RC34 D 19 College of ^fipsiicians! anb burgeons Htbrarp G-lj^f Op J3r. M. A- StARR Digitized by the Internet Archive in 2010 with funding from Open Knowledge Commons http://www.archive.org/details/textbookofnervou1915dana PLATE I NERVOUS DISEASES DANA The Fiest Formal Union of Neuro- anatomy and Art. {Ste-phmius, 1545.) " The avulsed calvarium hangs from a tree, showing the dura mater." TEXT-BOOK OF NERVOUS DISEASES FOR THE USE OF STUDENTS AND PRACTITIONERS OF MEDICINE BY CHARLES L. DANA, A. M., M. D., LL. D. PROFESSOR or NERVOUS DISEASES IN CORNELL UNIVERSITY MEDICAL COLLEGE; CONSULTING PHYSICIAN TO BELLEVUE HOSPITAL; NEUROLOGIST TO THE MONTEPIORE HOSPITAL; NEUROLOGIST TO THE woman's hospital; consulting PHYSICIAN TO THE MANHATTAN STATE hospital; EX-PRESIDENT OP THE AMERICAN NEUROLOGICAL ASSOCIATION; EX-PRESIDENT OP THE NEW YORK ACADEMY OP MEDICINE; CORRE- SPONDING MEMBER or THE SOCIETE DE NEUROLOGIE, ETC. EIGHTH EDITION WITH TWO HUNDRED AND SIXTY-TWO ILLUSTRATIONS, INCLUDING FOUR PLATES IN BLACK AND COLOR \ NEW YORK WILLIAM WOOD AND COMPANY MDCCCCXV Copyright, 1915, by WILLIAM WOOD AND COMPANY XHE MAPIiE PKESS YOKK FA PREFACE TO THE EIGHTH EDITION When the first edition of my book was published twenty-three years ago there was no adequate description of the anatomy of the nervous system in the English language. Hence, feeling the fundamental im- portance of this subject to an understanding of neurology, I gave much space to it. This need of anatomical knowledge of the nervous system is now abundantly supplied by general and special text-books. I have, therefore, left out a good deal of this part of my text. I have retained and revised what was especially useful in diagnosis and for reference, and have also changed, added to and improved the illustrations. In this I have received help and skilled supervision from Dr. J. F. Guder- natsch, of the Anatomical Department of Cornell University. A large part of the rest of the book has been completely remodeled and rewritten. This applies to the chapters on syphilis of the nervous system, including paresis, tabes and the serology of nervous diseases. Dr. David Kaplan, of the New York Neurological Institute, has furnished me with data on this last subject which represent large experience and bring the matter to date. The chapter on acute anterior poliomyelitis has been rewritten by Dr. Foster Kennedy in the light of recent discoveries, and the same has been done for the chapter on epidemic cerebrospinal meningitis by Dr. H. W. Frink. I am under obligations to both these gentlemen for their excellent work. Practically new chapters have been written on tumors of the spinal cord and tumors of the brain. Dr. Charles A. Elsberg has allowed me to use the statistics of his cases, so that I am able to present these topics from the standpoint of modern neurological surgery as well as of medicine. The chapters on methods of examination and diagnosis and on general symptoms have been thoroughly revised and an endeavor made to include the latest data. The number of epinomic signs and syndromes of neurol- ogy has grown to a burdensome length. A descriptive list of them has been prepared by Dr. Frink. The chapters on the psycho-neuroses have been rearranged and in parts rewritten and condensed. Neurasthenia has been pushed, as modern views demand, much more into the background. Hysteria and psychasthenia have been treated from the standpoint of descriptive rather than of " dynamic" or analytic neurology. This it seemed to me was the only way to make the subject intelligible in a work meant largely for iii IV PREFACE TO THE EIGHTH EDITION students and practitioners. I trust that I have, however, given a measure of justice to psycho-analysis, as it certainly has a place in the interpreta- tion and occasionally in the therapeutics of the minor psychoses. The subject of the disturbances of growth and metabolism and of nervous function due to glandular disorders has brought out a large literature in recent years, and this has been considered in the revision of this edition. The subject of therapeutics is difficult to discuss effectively in a trea- tise that aims to be of moderate size. Methods and drugs and points of view change so rapidly that I have decided to leave out the special chapter on this subject. Treatises on electricity, massage, exercise, diet, mechan- ical therapy, hydrotherapy, etc., are now many and accessible. Many new clinical and anatomical illustrations have been added. I am greatly indebted to Dr. J. B. Gere, of the Pathological Department of Cornell University Medical College, for beautiful photographs of spinal-cord and of nerve disease; to Dr. S. Wachsmann and the Montefiore Home for photographs of clinical cases; and to Dr. R. S. MacRobert, of New York Neurological Institute, for photographs illustrating methods of examination. A modest tribute has been paid to the fathers of neuro- anatomy in the reproduction and use of illustrations of the brain by Stephanus, Willis, and Ruysch. They represent anatomical illustration and art in the 16th, 17th and 18th centuries respectively. The part of my work devoted to psychiatry will appear in a separate volume. However, I have included in the present treatise articles on the minor psychoses, as well as a chapter on paresis. A treatise on neurology must usually be read, and studied in parts, hence that portion of the original preface has been retained which sug- gested to the reader a certain eclecticism of method in neurological study. The principle then set forth holds good now though the lists then given need revision. My thanks are due to my publishers for their helpfulness and cheerful co-operation. New York City, Sept., 15, 1915. FROM PREFACE TO THE FIRST EDITION As a special text-book the present work will be used by two classes of readers, one consisting of those who simply consult it for reference in connection with their cases, the other composed of students who desire to ground themselves systematically in a knowledge of neurology. To this latter class I venture some advice as to the method they should pur- sue. Neurology is a difficult branch of medicine to master, nor is there any royal road to it. Still, it can be made comparatively easy if its study is undertaken in a proper and systematic way. In using the present work, the student should first refresh his general knowledge of nervous anatomy as furnished in ordinary text-books. He should then go carefully over the anatomical descriptions here given of the general structure of the nervous system and of that of the nerves, spinal cord, and brain. A thorough knowledge of anatomy and physiol- ogy makes clinical neurology comparatively easy, and in fact reduces much of it simply to a matter of logical deduction. The student should next master the general facts of nervous path- ology, symptomatology, and etiology, for he will find common laws underlying apparently the most varying phenomena. Finally, he must begin to study the special diseases. The number of these is very great; in the present work I have described 176. Many of these are rare, and it would be wrong for the student to burden his memory with the details about them. He need know only of their existence and general physiog- nomy. There are, however, according to my enumeration, about 65 nervous diseases which are either very common or extremely important, and it is these that the student should master and make part of his work- ing knowledge. Since the distribution and names of the common and rare diseases may be a useful guide, I append here a table and a list : Peripheral Spinal Cord Brain Functional Totals Common and important dis- eases Pi,are 31 56 13 27 12 16 10 11 66 110 87 40 28 21 176 The common or important nervous diseases are: General. — Neuritis, multiple neuritis, degeneration, neuralgia, par- sesthesia (5). VI PREFACE Cranial Nerves. — Anosmia, optic neuritis, optic atrophy, ptosis, ophthalmoplegia, abducens palsy, headache, migraine, trigeminal neural- gia, facial spasm, facial palsy, tinnitus, vertigo, ageusia, wryneck (16). Spinal Nerves. — Cervical neuralgia, hiccough, brachial palsies, single and combined, brachial neuralgia, intercostal neuralgia, herpes zoster, lumbar neuralgia, sciatica, leg palsies (10). Spinal Cord. — Spina bifida, hemorrhage, pachymeningitis, lepto- meningitis, poliomyelitis, transverse myelitis, acute and chronic, second- ary degenerations, locomotor ataxia, the progressive muscular atrophies, bulbar palsy, muscular dystrophies, spinal irritation (13). , Brain. — Malformations, hypersemia, pachymeningitis, leptomenin- gitis, simple, tuberculous, and epidemic, abscess, hemorrhage, embolism, thrombosis, children's palsies, syphilis (12). Functional.- — -Epilepsy, hysteria, the tics, chorea, tetanus, neuras- thenia, spermatorrhoea, exophthalmic goitre, occupation neuroses, pa- ralysis agitans (10). New York City, 1892. TABLE OF CONTENTS CHAPTER I Page General Anatomy, Physiology, and Chemistry. The General Histology of the Nervous System — The Neuronic Architecture of the Nervous SJ^stem . . 1 CHAPTER II The Causes of Nervous Diseases 23 CHAPTER III General Pathology 28 CHAPTER IV General Symptoms 30 CHAPTER V Diagnosis and Methods of Examination. Examination of the Reflexes — Examination of the Disorders of Sensation — Cerebrospinal Fluid — Spinal Puncture 39 CHAPTER VI Hygiene, Prophylaxis, Treatment. Diet — Exercise — Hj^drotherapy — Massage — Osteopathy — Climate — Electricity — Radiant Energy — Psycho- therapy 60 CHAPTER VII Diseases of the Peripheral Nerves. General Pathology — Hypersemia and Anaemia — Angina — Neuritis — Degeneration — General Symptoms — Multiple Neuritis — Sensory-motor Type — Sensori-ataxic Type — Endemic and Epi- demic Types — Malarial — Acute Pernicious — Complicating Forms of Neuritis and Neuritic Degeneration — Tumors 75 CHAPTER VIII Motor Disorders of Cranial Nerves. The Ocular Muscles — General Symp- toms — The Ophthalmoplegias — Third Nerve — Fourth Nerve — Sixth Nerve — Muscular Asthenopia and Muscular Insufficiencies — Spasmodic Diseases vii VIU CONTENTS Page of the Ocular Muscles — The Motor Branch of the Fifth Cranial Nerve — The Facial Nerve — Facial Spasm — Facial Palsies — The Glossopharyngeal Nerve — The Pneumogastric Nerve and the Accessory part of the Spinal Accessory — Vagotonia — Laryngeal Nerve Supply — Spinal Part of the Accessorius and Upper Cervical Nerves — Torticollis — Paralysis of the Spinal Accessory — The Hypoglossus — The Mechanism of Articulation 95 CHAPTER IX Neuroses of the Motor Spinal Nerves. The Upper Cervical — The Lower Cervical and Brachial Plexus — The Thoracic or Dorsal — The Lumbar —The Sacral 126 CHAPTER X Sensory Neuroses of the Cerebrospinal Nerves. Parsesthesia — Neuralgia — Neuroses of the Nerves of Special Sense — The Olfactory Nerve — The Optic Nerve — Trigeminal Nerve — Headache — Migraine — Seventh Nerve — Acous- tic Nerve — Nervous Deafness — -Tinnitus Aurium — Vertigo — Glossopharyn- geal Nerve — Upper Cervical Nerves — ^Lower Cervical Nerves and Brachial Plexus — Intercostal Nerves — Lumbar Nerves — Peripheral Vasomotor and Trophic Neuroses 145 CHAPTER XI Diseases of the Spinal Cord. Anatomy and Physiology — Diagnostic Physi- ology 200 CHAPTER XII Diseases of the Spinal Cord. Malformations — Spina Bifida — Spinal Hemor- rhage — The Caisson Disease — Spinal Meningitis — Myelitis and Myelo- malacia — Poliomyelitis — Acute Ascending Paralysis 214 CHAPTER XIII Scleroses, Degenerations, Syphilis. Locomotor Ataxia — Lateral Sclerosis — The Combined Scleroses — Hereditary Spinal Ataxia — Hereditary Cere- bellar Ataxia 248 CHAPTER XIV The Progressts^e Muscular Atrophies and Muscular Dystrophies. Luetic Spinal Atrophy — Hereditary Muscular Atrophy of the Peroneal Tj'pe — Glosso-labio-larjrngeal Paralysis — Myasthenia Gravis — Amyotrophic Lateral Sclerosis — The Progressive Muscular Dystrophies — Pseudo- muscular Hypertrophj^ — Hemihypertrophy — Summary of the Hereditary or Family Nervous Diseases — Arthritic Muscular Atrophy — Occupation Muscular Atrophies 292 CONTENTS IX CHAPTER XV Page Tumors and Cavities of the Spinal Cord. Hydromyelia — Gliosis and Syringomyelia — The Recognition of Diseases of the Cauda Equina .... 318 CHAPTER XVI Anatomy and Physiology of the Brain. The Cerebellum — The Glands of the Brain — The Connecting Tracts — The Membranes — The Functions .... 333 CHAPTER XVII Diseases of the Brain and Its Membranes. General Symptoms — Malforma- tions — Purulent Cerebrospinal Meningitis — Epidemic Cerebrospinal Menin- gitis — Tuberculous Meningitis — Hydrocephalus — Alcoholic Meningitis — Hypersemia and Anaemia 367 CHAPTER XVIII Diseases of the Brain. Inflammations— General Paresis — Multiple Sclerosis — The Apoplexies — Softening — Sinus-thrombosis — Cerebral Palsies of Children 395 CHAPTER XIX Tumors of the Brain. Intracranial Aneurism 454 CHAPTER XX Functional and Degeneratr^e Diseases. Epilepsy 474 CHAPTER XXI The Psycho-neuroses. Constitutional Inferiority — Hysteria 488 CHAPTER XXII Psychasthenia 507 CHAPTER XXIII Neurasthenia. The Sexual Neuroses and Psychoses — Traumatic Nervous Affections 514 CHAPTER XXIV The Myoclonias or Twitching Spasms. Chorea of Sydenham — Hereditary Chorea — Spasmodic Tic — Fibrillary Myoclonia — -Myotonia — Tortipelvis — Tetanus — Tetany — Rabies and Hydrophobia 535 X CONTENTS CHAPTER XXV Page Exophthalmic Goitre and Hyperthyroidism v. 553 CHAPTER XXVI Professional Neuroses, Occupation Neuroses 560 CHAPTER XXVII Paralysis Agitans 667 CHAPTER XXVIII Trophic and Vasomotor Disorders. Hemiatrophy — Diseases of the Pituitary Gland — Acromegaly — Myxoedema — Cretinism — Angio-neurotic CEdema . . 576 CHAPTER XXIX The Disorders op Sleep. Insomnia — Hypnotism — Morbid Somnolence — Catalepsy — Trance — Lethargy — Perversions and Disturbances — Mesmer- ism — The Sleeping Sickness of Africa 586 CHAPTER XXX Cranio-cerebral Topography 600 APPENDIX The Function and Innervation of the Muscles. Muscles of Tongue, Palate and Pharynx — Muscles of Head and Neck — Muscles of Shoulder and Upper Extremity — Muscles of Arm, Forearm and Hand — Muscles of Back and Lower Extremities 605 PAET I DISEASES OF THE NERVOUS SYSTEM CHAPTER I GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY In studying the phenomena of hfe in tlie human body, we as physicians first learn about its normal structure and functions. We then note the new phenomena which develop when disease comes on, the causes which produce them, and the anatomical changes lying back of them; we group our facts and give the disease a name. Lastly we apply the methods by which the disorder can be expelled and future attacks prevented. In fine, we investigate our subject just as we do that of any branch of natural history. Our study divides itself, therefore, into Normal anatomy and physiology. Etiology, a study of the causes. Symptomatology, a study of the morbid phenomena. Pathology, under which we include a study of the morbid anatomy and physiology. Diagnosis, or the method of recognizing and separating out the dif- ferent groups of diseases. Prognosis, a forecast of the future course of the malady. Treatment and prophylaxis. GENERAL ANATOMY The nervous system is derived from the ectodermal germ layer of the developing ovum, and its constituents are modifications of epithelial cells. These cells in the embryo are of two kinds: neuroblasts, which develop into nerve-cells and fibres; and spongioblasts, which develop into a supporting structure called neuroglia (His). The nervous system is composed of: (a) Neurons, which form the nervous tissue proper, and are made up of nerve-cells, with their processes, one of which becomes an axis cylinder; and neuroglia. (6) Accessory tissue, consisting of connective tissue, blood-vessels, lymphatics, and epithelium. DISEASES OF THE NERVOUS SYSTEM These tissues are united together to form the central nervous system, consisting of the brain and spinal cord, and the peripheral nervous system. This latter is composed of nerve-fibres, and structures attached to the terminations of the nerves, called end-organs, and finally the ganglionic or sympathetic nervous system is included in the peripheral system. The Arrangement of the Nervous System. — The subdivisions of these parts, and their descriptions in detail, belong to general anatomy. But there have been so many special subdivisions, and particular names given to them in recent years, that I deem it necessary, in order to pre- vent confusion, to describe briefly the subdivision accepted by modern anatomists. The names here used are those adopted by the committee on anatomical nomenclature of the German Anatomical Society, and they have also been adopted by a large number of writers on neuro-anatomy. vAjO^/j, ''""MAL/c OPTIC VESICLE Fig. 1. -Showing divisions of embryonal human brain in third and fifth weeks. (His.) (Cunningham.) Beginning with the brain, we find that its particular subdivisions are based upon the embryological development of this organ. As will be shown in more detail later, the brain is developed out of three vesicles, known as the anterior, middle, and posterior vesicles (Fig. 1). The most anterior of these vesicles is the prosencephalon or anterior brain; the middle vesicle becomes the mesencephalon or mid-brain, and the posterior vesi- cle develops into the rhombencephalon or posterior brain. The anterior vesicle develops two secondary vesicles : the anterior por- tion of these, including the corpora striata, olfactory lobes and the cere- bral hemispheres, forms the telencephalon (Fig. 2, 1 2), while the hinder por- tion of this vesicle, which includes the thalamus and mammary bodies, forms the diencephalon (I^). The middle vesicle is the mesencephalon, and it includes the corpora quadrigemina and cerebral peduncles (II). GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY 3 The posterior vesicle is divided, from before backward, into three different parts: (1) the isthmus, which inchides the superior cerebellar peduncles and valve of Vieussens, and part of the cerebral peduncles; (2) the metencephalon or hind-brain, which includes the cerebrum and pons Varolii; and (3) the myelencephalon or after-brain, which includes the medulla oblongata. Prosencephalon (anterior brain). II and 1= Brain. ^5 in.) in diameter and are almost visible to the naked eye. The smallest are 0.7m (K500 in.) in diameter; so that the average diameter is rather greater than that of a white blood-cell. In shape nerve-cells are for the most part irregularly spheroidal, but some are pyramidal, others spindle- or flask-shaped, and others globular. They all give off one or more fine processes or poles and hence, in accordance with the number of these, the nerve-cells are often spoken of as multipolar, bipolar, or unipolar. In most cells one of the processes is continued on a long way and finally becomes a nerve-fibre. This process is called the axis-cylinder, or GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY 5 neuraxon or axon (Figs. 3, 4 and 5 and Plate II). The other processes are relatively short and are called protoplasmic processes, or dendrites. The nerve-cell, then, is a protoplasmic body giving off several dendrites and usually a single neuraxon, the whole forming the neuron. The dendrites branch off irregularly and subdivide, but never anastomose. In some parts of the nervous system they have upon them little nodules or buds, and in the cerebral and cerebellar cortex these are so numerous as to give Fig. 4. Fig. 5. Fig. 4. — -Nerve cell showing dendrites, axon, collaterals, a, Axis-cjdinder-bifur- cating at h; c, a collateral; d, varicosities of the dendrites. (Cajal.) Fig. 5. — Nerve cells from anterior horn of spinal cord. (Cunninghmn.) them the appearance of budded stalks. The dendrites are usually not very long, but in some cells they extend a very great way, reaching many times the diameter of the cell. The axis-cylinder process, or neuraxon, is given off directly from the body of the cell, as a rule (Fig. 5). It very soon becomes clothed with a thin sheath (myelin sheath) , and as it passes along gives off branches at right angles, which form what are known as the collaterals. The neuraxon and collaterals finally end by splitting up into a 6 DISEASES OF THE NERVOUS SYSTEM number of fine branches, which lose their myelin sheath and form the end-brush or terminal arborization. In some neurons instead of an end- brush, there are several minute ovoid bodies which lie upon the cell or its dendrites. They are called "end-buds" or terminal "buttons." They are seen especially in the medulla and pons, but not in the cerebral or cerebellar cortex. The axis-cylinder process or neuraxon does not anas- tomose with other cells either through its own end-brush or through the end-brushes of its collaterals. The end-brushes, however, pass in among the dendrites of other cells, and sometimes closely surround the cell-body. In this way one neuron comes into very intimate relation with others, but there is never any true union. Each neuron of the nervous system is an independent unit. Fig. 6. — Ganglionic nerve cells, ultra-microscopical illumination. (Marinesco.) The cell-body proper is composed of a cytoplasm. Within this lies the nucleus and within the nucleus a nucleolus. The body proper is not homogeneous, but is made up of a network of fine fibres or fibrillse which pass in bundles from dendrite to dendrite and from dendrite to the neuraxon, called the endocellular fibrillar network. Within its meshes and arranged in a rather definite manner are certain stainable bodies called Nissl or chromophilic granules (Plate II). The chromo- philic granules are arranged differently in cells of different function. They are believed to represent the functioning substance of the cell, while the fibrillse form a conducting part. The cell-body usually con- tains a little pigment. NERVOUS DISEASES Dana PLATE II B Nerve-cells Stained by Nissl's Method, with Toluidin Blue. Magnified 675 diameters. {Schaefer.) A, From anterior horn of spinal cord, monkey; B and C, from facial nucleus, dog; D, from reticular formation of pons Varolii, dog; C, shows Nissl degeneration, consequent on section of the facial nerve 15 days previously; a, a, axons. GENEKAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY 7 The nucleus of the cell is a comparatively large spherical body, which is also made up of a reticulated structure known as the chromatin network. The chromatin consists of granules resting on a network formed by the achromatic substance (hnin). The chromatin is practically identical with nuclein. Within the nucleus is a smaller body, known as the nucleolus, which stains still more intensely. Nerve-cells are surrounded by a pericellular space, but are not inclosed in capsules, excepting those of the posterior spinal and ver- tebral ganglia. The reason for this is that within the central nervous system there is no neurilemma (nucleated sheath of Schwann) ; such sheath being found in the peripheral system only. Marinesco has shown by studying the anatomy of the fresh nerve- cell by means of the ultra microscope some new details of the structure Fig. 7. — From a transverse section through the sciatic nerve, ep, Epineurium; p, perineurium; n, nerve fibres constituting a nerve bundle of fasciculus in cross- section;/, fat tissue surrounding the nerve. (Klein.) of the cell. The body of the cell is composed of a complex of colloidal substances whose particles are illuminated by the lateral light of the ultra microscope. Fig. 6 shows four cells taken from the ganglion of a dog, examined in the serum of the same animal. The nerve-cells of the sympathetic or vertebral ganglia are very like the central nerve-cells in the anterior horns. They are multipolar in shape and have dendrites and a neuraxon. The neuraxon goes to other neighboring cells, or it passes on to the spinal cord or the per- iphery. The cell is surrounded by a connective-tissue capsule lined with a flat epithelium, like those of the posterior spinal ganglia. The peripheral or terminal sympathetic nerve-cells lying in the viscera resemble those of the vertebral ganglia. Nerve-cells are classified in accordance with their shape and num- ber of processes. The multipolar cell is the common type and is found 8 DISEASES OF THE NERVOUS SYSTEM throughout the bram, cord, and sympathetic gangha. Bipolar cells are found chiefly in the column of Clark of the spinal cord and in spinal ganglia. Small nuclear cells and flask-shaped or Purkinje's cells are found in the cerebellum. Besides these there are described in the brain cortex angular, granular, pyramidal, globose, and spindle- cells. The nerve- fibres of the nerve-centres are found chiefly in the white tissue or white matter. In the periphery they form the nerve proper of gross anatomy. The peripheral nerve is composed of bundles of nerve-fibres called nerve fasciculi (Fig. 7). It is surrounded by a con- nective-tissue sheath called the sheath of Henle, or epineurium. From this sheath, connective- tissue fibres pass in and surround the fasciculi. The sheath of the fasciculus is called the perineurium. From the perineurium, strands of connective tissue run in among the ultimate nerve-fibres, forming the endoneurium (Figs. 8 and 9) . Lymphatic spaces lined with endothe- lium exist in the layers of the peri- and endo- FiG. 8.^A simple funic- sheaths. In the nerve-centres, the nerve- The "SpplreLf ^JmS'l^SS ^^I'^s, have no such sheaths, but are supported ated cells are sections of the by a connective tissue and neuroglia framework. ?Xnd*"%: Axi^'^iJiS; The nerve-fibre is a long fine strand of tie- 10, white substance of sue varying in diameter. It may be white or litZTn, neunlltaTer^el;: 8™y.. '^'^'^o-'ding to whether it has or has not a doneurium; p, perineurium; myelin sheath. It is composed from within out tiesZT7fl^Z)""^^' °^ Of (1) an axis-cyhnder, (2) a myelin sheath, and (3) a neurilemma. (1) The axis-cylinder is the essential part of the nerve. It is the prolongation of the neu- raxon of a nerve-cell and consists of protoplasm. It is itself made up of fine fibrillse (primitive fibrillse) which run longitudinally. The myehn sheath is developed, hke the axis-cylinder, from the ectoderm, and is closely related nutritionally to the axis-cylinder, which it protects and isolates. (3) The neurilemma or nucleated sheath is a dehcate covering forming the outermost sheath of the nerve. It is also of ectodermic origin. The sheath is absent in the fibres of the central nervous system and in some fibres of the periphery. Variations in the Types of Fibres.- — In accordance with the arrange- ment of the sheaths of the nerve-fibres, several kinds are described. The principal types are the medullated and non-medullated. Medullated nerve-fibres make up the bulk of the white matter of the brain and cord and cerebrospinal nerves. They consist of a mj^elin sheath and axis-cylinder, and may or may not have a neurilemma. GENERAL ANATOMY, PHYSIOLOGY, AND CHEMLSTRY 9 Fibres with myelin sheath, but without a neurilemma, make up the white matter of the central nervous system. Non-medullated fibres, or fibres of Remak, occur principally in the sympathetic system, but they are also found in the cerebrospinal nerves. They are grayish and faintly striated, and consist of axis-cylinders, with a thin, homogeneous, nucleated sheath, the neurilemma, lying directly upon them. Naked axis-cylinders are found in the peripheral terminations of nerves as well as in the brain and cord and sympathetic. Size. — The nerve fibres are of two kinds as regards size. The small fibres are about 2ijl or M2000 inch in diameter, the large 20 fx or M200 inch. The small fibres are connected with smaller cells, and either run a shorter course or are distributed to the involuntary muscular X , ■::% Fig. 9. — iSeetion of nerve. {Quain.) fibres of the blood-vessels and viscera. The motor fibres are larger than the sensory. The white fibres of the sympathetic nervous system are about one- third smaller than the ordinary cerebrospinal or somatic fibres. The peripheral nerve-fibres, except the optic, have no neuroglia; they terminate in fine fibrillae among epithelial cells, or in special end- organs. The central nervous fibres make up the white matter of the brain and cord. They are, like the peripheral nerves, the prolongations of the 10 DISEASES OP THE NEEVOUS SYSTEM neuraxons. They are composed of an axis-cylinder process and myelin sheath, but have no neurilemma, and probably no nodes. At fre- quent intervals each fibre gives off branches at right angles forming the "collaterals." Connections of Nerve-cells and Nerve-fibres. — One nerve-cell is never connected directly with another, so far as anatomical investiga- tion can show. One nerve process becomes an axis-cylinder, receives a myelin sheath, gives off collaterals, and finally breaks up into a fibrillary "end-brush" surrounding a cell, but not passing into it. There is physiological, but no apparent anatomical continuity. Fig. 10. Fig. 11. Fig. 12. Fig. 10. — MeduUated nerve fibre, a, Axis cylinder; n, nucleus; m, medullary sheath; c, node of Ranvier. Fig. 11. — MeduUated nerve fibre, showing mode of division. Fig. 12. — -Non-meduIIated nerve fibre, n, Nucleus; 6, striations. The Neuroglia. — The supporting tissue of the peripheral nerves is connective tissue only; that of the central nervous system is con- nective tissue and, in addition, a peculiar substance called neuroglia. The neuroglia or supporting tissue of the nervous centres is derived from the ectoderm. It is composed of cells with very numerous and finely ramified processes, which make a supporting network about the nerve-cells and fibres (Fig. 13). The cell-body is composed of granular protoplasm, lying in which is a large nucleus, within which is the nucleolus. The body of the cell is small in amount in proportion to the nucleus. The fibrillary processes form a felt-like network, and GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY 11 in regions where there is much neurogHa tissue this looks like a homo- geneous matrix. It is, however, made up of the fine fibrils. The Non-nervous Tissue — The Blood-vessels. — The peripheral nerves are richly supplied with blood. Each nerve receives arterial supply Fig. 13. — Phases in the development of neuroglia cells and fibrils. lA, Divid- ing neuroglia nuclei surrounded by protoplasm; 2B, protoplasmic processes more definitely formed; 3C, commencing condensation of protoplasmic processes producing darkly staining fibril; 4Z) and 5E, mode of attachment of the processes to a vessel wall and differentiation of foot into fibrils; QF, further development of fibrils; 7G, protoplasm almost entirely differentiated into fibrils and nucleus shrunken. (From figures drawn by Dr. George A. Watson.) from many different branches, but always from the same general source. The artery passes to the nerve-sheath obliquely, then divides dichoto- mously and sends branches a long distance up and down on the sheath. It may pierce the sheath, however, first, and then divide, as above de- 12 DISEASES OF THE NEEVOUS SYSTEM scribed. The dichotomous branches send off arterioles and capillaries, which form plexuses about the nerve fascicles. These are "the inter- fascicular arcades." The arteries subdivide in such a way as to pre- vent sudden impact of a large bloocl-stream into the tissue of the nerve. In this respect the nerve circulation resembles that of the brain and cord. The veins subdivide dichotomously, like the arteries. They freely anastomose with the muscular veins, so that muscular action helps nerve circulation. The veins of the superficial nerves connect with those of the deep nerves. The blood-vessels of the spinal cord and brain will be described later. Fig. 14. — Glia cells as impregnated in silver-chromate preparations. (After Ramon Cajal.) Lymphatic vessels and spaces are found in the epineurium and perineurium. There are no distinct lymphatics in the fasciculi, but lymph-spaces probably exist. THE NEURONIC ARCHITECTURE OF THE NERVOUS SYSTEM Having described the component parts, I shall now show the way in which these parts are arranged to form the nervous system. The nervous system, as already shown, is composed of single nerve units that are called neurons, and the neuron is made up of a cell-body and numerous processes, one of which is the neuraxon, the others the dendrites. GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY 13 The neuraxon has always the function of carrying impulses away from the cell; it is a cellufugal fibre; the dendrites, however, bring impulses to the cell, and are cellupetal in function. The dendrites are in contact with end-brushes or end-buds of the neuraxons of other cells, and in this way receive the nerve impulse and transmit it to the cell-body. The nervous system is thus a mass of neurons which are packed closely together, and form with each other most intricate rela- tions, but never connect directly one with another. No nerve-fibre or dendrite of one cell anastomoses with that of another, as blood-vessels do. Each neuron is anatomically independent. It is the purpose of this neuronic mass to receive impulses from within or without the body, to transfer and modify them, and to send out impulses in such way as properly to control the vital functions and keep the individual in proper harmony with his environment. The nervous system is a great receiving, regulating, controlling, and discharging machine, the machinery being the neurons, the force that works in it being called nervous energy. The neurons as a whole have this force stimulated or aroused in them. The nerve-cell co-ordinates and distributes it. The nerve-cells are massed together for the most part in the brain and spinal cord, forming the gray matter, while the neuraxons as distributors make up the white matter and the cranial, spinal, and sympathetic nerves. It is convenient to make a division, therefore, into the central or somatic nervous system, with its peripheral nerves, cranial and spinal, and the sympathetic or autonomous nervous system. The Somatic Nervous System. — Since the nerve-fibres of the brain and cord are white in texture, while the cells in mass are of gray color, it is very easy to distinguish the deposits of cells from the fibres and thus make subdivisions of the central nervous tissue. One portion of this gray matter is found deposited in the centre of the spinal cord, extending up to the floor of the medulla, thence underneath and around the aqueduct of Sylvius to the floor of the third ventricle. This is called the central gray matter. Another deposit, much larger in amount, covers the whole of the cerebrum and cerebellum, and forms the cerebral and cerebellar cortex. Smaller deposits make up the great hasal ganglia, corpus striatum, optic thalamus, and corpora quadrigemina, besides several small deposits (the small hasal ganglia cerebral and cerebellar), such as Luys' body, the red nucleus and Deiter's nucleus. The peripheral nervous system contains nerve-cells, as well as fibres. Their anatomical arrangement is easily understood, but their relation to the central nervous system is less simple. The sy?npathetic nervous system consists of (A) an autonomic or parasympathetic portion and (B) a sympathetic system proper (Fig. 15). The autonomic nervous system is in general distinguished from 14 DISEASES OF THE NERVOUS SYSTEM the sympathetic proper by the fact that it does not at first pass into any ganghon, but runs in cranial or spinal nerves until it reaches the periphery. It then enters a ganglionic mass, and from this it sends fibres to the peripheral organ. (A) This autonomic system consists of a mid-brain, a hind-brain Fig. 15. — Scheme of the autonomic and sympathetic system proper, according to Langley. and a sacral portion. The mid-brain autonomic system sends out fibres which run into the third nerve to the ciliary ganglion and thence fibres to the ciliary muscles and the sphincter of the iris. The hind-brain autonomic system sends fibres which run into the nerve of Wrisburg and the seventh nerve; the glossopharyngeal and the vagus. The fibres that run in the nerve of Wrisburg and the seventh GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY 15 nerve go to the submaxillary and the sublingual ganglia and thence supply the vasodilator nerves to the tongue, submaxillary and sub- lingual glands and secretory fibres and vasomotor fibres to the mucous membrane of the nose, soft palate and upper pharynx. It sends fibres via the glossopharyngeal nerve to the otic ganglion and these control secretion and vasodilation in the parotid gland. The most important parts of the hind-brain autonomic fibres run in the vagus nerve. They go to the jugular ganglion and the ganglion of the trunk and of the vagus and thence send motor and secretory fibres to the oesophagus, stomach and small intestines, as far as the ileo- cecal valve. The sacral autonomic system consists of fibres which pass out through the second and third sacral nerves and are connected with the ganglia of the hypogastric plexus. They send fibres which supply motor fibres to the bladder and colon and rectum, inhibitory fibres to the sphincter of the bladder and rectum. In a general way it is found that the autonomic and sympathetic systems antagonize each other in their action, thus the fibres of the au- tonomic system contract the pupil; those of the sympathetic dilate; the fibres of the hindbrain of the autonomic system inhibit the heart and stimulate movement and secretion, while the sympathetic fibres antagonize this action. The same is true though to a less marked extent of the sacral portion of the autonomic system. Autonomic or Para-sympathetic. Mid-brain portion. Bulbar portion. Sacral portion. Contracts pupil. Coronary blood-vessels. Inhibits heart-action. Dilates blood-vessels. Inhibits sweat-glands. Contracts muscle-walls of cesophagus, cardiac sphincter, stomach. Stimulates gastric-secretion. Dilates blood-vessels of rectum, anus. external genitals. Contracts muscles of colon, rectum, anus, external genitals, bladder, urethra. Sympathetic. Dilates pupil. Coronary blood vessels. Accelerates heart-action. Contracts blood-vessels. Stimulates sweat-glands. Relaxes muscle-walls of oesophagus, stomach, cardiac sphincter, Contracts blood-vessels of rectum, anus. external genitals. Relaxes same 16 DISEASES OF THE NERVOUS SYSTEM (B) The sympathetic system proper consists of the two chains of vertebral gangha, of their peripheral ganglia (cceliac, etc.), and of con- necting fibres. Both portions of the sympathetic system have efferent and afferent fibres. The efferent nerves never pass directly to the tissue to be in- nervated, but first go to ganglia, from which a second neuron sends out its fibres to the terminal organ or gland. The afferent or sensory fibres are very few compared with the efferent, so that the viscera have no sensibility except on great and peculiar irritation. The sensations evoked reach the cord but the brain and consciousness are reached through the somatic sensory fibres which in general refer the pain to the corresponding somatic segments. The sympathetic fibres to the viscera run mostly as independent nerves, but the fibres to the trunk and extremities and head run mostly in the trunks of the somatic nerves. I come now to a description of the general arrangement of these various nerve units; and here I must suppose that my reader has a knowledge of the ordinary anatomy of the subject. The nerve-cells of the ganglia on the posterior spinal roots fur- nish the best starting-point in an attempt to trace out the connections. These cells give off a single process, which quickly divides in a T shape. One branch of the T passes peripherally through a mixed spinal nerve to the skin, forming a sensory nerve. The other passes centrally, enters the posterior spinal roots, and breaks up into little filaments, which surround a nerve-cell in the posterior horn or analogous nuclei. This forms the first or outer sensory neuron. The outer branch of the spinal ganglion cell which went to the periphery as a sensory nerve was its dendrite, or protoplasmic process, which has evolved into a sensory nerve and is cellwpetal in function. The other process is the neuraxon proper and it is cellufugal, carrying impulses away from the ganglion cell into the cord. The next neuron begins as a cell in the posterior horn, or in like parts. It sends a neuraxon up the spinal cord, a collateral branch passes to the cerebellar cortex, while the direct fibre surrounds a cell in the optic thalamus. This forms the second sensory neuron. The cell in the thalamus gives off a neuraxon which passes to the gray matter of the cerebral cortex, and here it either directly affects the cells in this region or does it through the medium of another shorter neuron, which is called ''associative." Thus each sensory impulse from the periphery reaches the con- scious centres of the brain by passing along three or four neurons. The primary neuron in all cases lies mainly outside the central nervous system and forms a sensory nerve. The sensory nerves do not there- GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY 17 fore arise in the cord or medulla, but have their terminal nuclei there. Afferent nerves serve the purpose of conducting impulses which arouse sensation, and reflex action. The sensory nerves of the surface of the body are called extero-ceptive when they excite reflex action. They are more closely associated with the cerebrum and the activity of the skeletal muscles. The visceral nerves which excite reflexes are called proprio-ceptive. Thej" are asso- ciated with the cerebellum, and the visceral muscles. They give rise ''':_ A.Jifei/ron _y Fig. 16. — Diagram showing the arrangement of the neurons or nerve units in the architecture of the nervous system. M. Neurons I. and II., Motor neurons; S. Neu- rons I., II., III., sensory neurons; A. Neuron, associative or commissural neuron. to the tonic reflex which keeps the voluntary muscles at a certain degree of tension (Sherrington). (See also Fig. 18 p. 33.) The neurons of the brain cortex cannot yet be distinctly classi- fied, and I shall not attempt it here at all. The matter will be brought out more fully in connection with the anatomy of the brain. It is sufficient to say that nerve units connect together the cerebellum and cerebrum with the basal ganglia, the frontal lobes and the cerebellum, the two hemispheres of the cerebrum, and different areas of the cerebral cortex. Leaving out of consideration these neurons, which are largely psychic in function, we start with the large motor cells in the central 2 18 DISEASES OF THE NEKVOUS SYSTEM convolutions of the brain. These send down neuraxons, which pass into the spinal cord and surround the cells of the anterior horns. They form the upper or central motor neurons. The anterior horn-cells send off neuraxons, which pass out through the anterior roots and thence to the voluntary muscles. These are the lower or peripheral motor neurons. These motor neurons have a double function — that of excit- ing muscles to contract, and that of inhibiting muscular activity and causing them to relax. The efferent nerves are almost equally inhibitory and motor. With each voluntary muscular act impulses to contract, excite for example the flexor groups, and impulses to relax, cause the opposing extensors to relax. ^The motor mechanism is a motor-in- hibitory mechanism. This has been especially established by Professor Sherrington. Besides this there are groups of cells in the lateral horns and central parts of the spinal cord which send off neuraxons that also pass through the anterior root, but they leave the cerebrospinal nerves and enter the vertebral sympathetic ganglia. Here they in part surround the cells of these ganglia and have their terminals there. These sym- pathetic ganglion cells in turn send neuraxons, which pass in the sym- pathetic nerves to the peripheral ganglia, where they meet a third group of neurons. They also connect with the other gangha of their own class and send neuraxons through the posterior spinal roots to the cord. It is not known with what neurons higher up in the nerve-cent- res the lateral horn-cells are connected, but probably with cells in the thalamus. Such in outline is the neuronic architecture of the nervous system. I do not attempt here to work out the neurons of the special senses, nor to introduce the spinal cerebellar neuron. This will be done later. It is sufficient to say that the studies in this direction show a marvellous harmony as well as beauty in nature's scheme. The neuronic architecture is shown in the accompanying diagrams (Figs. 16 and 18). GENERAL PHYSIOLOGY The Peripheral Neurons. — The nerves which run between nerve- centres and end-organs carry impulses each way. They are, therefore, divided into the afferent, centripetal or ingoing, and efferent, centrifu- gal or outgoing. The old division into motor and sensory nerves will not answer, for there are many outgoing nerves which are not motor. The afferent nerves are: GENEEAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY 19 1. The sensory, including: These carry (a) Epicritic sensations and (6) Protopathic sensa- tions. Nerves of general / Pain nerves or pathic nerves, heat sensation. \ and cold or thermic nerves. f Contact, Tactile, including { Pressure, [ Locality. Nerves of special ,-, ^ , , , • , ^. < Nerves or muscular and articular sensation. sense. Nerves of special sense of smell, sight, taste, hearing and space. _ „ .^ „ Extero-ceptive. 2. Excito-renex nerves. { „ ^ . [ rropero-ceptive. Protopathic and Epicritic Sensibility. — According to Dr. Henry Head, the afferent nervous system is composed of two different sets of sensory fibres, called the protopathic and the epicritic. The protopathic set or system which is distributed to the skin and viscera, and to the muscles. (a) The fibres to the skin run in the peripheral nerves and give to it a low degree of sensibility. The fibres to the viscera run in the sympathetic and give to it also a low degree of sensibility. (b) A set of deep fibres to the muscles which supply the sense of deep pressure. These fibres run in the muscular nerves and are con- nected with the Paccinian bodies. The "protopathic system," then, as a whole, consists of fibres of sensibility which supply the skin, the viscera, and all parts of the body, inside and out. It furnishes a low degree of sensibility to the viscera, muscles and to the skin. This system enables one to appreciate a sense of pain and temperature, though not to a very delicate extent. There is another system of .afferent fibres which supply the skin alone, a purely cutaneous system, and this is called epicritic. This epicritiw sensibility enables us to appreciate light touch, the points of the cornpass, localization, and minor degrees of temperature, ranging between 22° and 40°C. Epicritic sensation gives us the sense of the point (a variant of ap- preciation of relative size). Protopathic sensation gives sense of pain apart from the knowl- edge of the pain-producing object. The epicritic fibres furnish a delicate and localized appreciation of pain. Protopathic fibres carry sensations which are badly local- ized, widely diffused and sometimes referred to other parts than that of the stimulation. The protopathic fibres are incapable of appre- 20 DISEASES OF THE NERVOUS SYSTEM elating light touch, and minor degrees of heat and cold, and pain appreciation is a diffuse tingling and thrilling sensation. This distri- bution of sensory fibres exists only in the peripheral nerves. In the nerve-roots and central nervous system the systems are fused. [The above description of the views of Dr. Head and his associates has been seen and corrected by Dr. Head, of whose courtesy I make acknowledgment.] The efferent nerves are: 1. Motor nerves, going to voluntary or striped muscles, heart muscle, smooth muscle, including the vasoconstrictor and dilator nerves. 2. The secretory. These act upon glands. Impulses to the blood- vessels (vasomotor) generally accompany the secretory impulses. 3. The inhibitory. These nerves control muscular movements, secretion, perhaps also nutrition. Central Neurons. — There are intercentral or associative neurons, which connect different parts of the cerebrospinal system together. Some of these connect symmetrical parts on each side together and have a coordinating function. Others connect higher with lower centres and carry ascending and descending impulses. End Organs. — In the peripheral nervous system we have end-organs. These are delicate and in some cases complex arrangements of nervous and other tissue at the periphery of the nerves. Their object is to allow the nerves to be irritated by special stimuli which would not otherwise affect them, e.g., light or sound. Their object is also the proper utiliza- tion of efferent impulses upon other tissues. There are end-organs, therefore, for both sensory or afferent and for efferent nerves. The end-organs of the afferent nerves are — eye, ear, taste-buds, corpuscles in the Schneiderian membrane, various tactile cells and bodies, the space sense-organ, the muscle-spindles. For the efferent nerves — neuro-muscular corpuscles in the vol- untary muscles, local ganglia about the arteries, local ganglia in the glands. Trophic end-organs are not known. In many cases the end- organ is nothing but the terminal fibre of the nerve. This loses both medullary sheath and neurilemma, leaving only the axillary cylinder. It then splits up into a terminal plexus, or else without splitting passes between and around the cells which it is to affect. The Work of the Cell-body of the Neuron. — In the working of these mechanisms the nerve-cell body is the agent which in some instances generates, but more generally distributes and directs the impulses which pass to it. The larger the nucleus of the cell in proportion to its proto- plasmic body, the more stable or less sensitive the cell. The larger the amount of protoplasm relative to the nucleus, the more active the dis- charging power of the cell. The nucleus is the part of the cell-body which GENERAL ANATOMY, PHYSIOLOGY, AND CHEMISTRY 21 is essential to constructive metabolism. By means of it the cell builds up its protoplasmic substance. When the nucleus dies, the cell may live or function for a time, but it lives only on what has been stored up; it can build no more and soon dies. Nerve-cells with few exceptions (spinal ganglia) have no centrosomes; they cannot divide and multiply. Once dead they cannot be restored. The nerve-fibres conduct impulses variously generated. These im- pulses travel at the rate of about 100 to 120 feet per second. It is less in visceral nerves (25 to 30 feet per second). Nerve-cells are ex- hausted by successive stimulations, but nerve-fibres can be continually excited and are practically non-fatiguable. There are no electrical currents in normal living nerves (Landois) except when an impulse travels along them. Then an electrical current travels along with the im- pulse. It is called the current of negative variation. The irritability or excitability of a nerve is the power it has of responding to a stimulus. When a constant electrical current is passed along a nerve its irritability is modified. This modified condition is called electrotonus. When a nerve-impulse passes up an afferent nerve and is then reflected along an efferent nerve, it is called a reflex action. The time required for this process is called the reaction time. This averages from 0.125 to 0.2 of a second. CHEMISTRY The specific gravity of nervous tissue is about 1.036; that of the brain is 1.038; of the spinal cord and nerves, 1.034 (Bischoff, Krause). The reaction is alkaline, but this is lessened by activity, owing to the development chiefly of lactic acid. The gray matter is less alkaline than the white. The nervous system has the following composition (Baumstark, quoted by Hammarsten) : White Matter Gray Matter Water in 1,000 parts 695.35 769.97 Solids 304.65 230.03 T, . I Cerebrin 1 ^ ^ , , „ ^ ProtagoiK^ } 25.11 10.08 [ Lecethm (neurm) J Insoluble albumin and connective tissue 50 . 02 60 . 79 Cholesterin 45 . 12 23 . 81 Nuclein 2.94 1.99 Neurokeratin 18 . 93 10 . 43 Inorganic salts 5 .23 5 . 62 Water makes up nearly three-fourths of nervous tissue, there being more in the gray than in the white matter and least in the sympathetic nerves. The inorganic salts amount to about 0.5 per cent. The largest single constituent is phosphorus (Breed) combined with potassium, 22 DISEASES OF THE NERVOUS SYSTEM sodium, magnesium, calcium, and iron, forming phosphate salts. Of the other constituents chlorid of potassium is the most important. Protagon is a very complex substance of a fatty character, con- taining nitrogen and united with glycerin-phosphoric acid instead of glycerin. It is said by some to be made up of two bodies, cerebrin and lecethin, the latter containing an ammonia compound called neu- rin. Protagon is especially found in the white matter. The gray matter and axis-cylinders contain globulins, nucleo-proteid and nuclein, a very important substance in cell metabolism. Nuclein (C29H49N9P3O22, Miescher) is composed of nucleic acid, a substance rich in phosphorus and a variable amount of albumin. The gray matter, i.e., the nerve- cells, contains also various albuminous substances. Nucleo-keratin is found in the neuroglia and medullary sheaths. The nuclein and the allied substance nucleo-albumin are called albuminoids (Halliburton). They both contain phosphorus and are found chiefly in the nucleus. The albuminous substances, called also proteids by Halliburton, have little or no phosphorus, and make up the most of the cell-body or cytoplasm. CHAPTER II THE CAUSES OF NERVOUS DISEASES Nervous diseases are produced in part by predisposing influences which may be likened to a fecund soil; in part they are due to exciting causes, which are like the seeds dropped upon the soil in the accidents of life. Heredity is the most serious and important of these predisposing causes, in particular of those neuroses that are constitutional and are not the results of bodily accidents. A nervous disease, however, is rarely directly inherited. Parents do not pass down special maladies, but only a general tendency to nerve disease, which is not developed into any distinct trouble unless some disturbing cause arises. Nervous parents may have children who have unstable, over-irritable, and inadequate nervous systems. Such persons have what is called a neuropathic constitution or diathesis. This diathesis may be transmitted when the parents, though not especially neurotic, suffer from syphilis, alcoholism, and diseases of malnutrition, like tuberculosis. So far as the office of parentage goes, persons of great talent in affairs, or great artistic genius in any direction, may be counted as neurotic and are very likely to have children of neuropathic constitution. This is less apt to be the case when one parent is of stable and lymphatic type. If two persons having not simply a nervous constitution but distinct nervous or mental disease marry, their children are liable to serious nervous or mental disease. The intermarriage of blood relations, such as first cous- ins, does not lead to neurotic children, if the parents are not neurotic themselves, and are of robust health and dissimilar temperaments. Injuries or even severe shock to the mother during the early months of pregnancy sometimes leads to nervousness in the offspring. The mother transmits neuroses more often than the father. There are certain rare nervous diseases which appear in different branches and members of a family, such as an uncle, cousin, nephew, and son. These diseases may pass also by direct inheritance from parent to child, or may skip a generation. They are called "family diseases," and are of the nature of congenital defects, like webbed fingers or club-foot. Degeneration is the name given to a condition in which there is a morbid deviation from the normal average in mental traits, physio- logical powers, or physical structure. It is a term most often used in connection with mental characteristics. It is most often an in- 23 24 DISEASES OF THE NERVOUS SYSTEM herited state, and the word degenerate is often used to indicate a person who has a hereditary neuropathic or psychopathic constitution. De- generacy in a moderate degree often accompanies great mental powers, especially of the artistic kind, and it is almost invariably associated with genius. It is quite compatible with mental soundness and a fair degree of physical health. It is then well to use the term, suggested by Walton, of deviation. Those who have unusual mental gifts and degenerate characteristics are called superior degenerates. The criminal and the insane and erratic and eccentric persons of weak judgment have also the neurotic constitution, and are called inferior degenerates. The weak-minded, imbecile, and idiots form the lowest class of degenerates, and are called the dehiles. Degeneracy of constitution whether inherited or acquired is a condition which fits the person for acquiring nervous disease. Age. — In infancy and early childhood, nervous diseases are rather frequent on account of the accidents at birth, the liability to infectious fevers, and malnutrition, and the high degree of sensitiveness of the yet immature nervous system. Still, a carefully watched infant is relatively safe. Motor disorders, such as paralyses, convulsions, and chorea, are much the more common troubles. At the time of puberty sensory disorders, such as headache, and migraine appear, and often epilepsy, hysteria, and disorders of sleep. Hereditary tendencies to nervous disease also begin to develop at this time or a little later. At the period of adolescence, the maladies already mentioned also may be brought out; but in addition neurasthenic, morbid sexual, hypo- chondriacal, and insane tendencies are seen. From maturity to the time when degenerative changes begin, forty to forty-five, the indi- vidual suffers from those nervous disorders brought on by accidents', injuries, prostrating attacks of sickness, over-strain, infections, in- dulgence in alcohol and narcotics, and the abuse of the bodily func- tions. At and after the climacteric, one sees oftenest such maladies as result from vascular disease, apoplexies, brain softening, also severe forms of neuralgia, and spasm. Sex. — Sensory and functional disorders are more frequent in women ; motor and organic disorders more frequent in men. Condition and Occupation. — ^No general facts will be laid down here. Celibates, however, it may be said, suffer more from nervous disorders than married people. It will be shown later that certain occupations entail special nervous disorders and that indoor life pro- motes functional nervous diseases. Work.^ — ^Hard, constant, excessive muscular work, leads to arterial sclerosis and the neuroses that result from a defective circulation. Mental work produces no injurious effect upon metabolism and is a THE CAUSES OF NERVOUS DISEASES 25 healthful form of activity, even intense and long-continued mental work being comparatively harmless. It is the worry and strain and emotional excitement associated with hard work that does harm and leads to neurasthenic disorders. Mental Attitude. — -The expectant and untrained mind is much more liable to nervous disease as the result of injury, shock, etc. Little evil can befall a prepared mind. The Freudean school asserts that a person who has been ''analyzed" is much less subject to the minor psychoses. Habits. — Excessive indulgence in alcohol is a most prolific cause of nervous disease, chiefly by the action of this substance on the blood- vessels and the stomach. Excesses in eating, in tea-drinking, irregu- larity in sleeping, and bad habits of working predispose to nervous dis- ease. Sexual excesses are usually the result rather than the cause of nervous disorders. They are the evidence of mental more than of nervous weakness. Bad mental habits acquired usually in early life as the result of wrongful education or a poor environment and example lead to many functional nervous disorders. By bad mental habits is meant especially the tendency to lay too much stress on trivial things, to worry and "fuss" over details, to have "precisions," to get "notions" and assume as guides in life foolish types of religion, philosophy or sanitation. The obsessive neurasthenic is one of the characteristic features of our American civilization. Climate and Civilization. — Nervous diseases are most frequent in temperate climates, and in those which are dry and elevated. They increase with the progress of civilization and the greater strain, com- plexity, and luxury of modern social life. Those organic nervous dis- eases which are largely dependent on vascular disease are frequent in the poorer classes, among whom syphilis, alcoholism and bad feeding prevail. Functional and degenerative disorders are frequent in the higher classes. Nervous diseases, if we except those of the degenerative type, prevail more in urban populations. Diathesis. — The rheumatic and gouty diatheses predispose to nervous troubles, more especially those which are of a peripheral and functional nature. Those allied conditions in which the products of tissue waste are not properly oxidized and eliminated, have a similar influence. Trauma and Shock. — Exhausting hemorrhages and trauma may be the direct cause of or may predispose to nervous disease. Trauma and mental shock may cause functional diseases, such as neurasthenia, or may lead to the development of insanity or indirectly to degenerative organic disease. Mental shock, and especially a fright, oftener than severe bodily injury, leads to the development of functional neuroses. However, it is only the weak, the neurotic or unprepared who can be thus affected. 26 DISEASES OF THE NEKVOUS SYSTEM Infections. — In comparison with their frequency, the infective fevers are not great factors in producing nervous disease, but practically they often play an important part. Scarlet fever is the most dangerous dis- order in this respect. Measles perhaps ranks next; then follow in- fluenza, diphtheria, typhoid fever, and pertussis. Among chronic in- fections syphilis ranks first; tuberculosis, malaria, the pellagra, and beriberi are also to be mentioned. The importance of syphilis which causes perhaps one-half of organic nervous diseases will be dwelt upon later. Poisons. — Tea, coffee, cocoa, tobacco and lead, mercury, copper, and arsenic, are to be placed among the causes of nervous disease. But methyl and ethyl alcohol are justly credited with exerting the most sinister influence on the nervous system of all poisons. Methyl or wood alcohol is a deadly poison whether drunk or inhaled in any considerable amount. Ethyl alcohol is generally classed among the narcotic agents. It has, however, a primary stimulating effect upon the organs of the circu- lation, respiration and digestion, and also upon the brain. Its effects vary enormously with the dose and with the constitution of the patient. It is a food in the sense that about 90 per cent, is used up in the body and transformed into heat and energy, like sugar. It is not, however, a very good food under ordinary conditions, but it can be used as such, in daily amounts not over Bii by many people, after adolescence. Alcohol is also, correctly, said to be a poison, though this may be said of almost any substance that is taken into the system, if used to excess. Alcohol, however, is a dangerous poison to a minority of people, either because they cannot use it temperately, a small dose leading them at once to excesses, or because it excites and disagrees with the functional activities of the system. Alcohol is always a poison when taken in ex- cess and in some people a small amount may be an excess. The de- generate types of nervous systems are very sensitive to alcohol, and usu- ally made worse by it, so that the use of it by degenerates tends to make them worse and extinguish this group of the race. On the other hand, alcohol by intemperate use, can lead to an acquired degeneracy in healthy constitutions. History shows that both those races which indulge excessively in alcohol and those which do not use it at all, either degenerate or do not progress. Alcohol causes a small percentage of epilepsy through inheritance, and a still smaller percentage of epilepsy among its users. It is a large factor in the pro- duction of acute mental disturbances, such as delirium tremens, but not a large factor in causing degenerative insanities or imbecility or idiocy. In my own experience, the percentage of alcoholism in the ascendants of the insane, imbecile and idiotic, is about 5. The ratio THE CAUSES OF NERVOUS DISEASES 27 of insanity to the population is about the same in prohibition as in other states in this country. In persons who inherit degenerate constitu- tions, that is to say, in persons with a weak and unstable nervous system, alcoholism is often only an expression of this instability, not a cause of it. For it is a sign of degeneracy to drink alcohol to excess or to have a morbid susceptibility to its effects. Alcohohsm and habits of alcoholic excess in this country almost always develop before the age of thirty, and if drinking were prohib- ited to those below that age, there would be but little trouble. Wine drinking, if we except champagne drinking, is almost never a cause of alcoholism in this country, and is a much less important factor than beer. This is largely, no doubt, due to the fact that wine is relatively little drunk in America. Beer and ale, if used in excess, are most in- jurious forms of alcoholic beverage, except to out-door workers, be- cause they lead to digestive and metabolic disturbances. It would be wise to forbid the use of alcohol to those under thirty, except in the form of light wines or beer, and those only to workmen and people who live out of doors. Even after this age it should only be given to the certified immunes, that is, to those able to drink moderately. It follows that, theoretically, at least, the sale of alcohohc liquors should be controlled by the Boards of Health. It would not be wise to abolish the use of alcohol altogether from civilization until we can be sure it has no good function, or until we can be sure that its abolition would not be followed by the use of more injurious substitutes. It is impossible to deny that civilization has satisfactorily advanced, despite alcohol, and has shown its finest types among those nations who have used it most. Owing to the fact that sanitation and civihzation have developed a rather more delicate type of nervous organization, and to the fact that sanitary care has kept more of the unstable and weakly alive, we cannot use alcohol as freely as formerly. Therefore, alcohol has got to be used more and more carefully, and under closer supervision and perhaps finally will have to be abolished absolutely from general use. Arterial sclerosis is a condition brought about by many of the factors that have been here enumerated as causing nervous disease. Its special and direct importance will be shown in the special chapters. Reflex Causes. — Among other causes are local disease of viscera, such as renal, uterine, and ovarian diseases, dyspeptic and liver dis- orders, visual and auditory troubles. Reflex irritations are distinc- tively exciting causes, but with few exceptions they cannot cause a nervous disease unless there is a predisposition to it. They may, how- ever, cause many distressing nervous symptoms, such as pain, spasm, and even convulsion. CHAPTER III GENERAL PATHOLOGY The following is a list of the forms of disease which affect the nervous system : 1. Malformations; incomplete development, or agenesis; defective development, or dysgenesis. 2. Hypersemia, anaemia, hemorrhage, oedema, and arterial and venous diseases. 3. Degeneration and atrophy, softening, sclerosis, classed as regres- sive processes. 4. Inflammations. 5. Tuberculosis and syphilis. 6. Tumors and parasites; 4, 5, and 6 being classed as progressive processes. 7. Nutritive and functional disorders, including disorders asso- ciated with metabolic and glandular defect, such as acromegaly and exophthalmic goiter. The pathology of most of the above types of morbid processes will be given elsewhere, and does not call for discussion here. Degeneration and Sclerosis. — By degeneration is meant in pathology a gradual death of the nerve-cells and fibres, or, in other words, of the parenchyma of the organ. The cells swell up, become granular and fatty, and then either break up and become absorbed or enter into a condition of a dead coagulum (coagulation necrosis). Degenerations may be acute or chronic, primary or secondary. Acute neural degeneration causes a condition known as softening or necrosis. It is due to cutting off of vascular supply, direct injury, and to necrotic and inflammatory poisons attacking the neurons. Acute degeneration may be followed by a reparative process, which is called a reparative or reactive inflammation, and which ends, perhaps, in produc- ing a cicatrix or sclerosis. Chronic degeneration is a slow neuronic death and is accompanied and followed by a proliferative process which results in the production of connective tissue and sclerosis or gliosis. Degeneration is by some writers classed as a degenerative or parenchymatous inflammation. Sclerosis is a process of connective-tissue proliferation, as a result of which the normal or injured parenchyma is supplanted by neuroglia 28 GENERAL PATHOLOGY 29 and fibrous tissue. The word sclerosis is usually employed in describ- ing degenerative diseases, though it indicates the result rather than the primary nature of the process. In the nervous system there is often an increase or proliferation of neuroglia tissue in the processes of de- generation. Exactly how large a factor this is cannot yet be said. In one form of sclerosis^ — multiple sclerosis — ^the process of neuroglia pro- liferation seems to be the primary one, nerve-cell destruction following. Hence this type of sclerosis is classed with the proliferative inflammatory processes. Degenerations are caused by certain poisons, such as arsenic, phos- phorous, lead and the poisons of infectious disease. Degenerations also result from obliterating arteritis, such as occurs in old age or from humoral poisons. Degenerations sometimes are due apparently to an inherent defect in the cell nutrition — a premature death of it; and to causes yet unknown. The question as to whether certain scleroses are forms of productive inflammation or of chronic degeneration is one that has been much debated in the past. It is quite certain now that most of the so- called chronic inflammations of the nervous centres are really degenera- tive processes, and that the primary trouble is in the parenchyma, and not in the connective tissue. Gliosis. — When the pathological process is the result of a prolifera- tion of neuroglia, not of connective tissue, it is called gliosis. Nutritive and Functional Disorders. — Under this head are included defects due to heredity, to disorders of the blood and blood-glands, to defects in metabolism, to poisons, extrinsic and autochthonous, and to local diseases. . CHAPTER IV GENERAL SYMPTOMS When the nervous system is disordered it produces various symp- toms, which are classified and receive names according to the parts affected and the kind of change present. The general name given to any kind of morbid nervous state is neurosis and the general name for any morbid mental state is psychosis. When the neurosis affects the motor sphere, whether in the brain or cord or nerves, it is a motor neurosis; when the sensory parts are disordered we have a sensory neurosis. In the same way we have trophic, thermic, vasomotor and secretory neuroses. The symptoms of nervous disease can be to a large extent divided in accordance with the kind of disturbances present. Now a function can be disordered in three ways. It may be exaggerated, lessened, to the point perhaps of entire loss of function, or it may be perverted. In order to indicate this, certain Greek prefixes are used. They are ''hyper," which means excess; "hypo," meaning diminution; ''a" or "an," indicating entire loss; and ''para," meaning perverted. Thus we have, for example, hypersesthesia, or excessive sensibility; anaesthesia, or loss of sensibility; and parsesthesia, which means perverted sensibility. Finally, nervous symptoms are often spoken of as objective or sub- jective. The former are those symptoms which can be seen or directly noted by the physician without depending on the patient's statements. The subjective symptoms are those which are felt by the patient, but give no outward sign. Thus headache is a subjective symptom, paralysis is an objective one. So far we have been grouping together only like kinds of symptoms; but it happens that one nervous disease may have quite different kinds, some being motor, some trophic or sensory. Thus nervous dis- eases practically are to a considerable extent classified not functionally but anatomically ; and we have spinal cord and brain diseases, gastric and sexual neuroses, and so on. Nervous sym'ptoms, however, are always grouped together in accord- ance with the physiological function disturbed. So that we have the following tabulation (see also Fig. 17) : 1. Mental and cerebral. 2. Motor and reflex. 30 GENERAL SYMPTOMS 31 3. Sensory (general, and special sense neuroses). 4. Trophic. 5. Vasomotor (angioneuroses) . 6. Secretory (secretory neuroses). Combinations of these groups of symptoms may affect various organs. They are called mixed neuroses. Combinations of mental and nervous symptoms form psychoneuroses. The particular symptoms which nervous diseases cause will be described and recorded under the several heads given above. 1. The mental symptoms include all those found in insanity, idiocy, and imbecility, and will not be given in detail here. The common symptoms met with by the neurologist are mental irritability, depression, r\/n Psychoses inesioncuroses Aesthesioneuroscs Secretory &iro£)}ii& VcLscmofor Fig. 17. — Diagram illustrating the principle of the classification of nervous symp- toms, aside from special sense neuroses. emotional excitement, morbid fears, volitional weakness and lack of self-control, persistent or fixed ideas, weakness of memory and of power of concentration, and a tendency to hypnotic and somnambulistic states. Certain symptoms due to disturbance of brain function are called cerebral or cerebellar. They include such conditions as vertigo, disorders of equilibrium, stereognosis, aphasia, cerebellar ataxia, and many symptoms connected with the special senses, which will be described in the special chapters later. 2. Motor Symptoms. — The symptoms of disordered motility are as follows : (A) Symptoms of exaggerated or perverted motility. Tremor: (a) Fibrillary and wave-like movements of muscles (myokymia) . (6) Tremor proper. Rhythmical spasm, athetosis. Convulsion. Myoclonia, a general name for muscular twitchings and including the choreic and tic spasms. Hypertonia and contracture. 32 DISEASES OF THE NERVOUS SYSTEM Forced and associated movements. Ataxia and asynergy. Exaggerated reflex conditions. (B) Sympto7ns of lessened motility. Paralysis and paresis, myasthenia, muscular atrophy. Loss of reflexes, superficial and deep. Hypotonia or lessened muscular tonus. The particular characteristics of these different symptoms will be best shown in the description of the special diseases, but a brief account will be given here. Tremor is the result of a disorder in the tonic innervation of muscles, and the cerebello-mid-brain mechanisms are important factors in many types. Muscles are kept normally in a state of slight tension with rhyth- mical impulses passing down at the rate of about twelve per second. When the rhythm and force of these normal impulses are interfered with we have tremor. The simplest form of tremor is one in which the normal rhythmic impulses have an apparently exaggerated force. This causes a fine tremor of eight to twelve vibrations per second (Fig. 19) . When there is an interruption to some of the impulses we have a coarse tremor. Here the vibrations are four to eight per second caused by a partial or complete dropping out of the alternate impulse. Various technical names are used in describing the tremors. We have the fine and coarse, as described Intention tremor is one that occurs on voluntary movement, and is opposite in kind to the passive tremor or tremor of rest, which does not increase on voluntary effort. Tremor is sometimes of a coarse, jerky, and inco-ordinate character, and these words are then used to indicate it. Fibrillary tremor or myokymia is a fine twitching of the individual strands or parts of muscles, and occurs usually when they are wasting from toxic or neurotrophic influence. Closely related to it are the wave- like contractions of the muscle-body without motor effect. Convulsions consist of abnormal and exaggerated muscular con- tractions occurring in rapid succession. Convulsions may be clonic, i.e., the muscles rapidly and alternately contract and relax in an ex- aggerated and irregular way; or they may be tonic, i.e., contracted suddenly and steadily for several seconds or even minutes. When a tonic muscular contraction is painful it is called cramp. Convulsions may be co-ordinate. In this case the patient moves the limbs and body in a more or less purposeful way. He throws himself about the bed, jumps, kicks, strikes, tears the clothes, etc. Convulsions are usually accompanied with loss of consciousness. Myoclonia is a term used to indicate muscular twitchings of various types, such as those of chorea and the "tics." GENERAL SYMPTOMS 33 Choreic rnovenients are sudden jerking, twitching movements of different groups of muscles. The movements are purposeless and are not under control of the will. Convulsive tic is a form of spasmodic movement confined to certain groups of muscles which work together for a common purpose, like those of the face, or eyes, or larynx. The movements in the "tics" are definite in character and are limited to muscles physiologically grouped for a special function. Thus we have tics of the muscles of expression, or of respiration, or speech, or locomotion. OPTIC TRACT TECTUM MESENCEPHALI 1 RED NUCLEUS JECTO-SPINAL TRACT , RUBRO-SPINAL TRACT METATHAIAMUS THAIAMUS BRACHIUM CONJUNCTIVUM ,- LEMNISCUS MEOIALIS ,V LEMNISCUS LATERALIS CEREBELLUM CORPUS STRIATUM CEREBRAL HEMISPHERE CEREBRO-SPINAL , ^ , ,. ., TRACT' ' X''-4 OLTACTORY NERVE Fig. 18. — Showing in detail the neuronic architecture of the nervous sj^stem in the human embryo. (Cunningham.) Athetosis is a name given by Hammond to a peculiar form of move- ment characterized by slow, successive flexion, extension, pronation, and supination of the fingers and hand and arm, or of analogous move- ment of the toes and feet. The motion rarely ceases in waking hours except for a short time. The contractions are forcible, steady, and even, and sometimes painful. The hand assumes characteristic posi- tions. A contracture is a tonic muscular contraction of long duration, i.e., 3 34 DISEASES OF THE NERVOUS SYSTEM days or months. A contracture may be functional or organic; and in order to test this, one must find whether it ceases during sleep or under an anaesthetic; if so, it is functional (see Hysteria). It may be para- lytic; i.e., due to paralysis of opposing muscles, or it may be due to a continuous spasm. In forced movements the patient suddenly and involuntarily is thrown forward, sideways or whirled about in various ways. Associated movements are those which occur involuntarily in a limb or muscle at rest when the corresponding limb or muscle is moved on the opposite side. Thus in hemiplegia the movement of the normal arm may excite a movement in the one paralyzed. The patient is given a piece of chalk in each hand, and each hand is placed upon a black- board lying on the table; attempts at drawing lines with the sound arm cause movements of a similar kind, but less perfect, on the paralyzed side. A|l«.^|/%l^/A#/^i//*AAAUJi/|yiiiAA/WWw/l^l^^ Fig. 19.- -Diagram of a fine tremor. Ten of the divisions on the lower line equal a second. Myoidema is a tonic spasm of a part of a muscle. It is produced by a sharp blow upon the muscle. This causes the muscular fibers to bunch up into a small tumor for several seconds. Its presence may indicate rapid muscular wasting from exhausting disease, or a toxic state. Idiopathic muscular spasm is a phenomenon of a similar nature. When the belly of a muscle is struck with a dull instrument, a welt of contracted muscle appears and lasts several seconds. It indictes an exaggerated muscular irritability. B. Symptoms of Lessened Motility Paralysis or akinesis is a loss of motor power. Monoplegia is a condition in which one limb is paralyzed; hemiplegia one in which one-half the body is paralyzed; and paraplegia one in which the two lower limbs are affected. Sometimes a double hemiplegia or diplegia occurs. The term paralysis is sometimes used to indicate loss of any kind of function, as paralysis of sensation or secretion. Paresis is a term used to indicate a partial paralysis. It is Hot GENERAL SYMPTOMS 35 to be confouiulcd with the term general paresis, whicli is a foiin of insanity. The Reflexes. — When an impulse, started in an afferent nerve, reaches the spinal cord or medulla and is thence reflected upon an efferent nerve, the result is called a simple reflex action. The process is an invol- untary one. It ordinarily occupies one-tenth to one-twelfth of a sec- ond. The afferent nerve may be an ordinary cutaneous sensory nerve, or it may l)e a special nerve whose function is to excite reflex action. These latter nerves are called excito-reflex. This kind is principally supplied to the viscera. In neurology we have to do with three kinds of simple reflexes: The skin or superficial reflexes. The deep reflexes. Fig. 20.— Babinski reflex. The visceral reflexes. The idiopathic or direct muscle response is not, strictly speaking, a reflex. All these may be exaggerated, modified, lessened, or absent. Further description of the reflexes will be given under the head of diagnosis. The simple reflexes are combined to form compound or higher and more complex reflexes, which underlie automatic actions. 3. Sensory Symptoms. — The sensory functions include all those belonging to the nerves of general and special sensation. Sensory nerves have a part in reflex action and in the inhibition of motor and other functions. The nerves of special sense when deranged show various phenomena, which will be described in more detail later. In general there may be depression or loss, increase or perversion of their function. In accordance with this we have: 36 DISEASES OF THE NERVOUS SYSTEM AncBsthesia, which is a loss of tactile sensibility. Analgesia, a loss of sensibility to pain. Thermo-ancBsthesia, a loss of sensibility to temperature. There may be loss of cold sense, of heat sense, or, as is usually the case, of both senses. The term anaesthesia is often used with a general meaning to indi- cate loss of all forms of sensibility. Anaesthesia in this sense is a symp- tom referred to the skin, bones, mucous membranes, special senses or viscera. The muscles have two kinds of sensibility: a sensibility to pain and pressure and a special muscle sense. Anaesthesia of the pain- pressure sense of muscle is called loss of muscular sensibility or muscular analgesia. Anaesthesia of the special muscle sense is one of the factors in causing a symptom known as ataxia. Ataxia is a symptom due to loss of the special sensibility of the mus- cle, articular surfaces and tendons, causing irregular and inco-orclinated movements. This special sense, sometimes called deep sensibility, in- forms the individual' of the degree and strength of muscular movements, and by it definite and co-ordinated movements are made possible. The weight of objects and position of the limbs are also determined by it. In static ataxia there is loss of the power to preserve perfectly the equilib- rium when standing. In locomotor or motor ataxia there is loss of power to co-ordinate the limbs properly in motion. In both these conditions there is also usually a loss of power to appreciate weights or the position of the limbs. Cerebellar ataxia is a form of inco-ordination and disturbed equilib- rium due to disease of the central organ of equilibration, viz., the cerebellum. Cerebellar ataxia is due to disturance of (a) the equilibrium mechanism, (6) and of certain co-ordinating and tonic mechanisms which enable one to move the segments of the body in proper degree and rhythm. Their disturbance causes symptoms called asynergy, adio- kokinesis, and hypermetria. These will be described later in connection with the diseases of the cerebellum. Aster ecgnosis. — The ability to recognize the form of objects held in the hand or on other parts of the body is called stereognosis , and its loss aster eognosis. It is a perceptive process, due to the fusing and elaboration of cutaneous and deep sensations, especially the latter. Astereognosis is present in peripheral or spinal lesions where there is complete tactile anaesthesia, and where, even with good tactile sense, there is loss of deep sensibility. As complete tactile anaesthesia is rare in cerebral lesions, the cause of astereognosis here may be due tp injury of the center for deep sensibility or of perception of form. The lesion in such cases is localized in the parietal lobe. The abihty to recognize not only the form but the nature of an GENERAL SYMPTOMS 37 object is called symbolia, and its loss asyniholia. The recognition of the nature of an object is only a higher elaboration of the preceptive work than that in which simply form is recognized. But the nature and location of the process are the same, practically, as for stereognosis.^ Hyper (Esthesia is an excessive sensibility to touch, contact, and other sensory stimuli. Hyperalgesia is excessive sensibility to pain, and is nearly identical with tenderness. DyscBsthesia is simply disagreeable parsesthesia. ParcBsthesia is a term applied to all the morbid general sensations except pain. The parsesthesias include such feelings as numbness, prickling, formication, flushing, burning, itching, coldness, tickling, various peculiar visceral sensations. Ordinarily in speaking of par- sesthesise, however, we refer to such feelings as numbness, prickling and creeping. Delayed sensation is a symptom in which an appreciable time exists, usually one or more seconds, between the time of applying a stimulus and its appreciation in consciousness. Normally a tactile sensation can be felt and responded to in less than one-tenth of a second. Transferred or referred or reflex sensations are those in which the irritation is made at one point and felt at another. Thus an irritation in the stomach causes a pain felt in the forehead. The whole class of so-called reflex pains are really transferred sensations, since in reality there is no reflex action in the process, as will be seen later. Allochiria is a peculiar form of transferred sensation, in which an irritation applied on one side of the body is referred to a corresponding point on the oppo- site side. 4. Trophic Disorders. — These consist, so far as relates to neurology, chiefly of hypertrophy and atrophy of nerves, muscle, cutaneous and mucous tissues, joint degenerations and various skin eruptions. The trophoneuroses, if they affect joints, are called arthropathies; if muscles, atrophies, hypertrophies, and dystrophies; or if with atrophy there is a great substitution of fat, the condition is known as lipomatosis. When nerves are affected there results degeneration. Trophoneuroses of the skin produce various symptoms, such as herpes, pemphigus, pig- mentation, leucoderma, alopecia and bed-sores. Trophoneuroses so called are not due directly to lesions of trophic nerves, but indirectly to disturbance of vascular, secretory, sensory and motor nerves. • 5. Vasomotor and Secretory Symptoms. — The nerves supplying the blood-vessels and secreting glands work together and are usually dis- ordered together. Separate disturbances of the vessels and glands, 1 The terms tactile amnesia (Burr) and gnosia and agnosia are used for these or simi- lar perceptive processes. 38 DISEASES OF THE NERVOUS SYSTEM however, occur. Angioneurosis is the term given to disorders of the vasomotor centre and nerves. Angiospasm is a condition in which there is increase of vasomotor tone and spasmodic contraction of the muscular coats of the arteries. Angioparalysis represents the opposite condition. Such disorders affecting the skin are shown by pallor and coolness or by flushing and heat. Angioataxia is a condition of varia- bility and irregularity in the tonus of the blood-vessels. The secretory neuroses affect the functions of the skin, mucous membranes and special glands. Hyperidrosis is an excessive sweat- ing. Anidrosis is excessive dryness. Paridrosis is a perversion of secretion in which peculiar odors or colors are noted. Hcemidrosis is the term applied to bloody sweating. The secretions of the internal organs are controlled by nervous influences, and their special disturbances often form part of the symp- toms of nervous diseases. Thus we have watery diarrhoea in Basedow's disease, and a peculiar membranous discharge from the bowel in asthenic states. The glands of internal secretion and particularly the thyroid and pituitary glands, have perversions of function which apparently lead to serious nervous symptoms, which will be described under the head of exophthalmic goitre, acromegaly, and dyspituitarism. CHAPTER V DIAGNOSIS AND METHODS OF EXAMINATION The diagnosis of a nervous disease may be simply a clinical one; that is to say, one may recognize it as belonging to a certain known and definite group of symptoms. Thus, in recognizing the phenomena of epilepsy, one makes a clinical diagnosis. In other cases, and es- pecially in all organic nervous diseases, the physician must make in addition a local and then a pathological diagnosis. That is, we must determine the seat and nature of the disease. A diagnosis is made by first getting all the obtainable facts in the patient's past history, then by learning from him all his subjective symptoms, and finally by making an examination according to the technical methods to be here described. In examining a patient, it is imperative that a careful search for diseases outside the nervous system first be undertaken. Then the morbid nervous phenomena should be investigated. The physician should make it an invariable rule to make this examination in a certain fixed and systematic manner. The best method is first to get the family and personal history, and then to go oyer the mental, cerebral and special nervous functions serially in the way indicated under the description of general symptoms. In addition to the routine methods we have often to make special tests to determine the existence of foreign bodies, fractures, tumors and disturbances of the blood, urine and cerebrospinal fluids. In investigating the family history, it is often necessary to make very direct and probing inquiries, for patients are, as a rule, inclined to forget or ignore the existence of nervous and mental disease among relatives. The existence of consumption and inebriety, epilepsy and sy- philis in the direct line are very important facts; so also are those con- cerning birth. The patient should be questioned closely as to his previous diseases, especially syphilis; also as to his habits in relation to sexual indulgence, indulgence in alcohol, and smoking. In women, the tea habit should be inquired into. The patient may be allowed to tell his own story first. Proper queries should be put to supplement this, and finally the patient should be asked to state those symptoms which to his mind are main and dominant. We will now go over the above points in detail. 1. The Physiognomy, complexion, and general nutrition are first noted. Many nervous disorders are compatible with a very healthy 39 40 DISEASES OF THE NERVOUS SYSTEM appearance, and patients often make the introductory apology, "I don't look like a sick person." An anxious look, restless manner, and excited or diffident speech, however, often show something wrong. The nervous trouble is usually serious in reverse proportion to the voluble anxiety of the patient to make his condition exactly understood. The character of the gait may reveal at once the nature of the malady. The dropped foot and flaccid swing of the leg in poliomyelitis and neuritis, the stiff shuffling march of paraplegia from myelitis, the waddling move- FiG. 21. — Photograph of roof of the mouth of a drunkard showing torsus palatinus. ments of juvenile muscular dystrophy, and the bent head and careful stamp of locomotor ataxia are almost of themselves diagnostic: Et verus incessu patuit morbus. The speech also often betrays the malady. The physician soon gets to recognize not only the striking symptoms of aphasia, but also the weak piping of paralysis agitans, the stumbling enunciation of paresis, and the peculiar dysarthrias of multiple sclerosis and bulbar palsy. As a rule, the occurrence of speech difficulties in adults is significant of organic and often serious disease. DIAGNOSIS AND METHODS OF EXAMINATION 41 In the chronic and constitutional nervous maladies note should be made of the marks of degeneration or the neuropathic constituent. The nature of this condition has already been described under the head of hereditary causes of nervous disease. The existence of degeneration im- plies an imperfect, unusual, or unbalanced development. The con- dition is usually shown in some nervous or mental peculiarity or defect in the individual, and degeneracy, as ordinarily understood, implies a neuropathic or psychopathic state. But degeneracy may also mean only a lessened vital resistance to certain forms of infection or injury, as, for example, in persons of a tuberculous tendency, who often have marks of degeneracy. These marks are called the stigmata of degeneration. They are of three kinds: anatomical, physiological and mental. Anatomical stigmala: Cranial anomalies, e.g.: Asymmetrj^ of cranium. Microcephalus. Peculiar shape of skull, trigonal, scaphocephalic, plagiocephalic. Facial asymmetry and ex- cessive prognathism. Large jaws. Deformities of the thorax, such as the pigeon-chest, the funnel-chest, the narrow barrel-shaped chest with rib set too obliquely on the spine. Deformities of the palate and uvula, including high narrow arch and the torus palatinus. Anomalies of the teeth, tongue and lips. Anomalies of the ej^-es: narrow palpebral fissure, muscular insufficiency, excessive astigmatism, nystagmus. Anomalies of the ears: badly placed, ugly shapes, asymmetry, adherent or lobeless ears, markedly conchoidal ears. Anomalies of the limbs, genital organs and bodj^ generally. Anomalies of the skin, excessive hairiness or absence of hair. The most imporant of the anatomical stigmata are deviations in the symmetry and shape of the skull, defects in the palate and under- jaws, badly shaped ears, badly set teeth, badly set ribs and a generally weak and badly developed body. Stress is laid upon the skull because its development corresponds with that of the brain. The palatal stig- mata are in general those which make the cavity of the mouth smaller, it being the fact that the mouth cavity increases in size as we ascend the vertebrate series. Abnormal palates are found in about 10 per cent, of normal people (Charon) and in from 46 to 80 per cent, of abnormal. The high, narrow palate is one oftenest seen by myself. The torus pala- tinus or longitudinal ridge on the hard palate is significant if it is well marked (Fig. 21). The importance of defective ears is based upon comparative observations. They are found in from 20 to 64 per cent, of more or less abnormal persons. While many of the stigmata have no significance in themselves, yet the presence of a number of them is of great importance, for neuroses or psychoses developed among this class have a much more unfavorable 42 DISEASES OF THE NERVOUS SYSTEM prognosis. It is especially among neurasthenics, epileptics, severe forms of hysteria and in the insanities that these signs are to be looked for and studied. Among normal men about two or three anatomical stigmata are often found; among lunatics, criminals, abortive types of paranoia and primary forms of neurasthenia the number is much greater. The physiological stigmata include tremor, tics, nystagmus and hereditary defects in the muscular system leading to atrophies. Ex- cessive or defective sensibility of the cutaneous and special senses, de- fects in speech, perversions of the sexual and other instincts are to be classed here. A diminished resistance to nervous and emotional strain is a most frequent physiological mark of neuropathy. Allied to this is the excessive sensibility to or craving for the action of tea, coffee, tobacco and alcohol. The mental stigmata include all those factors that make up the erratic, unbalanced and morbidly emotional individual. The specially morbid note in these persons is an excessive egotism, an intense self- consciousness, often with peculiar disturbances of the sense of personality. Mental retardation is often an important factor in the examination of a nervous patient. This is especially true in epilepsy and in the psycho- neuroses, and in the neuroses of childhood. The Binet-Simon and certain adult tests for intelligence are to be employed here. Physical defects due to disturbance of the functions of the glands of inter7ial secretion may need investigation, and this would come in a measure under the head of physiognomy, and nutrition. 2. Investigation of Symptoms of Disordered Motility. — In studying the attitude, expression, gait and speech, some notion of the condition of the motor functions has been obtained. Special disturbances of the various parts must then be investigated. Paralysis.^TliQ patient is made to move the arms, legs, trunk, head, facial muscles, eyes and tongue, etc., in all possible ways. If paralysis is found the degree of it in some groups of muscles can be measured by dynamometers. The ordinary hand dynamometer measures the degree of paralysis in the flexors. The average power of pressure on the dynamometer is, for an adult, 40 to 50 kilograms for the right hand, and 3 to 5 kilograms less for the left. A woman has about two-thirds of the power of a man. A good idea of the degree of paralysis can be got by making the patient take the physician's two hands with his own and squeeze each at the same time. A malingerer or hysteric will often in this waj^ unconsciously press much harder than he is aware. The physician's own ingenuity will suggest various Avays of testing the strength of the leg and thigh muscles, such as making the patient rise on one toe, climb upon a chair, push against an object with his foot, etc. DIAGNOSIS AND METHODS OF EXAMINATION 43 t^ = ^ rt Taken around glabella and maximal occip- ital point. Measured from B over through bregma to B, or opposite ext. aud. meatus. o : CO o S "5 7th to 18th years 03 0) O Ph' r^ o f^' : 04 S' o CO o fe' o o s o »o d o ■s 2 !i; o o tH o CO o CO r5 o o (M CO lO CO ^ CD g 2i 2 .So r / ^ .— a o CI ^ bCi H aj OS o3 03 13 CO > 03 TT^ o oi ^ rt 5 .^ go Eh c30 C3 ^ 44 DISEASES OF THE NEKVOUS SYSTEM Some paralysis being discovered, we try to determine its degree and type. Paralyses are of four types: 1. An upper neuron type, in which the lesion involves the cerebro- spinal motor neurons. The paralysis is then as a rule hemiplegic, and accompanied with spasticity and exaggerated deep and lessened skin reflexes; it is not accompanied with atrophy of the muscles, involved or with degenerative electrical changes. 2. A lower neuron type, in which the lesion involves the anterior cornual cells or anterior roots of the spinal cord, or the corresponding parts of the brain stem (pons-medulla). The paralysis is localized in the muscles supplied by the injured neurons; it is flaccid and associated with atrophy and the electrical reactions of degeneration. 3. A neural, or mixed nerve type of paralysis, in which the symptoms are like those of a lower neuron type plus sensory symptoms, such as pain and anaesthesia. 4. A psychic type of paralysis, in which the paralysis depends upon a mental state. Here the paralysis may be hemiplegic, paraplegic or monoplegic but it is not accompanied with spasticity, or atrophy, and it is generally accompanied with anaesthesia. Tremor is usually most noticeable in the hands and is tested by making the patient hold out the hands and arms at full length, spreading out the fingers at the same time. To determine whether the tremor in- creases on volitional movement, give the patient a full glass of water, let him hold it out for a moment, then bring it to his mouth slowly. If the tremor increases with this movement it is called "intentional." As a general rule, the tremor of organic disease is coarse in type and increased by volitional movement, and ceases during rest of the extremity. Func- tional tremors are usually fine in type and continuous, except on complete rest and relaxation. In most forms of tremor the hand and arm shake as a whole. In other forms the tremor involves only the fingers or hand or forearm and hand. Such tremor is called segmental. It is especially seen in paralysis agitans. As I have already said, tremor may be fine or coarse, i.e., four to six or eight to twelve per second. To determine this accurately a special apparatus is needed; but one can with a little expe- rience determine this fairly well by observation alone. While coarse tremor as stated is usually a sign of organic disease or of paralysis agitans, it occurs also in hysteria and grave conditions of alcoholism. Tremor that is hardly observable by the eye can be felt by placing one's hand against the extended fingers of the patient. Tremor of the tongue and lips and facial muscles must be carefully looked for. It is tested by making the patient close the eyes tightly and show the teeth or protrude the tongue. Facial tremor if very marked usually indicates a serious condition of nervous exhaustion, alcoholic DIAGNOSIS AND METHODS OF EXAMINATION 45 poison, or some other toxaemia or paresis. Tremor of the whole head due to the neck muscles must be distinguished from secondary shaking of the head due to a tremor of the trunk. Finally, it is to be remembered that tremor is a rhythmical disturb- ance and it should be distinguished from the jerky irregular ataxic move- ments of cerebellar-mid-brain disease. These always cease entirely when the patient is lying on his back and making no movements. Nystagmus is a form of tremor indicating cerebellar or mid-brain disturbance. Choreic movements, tics, associated and forced movements, and con- vulsions are the expression of certain diseases and are described elsewhere. The Examination of the Reflexes. — These are of three kinds: (1) the superficial or skin, (2) the deep, (3j the visceral. 1. A skin or superficial reflex is produced by scratching, pinching or irritating the skin. The result is a contraction of the muscles supply- ing the parts near or under the irritation. The skin reflexes which can be ordinarily brought out are the anal, bulbo-cavernous, plantar, cre- masteric, epigastric, abdominal, scapular, palmar, and certain cranial reflexes. The anal reflex is brought out by scratching the perineum. It causes a contraction of the sphincter ani. The bulbo-cavernous reflex is brought out by placing a finger on the urethra just back of the scrotum, and pinching or pricking the glans penis. This causes a contraction of the bulbo-cavernous muscle. The plantar reflex is produced by tickling or scratching the soles of the feet. This causes usually, when carefully done, a slight flexion of the toes. In many cases there is, however, no response. In irritable persons and children there is a sudden dorsal flexion of the foot, and often a contraction of the inner hamstring muscles. In pathological condi- tions involving the pyramidal tracts of the cord and the motor centres and tracts in the brain there is a dorsal extension of the great toe, some- times accompanied with a fan-like spreading out of the other toes. This is called the Babinski reflex (Fig. 20). A similar phenomenon is pro- duced by firmly compressing the muscles of the calf (Gordon reflex) , and by causing the patient to draw his leg up sharply flexing the thigh on the abdomen and the leg on the thigh (Strumpell reflex) ; or by pressing and drawing a blunt instrument or the finger sharply down along the inner side of the tibia (Oppenheim reflex) or by scratching the leg behind the external malleolus (Chaddock reflex). The cremasteric reflex is brought out by scratching the inner side of the thigh or the skin over Scarpa's triangle. It causes a drawing up of the testicle, not of the scrotum alone, on the same side. The abdominal reflex consists of a contraction of the abdominal recti 46 DISEASES OF THE NERVOUS SYSTEM muscles, caused by irritating the skin over the outer border of the rectus. The epigastric reflex consists of a contraction of the upper fibres of the rectus, caused by irritating the sldn of the same region higher up. The scapular reflex consists of a contraction of scapular muscles, caused by irritating the skin over them. The palmar reflex is produced by irritating the palms of the hands. It is obtained only in infants and the reaction is flexion. The cranial reflexes are the corneal and conjunctival, a reflex contrac- tion of the lids caused by lightly touching the cornea or conjunctiva with a bit of cotton or a camel's-hair brush; the pupillary -skin reflex, which consists of a dilatation of the pupil caused by scratching the skin of the cheek and chin; the supra-orhital reflex caused by striking a slight blow over the supra-orbital foramen; the naso-mental reflex, in which a contraction of the levator mentis is caused by a tap on the side of the nose. Skin or Superficial Reflexes Superficial Reflexes Method of Exciting Effect Localization 1. The Supra- A tap over the supra- Orbicularis contracts V-Vll orbital. orbital foramen 2. Conjunctival re- Touching cornea, or Orbicularis contracts V-Vll flex. conjunctiva 3. Naso-mental.. . . Blow on side of nose Levator menti con- tracts V-Vll 4. Palatal Touching palate Palate contracts IX-X 5. Pharyngeal Touching posterior wall of pharynx Pharynx contracts IX 6. Skin-pupillary . . Pinching cheek Pupil dilates V-cervical sym- pathetic 7. Scapular Stimulation of the Movement of C5-D1 skin over the scapula shoulder-blade 8. Palmar Irritation of the palm Flexion of fingers C8-D1 9. Epigastric Stroking from nipple Drawing in of epi- D7-D9 downward gastrium 10. Abdominal Stroking side of the Drawing in of ab- D9-D12 abdomen dominal wall 11. Cremasteric. . . . Stroking the ad- ductor region of the thigh Elevation of testis L1-L2 12. Plantar reflex. . . Stroking the sole Dorsal or plantar flexion of toes S1-S2 13. Bulbo - caverno - Pinching dorsum of Bulbous urethra con- S3-S4 sus. glans penis tracts 14. Anal reflex Pricking the peri- Contraction of sphinc- S5 neum ter ani externus DIAGNOSIS AND METHODS OF EXAMINATION 47 There are also palatal and pharyngeal reflexes. The superficial reflexes depend upon the integrity of the reflex spinal arc, and to a less extent upon the degree of cerebral influence. When present, they show that the spinal cord at the level through which the impulses travel is healthy. When absent, they do not necessarily indicate much of any- thing, for they vary in amount in different persons and at different ages being always more active in the young. In cerebral hemiplegia during and after the acute attack the abdominal reflexes are absent on the affected side. This reflex is also often absent in multiple sclerosis. The supra-orbital reflex is often absent in profound hemiplegia with coma. The deep reflexes are sometimes called tendon reflexes, though this' is not a strictly correct name, since they can be called out by striking perios- teum or muscle as well as tendons. The deep reflex in all these cases is not a true spinal reflex, but is due to the direct effect of the concussion or the sudden stretching upon the muscle itself which is in a condition of slight tonus. Those who accept this view speak of the deep reflex as indicating the myotatic irritability or muscular tonus. The deep reflex implies also the integrity of a reflex arc. The important deep reflexes are the patella-tendon reflex or knee-jerk; the ankle reflex or ankle-jerk; the biceps, supinator, pronator and triceps reflexes ; the scapulo-humeral reflex; the jaw reflex or chin-jerk; the light (or pupil- lary) and so-called accommodation (or ciliary) reflexes; the oculo-car- diac reflex. These are all present in health except the jaw-jerk. The reflexes are of diagnostic importance, for they are absent in some diseases, exaggerated in others, and in certain conditions new reflexes appear. These abnormal reflexes are the Babinski reflex, the Hoffman reflex, ankle clonus and certain pupillary reflexes. The patella reflex or knee-jerk consists of a sudden contraction of the quadriceps femoris, vastus internus, and subcrureus caused by striking the patella tendon when the leg hangs loosely at right angles with the thigh. This reflex may also often be produced by striking Fig. 22. — Getting the knee-jerk. 48 DISEASES OF THE NERVOUS SYSTEM the lower part of the muscle itself. The activity of this reflex is Id- creased if, at the same time that the blow is struck, a voluntary con- traction of some other muscles is made by the patient. Usually the patient is told to pull on his clasped fingers or tightly shut the hands. This process is called the re-enforcement of the knee-jerk (see Fig. 22). Such re-enforcement can be caused by irritating the skin and by various sensory or psychic stimuli. The nerve-roots involved are those, in man, of the second and third lumbar segments. The peripheral nerve is the anterior crural. The essential muscle is the vastus internus. The biceps reflex, a supinator reflex, and a pronator reflex are said by Babinski to be always present in normal persons. The first two are obtained by striking the lower tendon of the partly flexed biceps and the lower third of the supinator longus; the pronator reflex is obtained by striking the lower head of the ulnar bone. The triceps reflex or elbow-jerk is brought out by striking the triceps tendon while the arm is supported and the forearm allowed to hang down loosely at right angles to the arm. These reflexes occur in normal individuals. Deep Reflexes Tendon, Bone or Deep Reflexes Method of Exciting Effect Localization 1. Pupillary Expose to light; and Pupil responds to 1-111 ciliary gan- reflexes test accommodation to light, and glion accommodation Pons 2. Jaw reflex Tapping lower jaw which is relaxed half open Jaw closes V 3. Biceps Tapping biceps tendon Biceps contracts C5-C6 4. Supinator longus Tapping styloid pro- Supinator longus con- C5-C6 cess of radius tracts 5. Scapulo- Tapping vertebral Teres minor, infra- C5-C6 humeral border of scapula spinatus, etc., con- near base tract 6. Pronator Tapping head of ulnar Pronation C6-C8 7. Triceps Tapping triceps tendon Triceps contracts C7-Thl 8. Carpo- Tapping wrist Fingers flex C8-Thl metacarpal 9. Knee Tapping patellar Vastus internus, etc.. L3-L4 tendon contract 10. Ankle Tapping tendo- Achillis Calf muscles contract S1-S2 The jaw reflex or jaw-jerk is brought out by having the patient open the mouth and leave the jaw relaxed. A flat instrument Hke a paper- DIAGNOSIS AND METHODS OF EXAMINATION 49 cutter is then laid on the teeth of the lower jaw, and if this is struck smartly the elevators of the jaw contract. The light reflex is tested bj^ throwing a bright light into the eye; and the ciliary or accommodation response by making the patient look at a distant and then at a near object. The pupil normally dilates in the former case and contracts in the latter. When the light reflex is lost while the accommodation action remains, the condition is called the Argyll-Rohertson pupil. The reflex path is probably through the optic nerve, the primary optic centers, third nerve, ciliary ganglion and ciliary nerves. The oculo-cardiac reflex is produced by pressing firmly on the ball of one or both eyes. This causes a slowing of the heart beat. The reflex is usually absent in locomotor ataxia. Fig. 23. — Testing pupillarj^ reflexes, before an open window. The Hoffman reflex. — ^A sharp snap of the last phalanx of the middle finger causes flexion of the index and thumb. Its presence generally indicates a pyramidal tract lesion. Ankle clonus is caused by having the seated patient extend the limb and hold it rather firmly in a semiflexed condition. The physician takes the foot by the toe and heel and quickly flexes the foot on the leg. He thus suddenly stretches the calf muscles, and they undergo rhythmical contraction. This phenomenon does not occur in healthy people. It is found in involvement of the cortico-spinal tracts and it usually indicates organic disease of the cord. A pseudo-clonus sometimes occurs in which there are a few rhythmical contractions on sudden dorsal flexion of the foot, but the contractions soon subside. This is seen in exhaustion and toxic states and in hysteria. The deep reflexes may be decreased, delayed, absent, or exaggerated. Their exaggeration is common and not of special clinical significance. 50 DISEASES OF THE NEKVOUS SYSTEM The absence of the knee-jerk or ankle-jerk is of great significance, indi- cating in persons who have no paralysis of the muscles, locomotor ataxia, nem-itis or some toxaemia, such as follows diphtheria or exists in diabetes. The Electrical Conditions in Disturbances of Motility. — These can- not be understood without some description of the methods of using electricity, and hence the technic of electrical examinations for purposes of diagnosis will be described under the head of treatment. Examination of the Disorders of Sensation. — The object of examining the sensory functions is to see if they are exaggerated, perverted, delayed or lost, and to locate the extent of the disturbance. Patients differ greatly in their intelligence and power of description, so that great care must be taken in drawing conclusions as to sensory disturbances. In examin- ing the skin and muscle-senses, the patient's eyes should be closed and he should be carefully told to answer promptly whenever he feels the stimulus. It is best to insist that he always reply in the same way, e.g., using the word ''now" the moment the sensation is felt. Many ingenious instruments have been de- vised, and I have described some of them, but for ordinary purposes a camel's-hair pencil or a bit of cotton wool and a pin answer very well. Loss of cutaneous and deep sen- sibility produces anaesthesias and these anaesthesias like paralyses have different characters in accord- ance with the series of neurons in- volved. Anaesthesias, cutaneous and deep, may like paralyses be of different types in accordance with the part involved. 1. Upper neuron anaesthesias. If the upper sensory neiu'ons of the cerebrum are affected the anaesthesia is never abslutely complete, and it is associated usuallj^ with a hemiplegia. 2. Middle neuron anaesthesias. If a lesion lies in sensory neurons of the cord or brain-stem the anaesthesia may be of a dissociated type; i.e., there may be an anaesthesia to pain and temperature, but not to touch. ■ 3. Peripheral neuron anaesthesias may be complete, or have various de- grees of completeness, but they are not dissociated. Protopathic and Fig. 24. — Testing for ankle-jerk. DIAGNOSIS AND METHODS OF EXAMINATION 51 epicritic variations of cutaneous anaesthesia are all due to lower neuron and peripheral nerve involvement. 4. Psychic anaesthesias are rarely absolute. They involve the entire half of the body, or the entire body or one limb, and affect the special senses in a definite manner (see hysteria). The cutaneous sensations are: (1) The tactile sense, which includes pressure and contact; (2) the temperature sense, which includes the heat sense and cold sense; (3) the pain sense. The first two are special senses, the last is a general sense. To test the tactile sense, blindfold the patient and use the aesthe- siometer. This is an instrument with two rather blunt points, which Fig. 25. — Bringing out ankle clonus. can be separated or approximated. A hairpin or two ordinary pins can be used in its stead. Its use depends upon the fact that the power to appreciate the contact of two points on the skin gradually approxi- mated varies with the tactile sensibility of the patient. The tongue, finger tips and lips are the most sensitive points. The back, arms and thighs the least sensitive. The following table shows the average distance at which two points are appreciated as such by an intelligent adult : Tip of Tongue 1 mm. (V^s in.). Tip of toes, cheeks, eyelids ... 12 mm. Tip of fingers 2 mm. Temple 13 mm. Lips 3 mm. Back of hands 30 mm. Dorsal surface of fingers. . . 6 mm. Neck 35 mm. Tip of nose 8 mm. Foi-earm, leg, back of foot. ... 40 mm. Forearm 9 mm. Back . .60-80 mm. Arm and thigh 80 mm . The figures vary somewhat with the thickness or softness of the 52 DISEASES OF THE NERVOUS SYSTEM skin and with the dullness or keenness of the nervous organization. If the distances are double those given above, it may be considered in most cases abnormal. The sense of contact, which is a form of tactile sense, is tested by touching the skin very lightly with a hair or hair brush or bit of cotton. The sense of locality or power to localize a point on the skin that has been touched varies with the tactile sense and with the muscular sense. It is tested by placing the finger lightly on a given spot and telling the patient with closed eyes to place his finger on the part touched. He should come within 5 cm. In slight degrees of anaesthesia dependent upon disease of the sensori-motor areas of the cortex of the brain this is an important test. Further tests may be made by moving points along the skin and taking the patient to indicate the direction of the motion. To test the pressure sense, one may use the barsesthesiometer, an instrument made with a spring scale measuring the amount of pres- sure made. A simpler way is to have the patient rest the hands on a table and then try and determine the weight of different objects. The lightest weight that can be appreciated on the hands or face is one of about 0.02 gram (gr. ^i). Differences of light weights of 1 and 5 grams and of 25 and 30 grams are about all that can be ordinarily appreciated by the skin. Much smaller differences, of 0.5 to 2 grams, can be detected if great care is used. Weighted rubber balls may be used in the fore- going test. I prefer to use differently weighted metal bodies held by a wire. Pressure sense is acute on the forearm and abdomen, where locality sense is feeble; also on the brow, temples and back of the hand. By the use of these tests held freely in the hand we test also the musculo- articular or deep sensibility. The tem'perature sense is tested by test-tubes filled with hot and cold water, or by using hot and cold spoons, or roughly by breathing and then blowing on the part. A thermo-aesthesiometer may be used. This has a round, flat surface 1 cm. in diameter, and contains in its terminals thermometers by which the degree and differences in temperature may be noted. A small heated or chilled surface is appre- ciated much less easily than a large one. The indifferent range where objects are felt to be neither warm nor cold is from 27° to 30°C. (80.6° to 86°F.). Fine differences (0.2° to 1.5°C.) are appreciated above the indifferent range. Lower down in the scale, differences from 1° to 1.3°C. (2° to 3°F.) are appreciable. It may be considered a morbid symptom if temperatures of 60° to 65°F. are not felt as cold, or temperatures of 86° to 95°F, are not felt as warm; also ff between the ranges of 1°C. (32°F.) and 40°C. (104°F.) differences of 2°C. are not appreciated. A painful degree of sensitivenessto heat DIAGNOSIS AND METHODS OF EXAMINATION 53 Fig. 26. — Showing the segmental or root distribution of cutaneous sensory nerves on the right side, and the peripheral distribution on the left side. (Drawn by H. T.. Shannon.) 54 DISEASES OF THE NERVOUS SYSTEM Fig. 27. — Showing the segmental or root distribution of cutaneous sensory nerves on the left side, and the peripheral distribution on the right side. (Drawn by H. T. Shannon.) DIAGNOSIS AND METHODS OF EXAMINATION 00 or cold sometimes exists. These conditions are called hyperthermalgesia and hypercyalgesia (Skinner, Starr). When the heat or cold is intense, a sensation of pain is felt. Cold pain is produced more easily in some places, such as the elbow, than in others, as for example, in the finger tips. Cold pain is produced by temperatures of from + 2.8°C. to— 11.4^C. Heat pain is produced by temperatures of from 36.3°C. to 52.6°C. The 'pain sense is tested by pricking the skin with needles or the sharp points of an sesthesiometer. The faradic battery with metal points or a wire brush may also be used. Instruments for pinching the skin and measuring the sensibility by the strength of the pinch have been devised. The power of localizing pain is lessened in propor- tion to the analgesia. Pain and temperature sense are usually affected together. The 'paiii sensation of muscles, is tested by passing the faradic current through the part or by deep hard pressure into the muscle. The epicritic and protopathic sensibility as described under symptoms are tested practically by cotton-wool giving light-contact sense and Fig. 28. — Author's sesthesiometer. pin pricks giving sharp pain sense. The tests give an indication of the degree and location of peripheral nerve injury (Head). The vibration sense is tested by placing a low-pitched tuning-fork upon the bony prominences. The feeling of vibration thus communi- cated varies about as does the tactile sense, but it is said to be lost in diseases in which the spinal roots are involved, such as tabes and in serious transverse lesions of the cord. The tactile sense, as well as the other special and the general sensa- tions, may show a delay in conduction. The tactile and pain sense es- pecially should be tested on this point. The delay may amount to several seconds. It is especially characeristic of tabes dor sails. The stereognostic sense is tested by placing variously shaped objects in the patient's hands and asking him to name them. It depends upon the integrity of the tactile or of the deep, i.e., musculo-articular, sen- sibility or upon that of the stereognostic centre in the parietal lobe. The normal nerve-supply of the skin is shown in Figs. 26 and 27. Tests for Deep Sensibility. — Anaesthesia of the special sensory nerves of the muscles, joints and tendons causes ataxia and inco-ordination. Muscle anaesthesia causes chiefly a loss of weight sense or loss of power to determine weights. It is tested by the use of weights suspended by a 56 DISEASES OF THE NERVOUS SYSTEM string so as to exclude pressure sense ; also by causing the patient to squeeze a dynamometer up to a certain fixed number. In articular and tendinous anaesthesia there is loss of posture sense. It is tested bj^ the physician's moving the patient's limbs and having the blindfolded patient tell in what direction the movement is made. Or he is told to follow with one limb the movements which the examiner makes with the other, or the patient shuts his eyes and tries to put the tip of his finger to the tip of his nose (finger-nose -test) or the heel of one foot on the patella of the other leg (heel-knee test). Muscular, articular and tendinous anaesthesia usually exist together; Such ataxia shows itself in standing and in locomotion and other voluntary movements. Thus we have a static ataxia and locomotor or motor ataxia. Static ataxia, or inability to stand (or sit) without swaying or irregular movements, is tested by making the patient stand with the eyes closed and the heels and toes close together. Normally, the head moves not over an inch in this position, and the patient holds the head and body more rigid with the eyes closed than with them opened. In ataxic states the reverse is true, and decided swaying or even complete loss of equilibrium occurs with the eyes closed, or even with the eyes open, and the base narrowed by putting the feet together. This phenomenon is called the "Brauch-Romberg symptom." In static ataxia, muscular and articular sensations are both involved. Ataxia of motion is tested by observing the gait and the movements of the extremities. The patient cannot walk a straight line and cannot walk without watching the floor with the eyes. The arms cannot be moved in a co-ordinate way. With the eyes closed, the patient cannot place the finger on the tip of the nose or lobe of the ear or any indicated spot. Ataxia of motion involves especially the articular and tendinous sensations, but not these exclusively. It may be measured by noting how close in walking the patient keeps upon a given line 10 feet long; how near he can place the finger upon a centre of a board marked like a target. The patient is placed 10 feet away and made to walk directly at it and place the finger in the centre. Hypotonia or loss of muscular tonus is tested by having the patient lie on a couch. The physician takes the foot and lifts the leg by it, the knee being kept stiff. Ordinarily the leg cannot be carried quite to a right angle without the knee bending. If it does go to a right angle or beyond, there is hypotonia of the limb. The fingers, wrists, toes, ankles, knees show also abnormal flexibility. This condition is natural to some extent in children and it is sometimes a physiological characteristic of the adult. Cerebellar and Vestibular ataxia or asynergy.- — Ataxia of gait and station may be caused by lesions of the cerebellum and its connecting paths, DIAGNOSIS AND METHOD OF EXAMINATION 57 and of the vestibular nerve. These impair the apparatus of equihbrium and cause uncertain, jerky, staggering movements resemljling some- what the ataxia above described. The co-ordination of the arms is also involved. The tests and description of cerebellar and vestibular asynergy will be given under the description of cerebellar disease. Besides the foregoing methods of examination and testing for nervous conditions, there are a number of epinomic tests which are of impor- tance in investigating certain special neuroses. I have put the de- scription of these tests in connection with the disease to which they are related; but the following list of the tests collated for me by Dr. H. W. Frinck is given here as a matter of convenience in reference: Abadie's Sign (of Tabes) Mendel-Bechterew Test (of Vy. Tract Babinski's Test (of Chronic Paralysis) lesion) Barany's Test (of the Labyrinthine Reflex) Moebius's Sign (of Exophthalmic Goitre) Biernacki's Test (of Tabes) Quinquard's Sign (of Chronic Alcoholism) Brissaud's Test (of Organic Paralysis) Rinne's Test (of Hearing) Brudzinski's Neck Sign (of Meningitis) Romberg's Sign (of Ataxia) Chvostek's Sign (of Tetany) Rosenbach's Sign (of Hemiplegia) Erb's Sign (of Tetany) Schwabach's Test (of Nerve Deafness) Grasset's Test (of Hemiplegia), Stellwag's Sign (of Exophthalmic Goitre) Hoover's Sign of (Hemiplegia) Trousseau's Sign (of Tetany) Kernig's Sign (of Meningitis) Von Graefe's Sign (of Exophthalmic Mannkopf's Sign (of Simulated Tender- Goitre) ness) Weber's Test (of Hearing) McEwen's Sign (of Hydrocephalus) Wernicke's Hemiopic Pupillary Reaction Sense of Vision, Hearing, Vestibular Sense, Smell and of Taste. — See Cranial Nerves. Examinations of the X-ray are useful in determining the presence of fractures, bony growths, atrophies and deformities. Occasionally the X-ray photograph helps in the recognition of brain tumors. Blood examinations are necessary in acute inflammatory conditions, syphilis, pernicious anaemia, etc. It is hardly necessary to speak of the need of tests of the urine or of those which inform us regarding condi- tions in the gastro-intestinal tract. In many forms of nervous diseases every organ and fluid of the body must be examined so that the laboratory is an especially important aid to neurological diagnosis. Examination of the Cerebros'pinal Fluid. — The cerebrospinal fluid is normally a clear liquid with a specific gravity of 1006 to 1008. It is alkaline in reaction and contains a trace of serum-globulin and a sub- stance which reduces Fehling's solution. There may be one or two lymphoid and epithelial cells. In certain nervous diseases, such as tabes, spinal syphilis, paresis and in inflammatory diseases, various changes are found. The special characteristics are referred to under the different diseases. 58 DISEASES OF THE NERVOUS SYSTEM The technic of spinal puncture as illustrated and described for me by Dr. MacRoberts of the New York Neurological Institute is as follows: The cerebrospinal fluid can be obtained in a safe and simple manner by introducing a long hollow needle equipped with a stylet through the dura mater in the lumbar region of the spine. The needle is specially constructed for this purpose. The Hastings needle, the one generally used, is of strong flexible steel of fine bore with a handle large enough to allow of firm grasp. The needle should be boiled and the operation carried out under sterile precautions. The patient is best seated on a chair or the edge of his bed with his back rounded toward the operator. If weak or unable to sit up he may Fig. 29. — Spinal puncture. The needle is inserted along the lower edge of a spinous process marked by the tip of the thumb. lie on his side with knees drawn up toward his chest. In cases of brain tumor or of greatly increased intracranial pressure the patient should be on his side. In cases of posterior fossa tumor or marked distention of the ventricles the puncture can be performed with comparative safety if the foot of the bed be first elevated with shock blocks and allowed to remain in this position for eight hours or more after the withdrawal of the fluid. It has been found advisable to tell the patient in a few simple words what is about to be done, saying that he may expect the pain of a pin prick as the needle goes through the skin, but if he then keep very still the remainder of the procedure can be completed in a few moments without causing him further pain. DIAGNOSIS AND METHODS OF EXAMINATION 59 The point of election for the insertion of the needle is between the third and fourth or fourth and fifth lumbar vertebrae, in the median line or slightly to one side. The location is best obtained by making, with an applicator dipped in tincture of iodine, a straight line which unites the iliac crests. Where this line crosses the line of the spinous processes a space between the spines will be found by palpation with the thumb. The skin about this point should be sterilized with alcohol and tincture of iodine. The thumb of the left hand should be placed on the spinous process above the interspinous space with the edge of the thumb even with the lower edge of the bone. The needle should be grasped firmly Fig. 30. — Spinal puncture. Showing tlie position of the patient and the position of operator's thumb palpating the lower edge of a spinous process. with the base against the palm and the shaft steadied with the fingers, and the point placed just below the thumb edge and thrust smartly through the skin. It may then be more slowly pushed through the ligamentous structures until by the change in resistance it can be felt to have pierced the dura mater. The stylet should then be withdrawn and 4 or 5 cc. of the fluid collected in a sterile test-tube. The needle is then carefully but quickly removed and a small square of gauze fastened over the puncture point with adhesive. The fluid should be corked and placed in the ice box and should be examined some time within the next twenty-four hours. Following the puncture it has been the custom to have the patient lie flat on his back for twenty-four hours. It has been found recently that, by allowing our patients up sooner, fewer headaches resulted. The fluid is later tested for blood, excess of globulin, the Wassermann reaction, the number and character of cells, cholin, sugar, and bacteria. The special methods and indications are given under the description of meningitis, syphilis, cerebral hemorrhage and cerebral abscess, and cord tumors. CHAPTER VI HYGIENE, PROPHYLAXIS, TREATMENT In the treatment of nervous disease, the physician attempts to relieve distressing symptoms, to secure radical cure, and to prevent return. This calls for various measures which may be classed under the heads of general hygiene, diet, exercise, climate, hydrotherapy, mas- sage in various forms, electricity, drugs, external applications and surgical intervention. General Hygiene. — To secure and keep steady nerves and to pre- vent the supervention of organic nervous disease, would require a considerable reconstruction of the present social system. The struggle for existence and success is keener than it used to be. There is a smaller field for the slightly backward class, and more men fail in life and become parasitical than formerly when there was a wider field for the simpler manual occupations. I can only give some hints as to the kind of advice physicians should give to help along those who are very likely to be handicapped by a neuropathic constitution. Thus two people of very nervous tempera- ment or bad stock should not marry. Blood relations of the same tem- perament should not marry, and families with a psychopathic taint should not intermarry. Children should be brought up to eat slowly a mixed diet, to sleep early and long, to play in the open air, to learn self-control and obedience. Their parents should keep from them all infective fevers. Systematic study and work are good for all children. It is the strain due to defective vision, poor light and ventilation and unsuitable tasks that hurts the neurotic. Education and occupation are the best kind of builders up of healthy nerves. There are children, however, who cannot follow the ordinary educational lines and who must be specially trained in consequence. The queer and eccentric children with some twist, or precocious talent, need especial care. They usually must be brought up to follow lives on a low mental plane. Too many good farmers and artisans are spoiled by being made poor professional men, or being set up in responsible business positions. Adults need to keep in mind especially: moderation, exercise and the avoidance of a luetic infection. With these most need not fear the use of alcohol, tobacco, tea, coffee, or even occasional irregularities in sleeping and eating. Phys- ical and mental strains, infective fevers and toxins are prolific pro- 60 HYGIENE, PROPHYLAXIS, TREATMENT 61 moters of nervous disease. Syphilis stands out as the most important single factor in producing organic nervous diseases. If it could be re- moved we would have no locomotor ataxia, or paresis, less myelitis, and far fewer cases of apoplexy. Alcohol is a less important factor, but does much to produce mental disease, vascular disease, and hereditary degeneration. Diet. — There is no definite formula for diet in nervous diseases. The kinds of foods which are suitable for infants and children, and growing youths, are not equally suitable for the mature or the senile. There are some general facts, however, which may be laid down, re- garding the diet for the nervous; that is to say, for persons who have a more or less neurotic and unstable constitution. This class of per- sons seems to be, as a rule, unable to tolerate sweets or large amounts of flesh food, and they do best upon a mixed diet which contains a moderate amount of proteid together with vegetables and fruit. The question, whether neurotic patients do better upon the so-called "high" proteid of flesh or the "low" proteid of vegetables, does not seem yet to be settled, though the practice is, on the whole, rather more in favor of using the flesh proteid than the vegetable proteid, especially in the early half of life. There are four kinds of what may be called general diets prescribed for patients. I do not mean to include in these four, however, the special diets that are used in the acute and organic diseases, such as diabetes and fevers, serious gastric disturbances, and so on. These four diets are : first, vegetarianism ; second, the modified vege- tarian or purin-free diet; third, the Salisbury high-proteid diet, and, fourth, the Chittenden or "low diet." The pure vegetarian diet is one which has no scientific basis, and which seems to me, on the whole, as unwise, though it apparently suits certain constitutions, and is, if anywhere, especially suitable for those of rather advanced years. The purin-free diet is vegetarianism plus the use of milk and cheese. In this, there is a reduction of the proteicls, but an increase somewhat, of the fats and carbohydrates. The Salisbury diet consists of lean beef and hot water, and its use involves a large increase of the proteids and of the purin-bodies, while there is a decrease of the fats and carbohydrates. This diet is usually somewhat modified by the addition of bread or toast, and sometimes of fruits, so that the diminution in the carbohydrates is not so marked. The Chittenden diet is one in which the food, as a whole, is cut down in all its forms, the proteid being reduced nearly one-half. Ex- perience has shown, that all these different diets may lead to the same therapeutic results — that is to say, that a purin-free diet will cure a mi- 62 DISEASES OF THE NERVOUS SYSTEM graine or lessen the attacks, and that a Sahsbury or meat diet may do the same. The injmy caused by any form of diet is largely due to the fact that putrefactive changes occur in it, leading to toxaemias, or that there gets into the blood an excess of food or fuel; and if this diet is modified so that such an excess is lessened, without weakening the system, we can secure good results. In fine, the main factor in prescribing any diet, is to see that it is a digestive and assimilable one, and that there does not get into the system an excess of fuel, producing what is termed a ''hyperpyrsemia." In elderly persons the amount of nitrogenous food needed in the form of meat is usually less; and they often do well on a vegetarian diet. As a rule, however, vegetarians after a few years find that a return to some carnivorous food is indicated. Some neurotic persons seem to need a great deal of food but, as a rule, harm comes from full diets and one cannot get strong by stuffing. The frankly nervous, the hypomanic and psychasthenic patients should not use alcohol at all. Tea and coffee can be taken in very small amount and best without sugar. The various alkaline mineral waters may be used temperately with impunity, but none of them have much specific effect in relieving nervousness or curing the nervous temperament. Exercise. — ^As a prophylactic against nervous disorders, the value of exercise, especially if taken out of doors, can hardly be overestimated. Brain workers are better for moderate exercise, but they do not need much; and after twenty-five, severe intellectual work can rarely be done by persons in athletic training. Before the age of twenty-five, when the system is exuberant with vitality, hard study and hard physical exercise can be pursued successfully'- together by some. Persons of a neuropathic constitution are most benefited by regular exercise when it interests the mind. Indoor gymnasium exercise with the ordinary apparatus does little good except through the bath that follows it. In many forms of chronic organic nervous disease, exercise is to be prohibited. The most important line of effort in prophylaxis is the warding off of excessive or premature arteriosclerosis. The surest preventive of this is a good heredity. But, given an average constitution, one can delay arterial degeneration only by leading a perfect life, that is to say, the in- dividual must avoid all mental and physical strain and shock, alcohol, excesses in eating and tobacco. He must not get syphilis. He must keep the emunctories open and get as much as possible of fresh air. All of which is the council of perfection, except for children. Here wise parents can do much to prevent the development of arterio-sclerotic neuroses. Hydrotherapy. — Hydrotherapy is the science of applying water in the treatment of disease. The modes by which it is used in neurological therapeutics are: HYGIENE, PROPHYLAXIS, TREATMENT 63 I. General hydrotherapy: 1. Tonic hydrotherapy. 2. Sedative hydrotherapy. 3. Indifferent baths for mechanical purposes. II. Local hydrotherapy. The details of the use of these measures are now eml)odied in special treatises and works on therapeutics. Massage. — -The term massage may be made to include all the manipu- lations of the body for the purpose of curing disease. The different methods of applying it as classified by Jacoby are : Effleurage or gentle stroking. The maximum force to be applied here should not exceed the weight of the hand. Massage a friction or rubbing. Energetic strokes with one hand and strong circular or to- and-fro friction with the other. Petrissage or kneading. Tapoiement or percussion with the fingers, hands, or instruments. Frictional move- ments, passive, active and combined with movements are made by the operator. The physician may be reminded that a male operator is a masseur, a female a masseuse, and that the patient is massed. Massage accelerates the lymph and venous currents, and thus pro- motes absorption. It increases at least temporarily the number of red blood-cells (Mitchell). It increases the rapidity and force of the heart beat (except abdominal massage which slows the heart) and helps to relieve local congestions and inflammatory deposits. It presses and stretches the terminal nerve filaments, increases the irritability of motor nerves and the contractility of muscles. It may either increase or lessen the irritability of sensory nerves according as it is applied. Of the various forms of massage, tapotement is frequently useful and is the kind often used in neuralgias. It is applied not only with the fingers and hand, but also by the aid of rubber tubes known as muscle beaters, rubber balls with rattan or whalebone handles, percussion hammers and various percuteurs. Massage is of considerable value in certain forms of atonic neuras- thenia and hysteria associated with anaemia, dyspepsia and feeble circu- lation; in hemiplegia, in the paralyses of peripheral origin, in functional spasm, especially in some forms of writer's cramp and allied neuroses, in cerebral hypersemia, insomnia, constipation and in headache and some neuralgias, especially those about the head, neck, and arm. It is con- traindicated usually in heart disease, in advanced arteritis or when there is danger of dislodging a thrombus. Regular educational muscular movements according to a certain fixed schedule are used in the treatment of locomotor ataxia and paralysis. The details of these exercises will be given later. 64 DISEASES OF THE NERVOUS SYSTEM Osteopathy. — ''A system of therapeutics based upon the theory that many diseases are due to pressure upon the vessels or nerves by some dis- placed vertebra or other part of the skeleton, or to a condition of imbalance of the muscles moving any joint; the treatment is directed to the me- chanical correction, by means of manipulation, of the assumed osseous displacement or muscular imbalance, with the consequent repression of the abnormal reflexes and a restoration to normal of the circulation and the nerve impulses" (Stedman). It is a form of treatment which gets results, according to my experience partly by suggestion and partly by improving mechanical conditions, as does massage. It is dangerous in certain forms of nervous disease like tabes. It is useless in others like Parkinson's disease. It is dangerous also, like all special therapeutic systems, when it makes large and unfounded claims. Climate in Nervous Diseases. — The factors which make up a special kind of climate are: Purity of air; temperature; humidity; sunlight; rarefaction of air; ozone; wind; electricity; soil; trees, social conditions. Regarding these points, some facts are very well settled. The air in the country is purer than in cities. The air on the sea and at high levels is purer than in other localities. The temperature above the sea level chminishes about 1°F. for every 300 to 350 feet, and is less the dryer the air. Alterations in temperature are less near the sea and less in the southern hemisphere. The higher the elevation and the colder the air, the less moisture does it contain. About the factors of ozone and elec- tricity in the air little definite is known. As to sunlight, an excessive amount of it is said eventually to enervate patients. Climates are classified by Weber into marine, low-level inland and high-level inland. These all have great variations in quality, depend- ing upon their temperature, moisture, etc. As a general rule, warm marine climates and sea voyages are best for neurasthenic invalids of the irritable type. On the other hand, in atonic and anaemic con- ditions high inland climates are better, at least for a time. Such climates should not be too dry or windy. In organic degenerative diseases of the nervous system, marine climates and low levels are better. As a whole, it seems to be the con- clusion that plenty of fresh air is the essential in all climates and is better than any special climates. Relatively short changes from low to high levels or from temperate to tropic climates often leave a distinctly tonic effect. Germany, the Riviera, the Bermudas, the Azores, the West Indies, southern Colorado, Arizona and southern California are favorite places for sending neurasthenic Americans. Camp life in the Adirondacks or other forests is also found most useful. HYGIENE, PROPHYLAXIS, TREATMENT 65 ELECTRICITY IN NERVOUS DISEASES Technical Terms. — -There are certain technical terms which it is necessary to understand. Electromotive force (symbol, EMF) is the force which tends to set electricity in motion. An electric current results. The current strength (symbol, C) is the term used to express the capacity of the separated fluids to overcome resistance in their attempts to reach equilibrium or equalization again. This current strength, or simply the current, naturally is in proportion to the strength of the electromotive force, which is constantly dissociating the electrical fluids and generating the current. If, however, as is always the case, the electrical fluid meets resistance in seeking equilibrium, the resistance diminishes its current. Hence we have the formula known as Ohm's law: „ X X XI Electromotive force „ EMF. Current strength = ^ — ^- ; or L = — ^ — All bodies offer some resistance to electrical currents, and it is im- portant to have some standard unit of resistance for the sake of com- parison. Such standard unit has been adopted and is called an ohm. It is the resistance offered to a current by a certain piece of wire of definite size and length. A volt is the unit of electromotive force, i.e., it represents the force which will generate a certain amount of electricity in a second of time. A Daniell cell is not quite one volt strength. An ampere is the unit of working power or current strength. It is the current strength produced by one volt of electromotive force working against one ohm of resistance. A milliampere is one-thou- sandth of an ampere. A watt is the unit of work. When a given current flows along from a large into a small con- ductor, the quantity in this latter conductor in a given section is greater and the current is said to be denser. The instrument by which the strength of a current is measured is known as the amperemeter; in medical practice, only fractions of the ampere are used, and the in- strument is called the milliamperemeter. A rheostat is an instrument for interposing resistance in a current. Electrical Appliances. — -The batteries used in neurological practice are: the static, the faradic, the galvanic, and the high frequency. The current of the faradic battery varies in quality in accordance, (1) with the length and number of turns in the coil; (2) the form of the electrical wave; (3) the number of interruptions; and, (4) the strength of the battery. The currents from short coils of coarse wire have a lower potential and slightly more voltage. They are more eflicient in producing mus- 5 66 DISEASES OF THE NERVOUS SYSTEM cular contractions and are more irritating to the sensory nerves. These qualities are increased with slow interruptions of three or four per second. When muscular contractions and mechanical exercise with stimulation are desired, the short coils (primary or secondary) with slow interruptions are indicated. The current from long coils has a higher potential and less voltage; it has less power in contracting muscles and a different effect on the sensory nerves. When the interruptions are very rapid and the coil is very long, the effect on the sensory nerves seems more sedative; perhaps, in part, because of a change in the form of the electrical waves. The physiological effect of a current depends, as I have stated, in part upon the character of the wave of electrical force. If this is high and sharp, the stimulation is different from that produced by Fig. 31. — Author's electrode set. a wave which gradually rises to its height. D'Arsonval has devised an instrument for producing these blunt-topped waves, and at the same time reversing the current. The current is called sinusoidal. I am not aware of any special therapeutic results in neurology from this kind of current, though it is claimed that very slow wave-like currents affect better involuntary muscles. The Leduc current is a rapidly interrupted galvanic current, the period of duration of the current being one-tenth that of the intermission. It is claimed that when sufficiently powerful, 20 to 60 volts or more, it produces local anaesthesia and when passed through the head to produce general ansesthesia and sleep. Very rapidly interrupted and alternated currents of extremely high voltage are called high frequency currents. When passed through the entire body they lower blood pressure 5 to 10 points, and increase metabolism. When applied locally under the name of "violet-ray" curents they have an efficient counterirritant effect. HYGIENE, PROPHYLAXIS, TREATMENT 67 Galvanic Batteries. — -The ordinary accessories to tlie faradic and galvanic batteries are electrodes, rheostat and milliamperemeter. The electrodes needed for ordinary purposes are: An indifferent electrode measuring 5 cm. by 15 cm. A normal electrode, 10 sq. cm. A unit electrode, 1 sq. cm. A soft wire brush. Three handles: one 10 cm. and one 40 cm. long, one short handle with an interrupter. A milliamperemeter. A rheostat. (See Fig. 33.) Methods of Application. — Static electricity is applied for fifteen or twenty minutes daily or tri-weekly. For general tonic or sedative effects, sparks are drawn from all parts of the body except the face. In paralysis or spasm or pain, sparks are applied to the affected area. For headaches and cerebral parsesthesise the electrical breeze is very useful, but it must be strong. The faradic and galvanic currents are used for about the same time and intervals as the static. In some cases, however, the galvanic current should be given daily or even two or three times a day. As a rule, a course of electrical treatment should be continued for six to eight weeks, and then discontinued for a time. In general electrization, whether galvanic or faradic, the indifferent electrode is placed on the sternum, feet or back, and the other pole is carried over the limbs, trunk, neck and, if indicated, the head. In some cases, however, the two electrodes are applied together upon the different muscles of the body. In local electrization, the large elec- trode may be applied on an indifferent spot and the other applied to the affected limb or limbs, or the two electrodes may be used together on the same segment of muscles. There are special points at which the muscular contraction is most easily brought out. These are called the motor points. Electro-diagnosis. — When a motor nerve is cut off from its centre in the spinal cord, or when this centre itself is diseased, the nerve and later the muscle undergo a degeneration. As a result of this, their reac- tion to electrical currents is changed, and we get what is termed ''partial degeneration reactions" and "complete degeneration reactions," accord- ing to the degree of disturbance. These reactions are due mainly, if not wholly, to the degeneration in the terminal nerve-fibres and motor end- plates in the muscle. When the muscle alone is diseased, the reaction is not changed until very late. The change in irritability is due to the fact that as the nerve-fibre wastes it takes an electric current of comparatively long duration and considerable strength to stimulate it. The first effect is to cause it to lose its contractility or reaction to weak currents, then to extremely rapid, short currents like the static, then to the faradic, and last to the galvanic. Such change is known as the quantitative alteration in electric irritability. 68 DISEASES OF THE NERVOUS SYSTEM But besides this, the nerve and muscle are affected in a different way by the different poles of the galvanic battery. In normal nerve and muscle, a contraction is caused more readily by the negative pole than by the positive. But muscles with degenerated nerve supply sometimes respond as well or better to the positive pole. This forms what is called the qualitative or serial change in the irritability of the muscle. Finally, degenerated muscles respond more sluggishly than normal to the galvanic and faradic currents. The contraction, instead of being sharp and jerky, is sluggish and almost tetanic. This is called the modal change in irritability, and it is far the most important sign of muscular degeneration. The qualitative change is gotten only by placing the active elec- trode over the muscle, but the quantitative and modal changes may be gotten by placing the electrode over the nerve as well as over the muscle. In describing these changes, the following abbreviations are used: DeR = degeneration reaction. AnCC = anode or positive-pole closure contraction. CaCC = cathode or negative-pole closure contraction. AnOC = anode opening contraction. CaOC = cathode opening contraction. Te = tetanus. D = circuit is closed and current flowing. AnDTe = tetanic contraction while the positive pole is applied and the circuit closed. The sign > means greater than; <,less than. Thus AnCC < CaCC means anode closure contraction is greater than cathode closure contrac- tion. Degenerations in nerve do not occur except in lesions of the peripheral motor neuron and in very late stages of primary atrophy of muscles. Hence, when one finds degenerative reactions he can almost absolutely exclude disease of the brain, functional disease and primary disease of the muscle. The following rules may be formulated for testing for de- generation reactions : Use the faradic current first. The Faradic Current. — Use a secondary induction coil of wire 0.225 mm. in diam- eter and 800 m. long. The distance over which the coil moves is divided into a hun- dred parts. The strength of current is indicated by percentage or millimetres. Record the minimum necessary for muscular contraction, using the same elec- trodes and in the same way as in testing with galvanism. The Galvanic Current. — (1) Place the indifferent pole over the sternum and a 10 sq. cm. electrode over the muscle. (2) Pass the current for one minute. (3) Then find the minimum strength needed for a cathode closure contraction. (4) Then for an anode closure contraction. Repeat this test three times. (5) With a given cur- HYGIENE, PKOPHYLAXIS, TREATMENT 69 rent, note whether the cathode closure contraction is stronger than AnCC or other- wise. Test this three times. (6) Note the character of tlie contraction, if sharp or sluggish. (7) Test nerve in same way. The qualitative changes may be expressed by a formula like AnCC = or > CaCC; i.e., the positive-pole closure contraction is equal to or greater than the negative-pole closure contraction. Or, better, the minimum strength of current required to cause a contraction in the muscle is recorded for the positive pole and for the negative. Thus: AnCC 5 ma. or 8 cells. CaCC 4 ma. or 6 cells. Fig. 32. The following table and diagram (Fig. 32) show the diseases in which degeneration reactions may be expected: Table Showing the Lesion, its Results, the Names of the Diseases and THE Electrical Reactions Lesion of — Result Disease Electrical Reaction as to Qualitative 1 to 21/^. Paralysis, contractures. Hemiplegia from hemorrhage. Nerve: normal. Cortex to cord. Embolism, tumors, lateral sclerosis. Muscle: normal. 3, 4 and 5. Paralysis, degenerative Acute and chronic anterior Nerve: DeR. Cornua. atrophy of nerve and muscle. poliomyelitis. Muscle: DeR. 2 to 5 Paralysis, contractures. Amyotrophic lateral sclerosis. Nerve: normal Muscle: normal or partial 5 to 6. Degenerative atrophy, Progressive muscular atrophy, DeR. When disease is of muscle. bulbar paralysis. advanced. Later, degeneration of nerve. 70 DISEASES OF THE NERVOUS SYSTEM Table Showing the Lesion, its Results, the Names of the Diseases and THE Electric Reactions. — {Continued) Lesion of — I Result Disease Electrical Reaction as to Qualitative Nerve Paralysis, degenerative Neuritis, from wounds, tox- Muscle: DeR. atrophy of nerve and emia or pressure. muscle. Nerve: DeR. Muscle Wasting, paresis. Simple atrophy; primary or Nerve and muscle normal idiopathic myositis. Pseudo- until late in the disease. muscular hypertrophy; other types of primary myopathies. Rheumatic atrophy and pare- sis. It should be said, finally, that it is the sluggish contraction which is the most important element in showing degeneration; also that it is the muscle which should be tested most carefully, as only over it does one get the qualitative changes. Therapeutics. — ^Electricity is used as a counter-irritant and as a general mechanical tonic in states of muscular and nervous weakness. It is used in paralysis, spasm and pain, and for its supposed specific action in certain functional and organic diseases. . The faradic and static currents have a counter-irritating, stimulating and excito-reflex effect. The galvanic current has a sedative and anti- spasmodic effect. The high-frequency current promotes metabolism and temporarily reduces blood-pressure. The ''violet-ray" current acts mainly as a counter-irritant. Electrolytic, cauterizing, and cataphoric effects are also produced, but are rarely needed by the neurologist. A considerable portion of the effects of electricity are psychical, but they are not the less real or valuable. Radiant Energy in the form of X-rays, ultra-violet rays, radium emanations have been of more help to surgery than medicine in thera- peutics. The X-ray and radium are said to have some effect in neuritis and neuralgia, but the results are not uniform or as yet conclusive. The X-ray with the Coolidge tube and radium have some effect in destroying new-growths, and are being used in spinal-cord and cerebral tumors. PSYCHOTHERAPY Psychotherapy is a term applied to that form of healing in which mental influences are used to correct morbid processes. Psychotherapeutics has always been used in an irregular and casual way to cure disease. Modern therapeutics endeavors to formulate definite rules for its use and to indicate systematically the conditions for which it should be applied. HYGIENE, PROPHYLAXIS, TREATMENT 71 Hypnotism is the oldest and best known method. In hypnotic thera- peutics the patient is put into a partial or complete hypnosis. Sugges- tions and directions as to cure are then made, which the patient on coming out of his hypnosis follows. This method of treatment is uncertain and difficult and is of use only in a narrow field. It has been practically abandoned, though occasionally its use gives brilliant results, mainly in hysterical conditions, morbid habits of not long standing, and in the minor psychoses. It is more effective in the younger subjects. It is of no use in the serious psychoses. (See Hypnotism.) Suggestion with minor hypnosis is only a modification of the preceding method. Here the patient is simply persuaded into a quiet, receptive mood or into a slight degree of hypnotic trance, which is oftener really only a slightly somnolent state. Then suggestions are patiently and insistently repeated. This method is more simple and on the whole more useful than the first. It has to be repeated perhaps as often as a dozen times before the desired impression is secured. But it is fairly efficient in the pychasthenic, more particularly those with fixed ideas, morbid fears, attention pains and morbid melancholic moods. The treatment is most useful here also in cases that are not of very long dura- tion. It deserves a permanent place in neurological therapeutics, but it can only be applied by those who have a thorough knowledge of the patient, of the disease and, I may add, of themselves. It requires a steady and forceful and confident personality. Formally and strictly applied, it has not a wide field. In a simpler and common-sense way, however, the method can be used by any physician, and is used, no doubt, all the time, for it really consists in giving the patient the vital sense of proportion in his judgment of things. In many patients the harmful thoughts are not entirely subconscious. The patient knows them, but has not quite recognized their importance and the need of treating them sanely and definitely and of putting them in their right relation. The method of psychic analysis and reintegration. This is also called the method of Freud. It cannot be described in detail here. We assume that many of the morbid mental states and bodily perversions are de- pendent on the presence of a pernicious and perhaps long-forgotten sub- conscious activity. Below the field of ordinary conscious processes there is memory of some earlier event acting, unknown to the patient, upon his conscious mental states and inducing in him certain morbid ideas or emotional moods which cause his sickness. The operator, by studying and ques- tioning, by learning of his dreams, his ''running associations," by telling him to empty himself of his troubles in a kind of "mental catharsis," brings into consciousness certain subconscious states which relate to events of his past. Perhaps also he finds that this lurking idea or 72 DISEASES OF THE NERVOUS SYSTEM emotional state is doing mischief. He then confronts the patient with the facts, shows him what is the real cause of his obsession, persuades him of its unreasonableness or accustoms him to look upon it in a normal way. In other words, he brings the supposed subconscious cause of the trouble into consciousness, readjusts associations, and enables the patient to fight his trouble in the open. It is further claimed by the psycho-analyst that the symptoms from which the patient is suffering, the spasm, the obsession, the morbid fear, are really the expression of a subconscious desire or of unsatisfied in- fantile instincts, usually of a sexual origin. My views as to the merits of this doctrine are given in the chapter on psycho-neuroses. The Method of Re-education. — This may be called also the method of ''therapeutic talk." It is known as the method of Du Bois. It is really an application of the principles of suggestion and of readjustment, or re-education, used in the preceding methods. The patient has his condition explained to him, the aims of his physician are placed before him, the reasonableness of his cure, the unreasonableness of his mental state and of his point of view are persistently argued over. The patient is sometimes persuaded, sometimes bullied into health. All this is done, however, after first isolating the patient from everyone and clearing the ground entirely of exogenous irritation. The doctor, with the nurse, a necessary adjunct, has the field entirely to himself. Obedience is en- joined. It is, in fact, a rest and isolation cure, combined with forceful presentations of the reasonableness and possibilities of cure. No medi- cines are given, as a rule. This is a method now having much vogue, but it is only a modification of the Weir Mitchell cure, in which the 'psyche receives rather more dominantly the attention of the physician. In estimating the value of these cures it must be remembered that they are useful practically only in the psychasthenic and neurasthenic groups of patients. The mild recurrent melancholias so often mistaken for neurasthenia get well anyway. The melancholia of involution is little affected by these methods, nor are the major psychoses, if we ex- cept perhaps paranoia and early phases of such types of dementia prsecox as are largely of psychogenic origin. The early and abortive types of the anxiety psychosis may also be affected. Empirical Psychotherapy. — Therapeutic results are obtained by man- datory methods, by the method of extravagant promise or absolute assur- ance. These are the methods of the parent and guardian and of the charlatan and patent medicine label. Their success is more than counter- balanced by the evil done by inconsiderate assurance in improper cases, and by misleading persons to the use of measures not in the least indi- cated by their condition. The employment of psychotherapy in conjunction with religious HYGIENE, PROPHYLAXIS, TREATMENT 73 work has recently been advocated and in some cities successfully carried out. A general indorsement of this union of medical and ecclesiastical work cannot be given, and its usefulness must have very definite limita- tions. Still, under very rigid control, it may prove an effective measure and be especially useful in counteracting the, on the whole, bad influence of various types and schools of ''healers." One measure cannot cure all diseases or even all of one kind of disease, and the exploitation of infallible cures works an injury to society in the end. Psychotherapy by Emotional Shock. — This is seen typically in the exhortations of religious healers and the dramatic methods of charlatans. Philosophic Psychotherapy. — Most successful results are obtained among the always large number of suggestible and credulous minds by instillating a certain formula, squared by which their symptoms must cease and the disease disappear. This is the method of the Doctrinaire, or Philosopher, or Apostle. For example, it is asserted that God is good, God is all, therefore there can be no evil in you. Sickness is an evil, therefore it cannot exist. Therefore you cannot be sick. You are well. This doctrine ingeniously and eloquently poured into simple ears gradu- ally gains conviction, the patient receives assurance, the attention is distracted from morbid symptoms and the patient gets well — sometimes. The doctrine applied to many thousands always reaches some who only need this distraction of attention from themselves. They ignore symp- toms, and gradually their consciousness gets less sensitive to them. They harden their sensorium, and though they have conditions which would make some sensitive souls feel distressed, these conditions do not arouse their attention which is complacently and wisely directed elsewhere. The only objection to this method of therapeutics is that its funda- mental propositions are unproven, and its general application to surgery, infectious and organic disease is disastrous. But the philosophy of at- tention diversion to the healthy and holy side of life is wise and is indeed used by all physicians and in every form of scientific psychotherapeutics. An essential thing in all forms of systematic psychotherapy is to have the patient on the proper stage. He or she must be removed from all influences but those of the doctor and his attendants. The atmosphere must be such that only his personality and views and counsels affect the patient. Then the work must be kept up for at least a few weeks and often longer. With this environment, the results are reason- ably certain in proper cases. But without it, there is only a chance that the wisest counsel prevail. Then the physician himself must be suited to the kind of work. He need not have a commanding personality, but he must be simple and impressive and sincere, and he must have no unpleasant personal habits. 74 DISEASES OF THE NERVOUS SYSTEM The nurse, if there is one, must equally be a person of tact and experience, good personality and able to co-operate. It can be seen that the systematic application of psychotherapeutics, at its best, is not possible for all, and is not suitable for most. Its unsystematic or rather informal use will always be a part of the equip- ment of the wise physician. It makes it all the more imperative that physicians be good as well as wise. CHAPTER VII DISEASES OF THE PERIPHERAL NERVES Introduction. — The peripheral nervous system consists (1) of twelve pairs of cranial and thirty-one pairs of spinal nerves, with their root ganglia and terminal sense organs; (2) and of the sympathetic nervous system including the autonomic portion of Langley. The peripheral nervous system, therefore, to use the language of modern anatomy, is composed of peripheral motor neurons, peripheral sensory neurons, and peripheral ganglionic neurons (Minot). GENERAL PATHOLOGY Hyperaemia and Anaemia.— Under the head of hypersemia and anaemia there occur types of nerve irritation, leading to different forms of neuralgia, parsesthesia and motor weakness or irritation. Hypersemia Fig. 33. — Showing the cells of origin of the motor nerves in the anterior horns of the spinal cord, and the cells of origin of the sensory nerves in the posterior spinal ganglia. {Van Gehuchten.) and anaemia are, however, secondary conditions and are rarely recog- nized clinically. It cannot always be determined whether an irritated nerve is congested or anaemic, or whether the central part of the nervous system is not mainly at fault. Angina of the Nerves. — ^In arterio-sclerotic conditions of the blood- vessels of the posterior ganglia and nerves there occur spasmodic closures 75 76 DISEASES OF THE NERVOUS SYSTEM of the vessels or, in other words, anginal attacks, which cause severe neuralgic pains. The pathology of this condition will be referred to later. Inflammation of Nerves — Neuritis. — There are two principal forms of neuritis: (1) Interstitial and perineuritis. (2) Diffuse neuritis with parenchymatous degeneration or parenchymatous neuritis. The two forms may be acute or chronic. In the first type there is hypersemia, with sometimes extravasation of blood. An exudation occurs into the fibrous framework of the nerve, with migration of leukocytes. The inflammation may become suppurative or gangrenous. If severe, it destroys the nerve-fibres; but oftenest the axis-cylinders are not destroyed, and recovery takes place. In mild grades of perineuritis, such as occurs in sciatica, there is peri- neuritic exudation, a kind of serous inflammation with not much cellular proliferation. This perineuritis is probably the main factor in the typical neuralgias like sciatica neuralgia. Chronic interstitial neuritis and perineuritis are accompanied with hyperplasia of the connective tissue, compression and more or less destruc- tion of the nerve (Fig. 34). It may ascend or descend, and it is called, accordingly, ascending, descending, or migrating neuritis. It may affect only certain segments of the nerve, when it is called segmental neuritis or disseminated neuritis. Tuberculous and syphilitic neuritis are of the chronic interstitial or diffuse type. These latter forms rarely in- volve peripheral nerves, but rather the intracranial parts of the cranial nerves and the spinal nerve-roots in meningeal tuberculosis or syphilis. A syphilitic peripheral multiple neuritis is, however, thought to occur sometimes. Leprous neuritis is a very typical form of proliferating chronic perineuritis. Cancerous neuritis sometimes occurs and it is of the diffuse type, though sometimes an actual cancerous process invades the nerve. The second type is called parenchymatous or degenerative neuritis and this process of degeneration is the dominant one, so that the changes can be best described under the head of degeneration of nerves. Degeneration of Nerves. — This is a process in which the nerve-fibres gradually die; the myelin sheath and axis-cylinder disappear, leaving only a strand of connective tissue. There are three forms of nerve degeneration: (1) primary; (2) sec- ondary; (3) neuritic or toxic. 1 . The primary form is rare, slight in extent, and of little clinical sig- nificance. In it there is simply a gradual wasting and disappearance of the axis-cylinder and myelin sheath. It occurs in old age, wasting dis- eases and as part of locomotor ataxia. 2. Secondary degeneration or Wallerian degeneration. This form DISEASES OF THE PERIPHERAL NERVES 77 occurs when the nerve is cut across or compressed, or destroyed by inflammation, neoplasms or injuries. The essential part of the nerve-fibre, the axis-cylinder, is simply a prolongation of the process of a nerve-cell. Its next essential part is the myelin sheath. This is of epiblastic origin and consists of a hollow cylinder inclosed in a thin membrane and containing a fatty substance. In degenerative processes of peripheral nerves the medullary sheath is first affected, then the axis-cylinder, least and last the neurilemma. Fig. .34. — Interstitial neuritis. The myelin becomes turbid, splits up into fragments and droplets. The axis-cylinder also breaks up into fragments or swells up and becomes liquefied. Extravasated leukocytes pick up the products of disintegra- tion and form fat-granule cells. The neurilemma and its nuclei usually remain intact. The nerve during this time shrinks in volume and looks grayish and translucent, or grayish-red. The nerve finally becomes only a fibrous cord. Changes can be seen in the nerve within forty-eight hours, and by this time its irritability, which was first slightly increased, 78 DISEASES OF THE NERVOUS SYSTEM is lost. In about two weeks the disintegration of the myehn sheath and axis is practically complete. The peripheral end of the cut nerve shows a loss of nearly but not quite all the fibres as far as its termination. In the central end, the degeneration ascends at first only to the first or second node of Ranvier. Very soon, however, a change occurs in the cell from which the fibre springs. This change is called the reaction at a distance, or degeneration of Nissl. Thus when the neuraxon is injured the whole neuron suffers, but the peripheral end far the most. When a section is made between the spinal ganglia and the cord, the fibres all degenerate toward the cord, and even within it, but the peripheral fibres do not degenerate. Hence the spinal ganglia are the trophic cen- tres of the sensory nerves (see Fig. 35). Degeneration occurs in the motor nerves, also, when the cells of the anterior horns are destroyed. Hence these cells are the trophic S.G. ^ Fig. 35. — Showing effects of section of mixed nerves and sensory root. PMN., Sec- tion through mixed nerve; MR., motor root; S.R., sensory root; S.G., spinal ganglion; Pc, peripheral ganglion. centres for all motor nerves. The process of degeneration takes place at about the same time throughout the whole length of the nerve. The motor end-plates in the muscles are affected a little the earliest. About the cut end, little bulbous tumors may develop, which contain numerous nerve fibrils and connective tissue. The general law is that nerves de- generate in the direction in which they carry impulses, but this is not the whole case, as has been just described. If the injury to the nerve is permanent, a slow decay affects the whole neuron. Supposing for ex- ample a motor nerve is injured or inflamed at the point D. Immediately a degeneration takes place along the parts below to T, and in a few days a slight degeneration takes place in the cell-body C (see Fig. 36) . Within the central nervous system degeneration also occurs mainly in the direction of the nerve impulse. The axis-cylinder is first affected. There is sometimes a preliminary swelling or hypertrophy of this axis- cylinder. Degeneration with calcification of the nerve-fibres sometimes occurs. In associative or commissural fibres the degeneration extends only part of the length of the nerve. 3. Neuritic and toxic nerve degeneration. This form occurs in con- nection with neuritis, and will be described under that head. Its chief DISEASES OF THE PERIPHERAL NERVES 79 characteristics are that the degeneration attacks the nerve in segments, that the axis-cyHnders are not so much affected, and the myehn breaks up into small fatty droplets instead of into large masses. The same general laws apply to it as to Wallerian degeneration. Degenerative processes in the non-medullated nervous fibres have been observed in the fine fibres of the cornea and in the submucous and mesenteric plexuses of the alimentary tract. Regeneration of nerves is a process that usually follows degeneration. It occurs only in peripheral nerves — very little, if at all, in the nerves of the central nervous system of mammals. It is an unique process, in that the nerve is the only specialized tissue that can grow again after being destroyed. Regeneration occurs whenever the trophic centres are healthy, when the mechanical obstacles to a union of the divided fibre Fig. 36. are not too great, and when the peripheral nerve is not too completely atrophied. It occurs most quickly, therefore, when the cut ends are carefully apposed and when the separation has not lasted for a long time, i.e., for years. It progresses always from the central end toward the per- iphery. The fibres of the central stump grow out into the degenerated peripheral fibre. Union by first intention or second intention never occurs. Human nerves cannot be made to unite physiologically, but only anatomically. Regeneration may be complete in a few months in short nerves. In the sciatic it may take one or two years. When regeneration takes place, the axis-cylinders of the central stump swell and divide into a number of new cylinders which pierce or creep around the intervening tissue, enter in bundles the peripheral nerve, and become inclosed in new myelin sheaths and neurilemma. GENERAL SYMPTOMS The dominant symptoms occurring in the distribution of the peripheral nerves are paralysis and atrophy of muscles in a greater or less degree to- gether with sensory disorders. But since the mixed nerve carries with it some vasomotor and secretory fibres, there may also be congestion, oedema, and dryness or sweating of the skin. When a peripheral motor neuron is 80 DISEASES OF THE NERVOUS SYSTEM damaged only to a moderate extent or is irritated by any agent, twitch- ings of the muscle (myokinia) may occur. But direct irritation of the peripheral motor nerve does not cause spasms. NEURITIS AND MULTIPLE NEURITIS The common disorder of peripheral nerves is inflammation or neuritis. When this inflammation affects many nerves it is called multiple or poly- neuritis. When it affects one nerve or nerve group it is called mono- neuritis. The terms plexus neuritis and root neuritis may indicate more specifically the location of the disease. The following table shows the classification on an etiological basis of the different types of neuritis: Mono-neuritis Traumatic neuritis Reflex joint neuritis Occupation neuritis Rheumatic, infectious and toxic neuritis. Multiple neuritis Infectious neuritis Beri-beri, diphtheria, sepsis Toxic neuritis (a) Endogenous, diabetic, cachectic, puerperal and senile, etc. (b) Exogenous, alcohol, CO, CS2 sulphinal, lead, arsenic, mercury, etc. MULTIPLE NEURITIS— POLYNEURITIS Multiple neuritis has been divided into many types in accordance with the cause, the location of the process, and the complications. 1. The common type is a motor-sensory one involving all four ex- tremities and due oftenest to alcohol. Here the paralysis is a dominant feature. 2. Sometimes the sensory and ataxic symptoms are dominant, forming a sensori-ataxic type which is quite rare and is due more often to infections and other poisons than alcohol. 3. A pernicious and hemorrhagic type exists but is also rare. It is due to overwhelming toxaemia. There is also a pure motor neuronitis, multiple in character, due oftenest to lead; and there is a degenerative neuritis of the sensory neurons, especially noted in diabetes. It seems best not to have too many kinds of multiple neuritis, and in fact the motor-sensory type is the common and real disease; the others are terminal conditions, complications or sequelae. The Sensory-motor Type of Multiple Neuritis (Alcoholic Paralysis, Arsenical Paralysis, Diphtheritic Paralysis). — -This is the common form DISEASES OF THE PERIPHERAL NERVES 81 of the disease, making up fully 90 per cent, of all cases seen in the country. While sensory and other symptoms always accompany this type, it is the paralysis and atrophy of muscles which are the leading and chronic conditions. It is a malady coming on acutely, running a sub- acute or chronic course, rarely fatal, and characterized by weakness or paralysis of all four extremities, associated with atrophy, pain, tenderness and various vasomotor, secretory and trophic disturbances. It is a dis- ease pre-eminently due to poisons and infections, alcohol being far the commonest factor. It occurs oftener in the female, owing to the fact that women are more anaphylactic to alcohol than men, and perhaps also because of the predisposing influence of tea-drinking. Multiple neuritis is essentially a disease of early adult life. Almost all cases occur between adolescence and the period of degenerative changes, twenty to forty-five. Young children are very rarely subject to it, but cases have been reported occurring in children at the age of seven, nine, ten and fourteen. A few cases have occurred in persons over sixty, but they are only a little less susceptible than children. It occa- sionally occurs as an epidemic and is then due to some infection. Beri- beri is a particular type of toxic multiple neuritis. The sporadic forms of polyneuritis from alcohol and various poisons and infections occur without much reference to a seasonal influence. Probably more cases occur in spring and fall, owing to sudden changes in temperature. Epidemic influence like that causing cerebrospinal meningitis may increase the number of cases of multiple neuritis. Prac- tically, in this country, the question of drink settles the question of the distribution of polyneuritis. It is rare in the temperate rural districts and smaller towns, and much rarer in native Americans than in foreigners. Sexual excesses, exposure to cold and wet, insufficient diet, excessive tea-drinking and the presence of tuberculosis predispose to the disease. The same neuropathic tendency leading persons to excesses in alcohol, tea and to suicidal indulgence in arsenic is of some moment in leading to the development of neuritis. The list of the special and exciting causes is long and includes nearly all infectious fevers, many chemical and autochthonous poisons. The common infections are diphtheria, puerperal and other septic fevers, and endemic infections of unknown origin. Nearly every infectious fever and possibly malaria may be added to the list. Of chemical poisons alcohol heads the list, causing over two-thirds of the adult cases. Next come arsenic, lead and phosphorus, mercury, copper and trional. Wood alcohol is extremely poisonous, and may promptly cause a multiple neuritis. It has, however, a special tendency 82 DISEASES OF THE NERVOUS SYSTEM to cause optic neuritis and blindness. Even so small an amount as 3 oz. has caused blindness and 5 oz. blindness and death. Among the autochthonous poisons, diabetes and the metabolic products resulting from starvation and cachexia lead to multiple neuritis. Among the foregoing causes, arsenic and diabetes produce more often decided sensory symptoms. One attack does not confer immunity, but it generally compels prudence so that second attacks are rare. Symptoms. — The disease often begins with prodromata lasting several weeks. The patient suffers from numbness, slight pains, and weakness affecting especially the lower extremities. Sometimes a pe- culiar condition of mental confusion and weakness precedes the attack. Usually the symptoms come on rather suddenly. The patient suffers from pains and tenderness in the legs and feet, and is obliged to go to bed. There may be a fever for a day or two, the temperature rising to 102° or even 104°F., but this is not the rule. The pains and weakness increase. The muscles and nerves are very tender. The fingers, hands and arms are often similarly but less affected. At the same time the skin becomes reddened or slightly oedematous. The muscles of the legs grow weak, and in a day or two the patient is unable to stand. In a week or two there may be a complete loss of power in the anterior tibial muscles and a lesser degree of paralysis in the extensors of the hand (Fig. 37). Nearly all of the leg and forearm muscles become eventually involved. Atrophy sets in at the same time and very severe pains are present. The motor cranial nerves are in rare cases affected, and paralysis of the facial or of the third, fourth or sixth nerve has been seen. When the disease is fully de- veloped, which is within a fortnight, there is paraplegia with foot-drop, some degree of wrist-drop, muscular atrophy, and slight oedema, espe- cially of the feet. The skin reflexes are often, the knee-jerk and elbow-jerk usually, lost. There is some tactile anaesthesia, often with hyperalgesia. Temperature and pain sense are later lessened and slowed in trans- mission. The anaesthesia occurs in patches or diffusely. Muscle and articular sense are lost in the sensory or pseudotabetic form, and are usually somewhat involved in the ordinary paralytic form. Pain and sensitiveness continue. The nerves lose their irritability and the muscles show degeneration reaction, partial or complete, the characteristic being a great variability of reaction over different groups of nerves and at different stages of the disease, and an early loss of faradic and lessening of galvanic irritability. There is sometimes retinal hypersemia and even optic neuritis. Of the visceral nerves, the vagus seems oftenest to show signs of involvement, with rapid pulse and disturbances of respiration. In rare cases the phrenic nerve is involved. Three such cases were observed in the wards of Bellevue Hospital in two years. The sphincters are rarely affected DISEASES or THE PERIPHERAL NERVES S3 and then only for a few days. In such cases there is, perhaps, involve- ment of the cord or of the abdominal and pelvic splanchnics. In alcoholic and occasionally in other forms of nemitis, mental symptoms, such as a low, muttering delirium, are very often present, and occasionally a well-marked confusional insanity develops. (Korsakoff's psychosis.) The disease usually reaches its height in a week or two and then starts on a chronic course; but it sometimes happens that exacerbations occur, or that a paralysis and atrophy progress for several weeks before re- gression begins. In alcoholic cases there is often great general prostration; the patients lie for several weeks in a delirious condition, and finally develop pneumonia and die. In diphtheritic neuritis some of the eye and throat muscles (ciliary muscle and soft palate) are involved, while the extremities are usually but slightly or temporarily affected and the sensory symptoms are few. Some further details should be added. Motor Symytoms. — The character- istic paralysis of multiple neuritis is a quadruplegia, all four extremities being involved. The special characteristic is the foot-drop, which is indicative of alcoholic neuritis just as wrist-drop is of lead palsy. The paralysis is typi- cally a peripheral one. It affects the feet and legs, hands and forearms. It usually involves the anterior tibial muscles more than the calf muscles, but sometimes the reverse occurs. The muscles become wasted and flabby. They soon lose, in bad cases, all reaction to faradism, and they require a strong galvanic current to produce a contraction. In anterior poliomye- litis, on the other hand, the diminution in galvanic irritability comes on only after weeks or months. Hence an early loss of galvanic as well as faradic reaction is an important sign of neuritis. As the nerve and muscle recuperate, the galvanic irritability increases. After a time, if the par- alysis is great, contractures occur. The feet are extended, the wasted Fig. 37. — Alcoholic mutiple neuritis with foot- drop and wrist-drop. 84 DISEASES OF THE NERVOUS SYSTEM legs are flexed on the thighs and are almost immovable, and the patient's condition is one of pitiable helplessness. Sensory Symptoms. — Numbness, hypersesthesia, severe pains (dull and sharp), burning sensations, great tenderness all occur and are very marked symptoms. They are felt mostly in the feet, legs and hands. Hypersesthesia is usually followed by anaesthesia to touch and somewhat to pain and temperature. The transmission of these latter two sensations is delayed. The angesthesia sometimes occurs in patches, at other times diffusely over foot, leg and hand. Muscular and articular anaesthesia are common, and in the sensory form are the dominant symptom, causing an ataxia of gait and station. The other special senses are not affected ex- cept in rare cases in which there is optic neuritis. Vasomotor and Trophic Symptoms. — There is often oedema, some- times redness of the skin; occasionally the epidermis of the soles and palms peels off. Glossy skin and profuse perspiration are rare. Erup- tions and ulcers do not occur. Mental Symptoms. — The most common mental disturbance is that so often seen in acute alcoholism, viz., clouded consciousness and a low muttering delirium. This is associated with great general vital depres- sion. If a true insanity develops it is, as a rule, of the type known as Korsakoff's psychosis. The characteristic symptoms are a curious de- gree of forgetfulness and disorientation, together with many and varying hallucinations and delusions rapidly succeeding each other. These often relate to the pains and parsesthesia from which the subjects suffer. They think that there are gloves on their hands or that something is on their feet. They often think that they have been out walking or riding. They are talkative, incoherent and sleepless. Organic Centres. — The bladder is occasionally affected for a short time, the other centres not at all. This freedom from involvement of the sphincters is an important characteristic of the disease in distinguishing it from myelitis. The hlood shows a moderate degree of leukocytosis in uncomplicated cases. From the foregoing it will be seen that the dominant symptoms are parsesthesia, pains (burning, lancinating and dull), muscular tenderness, some anaesthesia, paralysis affecting especially the lower extremities and causing /oo^-c^rop, muscular wasting, with degeneration reactions; with no involvement of the sphincters; sometimes peculiar mental disturbances. The sensori-ataxic or pseudo-tabetic type of multiple neuritis is caused less often by alcohol and more often relatively by diabetes and the metallic and infectious poisons. Arsenic given medicinally in doses of one-sixth of a grain or more may cause such a neuritis. Multiple neuritis from lead is not often seen in painters who suffer only mainly from wrist- DISEASES OF THE PERIPHERAL NERVES 85 drop, but usually occurs when the poison is taken in large doses. The general course of sensory neuritis is much hke that of the paralytic form, but there is less paralysis and, on the other hand, there are more of the burning, tearing pains, a greater degree of anaesthesia, with a very decided muscular anaesthesia causing symptoms of a subacute locomotor ataxia. The paresis, muscular wasting, trophic changes, such as shedding of the epidermis and the electrical reactions, serve to distinguish the disease. A facial paralysis sometimes complicates this type. Endemic and Epidemic Types (Beri-beri or Kakke, Ignipedites, Acro- dynia, Malarial Multiple Neuritis). — Beri-beri or endemic multiple neuritis is seen in this country rarely, and only by accident. Beri-beri is the Indian name; kakke, meaning "the leg disease," is its Japanese name. Ignipedites is a name given by Indian physicians to probably the same disease. French physicians gave the name of "acrodynia" to an epidemic disease which prevailed in France and the Crimea in the early part of the last century. It was probably multiple neuritis. Epidemic multiple neuritis has been observed in two State hospitals in this country, in Alabama and Arkansas. It has also been observed in epidemics in New York, Connecticut and Vermont. The disease is due mainly to a monotonous and more or less exclusive diet of unpolished rice. It may be produced also by a monotonous diet of other starchy substances, such as wheat; over-crowding, bad hygienic conditions, heat, moisture and ex- posure favor its development. There are various types of this disease, in some of which the neuritic symptoms seem subordinate to those of other organs. The forms de- scribed are: The paraplegic or dry, the dropsical or wet, and the mixed and very acute forms. The symptoms generally resemble those of multiple neuritis, as already described, plus oedema, extensive serous effusions, serious heart weak- ness and gastro-intestinal disorders. I have observed a case of undoubted endemic neuritis (beri-beri) which ran precisely like an ordinary multiple neuritis. The paralysis affects especially the lower extremities, but in beri-beri there seems to be an especial tendency also to involvement of vasomotor and visceral nerves. The disease runs a course like other types of toxic neuritis. It can be cured by feeding with rice bran, adding other substantial food. Malarial Multiple Neuritis. — It is hkely that ''malarial" multiple neuritis is only a form of beri-beri which is not rare in some of the islands of the West Indies. Acute Pernicious Multiple Neuritis — The Heine-Medin Disease. — There is a form of multiple neuritis which comes on suddenly, progresses rapidly, and causes death in a few days or weeks. These cases usually 86 DISEASES OF THE NERVOUS SYSTEM show the ordinarj^ symptoms of neuritic paralysis, with final involvement of the cardiac and respiratory nerves, causing death. The neuritis is interstitial and hemorrhagic. The disease is due to an infectious poison which overwhelms the system before it has time to set up any inflammation or organic change. In these cases the anterior-horn cells of the spinal cord are also involved, and the disease is one that attacks the whole peripheral motor neuron. It is probable that Landry's paralysis and this severe form of multiple neuritis are due to the same cause, and that they are allied to, if not one phase of, acute anterior poliomyelitis forming a part of the Heine-Medin disease. This is the name given to the in- fection which causes the above-mentioned diseases as was as encephalitis. Pathology. — In multiple neuritis the disease affects the periphery of the nerves most, and extends up, very rarely reaching the roots. The Fig. 38. — Alcoholic neuritis parenchymous degeneration. anterior tibial and musculo-spiral nerves on the two sides are oftenest and most diseased. The process when mild in grade resembles a secondary degeneration following section of the nerve (Fig. 38). In severer cases there is evidence of interstitial inflammation as well as degeneration. This process, however, varies in degree at different points of the nerve's course. Hence it has been called segmental or disseminated neuritis. In some of these cases and in all acute pernicious cases there is still more interstitial inflammatory change; small hemorrhages occur, exudation takes place, and collections of leukocytes about the vessel walls and among the nerve-fibres are seen (Fig. 39). The muscles supplied by the dis- eased nerves undergo atrophy. This is usually simple and non-inflamma- tory. But sometimes there is an interstitial myositis with exudation DISEASES OF THE PERIPHEEAL NERVES 87 compressing the fibres (Senator). If the disease progresses, the nerve- fibres degenerate and their place is taken by connective tissue, and the same process occurs in the muscles. The spinal cord when examined by the help of Nissl and Marchi stains shows some involvement. The anterior-horn cells undergo the same degeneration as that which occurs when the nerve is cut across, and slight areas of degeneration are found in the posterior and lateral ' "*; \ ^^ : -?i:5^ 1 .. H Fig. 39. — Arsenical neuritis (parenchymatous) spinal nerve root. columns. The changes are very slight compared with those in the nerves, and in the writer's experience are secondary. It will be seen, therefore, that in multiple neuritis there may be: (1) simple degeneration; (2) parenchymatous inflammation with some evidences of interstitial neuritis (degenerative neuritis); (3) decided interstitial neuritis with degeneration of nerve-fibres. The differences depend on the intensity of the poison. Diagnosis. — Multiple neuritis must be diagnosticated from diffuse or transverse myelitis, anterior poliomyelitis, locomotor ataxia, spinal 88 DISEASES OF THE NERVOUS SYSTEM meningitis, and hemorrhage, and from Landry's paralysis. Practically, dijffuse myelitis is the disorder from which it has oftenest to be distin- guished. From this it is recognized, first, by investigating the cause and onset. Neuritis begins more slowly and with sensory prodromata; it affects the legs and feet, especially the extensors, and if it ascends it skips the hips and trunk and attacks the forearms. There is more muscular atrophy than in myelitis; the knee-jerks are absent. It pro- gresses more slowly, and after four or eight weeks gradually regresses. Fig. 40. — Interstitial neuritis, cross-section of 3d cranial nerve. Electrical degeneration reactions are more varied and decided. There is tenderness over the muscles and nerves and peculiar burning, darting pains. The cutaneous anaesthesia, if present, is not so extensive and complete, as a rule, while muscular anaesthesia is more decidedly marked. There is very rarely involvement of the sphincters nor are there bed- sores. There may be belt-like constrictions felt round the extremities, but not around the waist. The gradual improvement of the paralysis and atrophy and eventual recovery confirm the diagnosis of neuritis. DISEASES OF THE PERIPHERAL NERVES 89 The presence of neuritis of the cranial nerves would strengthen the theory of a general neuritis. From poliomyelitis it is distinguished by the presence of pain and other sensory symptoms, the early fall in galvanic irritability, the age of the patient and the etiology. From locomotor ataxia neuritis is distinguished by its more rapid onset, the presence of paralysis and atrophy of muscles, paresis, with degeneration reactions, and the absence of involvement of the organic centres and pupils. Spinal hemorrhage usually leads soon to a secondary diffuse mj^elitis easily distinguishable from neuritis by the characters above given. Here there is also usually pain in the back. Spinal meningitis is associated with characteristic pain, tenderness and stiffness along the back. Acute ascending paralysis in its typical form shows but very slight sensory disorders, and no wasting or change in electrical irritability. The complication of multiple neuritis and myelitis or posterior sclerosis is possible, but is very rare. In the former case the ordinary symptoms of myelitis are added to those of neuritis. In locomotor ataxia there is often some nerve degeneration and occasionally neuritis. The nerve degeneration probably causes only slow atrophic changes and paresis; the neuritis causes pains, anaesthesia, skin eruptions and local trophic disorders. Prognosis. — Alcoholic multiple neuritis is a serious disease, because of its associated conditions. Nearly one-half of my hospital patients have died, mainly because they continued the use of alcohol after paraly- sis appeared. They do not die of neuritis, but of alcoholism, pneumonia or of phthisis. Other forms of neuritis rarely cause death. The great majority recover almost completely. It may be from six months to two years before all symptoms disappear. The average time is about a year. Treatment. — The patient needs, first of all, rest in bed. The limbs are often extremely tender and the patient's pains excruciating. To relieve these the legs may be painted with menthol and enveloped in cotton batting. In other cases flannels wrung out in hot water and renewed every two hours give relief. Internally phenacetin, antipyrin or other coal-tar products may be given for the pains. Fluid extract of ergot in doses of 5i- to §ii. repeated in three hours sometimes relieves pain. In the early stages, salicylate of soda in doses of gr. xx. every two or three hours is recommended. If there is a great deal of depression from alcoholic poisoning, strychnine, gr. 3^^o Q- 3 h., and aromatic spirits of ammonia, §ss. q. 3 h., should be used. There is no drug which really cuts short the process. The best measures for this purpose are rest, thorough cleansing of the alimentary tract, abstinence from alcohol and a nourishing diet. 90 DISEASES OF THE NERVOUS SYSTEM After the acute stage is passed the labile galvanic current occasionally interrupted fmay be applied, 5 to 10 ma. for ten minutes three times daily. Later, by the sixth week, the faradic current, massage and careful exercise can be given. At this time or earlier (third week), strychnine, iodide of potassium, arsenic in small doses and tonics may be given. In old cases in which a great deal of paralysis and contracture have occurred, forcible extension of the limbs, the use of splints and rubber muscles are needed. With patience and perseverance the worst chronic cases can eventually be brought to a complete recovery. Complicating Forms of Neuritis and Neuritic Degeneration. — Neuritis and neuritic degeneration complicate many diseases, but they especially mark and modify subacute and chronic rheumatism, locomotor ataxia, diabetes, paralysis agitans, wasting diseases and old age. A neuritic degeneration almost always affects the nerves in the neigh- borhood of an old rheumatic joint. The chief result of this is to produce wasting and some paresis of the muscles moving the joint (Pitres and Vaillard). The process is a reflex atrophy (see Arthritic Muscular Atrophy) . In locomotor ataxia parenchymatous nerve degeneration is very often present. It does not produce the cardinal symptoms of this disease. It does, however, cause some of the anaesthesia, parsesthesia, muscular atrophy, sldn dystrophies and visceral crises. In diabetes the neuritis takes the form of the sensory type of multiple neuritis, and causes parsesthesias and neuralgias somewhat hke those of locomotor ataxia. The patient has sciatiC; intercostal and other neuralgic pains, burning or numb feet, and sometimes loss of tendon reflex. The upper extremities are rarely affected. In Wasting Diseases and Old Age. — In various wasting diseases, such as phthisis, cancerous cachexia, long-continued fevers, marasmus, and in senility, a simple parenchymatous degeneration of nerves, with atrophy, occurs (Arthaud, Koster, Jappa). The symptoms caused by these changes are very shght. They contribute to the weakness and wast- ing. In old age, the atrophy of the nerves is one cause of the lessened sensibiHty and activity of the skin and its underlying muscles. TUMORS OF NERVE Classification and Pathology-^Nerve tumors occur as : 1. Nerve hyperplasia. 2. True neuromata. 3. Fibro-neuromata. 4. False neuromata. 1. Hyperplasia or hypertrophy of nerve trunks is very rare. Gen- DISEASES OF THE PERIPHERAL NERVES 91 erally the increase in size is due to increase of the interstitial connective tissue. Sometimes, however, there is an increase in the number of fibres and thickening of the myelin sheath. 2. True neuromata are also very rare, and occur almost exclusively on spinal nerves. In some there is an increase in medullary fibres; in others only an increase of non-medullated fibres, i.e., only the axis- cylinders and neurilemma increase. They occur either singly or multiply. True neuromata are usually small, ranging from 1 cm. (two-fifths of an inch) to 6 cm. in diameter. They may be much smaller or larger. They are usually few in number, but may be very numerous. Gowers estimates in one case that as many as 1000 were present. Even larger numbers have been observed. They rarely cause serious symp- toms, but may produce local pains and paraesthesias. 3. Multiple fibro-neuromata, when numerous, constitute a condition known as neurofibromatosis (Recklinghausen's disease). This is a rare condition, but one of importance because sometimes these tumors develop on cranial nerves, gradually increase in size and become practically brain tumors. The following description is from an article upon this subject by Fraenkel and Hunt. The tumors occur in varying size from that of a pin's head to that of a goose egg. In some cases the manifestation of the disease is expressed by a solitary growth upon one nerve, and in other cases several hundred growths have been present. They occur on all cerebrospinal and sympathetic nerves except on the optic and the olfactory. The acoustic nerve has been shown to be particularly liable to the formation of fibro- mata and fibrosarcomata. The origin of these tumors is usually from the endoneurium, rarely from the perineurium and exceptionally from the epineurium. They are situated either centrally within the nerve or eccentrically on the periphery of the nerve. The general conformation of the tumors is spherical or elliptical and in generalized cases the nodular outgrowths give the nerve a convoluted appearance, sometimes resem- bling a rosary. Histologically, the fibroma nervorum is made up of connective-tissue elements, which present all the variations in structure between the fibroma molle and the fibroma durum. The neurofibromata are usually slowly growing tumors of a benign nature. Sometimes, however, they undergo a retrograde metamor- phosis and show cystic, fatty or myxomatous degeneration or they undergo sarcomatous transformation, assuming a malignant character. Neurofibromatosis depends often on a hereditary or congenital dis- position. It is frequently associated with more or less mental impair- ment, imbecility, and somatic stigmata of degeneracy. Scrofulosis and 92 DISEASES OF THE NERVOUS SYSTEM tuberculosis are often found in the histories of the cases. The tumors are not painful and cause no symptoms when confined to the skin. Fig. 41. — Neurofibromatosis. (Fraenkel and Hunt.) Neurofibromatosis may show itself in one of the following ways: (1) Tubercula dolorosa (painful tubercles, Wood); (2) multiple neuro- DISEASES OF THE PERIPHERAL NERVES 93 fibromata of the skin (molluscum fibrosum, Recklinghausen's disease); (3) neuromata or neurofibromata of a single peripheral nerve; (4) neu- roma plexiforme, pachydermatocele, and elephantiasis neuromatodes; (5) generalized neurofibromatosis; neurofibromata of the skin, cerebro- spinal and sympathetic nerves. 3. Neurofibromata of individual peripheral spinal nerves (isolated neuromata) are surgically well known. They express themselves clin- ically by parsesthesias and progressive motor weakness of the peripheral nerve type, and are of good prognosis when recognized. They occur most frequently on the median and sciatic nerves; they are rarely without symptoms and are prone to give rise to considerable hyperses- thesias and little paralysis. 4. False Neuromata. — ^This term is applied to the various nerve tumors in which a fibroma, myxoma, glioma, sarcoma, carcinoma or syphiloma grows upon or in the nerve. The fibroneuroma as above de- scribed is really a false neuroma and is the common form; glioneuroma has been observed on the auditory nerve. Syphiloma occurs only on the intracranial or intraspinal nerves. Carcinoma of nerves may be primary, but is generally secondary, and is of scirrhous or medullary type, rarely the colloid. Leprous neuritis sometimes forms neurofibromatous swell- ings. A few cases have been observed of multiple malignant neuromata. Trauma and hereditary influence are the etiological factors. The great nerve trunks are oftenest affected, the median, sciatic and crural ranking first. The tumors start from the perineurium; they are at first spindle- shaped, and maj^ grow very large. Sarcomatous cells are oftenest found in them; but they may be myxomatous, fibromatous or mixed. Etiology. — Three general causes exist for the production of neu- romata, viz. : 1. A hereditary or a neuropathic predisposition, which tends to cause the true, the multiple, and the plexiform neuromata. 2. In- juries, surgical operations; these cause especially the fibroneuromata of which the amputation neuroma is an example. 3. Diathetic, e.g., tuber- culous influences, and whatever produces the various tumor formations, sarcoma, carcinoma, form the third etiological factor. Neuromata of the plexiform type are often congenital. Alultiple neuromata may de- velop early in life. Men are far more subject to multiple neuromata than women. Symptoms. — Neuromata often cause no symptoms. Perhaps the most frequent evidence of their presence, however, is pain and some tenderness. The pain is exacerbating, and may be stopped sometimes by pressure on the nerve above the tumor. Parsesthesia, anaesthesia, paralysis, and reflex spasm may be present. Some forms of intract- able headache are possibly due to multiple neuromata. A neuroma upon one of the cranial nerves, usually the acoustic, may develop into and cause 94 DISEASES OF THE NEEVOUS SYSTEM the symptoms of a brain tmnor. Hence the presence of nem^ofibromata of the skin with brain symptoms should always suggest the possible presence of an intracranial growth. Multiple and plexiform neuromata cause symptoms less often than a single larger neuroma. A neuroma on the pneumogastric or other splanchnic nerve may cause severe symptoms. Fig. 42. — Neuro-fibroma, Recklinghausen's disease. Multiple (true) neuromata may last for years and cause no serious in- convenience. Malignant neuromata cause always such symptoms as would naturally follow irritation and compression of a nerve. The treatment of neurofibromata and of nerve tumors generally is usu- ally purely surgical when any treatment is indicated. CHAPTER VIII MOTOR DISORDERS OF CRANIAL NERVES The general distribution of the paralyses from injury or disease of the different motor nerves is shown in the accompanying table of cases. I am indebted to my friends Dr. Wm. P. Wilkin and Dr. Alexander S. Leverty, for the larger part of the work of compilation: Cranial-nerve paralyses (including 19 occurring in locomotor ataxia. . 206 Spinal-nerve paralyses 377 Multiple neuritis 114 697 The cranial nerves having motor function are paralyzed in about the following frequency : Facial, 136; Oculo-motor, 52; V, X, XI, 3. Taking all motor nerves, cranial and spinal, one finds that the brachial plexus and its branches are oftenest affected by paralysis, next the seventh cranial nerve, then the ocular nerves, and last the lumbar and sacral plexus. This represents the frequency in a neurologist's experience. In general practice and especially in surgical practice there would be fewer cases of cranial-nerve disease and more of lumbar and sacral palsies. THE OCULAR MUSCLES Anatomy. — The motor nerves of the eye are: (a) The third or oculo-motorius, supplj^ing the internal, superior and inferior recti, inferior obliquus, the levator palpebrse, the ciliarjr muscle, and constrictor of the iris. (6) The fourth or trochlearis supplying the superior oblique. (c) The sixth or abducens, supplying the external rectus. (d) The sympathetic, consisting of fibres from the cervical cord to the dilators of the iris, to its blood-vessels, and to the unstriped portion of the levator palpebrse and to the unstriped musculus orbicularis. The third and fourth nerves arise from a series of nuclei in the floor of the aqueduct of Sylvius. They leave the brain at the anterior edge of the pons. They run in the cavernous sinus and enter the orbit through the sphenoidal fissure. The sixth nerve arises from a nucleus in the floor of the fourth ventricle. It emerges at the posterior edge of the pons, runs in the cavernous sinus, and enters the orbit through the anterior lacerated foramen. The nuclear gray matter from which these nerves arise is made up of a series of nests of cells and each pair supplies a different set of muscles of the ej^e, as shown in the diagrams (Figs. 44 and 47). 95 96 DISEASES OF THE NERVOUS SYSTEM The nucleus of the sixth lies farther back in the floor of the medulla, but it belongs to the same serial deposit of gray matter and represents the continuation of the ante- rior horn of the spinal cord (Fig. 48). The motor nerves of the eye, third, fourth and sixth, are closely connected with each other and other nerves by a long commissure, the posterior longitudinal bundle. The fibres of the third and sixth nerves pass to their nuclei on the same side, then decussate and pass up in the inner part of the crusta to the frontal part of the prse- central convolutions of the cortex. A few fibres decussate and enter the nuclei of the opposite side. They are connected with the internal rectus nucleus. Optic cWasma Infundibulum Olfactory bulb Left corpus mamiUare Substantia perforata posterior Pedunculus cerebri Olfactory tract Optic nerve Abdiicens nerve Hypoglossal nerve Substantia perforata anterior Optic tract Tuber cinereum Oculomotor nerve Trochlear nerve Trigeminal nerve Facial nerve ■Acoustic nerve Nervus intermedius 'Glosso-pharyngeal nerve Vagus nerve Accessory nerve Hypoglossal nerve Fig. 43. — Showing the origin of the nerves at the base of the brain. Medulla Oblongata Medulla spinalis (cut) The fibres of the fourth nerve almost entirely decussate, running forward a long distance before they finally reach their nuclei. Thus it appears that the fourth is the only cranial nerve except the optic which largely decussates before reaching its nucleus. However, those fibres of the third which supply the internal rectus also decussate, as already stated. The arrangement and composition of the nuclei of the oculo-motor nerves as given by Bing is as follows : 1. A small-celled lateral nucleus, the nucleus of Edinger and Westphal. 2. A large-celled lateral nucleus. 3. A small-celled mesial nucleus. MOTOR DISORDERS OF CRANIAL NERVES 97 Of these the longest is the large-celled lateral nucleus. It contains, ranged one behind the other (a) The centre for the levator palpebra? superioris. (6) The centre for the rectus superior. (c) The centre for the rectus internus. (d) The centre for the obliquus inferior. (e) The centre for the rectus inferior. (See Fig. 44.) The fibres from (a) and (6) arise e:vclusively from the nucleus of the same side, those from (e) exclusively from that of the opposite side, while those from (c) and (d) arise from the nuclei of both sides. The mesial nucleus is the centre for accommodation. The Westphal-Edinger nucleus innervates the sphincter pupilloe. The mesial and lateral small-cell nuclei send fibres which pass first to a ganglion before going to the muscles they supply and they belong to the mid-brain autonomic system . In order to understand the peculiarities of eye palsies, to be de- scribed later, the relations of the sixth to that nucleus of the third nerve which innervates the internal rectus must be understood. In turning the eyes to one side, these two nuclei and their nerves act together, causing the external rectus of one eye and the internal rectus of the other to contract at the same time. The impulse from the brain which does this decussates and acts first upon the sixth, and through this upon the external-rectus nucleus of the same side. The impulse from this nucleus then goes to the third-nerve fibres of the same side and thence to the internal rectus. This can be better understood by the diagram, Fig. 45. Thus lesions in the brain at (a) cause paralysis of the sixth nerve of the opposite side and internal rectus of the same side. The eyes turn toward the side of the lesion. Lesions in the pons at (5) cause paralysis of the sixth on the same side and internal-rectus nucleus of the opposite side. The eyes turn away from the side of the lesion. The movements of the eyeball are made by the simultaneous action of several muscles. Most of them act as their names indicate. But the obHque muscles help to depress or elevate, and then help to rotate in or out according as the internal or external rectus is acting. The cortical centres for the eye muscles are not positively known. Lesions in the inferior parietal lobule sometimes cause disorder of the third nerve, but the dominant centres are in the prefrontal lobe at the base of the upper and middle frontal convolutions. As the optic nerve is the special sensory nerve of the eye, so the third, fourth, sixth and part of the seventh nerves are the motor nerves. By means of the optic nerve and its receptive and refractive apparatus, the form, color, movement and, to some extent, relations and distance of objects are determined. The motor nerves adjust the eye to near and 98 DISEASES OF THE NEKVOUS SYSTEM distant objects, inform us as to size and distance, and enable us to follow moving objects and to shift the gaze readily. They also assist in protect- ing the eye against injury. General Symptoms. — It is impossible always to disassociate diseases of the oculo-motor nerves from those involving their nuclei. Hence we must study here really the affections of the whole neurons. These are: (1) Paralyses or ophthalmoplegias, which may be acute, chronic, or progressive; (2) pareses or myasthenic states, called ordinarily muscu- FiG. 44. — The nuclei of the motor nerves of the eye. (Bing.) lar asthenopias; (3) spasms, such as strabismus, nystagmus, and ble- pharospasm; (4) reflex disorders. There are many special terms which are used to indicate the peculiar effects of various paralyses and spasms of the ocular muscles and nerves, and some of these I will define here: Erroneous protection is a condition in which the patient is unable to judge exactly of the relation of external objects to the body; for this relation is determined by the movements of the ocular muscles, and, these being weak, wrong sensations are conveyed to the brain. Vertigo may result from this disturbance of muscular sensation. MOTOR DISORDERS OF CRANIAL NERVES 99 Diplopia or double vision is a condition clue to the erroneous sen- sation resulting from eye-muscle palsy, and to the fact that the images of the object fall upon non-corresponding retinal fields. Diplopia is simple or homonymous when the false image is seen on the same side as the affected eye. When a red glass is placed over this eye two images are seen, the red one being on the side of the eye involved. Diplopia is heteronymous or crossed when the false image is on the side opposite to the sound eye. Conjugate deviation of the eyes is a condition in which both ej^es turn strongly to one or the other side. It may be paralytic or spas- modic. The mechanism is a complicated one and not perfectly under- stood. In general, destructive lesions of the brain cause a paralytic Fig. 45. — Diagram showing the probable relations of the nuclei of the sixth and of the internal rectus branch of the third to the brain. P. L. B., Posterior longitudinal bundle. deviation toward the side of the lesion, and irritative or compressing lesions the opposite effect. Destructive lesions in the pons cause a deviation away from the side of the lesions. The palsy then involves the sixth cranial nerve and the branch to the internal rectus from the third. The sixth-nerve nucleus is the dominant one, and impulses from the brain go to it first (see Fig. 45). In diseases of the motor nerves of the eye it is found that the paralyses occur in various ways, which may be best grouped as follows: Ophthalmoplegias 1. Paralyses of the third nerve. \ Acute. 2. Paralyses of the fourth and sixth nerves. / Chronic. 3. Progressive paralysis of all or part of these nerves. 100 DISEASES OF THE NEEVOUS SYSTEM THE OPHTHALMOPLEGIAS I. Paralysis of the Oculo-motorius or Third Nerve — Etiology. — The commonest causes are "exposure to cold" (which means some simple infection), and syphilis. Other causes are basal meningitis, intracranial tumors, injuries, compression from orbital tumors, the diphtheritic poison; excessive use of tobacco and alcohol, of morphine, or other drugs may be predisposing causes. Temporary palsy sometimes occurs in migraine or it may take the place of an attack of migraine. Partial or temporary palsies also occur in locomotor ataxia, in diphtheria, in certain primary muscular atrophies and in myasthenia gravis. There occurs, in rare cases, an acute inflammatory degeneration of the nuclei of the ocular-muscle nerves similar to acute anterior poliomyelitis. This con- dition has been called ''polioencephalitis superior" or upper bulbar palsy. Symptoms. — When all the muscles supplied by the third nerve are para- lyzed, there is dropping of the lid (ptosis, Fig. 46) ; the eye can be moved only out- ward, and downward and inward; there is, therefore, divergent strabismus and double vision (diplopia). The pupil is somewhat dilated and does not contract to light, owing to paralysis of the con- strictors of the iris; and there is loss of power of accommodation, so that the patient cannot read print close to him. The patient suffers much annoyance from the lid-drop and the double vision, and there are sometimes vertigo and pho- tophobia. Only one nerve is involved at a time, as a rule. The various eye muscles supphed by the third are rarely all attacked. The levator may escape almost entirely; the ciliary muscle and iris may also be but slightly involved; while these latter muscles are never involved alone in ordinary types of the disease. They may be paralyzed alone in diph- theria or in an attack of migraine. The affection usually runs a subacute course, lasting but a few weeks. Functional palsies last but a few days; syphilitic palsies are usually temporary (one to three weeks), but may relapse or become extremely obstinate. Periodical palsies occur every year or six months or even oftener; they last a few days or weeks and are accompanied at Fig. 46. — Paralysis of 3d nerve with ptosis. {Wildbrandt and Sanger.) MOTOR DISORDERS OF CRANIAL NERVES 101 first by some pain. They continue to recur for years. They may be associated with attacks of migraine and are migrainous equivalents. In diphtheritic eye palsies the first three or four nuclei of the series making up the origin of the third nerve are oftenest affected, causing paralysis of accommodation, paralysis of the iris and of the internal rectus, the three muscles concerned in accommodating the eye to near objects. Pathology. — In the rheumatic palsies there is a low grade of per- ipheral neuritis, and the same is true of most diphtheritic and other palsies of infectious origin. In syphilitic and tabetic palsies there is usually a specific basilar meningitis involving the nerve-roots. The meningitis may be slight or may amount to gummatous deposit. In functional and some periodical palsies there is a vasomotor disturbance causing congestion or anaemia or perhaps simply inhibition of the nuclear centres. Some periodical palsies have been found to be due to small tumors involving the nerve-root. In rare cases there is primary mus- cular atrophy of the eyeball nerves or primary degeneration of the nuclear centres. The nuclear inflammation forming "polioencephalitis superior" is a disease probably infectious and quite similar to anterior poliomyelitis. Special Symptoms. — One must determine how extensively and which of the muscles supplied by the third nerve are involved. If only the levator palpebrse, there is simply falling of the lid. If the external eyeball muscles are involved, we get: Limitation of movement of the globe. Strabismus and secondary deviation. Erroneous projection. Double vision or diplopia, which is either simple or crossed. If the motor fibres to the iris are paralyzed there is immobility and dilatation of the pupil and there is a loss of light reflex. If the light reflex is gone but accommodation persists, we have the so-called Argyll- Robertson pupil. Iridoplegia without cycloplegia is most significantly present in syphilitic conditions. Iridoplegia may also occur in infections like diphtheria and from toxic states and local disease. If the motor fibres to the ciliary muscle are paralyzed we have cycloplegia, or paralysis of accommodation. It also may occur in syphilis, tabes, paresis and in diphtheria. It sometimes occurs as a symptom in migraine. Paralysis of Convergence. — The internal recti muscles may be quite normal except that they cannot converge the eye. When this is the case there is also loss of the associated pupil contraction. This form of paral- ysis sometimes occurs. It is inferred that there are two separate centres 102 DISEASES OF THE NERVOUS SYSTEM for contracting the pupil. One associated with the Hght reflex and one with convergent movement (Turner). Paralysis of the levator palpehrcB, causing ptosis, is sometimes seen alone. A functional palsy of the lids sometimes occurs in anaemic and nervous people at the time of waking. It is a temporary morning or waking ptosis. Ptosis is a frequent and early symptom of myasthenia gravis. Paralysis of the sympathetic fibres of the eye causes the Horner syndrome, viz.: contraction of the pupil (myosis) from the unopposed ac- tion of the third nerve. There is also a slight retraction of the eyeball, a narrowing of the palpebral fissure, sweating of the face on the affected side and lessened intraocular tension. The pupil does not dilate when the skin of the cheek or neck is irritated, a condition known as loss of oculo-skin reflex and it does not dilate in the dark or respond to an in- stillation of cocaine. On the other hand the pupil contracts to near vision and convergence. The spinal centre is in the first dorsal and eighth cervical segment. The fibres pass out through the first and second anterior dorsal roots, scend in the cervical sympathetic, pass to the Gasserian ganglion and thence through the first division of the trigeminus to the pupil. II. Paralysis of the Fourth Nerve. — This is a rare affection and not always easily detected. The causes are much the same as those of palsy of the third nerve. The symptoms are slight convergent strabismus when the eye is moved downward and diplopia on looking down. There is defect in the movements of the eye downward and outward. III. Paralysis of the sixth nerve (abducens) is the most frequent of eye palsies, and occurs especially often in syphilis and in locomotor ataxia. It causes convergent strabismus and double vision. Progressive Ophthalmoplegia. — ^Besides the palsies already described, there occur certain forms which have a peculiar origin and course. They begin slowly, as a rule, and steadily progress. In some cases only do they reach a certain stage and then remain chronic. They often affect the third, fourth and sixth nerves together. In accordance with the muscles invaded, these palsies are called external, internal, partial and total. Thus if those branches of the third nerve supplying the iris and ciliary muscle are involved alone, it is ophthalmoplegia interna; if the other branches are involved, it is called ophthalmoplegia externa. Definition. — Progressive ophthalmoplegia is a degenerative disease of the nuclei of the motor nerves of the eye. It is in most cases the same disorder as of bulbar paralysis and progressive muscular atrophy. Etiology. — It develops between the ages of fifteen and forty, but may occur later. The sexes are equally affected. Lead, diphtheria, traumatism, syphilis, appear sometimes to be the cause. It may compli- MOTOR DISORDEES OF CRANIAL NERVES 103 cate locomotor ataxia; more often it forms part of progressive muscular atrophy. The sijm-ptoms are often not noticed until the disease is well advanced. The vision is not disordered, and there is only a gradual limitation of mobility of the eyeball. A slight drooping of the lids, causing a sleepy look, or a slight squint, usually divergent, is noticed. Then upon ex- amination it is found that the eyes are immovable and cannot follow the finger, except to a slight extent. The peculiar physiognomy which re- sults is known as the "Hutchinson face." The iris reacts to accommo- dation and light usually. Double vision may be present. Usually the patient accustoms himself to monocular vision. The disease lasts a long time, and it may become stationary. If complicated with progressive muscular atrophy, however, the course is relatively rapid, death occur- ring from the latter disease in two or three years. Pathological Anatomy. — In all progressive cases there is a degenera- tive atrophy of the nuclear cells. In a few stationary cases the anatom- ical change is that of neuritis. The treatment is that for the disease which it complicates or the con- dition which causes it. That is to say, it is the treatment for locomotor ataxia, progressive muscular atrophy, syphilis or lead poisoning. Iodide of potassium, strychnine, arsenic, and phosphorus may be given. Elec- tricity is of no value. General tonic measures and rest to the eyes should be employed. Muscular Asthenopia and Muscular Insufficiencies. — ^This is a term employed to indicate a lack of equilibrium of the muscles of the eye, as a result of which the visual axes cannot be kept parallel without an effort. This effort is often unconscious, and shows itself only by a ready tiring of the eye on attempting to read or by the production of headaches and cerebral paresthesias. Examination of the eye by means of prisms re- veals the special character of the trouble. The term is usually apphed to functional, congenital and neurasthenic weaknesses, not to those due to organic disease or severe toxaemias. When the eye muscles act normally, the condition is called one of orthophoria. When some of the muscles are weak it is called heterophoria. There are various forms of heterophoria, viz. : esophoria, a tending of the visual lines inward, from weakness of the externi; exophoria, a tending of visual lines outward ; hyperphoria, a tending of the visual line of one eye above its fellow. The condition is tested in various ways. The simplest is this: Re- fractive errors having been corrected, a series of prisms is placed over the eye, at first with the base inward, while the patient looks at a candle 20 feet distant. The prisms are increased in strength until the patient can no longer coalesce the images. The degree of prism is noted, and 104 DISEASES OF THE NERVOUS SYSTEM this indicates the strength of abduction or of the externi. The same process is gone through with for the interni, the base of the prism being out. The externi should overcome a prism of about 8°, the interni one of 23° to 25° or more. There are great individual variations, and there is also considerable variation in individuals. For details of the technic for testing heterophoria the reader can best consult special works. Muscular asthenopia is said to cause a disturbance of vision, vertigo migraine, cerebral parsesthesia, and pains in the head, more particularly in the occipital and cervical region. It may be a factor in producing choreic twitchings in the face. In neurasthenic persons it may cause a wider range of nervous symtpoms. It is said to be a factor in causing epilepsy, chorea, and hysteria. The author cannot accept this latter view, and believes that the importance of muscular asthenopia in causing general nervous symptoms is not great. Much of it, if not all, may be relieved after correcting refractive errors by helping the general health of the patient. The treatment of it, after all myopia, or hypermetropia, or astigma- tism, if present, is relieved, consists in building up the general health, in the systematic use of prisms for training the muscles, and the wearing of proper glasses. Sometimes graduated or complete tenotomies are advisable. SPASMODIC DISEASES OF THE OCULAR MUSCLES These are: (1) Conjugate deviation from spasm; (2) irregular and associated spasms from convulsive and irritative brain disorder; (3) nystagmus. Spasmodic conjugate deviation occurs from an irritating lesion of the ocular nuclei or of the brain in its cortical motor and mid-brain tracts. Irregular spasmodic movements occur in meningitis, hydrocephalus, and in lesions involving the semicircular canals. Peculiar associated spasms occur in hysterical attacks. Various spasmodic movements and contractions of individual eye muscles occur from ocular disease, errors of refraction, muscular weakness, and paralysis of certain eye muscles. Rhythmical spasm or nystagmus occurs as the result of hereditary visual weaknesses, of refractive errors of various kinds, in albinoes, and in chronic hydrocephalus. It is found usually in neurotic persons who have ocular defects, in multiple sclerosis, and sometimes in chorea. It occurs also in hereditary ataxia, tumors, especially of the cerebellum, and mid-brain, and in meningitis. It occurs in miners and is called miner's nystagmus. In nystagmus the oscillation of the eyeballs is usually lateral. It may be brought out when slight in degree by causing the patient to look MOTOR DISORDERS OF CRANIAL NERVES 105 steadily to one side. Vertical and a kind of rotating nystagmus some- times occur, and are due to lesions of the mid-brain and its vestibular connections. Spasm of the levator palpebr^ is sometimes seen and is usually tonic. The above troubles are all symptomatic, and their treatment de- pends upon correction of some local disease or serious cerebral lesion. THE MOTOR BRANCH OF THE FIFTH CRANIAL NERVE The anatomy of this nerve is described under the head of the neu- ralgias of the trigeminus. Nuc. iii E. W. Nuc. iii lat. Nuc. iii med. Nuc. iv. Locus coeruleiis Nuc. Mot. V. Nuc. vi. Nuc. Mot. vii. Nuc. sal. sup. Nuc. sal. inf. Trigonum vagi. Nuc. dorsalis x. Nuc. ambiguus. Nuc. xii. colliculus superior, corpus geniculatum Med. collicus inferior. N.iv. Pons. N. V. Nuc. sensor v. Eminentia teres. Nuc. vestibularis. Nuc. cochlearis. Nuc.'fasc. solitarius. Nuc. spinalis v. Nuc. com. cajal. Fig. 47. — Showing the position of the cranial nerve nuclei in the pons-meduUa. (Herrick.) The diseases of the motor branch of the trigeminus are rare, and generally symptomatic of some more general disorder. 106 DISEASES OF THE NEEVOUS SYSTEM Trismus (lockjaw) is the only important independent affection of this motor nerve. It is a tonic spasm of the muscles of mastication. Etiology. — It occurs in infants, usually through infection from the umbilicus. It is then known as trismus nascentium. It forms part of the symptoms of tetanus, tetany and rabies. It may be symptomatic of brain disease, and forms one of the manifestations of the epileptic fit and of hysteria. There may be a reflex trismus from irritations of the teeth and jaw and from gastro-intestinal trouble. It may be associated with a trigeminal neuralgia. Symptoms. — There is, as shown above, a symptomatic, an infec- tious, a reflex, and a hysterical trismus. In all, the symptoms are very manifest. The jaws are firmly locked and the masseters and temporals stand out. If the disease is unilateral, which is rarely the case, the lower jaw is pushed over toward the sound side. The treatment of both tonic and clonic spasms depends upon the cause. Symptomatically, morphine is to be given, and later the anti- spasmodics, such as the bromides and chloral. In rheumatic cases, hot applications and diaphoretics are indicated. THE FACIAL NERVE Anatomy. — The facial nerve has its primary origin in a single nucleus deeply sit- uated in the lower part of the pons (Fig. 48). It belongs to the same series of nuclei as the vagus, glossopharyngeal, and spinal accessory; in other words, it, is a prolonga- tion of the lateral horn of the spinal cord. It receives some fibres from the hypoglossal nerve which supplies the orbicularis oris, in part. The deep facial fibres curve down and out around the nucleus of the sixth nerve (Fig. 48). The cortical origin of the seventh nerve is in the lower part of the prsecentral convokition. The upper branch of the facial which supplies the muscles of the upper part of the face has a cortical origin from each hemisphere, so that a lesion of one cerebral hemisphere does not cause its paralysis. The fibres from the cortex pass down through the knee of the internal capsule and enter the crusta at the inner side of the pyramidal tract. They decussate and reach the nucleus. The nerve has its exit at the posterior edge of the pons, external to the sixth nerve. It has then to take a long course through the internal auditory meatus and Fallopian aqueduct. During its course here it receives communications from the nerve of Wrisberg and from the great superior petrosal nerve. It sends a motor branch to the stapedius muscle in the middle ear and it gives off the corda tympani nerve. The nerve passes out through the stylo-mastoid foramen and supplies all the muscles of the face, the stylo-hyoid, posterior belly of the digastric, the buccinator and the platysma myoid. It does not supply the soft palate or the muscles of mastication. The facial nerve is a mixed nerve and has a sensory part. The fibres of this sensory root arise in the geniculate ganglion. They pass centrally into the medulla to a small nucleus near that of the glossopharyngeal; the peripheral fibres pass in the nerve of Wrisberg and communicate with the motor portion of the seventh. Motor Function. — As already stated, the facial nerve is a motor nerve for the muscles of the ear and face and for the stapedius muscle, a muscle which antagonizes the tensor tympani. MOTOR DISORDERS OF CRANIAL NERVES 107 Sensory Function. — The nerve of Wrisberg and its ganglion contains a few vestigial fibres which supply cutaneous sensation to a small area in the concha and to the posterior wall of the external meatus (J. Ramsay Hunt). Special Sense. — Fibres of the special sense of taste originating in the medulla pass probably in the second and third branch of the trigeminal nerve, thence to Meckel's ganglion, then through the large superior petrosal nerve to the geniculate ganglion, on through it and thence in the trunk of the facial to a point near its exit from the skull; there they leave in the chorda tympani nerve to join with the lingual branch of the fifth nerve and supjaly the sense of taste to the anterior two-thirds of the tongue (Fig. 49). It seems probable that in some cases the fibres for the sense of taste come from the glossopharyngeal through the nerve of Wrisberg and thus pass into the chorda tympani. Secretory. — Fibres stimulating the secretion of saliva run in the nerve of Wrisberg, the geniculate ganglion and the chorda tympani to the corresponding side of the tongue, and when this nerve is irritated or paralyzed there are disturbances in saliva- tory secretion. Fig. 48. — ^Longitudinal section showing position of the nuclei of medulla. (Edinger.) Fibres which stimulate tear secretion pass in the seventh into the geniculate ganglion, thence through the great superior petrosal nerve, and thence to the lach- rj^mal branch of the trigeminus. The connections of the facial nerve with other nerves in its course through the aqueduct of Fallopius make it possible to determine the location of the different lesions which affect the facial nerve in this part. The facial nerve being mainly motor, its diseases are spasmodic and paralytic. The two common types are facial tic and facial palsy, but there are other minor forms. The spasmodic disorders are: (1) diffuse facial spasm or mimic tic and (2) spasm of single branches, including (a) blepharospasm and (b) nictitating spasm. Facial Spasm {Mimic Tic). — :This is a disease characterized by inter- mittent, involuntary twitchings of the facial muscles. It is always chronic and generally unilateral. Etiology. — It is a disease of middle and later life, and occurs oftener in women; there is usually a neuropathic constitution; it is not hereditary. The exciting causes are shock, injury, and local irritations. It often has a reflex cause, usually from irritation of some branch of the 108 DISEASES or THE NERVOUS SYSTEM trigeminus. It is sometimes associated with tic douloureux. It may have an origin in reflex irritation of the sensory part of the seventh nerve, viz., the geniculate ganglion and nerve of Wrisberg. Organic diseases, such as tumors and softening, affecting the nerve nucleus in the pons or the cerebral centres, cause a symptomatic tic, but not the true disease. Thus we may have a post-hemiplegic tic or a tic due to cortical lesion and associated with epilepsy. A facial tic may also be caused by irritation or disease of the nerve trunk or of its nucleus. It sometimes, though rarely, follows a facial palsy. Symptoms.^ — The disease usually begins slowly and the orbicularis muscle and zygomatici are earliest affected. It rarely goes above the <osterior superBcial and deep head and neck muscles (elevating and lateral movements of head). Jeep posterior and anterior back muscles. Trapezius in part (movement of head and throat). , icaleni (elevators of the ribs, breathing), longus colli. Diaphragm (breathing). ![ 'IctoraUsmajortanterior adduction of the upper arm), pectoralis minor, subclavius. „„„„., :.evator scapul». rhomboids (dorsal nerves of the scapula) (draws the scapula inward and "Pwardn "erratus anticus major (long thoracic nerve) fixes the shoulder blade and draws the same in jonjunction with the arm above the horizontal. I ;upraBpinatus (raises and rolls the arm outward), infraspinatus, teres minor (rolls outward). liubscapularis. teres major (rotates inward), latissimus dorsi (adducts the arm and draws it backward). I deltoid (raises the arm to horizontal). „ ,, , u v i-~ Biceps iflexor of forearm and supinator!, brachialis anticus (flexor of forearm), coracobrachialis. Ulloi communis digitorum (radial portion) (bends the terminal phalanges,, flexor longus Polliols end phalanx), flexo? carpi radialis, pronator radii teres, and Q»adratus, palmaris longus, thenar mSsoles (opponents of thumb flex tte primary phalanges and extend the terminal phalanges) lum- bricales 1. 2. seldom 3 (flex the peripheral phalanges and extend simultaneously the terminal &\!e?s^flxten8or of forearm), radial portion of brachialis anticus, supinator longus! (flexes and prinatls the forearm)? exteksor communis digitorum (extensor, of the peripheral, phalanges), extensor pollicis-abductor pollicis. supinator brevis, extensor carpi radialis and ulnaris. . 2^Flexor profundus digitorum (ulnar portion), see 20. Flexor carpi ulnaris. hypothenar. interossel (flex the peripheral phalanx), lumbrioales (3 and 4), see 20, adductor polliois. 9 1 Deep back muscles. j Intercostal muscles. SlDeep back muscles (extensor of trunk). f Intercostal muscles. i Intercostal muscles, abdominal muscles (rectus, external oblique) 'compressor of abdomen). Back (lumbar) muscles. Transverse abdominal, internal oblique- (compressor of abdomen). ] Oremaster, transverse, oblique. t External obturator, adductor of thigh, gracilis (adductor). Psoas (lumbar plexus), iliacus internus (draws up the thigh, flexes the trunk), quadriceps (extends the leg), sartorius. Gluteus medius, minimus (abductors of thigh), tensor vaginae femoris, pyriformis, obturator internus (abductors). Gluteus maximus (extends the thigh). „ ,, , , o , « ■ , jj „4. > Gemelli ; biceps, semitendinosus, semimembranosus (flex the leg), quadriceps femoris (adductor). J Tibialis anticus (raises the inner border of foot), peronei (raise outer border of foot), extensot digiti communis. . „ ,. .i. i.._. i- ^■ Gastrocnemius, soleus (plantar flexion of the foot), flexor digiti, tibialis posticus. I Small muscles of foot (flexor brevis, interossei, etc.). Levator, internal sphincter ani, sphincter vesicae. Sphincter ani, perineal musculature, bulbi cavernosi, etc. External sphincter ani. MOTOR DISORDERS OF CRANIAL NERVES 125 speech and of swallowing. Fuller details will be given under the head of Bulbar Palsy. Progressive Lingual Hemiatrophy. — A progressive hemiatrophy of the tongue sometimes occurs. It is analogous in all respects to facial hemiatrophy, with which it is sometimes associated. It is probably due to a low grade of degenerative neuritis of the nerve. CHAPTER IX NEUROSES OF THE MOTOR SPINAL NERVES Anatomy and Physiology. — ^The spinal nerves arise from the spinal cord by two roots, anterior and posterior. These roots unite outside the spinal canal to form, mixed nerves. The mixed nerves divide and go to their various destinations. There are thirty-three pairs of spinal nerves, viz.: Cervical 8 Dorsal 12 Lumbar 5 Sacral 5 Coccygeal 3 (all rudimentary). 33. The last two coccygeal nerves are microscopic in size, and the first pair is very small, so that practically there are but thirty sets of spinal nerves. The posterior roots are closely connected with ganglia lying in the intevertebral canal, and called intervertebral gangla, or ganglia of the posterior roots. These ganglia are the real origin of the great majority of the fibres of these roots. The mixed nerve is connected by fibres that come chiefly from the anterior root, with the sympathetic or prsevertebral ganglia. The distribution of the spinal nerves is shown in Plate III. For the purpose of conveniently studying the diseases of the spinal nerves, we divide them into six different groups, each having a somewhat definite work to do. These groups are shown in the accompanying table. Strands of Spinal Nerves Distribution Associated Ganglia of Sym- pathetic Group I. Upper four cervical. Group II. Lower four cervical and first dorsal. Group III. Upper six dorsal. Group IV. Lower six dorsal, except last. Group V. Twelfth dorsal, four lumbar. Group VI. Fifth lumbar and five sacral. Occipital region, neck. Upper extremities. Thoracic wall. Abdominal wall, upper lum- bar, upper lateral thigh surface. Lumbar region, upper glu- teal, anterior and inner thigh and knee. Lower gluteal, posterior First to fifth sacral thigh, leg, pelvic organs. First cervical. Second and third cervical, first dorsal. First to sixth dorsal. Fifth to twelfth dorsal. First to fourth lumbar. GROUP I. THE UPPER CERVICAL includes the first four of the spinal nerves. These divide into anterior and jjosterior branches. The posterior branches supply the muscles and skin of the back of the 126 NEUROSES OF THE MOTOR SPINAL NERVES 127 neck and the occiput. The principal nerves are the suboccipital and the great occipital. The anterior branches form the cervical plexus. Its principal branches are the auricularis magnus, occipitals minor, and phrenic. The special distribution of the nerves is shown in the table. (See Appendix.) The upper cervical nerves supply motion to the muscles which rotate the head and draw it back and sideways. One branch, the phrenic, supplies the diaphragm; others supply muscles which assist in fixing the thorax in forced inspiration. They innervate some of the hyoid and thyroid muscles, but have no influence on phonation or deglutition. This group of nerves is in close connection centrally with the trigeminal nerve, whose descending root reaches down into the cervical cord. The fibres to the scalp and face also anastomose with the trigeminus in their peripheral distribution to the scalp and chin. Special Anatomy. — A knowledge of the anatomy and physiology of the neck muscles and nerves is important in studying the more serious neuroses of this region. Muscles Function Nerve Supply _, , j^ . , f Wrinkles the neck, depresses the jaw and 1 „ . , The platysma myoid.. . S , , ,, ,, > Facial. [ angle of the mouth. j Recti capitis antici. . . . Flexion of head. C^ and C^. Recti capitis laterales . . Lateral movement and slight rotation. Antr. branches, C',^,^.? Scaleni med. and post.. Lateral movement and slight extension. Antr. branches, C^, ? „, , ., J Lateral movement; also flexion and 1 „ . , , ^„ oterno-mastoid -, ^ , . . , , ^ . , . , > Spmal accessory and C^ [ rotation of head to opposite side, j Spinal accessory and anterior Trapezius { Lateral movement and extension. , , , , _, , _,, [ I branches of C' and C*. T ^ ,. ,. f Lateral movement and rotation to same 1 . , . , , --,„ , „, Levator anguli soapuli. < . , > Anterior branches, C^ and C*. I side. j o , . ■j_. / x^ 1 , , , 1 1 Ext. div. of post., branches of Splemus capitis < Draws head backward. } 5, ' 1 ( cerv. nerves. C^, C'. ™ , , ... J Draws head backward, turns face to 1 _ , , , „, irachelomastoid < . , „ } Post, branches cerv. nerve, C^. ( same side; J /-.,, f„ 11,, .., -il Post, branches cerv. nerve C, Oomplexus < Draws head back or rotates to same side. > _, _ ^1,. . j Extends and slightly rotates to opposite 1 _ , , i ^, Obhquus supenos <, ■■, , , , ^ • i } Post branches cerv. nerve C. [^ side and bends to same side. J Obliquus inferior Rotates to same side. Post, branches cerv. nerve, C^. Rectus cap. post Extends and rotates to same side. Post, branches cerv. nerve. Ci. The movements oi flexion of the head are thus seen to be due to the sterno-mastoid and anterior recti capitis, innervated by the spinal accessory and by the C^ and C^ anterior branches). In forced flexion the hyoid muscles come into play, innervated by the branches from the descendens and communicans hypoglossi, C^, C*, anterior branches. The movements of extension are caused by the complexus, trachelo-mastoid splenius, obliquus inferior and recti capitis postici and somewhat by the trapezius. These are innervated practically entirely by the posterior branches of the cervical nerves — from C^ to C. The lateral movements of the head are caused by the sterno-mastoid, splenius, scaleni, trapezius, complexus, obliquus superior, rectus capitis lateralis, supplied by the spinal accessory, posterior branches of the cervicals C^ to C^, except the recti laterales, supplied by the anterior branches C^ Extension and lateral movements of the head are mainly done by the spinal ac- cessory and upper three cervical posterior branches. The sterno-mastoid gets some branches from the anterior cervical division C^, as do the recti capitis laterales. 128 DISEASES OF THE NEEVOUS SYSTEM Eolation of the head is caused by the sterno-mastoid, splenius trachleo-mastoid, complexus, obliquus superior and inferior, recti capitis postici and levator anguli scapuli. These are supphed by the spinal accessory and posterior branches of the cervicals C^ to C^, except the sterno-mastoid and recti capitis laterales, which is supplied by the C^ and C^ anterior branches. Hence, after divsion of the posterior branches of the cervical nerves C^ to C* there still remain innervations of the recti capitis laterales and levator anguli scapuli, sterno-mastoid and trapezius. When the spinal accessory is cut these other in- nervations remain and the head may yet be extended, bent laterally and rotated. The muscles which rotate the head to the same side are the recti capitis lateralis (slightly), levator anglui scapuli, trachleo-mastoid (posterior C^), complexus (pos- terior CS C^, C^) obliquas inferior (posterior C^). See diagram, Fig. 56. Antf-Brauck Rectus poslmaj. jliq-sup-ifint Coiiipl.e>ai; Otlic •ifm- , us Sensory branch to _ ::occinu£ CoTTiplexus Splentus TracheloMast Occipital major] Cervical ascend' Trans- colli Complexus Complexus MultifidSpin. Iriterspinales Semispinales Do- Fig. Do- 56.- ■Rectus lateralis C'C^ Recti atiteriorC'C^ SJD iapkrag'?^(Phren.)CV Slerno mastoid C* Levator anq-scap C^ Trapezius C^C* -^ fTrans-ceryical C^C^ ■ z\ Aiiri'ci/la ris Tna^^C^C ^ I*-* '^Supraclavicul-SrcCTJ" Diagrammatic representation of the anterior of the cervical nerves. and posterior divisions Those rotating to opposite side are the sterno-mastoid, obliquus superior (posterior Ci). The diseases of the upper cervical group are spasms, paralyses and neuralgias. Spasmodic Diseases. — ^Torticollis may be limited to and is usually associated with the cervical nerves, as has been shown, though it is usually primarily a spinal accessory neurosis. Tonic spas?n causing a rigid neck is a frequent symptom of meningitis, and forms part of epileptic and other convulsions. In oscillatory and rotatory spasms of the head the cervical nerves are involved. NEUROSES OF THE MOTOR SPINAL NERVES 129 Nodding Spasm, Oscillating Spasm. — ^This is a disorder occurring chiefly in children and characterized by rhythmical nodding or oscillating movements of the head. It occurs, however, in adults. Etiology. — The disease occurs in young children who are ansemic and badly nourished. Dentition, digestive disorders, eye defects, are causes. Sometimes it is only a kind of habit spasm, and occasionally this habit continues during life. It may be associated with or be a pre- cursor of torticollis. It occurs in hysteria, and sometimes in elderly people forming part of a family or senile tremor. Symptoms. — The condition in young children is usually a constant slow, mild, rhythmical, oscillating spasm. It is usually "outgrown." In adults it may assume the chronicity of a torticollis or it may be plainly part of a general senile or family tremor. The diagnosis is easily made by the symptoms. The prognosis and treatment depend upon the etiology. I have found bromide of potas- sium and hyoscine useful in elderly cases, and syrup of iodide of iron in children. The phrenic nerve arises from the third, fourth and fifth cervical, oftenest from the fourth and fifth (37 per cent.). Next from the fourth alone (25 per cent.) and then from the third, fourth and fifth (23 per cent.) and from the third and fourth (15 per cent.) (Schroeder-Green). The diaphragm is supplied by a few filaments of the intercostal nerves, but these cannot take the place of the phrenic and their function is slight (Cavalie). In some forms of stuttering the phrenic nerve is involved in a clonic or tonic spasm. Hiccough is a clonic spasmodic disorder of the phrenic nerve. It is usually due in its casual form to gastric disturbance, with flatulent distention of the stomach. When more chronic, it is caused by hysteria, neuritis, diaphragmatic pleurisy, or some pressure upon the nerve in its course. It is sometimes a prselethal symptom. I have seen cases in which it was probably a pure spasmodic neurosis, a form of tic. Ordinarily, hiccough can be stopped by simple carminatives like spirits of chloroform or lavender or a hot milk punch. In obstinate cases in which no known cause can be found, pilocarpin, hyoscine and bromides are useful. A most effective measure is to lay the patient supine over a thick bolster so that the head hangs back and the thorax arches up. An injection of morphine and atropine promptly stops some cases. Paralysis and Atrophy. — The cervical muscles are paralyzed in anterior poliomyelitis, progressive muscular atrophy, in jjachymeningitis 9 130 DISEASES OF THE NERVOUS SYSTEM hypertrophica, and occasionally in vertebral and peripheral disease or injury and in spinal-cord tumors. Some deformities and weakness in head movements result, but the most serious consequence is involvement of respiration through palsy of the phrenic. Paralysis of the Phrenic Nerve — Etiology. — Such paralysis may be due to disease or injury of the cervical cord and also to peripheral disease, to which the nerve is somewhat liable owing to its long course through the anterior mediastinum. Pleurisy, peritonitis, mediastinal tumors, and multiple neuritis are among the special causes of phrenic paralysis. Spinal-cord dis- eases such as tabes, acute ascending paralysis, and surgical injuries are, however, the commonest etiological factors. Symptoms. — In diaphragmatic paralysis, if bilateral, as is usually the case, the epigastrium and hypochondrium are drawn in in inspira- tion instead of rising. On slight exertion there are dyspnoea and in- crease of respiration. The cough is very weak. Diagnosis.- — If no other muscles than the diaphragm are involved, the cause is probably in the trunk of the nerve. Inflammatory disease of the diaphragm may cause a paralysis which is recognized by its painful character and the febrile reaction. Treatment. — This is to be guided by the cause. It need only be said that there is a motor point in the neck where by careful electriza- tion one can get a contraction of the diaphragm. In paralysis of the phrenic this fact should be borne in mind. GROUP II. THE LOWER CERVICAL NERVES AND BRACHIAL PLEXUS Anatomy and Physiology .^ — The anterior branches of the lower four cervical nerves and first dorsal nerve unite to form the brachial plexus. This gives off short nerves to the shoulder and trunk and long nerves to the arm. The mode of formation of the brachial plexus is shown in the dia- gram (Fig. 57). It is in accordance with the description by Cunning- ham. The short or upper branches supply the shoulder and intercostal muscles. The long or lower branches supply the arm and hand. The neurologist needs to know: (1) the muscular distribution of each nerve and the function of the muscle; (2) the cutaneous sensory distribution; and (3) the level of origin of the nerves. The Plate II, Jakob, Fig. 57, and the table, p. 132, give these points, and will be found useful for study and reference. They are based upon the investigations of Ferrier and Yeo, Thorburn, and also on Abbe's and my own experiments. NEUROSES OF THE MOTOR SPINAL NERVES 131 The Arrangement of the Brachial Plexus. — ^It is made up of three nerve trunks which in turn make up three cords, these cords giving off various branches, thus: 1. Trunk from sixth and seventh cervical roots 2. Trunk from eighth cervi- j cal and first dorsal roots \ 3. Trunk from fifth, sixth, seventh, and eighth cer- ^ vical and first dorsal I forms outer cord, which gives off forms inner cord, which gives off Ext. ant. thoracic. Musculo-cutaneous. Outer head median. Inner head median. Llnar. Int. cutan. Int. ant. thorac. Intercost.-hum. J. _, . , I Subscapular, forms posterior cord, „. „ , . , . ™ i Circumnex. which gives ott , , , . , I Musculo-spiral. N.THORACALIS ANrERIOR LATfRALIS n-CUTANEOOS Fig. 57. — -The brachial plexus. (Cunningham.) The following table shows the origin, muscular distribution, and effect of paralysis on the motor but not on the sensory nerves. This latter is indicated in Figs. 27 and 28. 132 DISEASES OF THE NERVOUS SYSTEM Nerves and Roots of Origin , Muscular Distribution Posterior thoracic. Fifth and sixth cervical. Circumflex. Fifth and sixth cervical. Suprascapular. Subscapular, short. Fifth and sixth cervical. Subscapular, long. Fifth, sixth, seventh and eighth cervical. Anterior thoracic. Musculo-outaneous. Fourth and fifth cervical. Musculo-spiral. Fourth, fifth, sixth, seventh and eighth cervical. Median. Fifth, sixth, seventh, and eighth cervical. Ulnar. Eighth cervical, first dorsal. Serratus magnus. Deltoid. Teres minor. Supraspinatus. and Infraspinatus and teres minor. Subscapulares. Teres major. Latiss. dorsi. Pectoralis major. Biceps and brachialis anticus. Triceps. Supinatores. Extensor carp. rad. Extensor carp. uln. Extensor comm. digit. Extensor p. i. pollic. Extensor s. i. pollic. Extensor oss. met. pollic. Pronatores. Flexor carp. rad. Flexor subUm. dig. Flexor profund. dig. radial half. Two lumbricales. Abductor pollic. Flexor pollic. Flexor carp, ulnar. Flexor profund. dig. ulnar half. Interossei. Two lumbricales. Flexor minor digit. Adductor pollicis. Inner half of flexor brev. pollicis. Function as Shown by Effect of Paralysis Posterior edge of scapula is rotated out when arm is raised and carried forward. Weakening of elevation of shoulder and of inspiration. Loss of power to raise arm. Weakened power to raise arm; head of humerus tends to fall. Loss of abductors; motion forward, and inward rotation of humerus. Loss of rotation of humerus outward. Weakens inward rotation of arm. Weakens power of elevating shoulder. Weakens power to depress shoulder and to pull arm backward and to side. Loss of power to pull arm down and forward and to shrug the shoulder. Loss of flexion of forearm. Loss of extension of forearm. Loss of supination. Extension of wrist lost except when fingers are flexed; lateral deviation. Impaired extension of first phalanges and wrist. Impairment of extension of thumb. Loss of pronation. Weakened flexion of wrist. Weakened flexion of second and third phalan- ges of first and second fingers. Loss of abduction and flexion of thumb. Weakened flexion of wrist; radial deviation. Weakened flexion of second and third phalan- ges of third and fourth flngers. Loss of flexion of first phalanges and of exten- sion of second and third. Diseases of the Lower Cervical Nerve Group and the Brachial Plexus.- — ^The nerves of this group are subject to the pathological dis- turbances common to all nerves. I shall describe them from the clinical side, which gives the following disorders: NEUROSES OF THE MOTOR SPINAL NERVES 133 f Combined arm palsies. Upper-arm type or Erb's paral3'sis. Paralyses , t ^ -r^, , , , . I Lower-arm type. Ivlumpke s paralysis. [ Paralyses of individual nerves. Spasmodic Disorders \ Occupation neuroses f Brachial neuralgia. Sensory Disorders \ Digital neuralgia. JNumb hands (acroparsesthesia). Neurotic oedema. Symmetrical gangrene. Spasmodic Disorders of the Arm. — The arms and hands are es- pecially subject to tremors, choreic and other spasmodic movements. The only spasmodic disorders, however, which may be said to be es- pecially located there are writer's cramp and allied occupation neuroses. These are described elsewhere. Brachial Paralyses, Arm Palsies. — These occur as combined or total-arm palsies, upper-arm and lower-arm types, and paralyses of single nerves. Combined paralysis of the brachial nerves is a condition in which all or nearly all the portions of the plexus and its branches are involved. Total-arm palsies make up about 6 per cent, of all peripheral paraly- ses, and are about one-fifth as frequent as single-nerve paralyses. Etiology. — They occur oftenest in men, but are not rare in infants, being then due to injuries during parturition. After infancy they are most frequent in early and middle life. The exciting causes are obstetrical injuries; wounds, cuts, fractures; deep-seated inflammations and tumors; shoulder dislocations; primary neuritis; crutch and other forms of mechanical compression; hysteria; in rare cases, spinal-cord and brain disease. The symptoms vary with the severity and extent of the lesion. With regard to severity, there are three degrees. In the first there is simply a transient palsy, due to lying too long on the arm. The arm feels heavy, numb and /'asleep." In a few minutes or hours this palsy disappears. In the second degree the nerves are so much compressed as to be mechanically injured. If the patient has been drinking hard, even moderate pressure may set up an inflammatory or destructive process that leads to quite a serious palsy. In the third degree the nerves are actually cut or torn across, or so compressed as to lose their anatomical integrity. The resulting symptoms are those common to all nerve injuries, viz., paralysis, wasting, changes in electrical reaction of the muscles. Pain, tenderness, anaesthesia, trophic, secretory and vasomotor disturbances are also present in varying degree. 134 DISEASES OF THE NEKVOUS SYSTEM The distribution of the paralysis, atrophy, and sensory disturbance depends, of course, upon the arm nerves chiefly involved. The cuts and table will enable one to see in any case where the trouble is localized. It is important to determine whether one is dealing with a total- arm palsy, an upper-arm type (Erb's palsy), or a lower-arm type. In Erb's palsy there is involvement of the deltoid, biceps, brachialis anticus and supinator longus, with at times paralysis of the supinator brevis, infraspinatus and even of all the muscles supplied by the median nerve. The lesion is either in the cord formed by the fifth and sixth Fig. 58. — Upper-arm palsy of left side. cervical nerves or a little lower in the brachial plexus, where the fibres supplying the musculo-spiral, circumflex and musculo-cutaneous lie close together. At all events, the lesion involves the central parts and upper cords of the plexus. The arm hangs by the side and the forearm cannot be flexed (Fig. 58). There are many variants of the upper-arm- shoulder paralysis due to surgical injuries, tumors, passive hanging by the hands by gymnasts, etc. The upper-arm type is especially frequent in infants and constitutes one of the obstetrical paralyses. The trouble is caused through com- pression and tearing of the upper cords of the plexus in difficult labor. Among thirty cases collected by Dr. A. S. Leverty, twenty-eight were NEUROSES OF THE MOTOR SPINAL NERVES 135 difficult head presentations. Sometimes there is a hemorrhage in the cord itself. In the lower-arm type (Klumpke's paralysis) the triceps, the flexors of the wrist, the pronators, the flexors and extensors of the fingers and the hand muscles are involved. The arm can be raised and the forearm flexed and supinated, but the hand is useless and the extension of the forearm is impossible. The lesion here involves chiefly the nerves from the seventh and eighth cervical and first dorsal roots (Fig. 59). If the lesion is in the nerve there will be atrophy, changes in electrical reaction, sensory disturbances, and often, if there is neuritis, pain over the nerves. The reflexes will be lessened or absent. If the lesion is in the spinal cord, symptoms in other parts of the body will be present, or, Fig. 59. — Lower-arm palsy and wrist-drop. if not, there will be no sensory disturbance, as in an arm palsy from anterior poliomyelitis. In rare cases arm palsies may be caused by spinal tumors or a local meningitis, in which case the oculo-pupillary centre of the cervical cord and the sympathetic may be involved. There are three common symptoms, however, of which it is often very important to analyze the cause. These are the loss of power for elevation of the arm and for flexion and extension of the forearm. Flexion of the forearm is performed by the biceps and brachialis anticus, and is helped by the supinator longus. These muscles are supplied by the musculo-cutaneous nerve, except the supinator, which is supplied by the musculo-spiral. Hence when a person cannot flex the forearm, the musculo-cutaneous is chiefly affected. 136 DISEASES OF THE NERVOUS SYSTEM Extension of the arm is done by the triceps, which is supphed by the musculo-spiral. Elevation of the Arm Outward.- — InabiHty to raise the arm is the common and striking symptom in combined brachial palsies. The arm is raised by a number of muscles. The deltoid acts first and most, but it can raise the arm only to a right angle. It is supplied by the cir- cumflex nerve from the posterior cord of the plexus. After the arm is raised to a right angle, it is further elevated by rotating the scapula, and this is done chiefly by the middle part of the trapezius (lower cervical and upper dorsal nerves) and by the serratus magnus, supplied by the posterior thoracic nerve. A number of other muscles combine to strengthen elevation of the shoulder, but this action can be abolished only by paralysis of the deltoid or trapezius and serratus magnus. Fig. 60. — Illustrating the formation of the brachial plexus; also the involvement of the plexus in degenerative neuritis. (Leszynsky.) The diagnosis of these cases involves, first, consideration of the seat of the lesion and the special nerves involved; next, that of the nature of the lesion. A recognition of the seat of the lesion and of the special nerves involved depends entirely upon the study of the distribution of the palsy and of the atrophy and sensory disturbance. The prognosis in arm palsies as a whole is usually good except for the total-arm palsies. Nearly all these cases get well, the duration of the incapacity being from two or three months to a year. Even in the severest cases recovery is possible after one or two years. If, however, the nerves are torn across, as in birth palsies, and the ends widely sepa- rated, recovery is impossible unless an operation is promptly done. The treatment consists in electrical applications, educational move- ments, mechanical support, with potassium iodide internally and absti- NEUKOSES OF THE MOTOR SPINAL NERVES 137 nence from alcohol. Local injections of nitrate of strychnine are useful, and massage should be used if it can be applied carefully. In brachial palsies due to severe injuries, dislocations, fractures, etc., in which there is evidence, from the extreme atrophy and absence of electrical reaction, that the nerve is entirely cut across and that the ends are not in apposition, a surgical operation is stringently needed. The nerves should be exposed and the ends brought as near together as possible. When this cannot be done, the nerves can be split and the ends sutured or anastomoses made, or the two ends are sewed together as Fig. 61. — Double paralysis of circumflex nerve. near as possible and placed in a tube. If the separation is over an inch, not much can be expected. In all these cases, however, it must be remembered that the two ends do not unite, but the central end grows down in the tract of the old degenerated peripheral stem. Paralysis of Single Nerves — Paralysis of the Posterior Thoracic Nerve — Etiology.- — This rare trouble usually occurs in male adults and is due to injury or sudden strains. Its paralysis may be part of a progressive muscular atrophy. The nerve goes to the serratus magnus. Symptoms. — When paralyzed, there is difhculty in raising the arm above the horizontal position and the movements of the shoulder are 138 DISEASES OF THE NERVOUS SYSTEM weakened. When the arm hangs by the side the lower angle of the scapula is drawn a little nearer the vertebral column and protrudes slightly. When the arm is held out horizontally the inner edge of the scapula protrudes and is drawn toward the middle line. When the raised arm is brought forward there is a deep groove formed between the inner border of the scapula and the thoracic wall (Fig. 65). The disease often runs a long course and is accompanied by pain. Paralysis of the Circumflex Nerve. — The nerve goes to the deltoid, teres minor, third head of the triceps and shoulder-joint. It gives sensation to the skin of the shoulder. It is very often paralyzed. The commonest causes are a fall or injury, dislocation and rheumatic inflam- mation of the joint. The arm cannot be elevated or rotated outward (Fig. 61). There are atrophy, anaesthesia and sometimes pain. Paralysis of the Suprascapular Nerve. — The nerve goes to the spinati muscles, teres minor and shoulder-joint. Disease of this nerve alone is rare. The supraspinatus rotates the shoulder in, the infraspinatus and teres minor rotate it out. When paralyzed, there is an impairment of rotation and some impairment of elevation of the shoulder. Paralysis of the Musculo-spiral Nerve {Wrist-drop, Lead Palsy, Compression Paralysis). — ^The distribution of this nerve is given in the table and cut. Its function is to extend and supinate the forearm, to extend the wrist and fingers, and slightly to adduct and abduct the fingers. It extends directly only the last or ungual phalanges, the first and second phalanges being extended by the ulnar nerve. Etiology. — The musculo-spiral, owing to its course, is the most frequently affected by paralysis of all the arm nerves. Pressure on the nerve during sleep — especially when the patient is intoxicated, crutch pressure, fractures, wounds, tumors, lead poisoning, arsenical, alcoholic and other forms of multiple neuritis are the causes of its disordered function. Symptoms. — The symptoms of this paralysis are ''wrist-drop," due to an inability to extend the wrist or fingers. The first and second phalanges can be extended somewhat by the interossei and lumbricales, but the last phalanges cannot be extended at all. The first finger is least affected. The fingers can be only slightly abducted, supination is generally lessened or lost; if the lesion is high up, the triceps is involved and the power of extending the forearm weakened. There may be atrophy of the muscles and degeneration reaction. A swelling over the tendons of the wrist-joint may occur. Some numbness and tingling exist, and occasionally there is anaesthesia in the distribution of the radial nerve on the hand. The disease lasts but a few weeks if due to com- NEUROSES OF THE MOTOR SPINAL NERVES 139 pression; for months if due to neuritis, lead poisoning or severe injury of the nerve. Eventually recovery takes place, as a rule. When the disease is due to lead poisoning there are some peculiarities in its course. Thus the supinator longus usually escapes; the palsy begins gradually and usually involves both arms; it may extend to the upper arm. Partial degeneration reactions are present. There is rarely any ansesthesia and but little pain. Often there is a lead line on the gum and a history of constipation and colic. In alcoholic and other forms of multiple neuritis there are pain and parsesthesia, both arms are involved, and the flexors and other fore- FiG. 62. — 'Wrist-drop from lead palsy. Fig. 63.— (a) Paralysis of radialis and median nerves with anasthesia (b) paraly- sis of radialis. {Leverty.) arm muscles are somewhat implicated. There are marked sensory disturbances. The legs are also affected. In compression palsy the supinators and often the triceps are involved. When the lesion of the nerve is high up, as in crutch paralysis, there is but little ansesthesia, and that is found on the anterior surface of the forearm, in the distribution of the external and internal cutaneous nerves. Lesion of the nerve lower down may give rise to some ansesthesia along the radial border of the forearm and back of hand, but the anses- thetic area varies a great deal. The diagnosis of the paralysis is easily made. The most important point is to find out the cause. The different characteristics of lead palsy, neuritic palsy and compression palsy have been indicated in the 140 DISEASES OF THE NEEVOUS SYSTEM description of the symptoms. One must be sure to exclude also pro- gressive muscular atrophy. The treatment consists of mechanical measures, such as electricity, massage, the application of rubber muscles and in bad cases the fixation of the forearm and hand in hyperextension by means of a splint and plaster-of -Paris bandage (Gibney). Internally in the early stage it is best to administer iodide of potassium and sulphate of magnesium (in lead palsy), the salicylates in neuritis; later, hypodermic injections of strychnine may be given. Static sparks, galvanism and other forms of electricity unquestionably do good in some cases. Paralysis of the median nerve is rare as an isolated trouble, and is almost always due to injury or neighboring disease. When paralysis occurs the grip is weakened. Flexion and abduction of the thumb and flexion of the first and second fingers are impaired. Atrophy of the thenar eminence may occur. The anaesthetic area varies, but is shown in the accompanying cut (Fig. 63). Paralysis of the Ulnar Nerve — Etiology. — The ulnar nerve is rather commonly affected by paralysis, the occurrence ranking next in fre- FiG. 64. — Paralysis of ulnar. quency to musculo-spiral palsy. It is rarely affected in lead poisoning, but is usually early involved in progressive muscular atrophy. It is sometimes attacked by a primary degenerative neuritis. Injuries are the common cause. The symptoms are shown by the table (p. 130). The hand cannot be closed tightly, the little and ring fingers being especially weak. The first phalanges are drawn back and the second and third phalanges flexed; when the interossei and lumbricales atrophy, the result is the "griffin claw" or main en griff e. The fingers cannot be adducted or abducted except feebly. There is anaesthesia over the area of distribution of the ulnar (Fig. 64) ; there may be pain and tenderness. NEUEOSES OF THE MOTOR SPINAL NERVES 141 Migrating neuritis is a serious but rare malady which deserves some mention. It occurs as a sequel to some wound of or operation upon a nerve. The local neuritis extends usually up the arm (ascending neuritis) . It is accompanied by intense pain, anaesthesia, paralysis and atrophy. The disease is very chronic and intractable. It has been relieved in some cases by resecting the posterior spinal roots. Morvan's Disease, Analgesic Pa- ralysis with Whitlow. — (Neuritic type of syringo-myelia.) This is a very rare disease, char- acterized by a slowly progressive pa- ralysis and atrophy of the hands and forearms, with analgesia and painless whitlows. It is probably always ei- ther a form of syringo-myelia or of leprosy. GROUP III. THE THORACIC OR DOR- SAL NERVES Fig. 65. — Paralysis of right poste- rior and thoracic nerve and serratus magnus muscle. Anatomy and Physiology. — The dorsal nerves are twelve in number. The first is the largest and belongs functionally to the arm nerves. The dorsal nerves carry motor and sensory fibres to the voluntary muscles, skin and other tissues of the trunk wall. They carry some splanchnic fibres to the lungs and abdominal viscera. They divide into anterior and posterior branches. The anterior form intercostal nerves, of which the first six are distributed to the wall of the thorax and the last six to the wall of the abdomen. All these nerves give off lateral and anterior branches. The posterior branches of the dorsal nerves are small and supply the muscles and skin of the back. The upper six dorsal nerves, including both branches, are mainly inspiratory in function. They also extend and rotate the dorsal and cervical vertebrae. The lower dorsal are expiratory nerves; they also assist in compressing the abdominal viscera and in flexing, extending and rotating the spine. Motor Neuroses. — ^The thoracic motor nerves are mainly involved in respiratory cramps and paralyses; sneezing, coughing, laughing and crying are symptoms in which they play a large part. In com- plete respiratory paralysis also these nerves are affected. But there are few motor neuroses that are limited to the thoracic nerves. The neuroses of these parts are mainly sensory and will be described later. GROUP IV. THE LUMBAR NERVES Anatomy and Physiology. — The lumbar nerves are five in number. The posterior branches supply the erector spinae, interossei, multifidus spinae and interspinales, and 142 DISEASES OF THE NERVOUS SYSTEM also the skin of the back. The anterior branches of the upper four unite to form the lumbar plexus. The fifth or lumbo-sacral nerve sends most of its fibres to the sacral plexus. The branches of the lumbar plexus are: (1) the ilio-inguinal, (2) ilio- hypogastric (from first lumbar), (3) genito-crural, and (4) external cutaneous (mainly from the second), (5) obturator (from third and fourth lumbar), (6) the anterior crural (from second, third and fourth — Plate II). The first four branches of the plexus are comparatively short and supply sensation WHITE RAMUS TE RAMUS NERVE TO HAMSTRING MUSCLES Fig. 66. — The lumbar and sacral plexuses. {Cunningham.) to the abdominal wall and external genitals. The last two are longer and supply the hip- and knee-joints, the muscles of the anterior inner and outer part of the thigh, the skin over this region and the inner side of the leg and dorsum of the foot (Figs. 27 and 28). The diseases of the lumbar nerves and plexus so far as they form independent disorders are mainly neuralgias. In making a diagnosis of lumbar-nerve disease, one should remember that of the six branches of the plexus the upper four are mainly sensory, the lower two mixed nerves. NEUROSES OF THE MOTOR SPINAL NERVES 143 Paralyses and spasmodic troubles of the lumbar nerves are not rare, but are usually symptomatic of some extrinsic and often serious disease. Etiology. — Hence it is well to catalogue here those affections which may produce lumbar palsies or spasms. They are pelvic tumors or injuries, impacted faeces, caries of the spine, psoas abscess, obturator hernia, hip disease and pressure of the foetal head. Symptoms. — When the upper two lumbar nerves are involved, only sensory symptoms in the distribution of their branches occur. If the next two are also involved, there may be trouble in extending the leg and flexing the hip on the trunk. The patient cannot raise him- self to a sitting posture. If there are irritation and spasm, the thigh is drawn up and adducted. In paralysis of the obturator nerve there are loss of power to adduct the thigh and cross the leg and weakness of outward rotation of the thigh. Anaesthesia over the inner side of the thigh may be present. In paralysis of the anterior crural nerve there are weakness of the muscles of the anterior region of the thigh, loss of power of extend- ing the leg, and anaesthesia or pain over the crural area. Paralysis of the posterior branches of the lumbar nerves causes weak- ness or paralysis of the erectors of the spine. The lumbar curve is very greatly exaggerated, the shoulders being thrown far back and the belly protruding. This condition occurs in progressive muscular dystrophy, particularly in the pseudo-hypertrophic form. GROUP V. THE SACRAL NERVES Anatomy and Physiology. — ^Tlie sacral nerves are five in number. The first four divide into anterior and posterior branches. The fifth has no anterior branch. The posterior branches escape through the posterior sacral foramina and supply the multifidus spinse and the skin over the sacrum and coccyx. The first three anterior branches, with the lumbo-sacral nerve and a branch from the fourth sacral, unite to form the sacral plexus. This lies upon the pyriformis mus- cle in the pelvis, and escapes at the lower part of the sacro-sciatic foramen. The great mass of the fibres go to make up the sciatic nerve. The roots of origin of the sacral and coccygeal nerves form the cauda equina. The branches of the sacral plexus are the superior gluteal, muscular, small sciatic, inferior gluteal, pudic, great sciatic, perforating, cutaneous and articular. These are distributed to the muscles, skin and joints of the buttocks, thighs, legs and feet. The sensory distribution is shown in Figs. 27 and 28. The sacral nerves are the main agents in station and locomotion. They control the legs entirely, also the posterior muscles of the thigh and buttocks; they give sensa- tion to these parts. They carry also fibres that regulate the sexual function, bladder and rectum. From the sacral portion of the cord there is an outflow of nerves to the autonomic system, thence to the pelvic organs. The diseases of the sacral nerves may be classified in a similar way to those of the brachial plexus. 144 DISEASES OF THE NERVOUS SYSTEM Spasmodic Disorders of the Sacral Nerves. — Tremor, rigidity, clonic and tonic spasms, myoclonus, athetoid movements all affect the lower extremities, but they are almost invariably part of some general or central disorder, such as chorea, paralysis agitans, hysteria, etc. Under the head of occupation neuroses there occur certain rare spasmodic troubles special to the legs. Saltatory spasm involves the legs alone. These disorders are, however, general neuroses. Peripheral Leg Palsies. — Paralyses of the lower limbs from disease of the nerves may be either combined or single, just as is the case with arm palsies. A combined sacral palsy is one in which all or nearly all of the branches of the sacral plexus are involved. Etiology. — Such palsies are due to much the same causes as those affecting the lumbar nerves, viz., injury, dislocation, hip disease, tumors and neuritis. Hysteria may cause a functional sacral palsy. Symptoms. — The symptoms are indicated by a study of the dis- tribution of the nerves, varying, however, in degree. The foot cannot be moved; the leg can be slightly extended by the anterior crural, but not flexed; the thigh cannot be extended freely or rotated perfectly. There is anaesthesia over the distribution of the sacral nerves; pain may be present; wasting and vasomotor and secretory disturbances occur unless the paralysis is functional. The course depends on the severity of the lesion. If the nerve is totally cut or torn across, it may require one or two years for perfect recovery which, however, occurs if the severed ends are properly approximated. The diagnosis of a sacral palsy is based on the history and on the distribution of the anaesthesia and of the muscular paralysis. The sacral nerves do everything for the lower limb except extend the leg, flex and adduct the thigh, and to some extent rotate it. They supply sensation equally extensively. The distinction from spinal-cord disease is chiefly based on the unilateral symptoms, the absence of disorder of the sphincters and the combination of paralysis, wasting and sensory troubles in the course of the sacral nerves. Single-nerve Sacral Palsies.— The symptoms of paralysis of single nerves are indicated by their function. The nerves rarely affected are the superior gluteal, muscular and small sciatic. The nerve oftenest affected is the great sciatic, and especially its anterior tibial branch. In the latter case a condition called ''drop-foot" is produced. In the pathology and treatment of sacral palsies there is nothing especial that can be said. CHAPTER X SENSORY NEUROSES OF THE CEREBROSPINAL NERVES As the most common disorders of the sensory nerves are neuralgias and paresthesias, I shall introduce the subject with a general description of these symptoms. PARESTHESIA LOCAL PARESTHESIA ACRO-PARESTHESIA, WAKING NUMBNESS The condition known as paresthesia is one which should be more familiar to physicians and be more commonly recognized and under- stood. Parcesihesia is the name given to a number of subjective sen- sations, such as prickling, numbness, creeping sensations, tickling and burning. It includes, in fact, nearly all the subjective sensations of the skin, except those of pain. It is a condition which is, therefore, extremely common, and in its mildest and most trivial character is much more often experienced than pain. When these sensations fix themselves in a certain locality, following perhaps the tract of the nerve or fastening themselves upon the hand or foot, they take on a certain clinical type, and deserve to have the name of a disease to just the same extent that a neuralgia does. Paresthesia, in almost all cases, implies simply a lower grade of irritation of the nerve-fibres that occurs in neuralgia, and is a kind of ghostly simulacrum of that disease. It very often precedes or accom- panies attacks of pain. There is sometimes a tingling of the teeth or burning in the face which has a shadowy likeness to a toothache or tri- geminal neuralgia. In the same way, one finds paresthesias affecting the head, causing sensations of pressure and constriction, of burning, and general undefinable discomfort, which are entirely comparable to headaches. In conditions of neurasthenia, paresthesias of the head are more common even than the headaches. Paresthesia sometimes follows the course of a nerve, as when one feels numbness of the hand if the ulnar is pressed upon at the elbow, or numbness in the foot where the sciatic is pressed upon, as when the legs are crossed. There is also paresthesia affecting one of the intercostal nerves or one of the crural nerves. On the other hand, paresthesia may affect all four extremities, so that they feel entirely benumbed or prickling. 145 10 146 DISEASES OF THE NERVOUS SYSTEM Parsesthesia is usually peripheral and neuritic, but it may be cerebral and due to cord or brain disease. Etiology. — Parsesthesia, whether local or diffuse, occurs rather more often in women than in men, and rather more often in the mature and middle-aged than in young people. It is most frequent in women of middle age, especially in those who are accustomed to hard work with the hands in washing and the exposure incidental to this. It also occurs in those who are obliged to walk a great deal and to be upon their feet, and it especially affects tailors, seamstresses, bookbinders and those who have to use, their hands constantly in some skilled mechanical work. It sometimes occurs in old age, being accompanied by evidence of gout or by glycosuria. It is associated with rheumatism and with alcoholism. It follows infections like typhoid fever and the grippe, and means in these, as in many other cases, a low grade of neuritis. Parsesthesias which at first are due to some direct or reflex nerve irritation may become a habit and form a part of a psychosis. Par- aesthesias are very common in melancholia and hypochondria. Cerebral paraesthesias occur in tabes, combined sclerosis and in thalamic lesions. Parsesthesia affects single cerebrospinal nerves just as neuralgia does, or it may be more generally distributed. In the latter case it affects often the feet and hands, and it is called acro-parcesthesia. We meet then with: 1. Cephalic parsesthesias, comparable to diffuse headaches. 2. Local parsesthesias, comparblae to local neuralgias. 3. Acro-parsesthesia, involving the feet or hands or both diffusely. 4. Psychic and cerebral parsesthesias. The cephalic parsesthesias are usually symptoms of neurasthenic or depression states. Acro-parsesthesia occurs in multiple neuritis, in glycosuria, and per- nicious ansemia. Local forms affect the ulnar, trigeminal, cervical, sciatic and lumbar nerves oftenest. Under the head of parsesthesic neurosis, the affection known as waking numbness or night palsy may be described. This is a disorder characterized by a temporary paralysis of an extremity, with numbness, noticed on first waking or after lying down for a time. It is a rare disease and little is known of its cause. It occurs in adults and usually in the neuropathic. Sometimes evidence of arthritism, arterial sclerosis or poor innervation of the vessels is present. The symptoms are much like those caused by temporary compres- sion of a nerve when the leg or arm goes to sleep. The paralysis is SENSORY NEUROSES OF THE CEREBROSPINAL NERVES 147 temporary and there is no ansesthesia. It is often a very obstinate condition but leads to no serious result. In some cases there is in parsesthesia undoubtedly a low grade of neuritis, and in other cases there is a congestion or slight degree of degeneration of the nerve. Underlying many of the parsesthesias of middle and late life is an arterial sclerosis or defects of metabolism with nerve degeneration. Many of the cold and heat sensations of this period have this vascular origin, to which is added an undue mental ir- ritability and sensitiveness. NEURALGIA Neuralgia is a condition characterized by pain in the course of a nerve or of nerves. It is not a distinct disease, but only a symptom, Anxiety psychosis Neurasth Eye strain ,/_ Neurasthenia ■--Pelvic trouble Diaphragm Fig. 67. — Diagrams showing the distribution of the cerebrospinal strands of nei'ves and the location of transferred pains and neuralgia. and the tendency is to do away with the term except to indicate a nerve pain. A large proportion of what were formerly called neuralgias 148 DISEASES OF THE NERVOUS SYSTEM are due to neuritis, to some form of organic disease or to some reflex irritation. There is, however, a certain percentage of persistent nerve pains for which we can find no organic basis, and as a matter of both necessity and convenience we still retain the word. We qualify it accord- ing to its cause and its location. Hence we have hysterical, gouty and neuritic neuralgias, and we have trigeminal, cervical, brachial, etc., neuralgias, and we can speak of neuralgias as symptomatic or idiopathic according as we know or do not know the basis of the trouble. But there is little if any idiopathic neuralgia for the observant physician. The most frequent form is the trigeminal; next in order come the brachial and sciatic. Symptoms. — The dominant symptom is pain. This pain is sharp, darting, boring, stabbing or burning in character. It comes on in Strands of Cerebrospinal Nerves Distribution Area 1 Trigeminus, facial, etc. Face and its orifices, anterior scalp. Area 11 Upper four cervical. Occipital region, neck. Area 111 Lower four cervical and first dor- sal. Upper six dorsal. Upper extremities. Area IV Thoracic. Area V Lower six dorsal except last. Abdominal wall, upper lumbar, up- per lateral thigh surface. Area VI Twelfth dorsal, four lumbar. Lumbar region, upper gluteal, an- terior and inner thigh and knee. Area Vll Fifth lumbar and five sacral. Lower gluteal, posterior thigh, leg. paroxysms of great intensity. In the intervals there may be no pain or it may be simply a dull ache. The pain runs along the course of certain nerves, though it is not confined necessarily to them, but may be somewhat diffuse. The skin and nerves are sometimes tender or even exquisitely sensi- tive. Firm pressure, however, is usually not painful. In about half the cases of long standing, tender 'points may be found which correspond to the exit of nerves from a bony canal or the substance of a muscle or fascia. In rare cases there is tenderness over the spine correspond- ing to that point where the affected nerves arise. Besides feelings of pain, there is often a sense of numbness, cold, tingling, or heaviness of the limb. Vasomotor, secretory and trophic disturbances may occur; but when these are pronounced one must suspect neuritis or an organic central disease. Muscular spasm is sometimes present. The paroxysms of pain may intermit regularly; sometimes they come on every day at the same hour. They are apt to be worse at night. The attacks of the disease often run a course of several weeks or months, and in some forms they last for years. SENSOEY NEUROSES OF THE CEREBROSPINAL NERVES 149 The painful sensations of neuralgia usually originate in the periph- eral sensory neuron. In some cases as in the neuralgia of tabes and perhaps sometimes in tic douloureaux and in the herpetic neuralgias, the posterior spinal ganglion is chiefly at fault; in others the irritation affects the entire sensory nerve. The central sensory neurons that take along impulses to the brain are rarely the cause of neuralgia, and local diseases of the cord and brain do not, as a rule, cause pain bj'- irritating these sensory pathways. Still there may be neuralgic pain from this cause; and ''central nervous pains" have been observed in brain tumors and after brain hemorrhage or softening, when these lesions have in- volved the posterior part of the optic thalamus. Pathology. — Many cases that used to be called neuralgia are now known to be forms of neuritis or perineuritis; e.g., sciatica and brachial neuralgia. Other forms are sometimes due to impaired nutrition of the neuron from ah obliterating arteritis (tic douloureux) ; still others are due to the irritation of nerves from the diathetic poison of gout, rheumatism and diabetes, or to extrinsic poisons, such as alcohol, lead and arsenic. In these cases the sensory nerves of the nerve-sheaths (ne-rvi nervorum) are affected. There remain many cases in which the trouble shifts from one locality to another or in which no special local or general irritation can be discovered. In these cases we assume that the pathogenic focus is in the spinal or cerebral sensory neurons and is a toxaemia or a psychosis. There are some forms of neuralgia which may be called "reminis- cent" or "hallucinatory." The patient, who is an impressionable and sensitive person, has had a genuine cause for neuralgic pains; but this, after lasting some time, has ceased, while the painful impressions con- tinue to remain in the cerebral cortex. The neuralgia is a morbid habit of feeling pain. Such neuralgias are promoted often by the use of morphine. There are neuralgic pains occurring oftenest in the legs, but sometimes in the back, shoulder or arms, due to spasms of thickened arteries and causing pain just as the pain of angina pectoris is caused. These are called anginal neuralgias. They are often accompanied with cramping and weakness of the muscles. Pathogenetically we can divide neuralgias into (1) toxic; (2) ex- haustion; (3) neuritic and perineuritic; (4) anginal; (5) psychic. Often there is a combination of these factors. Diagnosis. — This is based on the fact that neuralgic pains are shift- ing, paroxysmal, follow the course of nerves, are accompanied often by tender points and not accompanied by signs of organic nerve disease, such as paralysis, anaesthesia and tenderness over the nerve-trunk. Thermic sensations of burning or cold are rarely neuralgic, but are due to neuritis or some organic change. 150 DISEASES OF THE NERVOUS SYSTEM The treatment will be discussed under special heads. NEUROSES OF THE NERVES OF SPECIAL SENSE. THE OLFACTORY NERVE Anatomy. — -The olfactory nerves consist of a number of peripheral fibres that arise from the olfactory bulb, pass through the cribriform plate of the ethmoid bone, and are distributed to the mucous membrane of the superior and middle turbinated bones and the upper part of the nasal septum. The olfactory bulb is with its associated parts really a subdivision of the brain (rhinencephalon) and not a true nerve. The central olfactory fibres pass to the hippocampus, cornu ammonis, and convo- lution of the corpus callosum. Through cells in these areas they are put in connection with the optic thalamus and with the motor tracts. The olfactory nerves do not decussate. Posterior superior lateral nasal nerve Spheno-palatine ganglion _,.-— Lateral nasal nerve External nasal, nerve Anterior palatine nerve Middle palatine Posterior palatine nerve- Posterior inferior nasal nerve |_Lateral nasal nerve '■■- Naso-palatine nerve Posterior palatine nerve Middle palatine lu i m Anterior palatine ner-\ e Fig. 68. — Showing distribution of olfactory and trigeminal nerves in nose. {Cunningham.) The sense of smell is rudimentary in man, yet it is still the sense by which we can appreciate the most attenuated matter; for the triUionth of a grain of mercaptan is able to awaken a sensation in the mind, but has weight and dimensions so infinitely minute as to be quite beyond the power of the imagination to grasp. According to Valentin, we can percieve K200oooofa grain of oil of roses. According to Fischer and Penzoldt, one can perceive >^760000000 of a grain of mercaptan. 1 have found that one can perceive the odor from 4 cm. of a solution of oil of cloves, 1 to 100,000. Matter to be perceived as odor must be in a gaseous form. Odorous sensations cooperating with taste sensations form "flavor." Variety in odorous sensations depends probably upon the rapidity of molecular vibrations as in the case of light; SENSORY NEUROSES OF THE CEREBROSPINAL NERVES 151 and substances having similar relations in vibration have similarity in odor (Haycraft). Anosmia. — The olfactory nerve is affected clinically by loss of func- tion, or anosmia, increased sensitiveness of function, or hyperosmia, and perversions of function, or parosmia. Anosmia is far the most common disorder of olfaction. Etiology. — Its usual cause is disease of the mucous membrane of the nose. Injuries, inflammations and tumors affecting any part of the course of the nerve, its bulb or central fibers may also cause it. Unilateral cortical lesions in the uncinate gyrus may lead to partial Fig. 69. -Principal constituent elements of the olfactory nerve of a mammal. (Van Gehuchten.) . loss^of smell. It will be not entire, because each nerve receives fibres from both hemispheres. Paralysis of the fifth or seventh nerve may indirectly cause some anosmia. Primary degenerative changes due to syphilis of the nervous system, as in locomotor ataxia, and excessive olfactory stimulation cause anosmia. Anosmia occurs sometimes as a pure psychosis in hysteria, melancholia or in neurasthenic states. There may also be a congenital absence of the nerves. Diagnosis. — This is made by test odors. To test the sense of smell, a bottle of oil of cloves or of some familiar non-irritating odor may be 152 DISEASES OF THE NERVOUS SYSTEM used. To detect quantitative disturbance one may use six phials con- taining oil of cloves, in purity and in watery mixture of 1 to 10, 1 to 100, 1 to 1000, 1 to 10,000 and 1 to 100,000. Special olfactometers have been devised. The sense of smell for any single odor is lost in about three minutes, but returns after one minute's rest. Treatment. — -For functional anosmia, snuffs containing strychnine gr. 3'^o S'l^d gum acacia 5ij can be used. Weak galvanic and faradic currents are recommended. Usually there is in anosmia a local diseased condition of the nose which requires treatment. Hyperosfnia occurs only rarely and then in neurasthenic, hysterical or insane persons. In the latter it is more often a psychical phenome- non than a peripheral disorder. Hyperosmia can be cultivated, and this is done sometimes by the blind and by those engaged in certain pursuits, such as tea tasting and wine tasting. Hallucinations of smell occur in the insane, as just mentioned, and a few cases of epilepsy are reported in which the aura was a stench. In tumors of the temporal lobe irritating the olfactory cortical centre, "stench seizures" sometimes occur. When all olfactory sensations are disagreeable the condition is called kakosmia. Parosmia is a not infrequent condition. In it everything smells alike to the patient, or a pleasant odor is perhaps a peculiar or offensive one. This condition may be due to local disease, but is often a symptom of hysteria or the psychoses. THE OPTIC NERVE Anatomy. — The optic nerve is not a true peripheral nerve, but a tract of the brain, and it connects the retinal cells with the brain proper. Like other brain tracts, its fibres have a myelin sheath, but no neurilemma. The real origin of the nerve is in the retina, just as the olfactory nerve arises in the peripheral cells of the olfactory mucous membrane and the spinal sensory nerves arise in the spinal ganglia. The retina is a nervous tissue formed essentially of three layers of nerve-cells. From without inward they are: the layer of visual cells, the layer of bipolar cells and the layer of ganglionic cells. These different layers may be subdivided so as to give the following layers from without inward: 1. The layer of rods and cones. 1 „ • xi i j- • i n _ „, , , , , f Jbormmg the layer oi visual cells. 2. ihe external granular layer. J 3. The external molecular layer. 1 „ • ^^i i i- i • i n . T J 1 11 ^ l^ormmg the layer oi bipolar cells. 4. Internal granular layer. J 5. Internal molecular layer. 1 Forming the layer of 6. Ganglionic layer, with the fibres of the optic nerve. J ganglion cells. The layer of visual cells is subdivided, as seen in the figure, into that of the rods and cones externally and that of the external granular internally. This is, however, practically a layer made up simply of bipolar nerve-cells with prolongations more or SENSORY NEUROSES OF THE CEREBROSPINAL NERVES 153 sualjield less long which run to the external surface of the retina and there form a series of bodies known as the rods and cones. In the layer of bipolar cells are layers of cells with processes running horizontally, and in the internal molecular layer are larger horizontal cells, called by Cajal spongio- blasts. There are also in the retina terminal arborizations of cells whose origin is in the thalamus, corpora geniculata, or anterior tubercles. These carrj^ impulses to the retinal cells. The neuraxons of the ganglionic cells send fibres which unite to form the optic nerve. The optic nerves each contain about 500,000 fibres, which is about the same as the total of all the sensory fibres of the spinal nerves. They pass to the optic chiasm, where about one-third of the fibres cross, in man. In lower animals the decussation is greater. Those fibres which do not cross come from the outer or temporal third of the retina; those which do cross come from the internal or nasal two-thirds. A few fibres pass from one optic centre of the brain along the poste- rior border of the optic chiasm to the centre of the opposite side (commissure of Gudden). After leaving the chiasm, the fibres form the oftic tract. The tract curves up and back around the crus cerebri, and divides into a lat- eral and mesial root. These roots connect with the external geniculate body, the anterior tubercles of the corpora quadrigemina and the posterior gan- glion of the thalamus, i.e., the pulvinar. These ganglia are called the primary optic centres. Through the anterior tubercles of the corpora quadrigemina and by other means, the optic nerve is connected with the oculo-motor nerve, and thus reflex movements of the pupils, lids and eyeballs are brought about. From these primary optic centres, fibres enter the posterior part of the internal capsule, curve up and back toward the occipital lobes, forming the optic radiations of Gratiolet. The inferior longitudinal bundle is part of these radiations and connects the external geniculate body with the calcarine cortex (A. Meyer). They are finally distributed to the cortex of the occipital lobe, and in man chiefly to the cuneus and the parts about the calcarine fissure. It will be seen that each retina is connected with the occipital lobe of both hemispheres; further, that the outer or temporal half of each retina is connected with the occipital lobe of the same hemisphere, and the inner or nasal half of each retina with the occipital lobe of the opposite side. The upper part of each retina seems to be connected with the lower part of the cuneus, and vice versa (Fig. 70). Other associations exist by which the optic centres on the two sides are connected with each other and with other cranial nerves in the medulla. The optic nerve is a nerve of special sense of vision and has no other function ex- cept that of an oxcito-reflex character. Fig. 70. — Showing relation of occipital cortex and its visual cen- tres to the eye and visual fields. {Bing.) 154 DISEASES OF THE NERVOUS SYSTEM DISEASES OF THE OPTIC NERVE The optic nerve may be affected by nearly all forms of pathological change. For the neurologist, however, the especially important con- ditions are inflammations, degenerations, injuries and functional dis- orders. Inflammation of the nerve, or optic neuritis, may occur as a papillitis or inflammation of the head of the nerve, a neuro-retinitis or descending neuritis, or a retro-bulbar neuritis. Perineuritis is rare. Neuro-retinitis and papillitis are closely associated clinically and patho- logically (Noyes), so that practically only two forms of neuritis need be discussed separately. Papillitis and Neuro-retinitis — Etiology. — This condition is seen by '. .>^ J y A First DorsaL ^- 7'oi7:^oof. "In^Jicot. M Fourtli SacraL •/?./ / Jh^tTluoT. - --;^/i'/ ;?Qaf- Eighth Dorsal. First Sacral. Fig. 90. — Showing the arrangement of the gray and the white matter at different levels of the cord, also the columns and cell groups. 206 DISEASES OF THE NEEVOUS SYSTEM The direct cerebellar tract begins at the level of the first lumbar nerve. Its fibres originate in the vesicular column of Clark. They pass up to the cerebellum and go chiefly to the cortex of the vermis without crossing. Th.e\entral cerebellar tract, or column of Gowers, extends nearly the whole length of the cord. Its fibres come from the anterior commissure and the sensory cells of the opposite posterior cornu. They pass through the medulla and pons, turn and enter the cerebellum to end in the cortex of the vermis. A few fibres pass directly to the optic thalamus. The rubrospinal tract originates in the red nucleus; it crosses to the opposite side, passes down through the pons and medulla, runs in the lateral column of the cord forming a triangular or wedge-shaped tract along the anterior or ventral part of the Olive bundle Goll's column. Comma bundle Burdach's column Root-zone Lissauer's zone. Septo-marginal . Dorsal spino- cerebellar tract (Fleschsig). Lateral pyram- idal tract. Area of rubi o- spinal trac t Ventral spino- cerebellar tr (Gower's). Spino-thalanuc tr. Vestibulo- spinal tra( t Lateral funda- mental column Spino-olivary tract' (Helwig) . tecto-spinal tract. | tecto-spinal tr. direct pyramidal tract. Fig. 91. — Showing the tracts and columns of the spinal cord. Descending tracts are in red; ascending in green. The vestibulo-spinal is not as extensive as indicated and lies mainly in the ventral column. The rubro-spinal tract is not as sharply de- fined as indicated. crossed pyramid tract. It extends as far down as the sacral cord. Its fibres end in the anterior horns. The vestibulo-spinal tract arises in Deiter's nucleus, crosses and runs in the lateral column of the cord in front of the rubro-spinal. It may extend to the anterior column. It passes down as far as the lumbar cord. Its fibres end in the anterior horns. The tecto-spinal tract originates in the corpora-quadrigemina and takes a similar course to the preceding tract, a median part running in the anterior and a lateral in the lateral columns. Several other tracts are described. These together with the above three small tracts degenerate downward and are spoken of collectively as the extra-pyramidal tracts. , DISEASES OF THE SPINAL CORD 207 The posterior columns are made up of fibres that come from the posterior spinal ganglion and pass directly into the posterior columns, then ascend to nuclei near the medulla where they end. These fibres are called exogenous. The columns also re- ceive some fibres from the tract cells in the posterior horns; and they are called endoge- nous fibres. The exogenous fibres in the sacral lumbar and lower dorsal cord pass inward and form the postero-internal column or GoU's column. This is then small below and larger in the cervical region. They end in the nucleus of GoU's column (nucleus gracilis). The exogenous fibres in the upper part of the cord pass up, tying to the outer side of the column of Goll. They form the column of Burdach and end in the nucleus of Burdach (nucleus cuneiformis). Fig. 92 I. Showing the connections of the anterior and posterior roots and cornua with each other (Cajal). A, anterior root; B, posterior root; a, collaterals; d, end-brushes. II. Showing the association or short-fibre system of the cord (Cajal). a, anterior cornua cell; b, c, d, association fibres; e, posterior association fibres. The column of Burdach is thus not distinctly formed as a long fibre tract except in the upper part of the cord. The names of the short-fibre columns are the anterior and the lateral fundamental columns, the lateral limiting layer and the lower part of the column of Burdach. The posterior columns also contain three short-fibre columns whose cells of origin lie in the gray matter of the cord. The fibres in the cervical region lie in the shape of a comma (comma of Schultze), in the lumbar region in the shape of an oval (oval zone of riechsig), in the sacral zone in the shape of a triangle (triangle of Gombault). Besides this there is a zone of short fibres lying close to the gray commissure the whole length of the cord — ventral zone or posterior fundamental column. 208 DISEASES OF THE NERVOUS SYSTEM The Relations of the Different Parts of the Spinal Cord to the Peripheral Nerves, and with Each Other. — I will begin with a description of the way in which the anterior and posterior nerve-roots are conected to the cord; then describe the mode in which the different columns and cell groups are connected with each other; and finally I will indicate briefly the connections of the cord with the brain. The anterior nerve-roots are connected directly with the anterior-horn cells, of which they are processes, and together with which they form the peripheral motor neurons. It is possible that in man they send off collaterals before leaving the cord. The cells of the anterior horn are surrounded by two chief sets of "end brushes," one coming from the pyramidal and other descending tracts, the other from the pos- terior horns and roots. Thus these cells are in relation with impulses from the brain and from the periphery. The posterior nerve-roots originate in the cells of the spinal ganglia. These give an axon which divides and sends off a peripheral branch to form the sensory nerve, and a central branch which passes into the .spinal cord forming the posterior root. On entering the cord the fibres of the posterior root divide like a T and pass up for one or more inches and down for a short distance only. These root-fibres, with their cells of origin and the sensory nerve-fibres, form together the peripheral sensory neurons. The peripheral sensory neurons send their axons into the spinal cord through the posterior roots, and these axons divide, run up and down the cord for a short or long distance and then end in terminal arborizations about different groups of cells. 1. Some pass directly into the columns of Goll or Burdach on the same side and pass up to end in the nuclei of these columns. 2. Some pass to cells in the posterior horns of both sides and there end. 3. Some pass to cells of the column of Clark of the same side. 4. Some pass to cells of the anterior horns of both sides. ^ 1 There are, according to Ingbert, about 650,000 sensory fibres in the posterior spinal roots; each cutaneous fibre supplies from 1 to 3 sq. mm. of surface. The different parts of the spinal cord are connected by the short fibres which unite different levels of the cord, and by commissural fibres which unite the different halves of the cord. These short and commissural fibres originate in groups of nerve-cells lying in the central parts of the gray matter and which we have called column nerve- cells. They are small and multipolar. Some are distributed sparsely in the white columns. Fibres arise from them, run in the commissures and short-fibre tracts, and end in brushes which put the fibres in relation with various cell groups. DIAGNOSTIC PHYSIOLOGY General. — The spinal cord is made up of (1) peripheral motor and sensory neurons; (2) the upper or encephalo-spinal neurons; (3) short associative neurons. Lesions of the peripheral motor neuron cause motor irritation or a severe, flaccid paralysis and atrophy. Lesions of the peripheral sensory neurons cause pain, paraesthesia, ataxia and anaesthesia and secondary trophic troubles. Lesions of the upper or cerebrospinal neurons cause a spastic paralysis without atrophy. Lesions of the secondary sensory neurons cause little pain or sensory irritation, but lead to anaesthesia paraesthesia, ataxia, etc. The relations of the spinal cord to the brain will be described in connection with the anatomy of the brain. Peripheral Palsies ; Radicular or Root Palsies ; Nuclear Palsies. — If the nuclear groups of motor cells in a single segment of the spinal cord are destroyed, e.g. the fifth cervical segment, we get a partial paralysis of the muscles supplied from this segment, because these nuclear groups overlap and are not each assigned to a single segment. DISEASES OF THE SPINAL CORD 209 If an anterior root is cut across, we also get a partial paralysis, because muscles generally are supplied from several different roots as well as from several different segments. Hence a single segment or root palsy is rarely a complete one. On the other hand, a section of a motor nerve may cause a complete paralysis of a muscle or group of muscles. Hence a ■peripheral palsy may be complete. If the entire nuclear group of cells controlling a muscle-group is destroyed, as happens in anterior poliomyelitis, we also get a complete paralysis — nuclear palsy. Sensory Ansaethesia from Peripheral, and Radicular Lesion. — In the same way we get peripheral anesthesia over a def- inite cutaneous area when a peripheral sensory nerve is cut. When one posterior root is cut we got no anaesthesia; when, however, two or more are cut we get bands of anaesthesia known as radicular or segmental zones. These have been determined by animal experiment and operations and disease, so that we have charts showing the radic- ular zones produced by cutting two pos- terior roots at different levels. They are marked singly for purposes of con- venience, only (see Figs. 27 and 28). Automatic Centres. — The nerves and cells of the cord are arranged in com- plex groups which preside over certain functions or respond in a definite wa:y to certain stimuli. These are called the spinal automatic centres.. They are the cilio-spinal, secretory, vasomotor, genital, vesical and rectal.' The important parts of these centres lie deep in the gray matter on either side of the central canal, but nearer the base of the posterior horns. Lesions of the white matter or of the an- terior or posterior horns do not directly affect them. The cilio-spinal centre lies in the eighth cervical and the first dorsal segment. Its stimulation causes the pupils to dilate. The genital centres, including those for erection and ejaculation, reach from the first to the third sacral segment, inclusive. The bladder and rectal centres are in the fourth and fifth sacral segments, extend- ing up and down a short distance, the bladder being perhaps a little lower. The spinal vasomotor centres extend from the second dorsal to the second lumbar segments. The vasodilator nerves pass out by the anterior, the constrictor by the posterior root (Gaskill). The cells of the posterior horns are sensory in function and are connected with the tactile, pain, temperature and reflex fibres of the posterior roots. 14 Fig. 93. — Showing the relation of the spinous processes to the points of origin in the cord of the spinal nerves. The nerves are shown in black lines as they come from the spinal canal. The dotted lines show the level at which each nerve arises m the cord. 210 DISEASES OF THE NERVOUS SYSTEM Topography and Localization. — The neurologist and surgeon need to know, for purposes of diagnosis: 1. The relation of the spinal nerve-roots, at their point of origin, to the spinous processes. This is shown in Fig. 93. In general it will be seen that the different pairs of nerve-roots arise opposite the spinous process of a vertebra one or two seg- ments above those between which it makes its exit. Thus the sixth cervical originates opposite the fourth cervical spine, the sixth dorsal between the third and fourth dorsal spines, the first lumbar between the eleventh and twelfth dorsal spines. There is considerable variation in these relations. 2. The next point desired is the special function of each pair of nerve-roots anterior and posterior, and the level of the various centres in the cord. This is shown in the following table, based on that originally devised by Starr, modified by Mills, Sachs and myself from personal experiments and the clinical and pathological ob- servations of Thorburn and others. (See Appendix.) The blood-supply of the spinal cord is a subject of great practical importance; and I shall present the matter here in some detail. The spinal cord is supplied with blood by branches from the vertebral, ascending cervical and superior intercostal arteries above, and by the dorsal intercostal, lumbar and sacral arteries below. These send off small branches which enter the spinal canal through the foramen magnum above and the intervertebral foramina at the sides: they pierce the dura mater and are distributed on the pia mater and in the cord. The arteries that thus supply the cord are these : Primary Arteries Origin from Ending in Anterior spinal . . . Vertebral (from subclav.) Anterior median spinal artery. Posterior spinal . . Vertebral. Lateral spinal .... Vertebral. Lateral spinal .... Ascending cervical (from sub- Anterior and posterior spinal clav.). root arteries. Lateral spinal. . . . Superior intercostal (from sub- Anterior and posterior spinal clav.). root arteries. Lateral spinal .... Thoracic intercostal (from Anterior and posterior spinal aorta). root arteries. Lateral spinal .... Lumbar (aorta). Anterior and posterior spinal Lateral sacral (from int. iliac). root arteries. The anterior spinal arteries are branches of the vertebrals. They unite to form the anterior median artery, which runs down the whole length of the cord, receiving re-enforcements from the lateral arteries (Fig. 94). The anterior spinal arteries themselves nourish only a few upper segments of the cord. The anterior median artery is not, as has been taught, a true prolongation of the anterior spinals, but is really made up by the lateral spinals. In other words, the vertebral artery through its branches nourishes only the upper cervical region of the cord. The posterior spinal arteries are smaller than the anterior and unite on the posterior surface of the cord. They do not continue down as a posterior median artery — ^there is no such artery; but they help to form two plexuses on the postero-lateral surfaces of the cord. The lateral spinal arteries are derived from branches of the subclavian artery as far down as the second dorsal root; below this point by the thoracic and abdominal aorta and the internal iliac. It is an interesting fact that at or a little below the point DISEASES OF THE SPINAL CORD 211 where the blood-supply changes from the subclavian above the heart to the aorta below, pathological disturbances frequently occur (transverse myelitis). "^^ ^J'k ^^^ , '^.:^ sl^- Fig. 94. — The spinal cord, anterior surface, showing the nerve roots, root arteries, and anterior plexus. (Kadyi.) Root Arteries. — The lateral spinal arteries, after they enter the spinal canal, are called the root arteries. They pierce the dura mater and pass, some along the posterior and some along the anterior roots, to the cord. There are about eight 212 DISEASES OF THE NEEVOTJS SYSTEM anterior-root arteries (five to ten) and about sixteen posterior-root arteries (see Figs. 94, 95). The anterior arteries are twice as large (1 mm. in diameter) and one-half as numerous as the posterior. The root arteries of the cervical region are rather the more numerous. There is a large and constant anterior-root artery in the dorso-lumbar region. The last two lumbar, the five sacral nerves and the unpaired coccygeal nerve when it exists, are accompanied by small root arteries which do not reach up to the cord itself. The lower part of the spinal cord is supplied by large root arteries from the lateral spinal arteries. Hence, the theory of Moxon that the circulation here is feeble is not supported by Kadyi's investigations. The Plexuses. — The anterior root arteries pass to the anterior median fissure, and then divide, partly to form the anterior median artery and partly to form a rich plexus between the anterior roots; this is called the anterior arterial plexus . The posterior root arteries subdivide before they reach the cord, and send twigs to its lateral and ^ B Fig. 95. — -The arterial supply. A, The artery of the posterior fissure; B, the interfunicular; C, artery of posterior horn; D, of posterior root; E, of postero-lateral column. posterior surfaces which form the postero-lateral arterial plexus. The posterior-root arteries do not anastomose to any extent with each other or form a posterior spinal artery, as is done by the anterior-root arteries. There are, therefore, three relatively independent arterial plexuses : the anterior plexus, the two postero-lateral plexuses. Veins. — The veins of the spinal canal outside the dura mater have valves, those within it have none. The veins reach the pia mater and cord by passing along the nerve-roots. Hence, we have anterior and posterior root veins, corresponding to the root arteries, but more numerous, there being a total of forty or fifty. The anterior- root veins are more numerous than the posterior, but smaller (twenty five to tweiity). The veins are a little larger than the arteries, the anterior veins being 3^^ to 1 mm., the posterior IJ^ to 2 mm., in diameter. Thus we see that the posterior surface of the cord has more and smaller arteries, fewer but larger veins. The posterior surface is on the whole more richly supplied 5 to 10 1 mm 25 to 30 3-2 to 1 mm 16 3^^ mm 20 to 25 \}y-2 to 2 mm DISEASES OF THE SPINAL CORD 213 with veins, the anterior surface with arteries. The lateral surfaces are the least vascular. Anterior root arteries Anterior root veins Posterior root arteries Posterior root veins Vessels of the Cord Substance. — The cord is supplied by (1) central arteries which are branches of the anterior median, and by (2) peripheral arteries which come from the plexuses on the pia mater. These two systems have been called also the centrif- ugal and centripetal, respectively. They are not absolutely independent, but are in a good measure so. The central arteries nourish chiefly the gray matter, the per- ipheral arteries the white. Both systems are made up of "end arteries;" i.e., they do not anastomose with each other. Neither the central nor the peripheral arteries are distributed in accordance with anatomical relations or physiological functions. Each cell group, for example, has a vascular supply from several sources. The central arteries are given off from the branches of the anterior median at the bottom of the median fissure and number about two hundred, each spinal segment having six or seven. The accompanying central veins are small and their total capacity is less than that of the arteries, so that the central arterial pressure must be high, on account of the poor venous outlet (Kadyi). Some of the blood escapes by the peripheral veins. The peripheral arteries pass into the spinal cord for the most part along the various connective-tissue septa. There they branch and supply chiefly the white matter. They supply the apex and some of the deeper substance of the posterior horns and Clark's columns. The arteries of the posterior septum are the largest and most numerous, often reaching to the gray commissure. The peripheral arteries are smaller than the corresponding veins (0.04 to 0.2 mm.). The relation is just the reverse, therefore, to that of the central arteries and veins. The peripheral arteries are small, and after passing into the cord branch into minute vessels which pass up and down and soon become capillaries. The central arteries, on the other hand, continue large, and run up and down some distance before they are subdivided into capillaries. To sum up: The arteries predominate in total capacity in the anterior plexus and central arteries; the veins in the posterior plexuses and peripheral vessels. The central arteries are larger and longer than the peripheral. Hence the blood circulates more quickly and under greater pressure in the central gray of the cord. Conditions of enfeebled circulation would affect the posterior columns and roots more than the anterior and central parts of the cord. CHAPTER XII THE DISEASES OF THE SPINAL CORD There are about thirty diseases which may be classified as belonging to the spinal cord. Most of these are organic in character and come under the head of inflammatory and degenerative or system diseases. Func- tional disorders referable to the cord alone are rare; while of organic diseases, those that result from injury, syphilis and inflammation are the most common. The special diseases of the spinal cord are the following: 1. Malformations. — Myelocele, meningo-mylocele (spina bifida), men- ingocele, heteropia, amyelia, micromyelia, macromyelia, double cord. 2. Vascular Disorders. — Anaemia, hypersemia, hemorrhage, endarter- itis with aneurism, embolism or thrombosis, oedema. Secondary to these conditions are softenings and sclerosis. 3. Inflammations. — Meningitis, myelitis, multiple sclerosis. Second- arily, softenings, sclerosis. 4. Degenerations or Parenchymatous Inflammations. — Primary: pos- terior and combined scleroses, hereditary scleroses, progressive muscular atrophy and allied types. 5. Tuberculosis.- — Miliary, solitary, and meningeal, causing 2, 3, 7. 6. Syphilis. — Which may cause 1, 2, 3, 4 and 7. 7. Tumors. 8. Functional and toxic disorders. MALFORMATIONS Spina Bifida (Rhachischisis Posterior) Spina bifida is a congenital hernia of the spinal membranes, and sometimes of the cord, through a cleft in the vertebra caused by absence of the vertebral arches. It is often really a malformation of the vertebral canal rather than of the cord, though both conditions may be present. Etiology. — The condition is not very rare, about one child in 1,200 (French statistics) being affected. It is often associated with hydro- cephalus or with some other defect in development, such as ventral hernia, imperforate anus or pharynx. Hereditary influence is sometimes a factor. It is a true developmental defect, and is not due to a primary dropsy of the cord, as was once taught. It occurs rather oftener in females. 214 DISEASES OF THE SPINAL CORD 215 Forms. — There are three varieties described : 1. Spinal meningocele is a condition in which the spinal membranes alone protrude into the sac. 2. Spinal meningo-myelocele is a form in which the membranes and cord both protrude. 3. Syringo-myelocele (hydrorrhachis interna) is a form in which the fluid is in the central spinal canal, and the inner lining of the sac is formed by the meninges and thinned-out spinal cord. Anatomy. — -The first two forms are the most common and are called hydrorrhachis externa. The fluid here lies in the subarachnoid sac, and hence the wall of the protruding cyst is lined with the dura and arachnoid. The nerves and cord protrude into the sac in two-thirds of the cases (forming a meningo-myelocele), but in some of these only a few nerves are found. These structures, when present in the sac, as in men- a hca a bca a bca Fig. 96. u a u a u a, -Meningocele. Meningo-myelocele. Syringo-myelocele. walls; 6, cord; c, membranes. a, Vertebral ingo-myelocele, lie on its posterior and median surface. They are attached to and form part of the wall. The spinal nerves therefore start from the wall of the sac and go back into the vertebral canal. The tumor con- tains cerebrospinal fluid, and occasionally connective tissue and fat (Fig. 97). The external surface is often red and smooth, and there is some- times a depression on its median surface where the cord is attached. Symptoms. — Spinal bifida occurs almost always in the lumbar and sacral region, the reason being that the laminae here are the last to solid- ify. Usually but two or three vertebrae are involved. The tumor varies in size from 3 cm. (1 in.) to 15 cm. (6 in.) in diam- eter, and may have a broad base or be pedunculated. The outer skin is often glossy, or tough, thickened or ulcerated. Children with spina bifida are usually feeble, badly nourished and poorly developed mentally. Paraplegia occurs in half the cases, sometimes with anaesthesia and involvement of the sphincters. Talipes occurs quite often. 216 DISEASES OF THE NEEVOUS SYSTEM The prognosis is grave. Most subjects die unless treatment is applied, and even then the prospect is not very good. The prognosis is best for meningocele. The diagnosis is easy. It is generally only necessary to exclude con- genital tumors which happen to be located in the lumbo-sacral region. The most important question to decide is whether the cord and nerves are present in the sac. This may be assumed as probable if there is much paraplegia, anaesthesia and sphincter trouble, and if there is a depression on the median external surface. The introduction of an insulated needle connected with an electric battery may be tried. The treat?nent is strictly surgical, and then is of avail only in meningo- cele. Injections of Morton's fluid (iodine, gr. x.; potas. iodid., gr. Fig. 97. — Spina bifida. XXX.; glycerin, §i. Dose, §i) have been successful. These injections should be made in the lateral portion of the sac, and the child should be kept on the back. Puncture and withdrawal of fluid with compression is not a justifiable operation. Ligaturing or opening and excising of the sac are dangerous, especially if, as is often the case, part of the cord and nerves lie in the sac. In recent years, surgical results have been more favorable and warrant serious consideration. No surgical treatment should be attempted, however, until two or three months after birth, OTHER MALFORMATIONS Heteropia is a rare malformation in which masses of gray matter are found in abnormal situations. A false heteropia may be caused by manipulation of the cord in its removal after death. The displaced masses consist of nerve-cells or neuroglia. Amyelia, or absence of the spinal cord, can exist only when the brain is absent; but absence of the brain may occur without absence of the DISEASES OF THE SPINAL CORD 217 cord. In amyelia the spinal nerves are usually present. Amyelic monsters cannot live. Double cord is a very rare defect and involves only part of the cord except in cases in which there is a double vertebral canal. It occurs sometimes in connection with spina bifida. Double central canal is not rare. It usually involves only a part of the cord. The two canals are side by side. Asymmetry of the cord, usually due to abnormality in the course of the pyramidal tracts, is not extremely rare. Splitting of the cord and defects in development at special levels are occasionally observed. Micromyelia is a condition in which the spinal cord is abnormally short or small in size, and is not a very rare anomaly. The normal adult cord has a diameter in its various parts of 6 to 9 mm. (dorsal), 8 to 11 mm. (upper cervical), 15 mm. (cervical swelling), and 12 mm. (lumbar). SPINAL HEMORRHAGE (SPINAL APOPLEXY) This general name may be given to (1) spinal meningeal hemorrhage or hsematorrhachis, and (2) hemorrhage into the cord substance, or haematomyelia. 1. Spinal meningeal hemorrhage is far the most common form. It may be outside or inside of the dura, the former being rather oftener seen. Etiology. — It occurs in newly born children and in adults, and is more common in men than in women. Injuries, falls, fractures of the spine are the most frequent exciting causes. Severe convulsions from epilepsy, eclampsia, tetanus, chorea or strychnine and even severe muscular exertion may be a cause. Childbirth, purpura and the blood states following malignant infectious fevers, bursting of an aortic or vertebral aneurism, and cerebrospinal meningitis are rare causes. Symptoms. — In small hemorrhages there may be no symptoms. In large effusions there are sudden very severe pains in the back, extend- ing into the limbs with numbness, tingling, hyperesthesia and mus- cular spasm, especially of the back muscles. Later there may be weak- ness or paralysis and anaesthesia of the extremities, with disorder of the visceral centres. The reflexes are exaggerated. The distribution of the anaesthesia may be segmental unless the hemorrhage is very large. The symptoms reach their height usually in a few hours. Then ameliora- tion may occur, followed by slow recoveiy or with symptoms of chronic meningitis. Rarely death occurs early from exhaustion. Diagnosis. — A history of injury or childbirth, sudden onset of attack, with symptoms of pain and meningeal irritation with rigidity which rather rapidly subside, point to extra-dural hemorrhage. In haemato- myelia there are less pain and irritation, but more profound paralysis and 218 DISEASES OF THE NERVOUS SYSTEM anaesthesia. The same is true of crush of the cord from fracture or dislo- cation. In tetanus there is a slower development of the symptoms and trismus is present. Lumbar puncture will assist the diagnosis. The prognosis is grave in severe cases, but if the patient survives three or four days the prospect of partial or nearly complete recovery is good. The treatment is perfect rest in bed and the administration of remedies to move the bowels and relieve pain; leeches and other local applications are of doubtful value. If there is distinct evidence of fracture or disloca- tion surgical interference may be indicated. It is of no use to give styptics except in purpura, when mineral acids or suprarenal extract may be tried. Later, one may give iodide and mercury and use blisters to the back. 2. Hemorrhage into the Substance of the Cord (Haematomyelia)^ Etiology. — The condition is not very rare. It may be primary from dis- ease of the blood-vessels or purpura hemorrhagica; or it may be secondary to myelitis and tumors. Primary hemorrhage occurs sometimes in in- fancy, but usually in males between the twentieth and fortieth years. Infection, injuries, over-exertion and exposure, excessive coitus (Gowers), syphilitic disease of the blood-vessel, and convulsions are causes. The dis- ease sometimes occurs in old people with degenerated arteries, which break and lead to a spinal apoplexy, just as occurs in the brain. More often the condition in old people is a thrombosis. A special cause of spinal hemor- rhage is working under high atmospheric pressure, as in the caisson disease. The symptoms develop rapidly, with at first feelings of numbness or weakness for one or two hours or longer. Then there is a sudden para- plegia, with anaesthesia or ataxia or both. The anaesthesia is often dis- sociated, there being loss of pain and thermic sense with retention of considerable tactile sense. The sphincters may be paralyzed; the urine has to be drawn. The reflexes may be abolished at first, but soon return and become exaggerated with signs of pyramidal tract involvement. There is considerable pain in the back. If the lesion is high up, the arms and thorax are involved. The acute symptoms begin usually to subside at the end of seven to ten days and the disease takes the char- acter of a chronic myelitis. If improvement does not occur, evidences of acute myelitis or softening appear and the patient dies. Pathology. — The vessels involved are the central arteries, which sup- ply the gray matter and are under relatively high pressure. The rupture of the vessel, when due to disease, is caused by a fatty degeneration of the coats or endarteritis; miliary aneurisms, such as are found in the brain, rarely develop in the cord. Hemorrhage often precedes or be- gins a myelitis, of which it may be the cause or the result. The clot may be absorbed, leaving a cavity as in the brain; or the broken-down tissue may become the centre of a myelitic focus. The hemorrhage is usually single, but there may be several. Multiple capillary hemorrhages DISEASES OF THE SPINAL CORD 219 occur, but usually only from asphyxia and convulsions or in caisson dis- ease. It is possible that some of the cases of disseminated myelitis oc- curring after infectious fevers start from small hemorrhages. Hemor- rhage sometimes results from the invasion of the cord by a new growth, as in syringomyelia. Diagnosis. — The points to be noted are the sudden onset without long premonitory symptoms, and the absence of fever followed later by gradual improvement. There is much less pain and more paralysis than in meningeal hemorrhage, and the dissociation of cutaneous sensations is very characteristic. In acute softening there is less of the dissociation of sensations and usually a more extensive paralysis and the patient is syphilitic or aged. The disease is often mistaken for acute primary myelitis, which does in fact sometimes follow it. Prognosis. — This is often serious as regards life, and always serious as regards health. It depends on the extent and seat of the hemorrhages. Dorsal hemorrhages are more favorable, cervical the least. Treatment. — Absolute rest, ice bags to the spine, and small doses of aconite given early are all that can be tried, except the use of sympto- matic remedies. Treatment must be applied at once. The late treat- ment is the same as that for chronic myelitis. THE CAISSON DISEASE (DIVER'S PARALYSIS) The caisson disease is the name given to a group of symptoms char- acterized mainly by pains and paralysis which occur in persons who work in caissons or diving bells, and which are brought about by the sudden return from condensed air to the normal atmosphere. Etiology. — Persons employed in caissons or bells work usually under a pressure of from one to four atmospheres, which means a pressure of from fifteen to fifty pounds to the square inch. Accidents, rarely, if ever, occur if the pressure is not over one atmosphere, and they are rare if the person has not been subjected to the pressure for at least an hour. The effective cause of the symptoms, however, is a too rapid decompression. Different persons vary in susceptibility to the effects of this change in the atmospheric pressure, and those unused to the work or of maturer age and poor arteries are more liable to be attacked. Naturally, the disease is seen only in men, and during the working period of life. Hunger and exhaustion make a person more susceptible. The symptoms set in usually very soon after the patient has come out from the caisson, but they may be delayed for half an hour to an hour. They consist of intense neuralgic pains in the lower extremities, often affecting especially the joints. There is at the same time epigastric pain. Nausea and vomiting with weakness in the lower limbs, amounting in some cases to absolute paralysis, very soon appear. There may be 220 DISEASES OF THE NERVOUS SYSTEM headache, dizziness, and sometimes choking, coughing and oppression in breathing. If the paralysis is considerable, it is usually accompanied by anaesthesia. Disturbances in the sphincters, with retention of urine and constipation, may also be present. The symptoms vary very much in severity, from pain, weakness in the legs and nausea, up to frightful neuralgic attacks and complete paralysis, motor and sensory. The upper limbs are rarely affected. The disease lasts from a few hours up to several weeks. Death occurs in some of the very severe cases. The symptoms having reached their climax gradually ameliorate, and a com- plete cure is not infrequent. In some instances, however, the patient is left with a permanent paraplegia and the ordinary symptoms of a Fig. 98. — Spinal cord in caisson disease showing extensive destruction of tissue. transverse myelitis. Hemiplegia occurs in about one-fifth of cases and still more rarely a monoplegia. The disease in its mildest form is characterized mainly by severe pains, with some weakness and dizziness which usually soon pass away. The symptoms are spoken of as "the bends." When vertigo and staggering and some mental confusion are dominant, it is called ''the staggers," and the dyspnceic and coughing attacks are called "the chokes." Pathology. — When the patient is under atmospheric pressure in-the caisson, the blood is driven from the surface of the body, and the internal viscera, including the brain and cord, are congested. The sudden change from the abnormal to normal pressure produces a rapid flow of blood from DISEASES OF THE SPINAL COED 221 the internal organs to the periphery. The viscera not inclosed in bony cavities are enabled to relieve themselves of this congestion without much harm, but the circulation in the brain and spinal cord is less elastic; that in the spinal cord being less even than that in the brain. The result is that congestions and small hemorrhages ensue, producing a destruction •of the nerve-tissue. In other cases there seems to be a blocking up of some of the small vessels, with consequent softening of different portions of the cord and to a less extent of the brain. The most important ele- ment in causing the trouble is the escape of oxygen and carbonic-acid gas from the blood into the tissues or into the blood-vessels, forming gas emboli. It will be seen, however, that on the whole the serious organic changes consist in the vascular disturbances with rupture or obliteration of blood-vessels, and consequent destruction and necrosis of tissue. Following this is a reactive inflammation producing the phenomena of a reactive acute myelitis. Prognosis. — The painful and vertiginous types of the trouble nearly all get well. Over half of the paralytic cases recover. Prophylaxis. — The safest age for caisson workers is from twenty to thirty-five. They should have a good muscular system, good eyes, ears, lungs and hearts. The Anglo-Saxons and negroes seem to stand the work better than the Latin races (Pelton). They should not have the status lymphaticus. They should drink but little. The most important pre- caution of all is slow decompression, about two pounds per minute. Hill and Greenwood assert that pressures up to seven atmospheres (about 140 pounds to the square inch) can be safely borne, if the decompres- sion is slow enough. On coming out the workmen should not do any climbing, and should not take hot coffee, nor alcohol, as a drink. Treatment. — The most essential thing for immediate treatment and relief is immediate recompression in a hospital-lock provided for the pur- pose. The pressure (Pelton) should be gradually increased to about that under which the patient has worked. Then in a few minutes decompres- sion is very slowly given. When the disease has developed it can be treated only by symptomatic remedies. The patient should be kept quiet, and given, if necessary, hypodermatics of morphine. Dr. A. H. Smith recommends the use of ergot. Later on, the various neuralgic and paralytic symptoms may be treated on the same principles as those employed in myelitis. INFLAMMATION OF THE SPINAL MEMBRANES (SPINAL MENINGITIS) The meningeal inflammations acute and chronic are: Pachymeningitis. | Affecting the dura mater. Leptomeningitis. J Affecting the pia mater. ,, . ,.^. I Affecting both membranes Menmgo-myelitis. ■ i i i 222 DISEASES OF THE NEEVOUS SYSTEM EXTERNAL MENINGITIS, PACHYMENINGITIS EXTERNA Etiology. — The disease is rare, and always occurs secondarily to some other morbid process. This process is in most cases tuberculosis causing caries of the vertebrae. Other causes are suppurative inflammation, such as a carbuncle, in the neighborhood of the vertebrae, psoas abscess, puru- lent pleurisy, and the extension of a neoplasm. Tuberculous pachy- meningitis is so commonly associated with cord changes that it is de- scribed more fully under the head of Compression Myelitis. Symptoms. — The symptoms are those of irritation of the motor and sensory roots; later of compression of them and of the spinal cord. There occur local pains in the back, radiating pains, local tenderness, and hyper- sesthesia, twitching of muscles and rigidity of spine, progressive develop- ment of a fLpastic paraplegia, exaggeration of reflexes, and involvement of the sphincters. Anaesthesia occurs in severe forms, which takes the distribution of a root anaesthesia. The disease, when chronic, may extend to the other membranes and cord, causing chronic meningo-myelitis. Pathological Anatomy. — The inflammation, if acute, is generally a fibro-purulent one. The dura mater is covered by a layer of caseous, semisolid matter, often very thick and most extensive posteriorly. It involves the dura vertically for several inches. In chronic forms the deposit is made up of connective tissue and the cord is compressed. In purely suppurative forms the cellular tissue outside the dura is infiltrated with pus throughout a great part of the canal. The diagnosis is based on the presence of the primary local disease, a kyphosis, the radiating pains, and tenderness and by the combination of motor and sensory irritation and paralysis. Anaesthesia and sphincter troubles come late in the disease. The prognosis is bad, if the original disease is a serious one. Still, surprisingly good results are often obtained when the disease is taken early, especially in tuberculosis cases. The treatment consists in attention to the local caries or inflammatory focus. It is therefore purely surgical, mechanical and symptomatic. PACHYMENINGITIS INTERNA {Hypertrophic Cervical Pachymeningitis) Inflammation of the inner surface of the dura mater has been much written about, and but little seen. It is almost always only a syphilitic meningo-myelitis and it attacks the cervical region as a rule. Etiology: — The disease occurs always in adult life. It usually affects males. Syphilis and trauma are the causes. DISEASES OF THE SPINAL CORD 223 Symptoms. — The disease begins gradually with symptoms of irrita- tion (irritative stage) . The patient suffers from pain and stiffness in the neck. The pains radiate up to the occiput and down the back; numb- ness, prickling and pain are felt in the arms, more in one than the other. The pains exacerbate and are worse at night. Stiffness and cramps may affect the arms. Nausea and vomiting sometimes occur. After five or six months, symptoms of paralysis appear (paralytic stage). The arms are affected. They become weak, atrophy occurs, associated with contractures and rigidity. There is still pain, and in addition anaesthesia, hypersesthesia and trophic changes occur. Later, paraplegia, with rigidity, exaggerated reflexes, and spinal trepidation develop. The patient becomes weaker, and finally dies of exhaustion or from some intercurrent trouble. Usually it runs along and painful course, but if early recognized and treated it should be at least controlled. Pathology. — The disease starts as an inflammatory syphilitic exudate upon the surface of the dura. This leads to a chronic inflammatory process, and finally the cord is encircled and compressed by a dense laminated connective-tissue mass, which involves the pia and to some extent the cord substance. Diagnosis. — This must be made from tumor, myelitis, Pott's disease, wry-neck and progressive muscular atrophy. The history of injury, the slow progressive course, and the localization of the symptoms, their bilateral character and the pain, a history and laboratory evidence by lumbar puncture of syphilitic infection give adequate help. In spinal tumor the symptoms at the beginning are more sharply localized. They develop more rapidly and the course of the disease is shorter than in meningitis. Treatment. — The syphilitic origin of the disease must be borne in mind. Counterirritants, electricity, hydrotherapy and symptomatic remedies for the pain and spasms are indicated. ACUTE SPINAL LEPTOMENINGITIS (Inflammation of the Pia Mater of the Spinal Cord) Etiology. — This is so rare a disease, occurring alone, that I leave it out of the category of independent disorders. The description of cerebro- spinal meningitis covers sufficiently the ground. CHRONIC LEPTOMENINGITIS (NON-LUETIC) Etiology. — This disease, which used to be often diagnosticated, is now believed to be rare and always secondary to trauma, or an acute process, such as a cerebrospinal meningitis. It occurs oftenest in adults and in 224 DISEASES OF THE NEEVOUS SYSTEM males. Trauma, and especially concussion of the spine, used to be thought a frequent cause, but in most of such cases the trouble is simply a hypersemia or else is neuralgic and functional. The sympto7ns gradually develop after an acute meningitis or an injury, they are. similar in character to those of the acute process. There are pain in the back, increased on movement and radiating about the trunk and down the limbs, tenderness along the spine, stiffness of the back, twitching and spasms in the limbs with some weakness. The symptoms run an irregular course with periods of improvement. Pathological Anatomy. — The inflammation consists of a proliferation of connective tissue (productive inflammation of Delafield) . The result is a thickening and opacity of the pia mater and arachnoid. The diagnosis must be made from functional disease, meningo- vascular syphilis, myelitis, and vertebral caries. In vertebral caries the pain and tenderness are much more localized, and there is spasmodic fixation of the trunk. The pain is more continuous and dull, and is increased by lateral pressure and lessened by extension. There is usually also some deformity. If compression occurs there is exaggeration of the reflexes and paraplegia without much anaesthesia. Treatment. — Chronic meningitis not the product of syphilis is the relic of traumatism or of an acute process and the indications for treatment are simple. Rest is the essential thing. With this can be combined the sys- tematic and persistent use of counterirritants. The hot iron is usually best, because its wounds heal so quickly. Cupping is also useful if done vigorously and often. MYELITIS AND MYELOMALACIA— INFLAMMATION AND SOFTENING OF THE SPINAL CORD Inflammation of the spinal cord is known as myelitis. Softening of the cord is known as myelomalacia. The processes are practically associated. Acute myelitis always ends in some form of softening ; and acute softening is often followed by reactive or infective inflammation. If inflammation affects the gray matter only it is called poliomyelitis ; and if the anterior horns chiefly, it is an anterior poliomyelitis. Both the gray and white matter are usually affected, and myelitis or spinal softening may be dif- fuse, disseminated, or transverse in accordance with the distribution of the process. The forms are still further divided, in accordance with their location, into the cervical, dorsal, and lumbar. Myelitis is given different names also in accordance with its cause. Thus we have hemorrhagic myelitis, a form in which the process is due to or associated with a hemorrhage; compression myelitis, due to vertebral caries and the pressure of tuberculous exudate, purulent myelitis or abscess of the cord; and DISEASES OF THE SPINAL CORD 225 tuberculous and syphilitic myelitis which are really essentially necrotic and softening processes and not myelitic. The following table will show the different morbid processes and the six clinical types of myelitis: Forms of Myelitis and Myelomalacia Pathological Process Clinical Type An exudative, lymphogenous inflamma-1. tion due to infection. Anterior poliomyelitis, Landr3^'s acute ascending paralysis. An exudative, hemorrhagic or purulent in- 2. flammation due to infection. Acute transverse, diffuse and dissemin- ated myelitis. Abscess of cord (rare). A subacute degenerative and necrotic 4. process due to toxins. Subacute combined and diffuse cord degenerations (combined sclerosis). An embolic or thrombotic process. 5. Acute and subacute softening of cord. A parenchymatous degeneration with con- 6. nective-tissue proliferation due to vas- cular and toxic causes, or secondary processes. Subacute and chronic poliomj^elitis. Acute myelitis is a local or diffuse exudative inflammation with more or less necrosis and softening. (Type 2 of table.) Etiology. — It affects adults in early and middle life, and men oftener than women. It is rarely seen in old age or childhood. Heredity and neuropathic constitution are not important factors, though it is probable that there are some persons whose spinal cords are less resistant to infec- tion than others. Exposure to cold, bodily and sexual excesses, and violent concussion have some importance indirectly in favoring the entrance of infection. The list of local bodily infections which are the source from which the cord becomes infected is very long and includes general sepsis, peritonitis, appendicitis, enteritis, colitis, liver abscess, cystitis (urinary paraplegia) , gonorrhoea, osteomyelitis, and tonsillitis. It more rarely follows general infections, such as influenza, small-pox, scarlet fever, typhoid fever, rheumatism, diphtheria, and pneumonia. Sometimes the micro-organ- ism of acute poliomyelitis causes a definite transverse or diffuse myelitis. Myelitis has occurred associated with the toxaemia of pregnancy. In this and perhaps other infections or toxsemias the lesion may be due to thromboses from the increased coagulability of the blood or circulatory 15 226 DISEASES OF THE NERVOUS SYSTEM weakness. The infection of polyneuritis in rare cases may affect the cord also. Certain chemical poisons such as carbonic oxide, sulphide of carbon, and chloroform cause a necrotic and degenerative rather than an exuda- tive myelitis. Syphilis does not cause acute myelitis but only softening with hemor- rhages and chronic degenerative changes. The mechanical injury due to gas bubbles in caisson disease may destroy cord tissue and lead to inflammatory changes. Despite the above long list, acute myelitis is really a very rare disease. Symptoms. — The initial symptoms consist of feelings of numbness, usually in the feet and legs, which seem heavy and weak. Some pain may be felt about the back. The patient finds that he cannot walk easily, that he moves his legs with an effort and that they feel stiff. In one or two days some paraplegia with ansesthesia has developed, and if the lesion is in the cervical cord the arms are paralyzed also. Partial or complete retention or incontinence of urine and constipation occur at the same time. There may be some fever. In three or four days, sometimes in a day, the disease reaches nearly its height and the patient is paraplegic and confined to bed. If the patient is now examined it will be found that he cannot walk or stand, but can move his legs a little. He complains of a sensation like a band around his waist or at the level of the spinal lesion (girdle symptom). His legs feel numb and heavy, but there is little pain and no tenderness. Ansesthesia to touch, pain, and temperature and of deep sensibility exists, in varying degree according to the seriousness of the case, as high up as the lesion. The anaesthesia, if not total, is greatest to touch, next to temperature and to pain and least to sense of position and deep pain. The bladder is anaesthetic; the urine is retained, and has to be drawn. The bowels are constipated, and if enemata are given the faeces may pass away without his knowledge, owing to rectal ansesthesia. If the lesion is lumbar, there is abolition of the sexual power; but if dorsal or cervical, erections may occur without the patient's feeling them. When the lesion is above the lumbar cord also, the bladder may auto- matically and forcibly contract and expel the urine. In time the bowels regain some power. The paralysis in the limbs affects the extensors more than the flexors of the toes, the flexors of the feet and legs more than the extensors. The patient can push down his limbs better than he can draw them up and he can adduct better than abduct. The temperature of the limbs for a few days may be raised, but after this it may fall a few degrees below normal. The skin becomes rough, cold, congested; or excessive perspiration may take place. The general bodily temperature is usually normal throughout the disease, but in DISEASES OF THE SPINAL CORD 227 some cases septic fever develops of 102° to 104° and continues. The pi'og- nosis is then bad. Bed-sores may develop early, within a few days or weeks unless great care be taken. They appear oftenest upon the buttocks and heels, and are due to vascular weakness trophic disturbance, combined with pres- sure and pyogenic infection of the parts. Bed-sores are, however, not necessary if every possible care and precaution are taken. If the lesion is lumbar, the tendon and skin reflexes are lessened and the paralysis is somewhat flaccid. The muscles also tend to waste and show degenerative reactions. If the lesion is dorsal, as is more often the case, the reflexes are present, and after a time become exaggerated; Fig. 99. — Acute transverse myelitis of lumbar cord, showing distribution of anaesthesia.' Area in lines = total anaesthesia and analgesia. Dotted area = anal- gesia only. there is ankle clonus and extensor response on irritating the soles of the feet; contractures and spasms develop; the legs become drawn up and deformities are produced. If the lesion is so complete as entirely to cut across the cord, there may still be some excessive muscular tension, but the reflexes will be abolished. When the cervical region is attacked, the arms are involved as well as the legs, with a segmental lower motor neuron palsy, generally severer in the arms. There may now be also unequal dilatation of the pupils from involvement of the cilio-spinal centre. In extensive involvement of the upper part of the cord there will be paralysis of the intercostal muscles and disturbance of the heart's action. 228 DISEASES OF THE NERVOUS SYSTEM The disease, having in a few days reached its height, usually remains stationary for a few weeks, and then, should the patient live, imvproe- ment slowly sets in. In some cases evidences of extension upward or downward occur (ascending or descending myelitis) ; the symptoms be- come more severe, and in a few weeks, or oftener months, death occurs directly from bronchopneumonia or pyonephrosis. As improvement begins, a return of sensation is first noticed (one to six months); this is followed by return of more or less motion (six to eighteen months). Spasms and contractures now develop, owing to secondary degenerations. A certain amount of ataxia from posterior column degeneration, with a little anaesthesia of the skin, may remain, so Fig. 100. — Acute diffuse myelitis. that, if the patient has sufficient motor power to walk somewhat, he presents many features of "ataxic paraplegia." Some additional and continued improvement may be expected for from one to two years. A few cases get almost entirely well. The major- ity become more or less paraplegic and bedridden, in which condition they are regarded as cases of chronic myelitis, though no active process exists. Pathology. — The infection of the spinal cord in acute exudative types of myelitis is believed to be through the lymph paths of the nerves and not through the blood-vessels (lymphogenous myelitis). In the more severe hemorrhagic and in the purulent types it is a vascular infection (hematogenous). The early changes found are those of inflammation. DISEASES OF THE SPINAL CORD 229 hemorrhagic extravasation and softening. Often it is impossible to say whether the primarj^ process was inflammatory or due to a hemorrhage or softening. Macroscopicalh' , the cord at the affected part appears soft, swollen, and either red and hypersemic or pale and anaemic. In rare cases no change is apparent to the naked eye. In later stages the parts are white or gray, shrunken and hard. The cord may be reduced to a thin shred. The meninges about the affected parts are often thickened, inflamed, and adherent. Microscopical^, if the process is primarily inflammatory we find intense congestion, distended blood-vessels, emigrated white blood-cells \ ^^^^ Fig. 101. — Acute hemorrhagic myelitis. in great number, especially in the perivascular spaces, swollen axis-cylin- ders and oedematous swelling of the myelin sheath, red blood-cells, cells filled with fat granules known as compound granular corpuscles, or Gluge's corpuscles (Fig. 100). When the infection is very intense the hemorrhagic process is more active (Fig. 101). In fatal cases inflammation and softening continue; fresh areas, of cord are involved, much meningeal exudation takes place and finally death occurs. The process may in very rare cases be still more acute, suppuration and abscess occur, and here death ensues in two or three weeks. 230 DISEASES OF THE NERVOUS SYSTEM The inflammatory and softening processes above referred to are described in accordance with their appearance as red softening, yel- low softening or white softening. A form of so-called inflammation, known as inflammatory oedema, is also described. It is an abortive inflammation, ''a lymphatic congestion," analogous to vascular congestion. The diagnosis of acute myelitis must be made from hemorrhage, non-inflammatory softening, multiple neuritis, and hysterical or func- tional paralysis. Syphilitic spinal paralysis (Erb's type) is recognized by the cerebrospinal fluid tests. Spinal hemorrhage comes on suddenly and is not attended by fever. If meningeal, it is attended with pain and the condition soon remits. Acute softening not syphilitic usually occurs only in old people with spinal arterial sclerosis. In softening there is no constitutional disturbance, no leukocytosis, the process is slower, there is less pain and the dissociation of cutaneous sensations is less marked. In acute ascending paralysis the disease is progressive, there are no involvement of sensation, no atrophy, and little change in the electric irritability. In multiple neuritis the onset is slower, there are more pain, local tenderness and sensory disturbance, and the sensory loss is of the glove and stocking types; the bladder and rectum are rarely involved. In meningitis there are pain and tenderness in the back and limbs, rigid- ity, cramps, a little paralysis, and no bladder trouble. In hysterical paraplegia there are no marked atrophic changes, but little spasm or rigidity, no electrical changes, and the stigmata of hysteria may be found. The sensory disturbances are variable and somewhat characteristic (see Hysteria), and the knee-jerks are not greatly if at all exaggerated. Tap- ping of the spinal canal will enable one to exclude hemorrhage and to determine by the presence of a lymphocytosis that there is an inflamma- tory process. The serological tests will also determine whether it is a syphilitic process or not. The diagnosis of the location of the lesion is made by studying the height of the anaesthesia, the skin reflexes (see p. 46), the loss of sphincter control, and the distribution and extent of the paral- ysis. There is often a differentiation of the ansesthesia, as shown in Fig. 99. Prognosis. — The prognosis is worse the more complete and extensive the paralysis. It is worse in serious motor paralyses than when sensa- tion is chiefly involved. It is best in dorsal myelitis and worse usually in cervical myelitis, other things being equal. Bed-sores and slight fever are unfavorable signs; so also is severe involvement of the bladder and rec- tum. Recovery of sensation gives good hope of recovery of some motion. Total absence of recovery of sensation and motion after six months is very unfavorable. Improvement may be expected up to eighteen months after THE DISEASES OF THE SPINAL CORD 231 the onset, and in some cases even longer. In compression myelitis there is more chance of recovery than in the other forms provided that the com- pressing agent be removed. Treatment. — In the attack, the patient must be put to bed; dia- phoresis should be promoted, a laxative and colon washings given, and, in general, eliminative treatment followed. Urotropin and small doses of aconite and nitroglycerin may be prescribed. The bladder should be watched. After a week, moderate doses of iodide of potassium should be given. After about three weeks, if there is no fever, electricity may be applied carefully and strychnine administered in small doses. Bed- sores should be guarded against by the use of water-beds, or cushions, absorbent cotton, bathing the parts with alcohol and weak solutions of tannin. Infusion of buchu, boric acid and tincture of hyoscyamus will often help the bladder disturbance. Urotropin and sodium benzoate may be also needed. The frequency of syphilis as a cause of acute paraplegia (nearly one-half the cases) should lead to the persistent use of medication for this condition if indicated. After acute symptoms subside, the treatment is that for chronic myelitis. CHRONIC MYELITIS AND SOFTENING {Transverse and Com'pression Myelitis, ErVs Syphilitic Spinal Paralysis) Chronic myelitis is the name given to a disease characterized by a chronic inflammation of the spinal cord and to the chronic reparative processes which follow acute inflammation, injury and softening. Chronic myelitis is usually a mixture of inflammatory, reparative and necrotic processes and may be only the terminal stage of acute inflammation or softening. Forms. — Different names are given to chronic myelitis in accordance with the part of the cord affected. Usually the disease affects only cer- tain levels, and then it is called transverse myelitis. More rarely it is diffuse or disseminated, central or marginal. When caused by pressure from vertebral disease, it is called compression myelitis, though this is really often only an inflammatory oedema secondary to a peri-pachy- meningitis. Etiology. — The disease may be either primary or secondary. The primary form is somewhat the rarer. It occurs chiefly in adults and in early and middle life, and much oftener in males, and it is due to syphilis. Secondary chronic myelitis is a rather common form. It is really only the later stage of acute myelitis, softening, destructive hemorrhages and injuries. 232 DISEASES OF THE NERVOUS SYSTEM Meningitis may extend and cause a meningomyelitis. A neuritis may possibly ascend and cause myelitis, but such cases, if they occur are very rare. When all is said, chronic myelitis as usually seen is a syphilitic myelitis, or the result of injury, of hemorrhage, or of vertebral caries. Among 67 personal cases of secondary chronic myelitis there were 61 men and 6 women. The causes that can be assigned were: Syphilis, 23; injuries, 12; exposure, 3; acute infection, 6, of which 3 followed grip, 1 typhoid, 1 meningitis, and 1 mumps. Two were due to caisson disease; 4 were of arteriosclerotic origin occurring in the aged and 6 were due to tuberculosis. A study of the age shows that practically all the cases occur between the ages of twenty-one and fifty, and most of them between the ages of thirty-one and forty. Those cases occurring in the extreme of life are due to senile arterial changes or injury. Symptoms of Primary Chronic Myelitis. — When the disease begins primarily as a chronic affection the symptoms are as given below. And since nearly all such cases are of syphilitic origin, the description of pri- mary chronic myelitis is practically that of syphilitic spinal paralysis (Erb's spinal paralysis). The patient notices that his legs seem heavy and easily get tired; prickling and numb sensations are felt in the feet; occasionally a little pain develops in the back or there is a sense of constriction about the trunk. The legs are stiff, and tests often show that the reflexes are exaggerated, with ankle clonus and extensor response (sign of Babinski). There is but little wasting of them, however. The sexual power declines; the bladder gives some trouble, there being a tendency to retention; the bowels are constipated. After a few weeks or months there is a partial paraplegia, with rigidity of the limbs and exaggerated reflexes. Some anaesthesia exists, and occasional pain, which is not severe and is felt more in the back than the legs. Sensory loss is usually shght compared to motor loss. The muscles have later wasted somewhat, but show no decided changes to the electrical current. The bladder becomes more involved, the urine has to be drawn, it is often alkaline, and unless care is taken cystitis develops. The patient is still able to walk, but he does so with a stiff, shuffling gait which is characteristic (Fig. 102). The disease may show signs of slowly extending up and down, more often up. The arms become involved; weakness and stiffness, with some wasting, anaesthesia and pain, develop, or the disease may cease its progress and the patient remain partly paralyzed for years. The general health during the course of the disease deteriorates slowly; the patients often become anaemic and have an unhealthy pallor. Eventually the paraplegia may become complete, THE DISEASES OF THE SPINAL CORD 233 the patient is bedridden, the legs are atrophied, contractured and rigid, with more or less anaesthesia. Cystitis and nephritis develop, and the patient dies from these or other intercurrent diseases. Chronic secondary myelitis, which is the form often seen, presents eventually much the same picture as that just described. In this type, however, the symptoms are those of an acute myelitis first, then improve, then become stationary, and finally grow worse. Chronic compression myelitis, so called, is usually only a compression oedema with partial atrophy. It is due, as a rule, to vertebral caries and pachymeningitis, but its cause may be a spinal tumor or aneurism. Compression myelitis is distinguished from other forms by its slow onset and the presence at first of irritative or "root" symptoms. The patient suffers from pain and tenderness local- ized at a certain point in the spine. The pain radiates about the trunk or down the limbs and is increased on movements. At about the same time some motor weakness develops, usually in the form of paraplegia, one leg being often af- fected some weeks or months before the other. The muscles waste but slightly. The reflexes are exaggerated; twitchings, spasms and con- tractures finally occur, and there is developed a spastic paraplegia or quadruplegia. With this there is usually some anaesthesia, though it is not complete. The disease is oftenest in the dorsal or lower cervical region, and hence the sphincters escape until late. Ijocally, evi- dences of spine disease may appear early in the form of a kyphosis. A central or peri-ependymal myelitis can rarely be recognized with certainty. It produces less pain and irritation, but leads to muscular atrophy, disorders of sensations, such as thermo-ansesthesia, disturbance of vasomotor and secretory nerves and visceral centres. Pathology. — The pia mater is thickened over the affected region and often throughout the cord. The cord itself has a gray, discolored look at the affected level, and is usually shrunken or distorted and hard to the touch. In severe cases of secondary character it is reduced to a small size, and the membranes about it are thick and inflamed. In transverse myelitis a vertical area of only two or three inches is involved. The microscope shows that the prominent changes are loss of nerve struc- ture, great increase of connective tissue and increase in the number Fig. 102. — Chronic myelitis. 234 DISEASES OF THE NERVOUS SYSTEM of vessels, which often have thickened walls. In the more seriously diseased part little is seen but connective tissue. In parts less diseased some nerve-fibres are seen, many having evidences of partial disintegra- tion. There is also a good deal of amorphous material studded with nuclei. Stellate cells, granule cells, and nerve-cells in various stages of degeneration are present. In the parts less affected the signs of con- gestion and vascular irritation are more pronounced. Secondary degenerations occur above and below the seat of the myelitis (Figs. 113 and 114). In the typical syphilitic myelitis the meninges are thickened and there is a predilection for the inflammatory change to affect the lateral columns of the cord (Fig. 103). Fig. 103. — Syphilitic meningo-myelitis ; cervical region. Diagnosis. — This must be made from progressive muscular atrophy and amyotrophic lateral sclerosis, pachymeningitis and spinal tumor; from locomotor ataxia, multi'ple sclerosis and brain palsies. In progressive muscular atrophy there is a peculiar atrophy without involvement of the sphincters or sensory disturbance. Tumors usually cause much more pain; the symptoms come on slowly and are more uni- lateral at first and more definitely localized. A spastic paraplegia occurs in cerebral diplegia, but the arms are also sometimes involved, and there are no trophic or sensory troubles, nor is there involvement of the sphincters. In locomotor ataxia there is no great degree of motor paralysis, and there are peculiar ataxic and sensory disturbances. In the early stages of multiple sclerosis of the spinal type, however, the symptoms resemble closely a very slowly developing chronic myelitis. The diagnosis can be made by remembering that in multiple sclerosis patient is usually young, that there is absence of much sensory trouble, and THE DISEASES OF THE SPTXAL CORD 235 presence upon close examination of e3-e trouble, tremor, speech dis- turbance and paresthesias in multiple sclerosis the course is often a remit- ting one, the abdominal reflexes are usualh' lost and the bladder be- comes affected quite early. Paralysis from brain disease is almost alwaj'-s unilateral, painless, spastic and free from disturbance of the visceral centres. Prognosis. — Inflammatory processes have a tendency to cease when their reparatoiy and eliminative work is done. And this is true of mj-elitis. However, in the spinal cord secondary degenerations set in as soon as certain tracts are interfered with. Hence chronic myelitis, after a period of improvement, generally progresses, and the prognosis is not ver}- favorable. Still, patients may live comfortabh^ from five to twenty-five years. Dorsal myelitis is the most favorable form; compression myelitis from caries can also often be successful!}' treated. Syphilitic myelitis and meningomyelitis unless treated are liable to take a progressive course, Hke a locomotor ataxia. Serious involvement of the bladder is a bad sign, and naturally the prognosis is worse the more complete the paralysis. Treatment. — In the treatment of chronic myelitis the cause must be sought and treatment applied accordingly. If it is sj'philis a thorough- going course of anti-s^'philitic measures should be instituted. If it is a compression myelitis from Pott's disease, prolonged rest in bed for six months or more is usually adequate. Jackets and various forms of support are helpful but alone are not enough. If the cause is caisson disease, injury by fracture or wounds, rest and mechanical measures are the essential things. Various supplementary and symptomatic measures are to be used. Counterirritation in the form of fly-blisters, or the cautery, may be ap- plied and wet or dry cups used. The descending galvanic current along the spine should be tried; faradism and massage being used upon the limbs. Lukewarm baths. 90° to 98°F.. or half-baths with frictoin at 70° to 80°F., are likely to be useful, but these must be tried cautiously. The first baths should last not over five minutes and should be repeated only three or four times weekly. In later paraplegic and bedridden stages, electrical and hydrotherapeutic apphcations should be followed up patiently and persistentl}^ The patient may be allowed to remain and exercise in the lukewarm bath for some time. The patient may be given courses of tonic treatment, using phosphates, iron, arsenic, and small doses of strychnine. The more surelj' the disease is non-luetic, the more are simple tonic and mechanical measures alone indicated. For the bladder troubles, the internal use of boric acid, belladonna, ergot buchu, and urotropin are helpful. Salvarsan is of little or no value in long-standing chronic mj-elitis, unless the disease is progressing. 236 DISEASES OF THE NERVOUS SYSTEM ACUTE POLIOMYELITIS (Epidemic Poliomyelitis; Infantile Paralysis) This disease results from an infection of the body by a specific organ- ism, which produces acute constitutional symptoms of a reactive char- acter; this organism has an especial affinity for the central nervous sys- tem, whereby it usually produces an acute flaccid motor paralysis, in muscles segmentally grouped which subsequently undergo more or less atrophy. Etiology. — It occurs at all ages, but 60 per cent, of the cases are in children under three years. About 15 per cent, of the cases are in adults usually under thirty. The disease occurs epidemically and to a lesser extent sporadically. Almost all the cases appear in the summer months, especially in August and September. Epidemics occur rather more often on the coast board and in low-lying places; the principal epidemics described have been in Scandanavia from 1903 to 1906, Minnesota, New York 1907 and 1908, Victoria, Australia 1907, Massachusetts 1908, Vienna 1908. Smaller epi- demics have been reported from various parts of France, England and the United States. No specific cause can be cited as rendering the body liable to attack; all factors lowering vital resistance are probably contribu- tory to infection, such as malnutrition, fatigue, and other infectious dis- eases. The incubation period varies, but averages ten days in length. Bacteriology. — In the summer of 1909 Landsteiner and Popper suc- ceeded in producing the disease in two monkeys by intra-peritoneal injec- tion of emulsified spinal cord obtained from a fatal case of poliomyelitis. They failed, however, to transmit the disease from these to other monkeys. In September of the same year Lewis and Flexner working at the Rocke- feller Institute also succeeded in producing the disease in monkeys by means of intra-cerebral injection of poliomyelitic cord. The cords of the affected monkeys furnished a virus which by September 1910 had been propagated through twenty-five separate series of monkeys. The virus is found to pass readily through a Berkefeld or Chamberland filter. It withstands glycerinization, drying and freezing over prolonged periods, but is readily destroyed by heat and by comparatively weak antiseptics such as menthol and a 1 per cent, solution of hydrogen peroxide. In 1913 the virus was cultivated with difficulty by Flexner and Noguchi under anaerobic conditions; subcultures in the fifth generation have suc- cessfully reproduced the disease in monkeys. The minute colonies are com- posed of globular bodies averaging 0.15 to 0.3 micron in size; these occur in short chains, in pairs, and in masses, and are stainable by Giemsa's or Gram's methods. The virus can be inoculated with success into the brain; into the subdural space; subcutaneously; by introduction into the THE DISEASES OF THE SPINAL CORD 237 stomach and into the intestines. It has been shown that it can be carried from the ill to the well through the intermediate agencies of the house fly, the stable fly, Stomoxys calcitrans, and the bedbug. Experiments directed to prove an identical role on the part of mosquitoes and lice were negative. Flexner and Amoss, however, in papers published in 1914 believe that infection is local and by way of the lymphatics, and not general and by way of the blood. They believe the virus enters the body through the nasopharyngeal mucous membrane, and hence through the lymphatics of the olfactory nerve to the leptomeninges. An attack of poliomyelitis confers an active immunity against a second infection. This obtains apparently for at least three years. Successful vaccination against the virus has been achieved in the macacus but not as yet in man. It has been shown experimentally that the previous administration of Fig. 104. — Acute anterior poliomyelitis showing diffusely inflamed area on right side. urotropin will in the monkey increase the incubation period of the in- oculated disease and diminish its severity. Pathology. — Naked-eye appearances : The brain may be oedematous and show some vascular engorgement. The cord also may be oedematous with some wrinkling of the swollen and congested meninges. On section the distinction between gray and white matter may be diminished through softening especially in the severely affected areas, usually the cervical and lumbar enlargements. Similar softenings may be found in the brain stem or hemispheres. The spleen is enlarged and changes varying from coagulation to hemorrhage may be found in the kidneys and liver. The microscopic lesions in the brains and cords of fatal cases consist mainly of congestion and hemorrhage into the gray matter; the anterior horns being disproportionately affected. The entire spinal cord may be in- volved in a series of vascular accidents of varying degree. The lesions 238 DISEASES OF THE NERVOUS SYSTEM consist of a small round-celled infiltration in the perivascular spaces with the development of considerable oedema. Arterial thrombosis is never apparent, and the degeneration and necrosis of cells are invariably pro- duced by gross pressure of inflammatory exudates on the cells and the nutrient blood-vessels. A leptomeningeal infiltration of mononuclear cells is always present and is perivascular in position. The greater affec- tion of the anterior over the posterior horns is explicable by the richness of the vascular supply to the former; a similar consideration serves to explain the apparently disproportionate involvement of the lumbar and cervical enlargements (Figs. 104, 105 and 106). Fig. 105. — Acute anterior poliomyelitis. Same as previous figure enlarged, showing loss of cells in right-horn. Signs and Symptoms. — These may be conveniently described under different headings : (1) A prodromal period — lasting a few days; (2) an acute stage of a few hours to a week; (3) a stationary period — one to six weeks, usually two weeks; (4) a stage of improvement — six months to three or four years; (5) a chronic stage of residual incurable atrophic palsy. 1. In some cases the onset of the acute stage is so rapid that the pro- dromal period is practically eHminated, but the vast majority of patients complain of some general malaise, and headache of considerable severity with occasional vomiting. Drowsiness combined with irritability is usually present. There is generalized hyperaesthesia with pain in the back, THE DISEASES OF THE SPINAL CORD 239 in the neck muscles — which may also be rigid, and in the limbs; the pain is usually greatest in limbs subsequently paralyzed. Constipation is usual; there are present much generalized sweating and a moderate fever and leukocytosis in blood and spinal fluid. 2. The constitutional symptoms of the prodromal period may be carried on through the few hours or days of the second stage in which motor paralyses appear. These come suddenly and achieve their maxi- mum severity within a few hours. The affected limbs are toneless and tender. Sensory changes are very rare and transient. Sphincter dis- turbance is usual but quickly passes. The paralysis most often affects both legs, next one leg, next the arms and legs, and after this various Fig. 106. — Acute anterior poliomyelitis. combinations. The cranial nerve nuclei may be attacked, and in the not very common cases where the cerebrum is affected there may result hemiplegia of upper motor neurone type with some residual intellectual impairment. The term polioencephalitis superior has been apphed to those cases where the oculomotor nuclei are destroyed; affection of the nuclei placed lower in the brain stem has been called polioencephalitis inferior. Cases in this stage prove fatal usually from medullary in- volvement or paralysis of the respiratory muscles. 3. The paralysis remains at its height for from one to six weeks, and then improvement gradually sets in. In two or three weeks a wasting of the paralyzed limb may be noticed. It is flabby, its temperature is 240 DISEASES OF THE NEEVOUS SYSTEM lowered, and the reflexes may be gone. Slight tenderness may be present but there is no anaesthesia. 4. The stage of improvement lasts for from six months to several years. The paralysis gradually disappears, beginning in the limbs least injured. This continues until but one or two limbs are still affected. In them the muscles are wasted and show the reaction of degeneration, viz., loss of faradic irritability, retention and slowing of galvanic irrita- bility, and sometimes polar changes. In the leg, the anterior tibial and peroneal groups are oftenest affected; in the arm, the muscles of the shoul- der girdle. After eighteen months not much further spontaneous im- provement can be expected, but much can usually still be done by vigorous massage and suitably adapted exercises. 5. The temperature of the affected limbs is lowered several degrees; the skin has a reddish-purplish mottled look. The bones as well as the mus- cles of these limbs do not grow as well as those of the others. Hence in time the extremity is disproportionately small and short (Fig 108). Owing to the contraction of unopposed muscle deformities occur. The most frequent are talipes equinus, talipes varus and valgus. Deformities of the knees, and contraction of the plantar fascia also take place. Spinal curvature is com- mon in cases in which the supporting back muscles have been affected. The general health of the patient is usually good. A consideration of the path- ology of this disease will make evident the fact that many different clinical varieties must occur to correspond with the varied combinations of spinal and cerebral lesions produced The following distinct types may be mentioned: 1. Spinal poliomyelitis — a sudden feverish attack followed by the palsy of one or more limbs. 2. An ascending form in which, first, are involved the legs and later the abdomen and thorax. This type is often fatal. 3. A bulbar type, with palsies of the various pontine and medullary cranial nerve nuclei. 4. Polioencephalitis, from which a state of meningismus with spastic paralyses may ensue. Fig. 107. — ^Poliomyelitis, late stage showing atrophied and shortened leg. (Schoenborn and Krieger.) THE DISEASES OF THE SPINAL CORD 241 5. Ataxic polioencephalitis, by which is merely meant a specific inflammatory process, the maximum stress of which falls on the cerebellum. The clinical symptoms are briefly, an acute illness of short duration, fol- lowed by motor ataxia of the arms and legs, nystagmus, and explosive syllabic speech. 6. Abortive forms in which only general constitutional disturbances occur, and paralyses are either cjuite fleeting, or entirely absent. In these, diagnosis may be surmised from the co-existence of an epidemic of the disease, and confirmed by spinal fluid examination. The necessity of making correct diagnoses in these cases is indicated by the fact that polio- myelitis has been propagated through successive series of animals, from the nasal washings of an abortive case. Diagnosis. — The initial symptoms of poliomj-elitis are almost gen- eric for all infected conditions. Influenza must be thought of. Against it one puts the seasonal unlikelihood, it being a winter and spring disease. In poliomyelitis, catarrhal sjanptoms will be absent. Gastroenteritis is most frequent in the summer and early autumn, but in it the onset is less rapid, pyrexia less sudden, while somnolence, general hypersesthesia, and sweating are infrequent. Constipation rather than diarrhoea obtains in the early stages of the cord affection. Lumbar puncture should be performed early in all doubtful cases, particularly to differentiate the con- dition from tuberculous and diplococcal meningitis. The main char- acteristics of the three fluids may be here contrasted : Tuberculous meningitis: Clear, forming a fine clot on standing. Globulin content early increased. The normal reduction of Fehling's solution often lost. Usually an almost pure Ijaiipocytosis from the very beginning of the disease. In 85 per cent, tubercle bacilli may be demonstrated. Diplococcal meningitis: Fluid turbid and heavily albuminous. Fehling's test positive except in very chronic cases. Almost pure polymorph leukocytosis. Gram-negative diplococci may be easily demonstrated. (See also "Cerebro-spinal Meningitis".) Acute poliomyelitis: Fluid clear. Clot formation on standing slight. Globulin content slightly raised in early stages, and gradually rises for about three weeks after which it may sink to normal. Fehling's test normal. In the first three days of the disease there is usually an almost pure polymorph leukocytosis, as high as 1,000 per cubic millimeter. This is then replaced by an almost pure lymphocytosis of gradually diminishing severity. No organisms can be demonstrated by ordinary methods. The disease must also be distinguished from toxic polyneuritis, birth pal- sies and progressive muscular atrophy. A consideration of the following facts will usually make the diagnosis easy: 1. The age of the patient. 16 242 DISEASES OF THE NEEVOUS SYSTEM 2. The presence of an epidemic of poliomyelitis and its seasonal variations. 3. The abrupt onset and rapid development of extreme paralysis. 4. The tendency to improve. 5. The segmental character of the paralysis; the absence of bladder disturbance and objective sensory losses. Prognosis. — The patient rarely dies, either from the disease or its sequelae. In some epidemics, however, there has been a mortality of about 12 per cent. He always improves, but he recovers completely in only a small num- ber of cases. In these, no cell necrosis has occurred but only cell deterioration. Treatment. — The patient should be isolated in an airy room, the windows of which are well screened. Faeces, urine and nasopharyngeal discharges must be rendered inocuous by antiseptics as in a case of typhoid fever. A nasal spray of 1 per cent, hydrogen peroxide must be used and maintained for some months. Uro- tropin must be given immediately and maintained in large doses for at least two weeks. All persons exposed to poliomye- litic infection should be encouraged to use antiseptic nasal sprays and should be given urotropin gr. v. twice daily as a prophylactic measure. Rest is essential. The bowels should be opened and a diu- retic given (calomel gr. ii. ; tartrat. potas, gr. XX.). Lumbar puncture will aid diag- nosis and relieve, at least temporarily, Fig. 108. — Poliomyelitis, late spinal congestion. Sedatives may be re- stage, showing atrophy of leg and • j • j.u i j. a-*- t ;^^ ,•„ deformity of foot. {Schmibornand qmred m the early stages. Strychnme is Krieger.) to be avoided. A hot pack twice a day will relieve tenderness. Aconite and sweet spirits of nitre may be given to reduce fever. The limbs should be wrapped in cotton and defended from pressure. When hypersensitive- ness has disappeared gentle massage and passive movements should be instituted, for from five to twenty minutes five or six times a day. Elec- trical applications should be begun cautiously, at first three times a week and, when well borne, daily. Frequent systematic rubbings given by a relative are frequently more productive of good results than are less THE DISEASES OF THE SPINAL CORD 243 frequent treatments given by a trained masseuse. The child must be trained to try to use the hmb as much as possible : with this in view it is inadvisable to keep the patient confined to bed after the acute stage has passed : the child should be laid on a well-blanketed floor and encouraged to crawl and push its way about the room. For older children and adults much can be done by the studious performance several times daily of exercises adapted to develop the affected muscles and those adjacent to them. Generally speaking, it is a mistake to employ orthopaedic appa- ratus until two years have passed, nor should they be employed even then unless it be clear that developmental exercises are useless. The same attitude should be adopted toward operations for the correction of deformi- ties or for restitution of function by nerve grafting ; such procedures are often of the greatest value but must only be employed after much con- sideration in selected cases. Foster Kennedy. CHRONIC ANTERIOR POLIOMYELITIS {Remitting Spinal Amyotro'phy) This disease goes under the name of chronic poliomyelitis, but it shows a closer relation to obliterative vascular disease, than to a simple inflammatory process. Etiology. — In my experience syphilis has always been present; others give as causes lead, overwork, exposure and trauma. It occurs only in adults and in men more than women. Symptoms. — The disease comes on subacutely; that is to say, in one or two months. It reaches its height in three or four months and before the end of a year has come to a standstill. The symptoms then usually remain quiescent or improve for one or two years, then take a fresh start and present about the same course, involving new and usually neighboring groups of cells. It may progress no further after this second attack, but there sometimes occur fresh invasions involving finally the bulbar nuclei and leading to a fatal issue. The symptoms of paralysis or weakness precede the atrophy, which is a flaccid one, but in other respects it resembles the ordinary spinal amyotrophies. There are slight or no symptoms of involvement of the pyramidal tracts. There is sometimes slight fibrillary tremor. The cUsease usually affects the upper extremities and especially the shoulder girdle, but eventually involves the forearms and ascends to the cervical region (Figs. 109, 110). The tendency to cessation of progress and even to improvement has characterized cases reported by Eisenlohr, and Landouzy and Dejerine. Few cases with autopsy have been reported, and these showed vascular lesions with secondary degeneration of the anterior horn cells and some- times of the anterior fundamental column. 244 DISEASES OF THE NERVOUS SYSTEM The Prognosis in the past has been uniformly bad. Treatment. — The history of syphihs in my cases leads to the recom- mendation of an anti-syphilitic treatment for this trouble, and this must be very active and persistent. Fig. 109.- -Chronic anterior poliomyelitis, luetic right shoulder and left forearm and hand are most affected. Senile Paraplegia. — Paraplegia sometimes develops in the senile and does so in a somewhat characteristic manner. In the spastic form the paralysis comes on slowly, the legs are stiff, the gait shuffling, the steps very short and the feet are barely raised from the ground. There is some pain in the back, at times, but no marked sensory symptom. There may be decrease in reflexes or no change. The sphinc- ters are not involved. The gait and station suggest paralysis agitans, and some cases may be considered a symptomatic type of this trouble. A paraplegia spastica senilis described by Demange and Oppenheim THE DISEASES OF THE SPINAL CORD 245 is of arteriosclerotic origin and accompanied with signs of pyramidal de- generation. In the flaccid form of senile paraplegia the patient progressively but intermittently grows weaker in the legs, the muscles waste, the gait is feeble, with short steps and great difficulty in going up and down stairs. There is sometimes lumbar pain, and the sphincters may be involved. The patients sometimes improve, but even- tually become helpless, though the prog- ress is slow. I have had an autopsy in one case and found very marked arterio- sclerotic changes in the anterior horns of the lumbar region with softening. I think that in flaccid senile paraplegias and some of the rigid types there is arterial sclerosis of the anterior vessels with softening and at times small hemorrhages (Fig. 112). Starr thinks that some forms of pro- gressive senile paraplegia are neuritic and that others are due to a progressive muscu- lar disease (dystrophy) . ACUTE ASCENDING PARALYSIS (Landry's Paralysis) Acute ascending paralysis is a disease characterized by a rapidly [developing paralysis which begins in the legs and then involves in turn the trunk, arms, respiratory and throat muscles, usually- ending in death. There is little disturb- ance of sensation, no atrophy or changes in electrical irritability, and no involve- ment of the sphincters. It is thought to be related to acute anterior poliomyelitis. "The Heine- Wickmann under the name Medin disease" groups together: 1. Acute poliomyelitis. 2. Landry's paralysis. 3. Bulbar and pontine forms. 4. Encephalitic forms. Fig. 110. — Remitting muscu- lar atrophy. Patient is unable to raise the head. 5. Ataxic forms. 6. Polyneuritic forms. 7. Meningitic forms. 8. Abortive forms. This assumes that the same infectious or toxic agent may attack the different parts of the nervous system. This is practically the conception taught in the earlier editions of this work. However, all forms of acute ascending paralysis as it is seen clinically are not due to the poliomyelitic organism. 246 DISEASES OF THE NERVOUS SYSTEM Etiology. — The disease is a rare one. It occurs chiefly between the ages of twenty and forty; men are affected oftener than women. Ex- posure is an exciting cause, and it occurs sometimes after acute infectious fevers. The form of rabies known as ''paralytic" causes a disease which is apparently identical with Landry's paralysis. The form which is caused by the micro-organism of anterior poliomyelitis occurs naturally when epidemics of this disease are present. It is not yet proved that true ascending paralysis is always poliomyelitic. Symptoms. — There may be slight premonitory symptoms for a few days, consisting of numbness in the extremities, pain in the back or limbs and malaise. The first definite sign of the disease is weakness in the legs, which rapidly increases, until in a day or two the patient cannot walk. The paralysis soon involves the trunk, arms and the muscles of Fig. 111. — Lumbar coid m a case of senile paiaplegia, showing atrophy of anterior cornua secondary to vascular disease. respiration; the medulla is last affected, and then respiration becomes difficult; swallowing and articulation may be impossible. In rare cases there are facial and eye palsies. During the course of the paralysis there is little pain or sensory disturbance, but some degree of anaesthesia may occur. The deep reflexes are aboHshed. There are no vasomotor and no secretory disturbances, no noticeable atrophy, and no degenerative reactions in the affected muscles. The bladder and rectum are involved only in rare cases. There may be slight initial fever, but none occurs after the disease has well set in. The mind remains clear. The disease, as a rule, ends fatally, and it usually runs its course in less than a week. Death has occurred in forty-eight hours. On the other hand, death has been postponed three or four weeks. In other cases the disease stops short of the medulla. The patient THE DISEASES OF THE SPINAL CORD 247 becomes totally or nearly paralyzed below the neck. He then begins slowly to improve, and this improvement continues for one or two j^ears. Eventually a fair degree of health is obtained. Variations. — The disease has been known to begin in the medulla or cervical region and descend. Pathological Anatomy. — There are three pathological conditions which may cause the symptoms of acute ascending paralysis: (1) Acute poliomyelitis; (2) acute high transverse myelitis; (3) acute multiple neuritis. These different conditions may coexist at times in the same case. Usually as stated the micro-organisms of epidemic poHomyelitis anterior are the cause. In some cases the symptoms are due to paralytic rabies, in others, the streptococcus, the diplococcus and pneumococcus causing intense diffuse myeHtis have been found (Fig. 112). The prognosis is very grave, but not absolutely bad. If there is reason to suspect the case of being one of paralytic rabies, no hope can be offered. Diagnosis.— This must be made from the ordinary forms of acute poliomyelitis, acute myelitis, acute multiple neuritis and periodic family paralysis. Its acute ascending course, absence of fever, of anaesthesia, of atrophy, decubitus, sphincter troubles, and especially the absence of degenerative electrical reactions, of a family history with a history of previous attacks are sufficient to enable one to make the diagnosis. The age of the patient, and the presence or absence of an alcoholic history should be considered. Lumbar puncture should give help. (See anterior polio- myelitis.) Treatment. — This consists of warm baths or packs, colon washings, and ehminative treatment. Large doses of ergotin, gr. ij., every hour have been successful in one case. Urotropin should be given, and lumbar punctures should be made. CHAPTER XIII SCLEROSES, DEGENERATIONS, SYPHILIS Introductory. — The term sclerosis is somewhat misleading. Properly speaking, it is the fibroid (and neuroglia) induration which results from degeneration, destruction or inflammatory irritation. We speak of degenerative sclerosis, of an inflammatory and of a, neuroglia sclerosis, or of a sclerosis of mixed origin, according to the nature of the primary disease which caused it. Some writers look upon primary degenerations as parenchymatous inflammations. There is no objection to this point of view as long as we agree upon the essential character of the process. The words ''degeneration" and ''sclerosis" are often used to indicate the same thing, one being the pathological process, the other the anatomical result. I shall use the term "sclerosis" here in its pathological sense, meaning the process of hardening, in presenting a classification of the degenerations of the spinal cord. I Posterior spinal sclerosis (locomotor I ataxia). Lateral sclerosis. Combined sclerosis. Hereditary ataxia. Progressive muscular atrophy, amyotrophic lateral sclerosis. / Ascending and descending degenera- \ tions. f Chronic myelitis and sclerosis fol- Inflammatory, infec- I lowing destruction of cord.] tive. [ Multiple sclerosis. Spinal scleroses. Primary and degenera- tive. Luetic, toxic, connate. (Parenchymatous in- flammation) Secondary, ical. physiolog- The tendency of late years has been to classify diseases of the nervous system on an etiological basis, e.g., as syphilogenous, infectious, tubercular but I believe it still is best to follow the clinical and anatomical methods. Primary degeneration (parenchymatous inflammation) or primary sclerosis, as one may say for convenience, is a process which begins in the neuron itself and ends in its atrophy, with substitution of neuroglia and connective tissue. As to its nature, so far as the microscope shows us, it is a gradual decay and death of the neurons. In some sclerotic processes, like locomotor ataxia, the sclerosis is due to the destruction of the neuron by the toxic activity of the spirochceta pallida. The decay is accompanied by the development of irritating products, leucomains toxalbumins, which may produce so active a change in the connective 248 SCLEROSES, DEGENERATIONS, SYPHILIS 249 tissue as to lead to something resembling a secondary or reactive inflam- mation. This is never of high grade, however, and in some forms of tabes is very slight. Fig. 112. — Acute ascending myelitis (^Landry's paralj'sisj. Fig. 113. — Ascending degeneration in third cervical segment, from transverse lesion in second dorsal, showing degeneration of columns of Goll and cerebellar tracts. In progressive muscular atrophy the decay and death produce few irritating products, though enough, perhaps, to account for the fibrillary twitchings and occasional tremulous condition of the muscles. 250 DISEASES OF THE NERVOUS SYSTEM. The ultimate cause of these degenerative processes is the micro-or- ganism of syphilis, toxins, and poisons, and congenital defect in the vitality of the neuron, a condition called by Gowers abiatrophy. Secondary Degenerations of the Spinal Cord. — When any of the long- fibre tracts of the cord are cut across or destroyed, there soon results a degeneration. This extends up or down in accordance with the direc- tion in which the tracts carry impulses. Thus, when the crossed py- ramidal tract is cut across the degeneration extends down; when the column of GoU is involved it extends up. The degenerative process begins almost immediately and is complete in a few weeks. The myelin sheath swells, gradually breaks up and disintegrates; the axis-cylinder is involved next. At the same time the connective tissue and neuroglia proliferate and take the place of the wasted nerves. Finally, long tracts Fig. 114. — Descending degeneration in eighth dorsal segment from lesion in second dorsal, showing degeneration in direct and crossed pyramidal tract, vestibulo-spinal and comma tracts. of connective tissue have taken the place of the nerve tissue. The process may not be a complete one if the lesion does not entirely destroy the tract. Secondary degenerations complicate and add to the pathological change in all organic diseases of the cord. In brain disease, involving the motor tract, as in hemiplegia, secondary degeneration extends into the cord and adds to the seriousness of the disease. Degenerations of the spinal cord, however, do not extend up to the brain to any extent, except in the case of disease of the antero-lateral ascending and cerebellar tracts. Those forms of sclerosis found in chronic myelitis are similar to the connective-tissue scars following destructive inflammation elsewhere. A person who has a chronic myelitis has a cicatrix in his spinal cord. SCLEROSES, DEGENERATIONS, SYPHILIS 251 The sclerosis of inultiple sclerosis is probably inflammatory also, but it is a neuroglia rather than a connective-tissue cicatrix. Short Degenerating Tracts. — ^By the use of more delicate stains other degenerating tracts have been discovered. These are described under the head of the anatomy of the spinal cord. The short-fibre tracts degenerate only a little way up and down. SYPHILIS OF THE NERVOUS SYSTEM Syphilis begins to attack the nervous system in two ways: by round- cell infiltration and exudation affecting the blood-vessels of the meninges of the cord and brain; and by the development of a parenchymatous degeneration of the neurons. Hence we have: 1 . Meningo-vascular or exudative syphilis and 2. Parenchymatous or degenerative syphilis. 1. Meningo-vascular syphilis takes various clinical forms: (a) Cerebral syphilis. (6) Spinal syphilis. (c) Cerebrospinal syphilis. 2. Parenchymatous syphilis takes the forms of: (a) Paresis. (5) Tabes dorsalis. (c) Muscular atrophy. (d) Combined system degeneration. (e) Various combinations of the above. Epilepsy and optic atrophy are added by Head and Fearnsides. In many cases there is first the exudative and proliferative syphilis, attacking the blood-vessels and meninges, but in about half the cases this approach is so slight that the first noticeable effect of syphilis of the nerv- ous system is a degenerative process. Syphilis begins openly to attack the nervous system in from two to twelve years after infection, but it may occur within six months or as late as thirty years. If it occurs early the meningo-vascular form dominates and may be the only one. If it occurs late, the parenchymatous and degenerative process is the chief and often the only one. The causes leading the syphilis organism to attack the nervous system are a hereditary weakness or predisposition; abuse of the nervous system, especially in sexual excesses and in laborious occupation and, finally, the abuse of alcohol and food and the adoption of a mode of life which in gen- eral tends to weaken and exhaust the system. There is some evidence that mild and hardly noticeable forms of Syph- ilis are those most likely to attack the nervous centres later. It is a fact that in about half the cases the patients deny either knowledge of any infection or of the development of secondary cutaneous symptoms (rashes, sore throat, alopecia, pains, etc.). This apparent immunity of the cases 252 DISEASES OF THE NERVOUS SYSTEM with flagrant secondary symptoms may be due in part to the fact that these latter are more vigorously treated. The absence of any known pri- mary lesion is explained by the existence of an infection without local reaction, or by its being masked by a gonorrhoea. Of all persons affected by syphilis it is probable that in 2 to 10 per cent, the nervous system becomes involved. Men are rather more often affected than women with nervous syphilis, and rather more in pro- portion to the infection because they are more subject to the exciting causes. The conditions of modern civilization have increased the amount of nervous syphilis, and this increase is mostly in the degenerative types of paresis and tabes. Symptoms. — Since syphilitic changes may attack any part of the nervous centres, the symptomatology of nerve syphilis is necessarily a varied one. The reader can perhaps best understand the way in which the disease acts by having presented, first, a series of tables showing on the one side the clinical symptoms, on the other the chief anatomical changes that underlie them. I. PRODROMAL SYPHILIS. II. MENINGO-VASCULAR SYPHILIS (a) Of the Brain. Clinical Symptoms Severe headache, vomiting, vertigo, men- tal dullness, and irritability, attacks of somnolence or coma, convulsions, cra- nial-nerve palsies, optic neuritis, hemi- plegia, brain stem and bulbar palsies, (fe) Of Brain and Cord. Many of the brain symptoms as above, spastic paraplegia, etc. (c) Of Spinal Cord. Paraplegia, Brown-S e q u a r d paralysis. Muscular atrophy. Spastic para- plegia and ataxia. (d) Of the Nerve-roots and Nerves. Cranial-nerve palsies, cauda-equina symp- toms, local palsies of peripheral nerves. Muscular atrophy. (Hereditary and Acquired). Anatomical Change Syphilitic meningitis, arteritis and phle- bitis. Meningitis; diffuse, disseminated, or lo- calized meningo-myelitis. Meningo-myelitis, gumma, localized soft- enings from obliterative arteritis. Root neuritis, gummatous neuritis. III. PARENCHYMATOUS SYPHILIS (a) Of the Brain. Paresis. Bulbar palsy. (6) Of the Cord. Tabes dorsalis. Spastic tabes. Muscular atrophy. (c) Of Brain and Cord. Tabo-paresis. IV. COMBINATIONS OF II AND III. (Hereditary and Acquired). Meningo-encephalitis. Meningo-myelitis . SCLEROSES, DEGENERATIONS, SYPHILIS 253 I. Prodromal Syphilis. — The development of syphilis of the nervous system is often preceded by prodromal symptoms of a definite and recognized character. Such symptoms are especially apt to precede paren- chymatous syphilis. They can be arranged somewhat as follows : Meningeal Prodromata.- — These consist of annoying and often severe headaches, temporary attacks of cranial nerve palsy; attacks of dizziness; dyssesthesiae which are particularly referred to the head and back of the neck. They are symptoms which can be explained by the supposition that there is a slight grade of meningitis in some part of the brain or cord or else a slight degree of obliterative arteritis leading to local or general disturbances of the brain function. Luetic Neurasthenia. — A most common group of symptoms is one which may be called luetic neurasthenia. The conditions is charac- terized by some mental depression, inability to use the brain effectively or long, annoying disturbances in the shape of parsesthesia of the head and neck and extremities, weakness of the sexual function, and the general group of symptoms which come under the description of neurasthenia of a rather depressive and often hypochondriacal type. Luetic Deterioration. — In the milder forms of luetic neurasthenia, there is not so much an absolutely nervous exhaustion as a slight change in the character and in the effectiveness of the work of the individual. For a number of years before the development of a frank nervous lues, for example, the patient will have been recognized as having become a somewhat different person. He is not as attentive to his work or as keen in memory, or as efficient in his activities generally. He is more irritable and difficult to work with or to live with. Luetic Melancholia. — Parenchymatous syphilis, particularly of the paretic or tabo-paretic type is not infrequently preceded by a definite attack of melancholia and this may be of very severe hypochondriacal or even suicidal character, running its course like other forms of melan- cholia. Much less frequently the infection is introduced by an attack of mania which may be mistaken for the onset of true paresis. II. Meningo-vascular S3^hilis. — Of the Brain. — In its most common form this shows itself by a gradual development of severe and persistent headache. This is usually associated with vertigo, sometimes with nausea and vomiting. After the headache has developed and has lasted for a time, or even without much delay, there comes on sometimes an attack of hemi- plegia. Preceding the hemiplegia, or in some cases without the hemiplegia, there are paralyses of the cranial nerves, more especially of the nerves of the eye. Optic neuritis is somewhat frequent. The pupils may be irregular in outline and sluggish to light. There may be, before any paralyses develop, attacks of epileptic convulsions, either general or partial. Without any paralyses or with simply cranial-nerve paralyses, there may 254 DISEASES OF THE NERVOUS SYSTEM develop attacks of somnolence and coma. Even if such attacks do not appear, the patient often shows a mental irritability and weakness, a slowness of the reasoning process, and incapacity to fix the attention such as is observed in connection with brain tumors, only with nerve syphilis these symptoms are not usually so marked. Polyuria and poly- dipsia are symptoms which are occasionally met with. The disease may take the form of a meningo-encephalitis producing symptoms of mental and physical disturbance not distinguishable from early paresis (men- ingo-vascular paresis). Of Brain and Cord. — Very often some of the above groups of symptoms are associated with spinal disease. There is evidence of meningo- myelitis, or of obliterating arteritis and as a result, we have paraplegia; or the meningeal syphilis of the brain may be associated with degen- erative syphilis and we have lightning pains, ataxia and other signs of an occurring tabes. Of the Cord. — The symptoms in spinal syphilis are commonly those of a transverse myelitis involving most often the lower part of the dorsal and upper part of the lumbar cord. This myelitis usually comes on rather slowly with the ordinary symptoms of a chronic or subacute transverse myelitis, there being a progressive paraplegia with spasticity of the legs and a good deal of pain. The condition is known as syphilitic spinal paralysis (see p. 230). Spinal syphilis may show itself also by the development of gummatous nodules which grow from the meninges, press upon the cord, and produce the symptoms of a spinal tumor. Spinal syphilis more rarely develops itself in three or four different foci, produc- ing the symptomatology of disseminated myelitis. Finally, a spinal endarteritis may lead to focal hemorrhage and softening. Pathology. — I have already given some indications of the pathological changes produced by syphilis. The disease affects the nervous system (1) by producing a meningitis with exudation, (2) by producing gum- matous masses, (3) by producing an inflammation of the arteries, and (4) by so influencing the neurons so as to lead to their progressive degen- eration. Of all these forms of anatomical change it is the arteries that are most often affected, and particularly the arteries at the base of the brain. Syphilitic meningitis is characterized by the proliferation of round cells and the preponderance of an exudate which has a tendency to in- filtrate into the nervous tissues. The anatomical characteristics of the syphilitic gumma must be studied in special text-books. The inflamma- tion of the arteries attacks first the external coat and adventitia, produc- ing there an enormous multiplication of round cells. The external coat becomes weakened, and as a result there develops beneath it, between the intima and the elastic layer, another exudate which constitutes what SCLEROSES, DEGENERATIONS, SYPHILIS 255 is known as endarteritis. In syphilitic arteritis, therefore, there is both a peri-arteritis and an endarteritis; the former being usually the primary and most essential process. The endarteritis, however, as it develops gradually produces an occlusion of the arteries. This cuts off the circu- lation of the blood and leads to softening of the part. There is also a development of a hyaline degeneration in the arteries, which some regard as a very essential part of the anatomical change (Fig. 116). Diagnosis. — -The diagnosis of meningo-vascular syphilis is based upon the history of an infection, the irregularity and fugacity of the symptoms, a study of their peculiar clinical characters as described above. But all these things have been made of minor importance compared with those furnished by the reactions of the blood and the character of the cerebro- spinal fluid (see- Serology of Syphilis). The headache of syphilis is rather characteristic. It may attack any part of the head, but is usually unilateral or irregular, or again it may be bilateral in its distribution. The pain is very intense and sometimes exhibits a certain periodicity. It is not necessarily worse at night. It is apt to last continuously for from five days to three or four weeks. Headache of this character, followed by the paralysis of one or more cranial nerves or by an attack of hemiplegia, is extremely suggestive of syphilis. Optic neuritis is very liable to occur when the disease shows other evidences of being situated at the base of the brain. This optic neuritis is associated with contraction of the visual field, and a charac- teristic feature of this contraction is that it varies a great deal from week to week. Prognosis. — Unquestionably the outlook is much more favorable than it is for any other organic disease of the nervous system. When the syphilitic process has not produced so much arterial disease as to lead to obliteration of vessels and softening, a very great degree of im- provement and even a recovery may be expected. Headaches, cranial and other nerve palsies, parsesthesias and the neurasthenic and psychotic prodromata of exudative syphilis are usually curable. Lesions of the convexity are usually amenable to treatment. Syphilitic hemiplegia has a not much better prognosis than hemiplegia from other causes. Syphilitic myelitis has a not very good prognosis, but it is better than that of myelitis due to trauma. In all cases the results are enormously better the earlier treatment is instituted. Even bulbar and spinal paraly- ses, and meningeal or exudative paresis are curable or enormously relieved. Treatment. — A laborious life full of worry and anxiety, in which the patient attempts to help himself along with stimulants, is surely provoca- tive of nervous syphilis. These elements of cause must be removed. In recent years the treatment of syphilis by hypodermic injections 256 DISEASES OF THE NERVOUS SYSTEM of the bichloride or saHcylate of mercury has been widely adopted, and better results have been obtained than by inunction or by administra- tion by the mouth. One or two grains of the salicylate mixed with a liquid petroleum oil may be given twice a week. The effective treatment of nervous syphilis lies at present in the wise and persistent use of sal- varsan combined as seems needed with mercury and iodides, tonics and hygienic measures. The details are given under Paresis. Other drugs which are of value are the ordinary tonics, such as iron, quinine, and the bitters and mineral acids. Plenty of good food, out- door air, and all those things which will improve the general health of the patient are indicated. Syphilis of the Nerves. — Syphilis rarely affects the peripheral nerves; there are, however, occasional deposits of syphilitic exudate producing the ordinary symptoms of irritation and compression of nerves. There is said to be a form of multiple neuritis produced by syphilis, but its actual existence has not been demonstrated. Syphilis is sometimes known to attack the roots of the cranial nerves, producing a root neuritis. It attacks the roots of the spinal nerves when the spinal membranes are involved. In rare cases, where it involves only the anterior roots it causes a form of muscular atrophy. III. Parenchymatous Syphilis. — The syphilitic parenchymatous processes lead to locomotor ataxia, general paresis, tabo-paresis, pro- gressive spinal paraplegia, and sometimes to a form of muscular atrophy due to parenchymatous or vascular syphilis of the anterior horns. Meningeal syphilis may be combined with parenchymatous forms, so that we sometimes see locomotor ataxia associated with meningo-myelitis; and we see paresis associated with meningeal syphilis. Hereditary Syphilis. — ^Inherited syphilis will lead to anatomical changes and clinical manifestations resembling in all respects those of acquired syphilis. Inherited syphilis, in other words, may produce headaches, cranial-nerve palsies, hemiplegia, epilpsy, mental disorders juvenile paresis, feeble-mindedness, and degenerative spinal diseases. The disease probably is the cause of a considerable proportion of the cases of chronic hydrocephalus and of many of the cases of meningitis. The peculiarities of hereditary syphilis show themselves rather more in diffuse symptoms such as would be attributed to a meningitis of the convexity or circulatory defects; in other words, convulsions and mental weakness are rather more frequent, while hemiplegia and cranial-nerve palsies are com- paratively rare. Hereditary syphilis also rarely attacks the spinal cord, although it is not unlikely that it is a factor in the production of some of the hereditary diseases of that organ. Hereditary syphilis develops at any time from birth to the eighteenth year, but most commonly under the age of five years. Serological tests furnish the diagnosis. SCLEROSES, DEGENERATIONS, SYPHILIS 257 The presence of the Hutchinson teeth, the hazy cornea, and deafness or other ear trouble were the old diagnostic criteria of hereditary syphilis. LOCOMOTOR ATAXIA {Posterior Spinal Sclerosis, Tahes Dorsalis) Definition. — Locomotor ataxia is a chronic progressive luetic disease, involving primarily the posterior spinal ganglia, roots and neurons, and later the spinal cord and peripheral nerves. It is characterized clinically by inco-ordination, pains, anaesthesia, and various visceral, trophic and other symptoms, and anatomically by a degenerative sclerosis chiefly marked in the posterior columns of the cord and posterior roots, and to a less extent in the peripheral nerves. Forms. — Besides the common and typical form, there are anomalous and complicated types. Types. 1. Common form. 2. Neuralgic. 3. Paralytic. 4. With initial optic atrophy. /-I 1- X J f With muscular atrophy. Complicated „^.,, ,. , ^ -^ ^ With other scleroses. With general paralysis. forms. Etiology.— The disease occurs oftenest in middle life, between thirty and forty, next between forty and fifty. It may occur as early as the tenth and as late as the sixtieth year. In the very early cases it is usually due to hereditary syphilis. It is much more common in males (about 10 to 1). Hereditary influence is only indirect; i.e., the parents may transmit lues or a neurotic constitution. Diathetic influence is slight. Exposures to wet and cold, combined with muscular exertions, are effec- tive causes. Soldiers, travelers and drivers are rather more susceptible. Excessive railroad traveling, excessive dancing with exposure, favor the development of the disease. Excessive sexual intercourse, combined with irregular living, is a predisposing cause. Syphihs is the one important and essential cause. A personal his- tory of the disease is obtained in from 60 per cent, to 90 per cent, of the cases. Serological tests show almost invariably evidence of syphilis and statistics indicate that about 1 per cent, of those infected with syphilis become tabetic unless treated actively and by modern methods.^ Lack of * The following statistics from my own experience show the physiognomy of the disease in a cosmopolitan American city. Total cases, 248; males, 226; females, 22. Ages when disease began: Average age at time of onset, 40; beginning a year or two earlier in private patients and in those with a history of syphilis and active anti- syphilitic treatment (Collins). Average period between infection and tabes, 10 to 20, ranging from one and one-half to twenty-five years. This is much longer than Erb's estimate, but is reached by three independent studies of my cases. Average duration 17 258 DISEASES OF THE NERVOUS SYSTEM proper treatment for syphilis is believed to favor the development of the dis- ease, but a careful study of the statistics of my clinic and of his own cases by Dr. Joseph Collins seems to show that antisyphilitic treatment as formerly carried out does not prevent the disease if the other favoring conditions, such as exhausting work and sexual or alcoholic excesses, are present. Among exciting causes are profoundly depressing emotions, acute infec- tive diseases like typhus, pneumonia and rheumatism, difficult labors with severe hemorrhage, prolonged lactation and excessive smoking. Edinger lays great stress on the over-use of the limbs. Trauma as an apparently, exciting cause is noted in a very small percentage of cases (less than 1 per cent.). In my experience there has always been evidence that the disease was already in the system but had not been recognized in these cases. Symptoms. — ^The disease is generally divided into three stages: the initial or pre-ataxic, the ataxic and the paralytic. 1 . The pre-ataxic or initial stage. Previous to any other notable symp- toms, the patient may have for months or years attacks of sharp pains dart- ing through his limbs or about his trunk. With this he may have only Argyll-Robertson pupils and loss of knee-jerks and ankle-jerks or of ankle- jerks alone. To this may be added a little uncertainty of gait. Or instead of the pains he may have for a long time gastric crises. Occasionally nothing is noted at first but loss of sexual power or a weak bladder. A gradual development of optic atrophy and blindness may also pre- cede nearly all other symptoms. Ocular palsies and hypotonia may also be among the preataxic symptoms. In many cases the disease does^not advance beyond the preataxic stage, and this is particularly true of neuralgic and optic forms of tabes. When the ataxic process sets in, in addition to the symptoms men- tioned the patient notices a slight uncertainty in walking, especially at night; he has numb feelings in his feet. His sexual function becomes weak, his control over the bladder slightly impaired. He has temporary attacks of vertigo and of double vision. A sense of weariness oppresses him, even though he has made no exertion. He may have attacks of vomiting and gastralgia, and may suffer from constipation, with hemor- rhoids and rectal pains. Arthropathies may appear. Some hypotonia may be present. Such symptoms may last a few months or several years ; often the disease is kept in this stage. 2. The ataxic stage. The gait now becomes so unsteady that others of disease when seen by me, eight years; average duration of Ufe in five fatal cases, twelve years, ranging from five to twenty years. Comphcations: optic atrophy, 6 per cent. ; marked arthropathies, 5 per cent. ; with general paresis, 4 per cent. ; para- plegia, 1.5 per cent.; hemiplegia, 1.6 per cent.; eye palsies, 8 per cent. About 10 per cent, of 300 private cases were complicated with paresis — tabo-paresis. The compli- cation is smaller in dispensary cases. High tabes (arm tabes), 5 per cent.; cranial nerve palsies, 10 per cent.; ophthalmoplegia in 1 case, progressive muscular atrophy in 3, laryngeal palsies in 3. SCLEROSES, DEGENERATIONS, SYPHILIS 259 notice it; the patient has to have help, and when walking he has to watch his feet and the ground. If he stands with his eyes closed, he totters and may fall. His feet feel as though there was a layer of cloth or cotton between the soles and the ground. Paroxysms of lightning-like pains continue to attack the legs, chest and arms; tests show anaesthesia of the toes and feet or in areas on the legs and on the trunk. A sense of con- striction is felt around the waist. The sexual power is lost; the bladder is weak, and care has to be taken to empty it. The bowels are consti- pated; at times he has attacks of intense pain in the epigastrium, with vomiting and perhaps a diarrhoea coming on without cause. The pupils "i-^jt Fig. 115. — Meningo-vascular disease in syphilis of brain and cord. {Bruce.) are small often unequal and irregular and do not react to light, but do react to accommodation; vision is still good. The incoordination and pain and anaesthesia after a time affect slightly the arms. The muscles become relaxed and atonic. The face shows atonia; also there is often a pseudo-ptosis of one or both lids. This stage lasts several years. 3. The paralytic stage. After several years with various remissions and improvements, the patient loses altogether the power of walking. His legs are somewhat wasted, but the muscular strength is fairly good. The anaesthesia and ataxia are very great. The patient does not feel the prick of a pin or touch of the hand; nor with closed eyes does he know where his legs are. His bladder is anaesthetic and paretic, so that the urine has to be drawn. The pains are much less, but are still present at times. The arms are more involved, but never so seriously as to make 260 DISEASES OF THE NERVOUS SYSTEM them useless like the legs. The intelligence remains good, and the patient may continue bedridden for years, dying finally from some intercurrent affection. The following table shows the prominent symptoms in the usual order of their appearance : ^ First stage (half to thirty years) Second stage (two to ten years) Third stage (two to ten years) Motor [ Eye palsies. Ataxia. i Muscular weakness and hypotonia. Less. Increased. Paresis, increased. Increased. Increased. Paraplegia. Increased. Sensory 1 Pains. 1 Pains. Painless. Anaesthesia. Increased. 1 Excito-reflex Loss of knee-jerk. A.-R. pupil. Trophic Arthropathies. More rare. Rare. Visceral Sexual weakness. Vesical weakness. ■ Constipation. Gastric crises. Increased. J Decreased. Increased. Decreased. Special senses j Diplopia. ■ Optic atrophy. Rare. Rare. Deafness. Increased. Increased. Analysis of Symptoms. — A study of the symptoms in tabes produces enormous results in the way of clinical phenomena. The student should not, however, lose his sense of proportion in going over these data. After all, the serious things in tabes are not so numerous. They are his ataxia and loss of ability to walk well, his pains, sexual and bladder weakness, hypotonia, anaesthesia, crises, especially gastric, arthropathies, and optic atrophy. Locomotor and static ataxia are present very early, but only to a moderate extent. Tests, such as making the patient walk and stand with the eyes closed, noting the position of limbs and the weight of objects, will reveal an ataxia due largely to beginning anaesthesia of the joints and tendons. ^ The disease attacks different levels of the cord in about the following proportion : Optic tabes, 5 per cent. ; high (arm) tabes, 5 per cent. ; dorsal (abdominal and trunk) tabes, 20 per cent. ; lumbar and sacral tabes, 60 per cent. To these add tabo-paresis, 10 per cent. SCLEROSES, DEGENERATIONS, SYPHILIS 261 The patella-tendon reflex or knee-jerk is abolished very early in all typical cases. This constitutes a very important symptom, therefore. The ankle-tendon reflex is lost early in the disease, but not, as a rule, so early as the patella reflex. Inequality or diminution in the activity of these reflexes are significant when taken in connection with other symp- toms. An inequality is more im- portant than a general diminu- tion. These reflexes of knee and ankle may disappear early in the disease and return under treat- ment, but this is rare and later they may disappear again. The gait and station in ataxia are characteristic. In walking, the patient keeps his eyes on the ground and on his feet. The lat- ter he throws out rather forcibly, owing to over-action of the exten- sors of the foot. In watching such a patient walk barefooted, the extensor tendons can be seen to stand out with each forward movement of the limb. The foot is brought down sharply on the heel and the legs are spread apart a little. Turning a corner, turn- ing around and going downstairs are done awkwardly, and the pa- tient is apt to totter and fall. Walking on a chalked line is very difficult; so also is walking back- ward. The gait improves after the patient walks a while, and he will generally say that the prac- tice of walking does him good. Still, he soon gets tired (Fig. 116). Hypotonia, or relaxation and lengthening of the tendons and muscles, is usually an early and always an important symptom, but is sometimes not very marked until later in the disease. This is what causes the genu incurvatum; when present in high degree it is an unfavorable sign, for it is apt to lead to deformities and promote arthropathies (Figs. 118, 122). Myoclonic and tic-Hke spasms may affect the face and body. Myokymia (fibrillary and wave-like contractions of the muscles) is Fig. 116. -Showing station in second sta| of locomotor ataxia. 262 DISEASES OF THE NERVOUS SYSTEM an early and rare complication. It occurs usually in cases with rather rapid onset, and affects most the muscles of the lower limbs. Though not painful, it is a very annoying phenomenon. Severe rectal neuralgia, associated perhaps with hemorrhoids, is sometimes an early symptom. Persistent neuralgia and functional dis- turbance of the bladder and rectum should cause suspicion of ataxia. Lancinating or lightning pains occur and are very characteristic. The pains dart down the legs along the course of the sciatic, or they suddenly appear as patches of pain on the foot or leg or thigh ("spot pains ")• The pain comes unexpectedly and with such severity that the patient involun- tarily jumps or jerks the hmb. He speaks of his "jerking" and ''twitch- ing pains." The pains may affect the bowels or be felt as a squeez- ing sensation around the waist (girdle pains). The pains of ataxia are often the most obstinate and distressing symptom. They usu- ally come on in great intensity once or twice a month, and last for two or three days. They then leave the patient for a time. They are often worse in cold and damp weather. In some cases the pains are almost continuous, coming on, if not every day, at least two or three times a week. Such cases are associated with much cutaneous hyperses- thesia, especially during the attacks. This type of cases is called "the neuralgic." The patients rarely have as much ataxia, paresis or visceral troubles as the typical forms present, and in certain respects such cases are favorable. Sometimes the pains are accompanied with erythema or herpes zoster showing an acute involvement of a posterior ganglion. The pains of the disease continue well into the second and even third stage. Meanwhile the anaesthesia becomes much more marked. It af- fects most the feet and next the legs, rarely extending much over the thighs, but passing to the fingers and hands. The anaesthesia is greatest to pain, but touch and temperature sense are also involved. There is often de- layed conduction and polyaesthesia; and many other curious perversions of the cutaneous sense are noted. Some anaesthesia usually develops over the finger tips and hands, and sometimes a band of anaesthesia devel- ops about the trunk (Fig. 119). This trunk anaesthesia may be one of the early symptoms of the disease. The facial and cranial nerves are not much affected, but there may be trigeminal neuralgia. The olfactory Fig. 117. — Hypotonia in late tabes dorsalis. SCLEROSES, DEGENERATIONS, SYPHILIS 263 nerve is occasionally affected. In early stages of the disease when there is perhaps some specific disorder of the mucous membrane, parosmia occurs, the patient being annoyed with the sense of a bad smell. Later in tabes this sense may be lost. Optic atrophy occurs in about 6 per cent, of cases in my experience. Optic atrophy usually develops in the preataxic stage, and if a patient has reached the second stage without it, he will probably escape it altogether. Cases with ocular paralyses are slightly more disposed to it (Berger). It attacks the left eye oftener than the right. The atrophy begins sometimes with increased sensibihty to hght, flashes of hght, and muscai volitantes. Fig. 118. — Hypotonia in locomotor ataxia. With the failing vision, disturbance of color sense often and contraction of the visual field always occur. This contraction is irregular, with sector-formed defects; not hemiopic. The atrophy progresses slowly with slight remissions. It may cease its progress, but this is rare and blindness comes in about three years. Ophthalmoscopically, there may be seen slight evidence of congestion in the early stage ; later, pallor of the discs, which finally become grayish. The eye muscles are implicated in some way in nearly all cases of tabes. The fohowing are the disorders: (1) Loss of the hght reflex and myosis; (2) sympathetic-nerve ptosis; (3) paralysis of the external eye muscles. 264 DISEASES OF THE NERVOUS SYSTEM Paralyses of the ocular muscles (third, fourth and sixth) occur rather oftener in cases with evidence of exudative (meningeal) syphilis. Ocular palsies are early symptoms of the disease, occurring, as a rule, in the preataxic stage. 1. Loss of light reflex and pupillary rigidity. The pupils are small and sometimes uneven and irregular in shape; they respond to accom- modation, but not to light. In early stages the light reflex may be simply sluggish. In the late stages the pupils are often dilated. The Fig. 119. — Trunk and leg anaesthesia in early stage of tabes dorsalis. phenomenon is perhaps due to lesion of the ciliary ganglia. More probably it is due to degeneration of the mesial nucleus of the third nerve, or its connections. The Argyll-Robertson pupil is practically found only in tabes and in general paresis. It may be brought about by local disease, by a polioencephalitic or ganglionic infection, or it may be congenital. The ocular skin reflex usually disappears early. The myosis in tabes is due to paralysis of the sympathetic dilating fibres. 2. Sympathetic nerve ptosis. A slight drooping of one or both lids is not infrequent. It begins early and progresses slightly up to the later stages of the disease. It is due to paralysis of the cervical sympathetic- SCLEROSES, DEGENERATIONS, SYPHILIS 265 nerve fibres which go to the unstriatecl fibres of the levator palpebrae (tarsalis superior). 3. Paralysis of the external eye muscles. The external rectus is oftenest affected of single muscles, but the various branches of the third nerve taken together are oftener involved than the sixth. Of the third nerve's branches, the levator palpebrae and internal recti muscles are oftenest involved. There may be multiple palsies. These occur oftener with meningeal syphilis. Progressive ophthalmoplegia may be associ- ated with tabes. The ocular nerve palsies may be transitory or perma- nent. Those occurring in the preataxic stage are usually transitory, lasting a few hours, days or weeks. Cases have even lasted two years and got well. The permanent palsies develop usually in the later stages. The early palsies are usually due to a syphilitic exudation at the base of the brain; the late palsies are usually due to degenerative lesions of the nuclei of the ocular nerves. Violent neuralgic pains sometimes occur in the face, but it is rare; in fact, a characteristic of tabetic pains is that they hardly ever involve the trigeminus. However, it does occur that a typical tic douloureux may develop. Paralysis of the seventh nerve, though not infrequent in cerebral syphilis, is not a symptom in tabes. It is not even a premonitory symptom as are the ocular palsies. Spasmodic movements of the lips and face with smacking movements similar to those seen in some forms of spasmodic tic have been noted in two of my patients. The acoustic nerve is frequently affected, some form or degree of deaf- ness occurring in about one-fourth of the cases; but in the majority of instances the trouble is an accidental complication due to middle-ear disease. Primary atrophy of the auditory nerve is very rare, as might be expected, since this nerve is structurally not like the optic nerve. Its existence has been inferred on clinical grounds. There is a form of tabetic deafness of trophic origin due to a sclerotic condition of the middle ear (Treitel). The labyrinthine nerve is affected but rarely. Vertigo is not common, yet labyrinthine crises causing seizures like those of Meniere's disease have been observed. Dyscesthesia of the throat with attacks of repeated swallowing move- ments form what Oppenheim calls pharyngeal crises. Bulbar jparalysis as well as progressive muscular atrophy are rare association diseases and generally appear late in the course. More common are disorders of innervation of the larynx. These con- sist of laryngeal crises and paralyses of the laryngeal muscles. The characteristic paralysis of tabes is a ''posticus palsy;" i.e., a paralysis of the posterior crico-thyroid muscles. These are abductors, and their paralysis causes difficulty in breathing if both are affected and sometimes 266 DISEASES OF THE NERVOUS SYSTEM when one is affected. The paralysis is usually unilateral. The laryngeal palsies are usually early symptoms and usually are not serious. I have known, however, sudden death to occur in a patient subject to attacks of this type of palsy. Anaesthesia of the throat and larynx is very rare. In a laryngeal crisis there is spasm of the adductors or paralysis of abductors, with noisy, croupy respiration. The attacks come on sud- denly, the patient coughs and struggles for breath, and he may be seized with vertigo and fall down. The pulse may be very fast. The paroxysm lasts for a few minutes to several hours. The symptoms are very distressing, but not usually dangerous. Paroxysms of cough have been described as "bronchial crises." There are also cardiac crises, in which there are dyspnoea and rapid heart beat and sense of suffocation resembling angina. A much more frequent symptom is a rapid pulse which runs about one hundred and is small and weak. The heart is sometimes diseased, either from neurotrophic disturb- ance or luetic degeneration. The pulse is often small, rather rapid, and weak. The most common and distressing of the crises of tabes are the gastric. These sometimes occur in the preataxic stage and recur for a long time before the nature of the trouble is recognized. They consist of sudden attacks of intense pain extending from the groin to the epigastrium or encircling the waist, accompanied by vomiting and sometimes diarrhoea. The attacks are often associated with pains in the legs. They last two or three days, then usually pass away, but some cases last two or three weeks and leave the patient much exhausted and often more ataxic. Sometimes instead of or between the crises the patient has a continual diffuse, dull, abdominal pain. The arthropathies of- locomotor ataxia. Degenerative diseases of the joints, technically known as arthropathies, and spontaneous fractures of bones form important symptoms of tabes. They occur in 10 per cent. (Charcot) or 5 per cent, (author) of cases. The arthropathies are three or four times more frequent than the fractures. The joints oftenest affected are the knees, ankles and hips; but the elbow, shoulder, wrist, vertebral and pelvic bones and small joints may be attacked. Spontaneous fractures occur oftenest in the shaft and neck of the femur, next in the legs, forearm, humerus, and clavicle. The pelvis, scapula, vertebrae and under jaw may be fractured. Arthropathies are often accompanied by fractures, especially of the heads of the bones. The two sides of the body are about equally affected. The arthropathies are characterized by a sudden, apparently spon- taneous painless swelling of the joint. The symptoms may develop in SCLEROSES, DEGENERATIONS, SYPHILIS 267 twenty-four or forty-eight hours. In rare cases there is a history of some preceding rheumatic pains or of an injury. After a time there is an osseous hyperplasia of the joint, which becomes enlarged to enormous proportions. There is also a tendency to luxation of the joint. It crepi- tates on moving. There is no tenderness on pressure; the hand finds evidence of synovial exudation, roughened surfaces, and perhaps frac- tures of the enlarged parts. In the milder forms there are simply swell- ing from synovial exudation and some enlargement of the bones with roughened surfaces. After a few weeks this swelling may subside and the joint return to nearly its nat- ural size. In other cases the process progresses, the ligaments relax, the bones of the joint can be moved about freely, and luxations are easily produced. There is still no pain, but the limb becomes almost or entirely useless on ac- count of the loose and relaxed condition of the parts (Fig. 121). As time goes on, some absorption takes place and the head of the bone may almost disappear. The arthropathies have been divided into benign and malignant, but no sharp line can be drawn or cer- tain prognosis made in the early stage. The arthropathies appear in the prodromal and early stage of the disease in over half the cases, and are often at first unrec- ognized. One-third occur after the tenth year of the disease. The spontaneous fractures are usually brought on by a slight trauma, such as a fall. Violent muscular movements may produce them. They also are painless, as a rule. The fractures usually heal well, often with abnormal readiness, but occasion- ally there is delay, and often healing is accompanied by great throwing out of callus. Pathologically, the arthropathy is a rarefying osteitis. It does not differ anatomically from arthritis deformans, except that fractures may accompany it. Clinically, the chief difference lies in the abruptness, spontaneity and painlessness of the process. The disease, on the whole, cannot be considered specifically different from arthritis deformans, Fig. 120. — Arthropathy of shoulder. 268 DISEASES OF THE NERVOUS SYSTEM modified by the analgesia of the parts. It is due probably to a degenera- tive change in the nerves supplying the joints and bones. The process may begin in the cartilage, bone or ligaments. Eventually all these parts are involved. There is congestion of the synovial membranes with hydrarthrosis, then atrophy and rarefying hypertrophy of the epiphyses, relaxation of the ligaments, formation of osteophytes and bony stalac- FiG. 121. — Arthropathy of knees. tites. There may be a rarefying osteitis of the long bones, without much joint involvement at first. Various trophic disturbances of the skin may appear, generally late in the disease. The most common are herpes and lichen. Besides these, bullae, transitory erythema, urticaria, eczema, pemphigus, ecthyma, ulcers, ichthyosis and petechias have been described; but they are rare SCLEROSES, DEGENERATIONS, SYPHILIS 269 and often only accidental complications. A peculiar round perforating ulcer sometimes develops on the sole of the foot, often as the result of cutting a corn. In rare cases the nails and teeth fall out. In frankly syphilitic cases there is usually baldness. The skin of the tabetic patient loses its "tone" due probably to the general muscular atony and to weak- ness of the erectores pilarum. At any rate, the hand has a character- istic flabby feeling which is almost diagnostic of the disease. The face and cutaneous tissue generally show the same characteristic. A sense of great weariness and heaviness in the limbs, present con- stantly, no matter how much rest is taken, is a characteristic early symptom, and is due to an irritability of the nerves of muscular sensibility. Muscular atrophies occur sometimes in tabes. They are of three kinds: (1) a pro- gressive muscular atrophy due to local end- arteritis and resulting degeneration of trophic and motor cells (chronic poliomyelitis); (2) localized muscular atrophies due to degenera- tive atrophy of nerves; (3) a general wast- ing. Under the first head one finds ophthal- moplegia, bulbar paralysis and spinal myop- athies; under the second, wasting of certain groups of muscles in the legs or arms. Besides these, there is a generalized atro- phy which occurs in the paralytic stage and is due probably to a slight involvement of the anterior horns in the progressive process that affects the cord. Attacks of hemiplegia in rare instances occur in tabes. They are usually of tempo- rary character and occur early in the disease. They may come on late and are then more likely due to acute softening due to disease of the cerebral vessels of syphilitic origin. Acute paraplegia comes on occasionally also, and this sometimes almost disappears. The sexual power may be at first greatly exaggerated; but this is rare, and usually there is progressive weakness and loss of desire. The bladder and sexual functions are rarely entirely lost and rarely equally impaired in the first stage; one may continue good while the other is affected moder- ately. Usually the sexual function goes first. Some cerebral symptoms occur in tabes, chiefly in the early stage. They are insomnia, which may be very obstinate, and occasional vertigo. An irritability of temper and tendency to despondency, sometimes noted, Fig. 122. — Perforating of foot. ulcer 270 DISEASES OF THE NEEVOUS SYSTEM cannot be considered unnatural. Apoplectiform and epileptiform attacks are described, but are very rare, and should cause a suspicion of a com- plication. The disease in very rare cases terminates in general paresis. Usually if there is to be paresis it comes on with the tabes. When the disease developes frankly and typically as locomotor ataxia, paresis rarely supervenes. Course,— The disease has been termed progressive, but it is not so in a large number of cases. With proper treatment the symptoms can often be kept in control for years. The first stage may last twenty years or more; the second stage five to fifteen years. The total duration of the disease varies enormously, ranging between three and thirty years. A few acute cases have been observed, running a course of less than a year. Complications. — These are acute myelitis, generally syphilitic; lateral sclerosis, progressive muscular atrophy, hemiplegia from embolism or endarteritis, general paresis and heart disease. Tabo -paresis. — In a rather increasing number of cases one sees the symptoms of paresis and tabes develop together. In these cases the cerebral symptoms are usually more dominant and are always more seri- ous and important. These symptoms do not differ greatly from the ordi- nary early symptoms of paresis. The tabetic symptoms are often of less note and consist in loss of knee-jerks, some ataxia, lancinating pains, ocular palsies and vesical and sexual weakness. Taking a group of 100 cases of tabes I find about 10 per cent, are tabo-paretic. In these cases it is the paresis that has to be more carefully watched and dealt with. Like tabes, it can be arrested, but not so often as in the pure type. Pathological Anatomy and Pathology. — The characteristic changes in an advanced case are found in the spinal cord, posterior spinal ganglia and posterior roots, and to a less extent in the peripheral nerves. The spinal cord usually is reduced in size and flattened antero-posteriorly; the pia mater is thickened somewhat. One can see with the naked eye that the posterior columns of the cord are shrunken and have a grayish appearance. In early cases one sees that the part of the posterior column first affected is a vertical streak lying in the middle root zone between the posterior median (columns of GoU) and posterior external columns (columns of Burdach) (Fig. 123). The segments first and most affected are those of the upper lumbar and lower dorsal region. Besides this area the rim zone or column of Lissauer is also early involved. As the disease progresses it extends upward and spreads laterally so that finally all of the posterior column is changed into a dense connective-tissue mass through which only a few nerve-fibres run. The part last and least involved is that lying just posterior to the commissure (anterior root zone of Flechsig, ventral fundamental column) and that lying just SCLEROSES, DEGENERATIONS, SYPHILIS 271 Fig. 123. Fig. 124. Fig. 12.3. — ^Locomotor ataxia, showing areas affected in first stage at five different levels. Drawn from specimens in author's possession and from comparative study of over thirty other figures. Fig. 124. — ^Locomotor ataxia, second stage. 272 DISEASES OF THE NERVOUS SYSTEM mesial of the posterior horns (external part of the middle root zone) (Fig. 125). There is sometimes a degeneration of the antero-lateral ascending tract (Gowers' tract), and very rarely of the cerebellar tract. The pyramidal tracts are involved only in complicated cases. The cells and fibres of the column of Clark are often involved in advanced cases. The gray matter of both the posterior and anterior horns may show some degenerative changes, viz., decrease of the fibre network and atrophy of the cellular elements. The posterior roots are usually involved, the process extending as far as the spinal ganglia, which also show some degeneration, but the lesion is not strikingly marked here in all cases, and sometimes the spinal ganglia are nearly healthy, although the posterior columns are diseased. The anterior roots are fairly normal. The exact initial point of attack varies, and this accounts for the va- riation in the symptoms. Cases that begin with decided bladder and geni- tal symptoms probably start low down; cases which go for a good while with only ataxia, loss of knee-jerk, and pains begin higher; while in the brachial or arm-type cases the process begins in the cervical enlargement. The peripheral nerves are diseased in a large number of the ad- vanced cases. The nerves of the leg are most involved. The process is a degenerative atrophy or neuritis. It affects the extremities of the nerves first and slowly extends upward, seldom reaching the large trunks. Sometimes the disease begins in the optic nerves, but not in the bipo- lar visual cells of the retina which correspond to the posterior ganglion cells. The process here is an atrophy beginning at the periphery and extending brainward. The third, fifth and sixth nerves are occasionally involved; still more rarely the olfactory and' auditory. The vagus nerve and sometimes its nucleus and that of the glossopharyngeal are implicated, it may be, rather early in the disease. It is believed that these facts explain many of the laryngeal and visceral crises and the tachycardia. Pathology. — Syphilis often invades the nerve-centres before true tabes sets in and frankly shows itself in the form of inflammatory, meningeal, vascular and gummatous exudates, but often it does not betray its presence in this way but does its fatal work quietly through years of apparent health. All the time its poison is at work instituting a tendency to death and degeneration in certain parts of the nerve-centres. The parts which are usually first selected are the posterior spinal roots and the neuraxons which pass from the nerve-cells of the ganglia into the posterior roots and columns of the spinal cord. Locomotor ataxia, there- fore, is not primarily a disease of the posterior spinal columns, but of the peripheral sensory neurons. It is true that the posterior spinal ganglia are not always so seriously diseased as the posterior columns of SCLEROSES, DEGENERATIONS, SYPHILIS 273 the cord. This, however, is because the severity of the disease is first shown in the central neuraxons of the gangha and their collaterals, just as in alcohoHc neuritis the peripheral parts of the motor neuraxons are most and earhest affected by alcohol. The spirochceta toxin is carried in the peri-radicular spaces and starts often a radiculitis or root Fig. 125. — Tabes dorsalis, advanced stage; cervical dorsal and lumbar levels. degeneration, which extends into the cord. As the disease extends, it involves both the peripheral and central parts of these sensory neurons; that is to say, both the sensory fibres and the posterior spinal roots. Still later it attacks other portions of the nerve-centres. The syphilitic microbe and its toxins are brought to the nerve-centres in the blood, 18 274 DISEASES OF THE NERVOUS SYSTEM whence may pass into the lymphatic sheaths of the blood-vessels or are thrown out upon the serous membranes in the subdural sacs. In the circulation of the cerebrospinal fluid the toxins are carried along the serous sheaths which surround the cerebrospinal nerve-roots. The nerves, as they pass out from the spinal cord, are covered with three membranes; the dura mater, the arachnoid and the pia mater. The dura and the arachnoid surround them less tightly than the other. The dura becomes fused with the connective tissue supporting the nerves as they pass out, and the arachnoid becomes fused with the epineurium and perineurium. Now, fluid injected into either the subdural or sub- arachnoid spaces passes readily along the nerves for some distance (Mace- wen), and syphilitic toxins in the subarachnoid and subdural spaces of the spinal cord will thus have a tendency to infiltrate along these sheaths, but as it passes out of the vertebral canal or cranial cavity it meets mechan- ical obstacles, owing to the constriction of the parts, and there is therefore a certain damming up or accumulation of the poisonous material at these points of exit. Generally at this point it meets with the posterior spinal ganglion a highly organized structure with special vascular supply, and it therefore naturally deposits its poison upon this part, which furnishes much more opportunity for mischief than the non-vascular anterior roots. It is a fact that many of the initial symptoms of locomotor ataxia are thus connected with such troubles as would occur from an exudate trying to get out along the course of the spinal or cranial nerves. A frequent initial symptom, for example, is palsy of one of the third nerves or of the sixth or seventh nerves, due to exudates clinging around their roots. Still more frequently the initial symptom is a neuralgic pain in the course of the sciatic plexus, due to the effects of this poison upon the ganglia. It has been established that the syphilitic infection is due to the spirochceta pallida. These organisms have been found in tertiary forms of syphilis, and it has been shown that the spirochceta is alive and acting upon the nervous system, even in degenerative or parasyphilitic stage. Thus the degenerations of tabes are really only a later form of a constantly acting- infection and all the phenomena, both of syphilis proper and so-called parasyphilis are only different stages of the same pathological process. I assume in this description that it is a syphilitic poison which is al- ways at work as a cause of tabes dorsalis. This, however, may not neces- sarily be the case, for it may be the infection of other diseases; and the results of other poisons, such as ergot, pellagra, etc., may be explained in the same way. There is an inadequacy of the lymphatic and venous systems thoroughly to rid the spinal canal of the poisons that lie in it ; the body of the cord is cleared, but the roots do not get rid of the poison. As to why this process attacks some persons and not others, it can only SCLEROSES, DEGENERATIONS, SYPHILIS 2/0 be said that certain people are born with defective power of resistance as regards their nerve-centres, and that others induce this defective state by physical and other excesses. Diagnosis. — In the first stage the disease has to be distinguished from hereditary ataxia, multiple neuritis, chronic myelitis, spinal tumor, spinal syphilis, general paresis and neurasthenia. In hereditary ataxia the age, the history of the disease, and the absence of lightning pains are usually sufficient to distinguish it. Multiple neuritis, in its sensory or pseudo- tabetic form, sometimes resembles closely locomotor ataxia. The differ- ential points are given in the sections devoted to that disease. In myelitis there is more paralysis, generally exaggeration of reflexes, and an absence of disturbance of special senses. The serological formula in tabes varies, but it is about as follows : Ser. Wassermann — or -f- Cer. spinal fluid Wassermann + or — Globulin -f- Cells 30 to 50 This formula is changed to negative by active and thorough treat- ment. It becomes negative in very advanced periods of the disease. (See Paresis.) The diagnostic criteria of locomotor ataxia in all cases are the pres- ence of lightning pains, numbness of the feet, loss of knee-jerk, hypotonia, arthropathies, ataxia of station and gait, without much loss of muscular power, the presence of the Argyll-Robertson pupil, the history of syphilis and the slow onset of the disease. These criteiia apply especially for the early stages of the progressing disease. A lost knee-jerk, lightning pains, a positive serological formula and stiff pupils are usually quite enough to assure a diagnosis. Prognosis. — -In the first stage a large percentage may have the disease stopped and get practically well; and tabes dorsalis, if seen early and prop- erly treated, is no longer a progressive disease. In the second stage a cure is impossible, but great improvement may be secured and the patient made relatively comfortable for years. In the third stage little can be done except to relieve the symptoms, but life may be prolonged. In some cases the careful application of mechanical therapy (the Frenkel exercises) will enable patients to walk to some extent again. The malady is of course better controlled if the patient can afford to lead a regular and quiet life. This is one of the diseases in which the poor man has not as good a chance, because hos- pitals will not keep him long enough. Death usually occurs from some intercurrent malady or from kidney 276 DISEASES OF THE NERVOUS SYSTEM disease caused by the bladder trouble. Patients very rarely indeed die from the disease itself and its various ''crises." Treatment. — ^The treatment of locomotor ataxia is a subject the discussion of which cannot be made dogmatic, for the treatment depends very largely upon the patient and the stage and type of the disease. My experience is that any treatment depends enormously upon one's opportunities of getting the patient in the earliest stages. Supposing this be done, the first thing is to be quite assured that there is no trace of syphilis in the system as shown by clinical signs and by tests of the blood and cerebrospinal fluid. It is the fashion now to begin treatment by giving intra-venous or intra-spinous injections of salvarsan. Brilliant results have been secured and perhaps better or more uniform results than from the use of mercury and iodide. Injections of neo-salvarsan 0.9 may be given twice weekly until ten doses have been given. In the intervening days inunctions or hypodermics of mercury should be given in average doses. After this, hypodermics of salicylate of mercury, gr. i. may be given twice a week for six weeks. Then the patient should be given tonics and general hygienic measures. If at the end of two months, the serological formula is not markedly improved another course of injections is given. If the patient is much better or if the formula has become negative, the salvarsan need not be given oftener than twice a year. The serological formula may become normal under ordinary hygienic conditions especially if the patient lives in the open air. This is a clinical fact which should be borne in mind. If the disease is accompanied by evidences of meningeal (exudative) syphilis, the treatments may need to be doubled in intensity. It is sometimes stated that the iodides are not needed, or do no good in tabes, but this is not the case. After salvarsan and mercurial treat- ment patients sometimes improve further under the iodide of sodium in moderate or large doses. Indeed forty years ago nearly as much was claimed by good observers for massive doses of iodide as is now claimed for salvarsan. Along with these first medicinal measures, the physician should pre- scribe something which is also most important, and that is simply rest. Every patient with locomotor ataxia should at once have the importance of rest strongly impressed upon him, and the prescription of sixteen weeks a large part of the time in bed is sometimes advisable. Equally good results can be usually obtained, however, by obliging the patient to go through a simple and regular life, involving moderate walking and no hard physical work. Institutional life for three months is of enormous advantage. It is a rule to which I have seen hardly an exception that tabetic patients brought to the hospital improve in a striking way simply SCLEROSES, DEGENERATIONS, SYPHILIS 277 from the quiet routine of life there, and despite the thinness of city milk and the uncertain value of hospital eggs. The drugs which are used as tonics are mainly the nitrate of strychnia, iron, the preparations of arsenic and phosphorus. The various prepara- tions of the phosphates, such as glycero-phosphate of lime, the hypophos- phite of lime, phosphoric acid, seem to be of some benefit. Strychnine occasionally does good in small doses, but unless very carefully increased large doses may lead to disastrous results and it should always be given with caution. A great many other drugs may be given for the relief of symptoms. Thus ergot, belladonna, and urotropin sometimes help the bladder. The fluid extract of buchu in doses of twenty drops, combined with ten drops of the tincture of hyoscyamus, is also useful. For the pain, phenacetin is a drug which gives satisfaction. It may be com- bined with bicarbonate of sodium, with codein; or some of the other coal-tar products, such as antipyrin, antifebrin, pyramidon, maj'- be substituted. A teaspoonful of baking soda internally will sometimes stop the pains. Small doses of strychnine may be combined with this. Small amounts of strychnine can always be given for the sexual weakness, but the dose should rarely be made a large one. For the gastric crises, nothing is so good as a hypodermatic injection of mor- phine, and for the severe crises of pain an occasional hypodermic of morphine should be given. The locomotor ataxic, however, who becomes addicted to the use of morphine for his pains is indeed in a hopeless con- dition. In persistent constipation the diet should be light and mainly of vegetables and of liquids, such as milk, broths, etc. In persistent diarrhceal states I have found ichthyol of use. This drug also relieves the pains. The annoying insomnia is to be treated by fresh air, lukewarm baths and sea-shore life. If drugs must be used, bromide of lithium with a few grains of chloral, paraldehyde in not over thirty-drop doses or the veronal group are the best. In neuralgia of the rectum and bladder, suppositories containing iodo- form and belladonna, or codein, or antipyrin, may be used. Sometimes simple gelatin or gluten suppositories act very well. Some of the cases of rectal neuralgia or hypersesthesia are due to insufficient clearing of the lower bowel when a movement occurs, and if the patient washes out the bowel with a pint of warm water after each movement he is very much more comfortable. There is no diet which has a specific effect upon locomotor ataxia, but the patient should be given those foods which are non-fermentative and digestible. Hydrotherapy is of considerable benefit. The most efficient of the single measures is the lukewarm bath at a temperature of about 95°F. 278 DISEASES OF THE NERVOUS SYSTEM for ten or twenty minutes daily. After the bath it is well to have a little cold water poured over the back and then the patient should be diligently rubbed. In most cases a simple lukewarm bath is quite as effective as anything. In others the patient feels better if there is added to it some slight stimulant to the skin, e.g., two tablespoonfuls of pine- needle extract; or a regular pine-needle bath or Nauheim bath may be given. The Charcot douche given in moderate strength is helpful in cases that are not advanced or particularly weak. I have some hesita- tion in recommending any special watering-places or cures. I have had patients return benefited from the Hot Springs of Virginia and other American resorts. In Europe, the baths at Lamalou, France, and at Nauheim, Germany, have some reputation. Hot baths are sometimes injurious, and bathing may be overdone by the ataxic. Electricity is of use from its general tonic and reflex effects, and it perhaps exercises some direct influence on the diseased process. Strong galvanic currents (15 to 30 ma.) should be applied along the spine, through the trunk, and down the legs and arms. The combined galvanic and faradic current is even better, given in the same way. The faradic brush may be applied over the anaesthetic extremities and along the back. The actual cautery is occasionally efficient in stopping pains. It should be applied to the back as often as twice a month at least and some- times twice a week. Large dry cups may be applied rapidly and in great number (80 to 100) along the spine and along the course of the sciatic nerves. They make the patient feel better and relieve sometimes the sense of exhaustion. In very painful cases occasional wet cups and leeches are useful. I still find that suspension by the neck and arms is helpful in some cases. It is best adapted to persons in the second stage and to those who have a good deal of bladder trouble and pain. It is of little value in the paralytic stage, and must be used with care in the early stage and when patients are large and heavy. Suspensions should be given for from one to three minutes three times a week until twenty-five or thirty are taken. After three months a second course may be given. The treatment of locomotor ataxia by systematic exercises, known as the Frenkel method, is one that has been effectively used. It con- sists in having the patient go through regular exercises which teach him to co-ordinate the different groups of muscles of the trunk, legs and arms. The Frenkel method is one which can be given with advantage to persons who are passing into the second stage of tabes and in whom the disease is not making progress. It is especially indicated where there is not great hypotonia. It must be used very carefully and for a long time. It often enables the patient to walk better and use his arms better, but it does not especially affect the progress of the disease. SCLEROSES, DEGENERATIONS, SYPHILIS 279 Finally, it has seemed to me that those sufferers from locomotor ataxia do best who persistently and courageously fight against their malady. Those who, despite suffering and discomfort, will three or four times a year take medicinal treatment, and some hydro-therapeutic or electric measures such as will have beneficial effect upon their general nutrition and such as will buoy up their hopes and improve their mental condition, are quite sure to be rewarded. After a hard fight they will emerge into a state of comparative relief from their symptoms and secure a measurable degree of rest from the progress of their disease. LATERAL SCLEROSIS {Spastic Sjyinal Paralysis) Adult Form. — Spastic spinal paralysis or spastic paraplegia is a malady due to disease of the lateral columns and especially the pyramidal Fig. 126. — ^Lateral scleroses witti spastic paraplegia showing degeneration of pyramidal tracts. tracts of the spinal cord. As these tracts are diseased and injured in many ways and by many causes, spastic spinal paralysis is a very com- mon condition. It is symptomatic of transverse myeUtis, of multiple sclerosis and of tumors and other pathological conditions which cause pressure on the cord. It occurs in the cerebral diplegias. There is, however, one condition in which in adults the lateral columns and especially the pyramidal tracts undergo primary degeneration, and thus we speak of primary lateral sclerosis, causing a form of spastic paraplegia which is an individual disease (Fig. 126). It is a very rare condition; its existence was long doubted, but it has now been established. 280 DISEASES OF THE NERVOUS SYSTEM It is a chronic malady characterized by progressive spasticity and weak- ness of the legs and later of the arms, by increase of the reflexes, the de- velopment of contractures and by the absence of sensory disturbances and sphincter troubles. The disease develops between the ages of twenty and forty. The causes are unknown, except that there is one type of the disease which is of hereditary origin. Syphilis, acute infec- tions and toxic substances are sometimes associated with its develop- ment. Poisoning by certain kinds of lathyrus cause symptoms of the disease. In the hereditary or familial form the disease affects different mem- bers of many succeeding generations in the family. In the cases so far described it begins early in life, affects only or mainly the legs and runs a very slow course unaccompanied by pain, ataxia or visceral symptoms. Both the acquired and the familial forms may last for twenty or thirty years. The special symptoms of the disease are the same as those which occur in involvement of the pyramidal tracts from other causes, and are described under the head of myelitis. In the diagnosis of the disease one has to exclude pathologically the spinal form of multiple sclerosis, chronic transverse myelitis, compression paraplegia from vertebral disease and from tumors. The disease is so rare that particular pains must be taken to rule out these more common disorders. The Congenital Form (Little's Disease).- — Etiology .—The term spastic spinal paralysis, is also applied to an affection which is al- ways of congenital or infantile origin and is due, it is supposed, to a lack of development of the pyramidal tracts. This lack of develop- ment leads to a sclerosis of the lateral columns of the spinal cord and to symptoms of rigidity of the legs and arms, exaggeration of the re- flexes, with some real muscular weakness and atrophy. The disease is of prenatal, natal or infantile origin, being due to some developmental defect or, as Little supposed, to premature and forced deliveries, with probably some meningeal hemorrhage on the convexity of the brain. Through these causes the pyramidal tracts cease to grow, or, at least, this process is greatly delayed. In many cases there is a premature birth and difficult labor. Symptoms. — The malady is noticed within a short time after birth, usually within a year, but it may be delayed in family types to the fifth year or even later. Some cases of Little's disease may, it is believed, develop as late as after maturity. It is not my purpose to give a descrip- tion in detail of the symptoms of this trouble, because they are given under the head of cerebral diplegia or birth palsy. The only difference between ordinary cerebral diplegia and the disease under present con- SCELROSES, DEGENERATIONS, SYPHILIS 281 sideration is that in this latter form there are no marked mental defects; the child is not small headed and idiotic, nor does it have epilepsy or cra- nial nerve palsy or hydrocephalus. The brain seems to be spared except so far as its motor functions are concerned. It is convenient to separate this type of disease from the ordinary spastic cerebral palsies with mental defect, for the reason that the future of these cases is in some instances more hopeful. As they mature, the lateral columns occasionally gain in development and some increase in the strength and control of the limbs is ob- tained. I base this statement upon the experience of others. In several cases of Little's disease at the age of fifteen to twenty-five which I have seen, there has been no marked improvement. Mentally, how- ever, these patients are often very bright. ^ Children with this trouble on trying to walk are obliged to cross one leg in front of the other as they are helped along, giving them a characteristic ''cross-legged" pro- gression. The arms are less affected than the legs. The facial and throat muscles may be slightly involved. There is no pain. In some cases the disability increases as the child grows older, owing to the greater size and clumsiness of the patient. The arms become much stiffened and con- tractured, and the hands are flexed so that the patient can neither walk nor help him- self. Epilepsy and mental deterioration also may develop at the time of puberty or adolescence but they do not belong to the typical form. Prognosis. — ^The mild cases that learn how to walk and can use the arms and hands may grow up, slowly improving, and reach a good age and a fair degree of health. The severer cases rarely reach adolescence, but grow gradually more helpless and generally succumb to some intercurrent disease before they are twenty. Diagnosis. — -The disease is distinguished from ordinary cerebral diplegia (birth palsy) due to brain lesion by the absence of epilepsy, men- tal defects and microcephalus. (See Cerebral Diplegia.) Fig. 127. — Little's disease showing adduction of thighs and cross-legged progression. 1 It is due to American neurology to say that Dr. Seguin as long ago as 1879 said: "It is possible that tetanoid paraplegia in young children may be due to deficient cerebral development and consequently agenesis of certain tracts of the cord." 282 DISEASES OF THE NERVOUS SYSTEM From compression myelitis, the involvement of the arms and the absence of pain and disturbance of sphincters are distinctive. Hereditary- spastic paraplegia runs in families, begins at the fourth or fifth year, and involves chiefly the legs. Treatment. — This is altogether one of mechanics and attention to nutrition. The limbs must be persistently massed; tenotomies should be performed so as to straighten the legs; constant voluntary effort to use the stiffened muscles should be made. Resection of certain of the pos- terior roots has given some favorable results. Braces, roller crutches, etc., should be used. Patience is often greatly rewarded in this disease. THE COMBINED SCLEROSES By the combined scleroses is meant those forms of degenerative sclero- sis in which both the posterior and lateral columns are involved. There are several diseases in which combined sclerosis exists. They are: 1. Combined scleroses of profoundly anaemic and toxic states (Put- nam's type). 2. Hereditary spinal ataxia (Friedreich's ataxia). 3. Combined scleroses complicating general paresis. 4. Tabetic form. There are many cases reported in literature of combined scleroses, but the clinical pictures vary very greatly. These cases are probably in the most part forms of chronic myelitis or multiple sclerosis with ascending and descending degeneration. Marie has shown that the vascular supply of the spinal cord is such as rather to favor the development by extension of sclerosis in the lateral and posterior columns from a chronic leptomen- ingitis, and his suggestion that many of these cases are perhaps of syphil- itic origin accords with my experience and conviction. Some years ago, Cowers described a disease that he called ataxic paraplegia, the lesion in which, he believed, lay in the lateral and posterior columns. Most of the cases which belong to this clinical description I think can be properly classed either with the cases of locomotor ataxia, of multiple sclerosis or of some form of chronic myelitis. THE COMBINED SCLEROSES OF PERNICIOUS ,AN^MIA AND CACHECTIC STATES (Putnam^s Type) This form of disease was first described by Dr. J. J. Putnam, later by myself. It is certainly rather common in this country, being almost as frequent as multiple sclerosis. SCLEROSES, DEGENERATIONS, SYPHILIS 283 Etiology. — The disease occurs oftener in men. Eleven out of my twenty-three cases were in women. The age of most cases is between 50 and 65 (16 out of 23), and next to this between 40 and 50 (5). The youngest case was 36 (Rothmann's). There is often some family or personal neuropathic history. I have reported three cases occurring in one family. The disease follows acute infections like influenza, prolonged diar- rhoeal or dysenteric attacks, and serious dietetic errors. One patient had a malignant tumor. The most important cause is that associated with severe or pernicious anaemia, which in turn is attributed to gastro-intestinal disturbance and consequent toxaemia. Probably some intestinal micro-organism is behind the whole. Syphilis is not a cause. Often, if not always, how- ever, nervous symptoms develop before evidences of extreme or extended anaemia are present. Symptoms. — The initial nervous symptom is nearly always a persist- ent paraesthesia of the feet and hands associated with some weakness of the part. The paraesthesia is of the stocking or glovetype and gradually ascends the legs and arms. It is rather sharply limited. The tip of the tongue and nose may be affected. Following this there is a good deal of ataxia, with some loss of deep sensibility, and this symptom increases with the loss of motor power. Tactile, thermic and pain anaes- thesias are not marked until late in the disease, but the patient often has severe pains in the back and limbs and often complains bitterly of his parasthesiae. There may be some differentiation of sensibility as in syringomyelia. With the weakness of the legs there is at first an increase of knee-jerks, and sometimes ankle clonus and rigidity, so that the patient shows the symptoms of ataxic paraplegia. Later the spasticity may become less and the knee-jerks disappear. When the arms are involved, paraesthesia, weakness, awkwardness and a very slight anaesthesia develop and progress. The mind may be slightly weakened, the memory becoming defective and the patient emotional. The cranial nerves are not often involved, though optic atrophy has been once noted. The blood presents evidences of anaemia. Usually this is secondary, but in about 40 per cent, of cases there are the typical findings of pernicious anaemia. It should be noted that a large proportion of cases of pernicious anaemia have slight involvement of the spinal cord and show some acro-parsesthesia and motor weakness, but do not develop the disease. The disease progresses rather rapidly, though hopeful remissions of six to twelvemonths occur. In from six months to a year, if there is no re- mission, the process has nearly reached its height. During this time the patient emaciates, marked anaemia or pernicious anaemia sets in. The skin is sallow and pale, and pigmentation is seen. Diarrhoea occurs at times. 284 DISEASES OF THE NEKVOUS SYSTEM The bladder becomes weak and retention of urine follows; the control of the rectum is eventually lost, and the patient lies bed-ridden with paraly- sis and contractures of the lower limbs. The arms do not become so badly paralyzed. Prognosis. — Death usually occurs in about two years in the progress- ive cases. It may be prolonged to three years, and I have found that if the trouble is recognized early the process may be checked. The progno- sis is better in older patients. Pathology. — ^The disease is a toxic one, but whether due to the failure of some glandular tissue of the body to act or to infection or poison is not known. As the disease belongs to the degenerative period of life, it may fairly be supposed that in some cases the blood-making organs undergo premature decay and that the nerve centres are poisoned in consequence; Fig. 128. — ^Lesion of posterior column in pernicious anaemia. or the intestinal tract may be the origin of the poison since in the severe anaemias there is great atrophy of the mucous membranes. The process is classed as chronic degenerative myelitis by some writers, but I see no reason for regarding it as a true inflammation. As to the location of the trouble, the posterior columns of the spinal cord are the parts first and most involved (Fig. 128). The process espec- ially involves the postero-median parts. It appears to start in certain foci in the lower dorsal or cervical cord, and to spread thence. New foci apparently develop as the disease progresses; the lateral columns are also severely affected, and especially the crossed pyramidal tracts (Fig. 130). The other columns are involved, but less regularly and completely. The lower dorsal and lower cervical levels of the cord are especially selected. The anterior horns are finally involved, but the change SCLEROSES, DEGENERATIONS, SYPHILIS 285 )J+eili ,.■..'; --y-'- y-' i:h k^ ,1. i 5 I'i : ■ i • , •■, .-. ■ v. I ,' . *•'■■,, ■ ■ *.>■*;. 1,..' »■ ^ ■■ . #.f. 4^ Fig. 165. — Type of cell lamination in the post-central area. {Camyhell.) Fig. 166. — Type of cell lamination in the precentral area {Campbell). In the sixth layer is seen a cell of Betz. Cell Layers. — The cells of the cortex are also arranged in layers more or less definitely marked, and varying in different parts of the cortex. The type of arrangement as given by Campbell is as follows : 1. The plexiform layer containing the cells of Cajal. It is also called the molecular or first layer. 2. The layer of small pyramidal cells; the cells here are closely packed, and while most of the cells are pyramidal, some are polymorphous. This is called also the second ANATOMY AND PHYSIOLOGY OF THE BRAIN 339 layer. The cells are about the size of a white blood-cell; this layer is fused with the third layer. d&pass. tanpM, Fig. 167. — ^Left hemisphere, from without. 3. Layer of medium-sized pyramidal cells. Here the cells are all pyramidal and increase in size from above downward. f ror\A.CLlp^i^ Occtpltcul-Po^- Fig. 168. — The convolutions from above. 4. Layer of large pyramidal cells, lying on a level with the line of Baillarger. 5. Layer of stellate cells, known also as the granule layer or Kornerschicht. This is best represented in the calcarine cortex. 340 DISEASES OF THE NERVOUS SYSTEM 6. Internal layer of large pyramidal cells; this contains the giant cells of Betz, and in the visual cortex the large solitary cells. 7. Layer of spindle cells. Fig. 169. — Mesial surface. Association, commisural and projection fibres. Uncinate fasciculus below; superior longi- tudinal fasciculus above. Fasciculus occipito-frontalis superior Cingulum above; inferior longitudinal fasciculus below. Fig. 170. — Association tracts of the brain. (Villiger.) The small pyramidal and spindle cells measure about 10 X 18iu; the large, 20 X 40m( K200 X^'^oo in.). There are in the precentral convolutions giant cells (of Betz) which measure 125 X 55/^(3-^0 X }ioo in.). ANATOMY AND PHYSIOLOGY OF THE BRAIN 341 The cortical gray matter, as will thus be seen, contains layers of nerve-cells, into which nerve-fibres penetrate. These terminate, as do all fibres, in end brushes, which surround the receptive cells. An enormous number of fine fibres is given off Fig. 171. — Vertical section 'through brain immediately behind the concavity of the corpus callosum. 7, II, III, Frontal convolutions; CC, corpus callosum; V, lateral ventricles; In, island of Reil; S, fissure of Sylvius; CS, corpus striatum. by the cells; some of these form layers in the cortex and connect neighboring parts, others run out and connect more distant parts or pass down to lower levels. There are thus three kinds of fibres — afferent, associative and efferent — just as there are Fig. 172. — Section further back through caudate and lenticular nuclei, showing IC, internal capsule. three types of cells, and since nerve-cells and fibres are really parts of the same unit — the neuron — there are practically three kinds of neurons in the cortex. Association Fibres. — The different convolutions and lobes of the brain are con- nected with each other by association fibres (Fig. 170) and commissural fibres, and 342 DISEASES OF THE NERVOUS SYSTEM Fig. 173. — Section just in front of anterior commissure. Fig. 174. — Showing divisions of lenticular nucleus. OP, Operculum; //, optic nerves. *^r^ ti Fig. 175. — Horizontal section of bram at level of the knee of the corpus callosum, showing fornix. ANATOMY AND PHYSIOLOGY OF THE BRAIN 343 to the ganglionic masses below them by projection fibres. The association fibres consist of short fibres connecting neighboring convolutions and of five long tracts which connect neighboring or distant lobes (^Fig. 167). The Central Ganglia of the Brain. — The relations of the cortex and of the ganglionic masses of the brain and of the different associating tracts is shown in a series of photographs made by Prof. J. C. Dalton over twenty years ago. Nothing so good has been done since his work. Fig. 176. — Section through anterior bend and splenium of corpus callosum. The fornix is nearly removed showing 3d-ventricle. Fig. 177. — Section through midbrain and cerebellum, showing, 2, red nuclei, 3, acqueduct of Sylvius, 4, pons. THE CEREBELLUM In man it consists of a median portion or vermis and two lateral hemispheres. It is connected to the cord and rest of the brain by anterior, middle, and pos- terior peduncles. It is composed of an external layer or cortex of gray matter, of central white matter, and central ganglia. The gray matter lies in very close, nar- 344 DISEASES OF THE NERVOUS SYSTEM row folds, producing with the white matter an appearance on section called the arbor vitoe. The vermis and hemispheres are divided by sulci into a number of lobes or lobules. The vermis is divided into superior and inferior portions. Its further subdivisions and those of the hemispheres are shown in the cuts (Figs. 178?and Central lotule Mesencephalon occupying th« incisura anterior Lobulus culminis Lobulus lunatus Postero- superior lobule Folium % ermis Postero-inferior lobule Tuber vermis Incisura posterior Fig. 178. — Upper surface of cerebellum. {Cunningham.) 179) . In the white matter of both hemispheres is a nucleus of small multipolar cells, the corpus dentatus or ciliary body. To the median side of this, and belonging structurally to it, is a small nucleus, the emboliform nucleus. In the inferior vermis is a collection of larger multipolar cells, the nucleus fastigium or tegmental nucleus; Central lobule Anterior medullary velam Brachuim roniu^nctivuin I / Ala lobuli centralis Brachium pontis Fourth ventricle-__ ,y/n V •^fesg^l^^S-^ ^^ ]]\^^ „ „ , ... ,Tonsillacerebelli ■Horizontal Assure .Postero-inferior lobule Postero-interior lobule Lobulus gracilis Biventral lobule "" "^ 7 Pyramids luber vermis Fig. 179. — Under surface of cerebellum, tonsil on right side removed. {Cu7iningham.) just to the outer side, between it and the emboliform nucleus, is a small collection of cells, resembling those of the n. fastigii, called the nucleus globosus. The general arrangement of the cerebellar cortex is analogous to that of the cere- brum. Associative and receptive cells are found in the granular and molecular layers, and they send processes forming a rich network around Purkinje's cells, which are ANATOMY AND PHYSIOLOGY OF THE BRAIN 345 efferent in function. The comparatively small number of the large cells is in harmony with the view that the cerebellum is an organ that receives and adjusts nerve impulses for co-ordinate distribution. All parts of the cerebellar cortex are anatomically alike. The white matter of the cerebellum consists of nerve-fibres, some of which go to form the peduncles. Others form anterior and posterior commissures, running through the two extremities of the vermis and connecting the hemispheres. There is also a longitudinal commissure in the vermis. The white matter around the corpus dentatum is called the fleece. Fig. 180. — Transverse section through a fold of the cerebellar cortex. P, axon of Purkinje cell; F, moss fibres; KK', fibres from white matter of cerebellar fold ending in connection with dendrites of Purkinje cells; M, small cells of molecular layer; GR, granule cells; M', basket cells; GL, neurogha cell. (Cunningham.) THE GLANDS OF THE BRAIN The pituitary body or hypophysis cerebri is a small oval body lying in the sella turcica or hypophyseal fossa. It is composed of an anterior and a smaller posterior lobe. It is connected to the tuber cinereum above by a stalk or infundibulum which extends downward and is attached to the posterior lobe. The anterior and posterior lobes are separated by a clef t ; and a narrow strip of the posterior lobe bordering the cleft, has a special structure which is called the pars intermedia (Fig. 181). The anterior lobe is of ectodermic origin, is glandular in character and is developed from the primitive buccal cavity. It contains cells which are arranged in the form of convoluted tubes resting on a basement membrane. The blood supply is rich. The posterior lobe, like the anterior is supplied by a connective-tissue capsule which sends tuberculse into its substance. In the adult it consists mainly of neuroglia 346 DISEASES OF THE NERVOUS SYSTEM with a few rudimentary ganglion cells and nerve fibers. In the human embryo the nerve elements are much more prominent. The middle lobe or pars intermedia contains a connective-tissue framework and epithelial cells. It is less vascular and its cells are smaller than those of the anterior lobe. The pars intermedia contains also small cyst-like structures which contain a colloid substance something like that of the thyroid gland. The functions of the pituitary body as a whole and of its different parts, are de- scribed under the head of "Acromegaly." Its disease produces conditions which go under the names of hyper-pituitarism and hypo-pituitarism and dys-pituitarism. Acromegaly, giantism, disturbances of sexual function and of carbohydrate metabo- lism are among the disorders attributed to this organ, destruction of it not being com- patible with life. The pineal gland or epiphysis cerebri is a small organ about the size of a cherrystone placed between the posterior ends of the two thalami and attached by a hollow pedun- Foramen inter- ventriculare Anterior coiBmissure Third ventricle Corpus matnillare Subarachnoid tissue in cisterna basalis Infundibulum Hypophysis Posterior part of subarachnoid space Basilar part of the occipital Sphenoidal sinus Fig. 181. — Mesial section, showing pituitary gland and connections. (Cunningham. cle to the dorsal aspect of the mesencephalon. It lies quite near the anterior opening of the aqueduct of Sylvius, and its position is such that it is possible in certain mal- positions that it may occlude this aqueduct. It is a vestigial organ and is the repre- sentative of a median eye of invertebrates, developed from the second neuromere. It has no special sense function in vertebrates except possibly in the larval forms of the very lowest types. It is surrounded by a connective tissue capsule which sends trabeculse into the organ dividing it into small chambers. These contain tubules lined with cuboidal epithelium (Fig. 182). According to Krabbe, the pineal gland contains parenchymatous cells which he terms special pineal cells. There are a few neuroglia cells, but no nerve fibres except those that go with blood-vessels. The general statement has been that this gland degenerates at the seventh year. Krabbe has studied the anatomy of the pineal gland in children and in 100 adults. He finds no evidences of degeneration in the gland in adults and states that the struc- ture in a man aged ninety-two was similar to that of a child of fourteen. He describes the parenchymatous cells and believes them to be secretory in function. ANATOMY AND PHYSIOLOGY OF THE BRAIN 347 Investigations made by the writer with Dr. Berkeley, and the clinical and patho- logical observations made by a number of observers tend to show very strongly that the gland has secretory function, having to do with the processes of nutrition and growth. Its over-activity apparently produces increase in sexual, mental and somatic development. (See "Acromegaly.") Non-ventricular part of thalamus Groove corresponding to fornix. Quadrigeminal bodies Trochlear nerve Brachlum pontis Brachium conjunctivum Lingula Medulla oblongata Genu of corpus callosum Corpus callosum (cut) Cavum septi ■pellucidl Septum pellucidum Caudate nucleus Foramen inter- ventriculare Anterior commissure Anterior tubercle of thalamus Massa intermedia Third ventricle stria terminalis Tjenia thalami Trigonum habenulae Posterior commissure Stalk of pineal body. Pulvinar Pineal body Fig. 182. — Showing pineal gland, and basal ganglia. THE CONNECTING TRACTS OF THE BRAIN The white matter of the brain, as abeady shown, is made up of: [ Short. 1. Association fibres { Long. [ Commissural. 2. Projection fibres and interganglionic tracts. 1. The association fibres of the cortex have been already described. 2. The projection fibres are those which connect different areas of the cortex with the basal ganglia and the ganglionic masses of the pons, medulla and spinal cord. The projection fibres of the brain form the different pathways (a) by which the special and general sensations pass up to the brain cortex and (b) by which the volun- tary, automatic, and psycho-reflex movements of the body are brought about. The Efferent Tracts. — The cerebrospinal efferent paths are of two kinds: The direct or voluntary and the indirect or extra-pyramidal tracts. The direct motor or upper motor neuron tract originates from cells in the precentral 348 DISEASES OF THE NERVOUS SYSTEM convolutions; the neuraxons of these cells pass down and are gathered together in a narrow band which passes through and occupies the anterior two-thirds of the poste- rior segment of the internal capsule. The fibres continue on into the pons Varolii and medulla, and at the latter point give off some terminals which cross (except those for the sixth nerve) to the nuclei of the motor cranial nerves. The rest of the bundle passes on through the medulla, and 90 per cent, cross over at its lower portion, Crura Cerebri '<^/^^/i Medul+s Spinal Cord Spinal Cord Spinal Cord Fig. 183. Fig. 184. Fig. 183.^ — Showing the course of the pyramidal (cortico-bulbo-spinal motor) tract. Showing the upper and the beginning lower motor neurons. Fig. 184.- — -Diagram of the indirect motor tract. forming there the anterior pyramids. About 10 per cent, of the fibres do not cross, however, but continue on the same side. The crossed bundle passes into the lateral column of the spinal cord, forming the crossed pyramidal tract, which passes on, dimin- ishing in size as far as the sacral part of the cord. It gives off terminal end brushes which surround the cells of the anterior horns. The small uncrossed band of fibres continues on in the mesial part of the anterior column, forming the direct pyramidal tract, or column of Ttirck. The fibres of this tract cross over in the anterior commis- ANATOMY AND PHYSIOLOGY OF THE BRAIN 349 sure at different levels, and their teminals also connect with the motor cells of the anterior horns. From these horns the lower motor neuron passes out to the muscles. Hence the voluntary motor system is a two neuron mechanism. Each tract as it reaches the spinal cord contains about 80,000 fibres. The indirect efferent tract {long extra-pyramidal tracts) arises from nerve-cells in the anterior central convolution and, to some extent in the frontal lobe adjoining. Its fibres pass down into the internal capsule, mingling directly with those of the direct motor tract and giving off collaterals to the optic thalamus. The fibres pass through the cerebral peduncles, occupying their inner four-fifths, or motor part, and, finally, reach certain deposits of nerve-cells in the pons Varolii known as the pons nuclei. They surround these cells here with terminal end brushes. From these cells neuraxons cross the median line in the middle cerebellar peduncle and thence to the cortex of the lateral lobes of the cerebellum, where they in turn end. From here the nerve- cells send fibres to the nuclei of the cerebellum and Deiter's nucleus, thence down into the spinal cord, where they pass along mainly in the lateral fundamental columns, to connect finally with the anterior-horn cells. Other fibres from the cerebellar cortex pass to the dental nuclei, then cross to the red nuclei. Then some fibres pass spinal- ward in the lateral columns to the anterior horns. Thus it will be seen that the in- direct motor tract is composed of (a) a cortico-pontile neuron, (&) a pons-cerebellar neuron, (c) a cerebello-nuclear neuron, {d) nuclear-rubro-spinal and nuclear-Deiter- spinal, and (e) the peripheral motor neuron. The short extra-pyramidal tracts have been described (see p. 202), but they may all be grouped together here. They all end in the cells of the anterior or lateral horns of the cord. SUMMARY: EFFERENT TRACTS FROM BRAIN TO CORD 2 Neuron system from I. Pyramidal tract: (1) cortex of anterior central con- cerebellum, volution. (2) Spinal cord to muscle. 2 Neuron system from II. Short extra-pyramidal tracts. mid-brain. A. Tecto-spinal tract : (1) corpora-quadrigemina cross- ing to anterior horn cells, (2) to muscle. 2 Neuron system from B. Fasciculo-spinal tract: (1) Cajal's nucleus in pos- mid-brain. terior longitudinal bundle, uncrossed to anterior column, to anterior horn cells, (2) to muscle. 2 Neuron system from C. Rubro-spinal tract: (1) Red nucleus, crossing to mid-brain. antero-lateral tract, to anterior horn cells, (2) to muscle. 2 Neuron system from D. Vestibulo-spinal tract : (1) Deiter's nucleus crossing after-brain. in part to peripheral part of antero-lateral column 3 Neuron system from to anterior horn cells, (2) to muscle, vestibular nerve. 4, 5, or 6 Neuron sys- III. Long extra-pyramidal tract: Cortico-pontile; pons- tem . cerebellar ; cerebellar-nuclear ; nuclear-Deiter; Deiter-spinal; anterior horn cells to muscles. The direct or pyramidal tracts are all concerned with voluntary motion and in- hibition, and when the anterior-horn cells of the cord are cut off by a lesion of these 350 DISEASES OF THE NERVOUS SYSTEM tracts there is a spastic or upper neuron type of paralysis. The indirect and extra- pyramidal motor tracts are concerned in the co-ordination of bodily movement and in securing muscular tonus and synergy and in the higher reflex automatic acts. The course of the direct and indirect motor tracts is shown in the accompanying diagrams (Figs. 183, 184). The Afferent Tracts. — The next important pathways in the brain and cord are the sensory, and they are concerned in bringing tactile, muscular and general sensa- tions from the remoter parts of the body to the cortex of the brain. It will be easier Crura Cerebri Pons Pons Medulla PosTeriorRooT- Spinal Cord Fig. 185. — The afferent tract (3 neurons) for pain and temperature. to follow these tracts if we begin at the periphery and follow the course of the fibres up to their centres in the brain. The Cutaneous Sensory Tract. — A tactile irritation of the skin passes up the sen- sory nerve to a posterior spinal ganglion, where the cell body from which the fibre is derived is found. It passes directly through the gangUon, enters the posterior root of the spinal cord, and passes up to a group of cells lying in the posterior horns, where it meets and surrounds with its end brush a second sensory cell. A second neuraxon starts from the body of this cell; most fibres cross over in the posterior commissures ANATOMY AND PHYSIOLOGY OF THE BRAIN 351 of the spinal cord to the posterior column of the other side where they run up in the posterior columns and gray matter (probably in chains of successive neurons), reach the medulla and pons, and pass through this to the optic thalamus. Here they send terminals to another cell, which in turn sends a neuraxon to the cortex of the post- central convolutions. Thus the cortical centres of the direct sensory path are just posterior to the area from which the motor tract started. The direct tactile tract is made up of (1) a peripheral sensory neuron, (2) a series of intrasiiinal neurons, (3) a spinal-thalamic neuron, (4) a thalamic-cortical neuron The pain and temperature fibres take a similar course except that they cross over in the anterior commissure, do not run in chains of neurons and ascend in the lateral columns to the thalamus (Fig. 185). The deep sensory tracts convey impulses which originate in muscles, joints and the viscera. The impulses pass up sensory nerves through the posterior roots. Some now pass directly into the posterior column of the cord of the same side and ascend till they reach the upper end, where their end brushes surround the cells of the nuclei of the column of GoU and of Burdach. From there they cross to the other side in the sensory decussation. Some then go to the red nuclei and optic thalamus, where they terminate. Another neuron now carries the impulse on to the parietal convolutions . Another set of deep afferent impulses goes from the sensory roots to the cells of the col- umn of Clark, thence by the cerebellar tracts to the cerebellum. This indirect afferent tract is thus composed of (1) a peripheral sensory neuron, (2) a spinal-cerebellar neu- ron, (3) a cerebellar-thalamic, and (4) a thalamic-cortex neuron. Here, too, the mechanism is not so simple. Some of the afferent fibres do not go higher than the cerebellum, but are reflected to lower nuclei of the brain stem and thus automatic movements and the higher reflexes are brought about. The direct sensory tracts carry, for the most part, the sense of touch, pain, and temperature. Some direct sensory tracts are concerned with the sensation from the muscles and joints which have to do with co-ordination, and also with visceral sensations. It is through the indirect sensory and indirect motor tracts that the automatic and psycho-reflex acts are performed. SUMMARY: AFFERENT TRACTS FROM SPINAL CORD TO BRAIN 3 Neuron system. I. (1) Posterior gangUon cell to posterior column and Deep sensibility. nuclei of columns of Goll and Burdach, (2) cross- ing in medulla to optic thalamus, (3) to cortex of parietal lobe. 3 Neuron system. II. (1) Posterior ganglion cell to posterior horn, (2) Pain and temperature. crossing to antero-lateral column to thalamus, (3) to cortex of parietal lobe. 4 Neuron system. III. (1) Posterior ganglion cell to posterior horn, (2) Tactile sensation. partly crossing to chains of neurons in gray matter, (3) posterior and lateral columns to thalamus, (4) to cortex of parietal lobe. 2 Neuron system. IV. (1) Posterior ganglion to cells of Clark's columns, (2) Equilibrium, etc. uncrossed to direct cerebellar tract to cortex of vermis of cerebellum. 3 Neuron system. V. (1) Posterior ganglion to cells of intermediate gray Equilibrium, etc. matter of cord, (2) partly crossing to ventral cerebellar (Gowers') tract, (3) to anterior cere- bellar peduncles to vermis. 352 DISEASES OF THE NERVOUS SYSTEM Other Projection Systems. — The optic, acoustic, and olfactory projection tracts are described in connection with their peripheral nerves or end organs. THE MEMBRANES OF THE BRAIN The membranes of the brain are the dura mater, the arachnoid, and pia mater. The dura mater lines the inner surface of the skull. It is attached loosely to the concavity, but closely to the base. It splits into two layers to form the venous sinuses of the skull. The inner of the two layers at certain points projects inward to form membranous septa. These are known as the great longitudinal or cerebral falx, the lesser longitudinal or cerebellar falx, and the tentorium. Hence both venous sinuses and membranous septa are formed out of the inner layer. The outer layer forms the periosteum of the bone. The dura mater is supplied with sensory nerves, chiefly by the trigeminus but posteriorly by the vagus. The blood- supply wUl be described later. The arachnoid is a thin, transparent, fibrous, non-vascular membrane lying be- tween the pia and dura and continuous with the spinal arachnoid. It bridges over the fissures and the depressions at the base of the brain and forms between the pia and itself certain lacunae or cisterns. These are the central lacuna found at the beginning of the fissure of Sylvius, the callosal, those of the transverse fissures and of the lateral aspect of the pons Varolii. The space between the dura and arachnoid is called the subdural or arachnoid cavity. It is lined with epithelium and resembles other serous cavities. The inner surface of the arachnoid is connected with the pia by numerous delicate fibrous processes. The space between these membranes is called the subarachnoid space. It communicates with the subdural space by means of the foramen of Magendie, which lies in the part of the arachnoid that passes over the pons and medulla, closing in the fourth ventricle; also by means of the lateral recesses of the fourth ventricle. The subdural and subarachnoid spaces contain a serous fluid. It is called the cerebro-spinal fluid, and comes mostly from the choroid plexus of the lateral ven- tricles. The arachnoid contains no nerves or blood-vessels. It is described by some as a part of the pia mater. The pia mater lies beneath the arachnoid and is closely applied to the brain in all its folds. It is continuous with the spinal pia. It is very vascular and supplies the whole periphery and part of the interior of the brain with blood. It consists of two layers: an outer holding the larger vessels, and an inner delicate layer closely asso- ciated with the superflcial neuroglia of the brain. The pia mater folds upon itself and passes through the transverse fissure into the third and lateral ventricles of the brain. These vascular folds form the velum interpositum, which gives off a choroid plexus to the lateral and third ventricles. Another fold, the inferior choroid plexus, is given off to the fourth ventricle. The pia mater has vasomotor, but no sensory nerves. Functions of the Brain Membrane. — The dura mater, by its outer layer, acts as a periosteum; by its inner layer as a lymph sac. It is also, by virtue of its sensitiveness, a protection against injury and disease. It is supplied with sensory nerves, but is not, in fact, very sensitive normally and may be cut and handled without pain. Strains upon it, however, do cause some pain (Gushing). The arachnoid forms the inner wall of the sac. The pia mater is a vascular and nutritive organ. It is, however, also closely connected with the lymphatic system of the arachnoid. The blood and cerebrospinal fluid of the brain vary in amount. In congestion the cerebrospinal fluid can pass into the spinal subarachnoid cavity or be rapidly taken up by the absorbents. In anaemia and atrophy there may be compensatory increase of the fluid. The cerebrospinal fluid is a secretion and comes mostly from the choroid plexuses ANATOMY AND PHYSIOLOGY OF THE BRAIN 353 of the lateral ventricles which are covered by the ependyma. These can, if needed, pour out an enormous amount which flows into the third and fourth ventricles and thence through the foramina of Magendie and Luschka into the subarachnoid cavities of the brain and cord. It flows under a pressure of 5 to 20 mm. of mercury and is eventually absorbed by the blood-vessels (veins and sinuses of the brain). A little of it probably leaks into the cavity of the arachnoid proper. The cerebrospinal fluid is colorless, transparent, alkaline. Specific gravity, 1,007. Its composition is about as follows (Halliburton) : water 99, solids, 1. The solids consist of: Pi'oteids, 0.085; extractives, 0.060; salts, 0.865. The proteids consist of serum globulin, and allied bodies. (Albumin is a normal ingredient, according to Bernstein.) A substance that reduces Fehling' solution is present. There is no fibrin ferment, and the fluid does not coagulate on boiling, or there is only a trace of turbidity. Under the microscope it is practically free from cellular bodies. The normal amount of fluid ranges from 5ii- to §ii., being greater in the aged. There occur changes in the spinal fluid in inflammatory and degenerative diseases of the spinal cord, including syphilis, tumors of the cord and brain, and herpes zoster. The Blood-supply of the Brain and its Membranes. — The vascular supply of the scalp, skull, and dura mater comes from the external carotids; that of the eye, brain, and pia mater from the internal carotids and vertebrals. The arrangement is shown here: Occipital, inferior meningeal, arteries. Posterior auricular. [ Anterior. < Temporal \ Middle. [ Posterior. Ascending pharyngeal, posterior meningeal. Internal maxillary, middle meningeal, small meningeal. Anterior meningeal. Anterior cerebral. Middle cerebral. Posterior communicating. Anterior choroid. J Posterior meningeal. \ Inferior cerebellar. Anterior cerebellar. Superior cerebellar. Posterior cerebral. External carotid gives off Internal carotid gives off Vertebral and basilar give off The blood-supply of the meninges comes from the anterior, middle, and posterior meningeal arteries. These all come, except the small anterior meningeal branches and a small posterior branch, from the external carotid. The blood passes into the diploic veins, and from there passes chiefly into the lower occipital and lateral sinuses . Some of it, however, returns in the venae comites. It all returns down toward the base of the skull. The most important of the arteries is the middle meningeal, both on account of its size and its distribution above important functional areas. The blood-supply to the pia mater and brain substance comes from the internal carotid and the vertebral arteries. The branches of the former artery give off the anterior and middle cerebral, the posterior communicating and anterior choroid. The vertebral arteries give off the inferior cerebellar, while the basilar branch of the vertebrals gives off the transverse, anterior cerebellar, superior cerebellar, and pos- terior cerebral arteries. 23 354 DISEASES OF THE NERVOUS SYSTEM The anterior, middle and posterior cerebral arteries, are the three largest and most important. By their anastomoses the circle of Willis is formed (Fig. 161). From the circle of Willis and the beginnings of the three arteries mentioned, several groups of vessels, six in all, are given off. They enter the base of the brain and supply the great basal ganglia and adjacent white matter. They are called the central arteries; they do not anastomose, and they are the vessels usually affected in cerebral hemor- rhages of adult life. One set of these central arteries, the internal striate, passes up and supplies the two inner segments of the lenticular nucleus, the caudate nucleus and anterior part of the internal capsule. Another set, the external striate, divide into an- terior or lenticulo-striate branches which supply the lenticular nucleus and posterior Anterior meningeal artery Anterfor branch of middle meningeal artery Deep temporal arteries Infra-orbital artery Middle menin- geal artery Accessory meningeal artery Posterior superior alveolar artery •Bucciijator artery Mental artery Submental artery Posterior branch of middle men- ingeal artery Superficial tern-, poral artery Internal maxil lary artery Occipital artery' Inferior alveolar arteiy Mylo-hyoid artery Posterior auricular artery Sterno-mastoid muscle levator scapul3e_ muscle Trapezius muscle - Occipital artery- External carotid artery- External maxiUary artery- Lingual artery- Internal carotid artery Scalenus medius muscle ' Superior thyreoid artery Common carotid artery Fig. 186. — The external carotid, internal maxillary and meningeal arteries. (Cunningham.) part of the caudate. One of these larger than the rest is called the artery of cerebral hemorrhage. The other or posterior branch is called the lenticulo-optic and supplies the anterior part of the thalamus. According to Kalisko, the lenticular nucleus is supplied in part by a branch from the anterior cerebral. This he calls the artery of cerebral thrombosis. It is especially effected in severe gas poisoning. The cortical arteries are the terminal branches of the great cerebral arteries. They anastomose with each other but shghtly They are distributed very widely and carry much more blood than tLe central groups. Their distribution is shown in Figs. 18/ , 1 68. The cortical arteries are distributed in the pia, and from there they pass in two sets, a superficial and a deep, into the gray matter, and for a short distance into the white matter. They pass straight in at right angles to the surface. They ANATOMY AND PHYSIOLOGY OF THE BRAIN 355 have richly arborescent branches which do not anastomose; consequently a knife plunged straight into the brain does not cut many vessels. The capillaries are surrounded by spaces called perivascular spaces which serve as lymphatic channels. The neuroglia cells send processes which connect with or form passages to the vessel walls. Fig. 187. — Distribution of arteries on convexity of cerebrum. Middle cerebral in black-; anterior cerebral in gray; posterior cerebral in white. Fig. 188. — Arteries of mesial and inferior surface of cerebral, in black, gray and white as in previous figure. NoTE.^ — The caudate nucleus is supplied by the anterior and middle cerebral central arteries. The lenticular nucleus by the anterior choroid (part of its internal and external segments), by the anterior cerebral (caudate nucleus and external and middle seg- ments of lenticular), by the middle cerebral (caudate nucleus and middle and external segments of lenticular). The optic thalamus by the posterior communicating anterior choroid (external nucleus) and the posterior cerebral. (Fig. 209, p. 434.) 356 DISEASES OF THE NERVOUS SYSTEM The corpora quadrigemina and geniculate bodies are supplied by the posterior cerebral. The red nucleus by the posterior cerebral, and the subthalamic region by the pos- terior communicating. The internal capsule by the anterior cerebral and middle cerebral for its anterior part, by the posterior communicating, anterior choroid and the middle cerebral for its posterior part. None of the thalamus is supplied by the middle cerebral and none of the internal capsule by the posterior cerebral. All direct thalamus lesions must be posterior cerebral, or in minor degree posterior communicating. No direct capsular lesions would come from the posterior cerebral (Beevor). (See, however. Fig. 209, Bing.) The cortical areas of the brain supplied by the anterior, middle and posterior vary somewhat; the three chief systems anastomose at the parts where one region joins the other (Duret, Beevor). The cortical arteries do not anastomose with the central or ganglionic arteries which latter supply the centrum ovale. Foramenparietale I'teuis reriedra/is ^^ VmajiufiUarts externa Fig. 189. — ^The cerebral sinuses. {Hermann.) The blood of the convex and mesial cerebral surface, flowing up from the base, leaves the capillaries and enters veins. Thence it still passes upward, and for the most part enters the longitudinal sinus. The most of the vessels enter the posterior portion of the sinus and in a direction forward and upward; i.e., against the current in the sinus. The course of the blood-current is, therefore, opposed both to gravita- tion and to the venous flow. The veins of the cerebrum are: 1, the superficial cerebral; 2, the deep cerebral and, 3, the cerebral sinuses. 1. The superficial cerebral veins are venae comites. Those on the convex and mesial surfaces empty chiefly into the superior longitudinal sinus, as described; those on the basal surface and temporal lobes empty into the cavernous and lateral sinuses. These veins have no valves, and their walls are very thin and without muscular fibres. 2. The deep cerebral veins, or venae Galeni, receive the blood from the lateral ventricles and from some of the central arteries ANATOMY AND PHYSIOLOGY OP THE BRAIN 357 supplying the basal ganglia. They empty into the straight sinus. These deep or central veins when compressed lead to stasis and internal hydrocephalus. 3. The cerebral sinuses are fifteen in number. The important ones are the superior and in- ferior longitudinal, the straight, the lateral, the occipital, the cavernous, and the superior and inferior petrosal. They carry blood for the most part in a direction from before backward, and convey it eventually to the internal jugular (Fig. 189). Most of the blood of the convexity and mesial surface must pass into the longitu- dinal sinus, but there is a slight connection of some of the veins with the superior petrosal and straight sinuses. The superior longitudinal sinus also communicates slightly with veins of the scalp and with the facial vein. Some of the blood from the mesial surface also goes to the veins of Galen. On the whole, however, the system of the convex and mesial cerebral surface is a close corporation, the blood having to pass into the superior longitudinal sinus and Fig. 190. — Localization" of functions of the cortex of the brain. Median surface. torcular Herophili, where it meets that of the straight and occipital sinuses, and flows forward through the lateral sinuses to the internal jugular. The circulation of the basal surface is less isolated. All the basal sinuses communicate with each other freely, and there are slight communications between the veins of the scalp and the cavernous, lateral, and inferior petrosal sinuses. It is safe to tie any of the sinuses, except the lateral and the posterior part of the longitudinal. The cerebellar veins, superior, inferior, and lateral, empty into the straight, the lateral, and superior petrosal sinuses. None of the cerebral veins or sinuses have valves. The pressure of the blood as it goes through the external carotid is about 150 to 200 mm. of mercury. The pressure of the blood in the cranial cavity is much less (from to 50 mm. of Hgr., Hill). The pressure in the central arteries is greater than that of the cortical. The pressure of the venous blood is very slight and is ordinarily the same as that of the cerebrospinal fluid. Both arteries and veins are more delicate than the extra-cerebral vessels. Except in gray matter, the brain is not a very vascular organ, but this gray tissue 358 DISEASES OF THE NERVOUS SYSTEM ranks in richness of blood-supply with the lungs and liver. The amount of blood in the brain at any one time is only about 1 to 2 per cent, of the total blood in the cir- culation, or about four ounces (Ranke). THE FUNCTIONS OF THE BRAIN Cerebral Localization. — -The brain is the seat of conscious intelligence and other psychic activities. It exerts control and direction over voluntary movements, it is the seat of instinctive acts, and it regulates in a measure the vaso-motor, trophic, and secretory mechanisms of the body. The Prefrontal Lobes. — ^The prefrontal lobes, or that part of them which is in front of the precentral convolution, are concerned with the higher association processes and memories. The most anterior portion, according to Campbell, though of late development is poor in cortical cells. In the posterior part are centres for movements - .^- '"•""^'^^WHit Fig. 191. — Same. Superior surface. of the head and eyes, and in the left third frontal is a centre for speech or articulation memories. Injuries of the prefrontal generally lead to changes of character, indi- cated by peevishness and irritability of temper, mental enfeeblement, lack of power to concentrate the mind or to control the acts or emotions. The left lobe is said to be more important psychically. The Central Convolutions. — This part of the brain is called the sensori-motor area, because it is concerned in the receipt and emission of nervous impiilses which lead to voluntary motions of the body. Certain parts of this area are in relation with certain groups of voluntary muscles on the opposite side of the body. These areas preside not so much over single muscles as over those groups of muscles which act together in producing definite, purposeful acts. The motor area proper is confined to a narrow ANATOMY AND PHYSIOLOGY OF THE BRAIN 359 space on the posterior surface of the precentral convolution extending down to the bottom of the fissure of Rolando. It is co-extensive with deposits of the giant cells of Betz. (Sherrington and Greenbaum). The lower part of the central convolutions, known as the central operculum, is a centre for movements of the larynx, mouth, tongue and face. This area reaches to the lower bend or genu of the fissure of Rolando, near which is the centre for the neck. Above and occupjang the middle third of the convolution are centres for the shoulder, arm, hand and fingers, which extend up as far as the upper genu, near which is the centre for the trunk. Above this genu are centres for the hip, leg, toes, the area extending over into the paracentral lobules. Near the base of the first and second frontal convolutions is a centre for movements of the head and eyes. The exact arrangement of these centres, which has been deter- mined by experiments upon monkeys and other lower animals as well as by clinical and surgical observations on man, is shown in the accompanying Figs. 190, 191, 192. The posterior central convolution is probably the centre for cutaneous sensations. . , fRCCENTRAL ^-:rU ,POSTCENTPAL 'v.^Tl '^'^n si Fig. 192. — ^Localization of the functions of the brain. Lateral surface. The various sensori-motor centres are not sharply limited, but lap one over the other, so that the motor area for the arm, for example, extends over somewhat into that for the shoulder. The corresponding sensory areas are more diffuse, so that it takes a much more extensive destruction of a certain area of the cortex to produce an anaesthesia of the arm than it does to produce a paralysis of the arm. The strictly motor and sensory areas have closely adjacent to them psychic-motor and psychic- sensory areas, concerned in the higher elaboration of movements, sensations and reflexes. Bilateral Representation. — Those muscles of the two sides of the body which act together have a double representation in the brain. For example, each group of mus- cles used in inspiration has a centre in both hemispheres; consequently, when^one 360 DISEASES OF THE NERVOUS SYSTEM centre is destroyed no paralysis results, for the reason that the other centre continues its work. In the same way some of the muscles of the face, such as those for closing the eyes, have a double representation, and a lesion destroying the centre for the or- bicularis palpebrarum on one side will not usually cause paralysis, because of the con- tinued action of the centre of the other side. The more perfect and habitual the as- sociated action of the muscles of the two sides of the body, the more completely can one centre do the work of its associate. The best examples of the muscles having the double representation are the orbicularis palpebrarum, the muscles of the vocal cords, the muscles concerned in deglutition and in respiration. The muscles of the viscera and blood-vessels have no known representation in the cortex of the human brain. The special sensations have a bilateral representation also; but the more specialized the sense the less can one hemisphere take the place of the other. Fig. 193. — ^Localization of the functions of the brain cortex, showing the psychic areas. (Campbell.) Lateral surface. Parietal Lobes. — -The upper portion of the parietal lobe is the centre or region, for we include both the cortex and its underlying tracts, for association of memories of form, or more properly the perception of the form. When destroyed there is as- tereognosis or loss of perception of form; and if with it there is some involvement of the zone of language, there is asymboly; that is, an inability to tell what the object is even if the form is recognized. Lying anteriorly in the lower part of the parietal convolution below the parietal fissure, is an area for memories of muscular movements, and when this is injured there is ataxia, or loss of sense of position. The whole parietal lobe seems to have to do with association processes connected with the elaboration of sensations. It is a percipient or, as Mills calls it, a concrete memory centre, not a sensory centre. Even the ataxia which occurs here is a loss of perception, and not a pure sensory loss. In its posterior and inferior part, on the left side, we have part of the zone of language, particularly that concerned in memories of written words (reading centre). Near this is, perhaps, a centre for memories of the uses of things (praxic centre). ANATOMY AND PHYSIOLOGY OF THE BRAIN 361 Occipital and Temporal Lobes— Centres of Special Sense. — The special senses have two centres — the primary and the secondary. The primary centres are connected with the gangUa at the base of the brain; the secondary centres are situated in the cortex. The primary centre for vision is in the posterior part of the optic thalamus, the external geniculate bodies, and anterior corpora quadrigemina. The secondary centre is situated in the occipital lobe, and particularly upon its mesial surface and VISUO-SENSORY Fig. 194.— Localization of the functions of the cortex of the brain, showing the psychic areas. {Campbell.) Superior surface. about that part of the cuneus known as the calcarine fissure. Each occipital lobe is the centre for visual impulses from the corresponding half of the retina of each eye; for example, the left occipital lobe is the centre for vision of the left half of the retina of each eye. This relation is shown in the diagram (see Optic Nerve). Total destruc- tion of both occipital lobes, or even of a considerable part of them if the destruction involves the median surface, will cause bUndness. Destruction of one lobe causes only half-blindness or hemianopsia. 362 DISEASES OF THE NERVOUS SYSTEM The primary centre for hearing is in the posterior tubercles of the corpora quandri- gemina and the internal geniculate bodies. The secondary centre is in the cortex of the first, including the transverse convolution of the temporal lobe. Destruction of one temporal lobe causes some deafness in the opposite ear. This deafness, however, is not complete because the sense of hearing has a bilatei'al representation; each ear in other words, sends fibres to the temporal lobes of each side, although more fibres cross over than go to the lobe of the corresponding side. The consequence is that the loss of one temporal lobe is in a measure supplied by the other (see also Aphasia). The primary centre for smell is in the olfactory lobes. The secondary centre is probably in the anterior part of the limbic lobe, the uncus and in part of the hippo- campal convolution. Whether the tracts for the sense of smell are connected with the optic thalamus or other ganglia is not definitely known. The primary centre for taste is not known, but sensations of taste may connect ^POSTCENTRAL PSYCHIC-SENSORY JSrcr/VFRAL SENSORY Fig. 195. — -Same. Median surface. with the optic thalamus before passing into the secondary centre, which is in the hippo- campal convolution. Centres for Special Associations. — There are certain sensations, perceptions and movements, simple in character and frequently repeated, so that they finally get to be used almost automatically in their work. These impressions relate to the use of the muscles in speech, in writing and in gesture language; also to other frequently repeated purposeful movements of the limbs. The muscular movements in writing and speaking are so often repeated that certain areas in the cortex are set apart for the memories of these processes, memory being simply a revival and re-association of previously registered impressions. The visual sensations and the ideas elaborated from them, which are frequently repeated in learning to read, have also a centre which is set apart for them. This forms a centre for the memories of written language. In the same way there are auditory sensations and ideas elaborated and so frequently repeated as to be used automatically in acquiring language. These are stored up as ANATOMY AND PHYSIOLOGY OF THE BRAIN 363 auditory memories. We have what may be called motor memories connected with speech and gesture. These special memories have been found to have a certain localization in the brain. The centre for the memories of the articular movements of speech is in the posterior part of the third left frontal convolution; the centre for the memories of the movements of writing is not perfectly known, but is thought to be at the posterior part of the second left frontal convolution. The centre for the memories of gesture language is unknown. The centre for the memories of ordinarj^ co-ordinate movements is probably in the inferior parietal lobule. The centre for the visual memories of written language is in the angular gyrus, extending backward from there into the occipital lobe. The centre for the audtiory memories of spoken language is in the posterior part of the first and the corresponding upper part of the second temporal convolution. In right-handed people the language memory centres are in the left cerebral hemisphere; in left-handed people they are in the right hemi- sphere. The centres for the memories and associations connected with language together form a large area, known as the zone of language. The destruction of these memorj^ centres produces different forms of aphasia, as will be described later. In addition to, disturbances in centres are produced by remote lesions which cut off the associating fibres connecting these centres with each other or with motor or sensory centres proper, causing a phenomenon called diaschesis. The Centrum Ovale, Corpus Callosum, and the Associative Functions of the Brain. — The different parts and centres of the brain are connected together by the associating tracts and with lower levels by the projection fibres. The simpler and less developed centres of the two halves of the brain are closely connected by fibres that nm chiefly in the corpus callosum. The more highly specialized and less simple in function a centre, the less close is its commissural connection and the more inde- pendent is one half of the brain from the other. Thus the centres for the movements of the thorax in respiration are closely bound with each other; those for the purposefiil movements of the hands less so; those for receiving visual impressions are almost independent; and the centres for language associations which are still more highly specialized, are practically entirely independent. We infer that the higher mental functions, therefore, work either in one cerebral hemisphere or in the other, and that the two halves of the brain do not co-operate with each other in much of the higher intellectual work. This, however, may not be true for the very highest mental proc- esses, though persons have lived and shown fair intelligence without a callosum. The corpus callosum is the great commissural tract connecting the two cerebral hemispheres and their respective centres. The anterior commissure does some of the same work, being more specially connected with the function of olfaction. The posterior commissure has comparatively few bilateral connecting fibres, its function being more to connect the thalamus with the cranial nerve nuclei and other centres below. The Corpus Striatum. — This ganglion is in relation with the cerebral cortex slightly, with the internal capsule slightly, with the thalamus and subthalamic nuclei, and probably through these with the cerebellum and with nuclei in the pons. It is also in connection with fibres that come up from the muscle-sense tract, in the spinal cord. It seems most intimately connected with the thalamus and subthalamic ganglia. Its functions are therefore probably connected with supplementing automatic movements. Destruction, of this ganglion in the human brain produces at times motor and vaso- motor disturbances. Lesions of it seem to have to do indirectly with the production of tremor and rigidity. The Thalamus Opticus. — The thalamus is in relation by its projection fibres with the frontal, parietal, occipital, and temporal cortex. It is in relation below with tracts for cutaneous and deep sensibility. The fibres that go to the occipital cortex 364 DISEASES OF THE NERVOUS SYSTEM are connected with the optic tract, and have to do with the function of vision. The fibres that go to the temporal lobe are connected with the auditory tract, and have to do with the function of hearing. The optic thalami have a probable relation to the expression of emotions. Lesions of the posterior part of the thalamus will produce hemianopsia and central pain. Other than this, lesions of the optic thalamus produce no definite symptoms which enable us to make a local diagnosis, except through injury of neighboring parts. Disturbances of hearing have not certainly been traced to lesions in the thalamus. Lesions of the thalamus sometimes produce various forms of mobile spasm, but these are generally attributed to irritation of the fibres of the internal capsule, which go close to it. Hence, aside from pain, disturb- ances of vision and of deep sensibility, the optic thalamus also must be considered clinically a latent region. The Corpora Quadrigemina. — The anterior tubercles of the corpora quadrigemina, together with the external geniculate bodies, form part of the primary centres of vision, The anterior tubercles, however, have to do chiefly with reflex movements of the pupil and the ciliary muscles. The posterior tubercles of the corpora quadrigemina and the internal geniculate body are connected with the auditory nei've, and have to do with reflex movements associated with hearing and space sensations. They also appear to receive some fibres from the cerebellum; their injury or disease produces some disturbances in equilibrium and possibly in hearing. Owing to the fact that the nuclei of the third nerves and the red nuclei lie beneath the corpora quadrigemina, lesions of these latter produce nystagmus and paralyses of the third nerve, disturbances in equilibrium, and forced movements. Lesions in this neighborhood sometimes cause somnolent and stuporous states. The red nuclei are connected with the anterior cerebellar peduncles on the one hand and with the lenticular nucleus and optic thalamus and spinal cord on the other, and are concerned in securing muscular tone, synergy and co-ordination. The Cerebellum. — It receives impulses from the cerebral hemispheres, and these impulses go via the pons to the cortex of the lateral lobes. It also receives impulses from the vestibular and to some extent from the other sensory cranial nerves. These impulses go to the cortex of the vermis. The cerebellum also receives impulses from the spinal cord via the direct (dorsal) cerebellar tract and the column of Gowers (ventral cerebellar tract), but not from the posterior columns. These impulses go to the cortex of the vermis. The efferent tracts of the cerebellum are those to the cerebrum and those to the mid-brain, sub- thalamic region and spinal cord. The cerebello-cerebral fibres leave the cortex of the lateral lobe, go to the dentate nuclei, thence through the anterior cerebellar peduncles, crossing, in most part, to the red nucleus and thalamus, perhaps to the nucleus tecti, thence to the cortex of the brain. The cerebello-spinal fibres start from the cortex of the vermis, then to the roof and emboliform nuclei (intrinsic cerebellar nuclei), thence to the nucleus of Deiters mainly (paracerebellar nuclei of Horsley), thence to the spinal cord in the anterolateral tracts, and anterior cornua. Some cerebello-spinal fibres go from the red nucleus and tectal nucleus to the cord. Hence we have vestibular-spinal, the rubro-spinal and the tecto-spinal tracts convey- ing impulses down to the cord, originating mainly at least in the cerebellum. Horsley has shown that the cerebellar cortex always sends out its efferent impulses first to the various nuclei of the cerebellum. Stimulation of these causes tonic move- ments of the head. Stimulation of the paracerebellar nuclei (Deiter's,) in the pons also causes tonic movements of the body. It is when these intrinsic and paracerebellar nuclei are irritated that we get cerebellar fits. L. Hill and others have shown, as Jackson first averred, that the cerebellum causes tonic fits, the cerebral hemispheres clonic fits. There is a nervous circuit between the cerebrum, brain axis, cerebellum. ANATOMY AND PHYSIOLOGY OF THE BRAIN 365 and spinal cord. The cerebellum has thus the function of organizing the higher auto- matic and psycho-reflex movements, and through its relations with the eighth nei-ve and spino-cerebellar tiacts of enabling us to keep our equilibrium and maintain our relations in space. It also gives tonus and synergy to the muscles. The vermis or median lobe is the part which in man is most important in doing this work. The lateral lobes are concerned in acquiring new movements and dexterities and in syner- gizing different segments of the body (neck, arms, legs). (See also Figs. 218. p. 462.) The pons Varolii contains some of the cranial nerve nuclei and collections of nerve- cells which are connected with fibres from the cerebral cortex on the one hand and the cerebellum on the other. It also contains the long tracts of nerve-fibres that pass from the cerebrum down through into the medulla and spinal cord and transverse tracts of fibres which connect the two hemispheres of the cerebellum. Lesions in it cause disturbances in function of the cranial nerves and of the motor, sensory and commissural tracts. The medulla oblongata contains centres of the cranial nerves, and in it also are various reflex and automatic centres controlling and regulating the vasomotor system respiratory and cardiac rhythm, visceral movements and secretion. The olivary bodies are connected with the cerebellum, basal ganglia, and with the spinal cord. When injured, disturbances of equihbrium and co-ordination occur. The Latent Regions of the Brain. — There are certain parts of the cerebral cortex destruction and irritation of which produce no special and distinctive phenomena in man. These are the greater part of the temporal lobe of the right side and a portion of the temporal lobe on the left side. A part of the inferior parietal lobule also may be regarded as a latent region. The frontal lobe we have already spoken of as being concerned with certain mental functions, but lesions here often produce no symptoms, and may be to a certain extent regarded as latent. Brain Size and Weight.— The length of the brain averages 160 to 170 mm. and its transverse diameter is 140 mm. The female brain is usually shorter than the male. The average weight of the male brain is 1,358 grams; that of the adult female, 1,245 grams. The weight varies with age, sex, race, and intelligence, and with a number of other factors. The average weight of the brain at birth is 327.8 gm.; the brain grows rapidly until the age of four, then more slowly until the age of seven, then very slowly up to the age of sixteen to twenty. At about the age of forty-five in man and fifty in woman it begins to lose weight slowly, and at the age of eighty or over it has lost about 120 gm. (4 oz.). The brain of man weighs absolutely about 9 per cent, more than that of woman. Relative to the body weight, the brain weight of man is about 2 per cent.; that of woman is very little less. The sexual difference is extremely small. Brown finds that, after making all allowances, woman's brain weighs about one ounce less than man's. The brain weighs more in the civilized races, and more in certain of the civilized races than others; the brains of English, German, and Scotch weigh more than those of French, Italian, and Russian. Some of the African and Australian tribes have the smallest brain, the average negro brain weighing 1,250 gm. Caucasian race average brain weight is 1,335 grams. Chinese race average brain weight is 1,332 grams. Malay race average brain weight is 1.266 grams. Negro race average brain weight is 1,244 grams. Australian race average brain weight is 1,185 grams. 366 DISEASES OF THE NERVOUS SYSTEM The brain weight of highly civilized nations is somewhat larger than that of the uncivilized, and cultural activity in a race rather slightly increases the cranial capac- ity. When a brain weighs less than 1,130 gm. in man or 990 gm. in woman, it is called a microcephalic brain; if the weight is above 1,490 gm. in man or 1,345 gm. in woman, it is called a megalocephalic brain. Brain weight has a certain relation to inteUigence, which is not, however, an abso- lute one. Among a hundred men of more than average intelligence, the percentage of large brains would be about 25, whereas the percentage of large brains among per- sons of ordinary or low intelligence would not be more than 4 or 5. In estimating the importance of brain weight, one must consider the height, the weight or volume of body, muscular mass, and superficial area; these are called the somatic factors. The following formula has been devised by Snell for estimating the mental power of different animals. ^ Ks In this formula P represents the psychical factor or the amount of intelligence, H the brain weight, K the body weight, S for the somatic factor. The somatic factor has been estimated to be for mammals about 0.666. Applying this formula, we find that, expressed relatively the intelUgence of man equals 0.87; woman, 0.86; the ape, 0.42; the rabbit, 0.59; the birds from 0.167 to 0.09. The brain weight as compared with the body weight is not so great in man as it is in certain birds. The weight of the brain in the adult male as compared with the spinal cord is greater, however, than in any other race. That is to say, the spinal cord in the human subject weighs approximately 2 per cent, of the brain. In the anthropoid apes it weighs 6 per cent. ; in American mammals, from 23 to 47 per cent. The relative weight of different parts of the brain is about as follows: frontal lobes, 28 per cent. ; parietal lobes, 36 per cent. ; occipital, 10 per cent. ; temporal, 13 per cent.; island of Reil, 9 per cent.; pons, 1)4 per cent. The cerebellum weighs about one-eighth as much as the cerebrum. The proportion of the gray to the white matter in adults is 60 to 40 (Vierordt). CHAPTER XVII DISEASES OF THE BRAIN AND ITS MEMBRANES GENERAL SYMPTOMS General symptoms are developed when the brain and its membranes are subjected to irritating influences. These influences may be of various kinds, and are best illustrated by the irritant which occurs in the early stages of a febrile infection like meningitis. Similarly, general symptoms may be caused by conditions which benumb and depress or paralyze the activities of the brain. These influences are either mechanical, such as those of brain pressure, or perhaps severely toxic such as occurs in the advanced stages of an inflammation. Perversions of brain activity in various directions partly irritant, partly paralyzing also may occur. There are also disturbances of brain function due to irritative or destructive foci in the brain. The general symptoms of brain irritation are headache, vertigo, vomit- ing photophobia, mental irritabihty, insomnia, pecuhar feelings of full- ness and pressure about the head, noises in the ears or in the head, tenderness about the scalp, and in severe cases rigidity of the neck, con- vulsive symptoms and delirium. The symptoms of depression of the brain functions are often caused by an inflammatory exudate or serous effusion which compresses the brain and hence they are spoken of as symptoms of brain compression. These, as they progressively increase, are headache, mental dulness, restlessness, mental confusion, clouding of consciousness, delirium, dis- turbed sleep and finally stupor and coma. As these symptoms progress there occur a slowing of the pulse, increased blood-pressure, irregular respiration, and papillary cedema of the optic discs. Finally the coma deepens, the respiratory and bulbar centres become paralyzed; there is now a rapid pulse and lowered blood-pressure, paralysis of respiration and death. The focal symptoms of brain disease depend upon the location of the particular lesion. If it is in the motor area there will be symptoms of spasmodic character or of a local paralysis. If it is in the speech area there will be symptoms of aphasia. Besides the symptoms which are due to the local disease there may be general symptoms produced by the pathological condition. Thus, 367 368 DISEASES OF THE NERVOUS SYSTEM while a tumor of the brain will cause perhaps only focal symptoms of irritation or pressure, a suppuration in the brain will produce general symptoms such as are associated with sepsis, viz., chills, irregular fever, mental hebetude, prostration, emaciation, and sweats. The dominant focal symptoms are: local convulsions, hemiplegia, hemiataxia, hemianopsia, cerebellar ataxia and dyssynergy, cranial nerve palsies, and aphasia. Among these symptoms there is one which deserves some preliminary general study, because it is caused by lesions of different kinds and occurs in different forms of diseases. This symptom which we wish particularly to study here is aphasia. Aphasia. — Aphasia is a disorder of the faculty of language ; and it has a number of varieties, in accordance with the particular part of the brain involved and the particular portion of the mechanism of this faculty that is injured. By the faculty of language we include the processes by which we hear, see, and appreciate the meaning of symbols of language; and express to others by voice, writing, or gesture the content of our minds. Language has, therefore, a receptive side and an emissive side. We may have lesions in the brain which destroy that part of the lan- guage faculty concerned in our power of seeing and understanding written words or the gesture language. In reading understandingly one sees certain words; these words revive certain visual memories con- nected with past perceptions. Thus one sees the word "book;" this suggests to him past memories of form, color, tactile and other sensations associated with the past perceptions of books. There is a certain centre in the brain where these visual memories for letters and words are asso- ciated. When this centre is destroyed the memories are destroyed and the word ''book" or any other written word conveys no meaning. The patient can spell out the letters, he can see the letters, but he cannot read any more than if he had never been taught. The condition is known as alexia or ivord blindness. Again a person may have learned to associate certain gestures with definite ideas, as the motion of carrying a glass to the mouth suggests that of drinking, or the motions of using a knife and fork that of eating, or the motions of the deaf-and-dumb alphabet with certain words and ideas. These memories of gesture language are located in certain regions, and when they are destroyed the patient is no longer able to understand gestures or the sign language. This condition is known as sign blindness. When a person is not able to understand the significance or uses of things about him, he is said to have apraxia. For example he sees a knife and names it but he does not know its use. He knows its use but he does not know how to make the movements necessary to put it to use. In the former case he has sensory apraxia (imperception, agnosia are DISEASES OF THE BRAIN AND ITS MEMBRANES 369 other terms). In the latter case he has motor apraxia (also a form of agnosia) . A person hears certain words, as, for example, the word "knife." This conveys to him a certain idea of the form, color, and other properties associated with knife. The memories associated with the auditory per- ception of different words are stored up in a certain locality which is the centre for auditory memories. When this centre is destroyed the person hears spoken words, but they convey to him no meaning. All that is said to him sounds as if it were in a foreign language: he hears, but he does not understand. This condition is known as word deafness, or audi- tory aphasia. Varieties of auditory aphasia occur. In some cases for example, a patient cannot name objects seen, felt, heard or touched (anomia) . In communicating our ideas, we speak, write, and make gestures. In speaking we make use of the organs of articulation, and this use in- volves the fine adjustment of a delicate muscular apparatus. In the act of expressing ideas we have to bring into play the memories of the past muscular movements of this articulatory mechanism. These move- ments were learned by a slow and painful process during infancy. After the power of speech is acquired, the mechanism works readily and almost automatically, because we only have to send a stimulus to the centre which presides over the stored-up memories of the impulses to innervate properly the mechanism of speech. There is, therefore, a centre for the memories of the movements of articulation — a centre which is, of course, closely connected with the motor areas that directly innervate the larynx, pharynx, and oral and facial muscles. When a lesion destroys this centre for speech memories, a person is unable to reproduce the words necessary for expressing an idea; for example, he sees a knife, he knows what it is, but the memory of the motions necessary to express the word "knife" is gone. To him it seems that the name is gone, and that is the common way of expressing it. He cannot say the word "knife." The patient may wish to express the idea of pain. He feels the pain, he knows that he has pain, but he cannot revive those motor memories which are con- cerned in expressing the word "pain;" he cannot tell, therefore, in words what is the matter with him. When a person is thus troubled, he is said to have a form of motor aphasia for which the particular name given is aphemia. In the same way there is a centre for the memories of the muscular movements concerned in writing; and when a lesion destroys this centre the patient is unable to write, though he may be able to speak. This condition is called agraphia. There is a centre, less well defined, for the memories of the move- ments used in gesture language, and when this is destroyed the person 24 370 DISEASES OF THE NERVOUS SYSTEM is unable to express his ideas by gesture or sign language. This condition is known as amimia. A loss of memory of musical expression is called amusia. In some cases, patients are able to speak and write, but they skip words, repeat often, and talk confusedly. There is here a lesion of the tracts associating the language centres, and the condition is called con- duction aphasia, while to his stumbling speech the term paraphasia is given. Thus symptomatically and psychologically we have the following forms of aphasia: Sensory. Motor. Alexia. Sign blindness. Apraxia. Word deafness. Aphemia. Agraphia. Amimia. Amusia. Conduction aphasia. Mixed Forms. \ Paraphasia. [ Jargon aphasia. In the examination of a case of aphasia, questions to bring out the following points should be put to the patient: To test his power of expression: 1. Can he speak voluntarily or use ejaculations expressive of some emotional state? 2. Can he answer questions? 3. Can he count or repeat letters of the alphabet? 4. Can he repeat words? If he can talk, does he talk coherently, or is it a jargon, or does he skip words now and then as he talks? 5. Can he hum tunes? 6. Can he write voluntarily or to dictation? Can he copy? 7. Can he indicate his wants by gestures? To test his power of understanding language : 8. Does he understand the words spoken to him, or gestures made to him? 9. Can he name things seen, heard, felt or can he name abstract things, like darkness, beauty, sorrow? 10. Can he recall to his mind objects named to him? That is, if the word "poison" is spoken, does he know what it means? 11. Can he read silently or aloud? 12. Does he know the uses of things about him, such as how to use a knife and fork, or a pencil, and what is his general intelligence? By a series of questions of this sort we can make a diagnosis of the DISEASES OF THE BRAIN AND ITS MEMBRANES 371 psychological ciuality of his speech defect, and say that he has "auditory" aphasia, or "motor" aphasia, or a "visual" aphasia, and so on. The physician, however, can ignore this kind of diagnosis, to a large extent, and he had best try first to determine the anatomical location of the aphasia, and arteries involved in the lesion. By the method of ques- tioning which has just been laid down, the patient is discovered to have certain peculiar defects of language, and, in accordance with these de- fects, the physician is able to assign the trouble to one or another portion of the brain. 1. Thus, in one very common form of speech disturbance, we have a fronto-capsular lesion, i.e., a lesion injuring the left third frontal convolu- tion and often extending back so as to involve the internal capsule somewhat. Here, the patient has usually hemiplegia but no sensory disturbance, and his intelligence is quite good. The main characteristic of his speech defect is that of aphemia, that is, a complete inability to say a word, or, at least, only one or two simple words. With this mutism, there is also usually a loss of ejaculatory speech, and of responsive speech, of counting or association speech, and of ability to repeat words that are spoken. Yet, though thus completely aphemic, the patient can write and read well, and understand spoken language perfectly. The lesion here is in the third left frontal convolution, extending, perhaps, somewhat into the lenticular zone (Fig. 173), and due to involvement of one of the ganglionic arteries, which does not supply much of the corpus striatum. Sometimes there is an involvement of the cortical arteries. Pure frontal lesions are very rare, because the third frontal convolution is not sup- plied altogether by a single artery but by several. Occasionally this portion of the brain is rather extensively involved, and there may be with some hemiplegia, also evidences of difficulty in understanding spoken language. Sometimes the pure fronto-cortical lesion causes an aphemia, like this described, which is temporary, because the lesion is so small. This last form of aphasia is sometimes known as "pure motor" aphasia or "pure motor subcortical" aphasia. 2. The second type of aphasia is an "occipital " or " parieto-occipital " aphasia, known clinically as "pure alexia." In this group of cases, the patient can talk well, and understand, and write well, but he cannot read, except a few words or letters, and he can- not copy well. He has generally a hemianopsia, and sometimes the alexia is wrongly attributed to this. Usually there is with it some hemiataxia or hemianesthesia or hemiparsesthesia, and there may be a very slight temporary hemiplegia. The intelligence of the patient is good. These cases are quite rare also. They are due to a lesion in the occipital lobe, extending sometimes into the angular gyrus. This part of the brain is supphed by the posterior cerebral artery. When lesions of this 372 DISEASES OF THE NERVOUS SYSTEM artery occur, they involve the visual centre often, and also the so-called association or visuo-psychic centres concerned in reading. These are situated in the angular and in the second occipital gyri. While the lesion is 'usually due to involvement of the posterior cerebral, alexia may be caused also by lesions of the Sylvian artery, since it supplies some of the angular gyrus, but in this case we get some injury of the temporal cortex, and an alexia which is complicated with other symptoms (Fig. 196.) 3. A third group of aphasias is a "temporal or ''parieto-temporal" or the ''sensory aphasia of Wernicke." Here the patient is found to have no hemiplegia, or only a temporary and slight one, but his power of coherent speech is seriously injured. He has some voluntary speech, Fig. 196. — ^Lesion of Sylvian artery, and softening of temporo-parietal region, with aphasia . (Spiller.) and responsive speech, and may use words very fairly, even loqua- ciously, but he talks nonsense on account of the confusion, repetition and misplacement of words. He has, in other words, paraphasia or jargon. He cannot name objects shown him, and usually not objects that are heard by him, or felt, or smelt, or tasted. This constitutes a symptom known as "anomia," or "optic aphasia." He can read a little, but very poorly, and write a httle and copy, but this is all done very badly. In other words, he has some alexia and agraphia. He cannot understand questions or simple directions, or, if he does understand simple directions, he cannot understand comphcated ones, and there is, apparently, some loss of general intelligence. Patients act sometimes rather childishly and emotionally. Perhaps the dominant symptoms may DISEASES OF THE BRAIN AND ITS MEMBRANES 373 be said to be this inability to understand, and together with confusion and jargon or paraphasia of speech. With this speech defect there may be, as already stated, a temporary or slight hemiplegia. There is usually some hemiansesthesia or ataxia or astereognosis, or all three of them. There is sometimes a hemianopsia, which may not be permanent. Such persons im- prove a good deal in their intelligence, but rarely get entirely normal, and they have to be watched rather carefully. They do not show symptoms of any true dementia. The lesion, in these cases, is in the auditory and the auditory psychic area of Campbell, that is to say, in the posterior two-fifths of the first temporal convolution, extending, in severe cases, into the parietal lobe or into the second temporal. There is a cutting off, to some extent, of the association tracts between the auditory psy- FiG. 197. — Showing the zone of language and the localization of the four types of aphasia. I. Frontocapsular aphasia, characterized by aphemia. II. Parietooccipi- tal aphasia, characterized by alexia. III. Temporal or temperoparietal aphasia, characterized by anomia, some mind deafness, and paraphasia. A small lesion may cause only optic aphasia. IV. Frontolenticular aphasia, the common type of aphasia, characterized by aphemia with much agraphia, alexia, mind deafness,,and hemiplegia. chic and the visual area. The artery here involved is, of course, one of the terminal branches of the Sylvian, or perhaps the trunk of the Sylvian itself. In this latter case, when the lesion is extensive, the speech is apt to be very confused, and almost purely jargon, and there is more likely to be some hemiplegia. Occasionally, a very small lesion of the first temporal alone occurs, and here the patient is simply unable to name ob- jects seen, and often objects that are felt, or heard, or smelt, or tasted. In this case, we have what is called a "pure anomia," or inability to name things. Another term for is is ''optic aphasia." 4. The fourth group of aphasias is the "fronto-lenticular" or "len- ticular zone" aphasia and "mixed" aphasia. This is the most common of all aphasias. We here find a very extensive aphemia, that is, inability 374 DISEASES OF THE NERVOUS SYSTEM to talk voluntarily or even to say any words. There is a considerable loss of ability to read and reading is always imperfect; that is, there is some alexia; there is also some difficulty in understanding, at least compli- cated sentences; that is, there is some mind deafness. There is always a very decided hemiplegia, but rarely any sensory symptom. The lesion here involves the branch of the middle cerebral that supplies the corpus striatum, the internal capsule, and the fibres converging into it from the second and third frontal and precentral convolution. In other words, the lenticular zone is involved and part of the fibres that come from the temporal and frontal areas of speech are destroyed. In some way also, the association tracts from the occipital lobe must be affected, be- cause of the difficulty in reading. This form of aphasia, which I call, anatomically, the "lenticular zone" aphasia, is called by Marie, an anarthria plus the aphasia of Wernicke. The accompanying diagram will show the anatomical localization of the four different groups of aphasias. And I am sure that the student and physician, in attempting to make a diagnosis of aphasia, will find the task much lightened by turning his attention to the effort to locate, anatomically, the area of the brain and the artery involved. Conduction and Mixed Aphasia. — There are very few cases of pure conduction aphasia. When it occurs there is paraphasia and paragraphia ; the patient repeats words over and over in a kind of verbal intoxication, or mixes things so that the speech is almost gibberish. Still he can ex- press himself and can write, read and understand. The lesion is usually in the island of Reil or the convolutions about the fissure of Sylvius. It is a lenticular zone aphasia. Practically, conduction aphasia is usually mixed with a visual or auditory aphasia. Thus we have the following groups of aphasia : Clinical Type Anatomical Seat 1. Pure motor aphasia. Fronto-cortical aphasia. Pure agraphia. Fronto-cortical aphasia. 2. Pure alexia. Occipital or parieto-occipital aphasia. Apraxia. 3. Auditory aphasia. Anomic aphasia. Tempo ro-parietal aphasia. (Sensory aphasia of Wernicke). 4. Mixed and conduction aphasias. Lenticular and fronto-lenticular aphasia. MALFORMATIONS OF THE BRAIN AND ITS ENVELOPES Congenital malformations of the brain are of little practical importance, for in most cases the monsters cannot live and in all cases they are better dead. I shall simply give a brief enumeration of the important forms. DISEASES OF THE BEAIN AND ITS MEMBRANES 375 I Anencephaly. Abnormalities I Micrencephaly and microcephaly. of the brain. | Porencephaly. [ Absences or malformations of parts, e.g., cyclopia. Acrania. Meningocele. 1 I Encephalocele. its envelopes. __ , , , , [ Hydrencephalocele. Abnormalities of brain and Anencephaly is always present with acrania. In anencephaly the cerebellum and part of the basal ganglia may be present. In such case the child can live a short time. Micrencephaly and Microcephaly. — Micrencephaly is a condition in which the brain is only partially developed. If, as is usually the case, the cranium is also ab- normally small, it is called microcephaly. It is due, probably, in all cases to an in- herent defect in the growth of the brain. Virchow has asserted, however, that there is a form in which the abnormality is caused by a premature growing together of the cranial bones, a micrencephaly being a result of the mechanical condition. An adult cranium whose great circumference measures less than 43 cm. will contain a micren- cephalic brain. The normal minimum weight of the adult brain is 960 grams for man and 880 for woman. It should bear the ratio to the body at birth of 14 per cent., and of 2.37 per cent, in adult life (Vierordt). Porencephaly is often an artificial condition. It will be described later. In cyclopia there is an undivided anterior cerebral vesicle; the orbits form a con- tinuous cavity with a single rudimentary eye. Meningocele is a hernia of the brain membranes, arachnoid, and dura mater through a cleft in the skull. In encephalocele the brain also protrudes. Both these forms occur usually in the occipital region and almo«t invariably in the median line. In hydrencephalocele there is a sac with fluid contents. DISEASES OF THE MEMBRANES OF THE BRAIN The diseases to be considered under this head are anaemia and hyper- semia, inflammation of the dura mater or pachymeningitis and inflamma- tion of the pia mater or leptomeningitis. Anaemia and Hyperaemia of the Membranes of the Brain. — ^Ansemia of the membranes of the brain is a condition that cannot be separated from anaemia of the brain substance, and will be considered in connection with it. Hyperaemia of the brain membrane, so far as it relates to hyperaemia of the pia mater, must also be considered in connection with hyperaemia of the brain tissue. Dural hyperaemia, or congestion of the dura mater, is a condition which occurs as the result of injuries, sunstroke, and of cer- tain infective poisons, especially that of syphilis. The symptoms are those of pachymeningitis of the slight grade, and will be described under that head. They consist mainly of pain, occasional attacks of vertigo, and sensations of fullness about the head. The treatment is that for the beginning stages of a meningitis. Inflammation of the Dura Mater or Pachymeningitis Externa. — It has been the custom to describe two forms of pachymeningitis, the ex- 376 DISEASES or the neevous system ternal and the internal. Internal pachymeningitis, or hsematoma of the dura mater, is properly a hemorrhagic disorder, and is described under the head of Dural Hemorrhages. A true inflammation confined to the internal surface of the dura alone is of extremely rare occurrence. Pachymeningitis externa is a disease that involves, at first at least, the outer surface of the dura, and is usually of surgical origin and interest. Etiology. — Accidents, injuries, osteitis, caries of the petrous bone in mastoid disease, of the ethmoid bone in ozsena, necrosis, syphilis and ery- sipelas are the usual causes. The symptoms are local headache, fever, delirium, sometimes even convulsions and paralysis. In the severe cases the disease has usually extended and involved the pia. Pus is generally formed, and burrows between the bone and dura. The disease is recognized mainly by the discovery of the local cause. The course is acute or subacute. The treatment is a surgical one. Inflammations of the Pia Mater. — Cerebrospinal Meningitis. — In- flammation of the pia mater has the following types: (1) Acute puru- lent meningitis due to various forms of pyogenic organisms other than the meningococcus; (2) epidemic cerebrospinal meningitis due to a specific meningococcus infection; (3) tuberculous meningitis; (4) serous men- ingitis and (5) syphilitic meningitis. Most of all these forms of meningitis may be either acute or chronic the chronic form being usually simply a sequela of the acute. ACUTE PURULENT CEREBROSPINAL MENINGITIS Etiology. — Acute leptomeningitis is due to an infective process. The infecting organism is usually a streptococcus, pneumococcus, or staphylococcus. The meningitis caused by the meningococcus is of a special type described later. The streptococcus is the most frequent in- fecting organism. The infection is almost always secondary to a focal lesion or general infection. The commonest focal lesions are disease of the ear, and of the nose and accessory sinuses. Fractures of the skull and other wounds of the head, or spine may become infected and lead to meningitis. Over-doses of sal- varsan introduced into the spinal canal may cause by chemical irritation a meningitis. Brain abscess, and infected sinus thrombosis may be causes. Just now it is stated that purulent meningitis is seen most often by obstetricians from sepsis and by otologists and rhinologists. Pneumonia is the most frequent infective disease in which the pyogenic organisms are carried by the blood. After this come erysipelas, sepsis, scarlet fever, more rarely endocarditis, empyema, rheumatism, measles, typhoid fever and mumps. Alcoholism and a generally lowered state of vitality from malnutrition predispose to the infection. Insolation can DISEASES OF THE BRAIN AND ITS MEMBRANES 377 of itself not cause it. The disease is more frequent in males, and is dis- tributed through all ages of life, though it occurs oftener in the young. Symptoms.^ — ^The symptoms in the various types differ somewhat, but have a general similarity. They are to be broadl}^ grouped into the prodromal, the irritative, the depressive, and the paralytic stages. Prodromal symptoms are shorter and less marked in simple meningitis than in tubercular. They are really the symptoms of a general infection. The patient suffers from malaise, languor, headache, vertigo, irrita- bility, loss of appetite and vomiting. Of these sj'-mptoms headache is the most notable. [The description of the symptoms of epidemic cerebrospinal menin- gitis given later answers for the common purulent form, except for certain variant types.] Course and Duration.^ — The disease may begin suddenly, and the patient pass at once into the comatose state, dying in a few days. Usually the process lasts one or two weeks; it may be prolonged for several weeks. The prognosis is very grave, but it is less serious than in tuberculous meningitis and more serious than in the cerebrospinal form. The diagnosis is based on the presence of an exciting cause, such as disease of the ear or nose, trauma, infective fevers, and upon the presence of the symptoms given. It is usually easily recognized, the main difficulty being to distinguish it from other forms of meningitis. To determine this, it is advisable to make a lumbar puncture, draw off the fluid, and examine it for bacteria and the presence of cellular elements. (See Epidemic Cerebrospinal Meningitis.) Pathology. — The disease is a fibro-purulent or purulent inflammation. It involves usually the base more than the convexity, but the reverse may happen. The ventricles are often involved and may be independently inflamed. There are descriptions, therefore, of simple basilar meningitis, meningitis of the convexity, and ventricular meningitis or ependymitis. The inflammatory deposits are most conspicuous along the course of the Sylvian fissure and the vessels branching from it, about the optic chiasm, and at the posterior and under surface of the cerebellum and the sides of the pons. It may lie only in the subarachnoid cavity, but usually the arachnoid and sometimes the dura are implicated. There is increase of fluid in the ventricles and arachnoid cavities, and this fluid may be tur- bid. The surface of the ventricles may show an inflammatory process The micro-organisms found in meningitis are as already stated the pneumococcus, the streptococcus, the staphyloccus and a bacillus re- sembling that of typhoid fever. Still others have been described, and the process may be a mixed infection. Treatment. — Prophylaxis is the most important measure, as there is no specific treatment. Chronic disease of the ear and nasal sinuses should 378 DISEASES OF THE NERVOUS SYSTEM be attended to, and injuries of the skull treated with the strictest regard to antisepsis. When the disease is developed, the indications are to treat the original focus of infection (ear, etc.) to open the skull and decompress and drain in certain cases ; to combat the general sepsis. The treatment is therefore often a surgical one. To combat the infection there are im- munizing sera from which not much has been accomplished. Flushing the blood with intravenous injections of saline tranfusions, epidermo- clysis and proctoclysis may be tried. Urotropin and formaldehyde have not proved useful. Recently the use of watery solutions of sulphate of magnesium have been strongly recommended. EPIDEMIC CEREBROSPINAL MENINGITIS {Spotted Fever) Cerebrospinal meningitis occurs as an epidemic and endemic disease caused by the diplococcus intracellularis meningitis. It is a disease which has invaded practically all of Europe and has occurred in the East and in Africa. The most extensive epidemics have occurred in the United States, affecting all the states and spreading to Canada, but not to the countries in the South. The disease occurs sporadically in the large cities of the United States during the winter. The epidemic occurs periodically at irregular times. The disease occurs at all ages but children are the most affected. In New York City about two-thirds of the cases were in children under ten years of age, but about 16 per cent, occurred in persons over twenty and it may attack persons as old as fifty. The sexes are about equally affected. Race and nationality seem to have no particular influence although in the South the negroes are less affected than the whites. The disease occurs in the winter months, beginning sometimes in late October and ending in May but sometimes not beginning until the first month of the spring. Unusually cold weather seems to be associated with the height of the epidemic. The disease is most prevalent among poor and in over-crowded dwell- ings. In New York three-fourths of the patients lived in tenement houses. The disease is very shghtly infectious. Fatigue and ill health pre- dispose somewhat to its development. The infection occurs through the nose and throat, and nasopharyngitis predisposes to the entrance of the organism. The organism is carried by healthy individuals and the disease is propagated to a great extent through these carriers. It has been estimated that during an epidemic there are ten to thirty healthy carriers in proportion to each single person who actually has the disease. The nasal and oral secretions of the healthy carriers give positive cultures of meningococci. Usually the organism spontaneously disappears DISEASES OF THE BRAIN AND ITS MEMBRANES 379 from the nasopharynx of the carrier in a few clays, but there are in- stances in which a healthy person may harbor the coccus for one or more months. The invasion of the body by the meningococcus is probably accom- plished by the passage of the organisms from the nasopharynx into the blood stream, resulting in a primary bactersemia, and later the bacteria become localized in the meninges. It is possible that the meningococcus passes directly into the cranial cavity from the nose and throat by means of the lymphatics, but the weight of evidence is against the view. Pathology. — An exudation of serum, fibrin, and pus covers the pia of the brain and cord and infiltrates its meshes. There is some involve- ment of the superficial layers of the cortex in the inflammatory process. The blood-vessels of the meninges are engorged and surrounded by a cell infiltration. The inflammation extends along the sheaths of the cranial nerves and also into the ventricles. The ventricles are dis- tended with a mixture of cerebrospinal fluid and exudate, and their walls are red and oedematous. The cerebrospinal fluid is increased in quantity. In chronic cases the pia arachnoid may show some cicatricial thickening. The organism, a Gram-negative, biscuit-shaped diplococcus, is to be found in the exudate and in the cerebrospinal fluid, where it is seen in the polynuclear leukocytes and also free. It may be obtained by culture from the nasopharynx and from the blood. Symptoms. — The disease begins as a constitutional infection produced by the passage of the organism into the general system and this is fol- lowed by local symptoms produced by the localization of the organism in the meninges. The period of incubation— i.e., the time between the invasion and the development of the constitutional symptoms, probably ranges between one and five days. The constitutional symptoms corresponding to the period of simple bactersemia are very much like those of influenza. The disease comes on acutely with chilly feelings or a severe chill. There is fever, throbbing headache, restlessness, irritability, and vomiting. The mind is clear but the patient is over-sensitive. Often the pupils are dilated and slug- gish, and there may be photophobia. A little early stiffness of the neck and tenderness at the angle of the jaw may be present. Herpes and a petechial eruption are occasionally seen. Sometimes there is irregularity in breathing, particularly while the patient is asleep. Deep and un- controllable sighing is quite common. The temperature varies; some- times it is very high, and sometimes low. In young children there may be a little bulging of the fontanelles, and in adults Macewen's sign can often be obtained. It consists in a dull tympanitic note brought out by percussing with the finger over the fronto-parietal region of the skull. It' is most easily obtained if the patient's head is lifted a little and bent 380 DISEASES OF THE NERVOUS SYSTEM slightly toward the side to be examined. The sign indicates an excessive amount of fluid in the ventricles. Blood examination at this stage usually shows a moderate leukocytosis with relative polynucleosis. Lumbar puncture reveals an increase of pressure. The fluid is usually perfectly clear, however, although a few free meningococci are sometimes to be found. These symptoms usually last for twenty-four to thirty-six hours, when the meningeal symptoms make their appearance. In some cases the pre-meningitic symptoms are entirely absent, and in others a history of them is obtained only by careful questioning of the family. The stage of active meningitis begins with an increase of all the fore- going symptoms. The headache becomes terrific and the patient com- plains of it bitterly. A number of general convulsions may occur, par- ticularly in children. Sometimes consciousness is suddenly lost. There is extreme restlessness and frequently delirium. Vomiting is frequent and severe, and in many cases uncontrollable. Usually it is independent of nausea, and it may be projectile. The patient suffers severe pain in the back of the neck, along the spine, and in the legs. The neck becomes stiff, the head may be retracted, and in some cases there is rigidity of the spine or opisthotonos. Photophobia is often very marked. The patient is greatly prostrated. Active delirium and restlessness alternate with deep stupor, in which, however, the patient usually shows some response if he is disturbed. There may, however, be periods in which the mind is clear. The patient's face bears an anxious expression and often is cy- anosed. The rigidity of the neck may be so great that with a hand placed under the patients head one can lift his body from the bed with- out there being any appreciable bending of the neck. An attempt to move the neck in any direction is very painful, and there is tenderness to pressure on the posterior neck muscles. The pupils are generally dilated, and react to light sluggishly or not at all. Often there is some strabismus, which may be transitory. Other cranial nerve palsies may occur, and there may be clonic or tonic spasms of the extremities. In young children there is pronounced bulging of the fontanelles. Tache cerebral is readily obtained. Kernig's sign is early and constant (Figs. 195, 198). This test is made by flexing the patient's thigh to a right angle on the abdomen and then attempting to extend the leg on the thigh. This attempt, in meningitis cases, is resisted by a sudden spasm of the ham- string muscles and the patient cries out in pain. The abdominal reflexes are often missing. The tendon jerks frequently are absent, but they may be exaggerated. Rarely there is a dorsal flexor response of the toe. Brudzinski's signs of meningitis may be present. In the first sign, if one of the patient's thighs is flexed upon the abdomen as far as possible (the leg being flexed on the thigh), the patient then draws up the other into a similar position. In the second, with one leg^and thigh extended and DISEASES OF THE BRAIN AND ITS MEMBRANES 381 the other flexed as before, the flexed Hmb is lowered to the extended position by the examiner, whereupon the patient draws up the other limb to a position of flexion on the abdomen. The ''neck sign" of De Lepinay can usually be obtained. It consists of a flexion of the knees and thighs which occurs when one attempts to bend the patient's neck forward. Most cases of meningitis show some respiratory irregularity. Connor and Stilmann found Cheyne-Stokes breathing in 53 per cent, of their cases and Blot's breathing in 27 per cent. In only 5 per cent, of all their cases did they fail to find some sort of irregularity of breathing. Blot's breathing ("cerebral breathing") is thought to be typical of meningitis as distinguished from other cerebral conditions causing disturbances of breathing. It is characterized by periods of apnoea of varjdng length and occurring at irregular intervals (as distinguished from the rhythmical Fig. 198. — The Kernig sign. periods of apncea in Cheyne-Stokes breathing which alternate with periods of gradually increasing and gradually decreasing respiratory movement), constant irregularity in the rhythm and the force of individual respira- tions, and the frequent occurrence of deep sighing respirations. The temperature in meningitis is usually very irregular. It may be extremely high (106° F.), or it may be little above normal. A septic tem- perature may occur, or the fever may be high continuously. The pulse rate is also irregular. It may be very slow — 50 or less — -but more often it is rapid. The pulse may be intermittent or show frequent changes in rate. Generalized skin eruptions occur in only a small percentage of cases. Petechise if present are usually profuse, and are distributed all over the trunk and extremities. Purpuric eruptions do occur, but are less common. Herpes, however, is a skin condition very commonly found. 382 DISEASES OF THE NERVOUS SYSTEM Lumbar puncture shows that the intra-spinal pressure is abnormally increased. The fluid is turbid, globulin and albumin tests are strongly positive, and cells are numerous, most of them being polynuclears — • 100 per cent, in some cases. Stained smears from the sediment obtained by centrifuging show the meningococcus^ — in varying numbers — i.e., Gram-negative, biscuit-shaped cliplococci, both intra- and extra-cellular. Cultures show a growth of the meningococcus after eighteen to twenty- four hours' incubation. Elser and Huntoon were able to make a posi- tive diagnosis from the examination of the spinal fluid in 92.5 per cent, of over 200 cases examined. No altogether satisfactory serological tests for meningitis have yet been devised. Blood examination shows a leukocytosis of between 20,000 and 45,000. The polynuclears are markedly increased, there is a relative decrease in the large mononuclears, and in very young children a lymphocytosis had been observed in rare instances. The meningococcus can be obtained from the blood by culture in a certain number of cases. Elser had positive results in 26.5 per cent, of the cases examined. Course and Prognosis.- — The disease may run a short and malignant course killing the person in a few hours or one or two days. In moderate cases it lasts two or three weeks. A large number of different varieties of the disease have been described, such as the abortive form, the ful- minating form, and the typhoid form. Affections of the joints are the most frequent complications of the disease. There may also occur septic pneumonia, pyelitis, ulcerative endocarditis, pericarditis, neuritis, and phlebitis. In some cases the disease leaves a chronic hydrocephalus, and there may be some permanent mental impairment. Permanent paralysis of some of the cranial nerves, or, much more rarely, of one or more of the extremities may occur. Deafness is quite common, and blindness and other eye troubles sometimes are observed. A certain type of case lapses into a state of chronic meningitis, which runs a course of a few weeks' to two or three months' duration, and usually terminates fatally. It is characterized by extreme emaciation and severe cerebral pressure symptoms. The temperature is extremely irregular. It may be normal or subnormal, or, after having been low for a considerable time, suddenly rises and remain high and irregular. Chills may occur irregularly. The emaciation is most remarkable. Though many of these patients take large quantities of food and appear to digest it properly, they become rapidly reduced to skeletons, the like of which is hardly to be seen in any other disease. The patient is usually drowsy and stupid but there may be active delirium. Complete coma occurs in the final stages. The patient's eyes are wide open and the face ex- pressionless. Spasms and convulsions are common. Vomiting is fre- quent and the bowels are constipated. Macewen's sign or bulging of DISEASES OF THE BRAIN AND ITS MEMBRANES 383 the fontanelles may be present. Rigidity of the neck and Kernig's sign usually continue present, but not always so. The respirations are slow and irregular, and the pulse rapid, weak, and intermittent. Strabismus or some cranial nerve paralysis is usually present. The tendon jerks are absent or diminished. Incontinence of urine and faeces is practically invariable. Bed-sores develop readily. Another form of chronic meningitis, the so-called posterior basic meningitis is distinguished by the fact that there occurs a shutting off of the communication between the ventricles and the sub-arachnoid space. The inflammatory process thus becomes encapsulated in the ventricles, and whatever inflammation was going on in the subarachnoid space usually subsides so that the fluid obtained by lumbar puncture may be sterile. More commonly, no fluid at all is to be obtained. Fig. 199. — Cerebrospinal meningitis, terminal stage. The condition of posterior basic meningitis may develop at the outset of the infection, but more commonly it appears in the course of a sub- acute or ordinary chronic meningitis. The symptoms are those of a rather mild sepsis with marked cerebral pressure. The rigidity of the neck and retraction of the head are ex- treme, and a high degree of opisthotonos is the rule. The patient is apa- thetic and stuporous, and convulsions are very common. Usually the patient lies motionless with the extremities rigid in tonic spasm. The "hydrocephalic cry" which is altered at intervals without relation to pain or other stimulation is a common symptom. The eyes are wide open, the lids retracted, the pupils do not react to light, and there may be some exophthalmos. Various palsies are present. The patient may remain in this condition for several weeks. There is practically always a fatal termination. The prognosis of the usual form of meningitis is always grave. The 384 DISEASES OF THE NERVOUS SYSTEM mortality in different epidemics has varied from 20 to 80 per cent. Only a small percentage of those cases that become chronic recover. Sophian thinks that the development of Biot's breathing in either acute or chronic cases is of prognostic value indicating almost without exception a fatal termination. Diagnosis.— The differential diagnosis of epidemic cerebrospinal meningitis is to be made from tuberculous meningitis, from meningitis caused by strepto- and staphylococci, the pneumococcus, and other pyogenic organisms; from poliomyelitis with meningeal symptoms, and from the so-called meningismus which occurs in various infectious diseases. Meningitis due to streptococci, pneumococci, and similar organisms presents the same clinical picture that is found in epidemic meningitis. The history of an epidemic, on the one hand, or of some preceding focus of infection, as for instance otitis media or broncho-pneumonia on the other, may help toward making a diagnosis. Ordinarily, a positive differ- ential diagnosis can be made only through the results of the examination of the cerebrospinal fluid. In tuberculous meningitis as a rule the children are younger — two years or less of age. A family history of tuberculosis may be obtained, or some family form of tuberculosis can be made out. The child is apathetic and stuporous instead of irritable and restless. Cranial nerve palsies occur early. Rigidity of the neck and Kernig's sign are usually moderate and the temperature at first is not very high — 101 to 102. There is no eruption. The course is acute and death occurs in two to four weeks after the beginning of the disease. Lumbar puncture gives an abundant fluid, which usually is clear with white specks of fibrin floating in it. If the fluid is allowed to stand for a time a network of fibrin forms. There is a lymphocytosis instead of a polynucleosis. Care- ful search may reveal the presence of the tubercle bacilli, and cultures fail to show the diplococcus intracellularis or other pyogenic organisms. In the cerebral type of poliomyelitis the patient is apathetic and stuporous, but he can be roused and when this is done his mind is quite clear and he can answer questions intelligently. Though there is rest- lessness and irritability these symptoms are much less marked than in meningitis. Rigidity of the neck and Kernig's sign, if present, are not very marked. The profound disturbances of respiration and pulse which occur in meningitis are absent in poliomyelitis. The paralyses in polio- myelitis occur early and are comparatively extensive, while in meningitis the palsies occur later and ordinarily are less extensive. Usually in poliomyelitis the constitutional and the cerebral symptoms rapidly im- prove as soon as the paralyses occur, while in meningitis the occurrence of the palsies is ordinarily attended by an exacerbation of the constitu- tional and cerebral symptoms. The fact that poliomyelitis is a summer DISEASES or THE BRAIN AND ITS MEMBRANES 385 disease and meningitis a winter one should not be forgotten in making a diagnosis. The fluid obtained by lumbar puncture in poliomyelitis is clear with white flecks floating in it, and, in fact, appears altogether like that ob- tained in tuberculous meningitis. There is, however, less tendency for it to form a fibrin network on standing, and, if a network does form, it is less dense than in the tubercular condition. The fluid is usually less abundant and under less pressure. Globulin tests are positive, but less strongly so than in meningitis. The cells are increased but not to the ex- tent to be found in meningitis and there is usually a lymphocytosis. In short the fluid findings of poliomyelitis are much more likely to be con- fused with those of tuberculous meningitis than of the epidemic variety. Bacteriological examinations of the fluid are negative for tubercle bacilli and other organisms, and the inoculation of guinea-pigs as a test for tuberculosis is negative. The diagnosis of meningitis from meningismus is usually not diffi- cult. The presence of the primary disorder causing the meningismus can usually be made out. Unless there is delirium the patient is clear and intelligent and, though irritable, does not show the same crying and com- plaining that occurs in meningitis. Kernig's sign, bulging of the fon- tanelles, and Macewen's sign are moderate or absent. Lumbar puncture settles the question if the diagnosis cannot be made without it. Treatment. — Prophylaxis. — Cases of epidemic meningitis should be quarantined until cultures from the nasal and pharyngeal secretions are negative for the meningococcus. Healthy people who come in contact with these patients should be examined in the same way, and if their cultures are positive they should be quarantined also, and directed to use a disinfecting spray of hydrogen peroxide, preceded by salt solution, until negative cultures are obtained, thus doing away with the danger of their infecting others or of becoming infected themselves. The general treatynent is directed toward supporting the patient and combating the toxaemia. Sustaining food should be given and such stimu- lants as digitalis when indicated. Bromides, chloral and morphine are used as sedatives. Ice bags to the head and cool bathing for high tem- peratures are also employed. Lumbar puncture is employed for the purpose of diminishing pressure by draining off the fluid. The specific treatment consists in the administration of the Flexner serum. A lumbar puncture is done and the cerebrospinal fluid allowed to flow until it comes from the needle at the rate of one drop every three to five seconds, which indicates roughly that about the normal degree of pressure has been attained. Then from 5 to 40 cc. of the Flexner serum, depending on the age of the patient, is slowly and carefully in- jected into the spinal canal through the puncture needle. The safest 25 386 DISEASES OF THE NERVOUS SYSTEM method of injecting the serum is that of allowing it to flow into the spinal canal by gravity, using instead of a syringe a funnel attached to a twelve inch tube, and watching the blood pressure meanwhile as an index of when the flow should be stopped. A fall in blood pressure of 10 mm. of mercury indicates that enough serum has been injected (Sophian). The injections are repeated every day for three or four days and then less often. The best guide to the proper frequency of the injections is the examination of stained smears from the sediment from the fluid. As the patient responds to treatment the number of the cocci decreases and those that are found are intra-cellular. Numerous and extra-cellular organisms indicate that an injection is required on the following day. The use of this serum has given very satisfactory results. In different epidemics where a mortality of from 50 to 90 per cent, occurred in those cases not treated with serum, the mortality was reduced to between 13 and 50 per cent, by its use. H. W. Frink, M. D. TUBERCULOUS MENINGITIS This is a form of meningitis due to infection with the bacillus tuber- culosis. It differs pathologically from other forms in the character of the infective organism ; anatomically, in the fact that the inflammation is usually and chiefly basilar and never purely purulent; etiologically, in that it chiefly affects young children; and symptomatologically, in the presence of prodromata and a more irregular course. Etiology. — Tuberculous meningitis occurs chiefly between the ages of two and ten, oftenest between two and five years, sometimes in in- fancy, rarely in adult life, very rarely after the age of fifty. Males are rather more subject to it. A hereditary history of phthisis, bad hygienic surroundings, and the presence of tuberculosis elsewhere in the body predispose to it. Tuberculous milk is probably one source of the in- fection. The eruptive fevers, especially measles, blows on the head, and great emotional excitement appear to act as exciting causes. Symptoms. — The child in the prodromal stage becomes peevish, irri- table, loses interest in its play, the appetite is gone, and there may be explosive vomiting. Sleep is restless and disturbed. Paroxysms of severe headache occur. After two or three weeks, or sooner, these symptoms increase; the child becomes dull, moody, weak, and takes to his bed. He lies there with clouded consciousness, dozing at intervals; often the cheeks are ruddier than usual. Occasionally he utters a piercing cry as if in great pain. He is easily disturbed by noises, and shows himself generally hypersesthetic. He sighs deeply, picks his lips or the bedclothes. The neck is now felt to be rather stiff; the reflexes are in- creased; and the abdomen retracted or perhaps distended. The tem- perature runs an irregular course but is not high. The pulse is slowed DISEASES OF THE BRAIN AND ITS MEMBRANES 387 and irregular, sometimes becoming very fast on slight exertion. The respiration is disturbed and sighing. The pupils are often uneven, usually contracted, and do not react well to light. The patient finally passes into deep coma, with rise of temperature. The picture is now one of the last stages of meningitis, and death occurs in about three weeks. The special symptoms and signs of meningitis of other types are present in this later stage. The condition of the cerebrospinal fluid is described elsewhere. (See Epidemic Cerebrospinal Meningitis). Pathological Anatomy. — In rapidly fatal cases, with severe symptoms, there may be only an intense congestion of the brain with numerous miliary tubercles in the pia mater at the base and over the convexity. Here we must assume that a bacillary toxin causes the symptoms. In most cases there are decided deposits of tubercles at the base, with fibrinous inflammatory deposits about the optic chiasm, along the fissure of Sylvius, at the sides of the pons, and elsewhere. Miliary tubercles are seen scattered over the convexity and in the choroid and ventricles. They are generally found in the spinal membranes also, especially over the Cauda equina. The tubercles lie beneath the pia surrounding the small vessels. They may coalesce into large tuberculous nodules. There is usually an increase in the arachnoid fluid, and in most cases an increase in the ventricular fluid. Somewhat rarely there are very great distention of the ventricles and compression of the convolutions. Small spots of softening may be seen from obliteration of the vessels by the tubercles. The bacillus tuberculosis is found in the tuberculous nodules. Diagnosis. — As regards the form of the disease, this is based on the hereditary history, the age, the existence of tuberculosis of the lungs or other organs, and the peculiar prodromata of the disease. Occasionally tubercles can be seen on the choroid. Lumbar puncture of the spinal canal with withdrawal of fluid and its examination furnishes the most certain method of diagnosis. (See diagnosis of meningitis.) Prognosis. — This is usually absolutely bad, yet post-mortem observa- tion of patients dying with practically no inflammatory change makes it seem possible that the disease might be checked, and a good many cases are reported in which this apparently has been done. Some of these are, however, probably cases of hereditary syphilis. Treatment.- — So far as is now known, this is not different from that given under the head of meningitis elsewhere. HYDROCEPHALUS Hydrocephalus is a condition in which there is an excessive amount of fluid in the ventricles and subarachnoid or subdural cavity of the brain. When this fluid is in the ventricles it is called internal hydro- cephalus, and practically most hydrocephalus is internal. In external 388 DISEASES OF THE NERVOUS SYSTEM hydrocephalus the fluid is in the subarachnoid or subdural spaces. Ex- ternal and internal hydrocephalus may coexist. Hydrocephalus is a symptom of several morbid conditions, but in general we may divide it into the chronic primary and secondary symptomatic forms. Chronic Primary Hydrocephalus. — This is a disease of infancy, char- acterized by a gradual enlargement of the head, with mental deficiency and symptoms of brain irritation caused by an accumulation of fluid in the ventricles of the brain. Chronic hydrocephalus is not an inflammatory process, but one due to mechanical causes or to defects in structure or development. The fluid always accumulates in the ventricles of the brain; hence chronic hydro- cephalus is always internal. The so-called external forms of hydro- cephalus are inflammatory or else are secondary to meningeal hemorrhage or brain atrophy. Etiology. — Four out of five cases begin at birth or within the first six months of life. Congenital anomaly, intra-uterine meningitis, syphilis, 'Si^- • • \ •■:"*'" 1-^-W ^ ^ ~1^i ^^^^^^^^^^^^^^^^m^^^m Fig. 200. — Chronic hydrocephalus. alcoholism, lead poisoning in the parents, and some unknown family taint predispose to the disease. Malnutrition and rickets are also factors. Symptoms. — The head may be so large at birth that instrumental help is needed. More often the parents notice a gradual increase in the size of the child's head, beginning soon after birth. The forehead bulges, the occiput stands out, the fontanelles and sutures widen, and pressure shows evidence of fluctuation. Meanwhile the face does not grow much and the result is to give triangular shape to the head. It may measure twenty-four, twenty-seven and one-half (Minot), thirty-two (Bright), and even forty-three inches (Klein) in diameter. These extreme measure- ments are reached only after one or two years. With this abnormal growth of the head, mental and physical symptoms appear. The infant is restless and irritable; its appetite may be good, but the general nutri- tion is poor and its bodily growth is retarded. The mind does not de- velop; usually it does not or cannot learn to walk. It may be unable DISEASES OF THE BRAIN AND ITS MEMBRANES 389 to support the weight of its head. There is strabismus and sometimes optic atrophy. The pressure of the dropsy thins the orbital bones and forces down the axis of the eyeballs (see Fig. 200). Vomiting, coma, and con- vulsions eventually appear, and the child usually dies of exhaustion or some intercurrent disease in two or three years. In some cases the trouble is less serious, it ceases to progress, the bones solidify, and the child grows up with good intelligence. The hydrocephalus which is associated with the brain atrophy of insanity and old age (hydrocephalus ex vacuo) or with general dropsical conditions has no kinship with the process we are now describing. Pathology. — The disease is due to the gradual accumulation of a serous fluid in the ventricles of the brain. The cause of this is an in- flammatory or developmental obliteration of the aqueduct of Sylvius or the foramen of Magendie and the adjacent lateral foramina of Luschka. This prevents the escape of the ventricular fluid into the subarachnoid cavity. Contributing factors are congenital or acquired defect in the villous processes of the pia arachnoid, by which the fluid normally is able to pass into the venous circulation; and a rachitic and easily yielding skull. Hydrocephalus is not, therefore, an inflammatory but a mechan- ical process. The lateral ventricles when the cerebrospinal fluid is secreted are principally and often solely affected, and these are so distended as to press out their cerebral walls, flattening the convolutions and turning them into a thin shell often less than a quarter of an inch in thickness. Sometimes only one lateral ventricle, and in rare cases only the fourth ventricle, is affected by the dropsy. The diagnosis has to be made from rickets and an acute inflammatory process. In rickets the head is square, the fontanelle does not bulge, the enlargement is less, and there are signs of the disease in the bones elsewhere. Prognosis. — The congenital cases usually result fatally in a few months, or at least before the third year. Those developing in infancy may persist for four to six years; and in mild cases the disease ceases to progress and a fairly healthy adult life is reached. Treatment. — A great many measures have been recommended, but there is no unanimity about them. The inunction of mercury and the administration of iodide of potassium combined with intravenous in- jections of salvarsan are indicated if tests show the presence of syphilis. Tapping the ventricles in various ways has been recommended, but I have seen no good results. Tapping the spinal cord every three days and later every week is said to be of some service. Secondary or acquired hydrocephalus is usually caused by an attack of acute meningitis, or by tumors. It may also be due to ependymal in- flammation and to obstruction of the veins of Galen by thrombosis or 390 DISEASES OF THE NERVOUS SYSTEM other mechanical cause. In acute meningitis this is a frequent condition and cause of death. In other cases infants survive the meningitis and, with a growing head, develop symptoms later of the hydrocephalus. In the acute conditions the symptoms may run a varying course owing to the partial and irregular escape of the fluid. The patient, if the disease progresses, becomes stupid and comatose and develops paraplegia and rigidity of the body, retraction of the head and trunk, and great emacia- tion. The treatment is the same as that for the primary form and con- sists in puncture of the ventricle or corpus callosum and withdrawal of fluid or a decompressive operation (Gushing). The results of surgical interference have so far not been satisfactory. ALCOHOLIC "MENINGITIS" (SEROUS MENINGITIS, "WET-BRAIN") Alcoholic meningitis is a clinical term used to indicate the peculiar group of cerebral symptoms which is seen in persons who have succumbed to the effects of prolonged alcoholic intoxication. The disease is not a true meningitis but an acute toxaemia of the brain with serous effusion; but it may be called, for the purpose of convenience, an alcoholic serous meningitis. It should not be confounded with the acute serous menin- gitis following infections and trauma. Etiology. — -The disease occurs oftenest in men simply because of the more frequent indulgence of the male sex in alcohol. It rarely develops until a person has been drinking eight or ten years, and, consequently, affects people oftenest between the ages of thirty and forty. The excit- ing cause is commonly alcohol and in this country whiskey or what are known as ''hard drinks," but beer and ale will accomplish the same re- sult. I have rarely seen the disease in wine drinkers. The persistent use of morphine, cocaine, and chloral may lead to much the same con- dition. The exciting cause is usually a continuous drinking bout of two or three weeks, ending in delirium tremens. The delirium tremens, however, is not by any means always present. The patient may pass from a condition of prolonged intoxication into the condition of alcoholic meningitis or "wet-brain." Symptoms. — In case delirium tremens has occurred, the patient after two or three days of prolonged delirious excitement gradually sinks into a semicoma. This is accompaDied by a muttering delirium. The patient is sufficiently conscious to have flitting delusions and hallucina- tions of sight and hearing. At this time he is able to drink and take food; the pulse is rather rapid, the temperature is usually normal or may be raised one-half or one degree. The skin is hypersesthetic and pressure upon the muscles of the arms or legs or abdomen causes pain. The pupils are usually rather small. Often at this time conjunctivitis and keratitis may appear. After a few days the patient's stupor becomes deeper and DISEASES OF THE BRAJN AND ITS MEMBRANES 391 he can be aroused only with difficulty. The arms and legs are now somewhat stiff, the reflexes are exaggerated, the neck is somewhat stiff and slightly retracted, and attempts to move it cause expressions of pain. The Kernig sign is present. The abdomen is retracted and the skin and muscles are still very hyper aesthetic. The lids are closed. The pupils are small and do not react well to light. The tongue is coated and usually dry, and urine and faeces may be passed involuntarily. The patient may linger this way for several days more. The pulse becomes rapid and feeble, the extremities are stiff and cold. The skin is dry and loses its elasticity, so that when pulled up between the fingers it stays in folds. "Putty skin " is a good name for this. The coma deepens, the temperature may rise to 103° or 104°, and symptoms of pneumonia may appear as the scene closes. On the other hand in some cases the patient does not pass into the worst stage, the mind becomes clearer, the hypersesthesia lessens, food is taken better, and the bowels are moved voluntarily. Improve- ment continues and in three or four weeks the convalescence begins. Pathological Anatomy. — I have made autopsies and careful micro- scopical examinations in over twenty cases of the character just described. In nearly all the brain is found to be somewhat pale, the arachnoid con- tains two or three ounces of serous fluid, the subarachnoid space is satu- rated with fluid, and the ventricles are dilated. Sections through the brain sometimes show punctate hemorrhages and in rare cases spots of hemorrhagic extravasation are seen surrounded by softening. Oc- casionally the beginning of a suppurative cerebral meningitis will be seen. Microscopic examination shows in the uncomplicated cases that there is no true inflammatory process. There is often congestion, but not always; the commoner condition is an oedema of the brain tissue, the perivascular and pericellular spaces being dilated. The nerve-cells show various stages of degeneration, not pigmentary in character, however. The chromophilic granules are often unstained, or, if stained, have lost their true relations, and seem broken down. The cell outlines are irregu- lar; the nucleus lies near the periphery of the cell, and in some cases has broken out and escaped from it. Sometimes there is a large number of neuroglia cells in the pericellular spaces. The disease is un- doubtedly, primarily at least, a toxaemia not due directly to the influence of alcohol but to the poisons which have developed in the body as a result of the condition of inanition and the paralysis of the digestive function caused by the prolonged ingestion of alcohol and abstinence from food. The cell degeneration is more like that which is known as "degeneration from a distance," such as is seen in nerve-cells when the neuraxon is destroyed. It is a degeneration which affects especially the body of the cell and not so much the nucleus; hence the remarkable power of recovery from this condition which so many people show. 392 DISEASES OF THE NERVOUS SYSTEM The diagnosis of the disease is to be made from ordinary suppurative meningitis, from acute serous meningitis due to infection, and from acute encephalitis. In most cases the history of the patient is quite sufficient to estabhsh the diagnosis. The symptoms of themselves are almost identical with those of ordinary acute suppurative meningitis. The only distinctions which I have been able to observe are that in sup- purative meningitis there is more fever, there is less of the low delirium, hallucinations are rare, and there is an earlier and more profound coma. In other words, it is an acuter and more severe malady than alcoholic meningitis. The absence of convulsions and paralysis and the presence of hypersesthesia, rigidity, and contracted pupils, as well as the absence of pyrexia, are usually sufficient to distinguish the disorder from enceph- alitis or encephalitis complicated by alcoholic meningitis. Lumbar puncture will settle the diagnosis and often prognosis. The cerebro- spinal fluid is usually in excess and under great pressure; it is clear and colorless, unless there has been a superimposed infection as sometimes occurs, when evidence of inflammation will be found. The prognosis is bad when the disease has become well developed and when decided coma and rigidity have set in. A prognostic criterium which I have long used and which is fairly accurate is this : if the patient has not a very stiff neck and back he will get well. The treatment of the disorder should be instituted at the very be- ginning. If there are still any relics of the debauch, as shown in the condition of the stomach or intestinal tract, the stomach should be washed out and, at all events, a thorough purge should be given. The patient should then be fed most liberally with hot milk given every two hours; beef tea and an egg beaten up in milk may also be given, but the condition of practical starvation on the part of the patient should always be borne in mind. Stimulants in the shape of whiskej^ should not be administered if it is possible to avoid it, but strychnine in doses of one- sixtieth of a grain every two hours is often useful. An ice cap may be applied to the head and at times leeches or large blisters seem to be useful, applied to the back of the neck. The patient, however, should not be much depleted. Large doses of ergot have been recommended. When he becomes comatose it means that the ventricles and arachnoid cavities are becoming filled with water. At this time tapping the spinal cord may be tried. I have done this in a number of cases and have at times re- moved two or three ounces of fluid with some amelioration of the symptoms and never any bad results, but I have never seen it do any permanent good. CEREBRAL HYPERiEMIA AND AN.EMIA Active Cerebral Hyperaemia.- — The circulation of the blood in the brain is regulated mainly by the vasomotor splanchnic nerves, a rather DISEASES OF THE BEAIN AND ITS MEMBRANES 393 clumsy system by which a mass of blood is thrown into the abdominal cavity, or pressed out of it, thereby decreasing or increasing the cerebral flow. In addition to this, the cerebral blood-vessels have a nervous supply which probably controls the finer and the more local variations in calibre. The total amount of blood in the cranial cavity is always about the same. There can be no cerebral hypersemia, therefore, characterized by a large increase in the intra-cranial content of blood. But the flow of blood through the brain may be very much increased in rapidity; so that the amount of blood flowing through the brain in a given time is greater than the normal, or average. This physiological cerebral hypera^mia accompanies conditions of over-activity and over-stimulation of the brain, and is associated with increase in mental activity; but cerebral hypersemia is here a consequence or associated phenomenon rather than the cause, and there is no patho- logical condition which can be, strictly speaking, called "cerebral hy- persemia." Passive or venous cerebral hypercemia occurs when there is some block- ing of the venous current and damming back of the blood. Here the venous stasis causes an increase in intra-cranial pressure and an anaemia of the capillaries. The blood current is slowed-up and in addition to that, the proportion of venous blood to arterial is increased. The blood sup- ply is not of as good quality as normal, hence, in passive hypersemia of the brain there may be symptoms. They, however, are usually very much like those of cerebral ansemia. They consist in a sense of fullness of the head, headache, dizziness, sometimes faintness and there may] be dullness and drowsiness, irritability and depression, in fact, all such symptoms as are found in slowly acting, badly nourished or toxic brains. Cerebral anaemia is a more definite pathological condition. Acute ansemia of the brain occurs as the result of severe general hemorrhage, or of sudden intra-cranial pressure as in cerebral hemorrhage, and of acute serous effusion. It may be caused by a weak and diseased heart. It occurs from sudden dilatation and paralysis of the vaso-motor splanchnic area, and it probably occurs sometimes in cases of extreme fright. It is caused also by certain toxic conditions, as in the vaso-motor paralysis of chloroform poisoning. It may occur in conditions of impeded circula- tion of the lungs and in mechanical obstruction in the flow of blood in the neck. When the condition is of acute onset, there is dilatation of the pupils, respiration deepens and is irregular; there is dizziness, slowness of the heart beat and rise of arterial pressure. This is followed by a rapid heart beat, rise of arterial pressure and finally respiratory paralysis. In very acute ansemia, such as is caused by ligature of all the arteries carrying blood to the head there is loss of consciousness and convulsions. 394 DISEASES OF THE NERVOUS SYSTEM When the cerebral anaemia is of slow onset and raore or less chronic in character, the symptoms are those referred to in large measure under the head of "passive hypersemia." There is headache, tinnitus, vertigo, dullness, drowsiness, irritability, insomnia and depression. There may be dimness of vision and hearing, and muscular weakness and the general symptoms of a poorly acting brain. As a matter of fact, however, it is probable that in cerebral anaemia these symptoms are due as much to the presence of toxic elements as to the insufficiency of blood. In serious forms of general anaemia and even in the most severe types of pernicious anaemia no very marked cerebral symptoms occur. CHAPTER XVIII DISEASES OF THE BRAIN These diseases, like those of other parts of the nervous system, consist of malformations, vascular disturbances, inflammations, soft- enings, hemorrhages, degenerations and scleroses, chronic infections, tumors and functional disorders. THE INFLAMMATIONS OF THE BRAIN The important forms are acute suppurative encephalitis, acute exu- dative forms of encephalitis, chronic meningo-encephaHtis (general paresis) and multiple sclerosis. ACUTE SUPPURATIVE ENCEPHALITIS {Abscess of the Brain) Brain abscess is a suppurative inflammation which affects the par- enchymatous and other structures of the organ. It is always a focal disease, but may be single or multiple. Etiology.' — The primary cause of all forms of brain abscess is practi- cally a microbic infection. The form of microbe, its mode of entrance, and the part of the brain attacked vary greatly. The predisposing causes relate chiefly to age and sex. Brain abscess rarely occurs before the first year or after the fiftieth year of life. It is rather frequent in young people, and occurs on the whole oftenest between the ages of ten and thirty. Males are more often affected than females in the ratio of about three to one. The exciting causes are chiefly disease of the ear, of the nose,^ and of the cranial bones, injuries, remote suppurative processes, and septic and other infections. Chronic inflammation of the middle and internal ear is the most common cause of brain abscess, especially when that dis- ease affects the tympanum and mastoid cells. Only a small percentage of abscesses (about 8 per cent.) follow acute otitis media. This is the reason more cases occur in adults. Caries of the ethmoid and nasal bones and of the orbital cavity leads to brain abscesses in a considerable proportion of cases. After chronic ear and bone diseases injuries are ^ In 9,000 consecutive autopsies at Guy's Hospital there were fifty-seven brain abscesses due to ear diseases and one due to nasal disease (Pitt). 395 396 DISEASES OF THE NERVOUS SYSTEM the most frequent cause, making up about 50 per cent, in all. The injury may be a compound fracture with direct infection from the open wound, or the abscess may be the result of ccntreccup and may develop in a part of the brain opposite to that which was injured, or the abscess may de- velop below the point injured, there being apparently healthy tissus between the surface of the brain and the diseased part. These abscesses develop through laceration of brain tissue and subsequent infection of the wound with organisms. The most common remote suppurative processes which are followed by brain abscess are tuberculous inflamma- tion of the lungs, fetid bronchitis, and empyema. Brain abscess may develop, however, from distant points of suppuration on the extremities or in almost any part of the body. General sepsis may lead to the pro- duction of brain abscess. Among the infectious fevers which are compH- cated with brain abscess are diphtheria, typhoid and typhus fevers, ery- sipelas, small-pox, the grippe. The oidium albicans or thrush may also be a cause. Brain tumors sometimes become surrounded by a suppura- tive encephalitis or may break down with the formation of mixed sup- purative and neoplastic tissue. Tuberculous tumors are most frequently accompanied by suppurative encephalitis. Symptoms. — Brain abscesses take sometimes an acute and sometimes a chronic course. In acute cases the symptoms develop rapidly and the disease runs its course in a few days or weeks. The symptoms come under the general head of those of pressure, those of poisoning from the diseased focus, and local symptoms due to irritation or destruction of certain special areas of the brain. The pressure symptoms are those of headache which is often very severe and persistent, vomiting which is quite fre- quent, though not invariable, vertigo, and a condition of mental dullness which may pass into a delirium ending finally in coma. Optic neuritis often occurs. The pupils are apt to be irregular, but furnish no definite indications. The pulse is usually slow, ranging from 60 to 70, but it varies a great deal. The temperature is normal or subnormal, as a rule, but this also varies, and it may rise several degrees above normal, always running an irregular course. The blood may show an increase in leuko- cytes and especially of the polymorphs. This is not always the case, however, and absence of leukocytosis does not exclude abscess. The toxic symptoms are those which we get in septic poisoning; namely, prostration, irregular fever, emaciation, anorexia, and such mental and sensory disturbances as have already been referred to. As a result of local irritation or destruction, there occur convulsions, paralysis, aphasia, and disorders of some of the special senses. Convulsions are not very common. When they occur they are generally of an epileptic character. The paralysis is usually in the form of hemiplegia. The cranial nerves are not often involved, if we except the optic. The urine is said to show DISEASES OF THE BRAIN 397 a diminution in chlorides and an increase in phosphates. The patient dies finally in coma from exhaustion. In the chronic form of brain abscess the symptoms may for weeks, months, or years, remain practically latent, after the exciting cause has been at work and after the abscess has been established. The patient during this latent stage may suffer from headache, vertigo, mental irritability, and depression; he may at times have a convulsive attack. Occasionally there will be an exacerbation of the disease, at which time he suffers from intense pain, vomiting, perhaps delirium or a convulsion. From this he recovers and continues in a fairly good state of health again. After a variable period, usually of weeks or months, the terminal stage sets in. This terminal stage of the chronic form may assume very much the characters of the acute form already described. In other cases it shows itself by a sudden apoplectic or epileptic seizure or a sudden attack of coma, in which the patient sinks and rapidly dies. These terminal phenomena are due to the fact that the abscess, which has been previously encysted and quiescent, suddenly breaks into a lateral ventricle or through the surface of the brain, or to the fact that a hemorrhage occurs into the abscess. Complications. — The common complications of brain abscess are a phlebitis of the superior petrosal and lateral sinuses and a meningitis. The phlebitis accompanies abscesses that are caused by disease of the ear. The meningitis may be caused by ear disease, but more frequently accompanies abscesses due to injury. When phlebitis is present there will be found an oedema about the ear and neck and a hardness of the jugular veins. In meningitis there is apt to be more rigidity of the neck, more pain, and there are often cranial-nerve paralyses. Pathology. — Acute suppurative encephalitis resembles acute suppura- tive myelitis in the intimate nature of the changes that take place. There is an intense congestion of the part, which gives it a reddened appearance and which used to give to this process the name of red softening. This condition, however, is only the initial stage of the suppurative inflamma- tion and does not deserve to be ranked as a special form of inflammatory process. As the process continues the parts become crowded with leuko- cytes and infiltrated with inflammatory exudate. The nerve-fibres and cells are destroyed, in part mechanically, in part by the poisonous influ- ence of the pyogenic organisms. The nerve-cells lose their normal con- tours, swell up, and disintegrate; the neuroglia cells absorb the broken- down detritus and swell up, forming what are known as granular cor- puscles; the leukocytes increase until a purulent mass is formed. The total result is a mixture of broken-down nerve-fibres and cells, leuko- cytes, and granular bodies. Bacteriological tests show the presence of various pyogenic microbes. Usually staphylococci or streptococci; 398 DISEASES OF THE NERVOUS SYSTEM rarely the pneumococcus. The abscess thus formed varies in size from 1 cm. to 6 or 8 cm. in diameter (two-fifths of an inch to three inches). It is generally somewhat round, and if the case is chronic a fibrous wall is formed. It takes from three to four weeks for such a wall to develop (Fig. 201). Brain abscesses are usually single, occasionally there are two or three. In some conditions they are multiple, that is to say, there may be fifteen, twenty, or more. Multiple brain abscesses are always small and are usually due to pysemic infection. Location. — Brain abscesses involve the cerebrum oftener than the cerebellum, in the proportion of about four to one (Barr) ; two to one (Le Fig. 201. — -Abscess of cerebellum. Fort and Lehmann). They occur rather oftener in the right cerebrum. They are very rare in the pons and medulla. The cerebral lobes oftenest affected are the temporal and frontal. In the cerebellum it is the lateral hemispheres that are most frequently attacked. The seat of the abscess has important relations to the cause. Abscesses due to ear disease are almost always either in the temporal lobe or in the cerebellum. If the ear disease is in the tympanum, the cerebrum is usually the seat of the abscess. If the disease is in the mastoid cells, the cerebellum is usually the part affected. If the disease is in the labyrinth, the abscess is also more apt to be in the cerebellum. This distribution of the seat of the disease is due to the anatomical relations of the bony parts to the temporal DISEASES OF THE BRAIN 399 lobe and cerebellum, respectively. Small veins from the temporal lobe and small veins from the tympanum connect with the superior petrosal sinus, and thus establish a pathway of infection. Often there is a sinus thrombosis and inflammation. Similarly small veins from the cere- bellum and the mastoid and labyrinth connect with the lateral sinus, and here too a phlebitis may be established. Brain abscesses due to injuries are more frequent in the frontal and temporal lobes. What are known as idiopathic brain abscesses — that is, those which arise without any known cause — are most frequent in the frontal lobes. This is because most such cases are due to an unrecognized affection of the nasal cavities and ethmoid bone. Infection from the nasal and ethmoid cavities may lead to sinus thrombosis also, or to a localized purulent meningitis, some- FiG. 202. — Showing the points where the trephine is usually applied and the relations of the sinus. The divisions of the lines indicate quarter-inches. (Lancet.) times called extra-cerebral abscess. Such infections sometimes cause also only a local non-purulent meningitis involving the optic nerves. Brain abscesses due to suppurative processes in the lungs and pleura are probably embolic; and, as the emboli are carried up into the middle cerebral artery, the brain abscesses having this origin are situated in the field supplied by this artery. In children under ten, in whom brain abscess is usually due to ear disease, the cerebellum is more apt to be affected. Course. — Acute abscesses last from five to fourteen days, rarely over thirty days. Traumatic cases run the shortest course. Chronic ab- scesses may have a latent period of weeks, months, and in rare cases even one or two years. When terminal symptoms come on, death occurs in a 400 DISEASES OF THE NEEVOUS SYSTEM few days. In a few cases brain abscesses have been spontaneously evacuated through the nose. Aside from this, the termination is always a fatal one unless surgical interference takes place. There is sometimes a recurrence of the abscess after an operation. Prognosis. — The outcome of brain abscess is always a fatal one unless surgical interference takes place. There is sometimes a recurrence of the abscess after an operation. Diagnosis. — The diagnosis of brain abscess is based upon the history of injury, aural or nasal disease, or remote suppuration, upon the general symptoms of sepsis, upon the presence of headache, vomiting, slow pulse, normal or subnormal and irregular temperature, a local tenderness of the scalp and rise of temperature over the seat of the lesion, hebetude, de- lirium, optic neuritis, rapid wasting, and diminution of chlorides in the urine and leukocytosis with a high polymorph, count. Lumbar puncture may help to exclude a meningitis. Puncture of the brain itself has been recommended, and it may be tried under conditions which allow prompt operation if indicated. The diagnosis of the location of the abscess is based upon the history of its cause, whether from injury, ear disease, emboli from the lungs, or nasal disease; also upon the presence of hemiplegia, local convulsions, tenderness and rise of temperature of a certain area of the scalp. As brain abscesses are apt to affect latent regions like the temporal and frontal lobes, local diagnosis is often difficult. The diagnosis must be made from tumors of the brain, meningitis, and phlebitis of the sinuses. The differential points are given under the heads of the diseases mentioned. In some cases of otitis media symptoms of abscess occur, but there is an exquisite superficial tenderness limited to the region of the trigeminus. These cases recover and are perhaps due to a dural congestion (Gushing). Sometimes, also, in otitis symptoms of the acute serous meningitis of Quincke occur, which are reheved by lumbar puncture. Sometimes with nasal disease there maj^ develop optic neuritis, headache and other symptoms of brain involvement, but no general symptoms, and no symp- toms of serious brain irritation. These are cases of localized frontal meningitis and they get well. Treatment. — The actual treatment of a brain abscess after it has de- veloped is, as already stated, exclusively a surgical one. The successes so far have not been very great, but they have been sufficient to justify operation and to furnish greater hope for the future, when a more exact diagnosis can be made and a wider surgical experience has been obtained. According to Gushing, the percentage of recoveries now is about fifty. The accompanying figure shows the points to be located in trephining for abscess from ear disease. Something is due to the patient in the way of prevention, especially in cases of persons who have chronic aural or DISEASES OF THE BRAIN 401 nasal disease with carious processes. These should be carefully watched and treated. ACUTE NON-SUPPURATIVE ENCEPHALITIS {Hemorrhagic Encephalitis, Hemorrhagic Polioencephalitis) Acute non-suppurative encephalitis is an exudative and sometimes hemorrhagic inflammation of the brain, characterized by symptoms of general infection or toxaemia, of severe cerebral irritation, and the signs of local cerebral lesion. It is sometimes spoken of as a non-suppurative encephalitis and as a curable encephalitis. The disease occurs as one form of the infection when an epidemic of anterior poliomyelitis occurs. In some of its forms, it is probably a rather common affection, complicat- ing especially the acute infectious fevers of childhood and of influenza. The disease then may be grouped in the following manner: Acute non-suppurative encephalitis. 1. Heine Medin type of encephalitis due to the microbic infection which causes infantile paralysis. It is the encephalitic expression of an infantile paralysis infection. 2. Acute encephalitis, due to various other infections. 3. Polioencephalitis superior and inferior, due to influ- enzal and other infections. 1. When the inflammation is due to the poliomyelitic infection it occurs as a complication of the myelitis, as in Landry's paralysis; or it may occur as a separate focal lesion. Such cases have been noted in epidemics. Fortunately the complication is rare. 2. The ordinary type occurs rather more frequently in children, and a special children's type has been described. It attacks the cerebral hemispheres, sometimes the cortical gray matter alone, very rarely the cerebellum. 3. The mid-brain, pontine and bulbar forms are seen in the adults, most frequently in association with alcoholism and influenzal infections. Etiology. — -The disease is caused by the same infection as that of epidemic poliomyelitis; by other acute infections, especially those of influenza, of the fevers of childhood, of otitis media, and other nearby infected centres; it is one of the results and complications of serious alcoholism. It occurs most often in the young — except in Type 3 caused by alcohol and influenza. In some cases, trauma, when associated with some slight infection and perhaps laceration of the brain, causes it. Encephalitis has been known to follow a malignant endocarditis, and in connection with the infections of the puerperal state. 402 DISEASES OF THE NERVOUS SYSTEM Symptoms. — Symptoms of the disease, when it affects the medulla or mid-brain (Type No. 3), are described under the heads of acute bulbar palsy and acute ophthahrioplegia. When the disease attacks the hemi- spheres, it begins, as in most of the cases, rather suddenly and without notable premonitory symptoms. The patient is seized by headache followed by fever sometimes reaching 105°F. This may be associated with vertigo, vomiting, photophobia and delirium. In children there may be convulsions. The symptoms of irritation disappear and are followed by a condition of semicoma or stupor. The patient can generally be partly aroused, and he does not usually have the stiff neck or the small pupils of meningitis. The respirations are shallow and frequent, the pulse is rapid and feeble. As the disease progresses, the deep reflexes are dimin- ished and later the sphincters may be involved. After the patient has lain in a semicomatose condition for several days, he may become less stupid and more irritable and restless; or after two or three weeks of comparative stupor he may begin gradually to improve and, in a few weeks more, convalescence takes place, leaving him well or with some motor or mental defect. In some cases an epileptic convulsion may occur in the early part of the disease. Again, as the disease develops, aphasia and paralysis of the arm or leg, or hemiplegia, may appear. In accordance with the location of the inflammation, the patient may have disturbances in the motor sphere, or he may have hemianopsia, hemiataxia, or disturbance of the cranial nerves, such as nystagmus, or eye palsy, or difficulty in speech and deglutition. Children may be left with a mental defect, and prob- ably mild attacks of this disease are often the cause of such defects, or of epilepsies, attributed, perhaps, to an ordinary infective fever. An optic neuritis may also occur. Course and Prognosis. — -The disease is always serious but is not by any means always fatal. It may in its milder form run a course of two or three weeks, the patient gradually coming out of his stupor and making a slow recovery. In other cases the coma continues to deepen and the patient dies of exhaustion, and in still other cases the disease passes into a chronic state in which he lingers for weeks and even months. Diagnosis. — -The Heine-Medin tj'^pe of encephalitis may be suspected if the symptoms develop in the course of an epidemic of poliomyelitis. The third or alcoholic and influenzal type of the disease occurs in adults. The second or common type is seen oftenest in children and is probably often mistaken for meningitis. It is to be differentiated from this by the sudden onset with coma, the absence of projectile vomiting, and of pinhole pupils, stiff neck, hypersesthesia, and rigidity of the limbs. The presence of hemiplegia or local paralysis, or the occurrence of an epileptoid attack would point to encephalitis. The previous occurrence of an attack of DISEASES OF THE BRAIN 403 grippe in the young, or of exposure to the sun or acute alcoholism in the adult would lead to the probability of an encephalitis. By the help of lumbar puncture fluid can be withdrawn from the spinal sac, and exami- nation of it will exclude meningitis. Pathological Anatomy. — The pathological process underlying this disease consists of an acute inflammation with intense congestion and numerous small hemorrhages and capillary emboli. There is some hem- orrhagic exudation as well as infiltration of leukocytes, with a certain amount of softening of the cerebral tissue in the neighborhood. The parts most frequently affected are variously stated to be the frontal and occip- ital lobes, the central convolutions, the semi-ovale, the temporal lobes, the base of the brain, and the corpus striatum. In four cases which I have examined, the process was in the temporal lobes, the parietal lobule, the mid-brain and the corpus striatum. It has been known to affect the cerebellum. If the process is a mild one, the hemorrhage and exu- date are absorbed, and the injured brain tissue is gradually replaced by connective and neuroglia tissue. In this way small foci of sclerotic tissue are formed and the patient may afterward suffer from symptoms due to this condition. In the severer cases the softening becomes more exten- sive, larger hemorrhages occur, and in one case I have seen a massive apoplexy as the terminal stage. Treatment. — ^The patient should be kept quietly in bed and should be given an active purge. Calomel is usually employed, but croton oil has seemed to me to be much more efficient as an eliminative and counter- irritant. The kidneys should be kept active, an ice cap placed upon the head, and leeches placed at the back of the neck. Urotropin may be given. The treatment after this can be only that of elimination, seda- tion and support. If the patient is stuporous and has a high fever small doses of aconite should be given. If he is asthenic, he should receive strychnine. The nourishment should be carefully attended to and, if he suffers pain, he should have morphine. Chloral and bromide seem to be the most efficient agents for relieving the restlessness and insomnia. GENERAL PARESIS {General Paralysis of the Insane — Dementia Paralytica) General paresis is a disease of the brain characterized clinically by peculiar associated physical and mental disturbances, which end eventu- ally and usually in general paralysis and mental deterioration; anatom- ically it is characterized by a syphilitic parenchymatous meningo- encephalitis. The disease cannot always be distinguished clinically from meningo-vascular syphilis. Etiology. — -Paresis, is a disease of modern civilization and of syphiliza- tion. It was a medical curiosity a hundred years ago; now it is extremely 404 DISEASES OF THE NERVOUS SYSTEM frequent in our asylums, in neurological clinics, and in private practice, and it has become much more common of late years partly because it is more frequently recognized. It is found in all the civilized races of Europe and America. It affects even the inferior races living among civilized people, and is found, for example, among the negroes of the United States, affecting especially the negresses (Dieffenbach) . It is said to be rare among Chinese and Japanese and in Moham- medan countries even where syphilis prevails. It is more frequent in countries where social conditions lead to the spread of syphilis. It is rare, as a rule, in rural communities as compared with cities. However, in countries where there is enforced military training and a period of barrack life, the disease becomes prevalent in the country. It occurs oftener in men than women. In my private practice the ratio is about 10 to 1 ; in hospital practice it is about 3 or 4 to 1. It occurs oftener in the married absolutely but not relatively. It is a disease of middle life, most cases occurring between thirty and fifty; and few cases develop before or after these ages. The women when attacked are a little younger than the men. Neuropathic races and neuropathic persons are more susceptible to the disease. Paresis is sometimes (0.03 per cent.) directly transmitted. It then appears between the ages of ten and twenty and is known as juvenile paresis. In this form males and females are about equally affected. Occupation is a predisposing cause in just about the proportion with which the occupation leads to exposure to syphilis. In Europe it is very frequent among military officers in some countries and it is very rare among the Catholic clergy. The excessive use of alcohol, excessive mental exertion, if combined with emotional strain and excitement, sexual excesses and abuse are predisposing causes. A certain popular idea that the disease is the result of perverted sexual indulgence is not correct. It has now become firmly established that paresis is always due to a syphilitic infection, which leads to a parenchymatous degeneration of cerebral neurons; just as tabes is due to a similar affection of the spinal neurons. It occurs in about 1 to 2 per cent, of all luetics and makes up about 10 per cent, of our asylum population. It comes on oftenest between the tenth and twentieth year after the infection. Symptoms (Prodromata) .— Paresis is sometimes preceded by a pro- dromal period which may last for four or five years, more often perhaps a year. During this time the patients suffer from symptoms that re- semble somewhat those of neurasthenia, sometimes those of a mild anxiety psychosis. The patient shows a change in character. He is more irrita- ble, less able to concentrate, has some memory defect, and is less efficient DISEASES OF THE BEAIN 405 in his work. He has headache, insomnia and cerebral and spinal dys- sesthesias, and parsesthesias. He worries about himself and is termed hy- pochondriacal. At this stage he often has quite clear insight and appre- ciates his danger. But unlike cases of true neurasthenia he cannot do efficient work, though he may wish to do so. If there is a little tempera- mental elation he may work unwisely and foolishly. It sometimes hap- pens that the peculiarities of mind and conduct are so slight that, it is only in retrospect, and after serious conditions develop that the family remember the early changes. In more advanced cases the patient may suddenly develop a con- vulsion, or some change in the pupils and reflexes may appear. Physical examination may not reveal any objective changes, except that his fluids may show positive reaction to the Wassermann and other luetic tests, but this is rather easily controlled by treatment. Such patients under active treatment may get well; but if the trouble is not recognized, some form of cerebral syphilis eventually develops. Even if this luetic neurasthenia progresses till some characteristic mental and physical symptoms of paresis or tabo-paresis, or meningeal syphilis develop, the patient may get well or at least secure a complete remission. The preparetic condition is a curable one. But besides these neurasthenic and anxiety prodromata, paresis may be preceded by a distinct functional psychosis. This takes the form oftenest of a luetic melancholia, more rarely of a mania. The patient may and generally does emerge from this and he may then remain well, but more often unless treatment is unusually effective, he begins to de- velop the characteristic signs of general paresis. Very rarely paresis comes on suddenly like an explosion. Finally and not rarely paresis is preceded by distinct symptoms of cerebral or cerebrospinal meningeal syphilis, or by a tabes dorsahs. Thus paresis is approached in four different ways, clinically speaking : 1. By a luetic neurasthenia, or anxiety psychosis. 2. By a luetic melancholia or mania. 3. By a meningeal cerebral or a spinal syphilis. 4. In a gradual or explosive manner. General paresis is classified in various and numerous ways. Many descriptions have been based on a study of custodial and more or less advanced cases. The description and classification I give are based largely on a per- sonal study of cases as I have seen them develop and pass into hospital conditions or into states of improvement and remission. The various, phases of advanced paresis have little practical interest. The disease develops in 1. An excited form. 406 DISEASES OF THE NERVOUS SYSTEM 2. A depressed form. 3. With quiet dementia. 4. In an explosive form. These various types may change in the course of the disease. In my experience about one-third are at first seriously depressed, less than a third maniacal and more than a third pass with perhaps some slight excitement or delusional activity into a progressive dementia; that is to say, unless the course is modified by treatment. In all cases some meningeal symptoms may be present. Symptoms of Onset : Active Type.- — In the first type of cases the pa- tient begins by showing unusual irritability of temper; trivial things an- noy him, and his bad humor and change of disposition become notice- able in his family relations and in his business. He is fretful; complains of being easily fatigued ; loses interest in his affairs, and is unable to fix his attention for any length of time upon them. He makes occasional mis- takes of judgment, and does some extravagant or foolish thing in the way of purchasing or selling. He is totally lacking in insight. This condition of irritability is followed by one of great mental exaltation. The patient becomes very happy and cheerful and confident; he feels better than he ever did before in his life. He talks excessively, and is effusive and j ocose when he used to be sober and reserved. He develops great schemes for the future, he lavishes money uselessly in making presents to his family and friends, or in some extraordinary business venture, and imagines himself possessed of immense wealth or great power. He has, in other words, delusions of grandeur, or megalomania. This condition of exaltation is interrupted by outbursts of violence, especially if it leads him to indulge in drink, as is often the case. In the course of three or four months the symptoms become so marked that the family recognize the seriousness of his state, and he is confined in some institution where he can do himself and others no harm. Under institutional regime he now becomes somewhat more quiet; his exaltation softens down. His symptoms may even remit, and for a time he becomes nearly or quite rational. But after some months he begins to show distinct signs of dementia; the memory becomes weak, he forgets recent events, mislays things, makes mistakes in his accounts, is unable to add correctly; he can- not write a letter coherently, or if he does there are mistakes in spelling and elisions of letters. During or before the exaltation there gradually appear physical symp- toms which are very characteristic. The patient's hands become tremulous, and his handwriting is so affected that his signature often cannot be recognized. There is distinct and decided facial tremor, particularly apparent if the patient is made to close the eyes and stretch the muscles of the lips so as to show the teeth. DISEASES OF THE BRAIN 407 There is marked tremor of the tongue, all this tremulousness being much more exaggerated than is seen in other diseases, except occasion- ally in acute alcoholism. The speech becomes stuttering and thick, and he cannot pronounce long words clearly. On examination of the reflexes it is usually found that the knee-jerks are exaggerated. The pupils are almost always uneven and, as a rule, react badly to light though fairly well to accommodation, showing, in other words, the Argyll- Robertson pupil; sometimes they do not react either to light or accom- modation. The fundus oculi is normal. There is an early and decided weakness of the sexual function. The bladder may also become weak. The appetite and vegetative organs remain in fairly good condition. The patient often suffers from per- sistent insomnia. During this time he also has occasionally vertiginous, syncopal, or apoplectiform attacks. In the latter he falls down and perhaps has hemiplegia lasting for a few days or a few weeks. An epileptic convulsion may occur. In some cases the knee-jerks are abolished, and there is some ataxia and evidence of a posterior sclerosis. The general muscular power is much diminished, and the patient is unable to take long walks or do any great amount of physical exercise. In the second stage the most striking feature is the gradual onset of dementia. The patient now becomes more quiet and is incHned to sleep during the daytime. He takes little interest in affairs about him, is extremely forgetful, and is often unable to recognize even his intimate friends. He no longer knows the day of the month nor the year, and cannot tell one anything about current events of the day. He becomes gradually careless about his person, and has to be watched while at his meals lest he spill food on his clothes, and at the toilet lest he soil him- self. Finally, he needs to be cared for as if he were a child. At this late period, again, attacks of an apoplectiform character may come on, leaving him temporarily or perhaps permanently hemiplegic. His appetite often continues good, sometimes voracious, and he may gain flesh. He is apt at this time to have periods of excitement in which he has delusions of persecution, or he may have some slight delusions of grandeur. One patient of mine used to weigh himself every day and think he was gaining ten pounds each time. He kept on till he thought he weighed nine hundred pounds. In the last scene of all he becomes bedridden and helpless, terminal febrile attacks occur, and he finally dies of exhaustion. The somatic symptoms during this last period consist of increased tremor, disturb- ances of speech, and gradual muscular weakness until the patient be- comes helpless. The average duration of the disease is about three years. There 408 DISEASES OF THE NERVOUS SYSTEM are some acute, galloping forms in which the patient dies within a year, and there are some cases in which the patient reaches a stage of partial or complete dementia and remains in this condition for ten or fifteen years. The Depressed Type. — In this form the disease begins with symptoms resembling those of neurasthenia and hypochondriasis. The patient complains of disagreeable sensations about his head, hemicrania, pain in his limbs and back, inability to sleep, disorders of the stomach, and vague sensations of discomfort and oppression which he is unable dis- tinctly to describe. These are the patients who are often treated as neurasthenics for a long time, and at first show hardly any physical or mental symptoms suggestive of the real trouble. Careful examination, now, however, will show some objective physical changes including a positive serological formula. Now evidences of mental disturbances appear, and they are mostly those of dementia with perhaps delusions of persecutions and suspicion. These delusions may be accompanied by occasional outbreaks of excite- ment and violence, but the paretic is rarely homicidal and, it may be added, rarely suicidal, except when he has distinctly a melancholia. After dementia has set in, the physical symptoms of tremor, scanning speech, and tremulous handwriting all become noticeable, and the final stage resembles that of the other form. Dementing Type. — In a third type there is a primary dementia. The disease begins without any excitement or any special depression, with symptoms of forgetfulness, lack of attention to business, and incapacity to do work. The patient makes mistakes in his calculations, mislays and forgets things, and soon is found by his employer to be of no use. He is often good-natured, not unhappy, and without distinct delusions of any kind. The somatic symptoms of tremor, fixed pupils, and exaggerated reflexes may appear, but sometimes are entirely absent. Scanning speech is not always present, or comes on late. Tabo-paresis. — Some symptoms of tabes are present in almost 10 per cent, of paresis. It is the paresis that is the early, dominant and important factor; for tabo-paresis is really and practically paresis. The tabetic symptoms are of the minor kind: Argyll-Robertson pupil, lightning pains, loss of knee-jerk, some hypotonia and more rarely some ataxia. When tabes becomes fully and first developed, paresis rarely appears (3 to 5 per cent, of cases). Meningo-vascular or Pseudo-paresis. — There are some cases of paresis in which symptoms of exudative syphilis introduce and accompany the disease. The patient has at first eye palsies or attacks of hemiplegia with intense headache followed by convulsions. It is recognized that he has a syphilitic exudate pressing upon some part of the brain, either DISEASES OF THE BEAIN 409 the base or the convexity, usually the former. Under proper treatment, this resolves and he gets over the paralysis and the seizures, but it is now found that his mind is slightly affected. He has no delusions perhaps, and no immediate exaltation, but his memory is impaired, judgment weakened, his emotional condition is one of excitability, and he has to give up business and live a quiet, inactive life. If he does this, in some cases the disease becomes arrested, and he remains fairly well for a number of years. Cases of apparent recovery have been reported, but unless treatment controls the situation, dementia finally sets in. In these cases there is both a parenchymatous and a meningeal syphilis. >^/ Fig. 203. — Left hemisphere in a case of general paresis. The praifontal area of higher association is grossly wasted. The psychomotor area shows some but much less waiting. The temporal, parietal and insular regions of associations are acutely changed. The after part of the temporal region of association and the anterior part of the parietal show less acute change and more wasting. The visual center and the visuopsychic cortex around it are intact. (Bolton.) Alcoholic Pseudo-paresis. — Persons who have for long periods of time continuously and excessively indulged in alcohol may develop, and generally do, a condition of mental weakness which to a certain extent simulates paresis. If these patients have not had syphilis, however, it is not a true paresis. The patients become weak-minded, feeble in judgment, poor in memory, their moral instincts get out of control, and acts of extraordinary selfishness, bestiality, and besotted- ness are manifest. They gradually become more enfeebled, and finally enter a condition of dementia if they are not carried off by some in- tercurrent disease, as is often the case. These cases, however, do not present the physical symptoms of paresis. They do not have the speech disturbances, the paralyses, or the apoplectiform seizures that 410 DISEASES OF THE NERVOUS SYSTEM characterize the true disease, and if they can be kept from alcohol they may remain in a state of partial dementia for many years. Diagnosis. — The diagnosis is based on the history of syphilis, the positive serology, the facial and tongue tremor, irregular and rigid pupils, exaggerated or irregular knee-jerks, the disturbance of speech, weakness of memory, loss of power to work, irritability and change of character, change in the handwriting, and presence of convulsive or apoplectiform attacks. Examination of the cerebrospinal fluid and blood shows serum Fig. 204. — Early general paresis. A small cortical artery is shown with a begin- ning injection of round and plasma cells; dying pyramidal cells are also to be seen (X320). (Bolton.) Wassermann + , cerebrospinal fluid, Wassermann + , cells per cubic milli- metre + + , globulin + , colloidal gold+ (see table, page 412). Pathological Anatomy. — The dura mater is adherent to the calvarium in places or throughout. In old cases there is sometimes a pachy- meningitis interna with hematoma. The pia mater is cloudy and thickened, especially over the frontal and parietal regions, less on the base and never over the occipital lobes. The brain is atrophic in old cases, with shrunken convolutions and local areas where the brain has especially atrophied. The ventricles are dilated and show a granular surface. The loss of brain weight averages about five ounces and is most in the frontal lobes. Microscopically the cortex is irregularly thinned, and the arrangement of cell layers is distorted. The third and next the DISEASES OF THE BRAIN 411 second layers are the most affected. The nerve-cells show all stages of degeneration. The cortical fibres also are atrophied and in the last stages of the disease have quite disappeared, the supra-cortical radial fibres being the first to go. The neuroglia tissue is greatly increased and shows evidence of active proliferation and finally forms sheaths about the blood- vessels. (Fig. 205.) The blood-vessels are especially affected. There is an active for- mation of new vessels, with numerous dilated capillaries and widened adventitial lymph spaces. These spaces are filled with cells which are partly lymphocytes and partly plasma cells. This vascular change. Fig. 205. — Very advanced general paresis. A vessel of the gray matter showing par- tially organized periarteritis, and outside of this a neuroglial sheath ( X 320) . (Bolton.) with plasma cells, is one especially characteristic of paresis (Vogt, Alzhei- mer). The cells are large, round or irregular in shape, with a proto- plasmic body. It is not known whether they come from connective tissue or the leucocytes of the blood. In other parts of the brain focal, diffuse and tract degenerations occur. The cerebellum is affected like the cerebrum but to a less degree. The spinal cord shows degenerations in both the posterior and lateral columns in about two-thirds of the cases. Some degenerative changes occur in the sympathetic system and peripheral nerves. The treponema pallida has been found deep in the cerebral tissues, but the organisms are few in number and difficult to detect. The explanation of how they 412 DISEASES OF THE NERVOUS SYSTEM cause such an extraordinary disintegration of the master tissue of the body- makes a long chapter in pathology, and one not yet completed. Prognosis. — The prognosis was formerly thought to be invariably bad. If one sees the patient, however, in the earliest stage, removes him at once from sources of excitement, and makes him live quietly, using anti-luetic and tonic treatment, one can sometimes check the disease, and practically cure it. When the disease has well entered upon its course it is incurable by any measures as yet known. The prognosis is better the more the symptoms and serological formula suggest meningeal syphilis. It is better in cases in which the patient shows "insight" as to his condition; better when the mental symptoms indicate a functional psychosis more than a deterioration. Speech defects are of unfavorable omen. Tabo-paresis has about the same prognosis as paresis. When meningo-vascular syphilis exists, if a tabes develops paresis does not, as a rule, and the outlook is not so serious. Too much importance may be placed on the serological formula. It may be partly positive and yet the patient may do very well. Positive reactions may disappear under the influence of time and good hygienic conditions alone. Laboratory Tests for the Presence and Differentiation of Syphilo- genous Nervous Disease. — The serological formulae obtained by testing the blood serum and the cerebrospinal fluid is here given for the three important luetic nervous diseases — meningo-vascular syphilis, tabes dorsalis, paresis and tabo-paresis. This table was prepaied by Dr. David Kaplan of the N. Y. Neurological Institute, and is the result of over two thousand complete examinations. Many of the fluids tested were from my own patients. I have had occasion to follow this work and the formulae and conclusions given agree with my observations up to the present time. The Serological Formulae Found in Syphilitic Nervous Diseases Cells per oc. W.R. in. C.S.F. Globulin. Fehl. Reduct. Colloid. Gold W.R. in Serum I. Meningo-vascul a r Average Plus Excess Present Present Plus Lues 80 to 200 in 65 in 65 in 95 less than in 80 per cent. per cent. per cent. 2 per cent. per cent. (Cerebral, Spinal or Extremes Cerebrospinal). to 2000 II. Tabes Dorsalis Average Plus Excess Present Absent Plus 35 to 60 in 40 in 33 in 99 in 65 per cent. per cent. per cent. per cent. Extremes to 350 DISEASES OF THE BRAIN 413 The Serological Foemul^ Found in Syphilitic Nervous Diseases . — Continued . Cells per cc. W.R. in C.S.F. Globulin. Fehl. Reduct. Colloid. Gold W.R. in Serum III. Paresis Average Plus Excess Present Present Plus 18 to 35 in 85 in 75 in 100 in 95 in 95 and Extremes to 250* per cent. per cent. per cent. per cent. per cent. Tabo-paresis 20 to 40 Plus Excess Present Present Plus in 85 in 70 in 100 in 75 in 95 per cent. per cent. per cent. per cent. per cent. The Wassermann reaction on the spinal fluid was performed by using 0.5 cc. of the medium, with two antigens; one containing cholesterin. The globulin content was determined by the butyric acid ammonium sulphate method. The cells were counted by the Fuchs-Rosenthal method, the same day the fluid was obtained. Meningo-vascular Lues. — This form represents the serology of the earliest in- volvement of the nervous system. The increase in cells may appear before a positive Wassermann reaction or a globulin excess, and may persist until cerebrospinal func- tions are impaired and detected by the neurologist. The cell count, which is the chief feature of this pre-eminently exudative process, may reach into the thousands, but is very readily influenced by treatment. The positive fluid Wassermann reaction dis- appears in such a case very quickly, and if a great many cells of the polynuclear variety are present, thus giving rise to the absent Fehling reduction, the latter returns also with the fall in the pleocytosis (increase in cells). The majority of cases with this form of luetic nervous involvement show on an average from 80 to 180 cells per cubic millimetre after moderate therapy. In this form of lues of the nervous system one is able to do away entirely with abnormal findings in less time and with fewer remedies than is the case with tabes or general paresis. Some cases remain uninfluenced after the cell count reaches 20 to 30 per cubic millimetre, using the intravenous method of salvarsanization. In case the intra-spinous route is selected, the pleocytosis is influenced more radically on a mechanical basis, depending chiefly upon the removal of the cells as one would evacuate an abscess. This is further corroborated by the persistence of the globulin excess and the positive Wassermann reaction which are to be regarded, therefore, as an expression of organic response not depending in toto upon the local disease. Tabes. — The early forms of this disease will give a serology closely resembHng that of the meningo-vascular form of lues. The cell count will then show its upper limit, the globulin will give an intenser precipita- tion than in the later stage, and the Wassermann reaction will be positive in both media. As the disease progresses and assumes its more or less permanent features, the serology also tends to settle to what constitutes the type of tabetic serology. The Wassermann reaction in the serum is positive, that of the fluid is negative, or only weakly positive, the excess of globulin is less marked or entirely absent. As the disease 414 DISEASES OF THE NERVOUS SYSTEM progresses and enters the final state, the entire serology may become negative. Unless a paretic accompaniment is detected, the above holds true for the majority of tabetics. If, however, paresis becomes an accompaniment, the serology tends to assume also the serological features of that disease. Therapy will find an obstacle in some cases in being unable to render negative a positive serum Wassermann (the "Wassermann fast" phenome- non). The Wassermann reaction in the fluid will also manifest itself together with a comparatively low cell count, and in a small number of cases one will be able to obtain the characteristic curve with the colloidal gold as found in general paresis. The latter form is entirely different from the form closest to the meningo-vascular type, in that the serology is easily rendered negative, and the high cell count (hyperlymphocytic tabes) will be found to fall with the very first salvarsan injection, al- though given intravenously. The fall may be a few hundred cells to the cubic millimetre. General Paresis. — Aside from furnishing the greatest number of cases that show pathological fluid and serum changes, this disease also furnishes the various abnormal constituents in its greatest intensity. The ''Wassermann fast type" is a feature of this disease more often than in the other forms of lues of the nervous apparatus. It also shows to the exclusion of the other forms of luetic disorder, the characteristic precipitation of colloidal gold, which is one of the most constant accom- paniments of this disease. In fact where one is able in rare instances even to influence the W^assermann reaction, one will find that the colloidal gold phenomenon still persists. In early paresis one will occasionally find an absence of a positive Wassermann reaction. In the cerebro- spinal fluid, the pleocytosis may also be very high, comparatively, showing thereby the common origin of all luetic nervous diseases, which start as a rule with a high cell count and probably with a meningo- vascular disturbance. The advanced form of paresis shows the full quota of abnormal findings, and as the degenerative process advances, one will sometimes miss the positive Wassermann reaction in the serum, while it may still be present in the cerebrospinal fluid. In the latter instance the cell count may be very near the normal cell count. In some cases only a few cells were encountered. That exceptions may be found in this form of neural lues must be admitted, so that one will here and there come across authentic paresis with a very high cell count, amounting to 200 or 300 cells per cubic millimetre; such extreme counts, however, are very rare. Kaplan. Treatment. — The first and most important thing in the treatment of paresis in its early stages is to adopt measures for the treatment of syphilis DISEASES OF THE BRAIN 415 which is the underlying cause. Before the advent of the use of sal- varsan this was done by the intensive use of mercury and iodides combined with ehminative measures, tonics and fresh air. These measures were not without distinct success and it even remains still to be proved that salvarsan treatment is more permanently efficacious than the older methods. There has been of late years so much more interest and effort in the treatment of paresis that enormously better results are being obtained, apparently, by the new method, but certainly to some extent by reason of greater interest now taken in therapeutics. The technical methods of using salvarsan and its preparations differ with different practitioners, and the final and best methods have not yet been agreed upon. The technic which the writer employs and which has been used to a large extent in the New York Neurological Hospital, is as follows: The patient is given an intravenous injection of 0.45 neo-salvarsan. After an interval of two days he is given another injection of 0.9 and this injection is repeated every third day until he has had ten injections. During the time that these injections are given he receives either a hypodermic of one grain of salicylate of mercury twice a week, or daily inunctions of mercury on the days on which he does not receive the intra- venous injection. After the patient has received the tenth dose, he is given a prescrip- tion containing gr. ^^^4 of bichloride of mercury and gr. x. of iodide of potassium and this he takes intermittently for the following four weeks. After this he is given an intra-venous injection of neo-salvarsan every month for five months and then is given another intensive treatment, if the remission or improvement is not completely satisfactory. The intra-venous injections are continued for a period of a year but with less frequency. They should be continued intensively irrespective of the condition of the blood and cerebrospinal fluids. The endeavor to measure the amount of treatment which should be given by the laboratory findings of these fluids is a mistake, or at least these things should not be too closely followed as therapeutic guides. Naturally, while carrying out this technic, a proper regard should be had for the general condition of the patient's health, and measures such as laxatives, tonics, fresh air and rest should all be instituted. The method thus outlined as a standard one needs variations in ac- cordance with the state of the disease and its rate of progress. When there is evidence of an associated meningeal exudate the number of injections may be increased and even brought more closely together. I have seen a very good result from giving 0.9 neo-salvarsan daily for nine days. It seems that the nearer one can come to safe toxic doses the 416 DISEASES OP THE NERVOUS SYSTEM better results will be achieved and for this reason it may be wise to give an intra-spinous injection of salvarsanized serum, or salvarsanized serum with the addition of neo-salvarsan itself in connection with this and in addition to the regular intra-venous injections. It may be neces- sary to give as many as thirty injections in the course of two or three months. There is still a difference of opinion as to the relative value of salvarsan and neo-salvarsan, it being generally believed that the salvarsan is about twice as powerful as the neo-salvarsan, but that it does not act more beneficially, except for the fact that it is stronger. The earlier in the course of the disease that treatment is given the better the outlook. On the other hand, in the third and terminal stage of the disease the treatment is useless and may even hasten the death of the patient. It seems wise in some more chronic cases to give a series of injections of mercury before giving salvarsan. Opinions also still differ as to the value of the intra-spinous injections of salvarsanized serum (Swift-Ellis method) and of the intra-spinous injections of the patient's serum to which salvarsan is added. I have not seen any convincing evidence that the method is a superior one, but there are some who very strongly support it. In the use of this method injections are given not oftener than once in ten days or two weeks, and the number of injections is generally determined by the effect of them upon the cerebrospinal fluid reactions. It has been found comparatively easy to reduce the number of cells to the normal amount in the cerebrospinal fluid by the various methods of treatment, but to make the blood and cerebrospinal fluid negative to the Wassermann reaction in true paresis is very difficult. The result of treatment in early cases is almost invariably to secure a satisfactory remission, and these remissions in many cases are practically like cures. The doctrine put forth by certain neurologists that when parenchy- matous degeneration of the brain sets in, treatment is useless, is certainly untrue and not founded on any sound pathological basis. We know per- fectly well that parenchymatous degeneration of locomotor ataxia is often arrested and patients live for thirty years or more. We also know that remissions have occurred in paresis lasting many years. Aside from the treatment thus outlined the therapeutics of paresis is mainly tonic and symptomatic, in the second and third stages. Various other specific treatments of paresis have been suggested and tried, such as the injections of nucleinate of soda and of tuberculin to produce artificial fever. I have not seen any successes from these forms of treatment. DISEASES OF THE BRAIN 417 Naturally the patient while undergoing specific treatment should be under close observation and be made to live a quiet and regular life. The influence of a life entirely in the open air is of great value in ameliorat- ing the luetic infection. I have seen the serological formula become negative just by this means. MULTIPLE SCLEROSIS Multiple sclerosis is a chronic and intermittently progressive malady characterized by some paralysis, usualty in the form of paraplegia, by coarse tremor, disturbances of speech, nystagmus, apoplectiform attacks, and various other cerebral and spinal symptoms depending upon the seat of the lesion. It is due to the development of sclerotic patches in the different parts of the brain and cord, which patches are for the most part the result of a neuroglia proliferation. The disease is probably an inflammatory rather than a degenerative one. It affects the spinal cord as well as the brain. Etiology. — It occurs rather more frequently in the male sex and is a disease of the first half of life. Multiple sclerosis is, in fact, one of the few chronic nervous disorders of organic origin developing at this time. Most cases begin between the age of twenty and thirty. Cases have, however, been observed in infants and children, but the trouble in its typical forms does not appear in the declining years of Kfe. While a few cases of hereditary multiple sclerosis have been reported, it is generally conceded that hereditary influence is very slight and even a hereditary tendency to degenerative disease or a neuropathic family history is rare though not unknown. Strumpell's theory that the disease is endogenous has not been sustained. There can be no doubt that the most important of all of the few known causes of multiple sclerosis is infection. This is so true that it may be called a post-infec- tious disease. The infectious disorders which are followed by sclerosis are typhoid fever, pneumonia, malaria, and the eruptive fevers. It has been known to follow also diphtheria, whooping-cough, erysipelas, dys- entery, cholera, and even rheumatism. Another important cause is excessive muscular exertion, such as occurs in professional dancers, and amateur or professional athletes. Workers in metallic poisons, such as lead, copper, zinc, and even manganese may develop sclerosis. The influence of trauma in causing multiple sclerosis has to be very carefully weighed. It is certainly very rare, but there seem to be some few cases in which trauma appeared to be an exciting cause. It is generally accepted that neither syphilis nor alcoholism are causative factors, although syphilis may be associated with the disease and perhaps produce a peculiar luetic form of it. 27 418 DISEASES OF THE NERVOUS SYSTEM The following data as to the frequency and cause of the disease were collected by Dr. C. W. 0. Bunker, in his neurological prize essay: Frequency. — Among 70,000 neurological cases in America the percentage of multiple sclerosis was 1 in 266, or 0.058 per cent. In Scotland it is about 2 per cent.; in Berlin, 0.85 per cent.; in Hamburg, 1 per cent. Age. — The following table, compiled by Bunker and myself, represents American cases. It makes the incidence of the disease too late. Practically, the cases occur between the fifteenth and thirty-fifth years. -10, 9 10-20, 17 20-30, 36 30-40, 45 40-50, 32 50-60, 24 60-70, 7 70-80, 4 The disease is twice as frequent in men as women — 129 to 67; statistics of Jelliffe, Bunker and myself. European statistics make the numbers about even. The dis- ease is seen more frequently in foreigners, perhaps, because our clinics are so largely foreign in population. My private cases are nearly all American. Symptoms.- — The disease begins insidiously. There may be a pre- liminary and rather short hemiplegic or paraplegic attack with some sensory disturbance. More often a comparatively short time after recovery from some acute disease the patient begins to suffer from weakness of the lower limbs with stiffness and some degree of heaviness, and numbness. The bladder is also a little weak, and it is difficult to retain the urine. These symptoms after a few weeks may disappear and the patient be well for months. If not, very soon the patient notices some unsteadiness in the gait, due not alone to weakness in the legs, but to an increasing degree of ataxia. He finds also that his hands are trembling and that this tremor increases upon voluntary motion. It is the type of tremor known as ''intentional." He has at this time also some indistinctness in speech, it being difficult for him to enunciate long words. These come out in a slow, syllabic utterance, as it is called, each syllable being spoken separately. He may have also a little trouble in swallowing. By this time he has had some sensation of numbness in the limbs, and some pains occasionally in the joints and extremities, but the sensory troubles are not permanent or not very marked and are mainly parsesthesic. If he is examined now it will be found that the gait is stiff and awkward, the patient walking unsteadily; or, in other cases, it may simply be the stiff, weak gait of moderate paraplegia. The Romberg symptom will be found to be present to some extent. The knee-jerks are exaggerated and ankle clonus and Babinski's reflex may be present. The abdominal reflexes are absent early in many cases. Ihe hands are DISEASES OF THE BRAIN 419 unsteady, and the movements are characterized by a jerky tremor, which may be so great that the patient has difficulty in dressing and feed- ing himself. This tremor disappears almost entirely if the patient lies flat upon his back. If he sits up, however, it may be seen perhaps in the muscles of the neck, causing the head to be oscillated, and constantly more or less regular tremor in the arms is present. As the disease pro- gresses the speech becomes more thick, and often is almost unintelligible in the severer cases. Examination of the eyes shows a nystagmus, per- haps only when the eyes are turned to one side, but often the jerky movements are seen, even when the patient is told to look directly at Fig. 206. — Pallor of entire optic disc except its nasal margin. {Holden.) an object. Evidence of impaired eyesight appears with pallor of the temporal side of the optic disc (Fig. 206), and a central scotoma may be noted. The tongue is protruded in a jerky way, and attempts at swallowing are often awkward. If a glass of water is handed him, the patient seizes it, but in carrying it to his mouth he agitates it so violently that the fluid is spilled and perhaps the tumbler drops from his hands. Examination of the muscular system shows weakness of the legs, less of the arms, but no complete paralysis. There is no marked atrophy of the limbs, and no change of any moment in the electrical reactions. There are many cases in which the jerky tremor, syllabic 420 DISEASES OF THE NERVOUS SYSTEM speech and nystagmus are not marked or develop very slowly, and more recent studies show that the most common symptoms are: 1. Bladder weakness. 2. Absence of abdominal reflexes. 3. Spastic paraplegia. 4. Optic atrophy. 5. Sensory defect (parsesthesise) . 6. Sphincter trouble. 7. Speech defect. Examintion of the cutaneous sense may show some little tactile anaesthesia in the limbs, but this is not always present and in general the cutaneous sensory disturbances are slight and evanescent. There is a certain amount of ataxia, which is not due to muscular anaesthesia but to inability to control and co-ordinate the movements. There is no loss of sense of weight or of pressure. Of the nerves of special sense, the eye is most frequently involved. The patient may have some diplopia from paralysis of one of the eye-muscles. The pupils react to light and accommodation. The involvement of the optic nerve may go on to an almost complete atrophy. On account of this the patient suffers from contraction of the visual field, central and other scotomata, and weakness of vision, but absolute blindness never occurs. Examination of the cerebrospinal fluid by lumbar, puncture shows the absence of a lymphocytosis. While the disease is running the course just described, the patient sometimes suffers from attacks of vertigo, and occasionally from sudden seizures resembling apoplexy, and even from epileptiform attacks. The mind is usually not much affected, and I do not recognize any special mental characteristics except a certain childishness of temperament. There may, however, be some slight dullness of the intellect, some hebe- tude, or even a slight amount of melancholia. In certain cases the patients are subject to attacks of impulsive laughing; that is to say, without any sufficient cause they suddenly break out in exaggerated laughter, from which they quickly recover themselves. These attacks are due the cerebral lesions. As the patient gets worse the paraplegia increases and he becomes finally helpless. The use of the arms is pre- served for a longer time. Involvement of the sphincter of the bladder occurs early, that of the rectum very late if at all. The progress of the affection is variable. It usually progresses slowly with some remissions, reaching finally in two or three years a chronic stage, in which the patient remains for several years without much change. At other times the progress of the disease is hastened by repeated ex- acerbations, accompanied by apoplectiform or hemiplegic attacks. DISEASES OF THE BRAIN 421 In still other cases the amelioration continues and remains permanent, and a practical cm'e takes place. Types. — The various symptom groups of the disease are classified as cerebro-cerebellar, and spinal. The cerebro-cerebellar symptoms con- sist in modifications of speech, attacks of vertigo and apoplectiform seizures, hemiplegia, intention tremor, labyrinthine symptoms, mental changes, optic atrophy and spasmodic laughing or crying. There is sometimes also a certain amount of deafness and perversion of taste and smell. In the not uncommon spinal type, the dominant symptoms are the spasmodic paraplegia, with some bladder and sexual weakness, and a slight amount of sensory trouble. There may be also some weakness and unsteadiness of the arms, but the cranial nerves are not involved. Fig. 207.- -Multiple sclerosis, showing lesions of the cord. Dr. Charles I. Lambert.) {From photograph by Aborted Types. — In some cases the nodules of sclerosis are so limited in number and so peculiarly placed that they give rise to very atypical and mild forms of the disease. Pathology. — Grayish nodules are found distributed through the brain and spinal cord (Figs. 207-208), They vary in size from a milli- metre to two or three centimetres in diameter (one twenty-fifth to one inch). They are of firmer consistence than is the surrounding brain substance, but are not quite so hard as is ordinary connective tissue. They consist microscopically of neurogha tissue, which some assert to be connected with the walls of the blood-vessels, as a rule. Very often the axis-cyhnders of nerves can be seen passing through the lesion. The nodules are found most frequently in the white matter of the brain, more especially in^the pons, internal capsule, and centrum ovale. They rarely 422 DISEASES OF THE NERVOUS SYSTEM begin primarily in the gray matter, but may invade it secondarily. The roots of the peripheral, especially of the cranial, nerves occasionally contain or are surrounded by these sclerotic masses. In the spinal cord they may extend up and down the gray and white matter for a considerable distance, or they may involve the whole cord at a certain level, turning it into a fibrous mass. The blood-vessels surrounding and in connection with these diseased areas show some evidences of thickening and increased vascularity, but no true inflammatory process, as a rule. The primary pathological change in multiple sclerosis is as yet unknown; many things point to its starting originally from small emboli or thrombi which lead to minute softenings, with a secondary reparative and sclerotic Fig. 208. — Multiple cerebrospinal sclerosis. {Charcot.) process. The fact that the disease follows infective fevers makes such an origin of it seem probable. On the other hand, pathological anatomy does not yet bear out this view, and it is possible that the disease begins by a primary degeneration affecting first the myelin sheaths of the nerve-fibres sparing the axis-cylinders and cell bodies, this being followed by a neurogha and connective-tissue proliferation which ends in the formation of the small islands of sclerosis. An important pathological peculiarity of the process is that, while it destroys the myelin sheaths of the nerves, the axis-cylinders remain intact for a long time, and con- DISEASES OF THE BRAIN 423 sequentlj^ conduction of nerve impulses takes place imperfectly, directly through the nodular masses. Course and Duration. — The disease runs a very irregular course Its prodromal stage is long and remissions of considerable length occur. The disease may last from ten to thirty years, the average duration being five to ten years. Death sometimes occurs from involvement of the nerves of the medulla, but more often from weakness and exhaustion or some intercurrent malady. Diagnosis. — The diagnosis in typical cases is not very difficult; but as, on the other hand, typical cases are not common, the disease has always to be studied with great care before certainty can be reached. The diagnosis is based upon the slow development of the disease, with attacks of vertigo, weakness, parsesthesia and uncertainty in gait; also upon the paralysis of the extremities, the intention tremor, ataxia, rigidity and contractures; upon the disturbances of vision, nystagmus; loss of abdominal reflexes, and the speech troubles. The existence of early paralysis with parsesthesise, or of parsesthesise alone followed by remission, is most significant. The presence of headaches, attacks of vertigo, apoplectiform attacks, and the pecuhar mental condition often furnish help. The age of the patient and the cause should also be taken into consideration. The disease must be distinguished from Friedreich's ataxia, spastic spinal paralysis, locomotor ataxia, dementia paralytica, bulbar paralysis, paralysis agitans, chronic meningitis, and hysteria. The points already given and those furnished under the heads of these different diseases must be utihzed in making these distinctions. In the pseudo-sclerosis of Westphal, there are no ocular symptoms. In diffuse cerebral sclerosis, also, ocular symptoms are rare and there are progressive dementia and paralysis. In multiple sclerosis the pupils are rarely affected. The method of exclusion may be used with advantage in reaching the diagnosis of this protean malady. Prognosis. — The prognosis, whUe not favorable as regards the ulti- mate cure, is somewhat favorable as regards a remission and improve- ment, and the disease on the whole is not so severe as is locomotor ataxia or the other degenerative disorders. A quiet regular life is very necessary, and at certain intervals the patient should go to bed for several days. Systematic re-educational exer- cises are to be used for very short periods. Electricity is of Uttle value except for symptoms. Hypodermic injections of the cacodylates; also those of salvarsan and of fibrolysin are useful. Urotropin may be given for short periods in large doses. Occasional courses of iodides and mer- cury are beneficial. Atropin and belladonna are useful symptomatically. If the case is taken early and the patient works for his cure hopefully and earnestly much may be accomplished. 424 DISEASES OF THE NERVOUS SYSTEM THE APOPLEXIES Apoplexy is a clinical term used to indicate a condition characterized by sudden paralysis, usually attended with loss of consciousness, and due to the breaking or blocking up of a blood-vessel in the brain. Apoplexy is a general term. Particular forms are described in ac- cordance with the cause of the apoplexy. These are: 1. Intracranial hemorrhage, from rupture of a blood-vessel (hemor- rhagic apoplexy). 2. Acute cerebral softening, from embolism or thrombosis (embolic or thrombotic apoplexy). APOPLEXY FROM INTRA-CRANIAL HEMORRHAGE (Cerebral Hemorrhage, Hemiplegia) There are four groups of intra-cranial blood-vessels : those of the dura mater, those of the pia mater, those supplying the basal ganglia and white matter, and those supplying the pons, medulla, and cerebellum. These last are chiefly branches of the vertebrals and are a separate group, subject to somewhat different mechanical conditions. Corre- sponding to this we have: 1. Dural or pachymeningeal hemorrhages. 2. Pial or subarachnoid hemorrhages. 3. Central or capsular hemorrhages. 4. Hemorrhages in the medulla, pons and cerebellum. It is the central hemorrhages (No. 3), due to rupture of the blood- vessels going to the great basal ganglia, internal capsule, and white matter, that constitute the great majority of cerebral hemorrhages seen by the physician. It is this class that I have particularly in mind in the following description. Etiology. — -At the time of birth and during infancy there is a slight tendency to intra-cranial hemorrhage owing to the accidents and injuries of labor. The hemorrhages at birth are usually meningeal and traumatic. After this period the liability is very small, but slowly increases up to the age of forty, when predisposition specially begins. Four-fifths of all cases occur after forty, and the tendency to hemorrhages increases in each decade up to eighty, when it diminishes absolutely and relatively. Males are slightly more predisposed than females. Rather more cases occur in cold weather, at high altitudes, in the temperate zone, and among civilized races. With the improvement in general health condi- tions and the greater average longevity, there is an increase in apoplexies. Hereditary tendencies are most important. This tendency is to arterial degeneration and often runs in families. Excessive eating and drinking. DISEASES OF THE BRAIN 425 excessive work, and anxiety of mind, in fine all the causes leading to arterial disease, predispose to cerebral hemorrhage. Syphilis is more often a cause of thrombosis. It was present in over 20 per cent, of my cases of cerebral hemorrhage, while kidney disease was present in one- third of the cases. Congenital anomalies, such as a narrow thoracic aorta or inherited deficiency in the strength of the walls of the blood-vessels, also play a part. Any sudden physical exertion, such as straining at stool, the ex- citement at coitus or of a passion, eating a large meal and drinking a great deal of fluid, especially alcohol, taking a cold bath, all ma}^ lead to rupture of an artery. Direct blows on the head may cause meningeal hemorrhage, rarely a central hemorrhage, for the central blood-vessels are well protected.^ The symptoms are the prodromal, those of the attack and acute stage, and those of the chronic stage. Prodromal symptoms are rare except in syphilitic cases. When present the patient suffers from headache, insomnia, dizziness, numb- ness of the hand and foot on one side, and a failure of memory for words. He may have "full" feelings or even pain in the head and bad dreams at night. Nosebleed and irregular heart action sometimes occur. Often attack comes on when the patient is feeling particularly well. The attack always comes on suddenly and may be accompanied (1) by convulsions and coma, (2) by coma alone, or (3) it may come without loss of consciousness. 1. Initial convulsions are rare and generally mean a meningeal hemorrhage. They occur, however, in young children. When present they are unilateral or partial, as a rule, but may be general. 2. The common mode of onset is with coma. The patient, without warning, suddenly becomes dizzy, loses consciousness, and falls. The face is flushed, the pulse hard and rather slow, the blood-pressure may rise to over 200 mm., the breathing is labored and stertorous, the cheek on one side puffs out with each expiration, the eyes are partly closed, the eye-balls fixed or deviated to the paralyzed side, the pupils are contracted and rigid, the skin is bathed in sweat, the limbs are relaxed, but some evidence of hemiplegia is present and the Babinski reflex can be usually elicited; the urine may be retained or it and the feces involuntarily evacu- ated. The urine is usually of rather high specific gravity and often con- tains albumin, even when there is no renal disease. The temperature in severe cases may fall below normal during the first twelve hours, even to 96° F., but this is not the rule. It is the rule, however, for the tem- 1 Among 324 cases of hemiplegia occurring in the Cornell clinic, carefully analyzed by Dr. T. W. Evans, he found less than 1 per cent, in which even the question of trauma could be raised. Among 120 private cases, there was a history of a blow and of syphilis in one. 426 DISEASES OF THE NERVOUS SYSTEM perature in a few hours to be K° or 1° higher on the paralyzed than on the sound side. If the case is rapidly fatal, coma continues, respiration often assumes a Cheyne-Stokes character, the pulse becomes fast, the blood tension falls and symptoms of bulbar failure occur; the temperature gradually rises, and usually reaches 102° or 103° F., until just before death, when it may sink again. Swallowing and speech become difficult, hypostatic pneumonia sets in, and the patient dies in from two to four days. In slower fatal cases the pa- tient regains consciousness partially and then enters a condition of stupor or mild delirium. He is restless and suffers from headache. The tempera- ture may continue normal for a time, but is usually higher on the affected side. At the end of two or three weeks it rises higher, pneumonia may develop, the patient becomes uncon- scious, and death ensues. In the favorable cases, which constitute the majority, coma, if present, gradually passes away in from one to six hours, leaving the patient's mind somewhat weak and confused and his speech disturbed, or more rarely the intelU- gence may not be at all disturbed. During the first few days or weeks after the attack the physician finds that the prominent symptom is the hemiplegia. This affects the arm and leg most and the face least. Only the lower two branches of the facial nerve are involved, and the patient can shut the eyes. The tongue, if protruded, turns to the paralyzed side; the uvula is turned in various ways and its position is of no significance. There is often some evidence of cutaneous anaesthesia of the paralyzed side, and less often hemi- anopsia and disturbances of hearing occur. In right-sided hemiplegia the patient, after recovering consciousness, is often unable to talk or to understand what is said. Examination shows that he has a motor or sensory aphasia (vide Aphasia). Fig. 209. — Chronic hemiplegia with contractures. {Curschniann.) DISEASES OF THE BRAIN 427 The deviation of the eyes and head to one side usually disappears in a day or two. Occasionally there is a temporary ptosis. The pupils at j&rst are contracted, that on the paralyzed side the more so; this con- dition disappears with returning consciousness. The paralysis of the arm and leg is usually flaccid at first, and the hmb falls heavily when hfted; the reflexes are lessened or abohshed. In a few days rigidity and exaggeration of the deep reflexes appear on the paralyzed side, with ankle clonus and dorsal flexion of the big toe. Sometimes, however, rigidity sets in at once. This symptom occurs when the blood has broken into the ventricles, and also in some menin- geal hemorrhages. The skin reflexes, particularly the abdominals are lost on the paralyzed side. The usual course of the temperature is for it to rise on the second and third day to 100°F. or 102°F., being 1^° to 1° higher on the paralyzed side. In a few days it gradually falls, so that by the eighth to the tenth day it is normal. If the temperature continues to rise after the fourth or fifth day, it is a sign of an extension or inflammatory reaction of the hemorrhage. Hence the thermometer furnishes a very important criterion of the seriousness of the case. The varying course of the apoplexy is shown in the foUowing diagram: Health line ' 3. Chronic stage. Improvement .\ Attack. 1. Death. 2 to 4 days. 2. Death. 2 to 4 weeks. The Chronic Stage, Hemiplegia. — The description of this condition applies almost equally to all forms of apoplexy with hemiplegia. At the end of a month, if fever and symptoms of cerebral irritation have subsided, the chronic stage may be said to begin. The hemiplegia has improved, the patient can move the leg and arm a little, sensory symptoms have lessened, the mind is clear, headache has disappeared. Improvement continues, though more slowly, for several months or even one or two years. During this time the patient is "a. hemiplegic." The hemiplegia affects the arm more than the leg, and the face least 428 DISEASES OF THE NERVOUS SYSTEM of all. The distal segments of the limbs, the feet and hands, are af- fected more than those near the trunk. The muscles that act bilater- ally, such as those of respiration, phonation, and facial expression, are but shghtly involved. The paralysis is not strictly a hemiplegia, for the muscles on the sound side are somewhat weakened, as tests will show. In severe cases, especially in old people, even the visceral muscles, especially those of the bladder, are weakened. At the onset of^the attack there is sometimes a temporary ''initial" rigidity of the muscles on the paralyzed side, or an "early" rigidity may develop in one or two daj'^s. There always develops at about the beginning of the second week a "late" rigidity. This, which at first is slight, gradually increases, and finally contractures affect the paralyzed limbs. The superficial reflexes, which at first were absent rarely reappear; the tendon reflexes Fig. 210. — Showing athetoid movements of hands. (Curschmann.) become much exaggerated, and clonus can be obtained in the leg and arm. The sound side shares to a small extent in these conditions. The contractures affect the extensors of the foot more than the flexors, and bring the toe down and the heel up. The leg is held nearly extended, and the limb in walking is swung around, the toe scraping the ground. The shoulder is adducted, the forearm flexed, and the fingers are tightly shut into the palm by the overaction of the flexors (Fig. 209). The facial muscles show a slight contraction and drawing to the affected side. The muscles on the paralyzed side do not waste. In infantile hemiplegia, however, the affected limbs grow less than those on the sound side. The paralyzed limbs may be the seat of peculiar disorders of move- ment. These consist of: Associated movements, tremor, ataxia, choreic movements, continu- DISEASES OF THE BRAIN 429 ous or athetoid movements (Fig. 210), spastic movements and cramps. Such movements, aside from those that are spastic, are rarely seen in the hemiplegia of adults. The electrical irritability may be at first shghtly increased or di- minished, but the change is small in amount and never reaches the degenerative stage. The direct muscular irritabilit}'- is at first increased on the affected side. Hemiansesthesia, if present at first, disappears to a great extent, leaving only residua about the feet and hands. Parsesthesise are com- mon. In rare cases the patient suffers great pain in the arm and leg. This pain is generally of a neuralgic or burning character and very obstinate and distressing. Cramping pains in the legs and arms are common in the severer cases. During the first few weeks after the onset, the temperature of the hemiplegia side is usually a very little higher than that of the sound side. Vasomotor disturbances, sweating, skin eruptions, and increased growth of hair are some of the rarer symptoms. The mental condition is sometimes affected. The patient becomes irritable, cries easily, and is in general more emotional. The memory is impaired, and the power of concentrating the attention and carrying on work is less. Sometimes a progressive mental deterioration sets in and epilepsy or insanity develops. The mental disturbance is greater in old people and depends somewhat on the size of the hemorrhage. Those forms which produce serious aphasia especially limit and lessen mental activity. Dural Hemorrhages. — Hemorrhages from the vessels of the dura mater are usually due to a rupture of the middle meningeal artery or vein or some of the cerebral veins as they pass to the sinus, and this is especially true in such hemorrhages as are the result of injuries to the head. The causes are blows on the head, obstetrical injuries, alcoholism, and the dementias. In dural hemorrhages the result of blows on the head, the clot is some- times intra-dural, lying in the arachnoid space, and sometimes epidural, lying between the bone and the dural membrane. The extra-dural hemorrhages are perhaps a little more common in surgical experience; the intra-dural in medical and especially asylum experience. In over one-half of extra-dural cases there is an interval of consciousness lasting from a few hours to two months, but usually only a few hours, between the accident and the time when distinctive cerebral symptoms de- velop. Then the patient gradually becomes dull, somnolent, and finally comatose. Along with the gradual or rather sudden loss of con- sciousness there develops a hemiplegia upon the side opposite the clot. This is usually not complete, though it may become so. It is much less 430 DISEASES OF THE NERVOUS SYSTEM common in extra-dural hemorrhage. Anaesthesia is rarely present. The reflexes are generally somewhat exaggerated, and there may be consider- able rigidity. Spasmodic movements of some kind occur in nearly half the extra-dural cases and in more than half of the intra-dural. These spasmodic movements may involve the whole of the affected side, or may simply affect the eyes and the facial muscles. They consist of irregular twitchings. The pupils are usually somewhat contracted, more so upon the paralyzed side. When there is a dilated pupil on the side of the lesion and a small pupil on the opposite side, it is known as the Hutchinson pupil, and means a severe brain compression involving the third nerve at the base. The eyes are generally both turned toward the affected side and away from the lesion. The pulse is hard, slow and full; the tension high; the respiration is rarely stertorous, though it may sometimes be so, and Cheyne-Stokes respiration may be present. In these cases the clot is very large and the compression great. Aphasia may be present if the clot is upon the left side. The temperature may be raised one or two degrees, or it may be normal. The progress of the disease is usually steadily fatal unless surgical interference is undertaken. The coma deepens, the respiration becomes stertorous and then embar- rassed, the pulse gets rapid and weak, and the patient dies. With surgical interference (since 1886), between two-third and three-fourths of the cases are saved (Scudder and Lund). Subdural hemorrhages not caused by injuries are due sometimes to the rupture of a meningeal artery, but usually to rupture of the veins of the pia mater or to the rupture of new vessels formed in a pachymeningeal exudate. This spontaneous hemorrhage is rare iii ordinary practice, but is not specially so in insane asylums or in large city hospitals. This is be- because the two great causes of this type of hemorrhage are the dementias and alcoholism. General paresis is the form of insanity with which it is of- tenest associated. In the case of alcohohcs, it is probable that injuries from blows are an exciting factor in the production of the hemorrhage, these occurring while the patient is in a state of intoxication. The symptoms of these hemorrhages are extremely variable, owing to the complicating influences of the insanity and alcohol. The patient, after suffering from headaches or vertigo, becomes suddenly comatose and shows marked evidences of hemiplegia and even of hemiansesthesia. Rigidity of the paralyzed side is often present, and sometimes spasmodic movements are observed. On the other hand, at times the paralysis can hardly be ob- served, and the patient is in a semicomatose state, has a muttering delirium, and presents the general aspect of a person suffering from the oedema or "wet-brain" of alcoholics. In dural hemorrhages occurring in paresis, the patient usually without warning becomes unconscious, and he often has some convulsive symptom and a hemiplegia develops. In DISEASES OF THE BRAIN 431 these cases there is often a rapid improvement, and the patient gets partly well, usually experiencing other attacks later. Pial Hemorrhages. — Hemorrhages from the pial arteries are rare and of small extent. The most frequent cause is trauma, associated perhaps with syphilis and alcoholism. In many instances very slight localizing symptoms occur, and no absolute diagnosis can be made. If the hemorrhage, however, is in the motor area of the cortex, local spasmodic movements and some hemiplegia are observed. The most characteristic symptoms are the sudden incomplete hemiplegia, involving, perhaps, mainly an arm or a leg, associated with local spasmodic movements, resembhng Jack- sonian epilepsy. Hemorrhages due to rupture of large pial veins occur most often as obstetrical injuries. The hemorrhages here may cause convulsions, paralysis and death, or lead to permanent mental and physical defects. Hemorrhages of the Vertebral Artery and its Branches. — Hemorrhage in the medulla is extremely rare. If of any size, it causes instant death; if small, it will leave symptoms similar to those of softening. Hemorrhage of the 'pons is also rare, but it makes up 2 per cent. of my cases. The cause is much the same as that for cerebral hemor- rhages, though syphilis is less often a factor. In nearly half the cases the hemorrhage is a large and immediately fatal one. In the majority of cases it is a small focal hemorrhage from rupture of a transverse or median branch of the basilar artery. The patient has some prodromal headaches and malaise for a few days, with vertigo and sometimes vomiting. Then he falls suddenlj^ as if by a lightning stroke, into a coma, usually very profound. There are twitchings of the face or of the limbs or both, but rarely any general convulsion (three cases). The face is flushed, there is a fall of tem- perature and the pulse is irregular, but not markedly increased or slowed; as a rule, the respiration is slow, 4 or 6 per minute, or more often irregular, and of Cheyne-Stokes type. The pupils are con- tracted to a pin-point, and do not respond to light, but may be uneven. There is convergent strabismus or conjugate deviation of the eyes. The limbs are at first stiff, but may be relaxed later and the reflexes increased. The patient cannot be aroused, but can be made to vomit. A few hours later the temperature rises, sometimes very high — 106 to 108 — but usually not. There are involuntary movements of the bowels, swallowing is impossible; evidences of dyspnoea are shown. The pulse becomes more rapid, cyanosis develops, the patient dies in six to twenty hours, usually with evidence of paralysis of respiration. If the hemorrhage is less severe, the coma is less profound or may 432 DISEASES OF THE NERVOUS SYSTEM be absent, the patient shows signs of agonizing suffering and difficulty in respiration, evidences of crossed paralysis, crossed anaesthesia; and disturbance of equilibrium and ataxia can be noted. In still smaller lesions we find no such severe general symptoms, but only those due to a circumscribed focus in one side of the pons, because the smaller lesions are unilateral. The syndrome of serious pons lesions then is : 1. Headache, malaise, vomiting. 2. Sudden and profound coma. 3. Twitching of the face and limbs or both. 4. Myosis and convergent strabismus or conjugate deviation (away from the side of the lesion) . 5. Slow, irregular breathing. 6. Irregular pulse. 7. Dysphagia. 8. Paratysis of limbs or crossed paralysis and exaggerated reflexes. 9. Gradual rise of temperature, sometimes to high point. 10. Death inside of twenty-four hours. Cerebellar Apoplexy. — Hemorrhage into the cerebellum occurs in 1 or 2 per cent, of all fatal cases. Its recognition is very difficult. There is sometimes a preliminary period of severe headache, lasting several days. In other cases the patient at once falls into a state of profound coma, with stertorous respiration. Vomiting sometimes occurs. There may be some hemiplegia, and if so this is on the side of the lesion, owing to this pressure on the motor tract. Distinct evidences of hemiplegia, however, are not always observed. The condition of the pulse and arterial system is very much like that of ordinary apoplexy, but the respiratory system is usually more seriously affected. Disturbances in the movements of the eyes and in swallowing, and in fact all those symptoms which show a pressure or irritation due to blood oozing into the fourth ventricle may be present. Death is almost sure to occur, and is inevitable if the hemorrhage, as is so often the case, breaks through and reaches the fourth ventricle. If the hemorrhage is small and does not break into the ventricle, the patient suffers from intense vertigo which obliges him to lie per- fectly still in a horizontal position. With or preceding this he may fall down and have convulsive movements or forced and irregular tonic contractions of the muscles; in other words, a cerebellar fit. If this passes away the vertigo also may pass, and the patient gets nearly well, but is left with some feeling of uncertainty in gait, and with tinnitus cerebri and tendency to vertiginous and cerebellar attacks. Pathology and Morbid Anatomy. — Spontaneous intra-cranial hemor- rhage is always due to the presence of diseased blood-vessels in the brain. This diseased condition consists of: DISEASES OF THE BRAIN 433 1. A degenerative arteritis which results sometimes in producing small aneurisms. 2. A fatty degeneration of the vessel walls. 3. Besides this, in most cases the larger blood-vessels are atheromatous. The arteritis may produce small or miliary aneurisms which involve only the smaller arteries, especially those of the central group. They may be fusiform or sacculated in shape; they range in size from one- fourth to one millimetre (Koo to 3^^5 in.) in diameter. They are usually not very numerous, but there may be as many as a hundred in the brain. They are the results, not of inflammation, but of a degenera- tion which affects first an area in the internal coat; this causes local weakness and consequent dilatation; secondarily there is a peri-arteritis. These aneurisms occur almost exclusively during the degenerative period of life. Fatty degeneration of the walls of the small cerebral arteries occurs in purpura, scurvy, marasmic conditions, and post-infective states, especi- ally in early life, and is the common cause of hemorrhage at that time. Atheroma affects the larger vessels only. It is indirectly a cause of hemorrhage by lessening the elasticity of the vessel wall. Atheroma is present in from one-eighth to one-fifth of all cases. Hypertrophy of the heart is a factor in causing hemorrhage, and such hypertrophy exists in about 40 per cent, of cases. Emboli lodged in the cerebral arteries may cause hemorrhage by suddenly stopping the arterial circulation and raising the blood-pressure. Hemorrhages are found by far the oftenest (20 per cent.) in the caudate and lenticular nuclei and adjacent parts. The lenticular and lenticulo-striate branches of the middle cere- bral are oftenest affected; next the branches of the anterior cerebral to the caudate nucleus and the lenticulo-optic branches of the middle cerebral. The branches of the posterior cerebral break more rarely. The parts affected in hemorrhage in fatal hospital cases, in order of frequency, are about as follows (Hoobler). Frontal lobe 24 Parietal lobe 14 Occipital lobe 6 Temporal lobe 14 Corpus striatum 62 Optic thalamus 25 Internal capsule 28 Ventricles 64 Corpus callosum 1 Corpora quadrigemina 1 Pise and cortical 8 Pachymeningeal 7 Pons 1 Cerebellum 3 28 258 434 DISEASES OP THE NEEVOUS SYSTEM These figures show that over three-fourths of all the hemorrhages occur from rupture of the central or ganglionic arteries. Cortex hemorrhages are generally small and may be subarachnoid or may break through into the arachnoid cavity. Ventricular hemor- rhages are almost always secondary to a rupture into the neighborhood of the basal ganglia. Pons hemorrhages occur usually in the median line. Cerebellar hemorrhages are oftenest due to rupture of the superior cerebellar artery. They usually cleave their way externally and break into the fourth ventricle. Dural hemorrhages are due to rupture of the meningeal veins and arteries and of the vessels in newly organized clots. They lie in the arachnoid cavity and flatten the convolutions. The reparative changes after a hemorrhage take the following course : 1st. Coagulation of the blood, which in a few days begins to soften and become absorbed. 2d. Formation of a fibrinous wall about the clot. This occurs from the seventh to the ninth day. 3d. Formation of a cyst with transparent fluid contents, and perhaps fibrous trabeculse running through it, twentieth to thirtieth day. 4th. Contraction of the cyst wall, which begins by the fortieth day. 5th. Secondary degenerations begin from the tenth to the fourteenth day. These de- generations vary with the location of the lesion. The most important one is that of the pyramidal tract. This is more or less completely sclerosed from the lesion to the spinal cord. The diagnosis of hemorrhagic apoplexy must be made from al- coholic coma, ursemic coma, diabetic, opium and other types of coma, epilepsy and hysteria; acute softening from embolism and thrombosis. From alcoholic coma the diagnosis is made by the odor of the breath, the incomplete coma, the equal pupils, the absence of low or unequal temperature, the absence of paralysis, the equality of the reflexes. From ursemic coma by the presence of albumin and casts in the urine, though their presence does not surely indicate ursemia; by the equal pupils, the temperature, the absence of hemiplegia and the presence of the physiognomy peculiar to cases of chronic Bright's disease. From diabetic coma by the odor of the breath and the condition of the urine. From opium poisoning by the history, the stomach contents, the presence of equal and contracted pupils, the slow respirations, the temperature, and the absence of paralysis. From epilepsy by the history of the onset with epileptic cry, the dilated and equal pupils, the biting of the tongue, the absence of hemi- plegia, the rather rapid return of consciousness. Hysterical attacks present little semblance to that of apoplexy; consciousness is not profoundly lost; the deep and superficial reflexes are not greatly changed; the hemiplegia is characterized by its flaccidity, DISEASES OF THE BRAIN 435 by its not involving the face, and by the presence of the ansesthesias and other hysterical stigmata.^ In embolic softening the earlier age of the patient, the presence of decided valvular heart disease, the parturient condition, the slighter degree and shorter duration of coma, the absence of serious disturbance of temperature, the onset first of paralysis and then of convulsive move- ments and coma — all lead to a presumption in favor of embolism. The presence, on the other hand, of a congested face, tense pulse, and throbbing carotids favors the existence of a hemorrhage. From thrombotic softening diagnosis is more difficult. The exist- ence of syphilis is in favor of thrombosis. The occurrence of pro- dromata, consisting of slight seizures quickly recovered from, the slighter degree of coma, the advanced age, hard atheromatous arteries, low blood-pressure, evidence of ansemia and asthenia, weak or fatty heart, the absence of stertorous respiration, flushed face, and unequal tem- perature not much lowered or raised, the slight pupillary disturbance, and absence of convulsions point to thrombotic softening. Evidence of a lesion in the pons or cerebellum unless there is syphilis suggests hemorrhage, while evidence of lesion in the medulla points almost surely to softening. The existence of a very high blood-pressure will often help the diagnosis in all cases, but especially from softenings. Several authors have found large granular macrophages containing red blood-cells in the cerebrospinal fluid in cerebral hemorrhage, but these are not always found (Hastings). The examination of the urine for haemoglobin has also been used as a diagnostic test. Lumbar puncture and the finding of blood in the cerebro-spinal fluid indicate a ventricular or surface hemorrhage. The chances in any case between the ages of thirty and fifty, in a city hospital practice, if there is no heart disease, are six to one in favor of hemorrhage, if syphilis can be excluded. The diagnosis of the location of the lesion has already been indicated, except for the central hemorrhages. These represent four clinical types: 1. That due to rupture of the lenticulo-striate branch of the middle cerebral with injury of the lenticular region and internal capsule in its more anterior part. This gives the picture of the usual type of hemiplegia as described with no marked sensory symptoms. 2. If the lenticulo-optic is involved, there is less hemiplegia and the leg is more involved, and there is involvement of sensation. '^ Hoover's sign of hemiplegia: The patient lies on his back. The examiner places his hand under the patient's heel on the side of the paralysis and directs him to lift the sound leg. In an organic hemiplegia the examiner feels that the patient's heel presses down against his hand, when the patient lifts the sound leg. This does not occur in hysterical hemiplegia, nor upon lifting the paretic leg, nor in the normal subject. 436 DISEASES OF THE NEEVOUS SYSTEM 3. If the thalamic arteries are involved, there is a temporary hemi- plegia with appearance of the thalamic syndrome. 4. Ventricular hemorrhages are usually the result of very extensive lenticulo-striate lesions, the blood finally bursting into a lateral ventricle and thence permeating into all the ventricles and even to the subarach- noid space, so that a diagnosis can be made or confirmed by a lumbar puncture. The symptoms are characterized by the severity of the coma, giving way of the medullary centres, a motor restlessness, increased rigidity of the limbs, and sometimes evidence of bilateral paralysis. The contracted pupils, loss of power over the sphincters, sweating, vasomotor disturbances, rise of temperature, irregular respirations, and Poster! oi communicatin" Antei'ior choroi < I Post, cerebral 1^ V \ /'■"^""/i/-yfr/y,>r A. Fig. 211. — Blood-supply of basal ganglia and internal capsule. {Bing.) rapid pulse indicate a serious cerebral lesion. The blood-pressure, which is high when there is a large clot on the brain substance, falls if it breaks into the ventricles. Prognosis. — The majority of cases apparently get over the first attack of apoplexy, but it is possible that many of these cases are thrombotic rather than hemorrhagic. My view, however, is that since the majority of hospital cases recover and since the majority of these are hemorrhagic there is a large number of recoveries. They are very liable to have an- other within one to five years. The minority recover from this. Few survive a third attack. The prognosis of the attack itself depends on the severity of the coma and paralysis, the disturbance of temperature and of DISEASES OF THE BRAIN 437 respiration, the evidence of rupture into the ventricles, the development of decubitus, the continuance of loss of control over the bowels and bladder. If profound coma continues four days there is little hope; if fever develops and continues steadily, or if there is initial subnormal tempera- ture, or if signs of bulbar compression and paralysis occur, the progonosis is grave. If the patient passes the first week with little or no fever and con- sciousness has returned, the prognosis is good. The presence of renal disease and of alcoholism is bad. Development of slight delirium which continues is unfavorable. Cerebellar and pons hemorrhages are very fatal, meningeal less so. The prognosis of the chronic stage has been given under symptoms. Improvement continues rather rapidly for three months, then very slowly. Im- provement may continue for one or two years. Complete recovery is very rare. The great danger after middle age is recur- rence of the attack. Something of the course of the apo- plexies in a large city hospital is shown by the following statistics: In three recent years there were ad- mitted to Belle vue Hospital 315 cases of cerebral apoplexy with hemiplegia. Of these, 115 died and of these 26 were autopsied. In 10 cases the hemorrhage involved the central arteries and broke into the lateral ventricles. In 16 the len- ticular region and capsule only were in- volved. Treatment of the Attack. — The patient should be laid in a horizontal position and kept quiet. Ice may be applied to the head and hot bottles at the feet, only if these measures seem indicated. They usually do no good. A laxative should be given, either one or two drops of croton oil or a quarter of a grain of elaterium. If there is evidence of intense cerebral congestion, the pulse being very full and hard and the heart beating strongly and blood-pressure high, bleeding eight to twelve ounces is justifiable. Ordinarily it is better to give tincture of aconite every twenty minutes for two or three hours. Fig. 212 . — D i a g r a m m a t i c representation of the internal capsule and the cortical tracts that pass through it. 1, 2, Fronto-occipito-pontine efferent; a, V, auditory, visual, afferent; F, cortico-spinal from face center; H, hand; A, arm; B, leg; *S, sen- sory tract. {Bing.) C, T, L, and the internal capsule are the parts most often involved in hemorrhage. 438 DISEASES OF THE NEKVOUS SYSTEM Administration of bromide of sodium and enemata of ergot have been advised, but are of doubtful value. After the first twelve hours, treat- ment must be symptomatic. Should delirium and other evidence of mental irritation appear, chloral hydrate or morphine and hyoscin are indicated, and an elaterium purge, given if the patient is not too weak. The use of iodide of potassium or mercury is not indicated unless the case is distinctly syphilitic. Great care should now be taken that the patient does not develop pneumonia. The mouth and pharynx should be cleansed antiseptically, and the patient should not be allowed to re- main in one position. If there is sufficient evidence of a meningeal or cortical clot, trephining should be seriously considered. At the end of three or four weeks or even earlier careful massage should be begun and continued daily. With it the faradic battery may be used on the affected limbs. When contractures develop the stabile galvanic current may be tried, though it does little good. Static sparks, however, are helpful; lukewarm baths should be tried and measures used to produce a hyperextension of the affected parts. Internally during this time the patient is to be given courses of iodide of potassium, tonics, and laxatives if needed. The patient should be made to live a quiet life, preferably in a warm, equable climate. The kidneys should be kept active and arterial tension only moderately low. For these purposes nitroglycerine should be given and at times small doses of chloral, and the diet should be simple and rather non- nitrogenous. Strychnine in very small doses (gr. Hoo) sometimes helps the contractures, as also do the bromides and phj^sostigma. APOPLEXY FROM ACUTE SOFTENING {Embolism, Thrombosis) Acute softening is a condition caused by the plugging of a blood- vessel with an embolus or thrombus, and is characterized by a com- paratively sudden (embolism), or gradual (thrombus), apoplectic seizure, the symptoms eventually running a course much like that of cerebral hemorrhage. Etiology. — Embolism occurs rather more often in women, throm- bosis in men. Embolism is rare in children; it occurs oftenest between the ages of twenty and fifty, thrombosis between the ages of fifty and seventy. The most important predisposing factors in embolism are acute or recurrent endocarditis, infectious fevers, profound anaemia, pregnancy, and blood dyscrasias; in thrombosis, arterial sclerosis from any cause. Severe gas poisoning leads sometimes to thromboses. The same causes which lead to the arterial disease which produces cere- bral hemorrhage also predispose to thrombosis, though in the latter condition atheroma and obliterating arteritis play the important part. DISEASES OF THE BEAIN 439 Symptoms. — In embolism there are rarely any premonitory symp- toms; the onset is sudden; it may begin with some convulsive twitch- ings, then follow hemiplegia and temporary loss of consciousness. Coma, however, is rarer than in hemorrhage, and if present is usually shorter. There is rarely vomiting, nor do we find the hard, pulsating arteries, flushed face, and severely stertorous breathing. The initial temperature changes are slight, but in a few days fever may develop. In thrombosis premonitory symptoms are frequent. In syphilitic cases there are headaches and cranial-nerve palsies. In these and other cases vertigo, temporary aphasia, insomnia, transient hemiplegia, numb- ness of the hand and foot, and drowsiness may be present. The onset is more gradual; the hemiplegia slowly develops, taking several hours, or days and coming on with remissions, finally the patient gradually becomes completely hemiplegic often without coma. The attack some- times is rather sudden, with no loss of consciousness, and it may occur in sleep. The temperature often has a sHght initial fall, f oUowed by a rise, just as in hemorrhage. In both embolism and thrombosis the hemi- plegia tends to improve very much in a few days or weeks unless the vessel obliterated is a large one. Embolism is rather more apt to affect the left side of the brain, though the difference is not great. The middle cerebrals are most frequently affected. Thromboses affect the vertebrals, basilar, and posterior cerebral arteries more often relatively than do hemorrhages; then the initial symptoms may not present the character of hemiplegia, but of a ponto-bulbar paralysis or sensory hemiplegia. Acute softening may kill within twenty-four hours, but, as a rule, the patient survives the onset, and if he dies it is not for several weeks. After the acute stage is over the patient passes into the chronic stage, which resembles in nearly all respects that of hemorrhage. After an acute softening, however, it is believed that there are more spastic symptoms and a greater tendency to mobile spasm. In embolism, owing to the youth and freedom from arterial disease, the mind is less affected; while in thrombosis the contrary is the case. In acute softening from gas-poisoning, a branch of the anterior cerebral artery which supplies the caudate and part of the lenticular nuclei is particularly affected so that a symmetrical softening of both lenticular nuclei is almost a characteristic of this accident. This is accompanied with coma and vasomotor and trophic disturbances of the limbs at times. In less severe cases there are disturbances of speech, tremor, irregular jerky movements and a temporary paraplegia with involvement of the deep reflexes. Intermittent Hemiplegia (hemiplegia sine materia). Not rarely there occur attacks of hemiplegia which last only a few hours or a day or two. There is often with them mental confusion and aphasia. These attacks 440 DISEASES OF THE NERVOUS SYSTEM are seen oftenest in cerebral lues; occasionally in epilepsy and migraine; and in other conditions in which there are arterio-renal defects. It is supposed that in some cases the paralysis is due to a local spasm of the cerebral blood-vessels (e.g., migraine); in other cases to the temporary activity of an obliterating arteritis (lues); or in other cases to a local oedema perhaps associated with a uraemia or other toxic irritation. Pathology. — The embolic plug cuts off the blood-supply from a certain area of brain tissue. In twenty-four hours this begins to soften. If the area is in the cortex it becomes red (red softening); if in the white and less vascular part, it is usually white with a few red punctate spots. The red softening gradually becomes yellow (yellow softening). The dead tissue gradually is absorbed, leaving a cicatrix or cyst. If the embolus contains infective microbes, there may be a local encephalitis and abscess. In thrombosis there are usually evidences of extensive atheroma or syphilitic arteritis. In those instances in which the thrombosis is caused by the blood state and a weak heart, little arterial change occurs. Athe- roma affects chiefly the internal carotids and the large arteries at the base, viz., the middle, anterior, and posterior cerebrals and the basilar and vertebrals. The branch of the anterior cerebral supplying the caudate and part of the lenticular nucleus is called "the artery of cerebral thrombosis." Among seventy-two cases of cerebral softening with autopsy collected by Dr. B. R. Hoobler from hospital records, the site of the lesion was as follows: Frontal lobe 6 Parietal lobe 17 Occipital lobe 4 Temporal lobe 9 Caudate nucleus 5 Lenticular nucleus 16 Optic thalamus 6 Internal capsule 6 Corpus callosum 2 Pons medulla 1 72 This shows that about half (35) involve the central and half the cortical arteries. The posterior cerebral is involved in about one-third of the cases. The parietal lobe and the caudate nucleus are most often affected. Most of these cases are thrombotic. Embolism almost always affects the great basal ganglia or some cortical branch of the anterior and middle cerebrals. The secondary changes after thrombosis resemble those after em- bolism; a thrombus, however, may lead to supplementary embolism DISEASES OF THE BRAIN 441 through breaking off of a clot, and both conditions may cause a complicat- ing cerebral hemorrhage. The Diagnosis. — The important points have been gone over under the head of hemorrhage. Very high blood-pressure, evidence of marked intra-cranial pressure, very sudden onset with profound coma, respiratory embarrassment, paralysis and fatal termination point to hemorrhage. Increased blood- pressure and temperature on the paralyzed side suggest hemorrhage. Lumbar puncture and withdrawal of cerebrospinal fluid may give definite eivdence of hemorrhage, but will not exclude it. Premonitory symptoms covering one or more days indicate softening. A preceding period of feeling unusually well precedes some acute and fatal hemorrhages. Slow steady loss of consciousness ending in very deep coma indicates hemorrhage. Recurrent mild apoplectic seizures are probably always thrombotic. Very slowly developing seizures, coming on over a period of several days are thrombotic. Embolism is distinguished from thrombosis by the age, the presence of endocarditis, of the puerperium or septic fever, and by its sudden onset, with perhaps some convulsive movements. The prognosis as regards the attack is somewhat better than in hemor- rhage, as a rule. In embolism it is good as regards recurrence; in throm- bosis, bad, unless the trouble be due to syphilis or some preventable cause like gas poisoning. The recovery from attacks is more complete in acute softening. After the chronic stage is reached, however, the prognosis is about the same in all forms. The treatment of the attacks consists essentially in rest and such attention to the bowels, kidneys, and heart as may be indicated. In thrombosis it may be important to give heart stimulants and arterial depressants, and for this purpose I advise the use of alcohol and of digi- talis, or strophanthus with nitroglycerin. Thrombosis is much associated with syphilis and treatment must be given accordingly. It is also often associated with weakened and senile conditions and here individual treatment involving tonic measures, and rest are indicated. The symptomatic treatment of the chronic stage is the same as in hemorrhage. SPECIAL FORMS OF HEMORRHAGIC OR THROMBOTIC SOFTENING Small or large foci of softening attack the brain in different parts causing many symptom groups. A description of these is given under the head of softenings, as this is the most frequent condition. Pseudo-bulbar paralysis or cerebral bulbar paralysis is a condition 442 DISEASES OF THE NEKVOUS SYSTEM characterized by double hemiplegia with involvement of the bulbar centres, so that there is difficulty in speech, mastication and swallowing. It is caused by a double lesion usually a softening involving the motor paths between the cortex and the nuclei in the centers of the motor V and of the VII, IX, XII nerves. Very rarely a one-sided lesion may cause this condition. The condition may occur in the aged and is then due to lacunar degeneration and softening. As one sees it in the clinic and hospital, pseudo-bulbar paralysis occurs oftenest as the result of a second hemiplegic attack affecting the sound side of the brain in an old hemiplegia. Occasionally it comes on suddenly and is caused by acute bilateral vascular lesions. The seat of the lesions is usually in the neighborhood of the lenticular nuclei, and they are so located that they involve the fibres from the lower third of the motor cortex where the centres for the throat, tongue and face lie. The consequence is that there is no great involvement of the arms or legs. The condition is a rare one. The symptoms are much like those of bulbar paralysis, but the diagnosis is easily made by consideration of the history, the absence of muscular atrophy and fibrillary tremor, and the marked involvement of the pyramidal tracts. The disease tends to improve rather than progress, as in true bulbar palsy. Thalamus Apoplexy. — A hemorrhage here causes usually a temporary hemiplegia, with anaesthesia, which latter symptom continues. There may be later dysthesise and pains of the extremities and perhaps athetoid or choreic movements. Sometimes there is hemianopsia; generally the deep reflexes are not exaggerated. A group of symptoms has been noted forming the thalamic syndrome. It consists of: 1. Hemiansesthesia. 2. Hemiataxia. 3. Involuntary and spontaneous movements. 4. Central pains. 5. Cutaneous hypersesthesia of the affected side, with overaction to pleasurable stimuli. 6. Hemianopsia; visual hallucinations. In some cases there is paralysis of emotional facial expression on the affected side, while voluntary control of the facial muscles is retained. Mid-brain softenings are not very rare. They are generally luetic or arterio-sclerotic. Hemorrhage and tumors are less common. Many different groups of symptoms may result. The accompanying Fig. 213 from Edinger, in connection with Fig. 214, shows how the different ''syndromes" result in accordance with the location of the lesion. (See also Tumors of the Brain.) Peduncular hemiplegia is usually due to a tumor, but there may be a softening, from a luetic meningitis or thrombosis. The lesionhere DISEASES OF THE BRAIN 443 causes a paralysis of the third nerve on the side of the lesion and hemi- plegia of the opposite side (Weber's syndrome). Softening of the Pons. — Softening in the pons is most apt to be in the ventral part, near the raphe and next to this in the dorsal part of the pons. The basilar artery or its transverse branches are involved. Lesions of the pons alone are rarer than those involving also the medulla, especially its upper part, because the basilar is not plugged as often as the vertebrals and its branches. The prodi'omal symptoms are not so long. In a typical attack due to plugging of the basilar, we get a softening of the deeper ventral parts of the pons. The patient suffers from hemiplegia, with some paresis on the opposite side to the hemi- Fig. 213. — ^Lesions of the midbrain and resulting symptoms. 2. Hemianopsia; 3. disturbances of pupillary light-reflex; 4. ipso-lateral paralysis of eye-muscles, contra-lateral hemiansesthesia, forced movements, tremors; 5, as in 4 plus crossed hemiplegia; 6, crossed motor and sensory disturbance with slight oculo-motor symp- toms on same side; 7, one-sided or double-sided oculo-motor paralysis, complete dila- tation of pupils, amblyopia; 1.8, hemiansesthesia, central pain, visual hallucinations. plegia. There is great exaggeration of reflexes and twitching spasms and coarse, irregular, jerky movements of the limbs, but no convul- sion. There is usually anaesthesia and ataxia on the side of the lesion, and the anaesthesia is dissociated. There is often forced weeping and laughter and dysarthria may occur. Forced movements are not common. When the lesion is high up and involves some of the transverse branches of the basilar, we get oculo-motor palsies and spasmodic disorders on the side of the lesion and paralysis and anaesthesias on the opposite side. When lower down, we get fifth and seventh nerve palsies on one side and hemiplegia on the other. ^ In basilar artery lesion, if the injury is large and severe, the patient develops disorders of deglutition and speech and ^Foville's Paralysis. — A "crossed hemiplegia" with paralysis of the sixth and seventh nerves on the side of the lesion and paralysis of the extremities on the opposite side. 444 DISEASES OF THE NERVOUS SYSTEM extension of the process and dies from paralysis of the bulbar centres. No great disturbances of temperature occur, nor are there marked pupillary symptoms. In the smaller lesions due to involvement of the transverse branches recovery may occur, leaving the patient with a crossed paralysis. Mid-brain, t' 1 To cerebellum. To cerebellum. Medulla. Cord. Direct cerebellar tract. Spino-cerebellar (Gower's tract). Temperature and pain tract. Tactile. Tactile. Muscle sense. Fig. 214. — Showing how le,sion8 in medulla and pons may cause focal symptoms by cutting off tracts and destroying cranial nerve nuclei. The motor tracts lie deep be- low the sensory and begin to cross in the lower medulla and cord. They are not indicated here. When the lesion is such as to cause paralysis of the facial nerve on the side of the lesion and hemiplegia on the opposite side, we have what is called the Millard-Gubler syndrome. Softening of the Medulla.- — Softening is rare, but much more frequent than hemorrhage. It occurs at the early apoplectic age — thirty to fifty, oftener in males, and is due to lues and the arterial changes associated DISEASES OF THE BRAIN 445 with alcoholism and nephritis. It has been observed as a result of work- ing in compressed air. A small lesion may cause tetraplegia; or it may be low down involving the leg fibres of the pyramidal tract after they have crossed and the arm fibres before they have crossed. This causes paralysis of the arm on the contralateral side, of the leg on the homo-lateral side (hemiplegia cruciata).^ A rather special syndrome is caused by thrombosis of the inferior cerebellar artery. The symptoms may come on slowly almost like a tumor. Finally a seizure occurs with symptoms which vary somewhat, but are about as in the following table: Homolateral side Contralateral side Paralysis Hemiplegia. of soft palate Cutaneous anaesthesia for pain and of vocal cord temperature of Trigeminus of facial of sympathetic Cerebellar ataxia + + + I Ataxia ? The cause of the symptoms is shown in Fig. 214. The tracts for muscular and tactile sensations have not altogether crossed in the medulla, hence we may get some evidence of ataxia on both sides. But the cerebellar tracts and peduncle injured have not crossed. CHRONIC PROGRESSIVE SOFTENING OF THE BRAIN Sometimes thrombotic processes affect especially and successively the smaller central arteries of the circle of Willis and lead to a gradual softening. With this process there develops a gradual hemiplegia coming on perhaps in stages during the course of six months or a year. With the hemiplegia there may be headaches, vertigo, paresthesia and anaesthesia, dysarthria with some mental deterioration. The pro- gressive character of the disease suggests a brain tumor or abscess. In very severe cases there may be optic neuritis and atrophy. Usually, after reaching a certain stage, the disease is arrested and the patient remains about the same for some years. ^ Hughlings- Jackson's syndrome is a condition produced by medullary lesions in which there is hemi-atrophy of the tongue and paralysis of the trapezius, sterno- mastoid, vocal cord and soft palate, all on the same side. 446 DISEASES OF THE NERVOUS SYSTEM Senile hemiplegia.- — -Marie has described a condition known as lacunar degeneration. It is characterized by the presence of cavities of various sizes (J^f o to ^^ inch) and irregular contour distributed through the brain. Through these cavities runs a sclerotic but permeable blood-vessel. The softening is not thrombotic but due to the disease of the vessel wall and interference with the nutrition by the ordinary peri-vascular exchanges. The presence of lacunar degeneration leads to attacks of hemiplegia which are usually mild and sometimes temporary. They may not be accompanied with loss of consciousness or abolition of the skin reflexes. Lacunar degeneration thus causes a hemiplegia of the aged. Lacunar degeneration, associated with arterial sclerosis is one of the factors in chronic senile paraplegia, characterized by short-step gait {demanche a petit pas). THROMBOSIS OF THE CEREBRAL SINUSES Sinus thrombosis may be non-inflammatory or inflammatory. Non- inflammatory thrombosis is due to a weak heart and usually occurs in children under the age of one year, in very old people, or at the end of exhausting diseases. Inflammatory sinus thrombosis is due to some neighboring bone disease, especially otitic disease. However, any disease of the bones of the skull and especially of the orbital and nasal cavities, may give rise to the thrombosis as nearly all the sinuses receive a small venous supply from the exterior of the skull. Sinus thrombosis from exhausted conditions and weak heart affects usually the longitudinal sinus. Otitic thrombosis affects the lateral (transverse) superior and inferior petrosal sinuses. Orbital thrombosis affects especially the cavernous sinus. The general symptoms of sinus thrombosis vary greatly according to the part affected, the extent of the obstruction, the primary cause, and the complications. There are, therefore, symptoms of cardiac weakness and marasmus, or of otitic, orbital, nasal or other cranial bone disease, with corresponding local and general symptoms of infection. The cerebral symptoms vary also but resemble those of an acute meningitis, or of a focal hemorrhage or softening. There occur head- ache, vomiting, stiff neck, delirium, coma, convulsions, hemiplegia, apha- sia. There is rarely temperature from the thrombosis alone. Some of the special sinus symptoms are as follows: In cavernous sinus thrombosis, there are dilated facial veins, pro- trusion of the eyeballs with orbital oedema, due to swelling of the retro- bulbar veins, sometimes choked disc, supra-orbital pain and involvement of the motor nerves of the eye (Fig. 215). DISEASES OF THE BRAIN 447 In thrombosis of the lateral (transverse) sinus, there is swelling and oedema over and about the mastoid, with tenderness in this region and along the jugular vein, in which a clot may be felt. There is pain locally and on lateral movement of the head, and on swallowing. If the thrombosis is not septic or complete and solid the subjective symptoms may be slight. If it is a septic thrombosis there are symptoms of general infection, and of cerebral involvement. These latter most often resemble those of meningitis, though lumbar puncture will not show lymphocytosis. Fig. 215. — Exophthalmus, thrombosis of cavernous sinus. {Weldebrandt and Sanger.) In thrombosis of the longitudinal sinus there is swelling especially of the frontal and parietal veins, causing the caput Medusce; sometimes nose-bleed, and in infants distended fontanelles. The prognosis in primary thrombosis is not good, but cases may recover. In infective thrombosis, the prognosis is very serious unless there can be surgical interference. The treatment of sinus thrombosis is symptomatic and surgical. Its details are largely in the hands of otologists, rhinologists and general surgeons. CEREBRAL PALSIES OF CHILDREN— INFANTILE HEMIPLEGIA AND DIPLEGIA The brain palsies of early life show themselves in the form of (1) hemiplegias; (2) diplegias or double hemiplegias, in which both sides of the body are involved; and (3) paraplegias, in which the lower limbs 448 DISEASES OF THE NERVOUS SYSTEM are chiefly or entirely involved. In these palsies, as in the same troubles of adult life, the loss of motor power is always accompanied by a rigidity and by some contractures and exaggeration of reflexes, in this respect distinguishing these paralyses from those of spinal origin. The seat of lesion in these cases is in the hemispheres of the brain, and it is the upper motor neurons which are involved; that is to say, that part of the direct motor tract which extends from the brain cortex down to the spinal cord as far as the anterior horns. The brain palsies of children are there- fore disorders of the cortico-spinal neurons, while the spinal palsies of children are disorders of the lower neurons. I have reserved the term Little's disease for those cases of cerebral diplegia due to agenesis of the pyramidal tracts. Etiology.- — The disease occurs rather oftener in males than in females, though the difference is slight. The vast majority occur in the first three years of life; about one-third of them are congenital. Injuries to the mother during the time of pregnancy, possibly diseases and emo- tional disturbances at this time, are factors in producing the congenital cases. Those cases that occur at the time of birth are due to tedious labor, the use of forceps, and other injuries at the time of parturition. After birth, the causes are those which lead to the production of intra- cranial hemorrhages, embolism, and thrombosis; these being injuries and the infectious fevers. Of the latter, pneumonia, encephalitis, whoop- ing-cough, measles, and scarlet fever are the most prominent. Syphilis is a cause in 15 to 20 per cent.; cerebrospinal meningitis and epileptic convulsions are also occasional causes. Symptomatology. — The disorder in about one-fourth of the cases begins with a convulsion, which may be unilateral, but is usually general in character, and may last for several hours. At the same time a febrile process develops, and this continues for several days. When these acute symptoms have subsided, or before this, it is noticed that the child is paralyzed upon one side, the paralysis involving the arm, leg, and face, as in adult hemiplegia, or perhaps involving both sides. This paralysis undergoes gradual improvement, the face recovering earliest and most, the leg next, and the arm least. As the child develops it is found that the paralyzed side fails to grow as fast as the other, and there may be from one-half to one inch or two inches of shortening in the arm or leg. The circumference of the limbs is less, the surface somewhat colder, and some vasomotor disturbance may be present. With the progress of the case a rigidity of the affected limbs develops; the heel becomes drawn up, so that there is talipes equino-varus or equino-valgus. The flexors of the forearm and of the wrist and fingers contract, as do also the adductors of the thighs. In general it will be found that there is a contraction of the flexors and adductors of the affected limbs. With this rigidity and the DISEASES OF THE BEAIN 449 contractures there are exaggeration of reflexes and clonus in most cases. In the disordered Hmbs pecuHar mobile spasms develop. These consist of athetoid, choreic, and ataxic movements, also sometimes tremors and associated movements. The choreic and athetoid movements are the most common. Along with the appearance of these symptoms it is noticed that there are disturbances in the mental condition of the child. It is usually back- ward in development, this backwardness ranging from simply feeble- mindedness to complete idiocy. Taking all cases, there is about an equal division between feeble-mindedness, imbecility, and idiocy (Sachs). Perhaps a little over one-fourth of the subjects have a fair intelligence. There is usually slowness in learning to talk, and in a small proportion of cases there is a decided aphasia. Such condition is rather more frequent with right hemiplegia than with left hemiplegia, though the rule is not an absolute one. In connection with the mental defect there may develop many of the peculiar moral traits associated with idiocy and low degrees of intelligence. Epilepsy very frequently complicates the disease; nearly one-half of the subjects suffer from this trouble. This epilepsy is in most cases general in character; in a few cases it takes the Jacksonian type, in a small number petit mal alone is noted. Examination of this class of sufferers reveals, aside from the paralysis described, various evi- dences of defective development. These are known as stigmata of de- generation; though they cannot be classed strictly among such, since they are acquired stigmata in most cases, rather than marks which are the result of primary deficiency in development. These stigmata con- sist of a microcephalic or a macrocephalic skull, cranial and facial asym- metry, prognathism, imperfectly developed teeth, and a high palatal arch. It has been found that, as a rule, in cases of cerebral hemiplegias of childhood the patient eventually has a slight flattening of the skull on the side of the lesion (Fisher and Peterson) . Finally, in a few cases there may be found defects in the special senses, such as imperfect hearing, deafness, deaf-mutism, and defects in vision, such as hemianopsia, and perhaps imperfections in smell and taste. Anaesthesia is never observed. The symptoms in the cerebral palsies of children, having passed the acute stage and having become somewhat ameliorated, enter into a chronic stage. This chronic stage begins within a few months after birth or after the onset of the disease. No great change occurs in the paralyses as the child grows older until he reaches the time of puberty, though there is a slight improvement in most cases. After the time of puberty, if the mental condition of the child is good, the physical symp- toms are apt to improve considerably. Morbid Anatomy .^ — The primary changes that lead to the cerebral palsies of children are: 1st, simple agenesis or lack of brain development, 29 450 DISEASES OF THE NERVOUS SYSTEM producing localized atrophy of the cerebrum and the condition known as porencephalus, a condition in which, owing to a congenital defect in nutri- tion, a cavity or depression exists in the cerebral hemispheres, this cavity reaching generally into the lateral ventricle. True porencephalus is found in about one-fourth of the cases, though no definite statistics can be given, owing to the different interpretations given to this term; 2d, hemorrhage, which is probably the most frequent of the single causes; 3d, embolism; 4th, thrombosis; 5th, meningo-encephalitis and polio- encephalitis; 6th, a diffuse cortical sclerosis. Many other terms are used to describe the pathological conditions found at the basis of the Fig. 216. — Atrophied brain with sclerosis and a cyst, from a case of infantile cerebral hemiplegia. brain palsies of children, but the principal causes of all are undoubtedly, as has been described, hemorrhages, encephalitis and softenings, and a defective development or agenesis. It is probable that in the hemi- plegics the original lesion is generally a central hemorrhage, less often a meningeal hemorrhage. After this, probably the most frequent con- dition is a porencephalus from some intra-uterine accident, which may have been defective nutrition causing anaemia and softening, or hemor- rhage, or thrombosis. Acute encephalitis or inflammation of the brain of the kind similar to poliomyelitis is a frequent cause. In double hemiplegias or diplegias of children the cause is in the vast majority of cases a hemorrhage due to some injury or disturbance at the time of labor. DISEASES OF THE BRAIN 451 In other cases of diplegia the lesion isadoubleporencephalus, which maybe either the result of an intra-uterine hemorrhage or simply a defective de- velopment. In the paraplegias the lesion is probably very much the same as in the diplegias, that is to say, either a meningeal hemorrhage or a brain agenesis. Occasionally a diffuse sclerosis has been found in these cases. Not infrequently, as the result of hemorrhages, there develop cysts which fill up the atrophied areas of the brain (Fig. 216). It is difficult to pre- sent accurately and definitely the relations between the pathological change and the clinical result, but it may be shown with some degree of correctness in the following table. Original Lesions Later Pathological Conditions Clinical Result Hemorrhage. Atrophy. Hemiplegia. Embolism. Lobar and diffuse sclerosis. Diplegia. Thrombosis. Cysts. Paraplegia. Agenesis. Porencephaly. Sensory defects. Encephalitis. Microcephaly. Mental defects. Epilepsy, etc. Diplegia or Birth Palsies. — That form of the brain palsies of childhood character- ized by double hemiplegias or diplegias has certain special characters which lead to its being often classed apart. These diplegias in almost all cases are congenital and are due either to injuries at the time of birth or to some disorders of intra-uterine life. There may be convulsions or a prolonged state of asphyxia at the time of birth. After recovery from this no special trouble is noticed with the child by the mother for some weeks or months, when it will be found that it does not use its arms or legs. Other convulsions develop, and eventually the features of a double hemiplegia with mental impairment and epilepsj^ are observed. In these cases the mental defect is much more decided than in the hemiplegias; indeed, few of these cases ever show any good amount of intelligence. Epilepsy is extremely common. The anatomical lesion in the cases is, as already stated, either a meningeal hemorrhage which has pressed upon and injured the cortical motor areas in each hemisphere, or it is a congenital porencephalic defect. (See Little's Disease.) Diagnosis. — The clinical diagnosis of cerebral palsies is to be made from the spinal palsies. The latter are distinguished by the fact that in the infantile paralyses of spinal origin there is no rigidity or exaggeration of reflexes, and there are electrical degenerative reactions of the muscles and decided wasting of the limbs with shortening. The mode of onset in cerebral palsies and their distribution in the form of hemiplegias in which the face is involved also indicate the seat of the lesion. The pathological diagnosis is by no means an easy one. Cerebral palsies occurring at the time of birth and accompanied at the time by general convulsions or asphyxia may be considered to be due to meningeal hemorrhage, espe- cially if the delivery of the child has been brought about by the use of 452 DISEASES OF THE NERVOUS SYSTEM forceps or if the labor has been long and tedious. Diplegias and para- plegias which are congenital are probably due to true porencephalus, pro- vided there was no difficulty at the time of labor and there were no con- vulsions or other serious phenomena after it. Cerebral palsies occurring after birth in the first, second, or third year of life are apt to be due to hemorrhage and encephalitis, less often to embolism or thrombosis. Hemiplegias developing after infectious fevers are likely to be due to hemorrhage. In diagnosticating the pathological lesion in such cases it must always be remembered that hemorrhage is much more frequent than embolism, and that thrombosis occurs only in children with very weak hearts and in marasmus. Course and Prognosis. — In all types of the disease the course is chronic and perfect cure is hardly possible, although in the slighter forms of hemiplegia nearly all traces of the paralysis may be absent. In the hemi- plegic form the patient often reaches adult life, and if his intelligence is not defective and he has no epilepsy the motor trouble improves a great deal and he may live a long and useful life. If epilepsy and mental defect are present, there ensues eventually a further mental deterioration, and such subjects rarely live much beyond the period of adolescence, or if they do they pass into the asylums for the idiotic and epileptic. The diplegic and paraplegic cases have a much worse prognosis both as to duration of life and as to improvement in symptoms, except occasionally the type described above as due to agenesis of the pyramidal tracts. The degree of intelligence and the absence of epilepsy are the two factors which measure the seriousness of these cases, as they do those of the hemi- plegias. As regards the significance of individual symptoms, the post- hemiplegic movements have a bad import; the presence of a microcephalic head or of decided marks of degeneration is unfavorable. Treatment. — The treatment, so far as the paralysis is concerned, is largely mechanical. The patient is benefited by occasional courses of electrical treatment which stimulate somewhat the nutrition and func- tions of the muscles. Massage and stretching of the contractured tendons and limbs also are helpful in my experience. The orthopaedic surgeon is able to render valuable assistance by occasional overstretching the con- tractured limbs and placing them in spUnts. Tenotomy may also be re- sorted to with advantage, as I have had occasion to see. The child should be encouraged above all, however, to use the limb as much as possible. He should be taught gymnastic exercises; running, walking, and bicycle riding are all measures which give great help. When the child's intelligence is good and there is little or no epilepsy, a great deal can be expected in the way of improvement as he grows older. So far as the epilepsy is concerned, it should be treated on the same principles as idiopathic epilepsy, except that great care should be had in DISEASES OF THE BRAIN 453 the use of the bromides; a thorough test must be made in order to de- termine how much of this drug will suppress the fits, and then its use must be graduated in the future in accordance with the knowledge thus obtained. The mental defects of the child can be helped only by proper training of the body and careful education of the mind. The question of operative interference in these cases has excited much attention. A priori it would not seem as though surgical interference could do good in relieving conditions in which there is destroyed or atrophic tissue. Still the subject must be dealt with empirically, and there have been some results which show that apparently a relief is obtained in a few cases by trephining the skull and if he then finds any evidences of compression from the presence of a cyst, this may be very cautiously opened. Resec- tion of the posterior spinal roots has been tried with some successes reported. CHAPTER XIX TUMORS OF THE BRAIN All forms of new growths are found in the brain, but the infectious granulomata, tubercle and gumma, and the sarcomatous type of tumors are the most common. Gushing found that 64 per cent, of brain tumors were gliomata and gliomatous. Elsberg's statistics show about the same proportion.^ ^ In order to present a working view of brain tumors from a surgical standpoint, Dr. Charles A. Elsberg has kindly furnished me an analysis of 100 cases subjected to radical, exploratory or palliative operation. The figures naturally are not the same as those derived from a series of post-mortem studies like those of Schlessinger. but they represent more accurately the kind of cases that come to the neurologist, I present only the salient features of his analysis. Location Cerebral 44 Cerebellar or pontine 28 Unlocalized 20 Cortical or extra-cortical 14 Deeply subcortical 68 Not locahzed 28 Frontal, central and parietal 21 Occipital and temporal 5 Thalamic, 3d ventricle and mid-brain 10 Hypophysis and peduncular region 8 Cerebellum 20 Cerebello-pontine angle 7 Pontine 1 Nature of Growth GHoma 33 Gliomatous cyst 3 Gliosarcoma 1 Sarcoma 3 Endothelioma 5 Adenoma 5 Teratoma , 1 Cholesteatoma 1 Neurofibroma 5 Simple cyst 2 Nature unknown (no examination) 13 Nature unknown (unlocalized) 28 100 Operative Result Tumor was removable and removed in 13 Tumor was partly removed in 5 Cyst (non-gliomatous) was evacuated in 2 Tumor could not be removed on account of nature or location, or the tumor was not localized in 80 100 454 TUMORS OF THE BRAIN 455 Etiology. — Brain tumors affect males oftener than females, the ratio being about as two to one (644 :320). Sarcomata alone seem to affect females about as often as males. Brain tumors occur with about equal frequency throughout all ages of life up to about fifty; one-third occur under the age of twenty (Gowers). During childhood tumors are about equally distributed throughout all ages (Starr). One-half of all the tumors of childhood are tuberculous; after this come gliomata, endo- theUomata and sarcomata. The gumma, ghoma and sarcoma begin to be more frequent after the age of twenty. Sarcoma and especially cancer occur in the middle and later ages of hfe; but brain tumors of any kind are extremely rare after the age of sixty. To sum up in tabular form, the relative frequency of the different kinds of tumors with regard to age is shown in the following : Childhood, tubercle, parasites. Early life, gumma, glioma, parasites. Early and middle life, sarcoma, endotheUoma, glioma, and gumma. Middle and late life, sarcoma, endothelioma, gumma, cancer. Heredity has a shght influence in predisposing to brain tumors. Blows on the head and other forms of injury to the cranium are exciting causes in a small proportion of cases. Practically all cancers and many sarcomata of the brain are metastatic in origin. Seat. — About one-fourth of all tumors are in the cerebellum, or cere- bello-pontine angle, and about one-fourth are in the frontal lobe. About one-twelfth of all tumors are in the basal ganglia and lateral ventricles, and a rather smaller number in the mid-brain and medulla. Tumors more often lie in the cortex and parts just below, than in the centrum ovale. In children the locations are a little different. Here nearly one-half of the tumors are in the cerebellum and pons. They are very much less frequent in the cortex cerebri but more common in the centrum ovale, basal ganglia and mid-brain. As the mid-brain as well as all the contents of the posterior fossa are nourished by the vertebral arteries and their branches, it follows that the majority of tumors in children are in this vascular area. Symptoms. — The symptoms of brain tumors vary extremely in ac- cordance with the location, the kind of tumor, the rapidity of growth, and the age of the patient. The general course of a case of brain tumor in an adult is somewhat as follows: The patient first notices a headache which is intense and persistent, and which has exacerbations of frightful severity. With the headache or between the attacks vomiting occurs, which is often not accompanied by any nausea. Sensations of vertigo, annoying parsesthesias, and convulsive movements affecting one or more This indicates that about 20 per cent, of brain tumors brought to a special hospital or neurological surgeon are operable. 456 DISEASES OF THE NERVOUS SYSTEM extremities develop, and there may even be general convulsions. Papill- oedema and optic neuritis may then appear and the patient finds that his eyesight is weak and progressively deteriorating. The mind becomes more or less disturbed, the mental processes are dull and slow, a feeling of hebetude and incapacity to attempt any mental exertion are present. As the disease progresses the intense pains and vomiting produce weak- ness and emaciation. Paralyses of various kinds develop and blindness ensues. Convulsions of a local or general character become more fre- quent, and finally the patient becomes bed-ridden and helpless. The course of the disease is not a steady one, there being often slight remissions, or there may be periods when progress seems to be arrested. After a period of time varying from one to four or five years death occurs from exhaustion or some intercurrent malady. The symptoms thus very briefly outlined are divided into general and focal. The general symptoms are: Headache. Vertigo. Vomiting. Papilloedema. , Mental defects. Besides these there may be general convulsions and speech disturb- ances. Headache occurs in from one-half to two-thirds of the cases; it is very severe and the pains are of a boring or lancinating character; they are so horrible that they often lead the patient to think of suicide. The pains are sometimes periodical, occurring every night or every other day, and suggest by their periodicity a malarial character. They are located some- times in the brow or in the occiput, while sometimes they are diffused all over the head; they are rather more frequent than otherwise in the neighborhood of the tumor. They are more frequent with cerebellar tumors than with those located anywhere else. They are less frequent with tumors situated in the peduncles and at the base of the brain. The pains are due to the increased intra-cranial pressure and to irritation and stretching of the dura mater by the encroachments of the new growth. Headache occurs in about the same proportion in children and adults, and it does not seem to bear much relation to the kind of tumor, although the pains are generally less with the gliomata, and they are more frequent with rapidly growing tumors whatever their character. With the pains there is often a local tenderness of the scalp and cranium which may be elicited by percussion, and in most cases there is greater tenderness in that part of the cranium lying over the tumor. Vomiting is a symptom which is almost as frequent as headache. The vomiting is often of a projectile character and not accompanied by TUMORS OF THE BEAIN 457 much nausea. Vomiting occurs, as does headache, more frequently' with cerebellar tumors. It is associated with rapidly growing tumors, such as syphilitic or tuberculous neoplasms. Vertigo is a general symptom which occurs in from one-third to one- half of the cases. The vertigo may be slight, such as is often felt from ordinary causes. Occasionally it is very severe and accompanied by forced movements. The severer forms and those associated with forced movements occur with tumors of the cerebellum and the parts closely connected with it. Papilloedema or choked disc is one of the most frequent and im- portant of all the general symptoms of brain tumor; it occurs at some period of the disease in at least four-fifths of the cases, more frequently in cerebellar tumors and in those of the mid-brain and great basal ganglia. It is rare in tumors of the medulla. It is less frequent and marked in the slow-growing tumors and in infiltrating gliomata of the hemispheres. The condition may run a somewhat rapid course and then improve a great deal or even for a time disappear; but ordinarily the course is pro- gressive and it ends eventually in neuritis and an atrophy of the optic nerve. Hence the examination of the eyes in brain tumors should be made a number of times in order to note the progress of the trouble. Primary atrophy of the optic nerve does not occur in brain tumors, except to a certain degree in some frontal tumors. The choked disc is caused mainly by the pressure of the fluid in the subarachnoid space, which space communicates with the connective- tissue sheath of the optic nerve. This presses the nerve upon the lamina cribrosa, the central vein of the retina is strangulated and oedema and swelling follow. (Parker states that the choked disc is due to a dispro- portion between the intra-ocular tension and the intra-cranial pressure, by reason of which the disc is pushed into the eye.) Generally there is a greater degree of papilloedema on the side of the tumor. Slight mental defects are almost always present in tumors of the brain. These defects consist in a slowness of the mental processes, a condition of hebetude, a tendency to attacks of somnolence, and sometimes a peculiar childishness, silliness and tendency to foolish joking. The memory is also usually somewhat weakened and the power of attention lessened. Such psychical defects are more frequent with tumors of the frontal lobes and more frequent also with large tumors. General convulsions occur in about one-fourth of the cases and more frequently when the tumors are situated in the cerebral hemispheres and cortex. There may be also apoplectiform attacks, from which the patient recovers in the course of a few days or weeks. More rarely there is a genuine apoplexy from the bursting of a blood-vessel in the neighborhood of the tumor. 458 DISEASES OF THE NERVOUS SYSTEM The local syinptomatology of cerebral tumors cannot follow always strictly the anatomical subdivisions of the brain. There are symptoms distinguishing tumors which lie above and those which lie below the tentorium because the pressure conditions are not alike. Fig. 217. — Showing relationships of tumor in the posterior fossae of the skull. In suhtentorial tumors pressure soon blocks the aqueduct of Sylvius and causes a hydrocephalus and pressure symptoms. Tumors of the hemispheres cause the change more slowly, consequently optic neuritis and the general brain symptoms of tumor may be less marked. TUMORS OF THE BRAIN 459 1. Tumors of the Prefrontal Area. — Tumors in this area may show no locaHzing symptoms. Nevertheless in a good proportion of cases some- thing characteristic may be observed. Sometimes there is mental dis- turbance, which is mainly of the nature of mental deterioration, perhaps it simulates a dementia paralytica, or there may be only a certain degree of fooHshness and silly jocosity ( Witzelkeit) . The pressure of a frontal tumor upon the olfactory nerves may cause disturbances in the sense of smell. Pressure on the optic nerve may cause disturbances of the visual field and papilloedema. But the pressure on the optic nerve being local and direct may produce at the same time an early atrophy of the central fibres shown by the presence of a central scotoma (Kennedy). Rarely, the tumor involves the orbit causing paralysis of the ocular muscles and protrusion of the globe of the eye. If the tumor grows backward there is gradual invasion of motor centres with irritation, showing itself by local convulsions, and later by paralyses. Occasionally in frontal tumors there is tremor of the hands, and a kind of ataxia of gait and station (frontal ataxia) . 2. Tumors of the Precentral Convolutions. — It is in this area that we are often able to make the closest and most accurate diagnosis of the localization of new growths, owing to the involvement of the different motor centres. These centres are at first irritated, with the result of producing local spasms or Jacksonian epilepsy. Such spasms are often preceded by sensory symptoms or aurse. As the tumor grows, the area of involvement becomes larger, spasms become more diffused, and gen- eral convulsions may finally appear, with hemiplegia. Besides the symptoms mentioned, there may also be motor aphasia and agraphia. If the tumor is subcortical the symptoms are more of the paralytic and spastic type and less of a convulsive nature than when the cortex is implicated. 3. Tumors of the parietal lobe show disturbances of sensation and of perception tactile, muscular and visual. Tumors in the anterior portion (post-central convolution) cause disturbances of cutaneous sensation; those in the superior lobule cause astereognosis; those in the inferior lobule, anteriorly loss of deep muscular sense with ataxia; those further back cause forms of visual imperception such as apraxia and if on the left side, alexia. If the tumor lies deep there may be hemianopsia. If the tumor is in the post-central we get with disturbance of cutaneous sensation, usually some motor phenomena due to irritation of the precentral. 4. Occipital Lobes. — Tumors of this region, if situated in the cuneus and calcarine fissure, produce homonymous hemianopsia partial or com- plete, the macular region escaping. If the tumor involves the other parts of the left occipital lobe and the cuneus is not seriously involved, there 460 DISEASES or THE NERVOUS SYSTEM may be a condition known as soul blindness, sensory apraxia or imper- ception consisting in an incapacity to understand the nature of the things which one sees. If the tumor extends up chiefly toward the angular gyrus, on the left side, there may be word blindness, along with some hemianopsia. If the tumor extends farther forward and deeply into the parietal lobe, there will be hemiansesthesia, hemiataxia, and perhaps a little hemiplegia owing to involvement of the fibres of the internal capsule. Hallucinatory symptoms may occur in occipital lobe tumors. 5. Temporal Lobes-^The temporal or temporo-sphenoidal area on the right side is very nearly a latent one. On the left side, tumors involving the posterior part of the first and upper posterior part of the second tem- poral convolution produce sensory aphasia. Tumors of the anterior and inner side of the lobes involving the uncinate and hippocampal gyri may produce dreamy states and disturb- ances of smell and taste (uncinate fits). Tumors lying further back may cause also third, sixth and seventh nerve trouble if they lie in the ex- ternal portion, but if posterior and internal may cause nystagmus and cerebellar symptoms. As the tumor gets deeper it may cause hemiansesthesia and hemiplegia, the symptoms of paralysis being more marked and earlier in the face and arm (Kennedy). 6. Tumors of the Corpus Callosum. — Tumors situated in this area are very rare. Their symptoms are somewhat characteristic and cor- respond somewhat with tumors situated in the ventricles of the brain; in other words, tumors which, beginning in the central parts of the brain, gradually extend outward toward the periphery. The symptoms credited to tumors of the corpus callosum are mental dullness, stupidity, dys- praxia and drowsiness, the patient often sitting for hours mute, re- fusing to speak, or lying in a half-somnolent condition. There are no paralyses of the oculo-motor or other cranial nerves and often no optic neuritis, or any severe headache and vomiting. There is no anaesthesia. The symptoms progress; locomotion is early impaired and later the patient becomes unequally diplegic. The disease gradually progresses and the patient dies in coma. 7. Tumors of the Great Basal Ganglia and the Capsule (the optico- striate region). — The general symptoms of tumors of this region re- semble in many respects those of tumors of the corpus callosum. The hebetude, however, may be less marked. There is usually a progressive hemiplegia which may be accompanied by anaesthesia and sometimes by choreic movements, if the tumor involves the optic thalamus and adjacent part of the capsule. Tumors of the corpus striatum cannot be diagnosticated by symptoms due to the irritation or destruction of this ganglion. The location can TUMOKS OF THE BRAIN 461 be only approximately estimated by the presence of symptoms due to lesion of the adjacent parts, viz., the internal capsule and the adjacent motor cortex. When on the left side, symptoms of aphasia of the motor type may appear. As the lesion extends there may be hemiplegia, and later, perhaps, irritative symptoms from involvement of the capsule and motor cortex. Tumors of the Optic Thalamus. — The same may be said of tumors here as of those of the corpus striatum. Lesions of the thalamus itself Fig. 218. — Showing the mechanism of crossed paralysis. Lesion at M causes paralysis of third nerve, lesion at F paralysis of fifth nerve with hemiplegia of opposite side. give no specific symptoms unless it is that of pain referred to the ex- tremities of the opposite side. But by extension we get hemiansesthesia of various types and involvement of the ocular muscles and reflexes. There is later even homonymous hemianopsia; peculiar forced movements and intention tremor may occur. The opinion that in lesions of the thalamus there is a paralysis of emotional expression has been sustained by Mills. The thalamic syndrome which is observed in acute lesions of 462 DISEASES OF THE NERVOUS SYSTEM this ganglion does not appear very characteristically in chronic progressive lesions. Tumors of the Pineal Gland, the Pituitary Body and Optic Chiasm.— The thalamus and adjacent region ('tween-brain or diencephalon) is a part of the brain which in man is not sharply limited. It may be divided into an upper or dorsal part, including the thalamus, geniculate bodies and pineal gland, and a lower or ventral part, hypothalamus, which in- cludes the corpora mammillaria, the pituitary body and infundibulum and the optic chiasm. Tumors of the pineal gland are generally tera- tomata or psammomata. They may cause disturbance of metabolism leading to adiposity and precocious mental, physical and sexual develop- ment. Secondarily, they cause neighborhood symptoms such as disturb- ance in ocular movements, optic neuritis, ataxia, and hydrocephalus from blocking the aqueduct of Sylvius. Tumors of the pituitary body may be associated with giantism if they occur before adolescence and ossification of the epiphyses, and with acromegaly if they occur after this period. As they grow they press on the chiasm and cause optic neuritis and atrophy and a characteristic bitemporal hemianopsia. The pituitary disease may lead to other dis- turbances of nutrition such as adiposity and increased sugar tolerance, andchanges in secondary sexual characters forming the Frohlich syndrome. (See Acromegaly.) Tumors of the Mid-brain. — The mid-brain includes the cerebral peduncles, the corpora quadrigemina and the aqueduct of Sylvius. In the base of the peduncles run the cerebrospinal tracts, above which is the locus niger and above this the tegmentum with sensory tracts, the anterior cerebellar peduncles, the red nuclei and the nuclei and motor nerves of the eye (Fig. 213). The anterior tubercles of the corpora quadrigemina and geniculate bodies have to do with the reflexes and co- ordination of the eye movements, the posterior with the functions of the eighth nerve. Tumors here are sometimes tumors of the peduncles, and sometimes of the corpora quadrigemina, according as they are superficial or deeply situated. The general character of the symp- toms is an association of hemiplegia with disturbances of the motility and reflexes of the eye, often ataxic symptoms and sometimes forced movements and disturbances of hearing. We would expect soon to get symptoms of ocular palsies and of brain compression from closure of the aqueduct of Sylvius. As many as six different groups of symptoms or syndromes have been described due to lesions here. Some of these are given under the head of acute softenings and hemorrhages. (See p. 443.) When a mid-brain tumor lies in a cerebral peduncle it produces "alternate hemiplegia." Thus if the tumor is in the anterior portion and TUMOES OF THE BRAIN 463 situated high up, there is a palsy of the third nerve on one side and a hemiplegia of the opposite side (syndrome of Weber). If it is in the pos- terior portion and a little lower it will cause third nerve paralysis on one side and tremor or choreic movements on the other (syndrome of Benedict). Tumors of the corpora quadrigemina cause pupillary inequalities and paralyses, oculo-motor paralyses, vertical and lateral nystagmus. If deep enough to irritate the cerebellar peduncle, forced movements, asynergy, and disturbance of equilibrium. Tumors of the pons cause paralyses of cranial nerves on one side and hemiplegia on the other. If the tumor lies near the fifth nerve, there may be a palsy of this nerve on one side and hemiplegia on the other side (Fig. 218). If the tumor is larger it may produce paralysis of the fifth, STRATUM GRISEUM CENTRALE AQU/CDUCTU3 \CEREBR1 MEDIAL- LONGITUDINAL / BUNDLE J„ .rrUH^^^'^^^k^ /^^^'^^^ LATERALtS NERVE SULCUS N. OCULOMOTORII Fig. 219. — Section through mid-brain. sixth and seventh nerves on one side and hemiplegia on the other (syn- drome of Gubler) . If situated somewhat superficially and on the lateral edge of the pons involving the peduncles, there will be forced movements of the body, either toward or from the seat of the lesion. An impor- tant sign of intra-pontile tumor (Spiller) is paralysis of lateral associated movements of the eyes. If the tumor is unilateral the eyes cannot be turned to the side of the lesion. Tumors of the medulla are rare and generally tubercular or syph- ihtic. The medulla is the seat of origin of the IX, X, XI in part and XII nerves. Through it run motor and sensory and cerebellar tracts. It is the centre for the automatic regulation of respiratory, cardiac and vaso- motor functions. The olivary bodies place it in close relation also with the cerebellum. Tumors here cause, therefore, paralyses of the tongue, disturbances of deglutition, speech and finally of respiration and cardiac action. Hemiplegia, hemiansesthesia and ataxic symptoms may develop. Tumors of the cerebellum involve often a study of the condition of the vestibular nerve, of the spinal apparatus of equilibrium, and of 464 DISEASES OF THE NERVOUS SYSTEM the cerebro-cerebellar connections. It involves the employment of the diagnostic tests previously given. The accompanying diagram of the cerebellar connections, modified from Thomas and made more com- plete by Dr. W. F. Schaffler, will be found useful. smioR muiiai.. CEKBaW-^ESWmilT^KT. mniNTH. MmLE VCWHCLE i[smuLo s?wLTmr. Post. Column fiucUi. Cm mi Cond FlLltT. OLIVE. CoLmii Of GoLi. CoLmn«<^imfiCH. Fig. 220. — Connections of the cerebellum witli cerebral cortex, vestibular nerve and spinal cord. (Schaller.) Tumors of the cerebellum are more often seen in children and they grow rather rapidly, so that a distinct picture of cerebellar dis- ease may be developed in a few months. The early symptoms are vomiting, headache and choked disc ; and the child may be first brought to the physician for stomach trouble. The stumbling ataxic gait then develops and with it a good deal of general muscular weakness or TUMORS OF THE BRAIN 465 parasthenia. Strabismus and nystagmus are often early symptoms. The patient has a weakness of one side not amounting to hemiplegia and there may be some facial nerve weakness on the same side as the lesion. There is diminution or loss of deep reflexes and some hypo- tonia. As the ataxia progresses, the patient may be unable to stand well, but inclines to fall backward or to one side; when he tries to sit, even, he may fall over and the hands be- come awkward and ataxic, though less so than the legs. There may be sudden seizures of violent vertigo, or loss of consciousness, with tonic irregular movements of the limbs, or perhaps only sudden short forced movements, throwing the patient to the floor (cerebellar seizures). These are less common in children than in adults. Other special symptoms of cerebellar disease elsewhere described may appear. These are nystagmus, jerky tremors of the hands, adiado- kokinesis, asynergy in locomotion, dysmetria, cerebellar catalepsy and the disturbance of the normal laby- rinthine reflexes as shown by Barany tests (see general diagnosis). As the tumor grows it usually stops up the aqueduct from the third to the fourth ventricle, and then an internal hydrocephalus develops and we get symptoms of brain compres- sion. The mind becomes apathetic and slow; headache and vomiting are less, blindness sets in, with paralysis of some of the motor nerves of the eye and dilated and fixed pupils. The neck is retracted and stiff and Kernig's sign is present. The pulse may be slow at first and later in the terminal stage may be signs of bulbar com- pression and paralysis. The patient emaciates and death occurs in one- half to two years ; though in some slow-growing tumors of adults the dis- ease may last ten years or more. Tumors of the lateral lobes of the cerebellum should theoretically cause especially ataxia of the extremities, on the same side and a tendency 30 Fig. 221.- — Attitude in a case of cerebellar tumor. 466 DISEASES OF THE NERVOUS SYSTEM to fall to that side. Tumors of the vermis should cause more disturbance of equilibrium and cause a tendency to fall usually forward or backward. Tumor of the ponto-cerebellar angle gives a rather special sympto- matology. It arises usually as a small fibroma on the auditory nerve. It grows very slowly and may undergo finally sarcomatous degenera- tion. As it grows it forms a hard, circumscribed, pedunculated mass pressing into the angle between the lateral and anterior surface of the pons and the middle peduncle of the cerebellum. The first symptoms are simply those of irritation and compression of the eighth nerve, with tinnitus, vertigo, and progressive deafness; then there may be pains in the trigeminal nerve and later perhaps some parsesthesia or anaesthesia Fig. 222. — P(j.sitiuii of head in cerebellar tumor. {Fraenkel.) over the distribution of this nerve with loss of conjunctival reflex. The facial nerve may become involved as the disease progresses. Usually not till after several years does the patient begin to show the general symptoms of brain tumor, and then those of cerebellar disease: headache, vomiting, choked disc, staggering, cerebellar gait, falling generally to the side of the lesion, cerebellar attitude. In about half the cases there are cerebellar seizures and there may be some hemiplegia on the side of the lesion; finally, symptoms of internal hydrocephalus and brain pressure appear. Multiple Tumors. — About one-tenth of all cases of brain tumor are multiple. Hence, in making a diagnosis of the localization of tumors this fact must be borne in mind. The tumors which are most frequently multiple are tubercle, cancer, and melanotic growths. TUMORS OF THE BRAIN 467 Pathology. — Tubercle is the form of tumor found oftenest in children. It is more often located in the cerebellum, but may appear in the pons or other parts of the brain. It may be a single or, as it is then called, a solitary tubercle, or there may be a multiple growth. The tumor is irregularly round in shape and varies in diameter from one and a half to two inches. It has a grajdsh-j^ellow appearance externally; internally, a yellowish or cheesy look. It is not vascular, but is often surrounded by softened or inflamed tissue. There may be an associated meningitis. The tumors, when solitary, usually start from the central parts of the brain, but they also develop on the meninges of the convexitN'', particularly in the parietal region, and sometimes they develop also at the base. The tumors develop usually from some infectious focus, starting in a blood-vessel of the pia mater. Microscopically, the tumor shows the ordinary appearances of tuberculous growths. It contains in its periphery many round cells, nuclei, and giant cells. In the centre there is usualty an amorphous substance, the product of degeneration and the breaking down of the ordinary substance of the tumor. The characteristics of the growths are the presence of the round cells and giant cells, the casea- tion and softening of the centre, and the absence of vascularization, with the presence of the bacilli. Syphiloma or Gumma. — Gummatous tumors of the brain are usually associated with syphilitic meningitis and endarteritis and are usually found upon the brain surface, oftenest on the base, next upon the con- vexity of the frontal and central convolutions. The process appears either in the form of a somewhat distinct tumor or in the form of an ir- regular thickened exudate lying upon the surface of the brain and forming what is called gummy meningitis. The gummata may attain great size. They start usually from the pia mater and are due, as in the case of tuber- cle, to the irritative action of the infective organism. The gumma is irregular in shape; it has a somewhat thick, grayish periphery and often a yellowish centre, the appearances differing with the age of the tumor. Microscopically, it is found to consist of small round cells and spindle cells with various broken-down nerve-tissue elements. Gummata are at the present time very rare forms of brain tumor, as their development is usually controlled by treatment. Actinomycosis is a form of infectious tumor which sometimes ex- tends from the face and neck into the brain, leading to inflammatory processes, however, rather than to true tumors. No other neoplasms of infectious origin attack the brain unless sarcoma be found to be of that nature. Cystic tumors are not uncommon and are the result of parasitic in- vasion, of injuries, and of the breaking down of a glioma or from a teratoma. 468 DISEASES OF THE NERVOUS SYSTEM Glioma is the most frequent form of brain tumor. It may occur in any part of the brain, but is most frequently found in the cerebrum. It is the only tumor which is peculiar to the nervous centres, being developed from the neuroglia tissue which forms the supporting structure of these centres. Glioma originates in the white or gray matter of the nerve-centres and not from the membranes or fibrous structures. It may Fig. 223.— Tumor of frontal lobe. (J. R. Hunt.) grow to a very large size and is the form of brain tumor which becomes the largest. Gliomatous tumors measure from three to eight or more centimetres in diameter. In appearance the glioma can be scarcely distinguished from the brain substance itself, but usually looks like either pale or congested gray matter, or it may have a yellowish or gelatinous appearance. The tumor is very vascular and it may show the results of hemorrhages. The central part sometimes breaks down, TUMORS OF THE BRAIN SYPHILIS 469 forming cavities or cysts. The tumor may grow very rapidly, infiltra- ting the normal tissue and in these cases there is hardly any definite boundary between it and the normal tissue. In other cases the tumor grows slowly, but rarely if ever becomes en capsuled. Microscopically, it is found to consist of small cells with delicate fibrous prolongations, Fig. 224. — Tumor of cerebello-pontine angle. these being the glia cells. Gliomata may undergo certain changes, e.g., a mucous degeneration of the cells takes place, forming a myxo- glioma. When there is with the neuroglia-cell proliferation a rich proliferation of round cells from the connective tissue, it is called a glio- 470 DISEASES OF THE NERVOUS SYSTEM sarcoma. When the tumor is situated near the surface, involves the membranes, and grows slowly, with an increase in fibrous tissue, it is called a fibro-glioma. When the gliomatous growth is very firm and hard, the fibrous portion of the glia tissue predominates; it constitutes a nodule such as is found in multiple sclerosis, and these hard gliomata are sometimes called neuro-gliomata. Sarcoma. — The sarcoma and its various modifications is next to glioma in frequency. It may be primary or secondary in origin. The sarcoma develops from the brain membranes or from the walls of the blood-vessels and nerve sheaths. Sarcomata may be single or multiple. They may be of all shapes and they grow often rapidly to very varying sizes. They often develop a capsule. They are white or grayish in appearance or may be somewhat yellowish, dependent on the predomi- nance of the different kinds of cells and blood-vessels. Microscopically, they are made up of small round cells, spindle cells, and other cells of various sizes and forms. They contain often considerable fibrous tissue. They contain blood-vessels, but are not richly vascular. The essential characteristic of the sarcoma is the rich development of round cells and spindle cells; in other words, its rich cellular contents. Sarcomata are peculiar in undergoing many modifications. Thus sometimes fibrous tissue develops largely and the tumor is called a fibro-sarcoma; sometimes the tumor undergoes mucous degeneration and is called a myxo-sarcoma. There may be a breaking down of the centre with the formation of cysts. There may be a development of pigment. Not frequently a sarcoma- tous process invades a glioma and we have a mixture of a sarcoma and glioma. Sarcomatous tumors sometimes have an alveolar structure. These tumors contain endothelial cells derived from the lymphatics. When sarcomata develop from the dura mater and are slow in growth there may be calcareous deposits in them and they are called psam- momata. Endothelioma develops from the endothelium of the blood-vessels and lymphatic spaces. It usually starts from the meninges and is often en- capsulated and therefore subject to surgical treatment. The fibroma is a very rare brain tumor, unless the Pacchionian bodies, when enlarged and hardened, may be so considered. Occasionally fibro- neuromata are found developing on the roots of the cranial nerves. Osteoma is not particularly rare, developing in the form of bony plates in the dura, falx, or tentorium. Osteomata in the brain substance are mere pathological curiosities. Angiomata occasionally occur in the pia mater in connection with vascular nsevi of the face, and also in the cortex and basal ganglia. They are also usually congenital. Enchondromata and lipomata are extremely rare. TUMOES OF THE BRAIN SYPHILIS 471 Cancer is relatively a rare affection of the brain, and is almost always of secondary origin. Parasitic growths. — Parasitic tumors are extremely rare in this coun- try. The forms which are found are the echinococcus and the cysticercus cellulosse. The echinococcus produces hydatid cysts, which may be large or small, few or many, and are usually all upon the surface of the brain. T hey are much rarer than the cysticerci. These latter form cysts which are usually multiple, slow in growth, lie upon the surface of the brain or in the ventricles, are encapsuled, and show no symptoms. Aneurisms are anatomically tumors, but clinically they present some special symptoms and hence are described separately. Diagnosis. — -It is necessary first to make the diagnosis of the presence of the tumor, next of its location, and finally of its nature. The existence of a brain tumor is determined by the presence of the characteristic general symptoms — ^headache, vomiting, vertigo, optic neuritis, mental disturbances, progressive course and local signs. The things which pro- duce somewhat similar symptoms are localized meningitis (syphilitic or tubercular), brain abscess, localized foci of arterial sclerosis; lead poison- ing, hysteria, acute internal hydrocephalus and certain toxaemias. The physician must bear in mind the age of the patient and the exist- ence of a tuberculous or syphilitic history, the history of an injury, of local tenderness, and the presence of some new growth in other parts of the body, particularly about the neck or thorax or in the lungs. Blood tests assist in excluding inflammatory and luetic conditions. Lumbar puncture and examination of the cerebrospinal fluid helps to determine lues and meningitis; brain puncture and the withdrawal of a very small cylinder of brain tissue which may be examined microscopic- ally has become a useful and safe operation. Percussion of the skull sometimes shows a duller note over the region of a tumor. Occasionally in young patients percussion brings out a cracked-pot sound. This only occurs when the intra-cranial pressure has loosened the sutures of the skull. In X-ray pictures one sometimes can see a darkened mass indicating the presence of a tumor. More often the X-ray only helps by showing changes in the bone, as in tumors of the sella turcica. Sometimes also in superficially placed tumors the plate shows broad lines indicating dila- tations of the veins of the diploe. The diagnosis of the location of the tumor is based upon the rules already given in regard to local diagnosis. The diagnosis of the nature of the tumor can often be made and should be attempted. About two-thirds of tumors in adults are gliomata. Tubercle and glioma are the most common in children. Cerebellar tumors are often gliomata, especially in adults. Tumors of the ventricles are usually gliomata or adenomata. Tumors that develop without 472 DISEASES OF THE NERVOUS SYSTEM many serious pressure symptoms are usually gliomata. Cancers are practically always secondary and sarcomata sometimes. Endothelioma are generally superficial, at first, starting from the meninges. The pres- ence of syphilitic tumors can be inferred from laboratory tests. Sudden exacerbations of symptoms due to intra-neoplasmic hemorrhage suggest glioma. Prognosis. — In extremely rare cases tumors of the brain appear to stop growing and become encapsulated and atrophied. Such tumors are of a tuberculous or syphilitic, perhaps sometimes of a gliomatous or sarcomatous, character. As a rule, the brain tumor grows steadily and the symptoms of the disease become more pronounced until death occurs. The prognosis is best for tubercle in children and gumma in adults. It is worse in cases of glioma and cancer. The disease lasts on an average two or three years, ranging from a month to eighteen years. Treatment. — Something can be done medically in cases of tuberculous tumors, syphilitic tumors, and possibly in the sarcomatous variety. In tuberculous tumors a general constitutional and strengthening treatment must be resorted to; fresh air, rest, tonics, and proper food being the main reliance. In sarcoma and glioma, some help may be obtained from the internal use of arsenic. Iodide of potassium sometimes helps non-syphi- litic tumors. Symptomatically we must give drugs for the relief of pain. The ice cap and leeching often help the headache. Should convulsions develop, the bromides should be used, just as in idiopathic epilepsy. In cases in which the location of the tumor can be made out, the ques- tion of surgical interference should be considered. The percentage of cases in which surgery can effectually help is small. It will include only those cases in which the tumor can be located; of those which can be located, only those which are in an accessible region, and finally, of those which are in an accessible region, it includes those whose removal will not leave the patient helpless, demented or aphasic. The removable tumors of the brain amount to less than 5 per cent. They are, in par- ticular, the endotheliomata, sarcomata and gliomata. These latter some- times degenerate and become absorbed after partial removal. Tumors can be removed with possible benefit from the superficial part of any portion of the cerebral hemispheres except the left temporal lobe. Here the patient if operated on would be made aphasic and probably hemi- plegic. Tumors can be removed from the cerebellum and the cerebello- pontine angle with occasional success and even with brilliant results. Tumors can be removed from the hypophysis, by the very expert. Tumors of the mid-brain, pons, medulla, and of the ventricles are not operable. In cases which are not operable, the decompressive operation of Gushing or puncture of the corpus callosum may often be done with advantage. TUMORS OF THE BRAIN SYPHILIS 473 Lumbar puncture can be employed to test and to a slight extent relieve the intra-cranial pressure. The measure should not be employed, however, in subtentorial tumors as the medulla may be pushed into the foramen magnum and death ensue. The ordinary pressure of the cere- brospinal fluid of 40 to 150 mm. of water may in brain tumor be increased to 250 and 900. INTRA-CRANIAL ANEURISMS Intra-cranial aneurisms are of two kinds — ^" miliary" and those of large size. The miliary aneurisms are minute dilatations of the vessels and are always multiple; they have been described under the head of cerebral hemorrhage. Large aneurisms affect only the large cerebral arteries at the base of the brain. The arteries are affected in the following order: middle cerebral, basilar, internal carotid, and anterior cerebral. The anterior and posterior communicating and vertebral arteries are occasionally involved, the posterior cerebral and inferior cerebellar very rarely (Gowers). Etiology. — Males are affected slightly oftener than females. Aneu- risms occur at all ages from ten to sixty; before ten and after sixty they are extremely rare. Heredity occasionally plays a part in predisposing to cerebral aneurisms. The exciting causes are embolism, especially when the emboli contain microbes, syphilitic disease, injuries, and in rare cases senile arterial degeneration. The symptoms are very indefinite; they resemble to a considerable extent those of tumor at the base of the brain; headache and vertigo, mental dullness and irritation, cranial-nerve palsies, and occasionally hemiplegia and convulsions are noted. Optic neuritis is rather rare. In a few cases the patient is conscious of a murmur or recognizes the pul- sating sensation in the head. Sometimes when the aneurism is in the vertebral artery, a murmur can be heard between the mastoid process and the spinal column (Moser). The diagnosis is often difficult; it is based on symptoms of tumor at the base of the brain pressing on cranial nerves and on motor or sensory tracts. The effect of carotid compression should be tried. The prognosis is not good. In perhaps the majority of cases a rup- ture of the vessel occurs in a few years; however, rupture is not the in- evitable event, and sometimes the disease becomes stationary or under- goes spontaneous cure. The treatment of the disease, if it can be recognized, is the same as that for aneurism elsewhere, so far as drugs are concerned. The use of salvarsan has produced good results; surgically, the common carotid may be tied (though this involves some risk) and perhaps the vertebral if the aneurism is believed to be connected with that artery or with the basilar. CHAPTER XX FUNCTIONAL AND DEGENERATIVE DISEASES- EPILEPSY Functional is a term applied to nervous diseases in which no known anatomical change underlies the morbid phenomena. Degenerative diseases are those due to or associated with a constitutional deterioration, or biogenetic defect. The number of diseases of functional character is steadily lessening, and the use of the term, therefore, is being supplanted by other qualifying words. Epilepsy is a disease of the functional and degenerative type, although the essential and dominant symptoms are due to various morbid condi- tions. It is a chronic nervous disorder characterized by periodical seizures attended by loss of consciousness and usually by convulsions. Mental disturbances may accompany or take the place of the convul- sions. The mental make-up of the patient is generally peculiar and if the disease progresses some mental deterioration sets in. It has no estab- lished pathological anatomy, although there are degenerative cell changes in the brain cortex. Symptomatic epilepsy is a form in which the periodic convulsive attacks are associated with gross organic changes in the brain, or is the expression of a definite epileptogenous irritation (toxic, vascular, psychic or reflex). Jacksonian or partial epilepsy is a form of epilepsy, usually sympto- matic, and is characterized by periodic convulsions affecting only certain groups of muscles, and often unattended by loss of consciousness. Hystero-epilepsy is not epilepsy, but a form of hysteria. Eclampsia or acute epilepsy is the name given to a single isolated attack of convulsions. It is generally of the symptomatic type. Ordinary or "idiopathic" epilepsy shows itself in three rather dis- tinct types of attacks, viz., that of severe attacks called the grand mal; that of minor attacks, the petit mal; and the rare larvated forms char- acterized by acute mental disorder and called psychical epilepsy or the psychical epileptic equivalent. Etiology. — Predisposing causes: Heredity is the most potent of any single influence. A history of epilepsy, insanity or some serious neuropathic condition is found in the family in over one-third of the cases and rather more on the paternal side. 474 FUNCTIONAL AND DEGENERATIVE DISEASES 475 In many other cases it can only be established that the family is a neuropathic one. The heredity is rarely direct; i.e., the parents are not epileptic in more than about 1 per cent, of cases. Alcoholism and the intermarriage of neurotic persons contribute to produce the convulsive tendency in children. The element of alcoholism, in my experience (private and public) is only about 5 per cent. It must be remembered that many parents reported alcoholic were not so at the time of the birth of the child. Powerful emotions during pregnancy, accouchement in- juries, and syphilis, have slight influence. The Wassermann test for syphilis in epileptics has brought out a small percentage of positive re- actions. Very important causes of epilepsy are cerebral or meningeal hemorrhage and attacks of encephalitis occurring in early life. Such attacks may lead to mental defects and paralysis; about 40 per cent, of these cases have epilepsy. More cases occur in the country than the city, more in temperate climates, and more among in-bred races. American statistics show a slight preponderance among males. The epileptic age is between birth and twenty, and still more definitely between five and fifteen. In three-fourths of the cases the disease begins before the age of twenty; in one-sixth of my cases, before the age of five. After twenty the danger of epilepsy is slight, and when it occurs it is usually due to accidental causes, like syphilis, alcoholism, or plumb ism. Idopathic epilepsy, however, may develop even after sixty. The accompanjdng table shows graphically the relation of age to the de- velopment of epilepsy, chorea, and neuralgias. Exciting causes are not present in the majority of cases. The most important are the occurrence of rickets at the time of denti- tion, injury to the head, sunstroke, acute infectious diseases and toxaemias, persistent reflex irritations and alcoholism. The impor- tance of traumatism when there has been a real injury to the brain is considerable. Simple concussions without organic injury do little harm. Masturbation is a real but rare exciting cause. The so-called reflex causes are ocular and auditory irritations, worms, gastro-intestinal weak- ness, dental irritations, and lesions involving peripheral nerves. Probably the gastro-intestinal tract and genital organs furnish the most important exciting irritations. X-ray examination often shows some defects in the position and functioning of the digestive tube with ptosis, kinks and a patent ileo- csecal valve. A chronic epilepsy may be brought out by peripheral irritations while more rarely there occurs only a reflex epileptiform neurosis, the convul- sions subsiding when the irritation is removed. Epileptic seizures of characteristic type may be caused by psychic 476 DISEASES OF THE NERVOUS SYSTEM states. In other words, an apparent epilepsy may be psychogenous. Some followers of psychoanalysis claim that the convulsive seizure itself is the expression of a suppressed wish or instinct of infantile origin. Symptoms. — The Onset. — The disease begins sometimes with attacks of petit 7nal which may last one or more years before anything more serious occurs. Then suddenly there is a convulsion. Occasionally nothing but petit mat attacks ever occur. In other cases without pre- vious warning the patient has a severe seizure. In six months or a year he has another, in the next year he has three or four and then they come on regularly every week or two. Percentage of total cases 60;^ 50% 40 X 30% 20% lO/o up to end of 5Vr 6'^ to 7th lit to to 3M.* to 4o*^ 4 p.' to, 51^.' to 60*^ to r '' "^. / \ r u ^^ N \ \ 1 1/ / \ V :i; ^ v.. ...^ Fig. 225. — Table showing percentage of cases of epilepsy, chorea, and neuralgia occurring at each half -decade and (after twenty) each decade. Double line, epilepsy; dotted line, chorea; single line, nuralgia. The Convulsion. — The patient often feels some premonitory symp- toms for a few hours or a day, consisting of general malaise, irritability, or giddiness. The attack begins in about half the cases with a peculiar sensation called the aura. Often also a loud cry is uttered and the patient falls unconscious to the ground. The face is pale, the eyes are open and turned up or to one side, and the pupils dilated. The head is drawn back or to one side, and the whole body is in a state of rigidity or tonic spasm. The arms are slightly drawn out from the trunk, the forearms and wrists extended or flexed, the fingers clinched or flexed in various ways, the legs and feet extended. This tonic stage lasts for fifteen FUNCTIONAL AND DEGENEKATIVE DISEASES 477 or twenty seconds; the face becomes congested and then livid from com- pression of the veins of the neck and stoppage of respiration. Gradually jerky movements of the face and limbs begin and the stage of clonic spasm sets in. The trunk and limbs are now alternately flexed and extended with violent shock-like contractions, the facial and eye-muscles twitch, saliva collects in the mouth, and as the tongue is often bitten it becomes stained with blood. The movements are sometimes so violent that the patient is thrown about the bed or floor, and occasionally a limb is dis- located, usually the shoulder. The urine often, and the faeces occasionally are passed. The temperature is raised 3^^° or 1° F., rarely more. The pulse, feeble at first, becomes frequent and tense, and then, as the attack subsides, becomes feeble again. The clonic spasm lasts from one-half to one or two minutes. It subsides gradually, and the patient sinks into a stupor, from which he can be roused with difficulty. This stupor is succeeded by a heavy sleep of several hours and a feeling of hebetude which lasts all day. Vomiting sometimes occurs as a terminal symptom. Immediately after the attack there is a temporary exhaustive paralysis, with loss of knee-jerk. The pupils contract again and often oscillate. There may be a slight amount of transient albuminuria or glycosuria. The earthy phosphates are found increased; urea is not. There is a dis- tinct lessening of hsemoglobin in the blood (Fere) and of hsematoblasts. Sometimes the attack is followed by others, and for hours the patient passes from one convulsion into another. This condition is called status epilepticus. It usually lasts less than twelve hours, but may last for one or more days and until finally death occurs from exhaustion. It develops only in the severer types. Symptoms of the Minor Attacks. — In the minor attacks {petit mat) the patient suddenly stops in anything in which he is engaged, the features become fixed, the eyes are open, the face is pale, the pupils are dilated, often slight twitching of the facial muscles or of the limbs occurs, and consciousness is lost. In a few seconds the attack is over, and the patient, who does not fall, resumes his work or conversation, being unconscious of what has occurred, except that he has had a "spell." Often there is a warning sensation of aura. This is felt as giddiness, sense of fear, numb sensations of extremities, flashes of light or blind- ness, or choking sensations. There may be a cry uttered. The minor attacks are in rarer cases accompanied by sudden forced movements; the patient runs a few steps; or turns round, or makes some automatic move- ments. This is called procursive epilepsy. Sometimes the patient sud- denly falls to the ground, as if the legs had given way. He immediately rises and is quite normal again. There is no vertigo or apparent loss of consciousness. This is a true "falling sickness." Sometimes he makes 478 DISEASES OF THE NERVOUS SYSTEM simply a sudden very violent nodding movement of the head. This is the epileptic "salaam spasm." Symptoms of the Psychical Attacks. — Sometimes the minor attacks are followed by outbursts of maniacal excitement or by sudden violent automatic movements, and in these states the patient may commit crimes of violence. In rare cases the patient passes into a somnam- bulic state, during which he performs accustomed acts, such as driving and walking, automatically and naturally (somnambulic epilepsy). This form of epilepsy may come on without a preliminary minor attack, and then it is to be considered a ''psychical epileptic equivalent." Minor attacks may end in convulsions of a co-ordinate type in which the patient jumps, kicks, throws the arms about as in hysterical attacks. These are called hysteroid convulsions. The seizure may consist of only a short tonic stage and a few twitch- ings of the limbs, the whole lasting but a few seconds. This is called an abortive attack. Under the influence of medication, the severe seizures are often reduced to abortive forms. Jacksonian or partial epilepsy is a form of the disease characterized by convulsive attacks affecting only a single group of muscles or a limb, and generally not accompanied by loss of consciousness. Jacksonian epilepsy is always symptomatic of some focal lesion affecting the cortical motor area of the brain. This may be a tumor, inflammation, or injury, or only some slight degenerative change in the cells of the cortex. This form of seizure is particularly significant of a slowly growing brain tumor or syphilis. The epileptic aura usually consists of a sensation of numbness, prick- ling or of a breeze beginning in the hand or leg and passing up to the head when consciousness is lost. Still oftener there is a peculiar sensation starting in the epigastrium and passing upward. More rarely there are special-sense aurse, such as a ball of light in one eye, noises, or voices or peculiar tastes or smells. Besides these there occur sudden desire to go to stool, feelings of giddiness, dreamy states, peculiar sensations in the head, and indescribable general sensations. The aurse may be divided into : Visceral — -epigastric, laryngeal, cardiac, rectal. Cutaneous sensations. Special senses — flashes of light, etc. Psychical — emotions, dreamy states, etc. Cephalic — giddiness, etc. The aura is thought to indicate the seat of the first discharge of nerve force, and its study is of most importance in connection with sympto- matic epilepsies, as will be shown later. The patient may have only the aura for a long time before the convulsion sets in. Sometimes there FUNCTIONAL AND DEGENERATIVE DISEASES 479 never is anything but the aura. This undeveloped form of epilepsy I have called paraepilepsy. Frequency of the Attacks. — Epilepsy with the severe attacks is the most frequent type, next come combinations of severe and minor attacks, and next minor attacks alone, while the psychical forms are the rarest. The severe attacks may come on only once or twice a year, and this commonly occurs during the development of the disease. The frequency gradually increases until they occur every month, or two or three times a month. Sometimes the fits occur in groups of four or five every month or two. In very bad cases convulsions occur every day. The petit-mal attacks are more numerous and often occur daily. Time of Attacks. — ^The moon and the seasons have no influence. More attacks occur during waking hours than during sleep; but two- thirds of the attacks occur between 8 a.m. and 9 p.m. Many patients have their attacks early in the morning just after awakening {matutinal epilepsy). According to Pierce Clark, the hour in which the greatest number of attacks occur is 9 p.m., and the fewest attacks occur between 4 and 7 p.m. State of Patient Between Attacks. — Epileptic patients often feel better for a time after the convulsion is over. After very severe attacks or a series of them there may be a clouded consciousness, amnesia and mental confusion for several days. They not rarely suffer from severe neuralgic headaches; the appetite is capricious, often in children it is voracious, but in older cases there may be anorexia; the bowels are usually con- stipated; the pulse is small, soft, and frequent in the young, later it is often slow. The blood pressure is usually low. Mental Condition. — -A gradual mental deterioration occurs in the great majority of epileptics, but it is slight in some and not very serious in others. It shows itself by feebleness of memory, irritability of temper, selfishness, incapacity to concentrate the mind or to carry out a purpose. In children great mischievousness and lack of moral sense, with vicious, impulses, may appear. The mental deterioration is dependent on those underlying factors which cause the disease. It is apparently associated with the excessive number of the fits, and the accompanying degenerative changes in the cortex of the brain. This is not always the case, nor is it generally true that deterioration occurs more often with petit mal. It is more marked in cases beginning very early in life, but this is true only when there are early and decided marks of physical and mental degeneration present. A certain rather small percentage of epileptics become either demented or insane. True epilepsy is rarely compatible with extraordi- nary intellectual endowments. Caesar, Napoleon, Peter the Great, and other geniuses may have had symptomatic fits, but not epilepsy. 480 DISEASES OF THE NEKVOUS SYSTEM Physical Condition. — Epileptics are usually undersized and of not very robust constitution (Fere). Some patients, however, present a very vigorous muscular development. They always present some of the marks of degeneration, physical, physiological, or mental. Such marks or stigmata are about ten times more frequent than in healthy persons. The physical stigmata are (Fere) short stature, cranial asym- metry (in 71 per cent.), short parietal or frontal arc, and triangular skull; in women high prominent forehead ; bad teeth badly placed, high palatal arch; facial asymmetry; prominence of occiput and lemurian hypophysis; differences in color, size, position and shape of pupils; astigmatism (in 75 per cent, of cases); badly shaped and placed ears; misplaced crown of scalp; low vital capacity; small genitals, atrophic uterus; greater develop- ment of left side; long fingers. Cranial deformities of pronounced type occur in epileptics associated with idiocy, hemiplegia and brain defects of early origin. Sometimes, apparently from a premature ossification of sutures, there are the peculiar shapes of the skull known as scaphocephaly, or steeple skull, and plagiocephaly, or obliquely deformed skull. The physiological marks of deterioration are a lessened muscular strength (as 35 to 50), habit choreas, a rather imperfect vision with excessive amount of astigmatism and functional muscular weakness. There is a lessened vital capacity, weak and slow digestion, often defective met- abolism and sexual atrophy or irritability. The psychical stigmata are mental feebleness, moral insensibility, a certain unreasonableness, lack of insight and; irritability. Some have wayward and vicious impulses, lack of will' power and sexual aberrations. There are cases of epilepsy, however, in which the mental powers are fairly normal and continue well preserved; and I am describing the non- custodial, non-demented cases. : Association Diseases. — Epilepsy may be complicated with a de- generative myoclonia (myoclonus epilepsy). It is sometimes seen in connection with torticollis and spasmodic tic of other types. Epileptic seizures occur in cerebral syphilis and general paresis. Epilepsy is regarded by some as not a disease, but a syndrome or symptom-complex due to various morbid conditions of the brain. It is true that epilepsy runs a varying course and presents different features in different individuals. The grouping of cases in accordance with the severity and type of attack, the degree of intelligence, the tendency to- ward deterioration, the physical and physiological defects, the tendency to arrest improvement, or increased severity of the disease is, therefore, desirable. Taking into account these factors, I find in my experience the following general types or groups. It is not pretended that this is an exact classification. Such a one is not yet possible. It is fairly accurate, FUNCTIONAL AND DEGENERATIVE DISEASES 481 as I have seen epilepsy and I have watched many cases for ten to twenty-five years. The types are: I. The Common Mixed Form. — The attacks begin about puberty. They are of haut- and petit-mal type, but it is the haut-mal that is trouble- some. The seizures settle down to four to six monthly and occur singly or in groups, by day or night. The patients have some morbid mental traits; they are of moderate intelligence and this deteriorates a little, but does not end in insanity or dementia. The physique is fairly good, often robust, as to the muscular system; there are generally defects of physiological function. These patients sometimes get well or get their attacks well under control. II. The Petit-mal and Psychic Type. — The attacks are of the psychic and mild type alone, at least for very many years. They come on, as a rule, in early childhood at the fourth and fifth year or even sooner. They increase until they occur many times daily. The patients are bright, often very bright and precocious children, and they do not de- ment as the years go on, except very late or unless haut-mal attacks set in. They may, however, develop mental peculiarities and defects. Physically, they are of delicate but sound physique. These cases do not get well. With this petit-mal type go most of the cases of cerebral automatism, psychical epilepsy, epileptic sleep attacks, etc. III. The Delayed Type, Epilepsia Tardiva. — ■ The attacks do not come on until after adolescence, and sometimes not till the climac- teric. They are of haut-mal type, but petit mal is usually present in some degree. There is sometimes a history of cause, such as infection, trauma, syphilis, overstrain and arterial sclerosis. The attacks are not very frequent relatively, i.e., one or two a month. They are con- trolled fairly easily, though not entirely. The patients are of fair intel- ligence, of higher degree at least than those of type I. They do not deteriorate very much. The epilepsy of advanced years is not of this type, but is a phase of senile dementia. IV. The Organic and Degenerate Type. — The attacks come on in con- nection with gross defects or disease or injuries of the brain. There is retarded development, idiocy, imbecility, perversions and lack of balance in the mental functions. Paralyses, tics, etc., are present. Here the epilepsy is only one expression of the morbid cerebral condition. The cases are hopeless, so far as cure is concerned. Underlying all the types there is, as a cause of the seizure, some psychic state or degenerative neuron change. It is very probable that this change is of very much the same character in all cases, and that the disease differs in course and symptoms because the changes vary in locality, extent and intensity. Even in idiots and paralytics the epileptic phenomena are 31 482 DISEASES OF THE NEKVOUS SYSTEM due to, or associated with, this special finer cellular degeneration, for the brain may be greatly injured, badly developed, full of cysts or tumors and disease, yet the epilepsy does not always appear. V. Psychogenous Epilepsy. — ^There is a disease in which the patient suffers from apparently true epileptic convulsion, of grand-mat type. Psychoanalytic and clinical study, however, shows that the convulsions are of psychic origin, due to some irritating mental state. The convulsion is thought to be the result of this disturbing state. Pathology. — The body of the epileptic shows sometimes skin erup- tions and ulcers, the result of treatment. There are often evidences of local injuries and fractures due to falls. The organs may show vices of conformation. The uterus is frequently infantile or sharply flexed. Deformation of the occipital bone or the atlas so as to produce narrow- ing of the upper spinal canal has been noticed. The brain may be unduly large or small, but there is nothing constant in this, nor is there an ab- normal difference in the weight of the two hemispheres. The convolu- tions show many anomalies, but there is in them nothing specific. On the whole the convolutional type is a simple one. The pathological change found in more or less advanced epilepsy is a progressive gliosis with cell degeneration in the cortex and basal ganglia, and the cornu ammonis. Turner finds some characteristic changes in the cells of Betz and blood-vessels. In old cases there is often a chronic leptomenin- gitis, and vascular changes due to the frequent congestions of the brain take place. These consist in varicose and fusiform dilatation of vessels with evidence of small hemorrhages. Slight degenerative changes in the nerve-fibres are also observed. While cortical degeneration and gliotic foci are formed in epilepsy, they are the expression of some pathogenic state not the cause of it. The epileptic fit itself is a special manifestation of the disease which may be of toxic or bio-chemical of psychic origin. It is not likely, however, that the psychic factor can do its work on any but a psy- chopathic brain. It is probable that in true epilepsy there is an insta- bility of certain cell layers of the cortex cerebri, and a tendency to degenerate. Whether this is due to a congenital weakness, or the action of endogenous poisons, or both we do not yet know. The many elaborate investigations that have been made to ascertain whether epilepsy is not due to some toxic cause or perverted metabolism or endogenous ferment, have not established anything. Even in alcoholic and lead epilepsy, the toxic agent is probably a secondary one. The diagnosis is based on the character of the attacks and has to be made from hysterical and various toxic and symptomatic convulsions. The aura, the scream, the quick loss of consciousness, the dilated, pupils, the tonic convulsion, the bitten tongue, the emptied bladder, FUNCTIONAL AND DEGENERATIVE DISEASES 483 temporary loss of reflexes are all characteristic. The hysterical patient sometimes, but rarely, loses complete consciousness, the epileptic almost always. Hysterical patients do not hurt themselves in falling or bite their tongue, and their muscular movements, while irregular and violent in character, are yet co-ordinate, i.e., they throw themselves about, kick, strike, etc. Their attacks often are produced by emotion and ended by some powerful mental or physical impression. They rarely occur in sleep. The slight rise of temperature in epileptics rarely occurs in hysterics. Petit mal and epileptic vertigo are distinguished by the sudden lapse of consciousness and by the sudden pallor and fixation of the eyes, dilatation of the pupils, and slight twitchings of the face. Nocturnal convulsions are usually epileptic. Eclampsia, or acute symptomatic and refiex convulsions, cannot always be distinguished from epilepsy. The history of the case, the irregular and often prolonged character of the fit, may enable one to make the diagnosis. Course and Prognosis. — Epilepsy shortens life to some extent; most subjects do not live beyond the age of forty or fifty. About 10 per cent, become demented or insane. Five or ten per cent, get well. The remainder reach a certain stage of severity in their disease and continue in it for years. This severity depends on the treatment, the nature of the attacks, and the extent of degeneration which the organism shows. While unquestionably treatment cures or suppresses the disease in some cases, it disappears spontaneously in others. Epilepsy developing after adolescence is not easily curable, but is rather easily controlled and is not so serious, except sometimes in old people. The prognosis of petit mal is worse than that of grand mal; that of the two combined is worse still, yet not hopeless. The psychical form of epilepsy is the least amenable to treatment. Epileptic insanity and dementia are incurable. Death occurs rarely in the attacks except in terminal stages. Yet the status epilepticus is always a source of danger. Epileptics who have only a moderate number of attacks six to fifteen yearly, can get along comfortably for years, doing their work and enjoying a fair share of the duties and pleasures of life. Treatment. — The first and essential rule of treatment is to take cases early and treat them vigorously from the start. Children who have had a few convulsions during the first three or five years of life should be treated as if they might develop epilepsy between the ages of ten and fifteen or earlier. The recurrence of a fit between the ages of five and ten should excite apprehension and call for the most diligent treat- ment. Another rule is that when epilepsy is recognized in children the case should be treated constantly for at least three years after all attacks have ceased. 484 DISEASES OF THE NERVOUS SYSTEM Constitutional Treatment. — -We should use measures that increase vasomotor tone and strengthen and steady the circulation. Nothing- does this better than water. Epileptics should be given showers, douches, cold sponge baths, or wet packs according to their needs and opportunities. They should also drink water freely. Again, the nerv- ous system is greatly steadied and quieted by mental occupation that interests one. Nothing is more unfortunate than the idleness often enforced on epileptics. I have seen the disease absolutely checked by having a boy learn a trade that he liked. A second important indication is diet, and the prevention of in- testinal decomposition. In "petit mat particularly an absolutely non- irritating diet, such as milk, fruit and bread, will quickly lessen or stop the attacks. Meats can be taken in moderation if eaten slowly. As a rule it is a little safer to keep meat out of children's diet for a time; but in adults it is not necessary, though it should be given in moderation. A practically salt-free diet is advisable part of the time. Removal of Irritating Causes. The rheumatic, gouty, and so-called tuberculous diatheses do not stand in any close relation to epilepsy. The condition known as lithsemia, however, in which there are insuf- ficient oxidation and excretion of products of tissue waste, needs atten- tion. Hence the use of bicarbonate of potassium, salicylate of sodium, the alkaline mineral waters, and a restricted diet are not rarely indicated. The importance of reflex irritations has been much over-estimated. Still they must be considered. The most serious are those arising from the gastro-intestinal tract, the sexual organs and the eyes. Phimosis if present must be relieved, and masturbation or sexual excesses stopped, if possible. It is admitted now that removal of the ovaries, even if diseased, never cures true epilepsy, though it may help hysterical convulsions. Astigmatism and hypermetropia should be corrected; also ocular insufficiencies if these are pronounced. Proper attention to the frequent constipation and dyspepsia is of course necessary. Specific Treatment. — ^Physicians who undertake the treatment of epileptics often do not realize the seriousness of their responsibilities. Many, I fear, simply give a little bromide, stop the meat, circumcise the boy, and say they think the child will outgrow it. But children do not outgrow it; they steadily get worse unless something definite is done, and well done for a long time. With proper, prompt, and pro- longed treatment, the attacks can be entirely controlled in 5 to 10 per cent, of cases, and I believe more. They can be greatly controlled in over one-half the cases, so that the patient may be able to continue his education and do some work in life. But this cannot be accomplished FUNCTIONAL AND DEGENERATIVE DISEASES 485 by any casual dosing or occasional consultation with some high authority. The physician should approach the responsibility of a case of epilepsy as he would that of a mortal surgical condition, in which much depends on knowledge and attention to all the details of a long technic. It is conceded that the colony treatment of epilepsy is the one which approaches most nearly the ideal in effectiveness, but it cannot be applied to all classes — at least, for a long time — and perhaps never to a certain rather large percentage. There must always, therefore, be a good many epileptics who have to be treated at their homes, and whose care must be directed by the family physician or the specialist. For this class of persons I have gradually evolved a somewhat specific treatment of epilepsy, which seems to produce the most satis- factory results in those cases in which a reasonable opportunity for therapeutic effort exists. That is to say, cases wdiich are not of verj^ long standing, and which have not already undergone serious mental deterioration, and cases in which the mental and physical degenera- tion, at the beginning, is not of a very high grade. The features upon which emphasis must be laid, in the treatment of epilepsy, are: First, the fact that the course of treatment about to be instituted is to last for at least two j^ears, and that all measures prescribed must be carried out with the greatest fidelity and exactness during that time, no matter how well the patient may seem, or how unnecessary regimen and drugs may appear to be. The preparation and outlining of treatment should receive the care and attention such as is given to a capital operation. Second, the use of the pure bromide of sodium or some other salt (there is no special merit in mixed bromides) in combination with the glycero-phosphate of soda or of soda and lime so that a patient takes on an average 60 grains of the bromide of soda and 20 to 30 grains of the phosphate, in twenty-four hours. To this combination iron and a little arsenic may be added at times. By the combination of proper soluble phosphates with bromide and ehminative measures, the depressing effect of the bromide can be greatly lessened and the patient can continue bright and active under a fairly large dose of the drug. This has been tested by me now for fifteen years, mainly in private practice, but also in dispensary work. The maximum dose of bromide which can be taken in this combination is sometimes as high as 90 grains a day, but rarely more, and not often as much. The phosphate should occasionally be omitted, and the form changed. Certain patients cannot take bromides, however, and are not benefited by them in any way. Third, I have found it often an efficacious plan in treatment to in- termit the medication for either one or two days in each seven. During 486 DISEASES OF THE NERVOUS SYSTEM these days, e.g., a Wednesday and Sunday, the drug is stopped, and in its place is given, three times a day, before meals, a tumbler of hot water, and with it an alkaline laxative. The ordinary tablet of rhu- barb and soda, with nux vomica, usually answers this purpose. Some- times 20 grains of bicarbonate of soda are enough. Its purpose is to flush out the stomach and bowel and cleanse the gastro -intestinal canal twice a week, thus preventing the accumulation of drugs and toxins. After meals on these days I sometimes give 10 drops of tincture of iron or some needed tonic. I have not seen any access of convulsion during the day or the day after such intermission. With this stomach treat- ment there should be in many cases a colon irrigation using a hot high bicarbonate of soda solution. This is given about once a week. Some- times in its place I use an injection of a pint of warm sweet oil at night followed by a simple enema in the morning. Fourth, an important measure which I employ in the treatment is the securing of active physical exercises for about twenty or thirty minutes, at least, three times a week. This must be done either by some active sport, like boxing, by exercises in the gymnasium, by tennis, skating, or by the simpler methods of chopping or sawing wood skipping the rope, dancing, or punching the bag; but the exercises of whatever kind should be short and to the point of free perspiration. After this exercise, the patient is given a cool bath. Delicate persons, women, and those who are unable to carry out such exertion, I direct to purchase a "hot-box" and take a hot-box sweat, followed by a cool bath, three times a week. These boxes are not expensive, and can be set up with little trouble. Fifth, the question of diet is attended to, as indicated above. The medication should be increased rather than diminished as the patient gets better; he should take more, if possible, at the end of the second year than at the beginning of the first, if the attacks are controlled. After four years one can feel safe, not before. There are few other drugs of any importance in epilepsy, though freak cures happen under all kinds of measures. Tincture of simulo seems to have some value in doses of 5i- ter in die upward. Atropine in very large doses (gr. 3^^o to j-^s) gradually increased sometimes helps petit-mal types strikingly. Veronal and luminal may sometimes be given in cases in which bromides have no effect, or to aid their effects. There are some cases in which a glandular defect exists, hypothy- roidism being the most frequent in my experience. Small doses of thyroid, gr. 3^ to gr. ii., may be indirectly helpful. The other glands may be used as indicated. They only act as adjuncts. Growing children must, as a rule, be taken from school. This is un- fortunate, but it seems indisputably necessary. The education, however, FUNCTIONAL AND DEGENERATIVE DISEASES 487 should be allowed to proceed and regular fixed short hours of study be given. The child should be allowed to live as nearly a normal life as possible. If old enough, some can be taught a trade or some handi- craft with advantage. The grown person should be urged to adopt or continue in some occupation, of which an out-door one is preferable, but any occupation is preferable to idleness. The most difficult cases after the degenerates and those with organic brain disease are the bright, precocious children who develop yetit rnal alone and who have daily attacks. They are usually not amenable to bromide medication in any form. After a thorough trial of this, however, even to the point of bromidation, and using every possible tonic and eliminant, the patient is best left to hygienic measures alone. This treatment of epilepsy always presupposes a preliminary elimina- tion of all reflex causes. If the epilepsy is local {i.e., Jacksonian) or even if it began as a local affair, it is advisable to operate, and if any source of irritation is seen in the cortex it should be removed. Oc- casionally this does good, but not always. Often epileptics are brought to us with scars on the scalp and a history of some old injury, perhaps even of fracture. If the epilepsy is general, however, and does not seem to have started from the injured zone, operations are of no use. It goes without saying that there are many epileptics who are too advanced in the disease or too degenerate for treatment and who need only custodial measures. CHAPTER XXI THE PSYCHO -NEUROSES CONSTITUTIONAL INFERIORITY, HYSTERIA AND ALLIED STATES, PHYCHASTHENIA Introduction. — The term psycho-neurosis is given to a number of dis- orders in which the nervous symptoms are in the main dominated or caused by the mental state. This mental state is a morbid one, but is not of sufficient degree to call for custodial care or justify applying to it the term insanity. It is just a minor psychosis. There is a difference of view, as to the grouping of the psycho-neuroses. One has to follow some practical method, and I include under this term; (1) The psychopathic diathesis or constitutional inferiority. (2) Hysteria and allied states. (3) Psychasthenia. (4) Certain mild and abortive forms of the major psychoses. The study of the pathogenesis of the psycho-neuroses takes one into difficult and unsettled fields, but the situation as between clinical and dynamic neurology may be stated as follows: The clinician finds that an apparently healthy person has gradually or suddenly developed an automatic state, a fixed idea, or a functional paralysis. He inquires into the causes and finds (a) a nervous heredity (e.g., the father alcoholic, the mother tuberculous) ; (6) the patient herself of nervous type and (c) a sufferer from an emotional strain or shock followed by the psycho-neurosis, (d) He learns the patient's mental make-up and intellectual equipment, the temperament and the bodily condition. The data gained under a, b, c, d, tell him the cause of the disorder and show along what lines to work for cure. This is descriptive or clinical neurology. But the dynamic neurologist goes further and searches into the intimate mental mechanism of the process by which the mind became thus mor- bidly changed. This mechanism according to some consists in a blocking of association processes and a limiting and re-grouping of neural units. Thus the phenomenon of double personality, hypnotism, hysterical crises, trance, are explained, if it is an explanation, by supposing that there is a break and re-grouping of the association- work of the brain. Ordi- narily the associations aroused by the stimuli of thought, emotion or sense have free play in accordance with what the individual's environment and training have developed. If he is hypnotized or obsessed this free play 48S THE PSYCHO-NEUROSES 489 of association does not take place. The patient hears and sees and thinks only what is suggested to him, by the hypnotizer or by his dominating idea. All his mental activity is along a particular group of associations. At the synapses of the nerve cells, there is a blocking off of the ordinary to-and-fro play of the neural currents. This same process occurs when the personality becomes changed. One neural unit of association ac- tivities underlies the personality of Mr. Hyde and another group those of Dr. Jekyl. This is the mechanism of trance-states of cerebral auto- matism, and even of absent-mindedness and intense attention. It may be applied to the explanation of an uncontrollable and consciously foolish fear or a hysterical paralysis. We do not know the exact psychic or physiological process by which this blocking, and re-grouping of associations takes place. It is done by effort of the will, by fatiguing the attention, by the action of powerful emotion, by endogenous ferments perhaps, and by chemical poisons. Whether the Freudian school accepts the dissociation theory I do not know, and it is difficult to find out. But if so it would be only a detail in their explanation of the mechanism of these same phenomena. With this school the forces of the subconscious would be the agent, which in struggling to fulfil desire caused blocking of the synapses, neural dis- sociations and the phenomena of altered personality, of hysteria and trance. The Freudian school finds that the subconscious mind is the re- pository of a libido or desire whose urgings have been with varying success repressed and forgotten. Here are stored up the memories and instinctive trends of infancy, the emotional shocks and painful experiences of child- hood. Though repressed and pushed back into the subconscious, yet they still exist as potent ''complexes" and may be a source of trouble- some mental states and bodily symptoms. The libido, unable to force itself into consciousness and express itself in the normal infantile way takes devious paths of activity and produces its obsessions, paralyses, etc., in trying to secure satisfaction. This is shown through psycho- analysis, by studying dreams and by various other diagnostic methods. The result leads to the view that the changed personality, the spasm, the anaesthesia, the paralysis, the exhaustion are the expressions of a suppressed wish. This psychic mechanism offers a satisfactory explanation to some. It is a poetical, but not an adequate, practical or always safe method for the practising neurologist. For to him as a clinician the causes leading to instability, obsession, the dominance of fear are not altogether or mainly subconscious. In most the influences of heredity, of a constitutionally weak brain, of physical disease, of obvious and conscious suffering, of maladjustments. 490 DISEASES OF THE NERVOUS SYSTEM poor insight and defective education are the vitally important things, often not easily recognized but obviously to be treated. The subconscious psychic factor is certainly often only an extra and minor element. It is the final slight impact which makes an unstable structure fall. Often it is not subconscious really, and if present has only an academic and metaphysical importance. Hence, I believe in treating the psycho-neuroses, by correcting the general instability, educating the inferiorly developed, removing irrita- tions, and trying to make a perhaps rather poor human system carry its proper load. True psycho-therapeutics is really a form of education or re-education. It belongs to no school, but is part of ancient medical art and physicians have known, and more or less applied, the method for centuries. CONSTITUTIONAL INFERIORITY. PSYCHOPATHIC DIATHESIS This is a term applied to a condition characterized by a congenitally unstable, nervous system, and more particularly an unstable mental condition. The patients, however, as a rule, are sufficiently under control of themselves and their morbid tendencies, to be responsible or partially responsible for their acts. It includes persons who suffer from "nervousness," constitutional despondency and constitutional excitement and morbid instincts. While constitutional inferiority may exist without developing any well-marked neurosis or psychosis, some episodical outbreaks often occur. When the trouble is more especially associated with weakness of will, feebleness of mind, incapacity to decide or inhibit, or to initiate, it is in particular a quantitative inferiority. When the mind is not retarded or intellectually defective, but is dominated by morbid instincts (sexual or criminal) or unsocial trends, the person is more particularly a case of qualitative inferiority and he is then sometimes called a psychopathic personality. Various major psychoses may develop on this soil. Constitutional inferiority exists as an independent state but it may underlie the following conditions: Neurasthenia. Psychasthenia. Hysteria. Hypchondria. Dipsomania. Compulsive and impulsive psychoses. Persons with a constitutional inferiority may, under proper environ- ment and educational guides, live fairly normal and useful lives. If, THE PSYCHO-NEUROSES 491 however, they are brought up in such a way as to allow indulgence in abnormal impulses or habits; if they thus acquire vicious modes of living or go to excesses in any direction, they may develop criminality or some of the formal types of insanity, such as paranoia or even dementing types of insanity, like dementia praecox. It is often somewhat difficult to say whether a person is simply a psychopathic personality, or whether he has not a constitutional inferiority in which criminal tendencies have been allowed to develop. It is often possible by the force of teaching and the influences of environment, to change the morbid tendencies, so that an inferior or psychopathic personality may become a fairly useful man. Great care should, there- fore, be exercised in applying to a patient the term "constitutional inferiority" to excuse criminality, since there may have been only a slight tendency which has grown through indulgence. HYSTERIA Hysteria has been defined as the expression of a symbolic conversion of psychic into physical states. This condition in which the psychical is converted into physical is said by followers of the Freudian School to be an index of the aspirations of the race. For the manifestations of hys- teria are thought to be the expression of subconscious instincts or desires. Such a view need not interfere with a pragmatic method of dealing with the subject such as I adopt in the following description, in which the mental traits of the hysterical, and the physical phenomena they show are presented in clinical narrative without forcing into them philosophical and questionable mechanistic interpretations. Hysteria is in its broadest sense not a specific disease but a morbid biological reaction, in which morbid bodily disturbances are caused by more or less subconscious mental states. Hysteria is a widely prevalent quality of the human character. It displays itself in brief and often trivial episodes, such as unreasonable emotional explosions of crying and laughing, and in motor and visceral crises. These are often to be considered only as symptoms of a natural instability, and they are often associated with other real and organically morbid conditions. When this reaction is very severe, persistent and disorganizing, we have major hysteria, which is distinctly a disease or fixed symptom-group having a definite origin, course and symptoms. A slight degree of this hysterical reaction is very common and can hardly be called abnormal. Nearly all women are said to be somewhat hysterical. When the condition occurs occasionally and under stress of emotion, it is episodical or minor hysteria. 492 DISEASES OF THE NERVOUS SYSTEM Etiology. — Of the predisposing causes of hysteria in general, heredity is the most important. In about 75 per cent, there is a history of hysteria, or some neurosis or psychosis in the parents. The disease is transmitted more often by the mother. Heredity is particularly apt to be important in the hysteria of children; it is a much smaller factor in hysteria of adult males. Hysteria is a disease of early adult life, most cases occurring between the ages of from fifteen to twenty- five in females; it occurs later in males. Hysteria attacks children between the ages of eight and fifteen, chiefly between eleven and fourteen. The trouble affects women more than men in the propor- tion of four to one, varying much with race, climate and occupation. Hysteria major, however, occurs oftener in men than women; it occurs in adult and middle life and there is often no hereditary history. These statements are based on personal experience of this type of hysteria as seen in this country. They do not correspond with the observations of European observers or of alienists, but are certainly true for the type of disease which I am describing. Hysteria occurs in all classes of life, but rather less frequently in the middle classes than among the poor and the very rich. Male hysteria is more frequent in the poorer classes who are subjected to the influences of alcoholism, poverty, injuries, etc. Hysteria is certainly much less frequent in its severer forms in this country than in some parts of Europe, par- ticularly France. In my experience it is much less frequent than epilepsy in the northern and eastern parts of this country. It occurs, how- ever, quite frequently in the negroes and also in the Latin races of this country. Bad methods of education and bad sexual habits undoubtedly tend to promote the development of the disease. The most important single exciting factor is powerful emotion, particularly fear. Other emotions of an allied character — excitement, sorrow, anxiety — may bring on attacks. The disease can be developed by imitation. Injuries combined usually with mental shock are fruit- ful causes of producing hysteria. The infectious fevers, syphilis, diffuse hemorrhages, the poisons — -lead, mercury, and tobacco — the adminis- tration of ether, mental and bodily and sexual excesses, are all important agents in developing the disease. Acute alcoholism is not very rarely followed by hysteria. General Symptoms. — Mental. — Mental hysteria shows itself clinically in certain morbid moral manifestations. The typical moral hysteric, for example, is intensely self-centred, craves for sympathy and is desirous of being always in the centre of the stage. To secure her ends she exaggerates symptoms, tells lies, practises cunning deceits and constructs false and ingenious stories. Such patients, in order to gain their end and excite human sympathy, will even injure and mutilate THE PSYCHO-NEUROSES 493 themselves or will prevent the healing of their wounds. In fact, all manner of devices are used to make it appear that they have some ailment or painful condition which demands continual attention. Without her headache, or exhaustion, or neuralgia, the hysterical woman knows, or her subconsciousness knows, she would not count in the household for more than she is worth there. Aside from the peculiarities of the hysterical mind along the moral lines, the hysterical person develops other characteristic mental phe- nomena. These consist of dreamy states, periods of amnesia and auto- matism and also disorders of mentality which go by the name of hysterical insanity. The great proportion of patients who have disturbances of bodily function such as paralyses, anaesthesias, neuralgias, may be practically free from the exaggerated form of moral hysteria, and appear only perhaps rather dull and depressed, nervous and apprehensive. Physical. — The varioua:.forms in which the somatic phases of hysteria show themselves may be grouped as follows: Convulsions; tremors; clonic and tonic spasms, paralyses of the type of monoplegia, hemiplegia and paraplegia; neuralgias and anaesthesias. There are also vasomotor and secretory disturbances; and disturb- ances of the autonomic system shown in vago-tonic and sympathetico- tonic attacks. The reflexes are to some extent disturbed. Special Types. — Hysteria minor or the hysterical temperament is characterized by an interparoxysmal condition of emotional weakness, nervousness, hypersesthesia and pains, and by crises of an emotional, motor, sensory or visceral type. In hysteria minor there are no perma- nent objective marks like anaesthesia and paralysis, and rarely convulsive seizures. The patient, who is almost always a girl or young woman, gradually develops an undue sensitiveness, the mind is depressed, and she gets easily alarmed. She has feelings of nervousness and lacks con- trol over the emotions, she laughs and cries very easily and yields to every impulse. She suffers from headaches, which are usually diffuse and often severe and chronic, and from spinal pains. She sleeps, as a rule, rather badly and often has disagreeable dreams. She has, under any little excitement, sensations of tickling, fullness or choking in the throat, forming the condition known as globus. Excitement also brings on attacks of trembling or chilly feelings which come and go. There is more rarely a considerable amount of vasomotor instability, as shown by flushings and by coldness of the extremities, and vago-tonic attacks. She has with more or less frequency distinct crises of an emotional character, during which she laughs or cries without apparent cause, or at least to an extent beyond her control. She may have attacks of 494 DISEASES OF THE NERVOUS SYSTEM vomiting or headache, or of intense mental excitement amounting almost to delirium. In some cases the patient has somnambulic attacks at night, or she may have under a little excitement attacks of cerebral automatism during which she involuntarily does things that she is entirely unconscious of when she comes out of the attack. The crises are fol- lowed by a copious discharge of very light urine. With all these symptoms there may be the moral traits above described. Hysteria major is characterized by interparoxysmal manifestations of anaesthesia, paralyses, contractures, tremors, peculiar mental condi- tions, and by paroxysms of an emotional, convulsive, or other serious nature. Hysteria major is what is usually meant when one speaks of hysteria as a disease; it includes also hystero-epilepsy. The onset may be gradual, but not infrequently it follows some shock, the first symptom being a convulsion, a paralysis, or some emotional outburst. The crises of hysteria may be: Psychical; motorial; sensory; visceral; angio-neurotic. The most common of the paroxysms of hysteria are emotional outbursts of crying or laughing; after this come motor disturbances in the shape of convulsions of various types or of hemiplegia or other form of paralysis. Besides this we have attacks of severe pain, forming neuralgic crises; attacks of nausea, gastralgia and vomiting, forming gastric crises; much more rarely there are prolonged attacks of hysterical coughing, hiccoughing, sneezing, or rapid breathing. The hysterical seizure may also take the form of attacks of trance and lethargy, catalepsy amnesia, and cerebral automatism. The emotional crises are episodes of the same nature as occur in minor hysteria. Hysterical convulsions have two rather well-defined types. One of them is the ordinary form of hysterical convulsions; the other is a much more severe disturbance in every way and is known as a hystero- epileptic or hysteroid attack. In the hysterical convulsion the patient, under the influence of some excitement, injury, or acute gastric disturb- ance, rather suddenly falls down and begins to go through various irregular movements of the, body, such as thrashing with the arms, kick- ing with the legs, throwing the head from side to side, rolling about on the bed or floor. In the more distinctly convulsive seizure the hands and arms and fingers are flexed, the legs and feet are extended, the eyes are generally closed, the eye-balls often converged or moved about irregu- larly, the pupils dilated. There is some lessening of sensation over the body and of the conjunctivae. The patient often utters noises or screams at intervals. She may bite her lips, but does not bite the tongue, nor does she ever hurt herself in her various contortions. The attack may last for half an hour to several hours, unless some measures are taken THE PSYCHO-NEUROSES 495 to break it up. In other forms of hysterical convulsion there is simply a general shaking or trepidation of the body as though the patient had a chill; in other cases again the main type of movement is that of opis- thotonos, the patient rising up upon the head and heels and arching the body in as tetanus. Again the attack may consist simply of a little rigidity of the body or of a series of rhythmical movements of the head or trunk or limbs, the patient sitting up and oscillating the head or sway- ing the trunk or moving the arms, uttering at the same time incoherent words. In still other cases the patient simply falls down and lies un- conscious, like a person sleeping, for a few minutes or even an hour. In children the attacks may be associated with peculiar noises and move- ments in imitation of animals, such as the growling of a dog or the mew- ing of a cat. This condition is called therio-mimicry. In some instances the attack may be accompanied by or may end in a condition of mental excitement approaching delirium. The patients while suffering from these seizures generally appreciate what is going on about them, and will often lespond to some stern order for them to cease or will be brought to a state of quietude by pressure upon some part of the body which provokes pain. In women in particular, pressure over the ovaries or epigastrium will abort the attack; the application of cold water or an emetic will do the same. After a hysterical crisis or sudden shock, the patient may be found to have a paralysis of arms or legs or one side of the body. The Symptoms of the Interparoxysmal State.- — Between the crises of major hysteria the patient may be in a fair condition of general health, but usually presents certain definite chronic manifestations of the disease. Sensory Symptoms — These consist of superiScial and deep hyper- sesthesia and ansesthesia and anaesthetic disturbances of the special senses. When all these forms of anaesthesia affect one side of the body we have a typical major hysterical condition which may last for years. Cutaneous anaesthesia occurs in three forms: the common form is that of hemian- aesthesia involving one-half of the body; next in frequency is the segmental anaesthesia involving an arm or a leg or part of the face or head; rarest of all the forms is a disseminated anaesthesia occurring in the form of patches. These various modes of distribution are shown in the accom- panying figures. The anaesthesia is a pain anaesthesia chiefly. The tactile and thermic sensations are less markedly affected. Loss of deep sensibility is rare. The anaesthesia is in some cases transferable by means of magnets or electrical irritants or by suggestion. The anaes- thesia can also be lessened or removed temporarily by the application of magnets or coins or pieces of metal. For example, if a silver coin is fastened upon the anaesthetic area, in the course of a few minutes 496 DISEASES OF THE NEEVOUS SYSTEM or a few hours there will be a zone of normal sensation under and around the coin. Sometimes the temperature of the skin upon the ansesthesic part is lowered 3° or 4° F., and upon pricking the skin blood does not flow. The anaesthesia is oftener upon the left side in the pro- portion of three to one. Hysterical anaesthesias are not accompanied by subjective sensations as are organic anaesthesias. The skin reflex is usually abolished, as is the conjunctival reflex. The deep reflexes are lessened or unchanged. Anaesthesias of some kind occur in a very large proportion of the serious forms of hysteria major. They are rare, however, in children and are rarer in women than in men, in the author's experience. Anaesthesia of the mucous membranes is present chiefly in hemianaesthesia ; it then involves the mucous membrane of Fig. 226. — Hysterical loss of color sense and limitation of visual field. Color sense absent in left eye and field contracted; in right eye field less contracted; order of appreciation of colors from without in was yellow, violet, blue, red, green. the mouth and throat, and to a less extent that of the nose and glottis. Hemianaesthesia is usually accompanied by some hemiplegia and often by some tremor. Segmental anaesthesia is also often accompanied by some degree of paralysis of the part. Visual anaesthesias. One of the most common of the permanent stigmata of hysteria is a concentric limitation of the visual field and a disturbance in the color sense. Complete loss of this sense may take place or there may be a variation in the ways in which the colors are per- ceived. There may be also a distinct diminution in the acuity of vision or even a complete loss of sight of one eye or both eyes. This hysterical amblyopia usually comes on suddenly and may last for weeks. It is not always a true blindness and the patient left to walk about a room does not run into obstructions. The visual disturbance is most common THE PSYCHO-NEUROSES 497 with hemianaesthesia. It is more marked on the affected side, but exists to some extent on the healthy side. The Hmitation of the fields is shown in the accompanying cut (Fig. 226). Hearing. There is sometimes a diminution in the acuity of hearing of one ear, and this occurs, if present, in connection with hemianaesthesia. There may also be a loss of hearing to high and low notes, while hearing is apparently fairly good to notes of medium range; and finally there may be a diminution in hearing by bone conduction, while hearing by aerial conduction is but little impaired, this being due probably to an anaesthesia of the acoustic nerve. Disturbances of taste in the form of anaesthesia or paraesthesia are quite frequent and are important signs in hysteria. The loss of the sense of taste may involve only the back portion of the tongue and the palate. The sense of smell may be abolished, but this usually occurs in con- nection with hemianaesthesia. Fig. 227. — The three types of distribution of anaesthesia in hysteria; hemianaesthesia, segmental and disseminated. Z, hysterogenic zones. Hyperaesthesias and neuralgias. Pure neuralgias are somewhat rare in true hysteria, but hyperaesthesia and pains of various kinds are not at all infrequent. Hyperaesthesia occurs in the form of patches at differ- ent parts of the body. These sensitive points may, when pressed upon, bring on paroxysms of various kinds, and they are therefore called the hysterogenic zones (Fig. 227). The most common seat of these zones in women is over the ovaries ; in men, in regions corresponding to the ova- ries and on the scrotum. Hysterogenic zones, however, may be found 32 498 DISEASES OF THE NERVOUS SYSTEM just beneath the mammary gland, on the epigastrimn, along the spine, and in other places. These zones are sensitive areas; they can be made to disappear by applications of electricity and by refrigeration and coun- ter-irritation. Hysterical patients often suffer from local headaches, which are apt to be confined to the top of the head or to the sides near the temples. The pain is severe, sharp, and boring, and may exacerbate with such intensity as to produce symptoms almost resembling menin- gitis. The spot-like pains are known as hysterical clavus. There may be also a peculiar persistent local pain of a similar nature to clavus called topalgia or place-pain. Hysterical patients occasionally have migraine, facial neuralgia, and intercostal neuralgia. Much more often they have pains along the spine, producing symp- toms of spinal irritation. Hysterical patients also have at times attacks of vagatonia and of palpitation and pains over the heart, constituting what is known as pseudo-angina. Such troubles are much more frequent in women. Arthralgias are painful joints in which the condition simulates a chronic arthritis but has none of the evidences of inflammation, except sometimes a slight oedema or effusion. Motor Symptoms. — The motor symptoms of hysteria are paralyses, myasthenia, contractures, tremor, and choreic and ataxic movements. The paralyses of hysteria take the form of hemiplegia, paraplegia, and monoplegias. Hysterical hemiplegia occurs usually rather suddenly, often as the result of some severe shock. The left side is more frequently attacked. The arm is most affected, the leg next, while the face is hardly ever involved. The paralysis is not an absolute one, and the patient is able to drag himself along. The deep reflexes are usually not exaggerated and they may be for a short time absent. The paralysis is thus a flaccid one. The gait of the patient is different from that of hemiplegia due to organic disease; in hysterical hemiplegia the patient drags the paralyzed leg after him, in organic hemiplegia the patient swings the paralyzed leg around in a half-circle (Fig. 228). This pecuharity of the gait, the absence of abnormal reflexes, the absence of paralysis of the face, and the presence very commonly of other hysterical stigmata are sufficient to enable one to make the diagnosis. Sometimes the face on the affected side is slightly drawn by a spasm, so that it appears to be paralyzed when it really is not (Charcot) . Monoplegias affect the arm or leg, very rarely indeed the face, occasionally the eye muscles, and most commonly of all the muscles of the larynx. Hysterical monoplegia is usually accompanied by anaesthesia of the affected part and by other symptoms of hysteria. In children hysterical palsies are almost always monoplegic, never THE PSYCHO-NEUROSES 499 hemiplegic. There are no serious atrophic changes or disturbances of the electrical reactions. Hysterical eye palsies show themselves in the form oftenest of an insufficiency of the internal recti. In hysterical palsy of the larynx the adductors are psychically involved so that the patient cannot speak loud, and the condition is called hysterical aphonia. The trouble often comes on suddenly, the patient finding that he cannot speak above a whisper. The paralysis is mental and therefore the adductors can be approximated in coughing. The trouble is distinguished from Fig. 228 — -Hysterical hemiplegia showing flaccidity of the paralysis. — (Loiide.) laryngeal inflammation by inspection of the affected part. The tongue and other muscles of articulation are in very rare cases also in- volved, and hysteria may produce symptoms resembling a bulbar paral- ysis. Paraplegia is a rather uncommon form of hysterical palsy; it is usually brought on by emotions of depressing character, often associated with some slight injury. It may be accompanied by a good deal of pain in the back, and the form of disease which was once known as "spinal concussion" consisted in many cases of hysterical paraplegia combined with traumatic disturbance of the spine. In hysterical paraplegia there 500 DISEASES OF THE NERVOUS SYSTEM is very little wasting of the limbs and no change in the electrical reac- tions. The deep reflexes may be somewhat increased or normal; they are never absent. There is never any prolonged or persistent ankle clonus, but there may be a short or spurious clonus due to a general exaggerated irritability of the nervous system. The sphincters are never involved except temporarily or through some complication. Myasthenia is a frequent, peculiar, and interesting symptom occur- ring in the interparoxysmal stage of hysteria. It consists of a more or less temporary feeling of weakness of an arm or of the legs or whole body. Fig. 229. — Hysterical contracture of hand. Thus a person in lifting a dish from the table suddenly feels the arm give out, and if not careful the dish is dropped ; or while walking the patients suddenly feel as though they had lost all power in the lower limbs. This myasthenic condition is generally temporary, but it may be so permanent as to produce a certain degree of monoplegia or paraplegia. The myas- thenic condition may precede a paralysis. It presents no objective signs in the way of electrical reaction; it involves a whole member, not a single group of muscles. Contractures.- — In some forms of hysteria there is a tendency for the muscles to undergo contracture under slight mechanical stimulation, much as pressure or a blow. This tendency to contracture in hysteria is called the contractural diathesis, and it is an important sign. The contractures may be temporary, disappearing soon after the exciting cause ceases, or they may develop independently and last for a long time. They usually but not always disappear in sleep, and under anaesthesia. They involve the legs, arms, and facial muscles, and may THE PSYCHO- NEUROSES 501 be associated with paralysis and anaesthesia (Figs. 229, 230). The con- tracture is usually a spasmodic, not a paralytic one. Tremor occurs in hysteria in a considerable proportion of cases, more especially those in which there are hemiplegia and hemiansesthesia. Hysterical tremor simulates all the various types. The common form Fig. 230. — Hysterical contracture of toe. is one in which the oscillations occur from five and a half to seven and a half times a second, and is jerky and irregular in type. It ceases for a time when the person is quiet or lies in the horizontal position; also during sleep. It affects the head and tongue as well as the ex- tremities, the latter more upon one side than the other. It may be chiefly in the lower limbs. Sometimes it has the type of an intentional tremor, ceasing on rest of the hand and increasing when the hand is moved, as Repose. Movement. Fig. 231. — Illustrating intentional tremor in hysteria. (Tourette.) in raising a glass to the lips (Fig. 231). It then resembles exactly the tremor of multiple sclerosis. There may be a slow tremor of four to five and a half oscillations per second; this persists during rest and is but little modified by voluntary movements. It imitates the tremor of paralysis agitans. Finally, there may be a rapid tremor of eight to nine oscillations per second. This also persists during repose and is but little modified by movements. It imitates the tremor of Basedow's disease, alcoholism, and neurasthenia. 502 DISEASES OF THE NEEVOUS SYSTEM Trophic Disorders. — -In hysterical paralysis a slight amount of atrophy occurs, but only such as would naturally follow disuse of the part. Cutaneous eruptions and dystrophies practically do not exist or, if pres- ent, are the result of complicating disorders. Visceral Symptoms. — Hysterical patients often suffer from dyspepsia and constipation, also from anorexia, and in some cases from vomiting or regurgitation of food. Occasionally the anorexia and vomiting be- come persistent; the patient refuses food or rejects all that is taken; she emaciates, becomes weak and bedridden, and develops into that partic- ular phase of hysteria known as anorexia nervosa. In these cases, along with the aversion to food and vomiting, there may be a great deal of gastralgia. The urine in hysteria is apt to be of low specific gravity. Some- times there is retention of urine; in extremely rare cases there is a con- dition known as ischuria and anuria, in which for several days extremely small quantities of urine are passed, owing apparently to a suspension of the functions of the kidney. Such cases should always be carefully investigated. Vasomotor symptoms are very common. The autonomic and sympa- thetic tonus is often involved, causing vaso-vagal attacks and gastro- intestinal disturbance with pain, diarrhoea and vomiting. These dis- turbances of the sympathetic are also shown by flushings and pallor, cold extremities, and at times an oedematous condition of one or more extremities. This oedema may be of the ordinary pale, waxy character, pitting upon pressure. In other cases it has a peculiar bluish tinge and it does not pit; the hands, which are the parts generally affected, are several degrees below the normal in temperature, and the limb resembles in some respects the condition in Raynaud's disease. Gangrene, how- ever, never supervenes. This form of oedema is known as the blue oedema of hysteria. There occur in hysteria febrile attacks, and much has been written upon the subject of hysterical fever, which does not exist. Hystero-epilepsy. — ^The form of hysteria which shows itself by the development of severe crises known as hystero-epileptic attacks is extremely rare in this country, at least in its typical phase. It has been particularly studied by the French writers Charcot, Richer, and others. Hystero-epilepsy, as this form of the disease is called, is a true hysteria and not epilepsy at all, nor a mixture of hysteria and epilepsy, though the name would suggest that that was the case. The typical attacks of hystero-epilepsy begin with certain prodromata consisting of a feeling of malaise and irritability which may last for several hours or a day. There is next the epileptoid phase, lasting from one to three minutes; THE PSYCHO-NEUROSES 503 third, a phase of contortions and grand movements, lasting one to three minutes; fom^th, an emotional phase, lasting from five to fifteen minutes; and, finally, a stage of delirium, lasting a variable time; the whole attack lasting from five to twenty minutes. In this country we occasionally see hysterical patients exhibiting one or two of these phases, but very rarely indeed do they ever go through the whole series. The patients who suffer from hystero-epileptic attacks generally during the interparoxysmal stage present many of the stigmata of hysteria, such as paralyses, contractures, and anaesthesias. Hysterical crises which take the form of convulsions or emotional seizures sometimes end or are associated with attacks of catalepsy or trance or attacks of amnesia and cerebral automatism. As these con- ditions all occur in other diseases than hysteria, they will be described elsewhere in connection with the subject of the disorders of sleep and of consciousness. Hysterical persons occasionally are attacked with violent and per- sistent hiccoughing or sneezing. Sometimes also there come on attacks of extremely rapid breathing or hysterical polypnoea, during which the respirations run up to fifty or seventy a minute. A hysterical cough sometimes occurs; and lasts for a long time. (Esophageal spasm with consequent dysphagia is another one of the somewhat rare phenomena of hysteria. Indeed, many of the symptoms referred to in this last para- graph may be grouped as tics or morbid mental habits, and not truly hysterical. Pathology. — There is no known anatomical change at the basis of hysteria. We do not find the marks of degeneration as we do in serious forms of insanity and epilepsy. Diagnosis. — The essential characteristics are the peculiar emo- tional condition of the patient, the past history of hysterical crises, the presence of the stigmata of hysteria, such as anaesthesias, limitation of the visual field, paralyses, and contractures. The variability of the symptoms, their susceptibility to influence under suggestion and rigorous moral measures, the absence of organic disease or of serious disturbance of nutrition, the sex and age, and the cause should also have weight in guiding us to our decision. The test of hysteria minor is simply the presence of a morbidly easy reaction of the mind upon the body. It has no absolute criteria. What is morbid must be determined by the judgment of the physician con- sidering the education and environment. Hysteria major is a rare disease and has a clear-cut syinptomatology. It has crises and the stigmata already described. Hysteria and Paychasthenia.- — -If a person has an obsession or fixed idea which consciously affects his mind and actions, but not directly his 504 DISEASES OF THE NERVOUS SYSTEM body or functions, he has psychasthenia, or an obsessive psychoneurosis. The obsession in hysteria is usually a subconscious one; in psychasthenia it is conscious. Diagnosis of special sympto7ns of hysterical manifestations. Hys- terical paralysis is characterized by the fact that there is no marked degree of wasting of the muscles, no electrical reactions of degeneration, the deep reflexes are preserved or exaggerated, and other marks of hysteria are present. Hysterical anaesthesia can generally be lessened over certain areas by the application of the magnet or can be made temporarily to disappear; it is peculiarly distributed in the way described under symp- toms and is associated with anaesthesias of the special senses. Hysterical contractures sometimes cease during sleep and generally under deep nar- cosis, and the use of an anaesthetic may clear up the case. They usually follow a fit, an injury, or an operation. They are somewhat increased on attempts to overcome them by force; they are usually associated with paralysis and anaesthesia and other hysterical symptoms. Hysterical convulsions. These differ from convulsions of epilepsy in the way best indicated by the following table : Hysterical Convulsions Epileptic Convulsions Brought on by the emotion or injury; The opposite in all these particulars, rarely any aura; no initial cry; move- ments co-ordinate; tongue not bitten, and patient never injures herself. Duration perhaps several hours with intermissions; consciousness generally preserved. Micturition and defeca- tion do not occur. No rise of tempera- ture; may be stopped artificially. Rarely occur in sleep. Although the differentiation here made usually holds good, there occur psychogenic convulsions in which all the phenomena of the epileptic fit are present. The hystero-epileptic attacks are so characteristic that a mistake could not be made. Prognosis. — The prognosis of hysteria in children is good. They generally get well, though in some cases there is a recurrence later in life. Minor hysteria under proper treatment usually gets well or at least is of little importance. The major forms when chronic are often intractable even under the best treatment. When a severe form of hysteria occurs in a person of feeble frame who is surrounded with a sympathetic family, the task of rescuing her from her disorder is a very arduous one. Traumatic forms of hysteria which are not infrequently associated with some actual physical injury are sometimes difficult to cure. Hysteria which is associated with some organic disease, such as THE PSYCHO-NEUROSES 505 a severe pelvic disorder or an organic affection of the central nervous system, has a poor prognosis. Hysteria in the male is generally curable, but it requires vigorous treatment, and spontaneous cure is by no means likely to happen. Major hysteria has a vastly better prognosis if proper treatment for it is instituted early. Treatment. — The treatment of hysteria maybe divided into mental, mechanical dietetic, and medicinal measures. By all odds, the most important factor in the treatment of hysteria is the mental treatment, and the most important primary measure to be taken is the isolation of the patient. She should be placed where she will not be surrounded by sympathetic friends; where life will be a regular one; where some occupation may be given which will engross attention, interest the mind, and call into play the physical activities. In the major forms of hysteria associated with anorexia, emaciation, anaemia, and possibly pelvic disorders, the "rest cure" as elaborated and carried out by Weir Mitchell forms by all odds the most successful means of treatment. In many cases of less severe character a partial rest cure in which the patient is separated from her family but is not placed under such severe restrictions may be all that is needed. In the case of children removal from home is often advisable, and the discipline of well-conducted schools is a most excellent measure. Re-educational treatment by therapeutic talks, by direct and indirect suggestion, by occupation, work, marriage, learning a craft or business; life in a sane environment, these are the real methods of cure. Psychoanalysis and the method of Freud have their adherents. The mechanical means used in hysteria are hydrotherapy, electricity, massage, and exercise, and they have symptomatic value. Of these measures hydrotherapy and electricity take the first rank. In hydro- therapy the douche or jet to the back, the shower and cold plunge, and the half-bath are the most efficacious. In the electrical treatment the static and faradic currents give some results. The static sparks often relieve contractures and lessen or remove the anaesthesias, and both forms of electricity seem to have a generally beneficial tonic effect. Doubtless the psychic element is the most important factor in this as in other mechanical measures. Massage is of some value in pro- moting nutrition and it also has a favorable sedative effect on many cases. Exercise, particularly of an active kind, such as stimulates the mind and interests one, is a measure of extreme value and one which has perhaps not been sufficiently appreciated. The drugs which can be recommended in hysteria are not numerous and their power is limited. Valerianate of zinc, asafoetida, iron, and the bromides are the most important of the nervines. In hysterical children a capsule containing two grains of valerianate of zinc and one of sulphate 506 DISEASES OF THE NEEVOUS SYSTEM of quinine is often efficacious. Gowers places more reliance upon the oil of turpentine in doses which should be increased to the point of strangury. In the treatment of hysterical convulsions the most efficient measure is the administration of an emetic, and this can be best done by giving hypodermically one-twelfth or more of a grain of apomorphine. Con- vulsions can be stopped sometimes by throwing water in the face or on the epigastrium; by firm and somewhat long-continued pressure over the ovaries; by the administration of valerian, aromatic spirits of ammonia, or compound spirits of ether. CHAPTER XXII PSYCHASTHENIA Psychasthenia is a chronic, non-dementing psychosis, characterized by the occurrence of imperative ideas by compulsive and impulsive acts, by morbid fears and conditions of agitating doubt. The term obsessive neurasthenia or obsessive psycho-neurosis is used by some instead of psychasthenia. Beard originally described many of these symptoms and classed them under the head of neurasthenia. Indeed, neurasthenia usually accompanies the condition, and many unstable people, when they get weakened or exhausted by work or disease, develop morbid fears and obsessions. So that part of the cure of psychasthenia is to treat the neurasthenia. Etiology. — The most frequent etiological factor is heredity; nearly one-half of my cases gave a history of direct or indirect mental disorder in the ancestry. I have seen several cases in which the obsessive psychosis occurred in mother and son or mother and daughter. Lues, tuberculosis, and alcoholism occur in the ancestry, but not so prominently as in other psychoses. The disease occurs in males and fe- males about equally. Symptoms of the trouble may be shown in children as young as eight or nine years; more than half the cases, however, de- velop between the ages of fifteen and twenty-five; nearly one-fourth of the cases between the ages of twenty-five and thirty, while one-eighth of the cases develop between the ages of ten and fifteen. A few cases occurred between the ages of thirty-five and forty, and it is seen again with some frequency at the time of the climacteric. I have seen three cases in which the disease developed after the age of sixty. The earlier the case develops, the more persistent, as a rule, are the symptoms liable to become. Among the most frequent exciting causes may be mentioned some kind of shock by fall or accident; next after this is alcoholic excess. Among other causes I have noted infectious fevers, an attack of chorea, a stroke of lightning, car sickness, educational strain. Engagements, marriage and pregnancy have been rather often a starting point of the disease. Symptoms. — The symptoms develop in four different types, though these are often intermingled, and, in most cases at least, three of the 507 508 DISEASES OF THE NERVOUS SYSTEM characteristic groups of symptoms are more or less present.^ These types are those characterized by (1) morbid fears, (2) by imperative ideas, (3) by a doubting mania, and (4) by morbid impulses. In 91 cases I find the order about as follows: Morbid fears, 37; obsessive and fixed ideas, 25; doubting manias, 18; compulsions and impulsions, 11. Psycho-analysis may show that these different groups have a different mechanism, but my experience is that they are clinically often allied and intermingled. Morbid Fear, Doubting Mania. — This disorder begins usually rather suddenly as a result of some slight shock, and perhaps of some causal and unimportant incident. A young man, while in church, or in a crowd, suddenly has a feeling of disturbance of consciousness, with something akin to vertigo. He is alarmed, the head seems full, the heart palpitates, and he feels as if he would faint. These attacks may be repeated. They have been called psychic seizures, or psycholepsy. Some of these seizures appear to be psychogenous vago-tonic attacks. After one occurs the patient becomes afraid to go in a crowd (agoraphobia) or in an enclosed place where there are people (claustrophobia; he may even be afraid to go out of doors without a companion. This state is accompanied with some general nervousness, depression, insomnia, and pressure or pain at the back of the head or vertex. He is often unable to pursue his work or study, and becomes worrying and introspective. After some months or a year the secondary symptoms disappear, but he may for several years feel a dread of going in certain places. Fear of storms and of lightning (astraphobia) may come on in much the same way, but this form of morbid fear is less disturbing as a rule, and often the patients are quite well as long as the sky is reasonably clear. Some patients are so sensitive, however, that they become nervous and depressed before storms, and can predict them as unerringly as do rheumatic or neuralgic sufferers. In fear of contamination and dirt (mysophobia) the patient is con- stantly washing the hands, changing the clothes, and trying to avoid contact with people. The sufferer will spend an hour or more in dressing and undressing or in preparing to go out. After a time almost all activity is curtailed by dread of contact with something, and the victim sits in a chair, or lies in bed with hands enveloped in cloths to protect him from the dust. All the interests of life are lost in this one absorb- ing idea and fear, though the patient is not demented or maniacal. ^ The condition of obsessive and fixed ideas has been called compulsive in- sanity. Compulsive acts may result, but they are accompanied by consciousness of the nature of the act, by feelings of anxiety, and by attempts at resistance. The victim of morbid impulses is said to be suffering from impulsive insaniiy. Here the impulses spring suddenly into consciousness, and acts follow at once without the patient being able to resist them. These impulses vary in character and severity from the impulse to touch a lamp post to that to commit a murder. PSYCHASTHENIA 509 There is a decided depression, but not a true melancholia. If the patient, however, is driven by .his attendants to do things he dreads, he cries and becomes excited and hysterical. Fear that the heart may stop or the breathing cease are also serious types of morbid fear. The patient is always feeling the pulse and asking for an examination of the heart and an assurance of its soundness. The number of fears is almost as great as the number of objective things. The more common types are mysophobia, agoraphobia, claus- trophobia, fear of riding in cars or carriages (in New York City of sub- ways) and astraphobia. There are also many ''occupation fears," such as that in which the barber fears to use his razor, the tailor to cut the cloth, the bartender to mix drinks, and the business man to sign his name to checks. These symptoms lun a course of one-half to three years. If properly treated, the patient recovers, with a tendency to relapse. Allied to the morbid fears are the morbid "philias" or anxious concern for the safety and health of the race, more particularly of the domestic animals. The patients cannot walk the streets without con- cern over the tail of the docked horse and will get up at night to fetch in a wailing cat which seems to him in real, not amorous, distress (zoophilia) . While morbid fears occur in very early life, often developing at puberty, doubting mania occurs rather later, usually after adolescence. The doubting habit is often a morbid outgrowth of an original morbid scrupulosity and attention to detail. The patient has usually been very orderly in habit and worried when things are not arranged or done in a very orderly way. The clothes and bureau drawers and the bric-a-brac of the room have all their set place, and a discomfort is felt at seeing them out of the regular spot. Then some disturbing emotion or sickness sets agoing an absolute distress of mind if things are not just so or are not perfectly clean. Then begin frequent washings, change of clothes, an hour is spent in arranging the articles on a bureau and mantle or in dressing the hair. The doubting mania sometimes seizes girls — -more rarely men — ■ who are about to be married, and engagements are repeatedly made and broken. Often the doubting habit is shown in connection with fear of fire, of burglars, or gas. The patient gets up at night a dozen times to feel the gas jets, to see if the doors are locked, to look under the bed, and do other whimsical things. These doubting states are often not very serious, and are only exaggerations of a naturally timid and scrupulous disposition. Alcoholism is at times associated with doubting mania. Obsession, Fixed Idea. — Obsessions and fixed ideas often relate to some rather trivial thing. In the obsessive constitution the association 510 DISEASES OF THE NERVOUS SYSTEM machinery seems to get clogged and ideas tend to stick pertinaciously in the mind. A slight suggestion or remark casually made with some especial rhythm or emphasis stays and bothers the patient. More often there are one or more ideas which stay and torment him. They are unpleasant ideas of possible mistakes made or injuries thought to have been done. They are usually absurd, and the patient knows per- fectly well that they are so, but they cannot be shaken off. They domi- nate and harass the sufferer who feels really like one " possessed." They lead to insomnia, nervous restlessness, hysterical outbreaks, and crises of the abdominal viscera, with diarrhoea and polyuria. Yet often the patient, if naturally intelligent and sensible, will keep himself in control, and show most of the time no emotion or evidence of suffering. These fixed ideas may attack a person periodically for years, or may after a few months disappear, not recurring at least to any extent. Shocks, acute sickness, and depressing experiences will bring them out. The syndrome of the fixed idea, not reaching the intensity of a delu- sion, may be part of a melancholia of involution or of manic-depressive insanity. Morbid Impulse. — Impulsive psycho-neurosis is the condition in which impulses spring suddenly into consciousness, and acts follow at once without the patient being able to resist them. The morbid im- pulses may be very slight and harmless, such as a desire to touch every lamppost passed on the street, to step on alernate stones of the pavement, to put the left foot first in going from a room, or they may lead to violent acts, such as murder, theft, or arson. When there is a morbid impulse to utter certain words over and over, it is called onomatomania. Psychic tics belong to the morbid impulses. A patient of mine, a clergyman, who was overworked and anxious, developed impulses to an automatic coprolalia and profanity while at work in his study, and even while preaching. Another clergyman had the same tendency along with a decided doubting mania. Morbid Compulsion. — When a morbid impulse rises into conscious- ness and is held more or less under restraint it is called rather artificially a compulsion. A patient while shaving started to cut himself. He never did do this, though he was obliged to give up doing this function himself for a time. He also felt an intense desire to jump overboard while on a ship, though he had had no melancholia at any time. The compulsion is often perfectly restrained, and I have known a man who went about for years with at times an almost overwhelming fear that there would come upon him an uncontrollable impulse to assault some one. Compulsive and Impulsive Manias. — When impulses and compulses involve a larger psychical sphere, we have the various conditions known as kleptomania and pyromania, homicidal and suicidal mania. But PSYCHASTHENIA 511 here, along with defective inhibition, there is often a morbid degree of criminal instinct or there may be present another psychosis, such as mania or melancholia or paranoia. Thus the sexual criminal acts and perversions are of the class of compulsions or impulsions, plus a con- genital and hereditary instinctive defect. A type of impulsion midway between the mild and neurasthenic and the deep-seated psychopathic cases are the impulses to drink leading to periodical inebriety. Here there occurs periodically, with or without the excitement of a single taste of liquor, an overwhelming impulse to drink, and the patient drinks until consciousness is lost. In fine, impulsive and compulsive insanity are generally part of the psycho-neurosis but they may occur as episodes in melancholia, mania, dementia prsecox, paranoia, and the criminal constitution. Hypochondriasis and Psychasthenia. — Hypochondriasis is a term applied to a morbid mental condition in which the patient thinks he is suffering from some physical disease. It is a much used and much abused term. Hypochondriacs vary much in the degree in which the symptoms, show themselves. In some cases the belief in the disease is so foolish and extravagant as to form a real delusion, as when a patient thinks he has a worm in the head or stomach or an absolute and permanent stoppage of the bowels. In these cases the hypochondria is only a manifestation of a melancholia, generally a chronic melancholia of involution. In more cases the patient's hypochondria is simply a manifestation of a morbid fear or an idee fixe or obsession. These are cases of psychas- thenia. A mild degree of hypochondria often characterizes chronic neurasthenia. Hypochondriasis, therefore, is only a syndrome which occurs in melancholia, psychasthenia and neurasthenia, and is not a separate disease. Hysteria and Psychasthenia. — Some at least of the mental traits and emotional crises of hysteria are forms of or episodes of psychasthenia. There is here a loss of control due to the sudden explosion of fear or anger from an inadequate cause. The interparoxysmal state of the hysterical is characterized often by morbid fears and obsessions and by psychic tics. Thus the psychasthenic, who despite his morbid fears is forced to do things he dreads, will go into an emotional crisis resembling an hysterical attack. Diagnosis. — The diagnosis of a psychasthenia, characterized by morbid doubts and fears, fixed ideas and morbid impulses, is usually very easy. Sometimes the condition is associated with a great deal of mental depression and anxiety, so that it may be something like a melancholia; but there is with it none of the profound melancholia, with suicidal feelings, and the condition is one more of anxiety and alarm than 512 DISEASES OF THE NERVOUS SYSTEM of true emotional depression. There is, furthermore, no difficulty or retardation of thought, nor any of the apathy of melancholia. Persons with fixed ideas or obsessions may seem at first to resemble, to a certain extent, paranoiacs, and such cases have been described as abortive types of paranoia. The fixed idea, however, is one which the patient realizes to be over-dominant and in a sense forced upon him; he does not accept it with sympathy and conviction as the paranoiac does; he is aware that it is, in a measure, delusional in character. In the case of morbid impulsive acts, the question of an underlying melancholia or other psychosis must be considered. The splitting up of the various clinical symptoms into special groups of anxiety psychosis, conversion hysteria, true (actual) neuroses, psycho-neuroses is a matter for psychologists. It does not seem to me necessary. Prognosis. — ^Psychasthenia associated with morbid fears and doubt- ing manias, occurring in early life, is a serious condition. If taken early in Jiand, however, and placed under rigid control, the patients have an excellent chance of recovering in one or two years. If, as is often the case, the patients are kept at home and not properly managed, the trouble becomes fixed, some mental deterioration develops, and a serious and incurable condition takes place. When the disease develops later in life, as an episode or as the result of some shock or exhausting effort, prompt treatment is usually effective; but here again, if the trouble be neglected and becomes fixed, the prognosis is more serious. Psychasthenic conditions associated with fixed ideas and compulsions are in my experience less serious. They often disappear under tonic treatment and rest in the course of a few months or a year, but there is the liability that they may return. An obsessive psychosis rarely develops in the degenerative period of life; if it does, it is serious and is apt to turn out to be a melancholia. This outlook is apparently much brighter than that taken by some writers. It is perhaps because neurologists see cases earlier and do not deal greatly with custodial patients. Treatment. — The treatment of psychasthenia in all its forms is essen- tially one of hygiene, tonic measures, occupation, restraint and peda- gogical training. Without such measures very little can be done; but with them all but the worst cases can be managed with fair satisfaction. This general statement applies to dipsomania and the drug habits, as well as to the obsessions and the morbid fears and doubts. It is as useless to treat these cases (except in the mildest forms) by medication and at home as it is to treat the drink habit or the major hysterical conditions. There are no specific drugs. In the mild types, characterized simply by fixed PSYCHASTHENIA 513 ideas and a certain amount of fear and doubts, hypnotic treatment is certainly of some use. This apphes especially in the younger cases. The method of psycho-analysis and mental catharsis, of hunting for the irritative subconscious complex, and explaining the mechanism of the obsession, sometimes cures, sometimes does harm. All the cases thus cured can be relieved by simpler measures, though in so far as psycho- analysis means pedagogics and re-education it is exactly in line with the older and long employed therapeutics. 33] CHAPTER XXIII NEURASTHENIA (NERVOUS EXHAUSTION, BEARD'S DISEASE) The condition which is called neurasthenia is often only an exaggera- tion or pathological emphasis of a neuropathic constitution. Persons of a highly nervous organization often have at various times, some symptoms of neurasthenia. Neurasthenia is a functional nervous disorder, characterized by nervous weakness and irritability, so that the patient is exhausted by slight exertions, reacts excessively to irritations and is largely or entirely incapacitated for his daily tasks. In popular usage, neurasthenia is a name often given to what is really some serious disorder such as melancholia. Nervous exhaustion is rarely a disorder by itself, but is more often a condition associated with some morbid mental state. Besides or with this general neurasthenic condition there are local neurasthenias, affecting especially vasomotor, gastric, enteric and sexual functions. There are evidences that the neuropathic constitution existed in all ages, but coherent descriptions of clinical types, like the neurasthenia of modern days, are not found in literature until the past century. The credit of calling attention to this condition most insistently, most acutely, and most successfully is due to Dr. George M. Beard, though he described conditions some of which are now placed in other groups. The evidence is rather conclusive that the human race now suffers relatively more from nervous irritability and exhaustion, in its various types, than it did in the past. We find also that neurasthenia is more frequent among the partly civilized and pioneer races such as that of the United States. One sees it in the white and yellow races, but not in the brown or black. The nervous temperament and the strenuous activity of North Americans are said to be due in part to the waves of cold air which pour down upon our Western and Northwestern States, causing quick changes of tem- perature and a cool, dry, stimulating climate. Neurasthenia prevails rather more in dry temperate climates, but it is by no means infrequent in the tropical regions, and is to be found in the West Indies and in the republics of South and Central America in its classical forms. White people living in the tropics, unless under the best sanitary conditions, become nervous, and Dr. Woodruff asserts that its cause is the excessive light acting upon non-pigmented skins. Neurasthenia is found rather more often in men than in women, but the difference is not great. 514 NEURASTHENIA 515 The neurasthenic age ranges from eighteen to fifty-five, but the larger proportion of cases is met with between the years of twenty and fifty. Occasionally symptoms resembling neurasthenia may be seen in children of the age of twelve or thirteen, and occasionally also there develops a kind of senile neurasthenia, which is, however, often associated with hypochondriasis, and some definite degenerative changes in the nervous or vascular system. In men neurasthenia occurs more often in the single; in women the relation is somewhat reversed, so that, taking both classes, the married and the unmarried are about equal. Neurasthenia does not much affect the people of the country and small towns, though it does exist there. In great cities the number of neurasthenic women, among the wives of laborers and artisans, is rather large, and this is the natural result of the strain of living with husbands who are dissipated, and of rearing large families of children in the close quarters of a tenement house. The disease is relatively more frequent in the educated classes. Hereditary influence plays a very considerable part in the develop- ment of neurasthenia. We can usually find that there is a history of migraine or some nervous irritability, of alcoholism or some psychosis upon one side or the other. A distinct history of the major neuroses or of severe mental diseases is rare, but there is no doubt that a very large proportion of neurasthenics come into the world with an over-sensitive and weakened nervous system. They may be strong enough to undergo the ordinary strain of life, but break down under some specially exciting cause. The exciting causes of neurasthenia are very various, but they can most of them be classed under the head of excessive mental strain, shock, sexual abuse, and the influences of exhausting fevers, of chronic infections like syphilis, and of excesses in alcohol. The practice of masturbation is one of the things for which neu- rasthenics very often keenly reproach themselves and over which much hypochondriacal brooding develops. Excesses of this kind, however, are usually a sign of a degenerative or unbalanced nervous system rather than a cause. The actual harm done is greatly exaggerated, however strongly this practice is to be reprobated. Excessive and unnatural indulgences, such as sodomy, coitus interuptus, etc., tend to weaken the nervous system and are causal factors of neurasthenia. Bad methods of education combined with excessive study may lead to nervous exhaus- tion. This is usually seen in ambitious students who are forcing their way under great disadvantages through professional schools and into professional practice. Young women, who are excessively devoted to social indulgences, sometimes break down with nervous exhaustion. 516 DISEASES OF THE NERVOUS SYSTEM The studies and training of the primary and secondarj^ schools may prepare the way for these catastrophes, but thej'^ rarely come before the eighteenth year, and then it is not the hard work of schools but the bad habits of life at the period of education which break down the student. Work and study alone do not cause neurasthenia, but are healthful except to certain persons who are of bad stock and are destined to be neurasthenic at the time of adolescence. Typical attacks of neurasthenia are undoubtedly brought on by the fright and shock incident to severe injuries or exposure to great danger, as in railroad collisions and other frightful forms of accident. A large proportion of the so-called "traumatic neuroses" are partly forms of neurasthenia ; and morbid mental states can in time induce a neurasthe- nia just as the reverse is true. Neurasthenia can be brought on also by excessive child-bearing, the drain of lactation and domestic trouble, great excesses in eating and drinking, and the strain of hard domestic life and of sickness and nursing. Neurasthenia sometimes follows an acute infection like that of typhoid fever or the grippe. It may also be induced by the infection of syphilis (see Luetic Neurasthenia), although this must not be confused with the neurasthenic symptoms which precede paresis and have an organic basis. Much weight has been laid upon the importance of eye-strain in producing neurasthenia, and, given a neuropathic constitution, there is no doubt that the defect in the refraction of the eye or in muscular equilibrium may cause, or at least keep up, a neurasthenic state. The Same is probably true of severe forms of gastric disturbance, and of disease of the pelvic organs, such as subinvolution, decided displace- ments, and chronic ovaritis or salpingitis. In men the existence of prostatic irritation, of irritable strictures, and hemorrhoids and fissures may start up neurasthenic symptoms. The existence in neurasthenics of a tendency to constipation, or what is popularly known as "biliousness," accompanied by a gouty or lithsemic diathesis, has been much dwelt upon, and at one time neurasthenia was thought to be largely the expression of a disturbed state of the metabolism — a phase only of gout or lithsemia. This tendency, however, is rather the result of the weak nerve-centres than the cause, though the two often act in a vicious circle. Prolonged and severe dyspeptic disturbances, especially when associated with atony of the stomach and bowels and the condition known as gastroptosis and enteroptosis, are exciting or maintaining causes. Hyperthyroidism and defects in other internal secretions may cause neurasthenic symptoms. I would sum up the leading causes of neurasthenia thus: 1. Hereditary nerve instability. 2. Overwork and worry. NEURASTHENIA 517 3. Severe shocks, with or without injury. 4. Infections. 5. Abuse of stimulants and narcotics. 6. Abuse of sexual functions. 7. Disorder of digestive functions, autoxsemia and glandular disturb- ances. On the whole, the causes are most often a bad heredity and foolish living. Symptoms. — The symptoms of neurasthenia are relatively definite, but a great deal of what is mental has been written into the disorder. In other words, the minor psychoses such as hysteria, psychasthenia, hypochondria, abortive melancholia have been often looked upon as neurasthenia. The time has come now, however, when these conditions should be recognized and treated separately, and thus greatly limit the field of the disease. For neurasthenia is, strictly speaking, the name to be applied only to a neurosis — a morbid nervous, not a morbid mental, state. It will be easier to understand what neurasthenia is by indicating first what it is not; in other words, by excluding certain quite well- known groups of psychic disorders. First, then, we exclude from the symptomatology of neurasthenia the mild types of recurrent melancholia or, as they are now termed, "the depressive phase of manic-depressive insanity." Second, we ex- clude the psychosis characterized by a dominant sensory complex. It includes patients who are continually complaining of dysthesias, headaches and head parsesthesias, stomach troubles and various bizarre forms of physical suffering and functional disturbance. The dis- ease is seen in young people and it is only an abortive or early type of the melancholia of involution. It is sometimes called anxiety psychosis. Third, we exclude the psychosis (psychasthenia) character- ized by obsessions and fixed ideas, of apprehensions, panics of doubts, abulic states, and psychic tics. Thus we remove from the realm of neurasthenia all the pure psychoses. These psychoses in mild and abortive types give many of the symptoms of neurasthenia, because they usually have a neurasthenic basis. Under the head of neurasthenia proper there are two types of a neurosis, one neurasthenia prgecox or neurasthenia of adolescence, and second, the neurasthenia acquisita or the neurasthenia produced generally later in life by Avork, worry, shock, infections, toxins, sexual excesses, and other causes already named. Besides these two types, or associated with them, we find local neurasthenias, affecting especially the heart, vasomotor centres, digestive or sexual organs. The general symptoms of primary neurasthenia are very much like those of the acquired type, and the following description fairly out- 518 DISEASES OF THE NEEVOUS SYSTEM lines the clinical picture of each. In the prsecox type, the asthenia is of a more simple character; there is less evident trouble with special organs, less mental distress and fewer psychic symptoms. The young person gives out all along the line, but the malady is apt to be more persistent, and after apparent cure remissions may occur. It is not a frequent disorder, and it may come on long after the ordinary period, which is about the eighteenth to the twenty-fifth years. Whenever this type of neurasthenia is followed by a serious psychosis, such as melan- cholia, we may infer that the original attack was really a mild psychosis. One of the first symptoms is an annoying insomnia. It is usually an early insomnia, i.e., the patient cannot get to sleep till late, and then sleeps badly, frequently awakening. Distressing dreams occur, and the patient wakes unrested and more tired than when he went to bed. The patient complains of a general feeling of mental depression. He can work, but soon gets tired. He can play but it exhausts him so that he cannot sleep or has a headache. All mental and physical effort has to be paid for by periods of rest. He wants to work and to play but he cannot. He is easily irritated at things which before caused him no annoyance, and becomes a source of domestic unrest and unhappiness. He suffers from a number of peculiar sensations which are called "cephalic parsesthe- siae." These are sensations of pressure on the top of the head, or a feeling of constriction around the temples, or a burning spot on the vertex, or tenderness of the scalp. Sometimes he has a sense of weak- ness or even pain in the back of the neck. All these things are increased by the use of the mind or by bodily exertion, and they are all more common in the acquired types. Headache occurs in perhaps one-half of the cases, the headache being usually either frontal or occipital; or both. It is often very persistent, and in fact a chronic headache, not due to tumor or meningitis or syphilis, is almost invariably of neurasthenic origin. This neurasthenic headache is usually diurnal only, coming on in the morning when the patient wakes up and lasting a good part of the day. It does not often keep him awake at night. In this point it is distinguished from the headaches of syphilis and of meningitis or of tumors. Women suffer from these headaches, and from pains in general, more often than men. They in particular have much pain in the back of the neck and along the spine. This keeps them from walking or being upon their feet, and it may develop into a form of trouble known as ''spinal irritation." The special senses are functionally affected. The patients can often see quite well, but their eyes soon tire; they cannot read a book long because it makes the eyes smart or produces some headache. Examina- tions of the neurasthenic's eye frequently show the existence of some refractive error, most frequently astigmatism and hypermetropia; de- NEURASTHENIA 519 fects in the ocular muscles, and especially weakness of the internal recti, often occur. Patients have frequently complained to me of a defect in visual memory. They see a thing or face but do not remember it again as readily as they used to. There is no limitation of the visual field in true neurasthenia uncomplicated by organic disease, but there is a morbid susceptibility to fatigue, particularly of the periphery of the vision, so that after long testing, objects in the periphery becomes less distinct, and a sort of artificial limitation of the field may be produced. In some cases an object which is brought from without into and across the visual field is seen in wider range than an object which is placed in the centre of vision and carried gradually out toward the periphery. This is the re- verse of the normal condition, and is known as "Foerster's shifting type." A slight drooping of the lids, and excessive mobility of the iris have been noted in neurasthenia. Neurasthenics of the acquired type sometimes suffer from tinnitus, which is very distressing and aggravates every other nervous symptom, but this usually occurs only in connection with actual disease of the middle ear, or in old people with degenerative changes in the cerebral blood-vessels. An excessive sensibility to noises, and even the pleasant sounds, like those of music, may be present. Neurasthenics sometimes cannot bear even the most enchanting melodies. A similar morbid sensibility to taste and smell may be present. But these are matters of minor moment and are due to temperamental or psychic complications. In neurasthenia the general muscular strength is lessened and easily exhausted although the patient may not have any actual atrophy. The cutaneous and deep reflexes are generally exaggerated. These conditions vary considerably, however, in different cases, and are more marked in the younger patients and those of a neuropathic constitution. The sexual function is irritable and weak and in certain cases this local trouble dominates the situation. But here a psychosis is often complicat- ing the neurasthenia. There is a considerable disturbance of the heart function in neuras- thenia. The most frequent condition is an acceleration of the pulse beat from very slight cause. A pressure over some painful point in the body will sometimes bring up the pulse from 80 to 90 to over 100, and it will remain there for one or two minutes. This is called ''Rumpf's symptom." Arhythmia and palpitation of the heart are less frequently observed. Cardiac weakness is an important condition in many forms of neurasthenia and underlies many of the asthenic symptoms. This is particularly true of the neurasthenias of more advanced life. The blood- pressure is usually low. There is an abnormal difference between the pulse-rate and pressure in the vertical and horizontal positions (Crampton test). 520 DISEASES OF THE NERVOUS SYSTEM The existence of some degree of thyroidism should be looked out for in patients with very irritable hearts. Ordinary valvular lesions have little to do with causing neurasthenia, but when a patient with a bad myocardium becomes neurasthenic his heart gives him much trouble. He feels its irregular beating and skipping and he hears its throb when he lies on his pillows; its action is disturbed by very slight exertion and slight anginal sensations are present. A great deal of emphasis has been laid upon the vasomotor disturb- ances of neurasthenia. The most common symptoms are those due to weak and irritable vasomotor centres, viz., cold hands and feet, flushings, pallor, poor reaction to the cold of the air or the bath. Vago-tonic and sympathetico-tonic seizures are more common in psychasthenia. Many of the symptoms which we now call "neurasthenic" were de- scribed by Dr. Prout and Dr. Golding Bird early in the last century and were held by these gentlemen to be due to oxaluria. This was a condition characterized by flatulent dyspepsia, melancholia, and nervous irritability and was thought to be due to defective metabolism, resulting in the pro- duction of an excess of oxalic acid. As a matter of fact the condition of the urine is of not much importance in the symptomatology of neuras- thenia, except in so far as it indicates an autotoxsemia, or a deficient ex- cretion of nitrogenous bodies, or evidences of slowed or quickened metabolism. The urine of neurasthenia is variable in specific gravity, but, on the whole rather low ; and the daily amount is below the average. The func- tional acitivity of the kidney may be lowered and the blood in patients with complicating cardio-renal disorder should be tested for abnormal nitrogenous constituents and for sugar, urinary examinations not being always sufficient. The digestion of neurasthenics is usually affected, and a large propor- tion of them are probably treated mainly for their stomach conditions. The neurasthenic has a feeble digestion, the gastric juice being deficient in acid and the gastro-intestinal motility slow. In a certain number a gastroptosis occurs, and the lower curvature drops below the umbilicus. The patient suffers from flatulence, acidity and epigastric discomfort, with anorexia and nervousness. There is atony of the gastric wall, but no very marked change in secretion, and no especial fermentation (Lockwood). It is only in patients who have abused themselves with alcohol or tobacco, or excessive indulgence in sweets, or with ravenous feeding that worse conditions are found. In people of more advanced age, feebleness of digestion is often associated with a more serious relaxa- tion of the stomach and intestinal walls, and a great deal of atony of the whole intestinal tract, with patency of the iliocaecal valve. In these cases, which we find particularly often in women, the prolpase of the in- NEURASTHENIA 521 testines, stomach, and perhaps of a kidney may cause a great many dis- tressing symptoms. The condition has been described by Glenard under the name of "enteroptosis," and it undoubtedly is an important factor in keeping up the neurasthenia of some women in adult and middle life. Its investigation has been much helped of late by bismuth feeding and X-ray photographs, and in all persistent neurasthenias with intestinal symptoms there should be a thorough study of the secretory, muscular and anatomical conditions of the gastro-intestinal tract. Among the most serious, though fortunately rare, symptoms of neurasthenia involving the digestive tract is the condition known as "mucous" enteritis. This trouble generally attacks women rather than men, and usually women between the ages of twenty-five and forty. It comes on after the patient has become exhausted by pro- longed domestic cares or fashionable dissipation, or some shock. It is one of the earlier symptoms of the nervous weakness, and begins with abdominal pain, followed by attacks of diarrhcBa, in which tubular casts are passed, or portions of such. This diarrhoea is painful, colicky, and alternates with periods of constipation. There is a somewhat spastic condition of the bowel, as though it were irritated and closed down upon the contents of the intestine. The term mucous enteritis is not strictly a proper one, since microscopical examinations and autopsical reports show that the substances thrown off are not mucous mainly and that there is no actual inflammation. The casts that are found in the stools are composed principally of albuminous substances, the product apparently of the decomposition or disintegration of the epithelial cells of the intestinal walls. While mucous enteritis sometimes occurs in persons who are profoundly asthenic without any decided neurasthenia, yet, in the great majority of cases, it is a symptom of neurasthenia, and can be successfully treated only on such a basis. The respirations in neurasthenia are generally normal, but shallow and deficient respiratory expansion sometimes exists. In women par- ticularly I have often found that there was an actual inability properly to expand the chest and inflate the lungs. The temperature is normal, and a very variable temperature of the skin is simply dependent upon vasomotor instability. The composition of the blood is often quite normal. Hosslin finds that even in those patients who appear to be anaemic there is a normal amount of haemoglobin; however, anaemia certainly exists in many cases, and there is no question that the use of iron is often of great benefit. Variations in the weight of the neurotic often occur. Neurotic pa- tients may gain or lose ten or twenty pounds within a few months. The secretions of the skin are usually increased, and the patient sweats easily and profusely. In other cases of a less irritative type the skin is inclined 522 DISEASES OF THE NERVOUS SYSTEM to be dry. Its nutritional condition is poor, the hair falls, and, according to Beard, there is a tendency to early decay of the teeth. Traumatic Neurasthenia, Traumatic Psycho-neurosis. — This condi- tion is nothing else than neurasthenia with a more than ordinary mental element of anxious receptiveness to physical impressions. The following description is in a degree a repetition, but it is given here as it is very nearly the actual record of a typical case. The patient is in an accident and has suffered from some physical injury, and great mental shock. He is perhaps helped to his home, and his sprains or bruises attended to. He goes to bed and sleeps; he wakes up the next morning feeling not quite so well as usual, but congratulating himself, perhaps, on having gotten off so easily. He resumes his work and finds that he can do it, though with not quite so much ease as usual and he very likely suffers from some pain due to a strain or bruise that he has received. In a few days — almost always within a week — he begins to notice that he is more nervous than usual, that little things irritate him which did not do so before, that his head seems somewhat confused, and that the effort to work is wearying. His sleep is disturbed, and he wakes up in the morning unrefreshed by his night's repose. He becomes somewhat despondent over his condition, and thoughts of paralysis or some other serious ailment annoy him. His head aches, the pain being more or less constant and diffused, and located usually over the forehead or at the back of the neck. He has unpleasant sensations in the head, such as that of constriction or pressure or scalding feelings. His back also is continually painful, and walking increases it. His nervousness be- comes more marked, and close examination shows a little, fine tremor in the hands. He has also sometimes creeping sensations over the body or numb feelings in the extremities. He tires very easily. He is emo- tional, and becomes more despondent as the days go on. Sometimes he has spots before his eyes, noises in his head, or ringing in the ears. Reading is laborious and increases his headache; so also does attention to work. His appetite becomes capricious and his bowels are consti- pated. He suffers somewhat from flatulency and dyspepsia. His heart palpitates easily, and the pulse is a little accelerated. Sometimes for a few days there is a little weakness about the bladder or irritability of that viscus. His sexual power is diminished. His blood pressure is lower than normal. Very slight excitement produces sweating of the hands or coldness of the extremities. He loses a little flesh. The picture, as may be seen, is very much like that of neurasthenia of the acquired type plus a little more of the physical and sensory complex. These symptoms may be several weeks in developing, and during this time he may perhaps consult a lawyer about his case. If so, the anxieties of litigation begin to add to and intensify his troubles. He consults a NEURASTHENIA 523 physician, and the physician finds the subjective symptoms that I have mentioned. Objectively, when examined, the physician will discover that the muscular power is somewhat weakened, that there is a certain amount of the fine tremor perhaps in his hands. The knee-jerks and elbow-jerks are exaggerated; there are tender points along the spine and upon the head. In making him stand with his eyes closed there is a cer- tain amount of static ataxia discovered. The pupils are often dilated and mobile, and examination of the visual field shows sometimes a slight contraction, at other times the "shifting type" already described. In many cases a degree of peripheral retinal anaesthesia will be discovered. The pulse will be found accelerated, and pressure on a tender point may send it up very rapidly; a slight exertion will also accelerate it. There will be something apparent in the physiognomy of the case which shows the man to be in a nervous and asthenic condition. Sometimes the pains from which the patient suffers in the back and the weariness in the limbs are so great that he remains a good deal of the time in bed. In all cases he will assert most positively that he is unable to work or to take that interest in his affairs that he has previously done. In a good many cases there will be added to the foregoing picture a number of symptoms due to some local injury; for example, the arm may have been wrenched or bruised, and the result may be a certain amount of neuritis and weakness or pain in that member; in other cases the back may have been so severely sprained that the typical symptoms of spinal irritation ensue, and this is particularly apt to be the case when women are injured; in other cases, again, the legs may have been hurt to such an extent that a sciatica or some other form of neuralgia develops. The foregoing symptoms, varying in amount and degree, will last, with little change, for a very long period of time. If the case goes into litigation, there is added the worriment occasioned by having to go through the disturbing experiences of trial by jury so that what was at first mainly a neurosis becomes a psycho-neurosis, or almost a psychosis. In many cases, after the trial has been settled and damages awarded or otherwise, the patient begins to mend, and in a certain proportion of cases he gets completely well. This is not invariably the rule. Spinal Irritation (Spinal Psycho -neurosis). — Spinal irritation is a form of neurasthenia in which, associated with the general neurasthenic symptoms, are certain special, painful symptoms, related chiefly to the sensory nerves of the spine. These cases have in the past been described under the head of "spinal anaemia" and "hypersemia." They may de- velop in traumatic neurasthenia. The patients are usually young women, between the ages of sixteen and twenty-five. The trouble is sometimes brought on by injuries or by a physical over-strain. Sometimes it seems to be associated with a natural weakness of the spinal muscles and a 524 DISEASES OF THE NERVOUS SYSTEM consequent curvature. Sometimes it follows acute infectious diseases. The patient begins by complaining of pain in the back — usually in the lower part — and also in the back of the neck. These pains occur on standing or walking, or any exertion, and are so severe that the patients, in the course of a few weeks or months, give up attempting to walk about. They get relief and comfort in bed, and so they go there and remain. The pains are of a heavy, aching character, increased until they become very sharp when attempts at movement of the trunk are made. There is a great deal of tenderness to pressure along the spinal processes, some of these processes being much more sensitive than others. The most sensitive points are usually in the back of the neck and the upper dorsal vertebrae, and down in the lumbar region. There is some pain also upon pressure alongside of the spinal processes. Painful points often vary, and even in a single examination the patient may complain, and complain honestly, of different sensitive vertebrae. Pressure on these points does not often bring out visceral symptoms, as the brothers Grifhn taught, but may cause faintness, nausea and tears from the pain. The patients suffer much from headaches. The arms are often weak, so that attempting to sew or write or hold a book causes pain in the neck and shoulders. The legs are also weak and the circulation is poor. There is sometimes palpitation of the heart and precordial distress. A certain amount of dyspepsia is always present, and constipation is the rule. The patients often have attacks of vomiting, and attempts to feed them require much care. The menstrual functions become irregular. The patient grows weaker and often becomes bedridden, especially if little attempt is made to overcome the symptoms by voluntary effort and attention to nutri- tion. These patients generally get well in from one to three years, but occasionally they sink into permanent invalidism. The symptoms are quite as much due to mental sensitiveness and disordered cerebrum as to any local spinal trouble, and the term psj'^cho-neurosis is a more correct one. Local Neurasthenia. — There are cases in which the specially weak and exhausted function is the heart and vasomotor centres, or the stomach and intestinal tract, or the sexual organs. The symptoms here are much the same locally as those described above under the head of neurasthenia. But the patient with a nervously weak stomach or heart or sexual organs may yet be strong enough to do a fair amount of work. It is only one or two of the somatic functions that are involved. Here the term local neurasthenia is a justifiable one. In many of these cases, however, a mental element develops and often local neurasthenias are also associated with a psychosis. Angiopathic Neurasthenia. — Here the patient has some general symptoms of neurasthenia, but in particular he has special symptoms NEUEASTHENIA 525 which consist of a sense of pulsation or beating, which involves the whole body. The tension of the pulse is low, the rate normal or slightly accelerated. He does not have palpitations of the heart, as in Basedow's disease, and there is no particular dyspnoea on exertion. The skin usually shows a striking degree of dermography, and there is an epigastric pulsation, as well as pulsation of the carotid. The pathology is suggested by the fact that one of my patients was rather promptly cured by adrenalin. And the trouble may be due to an inadequate activity of the suprarenal capsules. Neurasthenia Gravis. — In instances which are fortunately very rare neurasthenia assumes a very severe and serious type of exhaustion. The patients suffer from the typical symptoms in much the ordinary way, but the degree of weakness is very much exaggerated. Such patients have not only headaches and disturbed sleep, pains in the back and parsesthesise, digestive disturbances, and mental depression, but they speedily emaciate to a considerable extent. They take food in fair amounts, but it gives them no strength. The most careful applications of the "rest cure" secure for them only temporary benefit. They cannot walk far without intense fatigue and exhaustion, with subsequently severe headaches, or even attacks of vomiting and diarrhoea. Despite closest examination, no distinct signs of organic disease can be discovered, and I have known such patients to go on into an invalidism which has lasted for over eight years with final recovery. In these cases there is not an hysterical or even large hypochondriacal element. No amount of sug- gestion or "mind cure" has much effect upon them. They are not, in other words, hysterical, bedridden women, but often men who have reached or passed the middle period of life. Even these very worst cases may get well. Pathogeny and Pathology. — Victims of neurasthenia are persons who in all cases have either inherited or acquired a nervous system with lessened power for active functioning. There probably underlies in neurasthenia an impaired metabolic power. The nerve-cells break down too easily and build up too slowly. This defect is partly inherited, but is brought out in the struggles of maturer life. Glandular defects (thyroid, adrenals) may pronote the trouble and so may disorders of the autonomic system and the vasomotor centres. There may be cases of pseudo- neurasthenia in which the exhaustions and irritations are psychogenous. Neurasthenia then becomes a symptom of hysteria. Diagnosis. — The characteristic of neurasthenia is the easy exhausti- bility after mental or bodily effort, and the punishment that follows exertion. The mind is usually alert, there is no profound depression, and thought is not difficult or sluggish at first. There is some constitu- tional and usually some physical cause for the condition. In all these 526 DISEASES OF THE NERVOUS SYSTEM ways it differs from the mild abortive melancholias with which it is oftenest confused. The feelings of anxiety and depression, the self- concern are not in great excess of normal. The so-called "anxiety nem'osis" is not a form of actual neurasthenia, but an abortive form of hypochondriacal melancholia. There can be no question of this fact, and the attempt to erect it into a special disease is caused by insufficient experience. I have seen many cases who had attacks of this so-called malady when young develop true melancholia of involution later. The neurasthenic has no undue alarm, he does not tell his story with the voluble iterations of the anxious neurotic; if he does, there is a psychosis imposed on the neural exhaustion. The beginnings of dementia prsecox, mild types of confusional and exhaustion psychoses, early paresis, hysteria, psychasthenia, and malinger- ing are the other conditions which must be noted in making a diagnosis. The diagnostic criteria are given under the description of these disorders. The diagnosis of the cause involves a thoroughgoing study of the bodily condition, an examination for reflex irritations, for toxaemias, for bad environmental conditions and bad mental training and habits. A patient may be suffering from a number of bodily ailments, and if this person be at the same time of a somewhat nervous constitution the condition may resemble neurasthenia. Those persons having a very feeble digestion, with dilated stomach and an atonic condition of the ali- mentary tract, may get depressed, fretful, and sleepless; so a person suffering from some chronic uterine or ovarian or bladder trouble may present many symptoms of nervous irritation. These patients may have only a local neurasthenia or a real local disease with neurasthenia as a reflex condition. It must depend largely upon the good sense of the physician to measure the importance of the local troubles as compared with those of the general symptoms. I believe that the fully devel- oped type of neurasthenia is rarely brought out by local disease alone. Still, I have seen cases with neurasthenic symptoms cured for a time by washing out the stomach, and enormous relief to the nervous irritation to result from treating the condition of the blood or relieving the uterine disturbances. It should finally be remembered that some neurasthenia usually complicates the minor psychoses. Course and Prognosis. — There is such a thing as acute neurasthenia. This follows prolonged debauches and long periods of excessive mental strain, with loss of sleep. Such patients may present all the signs of neu- rasthenia, and get perfectly well in two or three weeks. Neurasthenia, however, is essentially a chronic disease, and when speaking of it we refer to this type of the disorder. It is a disease which comes on as a rule gradually, developing, however, in the course of a few months. It may, NEURASTHENIA 527 however, come on suddenly after shocks and accidents, and it may de- velop or follow rapidly after an acute infectious fever. It always reaches its height in a comparatively short time, and runs a course lasting from one or two to seven or eight years. This course is a varying one, and this variation is particularly noticeable when the patient begins to get well. The patient continues to improve for a time and then suddenly falls back, then goes forward again, and thus convalescence progresses. Complete restoration to health is possible and frequent, but the patient always has to take more care of himself than before. As a result of an attack of neurasthenia, men and women who have suffered from it are apt there- after to lead very saint-like and ascetic lives, and hence they as a rule live long. It used to be said by Dr. Beard that neurasthenics would have a long and happy old age. They pass through the valley of the shadow of death, but the experience may be a profitable, if not a pleasant one. Neurasthenia prsecox has a much more serious prognosis, especially as regards duration. This is the kind that may last seven or eight years, but recovery usually occurs. Acquired neurasthenia often lasts only from six months to two years. Local neurasthenias are also curable, except the sexual type, which is an obstinate affection if it is a purely neurasthenic one. Treatment. — Naturally, a measure of leading importance in the treatment of neurasthenia is rest, and the problem of how this can be obtained is the first one to confront the physician. In the severe types of neurasthenia, especially when it occurs in young women, the application of the "rest cure," which has been so ingeniously elaborated and perfected by Dr. Weir Mitchell, is undoubtedly the best , treatment. I do not find, however, that men submit themselves readily to this measure, and it seems to me to answer best for those neurasthenic women who suffer also from some hysteria and who are reasonably "sug- gestible" patients. A modified rest cure can often be secured by making the patient stay in bed until after midday lunch or lie down for an hour after each meal, and go to bed early in the evening. Business men will often cut their business hours down one-half if they are allowed still to continue some work. Experience shows that in most cases a rest is not needed for a very long period; i.e., not for over a few weeks. Some isola- tion or change may be imperative, but prolonged and absolute rest often is not. The patient is put to bed and kept there for several days, but is soon allowed to sit up for a part of the day, and in one or two weeks may begin to use exercises and enter upon a work cure. And it is often part of the program to lead the patient from abnormal passivity to more than ordinary activity. To accomplish this, I often substitute exercise for massage, sending a trainer to the patient, and later sending the patient 528 DISEASES OF THE NERVOUS SYSTEM to a gymnasium. Many patients do best by putting on a sweater on rising in the morning, exercising vigorously, then taking a cool bath, and then the breakfast. Later the exercise habit is inculcated, and this not strenuously, but in accordance with the constitution and tastes of the patient. Exercise is done to strengthen the muscles, help the circulation, in- crease the activity of the skin, and keep the mobility of the joints. But it also should be of a kind to arouse the attention, awaken interest and give a little aesthetic pleasure. This is often a difficult task, especially with women. In its place for them we must use hot boxes the masseuse, the trainer — or domestic work. Horseback riding is an efficient form of exercise. Many persons are greatly wedded to the exercise of walking, and it seems best to fit their needs. It is, however, a kind of exercise which does not take the patient's mind off himself and does not develop the respiratory functions so well as other measures do. Golfing fills in this lack, and this sport is doubtless of service in neurasthenia. The nurse is a potent feature in the situation. Some patients are almost purely exhaustion cases, and only want rest and feeding and sleep. They are better without a special nurse. In other cases and oftener a suitable nurse who is intelligent, tactful and interest- ing is a great help. Change of scene is usually very beneficial to neurasthenics, but traveling is injurious to them. They should be sent to some special place and be made to stay there. A tour along the Mediterranean coast or a trip to Europe often brings them back worse than when they went. Much the same can be said of trips to various places in the South or West. Some of the sanitaria in Germany, some of the places in the Riviera, Egypt and Bermuda, Nantucket, parts of North and South Carolina and Arizona, furnish good resorts for neurasthenics. They generally do better in the mountains, if the altitude is not too high, than they do by the sea- shore. Dry, windy, sunny climates like those of the Colorado plateau and parts of California and the Northwestern States are too stimulating for most cases. Much good may be obtained at the numerous sanitaria which exist in this country. Many of these are well conducted, and well supplied with all the modern appliances for treatment. It is, however, always a serious thing to send a neurasthenic to a sanitarium, for the reason that if he stays there too long he becomes contaminated with the atmosphere of invalidism about these places and develops hypochondriacal ideas as to his diet, his hver, his stomach, his sleeplessness, and his various sensory disturbances. In sending a patient to a sanitarium it is a wise plan to tell him not to stay, under any consideration, longer than six weeks; usually four is better. In the summer time great benefit can be secured NEURASTHENIA 529 by camping out in the woods and living a purely outdoor life, away from the conventionalities and restraints of civilization. The diet of young neurasthenics should be a nitrogenous one and my directions are that the patient can eat meats, fish, eggs, green vegetables and fruits. Milk can almost always be taken, at least for a short time. The older patients who do best upon milk, vegetables and fruit, with practically no meat. In general, tea and coffee, alcohol and tobacco, are to be entirely prohibited, but this is not an absolute rule. In some cases coffee is beneficial, in some tea does no harm, and in others a small amount of whiskey or dry wine and a cigar are also harmless. The physician has to determine this by the reactions and habits of the patient. Neurasthenics usually drink too little water and it is wise to prescribe a certain amount for them. Four or five glasses of water, which may be either plain or alkalinized, are to be taken daily, unless the patient has a dilated stomach when he should have a dry diet. In dyspeptic patients the meals should be small in amount and taken at fre- quent intervals; three light regular meals a dsLV and a little food in between form a regimen which usually answers well. Hydrotherapy, and massage are all measures which prove of service to the neurasthenic. Of these, hydrotherapy is the most useful, though its value can be overestimated. The ordinary prescriptions consist in the cold sponge bath every morning, and, if it is practicable, the use of a Charcot or a Scottish douche every other day. For women wet packs with massage are sometimes helpful, particularly in cases in which there are a great deal of nervousness and motor irritation. At night a lukewarm bath, at a temperature of 95°, for ten minutes, sometimes relieves the sleeplessness. Massage seems to me of not very much use in men, but it is often grateful and helpful to women, and when a great deal of rest is to be enforced it is essential to employ it for both sexes. Psycho-therapy is always to be more or less consciously applied. The subject has al- ready been discussed and if I do not say more here it is because the methods of DuBois, suggestion, hypnotism, etc., apply much more strictly to the minor psychoses. Neurasthenia proper needs physical attention to the body first, for when it is relieved the psychosis may disappear. It seemed to me that too much stress is often laid on the importance of minor troubles of the pelvic organs in women. Curetting and sewing up small tears rarely do any good. Serious uterine enlargement and prolapse, displacement and real disease of the ovaries require attention. If, in fact, the disorder of the pelvic organ gives recognized discomfort it should be corrected. Young men who are distressed by nocturnal emissions and who have 34 530 DISEASES OF THE NEEVOUS SYSTEM irritable urethras or distended vesicles may require some local treatment. But in these cases there commonly is also a psychosis and there is re- quired more moral than local therapeutics. The eyes have received and need to receive close attention for they are often even more neurasthenic than the stomach. Attention to errors of refraction and accommodation, and to poor muscular balance is im- perative. Poor eyes may keep up the neurasthenia and make the patient suffer from the discomforts of being unable to read or to do close work, as well as give rise to headaches and all kinds of cephalic parsesthesia. But most often the neurasthenia remains after the best oculists have done all that is possible. Many neurasthenics continue so though they have good eyes and can spend their resting hours in continuous reading. This is my experience and my answer to the insistent claims of certain ophthalmologists that profound long continued neurasthenia may be due to poorly corrected vision. Such cases are not seen by neurologists now. The bromide of sodium or potassium may be given to allay irritability and insomnia; it should be kept up for a limited time and then gradually reduced. At the same time or later the patient may be given a tonic mixture containing such drugs as the symptoms suggest. Quinine must be given carefully, as it causes increase of nervousness in many. Phosphoric and muriatic acids are the two mineral acids most often of use. These acids are usually better given after meals. The sac- charated carbonate of iron or Blaud's pills, if given, should be given gen- erously, i.e., in doses of thirty grains daily. The foregoing covers in a general way the measures to be used in treating neurasthenics. It is really the treatment of the neuropathic, and the constitutionally weak, also. THE SEXUAL NEUROSES AND PSYCHOSES Of the above disorders the neurologist has to deal chiefly with the vicious habits of masturbation (which may, however, be also a mani- festation of disease) and the sexual neuroses, spermatorrhoea and impotence. Masturbation and Spermatorrhoea. — Masturbation is the name given to the vicious habit of artifically exciting the sexual organs. It is very common among boys and less common but present among girls and adult men and women. It is usually only a vice due originally to low associa- tions and teachings among children. In some cases it is a disease or the symptom of a neurotic or insane constitution. Etiology. — It is most common between the ages of fourteen and eigh- teen, but may begin earlier. Even infants and very young children some- NEURASTHENIA 531 times masturbate, usually as the result of some local irritation which leads them to rub the genitals. A tight prepuce, eczema, or worms may- lead to the habit, but is it usually taught by a companion. The practice sometimes attacks schools almost hke an epidemic, for in every institu- tion a certain per cent, of the boys are sexually precocious or vicious, while the others are ignorant and innocent of the evils of the practice. Masturbation is relatively rare after twenty, but is practised by some throughout life even up to old age. Symptoms.— Masturbation, as ordinarily practised, leads after a time to a feeling of malaise, mental depression, disinclination to work, study, or to enjoy one's self as before. The appetite is a little impaired, the extremities easily get cold and perspire readily. Pecuhar numb feelings are felt in the hands and feet. There are an unnatural nervous- ness and irritability, and the power of concentrating the mind is a little weakened. The patients often have dilated pupils and hypersesthetic skin. After a time nocturnal emissions occur. The organs become ir- ritable and slight excitement causes erections. These symptoms may be slightly marked and pass away in a day or two, or until another indul- gence occurs. Masturbation is sometimes done to an extraordinary extent, even daily or twice daily for a considerable time. After a while the young man begins to find that he is not well and realizes that his habit is hurting him. Then if he be sensible and of healthy constitution he stops. Others are frightened out of it by friends or by reading the terrorizing stories printed in quack advertisements and circulars. Sometimes the fright thus caused leads the unhappy youth into a condition of hypochondriasis, which is helped on by the occurrence of nocturnal pollutions and the ner- vous debility resulting from his past indiscretions. In other cases in which there is a decided neurotic history, a genuine neurasthenia of a sexual type develops and annoys the patient for years. Masturbation rarely leads to insanity and is oftener a symptom than a cause of such disorder. It is perhaps most often associated with de- mentia prsecox. It is occasionally the cause of epilepsy. When this is the case the convulsive attacks are likely to put on a hysteroid phase and are accompanied by peculiar co-ordinated convulsions and emotional disturbance. Masturbation is the common cause of hystero-epilepsy in women. Diagnosis. — Many victims of the masturbation habit, who have come to recognize its evils and tried to stop it, develop a hypochondriacal con- dition, and feel sure that there is something in their faces which reveals to the world their trouble. This is not the case. But there is a certain physiognomy which in a measure characterizes the masturbator to such an extent that an experienced observer can detect it. The pale, pasty 532 DISEASES OF THE NERVOUS SYSTEM complexion, moist, furtive eye, dilated pupil, listless, restless, and de- pressed manner, the wet, flabby palms, and hypersesthetic skin, all help to tell the story. Locally, the penis is often reddened and more or less turgid, the scrotum relaxed, and a varicocele may be present. Examina- tion of the urine may reveal spermatozoa. The urine also is almost always of rather low specific gravity, and contains a great excess of phos- phates, both earthy and alkaline. Treatment. — The patient must be told plainly the necessity of stop- ping the practice. He must be impressed, but not terrorized. He should be kept out of doors at vigorous physical exercise, for sedentary and solitary work is always bad for such cases. He should be made to take cold-water baths and should sleep on a hard bed with light covering. He had better sleep with some one whose presence may exercise a con- trolling influence. He should not eat heartily at night, never just before going to bed. And what is still more important, he should not drink before going to bed. Sometimes it is well to have him wakened at an early hour in the morning, when he should empty his bladder; for emis- sions occur often early in the morning and are promoted by the irritation of a full bladder. Locally, cold-steel sounds may be introduced and allowed to remain for ten minutes, three or more times a week, or the psychophor or Ultz- mann's short catheter may be used. In bad cases with a great deal of prostatic irritation, local applications of nitrate of silver are needed. Internally, a mixture of tinct. opii, tinct. camph., and tinct, lupulin may be given at night, the ingredients being somewhat varied in amount to suit the case. Bromides, chloral, atropine, and salix nigra are also drugs which are often useful. The mechanical measures which have been de- vised for preventing erections, such as rings with sharp teeth, are rarely needed and rarely useful. They may even do harm by directing the mind to the affected function. I do not believe it right for the physician to prescribe fornication. It is not safe nor curative, apart from the moral aspect of the matter. It has always struck me also as pretty small business for a man purposely to select a wife to relieve him of the results of a weak will and vicious sen- sual indulgence. If marriage comes in the natural course of events, as it often does, so much the better. But to select a wife as a remedial agent for masturbation is unjust to the woman and a confession of moral and mental feebleness. Man is distinguished from the brute by his self- control. Let him bear this fact in mind and raise himself above the ani- mals by a determined effort of the will. Pure thoughts and chaste as- sociations, vigorous physical exercise, and a resolute effort to act a manly part will always be successful. NEURASTHENIA 533 TRAUMATIC NERVOUS AFFECTIONS {Traumatic Neuroses and Psychoses, Spinal Concussion) The present tendency of neurology is to deny the existence of any special nervous affection produced by trauma or shock. There may fol- low from these causes: 1. Surgical injuries. 2. Neurasthenic and morbid psychic states. 3. Hysterical states. 4. Hemorrhagic, inflammatory, and degenerative diseases. 5. Combinations of the foregoing. These troubles may follow not only railway but other injuries, but are especially liable to follow those associated with intense fright. 2. Traumatic neurasthenia or "traumatic neurosis," '"railway spine," does not differ from forms of neurasthenia produced by other causes, ex- cept that with it there may be certain sprains and surgical troubles and some morbid mental condition. Its special symptoms are described under the head of neurasthenia. 3. Traumatic hysteria is a rare affection in this country. It does not differ from hysteria produced by other causes, except for its sudden onset and occasional surgical complications. It is usually a hysteria major and has the characteristic stigmata of that type. In this city electrical injuries and frights have produced some classical cases of hysteria major. 4. There is considerable evidence that in some rare cases traumatism may produce minute multiple hemorrhages throughout the nervous centres. In such cases there are usually neurasthenic or hysterical symptoms and in addition symptoms of organic disease. Massive hemorrhages and serious mechanical injury of the nervous centres may be also produced by injury. Finally, it is a well-known fact that traumatisms may excite, in the predisposed and the infected, locomotor ataxia inebriety, insanity or may lead to the development of a cerebral tumor. It is the mental impression, the shock, much more than the physical injury, which produces the functional neurosis or psychosis. The symptoms may appear soon after the accident, or, after a period of relative health lasting some weeks the neurosis gradually develops. The most important practical point in connection with the subject is the diagnosis and the elimination of malingering. This is additionally difficult for the reason that the hopes and anxieties depending upon litiga- tion tend to cause introspection, exaggeration of symptoms and uncon- scious bias even in the most honest. The opinion among American neurologists tends to favor the reality of traumatic neuroses. "While malingering is not rare, yet if the patient has really a traumatic neuras 534 DISEASES OF THE NERVOUS SYSTEM thenia or hysteria the disease may not be a trifling one. Careful research, however, often tends to eHcit the fact that previous to the injury the pa- tient was an alcohoHc, syphihtic, or neurotic, and perhaps had already the beginning of his alleged traumatic disorder. In no part of clinical medicine is a careful and searching examination and weighing of symp- toms more urgently called for. The methods of carrying out such ex- aminations are given elsewhere. Special methods for testing anaesthesia are sometimes needed. The two sides of the body should be tested simul- taneously with concealed needles, beginning on the trunk, or the faradic current with a double-pointed electrode maj^ be used. There are few patients who can successfully deceive in an examination covering all the special senses. The treatment of these neuroses calls for no special notice here. CHAPTER XXIV THE MYOCLONIAS OR TWITCHING SPASMS (Chorea — The Spasmodic Tics) Myoclonia is, or should be, a general term used for twitching spasms of all kinds. Strictly speaking, it includes chorea, the convulsive tics, paramyoclonus multiplex, certain hysterical spasms, myoclonus epilepsy; also myokimia or fibrillary and wave-like twitchings, and rhythmic myoclonia. Myotonia, or tonic spasm, is a general term used to designate tonic spasms such as those of tetanus, tetany, the symptomatic tonic spasms of organic disease and hysteria, myotonia congenita or Thomson's disease and myotonia acquisita. Myoclonus is a special term indicating the twitching of a muscle. Myotonus indicates the tonic contraction of a muscle. The patient has a myoclonia; the muscle is in myoclonus. The term myoclonia has been much misused, and there is great confusion in the nomenclature. There are five more or less distinct clinical types, but these can be with sufficient accuracy reduced to three: 1. Infectious myoclonia, which is the chorea of Sydenham. 2. Psychogenic and degenerative myoclonias which include the spas- modic tics, and hereditary chorea. 3. The myokimia disorders, which include various symptomatic fibrillary and wave-like twitchings, and the so-called myoclonus multiplex of Friedreich. The twitching and tonic spasms and gross tremor often occur in the same disorder and we need terms like myoclonus-tonus and myoclono- tonia to indicate these combinations. CHOREA OF SYDENHAM {St. Vitus^s Dance) This is the common type of chorea, and is the disease ordinarily meant when the term chorea is used. It is a subacute disorder characterized by irregular, inco-ordinate twitching movements. The disease is a common one, forming about one-fifth of the nervous diseases of children. Etiology. — Most cases occur between the ages of five and fifteen. It is very rare under five. A few cases occur after twenty, and even up 535 536 DISEASES OF THE NERVOUS SYSTEM to old age, when a senile chorea is sometimes observed. It affects girls more than boys in the ratio of about 2.5 to 1. In adult life the dispro- portion is less marked. It is relatively rare in the negro race, especially in those of pure blood (Mitchell). It occurs in all climates. Most cases develop in the spring months, next in the autumn, next in winter, and last in summer. The seasonal influence varies in different localities. School attendance and work have something to do with these variations. The disease is more frequent in cities, and in the poorer classes. Hereditary influence is slight, but it exists. In a small percentage of cases one parent has had chorea, epilepsy, insanity, or a decided neuropathic constitution. The chief exciting causes are injury and fright, mental worry, and some infection, generally a rheumatic or tonsillar one. Fright or some emotional disturbance is a cause in about one-fifth of the cases. Acute rheumatism is given as a cause in very varying proportions, ranging from 15 to 20 per cent. Some authors lay much stress on rheumatism and, counting attacks of tonsillitis as evidence of rheurnatism, they find it a large causal factor. Endocarditis is developed in the course of chorea in about one-fourth of the cases, and this may exist without any other manifestations of rheumatism. Pregnancy is a cause of chorea, generally in primiparse and in young women under twenty-five. Chorea sometimes follows infectious fevers, especially measles, scarlatina and whooping- cough. Chorea is the prominent symptom in some septic cases, of which septic chorea (Sachs), and the serious mental disorder known as chorea insaniens are examples. Overstudy and the worry of examinations are factors in causing chorea in predisposed and badly nourished children; and anaemia and malnutrition underlie most cases. Symptoms.- — The disease may begin suddenly, but usually it develops slowly, and it is not till one or two weeks that the symptoms are decidedly prominent. It usually begins with irregular twitching of the hand or face on one side. The child winks, grimaces, jerks its head or shoulder, twitches its arms and drops things from its hand. The foot and leg become affected later and the child stumbles in walking. In two or three weeks the opposite side is involved, but usually less than the one originally affected. In three or four weeks the disease reaches its height. The patient's movements are then almost continuous. The hands can hardly be used and the child has to be fed and dressed; even walking is awkward and difficult. Speech is indistinct and confused from the irregular move- ments of the lips and tongue. An actual mutism may occur. The mus- cles of respiration may be involved so that the rhythm is uneven. The choreic movements usually occur both when the muscles are at rest and during volitional acts. In some cases the disease is chiefly characterized by inco-ordinate movements when purposeful acts are THE MYOCLONIAS OR TWITCHING SPASMS 537 attempted. In other eases voluntary movements can be readily per- formed, and the muscles twitch only when the limbs are at rest. The movements cease, as a rule, during sleep. But the child sometimes gets to sleep with difficulty on account of the movements. In severe cases attacks of mental excitement and even delirium come on for several successive nights. Apart from such incidents, the mind in chorea is usually dulled, the temper irritable, and the child much harder to manage (Fig. 233). The appetite is poor and capricious, the tongue coated, and the bowels are often constipated. The nutrition fails a little; there are anaemia and Fig. 233. — Chorea, showing grimace and shoulder movement. (Jacobsohn.) a tendency to loss of flesh. The eyes present nothing abnormal. Hypermetropia, astigmatism, and muscular insufficiencies exist, but not much more than in other nervous children. The child is often worse in the morning and improves toward night. Excitement and physical exertion make the movements worse. There is rarely any pain and never ansesthesia or tenderness. The muscles are weak but not actually paralyzed. The deep reflexes are somewhat lessened and the knee-jerk may be abolished. In a good many cases the so-called "tonic reflex" is present. When the patella tendon is struck the foot flies up, and instead of dropping back at once remains up for a second or more; or a second slight reinforcing jerk occurs. The 538 DISEASES OF THE NERVOUS SYSTEM electrical irritability of the muscles is, as a rule, increased, but there are no qualitative changes. Nocturnal enuresis occasionally occurs. The urine contains an excess of urea and phosphates and at the height of the attack the specific gravity may be increased. Forms. — -Maniacal chorea or chorea insaniens, is characterized by fever, choreic movements and great mental excitement — especially at night, with delirium, hallucinations and delusions. The patient has in effect a toxic or infectious psychosis. After two or three weeks the excitement lessens and the patient becomes dull and apathetic. Such cases usually occur in adult women, and they are often fatal. Paralytic chorea. In this form one arm or one side of the body be- comes rather weak and powerless. A few twitching movements are ob- served. This form occurs only in children and runs the same course as the spasmodic type. Chorea of adult life and senile chorea. The disease when it occurs in the second half of life attacks men rather oftener than women; it is of toxic or infectious origin, but not related to rheumatism. There is usually a neurotic family history of chorea. The attack is usually excited by emotional disturbances. It runs much the same course as juvenile chorea, but is rather more apt to become chronic. When it occurs in old men it is called senile chorea. This type is not to be confounded with hereditary or Huntington's chorea. Duration — Relapses. — ^The disease in this country lasts about ten or twelve weeks, ranging, however, from six weeks to six months. There may be great improvement followed by a relapse, and in this remittent manner the disease may last for years. If it lasts more than six months it should be called chronic. Relapses occur in about one-third of the cases and rather oftener in girls. Relapses occur oftenest within a year of the first attack and much oftener in the spring. After three years re- lapses practically cease. The number of relapses is usually but one, but the disease may recur eight or nine times. Relapses rarely occur in adults except in the chorea of pregnancy. Pathology. — Chorea has no definite anatomical basis, though the seat of the disease is in the brain. The irritant seems to come from and act first upon the blood-vessels, causing in severe cases intense hypersemia, with dilatation of vessels, small hemorrhages, and spots of softening. There are infiltration of the perivascular spaces with round cells and swell- ing and proliferation of the intima of the small arteries. In chronic cases the evidence of active vascular irritation is less, but there are perivascular dilatations and increase of connective tissue. The process suggests a low grade or an initial stage of inflammation. The cause of this is usually an infective micro-organism similar to that causing the rheumatic symp- THE MYOCLONIAS OR TWITCHING SPASMS 539 toms and the heart lesions. The infection, is sometimes and perhaps commonly due to the micrococcus viridans. In a considerable per cent, of cases (90 per cent. — Osier) especially in those of long duration, there are fibrinous deposits on the walls of the heart. The presence of points of irritation in the cortex and its meninges and in the deeper parts excites irregular discharges of nerve force and produces the choreic movements. The interruption of the voluntary nerve im- pulses by diseased foci makes these movements irregular. In para- lytic chorea the pyramidal tract is probably more seriously injured by some single large focus of congestion, exudation, or hemorrhage. Indeed, I have seen a true hemiplegia develop in the midst of an attack. In maniacal chorea the meninges and cortex are more involved. Diagnosis. — The disease is easily recognized by the peculiar twitching movements. It must be distinguished from convulsive tic, hereditary chorea, hysterical spasms which include saltatorj^ chorea and chorea major. The distinctions are not difficult and are given in connection with the descriptions of these disorders. Prognosis. — As regards life, the prognosis is very favorable. In this country death from chorea hardly ever occurs in children. It is more fatal in adults, and especially in pregnant women. This is the statement of European writers. I have never seen a mortality and many cases of chorea of pregnancy have been observed in my service at Bellevue Hos- pital. Nearly all non-fatal cases eventually get well of the chorea. Treatment. — The most important single factor in treatment is rest. The child should not be allowed to take violent exercise or to have any excitement. In most cases he should be taken from school, and in bad cases he should be kept in bed. In all cases he should be largely isolated and kept quiet. Cold packs should be given once or twice daily, if the movements are violent. Cold sponging or douching daily along the back is often sufficient. As specific remedies, arsenic should be given in doses of TTLv. of Fowler's solution t.i.cl., increased by one or two drops daily to fifteen or twenty drops or even more. If this causes nausea and gastric pain or headache, the dose should be stopped for a day and then resumed, if possible, where it was left off. The bromide of zinc is also an excellent remedy, when there is a hysterical element. CHoral is most useful in violent cases especially when the patient can not sleep. Hyoscine hydrobromate in doses of gr. 3^f oo is occasionally efficacious. The salicylates are now much used in the acute hospital cases. In chronic and obstinate cases hypodermic injections of the cacodylate of soda may be tried. A most potent remedy in some severe acute cases is the injec- tion of neo-salvarsan. 540 DISEASES OF THE NERVOUS SYSTEM HEREDITARY CHOREA {Huntington'' s Chorea) Hereditary chorea was first described by a Long Island physician, Dr. Waters, in 1842, later by Drs. Gorman and Lyon, and in ■ 1872 by Dr. Huntington to whom most credit for establishing the symptoms is due. The American cases have been observed chiefly in New York, Connecticut, New Jersey, and Pennsylvania. Cases have been re- ported also from Germany, France and England. The disease begins before thirty and not after fifty; it occurs about equally in males and females. It is almost always directly hereditary, either through father or mother, usually the latter. It begins without known cause by twitch- ings in the face; the movements then extend to the arms and legs. The movements are very much like those of chorea minor. They are, how- ever, apt to become more jerky and violent; the legs are especially sub- ject to jerky movements, causing difficulty and grotesqueness of gait. A patient of mine used to be frequentlj^ thrown out of bed by the move- ments. They can, however, often be voluntarily controlled for a time. The speech is early affected. There is no paralysis or disturbance of sensibility. The disease is attended by progressive mental deterioration, sometimes by a tendency to melancholia, and usually ends in dementia. Its course is chronic and very slow, lasting ten or twenty years. Post- mortem, chronic pachymeningitis and leptomeningitis with degenerative changes in the cells of the motor cortex have been found. Electric Chokea is a name sometimes and wrongly given to very violent forms of the ordinary chorea of Sydenham. The term was first applied by Dubini to a peculiar and progressively fatal spasmodic affection which has been observed almost solely in Italy, and which is perhaps of a podagrous or malignant malarial origin. M. Bergeron in 1880 also described an "electric chorea" in which the patients are attacked by sudden rhythmical spasms. This latter disease has a uniformly favorable course. Neither of these diseases resembles true chorea, nor do they have the character of the tics. The term electric chorea, therefore, is one that should be used, if at all, only with a qualifying explanation. PROcuRsrvE Chorea, or Dancing Chorea. — Laycock has described as a separate kind of chorea a rhythmical or trochaic form, which he says affects children, principally girls, and shows itself in spasmodic rhythmical contractions or in sudden rotating or procursive movements of the body. This has been called chorea procursive, or chorea festinans, by other writers. In many cases it is accompanied by vertigo, when the condition of the patient is similar to that of a person who has been whirling around a number of times. Such cases always have decidedly hysterical characters, although these procursive attacks may complicate ordinary chorea. Chorea Major is a manifestation of hysteria, and has been described under that head. It is not a chorea at all. THE MYOCLONIAS OR TWITCHING SPASMS 541 SPASMODIC TIC {Mental Tics, Habit Tics, Hysterical Tics, Local, General and Endemic Tics) Spasmodic tic is a disease to which the name of chorea is often, but incorrectly given. It is a very chronic disorder, and shows itself in the form of quick, electric-like spasms of certain groups of muscles or single muscles. With this there occur short tonic and rhythmical movements. The twitching movements differ from those of chorea in that groups of muscles physiologically related are affected and co-ordinated movements result. 1. There are local, habit and psychic tics. 2. Generalized tics with tonic and rhythmical movements. 3. Tics associated with other diseases, e.g., myoclonus epilepsy. 4. Hysterical tics. 1. Habit, Local and Psychic Tics. — There are many persons who go through life with some trick of speech, of gesture, or some peculiar grim- ace. It may be only a shrug of the shoulder, a twitching of the eyes, or a sniff. These various movements are tics of the co-ordinate kind. The spasmodic motion is of itself normal, but is inappropriate and misapplied. Such movements are often seen in children. They sometimes rep- resent abortive attacks of chorea, and sometimes they are the residuum of old attacks. In these tics, a reflex or central irritation often sets up the spasm. This irritation, such, for example, as that of an eye-strain or a nasal disorder is removed, and subsides from consciousness; but the subconscious feeling of it remains and produces a response, such as the grimace or the sniffling. Closely allied to habit tics are various local tics. Thus when the spasm is localized in the facial nerve, we have a facial or mimic tic (see p. 105). The spasm may become localized even in a single branch of a nerve, as that to the orbicularis, the zygomaticus, the diaphragm, or the tensor tympani. Spasmodic tic sometimes involves the trunk and legs. It may attack the muscles of expiration and the larynx, and then it has been called chorea of the larynx. Spasmodic tics are sometimes accompanied by explosive disturbances of speech. In these cases the patient at the time of the convulsive move- ment utters some obscene or profane words (coprolalia) , or involuntarily repeats the last words of the sentence spoken to him (echolalia) , or spas- modically imitates a gesture made to him (echokinesis) , or involuntarily exclaims the thought uppermost in his mind, perhaps revealing some secret against his will (tic de pensee). These speech-tics may occur with- out any other motor manifestation, and a tendency to exclamatory tics is not uncommon in ordinary life. 542 DISEASES OF THE NERVOUS SYSTEM 2. Generalized Tics {Degenerative Tics). — The convulsive movements here take a wide range and affect a number of groups of muscles, produc- ing quick, violent movements of the face, head, extremities and trunk, associated with short tonic spasm of the affected parts. Generalized tics affect children from birth or develop between the ages of six and sixteen years, and by preference the masculine sex. There is almost always a neurotic and sometimes a hereditary family history. The children are nervous, excitable, often retarded. The disease begins with attacks of spasmodic movements, affecting generally the head, face and upper extremities first, then perhaps involv- ing the whole body. The movements can be controlled for a time by the will, only to break out with increased violence later. They cease during sleep, which is generally profound. Degenerative generalized tics are chronic and incurable. When there is no gross change the brain shows an irregular arrange- ment and defective number of the cells of the motor cortex. Sometimes the brain is found damaged by infection, or an early cerebral diplegia. The treatment of tics is dealt with in connection with certain special types, e.g., facial tic, torticollis, writer's cramp. It is essentially hygienic and pedagogic. In the mild forms of habit spasm of children, bromide of zinc in doses of gr. ii. to tolerance is a very efficient drug. Spasmodic tic may be associated with epilepsy (myoclonus epilepsy). Of this there is a special familial type (Unverricht). Hysterical or psychogenous tics may simulate the local and the degen- erative forms. They are not so severe or general in their manifestations and are more apt to be manifestations of a sexual complex, the results of mimicry and a psychological contagion like the dancing manias of former centuries. The peculiar disorder of the Maine ''jumpers," characterized by sud- den violent movements on being touched or startled, is a form of endemic hysterical tic. The chseases known as latah, occurring in Malay, and myriachit, occurring in Siberia and Kamchatka, are also endemic and probably hysterical tics. FIBRILLARY MYOCLONIA (Myoclonus Multiplex of Friedreich) ' The exact nature of this rare disease has been misunderstood. It is probably due to some infection, but in the original case it followed a fright. It occurs mostly in adult males. In the description of the disease one cannot improve on that originally given by Friedreich. He states that it is a peculiar affection of the muscles, especially of the upper and lower extremities, showing itself in the form of short rapid contractions, reappearing at short intervals, which affect individual muscles of the arms and forearms, thighs, trunk and face. THE MYOCLONIAS OE TWITCHING SPASMS 543 It is not merely fibrillary or fascicular twitchings, but a spasm which involves the whole of the muscle which swells in a mass, and bulges every time, even when the contractions of the muscles were not marked enough to produce a noticeable motor effect on the limbs to be moved. Only at times, at an unusually marked contraction, one notices a slight change in the part to be moved, e.g., during many contractions of the biceps a slight flexion of the forearm or during some of the spasms of the supinator longus a slight supination of the hand. The spasms, are unrhythmical throughout and varying in extent, even though they are separated from each other only by short intervals. By placing the stethoscope on the affected muscles in themoment of their contraction, a loud and clear sound, resembling the first heart sound in every respect, can be appreciated. Every muscle shows a complete independence in relation to its indi- vidual contractions. Now it was this muscle, now that muscle, which twitches all for itself without any relation to the others, and if it some- times happens that the contraction of a muscle on one side takes place synchronously with another muscle of the same or other side, or even at times if two symmetrical muscles contract at the same time, it is only an accidental coincidence. The frequency and intensity with which the contractions of each single muscle ensue are not the same on different days or at different times of the day noticed. During the times of greater agitation there are 40 to 50 contractions per minute in one and the same muscle, while during the hours of greater rest only 10 to 20 contractions could be counted. Only rarely does it happen that the spasm ceases entirely, or almost entirely. The foregoing is based on Friedreich's description. It corresponds with several cases which I have observed except that sometimes the trunk muscles are greatly involved. It seems to be a toxsemic disorder, causing an irritation of the peripheral motor neuron. J. R. Hunt found hyper- trophy of the muscular fibres in one case. Much has been written into the original description and some cases have been classed as hysterical, but that cannot be the pathology of the originally described disease. In one case observed by mj^self the muscular phenomena were due to a general infection from which death resulted. MYOTONIA This is the general term given to tonic muscular spasms, and they may be irregular, rhythmical, localized, or very general in distribution. The myotonia may be acquired, and it is then called myotonia acquisita, or symp- tomatica. Not much is known of this as a definite malady. It occurs sometimes as an epiphenotnenon in progressive muscular dystrophy and myasthenia gravis. It is a not uncommon symptom of muscular exhaustion or deficient vascular supply. 544 DISEASES OF THE NERVOUS SYSTEM Thomsen's Disease {Myotonia Congenita). — This is a hereditary and family disease characterized by the development of tonic spasms when the patient attempts volun- tary movements. The disorder is very rare. Etiology. — Congenital myotony is practically always hereditary and runs in fami- lies. It affects males by preference and develops at the time of adolescence. Symptoms. — The patient notices that on trying to rise or walk his legs are seized with a painless spasm, which in a few seconds relaxes, but comes on again when the muscular movements have been repeated. If he closes his hands tightly a cramp occurs and he cannot relax the grip. If he shuts his eyes he cannot open them for a moment. The muscles of mastication may be affected, but the extremities are the Fig. 234. — Myatonia congenita. (Spiller.) parts most involved. The involuntary muscles are spared. The spasms are increased by cold and nervousness; they are lessened by muscular exercise. The muscles are somewhat hypertrophied, and the patient may present the appearance of a very strong man. The actual strength is fair, but less than would seem. The general health may be good, but the patients sometimes show the signs of low vitality in weak diges- tion, feeble sexual power, and susceptibility to cold. The electrical excitability of the nerves is normal, that of the muscles is increased, and there is produced a contraction tetanus by both currents. In addition, Erb describes a peculiar reaction produced by a strong stabile galvanic current. It con- sists in the appearance of wave-like muscular movements passing from cathode to THE MYOCLONIAS OR TWITCHING SPASMS 545 anode. This is not always present. The mechanical excitability of the muscles is also increased. Pathology. — The disease is probably a primary muscular dystrophy, a view sup- ported by its occurrence in the wasting dystrophies. There may be, however, a peculiar defect in innervation resulting from a congenital anomaly of the motor tracts. The muscular fibres are found to be hypertrophied, the striations indistinct, and the nuclei increased. The diagnosis is easily made by the characteristic tonic spasms. The prognosis is bad as regards cure, but the disorder does not shorten life. Treatment. — Dr. Thomsen, who first described the disease, states that active muscular exercise benefits patients. No specific measures are known. Symptomatic Myotonia and Paramyotonia. — Paramyotonia is the name given to a form of myotonia in which the symptoms deviate somewhat from the typical ones that appear in Thomsen's disease. Congenital paramyotonia is a family affection, resembling in this respect Thomsen's disease. The muscular rigidity is brought on not by voluntary movements, but by exposure to cold and often very slight degrees of cold. The tonic spasm is a long one and lasts for from a quarter of an hour to several hours. It affects the arms more than the legs. The facial muscles are prone to become rigid. The attacks are fol- lowed by some muscular weakness. In congenital paramyotonia the trouble is un- doubtedly a primary disturbance of the muscles; in other words, a myopathy. Symptomatic paramyotonia is noted most characteristically in a certain form of paralysis agitans. Here the patient, when attempting to walk or to rise from the sitting posture, is suddenly seized with an apparent rigidity of the muscles which pre- vents him from stirring. The trouble is probably more central than muscular. The myotonic condition appears in the progressive muscular dystrophies, and in spastic paralyses of spinal and cerebral origin. Ataxic paramyotonia is the name given to a disorder characterized by transient spasms like those of Thomsen's disease, associated with distinct ataxia and also with weakness and some anaesthesia (Gowers). This disease is probably located in the spinal cord and should perhaps be considered one of the forms of symptomatic para- myotonia. No special treatment can be given for either of the two latter forms of disease, of which very few examples have been observed. Hypotonia and Myatonia.- — A few cases have been observed in which from birth the muscles have shown a pe)sistent and extreme hypotonia. This forms a condition known as congenital myatonia (Fig. 234). It is thought by some to be a fo'm of dystrophy. Minor degrees of myatonia occur in many people and are not to be re- garded as morbid conditions. Hypotonia is more marked generally in the female sex, and it is developed by acrobats and freaks. It exists as one of the symptoms of Mongolian idiocy. TORTIPELVIS {Dystonia Musculorum Deformans) Cases of this curious disease have been described by several American neurologists, notably by Dr. J. Fraenkel who called it tortipelvis. Oppen- heim has described it under the name if dystonia musculorum deformans. It is a functional myotonic disorder characterized by peculiar tonic spasms of the muscles of the trunk and pelvic girdle, which are brought out especially on attempting to walk. The disease develops in early life, affects both sexes and various races. It is chronic in its course. 35 546 DISEASES OF THE NERVOUS SYSTEM When the patient attempts to walk his trunk and thigh muscles are seized with slow, irregular, tonic and sometimes clonic spasms, which bend the body forward, sideways, and twist it. The effect is to produce an awkward and grotesque and so-called ''dromedary" gait. The legs also are involved in the spasms, the neck and arms but slightly (Fig. 235). When the patient lies down or sits quietly the spasms cease to an extent, but begin again when he attempts voluntary movements. There are no disturbances of sen- sation or of the reflexes. The mind is clear. The spasms are much like the myotonia of spasmodic tic. The physiognomy of the disease, its re- sponse in a degree to pedagogical treatment, the absence of all evi- dences of organic disease all sug- gest that the malady is allied to the spasmodic tics. TETANUS Tetanus is an acute or sub- acute infectious disease character- ized by violent and painful tonic spasms with remissions and exacer- bations. It is due to infection by the tetanus bacillus. It is called idiopathic or medical tetanus when no open wound is found and trau- matic or surgical tetanus when such condition is present. When it attacks infants it is called tet- anus neonatorum; when the jaws alone are involved it is called lock- jaw, or trismus. A form which af- fects the face and throat is called head or cephalic tetanus. The idiopathic or medical tetanus is more chronic in its course. Etiology. — It has a special predilection for newborn children in some countries (West Indies) and to a less extent for puerperal women. It affects males more than females (4 to 1). After the first month of life there is practical immunity till after the tenth year. It then increases in frequency to about thirty. Most cases occur between the fifth and Fig. 235. — Torti pelvis — Dystonia mus- culorum. {Dj-. Goodhart.) THE MYOCLONIAS OR TWITCHING SPASMS 547 twenty-fifth year (Anders). It is rare after forty to fifty. It is much more frequent in dark races and in some tropical cHmates (West Indies, South and Central America). It is more frequent in some regions of the temperate zone than others because of an infected soil. It is more com- mon in hot seasons. It is caused generally by traumatism, and most often by contusions and penetrating soil infected wounds of the hands and feet, hence its prevalence in times of war. In cephalic tetanus there is traumatism of this extremity. In this country traumatism of the hands on Fourth of July causes many cases. Symptoms. — The disease sets in from five to fifteen days after infec- tion. It begins with feelings of stiffness in the neck and throat and some- times with chilly feelings. Gradually tonic spasms develop which involve the trunk and head muscles, causing opisthotonos and other forms of rigid spasm. Trismus, or lockjaw, also occurs. The spasms are attended with intense pain. The patient finally lies in a state of general rigidity interrupted by painful tonic spasms. The muscles are of stony hardness. The jaws are set and the facial muscles are involved, the eyes being partly closed, the corners of the mouth drawn up, causing the characteristic risus sardonicus. There may be profuse sweating; some leukocytosis is present; tissue metabolism is not much changed. Fever is generally present and this may rise as death approaches to 110° or 112°. The disease lasts from two to five weeks. There is evidence of irritation and congestion of the spinal cord and injured nerves, but no special anatomical changes are found. A specific bacillus producing a tetanizing poison has been discovered, the bacillus tetani. The diagnosis is based on the characteristic history and the peculiar spasms. In strychnine poisoning there is no initial trismus or epigastric pain. In rabies there is also no trismus, but a respiratory spasm on at- tempts to swallow. The prognosis is bad. About 80 per cent, of traumatic and 40 per cent, of slow idiopathic cases die. The treatment consists of complete rest and quiet in a dark room and the adminsitration of chloral, bromide, morphine, and physostigma. Successful results from injection of blood-serum of an animal which has had the disease are reported. A tetanus ''vaccine" has been obtained and lately used viiih success. This must be administered both intra- spinally and intravenously in maximum doses. TETANY (Tetanilla) Tetany is a subacute or chronic spasmodic disorder characterized by intermittent or persistent tonic contractions beginning in the extremities and associated with paraisthesiffi and hyperexcitability of the motor and sensory nerves. 548 DISEASES OF THE NERVOUS SYSTEM Etiology. — The disease is rare in this country, but relatively common in Europe, especially in Austria. It occurs with frequency during the second, third, and fourth years of life and again at the time of puberty. Its rate of frequency then slowly declines and it is very rare after fifty. It affects males much oftener than females up to the age of twenty; after that the difference disappears. It occurs mostly in the working classes. In infants rickets is often noted. The exciting causes are exhaust- ing influences like diarrhoea, lactation, sepsis, fatigue, mental shock, and fevers; also exposure to cold and wet. Alcoholism, dilatation of the stomach, and intestinal entozoa are also causes. It may be produced artificially by extirpation of the para- thyroid gland. The disease sometimes appears as an epidemic. Symptoms. — Tetany begins sometimes suddenly with symmetrical tonic contrac- tions of the hands; at other times there are at first sensations of numbness, prickling or pain in the extremities, with malaise and perhaps nausea; then spasms begin. The attacks affect first and most the upper extremities. The flexors of the forearm and hand are usually involved; the fingers are flexed at the metacarpo-phalangeal joint and extended at the other joints, and the thumb is adducted, producing the "accoucheur's hand." The forearm may be flexed and the upper arm adducted. The knees and Fig. 236. — The Trousseau symptom. feet are extended, the toes flexed, and the foot is inverted. In severe cases the muscles of the abdomen, chest, neck, and face are involved. Opisthotonos and dyspnoea may result. The muscles of the face and eyes develop contractions, and trismus sometimes occurs late in the disease. The muscles of the larynx, oesophagus and bladder may be affected. Fibrillary tremors are observed in the contracted muscles. The attacks are accompanied by parsesthesias and cramp-like pains. There may be some aboli- tion of sensation in the skin of the parts affected during attacks. The cramps last from a few minutes to hours or days. They occur during day and night and may wake the patient from sleep. Fever is sometimes present in epidemic cases. The disease has a tendency to recurrence. While it lasts, both during and between the attacks, peculiar phenomena are ob- served as follows: 1. Increased Mechanical Irritability of Motor Nerves. — The motor nerves show an abnormal irritability, so that on striking the motor point a sharp muscular contrac- tion is brought out. When pressure or a blow is made on the face over or near the exit of the facial nerve from its foramen, contractions of the facial muscles occur, es- pecially those of the lips. This is called the "facial phenomenon." By pressing on the artery and nerve of a limb a tetanic attack can be produced in the muscles supplied. THE MYOCLONIAS OR TWITCHING SPASMS 549 It is probable that it is the pressure on the nerve alone which causes the phenomenon which is called "Trousseau's symptom" (Fig. 236) 2. The electrical irritability of the muscles and nerves is increased, especially to the galvanic current. Thus a negative-pole closure contraction (CaCC) is brought out by a very weak current; and if a little stronger it causes a tonic contraction or cathode- FiG. 237. — The parathyroids. {Halstead and Evans.) closure tetanus (CaCTe). The positive-pole opening contraction (AnOC) may be tetanic, i.e., AnOTe, and there may be even a cathode-opening tetanus (CaOTe), a phenomenon not seen in any other disease. According to Gowers there may be a reversal of the polar formula, so that a positive-pole closure contraction occurs earlier than a negative (AnCC>CaCC). This is certainly rare. 550 DISEASES OF THE NERVOUS SYSTEM 3. An increase of irritability of the sensory nerves is shown by pressing upon them, when sensations of prickhng and formication appear along their course. There is an increase also in the electrical sensibility, shown by appreciation of very weak galvanic currents. The auditory nerve reacts to the galvanic current in about 15 per cent, of normal cases, and then only to strong currents and to only a partial extent; but in tetany it reacts in nearly all cases, and with comparatively weak currents (2 to 5 or 6 ma.) on anode closure, anode fixed, and anode opening (AnC Klang, AnDKl, AnOKl) (Chvostek). The phenomena of hyperexcitability above described vary considerably and rapidly during the course of the disease, and are not always present. Types of the Disease. — The disease varies in intensity and duration. This varia- tion depends much upon the cause, and there have been a number of types of the dis- ease based on the etiology. Thus we have: 1. Epidemic tetany. 2. Asthenic tetany due to lactation, diarrhoea, exhausting diseases, etc. 3. Parathyroid tetany, due to removal of the parathyroid glands. ^ 4. Reflex and toxic tetany from gastric dilatation and intestinal worms. When the spasms are continuous the disease lasts but a few weeks; when they are intermittent it may continue for months. Epidemic cases last but a few weeks. The disease may be said in general to last from a few weeks to a few months. It is rarely fatal. Patients are liable to a recurrence on return of the exciting cause. Pathology. — The phenomena of the disease indicate a congested and irritative condition of the gray matter of the spinal cord. The cause of this state is evidently in some cases (epidemic tetany) an infectious poison; in other cases as auto-toxin in the blood (parathyroid tetany), and in other cases it may be a rheumatic or some other toxic influence. Ergot is known to produce symptoms resembling tetany. In infantile tetany the irritation is apparently due to rickets and the reflex irrita- tion of disordered bowels. It is doubtful if any reflex influence can be invoked in adults. Tetany is a functional disease and the symptomatic expression of a toxaemia. This poison may be of different kinds, hence tetany has a claim to be called a distinct disease simply on clinical grounds. It has no such definite pathology as chorea or epilepsy. In the very few autopsies which have been made no definite organic lesion has been found. Diagnosis. — -The disease is usually easily recognized by the character of the spasms, their symmetrical nature, their course, and the phenomena of hyperexcitability of the muscles and nerves. Trousseau's symptom is found in no other disease. The "facial phenomenon," the peculiar electrical and mechanical irritability of the muscles and nerves, are very rare in other conditions. The sensory irritability, especially that of the acoustic nerve, is also characteristic. From tetanus the disease is distin- guished by the intermittency of the contractions, their feebler character, the fact that they begin in the extremities and extend to the trunk, and by the absence of trismus, at least until late in the disease. Treatment. — ^The cause should be removed if possible, lactation stopped, diarrhoea and indigestion corrected, the stomach washed out, worms expelled, rickets if present 1 A distinct form of tetany is due to disease of or removal of the parathyroid glands. It is believed that when tetany follows operations upon the thyroid gland itself, it is due to the removal of one or more of the parathyroids. These glands are, in human beings, very small objects — about half the size of a pea, four in number-;-the upper pair and the lower pair; and, according to Dr. Halstead, they have a definite vascular supply. In cases of tetany, due to partial or complete removal of these glands, the tetany can be kept under control by feeding the patients with these glands or the nucleo- proteid obtained from them. THE MYOCLONIAS OR TWITCHING SPASMS 551 attended to. Rest, nourishing food, and tonics are indicated. Symptomatically, bromide of potassium in doses of 5iss. to 3ij- daily with chloral furnishes the surest relief. Hyoscine in doses of gr. Jd^oo may be tried. Inhalation of chloroform or in- jections of morphine are needed in severe cases. Lukewarm baths may be of service; so also may ice bags to the spine. Parathyroid feeding or injection of the nucleo- proteid should be tried. RABIES AND HYDROPHOBIA Rabies is an acute specific disease, communicated to man from the lower ammals, and characterized by severe and painful tetanic convul- sions, paralysis and death. The disease occurs in nearlj^ all parts of the world, and is common throughout the United States. All mammals are susceptible to the disease, and birds also may contract it. Etiology. — The commonest source of infection is the bite of a dog; but the disease may be caused by the bite of cats, wolves, horses, cattle, and other domestic animals. The bites of wolves are the most dangerous, next those of cats, and next those of dogs. It is not communicated by the bite of a rabid man. Bites upon the face and hands, the exposed parts of the body, are more dangerous on account of the rich nervous supply and from the lack of the protection of the clothing. The disease may be transmitted by saliva upon an abraded surface. About one- sixth of persons who are bitten by rabid dogs, become infected. Men are more susceptible than women, and nearly half of the cases occur in persons under 20. Rather more cases occur during the months from April to September. The infection is contained in the saliva, and is secreted mainly by the parotid. Other glands may contain it, and it may be excreted in the milk. The blood and lymph are never virulent. In the infected person the virus pervades every part of the central nervous system, but is especially concentrated in the medulla. It exists also in the cerebrospinal fluid and large nerve trunks. The microbe of rabies has not been discovered. Whatever it is, it makes its way into the nervous system, along the nerve trunks. It has an incubation period of about fort}^ days, ranging between twenty and ninety days. The characteristic histological change in rabies is certain bodies dis- covered in the nerve-cells by Negri. They are called the Negri bodies. These are not easily affected by external agencies, even by putrefaction, and are easily stained and observed under the microscope. By means of observing these bodies, the diagnosis, after death, of the disease is very easily made. The Negri bodies are specific of rabies, and they have been thought to be protozoa. This has not been proved, and it is still most probable that the virus of rabies is an ultramicroscopical body which is in some way connected with tissue proteids which form the Negri bodies. 552 DISEASES OF THE NERVOUS SYSTEM Symptoms. — As a rule, there are no symptoms during the incubation period. When the disease develops, there is some numbness, tingling, and perhaps radiating pain at the point where the bite occurred. The person suffers from nervousness, anxiety and depression; the sleep is dis- turbed, and then some symptoms referable to the throat appear. There is early a difficulty in swallowing. In a few days the symptoms of mental excitement appear. The patient becomes more restless, nervous and insomnolent. He suffers from thirst, but cannot quench it, because at- tempts to drink cause a painful spasm of the throat. As a result,, while he desires to drink, the sight of water frightens and distresses him. Hence, the name hydrophobia. Then he begins to have attacks of convulsions, which affect first the muscles of the neck and throat and then involve the whole body, causing symptoms resembling tetanus. At other times, there are very active co-ordinated convulsions, like those of hysteria. Slight sounds or any mental excitement will bring on an attack. De- lirium and maniacal excitement complicate the condition, which lasts from one to three days. A general paralysis follows, and the patient dies in coma, from exhaustion, the temperature rising quite high. In cases where the infection is very severe, the system is overwhelmed by the virus, and the patient dies very quickly with an ascending paralysis which re- sembles a so-called Landry's paralysis (paralytic rabies). Diagnosis. — -In the early stages of the disease, the mental condition of the patient and the convulsive attacks are very suggestive of acute hysteria. The history of a bite and the person's respiratory spasms and throat spasms will generally exclude this. In tetanus, there is more or less continuous rigid spasm between the attacks; the history of the disease and the longer period of incubation in rabies enable us to distinguish it. Prognosis. — ^When the disease comes on, the issue is surely a fatal one. The careful application of the Pasteur treatment, by inoculation, reduces the mortality after bites from about 16 to one-half of 1 per cent. The treatment after the attack comes on is purely symptomatic, and consists in the use of morphine, chloroform and motor depressants, such as curare. CHAPTER XXV EXOPHTHALMIC GOITRE (FAJANPS DISEASE, GRAVES' DIS- EASE, BASEDOW'S DISEASE) AND HYPERTHYROIDISM Exophthalmic goitre is a chronic glandular neurosis characterized by easy fatigability, rapid heart beat, enlargement of the thyroid gland, protrusion of the eyeballs, tremor, and various neurasthenic, metabolic and vasomotor symptoms. Etiology.— Exophthalmic goitre occurs much oftener in women than men (four to one). It is a disease of early adult life, occurring chiefly between fifteen and thirty-five, very rarely in childhood, and never after fifty. It is apparently more common in the Anglo-Saxon race, but is not very frequent in America, at least in the Eastern States. I am informed that it is rather common in the Northern Central States. There is very rarely any direct inheritance of the disease, but the family is often a neuropathic one. As a rule, the patient is of a neurotic temperament. Anaemia and debilitating diseases promote its development. Syphilis is rarely present, and probably not a cause. Goitre and heart disease do not seem to predispose to the real trouble, but a goitre may lead to a symptomatic Graves' disease characterized mainly by cardiac disturb- ances and perhaps some cervical sympathetic symptoms. The most frequent exciting causes are powerful and depressing emotions and severe physical exertion. Rarer causes are injuries and infectious diseases, such as influenza, measles, scarlet fever, typhoid fever, and pneumonia. It sometimes develops after such infections without any previous nervous strain or shock. I have met no cases following directly upon traumatism. HYPERTHYROIDISM The symptoms grouped under the term exophthalmic goitre represent a phase in the pathological history of the thyroid gland. When this gland is atrophied and inactive we have hypothyroidism and myxoedema; when over-active, hyperthyroidism and exophthalmic goitre. Qualitative changes occur in which hyper- and hypo-symptoms are mingled. Exophthalmic goitre, according to Rogers, may be preceded by hypo- thyroidism, this may be followed by hyperthyroidism, and finally the fully developed type of exophthalmic goitre appears. Hyperthyroidism is a not infrequent condition, and is characterized by nervousness, emotional weakness, a kind of hypo-manic restlessness, 553 554 DISEASES OF THE NERVOUS SYSTEM insomnia, fatigability, moist skin, tendency to palpitations, and other of the minor vasomotor and secretory symptoms of Graves' disease. The condition may go no farther than such hyperthyroid state, or it may eventuate in the final and more definite disease. Symptoms. — The disease usually begins gradually, and the first symp- tom is in most cases rapid heart beat, associated with attacks of palpita" tion. The patient is nervous, easily fatigued, and has sometimes tremor. The next symptom is enlargement of the thyroid gland, and at about the same time the eyeballs begin to protrude. This order of develop- ment does not always take place, and occasionally one or more of the five principal symptoms is not present. The disease is usually one or two years in developing, the heart symp- toms being those which begin first and end last. With the symptoms above mentioned there occur many minor troubles which are more or less characteristic. The patient is usually very nervous and irritable; a dis- tressing insomnia may be present. There is almost uniformly a fine tremor (eight to nine per second) of the hands, less marked in the lower limbs and not present in the face or tongue. The reflexes are exaggerated. There is a tendency at times in walking for the knees suddenly to give way. The patient rarely has neuralgias, but does have burning or fever- ish sensations and headaches. The skin is rather reddened and moist, and the patient at times sweats profusely. Pigmentation and vitiligo are sometimes seen, and urticaria may develop. The electrical resistance of the body is much diminished, being 800 to 1,500 ohms instead of 2,000 to 3,000. There is sometimes a dermographic skin, as in other neuras- thenic states. The respiratory function is weakened and chest expansion often falls below one inch (Fiske-Bryson) . Attacks of a persistent watery diarrhoea occur. Anaemia is usually present. There is occasionally polyuria, more rarely glycosuria. A study of the urine shows an increase in general metabolic activity. The menses are irregular and amenorrhoea often exists. A slight rise in temperature may occur. The blood-pressure varies but eventually is somewhat increased. The major symptoms of the disease are: Mental and physical fatigue. Tachycardia. Goitre. Exophthalmus. Tremor. The minor symptoms are: Nervousness. Sweating. Insomnia. Lessened electrical resistance. EXOPHTHALMIC GOITRE 555 Subjective sensations of heat. Diarrhoea. Polyuria . Increased metabohsm. The sense of fatigue, and the easy fatiguability have been dwelt upon correctly by Rogers, as a fundamental symptom. It is practical^ the same condition as that found in neurasthenia proper. Next to this and as common, is the symptom of tachycardia. Symptoms in Detail. — The pulse beats from 100 to 120 per minute usually, but may rise to 160 or even 200. Its rhythm is usually steady; but palpitations occur easily, even without exciting cause. The patient may wake up at night with distressing attacks, something like those of angina pectoris, but the intense pain and sense of imioending death are ■ IPIH m ^r «K^^^^^^^H 1 Tri^ n ' 'P' ~^Ka- ^giii fcw^M iijijii^w 1 lM Fig. 238. — Exophthalmic goitre. usually absent. The heart is dilated and a systolic murmur is often heard at the base propagated along the arteries. Real organic disease, however, is rare. The arteries are dilated and soft. They pulsate strongly, particularly the carotids. A thrill is sometimes felt over the heart and always over the goitre. The arterial tension is normal or low at first; later it is high. The thyroid gland is usually enlarged symmetrically; later in the dis- ease the isthmus is affected and the three lobes of the gland stand out prominently (Fig. 238). If only part of the gland is involved it is oftenest the right lobe. A thrill is felt over it and a systohc murmur can be heard. The enlargement of the thyroid is slight or not detectable in a small per cent, of cases. The bulging of the eyes or exophthalmus is usually bilateral and even. 556 DISEASES OF THE NERVOUS SYSTEM If one eye is alone or more affected it is the right. The exophthalmus varies much in degree. It is not usually very great, but may be so ex- cessive as to prevent closing of the lids and to expose the insertions of the recti. The eyeball may be slightly enlarged (one-tenth) . The exoph- thalmus is due to the overaction of certain muscular bands which lie in the orbit (Miller's muscle) ; also to stimulation of the superior tarsal muscle which retracts the upper lid. These muscles are under the in- fluence of cervical sympathetic, and not the autonomic fibres. The orbital space, as the eye is pulled forward, is gradually filled by fat and connective tissue. Hence section of the cervical sympathetic does not lead to more than a slight relief of the exophthalmus. The pupils are normal and vision is not impaired, though myopia occasionally occurs. The fundus and visual field are normal. Paralysis of some of the eye-muscles is a rare complication. Weakness of the internal recti and exophoria are frequent. The inability to converge the eyes is known as Moehius' symptom. The lids show certain peculiarities. One of these, known as Von Graefe's symptom, consists in the inability of the lid to follow the downward movement of the eyeball. When the patient is told to follow the movement of the finger vertically downward the eyeball moves steadily, but the lid catches, as it were, and refuses to follow or does so in a jerky manner. Another symptom, known as Stellwag's symptom, is the widely open eye, showing white around the iris with infrequency in winking, especially of the upper lid. A tremor of the lids sometimes occurs. Course. — The disease progresses slowly. After a year or two it often becomes stationary for a long time. Cases of gradual spon- taneous recovery occur. The natural duration of most recoverable cases is two or four years. In those which do not recover, the disease lasts five, ten, or more years; the patient emaciates, the heart becomes weaker, albuminuria and dropsy appear, diarrhoea sets in, and the patient dies of exhaustion or is carried off by phthisis or some inter- current disease. Other cases, having improved up to a certain point, remain in this state for years. Complications. — Mental derangement occasionally occurs in the later stages of the disease. Hysterical crises, epileptic attacks, choreic movements, paralysis of the ocular muscles, muscular atrophy, paralysis agitans, Addison's disease, diabetes, locomotor ataxia, and local oedema have all been observed. With the exception of hysterical attacks, these complications are rare. Abortive Forms. — ^This name is applied to cases in which only a part of the distinctive symptoms develop. It generally means hyperthy- roidism with some of the major symptoms of exophthalmic disease. Tachycardia always exists; with it are tremor and moist skin, lessened EXOPHTHALMIC GOITRE 557 electrical resistance and nervousness. Or tachycardia and goitre may alone be present. Physiology. — ^The bio-chemical studies of the thyroid shows that it develops certain peculiar iodinized proteids which are characteristic of its secretory activity. The exact nature of the substances is not perfectly known. The action of the thyroid is to stimulate metabolism, and in- directly to promote certain functional activities. Its secretion seems necessary for the growth and development of all the tissues. The thyroid has a certain interrelation with the pancreas, adrenals, thymus and pituitary. Pathological Anatomy. — ^Post-mortem examination of the thyroid gland shows that it undergoes a true hypertrophy with increase of vas- cularity and of the glandular structure, and increase of colloid material in the alveoli. After the hypertrophy has reached a certain stage, the glandular epithelium degenerates and breaks down, filling the acini with degenerated cells. Later still there may be atrophy and fibrosis with sclerosis of the vessels, hemorrhages and cyst formation. In the nerve-centres the changes which have been found are small hemorrhages in the medulla and degenerated nerve-cells. In one case of about a year's standing, I found a very marked pigmentation and vacuolization of the cells of the vagus and glossopharyngeal nuclei. In another case of six months' standing, no marked changes could be seen in these areas, but there was a spot of softening at the junction of the pons and the cerebral peduncle. This was ante-mortem and had led to crossed paralysis just a few days before death. In other cases congestion and small hemorrhages in the medulla have been found. In still other cases there were no lesions, so that an actual anatomical change in the nervous centres cannot be said to be established. The heart is dilated and enlarged; endocarditis is sometimes present, oftener not; the arteries are dilated. Pathology. — Some writers now consider this disease primarily one due to a disordered function of the thyroid gland. This is not entirely the case. Graves' disease occurs in neuropathic persons. It is often associated with a neuropathic family history and often neuroses compli- cate the thyroid trouble. The exciting and fostering causes are often mental and nervous strain. While the symptoms of the disease, the nervousness, the insomnia, and the vasomotor disturbances are due to the hypersecretion of thyroid, the morbid disturbance often may well be fundamentally a loss of central nervous control. If the nervous centres can be kept quiet long enough, the demand on their part for this excess of thyroid juice gradually ceases and the patient gets well. This is the rationale of the prolonged rest which is the real and specific cure for the disease in its ordinary phases. 558 DISEASES OF THE NERVOUS SYSTEM Prognosis. — About one-fifth of the cases get well or practically well. Probably over half the cases, if they can be properly treated, reach a fairly comfortable condition of improvement. The cases in which symptoms come on quickly have the most favorable prognosis. In those with marked exophthalmus and goitre the prognosis is not so good. The duration of the disease in recovering cases is from two to eight years. A later development of hypo-thyroidism and myxoedema is not in accord with my observation though it may occur. I have recently seen three now healthy patients who had Graves' disease over twenty years ago. Diagnosis. — The disease can be distinguished by the persistent tachycardia, with goitre or exophthalmus, and in its early stage by the tachycardia with tremor, moist skin, sensations of heat, nervous- ness, emaciation, insomnia, lessened respiratory expansion, and electrical resistance. A symptomatic Graves' disease may sometimes be caused by a goitre pressing on the vagus or sympathetic and causing irregular heart beat and perhaps exophthalmus. In these cases the history of a long- standing goitre exists, the heart's action is irregular, the exophthalmus is usually partial and one-sided. In abortive forms it is necessary to have tachycardia and at least one other of the four major symptoms to make a diagnosis of Graves disease. Treatment. — Rest is the most important single thing. The patients sometimes need to be put to bed or kept on the back for one to three months. Sometimes a half-rest is enough, with freedom from excite- ment and worry, A sea voyage can be worked in with advantage. No special diet is needed, nor do climatic influences or baths or mineral waters have much effect, except in so far as they secure a quieter and better environment. The drugs used are numerous. Of these, the phosphates, the lime salts, iodine, strophanthus, aconite, belladonna, bromides, and iron have served best. In certain very mild cases with small goitres, thyroid extract in small doses does good, but as a rule it is harmful. Beebe and Rogers have obtained an anti-thyroid serum which, in acute and serious cases where there is great thyroid toxaemia, acts suc- cessfully. In the more chronic and quiescent cases it is not so efficient. Beebe has recently prepared another serum which is given hypodermic- ally. A serum from thyroiclectomized goats has given some results. The surgical treatment of exophthalmic goitre is not always successful; and it sometimes happens that symptoms of hypothyroidism develop later. It is best to get the patient into a relatively quiescent state by medical treatment and rest before operating. And it is advisable usually to begin with ligation of arteries in order to see whether this may not be sufficient EXOPHTHALMIC GOITRE 559 before any of the gland is removed. According to Rogers the ligation under local anaesthesia of one or more of the thyroid vessels or the removal of one-half or more of the thyroid gland yields about 50 per cent, of cures. Ligature of the arteries is safer but not so immediately efficient as thyroidectomy. If the patient needs for economic reasons prompt relief, with a certain attendant risk, he should try surgery, at least till we have better medical measures than we have at present. Surgery, or at least thyroidectomy, is dangerous in actively progressive cases and in those associated with a psychosis.^ The X-ray and radium are said to have value, and I have seen the latter accomplish results. For the palpitations, the ice bag placed over the heart and neck is helpful and may be used systematically. 1 S. Landstrom's statistics of cases of himself, Kocher, Mayo, Riedel gave cure or improvement in about 80 per cent. ; death in about 10 per cent. Landstrom in 38 cases reports cure in 48.7 per cent., improvement in 17.1 per cent., failure in 26.8 per cent., death in 7.4 per cent. Mayo's figures on cures or improvement 87.8 per cent., death, 12.2 per cent. CHAPTER XXVI PROFESSIONAL NEUROSES, OCCUPATION NEUROSES (Writer's Cramp and Allied Affections) Writer's cramp is a nervous affection characterized by spasmodic, tremulous, or paralytic disturbances, occurring when the act of writing is attempted. Since the introduction of the typewriter, writer's cramp has become less common. In its place we have, with relative less fre- quency, stenographer's and typewriter's cramps. Etiology. — It is a disease of modern times, and has been particularly noted since the introduction of steel pens, about the year 1820. A neuropathic constitution is often present, and sometimes there is a hereditary history. Men are much more subject to the disease than women. The most susceptible age is between twenty-five and forty. It rarel}^ occurs after fifty or before twenty. Clerks and professional writers are naturally much more subject to the disease. Excessive worry, intemperance, and all debilitating influences predispose to it. The chief exciting cause is excessive writing. But this is not all. The writing that is done under strain or a desire to finish a set task is the harmful thing. The style of writing is also an important factor. Writing done in a cramped posture with movements of the finger alone or with the little finger or wrist resting on the table is most injurious. Free- hand writing done from the shoulder according to the American system is least harmful. Shaded or heavy writing with sharp steel pens is also productive of harm. Copying is much more harmful than composing. Authors seldom have writer's cramp. The performance under strain of some specially prolonged task is the most common exciting cause. Injury and exposure to wet and cold occasionally start the trouble. Symptoms. — Writer's cramp very rarely attacks a person suddenly. The patient first notices a certain amount of stiffness occurring at times in the fingers, or the pen is carried with some uncertainty and jerky movements are made. He feels a sensation of fatigue in the hand and arm, and this may amount to an actual tired pain. The first symptoms may last for months or even years. The hand is rested as much as possible; new pens or penholders and new modes of holding it are tried. Often the patient, fearing the onset of the cramp, and as its result loss of employment, becomes anxious, worried, and mentally 560 PROFESSIONAL NEUROSES, OCCUPATION NEUROSES 561 depressed. Sometimes the trouble is worse when beginning a daily task, and it gradually wears off in a few hours. At other times exactly the reverse is the case. When the disease has reached its highest stage, writing becomes almost or entirely impossible. The moment the pen is taken in the hand and an attempt at using it made, spasmodic contractions of some of the fingers, or even of the arm, occur, the pen flies in any direction, and it is impossible to control or co-ordinate the movements. The rule is that, although writing cannot be done, all other complex movements are performed as well as ever. Thus the sufferer from writer's cramp may be able to play the piano, or paint, or thread a needle, or use the hand in any complex movements. This limitation, however, is not always present. Telegraphers, who use to some extent the same muscles as in writing, and who also often have to do a great deal of writing, are liable to suffer from both writer's and telegrapher's cramp at the same time. No evidences of actual paralysis are present in the affected muscles and there is rarely anaesthesia, but the arm aches and is sometimes tender. Sensations of numbness and prickling are present and occasionally a complicating neuritis develops. In rare cases vasomotor disturbances are observed. There are sometimes associated muscular movements of the other arm or of the neck. The various symptoms occur with different degrees of prominence, so that the disease has been classed under the heads of (1) the spastic, (2) the neuralgic or sensory, (3) the tremulous, and (4) the paralytic forms. These forms are, however, often more or less mixed. 1. The spastic form is undoubtedly the most common, and it has given to the disease its name. Cramp of some muscle or muscles is present in over half of the cases. The muscles of the thumb and first three fingers are oftenest affected, and in some cases the flexors, in some the extensors, are chiefly involved. In telegrapher's cramp it is the extensors, but in writer's cramp the flexors, that are mainly attacked. 2. The neuralgic form resembles the spastic plus sensations of fatigue and pain, which are quite severe and are brought on by writing. There may be tenderness over the arm nerves also. 3. The tremulous type, though rare, is very characteristic. The patient when attempting to write develops a tremulous movement of his hand and arm. This ceases when his attempts to write cease. The tremor usually affects most the fingers used in pen prehension, but it also spreads to the forearm and may even involve the entire extremity. Although this tremor develops on attempting to write, it does not de- velop on other voluntary movements. 4. The paralytic form, or that type in which muscular feebleness is the dominant symptom, is also rare. In the typical paralytic form the patient, as soon as he begins to write, feels an overpowering sense of weak- 36 562 DISEASES OF THE NERVOUS SYSTEM ness and fatigue in the fingers and arm. The fingers themselves loosen their grip and the pen may drop from the hand. Associated Symptoms. — Writer's cramp is essentially a motor neurosis, and its leading symptom is the impairment of a motor function. Other symptoms, however, both general and local, are always associated with it. These are mainly (1) psychical and (2) sensory, more rarely (3) vasomotor and (4) trophic. 1. Psychical Symptoms. — -The patient is often nervous, emotional, and mentally depressed at times. He suffers from insomnia and vertigo. Patients are generally unwilling to admit that there is any other trouble than the local one, and only careful examination may bring evidence of constitutional trouble. There are cases of purely mental writer's cramp. 2. Sensory Troubles. — ^These consist of pains, sense of fatigue, feel- ings of numbness, prickling, pressure, weight, tension, constriction, etc. Hyperesthesia and, more rarely, anaesthesia are also observed. The most common sensory symptom is that of aching and fatigue, and this is usually confined to the arm, and oftenest runs along the course of the rachal and median nerves. The cervical vertebrae may be tender and sometimes patients have a headache in the parietal region of the side opposite the affected arm. 3. Vasomotor, Trophic, and Secretory Disturbances. — ^The condition known as digiti mortui has been observed, coming on paroxysmally. In acute cases of writer's cramp with associated neuritis decided vascular changes may occur, such as passive congestion of the hand and arm, with swelling and turgescence of the fingers, and a sensation of throbbing. In bad cases the fingers will look as if they had chilblains. Electrical Reactions. — Ordinary tests will, as a rule, reveal very little change. Sometimes there is a quantitative increase, sometimes a decrease, of irritability to both forms of current. Pathology. — Neuritis is undoubtedly present in some forms of writer's cramp, so called. It is not present, however, so far as external tests go, m the typical neurosis. Nor are there any post-mortem ob- servations throwing light on the anatomy of the disease. We must believe, therefore, that it is a neurosis having no appreciable anatomical basis. While writer's cramp is often complicated with some neuritic dis- turbance leading to symptoms in the affected arm of pain, paralysis, tenderness over nerves, vasomotor disturbances, etc., there can be no doubt that the lesion in typical cases is central and involves the higher reflex centres and indirect motor and sensory paths. Little more can be said of the pathology than that it is an "exhaustion neurosis," and that its mechanism is allied to the spasmodic tics and especially to stuttering. The same is true of all the other forms of occupation PKOFESSIONAL NEUROSES, OCCUPATION NEUROSES 563 neuroses, and nothing need be said upon this point regarding them when they come to be considered. The diagnosis of well-marked cases of writer's cramp presents no difficulty. In the earlier stages, however, it may be confounded with a large number of disorders, viz., post-hemiplegic chorea, hemiataxia, paralysis agitans, progressive muscular atrophy, progressive locomotor ataxia, various forms of tremor, lead paralysis, rheumatoid arthritis, neuritis, cerebral and nerve tumors, and tenosynovitis. In many of these cases it is only necessary to bear in mind the history of the disease in order at once to reach a safe conclusion as to its nature. If there is a great deal of pain in the arm, with tenderness along the course of the nerves; if there is decided change in the electrical reactions; if there are sensations of tingling, numbness, etc.; and if the patient shows an absolute loss of power in the various groups of muscles, with some incapacity for doing other acts besides the one with which he is specially concerned, then the trouble is undoubtedly peripheral and due largely to an underlying neuritis. The prognosis in these cases is much more favorable. If, on the other hand, the dis- order comes on in persons who have done an excessive amount of writing; if it is associated with nerve strain; if the electrical reactions are but slightly changed, the sensory symptoms slight and the motor inco- ordination is marked, limited to the special class of work, and not ac- companied with absolute paresis, the disorder is central and needs both a different treatment and prognosis. Course and Duration. — Writers' cramp is a chronic disease. It begins insidiously and attacks one group of muscles after another as each is brought into play by new methods of writing. If the left hand is used, that, too, is liable to become affected. The course varies, how- ever; for a time progress may be arrested or improvement set in. When the disease becomes well established it will most often last a lifetime. Prognosis. — -The prognosis is unfavorable, yet not so much so as was once thought. Undoubted cases of complete recovery have been reported, even under unfavorable conditions. The prognosis is much more favorable if the patient begins treatment early and before marked spastic symptoms are present. It is more favorable in the neuralgic forms. Some patients who suffer from a mild form of the trouble manage, by the help of instruments or special pens, to do their work for years. The more acute the disease and the more evidently peripheral and neuritic its origin, the better the prognosis. In over one-fourth of the cases, patients who use their sound arm will not be affected in it. The facts stated regarding the cause, physiology, and general symp- tomatology of writers' cramp apply to the other forms of occupation neuroses. A few special details, however, will be given regarding these. 564 DISEASES OF THE NERVOUS SYSTEM The most common and important are musicians' cramp and telegraphers' cramp. Musicians' Cramp. — Under this head we include pianists' cramp, vioKnists' cramp, flutists' cramp, and the cranip of clarionet players. Pianists' cramp occurs usually in young women who are studying to become pro- fessionals or who are especially hard working and ambitious. The absurd "Stuttgart method" of teaching the piano, in which the motions are confined as much as possible to the fingers, predisposes especiallj^ to this disease. The symptoms are those of fatigue, pain, and weakness. The pains are of an aching character. They are felt in the forearm especially, but extend up the arm and between the shoulders. Spas- modic symptoms are rare. The right hand is oftener affected, but both hands even- tually become involved. Violinists' cramp may attack the right hand which holds the bow or the left hand which fingers the strings, but more often the left hand is affected. Clarionet players sometimes suffer from cramp of the tongue and of the laryngeal muscles. Flute players suffer not very infrequently from slight laryngeal spasms. A similar trouble affects elocutionists. The term mogophonia is applied to this type. Telegraphers' cramp affects especially those operators using the Morse system, which is still the one most widely in vogue. Contrary to the opinions of previous writers, Dr. Lewis believes that this neurosis is not a rare one and is destined to become more frequent. In this city the cramp is not rare, the proportion being about one in every two hundred. The technical name among operators for the cramp is "loss of the grip." In telegraphing, the extensors of the wrist and fingers are called most into play, and hence are most and earliest affected. The symptoms come on very slowly, the thumb and index-finger being first affected. The victim finds that he cannot de- press the key on account of spasm in these muscles, and he finds most difficulty in making the dot characters, such as h (....), or p ( ), or z (. . . .). When the flexors are most affected, the key is depressed with undue force and a dash is made instead of a dot. Sufferers from the "loss of grip" generally have writers' cramp also. While spasm is usually present, the disease may show itself simply in pain, paresis, and incapacity to co-ordinate the muscles. In sewing-spasm, which affects tailors, seamstresses, and shoemakers, clonic and tonic spasms attack the muscles of the hands on attempting to use them in the regular work. Tailors who sit crosslegged sometimes suffer from a peculiar spasm on assum- ing this position. Smiths' spasm, or "hephcestic hemiplegia" appears to have been observed only by Duchenne and Dr. Frank Smith. It occurs in persons engaged in pen-blade manu- facturing, saw straightening, razor-blade striking, scissors making, file forging, etc. In doing this work they have to use a light or heavy hammer, with which strokes are delivered very rajDidly and carefully. After a time spasmodic movements occur in the arm used, and the arm falls powerless. As in the cases reported, there are gen- erally hemiplegic symptoms, and also neuralgias, vertigo, and other cerebral troubles, the disease cannot be a purely functional neurosis. Drivers' spasm has been observed in veterinary surgeons by Dr. Samuel Wilkes. Milkers' spasm is an extremely rare affection, which was first described by Basedow and seems to occur in milkmaids, never in milkmen. Cigarmakers' cramp is very rare and often associated with some neuritis. Watchmakers' cra?np and photographers' cramp are also to be regarded merely as pathological curiosities. PROFESSIONAL NEUROSES, OCCUPATION NEUROSES 565 Ballet- Dancers' cramp. — Under this name certain painful and parah'tic troubles occurring in ballet dancers, especially j^remieres danseuses, have been described by Schultz, Onimus, and Kraussold. It does not appear that the trouble is really a co- ordinative functional one, but is rather neuralgic or the result of local strain upon the parts. The list of professional neuroses is made to include, besides those above given, cramps and co-ordinative troubles affecting artificial-flower makers, billiard players^ dentists, hide dressers, drummers, electrical instrument makers, stampers, turners, sewing-machine girls, money counters, weavers, painters and pedestrians. Prophylaxis and Treatment. — The introduction of typewriters, gold pens, and improved penholders has prevented somewhat the in- crease of writers' cramp. Stenographers rarely have it unless they write in long hand also. Persons who have to write a great deal should use large cork or rubber penholders and gold or quill pens with smooth paper. The best style of writing is that done from the shoulder, but Fig. 239. — Mathieu's instrument for writers' cramp. Fig. 240. — JN'ussbaum's instrument. this is a method that bookkeepers and those who have to keep accounts cannot easily adopt. Many nervous persons have a bad habit of gripping the pen very tightly and pressing down on the paper with excessive force. Fatigue soon results and painful sensations develop in the arm. Proper attention should be paid to the position of the paper written upon, the height of the desk, the light, and the sleeves of the coat or dress. The paper should be laid at an oblique angle to the edge of the desk, and not at a right angle as many writing-teachers are accustomed to direct. As some cases of "cramp" are undoubtedly cerebral, it is very unwise to attempt any extraordinary exploits in writing or to work with the ambition to put the writing-capacity to the utmost test. When the cramp is fully developed, the most essential thing is rest, and it is generally best to advise the patient to change his occupa- tion at once. Some rest, however, may be secured by getting a new form of penholder, holding the pen in a different way, using the unaffected arm, or using some form of mechanical appliance. The mechanical 566 DISEASES OF THE NERVOUS SYSTEM appliances are splints, rubber bands around the wrist, and various in- struments contrived to prevent spasm and throw the work of writing on new and larger groups of muscles. Instruments for writers' cramp are very numerous. Those that are of some value are Mathieu's, Nussbaum's, and some modification of Cazenave's (see Figs. 239, 240). All the various instruments have been of service, or have even been curative in some special cases, but not too much must be expected of them. As a rule, they are only pallia- tive. A cheap instrument that may prove satisfactory is that of Mathieu. In the medical treatment of writers' cramp the important agents are massage, pedagogics, and rest. CHAPTER XXVII PARALYSIS AGITANS (Shaking Palsy, Parkinson's Disease) Paralysis agitans is a chronic progressive disease, characterized by tremor, muscular rigidity and weakness, and by a peculiar attitude and gait, together with sensations of heat, pain, and restlessness. Etiology. — -The disease occurs more often among men than women in the proportion almost exactly of 2 to 1 (155 males, 82 females). More than two-thirds of the cases occur between the ages of 45 and 60, and the serious decade is the fifth. Practically no cases occur before 35 and very few before 40. In 127 personal cases the age of development of the disease was: 20-30, 2 30-40, 8 40-50, 40 50-60, 46 60-70, 24 70-80, 7 127 The disease is by no means rare in this country and affects the native stock seriously. Among 90 private cases 47 were of distinctly American stock, 13 occurred among Hebrews, and 8 among Germans, 8 among Irish. It is not especially an urban disease, many cases coming from small towns. It affects educated and professional classes more than it does laboring men. Fifteen per cent, of my private cases were lawyers, doctors and clergymen, one was an engineer and one a teacher. It is a disease of brain-workers more than muscle-workers. Hereditary influence has some importance. Among 90 cases there was a history of apoplexy, heart or renal cUsease in the direct ascendants in 19 cases, showing a weakness in the direction of the circulatory sys- tem. There was a direct heredity of paralysis agitans in 2, an in- direct (paternal uncle and aunt) in 2, and a family or senile tremor among 8. Thus about 15 per cent, show a hereditary weakness as regards tremor. Melancholia was present in the ascendants of 7 cases, and a tendency to anxious depression is very common among the patients 567 568 DISEASES OF THE NERVOUS SYSTEM themselves, three of whom had the melancholia of involution. Dia- betes was present in the ascendants in 2 cases and in 3 of the patients themselves. Not many families, aside from those referred to, were neurotic or rheumatic, and the children of the patients were al- most uniformly reported healthy. Some hereditary influence thus is present in 10 to 20 per cent., shown mainly in a tendency to benign tremor, stuttering or a tic, or in arterial sclerosis leading to cardiac cUsease and cerebral hemorrhage. Paralysis agitans is distinctly a disease of sober living. It is not caused by the ordinary departures from lectitude, such as dissipation, sexual, alcoholic, tobacco or food excess or even by lues. The thing most commonly observed is a regu- lar laborious and anxious life. The patient, if a man, has not been a vacation man, and his habits have been, if anything, abnormally regu- lar. Among direct causes, there can be no doubt that work and worry and psychic trauma are the most important. Shock and injury are noted in 2 per cent, of cases, but the influ- ence is not important in all of them. In the working classes, ex- posure and hard work are cited as causes, but there is usually a men- tal element of anxiety also. About 10 per cent, of men are smokers, very few (4 per cent.) are drinkers, and syphilis is almost never present (2 cases). Rheumatism has been put down as a cause, but few patients are really sufferers from articular rheumatism. Many, however, as the disease comes on, suffer from lumbago, sciatica and various forms of muscular pain, which are usually rather premonitions than causes of the disease. Fig. 241.— The attitude in paralysis agitans. PAEALYSIS AGITANS 569 Diabetes was present in three cases, but it is probably a coincidence. It sometimes follows acute infections and has perhaps a relationship to rheumatoid arthritis, or metabolic and autotoxic rheumatic condi- tions. The view that it is a "parainfectious" malady, due to the late action of an infection, has been suggested. Symptoms. — The disease is sometimes ushered in with an acute illness, or an attack of neuralgia. It usually develops slowly with some aching pains in the arm and a slight tremor in the fingers of one hand, oftener the right. For a period of several months the patient perhaps simply knows that he has an arm. Then the tremor becomes distinctly marked in the hand. It gradually extends and involves the leg of the same side, then the other side becomes affected. The neck, face, and tongue are rarely attacked by tremor, and then to a small extent. After or with the tremor there comes on a stiffness in the arms and legs, and indeed of the whole body. With this there is a general contracturing and shortening of all the flexor groups, so that the head and body are bent forward, the fingers are straight but are flexed as a whole on the metacarpus, the forearms flexed on the arm, the trunk is flexed forward on the thighs, and the knees are slightly bent. The attitude gives the idea of extreme senility (Fig. 241). The gait is slow, the steps are short and shuffling; the patient has trouble in starting, stopping, and turning corners, owing to the slowness in initiating new movements in the voluntary muscles. When once started, he may be unable to stop and has to run along. The speech becomes affected in one or two years. The voice is high-pitched, weak, and piping, or senile in quality. There is a slowness in getting out words or in start- ing a sentence, though after it is begun the words come rapidly. The condition is analogous to the festination in the gait. Along with the other symptoms, there are often, though not always, sensations of heat, burning, fever, and rarely of coldness. These sen- sations are felt most in the feet, legs, or arms diffusely. Often there is a general feeling of restlessness and nervousness. Aching pains and a sense of fatigue occur; neuralgic pains are more rare. There are al- ways a peculiar redness and flush in the faces of the patients. Some- times they sweat profusely. The temperature in the axilla is normal, on the skin it is sometimes increased. The appetite is excellent, often abnormafly great, and digestion is good. Visceral complications are rare. Muscular weakness comes on early; it slowly increases, but real muscular paralysis does not occur. The disease ends in rigidity, which makes the patient as helpless as if paralyzed, but the muscles pre- serve considerable functional power to the last. The deep reflexes are present and not, as a rule, exaggerated; but exaggeration and even clonus occur in a small percentage of cases. As the disease progresses 570 DISEASES OF THE NERVOUS SYSTEM the tremor increases in extent, and continues with little remission dur- ing all the waking hours; later the limbs get more rigid and in a way this rigidity drowns out the tremor; the patient becomes weaker and suffers keenly from sensations of fatigue and exhaustion. At last he can no longer walk; he becomes bedridden and is finally carried off by exhaustion or some intercurrent illness. It will be seen that the dominant symptoms in paralysis agitans are: 1. Tremor. 2. Rigidity, progressively increasing, with trunk deformities and contractures. 3. Muscular weakness. 4. Sensory, vasomotor and metabolic disturbances. Further details must be given regarding these symptoms: Fig. 242. — Festination in paralysis agitans. The tremor is a coarse one. It ranges from about 6 vibrations per second to 3.7. The average rapidity is 4 or 5 per second, which is about one-half the normal muscular rhythm. But the chief characteristic of the tremor is that it continues when the hand or limb is at rest, while voluntary motion causes it to cease. As the hand rests on the knee it shakes; as it is moved the tremor stops. When held straight out there is no shaking for a moment, but it soon begins. A glass of water is carried safely to the lips. The patient can control the tremor for a moment, especially in the early stages of the disease. These facts about the tremor apply in 90 per cent, of cases, but there are patients whose tremor is slight when the limb is at rest and is increased on volun- tary effort. The hands are affected in a characteristic way. The fingers and thumb are slightly flexed and held about in the writing-position; the tremor moves the fingers and thumb as a whole, and they vibrate PARALYSIS AGITANS 571 SO that the one pats the other gently. Sometimes the tremor is one of alternate supination and pronation of the forearm. The neck and face muscles are not usually or extensively involved, the shaking of the head being generally the result of the general bodily tremor. Sometimes one sees a tremor of the lips or neck muscles. The tongue and eye muscles are practically never involved in tremor. Rigidity. — The rigidity comes on early, and may be the first and even the only prominent symptom. It affects chiefly the flexors of the arms, head and trunk, and legs, producing a characteristic senile posi- tion. In rare cases the extensors of the neck are affected and the head is drawn back. Cramps occur, and there is always a sense of stiffness. The muscular movements are slow, especially the initiation of a move- ment. Once started, a motion may be quickly done. The gait is peculiar; the steps are short and shuffling; the patient may have dif- ficulty in starting; but once started he goes very well; or while walk- ing there may be a sudden running forward. This is called "festi- nation." Rarely there is a tendency to run backward or sideways. Some muscular weakness sets in early and slowly progresses, apparently with degenerative changes in the muscles. There is, however, no real nerve paralysis, but rather a muscular inefficiency due to the rigidity and not accompanied with degenerative electrical reactions. Tlie innervation of the facial muscles is weakened and the movements slow so that the face becomes smooth and expressionless and mask-like. The muscles are overirritable to mechanical irritation and the facial responses to blows are exaggerated. The voice in paralysis agitans al- ways becomes affected. It grows weaker, the singing power is lost, then capacity to talk long or loud. The lawyer has to give up trying cases. Speech is slow, difficult and the voice high-pitched, feeble and piping. Examination shows sometimes tremulous movement of the cords, but more characteristic is the slow, weak movement of the cords. In other words, the intrinsic muscles become weak and stiff and a little tremulous like the other muscles of the body. The handwriting in paralysis agitans soon shows characteristic features. The act of writing becomes difficult; the letters are formed slowly and painfully; they are drawn rather than written. The letters get smaller and more imperfect. Sometimes the writing is distinctly tremulous; more often the characteristic is smallness and lack of finish of the lines, the letters finally becoming obscured. From year to year the words are written more imperfectly and in smaller letters. Tremu- lousness, slowness, lack of finish of letters and progressive micrographia are the characteristic traits. The eye movements share the general tendency to functional arrest. The patient stares steadily ahead. Lateral movements both of the eyes 572 DISEASES OF THE NERVOUS SYSTEM and of head are difficult. But the vision remains good, and with glasses the patient continues to read though convergence is tiring. The sensory- nerves do not become involved except that pains of a neuritic or aching and rheumatic character occur. An initial attack of sciatica or brachial- gia is not uncommon. The muscular sensibility is exaggerated. This cause a feeling of restlessness that is very annoying. The vasomotor system is lacking in tone and stability. A characteristic flush early shows on the cheeks of the patient. The skin is ruddy, there is a tendency to sweats, and sensations of heat are often noted. The patient likes a cool room. The spinal centres are gradually weakened. Constipation is the rule; sexual power is gradually lost; the bladder after some years becomes irritable and there is a sense of frequent desire to micturate. This, of course, occurs often in the older cases, but it is a vesical not a ^yfC^^,^^ .-^-^^C^^-^ Fig. 243. — Showing progressive micrography. Above, handwriting in 1900; below handwriting seven years later. Fig. 244. — Showing tremor in paralysis agitans. prostatic trouble. Examination shows a congested condition about the neck of the bladder. The patient is often emotional, but the mind is not seriously affected. The urine is usually about normal, but contains an excess of phosphates. There may be polyuria and less often glycosuria. Forms. — A not very uncommon type is one with a great deal of tremor and hardly any rigidity, at least not until very late in the disease. About equally common is the type ''without tremor" and with rigidity, limitation of movements and deforming attitude alone. Almost always even in these cases a little tremor is sometimes seen. Unusual types of paralysis agitans are the hemiplegic or the monoplegic, and the retrocollic type. Course and Duration. — The disease slowly but steadily progresses until a full development of symptoms occurs, when it may remain sta- PAEALYSIS AGITANS 573 tionary. It takes about two years for the whole body to be affected, though this varies much. It lasts from three to twelve years or even more. In three cases of mine, death occurred in three, six, and eleven years. Death is due to exhaustion and may be preceded by what seems like a toxic delirium with perhaps some fever. The diagnosis must be made from senile tremor, multiple sclerosis, post-hemiplegic tremor, writers' cramp and wry-neck affecting the ex- -v\\^ :Ss$^' ^<"r^y** - Fig. 245. — Muscular changes in late paralysis agitans. tensors bilaterally (retrocollic spasm). Senile tremor occurs in the old. It affects the head and there is no rigidity. In multiple sclerosis the tremor is more jerky and is a tremor of intention; there are nystagmus, syllabic speech, and often apoplectiform attacks, eye trouble, and paralyses. Post-hemiplegic tremor is accompanied by a history of hemiplegia; there are paralysis and exaggerated reflexes and the disease is unilateral. The absence of exaggerated reflexes, the peculiar voice, gait and at- 574 DISEASES OF THE NEEVOUS SYSTEM titude, and the sensations of heat and nervousness often help greatly in the diagnosis. The prognosis is favorable as regards life ; unfavorable as regards 4 \ ''-"■«> ""f- '"^w^ 1 V* lii : 4 - ', 1^ ,-// "'• "-' J <:zM^^^' / / i, i Fig. 246. — Cells of the anterior horn of the spinal cord. The right row from a case of paralysis agitans, showing atrophy and pigmentation, the left row from a normal case. cure, and not very good as to bringing about a cessation of progress in the symptoms. The progress of the malady, however, can be delayed. Pathological Anatomy and Pathology. — The post-mortem changes are not very marked, and are seen mostly in the spinal cord and brain- PARALYSIS AGITANS 575 stem. There are congestion and dilatation of vessels in the gray matter, a diffuse increase of interstitial tissue, atrophy and pigmentation of cells. The disease has yet no established pathological anatomy. De- generative lesions have been found in the anterior cornual cells of the spinal cord, and these resemble somewhat those of senility, but the trouble cannot be explained as just a premature senility of the peripheral neurons, for extreme old age does not present the picture of paralysis agitans. Degenerative changes have been found in the muscles, but these are probably secondary and it is not possible to explain the vasomotor disturbance, the mental disturbances or even the tremor by a muscular lesion alone. The disease seems to impair first the automatically acquired activities, so that to do an accustomed act like writing requires a distinct and persistent voluntary effort. This would lead one to think that the trouble was at the point of co-operation between the voluntary and the automatic mechanism. The tremor and rigidity, without evidence of pyramidal disease, point also to this region and the extra- pyramidal tracts. And in old cases at least definite degenerations are found in these tracts. It is still unknown whether the exciting cause of the functional degeneration is a glandular defect, an infection, or a toxin. Dr. Thos. W. Hastings has made careful studies of the blood in seven personal cases of mine. In four of them evidences of infection by gonococcus or a micrococcus or both were found. Treatment. — -The most important measure is rest, mental and phys- ical, with plenty of fresh air. No special diet is indicated. Hot baths and mild massage are agreeable and helpful. I know of no climatic cure. The galvanic current produces temporary relief; it should be given daily. Hyoscine hyclrobromate, first used by Charcot and in- troduced into this country by Seguin, is of much value in relieving the tremor (gr. 3^1^0 increased). Codeine and morphine are of help if used carefully. A fairly good dose given once in the morning may be tried. Quinine and mineral acids are of much service in relieving the vasomotor and sensory symptoms. Bromide often helps the insomnia and restless- ness. Polyvalent vaccines and injections of neo-salvarsan are being tried. Full doses of asafoetida sometimes help. The mind in paralysis agitans is sometimes in an emotional, almost hysterical condition, and patients are easily made better for a time by some psychical influence. Violent exercise does harm. Regular systematic training exercises do some good, vibration is useless and so are hypnotism and osteopathy. CHAPTER XXVIII TROPHIC AND VASOMOTOR DISORDERS Progressive facial hemiatrophy is a disease characterized by a pro- gressive wasting of one side of the face. Etiology. — It begins oftenest in the young between the ages of ten and twenty. Females are more affected.^ There is in rare cases a hereditary history. Injury and infectious fevers sometimes start up the trouble. The left side is oftener attacked. Symptoms. — The disease begins very gradually and shows itself first in patches. The skin gets thinner, there is loss of pigment, hairs Fig. 247. — Facial hemiatrophy, early stage, showing alopecia and osseous depressions. fall out, including those of the lids and the areas may have a yellowish appearance. Sometimes the periosteum and bone are affected, and shallow depressions are formed which may be anaesthetic (Fig. 248). The subcutaneous tissue is most involved, the muscles suffer least, and there are no changes in electrical reaction. The muscles of mastication are usually spared. The bone undergoes general atrophy and the lower jaw may be reduced to two-thirds the normal size. The secretion of sebum ceases, but that of sweat may be increased. The temperature falls. There are a sinking in of the eye, narrowing of the lid, and dilata- 1 About one hundred cases have been reported. Among five, seen by myself, three were in females, two in males. The disease in all cases, so far as could be found, began between the tenth and twentieth years. 576 PARALYSIS AGITANS 577 tion of the pupil. There is sometimes pain and rarely anaesthesia. The tongue and very rarely the shoulder-girdle or the other side of the face may be involved. Spasmodic movements of the muscles of mastication have been noted (B. Sachs). Scleroderma sometimes, appears on the face or hands. The trouble may be com- plicated with other diseases of the nervous system, such as epilepsy, myoclonia and psychoses. The disease progresses rather rapidly at first, but finally comes to a standstill. It does not shorten life. Pathology. — There has been found a degenerative neuritis in- volving the fibres of the trigeminus and its descending root. The weight of evidence is in favor of the trouble being due to disease of the upper cervical sympathetic. The diagnosis is easy. Hemi- plegia with atrophy in children, congenital asymmetry, and atrophy from gross lesions of the nerve are distinguished by their stationary character or the presence of severe pain. Treatment. — There is no treatment known to be of service. Tonics, iodides, and electricity may be tried. Dercum has suggested resecting the trigeminal nerve. It would be worth while investigating surgically the superior cervical ganglion. Progressive facial hemihypertrophy is an ex- tremely rare condition only eleven cases having been reported. It is usually congenital in origin, but may develop in connection with giantism, as in a case of my own (Fig. 249). Fig. 248. — Facial hemiatrophj^ born and Krieger.) (Schoen- FiG. 249. — Facial hemihypertrophy oc- curring in a giant. DISEASES OF THE PITUITARY GLAND The pituitary gland is composed of an anterior part derived from the buccal ectoderm which has to do with nutritive changes and the development when diseased of acromegaly and giantism; a pars intermedia which produces a substance that stimulates unstriped muscle, produces diuresis and secretes a prod- uct which passes into the cerebral ventricles and has some influence on 578 DISEASES OF THE NERVOUS SYSTEM the growth and functions of the nervous system; a posterior part which consists mainly of neurogha. Feeding the anterior part to young animals seems to stimulate growth. The posterior lobe extract has no physiological effect. The pineal gland seems to have somewhat anal- ogous functions. Pathological and Physiological Effects Attributed to the Activities or Disorders of the Pituitary body Pineal body Adiposity. Sexual changes. Genital atrophy and infantilism. Acromegaly and gigantism. Polyuria. Control of carbohydrate metabolism. Lowered temperature. Co-ordinate action with other glands. Physiological action of extract of the gland. Pressor and depressor effect on blood- vessels. Galactagogic effect. Stimulation of muscles of pupil, uterus and intestines. Modifications of metabolism and bodily growth. Modification of carbohydrate metabolism. Adiposity (later sometimes a marked atrophy of fatty tissues). Early development of sexual organs and functions. Early bodily and mental maturity (Macro- genitosomia of Pellizi). Physiological action of extracts of the gland. Contradictory reports as to pressor and depressor effect on blood-vessels. Stimulation of unstriped muscular tissue of intestines, uterus, pupil. Vasodilatation of genitalia and kidney. Transitory diuresis. Glycosuria. Stimulation of metabolism (Berkeley). Three disorders are recognized clinically as due to disease of the pitui- tary gland; giantism, acromegaly, and the Frohlich syndrome, a condition characterized by stunting of growth, adiposity, and hypoplasia of the genitals (Fig. 250). Acromegaly is a chronic dystrophy characterized by gradual enlargement of the hands, feet, head, and thorax, and by a dorso-cervical kyphosis. Etiology. — It affects the two sexes nearly alike. It begins between the ages of eighteen and twenty-six, although a congenital case has been reported. No hereditary influence or definite exciting cause is known. The patients are sometimes naturally endowed with large extremities. Symptoms. — The disease begins with a gradual enlargement of the bones and soft parts of the hands, feet and head, including the nose, lips and jaws. In women there is amenorrhoea, in men, sexual weakness; slight rheumatic pains, headaches, malaise, mental hebetude, anaemia, and general weakness are present. The patient shows striking contrast between his bulk and his general strength. The skin is dry and there is polyuria. The hypertrophy affects the soft parts as well as bones. In these latter there are periosteal thickening and hyperplasia, with the result of producing increase in width more than length. The arms are not much involved, nor is the shoulder-girdle, except TROPHIC AND VASOMOTOR DISORDERS 579 the clavicle. The lower jaw is much more involved than the cranium. The tongue, lips and nose are enormously hypertrophied (Fig. 251) . The thorax is enlarged antero- posteriorly and flattened. There is characteristic cervico-dorsal kyphosis. There is sometimes dullness over the sternum due to persistence of the thymus. The pelvis may be enlarged, but the hip and leg bones are generally spared. The hands and feet undergo enormous hypertrophy (Fig. 252). The following are some of the measure- ments in the case that has come under my observation, reported by Adler, and in cases reported by Osborne and Packard: Fig. 250. — Frohhch syndrome. Tumor of hypophysis in boy of twelve. Adiposity, [defective development of genitals; choked discs, headache, nystagmus. Length of hand 7 . 6 to 8^ inches. Length of foot 11.7 to 123^^ inches. Cranial circumference 24 to 263''7 inches. Circumference of thorax 44 inches. The whole body may take part in the growth and some giants have acromegaly, if the disease begins quite early in life. There may be vasomotor and cardiac disturbances. Headache, and symptoms of brain tumor may develop owing to the enlarging pituitary body. The patient's mind usually becomes dull and apathetic. Disorders of metabolism occur with bulimia, and glycosuria. The vision is sometimes impaired and there may be temporal hemianopsia due to the pressure of the enlarged pituitary 580 DISEASES OF THE NERVOUS SYSTEM bodj^ on the optic chiasm. Ocular palsies may occur. The muscles may be at first hypertrophied. later atrophied. There are no paralyses and rarely any anaesthesise. The disease runs a very chronic course, lasting ten or twenty years. Pathology. — There has been found an enlargement of the pituitary body in nearly all cases, and it is probable that the disease is to be regarded as a perversion of nutrition accompanied by hypertrophy and defective function of the pituitary gland. Fig. 251. — Acromegaly. If pituitary disease begins before the epiphyses of the long bones are united there may be giantism; if it begins later, acromegaly. The diagnosis must be made from congenital enlargements, from so-called giant growth which affects single members, and from osteitis deformans. In the latter disease it is the shafts of the long bones and the cranium, not the face, which are involved. Prognosis. — Acromegaly is incurable, but it has been arrested, or at least has ceased to progress, and it may not greatly shorten life. Treatment. — Cases have been reported in which iodide of potassium and arsenic TKOPHIC AND VASOMOTOR DISORDERS 581 have arrested the disease. In general, the treatment is only symptomatic, but feeding with pituitary gland has been tried, and it sometimes does good. Removal of a portion of the gland by the surgeon may be required on account of the development of neigh- borhood symptoms. The use of X-ray and of radium is now being tested. MYXCEDEMA Myxoedema is a disease of the thyroid gland, but its sjaiiptoms are so largely nervous and mental that a brief description of it is justified here. It is a chronic disorder, due, as a rule, to an interstitial thyroiditis, and characterized by a solid oedema of the subcutaneous tissue, dry skin, loss of hair, subnormal temperature, mental dullness, and even Fig. 252. — Hand in acromegaly. insanity and idiocy. It has two forms — the congenital and infantile — causing a condition known as cretinism ; and an adult form constituting myxoedema proper. It occurs most often between the ages of thirty and fifty, and oftener in women (seven to one). It is seen oftenest in temperate climates. Hereditary influence, alcoholism, and syphilis are not predisposing factors; lead poisoning may be a cause. It begins slowly. The patient is languid and dull, and is unusually sensitive to cold. Voluntary movements are slow; the weight increases and a solid oedema which does not dent on pressure develops in the 582 DISEASES OF THE NEEVOUS SYSTEM face and extremities. The skin gets dry and rough, the hair begins to fall, the temperature is subnormal, 1° to 2°F. Mentally the patient is dull, depressed, and in one-fifth of the cases melancholia, mania, or dementia develop. The muscles are weak, the gait is slow, the voice hoarse and monotonous. There is considerable anaemia and the heart is weak. The pulse is slow and the arterial tension low. Albuminuria is present in 20 per cent, of cases, and hemorrhages may occur. The pallor, oedema, loss of hair, and mental hebetude give to the face a characteristic expression (Fig. 253). The disease may run a course of six or seven years, the patient dying of cardiac weakness or some intercurrent malady. riG.'^253. — A case of myxoedema in a man aged forty-four years. {Murray.) The disorder is due in most cases to a chronic interstitial thyroiditis which ^usually causes atrophy of the gland. It may be produced by artificial removal of the thyroid. The result of this is a defective action of the thyroid, and a consequent poisoning of the system and deposit of mucin in the subcutaneous tissue especially. The diagnosis is based upon the peculiar physiognomy due to the oedema, the loss of hair, subnormal temperature, mental hebetude, and atrophied thyroid. The prognosis is good if treatment is instituted early. TROPHIC AND VASOMOTOR DISORDERS 583 The treatment consists in the administration of the thyroid ex- tract in daily doses ranging from five to forty grains or even more. The results are most brihiant, and humanity owes much to Dr. George R. Murray, who first instituted it. CRETINISM Cretinism is a form of myxoedema due to atrophy, or defective functioning of the thyroid gland, occurring congenitally or during infantile life. The disease occurs endemically in parts of Europe, but only sporadically and with great rarity in America. Hereditary and family influences are at work in endemic but not in sporadic cretin- ism. It develops either directly after or in the first three years of life, and shows itself in a stunted growth both of brain and body, most cretins being idiotic dwarfs. The general symptoms are much Fig. 254. — A cretin dwarf, aged twenty. (Leszynsky.) like those of myxoedema in adults plus the retarded growth of mind and body. The deposits of solid oedema cause peculiar deformities and lead to a characteristic physiognomy. The mind is dull and placid, the muscles are weak, the abdomen is protuberant, the hands and feet are broad and thick; the patients are anaemic, the temperature is subnormal. The arrested bodily growth is such that on reaching adult age the stature may be only twenty-eight to thirty-three inches. Cretins usually die young, but some survive to the age of thirty and forty. The treatment is the same as that of myxoedema. Here, too, if the case is seen before adolescence, brilliant results can be obtained. ANGIO-NEUROTIC (EDEMA (Circumscribed (Edema; Quincke's Disease, (Edema Fugax) Angio-neurotic oedema is a disorder characterized by the rather rapid appearance of circumscribed and temporary swellings of the skin and 584 DISEASES OF THE NERVOUS SYSTEM subcutaneous tissue, and of the mucous membrane. The swellmgs appear upon different, generally exposed parts of the body. The mucous membranes, especially those of the respiratory and gastro-intestinal tracts may be involved at the same time or independently. The disease occurs oftenest in early adult life, the average age being from twenty to thirty, but it has been observed in young children and even in the aged. It occurs oftener in females than in males. Heredi- tary influence plays a part in some cases; and the disease sometimes runs in families. It occurs in neuropathic individuals and families. It is associated with other paroxysmal neuroses such as migraine, urticaria, certain forms of asthma, oedematous purpura and eczema. It occurs oftener in winter and oftener in the early morning hours. Exhausting occupations predispose to it. The exciting causes are sudden exposure to cold, slight traumatisms, fright, anxiety, grief, and the ingestion of certain kinds of food, such as apples, strawberries, or fish. A peculiar form of this oedema seems to develop in connection with menstruation. Symptoms. — The disease appears without much if any warning. In a few minutes or hours there develops a circumscribed swelling upon the face or arms or hands. This swelling varies in diameter from one- half inch to two or three inches. It may be dark reddish or rosy or it may be pale and waxy. It does not easily pit on pressure. There is sometimes a rise, sometimes a fall in local temperature. It is accom- panied by sensations of tension and stiffness, scalding, burning, and sometimes itching, but there is no actual pain. The swelling is usually single, but it may be multiple. It is located most often upon the face, next upon the extremities, particularly the hands; next on the body, then in the larynx and throat, and then on the genitals. The swellings last from a few hours to two or three days. Between the attacks the patient feels well. They are apt to return at intervals of three or four weeks to several months. Sometimes they are brought out only by cer- tain peculiar exciting causes, such as indigestion or mental anxieties or emotional disturbances. When the disease attacks the larynx or throat, serious symptoms of dyspnoea and suffocation may appear; surgical interference may even be called for, and death has been known to result. The oedema may attack the stomach, producing nausea, vomiting, and great gastro-intestinal distress, or the intestines causing colic and diarrhoea. The pathology of this neurosis is imperfectly known but is some- what as follows: The disease is associated with and is allied to a group of neuroses or angio-neuroses including migraine, urticaria, certain forms of asthma and purpura. TROPHIC AND VASOMOTOR DISORDERS 585 The victim possesses a constitutional defect which is sometimes inherited and transmitted. This defect may be a dysthyroidism, or other disorder of the endocrine cells. It shows itself in weakness in the chemistry and cellular functions of the gastro-intestinal tract, allowing entrance into the blood of heterogeneous proteins (anaphylaxis). The incident of an attack of oedema (anaphylactic shock) is brought about by depressing emotions, trauma, shock, exposure, certain foods ingested. Then the heterogeneous proteid in the system attacks the vasomotor nerves at certain points, because there is an irritant or extra-point of weakness at these points. There follows an excessive permeability of the vascular endothelium, and transudate of serum. Diagnosis. — The symptoms of the typical disease are so peculiar that they are easily recognized. The spontaneous appearance of the oedema, its recurrence at certain intervals, and the absence of serious pain and evidences of inflammation are sufficient usually to enable us to recognize it. Giant urticaria is a disease resembling neurotic oedema and prob- ably closely allied to it. The blue and the white oedema of hysterics only exists with other stigmata of hysteria. The prognosis, so far as cure is concerned, is not very good. The attacks, however, can be ameliorated, and the disease itself is not serious, as regards life except when it attacks the larynx. Most of the patients whom I have seen have eventually recovered. The treatment consists in the adoption of such measures as will give tone and stability to the nervous system, and the gastro-intestinal tract. In some cases great attention must be paid to diet. Internally, mineral acids and strychnine may be of some value. A systematic and pro- longed use of arsenic has been followed by cure; urotropin has been used with success, and so also has nitroglycerin, and horse-serum (in a case associated with purpura). The lactate of calcium 5i daily is sometimes helpful. Gastro-intestinal crises may require morphine. In laryngeal oedema, intubation should be promptly done. , CHAPTER XXIX THE DISORDERS OF SLEEP INSOMNIA, HYPNOTISM, MORBID SOMNOLENCE, CATALEPSY, TRANCE, LETHARGY, THE SLEEPING SICKNESS The most conspicuous phenomenon of sleep is the loss or partial loss of consciousness. With this the regulating mechanism of the mind is in abeyance. Mental processes go on, when at all, by free associations or in accordance with the emotional trend or as activated by sensory and somatic irritants. The cause of sleep is not exactly known. We know that sleep is a normal resting phase of brain action, a kind of cerebral diastole. It is probable that the brain neurons by their activity throw off products of metabolism which tend to benumb and inhibit their waking physiological functioning. It has been found that the brain is slightly anaemic during sleep, but this is part of the process not the cause of it. Other organs of the body rest somewhat also during sleep, and especially is this true of the muscular system. There are slight changes in pulse, respiration, blood-pressure and other bodily functions in sleep but these are described adequately in physiological text-books. Normal sleep varies much in accordance with age, sex, the individual, and, to a slight extent, with occupation, race and climate. The infant sleeps at first nearly all the time, later fourteen to sixteen hours, but its sleep is light. Young children sleep ten to twelve hours. The adult needs about eight hours, while the aged live healthfully with but six. Women need half an hour or an hour more than men. A few persons, generally men, need nine, ten, or even twelve hours' sleep daily; others require only six. Brain workers, as a class, take less sleep than laborers. Sleep is sounder and longer in cold climates and among northern races. The disorders and perversions of sleep include insomnia, morbid somnolence, morbid dreaming, sleep-walking, hypnotic and trance states. They are disarrangements of a normal function, brought about by various causes. Sleep itself may be the morbid expression of a disease, as in narcolepsy, the sleeping sickness of Africa, and the somnolence of the senile brain. Conditions allied to normal sleep exist in the hypnotic and trance states. Among all these conditions, practically, insomnia is the sleep disorder which most annoys humanity, and it comes next to food and raiment and human love in the concern of civilized man. 586 THE DISORDERS OF SLEEP 587 INSOMNIA Insomnia is a condition in which persons suffer from insufficient and disturbed or unrestful sleep. The term "insomnia" covers all these deficiencies, but the old Roman writers used the word exsovinia for a condition of excited wakefulness, such as came with morbid mental exaltation and the delirium of the Bacchic priestess. Insomnia is a symptom of many disordered physical and mental con- ditions, and it has been considered elsewhere in connection with the most important of these. It occurs in its most serious and persistent forms in painful diseases, in the psycho-neuroses, and in manic and depressed states. Here one sometimes finds cases of apparent entire absence of sleep for prolonged periods. This ahypnosis is not, however, absolute. Man can exist without sleep for about the same time that he can go with- out food, viz., 3 or 4 weeks, but he cannot live without it. Etiology. — Insomnia of mild grades occurs often apart from any very serious physical disturbance. It may be a hereditary or family trait not to sleep easily or well. More often it is acquired by bad habits of life, by disturbing environments, by anxiety of mind and by excessive use of drugs, tobacco, tea and coffee. It may be the result of endogenous irritants, such develop in cases of poor digestion, in faulty metabolism or in hyperthyroidism. Disease of the heart and arteries may lead to insomnia, and under this head come the cases which occur in Bright's disease with sclerotic arteries and anaemic brains. Disorders of the stomach lead to disturbed sleep oftener than to complete insomnia, and the liver, when inactive, causes somnolence rather than the contrary. Insomnia is a disorder especially of the active periods of life, though children suffer from it also. In their case it is usually due to some definite physical cause, such as pain, indigestion, emotional excitement; yet one not infrequently meets children who from early life go to sleep with difficulty. It is in this period of life that the active and bizarre disorders of sleep occur, such as sleep-walking, night-mare and pavor nocturnus. The aged have insomnia but do not suffer so much from it. Women have insomnia rather less than men; and brain workers more than muscle workers. Insomnia is more common in cold climates, largely because there is more brain work done there. High altitudes tend to cause insomnia, at least, till the system becomes accustomed to the change. Anxiety of mind and brain fatigue lead to insomnia; and very often to the morning type. The patient who carries a worry wakes at 3 or 4 A.M. instead of his usual hour. Insomnia is a warning symptom of the approach of neurasthenia and of certain mental disorders, especially melancholia and the obsessive psychoses. 588 DISEASES OF THE NERVOUS SYSTEM Summary: Insomnia is due to 1. Hereditary and constitutional conditioiis. 2. Habits of life: of work, of eating, of environment and of too violent exercise. 3. Irritative conditions such as pain, pruritus, indigestion. 4. Toxic agents, tobacco, tea, coffee, cocaine, morphine. 5. Endogenous toxins, gastro-intestinal, glandular.' 6. Vascular degenerative disease of the brain. 7. Psychic causes; fatigue, worry, the onset of psychoses. Treatment. — -The enumeration of the causes of insomnia, covers a large part of the discussion on treatment. For treatment involves a consideration of the cause, then the laying down of rules of hj^giene, the arrangement of daily work and play, the times of eating, diet, exercise, the hours of retiring and the amount of sleep needed. Nearly all these points have been discussed under special chapters. I shall discuss now only certain particular medicines and measures used for the symptom insomnia. The older physicians in treating sleeplessness, used to depend largely on hyoscyamus, camphor, opium, and the fetid drugs, such as asafcetida, musk, and valerian. Hyoscyamus is still used. The hydrobromate of hyoscine, in doses of gr. 3^foo to gr. i^^q or more, is the best form. Hyoscine is indicated in the insomnia of delirium and mania which is accompanied by motor activity. Chloral hydrate still holds its own as one of the surest of hypnotics. The dangers involved in its use have been somewhat exaggerated, though thej^ are sufficiently real. Doses of gr. X. and gr. xv. are often quite large enough, but in alcoholic insomnia it may be given in twice the above amounts, guarded with ammonia and digitalis. Bromide is one of the best hypnotics but it has to be skilfully used. The immediate effect is simply sedative, and sleep is not produced unless large doses are given. Some persons are even kept awake by average doses (gr. xv. to xx.). In insomnia, therefore, bromides are best pre- scribed in doses of gr. xxx. twice in the evening for not over four days. By the third evening sleep is generally secured. A bromide habit is rarely formed, and is in itself not seriously injurious. The bromides alone are hardly strong enough hypnotics for the insomnia of delirium and mania and they are not so efficient in the weak and aged. Paraldehyde ranks close to chloral in its value as a hypnotic. In some persons it disturbs the stomach, but not in all, and it may be used as a hypnotic for months without its power being impaired. It is a disagreeable drug, and there is nothing so far as I know, that palliates its offensiveness. I prefer, however, to prescribe it in 5i- doses poured upon a teaspoonful of pow- THE DISORDERS OF SLEEP 589 dered sugar. The paralydehyde habit may be formed and it is bad Hke all drug habits, but less serious than chloral or opium addiction. Amylene hydrate is a hypnotic of properties similar to those of paraldehyde, but less disagreeable. It is given in doses of about one drachm. Lupulin in large doses, gr. x. to gr. xx. is a fairly good hyp- notic. Chloralamide is more agreeable than chloral, though it acts practically in the same way. Sulphonal, trional, veronal, medinal and adalin, all act in much the same way; but vary in strength and quickness of absorption and elimination. Among the antispasmodics are several drugs which occasionally answer well in the insomnia due to nervous irritability. For example, tincture of valerian and spirits of lavender are sometimes adequate as sleep inducers. Ten grains of aspirin act sometimes as a harmless hypnotic. A bottle of beer, or a drink of whiskey are hypnotics but are dangerous medicines, for their effects wear off and larger doses are soon demanded. Safer measures are the taking of digestive food just before retiring or on too early waking. A glass of hot milk or hot malted milk often works successfully. Besides drugs, there are many hygienic or mechanical measures to which the physician may successfully resort — -listening to monoto- nous noises, reading dull or heavy books, counting, or keeping before the fancy some blank or wearying picture — ■ "A flock of sheep that leisurely pass by One after one; the sound of rain and bees Murmuring; the fall of rivers, winds and seas, Smooth fields, white sheets of water, and pure skies." In cases of purely bad habit insomnia, psycho-therapeutics even to the point of actual hypnosis may be tried. Mechanical remedies have nearly all for their purpose the with- drawal of the blood from the brain to the skin and abdominal viscera. Hot footbaths or warm general baths, cold douches down the spine, abdominal compresses, beating the limbs with rubber hammers, massage, all are at times efficient hypnotics. Persons who suffer from insomnia should sleep in cold rooms, the head should not be too high or very low, and in most cases they are better without late suppers, even though these be light. Mental work should be laid aside several hours before retiring and the evening devoted to quiet conversation and reading or amusements that do not actively excite the nerves. Many persons live in good health though they sleep in the day and stay awake at night. Despite this, it is true that the best time for sleep is at night, and that the old maxim, "Early to bed," is a sound one. Yet it is not the early bird that gets the Avorm so much as the bird that has slept enough. The human system 590 DISEASES OF THE NERVOUS SYSTEM requires a certain amount of sleep and should have it. The industrious and ambitious often try to train themselves to shorter hours, but though they may succeed for a time, nature will not be cheated out of her due and health suffers in the end. It is a widespread custom in some countries to take a short nap in the daytime, and the custom is a good one, for the weak and aged and over-worked. In early life, at least, a habit of going to sleep when desired can be formed by careful attention and exercise of the will. It is a most useful asset throughout life. PERVERSIONS AND DISTURBANCES OF SLEEP Sleep is said to reach its deepest stage in from one to two hours after it begins. There is then after this a gradual lessening of the depth of sleep. Probably there are great variations in this rule, for many persons seem in soundest slumber several hours after falling asleep. But, at any rate, there are lighter stages of sleep at its inception and toward its end. These are the favorite times for dreams, and at this period also there develop the peculiar phenomena of sleep-drunkenness. Dreams. — When sleep is perfect and profound, dreams afterward re- membered do not occur. Dreaming is, therefore, a morbid symptom, although often of trivial significance, especially if it occurs at about the time of natural waking, when slumber is, in its physiological course, passing into the lighter stages. In sleep, no matter how light, the action of the regulating centre, which directs thought, controls einotion, and exhibits itself in volition, is suspended; the physical mechanism, if excited to action at all, works without purpose, like a rudderless ship at sea. Ideas and emotions succeed each other by the laws of associa- tion, but are not properly correlated, and judgment and logical reasoning are gone. As a rule, dreams are made up of somewhat ordinary ideas and fancies incoherently associated, and shifting too rapidly to call up much feeling. AVhen from some point in the body painful sensory ex- citations do produce disagreeable images, emotions of a most violent kind may be felt. The Freudian interpretation of the dream as expressing symbolically or otherwise a wish is one that does not concern the clinician unless he believes in the value of psycho-analysis. Nightmare is a disorder nearly always symptomatic of an irritation in some part of the body. The usual causes of it are some digestive disturbance (repletion) and cardiac disease. Persons of a nervous tem- perament are more subject to it; and there are individuals who suffer from it all their lives. The popular belief that sleeping on the back favors it is, in general, a correct one. When nightmare occurs in cardiac disease THE DISORDEKS OF SLEEP 591 a certain position, semirecumbent or on the right side, must be main- tained, or the painful fancies will awaken the patient. Healthy people can get sound sleep whether lying upon the back, the side, or the stomach ; but light sleepers, and those with sensitive abdominal viscera, generally find that the position on the right side is the most comfortable and less provocative of unpleasant dreams. Prolonged mental or physical strain, excitement, and worry, predispose to nightmare. A high proteid diet, excess of sweets in diet, excessive use of strong liquors, coffee and tobacco, have a similar tendency. Pavor nocturnus, or night-terrors, is a sleep disorder peculiar to children. It is allied to nightmare on the one hand and sleep-drunken- ness on the other. It differs from the former condition in that the child continues to suffer from the distressing fancies for some time after he is awake. Night-terrors occur usually one or two hours after sleep has begun. The child wakes up screaming with fright, and per- haps runs about the room or seeks its parents for protection against some imagined harm. The disorder occurs in weakly, anaemic, nervous, or rheumatic children. It is due sometimes to what has been termed lithsemia or, as the older writers put it, rheumatism or gout of the brain. Digestive disturbances, worms, dentition, hereditary syphilis, mental strain, fright, and excitement are placed among the causes. It sometimes appears to be a paroxymal neurosis allied to epilepsy and some cases are really epileptic. The disorder is usually harmless and the prognosis favorable. Somnolentia, or sleep-drunkenness, is a condition of incomplete sleep in which a part of the faculties is abnormally excited while the other is buried in repose. It is a kind of acted nightmare or somnambulic delirium. The person affected, is incoherent, excited, and often violent. He experiences the delusion of some impending danger, and while under it acts of violence have been committed. A patient of mine was in the habit of waking and attacking his beloved wife. The condition is one of medico-legal importance, therefore, and has been discussed by writers on that science (Wharton and Stille). Minor degrees of it are often noticed in children and in adults who are roused from a very profound sleep. It at times becomes a habit and a most annoying or dangerous one. The disorder in its severe form- is fortunately very rare. The treatment of morbid dreams, nightmare, and pavor nocturnus must be directed to a removal of the causes. Tonics, cardiac stimu- lants, laxatives, antirheumatics, attention to diet and time of eating are especially important. Change in surroundings is often necessary. Among symptomatic remedies the bromides are the best, combined sometimes with alkalies and salicylates. In somnolentia the patient should be prevented from getting into too profound sleep. He may be 592 DISEASES OF THE NEEVOUS SYSTEM awakened once or twice during the night, or take a nap in the day- time. The head in sleeping should be raised and the body not too heavily covered. Somnambulism. — Somnambulism is a condition similar to hypno- tism or the mesmeric state. In it volition is abolished and the mind acts automatically under the dominance of some single idea. It is an acted dream. Sight, hearing, and nearly all the avenues of sense are closed. The sleepwalker avoids obstacles and performs ordinary acts automatically like an absent-minded man, which in reality he is. All those mechanisms which have been trained by constant repetition to act automatically like that which preserves equilibrium are active, and their powers may even be heightened, so that the somnambulist may walk along roofs or on dangerous roads and thread intricate passages without harm. The automatism of the somnambulist may continue for hours, until a journey has been performed or a task completed. He may carry out with success familiar mathematical calculations, write a letter, or work upon a picture, but he only follows along the lines established by constant iteration in his waking moments. He can originate nothing new. He is roused from his state with difficulty, and when out of it he re- members nothing of what has occurred. Somnambulism occurs oftener in neuropathic persons and it may be hereditary. Its exciting cause is often over-eating, or mental strain and excitement. Sleeping with the head too low is another cause. The habit being once established, however, attacks occur without apparent cause. The disorder occurs oftenest in young people about the age of puberty, and it then attacks the sexes alike. Later in life women are more often affected. The disease is fostered sometimes at school by the at- tentions of the schoolmates. The attacks are likely, after a time, to become periodical, occurring every week, fortnight, or month. The som- nambulic state may come upon a person in the daytime. It is then re- garded as spontaneous trance, or hypnotism. It is not the case, however, that persons who are easily hypnotized are usually somnambulists, though the reverse may be true. Somnambulism is a term that should include not only sleep walking, but sleep talking. In the treatment of somnambulism the patient's surroundings must be investigated, and unfavorable influences, such as excessive fatigue and excitement and such as may occur at school or from injudicious nurses, be removed. He should be prevented from sleeping too soundly, the head should be raised, the clothing light, the diet regulated. Remedies like iron, quinine, phosphorus, and cod-liver oil may be given. When the patient is discovered in the somnambulistic state he should not be awakened, or at least not until he is safely back in bed. THE DISORDERS OF SLEEP 593 HYPNOTISM, TRANCE, MESMERISM Major hypnotism is a morbid mental state artificially produced and characterized by (1) perversion or suspension of normal consciousness; (2) abeyance of volition; (3) a condition of suggestibility leading the patient to yield readily to commands or external sense impressions ; and (4) intense narrowing and concentration of the attention upon some idea or feeling suggested by the hypnotizer. Minor hypnotism is a state closely bordering on normal sleep in which there is a dulling of consciousness and a condition of suggesti- bility and narrowing of the attention field. ^ The proportion of persons of all ages found by Beannis to be hyp- notizable was about eighteen or twenty per hundred. Children up to the age of fourteen are very susceptible. After the age of fifty-five susceptibility lessens. Men are almost as easily affected as women; but persons of a docile mind and those trained to some degree of mental discipline and capacity for submission, such as soldiers and artisans, are more sensitive. In this country the percentage of hypnotizable sub- jects is less than it is in Europe. Hysterical and insane persons are not very susceptible. Those who have been mesmerized once are more easily affected afterward, and may even pass into the state involuntarily. Methods. — -There are two ways of inducing hypnotism: the fixation method and the suggestive method. The former and older plan, de- vised by Braid, is to make the patient fix his eyes for five to ten minutes on some bright object at a distance of six or eight inches from the eyes and a little above the horizontal plane of vision. Practically, it is some- times the custom among practising hypnotizers to give the patient a dose of paraldehyde. In the ''suggestive method" devised by Liebault and Bernheim the subject is placed in a chair in front of the operator. The operator then talks to the subject in a firm and confident voice, assuring him that he will go to sleep in a short time, telling him to make no resist- ance, that his sleeping will be natural, that nothing will be done to worry or fatigue him, that he will dream pleasant dreams, that he will wake up feeling better; then that he is feeling drowsy, his eyes are heavy, objects look confused, the lids are falling, they are closed — ^in a moment more the patient goes off to sleep. This requires some little time — five to fifteen minutes. It may fail the first time and succeed the second. Hypnotic states can be self-induced by vigorously fixing the atten- tion upon some object. The ecstatic states of the saints are forms of "•The definitions of hypnotism vary with the psychologists and the time of year. The above is only a descriptive definition with no reference to dissociations of personality or the thresholds of consciousness. 1 may add that the word hypnotism is used in two senses. It indicates a mental state and a general topic. 38 594 DISEASES OF THE NERVOUS SYSTEM hypnotism; so also are the trance states in which some clairvoyants and spiritualistic preachers place themselves; this same curious phenomenon is at the bottom of the "mind-healing" sciences, and it enters into rational therapeutics and orthodox religion. The capacity of the human mind for hypnotism or semihypnotic states is, therefore, a most curious and important fact. Symptoms. — The person who has been hypnotized at first sits or lies quietly in the position he has assumed during the manipulations of the operator. No notable physiological changes occur, as, for example, in the pulse, respiration, temperature, pupils, skin, etc. Some increase in the cerebral blood-supply, however, is said to be present. The patient will now respond automatically to any outside command or will be dom- inated by any idea which is suggested to him. He will talk, or walk, or run, or gesticulate, assume expressions of fright, anger, or joy, entirely in accordance with the command given. Apart from these commands, he is entirely dead to the outside world. He hears, sees, smells, tastes, and feels nothing. He can be burned, cut, or injured without showing any signs of feeling. At a suggestion he may be made cataleptic, somnambulic, or paralytic. This state is termed somnmnbulistic trance. If left to himself, he gradually sinks into a deep sleep, from which he can with difficulty be roused. After a time, rarely more than one or two hours, he awakes as from ordinary slumber. This latter state is called trance coma, or lethargic hypnotism. The phenomena of hypnotism depend upon the wonderful sensi- tiveness and quickness of the subject in responding involuntarily, with all his nervous energy, to outside suggestion. Dishonest persons may learn the latter trick and thus simulate the hypnotic state. Traveling mesmerizers utilize such persons largely; hence no confidence can be placed in the phenomena exhibited by them. Minor hypnotism is produced by the ''suggestive method" of hyp- notizing. By this latter plan patients are thrown into various degrees of the hypnotic state from slight drowsiness to lethargy, but they are not somnambulic, and do not become cataleptic or ansesthetic. Patients naturally come out of the mesmeric state through the channel of deep sleep or lethargy. Ordinarily they are dehypnotized by word of command, or by a pass of the hand, or any impression which the patient expects to be used for the purpose. Hypnotized persons have been observed to have a diminution in the spinal reflexes and a muscular hyperexcitability. They sometimes show a most extraordinary exaltation of visual, auditory, or other special sense. Therapeutics. — The practice of using major hypnotization is usually injurious, if repeated much, tending to exhaust the nervous force and weaken the will. It should be done only with the greatest care. Its THE DISORDERS OF SLEEP 595 utility in therapeutics I greatly doubt. It may relieve symptoms in the hysterical for a time, but it cannot be of permanent benefit and is likely to lead to actual harm. The induction of minor hypnotic states by suggestion is not harmful if carefully and moderately employed. Its practical results, however, are not great. It has its value in pedagogy, among children, in defec- tives, obsessives, and in morbid habits. The general popularization of hypnotism by means of mind cures. Christian science, etc., accomplishes its results at the expense of mental demoralization, and faith-healing institutes may be pernicious elements in society. MORBID DROWSINESS AND SOMNOLENCE This is a common symptom, which may be due to one or more of he following causes: 1. Old age when there is a weakened heart and cerebral arterial sclerosis. 2. Cerebral syphilis. 3. Endogenous toxae- mias such as result from constipation, gastric repletion, atonic intestines, and the metabolic disturbances in obesity. 4. Concussion of the brain. 5. Disorder of the pituitary or thyroid glands. 6. Climatic conditions such as extreme cold. 7. Constitutional defects. A very common cause of drowsiness is dyspepsia attended by some torpidity of the liver, the condition popularly known as "biliousness." Syphilis is more likely to cause insomnia, but in its parenchymatous form somnolent conditions may be produced which are of serious significance. Drowsiness occurs from the effects of severe cold. It sometimes develops when persons change their surroundings, especially on going to the sea- shore, for low levels and a high degree of atmospheric pressure seem to promote sleep. The drowsy state that sometimes follows concussion of the brain is a familiar phenomenon. Some persons, no doubt, acquire the habit of drowsiness. At first the trouble may have been induced by indigestion, "biliousness," or malarial infection, but it persists after the cause is removed. Such persons can hardly sit through a lecture, a church service, or any exercise requiring quiet and attention. Morbid somno- lence is sometimes produced by disordered activity of the pituitary and thyroid glands. The patients are unable to keep awake except when actively engaged in work. Sitting at a table or in the chair they fall asleep and sleep so profoundly that they fall to the floor. Morbidly Deep Sleep. — Certain persons, when they sleep, pass into an almost lethargic slumber. Persons who suffer in this way often sleep a longer time than normal. They are awakened with difficulty and then suffer with headache or disagreeable sensations throughout the day. Instances in which persons retire at the usual hour, but can with great difficulty be roused in time for the ordinary duties of the day, are not 596 DISEASES OF THE NERVOUS SYSTEM rare. Some of these are illustrations of the vice of indolence, but in other cases there is an absolute need of nine, ten, or even fourteen hours of sleep. This disorder of sleep is most liable to occur in the young and in those of nervous temperament. It often seems to be a constitutional condi- tion, for which nothing can be done. In other cases it results from over-feeding and indolent habits. Paroxysmal Sleep, Narcolepsy, Sleep Epilepsy. — It sometimes hap- pens that persons suffer from sudden attacks of unconquerable drowsi- ness; they fall off into slumber despite every effort of the will. These are more than drowsy sensations, for sleep, or a state resembling it, cannot be kept off. Some of these cases are of a purely nervous character, i.e., the trouble is not due to a toxsemia or to organic disease, but to a parox- ysmal change in the nervous centres of a vascular or chemical character, causing sleep. It may be that the patient is epileptic and sleep seizure takes the place of the ordinary epileptic spasms. Fig. 255. Cases of epileptic sleep, or narcolepsy, and allied forms are not of frequent occurrence. Females are rather more often affected than males, and the susceptible age is from fifteen to forty. The disorder is brought on sometimes by fright, over-strain, and humoral poisons acting on a pre- disposed nervous system. The course is chronic and relief is not always obtained. It should be remembered that syphilis, malaria, or ansemia, and indigestion may be elements in the trouble which are important, if not fundamental. Bro- mides in small doses are often useful factors in treatment. Change of occupation, of mode of life, or of climate may be essential to a cure. Catalepsy, Trance.^ — Most of the so-called cases of prolonged sleep, lasting for days or weeks, are cases of spontaneously developed mes- meric sleep in hysterical women, or cases of incipient insanity (dementia THE DISORDEES OF SLEEP 597 prsecox or stuporous melancholia). The phenomena in these cases may take the form of catalepsy, with waxy rigidity of the limbs, or of a trance state. In cataleptic states the limbs may be placed in various posi- tions and will remain there for several minutes (Fig. 255). In lethargy or trance states the patient may be plunged into a deep and prolonged unconsciousness, lasting from one day to several years. These are the ''sleeping girls" of the newspapers. Others are persons of a too ready susceptibility to mesmeric suggestion, who get into a morbid habit of going into mesmeric sleep spontaneously. The duration of the attacks of trance lethargy is from a few hours to ten years. Ordinarily, however, profound trance sleep lasts not more than a few days, while those cases in which the sleep is from mes- meric suggestion lasts but a few hours. The katatonic patients after a few weeks or months gradually awake, become excited, and then pass into a condition of dementia or into cata- lepsy again. Morbid Sleep from Organic Disease. — Prolonged and excessive sleep occurs as the result of syphilis of the brain, brain tumors, and the degen- erative changes in old age and insanity. It has been noted especially in tumors of the basal ganglia and third ventricle. Organic diseases of the brain tend to produce conditions of mental weakness, hebetude, or comatose states, rather than anything allied to sleep. Accidents of Sleep. — Owing to the fact that sleep is a resting state of the organism, and that many of its functions are lowered, or their cerebral control lessened, peculiar crises, or physiological and pathological disturbances of nervous equilibrium, occur. Attacks of gout, of asthma, and of pulmonary hemorrhage are most liable to occur during the early morning hours. Deaths and suicides occur oftener in the forenoon, but births oftener at night. Epileptic and eclamptic attacks occur with much frequency at night. Involuntary emissions of spermatic fluid, or gastric crises, and incontinence of urine are among the pathological inci- dents of sleep. Praedormitial Shocks. — Sudden attacks of starting of the whole body, shock-like in character, accompanied by peculiar feeling in the head or occiput, not infrequently attack persons as they are dropping off to sleep. They indicate fatigue and are of slight significance. THE SLEEPING SICKNESS OF AFRICA The sleeping sickness of Africa is a disease, caused by a species of flagellated protozoa, belonging to the genus Trypanosoma. This para- site multiplies in the blood, and causes a slowly developing malady char- 598 DISEASES OF THE NERVOUS SYSTEM acterized by somnolence, mental apathy, paralysis, and finally death. The Trypanosome which causes this sleeping sickness is the T. Gainhiense, first described by Dr. J. E. Dutton. Etiology. — -The disease prevails mostly along the west coast of Africa, but it has extended, thence, to other parts of that continent, particu- larly equatorial Africa. It was formerly confined to the negroes, but recently Europeans have suffered from it. It affects particularly natives who live upon the shore of the rivers and lakes. The Trypanosome is a worm-like parasite which is found among the red blood-cells (Fig. 256). They are not very numerous, and several microscopical examinations may be needed to find them. In late stages of the disease, the parasite is found in the cerebrospinal fluid, while early in the stage it is found in the lymphatic glands and channels. The common way of the propaga- FiG. 256. — The blood in sleeping sickness, showing spirillum. tion of the Trypanosome is through the tsetse-fly, and it is conveyed back to man by the bites of these flies. Symptoms. — ^It is stated by Dr. David Bruce that after the Trypano- some has infected the body, it may be from three months to three years before it gets into the cerebrospinal fluid, and begins to produce definite symptoms. During this stage of incubation, there is some enlargement of the lymphatic glands, and sometimes a fever of irregular type. When the disease begins to affect the patient, he becomes apathetic and dis- inclined to exert himself. Headache and indefinite pains are complained of. The pulse is rapid and weak. The cervical and other lymphatic THE DISOEDERS OF SLEEP 599 glands are enlarged, the gait is unsteady and shuffling, the muscular powers diminished, the voice is weak, and tremors of the tongue are noted. The symptoms increase until after weeks or months the patient is confined to his bed, lying in a somnolent condition. The patient then begins to emaciate, becomes semicomatose, and in two or three weeks the temperature falls and death occurs. Pathology. — Dr. F. V. Mott has shown that there is a characteristic appearance in sections of the brain, which is found in no other disease. This is a condition of meningo-encephalo myelitis, which affects especially the medulla and the base of the brain. The inflammation can be traced along the blood-vessels into the substance of the nervous system, the perivascular lymphatics being crowded with lymphocytes. Modern trypanosomiasis is, however, according to Bruce, a disease of the lym- phatic system, though its characteristic symptoms are shown upon the nerves and muscular system. Diagnosis. — The diagnosis is difficult in the early stage of the disease. Later, the examination of the blood and lymphatics, and the peculiar progressive weakness of the body, tremor of the muscles, unsteady gait and somnolence are characteristic. Pro gno sis. ^^E very case of the disease dies sooner or later, according to Dr. Bruce. Treatment. — Up to the present time the most effective drug has been some preparation of arsenic. The prophylactic measure for the sleep- ing sickness is to kill off the tsetse-fly. CHAPTER XXX CRANIO -CEREBRAL TOPOGRAPHY The object of cranio-cerebral topography is to map out upon the scalp the underlying fissures, convolutions, and other parts of the brain. Practically, the principal points to be determined are the position of the longitudinal, Rolandic, Sylvian, and parieto-occipital fissures and the lower outline of the brain. The measurements are based chiefly upon the known relations of certain landmarks on the skull to the parts beneath. These landmarks are the glabella, bregma, lambda, stephanion, asterion, and pterion, which are points at the junction of the various sutures with each other and with certain ridges or protuberances. Their position is shown in the cut (Fig. 252) except that of the glabella or prominence just above the nasofrontal suture. The inion is identical with the occipital protuberance. The following rules are based upon the observations of Heftier, Thane, R,eid, Horsley, Fraser, and myself: I. The longitudinal fissure. This corresponds with the naso-occipital arc. II. The fissure of Rolando. Measure the distance from the glabella to the inion; find 55.7 per cent, of this distance, and the figures obtained will indicate the distance of the upper end of the fissure of Rolando from the glabella. It should be about 48 mm. behind the bregma in male adults, 45 mm. in women, 30 to 42 mm. in infants and young children, respectively. The fissure runs downward and forward for a distance of about 10 cm. measured on the scalp, the real length being about 8.5 cm. The fissure makes an angle of about 67° with the anterior part of the longi- tudinal fissure. The lower third of it is more vertical, and the lower end is 25 to 30 mm. behind the coronal suture. The fissure is shorter in children. III. The fissure of Sylvius runs nearly horizontally, and lies either under or a little above the uppermost part of the parieto-squamous suture. This suture, the external orbital process, and the parietal eminence are the guiding landmarks by help of which the surgeon can often operate with- out marking down lines on the scalp. In children the fissure is sometimes higher and more oblique. n Reid's method of finding the fissure of Sylvius is to ''draw a line from 600 CRANIO-CEREBRAL TOPOGRAPHY 601 a point 134 inches behind the external angular process to a point % inch below the parietal eminence. The ascending branch starts from a point % inch back from the anterior end of this line, and 2 inches (5 cm.) back of the external angular process." IV. To outline the parieto-occipital fissure, find the lambda, mark a point 3 mm. anterior to it, draw a line through this at right angles to the longitudinal fissure, extending about 2.5 cm. (1 inch) on each side of the median line. This marks the position of the fissure. If the lambda cannot be felt, its position may be found by measuring the naso-occipital arc and taking 22.8 per cent, of it. This indicates the distance of the lambda from the inion or external occipital protuberance. The average Fig. 257. — Showing the position of the bony points on the cranium, the sutures, and the principal underlying fissures, also the basal outline of the brain. distance in male adults is 7.42 cm. (2% inches). It is greater in women than in men by a little over a millimetre. V. To outline the frontal lobes: The anterior end of the frontal lobes reaches to a point determined by the thickness of the frontal bone. This ranges from 2 to 8 or more mm. (3^2 to 3^ inch). The floor of the anterior fossa reaches in front to a level a little above the supra-orbital margin (16 mm., % inch — Heftier). It slopes down and backward, its pos- terior limit being indicated by the lower end of the coronal suture. VI. To outline the temporal lobe and the lower border of the cere- brum: The temporal lobe is limited above by the fissure of Sylvius, below by the contour line of the lower border of the cerebrum. This latter cor- responds to a line drawn from a point slightly (about 12 mm.) above the zygoma and the external auditory meatus to the asterion, and continued 602 DISEASES OF THE NERVOUS SYSTEM on along the superior occipital curve to the inion. The anterior border of the lobe corresponds to the posterior border of the orbital process of the malar bone. The temporal lobe is about 4 cm. (1% inches) wide at the external auditory meatus. A trephine, as Bergmann states, placed half an inch above the meatus would enter the lower part of the lobe. The middle of the lobe is in a vertical line from the posterior border of the mastoid process. A line from the upper end of the fissure of Rolando to the point of the process would pass through this important sensory area (Fig. 259). Lambda Inion Fig. 258. — Showing anatomical landmarks on skull. VII. To find the position of the central ganglia, viz., corpus striatum and optic thalamus, draw a line from the upper end of the fissure of Rolando to the asterion, practically a vertical line. This limits the optic thalamus posteriorly. A vertical line parallel to the first, a little in front of the beginning of the fissure of Sylvius, limits the corpus striatum anteriorly. A horizontal plane 45 mm. (1% inches) below the surface of the scalp at the bregma limits the ganglia superiorly. The ganglia lie about 35 mm. (13^ inches) below^the superior convex surface of the brain (Fere). VIII. To reach the lateral ventricles: A number of routes may be NERVOUS DISEASES Dana PLATE IV Composite Photograph Showing Relations op Cranial Surface to THE Fissures and Convolutions. {Alec Fraser.) CEANIO-CEREBRAL TOPOGRAPHY 603 taken. The lateral is recommended by Keen. Mark a point 13-^ inches behind the external auditory meatus and 13^^ inches above a base line made by drawing a line through the lower border of the orbit and the external auditory meatus. Trephine at this point and plunge the director into the brain in the direction of a point 23^^ to 3 inches vertically above the opposite external meatus. The ventricle lies at a depth of 2 to 23^ inches (5 to 5.7 cm.). Mr. Alec Eraser has devised a way of mapping out the fissures by means of a series of composite photographs, so taken as to show the rela- tion of the underlying parts to certain tapes tacked upon the skull. One of his figures is reproduced here (Plate IV). In applying this method the surgeon tacks the tapes on the shaved scalp. Then looking at the diagram, he finds where the point in the brain is that he wishes to reach and notes its relation to the median lateral circumferential tapes. Then as the circumference of the illus- trated head is to the circumference of the living one, so is the position of the area on the tapes in the illustrated head to the desired position of the same area in the living one. The illustration is a composite of several adult heads varying in circumference from 203-^ to 233^2 inches. The cranial measurements necessary to indicate the location of the important fissures and convolutions are given above. There are a num- ber of other methods, but no very important improvements upon the original one of Reid. I have repeatedly verified the methods given and see no reason to change them. Some surgeons, however, prefer the Ander- son-Makin method. According to this, the upper end of the fissure of Rolando corresponds to a point one-half to three-quarters of an inch behind the mid-point between the inion and glabella. The upper ex- tremity of the parieto-occipital fissure corresponds to a point one- quarter of an inch behind the mid-point between the line of the Rolandic fissure and the inion. As surgical approach to brain tumors is now done by large bone flaps, Dr. Charles K. Mills has outlined the various areas to be attacked, corresponding to the pre-frontal, post-frontal, parieto-temporal, mid- frontal, parietal and occipital areas. Having determined the location of the Rolandic, Sylvian and parieto-occipital fissures, the location of these flaps is easily determined. 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