord ; and from a study of the general and local symptoms it is not possible to determine that the tumor is specific. But in any case of tumor it is wise to give the patient the benefit of the doubt, and if under antisyphilitic treat- ment the symptoms sul^side rapidly, the diagnosis is clear. ' For the pathological bawiH of Erb's cases see Nonne, Deut, Zeitsch. f. Nerven., Oct. 1907. .Also Kenner, Deut. Zeitsch. f. Nerven., June, 1908. 694 SYPHILIS OF THE NERVOUS SYSTEM. III. Syphilitic Hyperplastic Inflammation. — Syphilitic hyperplastic inflammation with an infiltration of the membranes and of the brain or cord by small cells, is the usual accompaniment of exudative processes, but may occur without the production of gumma. It may cause chronic meningitis, or it may produce chronic indurative or sclerotic patches through the nervous tissues. If the meninges are chiefly involved the Fig. 293. --^iS^ ^i-^v.V. '':¥ '■ Area of encephalitis. ^-1, infiltrated pia mater. 5, focus of granulation tissue. C, zones in which glia and non-nervous elements are proliferating. D, layer of epithelioid cells lying along the edge of (£) white substance. Nissl stain. Magnification 15. (Barrett.) symptoms will be those of chronic meningitis (see pages 457 and 726). If the brain is aifected the symptoms will be very similar to those of general paresis (see page 540), for localized patches of encephalitis with degeneration of the neurons may occur (Fig. 293). I have seen a number of cases in which it was impossible to determine whether the patient had a disseminated syphilitic induration of the cortex or paresis, as all the mental and physical symptoms of paresis were present, but in which antisyphilitic treatment was followed by com- plete recovery, now permanent for fifteen years. This fact indicates the propriety of trying such treatment in cases supposedly paretic. If the spots of induration are widely scattered through the brain, cerebral axis and spinal cord, the symptoms will be identical with those of multiple sclerosis. The diagnosis may then be difficult, as Sachs ' and Spiller ^ have indicated. Ocular palsies, optic atrophy, difficulty of 1 Sachs. Phil. Med. Jour., 1898. 2 Spiller. Amer. Jour. Med. Sci., June, 1907. DIAGNOSIS. 695 speech, meutal changes, attacks of an apoplectic nature and a spastic gait with increased reflexes are common to both diseases. The development of an Argyll Robertson pupil, of rotary nystagmus, of a tremor which is distinctly increased by voluntary motions, of speech which is typically scanning rather than merely hesitating, the lack of complete remissions in the course of the case, its steady increase of symptoms and its im- provement under specific treatment are all in favor of a disseminated syphilitic process rather than of multiple sclerosis. In any case where the diagnosis is impossible a test of the spinal fluid or of the blood should be made, as the treatment and prognosis are very different in the two diseases. Occasionally the peripheral nerves are involved, causing symptoms of neuritis. IV. Parasyphilitic Diseases. — The parasyphilitic diseases are evi- dence of degenerative changes in the neurons. It is still uncertain whether they are produced directly by the toxic action of the syphilitic poison, or by the antitoxins produced in the blood as the result of the syphilitic infection, or in some other unknown way. That syphilis is the most important factor in the production of tabes, lateral sclerosis and paresis, is admitted by all, while some go so far as to say that it is the only factor. This question has been discussed in the chapters devoted to these three diseases and requires no further mention. V. Hereditary Syphilis. — Hereditary syphilis manifests itself in some cases by the production of nervous diseases. It may show itself in any or all of the lesions described in this chapter, the infiltration of the tissues and meninges being most common, though a combination of endarteritis and meningitis is sometimes found. ^ It occasionally causes cerebral atrophy in infants with its symptoms (see Chapter XXYIII). Many anomalies of development of the brain and some cases of hydro- cephalus are traceable to hereditary syphilis. Some cases of epilepsy are due to it and in every such case developing in childhood this factor should be eliminated. In one of my cases of epilepsy in which all such inheritance was denied, the appearance of Hutchinson ian teeth gave a clue and the epilepsy gradually ceased under specific treatment when bromides had failed. Cases of juvenile paresis, of juvenile tabes, and of juvenile spastic paraplegia have all been traced to inherited syphilis. The Wassermann test will in any case determine the exist- ence of hereditary syphilis. Diagnosis. — In any doubtful case, where the patient denies syphilis but where the combination of symptoms leads to the suspicion of its existence, it is the duty of the physician to have the Wassermann test of the blood made. The Wassermann reaction, briefly outlined, is as follows: Two tubes are prepared with the following contents. Tube 1. Extract of the liver and spleen of a syphilitic fetus, representing the syphilitic organism (or antigen), plus serum to be tested. Tube 2. Red blood cells of sheep or other animal, suspended in normal saline solution, plus serum of a rabbit which had been immunized to the par- ticular red blood cells used. Complement (a sul)stance necessary to ' Rfinke. Ubor Uoliirnveranderungen beiangeborenen Syphilis. Neurol. Centralbl., Nov., 1«J08. - 696 SYPHILIS OF THE NERVOUS SYSTEM. all immune reactions, which exists in tlie fresh serum of all animals), in the shape of fresh normal guinea-pig serum, is now added to Tube 1 and allowed to stand. At the end of a half hour the contents of Tube 1 are added to that of Tube 2. If the serum in Tube 1 has come from a syphilitic individual, its relation to the extract of antigen is a specific one, and complement will be absorbed thereby so that, when the contents of Tubes 1 and 2 are joined, no hemolysis of the red blood cells in Tube 2 will take place. If the serum is not from a syphilitic, complement will not be absorbed, but will remain active and cause hemolysis in Tube 2. Hemolysis is observed in the test tube as a tingeing of the whole field with a transparent red color. The red corpuscles have been disintegrated and the hemoglobin liberated. In the absence of hemolysis the solution of red cells remains turbulent and opaque. The test, of course, mvolves numerous controls and a careful standardizing of the hemolytic serum and of the syphilitic extract. In many cases of neurasthenia and of psychasthenia with marked fears of syphilis it is wise to subject the patient to this test : if his fear is confirmed a clue to treatment is obtained, if his fear has no basis he will be helped by the knowledge. A reaction of the spinal-cord fluid recently discovered by Dr. Noguchi at the Rockefeller Institute is an equally positive proof of syphilis. This is described by him as follows : 0. 1 c.c. of the spinal fluid to be tested is placed in a test tube whose diameter is 1 cm. or less. To this is added 0.5 c.c. of a 10 per cent, solution of butyric acid. The tube is now heated until the fluid is bubbling, and while still hot 0.1 c.c. of a normal (4 per cent.) solution of sodium hydrate is added. In nearly all spinal fluids an opalescence or cloud occurs, but in the spinal fluid of a syphilitic person the cloud soon separates into a definite floccu- lence which is characteristic. The flocculence usually appears in a few moments — rarely requiring more than 20 minutes. The reaction shown by Dr. Noguchi has the advantage of being positive in a quan- tity of 0.1. c.c. or less, and of being a qualitative as well as a quanti- tative test. What the flocculence may mean is not known, but it may consist of some globulin fraction which is found only in syph- ilitic or metasyphilitic disorders of the nervous system. That the re- action is not entirely quantitative is shown by the fact that many fluids from non-paralytic cases give much richer clouds than those from paresis, but in such cases the clouds do not flocculate. Nonne has elaborated further tests for the cerebro-spinal fluid. The reader is referred to his recent work on syphilis and the nervous system for their descri])tion.^ Treatment. — The treatment of syphilis of the nervous system may be by the use of salvarsan or by the use of mercury and the iodides. In all cases included in sections I, II, and III salvarsan should be used. In cases described in sections IV and V it is useless. The effects appear to be better when the remedy is injected into the veins than when it is given into the muscles. At least three injections * M. Nonne, Syphilis und Nervensystem. M. Karger, Berlin, 1911. "TREATMENT. 697 should be made at intervals of two weeks. There does not seem to be any danger of optic neuritis such as was at first feared/ For the details of the use of salvarsan the reader is referred to recent literature.^ If salvarsan cannot be used mercury may be employed. Mercury is to be used by inunction, one drachm of the ointment being rubbed into the skin daily until a slight physiological effect is pro- duced. It is well to mix the officinal blue ointment with an equal part of simple cerate or lanolin, as absorption is then more complete. The inunction may be made upon the back or upon various parts of the body and extremities, a new part being chosen daily. It is better absorbed if the part selected be thoroughly washed with warm water and soap and then with alcohol or ether before the application ; and after the application — which should be thorough and therefore should take half an hour — a tight flannel bandage should be applied over the part rubbed ; thus what is left on the surface is gradually absorbed. It aids materially in the effect if the patient takes a warm bath (98° F.) daily for twenty minutes. The production of salivation is to be avoided. If inunctions cannot be employed the mercury may be given by the stomach, the protiodide, in dose of from 1 grain to ^ grain three times daily, being the best preparation. The amount may be increased gradually until a slight diarrhoea is produced, and then a dose a little less than that which caused the diarrhoea may be kept up for some time. I prefer the French pills of Garnier and Lamoureux, as of uniform strength and obtainable everywhere, and I use from 3 to 12 of these pills daily. Corrosive sublimate may be employed — from -^Q grain to gL grain three times daily — in the same manner. The advantage of the method by inunction is that it does not affect the digestion unfavorably. The same advantage is afforded by hypoder- mic injections of mercury which some prefer. Iodide of potassium is to be given in connection with the mercury in gradually increasing doses, from 15 to 100 grains three times daily. It should be admin- istered, very largely diluted, in an alkaline water after meals, or else in a bitter tincture before meals. It is well to reduce the large dose of the iodide after a month, and then to continue it in 25-grain doses three times daily for two or three months longer. If free elimination of iodide is secured by the use of laxatives, of diuretics (of which water is best), and of warm baths daily, it can be taken for a long period. The use of salvarsan and of mercury in the parasyphilitic diseases is not recommended. They have no effect upon the course of tabes or paresis. In tabes JSTonne and Sachs affirm that the symptoms are occasionally relieved temporarily by salvarsan ; but not to any degree greater than by the use of mercury. I have not seen any benefit resulting from its use. In paresis it does not arrest the disease and frequently excites attacks of violent headache and mental irritability. In the course of the parasyphilitic diseases it is rare to observe anv active syphilitic process and it is only when such a process appears that I would use either salvarsan or mercury. 'See Sachs, Medical Kecord, Feb. 3, 1912. => Amer. Jour. Med. Science, 1910, p. 910 ; 1911, p. 751. CHAPTER XXXVIII. PELLAGRA. Pellagra (pelle, skin, agra, rough) is an acute disease character- ized by gastro-iutestinal, skin, and nervous symptoms, progressive in character, subject to remissions, but of a chronic nature. History. — The disease has been known in Italy for 150 years and has been steadily increasing there, as it is said that over 100,000 cases occur annually. It has been recognized in the United States since 1863, and it is now believed to have been the cause of many deaths in the prisons and camps in the South during our Civil War. Within the past two years it has been increasing rapidly so that last year about 10,000 cases were reported in various parts of the Southern and Western States. Etiology. — It occurs chiefly in the spring and summer. Sixty- five per cent, of the cases are women. While it may occur at any age, the majority of cases occur between the ages of 20 and 40. An alco- holic habit appears to predispose to the disease. It has been generally supposed that the disease occurs in consequence of eating corn or corn-meal, called polenta in Italy, which is musty or contains some mould or bacterium. The reason for this belief is that the disease appears only where corn is grown and used as a chief article of food, but only under conditions where the corn is not free from mould. It ceases when a corn diet is stopped. It does not prevail where corn is not used as a food. The poison producing it is unknown though several forms of bacteria and mould have been isolated from spoiled corn and certain chemical poisons are known to be present in spoiled corn which have been supposed to have been a cause. One author, however, Sambon, believes it to be due to the sting of the sand- fly. He claims that it is more common in moist places near the water and among field laborers who are exposed to the sting of the fly. He believes that the fly conveys a micro-organism to man, just as the mos- quito conveys the malarial organism and that the disease is due to this organism in the blood. After a careful review of these theories Xiles ' inclines to the first hypothesis. Symptoms. — The symptoms of the disease are classified under three divisions, gastric, skin and nervous symptoms. In the majority of instances they appear in the order named, but anyone of the three may usher in the affection. When it is fully developed all are present. The gastric symptoms consist of a loss of appetite, burning pains m the stomach, considerable flatulence, acid eructations and occasional vomiting, and a coated tongue, the edges of which are smooth and 1 Pellagra, G. M. Mies, M.D., W. B. Saunders and Co., 1912. 698 SYMPTOMS. 699 occasionally, sore. The mucous membrane of the mouth is red and the corners of the mouth become ulcerated. There is an extra flow of saliva. There are pains in the abdomen, considerable gas in the in- testines and a watery diarrhoea, attended by a peculiar characteristic odor, and by difficulty in the control of the rectum. The skin symptoms consist of an erythema with considerable swell- ing of the skin, especially upon the hands and feet and on the extensor surface of the arms and legs which appears symmetrically, and is sharply marked from the normal skin by lines of division. This eruption as it goes on leads to a thickening and rough appearance of the skin which becomes dark and even black, is thrown into folds and is very hard. It burns but it does not itch. It extends to the face and to the trunk in patches. When this eruption fades, the skin desquamates, occasionally leaving deep scars, occasionally healing till no trace remains of the eruption. The nervous symptoms are first burning pains in the extremities with formication, tremor of the hands, gradually increasing weakness and difficulty of movement and contracture in the muscles, occasional anaes- thesia in the affected skin, roughening and thickening of the nails, an increase of reflexes, but no change in the electrical reaction of the muscles. These indicate an invol vent of the peripheral nerves. Symp- toms referable to the central nervous system are also present. Headache, general malaise, sense of indefinite distress and vertigo are frequent complaints. Spasms in the legs, arms and back occasionally occur. Disturbance of speech is frequently complained of, not a true aphasia but a thickness of speech and a weakness of the voice. Pupils are usually normal and react to light. There is no strabismus or nystag- mus. The perception through the senses is rather dull but this is prob- ably owing to the mental condition rather than to any defect of the senses. Slight ataxia in walking and swaying with the eyes closed has been occasionally recorded. The mental symptoms are as noticeable as the nervous symptoms. Depression of spirits, irritability of temper and inability to fix the at- tention, unusual antipathies, occasional morbid fears and suicidal im- pulses are complained of in the majority of cases. The memory is im- paired and the power of attention is imperfect. Delusions and halluci- nations occur in about a fifth of the cases. The hallucinations are of sight and hearing. The delusions as a rule are of a depressed nature, of danger and of persecution. The prevailing mental state is one of apprehension, anxiety and depression. There is an undue emotional irritability and the patients are prone to outbursts of temper and to excessive weeping. The facial expression is one of melancholia, but the patients do not reproach themselves as a true melancholic does, and the danger of suicide is much less than in melancholia. Many of these patients drift into the lunatic asylums in the last stage of the disease and their cases are recognized as differing from those of the typical insanities.' ' See B. E. Tucker, Amer. Jour, of Med. Sciences, March 12, 1912. 700 FtiLLAGUA. Course. — The disease begins gradually with symptoms chiefly of malaise, indefinite pains and indigestion which form a prodromal stage lasting several months. This is usually followed by the stage of erythema when the eruption appears and rapidly develops, and in this stage the gastro-intestinal symptoms become more acute. This stage may last for several weeks or months and is followed by the stage of depression with the development of the nervous and mental symptoms which seem to be the culmination of the poison. A fever is not present. When the condition is fully developed it may remain stationary for several months and then under appropriate diet and treatment begin to subside. Almost all the symptoms disappear gradually and the patient may remain well for some time but a recurrence of the symptoms is always sure to occur, usually in the spring or summer time. After two or three periods of remission the condition becomes chronic and the mental change permanent. Prognosis. — About 13 per cent, of the patients die in the first attack of the disease, about 10 per cent, recover permanently after a light attack. The remainder go on to a series of attacks with remis- sions and finally become chronic invalids, many of them drifting into insane asylums. Treatment. — The most important measures to combat the disease are the removal of the patient to a cold climate where he is to take up his permanent residence ; and a change of diet in such a way as to eliminate all preparations of corn. To increase the nutrition in every possible way by carefully regulated diet and by aids to digestion, is part of the duty of the physician. Arsenic and chloride of sodium are to be used freely and persistently. Recently some favorable effects have been noticed from the use of atoxyl given hypodermically every second day in ^ grain dose gradually increased to three grains. Uro- tropin has been used in doses of ten grains four times a day with some success. The irritation of the stomach and intestines can be mitigated by large doses of bismuth and oxalate of cerium. The burning sensa- tions of the skin can be benefited by powders and by oxide of zinc ointment, but the course of the skin disease does not appear to be influenced by local treatment. The nervous symptoms may be miti- gated by the use of bromides and coal tar products. CHAPTER XXXIX. CAISSON DISEASE. Etiology. — Persons who have worked in caissons or in deep mines under high atmospheric pressure, or under the sea in divers' garments under high atmospheric pressure, are occasionally seized with paralysis on coming out into the ordinary air. This paralysis has, therefore, been ascribed to the sudden change of atmospheric pressure, it being supposed that the system accustoms itself to a very much increased pressure — say of four to six atmospheres — without difficulty, but that the sudden removal of this is the cause of the paralysis. Hence, at the present time, where individuals have to work subjected to such pres- FiG. 294. Caisson Disease. Air bubbles in the veins of tlie brain. (Larkin.) sures provision is usually made to remove this pressure very slowly. Thus modern mines and caissons have several intermediate chambers in which the workmen are advised to remain for one hour or more, thus accustoming themselves gradually to the ordinary atmosphere, and if this precaution is observed no ill results follow. It has been found that ordinary healthy men of good habits are very much less liable to the disease than men who indulge in alcohol freely, or who have some form of heart or kidney disease, or who are very fleshy. Workmen gradually become accustomed to endure pressure, and hence there is a greater danger for new hands than for the older workmen, 701 702 CAISSON DISEASE. Pathology. — A remarkable condition of air embolism in the veins and capillaries of the brain and spinal cord has been found as the primary lesion in these cases. The sudden relief from the extra- ordinary atmospheric pressure has the same effect on the blood that it has in champagne when uncorked ; gas is evolved in the fluid. The gas emboli act as other emboli act in obstructing the circulation and producing secondary infarctions and consequent softening. The extent of this softening may be large or small depending upon the size of the Fig. 295. Lesion of the cord in caisson disease. A large cavity is seen tilled with necrotic debris of the cord coagulated by the preservative. (Larkin. ) vessel blocked (Fig. 294). In the majority of cases the emboli are of microscopic size and located in the capillaries, hence the areas of infarction are minute, and as the anastomosis is sufficient to reestab- lish the circulation and nutrition there is a rapid restoration of function and a progressive recovery. In other cases where the infarctions are large and where softening of the brain or cord follow, there is a rap- idly fatal termination or a chronic state of paralysis remains. The lesions in the cord resemble those already described in the section upon myelomalacia and are shown in Fig. 295. There are hemorrhages in the cord diffusely scattered and surrounded by small regions of acute myelitis. There is an infiltration of the cord by small cells and by lymphocytes, and the development of fatty degeneration both in the cells and in the fibres of the cord. Vacuoles form on the cord. Air emboli have been found on the meninges of the cord in cases which were rapidly fatal. In the cases which survive a few days these air emboli are not found, but the infarctions and the degenerative changes of myelomalacia are everywhere seen. Occasionally the lesion in the cord is a transverse one only ; usually it is disseminated widely. Symptoms. — The symptoms of the disease develop very rapidly within a short time after the exit of the individual from the caisson into the ordinary air. These symptoms are very numerous and are mentioned in the order of frequency of their occurrence. Pain is the chief symptom and is usually felt in the back and in the limbs, so that SYMPTOMS. 703 the patient falls to the ground and is unable to move freely on account of the discomfort. This pain apj)ears to be located in the muscles, being a true myalgia. It is usually general but occasionally is limited to the muscles of the back, sometimes to the muscles of a single limb. It is attended by considerable tenderness of the muscles to pressure and any attempt at movement increases the pain. Pain in the joints is also very commonly felt and may give rise to considerable disability. This is next in frequency to pain in the muscles. It may be general, all the joints being affected, but it is usually confined to the knees, the hips, the shoulders, and the elbows. It is not attended by any swelling or redness, or heat, and therefore the disease can be distinguished from ordinary rheumatism. The symptoms next in frequency to these pains are symptoms referable to the ear. These are severe earache, tinnitus aurium, deaf- ness, and intense vertigo, together with the sense of prostration and vomiting which are not infrequent accompaniments of disturbed circu- lation in the ear. Rupture of the drum has been frequently observed and hemorrhages from the ear are not uncommon. The hearing may be temporarily impaired, but usually in the course of a few days these symptoms subside and the patient recovers his hearing completely. Another set of symptoms are those referable to the nervous system. They are both cerebral and spinal. The cerebral symptoms consist of headache, prostration, feelings of faintness, nausea, vomiting and ver- tigo, double vision, sometimes sudden blindness or deafness, and diffi- culties of speech and of breathing. Many cases have been observed in which a monoplegia or hemiplegia has ensued, and many patients have suffered from attacks of aphasia, either motor or sensory. Sometimes the general symptoms accompanying these cerebral symptoms are such as to indicate a great increase of intracranial pressure, the pulse tension rising to 200 or over, the pulse being slow, respirations sterterous or Cheyne-Stokes in character. Several cases that have presented such symptoms have been operated upon, a decompression operation for the relief of intracranial pressure having been done, and in these cases the dura has bulged and on opening the dura it has been evident that an effusion of fluid into the ventricles has produced this great pressure. In the majority of instances such decompressive operations have not been successful • in saving the life of the patient. Yet when such symptoms occur it seems necessary to attempt such relief, and tapping the ventricles is to be attempted if lumbar puncture has failed to relieve the symptoms. Many patients pass into a state of coma after these cerebral symptoms and die, but in some cases a gradual recovery ensues and the patients may recover entirely or may be left with only a slight hemiplegia or monoplegia, or a mild aphasia. The spinal symptoms are as common as the cerebral symptoms. Soon after exit from the caisson the patient complains of severe pains, wliich are usually felt in tlie legs and in the trunk, occasionally in the arms and ba(;k, and th(!se pains persist for many days through the ill- ness. The pains resemble in tlieir intensity those of tabes. They are attended by numbness and tingling or sensations of cold or heat. Soon 704 CAISSON DISEASE. after the onset of pain a feeling of weakness develops in the legs, which goes on rapidly to a condition of paralysis, both legs being totally par- alyzed, with loss of control of the sphincters. Anaesthesia accompanies the paralysis and extends a variable height upon the trunk. In the majority of instances the paralysis does not extend to the arms, but occasionally these also are affected and then the patient is totally par- alyzed and ansesthetic even up to the neck. In one case observed by me all the muscles below the deltoid were completely paralyzed for four days, but the ansesthesia did not extend above the eighth rib. In this case a gradual recovery ensued and after two months the man had entirely recovered. Variations in degree in all these symptoms have been observed. The majority of the patients develop the most com- plete paralysis within a few hours. In some cases the symptoms sub- side as rapidly as they have developed, and within a week the patient is up and about. In other cases, however, many spinal symptoms remain and after a month some of these patients present all the symp- toms of tabes. In other cases the symptoms are those of lateral scler- osis only, and in still other cases the symptoms are those of a general- ized myelitis. In fatal cases the lesions of all these three spinal dis- eases have been found at the autopsy. The prognosis, however, in the cases of spinal-cord affection developing after caisson disease is dis- tinctly better than when such symptoms develop under other circum- stances, and if the patients are promptly treated many recover who, under other circumstances, would have gone on to death or to a state of chronic invalidism.^ The causes of death, as in acute myelitis, are usually cystitis, bed- sores, or pneumonia. Diagnosis. — The diagnosis of the disease rests upon ascertaining whether the patient afflicted has been subjected to high atmospheric pressure and then has come back into the air very suddenly. Treatment. — A knowledge of the cause of this affection should lead employers to warn their workmen against coming immediately from a high to a low atmospheric pressure and should make it incum- bent in all works to have a graduated series of chambers through which the laborers should be made to pass from a higher to a lower pressure. When the symptoms have developed it has been recommended to imme- diately return the patient to the caisson or to place him in a pneumatic cabinet where a high atmospheric pressure can be immediately pro- duced, and then, by a gradual reduction of the pressure, to accustom him to the ordinary atmosphere. Such a pneumatic cabinet should always be accessible in works or mines where laborers are subject to this disease. When this is not feasible it has been recommended by those who have experience in this affection that large doses of ergot, one drachm every hour or every two hours, should be given. Bandag- ing the limbs tightly with an Esmarch bandage, and thus confining the blood to the larger cavities, has been employed with good effect. Later on the treatment should be that for acute myelitis. 'See " Luftdruck Erkrankungen," by E. Heller, W. Mager, and H. von Schrotten. — A. Holder, Vienna, 1900, . CHAPTER XL. PACHYMENINGITIS AND MENINGEAL HEMORRHAGE. Pachymeningitis or inflammation of the dura mater, may be lim- ited to the external or to the internal surface of the dura, or may in- volve both surfaces at once. PACHYMENINGITIS EXTERNA. Pachymeningitis externa occurs as a result of fractures of the skull, or follows any disease of the bones of the cranium, such as caries, especially after ear disease, syphilitic exostoses, tumors invading the bones, and erysipelas of the face. In septic cases it may lead to the formation of an abscess between the dura and the bone. The only symptoms produced are those of the original causative disease, and local pain, which may be increased by pressure and by percussion. In these cases trephining will lead to the evacuation of the pus. It may take the form of a connective-tissue proliferation, causing a thickening which is permanent. In a few cases a firm adhesion between the dura and the cranial bones, with the production of bony deposits and a solidification of the diploe, has been found. This may occur in early life and lead to the development of chronic headache with many neurasthenic and hys- terical symptoms which resist all forms of treatment. PACHYMENINGITIS INTERNA. Etiology. — This disease may result from the same causes as pachy- meningitis externa, especially from trauma, or may be a sequel of it. It is never possible in life to distinguish sharply between the two con- ditions. It may be a sequel of sunstroke. It has been found in many cases of chronic insanity, especially in paretic and senile dementia. It has been ascribed to chronic alcoholism. It has been found in cases of purpura and in persons dying of various infectious and wasting dis- eases ; a causal connection, however, has not been established. It may be due to syphilis. Pathology. — The result of an inflammation of the inner layer of the dura is the production of a thin vascular membrane covering it like a veil. The vessels in this are extremely delicate and rupture easily. As a result a hsematoma forms, flat and thin, but of sufficient size to compress the brain beneath and to cause symptoms. Some pathologists believe that in cases of hsematoma the hemorrhage from 45 705 706 PACHYMENINGITIS AND PACHYMENINGEAL HEMORRHAGE. congested vessels occurs first, then the clot becomes organized and adherent to the dura, and then new vessels form in it. Bevan Lewis believes this to be the invariable case in the chronic insane. The new membrane may be limited in extent or quite large. It may lie on any part of the dura. If the condition goes on for some time several layers of new tissue may form until the dura is several times its ordinary thickness. In such a thickened series of layers flat hsematomata are often found. The age of the various layers can be determined by their degree of hardness and vascularity. In the older, deeper layers there may be thin deposits of bone. In the superficial layers there is occasionally an adhesion to the pia. This is the condition found in chronic alco- holism and in terminal dementia. Symptoms. — The disease may be present for years as a chronic condition, causing dull headaches only or slight difficulty in thinking, with impaired memory, and may not be diagnosticated. It is by the occurrence of the hemorrhages which produce symptoms of a temporary apoplexy that the suspicion may be awakened of a pachymeningitis hemorrhagica. Thus an alcoholic subject who had had a sunstroke and had at various times suffered from blows on the head complained for some weeks of severe headaches, and suddenly had an attack of left hemiplegia attended by vomiting and a short general convulsion. In the course of two weeks all signs of the hemiplegia subsided and it was evident that no destruction of brain tissue had occurred. His head- aches continued and four months after the first attack a second with right hemiplegia and aphasia occurred which proved fatal. The au- topsy showed an extensive pachymeningitis interna with a large recent hsematoma on the left side and evidences of an old one on the right side. In any case where apoplectic symptoms occur and subside rapidly the possibility of hsematoma must be considered. During the attack and immediately after it the diagnosis of apoplexy is the only one possible. The pupil is dilated on the side of lesion, the head and eyes may turn toward the lesion, and a rigid state of the limbs may exist for some hours after the attack. The course of a case in pachymeningitis is sometimes very slow. The patient may suffer for several years from occasional attacks of severe headache lasting several days and then gradually subsiding, but leaving him with disagreeable cerebral sensations and a sense of inability to do his work. He may be aware of difficulty in fixing his attention upon business or matters of importance, and may suffer from difficulty in recollecting recent events. He may manifest all the symptoms of cerebral neurasthenia with occasional attacks of vertigo and prostra- tion. As time goes on he may have sudden attacks of unconsciousness, with or without twitching of a general character of the limbs, or with a true Jacksonian epileptic attack, and after such an attack he may be much prostrated for ten days or two weeks, being confused mentally, or almost stuperous, or being very irritable and restless. Then all these symptoms may subside and he may return to a state of apparent health and even be able to go back to business. But even when apparently PACHYMENINGITIS INTERNA. 707 well he will be known by those who are most intimately associated with him to show some changes in disposition and character which are unnatural. After a period varying from six months to a year the headaches may reappear, convulsions may be repeated, and inability to think and remember may again become noticeable. Attacks of hemiplegia, or of hemianopsia, or of aphasia may follow, and the patient may again be reduced to a state of invalidism. It is seldom that two remissions occur, but they have been observed. Finally, all the symptoms become intensified, the headache becomes continuous, the mental dulness becomes more marked, and the patient is obliged to go to bed, where he lies in a state of semi-stupor for weeks or even months. In this condition he can be aroused, will answer simple questions, but shows no interest in his surroundings or in his family, and is indifferent to his own condition. He will take food when it is offered, but seems to live in a dazed condition, and is very likely to neglect ordinary cleanliness. He sleeps heavily in the daytime and is wakeful at night, and very often has a muttering delirium during the night ; at times starting up in a state of alarm ; at times crying out as if in pain, and at times being restless and desirous of getting up and going out. A general condition of motor weakness gradually develops, even when the patient does not show the traces of apoplectic attacks. His movements become uncertain and ataxic, with considerable tremor of the extremities and even of the entire body. In getting up out of bed he will do so in a feeble manner, with much trembling and shaking of the trunk, and it seems difficult for him to retain his balance or to stand alone, oscillating movements of the body and of the head being constant. When he is up he seems unable to decide what to do, whether to remain standing or to sit down, and acts in a dazed manner. There is rarely any loss of sensibility to touch, temperature, oi* pain, but sometimes the patient appears to be deaf, and in some cases the eyesight is affected. The patient may remain in this condition for several weeks, grad- ually losing strength and weight, and finally dying of some compli- cation, such as obstinate constipation, cystitis, or bed-sores. A pro- gressive emaciation and a condition of extreme anaemia or of nephritis are not uncommonly observed in the course of the disease. Optic neuritis has been occasionally found. In these cases brain tumor may be suspected and cannot be excluded. Treatment. — It is so seldom that one can make a diagnosis of pachymeningitis interna hemorrhagica that it is almost futile to speak of treatment. Ice to the head, or, better, applications of the actual cautery, may relieve the headache. Purgatives of a drastic kind may also be used. When an attack of an apoplectic nature occurs it is to be treated as a cerebral hemorrhage. It is rarely possible to operate for the removal of such a hemorrhage at the time of its occurrence. There may be indications which justify an operation later, as in the following case : A child, aged nine years, had a severe fall on the head, that was fol- 708 PACHYMENINGITIS AND PAGHYMENINGEAL HEMORRHAGE. lowed by coma lasting several hours, after which a left hemiplegia was discovered. This subsided entirely in the course of two weeks, but he continued to have severe headaches, and after two months attacks of Jacksonian epilepsy on the left side, beginning in the hand, com- menced. These kept up with increasing frequency for six months, not being controlled by bromides. He was then operated upon by Mc- Cosh, a large bony flap being made and free access to the brain being had. The inner surface of the dura over the motor area of the brain was covered by a tliin, velvet-like layer of tissue which bled freely on being touched. This was adherent to the pia over the mid- dle third of the posterior central convolution, but was easily stripped off. It was removed by a curette, the hemorrhage, which was trifling, being controlled by pressure, and the dura was united and the wound closed. The child recovered from the operation and had had no return of the Jacksonion fits at the end of two years. The operation was undertaken on the belief that a cortical traumatic hemorrhage had left a cyst which was causing the epilepsy and which might be removed. The discovery of the condition of pachymenin- gitis interna hemorrhagica was a surprise. The ease with which the hsematoma was stripped off led to the idea that it was merely the remains of a flat clot, but microscopic examination showed it to be an organized membrane with capillaries identical with that found in pachymeningitis interna. MENINGEAL HEMORRHAGE. Hemorrhage from the middle meningeal artery occurs either with or without fracture of the skull as the result of trauma. In Fig. 296, the sites of the most common varieties of meningeal hemorrhage are shown. These hemorrhages are between the bone and the dura. The symptoms of such hemorrhage are general and local. After a blow or fall on the head, with or without apparent fracture, for fracture of the internal table may not be evident, the patient becomes comatose and is found to be paralyzed on one side, with the pupil on the side of the injury very widely dilated and not responsive to light. The coma may not be deep, the patient being rather in a deep stupor, and this may continue with varying degree until the pressure is relieved by trephin- ing. Or consciousness may be regained slowly, and then either (1) aphasia, or (2) hemiplegia, or both together, or (3) hemiansesthesia, or (4) hemianopsia are discovered according to the position of the hemor- rhage which causes a diffuse compression of one hemisphere. Such cases should be trephined as soon as the diagnosis is made and the torn vessel tied and the clot removed. Hemorrhage inside of the dura, either from its vessels or from sonie vessel in the pia mater is also caused by trauma with or without frac- ture of the skull. Hemorrhages which occur in infants during labor, from delayed or instrumental delivery, are usually inside the dura and result in a large clot spread out on the surface of the brain. In some MENINGEAL HEMORJRHAOE. 709 (iases the vessel torn is a large one and the clot is rapidly formed, pro- duces greftt pressure, and death follows. In other cases the vessel is a small one and the clot extends slowly ; the symptoms then gradually increase during several hours or even days until the bleeding ceases, and then subside more slowly still and only in part. In infants the existence of such a pressure by a large clot may prevent the process of development of the brain and lead Fig. 296, The meningeal artery and its relation to the fissures of Rolando and Sylvius. I., area of a hemor- rhage from the main artery, causing hemiplegia and aphasia ; II., area of a hemorrhage from the anterior branch, causing mental dulness and aphasia ; III., area of a hemorrhage from the posterior branches, causing sensory aphasia and hemianopsia. to atrophy and sclerosis. (See Chapter XXVIII.) The general symp- toms of pressure are diminishing consciousness to the degree of stupor, or even coma, headache, a slow pulse, vomiting, and possibly difficulty of respiration, and a rise of temperature. The local symptoms are those of compression of some part of the hemisphere. This is usually on the side of the injury, but occasionally on the opposite side. A dilated pupil on the side of the lesion, deviation of head and eyes to that side, and aphasia, symptoms of paralysis, or loss of sensation or of sight on the opposite side, are the local symptoms, and their value is to be estimated as in cases of cerebral hemorrhage. When these symptoms persist for more than two weeks there is evidence of con- tinued pressure, and it is not likely tliat a clot large enough to cause them will be absorbed, hence it must be removed. Neisser has made 710 PACHYMENINGITIS AND PACHYMENINGEAL HEMORRHAGE. the diagnosis in many cases by puncture of the skull and aspiration — the technique of which is more fully described on page 599. In one case he removed 180 c.c. of blood from the posterior fossa and saved the patient. In many cases this is merely a diagnostic procedure and is to be followed by trephining for the removal of the clot. The treat- ment, therefore, should be by trephining. The following case is a good illustration of this condition : A physician, aged forty years, was thrown from his carriage on i^ugust 17, 1889. He was slightly stunned by the fall, but had no wound, and was able to help his wife, who appeared to be injured, to reach home. For several hours he seemed to be suffering merely from bruises, and was able during the afternoon to converse with a patient and to administer a hypodermic injection. In the course of the even- ing, however, he became delirious, then stupid, and for the following three days he lay in a semi-comatose condition. On the morning after the injury he was found to be completely hemiplegic on the right side, and aphasic. When, after a week, his consciousness had fully returned, it appeared that the aphasia was purely motor, as he could understand what was said to him, and could read. The hemiplegia was attended by a partial anaesthesia, the paralyzed limbs felt numb, were less sen- sitive to touch and to pain, but acutely sensitive to cold and to heat. In this condition he remained until December, when he was brought to New York and admitted to Roosevelt Hospital. On examination, the patient, a large man, was unable to say any- thing, even " yes " or " no," the vowel sounds being the only sounds produced by effort. He evidently understood questions, and attempted to reply by gestures and by trying to write with his left hand. It was noticed, however, that he was mentally dull, so that the result of tests of muscular sense were not satisfactory, though he could be tested for tactile and pain senses, which were found to be impaired on the para- lyzed side. He was emotionally unstable, laughing too readily, and at times appearing to be much depressed. Ophthalmoscopic appearances were normal. Sight and hearing were normal. The right hemiplegia was partial. He could turn his eyes in all directions, but could not turn his head to the right. His left pupil was one-third larger than the right pupil, but both reacted normally. His face was slightly flat but not paralyzed, and his tongue protruded straight. His arm was almost totally paralyzed, the only motion possible being a slight abduc- tion at the shoulder. , His leg could be moved a little at the hip and knee, and when held up by two persons he could drag the leg forward a little, but could not stand alone. The hand was flexed and pronated, the leg was extended ; both were very rigid, and all the deep reflexes were greatly exaggerated, so that wrist and finger clonus, as well as patella and ankle clonus, were easily produced. He controlled his sphincters perfectly. There were no scars upon the head. It seemed probable that, as a result of the fall, there had been a rupture of a small vessel from which very slow hemorrhage had taken place ; the vessel was thought to be a vein rather than an artery, MENINGEAL HEMORRHAGE. 711 because of the very slow development of the symptoms. The situa- tion of the 'clot was thought to be upon the surface and not within the left hemisphere, and it was located upon the posterior part of the third frontal convolution and over the anterior central convolution in its middle third, as shown in the diagram. A flat clot in such a situation might fail to compress the face area and could produce an absolutely total motor aphasia, while a subcortical or capsular clot could hardly produce total permanent motor aphasia and paralysis of the arm with- out producing paralysis of the face and tongue. On the strength of this diagnosis it was thought best to operate. Meningeal hemorrhage. The situation of the clots causing aphasia and right hemiplegia. Removal resulted in recovery. (Starr and McBurney.) On December 13, 1889, McBurney trephined the skull. The tre- phine was applied at a point one and seven-eighths inches behind, and seven-eighths of an inch above the external angular process of the frontal bone, and the opening was then enlarged by the rongeur forceps upward and backward, the dura being laid bare over an oval area three by two inches. The dura did not pulsate. On opening the dura the pia was found to be very oedematous and discolored, and the surface of the brain was separated from the dura by a space half an inch in depth, and did not pulsate. The clot was seen lying beneath the pia upon the posterior part of the third frontal convolution and extending over the anterior central convolution in a thin layer (marked in lines on the diagram) into the fissure of Rolando, which was filled with a larger clot lying in the situation shown in the diagram and extending downward so as to fill up and distend greatly the cul-de-sac at the lower end of the fissure. The clot had not covered the lower third of the anterior central convolution and had not reached the upper quarter of the fissure of Rolando. The brain, at a distance of an inch about it, appeared to be healthy and pulsated, but the parts of 712 PACHYMENINGITIS AND PACHYMENINGEAL HEMORRHAGE. the cortex on which the clot lay were pulseless and stained a yellowish- red. After the pia had been incised the clot was removed little by little by fine sponges, at least a drachm of partly organized clot being taken out of the fissure of Rolando. The retraction of the brain from the skull was even more evident when the operation was com- plete. The operation was done under strict aseptic precautions, and was not followed by any rise of temperature. The wound was dressed in the open method, being packed with gauze and drainage tubes being inserted. Ou renewing the dressings three days subsequently to the operation it was found that the entire surface of the brain was pulsat- ing normally and that the brain surface presented a normal color and appearance. After a week the drainage tubes were removed, and after three weeks the wound had entirely healed, the level of the scalp at the bottom of the cavity being an inch below the normal level. On the evening of the day of operation the patient said " yes " and " no " for the first time since the injury, and after that time his recov- ery of speech and of power was progressive. After a few days it was noticed that he was rhuch more intelligent and no longer emotional. Power in his leg began to increase very soon, and two months after the operation he was able to walk with a cane. The return of speech was slow but continuous, and it appeared as if he were learning a new language. He repeated words after another until he had learned them. He talked in monosyllables for the first three mouths, then he began to put two words together, and then used short sentences of three or four words. Fifteen months after the operation he was able to practise medicine in his native town, was able to walk without a cane, could name at once any object shown to him, but in talking used only a few words at a time, not speaking fluently, wrote with his left hand, and had it not been for the loss of power in the right hand, might have been considered fairly well. The paralysis of the right hand remained, and with it a marked diminution of tactile and pain senses, two points being felt as one when two centimetres apart on the tips of the fingers. The muscular and temperature senses were perfect. He could move the arm and forearm in all directions with much force, and could supinate the hand, but movements below the wrist were very slight, the fingers being flexed and rigid. The reflexes were much less exaggerated than before the operation, clonus not being elicited except- ing at the ankle ; the head could be turned in any direction, and the pupils were equal. In this condition he remained for ten years, until he died of pneumonia. This subject being chiefly surgical the reader is referred for further details and for the technique of operation to Harvey Cushing's article on Brain Surgery in Keen's System of Surgery. CHAPTER XLI. CEEEBRAL MENINGITIS. Epidemic Cerebro-spinal Meningitis. Endemic Meningitis. Hydrocephalus. Septic Meningitis. Secondary Meningitis. Tuberculous Meningitis. Syphilitic Meningitis. Meningitis is an inflammation of the pia mater. It is an acute disease in the majority of cases, and is then of bacterial origin. There are several classes of cases : 1. Epidemic cerebro-spinal meningitis, or spotted fever, which is due to the invasion of the pia by the diplococcus intracellularis. 2. Endemic meningitis of infants due to infection by various micro- organisms. 3. Septic meningitis, which occurs with wounds, fractures, and operations, or after otitis media, and is due to the invasion of the pia by streptococcus or staphylococcus ; and secondary meningitis which occurs as a complication of pneumonia, ulcerative endocarditis, em- pyema, typhoid and typhus fever, influenza, the eruptive fevers of childhood, erysipelas, and in fact any form of disease due to a micro- organism. In these cases the germ of the disease attacks the pia mater, and has been found in it. The list of bacteria which have been detected in the pia is increasing daily, a large variety having thus far been found. There is no special bacillus of meningitis, though various investigators have isolated various forms and made a claim to its ex- clusive causative action. Thus Still has found a diplococcus allied to that of cerebro-spinal meningitis, Weichselbaum has described a men- ingococcus intracellularis, and Bibes a micrococcus lanceolatus. 4. Tuberculous meningitis, due to the direct invasion of the pia by the tubercle bacillus. 5. Syphilitic meningitis, due to an exudation of gummy material into the pia mater. The entire pia of the convexity and of the base may be involved in the inflammation, but certain forms of meningitis appear to select cer- tain localities. Thus in the epidemic form the entire pia, both of the brain and of the spinal cord, is invaded. In the septic and secondary forms the pia of the convexity is particularly affected, and that of the base may escape. In these cases the process may be localized in a small area. This occurs especially after wounds, after otitis media, and nasal disease. In meningitis of infants the base is particularly affected and hydrocephalus is a frequent result. In tubercular menin- gitis the pia on the base alone may be the portion inflamed, hence this form has been named basilar meningitis. In syphilitic meningitis the 713 714 CEBEBRAL MENINGITIS. pia on the base near the posterior perforated space and about the crus cerebri or at the sides of the medulla and pons is usually aifected. It is evident that this difference in location will cause a great difference in the symptoms of the various types. Pathology. — After a short period of hypersemia the congested pia becomes covered with an exudation of serum, lymph, fibrin, and pus. The serum infiltrates its meshes and collects between the pia and the brain, making the latter oedematous. It also collects below the arach- noid, lifting this so that it appears to contain cysts. The fibrin is de- posited in flakes on and in the pia, rendering it less transparent than normal and covering it and the brain with a thick opaque lymph. The pus is infiltrated through the meshes of the pia, and as it increases in Fig. 298. Acute meningitis of the convexity of the brain. The thickening of the pia, its infiltration with the products of inflammation, and the invasion of the cortex by cells is seen. amount fills up the space between the convolutions along the lines where the pia dips down, then it collects in the sulci, and finally may form masses of a yellowish-green color, covering the surface and filling up its irregular spaces. The cortex of the brain on which this serous and purulent exudation lies soon becomes affected; in fact, in every case one has to deal rather with a meningo-encephalitis than with a simple meningitis. The cortex is greatly congested. Wherever the pial vessels dip into the cortex, serum, lymph, and pus are exuded, and small hemorrhages and little collections of pus are found every- where in the cortical layers. Occasionally large areas of softening or little abscesses are found in the brain. The pia covering the cerebel- lum and the cranial nerves is similarly affected, and in cases where the process extends downward the pia along the entire length of the spinal cord may be inflamed and covered with lymph and pus. PATHOLOGY OF CEREBRAL MENINGITIS. 715 The pia extends into the ventricles, and hence a similar process goes on there, i^^sulting in an eifiisiou and consequent distention of the ventricles with serum, which soon contains flocculi of fibrin and pus. The cerebro-spinal fluid contains similar flocculi, cells, and free bac- teria, and hence by lumbar puncture and examination of the fluid obtained the diagnosis of meningitis can always be made. In cases in infants which last for more than a week a distention of the skull commences, due to this accumulation of fluid in the ventricles, and this results in a separation of the sutures, which goes on until a hydrocephalic shape of the head and a great increase in its size is produced. The collection of fluid in the lateral ventricles only occurs when an adhesion has formed in the membranes, closing the foramina and ob- structing the free communication between the ventricles and the sub- arachnoid space. The choroid plexuses of the ventricles and the epi- thelium covering them secrete the cerebro-spinal fluid, which is not a mere transudation from the blood. Being constantly secreted it must be drained away and absorbed. This drainage occurs through the cere"bro-spinal foramen and the two lateral forarhina of the fourth ventricle which establish a communication between the ventricles and the general subarachnoid space. The lateral ventricles communicate by the foramen of Monroe with the third ventricle, and this empties into the fourth ventricle by way of the aqueduct of Sylvius, the minute size of which secures a slow drainage. If any obstruction occurs to such a flow, either by compression of the aqueduct or by closure of these foramina, an accumulation of fluid will occur. In meningitis, particularly of the base, there occurs an adhesion of the membranes or an effusion of lymph which closes these foramina. Drainage being impossible fluid accumulates in the ventricles and causes hydrocephalus. Barlow^ believes that in many cases of congenital hydrocephalus and of chronic hydrocephalus developing slowly without symptoms of meningitis, a slight attack or a low grade of meningitis has occurred at the outset which has not been noticed or has recovered. In the majority of cases of meningitis in infants, if death does not take place before the third week, hydrocephalus appears. (See page 522.) In tuberculous meningitis there is rarely an exudation of pus. There is a deposit of miliary tubercles through the pia, both diffuse and in conglomerate masses. These are small white egg-shaped bodies, usually first appearing along the bloodvessels and always found on their walls. An exudation of serum lymph, and fibrin occurs, and these form a thick mucilaginous material in which the miliary tubercles are imbedded. This process is always much more marked about the base of the brain, where the many irregular spaces are filled with the new material. It may extend to the convexity, however, and then the exudation is more evident along the sulci and fissures, especially in the fissure of Sylvius. The miliary tubercles may be found scattered widely over the entire extent of the pia and in the ventricles. The ^ Allbutt's System of Medicine, vol. vii., p. 493. 716 CEREBRAL MEmNGlTlS. ventricles are always distended with cloudy serum in which fibriu and blood are found. This distention may be extreme and may produce hydrocephalus. The exudation surrounds the nerves upon the base and causes a neuritis. A diifuse encephalitis of the cortex or a hemor- rhagic encephalitis, either localized or general, may accompany tuber- cular meningitis. Masses of tuberculous material in a state of cheesy degeneration are also found in the membranes or in the brain in some cases. (Fig. 243, page 573.) Syphilitic meningitis is characterized by a serous and fibrinous exu- dation in the membranes attended by a deposit of mucilaginous material of a gummy nature which is semi-translucent, thick, and viscid, and which surrounds the nerves and fills up the spaces on the base or in the sulci of the convexity. It begins to appear along the bloodvessels, but soon becomes extensive. It is not attended by the production of pus, and there is not often any exudation of serum into the ventricles. The exudation invades the cranial nerves, especially the optic and ocu- lomotor, and causes a degenerative neuritis. The pia is thickened by a production of connective tissue which causes its firm adhesion to the dura and to the brain. Areas of softening on the cortex are commonly found. (See Fig. ^98, page 714.) The characteristic syphilitic endarteritis is always present in the arteries of the base. (See Fig. 288, page 687.) Lumbar puncture, first done by Quincke in 1891, is a valuable aid to diagnosis in all forms of meningitis. The patient is placed on the left side, the legs being drawn up and the body bent far forward. A line is drawn between the posterior-superior edges of the ilia, which will pass across the space between the third and fourth lumbar ver- tebrae. The lower edge of the spinous process of the third lumbar ver- tebra is found, and puncture is made by a long hypodermic needle just below this in children, and 1 cm. to one side of it in adults. The hypodermic needle is thrust in between the laminae, and should be pointed a little upward and inward. It can be felt to penetrate the membrane, and then its point can be moved more freely than in the solid tissue. The syringe is then unscrewed from the needle and the fluid allowed to run out. It must never be exhausted by suction. A small trocar may be employed in place of a hypodermic needle. A few cubic cen- timetres only of the fluid is to be taken in a test tube. The pressure under which it flows is to be noticed, and if it spurts out of the needle the internal pressure is abnormally high. This occurs in meningitis. As it will not run the patient's head and body may be elevated, and thus a few drops may be obtained. It is usually easy to obtain 10 c.cm. Normal cerebro-spinal fluid is clear, straw-colored and contains a few cells and not more than 0.2 per cent, of albumin. In menin- gitis it is cloudy, contains a little blood, floccculi of fibrin, polynuclear leucocytes, pus, and micro-organisms. It usually contains more albumin than normal. On standing the cerebro-spinal fluid coagulates, and the coagulum forms more quickly in cases of meningitis and is firmer than in health. It is the existence of pus, leucocytes, and bacteria which are the most important diagnostic signs of meningitis. Cultures should SYMPTOMS. 717 always be imde of the fluid. The finding of streptococcus, staphylo- coccus, pneumococcus, or diplococcus intracellularis, or of tubercle bacilli, makes it certain that a meningitis is present. In tuberculous meningitis mononuclear lymphoid cells are also found, while in the other forms poly nuclear cells are more common. When the diplo- coccus or pneumococcus is not found in the cerebro-spinal fluid it is occasionally found in the blood and in such cases direct injection of 10 c.c. of the antiserum into a vein repeated daily has resulted in cures. Symptoms. Epidemic Cerebro-spinal Meningitis. — The disease, though occasionally occurring in epidemic form, particularly in winter and spring, both in cities and in the country, is more commonly seen as a sporadic affection, and is quite rare. Children are more suscep- tible to the infection than adults. Unwholesome, dirty surroundings, malnutrition, and overexertion, either mental or physical, are supposed to make one more susceptible. Though infectious, the disease is not contagious, and is not carried in clothing. The period of incubation is not determined. The symptoms appear suddenly, with a chill, temperature of 104° F. or higher, full rapid pulse, headache, pains in the back, and vomiting, which is projectile. The patients feel very ill, are restless and irri- table, and soon complain of light and sound. Stupor and coma come on rapidly. Within a day or two of the onset the headache becomes very intense, and stiifness in the back of the neck appears, which is very painful. The head is held rigid or is drawn back, the back also becomes stiff, so that children can. be raised from the bed by lifting the back of the head, and a state of opisthotonus develops, attended by great pain in the back and limbs and extreme hyperaesthesia to all forms of sensation. The slightest touch causes great pain. In addition to the rigidity of the muscles there are occasional spasms of a clonic or tonic kind in the extremities, the face, too, being drawn, and tremor in the limbs may be observed. The pupils are dilated, and sometimes unequal, and do not react to light. Grinding of the teeth and strabis- mus are early symptoms. Within two days of the onset delirium of an active character begins, and this continues, alternating with stupor and coma, for a long time. The mind is always clouded and filled with illusions, and the patients have no memory of what has occurred when they recover. The delirium may at times be wild, and is always worse at night and when the temperature rises. After the third day an eruption appears in the majority of cases, though it is not an invariable symptom. It is petechial, diffused over the entire body, but deeper, and purple in spots. Herpes appears on the lips early ; erythema, pemphigus, and gangrene of the skin have all been seen in various cases in addition to the petechige. Any scratch on the skin leaves a dark red stripe, the tdche e^rehrale of Trousseau. The gastro-intestinal functions are affected from the start, nausea, vomiting, diarrhoea, or more commonly, obstinate constipation being present. The urine contains albumin, 718 CEREBRAL MENINGITIS. casts, and sometimes blood. It is sometimes retained, but may be passed involuntarily, as are also the feces. The spleen is enlarged. Contractures soon develop, and the limbs are flexed until paralysis ensues and they relax. The reflexes are increased. There are some cases of a malignant type which end within the first two or three days. Many cases prove fatal within a week. If the patients do not die of fever or of heart failure within a week of the onset they usually have a remission in the symptoms, but these return again, and the case may go on for many weeks with varying intensity. There is no typical temperature curve, and great variations are pos- sible. In one patient who recovered I saw a rise on five occasions during two months to 106° F., the ordinary variation being between 101° and 103° F., with occasional falls to normal. A sudden rise or a sudden fall is unfavorable. The temperature may be intermittent. The pulse may be rapid, especially in children, or it may be as slow as forty per minute. It is frequently intermittent. The respiration is irregular, at times of the Cheyne-Stokes variety, and attended by sigh- ing and yawning. It is sometimes difficult to feed and care for those patients, as they resist In their delirium all attempts at nursing, and rapid and extreme emaciation results. The most prominent symptoms during the long illness are headache, delirium, stupor, insomnia, rigidity of the muscles, especially of the neck and back, hypersensitiveness to touch, to sound, and to light, and a gradual loss of voluntary power. The cranial nerves are almost always seriously affected. Strabismus, which develops early, persists. It is often attended by nystagmus. Vision may be obscured by keratitis, and optic neuritis and atrophy may develop and finally render the patient blind, though in two patients I have seen a partial recovery of sight, sector-like defects in the visual field remaining. Deafness is a common symptom, due to otitis media or to an affection of the auditory nerve, and may be permanent, lead- ing to deaf-mutism in children. Facial paralysis is very common. Difficulty in swallowing and in articulation are observed in many cases. General convulsions may occur from time to time in the course of the case. Under the antiserum treatment of Flexner the symptoms rapidly subside and the patients regain their consciousness and in the course of a few days appear to be well. I have seen a child sitting up in her crib and playing naturally with her doll, two days after she had been in a state of coma with strabismus, opisthotonus, and occasional con- vulsions. If relapses occur when the injections are stopped, they may be resumed. The most permanent of all the symptoms is a state of mental apathy and indifference. Even after the delirium and stupor have passed oft' the patients may show no mental activity, may fail to control the sphincters, may not recognize their parents, may have no interest in persons or events, and may talk with great difficulty. I have seen these symptoms remain for several weeks after the temperature had become normal. In the patients who do not succumb to general exhaustion SYMPTOMS. 719 or die of a complicating pneumonia, arthritis, or cystitis, or bed-sores, there is a very slow return to health, and sometimes it is six months or a year before the mental and physical symptoms have entirely dis- appeared. The severity of the disease depends somewhat upon the character of the epidemic, some epidemics being very fatal, in some the mortality being 75 per cent., in others being as low as 20 per cent. In some epidemics the tendency to complications seems to be great. In sporadic cases the prognosis depends on the intensity of the symp- toms, but it is never well to give up hope, as very bad cases may recover. The treatment consists in supporting the strength by fluid food and alcoholic stimulants, given often and freely ; in the application of ice to the head and spine in bags, or of ice-water in coils ; in cool bathing when the temperature exceeds 102° F.; and in the free use of sedatives, of which bromides, phenacetin or acetanilid, sulphonal, and trional, and in many cases codeia or morphine, in small dos6 frequently re- peated, are the best. Strychnine should never be given. Some authors recommend ergot in large doses. It is questionable whether mercury or iodide of potassium are useful, though some writers urge their use. The discovery by Flexuer ^ of an antiserum for cerebro-spinal menin- gitis has been followed by its successful use all over the world. The method of its use is as follows : By lumbar puncture 15 or 30 c.c. of cerebro-spinal fluid is withdrawn, and the same amount of antiserum is injected directly into the spinal canal, slowly and carefully, to avoid the production of symptoms due to increased pressure. The injection is to be repeated every day for three or four days. The earlier the injection in the course of the disease the better the results. Its direct effect can be observed by testing the cerebro-spinal fluid daily for diplococci, which rapidly disappear under its use. By the use of the serum the mortality has been reduced from 89 per cent, to 25 per cent, in several severe epidemics. The antiserum is to be obtained at the Rockefeller Institute in New York. In the stage of recovery iodide is useful in doses of twenty grains three times a day for adults. 2. Meningitis in Infants ; Hydrocephalus. — Infants appear to be much more liable to the infection of meningitis than adults, and in them the picture of the disease dififers greatly from that already pre- sented. Both sexes are equally liable. The majority of cases occur during the first and second years of life, though no age is exempt. Barlow's statistics point to a greater susceptibility between the third and sixth months. Catarrhal diseases of the intestines, bronchi, or nose precede the onset in many cases. A history of injury to the head is obtained in not a few. The symptoms develop acutely, and are well marked within three days of the onset. Vomiting, convulsions, retraction of the head, screaming, and irritability of temper are the first symptoms, and are soon followed by sleepiness and languor, by bulging of the fontanelle, by strabismus and nystagmus, and by some inequality or abnormality in the action of the pupils, by champing movements of the lower jaw, ^ Jour, of Experimental Medicine, Jan., 1908. 720 CEREBRAL MENINGITIS. lips, and tongue, and by extensor spasms of the limbs, and in some cases by opisthotonus. Paralysis of the face and limbs is occasionally seen, but is' rare. The infant undoubtedly suffers from great pain, as is shown by the constant crying and screaming, which are kept up at night and by day, and by pulling its hair. This is an early symp- tom, and subsides later when the stage of hydrocephalus is reached. Irregularity of respiration is often noted, and the pulse is either very fast or very slow. The skin is flushed and shows the mark of any irritation as a broad red line or spot. The temperature varies greatly between 100° and 106° F. The abdomen is often retracted; consti- pation is more frequent than diarrhoea. One of the most noticeable symptoms is the rapid emaciation, and these children become mere skeletons in the course of a couple of weeks. Hydrocephalus usually develops in the second week of the disease. In addition to the in- crease in the size and change in the shape of the head, there is often a slight bulging of the eyeballs, and strabismus appears. This con- dition may persist for a long time, or it may gradually subside as the child recovers. The course of a simple meningitis may be rapid, and a fatal termi- nation may occur within a week. This rapid course occurs chiefly in the vertical and sometimes in basal cases. More often the symptoms persist a long time and the case runs a course which extends over several weeks. The variation is so great in different cases that no average duration can be stated. There are some cases which continue even for four or five months, and then recover. That a large percent- age die must be admitted. Authorities make the mortality from 60 to 80 per cent. It has been thought by some that children never recover from meningitis, but this is not my experience. I have seen some well pronounced cases in the Baby's Hospital which terminated in recovery. Even cases which develop hydrocephalus, if this is moderate, may come to a standstill and gradually get well. These patients may be left blind or deaf, or they may show a spastic gait for years, or they may show mental defects, but nevertheless they recover from the acute meningitis. Occasionally during the stage of improvement, or even after recovery appears to be complete, sudden death occurs. There is no disease, therefore, in which the prognosis is more uncertain. The treatment must be quite similar to that described in the epi- demic form. Leeches behind the ears, ice to the head and neck, or a cold coil, antipyretics, and cool sponging are of service in keeping down the temperature. The child should be nourished as well as possible on milk properly modified to its age, and on broths. It may be neces- sary to feed by gavage if there is much vomiting. There are no drugs which act on the bacteria, and hence there is no means of decreasing the pathological process. In some cases iodide has been of service, and it may be given if the stomach will retain it in one to three-grain doses four or five times a day. It is not to be forgotten that some cases are secondary to otitis media ; hence if any evidence of this is present free drainage must be secured SYMPTOMS. 721 by incising the drum or by operating on the mastoid. Cases are now frequently*^reported where the mastoid operation, followed by trephin- ing, has resulted in the evacuation of a small collection of pus and has been followed by recovery. 3. Septic and Secondary Forms of Meningitis. — The symptoms of a septic meningitis always develop after some one of its causative con- ditions has been in progress for some time. Hence they are preceded by the symptoms of a cranial fracture or operation, an otitis media, a chronic nasal disease, or an abscess, or erysipelas of the face. If, in the course of any of these affections the patient has a sudden chill, with rise of temperature, rapid pulse, severe continuous general head- ache, and delirium or convulsions, and if thus an illness begins which is evidently septic, is attended by a stuporous mental state, som- nolence, vertigo, vomiting, and a fever running from 101° to 104° F. daily, there is every reason to believe that a septic meningitis has de- veloped. The general symptoms mentioned usually appear before any local signs of brain disease. And these will depend largely on the area which is affected by the meningitis. Thus, in fractures of the skull which lie over the motor or sensory areas there appear local spasms, or aphasia, or paralysis, or disturbances of sensation, first hypersesthetic conditions, and later loss of sensation. After otitis media there may be no local signs of meningitis, as the temporal lobe on the base has no known function ; but if the disease advances upward on the left side a sensory aphasia may appear. If the meningitis is on the base the cranial nerves may become involved ; nystagmus, strabismus, twitch- ing, or paralysis of the face, grinding of the teeth, and optic neuritis develop. These sometimes occur after otitis media. If the meningitis starts from nasal disease there are no local signs excepting loss of smell and a greater degree of mental disturbance than in the other cases. The same general symptoms developing in the course of any of the infectious diseases will indicate that meningitis has begun as a compli- cation. The headache which is always present in meningitis is severe and causes great agony. The patient grasps the head and moans with pain even during his delirium and stupor. Children pull the hair, knock the head upon the pillow, and scream, even in their sleep. The sudden piercing cry of pain which wakens the child is known as the cry of meningitis. The pain varies in intensity, and is worse at night or when the temperature rises. The delirium is another characteristic symptom of meningitis. It is sometimes a low muttering delirium from which the patient can be momentarily aroused, but he does not answer questions intelligently. At times it is a more active delirium, the patient wishes to get out of bed, to dress and go out, or is wildly excited, and struggles with his nurses and has to be tied in bed. In the intervals of delirium there is a stupor which may deepen into coma. The fever of meningitis is high. It dose not fall or rise suddenly, as in thrombosis of the lateral sinus, or in pyaemic states, but runs a course between 100° and 104° F., and occasionally the patient may have a chill. The pulse, which at first was rapid^ varies as the disease 46 722 CEBEBBAL MENINGITIS. goes on. It often becomes intermittent and irregular. It may for a few hours be slow, even 40. It does not vary with the temperature. The general hypersesthetic state of all the senses is very characteristic of meningitis ; the patient begs to be left alone in the dark, starts at slight noises, and cannot endure to be touched or examined. There are twitchings of the muscles, which are increased by tapping, and there is often an increase in all the tendon reflexes. The neck and back become stiff and painful early in the course of the disease. And various cranial nerve symptoms indicate that the eifusion on the base has begun. The pupils are usually contracted at first, do not dilate in the dark, and do not react to light. Later they may become dilated, are often unequal, and the condition of the pupil varies from day to day. In some cases the pupil contracts to light, but immediately dilates again, and continues to change in size; this is termed hippus. Ptosis, par- alysis of some of the ocular muscles, with strabismus and nystagmus, soon develop. Optic neuritis occurs in about 60 per cent, of the cases. The facial nerves may be irritated, causing a twitching of the facial muscles and trismus or paralysis. As the case progresses general con- vulsions may occur, followed by coma. Monoplegia or hemiplegia may develop at any stage. There is an irritation or paralysis of the vaso- motor system shown by blueness of the extremities, a reddening of the skin when scratched or rubbed, and profuse sweating. There is ob- stinate constipation. The urine may be retained or may dribble. It often contains albumin, and is of high specific gravity and scanty. The spleen is enlarged. In the later stages there may be a tendency to bed-sores. The duration of a meningitis may be from four days to two weeks. In rapid cases all the symptoms appear in great intensity, the tem- perature rises high, the pulse is weak and rapid, coma comes on, and the patients have Cheyne-Stokes respiration, and die either in coma or in convulsions. In slow cases the stage of irritation gives place to a stage of paralysis and the patient is exhausted by the fever. I have seen several cases which ran a slower course and in which , symptoms persisted for four or even six weeks. In these cases remis- sions in the symptoms, with subsequent exacerbations, took place ; but the general symptoms of fever, headache, somnolence, and progressive emaciation were constant. The formation of abscess in the brain or the occurrence of thrombosis of one of the sinuses, either of which may be a complication, makes the prognosis hopeless. The diagnosis of meningitis is to be made by an examination of the cerebro-spinal fluid obtained by lumbar puncture (see page 706), and this should be tried in all doubtful cases. The diagnosis from abscess of the brain and from thrombosis of the lateral sinuses has been considered on pages 558 and 565. The prognosis in septic and secondary meningitis is always serious. Patients rarely recover. Nevertheless, the possibility of recovery has been proven in many cases in every variety of the disease, and hence SYMPTOMS. 723 it is always legitimate to hold out some hope. It is also to be remem- bered thai there are many cases presenting serious nervous symptoms quite similar to those of meningitis in their character, but not quite equal to them in severity, which pass off in the course of four, six, or eight days. I have seen many such cases in connection with otitis media. I have seen some in children complicating other diseases or appearing alone. We do not know what the pathology of such cases is. They have been ascribed to cerebral hyperemia, to serous men- ingitis, to oedema of the brain, to non-purulent encephalitis, and to direct toxaemia of the brain. The only means of diagnosis of these cases from meningitis is by watching their course and observing the recovery. Hence in the early stage of supposed meningitis of a mild type the prognosis must be guarded, as the diagnosis may be wrong. The treatment of septic meningitis or of secondary meningitis should begin by an active interference with the cause. Fractures should be trephined and a possible source of infection sought and free drainage opened. In middle-ear disease an early exploration of the mastoid cells and prompt opening of the skull, with drainage, are often neces- sary. In abscess of the brain and sinus thrombosis operation affords relief. In other cases operations are impossible. Absolute quiet in bed, ice to the head, brisk purgatives, leeches to the mastoid region or temples, blisters to the neck, and the free use of narcotics, the coal- tar products, and morphine are to be employed. The temperature is to be controlled by cool sponging rather than by baths, as it is impos- sible to move the patient into a tub. Antipyrine and phenacetin may help to reduce it if it rises above 103° F. The heart may require stimulation, and for this caffeine is better than either alcohol or strychnine. The delirium may be somewhat diminished by the free use of bromide and chloral. If the persistent headache is soon followed by optic neuritis it is probable that a large effusion has occurred, and it is possible that lumbar puncture may reduce this. In some cases it has succeeded and an improvement has followed. From 50 to 100 c.c. of fluid have been withdrawn daily for several days under strict antiseptic precautions. 4. Tuberculous Meningitis. — Children between the ages of two and fifteen years are more commonly affected by tuberculous meningitis than are adults, though no age is exempt. The symptoms of this form of meningitis are numerous and differ so widely in different stages of the progress of the case that all authors prefer to group them in periods. There are (I) a period of incubation, when the symptoms are slight and rather indefinite, but really well marked when one looks backward and contrasts the condition with that of previous health ; (II) the period of irritative symptoms ; (III) the period of paralytic symptoms and coma. I. In the period of incubation the child, without reason, ceases to gain weight and begins to lose, is fretful and uneasy, shows little desire to play, and cannot be amused or diverted for any length of time, very often manifesting a marked change in disposition and unusual likes 724 CEREBRAL MENINGITIS. and dislikes. At night it does not sleep soundly, wakes on slight noises and cannot be put to sleep, or groans and cries in sleep, and sometimes grinds the teeth. In the daytime it may be drowsy. Vomiting, occurring without apparent cause and recurring in attacks, is usually an early symptom, and is attended by considerable prostra- tion. Headache soon appears, being complained of by children who can talk and being shown by crying, tearing the hair, and beating the head in infants. During this period the temperature is usually 100° or 101° F. at night, but nearly normal in the morning, the pulse is more rapid than usual, and the respiration regular, but attended by yawning and sighing. The appetite is poor, the bowels are often constipated; occasionally there is diarrhoea. The urine is usually increased in amount, and may show high specific gravity and albumin in small amount. The prodromal stage is longer in tuberculous menin- gitis than in other forms. As tuberculous meningitis is secondary to tuberculosis elsewhere in about 80 per cent, of the cases, some symptoms referable to the lungs, or intestines, to the joints, or the bones, or the spine, to the brain, or to the lymphatic glands, are to be detected when the child is carefully examined. These symptoms should lead to the diagnosis, even in the stage of incubation. In any case of doubtful nature the Calmette test will prove the existence of tuberculosis ; and lumbar puncture will yield fluid in which tubercle bacilli can be found. This stage may last from four days to three or four weeks. II. The stage of irritative symptoms appears suddenly, usually with a general convulsion, followed by stupor. The child, who has grad- ually become weaker and sicker, complains of most intense headache, is hypersensitive all over, and cannot endure being touched, shuns the light and noise, and cries when disturbed. It is somnolent, but cries out in sleep, grinds its teeth, and rolls its head, wearing oif the hair from the occipital region. Then, without warning, it has a general convulsion, which may be repeated at intervals. When it comes out of the convulsion it is stupid and cannot be aroused, but cries when disturbed. There is usually some rigidity of the neck, which increases rapidly and extends to the spine, so that the entire body is stiff, and after a time the head is drawn backward. Sometimes the limbs become rigid and contracted. The reflexes are exaggerated. The pupils are contracted and do not respond to light, and are often of unequal size. There may be nystagmus and hippus. The temperature begins at this time to rise at night to 102° F., the pulse becomes faster and is now occasionally intermittent and irregular, and the respiration is irregular, a series of inspirations, each deeper than the last, being followed by a sigh and cessation for a few seconds. This stage may last for two or three days, or even ten days, during which time the stupor deepens, and the child sleeps more and more continuously, and it is harder to arouse or interest it. Delirium is a common symptom, the child talks to itself, occasionally screams out, and may not know its parents. Toward the close of the stage muscular twitching is frequent and par- SYMPTOMS. 725 tial convulsions may occur ; the abdomen is retracted, tdche c^rSbrale is well marked, and an ophthalmoscopic examination shows choked disks. The skin is dry, and ulcers may form about the ears or on the buttocks. In spite of the severity of the symptoms, however, the child may at times seem for an hour or more to be better, may be conscious and alert, and have no pain. But these intervals become infrequent as the last stage approaches. In this stage lumbar puncture usually shows the presence of tubercle bacilli in the cerebro-spinal fluid. III. The stage of paralysis or coma comes slowly. The child gets worse, the stupor deepens, so that it is impossible to arouse it, and para- lysis of some of the cranial nerves appears, causing strabismus, facial paralysis, or difficult swallowing, and general paralysis of the limbs, or hemiplegia. The pupils are now dilated and fail to act to light ; choked disk appears, if it has not before ; tubercles may be seen in the choroid ; the opisthotonus is more marked, and the abdomen is re- tracted. The temperature begins to rise in the morning as well as at night, the pulse becomes very rapid and weak, respiration is irregular and labored and of the Cheyne-Stokes character. It is almost impos- sible to feed the child, and emaciation is rapid. The urine is retained or is passed involuntarily. The coma deepens, and the child dies of exhaustion or in a convulsion. The duration of this stage is from two days to a week, depending on the nutrition and inherent strength of the child. A quarter of the cases die in the first week after the second stage begins, a quarter in the second, a quarter in the third, and the remainder within eight weeks. In infants whose fontanelles are not completely closed a condition of hydrocephalus is liable to develop and to go on increasing unil death. There is no doubt that almost all cases die, and we have no means of arresting the progress of the disease. Yet cases have been reported in which recovery has occurred, even when tubercle bacilli have been found in the cerebro-spinal fluid. Treatment is palliative only, the various symptoms being treated as they arise. It has been proposed to open the skull and drain the base. This has been done once with apparent success by Ord and Waterhouse, but their example has not been followed by others. As all cases are hope- less, such a measure, though theoretically of little value, may be worth a trial. It failed in one case under my care. Injections of tuberculin have failed to arrest the disease. The following case is a good illustration of the course of the disease : Girl, aged two years, of healthy parents, began to have slight attacks of sudden loss of consciousness with convulsions, on November 7th, and complained of headache, nausea, and vomiting. During the first two weeks these symptoms continued, and she was very fretful, dull, and stupid, emaciated rapidly, and had a temperatuse of 100° F., at night. During the third week the temperature reached 102° F., at night and did not go below 100° F., in the morning; her pulse was 120, regular, respiration regular; she had much headache, retained little food, cried out in sleep, and was drowsy and dull. On Novem- ber 28th she had a convulsion limited to the right side of the face and 726 CEREBRAL MENimiTW. right arm lasting an hour and a half, after which she slept for two hours and had a temperature of 104° F. There was no paralysis after the convulsion, but she failed to recognize her mother and nurse and kept her hand on the left side of her head, with signs of pain. Her pupils were dilated, reacted sluggishly, and dilated in the light, being undulatory. Her optic disks were normal. During the following week she failed steadily, being in a state of stupor, and finally of coma. Her temperature varied from 101° to 104 °F., pulse became 140 and intermittent, respiration sighing, irregular, and finally Cheyne-Stokes. She had one right-sided convulsion. Her pupils were contracted when she was let alone, but dilated widely when the eyes were open and un- dulated. The optic disks became cloudy and finally choked. The face was flushed, and there was tdehe Gi.r^brale. The abdomen became retracted. There was no paralysis, but the knee-jerks, which in the first weeks were absent, became exaggerated, and ankle clonus was obtained. She died on December 5th. The autopsy showed tubercles scattered over the pia, especially on the base and in the fissures of Syl- vius. There was much serum under the pia. The ventricles were greatly distended by cloudy serum. There was some oedema, but no inflammation of the brain and no apparent cause for the limitation of the convulsion to the right side, though there was more serum over the left hemisphere, about the motor zone, than elsewhere. In this case it was evident at the autopsy that no operation would have had any efiect to relieve the condition. 5. Syphilitic Meningitis. — The symptoms of this form of meningitis develop more slowly than those of the other forms, and are more easily and successfully treated. They resemble the symptoms of brain tumor, but are more rapid in their progress. The patients suifer from general sensations of discomfort in the head, from headache, which is often occipital, sometimes frontal, sometimes diffused over the entire head, and is usually worse toward evening, and keeps them awake in the night. They become nervous, cannot control the emotions, and can- not think quickly or keenly ; hence they are unfitted for business. They are subject to attacks of vertigo, and may at times vomit unex- pectedly without preceding nausea. After a time dimness of vision may be noticed, and an ophthalmoscopic examination shows an optic neuritis; or a third nerve palsy may suddenly occur, with ptosis, ex- ternal strabismus, and immobility of the eyeball and pupil ; or a facial palsy or sixth nerve affection, with internal strabismus may occur. It is more rare for the other cranial nerves to be affected although intense neuralgia of the face, deafness, and difficulty of swallowing have been observed. After some one or more of the cranial nerves on one side have become paralyzed a hemiplegia of the opposite side not infre- quently develops, not complete, but attended by increase of reflexes. Sensory disturbance is rare. Polyuria is a very common symptom in these cases. Occasionally a cerebellar gait has been noticed. In one of my cases general convulsions occurred several times before treat- ment began to produce an effect. The symptoms, therefore, are quite TREATMENT. 727 like those of tumor on the base of the brain. Sometimes the gummy exudation^'^is in the Sylvian fissure and causes compression of the blood- vessels supplying the cortex. In these cases sudden attacks of aphasia or hemiplegia may occur. The meninges of the convexity are occasionally involved in a syph- ilitic meningitis, and in these cases symptoms resembling a localized brain tumor, attacks of Jacksonian epilepsy, or of aphasia, followed by monoplegia, may occur. The headache may be localized at one spot and may be attended by tenderness on percussion of the head. Mental symptoms are prominent in these cases, resembling those of paresis, but with dementia more evident than delusions. The symj)- toms may be very irregular and intermittent, as in the basilar type, severe on one day, slight on the next ; they may vary in their location, and they are rarely as permanent and continuous as in brain tumor. The occurrence of an optic neuritis is the exception in menigitis of the convexity. The difficulty of diagnosis between a specific meningitis of the convexity, paresis, and brain tumor is so great that in every case where the question of diagnosis arises it is well to try antisyphili- tic treatment. If this fails operative treatment may be considered, for successful removal of gummy tumors has been recorded. The various symptoms named develop gradually in the course of a month or six weeks, increase in intensity, and prostrate the patient completely. But when treatment is begun they soon begin to subside, ^ and finally may entirely pass away, leaving the patient quite well. In cases of long duration, where the nerves or the brain have been seriously compressed, and where degeneration in the nerve trunks or in the brain tracts has been produced, the recovery is much delayed or may be incomplete. Thus in one of my cases which showed paralysis of the sixth, seventh, and eighth nerves .on the left side, and a marked cerebellar gait, with staggering toward the left, the cranial nerve palsy recovered, the gait became natural, but there has remained at the end of three years a sense of uncertainty of position and some vertigo on rapid walking and an exaggerated knee-jerk on the right side. In another case, after a period of occipital pain, worse at night, the right sixth and seventh, then the fifth, then the third nerves were paralyzed, and then a left hemiplegia appeared. These symptoms subsided under treatment, but after a few months deafness in the right ear and a marked staggering to the right ride, with polyuria, developed. These also subsided under treatment, and for ten years this man has been well and able to work, but suffers from vertigo. Prognosis. — The prognosis is fairly good in syphilitic meningitis, as treatment promptly causes the absorption of the exudation ; but it must not be forgotten that symptoms due to an actual destruction of cerebral tissue are permanent. Treatment. — Treatment, no matter at what stage in the course of syphilis, should l)e by means of inunctions of mercury. One drachm of })lue ointment is to be well rubbed into the body daily, and the absorption is increased by a daily bath in water of a temperature of 100° 728 CRBEBBAL MENINGITIS. to 108° F. for half an hour. This bath may be followed oy cool sponging to prevent too great sweating and taking cold. The applica- tion of mercury should be to a different part of the body each day, and this part should be bandaged, so that any ointment left on the skin may be absorbed and not rubbed off by the clothing. I prefer this method to that by hypodermic injection of corrosive sulDlimate, though this may be resorted to in some cases. Pills of mercuric iodide, red or green, usu- ally derange the stomach and their effect is less rapid than that of the inunctions. After the mercury has been given for one week iodide of potassium is to be begun in addition to the mercury. It is my rule to begin with a dose of twenty-five grains three times a day, as thus the coryza which is caused by small doses is avoided. The amount is then increased rapidly, one grain at each dose until three hundred grains daily are being taken. On reaching a dose of fifty grains it is better to increase the number of doses than to increase the size of each dose. If the iodide is given well diluted in water, in some gaseous mineral water or in milk, it can usually be taken for several months. The mercury should be continued with the iodide until marked improvement is manifest or until salivation is caused. This should, of course, be pre- vented by the use of a mouth wash of chlorate of potash, and by clean- ing the teeth after each meal and after each dose of medicine. Usually three ounces of blue ointment are sufficient but more may be needed. The general strength of the patient should always be kept up to the best point ; every possible means being used to promote health. An out-of-door life in good country air, moderate exercise, the best food possible, and a generous diet, with moderate use of stimulants and tonics of all sorts are to be advised. The benefit of a course of treat- ment at hot springs or in a sanitarium is partly due to the more health- ful surroundings than city life affords. PART III. FUNCTIONAL DISEASES. CHAPTER XLII. THE PAINFUL NEUEOSES. Neuralgia. Meralgia. Herpes Zoster. Eeferred Pains. Neuralgia is a disease of a sensory nerve characterized by pain in the course of the nerve or in its peripheral distribution. Meralgia diifers from neuralgia in that it occurs in the legs and only on standing and walking, but never at rest. This form is often at- tended by paraesthesia in the distribution of the nerve and by slight anaesthesia. Herpes zoster is a trophic disturbance of the skin situated always in the domain of a sensory nerve — and often attended by neuralgia. Referred pains are painful sensations felt in some region of the body, but actually due to some disease of an internal organ, whose central nervous mechanism is located in the part of the nervous system with which the region in which the pain is felt is connected. NEURALGIA. Etiology, — Neuralgia may be due to toxic agents of external or of internal origin ; organic or inorganic ; causing an irritation of the neu- rones, or of their axones. It may be caused by congestion, without or with a serous exudation in the sheath of the nerve, and consequent compression and irritation of the nerve fibres. It is often due to dis- ease in the bloodvessels accompanying the nerve, which prevents those processes of osmosis necessary to proper nutrition. It is also the first symptom of organic disease in the nerve fibres, or in the endoneurium, of a primary inflammatory character, since many cases of neuritis are preceded by attacks of neuralgia. It not uncommonly develops in nerves which pass through or near organs which are the seat of dis- ease. And it is a frequent manifestation of certain disorders of nutri- tion, indigestion, stomach or intestinal fermentation, rheumatism, gout, diabetes, anaemia, chlorosis, as well as of the various infectious diseases, especially malaria and syphilis. The majority of authorities, however, do not regard neuralgia as necessarily a peripheral disease, althougli its manifestations are periph- eral. The sensory nerves send their impulses inward to the gray mat- ter of the spinal cord or brain axis, where such impulses are primarily received in a peculiar substance — the gelatinous substance of Ro- 729 7S0 neuhalgta. lando — which appears to be a fiue felt-like structure containing many minute cells or nuclei. Thence these impulses are sent up by the cen- tral sensory tracts to the cerebral cortex, where they are consciously perceived. An irritation in the gelatinous substance or in the central tracts is capable of producing pain referred to the periphery, as many cases of organic disease in these parts prove. Hence it has been thought that some disturbance in nutrition of the central sensory re- gion is the usual cause of neuralgia, and this theory receives support, as Gowers has held, from the facts that pain may be felt in adja- cent parts of several nerve regions, may radiate into adjacent nerve regions, or may be felt in a region not that of the irritated nerve. Neuralgia may be hereditary. It rarely develops during childhood, but from youth up to old age no one is exempt. Women appear to suffer more than men. It occurs more frequently in nervous and hys- terical individuals, and emotional excitement is said to be a cause. It is particularly frequent in sensitive persons in a climate where damp- ness is common. Attacks bear a distinct relation to atmospheric changes ; a low barometer, a high degree of humidity, and a falling temperature combined are often exciting causes. Certain electric states of the atmosphere, of which as yet we have no precise knowledge, and no means of recording, are capable of caus- ing attacks ; and persons who are subject to neuralgia are conscious of sensations which coincide with changes in electrical tension. Thus many persons are warned by a neuralgic attack of the coming of a thunder shower, or of a severe storm, some hours before it arrives. Attacks occur with much greater frequency in winter than in summer. Exposure to cold is the most common cause of an attack, and when a nerve is painful a draught of air often causes a sudden paroxysm, while heat almost uniformly relieves. Patients are usually hypersen- sitive to cold in the painful area and instinctively protect it when exposed. Symptoms. — The symptom of neuralgia is pain. This is sharp, sudden, shooting along the nerve, and transient, being succeeded by an interval of freedom. The pain is often accompanied by numbness, tingling, or burning in the surface to which the sensory nerve goes ; and after a time it is followed by a tender sensitiveness of this part and also of the entire nerve trunk. Pressure on the nerve elicits un- usual tenderness and starts an attack of pain. Sometimes the pain is so severe as to cause reflex or automatic spasms in muscles whose motor centres are in close relation to the sensory root involved. Thus tri- geminal neuralgia often causes a twitching of the eyes and face ; inter- costal neuralgia, a bending of the entire trunk ; sciatica, a drawing up of the leg. Any movement, especially if sudden, is liable to start a paroxysm of pain, hence patients instinctively keep quiet, move cau- tiously, and avoid effort. Sometimes vasomotor and trophic distur- bances attend or follow a neuralgic attack. Such are flushing of the surface, unusual sweating, or herpetic eruptions on the skin, or falling of the hair. A distinct, unusual pulsation in the vessels of the pain- Pathology. 731 ful region or nerve can often be felt. A slight swelling of the pain- ful region may also occur. Herpes zoster is a frequent accompaniment of neuralgia in any nerve. It sometimes occurs without neuralgia but is always limited in its ex- tent to the domain of one or more nerves, as Head has demonstrated.^ It is particularly frequent in connection with neuralgia of the thoracic region. It may precede or it may follow an attack of neuralgia, or it may occur as the only symptom of the affection of the ganglia. The course of tlie disease is very variable. The pain occurs In par- oxysms and these vary in severity and duration. The pain may come every few seconds, lasting several seconds, for many hours. Such an attack may recur regularly every day at a definite hour. It often subsides at night, though this is not uniformly the case, and in some patients the pain is worse at night. Attacks rarely continue for more than three or four days when they are succeeded by a free interval of several days, weeks, or even months. Some women suffer from neu- ralgia with each menstrual period. The tendency to a recurrence after one attack is well known, and almost everyone is conscious of some nerve in his body of little resis- tance, liable to give him pain when he is ill. A person who has such a tender nerve rarely suffers from neuralgia in other nerves, and neu- ralgia is rarely bilateral. Occasionally it develops in different nerves at different times. If there is discovered a cause which can be re- moved, which is rarely the case, treatment of a rational nature can be instituted. But in the majority of cases palliative remedies have to be employed. When herpes occurs, with or without neuralgia, the dura- tion of the attack is longer. The vesicles remain for at least two weeks, often occur in successive crops and sometimes persist for two months. They may leave permanent scars. Pathology. — As fatal cases are rare no autopsies are on record. But surgeons have cut out both nerves and ganglia for the relief of neuralgia, and from the examination of these much information has been obtained concerning the pathology of the disease. All the various processes of neuritis, with degeneration, and atrophy in the nerve fibres have been seen. But the more important lesions appear to be in the neurone bodies which lie in the ganglia. These neurone bodies have been found in different stages of degeneration, with chromatolysis, vacuolization, and atrophy. Some neurones stain only faintly and are most irregular in outline, being so shrunken as to leave the space in which they lie almost empty. Some neurones are very small, without nucleus or necleolus. The fibres within the ganglion are often swollen, the medullary sheath is disintegrated, the axone is granular. In other fibres the sheath is full of a disintegrated mass in which the axone cannot be distinguished. In still others the sheath is empty. These changes are shown in Plate III. The connective tissue about the neurone bodies and also about the fibres has been found to be increased in density. The small bloodvessels in the ganglion are sometimes 1 Brain, 1895. 732 NEUBALQTA. closed by the proliferating tissue in their walls, and all forms of atheroma and sclerosis have been seen in the larger vessels. These changes are shown in Plate XXVIII., taken from Keen and Spiller.' Other authors have found marked thickening of the walls of the bloodvessels which lie in the endoneurium of the affected nerves, and this thickening may be present both in the media and intima. In many cases true atheromatous degeneration of the smaller arteries has been found. Similar changes have been found by Thomas in the ganglia on the posterior nerve roots in the early stage of tabes (see Chapter XIX.), and that disease begins with neuralgic pains. In cases of neuralgia attended by herpes, hemorrhages have been found in the ganglia. It is, therefore, probable that neuralgia has, in every chronic case, some organic lesion. While these changes are characteristic of chronic and long-standing cases of neuralgia, especially in cases of trigeminal neuralgia, it is not to be thought that they are present in the acute and transient cases. They are, however, indications of a long-continued malnutrition, and hence, we may conclude that in the acute cases some temporary malnu- trition of the sensory neurones is present. Treatment. — The causal treatment of neuralgia consists in elimi- nating all factors capable of producing the disease. These have been mentioned in discussing the etiology. Everything which tends to build up the strength and health of the patient ; a life with interests but without anxiety ; a healthful regimen with proper diet and exer- cise ; daily baths, either cold or hot, followed by cold sponging ; regu- lation of the digestive functions and of the bowels ; washing the kid- neys out by drinking an excess of water ; and adding to the diet those tonic medicines which occasionally strengthen the nervous system, such as cod-liver oil, quinine, strychnine, iron, arsenic, and the glycero- phosphates will eventually help to cure neuralgia. When any sus- picion of infection arises, especially when malaria is detected, a mer- curial purge followed by quinine in large doses (20 grains), or by Warburg's tincture (5ij) (which I consider superior to quinine in many types of neuralgia), will give relief. The quinine or Warburg's extract should be given in large doses four, and again three, hours be- fore the attack of neuralgia is due. In the malarial type a distinct periodicity in the attack is so common that this time can easily be reckoned. A small dose of the drug may be used between the attacks at intervals of six hours. When syphilis is suspected as a cause (and in such cases the attack usually occurs in the afternoon), mercurials, combined with large doses of iodide of potash, are indicated. In many cases of obstinate neuralgia climatic conditions must be considered, and very often a change of air will be of great benefit. A sojourn at the seashore, or in the mountains, according to the ex- perience of the patient as to the benefit previously derived from travel, will often appear to start a cure. The regime of a sanitarium, or ^Keen and Spiller. "On Resection of the Gasserian Ganglion," American Journal of the Medical Sciences, November, 1898. DESCRIPTION OF PLATE XXIX. Fig. 1. — Portion of the Gasserian ganglion at the entrance of the third branch of the trifacial nerve. The medullary sheaths are most irregularly swollen, and at the right of the field empty nerve sheaths are seen (method of Azoulay). Fig. 2. — Portion of the second branch of the trigeminal nerve near the Gasserian ganglion. The axis cylinders have entirely disappeared, and the medullary sheaths are greatly swollen. In many places the medullary substance of two or more nerve fibres has united into irregularly shaped masses (osmic acid stain). Fig. 3. — One of the nerve bundles within the Gasserian ganglion. Numerous swollen and irregularly formed axis cylinders may be seen. In most portions of the field these appear as drops of a red, hyaline-like substance, but in one portion an axis cylinder of considerable length may be seen. Fig. 4. — Bloodvessels from the Gasserian ganglion. The walls are greatly thickened, and the lumen of the large vessel has been almost entirely obhterated. In one place the innermost layers of the vessel have contracted from the outer during the process of hardening. Smaller vessels in the upper part of the field are entirely closed. Fig. 5.^A nerve bundle of the trigeminus close to the Gasserian ganglion. Only a few nerve fibres are present, and everywhere an abundance of connective tissue is seen. Three much swollen medullary sheaths are in the field. PLATE XXIX Fig^ 1. Fig. 2. .i4v. „>-- Fig. 3. Fig. 4. Fig. 5. ^ # TRIGEMINAL NEURALGIA. 733 water-cure, as well as the change of air, change of habits and sur- roundings, '^nd freedom from work or home care, are of great benefit to many patients. And if this can be combined with foreign travel by sending patients to some health resort abroad, much relief will be obtained. The dry climate of the Nile, the rarefied air of the Enga- dine, or high Swiss valleys, or Colorado, are very beneficial. The treatment of herpes zoster consists in making applications of zinc ointment in the early stage and protecting the surface by linen bandages. Collodion may be applied in the later stage when there is no longer any liquid secretion in the vesicles. If the vesicles fill with pus they should be punctured and washed with a solution of corrosive sublimate 1 to 5,000. The palliative treatment of neuralgia will be considered in discussing the special forms of the disease. SPECIAL FORMS OF NEURALGIA. Trigeminal Neuralgia. — Neuralgia of the fifth nerve. Tic dou- loureux. This is the most common form of neuralgia. Etiology. — Women are more subject to trigeminal neuralgia than men. It is a disease which is very uncommon under the age of twenty years, the majority of cases occurring between the ages of twenty-five and fifty years, but no age is exempt, and as the disease is a chronic one, the patients may suffer from it until the close of a long life. I have seen it in patients over eighty years of age. The disease is dis- tinctly an hereditary one, many patients admitting that their parents or grandparents have suffered from it. It develops more commonly in winter than in the summer, which gives support to the theory that the majority of cases are due to exposure to cold. Neurasthenic indi- viduals and those who suffer from anaemia, chlorosis, and gout, or diabetes, are more subject to the disease than others. Head^ has denied this very strongly, but my observations do not support his statements. Slight attacks of trigeminal neuralgia may occur in the course of any slight illness, such as a disorder of the stomach, an acute intestinal infection, chronic constipation, congestion of the kidneys, a bad coryza, or angina, or an attack of grippe. In many gouty or rheumatic indi- viduals it is a frequently recurring symptom of the general disorder. Some persons feel a sudden neuralgic pain in the trigeminal nerve while eating ice-cream or drinking very cold drinks. Attacks of neu- ralgia are not infrequent in connection with migraine. In many per- sons who are affected with astigmatism, or defective accommodation, neuralgia of the trigeminal nerve may follow any strain of the eyes. Ear disease is also said to have caused it (Moos). Such attacks are extremely temporary and need no special treatment further than the treatment of the causal condition, of which they are the effect. But trigeminal neuralgia may become a more serious and troublesome ^ Allbutt's System of Medicine, vol. vi., p. 732. 734 NEURALGIA. disease, and such cases require careful study. In chronic lead poison- ing it appears to be a not infrequent symptom. Many cases can be traced to an attack of some one of the infectious diseases, of which malaria, grippe, and typhoid are the most common. In this country when a patient has once had malaria, it is not uncommon for subsequent attacks of this disease to manifest them- selves exclusively by attacks of trigeminal neuralgia. Usually such attacks are daily in occurrence, at a definite time of the day, and last for a few hours and disappear as suddenly as they came on. Organic disease of any kind upon the base of the brain, or in the course of the trigeminal nerve, is capable of producing trigeminal neuritis, with a consequent neuralgia. The syphilitic affections of the base of the brain, also tuberculous affections, tumors of the brain, or fractures of the base of the skull, and aneurisms of the internal carotid artery, or of the circle of Willis, bullet wounds of the base of the skull, any disease of the bones of the head and face and catarrhal affections of the nose and throat, dental caries, and any functional dis- turbance of the eye or ear, are all capable of setting up a neuritis in contiguous nerves and producing either local or trigeminal neuralgia. Symptoms. — The chief symptom of trigeminal neuralgia is intense pain in the course of the nerve and upon the face, usually deep, some- times on the surface. This pain occurs in sharp paroxysms which are repeated frequently, a series of sharp, shooting pains being followed by an interval of relief. Pain is so intense as to be agonizing. It is frequently a burning pain. It makes the patient cringe, it causes a flow of tears, and in very many cases a sharp, quick contraction of the muscles of the face, a closing of the eye and drawing up of the mouth. The patient may even turn the head downward with pain. Changes in the condition of the atmosphere, an increase of the humidity or variations of barometric pressure or of temperature may excite an attack of pain. Some patients have annual attacks during the winter months and are free during warm weather. Any draught upon the face or exposure to cold, any movement of the muscles of the face, the acts of whistling, of talking, of chewing, of swallowing, are capable of arousing the pain, and the patient will very often starve rather than incur the pain of eating. Mental anxiety or intellectual efforts seem capable of starting up an attack. Pain shoots out into the entire dis- tribution of the trunk of the nerve which is affected. In some cases only one trunk is involved, in other cases two trunks, in most severe cases all three trunks. The most common form of neuralgia is the supraorbital, in which the pain is felt above the eye and at the notch or foramen through which the supraorbital nerve makes its exit upon the forehead, and over the forehead and in the hair as high as the ver- tex. Sometimes the pain radiates into the eyeball, and occasionally pain in the eyeball is the only manifestation of neuralgia of the supra- orbital nerve. When the infraorbital branch is the one which is affected, pain is felt upon the cheek and in the upper teeth, and espe- cially in the antrum and malar bone. It may then go as far out as TRIGEMINAL NEURALGIA, 735 the temple and the lobe of the ear. When the third or lower branch of the nerv^ is affected, the pain is felt upon the cheek and lip and in the lower teeth, also within the mouth, even in the tongue. I have seen cases in which the neuralgia was limited to the tongue, pain being felt chiefly in the side and lower surface of the organ, being much inten- sified by the act of swallowing, or talking, or chewing. The pain usually extends in chronic cases from the branch of the nerve first affected into the other branches, so that eventually the entire face is the seat of pain. In one patient a touch along the lower part of the cheek on the right side caused intense pain in the right half of the tongue, and there was also a tender spot over the ramus of the jaw just in front of the ear, but no anaesthesia or loss of taste. A very hypersensitive condition of the skin in the domain of the branch affected is quite frequent. There is a constant sense of tingling, and an unusual appreciation of little variations of temperature ; and a slight touch with the fingers, or with cotton-wool is disagreeable and painful. For this reason patients very often instinctively protect the affected part by applications of woollen cloths in order to keep the part at an even temperature and to avoid sudden and unexpected contacts. The face does not often show any change in color, but occasionally it is a little red and the conjunctiva and mucous membrane of the mouth and nose may be congested. Sometimes an increased pulsation of the bloodvessels accompanies the attack of neuralgia and the arteries may be felt to be fuller than usual. It is to be recollected that the most common lesion found in neuralgia is an arterial sclerosis, which may account for the thickening of the arteries that is felt. Sometimes the attack is accompanied by a secretion of tears or of saliva. Trophic changes in the skin of the face have occasionally been observed, the most frequent of which is the appearance of herpes zoster. Some authors have spoken of an unusual grayness of hair and of falling of the beard, but this I have never seen. Although the fifth nerve conveys the sensation of taste, this is not affected in any way in trigeminal neuralgia — a fact that proves that the taste fibres are independent of those of the fifth nerve proper. The nerve trunks are extremely tender during an attack of neuralgia at their exits upon the face, namely, over the eye, upon the malar bone, and upon the chin. Any pressure at these points produces very great increase of the pain during an attack. It must be remembered, however, that these points are normally sensitive to pressure. In Plate IV the distribution of the various branches of the fifth nerve in the face is shown. Course. — The course of the disease varies very much in different cases. Usually a neuralgia begins with a sudden attack, which lasts for several days and then passes off under the use of remedies. Such an attack may recur from time to time, and in the severer type the attacks become more and more frequent, so that the patients who have had l>ut one attack in one or two years, after a few years may be hav- ing an attack every week. In the most severe type of case the pain 736 NEURALGIA. comes on every few minutes, day and night, lasting at times for several weeks in this manner, and is quieted only by the strongest opiates, to recur as soon as the effect of the remedy has worn oflP. It is in the severer type of case that surgical interference is willingly permitted. In any case of facial neuralgia one attack predisposes to another, and patients who have once had an attack appear to be subject to the dis- ease for the rest of their lives. The intervals, however, may be so long (several years) and the attacks may yield so readily to a sympto- matic or causal treatment as to preclude any idea of the division of the nerve. I have several patients who never go through the winter without a severe attack. One such patient, who had been a sufferer for many years, escaped while spending the winter on the Nile. In many cases a warm winter climate is advisable. The general dele- terious effect of severe pain upon the constitution and nutrition of the patient is often demonstrated in cases of trigeminal neuralgia. The sleep is poor, the assimilation of food is impaired, even where the patients are not prevented by pain from eating ; disorders of digestion, especially constij)ation, are common, and a state of mental disquiet, apprehension, and hopelessness may develop ; in fact, some cases have gone on to melancholia and suicide. Diagnosis. — The diagnosis of trigeminal neuralgia is very simple; the only question that can arise is whether the neuralgia is based upon an actual neuritis, or is a purely functional disease. The existence of continuous pain, the paroxysms alternating with remissions rather than intermissions, continuous tenderness in the course of the nerves, the development of anaesthesia in the skin of the face, and the determina- tion of some organic disease as a cause, such as syphilis, tumors, etc., mentioned in the section of etiology, make the diagnosis of neuritis rather than neuralgia probable. The facts which are presented in the chapter upon referred pain should not be overlooked in making the diagnosis, and neuralgia should never be confounded with migraine. The pain in migraine is felt in the temple and side of the head, and is not located in the course of the branches of the fifth nerve. Treatment. — The treatment must first be causal; if an organic affection can be ascertained as a basis, it must be treated and removed. In malarial cases and in any case that is of uncertain cause, quinine should be tried. It is well to precede this by a mercurial purge — calomel yL grain every hour until 1 grain is taken — and to give the quinine in a dose of 20 to 30 grains, which should be administered within an hour and about three hours before the paroxysm of pain is due. Thus the maximum effect of the quinine, which is felt two hours after its ingestion, will coincide with the time of the paroxysm and will prevent its occurrence. While caries of the teeth is an occasional cause of neuralgia, and the condition of the teeth should always be investigated, as the pain may not be felt in the region of the infected tooth, yet in my experience this cause is an infrequent one, and many patients have had all the teeth removed, on the supposition that they were the cause of the affection, without any relief whatever. The so- TRIGEMINAL NEURALGIA. 737 called Riggs' disease, a dental exostosis to which great importance has been ascribed, is not a common cause of neuralgia, and its treatment is rarely, if ever, followed by relief of the pain, if this is truly neuralgic. In all conditions of trigeminal neuralgia the general health of the patient must be considered as well as the symptomatic treatment. These patients, as a rule, are anaemic, and are rapidly run down by the starvation that is consequent upon the pain of the act of chewing. It is especially important that every possible means should be used to build up the general health. They should be well fed by means of fluid food, reinforced by nutritive eneraata in case solid food cannot be taken. Alcohol, cod-liver oil, beef marrow or medullary glyceride, glycero-phosphate of soda, hypophosphites, arsenic, iron, and strych- nine should be used freely as general tonics, and the nutrition generally improved by daily baths and douches and by massage. A change of air is often the one thing that will make the symptomatic remedies efficacious, and the important thing is to secure a change from sea to mountains, or mountains to sea, according to the place where the patient is accustomed to live. In some persons the damp of the sea air tends to produce a return of the neuralgia, and in these persons a dry climate should be sought. A warm climate is essential, and one free from dampness. Egypt, lower Florida, southern California, are all excellent. There are no special baths or health resorts that claim to be efficacious in the treatment of neuralgia, but the regime secured in a foreign water-cure is sometimes of benefit. Remedies for the pain are very numerous. The most important are the newer analgesics, namely, phenacetin, 10 grains to 15 grains every two hours ; acetanilid, 5 grains every two hours ; antipyrine, 1 5 grains every two hours, or salophen, 15 grains every two hours. It is well to combine with each dose of these drugs a grain of caffeine and one- hundredth of a grain of strychnine to avoid their depressing action upon the heart. A hot alcoholic drink sometimes gives immediate relief. Quinine may be used with it. Aconitine is the best remedy. It is given in pill or tablet form, the French pills of Chapoteaux being the best preparation. Each pill contains g-i-g- grain {^ milligramme), and the remedy must be used with sufficient frequency to produce constitutional effects. It is iny rule to begin with one pill every four hours and decrease the intervals each day one-half hour until one pill every two hours is being given, or until constitutional effects are evident, namely, tingling of the tongue and fingers, sense of general weakness and feebleness of the pulse. It is well to combine with this a fiftieth of a grain of strych- nine during the first two days and then yo'w ^^ ^ grain of strychnine, when the aconitine is being given every two hours. If the strychniue does not agree with the patient, or produces twitchings, the dose of this may be reduced, or caffeine two grains, or spartein -gL- grain may be given in its place. Some heart stimulant is to be used in connec- tion with the aconitine, when the larger doses are given and the pa- tient should be warned against making any sudden muscular efforts, 47 733 NEURALGIA. and during the week or ten days of such treatment should walk very little. Gelsemium is the remedy next in favor, and this is to be begun in small dose, 10 drops of the tincture, or 10 drops of the fluid extract, being given every three hours, and the dose increased by 1 drop each time until the patient perceives physiological effects, which are a heaviness of the upper eyelids and a difficulty in opening the eyes. In one obstinate case 30 drops of the fluid extract every three hours uniformly cured the attack. This remedy may be kept up at the point wliich produces this physiological effect for several days and very often gives marked relief. In patients of my own this remedy has succeeded when aconitine had failed completely. The tincture of colchicum has been used with considerable benefit, especially in the gouty cases. The wine of colchicum may be given in 5- drop doses every two or three hours until a purgative action is produced, and then the dose may be decreased in amount, or the frequency of the dose may be lessened. The effect can be continued for several days. Butyl chloral hydrate in 5-grain doses every hour for 4 doses has been highly recommended as a remedy for neuralgia of the fifth nerve. Preparations of arsenic ; especially the cacodylate of sodium, ^ to :^ gr., three times a day, are of considerable service. They may be used in conjunction with the remedies already mentioned, or alone, great care being taken to avoid toxic effects. The most certain and satisfactory remedy is opium or morphine. This may be given in the form of extract of opium, or the tincture of opium in increasing doses, by the stomach, or morphine may be used hypodermically. In the majority of cases of trigeminal neuralgia it will eventually be used, although if possible patients should be pre- vented from resorting to it too soon, or too freely, as there is no disease in which the morphine habit is a more common sequel than trigeminal neuralgia. Yet in the paroxysms of pain it is imperative to give relief, and the mere fear of engendering the habit should not prevent one from using it in appropriate cases, especially in the early stage of the disease and until other remedies can have the time to exert a constitutional effect. Strychnine has been used by Dana hypodermically in large doses for the relief of various forms of neuralgia, a dose being begun of ^ of a grain and increased as high as -jig- of a grain hypodermically once in twenty-four hours. I have seldom seen any benefit result from this treatment, but I have seen severe strychnine poisoning ensue. In individuals who are the subject of arterial sclerosis nitro-glycerin is a remedy of considerable efficacy, which may be used in -^^ or yJ-^ of a grain and repeated with sufficient frequency to produce a percep- tible effect upon the arterial tension. Nitrite of sodium in 1- or 2- ,grain doses is equally efficacious. The extract of cannabis indica has some reputation in the treatment of neuralgia. It is to be given in tablet or pill form, ^ of a grain at a dose several times a day. TRIGEMINAL NEURALGIA. 739 Preparations of the bromides, and bromide combined with chloral may be oi some service in the lighter forms of neuralgia, but in the severe attacks do not have sufficient effect to quiet the pain. If a small amount of morphine be added to full doses of these two drugs a use- ful remedy is obtained. Local applications to the face sometimes give considerable relief. Applications of ice bags or freezing the face by a spray of chloride of ethyl applied to the point of exit of the branch of the nerve affected are sometimes of considerable service. The majority of patients, how- ever, prefer hot applications ; and a hot-water bag or a poultice or an application of cotton covered with oiled silk, outside of which a hot bag or a Japanese hand furnace can be placed, will often give consid- erable relief. Most of these patients prefer to protect the face by some woollen substance and thus avoid exposure to cold or to changes of temperature. Sometimes local applications of lotions and salves are of service. Weak chloroform liniment, evaporating lotions of opium and lead, or ointments containing aconite, morphine, atropine, and veratrine, have been used with some success. Rubbing the face with a menthol pencil may give relief. The face may be painted with coUodium in which iodoform, 1 to 15 grains, has been dissolved, a thick coating being ap- plied over the exit of the branch of the nerve affected. Camphor and chloral rubbed together into a paste and applied has been of benefit. Inhalations of chloroform may be given with good effect, but this, of course, must be used with great caution. Electricity in the form of galvanism, a mild, continuous current, with the positive pole upon the painful point and the negative on the back, has been recommended. The application should be five minutes in duration over each branch of the nerve and great care should be taken not to make or break the current during the application, it being be- gun very gradually by the aid of a rheostat. Sometimes a severe ver- tigo may be produced by applications of galvanism to the head and face. I have never seen any good result from this method of treat- ment. Massage of the face is usually very painful in conditions of neuralgia and has never in my experience been of much service in the treatment of the attacks. Vibrations maintained by a tuning-fork electrically vibrated, the end of the fork being in contact with the affected branch of the nerve, and vibrations with the end of the fingers of an expert masseur, have given relief in some cases. The actual cautery cannot be applied to the face on account of the scars which remain, and hence is not used in trigeminal neuralgia. Surgical Treatment. — For many years division of individual branches of the nerve has been practised for the relief of neuralgia, the nerve being merely divided or being divided and the central end stretched, or a considerable section of the nerve being removed. To reach the peripheral parts of the supraorbital or infraorbital nerves, semilunar incisions of the eyebrow or cheek are made in the natural 740 NEURALGIA. lines of the skin, and chiselling out of the orbital bones gives access to the nerve. Division of the third branch of the nerve within the mouth, as it passes into the ramus of the jaw, is a comparatively simple operation within the reach of almost any surgeon. The deeper opera- tions of division of these various roots at their exit from the skull re- quires much greater surgical skill and is very complicated and bloody. Recently surgeons have resorted to the injection of a IJ per cent, solution of osmic acid for the relief of mild cases of neuralgia limited in extent to one branch of the nerve. Two minims of the solution may be injected slowly through a very fine needle into the sheath of the nerve after it has been exposed as described. The osmic acid de- stroys the nerve fibre by dissolving its medullary sheath. This opera- tion may be performed under cocaine, but the injection of osmic acid causes severe pain, and therefore ether anaesthesia is preferable. I have known of two cases cured by this method. Another method of treatment is by the injection of 2 c.c. of a 95 per cent, solution of alcohol containing 4 grains of cocaine to the ounce into the branch of the nerve affected. This must be done at its deepest accessible point just at its exit from the skull by means of a stylet, 10 cm. long and 15 mm. in diameter. At the moment of in- jection severe pain is felt in the nerve and subsequently its area of the skin is anaesthetic for months. Success has been reported by many, ^ but is not very lasting as the pain is likely to recur after a year or less. Many unfavorable complications, such as severe cellulitis of the face and hard indurations, also perforation of the Eustachian tube and per- manent deafness, have occurred after such injections, and fatal results have been recorded. It is a method to be employed only by one who is expert. While such operations may give relief for some months or years, they are not positively curative, but they may be performed when the case is a chronic one and the neuralgia is limited to a single branch of the nerve. In the majority of cases the neuralgia eventually extends to other branches, and in such cases the more radical operation advised by Krause and Hartley simultaneously, of exsection of the Gasserian ganglion within the skull, may be performed. This operation is car- ried out by making a horseshoe-shaped incision through the skin, periosteum, and temporal and parietal bones, and laying down a flap which should be at least four inches in diameter, the base being half an inch above the base of the skull. The dura is not incised, but with the brain is then carefully lifted away from the base of the skull. The point of exit of the fifth nerve is determined, great care being taken to avoid injuring the optic and oculomotor nerves ; the free branches of the nerve are seized, the Gasserian ganglion is pulled outward through the dura, and the trunk of the nerve divided behind the gan- glion. Traction on the nerves is made and they are divided as far as possible from the ganglion, which is thus removed with a half-inch of the nerves. The result of this operation is to deprive the face entirely of sensation, and the operation is always followed by a permanent con- * Patrick. Jour, Amer. Med. Assoc, Dec. 11, 1909, gives references and technique. BRACHIAL NEUBALGIA. 741 dition of tmgling and numbness, with anaesthesia of the face, and loss of taste on one side of the tongue ; but is usually gives permanent relief from the neuralgia. The agonizing pain is so great that these patients are willing to submit to any operation that promises a cure. I have seen a large number of permanent cures in patients of my own upon whom Hartley has performed this operation, and Keen, Krause, Horsley and others have published many cases of cure. Recently, Frazer and Keen have obtained good results by dividing the central end of the nerve just behind the Gasserian ganglion, without removing the ganglion. Oervico-occipital Neuralgia. — Neuralgia of the posterior branches of the cervical plexus and especially of the great occipital nerve, which passes up the back of the neck and over the back of head to the vertex, is occasionally met with, though it is by no means as common as trigeminal neuralgia. Pain is felt along the course of the nerves in the neck, but particularly below the occiput and in the vertex. There are painful spots near the exit of the nerve at the base of the skull, over the occiput, and upon the vertex. The scalp is very likely to be tender when the pain is intense. Frequently the pain is bilateral, and very often is continuous rather than paroxysmal, though sharp attacks of pain may follow any motion of the head. The patients usually hold the head in a fixed position to avoid the pain produced by movement. Sometimes a swelling of the cervical glands accompanies the neuralgia. In one case reported by Johnson, the superior ganglion and its cord were fixed by adhesions which were freed by operation, after which the patient was relieved of the neuralgia. Occasionally an attack of trigeminal neuralgia is associated with the cervical neuralgia. This may be due to the central radiation of irritation, which is erroneously referred, or it may be due to a common cause producing both affec- tions. The characteristics of the disease are similar to those of trigem- inal neuralgia and the treatment is that of neuralgia in general. In one very obstinate case under my care, McBurney divided the great occipital nerve at its exit from the muscle. This had only a tempo- rary effect and it was several months before a spontaneous recovery ensued. Brachial Neuralgia. — The various nerves of the brachial plexus are occasionally the seat of neuralgia. The pain may be felt chiefly in and about the plexus in the neck, but usually shoots outward into the branches down the arm and into the forearm and hand. There are painful points where the nerves are superficial and can be easily com- pressed against the bones. Thus in the axilla, over the circumflex, near the deltoid, over the musculospiral as it curves about the humerus, over the ulnar at the elbow, and over the nerves at the wrist, pressure causes pain. The pain is made worse by any movements of the arm, and the patient instinctively keeps it quiet. The pain is usually worse at night, probably because of pressure exerted during sleep, and hence sleep is often interfered with. There is often much hypersensitiveness of the skin and a burning pain, which Weir Mitchell has named "caus- algia." Herpes zoster is a frequent complicating symptom, and the 742 NEVBALGIA. position of the vesicles corresponds exactly with the distribution of the cutaneous branches of the nerves. Glossy skin and trophic changes in the nails appear only when a true neuritis has developed. Intercostal Neuralgia. — While the intercostal nerves from their protected situation are usually exempt from injuries and wounds^ ex- cepting in cases of stab wounds of the back or chest, they are fre- quently the seat of neuralgia. The neuralgic pain is sharp and shoot- ing around the side of the chest and is often felt chiefly in the terminal filaments of the nerve in front or at its middle branch in the line of the axilla. At these two points of exit of the branches and also at the point of exit of the posterior branch near to the spine, points of tender- ness are usually present. If the upper dorsal nerves are affected the pain may also be felt in the inner side of the arm and may be attended by tachycardia. The pain is increased by breathing, by coughing, or by any movement of the chest. A dull, aching, pressing pain is felt be- tween the attacks, and patients usually like to support the side and compress it. Others cannot endure the touch of their clothing. In- tercostal neuralgia is very often followed by an attack of herpes zoster. The cases of Kaposi have proved that when herpes occurs the disease is sometimes due to a hemorrhage within the posterior spinal ganglion of the nerve affected. In other cases, a parenchymatous and intersti- tial neuritis have been found. The herpes may precede the neuralgia and the latter — especially in old people — may persist long after the eruption has subsided. Any of the ordinary causes of neuralgia may produce intercostal neuralgia. Intercostal neuralgia is frequently due to a secondary im- plication of the nerve from disease, either in the bones of the spine or chest, or in infectious conditions of the pleura. Thus empyema is occasionally complicated by intercostal neuralgia. It has occurred from pressure by aneurisms. Its relative frequency in women suggests that the same causes that produce mastodynia {q. v.^, a variety of intercostal neuralgia, may also cause the disease. Diagnosis. — The diagnosis of intercostal neuralgia is made from the exceeding sharp, shooting character of the pain, and by the existence of painful points upon the chest at the exit of the nerves. It may suggest angina pectoris when tachycardia occurs, but the course of the two diseases differs so widely that a diagnosis is easily reached. Pleurodynia, which is a muscular rheumatism of the intercostal mus- cles, or may be due to a rupture of muscular fibres after coughing, pro- duces a somewhat similar pain, increased by respiration ; but in this case the tenderness is not felt especially at the point of exit of the nerve. In pleurisy the pain is less exactly limited in its distribu- tion, and the physical signs of the disease enable a diagnosis to be reached. The fact that disease of the viscera may give rise to pain in the chest, as described in the section on referred pains, is not to be forgotten (see page 752). Treatment. — The treatment is by local applications along the course of the nerve ; blisters or the actual cautery over the exit of the spinal branches, and special measures such as are described in the treatment WMBO-ABt)OMmAL NEtJ&ALGlA. 743 of neuralgia in general. Edinger recommends a spray of chlorsethyl. For the r^ief of the herpes, applications of oxide of zinc ointment may be made, or the herpetic vesicles may be painted with collodion, to which a small amount (1 per cent.) of carbolic acid or thymol has been added. Care should be taken to avoid any septic infection of the exposed skin when the vesicles have ruptured. In old persons opiates should be very carefully given. Neuralgia of the Breast — Mastodynia. — This form of neuralgia is very rare and occurs chiefly in women. It may develop during pregnancy and is especially frequent during the last weeks. It occa- sionally occurs during lactation and may be so severe as to interfere greatly with nursing. It sometimes develops in women during the menstrual period, or in the course of uterine or ovarian disease. In one of my cases the attacks lasted three days at each monthly period for several years, requiring the use of morphine at the time. It also occurs as a direct result of carcinoma and other tumors of the breast ; but occasionally it occurs as a result of anaemic or neurasthenic states without concurrent afiection of the genital organs. In fact, any one of the general causes of neuralgia may be capable of producing mastodynia. The pain is usually located deep in the gland, is very severe, and occurs in paroxysms, but there is usually a dull, heavy feeling or aching pain all the time. Sometimes the pain is superficial, and the skin of the breast, especially about the nipple, is exquisitely sensitive ; the nipple may be congested and the entire breast red and swollen. Erb has seen a case in which the pain excited the function of the gland. There are tender points along the spinous processes of the second to the sixth dorsal vertebrse. The course of the case is a slow one. Sometimes the pain persists in spite of treatment until preg- nancy is over, or until lactation ceases. In addition to general measures of treatment for neuralgia already described, some relief attends the adjustment of a support to the breast, or a firm bandage to the breast. Local applications of cocaine in 4 per cent, solution to the nipple sometimes quiets the pain. Ointment of belladonna and aconite or evaporating solution of lead and opium may be of benefit. Nageli has afforded relief by stretching the nerve, the entire breast being firmly grasped in both hands and slowly but steadily lifted for twenty or thirty seconds several times a day. This is a simple method which should be tried in every case. Lumbo-abdominal Neuralgia. — The lower dorsal nerves and the lumbar nerves send branches over the back and abdomen. These branches are occasionally the seat of neuralgia, which resembles inter- costal neuralgia, to which it is strictly homologous. The pain shoots about the side of the body and is at times intense and often burning in character. There are tender points at the posterior, lateral, and anterior exits of the branches of the nerves. Neuralgia in this locality is especially likely to be attended by herpes. I have seen a case in which abdominal neuralgia followed intercostal neuralgia, both being attended by herpes. In this case, as in many others, the course of the 744 NEUBALGIA. case was slow, the pain lasting several weeks, and resisting all forms of treatment. The patient was a middle-aged woman, the subject of chronic arterial changes and cirrhosis of the kidneys. The pain in lumbo-abdominal neuralgia is often dull and continuous. It fre- quently extends downward into the groin and is felt in the penis or labium, on which parts herpes appears. This condition is not to be confounded with neuralgia of the testicles and ovaries, which is an affection of the sympathetic system supplying these organs. There are no special therapeutic indications in this form of neuralgia. Crural Neuralgia. — The anterior crural nerve, which extends down the inner surface of the thigh and leg and reaches the ankle, is occasionally the seat of neuralgia. The condition may develop in the course of sciatica. It is more common in men than in women, and usually develops after hard muscular work or long marches, exposure to cold, or injuries. It is to be remembered that pain in this nerve is often the first sign of caries of the spine. It is said that in diabetes crural neuralgia and sciatica are the most frequent forms of neuralgia produced. The most obstinate case of the disease which I have seen was in a diabetic patient. Painful points are found in the inguinal canal, on the front of the thigh, on the inner side of the knee, and on the inner malleolus. Herpes frequently appears. This form is not to be mistaken for the reflex pain often felt by women at the menstrual period or with ovarian disease in this locality. The treat- ment is the same as that for sciatica. Painful Knee. — Haffa^ has called attention to the causes of pain in the knee not due to crural neuralgia. They are (1) arthritic muscu- lar atrophy. Inactivity of the hip joint or knee joint from any cause may lead to an atrophy of the quadriceps femoris. This muscle holds the capsule of the knee joint tense and if it is relaxed the capsule may get between the patella and the condyle of the femur or between the condyles of femur and tibia, causing pain on walking. It is to be cured by rest in bed with massage of the muscle and its constant exer- cise by electricity till the atrophy is removed. (2) A second cause of pain in the knee is the dislocation of a meniscus in the joint. This follows slight injuries and causes a sudden pain and a flexion of the leg with rotation away from the injured meniscus, the hard edge of which can be felt and is tender. This needs operative treatment. (3) A third condition causing a painful knee is a mass of fat in the joint between the condyles growing from a papilla in the capsule. This causes a swelling and must be removed. Sciatica. — Sciatica is neuralgia of the sciatic nerve. It is a very conunon affection, more frequently met with in males than in females, and in adults than in persons under the age of twenty years. Persons between the age of forty and fifty years are most liable to the disease. Etiology. — Gout and rheumatism are the chief causes of sciatica. It can often be directly traced to exposure to cold. It is particularly frequent in diabetic patients. It may follow any of the infectious diseases or may be due to alcoholism. The poisonous agents which ^ Berliner klin. Wochen., 1904 ; Nos. 1 and 2. SCIATICA. 745 cause multiple neuritis may cause sciatica. Direct pressure upon the sciatic nerve by sitting in an uneasy position or in hard chairs with sharp edges, or pressure exerted from causes acting within the pelvis, such as accumulations within the rectum, pelvic inflammation, or tumors of the uterus or ovaries, or the condition of pregnancy may cause sciatica. Disease of the sacral or thigh bones may produce sci- atica. In one case under my care a long standing sciatica was finally explained by the development of an osteosarcoma of the pelvis. In- juries are a frequent cause of sciatica, as in falls upon the buttocks and fractures of the thigh. Lifting heavy weights has brought on sciatica. Workers who stand or move the legs at machines are particularly liable. The sciatic nerve is well supplied with bloodvessels, and it is possible that a venous congestion from pressure is an exciting cause of disturbance of function in the nerve in these cases. Sciatica often follows hemorrhoids and varicose veins in the leg. It is difficult to separate sharply sciatica from sciatic neuritis, and in many cases where the disease lasts a considerable length of time, it is reasonable to suppose that a neuritis is present. In fact, in all the cases in which an autopsy has been obtained, an interstitial neuritis with congestion of the vessels, hemorrhages in the sheath, and secondary degenerations in the nerve fibers have been found. Many cases are so short in their duration, the symptoms are so distinctly intermittent, and the cessation of the pain is so instantaneous either upon change of position or upon local applications, as to make it improbable that a true neuritis has developed. Symptoms. — The symptoms of sciatica are pain in the back of the thigh and in the outer side of the leg, referred quite distinctly to the course of the nerve. Sometimes when the upper branches of the nerve are involved pain is felt over the sacrum and buttock as high as the waist line. In other cases these branches escape. Usually the pain is most intense about the sciatic notch and down the back of the thigh to the knee. Sometimes it is limited to the lower branches of the nerve upon the outer side of the leg to the foot. In the severer type of case the entire distribution of the nerve is the seat of pain. The pain is not as diffuse as in muscular rheumatism, and when the patient is asked to indicate its position he follows down with the tips of his fingers the line of the nerve from its exit in the sciatic notch to the external condyle of the ankle. The pain occurs in paroxysms and may be agonizing in its intensity ; is usually in- creased by movement, by walking, especially by going up stairs, and can always be elicited by hyperextension of the leg upon the thigh and of the thigh upon the pelvis — a position which stretches the sciatic nerve. It is attended by tenderness at those points where the nerve can easily be compressed between the fingers and a prominent portion of the bone, namely, above the hip-joint near the posterior iliac spine, at the sciatic notch, at the middle of the thigh, behind the knee, below the head of the fibuhi, behind the external condyle of the ankle, and on the back of the foot. The pain is usually dull in character and constantly present, but there are acute exacerbations and these are 746 neuhalgia. often attended by sensations of burning or feeling as if water were trickling along the limb, or sharp, darting pains through the length of the nerve. The pain is a deep-seated one and is referred to the muscles or the bone, never to the skin. It may be increased by ex- tension of the leg and thigh together. It is also increased by abduction against resistance of the unaffected thigh (Bonnet's sign) and by flexion against resistance of the unaffected thigh (Martin's sign). Pains are usually worse at night, when the patient gets warm in bed, though, as a rule, warmth to the limb is grateful and the patients instinctively avoid exposure to cold. The gait is affected in sciatica, the patient limping and moving the entire limb stiffly. The toe is turned out, the leg is slightly abducted, and the patient instinctively rotates the spine to the well side. In ex- treme cases there may develop a tendency to lateral curvature of the spine away from the affected side. In the more intense cases the patient is confined to the bed by the pain, cannot bear his weight upon the limb, cannot bear to have it moved, and finds it easy only in one position, which varies in different cases. The leg is usually abducted, rotated outward, and flexed at the knee. It is best to allow the patient to ascertain the position in which he is most comfortable and to keep the limb in that position 'supported by pillows. The pain may be so intense as to cause reflex spasms and twitchings of the leg, and is very conunonly associated with tingling and numbness in the leg, or by sensations of pins and needles, the foot being asleep constantly. When the sciatica goes on to a neuritis, anaesthesia may develop in the outer side of the leg below the knee and in the back of the foot, and the muscles of the calf of the leg may be weak or even paralyzed and atrophied, showing a reaction of degeneration. The course of the disease varies very much in different cases. There is sometimes a sudden, acute onset which in cases due to exposure to cold or to rheumatism, or to gout, is often attended by a slight rise of tempera- ture and constitutional disturbances. Usually the onset is gradual. Pain occurs in paroxysms, which become more and more frequent and severe during several days, and by the end of a week the pain has become con- tinuous. In bad cases the patient limps or is unable to walk. " This con- dition may remain for several weeks or may subside gradually. Recovery ensues within three or four weeks, though in many cases months pass before the patient is free from pain. There is a great tendency to relapse in cases of sciatica. I have rarely known a patient to escape a second and third attack. Even when recovery has ensued, pain can be pro- duced by the slightest tendency to overstretch the nerve, or by too great exertion in walking. I have never seen a bilateral sciatica, though a number of cases are recorded. Even in the chronic cases improvement after a time occurs, but where a neuritis has been set up the pain is re- mittent rather than intermittent. In the cases which are due to pressure within the pelvis the pain is more likely to be in the periphery of the nerve and its terminal distribution than in the trunk upon the thigh. In the cases where neuritis is present there is usually tenderness along the entire course of the nerve, as well as at the painful points of Valleix. SCIATICA. 747 Herpes is a common complication of sciatica. It is sometimes very extensive over the back of the thigh and on the buttock. The her- petic vesicles may become large and confluent, so that the affection of the skin may give as much discomfort as the pain. In this condition the skin should be protected by applications of oxide of zinc ointment and bandaging with cotton-wool. French authors have called attention to the characteristic position of the limb in standing and in walking, which is characteristic of sciatica and serves to distinguish it from muscular rheumatism, or from hip- joint disease.^ In sciatica the thigh is somewhat adducted, the fold of the buttock is depressed, and the knee is slightly flexed, even when standing in an upright position. The entire spine is somewhat de- flected with convexity to the affected side. This is due to an instinc- tive effort to support the weight on the well limb. There is some- times a slight wasting of the muscles in the limb. Diagnosis. — The diagnosis of sciatica is usually evident from the characteristic symptoms, but it has been mistaken for disease of the hip-joint, for caries of the sacrum, and for lesions in the cauda equina. In hip-joint disease the pain is usually felt first in the inner side of the knee. It is not associated with tenderness along the course of the sciatic nerve, though pain may be felt all about the hip-joint. There is usually a limitation of the motion of the joint and possibly some shortening of the thigh, and a characteristic rigid position of adduc- tion with slight rotation is found in hip-joint disease. In caries of the sacrum, while the pain may be felt in the sciatic nerve, there are usually symptoms of tenderness along the sacrum, tenderness in the motion of the pelvic bones upon the sacrum, and the pain is less unilateral than in sciatica. A rheumatic affection of the sacro-iliac tendons and liga- ments does not produce pain below the exit of the sciatic nerve in the thigh. In muscular rheumatism the pains are diffused and are not located exactly in the course of the sciatic nerve. In affections of the cauda equina, meningeal thickening, hemor- rhages or tumors, the symptoms are rarely, if ever, unilateral in character. Considerable pain is usually felt over the sacrum, and incontinence of urine and feces, with anaesthesia about the rectum, are early symptoms which are not present in sciatica. Locomotor ataxia very often produces severe pains in the course of the sciatic nerve, and although the earliest sign of this disease is more often pain in the distribution of the crural nerve, sciatica may be its first symptom. In every case of sciatica, therefore, the knee-jerk should be tested and the pupil be carefully observed to detect any lack of reaction to light; these reflexes are not affected in sciatica. Anaesthetic areas should be sought for, as these are not present in true sciatica, but develop early in locomotor ataxia; Prognosis. — The prognosis in sciatica is fairly good as regards eventual recovery, but no statement can be made with regard to the duration of any particular attack, for although the duration of each 'See also II. Eliret, Mittheilungen aus der Grenzgebiet der Med. u. Chirurg., 1898, 695. 748 NEURALGIA. attack is somewhat in proportion to the severity of the symptoms, relapses are so common that statements in regard to duration are never precise. I have known cases to last two or three years and then recover. Hyde has given some interesting facts in his analysis of 200 cases which may be quoted here. The duration was less than one month in 45 cases; from one to three months in 70 cases; from three to six months in 29 cases ; from six to twelve months in 29 cases ; two years in 15 cases; three years in 6 cases; four years in 1 case; five years in 2 cases, and six, seven, and ten years in one case each. Forty-three of Hyde's cases had had previous attacks. Treatment. — In addition to the general statements with regard to the treatment of neuralgia, all of which may be applied to sciatica, a few special indications may be given. The cause must be removed if possible. In all cases active purgatives must be given at the outset. In cases due to cold or rheumatism salicylates and salophen are of the greatest service. Salophen or aspirin may be given in 15-grain doses every two hours. Absolute rest of the limb is most important, and the patient should be advised to remain upon the bed or couch, even from the beginning of the disease, being allowed to assume the position which gives the greatest comfort, as this varies in different individuals. All positions and all movements which intensify the pain should be avoided. I have not seen any benefit from fixation of the limb, either by a long splint, as has been recommended by Hammond, or in a plas- ter bandage as has* been suggested by others. Hot applications are of the greatest service both in the acute and chronic stage of the disease. The old-fashioned remedy of ironing the limb with a hot iron is of dis- tinct service. The application of flaxseed poultices is of benefit in the acute stage, but the most efficacious remedy is the application of the Paquelin cautery along the course of the nerve. This should be ap- plied daily from the very beginning of the disease. Or small fly blis- ters should be applied at the points of tenderness along the trunk, and should be repeated every other day. If the cantharides is applied by means of collodium the blisters need not be more than one-quarter of an inch in diameter, and as many as twenty may be placed in the course of the nerve. A very efficacious method of the treatment of sciatica is exposure of the limb to very high temperature in an apparatus in which hot air can be produced. Thus the temperature of the air may be raised to 250° or 300° F., the limb being encased in the apparatus. And such applications may be repeated twenty minutes in duration daily. It often gives much relief to expose the limb to a stream of steam along the course of the sciatic nerve. Hot- water massage is also of a great deal of service, such as is applied at Wildbad, Ragatz, and Bath, at Aix-les-Bains and at the Hot Springs of Virginia, the stream of water under pressure from twenty to thirty, or even forty pounds, being directed upon the limb, the painful points along the nerve being pro- tected by the hand. Long-continued hot sitz baths are often of ser- NEURALGIA OF THE TESTICLES OB OVARIES. 749 vice aud give the patients relief. Richfield Springs has a good repu- tation in tlfe treatment of sciatica, and I have known patients to recover after a course of sulphur baths there. Massage of the limb is sometimes of service if it is done with great skill and pressure is but lightly exerted upon the nerve trunk. Mud or sand baths heated to 100° F. are of great service. A spray of chloride of ethyl which causes local freezing sometimes gives immediate relief. Hypodermic injections into the nerve have been practised and recommended. Hypodermically an injection of chloroform, of a 2 per cent, solution of cocaine, or of a 1 per cent, solution of atropine, or simply of dis- tilled water, may be given. Good results have been obtained by hypo- dermic injections of normal salt solution at a freezing temperature, 32° F., not injected into the nerve but into the thigh near the nerve trunk, 30 c.c. being used daily. Osmic acid solutions are dangerous. In a few cases where the nerve has been felt to be much swollen puncture by a needle has resulted in the evacuation of serum from the sheath, and has given relief. Long needles are to be used under strict aseptic precautions. They are to be inserted and left in an hour, the limb being kept immovable during this time. Liniments and ointments containing belladonna or aconite may be rubbed in along the course of the nerve, and are sometimes of benefit. The general medicinal treat- ment by internal remedies may be conducted along the line already described in the treatment of neuralgia. Electricity I have never found of much service, but long-continued applications of galvanism of very mild strength (five or six milliamperes) from large sponges may be tried. In chronic cases where all other means have failed to relieve it has been proposed to stretch the nerve, and this is probably of service in cases where an interstitial neuritis has produced adhesions between the adjacent bundles of nerve fibres and constriction of the blood-vessels. A nerve may be stretched by using hyperextension of the leg, a pro- ceeding which has to be done under chloroform, as it is very painful. Or the nerve may be stretched by cutting down upon it just below its exit in the sciatic notch, dividing the sheath and stretching it with the fingers. Some cases have been reported of improvement after this procedure. I have seen one cure in a very obstinate case. I have seen several failures. Other cases have been reported in which nerve stretching has been followed by paralysis in the muscles supplied by the affected nerve. It should only be resorted to after every other means has been tried, and no positive promise of relief should be given. Neuralgia of the Testicles or Ovaries. — Neuralgia in these organs is usually the result of some congestion of the veins due to organic disease or to pressure. But occasionally it may be due to some one of the general causes of neuralgia already mentioned. The pain is very intense and most demoralizing. It causes great restlessness and much mental depression. It is occasionally attended by sexual excite- ment. Neuralgia of the ovaries may coincide with the menstrual function. 750 NEURALGIA. The treatment is first directed to reducing local congestion. This may be done by elevating the pelvis and letting gravitation empty the veins. Applications of heat, especially hot poultices to the testes, and hot vaginal douches or rectal injections, to be taken in a recumbent position, are usually of great service. Resort may be had to the stronger narcotics early, as it is better to cut an attack short than to allow it to go on. And attention to general measures for building up the health must not be omitted. Neuralgia of the perineum, of the prostate gland, and of the neck of the bladder in males, and neuralgia of the rectum in both sexes, is occasionally complained of. If no local disease is present and the affection is not traceable to lithsemia or to hemorrhoids, it is probably caused by local congestion. Massage of the perineum, the pelvis being raised, will give relief, or hot applications to the perineum may be effective. If these fail, massage of the prostate through the rectum, or stripping the seminal vesicles may do good. A continuous irriga- tion of the rectum with hot water by means of the double tube devised by Keyes is of much service in these cases. Sitz baths, either hot or cold, are of value. As in neuralgia of the genital organs prompt relief must be given by narcotics to avoid a state of mental depression. I have found an ointment or suppository of cocaine and hyoscyamus of great service. Weir Mitchell has seen this form of neuralgia as an early sign of tabes, an observation which I can confirm, having seen two such cases. I have also known it to occur in lithsemic individuals and in persons whose circulation was poor, after sexual excitement, both of a natural and of an unnatural kind. Coccygodynia. — Neuralgia of the coccygeal nerves is an exceed- ingly rare affection. But pain in the tip of the spine is very common, and has received the name of coccygodynia. It may be a local affec- tion, due to injuries, especially in women after confinement ; to falls on the seat or to caries of the spinal bone. It is usually in my opinion a referred pain of central origin, and may develop in very many func- tional or organic diseases of the nervous system. In neurasthenic, hysterical and ansemic persons, chiefly in women, it is a frequent com- plaint. It may be associated with pain in the nape of the neck or between the shoulders, with irritable spine, or with any hysterical state. It is a very common complaint in cases of traumatic neurosis. In the majority of cases it is a purely hysterical symptom, and is increased by any local treatment or by anything which directs attention to it. In several such cases where gynecologists have exsected the coccyx the pain has persisted after the operation. As the hysteria or neurasthenia passes off the pain is forgotten. Hence it should never be treated directly, but only as a symptom of a general condition. In the few cases which are true neuralgia the same treatment as that of sciatica will be applicable. REFERRED PAINS. 751 REFERRED PAINS. There are a great many conditions of disease in which more or less severe pain is felt in various parts of the body at a distance from a dis- eased organ. The explanation for the referring of these pains to a part of the body which is really not affected is as follows : The branches of the visceral nerves and of the general sympathetic nervous system enter the spinal cord at various It^vels throughout its entire length. Irritation sent into the spinal cord through these nerves set up sensory impulses in the various segments of the cord, each seg- ment receiving impulses from a certain organ. These sensory impulses are sent upward to the brain, and become conscious perceptions. They are referred by consciousness not to their actual point of origin, but to the part of the body from which sensations usually come when received at the particular segment irritated. Thus, as in general ex- perience, sensations and pains coming from the various segments of the spinal cord have been due to irritation in the surface of the body corresponding to these segments ; these various visceral sensations are referred to the surface of the body. Some examples of such referred pains will make this matter clearer. It is not at all uncommon in eyestrain to have a pain felt in the fore- head or in the back of the neck, neither of which parts is in direct connection with the eye. The pain produced by decayed teeth may be felt in the temple or behind the ear, instead of in the jaw. Severe pain in the back of the head is a common symptom of uterine disease or of inflammation of the bladder. Pain down the left arm is a common symptom of heart disease, and may be attended by hyperaesthesia in the region of the fourth and fifth dorsal nerves on- the chest. Pain in the wrist on the flexor surface is frequently felt in disease of the uterus, ovaries, or bladder. Pain under the right shoulder-blade is frequently felt in disease of the liver, and is often attended by hyperaesthesia in the domain of the eighth to the twelfth dorsal nerve. Pain under the left shoulder-blade is common in enlargement of the spleen. Pain between the shoulder-blades is a very common symptom of gastric affections of any kind. It may be attended by hypersesthesia in the epigastric region, and the nearer the disease to the cardiac end of the stomach — e. g., ulcer — the higher the pain is felt. In severe vomiting pain may be felt on the back of the arms or even down the back of the forearms. Pain across the small of the back is common in colitis or in impac- tion of feces within the colon. Pain across the upper sacral region is very common in uterine disease. 752 NEURALGIA. Pain over the outer side of the hip is usually due to ovarian con- gestion. Pain down the inner side of the les; is also due to the same cause. Fig. 299. J'/Dtcnyed teeth Neurasthenia (Bpinal irritation ) m/nsmia ^Neurasthenia Ovaries? The location of referred paius and their cause. (Dana. ) Area. Cerebrospinal nerves. Distribution. Associated ganglia of sympathetic. Distribution, I. Trigeminus, facial. Face and anterior scalp. 4 cerebral. Head. II. Upper 4 cervical. Occiput, neck. 1st cervical. Head, ear. III. Lower 4 cervical Upper extremity. 2d and 3d cervical, 1st Heart. and 1st dorsal. dorsal. IV. Upper 6 dorsal. Thorax. 1st to 6th dorsal. V. Lower 6 dorsal. Abdomen, upper lum- 6th to 12th dorsal, bar. VI. 12th dorsal and 4 Lumbar region, upper 1st to 5th lumbar, lumbar. gluteal, anterior and inner thigh and knee. VII. 5th lumbar and 5 Lower gluteal, posterior 1st to 5th sacral, sacral. thigh and leg. Lungs. Viscera of abdomen and testes. Pelvic organs. Pelvic organs and legs. Pain on the inner side of the knee is au early symptom of hip-joint disease. Pain in the heel is a frequent symptom in lithaemia, and may also be felt in ovarian disease. Dana^ has studied the location of these reflex or referred pains ' New York Medical Journal, July 23, 1887. REFERRED PAINS. 753 very carefully, and his figures (Fig. 299), which are here reproduced, demonstrate the areas of pain of sympathetic origin. Head ^ and Mackenzie ^ have added a few facts to Dana's statements. Head has called attention to the fact that when a viscus is diseased there may be detected by examination with a sharp instrument an area of hyperalgesia on the skin of the back and abdomen, each viscus Fig. 300. Gall Bladder Caecum and Appendix Ovary and Tube ^ / Stomach (Left Half) Kidney Ureter The general location and outline of the zones of cutaneous hyperalgesia for some of the abdominal viscera. Anterior view The maxima are deeply shaded. Only the left half of the gastric zone is given. The ureteral zone consists of a series of maxima (diagrammatic). (Elsberg and Neuhof. ) having its particular area. Figs. 300 and 301 show the areas related to the various viscera, as recently determined by Elsberg and Neuhof,^ whose results confirm Head's statements. There is hardly any viscus in which disease may not give rise to some of these referred pains, and it is evident that without some knowledge of the localities of these referred pains the presence of such pain might mislead the physician. I have had many patients brought to me for supposed spinal disease because of pain in the dorsal region and tenderness of the dorsal spine, with pain in the epigastrium, gen- eral weakness and neurasthenic conditions, and increased knee-jerks, who have been relieved by the correction of a chronic gastric dyspepsia. 1 Brain, 1893, 1894, 1890. == Brain, 1902. 4g *Araer. .Jour. Med, 8ci., Nov., 1908, 754 NEURALGIA. It is evident that in these referred pains the treatment must be directed to relief of the functional or organic disease in the viscus which is affected. It may be added that not infrequently sharp counter- FiG. 301. lOth Dorsal Spine -1st Sacral The general location and outline of the posterior parts of the zones (diagrammatic). irritation over the seat of pain is followed not only by relief of the pain, but also by improvement in the visceral condition which is its cause. It is also to be remembered that aneurism of the aorta and tumors of the thorax or abdomen often cause pain referred to the thorax or abdomen at a distance from the origin of the pain. CHAPTER XLIII. HEADACHE AND MIGRAINE. I. HEADACHE. Headache is a symptom of very common occurrence and its cause may be quite difficult to determine in any particular case. Its variety in intensity, duration and location is extreme. Etiology. — Its causes may be classified as follows: Organic. Injuries of the head and fractures. Meningitis, general or localized. Meningeal hemorrhage. Tumor or abscess of the brain. Arterial changes preceding apo])lexy. Syphilis of the meninges or brain. Toxic. The infectious diseases. Autointoxication from disease of the kidneys, the liver, the stomach and intestines, the ductless glands. Intoxication by alcohol and drugs, by lead and other poisons, by tobacco, by bad air in close places. From impaired blood states — Anaemia, Rheumatism and gout, Diabetes, Malignant disease, Hodgkin's disease. Reflex from disease of eyes and ears, nose and pharynx, teeth, viscera, uterus and ovaries. Neuralgia. Neurasthenia and the traumatic neuroses. Psych asthenia. Hysteria. Fatigue. Climatic changes. Symptoms. — The characteristics of some of these forms of headache require special attention, 755 756 HEADACHE AND MIGBAINE. Headache from organic disease is attended by so many other symp- toms which point to the diagnosis that it needs no special description, especially as it has been already discussed in connection with brain tumors (page 584), abscess (page 553), meningitis (page 717), arte- rial sclerosis (page 469), syphilis (page 691). Headache from toxic causes presents many varieties. All the infec- tious diseases begin with fever and this is usually ushered in or accom- panied by headache of a general dull intense kind increased by any effort of mind or muscle. The degree of the pain is proportionate to the height of the temperature and to the intensity of the infection. In many fevers, notably typhoid and typhus, the headache remains for a long time, but in the children's infections it subsides after a few days. Malarial headaches are very common and may be of any degree of intensity and in various positions. The particular characteristic is its tendency to come on suddenly ; to recur at a definite time of the day, either daily or every other day, or once a week, and to cease as sud- denly as it came after a few hours. Such headaches may be the only evidence of malarial infection ; though an examination of the blood will establish the diagnosis. The remedies are a systematic course of treatment by quinine, Warburg's tincture or arsenic. In nephritis the headache is due to the ursemic state and may be the first symptom of that condition. If very intense it may presage a con- vulsion, as in the puerperal state. If dull and continuous it may show a chronic uraemia or the state of arterial sclerosis to which the nephri- tis is due. It is then a pulsating headache made worse by efforts. Jaundice is sometimes attended by headache, and chronic cirrhosis of the liver even without jaundice is liable to cause it, as it interferes with the chemistry of digestion. Such a headache is not intense, is rather dull and felt all over the head. Gastric and intestinal intoxication is probably the most common cause of all the forms of headache. In dyspepsia the patient feels a dull, disagreeable feeling over the eyebrows which varies from hour to hour, being usually relieved by eating and then returning with renewed intensity about an hour after food. It is particularly liable to be severe on waking. In intestinal toxaemia the pain is usually felt over the entire fore- head, is dull and often throbbing in character and is increased by motions of the bead or by muscular effort. It is often associated with pains in and back of the eyes. Much relief is afforded in these cases by high irrigations as well as by brisk laxatives, especially aloes. In auto-intoxication by errors in the ductless glands, in myxoedema and Basedow's disease the headache may be very intense at times, but varies from day to day, and is not constant. Intoxication by alcohol and drugs causes very intense generalized pain, which persists until elimination is secured. It is dull and goes away very slowly and is attended by mental apathy, especially if the drug is opium. Lead causes very severe frontal pain, as does also mercury. Arsenic and copper do not often cause headache. HEADACHE. 757 A few nprsons are affected by tobacco unfavorably and after long- continued inhalation of tobacco smoke in a heavily laden atmosphere headache is not uncommon. The headache which follows confinement in the close atmosphere of a badly ventilated hall, theatre or church is familiar to everyone, though the exact nature of the poison producing it is unknown. It disappears in an hour or less after deep inspirations of fresh air. Certain states of the blood cause intense headache. Ansemia is the one most commonly observed, especially in young women. The pain is felt chiefly on the top of the head, but may also be over or behind the eyes and sometimes is felt as a band about the head. It is usually attended by constipation. Sir Andrew Clark used to say that he preferred aloes to iron in the treatment of ansemia in women. Rheumatic and gouty affections of the scalp are a frequent cause of pain in the head and are attended by tenderness of the scalp especially at the forehead and occiput. Sometimes rheumatic deposits can be felt in the fascia over the occiput. They may usually be relieved promptly by a shampoo with hot water followed by through massage of the scalp. The term reflex headache has been used to indicate a pain referred to the head although due to an irritation of some nervous mechanism or some branch of the trigeminal nerve by processes going on else- where. (See also Chapters XLII. and LVI.) The use of the eyes when there is difficulty in the focus from astig- matism or asthenopia or when there is defective action of the external muscles is liable to cause much irritation, and produces pain not only in the eyes but in the forehead and often in the back of the neck and occipital region. Such pain is usually felt after a use of the eyes either for far vision as in looking at pictures in a gallery or at the stage in a theatre, or else in near vision as in reading. If the cor- rection of the astigmatism or of near or far vision by glasses fails to relieve this headache it is well to examine the condition of the ocular muscles as an insufficiency of the internal or external recti, as the case may be, is often a cause for intense headache. Such insufficiency can usually be relieved by the use of prisms and division or advancement of the muscles is rarely necessary. Severe headache, especially in the temporal region, is occasionally due to an irritation in the internal ear and the condition of the ears should always be examined in a case of unilateral headache. Such headaches are often attended by vertigo. See Chap. LIII. Reflex headaches from irritation in the nose and pharynx are occa- sionally felt in the forehead and between the eyes. Children with ade- noids often complain of headache in this region. Pain in the head as the result of defects in the teeth is sometimes felt in the temporal region, sometimes in the occipital region. Lauder Brunton has called attention to this form of headache and believes that decayed molar teeth in the upper jaw cause temporal headache which is rather farther forward than that caused by decayed molars in the 758 HEADACHE AND MIGEAINE. lower jaw. Diseased molars in the lower jaw may also give rise to occipital headache, while decayed incisors or eye teeth are more likely to cause frontal or vertical headache. A headache is also frequently complained of in diseases of the ovaries and uterus, and is usually located in the back of the head just below the occipital protuberance. It is often relieved by lying down or by lying on the stomach, and appears to have some relation to traction upon the ligaments of the uterus and ovaries, so that rest in bed as a rule relieves this form of headache. There are numerous cases re- ported where a proper adjustment of the pessary holding the uterus in place has relieved immediately this form of headache, which has how- ever returned when the pessary was removed. Neuralgic headache is fully discussed under the chapter on Neuralgia of the Trigeminal Nerve. The headache of neurasthenia and of the traumatic neurosis is fully described on page 841. Psychasthenic headache has been alluded to on page 867. It is really a form of delusion of pain, and the characteristic of this form of headache is its long duration. When a patient complains that he or she has had a headache constantly for months or years without very great variation in the degree of the pain and without any evidence of the malnutrition which accompanies severe continued organic pain, the suspicion should be awakened that the pain is of a delusional nature or a subconscious pain. The reader is referred to the discussion of this subject on page 870. The only treatment that is successful for this type of headache is hypnotic suggestion. The headache of hysterical conditions differs from that of psychas- thenia in being sudden in its onset and agonizing in its character. It may be felt at the base of the brain behind the eyes ; it may be felt as a sharp deep agonized pain at the occiput, or it may be felt on the ver- tex. Patients will complain that they feel as though a nail were being driven through the head on top or at the back. Others will complain that they feel as though a band were being tied tight about the head. Such patients complain most bitterly of the headache, and until the hysterical paroxysm is over very little relief is afforded by ordinary remedies. Even hypodermic injections of morphine, J to | grain seen to have no effect upon this type of headache, though sometimes very minute doses of ignatia, nux vomica, strychnine or hyoscyamus repeated every 5 minutes until ten doses are given may relieve this form of head- ache. Hot applications to the feet, mustard foot baths, ice to the head or to the back of the neck, or a small blister to the back of the neck, may also be tried. Another type of hysterical headache is a dull continuous frontal pain which is increased by any action of the mind or effort of the attention. This resembles the psychasthenic headache in being more or less constant, and probably has the same origin in a subconscious state with suggestion. The headache of fatigue is a dull pain felt over the forehead near HEADACEE. 759 the line of the hair and back of the eyes. It is more liable to come on after fatigue of the mind in reading, studying or intense application of any kind and it is only to be relieved by rest. Climatic changes not infrequently set up a dull continuous headache. Thus persons who are obliged to live in a very warm climate or in a damp climate such as officials in the Philippines or travellers in the tropics are very liable to suffer from this form of headache. Those who climb high mountains or who go to high altitudes to reside in the summer, as in Switzerland or among the Rocky Mountains, not infre- quently suifer from headaches due to the elevation. Foreigners coming to America appreciate the unusual stimulus of our climate and not infrequently suifer from chronic headaches while they remain here. All such headaches if they require treatment are best treated by seda- tives of the nature of bromide, phenacetine, and the coal tar products. Treatment. — The treatment of headache will be determined en- tirely by the cause of the condition and when this can be ascertained and removed relief can be afforded. In some cases however the cause cannot be removed, as in organic diseases and in many forms of toxaemia. In such cases it is necessary to resort to symptomatic remedies. Of these the coal tar products are the most efficacious, phenacetine, acetanilide, aspirin and pyramidon being the best. As all of these drugs are more or less depressing to the circulation it is well to combine them with a mild heart stimulant, of which caffeine, ignatia, or brucia may be selected. It has also been found that if they are given very finely triturated they act much more promptly. And further the absorption in the stomach seems to be aided by the use of an alkali, hence a small amount of bicarbonate of soda should be added to the powder given. The following : R Phenacetine. gr. x Caffeine Citratis. gr. j Ignatia. gr. jhr bod. Ijicarb. gr. iij M. Triturate. Sig. Tal pulv. q. 3 hr. with a glass of water. is a very good symptomatic remedy for headache, though any of the coal tar products may be substituted for the phenacetine. Many of the headache powders sold by druggists and many of the proprietary remedies widely advertised have been found to consist of similar com- binations. This combination is the one in some tablets sold. It should be remembered that tablets act much more promptly if chewed up and swallowed well diluted with water. Another symptomatic remedy for headache is bromide in 20 or 30 grain dose. This is particularly indicated in headache from fatigue, from overwork and anxiety and from cerebral congestion due to any cause. It is also of service in the infectious forms of headache and in some of the toxic states. Some forms of headache are so intense as to require the use of opium. Codeine may be added to the powder already described in ^j^ or | grain 760 HEADACHE AND MIGRAINE. dose ; or may be given alone. In very intense pain hypodermics of morphine are needed as in the pain of typhoid fever or of brain tumor or meningitis. MIGRAINE. Migraine, hemicrania, or sick headache, is a functional neurosis characterized by sudden attacks of intense pain in one side of the head, often preceded by evidence of irritation of various functions of the brain and followed by digestive disturbances. Etiology. — Heredity plays an important part in the development of this disease. In almost all cases the history can be obtained of a similar aifection in some near member of the family. The disease develops at an early age. Infants are rarely affected, but children of the age of seven or eight may develop it, ^nd as a rule it remains as a chronic condition throughout life. If the disease does not develop in childhood, it commonly develops at puberty. There are no known predisposing factors to this disease, and it appears to be due to a con- genital defect in the chemistry of nutrition, rather than to any extra- neous cause. In-door occupations, or a sedentary life, tend to increase the liability in a person predisposed, and it is a well-known fact that those who work in the open air, or are engaged in occupations involv- ing considerable vigorous exercise, are never affected. The active cause of the attack is undoubtedly a sudden development in the sys- tem of a poison. This poison at first produces an excitement and stimulation of the brain causing a sense of well-being, of increased capacity and of exhilaration. This is soon followed by a secondary and more lasting effect of pain and suspension of the cerebral func- tions, and, finally, there is an elimination of the poison through the stomach, intestines, or kidneys, and a return to a normal state of health. The nature of this poison is still undetermined. Some have considered it uric acid ; others have thought it was a leukomain ; others have considered it xanthin. Although the manifestations are chiefly in the domain of the nervous system, it is generally admitted that this disease is one of nutrition. In some cases it has been thought that an insufficiency of the ocular muscles has been the cause of the affection ; an insufficiency of the interni resulting in a constant strain when con- vergence in reading is necessary, and an insufficiency of the externi leading to a strain when distant vision is practiced. On this theory attempts have been made to cure the disease by the use of prisms, by division of the ocular muscles, or by the use, in the case of insuffi- ciency of the interni, of strychnin, and in insufficiency of the externi, of cannibis indica. While it is possible that irritation from the eyes may act as an excitant of the nervous system, and then make it more liable to be affected by the poison of the disease, it seems improbable that these causes are sufficient to produce the affection. There are some who believe that migraine like epilepsy is a func- tional neurosis of the cerebral cortex and that the attacks are not of MiGBAINE. 761 toxic origin. Gowers ^ relates cases in which the two diseases alternate — and otfier cases in which one has succeeded the other. He cites the manifest irritation of certain cortical areas at the onset of the attack, and the exhaustion of cortical activity at its conclusion as evidence. I am inclined to regard the cortical symptoms as secondary to the toxaemia. Symptoms. — An attack of migraine begins with a prodromal period of excitement in about one-half of the cases, and this period lasts several hours. During it the patients feel particularly well and active. They are bright mentally, happy, and slightly exhilarated, and are likely, during this period, to over-exert themselves, or over-tire them- selves, in whatever line of occupation they may be interested. The attack begins with a sudden, intense boring pain in the temple and at the back of the head, or over the entire side of the head, attended by a sense of complete prostration, inability to think, hypersensitiveness to sound, light, and touch, so that the patient prefers to lie in bed in a quiet, dark room, wishes to be spoken to in whispers, and is annoyed if the bed be touched or shaken. There is also a hypersensitiveness to smell and taste. Appetite is wholly wanting, and nausea soon develops, which, as a rule, is followed by intense and frequent attacks of vomiting. The vomited material is first the contents of the stomach, which are usually excessively acid, and then bile mixed with acid mucus. The vomiting causes intense prostration, a very weak, rapid pulse, and very often pain between the shoulder blades and down the arms. Any food taken into the stomach is immediately rejected. Occasionally attacks of diarrhoea coincide with the vomiting. As a rule, very little urine is passed during the attack, and this is of high specific gravity, dark in color, and is loaded with uric acid, skatol and phosphates. Xanthin and leukomains have also been found in it. There is usually a very great increase in the excretion of phosphates. If the attack comes on in the daytime, the patient is immediately forced to go to bed by the intense pain and prostration. Many patients awake in the morning from a deep sleep suffering from an attack, and are unable to rise. The attack lasts from twelve to seventy-two hours. During the attack the patient, as a rule, feels cold, and heat is always agreeable, both to the head and to the body. In the majority of cases there is a pallor of the surface. The nose and extremities are very cold and dry, though occasionally there is an acid perspiration making them cold and clammy. Occasionally the face is much flushed, espe- cially on the side of the pain. In some cases the attack is preceded, or ushered in, by the appear- ance of a bright spot or star on one side of both eyes of any color, or flashes of light, or zigzag lines. These are true hallucinations of vision and they are not stopped by closing of the eyes ; as they are hemiopic in their distribution they indicate irritation of the opposite occipital lobe. They may be attended by temporary blindness which subsides ^ Clinical lectures on the Borderland of Epilepsy. Migraine. Brit. Med. Jour., Dec. 8, 1906. 762 HEADACBE AND MIOHAINE. with the pain ; such cases have been named ophthalmic migraine. In a few cases temporary paralysis of the third nerve has been seen. Occasionally ringing in the ears, or the sound of bells, or the running of a tune in the head, precedes or accompanies an attack. Occasionally tingling and numbness of a disagreeable kind are felt in the side of the body opposite to the side of the pain. Occasionally attacks of aphasia occur before, or in connection with the attack, and the patient is unable to express his desires and misplaces words and is reluctant to talk, although the power of talking is never suspended. Occasionally a sense of great mental confusion and inability to think is appreciated during an attack. Oppenheim has seen a case associated with vertigo and staggering gait which he considers of cerebellar origin. In the description of the aura in epilepsy (see page 814) very similar sen- sations will be described as occurring in petit mal and as the first symptoms of grand mal. Here again therefore Gowers finds a proof of some relation between the two diseases. In epilepsy the aura is only momentary in duration, while in migraine these sensations remain for twenty minutes or longer. All these symptoms indicate distinctly an irritation of the cortex of the brain, one hemisphere only being affected, namely, the hemisphere on the side on which pain is felt. For this reason, in some cases, it has been supposed that there is a con- gestion of the hemisphere and in other cases an anaemia of the hemis- phere, and on this theory ergot and nitroglycerin have been given, but are of little avail. During the attack of migraine the patient is ex- tremely somnolent and sleeps for several hours or even for two days, being however easily aroused. It is not uncommon for the patient to be slightly delirious when aroused from this sleep and to have no recol- lection of the delirium when the attack is over. The attack terminates gradually as a rule, the pain passing off. A very large flow of urine terminates it and in this urine an excess of uric acid, skatol, xanthin, or leukomains have been found. The patient feels much prostrated for a day or two and then returns to his or her normal state of health. The attacks recur with varying frequency in different patients. Some women have these attacks uniformly once a month, coinciding with the period. Many have attacks as often as once in two weeks and they may increase in frequency until the patient has one every few days, making the life one of invalidism. The attacks usually subside about the age of fifty, and old people are rarely subject to them. In women they usually cease after the menopause, and I have known severe attacks to cease after ovariotomy. They often cease during pregnancy. Prognosis. — The prognosis for recovery from the disease is not a good one, as attacks are liable to recur from time to time until the age of fifty, unless the habit of life can be radically changed. There is no danger of death in an attack, even though it is a very severe one, and although the symptoms of prostration may at times seen alarming. Treatment. — This consists of a general regime and treatment of the underlying digestive condition in the interval between attacks and in the treatment of the attack itself. MIGBAINE. 763 The genpral regime. Diet. — A simple but nutritious diet of small amount is to be followed rigidly. The tendency to over-eat should be guarded against. Fruit (except strawberries), cereals, eggs, fish, white meats, ham and bacon, game, vegetables of almost all kinds, custards, simple desserts made without pastry or sugar may be allowed. Rich sauces are to be avoided and also things which are fried. Eggs disagree with many of these patients and so does milk. Red meat may be allowed every other day in some form, but in some patients has to be excluded, though in these individuals ansemia is to be pro- vided against. Tomatoes, asparagus, eggplant, cauliflower, and salads made with vinegar, are often found to disagree, and if so may be excluded. Cheese, especially the French cheeses, are to be excluded. Sugar is to be used very sparingly and all candy is to be forbidden. Patients vary in susceptibility to tea and coffee. In some they are injurious. In some a strong cup of either will abort an attack. Alcohol in any form must be forbidden. Tobacco may be used in moderation. Patients subject to migraine should drink at least two quarts of water daily, but this should not be taken with meals. I have seen much benefit from the use of piperazine water. Any alka- line water may prove useful. Exercise in the open air, sufficiently active to cause a perspiration, is absolutely essential. Tennis, horse-back riding, rowing, and golf are the best forms of exercise and should be persisted in daily both in summer and winter. Baths are of much use; either a Turkish bath once a week; or a hot vapor bath followed by a cool sponge off, or a hot bath 106°, made alkaline by the addition of two handfuls of carbonate of soda, of ten minutes duration daily, followed by a cool sponge off. The object of the bath is to stimulate the cutaneous circulation and secure free perspiration. It may be taken on rising or on going to bed, if it does not prevent sleep. Massage is also of much service, especially deep and forcible massage, not only of the limbs, but also of the back and of the abdominal viscera. Swedish movements may be combined with massage. Treatment directed to the digestive functions is usually of much benefit. A saline laxative of which the best is the following : B Sod. Phosphatis exsic. 5 iv Sod. Sulphatis exsic. 5 x Sod. Salicylatis. 5 ij M. Triturate and cork tightly. Sig. One teaspoonful in a large tumbler of hot or cold seltzer water on rising is to be given daily. Once in two weeks either a mercurial purge, calomel, three grains, or blue mass, two grains, or a pill containing euonymin, podophyllin and aloin, each one-quarter grain, should be given. A good intestinal antiseptic is to be given after each meal, put into such a capsule as will dissolve in the intestine, but not in the stomach. 764 HEADACHE AND MlGJtAttJ^, So called " enteric capsules " can be obtained at the druggists, or any capsule can be coated with a mixture made up of salol four parts, shellac one part, alcohol forty parts, the capsule being dipped in this several times and allowed to dry.^ Permanganate of potash, one grain, or sulphocarbolate of soda, five grains, or resorcin, three grains, or benzoate of soda, two grains, with salol, three grains, or salol, five grains, put up in enteric capsules, are all of service and it is wise to vary the drug used. An occasional high large enema of salt solution is very beneficial in these cases. Patients who sufier from migraine are often anaemic and may need iron, but I have found that they take ovoferrin, or Gude's peptoman- gan much better than other forms of iron which often produce attacks of headache. Tonics of all sorts are to be given especially those which contain phosphorus. If there is any insufficiency of the interni, strych- nine may be given, but if the externi are insufficient it should be avoided. Any eyestrain is better relieved by prisms than by operation on the muscles. Seguin used cannabis indica in one-quarter grain dose with some benefit. It is of use when there is insufficiency of the externi. Some authors recommend the long continued use of arsenic ; others use bromides. Gowers recommends the long-continued use of nitro- glycerin, jIq gr., with strychnine, -^^ gv. t. I. d. A remedy of much service is thyroid extract. It is to be given in one gram dose twice or three times daily after meals and kept up for several weeks ; the dose being reduced if it produces an increase of more than 10 beats of the heart a minute above the normal, or if it causes a loss of weight. I prefer the English preparation of Burroughs and Welcome as being reliable in strength. The treatment must be kept up steadily for a year, or more, in order to secure such a permanent change in the habit of metabolism as to secure a cure. A course of treatment at Carlsbad is often beneficial to these patients. The treatment of the attack. — It is often possible to abort an attack by washing out the stomach with an alkaline solution several times and then leaving a strong solution of bicarbonate of soda in the stomach. If this is done promptly it usually arrests an attack. During the attack the patient is to be kept quiet in a dark room with plenty of cool fresh air, but kept warm by hot bottles, or warm flannel wraps. Heat to the head or ice bags may be used according to the preference of the patient. In many cases the coal tar products, antipyrin, phenacetin, acetani- lid, or pyramidon, in one full dose, alone or in some combination, will arrest the attack. In other cases a strong cup of black coffee or a powder of caffeine, three grains, or the fluid extract guarana, one drachm dose will do the same. In some cases very minute doses T oVu" grain of ignatia, repeated every ten minutes, till twenty doses are ^ See Treatment of Leukomain Poisoning by Dr. B. K. Rachford, Med. News, May and October, 1894. MIGBAINE. 765 taken, will mitigate the attack. In some cases a five grain dose of chloral hj^rate repeated every half hour will do the same. I have never seen a good effect from the use of nitroglycerin in the attack, though others recommend it. A full dose of ergot without or with bromide of potash may give relief. Every patient comes at last to rely on some one of these remedies, so if one fails it is well to try another. If everything else fails, a hypodermic of morphine will stop the pain and secure sleep and when the patient awakens the pain is gone. There is no disease, however, in which the danger of the morphine habit is greater and in which it needs to be used with such caution. I believe that it exerts a very detrimental effect on the metabolism, thereby predisposing these patients to a more frequent recurrence of attacks. CHAPTER XLIV. FUNCTIONAL PAEALYSIS. Myatonia Congenita or Oppenheim's Disease. Myasthenia Gravis, mittens or Family Periodic Paralysis. Myasthenia Inter- MYATONIA CONGENITA. Myatonia congenita or Oppenheim's disease is a rare condition occur- ring in infants and characterized by a general state of muscular weakness, probably due to a defective develop- ment of the muscular tissue. At the time when the child should begin to make voluntary movements, to flex and extend its limbs and to raise its head it does not do so, and it makes no eifort whatever at any motion. The muscles of the eyes, tongue, throat and respiration do not appear to be involved, so that the child can suck and breath. But at the age of three or four months the parents notice the entire lack of au- tomatic or voluntary contractions in the limbs, and usually at this time a physician is first consulted. On examination the child is found to be apparently paralyzed, but is usu- ally fairly well nourished, very often is fat and there is no atrophy in the muscles. If the limbs are placed in any position they remain there, and the joints are unusually movable. Thus the legs can be doubled up on the body (see Fig. 303) showing a lack of tone in the muscles which hold the joints in place. If the child is raised the head falls backward and it is entirely impossible for the child to sit up, as there is a complete lack of tone in the muscles of the spine. Pig. 302 shows such a child brought to my clinic on a stretcher ina- 766 Fig. 302. m n 1 ■ ^ vl .^ m TS K ^^^ # ^1 p^ ♦ - X ^^1 '^^ 'l^M ^uj ^^Bii^f^B m ri ^ n i.m t "Nm 1 K ^1 r J 'i '^^i j^B Case of myatonia congenita. Notice the complete rel ixation of all the limbs. The child was carried on a stretcher, though three years old. MYATONIA CONGENITA. 767 provlsed by his mother. It is impossible for the child to turn over , in bed. '^11 reflex activity is absent. There is no disturbance of sensation and no tendency to bed sores or to trophic changes. Elec- trical examination shows a lessening of irritability to both currents and in some cases a complete disappearance of reaction. As the child grows, the paralysis remains, though little by little slight movements may become possible, such as flexion of the arms and legs, and of the hands and feet, but such movements are extremely feeble and never are suflicient to enable the child to move with any degree of force, to crawl or to sit up. The muscles feel soft and flabby to the touch, but the skin is not cold as in poliomyelitis. There is no affection of the bladder or rectum and if the children survive the first year they are usually able to exercise a voluntary control over them. Fig. 303. Case of myatonia congenita showing complete absence of tone in the muscles of the thigh. (Kindnsss of Dr. W. Y. Spiller.) In the majority of cases thus far reported the children have died from some intercurrent disease before the end of thg third year. In those who have survived, a slight improvement has taken place, but in no case has recovery been reported. Examination of the muscles has shown an atrophic condition with infiltration of the muscle with fat and an appearance not unlike that observed in pseudo-hypertrophic paralysis. No nervous lesions have been found. In one case under my observation the child had reached the age of three years, showing no tendency to voluntary movement, and did not show a normal condition of intelligence. This child developed convulsions and died at the age of three and a half years/ * For complete record of all the cases up to date see J. V, Haberman, Amer Jour Med. Sciences, March, 1910, 768 FUNCTIONAL PABALYSIS. Another case shown in Fig. 303 was a child brought to the clinic in a state of good health and good intelligence, aged three, who had never walked, or sat up, and was able to move the hands only slightly and was not able to feed himself. There was no tone to the muscles, and the limbs were unduly flexible. The condition was stationary. MYASTHENIA GRAVIS. History. — This disease was first described by Erb in 1879 under the title of " a new probably bulbar group of symptoms." Between that time and 1893 single cases were reported by Oppenheim, Jen- drassik, Eiseulohr and others. Goldflam then analyzed these cases and drew a clinical picture, and in 1895 Jolly completed the clinical picture by discovering the peculiar electrical reaction of the muscles known as the myasthenic reaction, which is characteristic of the disease. All these writers believed that they were dealing with cases of bulbar paralysis in which either an unexpected recovery had ensued or in which no lesion had been found after death. During 1896—98 a large number of cases were reported from different countries, so that in 1891 Laquer ^ was able to give a pretty satisfactory summary of the disease, and since that time it has been universally recognized as independent of bulbar palsy. Oppenheim^ published a monograph on it in 1901, and in the same year Campbell and Bramwell collected sixty cases in " Brain." The best summary that has appeared in this country is that prepared by Henry Hun and published in the " Albany Medical Annals," January, 1904, where 117 cases are analyzed. In 1908 Palmer collected 126 cases in Guy's Hospital reports. I have been able to gather together about 250 cases from the literature up to April, 1912, and it is from their analysis that the facts here presented are obtained. Etiology. — Females are more commonly affected than males, the numbers being 148 to 92 in 230 cases. The age at which the disease appears is usually between twenty and thirty in both sexes. Cases have been recorded in children, as early as two. The oldest case on record was in a man of 72. The table shows the ages of cases col- lected. All races appear to be liable, and negroes are not exempt. The exciting causes appear to be over-exertion, over-work, fatiguing occupations, emotional excitement and some infection, particularly influ- enza. It has been noticed that the disease occurs as a complication of Basedow's disease and of goitre. It has also been seen in patients with myxoedema. It has developed during pregnancy or lactation in seven cases. In one of the ca^es pregnancy exerted a favorable effect. In several cases the termiiiation of labor has been followed by a rapid increase of symptoms. ' Volkmann's Sammlung Klinische Vortrage, No. 205. 2 Die Myasthenische Paralyse, S. Karger, Berlin, 190(>, MYASTHENIA GBAVIS. 769 Tabl,e. Age of Cases of Myasthenia Gravis. 1-5 5-10 10-20 20-30 30-40 40-50 60-60 60-70 3 1 27 78 51 38 9 7 It has been supposed to be due to some error of secretion in the ductless glands, the parathyroids and the thymus being both accused. Certain defects of metabolism, such as the deficient excretion of calcium salts in the urine and faeces, may be secondary or may act as causative factors.^ No definite causation is as yet determined. The rather sudden onset of the symptoms and a complete recovery in many cases has given rise to the theory of the toxic origin of myasthenia gravis, it being supposed that some poison developing in the body has a selective action upon the muscles, especially the muscles that are in the most constant use. Hence the early involvement of the eyes, of the muscles of the face, tongue and larynx, of the hands and of the muscles involved in walking. We know of a similar condition occurring sud- denly and passing off rapidly in the form of family periodic paralysis, though in that disease the muscles of the face appear to escape. Pathology. — The pathology of myasthenia is still undetermined. The many reports of negative findings in the entire nervous system makes it evident that the disease is not due to a central lesion, although in isolated cases minute hemorrhages in the pons and medulla and degenerations of the nuclei of the cranial nerves have been occasionally observed. Authorities all agree however that there is no uniform nervous lesion. In a certain number of cases too numerous to be explained on the theory of coincidence an enlargement of the thymus has been found after death. The glandular tissue itself is hypertrophied, and in many cases tumors of the thymus gland, fibroma and sarcoma, have been found. On the other hand, there are many cases that have been exam- ined where no trace of thymus enlargement has been observed, hence disease of the thymus cannot be regarded as an essential lesion of the disease. In the muscular system throughout the body and also in the glands and viscera of the body numerous collections of lymphatic cells along the course of the lymphatics and about the blood vessels have been observed. These have been called lymphorrhages and are so uniformly found that they may be considered as characteristic of myasthenia. Fig. 260, page 620, shows such a collection of lymphatic cells within the muscle in Hun's case. The origin of these cells is uncertain. The reason for their occurrence is unknown and their influence in pro- ducing the disease is not yet understood. But they have been found both in cases that have come to post mortem and also in muscle tissue which has been excised from living patients. Symptoms. — The disease begins as a rule with some form of stra- })ismus, either external or internal, producing double vision, which is the first thing noticed by the patient. This is often associated with 49 ' See R. Pemberton, Amer. Jour. Med. Sci., June, 1910, 770 FUNCTIONAL PARALYSIS. ptosis, but occasionally with paralysis of the orbicularis. This mode of onset occurred in 78 out of 190 cases. Weakness in the muscles of the face, tongue, pharynx, larynx and neck develop soon after the ocular palsy or may precede it. Difficulty of speech was the symptom first noticed in 33 cases; difficulty in swallowing in 16 cases and dif- ficulty in using the lips and tongue in 6 cases. The symptoms are not uniformly bilateral. The muscular weakness usually extends down- ward to the arms and hands and later on to the legs. This weakness at first consists in a general muscular lassitude and undue fatigue on exertion, so that a few movements exhaust the patient completely and make him incapable of using his hands for any length of time or for walking or going up stairs. The paralysis of the eyeballs and of the face and mouth give rise to an almost typical mask-like appear- ance of the face which is very like that seen in bulbar palsy (see Fig. 258) and hence the disease is usu- ally mistaken for this affection. It will be remembered however that in bulbar palsy the eyes are usually not affected, or if at all, very late in the course of the dis- The double ptosis and the Fig. 304. ease. Ptosis and strabismus in myasthenia gravis. Icon, de la Salpetriere, IV., 62. immobility of the face produce a rather sleepy, indifferent appear- ance, and the lack of facial expres- sion is very noticeable when the patient laughs or cries. In speak- ing the voice becomes nasal, and very often the speech is quite indis- tinct, and in fact in some cases has become impossible, the weak- ness of the laryngeal muscles be- ing visible to the laryngoscope. Swallowing is also interfered with, food causing choking and fluids regurgitating through the nose, and it is occasionally necessary to feed these patients with a tube. Sometimes dyspnoea of a severe character occurs and in the fatal cases reported failure of respiration or suffoca- tion from choking have been the causes of death. The muscles that are weak show no fibrillary tremor and do not undergo any atrophy. Therefore a patient suffering from the disease appears to be well nourished and differs decidedly from a person suffering from chronic atrophic paralysis. The muscles that are weak do not show any sign of reaction of degeneration, but if they are exercised by either gal- vanism, or faradism they show a progressively increasing lack of response to both currents so that they become inexcitable to any current, and this reaction, which is characteristic of the disease, is MYASTHENIA GBAVIS. Ill termed the^" myasthenic reaction." It is a condition of electrical fatigue exactly similar to the fatigue produced by voluntary effort. A similar fatigue can be demonstrated by percussing the muscles. There are no sensory symptoms whatever. The disease may also begin in the legs and extend upward to the trunk and arms, finally reaching the face ; or in the arms and extend to the legs. This mode of onset occurred in 58 cases. lu some cases it has been found that the muscles of the heart, bladder and the accom- modation of the eye and sphincter of the iris, have been affected as well •as the striped muscles, giving rise to symptoms of heart weakness and a difficulty in the control of the sphincters, but as a rule these symp- toms are absent. Various neurotic disturbances and hysterical attacks and conditions of mental depression in consequence of long-continued illness have been noted in various cases but are not essential parts of the disease. The course of the disease is a variable one and shows very marked irregularity in different individuals. The onset is rather sudden and the symptoms of general weakness and of bulbar palsy may develop within a few days or two weeks. As such a rapid course is never seen in a true bulbar paralysis the diagnosis in these cases is comparatively easy. In other cases, however, there is a much slower onset, but in any case within a month of the onset the general feebleness is well marked and the patients are confined to bed. But the rest in bed very often has a beneficial effect so that after two or three weeks the paralysis gradually disappears and the patient may get about again in comparative comfort and remain quite well for months. Thus, one patient under my care, a young girl whose ocular palsy had remained stationary for several years, has been confined to bed for three months at a time at different periods during the last three years, but is now quite able to be out and to walk about. In some cases during the period of greatest weakness, the muscles become quite flabby and even atrophic, but grad- ually regain their strength and form after long resting. This was the case in the patient just described, and in her case during the period of intense weakness when she could hardly turn over in bed, the heart sounds were distinctly feeble and the pulse irregular and rapid, show- ing that the heart was involved. She has never lost control of the sphincters. In another case under my care the weakness of the facial, ocular and tongue muscles was extreme, but the arms and legs were never suffi- ciently paralyzed to compel the patient to go to bed or to cease to feed himself. In this case the laryngeal muscles were the first to be affected and the imperfect speech followed by difficulty in swallowing and then by paralysis of the tongue oh one side with marked deviation were the most prominent symptoms. In one woman of 40, a sister in a convent who had done a great deal of hard work, tlie disease began with difficulty in the legs and weakness on going upstairs and in rising from a chair. This was followed by some weakness in the back, so that she could not lift heavy 772 FUNCTIONAL PAEALYSIS. objects, and within a month the arms were decidedly affected but not entirely paralyzed. She could feed herself but could not lift heavy objects. At the end of six weeks double vision developed, with external strabismus and a slight difficulty in talking. All these symptoms sub- sided gradually under rest but recur at any time that she attempts any labor, so that she remains. in a state of invalidism. The difficulty has been present for six months. The cases in which the symptoms have been limited to the muscles of the head have been contrasted with those in which the muscles of the entire body are affected and an attempt has been made to distinguish thus two types of cases, but it is probable that in every case there is some degree of weakness in all the muscles. In some cases complete recovery has been reported, which has remained for several years before the report was made. Diagnosis. — The diagnosis is made from the rapid development of double vision with ptosis of one or both eyes, difficulty in speech and in swallowing and a weakness of the muscles of the face, producing a typical lack of expression under varying emotions. Secondly, from the development rapidly of a condition of extreme weakness in the muscles of the limbs and in many cases of the head and neck. Thirdly, by the very rapid fatigue in all muscles on any exertion. Fourth, by the appearance of the electrical myasthenic reaction in the muscles, and lastly by the variation in the intensity of the muscular weakness and its prompt improvement under rest. Negative symptoms of equal importance are the absence of fibrillary tremor in the muscles affected, in the absence of muscular atrophy, in the absence of the reaction of degeneration and the absence of sensory symptoms. The diagnosis from bulbar paralysis can be made when the following points are considered. Bulbar palsy begins either as the result of an apoplectiform attack followed by permanent symj)toms or much more commonly by a slowly advancing paralysis beginning in the tongue, lips and larynx and advancing after several months to the face, but rarely affecting the motions of the eyes. In a bulbar paralysis the muscles that are weak soon atrophy, so that the tongue is very atrophic and is the subject of fibrillary twitchings and the face appears very thin. In bulbar palsy the orbicularis palpebrarum is frequently affected and the patients cannot close their eyes. Ptosis is infrequent, while in myasthenia, ptosis and ocular palsy are the first symptoms in more than 60 per cent, of the cases. In amyotrophic lateral sclerosis the symptoms may begin with a bulbar palsy, but here the history is that of a chronic, slowly advanc- ing bulbar palsy such as that just described, and when weakness develops in the limbs it is attended by spastic rigidity, by increased mechanical excitability in the muscles and a marked increase in the reflex activity. The rapid tiring of the muscles under exertion and the myasthenic reaction in the muscles to electricity are not observed in amyotrophic lateral sclerosis. Chronic atrophic paralysis beginning in the legs or arms, and, advanc- MYASTHENIA GHAVIS. 773 ing slowly^ might perhaps be mistaken for the type of myasthenia which begins in the limbs. But this disease is slowly progressive and does not recover under rest in bed. Muscles that are affected undergo a progressive atrophy and they do not show the myastheuic reaction. The fact that many of these patients are rendered markedly nervous by their long-continued illness and the fact that the symptoms of the disease tend to vary in intensity from week to week and show great improvement under rest has led to the diagnosis of hysteria in some cases. Hysterical weakness, however, generally improves after exercise and does not disappear after rest. Hysterical ptosis is due to a spasm of the orbicularis, never to a paralysis of the levator palpebrse. Stra- bismus is never present in hysteria unless congenital. The difficulty in deglutition in hysteria is caused by a spasm of the throat and liquids are actively rejected through the mouth, while in myasthenia the paralysis of the throat leads to a regurgitation of fluid through the nose. In hysteria the difficulty in talking takes the form of a whisper, articulation itself being good, while in myasthenia the speech is slurring and indistinct, and is manifestly due to a weakness of the muscles of the tongue and face. Paralysis of the vocal cords is not seen in hysteria. Dyspnoea, if it occurs in hysteria, is attended by rapid, shallow respirations and an occasional spasm of the diaphragm. In myasthenia the weakness of the respiratory muscles leads to continued difficulty in breathing, cyanosis and accumulation of mucus in the throat. In hysteria there is no myasthenic reaction in the muscles. A post-diphtheritic paralysis may present some symptoms resembling myasthenia, for in that condition the ocular muscles may be paralyzed and also the muscles of swallowing. The history of the onset after an attack of sore throat, the very rapid development of the paralysis after diphtheria, the absence of ptosis in diphtheria and also the absence of any paralysis of the face or mouth, the loss of knee-jerks in diphtheria and the appearance of ataxia in the limbs and the fact that in diph- theritic paralysis rest does not improve the condition, are the points of differentiation. Cerebral tumors of the pons and crura may give rise to local symp- toms somewhat resembling those of myasthenia gravis, but in that con- dition the onset of the symptoms will be much slower. The headache will be much more constant and severe than in myasthenia, vertigo and vomiting will occur early, and the early appearance of optic neuritis will make the diagnosis of a tumor clear. Prognosis. — It is difficult to give a satisfactory prognosis in myas- thenia because of the fact that cases run a most irregular course with rapid improvement under rest in some cases and with very little im- provement under any treatment in others. A rapidly fatal termination within two weeks has been observed in about 5 per cent, of the cases recorded ; in 45 per cent, of the cases a fatal termination has taken place within a year of the beginning of the symptoms, the course of the case being one of slowly increasing severity of weakness and paralysis with remissions or intermissions of shorter and shorter duration. In 774 FUNCTIONAL PAEALYSIS. 40 per cent, of the cases a state of chronic invalidism with intervals of marked improvement and of apparent recovery followed by relapse, every six months or a year has been the history. In the remaining 10 per cent, of the cases so far as known a permanent recovery has taken place. The prognosis appears to be better in the cases in which the onset is in the limbs than in the cases where the onset is in the eyes and face. The prognosis in the bulbar cases is better when the muscles of swallow ing and respiration are not invaded by the weakness. It is to be remembered that in fatal cases death has been due either to choking from inability to swallow, from suffocation or from respiratory paralysis. The less severe the symptoms and the shorter the attack of weakness the better the prognosis. But cases have been recorded where after a short and slight attack followed by great improvement a second attack has led to a fatal termination. Treatment. — Absolute rest in bed with the eyes closed in a quiet room where the patient will not talk is essential. But the patient's mind may be diverted by reading aloud or he may be so propped in bed to allow of his own reading, the book being held by a bed-rest. The confinement to bed need not be absolute, but the majority of the patients do better if rest is insisted upon. It IS important to maintain the nutrition in every way by all forms of food and by digestive tonics if they are needed ; the bowels should be kept open by laxatives or by abdominal massage and irrigations. There is no known antidote to the poison of the disease. Organic extracts, thyroid, parathyroid, thymus and pituitary have been tried in many cases, but the results are so contradictory that no statement can be made in regard to actual benefit derived from them. The con- tinued use of calcium lactate in 15-grain doses three times a day has appeared to be of some benefit in a few cases. Strychnin has been used by almost all observers, and some report benefit, while others believe that it does no good. Gentle massage may be used to keep the circulation and nutrition in good condition while the patient is in bed, but the muscles should not be exercised either by Swedish movements or by electricity. FAMILY PERIODIC PARALYSIS. This disease is characterized by the rapid development in the course of a few hours of a general paralysis of all the muscles excepting those of respiration and deglutition, lasting for a few hours or at most for three days and subsiding gradually, so that within a short time the individual is in the usual state of perfect health. The fact that such cases appear to be distinctly hereditary m the majority of instances, individuals in three or four generations of the family having been affected, and the fact that the disease has a tendency to recur at frequent intervals, has led to the name family periodic paralysis. History. — Cases of sudden onset of paralysis of short duration were FAMILY PEEIODIC PARALYSIS. 775 described by Gibney and Buzzard in 1882 and were thought to be malarial because of their rapid recovery under quinine. The first case of this disease was probably reported by Westphal in 1885, who likened his case to that of Gibney, but affirmed that there was no malarial element in it. From that time onward isolated cases were reported, until in 1898 E. W. Taylor ^ was able to collect about fifty cases of the affection and since that time I have found records of about fifty more. Symptoms. — The disease is undoubtedly due to the sudden develop- ment in the blood of a poison which acts very much as curare acts upon the muscular system, suspending its action completely, so as to produce complete paralysis of a flaccid type, and this is attended by an abolition of the reflexes and by a complete loss of electrical excitability, so that neither faradic or galvanic current will produce a contraction of the muscles at the height of the period of paralysis. The sensory side of the nervous system is in no way affected. In the majority of cases the bladder and rectum preserve their contractility; in some of the cases however it has been necessary to empty them mechanically during the attack. The facial muscles are rarely paralyzed, but the voice becomes weak and swallowing may be difficult. Eespiration con- tinues regular during the attack but a voluntary long breath is im- possible. Consciousness is preserved throughout and the mind is not affected. Patients suffering from this disease have attacks of varying severity. Sometimes there is a general feeling of weakness with intense fatigue on exertion not amounting to an actual paralysis. In other cases the patients have to remain in bed but can move some muscles. In the more severe type the paralysis is complete. It is known to develop usually at the time of puberty and lasts indefinitely throughout life. Goldflam has reported several cases in families. J. K. Mitchell and Flexner have made numerous observa- tions with regard to the analysis of the blood, urine and faeces, but have not succeeded in isolating the poison producing the disease. Several poisonous substances have been isolated from the faeces in normal individuals, which, when injected into the veins of animals, will produce temporary paralysis similar to that produced by curare, but in these cases of family periodic paralysis no one of these intestinal toxines has been as yet discovered. The only treatment that can be sug- gested is the free use of laxatives and purgatives as soon as the attack begins and in my case the patient had come to the conclusion that such treatment if begun early enough would limit the duration and severity of the attack. In a few cases the persistent use of alkalies, especially citrate of potash, has appeared to lessen the frequency of the attacks. The following case from ray clhiic has been carefully studied by Dr. C. E. Atwood, to whom I am indebted for the history. It is a typical case. The patient is a young man, aged eighteen, a Russian Hebrew. There were nine cases in patient's family, in four generations, all on his * Journal of Nervous and Mental Disease, 1898, vol. xxv., p. 637. 776 FUNCTIONAL PAHALYSIS. Fig. 305. mother's side, and transmitted through both males and females. These comprise brother, mother, mother's brother, two of grandmother's sister's grandchildren, two of great-grandmother's sister's children, and great-grandmother's brother ; all being typical cases. Maternal grand- father had asthma ; mother migraine. Second cousin choked to death during an attack, from vomited matter which he was unable to clear from the throat. Another died in an attack when a vein was opened to obtain a specimen of blood. The brother , 21, has had but one attack, at the age of 19. He awoke with complete flaccid motor paral- ysis lasting about an hour ; did not fully re- cover all day. The mother, 41, has had four severe attacks, viz., at the age of 17, 19, 32 and 37. Each attack lasted 24 hours, and aifected all four extremities, trunk and neck. She awakened each time completely paralyzed. Has had milder incomplete attacks, once to four times a year. In these, legs are usu- ally affected ; sometimes also the arms ; some- times only the fingers. All attacks have fol- lowed errors in diet. The affected uncle, 45, has had three attacks at intervals of about five years. A second cousin has attacks about once or twice a year, following over- eating on certain holidays. Another distant cousin had attacks for ten years at intervals, early in life, but lived to be seventy. For a year he was under continuous observation in Vienna. Attacks of all these relatives began early in life and ceased at or before the cli- macteric. The patient had measles only ; no convul^ sions ; no hysterical stigmata ; is bright and active. Health good except for constipation, large tonsils, and adenoids. Muscular build. Large quadriceps, es- pecially vasti externi, suggesting an associated dystrophy. (See Fig. 305.) This has been found in a few cases by others. Dystrophies in these cases must be associated conditions, as the paralysis is never per- manent. There was no dystrophy in the other cases of paralysis ex- amined in this family. That it should appear at all in a disease at- tended by flaccid paralysis is interesting. First attack of paralysis at 4; second at 12; the third at 14. Since 14, severe attacks at one time once a week, every Thursday ; later, once in three to six weeks ; minor attacks frequently. Severe attacks are typical, comprising a flaccid motor paralysis of the legs, arms, trunk and neck, without apparent involvement of the cranial nerves. Special features in this case : chronic constipation. Attacks follow errors in diet and emotional excitement. Attacks are Case of family periodic pa- ralysis. Notice "hypertropliy of thigh muscles. (Kindness of Dr. C. E. Atwood.) FAMILY PEBIODIC PARALYSIS. 777 more frequent in winter. Severe exercise does not produce attacks. Prodromal symptoms often occur, e. g., feeling of heaviness and weak- ness in the legs. Onset of attacks is always between midnight and dawn ; patient awakens paralyzed. If paralysis is at first incomplete, proximal muscles of legs are first paralyzed, then arms, then trunk and neck. Muscle and nerve irritability is lost ) reflexes, deep and super- ficial, are lost. These all return with return of power. The heart has a temporary mitral systolic bruit. Rectal sphincter is involved only in severest attacks. Then micturition is difficult, and the voice is less strong. Swallowing is accomplished ; but everything taken on first day is vomited. Vomiting, coughing and sneezing are difficult. There is no pain ; no disturbance of consciousness ; no parsesthesise ; no dis- turbance of sensation or muscular sense. The intensity of paralysis varies. Improvement may occur with subsequent greater onset arid return to complete paralysis. Duration of severe attacks, from a few hours to three and a half days. Erections lasting about five minutes occur in severe attacks, sometimes two or three times, causing em- barrassment when turned in bed. Moderate sweating occurs, but there is no thirst. If placed in erect or sitting posture patient faints. Urine analysis during attacks showed increased acidity, increased indican, a little albumin, increased sulphate excretion. Bacteriological study of faeces showed marked intestinal infection and fermento-putrefaction due to a clean-cut infection of the bacillus aerogenes capsulatus. Same was found in aifected mother and brother, in lesser degree ; and also, to a slight extent, in father, unaffected by the disease. In the intervals between the attacks there are no symptoms of any kind. Treatment has been by elimination. Mild diuresis by potassium citrate, mild catharsis, hygiene, and increased meat diet, have apparently rendered attacks milder, less prolonged, and less frequent. Attacks are prob- ably due to circulation of some toxic agent acting upon the muscles. CHAPTER XLV. THE SPASMODIC NEUROSES. MYOCLONIA. Chorea. Huntington's Chorea. Nodding Spasm. There are many forms of fuuctional nervous disease in which spasmodic contractions of the muscles form the prominent symptom. These have been grouped together and termed the myoclonia, myotilonus meaning a muscular twitch. Fibrillary twitching in a muscle has been called myokimia. A sharp distinction has been drawn between twitch- ings which occur in a single muscle or group of muscles causing a motion which cannot be voluntarily produced ; and twitchings which cause a movement which appears to have a voluntary conscious char- acter with an object and involves a certain co5rdinatcd action in the muscles affected. To the former the term spasm has been limited. The latter are called ties. This distinction will be observed in con- sidering the various types of myoclonia. CHOREA. Chorea minor, Chorea of Sydenham, or St. Vitus' Dance is a func- tional nervous disease, characterized by sudden rapid twitchings of any or all of the muscles, by some deficiency in the control of the muscles which twitch, and by mental irritability. Etiology. — Out of 2,239 cases treated in my clinic, 1,492 were females and 747 were males.' In the following table the age at the cime of onset is shown : Table. — Age of Onset of Chorea. 3 or under 4 5 6 7 8 9 10 11 11 26 60 111 134 190 201 197 167 12 13 14 15 16 17 18 19 20 20-30 148 171 135 86 67 47 34 16 19 34 It will be seen that the period from six to fourteen is that of maxi- mum liability, and that at the time of puberty (thirteen) there are more cases than just before or after. These statistics agree with those of other authors. No age, however, is exempt, as chorea does occur in adult life, especially in women during or after pregnancy. While children in all classes of the community are subject to chorea, a large majority of the cases are found among the lower classes, espe- cially among children living in tenement houses, under bad hygienic ^The statistics in this article have been collected by my clinical assistant, Dr. S. P. Goodhart. 778 CSOHEA. 779 surroundings and eating poor and badly cooked food. When well- nourished and well-fed children are affected it is usual to find some hereditary factor or some fright as the cause. Anaemia is a most frequent cause of chorea, many of the patients being pale and languid, and having functional or blood murmurs. The blood count usually shows a deficient number of red corpuscles and a low per cent, of hemoglobin, but this is not always present, even in patients who look pale.^ There is some evidence of heredity to be found in the histories, 334 of the children's parents having, had the disease in early life among my cases, i. e., 7 per cent. In 93 cases a brother or sister was simulta- neously affected. There is no definite relation between the infectious diseases of child- hood and chorea, and it rarely occurs as a direct sequel of these diseases. The relation of chorea to rheumatism has been studied by many writers. Many cases of chorea develop after an attack of acute rheu- matism, with or without endocardial complications. In some cases the diseases appear together ; in some they occur alternately. In a collec- tion of 2,500 cases made from various authors,^ I found that 26 per cent, had had an attack of rheumatism just preceding the chorea, and that 23 per cent, had a cardiac murmur. Among my own cases 372 had an attack of rheumatism preceding chorea. I do not consider the in- definite muscular pains, called " growing pains," of cliildren a positive evidence of rheumatism. Many patients complained of such pains before or during the attack. The statement may be made that a certain poison or microorganism in the blood under certain conditions produces either rheumatism or endocarditis or chorea, according to the varying susceptibility of the joints, heart, or brain in different persons. In 430 of my cases a cardiac murmur was found at the base of the heart in systole, or at the apex in systole. This was in sonle cases a blood murmur, and passed away when the disease subsided. In the majority of my cases (251), however, it has been a true endocardial murmur and has remained, leaving the heart permanently defective. It is this class of cases which has established the relation between endo- carditis and chorea. In a very large proportion of my cases no car- diac murmur was found. Chorea is a disease of the temperate climates and is almost unknown in the tropics. Negroes are rarely affected, but are not exempt. The occurrence of chorea chiefly in the spring months between March and July, which has been noticed by many authors and which my records confirm, is probably due to the fact that at this time the confinement of the children in the house in bad air, the inability to obtain out-of-door exercise in winter, and the mental strain of school result in a culmination of the malnutrition which is the chief predis- posing cause of the disease ; while the lesser number in summer and fall indicates a better nutrition of children who have had an out-of- ' TJniverHity Medical Maj^azine, December, 189f>. ^See article, Chorea, American Texthoolc of Disca.ses of Children, pp. 481-2. 780 THE SPimODlC NEUnOSES. door life. This is confirmed by the fact that such life has a great cura- tive influence on the affection. Fright is an exciting cause of chorea in many cases. Any mental excitement, such as long continued strain at school, sudden disappoint- ment or grief, may also produce the disease. The fright or shock may precede the disease a few days, or even a week. While it is possible that eye-strain from unbalanced muscles, or irri- tation of the naso-pharynx by adenoids or polypi may cause local twitching of the eyelids or face, they never cause true chorea, and treatment of the disease by the relief of muscular insufficiency of the eyes is useless in my experience. One attack of chorea predisposes to another, and in a large number of cases relapses occur. This is probably merely because the cause which produces the first attack is renewed after recovery. In one quarter of my cases relapses were recorded. Symptoms. — The movements occurring in chorea are spasmodic, unexpected, and inimitable. They cannot be arrested by the will for any length of time, but are much increased by attention, by excitement, or by any effort to restrain them or to use the muscles involved. In the majority of cases the twitching is momentary and slight, and does not exhaust the patient. In a few cases they are extended, violent and continuous, endangering the patient's safety and even his life. These movements interfere with voluntary acts, rendering them imperfect, awkward, excessive, or even impossible. When chorea is slight, such acts as dressing, writing, or playing the piano may reveal irregular motions not noticeable in a state of rest ; and often it is this unusual awkwardness in the performance of these acts, or nervousness, which attracts attention to the condition. When the disease is fully devel- oped, any movement involving fine coordination is impossible. While any muscle of the body may be involved in choreic movements, it is more common to notice them in the face and extremities than in the trunk. The child makes queer grimaces, especially when talking. The eyes are suddenly closed or opened, the mouth pouts, the cheeks twitch, the tongue if protruded is seen to be affected, and may be suddenly withdrawn, or even be cut by an unexpected snapping of the jaws together ; occasionally the laryngeal and respiratory muscles are affected, breathing is irregular, and noises are made in the throat. The neck is not as frequently affected as the shoulders, but the arms below the elbows are almost always involved, and irregular awkward motions of the fingers are always seen or felt if the hands are held. While the trunk muscles do not often appear to swell out in contraction, yet the entire body is uneasy and frequent changes of position are seen. The legs below the knees are in constant motion, but the thighs do not often twitch and the patient rarely falls, though he may stagger in walking. These motions cease during sleep. The iveakness in the muscles affected occasionally becomes so ex- treme that forcible movement is impossible, and paralysis may be sus- pected. The hands may be not only awkward but so weak that the CHOBEA. 781 acts of feeding or of dressing may be impossible, and the legs may give out so that the child may fall in walking ; the child may even be unable to turn over in bed. The weakness subsides as the child recovers and permanent paralysis is never observed. But when great feebleness develops and difficulty in talking and in swallowing occurs the prognosis is serious. I have known the inability to swallow to result in such a condition of malnutrition that the child died. Awk- wardness and ataxia are always noticed. The disease might be con- sidered as muscular in origin were it not that it is always attended by mental irritability and is often unilateral. Among 2,239 cases 770 were unilateral, the right side being affected a little more frequently than the left. If the disease begins as hemichorea it rarely becomes general. If it has once occurred as a hemichorea, it usually recurs as such. The mental irritability is usually noticeable early in the disease. It may be accompanied by inability to exert the mind continuously, and by depression of spirits. The child frets, is easily irritated, is quarrel- some, when previously he has been good tempered, cannot be amused, and is said to be naughty when in reality he is unable to exercise self- control. He may even act in a semi-imbecile manner, laughing too easily. These children are always incapacitated for study and should be taken out of school. The mental excitement may interfere with sleep. A child who is suffering from chorea is usually pale, badly nourished, has little appetite, is languid, is constipated, passes but little urine, and that of a high specific gravity, loaded with phosphates and urates. Examination often shows a loud systolic heart murmur which may be either functional and due to anaemia, or organic and due to endo- carditis. There is often obtained a history of muscular pains or of an attack of rheumatism, preceding or coincident with the onset of chorea. Headache is often complained of. There is usually a diminution of the tendon reflexes and a hyperexcitability of the muscles to electrical stimulation. Temperature, pulse and respiration are natural. The disease appears suddenly after a fright or after some mental strain or from general malnutrition, increases during the first two weeks, lasts for several weeks (ten is the average) and gradually sub- sides, but may recur a year later at the same season at which it first appeared. This description applies to the majority of cases of chorea. There are exceptional cases which require notice. In a few instances the motions are constant, excessive and violent, so that the patient will be thrown off a chair or out of a bed, and is liable to incur injuries. Unless these patients are protected by lying in bed and guarded by mattresses at both sides of the bed, and are kept asleep by narcotics, they are soon worn out and die of exhaustion. In a few cases the mental irritation rises to the pitch of acute mania, and acute delirium occasionally occurs in this form of the disease. In some cases the weakness is so much more apjiarent than the twitching that the case may be considered as one of paralysis. Usu- 782 TRE SPASMODIC NEVB0SE8. ally ataxia attends this weakness. I have known chorea to be diag- nosticated as infantile paralysis, and as locomotor ataxia, though other characteristics of these diseases were absent. In a few instances speech becomes affected early, and may be so indistinct as to be scarcely understood ; and in these cases grunting noises may be made in the throat. Sometimes nervous patients affected with twitching motions give vent to loud words unexpectedly, usually of a profane or obscene kind. This condition, known as coprolalia, is not choreic, but hysterical. So too is echolalia, in which the patient repeats the last word heard. Such patients often mimic motions and show other signs of hysteria. The joints may be inflamed, swollen and tender, when rheumatism attends chorea. In a few cases subcutaneous nodes, which are small, round, hard nodules appearing in many parts of the body, notably on the back and along the flexor surfaces of the extremities, are found. They are regarded as evidence of rheumatism, and have no relation to the chorea. Chorea occurring in adult life presents the same symptoms as in childhood, but is usually attended by much greater mental irritability and by marked depression of spirits ; in fact, a true melancholia not infrequently develops and there is danger of suicide. It may last three or four months and occasionally becomes chronic. Chorea is some- times seen during pregnancy. It develops in neurotic women during the third or fourth month and may be very severe and last for several weeks. It also occurs just after labor and is then attended by melancholia. Duration. — The duration of the disease varies greatly in different cases. In some it runs a rapid course and the child is well in a month. In the majority of cases it lasts about three months, passing away slowly. In some cases it lasts a year or even more, with vary- ing degrees of severity. Occasionally it becomes chronic. The large majority of the cases terminate in recovery, but there is always danger of a relapse, and many patients suffer from a third or even fourth attack, which usually occurs at the same period of the year at which the first seizure occurred. When a child dies of chorea it is because of exhaustion on account of the severity of the spasms. In the cases appearing during pregnancy, it may cease after the third month, or it may last throughout the pregnancy. In cases developing just after labor it is often severe, but not of long duration. In a few of these severe cases it is attended by mental symptoms, mania or melancholia, and may be fatal. In adult life chorea is apt to last two or three months, and if attended by melancholia, as it often is, may prove fatal. Pathology. — There is little ground for positive statements regard- ing the pathology of chorea or regarding the situation of the lesion. The lesions found by various authors ; minute hemorrhages or capil- lary emboli of the basal ganglia of the brain; vacuolization of the nervous tissue due to distension of lymphatic spaces around blood- vessels or nerve cells ; various types of degeneration in the cells of the CHOBEA. 783 cortex or spinal cord ; hyperplasia of neuroglia, are probably due t» the exhaustion produced by the disease. The unilateral character of the symptoms points to the brain as the site of the functional disturbance, and this is confirmed by the mental symptoms usually present. Prognosis. — This should be favorable, but guarded as to duration. The chances all favor recovery within three months, but it is to be remembered that some cases last much longer, and that many relapses occur. Another fact makes a guarded prognosis necessary. Some children are exceedingly susceptible to arsenic and hence cannot take the chief remedy ; or if it is given they develop arsenical poisoning, or even arsenical multiple neuritis. The more anaemic the child, the worse the prognosis. The more the mental excitement, the worse the prognosis. The development of rheumatism during the disease does not render the prognosis very grave, as few children die of rheumatism. It lengthens the duration of the chorea. The occurrence of endocarditis is more serious, as it may last through life and finally lead to serious cardiac disease. During the early stages, however, the cardiac insuf- ficiency rarely causes symptoms, and I have rarely seen a child with chorea suifer from oedema or dropsy. Nor have I seen a child with chorea develop hemiplegia from cerebral embolism. Diagnosis. — There are many diseases in which the chief and most prominent symptom is a twitching of the muscles. These should not be mistaken for chorea. Tic convulsif, which is a unilateral twitching of the face, is not chorea, as shown by its local limitation. (See Chapter XLVI.) Other forms of tic, such as tic of the head or shoulder or arm, are not to be mistaken for chorea, because they are strictly limited in the extent of the twitching, and have in distinction from choreic move- ments the characteristic of a tic, viz., that it is a simple act originating in an idea and coSrdinated for a special end, but controllable momen- tarily by eiFort. The maladie des tics convulsives of Gilles de la Tourette, consisting of a sudden convulsive twitching of any or all of the muscles of the body, resembles chorea, but may be distinguished from it by the facts that it is not attended by weakness or awkwardness in voluntary movements, the twitching is not increased or set up by voluntary motion, and only appears during rest ; there is no mental irritability ; the disease does not usually begin till puberty ; it is chronic and does not yield to arsenic. Habit tics should not be confounded with chorea. All children have a tendency to mimicry, and a child who is afflicted with habit tic makes motions which have the character of voluntary move- ments such as winking, pouting the lips, frowning, raising the eye- brows, turning the head, shrugging the shoulders, or moving hands and feet. These habit tics are not as quick and jerky as the twitchings of chorea, but after a time they arc not easily controlled, as they are at first, and hence resemble chorea. Voluntary control can, however, be exercised if the child's attention is forced to the necessity of it, and 784 TEE SPASMODIC NEUBOSES. in this condition moral treatment and general hygienic measures, such as baths, exercises and massage are of more service than medicine. Myoclonus multiplex is a spasmodic affection resembling chorea. But in this disease it is the muscles of the body and of the proximal portions of the limbs which are affected ; the face, hands, and feet are quiet. The spasms are bilateral and symmetrical ; they occur at intervals and are rapidly repeated, as many as ninety contractions of the muscles in a minute may occur. The movements are very violent, so as to throw the patient down if walking, or to hurl him off of a chair. The spasm can be brought on by tapping the tendons. During the interval between the spasms, a fibrillary tremor of the muscles may be seen. The disease may occur at any age, but is usually seen in adults after some fright or anxiety, in persons of a hysterical type. The hemichorea following hemiplegia is characterized by slow, irregular ataxic motions, excited only by voluntary effort or by emotion, and is rather a hemiathetosis than a true chorea. The history of its onset, following an attack of hemiplegia or some form of apoplectic stroke, distinguishes it clearly from ordinary hemichorea. Treatment. — Remove the patient from the bad hygienic surround- ings which have caused the disease, and send him to the seashore, if possible, for a life out of doors. Keep him quiet and without excite- ment in any case, and always take him out of school. Give him a nutritious diet of varied character, without restrictions, and aid digestion by a bitter tonic, by iron, and by laxatives. Long continued (half-hour) baths at temperature of 95° are to be given twice a day, the child being allowed to play in the water. No cold shocks are to be given. Rest in bed or on a couch is a good thing with older children, but a young child who will not rest should be dressed in merino and allowed to play on a bed, but not to run on the floor. Gentle massage of the entire body may be given daily for an hour. The child should see only one or two members of the family, so as to be kept free from excitement. It may be amused by reading. These means alone will result in improvement in two weeks. The improvement can be hastened by medicines. If rheumatism has preceded or attended the chorea, it is best to use salicylate of soda, salicin, aspirin, or oil of wintergreen freely. They may be combined with antipyrin or exalgin in small doses. If heart tonics are needed, camphor or caffeine are the best. The doses of all these drugs must be determined by the age and susceptibility of the child. If there be no history of rheimaatism, it is well to think of the pos- sibility of malarial infection as a cause of chorea, and periodical rises of temperature will indicate a mercurial purge, followed by the use of quinine or of Warburg's extract for at least a week. This often cuts short an attack of chorea. In all other cases arsenic is the chief remedy. I prefer Fowler's solution* It is to be begun in 3-drop doses three times a day, and in- creased 1 drop daily until a puffiness of the eyelids, nausea, or pain in the stomach are produced. Some children will stand only 10 drops a EEBEDITABY CHOREA. 785 day, others will take 40 drops a day without discomfort. It is my rule to increase the dose till physiological effects are produced, then to stop it for twenty-four hours, and then to give the dose just below the maximum for some days or even weeks, always watching for poisoning, and stopping if this appears. It should be begun well diluted and be given after meals only. If a child cannot take arsenic, chloral hydrate in 2 or 3-grain dose for a child of six may be given 3 or 4 times in 24 hours. This may be combined with bromide of sodium in 5 or 10-grain dose, and given after meals. The condition of the heart is to be watched carefully. Chloretone in dose of 5 grains three times a day for a child of six has been of service in some cases. The dose may be increased in fre- quency in older children. In some cases tincture of cimicifuga in half drachm dose repeated and increased 5 minims daily may do good. This is also of service in adults. In very severe cases where the motions are violent, the patient should be guarded from injuries by being kept in bed, mattresses being placed at the sides of the bed. In these cases begin with 5 or 6 drops of arsenic in milk every 6 hours, and increase the dose each time 1 drop, At the same time give bromide and chloral freely by rectum. It may be necessary to give inhalations of chloroform in these cases, to induce sleep. Sulphonal or chloralamide or veronal are also of ser- vice in these cases. Hypodermic use of morphine usually increases the spasms, but hydrobromate of hyoscine in -^^ grain, or less for a small child, may do good. In addition to these direct remedies the condition of anaemia must be combated by some form of iron. The albumenate of iron is the best form for children, as it is tasteless and can be given in milk. Chocolate lozenges containing iron may also be given. When all medicinal and other treatment fails, and the case seems chronic, a change of climate, especially to the seashore, is often of great benefit, but sea bathing is not to be recommended. High altitudes are not favorable to chorea. HEREDITARY CHOREA OR HUNTINGTON'S CHOREA. A form of chorea, developing in adult life, and distinctly hereditary in origin, was first described by Dr. Huntington, of Easthampton, L. I., after whom it was for a time named. He observed a family in which cases had developed in four successive generations. Others have recorded the history of other families similarly afflicted and now the disease is recognized all over the world. ^ The chorea begins about the age of forty, though a case has been seen as early as twenty -three. The motions begin in the hands and 'A. S. Hamilton has described twenty-seven cases, Amer. Journ. of Tnsan., Jan., 1908, and Brown inf< has collected the records of over one hundred cases from literature ; Neurographs, vol. i., No. 2, May, 1908. See also Curschmann, Deut. Zeitschr. f. Nervenheil., Oct. 15, 1908. 60 786 TEE SPASMODIC NEUROSES. feet and extend until the entire body is affected. They are excessive, so that speech, coordinated actions and gait are involved and the muscles are all in violent movement, rather more intense than in ordi- nary chorea. The spasms are increased by mental or physical exertion, but occur when the body is at rest. They cease during sleep. They are never unilateral only. After a time mental symptoms uniformly develop, and many of the cases have been observed in asylums. In some patients there is evi- dently a congenitally defective mind. In others a progressive dementia develops. The mind slowly fails, there is a loss of power of applica- tion and of judgment and of memory, and there is despondency and emotional irritability. In some cases suicidal impulses are felt. In quite a number of cases a more active type of insanity appears, delusions of persecution or of an exciting or anxious type, self-accusations and maniacal outbursts. In some the hebephrenic type of dementia prsecox has been observed. In the latest stage a gradually increasing spastic paraplegia develops, both legs and arms becoming rigid with increased reflexes and imperfect control of the sphincters. The disease may last many years. Certain associated symptoms have been noted by various observers, such as ptosis, an increase in the muscular tonicity, a slow response to painful sensations, and bulimia ; but they are not particu- larly characteristic. Males are more frequently affected than females. It may be trans- mitted through either side of the family, but Dana reports a family, where the transmission was wholly through the females. While it appears as a rule in adult life, there is a tendency for the disease to appear at progressively earlier ages in succeeding generations, and Sinkler has reported a congenital case of hereditary chorea. Hamilton observed in his cases many signs of degeneracy and of early senility. There are cases of senile chorea with dementia in which no inheritance can be traced. The lesion found is a sclerosis of the cortex of the brain scattered widely in patches especially about the blood vessels, and secondary to changes in the vessel walls. Sclerotic patches have also been found in the basal ganglia. The prognosis is bad and no treatment has succeeded in stopping the progress to dementia. NODDING SPASM. In some children soon after birth or within the first six months of life certain oscillatory motions of the head develop, usually lateral, sometimes vertical, and occasionally a mixture of both. These are very commonly associated with a condition of rotary or lateral nys- tagmus which appears when any attempt is made to turn either head or eyes. These nodding movements do not seem to incommode the child and are certainly not attended by pain. They are not unlike the nodding movements and slight rotary movements occurring in NODDING SPASM. 787 very old persons, though these are not associated with nystagmus. Nodding spasm is more common in children of neurotic inheritance and children who are badly nourished and the subject of rickets. It develops not infrequently in cases of hydrocephalus in mild degree in the early stage. It also occurs in tumors of the brain in children. The origin of the condition is wholly unknown, though in several cases when children subject to nodding spasm have grown up and have out- grown the spasm defects of vision of the nature of extreme astigmatism have been discovered. It seems, therefore, not unlikely that eyestrain may be the origin of these conditions. Little is known of the causa- tion. The spasm usually persists until the child gets to be a year old, when it is outgrown. I have rarely seen it last through the third year. As the disease is rarely seen in adult life it is evident that the majority of patients recover. There is no known form of treatment, but in these children the general health should be carefully attended to. Proper nutrition should be secured ; good air and good food, frequent bathing, and tonics, especially cod-liver oil and arsenic, are distinctly beneficial. CHAPTER XLVI. THE SPASMODIC NEUROSES. The Tics. Facial Spasm. Torticollis. Tortipelvis. Myoclonus. Myotonia. The term tic, which was at first used to indicate a lightning-like twitching of the face, and later was employed to describe all sorts of sudden spasmodic contractions of the muscles of the forehead, neck, shoulders or extremities, has of late been defined by Brissaud^ and Meige^ as "a primary act caused by an external irritation or by an idea and codrdinated for a special end." By repetition this act becomes a habit and finally occurs involuntarily without apparent cause or purpose. It may then increase in intensity and frequency and be associated with other acts. The movement is preceded by a sense of need and by an uncontrollable impulse ; to control it causes a sense of distress or anxiety, but it can be controlled momentarily by an effort. It ceases when the mind is strongly diverted from it and it is suspended during sleep. Charcot taught that it was a psychic disease. Friedreich called it a co5rdinated spasm of a remembered movement. The French School considers it a psychomotor affection. Tics are movements originally voluntary and hence initiated by a conscious impulse sent from the cerebral cortex ; and therefore have all the characteristics of a true intended coordinated action as distin- guished from a reflex or involuntary spasm. They are convulsive in character, because in the end they are wholly beyond conscious control and have no obvious purpose, but in fact become an annoyance to the patient. They have been called by some stereotyped movements. Children between the ages of six and thirteen are not infrequently found to acquire certain abnormal movements ; either by imitation of other children or because of some slight sensation which has been unpleasant. This movement is always voluntary in its character at first. But after a time it may be repeated so often as to become auto- matic and the child may be unconscious of it. Then it may become frequent, occurring every few minutes or almost constantly, and pro- duce unpleasant effects. The tic may consist of a winking of one or both eyelids, of wrinkling the forehead, of pouting the lips, of twitching the head, of shrugging the shoulders, of throwing the hands up or out, of twitching the fingers, of snapping the fingers, of catching the breath, of sighing deeply, of twitching the body, of kicking the legs, or of any other simple volun- tary movement. Some children make noises in the mouth or throat, ^ Brissaud. Tics et spasms de la face. Jour. deMed. et Chur. pract., Jan. 24, 1894. ^ Meige et I'eindel. Les Tics et leur traitment. Manon et Cie, Paris, 1902. Meige. Art. Tics. Traite de Medicine, tome x, p. 330, Paris, 1905. 788 Thb! Spasmodic nEUROSE^. 789 Of eVeH l-epeat words. Some children touch objects as they pass them. Some have impulses to count or to repeat phrases. In fact any mental or motor impulse of a morbid kind may pass into a habit. It is chiefly among neurotic children whose inheritance is bad and whose training has been imperfect and whose nutrition is not good that these habit tics develop. The exciting cause may be some slight irritation from imperfect eyesight, from nasal obstruction, from uncom- fortable clothing, from too great restraint at school, or some fright or emotional shock. It sometimes develops when the child first goes to school. The epidemic forms of chorea described as occurring in the middle ages were really tics from imitation, in persons of hysterical type. Tics are not confined to children. They often develop in adults. A tic being a physiological movement, it is evident that a coordinated group of muscles is usually involved. Every possible variety of move- ment has been observed, and to describe the forms of tic it would be necessary to mention almost every known act. Some special forms may be considered because of their frequency. Tic of the eyelids consists of a spasmodic closure of the eyes, attended by wrinkling of the forehead ; the eyes cannot be held open for any length of time and this gives the patient much inconvenience. This is not to be confounded with true blepharospasm (see page 791). Tic of the face is usually seen in the form of a grimace at first uni- lateral, later bilateral. There may be movements of the lips, of pouting, sucking, pursing, and all sorts of expressions of the face ensue, giving the appearance of depression or of mirth. Sometimes a local spasm in the risorii may occur. Thus a well- nourished young woman, not anaemic, suffered from a bilateral tic of the middle muscles of the face, so that she looked all the time as if smiling, and the upper lip was stretched tightly back across the upper teeth, and this impeded her speech, her eating, and gave her the appear- ance of laughing all the time, when she had no such desire. This con- dition came on very suddenly after a great mental shock, soon after the birth of her first baby, and persisted for six months. The chief dis- comfort lay in the great limitation of talking. There was no affection of the tongue or of the larynx, but all letters involving the use of the lips were imperfectly pronounced, especially when her attention was directed toward it. When her attention was diverted by an examina- tion of her eyes and throat the spasm relaxed and the lip assumed its normal appearance, but the moment she began to speak, or the moment that she tried to smile, the sudden contracture of the muscles resulted in a flattening of the upper lip against the teeth, where it was held rigid. She appeared to be perfectly well in every other respect ; there was a slight tendency to divergence of the eyes ; there had never been any pain or difficulty in chewing. There was no pain on pressure along the fifth or seventh nerves ; there was no apparent obstruction in the nose or throat. Sometimes it is only the corner of the mouth or side of the nose 790 TEE SPASMODIC NEUEOSES. which is raised. Sometimes the chin is drawn down. This is to be sharply distinguished from facial spasm (see page 791). Tic of the tongue may cause sucking or clicking sounds. Tic of the masseters may cause grating of the teeth. Habitual or spasmodic yawning is a form of tic. It is often uncon- sciously associated with certain places or with certain acts ; thus a person may yawn in church constantly, but not elsewhere ; he may always yawn when reading aloud, but not when reading to himself ; he may yawn in the presence of certain persons only ; he may yawn con- stantly if he is seized with the fear that he may yawn ; like all tics, yawning is produced by seeing another person yawn. Tic of the neck causes a shrugging of the shoulders or a turning of the head^ This simulates torticollis, and in fact Brissaud describes a mental torticollis which is a psychical form of wry-neck, differing in some respects from the other forms. In these cases the persuasion that some act will stop the spasm is followed by relief. Thus the pressure of a finger on the chin or on the side of the face, resting the head on the hand or on a chair will stop such a tic. The majority of cases of wry-neck are of this variety. (See p. 794.) Tics of the extremities are rare, the habit of tapping objects with the fingers, or of kicking with the feet being rather of the nature of bad habits than of true tic. Tic of respiration, of cough, and of swallowing, have been recorded, and I have recently seen a tic of the abdominal muscles, the young woman making movements of the hypogastric region constantly. Tics of speech (echolalia) in which the patient repeats the word last heard, or repeats some word over and over, are rather to be classed with hysterical phenomena. In all cases of tic the mental characteristics of the patient are to be studied, it being kept in mind that there is a psychical element in these cases. Fixed ideas, abnormal impulses, and peculiar ideas are often to be detected by questioning. This has to be particularly regarded in the treatment, suggestion and persuasion of possible relief being essen- tial to success. Course. — The usual history obtained is that of a slow onset of the tic following some mental shock or anxiety — its gradual development and then a long period without relief. During this period variations in severity from time to time occur. Sometimes there is spontaneous recovery. Sometimes treatment cures. In a few cases described by Gilles de la Tourette there is a gradual extension of the tic from one part to another. The disease begins at the age of six or eight by tic of the face ; this extends to the neck and head and shoulders, then to the hands and finally to the trunk and legs. Echolalia and respira- tory tic are added. As the disease goes on mental failure becomes apparent, the patients show queer obsessions, develop delusions and finally go on to dementia. The prognosis is fairly good when the disease begins in early life, as proper mental and physical training usually enables the child to overcome the habit. In adults the prognosis is not as good, for tern- FACIAL SPASM. 791 porary reqpvery is usually followed by relapses, and finally the disease becomes chronic. The disease does not shorten life. Etiology. — A neuropathic or psychopathic heredity can be ascer- tained in the majority of cases of tic. The disease is often directly inherited, several members of a family being aifected in different genera- tions. If it is not, a history of epilepsy, hysteria, neurasthenic im- pulses, phobias, migraine, or diabetes may be obtained, or some organic nervous disease in the parent may be found to exist. Imitation plays a part in the origin of the disease, as many children copy the motions of others. Imperfect training is also responsible for some cases. Children are very liable to begin spontaneously some bizarre motion and unless firmly corrected or punished for it will keep it up till it runs into a tic. Tics develop chiefly in children between the ages of five and ten. This is the age of greatest imitation and when habits are easily formed. Both sexes are liable. Some cases develop at puberty. Earely the disease appears in old age and then it is usually a part of a senile dementia. Treatment. — The recognition that tics are psychomotor in origin has led to the only satisfactory method of treatment. This is a method of self-control and self-education or psychomotor discipline devised by Brissaud and Meige. The patient must keep his mind on his spasm, must attempt to control it voluntarily, and in order to do this must go through regular exercises several times daily before a mirror, con- tracting the muscles which twitch and their opponents, so as to secure voluntary control of the muscles. This education of the muscles must be insisted upon and is best done in company with some one who has a firm control of the patient. Sometimes isolation from the family has a good effect on children. A system of rewards for success aids the treatment. It is useless to give medicine (e. g., arsenic), or to a°ttempt surgical treatment by division of nerves or tendons. But in view of the neurotic constitution of the majority of patients it is necessary to advise that form of life which will conduce to general improvement in health. FACIAL SPASM. A spasmodic twitching of the muscles supplied by the facial nerve is not an uncommon affection. It is usually primary, and not secon- dary to facial paralysis. All the muscles may be affected by the spasm, or it may be limited to the muscles about the eye, the orbi- cularis palpebrarum, when it is called " blepharospasm." Usually the zygomatic muscles and the levator auguli oris are affected with the orbicularis palpebrarum. When this is the case the spasm usually extends to the muscles of the chin. It is a unilateral affection at first, but may become bilateral. The slight bilateral twitching of the face sometimes seen in childhood or in hysterical individuals, the result of voluntary grimaces which have become automatic, is not included in facial spasm. Facial spasm is a disease of adult life, not usually developing until 792 TEE SPASMODIC NEUBOSES. after the age of forty years. It occurs in women more frequently than in men and much more commonly in persons of a neuropathic consti- tution or in those who are subject to migraine, anaemia, or cachectic conditions. A sudden mental shock has been known to produce it. A spasmodic contraction of the muscles anywhere in the body is rarely evidence of primary motor irritation. It is usually secondary to a sensory impulse, the contraction of the muscles being a response to such an impulse. Thus the act of winking is primarily due to an irritation of the conjunctiva, and blepharospasm can be usually traced either to some local disease in the eyelids, to some strain of the ocular muscles, or to some defect of vision. The most sudden or lightning- like contractions in the facial muscles are seen in connection with facial neuralgia, and it is probable that in the majority of cases of facial spasm the cause is to be found in some irritation in the domain of the trigeminal nerve. Thus irritation in the nose, or about the teeth, or even in the mouth or throat, or in the scalp, is sufficient to produce a facial spasm, and many cases can only be relieved by the removal of the source of irritation. It is not impossible that small sensory fila- ments of the trigeminal nerve may be the seat of neuritis without any attendant pain, and that the result may be a facial tic. In the severe spasm of torticollis it is possible for an extension of the spasm to the facial muscles to occur, so that the spasm not only turns the head, but twists the face. Cases have been recorded of facial spasm due to a pressure of tumors on the facial nerve within the skull. ^ Facial spasm has also been observed in irritating lesions of the facial area in the motor cortex of the brain, but these spasms are rarely as constant, as frequent, as sudden, or as extreme as those in true facial spasm. They are usually attended by spasms in the neck and arm and by other symptoms of cortical disease. Nevertheless, it is well to remember that a facial spasm may be the first sign of cortical epilepsy. (See page Symptoms. — The chief symptom of the affection is the sudden, lightning-like twitching of the muscles of the face. Such twitching occurs at intervals, or a series of twitches may occur rapidly, one after another, each paroxysm lasting several seconds, or even a minute. The final contraction may be a tonic one, so that the face is drawn up and is motionless for a few moments. Any attempt at looking fixedly at an object, or moving the face, or talking, or chewing is liable to be followed by a spasm, and sometimes a cold draught is sufficient to bring it on. Some patients complain that the spasm is set up by a strong light, under which circumstances it is possible that the irritation of the optic nerve is sufficient to set up a contraction. For this reason many patients protect the side of the face by a thick woollen pad, which keeps out light and cold. The spasm is never painful, and as there is no paralysis, voluntary motion is perfectly possible. There is no change in the electrical contractility of the facial muscles. In one case re- corded by Keen ^ the spasm extended from the facial nerve to the mus- ^Schultze, Virchow's Archiv, vol. Ixv., p. 385. Vuss, Neurol. Centralbl., 1886. * Transactions of the American Surgical Association, 1886. FACIAL SPASM. 793 cles of mastication, the tongue, and muscles of the neck. Occasionally a tender spot in the region of the fifth nerve or slight areas of anses- thesia may be discovered by careful examination, a fact which sup- ports the theory that the facial spasm is secondary to disease in the trigeminal nerve. The course of the case is usually a very slow one. The spasm begins in a few muscles, and extends to others until the entire face is involved, and the disease may remain for many weeks or months, and then sud- denly cease. It is particularly liable to return. Not infrequently there is no permanent recovery. The patient is liable to the spasm all his life. Hence the prognosis is unfavorable, especially if the disease has lasted more than a month. Diagnosis. — Facial spasm is not to be confounded with facial tic, for it does not have the characteristics of a tic. It is a sudden reflex act, chiefly in one or a few muscles, unconscious and not under volun- tary control. A tic is a complex act apparently with a conscious end in view and involves a co5rdinated movement which can be temporarily arrested. The facial spasm is usually a surprise to the patient. Prior to a tic the patient has an impulse to make the motion which gathers strength until he can no longer resist, and then he feels relief when the motion is made. Treatment. — Treatment should consist in a very careful attempt to discover some source of reflex irritation by examination of the func- tions of the eye, careful investigation of the throat and nose and of the teeth ; and the removal of such irritation if possible. In one patient whose entire left side of the face was in constant spasm examination showed that a marked defective visual power in the right eye had thrown all the work of vision on the left eye. This, too, was astigmatic and myopic. The proper adjustment of glasses resulted in a progres- sive cure, complete within a month, although the condition had been present for over a year. If no cause is found the disease must be treated symptomatically by hot applications to the face and by the free use of sedatives, of which bromide, cannabis indica, morphine, gelse- mium, and conium are the most useful. Electricity is of no use. Counter-irritation by blisters or cautery also fails to relieve. Stretch- ing of the facial nerve, which has been attempted, is usually followed by paralysis, but when this subsides the twitching returns. Some cases of facial spasm are of interest because of the possibility of locating the seat of irritation by means of hypodermic injections of cocaine. In two cases under my care the spasm had been of long dura- tion and had given the patients much discomfort and had failed to yield to any remedies. In neither case was there any localizable pain. Inas- much as the majority of such cases of facial spasm are due to irritation in the course of the trigeminal nerve, it was necessary to determine, first, whether the spasms were reflex, and, second, ifso, in which branch of the trigeminal nerve the irritation began. This was arrived at by injecting cocaine beneath the skin of the face successively at the point of emergence of the supraorbital, infraorbital, and dental branches of the trigeminal nerve. In the first case the injections had no effect on 794 TEE SPASMODIC NEUROSES. the spasm when the upper two branches were anaesthetized, but the injection at the mental foramen in the lower jaw was succeeded by immediate stoppage of the spasm for one-half hour, or until the local effect of the cocaine had subsided. This patient was then operated upon by Hartley, who exsected this branch of the trigeminal nerve at its exit from the jaw with the effect of permanent cure of the spasm. In the second case injection in the infraorbital branch of the nerve stopped the spasm, and here division of this branch was followed by permanent cure of the spasm. SPASMODIC TORTICOLLIS. Spasmodic torticollis -is a spasmodic contraction of any or all of the muscles moving the head upon the trunk. It consists of a quick, sharp contraction of the sternomastoid or of the trapezius muscles, causing a sharp, quick turning of the head toward one side, as if the patient were looking over his shoulder. In some cases this contraction is so sudden as to cause an almost lightning-like twitch of the head. In other cases the contraction is slower when the head is turned slowly, but none the less firmly to the side. Twitching occurs at intervals, may take but a second, and be succeeded by many other twitchings, so that the head turns eight or ten times in a minute, or it may be much slower, and then the head is frequently held for a minute or more in a fixed position. As soon as a relaxation of the muscle occurs the patient voluntarily turns the head back, only to find it jerked about again with much force and persistency. As time goes on in this affection the twitch- ings become more severe and either more rapid or more prolonged in duration. The latter is very common, so that these patients after a time find the head permanently fixed in the abnormal position, though the occasional relaxation of the spasm allows them to turn the head back occasionally. The usual form of torticollis is the one in which the sternomastoid muscle alone, or the upper fibres of the trapezius alone, or these two muscles together are affected. If the sternomastoid alone is involved the chin is elevated and drawn toward the opposite shoulder. If the trapezius alone is involved the chin is elevated to a greater degree and the head is thrown backward. If both muscles act together the entire head is turned over toward the affected side and rotated with the chin toward the opposite shoulder. The affection is liable to be of long duration, the spasm continuing for many weeks, with varying degrees of intensity. It usually relaxes during sleep. It may be produced by any voluntary effort, especially the acts of reading or looking fixedly at any object which involves the voluntary fixation of the head or eyes. As such a fixation may be a necessary act in the pursuit of a profession or work, Cruchet,^ whose studies of torticollis are the most complete, has classified these cases together and calls them the "professional type." He thus likens them to writers' cramp and other occupation neuroses. (See page 829.) He affirms that in these cases the spasm occurs only when the muscles of the neck * Cruchet. Traite des Torticolis. Masson & Cie, Paris, 1907. SPASMODIC TOBTICOLLiS. 1^5 are called ipto action by the effort to hold the head steady, as in fixing head and eyes on any fine manual work or writing. In this class of cases he says there is no spasm when the head is at rest, and he thus distinguishes this type from others. Patients sometimes find that there is some point upon the neck or head, pressure upon which will temporarily arrest the spasm, and they are inclined to produce pressure upon this point either by resting the head upon the back of the chair or lying with the support of a pillow or compressing this point by the hand. Cruchet holds that when this is so, the case must be classed with the tics, and he attempts to estab- lish a " mental type " of torticollis, in which the spasm is rather a psycho-motor act. Brissaud had previously distinguished a form of torticollis which he termed hysterical or mental because of the fact that some insignificant act would arrest the spasm by persuasion, and in such cases he found hypnotic suggestion of use. For one patient who had a serious form of spasmodic torticollis which failed to respond to any treatment I devised a mechanical apparatus containing a lever which pushed a pad against the lower part of the occiput upon the left side, which pad could be made to press more firmly by a movement of adduction of his arm, and this apparatus was worn for many months with great relief. The spasm is not, as a rule, accompanied by any pain or by any sen- sory disturbances, though the muscles may become wearied by their constant contraction and may feel tired and stiff. Occasionally there is acute neuralgia in the back of the neck or in the occipital nerve. Cruchet considers these cases as reflex in origin and cites instances in which relief of the neuralgia by division of some branch of the occipital nerve has cured the spasm. Curshmann has recorded a case due to irritation of the auditory nerve. The act of walking or talking may intensify the spasm, and any attempt at effort of the arms occasionally is attended by an increase in the contractions. Thus one patient at present under my observation is prevented from writing by the occurrence of the spasm. The muscles that are affected may be seen and felt to contract, but show no changes to the electrical reaction. The spasm may extend from the sternomastoid and trapezius muscles to any or all of the deeper muscles moving the head. The splenius is affected in many cases, and in a few cases the deeper muscles of the back of the neck are all involved in the spasm. Under these circum- stances it is not unusual for the spasm to extend to the opposite side or to affect the superficial muscles on one side and the deeper muscles on the other, which act conjointly in turning the head. Symmetrical muscles are rarely affected. If they are they usually act in harmony, producing a throwing of the head forward upon the chest or a turn- ing of it backward and an elevation of the chin. Sometimes in seri- ous cases of long duration the spasm extends to the muscles moving the slioulder, the rhomboids, the levator anguli scapulae, or the ser- ratus or pectorals; and in these cases movements of the arm are fre- quently associated with those of the head. (See Fig. 28G.) The arm 796 TEE SPASMODIC tJEUEOSE^. may be adducted and flexed, wrist and fingers flexed, or it may be thrown backward behind the body. Occasionally these spasms be- come so intense that the muscles of the back are also implicated, and the patient is no longer able to walk in an upright position and even while lying down is drawn around to one side, the curve of the spine being convex toward the unaifected side. This I have seen in one chronic case which lasted for five years and resisted all forms of treat- ment including division of the eleventh nerve. In cases where the head is thrown backward by contraction of the trapezii there is an associated movement of the frontalis muscle, and then the patient not only elevates the chin but has the appearance of looking upward forcibly, the eyebrows being raised. As time goes on the muscles that are afiected increase in size on account of their constant exercise, so that the neck becomes perceptibly larger. The course of the disease is a chronic one. The spasm begins in slight degree, increases in intensity, in frequency of attack and in duration of attack, so that within Fig. 306. three months of the onset the patient's life becomes a burden and all occupation has to be abandoned. In a few cases after a period of two or three mouths the spasms gradually diminish in frequency and cease, but they are particu- larly liable to recur from time to time, and these patients suffer from attacks of spasmodic torticollis throughout their lives. The dis- ease may occur after any illness. In many cases it continues for over a year with little intermission, some- times being moderate in degree, at other times increasing in intensity. Even the more severe cases occa- sionally recover, the spasms gradu- ally subsiding and finally disap- pearing. It is not attended by convulsions or by cortical epilepsy, though I have occasionally seen it accompanied by considerable men- tal agitation, a distinctly hysterical mental condition developing as a result of the irritation of the continuous spasm. Cases recorded by Brodie and Gowers in which the spasm ceased dur- ing an attack of insanity are interesting. There are no pathological observations to determine the exact cause of torticollis. The disease is not primarily muscular nor is it to be ascribed to any lesion of the eleventh nerve or motor nerves to the muscles, inasmuch as irritation of the motor nerves does not cause spasm. It may be regarded as reflex in some cases, like facial Tonic spasm of right trapezius, rhomboid and levator auguli scapulae, of two years' duration, following whooping-cough. SPASMODIC TORTICOLLIS. 797 spasm and blepharospasm, as the result of a sensory irritation in some one of the sensory nerves of the neck. In these cases neuralgia may precede the attack. It has been known to occur as the early symptom in spinal caries and in meningitis or tumors of the upper part of the spinal cord. In the vast majority of the cases no cause can be ascer- tained. The hypothesis of a cortical origin of this spasm receives a certain amount of support from the fact that the movements are quite similar to those produced by volitional effort, and in many cases mus- cles which lie upon opposite sides of the body, and are supplied by various nerves, join to produce the movement. The fact that the eyes are not uncommonly turned involuntarily to the side toward which the face looks and that the eyebrows are elevated when the head is turned upward supports this hypothesis. When the arm is involved the motions produced are such as would be produced in a normal in- dividual straining to move the head in an extreme position of rotation. Anyone can substantiate this fact who makes extreme voluntary eiforts to turn the head from side to side. Etiology. — The disease is more common in women than in men. It occurs in adult life between thirty and fifty years, though no age is entirely exempt. It usually occurs in persons predisposed to nervous disease by inheritance or subject to other forms of nervous affection, such as neurasthenia, migraine, or psychosis. It is not analogous to chorea nor does it follow attacks of chorea or facial tic. Occasionally it has been known to develop after a fall or an injury in which the head was forcibly twisted. It has been known to occur after exposure to cold and after an attack of rheumatism in the muscles of the neck. It has been known to follow otitis media and disease of the vestibule and labyrinth, and in very many cases some defect of vision has been discovered which indicates that eyestrain may be a factor. It has been ascribed to insufficiency of the ocular muscles and also to myopia and hypermetropia. Both these conditions undoubtedly cause an involun- tary straining of the muscles of the neck in the act of vision, and occip- ital headache. None of these causes, however, seems to me of sufficient moment to originate the disease. I am disposed to regard it as usually a cortical affection dependent upon some irritating cause acting upon the cortical centres which move the head. This irritating cause may be exhaustion from voluntary overaction of the muscles consequent upon defects of vision, and it may be considered as an affection anal- ogous to the many forms of occupation neuroses. If this theory is accepted the functional disturbance is to be located in the entire ner- vous mechanism involved in the movement of the head, the sensory elements and the motor elements being equally involved and thrown into a state of hypersensitiveness which results in slight sensations pro- ducing undue movements. Prognosis. — The prognosis in wryneck must be given with great caution, as the history of various cases shows such varied course. The more sudden the onset, the more intense the spasm, the more widespread the distribution, and the longer its duration the worse the 798 ' THE SPASMODIC NEUROSES. prognosis. The younger the patient, the less severe the affection, the greater the intervals between attacks, and the less the discomfort appreciated the more likely is recovery. Treatment. — The treatment of torticollis is unsatisfactory because we are ignorant of the cause of the disease. Various forms of nerve sedatives have been used. I have -found but little relief from large doses of bromide or from bromide and chloral. Valerianate of zinc and asafoetida have given somewhat better results, but the best effects have been obtained from large doses of tincture of belladonna, which may be gradually increased up to full physiological effect and usually cause a distinct improvement in the condition. Cannabis indica has also been of some service in the treatment of this affection. When the spasm is very severe and causes great distress opium in some form may be used, in the extreme cases by hypodermic injections of mor- phine. This usually gives considerable relief, but unfortunately leads to the morphine habit, to which several of my patients have succumbed. Hypodermic injections of atropine, ^w g^'^in increased slowly up to -^ grain once daily, into the muscles affected have given relief in some cases, but have failed in others. Electricity has no effect whatever. If it is tried it should be in the form of a mild, continuous galvanic current, as it is futile to produce contractions in muscles already in a state of spasm. Attempts to strengthen the opposing muscles by elec- trical exercise should not be made, for even the greatest voluntary con- traction in the healthy muscles is incapable of overcoming the involun- tary spasm. Counter-irritation to the back of the neck by means of blisters and by the actual cautery not uncommonly gives a certain amount of temporary relief and may be used frequently if the skin be not injured. Mechanical supports of all sorts are involuntarily sought for by the patient, and apparatus may be devised for producing a con- stant support of the head, a rod carrying a pad being attached to a corset and thus held in position. Torticollis has been treated by surgeons by division of the tendons of the muscles. This affords temporary relief, but does not stop the spasm, which returns as soon as the tendons have united. It has also been treated by division or by cutting out a portion of the eleventh nerve where it enters the sternomastoid muscle, or deep in the neck just at its exit from the skull. These measures certainly stop the spasm in the muscles supplied by the nerve divided, but very often after the division of the nerve the motion continues, and then it is dis- covered that the muscles lying deep in the neck upon the same side or upon the opposite side have originally been affected by the spasm. Keen has divided the upper four cervical nerves in efforts to paralyze these deeper muscles, an operation involving considerable difficulty on account of hemorrhage and on account of the depth to which the wound must be carried. In some cases this has given relief, but after the nerves have regenerated the spasm has returned. In a discussion before the American Neurological Association in 1897 the consensus of opinion was against surgical treatment, yet all agreed that it afforded the only prospect of temporary relief in severe cases. PABAMYOCLONUS MULTIPLEX. 799 SPASMODIC TORTIPELVIS. A spasm of the muscles attached to the pelvis, causing peculiar postures on standing and a very irregular gait, with twisting of the trunk, bending of the body and in extreme cases a tendency to walk on " all fours " has been described by various writers ^ under the names of tortipelvis, dysbasia lordotica, and dystoma musculorum deformans. When recumbent the spasms cease. On attempting to walk they appear, throwing the balance out and leading to remarkable positions and actions, which are so extraordinary as to lead to the idea of intentional simulation of disease. Several such cases have been considered hysterical, a suspicion confirmed by the success of treatment by suggestion. The symptom is not allied to athetosis, because it ceases with voluntary effort. It is not attended by paralysis, electrical change, anaesthesia or permanent deformity. Occasionally the muscles of the arms and trunk are also involved in the spasm. In Oppen- heim's cases the reflexes were diminished and the muscles seemed to lack a proper tone. The condition is to be grouped with the tics, but because of its peculiar type has received a special name. PARAMYOCLONUS MULTIPLEX. Paramyoclonus multiplex is a spasmodic affection of the muscular system, occurring bilaterally in symmetrically situated muscles attached at one or both ends to the trunk, and in muscles whose function is as- sociated with these, consisting of a series of violent clonic spasms of con- siderable rapidity and severity, occurring only at intervals ; and associ- ated with fascicular tremors of the affected muscles, persisting during the interval between the spasms. It is not accompanied by any distur- bance of sensory or motor functions, excepting by an increase of the superficial and deep reflexes. The spasms can be excited by irritation of the skin or tendons. The causation is uncertain ; fright and anxiety or muscular strain in lifting weights or walking have preceded the onset in some cases. Males in adult life are more commonly affected. Symptoms. — The symptoms are quite characteristic. The spasms are bilateral and symmetrical. They are limited to certain muscles. In many cases the quadriceps femoris and flexors of the leg, and the so-called upper arm group of muscles, the deltoid, biceps, and supinator longus, were affected. In some cases the muscles of the back were involved, and the muscles of the neck contracted. In a few cases the glutei and the face and the diaphragm were involved. In no case have the muscles of the hands or forearms, of the feet or legs been affected. The usual limitation of the spasm to the body muscles with those of the thighs and arms is very noticeable. The character of the spasm is also characteristic. It is a rapidly ^ Leszinsky, Jour. Nerv. and Ment. Disease, 1904, p. 33, Oppenheim, Neurol. Cen- tralbl., 1911, p. 1090. 800 THE SPASMODIC NEUBOSES. repeated clonic spasm occurring at intervals. In six cases, the rate of contraction has been counted. It has varied from 50 per minute to 180 per minute. In one of my cases it was about 90. It is not a sudden, single irregular muscular contraction, like that of chorea, but appears to be always bilateral and to involve several muscles of a physiological group at once, thus resulting in a series of movements, any one of which can be voluntarily made. In several cases a tonic contraction has occurred in one or more of the muscles affected, before or during the clonic seizure. In one of my cases the spasm of the diaphragm was tonic for one-eighth to one-quarter of a minute during each attack, and in the early attacks, the spasms of the back were tonic for some seconds. The clonic contractions continue, when once set up, for a varying time, from half a minute to ten minutes, and are suc- ceeded by a complete interval of freedom from spasm. This interval varies from half an hour to about one week. During the spasm itself, the resulting movements are of a very violent nature. The head is thrown about by the movements of the body, rendering the patient dizzy. The body is tossed about in the chair, so that there seems to be danger of the patient being thrown out upon the floor. If the spasm occurs while he is walking, he is quite liable to be thrown down. But this violence is not always present — for in two cases the spasms were never severe enough to cause a movement of the joints, and were only observed when the patient was stripped — being then of the nature of a fascicular twitching. In one case, such a fascicular muscular twitching was occasionally seen during the intervals in the muscles of the back and the pectorals. In the majority of the cases, any tapping of the tendons or any irri- tation of the skin was sufficient to produce a spasm. This seems to be an important point, for I am not aware that it has been observed in hysterical or choreic spasms. It is true that, in hysterical cases, cer- tain zones or areas can occasionally be found on the body, irritation of which may cause or may arrest the attack. But in this condition the spasm is produced by irritation anywhere on the skin — or by tapping the tendons at the knee and ankle — and was not associated with dis- turbances of sensation, which are characteristic of hysterical zones. The knee-jerk has been increased in about one-half of the cases. The skin reflexes were also increased in some cases. Mental excitement seems to have predisposed to the onset of the spasm in three cases. Had the disease been hysterical in nature, this would probably have been observed in a larger proportion. Voluntary effort stopped the spasm in four cases and made it worse in three cases. Had the dis- ease been hysterical, volition would probably not have influenced it favorably in the majority of cases. The spasm has ceased during sleep in four cases, but has continued in one case. In none of the cases have consciousness, motion, sensation, coordi- nation, or electric excitability been in any way affected — an important negative fact, since it proves at once that the condition is a functional neurosis, and makes it very unlikely that it is of an epileptic or an PAEAMYOCLONUS MULTIPLEX. 801 hysterical nature. In one case, which died of phthisis, a careful exami- nation by Professor Schultze, of Heidelberg, failed to reveal any lesion of the nervous system. It is evident from this review of the symptomatology that the char- acteristics of the disease are quite distinct ; that it can be diiferentiated from chorea, from hysteria, and from epilepsy. Is there any disease known which it at all simulates ? In tic convulsif, we have an affec- tion consisting of spasmodic contractions of irregular intensity and fre- quency, often attended by intervals of freedom. The resemblance to paramyoclonus multiplex is more than superficial, and has been noticed by several writers. But all seem to agree that in tie convulsif the face is usually chiefly, if not exclusively, aifected ; that the contractions are often single and unilateral, are liable to occur during voluntary motion ; that their intensity is not varied, but is quite uniform ; that the spasm is not produced or increased by external influences ; and that it is always a coordinate volitional motion which is produced. Gilles de la Tourette, it is true, has described a maladie des ties convulsif s, in which title he wishes to include those spasmodic affections described by various authors as jumping, coprolalia, myriachit. But here again there is a wide difference from paramyoclonus multiplex. It seems, therefore, as though the disease must be regarded as distinct from tic convulsif and as having a character of its own. There is a condition described by Henoch as chorea electrica which in some respects resembles paramyoclonus. Henoch says that in chorea electrica we have a combination of true choreic movements with clonic twitchiugs. The patients are quiet, and lightning-lijce twitches occur from time to time, perhaps every five minutes or more frequently, especially on the muscles of the neck and shoulders. There is also seen a twitching of individual muscles when the body is naked, not sufficient to move the limbs. These continue during sleep. The disease occurs between the ages of nine and fifteen, and is a manifestation of direct or reflex irritation of the nervous centres. The spasms affect the head, hands and feet, and implicate all the voluntary muscles, including the diaphragm and larynx, so that the patient may growl and bark. It is evident that while the character of the spasm resembles that in paramyoclonus, the distribution of the spasm is more extensive in chorea electrica. Prognosis. — In regard to the prognosis, it may be said that this is favorable. The majority of the cases have recovered quite rapidly under treatment. In some cases, however, relapses have occurred. Treatment. — The treatment which has been of most service has been the application of strong galvanic currents to the spine and neck, and the application of the anode to sensitive points in case these exist. Many nerve sedatives have been used, and also nerve tonics. The exact effect of these seems to be doubtful. In my own case, sedatives, tonics, and electrical applications had all been equally futile to arrest the attacks, but the patient had improved to a considerable degree under the varied treatment. Under galvanism to the spine, arsenic 61 802 THE SPASMODIC NEUE08ES. and chloral, recovery occurred. The hypodermatic use of arsenic in the form of cacodylate of soda deserves a trial. It is useless to discuss the nature of the disease from so few cases as are at our disposal. It has been regarded as a functional neurosis, and to this all must agree, both on account of the absence of any lesion, in one case examined by the most competent neuro-pathologist in Germany, and on account of the absence of symptoms of organic disease and the recovery of the cases. Whether it has a central origin and is produced by a hyper-excitability of the brain or spinal cord, induced by the sudden vaso-motor spasm accompanying fright or mental or physical strain, as Friedreich believed, or whether it may be a reflex spasm due to some peripheral irritation which, being conveyed to the spinal and medullary centres, produces the spasm reflexly, as another author has suggested, remains for the future to decide. MYOCLONUS EPILEPSY. This disease, first described by Unverricht, and lately studied by Lundborg, is an exceedingly rare affection combining some of the features of myoclonus with the condition of epilepsy. It is usually found in several members of the same family, though direct inheritance has not been observed. It begins in childhood and is chronic in its course. It begins as a nocturnal epilepsy the attacks at first occur- ring but seldom, but later increasing in frequency. The patient does not always lose consciousness ; but often awakens with a painful tonic or clonic and tonic cramp in different muscles. Sometimes rapid alter- nate muscular contractions occur in the extremities. Sometimes the contractions are so continuous as to make the limb rigid as in tetanus. Any attempt to move the limb increases the spasm and is painful. As these attacks increase in frequency a second stage of the disease is reached in which symptoms persist between the attacks. A tremor is always present, with fibrillary fascicular contraction in the muscles, beginning in the hands and gradually extending to involve all the voluntary muscles of the body. This tremor varies somewhat from day to day, and alternates with quick lightning-like twitches in the various muscles. The tremor stops during sleep. It is increased by effort. A general stiffness and rigidity of the muscles finally develops. The knee-jerks are exaggerated. An increase of saliva and of perspi- ration has been noted. Sensory symptoms are absent. After some years a gradual failure of mental power is observed, and hallucinations or states of exaltation or depression have occurred in some cases. The patients are finally confined to bed and die of cachexia. MYOTONIA CONGENITA. THOMSENS DISEASE. This disease, described in 1876 by Thomsen,^ who was himself affected, should hardly be included in a treatise on nervous diseases, 1 Archiv f. Psych., Bd. vi., s. 702 ; also Bd. xxiv., s. 918. MYOTONIA CONGENITA. 803 but may be mentioned, inasmuch as it produces a certain amount of paralysis. It is a congenital and inherited disease of the muscles, characterized by a marked hypertrophy of muscular fibres and a proliferation of the muscular nuclei. The muscular fibres diifer in shape from the normal, being more circular and not polygonal in transverse section.^ Symptoms. — The symptoms of the affection are a peculiar rigidity of the muscles occurring after they have been at rest for some time. The muscle is so firmly contractured that the patient cannot overcome the tonic spasm, and hence moves with great difficulty until the spasm relaxes. All the muscles of the body may be affected, and hence walking, running, and the use of the hands are very much hampered. The quick compensatory and automatic movements necessary in order to preserve the balance are so markedly interfered with that these patients constantly lose their equilibrium and are liable to falls. By repeated attempts at movement and by constant exercise these muscles may be limbered up, so that at the end of a long walk these patients may walk in a normal manner, but any period of rest or the ordinary rest of night is always followed by a renewal of the hard contraction of the muscles. Patients are hampered all their lives by this abnormal rigidity. Cold or damp weather, cold baths, great mental excitement, and sudden sensory impressions are said to increase the degree of the spasm. Active exercises decrease it. The patients have no other symptoms whatever, and hence the disease cannot be mistaken for anything else. The electrical contractility in the muscles is distinctly altered, a tonic contraction being produced by faradic excitement, and the reaction of degeneration being discovered on galvanic examination. Erb describes undulating rhythmical contractions starting from the cathode and passing to the anode. Mechanical excitability is increased, percussion of the muscle producing a sharp contraction of the muscle which remains for some time (fifteen to twenty seconds), producing a groove upon the skin. Pressure upon the muscles will also produce a firm contraction. As but thirty cases have been recorded in literature up to the present time, the affection may be considered rather a curiosity. There is no known treatment. ^ Jacoby, Journal of Nervous and Mental Disease, March, 1887. CHAPTER XLVII. TETANY. Tetany is a spasmodic affection of the muscles of the extremities, attended by a characteristic and abnormal excitability of both muscles and nerves to mechanical and electrical stimulation. Etiology. — It is a disease usually observed in children, and boys are more subject to it than girls. It may occur, however, in adult life, rarely, if ever, in old age. It is probably a toxic neurosis, due to the presence in the blood of some product of gastric or intestinal indigestion. It is most common in children suffering from rickets, and usually develops in the spring months, in children who have a poor digestion. It may occur in connection with severe diarrhoea ; with gastric dilatation and gastro-enteritis ; with the existence of intes- tinal worms ; and sometimes after typhoid and other infectious fevers. In adults, it may occur as a complication of pregnancy, or with lacta- tion. It has been seen after poisoning with chloroform, morphine, ergot and lead. It may develop in patients who have had the thyroid gland removed. It occurs as an epidemic in the spring in Austria, and chiefly among shoemakers and tailors. A similar epidemic has been observed in New York. This is probably due to a poison taken into the system from some article used in their trades. Symptoms. — The disease begins with numbness and parsesthesise of the hands and feet, attended by burning sensations. Then suddenly the spasmodic contractions of the hands and feet appear. The thumb and fingers are closely pressed together at their tips, making the hand assume a cone shape, though sometimes the thumb is flexed in the palm. Or the hand may assume the claw shape seen in progressive muscular atrophy. The wrist is flexed and adducted, and the forearm is held across the chest by the flexion of the elbow and adduction of the arm. Both arms are equally affected. The feet share in the spasm, being arched and inverted, the ankles extended and the legs held in rigid extension. In a few cases the trunk is affected, the body being bent forward, as a rule. Occasionally the jaw is fixed, and the facial mus- cles become rigid : the muscles of the pharynx, larynx and tongue may also be thrown into a spasm. Convergent strabismus has been observed. The muscles of respiration are seldom involved. The extent of the spasm differs in different cases ; in some it is limited to the hands ; in others it involves many muscles. The intensity of the spasm varies. It may last only a few minutes, and be so slight that voluntary effort can overcome it. In other cases it lasts longer, even 804 TREATMENT. 805 several ds^s, and the spasm is so severe that no force from without can stop it. It comes on suddenly and subsides gradually, but recurs in a series of attacks. The spasm is attended by pain in the muscles. In a patient suffering from the disease, pressure on any nerve or plexus will produce at once a spasm (Trousseau's phenomenon). A galvanic current passed through the nerve will cause tetanus of the muscles instead of a single contraction, and the electric reaction is altered, anodal opening contraction being greater than cathodal closure contraction (Erb's phenomenon), percussion of the motor nerves will start a spasm (Chvosteck's symptom), and either percussion or elec- trical stimulation of the sensory nerves may start a spasm (Hoffman's symptom). There is no loss of sensation. In some cases redness and cedema of the hands and feet, an excessive sweating, or a loss of the nails has been observed. The attack may be attended by a rise of temperature and a rapid pulse. The duration of an attack varies from one day to several months ; the spasms may occur only at long intervals, but the peculiar sensitive- ness of the nerves may last for a long time. Prognosis. — The prognosis is good in the majority of cases. When it occurs after thyroidectomy, or when the grastro-enteritis is compli- cated with dilatation of the stomach, the case may be prolonged and may be fatal. It is occasionally fatal in children, but usually because of the condition of the digestive tract. Treatment. — As the disease is due to a poison, it is wise to begin treatment by free purgation with castor oil. This is to be followed by the use of intestinal antiseptics of which salol and salicin are the best. The nutrition is to be kept up by careful feeding, and large amounts of water are to be given. Lavage of the stomach and high enemata are of much service. For the spasms, large doses of bromide with small doses of chloral are the best remedies. Opium may also be given. All effort should be avoided. Friction of the limbs with oil or liniments may relieve the spasm ; or the limbs may be wrapped in hot cloths. When the disease is very severe during pregnancy, it may be necessary to induce premature labor. When it occurs during lactation, the child should be weaned. When it occurs after thyroidectomy, thyroid extract should be given at once, and continued for months. Success has lately been attained by the use of calcium lactate in 15 grain dose three times daily. ^ 'Kinnicutt, Amer. Jour. Med. Sci., July, 1909. CHAPTER XLVIII. PARALYSIS AGITANS AND TREMOR. Paralysis agitans, shaking palsy, or Parkinson's disease^ is a functional disease of the nervous and muscular systems, characterized by tremor of the extremities, by rigidity of the muscles which pro- duces slow movements, abnormal postures and an unsteady gait with tendency to fall, and by abnormal sensations of varying intensity. Etiology. — The disease is more common among men than women.^ While occasionally appearing in youth it is rarely seen before the thirtieth year, and is far more common in the presenile period, as shown in this table. Table XIX. — Age of Onset. 20-30. 30-40. 40-50. 50-60. 60-70. 70-80. Total. Male 2 10 38 50 32 5 139 Female _9 i^ ^2 i^ -i _§? 2 19 54 90 45 9 219 Occupation appears to have no influence on the development of the disease. Direct inheritance can rarely be traced, though in the few cases occurring in youth, collected by Willige,^ a distinct inheritance could be traced in the majority. In 16 only of our cases, some relative had suffered from the disease. Gowers found an heredi- tary tendency in 15 per cent, of his cases, and WoUenberg con- sidered 26 per cent, of his cases due to the inheritance of a defective nervous system. A number of cases in one family have been observed by Borgherini and by others. But in view of the other etiological factors these conclusions seem to me questionable. The disease is usually attended by so many other evidences of old age that all authors regard it as a condition consequent upon senile changes. A tendency to tremor exists in all old people, even in those who do not have the disease, and some stiffness and slowness of motion often develops; hence it seems as if the symptoms were largely an exaggeration of the evidences of senile atrophy. The exciting causes of the disease appears to be emotional shocks, fright, worry, or anxiety ; traumatism, overwork, exposure to cold and hardship. The disease sometimes follows acute diseases, grippe, ma- laria, typhoid and pneumonia. Any or all of these causes produce a general weakening of the nervous system and thus start or hasten any ' Parkinson described it first in 1817. 2 The statistics in this article were prepared by my assistant, Dr. T. Stuart Hart, from the records of the Vanderbilt Clinic between 1888 and 1904. ^H. WiUige. Ueberparalysisagitansimjugendlichen Alter. Zeitsch. f. Neurologie, iv., 520, 1911. 806 SYMPTOMS. 607 process of retrograde evolution. I have seen many cases where it has followed soon upon a fright or a period of great anxiety. I have also known it to develop soon after a fall or blow. Thus a fall on the shoulder or an injury of one arm was soon followed in 31 cases by tremor in that arm. Sometimes the tremor develops in a part not the subject of direct injury. I have known it to occur as a sequel of neuritis, both in the arm and in the leg. Symptoms. — Tremor is the symptom most constant, and usually the one first observed. It is a rather coarse tremor, of variable inten- sity, occurring continuously. It consists of alternate contractions of flexor or extensor muscles, so that the joints are in a constant state of rhythmical motion. It can be arrested for a few moments by an effort of the will, but is equally severe in effort or in rest. Occasionally it ceases for a moment but is quickly resumed. The rate is about five or six vibrations to the second. It ceases during sleep. The origin of the tremor is an interference in the constant tone of the muscles, which is maintained by impulses going out from the motor centres. These impulses are so frequent as to be practically continuous in their effect on the muscles, but if they are impeded in their projection the effect becomes intermittent, and then in place of a normal muscular tone an intermittent tremor develops. The tremor begins, as a rule, in the hands or arms, one being affected for some months before the other (157 cases in 207). Occasionally it begins-in the feet or legs (23 cases in 207). Sometimes both arm and leg on one side are affected together (8 cases), and if the rigidity comes on rapidly a hemiplegia may be suspected. In rare cases the tremor begins in the head (3 cases), or is general from the outset (5 cases). When it begins in the hands, the index finger and thumb are usually affected before the other fingers. The affection is always noticeable in the handwriting, and this may first call attention to the disease. In 90 cases the tremor began on the right side of the body ; in 73 cases on the left side. There is always an extension of the tremor from the part first affected to other parts, until in the end all extremi- ties are affected. It is usual for the tremor to extend from an arm to the leg of the same side, but sometimes it appears in the other arm be- fore the leg is affected. If it begins in the leg, it extends to the arm of the same side sooner than to the other leg. The rapidity of exten- sion varies greatly. In the majority of cases one or two years elapsed before an extension to a second limb was noticed, but in a few cases such extension occurred within a few weeks. In one case no extension had taken place, although the right hand had been tremulous for twelve years and rigidity with lateropulsion was present. In another case all the extremities were involved within a year of the onset. Tremor of the head develops late in the disease in many cases. This is not to be confounded with the general oscillation caused by the tremor in the arms. In not a few a tremor of the jaw or of the lips and tongue was noticed, and in rare instances of the eyelids. The eyel)alls never oscillate. The patients often attempt to arrest the tremor by holding on to o06 PABALYSIS AGITANS AND TBEMOR. objects. The habit of these patients, of pressing the thumb against the first finger or of holding the fingers together to stop the tremor, has made the pen posture of the hand quite characteristic of the dis- ease. Active exercise or passive movements often mitigate the tremor for a time. The vibration of a railway train has the same effect. Any emotional strain, or the fact of being noticed increases it. Rigidity of the muscles is a symptom which follows the tremor after some years. Occasionally it is the first symptom to aj^pear and may exist for a long time without any tremor. It is felt in the muscles of the neck and back, at first, but soon extends to the ex- tremities, and finally to every muscle of the body. The stiffness of the neck and back lead to a peculiar attitude characteristic of the dis- ease. The head is bent forward, the shoulders are rounded, the body inclines forward, and all freedom of movement is lost. The arms are usually held to the sides, the elbows being flexed, the wrists extended and the first phalanges flexed. The other phalanges are extended. Abduction of the thighs is hampered and in walking the knees are approximated and the steps are short. The facial muscles are also stiff, so that there is little play of expres- sion and the face is like a mask. In fact it is often possible to recog- nize the disease from the peculiar facial expression, which shows none of the natural mobility in conversation and gives the idea of a lack of interest. The vocal cords may be so rigid that the voice is reduced to a whisper. There were changes in the voice noticed in 120 of my cases. In 59 the speech was monotonous ; in 31 it was slow; in 12 it was piping ; in 15 it was weak and feeble ; in 10 it was thick; in others it was jerky or tremulous. This rigidity hampers all movements, but it is not attended by paralysis in the early stages. If asked to look around the patient does not turn his head, but slowly moves his entire body, holding his neck rigid. In the last stage, however, the rigidity may become so extreme that the patients are confined to a chair or to the bed, and thus are virtually incapable of voluntary movement. Passive motions are then met by resistance, which is not true in the early stage. The rigidity leads to a slowness of action from the first, and this is noticed both in fine movements, such as writing or dressing, or in the larger ones of rising from a chair or walking or turning the head or body around. The stiffness of movement and the peculiar posture in walking lead to a characteristic gait. This is at first stiff and slow, as instinctive balancing movements are hampered and as the body is thrown forward the centre of gravity is really in front. Hence the patient often feels as if about to fall forward, and instead of standing erect hastens his steps almost to a run and finally stops himself by seizing some object to keep him from falling. This is termed propulsion or festination. Occasionally a tendency to step backward is noticed. Thus if a patient attempts to reach up for an object, throwing the body back- ward, he loses his balance, and steps back several steps till he runs SYMPTOMS. 809 against soirfething. This retropulsion may be set up by pushing the patient when he is standing still. Propulsion was present in 77 cases, retropulsion in 19 cases, and lateropulsion — a tendency to fall to one side — in 9 cases in my series. It is not a constant symptom. It may occur when there is no tremor. It never occurs without rigidity. The difficulty in balancing sometimes prevents patients from starting to walk. X have known several patients who, after being assisted to rise from a chair, had to be swayed forward and backward and given a little shove in order to enable them to walk. The power to begin stepping seemed lacking. Fig. 307. Fig. 308. Position assumed by patient juiFerlng from paralysis agitans. Rigidity of back in paralysis agitans. In spite of the rigidity, the muscles remain in good condition, and do not atrophy or show any electric changes. In the last stage an atrophy from disuse is quite common. In 188 cases the deep reflexes were normal in 90, were exaggerated in 68, and were diminished in 38. The knee-jerks were often unequal on the two sides, but this had no relation to the tremor. Contractions of the hands and feet occasionally develop in the later stages of the disease. The hands and fingers are then firmly closed and the feet in the posture of talipes equinus. The toes are occasionally drawn under the foot. When the face becomes rigid, the saliva and tears may not be retained. 810 PARALYSIS AGlTANS AND TEEMOH. The rigidity of the muscles is often accompanied by an aching feel- ing or a dull pain. There is also a great sense of restlessness, and the patients feel that they may get ease by a change of posture. Patients who walk with difficulty have to be helped out of the chair and helped about the room every little while, to obtain any relief. It is rare, however, for pain to be sharp or to keep a patient awake. Sometimes pain precedes the onset of tremor. Parsesthesise are very commonly complained of. Tickling, tingling, numbness, flushing, and sensations of heat and of cold are felt, usually in the tremulous limb, often all over the body. The most common sensation is a burning feeling in the skin, and the skin may feel hot to the touch even when there is no internal rise of temperature. There is never any anaesthesia. Hyperidrosis was observed in one quarter of my cases, but the sweating was not limited to the tremulous limbs. A rapid pulse was occasionally noticed. Insomnia is a frequent symptom, and often requires treatment. The functions of the bladder and rectum are never abnormal unless some complication (such as enlarged prostate or hemorrhoids) is present. The mind is never affected, but patients often become impatient, or even morbidly depressed at their helpless state, and crave sympathy and encouragement. A certain portion of the cases develop hemiplegia, but not more than is to be expected at the age affected. Course. — The disease is a slow one in its progress. It begins as a rule gradually and the tremor may last for many months before it becomes extreme or before rigidity develops. The posture and gait become characteristic only after several years. In any stage a remis- sion in symptoms may occur, and I have seen patients who had suf- fered for years and who were unable to walk without help or to talk above a whisper, restored to comparative health for some time. Any emotional strain may make the symptoms much more intense, or increase the rapidity of their development. Occasionally the disease begins suddenly after a fright or an emo- tional strain. A number of cases of sudden onset were recorded in France after the sieges of Strassbourg and Paris, and due to fright or anxiety. I have known several such cases. As a rule, the disease gets gradually worse, and after several years the patient is finally obliged to remain in a chair, and moves with great effort. In some cases the rigidity is the first symptom, and tremor does not ap- pear for several years, after the face and voice and gait are characteristic. The duration is indefinite, as patients never die of the disease, but they never fully recover. Prognosis. — This is unfavorable for recovery, but good for life. Diagnosis. — The symptoms are so characteristic that there should rarely be any difficulty in the diagnosis. It can be easily distinguished from multiple sclerosis by the facts that effort stops instead of increas- ing the tremor ; that the speech, though weak, is never scanning ; and by the fact that nystagmus does not develop. It cannot be mis- TREATMENT. 811 taken for hemiplegia because of its gradual onset, slow extension of symptoms, and the absence of any true paralysis. The tremor of paresis is finer, is not continuous while the hands are at rest, and there is no rigidity or characteristic posture and gait in paresis. Senile tremor usually begins in the head, and while it may invade the hands does not aifect the legs and is not attended by rigidity, or other symptoms of the disease. Senile paraplegia from arterial scle- rosis and secondary sclerosis in the spinal cord, is accompanied by actual paralysis and anaesthesia, with disturbance of the bladder and rectum and a spastic gait which is not like that of paralysis agitans. The posture of hands and of the body in standing, the mask-like face, the gait, and the tendency to festination are not seen in any other disease. Theories of the Disease. — The lesions described by various ob- servers, such as degeneration of the motor cells of the spinal cord; arterial sclerosis in the finer spinal vessels and a perivascular sclerosis of the anterior horns ; and degeneration of the motor cells of the cor- tex of the brain, appear to be lesions of old age and not characteristic of the disease. It is possible that an affection of the terminal plates of the muscular nerves may be the true lesion of the disease, but this is as yet only a theory. Gauthier believes the disease to be primarily muscular in origin, and there is much to be said in favor of this hypoth- esis Camp,^ in a careful review of the various theories and on the basis of fourteen cases with autopsies, reaches the same conclusion. Moebius believes it to be due to autointoxication from some error in the metabolism, due in some cases to parathyroid disease, and this theory has been urged by Lundeburg ^ and Berkeley.^ Gowers believes that the disease is of cortical origin, basing his state- ment on the fact that in his observation the onset of hemiplegia caused a cessation of the tremor. But I have seen two cases in which the onset of hemiplegia has had no effect on the tremor whatever. The pathology of the affection must still be considered as unknown. Treatment. — A life of congenial occupation without annoying care, varied by travel and by visits to health resorts, and under the wise direction of a good nurse who can give massage, is the best regime for the patient. Diet has no influence on the disease. A warm climate is to be sought, as patients suffer more and are more rigid in their move- ments in cold weather. In a chronic affection it is not wise to stop the patient's work, as he is then more likely to worry over himself. Warm or tepid baths of twenty minutes duration either morning or evening, not followed by cold shocks, afford relief to the rigidity. Massage is also of great service and may be kept up indefinitely. The lomi lomi method of Hawaii is of especial use. Passive motions often give relief to the feeling of fatigue and may lessen the tremor and rigidity. Vibration is also of benefit, especially the forms obtainable 'Jour. Amer. Med. Assoc, April 13, 1907. ^Deut. Zeitschr. f. Nervenheilkunde, 1904-5. *W. L. Berkeley, Medical News, Deceniljer, 1905. 812 PABALTSIS AGITANS AND TBEMOB. in the Zander institutes, where the entire body can be shaken, or vibratory pads can be applied to the spine. Electricity is of no use. Though the course of the disease cannot be affected by drugs, some of the symptoms can be mitigated. The tremor may be diminished, and even arrested for a time by the use of hyoscyamin. A tablet of -g-i-g- grain may be given at first every 6 hours, then more frequently, the dose being increased till the tremor is diminished, and then kept at that point. Thus in some patients 3 such doses daily are sufficient. In others twice this amount may be required. The dryness of the mouth and the dilatation of the pupil are evidence that enough is being taken, and the dose should not be pushed as it is a poison. The use of the drug may be kept up for years with benefit. It is aided by the simultaneous use of codeine in ^ grain dose. This is not to be increased beyond three doses daily. Duboisin sulphate in -^-^ grain dose may be used if hyoscyamin fails. I have not found it as efficacious. Cannabis indica in pills of ^ grain each, three or four times a day, may be of service. Small doses of tincture of veratrum viride (2-3 drops in water 3 times a day) some- times diminish the tremor. Tincture of gelsemium is often of service. Arsenic is a remedy which has some reputation, and though it does not affect the tremor it may lessen the rigidity. It is to be used with caution, never continuously. In the very last stage of the disease when the rigidity is painful and the patient helpless, the codeine may be increased in amount or morphine may be used to give relief. The use of parathyroid yL grain in tablet 3 or 4 times daily is urged by Berkeley, but is not successful in many cases. The rigidity is decidedly benefited by warm baths and by massage. The insomnia may require treatment by trional, veronal, or the bromides. Tremor. — Tremor is a symptom which may be present under various conditions. There are toxic tremors from alcoholism and lead and mercury poisoning. There is a tobacco tremor often asso- ciated with tobacco heart. Neurasthenia or any exhausting disease may lead to a fine tremor. These tremors can be cured by removing the cause. Hereditary tremor develops in some families in middle life without known cause. It affects the hands chiefly though after a time the legs, the head and the chin may be involved. It is never very in- tense but persists through life — the chief inconvenience being felt in the act of writing. It is increased by mental or physical effort, also by coffee and tobacco. Senile tremor appears in some persons after the age of fifty. It usually begins with a slight lateral oscillation of the head. Later the hands may be affected. It also persists to the end of life. CHAPTER XLIX. EPILEPSY. Epilepsy is a disease of the brain, characterized by the occurrence of attacks at intervals, there being no symptoms between the attacks. The attacks which occur may be of three kinds — slight attacks, or petit mal; severe attacks of convulsive nature, grand mal; or psy- chical attacks. The jpetit mal attack consists of a sudden loss of consciousness, last- ing a few seconds only, and often not appreciated by the patient. He may turn pale, his pupils may dilate, he may gave a slight start or twitch or sigh, or he may turn his eyes or head, or close his eyelids. He may stop, if walking, and sway as if about to fall. He may make some sound, smacking the lips, or attempting to speak, or groaning in a low tone, or merely catching his breath. If talking he may hesitate, stare in an abstracted absent-minded manner for a second, and then resume the conversation, wholly unaware that he has acted in a pecu- liar way. Some patients will suddenly run forward, or in a circle, for a few seconds, rapidly, without any apparent object and be unable to explain the act. This has been termed procursive epilepsy. It occurs in children and is often the first sign of the disease. It continues and is usually succeeded by attacks of grand mal, in which the running may constitute the aura. I regard this as evidence of cerebellar irrita- tion. Sometimes in infants a series of movements of the head and body, a swaying forward and backward, has been observed attended by a vacant stare. This has been called a tic de salaam. It is a form of petit mal and often goes on to grand mal. In some cases the patient becomes suddenly conscious of a sensation which is forced upon his attention and so occupies his mind that he disregards everything else. The sensation may be one of vertigo, or a sinking feeling, or a sense of distress in the stomach, or a palpitation of the heart, or an indefinite abdominal feeling. It may be a light, or a blurring of vision ; it may be a sound like a ringing in the ears, or a single tone, or a crash ; it may be a smell or a taste ; it may be a tingling feeling in the skin of any part of the body, usually in one limb. It may be the sensation of a twitching of some muscles, and the twitching may actually occur for a few seconds. Consciousness is lost for a few seconds after this abnormal sensation, and when regained the patient has a knowledge of the sensation only, which has then passed away. Some patients are slightly dazed after such an attack of petit mal ; l^ome may even go to sleep ; but the majority pay no at- tention U) the attack and resume their ordinary condition after it is 813 814 ' EPILEPSY. over. Many deny the occurrence of any attack, being entirely uncon- scious of it, and hence having no memory of it. Attacks of petit mal may be the only manifestation of epilepsy. As a rule they are not. The grand mal, or severe attack of epilepsy, consists of a sudden loss of consciousness, followed by a general convulsion, and by a period of stupor, from which the patient recovers with no memory of what has occurred. Epileptic convulsions are preceded in about one half of the cases, by a conscious sensation, which is recognized after a time as an inevit- able precursor and warning of an attack. It is called an aura. An aura was present in 750 cases out of 2,000.^ Its character was as fol- lows, in order of frequency : Table XX. Kind of Aura. Epigastric sensation 198 Vertigo 158 Numbness in one or both arms 76 Visual hallucinations or blindness 68 Mental states with fear or depression 51 Cardiac sensations 34 Auditory hallucinations 30 Abdominal sensations 25 Numbness in one or both legs 27 Numbness in the face 22 Respiratory sensations 16 Hallucinations of smell 8 Feeling of cold or heat 8 Nausea 6 Hallucination of taste 5 Numbness in the trunk 4 Sensation of thirst 4 Eush of blood to the head 4 Sleepiness 4 Hallucination of taste and smell Sensation of hunger Flow of saliva Diarrhoea Tremor A variety of sensations not included in this list may occur as aurse. Thus tremor of one extremity or an indefinite sense of tremor through the body, a twitching of some muscles, grinding of the teeth, a sucking motion or a clicking made by the tongue, a harsh cough or a sudden yawning may occur at the beginning of the attack before consciousness is lost. Some epileptics stop suddenly if walking, make balancing movements or turn about ; others start up and walk or run a few steps ; others run about in a circle and then fall in a fit. There is a dim consciousness of such acts and they are remembered afterward as the invariable precursor of an attack. Among the visual aura the seeing of colors, especially red, or of figures is not uncommon. One 1 The statistics in this chapter are based upon an analysis of 1,750 cases from my clinic, prepared for me by Dr. L. Pierce Clark, and on 250 consecutive cases in my private practice, EPILEPSY. 815 patient always sees a figure resembling little red riding hood, another sees a drageto head, anotlier the form of a new moon. To others the objects about them seem to grow smaller or larger as they lose con- sciousness. Hughlings Jackson has described an intellectual aura which he called a dreamy state. In this some recent events of the patient's life seem to pass through his mind rapidly, a sort of forcible reminiscence of experience which holds his attention. In a patient of F4r§ the attack began with a doubt of the existence of the Deity. It is therefore evident that in every form of mental activity of which we have recollection a sudden forcible presentation of the contents of the mind without volition may occur as an aura of an epileptic attack. The aura is uniform in each patient, and does not vary its character in different attacks. It is. more likely to occur in light cases. It is evident that the aura constitutes the attack of petit mal in many cases; and patients who have grand mal attacks with an aura often perceive the aura without having a convulsion. The duration of the aura is not more than a few seconds, but it leaves a memory, which is usually the only means the patient has of knowing that an attack an occurred. After the aura, or without any warning, there is a sudden loss of consciousness which is more lasting than in petit mal. A deep inspira- tion or expiration occurs, and if the glottis is fixed at the time this causes a groan or a cry, often low, sometimes sharp and loud. The head and eyes turn to one side, or upward or downward, or a fixed stare appears. The face turns pale. The pupils dilate. Equilibrium is lost and the patient falls heavily, often injuring himself. Then all the muscles become rigid, in a state of tonic spasm. The respiration ceases and cyanosis follows. The eyeballs bulge, conjunctival hemor- rhages occur. The jaw closes and the tongue is often bitten. The hands are closed, the thumb being shut in by the fingers, the limbs are sometimes flexed, sometimes extended. It is more usual for the arms to be bent and the legs straight. They are not symmetrical on the two sides. The stomach, bladder, seminal vesicles and rectum, if full, are sometimes emptied by the tonic spasm of the muscles. This tonic stage lasts from 5 to 40 seconds. A general convulsive movement of the face and limbs ensues, con- stituting the clonic stage of the attack. The motions are at first small in extent, and jerky in character, but soon become larger and faster and more violent, until the entire body is writhing in a general con- vulsion. The head is turned from side to side, the jaws snap, the eyes roll, the face makes grimaces, of a horrible kind, the tongue is thrust out, saliva flows, and the arms and legs are twisted in all possible positions and contortions. Injuries often occur in consequence, and dislocations of the joints, especially of the shoulder and jaw, may result. If the tongue has not been bitten in the tonic stage it may be in the clonic. Respiratory movements relieve the cyanosis but are irregular, often deep and stertorous, and the saliva and blood flow from the mouth and are churned into a froth which covers the lips. Every possible attitude may be assumed, and the position of the limbs is rarely sym- 816 EPILEPSY. metrical on the two sides. After a continuance of these convulsive movements for a varying period, from one to five minutes, the spasms gradually become less frequent and severe, and finally cease. Deep abdominal respiratory movements continue for some seconds at the end. The patient does not, however, recover consciousness immediately, but gradually comes to himself. He is at first dazed, does not know what has happened or where he is, and requires some time to return to his normal state of mind. His last memory is of the aura with which the fit began, and his first perception is usually of fatigue in the muscles, or of some injury received in the fit. In a majority of cases the patients pass directly from the convulsion into a comatose state, and cannot be aroused. In this the pupils are contracted, the body is cov- ered with sweat, and the odor of the breath is very oifensive. Others regain consciousness, appreciate that an attack has occurred, and then voluntarily compose themselves to sleep from which they may be wakened. Coma or sleep may last several hours. On waking, or on coming out of the coma, the patient feels dull, is much fatigued, has headache, and sometimes vomits. Occasionally a true paralysis of the exhausted muscles, or a state of aphasia, or of blindness or deafness or of impaired sensation, remains for a few hours. Babinski has observed an exaggeration of the deep reflexes with ankle clonus and extension of the great toe on scratching the foot (Babinski's reflex), also a loss of the anal reflex during and after a grand mal attack, and these physical signs he considers of great importance as differentiating an epileptic from an hysterical convulsion. A slight rise of temperature has been observed during an attack. The urine after an attack is often in- creased in amount and contains an excess of phosphates and urea, and sometimes albumen. Intestinal disturbances and diarrhoea occasionally ensue. As a rule, the patient has fully recovered after twenty-four hours and is then in his usual state of health. Every possible degree of severity of attack has been seen in epilepsy, from the slightest form of petit mal attack, up to a severe type of grand mal. In fact it is often impossible to determine to which class the attack is to be assigned, some authors describing as petit mal a condition which others consider grand mal. In many patients both types of attack occur. If the attack occurs during sleep only the patient may for years have no knowledge of his disease as he may not be awakened by the fit and may pass from it into a deeper sleep. Unusual fatigue or pain felt on waking, at intervals, should always excite suspicion of the possibility of nocturnal epilepsy. A convulsive movement of one-half of the body, quite similar in its characteristics to the general convulsion of epilepsy but not often attended by a loss of consciousness, first described by Hughlings Jack- son and hence called Jacksonian epilepsy, may occur. This is now regarded as a positive symptom of organic focal disease of the brain, and has been fully described on page 91. Jacksonian attacks sometimes occur in the course of epilepsy, but should not be classed with this disease. EPILEPSY. 817 Sometimes one graud mal attack follows another in rapid succession, so that the patient passes from the first coma into another convulsion and these alternate. The condition is then known as status epilepticus. As many as a hundred convulsions may occur in a day, and if they continue two or three days the result may be fatal. In this condition the temperature gradually rises to 103° or 105°, the pulse becomes rapid and feeble, the respiration rapid and irregular, and death occurs from exhaustion or heart failure. One quarter of epileptics die in status epilepticus. The thh-d type of epileptic attack has been termed a psychic attack or mental epileptic equivalent. These attacks differ from major and minor attacks in the fact that there is no aura and no apparent loss of consciousness. While the patients may seem dazed and peculiar, they appreciate their surroundings, and answer questions properly. On the other hand, the state of consciousness is not normal, for after the attack is over there is no memory whatever of anything done or said while the attack is in progress. This form of attack is rare. It dif- fers in each patient, and is not uniform in the same patient. Thus one of my patients who is fond of music will suddenly abandon anything he is doing, go in search of his violin, play it for a few minutes, and then carefully hide it away in some unusual place. He then lies down on any convenient bed or sofa, or on the floor, and appears to sleep for a few seconds or a minute, and on waking is unaware of having done anything unusual. If asked about his violin, he does not know that he has touched it, and is unable to find it. In two attacks it has been so well hidden that it has not been found until in the next attack he has gone to its hiding place to get it in order to play it again. This young man has never had a major or a minor attack. Another patient becomes suddenly overcome by a feeling of intense depression, a deep melancholia ; he feels that he has sinned ; he is inclined to commit suicide, but is oppressed by a sense of physical weakness and soon falls asleep, waking in a few minutes perfectly well and happy. Many patients who are said to present states of double conscious- ness are probably suffering from psychic epilepsy. A person may stop his work, or leave his home and wander away, start on a journey, go to some other place and be gone several days, finally coming to himself in a strange locality, and not know how he got there and be unable on his return to give any account of his actions, as he has no memory of what he has done during the period of ambulatory automatism, as it was termed by Charcot. Another form of psychic attack is less harm- less. A patient will suddenly get into a terrible rage without cause, will abuse relatives or friends, or even address perfect strangers with insults, will attack those who attempt to restrain him and may commit homicide in his rage. Others attempt to set fire to things about them or commit sexual assaults. The attack will subside as suddenly as it begun, and the patient has no memory of it. Thus one of my patients^ a devoted mother, has sudden attacks of this 62 818 EPILEPSY. kind iu which she has attempted to kill her children. Another, a young man developed such attacks after a fracture of the skull, and was cured by trephining. These attacks are called epileptic mania. Some psychic attacks are less severe, a sudden intense fear and dread of impending evil or danger takes possession of the mind, and the patient seems to suffer intensely. Such a patient may suddenly start and run for some distance, and on coming back to normal conscious- ness be unable to account for his conduct and have no memory of his mental distress. Some patients have a sudden arrest of thought and pass at once into a dreamy state from which they cannot for a time be aroused to full consciousness. When they awake they have the indis- tinct recollection of a dream but no acute consciousness of anything said or done in their presence while in the dreamy state. Some patients experience an overwhelming desire to sleep, fall asleep, nod or snore once, and then awake, being dazed for a moment afterward. Inasmuch as peculiar mental states often follow attacks of both petit mal and grand mal, it has been held by some authors that the psychic attack is always the sequel of a major or minor attack and should not be considered as an equivalent of an attack, but as a post- epileptic phenomenon. To this I do not wholly agree, as evidence is wanting of any form of minor or major attack preceding the mental state in many cases. The relative frequency of the different types of epileptic attack is shown in the subjoined table. Table XXI. — Character of Attacks in 2,000 Cases. Cases. Cases. a Grand mal and psychic 3 Grand mal 1,150 Grand mal, petit mal and psychic... 6 Grand and petit mal 589 Petit mal and psychic 2 Petit mal 179 Jacksonian 37 b Psychic 16 Diurnal and nocturnal 882 Grand mal and Jacksonian 10 Diurnal only 658 Grand and petit mal and Jack- Nocturnal only 380 soman It will be seen that every combination is possible, but that the psychic type is quite rare. The second part of this table shows the tendency to a special limi- tation of the attacks in some cases to certain times of the day. It has long been noticed that some epileptics have attacks at night only — others at certain hours of the day only. But the majority of cases have attacks both by day and by night. Dr. L. Pierce Clark, who has made a special study of the Daily Rhythm of Epilepsy,^ has pre- pare the annexed chart which is of much interest. The condition of the patient between the attacks may be that of perfect health and of normal mind. This is the rule in the beginning of the disease, or when the intervals between the attacks are long. 1 Medical News, July 18, 1903, EPILEPSY. 819 But the tendency is for these intervals to become shorter, and when attacks are occurring with considerable frequency the majority of patients cannot be called well or normal between the attacks. There is a gradual development of dementia in the chronic severe cases. The usual course of a case of epilepsy is as follows : The disease begins in early life. One convulsion is followed after a year or more by a second, and this, in turn, after a few months by a third. Then the attacks become more frequent, — two months or six weeks may be the usual interval in some cases ; in others, attacks occur every week ; in very severe cases two or three times a week. Not uncommonly several attacks occur within a few days, and then after an interval of two or three weeks another series of attacks occur. The tendency is for the attacks to increase in frequency, until a regular interval is es- tablished after five years, and at this interval the patient continues to have attacks all his life. As a rule, the course of the disease is modi- fied by medicines. These reduce the frequency of the attacks just in proportion to their previous frequency. Thus when a patient has had attacks once a week, treatment may prolong the interval to once in two months ; if he has had only two attacks a year, he may go three or four years without an attack if his treatment is kept up. When treatment is stopped the attacks recur. The general effect of the attacks upon the physical condition is not serious. Patients often suffer from injuries during an attack, and most epileptics can show scars, but the health may be good. The more serious effect of the disease is upon the mind. Children who suffer from epilepsy cannot /J ?n Table XXII. (?Jn 1 , ^ s V ■i (, r f ■?■ /,> „ /5 / 3 .5 <, '<" c. 7 r ,c „ /?. 77>1 W».' pS/) Kn Ult i-ttf ^ao .'TCJIt i-3M .-rnn 1 - \ - Vttc 1 I 1 Wse 1 1 1 I 1 1 1 1 1 1 1 1 ■ ■ • 1 1 1 1 1 1 ■ P I mm\ 1 Yj'^3^'ftB-\-7^idff'r\filf^^<^'f;romide the bromide may be safiily stopped for ten days in case of absolute need — as in an intercurrent illness — but after two weeks all the bromide has been eliminated from the system and no effects remain. (i3 834 EPILEPSY. It is well to add to any bromide salt which is used some glycero- phosphate of soda, 20 grains three times a day for' an adult being the usual dose. This seems to counteract some of the ill effects of bromide and at the same time is an excellent food for the nervous system. The two can be combined in the same solution. While bromide is being given patients should use little or no salt with their food. The ill effects of bromide are first to disturb the stomach by setting up a catarrhal inflammation. This may be avoided in some degree by giving it very freely diluted in water. Sometimes the use of poor preparations of bromide which contain arsenic is responsible for the irritation of the stomach. The second ill effect is the appearance of acne. Proper care of the skin, by hot baths and the free use of soap followed by massage, prevents this effect in some patients. In others the addition of small doses of arsenic given not longer than one week at a time will prevent the acne. The pustules should be pricked and the contents expressed, and an Fig. 309. antiseptic lotion or ointment applied. If the pustules become confluent and large thick crusts form the bromide must be stopped for two or three weeks even at the risk of attacks ; its elimina- tion hastened by increased water drinking and saline laxatives, and the surface dressed aseptic- ally until it heals. The third ill effect of bromide is its effect upon the mind. In all cases it dulls mental proc- esses, makes the perception less keen, the memory poor, and reasoning slower. This is in- evitable but these effects are temporary and cease when the bromide is stopped. In a few cases, which I have seen, bro- mide causes active mania or all the symptoms of the early stage of paresis. In these cases its use must be abandoned. Epileptics who are taking bromide should be watched for occasional cumu- lative effects, and when these appear the drug should be stopped for a time. The effect of bromide on the disease is to lengthen the interval between attacks, and they may even stop entirely after the intervals have been lengthened to several months. The dose should be kept up for two years after the last attack and then gradually reduced during one year and finally stopped. It is sometimes found that a rapid Bromide eruption iu an epileptic. TBEATMENT. 835 graduated increase in the amount of bromide given from 20 grains up to 150 grains at night, and then a gradual decrease to the original dose has a good effect. Such a course can be spread over six months and may be repeated once a year. Chloral hydrate in dose of 5 up to 15 grains three times a day may be added to bromide, and often aids its effects. This drug may be given by rectum in the status epilepticus. It cannot be continued very long without causing depressing effects on the heart. Tincture of belladonna has been given with bromide in gradually increasing doses. I have never had any favorable results. Tincture of digitalis has been given with bromide in cases where the heart was feeble. I have not been convinced of any effect upon the epilepsy. When bromide fails of effect or has to be stopped because of its ill effects tincture of simulo may be used in 1 up to 3 drachm dose three times a day. I have seen good effects from it and regard it is a valu- able remedy. It may be continued a long time, and often diminishes the number of fits. It may be given with bromide, thus enabling one to reduce the dose of the latter. Fluid extract of solanum caroliuense (horse nettle) in dose from 1 to 4 drachms three times a day is also a valuable remedy either with, or without bromides. Calcium lactate in doses of 15 grains three times a day has been of service in some cases. I have had success with this remedy, and it appears to have no ill effect. Antipyrin is the only one of the coal tar derivatives which thus far has been found to have any effect upon epilepsy. Given in 15 grain powder at night and 15 grains on rising, it sometimes mitigates the attacks. Chlorotone, a synthetic product of Parke, Davis & Co., will decrease epileptic attacks but renders the patient so stupid and dizzy that its use must be limited to a short time. It acts well to arrest a series of severe attacks. In a status epilepticus large doses of bromides and chloral by rectum may be of service, and sometimes the fits may cease under chloroform inhalations given carefully for several hours. In a few cases where an aura of some duration precedes the attack, the inhalation of amyl nitrite may arrest the attack. Pearls of this can be carried and broken in the handkerchief or a bottle filled with cotton wool soaked in amyl nitrite can be carried and inhaled. In some cases the aura consists of a numb feeling in one limb. A band tied tightly about his limb when the numbness is first felt has been known to arrest the attack. One of ray patients wears a strap about his wrist and pulls it tight when he feels the tingling in the fingers and thus aborts the fit. In senile cases or in cases due to arterial sclerosis, the use of nitroglycerine y,|jj grain two to five times daily, or of ni- trite of sodium •'{ grains four times daily, combined with heart stimu- lants, is. of much more service than the use of bromides. It is in these cases that chloral in 3 to 5 grain dose four times a day and also 5 grain 836 EPILEPSY. doses of potassium iodide has been of some service. In old persons a drink of whiskey will sometimes abort an attack. I have never found much effect from any remedy in petit mal, though the attacks may diminish under any of the lines of treatment described. Occasionally nitroglycerine in ^-i-^ grain dose two to four times a day has a favorable effect. Oxide of zinc 5 grains after each meal in capsule has been of some service in epilepsy. Borax 15 to 25 grains after each meal given in powder has done good. Forel, of Zurich, recommends a course of treatment by opium, the patient being put to bed and fully narcotized for six weeks, the opium being then slowly diminished, and a larger dose of bromide being given daily as the opium is decreased. I have not had good results from this course of treatment. I have known the opium habit to be induced. It is a method which is fallipg into disrepute. Opium and morphine should be avoided in epileptics. In an attack nothing can be done except to prevent injuries, to pre- vent if possible biting of the tongue, by placing some hard substance between the teeth and to prevent congestion of the head by loosening the neck band. The patient should always be permitted to sleep after the attack as long as he desires. A warm salt bath will relieve the fatigue of the muscles which is inevitable. Surgical Treatment. — There is a small percentage of cases of epilepsy open to surgical treatment. When a focus of disease in the brain can be exactly located surgery may remove it. In cases following injury or depressed fractures of the skull it is always possible that a spiculum of bone may be irritating the brain and in these cases its removal is indicated. I have published elsewhere^ numerous cases of this kind. In cases where the aura or attack is distinctly cortical in type, is uni- form, and is not attended at the outset by a loss of consciousness it is often possible to locate some focus of irritation if the case is studied with the facts concerning localization mentioned on pages 91 to 96 in mind. In such cases exposure of the cortex may reveal a plaque of adherent meninges or a small area of sclerosis or of gliosis and this niay be removed. So many tumors begin with epileptic attacks that this procedure is advocated whenever the attacks are distinctly localizable. In all non-traumatic cases with no localizing symptoms surgical treatment is entirely unwarranted and useless. Even in the cases in which it is indicated and properly carried out it is not uniformly cura- tive. Any scar in the brain, whether produced by disease or by a surgeon's knife, may act as an irritant and cause attacks. Hence after any operation on the brain, even for the removal of tumors or abscess, epilepsy may develop as a sequel. It is not surprising there- fore that even in cases of epilepsy, which have been trephined, and in which some focus of disease has been found and removed, the patient may have a recurrence of the attacks. The surgical treatment of even selected cases of epilepsy has not been as successful as was at first expected. ^ Brain Surgery, William Wood & Co. CHAPTEE, L. NEURASTHENIA AND THE OCCUPATION NEUROSES. Neurasthenia is a condition of exhaustion in the general nervous system. It may be general, all parts of the system being affected. It may be local, either the brain, or the spinal cord, or the vasomotor system being chiefly involved. Hence its symptoms vary widely in diflPerent cases, and are almost as numerous as the various activities possible in mind and body. Every act of mind or body is attended by certain chemical and physical changes in the cells of that part of the nervous system con- trolling the act. These go on for a time within physiological limits without harm. But if continued beyond those limits they result in exhaustion of the cells, giving rise to uncomfortable sensations of fatigue and inability to continue work. The initial state of the cells ; their degree of development ; their inherent strength ; and their state of nutrition determine the limit of their endurance ; for what is healthy effort to a strong man may be impossible overstrain for a weak one. The condition of exhaustion may be rapidly or slowly compensated for in different conditions or in different persons ; the capacity for recu- peration differing widely. But it is probable that permanent changes are rarely produced in cells by work and that a return to a normal state is the rule when sufficient rest is given. (See page 27.) Edinger however believes that if the cells of any part of the nervous system are overstrained by excessive work they may undergo a process of degeneration, which in turn may be followed by a secondary sclerotic process in the connective tissue which holds the cells together. When such sclerotic changes opcur they are permanent and form the basis of organic disease. Hence he believes that overwork may cause perma- nent inefficiency. His views are so important that the following summary is quoted : " The overfunctioning of a sound nerve, or normal functioning of a degenerated or intoxicated nerve causes fatigue which may amount to exhaustion, and the power of recuperation may be transiently or per- manently lost. Poisons, like alcohol and lead, reduce the recuper- ative power, and nerves subjected to strain from the occupation may experience exhaustion and ultimate paralysis and atrophy. Too little attention has been paid hitherto to the questions, What becomes of a normal nerve subjected to unduly great strain, or of a weak nerve of which normal functioning is demanded? and How does the central organ fare with these absolute or relative overdemands? A constitu- tional, defective composition of a part leads to atrophy when func- 837 838 NEUBASTHENIA AND THE OCCUPATION NEUEOSES. tional demands are made on it. A similar process occurs in another group of nervous affections which are not congenital but result from the action of poisons, as in the nervous affections after ergot poisoning, after pellagra and, above all, after infection with syphilis, as in tabes and paralysis. All these have an injurious influence even on neurones of which only normal function is demanded." • Etiology. — Predisposing causes are prominent in the production of neurasthenia. In the majority of cases it is possible to discover some factor in the heredity of the patient, which has induced an imper- fect development and consequent inherent weakness of his nervous system. One or both parents may have been the subject of some disease, either nervous or of any other character ; they may have been subject to intoxication by alcohol, drugs, or autoinfection ; they may have been old or feeble ; they may have been suffering from overwork or strain of a mental or emotional kind, or they may themselves have been neurasthenic. Anxiety or illness in a mother during pregnancy impairs the strength of the child's brain. Under these conditions the child is born with a nervous system incapable of the highest develop- ment, and unable to stand exhausting strains, and defective in recup- erative power ; hence he becomes an easy prey to the exciting causes of the disease in later life. This factor is often overlooked both by patients and by physicians, who are disappointed at the lack of result from treatment or from periods of rest, which otherwise would be effective. A firm building cannot be erected on a foun- dation of sand. The exciting causes are numerous. A life of too continued effort without proper periods of rest and sleep, without proper food and proper exercise, and harassed by the worry which the struggle for success entails is the history usually obtained from a neurasthenic. Often alcoholic or sexual indulgence is an added cause. Sometimes some acquired disease, especially syphilis, some infectious disease, e. g., grippe, or some chronic exhausting affection, like intestinal or gastric indigestion, nephritis, gout or rheumatism is the evident exciting cause. Sometimes severe shocks or intense anxiety or grief are productive of the disease ; and worry may be said to cause more cases than over- work alone. Thus family care or affliction, the distress of mind con- sequent upon money losses, or illness or ill conduct of some relative, an unfortunate love affair, a disappointed ambition, or intense remorse are causes of neurasthenia. A sudden change in one's habit of life may so disarrange the chem- istry of nutrition as to produce neurasthenia. Thus athletes who stop exercise, hard workers who rest completely, women who make a com- plete change of diet or who stop taking fluids in order to get thin, people who suddenly adopt some fad in their food, men who do un- usual work at night and sleep little by day, persons who subject them- selves to great changes of altitude or climate, are frequently liable to develop neurasthenia. Great excess in work, especially if attended by anxiety, is a very ETIOLOGY. 839 frequent cause. Thus college students after final examinations, pro- fessional men after an intense effort in the pulpit or at the bar, or medical men after trying cases, or engineers after unusual exertions often develop an acute neurasthenia. In women menstrual disorders, or uterine and ovarian disease are very likely to cause continued irritation and exhaustion of the nervous system. A laceration of the perineum with its secondary displace- ments of uterus and bladder may be a source of constant sensory irri- tation and discomfort", which after months may so wear upon the ner- vous system as to produce a severe general neurasthenia. Excessive maternal cares are also an occasional cause. In old age the condition is rarely seen, as the old are less subject to strain and to worry. Neurasthenia has been ascribed to " reflex irritation." By this is meant that in some organ of the body, e. g., eye, or ear or nose, or genitals, etc., a condition is present which causes constant nervous irri- tation ; and such constant irritation by the law of summation of im- pulses results in an occasional nerve storm which disturbs the nutri- tion of the nervous centers and leads to irregular and defective action. Some of the worst cases I have seen have been due to ulceration of the rectum long overlooked, or to chronic urethritis with. granulations. This subject is more fully considered in the chapter upon Reflex Neu- roses, page 909. Neurasthenia has also been ascribed to chemical poisons developed in the body by imperfect metabolism, uric acid poisoning, lithemia, oxaluria, or uremia being found in many cases. The uric acid dia- thesis is undoubtedly a factor in some cases, and there are many per- sons who become neurasthenic unless they take occasional doses of calomel, use alkaline waters constantly and refrain from a diet con- taining red meat and alcohol. It is a question whether chronic auto- intoxication by defective digestion is a cause or a result in neuras- thenics, but the two conditions are very frequently combined. Atonic states of the stomach with enteroptosis, distension of the intestines and chronic colitis, rectal diseases, especially bleeding piles, strictures and fissures, are certainly liable to give rise to neurasthenic states. The excessive use of alcohol, or tobacco, or coffee, or tea, may lead to the development of neurasthenia. The neurasthenia seen among syphilitics is occasionally due to that disease ; it is more commonly due to the exhausting effect of long courses of treatment; and it is sometimes entirely independent of the disease and due to the worry or fear that the patient feels in consequence of his infection, his anxiety to marry, or his fear of transmitting his disease to his wife or children. The importance of errors on the action of the ductless glands as a cause of neurasthenia has been urged by Hertoghe ^ and by Levi and Rothschild.^ Temporary cessation of secretion, or temporary increase 'Bull, de la Soc. Med. de Bruxelles, 1909. ^Etudes sur la, phy.sio-patiiologie du Coi-jjs Thyroide et do F liypophyse (p. 02), Doin, Pariw, 1908. 840 NEVEASTEENIA AND TEE OCCUPATION NEUROSES. of secretion of the thyroid gland may produce neurasthenic symptoms ; in the first case attended by minor signs resembling the symptoms of myxoedema, a sensation of chilliness, dryness of the skin and hair, swell- ing of the hands and neck, a slow pulse and diminished menstruation ; in the second case signs resembling those of Graves' disease, feelings of heat, sweating, emaciation, a rapid pulse and severe headache with too profuse menstruation. These can best be treated in the first case by administration of small doses of thyroid extract and in the second by the use of pituitary extract, or belladonna. Injuries of the body, especially if attended by mental shock and fright, are capable of setting up a severe type of neurasthenia. This is particularly frequent after railway and automobile accidents ; and has been termed traumatic neurasthenia or the " traumatic neurosis." It diifers from ordinary neurasthenia in the presence of more acute, intense and variable symptoms, in the prominence of mental symptoms, many of which are of an hysterical kind, and in its longer course. It is probable that the concussion of the body in such accidents produces a physical change in the cells, which for a long time hampers their normal metabolism and causes a permanent exhausted state. The fright probably causes a chemical change in them, with similar results. The disease is more common in men than in women. It is an auc- tion of adult life, but many cases develop early, the large majority be- fore the age of forty, during the period of greatest eifort. Occasion- ally children are affected, especially those who are pushed in school and are made to share in adult amusements. It is more common in cities than in the country. It is more common among the better classes and among the highly educated, than among the laboring class. Cli- mate does not enter as a factor, as it develops in both north and south ; on the plains and in the mountains ; but when a person is neurasthenic, change of altitude has a marked effect. It is more common in winter than in summer. It is more frequent among those people who live under constant strain and efibrt, with few rational amusements ; and who take little out of door exercise ; hence the disease is more com- mon among Americans than among Germans or English. It is a disease of civilization and incidental to the ambition of active men. In women it is often the outcome of a congenitally weak nervous system, over-trained in school, or subjected to disappointment in love. Sometimes ambitions beyond capacity, or the distress after marriage of an unhappy or a childless life produce it. Symptoms. — These may be classified into mental, cerebral, spinal, sexual, vaso-raotor, and sympathetic. The mental and cerebral symptoms are the most common. There is always a certain consciousness of our mental processes, attended by a sense of enjoyment or of distaste, but this in a normal person is never very vivid. The mind rarely watches itself as it acts, and is not conscious of its application, of storing up impressions, or of its acts of reasoning. Thus we have our attention fixed on the result, and not on the process of thought. If mental action becomes SYMPTOMS. 841 hampbred gne of the first results is to attract our attention to the process, and away from the end. The mind labors in its work. It becomes difficult to fix attention. It is hard to remember. It is im- possible to reason consecutively and accurately. Impressions leave little trace. Logical sequences do not follow from premises. And in consequence of this difficulty in mental activity a sense of discomfort arises, a distaste for mental occupation and a feeling of repugnance to effort or to labor. This is what the neurasthenic feels and complains of. He cannot put his mind on his work. He reads, but forgets what he has read. He cannot come to any conclusion about anything, living in a state of perplexity. He accomplishes nothing, and this fact alone causes annoyance and distress. He soon becomes irritable not only at himself and his state, but at his surroundings, family and friends. He wishes to be alone, and then broods on his condition, usually fearing insanity. As soon as he begins to notice his own sensations they all become intensified and many normal feelings usually unnoticed are thought to be symptoms. Thus pulsations in the head, feelings of distress in the head, pressure on the top or back of the head, particularly at the occi- put, constriction about the forehead, tenderness of the scalp or actual pain in the head are complained of. These are classed together as cephalic sensations, and are very common symptoms. The headache of neurasthenia varies in intensity during the day, being increased by mental exertion and relieved by food, it is worse in the morning, often subsides at night, and never keeps the patient awake. Rarely vertigo is felt as well as headache or if there is no actual dizziness there is a sense of movement of the body when at rest, and a feeling of unsteadi- ness in walking giving rise to much discomfort. Numbness in the face, or tongue, or extremities, cold or hot sensations there or in the back ; feelings of weakness in the legs and hands ; inability to use the eyes, unwillingness to listen to music or sounds of any kind, defective appetite and disordered taste, all indicate an imperfect state of function in the cortical centres of sensation. Subjective sensations, such as spots before the eyes, ringing in the ears, or a bad taste in the mouth are often complained of. In the majority of patients an inability to read is noticed. This may be due to a sensitiveness of the optic nerves, so that looking at anything is painful, or it may be due to an inability to focus the eyes, the accommodative apparatus being easily fatigued. More often it is due to the inability to concentrate the attention or to get up any interest in subjects. The cortical centres of motion also suffer. Patients complain of being tired all the time. A continued muscular effort is imjjossible. The grip is poor and becomes worse as efforts are repeated. Tremor of the hands and face and eyelids may be seen. Walking any distance causes undue fatigue. Talking and writing are irksome. The sub-cortical processes of association are equally involved. A face no longer suggests a name. Memories which were formerly grouped together are no longer associated. Hence any thought 842 NEUBASTEENIA AND THE OCCUPATION NEUROSES. becomes slow and more of an effort than usual, and mental application involving many associative processes is impossible. The defects of memory, which are noticed early, give rise to much alarm. The emotional element in mental activity is also affected. Nothing gives rise to a sense of pleasure. All processes become painful, and a sense of discomfort and distress attends all thousrht. A marked depression of spirits is complained of, and the patient fears melan- cholia. His depression differs from that of melancholia, however, as he never blames liiraself for imaginary ill-doing. The true melan- cholic has a conviction that he himself is to blame for his misery, that it is a just punishment for some supposed sin of omission or commis- sion, and that there is no hope. The neurasthenic ascribes his distress to every possible cause except to his own errors of life or work. Morbid fears, termed j^hobias, are very common in neurasthenia. This may be an indefinite sense of apprehension, or it may be a fear of certain things. Thus one patient may fear being in a room or hall or theatre or church, or any closed place, and will rush out into the open air for relief; this has been termed claustro-phobia. Another may fear open spaces, and will go long distances through narrow streets rather than cross a square ; this has been termed agoraphobia. Many fear to make any effort, and will not attempt any in consequence. Many fear some form of disease, and usually make themselves familiar with its symptoms, and then notice their own sensations and misinterpret them ; thus persuading themselves that they have the disease in ques- tion. Physicians often fear locomotor ataxia or paresis when neuras- thenic. The mistakes of the laity are easier to correct. Some have apprehensions about their family or friends, rather than about them- selves, are especially afraid of lightning, of journeys, of fire, etc. In a study of such morbid fears Stanley Hall has described over two hundred varieties which have been noticed. These morbid fears are very common symptoms of the disease, and when they overcome a patient suddenly give rise to attacks of anxiety which are very dis- tressing and may produce physical effects, such as palpitation, pallor, faintness, and exhaustion. Such fears are particularly common in neurasthenia due to alcoholism. The emotional control is very defective in neurasthenia. Patients are easily moved to tears, cry as they relate their symptoms and may act in a way suggestive of hysteria. In many cases there is a mingling of the symptoms of both diseases. Another mental symptom is some morbid impulse. The patient suddenly becomes possessed with an idea which he cannot resist. He may have an impulse to count objects, or to repeat certain words. He cannot sit down, and has to walk about ; he cannot listen, but must talk constantly ; he is beset by doubts, and questions everything ; is perplexed as to whether he has done right or wrong, whether he has told the truth, whether he has remembered important things, whether he is understood by his family or his doctor. Thus his mind, being occupied by these fears and impulses, and by these exaggerated sensa- SYMPTOMS. 843 tions, is inpapable of working along its accustomed lines and is beyond his control. Some patients influenced by both morbid fears and morbid impulses will suddenly leave home and wander away or go on journeys and only after some days realize where they are and the need of informing their family of their whereabouts. They usually return in a bewildered state. The view of Striimpell, that neurasthenia is largely a congeries of mental symptoms, with a disturbed process of thought (Gestortes Vor- stellungs leben), and that such physical symptoms as are present are largely due to auto-suggestion and to the effect of the mind upon the body, is in many cases quite correct. The psychasthenia is often more prominent than the neurasthenia. This is particularly true of the type which develops after accidents, the so-called traumatic neurosis. These mental and cerebral symptoms develop slowly or rapidly, come to their height, remain for some time, subside gradually until finally they all disappear and the patient recovers. Or they attain a certain height and remain for several years, being in abeyance for weeks at a time when life is uneventful and easy, but recurring when- ever an effort to work is made. Occasionally they last for the remainder of one's life. They rarely go on to insanity although occasionally a state of neurasthenia precedes general paresis ; and they never cause a fatal termination. The spinal symptoms develop in some cases to the exclusion of all others, but in many cases of cerebral type there are a few spinal symptoms as well. Pain in the nape of the neck and down the spine is the most com- mon symptom. This is constant and very annoying. It may become so intense as to keep the patient in bed ; he is afraid to move, and shows signs of pain on either active or passive motion. He is tender along the entire spine to pressure, and a light touch may give even more distress than deep pressure. The pain radiates from the back into the limbs, and about the body. Sometimes intense pain and ten- derness are felt in the tip of the sacrum, the so-called coccygodynia being very common in neurasthenia. Such cases should not be sub- jected to operation as the pain is rather a symptom of a nervous state than an indication of organic disease. Usually there is hypersensitive- ness to heat and to cold, especially to cold. The patients complain of undue sensitiveness to any impression, the light touch of the bed- clothes being sometimes annoying, and any jar of the bed distressing. This sensitiveness is greatest along the costal cartilages. There also seems to be some feebleness in motion. The body is moved slowly and with difficulty. The jar of standing or stepping is avoided. The legs give way under the body, though in bed resistance to their movement may be good. Patients often say and believe that they cannot stand or walk, yet under some sudden emotional excite- ment it is usually found that they can do so. These are tlie cases in which "miracles of healing" are observed. The muscles become 844 NEURASTHENIA AND THE OCCUPATION NEVBOSES. flabby and weak from inactivity, but they never lose their reaction to faradism, a most important point in a differentiation from myelitis. The reflexes are usually exaggerated, knee-jerks are high, and a spurious ankle clonus may be elicited, a sort of semi-voluntary pressure downward once or twice when the foot is suddenly pushed backward ; but a true Babinski reflex is never found, a most important fact in a differentiation from lateral sclerosis. But often if the knee jerks are tested again and again in quick succession the response becomes weaker and weaker, showing the easy fatigue of the muscles. An unusual mobility of the iris is often seen, the pupil first contracting and then dilating or oscillating under a strong light. The sexual reflexes are also increased, sexual desire may be excessive, erections and emissions too frequent, but the function imperfect and weak. The bladder may be irritable, so that urination is too frequent. It is rarely painful, and retention is rare. There is usually chronic constipation from an atonic state of the colon. This group of symptoms was formerly called spinal irritation. They are not exclusively spinal, as the mental attitude is never normal, and the attention is always concentrated on all the bodily sensations and functions. This type of neurasthenia is very common after accidents which involve a blow on the back, or a jar to the entire body or a severe fright. Traumatic neurasthenia is more common in cases where no physical injury can be detected. The symptoms have been ascribed to capillary hemorrhages in the cord, to concussion of the cord, to con- cussion or bruising of the spinal ganglia, to strains of the spinal liga- ments, to bruising of the intervertebral cartilages ; though it is ques- tionable whether any of these conditions have been shown to exist. They are really manifestations of an exhausted state and of an imper- fect function in the spinal mechanisms and in the cerebral centres which are connected with and control them. This has been proven by the fact that hypnotic suggestion often produces an immediate cure ; and that firm persuasion of relief by some inert agent often causes these symptoms to disappear. Such symptoms are often ob- served in anaemic young women, who have had menstrual disorders and who gradually sink into a life of invalidism, being bed-ridden for years, the despair of their relatives and the bane of sanitariums. After a number of months, or even two years of intense suffering from these symptoms, the patients usually recover. A few remain in a condition of chronic invalidism, confined to bed, and a burden to themselves and their friends. The vasomotor and syi apathetic symptoms of neurasthenia are almost always present, and in many cases are most intense. A certain tone of the blood vessels is present in health, and is essen- tial to normal activity. The flow of blood to an organ varies with the function of that organ, being intense when the function is being called Into play, and slight when the organ is at rest. Ordinarily one does not notice this alternation of flushing and pallor, or the corresponding lymphatic distension. But in some persons the vasomotor stability is SYMPTOMS. 845 easily affected by various influences. Thus a change in barometric pressure, in the electrical tension, or in the temperature of the air is enough to make the rings on one's finger tight or loose ; to make the tone of the muscles firm or relaxed ; to make the color of the face pale or flushed, to make the pulsations of the heart fast or slow. Corre- sponding changes in mental activity and in temper occur, as has been shown by Dexter, who found that in school children disorder marks from bad behavior coincide with windy days, low barometer and hot, moist air.^ These variations appear to be exaggerated in degree in neurasthenic persons, and when neurasthenia develops they become dis- tressing symptoms, particularly as the attention is frequently directed to them. Sensations of a rush of blood to the head, of flushing of the face or of various parts of tlie body, sensations of cold, accompanied by pallor of the skin are often felt. If a sharp point is drawn across the skin anywhere, but particularly on the trunk, a white line appears, and then a broad red band on each side of it, which may remain for ten minutes or longer. A consciousness of the pulsations of the heart or of the larger arteries is often present and is annoying, especially at night when it may prevent sleep. The heart may be constantly heard to beat from the impulses of the distended vessels in the ear setting up vibrations in the ear drum. A pain like that of angina pectoris in the chest, and especially down the back of the arms and elbows, is sometimes felt. In some cases pressure on a painful point such as the ribs may increase the heart beat 10 or 20 a minute. This is Rumpf's symptom. An actual weakness of the heart is often found, and a lowering of arterial tension, as shown by the sphygmomanometer. A feeling of unusual swelling of a limb may be complained of, and a slight oedema may often be observed, as shown by the rings, collar and wrist bands being tight. Many of the cerebral sensations already mentioned are probably due to corresponding vasomotor states in the brain, as shown by their sudden onset and disappearance. In addition to the rapid alternations of vascular tone, there are often long continued states of venous or arterial congestion of the same ori- gin. These are the conditions which led early observers to describe neurasthenia as cerebral and spinal congestion.^ There is a flushed appearance of the face, a blueness of the lips and tongue, a distension of the retinal arteries, a blueness and coldness with sweating of the extremities, indicative of venous congestion. In women menorrhagia is very common, and all the symptoms of a vasomotor type accom- panying normal menstruation are markedly exaggerated. Many of these cases find their explanation in defects of secretion in the ductless glands, especially in a diminution in the supply of thyroid secretion. The variations in secretion, either of perspiration or of urine, are probably also of vasomotor origin. A patient may be constantly bathed in a cold perspiration, or sudden secretion of abnormal quantity ma,y occur. I have seen one woman from whose hands the sweat 'Weather Influences, E. G. Dexter, The Macmillan Co., 1904, 2 Dr. Hammond, Nervous Diseases, 846 NEUEASTHENIA AND THE OCCUPATION NEUBOSES. dropped for periods of half an hour at a time. The flow of saliva may be increased, leading to frequent acts of swallowing, and this in time may lead to belching and to borborygmi, which are incessant. The flow of urine is often increased, patients finding it necessary to empty the bladder every hour. Jn other cases the urine is much diminished in amount. Much attention has been paid to the examination of the urine, by those wlio believe that neurasthenia is due to chemical changes in the body. In some cases oxaluria is present, crystals of oxalate of lime being found. In many cases an excess of uric acid is a constant con- dition, and the normal ratio of uric acid to urea (1 to 50) is changed, the ratio being 1 to 40, or even 1 to 30. In many cases the excretion of phosphates is increased, rendering the urine cloudy and even milky in appearance. In almost all cases indican is excreted in excess. It is rare for albumin to be found, though occasionally it appears for a fevv days without casts or epithelia. Temporary glycosuria is a symp- tom which is sometimes found. Any one of these conditions is an evidence of some defective process of metabolism, and is usually associated with some form of gastric or intestinal indigestion. It is, however, a mistake to ascribe the disease entirely to this cause, and those who consider neurasthenia as due to lithsemia are as much mistaken as those who formerly ascribed it to congestion of the brain. The general health suifers greatly in neurasthenia, and there are few diseases which cause such continued discomfort. Both mind and body are enfeebled. A loss of weight is common, and patients feel the weakness which attends it. Sleep is usually poor, it is interrupted, disturbed by distressing dreams, does not give a sense of rest, and sometimes insomnia becomes distressing. The patient may be restless at night, fearful of lying awake, and may have to resort to hypnotics to get sleep. More often he is drowsy after a meal, gets to sleep at once on going to bed, but awakes at four or five in the morning depressed and nervous, and does not go to sleep again. The insomnia of neurasthenia is an early symptom and one of the most distressing and difficult to treat. It is better to resort to suggestive treatment for this symptom than to give hypnotics, for these patients become dependent upon any means which has once given relief. For this reason massage, faradism, salt rubs, warm baths, physical exercises, respiratory exercises, inhalations of oxygen or of aromatic alcoholic solutions, various foods, and hop pillows are to be tried. As a rule a neurasthenic feels worse in the morning, complains that his night's rest has done him no good, and has no energy for the duties of the day. In some cases, however, the symptoms subside as the day goes on, and by evening the patient feels quite well and happy, and goes to sleep easily only to wake again at an early hour, depressed and listless. This type of insomnia is usually attended by marked intestinal indigestion, by indican in the urine, and by chronic constipa- tion ; and it is best treated by intestinal antiseptics, rather than by SPECIAL TYPES OF NEURASTHENIA. 847 hypnotics. The digestive disturbances usually last for a long time, often remaining when the other symptoms subside, but gradually they become less marked, and rarely in my experience lead to any gastric or intestinal inflammation. Hemorrhoids which may develop, however, are permanent, and often require subsequent operation. Blood tests occasionally show anaemia, but not uniformly, as the blood is often normal when patients appear pale. In many neurasthenics a marked tremor of the hands is present, especially when the fingers are extended. A tremor of the eyelids is seen when the patient is asked to shut the eyes. Special Types of Neurasthenia. Gastro-intestinal Neurasthenia. — Disturbances of digestion are very common in neurasthenics. A loss of appetite, or a craving for special articles of diet is frequent. Then it is found that many ordinary forms of food produce discomfort, flatulence, acidity of the stomach, heartburn, and even pain. Some patients feel much better after a meal, but in the course of an hour a sense of distension and discomfort in the stomach appears, and they raise sour fluid and gas. This is followed by oppression in breathing, flushes, palpitation, and finally sleepiness. Such symp- toms indicate an atony of the stomach wall. Some patients crave food frequently. Examination of the stomach contents often shows a lack of HCl. In some cases progressive emaciation affords proof that food is not assimilated. One patient, a physician, has an absolute suspension of all stomach digestion with vomiting of undi- gested food for two or three days at a time, when under the anxiety of a serious case. Intestinal distress is also common, and constipation is the rule. The abdomen is sometimes unduly distended with gas, and sometimes an atonic state both of the intestines and of the abdominal walls leads to a state of enteroptosis, which requires treat- ment. Various rigid forms of diet are often followed by neurasthenics, under the mistaken idea that they are suff*ering from dyspepsia, but rarely with good efiect. The most serious symptom of a digestive kind is a prolonged diar- rhoea, occurring chiefly early in the morning and causing great weak- ness during the day. This is accompanied by pain, by expulsion of gas, and is watery and profuse. In some cases diarrhoea occurs only under nervous strain or excitement, subsiding immediately when this is over. Thus one college professor, when neurasthenic, has profuse diarrhoea for several hours before his lecture, but only on his lecture days. Sometimes the diarrhoea is attended by the excretion of long, ropy or stringy masses of mucus, supposed to be casts of the intestine. They are white and firm, and consist of albuminous substances, with epithelia of the intestines upon them. They are only occasionally found in the stools, after specially painful movements, and are attended by an intense feeling of exhaustion. They are often preceded by severe colic. This condition has been described as mucous colitis. It is so uniformly associated with neurasthenia, and its degree corre- 848 NEUBASTEENIA AND THE OCCUPATION NEUBOSES. sponds so closely with that of that disease, that it is now regarded as a symptom. Neurasthenia from Errors of Secretion of the Ductless Glands. — There are certain types of neurasthenia that appear to be traceable directly to an auto-intoxication of the system due to imperfect action of the ductless glands. (a) The thyroid gland when secreting in excess gives rise to symp- toms resembling those of Basedow's disease, but in mild cases where the secretion is not sufficient to cause a very rapid pulse, a bulging of the eyes or a distinct swelling of the neck, all the symptoms of a neu- rasthenic type which are common in Basedow's disease may be present, and the underlying cause may escape the notice of the physician. The symptoms that are most marked in this type of neurasthenia are wake- fulness that is persistent and does not yield to ordinary hypnotics, a mental irritability, fretfulness, restlessness and excitability which is so intense as at times to awaken the suspicion that the individual is ill balanced mentally, and very marked vasomotor symptoms consisting of flushings of the surface, throbbing sensations of the head and all over the body, undue perspiration occurring suddenly, so that the patient will break out into a profuse sweat, feelings of fever without any rise of temperature, and intense congestive headaches. Sometimes in addi- tion to these symptoms there are feelings of tremor through the body and a great desire for fresh air which makes any confinement in a closed room, in theatres or churches impossible for the individual. This combi- nation of symptoms is often termed hysterical rather than neurasthenic and is particularly liable to occur in girls at the menstrual period and in women at the menopause. If the actual origin of these symptoms in the increased secretion of thyroid fluid is appreciated, treatment directed to relieve this condition will soon cause a relief of the symp- toms of neurasthenia which otherwise will resist ordinary forms of treatment. The use of belladonna or hyoscine which tend to decrease the secretion, the administration of rodagen or thyrodectin continuously for a considerable period, or the hypodermic use of the serum prepared by Beebee and Rogers for the treatment of exophthalmic goitre are of great advantage in the treatment of this condition. (6) Another type of neurasthenia due rather to a lack of secretion of the thyroid gland than to any excess in its activity is more common than the previous type. The extreme of this condition is seen in myxoedema, but there are a good many cases which do not go on to development of true myxoedema and yet present symptoms of a nervous kind which are present in that disease. These symptoms are an extreme degree of lassitude, and unwillingness to attempt any physical exertion or to take any exercise, with marked fatigue on minor eiforts ; a mental sluggishness and dullness foreign to the ordinary temperament of the patient, manifesting itself in a lack of interest, a willingness to sit for hours unoccupied and a marked loss of memory ; the patients appear to be indifferent to their own condition and show a lack of interest in things that ordinarily would excite them. They are at times somno- SPECIAL TYPES OF NEUBASTHENIA. 849 lent, sleep heavily but do not awake refreshed. They often suffer from muscular pains, especially about the nape of the neck and in the head. They do not perspire freely and the skin is apt to be dry and cracked and may be slightly pigmented ; brown spots appear on the hands or extensor surfaces of the extremities. The hair is dry, breaks off or comes out and is wholly lacking in the natural oil and may turn gray. Even the eyebrows may tend to fall. In some cases the skin may become slightly thickened as in true myxoedema. In this condition the patient is very liable to gain in weight constantly and a peculiar swelling of the extremities or of the body is not infrequently observed, which renders shoes and gloves too tight, rings too tight, though this is not attended by any oedema that can be discovered by pressure. These patients suffer extremely from cold. Their hands and feet are cold constantly. They have creeping sensations down the back and are liable to have what are termed nervous chills without any rise of temp- erature. The appetite is as a rule poor and there is obstinate constipa- tion. This is frequently attended by signs of intestinal fermentation and by the presence of indican in the urine. When a person with neurasthenia is found to suffer from this combination of symptoms, it is probable that her neurasthenia is really due to an imperfect secretion of thyroid glandular extract, and the only relief that can be secured is by the administration of thyroid extract which will be found to relieve, not only these symptoms, but also those of the accompanying neuras- thenia. The administration should be by small doses, one grain or possibly two grains once or twice a day for ten days, followed by an intermission of five days and a gradual increase of the thyroid up to five grains a day given preferably in divided doses during the twenty- four hours. (c) Another form of auto-intoxication giving rise to a marked type of neurasthenia occurs at the menopause, and is undoubtedly due to the fact of the cessation of the internal secretion of the ovaries. This is proven by the fact that a similar set of symptoms may be observed after removal of the ovaries in a person who has not reached the menopause. Some authors ascribe this set of neurasthenic symptoms to chemical changes going on in the liver and spleen and accompanied by intestinal toxaemia. Others ascribe it to the increased activity or abnormal activity of the thyroid gland which accompanies the cessa- tion of the activity of the ovary. The physiological actions of the ovary and thyroid glands are closely related, and many of the symp- toms occurring at the menopause of a nervous kind are similar to those already described are due to an excess of thyroid secretion. It is more probable, therefore, that the neurasthenia of the menopause is due to a lack in the blood of the internal secretion of the ovary. A certain amount of success has attended a treatment of this type of neurasthenia by the administration of luteine prepared from the corpus luteum of the pig. In some cases where the symptoms resemble Base- dow's disease it is wise to add rodagen or thyrodectin to the use of luteine. In other cases where the woman tends to become very fat 54 850 NEUBASTHENIA AND TEE OCCUPATION NEUBOSES. and sluggish at the time of the menopause, benefit is to be derived from the use of thyroid extract. Sexual neurasthenia is a type sufficiently distinct and common to warrant notice. It occurs chiefly in boys and young men, though girls sometimes suffer. It develops in persons who have indulged in self abuse, or in great sexual excesses. The mind being continually cen- tered upon sexual matters, there occurs a constant state of unnatural sexual activity in the spinal centres, which soon results in a condition of abnormal irritation and excessive secretion. Erections occur at slight provocation, emissions are frequent, at first at night with dreams, and later during the day, even caused by imagination ; and soon the excess is attended by a feeling of weakness, of parsesthesia in the organs, and of pain in the back. The erection becomes imperfect and emission premature, and natural sexual intercourse, if attempted, is found to be impossible. This at once affects the mind, causing great depression of spirits and a state of constant introspection and distress. This dis- tress is manifest in the facial expression, which is one of shame and dejection. The patient will not look anyone in the eye, and is reluctant to admit his symptoms or to confess his fault. The fear of being im- potent may entirely suspend erections and a state of psychical impo- tence results, which in turn may be followed by many neurasthenic symptoms. The attention being centered upon the body, external matters are neglected, and hence not remembered, and these patients complain of inability to work and failure of memory. Many of the vasomotor symptoms of neurasthenia are usually associated with these mental and spinal symptoms. There is usually some actual physical disarrangement of the sexual apparatus present in these cases, a chronic urethritis, or a prostatic enlargement or seminal vesiculitis with hyperemia and hypersesthesia of the deep urethra. All of these conditions must be looked for and treated in addition to the attention given to the neurasthenic manifes- tations. The condition is always recovered from if sufficient rest of the sexual organs is given. Some local treatments such as cold baths, the passage of cold sounds or urethral injections of a boric acid solution, or nitrate of silver solutions, may be used ; but the mind must be kept occupied by healthy pursuits and a strong mental persuasion of rapid recovery by treatment must be constantly exerted in order to secure a cure. This is the secret of the success of so many patent remedies, electric belts, etc., which are so widely advertised. Alcoholic Neurasthenia. — Alcoholic neurasthenia develops in persons who drink steadily for a long time, taking small amounts of alcohol frequently, but who do not appear to be intoxicated. Thus the cause of the condition may not be evident and may be concealed intention- ally. It presents many mental and cerebral and vasomotor symptoms, and tremor is usually very marked. These patients are always the victims of morbid fears. They often show progressive emaciation and I have known women to be reduced to 80 pounds. Digestive disturb- ances are very common. All the symptoms are temporarily benefited by SPECIAL TYPES OF NEURASTHENIA. 851 a drink of whiskey, but when it is cut off these patients sink into a state of extreme exhaustion witli great depression of spirits. These cases are particularly obstinate and the prognosis is worse than in any other type. Nicotine Neurasthenia. — In some persons the long-continued or the excessive use of tobacco sets up a nervous condition, the special symp- toms of which are a sensation of general weakness with rapid pulse and palpitation of the heart on exertion, diffuse pains in the chest, back and limbs, slight numbness and awkwardness of the fingers, fine tremor of the fingers, and disturbed sleep with fearful dreams and occasionally persistent insomnia. A few cases of this kind have had intermittent claudication or astasia-abasia.^ In many cases of neurasthenia the temporary or permanent cessation of the use of tobacco, tea, and coffee proves of great service. I have seen a case in a tea-taster who pre- sented identical symptoms to those caused by tobacco, and who recov- ered when he ceased to take tea. Syphilitic neurasthenia. — Syphilitic neurasthenia has no special features excepting for the fact that all the symptoms are worse toward evening and insomnia is very marked. Many patients with syphilis worry themselves into a neurasthenic state over the fact of having the disease. They should be given a thorough antisyphilitic treatment, preferably at Hot Springs, Ark., and then with the assurance that when this element is eliminated the treatment for the neurasthenic state may be successful. Climatic neurasthenia. — Climatic neurasthenia is a rather severe type of the disease which develops in those who go into and reside in a tropical country. Americans in the Philippines and Panama, and Englishmen in India or Africa are particularly liable to develop this type. It is the combination of heat and moisture which seems to affect the nervous system and when this is kept up night and day it produces serious effects. There is a feeling of general lassitude, an unwilling ness to make any effort, mental or physical, a complete exhaustion when such efforts are made ; and this is soon followed by headache, by insomnia and by imperfect memory and by great mental depression. Cases related by Woodruff^ may be quoted. " One young vigorous staff officer who seemed always cool complained to me one day that on a previous afternoon at five o'clock he found that he could not remem- ber anything he read. When he got to the end of a sentence he had forgotten the beginning. He was completely disabled until the next morning. A young surgeon informed me that before he was half through his rounds he daily discovered that he could not remember the patient's answers, and he always had to lie down thirty or forty minutes before the brain would work perfectly again." Such condi-' tions of exhaustion induce men to take stimulants and to depend too much upon them, for they give temporary relief. But no stimulation in the long run is of use and other more serious effects follow. A I Frankl Ilocliwart. Deut. med. Woch., Dec. 14, 1911. > Woodrufi. The Soldier in the Tropics. Phil. Med. Jour., April 7, 1900, 852 NEUBASTEENIA AND THE OCCUPATION NEUEOSES. state of mental irritability develops, an intolerance of criticism or of interference, and a lack of self control of which the victims are them- selves ashamed. The physical symptoms are those of general weak- ness of the limbs, inability to endure any exercise, irritability of the bladder, sexual impotence, and many vasomotor disturbances ; a rapid heart, dyspnoea, a cyanosis of the extremities, oedema, undue sweating and in women menorrhagia or metrorrhagia. Such a neuras- thenic state predisposes one to all the dangers of a tropical climate, to malarial fever, to typhoid fever, to dysentery and to sunstroke. This neurasthenic state has been ascribed ^ to an ansemic state of the central nervous organs on account of the constant dilatation of the arterioles in the skin due to heat. The body is kept cool by the radiation of heat and by the evaporation of perspiration secured by dilatation of the arterioles but this removes just so much blood from the brain and cord and diminishes their nutrition and functional capacity. Another theory to account for the neurasthenia of the tropics has been advanced by WoodruflP, namely, that it is due to the action of the actinic rays of the sun which destroy protoplasmic life. Pigment in the skin protects man from these rays, hence negroes, and brunettes are less liable to neurasthenia of the tropics than are blond whites. The actinic rays are supposed to stimulate the nerves and especially the vasomotor nerves, causing a dilatation of the vessels ; they are also supposed to stimulate the blood-forming organs ; and owing to the increased flow of new blood cells to the tissues cell growth is excited and new tissue is formed. It is the overstimulation of the constant action of the actinic rays which is supposed to cause the neurasthenia. The remedy suggested is to wear black under- clothing next the skin and white linen outside and avoid the sun. The only effective remedy in this condition is a change of climate. If patients can get to a high mountainous region where the nights are cool, they gradually improve. Thus the mountains of the Philippines or Simla in India are resorted to by travellers and residents when overcome by climatic neurasthenia. The other remedies for neuras- thenia, especially strychnine and tonics and cool packs, are of use in this condition. Neurasthenia in Childhood. — While the disease may develop at any period of life, attention has recently been called to its appear- ance in childhood. There are many nervous children, who when over-stimulated to work in school, or when subjected to too much excitement in social life become neurasthenic. They complain of head- aches, insomnia and indigestion ; are unable to apply their minds to study ; are fretful and irritable and hard to manage, and may even become depressed and anxious about their own condition. Chvostek has noticed that in nervous persons percussion over the branches of the facial nerve causes twitching of the cheek and lip muscles, and this sign is usually to be found in nervous children. Unless they are allowed to stop school work, and are sent to the country for rest and 1 Fales. Amer, Jour, Med. Sci., April, 1907. mAmosts. 853 play these symptoms may last some time. But under proper care, and hygienic surroundings they recover more rapidly than adults. Course. — The course of the disease is always slow and it lasts a long time, no definite limit being possible. In many cases a few cere- bral, some vasomotor, and very slight spinal symptoms develop, and the patient is obliged to give up work and to lead an idle life. If he is properly directed and treated he improves in a few weeks, and after three or four months is able to go back to work under restrictions. In some cases the cerebral symptoms are attended by very great mental de- pression, and melancholia may be feared. But true insanity, with de- lusions of guilt and impulses to suicide, rarely if ever develops from neurasthenia. Many of these patients drift into a condition of hypo- chondriasis, their only thought being of their symptoms, and their complaints being endless. This state often persists several months or even years, not, however, becoming worse, or leading either to death or to insanity, as the patient fears. In these cases relapses are frequent if recovery occurs. In some cases the spinal symptoms are most prominent, and in these the patient is often bedridden for months or even years, and usually drifts into a state of hysterical self-concentra- tion without depression of spirits. In the traumatic cases both spinal and vasomotor symptoms are fully developed, and the hysterical con- dition becomes well marked as the case goes on. These cases last for years, sometimes improving, especially if claims for damages are suc- cessful, but rarely recovering entirely for several years, and sometimes not at all. The cases in which mucous colitis develops are also very liable to relapses, especially under mental worry. Diagnosis. — The diagnosis of neurasthenia, like that of hysteria, is to be made by exclusion, and not hastily. It is to be remembered that neurasthenia is often a condition secondary to some other serious dis- ease, or may be the precursor of some organic affection. Hence the physician should be on the alert to detect a train of symptoms indica- tive of such diseases and should never neglect to make a careful phys- ical examination. Since paresis begins with the symptoms of neurasthenia, it is well to remember this fact ; and to test the condition of the pupils, which are rigid in paresis ; the state of the knee-jerks, which are lost or ex- aggerated in paresis ; the mental state of the patient, which is hopeful, excited, optimistic with lack of judgment, and errors of memory in paresis ; and the power of speech, which is tremulous and indistinct in paresis. Tremors of the face, eyelids and hands occur in both cases. Neurasthenic symptoms are not uncommon in many organic diseases, especially in multiple sclerosis, and in the arterial conditions which precede apoplexy. Headache, vertigo, insomnia, and inability to work continuously, or to remember are common premonitory symptoms of apoplexy. If the arterial tension is high, tlie heart hypcrtrophied, the urine of h)W specific gravity and contains a trace of albumen, and if the retinal arteries are seen by the o])hthalmoscope to be tortuous or 854 NEURASTHENIA AND THE OCCUPATION NEUROSES. to show sclerotic patches the patient is not suffering from primary neurasthenia. These diseases, however, gave rise to definite symptoms and physical signs of their own, which will be found in addition to the neurasthenic symptoms, and thus will lead to a correct diagnosis. Neurasthenia and hypochondriasis are closely allied, but the pre- ponderance of mental symptoms with absurd apprehensions regarding some particular organ or function, and, the absence of any spinal or vasomotor symptoms in the latter usually enables a diagnosis to be reached. A true mental and physical exhaustion, and attacks of sud- den morbid fears are present in the neurasthenic cases, and absent in hypochondria. Melancholia is distinguished from neurasthenia by the intensity of the mental depression, by the development of delusions of self-accusa- tion, by the loss of weight, persistent insomnia, especially the early morning waking, and by the facial expression which is often anxious, but never sad in neurasthenia. It is well to distinguish between neurasthenia and psychasthenia, though they are often associated. In the latter the mental symptoms are much more prominent than the physical and are only removed by psychical treatment. In the former though mental symptoms may occur they are of minor consequence and the treatment which succeeds is that which is addressed to the physical condition. The character- istics of psychasthenia are discussed in the next chapter. Prognosis. — It is evident from what has been stated in regard to the causation and to the course of neurasthenia that the prognosis is not very favorable. Recoveries occur after some time, as a rule, but relapses are very frequent, and can only be avoided by care in the regu- lation of life and of work. A person who has once had an attack of neurasthenia should therefore be particularly on his guard against over- work or anxiety. The less the evident predisposition to the disease, the better is the prognosis. The shorter the period through which the exciting cause has acted, the better the prognosis. The more rapid the onset of symptoms, the better the prognosis. A preponderance of cerebral symptoms implies a shorter duration than a number of vaso- motor and spinal symptoms. Vasomotor symptoms usually last longer than others, and are more difficult to treat. The prognosis in trau- matic cases is never good, absolute recovery being rare, even after damages have been obtained by litigation. The anxiety of legal pro- ceedings always intensifies and lengthens the symptoms. The prog- nosis in spinal cases is almost always bad. In sexual neurasthenia the prospect of recovery is very good, as the patients are young and suggestive treatment is effectual. The longer the period of time given up to rest and treatment, the better the chances of avoiding a relapse. Treatment. — A thorough examination of a person suffering from neurasthenia, with a view to eliminating all possibilities of organic disease, is not only necessary for the physician, but does much to obtain the confidence of the patient; and in neurasthenia his confidence is the first necessity. His mental state of apprehension can be met only by rational encouragement, based on frequent examinations ; hence per- TREATMENT. 855 sonal influejace is a factor in the treatment. The effect of mental sug- gestion upon neurasthenic and psychasthenic states has always been appreciated by physicians and many remedies which have been employed in its treatment — particularly electricity — have been suc- cessful chiefly in proportion to the expectation of cure which they ex- cited. Every physician appreciates the fact that he has a certain moral influence over some of his patients and not over others. He knows that his statements with some carry conviction and that this conviction is an important aid in the effects he tries to produce. This element of therapeutic success has of late been called to the attention of the public, and there is now some danger that it will be overesti- mated and possibly employed to the exclusion of other means which are also needed. Positive statements to patients are necessary in the management of neurasthenia, and, confidence once secured, hope and comfort may be instilled directly both by firm persuasion and honest admonition. The object sought is to put the patient's mind into a different attitude, to displace fear by confidence, to displace despair by hope, to displace timidity by self-reliance. It is well to enter fully into the trains of thought which have led to the patient's state of mind, for if they are discovered the means of counteracting them may be clear. The analysis of the origin of the symptoms may show how to persuade the patient that they are foolish and can be overcome. Such patients must be listened to patiently, must be argued with constantly, must have daily support and comfort and must feel that they are being continually thought of and worked over. The physician who can live with a neurasthenic, or travel with him and give him constant mental stimulus will do more in a week than one who sees him once daily for a month. One reason for the success of all those movements which involve the calling in of a supernatural element in. psychotherapy is the conviction conveyed to the mind of the patient that he has with him a supernatural assistance which is always present, "an ever present help in time of trouble," one to which, by prayer, he can at any time appeal. It is easier to attain success by treating such patients in a company where the hope of others and the confidence of the crowd stimulates the individual as no mere personal admonition can : hence the cures of Lourdes and of the Emmanuel movement in Boston which supply all these elements of success together. For the sceptical patient it is well to resort to more philosophical argument ; to interest him in his own psychological problems and to allow him to read and study such subjects as will give him information as to his own state, thus helping him to overcome foolish doubts, fears, and im- pulses by showing him their absurdity. For the ignorant and unedu- cated an analogous means is to resort to some agency to them mysterious and wonderful — like the sparks of frictional electricity — accompany- ing the treatment by assertions that it will cure. There is usually a tremendous unconscious waste of nervous energy when a person is suf- fering from nervousness. The muscles are held rigid, the body is seldom relaxed, and the mind is in an equally tiresome state of tension. 856 NEURASTHENIA AND THE OCCUPATION NEUBOSES. The cultivation of the faculty of relaxation of both mind and body is to be commended to ueurastlienics. Nothing should be done hastily, or under stress. A quiet mind will help a restless body.^ All the causal factors in a neurasthenic patient should be studied, and so far as possible eliminated at the very outset. All work should be stopped, and any source of anxiety removed if possible. Then the patient should have an entire change in his physical and mental sur- roundings and habit of life. For this reason travel in a foreign country, or a sojourn in a well-regulated sanitarium where the food is good, are very advisable. If these are impossible residence in the country on a farm, with a routine life and some exercise, is to be commended. In any event the mind of the patient should be urged into new channels, to the exclusion of those worries, fears and thoucrhts of work, which have induced his breakdown. The mind cannot be idle, and rest of one set of ideas is to be attained only by sub- stitution of a new set with some interests. Hence every kind of novel occupation, farming, carpentering, photography, sketching, the study of new languages or of art or of science, or some mechanical occupation ; the pursuit of fishing, or hunting ; new games, both out- door and indoor ; unaccustomed kinds of reading ; all these are valu- able, as pastimes during the period of necessary rest. The mere fact of leaving home and getting away from work and worry is sufficient in many cases to arrest many symptoms ; and a complete change of scene is all that is needed to secure a cure in the course of three or four months. It is rarely of use for a shorter period. Physical treatment. — In more severe cases it is necessary to enforce a rigid regime of life ; to make the patient remain in bed for breakfast, rise at ten and take a warm bath (98°) for ten minutes followed by a cool (80°) shower or sponge, and a brisk rub with a coarse Turkish towel, spend an hour twice a day in physical exercise followed by complete rest lying down ; spend another hour twice a day driving or riding on horseback ; rest an hour after meals ; keep himself entertained by being read to, or by playing games when in the house ; to advise massage, and to enforce an early hour of retiring. The alternation of effort, or some form of treatment and rest is to be kept up for a number of weeks. In extreme cases the rest cure of Weir Mitchell (see page 843) may be carried out, but it is rarely as successful in neurasthenia as in hysteria and I much prefer a regime which affords a variety of occu- pation to the rest cure. In all cases a careful regulation of the diet is to be observed, plenty of good nutritious food of every variety being given, simply cooked, and all stimulants being excluded. Coffee and tea may be used in moderation, if they do not excite the patient, and tobacco need not be forbidden unless it evidently disagrees. Rich food and too much food at one rneal are to be avoided. When milk agrees it may be taken freely. Occasionally, a simple restricted diet of milk, eggs, chicken 1 See Power Through Eepose, by Anne Payson Call. TREATMENT. 857 and vegetables with fruit, for a few days, will correct a state of indi- gestion witn loss of appetite, and then a full diet can be added. The object of all treatment being to increase nutrition, limited diet is bad. In all cases an ample supply of water is to be taken, to promote metab- olism and to aid in the elimination of waste products. Various forms of water treatment are of much service in the treat- ment of neurasthenia, as whatever promotes the flow of blood and lymph through the tissues increases nutrition. A warm (98°) bath, or a hot (104°) bath, followed by a cool shower or spray, is to be given daily. If it promotes sleep it may be given at night. Usually it is better in the morning. A spray douche thrown with some force upon the back and played over the entire body, beginning at a tem- perature of 90°, being run up to 105° and then down to 70°, the duration being about 4 minutes, is very stimulating to the circulation. A hot box, in which the patient sits with his head out, the air being heated to 150° for 10 or 15 minutes, followed by a spray douche for 1 minute, at first warm, then cooler, is excellent. A pack, in which the patient is enveloped in a sheet, wet with water at 80°, each limb being wrapped separately, and then rolled in three thick blankets, and in which he lies for 15 minutes, being sponged off when taken out with water at 75° and then rubbed briskly, is stimulating. Sponging the entire back with very hot and then with cold water for two or three minutes is excellent. Salt rubs, in which the surface is rubbed with cloths wet in brine or with hot or cold water in which sea salt is dissolved, the skin being dried rapidly afterward, are very refreshing. Massage is also of the greatest service in increasing nutrition by emptying the lymphatics thoroughly and promoting the venous circu- lation. It may be combined with either passive or active exercises, and these, if carefully graduated and increased daily, will restore the muscular tone. Some of these forms of external treatment should be given twice a day to every neurasthenic, each to be followed by a period of rest and a little simple food. The time may be determined by the patient, morning and late afternoon being the best, as water treatment at night often prevents sleep. Massage sometimes induces sleep. Electrical treatment, either faradism, galvanism, or static currents have never seemed to me to be of direct benefit, but may act by sug- gestion, and this is often necessary. General faradization is of service. The patient places the feet on one electrode, while the other is passed over various parts of the body, a mild current being applied. Gal- vanization of the spine may be given, one electrode being placed on the neck, and the other over the sacrum, or passed up and down the back, no interruption being made. The current should be mild (20 milliamperes with electrodes 4 inches in diameter) and may be con- tinued 10 minutes. Static electricity is convenient, as it can be ap- plied over the clothing, and the smaller sparks or the breeze are often 858 NEURASTHENIA AND THE OCCUPATION NEUROSES. agreeable. It is my experience that these forms of treatment are only successful when accompanied by constant positive statements that they will do good, and will relieve certain symptoms, and only when given by some one in whom the patient has confidence. Hence I regard the effect as chiefly due to suggestion. A change of climate is often of service in chronic cases. Anaemic and poorly nourished patients do well at the seashore, in mild climates, and should avoid the cold in winter. Southern California and Egypt are par- ticularly suited to such cases. Robust or fat patients do better in high altitudes, and for them Colorado or the Engadine may be advised. In spinal and sexual neurasthenia a light application of the Paquelin cautery to the spine, or a series of minute blisters, or the application of strips of plaster to the spine, plain or medicated with capsicum or belladonna, may give relief. Ice bags are sometimes of service. In the sexual cases the passage of cold sounds, or the insertion of medi- cated tampons is useful, acting chiefly by suggestion. In these cases mental occupation is necessary, and it may be well to keep such patients at work, and to urge exercise in the open air. They should also be put on a rigid diet, excluding all foods which excite the sexual appetite, viz. : oysters, meats, condiments and cheese. They should avoid alcohol, and never be given strychnine. Thyroid extract in small doses (1 to 3 grains twice a day) has a very beneficial effect in the cases which present vasomotor disturbances, or where the skin is dry, the hair brittle, the surface subject to irregular swellings, the muscles and joints painful, and where chilly sensations are frequent. In these cases it is found that the headache, lassitude and feeling of exhaustion gradually disappear under the use of the extract, but return of it is discontinued, hence the treatment has to be kept up for considerable periods of time. The use of drugs in the treatment of neurasthenia is to be avoided. Patients rely upon and expect much from them, and are disappointed. The disease is a slow one and no drug can be safely kept up for a long time. The market is full of nerve tonics which are largely alcoholic and contain strychnine. These do harm in many cases. If anything could be found which would supply nutrition to exhausted nerve cells, it would do good. Possibly the glycero-phosphates of lime and soda, or the cacodylate of soda, used either hypodermically or by the mouth may have this effect. They may be tried. The chief use of drugs is to combat certain symptoms. In some cases quinine, iron, or arsenic are of service in helping nutrition. When indigestion is present, bit- ter, non-alcoholic tonics, mineral acids, and intestinal antiseptics are often of use. The bowels should be kept open, and the kidneys active by the use of water. If laxatives are begun they often have to be kept up ; while massage of the abdomen, especially by a heavy ball of rubber filled with lead shot, and an occasional large enema will do equally well, especially where diet is regulated. Great nervous irrita- bility with intense morbid fear may be benefited by moderate doses of bromide of sodium, but this is not to be used constantly. The vaso- TSE OGCVFATION neuboses. S59 motor sympj^oms are sometimes helped by ergot or adrenalin if the pulse tension is low, or by nitroglycerine in ^^^ grain dose, or by 3 grain dose of chloral hydrate if the pulse tension is high. They are always benefited by water treatment. Headache is best treated by massage or shampooing, by cold cloths, or by static electricity. The coal tar products may be tried, but usually fail in neurasthenic pain. Minute doses of codein gL- grain, or of ignatia j-^q-^ grain repeated every 5 minutes for 10 doses often help pain and restlessness and fears, but both are to be used seldom, and with caution. Hyoscyamus in tincture, used in dose of 5 drops every 15 minutes for 3 hours, may also quiet fears. Tincture of lupulin is sometimes of service. Sleep- lessness is usually due to fear of lying awake in neurasthenia. A warm bath at night, massage at night ; or warm food on going to bed may secure sleep. Sometimes a little whiskey at bedtime, in milk, or a glass of beer, will have this effect. Placebos of all sorts are justifi- able. Occasionally trional or veronal may be needed ; if so, let one large dose (10 grains) be given once, and a smaller one on following nights, the patient being unaware of the change. These drugs should be given with some food, hot milk, or chocolate. Bromide may be used as an alternative. No hypnotic should be used continuously, lest a habit be fostered. THE OCCUPATION NEUROSES. PROFESSIONAL CRAMPS. The occupation neuroses are forms of functional disturbance whif.h arise as a result of the frequent repetition of some fine act of coor- dination. Their chief characteristics are that the symptoms are confined to a grouj) of muscles accustomed to act in harmony for the performance of some special professional action, and that it is only in the perform- ance of this action that they appear, the same muscles acting perfectly when called on to perform other acts. Any finely coordinated act requires the orderly and adjusted contraction of a series of muscles in proper sequence, and this is secured by impulses sent out from the educated set of interrelated nerve centres. If the act is repeated too often, fatigue results. If, in spite of the fatigue, the act is continued, structural changes in the mechanism underlying it may be caused. These changes may be in the cortical centres of direction ; in the sub- cortical tracts of transmission, i. e., the motor tracts, the spinal neurons, or the nerves ; or in the muscles. One or all give out under the excessive use. Symptoms of exhaustion follow, attended by a sense of fatigue, a feeling that continuance of the act is impossible, and finally some revolt in the mechanism, occurs such as a spasm of the muscles concerned, or a temporary weakness in them, and these may be attended by pain. In some cases the spasm or cramp in the muscles is the main symp- tom. In other cases it is a functional exhaustion which is felt giving rise to a sense of paralysis. In other cases still it is discomfort or 860 NEURASTHENIA AND THE OCCUPATION NEUROSES. even pain in the muscles which hampers or prevents the act and is attended by tremor. Cases have been classified as spasmodic, paralytic, or tremulous, according to the predominant symptom. The location of the symptoms will depend entirely upon the mus- cles involved in the act ; though as the majority of the occupation neuroses occur after repeated acts of fine co5rdination done by the hands, it is usually the upper extremity or extremities in which the symptoms appear. While the most common occupation is that of writing, and writer's cramp or writer's palsy is the most common of these neuroses, yet Fig. 310. Fig. 311. Writer's cramp, showing the spasm lifting the pen from the paper and arresting ail motions of the finger and thumb. almost any occupation which implies the constant repetition of a move- ment may lead to the disease. Figs. 310 and 311 show two positions spasmodically assumed on an attempt to write, the pen being with- drawn from the paper; at the same time pain was always felt at the base of the index finger. Thus telegraphers, typewriters, pian- ists, violinists, drummers, machinists, cigar makers, pressers and ironers, milkers, plasterers, those who use a hammer or brush, or scissors, those who sew or knit, are all liable to develop a cramp or palsy when they attempt to do their special work. The cramp may be felt in a TRE OCCUPATION NEUEOSES. 861 single muscje, in one or two fingers, or in the entire hand or in the wrist, or even in the entire arm and shoulder. The muscles affected become suddenly stiff, and voluntary motion is suspended for a few moments. If the accustomed posture of the hand is changed, or some other motion made, the cramp relaxes, but it returns when the attempt is made to resume the work. There is a distressing sense of strain and tension in the muscles, which sometimes is painful, and in many cases there is a fine tremor of the fingers. In many cases there is tingling and numbness in the extremity affected and occasionally this is attended by actual pain. In other cases, instead of a cramp there is a sudden giving out of power. The muscles relax in spite of effort, the pen or implement falls out of the hand. Yet there is no true paralysis, for any other motion that is attempted can be done well. The entire disease consists of a suspension of an overworked function. Occupation neuroses in the legs have been observed in those who work pedals, as in turners, polishers, sewing machine workers. Ballet dancers who stand on the toes are occasionally affected. The facial muscles are occasionally the site of occupation spasms. Musicians who blow the horn, trumpet or bassoon, artisans who use blowpipes in mak- ing glass or in chemical works have had spasms of the orbicularis oris. A cramp of the eyelids has been observed in microscopists and in watchmakers who use a lens constantly. It is my experience that occupation neuroses develop only in those persons who are subject to the other causes of neurasthenia and are in some degree neurasthenic. The occupation neuroses may then be the chief active symptom of the general affection, which will be found, however, to exist when it is searched for. Oppenheim agrees with this view. As a confirmation of this it may be noticed in every case that the individual is extremely sensitive about his trouble, greatly worried over it and presents many signs of extreme nervousness. Hence in the etiology, prognosis and treatment of the disease, the facts presented in the foregoing chapter on neurasthenia may be referred to. Roskan has found fibrous nodules within the muscle affected near its tendinous insertion in a number of cases and has removed these nodules surgically with good effect. This finding is, however, excep- tional, and in the cases in which it occurs all motions of the muscle involved are interfered with, not merely those involved in the profes- sional act. In quite a number of cases which have been traced to an actual neuritis in a single nerve the same fact has been evident, viz., that the cramp or pain is not limited to the professional action. In these cases tenderness along the nerve and some paraesthesia or anaesthesia have been found. In other cases a general arterial sclerosis has been found, and the onset of the symptoms suddenly and their relief when the position of the limb is changed, has been ascribed to vascular spasm, such as occurs in intermittent claudication. These three conditions then, though capable of producing symptoms 862 NEURASTHENIA AND THE OCCUPATION NEUBOSES. of professional cramps, should not be classed with occupation neuroses, but should be differentiated from them. The prognosis is good for recovery, provided a sufficient period of rest can be enforced. This rest must, however, be absolute rest of the function affected. If it is writer's cramp, the pen or pencil should not be touched or the fingers placed in the writing position for two years. Sometimes patients try to learn to write with the left hand, but, if they do, it is not unlikely that the cramp will develop in that hand also. Hence absolute cessation of writing is to be advised. If the rest is not of sufficient duration, a relapse is likely to occur. Treatment. — In addition to absolute cessation of the employment which has led to the neurosis, all the measures already described in the treatment of neurasthenia are to be employed. Massage of the hands and arms is particularly useful, with Swedish movements of re- sistance. In the early stage of writer's cramp, it is sometimes possible to give relief by suggesting unusual methods of holding the pen or by wearinar on the hand or wrist some form of bracelet which will hold it. Sometimes if the pen is wound with twine or is passed through a cork, it can be held in the palm of the hand and all pressure of the fingers can be obviated. These devices, however, only delay the prog- ress of the affection, which must be treated in the end with absolute rest. When writing is inevitable, a typewriter can be used. CHAPTER LI. PSYCHASTHENIA. PsYCHASTHENiA is a Condition of abnormal action of the mind manifesting itself in errors of judgment, in the dominance of certain ideas which are known as obsessions, in abnormal emotional states lead- ing to unwarrantable fears and states of anxiety, in abnormal impulses to actions which are known to be purposeless or foolish and in states of mental perturbation, of pain and of disorders of consciousness which render ordinary occupations impossible. The absence of hallucinations, illusions or fixed delusions and the absence of continued depression or excitement, separate this class of mental symptoms from those of insanity in its well-recognized forms. Etiology. — Psychasthenia as a rule develops in individuals who by their inheritance have been predisposed to a weakened nervous system, or who have acquired it by any of the causes which have been mentioned in connection with the etiology of neurasthenia. In fact, to discuss the causes of psychasthenia would be to repeat what has been stated on pages 838 to 840. Psychasthenia may develop as a complication of neurasthenia, and there are very few neurasthenics who do not present some symptoms of psychasthenia, but they are not necessarily associated. In the majority of cases of psychasthenia there does not appear to be any real weakening of the mind, as the term implies. There is however a lack of balance between the various powers of the mind, and a lack of judg- ment and of self-control. In a normal person morbid thoughts, morbid impulses and morbid feelings, if they occur, are looked upon in their true value and are controlled. All authors agree that an inherited weakness of the nervous system, a congenital condition of degeneracy, an inherent instability and enfeebled power of resistance to strain is to be found in almost all psychasthenic persons. Women are more frequently aflPected than men. The symptoms usually appear at the period of sexual development, may disappear, but recur under any anxiety. The disease usually develops in those who have been exposed to some great emotional strain or shock, and in those who have been unduly worried by family or business anxiety. Long-continued worry is as potent as sudden shock to produce it, especially if that is attended by a mental conflict iK'tween moral dicta and impulses toward conduct known to be wrong. Women contemplating divorce, clergymen wishing to change their call- ing because of religious doubts, business men adopting dishonest methods, persons who have entered on a course of conduct where con- 863 864 PSYCHASTHENIA. cealment, deception or deceit are constant, are subjected to such a mental conflict as to produce anxiety, and to lead to psychasthenia. A similar anxiety may be aroused by self-reproach as to past events. Many cases of sexual neurasthenia are largely psychasthenic in origin. The fears produced by reading advertisements of quack remedies for impotence, together with the self-reproach at past abuse or excesses produce a state of psychic impotence much harder to combat than any of the local urethral conditions accompanying the sexual neurasthenia, and may lead to psychasthenia. Symptoms. Morbid Fears, — One of the most prominent symptoms of psychasthenia is a condition of the mind which is attended by a sense of alarm and a constant apprehension destroying all sense of comfort. This does not appear to be reasonable or to be based upon any definite idea. It is an indefinite sense of fear without reason, but nevertheless dominates the mind as definitely as a reasonable alarm. No amount of argument as to the unreasonable nature of these fears appeals to the patient or succeeds in allaying the sense of distress. As examples of these morbid fears the following may be cited. The patient will be afraid to touch certain objects for fear of the object contaminating him. He will fear to touch a knife lest he may cut himself or lest he may be siezed with an impulse to hurt himself. He may fear to go up a flight of stairs or to come down a flight lest he fall. He may fear to go into a certain room in the house, though he cannot say why this room is distasteful. He may fear to stay in a chureh or theatre with- out any assignable cause. He may fear to walk across an open square and in consequence will go a roundabout way through narrow streets to avoid it, or he may fear to walk through narrow streets near high buildings, and hence make a long detour in order to go through wide avenues. He may be beset by a dread that anything he does is wrongly done. Thus one patient would spend hours putting on and taking off" her stockings, because of an indefinite dread that she had not put the right stocking on first. Another patient would go to the gas fixture forty or fifty times in succession and feel of it, fearing that it had not been properly turned off". A postman lost his job because he feared that he had not locked the last box from which he had made the col- lection properly, and would go back in order to try the box and thus lost time. One patient whenever she sees a match-box wonders if the matches in it are safe ; might they get out ? might they be lighted and set things on fire ? Hence she goes to the match-box and makes sure that it is shut, and may repeat this twenty times. She cannot reason with herself in regard to this, but she has this intense fear of matches. If she has a medicine she fears it will get out of the bottle and do some- one an injury. She goes every little while to feel if the bottle is corked. Another patient, in reading, reads very slowly and notices each word separately, fearing that she will skip a word, and this fear is so constant that it destroys the pleasure of reading. Another patient has a morbid fear of going out of the house ; he feels perfectly comfortable while at home, but nothing will induce him to venture out, though there is no reason whatever that he should not go. It would be possible to go on SYMPTOMS. 865 citing instances of these morbid fears indefinitely. Janet and Stanley Hall have tabulated over 200 examples of such fears, and Greek names have been given for many of them, such as, claustrophobia, fear of closed places ; agoraphobia, fear of open places ; misophobia, fear of dirt ; pyrophobia, fear of fire, etc. These fears are not continuous but occur from time to time in the mind of the patient, giving rise to great distress while they remain, but being entirely lost sight of for hours when the mind is occupied with other things, and if the mind can be very suddenly or forcibly turned into a different channel the fear suddenly disappears and the patient obtains relief. The origin of the fear is never clearly explained by the patient and as a rule is not known by him. Many of the fears are absurd and do not appear to have any rational explanation. Others are manifestly connected with some experience, but in a stronger mind would be com- bated or ignored, and it is the existence of the fear that is the proof of the psychasthenic condition. As we shall see a little later the origin of these fears is probably a subconscious mental process, the emotional equivalent of which appears in the domain of attention while the underlying processes of reasoning remain below the threshold of consciousness, and in proof of this it is urged that these fears can be quieted sometimes permanently but always temporarily by suggestion in a state of hypnosis. Morbid Impulses. — Another of the symptoms of psychasthenia is the development in the mind of a sudden morbid impulse to do some act which may be absurd or may be injurious to some one, and which, though contemplated, is not necessarily carried out. The idea of carrying it out however throws the mind into a state of anxiety which is immediately relieved when the act is done, and which remains if the act cannot be accomplished. Examples of these morbid impulses are the following. The patient will have an impulse in walking on the street to avoid treading on the cracks between the paving stones. He will have an impulse to count his steps. He will have an impulse to count the windows of the houses which he passes or to count the people passing him. He will have an impulse to touch certain objects in the room or to touch a certain part, for example, a button on another person. He will have an impulse to accentuate certain words in a sentence, to repeat a word over or to bring a certain word into every sentence which he utters. He will have an impulse to conceal certain objects or even to steal, there being no object in the theft excepting the gratification of the sudden impulse to take and conceal an object. He will have an impulse to destroy various objects or to set fire to things. It will be noticed that all of these impulses are things which would be natural in a little child, as all children delight in touching, conceal- ing, destroying, burning, etc. More objectionable impulses are those which involve an ilijury to others. 55 866 PSYCEASTHENIA. Thus one patient who lives with two aunts has a constant im- pulse to push these old ladies down stairs, and always hopes that when they step upon the rugs the rugs will slip so that they will fall. She also has an impulse to throw things at them, and although she has never done anything to injure the old ladies or given them any intimation in the course of five years of her actual feelings towards them, as she is dependent upon them, yet her life is made miserable by the presence of these constant impulses. I have known a mother who had a constant impulse to drown or to kill her children when they were infants, and who has been much perturbed by this impulse, though she loves her children and has never done them harm. In the majority of cases no reason can be assigned by these patients for the existence of the impulse. It comes suddenly into the mind with a desire to perform the act and is at once relieved when the act is performed, but gives rise to a state of anxiety if the act cannot be performed. Here again the theory of subconscious unrecognized mental proc- esses is brought forward to explain the condition. In normal mental processes every act is the outcome of a preceding logical train of thought. It is supposed that in the case of a morbid impulse the train of thought occurs below the level of consciousness and that only its conclusion comes within the domain of consciousness as an impulse. The basis of this theory is the fact that in many such cases, if the patient be hypnotized and questioned the train of thought leading to the impulse can be discovered ; and if in the hypnotic state this train of thought be combated and the act which is its logical out- come be shown to be either useless or harmful and a command be given to abandon the thought, the patient on recovering from the hypnosis will be temporarily relieved of the impulse and by repeated hypnosis will be cured. Morbid Ideas. — Morbid ideas may also appear as a symptom in psychasthenia, at times being attended by morbid fears and impulses, but at times being without either element. One patient had the morbid idea that all the Christians were going to kill all the Jews, and was then perplexed with the thought of what Mr. X, who was a Christian, would do when all the Jews were killed, his wife being a Jewess. One patient was perplexed with the idea that electrical influences were acting upon him constantly through wireless telegraphy and that every thought in his mind was controlled by a telegraphic message some- where in the air. A morbid idea which becomes fixed in the mind and which is accepted as true and made the basis of action is termed a delusion, and delusions of a temporary character are not infrequent in psychasthenia. When such delusions become fixed in the mind and so constantly present in the thought as to be made the subject of constant contemplation and of action, the individual is dominated by it and passes from the border land of psychasthenia into the admitted territory of insanity and belongs to the paranoiac type. As a rule such fixed delusions are rarely curable by hypnotism^ SYMPTOMS. 867 although JTanet has reported ^ several cases in which they have been relieved by suggestion. It is not certain that the effects of his treat- ment have been permanent, as the cases may have relapsed after the publication was made. Instability of conduct is so characteristic of persons suffering from psychasthenia that it may be mentioned as a symptom of this disease. The ordinary train of thought presents a definite order and succession of ideas, and there is a logical connection between them. Such a logical train of thought leads in turn to appropriate action and regu- lation of conduct which is manifestly based therefore upon adequate reason. In a psychasthenic individual there is to be found a tendency to most erratic and irregular lines of thought. Ideas occur to the mind without any apparent logical sequence and hence consciousness wanders from subject to subject just as the eye in a picture gallery travels from picture to picture, there being no logical connection between the im- pressions. Hence the contents of the mind are in a jumble and thought is lacking in continuity. A continuous conversation on a definite subject with such patients is impossible, for they jump from subject to subject and do not fix the attention for any length of time on any one. This instability of thought is therefore manifested by similar irregularity of conduct. The individual is liable to enter upon courses of action which a reasonable judgment would condemn and which are soon abandoned as leading nowhere or being futile. Continuity of effort in the conduct of business, in a course of study, in professional life or any definite occupation appears to be impossible and the indi- vidual will vibrate between a series of various occupations, going from medicine to law and thence to theology or abandoning the original profession for business or a business man may change his occupation year after year and thus in the end accomplish nothing. Such indi- viduals are very likely to enter upon foolish schemes and unwise under- takings and cannot be convinced of their foolish nature until they have squandered time and money needlessly. Psychic pain is another symptom of psychasthenia. This type of pain is usually gradual in onset, persistent day and night, not open to any relief by ordinary sedatives or even to morphine, and frequently persists for months or years. Its location may be such as to be inex- plicable on the basis of anatomical structure, as for example in a band about the middle of the arms. The most common pain is over the eyes, over the entire head or at a point over the occiput ; but it may be located in any organ or limb. The exclusion of disease in the part in which the pain is felt, the character and duration of the pain, and the fact that in spite of complaint the patient shows none of the inevit- able effects of actual suffering such as appear in organic disease or in in- cural)le neuralgia, force tlie idea upon the physician that the symptom is really an hallucination of ])ain. And the ])ro()f is afforded in such cases by its immediate relief l)y suggestion in hy[)nosis. ' N^vroses et Id^es fixes, p. 214, Paria, Alcan, 1898, 868 PSYCEASTHENIA. States of double consciousness may be included among the symptoms of psychasthenia. After a sudden mental shock, severe emotion or anxiety an individual may suddenly lose consciousness of his own per- sonality and pass into a state of second consciousness in which his per- sonality as related to his previous mental experience is lost. He is to all intents and purposes for the time being another person. In this state he has no recollection of his previous existence, of his name, address or occupation. He does not recognize places or persons, for- merly familiar, yet he has no difficulty in the use of language and he acts in a manner in accordance with normal moral restraint and in accordance with the social surroundings to which he is accustomed. He may remain in this condition for several days or even for years, and when he comes out of this state into his previous personality the events which have occurred during this stage of second consciousness are wholly obliterated from his mind, so that his conscious existence is continuous with that existence before the occurrence of the second con- scious state. The following examples of this condition from my own experience may be cited : A healthy young man, a football player on one of the university teams, while playing a college match was thrown heavily and for a few moments lay unconscious on the field. Within two minutes, how- ever, he got up and went on playing and during six minutes following he played well, he answered signals of the captain in a proper manner, although several of these signals involved complicated movements and he appeared to be in a normal state. He then suddenly became conscious that he was in a very nervous, trembling condition, and unable to play, and was led off" the field in a state of great nervous excitability, and for several hours was prostrated. When he came to himself, he had a recollection of the fall but he had no recollection whatever of the plays in which he had participated after the fall and which he had carried out with apparent consciousness and perfect accuracy. At the present time his memory extends up to the time of the fall and begins at the point where he felt the tremor and prostration, but he has absolutely no recollection of the six minutes subsequent to the fall during which, as already stated, he played a good game. A man twenty-four years of age, who had been for three months in a state of great mental anxiety, during which time he was decidedly overworked, suffered from an attack of the grippe in March, 1903. On the 24th of March, just as he was recovering from this attack, he went to his office as usual in the morning and about noon left the office to do an errand. He remembers going out and crossing Broadway and having to jump suddenly out of the way of a car, the motorman shouting "jump " as he did so. From this time for the subsequent week he has no recollection whatever of his actions and during this time he was lost to his family, who were correspondingly alarmed at his absence and took measures through the police to try and trace him. These measures, SYMPTOMS. 869 however, were futile and no trace was found of liim until March 31, when a friend met him wandering about in Harlem and recognized him and took him home. On arriving at home he recognized his friends, complained of intense headache, seemed much depressed and very nervous, and said that he had recovered his memory at about ten o'clock in the morning, when he found himself on the elevated railroad between 104th and 116th Streets, much to his own surprise. He got off at his regular station, 125th Street, but did not appear to recognize the streets and was much relieved when his friend appeared and guided him to his house. He remained in his house for the following few days suffering much from headache, but gradually recovered, and when I saw him on the 9th of April was in his ordinary health. He had apparently no recollection whatever of any events between the 24th of March and the 31st of March. On his return he presented a somewhat dishevelled appearance and was without money and had apparently had nothing to eat on that day, as he was quite famished. On cross-questioning, it was found that he had a dim recollection as of a dream of having gone to a hotel down-town for the night and of having come once to Harlem with a view of finding his home, but being unable to do so. Subsequent investigation proved that he had been one night at this hotel, as he was registered there in his own name. He was seen once during the week by a policeman whom he knows down-town, but this man, though knowing of the alarm that had been sent out, did not think it worth while to speak to him or take him home, but told him of the fact later on when he was regularly attending to his business. What his movements were during this period of six days he has not been able to ascertain. He did not go to his office and he did not come to his house. He was a man of good habits. There is no suspicion that he was making this an excuse for having gone on a spree, and nothing in his history points to any reason for such a sudden disappearance. He has been well ever since. It seems probable that the sudden shock or fright at the danger of being run over by a car threw him into a state of second consciousness, from which he emerged spontaneously at the end of a week. A perfectly healthy athletic man was much perturbed in his mind by a business transaction involving the payment of a mortgage. He went to the bank and drew the money to pay the mortgage and he remembers leaving the bank. Three days later he found himself in the railway station at St. Louis, and looked about him in surprise, not recognizing his surroundings. He inquired where he was and, when informed, had no recollection of how he had reached there. Realizing that he had come from New York and being told what day of the week it was, he immediately realized that he had been away three days and that his wife must be anxious about him. He went to the telegraph office at the station to send her word. He could not remember her name or his own or his address. He sat down in a bewildered manner 870 PSYCHASTnENlA. and after fifteen minutes the name of his clergyman in New York came to his mind and he immediately sent a telegram to him, announc- ing his whereabouts, asking him to tell his wife and stating that he was on his way home. The transaction at the bank next came to his mind and he was immediately anxious about the money which he had drawn and much relieved when he found the greater part of it still in his pocket, showing that he had not been attacked or robbed. Within a few minutes he became aware of all his previous existence. He returned home and was brought immediately to me. He appeared to be in perfectly good physical condition, but was much perturbed in his mind at his own experience and was incapable of doing his work or fixing his attention for any length of time upon it. He was emotional and apprehensive and fearful that this was the beginning of a mental breakdown. In the course of a month, however, under a good general regime he had entirely recovered from all these symptoms and for the past year and a half has been in his usual state of health and actively employed. I have seen a man who had been for three months in a hospital in a peculiar state of mind. He had no knowledge of his history previous to the time he had been taken to the hospital in a state of unconsciousness. On recovering consciousness there he did not know his name or anything about himself. After some weeks his skill in the use of tools convinced us that he must have been a carpenter. He has been set to work in the hospital at that trade. But he still has no clue to his previous person- ality. He appears to be a fairly educated, good natured, moral man. 1 could relate eight other cases within my own experience similar to these. Dr. Morton Prince has recorded the more extraordinary case of Miss Beauchamp,^ where during a considerable period of years the individual vibrated between three different personalities, the memories of each of which were separate from the others. Consciousness and the Subconscious. — In the discussion of these psychasthenic symptoms the term subconscious ideas, emotions and impulses has been constantly employed, and before proceeding to discuss the treatment of these symptoms by suggestion, it may be wise to con- sider what is meant by the term subconscious. Physicians use this term much more freely than psychologists. In fact, a considerable portion of the latter refuse to admit any sharp demarkation between the two states implied in the term. MarshalP says that the word consciousness is used to express two diverse meanings : first, psychic existence and, second, awareness. He likens our psychic existence (see Fig. 312) to a mass of fluid, the surface of which is thrown into waves, each big wave having its few minor waves around it, with ripples on their surfaces, but each wave being but a small mass as compared with the greater mass of fluid of which it is composed and by which it is surrounded. Consciousness in the sense of psychic life is represented by the mass of fluid. Consciousness in ^ Morton Prince. Dissociation of Personality, Longmans, Green & Co. 2 Consciousness, Henry Eutgers Marshall, LL. D., The Macmillan Co., 1909, p. 169 et seq. I am indebted to Dr. Marshall for the use of his figures. STmptOM^. S7i the sense of awareness is represented by the wave with its wavelets and ripplef^ A few only of our mental experiences are clearly present to the mind at one time ; an infinitely small number in comparison with the number of experiences which we have stored in memory. The reader^s atten- tion may at present be fixed upon this train of thought and he may be also slightly conscious of memories of psychological discussions or of medical cases illustrating some of the points thus far brought out. He may also be dimly conscious of the surroundings in which he is sitting, of the room and its furniture, of the state of his body as it rests upon the chair in comfort or discomfort. But he is not conscious of the events of the past day or week or month or year, though they lie ready to be recalled by any appropriate associated impulse. In that mass of conscious or unconscious memories there are mingled, as it were, all those precepts of conduct which constitute the moral system and which may be represented for the sake of the analogy as giving a specific Fig. 312. The figure represents a mass of fluid thrown into waves, each wave having ripples on its sides. (Courtesy of Henry Rutgers Marshall.) gravity to the fluid, thus determining in a way the height of its wave. If this analogy be admitted we can see at once that psychic conscious- ness is an element of our entire existence, while awareness is the quality of the thought of the moment. It will also be evident that the sub- conscious, if it be taken to be coincident with psychic consciousness as contrasted with awareness or with the mass of fluid, not thrown up in the wave, is enormous in its extent and complexity as contrasted with the conscious taken in the sense of awareness which is the thought of the moment. Those, then, who ascribe a great effect upon the mind both normal and diseased to subconscious influences, ideas, fears and impulses have certain facts in their favor. For it is clear that the mental life is infinitely wider than the mental state of the moment and must determine in largv. degree that state. It is therefore also clear that it is unwarrantable to make a sharp distinction between the con- scious and the subconscious. Marshall ' gives another diagram (Fig. 313), which he terms a neur- urgic pattern and which may be taken to represent, in a way, the physical l)asis of mental activity. Various groups of nervous elements, representing ideas or memories, are associated with one anotller in closer or looser relation, but no idea exists without some association. ^ L. c, page 37. 872 PSYCSASTHENIA. All of these nervous elements are in some measure active during life and they are so bound together that any emphatic activity in any one element must directly or indirectly influence the activities of all the rest of the elements of tlie system and must in turn be influenced by them. If the activity of some particular system of elements is mark- edly emphasized it will stand away in contrast from the whole great The figure represents the contents of the consciousness; a part, under A, heing present in attention, the portion P representing self-consciousness ; a part, under B, being outside the range of attention and hence subconscious, a part, under C, being so far removed from consciousness as to be almost inaccessible. mass of unemphatic activities. This condition is shown in the figure under the line A, which represents the ideas of which we have an acute sense of awareness together with the presentation of ourselves as think- ing of these ideas and hence being self-conscious or conscious of our own personality (shown in the diagram as P). The immensely complex nervous system is represented in an artifi- cially simplified manner as the groups of circles under line B. This corresponds to our psychic existence or general consciousness, which is not within the field of attention and of which, therefore, we are not aware. But any one of these groups of circles may be reached by means of the direct or indirect lines connecting it with the others, and any group may in turn be separated off from the others as was the group under line A and thus enter the field of attention. If we liken our memories and thoughts to the circles in the diagram it is easy to suppose that some of them may have a weaker association with the rest than others. These are shown under the line C. Only by means of a few lines of thought can we reach these memories. And it is conceivable that even those few lines may be severed, temporarily or permanently, in which case the memory cannot be reached. In the phenomena of aphasia we have studied permanent loss of associations. It is conceivable that in functional conditions a temporary suspension of associations may occur. And this may be taken as the basis of the dissociation of ideas which is hypothecated to explain some of the symptoms of psychasthenia and of hysteria. The suspension of the association cuts off the group from voluntary recollection. It prevents the " awareness " but it does not remove the group from " psychic SYMPTOMS. 873 existence " or deprive it of its power to influence that part of conscious- ness which IS implied in that term. To bring the group again into relation with the other elements — to reestablish the association — certain artificial means may be employed. This means is known as psycho-analysis. In this process a series of ' words, nouns and verbs, taken more or less at random are mentioned, one after another to a patient, and by a stop watch indicating seconds, a record is made of the time elapsing between the mention of the word to him and his utterance of the first word that comes to his mind when he hears the word mentioned. . This reveals the natural association in the mind between the two words and when this associated process goes on undisturbed by any previous emotional association with either word, it takes about a second or even less. If, however, the word uttered awakens a memory that is connected with an emotion, either conscious or unconscious, a certain perturbation of the mind ensues, which hampers the rapidity of the association and is indicated to the observer by the lengthening of the process of association, so that the interval is two, three or even five seconds.^ The words, then, at which such lengthened processes of association are present are taken as a basis for a possible train of thought, and by their reiteration to the patient may stimulate his memory so that it recalls an event of his life which he either wishes to conceal or has forgotten. If this can be recalled and can be described to the examiner, the mere act of confession appears to relieve the mind of a tension which has been the cause of the morbid idea, impulse or fear. It is thus that psychic analysis is not only a means of diagnosis, but a therapeutic measure in the study of psychasthenia. It seems as if a group of ideas which have been separated from the mental contents of the moment, but which nevertheless remain as part of the psychic life, and thus have a sort of consciousness of their own, are by this method reunited to the mental contents and are thus brought again into the field of attention, so that we became again aware of them. Or, to recur to Fig. 313, it seems as if the lines between B and (7, which for a time had been impassable, were again traversed by impulses and thus again the distant circles are associated with the central mass of nervous elements. For a normal action of the mind, therefore, a perfect assemblage of ideas, memories and experiences is a necessity and anything which interferes with such an association or which produces a dissociation causes a disturbance in the process of thought, and disagreeable sensa- tions of discomfort. We are all familiar with the mental stress which is felt when we see a face that is known, but cannot name the person. The mind makes a futile effort and gives it up. But suddenly — when we are thinking of something wholly foreign to the face — the name comes to the mind. An activity has been going on in the psychic con- sciousness not present in the domain of awareness, and the result is the ' See Association Tests, by Wood worth and Wells. Psychological Monographs, Dec, ^74 PSYCHASfHENlA. forcing into the field of attention of a thing apparently without natural association. Many persons have had the experience of going to sleep with an unsolved problem and of waking to find the solution clear to the mind. It thus appears that mental processes may go on outside the field of attention, the result of which comes spontaneously into that field, to the surprise of the individual. This is the explanation of many of the symptoms of psychasthenia. Some act of the psychic life occurs outside the domain of awareness.^ It leads to a fear, or impulse, or idea. That suddenly enters the field of awareness and is termed morbid because it is unexplained, unex- pected, not associated with anything else, and hence surprises and alarms the person. If the origin were known, if the process of thought leading up to it could be traced, it might seem a natural idea or it might be combated as either baseless, foolish or unreasonable. But, like a tune which runs in the head, it forces itself into the field of atten- tion and cannot be voluntarily combated or banished, largely because its origin is unknown. If any means can be found to trace the origin the mystery is solved, the alarm and mental perturbation vanish. Suggestion and Hypnosis. — The only known means of tracing the action of psychic life which is outside the field of awareness is to banish the element of awareness and get below it at the underlying psychic life. This can be done by putting the individual into the state of hypnosis in which the awareness is abolished but in which the psychic life appears to go on unhampered, the associations formerly suspended can be renewed, dissociations can be overcome and a train of thought be followed up from beginning to end or from end back to beginning. The mere act of relaxation, the condition of dreamy state without an entire suspension of awareness may in some persons suffice so that sug- gestion and questioning, without true hypnosis, may be possible. In other persons a deep sleep is essential. To hypnotize it is well to place the patient in a comfortable position, where relaxation of all the muscles is easy ; then to direct his eyes to a bright object held eight inches in front of and a little above his eyes, to tell him to fix his attention on the object and what is said to him, to assure him that his eyes are closing as he gazes, that he is going to sleep, that he is asleep, and as he goes to sleep it is well to press on the eyes with the fingers and to stroke the forehead. The slight change in color, the deeper respiration and the evident relaxation of the body will assure one that the subject is hypnotized. Now in this state a person will answer questions, and will perform acts in response to commands. If morbid fears, impulses and ideas have been present the reason for them will be related in response to questions. If states of double consciousness have occurred the patient will relate in detail all that occurred in the state of second consciousness. If some symp- tom due to a dissociation of ideas, memories or feelings has occurred, such as a fixed idea of pain, a so-called hysterical pain, or a functional ' For a discussion of Freud's theory that these acts of psychic life are based on sexual experiences, see page 888. SYMPTOMS. 875 ansesthesia, *or paralysis or blindness or deafness has been present the assurance that it is cured, that it will not recur when the person wakes will come true on waking — for the association has been reestablished in the dormant state. When the patient is wakened he has no recol- lection of anything which has occurred during the hypnosis, but he acts in accordance with suggestions and commands given in that state. Thus, if he has been told that he will remember the train of thought which has led to the morbid idea, fear or impulse and will see that that train of thought is unreasonable, he will do so and no longer entertain and fear the morbid thoughts. Thus the hypnotic suggestion cures the symptoms of psychasthenia. To ensure a permanent cure, however, the process must be repeated from time to time, the intervals being made longer and longer. In cases where hypnosis is not employed a similar result may some- times be attained when the person is merely put in a state of relaxation and is talked to seriously, being assured that his symptoms are curable and will disappear. But here the seance must be longer, it must be repeated more frequently and the result is of slower attainment. In this respect it resembles the result of mere argument, persuasion, or assurance, which is used by every physician in encouraging a timid person. The results, however, in hypnosis seem more definite and positive than when it is not employed. To awaken an hypnotized person it is only necessary to say " wake up," or to tell him to count 10 aloud and to wake as he says 5 ; or to stroke the forehead upward, telling him that he is waking. It must not be forgotten that any suggestion made during sleep will be retained on waking. Thus if when hypnotized the patient has been told that his arm is stiff and cannot be moved and spectators are shown that it is so for the time, he must be told before waking that the stiffness has gone and that the arm is perfectly well and natural, otherwise on wak- ing a paralysis or rigidity will be found present. And careless remarks made by the hypnotizer sometimes lead to serious mental impressions which remain as symptoms when the person comes out of the hypnosis. If a person has been once hypnotized he is more easily hypnotized afterwards. The process is not attended by exhaustion and seems to do no harm. It should be done, however, for a definite object only, never as a matter of amusement. While hypnotic suggestion is successful in some of these cases the majority of individuals cannot be hypnotized -and very few physicians care to employ this method. Even in France, where it is most in vogue, some authorities — Babinski, for example — have discarded it as either harmful or needless. Under these circumstances treatment must be by other means. The best of these is by education and persuasion. The method is to ex- plain fully and repeatedly to the patients the import of the symptoms present ; to ol)tain their confidence by patient listening to and interest in tlieir mental states of doubt, fear, impulse, or perplexity ; to argue out the imaginary element in the condition ; to urge the power of 876 PSYCHASTEENIA. controlling the thought and to impel the patients to make a constant effort to combat the fear or impulse. This is the method urged by Dubois aud by Babiuski. It awakens the belief in the prospect of a cure aud stimulates the courage of the patient. It implies the fact that by conscious effort the subconscious train of thought may be controlled. That effort must be constant, or frequently repeated, and must be aided by the encouragement and personal direction of the physician. It shows the patient the difference between real and imaginary symptoms and shows him what hope can do. It is effective in many cases and should always be employed by the physician. The psychasthenic should be treated not only mentally but her mind should be given constant occupation. This is to vary with every hour of the day, and should involve the use of the hands, sketching, wood carving, moulding, hammering out metals, amateur photography, various kinds of farming, carpentry, mechanical trades as well as games, picture puzzles, and reading aloud and committing poetry to memory. Any- thing which fixes the attention and occupies the mind will distract the thought from the morbid ideas, fears, and impulses. And if this is combined with a frequent change of surrounding, with new associations and helpful manners of life, an out-of-door country life and freedom from care, anxiety and effort, the symptoms may subside, at least for a time. Course. — Psychasthenia as a rule comes on very gradually, thougn occasionally it is seen to appear suddenly after a shock. As a rule the individual is aware of the fact that the mind is not acting in a proper manner, that ideas and impulses and fears little by little are taking possession of it, and that the control of the mental action is not what it should be. Finally various symptoms, such as those already mentioned, develop and take possession of the thought, so that all occu- pations are abandoned, diversion is impossible, and the time is occupied with the mental worries, anxieties or impulses. The fears sometimes reach a point where they are attended by the ordinary physical symp- toms of a normal fear, excessive perspiration, feelings of faintness and rapid heart. The same physical condition of anxiety may also develop in connection with impulses that are not gratified. Thus, if a patient restrain his impulse to touch certain objects or to j)ut on a garment in a certain way and forces himself to do an act contrary to his impulse, he gets into a state of greater anxiety, which is only relieved when he carries out the impulse to its completion. Thus the life is spent in a futile effort to satisfy the impulses, to argue out the ideas or to avoid any course in conduct which would produce a fear. Sometimes a change of scene and travel, sometimes the adoption of a course of treatment involving constant occupation, especially with the hands, as in arts and crafts, sometimes a sudden grief or emotional shock of a new character seem to alleviate the symptoms and result in their subsidence and patients are very often free from symptoms for a considerable time. But they are very prone to relapses and after any renewed emotional strain or physical illness, all the former symptoms COURSE. 877 recur. The underlying cause being a weakened state of the nervous system, it is hardly to be expected that a permanent cure can be obtained. Yet these patients receive great help from suggestive treat- ment or from persuasion and many of them under this treatment manage to lead a reasonably comfortable life. They are very liable, however, to become extremely dependent upon anyone who has succeeded in treating them by hypnosis or suggestion. A very small percentage of these cases finally drift into asylums, it being impossible for their families to put up with their constant eccentricities. For other means of treatment the reader is referred to the Treatment of Neurasthenia, page 854. CHAPTER LIL HYSTEEIA. Hysteria is a functioDal nervous condition, characterized by a permanent mental state, which may be termed the hysterical tempera- ment, and by sudden temporary attacks of mental or emotional or physical kind. 1. The hysterical temperament is manifested by an abnormally keen sensibility to all external impressions and sensations, by a high grade of imaginative power, by a susceptibility to suggestions, by an unusual desire for attention and notice, by variations in mood not due to appar- ent causes, by a lack of judgment, by a manifest incapacity to exercise control over thought, emotion and action, and by a tendency to act on sudden impulses. These characteristics lead to a mental state which is so distinct as to be recognized both by the laity and by physicians. The former consider it as evidence of wilfulness, of foolish fancies, of imagination, or of moral obliquity, and base this notion on the fact that every one has the possibility of showing such manifestations when a relaxation in self-control is permitted. They point to the fact that under intoxication, or in the state of mental exhaustion following pro- longed anxiety or grief, or after sudden shock from fright, the mental characteristics of the hysterical temperament may suddenly appear, and they hold that as this implies a condition of loss of self-control, it is merely necessary to urge the importance of such control and to punish those who will not exert it. The physician and psychologist, on the other band, regard the hysterical temperament as evidence of some de- fective interaction of those mechanisms of the nervous system through which mental and physical acts are harmonized. These mechanisms are located in the cortex of the brain, and are made up of cortical centres and their association tracts. Sensory impulses reaching the cortex normally cause mental perceptions which are recognized, are related to other perceptions or to actions, and awaken an orderly train of thought. In the hysterical person the sensory impulse may cause an abnormally keen perception ; a slight sound, or light, or touch, or pain being felt as almost too intense to be endured (hysterical hyper- esthesia), or it may awaken no perception whatever [hysterical anaes- thesia). Or if perceived, the perception may awaken no recognition (hysterical amentia), the patient manifesting the same symptoms as those who are mind blind, or mind deaf. (See page 122.) Or the perception, though recognized, may fail to awaken a natural train of ideas or lead to natural acts. It may even start an unrelated series of mental responses, which like our dreams bewilder ai7d distress the 878 HYSTERIA. 879 mind by their lack of continuity (hysterical insanity). And these illogical ideas may in turn lead to illogical actions as confused and absurd as the chaotic ideas of which they are the outcome (hysterical morbid impulses'). Thus in an hysterical patient the ordinary external impression does not produce the effect one would expect. It may, however, produce unusual effects. The capacity of imitation, which is normally inherent, is, in these persons, frequently excessive. If they see anyone doing something unusual (twitching, running, posturing, etc.), they have an intense desire, or an irresistible impulse to do the same. If a sufficiently impressive statement is made, accompanied by a command, they accept it without question, and obey. The power of judgment and of critical reason seems to be at times suspended, so that they are almost automatic instruments in the control of a stronger mind. This is termed suggestibility, the dictates of the stronger mind leading to acquiscence without reason. With this temperament there is invariably associated a hypersensi- tive emotional state. Things which should cause emotional effects may cause none. Or things which should cause very little effect may awaken most intense reactions. And the emotional effect may be un- duly prolonged, as well as intense, so that a smile will run into a fit of laughter or- a perfect paroxysm of merriment, or a slightly depressing circumstarice may cause long weeping, or these may alternate. It is in the emotional sphere that the lack of self control is most apparent. It seems as if the mechanisms of the brain which are called into play in emotional acts, once started run their own course and cannot be ar- rested, just as in the mental confusion the thoughts appear to be beyond control. It is true that a second emotional shock may serve to stop the action first started, in some unknown way ; and in fact there ap- pears to be no other means of controlling these emotional storms than by some very intense impression calculated to arouse another emotion. Other evidences of a lack of control are found in the excessive temper, the vanity and desire for notice, in abnormal shyness, or in morbid fears of an intense kind which these patients manifest. It may also be shown in a lack of power to arrest some reflex bodily function ; thus the act of coughing, or of swallowing, once begun cannot be arrested ; or a conscious and voluntary motion once begun has to be kept up, like the flexion or extension of a limb (hysterical tic), or the mainte- nance of some peculiar position (hysterical contractures). Even so-called automatic acts of the body, respiration, the heart beat, the peristalsis of the stomach and intestines, may become conscious and may be affected by the attention, either being hampered or hastened. And finally, the entire motor mechanism of the body may be thrown into action, as in hysterical convulsions. Persons of hysterical temperament are usually very introspective. They watch their symptoms, remember keenly all their variations of feeling, and exaggerate their suffering. They refuse to allow their attention to be directed to matters of interest not connected with their own state, and thus generally narrow their mental horizon, until all 880 HYSTERIA. thoughts are self-centred. This very attention to sensations serves to intensify the sensations, hence their suffering is usually out of all rational proportion to the objective symj)toms. This fact once appre- ciated by friends results in a lack of sympathy on their part, which is resented by the patient ; who in turn seeks to regain that sympathy by further claims to attention, even to the extent of simulating symptoms. Self-inflicted wounds, with consequent sores that will not heal, the vomiting of blood sucked from a tooth and swallowed, the high tem- perature obtained by friction on the thermometer either in the mouth or rectum, emaciation from supposed starvation, and vomiting of all food, are examples of such symptoms. The hysterical temperament is not, however, always manifested by emotional outbursts. Some individuals will not permit of any emo- tional expression. They repress all such tendencies, are unusually reserved and self-contained, yet they suffer from grief, anxiety, or fright, equally with others, and have not the natural relief which comes from emotional expression. In these persons hysterical symptoms often appear suddenly, though their friends declare that they are not hysterical. It is often more difficult to deal with these suppressed hysterical persons than with those subject to emotional outbreaks. The vasomotor system is also in an abnormal state in persons of hysterical temperament, as is shown by the pallor or flushing which attends their mental processes, and by the increased excretion of urine which follows a paroxysm of any kind. The hysterical temperament, while a chronic condition, may not be always in evidence. It is at times in abeyance or under control, and sometimes it is outgrown as life goes on. It is likely, however, to persist, and under conditions of mental or physical strain to reappear, often complicating the symptoms of other diseases. 2. The hysterical temperament rarely remains for any length of time without leading to sudden temporary attacks of a mental, emotional, or physical kind, which are termed hysterical paroxysms. These are usually caused by some emotional strain, but may occur without any ascertainable reason. They vary so greatly in their intensity as to be divided clinically into attacks of hysteria minor and hysteria major. The latter are quite rare in this country. (a) Hysteria Ilinor. — The attacks consist of sudden choking sensa- tions in the throat, as if the pharynx contracted (globus hystericus), of a tendency, which soon becomes imperative, to cry or to laugh, or to do both in succession, or of a violent, unreasonable outburst of temper. There is a feeling of great nervousness, apprehension or fear, a desire for air, a heat or chilliness throughout the body, and a restlessness or tendency to shout. The body is kept in motion as the patient wanders about or tosses in bed. Soon intense pain is felt in the top or back of the head, or in the eyes, or in the spine. Usually pallor or flushing accompany the attack. Sometimes sexual excitement occurs. The mental distress and excitement may lead to a condition like delirium. Sometimes the patient falls into a light slumber, and wakens in a nor- HYSTERIA. 881 mal state. More often the attack subsides gradually under the influ- ence of remedies. Occasionally it ceases suddenly, and the patient is at once quite well. In all cases a large flow of urine coincides with recovery. It is light in color, of very low specific gravity, and often contains an excess of phosphates. A sense of exhaustion and fatigue lasts for some hours or even days after the paroxysm. One attack may follow another for several days, during which the exhaustion increases, and recovery is then very gradual. A state of mental distress and of depression of spirits is sometimes observed, but usually the patient does not appear to regret the attack, though she rather dreads its return. After an attack the sleep is often disturbed for many nights, being rest- less and disturbed by dreams of an unpleasant kind. Occasionally somnambulism occurs. (6) Hysteria Major. — The attack begins with symptoms similar to those of hysteria minor, but in much greater intensity and longer dura- tion. The crying or laughing is violent and loud, or the outburst of temper so extreme as to be maniacal. After this the patient passes into a general convulsive seizure which consists of a series of struggles with those about her, kicking with the feet, seizing objects and push- ing them away with the hands, throwing the head and body about, arching the back till the body rests only on the head and feet in bed, or rolling about on the floor. In all these movements it would seem to the bystander as if there was some conscious motive in every act, and as a rule nothing is done which causes any injury of herself. She may bite others, but does not bite her tongue or hurt herself in her motions. Yet these patients rarely show evidence of consciousness in an attack, and usually deny any memory of its details. They cry and scream and fight. In such an attack the pupils are dilated, the eyes are rolled about or turned far up, the eyelids are often open, yet they do not seem to see. The respiration may cease for a time and the face become cyanotic. The limbs are alternately flexed and extended, but sometimes remain rigid for a time, and then again are set in motion. The tonic spasm is followed by clonic spasms as in epilepsy, and this by a series of contortions and extraordinary postures, the body resting on heels and head, in opisthotonos. The convulsive attack may last an hour or more, or recur in series for several hours, and this fact alone suffices to distinguish it from epilepsy. In a few cases delirium coincides with or follows the attack, the patient talking wildly and appearing to be subject to hallucinations. Usually the attack stops as suddenly as it began, the patient falls asleep and wakes in a normal state. Sometimes the attack subsides slowly. That consciousness is not wholly suspended is proven by the fact that strong external impressions often arrest the attack. Thus painful pressure on a nerve or sensitive organ, or sharp counter-irritation of the skin, or an emetic will arrest an attack. Sometimes a patient who is sub- ject to these attacks finds that a strong effort of the will prevents their occurrence. 3. Attaciks of either minor or major hysteria are sometimes followed 56 882 HYSTERIA. by conditions of loss of power of sensation, or of motion, which appear suddenly and disappear as suddenly. (a) Anaesthesia to pain is the most common symptom, touch and temperature .sensations being often but slightly aifected. This may be limited to one-half of the body, and is always far more complete than in organic hemianalgesia. In hysterical cases no pain is felt. In organic cases, as a rule, some sensation can be aroused. The anaes- thesia may be limited to the peripheral part of one limb or several limbs. In such cases it never resembles the anaesthesia of a peripheral nerve lesion in its distribution, and its upper limit is sharply defined, which is not the case in multiple neuritis. (See Plate IV., page 40.) The anaesthesia occasionally is found in irregular shaped areas, not cor- responding to any anatomical distribution of nerves. Usually it is difficult to draw blood in the anaesthetic area, pin pricks not bleeding as in other parts. Sometimes the analgesia can be removed by static electricity, sometimes by applying magnets or metals to the surface, sometimes by mere suggestion. It may be transferred by these agents to the other side of the body. The mucous membranes of the nose, mouth, and throat are occasionally analgesic. The skin reflexes are usually normal. (6) Anaesthesia of the retina to light and color is also observed. This causes a concentric diminution of the visual field for color and for light, and in some cases an apparent blindness of one eye. The blind- ness rarely takes the form of hemianopsia, and this fact serves to dis- tinguish it from a state due to organic brain disease. Usually one eye is much more affected than the other though total blindness has been observed. The normal color fields may be reversed or altered. Blindness of one eye usually coincides with hemianaesthesia. The pupil reflex is never abolished. (c) Hearing may be affected, and total deafness in one ear usually coincides with hemianaesthesia, though occasionally it occurs alone. Sometimes it is bilateral. High pitched or low pitched tones may be inaudible when ordinary sounds are heard. Bone conduction is affected equally with air conduction — a condition rather rare in non- hysterical deafness. (d) Loss of taste and smell on one side have also been observed, and often accompany hemianaesthesia. (e) Loss of motor power, or hysterical paralysis, is a very common symptom. This may occur either as a hemiplegia, or a monoplegia, or a paraplegia. It comes suddenly and is a total paralysis. The patient does not move the paralyzed part at all, and seems incapable of trying. The degree of the paralysis is much greater than in cen- tral brain disease, where as a rule some slight response to effort, ex- cept at the very outset, is the rule. The face is rarely affected. The muscles are usually relaxed. There is no increase of muscular tone, there is no rigidity, there is no increase in tendon reflex, and no change in electrical excitability. There is no tendency of the arm to assume the flexed posture, as in hemiplegia. It hangs like a flail. The leg is HYSTERIA. 883 Fig. 314. dragged in srttempts to walk, or the knee refuses to give any support to the body. (See Fig. 314.) In organic hemiplegia the knee is stiff in walking. The muscles con- trolling the voice may be affected alone, so that whispers only are possible. This is called aphonia. If the paralysis is paraplegic in kind, it is never attended by paralysis of the sphincters, or by bed sores and cystitis. A true clonus is not obtained, though a slight exaggeration of the knee- jerk is often present. Associated with hysterical paraplegia, but sometimes occurring without it, is a symptom described by the French as astasia-abasia. This is a temporary loss of power to stand or to walk, so that the patient collapses and is unable to rise. The attack may come on unexpectedly, and it seems as if the function of the cerebellum were suddenly suspended. It may be cerebellar hysteria, as other symptoms are clearly of cerebral origin. Sometimes a complete hysteri- cal paralysis is preceded by a series of temporary attacks of weakness in one arm or leg, which alarm the patient and tend to bring on a complete paralysis, much intensified by fear. Sometimes the paralysis appears in the form of a fixed contracture of some muscles, a limb, or a part of a limb, being constantly held in a rigid position. Voluntary motion is thus suspended by the rigidity. The postures assumed by the hand or foot in this condition are always peculiar, and do not resemble those which appear in organic disease. The contracture increases when attempts are made to overcome it. The contractured limb is often anaesthetic. Any muscle of the body may 1)6 thus affected. Sometimes tremor of one hand or one foot suddenly develops, either with or without paralysis. The tremor is usually coarse, the move- ments l>cing of a jerky kind. They are intermittent, and sometimes have a tendency to increase when an effort is made to control them, thus resembling the tremor of multiple sclerosis. A form of spasm which is not uncommon is hysterical singultus. The patient swallows Hysterical paralysis of the leg. (Icon, de la Salpetrifere. ) I have seen an organic hemiplegia 884 HYSTERIA. frequently, and as the saliva fails, air is swallowed till the stomach is distended with gas ; this is then belched up, and a part is expelled into the intestines. Hence the peristalsis produces rumbling noises which alternate with the belching and excite notice and cause distress. Respiratory spasms are sometimes noticed. Deep sighing, repeated Fig. 315. Fig 316. Hysterical contracture of the great toe. (Icon, de la Salpetri&re. ) Hysterica] contracture of both feet. (Icon, de la Salpetriere. ) yawning, or irregular agitated breathing may occur, with or without a true hysterical attack. Hysterical cough is loud, barking, constant and very annoying, and often leads to irritation of the larynx, when it is not started by such irritation. Blepharospasm of hysterical nature is occasionally seen. Any of these symptoms of sensation or motion may come on suddenly after a shock, or may follow an hysterical attack. Hysterical pain is another symptom which is very common. It may be anywhere, but is usually in the head or spine, or in a joint. It is very acute and the patients appear to suffer intensely. It is out of proportion to any apparent affection of the organ involved. Thus in the spine it is far greater than that due to meningitis. In a joint it is far more severe than that of rheumatism. If these pains are carefully studied, they will always be found to be true hallucinations of pain, rather than true pain. They are never affected by analgesic remedies, even by hypodermics of morphine. On the other hand, mental sug- gestion often cures them at once. They are mental pains and are only HYSTERIA. 885 to be reached by mental agents. Pain in the head may be either on top, as if a nail were being driven into the head, or it may be in the back of the head and neck, or it may be a general headache. Some- times it simulates migraine or neuralgia. It is usually attended by hypersesthesia of the scalp. Sometimes hysterical girls complain of steady constant pain in the head and eyes, which is not alleviated by any remedy and differs from all other forms of headache in its uniform character and duration. Pains simulating angina pectoris are not uncommon in hysteria, but are to be distinguished from the true disease by the absence of arterial sclerosis or myocarditis and their frequent recurrence without the intense agony and vascular spasm seen in true angina. Fig. 317. Hysterical spasm of the hand and fingers. Icon, de la Salpetriere, IV., 100. Pains in a joint, especially in the knee or elbow, wrist or ankle, which are hysterical in nature, are not attended by any heat or redness or swelling, but are usually associated with a spasm of the muscles when an attempt is made to move the joint. I have known a hyster- ical pain in the knee to keep a patient in bed for three years and to appear in the other knee when the leg had been amputated as a last resort. Hysterical patients usually are found to have some small circum- scribed areas of the skin which are very sensitive to touch or pressure, and irritation of wiiicli may bring on a true hysterical attack. These lie under the l)reaHt and ov(,'r the ovaries in women, and on the S(!ro- tum in men. They may also be found in the epigastrium or on the 886 HYSTERIA. spine. They sometimes disappear after counter-irritation, or after electrical applications, or on strong suggestion. Catalepsy sometimes develops in hysterical persons. This is a state of plastic rigidity of the limbs. They can be put in any posture and will remain so for a longer time than is possible by an effort of the will. Such a state may ensue on a general hysterical attack, or it may appear after a mental shock, or it may develop suddenly without cause. The patient as a rule seems to be in a semi-conscious state, is able to hear and see but takes no notice, and is unable to move or to speak. Sometimes there is a general anaesthesia in this condition. The facial expression is blank, or the eyes, if opened, seem not to see, and no response is made to questions. The condition may persist for hours or even for days, during which the patient has to be fed and kept clean, as a baby. Hysterical sornnolence is a peculiar symptom occasionally observed. It is a sleepy state in which the patient remains for days, being aroused only with difficulty to be fed, and relapsing into a semi-stupor. Some- times it is difficult to elicit even motions of swallowing, and the sleep resembles coma, respiration being slow and irregular or almost imper- ceptible, and the heart action slow and feeble. This is the state called trance, in which a patient may lie apparently dead for days, and then recover consciousness. The duration is usually only a few hours, but Krauss has described a case lasting thirty-two days. Narcolepsy is a temporary condition of sleep, which comes as sud- denly as an epileptic attack and passes off as suddenly, lasting only a few minutes or at most two or three hours. It cannot be distinguished from sleep, as the patient can be awakened by irritation. It appears commonly in hysterical persons. Gastric symptoms sometimes occur in hysteria. There is often a loss of appetite, a loathing of food, a craving for unusual articles of diet, and possibly an arrest of secretion of the gastric juice, and nausea. Occasionally vomiting of an obstinate kind occurs, all food being rejected, and rapid emaciation follows. Intestinal peristalsis may cease, leading to obstinate constipation, or in other cases is excessive with great evolution of gas, causing tym- panites and borborigmi. Phantom tumors of the abdomen are due to gas in the intestines. They usually simulate pregnancy, but disappear under ether. Suppression of urine for twenty-four or forty-eight hours occasion- ally occurs in hysterical patients, but does not lead to symptoms of uraemia as might be supposed. The menses are usually irregular in hysterical women. They may be excessive and lead to sensations of great prostration, which are really due to fear : or they may be very scanty or even suspended. In hysterical women the fear of pregnancy may stop menstruation for months. It is well known that all these vasomotor and sympathetic disturbances can be produced or cured by hypnotic suggestion, and in hysteria it often seems as if many of the symptoms Avere produced by auto-suggestions of hallucinatory origin. THEOBIES OF THE DISEASE. 887 It is not trije, however, that hypnosis can be more easily produced in hysterical individuals. Theories of the Disease. — We know very little regarding the origin and mechanism of hysteria, and for this reason many theories have been advanced to explain the sumptoms. Babinski ^ defines hysteria as a psychical state rendering the subject capable of autosuggestion, and manifesting itself both by primary and by secondary symptoms. The characteristic of the primary symptoms is that it is possible to reproduce them by suggestion with a rigorous exactness in certain persons and to remove them only by means of persuasion. The characteristic of the secondary symptoms is that they are directly subordinated to the primary symptoms. Suggestion is an act by which one attempts to enforce upon another the acceptance of an idea in itself unreasonable. Persuasion is an act of enforcing the acceptance of an idea which is true. For example, to tell a normal person that his arm is paralyzed and that he cannot move it constitutes a suggestion, for it is contrary to common sense. To declare to a person attacked with a psychical paralysis that his trouble is purely imaginary, that it can disappear instantaneously by an effort of his will and thus to obtain a cure is to act by persuasion. If this defini- tion is accepted it makes hysterical symptoms quite definite and gives them a character which organic symptoms do not have, for these cannot be reproduced by suggestion. Thus facial palsy of the peripheral type where the eye is always open, or radial palsy with paralysis of the supinator lougus in flexion of the arm could never be p; oduced by sug- gestion. On the other hand, hysterical contractures or paralysis or fears can only be removed by persuasion, other means being useless. This definition makes hysteria a purely mental malady under the con- trol of psychic phenomena, suggestion, autosuggestion and persuasion. It has been quite universally accepted and not only explains many of the symptoms, but also the remarkable cures by means which could act only through mental influence. If it is adopted it points the way to the only means of successful treatment, viz., mental persuasion. According to Janet hysteria is a purely mental disease and the physi- cal symptoms that are present are wholly the result of mental processes. The fundamental idea of Janet is that in a hysterical individual certain groups of ideas belonging to the psychic life are dissociated from the total mass of ideas, so that they are no longer brought into the range of consciousness by the ordinary process of association, but when they are brought into the field of attention by some apparently spontaneous internal energy, take possession of the field of attention to the exclusion of everything else and cannot be voluntarily banished any more than they can be voluntarily recalled. He calls attention to the fact that the common factor in all hysterical manifestations of whatever nature is the existence of a state of absent-mindedness or amnesia, and he characterizes the state in which the hysterical patient is as a state of 'Soc. de Neurolof^., Nov. 7, 1901. Revue de Neurologie, 1001. Art. Hysteric. Diitil ; Trait<5 de Medicine, vol. x., p. 668, 1905. 888 htstehia. somnambulism. In this state of somnambul ism the individual is entirely abstracted from his normal surroundings and his mind is centered upon some idea or scene or action to which he gives himself up entirely and be goes through a series of performances, just as a somnambulist does, which seem to the onlooker to be v/ithout reason but which are really the normal following out of the idea which is present to him in his state of somnambulism. In other words, his mind is occupied for the time being by a single idea, thought, or motive, which absorbs his consciousness and dominates his action, but this is in no way correlated with his normal state, and when the normal state is resumed he has no recollection whatever of what has occurred in the abnormal condition. The minor attacks of hysteria Janet would consider as examples of imperfect somnambulism. The phenomena of double consciousness he would consider the most perfect exhibition of an hysterical state. The retraction of the field of personal consciousness is with Janet the essence of the hysterical condition. If we recur to the discussion of the subconscious already given on page 870, and the diagram of Mr. Marshall, we can picture to our- selves the hysterical patient of Janet as having a mass of ideas forcibly thrust into his consciousness like the high rising of a wave above the level of the fluid by some force beneath (see Fig. 312) ; or as having a group of circles (see Fig. 313) forcibly detached from the remaining groups and thrust involuntarily into the range of consciousness and dominating the personality, the connections between the circles under A and B being severed temporarily. This theory certainly explains many of the symptoms of hysteria in an admirable way and has been found satisfactory by many authors. Its practical outcome is found in the method of treatment by hypnosis. In the hypnotic state Janet finds that by questioning he can penetrate to the dissociated group of ideas and by suggestion in that state he can bring these into their normal relation to the mental content, can link the groups together, can reestablish the broken connection and in that way arrest the condition of dissociation which prevents a normal con- trol of thought, idea and impulse. Thus the hypnotic treatment cures the hysterical symptoms. A theory of hysteria proposed by Breuer and Freud is different from the two already described. Freud ^ has called attention to the fact that our ideas, thought, emotions, experiences, and so forth, are natur- ally grouped in our minds into certain complexes, each of which is somewhat independent of the rest and yet influences the others. For example, all the thoughts and feelings connected with one's religious education or with one's political affiliations or with one's family life may be termed the religious complex or the political complex or the family complex. Every one of these complexes, even when not appar- ently within the field of attention, has an influence upon the subject within the field of attention and tends to modify any resulting con- clusion which would follow from the consideration of that subject. One 1 Hysteria and the Psychoneuroses. Trans, by A. Brill, 1909. THEOBIES OF THE DISEASE. 88& may be wholly unaware that the fact of such a complex is determining the thought or the conclusion arrived at, yet the complex nevertheless has its continuous and permanent effect. We express this by saying that a person is " unconsciously biased " in his thought. The mind, then, never acts freely, but is always influenced by these underlying complexes to a greater or less extent. The more fundamental and primi- tive the complex affecting it the more dominating will be the effect of that complex upon the mental activity. According to Freud the most fundamental of all complexes is the sexual complex, which, he believes, originates very early in infancy and maintains its dominating influence through life. He holds that all love, even the love of the child for its parent, is primarily sexual in origin. It is the tendency, however, of general education to repress the discussion of this sexual complex. Many ideas connected with it are unpleasant, mortifying or shameful, and the tendency of training is to combat its expression. For Freud, sexual abuse or sexual trauma exists in everyone at some early age. The constant effort to ignore or to repress these disagreeable and shameful experiences connected with the sexual complex produces a state of mental perturbation and this in turn causes a very morbid stress of mind, attended by unpleasant emotions. The group of ideas is being pushed into the background, is being detached from the conscious life by an effort attended by an emotion, but the emotion is one of anxiety to conceal the sexual com- plex, or of fear lest it be discovered, or of shame at its existence, and hence any thought which suggests the sexual complex tends to awaken the emotions connected with it. And if the repression is successful the complex after a time no longer consciously influences the course of thought. It is not thereby obliterated from the mind but remains in the domain of the subcon- scious. From the subconscious Freud imagines that there is a constant tendency for the complex to emerge and hence the process of repression is kept up all the time by an act of the mind which he calls " censure." Such an act is attended by more or less emotion and hence the indi- vidual may be constantly under an emotional strain. This emotion may then be transferred from the unconscious train of thought to some conscious train of thought, lending an emotional character to some mental act that otherwise would be without emotion. Freud thus explains the existence of morbid fears, that is the existence of a fear connected with some act which normally would go on without fear. If this complex energy with its emotion is diverted into a physical channel, Freud terms the process " conversion " and believes that it is the basis for many hysterical physical symptoms, such as spasms, con- vulsions or paralysis and anaesthesia. This hypothesis of " conversion " seems unreasonable, forced and artificial. He holds that every case of hysteria is the result of some preceding psychic trauma ; that such a trauma may be produced by any experience which produces fear, anxiety or shame. And in his opinion such experiences are always in the sexual sphere. S90 HysteMA. The treatment of hysteria which follows from this theory is neces^ sarily a psychic treatment. The object is to stop the mental processes of censure and repression by recalling to consciousness the experiences which have produced the psychic trauma, for he claims that when these are consciously considered and confessed the confession is attended by an emotional outburst which relieves the tension under which the mind labors. To elicit this confession may require hours of laborious self- examination under cross-examination — a process he terms " catharsis." He affirms that when this is complete the symptoms disappear. There are elements of truth in this hypothesis and there are many errors, hence a criticism must be offered. It is admitted that mental conflicts between natural impulses and moral ideas as acquired by edu- cation go on all the time in every mind. At times these cause very intense emotions and stress of mind, and frequently produce neurotic, psychasthenic and hysterical symptoms by apparently arresting normal inhibition, the susceptibility to suggestion being increased thereby. That all or even the majority of such mental conflicts originate in the sexual sphere experience contradicts. For the most fundamental mental comjDlex or instinct is that of self-preservation, rather than of reproduction. And the modern American is much more concerned with success in business, society, profession or politics, etc., than in his sexual life, though this may not be true in the country where this hypothesis originated. Nor do I believe that sexual ideas exert much influence before the age of puberty, while the idea that the child's love for its parents is based on sexual feeling is contrary to general accep- tation and is an offensive notion ; according to it a child should show aifection for the parent of the opposite sex only — which is not true. Another objection to the hypothesis is the method of treatment. In the search for forgotten shameful sexual experiences it is unavoidable that a set of immoral ideas should be installed or if latent awakened and made a subject of thought and discussion. I kno\y of patients who have been turned into confirmed hypochondriacs by self-examina- tion. Freud himself says that the treatment is applicable only to a small fraction of hysterical patients, those of the higher intelligence, with no trace of weakmindedness, and those with whom the physician is in intimate sympathy. But hysteria is not common among those of strong mind or high intelligence.^ Hence its scope is very limited, as the majority of hysterical patients are not open to it at all. Nor is its success in the few cases a proof of the hypothesis. It is really an adaptation of the Catholic confessional, which is more successful in relieving mental stress and is open to all classes, but the evils of Avhich have been long recognized. It is actually a method of suggestion, like Christian Science and many other processes, in which an intimate rela- tion of a confidential kind is established between the patient and the ^ " I could not conceive myself entering deeply into the psychic mechanism of a hysteria in a person who appears to me common or disagreeable and who would not be able to awaken in me human sympathy. The process is especially inapplicable below a certain niveau of intelligence. It is rendered difficult wherever there is the least tinge of weakmindedness." Hysteria and the Psychoneuroses, p. 84. ETIOLOGT. 891 healer untij the latter is able by personal influence to accomplish almost any result by decided commands or direct persuasion. The treatment then does not differ essentially from other methods of successful suggestive treatment, as it confessedly depends on an intimate sympathy between patient and physician, but is objectionable on account of its character. And furthermore it has to be admitted by Freud himself that a certain proportion of his patients leave him in disgust without benefit, while others have required years of treatment to reach a cure. The criticism is inevitable that the method has a very limited scope and has many objectionable features not shared by the direct persuasion of Babinsky or the hypnosis of Janet.^ Etiology. — The disease is much more common among females than among males. The age of maximum liability is from twelve to thirty, but children are often affected and women may develop hysteria at any age. Next to the time of puberty, the time of the menopause is the period in which the symptoms most commonly appear. The Hebrew race is peculiarly liable to develop the disease. The Latin races are more susceptible than the Teutonic. The disease is rarely seen in its extreme forms in this country, but appears to be increasing in fre- quency in cities under the stress of modern life. Heredity is the most important factor in its causation. A nervous, hysterical mother, or an alcoholic father, are particularly liable to pro- duce hysterical children. Blood relation between parents is often the cause of hysteria in the progeny. Exhausting disease of long stand- ing in the parents is a factor. Charcot held that every case was based on a bad heredity. Mental or emotional shock is the chief exciting cause. Such shocks come to every one, but in those who are predisposed to hysteria they have a surprising effect, often starting a long train of symptoms which may last a lifetime. Thus the fright occasioned by a railway or auto- mobile accident, by a stroke of lightning, by a sudden fall, maybe fol- lowed by hysterical symptoms either at once or after an interval of some days. Another cause is long continued anxiety or care. This acts like a series of little shocks continued with a summation of effect under which, as under a great shock, the weak nervous system gives way. Occupations which involve tense mental strain, the life of an engineer on a locomotive, that of a broker on the stock exchange, that of a responsible leader of politics, or banking, or any profession or business, may lead to nervous exhaustion, which shows itself in hys- terical symptoms. It appears to be the worry and emotional strain rather than the work itself, which induces the collapse. Any exhausting disease long continued, which saps the vital energy, especially diseases of the genital organs which involve much anxiety over the potency of the indivi 'I Lfll^ f^; /'A OCT 9 111 I 6^ ^ -^ C28 (747) MIOO COLUMBIA UNIVERSITY LIBRARIES 0062037498