ft.TLS, 190° chestnut -;t;ieet, philadelphia, pa. Colum&ta Winibtv&itv vDVS fnttieCitpofiSeto|9orfe College of ^fjpgtctans anb gmrgeong Reference library DISEASES OF THE NERVOUS SYSTEM GORDON DISEASES OF THE NERVOUS SYSTEM FOR THE GENERAL PRACTITIONER AND STUDENT BY ALFRED GORDON, A.M., M.D. (Paris) LATE ASSOCIATE IN NERVOUS AND MENTAL DISEASES, JEFFERSON MEDICAL COLLEGE; LATE EXAMINER OF THE INSANE, PHILADELPHIA GENERAL HOSPITAL; NEUROLOGIST TO MOUNT SINAI HOSPITAL, TO NORTH- WESTERN GENERAL HOSPITAL AND TO THE DOUGLASS MEMORIAL HOSPITAL; MEMBER OF THE AMERICAN NEUROLOGICAL ASSOCIATION; FELLOW OF THE COLLEGE OF PHYSICIANS OF PHILADELPHIA; CORRESPONDING MEMBER OF SOCIETE MEDICO-PSYCHOLOGIQUE DE PARIS, FRANCE; MEMBER OF THE AMERICAN INSTITUTE OF CRIMINAL LAW AND CRIMINOLOGY, ETC. SECOND EDITION, REVISED AND ENLARGED WITH ONE HUNDRED AND SIXTY-NINE ILLUSTRATIONS PHILADELPHIA P. BLAKISTON'S SON & CO. 1012 WALNUT STREET 1913 Copyright, 1913, by P. Blakiston's Son & Co. PREFACE TO THE SECOND EDITION In presenting trie second edition of this work I had again in view principally the general practitioner. The new facts which have been developed since the first edition I have endeavored to describe in a concise but at the same time complete manner. Each chapter almost without exception has been enlarged and among a number of additions the fol- lowing important articles may be mentioned: (i) Fracture of the Skull; (2) Concussion of the Brain; (3) Lumbar Puncture; (4) Cerebro-spinal Fluid; (5) Wasserman Reaction; (6) Radiculitis; (7) Psychoanalysis. Treatment has received special attention in accordance with the new data accumulated, such as administration of antimeningo coccus serum, of salvarsan, surgical procedures, etc. Alfred Gordon. 1812 Spruce St., Philadelphia. Digitized by the Internet Archive in 2010 with funding from Open Knowledge Commons http://www.archive.org/details/diseasesofnervouOOgord PREFACE TO THE FIRST EDITION In preparing this book for the medical public I had in view chiefly the general practitioner and the student. To both Neurology lias always appeared to be a difficult and an insurmountable subject and many a student hesitates to take up its study in earnest. The fault lies partly in the text-books and treatises in which neurological subjects are discussed from the standpoint of the expert neurologist. In my daily association with students and general practitioners I have invariably heard this con- tinuous complaint, viz., that they cannot get hold of a book on Neurology which could give them a plain and practical account of diseases of the nervous system. It is the want of such a work that I have endeavored to meet in the modest volume I am offering. The modern physician is not satisfied with a mere enumeration of facts. In reading a description- of any malady he wishes also to know the reason of the disturbed functions and the anatomical substratum of the morbid phenomena. Otherwise speaking he feels that he must know the relation of a certain manifestation to the normal and morbid physiology of an affected tissue or organ. The knowledge of pathology is therefore a sine qua non to every thinking man. This chapter must therefore precede any other in giving an account of a certain disease. As I am aiming almost exclusively to present Nervous Diseases from a practical standpoint, I naturally avoided too technical and debatable points of pathology, but on the contrary endeavored to present the most essential changes necessary for a thorough understanding of various clin- ical manifestations. In discussing the symptomatology I point out, whenever it is possible, the direct relation between certain phenomena and the pathological changes so as to give the reader an intelligent idea of the morbid symptoms. Each form of functional or organic nervous disease is also discussed from the standpoint of differential diagnosis. All possible affections which may simulate a given disease have been taken up seriatim and differences emphasized. The course of the diseases, their mode of termination, their prognosis and the etiology have been given full consideration. In describing the latter, the most well known and well established factors have been pointed out first. The reader may be surprised to find Etiology placed in some chapters before Symptomatology and in others immediately before Treat- vii Vlll PREFACE ment. This was arranged according to the importance Etiology plays in certain diseases or according to the amount of knowledge we possess of the causative factors in various diseases. Considerable space has been devoted to Treatment. Only the most useful and the best known devices, appliances, operations and drugs are described. Medications that are uncertain as to their therapeutic value are omitted or else only mentioned. True to my original aim I have endeavored to present to the reader only the most essential points, whether it was in Pathology, Symptomatol- ogy, Pathogenesis, Etiology or Treatment. The latest views, ideas and thoughts have been presented as far as it was possible. Intentionally I avoided details on disputable questions and omitted them altogether when- ever I could without sacrificing the clearness of the subject. A Chapter on the Method for examination of patients precedes the description of diseases of then ervous system. In it are indicated what phenomena are considered normal or abnormal. I have described here the motor, sensory and trophic phenomena, also the reflexes, the state of sphincters and electrical contractility of muscles or nerves. Finally a chapter on the Normal Anatomy of Brain and Cord, also Malformations of the Nervous System, has been added. Detailed de- scriptions have been omitted whenever it was possible and instead clear illustrations are given. On the whole I feel that I am presenting a practical book to the average physician, but if also the neurologist, the teacher, the advanced student may find in it some ready references which they may peruse in their scien- tific studies, my labors will be more than compensated. A word of thanks is due to the publishers. They have facilitated my task by allowing a large number of illustrations, without which no modern scientific work can be satisfactory. Alfred Gordon. 1430 Pine St., Philadelphia, CONTENTS CHAPTER I Anatomy and Physiology of the Central Nervous System Page Spinal Cord i Meninges or the Cord 9 Blood Supply of the Cord n Rhombencephalon " Medulla oblongata " Pons 15 Fourth ventricle 19 Mesencephalon (Middle Brain) 20 Area of Crura and Corpora quadrigemina 20 Diencephalon (Interbrain) 24 Area of Optic Thalami and Third ventricle 24 Cerebral Hemispheres. Telencephalon 26 Gray substance 27 White substance 3 1 Tracts 32 Cerebellum 4° Meninges of the Brain 43 Blood Supply of the Brain 46 Histological Elements of the Central Nervous System 49 Neurone Doctrine and Secondary Degeneration 5° Malformations of the Central Nervous System 52 CHAPTER II Method of Examination for Diagnosis of Nervous Diseases I. Motor Phenomena 59 II. State of Nutrition of Muscles 62 III. Electrical Contractility 63 IV. Sensory Phenomena 68 V. Special Senses • • 7° VI. Cranial Nerves 7* VII. Speech and Writing 7 1 VIII. Reflexes 71 IX. Sphincters 74 X. Vasomotor and Trophic Disturbances 74 CHAPTER III Cerebral Localizations Motor Centers 75 Speech Centers 78 Sensory Centers 79 ix X CONTENTS Page Special Sensations 80 Intelligence 81 CHAPTER IV Apoplexy Hemorrhage 82 Embolism 86 Thrombosis 86 Hemiplegia 9° Delayed Apoplexy 101 Intermittent Closing of Cerebral Arteries and Transitory Hemiplegia .... 102 CHAPTER V Encephalitis Acute Non-suppuratwe Form 104 Suppurative Form. Abscess of the Brain 106 Chronic Encephalitis II2 Infantile spastic hemiplegia n S Spastic Diplegia. Little's disease 7 i*7 CHAPTER VI Jacksonian or Focal Epilepsy I2 4 Epilepsia Partialis Continua 128 CHAPTER VII Aphasia I 3° CHAPTER VIII Hemianopsia I 4° CHAPTER IX Tumors of the Brain I 43 CHAPTER X Hydrocephalus l & 2 CHAPTER XI Diseases of the Basal Ganglia Optic Thalamus. Corpora Striata. Corpora Quadrigemina 167 CHAPTER XII Meningitis I 7 I CHAPTER XIII Thrombosis of the Intracranial Sinuses J 99 CHAPTER XIV Circulatory Disturbances of the Brain ANiEMiA. Hyperemia 2 ° 2 CONTENTS XI Page CHAPTER XV Fractures of the Skull ' 205 CHAPTER XVI Concussion of the Brain 209 CHAPTER XVII Diseases of the Cerebellum Tumors 213 Abscess 218 Cerebellar Heredo-ataxia 219 Hemorrhage and Softening 221 CHAPTER XVIII Diseases of the Medulla, Pons and Fourth Ventricle A. Acute Superior Polioencephalitis 223 B. Chronic Superior Polioencephalitis 224 C Acute Inferior Polioencephalitis 226 D. Chronic Inferior Polioencephalitis 226 E. PSEUDO-BULBAR PALSY 229 F. Myasthenia Gravis 231 G. Hemorrhage and Softening of the Medulla 234 H. Occlusion of Posterior Inferior Cerebellar Artery 235 I. Compression of the Medulla 235 Diseases of the Pons 236 Hemorrhage. Softening. Tumors 237 Crossed Paralysis 239 Peduncular Syndrome 241 CHAPTER XIX Diseases of the Spinal Cord A. Systemic Diseases of the Cord 242 I. Tabes 242 II. Spastic Paraplegia 256 Family Spastic Paraplegia 257 Paraplegia of the aged 258 III. x\taxic Paraplegia 259 IV. Friedreich's Ataxia 261 V. Acute Anterior Poliomyelitis 264 VI. Chronic Anterior Poliomyelitis 272 VII. Amyotrophic Lateral Sclerosis 272 B Non-systemic Diseases of the Cord 272 I. Myelitis 272 II. Hematomyelia 279 III. Divers' Paralysis 284 IV. Syringomyelia 286 V. Diseases of Conus Medullaris and Cauda Equina 291 VI. Disseminated Sclerosis 295 XU CONTENTS Page Secondary Affections of the Spinal Cord 302 I. Traumatic Lesions of the Cord 302 Concussion. Contusion 302 Sudden Compression. Laceration 303 II. Slow Compression. Tumors. Pott's Disease 306 CHAPTER XX Muscular Atrophies 314 I. Progressive Muscular Atrophy of Spinal Origin 314 Progressive muscular atrophy of infants 318 Amyotrophic Lateral Sclerosis 319 II. Myopathy 321 III. Primary Neurotic Atrophy 325 IV. Arthritic Muscular Atrophy 327 Amyotonia Congenita 327 Myotonia Atrophica 329 Diseases of Spinal Meninges 329 Intermittent Claudication of the Spinal Cord 334 CHAPTER XXI. Syphilis of the Nervous System 336 CHAPTER XXII Paresis 353 CHAPTER XXIII Lumbar Puncture and Cerebro-spinal Fluid. Wassermann Reaction 369 CHAPTER XXIV Diseases of the Peripheral Nervous System Neuritis 377 Neuroma 384 Multiple Neuritis 385 A. Alcoholic Multiple Neuritis 387 B. Lead Multiple Neuritis 389 C. Arsenical Multiple Neuritis 390 D. Diphtheritic Multiple Neuritis 390 E. Carbonic Gas Multiple Neuritis 391 F. Mercurial Multiple Neuritis 392 G. Puerperal Multiple Neuritis 392 H. Beriberi Multiple Neuritis 392 I. Lepra Multiple Neuritis 393 K. Senile Neuritis 394 Acute Ascending Paralysis 396 Periodic Paralysis 39 8 Diseases of Individual Nerves 399 I. Paralysis of Cranial Nerves 399 II. Paralysis of Spinal Nerves 415 A. Upper Cervical Nerves 415 B. Lower Cervical Nerves (Brachial Plexus) 416 C. Lumbo-sacral Nerves 425 CONTENTS Xlll Page Radiculitis 429 Neuralgia in General | 430 Neuralgia of Individual Nerves 435 Neralgia Paraesthetica and Intermittent Claudication 445 Herpes Zoster 447 CHAPTER XXV Functional Nervous Diseases Neurasthenia 451 psychasthenia 456 Hypochondria 458 Anxiety Neurosis 464 Hysteria 464 Epilepsy 482 Chorea 495 Athetosis 503 Dystonia Musculorum Deformans 505 Tic 507 Facial Spasm 514 Myoclonia 516 Tetany 519 Myospasm from Intense Heat 524 Myotonia Congenita (Thomsen's Disease) 525 Occupation Neuroses - 527 Paralysis Agitans 529 Akinesia Algera 533 Headache 535 Migraine 539 Vertigo 544 CHAPTER XXVI Traumatic Neuroses and Psychoses. Medico-legal Considerations 548 CHAPTER XXVII Diseases of the Sympathetic System Trophoneuroses. Angioneuroses Exophthalmic Goiter 560 Myxcedema 567 Acromegaly 572 Gigantism 575 Achondroplasia 576 Adiposis Dolorosa 577 Scleroderma 579 Facial Hemiatrophy 581 Facial Hemihypertrophy 583 Acroparesthesia 583 Angioneurotic (Edema 584 Hereditary (Edema of the Legs 585 Erythromelalgia 585 Raynaud's Disease 587 XIV CONTENTS Page CHAPTER XXVIII Nervous Symptoms Produced by Intoxications A Metallic Poisons 591 I. Lead Intoxication 591 II. Arsenical Intoxication 592 III. Mercurial Intoxication 593 IV. Carbon Monoxide Intoxication 593 V. Manganese Intoxication 594 B. Organic Poisons 595 I. Alcoholism '. 595 II. Morphinism 599 III. Cocainism 601 CHAPTER XXIX Nervous Symptoms Caused by Some Special Infections 602 Tetanus 602 Hydrophobia 605 Pellagra 608 Index 609 Diseases of the Nervous System CHAPTER I ANATOMY AND PHYSIOLOGY OF THE CENTRAL NERVOUS SYSTEM SPINAL CORD The cord covered, by three membranes is placed in the vertebral canal and extends from the upper border of the atlas down to the upper border of the second lumbar vertebra. It occupies therefore only two- thirds of the vertebral canal, viz. its cervical and thoracic portions. It is approximately a cylindrical body presenting two enlargements and a conical termination. Its length is about 45 cm. (18 inches) in the male and 41 cm. (16 inches) in the female. The cord is continuous above with the medulla and below it forms a thread-like termination (filum terminate) which extends to the coccyx to which it is attached (Fig. 1). The spinal cord is divided into the following segments: cervical, thoracic, lumbar and sacral or Conus Medullaris. The Cervical and Lum- bar segments are the thickest parts of the cord, viz. the enlargements mentioned above. The segments correspond to the following vertebrae. The Cervical enlargement, which supplies nerves to the upper extremities also gives origin to the phrenic nerve, extends from the third cervical to the second thoracic vertebra and has its maximum of development at the level of the sixth cervical vertebra. The portion of the cord above the enlarge- ment corresponds to the first two cervical vertebrae. The Thoracic segment extends from the second to the ninth thoracic vertebra. The Lumbar enlargement, which supplies nerves to the lower extremities, commences at the level of the ninth thoracic and terminates at the lower border of the first lumbar vertebra. Its maximum corresponds to the twelfth thoracic vertebra. The Conus Medullaris or sacral segment is the very small conical portion extending from the first to the second lumbar vertebra. Exterior of the Cord.— The cord is divided into two halves by an THE CENTRAL NERVOUS SYSTEM CIA VA FUNICUL US CUNEA TUS POSTERO-MEDIAN SULCUS- POSTERO-LA TERAL SULCUS POSTERO-LA TERAL , SULCUS POSTERO-MEDIA N S ULCUS CERVICAL EN LA R GEMENT FILUM TERMINALE LUMBAR ENLA R GEMENT CONUS MEDULLARIS OLIVARY BODY LATERAL FUNICULUS DECUSSATION OF PYRAMIDS ANTERIOR MEDIAN FISSURE SECTION OF MED ULLA OBLONGATA ||- ANTERO-LATERAL SULCUS {Line of ventral nerve-roots) ANTERIOR MEDIAN FISSURE Fig. i. — Posterior and Anterior Views of the Spinal Cord. {Morris, modified from Quain.) THE CENTRAL NERVOUS SYSTEM anterior and posterior median fissure. The anterior is a deep and broad fissure and contains a duplication of the pia-mater with its important blood vessels. The posterior median fissure is simply a sulcus, a septum. Each half of the cord is divided by two sulci into three portions. They are: the postero-lateral sulcus, which receives the posterior sensory roots, and the antero-lateral sulcus, which is the place of exit of the anterior roots. The portions of the cord between the sulci present in each half longi- tudinal columns, viz. posterior, lateral and anterior. Interior of the Cord. — A transverse section shows that the spinal POSTERIOR FUNICULUS _ • RETICULAR FORMA TION _ LATERAL FUNICULUS ANTERIOR WHITE COMMISSURE ANTERIOR FUNICULUS {After Morris' Anatomy. cord consists of a central gray and a peripheral white substance (Figs. 2, 3, 4, 5, 6 and 7). Gray Substance. — It presents two symmetrical halves united in the middle line by a gray commissure in the center of which is the central canal. The two halves with the intermediate commissure give the im- pression of the letter H. The central canal extends through the entire length of the cord. Each half of H-shaped gray mass has an anterior and posterior por- tion, called "cornua." The anterior cornu is distinguished by its larger, wider and thicker appearance than the posterior. Besides, it is separated from the periphery of the cord by the white substance. Between the an- terior portions of the two anterior cornua lies a bundle of fibers crossing the middle line, which constitutes the anterior white commissure. THE CENTRAL NERVOUS SYSTEM FASCICULUS CUNEATUS I FASCICULUS GRACILIS POSTERIOR SEPTUM - -DORSAL ROOT , DORSAL HORN ANTERIOR MEDIAN FISSURE VENTRAL HORN - " VENTRAL ROOT Fig. 3. — Cervical .VI. (After Morris' Anatomy.) Fig. 4.— Thoracic VIII. (After Morris' Anatomy.) THE CENTRAL NERVOUS SYSTEM Fig. 5. — Lumbar III. (After Morris' Anatomy.) Fig. 6. — Sacral IV. (After Morris' Anatomy.) Fig. 7.— Coccygeal. (After Morris' Anatomy.) Figs. 2 to 7. — Transverse Sections prom Different Segments of the Spinal Cord, showing Shape and Relative Proportions of Gray and White Substance in the Dif- ferent Segments Represented. (Morris' Anatomy.) 6 THE CENTRAL NERVOUS SYSTEM The posterior cornu is, thin, long and extends to the periphery of the cord. It presents a head, neck and base. The head is surrounded by the Gelatinous substance of Rolando. The latter is particularly.marked in the cervical region. In the upper thoracic segment of the cord there is a lateral prominence of gray matter situated between the bases of the anterior and posterior cornua. This is the so-called lateral cornu. It is not found in the cervi- cal or lumbar region. The anterior and posterior cornua retain their relative size through the entire cord, but the amount of gray matter in them varies according to the level of the cord. Thus in the lumbar and cervical enlargements they reach the maximum. In the midthoracic region they are at the minimum. The gray matter is composed essentially of cells. The latter are arranged in groups in each cornu. In the anterior cornu there are mainly: an external group which is the principal origin of the anterior roots and an internal group which sends out fibers for formation of the white com- missure (see above). A special group of cells (vesicular column of Clarke) is situated in the internal portion of the base of the posterior cornu. It extends from the eighth cervical to the third lumbar segment of the cord. The majority of these cells are the origin of the direct cerebellar tract of the same side (see below). The gelatinous substance of Rolando is rich in cells. White Substance. — The fibers composing it originate from the cells of the gray matter of the cord and from the cells of the brain and cere- bellum. They form systems of fibers or columns with different physio- logical functions (Fig. 8). I. Posterior Columns. — Uniform in the lower half of the cord, they are divided into two distinct columns at the level of the upper thoracic and cervical segments. The median bundle is the column of Goll and the outer bundle is the column of Burdach. They are composed essentially of fibers of ramification of the posterior roots. The fibers of the posterior roots emanate mostly from the cells of the spinal ganglia. The axone leaving its cell undergoes a division in the shape of letter T. One branch goes to the periphery and the other enters the posterior root. The fibers of the latter ascend and subdivide in as- cending and descending branches. The descending ones are mostly short fibers, give off collaterals and enter the cells of the gray matter. The ascending branches are long and short. The first extend to the medulla where they terminate in the nuclei of Goll and Burdach. The latter end in the cells of the gray matter. THE CENTRAL NERVOUS SYSTEM 7 The root fibers, upon their entrance into the cord, are divided into two groups: an external, which constitutes Lissauer's tract or marginal zone of Lissauer (in formation of which participate also axones from the posterior horns) and an internal which constitutes the posterior columns properly. Both branches of each root-fiber give off collaterals. They are short and long. The short ones end around the cells of the posterior cornua VIII Fig. 8. — Schematic Representation of the Situation or the Various Tracts of Fibers in the Spinal Cord. {Gordinier.) I. Direct pyramidal tract. II. Descending tract of Marchi and Lowenthal. III. Olivary or triangular tract. IV. Antero-lateral ground bundles of fibers. V. Antero-lateral ascending tract of Gowers. VI. Lateral limiting layer. VII. Direct cerebellar tract. VIII. Crossed pyramidal tract. IX. Lissauer's tract. X. Middle root zone. XI. Posterior root zone. XII. Postero-internal or column of Goll. XIII. Sep to-marginal tract. XIV. Comma tract of Schultze. XV. Anterior root zone. XVI. Cornu-commissural tract. and of the cells of Clarke; they also cross the gray commissure and end in the cells of the posterior cornua of the opposite side. The long collaterals end around the cells of the anterior cornua. They therefore carry to the motor cells of the anterior cornua peripheral sensory impulses, received from the periphery through the spinal ganglia. They therefore form a part of the arc reflex. In the posterior columns there are also fibers originating in the cord itself (endogenous). In the very anterior portion behind the gray com- missure there is the Cornu-cornmissural tract. Its fibers originate in the cells of the posterior cornu. They have an ascending direction. 8 THE CENTRAL NERVOUS SYSTEM In the cervical region there is a small bundle situated in the middle of the posterior columns and called comma of Shultze. A small bundle situated in the thoracic region at the periphery (Hoche's bundle), in the lumbar region on each side of the median septum and in the middle (Flech- sig's oval bundle) and in the sacral region on the posterior and internal portion (Gombault's and Philippe's triangle) are probably the same system of fibers. All these separate fasciculi have a descending course. The posterior columns proper are composed of sensory neurones conducting sensations from the periphery to the cerebral cortex and have therefore an ascending course. II. Pyramidal Bundle. Motor Pathway. — Originating in the motor area of the brain it descends toward the base through the internal cap- sule lower down through the pons and in the medulla forms the pyramids. In the lower part of the medulla the largest majority of the pyramidal fibers decussate with those of the opposite side. The non-decussating fibers (Tiirck's bundle) descend in the anterior columns of the cord occupying the portion near the median fissure. It is called "direct pyramidal tract." It extends down to the middle thor- acic segment of the cord. The decussating fibers (crossed pyramidal tract) after passing from the medulla through the anterior cornua on the opposite side of the cervi- cal cord (first and second segments) are placed very posteriorly in the lat- eral portion of the cord through its entire length. It is separated from the periphery of the cord by the direct cerebellar tract, except in the lum- bar region where the latter does not exist. The pyramidal fibers with their collaterals terminate around the cells of the anterior cornua. They are motor and centrifugal (descending); they transmit to the motor cells of the anterior cornua voluntary impulses from the cortical motor centers. III. Direct Cerebellar Tract (Flechsig). — The fibers originate in the cells of Clarke's columns and commence at the level of the first lumbar segment. They occupy the periphery of the postero-lateral portion of the cord. They are sensory and centripetal, have an ascending course and at the level of the medulla enter the restiform bodies (inferior cerebellar pe- duncle) to terminate in the cerebellum. They carry to the cortical cells of the latter impressions received by Clarke's cells from the posterior roots. IV. Gowers' Tract (Antero-lateral fasciculus). — The majority of its fibers originate from cells of the anterior cornua of the opposite side, al- though the exact source is not known. The bundle is situated antero- THE CENTRAL NERVOUS SYSTEM 9 laterally, in front of the crossed pyramidal and in front of and internally to the direct cerebellar tract. It commences at the level of the dorso- lumbar region. Its fibers are sensory and have an ascending course. Some of its fibers go to the cerebellum through the superior cerebellar peduncle and terminate in the cortex of the superior vermis. Others enter the restiform body. A few reach the anterior corpora quadrigemina. V. Antero-lateral Ground Bundle surrounds immediately the antero- lateral portion of the gray matter. It reaches the periphery only in front of the anterior cornu. Its fibers originate partly in the cells of the gray matter and serve for associating various levels of the cord. It contains also fibers descending from the cerebellum, red nucleus, Deiter's nucleus, corpora quadrigemina and optic thalamus. Loewenthal's bundle or anterior marginal fasciculus is a narrow band occupying the border of the cord between the anterior end of Gower's tract and the anterior median fissure. It originates in the roof nucleus of the cerebellum (nucleus fastigii) and terminates about the cells of the anterior horns. Its fibers are therefore descending. Roots of the Spinal Nerves. — The axones emanating from the cells of the anterior cornua, reach the periphery of the cord and form the anterior roots. After piercing the membranes of the cord they advance to the intervertebral foramina and beyond the spinal ganglia situated in those foramina they join the posterior roots to form a spinal nerve. The function of the anterior roots is motor (and trophic). The posterior roots are formed of sensory fasciculi coming from the periphery, penetrate the intervertebral foramina where they meet the spinal ganglia. From there, covered by the three membranes of the cord, they reach the postero-lateral sulcus and enter the cord. The spinal nerves formed of the junction of the anterior and posterior roots are 31 pairs in number. The roots of the first cervical nerve are horizontal. Beginning with the thoracic nerve the direction of the roots is very oblique, so that the last roots extend through a distance of several vertebras. MENINGES OF THE SPINAL CORD The cord is surrounded by three membranes: dura -mater, arachnoid and pia-mater. (Fig. 9.) Dura consists of one layer. It is a resistant membrane and contrary to the dura of the brain, does not constitute the internal periosteum of the spinal canal. It is separated from the bony walls of the spinal canal by a loose areolar tissue with a plexus of veins. IO THE CENTRAL NERVOUS SYSTEM It commences at the foramen magnum and terminates at the level of the third piece of the sacrum, while the spinal cord ends only at the level of the second lumbar vertebra. Below the cord the cavity of the dural sac is occupied by a bundle of nerves — cauda equina — in the midst of which is seen the filum terminale. Below the third sacral vertebra the dura becomes only a filament extending to the coccyx (coccygeal ligament). It is attached to the canal by ligaments and prolongations which together with the pia and arachnoid accompany and surround the spinal roots on their way to the intervertebral foramina. Ijigamentum dentieulat um INTERVERTEBRAL FORAMEN BODY OF VERTEBRA Periosteum Dura mater SUBDURAL CAVITY Arachnoid SUBARACHNOID CA VITY Pia mater Fig. 9. — Diagram showing Relations of Meninges to Spinal Nerve-roots. (Morris' Anatomy.) The inner surface of the dura is attached to the pia-mater by dentate ligaments through the arachnoid. Between the dura and the arachnoid there is the so-called subdural cavity which contains cerebro-spinal fluid. Arachnoid. — It covers the cord and the cauda equina and is continu- ous with the arachnoid of the brain. Between it and the pia there is the so-called sub-arachnoid space containing a reticulum the meshes of which are occupied by the cerebro-spinal fluid. The above mentioned dentate ligaments and the septum posticum keep it attached to the pia and dura. It gives off prolongations to the spinal roots. Pia. — It is the most internal of the three meninges. It is an extremely vascular membrane closely adherent to the cord. It is thicker in the spinal cord than in the brain. It is composed of two layers. It forms a THE CENTRAL NERVOUS SYSTEM II fold in the anterior fissure of the cord. It sends off prolongations to the spinal roots. At the level of the filum terminale it behaves like the dura (see above). Ligamentum dentate mentioned above is a fold of the pia and presents processes which are attached on the inner surface of the dura between the roots of the spinal nerves. BLOOD SUPPLY OF THE SPINAL CORD The main arteries of the spinal cord are three in number, viz. one anterior spinal artery along the anterior spinal fissure, two posterior spinal arteries, one on each side behind the line of entrance of the pos- terior nerve roots. These arteries give off branches which enter the cord to be distributed more in the gray than in the white matter. The spinal arteries originate from the vertebral arteries which pass through the inter-vertebral foramina together with the spinal roots. The veins are situated along the anterior and posterior fissures of the cord and laterally one on each side along the roots. They then pass through the foramina and open into the vertebral veins. Lymph spaces are found around the nerve cells and blood vessels. MEDULLA OBLONGATA Pons and Fourth Ventricle (Rhombencephalon) The medulla or bulb is the upward continuation of the spinal cord. It occupies the basilar groove of the occipital bone with its lower ex- tremity in the foramen magnum. Its upper extremity marks the be- ginning of the pons. Its length is 25 mm. (one inch), thickness 14 mm. (one-half inch) and width at the lower end one-half inch, at the upper end three-fourths of an inch. Its direction is almost vertical and only slightly inclined forward. Exterior of the Medulla. — It has an anterior, a posterior and two lateral surfaces, also an upper and lower extremities. The anterior median fissure of the spinal cord is continued on the anterior surface of the medulla, but obliterated in its lower portion on account of decussation of the pyra- mids. In the upper portion of the anterior surface are seen the Pyra- mids. They are two large bodies' thicker at the upper ends than at the lower, bounded laterally by a sulcus from which emerge the twelfth nerves (hypoglossi) . The sulcus corresponds to a similar antero-lateral sulcus 12 THE CENTRAL NERVOUS SYSTEM of the spinal cord from which emerge the anterior roots. The sulcus separates the pyramids from oval bodies called Olives. Laterally to each olive lies the Restiform body and in the sulcus between them emerge the roots of the ninth (glosso-pharyngeal) , tenth (vagus) and eleventh (spinal accessory) nerves. At the upper border of the pyramids INSULA ANTERIOR PERFORA TED - SUBSTANCE CORPORA MAMMIL- LARIA OLFACTORY TRACT HYPOPHYSIS L fi —N OPTICUS (II) , OPTIC TRACT CEREBRAL _, PEDUNCLE GANGLION SEMI- LUNARS (gasseri) OBLIQUE FASCICULUS TUBER CINEREUM N. OCULOMO- .-- TORTUS (III) LATERAL GENICULATE BODY N. TROCHLEARIS (IV) N. TRIGEMINUS (M. P.) N. TRIGEMINUS (V) N. ABDUCENS (VI) BRACHIUM PONTIS N. FACIALIS (VII) PARS INTERMEDIA N. ACUSTICUS ( VIII) \ N. GLOSSO-PHARYN- \ GEUS (IX ) N. HYPOGLOSSUS Fig. N. VAGUS (X) s. N. ACCESSORIUS XI > (spinal accessory) CER VICAL I CERVICAL II DECUSSATION OF PYRAMIDS ' 10. — Ventral Aspect of Brain-stem Including Mammillary and Optic Portions or the Hypothalamus. (Morris' Anatomy.) and olives, viz. at the lower border of the pons emerge the roots of sixth (abducens) and seventh (facial) nerves. On the Posterior surface of the medulla are seen the following elements. The lower portion is the closed part of the medulla, the upper portion, in which the two halves become separated, forms an open triangle as a part of the fourth ventricle. THE CENTRAL NERVOUS SYSTEM 13 There is a median sulcus, continuation of the posterior sulcus of the cord. The columns of Goll end in an elevation (clava) which contains a nucleus, STRIA 3IEDULLARIS\ THALAM y HABENULAR COM- MISSURE TRIGONUNA s HABENULJE V. EPIPHYSIS ,. MEDIAL GEN- ICULATE-*^ BODY - LATERAL GEN- 1 ICULATE BODY BRACHIUM QUAD R I- „ GEMINUM INFERIOR CEREBRAL PEDUNCLE A NTERIOR MED ULLARY VELUM BRACHIUM CONJUNC- TIVUM BRACHIUM PONTIS RESTIF0R3I BOD Y CALAMUS SCRIPTORIUS FUNICULUS GRACILIS .. FUNICULUS CUNEATUS FUNICULUS LATERALIS ^- INTERNAL CAPSULE CA UDA TE NUCLEUS SS\ \ TMNIA CHORI- OIDEA ^La. . — STRIA TERMIN- ALS THALAMI \ PULVINAR CORPORA QUADRIGEMINA -N. TROCHLEARIS LINGULA CERE- BELLI STRIM MEDUL- LARES ACUSTICI TRIGONUM VAGI (ALA CINEREA ) —NUCLEUS FUNICULI CUNEATI OBEX ^ NUCLEUS FUNICULI GRACILIS (CLAVA) POSTERIOR MEDIAN FISSURE POSTERIOR INTERMEDIA TE SULCUS Fig. 11. — Dorsal Aspect of Medulla Oblongata and Mesencephalon, showing the Floor of the Fourth Ventricle (Rhomboid Fossa). {Morris, Modified from Spaltehoh.) called nucleus of Goll (nucleus gracilis). Laterally and anteriorly there is another elevation containing the nucleus in which the fibers of Bur- 14 THE CENTRAL NERVOUS SYSTEM dach's columns terminate; it is called nucleus of Burdach (nucleus cu- neatus). Laterally are located the Restiform bodies, which contain ascending and descending fibers connecting the spinal cord with the cerebellum; they are the inferior cerebellar peduncles (Figs. 10, n). Pons Varolii. — It is a large mass of white substance situated between the medulla below and cerebral peduncles (crura) above. TELA CHORIOIDEA OF FOURTH VENTRICLE SOLITARY TRACT NUCLEUS N. VESTIBULI (VII) RESTIFORM BODY SPINAL TRACT OF TRI- GEMINUS NUCLEUS N. COCHLEARIS (VIII) N. VAGUS N. HYPOGLOSSUS PYRAMID — SPINAL TRACT OF TRIGEMINUS ■ - DECUSSATION OF PYRAMIDS Fig. 12. LATERAL CEREBROSPINAL FASCICULUS (crossed pyramidal tract) .„ VENTRAL CEREBROSPINAL FASCICULUS (direct pyramidal tract) -Diagram showing the Decussation of the Pyramids. (Morris' Anatomy.) The uppermost level represented is near the inferior border of the pons. Its anterior surface, containing the basilar artery, is situated on the basilar process of the occipital bone. It is continuous laterally with the middle cerebellar peduncles. The roots of the fifth nerve (trigominus) emerge on both sides of this surface. The posterior surface is continuous with the posterior surface of the medulla and forms with the latter the THE CENTRAL NERVOUS SYSTEM 1 5 floor of the fourth ventricle, covered by the cerebellum. The upper border separates the pons from the cerebral peduncles. It corresponds to sella turcica of the sphenoid bone. From it emerge the third (oculo- motor) nerve and the fourth (pathetic) nerve. The pons consist of super- ficial and deep transverse fibers between which pass the fibers of the cerebral peduncles from above to constitute the pyramids below. Fourth Ventricle. — It is a rhomboidal cavity situated between the medulla, pons and cerebellum. It is continuous below with the central canal of the spinal cord and above with the aqueduct of Sylvius. The Floor. — It is lined with the epithelium which is continuous with the ependyma of the central canal. Its superior half belongs to the pons, the inferior half to the medulla. It contains eminences and depressions. On each side of the median sulcus lies a longitudinal band (funiculus teres) which commences at the lower end (calamus scriptorius) as a grayish mass (ala cinerea) . Striae Acusticae, bundles of fibers arising in the nuclei of termination of the cochlear division of the eighth nerve, cross the floor of the fourth ventricle and divide it into two halves : upper and lower. Upper Half. — It contains the upper portion of the funiculus teres (see above), on each side of which lies the acustic tubercle (one of the origins of the eighth nerve). Between them there is a depression (fovea anterior, or fovea trigemini) which overlies the larger portion of the nu- cleus of the fifth nerve. Above this fovea lies a grayish mass (locus cceruleus), which is also a portion of the nucleus of the fifth nerve. Lower Half. — It contains below at the origin of the funiculus teres ala cinerea (see above), which corresponds to the nuclei of the ninth and tenth nerves. Mesial and above the ala cinerea is an eminence (trigo- num hypoglossi) which corresponds to the origin of the twelfth nerve. Externally to the latter there is another eminence (trigonum acusticum) corresponding to the eighth nerve. Roof of the Fourth Ventricle. — The anterior portion is formed by the cerebellum and superior cerebellar peduncles. It is covered by a lamina of white matter, the anterior medullary velum, the inferior portion of which is continuous with the white substance of the cerebellum. The middle portion is covered by the posterior medullary velum, which is also continuous with the white substance of the cerebellum. The inferior portion is covered by an epithelial membrane (tela chorio- idea), a double fold of the pia containing vascular processes, viz. choroid plexuses. The thickened lateral portions of the tela are called ligulae and the thickened portion of it at the calamus is called obex. The Margins of the fourth ventricle. i6 THE CENTRAL NERVOUS SYSTEM The superior borders are formed by the superior and middle cerebellar peduncles, the inferior by the restiform bodies and the terminations of the posterior columns of the cord. Interior of the Medulla and Pons. — Sections beginning from the lower level of the medulla upward show the gradual formation of the Fig. 13. — Section of Medulla Oblongata Near the Pons. (Santee.) a. Hypoglossal nucleus, b. Vestibular nucleus, c. Tractus solitarius. d. Restiform body. e. Substantia reticularis. /. Hilus of olivary nucleus containing cerebello-olivary fibers, g. Anterior lateral sulcus, h. Pyramid, i. Anterior median fissure, j. Anterior longitudinal bundle, k. Medial longitudinal bundle I. Nuc. tractus spinalis n. trigemini. m. Tractus spinalis n. trigemini. n. Lateral cochlear nucleus. 0. Ventral cochlear nucleus, p. Ascend- ing anterior cerebello-spinal, spino-thalamic and rubro-spinal tracts, q. Posterior lateral sulcus, r. Medial fillet, interolivary stratum, s. Anterior external arcuate fibers, t. Arcuate nucleus. Pyramids. They are formed, properly speaking, of the pyramidal fibers descending from the cerebral peduncles through the deep and superficial fibers of the pons. At the lower level of the medulla they begin to decussate to go down in the cord (see Spinal Cord, Fig. 12). Immediately behind the pyramids lies the median fillet or lemniscus. This bundle of fibers originates in the nuclei of Goll and Burdach (see above). As we have seen above, the sensory neurones of Goll's and Burdach's THE CENTRAL NERVOUS SYSTEM 17 columns of the spinal cord terminate in the lowest parts of the medulla in two nuclei (gracilis or Goll and cuneatus or Burdach). The function, however, of these neurones is continued: new fibers originate in the cells of these nuclei and ascend. They begin to decussate in the median line (raphe) with their fellows of the opposite side, immediately above the decussation of the pyramids and form a large sensory bundle, called median fillet (lemniscus) . At the level of the pons the median fillet spreads laterally on both sides; the lateral portions are called lateral GENU OF FACIAL NERVE NUCLEUS OF ABDUCENS ^ITilBUL^NERvI RETICULAR FORMATION DESCENDING ROOT OF FACIAL NERVE NUCLEUS OF FACIAL NERVE SPINAL TRACT OF TRIGEM- INUS ROOT OF VES- TIBULAR NERVE SUPERIOR OLIVE ROOT OF ABDUCENS CONTINUATION OF PYRAMIDS Fig. 14. — Diagram of Transverse Section of Inferior Part of Pons. {Morris, after Schwalbe.) The restiform body, not included, occupies the curved space lateral to the nucleus of vestibular nerve. lemnisci. Posteriorly lies a third bundle, called "posterior longitud- inal fasciculus," the function of which is to associate the nuclei of the cranial nerves (Figs. 13, 14). In addition to these three fasciculi there are a number of fibers running various courses. Such are the internal arcuate fibers, some of which connect one restiform body (inferior cerebellar peduncle) with the olivary body and the restiform body of the opposite side; others are destined for both cerebellum and cerebrum. The Olives are isolated masses of gray substance containing a dense mass of fibers. Between them and the pyramids pass the twelfth cranial nerves. There are also accessory olives situated about the main olivary bodies. The gray matter of the medulla and fourth ventricle contains chiefly nuclei of the last ten cranial nerves. The nuclei of the third THE CENTRAL NERVOUS SYSTEM ■.;•;; I nucleus of olfa ctor y ner ve NUCLEUS OF OCULOMOTOR NERVE NUCLEUS OF TROCHLEAR, NERVE NUCLEUS OF MESENCEPHALIC ROOT OF TRIGEMINUS PULVINAR OF \ THALAMUS x LATERAL GENIC- ULATE BODY NUCLEUS OF SUPE- RIOR COLLICULUS CHIEF MOTOR NUCLEUS OF-- TRIGEMINUS NUCLEUS OF FACIAL' NUCLEUS OF ABDUCENS *■ " ' NUCLEUS AMBIG UUS (VAGUS AND GLOSSO-PHARYNGEUS) " NUCLEUS OF HYPOGLOSSUS - NUCLEI OF OPTIC NERVE SENSORY NUCLEUS OF TRIGEMINUS NUCLEUS OF VESTIBU- LAR NERVE VENTRAL NUCLEUS OF COCHLEAR NER VE - DORSAL NUCLEUS OF COCHLEAR NERVE ~~ NUCLEUS ALJE CINEREjE {VAGUS AND GLOSSO-PHARYNGEUS) - SOLITARY TRACT (VAGUS AND GLOSSO-PHAR YNGEUS ) NUCLEUS OF SPINAL TRACT OF TRIGEMINUS NUCLEUS OF SPINAL ACCESSORY NER VE Fig. 15. — Scheme showing the Relative Size and Position of the Nuclei of Origin (Red) of the Motor and the Nuclei of Termination (Blue) of the Sensory Cranial Nerves. (Morris' Anatomy.) THE CENTRAL NERVOUS SYSTEM 19 and fourth nerves are situated in the uppermost portion, while the twelfth, tenth and eleventh in the lowest portions. The accompanying illustration shows sufficiently their anatomical seats without entering into a detailed description (Fig. 15). Sections of the medulla also show the seat of the three pairs of cerebellar peduncles and their relation to the restiform bodies, which are the continuation of the postero-lateral tracts of the cord and to the pons, which can be considered as a continuation of the middle cerebellar peduncles. LA TERAL NUCLEI'S OF VESTIBULAR NERVE L. - - RESTIFORM BOD Y MEDIAL BE- -WI— - SCENDING CEREBRO- PONTILE PATH •. MEDIAL LEMNISCUS CEREBRO- {CHIEF- ~LY FRONTAL) PONTILE RATH LONGITUDINAL (PYRAMIDAL) FASCICULI Fig. 16. — Diagram showing Connections of the Fibers of the Pons. (Morris' Anatomy.) The plane of the section is obliquely transverse or parallel with the direction of the brachia pontis. Sections of the Pons show the passage between its deep and super- ficial fibers of the longitudinal pyramidal fasciculi, which are the con- tinuation of the cerebral peduncles (crura) and which at the lower border of the pons emerge as pyramids proper. These fibers are situated in the ventral portion of the pons. In its dorsal portion (tegmentum) the struc- tures are continuous with those of the medulla below (Fig. 16). The pons also contains separate aggregations of gray matter, called Nuclei pontis. They are dispersed between the pontine and pyramidal 20 THE CENTRAL NERVOUS SYSTEM fibers. They receive a large portion of these fibers. They therefore form the following connections. (a) Fibers of cerebellar hemispheres end in the nuclei pontis of the opposite side. (b) Fibers of cerebral hemispheres end in the nuclei pontis, from which new fibers emerge and go to the opposite cerebellar hemispheres. AREA OF CRURA AND CORPORA QUADRIGEMINA (MESENCEPHALON) (MIDDLE BRAIN) It connects the medulla and the pons below with the forebrain above. Exterior. — It presents a ventral and dorsal surface. Ventral. — It is formed by the cerebral peduncles which at the upper border of the pons present two thick bundles. Ascending the latter diverge and produce the interpeduncular space, the floor of which is the posterior perforated space. The latter serves for the passage of blood vessels. The Crura spread in their upward direction and penetrate the brain under the optic tracts. Between their inner surfaces emerge the third (oculomotor) nerves. Their external surface, which is covered by the Temporal lobes, is surrounded by the fourth (pathetic) nerves. Dorsal Surface. — The roof of the middle brain is constituted by a lamina surmounted by quadrigeminal bodies. The latter present an anterior pair and a posterior pair. Externally each anterior body is con- nected by means of a white bundle (anterior brachium) with a ganglionic swelling called external geniculate body ; a posterior brachium connects each posterior quadrigeminal body with an internal geniculate body (Fig. 17). The anterior quadrigeminal bodies with their brachia and geniculate bodies belong to the optic apparatus, while the posterior bodies with their attachments form a part of the auditory apparatus. Posteriorly to the quadrigeminal lamina are seen the following elements : (1) a thick-white tract, viz. frenulum of the anterior medullary velum, situated between the anterior quadrigeminal bodies; (2) from each side of this tract emerge the fibers of the fourth (pathetic) nerve; (3) the ter- mination of the superior cerebellar peduncles which disappear under the posterior quadrigeminal bodies. Between the quadrigeminal bodies dorsally and the crura ventrally lies a funnel-shaped cavity, which connects the fourth ventricle below and the third ventricle in front, viz. aqueduct of Sylvius (Fig. 18). Interior of the Mid-brain. — A transverse section shows a ventral THE CENTRAL NERVOUS SYSTEM 21 and dorsal portion. In the ventral are seen the longitudinal pyramidal fasciculi covered by a pigmented stratum of gray matter (locus niger). The dorsal portion (tegmentum) consists of red nuclei, in which apparently terminate the superior cerebellar peduncles, the gray matter surrounding the aqueduct of Sylvius, the lamina supporting the quadrigeminal bodies and the continuation of other formations of the medulla. STRATUM ZONALB OF THALAMUS \ PULVINAB OF THALAMUS < STBA TUM ZONALE NUCLEUS OF SUPERIOR COLLICULUS MEDIAL GENICU- LATE BODY S. OPTIC TRACT LATERAL GENIC- — - ULATE BODY RED NUCLEUS EPIPHYSIS (PINEAL BODY) s CENTBAL GBEY STBATUM i v -— AQU^EDUCTUS CEREBBI - _ NUCLEUS OF MESENCEPHA LIC (DESCENDING) BOOT OF TBI- GEMINUS v NUCLEUS OF OCULO- MOTOR NERVE MEDIAL LONGITU- DINAL FASCICULUS INTEBPED UNCU- LAR FOSSA CEREBRAL PEDUNCLE SUBSTANTIA NIGRA FILA OF OCULOMOTOR NERVE Fig. 17. — Transverse Section through Level oe Superior Quadrigeminal Bodies. (Morris' Anatomy.) The median and lateral lemniscus of the medulla are here fully devel- oped. The largest part of the latter is connected with the nucleus of the cochlear nerve and terminate in the posterior longitudinal bodies; they have therefore an auditory function. The median lemniscus (see Medulla) in its upward passage, after having received sensory fibers from the nuclei of the cranial nerves, in reaching the mid-brain sends some fibers to the anterior quadrigeminal bodies and terminates in the Thala- 22 THE CENTRAL NERVOUS SYSTEM mus opticus and Hypothalamic nucleus, or body of Luys, which is situated immediately below the lateral and anterior nuclei of the Thalamus. From the latter fibers emerge and go through the internal capsule to the sensory area of the cortex. The posterior longitudinal bundles of the medulla are found here in CENTRAL GREY STRATUM STRA TUM ZONALE \ NUCLEUS OF INFERIOR COLLICULUS AQUMDUCTUS CEREBRI ->.. NUCLEUS OF MESENCEPHA- LIC {DESCEND- ING) ROOT OF TRIGEMINUS NUCLEUS OF TROCHLEAR--- NERVE MEDIAL LONGI- TUDINAL ' FASCICULUS BRACHIUM CONJUNC- TIVUM DECUSSATION * OF BRACHII CONJUNCTIVI POSTERIOR -i RECESS OF INTERPEDUN- CULAR FOSSA NUCLEUS OF LA TERAL LEMNISCUS LEMNISCUS {A CO US TIC) TERAL SULCUS OF MESENCEPHALON MEDIAL LEMNISCUS SUBSTANTIA NIGRA BASIS OF CEREBRAL PEDUNCLE SUPERFICIAL FIBRES OF PONS Fig. 18— Transverse Section through the Inferior Quadrigeminate Bodies. (Morris' Anatomy.) the most intimate connection with various nuclei of nerves supplying the eye muscles and other cranial nerves. The gray matter is the continuation of the same matter of the cord and medulla. Three cranial nerves are in connection with the mid-brain, viz. third, fourth and fifth nerves. The nuclei of the third and fourth form a continuous column of nerve-cells situated in the gray matter surrounding the aqueduct of Sylvius. A section through the posterior THE CENTRAL NERVOUS SYSTEM 23 quadrigeminal bodies shows the origin of the fourth nerves and through the anterior bodies the origin of the third nerves (Fig. 19). The nucleus of the third nerve is connected with the optic tract by means of neurones originating in the anterior quadrigeminal bodies, with the fourth, sixth and seventh nerves through the posterior longi- tudinal bundle (see Medulla) and with the eighth nerve through the same bundle and lemniscus. usiurlz,/tos(e NUCLEUS OF POSTE- RIOR COMMISSURE AND MED. LONG IT. FASC. MEDIAL LONGITUDI- NAL FASCICULUS MM. CILIA RIS (a) AND SPHINCTER "' IRIDIS (b) LEVATOR v- PALPEBRM \ 3 OBLIQUUS INFERIOR — ~.v- ^ RECTUS SUPERIOR "-'•'•'-*£<_ :4 RECTUS MEDIA LIS — »-.3 RECTUS INFERIOR - (I (JTuA. '4rbckl. _-— SUPERIOR GROUP (Nucleus of Edinger and Westphal) -INFERIOR GROUP Fig. 19. — Diagram of Longitudinal Section of Nucleus of Oculomotor Nerve. {Morris' Anatomy, after Edinger.) As to the nucleus of the fifth (trigeminus) nerve, its motor portion with the descending motor fibers is found in the mid-brain. The Red Nuclei are two round pigmented masses of gray matter, situated in the tegmentum under the anterior quadrigeminal bodies. They receive fibers from the cerebral cortex and corpus striatum, also send out fibers to the thalamus and spinal cord (Fig. 20). 24 THE CENTRAL NERVOUS SYSTEM AREA OF OPTIC THALAMI, THIRD VENTRICLE. DIENCEPHALON. INTERBRAIN The Optic Thalami are two voluminous ovoid masses of gray sub- stance situated in front of and laterally to the quadrigeminal bodies (see preceding chapter) and on both sides of the third ventricle. Close to each other in front they diverge posteriorly. THALAMUS \- - INTERNAL CAPSULE BUNDLE FROM ■ RED NUCLEUS TO THALAMUS ' - BUNDLE FROM RED NUCLEUS TO INTERNAL CAPSULE - RED NUCLEUS . DECUSSATION OF BRACHIA CONJUNCTIVA . - BRACHIUM CONJUNCTIVUM (superior peduncle) INFERIOR PEDUN- CLE (resliform body) BUNDLE TO CERE- --BELLAR CORTEX ' DENTATE NUCLEUS MEDULLA OBLONGATA Fig. 20. — Transparency Drawing showing the Origin, Course and Connections of the Superior Cerebellar Peduncles (Brachia Conjunctiva) in the Formation of "Stirling's Scissors." {Morris' Anatomy.) The upper surface (stratum zonale) is white. On its anterior portion it presents an elevation, called the anterior tubercle or nucleus. The posterior elevated portion is called pulvinar. There is also a medial nucleus. On the postero-internal portion of the same surface there is the trigonum habenulse. The external margin is separated from the adjacent caudate nucleus by a linear white substance (taenia semicir- cularis). THE CENTRAL NERVOUS SYSTEM 25 The lower surface is adherent to the cerebral peduncles. The outer surface is in connection with the internal capsule and caudate nucleus. The inner surface forms the walls of the third ventricle ; in its anterior half it is united with that of the opposite side by a gray commissure. The Geniculate bodies lie close to the Thalami. The external gen- ANTERIOR CORNU OF LATERAL VENTRICLE FIFTH VENTRICLE SEPTUM PELLUCIDUM COLUMNS OF FORNIX STRIA TER3IINALIS ANTERIOR C03I3IISSURE THIRD VENTRICLE MASS A INTER- MEDIA (Middle commissure) CHORIOIDAL SULCUS SUPERIOR COLLICULUS MEDIAL GENIC- ULATE BODY LATERAL SULCUS OF MESENCEPHA L ON PONS STRIA ACUSTICA MEDIAN SULCUS TRIG0NU3I HYP0OL0SSI RESTIFORM BODY CLAVA POSTERIOR FISSURE POSTERO-INTERMEDIA TE SULCUS POSTERO-LA TERAL SULCUS CORPUS CALLOSUM NUCLEUS CA UDA TUS FORAMEN INTER- VENTRICULARS (Monroi) ANTERIOR TUBER CLE OF THALA- 31 US STRIA MEDUL- LARS THALA31I HABENULAR C03I- 3IISSURE EPIPHYSIS SULCUS CORP. QUAD. 31 EDI A LIS INFERIOR COLLICULUS FRENULUM VELI LINGULA CEREBELLI FACIAL E3IINENCE AREA ACUSTICA TRIG0NU3I VAGI TUBER CUL U3I CUNEA TU31 FUNICULUS GRACILIS FUNICULUS CUNEA TUS LATERAL FUNICULUS Fig. 21. — Dorsal Surface of Diencephalon with Adjacent Structures (Morris, after Obersteiner .) iculate body is closely attached to the posterior extremity or base of the Thalamus (pulvinar) and through the anterior brachium is connected with the anterior quadrigeminal body (Fig. 21). Third Ventricle. — It is a single cavity situated between the optic thalami, beneath the fornix. The floor is represented from the anterior 2 6 THE CENTRAL NERVOUS SYSTEM angle backward by the posterior perforated space, mammillary bodies, tuber cinereum, optic chiasma. The lateral surfaces are formed by the thalami. The anterior border extends from the foramen of Monro down to the optic chiasma and contains the two anterior pillars of the fornix between which lies the anterior white commissure. The roof is formed of the tela chorioidea covered by the fornix, upon which lies the corpus callosum. The third ventricle communicates posteriorly with the aqueduct of Sylvius and anteriorly through two openings (foramina of Monro) with the lateral ventricles. In connection with the thalamic area must be mentioned the Epithala- mus or Epiphysis (pineal body) and the Hypophysis (pituitary gland). Epithalamus or Epiphysis (pineal body). — It develops on the roof of the third ventricle. It presents an ovoid body of 10 mm. long, situated posteriorly at the entrance of the third ventricle in the groove between the anterior quadrigeminal bodies. It is fixed by its adherence to the pia-mater and by its continuity with the walls of the third ventricle. From its base appear two bands (strias pinealis) which extend anteriorly upon the upper border of the third ventricle, one on each side. Just below and lateral to the epiphysis there is a small group of nerve cells, called the habenular nucleus. Hypophysis or Pituitary Body. — It lies in the sella turcica of the sphenoid bone. It consists of two portions : a large anterior or glandular lobe and a posterior or cerebral lobe. The infundibulum is continuous only with the posterior lobe. The glandular portion originates from a diverticulum of the buccal cavity. The cerebral portion comes from the floor of the third ventricle. The first is ascending and the second is descending in development. The other portions belonging to the interbrain are: optic chiasma, tuber cinereum and infundibulum. The latter is the apex of the tuber cinereum. They are all parts of the floor of the third ventricle. CEREBRAL HEMISPHERES. TELENCEPHALON They are two symmetrical masses of nervous tissue, ovoid in shape. They occupy the cranial cavity. Their surface is gray (cortex). The average length is 16 cm., width 13 cm., height 12 cm. They present a Convexity and Base. A longitudinal fissure separates the two hemispheres. From the cerebellum, over which lie the occipital lobes, and from the mesencephalon (see above) they are separated by a transverse fissure. Each hemisphere presents an external, mesial and inferior surface. THE CENTRAL NERVOUS SYSTEM 2 7 A number of fissures or sulci divide each hemisphere into lobes, lobules, convolutions. The adjoining illustrations are sufficient to give a correct idea of the division and subdivision of the cerebral cortex by the fissures, also of the Base of the Brain with the cranial nerves (Figs. 22, 23, 24, 25, 26, 27). As to the function of the various areas, see the chapter on Localizations. Fig. 22. — Gyri of Convex Surface of Left Cerebral Hemisphere. (Santee.) Fissura lateralis cerebri: a. Stem. b. Horizontal anterior ramus, c. Ascending anterior ramus, d. Posterior ramus, e, e. Sulcus centralis (Rolandi). /. Genu superius. g. Genu inferius. h. Sul. occipito-parietalis. i,i,i. Sul. frontalis superior. j,j. Sul. frontalis inferior. k, k. Sul. frontalis medius. I, I. Sul. paramedialis. m, m. Sul. praecentralis inferior, n, n Sul. praecentralis superior. 0. Sul. post-centralis inferior, p. Sul. post-centralis superior, q. Ramus horizontals and r, ramus occipitalis of interparietal sulcus. 5. Sul. transversus. /. Sulci superior and lateralis, u. Incisura prasoccipitalis. v. Sul. temporalis superior, w. SuL temporalis medius. Interior of the Brain. — A horizontal section through both hemispheres reveals the presence of gray and white matter, also of lateral ventricles. Gray Substance. — Besides the cortical gray matter there are also isolated masses called basal ganglia. The latter are three in number, viz. Thalamus, Caudate and Lenticular nuclei. The two last ones are also known under the name of Corpora striata. The thalamus was discussed in the preceding chapter (Fig. 28). Caudate Nucleus belongs to the wall of the lateral ventricle. It forms the floor in the upper portion and the roof in the lower portion of 28 THE CENTRAL NERVOUS SYSTEM CENTRAL SULCUS (ROLANDI) PARIETAL LOBE FRONTAL LOBE OCCIPITAL LOBE CEREBELLUM CENTRAL LOBE {INSULA) TEMPORAL LOBE PONS CENTRAL SULCUS OP INSULA Fig. 23. — Diagram of the Convex Surface of thf Left Cerebral Hemisphere showing the FrvE Principal Lobes of the Pallium. (Morris' Anatomy.) The opercular regions of the frontal, pacietal, and temporal lobes are removed to show the central lobe or island of Reil. Fig. 24. — Latero-superior Vtew of Brain, showing Fissures and Lobes. (Santee.) a. Longitudinal fissure, b. Frontal pole. c. Temporal pole. d. Impressio petrosa. e. Occipito- parietal sulcus, f. Occipital pole. THE CENTRAL NERVOUS SYSTEM 2 9 the lateral ventricle. The anterior portion (head) is closely connected with the internal capsule, the body is applied to the optic thalamus, the posterior portion passes around the posterior border of the capsules to r. 1) cu r^ cu 3 £ CO >H CO IH rt rf ifl cu ft £ 1— 1 Ja C/3 ft «j _o" U "0 V CU "C 1-1 3 to CU to CU M en id cu > -a 73 3 3 CO "d M M 1 '£ £ a -2 3 •5 r -o is CO cu O ri ^ a 0) n3 3 "1 t-l O 13 U -*-> 3 hi CO & "r^ ^0 "C ,3 O X cj t» Ph 9 CU S3 M ft O > T3 '-5 cu £ ;* "rt r^ O CU a O to O "£ *Q '/i P3 ca 2 '. rt _"£ C 1-0 45 3 CO u O .« 'fi ri ■»_• £ ft - SPINAL GANGLIA Fig. 29. — Scheme of Ascending Cerebro-spinal Conduction Pathways. {Morris' Anatomy.) 3 34 SOMJESTHETIC AREA OF CEREBRAL CORTEX CA UDA TE NUCLEUS INTERNAL CAPSULE LENTICULAR NUCLEUS" CEREBRAL PEDUNCLE TROCHLEAR NER VE THE CENTRAL NERVOUS SYSTEM MEDULLA OBLONGATA MOTOR NUCLEI OF CRANIAL NER VES DECUSSATION OF PYRAMIDS LATERAL CEREBRO- SPINAL FASCICULUS VENTRAL CEREBRO- SPINAL FASCICULUS VENTRAL ROOTS OF SPINAL NERVES VENTRAL WHITE COM- MISSURE SPINAL CORD Fig. 30.— Scheme of Descending Cerebro-spinal Conduction Pathways. (Morris' Anatomy.) THE CENTRAL NERVOUS SYSTEM 35 1. Sensory, which are the continuation of the median lemniscus (see Medulla). They terminate in the thalamus and subthalamic nucleus, from which new fibers emerge and go through the posterior portion of the posterior limb of the internal capsule to terminate in the sensory area of the cortex. The median lemniscus is joined by fibers from the sensory nuclei of the cranial nerves. 2. The cochlear branch of the eighth nerve also sends projection fibers through the lateral lemniscus to the posterior limb of the internal capsule, from which fibers go to the cortex of the temporal lobe. FIBRM PROPRIA SUPERIOR LONGITUD- INAL FASCICULUS STRIA TERMINALIS t OF THALAMUS CINGULUM UN CIS A TE FASCICUL US INFERIOR LONGITUDINAL FASCICULUS Fig. 31 — Schematic Representation or Certain of the Association Pathways of the Cerebral Hemisphere. (Morris' Anatomy.) 3. The optic radiations are mentioned above. 4. The superior cerebellar peduncles terminate mostly in the red nuclei (see Mesencephalon and Thalami), from which new fibers ascend and go to the sensory area of the cortex. Descending Fibers are the following (Fig. 30). 1. Pyramidal fibers, which originate in the cells of the motor area, pass through the largest part of the posterior limb adjacent to the knee and through the latter, down to the crura, pyramids, and after decussating 36 THE CENTRAL NERVOUS SYSTEM in the medulla descend in the cord and terminate around the cells of the anterior cornua. 2. Temporal Bundle originates in the cortex of the first two temporal gyri and passing through the posterior limb of the internal capsule descend into the lateral portion of the crura and thence to the nuclei of the pons. 3. Frontal Bundle originates in the cortex of the frontal lobe and Fig. 32. — Dorsal Surface of Corpus Callosum, Cerebral Hemisphere Cut Away to Expose It. (Santee.) passing through the anterior limb of the internal capsule ends in the nuclei of the pons. 4. Occipital Bundle (visual) originates in the cortex of the cuneus and calcarine fissure and passing through the most posterior portion of the in- ternal capsule ends in the anterior quadrigeminal body. THE CENTRAL NERVOUS SYSTEM 37 B. Association Fibers. — They connect different parts of the same hemisphere. They are the following (Fig. 31). 1. Superior Longitudinal Bundle which connects the frontal, temporal and occipital lobes. 2. Inferior Longitudinal Bundle which connects the occipital and temporal lobes. CA UDA TE NUCLEUS ANTERIOR PILLARS OF FORNIX Veins of Galen POSTERIOR PILLARS OF FORNIX Straight si?nis CERE- BELLUM CORPUS CALLOSUM (in section) SEPTUM - PELLUCI- BUM FIFTH VENTRICLE STRIA TER- MINALS OF THALA- MUS THALAMUS Tela ehorioidea (velum inter - positum) Chorio id plexus - FIMBRIA HIPPOCAM-_ f^H EMINENTIA COLL A TER- ALIS HIPPO- CAMPUS MINOR Fig. 2,2,. — Horizontal Dissection of the Cerebral Hemispheres. The fornix has been removed to show the relation of the tela ehorioidea of the third ven- tricle to the chorioid plexus of the lateral ventricles. (From a mounted specimen in the Anatomical Department of Trinity College, Dublin.) 3. Occipito -frontal Bundle. 4. Fibrse propriae connecting contiguous gyri with each other. 5. Uncinate Bundle connects the uncus with the frontal lobe. 6. The Cingulum (see illustration). C. Commissural Fibers. — They connect one hemisphere with the other. They consist of 38 THE CENTRAL NERVOUS SYSTEM i. Corpus Callosum (see above, Fig. 32). 2. Anterior commissure, the largest part of which connects one tem- poral lobe with another. 3. Hippocampal commissure connects the two gyri of the same name. Lateral Ventricles. — Each of these cavities commences within the frontal lobe, is directed backward, turns around the optic thalamus to go again forward and downward and terminate in the apex of the temporal lobe. It presents an anterior, posterior and inferior cornu and a central CORP. GEN. INT. Fig. 34. — Diagram of Principal Pathways of Optic Apparatus. ham.) {Morris, after Cunning- portion or body. Its relation to various portions of the brain has been already mentioned. It communicates with the third ventricle by means of the foramen of Monro situated in the frontal portion of the latter. The tela choroidea of the third ventricle is continuous into the lateral ventricles and the varicose mass composed of blood vessels found in the lateral ven- tricles is known under the name of choroid plexus. The anterior portions THE CENTRAL NERVOUS SYSTEM 39 TEGMENTUM FRENULUM VELI CEREBRAL PEDUNCLE SUBSTANTIA NIGRA INFERIOR QUADRIGEMIN- ATE BODY CENTRA E LOBULE ALA OF CENTRAL LOBULE ANTERIOR SEMI- LUNAR LOBE POSTERIOR SEMILbNAR LOBE SUPERIOR — SEMILUNAR ' LOBE HORIZONTAL FISSURE INFERIOR SEMILUNAR m ' -^ssgysjs- CLIVUS OF MONTICULUS LOBE POSTERIOR CEREBELLAR NOTCH FOLIUM OF VERMIS Yig. 35.— Diagram of the Dorsal Surface of the Cerebellum. {Morris'' Anatomy.) SUPERIOR CEREBEL- LAR PEDUNCLE POSTERIOR MEDUL- LARY VELUM MIDDLE CEREBELLAR PEDUNCLE FLOCCULUS ANTERIOR FOURTH MEDULLARY VENTRICLE VELUM L1NGULA NODULE UVULA B I VENTRAL LOBE LOBULUS GRACILIS ANTERIOR . LOBULUSS GRACILIS POSTERIOR INFERIOR. SEMILUNAR LOBE TONSIL TUBER VERMIS PYRAMID POSTERIOR CEREBELLAR NOTCH Fig. 36. — Diagram of the Ventral and Inferior Surface of the Cerebellum after the Removal of the Medulla Oblongata, Pons, and Mesencephalon. (Morris' Anatomy.) The tonsil of the right side is omitted in order to display the connection of the pyramid with the biventral lobe, the furrowed band of the uvula, and more fully the posterior medullary velum. The anterior notch is less evident than in the actual specimen. 40 THE CENTRAL NERVOUS SYSTEM of the two lateral ventricles are separated from each other by the Septum lucidum, a thin vertical membrane attached in front to the corpus callosum and the anterior pillars of the fornix. It consists of two layers, between which there is a closed cavity called fifth ventricle, which has no communication with other ventricles (Fig. 33). Fornix. — It is placed beneath the corpus callosum. It extends from the splenium to the anterior portion of the corpus callosum, to which it is attached by the septum lucidum. It presents a body and anterior and posterior pairs of bands (columns or pillars). The anterior pillars run forward and downward, and appear at the base of the brain as mammillary bodies. The posterior pillars curve backward, downward and forward and end in the uncus. The fornix is an association tract of the limbic lobe. The posterior pillars connect the hippocampal gyri by means of a lamina situated between them. Fibers emanating from the mammillary bodies (anterior pillars) go to the thalamus and crura of the same and opposite side. OPTIC APPARATUS In discussing the structure of the brain various portions of white and gray matter were mentioned in connection with the visual apparatus. A recapitulation of those elements will be useful in this place. The adjoining illustration gives a satisfactory idea of the entire optic apparatus (Fig. 34). CEREBELLUM It lies in the posterior fossae of the cranium, under the occipital lobes of the cerebrum, behind the medulla oblongata. Exterior. — Similarly to the cerebrum, the cerebellum is divided by fissures and sulci into lobes and lobules (Figs. 35 and 36). The adjoining illustrations give a sufficiently clear idea of the external appearance of the cerebellum. Interior. — A section of the cerebellum reveals in each hemisphere a gray cortical mass, central white matter and several ganglionic masses of gray substance (Figs. 37 and 38). Gray Matter.— It is presented by the Dentate nucleus and accessory nuclei. The Dentate nucleus, situated in the center of the white substance, presents an ovoid and folded lamina with an opening (hilus) . It resembles the olivary bodies of the medulla. Three accessory small nuclei are situated near the dentate nucleus. White Matter.— In addition to the central white mass, there are also THE CENTRAL NERVOUS SYSTEM 41 Fig. 37. — Median Section of Cerebellum, Pons and Medulla. (Santee.) a. Predeclivil sulcus, b. Arbor vita?, c. Declive monticuli. d. Postdeclivil sulcus, e. Folium vermis. /. Horizontal sulcus, g. Tuber vermis. /;. Postpyramidal sulcus, i. Pyramid, j. Prepyramidal sulcus, k. Uvula. I. Culmen monticuli. m. Postcentral sulcus. n. Central lobule. 0. Inferior colliculus of corp. quad. p. Cerebral aqueduct, q. Precentral sulcus, r. Superior medullary velum, s. Lingula. t. Medial longitudinal bundle, u. Fasti- gium. v. Inferior medullary velum, w. Nodule, x. Postnodular sulcus. Fig. 38. — Sagittal Section of Cerebellum, Cutting Nucleus Dentatus. (Santee.) a. Sup. semilunar lobule, b. Corpus medullare. c. Post, part quadrangular lobule, d. Nucleus dentatus. e. Ant. part of quadrangular lobule. /. Interior of dentate nuc. g. Central sulci, h. Bracnium pontis. i. Restiform body. j. Inf. semilunar and slender lobules. k. Hilus of nuc. dent. I. Biventral lobule. 42 THE CENTRAL NERVOUS SYSTEM DECUSSATION OF BRACHIA CONJUNCTIVA DENTA TE , NUCLEUS CORONA RADIATA THALAMUS LATERAL NUCLEUS OF THALAMUS ■ INTERNAL CAPSULE RED NUCLEUS VERMIS OF CERE BELL UM (displaced } ~-— AFFERENT CRANIAL '■" NERVES (VESTIBULAR CHIEFLY) EXTERNAL ARCUATE FIBRES < NUCLEUS OF FUNICULUS CUNEATUS NUCLEUS OF FUNICULUS GRACILIS SPINAL GANGLIA Fig. 39. — Scheme of Principal Ascending Cerebellar Conduction Paths. {Morris' Anatomy.) THE CENTRAL NERVOUS SYSTEM 43 three pairs of peduncles uniting the cerebellum with the brain, mid-brain and spinal cord. The situation, course and termination of the cerebellar peduncles have been sufficiently discussed in the study of each portion of the central nervous system (see also illustrations, Figs. 39 and 40). Fig. 40. — Scheme of Cerebellar Connections. {From Poirier and Charpy.) Efferent or centrifugal fibers in red. Afferent or centripetal fibers in blue. Small circles in the center are pontine nuclei. MENINGES OF THE BRAIN The brain is surrounded by three membranes, viz. dura-mater, arach- noid and pia-mater. (Fig. 41). Dura. — It consists of two layers: an outer which serves as endosteum 44 THE CENTRAL NERVOUS SYSTEM of the cranium, and an inner layer which sends processes between subdi- visions of the brain. The outer layer adheres to the cranium, especially along the sutures and at the base. The inner layer gives off prolongations, three in number, viz. falx cerebri, falx cerebelli, tentorium cerebelli. FOURTH NERVE Falx cerebri THIRD NERVE SECOND NERVE Superior sagit- tal sinus Inferior sagit- tal sinus Vein of Galen Straight sinus Superior petrosal sinus Falx cerebelli SEVENTH AND EIGHTH NERVES NINTH, TENTH, AND ELEVENTH NER VES TWELFTH NER VE SECOND CERVICAL NERVE Ligamentum denticulatum Vertebral artery N FIRST CERVICAL NER I 'E Inferior petrosal sinus SIXTH FIFTH NERVE NERVE Fig. 41. — The Cranium with Encephalon Removed to show the Falx Cerebri, the Tentorium Cerebelli, and the places where the Cranial Nerves Pierce the Dura Mater. (Morris, after Sappey.) Falx cerebri is a vertical projection in the longitudinal fissure attached in front to the crista galli, behind to the upper surface of the tentorium cerebelli. Falx cerebelli is a smaller process of the dura than the preceding one, which is inserted between the hemispheres of the cerebellum. It is at- tached behind to the internal occipital crest and above to the tentorium cerebelli. Tentorium cerebelli is an arched tent-like process between the cere- brum and cerebellum. The two layers of the dura become separated in certain places to form venous sinuses. They are fifteen in number — five paired and five single. THE CENTRAL NERVOUS SYSTEM 45 The paired ones are two lateral, two superior petrosals and two inferior petrosals, two cavernous and two occipitals. The single sinuses are the superior longitudinal, inferior longitudinal, the straight, the circular and the transverse. The accompanying illustrations give a satisfactory idea of their re- spective seats (Figs. 41, 42 and 43). Arachnoid.— It is situated between the dura and pia. It envelops the brain but does not penetrate the fissures. At the level of the fissures into 1MIXDIBULUM Internal carotid artery . CRISTA GALLI OPTIC NERVE Spheno-pariela! sinus Middle cerebral artery Anterior cerebral artery Posterior communi- cating artery Cavernous sinus OPTIC TRACT THIRD NERVE Cerebral peduncle Aqueduct of Sylvius MESENCEPH- ALON Falx cerebri Tentorium cerebell Straight sinus Superior cerebellar artery Posterior cerebral artery Superior petrosal sinus Free border of ten- torium bounding tentorial notch Transverse sinus Superior sagittal sinus Fig. 42. — Showing the Upper Surfaces of the Tentorium Cerebelli and the Ten- torial Notch through which the Mid-brain and Posterior Cerebral Arteries Enter the Middle Fossa of the Cranium. (Morris' Anatomy.) which the pia dips the two membranes are widely separated. This is the sub -arachnoid space containing cerebro-spinal fluid. On the vertex of the brain, along the longitudinal fissure, there are small nodules, outgrowths of the arachnoid, called Pacchionian bodies. Pia-mater. — It is an extremely thin and vascular membrane closely 4 6 THE CENTRAL NERVOUS SYSTEM applied to the cortex and continuing in the fissures. Together with the other two membranes it forms the sheaths of the cranial nerves. Special processes are sent off in the spaces between certain portions of the brain. In the cavities of the third and fourth ventricles they are known as Tela choroidea, the margins of which are wrinkled, forming a vascular fringe known as Choroid plexus. The latter is also found in the lateral ventricles. Meningeal branch of anterior ethmoidal artery Meningeal branch of pos- terior ethmoidal artery Middle meningeal artery OPHTHALMIC DIVISION OF TRIGEMINUS THIRD NERVE Cavernous sinus TROCHLEAR NERVE AUDITORY AND FACIAL NERVES Superior petrosal sinus Inferior petrosal sinus Petrosquamous sinus SPINAL ACCES- SORY NERVE Sigmoid sinus Posterior meningeal branch of vertebral artery Left marginal sinus Left lateral sinus Superior sagittal sinus Circular sinus Carotid artery ABDUCENS Basilar artery Basilar plexus of veins Auditory artery Vertebral artery j_ GLOSSOPHARYNGEAL I AND VAGUS NERVES ) ' Anterior spinal artery HYPOGLOSSAL NERVE SPINAL ACCES- SORY NERVE Right marginal sinus Occipital si7ius Right transverse sinus Fig. 43.— Showing Blood Vessels of Cranial Dura Mater and Cranial Nerves in the Base of the Skull. (Morris' Anatomy.) (On the left side the dura mater has been removed in the middle fossa.) BLOOD SUPPLY OF THE BRAIN The arteries of the brain are supplied by the two internal carotids and the vertebrals. A. The internal carotid arteries divide into anterior and middle cerebral arteries and posterior communicating. The anterior cerebral arteries supply the frontal and olfactory lobes, the optic nerves, corpus callosum and anterior perforated space. THE CENTRAL NERVOUS SYSTEM 47 Fig. 44. — Middle Cerebral Artery and Branches. {Gordinier, after Quain and Charcot.) Cent. Antero-lateral group of ganglionic arteries. 1. Inferior external frontal artery. 2. Ascending frontal artery. 3. Ascending parietal artery. 4. Parieto-temporal artery. Anterior cerebral artery Middle cerebral artery Internal carotid artery Poster o-median perforating Posterior cerebral artery Superior cerebellar artery A nlerior inferior cerebellar artery Vertebral artery Posterior inferior cerebellar artery Anterior com- municating artery Antero-lateral perforating Chorioid Posterior com- municating artery Posterior chorioid Basilar artery Cerebellum, cut Anterior spinal artery Fig. 45. — The Arteries of the Brain. {Morris' Anatomy.) (The cerebellum has been cut away on the left side to show the posterior part of the cere- brum. From a preparation in the Museum of St. Bartholomew's Hospital.) 4 8 THE CENTRAL NERVOUS SYSTEM The middle cerebral arteries, the largest of the internal carotid branches, supply the frontal, parietal and temporal lobes, and through the anterior perforated space branches to the basal ganglia. The latter branches are the " ganglionic." One of the lenticulo-caudate arteries which is the largest is called "artery of cerebral hemorrhage" (Charcot). The posterior commirnicating arteries join the posterior cerebral arteries. B. The Vertebral arteries, branches of the subclavian, meet at the lower border of the pons, unite and form one trunk, viz. basilar artery, which, running in the middle line of the pons, divides at its upper border into two posterior cerebral arteries. Cortical arteries External striate arteries Middle cerebral artery CA UDA TE NUCLEUS THALAMUS TUBER CINEREUM OPTIC TRACT ANTERIOR PERFORATED SUBSTANCE Internal striate arteries Fig. 46. — Diagram showing the Manner of Distribution of the Cortical and Central Branches of the Cerebral Arteries. (Morris' Anatomy.) The basilar artery gives off anterior, inferior and superior cerebellar arteries. At the base of the brain the internal carotids and the vertebrals join and form the Circle of Willis (see illustration). The characteristic feature of the blood vessels distributed in the substance of the brain lies in the fact that they are terminal, that is, they do not anastomose with each other. Cerebral veins do not accompany the arteries, but open in various sinuses (see chapter on Meninges). The latter are without valves (Figs. 44, 45 and 46). THE CENTRAL NERVOUS SYSTEM 49 HISTOLOGICAL ELEMENTS OF THE CENTRAL NERVOUS SYSTEM Neurone Doctrine and Secondary Degeneration The chief histological elements of the central nervous system are: nerve-cells, nerve-fibers, neuroglia. Nerve-cell. — It consists of a protoplasmic body in the center of which is a nucleus with a nucleolus. It has no capsule, but the cells of the sym- pathetic ganglia and of the spinal ganglia have a capsule. The proto- plasma contains granular masses, stainable by Nissl's stain, and they are called Nissl's bodies. Ramon y Cajal has recently (1903) devised a stain with which it can be demonstrated that the cell is composed of delicate fibrils (neurofibrils). In the majority' of the cells, particularly in those of large size, exists a granular mass of yellowish pigment. Each cell is provided with processes, one of which is the most important and called axone. Varieties of Cells.— They may be bipolar and multipolar. The latter are found throughout the entire nervous system, but pre- dominate in the anterior horns of the spinal cord, the cortex, medulla, basal ganglia. Bipolar cells are found in the posterior horns of the spinal cord, spinal ganglia, and in the molecular layer of the cerebellar cortex. In the Cerebellum there are two special forms of cells, viz. Purkinje's cells and basket cells. The first are flask-shaped with a very slender and long axone having a vertical course. The second have also a long axone which has a horizontal course. Cell Processes. — Some branches of a cell branch out, tree-like, divide and subdivide, but do not anastomose. They are the dendrites. One process, called axone, is the most important. It gives off collaterals. Soon after leaving the cell it receives a coating, called myelin and becomes a medullated nerve-fiber. Nerve-fiber.- — It is the prolongation of an axone. It consists of an axone, surrounded by a myelin sheath and of a delicate membrane, neuri- lemma, surrounding the latter. The myelin and neurilemma present constrictions (nodes of Ranvier). The sympathetic system contains nerve-fibers, called non-medullated, viz. deprived of a myelin sheath. Connective tissue joins nerve-fibers into fasciculi or bundles, which when united form peripheral nerves. The nerves terminating in the muscles or skin present at their ends special arrangements. The motor nerves end in motorial end plates, which are special expansions of granular protoplasm. The sensory nerves end in corpuscles, composed chiefly of connective tissue. 4 58 FRACTURES OF THE SKULL lated blood. In cases in which localized manifestations are on the same side as the fracture, it is advisable first to operate at the seat of the frac- ture. If the results are not satisfactory, operate on the opposite side of the brain. In fractures of the base which is not accessible, Cushing obtained encouraging results from decompressive operations in the subtemporal region (Ann. of Surgery, 1908). Recently lumbar puncture has been recommended in cases of fractures at the base of the skull. The diagnostic value of lumbar puncture has been mentioned above. Very favorable results have been reported in traumatic cases treated by puncture of the spinal canal and extraction of certain amounts of the cerebro-spinal fluid (Lenhartz, Meslier, Muret, Quenu, Fowler and others). The advantage, of this procedure are: (1) relieve intracranial pressure and through it the cerebral phenomena. (2) remove partly the microorganisms which are the cause of secondary meningeal manifestations; blood is an excellent medium for development of germs. In cases of injury to the head without an evident fracture of the skull, expectant treatment is most advisable. The patient is kept quietly in bed and watched very closely. At the appearance of localized or other disturbances, the therapeutic conduct is promptly to be decided upon. CHAPTER XVI CONCUSSION OF THE BRAIN The subject of concussion of the brain is of practical importance. The common belief that only in injuries accompanied by loss of consciousness there is concussion of the brain, is erroneous. There are many traumatic cases in which there is no genuine loss of consciousness, but only a vertigo, or a transient mental hebetude, or else a hazy recollection of the trauma — all cases in which symptoms of a grave nature may develop immediately or some time after the accident. An appreciation of these milder forms of cerebral concussion is of importance from a therapeutic standpoint, as if they are ignored or overlooked, serious consequences may follow. Pathogenesis. — The nature of concussion is still debatable. As autopsies are exceedingly rare, a pathogenesis cannot be established with any degree of precision. The following views are being held: (i) There is no material lesion in the nervous system and the disturb- ances are purely dynamic or else they are due to some perturbation in the cerebral circulation. (2) There are some material lesions: they may be seen either macro- or microscopically in the medulla and affecting particularly the cardiac and respiratory centers. (3) To others the reason of death lies in anemia of the respiratory center. The anemia is the result of a compression of the blood vessels produced by oedema of the brain. The oedema is caused by a paralytic vaso-dilatation brought on by the trauma. Concussion or shaking, jarring of the brain which will be discussed here as occurring without a gross injury of the brain may result from a direct injury, such as a fall on the head or a blow, and from an indirect injury, such as striking any other part of the body during a sudden fall, landing suddenly on the feet, falling on the buttocks, etc. In all these accidents there may be a profound loss of consciousness which may develop imme- diately or else the injury may be followed by a very slight and very brief unconsciousness, also by no loss of consciousness. In the latter cases almost invariably symptoms will develop within a few hours or even a few days. Symptoms. — In the severe cases after the patient recovers conscious- ness, he will present for some time a mental hebetude, a vague expression of the face. He will complain of pain in the head especially in the occi- 14 209 2IO CONCUSSION OF THE BRAIN pital region, of noises in the ears. He will suffer from insomnia; he is irritable and at the same time apathetic. He is mentally dull, cannot concentrate his thoughts. He shows exhaustion upon the least exertion. On the least attempt to do anything, he gets covered with perspiration. If he falls asleep, he awakens often. The pulse presents this characteristic feature, that it is easily compressible and increases in rapidity while the patient is under examination. Temporary glycosuria has been observed by some writers. In the mild cases, without much or any disturbance of consciousness the immediate effect of concussion will be pain in the head, some dizziness, sometimes vomiting and a general sense of feeling weak. The pulse pre- sents the same peculiarity as in the severe cases. Insomnia, restlessness, photophobia, loss of appetite, constipation are present. The mentality may suffer, but this will be considered later. Prognosis. — The outlook is variable. Even in the severest forms recovery may follow. On the other hand I have seen mild cases which lasted months and years and recovery remained incomplete. In the majority of cases, especially when there is no history of syphilis or alco- holism, when there are no complications, such as fracture of the skull, also when the treatment is instituted at the earliest possible moment, recovery may be complete. An incomplete recovery consists of a continuous as- thenic condition, loss of weight, pains in the head and change of disposi- tion. Irritability, dfnculty of adapting oneself to surroundings may re- main for years. In a certain group of cases mental phenomena of a graver nature may develop. Among the latter he following disturbances have been observed : (i) Transitory or permanent defects of intelligence especially of memory, disturbance of ability to retain impressions, retrograde amnesia. (2) De- lirium ending either in recovery or associated with profound mental altera- tions ending in Dementia. (3) Hallucinosis. I observed this manifesta- tion in five patients. There were no delusions. The hallucinations, mostly visual, lasted for months. The patients finally recovered. (4) Korsakoff's psychosis has been observed in individuals free from alcoholism by a number of writers. (5) Finally dementia may be the ultimate outcome of concussion of brain. In children, when concussion occurs at an early age, arrest of mental development may occur. I have had the opportunity to study several children, whom I happened to know before the injuries occurred, and a decided change in their mentality and intellectual power took place. Their progress in mental development was unquestionably arrested. Treatment. — The most essential principle of treatment is absolute CONCUSSION OF THE BRAIN 21 1 rest in bed which must be instituted as early as possible after the injury. This should be carried out not only in the severe cases, but also in the cases of the mildest nature. The various manifestations should be treated symptomatically. Sedatives, purgatives, light diet and particularly avoidance of stimulation, of excitement, of noise — is all that is necessary. Confinement to bed must be as long as possible. It is reiterated here, that neglect of recognizing the importance of earliest treatment may render the patients permanent invalids. CHAPTER XVII DISEASES OF THE CEREBELLUM The cerebellum contains two important portions: cortex and central gray nuclei. In the cortex we distinguish: hemispheres and vermis. These two parts are physiologically independent of each other. The hemispheres are in connection with the cortex of the brain and basal Fig. 78. — Cerebellar Tumor Involving Left Hemisphere and Vermis. (Original.) ganglia. The vermis — with pons, medulla and spinal cord. The hemi- spheres are concerned in voluntary movements, the vermis is concerned in coordination through the vestibular nerve with the nucleus of which it is in intimate connection. DISEASES OF THE CEREBELLUM 213 The function of the cerebellum is to control the equilibrium and regu- late movements. When the cerebellum is removed, motion and equilibrium are not entirely abolished because of supplementary action of the brain and of the labyrinth. Unlike the cerebrum, the cerebellum has its influence on the muscles of the same side of the body. Lesions of the cerebellum may have a slow and progressive course, such as tumors, and an acute onset, such as hemorrhage or softening. Tumors. — Among the tumors of the nervous system those of the cerebellum are the most frequent, and as to their nature, the tubercular form is most common. All the varieties of tumors met in the brain may also affect the cerebellum. Tubercle of the cerebellum is rarely solitary and never primary; it is always accompanied by pulmonary lesions. The point of departure of cerebellar tumors may be in the peduncles or in the meninges besides the cerebellar tissue itself. It may be located in one of the hemispheres or, as it frequently happens, in the vermis. Cerebellar tumors may be also located in the angle between the pons and cerebellum. They may arise then, either in the cerebellum itself or in the cranial nerves. As to the effect of a tumor on the neighboring tissue, see the Pathology of Tumors of the Brain. Symptoms. — The general symptoms common to tumors of the brain (see this chapter) are met with here also. However they present some particularities deserving special mention. Headache is quite frequently occipital and very tenacious; it radiates to the neck. Percussion over the area corresponding to the seat of the tumor provokes pain. Vomit- ing occurs quite early. Vertigo is also a precocious sign and persists through the entire course of the disease. It is present even when the pa- tient is at rest: sitting or lying down. Stewart and Holmes observed that when vertigo is present, objects and patient himself rotate from the side of the lesion to the opposite side. Among the symptoms especially characteristic of cerebellar tumors, titubation occupies the first place. It resembles to a great extent the station and gait of an intoxicated person and consists of oscillations of the body to the right and left alternately, with a tendency to fall backward or forward. The movements are therefore zigzag-like. This condition is observed in tumors of the middle lobe. When the patient walks, he widely separates his legs. Standing is difficult because of continuous oscillations. Standing on the leg corresponding to the lesion is less steady than en the sound side. When the patient shows a tendency to walk toward one side and holds the trunk and head inclined to the same side, the lesion is on this side. This cerebellar ataxia may affect not 214 DISEASES OF THE CEREBELLUM only the lower extremities (which is usually the case), but also the upper. When the patient attempts to point to an object, irregular incoordinate movements appear. Sometimes they are tremulous, but not like in insular sclerosis, because they do not increase at the end of the act; they rather disappear. Another disturbance of association of movements observed frequently in cerebellar diseases has been described by Babinski (1899) under the name of Cerebellar Asynergy. When a patient thus affected attempts to walk, the trunk does not follow the legs in their forward movements (Fig. 65). When lying on his back the patient attempts to sit up, he Fig. 79. — Cerebellar Asynergia. {Bouchard and Brissaud.) raises his legs and flexes the thighs on the pelvis. When seated on a chair the patient wishes to raise his leg, he flexes first the thigh on the pelvis and then only he elevates his leg; the latter movement is produced very abruptly; in order to put the foot back on the ground the leg first flexes over the thigh, then abruptly the latter becomes extended and the foot reaches the floor. The disturbed associated movements just described may be confined to one side and they are then spoken of as Hemiasynergy. The same author also called attention to the following cerebellar phenomena: (1) Difficulty of Regulating Movements. — When, for example, the patient attempts to walk, he will raise his foot higher than normally DISEASES OF THE CEREBELLUM 21 5 which means excessive flexion of the thigh over the pelvis. When he wishes to put his finger on the nose, the former will overstep the point of destination and reach the cheek. (2) Adiadochokinesia. — It consists of inability to execute rapid prona- tion and supination df the hand. If it is observed on one side it usually corresponds to the seat cf the lesion. (3) Cerebellar Catalepsy. — It consists of as pecial ability to maintain a fixed position longer than normally. When a patient lying on his back with his thighs flexed over the pelvis, and the legs over the thighs, raises his limbs, the latter will at first oscillate, but in a few moments the trunk and the limbs will become fixed. This fixation may remain several min- utes and is not followed by fatigue. Luciani called attention to hypotonia and asthenia of the limbs on the side of the lesion as characteristic of cerebellar tumors. General weakness (asthenia) is characteristic of crebellar diseases. It is probably due to inability to balance, to the loss of equilibrium of various portions of the body. The position of the head deserves special mention. In 1908 (/. Amer. Med. Ass'n) I called attention to a head phenomenon which I found of localizing value in a series of cases some of which came to autopsy. It consists of an increase of vertigo or headache or of both when the head is turned to the side of the seat of the cerebellar lesion. The tendon reflexes are usually altered. All varieties may be present, from diminution and loss to exaggeration. A unilateral change does not always correspond to the seat of the lesion. Sensations are usually not involved. Ocular Symptoms. — They are present in the majority of cases and con- sist of a more or less pronounced loss of vision. The eye-grounds show hyperemia, oedema, choked disc, optic atrophy. The pupils are frequently unequal. Nystagmus is almost a constant symptom. It is more marked in lateral than in vertical movements of the eyes. It is usually slow and of wide range toward the side of the lesion but of smaller range and more rapid toward the normal side. Palsies of cranial nerves are observed in tumors of cerebello-pontine angle. Skew deviation of the eyes is some- times observed. It consists of one eye looking downward and inward, the other outward and upward. Articulation of speech is not infrequently affected in cerebellar tumors. It is slow and explosive, but not quite as explosive as in insular sclerosis. Cerebellar tumor frequently causes an increase of cerebro-spinal fluid (hydrocephalus) . This is probably the cause of the mental disturb- 2l6 DISEASES OP THE CEREBELLUM ances, apathy and hebetude observed during the last period of the disease. The symptoms of compression of the neighboring tissue are various. Hemiplegia, hemiparesis or crossed hemiplegia may be observed. The hemiplegia is not spastic, the face is not involved and hypotonia of the muscles is present on the affected side. The toe phenomenon and ankle-clonus may be absent. The cranial nerves, particularly the third and sixth, may become involved. Course. Duration. Prognosis. — Headache and vertigo are usually the first symptoms. They may persist for several months before the titubation makes its appearance. Rarely amaurosis is wanting. Asthenia also appears early. Gradually convulsions develop. The duration of the disease is indefinite. It may last from several months to several years, although usually it runs a rapid course. The termination is fatal in cases which are not operated upon, although a few cases of recovery of tubercu- loma of the cerebellum have been reported. Rapid termination occurs in cases of compression of the medulla. Diagnosis. — In a certain number of cases the diagnosis cf a cerebellar tumor presents great difficulties. This is particularly seen in the initial stage, when only general symptoms are present, as it is well known the latter are common also to cerebral growths. When iu addition to the lat- ter bulbar symptoms appear, the presumption is in favor of a cerebellar involvement. In cases in which in addition to the general signs even one of the special symptoms should make its appearance, the diagnosis will readily be made. When the cerebellar involvement is decided upon, the question of its exact seat must be solved, as operative procedures depend upon it. When the tumor is unilateral, the characteristic symptoms will be present on the same side. A tumor of the median lobe will give bi- lateral symptoms. Particular stress must be laid upon hemiasynergy, upon the unilateral occipital headache with tenderness on percussion of the occiput, upon the position of the head, upon nystagmus and other ocular symptoms, upon the state of the reflexes on one or the other side, upon the state of the reflexes on one or the other side, upon involvement of one or several cranial nerves on one side. All these informations are of great utility, as the symptoms usually correspond to the seat of the tumor. As to other diseases with which a cerebellar affection may be con- founded, tabes particularly must be mentioned. Ataxia and loss of reflexes may be present in both, but the zigzag movements of a cerebellar is not present in a tabetic; the raising of the feet high and dropping them with force on the ground are characteristic only of the tabetic. Moreover other symptoms will help to decide the diagnosis. DISEASES OP THE CEREBELLUM 217 In Meniere's disease there are: vomiting, vertigo and even titubation, but absence of headache and of ocular symptoms will render the diagnosis comparatively easy. Treatment. — In cases in which syphilis is suspected, mercurials and iodides should be tried. Relief and removal of the distressing symptoms for a long time may follow from specific treatment in cases of cerebellar gummata. In two of my cases the ataxia, vomiting and headache disap- peared completely under iodides. Even in tubercular tumors of the cere- bellum iodides will be of great value, as I could ascertain it in one of my cases, in which all the symptoms except the blindness- disappeared. In the largest majority of cases surgical treatment alone is applicable. In the chapter on Tumors of the Brain operative procedures are fully discussed with especial reference to decompression ; the latter will at least decrease the intracranial pressure and thus relieve pain, making therefore life more tolerable. But where particularly lies the benefit of operation is the prevention of blindness and restoration of sight. In one of my recent cases the patient was relieved from his headache, choked disc disappeared, sight was restored and this condition lasted almost two years when he died from pneumonia. A large part of the skull of the occipital region should be removed. In dcubtful cases as to the localiza- tion, it has been advised to trephine first the right temporal region, thus relieving general intracranial pressure. If the cerebellar symptoms remain unaltered, another operation over the occipital bone should be attempted ater (sub tentorial operation). TUMORS OF PONTO -CEREBELLAR ANGLE These tumors originate either in the meninges or more frequently in the cranial nerves of the base of the brain, particularly in the eighth nerve, but sometimes also in the fifth, seventh, ninth, tenth, and eleventh nerves. They are usually solid, resistant and small. They contain fibrous elements (fibroma, fibro-sarcoma, neuro-fibroma) . In one of my cases there was a cyst {Old Dom. J. of Med. and Surgery, 1910). They are benign tumors and their extirpation if successful, usually leads to recovery. They occupy the space between the lateral border of the pons and the cerebellar hemisphere. This space corresponds to the pos- terior surface of the petrous bone at the level of the auditory meatus, into which enter the seventh and eighth nerves. Symptoms. — General symptoms of intracranial pressure are present, viz. Headache, vomiting, stasis of papillae. The latter is an early dis- turbance. It is frequently followed by optic atrophy. Headache is 2l8 DISEASES OF THE CEREBELLUM frequently localized in the occipital region. Pressure of the cerebellum produces the characteristic cerebellar symptom group (see above). Vertigo deserves special mention. Steward and Holmes assert that objects seem to move from the side of the lesion to the normal side, but the subjective sense of rotation of the patient himself is in the opposite direction. In tumors of the cerebellum the reverse condition of the latter is observed. Symptoms referable to compression of the nerve trunks at the base are the most characteristic feature. The auditory nerve particularly suffers and this is manifested by noises in the ear and soon by deafness on the affected side, while the ear itself is intact. Next in frequency are the facial and trigeminal nerves. The involvement of the former is manifested by a hemispasm and more frequently by paresis of the face. Hyperesthesia, neuralgia or else anaesthesia, also corneal anaes- thesia with loss of the corneal reflex are signs of involvement of the fifth nerve. More rarely the sixth, ninth, and tenth nerves are affected. Corneal areflexia, hypoacousia and paresis of associated movements of the eyes are according to Oppenheim characteristic of ponto-cerebellar tumors. Operation is the only treatment. Among various methods that of Krause is the most accepted. He operates on the occipital region and exposes the cerebellar hemispheres. The vicinity of the cerebellum and* of the medulla renders the operation very serious. The mortality indeed is very great. In so-called successful cases, the most distressing symptoms disappear, but the auditory manifestations especially are very little, if any, influenced by the operation. Vision may be only improved, except when there is already optic atrophy. In spite of the gravity operations are justifiable. Some very successful cases have been reported. Recurrences of the tumors are not known. ABSCESS OF THE CEREBELLUM Etiology.— Apart from metastatic abscesses, the most frequent cause is suppuration of the temporal bone. The latter is a common occurrence in chronic otitis media. Occasionally an acute otitis media may be the cause of a cerebellar abscess. The otitis itself may develop in the course of grippe, of pharnygitis, of exanthematous infectious diseases. Speaking generally, any cause leading to caries of the temporal bone, and its petrous portion particularly, will produce a cerebellar abscess. Pathology.— When the abscess is seated within the cerebellar sub- stance, the surrounding nervous tissue may be reduced to a minimum. As the abscess is usually secondary to caries of the neighboring bone (particularly temporal), the meninges will be found diseased. Phlebitis DISEASES OF THE CEREBELLUM 2IQ of the sinuses, especially of the lateral, is a frequent finding and throm- bosis is therefore equally frequent. The surrounding cerebellar tissue undergoes softening and destruction. The pus is thick, greenish and fetid. It contains the usual microbes of suppuration, viz. streptococcus, staphylococcus or pneumococcus. Symptoms. — In acute stage the otorrhea and the deafness, which have been present for a more or less long period, become suddenly com- plicated by grave cerebral symptoms: coma with general symptoms of infection. The characteristic cerebellar symptoms cannot be revealed. In the chronic stage the special cerebellar symptoms, described in "Tumors of the Cerebellum," are easily observed. In order to avoid repetition the reader is referred to that chapter. A few special manifes- tations are worth mentioning. Rigidity of the neck and retraction of the head, slow nystagmus, pronounced vertigo upon the least movement of the head, frequent occurrence of optic neuritis — are all not infrequently observed in abscess of the lateral lobe of the cerebellum. An abscess may compress the neighboring portions of the nervous system. A crossed paralysis, viz. involvement of any of the cranial nerves on one side and of the extremities on the opposite side, is indicative of pressure at the base of the brain. An abscess may remain silent for a long time, when it is not seated in the middle lobe and strictly limited. Most of the time it spreads to the neighboring tissue and produces great damage in the nervous tissue. Prognosis. — It is identical with that of cerebellar tumors in the chronic stage, but it is very serious in the acute stage. Diagnosis. — In the chronic stage it will be extremely difficult to differentiate the disease from tumor. Otorrhea or a diseased condition of the mastoid are suggestive rather of abscess than of tumor. In the acute stage the diagnosis presents sometimes insurmountable difficulties, as apart from the ear symptoms which may be overlooked, there is very little to make a diagnosis. The general condition is that of meningitis. Treatment. — Operative intervention is the only treatment and it should be done as early as possible. A large opening should be made in the skull and the pus evacuated, if the abscess is on the surface of the cerebellum; a puncture should be made in the cerebellum, if the abscess is within the latter. CEREBELLAR HEREDO-ATAXIA u Under this name P. Marie described a form of cerebellar incoordina- tion hereditary in character and occurring in several members of the same family. 2 20 DISEASES OF THE CEREBELLUM Pathology. — The characteristic condition of this affection is atrophy of the cerebellum, which is due to arrested development. The autopsy records show that in some cases in addition to the cerebellar atrophy there was also atrophy of the medulla and of the spinal cord. In Marie's first case there was also sclerosis of Goll's, Gower's and direct cerebellar tracts, finally marked diminution of the middle cerebellar peduncles. In some cases the changes were found to affect only the gray matter of the cere- bellum. The cerebellum is rarely alone affected. Symptoms. — The striking feature is the disturbance of coordination. The lower extremities are affected long before the upper. The usual cerebellar titubation with asynergia, asthenia, scoliosis, ocular disorders, exaggerated reflexes and integrity of sensory functions — otherwise speak- ing, all the symptoms found in cerebellar diseases (see above) are also found in Marie's disease. The characteristic features lie in the evolution of the symptoms. The onset is gradual. In some cases neurasthenic symptoms precede the disturb- ance of equilibrium. The patient complains of headache, pain in the back and general fatigue. The first signs appear early in life, generally between fifteen and twenty-five years of age. Disturbance of equilibrium, which is soon followed by a typical cerebellar ataxia, are the initial symptoms. They are soon followed by a disturbance of speech and voice : the speech is irregular, each word is accentuated and precipitated; the voice is monoto- nous and guttural. When the patient speaks, there is noticeable an exaggerated contraction of the muscles of the face. In the course of the malady incoordination of the upper extremities usually develops. Their movements become uncertain; a fine intention tremor is quite frequently present, so that delicate acts, such as writing, threading a needle, etc., are almost impossible. The psychic faculties are usually altered. Impairment of intelli- gence, of memory, irritability and indifference are the main disturbances observed. The disease is invariably progressive, although it may remain station- ary for a long time. In its lastp eriod there is absolute physical impotence : the patient is confined to bed and usually dies from some intercurrent disease. Diagnosis. — The history, the onset, the gradual development of the symptoms, the family character of the disease will enable one to differen- tiate it from tumors, hemorrhages or abscesses of the cerebellum; also from cerebral diplegia. Some difficulty is found sometimes in distinguish- ing it from Friedreich's ataxia. In favor of heredo-cerebellar ataxia will be the family character, the age at which it occurs, the slow development, DISEASES OF THE CEREBELLUM 221 cerebellar gait, asynergia, incoordination of the upper extremities, ataxia in writing, nystagmus, the peculiar facial mimicry, intention tremor, finally increased knee-jerks with ankle-clonus. The condition of the knee- jerks is particularly important, as their abolition is characteristic of Fried- reich's ataxia. The latter affection may present sometimes, especially at the beginning, normal reflexes; on the other hand, in Marie's disease, when the lesion reaches the cord, the reflexes may be abolished. In such cases the other symptoms will aid in making a diagnosis. Etiology. — A neurotic family history is not infrequently obtained. Alcoholism, tuberculosis in parents are reported by some observers. Con- sanguinity and syphilis have been noted in a limited number of cases. Some writers mention also infectious diseases and traumatism. The fe- male sex is apparently more frequently affected than the male. Members of the same family become frequently affected at the same age. Treatment. — Antisyphilitic treatment may be tried, but in view of the character of the lesion (see Pathology) no results can be expected from medications. HEMORRHAGE AND SOFTENING OF THE CEREBELLUM Hemorrhages are quite rare. In ioo cases of cerebellar diseases de- scribed by Krauss there was one case of hemorrhage. In 187 cases of apoplexy Starr found only four cases in the cerebellum. In the majority of cases they have been found in the hemispheres. Minute hemorrhages are less frequently observed than large ones. When a hemorrhage occurs, the blood is apt easily to break through the cerebellum and invade the fourth ventricle. The superior and inferior cerebellar peduncles are rarely the seat of hemorrhages, but the middle peduncle may be invaded by a hemorrhage originating in the pons. Softening of the cerebellum is extremely rare for the following ana- tomical reasons: (1) the angle formed by the basilar and cerebellar arte- ries interferes with the formation of embolism in the latter; (2) thrombosis is very rare in the cerebellum. Symptoms (of Hemorrhage). — The prodromal symptoms and the onset itself are identical to those of hemorrhage in the cerebrum. Pain in the oc- cipital region, persistent vomiting are very early symptoms. If the patient does not die immediately after the loss of consciousness, he remains coma- tose. When consciousness is regained, the striking differential sign will be the absence of hemiplegia, which on the contrary is so constant in apoplexy of cerebral origin. However, Mann (Monatschr. fur Psychiatrie u. Neurol- ogie, 1902) described cerebellar hemiplegia on the side of the lesion which 22 2 DISEASES OF THE CEREBELLUM presents the following characteristics: it affects all the muscles of the both limbs, while in cerebral hemiplegia only certain muscular groups are affected, such as flexors of the leg and of the foot, extensors of the hand; moreover, there is no contracture; the reflexes are exaggerated; there is no rigidity, no ankle-clonus, no toe phenomenon. After the patient recovered from the immediate effects of the attack, there is noticed an extreme general weakness (not a paralysis), so that the patient cannot even remain seated. Soon the other symptoms of cere- bellar diseases make their appearance, viz. cerebellar ataxia, etc. (see Cere- bellar Tumors) . If paralysis develops, it is almost never at the beginning, but later, and it is due to pressure upon the pons and medulla. In softening the onset will not be sudden, but progressive. Later the symptoms will be identical with those of hemorrhage. In view of a considerable anastomosis in the cerebellar arterial supply, softening is usually confined to a small area. CHAPTER XVIII DISEASES OF THE MEDULLA, PONS AND THE FOURTH VENTRICLE These three portions of the nervous system contain extremely impor- tant elements. The sensory and motor pathways, the nuclei of ten of the cranial nerves, centers for some of the vegetative functions — are all located here. In diseases therefore of this area the symptomatology is complex. There are, however, a few groups of symptoms which constitute special forms of diseases characteristic of involvement of this area of the nervous system. A. ACUTE SUPERIOR POLIOENCEPHALITIS (HEMORRHAGIC) Pathology. — The lesion consists of an inflammation with hemorrhages in the gray matter of the aqueduct of Sylvius. The latter is therefore found in a state of softening. Microscopically are seen dilated blood ves- sels, the perivascular spaces are filled with blood, a leucocytic infiltration is marked. Sometimes this pathological process extends forward into the floor of the third ventricle and backward into the fourth ventricle. The nucleus of the third nerve, also the fibers emerging from it, are therefore the main seat of the lesion. Etiology. — The most frequent cause is chronic alcoholism. Other intoxications, as carbonic acid, sulphuric acid, infectious diseases, alimen- tary intoxications (fish, meat, etc.) . are sometimes followed by superior polioencephalitis. Symptoms.- — The onset is acute. In the course of chronic alcoholism or an infectious disease the patient is suddenly taken with headache and vertigo. Soon somnolence and delirium make their appearance. Rapidly palsies of the eye muscles develop. Most cf the time both eyes are involved, but the degree of palsy is unequal. Sometimes an associated paralysis is observed; either both external recti or both internal recti are affected. Ptosis is not frequent. Wernicke (whose name is attached to the disease) claims that the sphincter of the iris is never involved. I have records of two personal cases with palsy of this sphincter. Optic neuritis, nystagmus may occur. 223 2 24 DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE In addition to the ocular disturbances, which are the characteristic feature of the disease, other symptoms are not infrequently observed. Ataxia, unilateral paralysis of the extremities, disturbance of speech, exaggeration or abolition of the tendon reflexes, disturbances of degluti- tion and of mastication may be met with. As the inflammation may become diffuse and be ascending or de- scending or both, cerebral as well as spinal symptoms may be associated. Polioencephalitis, superior and inferior (see further), and poliomyelitis are not infrequently combined (polioencephalomyelitis). The exten- sion of the inflammatory process from the gray to the white matter of the pons and medulla (which is not rare) will explain the above-mentioned additional clinical phenomena. Course, Duration and Prognosis.— The evolution of the symptoms is rapid. In the majority of cases the duration is from eight to fourteen days, although in one case it lasted sixteen weeks. Recovery was re- poited in a few cases. In one of my cases the palsy of the third and fourth nerves has been in existence two and one-half years. The patient is still living. The onset in this particular case was apoplectiform in nature with loss of consciousness. The palsy affected all the muscles supplied by the third and fourth nerves. In another case, a child of ten months, there was also loss of consciousness with involvement of the third, fourth and sixth nerves simultaneously. The child is now three years old, enjoying good health and the nerve palsy has greatly improved. In both cases the paralysis is bilateral, but unequally distributed to vari- ous muscles of the eye globes. In the case of the child the condition was preceded by an attack cf cough for a period of four or five days, evidently of infectious nature. In the first case the woman was ad- dicted to the use of alcohol. She was thirty-five years of age. Both patients present a nystagmus on lateral movements. The tendon reflexes were exaggerated in the woman. Treatment. — The general symptoms may be relieved by the usual medications, as for example the headache by opium, morphia or coal-tar products. Bleeding, purgatives and diaphoretics may be tried. Little reliance should be placed upon drugs. Iodides may do some good. B. CHRONIC SUPERIOR POLIOENCEPHALITIS (PROGRESSIVE NUCLEAR OPHTHALMOPLEGIA) The disease is characterized by a slow but progressive paralysis of the muscles of the eye. Pathology. — Atrophy of the motor nuclei cf the eye is the character- DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE 225 istic lesion. Chromatolysis with formation of vacuoles, also marked pigmentation of the cells of the nuclei, are the first stage of the affection. Later on the cells disappear and the nuclei are in a state of atrophy. In some cases the nuclei of all the motor nerves of the eye are affected, in others only the nucleus of the third nerve is diseased. Secondarily the roots emanating from the nuclei, the nerves themselves and the muscles innervated by the latter undergo degeneration. Etiology. — Infections, intoxications, syphilis may be the causes of the malady. It is occasionally observed in the course of organic diseases of the nervous system : disseminated sclerosis, tabes, paresis. Symptoms. — The onset is insidious. While there is no constancy in the order of involvement of individual ocular muscles, nevertheless ptosis and diplopia are the first symptoms in the majority of cases. As at the beginning, the palsy is not complete, the movements of the eye globe in certain directions are yet possible, but only after an effort. The facics of the patient is quite characteristic. As the upper eyelids are lowered, he holds his head thrown backward in order to be able to see objects; the forehead is wrinkled as the frontal muscles are trying to raise the palsied eyelids. The movements of the eye depend upon the muscles involved. In an advanced stage of the disease the eyes become immobile, and in order to see on the right or on the left, the head must be turned. External ophthalmoplegia is frequently associated with internal ophthalmoplegia. In such cases the pupil ceases to react to light or accommodation. Course. Duration. Prognosis. — When the lesion remains ccnfined to the ocular nuclei, the disease may last an indefinite number of years. The disease may begin unilaterally and remain confined to the same side. When it has a descending course and becomes complicated by an infe- rior polioencephalitis (see further, death may ensue in a very short time. At all events a nuclear ophthalmoplegia is an incurable and grave affection. Diagnosis. — From the acute form it will be distinguished by the ab- sence of general symptoms (see above), by the slow course. Opthalmo- plegia may be due to a lesion at the base of the brain, but in the latter case other cranial nerves are usually involved. In the course of infectious diseases or intoxications external ophthalmoplegia may be observed, but usually there are also palsies of the limbs, of pharynx, larynx, of face. These are cases of multiple neuritis and bear usually a favorable progno- sis : improvement is almost invariable. In Myasthenia gravis (see this chapter) there is also a weakness of the masticator muscles and the general asthenia is very striking. is 226 DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE Paralysis of the muscles of the eye may be the result of a disease of the orbit. In such cases the diagnosis is easy. Treatment. — There is practically no medication to rely upon. Mer- cury and iodides should be tried even in cases without a history of syphilis. Electricity may also be applied. C. ACUTE INFERIOR POLIOENCEPHALITIS (ACUTE BULBAR PALSY) Pathology. — The lesion is identical to that of acute superior polioen- cephalitis (see above). The difference lies only in the seat. Hemor- rhages and softening are the immediate causes. Here all other nuclei, ex- cept those of the third, fourth and sixth cranial nerves, are involved of only some of them. A not infrequent occurrence is the association of both forms of polioencephalitis. Etiology. — Infectious diseases and alimentary intoxications are reported to be the causes of the disease, which is, however, quite rare. Symptoms. — The onset is sudden or rapid and characterized by general symptoms, such as headache, chills, fever and pain in the neck. Loss of consciousness is usually present in hemorrhages, but not in softening. Soon the patient develops difficulty in swallowing and articulation; other- wise speaking, the picture is that of labio-glosso-laryngeal paralysis (see next chapter). The tongue, lips, palate are paralyzed. The food is regurgitated through the nostrils and the patient is threatened with suffo- cation. The pulse and respiration are irregular. Trismus is according to Joffroy a characteristic symptom. A comatose state sets in rapidly and death is the usual termination. Termination. Prognosis. — The disease usually lasts from two to six days. Some exceptional cases of recovery have been reported. Treatment. — Revulsion and counterirritation on the neck, also purga- tion, are the only means for the disease, which is almost invariably fatal. D. CHRONIC INFERIOR POLIOENCEPHALITIS (CHRONIC BULBAR PALSY) (LABIO-GLOSSO-LARYNGEAL PARALYSIS) The disease is characterized by a paralysis of the muscles of the lips, tongue, pharnyx and larynx. Pathology. — The lesion consists of a primary and progressive degenera- tion of the nuclei of origin of the cranial nerves, situated in the lower half of the medulla, viz. those of the seventh, ninth, tenth, eleventh and twelfth pairs. The alterations are most marked in the nucleus of the hypoglossus DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE 227 (twelfth). They consist of diminution or disappearance of the chromato- philic substance of the cells, of appearance of pigment within the cells, of a displacement or disappearance of the nucleus or nucleolus, finally of atrophy of all the prolongations of the cells. The nerve-roots emanating from the nuclei therefore appear very thin. The degree of involvement is not equal in all the cells of the same nucleus and in various nuclei. As to the participation of the white matter of the medulla in the pathologic process, all the writers are not agreed. Some claim (Ray- mond, Leyden, Dejerine) that the labio-glosso-laryngeal paralysis of Duchenne is not an autonomous disease, but is almost always followed by an involvement of the pyramidal bundles to constitute amyotrophic lateral sclerosis. In the ascending course of the latter disease bulbar symptoms almost invariably appear, but there is also a form in which the disease begins with labio-glosso-laryngeal palsy (see Amyotrophic Lateral Sclerosis). The muscles in which the affected cranial nerves are distributed also undergo atrophy, viz. those of the tongue, lips, pharynx and larynx. Etiology. — Syphilis, fatigue, Bright's disease have been reported in the histories of some patients, but there are no positive data as to the true etiological factors of this affection. Bulbar paralysis may occur in the course of amyotrophic lateral sclerosis, tabes, multiple sclerosis and syringomyelia. Symptoms. — The onset is insidious and slow. There is usually a brief prodromal stage during which the patient complains of pain in the neck and a numbness of the pharynx. Gradually the lips, larynx and the tongue become paralyzed. The latter particularly is affected first in the major- ity of cases. At the beginning there is only a weakness in the movements of the tongue, but it keeps on increasing until complete immobility is established. The letters that require the cooperation of the tongue aer imperfectly pronounced. The speech is therefore impaired (dysarthria). The tongue is flat, dixninished in size and presents fine fibrillary contrac- tions, also reactions of degeneration. In an advanced stage the atrophy cf the tongue is very marked; it then presents a depression and on palpation it is very soft. The speech is impossible (anarthria). Mastication and deglutition are difficult. The lips follow the tongue. It is the orbicularis muscle that is first affected, but soon other muscles suffer. The atrophy and paralysis of the lips interfere with the pronunciation of labial letters, with the act of blowing, whistling and laughing. Reactions of degeneration appear early. The lips being immobile, the mouth remains open and the saliva is con- tinuously dribbling. The facies is quite characteristic at this period. 228 DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE While the expression of the eyes shows total integrity of intelligence, the condition of the mouth gives the impression of a stupid and crying face. This contrast is typical of bulbar palsy. Paralysis of the palate is manifested by a change in the tone of the voice; the latter is nasal. The food is then regurgitated through the nose. When the pharynx is paralyzed, the food is likely to fall into the larynx, as the deglutition is very difficult. The patient is threatened with suffocation. Artificial feeding is then necessary. The paralysis of the larynx increases the danger: the glottis being open, the food easily falls into the larynx. The laryngeal condition, consisting of paralysis of the vocal cords and of all the muscles innervated by the recurrent nerve, produces dis- turbances not only of the voice, but also of phonation. The patient is unable to emit even a sound (aphonia). The gradual but progressive involvement of the medulla leads to cardiac and pulmonary disorders. The pulse is small, irregular and feeble; attacks of syncope are quite frequent. The least effort brings on dyspnea. The patient is unable to expectorate, to breathe properly: mucus accumulates in the bronchial tubes. An ordinary bronchitis becomes thus very serious. Broncho-pneumonia develops easily. Among other symptoms the condition of the reflexes should be men- tioned. Those of the extremities are very frequently exaggerated. This fact shows the close relation of bulbar palsy to amyotrophic lateral sclerosis. Course. Duration. Prognosis. — The disease is essentially progres- sive. Death may occur from three causes: inanition (because of the inability of swallowing), syncope or broncho-pneumonia. The latter may be of infectious or gangrenous nature when food enters the larynx. The usual duration of the disease is from a few months to a couple of years. Diagnosis. — The essentially chronic and progresssive course after an insidious and slow onset, the successive involvement of the tongue, lips, palate and masticatory muscles, atrophy of the muscles with fibrillary contractions and reaction of degeneration are sufficiently characteristic symptoms for the diagnosis of bulbar palsy. Palsy of the palate following diphtheria is accompanied by difficulty of swallowing and a nasal intonation of the voice. These cases will be recognized by absence of paralysis of the tongue and lips. Hemorrhage and softening of the medulla are sudden in onset and the symptoms are pronounced at the beginning. DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE 229 In Pseudo-bulbar palsy there is always a history of one or two attacks of apoplexy. The syndrome of labio-glosso-laryngeal paralysis is usually established after the second attack, but it has not the chronic character of the pure bulbar palsy. Besides, there is no muscular atrophy or fibrillary twitching. The spasmodic laughing and crying, impairment of intelligence, hemiplegic condition of the extremities are all typical of the pseudo-bulbar palsy (see this chapter). Asthenic bulbar paralysis (myasthenia gravis) is recognized by the predominance of the paralysis in the muscles of mastication and in the levator palpebrae (ptosis). The extreme exhaustion manifested in the muscles upon the least exertion, the absence of atrophy and the special electrical reactions are characteristic of myasthenia gravis (see this chapter). Treatment. — Counter-irritants, cautery on the back of the neck, also galvanism at the same level or direct electrization of the affected muscles; antisyphilitic remedies, belladonna or atropin for diminishing the saliva- tion, general hygienic measures are all the therapeutic means we have at our command for a palliative treatment. Unfortunately the disease is inevitably fatal. When difficulty of deglutition supervenes, artificial feeding is necessary. In case of imminent asphyxia, tracheotomy should be performed. E. PSEUDO-BULBAR PALSY This name is given to the syndrome of glosso-labio-laryngeal paralysis described in the preceding chapter, which is not due to a lesion in the medulla but in the brain. Pathology. — In order to understand the pathological mechanism of this symptom-group it is necessary to recall the following anatomical facts. The muscles of the tongue, face, lips, pharynx and larynx are inner- vated by two systems of neurones. One, lower or peripheral, connects the muscles with the nuclei of the medulla. The other connects the bulbar nuclei with the cortex, and especially with the Rolandic operculum (see Anatomy) ; the connecting fibers lie in the geniculate bundle of the internal capsule. A lesion of the first neurones will give place to the genuine bulbar palsy (labio-glosso-laryngeal) of the preceding chapter. A lesion affecting only the second (upper or cortico-bulbar) system of neurones will result in a loss of transmission of stimulation from the brain to the lower neurone, and we will have a pseudo-bulbar palsy. In the latter case the lesion must be bilateral, because each hemisphere innervates the 230 DISEASES OF MEDULLA, PONS AND EOUE.TH VENTRICLE muscles of both sides. A unilateral lesion of the cortico-bulbar pathway will therefore fail to produce the complete picture of labio-glosso-pharyn- geal paralysis. As to the nature of the lesion, it may be a hemorrhage, softening, cysts or patches of sclerosis (in disseminated sclerosis). According to Brissaud the lesion may occur bilaterally in the third frontal convolution, in the basal ganglia, in the cortex of one hemisphere and in the basal ganglia of the other. It may also occur in bilateral involvement of the motor segment of the internal capsule. In the majority of cases the lesion, which is either hemorrhage or softening, is extensive and involves the central ganglia and the internal capsule. Etiology. — Any condition which is apt to lead to a morbid state of the cerebral blood vessels and consequently to hemorrhage, embolism and thrombosis is the cause of the disease. Syphilis, arteriosclerosis, cardiac diseases are therefore the chief factors. Infantile Pseudo-bulbar Palsy has been also observed by Oppenheim and others in connection with diplegia (see this chapter) . It is due to an arrested development or malformation of the lower parts of the central convolutions. Symptoms. — In the majority of cases there is a hsitory at first of one attack of hemiplegia with some slight disturbance of phonation or deglutition. Soon a second apoplectiform seizure takes place and with it a complete picture of bulbar palsy is established. All the symptoms of a true bulbar palsy will be observed here. A pseudo-bulbar individual will therefore present: immobility of the facies, stupid expression of the latter, a continuously open mouth, dribbling of the saliva, paralysis of the muscles of the cheeks, lips, tongue, palate, of mastication, of the vocal cords, abolition of the pharyngeal reflex; finally a nasal intonation of the voice, dysarthria with or without aphonia, dysphagia or complete inability to swallow, difficulty of respiration with attacks of dyspnea (see chapter on Bulbar Palsy). In addition to these symptoms there are a few special signs charac- teristic of the pseudo-bulbar form. They are: absence of atrophy and of fibrillary tremor in the paralyzed muscles, preservation of normal electrical reactions and of the reflexes in the region innervated by the bulbar nerves. To this may be added a unilateral or bilateral hemiplegia, frequent involvement of the optic nerves (neuritis or atrophy), finally mental symptoms. The latter consist of a marked impairment of memory, of apathy, confusion and of dementia. Spasmodic attacks of laughing or crying are almost typical of pseudobulbar paralysis. The latter phe- nomenon is generally explained by an interruption of inhibition which DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE 23 1 is normally trasmitted to the bulbar nucleus of the facial nerve through the cortico-bulbar fibers. Course, Duration, Prognosis. — The disease is essentially progressive, but slow in its course. It may be interrupted or aggravated by slight apoplectiform seizures. The outlook is grave, as the termination is almost invariably fatal. It may last many years before death ensues. Diagnosis. — The sharply defined symptoms enumerated above will enable to differentiate this form from the true bulbar palsy (see also the latter). The acute bulbar palsy will be recognized mainly by the absence of mental symptoms and hemiplegia. Asthenic Bulbar Paralysis (myasthenia gravis) presents a series of characteristic symptoms, such as extreme exhaustion upon the least effort, ptosis and palsy of other ocular muscles, inability to hold up the head, a special myasthenic reaction to electricity, etc. Treatment. — Antisyphilitic medications may be tried even in cases without a clear specific history. As to the disturbance of respiration and deglutition, the same precautions should be taken as in the true bulbar palsy. F. ASTHENIC BULBAR PARALYSIS (MYASTHENIA GRAVIS OF ERB AND GOLDFLAM) Pathology. — Various changes in the central nervous system have been reported by a number of observers, but denied by others, so that in the state of our present knowledge there is nothing definite concerning the pathology of the affection. As in many cases, the autopsy findings have been absolutely negative, the disease is called: bulbar palsy sine materia. It should be mentioned, however, that degenerative changes in the thymus (Weigert, Hansemann, GoldfLam), tumors in the lungs, medias- tinum and thymus were found in some cases. Degenerative changes have also been observed in the thyroid and in the pituitary body. Epen- dymitis of the aqeuduct of Sylvius has been reported. The muscles are very frequently altered: infiltration of cells between the fibers, hyaline degeneration. Buzzard (Brain, 1905) gave the name "Lymphorrhagia" to the infiltrated groups of lymphocytes scattered between the cells of the nuclei in the medulla and he considers them characteristic of myas- thenia gravis. To explain the pathogenesis of the affection several theories have been advanced. Among them the most important is the pluriglandular view. According to it the altered internal secretions of various glands play an essential role in the myasthenic syndrome: profound changes 232 DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE in the glands have been found in a number of cases (see above). The pluriglandular apparatus has an effect on the bulbo-spinal motor system which is involved in myasthenia gravis. The disturbance of this apparatus has also a trophic effect on the muscles involved in the disease. Massolongo (Riforma Medica, 191 2) believes that what- ever the role of the glands with internal secretions may be, the clinical picture of the affection requires also a special inherent predisposition of an organic and morphological character of the bulbo-spinal centers. There is an inherent weakness of the gray motor centers of the mesence- phalon and the spinal cord which produces a muscular exhaustion following an exertion. Etiology. — The causes of the malady are as little known as its path- ology. The prevalent opinion is in favor of a toxic nature: in a number of cases the disease developed after infectious diseases, also in instances of auto-intoxication. Women are more frequently affected than men: children very rarely. Symptoms. — After a brief prodromal period, consisting of headache and occipital pain, also of vertigo, in the majority of cases the symptoms begin with ptosis. The patient is compelled to contract the frontal muscles to assist the levator palpebras in raising the eyelids. Very soon appears external ophthalmoplegia, so that strabismus and diplopia are among the earliest symptoms. When the facial nerve is paralyzed, both portions of it (upper and lower) are involved. This fact, together with the ptosis, gives the facies a peculiar aspect: the face is immobile, without wrinkles and somnolent, the head is raised when an object is looked upon. The muscles of mastication, the tongue, lips and the larynx are in a paretic condition. Mastication, deglutition, phonation are therefore affected. Gradually the muscles of the neck become involved. The patient cannot then hold up his head, which has a tendency to fall for- ward or backward. This is quite characteristic of the disease. In a still more advanced stage the muscles of the trunk, of the abdomen and of the extremities become similarly affected. The patient is unable to sit up or stand up and has difficulty in breathing; dypncea is marked on the least exertion. In the shoulder muscles the weakness is very marked. The patient is unable to hold up his arms. In spite of the paralysis or paresis of the muscles of a more or less long duration, muscular atrophy is extremely rare, but a very important feature of the affected muscles is their special response to electrical stimu- lation. This is the so-called myasthenic reaction. It is an exhaustion reaction and consists of a gradual diminution and finally of a loss of DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE 233 contraction, when a faradic electrical current is applied to the muscles. If after a brief rest the current is reapplied, the same phenmenon will be observed. This reaction is not observed from application of a galvanic current. Sensations, reflexes and the function of the sphincters remain intact. Subjective aching in connection with exhaustion is frequently present. The reflexes become exhausted by frequent tests. Mentality is also preserved. One of the most characteristic features of the disease is the remarkable variability in the paralytic phenomena. Thus, for example, at a certain time of the day, especially in the morning, the patient is able to move about and exercise his muscles, but he soon gets exhausted. He may begin to speak, but gradually the voice gets weaker and finally complete aphonia sets in. He may attempt to whistle or blow out a candle; at first the acts are normal, but a second or third attempt are almost impossible. Course, Duration Prognosis. — In the majority of cases the onset is with the muscles of the eyes and the disease has a descending course, but there are also observations showing that it may begin in the ex- tremities and ascend. Speaking generally, the evolution of the symp- toms is slow and progressive, although it may assume an acute form. Remissions are not infrequent and then the symptoms may also totally disappear. The disease may last ten or fifteen years. The prognosis is grave, as respiratory disturbances are not infrequent. Disturbances of deglutition are equally to be feared. Sudden death may ensue. The cases of so-called recovery are only long remissions. Diagnosis. — The characteristic symptoms described above, viz. the onset, gradual descending development of symptoms, characteristic faces, myasthenic reaction, finally the variability in the paralytic phenomena are all sufficient facts to enable us to make a diagnosis. From the chronic form of bulbar paralysis it will be distinguished by the absence of atrophy, fibrillary contractions and reactions of de- generation, and by the presence of the exhaustion. Treatment.- — It consists mainly of taking special care of the act of deglutition. The patient should be advised to eat very slowly. As soon as difficulty arises, artificial feeding must be resorted to. Absolute rest is indispensable in view of the extreme exhaustion upon the slightest effort. The presence of the myasthenic reaction in the affected muscles is a direct warning that electricity is a dangerous procedure; it must there- fore never be applied. Adrenalin, extract of thymus and thyroidin have given some favorable results in some cases (Raymond, Buzzard). Sezary (Semaine. Med., 1913) suggests that when adrenalin is given in ade- 234 DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE quate doses, benefit will be derived. He has seen cases in which sub- cutaneous injections of the whole extract or ingestion of fresh adrenals succeeded when the powder and epinephrin had failed completely. His experience demonstrates that certain slow changes in the adrenals, alone or with changes in other ductless glands, may induce a myasthenic syndrome. This justifies adrenal organotherapy, supplementing the adrenal with hypophysis treatment. Among other remedies, Iodides, arsenic, phosphorus, iron and strychina should be tried. G. HEMORRHAGE AND SOFTENING OF THE MEDULLA Pathology. — Hemorrhages of the medulla are rare and are produced by the same causes as usual cerebral hemorrhages. They frequently invade the fourth ventricle. Softening is more frequent. It is caused usually by thrombosis, but also by embolism. Etiology. — Traumatism of the cranium, infectious diseases, intoxica- tions, eclampsia are the causes of hemorrhage. Syphilis is a frequent cause of thrombosis and cardiac diseases of embolism (see Hemorrhage and Softening of the Brain). Symptoms. — The general clinical picture is that of labio-glosso- laryngeal paralysis (see this chapter). The difference lies in the rapidity of development of the symptoms. (a) Hemorrhage. — The sudden onset may be followed by immediate death. In some cases the patient may remain comatose for hours or even days. In another series of cases (less acute) consciousness is regained and then the picture of bulbar palsy becomes evident, viz. dysarthria, difficulty of mastication and of deglutition. To these symp- toms are added paralysis of the extremities, which presents several forms. The most frequent form is that of crossed hemiplegia, consisting of paralysis of the extremities on the side opposite to the lesion and of the ninth, tenth, eleventh or twelfth nerves on the side of the lesion. Besides a motor hemiplegia, there may be present a hemianesthesia. The symptoms will depend upon the seat of the hemorrhage. (b) Softening. — The symptoms are identical to those of hemorrhage. The difference lies only in the mode of development. There is usually a prodromal period, during which (especially in syphilitic cases) headache, somnolence, vertigo are present. The paralytic symptoms of the cranial nerves and of the extremities develop gradually. Course, Duration, Prognosis,— In hemorrhages of the medulla, with a less sudden onset, death is caused by a difficulty of respiration or by DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE 235 pneumonia from deglutition in the larynx. In softening, when death does not occur at the end of a few weeks, the symptoms usually show a tendency to improve. In specific cases especially the prognosis is very favorable. If the patient survives and a certain number of motor nuclei of the medulla were involved, atrophy with reaction of degeneration will remain in the affected muscles, but there is not the usual symmetrical distribution which is seen in typical chronic bulbar paralysis. Diagnosis. — It will be difficult in the acute cases, but when the symp- toms are less acute, the above symptoms (bulbar palsy associated with motor or sensory paralysis of the extremities) will enable one to make a diagnosis. Softening will be differentiated from the chronic bulbar palsy by the asymmetrical distribution of cranial nerve involvement and para- lysis of the extremities. Pseudo-bulbar palsy will be recognized by the history of apoplectic seizures, by the mental symptoms and the spas- modic laughing and crying. Hemorrhage will be differentiated from soft- ening by the mode of onset and evolution of the symptoms. Treatment. — It will be that of hemorrhage or softening of the brain. Antisyphilitic treatment should be insisted upon. When difficulty of deglutition makes its appearance, artificial feeding must be resorted to. H. OCCLUSION OF THE POSTERIOR INFERIOR CEREBELLAR ARTERY. Among the earliest typical cases of thrombotic occlusion of this artery, verified by autopsy is that of Huhn (N. Y. Med. Jour., 1897). Spiller has recently (1908) presented this subject in all its details. The general picture of the disease is briefly as follows: Sudden onset with or without loss of consciousness, unilateral paralysis of palate and vocal cord; difficulty of swallowing and expectorating; hemiataxia; syringomyelic sensory dissociation on the opposite side. In my case {International Clinics, V. IV) there was also right hemiasynergia, tendency to fall to the right side, pain in the neck, paresthesise on the left side where the objective sensory dissociation was present, paresis of the right side of the face, re- traction of the right eyeball, narrowness of the right pupil. Pathologically a softening is found in one half of the medulla. The area affected lies between the spot where the artery begins to pass to the cerebellum and the middle of the nucleus of the hypoglossus. The formatio reticularis with its nuclei, the descending root of the fifth nerve, the nuclei of the seventh and ninth nerves, also the spino-cerebellar tract are all involved. I . COMPRESSION OF THE MEDULLA The medulla may be compressed (a) suddenly by dislocation or fracture of the atlas or axis; (b) slowly by tumors in the medulla itself or in the neighboring tissues and organs, by caries or other diseases of the 236 DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE nieghboring bony tissue, by basal meningitis and quite frequently by aneurisms of the basilar or vertebral arteries. Pathology. — The tissue of the medulla maybe torn in the sudden cases. In the slow cases the bulb will be deformed or softened. Secondary degeneration is the consequence. . Symptoms.- — In cases of gradual compression pain in the occiput and neck are the first symptoms. The head is kept by the patient in an absolute immobility. The neck is hyperaesthetic, active and passive movements are extremely painful. When the compression extends downward to the cervical cord, pain will be present also in the upper extremities. The bulbar compression will be manifested by disturbance of deglutition, of respiration, of heart beats. Death may come on suddenly. In cases of aneurism of the basilar or vertebral arteries, in addition to the occipital pain, there will be also signs of cerebral arterio- sclerosis, viz. vertigo, noise in the ears, etc. The characteristic symptom is the intermittent bulbar manifestations (dysarthria, dysphagia, dyspnea, arhythmia, tachycardia). After they have existed for a certain time, they gradually improve and then disappear until the next attack. A paralysis of the extremities (hemiplegia or paraplegia) is almost always present. A very important symptom was pointed out by Hallopeau, Giraudeau and Killian. The heads of their patients were held in forced extension; as soon as they were flexed, respiratory disturbance occurred. When compression is produced by a tumor, in addition to the above symptoms, there will be also general symptoms of cerebral tumor and optic neuritis. Course, Duration, Prognosis. — In sudden compression death is usually instantaneous from respiratory paralysis. In slow compression the patient is constantly threatened with rupture of the aneurism or softening and destruction of the tissue of the medulla. Cardiac and respiratory disorders are usually fatal. In basal meningitis of specific nature the symptoms may retrograde when under treatment. The disease may last months or years. Prognosis is grave. Diagnosis. — Compression of the medulla must be differentiated from the typical labio-glosso-laryngeal paralysis. In the latter there is no pain in the neck, no motor or sensory paralysis of the extremities. Treatment.- — Antisyphilitic treatment should be tried in all cases, of compression of the medulla, even if there is no history of syphilis. There is no medication that could arrest this exceptionally grave disease. DISEASES OF THE PONS The pons is a very important portion of the brain. It contains sensory and motor tracts going to and coming from the brain. Through the DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE 237 middle cerebellar peduncles it is connected with the cerebellum. More- over a number of cranial nerves appear at the borders of the pons on their way from their respective nuclei in the medulla. Consequently the symptomatology of pontine lesions must be complex and vary from one case to another according to the part of the pons involved. HEMORRHAGE, SOFTENING, TUMORS Pathology.- — Hemorrhagic foci are usually located in the median line. They may spread downward or forward. They are rare. Softening is quite frequent. Thrombosis of the basilar arteiy is usually found. The destruction (softening) sometimes occupies the largest part of the pons; sometimes it is on one side and sometimes on both. In aged people the disintegration of the pcntine tissue occurs in small areas, which sometimes are multiple. Tumors are not frequent. Tubercles are the most frequent. Next in frequency are gummata. Gliomata may also occur. Cysts, cancer, abscess are very rare. (For a detailed desciiption cf the effect of tumors on the nervous tissue, see Tumors of the Brain.) Etiology. — Inflammatory or degenerative conditions of the arteries will lead to their rupture and produce a hemorrhage. Softening is caused by embolism and thrombosis; the formei is exceptional, the latter is caused by a syphilitic or atheromatous degeneration of the blood vessels. In cases of tumors, except gumma and tuberculoma, there is fiequently a history of traumatism of the head. Symptoms, (a) Hemorrhage. — The onset is sudden and loss of consciousness especially in large hemorrhages is always the first symptom. In small hemorrhages there may be no loss of ccnscicusness. When the patient has regained consciousness, one will observe the following symp- toms, which are quite characteristic of a pontine hemorrhage: (a) con- tracture of the extremities accompanied by generalized convulsions, or (b) unilateral epileptiform convulsions; (c) conjugate deviation of the eyes and of the head; (d) crossed paralysis. The conjugate deviation presents special features. If the pontine lesion is destructive and leads to a unilateral paralysis, the eyes will be turned toward the paralyzed side. If it is irritative and leads to unilateral convulsions, the eyes will be turned away from the affected side. Crossed paralysis is a very frequent occurrence. It consists of a palsy of the limbs on the side opposite to the lesion and of involvement of one or several cranial nerves on the side of the lesion. Besides the palsy the symptoms will therefore depend upon the cranial nerve involved. When the lesion is low down in the pons, the seventh nerve will be 238 DISEASES OE MEDULLA, PONS AND FOURTH VENTRICLE involved; there will be paralysis of the opposite side of the body and facial palsy on the side of the lesion. When the hemorrhage is near the upper border of the pons, the sixth nerve alcne may be involved. There will be palsy of the external rectus, hence internal strabismus. Myosis, dysarthria and dysphagia are quite frequently observed in pontine hemorrhage. Death occurs from cardiac or respiratory paralysis. (b) Softening. — The onset is rarely sudden, more frequently slow. In the latter case it is preceded by prodromal symptoms, such as headache and vertigo. The attack itself is immediately preceded by paresthesia in the limbs which are to be affected. Then gradually, but progressively, the paralytic symptoms make their appearance. Crossed hemiplegia is the usual result. Dysarthria and dysphagia are quite frequent. When the scftening is due to a thrombosis of the basilar artery, there are also general symptoms besides the crossed paralysis. They are: somnolence, potic neuritis, sometimes delirium. (c) Tumors. — Tumors may originate in the pons or in the vicinity at the base, most frequently in the ponto-cerebellar angle. For the latter see page 217. Similar to cerebral turners, pontine neoplasms present general and focal symptoms. The first are : headache, vomiting, vertigo, insomnia, optic neuritis, etc. (see Tumors of Brain). The headache which is fre- quently occipital may be accompanied by stiffness of the neck. Percus- sion of the occiput and of the first cervical spinous process may be pain- ful. Optic neuritis appears late or in the terminal stage of the disease. The focal symptoms are chiefly: crossed or alternate paralysis, which is slow in development. A very frequent occurrence is subjective pain in the paralyzed muscles of the limbs and face (when the latter is involved). The paralysis may be unilateral or bilateral. The cranial nerves-symp- toms depend upon the nerve or nerves affected by the tumor. In ex- ceptional cases the neives alone are involved and there may be no paraly- sis of the extremities. There are also cases in which the nerves are not involved and there is only paralysis of the limbs. This possibility will occur only in lesions of the upper part of the pons. When trie lesion is in the middle of the pons there may be a paralysis of the limbs also of the face on the side opposite the lesion, which then lies above the decussation of the central facial fibers. If the tumor is so extensive as to involve the medulla, cerebellum or other neighboring tissue, the symptomatology will be more complex: unilateral paralysis of the palate, vocal cords, tongue, ninth, tenth, eleventh, twelfth nerves. Polyuria, disturbance of respiration and of heart beat are indications of bulbar involvement. Vertigo, tituba- tion show involvement of cerebellar peduncles. Diminution of central DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE 239 visual acuity depends upon the involvement of the anterior quadii- geminal bodies; deafness upon a lesion of the posterior quadrigeminal bodies. Sensory disturbances are present mainly in tumors of the tegmentum (see Anatomy) ; there may be either hemianaethesia or generalized anaes- thesia. Hemianassthesia is quite frequent. Not infrequently it accom- panies the hemiplegia. The disturbance of sensation is according to Marinesco due to the involvement of the mesial fillet and formatio reticularis in its ventral part (see Anatomy) . Like in hemorrhage and softening, dysphagia and dysarthria are also pres- ent and even quite frequent in tumors. Dysphagia is caused by involvment of the medulla, dysarthria — by involve- ment of the central fibers of the hypo- ^jgf _r . — ,. glossus. Anarthria implies a bilateral lesion in the pons. FlG - So.-Tubercle in the Pons. . J . . r . , Section between the Points of Emer- Ataxia is frequent in pontine lesions. GENCY 0F THE ?TH AND STH Nerves> It is usually unilateral. It is probably {Flatau, Jacobsohn and Minor.) due to the implication of the fillet which produces sensory disturbances. When the gait and station are disturbed, the cerebellum or its peduncles are probably involved. Crossed Paralysis. — From the foregoing remarks it can be seen that crossed paralysis is a common feature of diseases of the pons. This form of paralysis may be motor and sensory. There are two varieties of crossed paralysis : superior and inferior. In the superior crossed paralysis (Weber's syndrcme) there is hemi- plegia on the opposite side and palsy of the oculo-motor nerve on the side of the lesion. The hemiplegia is total, viz. face, arm and leg are in- volved. The facial paralysis has the same characteristics as in cerebral hemiplegia, viz. only the lower half of the face is paralyzed. When this form of crossed paralysis is accompanied by a tremor of the paralyzed limbs it is called: "Benedikt's syndrome." The inferior crossed paralysis (Millard-Gubler's syndrome) is characterized by a paralysis of the arm and leg on one side and of the facial nerve, also sometimes of the abducens nerve, on the opposite side. In certain cases the hypoglossus or other cranial nerves participate. When the root of the sixth nerve is involved there is paralysis of the external rectus with internal strabismus, but when its nucleus is affected, the conjugate movements of the eyes toward the lesion will 240 DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE be abolished if the lesion is destructive, but if the lesion is irritative, there will be a conjugate convulsion of the eyes toward the lesion. Other nerves may also be affected. The involvement of the fifth is not at all rare. Tactile sensation will then be affected on the side of the lesion in the area of distribution of the fifth, and in the limbs on the opposite side. The eighth nerve is rarely involved, but if it is, there will be impairment of hearing and subjective sensations of sounds on the side of the lesion. Crossed paralysis may be exclusively sensory or else sensorimotor. In the sensory form there is anaethesia of the limbs and trunk on one side and of the face on the opposite. The reason of the crossed charac- ter of sensory disturbances is because the decussation of the sensory fibers takes place in the lower part of the medulla (see Anatomy). Prognosis. — It is unfavorable, especially in tumors. Only in syphilitic cases improvement can be expected. Diagnosis. — Pontine hemorrhage will be differentiated from intra- cranial hemorrhage in general mainly by the presence of alternate paraly- sis, but also by convulsions and hyperaesthesia of the affected limbs. The conjugate deviation of the head and eyes presents a very important diagnostic point. Landouzy put down the following rules : In paralysis In convulsions. Pontine Lesion Eyes turned toward the para- lyzed side. Eyes turned toward the un- affected side. Cerebral Lesion Eyes turned toward the non-para- lyzed side. Eye turned toward the affected side. Softening is recognized by prodromal symptoms. The evolution of tumors is slow and progressive. The distinction between extra -pontine and intra -pontine tumors is of great importance, as surgical intervention depends on it. The type of extra-pontine tumors is tumor of the ponto-cerebellar angle. The chief characteristics are as follows: In tumors of the angle the manifestations of intra-cranial pressure are associated with unilateral palsy (on the side of the tumor) of cranial nerves, especially fifth, seventh and eighth. Hypoacousia and vertigo with tinnitus are often the first signs of localization. To these are fre- quently added dysarthria, dysphagia and paresis of associated movements of the eyes when the patient turns his head to the side of the tumor. Besides, there are frequently cerebellar manifestations on the side of the tumor (asynergia, etc.). The course is progressive and gradually new symptoms appear. Tumor of cerebellopontine angle are usually encapsulated and benign. DISEASES OF MEDULLA, PONS AND FOURTH VENTRICLE 241 Their detection is based chiefly on symptoms of compression of the cranial nerves at the base. A simple decompressive operation is indicated in cases of a very precarious estate of the patient, in cases of multiple tumors and in cases of cancerous metastasis. In all other cases a radical opera- tion is advisable. If the localization is made, one cerebellar fossa should be opened. Intra-pontine tumors commence often by associated progressive paralysis of the lateral movement of the eyes, predominant on the side of the lesion. It is accompanied by motor and sensory disturbances on the side opposite the tumor (crossed paralysis.) The implication of the auditory nerve is only a late symptom. Treatment. — The management is limited to the specific treatment. Surgical intervention is almost impossible on pontine tumors. Peduncular Syndrome. — The cerebral peduncles contain motor and sensory tracts connecting the brain with the medulla and spinal cord. The sensory fibers He in the upper or dorsal portion, the motor in the lower or ventral portion (see Anatomy). Besides, the superficial origin of the third nerves lie between the crura near the upper border of the pons. Hemorrhage, softening, and abscess occur very rarely. Tumors are less infrequent. They are usually tubercular. The characteristic fea- ture of a lesion of the crus will therefore be a palsy of the third nerve on the same side and hemiplegia on the opposite side. If the tegmentum is involved, the hemiplegia will be accompanied by a hemianaesthesia. This is Weber's syndrome mentioned above. If the paralyzed limbs are affected with tremor, we have Benedict's syndrome. Ptosis, external strabismus, diplopia, mydriasis — are the ocular symptoms due to third nerve palsy. If the lesion of the crura is very superficial and slight, there will be only third nerve palsy without hemiplegia. If the tumor invades both crura and both third nerves, there will be bilateral opthalmoplegia and paralysis of the four limbs. Sometimes the affected limbs may pre- sent ataxia (Krafft-Ebing, Marinesco and others). 16 CHAPTER XIX DISEASES OF THE SPINAL CORD All the affections of the spinal cord may be classified into two great groups: systemic and diffuse. In the first the lesion is confined to a certain system of fibers (tracts) or to portions of the cord which have a certain definite function (cells of anterior cornua). In the second the lesion involves gray and white matter in a diffuse manner. A. SYSTEMIC DISEASES OF THE SPINAL CORD I. Tabes Dorsalis (Locomotor Ataxia) (Posterior Sclerosis) The first intimation of the existence of the disease was given in a vague manner by Romberg in 1851, but it is mainly Duchenne that presented a full and clear description of the malady. Fig. 81. — Posterior Sclerosis. {Original.) Pathology. — The chief characteristic lesion of tabes is a gray degenera- tion of the posterior columns and of the posterior roots. In advanced cases this condition can be seen even macroscopically. In the majority of cases the disease begins in the dorso-lumbar segment of the cord. The initial lesion is in the external portion of Burdach's columns, which 242 TABES 243 corresponds to the ascending intra-spinal fibers of the posterior roots. Lissauer's tract, the fibers of which surround the end of the posterior roots, is also involved in the first stage of tabes. In the dorsal and cervical segments of the cord Goll's columns are mainly affected. In advanced cases the degeneration affects both tracts (Goll's and Burdach's) through the entire cord. The posterior roots participate in the patholog- ical process, so much so that some authors are inclined to consider them with Nageotte as the point of departure of the affection. Nageotte con- siders radicular neuritis as the primary lesion of tabes. He also calls attention to a meningitis at the level of the posterior roots, which is now proven to be a constant condition. The spinal ganglia in which the posterior roots originate have been found altered in a number of cases (atrophy of the cells and of their prolongation). The sensory nerves, the peripheral sensory pro- longations of the spinal ganglia, are frequently involved. It can be therefore seen that the following important sensory elements are af- fected in tabes: peripheral sensory nerves, spinal ganglia, posterior roots, posterior meningitis, posteiior columns in the cord. Otherwise speaking, a disease of the ascending sensory neurones constitutes the characteristic lesion of tabes. The histological changes consist of an enor- mous diminution or of a complete disappearance of the white fibers. The fibers that persist \ Fig. 82. — Tabes. Sta- are markedly atrophied because of breaking up TION . legs Widely Sepa- of the myelin and of reduction in size of the rated, Feet Everted. axis-cylinders. They are all substituted by pro- liferated neuroglia and connective tissue. The blood vessels are frequently altered: their walls are thickened (endo- and peri-arteritis). When the disease ascends to the medulla, the roots of some of the cranial nerves become involved. The optic nerve is very frequently affected (gray degeneration). The roots of the fifth and eighth nerves, and the nucleus cf the tenth nerve, have been found degenerated in some cases. Changes in the cells of ciliary ganglion have also been observed. The gray matter is also sometimes diseased. In the posterior cornua and Clarke's columns the short fibers emanating from the cells are found 244 TABES degenerated or atrophied. The cells of the anterior cornua are rarely found altered except in cases of tabes associated with muscular atrophy. The meninges at the level of the posterior roots are thickened and present a certain degree of leptomeningitis. The pathogenesis of tabes is debatable. In view of the fact that it is a disease of the sensory neurone, it is difficult at present to determine at what level of the entire sensory neurone lies the point of departure of the pathological process. According to the majority the degeneration of the posterior roots and columns follows the initial alteration of the spinal ganglia. Others believe that the posterior roots open the disease and still others are inclined to believe that the initial lesion is in the peripheral sensory nerves. Symptoms. I. Motor. — (a) The station and gait are characteristic in a well-developed case of tabes. The patient experiences a difficulty in standing: for fear of falling he separates his legs widely and even then his body oscillates. When an attempt is made by him to close his eyes and bring his feet close together, there is a tendency to fall. The latter test is called "Romberg's sign." In walking there is an uncertainty, an incoordination of movements of the legs, there is ataxia. The patient lifts his legs high, feet everted, then throws them forward and laterally, and being unable to control the movements, he drops them on the ground with force. This ataxic gait is sometimes so pronounced that the patient is unable to make a few steps without falling. In initial stages of tabes the ataxia may be extremely slight. The patient notices then that he is unsteady in going up or down stairs or in walking in a dark place. When the incoordination is not marked in the gait, the ataxia may be revealed by making the patient to start to walk suddenly, stop suddenly or turn abruptly. Also can it be detected in a recumbent position: when he is told to raise the leg and touch with his foot your hand, this act will be accomplished after several lateral oscillations of the leg (Fig. 82). When the upper extremities are affected, there is a difficulty in per- forming fine or delicate acts, as writing, threading a needle, etc. When the patient is asked to touch the tip of his nose with the tip of his index, the latter will make several movements above and below or laterally before the nose will be reached. Another test for ataxia of the upper extremities is to bring the tips of the two indexes together after the arms have been widely separated. Ataxia in tabes is due to loss of sense of position in the muscles and articulations. (b) Hypotonia of the Muscles. — There is an unusual flaccidity and relaxation of the muscles of the affected limbs, so that the latter can be easily given any position. The entire limb, for example, can be extended TABES 245 and raised, so that it is brought in contact with the trunk. The knee- joint may be overextended and produce a genu recurvatum. In the upper extremities the fingers can be so extended as to form a right angle with the dorsum of the hand. The hypotonia may also affect the muscles of the trunk, so that exaggerated movements (passive or active) can be produced. II. Sensory. — They are subjective and objective. Tabetic pain is very characteristic and one of the earliest subjective symptoms. It is usually lancinating, knife-like, very sharp and brief. Sometimes it is burning or boring, associated with numbness or tingling. In the majority of cases it is paroxysmal and may last from a fraction of a minute to an hour. The attacks may be frequent or appear only at rare intervals. Between the paroxysms the skin may remain hypersensitive. The pain occurs usually in the lower extremities and ulnar side of the arms. It frequently in- vades the viscera and constitutes then a characteristic symptom, called "crisis." In gastric crisis the pain is in the hypochondrium and an attack may be followed by vomiting. There are also: vesical, renal, testicular, laryngeal and anal crises. Instead of being intermittent, the pain may remain permanent and fixed. This occurs in the so-called "girdle pain," which consists of a sense of constriction around the waist or chest. The phenomenon is frequent and typical of tabes. Among other subjective sensations there may be instead of or besides pain also various paraesthesiae, as numbness, tingling, coldness, burning, etc. They may affect any portion of the body, but more frequently the lower extremities. Loss of position of the limbs is present in a large number of cases. The patient is unable to tell the position of his limbs (lower most fre- quently) while in recumbent position. It can be elicited by closing his eyes and placing one leg over the other or by flexing and extending a portion of a limb. The objective sensory disturbances are: anaesthesia and analgesia. The loss of sense of pain and touch is met with in tabes over small areas. In the upper extremities it is usually on the ulnar side of the forearms. In the lower extremities it is usually on the soles of the feet: it is quite common for tabetics to have the impression of walking on cotton or on a carpet. The loss of pain-sense may not be confined to the skin; it may be present also in the deeper tissues, as muscles, bones, joints. Painless fractures or dislocations are not uncommon. Vibration sense (see page 69) is also lost. The following visceral analgesias are not infrequently observed in tabes: testicular, epigastric, mammary, tracheal, ocular; pressure on all these organs provokes no pain. Pressure 246 TABES on the ulnar nerve (Biernacki) and on the external popliteal nerve (Bechterew) causes no pain. Abade called attention to analgesia observed when the tendon Achilles is pinched. The loss of profound sensibility is sometimes a very early sign of tabes. In exceptional cases there may be hyperalgesia instead of analgesia. In such cases the slightest touch will cause unbearable pain. The temperature sense may also be altered to the same extent as touch and pain. In some cases there may be a perverted, sense, as for example a prick with a needle will give the sense of burning. Finally there are cases in which the localization of sensations is disturbed, as for example the touch or pin prick will not be felt by the patient on the spot touched, but on a remote part of the body. III. Special Senses, (a) Vision. — Among the earliest ocular signs in tabes is the Argyll-Robertson pupil. It may precede the ataxia ten or fifteen years. It is characterized by a sluggishness or loss of light reflex and preservation of the accommodation reflex. It means that when a light is thrown into the pupil, the latter remains immobile instead of contract- ing. This sign may be bilateral as well as unilateral. In advanced cases the accommodation reflex may also be abolished. In some cases Argyll- Robertson pupil may be only transitory, disappear and reappear. Erb called attention to the following phenomenon. Normally when the skin is pinched, the pupils contract; in early tabes this reflex disappears. Tabetic pupils are quite characteristic. In the majority of cases they are very small (myosis) . In some cases they may on the contrary be much dilated. They may also be irregular in contour and unequal: one myotic, the other mydriatic. Paralysis of the ocular muscles is frequent. It presents these peculi- arities, that it does not affect all the muscles equally and that it is transient in the early stages of tabes. Ptosis is very frequent, internal or ex- ternal strabismus is also quite common. The patient often complains of diplopia. The latter is a very early sign. These palsies disappear and reappear until the disease is well developed, when the ocular paralysis becomes permanent. As to the visual function, there is almost always a diminution of visual acuity. The loss of vision may be acute or more frequently chronic. Ophthalmoscopic examination reveals an atrophy of the optic nerve (gray atrophy). Optic atrophy and Argyll- Robertson pupil are early and certain signs of tabes, even when the other symptoms are hardly notice- able. In some cases the onset of blindness arrests the evolution of the TABES 247 other tabetic symptoms. Contraction of the visual fields especially for colors is not rare. (b) Audition. — When the trigeminal area becomes involved (see Pathology), the result will be a diminution of the acuity of hearing which may go to complete deafness, also various noises in the ears and finally vertigo analogous to that caused by ear diseases. (c) The olfactory and gustatory senses are occasionally found to be disturbed. IV. Reflexes. — Loss of patellar tendon reflex (Westphal's sign) is one of the earliest symptoms of tabes. At first the knee-jerks may be only diminished, but later they disappear entirely. The loss or diminu- tion may be unilateral, but more frequently bilateral. In doubtful cases it is necessary to have recourse to the method of reinforcement (Jen- drassik): the patient is told to place one hand in the other and to pull; a short blow over the patellar tendon will then enable more readily to determine whether the knee-jerk is present or absent. The patellar tendon reflex persists in the "superior tabes" (see Pathology). Loss of Achilles tendon reflex is very frequent in tabes. Its disappearance may occur long before that of the knee-jerk. The explanation of the abolition of the tendon reflexes is found in the breaking up of the reflex arc, the sensory portion of which is diseased. The cremasteric and anal are very frequently involved: they are dimin- ished or lost. V. Sphincters. — Disturbances of the function of the sphincter of the "bladder appears early in the course of tabes. Difficulty of expelling the urine is common. Some patients do not feel the necessity of urinating. In some cases there is on the contrary marked frequency and imperative micturition. In the advanced cases incontinence is the usual occurrence. The sphincter of the rectum is also commonly involved. Constipa- tion, difficulty of defecation, tenesmus, incontinence — all occur in tabes. VI. Trophic Disturbances.— They affect the general nutrition and individual organs or tissues. The majority of tabetics are usually pale, emaciated, with drawn features, sunken eyes. Among local dystrophies the arthropathies occupy the first place. They were first described by Charcot in 1868. They may be of atrophic and hypertrophic types. The first consists of a more or less complete destruction of the cartilage and of the bony extremities of the joint; the capsule is in a state of relaxation. Extreme mobility of the joint is the consequence. Luxation or subluxation of the bones occur then easily. Quite frequently the synovial membrane, which is either extremely thin or thick, contains an abundant serous fluid. In the hypertrophic variety 248 TABES there is formation of new bony tissue, of bony excrescences in the joint. The principal characteristics of tabetic arthropathies are: absolute absence of pain and a special oedema, which upon pressure does not leave a depression. As to the painless joint, it is so remarkable that patients thus affected will walk with dislocated joints without the least suffering. The seat of the arthropathy is mostly in the knee-joint; next in frequency is the foot, then the hip, shoulder, elbow and inferior maxillary bone. The "tabetic foot" deserves special mention. It presents a Fig. 83. — Bilateral Arthropathy of the Knee in Tabes. {Glorieux and Van Gehuchten.) thickening of the internal border of the foot and pathologically consists of an involvement of the ligaments, bones and articulations. The patient walks on the inner side of the foot, which is everted. The bones also suffer in tabes. There is rarefication of the osseous tissue. Spontaneous fractures take place with great facility: a very slight traumatism is likely to produce them. They are also painless. The formation of a callus is usually normal. Among other trophic disturbances should be mentioned: "perforating ulcer" (mal perforant). It consists of a painless ulceration on the plantar surface of the foot and especially at the level of the great and little toes. TABES 249 Muscular atrophy not infrequently occurs in the lower extremities, but also occasionally in the upper. Hemiatrophy of the tongue had been observed. Cutaneous disturbances, as herpes, gangrene, zona, hyper- hidrosis, falling of the hair and of the teeth, brittleness of the nails, oc- casionally develop in the course of tabes. Vasomotor disturbances are frequently observed. They are: coldness of the extremities, anemia or blanching of the extremity of a limb ("dead finger"), oedema of the extremities. There are at present two views concerning the pathogenesis of the trophic disturbances. According to some neurologists the latter are the result of changes in the cord, namely in its anterior cornua. Others believe in a peripheral origin, viz. in a neuritis of the nervous filaments distributed in the bones, articulations, etc. As to the arthropathies, carefully collected records show a history of trauma as an exciting cause, (see my contribution in Med. Record, 1909). VII. Visceral Disturbances, (a) Gastro-intestinal Canal. — Apart from "gastric crises," mentioned above in connection with sensory disturbances, tabetics not infrequently present digestive disturbances. Fournier called attention to "tabetic anorexia," in which the patient loses the sensation of hunger. The intestinal trouble consists of tenesmus, of imperative or frequent defecation. Diarrhoea is quite frequent in the preataxic period. (b) Larynx. — Laryngeal disturbances may consist of dyspnoea, parox- ysms of cough and "laryngeal crises." The pathogenesis of the latter is obscure. However most frequently a paralysis of the dilators of the glottis is found. The cause of the laryngeal disturbances lies probably in the involvement of the ninth, tenth and eleventh nerves. Among the rare symptoms may be mentioned the syndrome of Avelis, viz. hemiatrophy of the velum palatinum associated with paralysis of the recurrent nerve. It is particularly encountered in tabes of the upper portion of the cord. The paralysis of the internal branch of the eleventh nerve which produces the hemiatrophy of the palate is probably due to a meningeal involvement of the root fibers of that nerve. (c) Genital Apparatus. — It is frequently disturbed. Sexual desire with orgasm is increased at the beginning of tabes. As the disease pro- gresses, impotence gradually makes its appearance. Sterility is fre- quent in women. (d) Blood Vessels. — Atheromatous changes are present in many cases. This is probably the cause of paroxysms of "angina pectoris" occasionally observed in tabes. (e) Brain. — Mental disturbances are sometimes observed, especially 250 TABES toward the end of the malady. It is mainly cerebral depression, but may be also cerebral exaltation. Kraepelin speaks of hallucinatory psychoses occurring in the course of tabes, but he does not affirm that they are directly and exclusively conditioned by tabes. The amaurotic form of tabes is particularly associated with some mental manifestations such as delirium, hallucinations. Apoplectiform seizures may occur. The cere- bral symptoms occur probably in those forms of tabes which terminate in paresis. The relation of both diseases is remarkable. There are tabe- tics in whom symptoms of paresis develop and paretics in whom symp- toms of tabes are present. The involvement of the posterior columns in paresis is almost constant. Claude (Encephale, 10 Dec, 191 2) has recently brought forward anatomical facts showing the existence of syphilitic meningo-encephalitis in tabes, viz. sclerotic and gummatous nodules, old adhesions of the dura, sclerotic thickening of the pia, foci of hemorrhagic softening with periph- eral inflammatory reactions, foci of cedematous encephalitis. During life the patient presented a delirious state, confusion, disorientation. It is evidently a case of tabes with localized syphilis of the central nervous system, but not typical of paresis. Course, Duration and Termination, Prognosis. — All the cases of tabes are not identical to each other. Generally speaking, three periods are observed in the course of the disease : preataxic, ataxic and the terminal. This division is by no means present in every case. There are case s which are ataxic from the very onset. It is impossible to foretell in any given case the mode of the evolution of the symptoms. There are also mild and aggravated forms. In certain cases the symptoms reach a certain degree of development and remain stationary. Others progress very rapidly. In the "amaurotic form" the blindness and optic atrophy may be the only symptoms for years before other signs of tabes make their ap- pearance. The course may be extremely slow and last even thirty years, although on an average the disease lasts ten or fifteen years. The sphincter dis- turbances hasten the disease. Death is the usual termination. Tabetics usually die in cachexia, but also from some intercurrent disease (pneu- monia, tuberculosis). Sometimes infection from cystitis or pyelonephritis or else from a bedsore is likely to shorten the patient's life. The prognosis is therefore grave. Return of the patellar tendon reflexes in the course of tabes deserves some comment. After having been abolished for some time, the knee- jerk may reappear. This may happen either after an attack of hemiplegia, after the development of amaurosis, finally after a rigorous antisyphilitic TABES 251 treatment. The mechanism by which the return of the reflex is done is not entirely clear. At all events, that the reflex may return, it is necessary that the anatomical pathways are not completely destroyed, that the loss of the reflex is not absolute. Diagnosis. — The described seven cardinal symptoms are sufficient for making a diagnosis of tabes. The eye manifestations require special emphasis. The ocular symptoms enumerated above may be encountered in syphilis and therefore amenable to treatment; consequently the prognosis is more favorable. A few differential points are necessary. If ocular symptoms in tabes present marked fluctuation, they are due to syphilis itself and therefore improvement may be expected from treat- ment. If they are due to tabes, they are beyond reach. Myosis and reflex immobility of the pupil or dilatation of pupil with paralysis of accommodation apparatus are more likely signs of syphilis, hence amenable to treatment. Simple atrophy of optic nerves is very frequent in tabes, exceptional in syphilis. In tabes the papilla is pale from first; in syphilis the pallor comes on gradually after visual disturbances have developed. Benefit from specific treatment will also enable cne to differen- tiate these disturbances. Syphilitic iritis or perforation of palate are never encountered in tabes. Reflex immobility of pupils is one of the earliest and mcst frequent symptoms of tabes. It may precede ataxia for a number of years. Recognition of incipient tabes is of utmost importance. The following manifestations are to be borne in mind. Some disorder in the function of the bladder, such as delay in micturition, or slight incontinence after drinking; pain suggestive of rheumatism, neuralgia of the trigeminal and intercostal nerves; paresthesia in the perineal and scrotal regions; the ocular disturbances;, absence of Achilles tendon reflex; relaxation of muscles — hypotonia especially of the knees; tendency to ataxia which can be detected after a careful examination of individual muscles of segments of the limbs; lack of perception of vibration of the tuning fork when applied to the tibia; gastric crises — consisting at first of retching, salivation and hiccough. In spite of the facility with which a diag- nosis of tabes can be made, in some cases difficulties may be en- countered. The following are the affections from which tabes should be differentiated. Multiple neuritis presents in common with tabes pain, loss of knee- jerks, incoordination, Romberg's sign, but the symptoms absent in tabes are: tenderness of the nerve- trunks and sensory disturbances, which follow the distribution of the nerves. Finally Argyll-Robertson pupil is never present in neuritis. 252 TABES Multiple sclerosis can be recognized by nystagmus, intention tremor and characteristic staccato speech. Friedreich's ataxia presents like tabes loss of knee-jerks, but it is differentiated by the existence of nystagmus and special speech and by absence of pain. Cerebellar diseases (tumor, hemorrhage, etc.) simulate sometimes tabes, but the difficulty in walking is net like a tabetic ataxia, but a tituba- tion. Moreover cerebellar asynergia does not exist in tabes. Finally absence of pain and cf visceral disturbances are in favor of a cerebellar disease. Recent researches have demonstrated that in the course of tabes a lymphocytosis takes place in the cerebro-spinal fluid of the majority of cases. Wasserman reaction in the cerebro-spinal fluid is almost invariably positive in untreated cases. These two factors are certainly a valuable addition to the semeiology of tabes in doubtful cases. Etiology. — Syphilis plays the most important if not the exclusive role in the genesis of tabes. According to Fournier, 93 per cent, of tabetics present a clear history of syphilis. Erb and Oppenheim observed patients personally free from specific infection, but whose parents had had syphilis. With the discovery of Wasserman reaction the syphilitic origin of tabes is finally settled. The posterior sclerosis is produced not directly by syphilis, as the disease develops many years after the initial syphilitic lesion. Tabes belongs therefore to Founder's "parasyphilitic affections." As to other causes mentioned by some writers, they are: cold, exertion, excesses and traumatism. In my opinion they may be considered only as predisposing factors. The age at which tabes occurs is usually between thirty and forty, although juvenile tabes is not unknown. I have seen as young as eighteen and as old as sixty-five. Tabes is infrequent in women. It is rare in negroes. Treatment. — In view of the syphilitic etiology of tabes, mercury and iodides have been recommended, but it is doubtful if these drugs are capable of altering a sclerosis of the posterior columns. However as some favorable results have been obtained by competent neurologists, it is advisable to apply this treatment in every case of tabes. Mercurials should be given first. Inunctions or hypodermic injec- tions are preferable to internal administration. Precautions must be taken against mercurial intoxication. The patient should be instructed to observe himself closely. In case marked salivation, swelling or sore- ness of the gums and of the tongue, pain in the abdomen, diarrhoea, ele- TABES 253 vation of temperature are noticed, mercury should be discontinued with- out delay. In one case under my care the patient failed to report to me in spite of my urgent warning and kept on using the mercurial inunctions; only a few doses brought on such an enormous swelling of the tongue that a tracheotomy had to be performed for relief from threatening suffo- cation. In order to obtain the full effect of mercury, it should be admin- istered as long as there are no - signs of intolerance. As to the daily dose of mercury, it is advisable to begin with a small quantity, say a half of a dram, twice a day (in an adult). At the end of three or four days the dose can be increased to a dram, if there is complete tolerance. After a sufficiently long trial (say two or three weeks) mercury should be substituted by iodides. Sodium iodide gives the same therapeutic re- , suits as potassium iodide and has this advantage over the latter, that it is less apt to disturb the stomach. Its initial dose should also be small, say 10 minims of a saturated solution three times a day. At first the dose should be increased five minims every three days until the dose of 50 minims is reached. Then the increase should be very slow: about one or two minims every other day. The drug can be continued indefi- nitely until intolerance shows itself. The latter is manifested by loss of appetite, digestive disturbances (pain, eructation) and diarrhoea. The question of intolerance is a relative one. I have had patients who have been unable to take more than five minims t. i. d. and patients that could take 500 minims t. i. d. Arsenical preparations have been recently brought forward. The newly discovered Salvarsan by Ehrlich and Hatta, although a very valu- able drug in primary and secondary stages of syphilis is practically valueless in tabes. It may in some cases improve the general nutri- tion but it has very little or no effect upon the course of the disease. This subject will be discussed fully in chapter on Cerebro-spinal syphilis. Cacodylate of sodium, another arsenical preparation, administered hypo- dermically has also a tendency to improve the patient's general health, but it has no effect on tabes itself. Among other drugs used in treatment of tabes can be mentioned: chloride of gold, nitrate of silver and ergot. The latter has been recom- mended by Charcot to combat the genito-urinary disorder. The special symptoms of which the tabetics complain can be combated by usual medicaments. But there is one manifestation which requires special at- tention. This is " gastric crises." Because of the intensity of the pain, of frequent vomiting and the asthenia which the latter produces, gastric crises are the most distressing manifestations of tabes. The crisis is the result of motor and secretory reflex phenomena 254 TABES secondary to a hyperesthesia of the gastric mucosa. The latter is con- trolled by the pneumogastric and especially by the sympathetic. These two nerves send filaments to the major splanchnic through the solar plexus and from there through the rami communicantes they reach the posterior roots of the sixth, seventh, eighth and ninth dorsal pairs. To decrease the irritability of these nerves local applications have been ad- vised, such as ice, cauterization, spray of ethyl chloride over the region of the stomach ; internally chloroform, opium, etc. They all may occasionally give temporary relief. Surgical procedures have been more successful. Lumbar puncture with evacuation of 10 or 30 c.c. gave in some cases considerable relief, but still better results were obtained when the puncture was followed by some anesthetizing fluid such as cocain, eucain, novocain, stovain in the amount of 1 c.c. of 1 : 10 solution. Epidural injections have given similar results to Konig and others. None of these fluids could be considered absolutely safe, as accidents, such as paralyses or respiratory disturbances have been observed. Besides, not in all cases improvement has been noticed. A more satisfactory drug for intraspinal injection is magnesium sulphate. Its dose is 1 c.c. to each 25 pounds of body weight; jthe solu- tion is 25 per cent. With this drug fewer accidents and more frequent improvements have been observed. Recently operations of Foerster and Franke came to the front. Both have met with successes and failures. Both have for object to interrupt the sympathetic pathway of the gastric sensibility. Foerster attacks it at the level of the posterior roots of the sixth, seventh, eighth, ninth and tenth thoracic nerves. He cuts these roots on both sides. The opera- tion is a complex one and the mortality is quite high. However, excellent results have been obtained in some cases. Recurrences of pain have been also observed. The operation of Franke is decidedly less complex and attended by a lesser mortality, but the success is not as frequent as in Foerster's operation. Franke attacks the sympathetic pathway by tearing out the central ends of the intercostal nerves corresponding to the roots utilized in Foerster's operation. Both operations gave favorable and unfavor- able results. As the latter is considerably less serious than the first, it may be tried at first, and when no results, Foerster's operation may be undertaken. Sicard (Presse Medicale, 191 2) has recently devised a new procedure which seemingly gave him very satisfactory results. He advises to pene- trate after a laminectomy into the epidural space and without opening the dura tear out the spinal ganglia. It is therefore an extradural TABES 255 ganglionectomy. It has the advantage of avoiding an injury to the dura and consequently there is no loss of cerebro-spinal fluid. The number of spinal ganglia to be removed on both sides as well as the number of roots to be taken into consideration for the operation are to be determined according to the seat of the painful phenomena. Finally injections of alcohol or stovain into the point of emergency of the intercostal nerves (Koenig) have been tried. Exsner (Wiener klin. Wchn., 191 2) suggested a method of treating gastric crises of tabes by severing the vagus nerve on both sides. He found the nerve frequently diseased. Stretching the solar plexus has also been recommended and favorable results have been reported by some surgeons. (Audibert. These de Lyon, 1912.) External Treatment of tabes has been applied in various forms, but the majority of them present only a historical interest. Suspension, for example, or forced flexion of the spinal column, also elongation of nerves, were imagined with the object of improving ataxia and of reliev- ing pain. Massage is a good procedure for keeping up the nutrition of the muscles; so it is with electricity. Among all the mechanical means the "systematic exercises" of the ataxic limbs have proved to be most valuable. Frenkel, of Hayden, made a special study of them and brought them up to a regular method based upon the exact knowledge of physio- logic functions of various muscles. It consists of slow exercises of vari- ous segments of the limbs. The patient is taught to sit down, get up, bring his limbs together, raise one limb at the time, walk in a certain direc- tion, etc. By this procedure, kept up persistently for weeks and months, patients learn how to use their limbs and may become so skillful that they may dispense with their canes or crutches. In treatment of tabes general hygienic measures should not be neg- lected. Stimulants should be avoided. Good nutritious food and fresh air are indispensable. Hydrotherapy in the form of a Scotch douche or baths is a good adjuvant to other procedures. Special care should be taken of the sphincters. In case of incontinence of urine the patient must be provided with a special urinal. In case of retention catheteriza- tion is necessary. In case of cystitis the bladder should be washed out with some mild antiseptic, such as boric acid, and urotropin adminis- tered internally. Bed-sores must be taken special care of. Infection may originate from this particular source as well as from the bladder, and hasten death. Fatigue must be avoided, although mild outdoor exercises are advisable. Anorexia or other dyspeptic symptoms must be promptly remedied, 256 SPASTIC PARAPLEGIA as it is very important to have the patient to assimilate abundant and nutritious food. In recent years treatment with X-rays has been tried. Favorable results are being reported. It is claimed that the pain in the limbs and crises are particularly benefited. Incoordination may also improve. The exposure to the rays should last fifteen minutes and made at the level of the dorso-lumbar region. Twenty or thirty seances are necessary to obtain improvement. Juvenile Tabes. — Tabes has been observed in very young individuals and even in children of nine years of age. Hereditary syphilis can always be traced in the family history. Wassermann reaction is positive in a much larger proportion than in adults. Atrophy of the optic nerve is according to Barkan (Wien. klin. wchn., March, 1913) found in 80 per cent, of all the cases on record. The Romberg sign and ataxia are less frequent in children. Mercury does not arrest the progress of tabes. Only one case of juvenile tabes is on record with necropsy. II. Spastic Paraplegia (Primary Lateral Sclerosis) Pathology. — It consists of a primary degeneration and sclerosis of the pyramidal bundles in the cord. In the majority of cases recorded the lesion overstepped the boundary and some changes were found also in GolPs columns, occasionally also in the direct cerebellar tract. In a very few cases the lesion was strictly confined to the lateral columns. In my case (New York M. J., 191 2) only the pyramidal tract was involved; the lesion extended to the medulla at the level of decussation. The disease is rare. Etiology. — Except the family form of spastic paralysis, which will soon be described, nothing definite is known in regard to the causes of the affection. As the disease is very rare, very few cases are on record. The disease probably belongs to the category of congenital abnormalities of development of the central nervous system. Syphilis may produce the picture of lateral sclerosis. Symptoms. — The disease is- characterized by a slow development of spasticity with loss of power in the lower extremities. The gait gradually becomes more and more difficult and the patient is unable to raise his limbs off the ground: his feet scrape the floor. Upon passive movements great resistance is felt. The limbs are rigid. The abnormal reflexes which are usually found whenever the motor tract is involved (see hemi- plegia) are all present here, viz. increased knee-jerks and Achilles' tendon reflex, ankle-clonus, Babinski's phenomenon, Oppenheim's and paradox- ical reflexes. SPASTIC PARAPLEGIA 257 The sensations, the state of the bladder and rectum, the consistency and electrical reactions of the affected muscles are all normal. Family Spastic Paralysis. — The spastic paraplegia just described may sometimes be found in several members of the same family. Strumpell was the first to call attention to this occurrence. In the majority of cases the male members are more frequently affected than the female. The disease usually appears between fifteen and thirty years of age. In one family that came under my observation there were seven members affected with spastic paralysis: four of them began to show the spasticity at the age of three; six of them were males and one a female. In families thus affected there is usually present some degenerative basis; either insanities, various neuroses or alcoholism, consanguinity, syphilis. The symptoms Fig. 84. Lateral Sclerosis. (Original.) are the same as described above. The feet are frequently deformed and may present all forms of deformation, most frequently equinovarus. The lower extremities are mainly involved. The upper ones are rarely affected. In some cases this type of the disease remains unchanged until the end of the patient's life, but in the majority of cases, years after the onset, new symptoms develop and usually the symptomatology of multiple sclerosis becomes apparent. The involvement of upper extremities, the staccato speech, the intention tremor, nystagmus, optic atrophy — all are typical of the latter affection. It is interesting to note that the intelligence is invariably intact. Closely allied to family spastic paralysis stands Little's disease, which 17 258 SPASTIC PARAPLEGIA is a cerebro-spinal involvement of the pyramidal tracts, but the cerebral symptoms which are usually present from the very beginning make it a disease apart; they are: speech disturbances, strabismus, athetcid move- ments, epilepsy (see Cerebral Palsies of Childhood). Prognosis. — In the pure type of spastic paraplegia (which is rare) and in the family type the prognosis is unfavorable. The disease is pro- gressive. Complete paralysis with marked contractures is inevitable. Death is rarely the result of the malady itself, but mostly of some inter- current disease, as infection or tuberculosis. Diagnosis. — As spastic paralysis may be the ultimate consequence of myelitis, of amyotrophic lateral sclerosis, compression of .the cord, syringomyelia, Friedreich's disease, multiple sclerosis, the diagnosis of spastic paraplegia as a primary affection should be made very cautiously. The disease, as said above, is very rare and consequently can be diagnosed only after the special symptoms of the other diseases have been eliminated. The occurrence of the spastic paralysis in several members of the same family is the most important characteristic feature of the disease. Treatment. — It is the same as in Little's disease. See methods of Foerster, Schwab and Allison; also of S toff el (pages 122 and 123). PARAPLEGIA OF THE AGED Paraplegia of the aged has its orgin in vascular sclerosis in the brain or in the spinal cord producing a general disturbance in circulation and nutrition of the central nervous system. Paraplegia of cerebral origin is caused by "lacunar softening," a con- dition so frequently met with in senile brains (Marie, Ferrand and others) . It consists of perivascular degeneration of brain tissue (see page 83) in the basal ganglia irregularly distributed, and of a descending secondary degeneration in the pyramidal tracts. The posterior columns of the cord are also irregularly degenerated. The symptoms begin insidiously with- out apoplectic features. They consist of a spastic feebleness in the lower extremities (the upper limbs are but very slightly involved) which gradually develops into a total spastic paraplegia with or without sphincter involvement. Finally dementia, bed-sores and dysarthria make their appearance. There is usually an inequality of the paretic phenomena on both sides. The contractures, if present, are never so marked and constant as in the following type. In Paraplegia of spinal origin there is a disseminated and diffuse sclerosis in the posterior columns but especially in the lateral columns. The sclerosis is irregularly distributed at different levels of the cord. Clinically the paretic condition is symmetrically distributed in both ATAXIC PARAPLEGIA 259 lower limbs. The onset is, like in the first variety, insidious. The evo- lution of the symptoms is longer. There is frequently pain which is continuous and occasionally paroxysmal. The sense of position is fre- quently perverted. When the patient walks, his legs are widely sepa- rated. There is an uncertainty in movements and sometimes the legs suddenly give way. The tendon reflexes are exaggerated. The spasticity gradually increases; contractures in flexion-position develop. Finally ap- pear sphincter disturbances, general emaciation and tendon retraction. Bed-sores may precipitate the fatal termination. The preservation of the mental faculties is a distinguishing feature. The latter together with the slow course and the symmetrical distribution of the spastic and paralytic phenomena will enable one to differentiate it from the cerebral form. III. Ataxic Paraplegia (Postero-Lateral or Combined Sclerosis) Pathology. — The lesion consists of a degeneration and sclerosis of the posterior and lateral columns. In the posterior portion of the cord GolPs columns are mainly affected. In the lateral portion of the cord the crossed Fig. 85. — Combined Sclerosis. {Original.) pyramidal tract is chiefly and always involved, but quite frequently the direct cerebellar tract and occasionally Gowers' bundle are also diseased. The neuroglia tissue is proliferated. Changes are also observed in the cells of Clarke's columns. Symptoms. — From the pathological condition it can be seen that the symptoms of tabes and spastic paraplegia should be expected. When the posterior columns are more and earlier involved than the lateral, the 260 ATAXIC PARAPLEGIA predominant symptoms will be those of tabes. In case the crossed pyramidal tract is mainly affected, the chief symptoms will be those of spastic paraplegia. In the majority of cases the tabetic symptoms are few in number and the following are the manifestations of the disease: ataxia, Romberg's sign, spasticity and weakness of the lower extremities, spastic gait, increased knee-jerks, ankle-clonus, Babinski's sign, Oppen- heim's and the paradoxical reflexes. The sensations, if they are altered, present some objective changes. At first there is a slight diminution of sensations to all forms. Later in a fully developed case a more or less complete loss of sensations is observed. The anaesthesia is confined to the lower extremities and may reach the abdomen. Paraesthesia in the lower limbs, such as numbness, tingling and others, are an early symptom. They may persist during the entire course of the affection. The main tabetic symptoms : pain, bladder disturbance and eye changes are extremely rare. Loss of control of the bladder sphincter occurs only in the terminal period when bed-sores and poor general health indicate the approaching end. In Anemia or Pernicious Anemia the clinical picture is somewhat different from the usual type of ataxic paraplegia. Sensory disturbances appear at the beginning; they consist of paresthesia, diminished sensations and sometimes lancinating pains. In my case (New York M. J., 1909) there was a distinct syringomyelic sensory dissociation. Muscular weakness and paresis affect the upper extremities as well as the lower. The ataxia is more of a cerebellar type (see Cerebellum) than of a tabetic character. In pernicious anemia degeneration is found not only in the posterior and lateral columns, but also in other portions of the cord. In my case (loc. cit.) the condition was diffuse and involved also Gower's tract. In chronic intoxication with Ergot and in Pellagra degeneration of the posterior and lateral columns sometimes occurs. In ergotism — the symptoms are more of tabetic nature, while in pellagra more of the type of spastic paraplegia. Course, Prognosis.- — The outlook is unfavorable. The disease runs a rapid course: from a few months to two years. Diagnosis. — When the tabetic symptoms are marked, the disease is differentiated from true tabes- by the absence of the chief symptoms of the latter (Argyll-Robertson's pupil, optic atrophy, bladder disturb- ances, etc.) and by the presence of spasticity with the abnormal reflexes mentioned above. From Friedreich's disease, which is also a postero-lateral sclerosis, it will be differentiated by the absence of the chief symptoms of the first, Friedreich's ataxia 261 viz. nystagmus, disturbed speech, choreiform movements, early onset and family character of the disease. Multiple sclerosis will be recognized by intention tremor, staccato speech, nystagmus. Transverse myelitis in its chronic form assumes frequently the form of spastic or ataxic paraplegia, but in the former the sensory and trophic disturbances, sphincter involvement and the evolution of the symptoms will easily reveal the true nature of the disease. Etiology. — Syphilis may be the cause. The age at which the disease occurs is between twenty and forty. In the course of anemia, pernicious anemia, pellagra, ataxic paraplegia is sometimes observed. As the blood vessels are frequently found intact, the toxic element which is probably at work in pernicious anemia follows the route of the nerve fibers in the cord and produces a degeneration. In the absence of the above causes, a congenital weakness of the sensory and motor neurones of the cord is probably the true nature of the disease. Treatment. — Antisyphilitic drugs (mercury and iodides, also salvarsan) should be always tried. In cases of anemia, appropriate remedies (iron, arsenic) should be administered. As to the ataxia and spasticity, they will be relieved by the same means as advised in tabes and spastic paraplegia. IV. Friedreich's Disease (Hereditary Ataxia) This affection was described first by Friedreich in 1861 as a peculiar variety of tabes occurring in childhood and having a hereditary character. Pathology. — The spinal cord is diminished in size and very slender. The changes affect the white and the gray matter, but it is the first that is particularly affected. The most pronounced degeneration is in Goll's columns, which are involved through the entire cord. Burdach's tract is affected mainly in the lumbar region and only a small portion of it is sclerosed. Direct cerebellar and Gowers' tracts are invariably involved. The majority of observers believe that the crossed pyramidal bundle is also diseased, but considerably less than the posterior columns, and the degeneration commences later. The affection is therefore essentially a combined sclerosis. As to the gray matter, the cells of the posterior cornua are diminished in number and size. Clarke's columns present the most changes: the cells are atrophied and the number of fibers is considerably reduced. The neuroglia tissue which takes the place of the normal substance is prolifer- ated and more developed in the dorsal than in the lateral columns. The meninges are sometimes found thickened at the level of the posterior columns. The dorsal roots are also in a state of sclerosis. 262 Friedreich's ataxia Usually the changes just described are confined to the cord, but in a few cases the medulla and cerebellum were found also altered. The involvement of the latter organ led Marie to describe a separate type under the name of heredo-cerebellar ataxia (see the latter). The periph- eral nerves have also been found atrophic or degenerated. Etiology. — The fundamental characteristics of Friedreich's disease from the etiological standpoint is that it occurs in several members of the same family. The symptoms begin to appear before the age of fourteen. Edinger has proposed the following explanation (Ersatz-Theorie). When portions of the nervous system are not developed or not resistant, over- work may lead to their degeneration. Thus the beginning of the symp- toms at age of puberty in Friedreich's disease finds its reason. Cerebral diseases have been observed in relatives. Syphilis, alcohol, do not play any special role. Males are somewhat more frequently affected than females. Symptoms. Motor. — The first symptom to appear is disturbance of gait. Slight in the beginning, it gradually becomes more and more marked. The patient walks with his legs widely separated, and after he raises them he drops them heavily on the ground. At the same time the entire body oscillates for fear of falling. This ataxia is net tabetic, but of cere- bellar type. The station is also ataxic. While Romberg's sign is not present, nevertheless the patient's body oscillates to and fro, his feet change position very often, when he is told to stand still. The upper extremities are also affected, but here the ataxia appears later, long after the legs become affected. Choreiform movements are frequent: the gesticulations are present not only in the arms, but also in the head and trunk. As the patient sits unsupported, nodding movements of the head and swaying of the trunk are seen. Athetoid movements may also occur. The limbs are very weak and as a rule flaccid but there are no paralytic symptoms. Intention tremor is quite frequent. The reflexes are changed: the knee- jerks are usually abolished, but Babinski's sign is present. In some cases the knee-jerk is preserved until late in the course of the disease. The abdominal reflex gradually disappears. The tendon reflexes of the arms disappear later than those of the legs. Sensory. — General sensations are usually not affected, except per- haps in the last stage, when anaesthesia appears. Egger observed in his cases a diminution or loss of vibratory sense of tuning-fork. The special senses are not involved. The eyes, however, present one special symp- tom which is very frequent in Friedreich's disease, viz. nystagmus. It consists of brief and repeated movements of the eye-globes when the FRIEDREICH S ATAXIA 263 patient is told to turn his eyes to the right and to the left, without moving his head. The ocular movements are intact. Cerebral. — Vertigo is frequent. Intelligence is normal, except to- wards the end, when dullness and childishness make their appearance. The speech is almost always affected: it is slow, the articulation is diffi- cult, irregular; some word.; are pronounced abruptly, some with delibe- ration. During conversation the muscles of the face are affected with irregular contractions the movements of the face are ataxic. Fig. 86. — Characteristic Feet in Friedreich's Ataxia. {Bouchard and Brissaud.) Trophic. — There is a special deformity of the foot which is character- istic of the disease. It is of the equinus type: the foot is short, as if it was pressed antero-posteriorly; the plantar surface is concave, while the dorsal is prominent; the toes are "claw-like" because of forced extension. This condition is usually bilateral. In a few cases the same deformity was observed in the hands, which also assume the claw-like position (main en griff e) . Scoliosis, or more frequently kypho-scoliosis, is observed in an advanced stage; it is particularly marked in the dorsal region. The sphincters are as a rule intact. Course and Prognosis. — The disease is essentially progressive, but slow in the development. The earliest symptoms are : ataxia and hyper- extension of the great toe. The upper extremities become involved after the lower. The eye symptom and the peculiar speech appear still later. Within a period of five years the clinical picture is complete. The patient is then bedridden and loses all power in his limbs. Remissions have been observed, but any intercurrent acute disease hastens the course of the malady. It may last an indefinite number of years, but the outlook is invariably grave. Death usually occurs from some intercurrent disease. Diagnosis.— With tabes Friedreich's disease may be confounded 264 INFANTILE SPINAL PARALYSIS because of the ataxia and the loss of knee-jerks. In the latter affection there is no true tabetic incoordination (see Tabes), but a cerebellar titubation. Moreover the presence of choreiform movements, nystagmus, disturbance of speech, the absence of sensory disturbances, of sphincter involvement, of pupillary changes, will enable one to make the diagnosis of Friedrich's disease. In Multiple Sclerosis there are nystagmus and intention tremor, but in Friedreich's disease the nystagmiform movements of the eye-globes are only in transverse direction, while in multiple sclerosis they are in all directions. The tremor in Friedreich's disease is considerably less marked than in multiple sclerosis. The knee-jerks in the latter disease are usually increased, in the former abolished. Treatment. — It is purely symptomatic. There are no remedies for arresting the course of the disease, which is inevitably progressive. Massage and systematic exercises of the limbs (motor reeducation) maybe of benefit (see Treatment of Tabes). V. Infantile Spinal Paralysis (Acute Anterior Poliomyelitis) Pathology. — The lesion consists chiefly of an acute inflammation of the anterior cornua of the spinal cord. In recent cases the microscope shows dilatation of the blood vessels, leucocytic infiltration of their walls (arteritis); the ganglionic cells surrounded by this inflammatory tissue undergo degeneration and atrophy: their protoplasma (Nissl's bodies) becomes disintegrated, the nucleus and nucleolus fall out and the pro- longations (dendrites and axis cylinders), whose existence depends upon the integrity of the cells, undergo atrophy. The morbid process therefore consists of inflammatory softening. The latter may be found in small foci or occupy a large portion of the cornua. The destroyed tissue is gradually substituted by proliferated neuroglia, so that when the cord is examined, several years after the acute onset, one can see cicatricial tissue in the substance of the anterior cornua, and in the midst of it de- formed, dilated and thickened blood vessels. The entire cornu becomes diminished in size, and if the other is intact, the contrast between the two is striking. The anterior roots, which are the prolongation of the axis-cylinders emanating from the cells, undergo atrophy. The motor fibers of the body which are the continuation of these roots and the muscles supplied by the nerves of the anterior roots also suffer in their nutrition : the muscular fibers undergo granular degeneration and are replaced by fibrous or adipose tissues. The bones of the affected limbs participate in the general and progressive atrophy, so that an entire INFANTILE SPINAL PARALYSIS 265 limb may be arrested in its development and remain very small, if the disease occurred in infancy and was neglected. Etiology and Pathogenesis. — All the evidences are in favor of an infectious cause of the disease. The onset of the disease and the occasional epidemics occurring in certain localities leave no doubt as to its infectious nature. On two occasions within a period of two years I observed a series of eight cases and of twelve cases in one section of this city. Ac- cording to Marie, an embolus of infectious nature reaches the anterioi cornua through a branch of the anterior spinal artery. The most recent experimental investigations of Levaditi, Landsteiner, Popper, Flexner, Lewis and others have established the fact that the upper respiratory passages and the intestinal tract are the usual seat of the virus. Expectora- tion and the stools of an infected individual are * the sources of contamination (Kling, Weinstedt and Pettersson). Flexner, Clark and Frazer have demonstrated {Jour. Amer. Med. Assn., 1913) the ^ occurrence of the virus in the naso-pharynx of healthy persons who have been in close contact with an acute case of poliomyelitis. Contagion by dust, clothing and shoes has also been established. ^ „ ~ _ Fig. 87. — Degenera- Insects, such as flies, have been shown to carry TIO n of a Cell of the the virus and transmit it. The original idea of Anterior Corntt in a Wickman as to the fact that the disease is con- Case of Acute An " TERIOR POLIOHYELITIS. tagious is now completely confirmed. The exis- {original.) tence of epidemic foci may pass unobserved in large cities. The season of the year appears to be an important predis- posing cause of poliomyelitis. Summer and early autumn are the most favorite seasons. It seems that a certain immunity is acquired by a country in which an epidemic attack once occurred. Thus in Sweden the provinces affected severely in 1905 were apparently free from the dis- ease in 191 1. The immunity produced by an abortive attack will prob- ably explain the resistance of older children and adults. As to the nature of the virus, it is as yet impossible to determine it, as the microorganism has not yet been discovered. It has been shown by Flexner, Lewis and Levaditi that the unknown microbe belongs to the class of very small microorganisms as it is able to pass through the filter, a fact which is not observed with the majority of well defined microorganisms. Finally Rosenau has recently demonstrated that the barn-fly (stomoxys calcitrans) is a possible disseminator of the poliomy- elitic infection. Through this agency he succeeded in transmitting 266 INFANTILE SPINAL PARALYSIS poliomyelitis from monkey to monkey (/. Am. Med. Ass., 191 2). Ander- son and Frost were able to confirm it; they also succeeded in transmitting the infection to a fresh monkey with the emulsion of the cord of an animal infected by the flies. Infantile paralysis is a disease of early childhood. It should be borne in mind that it may also occur in adult life, but this is rare. The boys are more frepuently affected than girls. Among predisposing etiological factors trauma may be mentioned. I have seen a number of cases in which the onset dates from a fall. Starr mentions cases of poliomyelitis which developed immediately after exposure to cold, such as swimming in very cold water. Symptoms. — The onset is always sudden and resembles that of an acute infectious disease. The incubation period usually lasts from 1 or 2 days to 10 days. Fever, general malaise, aonrexia, vomiting, diarrhoea or else coryza, angina, bronchitis and sometimes convulsions are the usual initial symptoms. In some cases only one or two of them are present and in others they are so mild that they may be overlooked. In still others none of the premonitory symptoms is observed. The child suddenly becomes paralyzed. Some cases begin with symptoms of meningitis : rigidity of the neck and trunk, somnolence, agitation, headache, convulsive movements of the eyes, tenderness of the spine, general hyperesthesia, articular pain. Exanthemata or erythemata are not infrequently seen. Children fre- quently complain of pain in the back or in the affected limbs. In a day or two and sometimes later the principal symptom, viz. paralysis, makes its appearance, and it progresses so rapidly that it reaches its climax in twenty-four hours. It affects one limb, two symmetrical limbs (usually the lower extremities) or an arm and leg on the same side, or one arm; it may also involve an arm on one side and a leg on the other. In some cases all the four extremities may become paralyzed. In a few days the paralysis retrocedes: motility returns in certain groups of muscles, so that at the end of a few weeks the paralysis becomes fixed in one or two extremities. When the lower limbs are paralyzed, there may be: a thigh type and a leg type. In the former the muscles on the inner side of the thigh escape; the iliacus, glutei and the antero-external muscles of the thigh are affected. In the leg type the peronei and anterior tibial are mostly paralyzed; the posterior tibial less frequently. When the upper limbs are involved, there may be also two types: upper and lower arm types. In the first the scapular muscles, deltoid, biceps and supinator longus are paralyzed. In the second the muscles below the elbow except the supinator longus are affected. Finally an entire limb may be involved. INFANTILE SPINAL PARALYSIS 267 The most charcteristic feature of the paralysis is the flaccidity. The tendon reflexes of the affected limbs are either totally abolished or greatly diminished. This condition is the general rule. The following varieties have been observed: (1) exaggeration of both knee-jerks; (2) loss of one knee-jerk, and exaggeration of the other; (3) a transient exaggeration immediately preceding their abolition. The paralyzed muscles soon begin to degenerate and atrophy and their contractility gradually decreases. Their electrical irritability suffers a radical change: under faradism there is at first a diminished response and later complete loss of response. Under galvanism there is not only a quantita- tive, but also a qualitative alteration: reaction of degeneration appears quite early. Later all contractility to elec- trical stimulation is lost. Parallel with the increasing atrophy, the growth of the muscles is arrested, contractures are formed because of the predominance of the antagonistic mus- cles, and all these factors lead to de- formities. Among the latter the most common is pes equinovarus. Scoliosis, lordosis, are also met with. Finally the hands may also occasionally be affected: a claw-like hand is the most usual de- formity. Deformities of joints is a fre- quent consequence of poliomyelitis: paralysis of the muscles surrounding Fia 88.— Infantile Spinal Para- .... . lysis, Showing Atrophy of right the joints leads to a relaxation of the LoWER ExTEEMITY) AND scoliosis. latter, so that the heads of the bones fall from their sockets. In a certain number of cases the bony tissue of the limb may be arrested in its development and then one can see an adult with one or two extremely thin limbs. The skin covering such a limb is very thin, ulcerates after the slightest erosion, is always cold, cyanosed. The sensations and the sphincters are intact. In some cases I observed a marked tenderness in the paralyzed limbs (Figs. 88, 89, 90). Course, Termination, Prognosis. — The clinical picture just described may vary considerably. Thus the paralysis may be only transitory and last only a few days or else two to three weeks. In such cases the atrophy 268 INFANTILE SPINAL PARALYSIS will never develop. In other cases the disease spreads and involves the nuclei of the medulla, and if the ninth and tenth nerves are involved, the patient dies from bulbar symptoms. The typical cases when properly and early enough treated may regain considerable of the lost power in the limbs, but they never recover com- pletely. The majority of cases remain wdth some infirmity: either the pes equinus persists or the atrophy with some impairment of locomotion becomes permanent. In some cases, many years after the first attack, the patient is taken with another in some other limb, which then runs the Fig. 89. — Infantile Spinal Paralysis Showing Deformities of the Lower Limbs. Fig. 90. — Atrophy of Right Thigh. Sequela of Acute Anterior Poliomyelitis in Childhood. same course as the old spinal palsy. In a case that came under my obser- vation (Amer. Med., 1903) symptoms of amyotrophic lateral sclerosis began to develop a. propos of an injury years after the infantile spinal paralysis. Generally speaking, the prognosis depends a great deal upon the electrical reactions of the muscles. Reaction of degeneration is usually an unfavorable sign for recovery of the paralyzed muscles. Loss of faradic contractility is not always an indication that the muscular con- tractility will not return. I have seen cases of return of this form of INFANTILE SPINAL PARALYSIS 269 electrical reaction long after the onset of poliomyelitis. As to life, the outlook is good except when the medulla is involved. Diagnosis. — The sudden onset with its characteristic prodromal symptoms, the flaccid palsy, the loss of reflexes, the atrophy, are all typical enough for recognition of the disease. Nevertheless there is a certain group of affections with which acute poliomyelitis may be con- founded. There is a form of poliomyelitis in which the most conspicuous symptom is pain along the spine and in the limbs. The pain is consid- ered as being due to spinal meningitis. In some cases the onset is so tumultuous that epidemic cerebro-spinal meningitis is thought of. Pain in the head, rigidity of the neck, pain along the spine, Kernig's sign and even disturbance of consciousness may be present. The differential diagnosis will be as follows: Meningeal symptoms of poliomyelitis disappear rapidly and they are distinctly spinal. In cerebro-spinal meningitis they are both cerebral and spinal. In the first the paralysis is paraplegic monoplegic or hemiplegic and flaccid in type. In the second the paralysis is spastic and cranial nerves are frequently involved. The reflexes are diminished or lost in the first, but increased in the second. The toe phenomenon is absent in the first, present in the second. Lympho- cytosis in the cerebro-spinal fluid is characteristic of the first, leucocytosis of the second. In the first the fluid is clear, in the second cloudy. In the first there are very few polymorphonuclear cells, in the second — abundant. No microorganism is found in the cerebro-spinal fluid of poli- omyelitis, meningococci are found in that of cerebro-spinal meningitis. In serous spinal meningitis in which pain and paralysis also lympho- cytosis are present, the type of paralysis and the condition of reflexes are not the same as in poliomyelitis. In Multiple Neuritis are present: pain in the limbs, paralysis and altered reflexes like in poliomyelitis. The points of difference are: in the second there are general manifestations indicative of an acute infection; they are absent in the first. In the second paralysis extends further than in the extremities, the muscles of the trunk are involved. In the first the trunk is intact. In the second the palsy is irregular and more marked in the muscles of the hip and thigh. In the first the paralysis is regular and more marked in the most peripheral muscles of the limbs. Atrophy sets in much earlier in multiple neuritis than in poliomyelitis. Objective sensations are rarely disturbed in poliomyelitis; in polyneuritis hyper- esthesia is common and pain, burning, etc., are frequently complained of in the fingers and toes. Polyneuritis is frequently cured without leaving any trace, but this is not the case with poliomyelitis, in which deformities of the limbs are the rule. 270 INFANTILE SPINAL PARALYSIS Acute Transverse Myelitis is exceptional in children. Besides, involvement of the sphincters is common. In poliomyelitis the sphincters are not involved. Birth or Obstetrical Palsy follows a difficult labor or instrumental delivery; there are always sensory disturbances. The atrophy and objective sensory disturbances followed a radicular distribution. Cerebral Infantile Hemiplegia will be recognized by the spastic paraly- sis and the abnormal reflexes usually found in hemiplegia (see this chapter). In Hysterical Paralysis the reflexes are preserved and the electrical reactions are normal. The foregoing study permits the following grouping of varieties of acute poliomyelitis: (1) The Spinal Type. — It is the most frequently observed. (2) The meningeal type, in which the onset presents meningeal symp- toms (see above) . Generally in a few days they clear up and the picture of poliomyelitis becomes evident. (3) The polyneuritic type, in which there is hyperaethesia and pain in the affected limbs (see above). (4) The bulbar type, in which the nuclei in the medulla become involved in the course of poliomyelitis: the prognosis here is grave. (5) The abortive type, in which the symptoms are very mild, last but a short time and totally disappear. Treatment. — At the onset of the disease the child should be kept in bed and every effort made to reduce the temperature with cold - baths or spongings and coal-tar products (antipyrin, phenacetin). Cauterization and revulsion in the form of cupping or mild counter- irritation with mustard plasters along the spine may be of benefit. Urotropin taken by the mouth sets free formaldehyde, and the latter is then found in the cerebro-spinal fluid. It acts therefore as an antiseptic. It may therefore be useful. A child of three can be given 1-2 gr. two or three times a day. Pain can be combated by sedatives such as bromides. Purgatives should never be neglected. The naso- pharynx should be taken special care of (see Etiology), and treated with antiseptic irrigations or applications. Ergot has been advised internally. After the acute stage has subsided, an effort is to be made to improve the general nutrition by a substantial diet and hydrotherapy. Iodides can then be administered; cod-liver oil, arsenic, hypophosphites, are useful. Electrical treatment and massage must be instituted as promptly as possible. It is advisable at first to apply the galvanic cur- rent with the positive pole on the affected muscles. When improvement is noticeable, the faradic current will be more useful. Daily applica- INFANTILE SPINAL PARALYSIS 2J I tions of electricity for ten or fifteen minutes, aided by massage of the limbs and kept up persistently will render great service. When improvement is commencing to be noticeable, the patient is taught to exercise the affected muscles voluntarily. Such efforts on the patient's part are of great benefit. As to the duration of the electrical treatment and massage, it should be kept up indefinitely. I have seen cases which began to show signs of improvement at the end of one to two and even three years. To guard against contractures systematic exercises and gymnastics are advisable. When contractures and deformities are present, orthopedic applicances may be of some benefit. The use of mechanical apparatuses should not begin too early, as the weight of them will impose too much work on the muscles which commence to improve and thus overfatigue them. I have seen bad results from too early application of braces. On the other hand a properly applied apparatus may prevent a tendency to deformity. The time of using braces must be individualized in each case; no set rule can be given for all cases. If after a sufficiently long trial the orthopedic appliances fail, surgical intervention should be resorted to. In certain cases tenotomy of shortened tendons will place the deformed limb in a straight position, provided there is enough muscle left to be relied upon. Recently attempts have been made to connect muscles which are intact with the atrophied muscles. A healthy tendon or muscle is divided and sewed to the atrophied tendon or muscle, respectively. I have seen some very good results from such procedures. Instead of anastomosing muscles, healthy nerves have been divided and their central ends united with the peripheral ends of divided nerves in the atrophied muscles. Some good results have been reported. Recently Schwab and Allison, also Stoffel, devised methods to com- bat contractures and deformities. (See pages 122 and 123.) Prophylaxy of Acute Poliomyelitis.- — The above described results obtained from experimental investigations (see Etiology) gives us a clear idea of what preventive measures should be. It is necessary also to em- phasize the fact that the virus may resist a very long time. For example, the virus mixed with sterile milk or water kept at temperature of the room maintains its pathogenic activity during thirty-one days (Landsteiner and Levaditi). Salivary and nasal secretions are carriers of poliomyelitic virus and are chiefly the sources of contagion. As desiccation does not destroy the activity of the virus, contagion may take place through dried up secretions. Rigorous antiseptic measures applied to the nose and throat in cases of epidemics, or when in contact with individuals affected with the disease, are urgent. Isolation is equally urgent. 272 MYELITIS VI. Chronic Anterior Poliomyelitis and Amyotrophic Lateral Sclerosis They belong to the systemic diseases of the spinal cord and are described in the chapter on Muscular Atrophies. B. NON-SYSTEMIC DISEASES OF THE SPINAL CORD I. Myelitis A. Acute Myelitis Acute myelitis is an acute inflammation of the spinal cord secondary to an infection or intoxication. Pathology. — The lesion is essentially diffuse. It is irregular in distribution. When several foci are present, the disease is a disseminate myelitis ; when one focus extends upward or downward, it presents a diffuse myelitis. When the entire transverse section of the cord is involved, the disease is a transverse myelitis. According to the seat of the lesion, myelitis may be: cervical, dorsal, lumbar. Irrespective of the localization of the focus, the pathological anatomy in all varieties of myelitis is the same. The cord is usually congested, cedematous, and the diseased portions are soft. The softening undergoes three distinct phases : at first the stage of red softening, later that of white softening and still later the stage of sclerosis. In the first period (red softening) the blood vessels are dilated and their walls are infiltrated with leucocytes. The cells of the gray matter undergo disintegration, and the axis-cylinders are swollen and varicose. In the second period (white softening) necrosis takes place: the entire cord tissue is very soft and appears white; the cells are colorless and de- formed, without nuclei and prolongations. The nerve fibers lose their myelin sheaths, remain naked and unsupported. Ascending and descend- ing degeneration in the white columns becomes evident. Instead of the normal nerve tissue there is a large number of granular cells. In the third period there is formation of cicatrices at the expense of the neuroglia tissue, which at that time proliferates in abundance: instead of being soft the cord becomes hard. As death may occur during any one of these stages, the histological picture will vary according to when the pathological process was arrested in its development. The meninges usually participate in the myelitic process. They are thickened (especially the pia-mater) and adhere to the cord. If the cells of the anterior cornua are extensively involved, the anterior roots and the peripheral nerves undergo alterations and the muscles supplied by these nerves atrophy. With reference to the pathology of MYELITIS 273 myelitis it is interesting to say a few words of Buzzard's conception. He believes that myelitis is the result of a thrombotic softening of the cord and that the usual causes of myelitis produce an inflammatory process in the vessel wall which permits the formation of thrombi, resulting in softening of the cord. The special nature of distribution of the blood vessels in the spinal cord is such as to directly favor the occurrence of thrombosis, especially in the lumbar region where myelitis is so frequently met with. This peculiarity consists of a great length of the small arteries, especially of those supplying the lower portion of the cord. Acute myeli- tis is therefore, according to Buzzard, a thrombotic softening. Etiology. — Acute myelitis is always the result of an infection or intoxi- cation. Cold, traumatism, emotions which formerly were considered as direct causes, are in reality only predisposing factors. In favor of this view speak the occurrence of myelitis in the course of acute and chronic infectious diseases, also experimental investigations. Influenza, typhoid fever, small-pox, scarlet fever, pneumonia, gonorrhoea, whooping cough, measles, tuberculosis and syphilis are all diseases which may become complicated by myelitis. A neighboring inflammatory process in the meninges or vertebrae may extend to the cord and develop myelitis. During puerperium myelitis has been observed. In all these various infections of a general or local nature, either the microorganism itself or its toxin, and perhaps both, are the immediate causes of myelitis: they penetrate the cord through the blood vessels. Experimentally it has been proven beyond any doubt, I believe, that myelitis can be pro- duced by inoculations of various microbes and of their toxins (Roux, Yersin, Grancher, Manfreda, etc.). Symptoms. — In the chapter on "Pathology" various forms of myelitis have been mentioned. The most frequent of all is Transverse Myelitis. — The usual seat of this form is the dorso-lumbar region, but it may also occur in the cervical and dorsal regions. The onset in the majority of cases preceded by prodromal symptoms. They are: pain in the back, paresthetic disturbances (tingling, numbness, etc.), retention of urine, also some weakness in the extremities. The pain is not confined to the back, but it also radiates toward the limbs. The prodromal period usually lasts from a few hours to several days. After myelitis is established, the following symptoms become characteristic. Motor, — In the dorso-lumbar form the legs present a complete loss of power. If the myelitis is cervical, the arms are paralyzed. . The paralysis is flaccid in transverse myelitis of the dorso-lumbar cord, spastic in dorsal and cervical myelitis. The patellar tendon reflexes are abolished in the first, increased in the other two forms. Babinski's sign and paradoxical 2 74 MYELITIS reflex may be present in all the three forms. Curiously enough there is an antagonism between the latter two reflexes. At the beginning Bab- inski's phenomenon is quite rare, but the paradoxical very frequent. Later on, when contractures appear, the paradoxical becomes less marked, but Babinski's reflex more pronounced. Oppenheim's reflex may some- times be present. Ankle-clonus may and may not be present. As the disease gradually advances and passes into a chronic state, con- tractures develop. In the cervical form of myelitis the paralysis may affect not only the arms, but also muscles of the neck, the intercostal and abdominal muscles, the lower extremities, and the diaphragm, and thus cause difficult breathing. In myelitis of the eighth cervical segment there is also paralysis of the small muscles of the hands and of the flexors of the fingers. Sensory.- — They are constant. The patients complain of violent and continuous pain in the back, which in the cervical form radiates to the arms and in lumbar form to the legs. The tabetic girdle pain is present here. Sensations of cold or heat, tingling, burning, etc., are also very fre- quent. Objective sensory disturbances are quite marked. The sense of touch, pain and temperature in the upper and lower extremities and on the body (according to the seat of the lesion) is greatly diminished and sometimes completely lost in the areas innervated by the diseased seg- ments and below these segments. In some cases there is a dissociation of the three forms of sensations (see Syringomyelia). Trophic disturbances are usual. The affected extremities are cyanosed and cedematous. When a portion of the body undergoes pressure, ulcer- ation soon develops. This is particularly frequent in the sacral region, but it occurs also on the gluteal areas and at the level of the trochanters. The decubitus is only present in the dorsal and dorso-lumbar varieties of transverse myelitis. The ulceration is usually superficial, but it may be so deep as to expose the bone. In a case that recently came under my observation, there was at first a cervical myelitis, but later the lesion spread in a descending manner. The bedsores were so marked that over the entire lumbar region the cutaneous and muscular tissues were totally destroyed and the largest part of the lumbar vertebrae and of the sacrum were exposed. In the same patient the prepuce was totally gangrenous and a portion of the dorsum of the penis was ulcerated. Muscular atrophy of the lower extremities is rare, but frequent in the upper limbs in myelitis of the lower cervical cord, absent when the upper cervical segment of the cord is involved. If atrophy occurs, reactions of degeneration (CaCC > AnCC) are present. In myelitis with involvement of the eighth cervical MYELITIS 275 segment there will be, besides the above mentioned paralysis and atrophy, also small pupils and narrow palpebral fissure. Sphincters are always involved in the dorsal and dorso-lumbar mye- litis. At the beginning there is retention. Later incontinence develops. Both sphincters are almost equally involved. Cystitis is a frequent complication. Impotence frequently exists in myelitis of any segment of the cord. The clinical pictures of myelitis at various levels of the cord as just given are not always clear-cut. They may vary from one case to an- other. It depends upon the extent of the transverse section. If, for example, in the cervical form the lesion is sufficient to cut off all impulses passing through the diseased segment, the paralysis of the body below the lesion will be flaccid, all reflexes and sensations are lost. So it is with any other segment of the cord. If the lesion affects only one side of the cord, Brown-Sequard's form of paralysis will be present. Syphilitic myelitis, which is the most frequent of all form, presents certain special features. It will be discussed in detail in the chapter on Syphilis of the Nervous System. Tubercular myelitis may occur: (1) as a complication of cerebro-spinal tubercular meningitis (tubercular meningo-myelitis) , (2) as a complica- tion in Pott's disease (either as a compression myelitis or a secondary myelitis following a caseous pachymeningitis) ; (3) it may also occur in the course of Pott's disease as primary parenchymatous myelitic foci which develop without meningitis, without vascular and interstitial lesions, without caseation and very probably due to tuberculine circulating in the cord. This form of myelitis is analogous to lesions found in the liver and kidneys of tubercular individuals. Philippe and Cestan have re- ported such a form of myelitis (Rev. Neurol., 1899). Course, Termination, Prognosis. — While in the majority of cases the complete picture of acute myelitis develops within a period of from a few days to a few weeks, there are nevertheless cases with a sudden onset and rapid death. Usually the disease is progressive and becomes chronic. Great improvement and even complete recovery are possible, especially in cases that follow an acute infectious disease. In such cases the improvement in sensations precedes that in the motor power. The paraplegia is usually spastic. The improvement may be maintained for many years and the patient may resume his usual occupation and be only slightly disturbed by the sphincters. The greatest amount of dis- turbance occurs in myelitis of the lowest segment of the cord. Here the atrophy of the muscles and the condition of the sphincters are exceed- ingly obstinate. In some cases the disease acquires an ascending or de- 276 MYELITIS scending course. In the first case it may reach the medulla and then lif e is greatly threatened. In other cases the decubitus and cystitis, which may become purulent, are capable of killing the patient. Finally inter- current diseases are a source of great danger to myelitic patients. Re- missions in the course of myelitis are possible. The patient apparently improves to a great extent, and under the influence of an insignificant cause may suffer a relapse. The outlook in acute myelitis is always doubtful. Diagnosis.— Multiple neuritis is sometimes confounded with myelitis. Tenderness of the nerve trunks, the predominance of the sensory disturb- ances, integrity of the sphincters, irregular involvement of the motor ap- paratus and especial involvement of extensor groups of muscles — all these symptoms are characteristic of multiple neuritis. Besides, in myelitis the objective sensory disorder is of segmental type, and more pro- found. In neuritis the limits of the sensory disturbances are not well defined, the latter are present only at the periphery, and, what is im- portant, the tenderness of the nerve trunks is associated with anaesthesia or analgesia of the skin. Hysterical paraplegia may simulate acute myelitis. In the former there is usually a history of a shock or of a great emotion, of a traumatism. The onset is sudden. The reflexes and the sphincters are intact. There are no trophic disturbances. Finally the existence of hysterical stigmata will aid in making the diagnosis. Hematomyelia presents a very sudden onset besides the history of trauma. Spinal compression presents usually a gradually oncoming paralysis, but when the paralysis is sudden or rapid like in transverse myelitis, it is usually caused either by caries or malignant disease of the vertebrae. Besides, the pain in compression myelitis is unusually intense and agonizing. Treatment. — In the first stage of the disease the patient should be given absolute rest. A purgative should be administered. Pain will be controlled by salicylates and coal-tar products. In case of retention of urine catheterization is necessary. Local applications to the spine, as vesicants or cauterization, have been recommended, but in view of the sensory disturbances which are constantly present in myelitis, and the facility with which ulcerations are formed, it is advisable to avoid local irritation of the skin. Inunctions of mercury and internal administration of iodids should be instituted as soon as possible not only in syphilitic cases, but also when no history of direct infection could be obtained. (For the mode of administration and dosage, see Tabes.) In addition to the drug treatment hygienic measures are of great im- MYELITIS 277 portance. Special and prompt care must be taken of the bladder and of the decubitus, as complications not infrequently come from these two sources and death may ensue. Purulent cystis is often the result of infec- tion from repeated catheterization. Decubitus is also infectious in nature. It is therefore essential to see that urine and feces should not come in con- tact with the skin, that aseptic and antiseptic precautions be taken in the strictest possible manner. In cases of retention of urine, each aseptic cath- eterization must be followed by a washing out of the bladder with some mild antiseptic solution, as boric acid. By doing so, cystitis with all its grave consequences may be avoided. In order to avoid undue pressure of the skin, as the latter is usually the immediate cause of decubitus, the patient's position in bed should be frequently changed and the bed cloth kept smooth. After the acute stage has subsided, massage becomes indicated, but in this latter procedure great care must be exercised that the skin is not in- jured. In a patient under my observation a small erosion of the skin of one leg caused by a masseuse became ulcerated and during three and a half months the ulceration remained practically unaffected by various medications. Electricity is a valuable adjuvant to massage. It should be avoided in cases of spasticity, but it may be useful in flaccid paralysis. Both procedures are important in treatment of atrophy. Galvanism at first and faradism later or both alternately may be employed. Hydrotherapy in the form of sponging, or ablutions followed by gentle rubbing, is a good adjuvant in treatment of myelitis. Spasticity of the extremities can be relieved by passive movements and warm baths. Acute Diffuse Myelitis This is a rare disease. Pathologically it consists of a myelitic focus having an ascending (more frequent) or descending course. It is known under the name of acute ascending or Landry's paralysis. It will be described later together with multiple neuritis. Acute Disseminated Myelitis This affection consists pathologically of myelitic foci disseminated throughout the central nervous system. Its clinical picture is that of disseminated sclerosis. The latter will be described later. B. Chronic Myelitis Chronic myelitis as a primary affection occurs in traumatism, in com- pression of the cord, in infections of long standing (syphilis, leprosy), in 278 MYELITIS intoxications of long standing (diabetes, pernicious anemia, ergotism). The lesion and the symptoms are slow and long in development. They will be described in separate chapters. As a secondary affection it follows acute myelitis. It is only with this form that we will be concerned here. Pathology.- — If a myelitic cord is examined years after the acute onset, it will be found shrunken and small in size. A transverse cut will show that the cord is discolored and grayish in appearance. Histologically it presents a destruction of cells and disappearance of myelin around the nerve fibers, also in some places disappearance of axis-cylinders. Instead of the normal nervous elements, there is seen a large amount of proliferated neuroglia and connective tissue. Secondary ascending and descending degenerations are distinct. The meninges are frequently found thickened as a result of a previous participation in the inflammatory process. The walls of the blood vessels are also thickened. Fig. 91. — Chronic Myelitis, Showing Degeneration op White and Gray Substance. {Original.) Symptoms. — They are practically the same as in acute myelitis, when the latter are fully developed, viz. spastic paraplegia with the usual abnormal reflex phenomena, disturbances of the sphincters, sensory disturbances, as paresthesia, hyperesthesia or anaesthesia. What char- acterizes chronic myelitis particularly is the mild degree of the disturbances in the sphincters and in the sensory sphere. The spasticity on the con- trary is much pronounced. Atrophy of the affected limbs also occurs and the electrical contractility is usually diminished. Reactions of degenera- tion are rare. The extent of the loss of power in the extremities is various. HEMATOMYELIA 279 In some cases it is absolute, in others only partial and in still others extremely slight. Bed-sores are rare. Course, Prognosis. — While the disease is usually progressive, in a great many cases it presents stationary periods. At all events it is ex- tremely slow in development. The prognosis is unfavorable, as the destroyed tissue can never be replaced by healthy nervous elements. Death usually occurs from some intercurrent disease or from bulbar in- volvement, when the myelitis is of ascending character or when confined to the cervical cord. Diagnosis. — The history of the onset and the grouping of the charac- teristic symptoms are usually sufficient for making the diagnosis. Some- times, however, the diagnosis may present some difficulty. In progressive muscular atrophy, for example, the muscular wasting is regular and typical in distribution, while in chronic myelitis, if atrophy occurs, it is irregular. Moreover in the latter affection there are sensory disturbances and they are absent in the first. Finally the state of the sphincters will remove the least doubt, as they are intact in progressive muscular atrophy. Primary Spastic Paraplegia will be recognized by the absence of sphincter disorder, of sensory disturbances and of a history of an acute onset. A tumor of the spine will be recognized by its slow development and by the unusual intensity of pain. Treatment. — -It is largely the same as that of the advanced stage of acute myelitis (see above). II. Hematomyelia (Hemorrhage in the Spinal Cord) Hemorrhage in the cord may occur in the course of acute myelitis or specific myelitis, tumors, pernicious anemia and in cases of softening caused by an embolus or thrombosis. In such cases the hemorrhage is a secondary condition, merely an accident in the course of those affections. Hemorrhage in the cord may also be primary. It is with the latter exclusively that we will be concerned here. Pathology. — The hemorrhage may be capillary or in the shape of one or several foci of more or less large size. The favorite seat for primary hemorrhages is the gray matter, because of the great vascularity of the latter. They are particularly marked at the level of the enlargements. They may extend transversely or longitudinally; they may also occur in the central canal. The cervical region is the most frequent seat of trau- 280 HEMATOMYELIA matic hematom\-elia; the dorsal is next in frequency. On section the cord appears soft. At first the blood destroys the cells and the axis- cylinders undergo secondary degeneration. Later on, when the clot begins to disap- pear, the neuroglia tissue around it proliferates. Above and below the hemorrhagic foci tracts of nerve fibers will undergo ascending and descend- ing degeneration. The walls of the blood vessels are thickened and sclerosed. The hemorrhagic focus is eventually absorbed and replaced either by a scar or, as it happens in large hemorrhages, by a cyst, or a cavity surrounded by a thick capsule. Etiology. — Primary hematomyelia may be spontaneous or traumatic. In the spontaneous cases the underlying cause is a state of congestion of the cord. The hemorrhage then occurs after a great physical effort, viz. violent exercises, coitus, paroxysms of whooping cough. It has also been observed in purpura, in hemophilia, in cases of suppression of menses or of hemorrhoidal bleeding, sudden decompression (see Divers' Disease). Spontaneous hematomyelia is rare. Traumatism is the most frequent cause of hematomyelia. Fracture, dislocation of the vertebrae, obstetrical intervention, dystocia forced, inclination of the head, a blow or a fall on the back, a fall on the feet, are the usual causes. In such cases there may be a predisposing factor, such as preexisting vascular changes. Symptoms. — The onset is exceptionally sudden (Vulpian's spinal apoplexy), sometimes with loss of consciousness. The patient experiences a sharp pain, the seat of which depends upon the level of the injured vertebras. The patient falls and immediately paralytic symptoms, disturbances of sensations and of the sphincters appear. Sometimes these symptoms appear only a few hours after the onset, but pain in the spine is always present. The lower extremities are usually involved, but accord- ing to the seat of the lesion there may be paralysis of all four extremities or only of the arms. The paralysis is flaccid, when the lesion is in the dorso-lumbar segment of the cord; if it is in the cervical cord, the paralysis of the lower extremities will be spastic. The reflexes are exaggerated in the spastic palsy, abolished in flaccid paralysis. The reflexes as a rule are abolished immediately after the onset, irrespective of the level at which the injury occurs. The muscles gradually undergo atrophy. The sensory disturbances at the beginning will consist of a numbness and tingling in the affected limbs. Objective sensory disturbances are always present. They consist of a marked diminution of all forms of sen- sations or of a complete anaesthesia of the affected limbs. Sometimes a syringomyelic sensory dissociation may be evident: it consists of a loss of HEMATOMYELIA 28l n (T .... , XX 1 III. ^>-?b ! CO Will. -^^f\ II III _ iV. £0 rectus lateralis 11 to rectus antic, minor Anastomosis with hypoglossal Anastomosis with pneumogastric N. to rectus antic. major. N. to mastoid region. Great auricular n. Transverse cervical n. ==}JT. to Trapezius, Aug. Scap. and Rhomboid. Supra clavicular n. Supra-acromial n. Phrenic n. N. to levator ang. scap. Jf. to rhomboid Subscapular n. Subclavicular n. N. to peetoralis major. .Circumflex n. Musculo-cutaneous n. Median n. Radial n. Ulnar n. Internal cutaneous n. Small internal cutaneous n. Iliohypogastric n. Ilio-inguinal n. ..External cutaneous n. Genito-crural n. Anterior crural n. Obturator n. N. to levator ani A. to obturator int. JV. to ophincter ani Coccygeal n._ Superior gluteal n. JV. to pyriformis N. to gemellus super. _A T . to gemellus infer. If. to guadratus Small sciatic n. Sciatic n. Fig. 92. — Relation of Segments of Spinal Cord and Their Nerve Roots to the Bodies and Spines of the Vertebra. {After Dejerine and Thomas.) 282 HEMATOMYELIA diminution of sense of pain and temperature and preservation of the sense of touch. According to Minor, the latter occurs in central hematomyelia. A very important and early symptom which persists is a paralysis of the sphincters of the bladder and rectum (constipation and retention of urine). There is usually a rise of temperature at the beginning, but it goes down to normal in a few days. If death does not occur at the end of a few days, all the symptoms begin to improve and some of them may even disappear. The flaccid paralysis becomes spastic. Then the reflexes are increased. Ankle-clonus, Babin- Fig. 93. -Brown-Sequard's Paralysis Following a Gunshot Fracture of the Right Fifth Cervical Vertebra. Paralysis of the right upper and lower limbs and sensory disturbances on the left. The black indicates thermoanesthesia and analgesia; the portion shaded in lines — hypalgesia and thermo-hypsesthesia. ski's sign, paradoxical reflex, Oppenheim's reflex, make their appearance in the lower extremities. Muscular atrophy with reactions of degeneration develop rapidly. When the hemorrhage is in the cervical cord, the muscu- lar atrophy of the upper extremities is of Aran-Duchenne's type. The sensory disturbances usually disappear, but sometimes syringomyelic HEMATOMYELIA 283 dissociation persists. The condition of the sphincters improves to some extent. In hematomyelia of the lowest cervical segment, in addition to the spastic paralysis and muscular atrophy of the upper extremities and sen- sory disturbances of syringomyelic type, there are also oculo -pupillary disturbances, viz. myosis, narrowness of the palpebral fissure. When only the eighth cervical and the first dorsal segments are involved, the sensory disturbances occupy a longitudinal band at the inner border of the entire limb and the atrophy is limited to the muscles of the hands. When the seventh, sixth and fifth cervical segments are affected, the sensory dis- turbances are on the external border or on the anterior surface of the limb; the atrophy affects the muscles of the shoulder, arm and forearm. When the hemorrhage occurs in the upper cervical segment, paralysis of the phrenic nerve and of the diaphragm and consequently difficulty of respira- tion will ensue. In hemorrhages of the thoracic cord there will be spastic paralysis of the lower limbs, paralysis of the abdominal muscles, and in- volvement of the sphincters. When a hemorrhage occurs in the lowest part of the cord, viz. in the conus medullaris, there will be a special symptom-group which will be described in "Diseases of the Conus." If the seat of the hemorrhage is in one half of the cord, the clinical pic- ture will be that of Brown-Sequard's paralysis. It consists of a loss or diminution of sensations on the side opposite the lesion and of a motor paralysis on the side of the lesion; these motor and sensory disturbances are distributed in the portions of the body below the lesion. Course, Termination, Prognosis. — Death may occur in a few hours or days. In the majority of cases improvement follows. The prognosis can- not be made before the acute stage has subsided. When the hemorrhage is in the cervical cord, the outlook is very serious because of the involvement of the respiratory centers. Disturbance of the sphincters may lead to cystitis and ascending nephritis. Even in the most favorable cases complete recovery can never be expected. Diagnosis. — In Syringomyelia paralysis, muscular atrophy and sensory dissociation are characteristic, but a sudden onset with a very marked paraplegia are only met with in hematomyelia. Acute Myelitis will be recognized by absence of atrophy, and if the latter is present, it is usually slight. Besides, in myelitis the onset is not so abrupt and the paralytic symptoms are predominant. In meningeal hemorrhages there is a very intense pain in the back along the nerves, the spinal muscles are rigid, but no sensory dissociation. Treatment. — At the onset absolute rest is necessary. Immobilization 284 CAISSON DISEASE of the body is advisable in order to avoid another hemorrhage. The patient should lie on his side. Counter-irritants are to be avoided, as they may lead to ulcerations. Every effort should be made to avoid secondary infections. Ergot had been recommended. In the chronic stage massage, warm baths and systematic exercises (see Treatment of Spastic Paraplegia) should be applied. HI. Caisson Disease (Divers' Paralysis) Divers or workers in caissons, being under the influence of high at- mospheric pressure while at work, present nervous disturbances when they return to the surface. Pathology. — The most constant microscopical changes found in al- most every case are: congestion of brain and cord and internal organs in acute cases, softening in chronic cases. In the incurable cases of long stand- ing, in which the condition remains permanent until death, lesions of typical chronic myelitis are the usual findings. In one case that came under my observation the patient lived three months. The autopsy showed softened areas in the gray matter and in the posterior and lateral columns. Ascending and descending degenerations could be traced. Leyden reported a rupture of the dorsal cord. Etiology.— The immediate cause of the disorder of the nervous system is the lessened atmospheric pressure when the workers return from the caisson, but the primary factor is the increased atmospheric pressure beneath the surface. Symptoms. — Shortly after the return to the surface and after a pro- dromal stage, consisting of pain more or less severe in the large joints in the muscles, especially in those of the back, also in the epigastrium and sometimes over the entire body, a paralysis occurs. The most frequent form of this is paraplegia, but sometimes hemiplegia is observed. The pain is of a neuralgic character and it may be so severe that it doubles up the patient, hence the common term " the bends." The paralysis attacks more frequently the lower extremities than the upper. Sensations are always disturbed: there may be total anaesthesia in the limbs and at the same time intense pain. The onset and the character of the paralysis is very similar to that of transverse myelitis. If we take into consideration the frequent involvement of the sphincters (retention and constipation) and the sensory disturbances, the resemblance to myelitis will be complete. The involve- ment of the motor and sensory apparatuses may be complete or only partial or else unequally distributed. There may be no relationship between the objective sensory disturbances and the degree of paralysis; CAISSON DISEASE 285 either may be affected without the other. In some cases there may also be vertigo, headache, vomiting, slight confusion, convulsions and double vision. Prostration is present in severe cases. In fatal cases deep coma, irregular respiration and symptoms of cardiac paralysis announce approach- ing death. As a not infrequent occurrence may be mentioned small perforations of the ear-drums or such a congestion of the inner ear as to give the picture of Meniere's disease. Aphasia has been occasionally observed. Ecchymosis of the skin is also an occasional occurrence. Pathogenesis. — There are two views for the explanation of the symp- toms. According to one of them, the so-called gaseous theory, the blood while under high pressure becomes overcharged with gas (oxygen and carbonic acid). When the surface is reached, the gas attempts to escape through the lungs. In the meantime the superfluous gas circulates in the blood in bubbles, and may either form emboli or escape through the vessel walls into the surrounding tissues and consequently produce con- siderable pressure. The spinal cord suffers the most, because besides being situated in a hermetically closed cavity, it has a slow return circula- tion (because of a large number of plexuses). According to the other view, there is a congestion followed by stasis. The high pressure drives the blood from the periphery to the internal organs, especially to the nervous system. The blood vessels of the latter having no support from counter- pressure remain dilated. A paralysis of their walls takes place. When the atmospheric pressure is diminished, the paralyzed vessels cannot follow and stasis will be the result. Prognosis. — An attack may last from several hours to six days. When the symptoms are pronounced, death usually ensues. When the paralytic and other symptoms are mild, improvement and even recovery may follow. In some cases the myelitic symptoms persist indefinitely. Treatment- — It is that of myelitis (see above). Prophylaxis is the most important part of the treatment. Bad physical health, diseases of the kidneys and heart, alcoholism, obesity and hunger are all contra- indications for subjecting one's self to high atmospheric pressure. The return to the surface should be done in caissons supplied with locks, so that the reduction of pressure be made very gradually. Haldane and Boycott {Jour, of Hygiene, Cambridge, 1908) have laid out the following rules for gradual decompression: when a diver has been working at a depth of 144 feet for ninety minutes, he should ascend at once to 50 feet, then rest for ten minutes. From that time on he should come up 10 feet four times in succession with intervals of ten, twenty, thirty and thirty- five minutes, after which he leaves the water. SYRINGOMYELIA IV. Syringomyelia Syringomyelia is characterized by a formation of a cavity or cavities in the spinal cord. Pathology. — The macroscopical aspect of a syringomyelic cord is often quite characteristic. It may be flat, soft or fluctuating. Its cervical portion, the usual seat of the lesion, is unduly enlarged. A transverse section will reveal the presence of a cavity ; the latter may be single or multiple. It is mostly situated in the cervical segment, it may also occupy the entire length of the cord. Most frequently it is found in the posterior commissure and posterior cornua. In some cases it extends so far backwards that it destroys the posterior roots at their entrance into the cord. The white matter (lateral columns) becomes involved Fig. 94. — Hydromyelia of the Upper Dorsal Cord. (Striimpell.) when the cavity has destroyed the gray substance. The cavity may extend into the medulla. A large cavity may open into the central canal. Sometimes the entire pathological process consists of a primary enlarge- ment of the central canal. The latter constitutes hydromyelia. The contents of syringomyelic cavities is a fluid analogous to the cerebro-spinal fluid. Microscopically a gliomatous formation is noticed, in the midst of which lies the cavity. This tissue consists of glia cells and glia fibers. The cavity is due to a softening and collapse of the center of the gliomatous tissue. The normal nervous elements affected by the latter are naturally destroyed. The tracts of nerve-fibers degenerate according to their directions. Cavities may be also the result of inflammatory processes within the cord or of hematomyelia (see this chapter) . In such cases, instead of the characteristic elements of glioma, the cavity is sur- rounded by sclerosed tissue. These two forms, while clinically they may present the typical symptoms of the disease, are nevertheless not the SYRINGOMYELIA 287 true syringomyelia, which is essentially a gliomatosis and has for basis very probably a developmental anomaly: the nests of glia cells left in the central canal begin through some cause (trauma or others) to proliferate and lead to new formations. Etiology. — Trauma apparently plays an important role, as there are a number of cases on record, in which the symptoms begin to develop shortly after the shock. Guillain called attention to this fact that an infected wound may be the cause of syringomyelia by means of an ascending neuritis. Exposure to cold, exhaustion are also mentioned by some writers among the causes of the disease. The consensus of opinion is that a congenital defect of the central canal is the most frequent cause. Most frequently the disease begins at the age of between ten and thirty. There are also cases with an onset in infancy. Males are more frequently affected than females. Symptoms. — They are: sensory, trophic and motor. Sensory. — They constitute the sensory dissociation characteristic of the affection It consists of loss of sense of pain and temperature with preservation of the sense of touch. The involvement of temperature sense (thermoanesthesia) is a very grave sign. The patients often being un- aware of it. burn their hands. In one of my recent cases the hands and forearms were literally covered with cicatrices as a result of unnoticed burns. It is only accidentally the patient would notice that her hands came in contact with fire or a hot stove. Application of ice to the hands did not give her the slightest sensation of cold; she felt only the contact of a solid object. The loss of pain-sense (analgesia) is usually of the same intensity as that of temperature. The patient just mentioned developed a cellulitis of the palm of one hand and at no time she experienced pain. An incision was made by the surgeon without a general or local anaesthetic, the wound was cauterized and no pain was felt by the patient. She also had a dis- located shoulder on one side and she could not tell how and when it appeared. A close questioning elicited a history of a fall. She therefore did not feel the pain at the time of the dislocation. The characteristic sensory dissociation is not absolute in every case. Pain and temperature senses may be only diminished, or one may be more or less pronounced than the other. The tactile sense in advanced cases may sometimes be also affected. It must be also mentioned that sometimes the thermal sense may be perverted, viz. heat is taken for cold and cold for heat. Another charac- teristic feature of the sensory disturbances lies in their radicular distribu- tion, viz. longitudinal or parallel to the limbs. The upper extremities and the trunk are more frequently involved than the lower limbs. 288 SYRINGOMYELIA Subjective sensory disturbances are usually present. Pain is frequent, especially at the beginning. The sense of pressure is diminished. Astere- ognosis is sometimes observed. The existence of the sensory dissociation can be explained by this anatomical fact, that Gowers' tract, which contains fibers for conducting pain and temperature, originate in the cells of Clarke's column of the opposite side. As they cross the median line they are interrupted by the syringomyelic cavity, the usual seat of which is near the central canal (see Anatomy) . Trophic. — Muscular atrophy is almost constant. The cause of it is the involvement of the anterior cornua by the cavity. It has usually Fig. 95. — Syringomyelia. Deformity of Hands and Fingers, Cicatrices on Some Fingers as a Result of Self Amputation or Painless Ulcers. (Edematous Hands. the form of Aran-Duchenne's type (see Progressive Muscular Atrophy). Here we find the typical onset in the small muscles of the hands, the claw- like hand, the preacher's hand (Charcot). The latter is due to overac- tion of the extensors of the forearm, while the muscles supplied by ulnar and median nerves are paralyzed. The remaining muscles of the upper extremities as well as those of the shoulders follow the atrophy of the muscles of the hands. Later on the muscles of the back, intercostal and SYRINGOMYELIA 289 abdominal and finally those of the lower limbs undergo atrophy. Deform- ities of the feet follow. The atrophy does not always follow the regularity just mentioned. There are great variations in its distribution. The character of this distribution is chiefly radicular. Fibrillary contractions in the affected muscles are constant. Reactions of degeneration or only a quantitative diminution of electrical contractility may be present. Besides the muscles other tissues suffer in their nutrition. The skin may break and leave sores. It may become glossy, covered with vesicles and eruptions, the nails suffer, the phalanges are deformed. Morvan's disease, which is characterized by painless panaritiae, is a variety of syrin- gomyelia: the tendons and terminal phalanges become necrosed, are gradually eliminated and slowly a cicatrix is formed. During this entire process there is no pain. The perforating ulcer on the feet is frequent in syringomyelia. Raynaud's disease is sometimes observed, although it never reaches the phase of gangrene. Marinesco described an oedematous hand (main succulente), which does not pit on pressure, as pathognomonic of the disease (Fig 95). Arthropathies are quite frequent. It may be only hydarthrosis or a destruction of the epiphyses with subsequent dislocation. The bones also suffer in their nutrition. Spontaneous fractures occur. Formation of a callus is slow and difficult. Scoliosis associated with kyphosis is not infrequent. Vasomotor disturbances consist of cyanosis of the extremities, oedema, hyperhidrosis. Motor. — Paralytic symptoms develop slowly and progressively. Spastic paralysis is a frequent symptom. In some cases it is very pro- nounced and contractures deform the trunk and the extremities. In such cases the reflexes are exaggerated and the toe phenomenon will be present. When the posterior columns become involved, tabetic symptoms will be manifested, viz. ataxia, Romberg's sign, loss of reflexes. Various oscillatory movements, tremors, choreiform movements have been observed in some cases. The sphincters are usually not involved. Ocular symptoms are present when the lesion is in the lower cervical region, and in the first dorsal segment: myosis, retraction of the eyeball, narrowing of the palpebral fissure, Argyll-Robertson pupil, and defective sweating. When the cavity extends to the medulla, bulbar symptoms develop. Difficulty of deglutition, paralysis of the vocal cords, irregular action of the heart, facial paralysis, hemiatrophy of the face and tongue, ocular palsies *9 29O SYRINGOMYELIA with nystagmus are then observed. Sometimes the lesion involves the trigeminal nerve; anaesthesia over its area of distribution will be the consequence. Course, Termination, Prognosis. — The disease is essentially chronic and its onset is usually slow. It lasts may years, during which there may be periods of remission, but it is inevitably fatal. Death ensues either from bulbar involvement or from some intercurrent disease (tuberculosis, pneumonia, etc.). Diagnosis. — Progressive muscular atrophy of spinal origin has the same distribution of wasting as syringomyelia, but in the former the characteristic sensory disturbance of the latter is absent. Amyotrophic lateral sclerosis will be also recognized by the absence of sensory dissociation and of trophic disturbances. Syringomyelia presents in some cases tabetic symptoms (ataxia, loss of reflexes, Romberg's sign, arthropathies), but the absence of characteristic ocular symptoms, of sphincter disturbances of tabes will soon decide the diagnosis. Leprosy is characterized by muscular atrophy, sensory and trophic disturbances. The atrophic hand of leprosy resembles that of syrin- gomyelia, but the mutilations of the phalanges in the latter never reaches the extent of those of leprosy. The mutilated toes (rare in syringomyelia), muscular atrophy in the lower limbs (rare in syringomyelia), absence of scoliosis, absence of increased reflexes — are all in favor of leprosy. In the latter disease sensory dissociation is rare. In Morvan's disease the number of painless panaritise is considerable. Sensory dissociation is usually absent. Treatment. — It is only symptomatic The ulcerations will be treated antiseptically. For muscular atrophy massage and electricity are advis- able Care should be taken of the skin in giving massage, as erosions are likely to lead to ulcerations, which are slow in healing Surgical operations should be avoided Internally iodides, arsenic and iron may be given During the last few years favorable results have been obtained from treatment of Syringomyelia with X-rays. Among the most recent con- tributions to the subject may be mentioned that of Allaire and Denes (Gaz. Med. de Nantes Nov., 1912). Morvan's Disease Under this name is desrcibed an affection which has been observed in Normandy and which according to some neurologists can be considered as a clinical variety of syringomyelia, in which, in addition to the anaes- DISEASES OF CONUS MEDULLARIS 291 thesia and analgesia, paretic symptoms with muscular atrophy, there are also multiple painless panarities. However the sensory dissociation is usually absent. The sense of touch is affected as much as the pain and temperature senses. The sensory disturbances are distributed like in neuritis, viz. decreasing from the distal to the proximal end of the limb. VaJlteB/10-5- V. Diseases of Conus Medullaris and Cauda Equina The lower portion of the spinal cord, called conus medullaris, is sur- rounded by bundles of nerve fibers coming from lumbar and sacral seg- ments (cauda equina) The study of this important portion of the cord is com- paratively recent. Anatomically the conus corresponds to the second lumbar vertebra and its fllum terminale begins at the level of the middle of this vertebra. The conus comprises the last three sacral and coccygeal segments. Physiologically conus medullaris is a very important por- tion of the cord. It contains special and independent centers for micturition, de- fecation, erection, ejaculation and for the anal reflex. Diseases of the conus present a special clinical picture which deserves a separate description. Pathology. — All possible lesions of the spinal cord studied on the preceding pages may also affect the conus. We may therefore have those of myelitis, of hem- orrhage, of tabes, etc. Etiology. — Trauma is the most frequent of all causes. In cases of fracture of the lumbar and sacral vertebras, a fragment is likely to press directly upon the conus or the roots. Secondary myelitis will follow. Trauma may also produce a hemorrhage in the conus. A blow over the lum- bar region may produce a traumatic myelitis of the conus. An injury below the second lumbar vertebra will damage only the cauda equina; at the level of the second lumbar will involve the conus and the cauda equina. An injury immediately above the second lumbar vertebra will damage the conus alone. The lowest part of the spinal cord is not infrequently the seat of tumors. The latter may originate in the vertebrae Fig. 96. — Conus Medullaris (Ajter Raymond.) 292 DISEASES OF CONUS MEDULLARIS or in the cutaneous coverings or else be primary (sarcoma, lymphangi- oma, glioma, gumma). Abscess due to tuberculosis of the vertebrae may also involve the conus. Symptoms. Conus. — From the anatomical fact mentioned above, namely that the conus is the center for the third, fourth and fifth pairs of sacral nerves (Fig. 92), also from the physiological character of the conus, viz. that it contains special centers for micturition, defecation,, etc. (see above), there is no difficulty in presenting a clinical picture of Fig. 97 a. — Solid Portion Thermoanesthesia; Shaded, Reversed Temperature Sense. diseases of the conus. The symptoms therefore are (Figs. 97a, 97, 98): 1. Anaesthesia or sensory dissociation of the external genital organs, perineum, anus, inferior gluteal region and the postero-superior area of the thighs (saddle-shaped anaesthesia). It may also extend to the mucous membrane of the genito-urinary apparatus. The tenderness of the tes- ticles is intact. \ 2. Paralysis of the bladder and rectum. There may be incontinence or retention. 3. Involvement of the sexual function and of the sensation of ejaculation. DISEASES OF CONUS MEDULLARIS 293 4. Pain is a frequent but not constant phenomenon. If it is present, it may be of great severity. As to the motor power of the lower extrem- ities it is normal. The reflexes are equally unaffected. The onset of these symptoms is sudden in traumatic cases, slow in cases of tumors. As the conus is immediately surrounded by the nerve bundles (cauda equina), a lesion of one is bound to involve the other and the symp- tomatology therefore is complex. Fig. 97. — Solid Portion, Analgesia. Cauda Equina. — If a tumor is situated below the conus, the main symp- toms will be those of involvement of the roots, viz. pain and objective sensory disturbances. The pain is intense in the lumbo-sacral region and in the limbs, chiefly in the region of both sciatic nerves; it is constant and increased upon the slightest movement. The objective sensory dis- turbances may be only anaesthesia. They occupy the lower extremities beginning from the lower two-thirds of the thighs downward, the perineum, anus and the genito-urinary organs. There is anaesthesia of the mucous 294 DISEASES OF CONUS MEDULLARIS membranes of the urethra, bladder, vulva, vagina. The paralysis is flaccid at the beginning and during the entire course of the diesase. There is a high-steppage gait. Muscular atrophy appears early in the lower limbs. Reactions of degeneration are present. The reflexes Achilles, plantar and anal are abolished, but the knee-jerks are intact. The sphincters are disturbed. Bed-sores are frequently present. Perineum. Splid portion, Anesthesia to touch, pain and temperature. Perineum. Solid portion, thermo-anesthesia; shaded portion, reversed temperature sense. Fig. 98. Course, Prognosis. — In traumatic cases death may follow the injury, but usually the condition becomes chronic. The outlook is never favor- able, but much better in lesions of the cauda equina than of the conus. The involvement of the sphincters is permanent and eventually leads to the consequences described in myelitis. Diagnosis. — It is very important to localize the lesion in the conus or in the cauda equina. In the first case operative procedures are impossible, while in the second case they are indicated. Lesions of cauda equina are usually very painful, the pain radiates to- ward the legs, the symptoms develop gradually. The pain is present be- DISSEMINATED SCLEROSIS 295 low the second lumbar vertebra; flexion of the thigh over the pelvis while the leg is extended provokes pain. The function of the bladder and rectum improves. On the other hand pain above the second lumbar verte- bra, well-marked anaesthesia and especially sensory dissociation, exceed- ingly slight or no improvement at all in the function of the sphincter — are all symptoms of the conus. Treatment. — Not much can be expected from external applications, as counter-irritation, revulsion in traumatic cases. The rational treat- ment is surgical intervention. In cases where hemorrhage is suspected, a puncture in the spinal canal with evacuation of its contents may give immediate good results. In cases of fracture prompt removal of fragments is urgent. Tumors must be equally removed. Before an operation is undertaken, a detailed study of the case must be made as to the localiza- tion of the sensory and other disturbances in order to determine the exact field of the operation (Fig. 92). Epiconus In 1900 Minor called attention to lesions of that portion of the spinal cord which lies immediately above the conus (epiconus) . It is frequently involved in traumatisms and it presents a special symptomatology, viz. (1) integrity of knee-jerks, (2) integrity of the sphincters of bladder and rectum, (3) loss of Achilles reflex, (4) serious and protracted paralysis of the external popliteal nerve. Frequently lesions of the conus and epiconus are associated and not rarely the cauda equina is simultaneously involved. The special symptoms characteristic of each of these portions will enable one to make a diagnosis of the complex cases as well as when each of them is individually affected. VI. Disseminated or Multiple Cerebro-spinal Sclerosis (Insular Sclerosis) Pathology. — The disease is characterized by islets of sclerosis dis- seminated throughout the entire central nervous system. They can be seen also in some of the cranial nerves, viz. optic, olfactory and trigeminal. On transverse section yellowish spots of various sizes can be seen even with a naked eye. Their distribution is very irregular, they do not show any special predilection for any particular tract, but they are frequently found in the white substance of the cord, brain and cerebellum, in the anterior portion of the pons and in the pyramids of the medulla. Histologically 296 DISSEMINATED SCLEROSIS the sclerosed patches present a characteristic picture: the myelin is de- stroyed, but the axis-cylinders are remarkably preserved. The latter fact is probably the reason of absence of secondary degeneration. Sometimes the axis-cylinders are swollen, but they rarely disappear, except in very old cases. The neuroglia tissue in the diseased area proliferates abundantly. The blood vessels of sclerosed patches are sometimes, but not always, found altered (endo- and periarteritis) The cells are usually spared. As to the origin of the sclerotic process, some believe that proliferation of the neuroglia is the initial process, to others the initial lesion is a myelitis: the nerve-fibers are primarily affected and the neuroglia secondarily 4 Fig. 99. — Sclerosis op Pyramidal, Gowers' and Direct Cerebellar Tracts. {Original.) Symptoms. — In view of the disseminated character of the sclerotic lesions, the clinical picture naturally varies from one case to another and depends upon the seat of the sclerotic islets. If the lesion is in the poste- rior columns, symptoms of tabes will be present. If the lateral motor col- umns are affected, spastic paraplegia will be the symptom. If the postero- lateral columns are involved, ataxic paraplegia will be the result. If Gowers' tract is diseased, symptoms of syringomyelia will be observed. If the anterior cornua suffer, muscular atrophy will develop. If the inter- nal capsule has a plaque of sclerosis, hemiplegia will follow As the essential feature of the sclerotic process is its dissemination in various portions and at various levels of the central nervous system, a combination of symptoms of all the diseases mentioned is expected. In DISSEMINATED SCLEROSIS 297 fact there are several clinical forms of multiple sclerosis. They depend upon the predominance of one or another group of symptoms There is, however, a series of symptoms which are very frequently present in this disease and without which a diagnosis of multiple sclerosis is almost impossible. If they are not all always present, at the same time some of them at least will help to decide the diagnosis. They are: 1. Tremor. — It is of a special character: it appears only upon a volun- tary act. It is an "intention tremor." Should the patient, for example, attempt to carry a glass of fluid to his mouth, he is bound to spill it, and the nearer he gets it to his mouth the more the tremor will be pronounced, Fig. 100. — Plaques or Sclerosis in Pyramids and Various Nuclei. (Original.) so that the glass will strike the mouth and the teeth in all directions and he will fail to drink the contents. Writing is one of the first acts in which tremor is manifested: at first all the letters are irregularly, unequally separated and placed at different levels. In a more advanced state writing is illegible and then impossible. The legs, the trunk and the head may also be affected by tremor, but this is more rarely met with. The tremor may be at first unilateral or predominate on one side, later it becomes generalized. Not only upon voluntary acts irregular jerky movements are observed, but sometimes jerky spontaneous acts take place, such as sudden crying or laughing. They occur without any relation to a preceding emotion. 2. Disturbance of Speech. — The patient speaks slowly, pausing between words and between syllables. In advanced cases he accentuates each syllable. It is a scanning, syllabic, staccato speech. Besides, the pronunciation of certain letters is especially defective, viz. b, c, g and r. 298 DISSEMINATED SCLEROSIS As a rule the first words are pronounced distinctly, but soon the speech becomes indistinct. This is probably due to fatigue of the muscles con- cerned in speech. 3. Nystagmus. — It consists of an oscillation of the eye globes, when the patient turns his eyes to an extreme position laterally or vertically. Lateral nystagmus is more frequent than vertical. In advanced cases the least movement of the eye globes will produce a nystagmus. 4. Disturbance of Gait. — It is most commonly paretic and spastic. As soon as the patient commences to walk, the lower limbs become con- tracted, the legs extend over the thighs, the feet over the legs, and adduc- tion is extreme. Moreover the patient has a tendency to walk on his toes, he topples over when he walks quickly.' Sometimes there may be a gait which is spastic and ataxic at the same time. The patient stands with his feet widely separated and soon as he attempts to walk, ataxia is evident and there is a tendency to fall. Incoordination, if present, is of a cerebellar type (titubation) (see Cerebellar Diseases). Oppenheim speaks also of an oscillating gait, characterized by a generalized tremor as soon as the patient takes the first step. In advanced cases the paralysis and spasticity may be so pronounced that walking is impossible. 5. Reflexes are in the majority of cases increased in all four extremi- ties. Ankle-clonus, Babinski's sign, Oppenheim's and paradoxical reflexes may be present. In some cases the knee-jerks may be abolished. The five symptoms just described, and especially the first three, are almost always present in disseminated sclerosis. There are a few others which, while not very frequent, are met with in quite a large number of cases. They are: 1. Visual. — A partial optic atrophy is the most frequent. It may be unilateral or bilateral. In the earlier stages of the disease there is only pallor of the optic nerves, which is unequally distributed in both eyes. A progressive diminution of visual acuity, contraction of the visual field, central scotoma for certain colors are the other ocular changes. Transi- tory visual defect in one or both eyes is quite common. Occasionally palsies of ocular muscles occur. The sixth nerve is more frequently involved than the third. The reflexes of the pupils are very occasionally affected. 2. Apoplectiform or Epileptiform Seizures.— The first are more frequent than the latter. The onset of hemiplegia is accompanied by a loss of consciousness. The paralysis is usually transient, but it may persist. When the paralysis persists, the disease is considered as the hemiplegic form of multiple sclerosis. The hemiplegia may be complicated by aphasia. The apoplectiform attacks have a tendency to repetition. DISSEMINATED SCLEROSIS 299 This latter fact together with the transient character of the attacks makes the resemblance to the attacks in paresis very striking. Indeed errors of such diagnoses have been made. 3. Sensory Disturbances. — Abnormal subjective sensations, as ting- ling, cramps and sharp pain of neuralgic nature, are quite frequent. Headache is not infrequent. Neuralgia is in the domain of the fifth nerve. Hypaesthesia or hyperesthesia are occasionally observed. Anaesthesia is rare. Among the rare symptoms of multiple sclerosis may be mentioned: (1) Vertigo; Charcot observed it quite frequently at the onset; (2) bulbar symptoms, viz. difficulty of swallowing, acceleration of pulse, atrophy of the tongue, palsy with atrophy of facial muscles; (3) muscular atrophy (without RD.), (4) spasmodic and involuntary laughter, (4) mental feebleness, (5) disturbances of the sphincters, (6) trophic disturb- ances of the skin and articulations, (7) glycosuria and polyuria. Forms of Multiple Sclerosis. — Not all cases present the typical picture described above. In some cases the chief symptoms are either absent or very slightly manifest. Charcot called such cases "formes frustes." According to the seat of the lesions multiple sclerosis may be: cerebral, spinal or cerebro -spinal. Each of these may present varieties depending upon the level at which the lesion is found. Thus in the brain it may be: cerebral proper, cerebellar, pontine, bulbar, basal. In the cord it may be: cervical, dorsal, lumbar, sacral, or else according to localization on the transverse section it may be: posterior tract form (ataxic), postero- lateral form (spastic ataxic), lateral tract form (spastic), poliomyelitic form; syringomyelitic form. Oppenheim has called special attention to several uncommon types, viz. cervical, sacral, pseudo-tabetic and ocular. Cervical. — It is characterized by ataxia of the upper limbs, anaesthesia in hands and fingers, astereognosis. Sense of touch alone is involved, but not pain and temperature. The tendon reflexes of arms cannot be elicited. In the lower limbs spastic phenomena are present. Patho- logically there is multiple sclerosis in the posterior columns. Sacral. — Symptoms of bladder and rectum are prominent. Sensory symptoms are present in the anogenital region. Achilles and anal reflexes are absent. The disease runs in remissions. Pseudo-tabetic. — It presents the symptoms of tabes except Argyll- Robertson pupil and optic nerve changes which are different from those in tabes. The disease runs an acute course unlike tabes. In each of these three forms eventually in the course of the disease other symptoms of the main affection will make gradually their appear- 300 DISSEMINATED SCLEROSIS ance, but the symptoms just enumerated will throughout remain most conspicuous. In the ocular form the disease may begin in and remain restricted to the optic nerves for years before other symptoms will begin to make their appearance. Involvement of the ocular motor nerves may be associated. In the cerebral form the predominating symptom is hemiplegia. The latter may develop gradually, usually with remissions and exacerbations, or by apoplectic seizures. Hemiplegia following apoplexy usually dis- appears partially or even totally but has a tendency to recur: after several attacks it may remain permanent. In the basal or ponto-bulbar form the conspicuous symptom will be crossed paralysis. In the cerebellar form, titubation, nystagmus, asynergia are the most prominent symptoms. Course, Termination, Prognosis, — The course of the disease is very irregular. It is usually chronic and progressive, but there may be station- ary periods and even amelioration. Then again some external influence, as a cold, excesses, exertion, traumatism, an intercurrent infectious dis- ease, may aggravate the symptoms. At the onset there may be either exclusively cerebral symptoms (vertigo, headache, apoplectic attack) or only spinal symptoms, especially spastic paraplegia, or else visual symptoms. Prolonged amelioration is rare and recovery is still rarer. The disease may last from ten to twenty years, but also less than two years. Death may result from bulbar paralysis or from some intercurrent disease such as pneumonia. Diagnosis. — Tremor from, mercurial intoxication is similar in character to that of multiple sclerosis, but in the former affection the tremor may appear spontaneously, while in the latter only upon a voluntary act. Paralysis Agitans will be recognized by its passive tremor which usually disappears upon a voluntary act. Hysteria sometimes presents: vertigo, hemiplegia, tremor and difficulty of speech — all symptoms of multiple sclerosis. Buzzard says: disseminated sclerosis in its earlier stages is of all organic diseases most commonly mistaken for hysteria. The presence of sensory disturbances and of spe- cial stigmata will help considerably in making the diagnosis of hysteria. Westphal and others described a form of pseudo-sclerosis, in which except nystagmus all other symptoms of multiple sclerosis are present and from which patients make a complete recovery. The following symptoms are present: speech-defect, slowness in movements of the eyes and facial mus- cles, fixed expression, tremor, plus knee-jerks; psychic disturbances, apathy, sometimes delirium; finally apoplectiform attacks. The patho- DISSEMINATED SCLEROSIS 3OI genesis of the disease is obscure. Nothing of significance could be found at autopsies. However, in StrumpelPs cases a leathery consistence of the brain and a slight degeneration of the crossed pyramidal tract in the cord were found. In cerebellar tumors there may be titubation, nystagmus and optic neuritis, all symptoms of multiple sclerosis, but the persistence of intense headache and vomiting characteristic of tumors, abscence of spastic paralysis, absence of remissions and recurrences will promptly decide the diagnosis. In Paresis there may be disorder of speech, tremor and spastic gait, but the character of these disturbances is so decidedly different from those of multiple sclerosis that an error is hardly possible. The paretic speech is tremulous, but not syllabic. The tremor is constant and independent of voluntary effort; it is a fibrillary tremor evident chiefly in the tongue and lips. Besides, the mental symptoms of paresis will soon help to arrive at a conclusion. Positive Wassermann reaction and cytological examination of the cerebro-spinal fluid will render further aid in establishing the diagnosis of paresis. The hemiplegic form of multiple sclerosis may sometimes be confounded with a cerebral hemiplegia caused by a hemorrhage or softening, but the tremor, nystagmus and scanning speech are absent in the latter. More- over in disseminated sclerosis the apoplectiform attacks are usually transient and prodromal symptoms absent. Etiology. — Traumatism is considered as one of the causes. I have personally observed several cases in which the symptoms began to develop shortly after a severe trauma. Oppenheim observed the disease after intoxications with metals and carbonic dioxide. P. Marie considers in- fectious diseases as a frequent cause. Striimpell believes that multiple sclerosis is a congenital disease. Syphilis (congenital) is also considered by some as a cause. A neuropathic heredity has been traced in some cases. The affection appears usually at the age of from twenty-five to forty-five and rarely in children. Treatment. — Rest, nutritious food, avoidance of exertion and inter- nally iodides, arsenic, silver, cod-liver oil, may be tried, but little can be ex- pected from medications. If syphilis is suspected, salvarsan and mercu- rials may be tried. Electricity should be avoided, for fear of increasing the spasticity. For the latter warm baths, massage and passive movements are advisable. For treatment of apoplectiform seizures see "Apoplexy." Thiosinamin, fibrolysin, tiodine, which is thiosinamin-ethyl -iodide, have been used for absorption of sclerotic tissue. The last particularly has been tried in disseminated sclerosis. W. Murrell {Med. Press and Circular, 1909) 302 TRAUMA OF THE CORD obtained very favorable results. In his cases nystagmus, speech, ataxia have all improved greatly. The drug is administered hypodermically in 0.20 to i c.c. Injections are made daily. In one of his cases Murrell used 134 injections. Local swellings with rise of temperature are ob- served but they disappear soon. X-ray treatment has given satisfactory results to Raymond, Babinski, Marinesco, etc. Tremor, ataxia, speech, writing, also general health, all improved from repeated seances of exposure to X-rays. SECONDARY AFFECTIONS OF THE SPINAL CORD I. Traumatic lesions of the cord (concussion, contusion, sudden com- pression) . II. Slow compression of the cord (tumors, caries of the vertebrae, Potts' disease) . I. Traumatic Lesions of the Cord A trauma of the spinal column, whether it is a fracture, dislocation or else a blow, a shock of any sort (a fall on the feet or on the buttocks) may be followed by an injury of the cord itself. The latter under these circumstances may undergo a concussion, a contusion, a sudden com- pression, laceration or a complete severance. A. Concussion. — The modern investigations tend to prove that mate- rial changes may occur in the cord in so-called concussion. Rupture of small blood vessels, destruction of axis-cylinders, some rarefaction in the protoplasma of cells, have been found in some instances. Symptoms. — Immediately after the accident the following condition may be observed: paralysis of all or only the lower extremities; the reflexes are lost, the sensations are diminished and the sphincters are disturbed. Soon, however, all these symptoms begin to improve and gradually the patient recovers all the functions. The chief characteristic of concussion is its transitory nature. But if in some cases the above symptoms remain, evidences of an organic lesion of the cord are then present. In every case of concussion of the spinal cord, hysteria should be thought of. Charcot had shown long ago that disturbances following railroad accidents, particularly the so-called "rail- road spine," belong to traumatic hysteria. Stigmata of the latter should be looked for in such cases. B. Contusion. — It presupposes a trauma of greater severity and accord- ingly the symptoms are more pronounced. It is, however, difficult to TEAUMA OF THE CORD 303 draw a sharp line between concussion and contusion. In both cases some lesions mentioned above have been found in the cord and in both cases recovery may follow. The main clinical difference between the two lies in the longer duration, in the greater severity and in the greater possi- bility for the symptoms to remain permanent. It is important not to lose sight of this fact that an accident, whether it is a concussion or contusion, may be the exciting cause for development of chronic organic diseases of the cord, provided there is a predisposition or when the latter preexisted in a latent stage. The injury in such cases hastens the progress of the dormant preexisting affections. C. Sudden Compression, Laceration and Severance of the Cord. — Fracture and dislocation of the vertebrae are the causative factors in this condition. Pathology. — The state of the cord is various according to the intensity and the suddenness of the injury. Sometimes the cord at the level of the traumatism is reduced to an unrecognizable soft mass; above and below that level the nervous tissue gradually undergoes degenerative changes: chromatolysis of cells and ascending or descending secondary degeneration of tracts of fibers. Should a secondary infection occur, an abscess is likely to develop in the cord. When the traumatism of the cord is less severe, only a hemorrhage may occur either in the cord itself or in the membranes. Rupture of the roots or of the spinal ganglia may also take place. The secondary changes above and below the injured area will develop like in the first case, but to a less extensive degree. It should be borne in mind that in injuries of the cervical and upper dorsal segments the cord alone may be injured, because the roots at those levels leave the cord transversely. In injuries of the lower dorsal and lumbar segments there is usually a simultaneous involvement of cord and roots, for the latter have an oblique direction. Symptoms. — From the foregoing remarks it can be seen that the symp- tomatology will vary in each case. In injuries of the cervical and upper dorsal portions of the cord there may be only cord symptoms, while in injuries below that level there will be both cord and root symptoms. A fracture or dislocation of the vertebras will produce a deformity of the spine which can be recognized by the usual tests for surgical diagnosis. It must, however, be borne in mind that no deformity may be observable when a fracture-dislocation occurs at the level of the fifth or sixth cervical vertebra. The same may occur in tuberculous, non-suppurative caries of the vertebrae in the lumbar region. In such cases skiagraphy is of great aid. It can accurately determine the nature and the seat of the trouble. As to the condition of the nervous system, there is a distinction 304 TRAUMA OF THE CORD to be made between a simple sudden compression or a severe crushing of a segment of the cord produced by a fragment of the bone or by a dislocated bone. In both cases paralysis will be the most conspicuous symptom. When the cord is severely injured, the paralysis will be flaccid and absolute and the reflexes totally abolished. In simple com- pression, on the contrary, the paralysis is spastic and the reflexes are increased. The sensations will also differ in both cases. In severe injuries, when the cord is totally lacerated or crushed at the point of the injury, the parts below the lesion are totally anaesthetic to all forms (touch, pain and temperature) and even the subjective sensations are abolished. Immediately above the anaesthetic area there is a hyperaesthetic zone which is due to an irritation caused by the traumatic focus. In incomplete injuries, in simple compression pain will be present in the limbs and follow the course of the nerves. It is sharp, lancinating in character. The patient will also complain of cold, heat, tingling or other abnormal sen- sations. The objective sensibility is also disturbed: there is a diminution of all the senses or sometimes a dissociation (see Syringomyelia). In localizing the seat of the lesion it should be borne in mind that the upper border of the anaesthetic area is not at the level of the diseased vertebra, but it is decidedly lower (see Anatomy and Fig. 92). The sphincters are involved. Retention and constipation are frequent. Trophic disturbances are frequent. Bed-sores appear rapidly at points of pressure (sacrum, trochanter, heels). (Edema, eruptions, hydrarthrosis, muscular atrophy, are not infrequent complications. Injuries to the upper cervical region deserve special mention. Death may be sudden (involvement of the phrenic nerve). If the patient sur- vives, there may be paralysis of the muscles supplied by the cervical nerves, bulbar symptoms, radiating pain in the area of distribution of the cervical nerves. Erection of the penis, which may be persistent, is a common symptom in injuries at this level of the cord. In injuries of the lower cervical and upper dorsal segments of the cord, besides the symptoms described in the general symptomatology, there are special ocular signs, viz. myosis and narrowing of palpebral fissures. Course, Termination, Prognosis. — If the cord is totally destroyed at the level of the injury, recovery will never follow: a myelitis will be the consequence, and the patient may die from bed-sores or cystitis with ascending urinary infection. In cases of mild or incomplete compression, ascending or descending degeneration will be the result and the paralytic symptoms are permanent. In injuries of the cervical region death may be instantaneous. Dislocations bear a less grave prognosis than fractures. TRAUMA OF THE CORD 305 In some cases after prompt surgical intervention recovery had been observed. Treatment — When a sudden compression of the cord occurs, the patient must be handled with extreme care, as any additional movement is likely to increase the compression. Surgical intervention (laminectomy) is the only treatment in such cases. As it is sometimes difficult to tell the degree of the damage done, it is advisable to postpone the operation for some days and during that time observe the paralytic symptoms. When there is no tendency to improvement, a prompt operation is indi- cated. No operation is necessary in cases of progressive improvement. In cases of an evident fracture of vertebrae, a prompt intervention is urgent. In order to determine the exact area of the injury in the cord and thus outline the level at which an operation is to be performed, there are two means at our command. First, an exact and thorough study of the sen- sory and motor disturbances (see Anatomy, Fig. 92). Second, an X-ray examination of the spine. Surgeons are divided as to the time of interference: some are in favor of an early operation, others operate not before a few weeks have elapsed after the injury. I have seen favorable and unfavorable results in both cases. The following rules, however, may be kept in mind as a guide. Early Intervention is indicated: when the nervous disturbances may be attributed to a fracture of an arch of the vertebra; when skiagraphy shows the presence of the bullet (in case of fire arms) in the spinal canal; when in case of fracture with dislocation a bloodless reduction of the dislocation has failed and the patient presents signs of a partial lesion. Late Intervention is indicated: when early intervention was not done and the nervous symptoms do not improve spontaneously; when in the course of a partial lesion there is an ulterior aggravation of symptoms (compression by a callus). In cases of total lesion an operation is con- traindicated. The comparative value of early and late operations can be seen from Chipault's statistics: in early operations he obtained 6 per cent, recoveries, 6 per cent, improvements, 8 per cent, with no change and 79 per cent, deaths. In late operations he obtained 8 per cent, recoveries, 27 per cent, improvements, 39 per cent, with no change and 25 per cent, deaths. Some claim that hemorrhages into the spinal canal are not an indication for laminectomy. I have seen favorable results following prompt opera- tions in such cases. As to the irremediable conditions, such as paralysis, spasticity and sphincter disturbances, the treatment is the same as in the systemic diseases of the cord (see those chapters). 306 COMPRESSION OF THE CORD II. Slow Compression of the Cord. Tumors. Caries of Vertebras— Potts' Disease Etiology. — The cord may undergo slow compression from tumors or from deformed and displaced vertebrae. Tumors may originate in the cord itself, in the meninges, in the perimeningeal cellular tissue or in the spine. Displacement of the vertebras occurs frequently in caries of the bony structure of the spine — in Potts' disease. Here the changes in the cord are due not only to the direct pressure of the deformed vertebrae, but also to the tubercular pachymeningitis created by the vertebral disease. Pathology, Tumors. — (i) Those of the cord itself are not infrequent. Thus Schlesinger found that among 302 tumors of the cord 125 were within the cord substance. According to Flatau the relative frequency of extra- and intramedullary tumors is as 2.6 to 1. Tubercle is the most frequent of all. Its favorite seat is in the enlargements of the cord. It frequently coincides either with tuberculosis of the vertebrae or of the lungs. It originates from the blood vessels. It presents a hard mass which may undergo softening in the center. Around it the nervous tissue, which is under pressure, proliferates and secondary degeneration develops. Gummata are not very rare. Gliomatous tumors of the cord have been described in the chapter on Syringomyelia. (2) Meningeal tumors are usually benign in character. They originate on the inner surface of the dura. The most frequent is sarcoma. Other tumors are: psammoma, myxoma, fibroma, syphiloma, tuberculoma, lipoma and echinococci. The latter two develop externally to the dura. (3) In the perimeningeal cellular tissue in addition to the above tumors carcinoma has been ob- served. (4) Tumors of the vertebras are carcinoma and sarcoma. The first is usually metastatic (from cancer of stomach or other organs). The second originates in the surrounding tissue. The vertebrae thus affected are soft; the spinal column is bent and thus compresses the roots. Hydatid cysts are rarely met with in the vertebrae or in the cord; they originate from the intra- or perispinal tissue. The majority are formed in the posterior mediastinum or in perivertebral muscles. They invade the spinal canal through the intervertebral foramina. Exceptionally the vertebrae and cord remain intact. Usually there is scoliosis and compres- sion of the cord and roots; the destruction of the latter is very severe. Early laminectomy is urgent. (5) Tumors may also originate in the roots and in their meningeal coverings. Multiple fibromata are localized in the roots (neurofibromatosis) and they are most frequently found in the nerves of the cauda equina. The posterior roots are oftener involved than the anterior ones. Solitary fibromata are not accompanied by diffuse COMPRESSION OF THE CORD 307 neurofibromatosis; their point of departure is the dura. The most frequent seats of meningeal tumors are the dorsal region and the cauda equina. The state of the nervous tissue in cases of slow compression by a tumor lying externally to the cord is as follows: The cord is flattened or presents a depression. It is pale or else congested. At the level of compression the myelin is broken up, the axis-cylinder disappears; instead of it neu- roglia proliferates and around the blood vessels connective tissue prolifer- Fig. 1 01. — Tubercular Pachymeningitis. A Case of Tuberculoma of the Cord. {Original.) ates; the affected segment is therefore in a state of sclerosis. Above and below this level ascending and descending degeneration develops. The cells of the gray matter at the point of pressure are either in a state of atrophy or completely absent. The meninges are thickened and adherent. Primary tumors of the cord and meninges, even the most malignant ones, do not produce metastasis. When they originate in the vertebrae, meta- stases are not rare. In Potts' disease the destructive tubercular process of the bone (pus and fungosities) perforates the posterior vertebral ligament and reaches the dura; the latter becomes inflamed and proliferates (pachymeningitis), The tubercular masses covering the dura undergo softening, form small purulent collections at first, then later constitute a large abscess. On the other hand the tubercular masses may become organized, then scle- rosed. The thickened dura is found then surrounded by a fibrous mass. Whatever the process may be, the roots and the cord undergo compression. The changes in the cord are identical with those described above. They 308 COMPRESSION OF THE CORD are generally localized at the level of the peridural inflammation with secondary degenerations. Inflammation of the pia-arachnoid is excep- tional and may be due to a toxic infiltration along the roots. The involve- ment of the cord is explained in two ways: (i) mechanical action upon the cord, roots, vessels and lymphatics, and (2) the action of tuberculous toxins. An excellent illustration of the latter possibility is found in one of my cases (/. of Nerv. and Ment. Dis., 1904), in which the cord was found to be entirely free in the vertebral canal and yet most extensive tubercular changes were observed in the cord. A primary parenchyma- tous tubercular myelitis without meningitis, without caseation has been observed (see chapter on Myelitis). The cerebro-spinal fluid is always modified in all cases of compression of the cord. There is frequently an increase of albumen without an increase of cellular elements. Cells of the nature of the neoplasm in the cerebro- spinal fluid have been observed in the course of secondary cancer of the meninges. Symptoms. — A compressing body, whatever its nature may be, may affect the cord and the roots or each individually. As to symptoms of bony involvement (in cases of tumors of the vertebras), they are: local swelling, curvature, oedema, rise of local temperature, tenderness of the vertebrae and spontaneous pain. Root Symptoms. — Irritation of roots and spinal nerves produces pain, which is unusually intense and of a neuralgic type; it is continuous, but presents paroxysms of exacerbation brought on upon the slightest move- ment. As pressure is at work, the nerves are in a state of neuritis. This is the reason of the accompanying trophic (eruptions) and objective sensory disturbances (anaesthesia) in the areas of their distribution. Gradually loss of power and atrophy develop. The patient then presents a painful paraplegia. The latter is frequently observed in carcinoma of the spinal column. When the motor symptoms become pronounced, the pain usually subsides. The seat of the pain is of an important localiz- ing value. It is certain that the pain refers to the seat of the lesion or below it, but never above it. Cord Symptoms. — They consist essentially of motor and sensory paralysis. Gradually but progressively a loss of power becomes established in that portion of the body which lies below the lesion. The paralysis may be flaccid at the beginning and become spastic later or be flaccid or else spastic through the entire course of the disease. Spastic paraplegia is the most frequent. The reflexes are abolished in the first and increased in the second form. In the latter case there may be also: ankle-clonus, Babinski's sign, Oppenheim's and paradoxical reflexes. The objective COMPRESSION OF THE CORD 309 sensibility is usually altered. There may be complete anaesthesia to all forms of sensations or a sensory dissociation of syringomyelic type; finally there may only be a diminution of sensations (hypaesthesia) . Babinski in 1899 and 191 1 (Rev. Neur.) called attention to a form of spastic paraplegia without degeneration of the pyramidal tract. Clinically there is contracture of the legs in flexion, knee-jerks are not exaggerated, but the cutaneous reflexes are so much increased that stimulation of the skin of the paralyzed limb produces rapid flexion (defense movement). Since then the phenomenon has been observed in Potts' disease and it is due to a gradually progressing compression myelitis without marked degenerative changes. Consequently prompt removal of this compression may restore the patient to health. Sicard and Gutmann have recently reported such a case (Rev. Neur., 1912). The defense reflex may be of practical value. The sphincters are most of the time involved. Trophic disturbances are not infrequent. Bed-sores are formed in the points of pressure of the body (sacrum, gluteal regions, trochanter, malleoli). Muscular atrophy is frequent. The general symptoms just described vary according to the segment of the cord involved. Moreover certain regions present special symptoms. Compression of the Cervical Cord. — (a) When the upper segment is involved, pain will be present in the neck and shoulder, which at the same time will be anaesthetic. Paralysis of the muscles covering this region will produce an inability to rotate, flex or extend the head. All four extremities are in a state of spastic paralysis, the upper more than the lower. The sensations of the limbs are also involved. The special symptoms are: slow pulse, paralysis of the phrenic nerve (hiccough, vomiting, disturbance of respiration). The compression is at the level of the first four cervical segments which correspond to the first three cervi- cal vertebrae. (b) When the lower cervical segment is involved there are: pain in the arms, paralysis of all four extremities, muscular atrophy, objective sensory disturbances. The latter present the following distribution. When the lesion is at upper portion of the cervical enlargement the anaesthesia is at the level of the base of the neck. When the lesion is lower, the upper border of the anaesthesia descends to the upper limbs and is distributed in longitudinal bands parallel to the axis of the limbs. The special symptoms consist of myosis and narrowness of the palpebral fissure. When the compression is at the level of the fifth, sixth and seventh cervical roots, the atrophy will affect the muscles of the roots of the upper limbs (Erb's type) : deltoid, biceps, brachialis anticus, supinator longus. 3IO COMPRESSION OF THE CORD scapular muscles. When the eighth cervical and the first dorsal roots are under pressure, the atrophy will affect the muscles of the hands (Klumpke's type) . Compression of the Dorsal Cord. — The pain will be present around the trunk and the upper line of the anaesthesia will determine the level of the compression. The paralysis will affect the muscles of the trunk below the lesion and of the lower extremities. It may be flaccid, especially in complete destruction of the cord, or spastic. In the first case the reflexes are abolished, in the second exaggerated; the latter may be accompanied by ankle-clonus, Babinski's sign, Oppenheim's and paradoxical reflexes. The sphincters may be involved, and the more pronounced the more it ap- proaches the lumbar enlargement. Compression of the Lumbar Enlargement. — Pain in the lumbar region, in the abdomen below the umbilicus and especially along the crural and sciatic nerves; flaccid or spastic paralysis with lost or increased reflexes respectively; (the paralysis is flaccid in cases of complete interruption of the cord; spastic paralysis is in the incomplete lesions); constant involve- ment of the sphincters; anaesthesia in the paralyzed portions of the body — these are the symptoms observed. The last symptom deserves special mention. The upper border of the anaesthesia does not correspond to the level of the compression because of the oblique direction of the roots; it is therefore higher than the point of compression. The cremasteric reflex is lost. Achilles reflex is preserved. Atrophy is not observed in slight compression but marked in deep compression. Compression of the Sacral Cord. — The pain is chiefly in the area of distribution of the sciatic nerve. Paralysis affects the gluteal muscles, those of the posterior aspect of the thighs, of the legs and of the feet. Deformities of the feet are constant and they depend upon the groups of muscles the mcst affected (equino varus, or valgus). The knee-jerk which depends upon the integrity of the second, third and fourth lumbar segments are normal or increased. The Achilles' tendon reflex, which is controlled by the fifth lumbar and first sacral segments, is lost. The sphincters are involved. Anaesthesia occupies the postero-external aspect of the thigh, leg, foot, the gluteal and sacral regions. Muscular atrophy is present in advanced cases. Compression of the Conus Medullaris and Cauda Equina. — This condition is described in the chapter on Diseases of the Conus. Course, Termination, Prognosis. — The disease usually develops slowly and progressively, with vague painful and paretic symptoms. The clinical aspect will vary according to the localization of the tumor or of Pott's lesion. The most frequent form is that of spastic paraplegia, but COMPRESSION OF THE CORD 311 the disease may simulate any form of spinal cord lesion. When the tumor is of syphilitic nature, marked improvement in the symptoms is observed, if specific treatment is energetically administered. In Pott's disease the symptoms of caries of vertebrae may exist long before cord symptoms appear. Sometimes the cord symptoms appear long before deformity is observed. In Pott's disease the prognosis is more favorable when the motor symptoms are unaccompanied by marked sensory changes than when the latter are present. It is unfavorable when fLaccidity of the limbs follows spasticity. Death may ensue from bed-sores, ascending urinary infection cr in case of a malignant tumor (cancer) from generalization of the latter; finally from some intercurrent affection. In Pott's disease the patient may die from extension of the tubercular process to the lungs. Recovery may sometimes follow in Pott's disease, the abscess discharging externally. The prognosis depends greatly upon the segment of the cord involved. When the compression is in the cervical region, rapid death may follow. Tumors of the vertebra? which are almost always malignant, are more serious than tumors situated in the spinal canal. Among the latter the intra-dural are more benign and longer tolerated than the extra-dural. The prognosis in spinal tumors as a rule is very serious if surgery does not intervene. Diagnosis. — In its early period, before the paralytic symptoms are marked, the diagnosis is quite difficult. The pain may be confounded with simple neuralgia (intercostal or sciatic) or lumbago. Charcot said that bilateral pain is frequently of spinal origin. A careful examination of the spine may reveal a deformity and lead to the diagnosis. In cases of vertebral cancer and Pott's disease a deformity of the spine is almost always present. The first is mostly met with in old age; the de- formity is round, not angular; the pain is the predominant symptom and cachexia is marked. Cancer of the spine is usually secondary to cancer of theviscera. The latter is observed in young people; the deformity is angular; pain is present on pressure; there is cutaneous hyperaesthesia; the spine is kept rigid; subjective pain is but slightly marked; tuberculosis of other organs is frequently present. In some cases of Potts' disease there is no deformity of the spine; the latter may be slightly or not at all perceptible. Alquier out of fifteen autopsies has found eight where involvement of the spine could not be ascertained. In such cases rigidity of the spinal column, localized pain and spinal hyperaesthesia to heat will help to recognize the disease. A flaccid monoplegia or a band of anaesthesia indicates root in- volvement, while spastic paraplegia or anaesthesia below the lesion indi- cates that the cord is being compressed. The above mentioned contrac- 312 COMPRESSION OF THE CORD ture in flexion with the defense reflex may be of assistance in determining the degree of involvement of the cord. In the paralytic period the disease should be differentiated from tabes, myelitis, multiple sclerosis, multiple neuritis and hysteria. In Tabes the ocular symptoms and the ataxia will decide the diagnosis. Myelitis (diffuse) develops very rapidly; the motor symptoms appear at the beginning, pain is usually absent. In the transverse form the onset is usually acute, the paralysis is marked and the objective sensory disturb- ances are early. In Multiple Sclerosis the absence of sensory disturbances, the presence of tremor, nystagmus and the special speech are characteristic. In Multiple Neuritis the muscular atrophy appears very early; the nerve trunks and the muscles are tender on pressure ; there is no spasticity and there is usually a history of intoxication (alcohol, lead, etc.). In Hysteria there may be rigidity of the spine with hyperesthesia, but the sphincters are intact. There is no muscular atrophy. The sensory disturbances are marked. Finally the hysterical stigmata, the sudden onset of the paralysis after an emotion, will help to decide the question. Treatment. — In Potts' disease absolute rest and immobilization of the spine with orthopedic appliances are of great benefit. At the same time general hygienic measures with abundant nutritious food, also adminis- tration of iodides, iron, arsenic and phosphates should be kept up. Great care must be taken of the patient's skin, as bed-sores are easily formed, also of the bladder, as urinary infection is frequently the cause of death. If syphilis is suspected, mercurials with iodides should be used. As to surgical means, there are two methods: one consists of breaking the bones and putting them in place (redressement of Calot), the other is laminectomy. The first has given in some cases satisfactory results, but the majority of surgeons report disastrous consequences. A modification of this method, consisting of extension, followed immediately by fixation of the spinal column, has given better results. Laminectomy has been also suc- cessful in some isolated cases. In case of abscess of vertebrae which can be determined from gradually increasing spasticity and contracture of the par- alyzed limbs, also from the fever, the abscess must be exposed as promptly as possible by laminectomy or by a postero -lateral operation. The latter appears to give more satisfactory results. In Arch.f. Klin. Chirurgie, 1909, Wassiliev reports brilliant results by this prccedure and he cites very unfavorable results from laminectomy. If the operation is performed very early when pachymeningitis and cord symptoms have not yet made their appearance, there is hope for curing the patient. When the disease is old and the tuberculosis of the vertebras is cured, laminectomy is indi- COMPRESSION OF THE CORD , 313 cated. Finally the existence or non-existence of the defense reflex (see above) may assist in deciding the question of operative interference. The wisest procedure is to apply first the non-opetative treatment, and if there is no improvement in the paralytic symptoms, an operation should be attempted. Cases of spontaneous recovery have also been reported. In cases of compression by tumors the following considerations may serve as a guide. Cases of primary and isolated spinal tumors without angular deformity and without flaccid paralysis necessitate immediate intervention, especially when there are only root symptoms. It is better to perform a large laminectomy over four or five vertebras than to wait for cord symptoms which cannot always disappear. Tumors accompanied by grave deformities of the spine, tumors that show rapidly developing cord symptoms, present a grave surgical prognosis. In such cases an opera- tion can be undertaken only when an exact localizing diagnosis is made. In such cases the operation will be only palliative. In cases of flaccd para- plegia with loss of reflexes no operation should be performed. As to the seat of operation, the latter should be performed in cases of intra-dural and extra-medullary tumors at the level of the highest nerve-root involved. In view of the oblique direction of the roots emanating from the spinal cord it is important to remember that in an adult in order to determine the number of the roots at the level of a certain spinous process it is necessary to add to the number of the corresponding vertebra: one in the cervical region; two in the upper dorsal region; three in the lower dorsal region (sixth to eleventh vertebrae) ; the lower portion of the eleventh dorsal and the interspinous space immediately below correspond to the last three lumbar pairs. The spinous process of the twelfth dorsal and the space immediately below correspond to the sacral pairs. Below the second lumbar vertebra the cord is no more involved, but the cauda equina is (see Fig. 96). Cancerous vertebrae should of course never be operated upon. The immediate danger of operation lies in shock or hemorrhage or infection. The mortality depends much on the seat and the nature of the tumor. In the cervical region shock especially is to be feared by reason of proximity of the medulla. In the dorso-lumbar region where because of the frequency of diffuse infiltration of the meninges and roots extirpa- tion of tumors is very laborious, hemorrhages are frequent. Moreover the presence of vesico-rectal trouble and of extensive anaesthesia facilitates infection; the mortality therefore is high. In the dorsal region tumors are usually benign and strictly limited; their removal is quite easy. The mortality is not so great as in the other localizations. Tumors of bony 314 • MUSCULAR ATROPHIES origin present a high mortality, hydatid cysts still higher. Almost complete recovery follows removal of fibromata or sarcomata of the meninges. Removal of tumors of cauda equina has given but mediocre results. Before an operation is decided upon it is advisable to try for a short time a thorough course of antisyphilitic treatment. Severe pain in non-operable cases must be controlled by opium. CHAPTER XX MUSCULAR ATROPHIES Classification The chief forms of muscular atrophies are: I. Progressive muscular atrophy of spinal origin (Aran-Duchenne). la. Progressive muscular atrophy of infants. II. Progressive muscular dystrophy (myopathy), which embraces the following subdivisions: (a) Pseudo-hypertrophic type (Duchenne). (b) Scapulo-humeral or juvenile type (Erb). (c) Facio-scapulo-humeral type (Landouzy-Dejerine). III. Primary neuritic atrophy (Charcot-Marie-Hoffman-Tooth). IV. Arthritic muscular atrophy. I. Progressive Muscular Atrophy of Spinal Origin (Chronic Anterior Poliomyelitis) (Aran-Duchenne's Type) Pathology. — The main lesion consists of a gradual degeneration, atrophy and disappearance of the cells of the anterior cornua in the spinal cord. Under the microscope the cells appear small, their NissPs bodies have partly or totally disappeared, the nuclei and nucleoli are either on one side or outside of the cells, the yellow pigment is increased in amount. The number of the cells is considerably reduced. The prolongations form- ing at the periphery of the cord, the anterior roots suffer secondarily: they undergo degeneration and atrophy and appear therefore slender. The degeneration of the roots is continued into the peripheral nerves, but to a lesser degree. The nerve-terminations in the muscles are dis- tinctly degenerated. The muscular tissue also undergoes changes: granular degeneration transforms the fibers into indistinguishable masses, which are gradually absorbed; atrophy is the result. The disease is therefore an affection of the lower motor neurons. In cases with bulbar symptoms the nuclei of motor nerves are involved. The eleventh and twelfth are most frequently affected. The ninth, tenth, seventh and motor portion of the fifth may also be involved. MUSCULAR ATROPHIES 3*5 The above changes in the gray matter are accompanied by proliferated neuroglia tissue. The blood vessels are dilated and thickened. A pri- mary degeneration of the nervous elements of the gray matter is the essential characteristic of the disease. Symptoms. — The onset is characteristic. In the majority of cases the small muscles of the hands are first affected. The thenar muscles, the superficial first and the deep next, are first to be involved. The ball of the thumb then becomes flattened. The other small muscles, the lumbricales, and the interossei follow. The spaces between the meta- carpal bones are deep, the prominences of the bones become marked. Fig. 102. Fig. 103. Progressive Muscular Atrophy, Following Infantile Spinal Paralysis. The first phalanges are in extension, while the other two are in flexion. The hand is claw-like (main en griff e). Gradually the atrophy spreads tojihe muscles of the hypothenar, of the forearm, arm, shoulder, neck, thorax and lower extremities (Figs. 102, 103, 104, 105, 106). On the forearm the flexors are affected before the extensors, the exten- sors of the fingers before the extensors of the wrist. On the arm the atro- phy of the deltoid muscle alters the round contour of the shoulder, the head of the humerus becomes evident. Atrophy of the scapular muscles brings out prominently the borders of shoulder blade; atrophy of the 316 MUSCULAR ATROPHIES serratus projects the posterior border of the scapula like a wing (Fig. 106). Atrophy of other muscles of the extremities of the neck and abdomen will also change the contour of the bones and produce corresponding functional disturbances. The muscles that frequently escape rapid wasting are: the latissimus dorsi, triceps of arm, highest portion of trapezius, sterno- mastoid. The functional disturbances are first manifested in the hands. The patient's attention is attracted to an awkwardness in performing fine acts, such as writing, buttoning. When the atrophy advances, the patient becomes helpless and his attitude is quite characteristic when he attempts to do anything at all: he turns, tries to help himself with the entire body in an effort to do what the hands alone should have done. When the muscles of the neck are affected, the head cannot be held erect. Atrophy of intercostal muscles interferes with respiration, which then becomes exclusively diaphragmatic. Atrophy of the muscles of the lower extremi- ties will interfere with standing and walking. The other characteristic signs of the disease are : fibrillary or fascicular contractions of the affected muscles, also reactions of degeneration. The first can be observed almost continuously, and the least stroke intensifies them. They are probably due to an increased irritability of the cells of the anterior cornua in the cord. The reflexes are usually diminished for want of muscular tissue. There are no changes in sensations or in the sphincters. Course, Termination, Prognosis.' — The description as given above is met with in typical cases, but the onset as well as the successive stages of the disease may vary. Although it is slow and progressive, neverthe- less it may be arrested in its development at a certain period or for a certain time. It may last then many years, as long as fifteen or twenty. In other cases it may suddenly assume a subacute development and advance rapidly. Usually the patient dies from some intercurrent disease, as pulmonary tuberculosis. The disease may have also an ascending course and involve the nuclei of the medulla; the patient will die then from bulbar palsy. The disease may also present an atypical onset: instead of beginning in the muscles of the hands, it may affect first the muscles of the arm or of any other portion of the body. The atrophy may begin in the lower extremities. In such cases the anterior tibial muscles are first affected and the atrophy gradually extends to the muscles of the thigh, etc. When the disease begins in the medulla, atrophy of the tongue appears first: difficulty of moving the tongue in every direction, flatness of its muscula- ture, fibrillary tremor, difficulty in pronouncing words are the chief MUSCULAR ATROPHIES 3 J 7 symptoms. Other muscles follow: orbicularis oris, muscles of the soft palate and of the vocal cords. When this occurs, the speech becomes more and more indistinct, the voice is low, coughing is difficult, blowing with the lips is impossible. When the muscles of mastication (masseter, temporals and pterygoids) become affected, the patient cannot chew his food and the mouth does not close, swallowing is very difficult, food regurgitates through the nose and there is danger for food to enter the larynx; saliva dribbles constantly from the mouth. When the nucleus of the pneumogastric becomes involved, dyspnea and rapid heart action develop. When the disease begins in the medulla, the patient cannot live long and dies before atrophy appears in the limbs. The prognosis in all varieties of the disease irre- spective of the mode of onset is always unfavorable. Diagnosis.— Aran-Duchenne's disease is so typ- ical in its onset, in its course and its symptomatology that in the majority of cases the diagnosis is not difficult. Thus it will be readily differentiated from muscular atrophies occurring in the course of other organic diseases, as myelitis, tabes, hemiplegia, etc. From myopathies it will be distinguished by the onset, fibrillary twitching and RD. In syringo- myelia the sensory disturbances will decide the question. Multiple Neuritis will be recognized by the presence of pain and paralysis, also by the absence of fibrillary contractions. Hypertrophic cervical pachymeningitis presents a history of pain and the reflexes are usually in- creased. Arthritic muscular atrophy affects the muscles of hands, Arms Shoul- . • . . ders, Face and Legs. only in the vicinity of a joint which was previously diseased. Etiology. — Very little is known of the causes of this affection. Exposure to cold, trauma, exertion, have been reported as usual causes. Two cases came recently under my observation in one of which carrying daily large cakes of ice in the left hand for a period of three months was appar- ently the only accountable cause for the beginning of the atrophy in the same hand. The other patient sustained a blow with an iron bar over his shoulder. There was no erosion or any other apparent lesion. Shortly afterwaids he noticed that he could not thread a needle and had difficulty Fig. i o 4. — Progres- sive Muscular Atrophy Showing Involvement 3*8 MUSCULAR ATROPHIES in buttoning his vest. Atrophy began to develop in the most character- istic manner. Infectious diseases, intoxications, syphilis, have been also mentioned as causative factors. An attack of acute anterior poliomyelitis in child- hood may become later in life the point of departure of progressive muscular atrophy (Figs. 102, 103). The disease usually develops in middle lif e, more frequently in men than in women. In a few isolated cases it has been observed in several mem- bers of the same family (Ormerod, Holmes, Gee). Fig. 105. — Progressive Muscular Atro- phy Confined to Upper Arms, Shoulders and Thorax. Fig. ic6. — Atrophy of the Muscles of the Scapula (Same individual as Fig. 105). Treatment. — Massage and electricity (especially galvanism) are prac- tically the only means of treatment. They may prevent rapid wasting. General hygiene, nutritious food and avoidance of stimulants are indis- pensable. Mercuiials and iodides, tonics — arsenic, strychnine, phosphorus, may be useful in some cases. Progressive Muscular Atrophy of Infants. — Werdnig (189 1) and Hoffman (1893) described a variety of progressive muscular atrophy in infants which pathologically presents the picture of the disease just described, but clinically differs from it. The characteristic symptoms are as follows: In the midst of good health the very young child begins to lose power. Soon develops a distinct paretic condition of the limbs and of the musculature AMYOTROPHIC LATERAL SCLEROSIS 319 of the trunk, so that the child cannot sit up; the joints show a looseness; the muscular atrophy is generalized and symmetrical; there is some adi- posity; the reflexes are absent, the limbs are flaccid; there are no sensory disturbances, no involvement of the sphincters or of the cranial nerves. What particularly characterizes this affection is its family character and its short duration, viz. from two to three years. The disease is progressive and death takes place from some intercurrent disease, such as pneumonia, etc. The pathogenesis of the disease lies probably in a prenatal disturbance of the nutrition of the cells of the an- terior cornua which eventually ends in their destruction. The disease is probably allied to Oppenheim's Myatonia congenita. Amyotrophic Lateral Sclerosis (Charcot's Disease) Pathology. — The main lesion consists of atrophy of the cells of the anterior cornua of the cord and a sclerosis of the pyramidal tracts. This double lesion is found frequently in the corresponding portions of the Fig. 107. — Amyotrophic Lateral Sclerosis. (Original.) medulla, pons and brain. Thus a degeneration of the pyramidal tracts may be seen besides the cord in the subcortical tissue of the motor area, in the internal capsule, in the pons, in the medulla. The cells of the motor nuclei in the medulla may be affected at the time as the cells of anterior cornua. The nucleus of the hypoglossus is more frequently 320 AMYOTROPHIC LATERAL SCLEROSIS affected; the facial, the pneumogastric and the motor portion of the trigeminus are not infrequently involved. The large pyramidal cells of the cortex of the motor area of the brain may be equally affected in advanced cases (Fig. 107). The changes in the cord are more pronounced than in any other portion of the central nervous system. In view of the changes in the cells of the cornua, their prolongations, anterior roots and peripheral nerves must naturally suffer. Finally the muscles, in which the nerves terminate, also undergo degeneration and atrophy. Taking a general view of the subject, one may say that in amyo- trophic lateral sclerosis two systems of motor neurones are affected, viz. the cortico-bulbo-spinal and the peripheral or upper and lower. The first originates in the large pyramidal cells of the motor cortex, descends through the centrum ovale, internal capsule, crua, pons to terminate around the motor nuclei of the medulla, and especially around the ce]]s of the anterior cornua in the cord. The second neurone consists of the cells of the anterior cornua (or their equivalents in the medulla, namely the cells of the motor nuclei) and of their prolongations, viz., anterior roots and peripheral nerves. The degeneration of upper and lower motor neurons is often simul- taneous and apparently independent of each, other. The cause of this primary degeneration is still unknown. It is, however, certain that old organic diseases of the central nervous system may start the pathologic, process. Such a case I have observed (Amer. Med., 1903). As to the histological changes of the cells see the preceding chapter and of the fibers see the chapter on Primary Lateral Sclerosis. Symptoms. — The main features of the disease are: muscular atrophy and spasticity. They may both begin simultaneously or one follows the other. As the nuclei of the medulla may also be affected, there will be in addition to the above symptoms also bulbar disturbances. Cord Symptoms. — The lesion of the cells of the anterior cornua pro- duces muscular atrophy and as the disease is chronic, the condition in every respect is that of Aran-Duchenne's Muscular Atrophy. A detailed description of it is given in the preceding chapter. The lesion of the pyramidal tract produces spasticity. The latter is particularly observed in the lower extremities. The condition is that of spastic paraplegia. The gait is difficult because of the rigidity and the loss of power. The reflexes are increased. Ankle-clonus, Babinski's, Oppenheim's and paradoxical reflexes are also observed. When the upper extremities are involved, the arms are adducted to the trunk, the forearms pronated, the fingers in a claw-like position. The most frequent form of MUSCULAR ATROPHIES 32 1 the disease is: spasticity with weakness in the lower limbs and Aran- Duchenne's atrophy in the upper limbs, but there may be other varieties according to the portions of both systems of neurons most markedly involved. Bulbar Symptoms. — They are identical to the labio-glossolaryngeal paralysis of Duchenne. A detailed description of the latter was given in the chapter on bulbar paralysis, in which its relation to amyotrophic lateral sclerosis was also discussed. (See also preceding chapter.) Cerebral Symptoms. — In advanced cases in which the cortical cells and the subcortical white substance are involved, mental symptoms are observed. The patient is emotional and mentally feeble. There is a tendency to laughing and crying. Course, Termination, Prognosis. — As the disease may affect at the beginning each system of neurones (see above) separately, the onset may be various. When the upper extremities are first affected, the muscular atrophy will be the initial and remain as the most conspicuous symptom. When the lower extremities are first involved, spastic paraplegia will be the most noticeable symptom. In some cases the disease may begin in the medulla and remain as the most prominent symptom for a long time, as one of my cases shows {New York Med. Jour., 1907). The disease may last for years, if the bulbar symptoms are late to appear. The prognosis is invariably bad. Diagnosis. — From Progressive Muscular Atrophy of spinal origin the disease will be differentiated by the addition of spasticity and modi- fication of the reflexes. Multiple Sclerosis is recognized by its typical speech, nystagmus, tremor. Amyotrophic lateral sclerosis with bulbar onset should be differentiated from diseases of the medulla (see this chapter). Etiology. — Cold, trauma, exertion are mentioned as causes. It is a disease of middle life. In the case reported by me the boy was only fifteen, and the disease was far advanced. (Amer. Med., 1903.) Treatment. — It is only palliative. Proper hygienic and dietetic measures, and avoidance of stimulants should never be neglected. To relieve the spasticity, gentle massage and warm baths will be useful. Iodides and mercurials may be tried. Electricity and strychnia must be avoided. II. Progressive Muscular Dystrophy. Myopathies Pathology. — The lesion is in most cases confined to the muscular tissue. The nervous system is in the largest majority of cases intact. 322 MUSCULAR ATROPHIES In some cases atrophic (not degenerated) cells were found in the anterior cornua of the spinal cord. This condition may be secondary to the muscu- lar changes. The peripheral nerves, also some fibers in anterior roots, have been found altered in a few cases. The muscles are pale and atro- phied; their individual fibers are reduced in size and number; they be- come irregular and the striation disappears. The connective tissue be- tween the fibers is proliferated to a considerable extent (hyperplasia) Fig. 108.— Pseudo Hypertrophic Paralysis. (Climbing on his four.) and contains sometimes a large amount of fat cells. In some cases the muscular fibers instead of being atrophied are hypertrophied. In this form some fibers hypertrophy at first, but soon they atrophy and dis- appear, they are substituted by increased connective tissue or fat. Atro- phic and hypertrophic varieties depend on the amount of connective tissue or fat respectively. The disease is a primary muscular affection independent of changes in the cord or nerves. Symptoms. — (a) First Variety: Pseudo-hypertrophic Type. — It oc- curs usually in young children. The first sign of the oncoming trouble is noticed in walking. The child falls often and gets tired easily. Upon examination the size of the legs is found to be out of proportion with their MUSCULAR ATROPHIES 323 function; while the calf -muscles are enormous, their feebleness is strik- ing. On palpation the muscles are either firm or soft according to whether connective or fat tissue respectively predominates. Of other muscles of the lower extremities the extensor of the knees and the gluteus maxi- mus are particularly enlarged. As the atrophy spreads to the pelvis and abdomen, the patient has more and more difficulty in walking; in the latter act the pelvis moves markedly up and down; in rising from a seat the patient has to place first his hands on his thighs and help himself to get up. A very characteristic sign of this affection is the manner in which the patient leaves a recumbent position. He turns to either side with effort in his first attempt to sit up; then he tries to put himself in a kneel- ing position. After the latter is reached, he places his hands on his knees and then after several attempts he gradually gets up, supporting him- self continuously with his hands, which slowly slide up the thighs until the erect position is obtained. (He climbs on his four, Fig. 108.) The atrophy invades gradually the upper portions of the body. At this time lordosis of the lumbar spine and protrusion of the abdomen develop. The patient's gait is similar to that of a duck. As the disease is essentially progressive, the muscles of the thorax and of the upper extremities are gradually involved: the bones become prominent, the shoulder-blades move abnormally with each movement of the arms. The triceps of the arm is usually hypertrophied, the biceps is atrophied. The muscles of the hands usually escape. The loss of power has this characteristic feature that it develops simultaneously and in proportion with the muscular wasting. The re- flexes decrease also parallel with the degree of atrophy. Fibrillary con- tractions are absent. The electrical reactions are only diminished quan- titatively, but there is no RD. Sensations are intact. The sphincters are not affected. (b) Second Variety: Scapulo -humeral or Juvenile Type (Erb). — In this form the myopathy makes its first appearance in the muscles of the shoulder and upper arm, especially trapezius, serratus magnus, biceps and triceps. There is no hypertrophy. Gradually the atrophy extends to the muscles of the upper and lower extremities, also of thorax and pel- vis. The disease occurs in early youth. Electrical reactions are only diminished but there is no RD. There are no fibrillary contractions. (c) Third Variety : Facio-scapulo-humeralType (Landouzy-Dejerine). — The atrophy affects first the muscles of the face and particularly the orbicularis oris. In a well-developed case the mouth is enlarged, the occlusion of the eyelids is incomplete; the facies is expressionless. The patient is unable to blow, whistle and pronounce labial letters. 3 2 4 MUSCULAR ATROPHIES Gradually the myopathy extends to the shoulders and arms. The rest of the musculature is invaded later in the course of the disease. The affection makes its first appearance in adults. Electrical reac- tions are only diminished, but present no RD. Fibrillary contractions are absent. Other Varieties of Myopathy.— The three forms of myopathy just described received their names from the mode of onset or from the pre- dominant seat of the atrophy. Observations show that there may be other varieties, as for example Zimmerlin's, in which the myopathy com- mences in the upper musculature of the thorax and arm; Eichorst's, in which the femoro-tibial muscles are first affected. It is useless to multiply the names. Goweis in 1902 described a form to which he gave the name of "Distal Myopathy." It is characterized by atrophy confined to the muscles below the elbow and the knee. Course, Termination, Prognosis. — Irrespective of the variations in its onset, the disease is essentially progressive in character. There are periods when the atrophy arrives at a certain stage of development and remains stationary for a certain time, but finally resumes its insidious course. As to its duration, observations show that the earlier in life the atrophy begins, the shorter is its duration. The pseudo-hypertrophic patients rarely reach above twenty. The ultimate results of all the cases are absolute loss of power and confinement to bed. Death usually results from some inter- current disease (pneumonia, tuberculosis, etc.) . Life is in danger when the respiratory muscles and the diaphragm are involved. Diagnosis. — Generally speaking there is no special difficulty in mak- ing a diagnosis of myopathy by bearing in mind the essential features described above. There are, however, cases which present some obstacles in arriving at a positive opinion as to whether they belong to the myopath- ics or to progressive muscular atrophy of spinal origin. The fibrillary contractions and reactions of degeneration which used to be considered by the elder writers as pathognomonic of the latter, have been recently found to be not so constant. Moreover, there are undoubtedly cases in which some groups of muscles present the symptoms of one form, and other groups of muscles show symptoms of the other form of muscular atrophy (so-called mixed type) . The pathological records of some cases also favor this view. The old border fine can no more be considered as sharply defined in every case. There is a certain relation and affinity as to the origin between various forms of atrophy. However, for practical purposes it is a good plan to differentiate them according to the special symptoms described. Etiology. — Little is known as to the causes of this affection, except MUSCULAR ATROPHIES 325 that it is met not infrequently in members of the same family. It is probably due to some congenital defect. Treatment/ — Proper hygienic and dietetic measures are beneficial. Lo- cally massage and electricity are indicated. In administering the latter it should be borne in mind that muscles undergoing an atrophic process get easily fatigued. Violent electric contractions should therefore be avoided. Galvanism appears to have a better effect than faradism. As some cases of this affection present at the same time diminished or enlarged thyroid glands, administration of thyroid extracts or perhaps extracts of other ductless glands may be tried. m. Primary Neuritic Atrophy (Charcot-Marie -Hoffman-Tooth.) Pero- neal Type of Progressive Muscular Atrophy Pathology.— The lesion consists of an involvement of the peripheral nerves, of the spinal ganglia, of the posterior roots and posterior columns in the cord and of the cells of the anterior cornua. The most pronounced changes are those of the posterior columns, especially of Brudach's. The latter is similar to that of tabes. Hoffman believed that the original lesion is in the peripheral nerves (neuritis), which has an ascending course and eventually involves the posterior roots and the posterior columns of the cord. Histologically the cells are in a state of chromatolysis and finally atrophy. The fibers undergo the same degenera- tive process with subsequent sclerosis, as in tabes. The peripheral nerve- trunks show neuritis. The muscles are atrophied. Symptoms. — The disease begins in the majority of cases in the lower extremities, and especially in the peroneal group of muscles (extensors) and in the small muscles of the feet. The legs appear thin, emaciated. The contrast between the size of the legs, especially of their lower thirds, and the rest of the body is striking and quite characteristic. The gait is difficult (steppage). Foot-drop is evident. Deformities of the feet are very frequent (pes varus, equinus or equino-varus) . Gradually the small muscles of the hands and of the forearms are invaded. A claw-like hand develops. The atrophy rarely involves the proximal ends of the extremi- ties. It may extend and affect the muscles of the trunk and of the face, but this is rare (Fig. 109). Fibrillary contractions are observed and reactions of degeneration are sometimes present. Diminished response to faradism and galvanism is common. The reflexes are usually diminished or abolished. Sensory disturbances (objective) are rare. Pain and parassthesia may occur. In my case (see above) the disease began with excruciating 326 MUSCULAR ATROPHIES pain in the feet, but when the atrophy became pronounced, it gradually disappeared. Trophic disturbances (ulcerations) may occur. The sphincters are intact. Course, Termination, Prognosis. — It develops very slowly and may last an indefinite number of years. Life is not threatened. Remissions occur. Death usually occurs from some intercurrent disease (pulmonary or other) . Diagnosis. — In multiple neuritis the extensors of the legs are also frequently involved, but the sudden or rapid onset, the paralysis preceding the atrophy, the tenderness of the nerve- trunks, the eti- ology of the disease, will establish the diagnosis. From myopathies the disease will be differentiated by the appearance of the atrophy in the 5 distal ends of the limbs, by the fibrillary contractions and RD. Dejerine and Sottas have recently described a disease presenting some anal- ogy with primary neuritic atrophy. It is called "Interstitial hypertrophic neu- ritis." It is met with frequently in several members of the same family and it appears early in life. It is characterized by a muscular atrophy of the distal ends of the limbs, but it also presents a tabetic symptom -group, viz. ataxia, lancinating pain, Argyll-Robertson pupil. There is also a scoliosis or kyphosis. The periph- eral nerves which are accessible to pal- pation are markedly thickened (hypertrophied) . Pathologically it pre- sents an interstitial neuritis in the limbs, also a certain degree of degenera- tion in the posterior columns of the cord. Etiology. — The disease is of an hereditary character. Several members of the same family are frequently affected. Men suffer more than women. It is a disease of the second half of childhood. In one case reported by me (Jour. Nerv. and Merit. Dis., 1903) the disease developed after a pro- longed exposure to cold. Treatment. — Massage and electricity, orthopedic appliances and tenotomy, are all the means we have at our command. Fig. 109. — Primary Neuritic Atrophy. MUSCULAR ATROPHIES 327 IV. Arthritic Muscular Atrophy. — It accompanies acute and chronic inflammations of the joints. It affects the muscles in the immediate vicinity of the joints and rarely extends to the entire limb in the acute and subacute forms of rheumatism. In chronic rheumatism the atrophy extends to the muscles lying at a distance from the diseased joint. In the first case there may be only one special muscle affected or else the group of muscles surrounding the joint. In the second case the atrophy becomes diffused and involves the entire limb. Generally speaking the degree of atrophy is in proportion with the duration of the arthritis and ankylosis of the joint. When several joints are involved, the generalized muscular atrophy may give the impression of Aran-Duchenne's amyotrophy. The differential diagnosis will show that the electrical reactions show only a quantitative diminution, but no reactions of degeneration. Some authors believe that the inflammation of the joint spreads to the neigh- boring nerves and the atrophy is then due to the neuritis. Others think that the atrophy is caused by ischaemia produced by the swollen joint. Others attribute the atrophy to a functional inactivity. Finally a reflex action may play some role. There are some path- ological evidences that the sympathetic system may be the underlying cause of arthritic muscular atrophy. The disease tends to disappear when the joint gets well. Myatonia Congenita or Amyotonia Congenita In 1900 Oppenheim called attention to a heretofore unknown con- dition which is characterized by a generalized muscular weakness with- out paralysis. It is congenital in nature. The cranial nerves are not involved. The intelligence and general health are intact. The condition has a tendeny to improve. The symptoms are noticeable at an early age, viz. from birth. When the little patient is seated, the trunk bends forward, forming a marked kyphosis. Placed on his feet he is not only unable to stand alone, but, even when supported, his legs give way under him. That there is no paralysis can be seen from this fact, that when the patient is on his back he is able to move his arms and legs. The weakness (atonia) affects also the ligaments of various articulations, so that, the latter can be placed in hyperextension and in all possible positions. The muscles are soft but there is no atrophy. In the majority of cases the electrical reactions of the muscles are normal. In grave cases there is a diminished response or no response to electrical stimulation. The skin presents a certain thickness, reminding one of a myxedematous 328 MUSCULAR ATROPHIES skin. The knee-jerks are usually lost. General sensations and special senses are normal. The sphincters are not involved. The mentality is usually intact. The muscular atony is usually more marked in the lower extremities, but it may equally affect the thorax, neck and upper extremities. The muscles of the face, tongue, eyes, deglutition are not involved. The growth of the bones appears to be normal. As to the nature of the condition little is known. The two autopsies placed on record (Spiller, Univers. Penna. Med. Bull., 1905, and Baudoin, Semaine Med., 1907) reveal nothing characteristic for pathogenic in- Fig. no. — Myatonia Congenita. When Sitting Trunk Falls Forward. ferences. However, in some cases an increase of connective tissue and a considerable amount of fat between the muscle bundles were observed. Oppenheim believes that the disease is due to an arrest of development of the muscle-fibers. Some writers are inclined to believe in a disturb- ance of function of some ductless gland. Berti, among them, considers amyotonia congenita as a variety of congenital myxcedema. While Amyotonia congenita presents several features distinct from Myopathies, viz. the time of life at which it appears, the general involvement of all the muscles, absence of deep reflexes, the tendency to improvement, nevertheless the pathological findings which show that there is no essential difference in the two conditions, the not very infrequent occurrence of the same symptoms in both maladies— all these facts tend to show that Amyotonia congenita probably belongs to the great group of Myopathy ,[in which so many variations from the typical forms really exist. SPINAL MENINGITIS 329 The treatment consists of good hygienic and dietetic measures with massage and electricity. A natural tendency for improvement exists in this affection. In my two cases the improvement was remarkable. Myotonia Atrophica Rossolimo (Nouv. Iconogr. de la Salpetr., 1902) gave this name to an affection which was described before as Thomsen's disease with muscular atrophy by Noques, Tirol and Hoffman. It is characterized by mus- cular atrophy and a slow relaxation of muscles after voluntary con- traction. The atrophy is characterized by its distribution, viz. it affects the muscles of the face (myopathic face) , the sterno-mastoid, the vasti of the thighs and the extensors of the feet. The extensors and flexors of the forearm, the masseter and temporal muscles are sometimes affected. As to the relaxation of muscles after contraction the stronger the latter the longer is the former. The electrical reactions show a dimin- ished response to faradism. The knee-jerks are absent. Several members of the same family have been found to be affected, males more frequently than females. It appears usually at the age between fifteen and thirty, although Grund has recently reported cases of myotonia atrophica at the age of 47 (Munch. Mediz. Wchnschr., April, 1913.) The disease is essentially progressive. Pathologically it resembles Mycpathy as far as the muscles are concerned. Steinert found also degeneration of the posterior columns in the cord. Myotonia atrophica is probably one of the multiple varieties of Myopathy. DISEASES OF SPINAL MENINGES Spinal Meningitis. — There are three forms of this affection: I. Acute spinal meningitis. II. Chronic spinal meningitis. III. Hypertrophic cervical pachymeningitis. I. Acute Spinal Meningitis This form of meningitis is usually a secondary affection. In the majority of cases it accompanies an acute cerebral meningitis. In some cases it may be primary and strictly localized to the membranes of the cord. Pathology. — An incision of the membranes will reveal the presence of a serous or purulent exudate and adhesions between the pia, arach- noid and dura. The membranes are hyperemic. This condition may be localized cr else extend over the entire cord. The exudate is particu- larly marked on the posterior aspect of the cord and it covers also the cord and the roots. In the cerebro -spinal form the lesion is confined only to the pia and arachnoid. In the tubercular form there may be 33° SPINAL MENINGITIS either isolated tubercular nodules or else infiltrations in the pia around the blood vessels. When the meningitis is secondary to a disease of the vertebrae (caries), the lesion may be confined to the dura, but it may also extend to the pia; purulent formation is found then between the spine and the dura. The periphery of the cord is always in a state of myelitis, more so than the cortical tissue in cases of cerebral meningitis. Symptoms. — The onset is sudden. General malaise, fever and chills are the first signs. Pain along the spine is the most prominent symp- tom. The latter is extremely tender to touch and the patient immobil- izes his spine for fear the least movement will bring on pain. The pain radiates around the thorax and to the limbs, also to the pharynx and larynx. It is continuous, but it presents also paroxysms of exacerbation. The entire surface of the body is hypersesthetic. The neck and back are rigid. Kernig's sign is present. It consists of an inability to ex- tend the legs when the patient is in a sitting position. The reflexes are increased. The sphincters are involved. Vaso-motor disturbances are frequently observed, viz. erythemata and sweating. In cervical menin- gitis the following important symptoms are observed: vaso-motor dis- turbances of the face, pupillary disturbances, vomiting, dyspnoea, dysphagia . If the patient does not die, he enters into a phase of paralysis, which is an indication that the spinal cord became involved (meningo-my elitis) . The limbs are then totally paralyzed, flaccidity is complete. The re- flexes are lost, anaesthesia takes the place of hyperaesthesia and the sphincters are relaxed. For details see chapter on Myelitis. Course, Prognosis.— While death may occur on the second or third day, viz. during the first phase of the disease, it may, however, be pro- longed. In the majority of cases the termination is fatal, but recovery is possible, although very rare. Death is due to the involvement of the medulla. In recent years it has been shown through the study of cerebro- spinal fluid that there are benign and curable (ambulatory type) forms of meningitis and that they are more frequent than they were supposed to be in former years. Tubercular meningitis and meningo-my elitis may have an acute course, but as a rule they are slow and progressive. Infectious menin- gitis, primary or secondary, has generally a rapid course. The major- ity of the subacute form of meningo-myelitis are either tubercular or syphilitic. Diagnosis. — From acute myelitis the disease will be differentiated by the pain which precedes the paralysis. The latter, together with anaes- thesia, are much later symptoms in meningitis than in myelitis. SPINAL MENINGITIS 33 1 Tetanus will be recognized by trismus and paroxysms of contractions. Meningeal hemorrhage, which also produces pain and rigidity, is not accompanied by fever. Etiology. — Infection with a point of departure in the neighboring tissues is the chief cause of acute spinal meningitis. The necessary factor is a microorganism. Streptococcus and pneumococcus are the most frequent ones. Diseases of the spine, trauma, bed-sores, infectious diseases, septicemia, tuberculosis and syphilis, compression of the cord — are all causative factors in meningitis. Treatment. — Applications of ice to the spine, cauterization of the latter, hot baths, administration of iodides and mercurials when syphilis is suspected, hypnotics, sedatives, also lumbar puncture, have been advised (see also Cerebral Meningitis). II. Chronic Spinal Meningitis It is a common secondary lesion in various diseases of the cord. Occasionally it may follow an acute meningitis. As a primary affection it is found in senility, in lead intoxication, in alcoholism. Pathologically it presents thickened membranes. The condition of the meninges second- ary to cord diseases is given in each chapter. The symptoms of the primary chronic meningitis are analogous to those of the acute form except that they are less marked and may remain latent. There is pain in the back and in the neck. The rigidity of the spine is but slightly marked. Fever is absent. As a special form of chronic meningitis the following was first described by Charcot and Joffroy: in. Hypertrophic Cervical Pachymeningitis Pathology. — The lesion consists of a thickening of the dura in the cervical region of the cord. The thickened inner layer of the dura adheres to the pia and the outer to the endosteum of the spinal column. The cord surrounded by the dense fibrous tissue is naturally compressed. The roots, the posterior especially, are equally compressed. The elements of the cord and the nerve-fibers of the roots undergo degeneration and atrophy. For histological details of the latter see any of the acute or chronic diseases of the spinal cord. Symptoms. — The first phenomenon is pain in the neck. It is con- tinuous and presents paroxysms of exacerbation. It radiates to the occipital region, shoulders and upper extremities. It never follows 33 2 SPINAL MENINGITIS the course of nerve-trunks, but it is diffuse. Sensations of tingling, of numbness, also trophic disturbances (herpes) are also present in the painful areas. The entire spinal column is tender and the neck is held rigid. Objective sensibility is altered, hyperesthesia is present at first, but later when the nerves degenerate, anaesthesia will be observed. All these phenomena are due to compression of the posterior roots. Gradually (when the anterior roots become involved) the painful phase of the disease gives place to the period of paralysis and atrophy. They appear first in the upper extremities. The atrophy affects mainly the muscles supplied by the median and ulnar nerves. Flexion and adduction of the forearm and hand are impaired. The overextension of the wrist, extension of the basal and flexion of the middle and last phalan- ges gives the hand a special position (preacher's hand) that is characteristic of the disease. The atrophy of the thenar and hypothenar muscles is marked. RD. is present. Pain usually disappears at this period of the disease, but rigidity of the spine persists. Objective sensory disturbances, hyperesthesia, hypaes- thesia or anaesthesia are present in the affected areas. In a more ad- vanced stage of the disease (when the cord itself begins to suffer) the lower extremities become involved. Spastic paralysis with increased knee-jerks, ankle-clonus, Babinski's sign, Oppenheim's and paradoxical reflexes will be present. But there will be no atrophy in the paralyzed muscles. At this phase of the disease the sphincters become involved and the objective sensory disturbances are more marked; the latter are radicular, viz. they follow the course of the nerve- trunks. Bed-sores may also develop. The cerebro-spinal fluid presents lymphocytosis. Course, Termination, Prognosis. — The disease is very slow in develop- ment and lasts years. The painful period lasts usually several months. Death results either from the extension of the morbid process to the medulla (bulbar symptoms) or from an infection caused by extensive bed-sores, by purulent cystitis or else from an intercurrent disease (pul- monary tuberculosis, etc.). Arrests in the course of the disease, or even considerable amelioration (perhaps cure) of the symptoms have been reported. Diagnosis. — Potts' disease, meningeal tumors, syringomyelia may sometimes be confounded with hypertrophic cervical pachymeningitis. The deformity and pain produced by pressure upon the cervical spine in Potts' disease are characteristic. Meningeal or vertebral tumors have their special signs (see these chapters) . The diagnosis with syringo-myelia is sometimes difficult. The typical sensory dissociation especially will en- able most of the time to diagnose syringomyelia. SPINAL MENINGITIS 333 Etiology. — Injuries, prolonged exposure to cold, lead intoxication, alcoholism, syphilis, are all considered as possible causes of the disease. As a primary affection hypertrophic cervical pachymeningitis is rare. Most frequently it is symptomatic of syringomyelia, of meningo-myelitis of syphilitic or tubercular nature. It may participate in pachymeningitis of the base of the brain. Treatment. — Local counter-irritation and cauterization, warm baths, internal administration of mercurials and iodides may be tried. Little can be expected from this treatment. Massage and electricity may improve the muscular atrophy. CIRCUMSCRIBED SEROUS SPINAL MENINGES This condition of the meninges has been described only recently. It consists of a circumscribed accumulation (pseudo-cyst) of cerebro-spinal fluid in the sub-arachnoid cellular tissue. It is probably due to an inflam- mation and subsequent adhesions of the meninges which thus produce an increased secretion of the fluid in a circumscribed space. This pseudo- cyst is found in the dorso-lumbar region on the posterior surface of the cord. As to the Etiology, traumatism, bacterial infection, a toxic process, are considered the main factors. Symptoms. — Horsley insists upon unilateral pain at the onset. Hyper- aesthesia is marked Anaesthesia follows the hyperesthesia. It may affect all forms of general sensibility (touch, pain and temperature). Pressure at the level of the roots is painful. The anaesthesia may vary daily as far as its distribution is concerned. Paraplegia sets in insidiously, first in one leg and then in the other. It is usually spastic but flaccidity may follow spasticity. The reflexes are increased, the toe phenomenon is present. Ankle-clonus is frequent. Atrophy may be present. The sphincters may be involved. (Edema has been observed. The prognosis depends upon the early recognition of the affection and upon the promptness of surgical intervention. If abandoned, complete paraplegia with its usual complications (urinary infection, bed- sores, etc.) will develop. Laminectomy with puncture of the cyst, followed by washing it out with i : 2000 of bichloride is the only therapeutic procedure. HEMORRHAGES OF SPINAL MENINGES They may occur either on the outer or inner surface of the dura. Extra-dural Hemorrhages. — They usually occur in traumatism, frac- 334 INTERMITTENT CLAUDICATION OF THE SPINAL CORD ture, dislocation of the spine, penetrating wounds, caries and carcinoma of the vertebrae and meningeal syphilis. Intra-dural Hemorrhages. — The preceding factors may produce this form of hemorrhage but less frequently. Infectious diseases, acute and chronic inflammation of the meninges (especially syphilis), instrumental deliveries, hemorrhages in the cranial cavity— are all causes of spinal intra-dural hemorrhages. Symptoms. — They are: pain in the spine radiating to the limbs, rigidity of the spinal column, paresthesia, hyperesthesia. Paralysis is the most characteristic symptom. It is incomplete at first, but in two to three days becomes complete. Anaesthesia is also slight in the beginning, but becomes more and more pronounced later. The sphincters are usually involved. The knee-jerks are at first diminished, but later change according to the degree of compression. The course of the disease depends upon the amount of blood in the spinal cavity. Absorption of the blood may take place in slight cases and the symptoms then gradually disappear; recovery may take place in a few weeks. Frequently, however, complications set in: secondary meningitis, infection from bladder, bed-sores, etc. The prognosis depends upon the seat of the hemorrhage. If it is in the cervical region, death may be rapid from bulbar symptoms. If hemorrhage is abundant, cerebral anemia and syncope may follow. Generally speaking, the disease lasts from two to three months and usually leaves motor, sensory and sphincter disturbances. The diagnosis can be made chiefly from lumbar puncture. The treatment consists of absolute rest in bed. The patient should be placed on his side in order to avoid congestion of the meninges and lungs. Application of ice and counter-irritation at the level of the suspected hemorrhage are the next indication. Pain must be relieved by the usual means. Finally repeated lumbar punctures are very important. INTERMITTENT CLAUDICATION OF THE SPINAL CORD In 1906 (Reane Neurologique) Dejerine called attention to the follow- ing symptom group. In the midst of apparently good health the patient notices that while walking, one of his legs gets more easily tired than the other, but after a rest this fatigue disappears. For a long time, sometimes years, he will complain of this unilateral weakness, but sooner or later the other leg will become similarly affected. Gradually the interval after which the weakness of the limbs appears, becomes shorter and INTERMITTENT CLAUDICATION OF THE SPINAL CORD 335 shorter and a typical spastic paraplegia develops. In the beginning of the intermittent spinal claudication disturbances of the sphincters are observed, either retention or incontinence, but especially imperative micturition, frequently also sexual impotence. The patellar tendon and Achilles reflexes are exaggerated, more particularly in the limb whose function is disturbed. Ankle-clonus may be absent. The toe phenomenon is frequent. The terminal spastic paraplegia may take place in months or in many years. The latter is the most frequent oc- currence. In rare cases the condition may run an acute course, viz. the paraplegia develops shortly after the onset of the claudication. The diagnosis is to be made from intermittent claudication of peri- pheral origin. In the latter affection the condition is at first also unilateral; the walking brings on rapidly a fatigue, but there is pain which is frequently very intense; it is a cramp that affects the entire limb. But the reflexes are normal, there are no disturbances of the sphincters. On the other hand we find here the limb cold, and after a walk it becomes cyanosed, the cyanosis disappears after a rest. The most important sign of the clau- dication of peripheral origin is the disappearance of normal pulsations in the dorsalis pedis artery at first and then in the posterior tibial artery; sometimes the pulsation cannot be felt in the popliteal and femoral arteries. Skiagraphy often shows an atheromatous condition of these arteries. Recoveries may occur because of the supplementary collateral circula- tion, but most frequently the condition ends in gangrene Its etiology is therefore an endarteritis and atheroma; it occurs in cases of alcohol- ism, gout and syphilis, or else it may follow an acute endarteritis after typhoid fever in young people. A different condition is found in intermittent spinal claudication. Here the reflexes are exaggerated, the toe phenomenon is frequent and sphincter disturbances are present. Finally the arterial pulsations are normal and there are no vasomotor disturbances in the affected limbs; the patient feels no pain while walking but only a heaviness in the limbs. The Pathogenesis of the affection lies in a very slow process of arteritis which leads to a narrowness, of the lumen of the arteries supplying the segments of the dorso-lumbar region of the spinal cord. As to the etiology of the affection, infectious disease, such as grippe (case of Dejerine and Poix) may be the cause. The most frequent etiological factor is syphilis. The anti-specific medications, such as Salvarsan, mercury and iodides, when promptly administered may im- prove the condition or at least delay for a very long time the oncoming terminal Spastic paraplegia. CHAPTER XXI SYPHILIS OF THE NERVOUS SYSTEM The microorganism discovered by Schaudinn and Hoffmann as being the cause of syphilitic lesions is known under the name of spirochasta pallida. The syphilitic poison, whatever its intimate nature may be, appears to have a special predilection for the nervous system. Its effect upon the latter may be manifested in two different forms. In one of them, to which tabes and paresis belong, the lesions were supposed to be until recently not the direct and immediate result of syphilitic infection, but a late and secondary development (degeneration), against which the anti-syphilitic treatment is powerless. They are the "parasyphilitic affections" of Founder. Our former view concerning the relationship of syphilis to tabes and paresis must now be modified. Noguchi and Moore (J. of Exper. Med., Feb. i, 1 913) have found the spirochaetse in the brains of 12 paretics, and Noguchi found them in the posterior columns of a tabetic. In the brain, they are seen more numerous in the cortical than in the white matter, they are scattered in groups between the cells and neuroglia fiber. They are rare in the vicinity of the blood-vessels and not found in the walls of the latter. These findings have been corroborated by Levaditi, Marie and Bankowsky (Presse Med. No. 36, 1913). The parasyphilitic diseases are therefore very probably true syphilitic affections. The other form with which we will be exclusively concerned here is characterized by distinctly specific lesions due to the direct effect of syphi- lis. They are amenable to antisyphilitic treatment, especially at the beginning. Syphilis may affect the cerebro-spinal system at any period of its development. Generally speaking, however, cerebral syphilis occurs in the tertiary period, while spinal syphilis is an early occurrence, viz. in the first or second year, even as early as in a few months. In a syphilitic individual a trauma, excesses, especially of alcohol, and exhaustion are apt to be the exciting causes for the development of syphi- litic cerebro-spinal disturbances. 336 SYPHILIS OF THE NERVOUS SYSTEM 337 SYPHILIS OF THE BRAIN Pathology. — Meninges, brain tissue and blood vessels are affected. In the meninges the specific gummatous formation, which is the char- acteristic lesion of syphilis, may be diffuse or circumscribed. Gummata may be of various sizes. As soon as they are formed, they become a source of irritation and inflammation to the neighboring tissue. They Fig. hi. — Syphilitic Meningitis of the Bas l • nE Brain. (Flatau, Jacobson, Minor.) On the left are seen strong adhesions between the dura, pia and underlying cerebral tissue. extend into the latter along the sheaths of the blood vessels of the pia mater. Adhesions of the meninges and softening of the nervous tissue follow. The most frequent seat of gummata is the base of the brain from the chiasma to the pons. There the gumma starts from the pia and rarely involves the dura. The optic and oculomotor nerves, also the large basal arteries, especially the middle and anterior cerebral, are usually involved. When situated on the convexity, they are usually found in the frontal or parietal regions. The obliteration of the blood vessels will produce softening of the brain tissue. When the lesion is diffuse, the 338 SYPHILIS OF THE NERVOUS SYSTEM meninges are covered with a thick gelatinous exsudate. Histologically a gumma consists of a granulation tissue formed by proliferation of connec- tive tissue and endothelial cells. It is very vascular and presents casea- tion in some places. Round cells are in abundance along the adventitia of the blood vessels. Gummata primarily developed in the brain are rare. Diffuse gum- matous infiltration of cerebral tissue may also occur. It may affect all the three membranes or only the pia-arachnoid. The dura mater when in- volved becomes very thick and forms adhesions with the other membranes and brain tissue (gammatous pachymeningitis. The cortex is usually involved, the lesion will be then a syphilitic meningo -encephalitis. The cranial nerves, which are frequently involved in basal meningitis, may also be affected independently. In such cases there is a round cell infiltration in the epineurium; thick processes are sent out between the bundles of the nerve-fibers. The latter being compressed undergo even- A Fig. 112. — Large Gumma in the Pons. (Flatau, Jacobson, Minor.) tually degeneration or atrophy. The condition is therefore at first an interstitial neuritis. The chiasma, the optic and the oculo-motor nerves are most frequently affected. The fourth, fifth, sixth, seventh and eighth are next in frequency. The remaining four nerves are almost never involved. Syphilis has a special predilection for the blood vessels of the base of the brain, although specific arteries may be encountered in any portion of the brain. The lesion consists of a small gummatous formation or infil- tration in the walls of the blood vessels. Microscopically there is a cell hyperplasia in the lining endothelium of the vessel and the fenestrated membrane. The effect of the arterial lesion will be either thrombosis or rupture of the vessel. Softening of cerebral tissue or hemorrhage will be the consequence, provided collateral circulation is not established. Such is the case with occlusion of the terminal arteries in the internal SYPHILIS OF THE NERVOUS SYSTEM 339 capsule, basal ganglia, crura, pons and medulla and the nuclei of the cranial nerves from the third to the twelfth inclusive. In congenital syphilis the encephalitis is of the sclerotic form, rarely- softening. All the lesions enumerated may be observed in the same case. In fact multiplicity of lesions is frequent and characteristic of syphilis of the nervous system. Symptoms. — The foregoing remarks indicate that the clinical picture will vary with the localization of the lesion. A syphilitic basal meningitis, a diffuse meningitis of the cortex, a circumscribed gumma in various portions of the brain, a generalized syphilitic arteritis — all these forms naturally have their special symptoms. Irrespective of the form or of a special localization, cerebral syphilis presents a prodromal period charac- terized by the following symptoms. Headache is the most constant and the earliest phenomenon. Its essential feature is to present exacerbations, especially in the evening or at night, and to be deep seated in some part of the cranium. During the day it is dull or may disappear entirely. The headache yields with a remarkable facility to antispyhilitic remedies. Vomiting is quite frequent. It may occur without food in the stomach. Vertigo is also a common symptom. The general condition of the patient changes. He becomes apathetic, somnolent, languid, loses his appetite and in weight. In cerebral syphilis, due to a generalized specific arteritis, the symp- toms will be those apoplexy caused by arterial lesions, especially by arterio-sclerosis. Thrombosis is the usual occurrence. In such cases hemiplegia, aphasia develop slowly after a prodromal period and without loss of consciousness. The special character of these phenomena is that they improve rapidly and may even disappear under the influence of the specific treatment. In some cases there are brief and fugacious attacks of aphasia without paralysis of the extremities. Sometimes there is only some difficulty of articulating. In other cases the attack consists of peculiar sensations (tingling, numbness, etc.) on one side or in one limb. Syphilitic arteriti may affect the large trunks of the convexity or at the base. In such cases the entire artery may become obliterated or else aneurisms may form. A rupture of such a vessel will produce a sudden coma and death. Generalized or focal epileptiform convulsions are not infrequent. If they are frequent and not controlled, psychic mani- festations become evident, viz. slow cerebration, loss of inhibition, out- bursts of anger. In cerebral syphilis of meningeal origin the manifestations are dif- ferent from those of the previous form. When the meningitis is acute, 340 SYPHILIS OF THE NERVOUS SYSTEM the patient, after a period of intense headache, becomes stuporous. Hebetude is seen in his acts and speech. Coma may follow. In other cases the condition may be reverse; delirium, excitement, generalized convulsion take the place of the depression. In basal meningitis, during this acute stage, very frequently palsies of cranial nerves develop. The third nerve palsy (ptosis, strabismus) is the most common occurrence; it may be total or partial; the first is rarer than the latter. When the optic nerve is involved, there may be amblyopia, complete blindness or hemianopsia. Ophthalmoscopic examination shows the following possibilities: optic neuritis, choked disc, optic atrophy. The fourth and sixth nerves are sometimes in- volved. Uhtoff collected only twenty-seven cases out of 150. The same can be said of the fifth nerve; the sensory portion is more frequently involved than the motor. The seventh and eighth are occasionally affected and usually together. The facial palsy is usually of the peripheral type. It may be associated with a hemiplegia or the opposite side (crossed paralysis). It occurs when the facial never is affected with other struct- ures in the pons. The special feature of these palsies lies in their transitory character and in the fact that prompt administration of antisphilitic treatment is followed by marked improvement or even complete recovery. In exceptional cases the acute stage instead of improving becomes prolonged: the patient enters rapidly into a profound coma and never regains consciousness. Among the less frequent symptoms of basal menin- gitis may be mentioned: polydipsia, polyuria and glycosuria. In acute meningitis of the convexity epileptic convulsions will figure prominently instead of paralytic symptoms. They may be generalized or unilateral. Charcot believed that cortical epilepsy is one of the most frequent consequences of syphilis of the brain. In chronic syphilitic meningitis, in addition to paralytic or epileptic phenomena, there will be psychic disturbances. They consist of men- tal feebleness associated with emotionality, amnesia, apathy with semi- somnolence, automatism, impairment of memory and in advanced cases of dementia. Delusions and hallucinations may also be present. In cases of circumscribed gummata the symptoms depend upon their localization. Whether at the base, on the convexity or in the sub- stance of the brain the symptomatology will be that of tumors of the brain (see this chapter). Syphilitic Arteritis While every case of syphilitic meningitis is associated with some degree of endarteritis, there are nevertheless cases which present symp- toms of arteritis without meningitis. SYPHILIS OF THE NERVOUS SYSTEM 341 They occur most frequently according to Gowers within the first two years after the infection and in young individuals. Symptoms. — Headache exists for a long time before other symptoms. It is usually not very severe. It is diffuse, it disappears and reappears; it is increased by mental effort. Short attacks of dizziness are common. Sleep is usually poor. Psychic symptoms are especially conspicuous, viz., incapacity for work, mental fatigue, feebleness of intelligence, somno- lence, periods of excitement, irritability. Besides, a typical neurasthenic symptom-group is not infrequently observed in the course of syphilis. The symptoms are, generally speaking, those of neurasthenia from other causes, except the following features: In ordinary neurasthenia there are only paresthesia?, such as numbness, heaviness, etc. In syphilis there is pain, which is worse at night. Head- ache of neurasthenia is usually diurnal, in syphilis nocturnal. Antisyphilitic remedies will relieve the specific headache, but not the headache of neuras- thenia. Finally the posterior Wasserman reaction in syphilis and the negative one in neurasthenia will decide the diagnosis. Apoplectic seizures of various degrees occur; they may occur even dur- ing sleep. The seizure is usually transitory. Sudden impediment or loss of speech may occur very frequently. Transitory hemiplegia or mono- plegia or else a sensory disturbance on one side of the body may occur and last even a few minutes. The symptoms of endarteritis just enumerated are due to a general thickening of the intima, producing a partial occlusion of the blood vessels. These changes may eventually terminate in thrombosis of the diseased vessels and produce a permanent loss of function. Most serious symptoms occur when a basilar artery becomes occluded; a fatal termination is almost inevitable. Syphilitic Hemiplegia Closely associated with the subject of syphilitic arteritis is syphilitic hemiplegia. The latter is of very frequent occurrence and therefore of practical importance. Etiology. — It affects particularly individuals who have not been treated or imperfectly treated, also in cases of severe infection. It occurs at any period of the disease, but especially between three and fifteen years after the infection. It may coincide with the secondary mainfestations of syphilis. Men are more frequently affected than women. x\s predisposing causes may be mentioned: mental and physical fatigue, violent emotions and various infectious diseases other than syphilis. 34 2 SYPHILIS OF THE NERVOUS SYSTEM Hereditary syphilis plays a large part in syphilitic hemiplegia; it may occur as early as the age of five months and as late as thirty-three years. The classical infantile spastic hemiplegia has in a great many cases a syphilitic origin. Pathology.— The chief lesion is syphilitic arteritis. The syphilitic virus has a special predilection for the cerebral arteries. The syphilitic process either narrows or obliterates completely the lumen of the blood vessel, producing either a temporary cerebral ischemia or softening, or else a destruction of the arterial wall followed by a hemorrhage. Among all the arteries of the brain, those of the base are the seat of predilection. The terminal branches are less frequently affected. In order of frequency the following arteries are involved: Sylvian, anterior cerebral, basilar trunk, posterior cerebral, finally the branches of the Sylvian artery. The main syphilitic lesion, gumma, may form in the arterial walls round and hard tuberosities, but more frequently the syphilitic process consists of plaques, of diffuse infiltration. The artery loses its transparency, the plaques form a thickened prominence within and without the artery. The lumen is gradually being narrowed so that it presents but a small fissure, or it may be totally obliterated by a secondary process, viz. throm- bosis. In other cases the artery is dilated at a certain distance and thus aneurisms are formed. The latter are observed especially in the basilar and Sylvian arteries. Microscopically the following condition is found: thickening of the inner wall, leucocytic infiltration; the elastic inner membrane is fragmented, torn; the adventitia is always altered and the same leucocytic infiltration is observed. The muscular layer is the least involved. The process that follows is the terminal state. It consists of chronic arteritis, of a sclerotic transformation: the entire vascular wall became a dense fibrous tissue. Such vessels are easily ruptured. The cerebral tissue in view of the damage to the arteries undergoes either softening or suffers from hemorrhage. The first is the most frequent. The lesions in hereditary syphilis are precisely the same as just described. As to the spirochete, there are very few cases reported in which they were found in the nervous tissue. Symptoms. — Premonitory symptoms may appear weeks, months or years before the hemiplegia. Headache is constant, it is of nocturnal type and is ameliorated by specific treatment. At the same time the individual becomes irritable and depressed; all sorts of paresthesias appear. Two special symptoms SYPHILIS OF THE NERVOUS SYSTEM 343 make their appearance, viz. deafness or vertigo, also ophthalmic migraine. Following an effort and sometimes without cause an apoplectic attack occurs. It may be only slight with or without aphasic symptoms, or else it may be typical. Irrespective of the degree of involvement, the attack is usually transitory and lasts but a few days. On the other hand it may repeat itself in the same day or every few weeks or months. When later on the hemiplegia has a tendency to become permanent, it establishes itself by gradation: first the lower limb, a few days later the upper limb and then the face. In another form syphilitic hemiplegia is associated with a palsy of cranial nerves, especially the third nerve. Finally syphilitic hemiplegia may develop suddenly without premoni- tory symptoms similar to ordinary hemiplegias. Syphilitic hemiplegia, when definitely established, may present several clinical varieties according to the intensity of the motor disturbances and to the localization of the lesion. They do not differ from hemiplegias with other etiological factors (see chapter on Hemiplegia). Course, Termination, Prognosis.— In cerebral syphilis with a general- ized specific arteritis the attacks of hemiplegia or of cranial nerve palsies may be fugacious and brief; they may disappear and reappear or else dis- appear completely when under treatment. On the other hand because of repetition of attacks the damage done to the cerebral tissue or to the cra- nial nerves, especially in the meningitic forms of cerebral syphilis, may be so intense that the lesion remains permanent. Changes in the cranial nerves may become so profound (atrophy) that recovery is impossible. The same is observed in circumscribed gummata on the surface of the brain. Therapeutic intervention (salvarsan, mercury, iodides) undoubt- edly modifies considerably, and sometimes favorably, the course of the disease, but it is powerless in cases of hemorrhages and softening in the cerebral tissue. The prognosis, generally speaking, is good when the disease is treated early, but it is grave in advanced cases. It is good also when the Wasserman reaction becomes negative. It is grave especially in cases with epilepsy, in thrombosis of the medulla and in cases with pro- nounced mental symptoms. Cerebral syphilis, as a rule, is a serious disease. Diagnosis. — The chief characteristics upon which a diagnosis of cere- bral syphilis can be based are as follows: 1. Sudden onset of cerebral symptoms in an individual in the midst of apparently good health. 2. Headache of a special form (nocturnal exacerbation). 3. Palsies of cranial nerves. 344 SYPHILIS OF THE NERVOUS SYSTEM 4. Hemiplegia, monoplegia, focal or generalized epilepsy. 5. The course of the disease: disappearance and reappearance of symptoms, their brief duration; multiplicity of symptoms. 6. Disappearance or prompt amelioration of symptoms under the influence of salvarsan, mercury and iodides. 7. The exclusive presence of lymphocytes and increase of albumen in the cerebro-spinal fluid, positive Wasserman reaction in the cerebro- spinal fluid and in the blood (see chapter on Lumbar Puncture). 8. History of syphilitic infection. As to the differential diagnosis with paresis and tabes see the respective chapters. SYPHILIS OF THE SPINAL CORD Spinal syphilis may present itself in the following forms: (1) chronic syphilitic meningitis; (2) meningo-myelitis; (3) acute syphilitic myelitis; (4) Erb's spinal paralysis; (5) gummatous tumor; (6) syphilitic disease of the vertebras, which is very rare. Pathology . — In the majority of cases of spinal syphilis the lesion is a meningo-myelitis. As the point of departure in spinal syphilis is usually in the meninges, it is important to consider first the changes of the latter. The most frequent lesion of the membranes is pachymeningitis. The thickened dura adheres to the pia-arachnoid and through the latter to the cord. It may be diffuse or circumscribed. The cervical region is most frequently affected. Histologically, in early stages the typical specific infiltration will be found (see Cerebral Syphilis), but in an ad- vanced stage of the disease there is a fibrous meningitis consisting of a uniform sclerotic (connective) tissue. Solitary gummata are rare. In meningo-myelitis (the most common occurrence) the following lesions are usually found. Acute. — The pia-mater is infiltrated with round cells and is therefore thickened; its small blood vessels are the point of orgin for this infiltration. This can be traced in all the prolongations of the pia. In the gray matter of the cord the cell infiltration of the blood vessels is at its maxi- mum. All the vessels, arteries and veins in the cord are similarly affected. Syphilis has a special predilection for blood vessels. Thickening of the walls of the latter causes a narrowness of their lumen, hence poverty of blood supply and softening of nervous tissue. Degeneration and atrophy of cells (especially in the anterior cornua) follow. In the white matter the nerve fibers at first lose their myelin and later disappear; they soon become substituted by proliferated neuroglia. SYPHILIS OF THE NERVOUS SYSTEM 345 Chronic. — Cases of long standing are characterized by sclerosis, which affects the meninges and the cord. The entire section of the cord gives the impression of a fibrous tissue. Ascending and descending degenera- tions are seen parting from the original focus. Symptoms. — In the majority of cases the meninges are first affected and the cord follows (meningo -myelitis). The meningeal symptoms precede often the symptoms of myelitis by days and weeks. ■. ■ - vOS: — *&-■ V- y : s Fig. 113. — Syphilitic Meningo-myelitts. (Flatau, Jacobson, Minor.) Pain along the spine radiating toward the limbs is the first symptom. It is usually aggravated at night. It is due to involvement of the meninges and of the roots. Numbness and tingling (paraesthesiae) in the limbs usually accompany the pain. Soon the cord becomes involved. Then develops paralysis in both or in one of the limbs, particularly in the lower, and usuaUy in one more than in the other. It is flaccid at the beginning, but later spasticity develops. The sensations are usually disturbed. Anaesthesia or hypassthesia may occur. Sometimes there is a sensory dis- sociation like in syringomyelia. The sphincters are always and very early affected : incontinence is rare. The sexual power is impaired. Muscular atrophy may occur, but this is exceptional. The characteristic feature of syphilitic meningo-myelitis lies in the multiplicity of symptoms, in their unequal distribution on both sides of 346 SYPHILIS OF THE NERVOUS SYSTEM the body, in their variability and instability, in their disappearance and reappearance, finally in their modification when the patient is under treatment. A. Thomas considers as one of characteristic signs of spinal syphilis what he calls "intermittent spasm of the blood vessels of the spinal cord." The latter is manifested in transitory, mobile disturbances in sensations, motion and function of the bladder. He considers these symptoms as the premonitory period of syphilitic paraplegia. (See also spinal intermittent claudication of Dejerine on page 334. According to the predominant localization of the lesion spinal syphilis may assume the form of almost any of the cord diseases: transverse myelitis, tabes, ataxic paraplegia, etc. When the disease advances in spite of the treatment (sign of well- established and irreparable degenerative lesions), the patient is in the same condition as in chronic myelitis with an array of serious symptoms, such as bed-sores, incontinence, contractures, etc. Meningo-myelitis of the thoracic region is the most frequent occur- rence. Cervical meningo-myelitis is frequently associated with a basal meningitis. Here all four extremities are involved, and if the eighth cervical and first dorsal segments are affected, symptoms of the sympathetic will be present, viz. contraction of the pupil, enophthalmos, disturbance of secretion of the sweat glands. Meningo-myelitis of the lurribo -sacral region will present a flaccid paralysis with loss of all reflexes, severe sensory disturbances in the lower extremities, disturb- ances in the function of the bladder, rectum and genital organs. Meningo- myelitis may also present the Brown-Sequard's type of paralysis (see this chapter on page 283). Finally the roots of cauda equina may be affected with gummatous meningitis, producing a complex symptom- group which is most frequently unilateral (see page 291). Erb's Spastic Spinal Paralysis. — This form is quite common. It is characterized pathologically by a primary degeneration of the postero- lateral tract, also ascending cerebellar tract. Shortly after the initial chancre (in one of my cases six months) gradually, but progressively, paralysis develops in the lower extremities. There are no marked men- ingeal or root symptoms observed in the preceding form. Pain, if present, is very slight. Girdle-sensation, numbness and a diminution of the general sensibility are usually present. The gait appears to be spastic, but it contrasts strikingly with the real flaccidity of the muscles (hypo- tonia). There is no muscular atrophy. The knee-jerks are exaggerated, ankle-clonus, Babinski's sign, Oppenheim's and paradoxical reflexes are elicited. The sphincters are early and constantly involved. The upper extremities are usually intact. There are no pupillary changes. SYPHILIS OF THE NERVOUS SYSTEM 347 Course, Termination, Prognosis. — Spinal syphilis is rarely acute. The majority of cases are chronic. They may last years. Complete recovery is not frequent, although it may occur. Those cases in which evidences of well-established tract lesions are present bear a bad prognosis. It is also unfavorable in cases which show only slight improvement in spite of a prolonged energetic treatment. Under treatment acute symp- toms may disappear, such as pain, bladder disturbance, but disturbances of reflexes, of gait, may persist. Death occurs from some intercurrent disease or from septic infection following cystitis or bed-sores. Diagnosis. — The unequal distribution, the instability, the disap- pearance and reappearance of the symptoms, their prompt amelioration when under treatment, finally their disseminated character, are sufficiently typical in the majority of cases to make a diagnosis. Acute Syphilitic Myelitis. — Acute myelitis may develop as a direct consequence of syphilitic infection. Pathology. — Swelling of lymph sheaths in the vessels of the meninges and cord, rupture of small vessels and extravasation of blood corpuscles; degeneration of white and gray matter. Symptoms. — Sudden or rapid onset with the following premonitory signs: paresthesia and sharp pains in the lower limbs; stiffness of the back; cramps in the legs; retention of urine and feces; the reflexes are variable. Soon appear the following manifestations. Complete para- plegia. Profound sensory disturbances. In myelitis of the lumbar seg- ment the tendon reflexes are lost, in myelitis of the dorsal region the reflexes may be increased. The function of the sphincters is very early involved. Wassermann test is strongly positive. Course. — Improvement may occur rapidly after the treatment is instituted, but recurrences are frequent. In some cases it may terminate fatally in a short time. CEREBROSPINAL SYPHILIS A simultaneous involvement of the brain and the spinal cord is by far more frequent in syphilis of the nervous system than an isolated affec- tion of each of the two portions of the central nervous system. In the majority of cases the cerebral disturbances are more marked than the spinal; the reversed condition may occasionally occur. Not infrequently one series of symptoms disappears promptly when the treatment is energetic, or else some symptoms are so slight that they are overlooked. When the patient comes under observation, only the spinal or only the cerebral type may be present. A close investiga- 348 SYPHILIS OF THE NERVOUS SYSTEM tion will reveal in a large number of cases the syphilitic invasion of the entire cerebro-spinal axis with predominance of either cerebral (more frequent) or spinal symptoms. Syphilis of the Peripheral Nerves. — In spinal syphilis the roots are compressed by the thickened meninges through which they pass. The nerve-trunks in their course between the roots and their terminations may encounter syphilitic gummata in the tissues and undergo compres- sion. But the peripheral nerves may be primarily affected by the specific poison, resulting in s\p>hilitic endo- and perineuritis with obliteration of the blood vessels and subsequent degeneration of the nerve-fibers. Symptoms. — They are: neuralgia, neuritis, multiple neuritis and root neuritis. Syphilitic neuralgia occurs in the early stages of the disease. Among all the cranial nerves the fifth is particularly a frequent localization. Of the cervical plexus the occipitales are often affected. The intercostal nerves are not rarely involved. Among the nerves of the lumbo-sacral plexus the sciatic suffers most frequently. We find here the same clinical manifestations as in neuralgia and neuritis of any origin; the same motor and sensory symptoms and changes of reflexes. Syphilis rarely affects all nerves with equal frequency. Some nerves are rarely involved. Thus, for example, the seventh nerve palsy is not a frequent occurrence (see my contribution in Arch, of Diagnosis, 1908). For practical purposes it is important to bear in mind that if in any given case of peripheral nerve involvement of long standing, after having excluded all toxic or infectious causes, the condition persists, syphilis should be thought of and an appropriate treatment instituted. Syphilis and Mental Diseases. — Mental disturbances may be en- countered in the secondary or tertiary periods. Those of the secondary period are genuine psychoses of toxi-infectious nature in this sense, that they are due directly to the action of the specific poison and not to cere- bral lesions caused by syphilis. They usually appear at a time when the eruption or any other acute symptoms such as mucous patches, adenop- athies, etc., develop. The onset is usually sudden. Headache and in- somnia appear first. Hebetude, stupor, somnolence, mental obtusion, lack of orientation, sometimes delirium and hallucination with delusions of persecution appear next. These are the phenomena of confusional insanity. They present no special features distinguishable from those en- countered in similar states caused by intoxications or infections of any other origin, except with regard to the curative effect of the specific treatment. The psychoses of the tertiary period differ from the preceding ones in this respect, that they occur long after the initial infection and do not coincide with eruptions or other secondary manifestations. They are due SYPHILIS OF THE NERVOUS SYSTEM 349 to meningoencephalitis, obliterative endarteritis and accompany usually motor symptoms, such as epileptic or apopletic attacks, palsies of cranial nerves, etc. They are the manifestations of cerebral syphilis. The mental phenomena of the latter with the gradually oncoming dementia as well as their exacerbations and amelioration when under treatment have been already considered. As to their relation to Paresis see this chapter. The relation of hereditary syphilis to mentality is of great impor- tance. With the advent of Wassermann reaction it was made evident that certain organic nervous diseases, especially in childhood, are the result of hereditary syphilis. It is admitted, for example, that juvenile paresis is due to hereditary syphilis. But apart from organic nervous diseases, parental syphilis can be considered also as the cause of various mental abnormalities, such as imbecility, idiocy, of various psychoses of the young. Such individuals not infrequently present in childhood or at puberty some external manifestations of syphilis, such as pupillary disorders, iritis, etc. Treatment.— Salvarsan, mercury and iodides are the chief medications. Mercury, — There are three methods by which mercury may be adminis- tered: (1) inunction; (2) intra-muscular injection; (3) ingestion. Inunction is a very good method. In an adult 5 j of unguentum hy- drargyri is rubbed in twice daily. The regions of the body having most cellular tissue are to be selected for the inunctions. The upper and inner surface of the arm and the upper and inner surface of the thigh are pref- erable. Each time a fresh area is selected. It is advisable to wash off with hot water and soap the surface to be rubbed into. Sulphur baths are advisable during the mercurial treatment, as thus the mercury is con- verted into sulphate of mercury which is easily absorbed. The number of rubbings depends on the intensity of the symptoms and upon the tolerance of the individual. I am in a habit to continue the inunctions for two weeks in succession, if there are no symptoms of intolerance. The latter consist of salivation, gastro-intestinal disorders, swelling of the gums, febrile state. Each inunction should last twenty or thirty minutes. Intra-muscular injections are administered in soluble or insoluble forms of mercury. The latter are sufficient once a week, the former more frequently. By this method mercury is rapidly absorbed. It is indicated in urgent or very grave cases. The gray oil is the most appropriate preparation among the insoluble ones; biniodide, benzoate and lactate of mercury, among the soluble ones. The dorsal muscles or the gluteal region are the places of choice. The injections must be made deeply into the muscular tissue and all pos- sible antiseptic precautions must be taken during the manipulation. 350 SYPHILIS OF THE NERVOUS SYSTEM Finally mercury may be administered by the mouth when inunc- tion or intra-muscular injections cannot be used. Among all prepara- tions I prefer bichloride of mercury in doses of gr. 1/32 t. i. d. for an adult. Mercurial treatment should precede the iodides, particularly in cases with acute symptoms. The mixed treatment is appropriate when the dis- ease becomes fully established and after the acute symptoms (headache, etc.) have been partly subdued by mercury. For the dosage of iodides see Treatment of Tabes. The action of the drugs is more prompt in cerebal than in spinal symptoms. In cases of circumscribed gummata, when the medical treatment has failed, operative procedures if accessible should be resorted to, and the removal of the tumor be followed by an- tisyphilitic treatment. As an adjuvant to the above treatment may be mentioned: hydro- therapy, dietetic and hygienic measures. Recently Ehrlich and Hatta have discovered a new arsenical prepara- tion to which they gave the name of 606 and later of salvarsan. This has proven to be a powerful remedy which is capable to destroy the spir- ochaetae with great rapidity. Its usefulness in initial chancre and secondary manifestations is beyond question. In cerebro-spinal syphilis salvarsan has also been of considerable value but not to the same extent as in the preceding conditions. While in many cases, especially of cerebral syphi- lis, it succeeded in relieving the patient's condition with respect to the frequency and intensity of the short transient attacks, nevertheless in a great many cases it did not prevent recurrences. The drug may be introduced into the body by deep injections in the muscles or directly into the circulation by intravenous method. The intravenous method presents these advantages, that there is no pain and the elimination is rapid, although it may be followed sometimes by a chill, rise of temperature, nausea and vomiting. Dose and Method of Preparation. — Adult average dose is 0.4 grm. for women and 0.6 grm. for men. Dissolve the drug in 100 c.c. of hot sterile water, add drop by drop a 15 per cent, solution of sodium hydroxide (sterile and filtered) and agitate. A yellow precipitate will form. Con- tinue adding the solution and agitate after each drop until solution be- comes clear again. Then add sterile hot filtered water and bring total up to 300 c.c. Each 50 c.c. corresponds to 1 decigram of salvarsan. The injection is to be given at ioo° F. Very recently Ehrlich has recommended a modification of his first discovery. He calls the new preparation neosalvarsan or 914. It is given in doses of 0.9 which corresponds to 0.6 of salvarsan. The SYPHILIS OP THE NERVOUS SYSTEM 351 solution is made by simple addition of the substance to 300 c.c. of filtered, distilled water at room temperature and injected at this temperature. In treating with salvarsan it must be borne in mind that the dose may be repeated several times. This should be done when the symptoms are only slightly influenced or especially when Wassermann reaction re- mains or becomes again positive. It is always advisable to wait after each injection several weeks. In two of my recent cases I had admin- istered three injections at intervals of four, six and eight weeks with pro- gressive benefit. Contraindications. — Diseases of the heart, kidneys, affections of the optic nerves, cachexia, alcoholism, advanced degenerative changes, are the main contraindications. Unfavorable By-effects. — The following complications have been ob- served from the treatment with salvarsan. Cranial nerve involvement. It seems that there is a special predi- lection of arsenic for the eighth nerve. Not only salvarsan but also other arsenical preparations show the same characteristic feature. The toxic effect particularly occurs after repeated injections. In another series of cases there may be a simultaneous paralysis of several cranial nerves, such as seventh, eighth and ocular nerves. In one of my cases a facial palsy of peripheral type occurred six weeks following a second injection of salvarsan. In another case I observed two days after the first injec- tion a very marked myosis of the left pupil which remained unaltered two years later. Heuser {Mediz. Klinik, 191 1) reports a case of iritis in a few days after intra-muscular injection of 0.2 grm. followed by an intra- venous in- jection of 0.4 grm. of salvarsan, also a case of paralysis of vocal cords and soft palate after an injection of 0.5 grm., finally a case of choked disc with facial paralysis. In the first two cases there were also epileptiform con- vulsions. Heuser bases his belief in the neurotropic action of salvarsan also on the fact that arsenic could be detected in the urine (twenty-three cases) nine months and in the blood two months after the injection. Finger {fieri, klin. Wchn., 191 1) reports three cases of optic neuritis after salvarsan. In a third group of cases recurrences of nerve disturbances have been observed after salvarsan injections. It is difficult to say whether the drug is directly or indirectly the cause of the recurrences. These special neuro-reactions are still debatable. They usually occur some time after the injections. It is possible that arsenic has a special irritative local effect on the spirochaetas. 352 SYPHILIS OF THE NERVOUS SYSTEM Among other by-effects may be mentioned: cardiac arrhythmia; dis- turbed function of the bladder, especially retention; constipation; diarrhoea; jaundice (in one of my cases it appeared on the day following the first in- jection); disturbances of tendon reflexes; peroneus paralysis; epileptifor seizures. Fatalities have also been recorded. They may occur a f - days or severa 1 • JQ ks after the administration of the drug. CHAPTER XXII PARESIS GENERAL PARALYSIS OF THE INSANE (DEMENTIA PARALYTICA) It is a parasyphilitic disease characterized pathologically by a diffuse meningo-encephalitis and clinically by symptoms of progressive dementia. 1 ^ &f.- '*}■ Fig. 115. — Paresis. Transverse Section of a Cortical Blood Vessel, Showing Leu- cocytic Infiltration. (Bouchard and Brissaud.) Histologically the cells, fibers, neuroglia and blood vessels are found altered. The cortical cells are in a state of degeneration. In acute stage they are swollen, the chromatophilic substance disappears, the axis- cylinders and protoplasmic processes are enlarged. In chronic stage the cells are sclerosed, deformed and disintegrated. The neuro-fibrils, according to some, are pigmented and diminished in number. The nerve-fibers undergo a gradual destruction. At first they become varicose and then lose their myelin, also the axis-cylinder. The cortical and sub-cortical fibers are particularly affected. The neuro-fibrils of the cortical cells are generally affected. The intra-cellular fibrils are more involved than the extra-cellular, also those of the small pyramidal cells more than those of the large pyramidal ones. The neuroglia is in a state of hyperplasia. PASESIS 355 The changes of the blood vessels consist of leukocytic infiltration of their adventitia and thickening of the other coats, so that eventually they become stenosed and completely obliterated. Similar alterations are found in the lymphatic perivascular spaces. The above histological alterations, viz. atrophy of neurons and hyperplasia of vessels and connective tissue, are found not only in the cortex, but also in basal ganglia, pons, medulla and cerebellum, although to a lesser degree. Some of the cranial nerves show atrophy and Fig. 116. — Paresis. Longitudinal Section of a Cortical Blood Vessel, Showing Leucocytic Infiltration. {Bouchard and Brissaud.) degeneration. Optic neuritis and atrophy are quite frequent. The third and sixth are not rarely involved. Among other constant patholog- ical lesions should be mentioned a sclerosis of the posterior and lateral columns of the spinal cord, the first more than the second (Anglade, Wyruboff, Klippel). Less Constant Lesions. — Pachymeningitis (hemorrhagica) ; thickening of the skull with obliteration of the diploe and formation of exostoses; hyperemia and thickening of spinal meninges with adhesions to the verte- bras; proliferation of the neuroglia in the medulla and cord; atrophy of the cells of the anterior cornua in the cord and of the nuclei of the cranial 356 PARESIS nerves (third, sixth, fifth, seventh, also olfactory nerve) ; inflammation of spinal nerves. The sympathetic nervous system has also been found involved in paresis. The peripheral nerves show parenchymatous degeneration, atrophy and proliferation of connective tissue. These changes are more frequently seen in the lower than in the upper limbs. The cerebro-spinal fluid presents the following characteristics: increase in the amount, lymphocytosis, increase of albumen (Nonne's Phase I), also positive Wassermann reaction (see also page 374). Symptoms. — Three periods can be considered, viz. (1) initial period, (2) period of full development, (3) terminal period. 1. In the majority of cases the onset is very slow and imperceptible. Gradual changes take place in the physical and intellectual spheres before the typical symptoms begin to appear. The general appearance of the patient is altered: he is pale, the features are drawn and without expression. Epileptic seizures, generalized or unilateral, may occur long before the initial period; they may be motor or sensory; they may be of petit mal or grand mal character. Transient attacks of aphasia are not infrequent. Palsies of ocular muscles (strabismus, ptosis, inequality and irregularity of the pupils, diplopia) are common. Sudden loss of power in one limb, increased reflexes, various neuralgias, gastric and vesical crises, insomnia are also not infrequent. Various trophic or vaso -motor disturbances, as, for example, spontaneous fractures, falling out of the hair and nails, various visceral disturbances (vomiting, diarrhoea, palpita- tion, etc.) are observed. Mentally the patient is changed. He is irritable, depressed, indif- ferent. His memory is weakened. His aptitude for work is diminished and when he makes an attempt, an unusual effort is required. It is difficult to hold his attention in a conversation or transaction. Briefly speaking, he presents symptoms usually found in neurasthenia. Gradually these symptoms increase in intensity and others are added. Instead of being morose and apathetic the patient may be restless and excitable. The least contradiction angers him. He becomes egotistical and his moral sense is blunted: he has no obligations to his family, lies, deceives. Conventional laws are beginning to be ignored by him. Crimes against morality are of common occurrence. Sometimes the patient shows in the initial stage an exalted intel- lectual activity. Instead of depression he presents a sense of well-being. There is a hyperamnesia, a remarkable power of forming ideas, of creat- ing images, which is noticeable in acts and speech. He plans extrava- gantly, speaks extravagantly of his fortunes, of his properties. The same paresis 357 phenomenon is observed in various vegetative functions; exaggerated appetite, sexual and alcoholic excesses are frequent. These changes in the intellectual and moral spheres are forerunners of oncoming dementia. Physical signs begin to become conspicuous at this stage of the disease. An awkwardness in gait, in station, in doing fine work, in handling objects, a slight tremor of the hands, hesitation of speech, are beginning to be noticeable. 2. In the period of full development of the disease the physical and psychic symptoms are very marked. Physical. — i. Tremor is constant. It is generalized, but more marked in the hands. It . is fine, rapid and intentional. Instead of tremor, there may be only jerky movements of the fingers, which are noticeable upon a voluntary act. Tremor and instability are also present in the tongue, lips and muscles of the face. They are particularly noticeable in attempts to speak. The face when at rest gives the impression of immobile mask, but on the least emotion the facial muscles commence to contract excessively as if they awaken from a latent spasm. This mixture of paresis and spasm is quite characteristic of general paralysis of the insane. It demonstrates a disturbance of mimicry. 2. The speech is characteristic. It may be tremulous (ataxic) or spasmodic. In the first case the words are precipitated, then interrupted and then again continued, at the same time hesitated and repeated. In the second case the words and syllables are slowly pronounced and resemble those in multiple sclerosis. There is a dysarthria: the labials and gutturals are particularly affected. 3. The disturbance in writing is analogous to that of the speech: omission of letters, syllables, words, mistakes of grammer, repetition, poor spelling of letters, etc., are all typical. In an advanced period the writing is not distinguishable. 4. Visual disorders most frequently consist of myosis or mydriasis, irregularity and inequality of the pupils and reflex disturbance. Argyll- Robertson sign is frequent. A paradoxical pupil (response to light and not to accommodation), Piltz's sign (contraction of the pupil at the attempt to close eyelids) may occur. Ocular palsies, diplopia, nystagmus and changes in the eye grounds (optic atrophy) are not rare. According to Uhtoff optic atrophy is observed in 50 per cent, of paretics, while Galezowski's statistics show only 18 per cent. 5. Muscular weakness is contant. The face is mask-like, without expression. Sometimes there is loss of control over the facial muscles and the masticated food or liquids run out of the mouth. Paralysis, incomplete or transient, after an apoplectic seizure is frequent. Attacks 358 PARESIS of monoplegia or hemiplegia, of aphasia, of blindness or deafness, may occur. In the majority of cases they are temporary. An attack of this nature may be accompanied by loss of consciousness. Occasionally and in severe cases an attack may leave the patient permanently hemi- plegic. Spasmodic contractions are met with. Epileptiform seizures, while more frequent in the first period, may also occur at this stage. They may be generalized or focal. They may affect the sensory motor and vasomotor centers equally. Instead of convulsive attacks there may be fits of petit mal. Each attack of epileptiform convulsions leaves the paretic more exhausted than in ordinary cases of epilepsy. The gait is usually ataxic ; it may be also spastic. Romberg's sign may also be present. 6. The tendon reflexes are altered in 90 per cent, of cases. In the majority they are exaggerated, in a small percentage of cases lost. Bab- inski sign is rare, while the paradoxical sign is frequent. This peculiarity lies in the fact that the pyramidal tract is only slightly involved. Bab- inski sign is usually the expression of a well-defined degenerative lesion, while the reflex described by me is present in the earliest changes of the motor pathway (/. of Nervous and Ment. Dis., 1907). Ankle-clonus and Oppenheim's reflex are rare. 7. Cutaneous sensibility is usually diminished. Neuralgia, migraine, paresthesias may be met with. The special senses may be affected. Anosmia, changes of taste, of hearing, are not rare. 8. Trophic disturbances, such as falling out of the nails, of the teeth, herpes zoster, bed-sores, arthropathies, spontaneous fractures, are some- times observed. Vasomotor symptoms, such as tinnitus aurium, pallor, coldness, gangrene of the extremities, are common. There is a tendency to for- mation of hematomata. Hematoma of the ear is not rare. 9. Visceral Disturbances. — They are: increased digestive function in the exalted state, diminished in the depressive state, gastro-enteritis ; pulmonary involvement; circulatory disorders (aortitis); genito-urinary disturbances (impotence, sterility, suppression of menses); involvement of the sphincters of the bladder and rectum (retention or incontinence, frequent or imperative micturition). 10. The secretions may undergo changes. Excessive salivation and sweating have been observed. 1 1 . The toxicity of blood and it bactericidal power are increased. The cerebro-spinal fluid contains quite a perceptible amount of fluid and abundant lymphocytes. Wasserman reaction is positive and there is increase of albumen (Nonne's Phase I). paresis 359 12. The general nutrition as a rule is lowered at first, then gradually improves and the patient becomes stout. Later on there is again a loss which finally ends in cachexia. Psychic Symptoms. — Progressive enfeeblement of the mental faculties, viz. progressive dementia, which is accompanied by symptoms of other psychoses, is characteristic of paresis. Amnesia predominates. In the initial stage the loss of memory af- fects recent events. In the second period it concerns also old events. The patients forget even their age, birthplace, etc. Judgment, power of attention, will power are all markedly impaired. The patient is easily influenced, because of defect in the will power. As mental self-control is defective, the patient succumbs to the influence of impulse. Outbreaks of emotional nature are then seen. Theft, assaults, homicide, etc., are not rare. Performance of delicate acts is deficient. A very important symptom is the inability to recognize one's own infirmity in spite of the exuberant spirit. Such a patient does not appre- ciate the fact that he is ill, that he fails in performing his work. The want of knowledge of this infirmity leads to formation of ideas of self- importance, of expansive ideas. The latter are very common in paresis. The expansion or exaltation is noticeable in psychic, motor, sensory and vegetative spheres. The patient believes himself as possessing millions of dollars, of properties, of friends, of enemies. He is famous, his name is mentioned everywhere; he is a prophet, a god, etc. He is restless, does not sleep, eats abundantly, shows a tendency to excesses of all kinds. Gradually the moral sense becomes obtunded. The patient is unable to form new connected ideas, unable to orient himself; he is indifferent and becomes automatic in his acts. The latter are foolish, childish. The further the dementia advances, the less he is capable to take care of him- self; he is unclean about his person, indecent, uses profane language irrespective of the surroundings. During the progressive development of the dementia other symp- toms are observed, viz. delirium, confusion, delusions with or without hallucinations. The delusions in paresis are usually vague, unsystematized, unstable. Sometimes they are systematized and fixed, similar to those of paranoia. They may be depressive, persecutory or else expansive. They may be also hypochondriacal. Hallucinations not infrequently accompany the delusions. Hallucinations of various senses are not rare in paresis,espe- cially of sight and hearing. One of my patients had an olfactory halluci- nation: he smelled feces at the approach of food. A delirious state with great excitement and restlessness occurs episodic- 360 PARESIS ally during the entire course of paresis. A confusional state is present, par- ticularly in the second stage of the disease. The various delusions just mentioned may sometimes simulate other psychoses, but they never present the characteristic and well-defined features of the latter. 3. Terminal Period. — The physical and mental symptoms just de- scribed progress gradually toward a terminal stage, which is character- ized by complete deterioration and relaxation of sphincters. The hebetude, dementia, difficulty of speech, pupillary symptoms, ataxia of locomotion, tremors, have all reached the climax. New phe- nomena develop. Paralysis of the vesical and rectal sphincters, par- alysis of the pharynx, trophic disturbances, viz. bed-sores, render the paretic completely helpless and hasten his death. Apoplectiform and epileptiform attacks are fre- quent at this stage of the malady and may be the immediate cause of death. Not infrequently death occurs from an intercurrent disease (pneumonia or others). Forms. — (a) Simple De- mented Form. — It is very fre- quent. It is characterized besides the typical physical symptoms by a gradually developing dementia from the beginning to the end and not accompanied by delusions. The dementia is usually not marked, so that the patient continues for a certain period his usual occupation. Epileptic seizures are very frequent in this form of paresis. (b) Depressive Form./ — Marked mental depression is characteristic. Delusions of persecutory or hypochondriacal nature are present. In some cases the latter are so pronounced that melancholia may be thought of, especially when the ideas of unpardonable sin or impending evil and tendency to suicide are predominating. However these delusive ideas are unstable and do not present on a whole the picture of genuine melan- cholia. In this form there is a marked tendency to obesity. Fig. 117. — Depressive Form of Paresis. Expression Sad, Preoccupied and Demented (Bouchard and Brissaud.) PARESIS 361 Remissions are not so frequent as in the following form. (c) Expansive Form. — It is characterized by an exalted general atti- tude. Even in the absence of distinct delusions the patient's manner and expression betray a self-satisfaction. The paretic is happy, contented with everybody and everything. Sometimes the expansion may increase to such an extent as to cause marked restlessness and excitement, so as to simulate mania. However, taken as a whole, it is not a genuine mania. As to the delusions, they are of the exalted type. The pa- tient is a multimillionaire, prophet, god, great actor, writer, etc. (see above). In spite of his great self- importance, he is very easily in- fluenced and is submissive. (d) Circular Form. — It is char- acterized by alternation of depres- sive and expansive states. It "may be confounded with the manic-depressive psychoses. An examination for physical signs and repeated observation will decide the diagnosis. (e) Galloping Form. — In rare cases rapid evolution of symptoms is observed. The mental mani- festations are characterized by 'rapid deterioration of mind, loss of control of the sphincters, bed- sores, exhaustion and death. It is possible that the symptoms of paresis in such cases had been long at work and because of mildness of the manifestations they were overlooked. Under the influence of some exciting cause, the symptoms suddenly assumed a rapid course and promptly reached a fatal termination. (/) juvenile Paresis. — It is occasionally observed. In one of my cases, a young man of eighteen, the symptoms began to show themselves at the age of twelve. Signs of mental enfeeblement appeared first. Speech disturbances of the typical paretic variety developed gradually two years later. At the time I saw him, he presented the characteristic fine tremor, paretic speech, ataxia, exaggerated knee-jerks, irregular and Fig. 118. — Expansive Form of Paresis. Ex- pression Happy. (Bouchard and Brissaud.) 362 PARESIS unequal pupils and depression with hypochondriacal delusions without hallucination. He never had epileptic or apoplectic seizures. He is still living, but his dementia is getting progressively deeper. Wassermann reaction was strongly positive. Course, Duration, Prognosis. — Paresis is essentially a progressive disease. Its course may be slow or rapid. The course of the simple demented form is extremely slow. The expansive form runs a more rapid course than the depressive form. Complications, such as apoplec- tiform or epileptiform seizures, visceral complications, hasten the course of the disease. Paresis may present periods of arrest and improvement, so-called remissions. The latter consist of a partial or complete disappearance of the mental manifestations. These, so to speak, lucid intervals may oc- cur at any of the three periods of the disease. They may last from a few days to weeks, months and even years. They are usually the result of a proper and early instituted treatment. Remissions sometimes follow a traumatism or a protracted suppuration. In the majority of cases the physical symptoms persist. I have seen cases in which very great improvement of the physical signs also occurred during remissions. The duration of paresis is from a few months to several years (five and ten years). The second stage is the longest. The prognosis is unfavorable. Death may result from the invariably progressive course of the disease, ending either with a disease of the lungs (tuberculosis, pneumonia), diar- rhoea, bed-sores or extreme exhaustion. It may also end accidentally: suffocation from impaction of food in the larynx during an epileptic seizure; fractures of bones; finally suicide. Sudden death has also been observed. In such cases either there is an element of alcoholism, or a cardiac pathological condition, or a cerebral hemorrhage. In some rare cases no perceptible cause could be found. Sudden death occurs more frequently in middle aged individuals than in elderly or young persons. Diagnosis. — As the onset and various forms of paresis may simulate other diseases, the differential diagnostic points must be emphasized. The neurasthenoid state of the first period will be distinguished from genuine neurasthenia by the following considerations: A neurasthenic is capable of presenting a detailed account of his ills. He exaggerates his symptoms, it is true; nevertheless they are recited in the most connected manner. The patient repeats his recital often, but this repetition is not because of a genuine amnesia, but because he is anxious to analyze his troubles and to draw his physician's special attention to them. The neurasthenic appreciates his illness keenly. PARESIS 363 The paretic is incapable of giving a connected history of his trouble. He frequently interrupts his speech and forgets what he said previously. When reminded he does not attach any importance to it. Frequently the expression of his face does not correspond to the subject of his conver- sation: he may show a smile while speaking of death. A true neurasthenic appreciates keenly his trouble, while the paretic is incapable of recog- nizing properly the ills of which he speaks. In a true neurasthenic mental operations are readily exhausted but not disturbed or modified. The paretic presents a genuinely diminished intelligence. Want of power of criticism and of judgment, grave omissions, errors in daily acts, change of moral personality — are all met with in paresis but not in neurasthenia. Besides, the cerebro-spinal fluid presents certain charac- teristics in paresis (see page 375), which are not present in neurasthenia. Wassermann test on blood or on cerebro-spinal fluid is positive in paresis, but not in ordinary neurasthenia. The delirious state, the confusional state of the paretics may be confounded with similar states occurring in intoxications. These are the so-called cases of pseudo-paresis (alcoholic, saturnine and others). If the diagnosis is difficult at the beginninp, a subsequent study of the cases will clear it up. The delirious, confusional states are only transient and the obnubilation of intelligence is not as profound in intoxications as in paresis. The alcoholic subject usually suffers from hallucinations of a terrifying nature which become accentuated at night. He usually appears brutal and stupid, but not demented. The paretic presents a reverse condition. The alcoholic appears sad and preoccupied, the paretic indifferent and apathetic. If an alcoholic develops expansive delusions, they have to be brought out and can be ascertained only on a skillful questioning of the patient. The paretic does it unsolicited. The speech of the pa- retic is ataxic, full of repetition of syllables and words. The speech of the alcoholic is merely embarrassed, thick and tremulous. With- drawal of intoxicants is followed by a progressive amelioration; in paresis the symptoms are essentially progressive. Lymphocytosis is absent and Wassermann test is negative in alcoholism. Symptoms of lead intoxication (encephalopathy) may resemble paresis especially by the psychic manifestations. In both affections a neurasthenic state and mental attitude suggest an oncoming dementia, but in encephalopathy the dementia seems to dominate the entire situa- tion from the onset. The physical signs, such as cachectic appearance, difficulty of speech of a gross nature, motor disorder of paralytic nature affecting chiefly the extensor groups of muscles, abdominal colic, severe 364 PARESIS headache, the blue line on the gums, interstitial nephritis, arterio-sclerosis — are all found in lead intoxication. Finally, the regressive evolution of symptoms after an appropriate treatment and removal from the toxic influence is characteristic of lead intoxication. Lymphocytosis, increase of albumen in the cerebro-spinal fluid, positive Wassermann reaction, are characteristic of paresis, but absent in lead encephalopathy. Diffuse cerebro-spinal syphilis cannot always be easily differentiated from paresis. In both affections a neurasthenic symptom-group exists. Cerebro-spinal syphilis produces a profound neurasthenic state which resembles appreciably paresis, especially in the initial stage of the latter. The diagnostic difficulty is especially great, when in both affections Wassermann and Noguchi reactions are positive and lymphocytosis is present. In cerebro-spinal syphilis the onset is usually acute, in paresis slow and insidious. In the first a marked diminution of intellectual functions is present at the onset, in the latter the intellectual defect is less marked at first. In the first there is a continuous severe headache which is worse at night, in the latter this is not the case. In the first there is intellectual inertia, profound apathy, somnolence and a special dulness in the facial expression. Such a picture is not seen in paresis. There is never in cerebro-spinal syphilis the optimistic carelessness and general exaltation in the motor and sensory spheres which are observed in the expansive form of paresis. Alongside of the apathy and loss of memory the reasoning power may be totally preserved in the first, but this is not observed in paresis. Cerebro-spinal syphilis is usually accompanied by local symptoms. Very early are observed ocular changes, such as optic atrophy, con- traction of visual fields, palsy of other cranial nerves; symptoms of spinal cord involvement, such as exaggerated knee-jerks, toe phenomenon, ankle-clonus, sensory changes, involvement of sphincters. The course of cerebro-spinal syphilis is characterized by great variability and mobility of the symptoms which become either exaggerated or ameliorated. In paresis the symptoms are invariably progressive. The antisyphilitic treatment has a remarkably rapid effect on the symptoms of cerebro- spinal syphilis. Such a course of events is not observed in paresis. The richness of symptoms in syphilis is such that it is possible in almost every case to reveal some particular feature indicative of the existence of a localized focus in the brain and spinal cord. The incomplete, unequal and regressive character of cerebro-spinal syphilis is different from the PARESIS 365 global and progressive character of paresis. Variability and capricious- ness of the symptoms are typical of syphilis. Paresis should also be differentiated from tabes, from multiple sclerosis, tumors of the brain. The special symptoms of these affections will aid in the diagnosis. Etiology. — The chief determining cause is syphilis. It is not in the acute stage of syphilis that paresis develops, but years after the initial infection (from five to fifteen years). Since the discovery of Wassermann's reaction and in view of the findings of Spirochsetae in paretic brains by Noguchi and Moore (page 353), paresis has proven to be without reserva- tion a syphilitic affection. Plaut (Allg. Zeist.f. Psych, u. Neur., 1909) , for ex- ample, obtained in every one of his cases of paresis without a single excep- tion a positive Wassermann reaction. In a number of cases in which a history of syphilitic infection cannot be obtained, the Wasserman test is of utmost value. While formerly such cases kept us in doubt as to syphilis being the only cause of paresis, now with Wasserman' s discovery all hesita- tion must be set aside. Juvenile paresis showing a positive Wassermann reaction proves that congenital syphilis plays also a powerful role. The following peculiarities concerning the relation of the reaction to Paresis deserves special mention. (1) If the blood serum does not give a positive reaction, the cerebro-spinal fluid will be negative. If the cerebro-spinal fluid gives a positive result, the serum does the same. (2) If the serum gives a positive result, but the cerebro-spinal fluid a negative, the disease is not paresis, but cerebro-spinal syphilis. (3) The reaction of the cerebro- spinal fluid is more important in paresis than that of blood serum. (4) Mercury, iodides, salvarsan may render Wassermann reaction negative for a certain time. The predisposing factors are: undue and prolonged mental efforts, worry and anxiety; venereal and alcoholic excesses; traumata of the head; sunstroke. Men are more frequently affected than women. The age between thirty and forty-five is the most favorite. Juvenile paresis may occur as early as twelve years of age. As to the countries, the higher the degree of civilization, the larger the number of cases. The question of neuropathic hereditary influences is not entirely settled. However, not infrequently a history of some neurosis or insanity is traced in the antecedents of paretics. Treatment. — As soon as paresis is recognized or even suspected in its initial period, an antisyphilitic treatment should be instituted (see Treat- ment of Syphilis of the Nervous System) . However, in spite of the fact that paresis is a syphilitic disease, not much can be expected from the specific medications. The value of Ehrlich's new remedy, salvarsan, 366 PARESIS has been sufficiently tested to warrant some definite conclusions. If it does any good at all, it improves sometimes the general health, but it has no effect whatever upon the course of paresis. It may be given in conjunc- tion with mercury and iodides. The treatment should be instituted as early as possible. However, I have seen favorable results in prolonged treatment with iodides given progressively in very large doses (300 or 500 grains daily). In view of the fact that some authors observed unfavor- able results from iodides, the increase of the dose should be made very cautiously and upon the least sign of intolerance the drug should be dis- continued (see also Treatment of Tabes). My favorable results are meant only from the standpoint of some acute symptoms, such as headache, in- crease of tremor, insomnia, disturbance of hearing or of sight, also ataxia. It is worth while mentioning the fact that administration of salvarsan renders the Wassermann reaction negative. In spite of this the course of paresis is not altered. The hygienic and dietetic measures are more important. As soon as possible a paretic should be removed from his usual occupations. A quiet life (better in a sanitarium or in the country), avoidance of excitement and worriment, total abstention from stimulants, including tea and coffee, regularity in meals and sleep, a diet free from articles which are likely to produce fermentation, finally hydrotherapy (brief douches, baths, etc.) are all extremely beneficial. Any disturbance of the gastro-intestinal tract, of renal function, of sleep, should be immediately combated. Sexual intercourse must be avoided. If this plan of treatment is strictly carried out, remissions will occur early in the course of the disease and if a remission is not considered a cure (which is frequently done otherwise) and the above general treat- ment kept up with the same regularity and energy, the remissions may be prolonged for months or years. EARLY PARESIS Early Paresis. — The recognition of paresis in its earliest stages is of utmost importance both from a practical and medico-legal standpoints. At this stage the symptoms may be so slight that they are easily over- looked, proper measures are neglected and deplorable consequences follow. The patient is permitted to be at large, to travel, to transact business, to govern. It is only when grave errors are committed that attention is drawn to the patient's condition. Psychic Status. — The most delicate and the highest mental functions are invaded the earliest. They are therefore particularly noticeable in persons of culture. It is in the fulfilment of fine acts necessitating the PARESIS 367 most delicate discrimination that lapses and omissions are observed. Memory is affected early in the disease. It is slight and observed only at intervals in words and acts. Soon the patient shows omissions in his daily work; his writing shows lapses and repetition in letters, syllables and then in words. The amnesia leads to neglect in his own appearance. Punctuality of habits is neglected and the patient becomes indifferent. Usual reservation in language is replaced by loquacity and unreasona- bleness. He is conscious of the defect of his memory but he is not dis- turbed by it in the least. He does not try to avoid errors. He even ridicules his own mistakes. This indifference toward the amnesia is characteristic of early paresis. At the same time changes appear in the character, disposition and moral personality. The previously cheerful person becomes depressed, irritable, apprehensive. He loses interest in everybody; is neglectful of his duties, of his home. He resembles then a neurasthenic, but the resemblance is only apparent (see differential diagnosis). In other cases a totally opposite condition is observed. The usually conservative man has become exuberant in actions and spirits; he makes ambitious schemes, spends money in an unusual way. This exaltation is manifested not only in the intellectual and motor spheres, but also in the affective sphere. The paretic is self-contented, optimistic and he shows a tendency to excesses. Immorality is the natural consequence; sexual perversion is not an infrequent phenomenon. The alterations of the moral personality occurs often long before the intelligence is seriously affected. The exuberance is only superficial, it lacks in depth. With all his agitation the early paretic is not capable to accomplish much. The contrast between what he appears to be able to do or between what he promises to do and what he actually does is striking. He is easily fatigued. He is always ready to undertake work, but is unable to persist in it. A characteristic feature is the inability of the patient to realize his infirmity in spite of his exuberant spirit. He does not appreciate the fact that he cannot continue his usual occupation. Another interesting phenomenon is the early defect of criticism. When the patient is con- fronted with the most evident manifestations of his affection, he does not pay the slightest attention and rather ridicules them. To sum up, defective memory, weakened faculty of attention, of judgment, of criticism, perversion of moral sense, of disposition, radical change in conduct, inversion of affective faculties — all constitute character- istic psychic manifestations of the early period of paresis. 368 PARESIS Somatic Symptoms. Apoplectic seizures, epileptic seizures, attacks of aphasia — are the three groups of somatic manifestations that not in- frequently announce the oncoming paresis. The apoplexy may consist only of sudden mental hebetude, or of a genuine loss of consciousness followed by monoplegia, hemiplegia with or without aphasia. These phenomena arc usually brief and temporary. Epileptic seizures are mostly of petit mal form. They may be also of grand mal form, generalized or local. In the latter case (local) they may be of the sensory variety: sudden paresthesias, such as tingling or burning on one hand, on one side of the face. Aphasia may occur without hemiplegia. The attack is sudden and transitory, but it has a tendency to recur. Pupils.— In fifty out of fifty-nine patients studied by me from the standpoint of early paresis the pupils were found irregular and especially unequaL Sluggishness of light reflex is another frequent symptom. Ocular palsies are not very infrequent, but they are usually temporary. Reflexes. — The knee-jerks are mostly exaggerated. Babinski's sign is rare. Paradoxical reflex is not infrequent. Speech and tremor are both rarely altered in early paresis. Early paresis presents a specially important chapter from the medico- legal standpoint. By reason of gradually developing moral and affective perversions which announce the onset of the disease, the initial period of paresis is the real medicolegal period. It is then that misdemeanors are frequently misinterpreted and that the individuals are sent to prison. Early paretics not infrequently marry. The new life the patient leads in association with sexual excesses aggravates the condition. Early paretics are able to carry on their former habits and other acts concerning their usual occupation, but they do it automatically. The slight lapses of memory, the digression in their conduct apart from their regular work, the changes of disposition and sentiments — are usually all attributed to fatigue or absent-mindedness. They are permitted to have liberty of action and to continue in responsible positions until misconduct of grave nature and consequences occurs. It is at this period that illegal acts and crimes are most frequently committed. Its recognition and proper interpretation is a matter of grave importance. Civil capacity and legal responsibility should be considered as having no existence as soon as the earliest signs of paresis begin to make their appearance. CHAPTER XXIII LUMBAR PUNCTURE AND CEREBRO -SPINAL FLUID Lumbar puncture is a necessary procedure at bedside. It gives an opportunity for study of the cerebro-spinal fluid besides the therapeutic effect procured by extraction of a certain amount of the latter and by injection of soluble drugs or specific sera into it. Anatomical Points. — The arachnoid sac which does not contain the spinal cord, but contains only the nerves of the cauda-equina, extends normally from the second lumbar vertebra to the second sacral .vertebra. It is between these two extreme points that a puncture of the sac can be made. As. in children especially, the cord reaches lower down, it is ad- visable in every case not to puncture between the second and third lumbar vertebrae. The most reliable place is the space between the fourth and fifth lumbar vertebrae. A transverse line drawn between the crests of iliac bones passes exactly at the level of the spinous process of fourth lumbar vertebra. Immediately beneath is the fourth lumbar space. It is there that the puncture is made. Technic. — The position to be given the patient is variable: some prefer a lateral position, others prefer a sitting position. The latter presents certain inconveniences, namely a too rapid issue of the fluid or fatigue of the patient. When the patient b'es on his side, he is placed as near as possible the edge of the bed; the head is slightly raised on a pillow, the thighs are flexed as strongly as possible over the pelvis so that a marked posterior convexity of the lumbar spine is obtained and thus a maximum separation of the two vertebrae is produced. The skin is then washed thoroughly and painted with tincture of iodin. Rigorous antiseptic precautions must be observed by the operator with regard to his hands and the instrument to be used. Local anesthesia is not necessary. The trocar or a needle of 9-10 cm. in length and 0.8 mm. in diameter must be used. It must be fine but at the same time solid. In muscular or insane individuals a very strong needle is necessary. The operator then places the index of the left hand on the space to be punctured. The needle is then seized firmly with the right hand and plunged at a half of a centimeter laterally to the place outlined with the finger of the left hand. Slowly but progressively the needle is advanced toward the median line and slightly upward. At a distance of about 4 to 6 cm. in an adult and 24 369 370 LUMBAR PUNCTURE of i to 3 cm. in a child the needle reaches the spinal canal and per- forates the arachnoid sac. The cerebro-spinal fluid appears imme- diately at the outer end of the needle. When the desired amount is collected in a sterile tube, the needle is withdrawn abruptly, the punc- ture of the skin is obliterated with collodion. It is not always easy to enter the spinal canal. Frequently the needle strikes against the vertebrae; the needle is then slightly withdrawn and with a slight devia- tion from the original direction will eventually penetrate the canal. Sometimes it is necessary to withdraw the needle entirely and renew the operation. It may happen that instead of cerebro-spinal fluid blood appears at the outer end of the needle. The blood frequently rapidly disappears and clear fluid makes its appearance. If the blood persists, the needle must be withdrawn and a new puncture made; the blood was probably due to a puncture of a small intradural vein. Sometimes toward the end of the operation the patient is seized with cramps in the thighs; they are due to compression of some nerve filaments of the cauda equina. They do not last long. Accidents. — Lumbar puncture is, generally speaking, an innocuous operation. However, untoward symptoms have been observed. Vertigo, headache, nausea, vomiting, pain in the back, convulsions may occur, but they do not last longer than a few hours. Occasional death has also been observed especially in cases of cerebral tumors and when a too large amount of cerebro-spinal fluid was withdrawn. Sicard has formulated the following precautions to be observed in lumbar punctures. (i) Avoid lumbar puncture in brain neoplasms in which headache, nausea, vertigo increase when the patient is in dorsal position. (2) Before the puncture keep the patient in bed for 24 hours. (3) Always use the lateral position for the operation. (4) After the puncture keep the patient in dorsal position during 48 hours with the head but slightly elevated. (5) Except in special cases, withdraw only 4 to 8 c.c. of the fluid without aspiration. (6) Use a very fine needle 0.8 or 0.9 mm. to reduce to a minimum the meningeal injury. In cases of brain tumor observe the following rules: (1) Before the puncture, observe horizontal position in bed, the head but slightly raised, for 48 hours. (2) Puncture in lateral position, the head slightly lowered (Trend- elenburg's position). (3) After the puncture keep the same position with the head still lower for 24 hours; then horizontal position for 48 hours. CEREBROSPINAL FLUID 37 1 CEREBRO-SPINAL FLUID The study of cerebro-spinal fluid offers a great deal of information from a diagnostic standpoint. Cerebro-spinal fluid closed up in the ventricles and in the subarach- noid spaces is secreted by the choroid plexuses. Physical Properties. — Normally the fluid is perfectly clear. In pathological conditions it is generally turbid, purulent or lacteous, or else in exceptional cases also clear. In tubercular conditions it is floccu- lent and coagulates in large lumps. The color may change. When it is red, intra-meningeal hemorrhages are to be suspected. Whether the hemorrhage is of cerebral, bulbar or meningeal origin, or else in cases of contusions or fractures of the skull, the cerebro-spinal fluid will present either a frankly hemorrhagic color or a yellowish or greenish discoloration. Sometimes however the bloody fluid may originate from the tissues through which the needle passes before it reaches the spinal canal. The following differential features have been emphasized by Tufher. (i) The blood from an accidental cause coagulates; when it is from the cerebro-spinal fluid it does not coagulate, but presents a sediment at the bottom of the tube. (2) If the bloody fluid is centrifugated and presents a clear aspect, the hemorrhage was accidental and due to the puncture of the external tissues. On the contrary, if the fluid remains colored, the blood existed in the subarachnoid cavity. In the course of acute cerebro-spinal meningitis the cerebro-spinal fluid may be of yellowish color. The latter is probably due to small meningeal hemorrhages. Tension. — In cerebral tumors, in meningitis, in syphilis (secondary period) , in epilepsy there is increased tension. Chemical Properties. Reaction. — Normally it is alkaline. Albumen. — In normal condition albumen is either absent or it is present in extremely small quantities. In pathological states there is increase of albumen. In Paresis, Tabes, in Syphilis of the Nervous system albumen content is increased. The majority of acute states present hyper-albuminosis, such as tubercular meningitis, cerebro-spinal menin- gitis, meningeal symptoms accompanying infectious diseases and intoxica- tions (lead). When the quantity of albumen in above 0.25 or 0.30 grm. it is an indication of meningeal reaction. 372 CEREBROSPINAL FLUID Glucose. — Normally there is 0.50 gm. of sugar. Diminished quantity is indicative of meningitis, especially cerebro-spinal and tubercular. Chlorids. — Normally there is 7 grm. of chloride of sodium. Dimin- ished quantity is found in meningeal conditions, especially in the tubercu- lar form. Cytological characteristics. — In normal condition the cerebro-spinal fluid contains very few cellular elements. There may be a few rare lymphocytes, but no polynuclear cells. As soon as the meninges are inflamed, rapidly a lecocytosis develops. Lymphocytes and poly- nuclear are the two forms that figure prominently in pathological condi- tions. Speaking generally, according to Sicard polynucleosis is an indica- tion of a grave pathological condition, lymphocytosis of a less serious state or of a chronic condition. But to this rule there are many exceptions. Polynucleosis is observed in the acute stage of epidemic cerebro- spinal meningitis, sometimes also in the beginning of tubercular meningitis. The number of cells is in relation with the intensity of the disease. When the latter improves, the polynuclears are gradually substituted by lympho- cytes. Polynucleosis accompanies meningeal infections of a strongly microbic nature (except tubercular), viz. epidemic cerebro-spinal, other forms of meningitis, cranial traumatism, otitic complications. Tubercular meningitis is essentially a lymphocytic condition. Syphilis at any period (except the chancre) presents essentially a lymphocytosis. In acquired senilis, specific meningitis acute or chronic, specific meningoencephalitis, specific neuritis, also in hereditary syphilis — lymphocytosis is characteristic. In Parasyphilitic diseases, tabes and Paresis, lymphocytosis is constant. In Paresis during the paroxysms a temporary polynucleosis is observed. The same lymphocytic formula is observed in acute anterior Poliomyelitis, in Landry's paralysis, in Herpes Zoster, in Meningo-myelitis. Total absence of leucocytic reaction is observed in softening and hemorrhage of the brain, cerebral tumors, syringomyelia, disseminated sclerosis, myopathies, neuritis, provided there is no syphilitic factor. Chorea presents not infrequently lymphocytosis because not infre- quently it is associated with a meningeal reaction. Bacteriology. — Microorganisms may be discovered in the cerebro- spinal fluid either by direct examination, culture or inoculation. Great varieties of microbes have been found: streptococcus, pneumococcus, Eberth's, Loffler's and Pfeiffer's bacilli and coli-bacillus. The most in- teresting ones are: meningococcus of Wechselbaum and tubercle bacillus. The first is the specific microorganism of the epidemic cerebro-spinal CEREBROSPINAL FLUID 373 meningitis, the second of tubercular meningitis (see the respective chapters) . Wassermann Reaction. — In 1901 Bordet and Gengou discovered the fundamental principle known as "absorption or deviation of the comple- ment." They introduced the terms antigen and antibodies to designate: the former — a substance which injected into an animal will produce an immune serum; the other — an antagonizing substance which is necessary for the immunizing action of the serum. There is a complement in every normal serum which is specific for each animal. Moreover if the blood corpuscles of one animal are injected into another of a different species, these corpuscles disappear with the production in the serum of a specific hemolysin. If the antibody or the complement is removed, the specific hemolytic action of the serum is lost. If the antibody is linked up to the antigen in the presence of the complement, both the antibody and comple- ment become inactivated. The method of determining if the blood serum or cerebro-spinal fluid contains the antigen or the antibody is known under the name of deviation of the complement. The determination of the presence of syphilitic antibodies and antigen constitutes the basis of Wassermann's test. Wassermann reaction is regarded at present one of the most valuable assets in medicine. It enables to decide difficult and perplexing diagnoses in obscure cases. It is extremely useful in Syphilis and Parasyphilis of the nervous system. It is the consensus of opinion that when it is posi- tive, it means syphilis, but when the reaction is negative, it does' not ab- solutely exclude syphilis, as some factors may influence the reaction. There is at a present sufficiently large number of facts proving that in latent tertiary syphilis the Wassermann reaction is the more rarely observed the more energetic is the treatment with mercury. A positive reaction changes into a negative one under the influence of mercury. Bizzozero (Mediz. Klinik, 1910) made investigations with reference to iodides. In a series of cases he observed that when under the influence of iodides the morbid symptoms improve, the positive Wassermann reaction equally becomes negative. Nichols and Craig (/. Am. Med. Ass'n., 191 1) have shown that after ingestion of considerable amounts of alcohol within twenty-four hours of making the test a positive serum may be rendered negative, and in some cases the serum will strongly give a negative reac- tion for as long as three days after the administration of alcohol. A careful inquiry therefore should always be made regarding the recent use of alcohol before the Wassermann test is made. On the preceding pages the importance of the leucocytic formula in the cerebro-spinal fluid was strongly emphasized. It is interesting to ob- 374 CEREBROSPINAL FLUID serve that there is no parallelism between the leucocytic reaction and Wassermann reaction. The first, for example, may be very marked and the Wassermann test negative for the cerebro-spinal fluid. Diagnostic Significance of Four Reactions in Organic Nervous Dis- eases. — Wassermann reaction in blood serum and cerebro-spinal fluid, also lymphocytosis and increase of albumen contents in the cerebro-spinal fluid appear to have an important value for differential diagnosis. Nonne (Deutsch. Zeitschr.f. Nerv., 191 1) has recently made a complete study of these four reactions in 167 tabetics, 179 paretics, ninety-seven indi- viduals with cerebro-spinal syphilis, sixty-eight cases of multiple sclerosis, thirty-eight cases of brain tumors, fourteen cases of spinal tumors. His important conclusions are as follows: I. Lymphocytosis and increase of albumen (Phase 1). There is almost constant presence of lymphocytosis in syphilis and parasyphilis ; it is less abundant in parasyphilis than in syphilis of the nervous system. In a few isolated cases there is absence of lymphocytosis. In eleven cases of tabes the latter was absent; four of these cases were stationary, seven in incipient period. Among the syphilitic forms, specific hemiplegia is most frequently without lymphocytosis and without increase of albumen. The spinal forms of syphilis always present lymphocytosis and Phase I. In organic nervous diseases other than of syphilitic or parasyphilitic character these two reactions are slight. However, in two cases of Mul- tiple Sclerosis the first reaction alone was present. In brain tumors the two reactions are usually absent or very slight. In tumors of the cord seven times out of fourteen Phase I was present, but lymphocytosis absent. In syphilis without involvement of the nervous system the two reac- tions are observed in 20 to 30 per cent. The two reactions have a practi- cal bearing: they help to ascertain whether the nervous system is in- volved. In neurasthenics, alcoholics, epileptics, whether they are syph- ilitic or not, the amount of albumen is normal. The increase of al- bumen has no absolute value for a differential diagnosis of syphilitic and parasyphilitic diseases of the nervous system, but it permits to differentiate functional from organic nervous diseases. Lymphocytosis has no absolute value for differentiation of syphilitic and parasyphilitic affectious. II. Wassermann Reaction with Blood Serum and Cerebro-spinal Fluid. — (a) A positive serum reaction is, with very rare exceptions, an indication of syphilis. Nonne found it in 60 to 70 per cent, of tabetics, in 90 to 95 per cent, of paretics, in 80 per cent, of syphilitic arteritis. He be- CEREBROSPINAL FLUID 375 lieves that a positive serum reaction controls the prognosis in syphilitic individuals. He believes that a negative Wassermann is almost a certain indication that there is no paresis. The degree of the positive Wasser- mann has a certain significance. A very strong positive reaction is more frequently observed in paresis than in tabes or cerebro-spinal syphilis. (b) A positive reaction with the cerebro-spinal fluid is observed in 100 per cent, of paresis. The frequency is less marked in tabes and still less in cerebro-spinal syphilis. The test with cerebro-spinal fluid is more important in paresis than with blood serum. When serum gives a positive result, but the cerebro-spinal fluid a negative, the disease is not paresis. With these four reactions errors of diagnosis may be easily avoided. THERAPEUTIC INDICATIONS WITH REGARD TO CEREBRO-SPINAL FLUID. A. Lumbar Puncture and Withdrawal of Fluid. — The withdrawal of cerebro-spinal fluid, which should not be above 20 to 30 c.c, is indicated in the following conditions. In congenital Hydrocephalus some favorable results had been reported. Permanent or temporary improvement in uremia has been observed. In tumors of the cerebrum or cerebellum relief of headache, vomiting and oedema of the papilla has been reported by very competent observers. However, the greatest care must be exercised in such cases and only very small quantities may be withdrawn, as rapid decompression may produce a fatal syncope. Epileptic seisures have been ameliorated in intensity and frequency from occasional withdrawal of small amounts of cerebro- spinal fluid. The main indication for lumbar puncture is in Meningitis. In acute meningeal conditions, in tubeicular meningitis, amelioration of symp- toms has been noticed. The most favorable results have been obtained in non- tubercular forms, cerebro-spinal, serous, purulent and syphilitic forms of meningitis. In some cases of serous meningitis excellent results have been obtained after one or several punctures. In Syphilitic headache good results have been obtained. Lumbar puncture has a distinct therapeutic value in meningitis of bacterial origin but non-tubercular. B. Injections of therapeutic agents into the sub-arachnoid cavity. The sub-arachnoid method of injection is superior to the subcutaneous one in view of the greater absorbent power of the sub-arachnoid cavity. This fact is being utilized for introduction into the latter of drugs and sera. Antitetanic serum, antimeningococcus serum — have both proven 376 CEREBROSPINAL FLUID to be of great utility. Sulphate of magnesia is also injected into the sub- arachnoid cavity in cases of tetanus with satisfactory results. Cocain, eucain, novocain, stovain, have been injected for analgesic purposes; they produce a rapid, complete and sufficiently durable anaesthesia to permit operative procedures on the lower extremities and some abdominal viscera. Certain inconveniences have been observed, viz. vertigo, headache, nausea — of which all may last several days. Finally, this method has been utilized to relieve pain occurring in tabes, sciatica, lumbago (see these Chapters). Sicard advised the use of the epidural method for injection of analgesic drugs. This method is easier and is not followed by the unpleasant symptoms of the first (see for details page 443). CHAPTER XXIV DISEASES OF THE PERIPHERAL NERVOUS SYSTEM NEURITIS (INFLAMMATION OF NERVES) Pathology. — An inflammation of a peripheral nerve may be confined to the connective tissue surrounding the nerve (perineuritis) or lying be- tween the individual bundles of the nerves (interstitial neuritis) or else to the nerve fiber itself (parenchymatous neuritis). In the majority of cases these forms are combined. Fig. 119. — Neuritis of Sciatic Nerve. (Original.) Examination shows a tumefaction of the tissue surrounding the nerve. Dilatation of the blood vessels, extravasation of blood and sero-fibrinous exsudation infiltrating the sheath, leucocytes around the vessels are the first changes. Gradually these alterations extend to the interstitial tissue which proliferates. The exsudation within the sheath produces pressure on the nerve-fiber itself. In the latter the myelin becomes broken up into fine particles. When the disease advances, the axis- cylinder itself takes part in the morbid process. It undergoes degener- ation and in more pronounced cases it atrophies and disappears. In cases of primary parenchymatous neuritis, viz. without the involvement 377 378 NEURITIS \, Inf. Hcemorrhoidal of Pudic Superficial Perineal of\ Pudic and Inferior Pudendal of small Sciatic Fig. i 20. — Distribution of Sensory Nerves in the Skin. {Flower.) NEURITIS 379 of the interstitial tissue, and which occurs as a result of a toxic influence, there is an exsudation with leucocytes around the blood vessels, segmen- tation of the myelin and breaking up of the axis-cylinder, swelling of the media of the blood vessels and proliferation in the adventitia. S 1 Fig. 121. Fig. 122. Sensory Radicular Distribution. {Bouchard and Brissaud.) Letters C, D, L and S indicate respectively cervical, dorsal, lumbar and sacral roots. In the form described by Gombault under the name of periaxile segmentary neuritis only some segments of the nerve-fibers are affected and the lesion consists of fragmentation of the myelin sheath, the axis- cylnders remaining intact. It is observed in toxic or infectious, also traumatic neuritis. 3 8o NEURITIS Recovery in neuritis corresponds to a process of regeneration. The latter consists of longitudinal division and sub-division of the portions of the axis-cylinder which remained intact and of gradual growth of the new filaments until they reach the tissues innervated by the nerve before its destruction; at this stage of their development they become covered by myelin. C8.D1 L 1.2 L3- Fig. 123. Letters C, D, L and S indicate respectively cervical, dorsal, lumbar and sacral roots. Etiology. — Infections, intoxications and local factors are the chief causes of peripheral neuritis. Microbes or their toxins in infectious diseases frequently produce a multiple neuritis, but they may also be the cause of a localized neuritis. An infected wound is likely to set up an NEURITIS 38l inflammation of a nerve. Trauma sets up an inflammation, which may involve also a nerve. An inflammation of a finger may extend to a nerve or nerves of the arm (ascending neuritis) . In such cases the neuritis is an upward extension of an inflammatory or septic process along a nerve or nerves. Suppurating joint or acute synovitis, diseases of bones (fracture, Fig. 124. Letters C, D, L and 5 indicate respectively cervical, dorsal, lumbar and sacral roots. tumor, caries), dislocations may produce a neuritis in the immediate neighborhood. Diphtheria, syphilis, cancer, tuberculosis may set up a toxic neuritis. Neuritis due, so to speak, to cold is often of toxic or infec- tious nature. Intoxications with alcohol, metals, carbonic acid, may pro- duce a localized neuritis. In the course of diabetes, gout, chronic rheu- 382 NEURITIS matism, not infrequently a neuritis (sciatica for example) is observed; autointoxication is probably the immediate cause. Finally neuritis may be the result of localized ischemia or anemia caused by partial or complete blocking of an artery such as embolus. Symptoms. — In diseases of peripheral nerves sensory disturbances are very conspicuous. The adjoining plates will render considerable assistance in the study of various sensory areas (Figs. 120, 121, 122, 123 and 124). The main sign of an inflammation of a nerve is pain. It is usually intense and aggravated by movement, or anything producing a congestion of the limb. The slightest touch or pressure upon the nerve produces pain. In ascending neuritis there is an excruciating burning pain along the nerve-trunk, also various paresthesias, as formication, numbness, etc. The objective sensory disturbances in the area of distribution of the nerve consists at first of hyperesthesia, but later of hypaesthe- sia or anaesthesia. Sometimes there is a perverted sensibility, such as cold taken for heat and vice versa. Motor disturbances usually ac- company the sensory. Twitching and tremor are present at the begin- ning, but when the condition becomes chronic, paralysis of the muscles innervated by the affected nerve may ensue. Trophic disturbances of the skin are frequent (cedema, cyanosis, erythema). Atrophy of the muscles with reactions of degeneration is very frequent. Sometimes the muscular atrophy may assume the type Aran-Duchenne (see this chapter). In a case recently reported by Long (Nouv. Iconogr. de Salpetr., 19 1 2) the atrophy first appeared in the left hand, three years later in the right and after twelve years it was a typical Aran-Duchenne form. Mi- croscopically the following changes were found: atrophy of a large number of nerve-fibers, proliferation of connective tissue between the fibers, hypertrophy of vasa nervosum; atrophy of muscular fibers; the spinal cord was intact except a few isolated cells in anterior cornua of the cervical enlargement which were found altered. A neuritis may be only motor or sensory, and then the symptoms are either only motor or only sensory. In the majority of cases it is of a mixed type. In lead intoxication the motor phenomena of neuritis are almost exclusively present. In syphilis sensory symptoms alone may be present for a long time. An insolated neuritis of a sensory filament of a nerve-trunk was reported by me in /. of Am. Med. Ass'n, 1909. It was a record of four patients in whom the lower cutaneous branch of the musculo-spinal nerve was involved. The symptoms were exclusively sensory (subjective and objective) ; motor and trophic disturbances were absent. Course, Termination, Prognosis.- — Neuritis usually lasts a long time — NEURITIS 383 from a few weeks to a few months; it may also last years. Its prognosis is, as a rule, good. It depends upon the cause. Recoveries are frequent. Rapid improvement predicts recovery. Neuritis of rheumatism and gout usually lasts a very long time. As a rule neuritis of long duration presents a bad prognosis as to complete recovery. The best outlook is in neuritis of traumatic origin. Diagnosis. — Marked changes in objective sensations, especially anaes- thesia over the area of distribution of the nerve, paralysis with atrophy and reaction of degeneration, are the chief and certain symptoms of neuritis. Treatment. — Rest of the affected limb is the first indication. Ideal rest is obtained when the limb is immobilized by appropriate means. This procedure gives considerable relief from pain. In addition to immobilization local applications of extreme cold or extreme heat are advisable. A very good procedure is to place the affected part in very hot water for about half an hour or even an hour, two or three times a day. In two cases the only relief could be obtained from a continuous immersion of the arm in hot water. Counter-irritation by fly-blister and sedative applications may be useful in relieving pain. In ascending neuritis the pain may be so intense that relief can be obtained only by opening the wound and removing the cause of irritation; if the latter does not help, nerve section or even amputation are the only means. Internally coal- tar products, sedatives should be given. Morphine should be resorted to as a last resort. Massage can be used only when the acute symp- toms, and especially pain, have subsided. Massage and electricity are advisable for combating the atrophy. When this treatment has failed and the pain is intense, surgical inter- vention, as nerve-stretching or nerve-section, is indicated. Neuritis of Ischemic Origin. — A total or incomplete obliteration of a blood vessel may produce irritation and cause a degeneration of a nerve and subsequently a paralysis. In such cases there is an insufficient blood supply to the nerve and muscles either by compression (ligature, surgical apparatus, tumors, enlarged glands) or by embolus, thrombus, obliterative arteritis. Cases of this kind have been reported by Broca, Charcot, Delherme, Babinski, Liston, Mally, Volkmann and others. The symptoms are: paralysis and atrophy with the reactions of degenera- tion. Pathologically there will be degeneration of the nerve, if the ischemia is complete and durable. In incomplete interruption of circula- tion the paresis of the limb disappears as soon as the obstacle is removed. Hypertrophic Interstitial Neuritis of Dejerine. — See chapter on Muscular Atrophies. 3§4 NEURITIS NEUROMA Neuroma tumors may be isolated and single or multiple. They usually grow in the sheath of a nerve-trunk. They may be soft (lipo- mata, myxomata) or hard (nbro-neuromata). The former are benign, the latter malignant. The first are usually single, they cause pain along the nerve, they are tender to touch; they can easily be removed. The malignant form (fibro-sarcoma) has a special predilection for the sciatic nerve. It has a progressive course and destroys the nerve, pro- ducing besides pain also paralysis and atrophy of muscles. Operation is the only remedy, but the prognosis is not certain. Multiple neuromata are fibrous tumors sit- uated either in the skin or on the nerve- trunks. They are usually diffuse. They are known under the name of Recklinghausen's disease. When they are located on the nerves they occupy the en- do-neurium; degeneration of the nerve-fibers follows. Pain is present and paralysis gradu- ally develops. Not infrequently the cranial nerves are the seat of these tumors. When the spinal roots are affected, the tumors may be found also in the cord. The subcutaneous tumors are usually soft and consist of plexiform bunches of nerve-fibers described by Verneuil. Other symp- toms: (i) pigmentation of the skin in brownish patches usually situated on the trunk and upper portions of the limbs; (2) mental manifestations, such as depression and diminution of intelligence. The prognosis is uncertain (Fig. 117). Neuroma following amputation consists of a bulbous mass developing on the proximal end surface of a divided nerve. It is due to a rapid growth of the sectional axis-cylinders and proliferation of the surround- ing connective tissue. It is usually very painful to pressure and thus it prevents from using an artificial limb. Twitching of the muscles is also observed and such a neuroma may be the point of departure for epileptic seizures. Removal of such tumors is a necessity but recurrences are. frequent. Fig 125. — Neurofi- bromatosis (v. Reckling- hausen's disease.) MULTIPLE NEURITIS 385 MULTIPLE NEURITIS Pathology. — The lesion described in the preceding chapter is the same when several nerves are simultaneously affected. The special feature of polyneuritis lies in the fact that the atrophic state of the nerves is more marked than the inflammatory, and that perineuritic and inter- stitial changes are extremely slight, while the nerve-fibers are markedly altered. Another peculiarity is found in changes in the central nervous system. Thus poliomyelitic foci in the cells of the anterior cornua, de- generative condition of the posterior columns (see my case in Amer. Medic, 1905), inflammation of the bulbar nuclei, have been observed Fig. 126. — Lead Multiple Neuritis, Showing Involvement 01 the Posterior Columns or the Cord. {Original.) sometimes. Evidently the chemical poison which is the chief cause of multiple neuritis affects almost all parts of the nervous system but to a different extent in various cases: sometimes the peripheral nerves, motor or sensory or both, at another time the nerves and the cord. Etiology. — Intoxications are the most frequent causes. Alcohol oc- cupies the first place. Lead, mercury, arsenic, phosphorus, carbonic acid gas, intoxications of alimentary origin (ptomain) are also frequent causes. Infectious diseases play a great role. Typhoid fever, diph- theria, small-pox, erysipelas, pneumonia, scarlet fever, grippe, dysentery and tuberculosis are not infrequently accompanied or followed by multi- ple neuritis. In these cases the toxins are the immediate cause. In lep- rosy and beriberi the neuritis is due to a direct action of microorganism. Syphilis, malaria, diabetes, puerperal state, tobacco and arterio- sclerosis are less frequent causes. 25 386 MULTIPLE NEURITIS The interstitial hypertrophic neuritis of Dejerine and Sottas is described in a separate chapter. Symptoms. — Irrespective of their causes all forms of polyneuritis present a common clinical picture, which will be described here; the special symptoms characteristic of each variety will be mentioned later. General Symptoms. — In the subacute form there is usually noticed a rapidly progressing loss of power in the lower extremities. The paraly- sis is more marked in the lower part of the limb than in the upper. When the disease advances, the upper extremities become affected. As the ex- tensors are particularly involved, various abnormal attitudes of the limbs are acquired. Wrist-drop and foot-drop are characteristic of polyneuritis. The gait is typical: being unable to flex the foot, the patient is obliged at each step to flex the thigh on the pelvis in an exaggerated manner. This raising of the thigh with the toes of the foot hanging down is analogous to the gait of horses. It is called "steppage gait." The paralysis is flac- cid. While it affects most frequently the extensor muscles, it may never- theless attack any muscle or group of muscles. The tendon reflexes are abolished or markedly diminished. Gradually the muscles of the thorax and abdomen become affected and when the bulbar nerves are invaded the condition is alarming. The electrical reactions of the paralyzed muscles present the following changes. At first there is a diminution of response to f aradic and galvanic currents. Later on the faradic contractility is abolished and reactions of degeneration are manifest. Muscular atrophy follows the paralysis, and its beginning corresponds to a destruction of the axis-cylinders of the nerves distributed in the mus- cles. It is very frequent in polyneuritis, and it may make its appearance at any stage of development of the paralytic symptoms. Sensory disturbances are very common. They consist of all sorts of abnormal sensations, as tingling, burning, numbness, etc.; of spontaneous pain or of pain produced by the slightest pressure or movements of the limb; of objective modifications of all forms of sensations (hypassthesia, anaesthenia, hypalgesia, analgesia) ; of loss of muscular sense (patient is unable to appreciate the position of the affected limbs). Vaso -motor disturbances are occasionally observed, especially in poly- neuritis of external origin (traumatism, compression, tumors). They are ulcers, eruptions, oedema, hyperhidrosis of hands and feet. The sphincters are usually intact. In the course of multiple neuritis paralysis of cranialnerves may occur. The oculo-motor, the abducens, the optic, the facial, the pneumogastric are sometimes involved. Strabismus^ diplopia loss of light reflex, optic MULTIPLE NEURITIS 387 neuritis, amaurosis, facial palsy, paralysis of the vocal cords, tachy- cardia, difficulty of respiration — are all observed occasionally. The acute form of multiple neuritis differs somewhat from the preced- ing form. The onset is here sudden, accompanied by chills, fever and headache. Rapidly the symp- toms develop. Paralysis, loss of reflexes, objective sensory disturbances, etc., appear in the same succession as in the previous form. Course, Termination, Prognosis. — In the sub -acute form recovery follows the ma- jority of cases. The paralysis, the atrophy, the sensory dis- turbances gradually improve, the electrical reactions become normal. In some cases the paralysis persists, retraction of tendons and immobilization of joints take place and the pa- tient is permanently crippled. In exceptional cases the respiratory muscles are paralyzed and death may ensue from pulmonary complica- tions. The acute form (not to be confounded with Landry's type of polyneuritis) bears practically the same prognosis as the subacute. Fig. 127. Double Wrtst-drop in a Case of Lead Palsy. Special Features A. Alcoholic Polyneuritis. — This is the most frequent of all forms of multiple neuritis and more in women than in men. The disturbances are both motor and sensory. The latter are very conspicuous; they may be pronounced while the motor power is only slightly disturbed. The objective and especially the subjective sensory disorders described in the preceding chapter are particularly well developed. The patient complains early of unusually severe pain, which may be lancinating, tearing or pull- ing. The least pressure shows exquisite tenderness. The pain leads to' insomnia, to mental depression. Cramps in the calves of the legs, muscular twitchings occur frequently. Intestinal or gastric pain occurring in paroxysms is common. Muscular hyperaesthesia is particularly marked; the least pressure, even the contact of bed-clothes is intolerable. Cutaneous anaesthesia in the limbs is usually only in their distal portions. 388 MULTIPLE NEURITIS The paralysis, gait, reflexes are as described in the general sympto- matology. However in alcoholic neuritis the bilateral paralysis of the extremities has a tendency to generalization ; it gains the proximal ends of the limbs, the thorax, the abdomen. The muscular atrophy is pronounced. The paralysis and atrophy produce deformities of the limbs. When the muscles of the back are involved, there is lordosis. When the glutei are involved, the patient is unable to rise from his seat. In paralysis of the abdominal muscles the expelling power of the bladder and rectum is weakened. If the muscles of the neck are affected, the head cannot be moved voluntarily. Involvement of the intercostal muscles interferes with respiration. Very rarely the muscles of the face are involved. Tremor of the tongue and weakness of the muscles participating in articu- lation of words may give the impression of paresis. Among the vaso -motor disturbances oedema of the lower limbs is very common. Profuse sweating is frequent. Ocular disturbances are frequent. Retro-bulb ar optic neuritis, central scotomata, strabismus, ptosis and sluggish pupillary reflex to light — are all met with in alcoholic polyneuritis. Finally psychic disturbances are sometimes observed. Besides various manifestations which are usually encountered in alcoholism, such as delirium, confusion, stupor and hallucinations, there is a special symptom-group which sometimes accompanies polyneuritis. This is the so-called Korsakoff's psychosis. It consists chiefly of confusion with illusions of identity, loss of orientation, of memory. Delirium and hallucinations may also be present. The amnesia covers the period for recent events and for the time of the illness. The various lapses of memory are filled by the patient with a great variety of fabrications or confabulations. The patient described events and occurrences which never took place and does it with minutest details. The amnesia for recent events is considerably facilitated by an inability to associate or synthetize new facts. The disturbance in associa- tion of ideas is limited to new perceptions and conceptions. The vol- untary psychic activity is diminished, but the patient preserves the old intellectual acquisitions and his judgment on those old events is intact. The affection lasts from a few weeks to several years. It terminates either in death, or in a more or less complete recovery, or else in dementia. The course of alcoholic polyneuritis is usually subacute, lasts several months or even longer and in a large number of cases results in recovery. In some cases the recovery is incomplete: deformities of the extremities MULTIPLE NEURITIS 389 remain permanent. Pulmonary tuberculosis is not infrequently the cause of death in the protracted cases. The prognosis is serious, as bulbar symptoms may develop at any time and hasten death and on the other hand deformities render the patient powerless. The acute cases have ordinarily an unfavorable prognosis. In a case that I had under my observation for eight months there were besides the involvement of the limbs also bulbar symptoms (difficulty of deglutition and of respiration, tachycardia) and mental disturbances; the patient nevertheless made a perfect recovery. Recurrences of attacks is another special feature of the alcoholic multiple neuritis. B. Lead Neuritis (Lead Palsy). — Pathologically it presents the'periaxile segmentary form of Gombault (see Pathology). The paralysis presents a strictly localized character. The upper extremities are the seat of predilection for lead palsy. The latter is bilateral and symmetrical. It may present the antribrachial type, Aran-Duchenne's type, also the scapulo-humeral type (see Muscular atrophies, also my contribution in New York Med. Jour., 1906). The first is the most common. Gradually and insidiously the extensors of the fingers, except the long supinator and the long abductor of the thumb, are paralyzed. This leads to a special attitude of the hand. The latter is in pronation and flexion (wrist- drop), also the fingers are slightly flexed. Muscular atrophy with reactions of degeneration, also loss of 1 en exes develop quite early. Cyanosis of the hands is frequent. Sensory disturbances are usually absent. In some cases the onset of paralysis is preceded by some pain cr parassthesia. Optic neuritis, amblyopia, contraction of the visual field are sometimes observed. In rare cases lead palsy may become generalized. It may invade the lower extremities and even the trunk. When the lower extremities are affected, the peroneal muscles are paralyzed, but the tibialis anticus is usually spared. In acute cases of lead intoxication the paralysis may become general- ized. Paralysis of the diaphragm and intercostal muscles causes dyspnoea and the condition becomes alarming. Ordinarily the disease has a chronic course. In a large majority of cases the symptoms improve and even dis- appear completely. Recurrences are not infrequent. The prognosis of lead neuritis is as a rule favorable, but the damage done by lead on other organs and tissues may be so great that life is in danger, as for example, in cases with renal complications. In making the diagnosis, some aid may be obtained from other symptoms of lead poisoning, such as the blue line on the gums, history cf colic, encephalopathy, tremor. The distinction 390 MULTIPLE NEURITIS between wrist-drop from lead neuritis and that from musculo-spiral palsy lies in the retention of power of the long supinator muscle in the first affection. Schoenfeld (Mediz. Klinik, May, 1913) has recently called attention to the importance of examination of blood in lead poisoning. He points out as a characteristic finding the basophil granulation in the red cor- puscles and the change in tint of these basophil substances under the action of certain stains. These blood findings are also useful to control the progress toward recovery; in 18 of 40 patients thus kept under ob- servation the blood returned to normal after the patients had changed their occupation. Negative findings exclude lead intoxication. C. Arsenical Neuritis (Arsenical Paralysis). — This form is a mixed type of neuritis : motor and sensory. It affects the four extremities, the lower before the upper, and while the extensors are particularly involved, the paralysis affects also the flexor muscles (unlike alcoholic and lead palsies). Ataxia is frequently observed, and this in addition to the loss of reflexes, gives the impression of tabes (arsenical pseudotabes of Dana). Psychic disturbances are sometimes observed. Muscular atrophy with reactions of degeneration, sensory and trophic disturbances are identical with those of other forms of polyneuritis. Subjective sensory disturb- ances are usually conspicuous and persistent. They are: severe tearing pain in the limbs, burning in the toes and soles of the feet. The skin and muscles are very tender to pressure. This hyperalgesia is frequently associated with anaesthesia to touch, pain and temperature. The atrophic paralysis may spread and involve not only the proximal ends of the limbs, but also the diaphragm (the intercostal muscles usually escape) . Tremors and spasmodic contractions have been observed. Loss of vibratory sense or osseous sensibility in the lower extremities was observed by Byrnes (/. Amer. Med. Ass'n., 1909). Among other symptoms of arsenical neuritis may be mentioned: pigmentation of the skin, vesicular eruption, erythematous and cedematous condition of the soles and of the palms. Epileptiform convulsions have occasionally been observed. The prog- nosis in arsenical neuritis is usually favorable. In 1900 an epidemic of arsenical neuritis occurred in Manchester in beer drinkers. The analysis of the beer discovered the presence of arsenic. The arsenic was derived from sulphuric acid employed in preparation of glucose used in brewing cheap beer. Arsenical neuritis may occur from medicinal use of arsenic. Thus it was observed in patients suffering from chorea who used arsenic. D. Diphtheritic Neuritis (Diphtheritic Paralysis).— It usually occurs during convalescence from diphtheria. It affects first the palate and develops slowly. At the beginning there is only some disturbance in MULTIPLE NEURITIS 39 1 phonation and deglutition, but gradually the difficulty becomes greater. The palatine velum hangs down and does not contract during phonation. The mucous membrane is anaesthetic. The food passes through the nose. As the paralysis extends to the pharynx and larynx, the epiglottis does not close the larynx; food penetrates into the respiratory passages and produces pneumonia or else direct asphyxiation. This is also aided by anaesthesia of the larynx. The phonation is nasal, labial letters are pronounced indistinctly. When the paralysis reaches the recurrent laryngeal nerve, hoarseness and aphonia will be the result. The eye muscles are infrequently involved. Strabismus with diplopia, ptosis, disturbed pupillary reflexes (paralysis of accommodation with preserva- tion of light reflex), amblyopia are all observed. When the paralysis shows a tendency to generalization, the lower extremities become affected after the palate, pharynx, larynx and eyes. Like in the preceding forms the antero-external group of the leg muscles are paralyzed. Steppage gait with foot-drop is present. Ataxia, loss of muscular sense, loss of reflexes, intense pain gives the impression of tabes (pseudo-tabes). The muscles of the upper extremities, also of the trunk, may follow those of the lower. When atrophy develops, it is usually rapid and reactions of degeneration are present. The muscles of the tongue, lips and face may be affected. When the pneumogastric and the phrenic nerves are involved, cardio-pulmonary disturbances are present. The course of the disease depends upon its intensity and localization of the poison. When bulbar symptoms are present, death usually ensues. Dyspnoea, syncope, asphyxia, aspiration pneumonia are all the causes of death. Generally speaking the prognosis is favorable, especially when the paralysis is confined to the palate. In the latter case the dura- tion is about two to three weeks. When the paralysis is generalized, it lasts several months. The special feature of diphtheritic palsies lies in rapid development and the instability of the motor symptoms, which disappear in one place to appear in another and then again reappear in the first. E. Carbonic Gas Neuritis. — In cases of intoxication with carbon dioxide the following special symptoms are present: anaesthesia of the extremities; paralysis at first of antero-lateral muscles of the legs and later of the muscles of the upper extremities; preservation or increase of tendon reflexes; trophic and vaso-motor disturbances; mental dis- turbances (particularly amnesia). Recovery follows almost in every case. Mental feebleness and anaesthesia remain a long time, even in the most favorable cases. 39 2 MULTIPLE NEURITIS F. Mercurial Neuritis is manifested by paresis or paralysis of the antero-external groups of muscles of the legs and sometimes also of the upper extremities, by anaesthesia in certain areas and hyperesthesia in others, finally by enfeeblement of the mental faculties. G. Puerperal Neuritis may occur either during pregnancy or after labor. In the first case usually the nerves of the lower limbs are involved, in the second case the nerves of the upper limbs are affected. Here the neuritis presents certain peculiarities in its localization. It affects especially the terminal branches of the median and ulnar nerves, and mostly on the right side. Sometimes it becomes generalized and then involves the lower limbs. Sensory disturbances are slight. The disease is probably infectious in nature in cases which occur after labor. In neuritis occurring during pregnancy the etiological factor is probably disturbed metabolism. It usually ends in recovery. H. Beriberi or Kakke. — Under this name is known a form of endemic or epidemic multiple neuritis, considered infectious in origin, and met with in South America, Japan, Philippines, China. Its microorganism has not yet been discovered. It is supposed to enter the body thiough the gastro-intestinal tract. The disease has been observed in connection with prolonged consumption of mouldy. rice. A very recent experimental study by Strong and Crowell {Philippine Jour, of Science, 1912) shows that there is no evidence which could suggest that beriberi is an infectious disease, that beriberi in the Philippine Islands is due to a prolonged consumption of a diet which lacks certain substances necessary for the normal physiologic needs of the human body. For prevention and cure of beriberi it is necessary that man shall be supplied with a liberal nutritious diet suitable to the physiologic needs of the body. As to the question of rice, although a rice containing 0.4 per cent, of phosphorus will prevent the appearance of polyneuritis in fowls, nevertheless from these two authors' experiments it is evident that beriberi in man may be produced by rice containing 0.37 per cent, of phosphorus pentoxide. The higher the phosphorus content of rice the less is the liability of that rice to produce beriberi. The following are the chief symptoms of the disease. As an early manifestation must be mentioned a cardiac syndrome consisting of smallness and rapidity of the pulse, dilatation of the heart, systolic murmur. Attacks of cardiac insufficiency are not rare (Edema is another frequent early symptom. It affects the lower extremities, abdomen, neck and face, also the viscera; pericarditis is not rare; increase of cerebro-spinal fluid in the spinal canal has also been observed. Albuminuria is usually absent. Absence of fever is the rule. The nervous symptoms which usually MULTIPLE NEURITIS 393 develop after an acute onset consisting of nausea, vomiting, oppression, are: (1) paralysis of the lower extremities and especially of the antero-external group of muscles; the gait, reflexes, attitude are same as in other forms of polyneuritis. The upper extremities, thorax, may also become involved, (2) anaesthesia cf the affected limbs and spontaneous pain, (3) muscular atrophy, (4) mental disturbances similar to those of alcoholic polyneuritis, (5) respiratory disturbances. The latter symptom, also the above-men- tioned cardiac condition, are due to the involvement of the vagus and phrenic nerves. Rapid development of cardiac and pulmonary disturb- ances is characteristic. Beriberi has been observed under three varieties: (1) the humid or hydropic form in which the oedema is most conspicuous; (2) the dry or paralytic form in which there is no oedema but paralysis of the muscles is the most prominent symptom; (3) mixed form in which the symptomatology is complete. In all forms of the disease the cardiac symptoms are invariably present. The duration of the disease is from a few weeks to several months. The prognosis is grave, although recovery is possible. Death usually occurs from the cardiac failure or from some inter- current disease. The treatment does not differ from that of other forms of multiple neuritis. Special attention, however, should be given to the condition of the heart. Lumbar puncture may be useful in view of the increased tension of the cerebro-spinal fluid. I. Lepra Neuritis (Nerve Leprosy). — In this form of leprosy the changes are found almost exclusively in the peripheral nerve trunks and their cutaneous branches. Changes in the central nervous system, if present, are secondary to the general leprous infection. Thickening of the nerves (and sometimes nodules) is very frequent in the early period of the disease, but in the advanced stage atrophy is usual. The leprosy bacillus is found in the connective tissue between and around the nerve- fibers. This tissue proliferates and through pressure on the nerve-fiber itself produces its atrophy (Figs. 128 and 129). Clinically trophic disturbances are observed. The joints of the phalanges are commonly affected. Ulcerations with subsequent necrosis are characteristic. The phalanges gradually fall off. The process may begin at the nail and then progressively involve the entire digit. The skin presents patches of discoloration of bronze color. Small areas of thickened skin on the ears and face have been observed (due to a local accumulation of bacilli). In the majority of cases the hands present a claw-like appearance with marked atrophy of Aran-Duchenne's type (see this chapter). Reac- 394 MULTIPLE NEURITIS tions of degeneration are present. The atrophy may not be confined to the extremities but also involve the muscles of the trunk and face. Objective sensory losses are usually found over the distal portions of the limbs. Syrin- gomyelic sensory dissociation is common, but at the beginning there is a long period of hyperesthesia with pain in the ex- tremities. The relationship between syringomyelia and leprosy is according to competent authorities a very intimate one. Prognosis is, generally speaking, un- favorable, although the disease may last several years. The disease may be also self-limited. [ The treatment, is mostly hygienic. Alteratives, also strychnia, may be tried. J. Senile Neuritis has been described particularly by Oppenheim. He observed a slight paralysis of the distal ends of the limbs, which develop very gradually. Pain and hyperesthesia may or may not be present. The degenerative state of the peripheral nerves is probably due to the deficient blood supply caused by athero- matous arteries characteristic of old age. Fig. 128. — Leprosy. Muti- lated toes, ulcerations, self-amputa- tions, constrictions around phalan- ges. Patches of discoloration of skin of the face and feet. Fig. 129. — Leprosy on Hands. Diagnosis of Multiple Neuritis. — The above described general symp- toms of polyneuritis and the special symptoms of each individual form MULTIPLE NEURITIS 395 are sufficiently characteristic for establishing the diagnosis. There are, however, a few maladies which sometimes resemble closely polyneuritis. They are: Acute myelitis, anterior poliomyelitis, tabes. When the paralysis due to myelitis is spastic, the disease cannot be confounded with polyneuritis, as the state of the tonicity of the muscles, the reflexes, the gait and the condition of the sphincters are radically different in the two affections. The flaccid form of myelitis presents some difficulty of differentia- tion. Here the anaesthesia is much pronounced and affects almost sym- metrically the entire lower extremities (in polyneuritis the objective sensory disturbances are slight); the involvement of the bladder and rectum, also sexual disturbances, are constant (in polyneuritis they are usually absent); bed-sores, also ulcerations of the heels are constant (absent in polyneuritis) . In acute anterior poliomyelitis the paralysis affects chiefly the roots of the limbs while the distal ends are not involved (in polyneuritis the condition is reversed); the cranial nerves are rarely affected (in poly- neuritis frequently) ; fibrillary contractions are present (absent in poly- neuritis); sensory disturbances are absent (present in polyneuritis); the distribution of paralysis, of atrophy and electrical contractility is symmetrical (irregular in polyneuritis) . In tabes the etiology is different from that of polyneuritis. In the former the presence of Argyll-Robertson's pupils, of disturbances of the sphincters, of optic atrophy, gastric crises, exaggerated superficial ab- dominal reflexes, thoracic bands of anaesthesia, finally the absence of tenderness of the nerve-trunks (so characteristic of polyneuritis) will decide the diagnosis. Lymphocytosis of the cerebro-spinal fluid is present in tabes, absent in polyneuritis. Treatment. — The first indication is to remove the cause. In cases of intoxication with alcohol the patient should be isolated; in poisoning with lead or mercury the patient is advised to discontinue his occupa- tion. In diphtheritic polyneuritis administration of antitoxin may be of benefit. In syphilitic cases mercury and iodides are indicated. Iodides are also beneficial in lead neuritis. In rheumatic cases salicylates, iodides, diuretics, diaphoretics are of use. When pain is present, it will be relieved by rest in bed, hot applications or hot baths, also by coal-tar products (aspirin, phenacetin, etc.), sedatives (bromides). Morphia should be avoided. When the acute stage has subsided, massage and electricity will be very useful for combating the oncoming atrophy and perhaps for relieving the pain. As soon as pain has subsided, the patient should be allowed to use his limbs and this is aided by daily 39^ MULTIPLE NEURITIS massage, electricity and systematic exercises especially in cases of in- coordination. When permanent contractures with deformities are present, surgical intervention is necessary. General hygiene, good nutritious food are essential, and in case of emaciation, iron and tonics should be added. ACUTE ASCENDING PARALYSIS (LANDRY'S PARALYSIS) Pathology. — The findings in Landry's paralysis are not constant. Frequently are seen changes in the spinal cord. In such cases there may be disseminated inflammatory foci (myelitis), or a certain degree of anterior poliomyelitis or else changes in the pyramidal tracts. The cells of the anterior cornua appear smaller, their contour is irregular. Their chromatolysis is marked. The most pronounced changes in the majority of cases are seen in the lumbar enlargement, which corresponds clinically to the earliest involvement of the lower limbs. The axis cylinders and their myelin sheaths are in a state of degeneration. The neuroglia cells are smaller and in a state of degeneration; sometimes hyperplasia is present. The blood vessels may be normal or more frequently present a leucocytic infiltration; in the latter case rupture may take place; puncti- form hemorrhages are not rare. The roots, especially the anterior ones,, and the peripheral nerves are involved. Disintegration of myeline and subsequently atrophy of the axis cylinders are characteristic. When the meninges are involved, they present all degrees of inflammation. The alterations just described are not all present in every case. In some cases there are only central changes. In some cases there are evidences of neuritis. In other cases there may be a combination of spinal and neuritic lesions with or without involvement of the medulla. The ob- served alterations are variable as to their localization and intensity. Acute ascending paralysis is an affection of the peripheral motor neurone involving separately or simultaneously every constituent portion of the neurone. Finally in a certain number of cases no lesion whatever has been discovered. The cerebro-spinal fluid presents also a variability in findings. The leucocytic formula may be either normal or exaggerated. Lymphocy- tosis or polymorphonucleosis have been found. However it may be said with some degree of certainty that abundant leucocytosis is in favor of central origin (spinal cord) of the disease, while absence of leucocytosis is in favor of its neuritic origin. Etiology. — All the evidences point to an infectious origin of the malady. It may develop in the course of various infectious diseases or independently. By means of injections o'f cultures in animals typical MULTIPLE NEURITIS 397 Landry's paralysis had been reproduced experimentally (Thoinot and Masselin). The microorganism has not yet been discovered. A recent case reported by Bolten (Berl. klin. Wchn., Jan., 191 1) seems to confirm the contention that Landry's paralysis is the result of intoxication of the spinal cord, the toxin interfering with the function of the spinal and bulbar centers. The toxin seems to affect the motor neurone alone. On this basis 600 c.c. of cerebro-spinal fluid were withdrawn and substituted by 540 c.c. of non-toxic fluid, viz. salt solution. The patient recovered. Among other etiological factors may be mentioned: cold or extreme heat. Men are more affected than women, adults more frequently than children or aged individuals. Symptoms.- — The clinical picture may be either that of poliomyelitis or polyneuritis. There is almost always a prodromal stage lasting from hours to weeks. General malaise, pain in the back and various parses- thesias (tingling, numbness) in the limbs usually precede. The onset is always rapid or even sudden. The disease in typical cases begins with paralysis of the lower extremities. Rapidly the upper extremities and the trunk become invaded. They are followed by the muscles of the head, neck, the tongue, palate, larynx and pharynx. Difficulty of deg- lutition and of respiration, slowness or else acceleration of the pulse appear. The patient dies from asphyxia. Ocular palsies, as well as facial palsy, have been observed in some cases. The paralysis is flaccid. The reflexes, deep and superficial, are abolished. Sensations are very slightly or not at all disturbed. The sphincters are usually intact. The muscles are usually normal and no reactions of degeneration are present. Fever is, as a rule, absent. In atypical cases the onset of paralysis may be preceded by chills, headache, malaise. The paralysis of the lower extremities is gradually followed by involvement of the sphincters and of the upper extremities and sometimes of the medulla. Among the atypical cases can also be mentioned those in which the paralysis begins with the upper extremities. In another series of cases there may be also the typical picture of multi- ple neuritis with pain and tenderness of the nerve-trunks, etc. Course, Termination, Prognosis. — In the majority of cases the termination is fatal. Recovery rarely occurs. In the latter case the symptoms gradually improve. The patient may recover, particularly when the bulbar symptoms disappear. In such cases muscular atrophy will be observed; it makes its appearance at the end of a few weeks. The atrophy is then diffuse. Reactions of degeneration are absent; there is only a diminution of response to electric stimulation. In fatal cases the duration is only of a few days (three to eight) . 39§ MULTIPLE NEURITIS Diagnosis. — The rapidity of invasion, the mode of development of the paralysis, viz. the ascending course, are characteristic of the disease. From acute anterior poliomyelitis it will be differentiated by the rapid course and bulbar involvement. Besides, the prodromal symptoms such as malaise, gastro-intestinal disturbances, etc., are more pronounced in acute poliomyelitis. Asymmetry in the involvement of muscles on. both sides of the body is characteristic of poliomyelitis. Rapid improvement in some muscles within a few days, early atrophy and RD are typical only of poliomyelitis. Treatment. — At the appearance of the first symptoms the chief in- dication is elimination of the infectious element. This can be accom- plished to some extent by proper elimination. Diuretics, purgatives and diaphoretics should be administered. Cauterization of the spinal column is advisable. Internally ergot, and in specific cases, mercury may be of some use. If the patient survives, and paralysis with atrophy becomes chronic, massage and electricity are useful. PERIODIC PARALYSIS Westphal in 1885 first called attention to an affection which is analo- gous to polyneuritis. It is characterized by attacks of paralysis in the intervals of which the patient is perfectly normal. The attacks may last from several hours to two days. The palsy affects all voluntary muscles except those of the face, tongue, pharynx, mouth, eyes, also those of the sphincters. The lower as well as the upper extremities may be equally involved, although the latter more frequently than the first. The paraly- sis develops rapidly and it is flaccid. It is usually preceded by a tingling sensation and even some pain. As a rule the lower extremities are first attacked, then follow the trunk and arms. Sometimes some muscles of the neck are affected: the patient can move his head when it lies on a pil- low, but he cannot raise it. The muscles innervated by the cranial nerves are intact. The sphincters are intact and if constipation is pres- ent, it is due to a paralysis of the abdominal muscles. The return of power is done in a manner reverse to the development of paralysis. Thus the arm first and the legs last regain their motility. The reflexes are abolished or markedly diminished. The electrical contractility of mus- cles and nerves is markedly diminished or totally abolished, although the muscles may contract when the nerves are being stimulated. Sensations and pupillary reflexes are normal. A very frequent symptom is sweat- ing during the attacks which may last between fifteen minutes and two days. The attacks may be severe or complete and mild or incomplete. In the latter case there is only paresis of the muscles. DISEASES OF CRANIAL NERVES 399 As to less frequent symptoms, there may be disturbance of circulation: dilatation of the heart; weak and irregular pulse; pallor of the face; ex- cessive thirst; congestion of the conjunctivae and of the respiratory tract, slight spasm of the affected muscles. Consciousness is preserved. The etiology is not definite. The hereditary character of the disease is well known. Holtzapple reports seventeen cases in four generations. The exciting causes may be either rest or exertion. In the first case the attacks occur after a night's sleep or after the patient is seated and rests a certain time. In the second case violent exertion brings on an attack. In one of my patients, girl of fourteen, an attack occurred after a long walk or dance; she would develop paralysis first in the arms and then in the legs; it lasted from twenty-five minutes to eight hours. Her uncle was similarly affected. Holtzapple observed attacks after indulgence in food. The prognosis as a rule is favorable to life, because the attacks become less frequent with advancing age. Holtzapple, however, observed six deaths during attacks. Difficult respiration during an attack is of serious moment. The pathogenesis of the disease is unknown. A diminished secre- tion of urea was found in several members of the same family. Necrop- sies have been negative. The trouble probably lies in a developmental defect of the muscles themselves. Treatment.— Avoidance of overexertion, of stimulants, of over-feeding is indicated. In my case mentioned above I succeeded in shortening the attacks with, the administration of sodium bromide in gr. x doses every two hours. Massage was also used in my case. The results were satisfactory. DISEASES OF INDIVIDUAL NERVES I. Paralysis of Cranial Nerves A. Paralysis of the First Nerve (Olfactory). Symptoms. — Loss or impairment of the sense of smell (anosmia) is the prominent symptom. As the taste is dependable to a large extent upon the integrity of smell, the former will be involved when anosmia is present. When the olfactory nerve is in a state of irritation, hallucinations of smell (parosmia) may occur. Subjective sensations of smell may occur as an aura preceding epileptic seizures in cases of tumor of the uncinate portion of temporo- sphenoidal lobe. Etiology. — Diseases of the nasal cavities (in the upper portion of which are distributed the fine olfactory nerves), viz. tumors, catarrh, dis- eases of the bony structure, meningitis at the base, trauma of the nose, 400 DISEASES OE CRANIAL NERVES fracture at the base of the skull in the anterior fossa — are all apt to irritate or destroy the filaments of the olfactory nerve. Treatment. — Removal of the cause is the sole therapeutic indication. B. Diseases of the Second Nerve (Optic) are discussed in the chapter on Diseases of the Brain. Optic Neuritis and optic atrophy were mentioned and their significance emphasized in intra-cranial diseases (especially tumors, abscess of the brain, basilar meningitis, choked disc in cerebellar tumors), in tabes, insular sclerosis, Freidreich's ataxia, paresis. It may be added here that optic neuritis may be encountered also in acute febrile diseases, toxic conditions (see polyneuritis), anaemia, diseases of orbital region, Bright's disease, gout. Optic atrophy may also develop in syphilis, malaria, diabetes, abuse of tobacco, narcotics or other drugs (quinine). In optic neuritis the disc is swollen and hyperemic. When the veins become greatly distended and oedema develops because of the interference with the return circulation, the condition is "choked disc." Vision may remain intact in optic neuritis and even in choked disc. On the other hand, there may be various degrees of impairment of vision or complete loss of vision. Loss of vision (amaurosis) may occur in the course of infectious diseases and develop very rapidly. Widal, Joltrain and Weill (Presse. Med., 1909) report a case of amaurosis in typhoid fever accompanied by cedema of both papillae and hypertension of the cerebro-spinal fluid. Two lumbar punctures produced complete recovery from the eye symptoms. Optic atrophy may be primary like in tabes or multiple sclerosis, or secondary following optic neuritis. In primary optic atrophy the loss of vision is usually gradual. Besides, there may be loss of vision only in one portion of the visual field, either centrally or peripherally. Neuritis and atrophy of the optic nerve may be the result not only of organic diseases of the nervous system (see above), but also of localized disturbances in the nerve itself, such as embolism of the central artery, hemorrhage and trauma. The pupillary reactions are frequently affected in diseases of the optic nerve; impairment or loss of light reflexes is common. Diseases of the optic chiasm were discussed in the chapter on Hemi- anopsia. C. Paralysis of Third, Fourth and Sixth Nerves (Opthalmoplegia.) Symptoms. — They are general and special to each of the three nerves. General. — Strabismus is very conspicuous. It consists of a deviation of the eye globe. In the view of the paralysis the excursion of the latter is limited. For example, if the external rectus (sixth nerve) is involved, the DISEASES OF CRANIAL NERVES 40 1 eye globe will move in every direction except externally. Under secondary deviation is understood a deviation of the normal eye when the paralyzed eye endeavors to fix an object. This secondary deviation is always greater than the paralytic deviation. The consequence of strabismus is double vision (diplopia) because the image of an object will be formed in both retime at various levels. Diplopia produces not infrequently vertigo and headache. Vertigo may disappear when the vision of the diseased eye is entirely removed or else corrected by some means. Special Symptoms, (a) Paralysis of the Third Nerve (Oculomotor.) — It may be complete or partial. In complete palsy the levator palpebral superioris, the superior, inferior and internal recti, the inferior oblique, the sphincter of the pupil and the ciliary muscles are all involved. Ptosis is the striking symptom. The upper eye-lid is lowered. The eye globe is deviated externally and downward. The pupil is dilated, does not react to light or to accommodation. Diplopia is marked: one image is placed higher than the other. In partial paralysis any one of the muscles may be affected. When the internal rectus alone is paralyzed, there is divergent strabismus: the images in both eyes are at the same level. When the inferior rectus is paralyzed, the eye looks upward and outward, the head is lowered. In paralysis of the superior rectus, the head is raised and thrown backward. When the inferior oblique is involved, the eye is directed downward and inward. In nuclear lesions of the third nerve the internal muscles may escape, but the external are paralyzed. (b) Paralysis of the Fourth Nerve (Pathetic).— The superior oblique is involved. The eye globe looks upward and inward. Diplopia exists only in the lower part of the visual field. (c) Paralysis of the Sixth Nerve (Abducens).- — The external rectus is involved. The eye globe is turned inward (convergent strabismus). Ocular palsies may be confined to one nerve or to all the three nerves. As to the third nerve several branches may be affected at the same time. Ophthalmoplegia is present when all the muscles of the eye are involved or at least the muscles innervated by two nerves, one of which is the third nerve. In external ophthalmoplegia the extrinsic muscles of the eye, in internal ophthalmoplegia the intrinsic muscles (the sphincters of the pupil and the ciliary muscles) are paralyzed. Ophthalmoplegia may be nuclear and peripheral. The first is the most important. When it is nuclear and external, it is usually bilateral. The facies of the patient is then characteristic: the ptosis gives a drowsy, 26 402 DISEASES OF CRANIAL NERVES sleepy expression, the eye globes are immobile, but the pupillary and accommodation reflexes are normal. Alternating ptosis is quite frequent. When the ophthalmoplegia is nuclear and internal, mydriasis is present and the pupils do not react to light, to accommodation and to convergence. Pupillary reactions. Iridoplegia is present when the light reflex of the pupil is lost. If with the latter the accommodation reflex is preserved, we are in presence of Argyll-Robertson pupil. The lesion in this phe- nomenon is very probably in the ciliary ganglion according to the researches of Marina and others. Paralysis of accommodation or Cycloplegia is met with in syphilis and diphtheria; it is due to a palsy of the third nerve. Associative Paralysis: (i) Paralysis of Conjugate Movement. — It consists of abolition of a movement common to both eyes, for example, paralysis of internal rectus of one eye and of external rectus of the other. This form is not produced by a paralysis of the nerves themselves, but by a lesion of the sixth nucleus which besides his numerous other connections it is associated through the posterior longitudinal bundle with that part of the third nucleus which supplies the internal rectus muscle of the op- posite eye. (2) Paralysis of convergence of internal recti of both eyes is probably due to a lesion of the nuclei of the third nerve. It is usually associated with paralysis of accommodation. Ocular palsies are frequent in tabes. Their onset is sudden and characterized frequently by a paralysis of the third nerve and their predilection for the pupil (Argyll-Robertson). Course, Termination, Prognosis. — They depend upon the cause. The course is variable, it may disappear and recur. In an advanced stage of tabes it may remain permanently. In syphilis ocular palsies are slow in onset, but progressive. They also affect chiefly the third nerve. Like in tabes they are peripheral in origin. They may be promptly ameliorated by salvarsan. mercury and iodides. In multiple neuritis ocular palsies disappear when the other symptoms improve. Ocular palsies following infectious diseases usually recover. In tumors, hemorrhages, fractures, the ocular palsies are incurable. Nuclear palsies do not recover. Etiology. — Diseases of the brain may produce a conjugate deviation of the eyes, also a paralysis of the levator palpebral (blepharoptosis). Tumors or other lesions at the base of the brain frequently produce ocular palsies in addition to crossed hemiplegia. In Polioencephalitis superior (see this chapter) nuclear ophthalmoplegia is a frequent accompaniment. In tabes and in paresis the third nerve is frequently involved. The same is observed occasionally in multiple sclerosis and in syringomyelia. In polyneuritis (see this chapter) ophthalmoplegia is observed. Various DISEASES OF CRANIAL NERVES 403 infectious diseases, intoxications may be the cause of palsy of the ocular muscles. Syphilis plays a prominent role in the causation of this paralysis. Traumatism of the orbital region, compression, fractures at the base of the brain, aneurisms of the arteries at the base, thrombosis of the cavernous sinus, tubercular meningitis or gummata at the base will all involve the motor ocular nerves. Sinusitis, frontal and ethmoidal, may be the cause of ocular palsies either directly or through an inflammatory condition of the orbit. Finally ocular palsies may be congenital. Treatment. — Removal of the cause is the first indication. In syphilis mercury and iodides are necessary. In toxic or infectious cases blood- letting and sweating may be of benefit. Hypodermic injections of strych- nia yield sometimes good results. Electrical stimulation of the palsied muscles may be useful. Diplopia can be corrected either by special glasses or surgical means. The tendency to remissions is great. Nystagmus.— It is an associated oscillation of the eyes which is accomplished symmetrically and more or less rapidly. Most frequently the nystagmus is horizontal, sometimes it is vertical. It may be also rotatory. In the majority of cases nystagmus is a convulsive phenomenon occurring mostly upon voluntary movement of the eyes. It is due to an irritative lesion of the center which controls associative movements. In exceptional cases it is unilateral. Nystagmus may be congenital or hereditary. It may be present without any eye lesion or else it may accompany cataract, albinism, coloboma, strabismus, errors of refraction. Myoclonic nystagmus described by Lenoble and Aubineau is a con- genital condition consisting of nystagmus accompanied by facial asym- metry and tremor of the muscles of the face and increased reflexes. Nystagmus is most frequently acquired. It may be the result of impaired vision and when the latter improves, the former disappears. Occasionally it is observed in normal individuals during fixation of the eyes on an object. It is met with in the following diseases of the central nervous system : Multiple sclerosis, Friedreich's disease, heredo-cerebellar ataxia, syringomyelia, very frequently in cerebellar diseases, in lesions of the quadrigeminal bodies. In diseases of the medulla when the lesion extends to the pons nystagmus is observed. Here the posterior longi- tudinal bundle or the centers of association are involved. Thus it is met with in Pseudo-bulbar palsies and in tumors or focal lesions of the pons. In diseases of the brain nystagmus may occur. It may depend upon the distant effect of the cerebral lesion on the posterior cerebral fossa. It may depend also on a lesion of the cortical areas which control the movements of the eyes, viz: frontal lobe, angular gyrus, visual area. 404 DISEASES OF CRANIAL NERVES Vestibular Nystagmus.- — The involvement of the labyrinth causes nystagmus. The reflex is transmitted to the oculo-motor nerve from the vestibular apparatus through the posterior longitudinal bundle and cerebellar peduncles. The nystagmus is due to a displacement of the endo-lymph fluid in the semicircular canal. Horizontal nystagmus is caused by excitation of the horizontal canal, vertical by excitation of the superior vertical canal, rotatory by excitation of the posterior vertical canal. Barany 's test consists of rotation, caloric and galvanic excitation. Inrotation (patient is seated on a turning chair, head erect) the nystagmus is in a direction of the rotatory movement, but as soon as the rotation is interrupted, the nystagmus is the opposite direction. Nystagmus of thermic origin pro- duced by irrigation of the ear is in a direction opposite to the irritated ear if the water is cold and vice versa if warm water is used. In galvanic test if the cathode is applied to the mastoid, rotatory nystagmus is in the direc- tion to the same side. If the anode is applied, the nystagmus is to the opposite side. When the labyrinth is destroyed, the above tests, especially the caloric test, will not be able to produce the least nystagmoid movement of the eye globes. Absence of reflex therefore is an indication of disease of the labyrinth. Barany has observed that 60 per cent, of normal individuals present a slight nystagmus on looking to the extreme right or left. In labyrinthine disease on one side the nystagmus is very evident when the eyes are turned to one or the other side, while normally it is present on looking to either side. Besides, pathological nystagmus is generally well developed, while normally it is very imperfect. Continuous nystagmus associated with vertigo is present in purulent disease of the labyrinth. In cerebellar abscess there is also nystagmus associated with vertigo, but here the nystagmus is directed to the side of the lesion, while in labyrinthine diseases to the sound side. Vestibular nystagmus differs from nystagmus of multiple sclerosis or of other ocular disorders in that the former consists of unequal oscilla- tions, one movement being less rapid than the other and the oscillations become increased when the eyes are turned toward the shorter movement, diminished if the eyes are turned toward the slower movement. Miners' nystagmus is an occupation disorder occurring in individuals working in coal mines. It is of the rotatory form. There is a benign variety which does not interfere with work and a grave variety which is much less frequent. The nystagmus occurs in the benign cases only when the eyes are directed upward. In the severe cases it will occur when the eyes are directed downward. The individual sees objects moving continuously DISEASES OF CRANIAL NERVES 405 before him (dancing) which of course prevents him from working. The cause if this nystagmus lies in forced elevation and oblique direction of the eyes and poor light in the mines. Perhaps the displacement of labyrinthine endo-lymph caused by forced elevation of the head is the cause of the nystagmus. Hippus.— It consists of alternating dilatation and contraction of the pupils. It sometimes accompanies nystagmus. It is probably due to a dynamic disturbance of equilibrium in the movements of the iris. D. Paralysis of the Fifth Nerve (Trigeminal). Symptoms. — As the fifth nerve consists of sensory and motor portions the symptoms will be sensory and motor. Sensory.— The area of the skin supplied by the nerve will be anaesthetic, viz. conjunctiva, cornea, cheek, nose, lips, mouth, gums and tongue. The external auditory meatus and the skin over the largest portion of the lower maxilla are not involved. The corneal and lid reflexes are abolished. The secretions of the eyes and nose are impaired. Smell and taste are also impaired. Trophic disturbances are frequent. Herpes is common and herpes zoster ophthalmicus is due to neuritis of the first branch of the fifth nerve. Neuroparalytic Keratitis is not infrequent. It is characterized by an inflammation and ulceration of the eye. According to the latest view on this affection the keratitis is not due to a disease of supposed trophic fibers of the fifth nerve, but to an irritation of the trigeminal fibers. Motor. — The masticatoy muscles (masseter, temporal and pterygoids) are paralyzed and in unilateral paralysis the tongue instinctively pushes the food toward the normal side in the act of mastication. The unilateral involvement of the pterygoid muscles produces a deviation of the lower jaw to the paralyzed side when the mouth is opened. The affected muscles undergo atrophy and in this case they show reactions of degeneration. When the paralysis of the fifth nerve is incomplete, as it happens in cases of compression, the sensory and motor symptoms are also incomplete. There will be only diminished sensations (hypaesthesia) instead of anaes- thesia, but the continuous irritation from the compression will produce pain. If the condition continues, complete anaesthesia will finally develop. In diseases of the pons the spinal rcot of the fifth nerve may be involved; there will be facial anaesthesia on the same side and paralysis of the arm and leg on the opposite side. The prognosis depends upon the original cause. Etiology. — The fifth nerve is rarely affected primarily. It usually becomes involved in the course of other diseases. Lesions at the base of the brain (meningitis, syphilis, tumors, hemorrhages, aneurism), otitis 406 DISEASES OF CRANIAL NERVES media, caries of the sphenoid, trauma of the orbits or of the maxillae — are all causes of disease of the fifth nerve. It is rarely involved in polyneuritis. It may also be affected in tabes, in syringomyelia and its motor nucleus in bulbar palsy. Unilateral trigeminal palsy associated with paralysis of the eighth nerve is frequently met with in tumor of the ponto- cerebellar angle (see this chapter). Treatment. — Removal of the cause is the first indication. Specific drugs should be administered when syphilis is suspected. Operations for removal of growths, etc., will be resorted to when medications fail. Pain and atrophic disturbances will be treated with appropriate remedies. The eye should be protected against injury or irritation. E. Paralysis of the Seventh Nerve (Facial) (Bell's Palsy). — Facial palsy may be of cerebral, nuclear and peripheral origin. The latter two are identical in their manifestations. The description that follows will be that of peripheral facial palsy. In discussion on diagnosis the differ- ential symptoms of cerebral facial palsy will be given. Etiology. — Cold is a frequent cause (rheumatic paralysis). Trauma is next in frequency. It may be produced by fracture of the petrous bone, by a blow or operative procedures over the parotid region, and in obstetrical cases by pressure of the nerve against the pelvis or else by the forceps. Lesions in the vicinity of the nerve, as tumor, abscess, exostosis, caries of the petrous bone, otitis media are apt to affect the nerve. Infec- tious diseases and intoxications are occasionally accompanied by facial palsy. In the tertiary period of syphilis it may be encountered. An isolated involvement of the seventh nerve in the course of syphilis is rare. In the Archives of Diagnosis, 1908, 1 published records of six cases in which Bell's palsy was caused by syphilis. In two patients the facial paralysis occurred as early as two months after the initial chancre. It may appear in the course of polyneuritis, alcoholic or lead. It may develop in syphilitic meningitis at the base of the brain, also in the course of tumors of the cerebello-pontile angle. Neuropathic predisposition plays a certain role. Pathology. — The morbid changes consist of a parenchymatous neuritis. The usual seat of the lesion is either in the Fallopian canal or in the stylo- mastoid foramen, or else at its exit from the latter. Symptoms. — The onset of facial palsy is sudden in cases due to cold. It is usually preceded by pain in the neck or back of the ear. It develops slowly when caused by other factors. In Bell's palsy the striking symptom is asymmetry of the face. The latter is deviated to the normal side. The muscles on the entire paralyzed side have lost their normal tonicity and the voluntary motility: they are DISEASES OF CRANIAL NERVES 407 relaxed. The naso-labial fold is only slightly marked and the angle of the mouth is lowered. The wrinkles disappear. The asymmetry becomes more evident when the patient attempts to laugh, to show his teeth. Blowing and whistling are impossible. In the latter acts the air raises the cheek. Liquid food runs out of the mouth on the paralyzed side. The forehead on the affected side is also smooth and when the patient is told to wrinkle it, the muscles on the paralyzed side remain immobile (paralysis of the frontal muscle). The eye remains widely open because of the paralysis of the orbicularis palpebrarum. As closure of the eye is impossible, the conjunctiva and cornea being continuously exposed, become inflamed. Bell called atten- tion to the rotation of the eye globe upward and outward at the attempt to close the eye. The tongue when protruded appears to be deviated. The taste on its anterior two- thirds is sometimes diminished or abolished (involvement of the chorda tympani). The palate is relaxed and the uvula deviated. The sense of taste is perceived by the mucous membranes of the tongue and soft palate including the palatine arches. The anterior two- thirds of the tongue are supplied by the chorda tympani, the posterior third by the ninth nerve, the soft palate by a branch fiom Meckel's ganglion. The facial nerve may be regarded as a mixed nerve. It has a ganglion (gen- iculate). The sensory fibers are: the great superficial petrosal, the small superficial petrosal and the external petrosal supplying the carotid plexus of the sympathetic. The geniculate ganglion sends fibers running with the seventh nerve in the Fallopian canal, also fibers anastomosing with the fifth and eighth nerves. The sen- sory fibers of the seventh nerve are represented in the auditory nerve, the tympanic plexus (supplied by the petrosal nerves), the auricle and the external auditory canal. R. Hunt (/. Nerv. and Ment. Dis., 1907) has brought out a syndrome of the sensory branches of the seventh nerve occuriing in facial palsy. The symptoms are: (1) pain in the ear and mastoid region. The pain may be of neuralgic character. Anaesthesia or hyperesthesia in the region of Fig. 130. 4o8 DISEASES OF CRANIAL NERVES concha and external auditory meatus, also hypaesthesia in the chorda tympani distribution. (2) Herpetic otalgia, herpes oticus. The eruption is distributed over the tympanum, external auditory canal and concha. (This is the herpetic area of the geniculate ganglion.) (3) Reflex facial twitchings and spasms. The hearing is sometimes affected. There may be also hyperacuity of hearing. The electrical contractility of the paralyzed muscles is very important. While in some cases (mild) it is normal, in others it is altered quantita- tively and qualitatively. Reactions of degeneration are present. Among the rare symptoms of peripheral facial palsy may be mentioned absence or diminution of sweating of the skin on the affected side. This is observed in the severe forms. Vaso-motor disturbances are sometimes observed. Course, Termination, Prog- nosis. — Facial palsy due to ob- stetrical causes has a favorable course, it lasts usually from eight to fifteen days. When the nerve is destroyed by a fractured bone, the paralysis is incurable. In cases of facial palsy due to the middle-ear diseases the outcome is serious : the palsy persists. In cases produced by cold (the most frequent variety) the palsy may be mild and complete; recovery follows in from two to three weeks. In other cases in which slight reac- tions of degeneration are present, the disease may last two to three months. In cases in which the faradic contractility is absent for a period of two to three months, the prognosis is uncertain but not always serious, as I have seen such cases improve greatly after persistent electrical stimulations. In grave cases, in which the reactions of degeneration are marked or elec- trical irritability is entirely absent, recovery may be only partial or not at all. In cases with incomplete recovery the paralyzed muscles become permanently contracted. The contractured muscles draw the mouth Fig. 131 — Right Facial Paralysis showing Involve- ment ONLY OF THE UPPER FACIAL BRANCH. DISEASES OF CRANIAL NERVES 409 toward the paralyzed side, the naso-labial fold is deep, the palpebral fissure is small, so that the impression may be formed that the paralysis is on the normal side. It is then sufficient to observe the patient in volun- tary acts (laughing, showing teeth, etc.), to determine which half of the face was originally paralyzed. In facial palsy of nuclear origin the prog- nosis is very serious. Diagnosis. — The deviation of the face, the lowering of the angle of the mouth, the disappearance of wrinkles, the inability to close the eye and to wrinkle the forehead are sufficient symptoms to recognize a peripheral facial palsy. Cerebral Facial Palsy. — A cerebral lesion producing a hemiplegia quite frequently involves also the face. This is due to an involvement of the fibers passing from the cortical center of the face through the internal capsule together with those from the arm and leg centers and joining the nucleus of the facial nerve in the pons on the opposite side (see Anatomy). Therefore a facial palsy of cerebral origin almost always accompanies a hemiplegia. In addition to this the following symptoms are characteristic of cerebral facial paralysis : (a) preservation of the musculus frontalis and orbicularis palpebrarum. This is possibly due to the fact that the upper branch of the facial nerve receives its fibers from a different tract (perhaps from the third nerve at the lowest level of the peduncles or sixth nerve) or else it is innervated by both hemispheres; (b) normal electrical reactions of the affected muscles; (c) absence of atrophy; (d) paralysis of the voluntary movements of the mouth and preservation of emotional movements. To sum up, in cerebral facial paralysis only the lower part of the face is affected, while in peripheral or nuclear palsy the upper and the lower halves of the face are paralyzed. An isolated involvement of the upper branch of the facial nerves has occasionally been reported. The accompanying photograph shows a paral- ysis of the right musculus frontalis and orbicularis palpebrarum without any other central or peripheral symptom (Fig. 131). In this case there was also a dilatation and a sluggish fight reaction of the pupil on the same side. The condition of the pupil points to a physiological connec- tion of the seventh with the third nerve. Indeed Mendel long ago main- tained the idea that the upper branch of the facial nerve takes its origin in the lower part of the oculo-motor nucleus (Neurol. Centralblatt., 1898). He found atrophy of the cells in this nucleus after section of the upper facial nerve in rabbits. Besides, in bulbar palsy and amyotrophic lateral sclerosis there is a comparative escape of the muscles of the upper face, while those of the lower are involved. In spite of this evidence, not all 41 DISEASES OE CRANIAL NERVES are agreed on a separate origin of the upper branch of the facial nerve. The majority of writers believe that it arises from the dorsal part of the facial nucleus. In nuclear palsy there is frequently a simultaneous involvement of the seventh and the sixth nerve in view of the contiguity of the nuclei, also involvement of the limbs. Double Peripheral Facial Palsy (facial diplegia) may simulate bulbar and pseudo-bulbar palsies. In the first there are usually present abnormal phenomena of deglutition, phonation and respiration, in the other there is an apoplectic seizure with cerebral phenomena. In facial diplegia the expression is characteristic. The mouth is open, saliva is continuously running, also tears are on the cheeks. The eyelids and the entire muscu- lature of the face are immobile. The voice is nasal; mastication is diffi- cult. Facial diplegia is due most of the time to a bilateral lesion of the petrous bones or basal meningitis, also to bilateral tumors of the cerebello- pontine angle. In the latter case there is also bilateral deafness. In peripheral facial palsy it is important to determine the seat of the lesion. It can be facilitated by the following special symptoms: (a) A lesion between the stylo-mastoid forearm and the periphery will involve only the facial muscles, (b) A lesion in the Fallopian canal below the geniculate ganglion will involve also the taste on the anterior two-thirds of the tongue (chorda tympani) and the hearing, (c) A lesion at the level of the geniculate ganglion and above it will involve the palate in addition to all the other symptoms except the taste, (d) A lesion at the base of the brain will involve simultaneously the eighth nerve, and deafness will appear at the same time as paralysis of the face. Treatment. — Removal of the cause is the first indication. Tumors, abscesses, otitis media will be treated accordingly. When a syphilitic history is present, iodides and mercury will be given. Cases which are due to cold and which come under observation at or shortly after the onset of the palsy should be treated at first with vesicants or bloodletting back of the ear. Warm applications, sedatives or coal-tar products (salicylates, aspirin, phenacetin) will relieve pain. At the end of ten days after the acute symptoms have subsided, galvanic applications to the paralyzed muscles must be instituted. At first they will be daily, but later every other day ten to fifteen minutes' application is sufficient. At the end of a few weeks faradism can be substituted for the galvanism. Massage is a good adjuvant to electricity. A daily rub for five to ten minutes will be useful. Secondary contractures are not easily remedied, although the treatment should be kept up indefinitely. I have obtained good results in very old cases after a prolonged treatment. The surgical treatment DISEASES OF CRANIAL NERVES 411 consists of anastomosis of the facial nerve with the spinal or with the hypo- glossus. Satisfactory results have been obtained from both procedures. F. Paralysis of the Eighth Nerve (Auditory). Etiology. — Middle ear or labyrinth diseases, trauma, caries and other diseases of the bones at the base, meningitis (especially its cerebro- spinal form), syphilis — may all be accompanied by a neuritis of the eighth nerve. A basal disease rarely involves only the eighth nerve, the facial nerve also suffers. Degeneration and atrophy of the eighth nerve are occasionally observed in tabes, paresis, multiple sclerosis. A prolonged use of quinine may produce degeneration of the acoustic nerve. Symptoms. — As the eighth nerve consists of two portions with differ- ent functions (see Anatomy), the symptoms will differ according to whether the cochlear or the vestibular nerves are involved. (a) Cochlear Nerve Symptoms. — The function of this nerve is hearing. Disturbances of hearing will result from diseases of this nerve. Tinnitus Aurium is one of the symptoms. It is characterized by subjective sounds, as ringing, buzzing, etc. If the condition continues, deafness may be the result. In making a diagnosis it should be borne in mind that tinnitus 'may be caused by any disease of the auditory system or by general diseases. Thus anaemia, hyperemia and aneurism of cerebral vessels; otitis externa or media, cerumen in the external ear — are all apt to produce peculiar sounds. Deafness, partial or complete, may be caused by a lesion of the cochlear nerve as well as by diseases of the ear itself. The differential diagnosis is based upon the following facts. If deafness is due to a disease of the middle ear, the tuning fork is not heard when kept near the ear, but heard when applied to the temporal bone. In a disease of the cochlear nerve the fork is not heard when applied to the bone. In diseases of the middle ear hearing is increased in a noise but not increased in diseases of the cochlear nerve. Congenital or hereditary deafness is probably due to a primary atrophy of the eighth nerve. (b) Vestibular Nerve Symptoms. — Anatomically the nerve originates in the semilunar canals and labyrinth. Its function is to maintain equilibrium. The reason of it lies mainly in the connections existing between the nuclei of this nerve and the cerebellum, which is the chief organ of equilibrium. An affection of the nerve, or of its nuclei, produces vertigo. The nuclei connecting the vestibular nerve with the cerebellum (middle and lateral lobes) are: vestibular, Deiter's, and dorsal auditory (see Anatomy). It should not be forgotten that vertigo may result from other causes 412 DISEASES OF CRANIAL NERVES besides a lesion of the vestibular. Thus diseases of the ear, of the ocular apparatus, of the brain, of the viscera, may be attended by vertigo. It may also be present in hysteria and neurasthenia. A detailed discus- sion on the differential signs in those various affections will be properly given in the chapter "Vertigo." The reader is also referred to text- books on ear diseases for a detailed study of tinnitus aurium and deafness. Prognosis. — It is unfavorable, as the underlying cause is a degenera- tion and atrophy of the auditory nerve. Treatment. — Electricity, and especially the galvanic current, may sometimes relieve the tinnitus aurium (anode to the diseased ear), but not much reliance can be placed on it or on medications. Pilocarpin, quinine may sometimes yield some results. Counter-irritation over the temporal bone (petrous bone) and strychnia may be of some use. In syphilis iodides and mercury give good results. G. Paralysis of the Ninth Nerve (Glossopharyngeal) . Etiology. — Tumors, gummata, aneurismus, injuries, thrombosis of the jugular veins — are the causes in the diseases of the ninth nerve. An isolated paralysis of this nerve is rare. Symptoms. — Some of the motor and sensory fibers of the ninth nerve are intimately connected with those of the tenth nerve so that it is difficult to say that the functions of the first depend upon one or the other nerve. However, it is admitted that when the ninth nerve is in- volved, the following symptoms are observed: Anaesthesia of the pharynx, palate and middle ear, ageusia (loss of taste) of the posterior third of the tongue and gums, difficulty of swallowing, of phonation and respira- tion (paralysis of some muscles of larynx, pharynx and oesophagus). H. Paralysis of the Tenth Nerve (Pneumogastric) . Etiology. — In alcoholic and diphtheritic polyneuritis the tenth nerve is frequently involved. Various diseases at the base of the brain or skull (meningitis, tumors, hemorrhages, etc.), suppuration or tumors of the neck and mediastinum, injuries on the neck, pericarditis, ligation of the carotids, are all apt to injure the vagus. Finally a neuritis of rheumatic nature not infrequently involves the recurrent laryngeal nerves (see Anatomy) . Symptoms.— Paralysis of the larynx, fauces and palate are the most prominent symptoms. Phonation is disturbed. Speech is nasal. Swal- lowing is only slightly disturbed. In unilateral paralysis there is acceleration of the heart-beat, also irregularity of respiration. The most important symptoms are those concerning the larynx. This organ is supplied by two branches of the tenth nerve, viz. superior DISEASES OF CRANIAL NERVES 413 laryngeal and recurrent laryngeal. The first contains sensory fibers for the mucous membrane of the upper portions of the larynx and motor fibers for the epiglottis and cricothyroid muscle. The second supplies all other muscles and the mucous membrane of the larynx below the vocal cords and trachea. The vocal cords are paralyzed and remain immovable during phonation and respiration. In unilateral paralysis the voice is hoarse and a deep inspiration produces stridor, there is no cough. In bilateral paralysis aphonia and dyspnoea are present, stridor only on deep inspiration, and there is no cough. In nuclear involvement (which occurs in bulbar palsies) of the vagus respiratory and laryngeal palsies are manifested by rapid heart action and Cheyne-Stokes' respiration. There is usually an associated paralysis of the muscles of the soft palate and of pharynx. Paralysis of the palate is manifested in disturbed phonation. Prognosis.- — It is always grave. A nuclear palsy of the vagus caused by a hemorrhage or embolism may produce sudden death. Treatment.— In syphilis mercury and iodides may be of benefit. In palsy of the vagus, caused by alcohol, stimulants are urgent: alcohol should be then freely administered. Iodides are useful in lead laryngeal paral- ysis. Electricity applied externally to the larynx may be of benefit. Paralysis of the recurrent laryngeal nerve deserves special mention. The paralysis may be total or partial. In the first case the vocal cord is in an intermediary position and all the muscles supplied by the nerves are paralyzed. In the second case the vocal, cord is immovable in median position, which is due to paralysis of the abductor, viz. posterior crico- arytenoid muscle. The partial paralysis is more frequent than the total, on the left side more than on the right and oftener in men than in women. The affections in which involvement of this nerve is observed are : (a) diseases of the thyroid gland, goiter especially, in which case it is due to com-, pression. Thyroidectomy is not rarely followed by paralysis of the recurrent nerve. In this case it may be due to pulling, pinching with forceps during the operation or else by a cicatrix during the healing process. Most fre- quently the paralysis is partial. If it is unilateral, the sound cord may compensate the impairment of the affected cord, but in bilateral paralysis there are aphonia and respiratory disturbances, (b) Aneurism of the Aorta and Aortitis. — The relation of the left recurrent nerve to the concave portion of the arch of the aorta explains the frequency of the nerve paralysis in aneurism. Not only compression of the aneurism, but also an inflammatory process spreading from the outer wall of the aorta to the nerve is the cause of paralysis of the latter. The right recurrent nerve is very rarely affected. More rarely is observed a bilateral in- 414 DISEASES OF CRANIAL NERVES volvement. (c) Cancer of the oesophagus causes the disease through compression of the peritracheo-laryngeal glands which are frequently infiltrated in cancer of oesophagus, (d) In cancer of the lungs and (e) in pulmonary tuberculosis, the infiltrated pretracheobronchial glands being in contact with the recurrent laryngeal nerve, produce its paraly- sis, (f) Mitral stenosis has occasionally been the cause of paralysis of the left recurrent nerve, (g) In Tabes paralysis of this nerve has been ob- served and it is probably due to a peripheral neuritis (Dejerine and Petren) . (h) Toxic and infectious causes: lead, arsenic, typhoid fever, diphtheria. (i) Finally there may be a primary neuritis the cause of which cannot be ascertained. I. Paralysis of the Eleventh Nerve (Spinal Accessory). Etiology. — Colds, trauma of the neck, diseases of the cervical vertebras, abscesses of the neck, tumors in the same vicinity, cervical myelitis — are all apt to compress, irritate or destroy the eleventh nerve. Finally a primary neuritis is also possible. Symptoms. — The inner branch of the eleventh nerve is in connection with the tenth nerve and controls phonation movements of the larynx. We will therefore be concerned here exclusively with the external branch. The latter innervates the muscles sterno-cleido-mastoid and trapezius. In unilateral paralysis of the first muscle that of the opposite side will bend the head towards it and the face will turn towards the paralyzed muscle. The deviation will be only slight, as the sterno-cleido-mastoid muscle is, besides the eleventh, also supplied by second and third cervical nerves. When the paralysis is bilateral, the head cannot be held straight, but falls backward. When the trapezius is involved, a marked deformity ensues. The function of this muscle is to turn the head backward and elevate the shoulder. In case of paralysis, the serratus not being counteracted rotates the scapula so as to project its inner angle upward, and when the arm is brought forward, the scapula is no more held against the thorax, but projects. In bilateral paralysis shrugging of the shoulder is impaired. In advanced cases atrophy, reactions of degeneration, also contrac- tures develop. The deformity is then pronounced. Trapezius and sternomastoid muscles may be paralyzed in the course of progressive muscular atrophy, in syringomyelia, in tabes, when the cells of the an- terior cornua of the upper portion of the cord become involved. Prognosis depends upon the cause. Syphilis has the most favorable outlook. Treatment consists of electricity and massage. In cases of tumors an operation is indicated. Old cases with contractures will be treated DISEASES OF SPINAL NERVES 415 by tenotomy or orthopedic appliances. In syphilitic cases mercury and iodides should be given. J. Paralysis of the Twelfth Nerve (Hypoglossus). Etiology.- — Diseases at the base of the skull (tumors, hemorrhages, caries), aneurism of vertebral arteries, dislocation of upper cervical vertebrae, direct injury — are all causes of paralysis. Symptoms.— In unilateral paralysis the tongue is deviated towards the side of the paralysis. This can be explained by the action of the genio-hyo-glossus of the normal side. The affected half of the tongue is atrophied, flabby and wrinkled. Fibrillary contractions are marked. Reactions of degeneration are present. Hemiatrophy of the tongue does not interfere to a great extent with mastication and deglutition. These two acts are decidedly impaired in total paralysis .of the tongue: the latter lies motionless in the mouth. Articulation of words is indistinct in unilateral paralysis, but marked in bilateral. In nuclear disease paralysis of the tongue is associated with paralysis of the lips. Prognosis. — It depends upon the cause. Recovery has been observed in syphilitic cases. Treatment. — The original cause must be removed whenever it is possible. Electricity may be of some use. Mercury and iodides should be given, when syphilis is suspected. II. Paralysis of Spinal Nerves A. Upper Cervical Nerves The third and fourth cervical roots give off filaments to form a nerve the function of which is of extreme importance. This is the phrenic nerve. Paralysis of the Phrenic Nerve Etiology. — Cervical or mediastinal tumors, infectious diseases (diph- theria, etc.), intoxications, toxic polyneuritis, (alcohol, lead, carbon monox- ide), Potts' disease, dislocations or fractures of the vertebras, finally diseases of' the upper cervical cord (hemorrhages, tumors, myelitis, poliomyelitis, pachymeningitis, syphilitic meningitis) — are all causes of phrenic nerve palsy. Occasionally the phrenic nerve is involved in tabes. Finally in pleurisy and peritonitis the branches of the nerve distributed in the diaphragm may become affected. Symptoms, — As the function of this nerve is mainly to innervate the diaphragm, a unilateral paralysis of it will produce disturbances of 416 DISEASES OF SPINAL NERVES respiration. Dyspnoea is the chief symptom. It is marked upon the least effort. The acts of coughing, expectoration, defecation and even talking increase the respiratory disorder. In severe cases, when the paraly- sis is bilateral, the dyspnoea is pronounced even when the patient is at rest. The patient is then threatened with asphyxia. On inspection the diaphragmatic phenomenon of Litten is noticeable. It consists of an epigastric depression during the act of inspiration and of a protrusion in the act of expiration. Pressure over the scalene muscles or between the lower insertions of sterno-mastoid muscle produces pain. Course, Prognosis. — The disease is serious and the danger lies in asphyxia or when bronchitis or pneumonia develop. In the latter case the respiratory trouble is increased. The prognosis is graver when the paralysis depends upon a cord lesion than upon neuritis. It is also very grave in diphtheritic and quite serious in alcoholic neuritis. In making a diagnosis it should be borne in mind that hysteria sometimes simulates phrenic paralysis. The special stigmata, the sudden onset of the palsy after an emotion will decide the diagnosis of the hysteria. Treatment. — Removal of the cause as promptly as possible is the main indication. CouDter-irritation applied to the space between the bellies of the sterno-mastoid muscle may be of benefit. Strychnia and electricity are advisable. Asphyxia can be relieved by inhalation of oxygen. Anti- syphilitic treatment should be instituted when specific disease is suspected. B. Paralysis of the Lower Cervical Nerves Paralysis of the Brachial Plexus The last four cervical roots and the first dorsal constitute the brachial plexus. A lesion of the roots cannot be differentiated from that of the plexus itself. In the description that follows we will be concerned with various palsies of the upper limb caused by a lesion extending from the point of emergency of the roots from the cord till the point where the individual nerves leave the plexus. Etiology.— Injury of the shoulder, dislocation or fracture of the head of the humerus and of the clavicle, operative procedures in the same region, obstetrical manceuvers, forceps, forced reduction of dislocated humerus, forced extension or abduction of the arm, heavy weights on the shoulders (stone or hod carriers)- — are all traumatic causes. Tumors or abscesses in the vicinity of the plexus, diseases of the bony tissue (caries, exostosis of the vertebrae or clavicle), localized meningitis, finally a neuritis of rheu- matic, toxic and infectious nature are the non-traumatic causes. Cervical rib may be also the cause of the inferior type of brachial palsy (see below). DISEASES OF SPINAL NERVES 417 Symptoms. — The paralysis of the upper extremity is total when the lesion affects the entire plexus; partial, when only a portion of the plexus is involved. Total Paralysis. — It is rare. It is usually preceded by a numbness or pain of neuralgic character. The entire upper limb is paralyzed. Abduction, adduction, flexion, extension, movements of the hands are all abolished. The electrical reactions are at first only diminished, but later show RD. Atrophy develops rapidly and appears first in the upper portions of the limb. The skin is cold and its secretions are dimin- ished. Sensations are abolished. There is complete anaesthesia to touch, pain and temperature over the entire arm except on the internal aspect of the arm which is also innervated by the second and third dorsal nerves. As the first dorsal nerve enters into the formation of the brachial plexus, in addition to the above symptoms there will be also oculo- pupillary manifestations, viz. myosis, narrowness of the palpebral fissure. The course and prognosis depend upon the intensity of the involve- ment. While recovery is possible, however in the majority of cases partial paralysis and atrophy remain. Retraction of the tendons results in deformities of the arm or hand. Most frequently the paralysis is at first total, then gradually becomes limited to one or two nerves, chiefly musculo-spiral and circumflex. Partial Paralysis. — It presents two main types : Superior and inferior. (a) Superior Type (Erb's paralysis). — The following muscles of the shoulder and arm are involved: deltoid, biceps, brachialis anticus, supi- nator longus, supinator brevis, supra- and infraspinatus and the clavicular end of the pectoralis magnus. These ^muscles are controlled by that part of the brachial plexus which takes its fibers from the fifth and sixth cervical roots. The disturbed function consists of an inability to flex, supinate and abduct the arm. The latter is in a state of extension. The forearm and hand are pronated. Atrophy with partial or complete RD develop rapidly. Sensations as a rule are not disturbed, but there may be anaes- thesia in the areas of distribution of the circumflex and musculo-cutane- ous nerves. The symptoms just enumerated may present variations as to their intensity. Finally, the paralysis may be bilateral. The upper type of brachial palsy is particularly frequent at birth. It is the so-called Birth or Obstetrical Palsy (Duchenne). It is almost always due to instrumental delivery (forceps) or difficult labor. The finger or tenaculum introduced to facilitate the delivery, also the forceps 27 4i8 DISEASES OF SPINAL NERVES may press directly on the shoulder and the brachial plexus. In breech presentation it is very frequent. The symptoms, course and prognosis are as described above with this difference, however, that the prognosis is more favorable. Complete recovery is not infrequent. (&) Inferior Type (Klumpke) . — Anatomically it is due to a lesion of the seventh, eighth cervical root and the first dorsal. It is characterized by paralysis of the flexors of the hand, thenar, hypothenar and interossei muscles. The hand is claw-like. Atrophy sets in early. Sensory disturbances consist of anaesthesia on the inner half of the forearm and hand (ulnar nerve) ; sometimes it extends to the area of distribution of the median nerve. Pain is not infrequently present. As the first dorsal nerve is involved, oculo- pupillary symptoms are present. They are: myosis, sluggish contraction of the pupil, re- traction of the eyeballs and narrowness of the palpebral fissure. (c) Complex Type. — The two types of partial paralysis of the brachial plexus do not always present themselves as accurately local- ized as described above. Sometimes the lesion extends to neighboring roots. In other cases there may be a combination of both types, but each or only one of them is par- tially involved. In such cases the sympto- matology is naturally complex. Treatment. — It is that of neuritis in general (see this chapter). Re- moval of the cause is the main indication. Pain is relieved by appro- priate means. Electricity and massage constitute the most important part of the treatment. Paralysis of Individual Nerves of the Brachial Plexus. (a) Long Thoracic Nerve (from fifth and sixth cervical roots). — It supplies the serratus muscle. Etiology. — Injury or prolonged pressure on the neck (in carrying heavy weights on the shoulder), gunshot wounds of the neck, continuous elevation of the arm (as in plastering ceilings) , infectious diseases (diph- theria, typhoid fever, grippe), finally exposure to cold — are the causes. Symptoms. — Pain may or may not precede the onset of paralysis of the serratus muscle. The function of the latter is to rotate the scapula when the arm is put forwards, also to hold the scapula against the thorax. Fig. 132. — Showing Position or the Arm in Birth Palsy. DISEASES OF SPINAL NERVES 419 In case of paralysis of the muscle the arm cannot be raised above a horizontal position and in the attempt to put the arm forwards the inner border of the scapula becomes separated from the thorax (wing-like). The Course and Prognosis depend upon the cause. The disease lasts many months. Traumatic cases are the most unpromising. Rest of the arm (sling) is advisable. Massage and electricity should be instituted as early as possible. (b) Supra-Scapular Nerve (from fifth, sixth, also fourth roots). — It supplies the supra- and infraspinatus muscles. Fig. 133. Fig. 134. Figs. 133, 134. — Paralysis of Serratus Magnus Causing Displacement of Scapula when the Arm is Held Forward. (From Nour. Iconogr. de la Salpetriere.) Etiology — Injury to the shoulder, dislocation of the humerus, pressure upon the shoulder are the causes of palsy of this nerve. Symptoms. — Pain may be present at the onset. The function of the infraspinatus muscle is to rotate the humerus outward. Its paralysis abol- ishes this function, and this is seen in the act of writing and sewing. The patient thus affected is unable to move the pen on the paper. Paralysis of the supraspinatus interferes with the act of raising the arm, also permits the head of the humerus to lose its firm position in the glenoid cavity. The muscles eventually atrophy. Pain is frequent and anaesthesia over the scapula is present. Treatment. — Same as above. (c) Circumflex Nerve (from fifth and sixth cervical roots). — It supplies the deltoid and teres minor muscles; also the skin over the deltoid and the articulation. 420 DISEASES OF SPINAL NERVES Etiology. — Injury, dislocation and contusion of the shoulder, compres- sion (crutch), lead poisoning, grippe, diabetes, finally exposure to cold are the usual causes of palsy. Symptoms. — The function of the deltoid muscle is to elevate the arm. Its paralysis abolishes almost all power of raising the arm. The atrophy of the muscle changes the shape of the shoulder. Anaesthesia is present over the skin covering the muscle. Adhesions may form in the joint and produce ankylosis. Reactions of degeneration appear quite early, except in cases of contusion. Fig. 135. — Paralysis or Serratus Magnus. Position of Scapula when Arm is Abducted. Further Elevation of Arm is Impossible. (Starr.) (d) Musculo-Cutaneous Nerve (from fifth, sixth, also seventh roots). — It supplies the biceps and brachialis anticus, also the skin over the radial side of the forearm. Etiology.- — Trauma, pressure, dislocation or fracture of the head of the humerus are the usual causes of palsy. Symptoms. — Paralysis of the flexors of the forearm is the chief symp- tom. It is particulary marked when the arm is supinated. Atrophy and RD. develop rapidly. There is also anaesthesia on the outer side of the forearm. (e) Musculo-Spiral Nerve (from sixth, seventh and eighth roots). — It supplies the triceps, supinators and extensors of the hands and fingers; the skin of the lower half of the arm, of the back of the forearm, also of the dorsum of the thumb and of the first three fingers. Etiology. — The superficial position of the nerve renders it very sus- ceptible to injuries. Trauma is therefore the most frequent of all causes DISEASES OF SPINAL NERVES 421 of musculo- spiral paralysis. Compression, acute or chronic, is a common occurrence It develops during a profound sleep, especially in an intoxi- cated state, when the head rests on the arm a number of hours (Saturday night paralysis). It also occurs during a deep narcosis when the arm is held against a hard support. The superficial position of the nerve in the axilla is the cause of its palsy when the patient walks on crutches (crutch palsy). Violent muscular exercise, fracture of the humerus may also produce a musculo-spiral palsy. Infectious diseases, intoxications (alcohol, lead, arsenic) are predisposing causes. Potain mentions hypo- dermic injections made close to the nerve as an occasional cause of its paralysis. Fig. 136. — Position of the Hand in Musculo-Spiral Palsy. (Oppenheim.) Symptoms. — The onset is usually sudden It may be preceded by some paraesthetic disturbances (tingling, etc.). As the main function of the nerve is extension, its paralysis will place the hand in a special position. The latter is flexed, adducted and semi-pronated; the fingers are equally flexed and the thumb is adducted. There is a gradation of paralysis of extension in the fingers, most pronounced in the fourth finger and least marked in the first (Gowers). Extension of the wrist is impossible (wrist-drop). The forearm is also flexed and pronated. The state of sensations depends upon the seat of the damage dene to the nerve. In the majority of cases the injury occurs near the musculo- spiral groove; the sensibility is here only exceptionally disturbed. When the nerve is damaged above that level, anaesthesia will be present over the area supplied by the sensory filaments of the nerve (see above). The paralyzed muscles gradually undergo atrophy and changes in elec- trical irritability. The latter is at first diminished, but later presents reac- tions of degeneration. Course, Termination, Prognosis. — They depend to a large extent upon the cause. Crutch palsy improves rapidly when the crutches are 42 2 DISEASES OF SPINAL NERVES abandoned. The usual cases of pressure palsy improve and recover. When the nerve is severely injured by fracture, dislocation or contusion, recoven* may also fellow, but slowly. The prognosis is almost in direct relationship to the electrical condition of the muscles. Diminution of response has a favorable outlook, RD. — is unfavorable. When the nerve is completely severed, restoration of function will be possible only after a reunion of the stumps. Treatment. — Removal of the cause in cases of compression is the first indication. As alcoholic individuals are particularly apt to suffer, they should be insisted upon abstaining from alcohol. When the nerve is completely severed, suturing of the two ends is necessary. Electricity and massage are the usual procedures for restoring the function of the paralyzed muscles. They should be instituted as early as possible. Mechanical apparatus have been recommended for the relief of flexion of the wrist and fingers; their object is to place the latter in extension. (J) Ulnar Nerve (from eighth cervical and first dorsal roots). — It supplies the flexor carpi ulnaris, the inner portion of the flexor digitorum profundus, the interossei of the hand, the muscles of the little finger, the palmaris brevis, the last two lumbricales, hypothenar, the adductor pollicis. It gives off sensory fibers to the integument of the hypothenar to the front of the fifth finger and half of the fourth finger, to the back of the fifth, fourth and half of the third finger. Etiology. — The superficial position of the nerve at the elbow and wrist predisposes it to injury. Pressure more or less prolonged, laceration of the nerve, fracture and dislocation at the level of the elbow, diseases of the olecranon or internal condyle of the humerus, forced flexion, occupations requiring a prolonged flexion, especially in alcoholic or cachectic individuals are all causes of paralysis of the ulnar nerve. Overextension of the arm during surgical operations is apt to produce palsy of the ulnar nerve. It is probably due to stretching of the nerve in the axilla. In sleep a similar phenomenon may be observed when the individual sleeps with arms ele- vated, abducted and behind the head. Such a case I placed on record {Old Dominion Journal of Med. and Surg., 1909). Symptoms. — Paralysis of the above enumerated muscles will be present. The most characteristic disturbance is the paralysis of the interossei. The normal function of the latter consists of flexion of the first and extension of the last two phalanges, also adduction and abduction of the fingers. When paralysis occurs, the fingers show an exaggerated extension of the first and flexion of the last two phalanges. This is the claw-like hand. The condition, however, is only partial; more pronounced for the fourth and fifth fingers than for the index and middle fingers, because the latter DISEASES OF SPINAL NERVES 423 have their lumbricales (median nerve) preserved. Adduction of the thumb, abduction and adduction of the fingers, lateral movements of the little finger are impossible. The hand is slightly deviated toward the radial side. Sensory Disturbances are habitually present. Besides pain and hy- peresthesia there may be hypsesthesia or anaesthesia. The latter will be present over the internal surface of the hand (palm and dorsum) , little finger Fig. 137. Fig. 138. Figs. 137, 138. — Showing Sensory Loss and Abnormal Position in Injuries of Ulnar Nerve. (Bowlby.) inner half of the fourth finger, internal and dorsal surface of the first phalanx of the third finger. Atrophy and reactions of degeneration develop some time after the onset of the palsy. Course, Prognosis. — In slight traumata the termination is favorable after a few weeks' duration. In lacerations or severance the prognosis is unfavorable as to recovery of function. Treatment. — Same as in musculo-spiral nerve palsy. R. Hunt described in 1908 an occupation neuritis due to an involve- ment of the deep palmar branch of the ulnar nerve. This nerve is purely motor and innervates the following intrinsic muscles of the hand: those forming the hypothenar eminence, also some of the thenar region, viz. abductor pollicis and inner head of the flexor brevis pollicis. It innervates as well the palmar and dorsal interossei and the two inner lumbricales. The symptoms are: atrophic paralysis of all the intrinsic muscles of the 424 DISEASES OE SPINAL NERVES hand innervated by the ulnar nerve; reactions of degeneration; total absence of objective sensory disturbances in the ulnar nerve distribution. The lesion, which is usually due to compression, is below the point where the sensory branch is given off and before the deep branch breaks up into muscular branches. Hunt observed the neuritis in a jeweler, machinist, brass polisher. H. Gessler in 1896 (Mediz. Corresp.-blatt., Wurtemberg, Bd. LXVI) de- scribed a type of muscular atrophy of the hand in gold polishers, which resembles in many respects that of Hunt with this difference that there was paresthesia in the distribution of the ulnar nerve. (g) Median Nerve (from sixth, seventh, eighth cervical and first thoracic). — It supplies the superficial anterior (except the flexor carpi Fig. 139. Fig. 140. Figs. 139, 140. — Showing Areas of Sensory Losses in Injuries of Median Nerve. (Bowlby.) Horizontal lines, total anaesthesia; vertical lines, partial anaesthesia. ulnaris) and the deep muscles of the forearm (except the inner half of the flexor digitorum communis). In the hand it supplies the opponens pollicis, the flexors of the thumb, the abductor brevis and the two outer lumbricales. It also gives off sensory branches to the radial part of the palm, inner part of the thenar and the palmar aspect of the first three fingers and the radial side of the fourth finger, also the dorsum of the last two phalanges of the thumb and first three fingers. Etiology. — Trauma (blows, cuts, fracture) in the lower part of the fore- arm, compression (tumor, callus, Esmarch's bandage, etc.), violent muscular DISEASES OF SPINAL NERVES 425 efforts are the most frequent causes. Certain occupations (tinners, joiners cigarmakers, milkers) may sometimes involve the muscles innervated by the median nerve. Finally infectious diseases may occasionally cause a neuritis of the median nerve. Symptoms. — Paralysis of the muscles enumerated above will give the hand a peculiar attitude. The position of the fingers is quite char- acteristic. The last two phalanges are held in forced extension by the interossei (ulnar nerve) more on the radial than on the ulnar side; the thumb is extended, approached to the index and its opposition is impossi- ble. The wrist is in extension and slightly abducted. Pronation of the hand is impossible. Sensory Disturbances are usually present. They may consist of pain, hyperesthesia and especially anaesthesia. The latter extends over the two outer thirds of the palm, the palmar surface of the first three fingers and radial side of the fourth finger, the dorsal aspect of the last two phalanges of the thumbs, second and third fingers and radial side of the fourth finger. Atrophy with reactions of degenerations appear early. Trophic and vasomotor disturbances of the skin are quite frequent (herpes, glossy skin, loss of nails, hyperhidrosis, cyanosis). Course, Prognosis and Treatment are identical to those of the ulnar nerve. C. Paralysis of Lumbo-sacral Nerves Paralysis of the Nerves of the Lower Extremities (a) Anterior Crural Nerve (from second, third and fourth lumbar roots). — It supplies the ileo-psoas, extensor cruris quadriceps, sartorius and partly the pectineus and the middle adductor muscles. Its cutaneous branches are distributed over the antero-internal surface of the thigh (lower two-thirds) and internal aspects of the leg and foot. Etiology. — Palsy of the crural nerve is rare. Diseases of the vertebras, pelvis and femur, dislocation of the hip-joint, psoas abscess, hernia, aneurism, trauma in the groin or thigh are the main causes. It may become paralyzed in infectious diseases (typhoid fever, diphtheria) also in alcoholism. Finally, in anterior poliomyelitis the muscles supplied by the anterior crural nerve are especially involved. Symptoms. — The distribution of the nerve indicates that its paralysis affects especially the extensors of the knee and flexors of the thigh. Paraly- sis of these muscles will cause a loss of the knee-jerk. Sensory Disturbances consist of hypaesthesia or anaesthesia over the 426 DISEASES OF SPINAL NERVES area of distribution of the cutaneous fibers (see above). Pain is frequent. Atrophy and reactions of degeneration appear early. (b) Obturator Nerve (from second, third and fourth lumbar roots). — It supplies the gracilis, adductor longus et brevis. Its sensory fibers are distributed over the inner side of the thigh and inner and upper third of the leg. Etiology.- — Obturator hernia, compression by tumors or in difficult labor are the usual causes. Symptoms. — Adduction of the thigh and its outward rotation are affected. The patient is unable to cross and approach the thighs, also to turn the foot inward and outward. Loss of sensation will be present over the area of distribution of the cutaneous filaments. Atrophy and reactions of degeneration develop early. (c) Paralysis of the Gluteal Nerves (from the lumbar and sacral plexus) . — They supply the gluteal muscles, the pyrif ormis and the tensor fasciae lata?. Etiology. — Diseases of sacrum and pelvis, fractures, tumors are the usual causes. The disease is rare. Symptoms. — Paralysis of the above muscles produces loss of rotation, abduction, extension and flexion of the thigh. Station, gait and climbing are difficult. Atrophy develops rapidly. (d) External Cutaneous Nerve (from second and third lumbar nerves). Symptoms. — As it supplies the outer aspect of the thigh, its paralysis causes a difficulty of walking and standing (see alsoMeralgia paraesthetica). (e) Paralysis of the Sciatic Nerve (from Sacral Plexus). Etiology. — Pressure by tumors of the pelvis or femur or fractures, by the head of the foetus in difficult labor, by forceps in instrumental delivery, neu- ritis of toxic (alcohol, lead, arsenic) or infectious nature, septic processes of the pelvis — are all causes of total paralysis of the sciatic nerve. Complete palsy is rare. More frequently are met partial palsies of the sciatic nerve, viz. paralysis of the peroneal nerves. In the latter case infection and intoxications play a prominent etiological role. Puerperal neuritis has a special predilection for the external popliteal nerve. Fracture of the fibula, forcible extension, occupations requiring a stooping position are also causes of peroneal nerve palsy. Symptoms. — In total paralysis of the sciatic nerve (which is exception- ally rare) the foot, the leg, also the flexors of the leg situated on the thigh are all completely powerless. The motility of the entire limb is abolished with the exception of extension of the leg, which is controlled by the crural nerve (see above). The leg is held rigid. The gait is possible to DISEASES OF SPINAL NERVES 427 a certain extent because of the integrity of the extensor muscles (crural nerve). Atrophy and anaesthesia are common. Sometimes trophic disturbances, such as herpes, etc., are present. In paralysis of the external popliteal nerve the following muscles are involved : (a) The peroneal, the function of which is to extend, abduct and rotate (externally) the foot; (b) tibialis anticus whose function is to flex, adduct and rotate (internally) the foot; (c) extensor communis digitorum which extends the first phalanges of the toes; (d) extensor of the great toe which extends its first phalanx. When these muscles are paralyzed, the action of the posterior muscles predominates; dorsal flexion of the foot is impossible. The latter is in a state of foot-drop and of equino-varus. The gait is of steppage character (see Multiple Neuritis) . In an advanced stage contractures place the foot and its constituents in a fixed abnormal position. Sensations are usually diminished. There may also be total anaesthesia. The latter is distrib- uted over the antero-external surface of the leg and the dorsum of the foot and toes. Trophic and vaso-motor disturbances, atrophy of the muscles, changes in the electrical reactions are the same as in any other nerve palsy. Special mention must be made of paralysis of the external popliteal nerve occurring during protracted labor. This nerve originates from the lumbo-sacral trunk which is formed by the union of the smallest part of the fourth and the entire fifth lumbar nerves. The trunk at its formation is situated on the ala of the sacrum under cover of the psoas. It descends into the pelvis and passes over the brim of the true pelvis. It stands to reason that a protracted labor with head pressing unduly against the brim of the pelvis, or else forceps, are apt to injure the exposed lumbo-sacral cord, and therefore, the peroneal nerve. Besides this grave form of peroneal neu- ritis there is also a slight form of neuritis occurring in puerperium and which is usually transient. It is probably tonic in nature. In both forms there is pain on pressure, paretic state of the muscles, spontaneous pain, ob- jective sensory disturbances and sometimes oedema with cyanosis of the limb. The diagnosis should be made between neuritis of the peroneal nerve and puerperal phlebitis. Failure of recognizing the true condition may lead to very grave errors of treatment. When for example a neuritis is taken for phlebitis, it means a strict and prolonged immobilization of the limb. This will lead eventually to muscular atrophy, loss of power in the limb, trophic disturbances, otherwise speaking to a protracted infirmity. When on the other hand a phlebitis is taken for a neuritis, it means 428 RADICULITIS mobilization of the limb, electrization and massage of the muscles. In both cases the respective procedures are dangerous. A very careful ex- amination for characteristic symptoms of each of these affections will en- able one to avoid errors. When the internal popliteal nerve is involved the following muscles muscles are paralyzed: (a) Triceps suras (which normally produces plantar flexion) ; (b) tibialis posticus, which is an extensor, adductor and rotator (internally) of the foot; (c) flexors of the toes, viz. of their first phalanges; (b) interossei (which normally flex the first phalanges and extend the others) . Paralysis of these muscles leads to dorsal extension of the first phalanges of the toes and plantar flexion of the last two phalanges (claw-like), to loss of plantar flexion. Rising on the tips of the toes is impossible and walking is difficult. Sensory Disturbances (hypaesthesia or anaesthesia) are present on the plantar surface of the foot and on the postero-internal surface of the leg. RADICULITIS. Under this term is understood a primary inflammation of the roots of nerves. Secondarily the nerve roots may be involved in lesions of peripheral nerves or of the spinal cord (poliomyelitis, syringomyelia, etc.) and in traumatic injuries. But radiculitis means a primary infection or toxic inflammation of the roots and their coverings. Symptoms. — The onset may be rapid or slow. The first is rare; it may occur in the course of cerebro-spinal or spinal meningitis. The second, which is the usual onset, consists of slowly but progressively developing manifestations. As a type the most frequent form of radiculitis will be described here, viz. cervico-brachial radiculitis of tertiary syphilis. Gradually and without apparent cause various paraesthesias and pain develop in the cervical region. Pain is continuous but is interrupted by paroxysms of a violent character. The latter are brought on by an effort, cough or sneezing. The exacerbations make the patient scream and Dejerine with others described this phenomenon "sign of sneezing" as a reliable symptom of the disease. The pain radiates along the course of the nerve-trunks. Pressure provokes pain. It can be elicited at the level of the sixth transverse process (Erb's point). Gradually the nervous filaments undergo degeneration and destruction and the hyperaesthesia is replaced by anaesthesia ; pain then disappears. Not only the skin but also muscles, bones and joints have lost their sensibility. Perception of position and stereognostic sense are abolished. For this reason ataxia RADICULITIS 429 becomes evident. Whether the loss of sensation is complete or incomplete, it is always radicular in distribution and the latter is characteristic of the disease. When the lesion affects also the motor roots as well as the sensory there will be present also motor and trophic symptoms in addition to sensory. Paresis or paralysis, atrophy of the muscles with reactions of degeneration gradually make their appearance. When atrophy affects only a certain group of muscles, the antagonistic muscles through their overaction will produce deformities of the limbs. The tendon reflexes are at first diminished, but later disappear. Trophic disturbances are not infrequent. Herpes zoster may accompany a radiculitis of the sen- sory roots. A very important diagnostic sign we find in the cerebro-spinal fluid. As the usual etiological factor is syphilis (also tuberculosis), a more or less marked lymphocytosis is found. Similar to neuritis and neuralgia there are also radiculitis and radic- ulalgia. In the latter the lesion consists only of irritation, but not of destruction. The pain is very severe, paroxysmal in type and hyper- esthesia of radicular distribution is present. Chipault and Lefur described a neuralgia of the eighth, ninth, and tenth dorsal roots which manifested itself as a tic douloureux of the abdomen. Neuralgia of the roots may be the initial stage of radiculitis. Forms of Radiculitis Cervico-brachial. — It was described above as a type. If the eighth cervical and first dorsal are involved, oculo-pupillary symptoms will be also observed, viz. myosis, retraction of the eye globe and narrowness of the palpebral fissure. Dorsal. — Vaso-motor disturbances, such as herpes zoster, will be observed. Such was the case of Dejerine and Thomas in which the erup- tion was strictly limited to the area of the right eighth dorsal root. Oppen- heim observed paralysis of the abdominal muscles and loss of their reflexes, meteorism, pain and sensory disturbances in lesions of the eighth and ninth dorsal roots. Lumbar.- — It is quite frequent. It presents the picture of neuritis of the anterior crural nerve (see this chapter). Lumbo-sacral. — Its requisition is very important. It constitutes the so-called "radicular sciatica." The differential diagnosis between it and the classical sciatica is as follows: in the latter the objective sensory disturbances (hyperesthesia or anesthesia) are distributed irregularly. In the former the sensory disturbance will be present in a longitudinal 43 O NEURALGIA form regularly distributed and running parallel with the axis of the limb. Besides, the pain is spontaneous and brought on not exclusively on motion, also it is very persistent and present in regions others than the ischio- sacral notch (see my monograph in /. Amer. Med. Ass'n, 1910). Sacro-coccygeal. — The radiculitis of the cauda-equina is accompanied by disturbances of the function of the sphincter and genitalia. Etiology. — Any cause that is capable to produce an inflammation or degeneration of the roots in their intra- or extra-dural course can be con- sidered as an etiological factor. Tumors, hemorrhages, cold abscess, aneurism — are all apt to bring on a mechanical irritation of the tissue surrounding the root or else to disturb the circulation and nutrition of the latter. Tuberculosis (in Pott's disease) and cancer (of the vertebra?) are almost always the causes of radiculitis. Hypertension of the cerebro- spinal fluid in tumors of brain and in tabes have also been considered as causes. More important etiological factor is an infectious process. Here an inflammation of the meninges occurs first and of the root subsequently. After syphilis, tuberculosis is the most important factor (pachymenin- gitis, leptomeningitis). Toxic (lead) and infectious agents have a special predilection for nerve-roots. Prognosis is quite serious. In syphilitic cases it is most favorable. Treatment. — It depends on the cause. Operations (laminectomy, re- section of posterior roots) are advisable only as a last resort. (For more details consult the thesis of Paris by Camus, 1908.) NEURALGIA (IN GENERAL) Definition and Nature. — Under this term is understood a paroxysmal pain over nerve trunks and their branches. Neuralgia is not a morbid entity, but only a symptom, as it may accompany any affection of the nerves. Pain in the nerve may be also present when no lesion of the latter is evident. A sharp distinction between neuralgia and neuritis cannot be established. The fact is that in a number of cases of recent neuralgia degenerative changes have been found in the nerves. From my personal pathological studies (New York Med. Jour., July 21, 1906) it can be seen that the occurrence of degeneration of the peripheral nerves is frequent in neuralgia, that neuralgia is probably a primary neuritis and that changes in the walls of the blood vessels play a certain role in the causation of the degenerative condition of the nerves. Etiology. — From the foregoing remarks it is evident that the causes of neuritis (see this chapter) will be also those of neuralgia. Besides neuritis, neuralgia may be due to exhaustion caused by de- NEURALGIA 43 1 bilitating and protracted diseases, such as cancer, anaemia, to infectious diseases (grippe, typhoid fever, malaria), to syphilis, to intoxications (lead, arsenic, etc.). Exposure to cold is a frequent factor. Overwork and great excitement, errors of ocular refraction, often provoke neuralgic pains. In the so-called reflex-neuralgias irritation of remote organs (uterus, testicles, etc.) produces a neuralgia of the face or elsewhere. Among the predisposing causes should be mentioned the neuroses (hysteria, neurasthenia), gout, rheumatism, diabetes, tuberculosis. Symptoms.— The chief symptom is pain. Its seat is along the nerve. Generally it is manifested in violent paroxysmal attacks between which the patient is free from pain, but in some cases a slight pain persists in Fig. 141. — Neuralgia of Three Months' Duration. Degenerated Nerve -bundles in the Immediate Vicinity of Thickened Blood Vessel. (Original.) the intervals. The paroxysms may last from a few seconds to several minutes; they may recur several times in one hour. The pain may be tolerable or extremely violent, be confined to one or several spots on the skin or else spread from an initial point to several ramifications of the nerve. It appears spontaneously or brought on by the least touch, motion, cold air, mastication, etc. A characteristic symptom of neuralgia is found in the tender spots. They are very small areas situated over the course of the nerve and its branches; they are extremely tender upon slight pressure. In addition to these two constant symptoms there are others that occur more or less frequently. They are sensory, motor, vaso-motor, trophic and secretory. 43 2 NEURALGIA Sensory. — They may be hyperaesthesia or anaesthesia over the area of distribution of the affected nerve. Motor. — Twitching during the paroxysms (in tic douloureux) or else spasmodic contractions (painful cramps) are sometimes observed. Secretory. — They are ptyalism, hyperhidrosis, watering of the eyes. Vaso -motor. — Pallor or redness of the skin is sometimes observed during the paroxysms; angioneurotic oedema has been seen in association with neuralgic paroxysms. Trophic Disturbances accompanying neuralgia are: oedema and herpes. Neuralgia of long duration may affect the general health. The patient is depressed, irritable, loses his appetite, cannot sleep and finally begins to lose in weight. Course, Duration, Prognosis. — Neuralgia is usually of long duration. The paroxysms may occur at regular or irregular intervals. It may after a certain time disappear completely or else become chronic. In cases of malarial origin the neuralgia presents a special feature: it may substitute a malarial attack or accompany it, viz. occur at certain intervals. Mala- rial neuralgia affects mainly the fifth and the sciatic nerves. In grippe the supra-orbital nerve is most frequently affected. Neuralgia of diabetic origin has a special predilection for the sciatic nerve. The course and prognosis depend upon the cause. Neuralgia caused by neuritis is serious, because it recurs easily and readily becomes chronic. Malarial and syphilitic neuralgias are usually amenable to treatment. The outlook is bad when there is a constitutional unfavorable basis (tuber- culosis, diabetes, etc.). Diagnosis.— Pain along the course of the nerve, its tendency to radia- tion, its remittence or intermittence, finally the tender points are all suf- ficiently characteristic symptoms for making a diagnosis of neuralgia. Sharp pain may occur also in tabes (tabetic crises), but in the latter it does not follow the course of a nerve trunk and the tender points are ab- sent. It is present in tumors located in the vicinity of the spinal roots. In neuritis pain is usually present, but it is continuous, while in neu- ralgia it is paroxysmal. Neuritis will be also recognized by motor, sensory and trophic disturbances, which are absent in neuralgia. Treatment.— (i) Relief from pain and (2) removal of the cause of neuralgia are the chief indications. The pain can be relieved by sedatives. Morphine will give almost immediate relief, but its repeated administration is to be feared in view of the habit which is easily acquired. Coal-tar products (aspirin, sodium salicylate, phenacetin, etc.) without or with small doses of codein has given me satisfactory results. Local counter- NEURALGIA 433 irritation (cantharides) has given me excellent results in a number of cases where a host of internal medications have failed. Malarial and grippal cases need an energetic treatment with quinine. Syphilic cases require mer- cury and iodides or salvarsan. In very obstinate cases, even without a syphilitic history, the specific treatment should be tried. Good results have been obtained by me from Strychnia in gradually increasing doses. In one case of obstinate cervical neuralgia in a middle- aged woman, when all possible medications had been tried during a period of two years and operative procedures had been almost decided upon, strychnia was given as a last trial and curiously enough the pain dis- appeared completely. Thyroid extract has also given me very satisfactory results in four cases when everything else failed. Electricity has given some relief to a very limited number of my patients. Galvanism and sometimes faradism as well as static electricity had been used by me in those cases. Care should be taken to apply weak currents (of 12-15 milliamperes) to the affected nerves, especially in facial neuralgia. The general nutrition, a constitutional diathesis, a neuropathic state, must be taken special care of. Appropriate hygiene and diet, proper and regular elimination (bowels, kidney), hydrotherapy, massage, regular mode of living, avoidance of stimulants (alcoholic beverages), reduction of obesity if it exists, administration of arsenic and iron in cases of anaemia, a special diet in diabetes, exercises for those who lead a sedentary life and on the contrary much rest for those whose occupation is exhausting — -all these points should invariably be taken into consideration in treating a case of neuralgia. Injections of alcohol into the nerve trunks in facial neuralgia at the points of their exit in the bony foramina has proven to be a very useful procedure. Five minims of 80 per cent, of alcohol should be injected without a preliminary local anaesthetic. The injection can be repeated every few days. In the majority of cases the pain disappears immediately after the injection. Burning and swelling of the surrounding tissue ap- pear rapidly and persist for several days, but disappear without leaving any trace. For supraorbital neuralgia injections are made in the supra- orbital notch, which is easily felt. For the second branch, alcohol is injected into the infraorbital foramen. For the third branch of the fifth nerve injections are to be made into the mental foramen of the lower jaw. I have obtained in this manner considerable relief in a number of instances. I have also observed that when several injections into the infraorbital nerve were not successful, subsequent injections into the supraorbital 28 434 NEURALGIA nerve gave relief for weeks. Should the alcoholic injections made at the exit of the sensory nerves on the face fail, deep injections are to be carried out. It must be mentioned immediately that the first branch of the trigeminus should not be treated with deep injections, as the second, third, fourth and sixth cranial nerves, also large blood vessels, are in im- mediate vicinity of the ophthalmic branch and the risk of their injury is very great. Deep injections into the second branch are made with a needle inserted at the lower border of the zygoma; 0.5 cm. behind a perpendicular running from the posterior edge of the orbital process of the malar bone it is diverted upward; at a depth of 5 cm. the needle reaches the nerve as it leaves the foramen rotundum to enter the sphe- nomaxillary fossa (Levy, Baudouin, Patrick). For the third branch of the trigeminus the needle is inserted also at the lower border of the zygoma but 2.5 cm. in front of the anterior root of the zygoma; it is directed backward and upward. At a depth of 4 cm. it reaches the nerve. The directions just given are not absolute, as the anatomical interrelations between foramina and bony structures through which the nerves pass van* considerably in some individuals. Considerable experi- ence is necessary on cadavers before an attempt can be made to perform a deep injection on patients. Superficial and deep injections do not always cure the sufferer, but they may give enormous relief for a very long time. In case alcoholic injections after several attempts fail, a radical operation for removal of the Gasserian ganglion or for cutting off only the sensory roots may be resorted to. The radical operation, unfortunately, does not remove pain in every case, besides being dangerous. For more details on the technic of deep injections of alcohol consult Levy and Baudouin (Presse Medicate, 1906), also Patrick (/. Am. Med. Ass'n., 191 2). While alcoholic injections are an excellent procedure for relieving nerve pain, nevertheless it must be borne in mind that grave damage may be done to the nerve trunk when the latter is placed in intimate contact with alcohol. I have recently shown it in an experimental study (Transac- tion of Amer. Xeurol. Ass'n., 1913). When all the means enumerated above fail to give permanent results, surgical intervention must be resorted to. The latter is indicated not only in cases of compression of a nerve by a tumor and abscess or of any other pathological condition in the vicinity of the nerve, but also in all cases of neuralgia which ordinary therapeutics, including the injections of alcohol, fail to relieve. The operative procedures consist of nerve-stretching, neurectomy and neurorhexis. In the first the results are frequently only transitory. The second consists of a resection of a certain portion of the NEURALGIA 435 affected nerve. The first two methods fail to give permanent results. The third method is based upon the pathological fact that a violent tearing out of a nerve produces permanent cellular changes in its nucleus (Van Gehuchten). This procedure has given by far better results than any other. NEURALGIA OF INDIVIDUAL NERVES A. Neuralgia of the Fifth Nerve (Facial Neuralgia). Etiology. — All the causes mentioned in the chapter on neuralgia in general are also factors in facial neuralgia. Special stress must be laid upon cold and malaria, which are frequent causes. Lesions in the area of distribution of the fifth nerve, viz. diseases of the alveoli, of the ear, of the cavities of the face (nose, frontal sinus), of the eye; diseases of the bones of the face through which the nerve passes, trauma- tism, inflammation, are all local causes of facial neuralgia. The disease is very frequent and is observed usually in the middle age. It is more frequent in females than in males. Pathology. — The nerve had been frequently found in an inflammatory state or in a state of degeneration. Atrophy of the cells of the Gasserian ganglion have been also observed in a few cases. Endarteritis has also been found. Symptoms. — Pain is the chief and sometimes the only symptom of the disease. It is agonizing in character. Its onset may be preceded by some paresthetic disturbances. Among the three branches of the nerve the first (ophthalmic) is most frequently affected. The tender points characteristic of neuralgia in general (see above) are: for the ophthalmic branch — supraorbital (at the level of supraorbital foramen) ; nasal (upper part of the nose) ; for the supramaxillary branch — infraorbital (below the lower eyelid) ; malar ; for the inferior maxillary branch — temporal (in front of the ear) ; labial (on the lower lip) : mental (place of exit of the mental nerve on the chin). The nature of the pain (tearing, boring), its paroxysmal character, the tendency to radiation over the branch affected and even beyond its boundaries, repetition of the attacks at regular or irregular intervals and their duration (a few seconds) , the development of the pain from the least influence (cough, touch, mastication, change of temperature of the food, etc.), are described in the chapter on neuralgia in general. The less constant symptoms of facial neuralgia are: secretory, vaso- motor, trophic, sensory and motor. Secretory. — During an attack there may be an increased nasal secre- tion, also watering of the eye and salivation. 436 NEURALGIA Vasomotor. — The conjunctiva on the affected side is congested, the eyelids may be cedematous and the face flushed. Trophic Disturbances consist chiefly of herpes, which is not infrequent in neuralgia of the first branch (ophthalmic herpes zoster). Falling of the hair and hemiatrophy of the face are occasionally observed. Sensory disorder in the form of hyperesthesia is frequent at the begin- ning, but anaesthesia is observed in older cases. Photophobia is frequent. Hearing and taste are sometimes affected. Motor phenomena are not rare. They consist of convulsive move- ments of the muscles of the affected side of the face accompanying the paroxysms of pain. This is the so-called "Tic douloureux." It is the most obstinate form of facial neuralgia. Course, Termination, Prognosis.- — In the majority of cases neuralgia increases in intensity at first, then gradually decreases. It runs an indefi- nite course. It may disappear completely, but usually persists. Recur- rences are frequent. The prognosis is serious. Diagnosis.- — See this chapter on neuralgia in general. Treatment. — It is the same as of neuralgia in general (see above). The surgical treatment consists of stretching the nerve, section or excision of a portion of it or tearing out the nerve (neurorhexis) as near as possible the bony foramen from which it emerges. The latter procedure gave better results than the former. The reason of it lies in this observation (Van Gehuchten and others) that the cells of Gasserian ganglion corre- sponding to the torn-off nerve degenerate more readily and permanently. Finally removal of the Gasserian ganglion may be resorted to in protracted cases in which the above means have failed. In treating a case of facial neuralgia it should be borne in mind that a diseased condition of the nose, ear, eye, teeth, of the sinuses may be the immediate cause of the pain. An examination of these organs is indis- pensable and a proper treatment is to be applied if indicated. In elderly individuals I have obtained very satisfactory results from nitroglycerine given either by the mouth or hypodermically. Favorable results in treatment of tic douloureux have been obtained from injections of alcohol into the nerve trunks as they appear on the face, or into their roots near the foramina from which they emerge. Schlosser, Levy, Baudouin, Ostwald, Brissaud, Patrick, Hecht and myself have published encouraging reports. For details see page 433. B. Cervico -occipital Neuralgia.- — Under this name is understood a neu- ralgia of the upper four cervical nerves. In the majority of cases it is the major occipital nerve that is involved, but the minor occipital, great auric- ular, superficial cervical and superclavicular nerves may also be affected. NEURALGIA 437 Etiology. — Cold, trauma, aneurism of the vertebral artery, infectious diseases (grippe, typhoid fever and others), syphilis, lead poisoning, auto- intoxication, gout, diabetes, anaemia are the causes of occipital neuralgia. It is also observed in cervical Potts' disease, cervical adenitis, hypertro- phic cervical pachymeningitis, localized tumors of the neck or of the cer- vical vertebras. Symptoms. — The classical symptoms of neuralgia in general are present. The pain is unusually severe. It is continuous with paroxysms of exacerbation. The slightest movement, cough, sneezing, cause an attack. The patient complains of a sensation of burning, tearing, stabbing in the neck. The tender points (of Valleix) are present. The most important and constant is situated midway between the mastoid process and the first cervical vertebrae,, at the level of emergency of the major occipital nerve. Other points: posterior border of the sterno-mastoid muscle; mastoid proc- ess. In severe cases there is also hyperesthesia of the skin of the neck, tenderness between the scapulae and in the supraclavicular fossae, loss of hair in the same region, myosis on the affected side. Prognosis. — It is usually good, but the disease is apt to recur. In one case under my observation it recurred six times in four years. The dura- tion is uncertain. It may last several weeks or months. In the above case the last attack lasted three and a half months. Diagnosis. — The characteristic tender spots, the radiation of the pain along the nerve are typical enough to make a diagnosis. Treatment. — Cauterization, blistering, application of iodine, blood- letting all over the painful area, will be of benefit. In the case mentioned above the local treatment was of no avail. Relief and subsequent recovery followed administration of strychnia in ascending doses. For details see Treatment of Neuralgia in general. C. Brachial Neuralgia. — This is a neuralgia of the brachial plexus. Etiology. — Traumatism of the brachial plexus, fractures and disloca- tions of the humerus, injury of a peripheral nerve, as a burn, compression, bite of the finger, etc. (reflex neuralgia), infectious diseases (grippe, malaria, typhoid), diabetes, gout, anaemia, neuraesthenia, are all causes of this affection. Symptoms. — Usually the pain affects one tor two nerves of the entire plexus. The ulnar nerve is most frequently involved. The pain follows the course of the nerve and is characterized by paroxysmal attacks between which the patient is free from pain. The position of the limb is character- istic. The patient holds it with the unaffected hand as if the arm were fractured. 43^ NEURALGIA The tender spots are found on the nerves of the plexus : for the musculo- spiral nerve the groove of this name on the arm; for the circumplex nerve the deltoid area; for the ulnar nerve the elbow; for the median nerve the wrist. Paresthesia, hyperesthesia or anaesthesia of the skin, hyperhidrosis, are frequently present. Prognosis is the same as in neuralgia in general but it depends greatly on the cause. Diagnosis. — The radiation of the pain along the nerve-trunks and the tender points are characteristic enough to recognize the disease. In compression of the cervical spin al cord pain may be present in the arms, but there are always other symptoms, as paralysis, atrophy, anaesthesia. In arthritis, in rheumatism, the pain does not follow the nerve-trunks. Treatment. — It is the same as in other forms of neuralgia. When local treatment and internal medications are of no avail, an operation must be resorted to. D. Intercostal Neuralgia. Etiology. — The causes of this affection are : cold, contusion of the nerves, fracture or diseases of the ribs, compression from within or without, diseases of the lungs or pleurae, an inflammation of which is transmitted to the nerves. As predisposing factors may be mentioned cachexia, anaemia, protracted diseases, syphilis, gout, chronic rheumatism. It is more frequently met with in women than in men. Symptoms. — The pain is usually unilateral and involves several inter- costal nerves. It may be spontaneous and then is usually deep seated. Similar to neuralgia in general, it presents paroxysms which are extremely severe. As the movements of the thorax provoke pain, the patient holds his chest immobile, avoids deep respiration and speaks with a low voice. The pain frequently radiates to the back and to the arm. The tender spots characteristic of neuralgia are here three in number: anterior, lateral and posterior. The first is at sternocostal articulation, the second on the axillary line, the third near the spinal processes. The skin on the affected side is hyperaesthetic. Herpes quite frequently accompanies or follows intercostal neuralgia. Prognosis. — It depends upon the cause. The disease is usually persistent. Diagnosis. — The above-mentioned character of pain, its radiation along the nerves, the tender points, are all typical enough for the diagnosis. Pleurodynia is recognized by a diffuse pain. In mastodynia the pain is in the breast. Tumors of the spine or spinal cord may present neuralgic NEURALGIA 439 pain as the only symptom for a long time before pressure symptoms make their appearance. Treatment. — The first indication is removal of the cause. General dyscrasic condition, compression, fractures, etc., must be taken care of. For relief of pain counter-irritation, cauterization and local applications of revulsive measures are of great benefit. Obstinate cases should be treated surgically (stretching, resection, tearing out of the nerves). For further details see Treatment of Neuralgia in general. For Franke's operation see page 254. E. Sciatic Neuralgia. Sciatica. Etiology. — The causes are local and general. Local. — Cold; trauma (contusion, injury of the nerve by a bony frag- ment) ; prolonged pressure against a hard object while sitting; compression in the pelvis by tumors and abscesses or by the head of the foetus during a protracted labor; inflammation of the meninges extending to the roots of the sacral plexus and then to the sciatic nerve. General. — Gout, chronic rheumatism, malaria, diabetes, intoxications (lead, carbonic acid gas, alcohol), infectious diseases (grippe, typhoid fever, puerperal infection), syphilis. Certain occupations predispose to sciatica. It has been observed quite frequently in tailors and dressmakers. Sciatica is a common affection. It is met more frequently in men than in women and particularly between thirty and fifty years of age. Symptoms. — They are sensory, motor and trophic, as the nerve is a mixed nerve. The first and chief symptom is pain. It is localized on the posterior aspect of the limb. It is continuous and paroxysmal. The pain may present various forms and degrees. In some cases it is tearing, burning; in others it is pulling. The paroxysmal exacerbations may be of an un- usual severity. The pain may affect the entire nerve or ou ly segments of it. It may radiate also during a paroxysm to the lumbar region, perineum and genitalia. The pain is usually worse in walking or standing. It is increased when the leg is extended. Exposure to cold aggravates the condition. Atmos- pheric changes also increase the pain. The tender points characteristic of neuralgia are: (i) In the groove between the trochanter and the tuber ischii, (2) in the middle of the pop- liteal space, (3) below the head of the fibula, (4) behind the external malle- olus, (5) dorsum of the foot, (6) Gara {Deut. mediz. Wochenschr., 191 1) has recently called attention to an abdominal tender spot in sciatica which he found constantly present in 1 18 out of 124 cases. It is elicited by press- 440 NEURALGIA ing the finger into the abdomen over the spinous process of the last lumbar vertebra on the affected side. He locates the point at a finger- breadth below the umbilicus and two fingerbreadths to the side of the median fine. The pain from compression radiates down the sciatic nerve. The skin over the posterior aspect of the limb is usually intact, but sometimes a diminution of sensations is present. In grave cases of sciatica complete anaesthesia has been observed. Parsesthesise, as numbness, cold- ness, tingling, etc.. are frequently present. Motor Disturbances consist of some loss of power, fibrillary contrac- tions, cramps in the musculature of the leg. Gradually, if there is no improvement, the weakness increases. The patient assumes certain fixed attitudes. In order to avoid extension of the nerve the patient places the leg in flexion, the trunk naturally bends toward the sound side; a scoliosis with the convexity to the affected side is therefore formed. Eventually this sciatic scoliosis becomes permanent. Sometimes the curvature of the spine is in the opposite direction, viz. the concavity is toward the diseased side. The patellar tendon reflexes are usually increased on the diseased side, although decreased and normal reflexes have been observed. The Achilles-tendon reflex is usually lost. The trophic disturbances show wasting of the muscles. There may be a simple flabbiness of the muscles or a genuine atrophy with qualitative electrical alterations (RD.). In such cases the changes of the nerve are profound. The skin of the limb is bluish, its secretions are diminished, eruptions (herpes, acne, erythema) may develop. Course, Termination, Prognosis. — In the mild forms the pain is only paroxysmal. Recovery follows in a few weeks. In the severe forms the pain is continuous, persistent and lasts months or years. In such cases deformities develop and the patient never recovers. Between these two extremes there are many intermediary forms. The prognosis is particu- larly unfavorable, when atrophy with reactions of degeneration is present. The chronic form is less hopeful than the acute. Double sciatica has an unfavorable prognosis, as it is usually the con- sequence of diseases of the spine or of the spinal meninges. It may also occur in diabetes. Diagnosis. — The exact seat of the pain on the posterior aspect of the leg and the characteristic tender points are usually sufficient for making a diagnosis. There is another important diagnostic test which is de- cidedly pathognomonic. It consists of overextending the leg of the patient when he is in a sitting or lying position; this manipulation brings on pain. It is the so-called "sciatic phenomenon" of Lasegue. NEURALGIA 441 In some affections of the lower limbs pain may simulate sciatica, but a careful examination will reveal the true condition. In muscular rheumatism the pain is diffuse and the tender points are absent. In arthritis of the sacro-iliac or hip-joints pressure from outside causes pain only at the level of the articulation. In sacro-coxalgia Lasegue's sign is also present. The following differential phenomena, according to Gueit (Gas. des hop., 1910) may be of use: when the patient lying flat on his back flexes his legs and an attempt is made to continue flexion of the thighs, a sharp pain in the upper and inner portion of the buttock will be felt in sacrocoxalgia, but not in sciatica. In the latter the preexisting pain is not augmented. In Meningo-myelitis or tumors of Cauda equina there is in addition to the pain in the limb also involvement of the sphincters. Besides, the symptoms are generally bilateral. An examination of the pelvic organs should be made in cases of pressure- sciatica, as a local unsuspected lesion may be the direct cause of the affec- tion (tumor, adhesions, etc.). For radicular sciatica see page 429. In all such cases an X-ray examination must be made. Treatment. — In cases of compression by a tumor or other elements removal of the cause is the first indication. The sciatica itself can be relieved first of all by rest. It should be a rule to put every patient suffering from this disease to bed in order to se- cure absolute rest for the limb. When the patient is restless, the limb should be immobilized by means of a board and a bandage. Otherwise speaking, it should be treated as if fractured. Every four or five days the bandage will be taken off for an hour or so and a gentle massage given to the leg. In the majority of cases absolute immobilization gives relief. Ame- lioration of pain can be also obtained from cauterization, counter-irrita- tion or any revulsion over the course of the sciatic nerve, from blood-let- ting (leeches or scarification), from local spraying of chloride of methyl, and especially from hot baths. The latter can be given once or twice a day. The patient sits in the tub fifteen or thirty minutes. In very obstinate cases I obtained considerable relief from prolonged baths — even two hours at a time. Galvanism, the faradic brush and static electricity may sometimes be useful, but as a rule these means cannot be relied upon for relieving pain. Internally the following drugs can be given: salicylates, any coal- tar product, iodides and bromides. Aspirin and salophen are superior to salicylates and in combination with small doses of codein have proven to be very useful in my hands. Very satisfactory results I have also obtained 442 NEURALGIA in some cases from strychnia in gradually increasing doses. In cases with a history of syphilis or malaria, mercury and quinine respectively will be useful. As sciatica frequently occurs in gouty and rheumatic individuals, autointoxication probably plays an important role. In such cases avoid- ance of nitrogenous food (meats) is advisable. In some of my cases a return to a meat diet has decidedly aggravated the sciatica. Alcoholic beverages are absolutely forbidden, because alcohol has a special predi- lection for peripheral nerves. Purgation at regular intervals is advisable. In favorable cases when the pain has subsided, the patient is allowed to exercise moderately his limb. In order to combat the wasting caused by disuse, massage should be then instituted: first gentle manipulation every day for ten to fifteen minutes, later deeper rubbing for a longer period of time. Faradism may be added to the massage in order to accele- rate the return of power in the muscles. Recently injections of alcohol into the sheath of the nerve or in the immediate vicinity have been recommended, but some observations show that this procedure may be followed by disastrous results; permanent paralysis and marked trophic disturbances have been reported. For the limitation of alcoholic injections see page 434. The same remarks can be applied to injections of osmic acid advised by some writers. Relief has also been obtained from injections of saline solutions (chloride of sodium, sulphate of magnesium) or of plain distilled water or else of sterile air. A subcutaneous injection of salts does not differ from an ordinary saline infusion. The dose is from 50 to 100 c.c. It has a mechanical and analgesic effect on the nerve. It may be made more effective by adding cocain or morphine according to Schleich's formula (feeble solution: cocain, 0.01; chloride of sodium, 0.20; morphia, 0.02; aqua, 100 grm.). Cordier recommended injections of sterile air in the close vicinity of the nerve, believing that the distention of the tissues stretches the nerve filaments. The injection is made in the lumbar region, at the external surface of the thigh and above the head of the fibula. The quantity of air injected at each of these points is 50 c.c. The injected area becomes pale. Vigorous massage must follow. Deep injections of chloride of sodium or sulphate of magnesia in 4 per cent, solutions have given better results. The point at which the injection is to be made lies at the junction of the inner third with the two outer thirds of a line drawn between the sacrococcygeal articulation and the postero-external border of the great trochanter. A long needle is inserted at a depth of 4 to 9 cm. and 40 to 60 c.c. of the solution are injected. NEURALGIA 443 These injections may be repeated several times. In one case I made six such injections at intervals of five or six days. Recently "spinal anaesthesia" has been proposed for the relief of sciatic pain. This procedure (see chapter on Lumbar puncture) which is em- ployed by surgeons in operations upon the lower part of the body, gives excellent results also in sciatica. Its efficiency is due to the effect of the injected drug upon the roots. The medications used in these cases are cocain or stovain (see Schleich's formula above). Epidural injections have given in some cases satisfactory results. Through the sacrococcygeal space are injected two centigrams of cocain solution (i per cent.) or 5-20 c.c. of saline solution to which one or two centigrams of cocain are added. The needle is inserted at a depth of 3 or 4 cm. This method while less efficacious than the preceding one as far as duration of relief of pain is concerned, is nevertheless useful. It can be applied not only in sciatica, but also in gastric and vesical crises of Tabes (Laven, Centralbatt f. Chir., 1910), in lead colic (Achard, Gaz. hebd. de med. et chir., 1901), in cancer of the vertebrae (Chipault, Congres de Chir., 1901). Langbein (Deut. Mediz. Wchn., 1913) uses 1 grm. of novocain mixed with one quarter its weight of sodium bicarbonate and one-half its weight of sodium chlorid. The powder is dissolved in 100 c.c. of distilled water, cold, and the mixture is injected. After the injection the patient reclines with the shoulders raised and the legs low. If there is a tendency to weakness, the patient is laid horizontal for a few minutes. In fifteen to twenty minutes all symptoms of sciatica disappear, but he keeps the patient in bed for two days. Out of twelve patients seven were permanently cured. For details of epidural injections consult Sicard and Cathelin (Compt. rend. Soc. de Biol., 1910). Rosi {Policlinico, 1912) has applied in eleven cases Baccelli's method of intravenous injection of a very weak solution of carbolic acid in treatment of chronic sciatica (1 per cent, or 1 per thousand solution). He obtained complete recoveries; from ten to sixty injections were required for the purpose. In grave and protracted cases, when the above treatment has failed, surgical procedures must be resorted to. Nerve-stretching, excision of portions of the nerve sometimes give relief. For more details see Treat- ment of Neuralgia in general. Recently Pers (Ugeskrift fur Laeger, Copenhagen, October, 1911) devised a method called "neurolysis," which he applied to seventy cases. After a considerable interval forty-seven out of fifty-eight were found permanently cured as also two others after a second neurolysis. The method consists of freeing the sciatic nerve from all adhesions that may 444 NEURALGIA have developed as the result of perineuritis and which according to the author are responsible for persistent sciatica. The incision along the back of the femur, from the lower margin of the gluteus maximus down to and below the middle of the thigh. The nerve is sought between the vastus externus and biceps and with the finger detached from its adhesions, work- ing always from below upward to the sciatic foramen. Baracz modified this method: he works his finger through the gluteus maximus fibers down to the nerve where it enters the notch and breaks the adhesions. The methods of Pers and Baracz may be successful in uncomplicated typical sciatica without two much atrophy. If the sciatica is associated with pain in adjoining regions, especially along the crural nerve and up- ward in the lumbar region, there is evidently an involvement of the plexus and no relief can be expected from neurolysis. To detect an old fracture or old arthritis of the lowest lumbar vertebrae or of sacro-iliac articulation, which all may simulate sciatica, an examination with Roentgen rays is necessary before neurolysis is decided upon. F. Lumbar Neuralgia. — This form concerns the branches of the lumbar plexus. Etiology. — Cold, rheumatism, grippe, malaria, diabetes, anaemia are the general causes. Locally the nerves may be compressed by pelvic, renal and mesenteric tumors. Symptoms. — According to the branches of the plexus involved the neuralgis will be present in different areas. (a) In Lurnbo-abdominal neuralgia the pain is in the lumbar region, lower abdominal area, scrotum and spermatic cord. To this category belongs also neuralgia of the testicle. During the paroxysmal attacks the abdominal muscles may become contracted. The skin may be hy- peraesthetic, but in old cases it is usually hypaesthetic. Trophic and vasomotor disturbances (herpes, etc.) may be observed. Spontaneous ejauculation may occur. (b) Obturator neuralgia is associated with an obturator hernia. The pain radiates to the internal surface of the thigh. (c) In Crural neuralgia the pain extends along the course of the crural nerve to the inner surface of the leg and foot. The tender spots are found in the inguinal region, on the inner surface of the knee, internal malleolus and inner border of the foot. (d) Neuralgia of the femoro-cutaneous nerve is known under the name of Meralgia paraesthetica. It was first described by Roth and Bernhardt in 1895. As the name implies, there is not only a neuralgic pain, but also paraesthesia. The latter consists of a burning sensation and a numbness over the antero-external surface of the thigh. The burning sensation is NEURALGIA 445 slightly decreased by flexing the thigh on the pelvis and the leg on the thigh. The paresthetic disturbances are continuous irrespective of the position of the body, but the walking or any displacement of the limb increases their severity and sometimes causes intolerable pain. Generally rest gives relief, but in some cases immobility increases the suffering. The tactile sense is diminished and the patient feels at each contact that something is interposed between the thigh and the hand applied to it. In some cases the least touch provokes severe pain. The diminished tactile sense (hypaesthesia) is frequently associated with a diminished pain sense (hypalgesia) and thermic sense. The tender spots characteristic of neuralgia in general are found here at the point of emergency of the external cutaneous nerve from the crest of the ilium. The causes of meralgia paraesthetica are: trauma, cold, infectious dis- eases, intoxications, constitutional diseases. The most frequent cause is injury. The anatomical relations of the external cutaneous nerve make it vulnerable: the nerve is placed in a muscle indispensable to standing and walking (psoas) and in a muscle (fascia lata) the contraction of which in walking presses upon it and stretches it. The anatomical examination made in a few cases has shown that the affection is due to a neuritis. Treatment— A prolonged rest may in some cases give relief. Sulphur baths, massage, galvanism, internal administration of iodides have been advised. In obstinate cases resection of the nerve is the only means to be used. Meralgia paraesthetica is not infrequently associated with inter- mittent claudication." This phenomenon, to which Charcot first called attention in 1856, is characterized by intense pain in the calves of the legs and difficulty of walking appearing a few minutes after the patient begins to walk. As soon as he sits down, the pain begins to subside and finally disappears. According to Charcot, the paroxysms are due to contraction and obliteration of the arteries in the affected limbs. Men are much more frequently affected than women. Russians and especially Hebrews are particularly predisposed to this affection. Erb made a statistical study of the etiological factors {Munch. Med. Wchn., 1910) and he reaches the following conclusions: Syphilis which is so fre- quently the cause of arteritis, plays apparently a slight role in intermittent claudication. He found only 8 per cent, cases of syphilis. Gout, diabetes, alcohol play a very negligeable part. Tobacco, however, is apparently a prominent etiological factor. Over a half of Erb's cases were heavy smokers. 44^ NEURALGIA Besides these general causes, local causes have an important place. Cold is particularly frequent. As to the clinical forms of intermittent claudications the following are observed: (i) Mild form, in which the entire condition consists of a sensation of weakness and fatigue, slight pain, some paraesthesia. They all disappear when the patient walks slowly. (2) Severe form in which all the symptoms are very pronounced from the beginning. (3) Association with Meralgia paraesthetica (see above). (4) Vaso-motor form, in which the vaso-motor disturbances predomi- nate over the other symptoms. Here the individual complains of "dead feet." The latter appear either pale or red. Goldflam {Neur. Centr., 1910) described the following phenomenon. Normally when the leg is extended several times in succession, and then left to drop, the foot becomes very pale; the pallor gradually disappears to be replaced by a hyperaemia. In cases of intermittent claudication this phenomenon is produced with great facility and is very pronounced. The diagnosis depends considerably upon the state of the pulse in the pedis dorsalis artery and posterior tibial artery. The pulse may be very small or totally absent. For differential diagnosis with spinal intermittent claudication see page 334. The prognosis must be made guardedly, as the condition may precede an eventual gangrene through the endarteritis. However great ameliora- tion may be expected from proper management. Treatment. — An effort should be made to remove the cause. The general and local circulation must be improved. Rest in a horizontal position, local heat and perhaps galvanism — may all be of benefit. In meralgia paraesthetica the intermittent lameness is clinically the exact reproduction of Charcot's type, but the etiological factor is different. G. Rare Forms of Neuralgia. (a) Coccygodynia or Coccygeal Neuralgia. — It occurs mostly in women. Trauma is a frequent cause. Cold, protracted labor may also produce it. Hysteria and neurasthenia are the predisposing causes. The disease is characterized by a pain in the region of the coccyx. The pain is increased in walking, defecation, micturition. The normal sitting position is ex- tremely painful. Rectal examination shows extreme tenderness of the coccyx. The treatment consists of faradization of the coccyx and rectal supposi- tories of opium or cocain. When hysteria or neurasthenia are the under- lying causes, an appropriate treatment of these affections is the first HERPES ZOSTER 447 indication. The disease is generally very obstinate. Extirpation of the coccyx is sometimes the only remedy. (b) Spermatic neuralgia is characterized by pain along the spermatic cord extending to the scrotum and epididymis. (c) Perineal neuralgia is characterized by pain radiating from the perineum to the penis, producing a desire for micturition which then be- comes painful. (d) Vesical, rectal, urethral neuralgias may also occur. (e) Metatarsalgia (Morton's disease), which is characterized by pain in the fourth metatarsophalangeal articulation, sometimes also in the second, is considered by some as the result of articular or bony changes and by others as due to a neuralgia of the external plantar nerve. Tightly fitting shoes or prolonged standing are probably the causes of this affection. The treatment consists of avoiding pressure on the heads of the metatarsal bones. Wide shoes may help. In protracted cases removal of the head of the affected metatarsal is the only means. HERPES ZOSTER (ZONA) This affection should be considered here, as it is very frequently asso- ciated with neuralgia (see preceding chapter). It is characterized by an acute vesicular eruption developed upon an erythematous base and following the course of a nerve or several nerves; it is accompanied by neuralgic pain. Pathology. — The lesion of herpes zoster is not definitely settled. Some believe that it is due to a peripheral neuritis. Others bring forward ample proofs of an involvement of the spinal ganglia (acute inflammation or hemorrhage) or of the Gasserian ganglion in cases of zona following the course of the fifth nerve. When the spinal ganglia are at fault, a secondary degeneration develops in the posterior roots and hence in the peripheral nerves. Some observers believe that the primary lesion lies in the spinal cord. The researches of Hedinger (Deut. Ztschr. f. Nerv., 1903), of Dejeine and Thomas {Revue Neur., 1907), of Head and Campbell (Brain, 1909) show that the ganglia and both portions (peripheral and central) of the roots are chiefly involved. Laminiere (Rev. Neur., 1907) has shown also a degeneration of communicating branches of the sympathetic which corresponded to changes in the lateral columns of cells in the gray matter. A radiculo-ganglionic lesion accompanied sometimes by a lesion in the cord is the anatomical basis of herpes zoster. Head's explanation of the pain and eruption is very interesting. A lesion of a spinal ganglion will produce a continuous sensory impression 448 HERPES ZOSTER which is directed towards the centers, hence the pain. On the other hand, a peripheral sensory fiber containing vasomoter fibrils of a centrif- ugal nature will transmit to the cutaneous blood-vessels the irritation caused by the lesion in the ganglion; hence, the erythema upon which the herpetic eruption appears. As to the pathogenesis of zona, within the last few years micro- organisms have been observed by some authors in cultures obtained from the cerebro-spinal fluid of patients suffering from herpes zoster. Very recently (Deut. Mediz. Wchn. N° 18, 1913) Sunde reported a case of ophthalmic zona which came to autopsy, and a coccus was found in large quantities in the Gasserian ganglion. The micro-organism was es- pecially in abundance in the hemorrhagic foci and small blood-vessels. The ganglion was in a state of acute inflammation; small hemorrhages and round-cell infiltration were marked. Symptoms. — Pain and eruption are the characteristic signs of the affection. Pain is shooting in character. According to the localization of the zona special symptoms will be present. (a) Intercostal Zona. — This is the most frequent form. The onset of the disease is usually preceded by fever and general malaise. The de- velopment of the vesicles may be sudden or gradual but commonly it ap- pears on the third or fourth day. Pain may precede the eruption or follow it. Besides pain there may be a burning, itching or tingling sensation. The eruption may follow the course of an intercostal nerve, anteriorly and posteriorly, but sometimes it has a different direction and crosses the nerves. At first the skin is only erythematous, but soon elevations are noticeable in areas. The center of the latter becomes a vesicle; the con- tents of the vesicle is at first serous, but later becomes purulent and hemor- rhagic. The lymphatic glands in the vicinity become enlarged in the majority of cases. The bleb soon dries; on the fifth or tenth day a crust is formed and when it falls off, a small scar is left. Usually when the rash begins to heal, the pain disappears, but in elderly individuals it per- sists months after the eruption has ceased to exist. The affected area presents objective sensory disturbances: there may be hyperesthesia or anaesthesia. The latter is present especially over deep and extensive scars. • The affection lasts from several days to several weeks. (b) Ophthalmic Zona. — There is usually no prodromal period. The symptoms may consist only of an erythema with cedema of the eyelid. In the majority of cases vesicles appear with very violent neuralgic pain on the forehead near the middle fine, on the upper eyelid, and the base of the nose. The center of the eruption is the supraorbital foramen. Sometimes HERPES ZOSTER 449 the rash extends to the side of the nose down to the alae. Hutchinson pointed out the seriousness of the latter occurrence: he observed in such cases almost invariably ulceration of the cornea. Grave symptoms frequently accompany ophthalmic zona, viz. con- junctivitis, keratitis (neuro-paralytic) , iritis, suppuration of the cornea and of the entire globe. Optic neuritis, paralysis of the third and sixth nerves have been observed in some cases. (c) Zona, of the face may affect all the sensory branches of the fifth nerve. It may involve the lips, nose and cheeks. The tongue is also occa- sionally involved. R. Hunt has shown that a herpetic eruption over the auricle and in the external meatus is due to an inflammation of the geniculate ganglion (geniculate poliomyelitis) . It is frequently associated with palsy of the seventh nerve (see chapter of peripheral facial palsy). (d) Zona, of the extremities may follow the exact course of individual nerves or, similarly to intercostal herpes, present an independent or irregu- lar distribution. In a case of cervico-brachial herpes zoster observed by me {Amer. Medicine, 1909) on an aged woman excruciating pain was followed three days later by a vesicular eruption extending from above the left scapula down over the external aspect of the left upper limb to the wrist. (e) Zona may occur on the abdomen, dorsum, neck and genitalia. Course, Prognosis. — Apart from the complications of the ophthalmic form, the prognosis is usually favorable. Recurrences take place, although rare. In one of my patients, a man of twenty-nine, three attacks occurred within two years and invariably in the same area (intercostal). Bilateral herpes is also rare. Etiology. — Trauma and cold, intoxications with carbonic acid gas, lead and arsenic are not infrequently traced as causes. It has also been observed in the course of infectious diseases, (grippe, pneumonia, etc.) of diabetes. Finally zona may develop independently as a primary affection with a febrile onset and glandular swelling; other- wise speaking, as an infectious disease. The analogy with the onset of acute anterior poliomyelitis is striking. Epidemics of zona have been observed by a number of writers. Herpes may occur at any age, but pre- dominately under the age of twenty-five. Infants are also subject to it. Aged individuals are peculiarly susceptible to it. Treatment. — The primary form should be treated as any other infec- tious disease by diuretics, purgatives and rest in bed. The secondary or symptomatic form will be managed according to the original malady. Locally the vesicles should never be pierced. Applications of sedative ointments or liniments with opium and cocain and later during 29 45° HERPES ZOSTER the drying stage of zinc powder are usually sufficient. I obtained good results with application of ichthyol. For relief of pain bromides and coal-tar products can be given. In protracted cases arsenic and galvanism are advisable. Resection of the posterior spinal roots at the level cor- responding to the area involved has.been recommended and good results reported {Leriche. Lyon Chir., 19 12). CHAPTER XXV FUNCTIONAL NERVOUS DISEASES NEURASTHENIA (NERVOUS EXHAUSTION) Under this name is known a symptom-group the chief characteristic of which is a persistent neuro -muscular fatigue with general irritability. Beard (in 1880) was the first who grouped together the individual symptoms of the disease which prior to him were attributed simply to nervousness. Symptoms. — (a) Diminution of Muscular Energy is the most promi- nent symptom. It is manifested in a lassitude which interferes with the patient's daily work. The usual physical exercises cannot be carried out. The least exertion brings on an undue fatigue. In some cases the tired feeling is felt only in the legs, in others in the entire body. In extreme cases the patient refuses to stand, walk or move from one place to an- other. The characteristic feature of this fatigue is that it is particularly marked in the morning. In such cases the sleep is not refreshing to the patient; in getting up he feels as if he had not spent the night in bed. In other cases the fatigue is not continuous, but paroxysmal. The patient gets some relief for a few hours and then suddenly upon a slight exertion or even without it begins to feel the fatigue. (b) Neurasthenics very frequently complain of backache. In some cases it is not a genuine pain, but a pressure, a burning or only an inde- scribable discomfort along the spine. These sensations usually increase upon exertion or more or less prolonged standing. The skin over the spine is over-sensitive, so that the least touch or even the contact of the clothes makes the patient uncomfortable. The most frequent seat of the back- ache is the lower (sacral) portion of the spinal column. (c) Headache is a common occurrence. It consists frequently of a sense of fullness, heaviness, pressure or of a band-like constriction around the head (neurasthenic lead-cap). It is sometimes confined to the fore- head, the temples, but most frequently to the occipital region. In some cases instead of fullness there is a sensation of emptiness, of a vacuum. In other cases the headache is accompanied by vertigo, noises in the ears, dimness of vision. The headache may be continuous or paroxysmal. It is particularly evident in the morning. 45i 452 NEURASTHENIA (d) Gastro -intestinal Disorder is a very frequent symptom. In the mild form there is usually preservation of appetite. The patient enjoys his food, but an hour after the meals a discomfort appears. Fullness and pressure in the epigastrium, eructations, are tormenting the patient. At the same time he feels oppressed, flushes of heat go to his head, the heart palpitates. He feels somnolent, heavy. This condition lasts during the entire process of digestion. Constipation is a usual accompaniment of neurasthenic dyspepsia. As to the chemical processes of digestion, the latest observations show that they are about normal in mild cases. The dyspeptic symp- toms are due to a deficient motor innervation of the gastro-intestinal tract fatonyj. It is also interesting to notice that in spite of the digestive disorder the patients do not lose in weight. When, however, the digestion is considerably disturbed, the general nutrition suffers. In such cases there is a marked diminution of free hydrochloric acid and the constipation is obstinate. The patient loses in weight, he is emaciated and pale. (e) Insomnia, while not constant, is quite frequently met with. Either the patient has great difficulty to fall asleep or awakes several times during the night in a state of anxiety and excitement. As a rule the neurasthenic sleep is incomplete. Terrifying dreams are very frequently observed. (/) Mental Fatigue almost always accompanies muscular fatigue. The majority of the neurasthenics suffer from inability to concentrate their thoughts upon one subject for any reasonable length of time. They are absent-minded, incapable to solve problems of a more or less complex nature. Figuring becomes a difficult task. A long conversation on a serious subject is impossible. They are hesitating in their actions and decisions. The least attempt to resume their usual mental occupation brings on headache. In some cases, however, the patient retains his mental energy, but the muscular fatigue is persistent. In others the mental fatigue appears only after a certain number of hours' work. There are great variations in the degree and intensity of the mental exhaustion. The disposition of the patient usually undergoes a change. He is depressed, discouraged, highly irritable, cannot stand contradiction. He avoids his best friends, wants to be let alone. He is pessimistic and does not find pleasure in anything. (g) Circulatory disturbances are very frequent. Cardiac palpitation occurs upon the least emotion. Sensations of cold or heat along the spine or in the extremities, lowering of vascular tension, are all due to deficient NEURASTHENIA 453 tone of the vasomotor apparatus. The patient's skin and especially the hands and feet are moist, clammy. The six symptoms just described are characteristic of neurasthenia. There are some minor manifestations which are not observed in every case, but frequent enough to deserve mentioning. Sensory disturbances may be: hyperesthesia generalized or, more frequently, localized; various paresthesias, as tingling, numbness, burn- ing, itching or pain. Pruritus ani, prurigo in general may occur in neuras- thenics. Neurasthenia predisposes the individual to neuralgia. The special senses may also be disturbed. The eyes get easily fatigued. As soon as the patient begins to read, the letters become blurred and the eye- globes painful. The hearing is somewhat affected; the least noise gives a painful sensation in the ears; various noises are heard by the patient The taste and smell also suffer sometimes. Motor disturbances are sometimes manifested in cramps in the legs and tremor. The latter is seen in the tongue and fingers. Vertigo is occasionally observed. It may be continuous or paroxysmal. In the latter case it occurs mostly in the morning. Secretory disturbances are various. Either there is a diminution or increase of secretion. In the first case the skin and the m.ucous membranes are dry; the quantity of urine is diminished. In the second case the perspiration and the amount of urine are abundant. Polyuria and phosphaturia are sometimes observed. Sexual Disturbances are as a rule present In the average case it consists of some degree of impotence. In some cases, however, the disor- der in the function of the genitalia is so predominant that the disease deserves the special name: "sexual neurasthenia." Sexual excitement, priapism, nocturnal emissions, premature ejaculation, spermatorrhea, burning sensation in the urethra, extreme tenderness of the scrotum, testicles, penis, are all symptoms of this form of neurasthenia. Added to the other symptoms of the disease, they put the patient in a state of extreme anxiety. Gradually his sexual desire decreases and even disap- pears. The latter together with impotence and frequent emissions has an unusual depressing effect on the sufferer. Course, Prognosis. — When the cause is removed and the proper treatment promptly instituted, recovery may follow in a few weeks. The duration of the malady depends upon these two factors. It should, however, be borne in mind that recurrences are not infrequent. Generally speaking, the prognosis is favorable. Except the cases in which the neurasthenic symptoms are secondary to some grave disease (tuberculosis, syphilis, etc.), neurasthenia is a curable disease. 454 NEURASTHENIA Pathogenesis. — There are no absolutely certain facts concerning the nature of neurasthenia. Various theories have been advanced. Whether in the course of neurasthenia some toxic material circulates in the blood and produces the fatigue in the nervous and muscular systems, or else the nervous system is affected first from overuse and the other organs are disturbed in their nutrition secondarily, it is impossible to tell in the state of our present knowledge. The cells of various cerebral centers primarily and systems of neurones secondarily are probably disturbed in their function. Diagnosis. — Continuous fatigue in the physical, intellectual and moral spheres, especially manifested upon the least physical or mental exertion, irritability, prolonged backache and headache, insomnia, gastro- intestinal atony, are all symptoms sufficiently characteristic of neuras- thenia. Each of these symptoms individually taken may be observed in otherMiseases. It is therefore essential that the entire symptom-group be present. There are some organic nervous or mental diseases which may be preceded by a prolonged period of symptoms resembling neurasthenic manifestations. The prodromal stage of paresis, of melancholia and mania are characterized by such symptoms. They resemble, however, neurasthenia only superficially, they never present the true and complete picture of the latter. They are merely neurasthenoid (neurasthenia-like). The differentiation is therefore necessary, as the prognosis will be radically different. Neurasthenic symptoms may be observed in the course of cerebral tumors, of tabes, of cerebral syphilis, of exophthalmic goiter. A careful examination is necessary in every case with a neurasthenic symptom- group, as an organic disease of the nervous system may be overlooked. This occurrence is particularly noticeable during the earliest period of paresis (see page 436). Neurasthenia is frequently associated with hysteria. As the symp- toms of the former are mostly subjective and of the latter objective, there will be no great difficulty in differentiating them. Etiology. — Neurasthenia is a very common affection. Excesses of any kind, sexual, alcoholic and others, masturbation, prolonged and uninter- rupted intellectual effort, depressive emotions, fright, anxiety, worry, traumatism, are the most frequent causes of nervous exhaustion. Neuras- thenia is also observed in syphilis; it follows an attack of grippe, typhoid fever or any other protracted infectious disease. The symptom-group accompanies also tuberculosis, anaemia, chlorosis, lead intoxication. This is the so-called "Neurasthenia symptomatica." NEURASTHENIA 455 A neurasthenic state develops more easily in individuals with a path- ologic heredity (nervous or mental diseases), as their predisposed nervous system is more apt to succumb under the influences mentioned above than in persons with a normal make-up. It should not be forgotten that nervous exhaustion may occur in any individual subject to the effect of the described causative factors. The latter may make a perfect recovery, while the former is easily apt to suffer recurrences. Treatment. — The first indication in neurasthenia is to combat its most distressing symptom, viz. fatigue. Physical and mental rest is the most important element of the treatment. When the tired feeling is marked, the patient must be put to bed. While in bed all mental exertion should be avoided. He should be kept away from all possible causes of worriment or excitement. The latter can be accomplished when the patient is isolated and placed under the immediate care of an intelligent nurse. Full and nutritious feeding is necessary. The state of gastro- intestinal digestion should be taken into consideration in each individual case. Atony of the bowels can be combated by massage of the abdo- men, by internal administration of laxatives, among which cascara sagrada is the most desirable, by daily ingestion of sufficient amount of fruit or green vegetables. Atony of the stomach can also be remedied by local massage over the gastric region and by administration of bitters, as mix vomica with gentian, nitro-muriatic acid, etc., immediately before meals. The feeding should be done regularly three or four times a day. Stimulants of all kinds are forbidden. Coffee or tea may be given in very small amount and forbidden, if the sleep is disturbed. In selecting a diet, special attention must be given to the character of food. Some patients, for example, refuse to drink large quantities of milk. It is advisable in such cases to have the patient take the milk at first in very small quantities and then very gradually increase the amount of it. By doing so I have succeeded in a number of instances in making the most stubborn patients drink large quantities of milk. Milk is an ideal food for patients confined to bed. If in exceptional cases it causes gastric disturbances in spite of all possible precautions, it should be discontinued. Vegetables, fruit, small amount of meat, eggs, custards, are very nutritious articles for neurasthenics. Sweets and very starchy food should be avoided. Drugs are not absolutely necessary in every case. Glycero-phosphates, lecithin are excellent in asthenic conditions of the nervous system. Iron and arsenic are indicated if the blood examination shows a state of anaemia. In case of insomnia or only disturbed sleep sedatives should be avoided as long as possible. It can be combated sometimes by simple measures, as a cool sponge bath] or a tepid general bath of ten minutes' 456 PSYCH ASTHENIA duration; sometimes also by the application of a cold wet towel to the neck. If these means fail, bromides, veronal, sulfonal, trional, medinal^ may be tried. Ten grains of any of these drugs is generally an average dose. Of course the latter will be increased according to the indications. A prolonged rest in bed is not without some inconveniences, as it may lead to loss of appetite, to retardation of all the functions, to muscular wasting. To obviate these disturbances, S. Weir Mitchell recommended to associate with the rest in bed also massage, passive movements and electricity. At first these measures may be applied daily, but later only every other day. By these means the general nutrition is benefited considerably. Hydrotherapy is a very useful adjuvant. It can be administered as a brief douche, a sponging or ablution. The temperature of the water will vary according to the case. Some patients cannot stand cold, some pre- fer tepid water, others feel better after hot water. A gentle general rubbing after the application of water is not to be neglected. In mild cases of neurasthenia the rest may not necessarily be absolute. The majority of the cases are walking neurasthenics, who are compelled to work. In such cases a brief relaxation from work at the beginning of the treatment should be insisted upon. If it is impossible to obtain it, the patient is instructed to take rest as much and as often as he can. An hour's relaxation once or twice a day will be very refreshing to him. Cool sponge-baths, mornings and evenings, followed by a hard rub, will help the patient considerably. He must retire early, avoid all excitement, in- cluding sexual life; otherwise speaking, he must secure as perfect rest as possible. Smoking must be done very moderately and avoided at night, as it is an excitant to some individuals. As to diet, etc., see above. NEURASTHENIC PSYCHOSES (PSYCHONEUROSES) (PSYCHASTHENIA) Closely associated with neurasthenia are certain mental disturbances which develop in individuals especially predisposed, viz. neuropaths. The latter present a special make-up of their nervous system which be- comes easily affected from the least cause. They are individuals whose nervous or mental equilibrium has not a solid basis and is constantly threatened with a break. They are usually burdened with a heavy heredi- tary predisposition. They are peculiar, very emotional, impressionable, self-analyzing, extremely sensitive and scrupulous. They are eccentric, dreamers, with romantic tendencies. They are subject to attacks of great anxiety, to morbid fears, obsessions. The mental symptoms may appear at various periods of the patient's life, but they ordinarily develop PSYCHASTHENIA 457 when the organism is in a state of exhaustion. In such individuals the neurasthenic symptom-group develops with the greatest facility and is characterized by its special tenacity and persistence. The nerasthenic manifestations may disappear, while the psychic dis- turbances will persist. Usually, however, they develop and improve simultaneously. The chief characteristics of the mental symptoms is complete lucidity of the patient's mind, his complete consciousness of their presence. The patient realizes the absurdity of the phenomena, but is unable to overcome the irresistible sensations. A brief description of the psychic disturb- ances is necessary. A. Phobia or Fear. — The most common form of fears is agoraphobia. The patient fears an open space. He will avoid crossing a large avenue, a boulevard or a field. Monophobia is the fear to be alone. Claustrophobia is the fear of a closed space. The number of phobias can be increased indefinitely. To this category belongs also a special phenomenon, viz. fear of touch- ing objects; it is called: Delire du toucher. A fear naturally develops a state of anxiety. If, for example, the patient suffers from agoraphobia and he is compelled to cross a field, he will become agitated, he will tremble, he will make several attempts to do the act, his heart will palpitate violently, he will perspire abundantly. The state of anxiety will be so great that he will have to abandon his plan, or else to procure himself someone who will accompany him. B. Doubts or Indecision. Folie de Doute. — A patient affected with morbid doubts may reveal the state of his mind in every act of his life. In writing a letter, for example, he will not be certain whether the proper sentences were used or his signature is placed. He will therefore tear up the envelope, read the letter over again or write another. He may repeat the same act several times. Another patient after turning out the gas in his room at night may be tormented by the possibility of a mistake. He will get up, light the gas again, turn it out and a few minutes later per- form the same act over again. Not being sure of the correctness of their actions, such patients are continuously in a state of doubt. This condition may interfere with their daily occupations, with their sleep and with their whole life. They feel unhappy. The French call it madness of doubt (Folie de doute.) C. Obsessions, — Under this term is understood a state of mind when a certain thought, idea or image invade it and the patient is unable to free himself from them. He realizes the groundlessness, the absurdity 45^ HYPOCHONDRIA of such tenacious ideas or images, but he finds himself powerless in the presence of the irresistible force. Kleptomania, for example, is a variety of obsessions. The patient cannot resist the irresistible impulse to steal insignificant objects. One of my female patients cannot resist a knife. She would get paroxysmal desires to kill, although she would make efforts to avoid the sight of sharp instruments. Another patient was obsessed by the idea that her child will die at a certain hour. In some cases the ob- sessions may be so intense that irresistible impulses follow and crimes are committed. What characterizes the obsessions is the complete lucidity of mind. The patient is perfectly conscious of the criminality or absurdity of a cer- tain act. He struggles against the thought and impulse, he suffers morally. He may sometimes overcome the fight, but sometimes he succumbs and commits a criminal act. D. Abulia or Deficient Will. — The patient thus affected has not the power to do a certain act. One of my patients while walking on the street could not step over a loose brick or a leaf of a tree. Another patient in going to his business office could not walk over the shortest distance, but had to select the longest route. Should these patients, recognizing the ridiculous side of their condition, attempt to overcome it, they would be thrown into a state of anxiety, would tremble and even cry. Their suffering is indeed great. E. HYPOCHONDRIA This affection is quite frequent. It is related in some of its manifes- tations to neurasthenia, but it is different from the latter in its essential features. It occurs mostly in men before middle life. It develops usually upon a neuropathic basis (see preceding chapter) . Symptoms. — The disease is characterized by a vivid sense of having one or more organs in a diseased state. Functional changes are usually absent, but if they are present, they are extremely slight. The patient complains of a multitude of vague symptoms referable to one or several organs. Believing that a special organ is affected, he - will observe its function from day to day or from hour to hour and the most insignificant symptom will be interpreted by him as pathognomonic of some disease in a state of development. If he concentrates his thoughts upon the digestive apparatus, he will adopt a special diet, exclude important articles of his food, change the regime every few days until a new symptom from another source will make its appearance. At once he will give up the idea of the gastro-intestinal tract andattribute his illness to another HYPOCHONDRIA 459 organ. Should he notice an increase or diminution in the daily quantity of urine or a change in its color, his thoughts will be transferred to the kidneys. One of my patients, a male of thirty-eight,, believed two years ago that his testicles were in a diseased state. He began to wear a sus- pensory padded with cotton in the hottest days of July, would apply extreme heat mornings and evenings, so that the scrotum became in- tensely erythematous. Six months later, subsequently to exposure, he developed a mild lumbago. At once he began to believe that the origin of his troubles lies in the kidneys. He removed the suspensory with the cotton, also all application from the testicles and abandoned himself to the analysis of the renal function. His urine had to be examined chemi- cally and microscopically every week. Gradually he took up each of the abdominal and thoracic viscera and now he is under the impression that his brain is affected. In spite of his continuous preoccupation over his body, he never missed a day in attending to his affairs (he is a success- ful broker). In another series of cases the patients complain of symptoms referable to the entire organism. One of my patients, a male of thirty-eight, has disagreeable sensations in the region of the liver and of the stomach, in the epigastrium; he has palpitation of the heart, numbness and aching in the limbs, shortness of breath, pressure and pulling sensations about his head. A thorough examination failed to reveal physical signs of diseases of any organ. What is striking about these patients is good general development of the body. The above mentioned patient has been suffering for several years and still he has a healthy appearance. In some cases, however, they lose in weight, especially when they restrict their diet to a very limited amount of food and exclude all nutritious articles. The improper food selected by the patient himself may lead to gastro-intestinal disturbances. Believing that he may be affected with some pulmonary disease, the patient will take special precautions against cold. He will- fear to go out and when he does go out, he will put on chest protectors, scarfs, abdominal binders, etc. Overheated he then exposes himself to cold more readily and frequently takes cold. All these disturbances are usually slight, but the patient will always exaggerate them to an unusu al degree. A very frequent form of hypochondria is the one pertaining to the sex- ual function. Impotence is a common complaint. In reality there is only a fear and anxiety about the sexual power. In the majority of cases the patients are capable to perform the act. It is therefore a "psychic impotence." Being preoccupied by their ideas, they look for causes 460 HYPOCHONDRIA of their impotence, and should masturbation be present in their history, they attribute the former to the latter and believe themselves incurable. An occasional emission will reinforce such a belief. Another characteristic feature of the hypochondriacs is the tendency to speak to everybody about their ills. Not infrequently they keep accur- ate records of the slightest changes in their condition. One of my patients used to send me every fourth day a most detailed account of his hourly state of health. Going from physician to physician, from hospital to hospital, they carry with them their records and insist upon reading them. During the examinations they watch the expression of the physician's face all ready to interpret in their own way the least change. The edu- cated hypochondriac frequently consults medical books, looks for his symptoms in various diseases and usually in the most incurable affections. His fear and anxiety are naturally increased and the more he examines himself the more he becomes convinced of the hopelessness of his condition. Desperate he leaves the regular physician, procures patent medicines, con- sults quacks, takes up osteopathy, faith cure, Christian science, etc. Some- times he keeps on taking medicines and follows at the same time some of the above special cures. This state of affairs continues months and even years. Diagnosis. — The disease is frequently confounded with neurasthenia. In the latter there is the typical chronic fatigue manifested upon the least mental or physical exertion, the striking irritability upon the least provoca- tion. In hypochondria these symptoms are absent. It is true that in some cases of neurasthenia there may be present some hypochondriacal ideas. In the majority of cases each of these affections is separately encountered and the typical pictures characteristic of each are observed. A hypochondriacal condition is observed as an early stage of some forms of insanity. The qualitative mental changes of the latter are not found in hypochondria. Etiology. — A neuropathic basis is commonly observed. Idleness, monotony, absence of interest in any special pursuit in life, sedentary occupations, alcoholic or other excesses, lowering of the general nutrition, are all important causes of hypochondria. It is frequently observed in advanced medical students and young practitioners. It is also observed in those individuals who, after having accumulated large means, retire at a too early age and begin to lead a life free from activity. Course. Duration. Prognosis of Psychoneuroses. — The phenomena described above do not necessarily accompany the psychoneurotic indi- vidual through his entire life. They are but episodic manifestations. They may appear at any time upon the least accidental psychic shock HYPOCHONDRIA 46 1 or after some intercurrent disease. They disappear after an appropriate treatment, but may reappear with the greatest facility. These individuals possess a special make-up of their nervous system, whose reaction to external and internal stimuli is different from that of normal individuals and demonstrates an extraordinary emotionality, impressionability, ex- tremely prompt response to the slightest stimulation. Instability of character, of disposition and of mentality — are the chief characteristics of the psychoneurotic or psychasthenic individuals. Recovery is possible, but usually the condition lasts many years. It may disappear with advanced age. Treatment of Psychoneuroses. Psychotherapy. Psychoanalysis. — Before psychotherapy and psychoanalysis are discussed, a few words will be said of the general management of the cases. Psychoneurotic individuals are persons with a specially unstable nervous system. Ap- propriate hygienic measures should be always applied. Careful dis- crimination must be made in the choice of occupation. A career free from great mental strain and from multiple obligations should be chosen. A simple life, regular habits, avoidance of alcohol and of sexual excesses, hydrotherapy, outdoor moderate exercises, are essential. The nature of the manifestations observed in psychoneurosis is a sufficient indication of the fact that mere medications or physical means will fail to relieve the tormenting thoughts of psychasthenic individuals. The chief element which is affected is the psyche. The treatment in such cases must therefore be before everything else psychic. The main object consists in enabling the sufferer to overcome the distressing thoughts by means of mastering his will power and thus dislodge the obsessive thoughts which are so persistent and so tenacious. The chief aim in psychotherapeutics is to reach the point where the patient begins to gain conviction of the curability of his case. As soon as this is attained, recov- ery follows rapidly. But to reach that conviction requires considerable effort on the part of the physician. It can be accomplished by gradual and repeated suggestive influence. Suggestion can be exercised in any form for practical purposes. Thus advantage was taken of this idea by various sects and lay persons. The history of Lourdes in France, of Christian Science, of hypnotism, of Emmanuel movement, as well as of mesmerism with its theory of magnetism — all proves that suggestion has given even with the unscientific methods some good results in some selected cases. The scientific conception of suggestive methods begins with Bernheim of Nancy. Suggestion consists of forcing into the patient's mind the idea or the conviction of his eventual recovery. This conviction can be gained in two ways: either blindly or by the way of 462 HYPOCHONDRIA reasoning. In the first case the patient falls entirely under the healer's influence, such as is seen in hypnotism or in the methods employed by various cults or religious movements. In the second case, viz. with the method of reasoning the conviction is gained by the patient after one or several seances of logical and reasonable demonstration of the unfounded cause of anxiety or worry over ills that are not in existence. This method is persuasion. It is rational and scientific. We have here an exposi- tion of facts in the interpretation of which the patient himself partici- pates intelligently. It is most enduring as the patient's intelligence is thereby trained in the method of observation. A hypochondriac, for example, complains of his heart, kidneys, liver, which undergo degen- eration in his judgment. The first requisite in such a case is the necessity of the patient's confidence, and this can be accomplished by giving the patient our close attention. He must feel that we are ready and anxious to hear his account from beginning to end, that we, as a friend, are interested in his welfare. We must exhibit a certain amount of sympathy. Sincerity and actual charitable spirit must be present in the physician. The next step is to teach the patient that his heart is not diseased by teaching him to count his own pulse, by pointing out the absence of various disorders in the lungs, kidneys. If the patient's fixed idea has a relation to the kidneys, examine his urine in his presence, demonstrate the absence of abnormal elements indicative of a renal lesion. Should the patient actually suffer from some slight disturbances in the domain of any of his organs, an appropriate treatment must be instituted. Special stress must be laid on the slightest improve- ment, on the disappearance of symptoms, so that the patient will see in it each time a new reason for encouragement. If the patient's expression, tone of voice, reveals still some skepticism, it must be at once attacked with gentle vigor, always basing oneself on facts which should be actually demonstrated to the patient. In such a manner the patient will succumb to evidences brought before him and accept our statements as incon- trovertible. Considerable tact and skill must be displayed by the phy- sician. By tactful and encouraging words or demeanor he will be able to exercise a powerful influence in his attempts to dislodge the morbid fixed ideas. Psychotherapy understood in its proper sense is useful in management of all ills, but finds its greater field of usefulness in psycho- neuroses. Psychoanalysis. — In spite of the excellent results obtained in a large number of cases by the methods of persuasion and suggestion, there are nevertheless instances in which psychotherapeutics are insufficient and sometimes meet with total failure. A new psychological method has been HYPOCHONDRIA 463 developed by Breuer and especially by Freud, which enables one to get a deeper insight into the nature and origin of the morbid phenomena of the psychoneuroses. It is based on the principle that the mental processes of an individual are composed of two separate groups. One of them controls the conscious acts of daily life. The other group constitutes ideas and wishes which occurred to us some time ago, but which the conscious ego endeavored to reject, but as they cannot be annihilated totally, they remain in a latent state and therefore subconscious. The conscious personality continuously strives to absorb the latter by assimilation, but when it is incapable to do so, a conflict is established between the two groups of thoughts, and then various phenomena characteristic of psy- choneuroses make their appearance. Normally the conscious ego is victorious in the conflict, the subconscious ideas are assimilated and destroyed, but if the latter are only pushed to the background, they will invariably emerge and under the influence of any exciting cause will manifest themselves in some morbid act or morbid tendency. The latter may not take the form of the original idea or wish, but some other form and become thus merely a substitute. If there is a possibility of bringing to the surface the subconscious mental presentations so that the conscious ego will thus be enabled to meet them, get full control over them and assimilate them, the morbid mental phenomena will be removed. One of the procedures utilized for this purpose is the free association" method. The patient is told to concentrate his thoughts on his special psychoneurotic disturbance and bring to his memory all possible ideas that occur to him at that time. He must be reminded not to select only those ideas that in his opinion are proper to relate but repeat indiscriminately every insignificant fact, no matter how ridiculous or improper it may appear. In the mass of expressions and sentences uttered by the patient who is unburdening himself from long forgotten thoughts the so-called pathogenetic link will be found. It will be seen that the patient's suffering is but the outer expression of an effect produced by ideas or wishes concealed in the subconscious world of the individual's ego. When they are brought to light, the patient sees them one by one passing before him, he gains control over them and finally frees his personality from them. The psychoanalysis just described is unquestionably a very useful procedure. Unfortunately in its practical applications it presents some difficulties. The most objectionable among them is that it consumes considerable time and many seances with the patient before we succeed in having him unburden himself freely from all his thoughts, especially those of a disagreeable nature or of a sexual character. Freud claims that 464 HYSTERIA the chief causal factor in all psyche-neuroses including hysteria is always a sexual moment, a sexual trauma occurring some time in the patient's life even in childhood; that the psychoneurotic phenomena are the result of the conflict between libido and sexual repression and the symptoms are a sort of as compromise between these two psychic streams. This conception of a sexual factor as being the only etiological factor in psychoneurosis is not as yet accepted by all. F. ANXIETY NEUROSIS A state of "anxiety" is found in every form of psychoneurosis, but there the anxiety is a secondary phenomenon or the consequence of other disorders of the psyche. Freud has isolated a clinical picture in which all the components of the symptom-complex are grouped around the main symptom of anxiety. He termed it "anxiety neurosis." He places it among the psychoneuroses. The symptoms are as follows. (1) General Irritability. — Auditory hyperesthesia is frequent. The latter causes insomnia. (2) Anxious Expectation. — It is associated with a pessimistic view of things. The patient interprets every occurrence as an unfavorable omen to himself. This is the most important symptom of the neurosis. The anxiety may develop even without an associated idea, such as fear of going insane or of sudden death, or else it may be connected with disturbances of the somatic functions, viz. cardiac, respiratory, vasomotor, gastro- intestinal, muscular, vesical, etc. Freud considers this syndrome as having nothing to do with neurasthenia, contrary to the custom of many who consider it only as manifestations of neurasthenia. There can be no doubt that among the multiple intermediate and unclassifiable forms of psycho- neurosis and nervousness there is a variety in which the chief symptom is "anxiety." The latter predominates in the clinical picture and every other symptom is subordinate to and dependent on the state of anxiety. Similarly to other forms of psychoneurosis Freud considers here sexual influences as the direct etiological factor. The treatment there- fore should be psychoanalysis. HYSTERIA Hysteria had been known to the most ancient writers. For a long time it was considered as the result of disturbed function of the genitalia and especially of the uterus. Only in the seventeenth century (Lepois and Sydenham) a more modern view was proposed and the cause of hysteria was placed in the brain. With the advent of the school of HYSTERIA 465 Salpetriere and Charcot at its head, the disease was given the first proper interpretation and placed upon a solid scientific basis. Since then hysteria has been considered as a well-defined morbid entity which presents its special physical and psychic stigmata. Charcot said that it was essentially a pyschic malady and this was practically accepted by all. Fig. 142 Fig. 143 Figs. 142, 143. — Hysteric Hemianesthesia. (Original.) Anaesthetic parts are shaded. Symptoms. — The clinical manifestations of hysteria are: Sensory, Motor, Psychic and Visceral. I. Sensory Disturbances. — They affect the general sensations as well as the special senses. Anaesthesia is the most frequent occurrence. It is characterized by its mobility; it disappears and reappears; it changes in its intensity under the influence of the most insignificant cause. The seat of hysterical anaesthesia presents a great variability; it may be con- fined to very small areas on the limbs or, what is more frequent, to geometrical segments of the latter, and we speak then of glove-like or stocking-like anaesthesia. Finally an entire half of the body may be affected and we say hemianaesthesia. Among other regions in which the loss of sensations is frequently observed is the pharynx. The anaesthesia may not be absolute; we then deal with a diminished sensibility or hypaes- thesia. Loss or decreased sensation may be observed in regard to all forms of sensations — touch, pain and temperature. 3° 466 HYSTERIA Hysterical anaesthesia, generally speaking, does not inconvenience the patient and has to be looked for. Thus the patient writes, sews, takes a good hold of fine objects. The anaesthesia may be associated with or substituted by hyperes- thesia (excessive sensibility) . The latter is rarely generalized; it is usually confined to a limb, to a segment of a limb, to a very small area. It is also characterized by its mobility, disappearance and reappearance. There are certain zones (hysterogenetic) which are quite constantly found hypersesthetic, viz. the spinal column, inframammary regions, sternum Fig. 144. — Hysteric Anaesthesia. Ansesthetic parts are shaded. Fig. 145. — Hysteric Anaesthesia. and the groins. Hysterical headache is not infrequent and may assume the form of migraine or syphilitic headache by its exacerbations at night. Very frequently the pain is only a hyperesthesia of the scalp. Clavus is a characteristic hysterical pain; it is confined to a very limited area of the vertex of the head. Occipital, nuchal, intercostal, coccygeal pain may occur in hysteria. Hysterical pseudomeningitis is a well-known syndrome; general malaise, anorexia, insomnia, headache and delirium are present. What will differentiate it from true meningitis is the persistent absence of fever, also the pathological state of the cerebro-spinal fluid (see the latter) . HYSTERIA 467 Paresthesias in the form of coldness, numbness, burning, etc., are quite common in hysteria. The so-called "globus hystericus" is a well- known paresthetic phenomenon. It consists of a sensation of pressure in the throat or of a ball passing from the epigastrium to the throat. The special senses also suffer sometimes in hysteria. Taste, smell, audition and vision are not infrequently involved. Perverted hysterical taste and smell are well known. Hysterical deafness is usually unilateral. Special mention must be made of visual disturbances. Total amaurosis is rare, but contraction of the visual fields, disturbed perception of color and disturbed accom- modation are quite frequent. As to the contracted field, it is concentric in the majority of cases; it is more often bilateral than unilateral. The latter is in the ma- jority of cases on the side where sen- sory losses are most marked, as for ex- ample in case of hemiansesthesia. Visual acuity is usually intact. To this symp- Fig. 146.— Hysterical Con- tom is sometimes added disturbance of ™ A ™™ °* THE Arm " {GiUes de la 1 oureue.) color perception; it may be dyschroma- topsia or achromatopsia: the colors are either confused or not at all recognized. II. Motor Symptoms. — Paralysis and contractures are very frequent. Palsies of a segment of a limb, of an entire extremity or extremities — monoplegia, hemiplegia and paraplegia — may occur. Hysterical palsies and contractures vary considerably from the standpoint of their onset, duration and mode of disappearance. They may last but a few minutes or persist for years. Their essential feature lies in complete absence of objective signs of organic nature. In hysterical hemiplegia, for example, there is no spasticity of the muscles, no increased or abnormal reflexes. • As to contractures, they may affect only the fingers or the toes, a limb, the muscles of the face or the neck. Blepharospasm or contracture of the orbicularis palpebrarum, facial hemispasm are not very rare. A spasm of the eyelid will produce a ptosis or rather a pseudoptosis. When the head is thrown back, the ptosis disappears. The ocular muscles may similarly be affected. An insufficiency of the internal rectus is not infrequent. Diplopia is sometimes observed. A. Westphal observed pupillary rigidity with myosis when the patient concentrated his mind upon it, but the pupils reacted normally when his mind was diverted. The essential feature of hysterical facial spasm is its disappearance during sleep. Facial palsy is sometimes observed, but it affectsjonly the 468 HYSTERIA lower half of the face. It is recognized by this sign that when the affected muscles are held with the fingers and moved, no muscular relaxation is noticed, but on the contrary a certain amount of resistance is felt. Be- sides, the palsy may become ameliorated, disappear and recur almost every day. Hysterical torticollis may be due to a hysterical palsy or contracture of the muscles of the neck. In the first case the head can easily be placed • in a straight position, but it returns as soon as it is abandoned. The latter form may disappear suddenly and reappear. Contractures may also affect the joints. The hip and the knee 'are the most frequent seats of this condition. Hysterical coxalgia can be recognized by the absence of swelling and of increased local temperature, of pain when the great trochanter is percussed and b)' the absence of crepitation and subluxation. Hysterical individuals may be affected with choreiform movements or present special tremors. The former may simulate Sydenham's chorea with its irregular and arhythmical move- ments or more frequently presents rhythmical movements affecting one limb, the face or the neck and occurring at regular intervals. Sometimes the movement resembles dancing (saltatory chorea), or swimming (nata- tory chorea). One of my patients, a girl of twelve, with distinct hysterical stigmata, would suddenly jump off the chair and reproduce for one minute^ dancing movements. This came on in attacks, five or six a day, and she suffered from it eighteen months. Hysterical tremors are polymorphous in character. They may fre slow, rapid, slight or pronounced. The}' may be oscillatory or of a large amplitude. They are present when the patient is at rest and become increased by excitement. An interesting motor phenomenon occurring in hysteria is Astasia- abasia. It consists of a functional impotence in gait and station; the patient cannot stand and walk, but when seated or bed-ridden he is able to perform all movements with his limbs. Various degrees of this motor disturbance may be present, from absolute inability to preservation of some movements. III. Psychic Symptoms, (a) Suggestibility. — The most essential and characteristic feature of hysterical individuals is suggestibility. They' are easily influenced to change their thoughts, to do certain acts, to acquire certain sensations in the general sensorium or in the sphere of the special senses, to execute or to adopt certain motor phenomena. Experimental palsies, anaesthesias, contractions, modifications of the personality are the best illustration. The hysterical persons are not conscious of the suggested act, they do not understand it, they do not connect it with HYSTERIA 469 their own personality; the suggested ideas develop in them automatically without the controlling power of the will. For the same reason self- suggestion is also observed ; they have a remarkable tendency to reproduce what they have once seen or heard because of extreme sensibility and impressionableness of their psychic centers, so that real hallucinations are formed. In a case reported by me in the American Journal of the Medical Sciences, April, 1906, numerous psychic phenomena of autosuggestion developed under the influence of reading or seeing. (b) Amnesia. — Disturbance of memory is a frequent occurrence in hystericals. Variability of answers, untruthfulness, contradiction, capriciousness, inconsistencies in their mode of living and conduct are the result of temporary amnesia. The loss of memory may be: localized when events of a certain period are forgotten; systematized, when several events concerning several persons or several periods are forgotten; general, when the hysterical forgets his entire past life (see Sidis and Goodhart on Multiple Personality). A person thus affected may lose all remembrance of his previous life; he possesses a new personality. In some cases the patient passes through two or more different short periods of life as two or more personalities (double consciousness) and one is not aware of the other, so that the lives the two personalities lead may be diametrically opposite to each other. (See case of Mary Reynolds — double consciousness by Weir-Mitchell and my case reported in the American Journal of Medical Sciences, 1906.) (c) Disturbance of Speech. — Mutism occurs in hysteria. The patient is not only unable to utter words in a loud or low voice, but is also unable to emit a sound, although the movements of the lips and tongue are well preserved. Stuttering may also occur in hysteria, but it is different from the usual ^stuttering acquired in childhood. In the latter case the patient is able to make himself understood. He pronounces whole sentences, but stutters especially in pronouncing t certain letters, such as m, t, b. After the stoppage is overcome, he goes on speaking clearly until again the special letters interrupt him. More- over when the patient speaks very slowly and deliberately, the stuttering may disappear. The stuttering is particularle noticeably in rapid speech. Hysterical stuttering is present not only for special letters, but it is con- tinuous. The patient stutters at every word or syllable and during his entire conversation. The words are pronounced by him slowly as he has difficulty in emitting all verbal sounds. His entire speech is slow and the syllables are pronounced with prolonged effort. Finally hysterical stuttering occurs after a violent emotion, such as railway accident, etc. Aphasia is not frequent in hysteria. Motor and sensory aphasia 470 HYSTERIA have been observed. They presented all the typical features of genuine aphasia, except the onset, course and termination. Hysterical aphasia may occur after an emotion, imitation or suggestion. It is variable in its course, disappears and reappears and always ends in recovery. Agraphia in association with aphasia has also been recorded. It has the same characteristics as aphasia. (d) Somnambulism. Catalepsy. Lethargy. — The first consists of a sleeping state, during which the patient leaves the bed and walks around. In such a manner he is likely to walk through the window and fall down on the roof or on the street. Catalepsy is characterized by a rigidity of one or several extremities which, placed in a certain position, may remain so for hours. The patient hears what is going on around him, but unable to control himself; he is probably under the influence of a hallucination. External stimulations (cold water, electricity) frequently arouse the patient. Lethargy is characterized by a sleep which comes on suddenly and during which the patient reacts to external stimulation; there is also no relaxation of the muscles, but some rigidity; the masticator muscles are contracted, the eyelids are animated with a tremor. When the patient awakes, he has no recollection of the attack of sleep. (e) Hysterical Fainting. — With or without premonitory symptoms the patient suddenly feels fainting and falls. The eyes are half-closed. The hands are animated with slight twitchings. The face is pale, but the heart beats are normal. (/) Hysterical Paroxysms (Major Hysteria of Charcot). — They remind of epileptiform seizures, but they differ essentially from the latter. An attack is usually preceded by prodromal symptoms which are of a psychic order. The patient becomes sad, depressed or else very excitable; sometimes he has visual or auditory hallucinations. An aura is present, but it is slow in coming on; it is usually the well known "globus hystericus" (see above). Then follows the attack. The patient becomes unconscious or rather semiconscious, as in falling he rarely injures himself, and in some cases he places himself comfortably before he is seized with the convulsions. The latter are at first tonic. The entire body is tetanized: the respiration stops, the arms and legs are stretched out, the face is cyanotic. Rapidly the clonic contractions appear. Unlike those of epilepsy they are of wide range and last longer. The body assumes all sorts of attitudes. Contortions characterize this phase of the attack. Flexion or extension of the trunk and of the limbs, throwing the legs in all directions, rolling the body from one side to another, position of opisthotonos, grimaces of the face — these are the various positions rapidly succeeding each other. At the same time the patient screams, HYSTERIA 471 cries or else laughs, tears his clothing, etc. Under the influence of hallu- cinations accompanying this period the patient soon develops passionate attitudes. They are expressed in the gestures and the display of the muscles of the face. The images he sees in his dream express alternately sadness, anger, terror, joy or ecstasy. In my case referred to above the patient stood in the middle of the room in the attitude of an orator, reciting a pathetic story which she read, ges- ticulating, raising and lowering her voice, crying and laughing. Gradually the violent movements cease, relaxation takes place and the patient enters into the last period of the paroxysm which Fig. 147. — Hysterica! Paroxysm. {Charcot.) Opisthotonos. Fig. 148. — Passionate Atti- tude in a Hysterical Attack. (Gilles dc la Tourette.) is characterized by a state of delirium. He talks in a low tone of voice, speaks of a certain recent event, which has made a special impression on him. In some cases instead of a delirium the attack may terminate by an out-burst of laughing, singing or crying. Little by little and sometimes suddenly the patient becomes silent, awakens and the attack is over. The patient may feel somewhat fatigued, but he does not present the profound exhaustion following an epileptic seizure. An attack usually lasts from ten to fifteen minutes, but the periods of grand movements and of delirium may last hours. The paroxysms do not always present the typical picture just described. They may vary considerably. In some cases the convulsive movements begin suddenly without an aura. In others the delirium — as a final phase — is absent. In still others there are only tonic movements. The move- ments themselves may be prolonged or unusually brief. They may be limited to a certain limb or portion of the body. In one of my recent 472 HYSTERIA Fig. 149. — Hysterical Paroxysm. Are de Cercle. (Gilles de la Tourelte.) cases the convulsions simulated Jacksonian epilepsy, as they were confined to the right arm and leg. The hysterical paroxysms are rarely isolated. They repeat them- selves sometimes at regular intervals^upon the least emotion. Sometimes one attack follows another and this condition may last hours and days. The patient is then in status hystericus. Hysterical paroxysms may be associated with epileptic seizures. Such a combination is called hystero- epilepsy. The latter should, how- ever, not be considered as a special entity, as it is only an association of two independent neuroses, each with its character- istic symptoms. The So-called Hysterical In- sanities. — The last two stages of a typical hysterical paroxysm can be placed under this heading. The delirium and the hallucina- tions under which the patient is laboring at that time render him totally irresponsible. Hysteria may simulate various mental affec- tions. Attacks of depression or else of excitement, persecutory or other delusions associated with hallucinations may give the impression of classical psychoses. Hysterical melancholia, hysterical mania, hys- terical paranoia have been described, but they are not genuine forms of mental alienation. A careful analysis of the symptoms will show that attacks of delirium, of depression, of excitement and even periods of appa- rent mental derangement are only episodes in the life of hysterical in- dividuals; they are equivalents of ordinary hysterical paroxysms, just as psychic phenomena take sometimes the place of ordinary epileptic seizures. The course, character, duration and the consequences of the psychic manifestations are not at all those of the typical psychoses; they are all a sort of transformation of ordinary hysterical paroxysms and they all may be the result of autosuggestion. The conception of hysterical insanities as morbid entities is erroneous. IV. Visceral Disturbances. — The larynx may be the seat of various symptoms. Aphonia is not infrequent, it comes on suddenly; patient is unable to raise his voice; he only whispers. The laryngoscope reveals total absence of palsy of laryngeal muscles. Various laryngeal noises are also observed. Hiccough is a familiar phenomenon. It develops suddenly and may disappear suddenly. It occurs in attacks lasting a HYSTERIA 473 certain number of days or weeks. It is usually very loud and may resemble the barking of a dog. Hysterical cough is paroxysmal. It usually appears upon emotion and disappears when the patient's mind is diverted toward a special subject. It disappears when the patient is asleep. Hysterical dysphagia consists of an intermittent spasm of the pharynx and oesophagus. If it is continued, it will lead to inanition. The same can be said of hysterical anorexia, which may become associated with attacks of vomit- ing. These gastric disturbances may be only temporary or persistent. In the latter case they are quite serious. Tympanitis is sometimes observed; it may be partial or generalized. It may simulate pregnancy. Hysterical pseudo-peritonitis may occur. Fig. 150. — Hysteria. Symmetrical Erythematous Patches on the Chest which Developed Rapidly after a Violent Emotion. It may be accompanied by hyperesthesia of the abdomen, vomiting, constipation, but fever is absent. It may also simulate appendicitis. In one of my cases the patient was operated without my knowledge and the appendix was found absolutely normal. Shortly after the operation the hyperassthesia of the same region returned. Hysterical anuria and dysuria, also retention of urine, are occasionally observed. One of my female patients, after a series of shocks — death and diseases in the family — developed anuria which alternated with re- tention of urine. Polyuria is not an infrequent phenomenon. Sexual function may be altered. Either there is an increase or diminution of sexual appetite; sometimes there are perversions of sexual desire. Vasomotor disturbances are seen as sudden flushing or pallor of the skin, dermography. The latter consists of persistency of a red line 474 HYSTERIA on the skin when the latter is slightly irritated. Erythematous patches following a violent emotion may occur. Various spontaneous hemorrhages (independently of cases of malingering to which hysterical patients are subject) may be observed: ecchymosis, epistaxis, hematemesis, hematuria, hemoptysis (Fig. 150). They are, however, rare. The menstruation may be disturbed. Hysterical fever cannot be denied. It develops sponta- neously and comes down without any treatment. The oscillation is of very wide range. It may be present without the usual accompanying symp- toms, viz. headache, rapid pulse, thirst or burning of the skin. Hysterical fever is rare. Malingering should always be excluded. Trophic disturbances occasionally occurring in hysteria are : oedema, ulcerations, sudden falling out or graying of the hair. I have ob- served a remarkable case of gangrenous patches on the chest and arms which leave no doubt as to their hysterical nature. They developed rapidly after a fright, began with a simple erythema, persisted for a long time without spreading, but commenced to improve and finally recovered when electricity was applied a few times. Another shock occurred, again a few patches appeared and again they disappeared when electricity was resorted to. Course, Duration, Prognosis. — The above described sensory, motor psychic and visceral symptoms are not always present in every case. There are mild and severe cases. The disease usually runs a chronic course. The symptoms develop gradually under ordinary circumstances. They may, however, develop rapidly when a shock, an emotion, are the etiological factors. The essential characteristic feature of hysteria is the great variation in the manifestations. Some symptoms disappear, but may reappear upon the least emotion; others persist. The prognosis as to life is good, except in cases of marked visceral disturbances, as spasms of the larynx or dysphagia or persistent anorexia. Hysteria as a rule is a curable affection, but in some cases it may last indefinitely. The duration and the prospects of recovery depend upon the circumstances, surroundings, mode of living and the treatment. The hysterical paroxysms are difficult to combat, they are exceedingly stubborn. The contractures are also difficult to cure. In children the disease is less persistent than in adults and the usual stigmata are frequently absent. The symptoms are rather single; either mutism, or a paralysis, or a contracture, etc. Diagnosis.- — In making a diagnosis of hysteria, first of all organic diseases should be eliminated. Hysterical palsies, for example, may simulate hemiplegia or monoplegia. The following are the differential signs of the two forms of paralysis. HYSTERIA 475 Organic Hemiplegia i. Spasticity of the affected limbs. 2. Tendon reflexes abolished im mediately after the onset, but later exaggerated. 3. Babinski sign present. 4. Oppenheim's and paradoxical reflexes present. 5. Ankle-clonus present. 6. Voluntary movements on the paralyzed side are abolished. 7. Abdominal and cremasteric re- flexes are usually, esepcially at the beginning, diminished or abolished. 8. The course is regular: spas- ticity follows flaccidity; im- provement is gradual. Hoover's "opposition move- ment" phenomenon is present. (When the patient makes an effort to raise the paralyzed leg, there is a very energetic "opposition movement" in the other leg; a hand placed between the heel of the sound foot and the bed will appre- ciate the degree of force dis- played during the opposition movement). Hysterical Hemiplegia 1. Flaccidity of the affected limbs. If some rigidity is present, a volun- tary resistance during passive movements is felt by the hand of the examiner, showing therefore the patient's ability to oppose the movements. In organic hemi- plegia the patient is unable to resist. 2. Tendon reflexes intact. 3. Babinski sign absent. 4. Oppenheim's and paradoxical reflexes absent. 5. Ankle-clonus absent. 6. Voluntary movements on the paralyzed side are not totally abolished. 7. Abdominal and cremasteric re- flexes are normal. The course is irregular: flac- cidity or some rigidity remains; the disturbances may change from time to time, become aggravated or ameliorated or else last a very short time. Hoover's "opposition move- ment" phenomenon absent. A hand placed between the heel of the sound foot and bed will fail to find the counter- pressure of the latter. 476 HYSTERIA Sicard has recently called attention to the following phenomenon. If an elastic bandage is applied tightly to the paralyzed and non-paralyzed symmetrical limb at equal distances from the periphery (usually in the middle of the limb) , it is observed that in functional cases the vaso-motor disturbance will be equal on both sides, but unequal in organic cases. For other additional reflexes observed in organic and absent in hyster- ical hemiplegia see pages 92, 93, 94. Hysterical paraplegia will be recognized by the integrity of the reflexes, absence of Babinski's, Oppenheim's and paradoxical signs or ankle-clonus. Hysterical contractures are usually sudden in onset, disappear under suggestion and under general anaesthesia. Hysterical paroxysms (see above) are differentiated from epileptic seizures by the following signs. Epilepsy Hysteria 1. Onset abrupt. Patient drops 1. Onset after an emotion. Patient unconscious. feels the oncoming attack and takes measures not to fall. Semi-consciousness. 2. Patient bites his tongue. 2. Patient does not bite the tongue. 3. Involuntary micturition or de- 3. Xo involuntary micturition or fecation. defecation. 4. Convulsive movements of 4. Convulsive movements of wide small range. range. 5. Pallor of the face after a seizure. 5. No pallor of the face. 6. Exhaustion and profound sleep 6. Either immediate return to usual after a seizure; also some occupation or a superficial sleep mental hebetude for hours after an attack. Mentality following. clear. Sensory disturbances are observed in organic diseases of the nervous system as well as in hysteria. The characteristic features of hysterical anaesthesia was sufficiently described in the symptomatology (anaesthesia of segmentary or hemiplegic form). Hysterical neuralgia will be recognized by the absence of tenderness of the nerve-trunks characteristic of true neuralgia. Hysterical headache may sometimes simulate headache caused by a cerebral tumor, but the mental condition accompanying the latter dullness, apathy, etc.) in addition to other objective symptoms will reveal the true nature of the headache. Besides, the failure of pain to affect the general condition of the patient is strongly in favor of hysteria. Pain caused by an organic disease, such as deep-seated tumors and other affec- tions, produces usually a reaction on the general health. HYSTERIA 477 Etiology. — The disease is very frequent. It occurs not only in adults, but also in children and aged individuals. Both sexes are subject to it. The causes are predisposing and exciting. Predisposing. — Heredity plays an important role. Functional nervous diseases, insanities, alcoholism, tuberculosis, syphilis in the parents, are predisposing factors in the offspring. Excesses of all sorts, unhygienic mode of living, sedentary life, certain occupations accompanied by a great mental strain and anxiety are all predisposing causes for hysteria. Among the exciting causes the most common is a shock, an emotion. It frequently follows accidents. Traumatic hysteria is a very well-known condition. In such a case hysteria is due exclusively to the fright and to the psychic shock. In the chapter on traumatic neuroses the subject will be considered in detail. In the chapter on "Psychic Symptoms" it was mentioned that the chief characteristic of hysteria is "suggestibility." Suggestion therefore is also one of the causes of hysteria. It may be in- duced sometimes by the examining physician, if he is not careful in his procedures. In examining, for example, for sensory disturbances, care must be taken not to speak to the patient of the desired element of investi- gation. An excellent precaution is to have the patient blindfolded during the examination and not to ask any questions of her while you test with a pin various areas of the skin. There is no doubt that she will make some defense movement if the prick causes pain. Or else blindfolded she may be asked to say ''yes" the moment she feels pain or the touch of an object. Moreover she should never be spoken to of possibilities of loss of power in one or more limbs or segment of a limb, or loss of memory, of a grave outlook, etc., etc. The patient may be suggested various disturbances of function in the motor, sensory, vaso-motor, vegetative and psychic spheres with great facility. Protracted diseases of any nature may be a cause of hysteria. Intoxi- cations (alcohol, lead, mercury, morphin, C0 2 ) are also among the exciting causes of hysteria. Hypnotism, especially when repeated, develops hysteria. The provocative factors of hysteria are therefore multiple. However it should be borne in mind that this malady can develop only in individuals who are predisposed to it. A special morbid basis is essen- tial : a physical or mental shock will have no special effect on an individual free from hereditary taint, but will disturb considerably the nervous sys- tem of a person whose make-up is morbid through heredity or otherwise. Nature of Hysteria. — At the present time two views are held concern- ing the nature of hysterical phenomena, viz. the so-called psychological and physiological. According to the first, hysteria is a psychic disease. Charcot, Moebius, 47§ HYSTERIA Strumpell, Bernheim and Janet see in hysterical manifestations either disturbance of the faculty of mental representation or autosuggestion (see above), or else double or multiple personality; finally a narrowing of the field of conscience. Anaesthesia, the most important according to Charcot of all stigmata, may be explained by Janet's "restrictions of the field of consciousness." The hysterical patient perceives only a small number of sensations and gradually they may entirely disappear and he thus becomes anaesthetic. For the same reason memories of movements may be lost, hence hysterical paralysis. In astasia-abasia, for example, the field of consciousness is so limited that the patient loses the association of mental operation only for standing or walking, but for no other act. Hysterical paralysis is sometimes due to a suggestion. The patient adopts, for example, the idea of not being able to walk. This idea may re- main in the subconscious state without the patient's knowing it. Fixed idea is a very important element in hysteria. It plays a predominant role in the fife of the hysterical individual : other ideas and experiences have no restraining influence on the fixed idea. Consequently suggestion is a very potent factor when ideas are forced on a hysterical person by another person. Such is the case in hypnotism. There is introduced into the patient's mind the idea of the hypnotizer's personality, which finally becomes predominating. It is therefore evident that what character- izes especially the hysterical individual is the want of mental synthesis, the inability of grouping individual ideas. He will attach himself to one fixed idea and the others will remain in a subconscious state. Hyster- ical paroxysms cannot be totally explained by the conception of "restric- tion of the field of consciousness." It is true that a paroxysm is the result of the workings of some predominating idea which was brought on by other secondary ideas of past experiences. Nevertheless during a hyster- ical attack consciousness has disappeared and consequently restriction of the field of consciousness is not altogether applicable here. According to the physiological theory advanced by Sollier, the hyster- ical stigmata or paroxysms are of purely physical nature. Here the primary disorder is a functional disturbance of the entire brain or only of some of its centers. The sensory, motor, psychic, trophic, visceral and circulatory phenomena will depend upon the centers involved. An in- hibition of the latter produces the corresponding symptoms. In the chapter on Psychoanalysis (page 462) the ideas of Breuer and Freud on the origin of Psychoneuroses were discussed in detail. These two authors consider also hysteria as a form of psychoneurosis. They argue that the various physical manifestations of hysteria are only substitutions for the mental disturbances, stress and unpleasantness (to all HYSTERIA 479 of which they give the name "affect") created by past experiences. The latter are never annulled but remain in the subconscious mind. The distress produced by these latent thoughts which are continuously at work, must be relieved and this is frequently done in hysteria through some physical occurrence as a substitute. Breuer and Freud state that this power of "conversion" of mental disturbances into physical manifes- tations is characteristic of hysteria. When the conversion does not take place, the result will be evidenced in various phenomena characteristic of psychoneuroses such as obsessions, phobias, etc. (see Psychasthenia.) By reason of the efforts of the patient to relegate the painful ideas into subconsciousness, Breuer and Freud call all psychoneuroses as "defense- neurosis." In all psychoneuroses they find that the repressed disagree- able thoughts are always of sexual nature. At some time during the patient's life even in infancy a sexual shock of some kind occurred. The contention as to hysterical phenomena being the result of sexual ex- periences, hidden in the subconscious world is not accepted by all. The psycho-analytic method of treatment of hysteria is based on bringing to light all concealed and repressed ideas and thus have them asimilated by the conscious ego (see chapter on Psychoanalysis). Recently Babinski {Semaine Medicale, 1909) proposed a new revision of Hysteria, from the symptoms of which he removed some manifestations which had been and are still considered by many as belonging to the great neurosis. Dismembered in this manner Hysteria is called by Babinski "pithiatisme." In the first place he believes that in a great many cases the phenomena so-called hysterical are the result of malingering. In the next place a number of symptoms which are not brought on by suggestion are fictitious, for example : fever and anuria. In the third place symptoms such as erythema, ecchymosis, ulcerations, gangrene, oedema — are in- tentionally brought on; they are fraudulent, they are the result of local applications used by the patient for the purpose of arising sympathy or otherwise. Babinski believes that genuine pithiatic symptoms are those which can be produced and removed by suggestion. Suggestion thus under- stood implies the idea of the will being the master of the entire situation. Babinski does not believe that emotion is the cause of hysteria. It is a factor totally different from suggestion. Finally he removes from hysteria also exaggeration of tendon reflexes and vaso-motor disturbances of the skin. To sum up: Babinski considers the phenomena of hysteria or pithia- tisme depending essentially on the psychic surroundings or on the predisposition of the patient. They are the result of suggestion or auto- 480 HYSTERIA suggestion, they are subordinate to the will of the patient. Their muta- bility is characteristic. Babinski's views as to limitation of phenomena belonging to hysteria are too radical. While it is true that simulation (conscious or unconscious) is met with in a number of cases of circulatory, secretory or trophic dis- turbances, nevertheless observations reported by competent men prove conclusively that hysteria per se is capable to produce these disturbances. Treatment. — It was mentioned above that hysteria develops usually in individuals with a morbid hereditary history. A tendency to hysteria or to other nervous affections is therefore great. Consequently prophy- laxy should play a considerable role in treatment of hysteria. Nothing should be neglected in building up the body. General hygiene, hydro- therapy (cold or warm water — according to the tolerance), massage, moderate outdoor exercise, good nutritious food are the general measures. As these individuals are very impressionable, special care must be taken in selecting occupations. The latter must be free from emotional incidents as far as possible. The entire life of hystericals must be so regulated as to avoid shocks, emotions of any nature. As they show a tendency to suggestion and autosuggestion, they must avoid emotional and pathetic scenes, and read only books free from fantastic and terrifying descriptions. The meals, sleep and healthful recreation must be regulated and strictly adhered to. Stimulants, including tea and coffee, are forbidden. Smoking should be avoided as much as possible. Sexual intercourse must be extremely moderate. The summer months should be spent in a quiet place, free from undue excitement, although some pleasurable entertain- ment is permissible. It is also advisable to take short vacations during the working months of the year. By all these preventive measures, applied at an early age of life, the patient will grow up physically and mentally strong and his resistant power to shocks or violent emotions will be thus greatly increased. The treatment of hysterical manifestations is largely psychic. In presence of localized palsies, contractures, anaesthesias, globus hystericus, aphonia, paroxysms, etc., all symptoms of psychic nature, the physician must endeavor to convince the patient that the trouble is not serious, is curable and that by strict adherence to the rules of the treatment he will make a complete recovery. This suggestion and persuasion (psycho- therapy) must be repeated frequently and its manner must be modified according to the variations of the patient's condition. It must be done carefully and tactfully. By doing so the patient will gain confidence and a new orientation of the mental processes will thus be brought about with HYSTERIA 481 the greatest facility. All that was said of the psychotherapeutic procedures in the chapter on Psychasthenia is applicable to Hysteria. As to Psychoanalysis, it was mentioned that Freud considers Hysteria as a psychoneurosis and therefore the psychoanalytical method finds its proper place in treatment of Hysteria. It is perfectly legitimate to employ some physical means in order to reinforce the ordinary suggestion. For example, a mild electrical current applied to the neck in case of aphonia or globus hystericus, to the muscles of a contractured segment of a limb may give excellent results. In such cases the electricity does not possess a special curvative power, but acts as a suggestive agent. Simple manipulations of the affected region when administered regu- larly, so as to impress the patient as if it were a necessary procedure, may yield some results. Hysterical backache, headache, neuralgia of long standing have been cured by application of liniments, water-bags, etc. Hypnosis, which at a certain period reigned in treatment of functional nervous diseases, should never be employed. Hypnotic sleep exaggerates the suggestibility of the individual and practically induces hysteria and consequently aggravates the preexisting hysterical state. It should be totally abandoned. When the hysterical phenomena are marked, isola- tion should be practised in conjunction with suggestive treatment. Removal of the patients from their usual surroundings has given me excellent results. The patients gradually get accustomed to their new life, become obedient and fall entirely under the physician's control. This method gives the patient a simple, quiet and regular life; all sources of irritation are thus removed. In cases of marked emotionality, restless- ness, tremor and spasms, rest in bed is very beneficial. Massage, hydro- therapy will then be excellent adjuvants in preventing wasting and sluggish digestive function (Weir-Mitchell's plan). Medications will be administered only in cases of special indications. Restlessness, undue emotionality (crying, laughing, etc.) can be combated by bromides and tepid baths. For insomnia small doses of veronal or bromides can be given. When the patient gets accustomed to the drug and expects his capsule at night, veronal should be substituted by some placebo, as bicarbonate of soda, or plain flour. Gastro-intestinal dis- turbances should be treated accordingly. A hysterical paroxysm may be prevented by sprinkling cold water on the face or by inhalation of a few drops of ether. In one of my female patients I succeeded in checking a paroxysm by an application of a faradic current to the left hand. In another of my male cases the patient regained consciousness after a slight pressure was exercised upon the left hypochondrium. 31 482 EPILEPSY Speaking generally, very little medication is necessary in treatment of hysteria. To sum up, rest, tonifying measures, isolation, suggestion in a waking state (not hypnosis), persistent efforts to remove fixed ideas, which are so characteristic of the disease by persuasion or psychoanalysis — this is the main treatment of hysteria. EPILEPSY Epilepsy is characterized by a sudden loss of consciousness with (or without) convulsions. Forms. — • I. Focal or Jacksonian Epilepsy. II. Major Epilepsy (Grand Mai). III. Minor Epilepsy (Petit Mai). IV. Equivalents of Epileptic Attacks. I. Focal or Jacksonian Epilepsy See description in Diseases of the Brain. II. Major Epilepsy (Grand Mai) Essential or Idiopathic Epilepsy. Symptoms. — The disease is essentially paroxysmal. A seizure is usually preceded by prodromal symptoms or aurae (warn- ings). The latter may be of long or short duration, may be of motor, sensory, psychic, vaso-motor or visceral nature. A motor aura consists of tremor, of twitching affecting one or several muscular groups, vertigo, of automatic movements, as running, striking, etc. A sensory aura may present itself as an epigastric pain, headache, sensation of cold or heat, of numbness, of tingling (usually in the limb in which the convulsion will commence), sensation of a vapor. Olfactory and gustatory, visual and auditory disturbances may also constitute an aura. The "dreamy state" described by H. Jackson as uncinate fits is accom- panied by a smacking of the lips which suggests a gustatory phenomenon. "Sudden darkness" is a frequent visual aura, special colors, images may also appear. Sounds of a whistle, bell or of a voice — are the auditory aurae. A psychic aura is particularly interesting, as it shows the role of the brain in the causation of epileptic seizures. It may consist of a delirious state, of a depression or else of gaiety, of terror, of sudden appearance of old images, etc. EPILEPSY 483 Vasomotor aura presents itself as a sudden pallor or else redness of the face, perspiration. Visceral aurae are: cardiac pain, palpitation, dyspnoea, laryngeal spasm, nausea, vomiting, cramps. Epigastric aura is one of the most common. It may be a pain associated with nausea, which rapidly ascends to the throat or to the head and is followed by loss of consciousness. Cephalic aura consists of a sensation of "fulness" or "rush of blood" in the head. It is accompanied by giddiness. Aurse do not always pre- cede an attack. The latter may come on suddenly without the least pre- monitory symptoms. In exceptional cases an attack may be prevented or checked at the very onset by certain manipulations as, for example, by tightly constricting the wrist. In such cases the patient carries a small girdle on the wrist with a string attached to it. As soon as an aura appears, the patient immediately pulls at it; constriction is then produced. One of my patients could inhibit an attack by putting some food in his mouth as soon as his aura (pain in the epigastrium) appeared. It may be arrested also by strong sensory impressions, such as smelling strong odors, or a splash of cold water on the face. Sometimes inhalation of amyl- nitrite is sufficient. The seizure itself is characterized by the following symptoms. The patient becomes pale, screams and falls unconscious. Immedi- ately tonic convulsions appear. The entire body becomes rigid. The head is turned backward or to one side. The arms and legs are extended, the hands are closed and the thumbs adducted. The trunk is immobile. The eyeballs are bulging and turned, the pupils dilated. The face, which was pale at the beginning, becomes bluish and bloated. The tongue if protruded between the tight jaws bleeds. Involuntary evacuation of urine frequently occurs, probably because of a convulsive spasm of the walls of the bladder. The disturbance of circulation (venous stasis) pro- duces sometimes ecchymoses and epistaxis. The duration of the tetanic phase is about hah a minute. The following phase, which lasts from a half to several minutes, is characterized by clonic convulsions. Here the rigidity is replaced by convulsive seizures. The latter rapidly follow each other, are abrupt and irregular. The muscles of the entire body twitch. The head turns from side to side, the grimaces of the face are frightful. The cyanosis disappears as the breathing becomes less difficult. Saliva froths at the mouth. The alternative closure and opening of the jaws cause the tongue to be bitten. The eyeballs turn rapidly in all directions. The body is covered with perspiration. The muscular contractions of the limbs may be so violent that dislocations occur. Gradually the twitchings subside and the patient, 484 EPILEPSY exhausted, enters the third stage of the paroxysms. In the majority of cases he falls asleep. The sleep is profound and may last several h|ours. Awakened he has no recollection of his attack. Sometimes the amnesia covers a period of several hours preceding the attack (retrograde amnesia). Sleep does not always terminate an attack. The patient then opens his eyes, is confused and remains so for some time. In exceptional cases the patient in sleep turns on his face and as the sleep is very deep, asphyxia may occur. Certain objective phenomena usually accompany or follow an epi- leptic seizure. The temperature rises during an attack, but not above 0.5 . The pupils are dilated during the tetanic phase, but myotic in the subsequent phase. The pupillary reflex is abolished. The tendon and cutaneous reflexes are exaggerated. I have observed frequently increased knee-jerks, also sometimes ankle-clonus and Babinski sign, but paradoxical reflex is met more frequently than the latter. This state of tendon reaction is continued for some time after the attack. Relaxation of anal sphincter is frequent during a seizure. Paralytic phenomena sometimes follow an attack. They are the result of cerebral exhaustion. Hemiplegia, monoplegia, asphasia may occur, but they are usually of a transient nature. Concentric contraction of the visual field and ocular palsies are occasionally observed. The general nutrition usually suffers, especially when the attacks are frequent. Phos- phaturia and albuminuria are not infrequent. The toxicity of urine is diminished before and during the attack, but increased after an attack. The toxicity of the blood serum is increased during an attack and the toxicity of the cerebro-spinal fluid is increased after an attack. The mental depression following an epileptic seizure presents varia- tions from a slight hebetude to stupor. It increases in intensity when the attacks are frequent. In the latter case a feebleness of the mental faculties develops gradually and if the attacks are not kept under control, a true and permanent dementia will ensue. In children frequently repeated epileptic seizures will gravely interfere with the intellectual development and mental arrest will be the result. Status Epilepticus. — Under this term is understood a series of attacks which rapidly follow each other without the patient regaining conscious- ness. This form of epilepsy is very grave. Death may occur during the convulsive period or during the stage of stupor. However the condition is not invariably fatal. When the intervals between indi- vidual series of attacks increase, the patient may regain consciousness and improve. EPILEPSY 485 Atypical Epileptic Seizures. — An epileptic attack as described above does not always present itself in its typical form. It varies in different individuals and even in the same individual. The phase of tonic convul- sions, for example, may be extremely brief and this phase may represent the entire attack. In other cases the clonic phase may be unusually prolonged even as long as 15 minutes. The clonic contractions may be of wider range than in typical epilepsy and they may give the impression of a hysterical paroxysm; they are therefore called "hysteroid." They usually alternate with typical epileptic convulsions, but when they do not, the diagnosis between hysteria and epilepsy is very difficult and remains necessarily in suspense until a classical attack occurs. There are cases in which clonic convulsions may not affect the entire body. When the tonic phase alone exists, the convulsive movements pass into muscular relaxation without the clonic spasms. It is a serious condition : symptoms of asphyxia are marked toward the end of the attack. The rigidity of various muscles is quite pronounced: tetanic contraction of the jaws and opisthotonos may be observed. In some rare cases the attack may consist only of a sudden loss of consciousness with falling but without convulsions. This is the so-called apoplectic form of epilepsy. Usually such attacks alternate with convulsive attacks. In spite of the fact that loss of consciousness and inability to recall the attack are characteristic of epilepsy, nevertheless there are caess 1 in which the individual is conscious of what is going on during an attack but forgets after the attack is over, also are there cases in which con- sciousness is preserved as well as memory after the attack. I have seen cases in which patients witnessed the attacks. Such cases may be taken for hysteria if they do not alternate with typical seizures. They are called hysteroid. There are other atypical occurrences among varieties of epilepsy. The initial cry and the biting of the tongue may be absent. In some cases the seizures occur only at night and therefore are not recognized until an occasional bleeding of the tongue or an involuntary micturition attracts attention. The deviations from the regular type of epilepsy may be multiple, but what characterizes all the typical varieties of epilepsy is the total inability of the patient to realize what occurred during an attack when conscious- ness is regained. m. Minor Epilepsy (Petit Mai) It is characterized by a sudden loss of consciousness lasting but a few seconds. In the midst of a conversation, of reading, of playing an in- 486 EPILEPSY strument, of eating, etc., the patient suddenly becomes pale, interrupts the act he is doing and loses consciousness. In a few seconds the attack is over and he resumes his work. Like in major epilepsy there is total amnesia of the attack after consciousness is regained. The attack is usually so brief that the patient does not fall. In a great many cases some sen- sation precedes the attack, such as sudden light or appearance of colors. Petit mal may present itself in various forms. In some patients an attack is noticed from sudden staring of the eyes. Others are taken with a sudden vertigo of a very short duration. One of my patients would be taken several times a day with a sudden inability to realize where he was. IV. Epileptic Equivalents They consist of motor, sensory, visceral and psychic manifestations which are sudden in onset and accompanied by total loss of consciousness. To this group belongs the so-called ambulatory form of epilepsy. The patient thus affected usually without and exceptionally with a few pre- monitory symptoms, as headache, depression, etc., suddenly leaves his home and automatically wanders away; sometimes he gets on the train and travels hours or days. Suddenly he realizes his situation and is astonished to find himself removed from his home. Similarly to genuine epilepsy there is total amnesia of the accomplished act. In another form of the disease there is an irresistible desire for sleep. It comes on suddenly and in attacks. It may terminate with a mild delirium. Equivalents of epilepsy are also seen in a sudden irresistible desire to do unusual acts. One of my patients had attacks in which he would suddenly undress himself. Another, a clerk, would suddenly unbutton his trousers in his office and urinate. Various subjective sensations or hallucinations, visceral disturbances, as pain in the abdomen, sudden desire, for defecation, attacks of angina pectoris, of migraine, of syncope, of asthma, may be considered as equiva- lents of epilepsy when their onset is sudden, their duration is short and when there are no organic visceral changes that could account for the disturbance. The so-called psychic epilepsy belongs to the same group. The pa- tient thus affected is suddenly taken with a desire to do the most absurd and impossible acts. Sometimes he attacks, injures or kills. Not in- frequently he is prompted in his actions by various auditory or visual hallucinations. The seizure terminates as suddenly as it began. Some- times it is followed by a state of exhaustion or depression. In some cases the onset of the attack is preceded by headache, undue irritability, depres- EPILEPSY 487 sion. The characteristic feature of the seizure is the complete in- ability of the patient to recall a single act committed by him during the attack. Pavor Nocturnus or Night Terrors. — In adults the condition occurs in the beginning of sleep. Suddenly the body is seized with a very pro- nounced jerk and the patient at once sits up. He wakes up, is frightened and the heart beats rapidly. Considering the fact that individuals suffer- ing from this manifestation are usually epileptics, the relation of it to epilepsy becomes evident. In children the same sudden onset with the state of anxiety is present but in addition to it there are also frightful hallucinations with amnesia. The latter manifestations are assuredly analogous to some equivalents of epilepsy mentioned above. There are cases on record pointing to the fact that night terrors are in reality attacks of petit mal, also that they eventually are substituted by genuine attacks of epilepsy. Epileptic Character. — Epileptic individuals very frequently present, certain peculiarities of character which deserve to be mentioned. Irrit- ability, quite pronounced, is characteristic. Anger is brought out upon the least provocation. With it maliciousness is frequently exhibited even toward his own people. Obstinacy is another feature; it is quite pronounced. It frequently accompanies a morbid egotism. This leads to excessive impulsiveness in actions and words ; it is exhibited even in the most insignificant occurrences. The obstinacy naturally shows deficient judgment and illogicality. Moral sentiments are also at fault. The morbid egotism mentioned above develops an unforgiving spirit toward those who happened to injure them even slightly. A certain revenge- fulness and even cruelty are not infrequently noticed in the epileptics in their relation to others. Course, Termination, Prognosis. — There are great variations in the course and duration of epilepsy. Some patients may have several seizures daily, others only one attack in weeks or months; some only during the night, others during the day. Petit mal attacks occur only during waking hours. Some epileptics have only attacks of grand mal, others only of petit mal and still others have both forms. Intervening diseases some- times arrest the seizures. Gastro-intestinal disturbances have a consider- able influence upon the frequency of the attacks. Constipation is fre- quently followed by seizures. Use of stimulants and excesses of any kind increase the intensity of individual attacks as well as their frequency. Menstruation may have some relation to attacks of epilepsy. In a certain group of cases the seizures occur only around the time of men- struating periods. Perhaps the disturbance in the internal secretion of the 488 EPILEPSY ovaries plays a certain direct or indirect role on the cerebral irritation thus producing convulsions. Oncoming puberty, which ordinarily is associated with physiological disturbances in the organism, usually increases the severity or frequency of epileptic seizures. Sometimes epilepsy makes its first appearance at puberty. Not infrequently the disease begins with attacks of petit mal which gradually may pass into major seizures. Sometimes both forms coexist. When the disease commences early in infancy, it has a slight tendency to disappear at the age of ten. The same tendency is also observed in middle life. Life is not directly threatened by epilepsy, except when injuries occur in falling. Status epilepticus is very dangerous to life. In the majority of cases epilepsy is not curable. Recoveries follow very occasionally. The disease may last many years. Petit mal is more obstinate and less easily influenced by treatment than grand mal. Mental failure frequently accompanies the advanced cases of epilepsy and in such cases dementia is the ultimate outcome. Epilepsy beginning in early life interferes with the mental development and imbecility is frequent. Alcoholic and syphilitic epilepsy are sometimes amenable to treatment and present a favorable prognosis. Diagnosis. — In the majority of cases the individual phenomena of an epileptic seizure are so typical that there is no difficulty in making a diag- nosis. In the atypical or irregular forms (see above) the symptoms may simulate other affections. A hysterical paroxysm is recognized by the following symptoms. It never occurs at night during sleep. Its onset is not sudden and not accom- panied by an initial cry. There is no biting of the tongue and no involun- tary loss of urine or feces. The muscular twitchings are of wide range, and they possess a "purposive" character. The pupillary reflexes are intact. The attack does not terminate by stupor or coma, but by an outbreak of laughing or crying. If sleep occurs, it is superficial and not as profound as in epilepsy. Mental failure or dementia, which is observed in epilepsy of long standing, is not present in hysteria. Hysterical attacks are amenable to treatment sometimes by suggestion. Uremic convulsion occurs without an initial cry or without an aura. There is no biting of the tongue. The urinary examination will show quantitative and qualitative changes characteristic of diseases of the kidneys. Epileptiform convulsions may occur in cases with increased intra- cranial pressure, as tumors, meningitis, etc. They may also occur in EPILEPSY 489 the course of paresis. It is therefore important to eliminate organic diseases in every case presenting a history of convulsions. Attacks of petit mal present no diagnostic difficulty from their sudden onset, sudden termination, extremely brief and absolute loss of con- sciousness. The psychic form of epilepsy presents at times great difficulties, as irresistible impulses are observed in the course of psychoses accompanied by delusions and hallucinations. The diagnosis will be made by exclusion. The equivalents of epilepsy (see above), as attacks of vertigo, syncope, angina pectoris, etc., will be easily recognized by eliminating visceral diseases (labyrinth, heart, etc.). In aural vertigo there is no loss of consciousness. In syncope which usually follows an emotion or occurs in an overheated room loss of consciousness takes place, but when the latter is regained, it is perfect from the beginning. The same is not observed in epilepsy. Gowers believes that repeated attacks of syncope which are due to cardiovascular changes with alteration in the cerebral circulation produce a lessened resistance of the nervous mechanism of the cardio- vascular apparatus, the reflex becomes easier and loss of consciousness more sudden. He believes that a change in nerve elements of the brain occurs in syncope and by virtue of frequent repetition of the attacks, the state of nerve elements acquires a tendency to spontaneous development which is characteristic of petit mal. Thus an ordinary faint or syncope may eventually develop into minor epilepsy. Pathogenesis of Epilepsy. — Post-mortem examinations have given nothing definite as to a possible organic cause of essential epilepsy. In spite of this fact experimental investigations (Fritch, Hitzig, Charcot, Francois-Frank and others) have shown that convulsions are essentially a cortical phenomenon. An irritation of any area of the cerebral cortex, but particularly of the motor area, produces convulsive seizures. Francois- Frank has also shown that cortical irritation is followed by vascular spasm, by changes in cardiac rhythm, by dilatation of pupils, by incontinence of urine and feces; otherwise speaking, by the symptom-group observed during an epileptic seizure. It is now generally conceded that the point of departure of a con- vulsive attack or of petit mal attack lies in the cortex, which at that moment undergoes an undue irritation. Various factors are supposed to cause this irritation. Anaemia, hyperemia of the brain, toxic sub- stances originating in the organism (autointoxication) or introduced from without (alcohol, etc.) are considered the immediate exciting factors capable of producing a cortical discharge in the form of grand mal, petit mal or equivalent of epilepsy. 490 ' EPILEPSY In favor of the cortical origin of epilepsy speaks also the fact that mental feebleness and dementia develop in the course of the disease. The toxic nature of essential epilepsy is corroborated by the influence of gastro-intestinal disturbances upon the intensity and frequency of its manifestations, by the degree of toxicity of the urine and of the cerebro- spinal fluid before and after the attacks. Not infrequently at autopsy of epileptics old meningeal or cortico-meningeal lesions are found. Such in- dividuals may have had attacks in their younger years which gradually dis- disappeared or else occurred since at very rare intervals. Recurrences in such cases take place a. propos of an autointoxication (gastro-intestinal, renal, glandular). Experiments on animals show that an intoxication which is without an effect on a normal animal is a cause of epileptic seizures in an animal presenting old cortico-meningeal lesions (Claude and Lejonne, Comptes-rendus de Soc. de Biologie, 1910). Guidi (Rivista sperim. di Friniatria, 1908), has also shown experimentally that there are profound metabolic changes in epileptics: transformation of proteids is not like in normal individuals; the most manifest deviation is seen in the production of urea, hence an acid intoxication takes place; excess of ammoniacal compounds is found in the urine of epileptics. Removal of these chemical compounds by an appropriate diet reduces the number and intensity of seizures. Etiology. — The causes of epilepsy are predisposing and exciting. A neuropathic heredity (epilepsy, other neuroses, insanity in the family), consanguinity, hereditary alcoholism, syphilis, lead poisoning, are all predisposing factors. The influence of heredity is considerable. The exciting causes are : infections, intoxications, traumata. Among infectious diseases scarlet fever, typhoid fever, measles and smallpox play a con- siderable role. Gastro-intestinal disturbances are a common cause for epileptic convulsions. Alcohol taken in large doses and for a prolonged period of time is a potent factor in epilepsy. It is possible, however, that arterial degeneration with the rise of blood pressure are apt to produce cortical irritation and therefore epileptiform attacks in aged individuals (Senile Epilepsy). Among other toxic elements capable of causing epilepsy can be mentioned: lead, mercury, cocain, morphin, chloroform, ether. Syphilis may produce epilepsy without definite anatomical lesions, especially during the secondary period. Gout, diabetes, ansemia, cardiac diseases and circulatory disturbances in general are also considered as causes of epilepsy, but their relation to the disease is not entirely elucidated. Alcohol may be an exciting cause for attacks in an epileptic individual or per se be a factor in development of EPILEPSY 491 epilepsy in a neuropathic individual. In chronic alcoholic persons who used alcohol to excess for a very prolonged period, withdrawal of alco- hol is likely to bring on epileptic seizures. This is observed particularly in asylums. Traumata may cause epilepsy either through direct injury of cerebral tissue or only in cases of concussion without apparent lesions. In considering trauma as a cause it should be borne in mind that the symptoms of epilepsy may appear shortly after the accident or a long time later, as late as ten, fifteen or twenty years. Peripheral injuries, viz. of an eye, ear, nasal fossae, of a nerve, may be the direct cause of convulsive seizures. The relation of epilepsy to visceral diseases, as uterus, intestinal worms, polyps in the nose or ear, errors of refraction, etc., is maintained by some observers, but not definitely proven. These cases are the so- called "Reflex Epilepsy." A large number of cases date from infancy. Infantile convulsions or petit mal occur frequently during the period of dentition. If they persist, they become distinctly epileptiform at puberty or even earlier. Infantile convulsions may cause permanent hemiplegia. Epilepsy may occur at any age, but in the majority of cases between the age of ten and twenty. There are cases in which epilepsy occurs only during or immediately before the menstrual periods. There are also cases in which epilepsy begins only at puberty. The period of meno- pause has sometimes a favorable influence on epilepsy. As a rule the disease is rare in old age, but senile epilepsy is well known. Treatment. — In the chapter on the Pathogenesis of Epilepsy cortical irritation was considered as the underlying immediate cause of the epileptic discharge. The main indications therefore are: (1) Removal of factors producing cortical excitability and (2) modification of the latter when it exists. 1 . The first is by far the most important. An effort must be made to place the patient in such conditions as to reduce to a minimum auto- intoxication. This can be accomplished by an appropriate regime and hygienic rules. It has been my experience that removal of meat from the diet is very beneficial. Starchy food and sweets should be avoided or at least considerably reduced in quantity. The patient should be instructed to have his meals at regular hours and not to take food between the meals. The latter should consist of milk, soft-boiled eggs, vegetables, fruit. These articles can be combined in such a manner as to suit the patient's taste and afford variations in the meals. Stimulants, also tea and coffee, should be avoided. The amount of food for each meal should not be abundant. The mastication must be thorough and the eating should be slow. 49 2 EPILEPSY As I have shown with other observers {New York Med. Jour., 1906), table salt is to be avoided entirely or taken in a very small amount. I have found that there is a decided relation between the intensity and the frequency of the attacks and the amount of table salts used with food. In order to avoid aversion for saltless food a gradual reduction of the amount of salt is recommended. Experimentally it has been shown (Paderi, Archivio di Farm, sperim. e Scienze affin., 191 1) that the effect of bromides on the organism is greater and more rapid when the diet is salt-free. Proper elimination plays a prominent role. A purgative administered regularly once a week and oftener, if there is a tendency to constipation, is a necessity. Special emphasis should be laid on regular bowel move- ment, as there is almost a direct relationship between constipation and epileptic attacks. All sources of autointoxication must be removed. The patient is also instructed to keep his mouth and teeth in as perfect condition as possible. The mode of living, occupation, sleep, etc., must be well regulated. The patient must go to bed early and get up late, so as to get a great deal of rest. An hour's sleep during the day is also desirable. He must take two or three walks a day, each of such duration as not to feel fatigued. Fatigue as a rule must be avoided. All sporty exercises are forbidden. The occupation must be of such a nature as to give a minimum of mental or physical strain. Crowded places, as theatre, church, etc., must be avoided. While complete seclusion is not advisable because of the mental depression it leads to, entertainments, society gatherings, games, etc., should be avoided as much as possible. Hydrotherapy is useful. Shower baths, warm or, better, cold, of half a minute's duration and followed by a gentle general massage — once or twice a day — are very beneficial. A sojourn in the country several times during the year is advisable. If the patient is a young boy or girl, they shouid be kept out of school. Mental work in general should be done as little as possible. Sexual intercourse should be avoided. Marriage is not to be advised, first because of the probability of sexual excesses, next in view of the offspring which, as statistics show, in the majority of cases inherit a neuroyathic tendency. Imbeciles, idiots and otherwise mentally deficient have come from parents suffering from epilepsy. This is the regime to which I usually submit my patients suffering from essential epilepsy. Careful examination should be made in regard to local irritation, as polyps of the nasal cavities, to errors of refraction, to intestinal parasites, to scars, to injured peripheral nerves, to visceral diseases. Correction or EPILEPSY 4Q3 removal of these factors should be made as early as possible in the course of the disease. Incases of toxic origin rapid elimination of toxic products must be insisted on. In cases of traumatic epilepsy a thorough search should be made for any depression of the cranium or for a localized pachymeningitis. Surgical intervention is of course necessary in such conditions. In traumatic epilepsy of long standing operative procedures should be undertaken. An effort should be made to localize the lesion. A Roentgen-ray examination should be of considerable assistance in such cases. In absence of any localizing cause on the cranium or at the periphery surgical procedures should be undertaken in idiopathic epilepsy with grea4 hesitation. When the seizures are rare and slight, also when medica- tions improve the condition, no operation should be thought of. In grave cases which are rebellious to medical treatment and which by virtue of the intensity and frequency of the attacks endanger life, surgical inter- vention may be considered. Trephining and osteoplastic operations have been performed but without permanent success. Recurrences of attacks is the rule, although amelioration has been obtained in some cases. In one of my cases I had an osteoplastic operation performed over the motor area of one side and six months later over the other side. Considerable improvement followed not only with regard to the frequency of the seizures, but also the girl (twelve years of age) began to show con- siderable mental progress. Operations on the cervical sympathetic nerve have been advised by Chipault and Jonneseo, but the results so far obtained do not apparently justify their use. Giacomelli (Gaz. degli Osped. e delle Clin., 1912) recommends for essential epilepsy intra-spinal injections of stovain. After withdrawing by lumbar puncture about 8 c.c. of cerebro-spinal fluid he injects 0.08 grm. of stovain diluted in 1 c.c. of physiological solution of chloride of sodium. In one of his cases there was complete recovery, in other cases a considerable reduction of the number of seizures and of their intensity followed. 2. Cortical excitability will as a rule be diminished by the just outlined method of general management. However in some cases the latter may not be sufficient. Medications are very frequently necessary. Bromides is the most efficient remedy according to the majority of observers and among all the salts sodium bromide is the best tolerated by the patients. Its doses are given according to the age. I have had better results in giving small doses frequently repeated than large doses once or twice a day. I usually prescribe for an adult at the beginning gr. x every three hours and at the end of three or four days every two hours. If the attacks 494 EPILEPSY are not controlled, the patient is told to take it every hour. When an improvement is noticed at the end of a few weeks, the same amount is given only every four hours. An excellent adjuvant to the bromides is one of the coal-tar products, and I always combine sodium bromide with antipyrin in gr. v doses. This treatment must be kept up for several months. If bromide acne appears, the drug must be discontinued for a few days and substituted by sulfonal or trional. The acne can be relieved considerably by administration of arsenic in two, three, or four drops of Fowler's solution. It should be continued as long as bromides are given. In the majority of my cases I persist with the bromides in spite of the acne and no special ill effects have I noticed. In severe cases the gr. x dose every two or three hours may not be sufficient. Fifteen or twenty grains may then be given. Bromides sometimes produce a marked general depression with impairment of memory, but the latter are only temporary phenomena. On the contrary, in a number of instances in my experience a prolonged administration of bromides was followed by a very marked lucidity of mind because the drug succeeded in removing the seizures for long intervals. The so-called bromide dementia has never been observed by me. When a marked intolerance is noticed (which is exceptional) bromides may be substituted by other drugs, as extract of opium in doses not above fifteen grains a day, extract of solanum carolinensis in doses of from fifteen to sixty drops, adonis vernalis, digitalis, atropine, belladonna, camphor, valerian. Any of these drugs can be given conjointly with or in place of bromides. It should be, however, borne in mind that bromides are the most efficacious of all the drugs mentioned. When a syphilitic history is present, mercurials and iodides are indicated. Iodides are also useful in cases with a history of lead poisoning. There is a certain class of epileptics which I found can be benefited by the administration of thyroid extract. These individuals show signs of hypothyroidization. I made a special study of them (Therap. Gazette, 1907) and the entire series of my patients benefited considerably from this medication. The epileptic seizures became less and less frequent and the mental condition improved to a remarkable degree. Leubuscher {Deut. Medi. Wchn. No. n, 1913) advocates adminis- tration of phosphorous in view of its good effect in tetany. He gives it in 10,000 parts oil. In his cases he obtained considerable reduction of the number of seizures. The treatment of epilepsy must be kept up in the most rigorous and persistent manner and efforts be made to control the attacks. The CHOREA 495 disease itself has a very deleterious effect upon the mental condition of the patient. In children it interferes with their intellectual growth. Mental arrest follows if the intervals between the seizures are not length- ened. When it is difficult to carry out the above outlined treatment concerning the general condition of the patient it is advisable to place him in a special institution for epileptics (colonies or state asylum). Treatment during an Epileptic Seizure.— In the chapter on sympto- matology mention was made of cases in which an attack can be cut short by quick constriction of a limb when the aura appears in that limb. I also mentioned a patient whose seizure was arrested at the very onset as soon as he put some food in his mouth. An attack can also be checked by inhalation of a few drops of amyl nitrite. If in spite of these means the attack continues, it is advisable not to interfere with it except in so far as to watch that no injury should occur to the patient when he falls. In view of the disturbed circulation, it is urgent to loosen the clothes, collars, neckties and the end of a folded towel, of handkerchief or else a piece of wood be placed between the jaws so as to avoid biting of the tongue. When sleep supervenes, the patient should not be awakened, as otherwise the post-epileptic depression increases. CHOREA (OF SYDENHAM) (St. Vitus Dance) Symptoms. — The onset may be rapid or gradual. The former follows usually a severe shock. In the majority of cases the symptoms develop gradually. A few prodromal symptoms precede the appearance of the characteristic symptoms. The patient (who is usually a child) becomes irritable, morose, inattentive. Gradually he gets restless and awkward movements are noticed in the arms and legs. Objects fall out of his hands, grimaces are noticed on his face. The characteristic choreic symptoms may begin in one leg or in one arm. They soon become generalized. The patient's musculature is continuously contracting. The movements are involuntary, irregular and incoordinate. When the upper extremity is affected, he is unable to take hold of an object and keep it for a certain length of time, is unable to feed himself, to write. In attempt to approach his hand to an object, a series of various incoherent movements will be produced before the hand reaches it. The fingers separate, approach, flex, extend. The entire limb supinates, pronates, is abducted or adducted. The shoulder is raised, lowered, pushed backward or forward. The leg is in constant motion, moves in every direction when the patient is at rest. The toes 496 • CHOREA flex, extend, the foot turns inward, outward, the legs bend or extend. When seated the patient crosses his knees, approaches or separates them. The gait is also irregular. The face is continuously agitated, so that various expressions are assumed by the patient. He closes and opens his eyes, rolls the eyes in every direction, the head rotates, the lips pout. Continuous contraction of individual muscles of the face and forehead is noticed one after another. The tongue is continuously moving from side to side, forward and backward. The speech is disturbed because of irregular contractions of the respiratory muscles, especially of the dia- phragm. The articulation of words, emission of sounds, is difficult. Ziemsen observed with the laryngoscope irregular movements of the vocal cords. When the muscles of the palate and pharynx are affected, deglutition is difficult. The muscles of the trunk and pelvis participate in the movements. To sum up, the muscles of the entire body are in a state of frequently interrupted involuntary contractions, which are rapid, irregular, pur- poseless. Voluntary movements increase the contractions, although a reverse condition may also be observed. Emotion, excitement, may increase the twitchings, but sometimes they have an inhibitory effect. In writing the following conditions may occur: (1) The power of control over the movements may be complete. (2) General choreic movements may be very slight or else much incoordination may be present. The movements usually disappear during sleep. Sensory symptoms may be present in the form of parassthesiae.and even tenderness of the muscles. Diminished objective sensibility is quite common. Chorea is frequently accompanied by the following symptoms. The reflexes may be normal, increased or diminished. W. Gordon described the following phenomenon. When, the patient being in dorsal position, a short blow is struck over the patellar tendon, the leg will at first respond like in an ordinary knee-jerk, but instead of coming down immediately after, will remain suspended in the air some time and gradually come down. It is probably due to a prolonged contraction of the quadriceps muscle. This reflex is not absolutely constant, but it has never been observed outside of genuine chorea. The pupils are often dilated. Inequality of pupils and hippus has been observed. Occasionally optic neuritis is met with, a fact which led to the view that acute chorea is of infective origin. The pulse is rapid and quite frequently a mitral lesion is present. Urea is increased. Phosphaturia is present. CHOREA 497 The mental faculties are sometimes involved. Mental dulness, diminished attention, weakness of memory, excitability or else depression are not infrequent. In exceptional cases a delirium with confusion and hallucinations may develop. In some cases the mental symptoms may become permanent and develop into dementia. The general nutrition suffers when the muscles of deglutition are involved. Forms of Chorea. — Sydenham's chorea may be pronounced and very slight. Between these extreme forms there are many intermediary forms. It may affect one side of the body and in then called hemichorea. When there is a marked weakness or a paretic condition of the extremities, it is called paralytic chorea. The loss of power and of voluntary movements in paralytic chorea may be generalized or confined to one or two extremities (mono-, hemi-, or paraplegic forms). The paralysis is flaccid and there is no involvement of reflexes, of sensations or of sphincters. The prognosis is good. In the pronounced or grave variety of chorea the twitchings are so intense that the patient is obliged to be in bed. I have seen patients whose movements were so violent that they were thrown out of bed. In such cases walking is impossible. The agitation continues even during sleep. The violent muscular contractions lead to traumata and such a patient is often covered with ulcerations. Mental disturbances frequently accompany grave chorea. Death is the usual termination. Chorea of pregnancy occurs usually in primiparas during the first half of pregnancy. It is a grave affection, as the muscular twitchings are very severe and interfere with sleep. It is frequently complicated by cardiac diseases with fever, also mental disturbances. The gravity of this form of chorea lies also in the spontaneous interruption of pregnancy. Course, Termination, Prognosis.— The average case of Sydenham's chorea lasts about one or two months. Periods of amelioration and aggravation are observed in the course of the disease. Recovery is the usual result. The younger the child the better is the prognosis. Chorea occurring in youths presents a serious outlook, as it lasts longer and has a tendency to become chronic. The grave form of chorea has an un- favorable prognosis: death is due either to exhaustion or to the mental symptoms. The more marked the latter are, the more serious the out- look is. Chorea of pregnancy is a serious malady (see above). Recurrences are very frequent in chorea. After the patient has made apparently a complete recovery, a shock, an emotion, promptly brings on another attack. Some patients have every year an attack of chorea 32 498 CHOREA during several years. Chorea is sometimes associated with other neuroses, viz. hysteria, epilepsy. Diagnosis. — The rapidity, irregularity and incoordination of muscular contractions are sufficiently characteristic signs for diagnosis. Occasion- ally hesitation is experienced and the only affections with which chorea may sometimes be confounded are: tic, myoclonia, athetosis and Hysteria. Tic and Myoclonia are recognized from the suddenness and instan- taneity of twitchings which are confined only to a certain portion of the body. In tic the movements are coordinate and purposive, while in chorea they are distinctly incoordinate. The tic movements can be controlled to a certain extent, choreic movements cannot be controlled. The former are not disabling, the latter are. In Athetosis the movements are slow and regular and affect mostly the fingers or toes. In Hysteria the movements are coordinate and they usually develop suddenly after an emotion or during an hysterical paroxysm. Etiology. — Sydenham's chorea is a disease of young age and affects children from the age of seven to puberty. Girls are more predisposed than boys. It may, however, occur at any age. The predisposing causes are a neuropathic hereditary tendency which can be traced in the majority of cases, constitutional diseases (tuberculosis, etc.), anaemia or a debilitated state originating from any cause. Infectious diseases, among which acute inflammatory rheumatism occupies the first place, are not infrequently accompanied or rather followed by chorea. Scarlet fever, measles, diphtheria, smallpox may be followed by chorea. In secondary syphilis chorea has been observed and the former may be the cause of the latter. Recently Milian (Bull, et Mem. de Soc. med. d. hop. Paris, 5 Dec, 191 2) reported fifteen cases in which stigmata of hereditary syphilis were present. Moreover, in eight cases out of thirteen Wassermann reaction was positive. He calls attention to von B okay's case in which Salvarsan cured the chorea. Rheumatism, however, is most frequently followed by chorea. The car- diac condition which is so frequently met with in chorea is probably due to the preexisting rheumatic affection. Pregnancy is an important factor in causation of chorea, especially in cases with a history of previous attacks (see above). Fright, emotion, traumatism are frequently the immediate causes of chorea, especially in predisposed individuals. Predisposed children being in company of choreic ones may sometimes develop the disease by imitation. Chorea is rarely observed in negroes. CHOREA 499 Pathology. — Hyperemia, punctiform hemorrhages and perivascular alterations have been observed by some writers. Hudovernig (see below) found cellular changes analogous with those of anterior polio- myelitis, especially in thalamus opticus. Proliferation of connective tissue was observed in the spinal meninges. It is possible that these changes are due to a toxi-infectious process. There are also many observations on record with absolutely negative findings. Bignami and Nazari (Presse Med., 191 1) report two cases of hemi- chorea, in one of which was found a solitary tubercle in the left superior cerebellar peduncle, in the other a hemorrhagic focus in the same spot as in the first case. Pathogenesis of Chorea. — The post-mortem investigations present nothing definite for the localization of the disease. There is a tendency at the present time to consider chorea as infectious in nature. The bacteriological works, especially of Pianese, favor this view. This author found in the spinal cord a bacillus with the cultures of which he made successful inoculations. Microorganisms in the brain were also found by other authors. Poynton and Paine isolated from the cerebro- spinal fluid a diplococcus which after an inoculation into a rabbit produced muscular twitchings. The diplococcus was found in the pia-mater and brain in choreic patients and in the rabbits. The cytological examination of the cerebro-spinal fluid showed in a number of instances a marked lymphocytosis. In favor of the infectious origin speaks also the occurrence of chorea with infectious diseases. I have seen cases in which chorea developed subsequently to a localized inflam- matory focus; the twitchings appeared at the time of formation of pus. The choreic movements disappeared with the removal of the infectious focus (/. Amer. Med. Ass'n., 1910). That acute chorea is due to an in- fectious agent is highly probable. In this respect it is analogous to acute anterior poliomyelitis with this difference, that in the latter the motor cells are being destroyed, hence the paralysis; in the former the motor cells of the cortex and of the nuclei of the medulla undergo irri- tation, hence the twitching. The destructive effect in the latter and the irritating effect of the former point to a difference in the virus in the two affections. Hudovernig's pathologic report (Arch. f. Psych., 1903) shows a very great resemblance between the cellular changes of both affections. It must not be forgotten that the bacteriological investigations cannot as yet be accepted as absolutely conclusive. Charcot's and Joffroy's views cannot be neglected. According to them there is an inherent degenerative predisposition of the motor apparatus, which is brought in evidence as soon as some special cause 500 CHOREA disturbs the latter. This cause may be an acute inflammatory rheumatism or any infectious disease. Treatment. — It was mentioned above that a neuropathic tendency is found in the majority of cases. The indication is therefore to improve the general condition of the patient by proper dietetic and hygienic measures. In cases of anemia, tuberculosis or other constitutional diseases an effort should be made to improve these conditions. In an average case of chorea factors leading to emotion, excitement or depression must be removed. Mental strain is contraindicated. The child should not be sent to school. The patient's life should be so regulated as to give him a sufficient amount of rest, good sleep, proper and nutritious food in moderate quantities. I am in a habit to remove meats and all stimulants, including tea and coffee. Elimination must be taken proper care of. An occasional purgative is beneficial. When the twitching is marked and does not show any tendency to improve, I keep the patient in bed. This procedure has given me excellent results in many obstinate cases. Cool spongings followed by a gentle massage have a sedative action on the patient's nervous system. In cases with violent twitch- ings the patient must be guarded against unjury and being thrown out of bed. Among all the medications the most reliable ones are: arsenic and antipyrin. It is advisable to commence the treatment with the first. Its administration should begin with very small doses and only very gradually increased, as otherwise intolerance will be exhibited very early. An average child of ten should be given TTtiii t. i. d. of Fowler's solution during the first two or three days. If after two or three weeks of treatment no improvement is noticed, the arsenic should be substituted by antipyrin. To a child of the same age gr. j of the latter can be given every two hours. I have frequently obtained very good results with antipyrin when arsenic failed. When none of these drugs yields results, the patient should be kept in bed and given bromides. The latter may be given conjointly with arsenic. When there is a history of rheumatism, sodium salicylate, aspirin or salophen give very satisfactory results. As to individual doses of all medicaments, they will have to be modified according to the age and the intensity of symptoms, but it is always wise to commence with very small doses. A very gradual increase in the amount is always preferable to a rapid increase, as it establishes a satisfactory tolerance. The patient must be frequently observed, and with the first signs of physiological intolerance, the given drug must be immediately discon- tinued and in a day or two substituted by another. CHOREA 5OI Among other drugs chloral hydrate is to be recommended for con- trolling the twitching when other remedies fail. In chorea of pregnancy artificial termination of pregnancy may be considered. The latter should be undertaken when the indications are strong, viz. when life is endangered by exhaustion, cardiac or renal lesions or mental disturbances. The latest researches of Loeb and J. B. MacCallum, also of W. G. MacCallum and C. Voegtlin, show a certain relationship between various twitchings and calcium metabolism also the function of the parathyreoid glands. A trial of calcium salts and of parathyreoids in chorea is there- fore indicated. Finally Marinesco (Semaine med., 1908), advises the use of intra- spinal injections of magnesium sulphate (25 per cent.). He withdraws first a certain amount of cerebro-spinal fluid and injects the same amount of the drug. The amount injected is 1 c.c. to each 25 pounds of bodily weight. Marinesco obtained very satisfactory results in every one of his cases. He advises against the use of this drug in grave cases, in chorea of pregnancy and in cases of chorea dependent on an organic disease of the central nervous system. CHRONIC CHOREA HEREDITARY CHOREA (Huntington's Chorea) This disease, which has no relation whatever to Sydenham's chorea, was known before Huntington, but the latter was the first to call special attention to three important elements of the affection, viz. heredity, onset at the age of thirty or forty and mental symptoms. Symptoms. — The clinical picture differs little from that of Sydenham's chorea. Like in the latter, the movements are arhythmical, irregular, incoordinate. The onset is slow and the twitchings at first appear on the lower half of the face. Gradually they spread to the upper and lower extremities, also the trunk. When the muscles of the palate and pharynx become involved, the deglutition is difficult. The tongue is particularly affected, so that the speech becomes indistinct and nasal in tone. When the diaphragm is involved, the respiration is disturbed. The reflexes are exaggerated. Muscular weakness is usually present, but there is no atrophy; neither are there changes in the electrical reactions. Sensations are intact. The distinguishing features of Huntington's chorea are: 502 CHOREA i. It occurs in adult life. 2. The movements are slower and not as frequent as in Sydenham's chorea. 3. The muscles of the eyeglobes are usually not involved. 4. The upper part of the face is rarely affected. 5. The gait is characteristic. It is analogous to that of an inebriate. The patient makes a few rapid and awkward steps, then stops suddenly; leans forward, looks at the ground and then again advances with small steps. All this is done rapidly and with variation. 6. Voluntary effort may repress the twitchings, so that at that time the patient is able to execute delicate acts, as writing or threading a needle. 7. Rest decreases the intensity of the twitchings. 8. As the disease advances the mentality suffers. Extraordinary irritability is constant and is frequently one of the earliest symptoms. Defective power of attention in execution of physical as well as of mental acts is one of the most essential characteristics. Gradually the memory for recent and old events weakens and the conceptions become retarded. The patient is depressed and the intellectual faculties become feeble. There is a tendency to suicide. Dementia is the ultimate result. The mental phenomena as a rule follow the motor phenomena, but in some cases they precede. Course, Termination, Prognosis. — The disease lasts many years, develops slowly, but it is essentially progressive. Death occurs either from disturbance of deglutition and respiration or from the extreme mental hebetude or else from some intercurrent disease. Diagnosis. — The differential diagnosis with Sydenham's chorea is given above. In tic the movements are abrupt and always the same. Etiology. — In the majority of cases there is a direct heredity or a general neuropathic taint. There are cases on record in which several successive generations were affected with this disease. Huntington observed that when one member of an affected family escapes, his off- spring are free from the disease. In other cases there may be a family history of epilepsy or hysteria. Both sexes are equally affected. The disease occurs in adult life between thirty and forty. Emotions have a very important influence upon the development of the malady. Traumata and pregnancy are equally exciting causes. Pathogenesis of Huntington's Chorea. — Post-mortem examinations show an anatomical basis of the disease. Atrophy of the cortex and espe- ATHETOSIS 503 daily of the motor area, thickening and adhesions of the meninges, diffuse meningoencephalitis, have been found. Microscopically disseminated foci of round cells in the cortex and white matter have been seen quite frequently. Whether a parenchymatous degeneration of the neurons or a vascular alteration is the primary condition, it is difficult to say. There is a possibility of hereditary malformation of the central nervous system. The relation between the anatomical findings and the clinical picture of the disease is not entirely established. Treatment. — When treated early the patients may derive some benefit from good hygienic and dietetic measures, from bromides, arsenic, chloral, antipyrin. As a rule all these means are only palliative. The disease is progressive and incurable. ATHETOSIS It is characterized by continuous, slow, involuntary movements, mostly of fingers and toes, occurring even during sleep. Unilateral Athetosis (Hemiathetosis) stands in close relation to hemi- plegia (see this chapter). Bilateral Athetosis. This is mostly a congenital condition appearing in infancy and accom- panied by mental symptoms. Symptoms. — The onset may be insidious or rapid. In the latter case it is preceded by a fright, or a convulsive seizure. In the majority of cases the course is progressive, affecting the face, extremities and trunk in successive order. The muscular movements are involuntary, slow, of wide range. When on the face, expressions of fright, joy, laughing or crying, of contemplation, etc., will be alternately observed. The eye- globes, tongue, usually participate. In the upper extremities the fingers are mostly affected. There will be a continuous display of flexion and extension, also of abduction and adduction. Sometimes the wrist, forearm and arm are similarly affected. The functional disability is therefore evident. In the lower extremity the toes and ankle are mostly involved. When the neck is affected, there will be an oscillation of the head in all directions. The trunk is rarely invaded. Rigidity of the muscles is another characteristic feature of the disease. It, becomes marked upon a voluntary act. The spastic condition causes deformities of the limbs and this is especially marked in the lower extremi- ties. The gait is spastic and difficult. The reflexes are exaggerated. When the muscles of the lips and tongue are affected, the speech is diffi- cult ; it is usually slow and dragging. The muscles are hypertrophied from 5°4 ATHETOSIS continuous movements, but there are no changes in their electrical re- actions. Relaxation of the joints and subluxation of the phalanges are sometimes observed. Fig. 151. — Position of Fingers in Athetosis. (Slriimpell.) ATHETOSIS 505 Mental symptoms are the third characteristic sign of double athetosis. They are congenital and noticeable early in life. They consist of a feeble- ness of all the intellectual faculties. In exceptional cases the intelligence is preserved. Course, Prognosis. — As all other functions of the body are intact, the disease does not endanger life. Death occurs from some intercurrent affection. The disease itself is stationary and lasts many years. Pathogenesis. — The lesions found post-mortem have not been con- stant, so that positive conclusions as to the nature of the disease cannot be drawn. Cortical lesions, malformation of convolutions, pachymen- ingitis, asymmetry of the hemisphere, of the cerebellum, of the medulla, cerebral sclerosis, have all been observed in cases with positive findings. There are also cases in which the central nervous system was found to be normal. The consensus of opinion is that bilateral athetosis is caused by a bilateral irritation of the motor area or the motor pathway. Etiology. — A hereditary neuropathic taint is present in the majority of cases. Syphilis, alcoholism, epilepsy, insanity, etc., are not infre- quently traced in the family. Premature or difficult labor is a potent factor in causation of cerebral disturbances and therefore of double athetosis. Infectious diseases and traumata occurring in early infancy are sometimes the direct causes of the disease. In some cases no appreciable cause can be found. The dis- ease occurs in early infancy, although later development has also been observed. Treatment. — Sedative medications (bromide, chloral), hydrotherapy, may be tried, but very little can be expected from them. Systematic exercises, with the purpose of controlling the movements, kept up for a long time and carried out persistently and patiently, may yield satisfactory results. DYSTONIA MUSCULORUM DEFORMANS (DYSBASIA LORDOTICA PROGRESSIVA) Oppenheim in 191 1 (Neurolog. Centralblatt.) described a symptom- group which he characterized as "a disturbance of muscle tone." Prior to him Ziehen (Allg., Ztschr. f. Psych., LXVIII) observed an analogous condition to which he gave the name of "tonic torsion neurosis." The disorder, according to Oppenheim, has some resemblance to Huntington's chorea and more to athetosis, so that his diagnosis of the condition at first was between hysterical lordosis and idiopathic bilateral athetosis. Symptoms. — The main features of the disease are : a deformity about 506 ATHETOSIS the pelvis and spasms of the muscles surrounding the pelvis, also twitchings in other muscles. The disease may begin with twitchings in the upper limbs, but it is in the muscles of the pelvic girdle that they are most marked. The twitchings are evident while standing or walking, but not in a lying position. The deformity, which is persistent, consists of a marked lordosis of the dorso-lumbar region with a lateral inclination of the pelvis. The gait is especially peculiar, it resembles the movements Fig. 152. — Dysb asia Lord otica Progres- siva or Dystonia Musculorum Deformans. (Oppenheim.) Fig. 153. — Same as Fig. 152. of a quadruped. While the patient walks, he is affected with movements of a clownish character ; the fatigue and strain caused by such movements brings on a perspiration and rapidity of pulse. The muscular twitchings are either a rhythmical tremor, or rhythmical clonic contractions, especially in the lumbo-abdominal muscles. Tonic contractions are seen especially in the upper extremities. On passive movements a distinct hypotonia is observed even in the muscles which are affected with tonic contractions. (Figs. 152 and 153.) TIC 507 The disease presents no evidences of organic involvement of the nerv- ous system. The tendon reflexes are sometimes diminished. There is no paralysis, no muscular atrophy, no electrical change. Sensations, sphinc- ters, cranial nerves, psychic sphere — are all intact. The course is progress- ive. The disease occurs in children from eight to fourteen years of age. Treatment. — Oppenheim obtained relief from metallotherapy in one case. J. Fraenkel obtained satisfactory results from intraspinal injections of sulphate of magnesium and from reeducation movements. The nature of the disease is unknown. TIC It consists of abrupt involuntary contractions of a muscle or groups of muscles. Symptoms. — The sudden involuntary contraction of muscles has a convulsive character and it may be clonic or tonic. In the first case the individual contractions are separated by intervals of rest. In the second case the contractions are so near each other that they give the impression of a prolonged contracture. Unlike chorea the tic is characterized by coordinate movements, by systematized movements. At the beginning of their development these movements consisted of muscular con- tractions executed for a certain definite purpose, but in an exaggerated manner. For example, tic of the eyelids produces exactly the act of their sudden closure done to protect the eye from penetration of a foreign body. Little by little, when these movements are frequently repeated, they become a matter of habit and necessity. Tic is therefore a disease of habit, a habit which through its persistency acquires a morbid character. Tic may affect one muscle, if this muscle by itself has a certain func- tional purpose. What is important is the fact that the invaded area does not correspond to a well-defined anatomic distribution of a certain nerve or nerves contrary to what is seen in spasm. In the majority of cases several muscles contract simultaneously, as their associated action is necessary for execution of certain acts. Occasionally a certain portion of a muscle may be affected by tic; it occurs in those muscles various portions of which have different functions (deltoid, trapezius, etc.). The form, intensity and rapidity of the twitching vary from patient to patient and in the same patient. Tic has a tendency to spread and invade other functions, so that the twitch of the face may be accompanied by a sudden protrusion of the tongue or by a laryngeal noise, by a scream or by a certain gesture in other parts of the body, such as sudden kick of the foot. 5 o8 TIC Gilles de la Tourette describes various tic movements accompanied by coprolalia, viz. enunciation of profane words. Another characteristic feature of tic is the possiblitiy with a certain effort of retarding or sup- pressing the muscular contraction. The reason of it lies in the cortical nature of the phenomenon. Tic usually disappears during sleep. Sensations , reflexes, sphincters are intact in tic. The affected muscles may sometimes become hypertrophied, but this condition is only functional in nature. Forms Tic of the Face. — The most frequent tic of all the muscles of the face is that of the eyelids (palpebral tic). It is usually bilateral. The neigh- boring muscles frequently participate. The eye-globes frequently partici- pate (nystagmoid tic). The latter symptom is not observed in blepharo- spasm. Next to the eyelids the lips are the most frequently involved. Grimaces are the result of tic of the lips. Fig. 154. Fig. 155. Figs. 154 and 155 — Mental Torticollis and Correction Gesture. (Brissaud.) Any other muscles of the face, including the platysma, may be affected by tic. When the tongue is involved, all sorts of noises may be heard from the movements of the tongue. When the masticatory muscles suffer from tic, abrupt and repeated lowering or raising of the lower jaw will be noticed. Tic of the Neck. — Sudden and repeated rotation, flexion or extension of the head will be noticed according to the muscles involved. The flexors are more frequently affected than the extensors. Tic of the neck is frequently associated with tic of the face or of the shoulders. TIC 509 Torticollis in the form of tic (mental torticollis of Brissaud) or spas- modic torticollis may present itself as sudden simple rotatory movements, rotation with flexion or rotation with extension. The tic may consist of a single movement or of a series of successive movements. Among all the muscles of the neck the sterno-mastoid is the most frequently affected. During the contraction the face is turned to the opposite side, the head is inclined on the same side and the ear touches the shoulder. When the upper part of the trapezius and splenius muscles are involved, together with the sterno-mastoid, the head, besides being inclined, is also drawn backward. When the muscles of the anterior part of the neck are in- Eig. 156. — Spasmodic Torticolis. Patient Seen in Attempt to Correct Position of Head. volved (which is rare), the head falls forward so that the chin touches the chest. Rotatory tic of the head is often combined with elevation of the shoulder. The patients frequently complain of pain or of a drawing sensation in the neck. This is of course due to the repeated muscular contractions. Rest, physical and mental, decreases the intensity of the tic. Fatigue and emotions increase it. The patients use all sorts of subterfuges to 510 TIC remove the torticollis. The most frequent attitude is to keep a finger on the chin. The onset of the affection is usually insidious. The disease originates from some false position adapted at first for the purpose of relieving pain in the neck or from unavoidable positions in certain occu- pations which require special gestures or, as some pretend, from visual disturbances. The act is repeated a number of times, at first voluntarily, and finally involuntarily. The condition is thus established. In cases of long standing the involved sterno-mastoid muscle may become hyper- trophied, and the muscle of the opposite side atrophied for want of exercise. Tic of the shoulder consists mostly of a sudden raising of the shoulder. The habit is acquired through some discomfort felt from the clothes covering the shoulder or else from some pain in that region. Tic of the arms, hands and fingers occasionally occurs. Tic of the Trunk is a rare condition. In a case reported by me (Amer. Medicine, 1906) the tic consisted of abrupt salutations: the recti muscles of the abdomen contracted. Tic of the Lower Extremities may be manifested in sudden bending of the knees, jumping, kicking, changing of the steps. Respiratory Tic. — It presents itself as abrupt inspiratory or expiratory movements. It is frequently associated with contractions of the muscles of the naso-pharynx. The sounds of snuffing, of snoring, are heard. Laryngeal Tic is manifested either in sudden laryngeal sounds, as grunting, barking, etc., or shouting certain syllables or words (Verbal Tic) . The latter is particularly encountered in a symptom-group known under the name of Tic Convulsif. — This condition was described first by Guinon and Gilles de la Tourette. It begins with the muscles of the face. Blinking of the eyes, pouting the lips, protruding the tongue, grimacing the face, blowing, whistling, chattering of the teeth — all these acts are done with extreme rapidity, convulsive-like. Frequently the tic does not remain confined to the face; the neck, upper and lower extremities and trunk participate. Thus the patients will also exhibit sudden raising and lower- ing of the shoulders, propulsion, retropulsion or lateral movements of the trunk, rubbing of the hands, raising of a finger to the nose, ear or mouth, stamping of the feet, jumping, dancing, etc. All these movements are spasmodic, abrupt, rapid, but they systematically succeed each other. They may be arrested temporarily, but at the expense of a painful struggle and intense anxiety. Voluntary acts arrest the movements. They disappear during sleep. Another characteristic manifestation of tic convulsif is coprolalia or TIC 511 echolalia. The first consists of a sudden use of obscene words in the midst of an ordinary conversation. In echolalia the patient repeats sounds, words or syllables heard around him. Sometimes he repeats movements seen by him (echokinesis) . All these acts are done under the influence of an irresistible impulse. The patient is always fully conscious of his uncontrollable movements. To mask them he frequently executes some voluntary acts in rapid succession, but it is not an easy task to over- come the involuntary movements. For the same reason the patient is seen doing the most peculiar acts, as spending considerable time in counting the buttons on his coat, the number of stores on the street, his own footsteps, etc. Here the motor phenomenon of Gilles de la Tourette's disease may be absent, but imperative conceptions compel the patient to utter certain words or sentences or perform certain acts. This is a Psychic Tic. Tic of Salaam (Spasm nutans). — It is a rare affection and occurs mostly in infants. It consists of the act of flexion of the head and the upper part of the trunk repeated a great many times — from twenty to fifty in a minute. It is, otherwise speaking, a convulsive salutation. The affection is also spoken of as "head nodding." In some cases the phenomenon assumes the character of epilepsy. Thus it may be preceded by a sudden pallor of the face, staring and dilatation of the pupils; there may be also a loss of consciousness. In some of these cases genuine epileptic convulsions develop later. Course, Duration, Prognosis. — The tendency of tic is to spread and invade other portions of the body. The duration is uncertain. Recur- rences are frequent. The younger the individual, the more is the case hopeful as to recovery. Rest, mild diversion, quit life have a beneficial effect. Fatigue, emotions, aggravate and prolong the condition. The longer the tic lasts the more obstinate is the case. Among all the forms Tic convulsif has the gravest prognosis. It has an essentially progressive course. The mental symptoms which accompany the motor phenomena may terminate in dementia. Tic in the aged presents also an unfavorable prognosis. The other forms of tic are amenable to treatment if properly managed. Diagnosis. — Although the symptoms of tic are typical, nevertheless it may be confounded with some other affections. It is spasm that is sometimes difficult to differentiate from tic. In tic the movements are purposive, only in an exaggerated form. In spasm one fails to find the least tendency to reproduce physiologic acts.. Spasm of the face, for example, has no resemblance to ordinary mimicry In tic the muscles involved do not correspond to a weD -defined anatomical 512 TIC distribution of a nerve. In spasm, on the contrary, the convulsive movements are produced solely in the area of distribution of a nerve. Tic disappears during sleep, spasm does not. An effort of will or attention is capable to arrest or inhibit tic, but not a spasm of the muscles. In the majority of cases of tic we find a hereditary predisposition to some nervous disorder. This is not observed in cases of spasm. Paramyoclonus multiplex is recognized by its clonic contractions, which are sudden, quick in succession, irregular, arhythmical; they may involve not only an entire muscle, but also a portion of it and usually they affect symmetrical muscles. They occur more frequently in the lower extremities than in any other portion of the body; the face never participates. They cannot be controlled by the patient. They can be easily brought on by stimulation of the skin. Myokymia is characterized by fine fibrillary contractions, also by vaso-motor and sensory disturbances. In chorea the movements are incoordinate, irregular and not convulsive. Pathogenesis. — As said above, tic is a functional disturbance in which repetition of the act is influenced by an imperative desire, so much so that if the patient resists the act he suffers, but after the movement is produced there is great relief and satisfaction. It is therefore evident that the mentality plays a certain role in the phenomenon. It is because of a deficient will power that the twitchings become automatic, that a habit is established. In fact tic occurs usually in individuals with peculiar ideas, desires and tendencies. They are eccentric, not stable, and fre- quently develop fixed ideas, obsessions. For this reason Brissaud con- siders tic as a "psychic" malady. Etiology. — Tic can be considered as a degenerative neurosis. A neuropathic heredity plays an important role. Insanity, epilepsy, hysteria, tic, tuberculosis, diabetes, organic nervous diseases, are not infrequently traced in the family histories of individuals affected with tic. Bad hygienic surroundings, habits, excesses, undue cerebral fatigue, are predisposing causes of tic. Local irritation and local lesions (eyes and naso-pharynx) are the exciting causes. Tic is rare in very young children. It usually occurs at the age when great physiological changes take place, viz. puberty, menopause, old age. Treatment. — In view of the neuropathic make-up of individuals suffering from tic, the first indication is to improve their general health with proper hygienic and dietetic measures. Such patients must lead a life free from excitement or emotions of any sort. TIC 513 As to the tic itself, Brissaud, Meige, Feindel and others obtained satisfactory results from special physical and psychic methods. The first consists either of voluntary immobilization followed by systematic exercises. When immobilization is used, the patient is taught to im- mobilize the affected muscles for a gradually increasing period of time. The sittings are held daily, at first only for a few seconds and only two or three times a day. Gradually the number of exercises is increased and the duration of each is prolonged. Patience and perseverance are re- quired. A child must be placed in charge of some trained person. An adult can be taught how to proceed. He is advised to have before him a mirror so that he can watch the procedures. When a certain amount of control has been obtained by the patient, the next method is taken up, viz., voluntary movements. Exercises consist of replacing the involuntary movements of the tic by voluntary and correct movements. By being voluntarily contracted, but in a slow, deliberate and correct manner, the affected muscles fall under control and thus become trained. Here again the first seances and their duration must be brief. Gradually they are increased. These exercises should be made under control of an observer or the patient can train himself gradually in the performance. In the latter case he places himself before a mirror and is thus able to acquire a certain skill in the treatment. The psychic method plays an enormous role. It consists of continuous encouragement and of pointing out to the patient the necessity of training his will power in overcoming the involuntary movements. Isolation, rest in bed, removal from usual surroundings, together with the above physical and mental training will give in a number of cases satisfactory results. As to medications there is none to be relied upon. The usual sedatives may be employed. The most recent researches of Loeb and J. B. MacCallum, also of W. G. MacCallum and C. Voegtlin, show a certain relationship between abnormal motor phenomena and calcium metabolism, also the function of the parathyreoid glands. A trial of calcium salts and of parathyreoids in tic is therefore indicated. In spasmodic torticollis operative procedures have been attempted. They consist of excision of a portion of the spinal accessory nerve on the affected side and of section of the posterior primary divisions of the upper cervical nerves on the opposite side. I have seen failures from this operation. 33 514 TIC FACIAL SPASM Symptoms. — Spasm of the face commences with clonic contractions which as they advance gain in rapidity and at the height of the attack are replaced by tonic contractions. As the latter subside, clonic con- tractions reappear and remain until the attack is over. The entire cycle lasts but a minute. The following individual features are observed during the paroxysm. The forehead on the affected side is wrinkled, the orbicularis palpebrarum closes the eye; there is a simultaneous con- Fig. 157. — Facial Spasm on the Left. traction of the frontalis and orbicularis palpebrarum. This phenomenon is called by Babinski paradoxical synergia. The zygomatic muscles deviate the angle of mouth. The nose is curved toward the affected side and the chin presents a characteristic depression on the affected side. The muscular contractions may be either fascicular, tremulous or coarse, of a wider range (Figs. 157 and 158). The muscles involved in facial spasm correspond to the well-defined anatomical distribution of the seventh nerve and according to the case the convulsive movements predominate in the upper or the lower portion of the nerve. Facial spasm occurs during sleep. No effort of will or attention is capable to arrest or prevent an attack. TIC 515 The most frequent form is hemispasm, but double spasm has also been observed. Meige described a median facial spasm, which consists of bilateral convulsive movements predominating near the median line of the face. Both orbiculares palpebrarum are most markedly involved. Pathogenesis. — The stimulation of the muscles supplied by the seventh nerve may originate in the seventh nerve itself or in its nucleus or else in any of the sensory fibers of the fifth nerve. Blepharospasm is an ex- ^f ' '" ''4&L ^I^^^H ""1 B Fig. 158. — Facial Palsy on the Left Produced by Injection of Alcohol into the Left Facial Nerve. ample of stimulation of the orbicularis through an irritation of the sensory fibers of palpebral mucosa. Facial spasm has been observed in attacks of neuralgia, in connection with carious teeth. Not infrequently the spasm is found limited to both upper and lower eyelids. It is called blepharo- spasm. The contractions may be tonic or clonic. In the first variety the eyelids are persistently closed during the paroxysm, which may last many minutes. It is due to some irritation of ocular branches of the fifth nerve. The clonic variety consists of rapid winking movements. The majority of cases show that the cause of facial spasm lies in the peripheral portion of the seventh nerve. The occurrence of facial spasm in cases of facial palsy speaks in favor of peripheral cause of the spasm. 516 MYOCLONIA Neuroma on the facial nerve has been found in such cases (A. Thomas, Rev. X enrol., 1907). While an irritation of the peripheral end of the nerve is the most common cause, nevertheless facial spasm has been observed also in organic lesions of the central nervous system. In cases of meningo- encephalitis, of pseudo-bulbar palsy, of the pons, facial spasm has been observed. Finally the above-mentioned cases of involvement of the fifth nerve show that a lesion of any portion of the reflex arc (sensory fibers, nucleus of the seventh nerve, motor fibers of the facial nerve) may be the cause of facial spasm. Course, Duration, Prognosis.- — It is usually rebellious to medical treatment. It may have intermissions. It lasts ordinarily an indefinite time. It has no effect on patient's life. Treatment. — If a local cause of irritation can be detected, its removal is necessary. Freezing of the face on the affected side has been recom- mended by Weir Mitchell. The most effective method of treatment is in- jection of a few minims of 80 per cent, alcohol into the nerve at its exit of the stylomastoid foramen. It has given me the most gratifying results. The spasms ceased for periods ranging from eighteen months to three years. The facial palsy which follows immediately the injection disap- peared at the end of five or six weeks in every case. In some cases returns of spasm were treated with repeated injections. No bad results followed repetition of injections. An experimental study {Trans. Amer. Neurol. Ass'n.. 1913) on dogs has shown me that injection of alcohol into a motor nerve is followed by very slight pathological changes in the perineurium, but the nerve fibers themselves remain unaffected. In cases of double facial spasm the injection should be made into each nerve separately and only after the palsy disappeared on one side. Alco- holic injections into the nerve may be tried even in cases of organic diseases of the nervous system. MYOCLONIA The disease is characterized by sudden unsystematized, involuntary clonic contractions similar to those produced by an electric shock. They may be localized or disseminated. The following varieties belong to the group myoclonia. I. Paramyoclonus [Multiplex of Friedreich. II. Familial Myoclonia with Epilepsy of Unverricht. III. Myokymia. IV. Electric Chorea of Bergeron-Henoch. V. Dubini's Chorea. MYOCLONIA 517 Pathogenesis of Myoclonias. — The symptom myoclonia may be en- countered in the course of various organic and functional nervous diseases. That it may be an independent affection is true. That it may be merely a hysterical phenomenon is also correct. It is accepted by the majority of observers that myoclonia is to be considered as an episodic manifesta- tion in neuropathic individuals. As to the physiological basis for myoclonic twitchings, the views are divided. The majority believe that the condition is due to an irritation of the cells of the anterior cornua of the spinal cord. The latest researches, particularly of Loeb and J. B. MacCallum, also of W. G. MacCallum and C. Voegtlin, show a certain relationship between various twitchings and calcium metabolism, also the function of the para- thyreoid glands. I. Paramyoclonus Multiplex Symptoms. — The myoclonic contractions, which are sudden and lightning-like, appear first in the lower extremities, but they may be- come generalized. The face as a rule is respected. The contractions are unequal, irregular and arhythmical. They may affect individual muscles or groups of muscles. Their frequency may vary: they may occur every few minutes or every half hour. They may be mild or so violent as to move the affected part. When in the lower limbs, the locomotion is disturbed. When in the upper limbs, movements of flexion, extension, supination, etc., are observed so that the usual occupation of the patient is impossible. Usually symmetrical muscles on both sides of the body are affected. The muscles of the limbs are more frequently affected than those of the trunk. If the muscles of the pharynx, larynx and diaphragm are affected, disturbance of deglutition and of respiration, respectively, will be observed. The nutrition of the muscles as well as their electrical reactions are intact. Sensations are normal. The reflexes are exaggerated. The muscular contractions cease during sleep; they may be sometimes arrested or lessened by a voluntary effort, although in some cases a reverse condition is observed. Emotion increases their intensity and frequency. Course, Duration, Prognosis. — The disease is progressive and its onset is insidious. It may last indefinitely. Cases of recovery have been reported. Recurrences are very frequent. Diagnosis. — The sudden lightning-like clonic contraction is sometimes sufficient for the diagnosis. In chorea the movements are not so abrupt and their range is wider than in myoclonia. 518 MYOCLONIA It is with tic that the differential diagnosis presents sometimes great difficulties. However in tic the movements are coordinate and systema- tized. Etiology. — Paramyoclonus is found sometimes associated with organic and functional nervous diseases, also insanities. A neuropathic heredity exists in the majority of cases. Infectious diseases, intoxications, fatigue, emotion and traumatism are among the exciting causes. Treatment. — When the condition is only a symptom of other diseases, the first indication is to treat the latter. As in the majority of cases myoclonia develops in neuropathic subjects, much attention should b'e given to the general health. Hydrotherapy, moderate exercises, proper mode of living, avoidance of excitement and worry, nutritious food, etc., are necessary (see chapter on Neuropathy) . For controlling the muscular contractions sedatives may be employed, viz. bromides, antipyrin, chloral and others. Arsenic has been advised. Confinement to bed may sometimes be of service. Not much can be expected from medications. The above-mentioned remarks concerning calcium metabolism and the function of the parathyreoid glands (see Pathogenesis) indicate the use of calcium salts and of parathyreoids in myoclonias. II. Familial Myoclonia with Epilepsy (Unverricht's Type) This form presents an association of paramyoclonus multiplex with epilepsy. The latter may occur only early in life and then disappear, to be substituted by myoclonic twitchings, or else accompany the myoclonia. The occurrence of the same condition in several members of the same family is a striking feature of the disease. In Unverricht's first case (1.891) five brothers and a sister were thus affected. ILL Myokymia It is characterized by continuous fibrillary contractions. The muscles of the extremities are most frequently involved, although other parts of the body may be also affected. Sometimes pain and hyperhidrosis accompany the muscular twitchings. In one of my cases myokymia of the right lower half of the face was associated with myoclonia of the upper half of the face. The least mechanical irritation increased the contrac- tions. The affected muscles presented a decreased faradic and galvanic irritability. TETANY 519 IV. Electric Chorea (Bergeron-Henoch) It consists of sudden twitchings rapidly, but rhythmically, repeating themselves. An attempt to control them increases their intensity. They disappear during sleep. They may affect any portion of the body. The movements are so frequent and intense that the patient is obliged to give up his usual work. The nutrition of the affected muscles is not disturbed. The prognosis is usually good. In a large number of cases the disease was associated with gastric disturbances and improvement of the latter was followed by disappear- ance of the muscular twitching. Autointoxication is therefore supposed to be the cause of the affection. In some cases it may be a manifesta- tion of hysteria, as instances of recovery from suggestion prove. V. Dubini's Chorea By its manifestations it resembles the electric chorea of the preceding chapter, but by its course, duration and termination it differs. The onset, which is abrupt and sudden, is accompanied by pain. The latter affects the head, neck and lumbar region. The twitchings are rapid, appear first in the upper extremities and soon spread. Although they are rhythmical, nevertheless they are constant. In the course of the disease not infrequently are observed genuine convulsive seizures without loss of consciousness. Fever is also present in the majority of cases. The disease is progressive. Gradually the twitchings and the con- vulsive seizures increase in intensity and frequency, a comatose state supervenes and death follows. The duration of the affection is from sev- eral days to four or five months. The sudden onset, the pain, the accompanying fever and the associated pulmonary diseases (which are quite frequent) are in favor of an infectious origin of the affection. Post-mortem investigations have shown in a number of cases congestion and inflammation of the meninges, also of the cerebral tissue. TETANY It is characterized by bilateral, intermittent, painful cramps, especially in the hands. Symptoms. — The muscular spasms occur in attacks. The latter are 520 TETANY usually preceded by a few premonitory symptoms, viz. parses thesia (tingling, numbness, etc.), general malaise and sometimes by a mental depression and vertigo or headache. In the majority of cases cramps appear first in the fingers. The attitude of the hand is then very char- acteristic: it is either in a writing position or in an obstetrical position, viz. the fingers are extended, the first phalanges are flexed, the thumb is against the palmar surface of the other fingers, the entire hand is flexed. Variations in this attitude are observed. When the interossei and lumbri- cales are affected, the hand is in a claw-like position (main en griffe.) Fig. 159 — A Case of Tetany During an Attack. (Oppenheim.) When the contracture spreads and involves the arm, the latter is in a forced flexion and applied to the thorax. If the lower extremities are affected, the flexors of the foot and toes are found mostly in a state of tonic contraction. The toes are flexed and adducted, the feet are arched; the attitude of the foot is equino-varus. Tetany may also affect the muscles of the trunk, abdomen and neck and in rare cases the ocular muscles. When the neck muscles are involved, TETANY 521 the head is bent forward and the chin touches the chest. Rarely the dorsal muscles are involved: the head is then drawn backward and the body is in opisthotonos. The muscles of the face, of the tongue, of the ocular muscles, are occasionally affected. The diaphragm, larynx, may participate and then the patient is threatened with suffocation. If the sphincter of the bladder is affected, retention of urine will be present. The tetanic contractions are usually very painful and the least attempt to move the affected parts increases the pain. Voluntary movements are impossible. During an attack the temperature is slightly elevated and the pulse is accelerated. The spasm may persist during sleep, although as a rule it is less severe. In addition to the above clinical picture the following characteristic symptoms are observed in tetany. 1. Trousseau's Sign. — This observer found that compression of the biceps or immediately below the inferior insertions of the deltoid and in the lower extremities upon the internal surface of the thigh a tonic contraction of the corresponding muscles will be produced. V. Frankl- Hochwart has shown experimentally that compression of the nerve-trunks is the cause of the contracture. Trousseau's phenomenon is pathog- nomonic of tetany. 2. Chvostek's Sign. — Percussion or any mechanical irritability of a motor or mixed nerve or the muscles of the face produces vivid muscular contraction. This so-called facial phenomenon is observed in the domain of the seventh nerve. The symptom is not constant. 3. Homnann's Sign. — Pressure upon sensory nerves produces marked pain or paresthesias. Their electrical excitability is also increased. 4. Erb's Sign.— The electrical excitability of motor nerves is increased so that a very mild galvanic or faradic current gives a prompt and marked muscular contraction. The contraction is not brief as in normal con- dition, but prolonged, and may not subside until the current is inter- rupted. The anodal closure or opening contracture is more prompt than the cathodal closure contraction. Increased response to galvanism is more frequently observed that to faradism. Erb believes that not all the nerves are equally apt to be easily irritated. The ulnar nerve is the most frequently responsive. 5. Schlesinger's Sign. — If the extended lower limb is forcibly flexed over the pelvis, a spasm will appear in the extensors of the knee and the foot is placed in the position of extreme supination. Among other symptoms, although not constant, may be mentioned vaso-motor and trophic disturbances, such as hyperhidrosis, herpes, oedema, falling out of the nails, muscular atrophy. During the attack 52 2 TETANY the face may be flushed, the extremities are cyanosed, the hands are red. These symptoms disappear at the end of the attack. The reflexes are usually normal, but an increase or diminution is sometimes observed. The objective sensations are not modified except when hysteria is asso- ciated. Indicanuria is so frequent in children that it may be considered pathognomonic. Glycosuria has also been observed. Occasionally epilepsy, exophthalmic goiter and myxcedema have been found associated with tetany. Course, Duration, Prognosis. — The attacks may occur every day or only after more or less prolonged periods: the intervals may be hours or days. The individual spasms may last from a few minutes to several hours. The entire disease may consist of but a few attacks or else may last weeks or months. Recurrences are not infrequent and in some cases they appear at regular intervals during a number of years. The prog- nosis in the majority of cases is favorable. It depends, however, upon the cause. Cases with gastric dilatation or with exophthalmic goiter present an unfavorable prognosis. Moynihan has shown that gastric tetany can be relieved by gastro-enterostomy. When the respira- tory muscles are affected, life is threatened. Children particularly are in danger of asphyxia due to spasm of the glottis. Diagnosis. — The symptoms of tetany are ordinarily so typical that the diagnosis is made without difficulty. In tetanus the spasm begins with the muscles of the jaws and neck, the hands and ringers usually escape, the temperature is elevated from the beginning. A pseudo- tetanus, in which there are generalized contractures with albumin in the urine, is met with frequently in children and bears an unfavorable prognosis. In hysteria tonic muscular contractions (pseudo -tetany) are unilateral, painless and not accompanied by Trousseau's and Erb's signs. Latent cases of tetany without spasms have been described. Here the only symptoms of the disease will be Chvostek's and Erb's signs, also some numbness or tingling of the hands. Etiology. — Certain occupations play a predisposing role. According to Frankl-Hochwart's statistics, among 314 patients 141 were shoemakers and 42 tailors. Certain countries are more affected than others. The disease is frequent in Sweden, Austria and Germany. Epidemics of the affection have been observed. Men between fifteen and twenty-five are particularly attacked. In women the disease occurs during pregnancy or lactation and puerperal state. Infection and intoxication play a predominant part in the causation of tetany. In the course of infectious diseases, such as typhoid fever, TETANY 523 grippe, scarlet fever, measles, malaria, or during convalescence tetany may occur. Among toxic conditions gastro-intestinal disorders are very frequently the cause of tetany. Gastro-enteritis is almost the only cause of tetany in children. Tetany has also been seen in association with albuminuria. Extirpation of the thyroid gland has been followed by tetany. Here also a toxic element is the cause of the disease. It is prob- able that in such cases the parathyroids were removed together with the thyroid gland. Removal of the parathyroids has been seen to be followed by tetany. Rickets, osteomalacia, intestinal worms, use of alcohol, ergot, inhala- tion of alcohol, may be also accompanied by tetany. Local irritation, as the use of a stomach pump or passing of a sound, percussion of the region of the stomach, cold, emotion, exertion, are the exciting causes. Pathogenesis. — No reliance can be placed upon the findings at the autopsies. The results are contradictory and in the majority of cases negative. Mention can be made of some poliomyelitic changes in the cells of the anterior cornua of the spinal cord. The consensus of opinion is that in the majority of cases the disease is due to a toxic or infectious condition. The occurrence of it in connection with gastro-intestinal disorders, with removal of the thyroid or parathyroid glands, with in- fectious diseases, speaks in favor of the above view. Experimental re- searches and close observations on operative cases are strongly in favor of parathyroid insufficiency as being the pathogenetic factor in tetany. But what toxic element is at work, it is at present difficult to ascertain. Gastric Tetany. — In this form of tetany an ulcer of the stomach with resulting pyloric stenosis and gastric dilatation have been found. There is usually hyperchlorhydria. The typical symptoms of tetany are present. The intensity of the spasms may vary from case to case. Sometimes they are so slight or occur so rarely that tetany may be overlooked. In the majority of cases the spasms are severe. The only radical measure in such cases is gastro-enterostomy. As to the patho- genesis of gastric tetany, it may be presumed that a toxic element from the stomach is added to the already existing parathyroid insuffi- ciency. Langmead has recently called attention to tetany in association with dilatation of the colon. The spasms occur regularly and appear to be in intimate relation with the character of the feces. The condition is met with in children of arrested physical and intellectual development. Treatment. — Removal of the cause is the first indication. Gastro- intestinal disorders should be remedied by intestinal antiseptics, enemas and emetics if necessary, but the stomach-pump must be avoided (see 524 TETANY reasons above). In cases of operations on goiter care must be taken to avoid total thyroidectomy. When the latter is unavoidable, thyroid or parathyroid extract must be administered internally. When exposure to cold is the cause, warm baths and diaphoretics are of benefit. The spasms can be relieved by sedative medications, such as bromides, morphine, chloral. Rest in bed is an excellent measure in some cases. Trousseau advises application of ice to the spine. On the other hand, tepid baths administered several times a day for ten to fifteen minutes may be of great benefit. Galvanism may sometimes render good services. In one of my cases absolute rest with milk diet gave me very satisfactory results. In the gastric form of the affection, which is rebellious to treatment, gastro-enterostomy should be undertaken without delay, as excellent results have been reported. In cases of asphyxia due to spasm of the glottis, hypodermic injections of pilocarpine or application of a wet cloth to the neck may be useful. Digitalis is advised by Gowers. The researches of Loeb and J. B. MacCallum show that there is a great relationship between a tetany and reduction of calcium salts in the organism. An analysis of blood taken from a dog during tetany shows an amount of calcium which is only about a half that of a normal dog on the same constant diet. It is also known that the parathyroids control the calcium metabolism, so that upon their removal a rapid excretion deprives the tissues of calcium salts. For these reasons administration of calcium salts or of parathyroids is indicated in tetany. The intravenous method is the best for administration of calcium salts. Forty to 80 grains of calcium lactate are diluted in 400-500 c.c. of normal salt solution. The injection can be repeated in twenty-four hours if necessary. The para- thyroids may be administered by mouth, intravenously or by grafting. The intravenous method gave the best results. Krabbel {Beitr. z. klin. Chir., 191 1) has recently reported excellent results from implanting para- thyroid bodies in the tibia of one patient and in the preperitoneal space of another patient. MYOSPASM FROM INTENSE HEAT This condition was first described in 1904 by Edsall (Amer. J. Med. Sciences) and later from a larger number of cases by Cameron (/. Am. Med. Ass., 1909). Symptoms. — In working men exposed to intense heat (i4o°-235° F.) such as iron- workers or men employed in firerooms of vessels, a very painful tonic spasm of the muscles develops spontaneously or upon the least volun- tary effort. An attack lasts from half a minute to a minute and occurs THOMSEN ; S DISEASE 525 very frequently during the illness, the duration of which is about twenty- four hours. A sense of exhaustion and soreness with tingling in the muscles remains for some time. Between the individual spasms a fibrillary con- traction of the affected muscles is distinctly noticeable. The muscles of the forearms and legs, also the abdominal muscles, are usually involved. The condition grossly resembles tetany, but the special symptoms char- acteristic of the latter are absent, viz. Erb's, Trousseau's and Hoffmann's sign. The mechanical irritability of the muscles is increased. The re- flexes are normal, there are no changes in the general objective sensi- bility (touch, pain and temperature), in the sphincters and in the pupils. Etiology. — The spasms are due to the intense heat of the atmosphere in which the work is done. They occur, according to Cameron, usually after the men have been working for some time. Men on the day turn would be attacked toward the close of the morning's or afternoon's work, and the night-turn men toward midnight. Overwork is probably one of the predisposing factors. Alcoholism is probably another predisposing cause. Cameron observed that the spasms occurred mostly in hard drinkers. Lowered muscular tone from other causes is also one of the predisposing elements. Prognosis. — One attack usually predisposes to others. Recovery is possible. However, fatal cases have been reported. They may be due to a spasm of heart muscle. Pathogenesis.- — Nothing definite is known as to the nature of the disorder. Disturbances of metabolism producing some degenerative con- dition of the muscles have been suggested. Treatment. — It must naturally be symptomatic. Pain may be re- lieved by the usual remedies or by a general anaesthetic. A mild inter- rupted faradic current gave Cameron. some satisfactory results. THOMSEN'S DISEASE (Myotonia Congenita) Symptoms. — The chief symptom is a sudden tonic contraction of a group of muscles when an attempt is made to make a forced movement. At first there is an inability to continue the movement, but gradually and very slowly the muscles relax and the affected limb is able to perform the act. The stronger the contraction is the longer is the relaxation. When later an attempt is made to stop or to modify the movement, a new spasm occurs. As the lower extremities are the usual seat of myotonia, it is in the act of walking that the phenomenon is mostly observable. All the muscles may become affected, especially those of the extremities, but 526 thomsen's disease those of the trunk and neck are less frequently involved than those of the extremities. Exceptionally the muscles of the tongue and the masti- catory muscles are involved. The muscles of deglutition and ocular muscles are very rarely affected. The myotonic spasm is increased by reflex acts, such as sneezing, coughing, etc., by cold, exertion and especially by an emotion. On the contrary it is decreased by heat, physical and mental rest. The muscles affected by myotonia are generally hypertrophied, but their power is diminished. The following special signs are characteristic of the myotonic muscles and of the nerves distributed in them. Erb called attention to the fact that mechanical irritability of the nerves is normal or diminished. Their electrical reactions are as follows. The faradic and galvanic irritability of the nerves are quantitatively normal when a moderate degree of stimulation is applied. Should the stimulation be prolonged, a persistent tonic contraction will follow. Mechanical irritability of the muscles is increased. The electrical contractility presents here a special feature. With the galvanic current, the KCC and AnCC are equal. The con- tractions are sluggish and continue long after the excitation has ceased. With the faradic current the contraction also lasts a long time (about a half of a minute) and undulation of the muscle is observed. These phe- nomena constitute the so-called "myotonic reaction." Psychic disturbances and epilepsy are sometimes associated with Thomsen's disease. Muscular atrophy and multiple neuritis have also been reported in connection with myotonia congenita. Course, Duration, Prognosis. — The disease makes its first appear- ance in infancy, but becomes well developed at the age of twenty. Exer- tion aggravates it. It may be arrested, but frequently recurs. Life is not threatened, but the affection is incurable; it lasts all life. Diagnosis. — The special characteristics of the disease are sufficient for diagnosis. Eulenburg described a "paramyotonia congenita," which is recognized by a symmetrical muscular rigidity without Erb's myotonic reaction; it usually follows exposure to cold. Etiology. — Heredity plays a predominant part. Thomsen traced over twenty members in his own family. Males are more frequently affected than females. Pathogenesis. — The consensus of opinion is that the disease is a form of myopathy (see this chapter). Histological studies show hypertrophy of muscular fibers and proliferation of their nuclei. The central nervous system is intact. Congenital abnormal development is probably the true nature of the malady. OCCUPATION NEUROSES 527 Treatment. — All the factors that are apt to increase the muscular tonicity (see above) should be avoided. Moderate exercises and rational gymnastics may be beneficial. OCCUPATION NEUROSES (Occupation Spasms) Under this name is understood a motor disturbance consisting of a sudden cramp in a group of muscles used in certain acts and brought on exclusively during the execution of those acts. This functional disturbance may occur in any portion of the body, but more particularly in the upper extremities. The most typical form of these neuroses is Writer's Cramp It develops slowly. At first the patient notices a certain fatigue and stiffness in the fingers while writing, so that he is obliged to rest for a while. Soon he finds that the interval of rest must be increased; the difficulty of writing appears as soon as the act is commenced. He is forced to have recourse to various positions, use both hands, etc., and finally give up completely the act of writing. The phenomenon may present itself in the spastic, paralytic and tremulous forms. Spastic. — A sudden extension of the index -and flexion or adduction of the thumb or vice versa is the initial manifestation. Sometimes the medius and the other fingers suddenly flex. In some cases the condition extends to the forearm, arm and shoulder. This is due to the fact that in the act of writing participate not only the muscles of the hand, but also those of the forearm and arm. The cramp will persist as long as the act of writing is insisted upon. In advanced cases pain is present, but only when an attempt is made to write. The characteristic feature of the condition is the appearance of a spasm in the muscles of the hand only in the act of writing, but absence in any other act executed by the same muscles. However in cases of long duration any fine and delicate act of the fingers may bring on an attack. Paralytic Form.— It consists of a sudden sensation of fatigue and numb- ness in the hand while writing. The hand remains applied to the paper. As soon as the penholder is removed, the sensation disappears. A true paralysis of the adductors of the thumb has been observed. 528 OCCUPATION NEUROSES Tremulous Form. — Instead of a spasm or paresis there may be only a tremor in the fingers or in the entire arm during the act of writing. The course of writer's cramp is usually chronic. Periods of ameliora- tion and aggravation are observed. It may last an indefinite time. Recurrences are frequent. Recoveries are possible. Other Occupation Spasms Pianist's Cramp. — It is mostly of the paralytic form. It manifests itself as a sudden functional inability to continue playing. The right hand is more frequently affected than the left, although both hands may become involved. Violinist's Cramp may present itself in the paralytic and spastic forms and mostly in the left hand. Telegraphist's Cramp presents the same course and the same varieties as writer's cramp. In one of my patients there was also pain along the nerve-trunks of the right arm. Shoemaker's, Tailor's, Seamstress's Cramp occurs in the muscles which are exercised in the act of sewing or cutting with large scissors. In blacksmiths the cramp is localized in the biceps and deltoid muscles. The lower extremities are rarely affected. Dancers' Cramp occurs usually in the thigh muscles. The face is also rarely involved. In watchmakers and trumpet- players a spasm of orbicularis palpebrarum has been observed. Pathogenesis. — Various opinions have been expressed concerning the nature of occupation neuroses. Some believe in a muscular origin, others that the condition is due to an irritation of the peripheral nerves and still others in a central cause. The fact that a muscle or a group of muscles will be thrown into a state of spasm only when they are called upon to execute a certain given act and contract normally in other acts speaks against the muscular theory ( muscular traumatism and myositis). On the other hand, there are many individuals who write daily a great deal, pianists who play long hours, and still do not become affected with writer's or pianist's cramp. It is evident that only a certain class of persons is subject to this nervous disorder. The latter is met with in predisposed or neuropathic individuals and the dis- turbance is dependent upon a mental cause. It is sometimes associated with other mental disturbances, as phobias, abulias (see Neurasthenic Insanities). Not infrequently it is observed in several members of the same family. Some writers believe with Duchenne that there is a disturb- PARALYSIS AGITANS 529 ance in the center of coordination. Others think that the cramp is due to a neuralgia caused by molecular changes in the sensory fibers. Treatment. — Abstention from work which causes the cramp is the first indication. General hygienic measures with massage and hydro- therapy are beneficial in view of the neuropathic make-up of the patients. Local massage may be of use. Electricity has given no appreciable results. No special internal medication is to be mentioned. I obtained very satisfactory and sometimes perfect results from Bier's method. In a series of cases (published in the Therapeutic Gazette, 1908) I applied this treatment systematically in every case to the exclusion of all other treat- ment and the patients were allowed to continue their work moderately. I found that in some cases a few applications of the bandage above the elbow — for an hour twice a day — gave the patients great relief. Complete recovery also followed in some cases. It is apparent that the circulation in the affected muscles has somewhat to do with the cramp. Reeducation is a good method. The patient is advised to practice his usual exercises for a very brief time every day and gradually increase the duration and the number of the seances. Benedikt {Wiener klin. Wochenschr., 191 1) ob- tained very satisfactory results from injections of 2 per cent, solution of phenol. He believes that the original source of occupation neurosis is in the tendons and muscles. Tender points can always be found in the tendons of the wrist and hand or in the heads of the tendons in the elbow or shoulder. Injection of weak phenol solution at these tender points restores the condition to normal. Sometimes several injections are necessary. PARALYSIS AGITANS (PARKINSON'S DISEASE) Shaking Palsy Symptoms.— Tremor, attitude, gait, fades, are the elements presenting special features in the disease. Tremor. — It is present in the majority of cases. It is passive in character, viz. it is present when the body is at rest. It usually disap- pears upon voluntary movements, but returns if the latter are sustained. The tremor may affect the entire body, but more frequently the upper extremities and particularly the hands and fingers. Sometimes all the ringers are agitated, but the thumb is especially affected. It moves to and fro over the palmar surface of the other fingers in a continuous and slow manner; its oscillations remind the act of rolling pills or crumbling bread. The tremor is rhytnmical. The tremor decreases from the distal end toward the root of the limb, so that it is not perceptible at the shoulder. 34 53° PARALYSIS AGITANS In the lower extremities the foot is particularly affected. When the patient is seated, the toes are held against the floor, but the heel keeps on striking the floor in a continuous and rhythmical manner. The tremor of the head is usually transmitted by the arms. When there is a primary tremor of the head, it is the result of a contraction of the neck muscles. The tremor is then perceptible on the lips. The patient gives then the impression of muttering silently. The tremor of the extremities as a rule does not interfere with ordinary active movements, provided the latter are not prolonged. Writing is difficult. The tremor disappears during sleep. Emotions and exertion increase it. A continuous vibration of the body occur- ring, for example, in traveling de- creases the intensity of the tremor and renders the patient more comfortable. Attitude. — In a typical case the pa- tient's head is inclined forward and as if fixed to the trunk, the back is curved (kyphosis). He holds himself rigid, turns, walks, sits down, gets off his chair as one rigid mass (rod-like) . This con- dition is due to the muscular rigidity which is so characteristic of Parkinson's disease. Upon passive movements considerable resistance is felt. Al- though in the majority of cases the body is in a state of semiflexion, there are also cases of extension type; the head is held backward, and instead of kyphosis there is lordosis of the spine. The movements of the body are naturally slow and monotonous. It is well to remember that the muscular rigidity is not that of organic nervous nature, as for example is spastic paraplegia or hemiplegia. It is a rigidity of a cadaver, as Bloch has well said. The rigidity produces a certain degree of muscular weakness, which disables the patient for work or even for ordinary voluntary acts. A true paresis may occur only in advanced stages. The patient is in a state of restlessness; there is a tendency to change position. Gait. — In mild cases the only peculiarities noticeable are small quick steps and slow gait. In advanced cases the following is observed. When the patient attempts to walk, he inclines the body forward, steps first on his toes and then for fear of falling he is obliged to accelerate his gait and Fig. 160. PARALYSIS AGITANS 53 1 run. In some cases there is only an accelerated gait (f estination) , in others a distinct tendency to fall forward (propulsion). The latter is easily noticed when a slight push is given on the back. The patient will keep on running until an obstacle is met. The same phenomenon is observed when the patient is pushed backward (retropulsion) or laterally (lateropulsion). Facies. — It is typical. It is characterized by immobility of features. It is mask-like. It gives the impression of astonishment, surprise, fright. This expression is independent of the inner feelings of the patient. The conditions of the facies as well as the fixed expression of the eyes are due to the rigidity of the facial and ocular muscles, respectively. Other Symptoms.— The speech is not infrequently changed. Mo- notonous voice and rapidity of words are its characteristics. Sensory disturbances are only of a subjective nature. The patients frequently complain of rheumatic pain in the limbs, of muscular fatigue and numbness. Sensation of heat is continuously present and sometimes accompanied by abundant perspiration. The tendon reflexes are either normal or diminished or more frequently exaggerated. Ankle-clonus, Babinski and paradoxical phenomena are absent. Trophic disturbances are usually absent. In some cases there is a thickening and hardening of the skin, especially on the face. Rheumatoid deformities of hands and feet are also observed. Muscular atrophy occurs in the last stages of the disease. Vaso-motor Disturbances have been mentioned above. They consist of a feeling of heat and hyperhidrosis. Cyanosis of the extremities, also localized oedemata, may occur. The mentality is usually intact. However there is a certain degree of indifference, apathy and even depression. There is an intellectual fixation alongside the physical fixation. This mental condition varies from time to time in the same individual. In some cases there may be mental failure and even a certain degree of dementia. Course, Duration, Prognosis. — The course of paralysis agitans is slow. In some cases it develops suddenly, as for example in cases following a trauma or emotion. In the majority of cases it commences with a tremor in one extremity, most frequently in one of the upper extremities. Gradu- ally the lower extremity of the same side becomes affected. Later the opposite side is invaded. The development of the symptoms is slow, but progressive. Several years elapse before the disease is generalized. Remissions with amelioration of the symptoms may occur, but no com- plete disappearance of the latter. The disease may last many years, from ten to thirty. Death usually 532 PARALYSIS AGITANS intervenes from some intercurrent disease, particularly from pneumonia. If this is not the case, the disease progresses until complete physical dis- ability occurs. The patient is then confined to bed; bed-sores and cachexia hasten death. Forms. — The above clinical picture presents the most frequent variety of paralysis agitans. There is also a form, in which the tremor is absent and the disease is then recognized by the rigidity, f acies and attitude. In another variety the tremor alone is present or else the rigidity is extremely slight. In some cases the tremor is not only passive, but also intentional (similar to that of multiple sclerosis). There are also cases in which instead of flexion of the trunk and extremities there is extension. Diagnosis. — The symptoms as a rule are so typical that an error in diagnosis is rare. The variety without tremor may present some difficul- ties. The tremor may lead sometimes to an erroneous diagnosis, especially when besides being passive it is also intentional. Multiple sclerosis should be then thought of. But in the latter disease there are also nystag- mus, scanning speech and absence of passive tremor. In paresis the tremor is also intentional, but there are also changes in speech, in the pupillary reflexes; finally mental symptoms. A senile tremor is usually passive and intentional, but it is not asso- ciated with other symptoms characteristic of paralysis agitans. Besides, in the senile form the head is generally affected early. Hysteria may simulate paralysis agitans by its tremor. In one of my cases it was limited to one hand, but the movements were somewhat of wider range, they were present when the arm was at rest and upon volun- tary movements; the characteristic attitude, f acies and gait of Parkinson's disease were absent. Organic hemiplegia may sometimes be accompanied by a tremor on the same side and thus simulate a form of paralysis agitans in which the tremor is confined to one hand. In the majority of such cases the diagnosis will not be difficult in view of the characteristic symptoms of both affec- tions, but there are cases which present almost insurmountable difficulties in differentiating them. Pathogenesis. — The post-mortem findings present nothing definite as to the nature of the disease. While some autopsies are negative, others show perivascular sclerosis in the gray and white matter of the brain or spinal cord. The most recent work on the subject is that of G. Maillard, who considers the disease as due to arteriosclerotic changes in the red nucleus. On the contrary a great many observers found changes only in the muscles, viz. nuclear proliferation in the sarcolemma, atrophy of AKINESIA ALGERA 533 some fibers, diminution of muscle-spindle, and they place the disease among myopathies (see this chapter). In the peripheral nerves increase of interstitial tissue and slight degenerative changes in the fibers have been found in some cases. Some writers believe that the disease is due to some toxic agent pro- duced by the parathyroid bodies (Lundborg, Berkeley and others). They base their opinion on this observation that the symptoms following parathyroidectomy resemble much those of paralysis agitans. Until a solid anatomical basis is found paralysis agitans should be considered as a functional nervous disease. This is the opinion of the majority of authors. Etiology. — The exciting causes are traumatism and emotions of a depressive character. A neuropathic tendency plays an important predisposing part. The disease affects both sexes, but men more frequently than women. The usual age at which it occurs is beween forty and sixty, although it may develop before twenty, as some records show. Treatment. — The tremor is sometimes ameliorated by trepidation in a carriage or train. One of my patients obtained great relief by riding on a train two hours every day. For the same reason I treated another of my patients with a very frequently interrupted faradic current and succeeded in diminishing the intensity of the tremor. Internally hyoscine hydrobromate in gr. i/ioo doses two or three times a day relieves sometimes the tremor as well as the rigidity. Among other drugs may be mentioned: cannabis indica, codein, opium, arsenic, bro- mides, veratrum viride. Warm Baths are useful to control the rigidity. Massage gently applied may do some good. Systematic exercises sometimes give satis- factory results in decreasing the rigidity. Rest, which is so beneficial in other neuroses, is contraindicated here. However violent exercises or undue fatigue must be avoided. According to the latest researches, particularly of Loeb and J. B. MacCallum, also of W. G. MacCallum and C. Voegtlin, there is a relation- ship between various twitchings and calcium metabolism and the function of the parathyroid glands. There is consequently a therapeutic indi- cation for the use of calcium salts and of parathyroids in paralysis agitans. AKINESIA ALGERA Under this name Moebius in 1891 described a symptom-group char- acterized by an inability to move about because of pain. If some slight movements are possible, the condition is called "dyskinesia algera." 534 AKINESIA ALGERA At first only forced movements are painful, but gradually the most insignificant displacements become intolerable. The pain appears upon voluntary movements, persists after the movements ceased. Little by little any movement becomes impossible. There is, however, no genuine paralysis. Pain is present not only in the muscles executing movements, but it extends to the remotest parts of the body. Thus headache may develop. The latter may appear upon the least mental exertion. Sometimes mental fatigue provokes pain in the entire body, especially in those muscles that are called upon to contract. In some cases a light causes pain (dysopsia algera). In Oppenheim's patient the sight of white objects caused pain. In analog}' with painful motions the light phenomenon can be called "painful photophobia." It is particularly noticeable upon attempts of reading. Some patients feel a fatigue in the head and severe headache upon the least effort to read, write or speak. It is the so-called apraxia algera. In Erb's case hearing caused pain, so that the patient could not converse. In another of Oppenheim's patients ingestion of food awakened pain, while there was no disease of the stomach. Xeftel described an analogous form under the name of "atremia," where the patient while lying can move around without pain, but as soon as he attempts to get up, he suffers in the back and head. Course, Duration, Prognosis. — In the majority of cases the disease is rebellious to treatment and may last an indefinite time. Ameliorations occur, but recurrences are frequent. Complete recovery is also possible. Mental disturbances, as delusions and hallucinations, not infrequently develop in the course of the disease. Some patients die in a fully developed psychosis. The prognosis is bad in the majority of cases. Etiology and Pathogenesis. — Functional nervous diseases (neuras- thenia, hysteria, hypochondria) play a very important predisposing role. Their association with akinesia algera is quite frequent. The consensus of opinion is that the condition is a neurosis and should be placed alongside other well-known functional nervous diseases, that the pain is central with complete integrity of the nervous tissue. It is a fixed sensation, a disease of attention, a painful hallucinatory obsession. Treatment. — Suggestion and autosuggestion are the main elements of treatment. What a patient can accomplish by exercising his will power and with a continuous effort to help himself can be seen from Erb's case, in which the patient after remaining in bed nineteen years finally learned how to convince himself that his trouble was not real, that it was only hallucinatory. The patient improved considerably. HEADACHE 535 Reeducation of movements with a great deal of persistence and per- suasion tactfully carried out, aided by gentle massage, can in some cases accomplish much good. Oppenheim's patient mentioned above recovered by wearing blue glasses, also by taking arsenic. HEADACHE Cephalalgia Headache is not a disease but a nervous symptom accompanying various diseases of various organs. It is a very frequent phenomenon and in the majority of cases has no special diagnostic significance. Some- times, however, it is so conspicuous by its intensity and character that its diagnostic value is enormous. Diseases and Conditions that Cause Headache 1. Circulatory Disorders. Anaemia, Hyperaemia. — In all forms of anaemia headache is a common occurrence. It is particularly observed when an effort, is made by the patient. In aortic diseases cerebral anemia occurs. The headache is then accompanied by vertigo. In passive hyperaernia pressure on the neck (tumors, tight collars, etc.), in active hyperaernia certain drugs (caffeine, nitroglycerine, alcohol), undue physical and mental effort, violent emotions, overheated room, cardiac diseases, especially mitral lesions, are the causes of pain in the head. 2. Meningitis (localized or diffuse) and meningeal hemorrhages, also acute encephalitis. 3. Toxaemia in infectious diseases. Perverted metabolism, such as met with in diabetes, gout, chronic rheumatism, nephritis, albuminuria, uraemia, gastro-intestinal disorders (constipation); intoxications, such as lead, alcohol, tobacco, opium, carbonic acid gas. 4. Syphilis through a degenerative condition of the blood vessels or gummatous deposits in the meninges. 5. Increased Intracranial Pressure through tumors, aneurisms of the brain (see Tumors of Brain). 6. Hysteria, Neurasthenia. 7. Traumatism of the head. 8. Diseases of the skull and of its cavities (nasal, frontal, etc.). 9. Reflex irritation, such as errors of refraction, naso-pharyngeal adenoids, diseases of genitalia, etc. Pathogenesis. — It is generally conceded that headache is directly due to an irritation of the sensory filaments distributed in the meninges of 536 HEADACHE the brain, especially the dura. In organic cases the irritation is caused by some intracranial disease. In the functional cases some irritant (toxic or other) circulating in the blood vessels reaches the sensory ends of the meningeal nerve-supply. Character of Headache in Various Diseases 1 . Headache due to anaemia, chlorosis or loss of blood is usually diffuse and accompanied by a sensation of pressure. It is severe and aggravated upon an effort. Vertigo is frequently present. In hyperemia (passive or active) the headache is diffuse, continuous and of a throbbing nature. Horizontal position aggravates it. In cerebral congestion caused by a cardiac lesion, there is a venous stasis and aedema of the brain. The headache is accompanied by somnolence and even delirium. 2. In Meningitis the headache may be localized or generalized. It is usually continuous and presents exacerbations. In this disease the headache is of diagnostic importance, especially in the epidemic cere- brospinal or tubercular form of meningitis, in which it is a very early symptom and of great severity. In tubercular meningitis headache is less intense than in the epidemic meningitis. In the latter the pain extends down to the neck and spine. In both forms the headache is persistent. In Pachymeningitis the pain in the head is at first localized. 3. In infectious diseases headache appears in the prodromal period and usually persists through the entire course of the disease. It is usually dull and continuous. In influenza the headache is diffuse and particularly marked in the frontal sinuses. This disease is frequently followed by headache of unusual severity. Headache caused by poisons is usually frontal, dull and quite severe. It persists even after the other symptoms have disappeared. 4. Imperfect metabolism of gastro-intestinal origin (constipation) produces a temporal or occipital headache. 5. Syphilitic headache is mostly nocturnal in character. It is dull, continuous, with nocturnal exacerbations. It may be general or confined to one side of the skull. It is frontal or parietal most frequently. In the latter case the skull is tender on percussion. Headache is the earliest symptom of cerebral syphilis. It is due to involvement of the meninges by gummata. It is usually relieved by antisyphilitic remedies, but recurs readily. HEADACHE 537 6. Headache is a very significant symptom in intracranial diseases, as tumor, abscess, etc. It is of unusual severity and persistent. Percus- sion or deep pressure on the skull provokes pain. In case of abscess due to an ear lesion, a disease of the nose, of frontal sinus, etc., the pain will be localized in the corresponding regions. In tumors of the cerebellum the headache is dull, boring and sometimes sharp, but always intense. It is localized frequently in the occipital region. 7. In Neurasthenia the headache is in the form of continuous pressure or constriction; it resembles a sensation of a tight iron band around the head. It is usually diffuse, but it may be also localized. Neurasthenics often complain of various distressing sensations in the head, as heat, emptiness or else fullness, which alternate with or accompany headache. In Hysteria the headache is in the vertex. It is boring and localized, as "clavus," viz. a sensation of a nail being driven into the head. 8. In traumata of the head and diseases of the skull the pain is usually circumscribed and corresponds to the injured or diseased area, although it may spread from the latter and become diffuse. 9. Reflex Headache is quite common. When it is due to errors of refraction, it is frontal or temporal. Nasal diseases give headache in the vertex or temporal region. Persistent headache is present in diseases of the auditory apparatus. A thorough examination in each case of head- ache for possible reflex causes is necessary. Treatment* — Determination of the cause of headache is the first indi- cation before the treatment is instituted. The above described etiological factors should be borne in mind. Every case must be scrupulously ex- amined, as the complete removal of the headache presents frequently great difficulties. As soon as the cause of headache is ascertained, all medical or surgical measures should be employed for its prompt removal. In cases with circulatory disturbances the latter must be relieved. In anaemia rest, absolute or partial, according to the case, is necessary. Good nutritious food, iron and arsenic are appropriate. In hyperaemia, besides the removal of the original cause, purgatives are indicated. In meningitis purgations and local abstraction of blood are useful. In gastro-intestinal disorders their immediate correction is necessary. Regulation of diet, avoidance of nitrogenous food, of intoxicants, light but nutritious food according to the case, daily evacuations, also administra- tion of hydrochloric acid in case of diminished secretion in the stomach, and of an alkaline in case of increased secretion of hydrochloric acid, ad- ministration of a stimulant stomachic in case of sluggish digestion — all these means are indicated for treatment of headache. Headache caused 538 HEADACHE by certain poisons will be treated by measures of elimination and anti- dotes, also by removal of the patient from exposure to the poisons. In cases of lead poisoning besides systematic elimination, iodides are beneficial. Symptomatic Treatment of Headache. — Relief of pain can be ob- tained from coal-tar products. Aspirin, sodium salicylate, antipyrin, bromides, are useful. A combination of aspirin, gr. v, phenacetine, gr. ii, and caffein citrate, gr. j, administered every two hours, has given me very satisfactory results. In case of malaria quinine is advisable. For head- ache of syphilitic nature salvarsan, mercurials or iodides or both will give relief. Local application of extreme heat or cold, counter-irritation, appli- cation of menthol, alcohol, chloroform, ether, may give some relief. Complete isolation with absolute rest sometimes gives relief. In organic headache (tumor, abscess, etc.) surgical measures are the only means, as it is unusually rebellious to internal medications. Morphia should be avoided, as the pernicious habit is easily acquired. In infectious diseases headache is caused by a slight inflammation of the meninges, which is indicated by increase of pressure and of albumen in the cerebro-spinal fluid. In such cases a lumbar puncture, and with- drawal of 10-20-30 c.c. of cerebro-spinal fluid, may give considerable relief. Recent observations of Roger and Baumel (Revue 1 , de Med. No. 1, 1 91 3) are very conclusive. Muscular or Indurative Headache This form of headache is due to a rheumatic infiltration, a chronic myositis of the muscles of the neck. The cervical muscles are swollen and tender to touch. The tenderness exists at the insertions of the ten- dons, spinous processes, mastoid processes, clavicle and the muscles themselves. In subacute myositis, the swelling of the muscles has an elastic consistency. In chronic myositis hard nodules are felt in the muscles. The skin covering the swollen muscles is infiltrated and thick. Histological studies show that the infiltrations are due to a proliferation of the connective tissue, at first cedematous, later fibrous and accompanied by endoperiarteritis (Lorenz and Stockman). It is therefore a trophic disturbance. The above mentioned tenderness is present not only in the insertions of the tendons but also in the infiltrated masses. Muller (Deut. Ztschr. f. Nerv., 19 10) calls attention to hypertonicity of the affected muscles: the muscles of the neck are rigid and when the patient wishes to turn his head, the entire body moves with it. The pain produced by muscular induration MIGRAINE 539 may be diffuse and affect the entire head, or migrainic affecting one side of the head or else neuralgic. These three varieties may alternate in the same individual. Pathogenesis. — The headache can be explained either by an involve- ment of the sensory fibers passing through the infiltrated muscles, or by irritation of the sympathetic. In fact Norstrom and Hartenberg observed in migrainic patients a swelling of the upper and middle cervical sympa- thetic. It stands to reason that in swelling of the muscles of the neck the sympathetic is being irritated, hence the headache. Miiller also believes that the swollen muscles compress the jugular veins, the return circulation is interfered with, a cerebral congestion is the result, hence the headache. Hartenberg believes that the chief cause of these muscular infiltrations lies in an insufficiency of arterial, venous and lymphatic circulation which is the result of an insufficiency of muscular activity. Cold, which decreases the nutritive and circulatory conditions, is capable to produce the lesion. Treatment. — Avoidance of cold, general hygiene, hydrotherapy, are measures not to be neglected. Galvanism applied to the affected muscle in the beginning is very useful. Massage should be employed only after the acute symptoms have subsided; it is contraindicated when the muscles are very tender. Internally cannabis indica has been advised by Harten- berg in very rebellious cases. Migraine. Hemicrania Under this name is known a variety of headache which occurs in paroxysms, is confined to one side of the head, and is accompanied by nausea, vomiting, vertigo and ocular phenomena. Symptoms. — The onset is usually, but not always, preceded by prodromal symptoms. They may be either depression, apathy, somno- lence or, on the contrary, exaltation with a ravenous appetite. The pain sets in ordinarily in the morning, when the patient gets .awake. At first it is dull, but gradually increases as the day advances. It soon becomes severe and even intolerable. The patient compares it to a tearing, breaking, boring. The least motion of the head or the act of coughing, sneezing, exaggerates it. It is confined mostly to one side of the head and to the left most frequently. It may also spread to the other side. It may also alternate in various attacks. It may appear first in the orbital or temporal region and from this point spread to the entire half of the head. During the attack an irritation of any of the special senses aggravates the pain. A loud sound, a sharp odor, a bright light, become intolerable. 540 MIGRAINE For this reason the patient instinctively isolates himself, closes up the windows, the doors, and seeks quietness. When the headache reaches the maximum, vomiting ocurs. This is a frequent symptom and it may take place also at the onset of an attack. It is accompanied by vertigo. The patient rejects a bilious, mucous fluid. It is brought on by the least amount of fluid or food and sometimes it occurs before breakfast. There may be several attacks of vomiting. Sometimes an attack of vomiting ends the attack of migraine. The patient then feels considerably relieved and the headache rapidly disappears. In a large number of cases the final vomiting spell is followed by a deep sleep. When the patient gets awake, he is totally relieved and feels well. Sometimes, however, there is a feeling of lassitude and a dullness in the head which persist for several days. As accessory symptoms of migrainic attacks can be mentioned lachry- mation, unilateral or bilateral, sweating, polyuria, coldness of the extrem- ities and exceptionally hemorrhages. Forms of Migraine i. Ophthalmic Migraine. — It is characterized by visual disturbances at the onset of an attack. The most important among them is a scotoma (scotoma scintillans) . It is a dark-grayish spot in the visual field, sur- rounded by a bright border of various colors. Sometimes instead of a scotoma the visual field is covered by glaring zig-zag lights. In other cases there is a distinct blindness of the visual field in the form of homon- ymous hemianopsia. There may be also a transitory amaurosis, photo- phobia with interocular pain. In some cases the migraine may be accom- panied or followed by temporary ophthalmoplegic symptoms such as strabismus, diplopia, ptosis, loss of accommodation. In exceptional cases they remain permanent. Ophthalmic migraine may be associated with motor, sensory and psychic phenomena. A paresis of the side opposite to the seat of head-pain, paraesthesias on one or both sides of the body are sometimes observed. Aphasia (motor) with agraphia is occasionally observed. One of my patients had three attacks of a mild right hemiplegia with slight aphasia. During these attacks the right knee-jerk was increased and the paradoxical reflex was evident. These manifestations lasted but a few hours. Cerebellar ataxia with its characteristic disturbance of equilibration was observed by Oppenheim. All these phenomena are usually transitory and last a short lime; MIGRAINE . 541 they may precede the headache and vomiting or persist during the entire attack of migraine. The psychic symptoms are various. There may be only a temporary amnesia, visual hallucinations, depression, apathy, which disappear with the attack. There may also develop true psychoses, as Krafft-Ebing, Mingazzini and myself have shown. In my recent study {Journal of American Medical Association, January 5, 1907) of the subject, I described twelve personal cases of typical psy- choses observed in migrainic attacks. In all of them I have invariably found three mental states, viz. (1) Confusion, (2) stupor, with hallucina- tions and unsystematized delusions, and (3) delirium. The hallucinations were mostly visual, although auditory and gustatory were also found in some of the twelve cases. The confusional state predominated in all my patients. It was quite frequently accompanied by illusions of identity, incoherence of thoughts and disturbance of orientation. The delusions were of a fleeting character. In the majority of cases the mental symptoms developed during the attacks when the headache reached its climax and disappeared with the headache. 2. Hemicrania Sympathico-tonica (White Migraine) . — It is char- acterized by symptoms of irritation of the cervical sympathetic nerve, viz. pallor of the integument of the head on the side of the pain; the temporal artery is of high tension, the local temperature is lowered; there is also retraction of the eyeglobe and mydriasis. 3. Hemicrania Sympathico-paralytica (Red Migraine) is characterized by symptoms of paralysis of the cervical sympathetic nerve, viz. redness of the integument of the head on the affected side, lachrymation, photo- phobia, contraction of the pupil, elevation of the local temperature, uni- lateral hyperhidrosis; the temporal artery is distended. 4. Migraine in its Relation to Epilepsy. — It is well known that both neuroses may be associated and that the first may be equivalent to epileptic seizures. That the analogy between them is considerable can be seen from this fact that in both the attacks are sometimes preceded by auras (motor, sensory, psychic), that the onset may be sudden, that an attack is followed by a sense of exhaustion and sometimes by unilateral paralysis or paresis. Finally the abortive cases of migraine (see below) are almost identical to attacks of petit mal. 5. Abortive Migraine. — It consists of incomplete attacks (without vomiting or vertigo) or else sudden attacks of vomiting without headache. Sometimes it is only an attack of vertigo. This form alternates frequently with the typical attacks of migraine. Course, Duration, Prognosis.— A typical attack commences in the 542 MIGRAINE morning, gradually increases in severity and terminates in the evening with vomiting and sleep. When he wakes up, he feels refreshed and well. An individual attack may last from a couple of hours to twenty-four or forty-eight hours. In some patients there is a great regularity in the periodicity of attacks. In such cases it comes on once every month or every two months. In women it may coincide with menstruation. In other cases the attacks are irregular and develop only after some excess (alcoholic or other) or after some indulgence in eating. The disease lasts many years or even the entire life. Usually with advent of old age it decreases in intensity and disappears. Sometimes an intercurrent disease, a trauma or shock, makes migraine disappear completely or for many years. In such cases some other neurosis (hysteria, epilepsy, etc.) develops instead of it. In some patients after many years of periodical and regular attacks the interval between them becomes shorter and shorter and finally the headache becomes continuous. A sort of migrainic status is thus estab- lished. The Prognosis is unfavorable as to recovery, but not to life. Diagnosis. — The symptoms ordinarily are typical enough for a correct diagnosis. In cerebral tumors there are also severe headache and vomiting, but there is no interval of "well being" characteristic of migraine. Besides, the changes in the eyegrounds will aid in the diagnosis. Tabes and Paresis may present migraine as one- of their initial symptoms. Syphilitic and malarial migraine will be promptly relieved by mercurials and quinine, respectively. The diagnosis between ophthalmic migraine and neuralgia of the ophthalmic branch of the trigeminus is not always easy. The fol- lowing differential signs may be useful. In migraine there is nausea, vom- iting. In neuralgia they are absent. In migraine the pain is continuous for several hours. In neuralgia it is in paroxysms. In migraine remissions are of long duration — hours or days. In neuralgia they are very brief. In migraine photophobia, but not in neuralgia. In migraine scotomata, but not in neuralgia. In migraine the pain often spreads to the other side of the head. In neuralgia the pain is strictly confined to one side. Etiology and Pathogenesis. — The most important element in the etiology is predisposition. The disease is very frequently found to be hereditary. A neuropathic personal or family history is frequently found. An association of migraine with an arthritic diathesis is a common observation. The most recent researches in the domain of physiologic chemistry lead to the view that migraine finds its explanation in autointoxication. Whether it is uric acid or a special ferment or ptomain is not completely elucidated. The fact is that in a large majority of cases there is an ele- MIGRAINE 543 ment of gastro-intestinal disorder and that arthritic, gouty, asthmatic, obese and constipated individuals are most frequently affected. Our recent studies on the function of the ductless glands permit also to suppose that their derangement is apt to play a certain part in the causation of migraine. Suffice it to mention the fact that in pregnancy, for example, there is a functional hyperactivity of the thyroid gland and migrainic patients are frequently free from attacks of migraine at that time. General lassi- tude, anorexia, constipation, obesity, falling of hair, are observed in migrainic individuals, also in cases with diminished thyroid function. A poison, whatever its source may be, circulating in the blood vessels irritates the sensory filaments of the dura and produces the syndrome of migraine. As exciting causes may be mentioned: disturbances of digestion, excesses, masturbation, irregular sleep, physical and mental fatigue, emotions, lack of fresh air, certain odors, loud sounds. Pregnancy may provoke or arrest the attacks. Women are more often affected than men. Treatment. — A patient suffering from migraine should be energetically treated between the attacks. Proper measures taken at that time will in a great many cases succeed in increasing the intervals between individual attacks and in some cases accomplish a complete cure. As said above, migraine is due in the majority cf cases to autointoxi- cation. It is therefore toward the latter that our therapeutic efforts must be directed. Each migrainic individual should first of all be put on a special diet and follow a certain mode of living. It has been my practice to remove from the diet meats, stimulants of any kind, including tea and coffee, sweets, pastry. Starchy food is allowed only in extremely small quantities. Milk is of course desirable, but it is not tolerated by every patient; in the latter case I substitute it by either butter milk, skimmed milk, kephir, koumyss or else by plain water. In a great many cases, however, I succeeded in having the patient accustomed to milk by giving it at first in very small quantities and then very gradually increas- ing the amount. The amount of food of each meal should be moderate and taken regularly. Fruit, eggs, milk, green vegetables, crackers, cus- tards, junkets, gelatin, are the only articles allowed. As drink, plain water or some gaseous mineral water, like apollinaris, lemonade, are permissible. Saline purgatives should be administered at first twice a week and later once a week for a long period. Hydrotherapy in the form of cold shower baths of a minute's dura- tion and followed by massage twice a day is very beneficial. Daily walks two or three times a day of an hour each are advisable. 544 ' VERTIGO A quiet life, free from undue emotions, retiring early, are also necessary. Tobacco must be used very moderately and, if possible, abandoned. As to medications, a stomachic, as nux vomica together with hydro- chloric acid and gentian, taken regularly before meals is of benefit. Any dyspeptic disturbance should be corrected and treated accordingly. A thorough examination of all the organs must be made as early as possible and treated if necessary. In case of anaemia, iron and arsenic should be given. Arsenic is highly praised by Oppenheim. The urine should be frequently examined. Any diseased condition of nasal, pharyngeal and other cavities, errors of refraction, diseases of the ears, etc., must be taken care of. When an attack of migraine occurs, all. food must be immediately withdrawn, the patient put to bed, the room made dark and all noises avoided. An ice cap or else a hot water bag are to be applied to the head. Application of menthol, a spray of ether or alcohol to the forehead or temples may give some relief. The vomiting is sometimes controlled by internal administration of small pieces of ice or chloroform water. All these means should be tried before medications in every case of migraine. The following are the remedies advised for combating the pains : Coal-tar products, aspirin, salicylates, phenacetin, antipyrin. A combina- tion which has given me satisfactory results in many cases is: Aspirin, gr. v, phenacetin, gr. ii, caffeine citrate, gr. j, to be taken every hour or two hours until relieved. Aspirin and codein form a good combination. Bromides succeed sometimes when other drugs fail. In the sympathico-tonic form of migraine (see above) nitro-glycerine is useful. In the sympathico-paralytic form (see above) ergot is advisable. Amyl-nitrite in inhalations (five drops on a handkerchief) sometimes cuts short an attack. Morphia should be avoided. In the discussion on the pathogenesis of migraine its relation to dis- turbances of thyroid function was indicated. In a series of cases studied and reported by me in the Therapeutic Gazette, 1907, I showed the very satisfactory results obtained in the treatment of migraine by thyroid extract, when every other medication failed. This treatment will yield good results only in, so to speak, "thyroid cases," viz. in cases with a deficient thyroid function. VERTIGO Vertigo is not a disease but a symptom. It may be encountered in various diseases and conditions. In Meniere's disease it is the most con- spicuous symptom. VERTIGO 545 Nature of Vertigo. — The relation of the body to surrounding objects determines the so-called "sense of space." Should this relation be dis- turbed, our orientation and equilibration in the space will become irregular and vertigo will ensue. In vertigo the orientation is first lost and the loss of equilibrium follows. Orientation is the result of centripetal function. The pathway con- trolling it is composed: (i) of sensory fibers going from the periphery through the spinal ganglia and posterior columns, also direct cerebellar tract of the cord to their termination in the cortex of the cerebrum and cerebellum, respectively; (2) of auditory fibers originating in the labyrinth and ending either in the temporal lobe of the brain (cochlear nerve) or in the nucleus of Deiter in the medulla (vestibular nerve). Equilibration is a centrifugal function. Its pathway consists of fibers of the pyramidal tract and of the descending cerebellar tract, also of Monakow's bundle, which all end in the anterior cornua of the spinal cord. Disturbance in the harmonious function of those pathways or centers of orientation and equilibration results in vertigo. Diseases and Conditions in which Vertigo Occurs 1. Disorders in the Hearing Apparatus. — Any disease of the ear, from a grave affection of the labyrinth to a simple accumulation of cerumen in the external ear, may produce Vertigo. The most important form of aural vertigo is the so-called Meniere's disease. Suddenly the patient hears a noise in the ear, which is immediately followed by dimness of vision or double vision and sometimes nystagmus. Vertigo sets in at once and the patient falls. At that time he feels the floor sinking and himself revolving in a circle. He is nauseated and begins to vomit. Headache, coldness of the skin, pallor, accompany the vertigo. After the attack is over, the noise in the ear persists. If the attacks are frequent, hypoacusia or deafness gradually develops and vertigo becomes chronic. The paroxysms usually last a few minutes. They may occur every day, week, month or even at longer intervals. The disease may last an indefinite time, but when recovery takes place, total deafness is established. The disease is probably due to a labyrinthine involvement (progressive degeneration of the nerve-ends) ; a lesion of the cochlea causes deafness, and of the semicircular canal vertigo. The labyrinth is innervated by the vestibular branch of the eighth nerve. This branch is connected with the cerebellum, center of equilibrium. The ocular symptoms are due to the anatomical relation between the vestibular nerve and the nucleus of the 35 . 546 VERTIGO oculomotor nerve. To sum up, vertigo, tinnitus auris, hypoacusia, or deafness constitute the chief characteristic symptoms of Meniere's disease. Nausea, vomiting and nystagmus are not necessarily always present. Diseases of the inner ear, of the middle ear, of the external ear, of the acoustic nerve, injuries of the petrous bone, meningitis, tabes, syphilis, gout, anaemia, arteriosclerosis, may be accompanied by Meniere's symp- tom-group, but it has also been observed in persons free from any disease. In such cases a vaso-motor disturbance in the labyrinth is probably at fault. Forms of Meniere's Disease, (a) Charcot described a continuous form of the disease. The auditory disorder and the hypoacusia, also the vertiginous state are permanent, so that the patient's gait is that of cere- bellar type (gait of an inebriate). In addition to this continuous condi- tion the patient has paroxysms of the acute form. (b) In the mild form of Meniere's disease, which is the most frequent, all the typical symptoms are present but they are exceedingly slight Here the manifestations are not always referred to the real cause. Stom- ach, kidney, uterus, anaemia are frequently incriminated. (c) Incomplete forms (formes frustes) were described by Frankl- Hochwart. One or two chief elements of the disease "may be wanting. Thus we observe: a form with hypoacusia, a form without tinnitus, a form with a mild vertigo. (d) Apoplectic form is probably due to a labyrinthine hemorrhage. Its onset is sudden. It is usually accompanied by a complete and irre- mediable loss of hearing. This is the form described originally by Meniere. Loss of consciousness is sometimes present. It is usually met with in individuals suffering from syphilis, tabes, arteriosclerosis, nephritis, infectious diseases. In traumatism of the ear labyrinthine hemorrhage and therefore apoplectic form of Meniere's disease may occur. In making a diagnosis of Meniere's disease, syncope, hysterical or epileptic attacks, indigestion, should be thought of. To determine laby- rinthine involvement, the tests described in the chapter on Nystagmus must be taken into consideration (seepage 363.) 2. Disorders in the Visual Apparatus. — Ocular palsies, diplopia, nystagmus, sudden passage from obscurity to light, are common causes of vertigo, and if the latter is severe, nausea and vomiting may follow. 3. Disturbances in the Central Nervous System. — Tabes, multiple sclerosis, may be accompanied by vertigo. In the latter affection it is quite frequent and it is an indication of bulbar involvement. VERTIGO 547 In diseases of the brain (tumors) and especially of the cerebellum vertigo is one of the most important diagnostic symptoms. In cerebellar conditions the vertigo is of a rotatory type. Softening of the brain, circulatory changes, atheromatous condition of the cerebral blood vessels, are accompanied by vertigo. Diseases of the medulla, cerebellar peduncles and vestibular nerve, all partaking in the control of equilibrium, will cause vertigo. 4. Disorders of Metabolism (Toxic Causes). — Nephritis, gout, dia- betes, gastro-intestinal disturbances, migraine, are not infrequently accom- panied by vertigo. Infectious diseases, intoxications (alcohol, lead, coffee, tobacco, quinine), intestinal parasites produce vertigo. 5. Hysteria and neurasthenia, exophthalmic goiter, mental diseases, may be accompanied by vertigo. 6. Visceral Disturbances. — In cardiac diseases, especially diseases of the aorta, vertigo is not infrequently present. Dilatation of the stomach may cause dizziness, but here the latter is probably due to auto-intoxica- tion. Reflex vertigo may originate in the uterus, bladder, liver, kidney, larynx, nasal cavity. 7. Sea-sickness, traumatism, insolation are accompanied by vertigo. 8. Paralytic vertigo (Gerlier), which has been observed in Switzerland, is characterized by vertigo, weakness of the limbs and of the muscles of the neck, ptosis. It occurs in paroxysms. Nothing is known of the nature of this endemic affection. Treatment. — The treatment of vertigo is closely connected with the management of the diseases in which it occurs. An effort must be made first of all to remove the cause. In Meniere's disease Charcot's advice has given me the best results in some cases. Quinine hydrochlorate given in small doses, but frequently repeated, with milk diet and avoidance of stimulants yield very satis- factory results. The administration of quinine is based on this fact, that vertigo subsides with increase of deafness. Quinine weakens the hearing and it may therefore be useful. At first it will increase the noises of the ears and even the vertigo, but if persisted in, it will eventually relieve the vertigo. Aspirin, sodium salicylate, pilocarpine in hypodermic injections (5-10 minims of 2 per cent, solution every other day) may be useful. Purgatives, hot foot-baths and bleeding may be beneficial. Babinski obtained some relief from lumbar punctures. Krause, in 1902, and Frazier, in 1909, attempted intracranial division of the auditory nerve for relief of intractable tinnitus and aural vertigo respectively. The results have been encouraging. CHAPTER XXVI TRAUMATIC NEUROSES AND PSYCHOSES RELATION OF ACCIDENTS TO FUNCTIONAL NERVOUS DISEASES AND PSYCHOSES; MEDICOLEGAL CONSIDERATIONS The modern requirements in every sphere of human activities are highly contributory to accidental injuries. Traumata may lead to sur- gical and nervous disturbances. As we will be concerned exclusively with the latter, it is necessary first to emphasize what factors are the most frequent causes of this disorder. Among all accidents, those caused by conveyances (railroads or trolley cars) give the largest contingent of victims. Organic injuries, such as fracture or dislocation of the vertebrae, tearing of the spinal cord or of peripheral nerves, fracture of the skull, followed by tearing of or hemor- rhages in the cerebral tissue are discussed in their respective chapters. Functional nervous disorders and psychoses exclusively will be discussed here. The subject is an extremely important one for two reasons: (i) traumatic functional neuroses are exceedingly frequent; (2) the neuroses are very frequently misunderstood, and therefore a proper estimate of their value is not always given. The functional nervous diseases produced or perhaps brought out by such accidents are: hysteria, neurasthenia, chorea, paralysis agitans, amnesia. A review of the clinical pictures is necessary for a proper appre- ciation of the medicolegal questions. Hysteria. — When a collision, for example, occurs, the shock and the fright into which the passenger is thrown are sufficient causes to disturb the workings of the entire central nervous system or of its main centers. Supposing he or she during that time is thrown even not violently against the seat of the car, the anticipation alone of a possible severe injury or of a fatal injury, or else of immediate death, is capable of putting out of order the function of the nervous system. During the first few days, or even weeks, the patient still dreads that his life is in danger. The essential features of hysteria are: Sensory Symptoms. — Hyperesthesia in areas or along the spine; loss of sensations in the distant segments of the limbs (glove-like and stocking- like anaesthesias); anaesthesia confined to an entire half of the body (hemi- 548 TRAUMATIC NEUROSES AND PSYCHOSES 549 anaesthesia) ; anaesthesia of the pharynx, of the conjunctivae, of the retina. The latter will be manifested by a contraction of the visual field. The special senses may also be affected. Sudden partial blindness or deafness (without material changes in the eyes or ears) , vomiting occurring immedi- ately after the accident and continuing for several days or weeks without any relation to the food and with an excellent appetite — all these symp- toms occur quite frequently. Motor Symptoms. — Palsies, contractures confined to segments of limbs, are not infrequent, but they are never accompanied by disturbances of reflexes usually seen in organic nervous diseases. The absence or ex- aggeration of knee-jerks, toe phenomena, muscular atrophies with reac- tions of degeneration accompany organic but not hysterical palsies. The function of the viscera may also be disturbed. Hys terical aphonia, anuria, polyuria, retention of urine, are well known phenomena. In a case published by me in the Medical Record, August, 1900, a woman be- came anuric after an emotion. Psychic symptoms are quite frequent. Temporary amnesia, capri- ciousness, inconsistencies in the ideas and conduct, dissociation of per- sonality, hallucinations are possible occurrences in hysteria. Hysterical subjects are easily influenced to change their thoughts, to do certain acts, to acquire certain sensations, to execute or to adopt certain motor phe- nomena. Suggestion or auto-suggestion plays an enormous role. I have also seen cases of hysterical paroxysms following railroad accidents. A young girl of seventeen, while lying in bed, contorted herself, assumed the position of opisthotonos, was animated with a generalized tremor, so that she was unable to utter a word distinctly. These are the characteristic manifestations in the motor, sensory, and psychic spheres of a hysterical individual. Of course, not all of them are simultaneously found in every case, but when some symptoms are present in the form and intensity as described above, the diagnosis of hysteria can be made without hesitation. Neurasthenia. — This neurosis frequently follows accidents. Its main features are: physical exhaustion and undue irritability, so that the patient cannot stand the least contradiction or annoyance. The neurasthenic feels fatigue upon the least exhaustion. Mental processes are also slug- gish; the least mental effort disables him from continuing his work. The patient complains also of backache, headache, and insomnia. The symptoms are mainly subjective. The objective symptoms are very few; tremor of the hands, increased reflexes, cold, clammy skin, and the special facies. The latter is that of a tired and depressed individual and one full of anxiety. 550 TEAUMATIC NEUROSES AND PSYCHOSES Chorea, with its irregular and incoherent muscular movements rapidly following one another, is too well known to dwell upon. It does not frequently follow accidents. Within the last three years I have seen only two cases, and then there was a history of previous attacks. The shock of the collision was immediately followed by a new attack of chorea. The same remarks can be made about Paralysis agitans. In one case of a middle-aged woman the onset of the shaking palsy was traced to twenty-four hours after a trolley accident. In two other cases (a man and a woman) the shock of the accident was the direct cause of aggravation of the previously existing symptoms, which from that time kept on increas- ing in intensity. As it is well known, the disease is characterized by a continuous tremor affecting mainly one or both hands when the latter are at rest, and by a fixed attitude of the body, mask-like expression of the face. Amnesia. — This term is applied in practice to an acquired diminution or loss of memory. There are two classes of amnesias: functional and organic. The characteristic feature of organic amnesia consists of its permanency and progressive evolution. Here, by virtue of an organic alteration of nervous elements, the memory for recent events is first affected, as new impressions can no more be associated and preserved. The gradually old intellectual acquisitions become effaced. Functional amnesia is due to the profound emotional shock caused by trauma, viz. psychic trauma. The sudden commotion of the nervous system thus created disturbs the mechanism of association of ideas with regard to time and place and produces amnesia. Functional amnesia presents by far more variations than organic. It may concern only a certain group of ideas; it may be general, in which all past events are lost; it may be partial, as in cases of double personality, a striking example of which was reported by me in the American Journal of the Medical Sciences, 1906; it may be localized, when it concerns only a certain princi- pal fact of life. Sometimes, in addition to the latter, a certain period of time immediately preceding it is forgotten. We then speak of retro- grade amnesia. When the period of time following the principal event is forgotton, there is an anterograde amnesia. These two forms may be combined. Within the last three years I have seen two cases of retro- anterograde amnesia which followed a fall from a trolley car in which the patients struck their heads against the ground. In one of them there was loss of consciousness; the patients were confused for only ten minutes. A careful examination revealed in both cases no symptoms of an organic lesion. Subsequent events showed the diagnosis in ,both cases to be cor- rect, as the patients made an uneventful recovery. TRAUMATIC NEUROSES AND PSYCHOSES 55 1 Medicolegal Considerations. — In the presence of an individual who, having sustained a trauma, complains of disturbances of a nervous or psychic order, the following medical problems must be solved: (i) Is the alleged disorder genuine or simulated, and what is its nature? (2) Should the symptoms observed be attributed to the traumatism? (3) If the latter is correct, what is the degree of incapacity for work, what are the prognosis, duration, and termination of the malady? 1. The determination of simulation is as a rule not difficult. When an affection is characterized by objective signs, they will be easily recog- nized. It is absolutely impossible for anyone to simulate an anaesthetic pharynx, and anaesthetic conjunctiva, a contraction of the visual field, a genuine hemianesthesia, a genuine plus reflex. These stigmata taken together constitute the typical picture of hysteria. This malady is a well-defined morbid entity, which, except in rare cases, cannot be con- founded with any other nervous affection. It is true that an hysterical paroxysm with its screaming, laughing, and various motor and psychic phenomena, can be to a certain extent simulated, but an experienced physician, familiar with the disease, will have no difficulty in recognizing it. The psychic symptoms of hysteria are not ordinary symptoms; they present a special physiognomy, the main points of which can never be guessed by the simulator. It is the entire picture of the attack that should be taken into consideration, not individual elements. To illustrate to what errors a non-f amiliarity with the disease may lead, the following is a case in point. An attorney requested me to examine one of his clients, who claimed to have developed a hemiplegia after a broken trolley wire struck him. The physician in charge pronounced it an apoplectic stroke (there was a partial loss of consciousness) caused by the electrical current of the wire. The patient presented a limping of his left leg and some inability to use the left arm. The condition persisted almost two months. Examination showed a state of affairs totally differ- ent from typical hemiplegia which is due to a brain lesion. The knee-jerk was not exaggerated, and the abnormal reflexes (toe phenomenon, ankle- clonus) usually found in such cases were absent. The entire left side was also wholly anaesthetic. It was unquestionably a case of hysteria. The case was settled out of court, and the patient made a complete recovery shortly after he got his money. In fact, I cured him with a few seances of a strong suggestive static breeze. A woman was in a slight railroad accident. She was only thrown on the floor of the car and there was no surgical injury. When picked up she could not hold herself in an erect position nor could she walk. The legs gave way under her. The condition was pronounced grave and a diagnosis 552 TRAUMATIC NEUROSES AND PSYCHOSES of complete paraplegia due to a hemorrhage in the cord was made. I saw the patient since then on several occasions. There was no paralysis, as she could move the limbs easily while in bed, but could not use them when standing or walking. The reflexes were normal, sensations normal, and the sphincters intact. It was a case of hysterical astasia-abasia, from which she fully recovered after a course of rest treatment with good feeding. It is highly important to always bear in mind the possibility of suggestion during an examination. Suggestion is also one of the causes of hysteria. The examination therefore must be conducted very carefully. Sensations should be investigated by having the patient blindfolded. No question should be asked of her while the test is being made with a pin over various areas of the skin. There is no doubt that she will make some defense movement if the prick causes pain. Or else the patient may be asked to say "yes" the moment she feels pain or the touch of an object. No mention should be made to the patient of possibilities of loss of power in one or more limbs or segment of a limb, of loss of memory, of a grave outlook, etc. Various disturbances of function in the motor, sensory, vaso-motor, vegetative and psychic spheres may be suggested to a hys- terical patient with great facility. Far more difficulty is encountered in the medicolegal consideration of neurasthenia. This malady is characterized almost exclusively by sub- jective manifestations. When a patient, having met with an accident, complains of fatigue, backache, headache, insomnia and irritability, there are no means of verifying the presence or absence of each of these symptoms. Nevertheless, when the disease is pronounced, the general aspect of the individual will aid in forming an opinion as to the veracity of his complaints. There is usually loss in weight, loss in strength, pallor of the face, depression, cold and clammy skin, tremor. When the case is mild, the latter symptoms are absent, and then one has to rely upon the first series of subjective disturbances. I have seen time and again that, in spite of an enormous shock following for example a collision or a fall from a height, only a few subjective symptoms would be complained of. In such cases the expert's opinion before the jury and court should be expressed thus: "If the subjective symptoms the plaintiff claims to suffer from are correct, he is neurasthenic; but I have no means of verifying them." In such a manner justice is done to both sides, defendant and plaintiff. The expert's role, I believe, is to present medical facts and explain their meaning to a lay jury. The decision as to the relation of these symptoms to the accident is left entirely to the impartial judgment of the twelve men. TRAUMATIC NEUROSES AND PSYCHOSES 553 In the majority of traumatic cases hysteria and neurasthenia are combined. This facilitates considerably the problem, as objective signs are almost always present, and if there is a certain hesitancy in the mind of the physician in accepting the subjective symptoms, due credit should be given to the objective phenomena of the victim of the accident. Chorea and Paralysis agitans developed after a trauma present no difficulty in being recognized. Simulation in such cases is an impossibility and the objective symptoms are too evident to be contested. Considerable difficulty will be encountered when amnesia is the only symptom produced by the accident. If the loss of memory is associated with hysteria, the symptoms of the latter will render sufficient aid, but when hysterical stigmata are absent, the difficulty becomes very great in deciding the question whether the amnesia is genuine or not. In such cases the examination should be repeated, the individual must be tested with great perseverance and patience, he must be questioned as closely as possible and for a prolonged period of time. Great skill and tact are necessary in investigations of this sort, and in some cases one may arrive at a positive opinion. 2. The second medicolegal problem is, as I said above, to determine whether the maladies discovered in an individual are attributable directly to the accident. While a shock, physical or mental, is a frequent cause of functional nervous diseases, one cannot nevertheless be affirmative in a given case unless the state of health prior to the accident is known to the examiner. Hysteria, neurasthenia, chorea, or paralysis agitans may have existed before the plaintiff sustained the shock. It should, however, not be forgotten that if any of those affections existed before, the newly sus- tained shock will aggravate it. A person, for example, is supposed to be in the process of recovery from a neurosis. Should he at that time sustain an injury, the primary disease is likely to return and present itself in a more pronounced form than in its first attack. Individual predisposition should also be taken into consideration in giving an account before court and jury. Neuropathic individuals are more apt to suffer from any of these neuroses than normal individuals. They are predisposed, and the least shock, a comparatively slight trauma is sufficient to disturb the workings of their nervous system with the greatest facility. Individuals whose vitality, and therefore resisting power, has been lowered following a protracted infectious disease, lead intoxica- tion, alcoholism, syphilis, are inclined to respond with unusual prompt- ness to the effects of a shock. The neuroses will find a fertile soil for their development. It is consequently important to surround one's self with this precaution in giving an estimate of the degree of the individual's 554 TRAUMATIC NEUROSES AND PSYCHOSES suffering. Not only the neuropathic tendency or the existence of a previ- ous serious disease of the victim of the accident must be known to the phy- sician, but also the knowledge of the previous conduct and habits of the injured individual is absolutely necessary. Sexual or alcoholic excesses, sleepless nights, irregular moc^e of living in every respect — are all predis- posing elements for neuroses. The question of just compensation con- sequently can be decided after a complete knowledge of all factors con- cerned in any given case. In view of the fact that trauma may actually incapacitate an individual for a long time, the possibility of malingering should never be overlooked. In cases in which the symptoms are chiefly subjective there may sometimes be some difficulty in differentiating true neurasthenia from malingering. Here the knowledge of the victim's previous habits and conduct is essential. However a malingerer usually exaggerates especially objective mani- festations, such as paralysis or hyperesthesias. He will often speak of loss of consciousness during the accident and at the same time give a correct account of all happenings. Such an individual should be thor- oughly scrutinized and with a skillful manner of questioning and of elicit- ing the objective symptoms there will be no great difficulty in the majority of cases to arrive at a proper diagnosis. 3. The last proposition of our problem is to determine the degree of incapacity caused by the accident, the consequences, duration, and ter- mination of the latter. Hysteria and neurasthenia are not synonymous of simple nervousness, as some pretend. They are well-defined diseases of the nervous system. They do incapacitate for mental or physical work, but only to a certain extent.' A neurasthenic with his chronic physical and mental fatigue is certainly not able to accomplish much. The disease presents variations in its intensity. If the symptoms are marked, the patient must go to bed, as rest is the most essential element of the treatment. Then there is no question of work. When the case is mild, and the fatigue is not particu- larly marked, and the backache with the headache only occasionally dis- turb the patient, a certain amount of work can be done by the patient, and with the proper regulation of the patient's mode of living he can be made to feel quite comfortable. Hysterical patients are not very much disturbed in their daily life when the psychic symptoms are not present. One may bear the anaes- thesias, hyperesthesias, and tremor, also contraction of the visual field without being particularly annoyed. But, as a rule, there are psychic phenomena, viz. great emotionality, irritability, crying spells, restlessness, impressionableness, etc. Such patients are unfit for work. Their mental TRAUMATIC NEUROSES AND PSYCHOSES 555 concentration and application are of a very short duration. Whatever they commence, they are unable to finish. Their association of ideas is incomplete because of instability and want of depth of mental processes. For this very reason good work cannot be expected from those in whom psychic disturbances are marked. Even in those cases which are appar- ently free from mental symptoms, the least emotion or undue exertion will bring forward the above psychic manifestations so characteristic of hysteria, because the nature of the disease predisposes them to an unusual responsiveness. The degree of incapacity will depend upon whether the psychic symptoms are present or not, also upon the intensity of the latter. Chorea and paralysis agitans incapacitate for physical work consid- erably, but only partially for mental work. When amnesia occurs, a disability will ensue if it is of a generalized character. If it concerns only a certain past period of life (retrograde or anterograde form), there is no disability whatever. The individual is perfectly able to earn a living, as the memory is good for all events except that one. A proper discrimination as to degree of incapacity in all the neuroses is always possible when the above elements are taken into consideration. Not infrequently we are asked about the future of the victims of accidents. My answer is that while the neuroses are curable affections they nevertheless may last an indefinite time. The results depend upon many circumstances, viz. treatment, surroundings of the patient, previous general health, ability or inability to carry out certain instructions, outside influences, and finally the individual make-up. In exceptional cases the disease may last for years in spite of treatment. It should not be forgotten that recurrences are possible, and in fact are not infrequent. In the majority of cases the patients make a good recovery. The latter is possible in hysteria, neurasthenia, chorea, and amnesia, but not in paralysis agitans. Psychoses.— Mental disorders present a very important chapter from a medicolegal standpoint. A shock caused by an accident is likely to produce mental disturbances, but between the latter and distinct, well- defined, mental affections there is a great difference. Hysteria is, properly speaking, a psychic disturbance, and its mental phenomena described above belong by right to this chapter. They have been sufficiently emphasized on the preceding pages. Amnesia is an- other psychic phenomenon. It is also f amilar to us. When after a trauma concussion of the brain is suspected, the period of unconsciouness may be followed by some mental symptoms, viz. dullness and sometimes con- 556 TRAUMATIC NEUROSES AND PSYCHOSES fusion, inability to recall the circumstances of the accident (retrograde, anterograde or retroanterograde amnesia), inability to exercise the power of attention; disturbance of judgment, marked indecision in undertakings. All these symptoms are aggravated by insomnia which is usually present in such cases. These patients are ordinarily conscious of their mental deficiency and we observe that anxiety and worry over their condition and their future are added to the above disorder. When the immediate symptoms of brain injury are severe, there is usually a cerebral hemorrhage or fracture of the skull. A cerebral con- tusion, followed perhaps by minute disseminated hemorrhages, is apt to be followed by mental symptoms, such as vertigo, delirium, confusion, loss of memory, and coma. In such cases the patient either dies or gradu- ally recovers his mental faculties. Korsakoff's psychosis may be the result of a severe injury. We then have a marked confusion, illusions of identity, disorientation of space and time, loss of memory for recent events and fabrication. Alcoholism is usually the underlying cause (see for details page 347). In infancy and childhood the condition is somewhat different. When a child in the process of development undergoes the effect of a severe cerebral injury, the result may be very serious. If epilepsy follows, there is no doubt that the mental growth will be interfered with. Faulty cerebral development may follow a grave injury to the head, irrespective of epilepsy. The prognosis, therefore, is guarded when arrest of intel- lectual development is observed shortly after a cranial injury. The relation of paresis to traumata is a question of great importance. There are some alienists of note who believe that an injury is apt to develop paresis, others find no relation between the two. Paresis is an incurable disease; it is one of the most serious of mental affections. If its symptoms are noticed first after an accident, one "is naturally apt to attribute them to the latter. At the Congress of the French Neurologists and Alienists in 1906 this question was the subject of a special report, so important it was considered. It was discussed at length by the most brilliant minds. It is frequently brought forward as of a special import, as courts and law- yers are waiting for our decision. In the light of our present knowledge concerning especially the com- plement fixation test it may be safely said that paresis is not and cannot be caused by an accident. It is a mental disease of a slow, but progressive evolution, and characterized pathologically by a gradual degeneration of cerebral tissue. If its symptoms become sometimes conspicuous after a trauma, is it reasonable to suppose that a cerebral degeneration sets in immediately and at once produces the symptoms the development of EPILEPSY IN RELATION TO TRAUMA 557 which require a long period of morbid changes in coritcal cells? In my judgment the disease existed before the accident, and the latter served only as an exciting cause for its more rapid development. Medical records are abundant with examples of this nature in other affections. The mental manifestations of paresis, in its expansive or depressive forms, the physical signs, such as irregular and unequal pupils, the dis- turbance of the pupillary reflexes, characteristic speech, tremor of the tongue and hands, changes in the reflexes (loss or increase), apoplectiform or epileptiform seizures — it is not conceivable that these symptoms will explode shortly after an accident without being in existence prior to the accident. We are now in possession of bio-chemical means to determine the nature of paresis, so that in doubtful cases they render the most valuable aid in diagnosis. Positive Wassermann reaction of blood serum and cerebro-spinal fluid, increase of albumen in the latter, finally lymphocytosis — are all pathognomonic of paresis. The following peculiarities concerning these reactions are worth mentioning, (i) If the blood serum does not give a positive reaction, the cerebro-spinal fluid will be negative. If the cerebro-spinal fluid gives a positive result, the blood serum does the same. (2) If the serum gives a positive result, but the cerebro-spinal fluid a negative, the disease is not paresis, but cerebro-spinal syphilis. (3) The examination of the cerebro-spinal fluid is more important in paresis than that of blood serum. (4) Mercury, iodides, salvarsan may render the Wassermann reaction negative for a certain time . If after a trauma these reactions are found to be present, there can be no doubt that paresis existed before the trauma. The latter per se cannot create any of the four reactions mentioned. EPILEPSY IN RELATION TO TRAUMA It is a well-known fact that trauma may cause epilepsy. A fracture of the skull may occur without any material evidence on the scalp. In such cases the inner table alone may be injured and a fragment may press directly on the cortical layer of the motor area. In other cases there may be some cicatricial tissue with thickening of the meninges producing an irritation of the motor area. In still other cases no appreciable lesion of the motor cortex had been found and still epilepsy developed after a trauma (Raymond). According to Kocher and Pitres the cause of epilepsy in such cases is the increased pressure of the cerebro-spinal fluid. In every case of traumatic epilepsy one must hear in mind the possibility of preexisting epilepsy or of the individual being predisposed 558 EPILEPSY IN RELATION TO TRAUMA to it through alcoholism or syphilis. In such cases the trauma plays the role of a contributory factor. The following case of epilepsy is of interest fron a medicolegal standpoint. The man I was called upon to examine fell off a ladder while working at a building. He struck his head and became unconscious. In the evening of the same day he had an epileptiform seizure with generalized convulsive movements. He was taken away from work, kept at home, and treated for epilepsy. Since then his mental faculties became ob- tunded. His memory began to fail. He was unable to give an account of himself. He would make grave mistakes. Would ask for a fork when he wanted a knife; would forget his wife's name. He would try to get in his neighbor's house instead of in his own. He also had out- breaks of extreme furor, in which he would use profane language, and even strike, so that the lives of his relatives became endangered. After the explosion of this passion subsided, he had no recollection of what occurred. When I saw him months later he presented marked tremor of lips, increased reflexes, pronounced confusion, and a vague expression of the eyes with dilated pupils. He could not give me an explanation of those outbreaks of passion, of which he had no recollection. The patient had evidently been suffering from epileptic dementia. The attacks he pre- sented were psychic in form, but of epileptic nature. The question arose in court whether the trauma was the direct cause of his mental condition. A very careful search into the man's previous, life, with data obtained from his wife, who was separated from him before the accident, also from his relatives and friends, revealed the fact that while the man never had a seizure prior to the trauma, and was mentally clear as he worked in the same place for seven years, he nevertheless had a syphilitic infection and used alcohol to an unusual excess. My opinion, based upon all the facts of the case, was formulated as follows: "The trauma was an exciting cause to the epilepsy with its dementia, but the patient was predisposed to the disease. Any other cause would have produced the same condition. It is also possible that the mental affection would have developed without any apparent cause, as chronic alcoholism and syphilis are sufficient etiological factors in epilepsy." An accident may be the exciting cause of a delirium or a delirium tremens in an individual prof oundly intoxicated with alcohol. Similarly I have seen a confusional state developed in individuals with a previous alcoholic history. A thorough investigation of the personal antecedents, is absolutely indispensable in cases of this category. EPILEPSY IN RELATION TO TRAUMA 559 The previous study of trauma in relation to nervous and mental dis- orders leads to the following conclusion. In cases of railway or other injuries caused by neglect of those who have in charge the management of transportation cars, it is no more than just that the injured person should be compensated for disability. On the other hand simulation or exaggera- tion of incapacity should be condemned. The physician is indispensable to the law. In the name of justice he must be invariably reserved in his statements. His opinion must be formed after a thorough study of each individual case. He must not forget that, while some severe trau- matisms may produce mild symptoms, some insignificant traumata may cause marked disturbances of the nervous system. The degree of the disability and the prognosis of the affection vary in each individual case. The recognition of the affection, the recognition of the influence of the accident upon its manifestations, finally the discrimination of. a genuine malady from a simulated one — all these elements can be acquired only when the physician is properly prepared. CHAPTER XXVII DISEASES OF THE SYMPATHETIC NERVOUS SYSTEM TROPHONEUROSES. ANGIONEUROSES Exophthalmic Goiter (Grave's or Basedow's Disease) This affection is characterized by four cardinal symptoms: enlarge- ment of the thyroid gland, exophthalmos, tachycardia and tremor. In the majority of cases they are all present. Symptoms. — In typical cases the disease develops slowly. In excep- tional cases all or almost all the characteristic symptoms appear suddenly or rapidly. i. The most constant and earliest symptom is tachycardia. The least muscular effort or emotion brings on an attack of palpitation which may be so pronounced that the heart beat can be perceived at a distance. Arhythmia with signs of asystoly (cyanosis, oedema, vertigo) is observed sometimes. Precordial pain is frequently present. The carotid arteries are full and beat violently. The pulse is about 120 and more per minute. Examination of the heart shows nothing abnormal in most cases, although in advanced stages it may become dilated and a mitral insufficiency develops. 2. Gradually the neck begins to get large and a goiter develops. The enlargement may affect the entire thyroid gland or only one lobe. The goiter is soft and vascular. A systolic bruit is often heard on auscul- tation. The goiter is not painful, but when it increases rapidly, it pro- duces symptoms of compression and threatening suffocation. 3. Exophthalmos is very frequent, but not constant. It is usually bilateral and occasionally unilateral. It may be more marked on one side than on the other. The protrusion of the eye globe gives the face an expression of fright and anger. Closing of the eyelids is impossible. The exposure of the conjunctiva and cornea leads to inflammation and ulceration. Lachrymation is frequent. The palpebral fissure is some- times very wide because of a contraction of the levator palpebree and the patient is then unable to wink. This is Stelwag's sign. There are other signs that accompany exophthalmos. Von Graefe's sign consists of an 560 EXOPHTHALMIC GOITER 56l inability of the eyelid to follow the movements of the eyeglobe; when the eye is in the act of moving upward or downward, there is a delay in the movement of the eyelid. Mobius' sign consists of difficulty of convergence. External ophthalmoplegia has been observed. The visual acuity is diminished. Hyperaemia of the retinal blood vessels is sometimes noticed. /&L g^^ 1 1 - fjff: ; t\ Fig. 161.— Exophthalmic Goiter. The eye-grounds are usually normal. Amblyopia, diplopia, are very rare occurrences. 4. Tremor is very frequent and it is .particularly significant in the in- complete forms. It is a fine, vibratory tremor. The oscillations are rapid (eight to ten per second). It is localized or generalized. It affects more frequently the hands, but also the head, trunk and the lower extremi- ties. It is present during rest, but more marked on voluntary acts and emotion. 36 562 EXOPHTHALMIC GOITER Besides the above four typical signs there are others which may occur now and then in the course of Grave's disease. Motor Symptoms. — They are: paresis of the lower extremities or of the facial muscles, hemiplegia, monoplegia, but they are all transitory. Cramps, contractures, tetany, epilepsy, occur occasionally. The reflexes are usually unaltered, but they may be increased or decreased. Sensory Symptoms. — Pain of a neuralgic character, especially in the eyeglobes, face, neck, also in the precordial region, in the arms, is not infrequent. Vasomotor and Trophic Disturbances. — Flushes of heat in the head, profuse perspiration, slight elevation of temperature several times during the day, are quite commonly observed. The skin may be the seat of various eruptions. Urticaria, oedema, pigmentation, vitiligo, occur sometimes. Association of Grave's disease with scleroderma has been reported. Falling out of the hair is not in- frequent. Increase of galvanic reaction in the skin and various tissues is frequently observed. Nutritive changes are present. There is usually emaciation and general weakness. Urinary Symptoms. — Polyuria, albuminuria, glycosuria, occur. The first is quite frequent. Digestive Disturbances. — Anorexia or else polydipsia, vomiting, diar- rhoea are observed. Excessive salivation and j aundice are rare occurrences. Respiratory Disturbances.— Dyspnoea, dry cough, inability to execute forced inspiration (Bryson's sign) are observed. Disturbed Genital Function.— Atrophy of the organs and of the mammary glands, impotence, diminution of sexual desire, amenorrhcea, occur. Psychic Disturbances.— Restlessness, irritability, instability, changes from gay and happy mood to depression, sadness, and anger are frequently present. Insomnia is the rule. Hypochondriacal ideas frequently develop. In some cases delirium and confusion with hal- lucinations occur. Forms. — The above clinical picture is that of typical cases of ex- ophthalmic goiter. There are cases in which not all of the four chief symptoms are present (incomplete forms). Grave's disease without goiter or else without exophthalmos is not very rare. Other forms are marked by a slow development of symptoms (chronic). Others (acute) are conspicuous by rapidity of development. Association of Grave's disease with hysteria is frequent. Some- times it is seen with epilepsy and occasionally with tetany, chorea, tabes, syringomyelia. I reported a case of exophthalmic goiter associated with EXOPHTHALMIC GOITER 563 paralysis agitans {New York Med. Jour., December 31, 1904). Finally it may be also met with in diabetes, myxcedema and insanity. Course, Duration, Prognosis. — The symptom-group is rarely com- plete, but the phenomenon which is never wanting and which appears at the onset of the malady is the cardiac disturbance. The acute and chronic varieties have been mentioned. The course is rarely regular: it varies from individual to individual. Amelioration and aggravation of the symptoms occur frequently. In the majority of cases the duration is protracted. It may last many years or indefinitely. Recoveries rarely occur. The prognosis depends upon the severity of the symptoms. Anorexia, diarrhoea, al- buminuria, produce cachexia and hasten death. Sometimes death comes on very rapidly. Compression of the trachea by the goiter, asystoly, may be the cause of rapid death. In some cases an intercurrent disease leads promptly to a fatal termination. Pulmonary tuberculosis is not rare. In the most favorable cases some traces of exophthalmos, of goiter, remain. Diagnosis. — The typical form will be recognized from its cardinal signs. Difficulties will be encountered in the abnormal or incomplete forms. In such cases the constant presence of tachycardia and very frequent presence of tremor will aid in tracing the affection. Hysteria associated with chlorosis may simulate Grave's disease, but the cardiac disturbance in the chlorosis is not as continuous as in exophthalmic goiter. Simple goiter with tachycardia may also embarrass the diagnosis. It is the general picture of the disease that should always be taken into consideration while making a diagnosis. Etiology. — A neuropathic heredity can be traced in the majority of cases. Acute infectious diseases (typhoid fever, inflammatory rheuma- tism, grippe, scarlet fever, pertussis) may be followed by Grave's disease. Syphilis, lead intoxication, tuberculosis, ordinary goiter, are all predispos- ing factors. Among the exciting causes may be mentioned violent emotions, traumata, excesses (sexual and others), pregnancy. Pathogenesis. — The pathological investigations show changes in the thyroid gland, thymus, cervical sympathetic nerve and medulla. The thyroid gland may present many morbid varieties from a simple conges- tion to the most pronounced lesions. Generally there is hypertrophy of the entire gland, of one lobe or of a portion of a lobe. The vesicles are increased in size, the epithelium is hypertrophied; the intravesicular colloidal substance is usually diminished in quantity; in the parenchyma of the gland there are lymphoid masses which are considered character- 564 EXOPHTHALMIC GOITER istic of exophthalmic goiter. The connective tissue of the gland is abnormally developed. The blood vessels are dilated and their walls are thickened. The same condition has been found in the thymus. , The cervical sympathetic ganglia and nerve have also been found altered (proliferation of connective tissue, multiplication of blood vessels, atrophy of cells). In the medulla dilatation of blood vessels and hemorrhages, atrophy of the restiform bodies have been reported by competent observers. In view of such a great variety of pathological changes the nature of Grave's disease remains as yet obscure. The most favorable view held at present is that the thyroid gland is the only cause of the affection. The theory is as follows. The function of the thyroid gland consists of extracting from the system a toxic product and neutralizing it before it is thrown into the general circulation. The neutralization is performed by a special product secreted by the gland. In Grave's disease there is an excessive secretion of the gland (hyper- thyroidization) . The toxic product thrown into the circulation irritates the cervical sympathetic nerve, which then produces the symptoms of the eyes, of the heart and the goiter. This theory cannot satisfactorily explain why, if a toxin is at fault, it excites only the cervical sympathetic nerve and not also the other sympathetic nerves. It also fails to explain cases with enormous exophthalmos with insignificant thyroid enlargement, cases with enormous goiters and insignificant exophthalmos, cases of unilateral Grave's disease. On the other hand there is a number of cases on record showing that many nervous diseases become complicated with symptoms of Grave's disease when in the later stages bulbar symptoms make their appearance. Such are the observations on tabes, amyotrophic lateral sclerosis, polioen- cephalitis, pachymeningitis cervicalis, syringomyelia. Experimental phys- iology (Filehne, Bienfait, Durdufi), post-mortem examinations collected by H. Klein {Deutsche Zeitschrift f. Neurologie, 1904) show changes in the medulla and pons, especially hemorrhages. In a case observed by me (N. Y. Med. Jour., 1905) a woman suddenly developed a paralysis of the third, fourth and sixth nerves unequally distributed on both sides. A few days later she noticed a gradually coming-on prominence of both eyes. A week later a goiter with tachy- cardia and tremor began to develop. There was also von Graefe's sign (Fig. 161). All these considerations tend to prove that the phenomena of Grave's disease are due to a bulbar disturbance: the vaso-dilators of the head EXOPHTHALMIC GOITER 565 and heart which have their deep origin in the medulla and situated in the cervical sympathetic nerve, undergoing irritation, send a constant afflux of blood to the thyroid and to the retrobulbar vessels; the filaments going to the heart give rise to tachycardia. Treatment. — The first indication is to place the patient in a con- dition of keeping his nervous system free from emotions or shocks. Rest, proper hygiene, regular and quiet mode of living and removal from the usual surroundings — better in the country — are all beneficial means. He should avoid exertion, climbing stairs, mountains. The tachycardia which is always present, and which is distressing, will thus be ameliorated. All stimulants, including tea and coffee, the use of tobacco, must be avoided. Sexual intercourse is forbidden. Marriage is also contra- indicated. The diet should be nutritious, but not abundant. Milk is an ideal food in such cases. Constipation is to be avoided, but powerful purga- tives should not be administered. Internally the following remedies can be tried, but not much reliance can be placed on any of them: Bromides, salicylates, digitalis, strophan- thus, iron, quinine, belladonna, iodides and injections of iodine into the thyroid gland. Lancereaux reported very brilliant results from the use of quinine sulphate with and without ergot. Forchheimer obtained very good results from neutral salt of quinine hydrobromate in gr. v doses three or four times daily. The drug should be administered for a very long time, viz. months. In case of persistent tinnitus, which the drug is apt to produce, it is advisable to discontinue it for a certain time and then resume it. Opotherapy sometimes gives satisfactory results. Thyroid extract, thyroidin, iodothyrin, antithyroidin, of Moebius, extract of parathyroid glands, of thymus or of suprarenal capsules are administered internally. Milk of horses whose thyroids had been extirpated (Lanz), thyroidectin, viz. desiccated blood of thyroidectomized sheep, a serum prepared by Rogers and Beebe by the use of nucleoproteid and thyroglobulin from normal and pathological glands, finally injection of serum of thyroidec- tomized dogs (Ballet and Enriquez) have also been advised. Rogers and Beebe's investigations with their serum concerned a series of 480 patients in 1909. The results were as follows: 15 per cent, totally cured; 10 per cent, cured from their subjective disturbances, but exophthalmos or goiter remained unaltered; 50 per cent, improved; 17 per cent, derived no benefit; 8 per cent, died from the progress of the disease. The results are better in acute than in chronic cases. 566 EXOPHTHALMIC GOITER It is impossible as yet to form a definite opinion as to the therapeutic value of these specific preparations. Electricity, especially galvanism with the negative pole on the goiter, stabile galvanization of the sympathetic may sometimes render some service. X-ray and radium therapy are reported to be beneficial. While radiotherapy may have a favorable effect on the general health and the nervous manifestations, it has no effect on the goiter, exophthalmos and very slight effect on the tachycardia. On the other hand radio- therapy 'may produce perithyroid adhesions which may be a disturbing element in ulterior operative procedures. Moreover, X-rays may produce a thyroid insufficiency with myxcedema or else may aggravate the preex- isting manifestations, as some observations tend to show. While the medical treatment rarely gives permanent results, surgical intervention meets with somewhat greater success. Operations should not be performed in every case. In grave or advanced cases secondary organic changes develop in the thyroid, myocardium, periocular tissues, so that complete disappearance of symptoms cannot be expected. Pa- tients who reach a state of cachexia should not be operated upon. Surgical intervention is indicated in cases which continue to progress in spite of the best medical treatment; in cases in which there are evidences of compression of the trachea, oesophagus and recurrent laryngeal nerve; in cases with a very acute onset; in cases with grave nervous and mental symptoms; finally in cases with a marked lymphocytosis according to Kocher. In the majority of cases exophthalmic goiter is rebellious to medical treatment. However, an attempt with internal medications should be made almost in every case. Horsley recommends about six weeks of such treatment and if the results are meager, surgical intervention should not be postponed. On the other hand the best results are obtained if the patients are operated on very early in the course of the disease. Thyroidectomy and ligation of the thyroid arteries are very serious operations and exceptionally give favorable results. Complete recoveries have been reported. If under recoveries is understood a total disappear- ance of all subjective and objective symptoms, they are exceptional. In the majority of cases considerable amelioration is obtained. Thyroidec- tomy should always be partial in view of the great danger following removal of the parathyroid glands. In ligation of thyroid arteries there is danger of including the recurrent laryngeal nerve while tying the in- ferior thyroid artery. The danger is in producing laryngeal paralysis and inhalation pneumonia. Ligation of the superior thyroid artery is, according to Mayo, safe. MYXCEDEMA 567 Removal of the cervical sympathetic ganglia with their cord gives, according to the statistics of Jonnesco, the most satisfactory results. He advises bilateral extirpation of all the three ganglia with the cords. The results obtained by him have been uniformly perfect. Chalier (Lyon Chirurgical, 191 1) in an extensive study confirms Jonnesco's statement, viz. that operations on the sympa- thetic are in every respect superior to thyroidectomy. All reflex irritation of the sym- pathetic from some remote affec- tion, as fibroid uterine tumors, nasal polyps, etc., should be re- moved. If the syndrome of Grave's dis- ease occurs during the course of a spinal or other organic nervous dis- ease, no operation should be per- formed. MYXCEDEMA (CACHEXIA STRUMIPRWA) This disease was first described by Gull in 1873 and in 1877 by Ord, who gave the above name. Symptoms. — There are three cardinal symptoms that charac- terize the affection, viz. (1) swell- ing of the skin, (2) atrophy of the thyroid gland and (3) mental deficiency. 1. The swelling, which is due to a mucous or mucoid infiltration of the skin, is first noticed on the face. The latter is large, round and its skin is of a yellowish tint (wax-like), dry and without hair. On palpation the skin feels hard, not depressible and thick. The nose is enlarged, the eyelids are swollen and droop, the lips are thick, the forehead is wrinkled. The facies is without expression and appears stupid. In other portions of the body the skin is equally thick. The trunk is less affected than the extremities. The fingers and toes are large, the nails are thick, hard and brittle. 100 i i®S& 90 J r^mmPQk •'** ■Bl 80 J Mfr$*j$k. 70 1 JhHb 60 J tBL 50J JUK 1 H ■He ' ■895 Fig. 162.— Myxedematous Idiocy. (Bourneville.) 568 MYXCEDEMA The hair of the body atrophies and falls out, perspiration ceases, the skin is dry and scales off. In the subclavicular region and in the axilla pseudo-lipomatous masses are found. The mucous membranes are equally swollen, pale and dry. The tongue is thickened and if the pharyngeal and laryngeal mucous mem- Fig. 163. — Infantile or Congenital Myxcedema. branes are swollen, dysphagia and changes of the voice are present The teeth also suffer in their nutrition and fall out. 2. The thyroid gland is usually atrophied. Very exceptionally it is hypertrophied. 3. The mental condition is marked by deficient intellect. The patient is somnolent, apathetic, his answers are slow, memory decidedly impaired. He is usually irritable. In some cases delirium and hallucinations develop. MYXCEDEMA 569 Other Symptoms. — The heart-beat is weak, the pulse is small and irregular. Hemorrhages, and especially uterine, are frequent. The temperature is below normal. Constipation is the rule. Patients often complain of pain in the extremities and neck, of deafness, tinnitus aurium, vertigo and headache. The reflexes are not altered. There are no objective marked sensory disturbances except some diminution of sensations. The patient is unable to do any work. He is slow in words, actions and thinking. The gait is hesitating. The torpitude is general. Forms. — The clinical picture just given presents a fully developed type of myxcedema of adults. But there are also incomplete forms in which only some features of the typical form are present and in which thyroid medication proves to be beneficial. Infantile or Congenital Myxcedema. — What characterizes this form is the arrest of physical and intellectual development. Physically these patients are dwarfs. The head is narrow in front, the anterior fontanelle persists. The dentition is delayed. The mouth is open, the saliva dribbles continuously. They begin to walk and speak late. The genital organs are not developed. The usual signs of pubes- cence are absent. These patients eat abundantly and are much consti- pated. Intellectually they are idiots. When they grow up, they have the appearance as described in the first chapter, except that intellectually they show they have never developed. Such children are usually born from tubercular, alcoholic or syphilitic parents. Infantile myxcedema may present various degrees. It depends upon the amount of thyroid gland preserved. Brissaud's "infantilism" be- longs to this class. Here we find an arrest of physical development (small size, undeveloped genitalia, dry skin without hair, etc.), but there is some degree of intellectuality sufficient for certain occupations. These individuals are small adults. They constitute the so-called "partial myxcedema" cases. Cases of infantilism described by Lorrain do not enter here. They are small individuals, but do not possess the attributes of infancy. MYXCEDEMA STRUMIPRIVA (OPERATIVE MYXCEDEMA ) This form may follow extirpation of the thyroid gland or of a portion of it. The symptoms begin to develop some months after the operation, although they may appear sooner. The initial symptoms are: a lassi- tude and weakness in the limbs. Gradually the skin becomes infiltrated 5/0 • • MYXCEDEMA and the hair begins to fall out. At the same time the characteristic mental hebetude (see above) makes its appearance. The intensity of the symptoms varies^from case to case and depends also upon the age at which the operation is performed. The younger the individual, the graver the symptoms are. A point of great importance is the fact that partial thyroidectomy is followed by far less grave symptoms than total extirpation of the gland. Gley and Vassale have called attention to the great relationship of the parathyroid glandules to physical and mental conditions. Preservation of these glandules is never to be forgotten in operations on the thyroid gland. Sporadic Cretinism. — It presents, physically and mentally, the same infantile myxcedematous type as described above. The only difference is found in the thyroid, which is here hypertrophied instead of being atrophied. The hypertrophy coexists with insufficient function: instead of glandular tissue there is proliferation of sclerotic tissue. Consequently from the standpoint of function it is identical to an atrophic thyroid. This type is particularly observed in certain localities of Switzerland, Austria, Italy and France. Climate and geological conditions, the water and the air, have probably to do with the disease. Course, Duration, Prognosis. — In the typical form the course is slow and progressive. The physical and intellectual infirmities gradually in- creasing lead to a profound deterioration. The skin gets very hard, the functions of the viscera are more and more disturbed. The temperature goes down, the secretions become less and less. However, remissions occur, but these periods of amelioration are only temporary. Eventually the mental hebetude increases and a pronounced cachexia sets in. Death is the ultimate result. Death may occur from some intercurrent disease, among which pulmonary tuberculosis is the most frequent. The prog- nosis is therefore unfavorable. In the operative myxcedema, as men- tioned above, the prognosis depends upon the integrity of the parathy- roid glandules and upon the amount of thyroid gland removed and finally upon the age at which the operation is performed. Grave immediate symptoms may also occur after the operation, viz. convulsive seizures and tetany. The latter particularly may persist for months or cause death. These phenomena are due to the removal of the parathyroid glands. In infantile myxcedema the individual cannot be considered diseased; his health is as a rule good. His condition does not lead to cachexia. He simply presents a morphological or functional anomaly which does not compromise his existence. Diagnosis. — In the majority of cases the characteristic symptoms of MYXCEDEMA 57 1 the skin, of the thyroid gland, of the intellectuality, of the attitude, .are sufficiently typical for the recognition of myxcedema. Some difficulty may be encountered in the diagnosis of the incomplete forms. Infantile myxcedema will be recognized from the arrested development; there is no cachexia. Etiology. — Heredity plays a certain role. Alcoholism, tuberculosis, diabetes and syphilis in the parents are considered as predisposing causes. Pregnancy, lactation, menopause, which are accompanied by conges- tion of the thyroid, perhaps have a predisposing influence. Direct' causes are: infectious diseases which may lead to an inflamma- tion of the thyroid gland ending in its sclerosis and atrophy. Tumors of the gland may produce the same condition. Acquired myxcedema may occur at any age, but more frequently in children than in adults. Females are more predisposed than males. In infantile myxcedema the cause of congenital absence of the thyroid gland is unknown. Pathogenesis. — Pathological investigations show that in congenital infantile myxcedema the thyroid gland is totally absent. In the adult form of myxcedema there is degeneration of the normal tissue of the thy- roid gland, proliferation of connective tissue. The subcutaneous tissue is infiltrated with mucin. There is hyperplasia of adipose tissue. The suppression of the function of the thyroid gland is the cause of the disease. Normally the thyroid elaborates a special substance which, thrown into the circulation, destroys the usual toxic elements of the organism. When the thyroid is diseased, its antitoxic substance is either absent or perverted. The result is myxcedema. Internal administration of thyroid extract supplies the organism with the necessary elements and amelioration of symptoms follows. That the parathyroid glands partici- pate in this useful function there can be no doubt (see above). Treatment. — The foregoing remarks lead logically to the therapeutic indication. Thyroid extract administered internally is almost the only remedy for the condition. As it may produce tachycardia, cerebral irritation, headache, insomnia, dyspncea and sometimes albuminuria, its administration should be watched. It is advisable therefore to com- mence with small doses — for an adult gr. j or ii t. i. d. and for a child a fraction of a grain. The initial dose can be gradually increased. General hygienic measures should not be neglected. A vegetable diet is preferable to any other. Good results have been obtained with thyroid not only in the acquired form, but also in the congenital form. 572 ACROMEGALY ACROMEGALY This disease was first described by Pierre Marie in 1885. Symptoms. — The essential and characteristic signs of the disease are enlargement of the osseous and other supporting tissues, especially notice- able in the distal ends of the extremities (hands, feet) and the head. The hands become first affected. They are thick and wide. The bones and the soft parts are involved. The skin is hard and free of oedema. The thenar and hypothenar eminences are very large. The fingers are enormous, but not deformed and their function is preserved. The fingers may be only wide (transverse type) but also long and wide (longitudinal Fig. 164. — Acromegaly. (Marie.) type). The size of the hand is out of proportion with the rest of the upper limb which is only slightly enlarged. The feet are equally enlarged. The toes and the soft tissues are thick and wide. Here again the contrast between the size of the feet and the legs is striking; the latter are only slightly involved. On the head the occipital protuberance is prominent. The cranial bones are irregularly thickened. It is particularly the face that shows hypertrophy. It is elongated, the forehead is low, but the orbital and zygomatic arches are very prominent, the eyelids are thick- ened. The nose is enormous. The lower jaw is very much hypertro- ACROMEGALY 573 phied, the chin is projected (prognathism) and large. The lips are heavy and the lower one is everted. The tongue is thick (macroglossia) and broad. The palate, tonsils, uvula and larynx are all hypertrophied. The vocal cords are thick. The mucous membranes are hypertrophied so that difficulty of deglutition may occur and the voice is deep. The skin of the face is dry and brownish. The hair of the head is thickened. The vertebral column presents a cervico-dorsal kyphosis. The sternum is thickened and projected; the clavicle and the ribs are large; the costal cartilages are ossified. Besides the deformities there are other constant symptoms: Headache and Amenorrhea. They often appear at the onset of the disease. Other Symptoms. — Not infrequently ocular disturbances occur, viz. scotomata, diplopia, strabismus, diminution of visual acuity; also optic neuritis and optic atrophy. Blindness and hemianopsia with Wernicke's pupillary reaction are occasionally observed. In a large number of cases simple amaurosis with pupillary immobility is observed. The viscera sometimes participate in the hypertrophy. Cardiac hypertrophy is particularly troublesome. Palpitation, arhythmia and dyspnoea, also attacks of syncope are quite frequent. The abdomen is large, the mammary glands and the uterus are atro- phied. The male genitalia are enlarged, but impotence is present. In the female the clitoris may undergo hypertrophy. Sterility is frequent. Polydypsia, polyuria glycosuria are quite frequent. Sensations are normal, but pain in the extremities and spine is fre- quent. It is increased by fatigue, pressure and exposure to cold. The pain may be of neuralgic or rheumatoid type. The reflexes may be normal, increased or diminished. There is a general sense of continuous fatigue, although the movements of the limbs are not involved. The patient is as a rule apathetic and of slow mentality. Focal epilepsy has been observed in exceptional cases. Course, Duration, Prognosis. — The course is usually slow but pro- gressive. The disease may follow an acute or chronic course. The latter is the most frequent. Amelioration and temporary arrest occur. It usually lasts twenty or thirty years. Cachexia and death are the habitual termination, unless the patient dies from some intercurrent disease. Men are more frequently affected than women. Diagnosis. — The differentiation with Myxoedema is comparatively easy. In the latter disease the enlargement of the extremities is due to a mucoid infiltration of the skin. The round, puffy face of a myxcedema- 574 ACROMEGALY tous is essentially different from the oblong face and prognathism of an acromegalic. In Elephantiasis only the skin and the cellular tissue are involved. It affects only one side and an entire limb. Hypertrophic Pulmonary Osteo -arthropathy (Marie) is characterized by paw-like hands, the ends of the fingers are enlarged and the nails are thick and brittle. The lower jaw is not involved. The disease is usually associated with cardiac or pulmonary diseases. In Osteitis deformans (Paget) there is a marked deformity of the legs, but the hands and feet are intact. The cranium is deformed, while in acromegaly the face is involved. Etiology. — Heredity plays a small part. Diseases of the nervous system (chorea, hysteria, tabes), mental diseases, infectious diseases, alcoholism, syphilis, gout have all been considered as predisposing factors. As exciting causes can be mentioned trauma and violent emotions. The disease occurs mostly at the age of between twenty and thirty-five. Females are somewhat more frequently affected than males. Pathogenesis. — Pathological investigations show that in a large majority of cases the pituitary body was found diseased (degeneration, tumor, hypertrophy). In a number of cases the thyroid gland, the thymus, suprarenals and pancreas were altered. Although in some observations the pituitary gland was found intact, the consensus of opinion at present is that a disturbance in function of this glandular body is the only cause of acromegaly (hyperpituitarism). There are clinical and experimental reasons to believe in physiological interrelation between all those glands. The nutrition of the body and cartilaginous tissues is supposed to be controlled by the pituitary body. Therefore any disturbance in its normal function is followed by the manifestations characteristic of acromegaly. Treatment. — The foregoing remarks lead to therapeutic indications. The pituitary body, thyroid and thymus glands have been administered in acromegaly. Some favorable results have been reported, particularly from the use of the pituitary gland. Iodides, mercury, iron, ergot and arsenic may be tried. Other manifestations of the disease are treated sympto- matically. General hygienic measures should not be neglected. Opera- tive procedures for the purpose of removing the hypophysis date from 1907. Schlosser was the first to extirpate a tumor in a man. Surgeons have since attempted operations through different routes. The latter are: (1) nasal route combined with resection of the upper maxilla and orbit; (2) buccal route with resection of hard palate; (3) transverse supra- GIGANTISM 575 hyoid pharyngotomy; (4) temporal intra-cranial route; (5) trans-frontal method (Frazier), viz. through anterior cranial fossa. GIGANTISM A condition allied to acromegaly is gigantism. Under this name should be understood a condition which is characterized not only by a size of the body above the normal, but also by physical and mental anomalies. Symptoms. — The most important feature is the excessive size of the body. The height may reach nine or ten feet. The un- usual growth of the body commences at puberty and in exceptional cases in childhood and continues above the average age. In spite of their enormous size the giants complain of muscular weakness. As a sign of abnormality should be also mentioned atrophy of genital organs, the con- sequence of which are impotence, sterility, amenorrhea. The mentality is also below normal. Such patients are apathetic, childish, without initia- tive and with a poor memory. As additional manifestations they infre- quently present continuous headache, visual disturbances, polyuria and especially gly- cosuria. Gigantism and acromegaly is one affection. If the progressive increase in size makes its appearance in childhood or youth, the result will be gigantism. If the size continues to increase beyond the age at which growth ceases normally, the result will be associated with acromegaly (see this chapter). The individual is therefore an acromegalic giant. Acro- megaly is consequently the gigantism of the period of full development. Gigantism is acromegaly of the developmental period. The association of gigantism and acromegaly may occur not only in two successive periods, which is the rule, but also simultaneously long before the epiphyseal cartilages become ossified, at a period very near birth and even before birth. In the latter case the hypophyseal hypertrophy, which is very Fig. 165. — Acromegalic Giant. (Brissaud and Meige.) 576 ACHONDROPLASIA probably the common cause of both gigantism and acromegaly, may sometimes be congenital. Gigantism may be associated with infantilism. In such individuals the attributes of an infant are present, as, for example, absence of ossification of the cartilages between the diaphysis and epiphysis. Moreover, there are also atrophy of the genitalia, undeveloped muscu- lature, tender skin, absence of hair of the pubis and axilla. The mentality is that of a young child. An infantile giant may also become acromegalic. Course, Duration, Prognosis. — In the majority of cases the course is slow, although acute cases have been reported which ended fatally at an early age. Ameliorations have been observed. The condition is essentially progressive. The patients usually die from some intercurrent disease, particularly pulmonary tuberculosis. Pathogenesis and Etiology. — Pathological investigations show in the majority of cases a diseased condition of the pituitary body, either hyper- trophy or tumor. In some cases hypertrophy of the thyroid gland was found. The prevalent opinion is that gigantism is due to the persistence of the epiphyseal cartilages beyond the normal age and continuation of the growth of the skeleton. Ossification of cartilages is controlled mainly by the pituitary bcdy, but also by the thyroid, testicle and other glands with an internal secretion. A morbid condition of the latter will interfere with the process of ossification. Speaking generally gigantism is by it- self an indication of a degenerative make-up. Gigantism may follow infectious diseases, intoxications. It affects males more frequently than females. Treatment. — Pituitary or thyroid gland may be tried, although no genuine satisfactory results have been reported. ACHONDROPLASIA It is a congenital malformation characterized by a smallness of sta- ture and due to a deficient ossification of the cartilages of the long bones. The first good description was given by P. Marie in 1900, although the condition had been known long before. Symptoms.,- — Soon after birth the unusually small size and short limbs are noticed. The growth is retarded and the individual reaches adult life, but is a dwarf. The following three peculiarities characterize achondroplasia: dwarf - ishness, micromyelia and macrocephalia. The trunk and head are of normal size, but the limbs and particularly the lower are unusually short (micromyelia) . The contrast is striking and ADIPOSIS DOLOROSA 577 typical. The proximal ends of the limbs (arms and thighs) are more affected than the distal ends. Normally when the individual is in a standing position, the medius reaches the lower third of the thigh. In achondroplasia the same finger hardly reaches the trochanter. Marie called attention to a special position of the fingers. When an attempt is made to bring them together, only the first phalanges are approached, the last two are separated. The head may be of normal size, but not infrequently is enlarged (macrocephalia). Sometimes it resembles a hydrocephalic head. Other Symptoms. — The scapulae are short. The pelvis is small. The muscula- ture on the contrary is well developed. The genitalia are normal in size and function. The mentality in the majority of cases is normal. In Marie's cases, however, it was deficient. Pathogenesis and Etiology. — Various views have been advanced as to the nature of achondroplasia. Some believe in a hereditary degenerative state of the cartilages. According to others a toxic material influences in some manner the nervous system, which in its turn pro- duces trophic disturbances of the cartilages. Perhaps the ductless glands are the source of the toxic material. Treatment. — Not much reliance can be placed upon the administration of thyroid extract, which has been advised. Fig. 166. — Achondroplasia. (Apert.) ADIPOSIS DOLOROSA It is characterized by deposits of fat in various portions of the body. These deposits are painful upon pressure. ; The disease has been observed and described by clinicians under various names, but Dercum was the first to give this clinical entity the name of adiposis dolorosa. Symptoms. — Four main symptoms characterize the disease, viz. fat 37 578 ADIPOSIS DOLOROSA formation, pain, asthenia and psychic symptoms. The striking feature is the nodes of fat distributed all over the body. The deposition of fat may be not only nodular, but also circumscribed diffuse or generalized diffuse. The nodular is the most common form. The extremities are particularly involved. At first the bunches of fat are noticed in the lower and upper extremities; the trunk is affected later, but the face, hands and feet have been spared in all observed cases. When these nodes are compressed, the patient suffers considerable pain. In the nodular form the size of the nodules may be as small as a pea but more frequently they are of the size of an orange. They are usually mobile, but they may be adherent to the skin. In the circumscribed diffuse form the mass may involve a segment of a limb or an entire limb. The mass may be smooth or lobulated. The generalized diffuse form usually begins on the abdomen and then spreads gradually to the trunk and extremities. The hands and feet which usually remain unaffected in the two other forms, here are not in- frequently involved, but the fatty deposits are seen only on the palmar surface of the hand (thenar and hypothenar regions) and on the soles of the feet. The skin over the involved area is usually white and soft. Pigmenta- tion of the skin with atrophy and diminished sweating have been observed. Other Symptoms. — The patient often complains of spontaneous dull aching, also of numbness, coldness or else burning. Pain is never absent. It may be spontaneous or elicited by pressure. In well-advanced cases it may be unusually severe. It does not follow the course of nerve trunks. Besides being continuous, the pain may present paroxysms of unusual severity. At each of such paroxysms the size of the fatty mass increases, but recedes at the disappearance of pain. There is usually a marked diminution of objective sensibility or complete anaesthesia in the affected areas. Muscular weakness is a frequent symptom. Sometimes it is so marked that the patient is compelled to remain in bed. Headache, hemorrhages in the nasal cavities occasionally occur. One of my patients presented retinal hemorrhages appearing without a strain, effort or any traceable cause. Contraction of the visual fields, scotomata, amaurosis have been observed. Psychic symptoms are quite frequent. Change of disposition, marked irritability are common. In some cases there is also mental exhaustion. Delusions of persecution, dementia have also been observed. Vaso-motor disturbances are not rare. Cyanosis of the limbs, oedema, ecchymosis upon the least trauma or without any apparent cause have been also observed. SCLERODERMA 579 Among the rare manifestations arthropathies have been recorded. Course, Duration, Prognosis. — The disease is essentially progressive. Its duration is indefinite. Death usually occurs from some intercurrent disease, pulmonary especially. Diagnosis. — The disease cannot be confounded with myxcedema, first because of its localization and next because of the pain upon compres- sion of the enlarged areas of the skin. Etiology. — A neuropathic tendency was traced in almost every case so far reported. Alcohol and syphilis may have some etiological impor- tance. Disturbances of sexual functions (menopause, menorrhagia, abor- tion) have been reported as immediately preceding the onset. Trauma, emotion, exposure to cold have been traced in some cases as immediately preceding the onset. Women are more frequently affected than men. Pathogenesis. — Autopsies have so far failed to reveal the true nature of the disease. Cases have been reported with morbid changes in the thyroid gland and pituitary body. In one of Dercum's patients there was an interstitial neuritis with a moderate sclerosis of Goll's columns. The general opinion is that the affection is due to a connective tissue dystrophy (fatty changes) with an involvement of the nerve-fibers (neuritis) . Treatment. — The foregoing remarks suggest a trial of thyroid therapy. In my case mentioned above I obtained amelioration of the parassthetic disturbances from internal administration of extract of parathyroid glan- dules. Avoidance of fatigue and general hygienic measures are not to be neglected. Pain may be relieved by coal-tar products. Prolonged rest in bed may be beneficial. SCLERODERMA It is chacarterized by an induration of the skin and its adherence to the subcutaneous tissue. Symptoms. — Before the formation of the characteristic skin the patient complains of distressing subjective sensations, as pain, itching, tingling, etc. Soon cedematous spots appear on the skin; they may remain cir- cumscribed (morphea) or merge one into another and become diffuse. Gradually atrophy of the skin develops, so that in an advanced stage the skin is thin, tense, hard and glossy. Pigmentation, vitiligo are noticeable. The skin cannot be wrinkled. In still more advanced period the atrophy progresses and involves the underlying tissues. The subcutaneous, muscular and osseous tissues become invaded. 580 SCLERODERMA The disease may be localized (morphea) or diffuse, bilateral or uni- lateral. The most frequent seat of the affection is on the face, neck, upper part of the thorax and upper extremities, especially the hands. When the face is involved, the features are drawn, immobile, mask-like, the lips are thin and the display of the muscles in emotions is of course disturbed. Mastication and speech are difficult. When the hands are affected, the fingers are rigid, thin and retracted (sclerodactyly) . Constriction and subsequently spontaneous amputation of phalanges may occur. Raynaud's disease may sometimes complicate sclerodactyly. When the neck and thorax are affected, the respiration may be inter- fered with. Sclerodema has been observed in association with trophic disturbances in other parts of the body, viz. atrophy of one-half of the body, facial hemiatrophy. Course, Duration, Prognosis.- — In the large majority of cases the disease is essentially progressive. Cachexia and death are the ultimate terminations. Frequently the patient dies from some intercurrent disease. Scleroderma is sometimes seen complicated by facial hemiatrophy and erythromelalgia. Raymond and Guillain have observed a case of generalized sclero- derma with bilateral ocular palsies and nystagmus. The latter symptoms were due to sclerosis of the ocular muscles, but not to a genuine paralysis. Etiology. — Scleroderma has been observed in the course of lepra, syringomyelia, paresis and tabes. A neuropathic and arthritic predis- position play some part. Traumatism, emotion, exposure to cold, pregnancy have been considered as etiological factors. The disease may occur at any age, even in infants, but particularly between twenty and forty years of age. Women are more frequently affected than men. Pathogenesis. — Pathologically the affection is characterized by a sclerosis affecting the skin and subcutaneous tissue. Atrophy and retraction follow. The bloodvessels are also involved: a peri- and end- arterities are always present. These facts, however, do not explain the nature of the disease. Some authors believe that the vascular lesion, which is so constant, is the only cause of the disease. Others consider it as a trophoneurosis or angio- trophoneurosis. Others believe in a sympathetic origin and still others in infection. Finally a disturbance of the function of the thyroid gland may have something to do with the scleroderma. Treatment. — Thyroid extract, iodides, local massage, electricity, also hypodermic use of thiosinamin (15 per cent, of alcoholic solution) every FACIAL HEMIATROPHY 581 other day are all the remedies advised, placed on any of them. Not much reliance can be PROGRESSIVE FACIAL HEMIATROPHY Symptoms. — In the majority of cases one or two brownish spots appear first on one side of the face. The underlying skin begins to appear glossy and to undergo atrophic changes. The subcutaneous cellular Fig. 167. — Facial Hemiatrophy. tissue follows. A depression develops because of the disappearance of fat. The skin of the nose, orbit, cheek and lips becomes thin and wrinkled. Other trophic disturbances occur. The teeth, the eyebrows fall out, the secretion^of the sebaceous and sweat glands is diminished. 582 FACIAL HEMIATROPHY The face is asymmetrical and drawn to the normal side. The contrast between both sides is striking. The function of the muscles of the affected side is as a rule but slightly disturbed, as there is no paralysis. Their electrical reactions show no reactions of degeneration. The faradic con- tractibility is increased. However, an associated hemiatrophy of the masticators, of the tongue and of the palate which is occasionally observed, may interfere with mastication. The bones of the face participate in the atrophy in advanced cases. The frontal, molar and the maxillary bones, the nasal cartilages all atrophy and shrink. Sensations are generally not disturbed, although in my case (N. Y. Med. Jour., January, 1907) they were markedly altered. The patient frequently complains of paresthesias (chilliness, numbness, etc.). Pain of a neuralgic character is sometimes present. In my case the disease began with severe pain around the right infraorbital foramen, which lasted two days; a few days later the hemiatrophy commenced. Course, Duration, Prognosis.- — The hemiatrophy is not always pre- ceded by appearance of brownish spots. In my case a trigeminal neural- gia of two days' duration was almost immediately followed by the begin- ning of the atrophy. The disease is progressive. Sometimes the other side of the face becomes involved. Sometimes only a few symptoms are present (incomplete form). Sometimes other parts of the body may be implicated. The prognosis as to life is good. The disease has been observed in association with tabes, syringomyelia, multiple sclerosis, epilepsy, chorea, facial tic. Dilatation, contraction and immobility of the pupil, also congenital palsy of ocular muscles, have also been observed. Etiology and Pathogenesis. — Facial neuralgia, trauma, infectious diseases or localized infectious processes, organic nervous diseases (syringo- myelia) are all possible causes. There are at present two views concerning the nature of the affec- tion. According to some observers, a primary atrophy of the subcuta- neous cellular tissue is the essential feature. Others believe that hemi- atrophy is of a nervous origin (trophoneurosis) and any of the following nerves is supposed to be the immediate cause: the sympathetic, trigem- inus or facial. That the Gasserian ganglion and a cerebral lesion may also produce the disease, there are pathological proofs. Treatment. — Local massage and galvanism may be tried. Gersuny's method of subcutaneous injections of paraffin should be undertaken for remedying the asymmetry of the face. The latter, however, is only an esthetic measure. ACROPARESTHESIA 583 FACIAL HEMIHYPERTROPHY It is a congenital condition. It is due to an anomaly in the develop- ment of the face. It is sometimes associated with a congenital hernihy- pertrophy of an entire half of the body. It consists of an increased de- velopment of the bony as well as of all the covering tissues of one side of the face. The skin is thick and coarse. Not only the superficial bones are involved, but also the palate, basilar process and sphenoid bone. ACROPARESTHESIA Symptoms. — The disease is characterized chiefly by paresthesias in the distal ends of the extremities, viz. fingers and occasionally in the toes. The patient complains of a burning, tingling, pins and needles' sensation, of pain in the tips of the fingers or toes. The hands are most frequently involved. The disturbance is continuous, but in some cases it becomes aggravated at night or early in the morning. It is also worse when the fingers come in contact with cold objects, when exposed to cold or when placed in cold water. The affection is usually bilateral. There are no objective disturbances. The color of the skin is normal. The power of the hands as well as their function is also intact. Course, Duration, Prognosis. — The onset is slow in the majority of cases. The disease lasts many years. Recovery is possible, but not fre- quent. The prognosis is good as to life. The disease incapacitates to a certain extent for physical work, as the latter increases the parsesthesias. Etiology. — Undue exertion, exposure to cold are the exciting causes. Anaemia and pregnancy are predisposing factors. Women are more frequently affected than men. Washerwomen are particularly apt to develop the disease. The affection rarely occurs before the age of thirty. Pathogenesis. — The disease is considered as a vaso -motor neurosis. Through some influence the arteries are in a state of spasm and thus the sensory nerve-endings not sufficiently nourished are being irritated. In advanced cases it is not impossible that a neuritis develops. Treatment. — Avoidance of fatigue and of exposure to cold is the first indication. Arsenic, phosphorus, iron and quinine have been recom- mended. I have obtained favorable results from nitroglycerine. Very recently I studied a series of cases in which I have systematically applied Bier's method of induced hyperemia. The results were very encouraging. A bandage was applied for an hour twice daily at the middle of the fore- arm (see, for details, Therapeutic Gazette, 1908). Galvanism and static electricity have also been advised. 584 ANGIONEUROTIC (EDEMA ANGIONEUROTIC (EDEMA (QUINCKE) Symptoms. — The disease is characterized chiefly by paroxysmal swellings of the skin which are circumscribed and not inflammatory in nature, The mucous membranes are equally apt to be involved. The favorite seat is on the face and lips, but may also occur on the scalp, forehead, palate, pharynx, larynx and viscera (stomach and intestines) , in rare cases in the brain (Osier) . The articulations may also be involved by a sudden effusion. In one case, a girl of eighteen, the swellings appeared in the upper eyelids. The circumscribed swelling resembles urticaria; it is round, of about 2 cm. in diameter; it is usually white, but it may be also somewhat reddish. It does not pit on pressure. The swellings may last a few hours or days. They frequently dis- appear from one part of the body to appear in another. The patient does not suffer any special discomfort except some tension and stiffness, but no pain. When the mucous membranes are involved, the disturbance may interefere with the function of the pharynx, larynx, etc. Difficulty of swallowing and of breathing will ensue, and in some cases trache- otomy may become urgent. In exceptional cases albuminuria and hemo- globinuria have been observed. When the gastric and intestinal mucous membranes are affected, vomiting and diarrhoea will follow. Course, Duration, Prognosis.— The disease may disappear sponta- neously. In some cases it may last indefinitely. Recurrences are frequent. The prognosis, while not unfavorable, is, however, uncertain, as the disease is a very stubborn one. It is serious when the larynx is involved. Diagnosis. — The paroxysmal character of the swellings, their circum- scribed appearance, the absence of pain and the fact that they do not pit on pressure are all sufficiently typical signs for the diagnosis and cannot be confounded with other affections. Etiology. — Heredity and neuropathic tendency play a prominent role. It is sometimes observed in several members of the same family. It is not infrequently associated with hysteria, epilepsy, exophthalmic goiter. Physical and mental exhaustion are also predisposing factors. As exciting causes may be mentioned: cold, trauma, emotion, also toxic substances, such as alcohol and tobacco. Malarial poison may also produce the disease. Males are more frequently affected than females. It usually occurs in young age. Pathogenesis. — The disease is probably a vaso-motor neurosis, result- ing either in a paralysis of the vaso-constrictors or stimulation of the ERYTHROMELALGIA 585 vaso-dilators. The consequence is a serious exudation. The latter is in the subdermal tissue. Treatment. — In view of the neuropathic taint present in the majority of patients, a well-regulated life with plenty of rest and moderate exercises, also nutritious food, hydrotherapy, massage and other hygienic measures are the first indication in the treatment. As drugs the following can be recommended: strychnia, atropin, quinine, nitroglycerine. The condition of the pharynx and larynx should be watched, as operative measures may be necessary. Alcohol, tobacco, or other stimulants are forbidden. HEREDITARY (EDEMA OF THE LEGS (MILROY'S DISEASE) It was first described in 1892 by Milroy. Symptoms. — (Edema may appear soon after birth or develop at the age of puberty. It is ordinarily confined to the legs up to the knees, but it may be confined only to the ankles or even reach the thighs. The skin of the swollen parts is usually pale. Acute attacks have been described. In such cases the swelling increases, the skin becomes red and some constitutional symptoms are present, viz. chills, fever, vomiting and pain in the abdomen. The chief features of this disease are: (1) absence of all local or general causes of oedema, (2) duration — mostly from birth, (3) the familial character and the presence of a neuropathic tendency in the family, viz. cases of epilepsy, imbecility, insanity. In Milroy's case he traced twenty-two individuals among ninety-seven persons in six generations. Treatment. — Continuous bandaging is the only measure that is capable to give relief. ERYTHROMELALGIA It is characterized by reddening of the skin and paroxysmal pain in the feet and sometimes in the hands. It was first described and named by Weir-Mitchell in 1878. Symptoms. — The feet are more frequently affected than the hands. Pain is the first symptom to appear. The great toe is particularly painful at the beginning. The pain occurs in paroxysms and later the intervals become shorter and shorter. The pain is excruciating and is relieved by recumbent position and cold. Station, gait, pressure or heat and exercise increase it. When the hands are affected, the patient gets relief by 586 ERYTHROMELALGIA crossing the arms over the breast or by raising them above the head. Such an attitude removes the blood from the periphery. Soon redness of the skin develops. At first it is rosy-red, but later purplish-red. It is especially marked on the last phalanges. The veins are distended and a swelling is noticeable. The local temperature is increased and a hyperhidrosis takes place. The sensations may be di- minished or increased. While in the majority of cases the distal ends of the limbs are involved, nevertheless the pain and redness may sometimes extend to the entire limb. The symptoms may be observed also on other parts of the body, viz. face., ears, testicles, chest, back, also in rare cases in the mucous membranes of the mouth and throat (Seeligmiiller.) Other Symptoms. — During the paroxysms headache, vertigo, tinnitus aurium and even syncope may occur. Hardening of subcutaneous tissue, thickening of the nails, also oedema occasionally occur. Atrophy of the muscles of the extremities may develop in advanced cases. A dim- inution or increase of electrical contractility has been observed, but no reactions of degeneration. Erythromelalgia is sometimes observed in the course of organic or functional nervous diseases, also in diseases of peripheral nerves. Course, Duration, Prognosis.— Amelioration and aggravation in the course of the disease are observed. It lasts an indefinite time. The prognosis is unfavorable, as the disease is rebellious to treatment. Diagnosis. — The cyanosis, local elevation of temperature, swelling, are sufficiently characteristic symptoms for diagnosis. Erythromelalgia should be differentiated from angioneurotic oedema, acroparaesthesia and initial stages of Raynaud's disease (see these chapters). Mention should be made of so-called incomplete or allied forms of erythromelalgia. I observed a patient whose affection began with red- ness of the hands and the pain appeared only later; cold would increase the pain; exercise had no effect upon 1he pain. It is therefore a case which apparently belongs to the group of ervthromelalgia and yet not typical. l Etiology. — Cold, physical fatigue, are predisposing factors. Infec- tions, rheumatism, syphilis and accompanying nervous diseases, such as hemiplegia, cerebral tumors, multiple neuritis, neuralgia, hysteria, neurasthenia, etc., are the main causes. Pathogenesis. — The majority of observers consider the disease as a vaso -motor neurosis. Whether it is due to a paresis of the vasoconstrict- ors or to an irritation of the vaso-dilators, it is difficult to say. According 1 American Medicine, August, 1907. RAYNAUD'S DISEASE 587 to some the spinal gray matter is the source of the disease. Others believe in a disease of the peripheral arteries. Weir-Mitchell thinks that there is a neuritis of the sensory nerves. Treatment. — Electricity, application of cold and sedatives for relief of pain are the only measures employed. Perhaps Bier's method of artificial hyperasmia may render some service. If syphilis is suspected, and even without evidences of syphilis, mercury and iodides, also salvarsan may be tried. Removal of small portions of nerves or stretching of nerves, also amputations of toes or fingers, are to be attempted as a last resort. RAYNAUD'S DISEASE It is characterized by an ischemia or a hyperemia of the extremities which may terminate in a symmetrical gangrene. It was described by Raynaud in 1862. Symptoms. — The disease presents three phases. The first is char- acterized by local ischemia, the second by a passive hyperemia, the third by gangrene. First Phase. — Suddenly the fingers or toes or in exceptional cases the entire hand or foot become pale, wax-like and cold. This is accompanied or preceded by a numbness, tingling or severe pain. The condition may disappear rapidly or be followed by a cyanosis (second phase). In the latter case the skin becomes blue. Then the pain increases. The part is swollen but not cedematous. The anemia produced by pressure with a finger is slowly obliterated. The temperature is lowered. The pallor alternating with cyanosis (local asphyxia) or cyanosis without the pre- ceding pallor, when repeated may become very frequent and then the patient enters into the third phase of the disease, characterized either by active hyperemia or gangrene. The blue color of the skin turns red. The part gets hot and throbs. This period lasts a variable time. It may be entirely missing. The asphyxia may persist and the circulation not be reestablished. In such a case the final period of gangrene is to be feared. Gangrene may follow even the first stage of syncope : in such a case the parts never recover the circulation. Dark spots and vesicles appear; the serum of the latter dries up and a crust forms. When the latter falls off, an ulcerated surface is noticed which is slow in healing. The gangrene may extend and involve an entire phalanx. The skin is parchment-like, the phalanx mummifies, a line of demarcation is formed and gradually the gangrenous part becomes detached. Cicatrization of the stump may be rapid or slow. During the entire process fever is absent, but the pain is excruciating. The suffering disturbs the patient's sleep, digestion and the general nutrition. 588 Raynaud's disease The phase of "pallor" and that of "cyanosis" correspond to "Ray- naud's "local syncope" and "local asphyxia," respectively. They are, properly speaking, two successive stages of the disease and they eventu- ally terminate in the final phase of symmetrical gangrene. However, the first two periods may exist a long time conjointly or separately without entering the final stage. Also the local syncope may exist a long time before the second period makes its appearance. Local syncope occurs in attacks lasting but a few minutes or several hours. The patient complains of "dead fingers." The attacks recur sometimes with great regularity. They may occur either daily for some time or every two or three months. They are usually precipitated by exposure to cold. The place of local asphyxia (cyanosis) is mostly preceded by attacks of local syncope and it may last days, weeks or months. In mild cases it may disappear, but in severer cases it is followed by the phase of sym- metrical gangrene. Other Symptoms. — Objective sensations are usually decreased. Anaesthesia is common in the period of syncope. Parassthesia is present in the period of asphyxia. Hyperassthesia is usually present after a paroxysm. Atrophy of the neighboring muscles with impairment of motion is not infrequent. Pupillary contraction, tinnitus aurium, albu- minuria, may occur. Mental depression is a frequent accompaniment. Not only the fingers and toes, but also the heels, coccyx, the ears, nose and the prominent bones of the cheeks may be affected. The disease is usually symmetrical and bilateral, although unilater- ality has been observed. Complications. — Panas reports a case in which there was a relation between the cyanotic attacks and the condition of the arteries in the fundus oculi: when cyanosis occurred, the arteries were contracted; when the former subsided, the latter became normal. In some cases Raynaud's disease was preceded or followed by attacks of transient hemiplegia, monoplegia, aphasia or amblyopia. Epileptic convulsions have been observed in association with attacks of local cyanosis. Hemoglobinuria is a rare complication. Purpura and urticaria have been exceptionally observed. Course, Duration, Prognosis. — The course may consist only of the first or of the first and second phases. More frequently the entire cycle is observed. The duration of the disease is from one to three months. After an interval of months or years another attack may occur and affect another part of the body. Life is usually not endangered, except when suppuration with septic infection is present. RAYNAUD S DISEASE 589 Diagnosis. — The disease is easily recognized from its typical symptoms. Local gangrenous patches may also be observed in hysteria, but the necrosis is superficial and no changes of the blood vessels are found. In lepra phalanges fall off without pain. Gangrene caused by in- toxication with ergot presents special features characteristic of ergotism. I saw a case of local gangrene caused by subcutaneous injections of adrenalin above the lesion. There are also allied cases in which the picture of Raynaud's disease is not complete, cases that present intermediary forms, which may resemble Fig. 168. — Symmetrical Gangrene. (Dehio). one or another of the localized neuroses. In the case referred to in the chapter on Erythromelalgia the symptoms were those of Raynaud's disease and of erythromelalgia. Etiology. — The disease occurs not infrequently in the course of organic nervous diseases and psychoses, also in functional nervous disorders. Infectious febrile diseases, syphilis, tuberculosis, lepra, diabetes, may be accompanied or followed by Raynaud's disease. It has been observed in the course of scleroderma. Anaemia and a congenitally small aorta play an important predisposing part. Emotion, trauma, menses, cold, are exciting causes. Zenner observed a case due to caffein poisoning. Young persons, and especially women, are more frequently affected than old individuals and men. Pathogenesis. — Pathological investigations show a localized involve- ment of the blood vessels (arteritis) and of the nerves (neuritis), but these changes are secondary. The majority of observers consider Raynaud's disease a vasomotor neurosis (see also erythromelalgia). Raynaud believed that the vaso-motor centers are in a state of irritability; the 590 RAYNAUD S DISEASE irritant comes from the periphery (cold), the paroxsysmal contraction of the blood vessels comes from the center. Treatment. — Rest and local applications of heat are important. Good hygienic and dietetic measures are necessary. Pain can be combated by usual means, such as coal-tar products, etc. Amylnitrite in inhalations during the attacks may give relief from pain, but it is not always effective. Nitroglycerine and ergot have also been recommended. Bier's method, consisting of an application of bandage above the affected parts for an hour twice daily, will induce an artificial hyperaemia and give relief. It may be of benefit in the first two phases of the affection. Cushing's method may be very useful. He bandages lightly the limb, then applies a tourniquet for a minute or two. When the latter is removed, a large amount of blood rushes to the parts which were made anaemic. It may be tried several times a day. Barlow recommends the following procedures. "Immerse the limb into a basin containing salt and tepid water; one pole of a constant current battery is placed in contact with the upper part of the limb above the level of the water, the other pole in the basin. The current is then made and broken at frequent intervals so as to obtain moderate contractions of the limb. The patient is also told to move the digits while electricity is being applied." He claims that considerable relief is obtained in cases of recurrent local asphyxia. High-frequency current has also been recommended. In case of gangrene antiseptic applica- tions with rest of the limbs are the only measures besides operative considerations. CHAPTER XXVIII NERVOUS SYMPTOMS PRODUCED BY INTOXICATIONS A. METALLIC POISONS I. Lead Intoxication Lead may affect the central and the peripheral nervous system, also produce psychoses. Pathology. — A striking and at the same time a common feature is arteriosclerosis. It is not, however, conclusively proven that lead is the direct cause of the thickening of the vessel walls. In the brain the meninges are altered: pachymeningitis, leptomeningitis with oedema and atrophy of the cerebral tissue are frequently observed. Changes of the cortical cells, proliferation of the nuclei in the vessel walls and of the glia-cells about the vessels have been found. In the cord chronic anterior poliomyelitis may occur. In a case under my observation I found microscopically degeneration of the posterior columns {American Medicine, 1905). The peripheral nerves present parenchymatous degeneration. For details see the chapter on multiple neuritis. Symptoms, (a) Cerebral Encephalopathy. — In acute poisoning head- ache, vertigo, epileptiform convulsions, delirium and hallucinations, also coma, are observed. The chronic form may be accompanied by persistent headache, also convulsions, attacks of hemiplegia, attacks of aphasia, finally by psychoses (see below). Functional nervous diseases may be induced by chronic lead poisoning. Choreiform movements, hysterical hemianaesthesia have been observed. Generalized or focal epilepsy are not infrequent. The psychoses due to lead may present themselves in the form of a delirium or more frequently by stupor. Delirium usually lasts weeks. Symptoms of paresis are not rare, but they do not present the typical picture of this psychosis. The condition is therefore called "pseudo- paresis" (see Paresis). Delusions of persecution with terrifying hallucinations occur. The latter are particularly present in cases which are at the same time alcoholic. Eye Symptoms.- — Palsies of the ocular muscles, also nystagmus, may occur. Hemianopsia, usually homonymous, amaurosis are usually 59i 592 NERVOUS SYMPTOMS PRODUCED BY INTOXICATIONS transitory. In some cases profound changes in the optic nerves take place. (b) Cord Symptoms.— Muscular atrophy due to poliomyelitis is not rare. Aran-Duchenne's type of atrophy with the claw-like hand is oc- casionally seen. Erb's type has also been observed (see Progressive Muscular Atrophy). (c) Peripheral Nerve Symptoms. — See chapter on Multiple Neuritis. Course, Duration, Prognosis. — Cerebral symptoms present a serious outlook. When optic neuritis is established, the prognosis is doubtful. Blindness may be the outcome. When cardiac or renal complications occur, the prognosis is grave. In most favorable cases the prognosis should be guarded, as recurrences are not infrequent. Finally the patient's future will depend upon whether he is removed from the influence of lead or not. Etiology. — The occupations of painters, compositors, occupations re- quiring a free handling of lead and lead colors, especially lead carbonate in a dry state, the use of certain cosmetics, the use of water kept in lead vessels, are the usual sources of lead intoxication. Treatment. — Elimination of lead is the first indication. Purgatives and diuretics are necessary. In view of the obstinate constipation characteristic of lead poisoning, moderate doses of atropine should be given with the purgatives in order to control the intestinal spasm. To enhance diuresis large quantities of water are to be given. Baths of potassium sulphuret will help elimination of lead: to six inches depth of water six ounces of potassium sulphuret are added. Potassium or sodium iodide internally is excellent. Exclusive milk diet at first and later milk and vegetable diet, hydrotherapy, avoidance of stimulants, including tea and coffee, are the main elements of the treatment. In- somnia, headache, gastric disturbances, convulsions, are treated accord- ingly. Venesection and lumbar puncture may be useful in cases of cerebral congestion. Paralysis is to be treated with massage and electricity. II. Arsenical Intoxication Pathology. — No special lesion has been found in the brain. In the spinal cord there may be involvement of gray and white matter (myelitis). Changes in the peripheral nerves are the most important. See Multiple Neuritis. Symptoms. — In chronic cases there may be present cerebral symp- toms, viz. impairment of memory, of intellectual faculties. In acute cases delirium and confusion are the usual manifestations. NERVOUS SYMPTOMS PRODUCED BY INTOXICATIONS 593 Eye symptoms may be amblyopia or amaurosis, ocular palsies. Puf- finess of the eyelids, congestion of the conjunctivae, are quite common. When the cord is involved, symptoms of myelitis are evident. For the symptoms of arsenical neuritis see chapter on Multiple Neuritis. Course, Duration, and Prognosis are usually favorable and similarly to all toxic conditions may be serious, if the intoxication is severe and protracted. Etiology. — Wall paper, colored dress goods, arsenical paint, rugs, carpets, crayons, beer (glucose in England), medicinal arsenic and finally intentional absorption are all sources for intoxication. Treatment. — See preceding chapter. III. Mercurial Intoxication Pathology.- — Cerebral oedema, degenerative changes in the tracts of the cord and in its gray matter, atrophy of the nerve fibers, sclerotic changes of the vessel walls, are the usual findings. Symptoms — Cerebral excitement or else depression, vertigo, tremor, choreiform movements, convulsions, muscular weakness, disturbance of speech (caused by tremor of lips), diminution or loss of cutaneous sensibility, amblyopia and sometimes optic neuritis are the chief symp- toms. In advanced cases impairment of memory, confusion and mild dementia may develop. Tremor is the most conspicuous symptom. At first it is very slight. Soon it becomes marked upon the least emotion. The range of the tremor may be small or gross. While at first it is present only on voluntary acts, later it appears even when the patient is at rest. The hands and lips are affected first, but later all extremities and face become involved. The twitchings may sometimes be very violent. In the chapter on Multiple Neuritis details are given concerning the manifestations of the peripheral nerve involvement. Course, Duration, Prognosis. — See the previous chapter. Etiology. — Manufacture of mirrors, of rubber, the use in paints, in artificial flowers, in aniline colors, in fireworks, in mining, in smelting, in manufacture of thermometers, finally the use for medicinal purposes are all the usual sources of mercurial poisoning. Treatment. — See preceding chapter. IV. Carbon Monoxide Intoxication Acute poisoning may be followed immediately or after a considerable time by persistent disturbances in the nervous system. 38 594 NERVOUS SYMPTOMS PRODUCED BY INTOXICATIONS Pathology. — Diffuse encephalomyelitis is the characteristic feature of the changes in the central nervous system. Hyperemia, hemorrhages in the cortex, in the basal nuclei, in the internal capsule, foci of soften- ing particularly in the lenticular nuclei (see Diseases of Basal ganglia), poliomyelitic foci in the cord, peripheral neuritis, are all conditions met with. Symptoms. — Immediately after the poisoning the following nervous disturbances are observed: headache, vertigo, nausea, vomiting, muscular weakness, relaxation of sphincters and loss of consciousness. Muscular twitching and even convulsions may occur. If consciousness is regained, there is a marked mental hebetude. The permanent nervous symptoms are : neuritis, neuralgias, anaesthe- sias, choreiform movements, intention tremor and scanning speech. For details on neuritis consult the chapter on Multiple Neuritis. Ocular palsies, nystagmus, partial or complete blindness may occur. Paralysis, hemiplegia or monoplegia and mental disturbances are not rare. They may develop weeks after the poisoning. Amnesia is quite common. In my case {New York Medical Jour., 1906) there was persist- ent antero-retrograde amnesia. Confusion or stupor with or without hallucinations may last from weeks to two or three months. The para- lytic symptoms may be permanent. Cases with total dementia have been recorded. All these sequelae may appear immediately or weeks after the poisoning. It is important to know that sometimes very serious sequelae develop in cases of mild poisoning. Course, Duration, Prognosis. — Recovery may follow in a very short time. It depends frequently upon the promptness with which treatment is instituted. The prognosis should be guarded in view of the sequelae that may follow some time after the poisoning. Cerebral manifestations are usually of grave omen. Etiology. — Illuminating gas, gas works, furnaces, heating apparatuses with poor draughts, tar distilleries, chemical factories, laboratories, are the sources of carbon monoxide poisoning. Treatment. — Removal from the poisoned atmosphere, inhalation of oxygen, venesection followed by injection of normal salt solution, ad- ministration of stimulants, are the immediate measures. Prophylaxis is the most important feature. V. Manganese Intoxication. L. Casamajor has very recently (/. Am. Med. Assn., 1913) called attention to a symptom-group produced by poisoning with manganese. The cases that came under his observation occurred among workers in the NERVOUS SYMPTOMS PRODUCED BY INTOXICATIONS 595 separating-mill connected with a mine from which zinc, iron and manganese are the principal products. The handling of the ground ore is done in a dry state; the air which the workmen breathe is laden with a very fine gray dust. As zinc and iron have never produced a poisoning similar to the one under discussion, the author concludes that manganese is very probably the etiologic factor. Symptoms. — A man working in this dust for a period of six months or more begins to notice a difficulty of walking down an incline. If he does, he must run fast until he meets an object (propulsion). Walking up a hill or on a level ground is normal. Walking backward is impossible and if an attempt is made, the patient falls (retropulsion). In addition to the disturbance of gait, the patient may have pain and stiffness in the legs. The picture resembles that of paralysis agitans. Other Symptoms.- — Defective hearing, slurring speech, mask-like f acies, tremor of the tongue and hands, unsteady station. These symptoms were not found simultaneously in all the patients. Sensations, reflexes, sphincters, are all normal. Course, Prognosis. — The course is chronic. When removed from the dust the patient may improve, but this is not in every case. The outlook is poor. Pathology. — In one case that came to autopsy the following condition was found. Perivascular spaces of the lenticular nuclei and of the outer part of optic thalami are larger than normal. No other gross changes have been observed. B. ORGANIC POISONS I. Alcoholic Intoxication. Alcoholism Alcohol has a special predilection for the nervous system. Its effect upon the nervous system differs in acute and chronic intoxications. Acute Alcoholism. — Mental symptoms are predominating. Delirium, confusion and stupor with or without hallucinations and illusions are the characteristic features. They are fugacious, transitory. Recovery usually follows. The promptness of the recovery depends upon the effect of alcohol on different individuals. When the alcoholic abuse is frequently repeated, a state of chronic alcoholism develops. Chronic Alcoholism. — When the absorption of alcohol is slow and prolonged, the changes it produces in the individual's physical and mental spheres of life are enormous. 596 NERVOUS SYMPTOMS PRODUCED BY INTOXICATIONS Physical Symptoms. — Gastro-intestinal disturbances are marked by anorexia, vomiting, constipation or else diarrhoea with bloody discharge. Tremor is common. It is passive and intentional. It affects the extremities as well as the tongue and lips. Epileptic convulsions are not rare. They may be unilateral or generalized. Attacks of apoplexy followed by paralysis occur. Neuritis is very common. For details see chapter on Multiple Neuritis. Vaso-motpr disturbances, increased mechanical irritability of muscles and nerves, various subjective disturbances, as pains, coldness, numbness, palpitation, fears, general weakness, are all accompanying symptoms. Ocular disturbances are not infrequent. They are: contraction of the visual field, scotomata, optic atrophy and optic neuritis. Mental Symptoms. — In my series of 277 cases of chronic alcoholism (/. of Am. Med. Assn., 1907) the mental disturbances followed repeated subacute attacks or repeated attacks of delirium tremens, but also in- sidiously and progressively without preliminary acute symptoms. The mental state consists chiefly of a gradually developing intellectual feeble- ness, viz. dementia. Before the latter becomes conspicuous, the patient begins to show undue irritability which at first is noticeable only to the immediate surroundings. At the same time appears a weakness of the will power and of energy. The patient soon becomes depressed, his memory clouded, the power of application for work, physical or cerebral, decidedly impaired. The sadness, the realization of his physical and mental impotence, lead him directly to delusive ideas which become intensified by hallucinatory images, and criminal tendencies are not infrequently observed. Gradually the moral sense, the sense of propriety, becomes deteriorated, conventional laws are totally ignored. The patient becomes apathetic, brutal. His cerebral functions are totally disorganized, the judgment infantile and dementia is permanently established. An acute episode in the course of chronic alcoholism is seen in Delirium Tremens. — As the name implies, it is characterized by a delirious state and tremor. The condition usually develops gradually. At first there is a state of restlessness, insomnia with hallucinations; tremor appears in the hands and tongue. These few symptoms continue to increase in intensity. The patient becomes very restless, very talkative, attempts to get up, if he is in bed, runs from place to place; talks aloud and appears to converse with imaginary persons. His actions are prompted by various hallucinatory images. Through the visual apparatus he sees NERVOUS SYMPTOMS PRODUCED BY INTOXICATIONS 597 terrifying animals, assassins, robbers, executions, fires, etc. Through the auditory apparatus he hears threats, oaths, bad names, etc. Through olfactory apparatus he perceives the most repugnant odors. Through the gustatory apparatus he tastes nauseating substances. Through the sense of touch he believes himself undergoing torture. Being under the influence of these hallucinations the patient is excited, defends himself, threatens, strikes or else is terrified and lays immobile. Orientation in time and space is frequently affected. A patient, for example, may re- member the objects of his room, but does not recognize that he is in his own room. A feeling of great anxiety is ever present because of the hallucinations, but when the patient reaches the climax of his delirium, the anxiety is considerably diminished and may even disappear. He then becomes indifferent to the hallucinations. These mental phenomena are accompanied by a tremor affecting the hands or the upper and lower extremities or the latter and the face. In some cases the tremor continues even in sleep. The speech shows an incoordination: stumbling over syllables and words, difficulty in pronun- ciation. The loss of appetite is striking during the delirious state. The pulse runs from 80 to 115 and is dicrotic. The temperature is usually normal, but it may rise as high as 103 and even 105 . Constipation is the rule. Albumen is present in the majority of cases. The polynuclear leucocytes of the blood are increased and the mononuclear are decreased (Elsholz). The duration of an attack is usually from two to eight days, according to its intensity. It usually ends in a deep sleep lasting from twelve to thirty hours. When the patient awakens, the delirium and hallucinations are gone, orientation is good, but some mental hebetude remains, the mem- ory is somewhat cloudy and a slight tremor persists. Alcoholic serous meningitis (wet brain) is quite frequent in chronic alcoholism. It usually follows an attack of delirium tremens. In such a case the patient from the onset enters into semi-comatous state. Delir- ium is present but not of a violent nature. The patient though affected by hallucinations lies motionless and mutters in a low voice. He can hardly be roused for food. The pulse is rapid, temperature normal. The pupils are small. Hyperesthesia is marked. Recovery is possible especially in those cases in which the neck is not rigid (Dana). In cases with rigid neck Dana believes the patients die. In such cases the semi- comatose state gets deeper and deeper, general rigidity develops, the abdomen gets retracted. If recovery occurs, the condition normally lasts about four weeks. The prognosis depends upon whether complicating conditions are 598 NERVOUS SYMPTOMS PRODUCED BY INTOXICATIONS present or not. It is grave in cases of trauma, infectious diseases, neph- ritis, cardiac weakness, pneumonia. Recurrences are not infrequent. An attack may come on from some insignificant cause, as slight trauma or an ingestion of a small amount of alcohol after a period of abstinence, or else from an intercurrent febrile disease. Pathology of Alcoholism. — Little or no special changes are found in acute alcoholism. In chronic alcoholism the most constant alterations are observed in the blood vessels. Atheromatous changes of the minute blood vessels are conspicuous. They are enlarged, tortuous. In their vicinity neuroglia cells are abundant. The perivascular spaces are filled with lymphoid cells. Pachymeningitis and leptomeningitis, oedema of the brain, pachy- meningitis hemorrhagica are all quite frequent. Degeneration of the cortical cells, especially in the motor areas; also of the descending tracts in the internal capsule and in the spinal cord; finally in the cells of the anterior cornua in the cord have been repeatedly found. In delirium tremens there is a great tendency to minute hemorrhages especially in frontal and central convolutions of the brain, frequently also in the gray matter of the aqueduct of Sylvius and around the third ventricle. As to the peripheral nerves, they particularly are affected by alcohol (see chapter on Multiple Neuritis). Korsakoff's psychosis is described in connection with Alcoholic Multiple Neurit's. Treatment. — Withdrawal of the poison is the first indication. Some believe in gradual reduction of the beverage, others in sudden removal. This can be well accomplished by isolation. Sleep must be induced by all means. Bromides (twenty* grains), paraldehyde (one dram), chloral hydrate (30-60 grains), sulphonal (gr. x), trional (gr. x), veronal (gr. x) are good drugs for insomnia. Morphia (gr. 1/8) associated with chloral (gr. xx) is especially advantageous. A tepid bath of hah an hour's duration is also useful in insomnia. As withdrawal of alcohol may cause collapse or exhaustion, a hypodermic injection of ergot may be very efficient. In case the patient is exceedingly restless, or violent, a hypodermic injec- tion of apomorphine (gr. 1/10) is advisable. After sleep is secured, the regular treatment will consist of a complete or partial rest, moderate outdoor exercises, regular meals, hydrotherapy and internal administration of strychnia. Milk should be the main article of food. Meat is to be avoided. Purgatives (preferably saline) are useful when given two or three times a week. Coffee and tea are to be avoided. Bromides administered regularly and for a prolonged period gave me NERVOUS SYMPTOMS PRODUCED BY INTOXICATIONS 599 very satisfactory results. Hyoscine hydrobromate is also useful. Cap- sicum associated with strychnia is commendable. For the treatment of alcoholic neuritis see chapter on Multiple Neuritis. In the majority of cases of chronic alcoholism institutional treatment is the most efficacious. II. Morphine Intoxication. Morphinism There are two phases to be considered in the history of morphinism. One is due to chronic intoxication, the other to abstention from the poison after a long period of intoxication. Symptoms of Chronic Intoxication. — They usually appear after a few months of use of the poison. The exact time of their development varies with the individual. The symptoms are physical and mental. 1 . The physical manifestations concern the motor, sensory and vaso- motor apparatuses. Motor. — They are: muscular weakness, tremor, incoordination, car- diac weakness, vesical weakness and disturbance of ocular accommoda- tion. Epileptiform convulsions, attacks of angina pectoris are sometimes observed. Sensory. — They are: superficial or deep anaesthesias, hyperesthesias or paresthesias. Vaso-motor. — Diminution of secretions. When the salivary and seba- ceous glands secrete insufficiently, there is a dryness of the mouth and throat, a dryness of the skin. In the latter case the skin is easily irritated and furuncles easily develop. The gastric and pancreatic juices are also diminished and gastro-intestinal disturbances are frequent. The men- strual flow is suppressed. The amount of urine is decreased and renal congestion with albuminuria are sometimes observed. Impotence is frequent. Perspiration on the contrary is increased. A morphinomaniac has a pale face, cold extremities, his temperature is below normal. Cachexia gradually develops and may become alarming, if prompt treatment does not interfere. 2. Psychic manifestations are constant. The patient notices a grad- ual loss of mental energy. His will power is diminished. His thoughts and acts are characterized by an indifference, by apathy. His moral conceptions weaken equally and the tendency to deceive is marked. At the same time he is irritable, has outbreaks of anger upon the least pro- vocation so that at times he may become dangerous. Gradually the mem- ory becomes more and more impaired, the mental faculties low (dementia). Depression is marked and may lead to suicide. (See my article in the 600 NERVOUS SYMPTOMS PRODUCED BY INTOXICATIONS Jour, of Am. Med. Ass., July, 1908.) Insomnia is very marked. Noc- turnal hallucinations of a terrifying nature torture the patient, but they usually disappear during the day. Hallucinations of sight are more frequent than any other form. Symptoms of Abstinence. — They depend upon whether the suppres- sion of morphine is gradual or sudden. In case of gradual removal of the drug there is a certain general malaise with loss of appetite, nausea, vomiting and diarrhoea. Noises in the ear, peculiar visions are present. Neuralgic pains, a general sensation of cold distress the patient. He is also in a state of anxiety and depressed. When the suppression of morphine is complete and sudden, there is, first of all, a sense of great muscular fatigue. The perspiration is abund- ant, the tremor is generalized. Violent abdominal pains with vomiting and diarrhoea are sometimes extreme. A delirium with hallucinations develops rapidly. In some cases there may be a cardiac and respiratory failure and death may be imminent. A hypodermic injection of morphine promptly administered may save the patient. The outlook in morphinomania is unfavorable, as recurrences are frequent. Treatment- — Withdrawal of the poison is the main indication. It can be accomplished very gradually, or rapidly or else abruptly. The gradual method is applicable to patients that are very weak and more or less cachectic; syncope is to be feared in them. In such cases hypo- dermic administration of strychnia fgr. 1/60) several times a day is to be recommended. In robust individuals the rapid suppression is advisable. With the abrupt method one runs a great risk. When cardio-vascular disturbance is present, a sudden withdrawal may bring on a collapse or even death. Whatever method is adapted, during the withdrawal particular atten- tion should be given to the patient's general strength. Alcohol can be used freely. In extreme cases when collapse is threatened, a hypo- dermic of morphia will improve the condition. The gastro-intestinal disturbances, which are so conspicuous, are relieved by sodium bicarbonate. Good hygienic measures, hydrotherapy (cold douches), massage, nutritious food are not to be neglected. Finally the question of prophylaxy is the most important. Morphine should be prescribed only in the most urgent cases and a patient should never be trusted with a syringe and the drug. Institutional treatment is always adorable. TETANUS 6oi Cocaine Intoxication. Cocainism The symptoms of acute cocaine intoxication are: general excitement, restlessness, talkativeness, paraesthesia of the extremities and tinnitus aurium. Sometimes instead of excitation there is on the contrary cere- bral depression, nausea and vomiting, abundant sweating, cold extremities, tachycardia. Epileptiform convulsions may occur. Recovery may fol- low, but death may also take place either in syncope or in convulsions. In chronic cocainism there is at first a tendency to muscular and mental agitation. Gradually the latter becomes permanent. A feeling of exhilaration in the mental and sensory spheres is quite marked. The patient exhibits great activity. Insomnia develops. Hallucinations make then their appearance and persecutory delusions may accompany the hallucinations. In advanced stages of the disease the exaltation period is replaced by a period of depression and even stupor. Sensory phenomena are quite conspicuous. Various paraesthesias are present. Not infrequently the patient complains of a sensation of insects crawling under his skin. Very often there are sharp pains in the extrem- ities. Anaesthesias may be present. The acuity of vision is diminished. Tachycardia, rapid respiration, sweating, anorexia, diarrhoea and emacia- tion are the usual symptoms. In advanced cases cachexia and mental enfeeblement (dementia) are very marked. Syncope may occur at any time. (See my article in the Jour, of Am. Med. Assn., July, 1908.) Treatment.— S ee the preceding chapter. It may be added, however, that sudden withdrawal of cocaine is preferable to the slow method. CHAPTER XXIX NERVOUS SYMPTOMS CAUSED BY SOME SPECIAL INFECTIONS TETANUS (LOCKJAW) The disease is due to a special bacillus discovered by Nicolaier in 1885. Pathology. — There are no characteristic changes in the brain and spinal cord, but the toxins elaborated by the tetanus bacillus reach the central nervous system through the nerve trunks. Hyperemia, oedema and minute hemorrhages in the brain and meninges have been observed. Congestion and softening, also changes in the cells of the cord and medulla have been found. Symptoms. — A few days after the infection the muscles of the jaw and the neck become rigid, producing the symptom trismus, or "lockjaw." The patient is unable to open the mouth freely. The masseters are very hard to touch. The facies is immobile, forehead wrinkled, corners of the mouth retracted, lips protruded, eyes partly closed. This expression of the face is given the name of "risus sardonicus." Gradually the bilateral tonic spasm increases and spreads. The muscles of the trunk and abdomen become involved. The patient's body is arched, resting on his heels and the back of his head (opisthotonos). The spasm of the diaphragm interferes with the breathing. The involvement of the pharyngeal muscles produces difficulty in swallowing. Gradually the muscles of the extremities become involved, although the hands are usually spared. The patient is confined to bed, his entire musculature is rigid, the breathing is difficult, the jaws are tightly pressed against each other, the head is drawn backward. The last touch or handling of the patient causes a convulsive contraction. Sometimes the latter occurs spontaneously. It causes violent pain. Voluntary urination and defeca- tion are impossible. Constipation and retention of urine are present. Sweating is a constant symptom. The temperature rises only before death. The pulse is accelerated. A moderate degree of leucocytosis has been observed by Brown and others. Sensations are normal. The mentality is preserved. Course, Duration, Prognosis.- — In the acute case death may occur in a few days. Life is in danger either from asphyxia (laryngeal spasm), 602 TETANUS 603 cardiac paralysis, profound exhaustion or excessive rise of temperature. In subacute cases the outlook is favorable. In the acute forms the mortality is high in cases occurring early after severe trauma. The most favorable outlook is in cases which develop late after trauma. Complications may develop or follow tetanus. Among them should be mentioned: nephritis, bronchitis or bronchopneumonia from difficulty of expectoration, rupture of muscles, fracture of the spinous processes from violent spasms. Sometimes for months after recovery a stiffness of muscles persists. Mental disturbances, suppuration of submaxillary and parotid glands have also been observed after recovery from tetanus. Diagnosis. — Strychnine poisoning and hysteria should be always thought of in making a diagnosis. In the first the onset is rapid; gastric disturbances or tetanic contractions of the extremities appear first, while in tetanus trismus is first. Besides, the convulsions are violent and present periods of complete relaxation; the retina is hyperesthetic and- objects look green; duration is brief — either recovery or death. Be- sides, a history of poisoning is present. In hysteria there is no trismus. The presence of hysterical stigmata will decide the diagnosis. Hydrophobia is recognized by absence of trismus and opisthotonus by violence of repiratory spasms and by psychic disturbances. Etiology. — The usual mode of infection with Nicolaier's bacillus is through a wound. The epidemic or sporadic character of tetanus is well known. It is more prevalent in the hot than in the cold months of the year. Infection may also take place in new-born children through the um- bilicus. This is tetanus neonatorum. Trismus is here the most con- spicuous symptom, which prevents the infant from taking nourishment. The prognosis is very grave. Treatment. — Prophylaxis, consisting of rigorous antiseptic measures, is urgent in every case of infection. As soon as the wound occurs, thorough disinfection and thorough cauterization of it must be done as promptly as possible. Immediately or very shortly after, antitoxin as a preventive measure should be used. About 500 units should be injected for this purpose. The early adminis- tration of antitoxic serum is of great importance and Behring urges its administration within the first twenty-four hours. Unless it is injected before trismus makes its appearance, its value is very small. Vaillard {Bull, de VAcad. de. Med., 1908) injected the serum as a prophylactic measure in 13,124 horses and none contracted tetanus. His observations also show that when tetanus developed notwithstanding the preventive 604 TETANUS injections, its course was milder. The high mortality depends largely upon the late use of the serum. As to the amount to be injected, it is advisable to inject at first 2.25 grm. and 0.5 grm. for each of the following doses. In extreme cases instead of subcutaneous injections the serum may be injected into the spinal canal after a small amount of cerebro- spinal fluid is allowed to escape. Although satisfactory results have been reported, nevertheless a great many failures have been observed. General measures should not be neglected. Absolute quiet, avoidance of manipulation of the body are necessary. Feeding should be done through the nasal cavities, if trismus exists. Bromides, chloral, morphia, warm baths will be utilized for relief of the tonic spasms. In spite of the spasms, cardiac stimulants are sometimes necessary. Alcohol, strychnine, digitalis, camphor are the most useful drugs. The latter three are to be given hypodermically. Care must be taken of the functions of the viscera. In 1905 Meltzer proved experimentally that magnesium possesses an inhibiting power over the processes of the body, viz. inhibits respiration and produces paralysis when given by intravenous injections; when in- jected subcutaneously, it produces narcosis with muscular relaxation; when injected into the spinal canal it produces anesthesia. Since then it has been shown that an intraspinal injection of magnesium sulphate in cases of tetanus controls the convulsions and leads to recovery. The quantity to be injected is 1 c.c. of 25 per cent, solution to each 25 pounds of body weight. The injections may be repeated several times in case the results of the first injections are nol entirely satisfactory. Kocher (Corresp.-Blatt f. Schweiz. Aerzte., 1912) believes that 15 per cent, or even 10 per cent, solution of magnesium sulphate is sufficient. The greater susceptibility of children to toxic action must be borne in mind: 2 c.c. should be the maximum dose for a child. In view of the cumulative action of the drug, repetition of the dose should be made not oftener than at an interval of twenty-four hours. Kocher warns that if no cerebro-spinal fluid flows upon lumbar puncture, injection of magnesium sulphate is dangerous. The injections of magnesium may be associated with administration of antitetanic serum. This latter com- bined method has given highly favorable results. Baccelli treats tetanus with injections of carbolic acid in 2 per cent, solution. He commences with a daily dose of 0.30 to 0.50 watching care- fully the condition of the urine and the patient's tolerance. Gradually he increases the dose to 1 grm. in several injections in twenty-four hours. In very grave cases higher doses may be given. Baccelli claims (Berl. HYDROPHOBIA 605 klin. Wchn., 191 1) that with his method the mortality in his ordinary cases has been only 2.12 per cent, and in the very grave cases 18.5 per cent. Atkey (Lancet, 1913) basing himself on the effects produced by Souttar by injecting paraldehyde and ether intravenously, used these drugs in conjunction with copious injections of normal saline solution in treatment of tetanus. In a case of a boy of 19, he made twelve intravenous injections of a solution of paraldehyde and ether, 5 c.c. each in 150 c.c. of normal solution and the result was excellent. The boy recovered. The drugs apparently act as an anaesthetic and hypnotic. Under the name of Cephalic Tetanus is described a variety of tetanus which is characterized by spasms confined mainly to the head and face and which results from injuries of the head. Spasm of the facial muscles, then trismus, spasms of pharyngeal, laryngeal and respiratory muscles are the symptoms observed. The rigidity may spread to other muscles. This form is also called hydrophobic tetanus, because of the spasmodic contractions of the oesophagus. The majority of cases ends fatally. Hydrophobia. Rabies. Lyssa The infectious element of this disease is not yet known. It attacks animals and from them is communicated to man by inoculation through a bite. The exact nature of the virus is unknown. Pathology. — In 1900 Van Gehuchten and Nelis discovered what they believed to be of diagnostic value, changes in the intervertebral ganglia, consisting of proliferation of cells, probably from the capsules of the ganglia. The ganglionic cells themselves degenerate, inasmuch as the pro- toplasma with the nucleus in some cases totally disappears and is re- placed by the newly formed small cells. These changes were found not only in the spinal, but also in the pneumogastric and Gasserian ganglions. Babes also described a so-called "rabic tubercle," consisting of an accumulation of embryonal cells around the vessels and nerve cells of the anterior cornua of the cord and in the medulla. The cells of the latter undergo degeneration, viz. chromatolysis with disappearance of the nuclei; the pericellular spaces are dilated and filled with the characteristic embryonal cells. I have seen the same "rabid tubercles" around the pyramidal cells of the cortex in several cases of rabies. In 1903 Negri's bodies became a new element in the diagnosis of rabies. They are found in the nerve cells and in their largest dendritic prolongations especially of the cornu ammonis but also of the cortex, pons, medulla and spinal ganglia. These bodies are of 1 to 27 ,« in size, round or oval; they are surrounded by a hyaline membrane. They are very resistant and may be pre- 6o6 HYDROPHOBIA served for many days when the tissue is preserved in glycerine. The method for bringing them out to the best advantage is as follows: Impregnate the tissue in a solution with a sublimate base, especially a sublimate with acetic alcohol of Gilson or sublimate of von Lenhossek. Next step is to cover the piece with a slight layer of thick celloidine, dry in the air for a few minutes, place tissue in: alcohol absolute 50 c.c.+ chloroform 5 c.c. Avoid xylol for clearing, but use toluidin or cedar oil. It is admitted now that Negri's bodies are pathognomonic of rabies. As to their nature, Negri believes them to be parasites undergoing degeneration. Fig. 169. — Rabies. Spinal Ganglion. {Original.) Symptoms. — After a period of incubation, which may last from a few days to two months (in children shorter than in adults), the infected part produced by the bite of the animal begins to itch and burn; some- times there is pain, lancinating in character. Headache, insomnia and anorexia develop next. The patient becomes depressed and irritable. A state of anxiety appears and this is increased by a rigidity of the muscles of the throat and difficulty in swallowing. At the same time the tempera- ture rises slightly, the pulse is accelerated. Soon another phase of the disease makes its appearance. It is char- acterized by restlessness, cerebral excitement and hyperaesthesia. The least noise, a bright light, a slight breeze causes a spasm. The latter affects especially the muscles of the larynx and pharynx. As there is intense thirst, the patient makes great efforts to drink, but an attempt to HYDROPHOBIA 607 take water or the sight of water or even the suggestion of it produces a pain- ful spasm of the pharynx and larynx (hydrophobic spasm). Dyspncea is then pronounced. The fear of water (hydrophobia) is characteristic of the disease. The difficulty of swallowing makes the saliva dribble from the mouth. The temperature goes up to ioi°-io3°. At the same time a delirium with hallucinations and delusions develop. The convulsive at- tacks which were at first confined to the muscles of deglutition and respiration, now become generalized and may be so frequent that asphyxia occurs. They are brought on by the same excitants which produce the spasm of pharynx and larynx. During the convulsions patients make noises resembling the barking of a dog. The delirium is usually marked during the paroxysms; the patient may become violent, inflict injuries on himself or bite others and spit at them. This phase of the disease usually lasts one or three days. This may be followed by a para- lytic stage, during which all the previous symptoms gradually abate and the patient becomes quiet. Gradually he sinks into unconsciousness and dies. Paralytic Rabies.— In exceptional cases the convulsive phase is absent and there is only the paralytic phase. It is a very grave condition. It usually follows very extensive bites. Paralysis rapidly develops and assumes the type of acute ascending paralysis. Death follows from respiratory or cardiac paralysis. Course, Duration, Prognosis. — The rapidity of development of the symptoms depends upon the part of the body bitten. Wounds on the face present the greatest danger. The prognosis is grave, except when antitoxic treatment is early instituted. Death usually occurs within two or five days. Diagnosis. — -In tetanus there is also a spasm of deglutition and of respiratory muscles, but the presence of trismus and absence of fear of water will decide the diagnosis. Hysterical paroxysms may sometimes simulate rabies (pseudo-hydro- phobia), but the special symptoms of hysteria will soon determine the disease. Treatment. — As soon as the bite occurs, a ligature is placed above the wound and kept in place several hours. The latter should be enlarged and a thorough cauterization, followed by an antiseptic dressing, be done. It should be left open and free drainage given for several weeks. As soon as possible the antirabic serum should be employed. Since Pasteur introduced this treatment, the mortality has greatly decreased. The other symptoms of the disease are treated accordingly. Perfect quiet, sedatives (bromides, chloral, chloroform, morphia), rectal feeding, if not possible by the mouth, are practically all that can be done. 608 HYDROPHOBIA NERVOUS MANIFESTATIONS IN PELLAGRA The nature of the disease is still unsettled. The old view with relation of the disease to ingestion of ma'ize is not held by all and considered as doubtful. Sambons idea in regard to an infectious origin of the disease is viewed with favor. The endemic mode of propagation, its appearance at certain seasons, the failure of all prophylactic measures based on the theory of ma'ize, all speak in favor of infection. It is supposed that pellagra is an insect-borne disease. The manifestations of pellagra concern the gastro -intestinal tract, skin and the nervous system. The affection begins usually with gastro-intestinal and psychic dis- turbances. Gastritis and diarrhoea are frequent in the early stages. The patient is depressed, indifferent to surroundings and to his own appearance, or else may have maniacal outbursts. He is forgetful, commits errors of serious nature. Sudden lapses of memory resembling Petit Mai, vertigo are not infrequent. Exceptionally delusions and hallucinations are observed. Suicidal tendencies are not rare. Paresthesias, headache, pain in back or in the extremities of tabetic nature, hypaesthesias or anaesthesias, tremor of the hands and tongue resembling that of multiple sclerosis, muscular spasm, Romberg's sign, increased patellar tendon re- flexes, occasionally Babinski's sign — are symptoms which may all or only some of them be encountered in Pellagra. Eye Symptoms. — Early forming cataracts are frequent. Retinitis and optic neuritis are not uncommon. The choroid is quite frequently involved. The skin manifestations consist of symmetrical in shape and position erythemata resembling sunburn; they are seen on the exposed parts of the body, especially on the hands. Desquamation is the ultimate result. Changes in the postero-lateral tracts of the spinal cord have been found in some cases. Treatment. — Mild cases require no treatment. In cases with severe gastro-intestinal disorders and asthenia, arsenic is indicated. Nutri- tious diet with rest are the chief measures to be relied upon. The mortality is quite high. INDEX Abasia, 468 Abducens nerve, 401 Abscess of brain, 106 course, 109 diagnosis, no etiology, 106 pathology, 107 prognosis, no symptoms, 108 treatment, in Abscess of cerebellum, 218 Abstinence, 600 Abulia, 458 Achilles tendon reflex, 247 Achondroplasia, 576 Acromegaly, 572 Acroparsesthesia, 583 Acustic, striae, 15 tubercle, 15 Acute ascending paralysis, 396 Adiadochokinesia, 215 Adiposis dolorosa, 577 Adipositas, 154 Affect, 479 Agenesis, 117 Agoraphobia, 457 Agraphia, 78, 131, 149 Akinesia algera, 533 Ala cinerea, 15 Albumen, 371 Alcoholic injections, 433, 436 intoxication, 595 neuritis, 387 Alexia, 78, 131, 133, 150 Allochiria, 69 Alternating ptosis, 402 Amaurosis, 467 Amaurotic form of tabes, 250 Ammonis cornu, 81 Amnesia, 153, 367, 469, 484, 550 verbal, 135 Amusia, 132 Amyotonia congenita, 327 Amyotrophic lateral sclerosis, 319 Anaesthesia, 68, 245, 292, 309, 333, 387, 391, < 417, 4i8, 427, 465 Analgesia, 245 Anarthria, 227 Anemia of brain, 202 Anencephalia, 52 Aneurism, 87, 144 Angioma, 144 Angioneuroses, 560 Angioneurotic oedema, 584 Angular gyrus, 131 Ankle-clonus, 71, 90 Anosmia, 399 Anterior commissure, 3 cornua, 3 crural nerve, 425 poliomyelitis (acute), 264 39 Anxiety, 457 neurosis, 464 Aphasia, 78, 96, 130, 149, 469 of conduction, 133 congenital, 135 motor, 130 optic, 135 sensory, 132 transcortical or pure, 134 Aphemia, 78, 130 Aphonia, 228 Apoplexy, 82 delayed, 101 ingravescent, 89, 101 spinal, 280 Apraxia, 138, 154 Aqueduct of Sylvius, 21, 163 Arachnoid, of brain, 45 of spinal cord, 9 Aran-Duchenne, 314 Argyll-Robertson pupil, 246, 402 Arhinencephalia, 55 Arsenical intoxication, 592 paralysis, 390 preparations, 253 Arteries, anterior cerebral, 46 basal, 48 internal carotid, 46 middle cerebral, 48 posterior cerebral, 48 vertebral, 48 Arthritic muscular atrophy, 327 Arthropathies, 247, 289 Ascending neuritis, 387 paralysis, 396 Asphyxia (local), 587 Association area, 79 method, 463 Astasia-abasia, 60, 468 Astereognosis, 69, 79, 95, 150 Asthenia, 215 Asthenic bulbar paralysis, 231 Asymbolia, 69 Ataxia, 60, 167, 213, 220, 242, 244 Friedreich's, 261 Ataxic paraplegia, 259 Athetosis, 61, 116, 167, 262, 503 Atonia, 327 Atremia, 534 Atrophy, 62, 116, 267, 288, 332 arthritic, 327 dystrophy, 321 of brain, 114 of muscles, 314, 417 primary neuritic, 325 progressive of infants, 318 progressive of spinal origin, 3i4 Auditory apparatus, 20 nerve, 411 Aura, 470, 482 609 6io INDEX Automatism, 469 Autosuggestion, 469 Babinski, 73, 91, 479 Bar any, 404 Basal ganglia, 27, 152, 167 meningitis, 340 Base of brain, 151 Basedow's disease, 560 Bell's palsy, 406 Benedict's syndrome, 239 Beriberi, 392 Betz cells, 76 Birth palsy, 417 Blepharospasm, 467, 515 Brachial neuralgia, 437 plexus, 416 Brachium, anterior, 20 posterior, 20 Breuer, 463 Brissaud, 569 Broca's aphasia, 130, 131, 192 Brown-Sequard's paralysis, 283 Brudzinski's sign, 177 Burdach's columns, 6, 243 nucleus, 14 Cachexia strumipriva, 567 Caisson disease, 284 Calamus scriptorius, 15 Cancer of vertebras, 311 Capsule, external, 30 internal, 31 Carbonic gas intoxication, 593 neuritis, 391 Catalepsy, 470 cerebellar, 215 Cauda equina, 10, 293 Caudate nucleus, 27 Centers, 75 auditory, 78, 80 gustatory, 80 intelligence, 81 motor, 75 olfactory, 80 sensori-motor, 76 sensory, 79 speech, 78 stereognostic, 79 visual, 80 writing, 78 Central canal, 3 Cephalalgia, 535 Cerebellar abscess, 218 asynergy, 214 ataxia, 213, 262 catalepsy, 215 heredo-ataxia, 219 peduncles, 19 posterior inferior artery, 235 Cerebellum, 40, 212 Cerebral hemispheres, 26 hemorrhage, 82 localization, 75 Cerebritis, 104 Cerebro-spinal fluid, 371 Cervical enlargement, 1 nerves, 416 pachymeningitis (hypertrophic), 331 Cervico-occipital neuralgia, 436 Charcot, 288, 319 Choked disc, 147, 215, 400 Choleastoma, 145 Chorea, 61, 116, 167, 262, 495 chronic, 510 hereditary, 501 Huntington's, 501 electric, 519 Dubini's, 519 Choroid plexus, 15, 46 tela, 15 Chronic chorea, 501 Chwostek's sign, 521 Cingulum, 37 Circle of Willis, 48 Circumflex nerve, 419 Circumscribed serous spinal meningitis, 333 Cisterna magna, 184 Clarke's column, 6, 288 Claudication, 334 Claustrophobia, 457 Clavus hystericus, 466 Claw-like hand, 288, 315, 422, 428 Cocaine intoxication, 601 Coccygodynia, 446 Cochlear nerve, 411 Columns, 6 of Burdach, 6 of Goll, 6 Combined sclerosis, 259 Commissural fibers, 37 Commissure, 3 anterior, 38 Compression of medulla, 235 of cord, 303 hippocampal, 38 Concussion of brain, 209 of cord, 302 Conjugate deviation, 89 movement, 402 Contraction of visual fields, 467 Contracture (secondary), 92, 153 Contusion of cord, 302 Conus medullaris, 1, 291 Conversion, 479 Convulsions, 147, 150, 151, 163, 176, 192, 483 Coprolalia, 508 Cord, 1 Cornua, 3 anterior, 3 lateral, 6 posterior, 6 Corona radiata, 31 Corpora quadrigemina, 169 striata, 27, 168 Corpus callosum, 31, 152, 153 Coxalgia, 441, 468 Craniorrhachischisis, 57 Cretinism, 570 Crisis, 245, 249 Crossed pyramidal tract, 8 paralysis, 234, 237, 239 INDEX 6ll Crura, 19, 20 Crural (anterior) nerve, 425 neuralgia, 441 Crutch palsy, 421 Cushing, 121 Cyclopia, 55 Cycloplegia, 402 Cysticercus, 145 Cysts, 145, 158, 162, 306 Dancer's cramp, 528 Deafness, 411 Decompression (cerebral), 160 Decubitus, 277 Decussation, 8 of pyramids, 1 1 Defense-neurosis, 479 Deficient will, 458 Degeneration, ascending, 242 descending, 85 Dejerine, 334, 383 Delire du toucher, 457 Delirium, 108, 176, 192, 210, 471 tremens, 595 Dementia, 153, 258, 357, 484, 601 paralytica, 353 Dendrites, 49 Dentate ligaments, 10 nucleus, 40 Diaphragmatic phenomenon, 41 Diphtheritic paralysis, 390 Diplegia, 61, 117 Diplomyelia, 58 Diplopia, 246, 401 Direct cerebellar tract, 8 pyramidal tract, 8 Disseminated sclerosis, 295 diagnosis, 300 forms, 299 pathology, 295 prognosis, 300 symptoms, 296 treatment, 301 Divers' paralysis, 284 Double personality,. 469 Doubts, 457 Dreamy state, 482 Dubini's chorea, 519 Dura mater, of brain, 43 of spinal cord, 9 Dysarthria, 227 Dysbasia lordotica progressiva, 505 Dyschromatopsia, 147 Dyskinesia algera, 533 Dysopsia algera, 534 Dystonia musculorum deformans, 505 Dwarfishness, 576 Early paresis, 366 Echinococcus, 145, 158 Echokinesis, 511 Echolalia, 131, 511 Ehrlich, 253 Electrical contractility, 63, 270 Electric chorea, 519 Elephantiasis, 574 Embolism, 86 Encephalitis, 104, 354 acute, 104 chronic, 112 congenital, 115 non-suppurative, 104 suppurative, 106 Encephalocele, 55 Encephalopathy, 363, 591 Endothelioma, 144 Epiconus, 295 Epicritic, 70 Epidural injections, 443 Epilepsy, 117, 482 equivalents, 487 major (Grand Mai), 482 minor (Petit Mai), 485 psychic, 486 reflex, 491 senile, 490 partialis continua, 128 Jacksonian or focal, 124 diagnosis, 126 etiology, 127 pathology, 124 prognosis, 126 symptoms, 124 treatment, 128 Epileptic character, 487 Epiphysis, 26 Epithalamus, 26 Equilibration, 545 Equino-varus, 427 Erb, 231, 309, 323, 346 Erb's paralysis, 417 sign, 521 Erythromelalgia, 585 Exophthalmic goiter, 560 Exsner, 255 External cutaneous nerve, 426 Fabrications, 388 Facial hemiatrophy, 581 hemihypertrophy, 583 hemispasm, 467 nerve, 406 neuralgia, 435 paralysis, 406 spasm, 514 Falx cerebelli, 44 cerebri, 44 Family spastic paralysis, 257 Faradism, 63 Fatigue, 455 Festination, 531 Fibers, ascending, 32 association, 37 commissural, 37 descending, 35 frontal, 36 inferior longitudinal, 37 internal arcuate 17 motor, 35, 49 occipital, 35 occipito-frontal, 37 sensory, 35, 49 6l2 INDEX Fibers, superior longitudinal, 37 temporal, 36 uncinate, 37 Fibril ary contractions, 316, 325, 51S Fillet, 16 Filum terminale, 1 Fissures,. 3 longitudinal, 26 Fixed idea, 478 Flechsig's tract, 8 Flexner, 194, 265 Focal epilepsy, 1 24 Folie de doute, 457 Foot-drop, 386, 391, 427 Fornix, 40 Forster's operation, 122, 254 Four reactions, 374 Fourth ventricle, 15 Fovea, 15 Fracture of skull, 205 Franke, 254 Free association method, 463 Frenkel, 255 Freud, 463 Friedreich's ataxia, 220, 261 Frohlich's syndrome, 154 Funiculus teres, 15 Galvanism, 65 Ganglia, basal, 27, 167 Gasserian, 434, 436 spinal, 243 Gas poisoning, 169 Gastric crises, 249 tetany, 523 Gelatinous substance (Rolando), 6 General paralysis of the insane, 353 Geniculate bodies, 20, 25 Gigantism, 575 Gilles de la Tourette, 508 Gill's operation, 100, 121 Girdle pain, 245, 274 Glioma, 143, 158 Globus hystericus, 467 pallidus, 30 Glosso-pharyngeal nerve, 412 Gluteal nerves, 426 Goll's columns, 6, 243 nucleus, 13 Gordon, 73, 91, 93, 150 Grand Mai, 482 Graves' disease, 560 Gray substance, 3 Gumma, 144, 340 Haines' operation, 184 Hallucinations, 359 Head, 70, 447 Headache, 146, 339, 451, 535 indurative, 538 Hearing, 70 Hematoma, 171, 206 of the ear, 358 Hematomyelia, 279 Hemianesthesia, 94, 98, 150, 167, 465 Hemianopsia, 80, 140 Hemiasynergy, 214 Hemiataxia, 167 Hemiatrophy, 415, 581 Hemichorea, 497 Hemicrania, 539 Hemihypertrophy, 583 Hemiplegia, 61, 90, 167 crossed, 234 infantile, 112 syphilitic, 341 Hemispasm, facial, 467 Hemorrhage, cerebral, 82, 97 diagnosis, 96 etiology, 85 localization, 98 pathology, 82 prognosis, 96 symptoms, 88 treatment, 99 Hemorrhage of cerebellum, 221 medulla, 234 meningeal, 100, 113, 333 of pons, 237 in spinal cord, 279 Hereditary chorea, 501 syphilis, 349 Heredo-ataxia (cerebellar), 58 Herpes zoster, 436, 447 Heterotopia, 58 High steppage gait, 60 Hippocampal commissure, 38 Hippocampus, 81 Hippus, 405 Hoche's bundle, 8 Hoffmann's sign, 521 Hoover's phenomenon, 475 Horsley, 161 Horsley's operation for epilepsy, 128 Hunt, R., 407, 424 Huntington's chorea, 501 Hydatid cysts, 306 Hydrocephaloid anemia, 202 Hydrocephalus, 114, 162 acute, 162 chronic, 162 diagnosis, 164 etiology, 165 external 162 internal, 114 pathology, 162 symptoms, 163 treatment, 165 Hydromyelia 286 Hydrophobia, 605 Hypaesthesia, 68, 465 Hypersesthesia, 68, $33, 387, 466 Hyperalgesia, 246 Hyperemia, 203 Hpyerplasia, 54 Hypertrophic cervical pachymeningitis, 331 Hypertrophy, 62 of brain, 115 Hypnotism, 477, 478, 481 Hypochondria, 458 Hypoglossus nerve, 415 INDEX 613 Hypophysis, 26 Hypoplasia, 52 Hypothalamic nucleus, 22 Hypotonia, 61, 215, 244, 506 Hysteria, 464 Hysterical hemiplegia, 475 insanities, 472 paroxysms, 470 Hysteroepilepsy, 472 Hysterogenetic zones, 466 Hysteroid, 485 Idiot, 569 Incipient tabes, 251 Indurative headache, 538 Infantile hemiplegia, 112, 115 spinal paralysis, 264 Infantilism, 569, 576 Ingravescent apoplexy, 89, 101 Insomnia, 148, 452 Insular sclerosis, 295 Intention tremor, 262, 297 Intercostal neuralgia, 438 zona, 448 Intermittent claudication, 445 of spinal cord, 334 closing of cerebral arteries, 102 Internal capsule, 31, 99 Intracranial pressure, 145 Iridoplegia, 402 Jacksonian epilepsy, 124 Janet, 478, Jargonaphasia, 132 Jendrassik's sign, 247 Juvenile paresis, 261 tabes, 256 Kakke, 392 Keratitis, neuroparalytic, 405 Kernig's sign, 176, 192, 330 Kleptomania, 458 Klumpke, 310, 418 Knee-jerk, 72 Kocher's operation for epilepsy, 128 Kojewnikoff, 128 Korsakoff, 210, 388 Krause, 166, 218 Laceration of cord, 303 Lacunes, 83, 258 Laminectomy, 312, 333 Lancinating pain, 245 Landouzy-Dejerine type of muscular atrophy, 323 Landry's paralysis, 396 Larnygeal nerve, 413 Lateropulsion, 60, 531 Lead intoxication, 363, 591 palsy, 389 Lemniscus, 16 lateral, 17, 21 median, 17, 21 Lenticular degeneration, 168 nucleus, 29 zone, 137 Lepra neuritis, 393 Lethargy, 470 Letter blindness, 133 Leucocytosis, 372 Liepman, 138 Ligula, 15 Limbic lobe, 81 Lissauer's tract, 7 Little's disease, 112, 117, 257 diagnosis, 120 pathology, 117 prognosis, 120 symptoms, 119 treatment, 120 Local asphyxia, 588 syncope, 588 Lockjaw, 602 Locomotor ataxia, 242 Locus niger, 21 Long thoracic nerve, 418 Longitudinal fissure, 26 Lumbar enlargement, 1, 310 neuralgia, 444 puncture, 128, 160, 166, 182, 208, 369, 375 Lumbo-abdominal neuralgia, 444 -sacral nerves, 425 Luys body, 22 Lymphocytosis, 189, 358, 374 Lymphorrhagia, 231 Lyssa, 605 Macrocephaly, 55, 576 Magnesium sulphate, 604 Main en griffe, 288, 315 Malformations, 51 Mai perforant, 248 Mammillary bodies, 40 Manganese intoxication, 594 Marantic thrombosis, 199 Marie, P., 78, 136, 219 Mastodynia, 438 Median nerve, 424 Medico-legal considerations, 551 Medulla oblongata, 11, 234 compression of, 235 Meniere's disease, 545 Meningeal hemorrhage, 100, 113 Meninges, of brain, 43 of spinal cord, 9 Meningitis, 171 leptomeningitis, 173 acute, 173 "B non-tubercular, 173 circumscribed, 178, 333 gonoccal, 180 grippal, 179 in lcoholics, 181 in old age, 181 otitic, 180 pneumococcus, 179 serous, 178, 597 syphilitic, 180, 340 traumatic, 179 typhoid, 179 tubercular, 185 aseptic cerebro-spinal, 198 614 INDEX Meningitis, leptomeningitis, aseptic cerebro- spinal basal, 340 basic, 193 chronic, 197 epidemic, 190 spinal, 269, 329 Meningismus, 178, 182 pachymeningitis, 171, 331, 344 Meningocele, 55 Meningococcus, 174, 372 Meningoencephalitis, 250, 338 Meningomyelitis, 344, 345, 346 Meningomyelocele, 56 Mental disturbances, 148, 153, 210, 220, 341, 348, 356, 359, 366, 388, 562, 595, 599 Meralgia paraesthetica, 444 Mercurial neuritis, 392, 593 Metallic poisons, 591 Metatarsalgia, 447 Microcephalia, 53 Microgyria, 53, 115 Micromyelia, 576 Midbrain, 20 Migraine, 539 Millard-Gubler's syndrome, 239 Milroy's disease, 585 Mirror writing, 131 Mobius' sign, 561 Monophobia, 457 Monroe foramen, 38, 163 Moria, 148 Morphea, 579 Morphine intoxication, 599 Morvan's disease, 290 Motor area, 75, 149 pathway, 8 phenomena, 59 Multiple neuritis, 385 alcoholic, 387 arsenical, 390 beriberi, 392 carbonic gas, 391 diphtheritic, 300 lead, 389 lepra, 393 mercurial, 392 puerperal, 392 senile, 394 Multiple sclerosis, 295 Musculocutaneous nerve, 420 -spiral nerve, 420 Myasthenia gravis 231 Myasthenic reaction, 232 Myatonia congenita, 327 Myelitis, 272 acute, 272 diagnosis, 276 etiology, 273 pathology, 272 prognosis, 275 symptoms, 273 treatment, 276 chronic, 277 diffuse, 277 disseminated, 277 syphilitic, 275 transverse, 273 Myelitis, tubercular, 275 Myelocele, 56 Myoclonia, 61, 516 Myoclonia with epilepsy, 518 Myokymia, 518 Myopathy, 321 facio-scapulo-humeral type, 323 pseudo-hypertrophic type, 322 scapulo-humeral type, 323 Myosis, 246, 309 Myospasm, 524 Myotonia atrophica, 329 congenita, 525 Myotonic reaction, 526 Myx oedema, 567 strumipriva, 569 Natatory chorea, 468 Neck reflex, 177 Negri's bodies, 605 Neosalvarsan, 350 Nerve-cell, 49 -fibers, 49 -stretching, 434 Neuralgia, 430 Neurasthenia, 451 sexual, 453 symptomatica, 454 Neurasthenoid, 362 Neurectomy. 434 Neuritic atrophy, 325 Neuritis, 377 ascending, 381 etiology, 380 hypertrophic interstitial, 383 ischemic, 383 multiple, 385 occupation, 423 pathology, 377 periaxile, 379 prognosis, 382 segmentary, 379 symptoms, 382 treatment, 383 Neurofibrils, 49 Neuroglia, 50 Neurolysis, 443 Neuroma, 384, 516 Neurones, association, 51 doctrine, 49 motor, 8, 50, 320 sensory, 8, 9, 51, 243 Neuroparalytic keratitis, 405 Neuropathy, 456 Neurorhexis, 434 Neuroses (occupation), 527 Neurotropic action, 351 Night terrors, 487 Noguchi, 365 Nonne's Phase I, 356, 358 Nucleus, accessory, 40 caudate, 27 cuneatus, 14 dentate, 40 of fourth nerve, 22 gracilis, 13 INDEX 615 Nucleus, hypothalamic, 22 lenticular, 29 pontis, 19 red, 21, 23 of third nerve, 22 Nystagmoid tic, 508 Nystagmus, 172, 215, 298 acquired, 403 congenital, 403 horizontal 403 miners', 404 rotatory, 403 vertical, 403 vestibular, 404 Obex, 15 Obsessions, 457 Obstetrical palsy, 417 Obturator nerve, 426 neuralgia, 444 Occupation neuritis, 423 neuroses, 527 spasms, 527 Ocular palsies, 402 Oculo-motor nerve, 401 -pupillary disturbances, 283, 417, 41^ (Edema (of brain), 145 angioneurotic, 584 hereditary, 585 of the papilla, 172 Olfactory nerve, 399 Olives, 12, 17 Ophthalmic herpes zoster, 436, 448 Ophthalmoplegia, 170 progressive nuclear, 224, 401 Opisthotonos, 602 Opotherapy, 565 Oppenheim, 73, 91, 327, 505 Optic aphasia, 135 apparatus, 20, 40 atrophy, 215, 246, 298, 400, 573 nerve, 400 neuritis, 109, 147, 176, 243, 400, 573 radiations, 32 thalamus, 167 Osteoma, 145 Pachymeningitis, 171, 307, 331, 344, 355 hemorrhagic, 111 Palpebral tic, 508 Papilla, 217 Paradoxical reflex, 73, 91, 150 Paresthesia, 68, 467 Paragraphia, 131, 132 Paralysis agitans, 529 acute ascending, 396 alternate, 151 associative, 402 Brown-Sequard's, 283 complete, 61 crossed, 151, 234, 237 flaccid, 61, 90, 266, 398 family spastic, 257 incomplete, 61 motor, 153 obstetrical, 120 Paralysis, periodic, 398 spastic, 61, 90, 346 spinal (infantile), 264 Paralysis of cranial nerves, 399 first nerve, 399 second, 400 third, 401 fourth, 401 fifth, 405 sixth, 401 seventh, 406 eighth, 411 ninth, 412 tenth, 412 eleventh, 414 twelfth, 415 Paralysis of spinal nerves, 415 upper cervical, 415 phrenic, 415 lower cervical, 416 brachial plexus, 416 lumbo-sacral, 425 Paralytic chorea, 497 Paramyoclonus multiplex, 517 Paramyotonia congenita, 526 Paraplegia, 61, 117, 119, 132, 256, 258, 259 Parasyphilitic diseases, 252, 336 Paresis, pathology, 353 diagnosis, 362 etiology, 363 prognosis, 362 symptoms, 356 treatment, 363 Parkinson's disease, 529 Pathetic nerve, 401 Pathogenetic link, 463 Pavor nocturnus, 487 Peduncles, cerebral, 19 cerebellar, 19 Peduncular syndrome, 241 Perforated space (posterior), 20 Pellagra, 608 Perineal neuralgia, 447 Periodic paralysis, 398 Peroneal nerves, 426 type of muscular atrophy, 325 Persuasion, 462, 480 Petit mal, 485 Phobia, 457 Photophobia, 187 Phrenic nerve, 415 Pia mater of brain, 45 of spinal cord, 9, 10 Pianist's cramp, 528 Pineal body, 26 Pithiatisme, 479 Pituitary body, 26, 154, 574 Pleurodynia, 438 Pneumogastric nerve, 412 Polioencephalitis, 104, 112 inferior, 226 superior, 223, 224 Polioencephalomyelitis, 224 Poliomyelitis (acute), 264 etiology, 265 diagnosis, 269 6i6 INDEX Poliomyelitis, pathogenesis, 265 prognosis, 267 symptoms, 266 treatment, 270 Polynucleosis, 372 Pons, 16, 19, 236 Ponto-cerebellar angle, 217, 240 Popliteal nerves, external, 427 internal, 428 Porencephaly, 52, 114 Posterior columns, 6 cornua, 6 longitudinal bundle, 17, 22 sclerosis, 242 Postero-lateral sclerosis, 259 Potts' disease, 307 Preacher's hand, 288, 332 Precipitin reaction, 181, 183, 195, 198 Prefrontal lobe, 81 Primary lateral sclerosis, 256 Progressive muscular atrophy, 314 Projection fibers, 31 Propulsion. 60 Protopathic, 70 Pseudo-bulbar palsy, 229 -hypertrophy, 62, 322 -meningitis, 181, 466 -paresis, 363 -ptosis, 467 -tetanus, 522 -tetany, 522 Psychic blindness, 133 Psychanalysis, 462 Psychasthenia, 456 Psychoneuroses, 456 Psychotherapeutics, 461, 480 Ptosis, 246, 401, 402 Puerperal neuritis, 392 Pulvinar, 24 Pupils, 368 Purkinje's cells, 49 Putamen. 30 Pyramidal bundle, 8 crossed tract, 8 tracts, 8, 19, 35 Pyramids, 8, 16 Quadrigeminal bodies, 20, 152, 169 Quincke, 182, 584 Rabies, 605 Radiculalgia, 429 Radicular anaesthesia, 69, 287, 332 sciatica, 429 Radiculitis, 428 Railroad spine, 302 Raynaud's disease, 587 Reactions of degeneration, 66, 267, 316, 332, 4i7 Recklinghausen's disease, 384 Recurrent larnygeal nerve, 413 Red nucleus, 21, 23 Reflexes, 71 abduction, 93 Achilles', 71, 247 adduction, 93 Reflexes, anal, 73 ankle-clonus, 71, 90 anticus, 94 Babinski's, 73, 91, 94 biceps, 72 Chadwick's, 91 Claude's, 94 contralateral, 71 cremasteric, 73 Gordon's, 73, 91, 93 Grasset's, 93 hand, 93 interossei, 92 Marie's, 94 masseter, 71 neck, 177 Oppenheim's, 73, 91 patellar, 72, 247 plantar, 73 Schafer's, 91 thumb, 93 triceps, 71 wrist, 93 Reflex neuralgia, 437 Regeneration, 380 Remissions, 366 Restiform bodies, 12, 14, 19 Retropulsion, 60, 531 Rhachischisis, 58 Rhombencephalon, 11 Risus sardonicus, 602 Rolando, 6 Romberg's sign, 59, 244 Roots of spinal nerves, 9, 243 Rossolimo, 329 Sacro-coxalgia, 441 Saddle-shaped anaesthesia, 292 Salvarsan, 253, 350 Sarcoma, 143 Schlesinger's sign, 521 Schwab's and Allison's operation, 122 Sciatic nerve, 426 neuralgia, 439 Scleroderma, 579 Sclerosis of brain, 114 combined, 259 disseminated, 259 insular, 295 lateral, 256 multiple, 295 posterior, 242 postero-lateral, 259 Sclerosis, 263 Self-suggestion, 469 Senile neuritis, 394 Saltatory chorea, 468 Sensory aphasia, 132 dissociation, 69, 287 neurones, 8 phenomena, 68 Septum, 3 lucidum, 40 Serotherapy, 194 Serous spinal meningitis, ^^$ Sexual neurasthenia, 453 INDEX 617 Sexual trauma, 464 Shaking palsy, 529 Shoemaker's cramp, 528 Shultze's comma, 8 Sicard, 254, 476 Sight, 70 Sinus phlebitis, in cavernous, 200 longitudinal, 200 transverse, 200 Smell, 70, 151 Softening of brain, 86, 97 diagnosis, 96 etiology, 87 localization, 98 pathology, 86 prognosis, 96 symptoms, 89 treatment, 99 Softening of cerebellum, 221 medulla, 234 pons, 238 Somnambulism, 470 Spasm, facial, 514 nutans, 511 Spastic paraplegia, 256 spinal paralysis, 346 Spatapoplexie, 101 Speech scanning, 297 spasmodic, 357 Spermatic neuralgia, 447 Sphincters, 74, 274 Spina bifida, 55 Spinal accessory nerve, 414 anaesthesia, 443 apoplexy, 280 arteries, n cord, 1, 242 meningitis, 269, 329, 333 nerves, 9, 415 paralysis (infantile), 264 paralysis (spastic), 346 veins, n Status epilepticus, 484 Stelwag's sign, 560 Steppage gait, 386, 391, 427 Stereognosis, 69, 79 Stigmata, 465 Stoffel's operation, 123 Strabismus, 400 Striae acusticse, 15 Strumpell, 104 Strychnin poisoning, 603 Stuttering, 469 St. Vitus dance, 495 Subarachnoid space, 45 Subdural cavity, 10 Suggestibility, 468 Suggestion, 461, 469, 478, 480 Sulcus, 3 median, 13 Supra-scapular nerve, 419 Sydenham's chorea, 495 Sylvius aqueduct, 21 Sympathetic nervous system, 560 Syphilis, 117, 336, 365, 372 Syphilis of brain, 337 cerebro-spinal, 347, 364 congenital, 365 hereditary, 349 of peripheral nerves, 348 of spinal cord, 347 Syphilitic arteritis, 340 hemiplegia, 341 meningo-encephalitis, 250 mental disturbances, 348 Syphiloma, 144 Syringomyelia, 286 Systematic exercises, 255 Tabes dorsalis, 242 juvenile, 256 Tabetic foot, 248 Taches cerebrales, 176 Taenia semicircularis, 24 Taste, 71, 151 Tegmentum, 19, 21 Tela chorioidea, 15, 38, 46 Telegraphist's cramp, 528 Telencephalon, 26 Temporal lobe, 80 Tender spots, 431, 435, 437, 439 Tenotomy, 271 Tentorium cerebelli, 44 Testicular neuralgia, 444 Tetanus, 602 cephalic, 605 neonatorum, 603 Tetany, 519 gastric, 523 Thalamus opticus, 22, 24, 167 Third ventricle, 25 Thomsen's disease, 525 Thoracic nerve, 418 segment, 1 Thrombosis, 86, 88, 199, 237, 342 Tic, 61, 507 convulsif, 510 douloureux, 436 of face, 508 of head, 509 of neck, 508 laryngeal, 510 nystagmoid, 508 palpebral, 508 psychic, 511 Salaam, 511 Tinnitus aurium, 411 Titubation, 60, 213, 2g8 Torticollis, mental, 509 spasmodic, 509 Tracts, 7 antero-lateral, 9 direct pyramidal, 8 Flechsig's, 8 Gower's, 8 Loewenthal's, 9 Transcortical aphasia, 134 Traumatic lesions of cord, 302 neuroses, 548 psychoses, 555 Tremor, 61 6i8 INDEX Tremor, intention, 262, 297 passive, 530 Trigeminal nerve, 405 Trigonum, acusticum, 15 habenulae, 15 hypoglossi, 15 Trismus, 602 Trophoneuroses, 560 Trousseau's sign, 521 Tuberculoma, 144 Tumors of brain, 143 course, 155 diagnosis, 156 etiology, 158 pathogenesis, 154 pathology, 143 symptoms, 146 treatment, 158 of cerebellum, 213 cord, 306 pons, 238 Turck's bundle, 8 Ulnar nerve, 422 Uncinate fits, 151 Unverricht's myoclonia, 518 Velum, anterior medullary, 15 posterior medullary, 15 Ventricles, fifth, 40 fourth, 15 lateral, 38 Ventricles, third, 25 Verbal amnesia, 135 Vertebral cancer, 311 Vertigo, 109, 147, 263, 411, 544 Vestibular nerve, 411 Violinist's cramp, 528 Visual apparatus, 40 disturbances, 467 Vomiting, 147 Von Graefe's sign, 560 Wasserman's test, 117, 126,351,356,358,365, 373, 374 Weber's syndrome, 239 Wechselbaum's meningococcus, 372 Weir-Mitchell, 585 Wernicke's aphasia, 78, 130, 133 area, 78, 137 polio-encephalitis, superior, 104 pupil, 141 Westphal's sign, 247 Wet brain, 597 White substance of brain, 31 of spinal cord, 6 Word-blindness, 78, 133, 150 -deafness, 78, 132, 151 Wrist-drop, 386, 421 Writer's cramp, 527 Zona, 447 Zoster, herpes, 436, 447 COLUMBIA UNIVERSITY LIBRARIES This book is due on the 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