«C3 A v <3 s I Columbia Bntoerarttp inUjeCttpoflraigtirk College of JJfjpgtcianst anb burgeons Hifarar? Digitized by the Internet Archive in 2010 with funding from Open Knowledge Commons http://www.archive.org/details/textbookofnervouOObing A TEXTBOOK OF NERVOUS DISEASES FOR STUDENTS AND PRACTISING PHYSICIANS IN THIRTY LECTURES BY ROBERT BING DOZENT FOR NEUROLOGY AT THE UNIVERSITY OF BASEL ONLY AUTHORIZED TRANSLATION BY CHARLES L. ALLEN, M.D., Los Angeles, Calif. WITH ONE-HUNDRED AND ELEVEN ILLUSTRATIONS IN THE TEXT 9ooo NEW YORK REBMAN COMPANY in. 148 and 145 WEST 86th STREET Copyright, 1915, by REBMAN COMPANY New Yob k All Rights reserved I'llISIIl) IN AMKltll'A Preface When it was suggested by my publishers that I should write a text-book of nervous diseases adapted to the needs of students and practical physicians ; in size between the summary compendiums and outlines and the large refer- ence works and handbooks of neurology. I found upon inquiry among my colleagues so general an agreement that such a book was desirable, that I no longer hesitated to undertake its preparation. When I decided to present my material in the form of lectures, it was because of the recollection that in my student days, the reading of text-books in lecture form was to my fellow stu- dents and myself like an oasis in the midst of the tiresome work of preparing for examinations. On the other hand, I did not conceal from myself that the systematic arrangement of the material, as compared to its presentation in lectures, had in general the advantage of greater clearness and better adapta- tion for quick reference. On this account I have endeavored to combine the advantages of both methods of arrangement, by the introduction of titles, sum- maries in tabular form, and recapitulatory headings. I have also laid great stress upon a complete index. In the arrangement of the subject matter, I have subordinated the cus- tomary topographical and pathologico-anatomica] classification almost en- tirely to the etiological and pathologico-physiological, as a glance at the table of contents will show: I was so enabled, for example, by grouping together the syphilogcnic diseases of the whole central nervous system, through the con- sideration in common of the most varied disturbances of conduction in the territory of the peripheral nerves, by the clinical synthesis of the diverse varieties of spinal "Transverse lesions,"' by handling in common the "Dyskine- sias," the "Dysglandular Symptom-complexes," etc.. to avoid much repeti- tion. This was all the more necessary, since in spite of my plan of limiting the si/e of my text-book, I found it necessary to treat somewhat at length, subjects of special Importance to the practicing physician; in fact in some instances to go more into detail than has so far been done in the larger works: so for example, as to the nervous manifestations of arteriosclerosis, the treat- ment of tabes, of neuritis and neuralgia, the psychology of the neurasthenic, etc. Further in favor of this plan, was the fact that with regard to localization; by referring to my "Compendium of Topical Diagnosis of Diseases of the Brain ami Spinal Cord" from which a few loans have been obtained, I have been enabled to treat the matter in a manner very condensed, but adequate lo the scope of this book. With all justice to my endeavor to presenl particularly my own experiences and my own views, I believe thai 1 have been careful enough in imparting the vi PREFACE observations and opinions of other authors to hope that my book may present to the non-neurologist a well-rounded view of our specialty, which unfor- tunately still suffers in some places from insufficient consideration in the clinical courses of instruction. In this connection I have refrained from forcing to the front the views of any particular school, rather have I endeavored to exercise a certain eclecti- cism, which is justly considered as the scientific signature of our country, in which the German and the Latin manner of thought and investigation blend, and reinforce one another in harmonious fashion. With regard to the illus- trations of my book, it was not my intention to prepare an atlas of rare and atypical cases, but rather to bring before the reader the instructive and char- acteristic of the more important morphological anomalies. Where my own photographs and sketches have been insufficient, I have been aided in the most friendly manner by my colleagues who have put their material at my disposal. I am indebted in the highest degree to Doctors Eduard Hagenbach, F. de Quervcdn, B. Stahelm, E. W'uland, E. Villiger, B. Block, P. Knapp, and H. Iselin of Basle, A'. Frey and E. Bircher of Aarau, E. Buppaner of Samaden. All the photomicrographs were kindly prepared by my Assistant Dr. Leuen- berger from preparations of my collection, Basle Robert Bing. In translating this thoroughly useful, practical and up-to-date book, it has been endeavored to present the author's views as nearly in his own language as is consistent with clear and readable English. An occasional foot-note of explanation, or of reference to matters which have come up since the German Edition was published, has been added. ..,„. .^^^ r TRANSLATOR. Table of Contents Lecture I. — Diseases of the Peripheral Nerves — A. Disturbances of Conduc- tion — 1. Spinal Nerves. Page 1 Lecture II. — Peripheral Nerves (Cont'd) — 2. Cranial Nerves — B. Neuritis and Polyneuritis. Page 26 Lecture III.- — Peripheral Nerves (Cont'd) — C. Neuralgias — D. Treatment. Page 45 Lecture IV. — The Dyskinesias — A. Tremor — B. Fibrillary Contractions — C. Muscular Spasms. 1. Local Spasms — 2. Occupation Spasms — 3. Myo- klonias — L Tetany. Page 63 Lecture V. — The Dyskinesias (Cont'd) — D. Choreiform Diseases. 1. Chorea Minor — 2. Hereditary Chorea — E. Athetoses — F. Paralysis Agitans — G. Myotonia — H. Congenital Muscular Atrophy — I. Periodic or Paroxysmal Paralysis — J. Myasthenia. Page 80 Lecture VI. — Progressive Muscular Atrophies — A. Myopathic Form — B. Neural Form — C. Spinal Form. Page 97 Lecture VII. — Spastic Spinal Paralysis — Amyotrophic Lateral Sclerosis and Progressive Bulbar Paralysis. Page 111 Lecture VIII. — Hereditary Family Ataxias — I. Friedreich's Disease — II. Cerebellar Heredo Ataxia — III. Infantile Progressive Hypertrophic Neuritis. Page 123 Lecture IX— Multiple Sclerosis. Page 132 Lecture N. — Further Diffuse Diseases of the Spinal Cord — A. Spinal Gliosis and Syringomyelia — B. Hematomyelia — C. Transverse Lesions of the Spinal Cord — 1. Genera] Symptomatology 2. Diffuse Myelitis 3. Tumors of the Spinal Cord 1. Lesions of the Spinal Cord due to Affec tions of tin Vertebral Column. I'm,/'' 1*8 I.i' ii i; i XI. The Syphilogenic Diseases of the Central Nervous System A. Tabes 1 )orsalis. P I vii viii TABLE OF CONTEXTS Lecture XII. — The Syphilogenic Diseases, etc. (Cont'd) — A. Tabes Dorsalis {Cont'd). Page 182 Lecture XIII. — The Syphilogenic Diseases, etc. (Cont'd) — B. Progressive Paralysis of the Insane (General Paresis). Page 200 Lecture XIV. — The Syphilogenic Diseases, etc. (Cont'd) — C. Syphilogenic Combined System Diseases — D. Cerebro-Spinal Syphilis. Page 213 Lecture XV. — Arteriosclerosis of the Nerve Centers, Page 222 Lecture XVI. — The Acute Infectious Diseases of the Central Nervous Sys- tem — A. "Essential Infantile Paralysis'" (The "Meine-Medin Disease") (Anterior Polyomyelitis) — B. Epidemic Cerebro-Spinal Meningitis. Page 235 Lecture XVII. — Encephalorrhagia and Encephalomalacia (Supplementary) Atypical and Extracapsular Hemiplegias. Page 25(5 Lecture XVIII. — Aphasia, Apraxia and Agnosia. Page 272 Lecture XIX. — Tumor Formations, Inflammations, and Disturbances of Circulation in the Brain and its Membranes — A. Brain Tumor — B. Brain Abscess — C. Purulent Cerebral Meningitis — D. Tuberculous Menin- gitis — E. Internal Hemorrhagic Pachymeningitis — F. Thrombosis of the Brain Sinuses — G. Non-suppurative Encephalitis — 1. Polioencephalitis Superior Hemorrhagica — 2. Acute Hemorrhagic Encephalitis of Adults — H. The Circulatory Disturbances of the Brain. P (l 9 e 288 Lecture XX. — Diseases of the Cerebellum — A. Tumors — B. Abscesses — C. Serous Meningitis of the Posterior Fossa of the Skull — D. Hemorrhages and Softenings — E. Agenesis and Atrophies — F. Infectious Diseases. Page 306 Lecture XXI. — Malformation, Congenital and Early Acquired Defective Conditions — A. Hydrocephalus — B. Cranial and Spinal Ectopics — C. Congenital Nuclear and Muscle Defects. Page 317 Lecture XXII. — Infantile Spastic Hemiplegia and Diplegia; Little's Dis- ease; Idiocy. Page 327 Lecture XXIII. — Dysglandular Symptom-Complexes — I. Basedow's Disease. Page 345 TABLE OF CONTENTS ix Lecture XXIV. — Dysglandular Symptom-Complexes (Cont'd) — II. Myxe- dema — III. Adrenal Insufficiency and Addison's Disease — IV. Acromegaly ■ — V. Other Dysglandular Syndromes — Adiposo Genital Degeneration — Dyspinealism. Page 358 Lecture XXV. — Diseases of the Sympathetic, Angio- and Tropho-Neuroses — A. Diseases of the Sympathetic — B. Acroparesthesia — C. Angiospastic Dysbasia — D. Angiospastic Symmetrical Gangrene, Raynaud's Disease — E. Scleroderma — F. Neurotic Dropsy — 1. Circumscribed QOdema of the Skin — 2. Intermittent Dropsy of the Joints — G. Erythromelalgia — H. Facial Hemiatrophy and Hemi-hypertrophy — I. Herpes Zoster. Page 369 Lecture XXVI. — Epilepsy. Page 388 Lecture XXVII. — The Psychoneuroses — A. Psychoneuroses and Neuro- pathic Diathesis — B. Neurasthenia. Page 403 Lecture XXVIII. — The Psychoneuroses (Cont'd) — B. Neurasthenia (Cont'd). Page 418 Lkcttre XXIX. — The Psychoneuroses (Cont'd) — C. Hysteria. Page 436 Lecture XXX. — Migraine. Page 454 Index to Authors Page 463 General Index Page 467 List of Illustrations FIO. PAGE 1. Electric Irritability in "Reaction of Degeneration" 11 2. Myographic Curves on Direct Stimulation of a Muscle with the Galvanic Current 12 3. Motor Points of the Xeck and of the Trigeminus 1(3 1. Motor Points of the Face 17 5. Motor Points of the Upper Extremity. Outer Side 18 6. Motor Points of the Upper Extremity, Inner Side 18 7. Motor Points of the Lower Extremity, Anterior View If) 8. Motor Points of the Lower Extremity, Posterior View 20 9- Paralysis of the Serratus on the Right Side ' 21 10. Paralysis of the Suprascapular Nerve on the Right Side. Atrophy of the Supra- and Infra-Spinatus 21 1 1 and 12. Ulnar Paralysis (Trauma), "Claw-hand" 22 18. Ulnar Paralysis (Neuritis) 22 11. "Wrist-Drop" in Paralysis of the Radial Nerve 22 1 5. Traumatic Paralysis of the Peroneal Nerve 23 Hi and 17- Areas of the Different Sensory Nerve Trunks and Their Branches 24 18. Left-Sided Oculomotor Paralysis. Ptosis 27 If). Paralysis of the Internal Rectus on the Right Side 28 20. The Sensory Nerve Supply of the Head 80 21. Bilateral Abducens Paralysis :U 22. Left-Sided Peripheral Facial Paralysis 32 23. Left-Sided Peripheral Facia] Paralysis, Upon Attempt to Close Both Eyes 34 2 1. Diagram of the Different Categories of Fibers in the facial Nerve 35 25. [mage "I the Vocal Cords on Inspiration 38 26. Tuberculous Polyneuritis 12 27. Hereditary family Essential Tremor, Handwriting til 28. "Uisus Sardonicus" in Traumatic Tetanus (i(i 29- Spasmodic Torticollis (if) xii LIST OF ILLUSTRATIONS FIG. PAGE 30. Parathyroid Tetany 76 31. Athetosis 86 32. Paralysis Agitans. Handwriting 87 33. Typical Attitude in Paralysis Agitans 88 34. Rachitic Myopathy 93 35. Alteration of the Muscle in Progressive Muscular Atrophy 98 36. Progressive Muscular Dystrophy 100 37. Progressive Muscular Dystrophy. (Type Erb) 102 38. Excessive Clubbing of the Foot on Account of the Gastrocnemius in Progressive Muscular Atrophy 103 39. The Babinski Reflex 114 40. Alteration of the Spinal Cord in Amyotrophic Lateral Sclerosis 117 41. Friedreich's Disease 125 42. Alteration of the Spinal Cord in Friedreich's Disease 126 43. Atrophic Cerebellum of a Patient Aged Forty Years, with Hereditary Ataxia 130 44. Normal Cerebellum of a Man Aged Forty Years 130 45. Multiple Sclerosis 133 16. Multiple Sclerosis. Handwriting 135 47. Central Gliosis Passing Over Into Syringomyelia 144 48. Extent of the Anesthesia in Transverse Lesions at Different Levels. ... 150 49- Complete Severing of the Spinal Cord at the Level of the Yllth Cervical Segment 151 50. The Brorvn-Sequard Symptom-Complex 155 51. The Broirn-Sequard Symptom-Complex 156 52. Tracts which Degenerate Upward and Downward 158 53. Paraplegia, with Extreme Contracture in Flexion 161 54. The Different Categories of Fibers in the Posterior Roots and their Con- tinuation in the Spinal Cord 167 55. Structure of the Posterior Columns 168 56. Lumbar Tabes (Cervical Region) 169 57. Lumbar Tabes (Dorsal Region) 170 5S. Lumbar Tabes (Lumbar Region) 171 59. Genu Recurvatum 174 60 and 61. Root Fields and Peripheral Nerve Areas 176 LIST OF ILLUSTRATIONS xiii FIG. PAGE 62. Typical "Radicular" Disturbances of Sensibility in a Case of Tabes Dorsalis 177 63. Tabetic Foot ' 178 61.1 65. L Exercise Treatment in Tabes Dorsalis ]<)S 66. J 67. Changes in the Spinal Cord in Syphilitic Spinal Paralysis 214 68. The Combined Tabes 215 69- Status Lacunaris Cerebri 228 70. Pes Equinovarus as a Result of Anterior Poliomyelitis 238 71. Paralytic Pes Cavus as a Result of Anterior Poliomyelitis 239 72. Infantile Spinal Paralysis. Late Stage 240 73. Infantile Spinal Paralysis. So-called "Hand Walker," etc 241 74. The Blood Supply of the Spinal Cord. Injected Preparation (Carmine Gelatine) 244 75. Acute Anterior Poliomyelitis in Childhood 245 76. Anatomical Points for Guidance in Making a Lumbar Puncture 253 77. Introduction of the Needle in Lumbar Puncture 251 78. Arterial Supply of the Cerebrum and the Basal Ganglia 257 79- The Internal Capsule and Corona Radiata 258 80. Visual Tract and Pupillary Reflex Paths 2.">9 81. Cerebral Hemiplegia from Capsular Hemorrhage on the Right Side 260 82. The Cortical Speech Centers and Their Connections 274 83. Seat of a Cortical Motor Aphasia 275 84. Papilledema. Sarcoma of the Left Cerebellar Hemisphere 291 85. .Motor. Sensible and Sensory Cortical Fields 292 86. Motor, Sensible and Sensory Cortical Fields 298 87. Serous Meningitis of the Posterior Fossa of the Skull. Ideal Sagittal Section 314 .ss .-ind 89. Congenital Hydrocephalus 818 90. Superior Occipital 1 1 vdroecphalocele ;!21 91. Occult Spina Bifida with Local Hypertrichosis 322 92. Open Spina Bifida (Cervical Meningocele) 323 93. Diplegia Spastica Infantilis :;:;i 94. Diplegia Spastica Infantilis xiv LIST OF ILLUSTRATIONS FIG. PAGE 95. Pithecoid Idiot 336 96. Eye-Ground in Amaurotic Idiocy 338 97. Microcephalous Idiot 339 98. Mongoloid Idiot 340 99. Alpine Cretin 342 100. Tragic Look in Basedow's Disease K 3-16 101. Basedow's Disease 347 102. Myxoedema ' 360 103. Plan of the Sympathetic Nervous System 370 104. Homer's Symptom-Complex 372 105. Sclerodactylism, with Formation of Necroses 378 106. Scleroderma 379 107. Herpes Zoster of the Trunk 384 108. Herpes Zoster of the Face 385 109. Modelling's Disease 387 110. Typical Localizations of Hysterical Topalgias (shaded) and Anesthesias ( Black) 438 111. Hysterical Self-Injury. Pseudo Pemphigus 451 LECTURE I Gentlemen : In this course of lectures I will endeavor to guide you in a way differing somewhat from the customary one, through the extensive and interesting subject of clinical neurolog}'. I will not treat separately the general symptomatology and diagnosis of nervous diseases, neither will I precede the account of individual affections by a special consideration of their pathology as is customary in most text-books. It seems to me more correct didactically, to proceed directly, without the usual introductory remarks, to the study of the clinical pictures and to describe the methods of examination and the inter- pretation of the different neurological symptoms and syndromes, as digressions at the points where they fit most naturally and with least compulsion, into the general presentation of the subject. The same considerations apply to the anatomical, physiological and pathological data, which are indispensable for an understanding of the clinical material. However, care will be taken that the treatment of these matters is sufficiently restricted not to interfere with the clinically useful character of the book. Since practical neurology is to be treated, and above everything the point of view of the non-specialist is to be borne in mind, rare affections are brought forward and described it is true, but those diseases of the nervous system which on account of their frequency are met with in daily practice are more particu- larly considered and carefully described. We will also lay great stress upon questions of treatment and will endeavor to prevent occupying ourselves so far with the "School Cases" and their great symptom-complexes, that the "minor" neurology of office practice, an important and thankful field for the development and exercise of professional ability and therapeutic tact, is not forced into the background. Diseases of the Peripheral Nerves The affections of the peripheral nerves agree so generally in their diag- nostic relations, that, we can readily consider their symptoms from two clinical points of view. Without doing' violence to the facts, we will hence consider in common all disturbances of conduction, whether of traumatic, neuritic or neo- plastic origin, and after that will take up peripheral nervous irritative symp- toms, which in part have their origin in neuritic processes, in part as neuralgias, can claim a certain clinical autonomy. Considered on the contrary from the standpoint of pathology, the diseases of the peripheral nerves present a great multiplicity of clinical pictures, as many 1 2 LECTURE I in fact as there are peripheral nerves, and their recognition and treatment pre- supposes intimate familiarity with anatomical and physiological data. In order therefore to adhere to our plan and to avoid burdening you with tiresome repetition and unnecessary details, I will consider the material under the following divisions : 1. The peripheral disturbances of nerve conduction. a. In the spinal nerves. b. In the cranial nerves. 2. Special remarks on neuritis and polyneuritis. 3. The neuralgias. 4. The treatment of the diseases of the peripheral nerves. A. Disturbances of Conduction Etiology Traumatic influences of various kinds play a very important role as start- ing points for destructive lesions of the peripheral nerves. In the first place we would mention wounding or severing of the nerve trunks as they occur in the extremities in stab or incised wounds, from penetrating projectiles, some- times from the splinters of a fractured bone, etc. A single severe compression if it affects nerves exposed in a superficial position may also cause interruption of conduction, so for example in one of my cases a musculo-spiral paralysis was caused by the patient being struck over the region where this nerve passes around the outside of the humerus, by a falling boiler. Most frequently, how- ever, the duration of the compression is longer. So, paralyses of the brachial plexus are produced by the pressure of a crutch, by the nerve plexus being caught under the clavicle in over-elevation of the arm in prolonged narcoses, or as a result of dislocations of the shoulder. Paralyses of the musculo-spiral nerve are sometimes produced in prisoners by too tightly tying their arms.* Peroneal paralysis has been known to arise in agricultural laborers from pro- longed squatting; in fact falling asleep with the knees crossed has been some- times known to seriously injure the compressed peroneus. Other "sleep- paralvses" are seen not infrequently in the musculo-spiral and ulnar nerves, in persons who sleep at night, the head upon the outstretched arm, or in those who enjoy a noonday nap their elbows resting upon the table. On account of constantly supporting the elbows against the hard table edge, "professional paralyses" of the ulnar nerve occur in glass workers, xylographers, telephone operators, etc. A cervical rib can produce paralysis of the dilator pupillas and tarsalis superior muscles, by pressure upon the sympathetic nerve in the neck. Too tightly put in plaster, in extension apparatus, compression by growing bone callus or by a tumor, the application of the Esmarch bandage in checking hemorrhage, may, on account of their relative frequency, be mentioned from * This refers to the habit of the Continental Police of pinioning the prisoner's arms behind his back. (Translator.) DISEASES OF THE PERIPHERAL NERVES 3 an almost endless list of causes of compression paralysis. Finally, strain and overstretching of nerve trunks can cause a break in conduction, which, not to speak of obstetrical paralysis in children forcibly extracted, plays a part in accident practice. For example, I saw in a stableman a serratus paralysis produced by a horse whose hind leg he was holding to be shod, suddenly pulling it loose; in a plasterer, median paralysis due to the sudden jerking out of his hand of a rope by the fall of a heavy box of plaster to which it was attached, etc. "Neuritic processes" represent the second subvariety of disturbances of peripheral nerve conduction. Indeed, the term neuritis, though sanctioned bv clinical use, cannot stand a strictly scientific criticism. Rather do we find in this group of diseases, not only inflammatory, but also primarily degenerative processes, and we can assure ourselves that even in histopathological relations, as we will see directly, the lines between these two categories cannot be too sharply drawn. The list of causes which have been held responsible for neuritis is varied enough. Particularly often are there in the history, statements about exposure to cold. Vague and unsatisfactory as this idea is, we cannot get rid of it. To select one example: in the so-called rheumatic facial paralysis, in which, according to the findings of Minkowski, Dejerine and Theohari and others, there is a neuritic or degenerative process affecting particularly the peripheral portions of the facial nerve, Remak found in 45%, I in 58%, and other neurologists in as high as 70% of the cases, a history of exposure to cold, which not rarely appeared to have acted specially upon the affected side (a draft). Neuritis from overuse has undoubtedly been demonstrated. Frank Smith first described a ''Hammer Palsy" which he saw develop in the right arms of smiths in the Sheffield Iron Works, after excessive exertion ; Coistcr called attention to an atrophy of the small muscles of the hand, with pain in the median distribution, which occurs in cigar-makers and a similar involvement has been observed in milkmaids, drummers and locksmiths; musculo-spiral paralysis is found in weavers, ulnar paralysis in oarsmen, etc.* Now, however, in these neuritides from cold and from overfunction, it is very much of a question if these factors are exclusively responsible for the production of degenerative processes in the affected nerves. Very often further inquiry develops the fact that the real cause of the disease lies deeper, that for example alcoholism, disturbance of nutrition, or infectious diseases, have so modified the nervous system of the patient that these accidental noxious influ- ences can exert a pathogenic action. When on any account, metabolism in nerves is so altered that material for the replacement of the constituents used ' These neuritides from overuse have been grouped, tdgether with the pressure paralyses of certain nerves, in persons of definite callings as "professional pareses." To these must he added certain "atrophies from overuse," which probabrj do no! depend upon neuritic processes, inii .Hi- ni myopathic nature, since tin- affected muscles do no! correspond in their grouping to those supplied by a definite nerve, the electric examination gives no indication of the neuro- genic origin of the symptoms, neither are there pain, sensitiveness to pressure in the nerve, nor sensory disturbances. For example, in glass-blowers, an atrophj of the muscles of the cheeks has been observed, .nut I saw in ;i si swhal anemic dentist an atrophy of the deltoid muscle, apparently from constantly holding up the electric drill. 4 LECTURE I up in functionating, is available in less quantity than normal, as Edinger has shown, the addition of noxious influences in themselves slight, can so disturb the already unstable trophic equilibrium, that clinical and anatomical manifes- tations of disease are produced. On the other hand, in the cases in which, in a previously healthy person, after severe chilling, a neuritis develops, as in other rheumatic troubles, the supposition that the injurious influence of the refrigeration through reduction of the general and local resistance of the organism has opened the door to some sort of infectious process, and has only indirectly acted in producing organic lesion, is altogether plausible. In toxic and infectious neuritides, as well as in those occurring in the course of general nutritional and metabolic disturbances, we can base our views as to etiology upon a firmer foundation. Of all exogenic poisons alcohol stands at the head, indeed not in the sense that a single alcoholic intoxication, even the most intense, is able to set up neuritic processes, but rather that chronic alcoholism in part causes neuritides by itself alone, and in part produces a very decided predisposition to them, on which account, for example among the victims of "professional pareses," drinkers are present in very high percentage. Oppenhcim hence speaks with justice of "toxico-professional" paralyses. Chronic lead poisoning also plays an important role ; type-setters, painters, glaziers, type-founders, and polishers, etc., are, as is well known, particularly exposed, and these callings also furnish their contingent to the toxico-pro- fessional cases. Contrary to alcohol and lead, arsenic can set up neuritides after acute poisoning. (I saw a case after an attempt at suicide.) Other poisons which may be mentioned here are, ethereal oils, carbon bisulphide, car- bonic oxide, dinitro-benzol, anilin, phosphorus, mercury, copper and silver. In one of my cases, in a worker at a factory for chemicals, combined intoxica- tion, with the vapors of bromide of methyl and methyl alcohol had preceded the outbreak of the polyneuritic symptoms. Among infectious diseases which can set up neuritis are to be mentioned typhoid fever, influenza, pneumonia, erysip- elas, diphtheria, gonorrhea, septicemia (puerperal), malaria, relapsing fever, syphilis, amebic dysentery, and tuberculosis. In these, the respective bacteria are not to be found in the affected nerves, so that only toxine action can come into question. Lepra alone produces multiple foci in the peripheral nervous system by penetration and proliferation of bacilli ; since, however, the clinical picture of this disease in no way agrees with that of the other neuritides, but presents considerable similarity to that of syringomyelia, we will put off its consideration to the chapter devoted to this disease of the spinal cord (Lec- ture X). Cachexia and autointoxication also furnish a frequent basis for the devel- opment of neuritic phenomena: carcinomatosis, diabetes, gout, anemia, senile marasmus, pregnancy,* leucemia, chlorosis. By local poisoning, neuritis occurs after ether injections in the neighborhood of a nerve, by local infection (with bacterial invasion which often ascends along the nerves) in connection with * The very frequent accompaniment of the neuritides of pregnancy by pernicious vomiting (hyperemesis gravidarum), speaks for their autotoxic origin. DISEASES OF THE PERIPHERAL NERVES 5 septic wounds, phlegmons, etc., by local disturbance of nutrition, in the distribu- tion of vessels with arteritic or arteriosclerosic lesions. It is unexplained whether the form of polyneuritis known as Beri-beri or Kakke, endemic in southeast Asia, Japan and Brazil, is to be attributed to a poison which is formed outside the body and introduced with the rice eaten, or to a toxine formed in the body itself by microbes which have entered it, that is, whether this affection is to be counted among the toxic or among the infectious neuritides. Finally, after traumatic and neuritic influences, the third and last category of causes of disturbances of conduction in the peripheral nerves, tumor forma- tion, must be considered. In this, mainly true neoplasms, seldom the infectious granulomata — for example, gummata — are concerned. Carcinomata frequently involve the neighboring nerve trunks, cancer metastases in the axilla for example attacking the brachial plexus. Sarcomata, fibromata, myxomata, lipomata, may' arise from the nerve itself. These are designated as "neuro- mata," though it is not entirely proven that the nerve tissue itself takes part in the tumor formation. However, the nerve connective tissue plays the chief part in the neoplastic proliferation. Besides solitary "neuromata," * there are plexiform neuromata which de- velop along the whole of a nerve trunk or branch, and finally a "general neuro- matosis" (Recklinghausen's disease). While these latter often cause pain, it is remarkable that they only infrequently cause any considerable disturbance of conduction in the affected nerves, though on account of the deformity which they produce and also for the reason that fibromatous growths show consid- erable tendency to undergo sarcomatous degeneration, they are of surgical importance. Pathogenesis and Pathological Anatomy The injury of a nerve at any part of its course, has as a result degenerative changes in its parenchyma: the axis cylinder shows the earliest changes, it becomes tortuous, varicose, and at length breaks up into very fine granules. Soon after, the medullary sheath begins to undergo changes, the myelin of which it is composed breaks up into fatty droplets which are at length absorbed. Certain proliferative changes in the interstitial tissues proceed parallel with the degeneration of the parenchyma; the sheath of Schwann shows considerable increase in its nuclei, which arrange themselves in columns. According to the law of Waller, "A nerve filler can only preserve its anatomical and physiological integrity, while it remains in uninterrupted con- nection with its Living cell of origin"- the degeneration described occurs throughout the peripheral portion of (he severed nerve. The same changes are Usually found, also, for some distance in the central stump. Of course, the same lesions are found in man after the severing of a nerve; simple squeezing, or compression which lasts longer, produce in the nerve ill . which have their seat upon the smallest skin nerves seldom give any other symptoms than mure ur Irs-, sensitiveness to pressure; these .ire the so-called "tubercula dolorosa" of the surgeons. 6 LECTURE I changes of the same character, but quantitatively less severe. However, the anatomical picture produced by toxemic injury, refrigeration, etc., is often so similar to the traumatic nerve degeneration, that Babinski could remark, that apart from tumor-formation, all diseases of the peripheral nerves are to be attributed to neuritis. In spite of this, I cannot decide, like other neurolo- gists, to denominate the disturbances of conduction after external violence as traumatic neuritis, and on this account, in speaking of etiology, I kept the two categories sharply separated. For the rest there are acute infections and toxic neuritides, in which the inflammatory nature of the process is quite plainly marked by extensive infiltration of the connective tissue endoneurium, epineu- rium and perineurium, by hyperemia of the nerve, diapedesis of white corpuscles, etc. Of clinical importance is the tendency of the sheath of Schwann to undergo proliferatory processes as already mentioned; the nuclei arranging themselves in rows, form in the affected parts columns, along which from the central stump regeneration of the axis cylinder, followed by that of the medul- lary sheath, can occur. In severed nerves, regeneration is possible even years later, as is shown by the results of reunion of the cut fibers by nerve suture undertaken long after the injury. The General Symptomatology of Interruption of Conduction Since the peripheral nerves have to conduct stimuli of varied kinds, cen- tripetally the different forms of sensation, centrifugally motor, trophic and vasomotor impulses, and since disturbance of their capacity to conduct can impair any of these, it is necessary here to present to you a short review of the clinical physiology of each of these functions. I. SENSIBILITY This term in no way expresses a simple conception. The clinic, however, does not go so far in its analysis as experimental physiology which differen- tiates a sense of pressure, sense of position, sense of cold, sense of heat, sense of pain, sense of movement, etc. It limits itself rather in the testing of "general sensibility" (i.e., the afferent impulses which do not come from the eye, from the ear and from the gustatory and olfactory apparatus) to the investigation of four chief qualities. a. Touch sense. We test this by stroking the part with a wisp of cotton, a brush, the finger, etc. Its diminution is called tactile lvypoesthesia, its loss, tactile anesthesia. In pathological increase of sensitiveness to touch, whereby this is found painful, we speak of tactile hyperesthesia. b. Temperature sense. To study this, we test the ability of the subject to distinguish between cold and warm objects. The condition of impaired or absent temperature sense is called thermohypoestbesia or thermoanesthesia as the case may be. The opposite condition is thermic hyperesthesia. c. Pain sense. Pinpricks, pinching of a fold of skin, etc., furnish infor- mation as to this. Diminution = h'ypalgesia. Loss = analgesia. Increased sensibility to pain is called hyperalgesia. DISEASES OF THE PERIPHERAL NERVES 7 d. Deep sensibility (also "Bathyesthesia"). In contradistinction to the previously enumerated "Superficial-sensibilities," whose seat is in the integu- ment, we understand under the above name the sum of the centripetal stimuli, which stream to our central nervous organs from the muscles, tendons, bones, joints, etc. A part of these pass the threshold of consciousness in the brain, and inform us as to the position of our limbs, the angles at which our joints are bent (sense of position), the extent of a movement which is being carried out (sense of movement), etc. Here belongs also vibration-sense (palles- thesia), which is felt when a vibrating tuning-fork is placed upon a super- ficially seated part of the skeleton. Another part of these influences, however, does not come into consciousness, but regulates, subconsciously, the motor mechanisms, which come into action in all complicated and combined move- ments, such as walking and standing. It makes possible in this way the main- tenance of equilibrium, the harmonious course of locomotion, the synergistic action of related muscle groups, etc. More or less marked defects in deep sensibility manifest themselves by the symptoms of ataxia (hypotaxia), incoordination, asynergy, etc. Since in rec- ognizing an object by feeling it with closed eyes, the sense of position and sense of movement of our fingers plays the chief part, stereoanesthesia ( inabil- ity to recognize form by palpation) is also an expression of disturbed deep sensibility. Loss of vibration-sense' is called pallanesthesia. As Head has shown, the fibers conveying deep sensibility run in the muscle nerves, on which account they are uninfluenced in lesions of the cutaneous nerve branches. II. MOTILITY Under motility in the broader sense, we understand, not only voluntary movements, but. also the phenomena of the tonus and the reflexes. a. Voluntary movement. Complete abolition of the ability to perform voluntary movements, we denominate paralysis, partial disability, paresis. We Bpeak of a paralysis of individual muscles when a few muscles, of plegias when whole limbs, or at least portions of limbs, are affected; if a single extremity i- involved we call it a monoplegia, if two extremities on the same side, a hemiplegia, if two symmetrica] extremities, a diplegia (when it is the two legs the term paraplegia is used) ; an extension to three or to all four limbs becomes respectively triplegia and tetraplegia. h. Tonus and Reflexes. A mechanical stimulus (striking or stroking) ap- plied to certain parts of our bodies (certain tendons, bones, skin regions) under normal conditions produces certain definite motor phenomena (a contrac- tion of certain muscles); we speak of them as reflexes. Besides Ibis, there stream (probably from tin; whole surface of our bodies and from all the skeleton) to our central organs, continued subconscious centripetal stimuli of little intensity, which by reflex action keep up a moderate continuous contrac- tion of all our muscles. This is tin 1 tonus. We can define this in the follow- ing ti mis: "The definite degree of tension which gives our muscles the ability to reply promptly with a contraction to voluntary impulses which reach them." That in normal life, the stimuli which produce the tonus are continually at 8 LECTURE I work is proved by the fact that in healthy persons the tonus is never relaxed, even in sleep. The muscles of the sleeper are never so completely relaxed as in the corpse; and only in an advanced stage of narcosis does the tonus give way. Abolition of the reflexes and of the tonus we call respectively areflexia and atonia; diminution of these, hypo-reflexia and hypotonia; for the exag- geration of these phenomena on the other hand the expressions hyper-reflexia and hyper-tonia are used. I have prepared a table in which the different reflexes and the methods by which they are elicited are exposed, in which the differentiation between skin, tendon, and bone reflexes is shown, and the most important reflexes are empha- sized. One can expect to elicit all the reflexes only in very young persons. After puberty the great majority of them cannot be obtained even in perfectly healthy individuals. As to the condition of tonus, we estimate it partly by determining the tension and resistance of the muscles by palpation, partly by noting the resistance which is opposed to passive movements. Skin Reflexes. Method of Eliciting. 2. Biceps reflex 3. Triceps reflex 4. Scapulo-hume- ral reflex 5. Radius reflex 6 7 8 9.1 10-3 11 12. Patellar reflex 13 14. Achilles reflex 15 16 Scapular reflex Palmar reflex Epigastric reflex Upper abdominal reflex .Middle abdominal and lower abdominal re- flex Cremasteric reflex Gluteal reflex Plantar reflex Anal reflex Irritating the skin over the scapula Striking upon the biceps and tendon Striking upon the tri- ceps tendon Striking upon the infe- rior internal angle of the scapula Striking upon the sty- loid process of the radius Irritation of the palm Stroking from the mam- ma downward Stroking the skin of the upper abdomen Stroking the skin of the abdomen in the mid- dle and lower parts Stroking the inner side of the thigh. Striking upon the quad- riceps tendon Stroking the nates Striking upon the Achil- les tendon Stroking the sole Irritating the perineum Contraction of the mus- cles over the scapula Flexion of the forearm Extension of the fore- Supination of the fore- arm Flexion of the fingers Retraction of the epi- gastrium Retraction of the ab- domen Retraction of the ab- domen The testicle is jerked upward Extension of the leg Contraction of the glu- teal muscles Extension of the foot Flexion of the toes Contraction of the ex- ternal sphincter ani muscle III. TROPHIC FUNCTIONS The Law of Waller already mentioned applies also to the muscles, for whose anatomical integrity an uninterrupted connection with the cells of the anterior horns of the spinal cord, or in the case of those innervated by the cranial nerves, with the motor nuclei of the brain axis, through their nerves DISEASES OF THE PERIPHERAL NERVES 9 of supply, is presupposed. If, in a motor nerve, conduction is broken, the muscles supplied by it undergo a degenerative process which we call neurogenic degenerative atrophy. Histologically this is characterized by the disappearance of the contractile element (by granular, albuminous and fatty, less frequently by hyaline and vacuolar, degeneration), and its replacement by fatty and connective tissue. These degenerative processes, under conditions which admit of a reestablishment of the connection between the muscles and the tropho- motor cells of the central nervous system, can be repaired by regenerative processes ; the histological criteria of these last ai-e, among other things, the proliferation of the nuclei of the sarcolemma surrounding the muscle fibers, the appearance of polynuclear giant cells, "myoblasts," from which muscle fibrils are formed anew, the presence of very voluminous muscle fibers, etc. A clinical sign of neurogenic atrophy is the notable electro-physiological altera- tion, first carefully studied by Wilhelm Erb in 1872, and called by him "Reac- tion of Degeneration" (R. D.). To the symptomatology of this we will return farther on in this lecture. Besides this, the central nervous system through the peripheral nerves exerts a trophic influence upon the skeleton ; when this is cut off in growing individuals, there is more or less limitation of further bone growth in the affected territory. Abnormal fragility of bones can also occur under these conditions. IV. VASOMOTOR FUNCTIONS Experimental physiology teaches us that two sorts of fibers pass from the sympathetic, through the peripheral nerves to the blood vessels, those which narrow ("vasoconstrictors") and those which increase ("vasodilators") the calibre of the vessels. Dilator fibers indeed are only recognizable in a few nerves, for example, in the sciatic. Vasoconstrictor fibers on the contrary are quite generally recog- nized as constituents of peripheral nerves. Nevertheless, in man, only rarely (that is only in the earliest stages) are we able to observe the picture which the physiologist sees in animals after destruction of the vasoconstrictor elements, namely, redness and heat of the skin. Much more common is the apparently opposite picture which can follow such an injur}', or even without one being definitely determined. Namely, the integument of the parts cut off from vasomotor innervation becomes cyanotic and cold. The cyanosis is explained by the chronic inhibition of the capillary circulation, on account of the removal of the vascular tonus, the coldness, by the changes in nutrition of the skin resulting from this cyanosis. The mechanism of the sweat secretion is very similar to that of the vasomotor innervation, the secretory fibers being mostly closely mixed in with the vasomotor fibers. Their destruction has as a result, lessening or inhibition of tin- sweat secretion (hypoidrosis or anidrosis). The opposite anomaly is called hyper-idrosis. After this general diagnostic excursion, we will proceed to the description of the sensory symptoms which accompany the disturbances of conduction in peripheral nerves. Apart from traumatic severing of nerves, there is seldom total anesthesia 10 LECTURE I of the integument, more usually there is a general reduction of superficial sensibility. Very frequently, however, the different qualities of sensation are affected in different degrees, so, for example, touch sense is intact, while pain and temperature senses are diminished. In neuritides, very often the dis- turbances of sensibility are not equally distributed over the whole area supplied by the affected nerves, but increase in intensity toward the periphery, which is perhaps connected with the increased distance of the parts there situated from the trophic centers. In diseases of a number of nerves, as in neuritis of plexuses and in polyneuritis, bathyanesthesia or bathyhypoesthesia can make itself felt in disturbances of sense of position or of movement, namely as ataxia or incoordination. A disturbance of vibration-sense is recognizable when cer- tain nerves are put out of function, particularly over the corresponding bones. Disturbances of deep sensibility are usually accompanied by reduction or loss of tendon reflexes. The motor symptoms in destructive lesions of nerves, consist in losses of function, varying from slight paresis to complete paralysis. They are always accompanied by reduction of muscular tonus with regional hyporeflexia or areflexia, with more or less marked neurogenic muscular atrophy and with the reaction of degeneration already mentioned, which will now be discussed. THE REACTION OF DEGENERATION (Erh, 1872) Let us first get clearly the normal electric reactions of the human muscles as they are brought out in the usual clinical-neurological examination. In this, we use the so-called unipolar method of stimulation, in which a large ("'indifferent") electrode is placed upon the chest or back of the person being examined, a smaller one (as a rule the 3 cm square electrode, Stmt zing's "Normal electrode") upon the muscles or nerves to be tested. Contraction of muscles occurs with the Galvanic current, not when it is slowly raised, but on sudden variations of current strength (that is, on making or breaking the galvanic circuit, naturally also, upon conducting through them the faradic current with its rapid alternate making and breaking). With a weak galvanic current, a contraction is obtained only when the negative pole is used as stimulating electrode, and the current is closed ; Kathodal closing contraction (KCC). If, now, the current is made stronger and stronger, we obtain next an Anodal closing contraction and an Anodal opening contraction (AnCC and AnOC), (the sequence of these two reactions is different in different individ- uals, AnCC usually appears before AnOC), only with much stronger current is a Kathodal opening contraction (KOC) obtained. If, now, with the current strength which has sufficed to produce KOC, the current is closed, the negative pole being on the point to be stimulated, we get, not a short contraction, but one which persists while the electrode is applied, Kathodal closing tetanus (KCTe). A still more powerful current is needed to produce Anodal closing tetanus (AnCTe), while in normal man, Anodal opening tetanus (AnOTe) does not occur. It is self-evident that KOC can only occur when the muscle was not just before the interruption of the current in a condition of tetany; KCTe can be DISEASES OF THE PERIPHERAL NERVES n avoided even with a very strong current, however, if with careful avoidance of sudden current variations, the number of milleamperes necessary to produce KOC are gradually introduced by use of the rheostat. Finally it must be added that between the results of '"indirect" (through the nerve) and "direct" (to the muscle itself), galvanic stimulation, there is this difference, that in the last method, the effect of opening the circuit is generally less. In "Reaction of degeneration" (the clinical physiological criterion of dis- turbed or interrupted trophic connection between nervous system and muscle), we find in contradistinction to the normal relations described above, the fol- lowing phenomena. The galvanic and faradic irritability through the nerve grow less and less, until, in case there is complete interruption of conduction in the nerve supplying the affected muscle, by the end of fourteen days it has completely disappeared. Direct faradic irritability of the muscle acts sim- 1 0, 20. 30. W. 50- 60. 70. 80. week. W. W- w. w. w« w. w. ,'' -**„ *,^ ___ Direct galvanic irritability. t *^ **^ mum Direct faradic irritability. * H, ** \ Indirect galvanic and faradic irritability. *x \ ^ s x x X. s * x ^ i! x x. 'k^ Electric Irritability in "Reaction of Degeneration.' ilarly. Direct galvanic irritability, on the contrary, first increases, to fall again at the end of about two months, in case the trophic connection is not in tlie meantime reestablished. If conduction is later renewed, galvanic irrita- bility and later faradic irritability of the muscle direct, come back, and at length electric stimulation of the nerve is again followed by contraction. If conduction is not restored, however, after from one to one and a half years, even galvanic irritability of the muscle is permanently lost. With these quantitative anomalies of irritability, qualitative changes go hand in hand. There is an alteration of Hie character of the contraction on galvanic stimulation; instead of the normal quick contraction we get a slow "vermicular" response, further a modification of the so-called contraction formula. While normally KCC appe.-us with a weaker current than AnCC in reaction of degeneration this relationship is reversed. We indicate this by the formula AnCC>KCC. Instead of complete reaction <>t' degeneration, we find in slight neurogenic-trophic disturbances of the muscles different Varieties of "partial R. D.," to consider all of which would fake us too I'm 12 LECTURE I afield. Stintzing has distinguished no less than thirteen varieties of partial R. D. We must content ourselves with a description of the most frequent of these varieties. In this nerve irritability is preserved, usually faradic irrita- bility of the muscle also, but there is galvanic overirritabilty, reversal of the polar formula and as the most important criterion of the R. D., slow character of the contraction. While in complete R. D. slow return to the normal — in case this occurs — requires from three to seven months, in partial R. D. under proper treatment restitution can begin in six weeks. Very rapid recov- ery can only be expected in such peripheral paralyses in which R. D. does KAKAKAKAKAKA Fig. 2. Myographic Curves on Direct Stimulation of a Muscle with the Galvanic Current. K = Cathode; A = Anode. Upper curve, normal reaction; lower curve, "Reaction of Degeneration." not appear, however. The normal irritability of the most important nerves and muscles is shown in the following tables. According to Stintzinc/, the nerves can be arranged according to their galvanic irritability in milleamperes as follows: ; Lower Limit. Upper Limit. Average. 1. N. musculo-cuta- 1. N. musculo-cuta- 1. X. musculo-cuta- 0.05 i 0.28 neous 0.17 2_ N. accessorius. . 0.10 2 X. accessorius . . 0.44 2. X. accessorius . . 0.27 3. N. ulnaris upper 0.2 3. X. ulnaris upper 0.9 3. X. ulnaris upper 0.55 4. N. 0.2 4. R. mentalis .... 1.4 4. X. medianus. . . . 0.9 5. N. medianus. . . . 0.3 5. X. medianus . . . 1.5 5. R. mentalis 0.95 6. N. cruralis 0.4 6. X. 1.7 0. X. 1.05 7. N. 0.4 7. X. peroneus .... 2.0 7. X. peroneus . . . 1.1 8. R. mentalis 0.5 8. R. zvgomaticus . 2.0 8. R. zygoniaticus . 1.4 9. N. ulnaris lower 0.6 9. R. frontalis 2.0 9. R. frontalis .... 1.45 10. R. zvgomaticus . 0.8 10. X. tibialis 2.5 10. X. tibialis 1.45 11. R. frontalis 0.9 11. X. facialis 2.5 11. X. ulnaris lower 1.6 12. X. radialis 0.0 12. X. ulnaris lower 2.6 12. N. facialis 1.76 13. N. facialis 1.0 13. X. 2.7 13. N. 1.8 DISEASES OF THE PERIPHERAL NERVES 13 TABLE SHOWING THE IRRITABILITY OF THE MUSCLES ACCORDING TO STINTZING. Galvanic Irrita- Size of the Elec- Muscle. bility in M. A. trode in cm'- M. trapezius 1.6 1- M. deltoides 1 5-2.0 12 M. pectoralis major 0.4 6 M. pectoralis minor 0.1—2.5 6 M. serratus magnus 1.0-8.5 12 M. brachialis anticus 1.1-1.7 3 M. extensor communis digitorum 0.6-3.0 3 M. extensor carpi radialis 0.8 3 M. extensor brevis pollicis 1.5-3.5 3 M. pronator radii teres 2.5 S.8 3 M. flexor sublimis digitorum 0.3-1.5 3 M. flexor carpi ulnaris 0.9-2.9 3 M. abductor minimi digiti 2.5 3 M. rectus femoris 1.6-6.0 20 M. vastus internus 0.3-1.3 20 M. tibialis anticus 1.8-5.0 1 2 Finally as concerns the trophic and vasomotor disturbances, in destructive diseases of the peripheral nervous system, they are less regularly found than might be expected from the fact that through the "gray rami communicantes," sympathetic fibers run into all the peripheral nerves. As already said, redness and local raise of temperature occur only as fleeting symptoms, later in the place of hyperemia, there is cyanosis and abnormal coolness of the integument. Local hyperidrosis is not infrequent. I will mention now the oedema which occurs in infectious polyneuritis. In beri-beri this symptom is so frequent, that we can speak of a hydropic form of this tropical disease, the "moist" form of beri-beri. The skin in all forms of breach of conduction can be shiny and atrophic (the "glossy skin" of the English), and shows then an increased vulnerability, so that the smallest injuries will not heal. Further, vesicular eruptions in the skin areas deprived of innervation, also falling off of nails, collections of air in the nail substance, hypertrichosis or falling out of the hair, and retardation of growth or thinning of bony parts are sometimes seen. SPECIAL SYMPTOM ATOLOCxY OF THE DEFECTS OF FUNCTION IX Till: DIFFERENT PERIPHERAL NERVES In order to understand the motor and sensory symptoms corresponding to lesions in the territories of distribution of definite nerves, an accurate acquaint- ance with the functions of the various peripheral nerves, as well as with those of the muscles supplied by them is, of course, necessary. A synoptic table which will present this information most expeditiously and in most condensed form is hence introduced at this point. 14 LECTURE I A. Plexus eervicalis Nervi cervicales N. phrenicus I. The Spinas Nerves 1. Motor Functions Musculi profundi colli Flexion, extension, turning the neck Mm. scaleni Raising the ribs on inspiration Diaphragm Inspiration B. Plexus brachialis N. thoracicus ant. M. pect. maj. and min. N. thoracic, long. M. serratus magnus N. dorsalis scap. M. levator scapulae Mm. rhomboidei N. suprascap. M. supraspinatus M. infraspinatus N. subscapular M. latissimus dorsi M. teres major M. subscapularis N. circumflex (axillaris) CM. deltoid ( M. teres minor N. musculo-cutan. M. biceps bracchi M. coraco-brachialis M. brachialis anticus N. medianus M. flexor carpi radial N. medianus N. ulnaris Nervus radialis (muscu- lo-spiral) M. palmaris longus M. flexor sublim. digitor M. flexor long, pollicis M. flexor prof, digit (ra- dial half) M. pronator radii teres M. abductor brev. pollicis M. flex, pollicis brev. M. opponens pollicis M. flexor carpi ulnaris M. flexor digit, prof, (ul- nar half) M. adductor pollicis Mm. hypothenares Mm. lumbricales Mm. interossei M. triceps bracchi M. supinator longus* Adduction, and drawing the arm down- ward and forward Fixation of the scapula on raising the arm Raising the scapula Drawing the scapula upward and inward Elevation and outward rotation of the arm Outward rotation of arm Inward rotation and adduction of the arm toward the back Inward rotation of arm Raising the arm to a horizontal position Outward rotation of the arm Flexion and supination of the forearm Elevation and adduction of the forearm Flexion of the forearm Flexion and drawing to the radial side of the hand Flexion of the hand Flexion of the middle phalanges of fin- gers II-V Flexion of the end phalanx of the thumb Flexion of the end phalanges of fingers II and III Pronation of hand Abduction of metacarp. I Flexion of proximal phalanx of thumb Opposition of metacarp. I Flexion and drawing to the ulnar side of the hand Flexion of end phalanges of fingers IV and V. Adduction of metacarp. I Abduction, opposition, flexion of little finger Flexion of proximal, extension of other phalanges Same action as preceding, also spreading apart and bringing together fingers Extension of forearm Flexion of forearm * The term "supinator longus" is, in fact, incorrect, since, as shown by electric stimulation, the muscle has no effect in causing supination, but produces rather slight pronation. The des- ignation "brachloradialis" would hence be preferable, but though used by anatomists, it has never been adopted by clinicians. DISEASES OF THE PERIPHERAL NERVES 15 B. Plexus brachialis C. Nervi thoracales D. Plexus lumbalis N. cruralis N. obturatorius E. Plexus sacralis X. gluteus sup, X. gluteus inf. X. ischiadieus (sciatic) (a) X. peroneus («) prof. (li) superf. (b) N. tibialis (ant.) M. extensor carpi rad. Extension and drawing to radial side of hand M. extensor digit, comm. Extension of proximal phalanges of fin- gers II-V M. extensor minim, digit. Extension of proximal phalanx of little finger M. extens. carp, ulnar Extension and drawing to ulnar side of hand M. supinator brevis Supination of forearm M. abductor pollicis long. Abduction of metacarp. I M. extensor pollicis brev. Extension of proximal phalanx of thumb M. extensor pollicis long. Abduction of metacarp. I and extension of end phalanx of thumb M. extensor indicis prop. Extension of proximal phalanx of index finger Mm. thoracis et abdom- Raising the ribs, expiration, abdominal inis pressure, etc. M. ileo-psoas M. sartorius M. quadriceps M. pectineus M. adductor longus M. adductor brevis M. adductor magnus M. gracilis M. obturator extern. M. gluteus med. \1. gluteus min. M. tensor fascia? lata? M. piriformis M. gluteus max. i~ M. obturator intern. Mm. gemelli ( M. quadratus femoris M. biceps femoris .M. semitendinosus M. semimembranosus M. tibialis anticus M. extens. lung, digit M. extens. halluc. long. M. extens. digit, brev, M. extens. halluc. brev. Mm. peronei M gastrocnemius M. soleus M. tiliialis'posticus M. flexor long, digit. M. flex, halluc. long. M. flex, brev. digit. M. Ilex, halluc. brev. Mm. plantares pedis X. pudendus Mm. perim teres ri gpbinc Flexion of the thigh Inward rotation of the thigh Extension of leg on thigh Adduction of the thigh Adduction and external rotation of the thigh Abduction and inward rotation of the thigh Flexion of the thigh External rotation of the thigh Extension of the thigh External rotation of the thigh Flexion of the leg Dorsal flexion and supination of the foot Extension of the toes Extension of great toe Extension of tin- toes Extension 'if great toe Dorsal flexion and pronation of foot Plantar flexion of the foot Adduction of the foot Flexion of the end phalanges II-V Flexion of mil phalanx I Flexi f tin- middle phalanges II-V Flexion of middle phalanx I Spreading, bringing together and flexion of Hie proximal phalanges of II"' Iocs Sphincter muscles of tin- pelvic organs, co-operation in the sexual act 16 LECTURE I In the first column of this table will be found the names of the different nerves, in the second, those of the muscles supplied by them, in the third the movements which are produced when these muscles act. The ability or in- ability to carry out these movements informs us as to the state of the respective muscles, whether they are paralyzed or not. The investigation of course must include a study of the electric contractility as well as that of voluntary movement. For this, an acquaintance with the electric motor points of the muscles is necessary. These are shown — as they usually occur in normal persons — in Figures 3 to 8. In the investigation of pathological M. splenius M. sternocleidomastoideus M levator anguli scapul N. dorsalis scapul N. axill N". thoracicus longu; ) The mucous membrane of (b) The mucous membrane of iris the antrum and the lower the cheeks, the lower jaw, parts of the nose the floor of the mouth, the tongue (c) The mucous membrane of (c) The mucous membranes of (c) The lower teeth the frontal sinuses and the the upper jaw and of the upper parts of the nose palate to the palatopharyn- geal arch (d) The upper teeth In total or partial destruction of one of the three branches, there is of course anesthesia or hypoesthesia in the corresponding parts. Further, in lesion of the ophthalmic branch, there is loss of the conjunctival and corneal reflex (closing of the eye on touching the conjunctiva or cornea with a blunt object, for instance, the head of a pin, also of the reflex of sneezing produced by tickling the upper part of the nasal mucous membrane. In lesion of the max- illary branch the sneezing reflex is absent when the lower part of the nasal mucosa is irritated, as is also the palate reflex, that is the movements of swallow- ing or of retching produced by tickling the soft palate. It is to be remarked here, however, that all of these three reflexes are inconstant and subject to great individual variations. Hence only the absence of these phenomena is of diagnostic importance. It is well to recall, too, that the sensation of pricking perceived on inhaling ammonia or acetic acid is produced not through the olfactory nerve hut by irritation of sensory ter- minations of the trigeminus. If the trigeminus is out of function these do not occur, neither do the reflex Bymptoms produced through if (watering of the eves, changes in the pulse, arrest of respiration). Besides conducting common sensation the trigeminus contains also fibers for special sense. The mandibularis, through one of its branches, the lingualis collects the taste fibers from the anterior two-thirds of the tongue; these pro- ceed then through the chorda tympani to the facial trunk, to return again to the trigeminus after they have run some distance in the facial. Since disturb- 30 LECTURE II ances of taste (ageusia, hypogeusia) occur most commonly in facial paralysis, we will postpone the discussion of the methods of testing taste, until we come to speak of the facial nerve. The motor trigeminus fibers are included in the third branch and are distributed to the muscles of mastication, the anterior belly of the digastric, the mylohyoid, the tensor tympani and the tensor palati muscles. Unilateral paralysis of the muscles of mastication (masseter, tem- poral, pterygoids) is called monoplegia masti- catoria. In this, lateral movement of the lower jaw is only possible toward the para- lyzed side-, since only on the sound side are the pterygoids still able to contract. By palpation the absence of contraction in the masseter and temporal of the affected side can also be noted. In diplegia masticatoria, the lower jaw drops and all lateral motion is impossible. Further, there is no jaw reflex — the contraction of the masseters on striking upon the finger laid upon the lower row of teeth, which can be obtained in most normal individuals. The paralysis of the anterior belly of the digastric and of the mylohyoid, sometimes produces a sense of relaxation on palpation in the muscles of the floor of the mouth on the paralyzed side; we have little informa- tion as to the symptoms of loss of function on the part of the tensor tympani and the tensor palati. It is certain that in most cases they remain latent ; here and there, however, there appear to be anomalies in the position of the palatopharyngeal arch and poor hearing for low tones. Besides the motor and sensory fibers mentioned, immediately after their exit from the skull all of the three divisions of the trigeminus have sympathetic fibers added to them, and these fibers accompany these divisions and their branches in their further course. The meeting points of these sympathetic fibers (they arise altogether from the plexus accompanying the head arteries) with trigeminus neurones, are at certain nodes or ganglia (ganglion ciliaris, sphenopalatinum, oticum, linguale). Hence, under some circumstances, a lesion of the first division can have as a result a narrowing of the palpebral fissure and a myosis of the pupil on account of paralysis of the tarsalis superior and dilator pupilla? muscles innervated by the sympathetic, a lesion of the second division, by paralysis of the orbital muscle at the base of the orbit, an "enoph- thalmus," that is, a sinking in of the eyeball. All of the three branches carry sympathetic fibers to the blood vessels and sweat glands, on which account in interruption of conduction we almost always find in the anesthetic parts heat The Sensory Nerve Supply c Head Hatched: Trigeminus 1. The ophthalmic branch 2. The maxillary branch 3. The mandibular branch White: Vagus v. Nervus auricularis ,-agi Black: Cervical nerves O, Great occipital nerve o, Lesser occipital nerve A, Great auricular nerve DISEASES OF THE PERIPHERAL NERVES 31 and redness (in fresh cases) or cyanosis and coldness (in older cases), as well as anidrosis. Other neurones originally sympathetic, but running in the trigeminus, which must be mentioned, are fibers regulating the secretion of the nasal mucus, contained in the ophthalmic and maxillary divisions. Upon their failure to functionate depends the abnormal dryness of the nasal mucosa which occurs in paralyses of these nerves and leads secondarily to reduction of the sense of smell. Finally, there are other secretory fibers coming from another cranial nerve, the facial, the already mentioned lingual nerve containing those for the salivary secretion, the lacrymal nerve (a branch of the ophthalmic) those for the tear secretion. Hence these secretions can sometimes be affected, in troubles of the fifth nerve. 6. The Abducens. — To the sixth nerve belongs the motor innervation of Fig. 21. Bilateral Abducens Paralysis. the external rectus muscle which turns the eye about a vertical axis outward, abducts it. Isolated abducens paralysis has Inner as a result a deviation of the affected eye inward (see Fig. 21). Winn the abducens is affected, together with the oculomotorius and trochlearis (ophthalmoplegia totalis), the eve remains immovable, directed to the front. 7. The Facial. — This nerve supplies all the muscles of the lace (including the buccinator, excepting the levator palpebral superioris, which is innervated Dy the oculomotor) from the frontal muscle to the pla t vsma mvoides, and besides these, the stylohoid, the posterior belly of the digasl ric and the stapedius. The failure of the motor function of the facial nerve produces the clinical picture of facial paralysis (<>r prosoplegia) which when unilateral is denomi Dated monoplegia facialis (see Figs. '2'2 and 28), when bilateral, diplegia facialis. It is also called Bell's paralysis. The paralyzed half of the face is devoid of the mimic movements, masklike, immovable, expressionless; the naso labial fold 32 LECTURE II is obliterated, the forehead cannot be wrinkled, the eye— on account of the paralysis of the orbicularis palpebrarum — cannot be closed (lagophthalmus), the angle of the mouth hangs down. The falling downward of the back portion of the tongue betrays the paralysis of the stylohoid and the posterior belly of the digastric, an abnormal acuteness of hearing and a sensitiveness to deep tones (oxyakoia, hyperacusis), that of the stapedius whose function it is to close the fenestra ovalis of the drum cavity by drawing on the stapes. From the neighborhood of its origin also, the facial trunk carries along with it other sorts of centrifugal fibers, namely those for the tear secretion and those for the .salivary secretion; both of these sets of fibers as we have already heard, enter Fig. 22. Left-Sided Peripheral Facial Paralysis. the trigeminal nerve, while on the other hand the taste fibers from the anterior two-thirds of the tongue leave the trigeminus for some distance and join the facial. Fig. 24* will bring before you the anatomical relations of this somewhat complicated exchange of fibers. They contribute chiefly to the fact that according to where a break in the conduction in the peripheral course of the facial has occurred different clinical symptoms develop. I have presented these in tabular form on page 33. Testing of sense of taste on the tongue is carried out in the following manner. The subject is instructed to tell the taste of the solutions which are applied to his outstretched tongue without drawing it back into his mouth again. These precautions are to prevent the falsification of the results by their perception by the mucous membrane of other parts of the mouth which are innervated by the glossopharyngeal. A card bearing the words "bitter, sour, DISEASES OF THE PERIPHERAL NERVES 33 salty, sweet," is placed before the patient and he is asked to indicate upon it what he tastes as his tongue is touched successively by cotton brushes wet with ' tinct. gentian, vinegar, salt solution, and syrup. After each test the tongue must be washed off with a wisp of wet cotton. The eyes are best covered during these tests. BREACHES OF CONDUCTION' IX THE FACIAL NERVE. a. Outside of the cranial cavity. Almost exclusively monoplegia facialis (Exception: facial paralysis from double otitis). a. Distal to the origin of the chorda tympani. (Area 1, Fig. 24.) Symptoms: paralysis of the facial muscles; if the lesion is very far toward the periphery, that is beyond the pes anse- rinus, some of the branches may escape. b. In the Fallopian canal, between the chorda and the origin of the stapedius (Fig. 24, 2). Symptoms: facial paralysis; loss of taste on the anterior two-thirds of the tongue; salivary secretion altered. e. In the Fallopian canal between the origin of the stapedius and the geniculate ganglion (Fig. 24, 3). Symptoms: facial paralysis, loss of taste on the anterior two-thirds of the tongue, salivary secretion altered, hyperacusis. d. Between the meatus acusticus interims and the geniculate ganglion (Fig. 24, 4). Symptoms: facial paralysis, no taste disturbance, alter- ation of the salivary secretion, often nerve deafness (see below) through involvement of the auditory, only when this is not present, hyperacusis, loss of the affective and reflex tear secretion. b. Within the cranial cavity — "basal" lesions of the facial. Not infre- quently diplegia facialis (basal gummatous meningitis). Symptoms: as above under J generally involvement of a number of basal nerve roots (al)dueens, glossopharyngeus, vagus, accessorius, hypoglossus) ; general brain symptoms (vertigo, vomiting, headache). 8. The Audit or i/. — The eighth cranial nerve is made up of two portions each of differenl function: the nerve of hearing proper, the cochlear, and the nerve of orientation in space, the vestibular. The firs! stands in connection with the cerebrum, the latter with the cerebellum, the organ for the preserved ion of the equilibrium. Loss of function of the cochlear nerve produces impairment of hearing to complete deafness (hvpacllsis or anacusis ) . Since, however, these disturb- gmces may also be produced by affections of the sound-conducting apparatus, of the middle or of the outer ear,* the chief characteristics of "hardness of * A lesion in the perceptive apparatus in the cochlea of the lahyrinth (thai is. In the organ of Corti) gives the same symptoms as a breach of conduction in the cochlear nerve. 34 LECTURE II hearing or deafness of nerve origin" must be considered. These are: 1. The reduction or loss of hearing through bone conduction. 2. Partial loss of per- ception for the notes of the scale. The first of these phenomena is determined, a, by Schwabach's test. In this the time during which a vibrating tuning-fork placed upon the parietal bone, the teeth, or the mastoid process, can be heard, on the one hand, by the person under examination, and on the other by the normally hearing examiner, is com- pared. In impairment of hearing from nerve lesion, there is shortening of the period during which the sound can be perceived through bone conduction; in Fig. 33. Left-Sided Peripheral Facial Paralysis, Upon Attempt to Close Both Eyes. nerve deafness bone conduction may be lost. In middle ear affections on the contrary the tuning-fork applied to the bone is heard longer than normal. This method is naturally only applicable in bilateral disturbances of hearing. b. By "Rinne's test." — In this, a vibrating tuning-fork is placed upon the mastoid process; when the patient ceases to hear it there (that is, bone con- duction is no longer perceived), the fork is transferred to opposite the ear. The normal person now hears the sound anew and this is denominated a positive result ("Rhine positive"). In disease of the sound-conducting apparatus (where the perception through air conduction is diminished, but through bone conduc- tion unaltered) in the second part of the test, sound is no longer perceived, it is negative. In nerve "hypoacusis" on the contrary, there is, as a rule, "positive Rhine" DISEASES OF THE PERIPHERAL NERVES 35 provided, of course, that there is not a high degree of defect of hearing. In this latter case, as in nerve deafness, hearing through air conduction is con- siderably reduced or lost. c. Bv "Weber's test." If in a normal individual a vibrating tuning-fork is placed on the vertex it is heard in both ears; if now one ear is plugged up, the sound is "lateralized" on the side upon which air conduction is in this manner To the N. lacrymal. trige G. = Gangl. genl Fig. 34. Diagram of the Different Categories of Fibers in tlie Facial Nerve. interrupted. This "lateralization" of the sound occurs spontaneously on the side of impaired hearing in persons with diseases of the sound conducting ap- paratus ("positive Weber"), Wmle the person with nerve deafness lateralizes t<> the -.111111(1 side ("negative Weber"). Defects in the perception of the notes of the scale .ire tested by the so-called Galtoris whistle. Deafness for the upper notes of the scale in particularly characteristic of affections of auditory nerve tracts as opposed to those of the middle and outer ear. In "nervous hardness of hearing" the perception of words witli sharp consonants and clear vowels (as sheriff, swagger, fish, wasp), 36 LECTURE II suffers especially, while those with dull consonants and vowels (as brooder, hunger, ore, worm*) are much better understood. The opposite is the case in non-nervous hypoacusis. The diagnosis of nerve hardness of hearing and deafness must be made in general by exclusion, after examination of the drum membrane, when the hearing is not improved after Pollitzerization of the ear, etc. As to the value of that peculiar phenomenon, the so-called paracusis of Willis for the diagnosis of nerve deafness, opinions arc divided. Many otologists are of the opinion that nerve deafness paradoxically makes itself less felt in a noisy place (in a railway car) than in a quiet one; according to others still, in middle ear disease "Paracusis Willisii" can also occur. The vestibular nerve transmits to the cerebellum information as to the position in space of the body, especially of the head. The sensory epithelium of the ampulla 1 , the utricle and the saccule of the labyrinth are stimulated to function through the hydrostatic relations of the fluid in the semicircular canals (which are arranged according to the three planes of space). If now there is a contradiction between the condition of stimulation and the actual position in space of the body, the resulting confusion gives to the patient the illusion of rotary movements, either of his own body or of surrounding objects, which we denominate rotary vertigo. The last can arise from interruption of conduction in the vestibular nerve and then shows itself sometimes by moderate uncertainty of gait and difficulty in turning, as well as by holding the head stiffly. We denominate "'galvanic vertigo," a manifestation which appears, when in the person under investigation, a constant current of gradually increasing intensity is conducted through the head at the level of the ears. When a cur- rent strength of about 5 to 6 milleamperes is reached, the normal person has the feeling that he is going to fall toward the side on which the positive pole is, and usually inclines his head to that side. If one vestibular nerve is out of function, however, in many cases the patient in this experiment has each time the feeling that he is sinking toward the side of the injured nerve regardless of the position of the anode or the cathode. More applicable to the clinical investigation of the vestibular apparatus is Barony's test, that is, the examination for caloric nystagmus. Each vestibular apparatus is in anatomical and physiological connection with the eye muscles through the fasciculus longitudinalis posterior, on account of which, irritation of the ear by syringing with hot or cold water produces rhythmical horizontal movements of the eye. (Probably through the differences of temperature there are produced movements of the endolymph, which irritate the nerve structures of the labyrinth.) In healthy persons, the syringing of the ear with cold water produces nystagmus toward the opposite side; if hot water is used, the nystagmus on the contrary is toward the side of the syringed ear. If the vestibular nerve is paralyzed, no caloric reaction can be produced from the corresponding ear. The healthy side generally reacts normally, though occa- sionally irritability on this side is also somewhat reduced. 9. The Glossopharyngeal Nerve. — This nerve is mainly sensory? but has a * For the German words given by the author, English words of as similar sound as possible have been substituted. — Translator. DISEASES OF THE PERIPHERAL NERVES 37 motor division which supplies the stylopharyngeus muscle, an elevator of the pharynx. The glossopharyngeal transmits sensory impressions from the upper- most part of the pharynx and from the middle ear, as well as taste perceptions from the palate and from the posterior third of the tongue. On this account, ageusia in the last-mentioned region is the most important symptom of its loss of function ; also an anesthesia of the pharynx can be recognized, as well as loss of the pharyngeal reflex. This last, however (in correspondence with what was mentioned in connection with the palate reflex), can only be consid- ered as a sign of breach of conduction in the glossopharyngeal when there is a difference between the reflexes on the two sides. The stylo-pharyngeus muscle, as elevator of the pharynx — in which function it unites itself with the pharyngo- palatine, supplied by the vagus — is of too little importance for motor symptoms to occur from its elimination alone. 10. The Vagus (or Pncumogastric) Nerve. — The vagus is a mixed nerve. On the one hand it supplies the muscles of the palate, pharynx, larynx, trachea and bronchi, as well as those of the oesophagus, stomach and small intestine, with motor fibers, and carries inhibitory fibers for the heart and vasomotor fibers for numerous vessels ; on the other, it is the sensory nreve for the dura mater, the external auditory canal,* the lower portion of the pharynx, the Larynx, the trachea and bronchi, the oesophagus and the stomach. Total dou- ble paralysis of the vagus has no symptomatology, since it is incompatible with further existence. On the other hand, a partial double, or a total single vagus paralysis, can occur clinically. In the last case there is a unilateral paralysis of the uvula, pharynx and larynx. One-half of the velum palati hangs flaccid down, on which account the voice becomes nasal. One vocal cord is immovable in the middle or the cadaveric position, since both the closing and the opening muscles of the glottis are put out of action. Still, the voice may remain normal through compensatory overaction of the other vocal cord. Usually, indeed, it is somewhat hoarse, changing to falsetto. On the other hand, disturbances in swallowing are almost always minimal, since hemi- pharyngoplegia, on account of the mutual interlacing of the muscular fibers of the pharynx, is without great functional importance. An increased fre- quency of the heart beat — a tachycardia — has very seldom been observed in unilateral loss of function of the vagus ; tin- same thing is true with regard to disturbances of respiration (in the sense of slowing and irregularity of the breathing). In complete paralysis of the vagus, only a few of the .above- related symptoms are present, sometimes these, indeed, are only partially de- veloped. So, instead of total paralysis of the vocal cords, there is only paraly- sis of the posterior cricoarytenoid, the opener of the rima glottidis. (See Fig. 25, C.) From this "posticus paralysis," when it is bilateral, there results an interference with respiration, while phonation is normal. 11. The Spinal Accessor;/ Nerve. This nerve alone supplies the sterno- cleidomastoid muscle, while in the innervation of the trapezius it is assisted by •The researches of J. /.'. Hunt seem to show thai the facial nerve has also a sensory nini which supplies a cone-shaped area including the external auditory canal and a mal] portl >f the outer ear. — Translator. 38 LECTURE II the upper cervical nerves. If the spinal accessory is destroyed, there is a com- plete paralysis of the sternocleidomastoid. Hence, in one-sided lesion, it is impossible to turn the chin completely toward the opposite side, while in bi- lateral paralysis there is also a tendency for the head to fall backward. On the other hand, the paralysis of the trapezius is incomplete and makes itself evident only in impaired force and extent of the elevation of the arm. 12. The Hypoglossal Xerve. — The hypoglossus can be described briefly as the nerve supplying the tongue muscles. Indeed, its part in the innervation of the lower muscles attached to the hyoid bone (sternohyoid, sternothyroid, omohyoid), which serve in part to fix the larynx, is only apparent. In the so-called "ansa hypoglossi," through which this innervation occurs, there run fibers from the cervical nerves, which only enter the hypoglossus through Image of the Vocal Cords on Inspiration. A. Normal. B. Left-sided vagus paralysis (Kecurrens paralysis). C. Bilateral posterior arytenoid paralysis. anastomoses. Bilateral paralysis of the hypoglossus, of course, produces a total "glossoplegia" ; following the influence of gravity the tongue lies im- movable upon the floor of the mouth; speech is unintelligible, eating is consid- erably interfered with. In unilateral paralysis (hemiglossoplegia), on the other hand, the motor disturbance is relatively small; indeed, talking and eat- ing are usually not at all, or scarcely, disturbed. This is on account of the multiple interlacements between the muscle fibers of both sides of the tongue. If, on the contrary, the patient is asked to show his tongue, a deviation of the tip of the tongue toward the side of the paralysis occurs, on account of the preponderance of action of the genioglossus on the sound side. The Occurrence of Peripheral Interruptions of Conduction in the Cranial Nerves Although in Lecture I (in speaking of the etiology of disturbances of con- duction in peripheral nerves) we had the cranial nerves also in mind, and for example, considered rheumatic facial paralysis, I will, in this connection, bring before you the conditions under which paralyses of a peripheral nature of indi- vidual cranial nerves come under observation. According to the localization of interruption of conduction of this nature, in a given cranial nerve, we must take into consideration certain etiological factors. The olfactory nerve is involved most frequently in injuries to the head with DISEASES OF THE PERIPHERAL NERVES 39 or without fractures of the skull, but also in tumors, abscess and meningitis in the anterior fo.ssa T)f the skull. The optic nerve can be affected in syphilis, acute infectious diseases, poison- ing (e.g., with lead, arsenic, nicotin, santonin, iodoform, quinine, alcohol), in severe anemias {e.g., after hemorrhages from the stomach or metrorrhagia), in auto-intoxications (diabetes, nephritis), also by neoplasms, whether these arise from the nerve itself or from neighboring structures {e.g., from the orbit) ; finally, it is not seldom wounded in attempts at suicide (by a shot in the temple). Etiological factors for disease in the course of the eye-muscle nerves are: fractures at the base of the skull (the abducens is especially endangered on ac- count of its exposed position at the point of the pyramid of the petrous bone) ; compression by aneurisms of the internal carotid or by tumors ; otitis media (abducens paralysis); acute infectious diseases; diabetes, syphilis, nephritis, gout, alcoholism, chronic lead poisoning. Peripheral interruptions of conduction in the trigeminus rarely affect sin- gle branches only; apart from injuries of the bones and the soft parts of the face, in the main only tumors, tuberculous and syphilitic processes of the bones which contain the foramina of exit of the nerves, and of their periosteum come into consideration as causes. The Gasserian ganglion and the common trunk of the nerve are, however, much more frequently affected and, indeed, almost always unilaterally (aneurisms of the internal carotid, tumors of the internal carotid, transverse basal fractures behind the sella turcica, hypophysis tumors, etc.). The facial, as said before, is by far most frequently affected by "rheu- matic" influences; that is, without any other recognizable etiology than ex- posure to cold. In "idiopathic facial paralysis" even this etiological factor is sometimes not to be made out. Other causes are: otitis media, meningitis, tumors in the posterior fossa of the skull, diseases of the petrous bone, syphilis, erysipelas, diphtheria, protective inoculation against, rabies. Finally, are to be mentioned traumata, which are quite common in the course of the facial nerve ( fall against a window-pane, sabre cuts in duels). The auditory nerve is affected by tumors, diseases of the meninges and C&ries of bone in the posterior fossa of the skull, as well as by inflammations of the middle car, corresponding to its close relations with the facial nerve, quite as frequently as this latter. Also fractures of the base of the skull some- times involve it. Besides different acute and chronic infectious diseases (typhoid fiver, diphtheria, influenza, scarlel fever, parotitis, tuberculosis, syphilis), leu- cemia lias been specially mentioned as an etiological factor in auditory neuritis. Isolated paralysis of the glossopharyngeal nerve scarcely ever occurs. Other nerves are usually involved at the same time, particularly the vagus, especially where the interruption of conduction is of traumatic origin (fractures of the base, tumors, the pressure of aneurisms, or sinus thromboses, etc.). The vagus can be a Heeled Iii many kinds of poisonings {e.g., carbonic oxide, morphine, atropin, phosphorus, lead, arsenic, alcohol), as well as in connection with dif- ferent acute infectious diseases. Paralysis of the recurrent laryngeal nerve is, as a rule, caused by local anomalies in the neck or in the mediastinum (si ruina, tumors, enlarged glands, etc.). 40 LECTURE II The spinal accessory and hypoglossals are particularly exposed to trau- matic injury and upon the basis of the same causal factors as the glossopharyn- geal and vagus. Hypoglossal neuritis occurs also in intoxications (alcohol, lead, arsenic, carbonic oxide). B. Special Remarks on Neuritis and Polyneuritis I. IRRITATIVE SYMPTOMS While we might consider disturbances of conduction, whose general and spe- cial phenomenology we have already learned, as a general characteristic, as well of traumatic and neoplastic, as of neuritic diseases of the peripheral nerves, we must now enumerate the irritative symptoms, which play a not less important role in the clinical picture of the last-mentioned nosological group. They manifest themselves to by far the greatest extent as disturbances of sensation: that is, as pain along the course of the affected nerves. They may have a shooting, stabbing, or boring character, are sometimes described as a burning on the surface of the skin (the "causalgia" of Weir-Mitchell). From these pains proper, they pass by imperceptible degrees to the "paresthesias," the more or less painful abnormal sensations which are often felt in the terri- tory of the affected nerves: formication, prickling, tickling, subjective feeling of cold or heat, etc. Also hyperesthesia to touch, on account of which the pres- sure of the clothing or of the bedclothes may be unbearable, is not infrequent. Here and there, also, spontaneous pains are projected into skin regions in which, by objective tests, touch and pain sense have been proved to be reduced (hypesthesia dolorosa, hypalgesia dolorosa). Further, it sometimes occurs that pains, at least for a time, are located in the joints, and so arthritic dis- eases are simulated. The irritation of fibers conducting centripctally can ex- ceptionally be accompanied by exaggeration of reflexes. The conditions under which the influence of the disease in exaggerating the reflexes, overcomes the effect of lesions of the afferent or efferent part of the reflex arc in destroying the reflexes, are not clear to us. Perhaps in such cases the perineuritic processes predominate anatomically over the parenchymatous, since the spontaneous pains may well depend upon perineuritic changes. That in general, the irrita- tive phenomena in the centripetal fibers (pain, hyperreflexia) occur most usu- ally in acute neuritides and polyneuritides, agrees with what was a priori to be expected. Motor irritative phenomena are, on the other hand, very infrequent, and have been described almost exclusively in multiple neuritis, e.g., cramps in the calves in localization of the disease in the legs. Babinski asserts that there is often a latent predisposition to cramps in the calves which can be provoked by faradization ; I have so far not been able to convince myself of this. On the other hand, I saw a peculiar muscle wave ("myokymia"), which has also been corroborated by Remdk, once in weak development in the peroneal muscles in a tuberculous polyneuritis, very plainly in the biceps brachii in an alcoholic neuritis. DISEASES OF THE PERIPHERAL NERVES 41 II. PALPATORY FINDINGS Abnormal findings by palpation of superficially situated nerves are quite frequent. In the first place, there is more or less marked sensitiveness, so that pain is produced by moderate pressure — upon the brachial plexus above the clavicle or in the axilla, upon the nerve structures in the internal bicipital sulcus, upon the ulnar on the epicondyle, upon the musculo-spiral in the groove on the outside of the humerus, upon the peroneal behind the head of the fibula, upon the sciatic between the flexors of the knee, etc. A certain familiarity with the intensity of the pressure which normally is necessary to produce pain in these different places is needed in testing for this phenomenon ; for example, when it is necessary to elicit it by comparison with the healthy corresponding nerve on the opposite side (in polyneuritis). Less frequent than abnormal sen- sitiveness to pressure, but by no means infrequent, a thickening of the nerve trunks can sometimes be felt upon palpation. This can well be the expression of a perineuritic process ; such a process appears to be the cause of the sensi- tiveness to pressure of nerves afflicted with neuritis, which can occur in purely motor branches and is in this case attributable to the irritation of the "nervi nervorum." In order to determine by palpation that there is a definite swelling of a nerve, industrious practice in palpation of normal peripheral nerve trunks is even more necessary than in the testing of sensitiveness to pressure. It is well, also, not to lose any opportunity which is presented to control one's pal- patory findings anatomically. For example, I have found on autopsy, or on operative exposure, nerves which appeared swollen by palpation actually two or three times as thick as the corresponding portions of normal nerves. III. THE DIFFERENT CLINICAL FORMS The neuritides may be grouped according to different principles ; for exam- ple: 1, according to their course into acute, subchronic and chronic; 2, accord- ing to the nature of their symptoms into motor, "mixed" and sensory; .'5. ac- cording to their extent, into mononeuritides, disseminated neuritides, plexus- neuritides and po] yneuritidcs.* The acute neuritic diseases belong for the greater part to the etiological * Of late attention has been directed to the "root-neuritides" ("radiculitides"). These conditions differ anatomically from the ordinary neuritides by being located proximalrj to the plexuses for the extremities— that is, in the region of the spinal nerve routs; clinically, by the t'.iet thaPthe pains arising in consequence of them an- located, not in the territories of the peripheral nerves, hot in the spinal segmentary areas of innervation. (See, farther cm. Lecture XI.) Etiologically, infections and intoxications play the chief role, as in ordinary neuritides. Ordinarily the disease manifests itself in two phases: :[ stage of irritation with attacks of pain, hyperesthesia of the shin, and sometimes exaggeration of reflexes, and a stage of destruction with anesthesia, paralysis and absence of reflexes. The pains have the "lanci- nating" and specially severe character typical for radicular affections {e.g., for tubes or carcinoma of the vertebra; — sec below). They are often sel up bj movements or by over- exertion — in specially characteristic manner by sneezing. Sensitiveness to pressure, or u| palpation of accessible peripheral nerves, i.s not present or scarcely perceptible. Painful points nvcr the spinous processes are recognizable, however, 42 LECTURE II group of infectious and rheumatic neuritides ; they also arise, however, upon the basis of intoxications ; for example, in lead, arsenic, and carbonic oxide poisoning. In my first lecture I mentioned a case due to the combined action of bromide of methyl and methyl alcohol, which had an acute course. The so-called "acute curable ataxia" of drinkers is a by no means seldom form of alcoholic polyneuritis ; an "apoplectiform alcoholic paralysis" was first shown on autopsy, by Eichhorst, to be a polyneuritis beginning hyperacutely. Cer- tain polyneuritides which ascend from the lower extremities in "foudroyant" manner and rapidly cause death by paralysis of the heart and respiratory nerves are denominated the "neuritic form of Landry's paralysis"; if an infectious basis is here the rule, this unfavorable course can also be a manifestation of alcoholic polyneuritis. Chronicity distinguishes in general the arterio-sclerotic, senile, anemic, cachectic, diabetic, and also the majority of alcoholic and tuber- culous neuritides and polyneuritides. In carrying out the division into motor, "mixed," and sensory neuritides, it is at once apparent how infrequent the last are; apart from the neuritis of Fig. 26. Tuberculous Polyneuritis. General Muscular Atrophy. the external cutaneous nerve of the thigh, the so-called "paresthetic meral- gia," we find in the literature very few descriptions of sensory neuritides {e.g., isolated neuritis of the lateral cutaneous peroneal, of the internal cutane- ous of the thigh, of the chief sensory branches of the radial), and also those polyneuritides in which every electrodiagnostic and dynamometric sign of an in- volvement of the motor fibers is absent, form a quite small minority. Pure motor neuritides are, on the other hand, more frequent and most remarkable, even when the affected nerves contain sensory fibers. A certain "electivity" in the action of the disease-producing cause, which we will consider more fully farther on in this lecture, is evident in these cases. Nevertheless, I have found, that by the use of finer methods of testing sensibility {e.g., by the tuning- fork), a large number of mononeuritides and polyneuritides, which at first sight appear to be purely motor, later turn out to be of the "mixed" form. The above mentioned "meralgia paraesthetica" is a not so rare clinical picture described by Rotli and Bernhardt. The peculiar relations of the external cutaneous nerve in its passage through the fascia of the thigh, appear to pre- dispose this nerve to destructive lesions. As exciting causes, infectious diseases, overexertion, abnormal static relations (flat foot) diabetes, arteriosclerosis DISEASES OF THE PERIPHERAL NERVES 43 and nicotinism, have been mentioned. The disease is usually unilateral. The patients complain of the most varied paresthesias and spontaneous pains in the region of distribution of the external cutaneous nerve; often certain points are also very sensitive to pressure. The hyperesthesia of the skin can cause the friction of the clothing to be painful. Objectively hypesthesia or anesthe- sia in a more or less extensive portion of the territory in question is found. Meralgia is in general persistent and shows a tendency to relapses. We speak of mononeuritis when the territory of only one nerve is affected, of total or partial plexus neuritis when a nerve plexus, for example the brachial plexus, is attacked in its entirety or witli the escape of single branches. In a disseminated neuritis single nerves are attacked at the same time without any regularity in the distribution and development of the paralysis. In contradis- tinction to this, we reserve the term polyneuritis for cases of more or less diffuse disease of the peripheral nervous system, which present a nearly sym- metrical distribution and run a regular course. We will now devote some space to these forms. According to their extent, poh-neuritides may be divided into those of paraplegic or tetraplegic type, those which run their course as symmetric arm plexus neuritis without involvement of the legs, and finally those in which the cranial nerves are exclusively attacked or are affected along with the others. It is noteworthy that according to the special etiology, different topographical and physiopathological peculiarities are regularly present. Lead neuritis avoids the sensory element and affects (where by overexertion of special groups of muscles the localization is not influenced in the sense of the "toxico-pro- fessional" paralysis of Oppenheim (sec above), particularly the muscles of the forearm supplied by the radial nerve; very rarely, generalized lead paral- yses, in which loss of motor power affects the territory of one nerve after another symmetrically, either in exacerbations, or in rapid succession, some- times under febrile movement, occur. In these, however, the predisposed muscle groups are more seriously affected than the others. Arsenical polyneuritis, which usually produces both motor and sensory symptoms, as far as its motor components are concerned, presents in so far the contrary of saturnine polyneuritis in th.it the lower extremities are attacked by preference. Among 72 cases of Brouardel's they were affected first in 69 cases, exclusively in .'55 cases. However, when the anus are affected, the atrophic paralysis of the ulnar and median nerves by far predominates, while the radial paralysis typi- cal of lead intoxication occupies the background. Characteristic of diphtheritic polyneuritis is, on the one hand its preference for certain centripetal nerve fillers from the lower limbs, which lead to a "post diphtheritic ataxia"; on the other a decided affinity for motor nerves from the proximal parts of the cerebro- spinal apparatus. Wwr the paralysis of the palate and the muscles of accom- modation of the eve ( the ciliary) by far predominate. Fortunately the pharyn- geal, diaphragm, and heart paralyses, so dangerous to life, are rare; the exten sion of the polyneuritic process to the innervation of the neck and external eye muscles is also rare. I only once observed a symmetrica] accessorius paral- ysis. However, diphtheritic paralyses of tin- muscles of the extremities, which arc guite inconstant in their topographical distribution, also occur. The 44 LECTURE II polyneuritides from the inhalation of sulphide of carbon affect mainly the flexors of the hand and the extensors of the foot. These few examples may serve as instances of the characteristic choice which certain nerve poisons exer- cise, with noteworthy regularity, in the production of polyneuritic symptom- complexes. This "electivity" speaks in the sense of Ehrlich's doctrine of "organotropy," for the idea that certain poisons possess certain chemical affinities for certain cell substances. According to the chemotherapeutic investigations of Ehrlich the different arsenic preparations, for example, may be arranged in an almost continuous scale as regards their "neurotropy." In spite of this, in the assump- tion of an elective chemical action of the poisons causing neuritis, we come up against the fact that clinically as well as anatomically, the symptoms mani- fest themselves preponderantly or exclusively in the periphery of the neurones in question. A second factor comes here into the question: the greater the distance from the trophic centre (that is, from the spinal cord and the spinal ganglia) the less the power of resistance of the neurone to the action of toxic substances. Here the "Ersatz" theory of Edinger, already mentioned in Lec- ture I, can with advantage be taken into consideration. LECTURE III Diseases of the Peripheral Nerves C. The Neuralgias Pains which occur in attacks and radiate throughout the territory of dis- tribution of peripheral nerves we call neuralgia.* As to the material basis of this characteristic symptom, we are very poorly informed ; however, in the vast majority of cases in which the nerves attacked have been examined mi- croscopically, they have been found entirely normal. Oppcnhcim suggests with justice, that fine disturbances of nutrition of the nerve (perhaps also of its nervi nervorum) lie at the base of the neuralgias, disturbances which only exceptionally lead to visible alterations, which then approach the anatom- ical picture of neuritis. The cause of these disturbances of nutrition is only in a minority of the cases of mechanical nature; for example, a stasis of the veins accompanying the nerve, through which the latter is compressed in a bony canal or in some other channel (Bardenheuer) , as perineuritic adhesions, osteitis, periostitis, tumors, cicatrices, etc., which exert a traction or com- pression upon the nerve, or, finally, a trauma ; usually, however, general toxemic influences are to be held responsible. Of the infectious diseases, influenza, malaria and syphilis play the greatest role in the etiology of the neuralgias; of diseases of metabolism, diabetes and gout, of other auto-intoxications, chronic constipation, of exogenic poisonings, alcoholism. Undoubtedly the "nervous" (viz., neurasthenics and hysterics), are just as much predisposed to neuralgias as anemic and chlorotic individuals. Further, the influence of refrigeration, especially of circumscribed nature, as, for example, a draught 01 e side in trigeminus neuralgia, sitting on cold stones in sciatica, is not to be denied. I have already indicated what can be said about the rheumatic origin of diseases of the peripheral nerves. Finally, the so-called "reflex" neuralgias are to be considered; problematic is this method of origin for such cases in which facial neuralgias have been brought into causal connection with a retroflexion of the uterus. Better founded, on the contrary, are the very frequent connection of trigeminal neuralgia with Uncorrected anomalies of refraction of the eve, caries of the teeth, suppuration of the middle ear, catarrhs of the frontal sinus and of the ant ruin of High/more, that of sciatica with flat foot, etc. Puberty and the period of retrogres * There are, indeed (.-is complement to the root neuritides described in the footnote on page II), radicular neuralgias (root neuralgias, radiculalgias) in which the pain radiates, not in the anatomical distribution 01 the peripheral nerves, I < 1 1 1 presents the character of the lancinating pains of tabes dorsalis, to be described in Lecture XI, 45 46 LECTURE III sive change, seem the times of special predisposition for neuralgias. Men are affected much more frequently than women ; nevertheless, during pregnancy and in the puerperium, the latter are decidedly more susceptible. THE GENERAL SYMPTOMATOLOGY OF NEURALGIA The attack of neuralgic pain either appears with lightning-like suddenness, or unpleasant sensations (a feeling of heat, of tension, of formication, etc.), in the distribution of the nerve affected, precede it by some time. Between the single attacks there is either entire freedom from pain, a dull pain of little intensity, or a feeling of irritation and soreness of the neuralgic nerve is complained of. Sometimes definite exciting causes can be held responsible for the outbreak of the attack of pain; for example, sudden movements, a cold draught, meteorolog- ical influences, mental excitement; often, however, we seek in vain for such exciting factors. The pain has usually a tearing, dragging character, sometimes it is de- scribed as burning, cutting or boring; it is usually not continuous, but con- sists of paroxysms rapidly following one another ; frequently these are so in- tense that the patients grow pale, break out in a sweat, groan, or even scream. The duration of the attack is varied, sometimes very short (a quarter or half minute), mostly a few minutes: sometimes, however, lasting for hours. Char- acteristic of neuralgia is the fact that the pain is always quite sharply lo- calized by the patient so that he sometimes can demonstrate to us the whole course of the painful nerve with anatomical correctness ; in severe cases, indeed, the irradiation of the pain into a neighboring nerve territory is a frequent occurrence at the height of the attack. Objective accompanying symptoms are only to be made out in part of the cases ; most frequent appears to me to be the reddening of the integument in the region of the affected nerve ; this can, indeed, outlast the attack and can be observed between the attacks. The same remarks apply to the much more infrequent finding of a slight oedema of the skin. I would mention, further, the motor symptoms accompanying severe at- tacks of neuralgia: a tension of the muscles of the region during the attack, sometimes, also, clonic contractions. In neuralgias of the extremities of long duration, in consequence of the position which the patient causes the affected limb to assume in his effort to avoid the pain, there is often a decided degree of emaciation. A hyperesthesia of the skin in the affected territory can quite frequently be made out and, on account of this, pressure and the friction of the clothing often become unbearable. Less frequently there is slight hypoesthesia. More im- portant for diagnosis is the sensitiveness to pressure of the affected nerve, which is mainly limited to definite points in its course (where it leaves a bony canal, perforates a fascia lies upon a firm tissue) ; exceptionally, however, af- fects the whole of its course accessible to palpation. These so-called "Valleix points" can, as a rule, also be demonstrated independent of the attacks of pain; pressure exerted upon them can, indeed, provoke the attack, while, on the other hand, in many cases firm pressure upon these points during the attack is perceived as ameliorative. In describing the special forms of neuralgia we DISEASES OF THE PERIPHERAL NERVES 47 will further consider the Valleix points, which, however, are no necessary ac- companiment of neuralgias.* THE MOST IMPORTANT FORMS OF NEURALGIA A. Trigeminus Neuralgia Among the general causative factors of this very frequent form of neuralgia, for which, also, the names of prosopalgia and "FothergUTs face pain" are occa- sionally' used, diseases of metabolism and infectious diseases play a great role. Among these there is in so far a certain electivity in that, for example, malarial and influenzal neuralgias affect the first branch, diabetes and syphilis the third branch, by preference. This last in contradistinction to the first branch which is almost without exception affected on one side alone, can be bilaterally involved. (Specially characteristic of syphilis is the bilateral Seeligmueller's neural- gia of the auriculotemporal nerve, in which the pain "like a child's comb" runs over the vertex from one ear to the other.) Among local causes, caries of the teeth, pyorrhoea alveolaris, osteitis alveolaris in people without teeth ("nev- ralgie des edentes"), empyema of the antrum of Highmore, or of the frontal sinus, diseases of the nose, ears and eyes, must particularly be considered and in obscure cases there should be no hesitation in enlisting the services of the dentist; the oculist or the oto-rhinologist. Not infrequently the attack of pain comes on at some definite time of day. for instance, on awakening in the morning, or at night (nocturnal neuralgia of Oppenheim). Chewing, yawning, sneezing, pressing the teeth together, blow- ing the nose, speaking, often set up the attack, so that many of these patients live in continual anxiety with regard to these "provocative agents," withdraw from all society, nourish themselves only insufficiently with liquid food, etc. Sometimes they discover for themselves certain procedures which can cut short the attack; for example, in neuralgia of the mandibular branch, the production of negative pressure in the mouth by movements of sucking, the mouth being closed, in that of the ophthalmic branch by energetic pressure upon a Valleix's point. The whole trigeminus is seldom affected, by far most frequently the ophthalmic branch, particularly its division supplying the forehead ("supra Orbital neuralgia"); there is also a "ciliary neuralgia" which has its location behind the eyeball. Neuralgia of the maxillary branch affects by preference the infra-orbital nerve; it is somewhat more frequent than that of the man- dibular. On page +8 the Valleix pressure points in trigeminus neuralgia are exposed in tabular form. ' II i-. somewhat impressive thai pressure upon Valleix's points causes local pain instead nf pain projected into !!»• terminal branches of tin- nerve. We know, for example, thai irritation sei up in an amputation stump is often perceived as pain in Angers or lues. Prob- »blj the sensitiveness nf tin- pressure points depends upon .'in irrilati f the nervi nervorum. 48 LECTURE III I Branch II Branch III Branch 1. "Supraorbital point," over 1. "Infraorbital point," at the 1. "Mental point," over the the supraorbital fora- exit of the infraorbital mental foramen men nerve in the canine fossa 2. "Nasal point," somewhat inside of the inner can- thus 3. "Palpebral point," laterally from the upper eyelid (exit of the lacrymal nerve) J. "Malar point." at the exit of the malar branch on the zygoma (For. zygo- matico-faciale) 3. "Dental points" on the upper gum "Auriculotemporal point," in front of the ear on the zygomatic process "Temporal point" 4. "Parietal point," both in the course of the auri- culotemporal nerve, be- tween the auriculotem- poral point and the ver- tex 5. "Dental points," on the lower gum Of the occasional, but in no way regular, accompanying symptoms of tri- geminal neuralgia, the sensible and sensory disturbances must next be mentioned ; most frequently a hyperesthesia in the skin territory of the diseased nerve is to be found. This is usually particularly marked, on touching the part with a cold object (a piece of metal) ; many patients evince great pain from this test. In inveterate neuralgias we find, however, here and there a slight reduction of cutaneous sensibility, while cases with actual anesthesia, are hardly any longer to be classed as trigeminal neuralgia. The attacks are often accompanied by photophobia, less frequently by narrowing of the visual field, difficulty in hearing or disturbances of taste. Vasomotor, secretory and trophic disturbances have been observed. I will content myself with mentioning the most important and interesting: heat and redness of the skin of the face, of the conjunctiva and of the mucous membrane of the mouth (upon which, indeed, small extravasations of blood may occur), increased salivation, lacrymation, rhinorrhoea, cedema, chemosis of the conjunctiva, the eruption of herpes vesicles; in inveterate cases, circumscribed falling out of the hair, decolorization of the hair, and atrophy of the integument. Finally, accompanying motor symptoms, are the chronic con- tractions of the muscles of the face, less frequently of the masticatory muscles occurring with severe attacks, which justify the denomination "tic douloureux" or "spasmodic epileptiform neuralgia" (Trousseau). The prognosis of trigeminal neuralgia cannot be made on general principles, but only through intimate acquaintance with the clinical peculiarities of the individual case. There are forms which are cured completely and finally in a few weeks, while, on the other hand (particularly in old age), there are those which are refractory to every kind of therapy and which drive the patients to despair, so that suicides have been frequently observed. Even in mild cases the tendency to recur is very common, so that the history of the disease lasting years and decades is nothing unusual. The clinical peculiarities by which the prognostically more favorable cases are distinguished from the more severe ones are the following: "Neuralgia major" has the most marked paroxysmal character in that DISEASES OF THE PERIPHERAL NERVES 49 the pain is not present between the single attacks ; these, however, begin with lightning-like suddenness and usually with great severity. In the most severe cases, however, they follow so close upon one another that they make the impres- sion of a continued pain. Typical is the provocation of these attacks by move- ments, by speaking, by chewing, by swallowing. The pressure points are very often not at the points of exit of the nerves, but upon the tooth-alveoli. In general, only one branch is affected, usually the maxillary; later, however, the ophthalmic and mandibular may be involved. Sometimes in the course of the disease there occur spasms which are now of involuntary character ("tic douloureux"), and again are voluntary (chewing movements, grimacing) ; secretory and vasomotor disturbances occur. In "neuralgia minor," on the other hand, even between the attacks there is a certain amount of pain, from which the attacks distinguish themselves as exacerbations. Valleix's points are to be found particularly at the points of exits of the nerves, even between the paroxysms. The localizations of choice are the first and third branches, the second is less frequently affected, only very exceptionally the trouble involves the whole of the trigeminus. Motor, secretory and vasomotor symptoms are absent. Peripheral etiological factors (affections of the nose, the eye, the teeth) can often be made out. b. Occipital Neuralgia This is less frequent than trigeminal neuralgia and attacks the nervus oc- cipitalis major, oftener than the nervus occipitalis minor. Valleix's points are, for the first, midway between the mastoid process and the upper cervical verte- bra- on the linea nucha 5 superior laterally from the insertion of the ligamentum nucha; for the latter nerve, the region between the insertions of the sternoclei- domastoid and the trapezius, as well as over the mastoid process itself. The pains radiate over the occipital region to the vertex and are often bilateral. Etiologically, besides traumatic influences affecting the region of the neck, gout, malaria, influenza and typhoid fever come into consideration. c. Neuralgia of the Phrenic Nerve This rare neuralgia depends particularly upon local causes, pleurisy, peri- carditis, aortic lesions, fractures of the clavicle. The attack of pain is often initiated by coughing, swallowing or drawing a deep breath; the pain radiates from the base of the thorax toward the neck, sometimes also toward the Bhoulder, the mastoid process, or the hand (which is explained by the anas- tomoses of the phrenic with the other branches of the brachial plexus). The following pressure points have been described: I. At the insertion of the diaphragm into the tenth rib, somewhat lateral from the linea alba ("bouton diaphragmatic) lie" of the French) . '-'. In I he neck, in front of the scalenus ante rior. 3. At the border between the bone and cartilage of the fifth rib. 50 LECTURE III d. Brachial, Neuralgias Apart from ulnar neuralgia in which gout is always to be considered in the etiology, true brachial neuralgias or "brachialgias" are rare. (On this account be cautious in making this diagnosis.) Neuralgiform pains in the arms should always arouse a suspicion of disease of the vertebra 3 or the meninges and tumors in the region of the plexus. Pressure points are, for the musculo- spiral the place where it runs around the humerus in the sulcus spiralis ; for the ulnar, back of the internal condyle ; for the median, the bend of the elbow and the palmar surface over the radio carpal joint. e. Intercostal Neuralgia This localization of neuralgia is again a much more frequent one. Together with the causative factors for neuralgias in general, a number of pathological conditions of the thorax and its contents must be specially mentioned; such are scoliosis, kyphosis, fractures of the ribs, pleurisies, aortic aneurisms. The great majority of cases, however, can be referred to general deleterious influences (infectious diseases, anemia, cachexia, etc.). It must be especially pointed out that tumors of the spinal cord for a long time may have their true character concealed under the harmless mask of an intercostal neuralgia. As concerns the prognosis, many cases of intercostal neuralgia are characterized by extreme persistence, almost all, by a tendency to relapse. The pressure points are quite characteristic: the "vertebral point" lies just along the spinal column at the level of the affected nerve, the "posterior perforating point," upon the sternum, or the rectus abdominis, close to the middle line. f. Mastodynia Neuralgia of the mammary glands or mastodynia, is a special variety of intercostal neuralgia which is limited to the definite branches of the second to sixth intercostal nerves innervating the breast. This extremely obstinate complaint, which is identical with Astley Cooper's "irritable breast," affects almost solely women, often appearing for the first time during pregnancy or the puerperium. The attacks of pain sometimes coincide with menstruation. The skin over the mamma, especially about the nipple, is extremely hyperesthetic during the attack: sometimes also, red and swollen. Erb has described the spontaneous discharge of a milky fluid at the height of the attack of pain. Painful points are the vertebral points of the above mentioned five intercostal nerves. g. Lumbar Neuralgia This very rare form of neuralgia, according to its location, is divided into lumbo-abdominal, crural, and obturator neuralgia. One should be on his guard in these cases quite as much as in brachial neuralgias against overlook- ing local causes (lumbar vertebra?, tumors in the pelvis). DISEASES OF THE PERIPHERAL NERVES 51 h. Sciatica Sciatica neuralgia, or sciatica, also called Cotugno's disease, after the classi- cal description of this author (1764) is the most frequent of all the forms of neuralgia. It occurs in men somewhat oftener than in women, by preference in the fourth and fifth decades of life. Its causes are manifold, particularly often, exposure to cold, as by sitting on cold stones, camping on wet ground, etc., are accused. Also trauma plays no small role. This sometimes is not directly to the pelvis but may affect the periphery of the lower extremity (I saw a particularly obstinate case arise in immediate connection with stumbling, the foot being caught, through which the limb suffered a torsion). On account of causing compression of the nerve within the pelvis, retrouterine hematocele, chronic constipation with fecal impaction in the rectum, tumors of the sacrum (carcinoma metastases) also tuberculous osteitis, etc., act as causative factors of sciatica. Quenu has also called attention to "varicose sciatica" in conse- quence of dilatation of the veins accompanying the nerves to the hip. All these eventualities must be considered in taking the history as well as in estimating the condition (examination by rectum or vagina). General toxic causes which according to experience often form a substratum for sciatica, are particu- larly gout, diabetes (in which the otherwise very rare — and always sugges- tive of vertebral disease — bilateral sciatica sometimes occurs), syphilis, tuber- culosis, malaria, typhoid fever, influenza, gonorrhoea. Finally those cases which are found in patients with flat foot or with diseases of the generative organs or of the rectum are to be classed as reflex neuralgias. Sciatica can begin quite suddenly or the disease picture unfolds itself little by little to its full height. The paroxysms of pain are either separated by in- tervals of entire freedom, or (this applies to the majority of cases), there persists also between the attacks proper, a dull feeling of pain along the course of the affected nerve (in the region of the loins and the buttocks, the posterior surface of the thigh, in the popliteal space, on the outside of the leg, and foot). The pains of sciatica are increased by cold, by sitting and by walking, while lying and standing are in general most tolerable. However, very frequently it night, without any apparent cause, the severest paroxysms occur. Vullcix's points are sometimes absent in otherwise typical eases of sciatica but in the very great majority of cases one or other of these points is to be recognized as sensitive: 1. "Lumbar point" over the spinous process of the fifth lumbar vertebra; '2, "Ueosacral point" near the posterior superior spine of the ilium; .'5, "Gluteal points" over the great sciatic foramen and in the told between the trochanter and the tuber ischii; 4, "Popliteal point" in the middle of the popliteal space; •">. "'Peroneal point" behind the head of the fibula; ). With ordinary hypnotics, if the pain is severe, almost nothing is accomplished. Tin- following mixture will give good service: \f. Potass, bromide 10.0 (3iiss.) Chlorali hydrati 5.0 (gr. Ixxv) Antipyrini 3.0 ( gr. xlv) Codeini phosph 0.4 (gr. vi) Aq. menth. pip ad 150.0 (, z ,v) .M.S. Tablespoonful at night. The injection of morphine "or of one of its modern succedanea in neuritis and polyneuritis should never, or only wry exceptionally, be resorted to. When the period <>f severe spontaneous pains has been overcome so thai these, in spite of intense sensitiveness to pressure which persists, occur with less intensity, thai is, after about four or five days, the application of heat nniy be begun; particularly applicable are apparatus for hot air, for example. liirr'.s boxes. By the application of the so-called "Phoenix" ("Phenix a I'air chaud"), 58 LECTURE III which permits an intensive thermotherapy to be carried on in bed without dis- turbing the patient, we may obtain at the same time a conveniently regulated diaphoresis. Encouragement of the sweat secretion is often considered as of itself a curative agent which acts upon the cause in toxic polyneuritis, and in any event contributes to the alleviation of polyneuritic pains. The duration of tins sort of a sweat bath (to be given once a day) should be from 10 to 15 minutes ; c;i refill control of the pulse is absolutely necessary where the heart is not entirely intact. When the treatment with hot air is inapplicable on ac- count of weakness of the heart, or for some other reason, the application of moist heat (two to three times a day from one-half hour to one hour) is proper, in spite of its inconveniences; nevertheless the application of a compress which is best soaked with warm mildly stimulating decoctions (camomile, matricaria) as well as the envelopment of the part with waterproof tissue, is the more disagreeable for the patient the larger the skin surface which must be treated. Linseed meal poultices are often unpleasant on account of their weight, the same is true of moist compresses kept warm by the thermaphore; the dry thermaphore is often found disagreeable. During this time we should endeavor to dispense with the analgesics pre- scribed at the start, or at least, only to order them at night. I often substi- tute for these drugs aconitin or colchicin, which are withdrawn as soon as the curative effect of the warm applications is plainly evident. Aconitin is best given once or twice a day in the form of 1/10 nig (gr. 1/600), taking care to use an active preparation (Merck's or dill's preparation), colchicin in the form of tinctura colchici, 5 drops t. i. d. (eventually tinct. aconiti, tinct. colchici, aa 10 drops t. i. d.). The withdrawal of the internal analgesics is facilitated by some external applications, which are to be recommended upon psychological grounds, since they occupy and divert the patient. Massage (also the rubbing in of oint- ment) is to be avoided as long as the acute painful stage is not certainly over; also any stimulating electric application. On the other hand the stabile treat- ment of the nerve trunks with the anode (application of the electrode upon the place of greatest sensitiveness to pressure for from 3 to 5 minutes) with weak current (3 to 5 milliamperes an electrode of the size of a dollar), with the cautious turning on and off the current is to be unqualifiedly recommended. For rubbing on, fluid liniments applicable without pressure are suitable. Of these the following are examples: 1. 01. juniperi, 2.0; ol. terebinthini, spts. camphori, spts. saponis, liq. amnion, caust., aa., 12.0. (Shake well.) 2. Camphor, chloral hydrat, aa., 20.0. 3. Chloroform, 10.0; liq. amnion., 40.0. 4. Yeratrin., 1.0; chloroform, alcohol, aa., 24.5 (cave oculos). Upon areas which are the seats of annoying paresthesia, pieces of flannel soaked in the following solution can be laid on: Menthol, guaiacol, aa., 1.0; alcohol abs., 20.0 (cave oculos). DISEASES OF THE PERIPHERAL NERVES 59 4. THE TREATMENT OF NEURALGIAS In every fresh neuralgia of any considerable intensity, bodily rest is an urgent requisite ; in trigeminal or intercostal neuralgia, as a rule, keeping to one's room ( in which case visits, etc., are to be avoided) is sufficient. In sciatica, rest in bed should be enforced. In this the painful limb should be carefully placed in such a position as relaxes the affected nerve as much as possible. This is best accomplished by placing a roll of felt under the bend of the knee ami keeping the extremity in a position of moderate abduction with sand bags. Sometimes (in varicose sciatica, for example) it is desirable to raise the foot of the bed. A patient with sciatica should not leave his bed until the neuralgic paroxysms have ceased, or at least have plainly abated. He should be told in advance that it will be necessary to remain in bed two or three weeks, perhaps longer: at the same time antineuralgic remedies should be given and in suffi- cient doses — a point on which I lay considerable stress, since many failures are due to too timid medication. In sciatica, salicylate of sodium should always be tried first. This has a decidedly more energetic effect than aspirin. This last is to be preferred only in people with sensitive stomachs. I£ Sodii salicylat, 20.0 (5v) ; syr. aurantii cort., -10.0 (3x) ; aq. menth. pip., ad 300.0 (§x). M.S. A tablespoonful 4 times a day in half a glass of water after meals. To avoid disturbance of the stomach a solution of about 30 grains of sodium bicarbonate can be taken after the salicylate. The daily dose can soon be reduced to from two to three grammes (30 to 4o grs.) a day. As to the other antineuralgics, I would refer to the prescrip- tions recommended for neuritic and polyneuritic pains. Particularly in tri- geminal and occipital neuralgia some other drugs are applicable, sometimes with quite happy effects: Migranin (citrate of antipyrin and caffein), in sin- gle doses of grin. 1.00 (15 grs.), butylchloral or its combination with pyra- midon. trigemin (which must only be used when it is of a clear, white color), in single doses 0.5 (71/2 grs.), atropin or methylatropin, the first in doses of 0.0005 (gr. 1/120), of the last 0.002 (gr. 1 30) ; finally tinct. gelsemii, 10 to 15 drops. Aconitin, already mentioned in the treatment of neuritis, is also frequently a very efficacious remedy in trigeminal neuralgia, especially when it is used for a long time with a daily dose of saline laxative. The thermotherapy of neuralgia is the same as that already indicated for neuritis and polyneuritis. In the treatment of sciatica, especially in subchronic and chronic cases, besides this, the following procedures are suitable: Electric lij;ht hat lis, hot air douches, sun bat lis, strain douches, "FangO-packs," hot baths. Cold is in general only found agreeable in fresh cases of facial neuralgia, and in these may even work curatively; however, on this point there are so great individual differences that it is necessarj to try it out from case to case. Freezing the skin over the Valleix points with the chloride of ethyl spray used for local anesthesia deserves special mention. It can he tried in all forms of neuralgia with plainly localized sensitiveness to pressure (in the neighborhood of the exes great caution is necessary; the eyes must be carefully covered with cotton). Naturally the freezing of the skin areas, which is made evident hy 60 LECTURE III their white color, can only last a few seconds ; otherwise there is danger of circumscribed necrosis. After the freezing the affected part must be carefully- rubbed with some simple ointment. This application can only be repeated after the reddening of the affected part (which sometimes persists long after the application of the chloride of ethyl) has disappeared and the parts look normal again. Other "revulsives," whose application is naturally restricted for the most part to the trunk and extremities, are blistering with cantharides, paint- ing with iodine, dry cups, mustard plasters, capsicum plasters, faradization with the wire brush, ignipuncture, etc., also local bleeding (with wet cups or leeches) is occasionally applied. In all these applications care should be taken that the integument should not be injured to an extent which would prevent the later ap- plication of the electric current. In galvano-therapy the stabile application of the anode to the pressure points is to be preferred. In persistent neuralgias the stabile application to the diseased nerve, the two electrodes, of about the size of a dollar, being placed, one upon the most proximal and the other upon the most distal point in the course of the nerve, which is accessible, a current of about five milliamperes gradually introduced and allowed to pass for from five to ten minutes (whether its direction is ascending or descending is a matter of indifference), can be used. In fresh cases of neuralgia, massage, apart from light vibration of the painful points is to be avoided ; in old cases, on the other hand, particularly in sciatica, petrissage of the affected region is an important curative measure, particularly when properly carried out vibration of the nerve trunk is added to it. In sciatica also, "bloodless stretching" of the nerve is a mechanical thera- peutic procedure much to be recommended; it is especially easy to carry out. The leg, extended at the knee, is raised from the bed as in Lasegiie's test (see above, page 51), but only so far as it will go without provoking any consid- erable pain, and is held for several minutes in this position; at the end of this time it is stretched somewhat more strongly, until severe but still tolerable pain is produced, and then carefully laid down again. It will often be noticed that from one treatment to another, the distance through which the leg can be bent without pain increases. "Bloody nerve stretching" is now quite abandoned. A useful procedure is the injection treatment of neuralgia. We distinguish: 1. Langc's method of perineural infiltration. This is a modification of Schleich's infiltration anesthesia. In the trigeminus a few cubic centimeters of the following solution: Beta-eucaine, 0.1; normal salt solution, 100.0 — are in- jected over Valleix's points in the immediate neighborhood of the affected nerve branch. A stovain-adrenalin solution according to the following formula is also used: IJ Sol. adrenalin (1%), gtts. v-x; stovain, 0.1-0.2; sol. sodii chloridi (0.8%), ad 100.00. In the sciatic large quantities (70-100 cc.) are injected. A Schleich infiltration is made over the point of exit of the nerve (in the middle of a line joining the trochanter and tuber ischii) and a- 10 cm. long cannula is passed carefully through this down into the nerve (which is here 1% centimeters in diameter) when a sensation of pain (and often a con- traction of the muscles of the leg) announces that the cannula has entered the nerve. The injection is then made by means of a syringe or an irrigator. Whether the use of ice cold solutions gives better results is questionable, how- DISEASES OF THE PERIPHERAL NERVES 61 ever, the action of simple physiological salt solution without the addition of an anesthetic sometimes is very satisfactory. The therapeutic effect of this pro- cedure is perhaps purely mechanical; a swelling, perhaps a stretching of the nerve which can then produce a cure by reactive inflammation is its result, accoi-ding to Lavge. 2. Neurolytic injections. In these methods we endeavor to injure the nerve by the injection of different solutions, also in a way, to resect it chem- ically. We reserve them on this account for severe cases, since they have as a result anesthesia of the skin, and avoid them in mixed nerves (for example, the sciatic) in order not to risk a motor paralysis. Further, they should not be made into the supraorbital canal, since this, in many individuals, communicates witli the orbit, the penetration into which of neurolytic solutions might se- verely injure the optic nerve. We will not discuss all the substances which have been applied as neurolytics (ether, carbolic acid, silver nitrate, chloroform, etc.), but will only indicate two solutions from which we have sometimes seen good results: (a) 1% osmic acid solution; (b) 80% alcohol, with the addition of an anesthetic ("Schlosser's injections"). Formulas to be recommended are: Alcohol (80%), 20 cc; menthol, 0.4 ; novocain, 0.2; or alcohol (80%), 20 cc. ; stovain, 0.2. In the branches of the trigeminus, one to one and a half cc. are injected into the nerve, if possible, or at least into its immediate neighborhood ; OstmiU, indeed, seeks these branches directly upon the base of the brain (in the foramen ovale or the foramen rotundum), for which he uses a specially bent (bayonet formed) cannula, passing it from the mouth; Levy and Baudoin pass the needle in front of the coronoid process of the lower jaw, or between this and the articular process through the cheek. These methods, however, have not been universally accepted. In every injection of such solutions, the penetration of a vessel must be carefully avoided by first inserting the empty needle and drawing out its contents. The injection of osmic acid, a drop at a time, into the nerve branch laid bare by operation, and indeed, from the exposed base of the skull into the Gasserian ganglion, has been carried out. This brings us to the surgical therapy proper of prosopalgias. The extra cranial methods are easiest; simple section (neurotomy) has been given up, since rapid reunion incurs, and for it has been substituted nerve evulsion after Thiersch-Witzel (neurexaresis) which permits a tearing out of the affected nerve from the base of the skull to its terminal ramifications. In spite of this, many prosopalgias which have been subjected to neurexaresis recur, as do many cases treated with neurolytic injections. While, however, the last can lie repeated a number of times, after evulsion of all three branches of the trigeminus, the trouble iniist 1m attacked at its root, in the fullest sense of the word and extirpation of I lie Gasserian ganglion must he performed. Fedor Krause, the originator of this operation, states that in sixteen years he has performed it sixty-four limes and has never observed a recurrence. In two cases, which I know from my own observation, recovery also occurred. However, this severe operation temains a last resource, in the fortunately rare, specially obstinate cases of prosopalgia. In severe intercostal neuralgias the corresponding posterior roofs have been divided with good effect. Neurectomy has been done not so infrequently in 62 LECTURE III occipital and spermatic neuralgias. In the X-ray treatment of neuralgias I have had no personal experience. Finally, it must be remarked that a change of diet to a purely egg, milk and vegetable regime, can be of the greatest use in neuralgia, and that of course the treatment of any recognizable underlying disease must never be neglected (iron, quinine, mercury, iodide of potassium, etc.), that in all senile forms of neuralgia an arsenic cure should not be left untried, and finally, that the bal- neotherapy of neuralgias is entirely similar to that recommended for neuritis. LECTURE IV The Dyskinesias Gentlemen : In neurology, abnormal motor processes which occur in part as symptoms accompanying different diseases of the nervous system, in part are to be considered as autonomous pictures "sui generis," play an important and interesting role. In this lecture and in the next one we will occupy our- selves with these "Dyskinesias." A. Tremor As tremor we designate involuntary rhythmic oscillatory movements, which affect now all the muscles, again only single groups. According to the rapidity of the oscillations, we speak of rapid or slow tremor (the extremes are about between 4 and 10 oscillations per second), according to the amplitude of the excursions, of coarse, medium and fine tremor. The coarsest form is denom- inated tottering, the finest as vibrating tremor or thrill. If the tremor is absent during rest, appearing only upon carrying out movements, it is called intention tremor. A particular kind of tremor of the fingers is the so-called Quinquaud's phenomenon, which was formerly considered a pathognomonic symptom of chronic alcoholism, but can occur in all disease conditions of which tremor is a symptom. If the examiner presses his hands against the tips of the spread fingers of the patient, he will perceive in these a peculiar unrest in the articulations, in which, presumably on account of a tremor of the interossei, the joint ends of the phalanges are pulled from side to side. Within normal limits, tremor, as is well known, occurs on shivering, in great fatigue, and in the emotion of apprehension; also the tremor of old age, as long as it remains within moderate bounds, may be considered as a physiological phenomenon; with pathological tremor of the most varied origin, on the other hand, we will meet ill the further COUrse of these lectures, quite frequently as a symptom of many functional and organic nervous diseases. A! present, however, we will only consider that form of tremor which in itself constitutes a disease. ESSENTIAL TREMOR In this disease, which occurs mainly as a hereditary family affection, the single symptom is a rhythmical tremor of small amplitude, but without char- acteristic tempo. Nevertheless, it is in general more rapid in young person-,. slower in old ones. It ceases during sleep, sometimes also while the patient is awake, hut in absolute physical ami mental rest; in any event, it is much less marked undi r these la^l conditions. Many patients can control the tremor 63 64 LECTURE IV by an effort of the will, while in others such an attempt, on the contrary, increases the tremor. The carrying out of voluntary movements can arrest the tremor ; one of my patients, for instance, was able, by strongly clenching the right fist, to arrest the tremor in both hands ; on the other hand, how- ever, an intentional character of this essential tremor is occasionally observed. All the voluntary muscles can be affected. The symptom is often exclusively observed in the hands. Tremor of the legs can sometimes interfere with walk- ing, that of the tongue with speech. Frequent complications are malforma- tions, epilepsy, psychoses, on which account the French speak of "tremor of the degenerate." Here and there alcoholism of the parents has been accused of being an etiological factor; nevertheless, there are families with tremor in which the ascendants have been characterized by great abstemiousness. As exciting causes, overexertion, strong emotion, infectious diseases, have been mentioned. The disease is now congenita], again it begins during childhood or puberty, or even later. The fortieth year of life is about the farther boundary. ZM "P*^' ^Z^<^^^X^^M4.1 Fig. 27. Hereditary Family Essential Tremor. Handwriting. A. Before treatment. B. During treatment. The tremor may be stationary, may progress, or, rarely, may grow less, but never ceases entirely. Therapy can accomplish but little. Regnault claims to have obtained improvement by limiting the amount of alcohol used; on the other hand, in a family with tremor, described by Ndgy, the persons who drank most had the least tremor. In the particularly severe case under my own observation, whose handwriting is shown in Fig. 27, after the taking of wine the tremor much decreased, while after coffee it decidedly increased. Bromides, veronal, adalin, scopolamin, were without influence, while, on the other hand, the patient reacted to the "pilula? hyoscyami composite" (extr. hyosc. zinc, oxid., aa 5.0 (gr. Ixxv) ; extract, valer. 10.0 (oiiss). M. Fiat pil. No. 100. S. 3-5 per day) with decided improvement of the tremor, so that the formerly indecipherable handwriting became legible again (see Fig. 27). B. Fibrillary Contractions We give this name to a symptom of motor irritation which manifests itself in isolated and successive contractions of the single fiber-bundles of a muscle. To motor manifestations proper, these contractions, which are usually of lightning-like rapidity and plainly perceptible both by inspection and pal- THE DYSKINESIAS 65 pation, do not, as a rule, lead, though occasionally when the phenomenon is very marked in the thenar muscles, a slight twitching of the thumb may result. Fibrillary contractions occur in functional neuroses and can, indeed, be provoked in normal people (for example, by refrigeration) ; but they are of most importance clinically when they inform us of a pathological process in the anterior horns of the spinal cord (see, farther, Lectures VI and VII). A variety of fibrillary contractions is myokymia. C. Muscular Spasms In the large group of symptoms of motor irritation (hyperkinesias) which we denominate "cramps" or "spasms," two categories are to be separated from each other: tonic and clonic spasms. Contractions of single muscles, or of muscle groups, of short duration repeating themselves in fits and starts are characteristic of clonic, pro- tracted contractions of tonic spasm. By a succession of clonic and tonic spasms the complicated irritative phenomena denominated as convulsions arise. These we will have to study more closely under epilepsy. They con- stitute the most important symptom of motor irritation of cerebral origin. Also by the action of pathological irritants upon the spinal motor tracts in the lateral columns, there occur sometimes spasmodic phenomena ; for example, the so-called Brown-Sequard's spinal epilepsy, a spontaneous clonic twitching occurring in attacks, a more or less severe shaking of the affected extremities on account of alternating contractions of their extensors and flexors. The spasm-producing properties of many animal and vegetable poisons are known to you ; for instance, those of the toxine of tetanus and of strychnine. We will not, however, enter into these things, but will turn our attention to the "idiopathic" forms of spasm. 1. LOCAL SPASMS As to the etiology of these conditions we are very poorly informed ; we can only say that neuropathic individuals and those afflicted with neuroses and psychoses are predisposed to localized muscle spasms, and that in a good part of the cases irritants, working reflexly, appear to be the starting-point for the affection. So, for example, carious teeth, arthritis of the jaw-joint, suppuration of the jaw cavities, eye diseases, have been held responsible for spasms of the .jaw and facial muscles. Only seldom can a disease process, (Forking as a direct irritant upon the nerves of the muscles affected with spasm, lie discovered: for example, in facial spasm, aneurism of the vertebral artery, gumma at the base of the brain, or caries of the petrous bone, etc. The most important types of localized muscle spasm are: 1. Masticator// Spasm. — While tonic spasms of the masticatory muscles (the so-called lock-jaw or trismus) play a notable clinical role as a symptom of tetanus, of meningitis, and of other general diseases, as a purely local disease manifestation they are rare, occurring most frequently in connection with inflammatory lesions of the jaws. Clonic spasm is somewhat more fre- 6() LECTURE IV quent, a gnashing of the teeth occurring in the form of attacks at more or less short intervals. The prognosis is in general favorable. Oppenheim recom- mends in the treatment of fresh cases in which a rheumatic influence may be in action, diaphoresis; further, some derivative procedure (cantharidal plaster over the temples or upon the mastoid process, eventually the actual cautery to the back of the neck), sedative drugs, and the galvanic current. 2. Mimic cramp, or facial spasm, one of the most frequent locations of peripheral spasm, is usually clonic ; a tonic facial spasm is the cause of the peculiar facial expression in tetanus, which has been called "liisus sardonicus" (see Fig. 28). It seldom affects all the facial muscles, but is usually restricted, either to the muscles of the mouth and chin (Orbicularis oris, Levator anguli Fig. sjb "Risus Sardonicus" in Traumatic Tetanus. oris, Mentalis, Zygomaticus), or to the Orbicularis palpebrarum, in which latter case, when the spasm is tonic, we speak of blepharospasm. The expres- sions nictitation, spasmus nictitans, and blepharoclonus denote specially the clonic forms of lid spasm. With the exception of spasms of the lids, which in many cases are relatively easy to treat and to cure permanently, the prognosis of facial spasm is in general not good, since it is an obstinate complaint and even after it is relieved recurrence is to be feared. Further, there are unfortunately many cases which cannot be cured and the patient must then choose between the facial spasm and an incurable facia] paralysis; that is, he must permit a neurectomy or neurexaresis. In these cases, also, in patients hereditarily neuropathic, one is never sure that the spasm may not later attack some other nerve. As regards the therapy of facial spasms, apart from the last resort above mentioned, pathological conditions which THE DYSKINESIAS 67 might set up the trouble by reflex influences should be removed (diseases of the eye, nose, accessory sinuses, and ears, caries of the teeth, retained wisdom teeth, etc.). In the way of medication, a trial of bromide of potassium in daily doses of at least -i grms (oj), or of scopolamin, twice daily, 0.0005 (gr. 1/120) is justified. Unfortunately, these medicines only exceptionally help. Edingcr recommends antipyrin (in combination with bromide of potassium). I have never seen any result from this in facial spasm. From electrical treat- ment stabile applications the anode over the trunk of the facial nerve, or the whole pes anserinus (under the general rules mentioned for the treatment of neuritis, on page 55), I have seen — with sufficient patience and persistence on the part of patient and doctor — occasionally the best results in spasm of the orbicularis oris, only exceptionally, however, any favorable influence worth mentioning in the other localizations of facial spasm. Still another electro-therapeutic procedure comes into consideration in blepharospasm, the stabile application of the anode to a recognizable "pressure point" (that is, in some place from which an attack of spasm can be cut short by firm com- pression, acting reflexly) ; this is usually one of the already mentioned Valleix points in the region of the ophthalmic division. When the above-mentioned methods fail one must turn to the neurolytic injections (after Schlosser), which have been described at length under the treatment of neuralgia. Eighty per cent, alcohol with a local anesthetic (see formulae on page 61) is injected through a cannula inserted between the external auditory canal and the mas- toid process in the neighborhood of the stylomastoid foramen indicated by the styloid process; that is, into the facial nerve at its point of exit. If the injection is successful a facial paralysis which lasts for several months is pro- duced; if this is not to be obtained, this result is reached by exposing the nerve and stretching it vigorously (pulling it up with a "squint hook"). In favorable cases after the facial paralysis has recovered, spasm does not return; unfortunately, there are cases enough which have recurred both after alcohol injections and after nerve stretching. To be distinguished from facial spasm is the psychogenic, isolated "tic facial" of the French writers (Brissaud, Meige, Feimlcl), which is a twitching or grimacing which has become a habit, which has the character of voluntary movement, and is to be treated and cured by exercises. The object of these exercises is gradually to carry out definite movements witli the facial muscles affected willi tie upon command, later to the heat of a metronome; between these, however, to keep the face still. I. .iter the patient is allowed to practice complicated and more continued series of movements of head, neck, and face niiisrlrs until these follow quietly without being interrupted by spasms. All these exercises are to be earned out before a mirror, When there is an emotional basis for facial tic, Dubois has accomplished cures by pure psycho- therapy. .'5. Spasm of tin- Tongue (Glossospasm). — Isolated spasms of the tongue are excessively infrequent. They can he tonic and clonic. As reflex causes setting them oil', inflammatory affect ions of the buccal cavity have been espe- cially mentioned. The prognosis is usually not had. Therapeutically stabile galvanization, the anode on the hvpoglossus, is particularly recommended; 68 LECTURE IV in a case of Lange's this nerve also was stretched and later resected, but only division of the genioglossal muscles effected a definite cure. 4. Spasm of the Pharyngeal Muscles (Pharyngism). — When this form of spasm docs not arise upon the basis of a general disease of the nervous system, for example, hydrophobia or tetanus, or is not an expression of a local organic disease (retro-pharyngeal abscess, carcinoma of the oesophagus, a foreign body, e.g., a fish-bone in the mucous membrane), it is usually a symptom of hysteria. 5. Spasm of the (Esophagus (CEsophagism) . — To this phenomenon the remarks made regarding pharyngism equally apply. After tonic spasms of the lower parts of the oesophagus or of the cardiac orifice of the stomach, dilatations of the upper part of the oesophagus have been observed. (3. Spasm of the Larynx (Laryngismi). — This form of spasm is not at all infrequent in children, particularly in rachitic infants. We will later have the opportunity of referring to its relation to the tetany of infants. It occurs in the form of tonic contraction of the muscles closing the glottis (thyroarytenoids and interarytenoids) coming on in attacks which may lead to severe dyspnoea and cyanosis, indeed, to asphyxia. Well-known popular remedies like douching the child with cold water, the production of vomiting by tickling the back of the throat, etc., sometimes suffice to cut short the attack; on the other hand, tracheotomy may be necessary. Along with the treatment of rickets, which is often present, the effect of the different internal sedatives is usually very satisfactory, so that the prognosis in general may be said to be favorable. To be recommended is a prescription of Babinski: K< Potassii bromid, 1.5-3.0 (gr. 20-4.5); tinct. moschi, 1.0-2.0 (gr. 15-30); syr. simp., 15.0 (Yi oz.) ; aq. dest., q.s., ad 100.0 (3 ozs.). M.S., a dessert- spoonful every two hours. Reflex spasm of the larynx as a result of laryn- gitis occurs both in children and adults. 7. Spasms of the muscles of the throat, neck and shoulders are not very frequent, but are usually characterized by great persistence. They occur nearly always in neuropathic individuals ; exciting causes are, in the psychical sphere, fright; depressive emotions, etc.; in the somatic, local trauma, dis- eases of the vertebral column or of its ligaments, etc. Not infrequently its etiology is obscure. We distinguish different forms of spasm which are quite characteristic in their manner of occurrence. Tonic unilateral spasm of the sternocleidomastoid is called spasmodic tor- ticolis (Caput obstipum spasticum) ; in this the patient turns the head and raises the chin toward the sound side, while, on the affected side, ear and shoulder are approached to one another. The sternocleidomastoid, on the side on which the head is drawn down, stands up under the skin like a firm cord (see Fig. 29). This affection is not to be confused with the harmless, temporary stiff neck due to a rheumatic affection of the sternocleidomastoid; characteristic of this latter is the decided painfulness of the muscle upon pressure and in passive movement.* Clonic unilateral spasm of the sterno- * I once saw, however, in a patient with hysterical predisposition, a spasm of the sterno- cleidomastoid develop as a sequel to a rheumatic stiff neck. THE DYSKINESIAS 69 cleidomastoid proceeds in the form of attacks of twitching through which the head is pulled by fits and starts into the position described above. Spasm of the sternocleidomastoid is usually combined with one of the trapezius, which is also supplied by the spinal accessory nerve ; the head then is either clonically thrown backward or there is a tonic "retrocollis." Rotary spasm of the head depends upon clonic contractions in the obliquus capitis inferior; nodding Fin. 29. Spasmodic Torticollis. spasm (spasmus nutans) (called also "salaam spasm" after the method of salutation of the Orientals) upon those of the deep muscles of the neck (rectus capitis, longus colli, etc.), usually with involvement also of the sternocleido- mastoids. This last-mentioned form of spasm is observed especially in children, and, according to Kassowitz, is always due to rickets, which, however, other pediatrists deny. The following mainly tonic spasms should be mentioned: spasm of the splenius, in which the head is drawn backward toward the affected side and at the same time is somewhat rotated, while laterally from the cervical portion of the trapezius the contracted muscle stands plainly out; spasm of the rhomboid, which draws the scapula into an oblique position so that its median border runs oblique from below and within to above and 70 LECTURE IV without ; finally, the rare spasms of the Levator anguli scapulae, Platysma myoides, and Omohyoid. In connection with treatment I would recommend, after correction of what- ever causal factor can he found, next to take care that the patient is isolated for several weeks in order to reduce external irritants, particularly psychical ones, to a minimum. He should be allowed to see no one except those with whom he is entirely familiar, and about whom he does not have to trouble himself, besides the doctor. Psychotherapeutically, one should untiringly endeavor to keep the patient placid and confident. In addition, bromide of potassium should be given in daily doses of 3 or 4 grammes (grs. 45-5 j ) (best in one dose in the morning), eventually combined with grm. 1.0 (gi's. 15) of anti- pyrin. Also scopolamin hydrobrom., 0.0005 (gr. 1/120) twice a day is worth trying. At the same time the stabile application of galvanism with the anode and a current gradually raised to 3-5 milleamperes should be used. Points of application: 1. Pressure points. 2. The motor points of the affected muscles (see Fig. 3, page 25). 3. Those of the nerve trunk in question. In connection with this, I am accustomed to faradize the antagonists. Spring- supporting apparatus, with a head holder which opposes the pathological anomaly of position in tonic spasm of the neck muscles, sometimes aids our therapy decidedly ; one of my patients, a machinist, very intelligently con- structed such a thing for himself. I would decidedly warn you against rigid apparatus (plaster, etc.), in spite of its recommendation by StriimpeU. Op- penhcim recommends, further, the application of derivatives at the back of the neck (blistering plaster, a hair seton, and particularly the actual cautery) ; I am, however, no great friend of these procedures. If isolation, psycho- therapy, drugs, galvanization, etc., supported by careful stretching massage and prolonged tepid baths, are without effect, neurolytic injections are to be considered, which, however, in order not to injure the important neighboring structures should only be made into the exposed nerves of the muscles affected with spasm; however, it is more rational to undertake the mechanical rather than the chemical injury of the nerve; that is, stretching it until paresis be- gins ; neurectomy is the next most radical procedure. It, like myotomy of the muscles affected with spasm, has found more partisans among the surgeons than among the neurologists, who have often after the operation seen the spasm pass over to neighboring, up to this time sound, muscles. Even the Kocher-de Quervain operation, in which successively the follow- ing muscles are cut : Trapezius, Splenius, Sternocleidomastoid, Complexus and Obliquus colli — with subsequent curative gymnastics, does not produce a cure in every case; in the case shown in Fig. 29, which did not react to other methods and in spite of being easily hypnotizable, not even to hypnosis, myotomy in- deed, effected a cure. 8. Localized Muscular Spasms in the Extremities. — These are, on the whole, quite rare. Tonic forms of spasm, sometimes unilateral, sometimes bilateral, have been observed among others in the following muscles: Pectoralis major, Latissimus dorsi, Deltoid, Biceps, Supinator Longus, the flexors of the fingers, the adductors, Gastrocnemius, and Tibialis anticus, clonic forms in the Ileopsoas and the Peronei. THE DYSKINESIAS 71 Not to be confused with these true, local spasmodic conditions of the mus- cles of the extremities, are the painful cramps which, as is well known, occur after fatiguing marches, long swimming, mountain climbing, etc. ; also in healthy people, in the calf muscles (less frequently in the Abductor hallucis, Quadriceps, Tibialis anticus) and for which certain individuals have an ex- aggerated predisposition so that relatively slight exertion suffices to set up cramp. In contradistinction to true clonic spasms, cramp is relieved by mas- sage and passive stretching. 9. Spasm of the diaphragm in tonic form is very rare and is almost never a disease in itself; it is most frequently observed in tetanus. All the more familiar is clonic spasm (singultus) which occurs in many healthy people, now reflexly (as from drinking strong spirits), again without apparent ground as an occasional temporary and entirely harmless phenomenon. Obstinate pathological forms of singultus occur in diseases of the cervical region of the cord (in the neighborhood of the diaphragm center) further in peripheral irritation of the phrenic nerve (aortic aneurism, mediastinitis, pericarditis, pleurisy, etc.), occasionally, also, as an apparently idiopathic and localized spasm. Familiar popular means of cutting short singultus (holding the breath, sipping cold water, etc.) fail in severe forms. Energetic faradization of the region of the stomach with the wire brush often succeeds here. Other derivatives to be applied in this last location are sinapisms, blisters, igni- puncture, chloride of ethyl spray. Of internal sedatives, besides bromide of potassium, opium is recommended. Lubordc has introduced rhythmical trac- tions of the tongue, which sometimes succeed. Finally, the galvanization (anodal) of the phrenic nerve comes into consideration. Complicated respiratory spasms which, indeed, only occur in the hysteri- cal, are yawning spasm, sneezing spasm, screaming spasm, snoring spasm. These are of psychic origin, '■tics.'' like voluntary movements, not true spasms, and their treatment, mutatis mutandis, has to he carried out from the points of view considered under facial tic. We must still mention that there is a diseased condition characterized by the most manifold motor automatisms which has been described as "Myospasia convulsiva," "Maladie des tics," or ''general tic." This occurs chiefly in individuals of neuropathic constitution beginning in childhood, and is often accompanied with psychic disturbances; for example, the compulsory ejaculation of certain, sometimes senseless, often obscene or blasphemous, words (Koprolalia). Often there is also the com- pulsion to repeat words heard or gestures seen (Echolalia. Echopraxia). There are very severe incurable cases of this disease. In one mild case which I saw, cure resulted from a "mast cure,*' combined with the most rigid isolation in a dark room, to which, later, a course of re-education of movements was added; in other cases considerable improvement; these were in children. The older the individual the worse, in general, the prognosis. Drugs and physical therapeutics are useless, so is hypnosis. Similar to myospasia convulsiva, but of favorable prognosis, usually recovering spontaneously after weeks or months, is the "saltatory reflex spasm" described by Bamberger, in which the patients on standing break into jumping movements, while on sitting .and lying they hold themselves quite normally. 72 LECTURE IV 2. "OCCUPATION SPASMS" From the disease pictures summed up under the name of "Local Muscular Spasms" we separate a pathogenetically unique nosological group which is distinguished by its great practical importance. Its members have been called "occupational spasms," also (after Benedikt) "co-ordinatory occupation neu- roses," and had already been recognized and their clinical peculiarities noted by Bell and Duchcnne, namely, as spasms which appear only in connection with a definite activity acquired by practice. They usually appear not only as spasms (which practically always are of tonic character), but not at all infrequently as tremor, and occasionally as a refusal to act on the part of the affected muscles, which last it has been attempted to separate off as the "paralytic" variety of the occupation neuroses. As a diagnostic criterion the limitation of these different dyskinetic disturbances, not only to definite motor acts, but also to the muscle groups acting together in this function, is of decisive importance. For the occurrence of this affection two factors are necessary: 1. A neuropathic disposition (usually hereditary). 2. The fre- quent repetition of the act in question (usually through the carrying out of one's calling). That along with these, alcoholism, nephritis, chronic lead poisoning, and a number of other general injurious influences contribute to the production of occupation spasms, is made probable by a number of striking observations. Of local predisposing factors, diseases of the tendon sheaths (as ganglion), farther, exostoses on the bones, muscular and articular rheu- matism, have been mentioned. As exciting causes, somatic and psychic trau- mata have been especially frequently determined. The best-known example of occupation spasm is writer's cramp ("crampe des ecrivains," graphospasm). It occurs usually either on beginning writing, or only after some lines or pages have been written, as a tonic contraction of the flexors of the fingers ; much less frequently there is spasm of the extensors or abductors ; occasionally the hand begins to tremble or the fingers lose power and let fall the pen ; cramplike pains can occur also.* Other occupation spasms of the upper limbs are: Milker's cramp, described in 1851 by BasedoTc; farther, piano player's, telegrapher's, shoemaker's, drummer's, tailor's, cigarmaker's, violinist's, 'cellist's, flutist's, seamstresses', sawyer's, newspaper folder's, smith's, zither player's, and watchmaker's cramps. In the legs there occur ballet-dancer's cramp ; in the muscles of the mouth and tongue, trumpeter's and clarinetist's cramps. Oppcnhclm has described a "shaving cramp" ("keirospasm" or "xyrospasm"). The most "modern" spasms are "daktylographer's" and "automobilist's" cramps. These occupation neuroses are not to be confused with the professional pareses — which are mainly of neuritic nature — already mentioned. I have, however, seen develop in a cigarmaker, a year after recovery from a typical cigar-roller's cramp, a not less typical cigar-roller's paresis, with atrophy of the small muscles of the hand, which then, after rest and later change of occupation, entirely disappeared. This last, on account of the great obstinacy * For the vasomotor form of "writer's cramp" see Lecture XXV. THE DYSKINESIAS 73 of many occupation spasms, is not very infrequently the end result of vain therapeutic endeavors. A considerable number of the cases, however, are fortunately not refractory to the means of treatment at our disposal. First, the specific harmful occupation must be entirely given up for a period of weeks, during which time complete therapeutic repose is to be sought for in the first place, since anxiety about the spasm and fear as to the eventuality of having to give up one's calling, usually greatly depress such patients and serve to keep uj) the neurasthenia which furnishes the basis of the occupa- tional neuroses. Prolonged tepid baths, Swedish gymnastics, stabile anodal galvanization of the nerves and muscles in question (with avoidance of all sudden variations of current strength), a sojourn in the mountains or at the seaside, a course of arsenic or iron, are to be prescribed, according to cir- cumstances. Later, the patient is allowed to carefully take up again the "crit- ical" occupation, in which case this is to be undertaken in the most rational and easy manner possible; writing, for instance, with a soft, not too sharp, steel pen in a thick cork penholder, or, even better, special "writers' cramp" penholders, as, for example, that of Zabludowski, or "Nussbaum's bracelet," which is held by the spread fingers; also by finding out the easiest position for the hand. I let the patient at first go over for a few minutes at a time a very coarse copy placed on tracing paper, later writing it smaller. Little by little he is allowed to practice longer, and finally to write without a copy. For patients with pianist's cramp, Zabludowski recommends practice on a juvenile piano, which has a considerably smaller scale than the regular instrument. Telegraphists should, when possible, exchange the Morse appa- ratus for that of Hughes. 3. THE MYOKLONIAS Under this name we include some rare, pathogenetically still obscure, dis- ease conditions whose common chief symptom consists in clonic contractions of the individual muscles which usually produce but little movement, or none at all. Three chief forms may be distinguished. 1. Paramyoklonus multiplex (Friedreich') occurs usually about the fiftieth year of life, sometimes set up by a psychic or somatic trauma, infectious diseases, excesses, and sometimes without any recognizable cause. It shows itself in attacks lasting a minute or so (of irregular rhythm and a frequency of 10 to 15 per minute) of lightning-like contractions of different, usually symmetrical muscles of the trunk and extremities; for example, the latissimus dorsi, gastrocnemius, quadriceps, pectoralis, rectus abdominis, biceps brachii, triceps, ete. The contractions are most marked during rest (in sleep, indeed, they usually stop, or at least grow ]e S s) ; on movement they are less. In one case I described a coincidence with congenital defect of the pectoralis, which is, of course, to be considered as a stigma of congenital deficiency of the muscles. 2. Myoclonus Epilepsy (Unverricht) . — Symptomatologically particularly Characterized by the myoklonic contractions affecting the tongue, the phar- yngeal, and diaphragmatic muscles, this affection appears almost always in 74 LECTURE IV several children of a family, not rarely in several successive generations. Note- worthy also is its association with occasional epileptiform attacks occurring particularly at night, which sometimes show themselves as precursors of the myoklonic manifestations (the last usually begin about the tenth year of life). The myoklonic phenomena increase on movement and in psychical ex- citement. The tendon reflexes, as well as the mechanical irritability of the muscles and nerves, are increased. Life is seldom directly threatened (for example, by aspiration pneumonia as a sequel to myoklonic spasms of the pharynx). The patients can live to be as old as seventy years, in which case there is usually gradual mental failure and termination in "Dementia myo- clonica" and marasmus. 3. Nystagmus-myoclonia (Lcnoble-Aubineau). — A rare affection, almost exclusively observed in the Celtic families of Brittany and Great Britain, in whom as a congenital and stationary condition contractions of the external eye muscles, as well as of the extremities, which are increased by cold and by tapping the muscles, but can to a certain extent be voluntarily controlled, have been observed. The reflexes are usually much exaggerated, different trophic and vasomotor disturbances (for example, deformities of the teeth, asymmetry of the face and the body, local sweating, circumscribed oedema, lividity of the skin) are also found. Therapeutically, stabile galvanization of the muscles with the anode, warm baths, bromide of potassium, chloral hydrate, can be used in the myoclonias as fulfilling to some extent the symptomatic indications. 4. TETANY As characteristic of this disease, given its name by Corvisart, we can con- sider tonic spasms, which specially affect the peripheral portions of the limbs, occur in attacks, and are usually combined with more or less decided pains. It is usual to separate a "primary" tetany from the different "secondary" or symptomatic tetanies. The first, whose etiology is still obscure, attacks, as a rule, previously healthy and strong males between fifteen and twenty-five years old, and by preference those carrying on some definite calling, particu- larly shoemakers and tailors, more rarely joiners and locksmiths. Cold and damp weather favors the onset of the disease; the majority of cases occur in the months from January to April. The geographical distribution of the disease is striking; it is most frequent in the neighborhood of Vienna and Budapest, while it is quite infrequent in France, and has decreased in fre- quency from decade to decade ; in Basle and its neighborhood tetany of workers is practically unknown. I sought in vain for years for a case and have had no word of any definite observation by any one else, a fact all the more remark- able since, in the not very distant Heidelberg, primary tetany is compara- tively frequent. The "secondary" tetanies occur in the course of different affections, and hence are grouped as follows: 1. Gastrointestinal tetany, which is occasionally observed in profuse diarrhea or, on the other hand, in obsti- nate constipation ; also in dilatation of the stomach and stenosis of the pylorus, in carcinoma of the stomach, appendicitis, intestinal helminthiasis and chole- THE DYSKINESIAS 75 cystitis. 2. Intoxication tetany, which can occur, for example, in ergotine, lead, opium, atropin, and alcohol poisoning. 3. Tetany in acute infectious diseases; this is occasionally observed; sometimes in the beginning, sometimes in the course of typhoid fever, dysentery, measles, cholera, scarlatina, influenza, diphtheria, malaria, acute rheumatism, etc. -i. Maternal tetany, which devel- ops in pregnant, puerperal, and nursing women, and was first described by Trousseau under the name of "contracture rhumatismale des nourrices." 5. Parathyroid tetany, the result of destruction of the parathyroid glands. It is not long since tetany occurring after extensive or total thyreoidectomy was regarded as due to the loss of this gland. To-day, however, clinical and experimental proofs have been furnished by Vassale, Generali, Moussu, Pineles, Erdhcim, de Quervam, Hagenbach, Isdln, and others, that usually the loss of the accessory glands to the thyroid provokes the onset of tetanic phe- nomena. 6. The tetany of children, which, as a rule, occurs in rachitic children and in those suffering from gastrointestinal disturbances. The theory that all varieties of tetany depend upon an insufficiency of the parathyroid glands is not proved, but has much to recommend it ; their function is plainly an anti- toxic one; it can be conceived that tetany occurs not only when the para- thyroids are destroyed, but also if their ability to neutralize exogenic or endogenic poisons is not sufficient. In the tetany of workers and maternal tetany, hypothetical toxines entering the organism or produced in it have long been spoken of. According to Ycmase, however, a number of cases of the tetany of children depend upon hemorrhages into the parathyroids (probably at birth). The symptomatology of tetany is characterized by a number of exceed- ingly typical phenomena. Sometimes (namely in primary tetany) there is complaint of certain prodromal symptoms, namely, paresthesias in the hands and feet, general malaise, muscular unrest; from these prodromes patients who have already had tetany are able to recognize the oncoming new attack. Tin se last are entirely dominated in their clinical aspect by the attacks of spasm which arc usually preceded by a feeling of tingling in the affected limbs, tin- so-called "sensory aura." The upper extremities are most fre- quently and severely affected, particularly the hands, whose characteristic attitude Trousseau lias described ill a classical manner. The thumb is brought into a forced position of adduction, the closely approximated remaining fingers are bent against it predominantly at the metacarpophalangeal joints; also the palm of the hand forms a hollow, on account of the approximation of its radial and ulnar borders, and the whole hand takes the wedge shape assumed by the obstetrician lor vagina] examination ("accoucheur's hand"). The wrist is tonically flexed, and in extensive spasms, the elbow also, while by firm contraction of I hi' pectoralis, the upper arm is pressed against the thorax; only exceptionally does the arm take a position of extension. Extension at the hip and knee joints, on the contrary, is typical in tetanic spasm of the legs; the feet, however, imitate the "obstetric hand" by approximation of the maximally flexed toes, under which the overadducted big toe is forced, hollow- ing the sole. In severe tetany, tonic spasms may affect the face muscles; Escherich has called at lent ion to a trunklike protrusion of the lips occurring 76 LECTURE IV in this case. In involvement of the masticatory muscles trismus occurs ; in that of the external eye muscles, squinting ; sometimes the long muscles of the back and the sphincters are also affected. Particularly important are spasms of the glottis which for a long time were regarded as the privilege of infantile tetany, which, however, Pineles has shown are not so infrequent in that of adults. The spasms of tetany are usually symmetrical, but one side can be Fig. 30. Parathyroid Tetany. — Position of the hands in the attack (instantaneous photograph). affected before the other, or to a greater extent. As to the length of the attacks, they vary from a few minutes to several days ; in cases of the last sort, ulcers have been known to be produced by the unintermitting pressing together of the fingers. As already said, more or less manifest painful sensa- tions usually accompany the tetanic spasms. Besides the tonic spasms (which when they are set up by voluntary move- ment are called "intention spasms"), tetany presents a number of exceedingly interesting motor phenomena, which occasionally, in rudimentary or "latent" tetany, can occur without an attack of spasm; on the other hand, they need not all be present in typical tetany. These are Trousseau's, Clivostek's, Erb's, and the Pool-Schlesingcr phenomena. THE DYSKINESIAS 77 1. Trousseau's sign: If with the fingers energetic pressure is exerted upon the nerves of an extremity (especially upon the nerves of the upper arm in the internal bicipital sulcus), or if a'limb is firmly bound with a rubber tube, after a few seconds or minutes a typical attack of spasm occurs in it. 2. Chi'ostek's sign: Tapping the trunk of the facial nerve in front of the ear, or even stroking this region, sets up contractions in the muscles of the face. 3. Erb's sign : There is galvanic ovcrexcitability of the muscles and nerves. The cathodal closing contraction appears with a very weak current (in the case of the girl shown in Fig. 30 with one-eighth milleampcre on the ulnar). It is further characteristic that the anodal contractions (AnOC and AnCIC) can be obtained with a comparatively weak current. Finally, according to Mann and Thicmich in infantile tetany, and according to Pineles, sometimes in that of adults also, it is typical that contraction on cathodal opening occurs below milleampercs and that AnOC is greater than AnCIC. Faradic overirritability is rarer. 4. The Pool-Schlesinger sign ("leg phenomenon"): If in the time between attacks the leg extended at the knee is flexed at the hip (or if the patient lying on his back is ordered to sit up, his knees being kept pressed together), after a few minutes, or often after a few seconds, there occurs a spasm of extension at the knee and a tonic spasm at the ankle, with severe pain. The spasm, as a rule, is limited to the extremity under examination, and usually ceases when flexion at the hip is relaxed. Galvanic hyperexcitability of the sensory nerves, occasionally observed in tetany, is known as Hoffman's phenomenon; the supraorbital nerve, for in- stance, reacts to 0.2 instead of to 1.0 milleampcre. According to Chvostek, Jr., and v. Franhl-Hoclncart, galvanic overexcitability of the nerves of hear- ing and taste can also occur. It remains for us to enumerate a number of rare and inconstant symptoms of tetany. First, along with the tonic spasms there occur more or less severe attacks of clonic contractions which sometimes are accompanied by alteration of consciousness, are "epileptiform"; this is most frequent in the tetany of infants, since in general infancy is predisposed to eclampsia. Moderate rise of temperature, or, on the contrary, hypothermia is some- time observed. Other trophic and vasomotor accompanying phenomena are a certain puffiness of the face which in primary tetany has even been described as the ".tetany countenance"; further, there is sometimes a redness or cyanosis of the face or of the extremities, oedema of the hands and feet, also of the joints, falling out of the nails and hair, and finally cataract. Trophic symp- toms are in general characteristic of severe chronic relapsing tetany. Of psychic phenomena, hallucinatory conditions in sequence to the attacks of Spasm are to he mentioned. Tin tendon reflexes are, as a rule, exaggerated* The course and prognosis of tetany vary within comparatively wide limits. Maternal tetany always runs an acute and favorable course; also cases occur- ring in infectious diseases and intoxications always recover, the cause ceasing to act. In parathyroid tetany, all depends upon the amount of uninjured para- thyroid substance left; total ablation of these bodies causes chronic tetany. 78 LECTURE IV Gastrointestinal tetany is a severe disease, with a tendency to relapse and to pass over into chronicity. The prognosis depends above everything else upon whether the stomach or intestinal affection can be removed. In infantile tetany, attacks of eclampsia, or spasm of the glottis, may cause death. As to primary tetany, Pineles writes that the prognosis in a certain portion of the cases is quite favorable, since the disease is recovered from in a longer or shorter time, that however, in a not inconsiderable remainder of them, it takes a chronic course through many years, relapses occurring chiefly in the "tetany season." In this form, trophic disturbances are comparatively fre- quent, nevertheless its prognosis as to life is favorable, since no case of death from idiopathic, uncomplicated tetany has so far been observed. From a diagnostic point of view, with regard to the "spasmophilia" of infancy as well as to the tetanoid conditions of adults, reference must be made to the fact that galvanic overirritability is the single constant and obligatory symptom of tetany, around which then the remaining symptoms can group themselves in different combinations. In a child in a condition of latent tetany, an inter- current digestive disturbance or taking cold can at any time lead to an out- break of spasm in the extremities or to spasm of the glottis. Arthrogryposis, an affection of the first years of life, which is charac- terized by tonic spasms and positions of contracture of one or several limbs, lasting from days to weeks, seems to have relationships with infantile tetany ; the legs are fixed now in extension, again in flexion, the arms spasmodically flexed. Recovery usually occurs ; Strwmpell, however, has seen two cases end fatally (with negative post-mortem findings). Important for its distinction from true tetany, is the absence of mechanical and galvanic irritability of the nerves. The therapy of tetany must, of course, be a causal one in all secondary forms ; so, for example, in lactation tetany, nursing must be forbidden ; in gastric tetany, according to conditions, lavage is to be practiced or — and not too late — pyloroplasty or gastroenterostomy is to be recommended, etc. In the tetany of workers, in order to prevent recurrence, too great crowding together of these people is to be prevented, and they are to be protected from the influence of cold; further, alcohol is to be forbidden them; eventually change of residence and calling should be insisted upon. Parathyroid tetany ought to be dying out on account of the increasing care on the part of sur- geons to avoid injuring the parathyroids in goiter operations. For all varieties of tetany rest in bed and tepid baths of long duration are to be recommended. The diet should be purely ovolacto-vegetarian (with- drawal of meat, in animals experimentally made subject to tetany, reduces the frequency and intensity af the spasms). Of drugs, the alkaline bromides (in daily doses of from 3 to 6 grms — gr. xlv-ojss.) come specially under consid- eration in adults; as does scopolamin, 0.0005 (gr. 1/120), twice a day; this last acting specially on the pains, for which also chloral hydrate, salicylic preparations, antipyrin, pyramidon, lactophenin, etc., may be tried; occa- sionally morphine is necessary. Loeb and McCallum recommend calcium lac- tate, which reduces the overirritability of the muscles in animals without para- thyroids. A teaspoonful of a ten-per-cent. solution is given three or four THE DYSKINESIAS 79 times a day. The great hopes which had been placed upon organotherapy with parathyroid preparations have not been realized. The transplantation of parathyroid tissues also has given no result worth speaking of, since these do not remain capable of functioning in their new location, but speedily perish, although the transplanted structures, so long as they are not reab- sorbed, act favorably. In the treatment of infantile tetany, on account of its frequent relation to rickets, along with bromide of potassium (see formula, page 68) and chloral (0.15 to 0.5 — grs. 2 to 7 — by clysma), phosphorus, best given in the form of Kassowitz's emulsion, plays, with right, a considerable role. I? Phosphori 0.1 (gr. V/ 2 ) 01. amygdal. dulc 10.0 (oijss.) Sacch. alb., Gunimi mimosa aa 5.0 (gr. lxxv) Aq. dest., q. s. ; ad emuls 100.0 (§iij) M. S. A teaspoonful once or twice a day. LECTURE V The Dyskinesias D. The Choreiform Diseases Involuntary, quick movements, co-ordinated, indeed, but nevertheless of aimless and contrary character, which are not rhythmic but rather give the impression of a jerking, continuous unrest of the affected extremities, we designate chorea; the name comes from the Greek (X"l""> = dance), and is in so far suitable, since when the lower extremities are affected, the gait of the patient can degenerate into dancing and hopping. The most frequent, and practically by far the most important, form of chorea is the disease known as Chorea minor, Chorea sancti viti ("Yeitstanz," "Danse de Saint Guy"), or Sydenham's chorea; much more rare is Huntington's degenerative or hered- itary chorea, which is also an autonomous disease. Usually of symptomatic indication, on the other hand, are hysterical chorea (also "chorea major"), which occurs auto-suggestively by imitation of chorea minor, and not infre- quently leads to school or class epidemics, chorea in lesions of the cerebellar peduncles (see Lecture XX), as well as pre- and post-hemiplegic chorea, which sometimes is observed as the precursor or result of an apoplectic attack from cerebral hemorrhage. This last variety of chorea, affecting only the extremities of one side, is also a hemichorca. Its presence justifies the conclusion that there is a hemor- rhage into the optic thalamus, the lenticular nucleus, or into the most pos- terior part of the internal capsule of the opposite side of the brain. The affected extremities show lively jerking or shaking movements ("hemiballism"), which are absent during sleep, but cannot be controlled voluntarily, even may be exaggerated upon attempting this. The designation of certain clinical pictures described as great rarities by the name "Electric Chorea" is incor- rect. In these cases the involuntary movements follow one another with lightning-like rapidity, in the form of violent jerking. Henoch's form of electric chorea is probably an atypical myoclonia, that of Bergeron, a hys- terical phenomenon. On the other hand, Dubini's electric chorea, a disease observed in Lombardy, in which besides muscular twitchings there is fever, pain in the neck and back, epileptiform attacks, loss of faradic muscular irritability, pareses, etc., and which usually terminates fatally, is probably an infectious disease of obscure etiology. 1. CHOREA MINOR, SYDENHAM'S CHOREA This disease, in the great majority of cases, attacks children, chiefly girls, particularly between the sixth year of life and puberty. Younger children are only exceptionally attacked, also the disease is very rare between fifteen 80 THE DYSKINESIAS 81 and twenty-five years (these "juvenile forms" nearly always occur in females). Cases occurring at a later age are great rarities, though even "senile forms" have been known. In the etiology neuropathic predisposition probably plays a considerable role (heredity is here and there recognized). In the majority of cases, bow- ever, the action of some infectious influence is decisive. In the first place must be mentioned acute rheumatism and endocarditis, which often precede the attack of chorea minor, accompany or follow it. Less frequently a connec- tion with the acute exanthemata, typhoid fever, pneumonia or erysipelas has been determined. Exposure to cold has often been accused; chorea minor, indeed, is more frequent in cold, moist localities and in the winter months. In the juvenile forms pregnancy always plays an important role. It is seen in young primiparae, who are attacked in the early months of pregnancy. The disease but rarely begins suddenly. As a rule, the family notices first a general unrest with psychical disturbance (anxiety, ill humor, unsociableness), as well as the appearance of illness and poor appetite; in this stage also many patients complain of weakness and vague painful sensations in the limbs. Next, movements become clumsy and finally plainly jerky; there is also involuntary grimacing. Once the disease picture has reached full devel- opment, the choreic character of the movements is unmistakable; we notice a continual gesticulating, there is no longer any quiet sitting or standing, short, motiveless movements occur, now in the hands and fingers, again in the feet, now an arm, now the head, and again a leg is jerked hither or thither; a wrinkling of the forehead, a smacking with the tongue, winking, protruding the lips, shows involvement of the muscles innervated by the cranial nerves. The French speak very appropriately of a "Folie musculaire" ("muscular madness"). In walking, the patient sometimes gives the impression of a "jumping jack.*' which moves when the string is pulled. In severe eases. however, walking is impossible, and the patient, condemned to keep her bed. throws herself about in alarming fashion. The muscles of the diaphragm, the oesophagus and the larynx may be involved (irregular breathing, disturbances in swallowing, broken speech) even in the pupils, alternating contraction of sphincter and dilator, a so-called "hippus," independent of the influence of light, accommodation or convergence, has been observed. Chorea almost always begins in the upper limbs and in the face, a Heeling the legs later. Not infrequently the limbs of one side are attacked first; as a rule, however, this original "hemichorca" sooner or later passes over to the other side also. Psychical excitement, even., the consciousness of being observed, increases the choreic phenomena, which, however, cease during sleep- only in the Very severe, fatal cases the intensity of the movements does not permit sleep, and the patient perishes from exhaustion ("Status ehoreicus" ). In chorea minor the psyche scarcely ever remains entirely intact; how- ever, its anomalies are usually inconsiderable; irritability, a high degree of dist ractibility, an ill-humored deportment, lability of mood. In the above- mentioned malignant eases, fortunately rare, on the contrary there may he hallucinatory delirium, and even maniacal attacks. 82 LECTURE V The rare symptoms of chorea are: slight rise of temperature (only in status choreicus and in complications with polyarthritis, endocarditis acuta, etc., is there high fever), motor weakness ('"Chorea mollis," incorrectly "Chorea paralytica"), pain along the spinal column. On account of the anemia often developing with chorea minor, sometimes anemic heart murmurs occur, which should not he confused with those of complicating endocarditis. On tapping the patellar tendon, it is sometimes noticed that the foot, which is drawn up by the contraction of the quadriceps, does not sink down again immediately, hut remains raised a few seconds before falling (''tonic reflex," Gordon's symptom). In general the prognosis is favorable, and recovery occurs with gradual lessening of the symptoms from one to three months after the beginning of the disease. However, there is often a tendency to relapses (these occur in about 25 per cent, of the cases). Less favorable in prognosis are the choreas oc- curring after the acute exanthemata and typhoid and those in adults in which the danger of endocarditis is very great. Most dangerous, however, is un- doubtedly chorea gravidarum with a mortality of 30 per cent. As to the pathological anatomy of Sydenham's chorea, the lesions found in the corpus striatum and in the optic thalamus by some investigators are of interest. Occasionally multiple emboli have been found in these parts, to which our attention had been already directed on account of symptomatic hemichorea, in cerebral hemorrhages (Broadhurst) ; more frequently the so- called "chorea bodies" (Elischer, Jakoteenko), concentric stratified, highlv re- fractile bodies are seen in the vascular sheaths. However, all these findings are still the subject of controversy. The therapy of chorea minor, when carefully carried out, usually gives very satisfactory results, and hence will be considered at length. On account of the great preponderance of the disease in late childhood and about puberty, I will give the doses of medicine for children of from ten to twelve years; from these, those suitable for younger and older patients can easily be cal- culated. Every case, even the mildest chorea minor, is during the whole duration of the disease to be prevented from going to school, to public nurseries, etc., also at home or in the hospital as strict isolation as possible should be en- forced. The patient should sleep in a single room, should not eat or play with the other children, and in the most severe cases, apart from the physician, should see only one and the same nurse. As a rule, the children can be allowed to amuse themselves with picture books, quiet games, dolls, etc.; also by unexciting reading (best read for them). Everything, however, in small "por- tions" only, with the interposition of long periods of complete rest. A full measure of rest in bed is to be prescribed, even in very mild cases ; for example, from 8 p.m. until 10 a.m., and from 2 till 4 p.m.. namely, sixteen hours in bed. In severe cases we order strict rest in bed until there is decided improvement ; in very severe ones, besides this, the sick-room is kept dark, and by suitable padding care is taken that the patient does not bruise or injure herself. The diet should be purely ovolacto-vcgetarian ; alcohol, coffee, tea, spices, and very salt food should be forbidden. To prevent constipation we give corn bread THE DYSKINESIAS 83 and plenty of fruit; further, taking as much milk as possible is of use; for example, a half cup every hour, eventually yoghurt, kefir, curds, etc. Where pure milk proves unpleasantly laxative, the addition of about a teaspoonful of the purest powdered gum arabic to the quart is to be recommended (mix first with some cold milk, add the rest of the milk and boil the whole). Daily tepid baths of about twenty minutes' duration, followed by rubbing down with water at from 22° to 25° C, act favorably also. On the other hand, you are expressly warned against cold douches and similar applications. From among the large number of drugs which have been introduced in the therapy of Sydenham's chorea, only three appear to me to have a specific effect: ar- senic, antipyrin and cannabis indica. Arsenic is incontestable the most effi- cient of the pharmacological agents, and should hence be preferred. In using it, it is desirable to begin with small doses after well-known principles, and gradually to increase them to large and very large doses. Chorea patients are especially tolerant of arsenic, and it almost never has to be withdrawn on account of digestive disturbances, herpes, or conjunctivitis. To antipyrin also great activity is attributed, especially by French pediatrists. Neverthe- less, whA it is the only drug given it is necessary to exhibit it in such large doses (3.0-6.0 grm. a day in children from 6 to 10 years old, 5.0-6.0 grms. in those from 10 to 15 years), that we must reject this method as too dan- gerous. The matter is otherwise in its combination with arsenic preparations in which small and safe doses of antipyrin may prove a good adjuvant to the chief drug. A combination of arsenic acid and extract of cannabis indica (provided a good and fresh extract is used) gives even better results. The "Pilulae cannabinae composita?," which I have recommended, have proved very useful. I£ Extract, cannabis indica; 0.3 (gr. 41/>) Acidi arsenios 0.04-0.12 (0.15) (gr. % to 1 ,'.-,) Quinin sulphat 1.0 (gr. xv) Extract, valerian q. s. Ut. f. pil. No. NXX. M. S. — One pill three times a day after meals (daily dose of extract can- nabis ind., 0.03, and acid, arsenic, 0.004-0.012, or eventually 0.015). Oilier prescriptions are: II Scil. Fowleri . t gtts. xxx-1 A(|. menth. pip 80.0 Sr. simp] 20.0 M. S.— A dessertspoonful in water three linns a day after meals. K Sol. Fowleri 10.0 Spts. melissse co 30.0 M. S. Ten (hops in milk, from one to three times a day. 84 LECTURE V I? Sodii arseniatis 0.01-0.03 (gr. %-V 2 ) Antipyrin 5.0 (gr. lxxv) Syr. aurantii cort 50.0 (§jss.) Aq. dest q. s. ad 150.0 (%v) M. S. A teaspoonful after meals. Daily dose, gr. 1/60 to gr. 1/20 sodium arseniate, and 71/> grs. antipyrin. Besides these drugs, it may be necessary to prescribe hypnotics for a longer or shorter time, as it is of great importance that the patients sleep long and deeply; it should naturally be endeavored to get along with the most harmless hypnotics possible and in order to prevent habituation, to change them often, using, for example, adalin, bromural, veronal and trional. Occa- sionally an evening dose of 2.0 grms. of bromide of potassium will be sufficient ; salicylic preparations are only indicated in choreas with rheumatic and cardiac complications. I recommend to you further (but only after the irritative symptoms of chorea are plainly on the decline) to let the patient practice slow, rhythmical movements of the extremities upon command, best while lying in bed. Even in the early stages as well as at the height of the disease, however, the undertaking of rational breathing exercises is to be recommended, since many chorea patients breathe badly and superficially. To this end I place a pillow under the sacral region of the patient lying on his back, and have him practice, for five to seven minutes at a time, deep, rhythmical inspiration and expiration, this once or twice a day. Whenever it is possible after the cure, the patient should spend from four to six weeks at some quiet resort. In anemic or delicate children a combination of this with salt baths or a course of iron is advantageous. Chorea gravidarum can furnish a cause for artificial abortion ; Sarvay gives the following indications for this: Prevention of the taking of nourish- ment by the violence of the irritative phenomena, loss of strength, decided alteration of the psychical functions. 2. HEREDITARY CHOREA, HUNTINGTON'S CHOREA In 1882 the American physician, Huntington, on Long Island, called atten- tion to a rare affection, which characterized in the main by progressive chorei- form muscle unrest and dementia, attacks chiefly individuals from 35 to 40 years old, and is usually marked by decided heredity. Sporadic cases, in- deed, sometimes occur, also it may begin between 25 and 30 or between 40 and 55 years of age, while only very exceptionally do the initial symptoms commence about puberty or even earlier. The disease has the tendency to begin at an earlier average age in each succeeding generation ("anticipatory heredity"). Still less frequently than in America, the disease occurs in the Old World, particularly in England and Germany. As exciting causes, psychic traumata, exposure to cold, the puerperium, excesses, have been brought forward. Syphi- lis and the acute infectious diseases, on the other hand, appear to play no role at all. Like chorea minor, the affection usually begins in the arms and in the THE DYSKINESIAS 85 face, later to attack the legs; also it is sometimes at the start a hemichorea. In contradistinction to Sydenham's chorea, however, patients with Huntington's disease when in a state of complete quiet and abstraction can suppress the shaking in carrying out intentional movements so that they can pursue their occupations. In sleep there is usually complete quiet of the muscles; psychi- cally a depression is evident which cannot be said to be motiveless, since the patients are only too well aware of the incurable nature of the family scourge which is coming upon them and of the mental failure which is approaching; in this stage suicide or attempts at this are common. Later the patient becomes apathetic with episodal conditions of excitement, the memory fails, sometimes there are delusions of grandeur or of persecution, also periods of hallucinatory confusion, mental failure continually advances, and there can be finally com- plete dementia. There are, however, cases with relatively little disturbance of intelligence, and there is even a variety of the disease beginning at puberty, which usually becomes stationary after some time, and does not alter the mental personality. Apart from this last form, the prognosis is very un- favorable; the disease can, indeed, last for twenty or more years, so that the patients not infrequently live to be sixty or seventy years old; death ensues in dementia and marasmus, or from intercurrent diseases. In its pathologico-anatomical relations we have a number of heterogeneous findings whose interpretation is not yet possible. The newest investigations are those of Alzheimer. He finds very grave changes in the cerebral cortex, as well as in the corpus striatum and in the nucleus ruber, namely, a sur- rounding and in part a destruction of the ganglion cells by the "amoeboid" elements of the glia. The therapy is purely symptomatic, and, as such, gives little prospect of results. Only in very early stages arsenic and scopolamin (V->-2 nag. per dose once a day) appear to be able to procure alleviation. It may be attempted to modifv somewhat the choreiform unrest by sedative hydriatic procedures, curative exercises, etc. It is best to bring these patients as soon as possible into the seclusion of an institution where, apart from the elimination of all irritants, intelligent care of the episodal states of excitement (baths, opium, veronal, rest in bed, etc.) can best be carried out. In the latest stages the care of the disturbed and demented patient is particularly difficult. E. The Athetoses In the year 1871 attention was called by the American neurologist II a m- monil to a peculiar phenomenon of motor irritation which he designated by the name Athetosis (afleTOff = restless). The hands and feet, that is, the individual fingers and toes of the patients, make constant, slow excursions^ beginning with hyperextension (analogous to the movements id' the tentacles of an octopus), which do not cease during sleep, hut up to a certain point can he controlled by an effort of the will. Fig. .'{1 shows one phase of athetoid movements of the hand after an instantaneous photograph. It refers to a child in whom the athetosis developed as a result of an acute encephalitis. In fact, focal lesions which are situated in the parts of the thalamus, some 86 LECTURE V times also in those of the lenticular nucleus, bordering on the internal capsule (more rarely also in the internal capsule itself, that is, in the posterior third of its posterior limb), can produce not only hemichorea, as already mentioned, but also hemiathetosis, on the opposite side. The last, indeed, is much more frequent than cerebral hemichorea. Children are particularly predisposed. There are indeed transition forms between hemichorea and hemiathetosis ; also in lesions of the basal ganglia on both sides there is a bilateral athetosis. Besides this symptomatic athetosis there is also an idiopathic form, the so-called "double athetosis," a disease in itself without a certainly determined anatomical substratum, in which the motor phenomena described present the only pathological manifestation, which usually is present from birth or develops in earliest childhood. Here, besides the extremi- ties, the muscles of the face and neck are also affected ; the patients continually distort the mouth, make faces, constantly turn and twist the head, and, on account of athetosis of the tongue muscles, are much embarrassed in speaking. The condition is a stationary one, although remissions occur. Whether their occurrence is to any extent favored by drugs, as arsenic, bromides, cannabis, or scopolamin, or by hydriatic procedures, is, however, a question. Related to double athetosis, probably, is a rare progressive and incurable disease, that Oppcnhcim has described as "Dysbasia lordotica progressiva," Ziehen as "torsion neurosis." It affects children between eight and fourteen years old (mainly Rus- sian Jews). The affection is characterized by a lordoscoliosis of the lower portion of the verte- bral column, evident only on walking and standing, usually disappearing when the patient is lying down, along with flexion and external or internal rotation of the legs. In the rotators of the thigh, also, in the tibialis anticus and in the biceps, along with tonic tension, clonic contractions may occur. The electrical irritability is unaltered. Athetosis (instantaneous photo- graph). F. Paralysis Agitans By this name James Parkinson, in 1817, denominated a disease charac- terized by muscular rigidity and peculiar shaking movements, which in honor of its discoverer has also been called Parkinson's disease. Paralysis agitans appears in the vast majority of cases between the fortieth and the sixtieth years of life, and in general affects by preference the male sex; many adthors assert that the poorer classes of the population are more frequently attacked. Unexplained conditions of local nature appear here to exert an influence; since in our neighborhood, where the disease is relatively frequent, there is no evidence of a less predisposition to it in the higher classes of society. Its THE DYSKINESIAS 87 etiology is quite obscure; mental stress, physical overexertion, exposure to cold and trauma, have been accused; it is probable that these factors may occasionally play the role of exciting causes (namely, when the first symp- toms of the disease appear directly in an overstrained or traumatized limb), but in a majority of cases there is nothing of the sort, and we must confess our lack of knowledge with regard to the real causal connection. The hered- itary occurrence of the disease has been only noted as a great rarity. Parkinson's- disease usually begins gradually and unremarked; the patient notices a slight general fatiguability, little by little a temporary tremor, which is at first limited to one hand (oftener the right), but gradually becomes a permanent symptom, and finally attacks the remaining extremities one after another. At the same time a continual tension of the muscles which slows all movements is added to the tremor; in a minority of the cases the last phe- nomenon precedes the appearance of the tremor. In the fully developed disease the tremor presents the following charac- Fig. 32. Paralysis Agitans. Handwriting. tiers: it is rhythmical, slow (two to five oscillations a second), persists during complete rest, to cease during skip, and in the hands imitates certain com- plicated movements, namely, "pill rolling" and "coin counting." On carrying out a movement the tremor usually lessens, and indeed, when the disease is not far advanced, may cease, so that, for instance, a hunter, on aiming, can keep still and make the shot. Less frequently the tremor increases somewhat upon movement. Excitement always increases the shaking. While at the start, writing, eating, dressing, etc., are still possible, in the advanced stages (tin- disease can last twenty years or more) these functions are impossible. The tremor of the legs makes itsc] f evident bv the patient, when seated, "beat- ing time" with his foot; Usually the oscillations in the upper and lower ex- tremities are synchronous. The attitude of Parkinson patients is, as a rule, uncommonly typical (see Fig. •'!•■>,. The hiad and trunk are held forward, arms and legs addueted. knees and elbows flexed, in the hands the metacarpophalangeal joints flexed, the middle and end phalanges extended, the finger-tips of the thumb and index finger held together. This attitude is maintained so firmly that all the joints mighl be thougl I > be contractured. (Only exceptionally do the pa- LECTURE V tients stand straight upright in statuesque stiffness — Charcot's "extension type"). The features have the immobility of an' antique mask, only the eyes move in a lively manner; in looking, turning of the head is avoided, the patient turns himself rather, as a whole, about his axis. This occurs slowly with the aid of a number of small steps ; especially all locomotion is usually begun with very slow, deliberate steps, so that the gait, indeed, has something solemn about it; little by little, however, the steps often become more rapid, "tripping," the bent forward pa- tient "runs after his center of gravity," in order not to fall, and finally, in order to stop his for- ward movement, has to bring up against the wall or some other object. This we call "pro- pulsion." Analogous phenomena are "retro- pulsion" and "latero-pulsion," which we make evident by giving the patient a slight push back- ward or sideways. Parkinson patients, on ac- count of the hypcrtonus of all their muscles, as well as on account of the fact that they can only carry out an intentional movement with more or less delay, no longer have the power of regaining the balance of their bodies which have been put out of equilibrium. Disturbances manifest themselves also in the speech muscles ; the voice is weak and plaintive, the speech slow, sometimes chopped off, remind- ing one of that of a rider upon a horse which is trotting fast. In two of my patients I ob- served a continual chattering of the teeth. Sensibility is for objective tests practically always normal ; exceptionally there is slight re- duction of sensation in the hands and feet. On the other hand many patients complain of drawing sensations in the extremities, of cramplike pains in the calves, of paresthesia in the face ("as if something was crawling on it"), prin- cipally, however, of a burning heat of the surface of the bod}*, particularly upon the abdomen and upon the back. On this account patients with paralysis agitans gladly uncover themselves while in bed. The reflexes are usually exaggerated, less frequently normal. The electric muscular irritability is unaltered. The "paradoxal foot phenomenon" of West- phal is often found; if one brings the foot of the patient into a position of extension (dorsal flexion) the tibialis anticus, extensor digitorum, and peronei contract, since their origin and their insertion are brought nearer together, so that the foot remains for a long time in the position given it. In most cases there is often a very distressing salivation. Hyperidrosis is rarer. A not very infrequent variety of paralysis agitans is Parkinson's disease without agitation. In this, with otherwise typical symptomatology, the characteristic tremor is absent. Typical Attitude in Paralysis Agitans. THE DYSKINESIAS 89 The prognosis of the disease is not unfavorable as to life, but very un- favorable as to recover}' ; remissions occur, but unfortunately they are not usually of long duration. In the late stages the fate of the unhappy invalid is a wretched one, since with fully retained intelligence he is condemned to absolute helplessness, and must depend upon outside aid for the most ele- mentary needs (for example, for turning himself in bed, wiping off the saliva that flows from his mouth). Death follows either in marasmus or from inter- current diseases, among which cerebral hemorrhage is relatively frequent. What is the anatomical basis of this terrible disease? As to this question it was formerly classed among the "functional neuroses." To-day, however, it is known that in the spinal cord in Parkinson's disease, proliferation of the glia and abnormal pigmentation of the cells of the anterior horn, together with changes in the vessels, are to be found (Redlieh, Sander, Dubief, Ballet), also the muscles show changes according to some authors (Blocq, Gautliier) . Nevertheless, it is not far-fetched to consider these not very characteristic lesions, which may be interpreted as an accentuation of senile changes, as only secondary, and to think of another "primum movens," perhaps in the glandular apparatus of our bodies. So Roussy and Clunet would make the parathyroid glands responsible, claiming to have found in them changes which they desig- nate by the expression "Hyperparathyroidosis." According to Haberfeld, indeed, the changes found are due only to senility. The question is still open. With regard to the treatment of Parkinson's disease, Oppenhcim's remark is recommended for your consideration: "The physician can in this disease do much harm and little good." In order to avoid the first, one should refrain from trying cold water treatment, exercises, sun-baths, vigorous massage, faradization, while — indeed, seldom enough — carrying out careful passive movements, giving tepid baths (or the cooler, indifferent warm baths, and even electric baths) and a mild vibration massage can alleviate the symptoms. More useful as palliatives are certain drugs, above everything hydrobromide of Bcopolamin, either in the form of injections or as tablets. Of this, from ().()()()•_' to 0.0004 (1/300 to 1 1.50 gr.) once or twice a day: also duboisin sul- phate in the same dose, usually moderates the tremor and muscular tension con- siderably. I have given both medicines, sometimes alone and sometimes com- bined, for years, without having observed any symptoms of intoxication. One must not decide upon this continuous method of administration too soon, since it is a last refuge. As long as the disease is not at a too advanced stage it is well to give this medicine from time to time for a period of twenty days; in the interim an arsenic cure is to be recommended; for example, Sol. Fowleri, in doses of two drops, increasing from twice to seven times a day. and then slowly returning to twice a day; also the drinking of arsenic containing waters [Dwrkhevmer-Mawquelle, Val Sinestra, Levico) can be carried out after the manner recommended at these resorts. Of other drugs recommended instead of Bcopolamin and duboisin, from my own experience I can mention as occa- sional! v efficient, tinct. veratri viridis (two or three times a day, 8-4 drops in thin mucilage). To be strictly avoided are all drugs increasing sweating, namely, the salicylic preparations. Most patients who have been given para- thyroid substance experimentally have reacted with an increase of their trouble 90 LECTURE V (I could convince myself of this in one case), which would support the views of Roussy and Clunet. Placing the patient among as quiet surroundings as possible, preventing all exciting visits, pleasures, etc., are important ; still more important in the later stages is the choice of a nurse of inexhaustible patience and great skill in carrying out all the technique of the difficult and tiresome nursing. G. The Myotonias A peculiar disease condition was made known by the Schleswig physician Thomsen in 1876. His material was furnished by his own family, which in five generations had presented over twenty cases. This "Thomsen's dis- ease" later received the name of Myotonia congenita. In the vast majority of cases it is observed as a hereditary family complaint ; sometimes consan- guinity of the parents is to be recognized; very frequently in the relatives a heaping up of the most varied psychoses and neuropathies are found. As exciting causes acting upon the latent predisposition, physical excesses play the greatest role; rarely fright or trauma, very rarely infectious diseases, are accused. The disease is either first noticed in early childhood, or it begins about the time of puberty, or somewhat later (for example, at the time of entering the army). Ninety per cent, of the cases are in males. In general the symptoms of the disease increase slowly and continually during a number of years, then become stationary; later, indeed, the intensity of the symptoms may again decrease, though such remissions are not very frequent ; a recovery, on the other hand, is excluded. The disturbance pathognomonic of Thomson's disease is characterized by the persistence of a condition of muscular contraction opposed to the intended end on the carrying out of a voluntary movement. If the patient, after long periods of rest, attempts to carry out any movement, this follows in the promptest manner ; the groups of muscles brought into contraction together can, in spite of every effort of the will, be relaxed again only after from 5 to 30 seconds. For example, if we ask the patient to shake hands, he cannot let go for some time. If, however, the patient undertakes the movement a second, third, or fourth time, we notice that the inhibition of relaxation ceases each time after a shorter interval, so that finally the movement can take its normal course. If these patients march, at first they stand as if rooted to the ground, then' follow the first most difficult steps which are interrupted by repeated tonic contractions. Little by little, however, "the machine gets going," and finally long distances are traversed without any trouble; the patient can even dance. All voluntary muscles can be the seat of the myotonic disturbances of movement. However, as a rule, the muscles of respiration are free, those of the trunk and neck less involved than those of the limbs, and of these last the upper less than the lower. The muscles of the face, of mastication, the external eye muscles, the tongue, and even the muscles of the pharynx and larynx, can be affected; while the myotonic dyskinesia makes itself apparent on grimacing and putting out the tongue in most cases; in phonation, turning the eyes, swallowing and chewing, it is THE DYSKINESIAS 91 seldom observed. Psychical excitement, cold and dampness increase the myo- tonic symptoms. Almost always there is increase in volume of the muscles, on account of which the patients, in spite of usually somewhat subnormal strength, present an athletic appearance. The tendon reflexes are either normal, they exhibit tonic contractions, or they are reduced ; they are seldom absent. The mechanical and electric mus- cular irritability are, as Erb has shown, very characteristically altered. Per- cussion of the muscles sets up the formation either of a depression or of a swelling which does not disappear for from 5 to 30 seconds. Direct faradic stimulation of muscles shows increased irritability; on stimulating with strong currents persistent myotonus appears, on continued strong faradization there is sometimes a decided "muscle wave." Direct galvanic irritation brings out the most striking anomalies; slow and persistent character of the contractions, abnormally low threshold of irritation, loss of the contractions on opening the circuit, AnCIC = or >KC1C; besides this, sometimes upon the stabile appli- cation of strong currents there are rhythmical undulations from the cathode to the anode (sometimes only appearing after repeated application of the current). These different anomalies Erb sums up as the "Myotonic reaction." As a pathological change a uniform hypertrophy of all the fibers depending upon an increase of the undifferentiated protoplasm is found, while the con- tractile substance and its morphological expression the striation is defective. The sarcolemma shows increase of nuclei, the fibrilli vacuolization. Besides this there is slight increase of the interstitial connective tissue. Knoblauch thinks that there is an abnormal preponderance of the red, slow-acting muscle fibers as compared to the light, quick-acting ones, and that there is a hyper- trophy of the first. Along with these muscle changes, opposing relations of innervation can play a role, as Jaqnct has shown by myographic investigations. From a therapeutic point of view, above everything else prevention of the factors, which are shown by experience to work unfavorably, is important ( keeping the patient warm, prevention of emotional excitement, suitable occupa- tion). Further, systematic exercises, warm baths and massage can alleviate the troubles. The drugs here and there recommended (strychnine, antipyrin, testicular and thyroid extract, atropin, iodide of potassium), since they are useless, have been entirely given up. A moderate amount of alcohol, on the contrary, acts favorably. The formerly practiced nerve stretching, which has u its object causing decrease of the muscular hypertrophy by injuring the nerves, is to be warned against. Varieties of myotonia are: I'. Myotonia atrophica, a combination of Thom- sin's disease with progressive muscular atrophy, to be described in the nexi lecture ; this is not at all rare. -. Myotonia acquisita {Talma, Jolly), an incurable condition character Bed by the myotonic reaction which follows infectious diseases and traumata, in which, however, there is usually a certain muscular rigidity even when al ii^l, while long-continued movement favors the occurrence of myotonus. .'5. Paramyotonia congenita (Eulenberg), \f the limbs, which, as a rule, remain intact even in the later course of the disease. In the muscles under- 97 98 LECTURE VI going atrophy, reaction of degeneration is almost never observed, but only simple quantitative reduction of irritability. Fibrillary contractions are also absent. The usually symmetrically affected muscles show a particularly char- acteristic peculiarity, namely that, along with the atrophic processes there occur changes which cause an increase in volume of the affected parts and striking abnormalities in the form of the body; the combination of this "pseudo- hypertrophy" of certain muscles with atrophy of others is comparatively reg- ular; sensibility, the function of the sense organs, the sphincters of the bladder and of the rectum remain normal. Along with cases impressing themselves by typical heredo-family occur- rence in which transmission occurs chiefly through the mother, who herself, however, can remain free from the disease — I designate this last as "maternal metrapectic inheritance,"* — come also sporadic cases, namely, in the "juvenile scapulohumeral type" (Erb) which we will consider more at length under the discussion of the special forms of dystrophy. Not infrequently infectious diseases, trauma, or overexertion give the signal for the outbreak of the disease; the same factors also may cause new progress after temporary arrest. Pathological J natomy The disease process which lies at the basis of this trouble has been thor- oughly studied by Erb and Marinesco. Macroscopically the "fish flesh" ap- Fig. 35. Alteration of the Muscle in Progressive Muscular Atrophy. Hematoxylin-eosin-osmic acid stain. Gastrocnemius. Zeiss Oc. 2, Obj. E. pearance of the affected muscles, which are plainly distinguishable from the healthy brown-red muscles, but hardly at all from the surrounding fatty tissue in color, is particularly striking. In sections (see Fig. 35) the striking in- equality in size of the fibers is first noticed. While, according to Erb, the extremes in the normal cross sections of the fibers vary between 20 and 80 * A?rexc"'==To avoid, tt\ ht)tt)p = The mother. THE PROGRESSIVE .MUSCULAR ATROPHIES 99 microns and ninety per cent, of the fibers measure between 20 and 60 microns, among a larger or smaller contingent of such normal sized fibers are found numerous atrophic ones measuring from 7 to 15 microns, also hypertrophic ones of 100, 125 even 200 microns and more. The hypertrophy of the fibers seems to represent the first stage of the process, the atrophy the latter one; it finally passes over to entire loss of fibers when only empty sarcolemma sheaths are left. One need not think that in pseudo-hypertrophic muscles the thick, and in macroscopically atrophic muscles, the thin, fibers especially pre- dominate, since -nothing is more like the parenchyma of an atrophic muscle in dystrophy, under the microscope, than the tissue of a pseudo-hypertrophic one. Determining the total volume, indeed, is the smaller or greater degree of proliferation of fat and connective tissue. Both can remain within moderate bounds or may reach an excessive degree. The muscle fibrilli almost all show a considerable increase of both the border and of the interior nuclei. Many are fragmented, split, or show in the central part, round vacuoles that look as if punched out. However great the atrophy of the fibers, the striation is plainly preserved everywhere. In the areas most diseased the longitudinal striation usually seen is increased to definite fibrilla- tion; upon longitudinal sections the muscles sometimes appear bordered with rosary-like swellings. The intramuscular vessels undergo to a great extent en- doarteritic and mesoarteritic changes which can cause great thickening of their walls; in the periarterial connective tissue, and also in the perimysum, consid- erable increase of nuclei is often found; this can increase to definite areas of infiltration. In contradistinction to these constant changes in the muscular tissue, the lesions in the anterior horn cells of the spinal cord, found in only exceptional cases, fall entirely into the background. Since, however, the trophic centers for the muscles lie in these last, it is conceivable that functional alterations of these cells are responsible for the perverse method of develop- ment and disturbances of nutrition in the muscles, even in those cases in which the anterior horn cells appear morphologically unaltered. CLINICAL BEHAVIOR OF THE DYSTROPHIC MUSCLES Upon investigating the muscles of dystrophic patients we will find naturally different conditions depending upon whether we are observing an atrophic or a pseudo-hypertrophic region. In the first case the muscles arc either uniformly thin as a whole, which process can go so far that the affected portions of the extremities look like those of a skeleton, or in the jniddle of the muscle among the atrophic parts. round, sometimes globular prominences, are found. This phenomenon, which sometimes is first seen when the muscle contracts, is entirely pathognomonic of muscular dystrophy and appears as "partial pseudo-hypertrophy" to present a transition to the second manner of appearance, dystrophic myopathy. In tliis last form the affected muscles increase in mass to such an extent that ath- letic contours are produced (Fig. 86). On palpation one experiences in them either a firm, often peculiar fluctuating rubber-like resistance, or (in advanced stages) on the other hand, a peculiar dead feeling. Here and there I have seen 100 LECTURE VI linear atrophies of the skin like the well known striae of pregnancy in women, over such pseudo-hypertrophic muscles. It can happen that a muscle whose ability to functionate is almost suspended by the dystrophy, presents a normal outline; in it the atrophic and the pseudo-hypertrophic process balance one another. That atrophic or pseudo-hypertrophic muscles shorten and produce contractures like that in Fig. 38, as well as that visible in the left leg of the boy in Fig. 36, is not a frequent occurrence. With the morphological anomalies related, disturbances of function go hand in hand ; the strength and the extent to which the affected muscles are able to Fig. 36. Progressive Muscular Dystrophy. Pseudo-hypertrophic form. contract, in the course of the disease continually grow less; at the same time the electrical and mechanical irritability continually decrease, to be finally lost. The exceedingly rare cases, in which, besides this, indications of reaction of degeneration are found, are usually otherwise atypical and probably should be considered as transition types to the neural and spinal forms of progressive muscular atrophy. The tendon reflexes in the affected region decrease and are finally lost. Progressive muscular dystrophy has its "favorite muscles." Other muscles it attacks only exceptionally, still others it regularly avoids. As to this peculiar THE PROGRESSIVE MUSCULAR ATROPHIES 101 electivity, you may be guided by the following table in which I have also indicated, by italicizing, the muscles which are particularly predisposed to pseudo-hypertrophy. Usually Affected. Pectoralis major (pars ster- nalis) Pectoralis minor Latissimus dorsi Serratus anticus major Rhomboideus Trapezius (especially pars inferior) Erectores spinae Deltoid Biceps brachii Brachialis antieus Supinator longus Olutei Quadriceps Adductores femoris Peronei Tibialis anticus Gastrocnemius Orbicularis oris Orbicularis palpebrarum Less Frequently Affected. Sternocleidomastoid Infraspinatus Supraspinatus Levator scapula; Coracobraehialis Triceps Teres major Teres minor Abdominal muscles Sartorius Tensor fasciae Iatae Exceptionally Affected. Diaphragm Masticatory muscles Eye muscles Heart muscle Tongue * TYPICAL ANOMALIES OF CONFIGURATIONS AND ATTITUDE Looking at the nude bodies of dystrophic patients we are impressed by the fact that the deep depressions of the atrophic parts and the great swellings of the pseudo-hypertrophic ones, join immediately on to the well- formed portions of the limbs. On this account the whole figure takes on an inharmonious out- line. Besides this, however, we come face to face with a number of exceedingly characteristic local disfigurements, for example, the picture of scapula- alatse which occupied us already in the first lecture; this occurs on account of elimination of the action of the serrati (see Fig. 37). If to this is added, how- ever, that of the pectorales, the trapezii and the latissimus dorsi, the shoulder- blades with the attached arms become loose appendages of the thorax, and we speak of "loose shoulder." The atrophy of the sternal portion of the pectoralis causes a flattening of the thorax; under certain circumstances, indeed, the sternum and ribs form a sort of a fissure. Decided lordosis occurs on account of the fact that extension at the hip-joint is impaired and the center of gravity must be moved forward; tin' weakening of the contractions of the abdominal muscles can increase this disfigurement to the highest degree; between the approximated shoulder-blades and the hips, a number of cross folds produced by the forcing together of the soft parts are observed. The "gnomes calves" occur on account. of the pseudo-hypertrophy of the gastrocnemii, the "tapir snout" through the trunk like increase of volume of the upper lip. Through imperfections of the orbicular muscles of the eves and the mouth the "myopathic fades" is produced, the gloomy rigid "Sphinx countenance." If •Our patient of Fig. 36 showed {.Teat enlargement of the tongue. Besides this nn enlarge- ment of the heart was demonstrable by percussion and by orthodiagraphy. 102 LECTURE VI such a patient laughs, however, on account of the imperfect function of the orbicularis oris, his mouth is drawn into an ugly fissure ("transverse laugh- ing") while the two nasolabial folds impose themselves ps deep vertical furrows, the "coups de hache" of the French; the atrophy of the trunk muscles can lead to the so-called "wasp waist." INDIVIDUAL FORMS OF PROGRESSIVE MUSCULAR DVSTROPHY We have passed before us the characteristic anomalies which fully de- veloped cases of dystrophy present. We must now, however, go into the Fig. 37. Progressive Muscular Dystrophy. ('type "Erb.") Winged Scapula. definite grouping of symptoms which, characterized also by peculiarities as to the time of beginning and course, impose themselves as special types of dystrophy. We distinguish: 1. Forms with initial and preponderant involvement of the pelvic girdle and thigh muscles. a. Atrophic variety (type, Leyden-Moebius) . b. Pseudo-hypertrophic variety (type, Duchennc-Gr'u singer). 2. Forms with initial and preponderant involvement of the shoulder- girdle and upper arm muscles. a. Juvenile, scapulo-humeral variety (type, Erb). b. Infantile, facio-scapulo-humeral variety (type, Landouzif-Dejerlne). The pelvic-femoral forms (la and lb) begin, as a rule, in childhood and affect by preference boys. The atrophic variety usually begins later than the pseudo-hypertrophic; the first usually in the 8th to 10th year of life, the latter about the 3d to 5th year. The first symptom which usually strikes the THE PROGRESSIVE MUSCULAR ATROPHIES 103 parents is a waddling gait caused by weakening of the gluta'i. The children fall easily and experience great difficulty in climbing stairs. Often, on walk- ing, they let the feet fall stamping, a result of the quadriceps paralysis. A lordosis is usually early apparent also. If we lay such a child upon the floor and then let him rise, on account of the weakness of his back, thigh, and leg extensors, he is compelled first to assume the position of a quadruped and then, by aid of his hands, to climb up his legs, as it were. In the pseudo-hyper- trophic Duchenne-Griesinger type, the great increase in volume of the calves and buttocks makes itself early evident (Fig. 36) ; while in time the disease also involves the shoulder-girdle and upper arm muscles, pseudo-hypertrophy Fig. 38. Excessive Clubbing of the Foot on Account of Contracture of the Gastrocnemius in Progressive Muscular Atrophy. usually dots not occur in these; later, indeed, it falls off in the glutei and gastrocnemii to give place to atrophy. The muscles of Hie face are later and less regularly affected in the hypertrophic variety than in the atrophic. Of the two shoulder-girdle upper arm types, that of Erb, the "juvenile" (2, a, sec Fig. 37) begins in youth (or even during the third decade of life) and usually develops \rv\- slowly, while the Landouzy-Dejerine, or "infantile" variety, commences in early childhood and spreads somewhat more rapidly. In tin- muscles of Hie shoulder-girdle and upper arm mentioned in the table on page 101, in I lie infantile type at the same time in those of the face, an increasing weakness and atrophy become apparent. In ErVs type pseudo- hypertrophy is common (in the deltoid, triceps and infraspinatus), in the Landouzy-Dejerine type il is almost always absent. Only when the last has later attacked ihe legs also, it may cause increase in volume of the calves, like Erb' s t vpe. After we have exposed the four chief forms of progressive muscular dystrophy in their chief differential points, we will not neglect to point out how very frequently atypical cases come under observation; whether it is, that the time and the topography of the commencement of the diseases do not 104 LECTURE VI agree with one another (for example, when a pelvic and thigh form does not begin until the end of the twentieth year or even later), or that the grouping of the affected muscles, or the order of their involvent presents unusual relations. Differential Diagnosis All in all, one can say that progressive muscular dystrophy in its fully developed stage can hardly be confused with any other disease; as to its special differences from the "spinal" and the "neural" forms of progressive muscular atrophy, I will refer in this connection to a description of these last. In the early stages, on the other hand, one must be on his guard against diagnostic errors. The abnormal manner of rising occurs in beginning spondylitis, in circumscribed acute lumbar polyomyelitis, in abnormally localized post diphtheritic paralysis, in rickets; the waddling gait in rickets and osteomalacia; the "gnome-like" calves in spastic spinal paralysis and in hydrocephalus; myotonia congenita runs its course with increase in volume of the muscles, this can occur locally, also, as a congestive phenomenon in throm- bosis of veins ; finally, there is a genuine congenital muscular hypertrophy. Tlje opposite to the last condition is furnished by congenital muscle defects which, indeed, attack by preference the "favorite muscles" of dystrophy (Erb, Bing, etc.), but are almost always asymmetrical. Course and Prognosis Though pauses for ten, fifteen, and even thirty years may interrupt the progress of muscular dystrophy, nevertheless its course is almost always steadily, though slowly, progressive. In general it is the rule that in the average the cases beginning early and the pelvic-femoral forms run the most rapid course. Such patients, as a rule, die comparatively young, since the earlier they become permanently bedridden, the more threateningly hangs over them the Damocles' sword of tuberculosis and broncho-pneumonia, to which most of them fall victims. Patients with the juvenile type of the disease, how- ever, may reach the age of seventy years or more. That progressive muscular dystrophy is the direct cause of death is exceedingly rare ; in such cases the result depends upon the involvement of the diaphragm and respiratory muscles ; still rarer, however, are recoveries of typically dystrophic children, as they have been observed by Erb and Marina. Treatment What can, and shall we do, not to put a stop to this fatal disease (since this does not lie within our power), but at least to oppose its progress as much as possible? In the first place it is necessary to improve the general condition as much as possible by good air and by nourishing food, not too rich in fats and carbohydrates. Care should be taken that daily such an amount of exer- cise and movement as his condition permits, without the production of a feeling of fatigue, should be carried out by the patient. "Ne quid nimis" THE PROGRESSIVE MUSCULAR ATROPHIES 105 should also be the motto in treatment by massage and electricity ; the last I apply only in the form of the "electric hand" with very weak faradism, or as galvanization, and I would warn you directly against producing faradic con- tractions of the muscles. I have here and there seen good results from salt baths beginning with very weak concentrations and increasing gradually to moderately strong ones, also from prolonged tepid baths and corresponding indifferent thermal ones, that is, a decided improvement in the function of the muscles. As regards attempts at medication, however, I can only report failures from the much used strychnine and from thyroid and hypophysis preparations; also the results of injections of muscle juice vaunted by certain enthusiasts have, as was to be expected, remained unconfirmed. Not infrequently we are in a position to call in the aid of the orthopedist; in con- tractions of the dystrophic calf muscles tenotomy of the Achilles tendon may be of use; in very slowly progressive cases with suitable topographic distribu- tion of the still healthy and of the diseased muscles, tendon transplantation, in "loose shoulders" fixation of the scapula' to the thorax (scapulo-pexia) or approximation of the scapula; to one another, further, the correction of scoliosis by supportive apparatus, etc. From a prophylactic point of view I would advise you to take to heart the idea that every intercurrent disease in dystrophic patients, even the slightest one, and especially bronchitides, should be treated from the start with special care. B. The Neural Form This variety of progressive muscular atrophy is characteristically an heredo-family disease. "Erratic" cases are quite rare, while, on the other hand, up to thirty cases have been counted in the same family. The male members of these families are attacked by preference, if not exclusively. As exciting causes, infectious diseases and overexertion are accused. This form is also called "The Charcot-Marie type" of progressive muscu- lar atrophy. Besides these two authors. Tooth and Hoffmann have rendered service in establishing the clinical picture of this disease. The term "neurotic" muscular atrophy, introduced by the last, should naturally be corrected into "neural," since the disease has not the least thing to do with the neuroses. Symptomatology, Course In certain rare forms of lead paralysis, in which the small hand muscles are albeted with paralysis and atrophy before the radial muscles. The antecedents of the patient (occupation, lead colic) and the objective signs of saturnism (blue hue on (he gums, tremor, hard pulse, anemia) are to be looked for. Still other intoxications (arsenic, alcohol, bisulphide of carbon) produce similar clinical pictures. The differential points between this disease and amyotrophic lateral sclero sis, syringomyelia and spinal gliosis, spondylitis and hypertrophic cervical 110 LECTURE VI pachymeningitis are furnished by the difference in the reflexes and in the sen- sibility. They will be described in connection with these diseases. At this point we might also consider the so-called "chronic anterior polio- myelitis," a disease whose separation from spinal progressive muscular atrophy is still sub judice. It is a rare disease of mature age, which is possibly of auto- toxic origin — for example, a number of cases in diabetics have come under ob- servation. The disease begins usually in the legs, more rarely in the muscles of the shoulder-girdle, as weakness which, little by little, passes over into paraly- sis and progresses by no means so slowly as the Aran-Duchenne disease. Ex- tensive segments of the extremities are attacked simultaneously, reaction of degeneration and paresis or paralysis precede the atrophy, and its extension is not by leaps. The disease can come to a standstill, indeed recoveries have been observed. On the other hand a relatively rapid fatal termination (in from 1 to 3 years) as a result of asphyxia, aspiration pneumonia, etc., is to be feared. Prognosis and Treatment The prognosis of progressive spinal muscular atropny as regards recovery or even as to definite standstill, is altogether bad. Nevertheless the progres- sion of the disease is almost always a very slow one extending over decades, and a direct menace to life through bulbar paralysis or respiratory failure is comparatively rare. The treatment can only be inspired by the hope of slow- ing and stopping the process for a time and of supporting functional compen- sation. Moderate and careful exercise and massage of the muscles not yet affected is the most important thing; also labile electrical treatment with weak currents, the cathode on the affected muscles, can act favorably. I would warn you, however, against all energetic measures, also against faradization, and advise you above everything to leave the already affected muscles entirely at rest. Further, galvanization of the spinal cord, the technique of which will be considered in the next lecture will come into consideration. As to drugs, strychnine injections (0.0015 (gr. 1/40) once a day) may be tried according to Goiters' recommendation ; the formerly much-vaunted silver nitrate and ergot cures are certainly entirely useless. Bath cures and hydrotherapy also ac- complish nothing; when the patient demands them one should limit himself according to the motto "non nocere," in that care is taken to avoid excesses, and above everything, cold water treatment. LECTURE VII While in the last lecture we have become acquainted with clinical pictures whose anatomical substratum has its location partly in the peripheral (spino- muscular) motor neurones, partly in their end apparatus the muscles, we will turn our attention to-day to degenerative affections in which the central (cor- tico-spinal) motor neurones are affected by the disease process. Since this occurs with avoidance of neighboring but physiologically different fiber sys- tems, that is in elective manner, we speak of "system diseases" in contrast to the "diffuse diseases" of the nerve centers (as, for example, inflammatory af- fections, multiple sclerosis, syringomyelia, etc.). We will begin with the study of the so-called spastic spinal paralysis, al- though this clinically very typical disease picture, as I will remark in advance, is not to be considered as the expression of a unique and definite disease of the spinal cord, but rather presents a syndrome which occurs in the course of a heterogeneous, but without exception, organic set of spinal affections, either to establish itself definitely or to pass over into other symptom complexes. Spastic Spinal Paralysis In 187-5 and 1876 Erb and Charcot, independent of one another, under the respective designations of "Spastische Spinalparalyse" and "Tabes dorsal Spasmodique," described for the first time, a disease condition which they cor- rectly suspected, and which we to-day know certainly, to depend upon more or J'-- symmetrica] lesions of tic spinal pyramidal tracts, in whose territory cortico-spina] conduction is broken. Although indeed it was originally as- sumed thai this must be always an elective degeneration, a system-disease, of that neurone, later experience has proved the untenableness of this view; so, for example, compression of the spinal cord (by a tumor an exotosis of the vertebral column, etc.) can manifest itself for a long time only by symptoms of involvement of the pyramidal tract, in other words by the phenomena of spastic spinal paralysis. The same thing occurs in "multiple sclerosis," which is characterized by the dissemination of anas of proliferated glia throughout all the nerve centers, also in many brain diseases, for example in internal hydro- cephalus. It hence seems as if the pyramidal tracts, mainly in their distal por- tions, arc easily disturbed in their integrity by the most varied pathological conditions. On this account one must always lie aware that in making what is, as we will sic, the exceedingly easy diagnosis of "spastic spinal paralysis" he is men- tioning only a syndrome, not a disease, and should investigate industriously 111 112 LECTURE VII and carefully further, if new symptoms do not present the situation in another light. The clinical picture usually unfolds itself so slowly and gradually that only after years does it unmask itself as an expression of one of the disease conditions mentioned above. There remain finally, however, as Minkowsky, Bernhardt, Dejerme, Schultze, and others have shown, still cases which prove to be due to a purely systemic degeneration of the cortical-spinal tract in its spinal portion, a degeneration with which a decided reactive or com- pensatory glia proliferation (sclerosis) goes hand in hand. Indeed, we have learned through St rum pell that true spastic spinal paralysis can occur as a hereditary family disease, a proof that it may depend upon endogenic factors. A reduced power of resistance of the pyramidal tracts from the start, perhaps also plays a role in those forms which are set up by exogenic factors; for ex- ample, anemia, carcinosis, syphilis, the puerperium, lead poisoning, lathyrism (chronic intoxication hy the use of spoiled chick peas — lathyrus cicera and L. sativa). All these factors, however, frequently lead, instead of to isolated pyramidal degeneration to simultaneous involvement of the lateral and posterior columns, the so-called system diseases in which motor and sensory phenomena are united. This last is also the case in pellagra, an intoxication from spoiled corn-Hour endemic in Lomhardy, in Roumania [and of recent years observed in the United States — Translator]. Symptomatology In full development of "spastic spinal paralysis" there are in both legs, only in a small number of cases also in the arms, the anomalies which we denominate the spastic symptom complex, and which pathologically are to be referred to the elimination of the function of the pyramidal tracts in a longer or shorter portion of their spinal course. Physiology of the Pyramidal Tracts The pyramidal tracts or cortico-spinal tracts have, as is known, their origin in the motor region of the cerebral cortex. They pass through the internal capsule, the crus cerebri and the pons to the medulla. There a separation takes place into the lateral pyramidal tract which passes, by way of the "decussa- tion of the pyramids," into the opposite lateral column of the spinal cord, and the anterior pyramidal tract which descends on the same side in the anterior column. Both categories of fibers end about the cells of the anterior horn at different levels of the spinal cord, those of the anterior pyramidal tract, how- ever, only after they have passed through the anterior commissure of the spinal cord, that is, have crossed to the opposite side. Through these pyramidal tracts, now, the psycho-motor impulse is trans- mitted from the cortical motor centers of the cerebrum to the spinal centers of the different muscles of the body. But not exclusively by this path. Rather are these chief motor tracts assisted in their task by a number of accessory motor tracts, by fiber tracts which I have included under the name "sub-cortico- spinal tracts"; their points of origin (the red nucleus, the optic thalamus, the SPASTIC SPINAL PARALYSIS 113 roof of the mid brain, Dcitcrs' nucleus) are connected with the motor region f the cerebral cortex, so that, thanks to these last, even on elimination of the pyramidal tracts, part of the psychomotor impulses can be transmitted in a roundabout way to the cells of the anterior horn and hence to the muscles. Now, however, the pyramidal tracts also exercise an influence upon the im- portant mechanism of the regulation of the tonus already mentioned and also upon the production of the tendon and bone reflexes. They exercise an in- hibition, in that they take care that reflex action and the degree of tonus does QO ,o beyond a definite and useful degree. Without this inhibitory action of the pyramids, tonus and reflexes would not present such constant relations as ually the case. If, however, the pyramids are destroyed, hypertonia and rreflexia take the upper hand to a degree hindering motion, so that the lis cannot be sufficiently relaxed and even the mere jarring of the body in aotion suffices to set up reflex contractions. ifkr this short physiological introduction, it is understandable that in I quence of the vicarious action of the subcortico spinal tracts* the affected extremities arc not entirely paralyzed, but only more or less paretic; this >is, however, affects nearly all the muscles of the limbs in question, to the extent. In the paretic muscles a spastic rigidity is present, as we can v convince ourselves by palpation, by testing resistance and by passive . -merits. On the other hand there is no atrophy of the affected muscles — rt from an atrophy from disuse which occurs late and is never very great — as the muscles remain in spite of the pyramidal affection, in uninterrupted con- ■ < tion with their trophic centers, the cells of the anterior horn. For the same i .mi, along with anatomical integrity of their structure, the muscles preserve their electric irritability. The gait of such patients, in consequence of the paresis and rigidity of the . i< altered, in very characteristic fashion. Since the rigidity is especially in uked in the glutei, the quadriceps, the adductors, and the gastrocnemius, the leg is extended rigidly at the knee and hip, the thigh is held in adduction, the are raised from the floor so that the patient sometimes walks only on the | s of liis feet, and in order to prevent falling backward, must shift his center <>l gravity forward, which forces him to make use of a walking-cane. Locomo- - carried out with short, audibly dragging and careful steps; since the Joints are held by the muscular rigidity in a condition of extensive "muscular ankylosis" (Striimpell) the feet can scarcely be raised from the floor, but must alternately be swung forward in a lateral arc one around the other ("heliko- podia" "circumduction"). Sometimes a "sec-sawing" of the body at every itep is noticeable. In long duration of the disease, the anomaly of position of the leg is more and more fixed, on account of structural shortening of the icrtonic muscles so that a true pes equinovarus can develop. Only excep- tionally are there contractures in flexion at the knee and at the hi]), an oc- currence that puts an end to the patient's ability to walk. In the rare "ascending" cases of spastic spinal paralysis, in which after the •Besides this, usually only the lateral pyramidal tracts, not the anterior pyramidal tracts, fectecl. 114- LECTURE VII legs, the upper extremities are also affected, the arms, on account of t equal distribution of rigidity and paresis, usually take the following po adduction, flexion at the elbow, wrist, and finger joints, pronation. The "spastic symptom complex" is completed by anomalies of reflexes and associated movements. The exaggeration of the tendon and bone reflexes in the region of the paretic muscles (which is, pathogenetically, the analogue of hypertonia) is usually very great. At the same time as this hyperreflexia in the affected musculo-tendinous apparatus, there can be demonstrated (he phenomenon known as "clonus" ("trepidation epileptoide" of the French). For example, if the tendo Achillis is suddenly stretched by pushing up the anterior portion of the foot, rhythmical oscillations of the foot, which last as lo the tension of the tendon is kept up, are produced. This is by far the most frequent clonus — "ankle clonus." "Patellar clonus" cannot infrequently be Fig. 39. The Bablnski Reflex (instantaneous photograph). produced by suddenly drawing down the patella and attempting to hold it in tin's position. On the other hand "wrist," "finger" and "elbow clonus" are rarely demonstrable. The behavior of the skin reflexes is much less constant than that of the tendon reflexes; the plantar reflex is found exaggerated with a certain degree of regularity, but the degree of reflex reply to skin irritation is very difficult to estimate as to its pathological indications. All the more valuable to us are certain abnormal skin reflexes which occur when the pyramidal triers are interrupted. Of the first importance is the Babinski phenomenon, in which irritation of the sole of the foot causes a rather slow, tonic hypercxteiiMon of the great toe (see Fig. 39), sometimes with accompanying plantar flexion 01 fanlike spreading of the other toes. In the second place stands the Opp nlwim reflex, in which the same reaction is produced by firmly stroking the skin on the inner surface of the leg. A pathological tendon reflex is the J BecMerew phenomenon ("dorsal foot reflex") ; in lesions of the pyramidal SPASTIC SPINAL PARALYSIS 115 percussion on the lateral part of the dorsum of the foot (over the 4th and 5th metatarsal bones) may cause plantar. flexion of the toe.s ; normally there is either no reflex or extension of the toes. That the "pathological reflexes" occur normally in children in the early months of life, depends most probably upon the fact that the pyramidal tracts develop their full function only after their axis-cylinders have taken on their medullary sheaths in the course of the early part of the post-embryonal period. A satisfactory pathological explanation of these phenomena is wanting. We can at most conceive that the reflex irritation, if it is not inhibited from above (that is, from the cerebrum) breaks into paths otherwise closed to it. The same may be the case with the voluntary impulse intended to produce a definite movement of the paretic muscles so that it comes to set up unintended "asso- ciated movements." When, for example, the leg cannot be drawn up on the body, without decided dorsal flexion of the foot occurring at the same time, we speak of the tibial phenomenon or "Striimpeirs sign" ; compulsory hyperex- tension of the big toe under the same conditions furnishes the toe phenomenon; in the hand, the radial phenomenon occurs (compulsory dorsal flexion of the hand with palmar flexion of the fingers), further the pronation phenomenon (compulsory pronation when the forearm is bent), etc. Sensory, vasomotor and trophic disturbances, also anomalies in the empty- ing of the bladder and rectum, as well as those of the sexual functions, do not belong to the picture of spastic spinal paralysis. Course (iiiil Prognosis The disease begins, as a rule, gradually and little marked, at first with only subjective, often but temporary difficulties; after long walking the patient feels weakness and sense of tension in the legs, symptoms which can increase to a "spinal intermittent limping" (Dcjcrhu) which, however, for a long time dis- appear again when the patient is rested. Only later do they become permanent and then the objective symptoms of the disease grow more and more plainly apparent. If the clinical picture does not, as is usually the case, unfold itself as the initial stage of some other disease more rich in symptoms, but definitely restricts itself to the spastic symptom-complex, the progression is usually a very slow one, so that the patients may remain capable of movement for ten or twenty years or more, indeed, it may conic to a stop when the disease lias made relatively hut little progress. The prognosis as to life is hence not unfavorable in the true spastic spinal paralyses, which usually commence between the ages of 20 and 40 years; only when the patients (generally on account of con tractures in flexion of the legs) have become bedridden, it is impaired by the danger of hypostatic pneumonia. As to the prognosis of the symptomatic form, no genera] statement can be made. It depends naturally upon the nature of the disease causing the syndrome "spastic spinal paralysis"; fur example, where it depends upon compression of the cord by a tumor which .can lie re- moved by operation, cure is possible. 116 LECTURE VII Treatment In the very first place it is necessary to warn these patients against ex- hausting gymnastic exercises in which many of them are inclined to seek cure, and to make clear to them the necessity of prolonged rest and self-care. The rigidity can very often be alleviated by careful massage and passive movements ; protracted tepid baths work in the same way. The practice of active move- ments is best carried out in the bath, since the influence upon their weight which the limbs experience on account of the buoyant effect of the water (this can be increased by the addition of salt) aids greatly in the work to be accomplished, while on the other hand the diminution of the stimuli from the skin acts to some extent in reducing the tonus. On account of the long duration of the disease drugs should be used but sparingly ; though occasional courses of bromides or scopolamin (for dosage see Parkinson s disease, page 89) can be recommended without hesitation. Faradization is strongly contraindicated ; if stabile galvanization of the rigid muscles or their nerves or the "galvanization of the spinal cord" (two large electrodes, one on the neck and one in the sacral region, gradual introduction of a current of a few milleamperes, let it pass for 5 minutes, slowly reduce it, change the poles and repeat the procedure) is of any use is questionable ; in any case it does no harm. In contractures, ortho- pedic surgery is to be considered (tenotomy, supportive apparatus). The most modern surgical procedure recommended for spastic paresis is "Rhizo- tomia posterior" proposed by Forster. Based upon the idea that the produc- tion of tonus depends upon peripheral stimuli which are transmitted through the posterior roots of the spinal cord to the cells of the anterior horn (see page 8) several pairs of these roots are resected in order to make movements more easy bv getting rid of the rigidity. We will consider this operation again later in describing Littlc^s disease, where it may have a good result (provided it is followed by thorough and conscientious gymnastic after-treatment). In spastic spinal paralysis, on the other hand, it seems to promise less. In the only case with which I am acquainted from my own observation, it remained without effect. As to the causal treatment of the forms dependent upon exogenic factors, we need not consider it further at this point; the therapy of nervous syphilis will find a connected description in a later lecture. Amyotrophic Lateral Sclerosis and Progressive Bulbar Paralysis These names have become attached to two symptom-complexes which do not represent different diseases at all, but only describe a different localization of one and the same affection; in the one case, in the spina], in the other, in the bulbar nerve centers. This affection, however, presents itself anatomically as a primary, progressive degeneration of the whole motor tract due to endogenic factors (though occurring as a hereditary family disease only in a continually decreasing number of cases). Parenchymatous degeneration of the neurones with reactive proliferation of the supportive tissues lies at the base of both diseases. In amyotrophic SPASTIC SPINAL PARALYSIS 117 lateral sclerosis these changes affect the cells of the anterior horn of the spinal cord and their processes, in progressive bulbar paralysis the motor nuclei of the medulla (the homologues of the anterior horn cells). Besides this, the fibers of the pyramidal tracts going to the diseased spinal and bulbar nuclei (cortico-spinal and cortico-bulbar) degenerate throughout their whole extent. Indeed, the motor intersegmental fibers of the antero-lateral tract in the spinal cord and the cortical fibra? propria? in the cerebrum have been found altered. Both disease forms are rather rare, and in the great majority of cases occur between the 30th and the 55th years of life, only very exceptionally in youth or in childhood; amyotrophic lateral sclerosis on an average, earlier than bulbar paralysis, the first beginning mainly in the 4th, the last in the 5th or 6th decades. As exciting causes exposure to cold, exhausting diseases and over- exertion, have been noted here and there. It is often found in amyo- trophic lateral sclerosis that these have affected particularly the hand in which the symptoms of the dis- ease begins. Here and there pro- gressive bulbar paralysis also has occurred in connection with severe fatiguing of the muscles of the lips, cheeks, and tongue (in players of wind instruments and glass blowers). Any special pref- erence of one sex over the other is not apparent. The unity of the two disease forms is indicated by the fact that every amyo- trophic lateral sclerosis sooner or later is combined with progressive bulbar paralysis, provided that the patient is not earlier removed by some intercurrent disease. That this statement is not re- versed, that is. that progressive bulbar paralysis may occur without spinal symptoms, is explainable from the fact that it puts an end to life, and hence to the further extent of the degenerative process, relatively early. Alteration of the Spinal Cord in Amyotrophic Lateral Sclerosis. Weigert-Pal Stain. ' Symptomatology In sketching the symptomatology we will take as an example a typical case which, beginning with spinal symptoms, ends with bulbar involvement, and which has been denominated as Charcot's disease, alter the great neurologist who de- scribed it for the first time in 1865. As to the name amyotrophic lateral sclerosis, proposed by Charcot himself, it characterizes in a happy manner the clinical picture which represents the combination of two syndromes already studied by us. namely, 1, that of spinal 118 LECTURE VII progressive muscular atrophy (the expression of degeneration of the peripheral motor neurone), and 2, that of spastic spinal paralysis (that is, pyramidal tract — or lateral sclerosis, an expression of the degeneration of the central motor neurone). The disease begins in the upper extremities and almost never in both at the same time. The right hand usually shows the first anomaly and in the progress of the disease the symptoms generally predominate for a considerable time on the one side. The patient first notices a rapid tiring and a feeling of weakness, awkwardness and stiffness, often also of tension, particularly in the finer move- ments ; soon objective anomalies appear, before anything else a progressive wasting of the small muscles of the hand. The abductor pollicis brevis usually atrophies first, then little by little the other muscles of the ball of the thumb, the hypothenar muscles and the interossei. The different deformities which we met in the last lecture in spinal muscular atrophy occur here in an analogous manner," namely the "ape-hand," "claw-hand," and "skeleton-hand." On the other hand, the paretic symptoms are much more marked than in the Aran- Duchenne disease, and the paresis is accompanied with such manifest hyper- tonia that in the early stages of the disease there may be active contractures which fix the finger and wrist joints in flexion. Also spastic adduction of the arm, flexion and pronation of the forearm occur. However, the spasticity in the upper extremities diminishes again with the progressive atrophy (this soon affects also the extensor side of the forearm and shoulders). As long as the muscles needed for their production are present, exaggera- tion of the tendon and periosteal reflexes are to be found ; now and then wrist clonus also can be elicited. In the atrophic muscles fibrillary contractions are very manifest. As in progressive spinal muscular atrophy and on account of the same conditions (namely, the gradual progress of the degeneration from fiber to fiber in the individual muscles) reaction of degeneration is found only in places (particularly in the thenar muscles) and only in incomplete develop- ment ; in general, faradic and galvanic irritability are reduced in proportion to the degree of muscular wasting. For a comparatively long time, the lower limbs remain normal except for a marked increase of reflexes. Little by little, however, the patient experiences a weakness and tension in the legs; also the whole spastic symptom-complex, as we have already described it (see page 112), develops. (Only the Babinshi and Oppenheim reflexes are comparatively frequently absent.) Here, in con- tradistinction to the arms, the amyotrophic component is entirely subordinate to the spastic one and sometimes atrophy of the muscles of the leg and foot oc- curs only late in the disease. Trophic, sensory, and sphincter disturbances are absent during the whole of the disease. On the other hand, the last stage of the inexorably progressive affection is a period of grave increase in the symptoms on account of the ad- dition to them of progressive bulbar paralysis (glosso-labio-pharvngeal paraly- sis). The first description of this last we owe to Durhcnne (1860). Its prodromal symptoms are: Slight difficulty in speaking and in the other movements of the tongue, occasional difficulty in swallowing, inability to whistle, etc. Little by little the disturbances of function in the muscles of the tongue SPASTIC SPINAL PARALYSIS 119 and lips, of mastication, of swallowing and of the respiratory muscles become more marked. The speech becomes continually more inarticulate. The pro- nunciation of the Unguals, particularly of r and 1, is first affected; soon after that of the labials ( f, w, p, b). Among the vowels, o and u, whose production is favored by the contraction of the orbicularis oris, are worst articulated. On account of involvement of the muscles of the velum palati there is difficulty in the formation of the gutturals (g and k) ; this also gives the voice a nasal sound, since the nasal cavities can no longer be shut off from the pharynx and act as resonance chambers. Besides this, the speech becomes monotonous on account of weakness of the larynx muscles. The paralysis of the velum palati, as well as that of the other pharynx muscles, causes appreciable disturbances in swal- lowing; food, particularly that of a liquid character, easily gets into the larynx and produces attacks of choking, or it regurgitates through the nose. Chewing, on account of the progressive paralysis of the masseter and temporal muscles, becomes more and more difficult ; the involvement of the internal pterygoid makes itself evident, in inability to draw the jaw laterally in order to grind the food. The taking of nourishment is rendered difficult by still other factors; the tongue can no longer shove the bolus between the teeth and throw it back again, on which account it remains caught between the gums and the cheek ; also, it can- not form a trough to direct the fluids into the gullet, hence the patient when drinking must throw the head backward. The saliva also is imperfectly swal- lowed and flows out of the corners of the mouth. Objective examination shows that the functionally so seriously affected muscles are also attacked by degenerative atrophy, although, corresponding to the condition of the small muscles of the hand, reaction of degeneration is to In found, as a rule, only in a few muscles (lips and tongue) and scarcely ever in complete development. The tongue is flattened, markedly wrinkled, as if its coating of mucous membrane had become too large for it, and is the seat of parked fibrillary contractions. These last are usually very evident in the lips also, while here the atrophy is usually concealed on account of fatty deposit. Tin velum palati hangs down like a thin, sagging curtain. The temporal fossa presents a depression, and occasionally fibrillary contractions are to be ob- served in the masseters. The masseter reflex is usually exaggerated. The palate and pharyngeal re- flexes .-in' almost always diminished or lost. The face of the victim of bulbar mralysis presents a characteristic change. The lower part in contradistinc- tion to the expressive eyes and perfectly movable forehead, is of mask-like im- mobility : the mouth, on account of the paresis of the orbicularis and the con- tracture of its antagonists, is half open, and extended laterally ("rire transversal") or its angles hang down deeply, which gives to the physiognomy a laciiiiiosc expression. The skin over the emaciated muscles of the checks and chin shows exaggerated folding, only very exceptionally is the upper facial region affected. This condition, which the mental clearness renders specially shocking, finally terminates either by vagus paralysis (permanent severe tachycardia, "Mi weakness) or paralysis of the diaphragm. Every bronchitis, besides this, contains in itself the greatest danger to life, since coughing anil expectoration 120 LECTURE VII are impossible. Also, the danger of aspiration of particles of food into the lungs cannot indefinitely be avoided in any of these patients. Course and Prognosis By far most frequently we see Charcot's disease begin in the small muscles of the hand and develop further in the manner sketched above; only very ex- ceptionally are the muscles of the shoulders attacked before those of the hand (scapulo-humeral form). Another rare method of beginning is that the spastic paralysis of the lower limbs precedes the spastic atrophic manifestations in the upper ones. This is also a further proof that spastic spinal paralysis in its later course presents itself as only a partial symptom of another disease process. Finally, we have as a third variety, the cases which begin at once with bulbar symptoms. In only a very small number of these cases an extension of the motor disturbances to the muscles of the neck, the shoulders and the upper extremities occurs ; most of them die too soon. The prognosis both as to life and as to recovery is absolutely unfavorable. Death occurs in from six months to ten years. Relatively the best prognosis is given by the form beginning as paraplegia ; by far the most unfavorable, by primary progressive bulbar paralysis, of which no case lives beyond the fifth year of the disease, only few beyond the second year. Differential Diagnosis In amyotrophic lateral sclerosis, the resemblance at the start to progres- sive spinal muscular atrophy is great ; the hyperreflexia as well as the tendency to contracture, however, enable us to exclude this as well as the other varieties of progressive muscular atrophy; also the progress of the atrophy is much more rapid in Charcot's disease than in that of Aran-Duchennc. We have al- ready alluded to the liability to confusion of the paraplegic form of amyo- trophic lateral sclerosis with spastic spinal paralysis, an error from which reservation of opinion until the disease has developed further, will protect us. Finally, it must be mentioned that an affection of the spinal cord which we will consider in the next lecture, namely, syringomyelia, may begin with atrophy of the small muscles of the hand and spastic phenomena ; however, there are al- ways disturbances of sensibility peculiar to it, which are without exception ab- sent in Charcot's disease. As to the glosso-labio-pharyngeal paralysis, an acquaintance with the clini- cal peculiarities of myasthenia (see page 9-1) will permit distinguishing the two diseases without difficulty. Similar pictures are given also by supranuclear pseudo-bulbar paralysis (see Lecture XV) which occurs through symmetrical interruption of the neurones passing from the cortex to the motor nuclei of the bulb, from foci of disease of arteriosclerotic origin. These occur, however, in connection with repeated minor apoplectiform attacks and there is neither mus- cular atrophy, reaction of degeneration, nor fibrillary contractions, but usually disturbances of intelligence as well as those in the function of the bladder. Further, disease pictures resembling those of bulbar paralysis occur in. SPASTIC SPINAL PARALYSIS 121 neuritis of the motor nerves arising from the medulla (for example, after diph- theria), also from acute inflammatory febrile processes (polioencephalitis in- ferior), from hemorrhages and softening of the medulla (embolism and throm- bosis of the basilar artery) beginning acutely, and finally from compression of the medulla (through tumors, caries of bone, etc.). In these last, however, there is never complete symme'try on the two sides; besides, there is local sensi- tiveness to pain, often headaches and vomiting. Also further symptoms may be found, as, for example, involvement of the sensory portions of the trigeminus, of the acusticus, etc. We must consider here "superior bulbar paralysis" or the ophthalmoplegia chronica progressiva of Graefe, which presents an analogue of progressive glosso-labio-pharyngeal paralysis and may be combined with this. This is char- acterized by a constantly progressive, gradual atrophic paralysis of the differ- ent eye muscles which, so long as it is not combined with glosso-labio-pharyngeal symptoms, naturally does not threaten life. Much more frequently, indeed, progressive ophthalmoplegia is not a definite primary degenerative disease like progressive bulbar paralysis, but a part of different organic diseases of the brain and spinal cord, as, for example, lues cerebrospinalis, tabes dorsalis, dementia paralytica, sclerosis multiplex, etc. There are also neuritic ophthal- moplegias ; further, acute or subacute attacks arising upon an inflammatory basis (polioencephalitis superior) as well as those which are caused by local processes in the brain and at the base of the skull (for example, meningitides, tumors, etc.). Treatment The treatment of amyotrophic lateral sclerosis comprises that of its com- ponents, spina] progressive muscular atrophy and spastic spinal paralysis. In progressive bulbar paralysis, arsenic, iodide of potassium and strychnine are given, although a favorable, or even a palliative effect of these medicaments has not been recognized with certainty. Of electro-therapeutic procedures galvan- ization transversely through the medulla oblongata (electrodes the size of a dollar upon both mastoid processes, two milleamperes current strength, slowly introduced and reduced again), as well as the production of swallowing move- ments, are recommended. (Anode on the back of the neck, cathode moved up and down on the side of the neck.) We can counteract the flow of saliva by atropin, scopolamin, or duboisin in doses of 1 J_. nig. (gr. K2o)- * s long as Bwallowing is appreciably impaired the nourishment should be half liquid and should contain as many calories in as small a volume as possible (nutritive preparations, meat- juice, etc.). In advanced stages tube feeding must be re- sorted to. In order to aid you in obtaining a comprehensive view of the different forms of primary degeneration of the motor tracts as well as of the muscles as we have learned them in the last two lectures, I have prepared tor you a synoptic table showing these affections and their most important differentia] characteristics. 122 LECTURE VII _• , a ion o o o o 03 O + A 03 A 1 g 03 c o o o o o "S 3 Oj + 3 3 £ £ ft M ft « ft _- -d . cS 111 o o © o o O 03 ^ 0^ o3 Fh U tH u t- u 03 «-• -s "3 s 3 0j oj o - o 1 o 1 1 o 1 o 1 O 1 03 O = i o 03 — ' to 1—1 1-1 5^ O + + + T + + + _C__; _>. >. >., j>> ■- k >> "j; "3 • c5 q 03 — ■ 3 b u P "3 03 03 k 1 *- Ph « P « Lh tH c C "S M 53 "3 o o o o to T3 0j "3 "3 o O 0, 33 5 03 o o 3 03 "g Ml 3 .2 "G 0? 3 — IS to is — s IE < < 03 to C tog° O o3 ft -< C 0) -^ ^ rn to T3 03 "3d TS 03 03 ^ '^~ [3 to b/. w a 1^ 03 n' 03 5S 2 03 ^C .2 O _S ^ _03 W Cj- Z £~ 03 ^ .2 03 :t '5 c '5b « 0) is o to — to o .SP ft J o | c x to "3 W S 03 g CO 03 X T3 t^ 1 c* a "3 3 X X 3 0j -U > - 3 3 a 03 03 3 03 3 e .g o o 2 O , - -1 _g '« 03 ■ .* 3 to o .33 3 03 to Q 03 tp 1 o 03 5 < — to 03 J "£> 2 03 S. to to O _2 to hJ 03 eS O O a) 03 3 C 3 to j x - 03 « -m c 03 *&3 "3 03 ft x £ > '3 ^ 2 c .9 c o = 3 03 "3 _Z a> c3 ■g ft « ft s 8 s += » s f < X' ^S.2 O 8*3 — o3 ft : >> 03 a o to "3 >> ft OJ u "C "£ 03 03 > ft 2 to S "I i-s — 03 2~ i 0j _3 S to 3 3 r 3 03 "C ^o3 = Oj "3 Oj -= tt 'E 4J 03 'x O — 03 ^. X 03 'x j>> "3 5 ft 3 "3 O CO 3 O ft "3 g "ft CO o en o3 — X c 03 3 03 4,- X - -C5^ to 2 ft, ~ > s & • »'<£ o ~« £ >> ft 03 03 > ft 3 3 ._ ^> ft 'a* 2 3 ft ^3 O 3 •— 03 < ft l-H O} - J3 ' ft O ^ 03 ^ 3 g M g ft o .2 o g to § to « to J5T ^ 0j ft "el H EC 03 03 _03 3 ft = 03 "3 — c V U oj to _03 o "o >> B < CO pq _> *03 £ bfl i LECTURE VIII The Hereditary Family Ataxias Gentlemen: Since we have in the preceding lectures become acquainted with the dyskinesias, the progressive muscular atrophies, spastic spinal paraly- sis, amyotrophic lateral sclerosis and progressive bulbar paralysis, a series of disease conditions which characterize themselves by preponderant hereditary- family occurrence, we will consider to-day some specially interesting representa- tives of the heredo-degenerative nervous diseases. These are the hereditary family ataxias which since their clinical groundwork was laid by the great Heidelberg clinician Friedreich (1861-1863), have always held medical interest ; of late, however, have gained in actuality in that alongside of the typical clini- cally and anatomically well-founded picture of "Friedreich's disease" certain other varieties have become known. We will first make the acquaintance of the classical type of the disease. I. Friedreich's Disease (Spinal Heredo-ataxia) Sy m ptomat ologi/ The fundamental symptom of this disease, which begins, as a rule, in child- hood, is static and locomotor incoordination which, from a scarcely noticeable Beginning, progressively increases to the highest degrees of intensity, and al- most without exception affects first the legs, only later the arms. The parents of such a child, which usually has learned to walk early and well, but as time progresses begins to complain of weariness in the lower extremities, notice that little by little its gait becomes uncertain, spraddling and jerky. Soon a "wabbling in the loins" is added to this and the gait comes more and more to resemble that of a drunken man. Finally, the excessive degree of this ataxia no longer permits locomotion and the patient becomes permanently bedridden. At the same time a^ the locomotor, static incoordination has also developed: even when sitting quietly, the body sways from side to side, each limb when held free, and also the head, oscillates irregularly. This ataxia is, as we may assume from the description of the pathological anatomy of the disease, the clinical corollary of defeneration in the posterior columns and in the spino-cerebellar tracts of the spinal cord. liotli these systems conduct impulses, which belong in the category of deep sensibility (see page 7), and which have come by way of the peripheral nerves through the posterior roots into the spinal cord, toward the brain. While now one portion of these impulses passing up into the posterior columns and proceeding by way 1 2:5 124 LECTURE VIII of the optic thalamus to the cerebrum exerts a quite general movement regulat- ing action, the second portion which passes by way of the direct cerebellar tract and column of Gowers, to the cerebellum, enters into the special function of maintaining the equilibrium on standing and walking. The incoordination in Frc'ul retch's disease is now a mixed form of these two types of ataxia. The one component, which is to be considered as cerebellar in nature, manifests it- self by severe disturbances of equilibrium which we have just mentioned, through staggering and wabbling on locomotion, through swaying of the body also while sitting quietly. We will have an opportunity to further consider these symp- toms when discussing diseases of the cerebellum. Exceedingly frequent further, are choreiform movements, which, however, are of much less intensity than those in true chorea. Now it is a constant play- ing of the fingers, again an uniest of the muscles of the neck and face. I have occasionally noted it only in the upper lid. Athetoid movements have been described as a rare occurrence. In isolated cases, muscular paralyses have developed in the extremities of patients with Friedreich's disease. It appears to me quite doubtful that these are uncomplicated cases of the disease, since in one such case of my own I was able to recognize anatomically the combination with progressive muscular atrophy, while, on the other hand, neuritic processes have been found. Again, speecli disturbances are among the most regular phenomena in spinal heredo- ataxia ; in advanced cases they are almost never absent. The words are brought out slowly, with difficulty, and often in a slightly scanning and explosive manner (hesitating speech). A frequent changing into falsetto (bitonality) has been pointed out by Dejerine, Thomas and myself. Soca has noted disturbances of articulation in the pronunciation of certain letters, namely 1, k, v and i. Among disturbances of the eye muscles, nystagmus takes the first place on account of its great frequency. In the early stages of the disease, however, it is generally absent. Usually it appears after from 3 to 5 years' duration of the affection, sometimes only later; in but very few cases is it absent entirely. As a rule, it is a dynamic horizontal nystagmus, that is, made up of rhythmical contractions of both eyes which occur in the horizontal plane and are set up by looking to one side. Almost never, on the other hand, do we find a static nystagmus, that is, one present during rest. Paralyses of the eye muscles are exceedingly rare in Friedreich's disease. The tendon and bone reflexes are diminished in the early stages, entirely lost later. The reflexes in the lower extremities disappear first, in the upper extremities only some years later. In almost all cases Babinski's foot phe- nomenon is present in typical form, occasionally a slow hyperextension of the big toe can be produced also by stroking the inner side of the leg (Oppcnheim's reflex). In general the skin reflexes are undisturbed, as well as those of the mucous membranes and of the pupil. Particularly interesting are certain deformities which are typical accom- paniments of Friedreich's disease. A peculiar change in the form of the foot in the great majority of cases early develops ; the dorsum curves upward so that the foot assumes a short, compressed and concave form (pes cavus) ; the toes, particularly the great toe, become permanently held in dorsi-flexion at their THE HEREDITARY FAMILY ATAXIAS 125 metatarsophalangeal joints, the extensor tendons of the great toe give the im- pression of being considerably shortened and stand out on the back of the foot; finally? the foot assumes a more or less pronounced equinus position (see Fig. 41). Along with this typical deformity, there are other less marked varieties of the ''Friedreich's foot." In the production of this deformity the '"balancing" action, the continual contraction on the one hand of the tibialis anticus and posticus muscles, and on the other of the muscles of the calf and sole, due to the ataxia in gait, plays a great part ; besides this — as the dorsal con- traction of the great toe, which may be considered as a permanent Babinski re- flex, indicates — the continuous irritation of the sole on standing and walking contributes to this too. I have seen this hyperextension disappear entirely again after the patient had become bedridden. Besides this, a more or less marked curvature of the verte- bral column occurs in time in most cases. This is mainly a scoliosis, though sometimes a kyphosis, more rarely a lordosis. A satisfactory ex- planation of this symptom has not 3 r et been given. The same remarks apply to a deformity of the hand described by Friedreich as a rarity, which was indicated in one of our cases (permanent hyperextension of the thumb). I will mention also some unusual symptoms of Friedreich's disease. These are mainly certain sensory disturb- ances. Diminution of the sensibility of the skin occurs only in the latest stages, for example, in one case I could trace its first beginning only after the disease had existed for 37 years. It occurs chiefly on the feet. The muscular sense and the vibration sense can be affected early, but in most cases remain long intact. In the patient mentioned above a reduction of stereo-esthesia in the right hand occurred first, with superficial hypesthesia in tlie feet. Among sensory irritative symptoms, Bramwell and I have de- scribed painful cramps of the calves. Many patients complain of attacks of rotary vertigo or even of a permanent state of dizziness of little intensity. Further, the disturbances of vegetative function which have been occasionally Observed in Friedreich's disease should be considered. These are profuse saliva- tion, polyuria, hyperidrosis, oedema, diabetes, dyspnoea, jerky respiration, dis- turbances in swallowing, late beginning of the menses. The sphincters of the bladder anil the rectum practically always functionate correctly, at most, slight degrees of urinary incontinence occur. Impotence is never present. In con- clusion the rare psychical disturbances must still he considered. One patient studied by me was imbecile to a high degree, at the same time vicious, impulsive, and given to coprolalia. Other authors have seen a combination with idiocy. Slight defect in intellectual development occurs somewhat frequently; usually, however, the thoroughly normal intelligence of such patients is in marked con- Fig. 41. Friedreich's Disease. Typical Foot Deformity. 126 LECTURE VIII trast to the weakminded appearance which tlicy make in consequence of their stumbling speech and their often dull expression of countenance. Pathological Anatomy The spinal cord in Fried nidi's disease is thin and small to a degree observed in no other disease; also, since the duration of the disease makes no difference in this, it is evidently not an atrophy but a hypoplasia of the organ. We consider the columnar degenerations which we find in the spinal cord in Friedreich's disease as consequences of an arrest of development ; these regularly affect the posterior columns, the direct cerebellar tracts, and the lateral pyrami- dal tracts, sometimes also Grower's columns and the anterior pyramidal tracts. Fig. 42 shows you the characteristic picture with the Weigcrt-Pal stain for the '■3&*?r v/!!"* Fig. 42. Alteration of the Spinal Cord in Friedreich's Disease. Weigert-Pal Stain. medullary sheath. Only very exceptionally have these typical combined columnar scleroses been seen to develop in a well-formed and in no way hypo- plastic spinal cord (Ed. Mutter). The connection of the ataxia with the de- generation of the posterior columns and the spino-cerebellar tracts we have already brought out. Upon comparing the anatomical and the clinical pictures, however, the fact that in spite of extensive alteration of the posterior columns so little sensory defects are to be found appears paradoxical. It seems as if, in system diseases which affect the youthful organism the nervous conduction paths so organize themselves, that by a roundabout way through the gray sub- stance the loss of the posterior columns is partially compensated. The sub- stratum for nystagmus, choreic unrest and disturbances of speech is anatomi- cally not yet made clear. Hypo- or areflexia appears to correspond to the loss of function of the fibers of the posterior roots. The degeneration of the pyrami- dal tracts usually manifests itself only by Babimki's, though sometimes also by Oppenheim's, reflex. THE HEREDITARY FAMILY ATAXIAS 127 Course and Prognosis The beginning of the disease, in the great majority of cases, falls between the 6th and the loth years of life. An earlier commencement (3 to 4> years) is, however, not exactly rare, neither are cases which present their first symptoms at from 15 to 20 years. Unusual, however, are the "late forms" of the disease in which the initial symptoms appear in the third decade of life. As to the sequence of disease symptoms, they usually begin with ataxia of the legs and hyper-extension of the great toe; next the patellar reflexes disappear. The speech disturbance generally is not long delayed, neither is involvement of the arms, while nystagmus is postponed somewhat longer. Nevertheless, within 5 or 6 years after its beginning, the disease picture is usually present in a typical manner. Further progression occurs then, as a rule, much more slowly, so that the patients can live 3 or 4 decades longer, until some intercurrent disease (usually broncho-pneumonia) causes death. Remissions are rare, more frequent are sudden exacerbations in connection with intercurrent acute in- fections. Etiology and Pathogenesis In spite of the "erratic" cases which not so rarely come under observation, Friedreich's disease is in general characterized by the following criteria, as an essentially heredo-degenerative disease, corresponding to a pathological variety of the species. It attacks, as a rule, several members of the same generation ( homologous heredity) and occurs in these mainly at about the same age (homo- chronous heredity) ; it is not to be referred to external factors during intra- or extra-uterine life (traumata, intoxications, infections); in such factors an ex- citing cause can at most be recognized. The injury has already affected the germ (endogenic basis); and finally from the moment of its beginning it is marked by unceasing progression. That Friedreich's disease not only as a disease of the single individual, but also as that of a line of descendants, manifests a progressive and severe degenerative character, is shown from the confirmed fad that from generation to generation the age at which it begins, becomes lower and lower. Also it can happen that the disease skips several generations to appear again (by atavistic reversion), which is denominated latent heredity. The most convincing example of latent heredity is furnished by a genealogical (iii prepared by h'onrad Fret/, which refers to a large group of cases of Friedreich's disease in a village of tin- Swiss Jura. The author was able, by re- ferring to tie- church records, to recognize the common descent of these patients from an ancestor of the sixteenth century. In Hie descendants of this man, dis- tributed among six collateral lines, the disease, however, only appeared in t lu- ll th or 12th generation, although in the intervening generations consanguine marriages with tin- loss of ancestors and a history of heavy drinking was often Doted. As signs of congenital defect of certain parts of the central nervous system mav he mentioned the frequent accompaniment of Friedreich's disease by the most varied congenita] defective conditions and malformations, for example, by hypospadias, facial asvnnnetrv, "mongoloid" conformation of the face, anoma- 128 LECTURE VIII lies of the central canal of the spinal cord, ectopy of the gray matter of the spinal cord, etc. Or the combination with other heredo-degenerative diseases also occurs ; so, for example, I have described a patient with hereditary ataxia and muscular dystrophy at the same time, and Kollorits has seen the combination of Friedreich's disease and Huntington's chorea. Now how this congenital de- fective condition ("abiotrophy" according to Gowers) leads to the gradual degenerative destruction of certain tracts, there may be different conceptions. Jendrdssik writes: "In many families striking peculiarities occur. Some lose their hair prematurely, in others the pyramidal tracts degenerate." Raymond has spoken of "premature senescence," Edinger of the using up, I of the wear and tear of the congenitally defective systems. As causes for the first appear- ance of a heredo-degenerative disease in a definite family line, certain "germ injuring" or "blastophthoric" factors cannot infrequently be made responsible. In Friedreich's disease it is very often alcoholism in the parents (in many cases there is a spontaneous statement that the affected children were begotten while the father was in a drunken condition!) Further, consanguinity of the parents, advanced age, or great disproportion in their ages. As exciting causes of the disease we find exceedingly frequently the history of having passed through some exhausting disease (variola, typhoid fever, scarlatina, measles, pneumonia, pertussis, meningitis, influenza). Treatment Unfortunately we can in no way influence the disease process in Friedreich's disease. In order to delay its course as much as we can, it is indicated that the patients be placed as soon as possible under the best hygienic conditions and under continued medical care, for example, best in an institution. In every case care should be taken that they get plenty of bodily rest, best lying out in the fresh air, while the amount of daily exercise should be very carefully regulated so that overexertion, which is under all circumstances injurious, is avoided. By careful massage it should be endeavored to strengthen the muscles still capable of function and to effect some correcture of the foot deformity. In order to relieve the ataxia the F 'renkel-Le yden compensation therapy which we will con- sider at length under the discussion of tabes in the next lectures, should be tried in every case; however, I have only once obtained a decided effect which was lasting. From time to time strychnine cures (for adults daily 0.002 to 0.005 (gr. 1/30 to gr. 1/12) subcutaneously, or 4 times a day 5 to 10 drops of tinct. nucis vomica?), are also to be recommended. In order to prevent bed-sores, in bedridden patients there should be the most thorough cleansing and care of the whole back, as well as proper attention to position in bed. II. Cerebellar Heredo Ataxia In the year 1893 Senator called attention to some cases of hereditary ataxia in which, along with the spinal lesion which we have described (see page 125) there was ^also a striking diminution in size of the cerebellum, and in the same year P. Marie attempted to construct from these atypical cases a disease picture to which he gave the name "heredo-ataxie cerebelleuse," and THE HEREDITARY FAMILY ATAXIAS 129 which was to bo separated both clinically and anatomically from Friedreich's disease. According to Marie's description, the marked cases present the picture of a disease which begins after the twentieth year of life with a slowly and pro- gressively increasing unsteadiness in walking and standing; sometimes, too, with pains in the loins or in the lower extremities. After from one to three years the ataxia involves the arms also, while at about the same time dis- turbances of speech and of vision become evident. Optic atrophy and narrow- ing of the visual field, often also a loss of the pupillary reaction to light, are found. The patellar reflexes are exaggerated, or at least of normal activity. Often there are also other spastic phenomena; for example, ankle clonus. Fre- quently a certain mental weakness is observed. Nystagmus and disturbance of speech develop as in spinal hereditary ataxia. On the other hand, the deformities of foot and spine so characteristic of the latter are absent. Quite often there are disturbances of sensibility, less frequently those of swallowing and of the bladder; sometimes there is hypertonia of the muscles, again chorei- form movements. This presents a symptom complex differing in many impor- tant points from Friedreich's disease, which seems to justify definite separa- tion from affections resembling it in the disturbances of coordination and occurrence in families ; but it has been shown that the boundary line cannot be sharply drawn either from an anatomical or from a clinical point of view. As to the post-mortem findings, it suffices to point out that Thomas and Roux in one case which corresponded particularly well with the above description of Marie, have reported a negative finding as regards the cerebellum, with hypo- plasia and systematic degeneration of the spinal cord, and that I found in a patient who presented clinically not a single one of the symptoms character- istic for Marie's type, the most profound alterations of the cerebellum which have ever been observed in the hereditarily ataxic. The weight of the cere- bellum was reduced from the normal figure of 145.2 grms. to 43.2 grms. (see Figs. 43 and 44). From a clinical point of view, we know, too, that Marie's type of hereditary ataxia can occur just as frequently in childhood as that of Friedreich in adult age; that in it there may be intact sensibility and an intact Optic nerve; that scoliosis and pes cavus with dorsal contracture of the great toe occur now and then, that loss of the pupillary light reflex is usually absent, etc. Exaggeration of the reflexes has best preserved itself as a clinical cri- terion; hut with observing that the patellar reflex, while abnormally lively at the start, can he lost during the course of the affection, this last distinction must tail. Also hereditarily ataxic brothers and sisters who, while presenting an otherwise identical picture, show a different condition of I he tendon re- flexes, are not at all rare. Ileiiee, we can recognize cerebellar-Iieredo-at a \ ia only as a relatively characteristic and frequent variety of the true Frii dreieli's disease, and at the bedside must refrain from making a diagnosis as to the ana- tomical distribution of the lesion with too great positiveness (especially as to the involvement or not of the cerebellum), Indeed, I have been able to recognize experimentally that in the dog the same ataxic symptom < iples can be produced, according to choice, either by a lesion of the cerebellar tracts and spinal cord or by destruction of their endings in lh<' vermis cerebelh. 130 LECTURE VIII Fig. 43. Atrophic Cerebellum of a Patient Aged Forty Years, with Hereditary Ataxia. Fig. 44. Normal Cerebellum of a Man Aged Forty Years. THE HEREDITARY FAMILY ATAXIAS 131 This discovery bridges over physiologically also the clinically as well as ana- tomically already obliterated boundaries between the Frcidrcich and the Mark- types. As regards etiology and therapy, there is nothing to add to what has already been stated in connection with the discussion of Friedreich's disease. III. Infantile Progressive Hypertrophic Neuritis In the year 1890 Dejerine described a "special form of Friedreich's disease with muscular atrophy and disturbances of sensibility" which since then has been recognized as a clinically and anatomically quite special disease. The affection begins in childhood, occurs almost always in children of the same family, and has the following symptoms: Marked ataxia of all four limbs; pes equinovarus excavatus ; kyphoscoliosis ; nystagmus — so far criteria of Friedreich's disease. To these are added, however, loss of the pupillary light reflex, psychical abnormalities, marked disturbances of sensibility, and marked muscular atrophy in the peripheral parts of the extremities, neuralgiform pains. Further, a very great hypertrophy of the peripheral nerve trunks, recognizable on palpation, is found in these patients. This depends, as post mortems have shown, upon a very great increase of their fine and coarse connective tissue, which proliferated in great circles, surrounds the partly preserved, partly degenerated, nerve fibers. The nerve trunks, on account of this, are thickened to double their normal size, which particularly gives to the cauda equina a characteristic appearance. In the spinal cord there is a systematic affection of the posterior columns. LECTURE IX Multiple Sclerosis Gentlemen : The disease with which we must now occupy ourselves and which was first recognized as a disease picture sm generis and studied by Vulpian and Charcot (in the sixties of the last century) must, since of late our attention has been directed to its rudimentary and atypical forms, be considered as a comparatively frequent nervous disease. I see many of these cases among a country population and this experience agrees with that of other neurologists. Multiple or disseminated sclerosis of the brain and spinal cord, the "sclerose en plaques" of Charcot, takes, in the circle of nervous diseases, a special posi- tion, in consequence of a remarkable antithesis which exists between its clinical and its pathologico-anatomical habitus. Namely, while from autopsy to autopsy the topographical distribution of the disease foci varies so much that the preparations from the brain and spinal cord in two cases never represent the same picture, clinically a number of types can easily be separated in which the individual cases may present a remarkable similarity. Nevertheless, while no region of the cerebro-spinal apparatus is avoided by the lesions of multiple sclerosis, on comparing the different sections it is clear that the sclerotic foci plainly show a predilection for certain regions; in the pons they affect chiefly the basal regions ; in the cerebrum, the walls of the ventricles and the corpus callosum; in the cerebellum, the medullary substance; in the spinal cord their preference for the white matter is so evident that ltibbert in his time affirmed that, the borders of the gray matter set an im- passable limit to the extension of the sclerotic foci — a view which to-day is indeed disproven. Pathological Anatomy Fig. 45 shows you with how little reference to the structural arrangements of the nervous elements the sclerotic foci are bounded. Macroscopieally these plaques, whose number in the brain and spinal cord can vary from a few to many hundred, present a reddish or yellowish gray appearance; on palpation they give a feeling of increased resistance. Microscopically the sclerotic tissue presents itself as a thick felt-work of proliferated glia through which many nerve fibers still pass. These last have, indeed, mostly lost their medullary sheaths, but the naked axis cylinders are preserved ; indeed, a new formation of axis cylinders appears to have occurred. This persistence of the conduct- ing elements makes the absence of secondary ascending or descending degen- erations in multiple sclerosis explainable; it speaks for the fact that the glia 132 MULTIPLE SCLEROSIS 133 proliferation is the primary, while the destruction of the medullary sheath is usually a secondary phenomenon (to which, indeed, the phagocytic action of the glia cells contributes). Also the ganglion cells in the foci are preserved; on the other hand, the blood vessels are usually markedly affected ; they are thick- ened, infiltrated, and the lymph channels of the adventitia are filled with granular cells. Etiology From an etiological point of view, multiple sclerosis presents much that is puzzling. The most widespread opinion, that firmly held by P. Marie, attributes to it an infectious basis. Previous typhoid, variola, measles, scar- latina, have frequently been brought into causal connection with it. The occurrence of great epidemics of influenza naturally must also be considered among possible etiological factors. Less frequently puerperal infec- tions, diphtheria, erysipelas, chol- era, dysentery, and malaria have been held responsible. Also pre- vious syphilis we find occasionally noted; indeed, one of the cases reported by Thomas and Long is remarkable on account of the ana- tomically recognized combination of luetic meningo-myelitis and true multiple sclerosis. In the second place, poison- ings by metals have been brought forward as causal factors, chiefly by Oppcnhcim. In these cases tin active agent has usually been lead, but some cases have also been attributed to intoxication by mercury, zinc and manganese. Among non-metallic poisons, carbonic oxide must be mentioned, since in a number of observations there has been a history of poisoning by emanations from coal. Finally, in a few publications the disease lias been referred to a trauma or to a violent emotional disturbance. Where so varied a list of factors has been brought into etiological relation with one and the same disease, we are quite justified in seeing in these factors rather "provoking agents" than direct causes, and in looking deeper for I lie actual causes. This is also the view of Strumpell, who, among 24 cases ob- Krved in Erlangen, was never able to prove the connection with previous infections or intoxications. Apparently multiple sclerosis is not an exogenic, bui in endogenic affection dependent upon the congenital makeup of the nervous system, like syringomyelia to be described later, since a combination of the two diseases occurs. This agrees with the experience thai multiple sclerosis, as a rule, is a disease of early life; its beginning is usually at the Fig. 15. Sclerosis. Section through the Medulla, Weigert-Pal Stain. 134 LECTURE IX end of the second or the beginning of the third decade. In children, indeed, the disease is quite rare ; here one cannot be too cautious about the diagnosis if one does not wish to incur the danger of being contradicted by the autopsy or by the further course of the disease. The diagnosis "infantile multiple sclerosis," however, was confirmed, for example, in one case of Eiclihorst, which was also remarkable in that the mother of the child died of the same disease; however, hereditary occurrence of the disease is extremely rare. Also, the histo-pathological picture seems to me to speak decidedly against an inflam- matory basis for the disease as assumed by different authors; much rather, it appears to me as the correlate of a congenital tendency to perverse growths somewhat resembling tumor formations. The Russian neurologist Bossolymo goes so far as to consider multiple sclerosis, gliosis and glioma of the central nervous system as three stages of intensity of the same process. Symptomatology When now we come to sketch the clinical s3 - mptoms we will confine ourselves at first to the typical, fully developed cases, upon which Charcot has based his classical description. In the foreground of the picture stand disturbances of motility, above everything, tremor. This presents such sharp criteria in multiple sclerosis that in spite of the appearance of tremor in all possible nervous diseases it can be considered the most characteristic symptom of this disease. This is the so-called "intention tremor" which is absent during rest and only shows itself on voluntary movements (much more rarely on automatic, reflex, or associated movements). It disappears during sleep; under the influence of emotional disturbances and exertion it increases and its oscillations are proportional in their range to the extent of the movements carried out ; the further the in- tended movement goes, the more rapid the oscillations become, but in spite of this they preserve a certain rhythm. In a classical experiment the patient is solicited to carry a glass of water to his mouth. The moment the hand begins to move the oscillations com- mence ; they increase in extent and violence the nearer the glass gets to the lips ; it strikes clinking against the teeth, the contents are spilled, and the tremor ceases only after the glass has been taken away from the patient. If the hand of the patient is laid upon his bed and he is made to bend one single finger, this isolated intended movement suffices to set the whole hand, even the entire arm, into violent oscillation. The tremor of multiple sclerosis is a massive one, affecting the extremities as a whole, and increases in intensity toward their roots. In like manner intention tremor can be provoked in the lower extremi- ties; the head and neck also present it; as long as the patient keeps his head on the pillow they are quiet, but upon raising it up or turning it sideways the most violent nodding and rotating oscillations commence. The handwriting, even in the early stages of the disease, assumes a characteristic appearance (as in Fig. 46). Not only the impulse necessary to carry out a movement, but also that needed to keep a certain position, sets up the tremor. So the body of the MULTIPLE SCLEROSIS 135 patient, as soon as he attempts to hold it upright, is shaken violently up and down, or forward and backward. In the arms, as in the legs, the tremor may be first unilateral, or at least predominating upon one side, only later to spread to the opposite side. As to the material basis of this symptom, there are various hypotheses which can be as little proved as disproved. Charcot considered it the expres- sion of the leaping over of the stimulus from one neuron to another ( on account of the loss of insulation by destruction of the medullary sheath). Later authors explain the intention tremor as a focal symptom produced by sclerotic plaques in the pons, in the corpora quadrigemina, in the thalamus, and finally in the cerebellum. Less characteristic is the disturbance of gait in patients with disseminated sclerosis. Its study is considerably interfered with by the tremor which is at the same time set up on walk- ing. In general, however, three sorts of gait abnormalities can be distinguished: the spastic, the spastic cerebellar, and the purely cerebellar gait. In the first, with the beginning of locomotion there is tonic rigid- ity of both lower limbs ; they arc extended at all joints, and besides this held in adduction. The pa- tient balances himself on the tips of his toes, as we have described I- IG - 4) The Hemiparetic Form. — In this form spastic paresis and loss of the abdominal reflexes are present only on one side; with these intention tremor may DCCUr on one side only. Here and there disturbances of sensibility are found upon the opposite .side in such cases, also the Browrb-Sequard symptom-complex of half-sided spina! cord lesions. In conditions of this kind a positive diagnosis, indeed, can scarcely lie made before the autopsy, but with very slow and inter- mittent progress of thai symptom one should not forget to think of multiple Sclerosis. In one case of Oppcnhcim this diagnosis could be confirmed. (i ) Paraplegic Form. — In contrast to the two already related, this atyp- ical form is extremely frequently observed. In the initial stages the picture of spastic spina] paralysis may lie present; in characteristic form, however, ii -mi 1 1 \ loss of the abdominal skin reflexes, changes in the optic nerve, or slight intention tremor in the upper extremities may direct attention in the right direction. Slight disturbances of sensibility and of the bladder function occur in an appreciable number of these rases. In the further course very severe spaslir conditions may develop: either only the lees, or all four extremities Income eont raet ured little bv little, the arms in flexion, tin' legs usually in extension, but often likewise in flexion. Th<- tendon reflexes are exaggerated in He highest degree, and on testine- them marked clonus is produced. Later ' Willi Hie exceptions mentioned in note "" page 135. 140 LECTURE IX permanent organic contractions of the tendons and muscles can occur (I once saw the knees gradually drawn up to the chin on this account), when the reflexes can no longer be obtained. (cl) The Amyotrophic Form. — I have already cursorily mentioned this, and indicated that it can imitate amyotrophic lateral sclerosis. There is atrophic paralysis of the muscles of the trunk and limbs, usually with complete reaction of degeneration and permanent contractures in the paralyzed region, great increase of the reflexes and bulbar symptoms. Differential Diagnosis The special peculiarity of multiple sclerosis, in beginning often with rudi- mentary and atypical symptoms not infrequently leads to its confusion with other nervous diseases. In the early stages, when the skin reflexes are normal and there is yet no spasticity of definitely organic character (that is, no Babinski or Oppcnhcim reflex or ankle clonus, etc.), a wrong diagnosis of hysteria is sometimes made even by experienced observers, a mistake which is all the more frequent since the combination of both diseases is, as I have already remarked, not at all infrequent. In such cases it is well to be very cautious and to put off making a diagnosis and prognosis until the further course of the disease has been observed. In those rudimentary cases where tremor is the only symptom (they are exceedingly rare) a consideration of the typical character of this tremor will make a confusion with chorea or paral- ysis agitans impossible. A differential diagnosis between this and mercurial tremor may be more difficult; this is often an intention tremor, which, how- ever, does not cease entirely during rest. In the course, or as a consequence, of infectious diseases, disseminated myeloencephalitis, whose symptomatology presents a great similarity to that of multiple sclerosis, may occur. This runs a course with elevation of temperature, and is usually characterized by some focal symptoms which point to large inflammatory foci in the brain axis ; for example, an alternating hemiplegia with paralysis of the face on one side and of the extremities on the other. Acute onset does not suffice to exclude mul- tiple sclerosis ; this also, as we will soon see, may commence acutely. In many cases only the further course will clear up the diagnosis. Cerebro-spinal syph- ilis can also simulate multiple sclerosis. Lumbar puncture is very valuable here when syphilitic infection has not been definitely shown. According to Long and others, in multiple sclerosis there is no lymphocytosis, or only a minimal one is found. In general, also, the Wassermann complement-fixation reaction is a good differential guide. However, I once saw a positive result from this in a case which proved on autopsy to be certainly multiple sclerosis, although there was neither a history nor any objective signs signifying the assumption of previous syphilis. (It was in a virgin.) In the picture of progressive paral- ysis, also, spastic paresis can figure, and this also shows tremor, apoplecti- form attacks, and speech disturbances. Here, however, the tremor is rapid and fine and affects chiefly the ends of the extremities ; the speech, too, is not scanning and chopped off, but hesitating and drawling. Finally, also, the MULTIPLE SCLEROSIS 141 psychical disturbances are of quite definite character. It is to be noted that in a number of cases the combination of progressive paralysis with multiple sclerosis has been observed (for example, by Schultz). Those rare cases of disseminated sclerosis which begin in an apoplectiform manner can present difficulty, since post hemiplegic tremor after apoplexy can exceptionally have a typical intentional character. In such cases one must often await further developments before coming to a conclusion. Nevertheless, in suddenly or gradually occurring hemiplegias in young persons in whom alcoholism, brain tumor, syphilis, heart disease and hysteria can be excluded, the possibility of multiple sclerosis should not be lost sight of. Many points in common are shown by the symptomatology of multiple sclerosis on the one hand, and of the hereditary ataxias on the other, namely, nystagmus, scanning speech and cerebellar atactic phenomena. However, there can be no confusion with the spinal variety of Friedreich's disease, since it is characterized by loss of tendon reflexes, by scoliosis, and by the foot deformity ; besides the tremor combined with ataxia peculiar to it has entirely a different character from that of multiple sclerosis (see the previous lecture). Rather is there a possibility of confusing multiple sclerosis with Marie's cerebellar heredo-ataxia, since in this the reflexes are exaggerated, scoliosis and foot deformities are absent, and the tremor may have a definitely intentional character; further, changes in the optic nerve occur. Here family character would speak for heredo-ataxia; besides, in this last there is usually beginning with purely cerebellar symptoms. Brain tumors sometimes produce clinical pictures similar to multiple sclerosis, as Hruiis ami Xonne have shown. The differential diagnosis can be greatly involved, when on account of complicating hydrops ventriculorum the multiple sclerosis is accompanied by symptoms of brain pressure (continued headache, oedema of the papilla, etc.). In general, however, these last are not so in- tense, and particularly not so progressive, as in intracranial tumors. Besides, these are exceptionally rare cases. Again, the differentiation between tumor of the spinal cord and multiple sclerosis is sometimes not easy. I intend to consider this practically important point in a later lecture when describing tumors of the spinal cord. Finally, Westphal's "pseudo-sclerosis," a rare ami not yet sufficiently explained disease, in which, anatomically, the findings have been negative or only a very inconsiderable degree of diffuse glia proliferation, must lie mentioned. The clinical picture has great analogies with that of multiple sclerosis; still, on the one hand nystagmus and optic nerve changes are regularly wanting, and on the other, in pseudo-sclerosis, along with the nearly constant occurrence of epileptiform attacks, there are other symptoms ffhich do not belong to true sclerosis; namely, deep apathy and dementia, delirium, outbreaks of violence and a peculiar slowing of the movements of the lace and eye muscles. Here, also, upon a proper estimation of the symp- toms, mistakes can be avoided all the more, since pseudo-sclerosis is a disease of childhood, while multiple sclerosis, as has already been indicated, almost never occurs in children. 142 LECTURE IX Course and Prognosis We can distinguish three methods of beginning of multiple sclerosis. It can begin insidiously, and this is most frequent, and usually then announces itself by "outpost symptoms," as Oppenheim's appropriate expression has it, for example, by disturbances of the eye muscles with double vision or by changes in the optic nerve (often wrongly considered as retro-bulbar neuritis), or by bladder troubles; to these are usually added spastic symptoms in the legs, which little by little increase in intensity ; less frequent is a rapid (acute or sub- acute) beginning, usually introduced by severe vertigo, still more rarely sudden brutal onset, with an apoplectiform or epileptiform attack. The disease itself runs a more or less chronic course, either progressively or in exacerbations. In the first case the disease usually leads to death in from 5 to 20 years. Proximal causes of death are: intercurrent diseases or the occurrence of bulbar disturbances, cachexia with bedsores, or an apoplectic attack. There are cases described as acute multiple sclerosis, which in from 3 months to 2 years de- velop rapidly the most severe symptoms and end fatally. Probably these, or at least part of them, are instances of another disease, namely the post infectious disseminated myeloencephalitis mentioned in the discussion of differential diag- nosis. When remissions occur they can last for years ; in one of my cases affecting a 50-year-old lady, the history mentioned that at the beginning of her third decade of life she had suffered from a temporary spastic paraplegia with tremor; a nearly thirty-year remission must hence be assumed in this case. The possibility of recovery, or at least of a definite standstill, of the disease is affirmed by a few authors, but these reports must unfortunately be received with great reserve, as the above case with a recurrence after 27 years shows. Important from the point of view of prophylaxis is the condition that new exacerbations are often started up by overexertion, getting chilled, infectious diseases, the puerperium, etc. Treatment After entering upon hospital treatment considerable improvement (which is mainly to be attributed to the physical and psychical rest which the patient gets) is often observed. Also in patients cared for at home, the chief weight is to be laid upon the keeping away of causes for overexertion and emotions. In this connection it is to be remembered that these patients often have a strong libido, whose gratification or even excitation is to be suppressed as far as possible. This point must be considered also in the choice of nurses ; an endeavor must be made to strengthen and build up the patient. Warm baths are just as injurious as cold water treatment. The same remarks apply to strong electric applications. Of all drugs, only arsenic seems to have any active effect in the way of favoring remissions. I give it either in the form of Folder's solution or as the Asiatic pill (raising it as rapidly as possible to considerable doses, 7 drops of Fowler's solution or 0.005 (gr. %s) of acid, arsenios, t.i.d.), eventually also in combination with quinine or iron, or, however, if there is no effect after 3 weeks from this medication, subcutaneously as sodium cacodylate, 1 ampoule containing 0.05 (gr. %), later 0.1 to 0.15 (gr. 1^4 to gr. 2). LECTURE X A. Spinal Gliosis and Syringomyelia The two disease conditions of which we will now give a common description cannot be separated from one another either symptomatologically or upon a basis of their pathologico-anatomical substratum, and their pathogenesis; since spinal gliosis not only is a condition regularly antecedent to the occurrence of the cavity formation which we call "syringomyelia," but both conditions are usually found alongside of one another in the spinal cord of one and the same patient. Pathological Anatomy A typical syringomyelic spinal cord can macroscopically closely resemble a tube, justifying the characteristic appellation introduced by Ollivier cV Angers. The former view that it was the product of an ectasy and a hydrops of the centra] canal must be given up, since the entire independence of the cavity formation from the spinal canal can often be made out. From other cases there is evidence that the confluence of the ependymal canal and the syringomyelic cavity have occurred secondarily only. This last usually begins at the pos- terior gray commissure and extends more or less irregularly in the cross section into the gray substance of the posterior and anterior horns. In a longitudinal direction it extends from the cervical region (at which level the cavity formation usually begins) in the majority of cases, getting smaller continually, into the middle or lower thoracic region. There are, however, instances in which if has extended deep into the lumbar region, indeed, into the conus terminalis. On tin- other hand the process can ascend until the cavity opens into the 4th ven- tricle of the brain axis and involves an extensive part of the medulla, when "syringo-bulbia" can properly be spoken of. Microscopical examination now furnishes information as to why these cavi- ties occur. Their walls consist mainly of a thick zone of firm, greatly pro- liferated, neuroglia rich in cells"-*— that is of the same tissue as the neoplasms which develop from the supporting substance of the nerve centers, the gliomata. While glioma, however, spreads from its point of origin diffusely in every di- fection without its growth following any definite path determined by anatomical relations so far as can be recognized, the process upon a basis of which syringo- myelia occurs "central gliosis of the spinal cord"- is characterized by more or less cylindrical or prismatic outline, by excessively slow growth and tendency to quiescence for long periods, and by exceedingly sharp separation from the healthy tissues. Peculiar to it, further, is its tendency to break down and to 148 144 LECTURE X form cavities ; still, cases occur in which the last are absent or only rudimentary, and in which disease-pictures, not to be separated clinically from syringomyelia, depend upon a substitution of solid masses of glia for central portions of the spinal cord. Above and below, too, a portion of solid gliosis is often joined to the glia tube of syringomyelia. Hence, when we in the following, for the sake of simplicity, speak always of syringomyelia, it must be understood in advance that our remarks apply throughout to both conditions which can be separated neither clinically nor anatomically. The syringomyelic cavities have a lining of ciliated cylindrical cells only in front where they have become united with the central canal, some of the ependy- Fig. 47. Central Gliosis Passing Over Into Syringomyelia. Weigert-Pal Stain. mal lining of which has remained. Finally, where the gliomatous masses have overstepped the boundaries of the gray substance into the white columns, as- cending or descending degeneration of the long fiber tracts, for example, of the posterior columns or of the pyramidal tracts results. Etiology Although a varied line of exciting causes have been held responsible for the outbreak of syringomyelia — that is, for its first clinically determined symptoms — there can be no doubt that it is at the base a congenital process, namely that the affected individual, on account of a congenitally defective structure of the spinal cord, is condemned to defective growth and degeneration of the central glia leading to disturbances, first unnoticeable, but gradually becoming more marked. The disease affects men about three times as frequently as women ; it shows peculiar variations in its geographical distribution; for example, while quite frequent in Vienna, in our neighborhood (northwest Switzerland, southern Baden and upper Alsace) it is excessively rare. SPINAL GLIOSIS AND SYRINGOMYELIA 145 Symptomatology The most characteristic symptom of syringomyelia is the so-called "disso- ciated anesthesia," that is, loss of pain and temperature sense with intact touch sense and deep sensibility. This peculiar disturbance, first thoroughly studied by K abler and Schultze, has been denominated the "posterior horn type of sensory anomaly." As to its anatomico-physiological basis we have quite accu- rate knowledge. Certain definite short fibers from the posterior roots end in the posterior horn of the spinal cord on the same side. From this point the impulses transmitted centripetally by these are conducted farther through the gray sub- stance to the lateral columns of the opposite side and through them to the optic thalamus and the cerebrum (by way of the "tractus spino-thalamicus"). The senses of pain and temperature are conducted exclusively by these paths, hence in destruction of the posterior horns or of the central gray matter (through which the "tractus spino-thalamicus" passes) the perception of these sensations is lost, while to touch sense and deep sensibility, still other paths re- main open (the posterior columns, etc., see Fig. 50). In typical cases of syringomyelia corresponding to the predominant distri- bution of the pathologico-anatomical process in the upper segments of the spinal cord, the dissociated loss of sensation is found in the upper limbs and on the chest and trunk. The patients themselves often become aware of these anomalies, since they are struck by the absolute painlessness of accidental burns or cuts and the absence of feeling of cold in winter. It is remarkable also that in taking the history of individuals in whom syringomyelia has only been diag- nosed in the third or fourth decade of life through the onset of other symptoms, we sometimes meet with the statement that even as children they had been re- markable through experiencing no pain from wounds and burns. If the dissociated loss of sensation presents nothing alarming to the pa- tient or his surrounders, on which account the physician is generally not called into counsel, the two other so-called "cardinal symptoms" of syringomyelia present the more striking and disturbing anomalies; namely, the progressive muscular wasting usually limited to the upper extremities and the shoulders and the manifold tropho-vasomotor disturbances equally affecting by preference these parts. The muscular atrophy is usually for a long time unilateral or, at least, much more marked on one side; only in the later course of the disease does it become more or less symmetrical. It begins almost always in the small muscles of the band, especially in those of the first intermetacarpal space and of the thenar and hypothenar regions. By degrees deformities which we have fully described in Lecture VI under Spina] Muscular Atrophy, of the type Artin-Diicliiiinf. occur. The "leaping over" of the atrophy from the hand to the shoulder muscles OC- eui's less frequently in syringomyelia than in tin- Aran-Duch-ervnedisca.se; some- what more frequently regular ascent, in which first the forearm, and then the arm muscles are attacked, is observed. In the forearm it is remarkable that the extensor muscles long remain intact; the preponderance that they gain over the atrophied muscles of the median and ulnar distribution sometimes results in a permanent contracture of the hand in a position of hyperextension which it 146 LECTURE X has been attempted to characterize, not very happily, as the '"preacher's hand." Much less frequently we meet syringomyelic muscular atrophy in the muscles of the trunk and leg; these have a somewhat variable manner of appearance and can give rise to different anomalies of position (for example, pes equinovarus. pes calcaneus, clawfoot, etc.). In the neck the sternocleidomastoid, the scaleni and the trapezii are affected by the muscular wasting. The affected muscles almost always atrophy, not as a whole, but after the "fascicular" type, that is, one bundle after another. The proximity of degenerating and intact muscular fibers brings it about that, like in spinal progressive muscular atrophy, re- action of degeneration is often very difficult to recognize, and then is only partial. Fibrillary contractions are usually plainly manifest; they are found most easily in the deltoid. The vasomotor trophic anomalies affect, above everything else, the skin and its derivatives — hair, nails, etc. A firm oedema of the back of the hand, cold- ness and livid coloration cf the integuments (Marhwaco's '"succulent hand") is frequent. A similar thing is sometimes observed in the foot. Further, ab- normal dryness, scaling, and fissuring of the skin, tendency to herpetic erup- tions, separation of the epidermis and superficial ulcerations are observed. In the origin of these last phenomena, a considerable role should be attributed to the fact that the patients injure their analgesic and thermo-ancsthetic hands by striking them against things, rubbing their wounds, handling hot or very cold objects. There is also a more or less extended atrophy of the skin with loss of its normal superficial outline, the so-called "glossy skin." I would men- tion further hyperkeratosis of the palms and soles, brittleness or falling out of the finger nails, loss of the skin hairs. The trophic disturbances affecting the subcutaneous connective tissue and the bones of the phalanges are particularly severe. Their points of origin are usually areas of suppuration which, starting in the fissures of the cracked skin, whose circulation also is much impaired, do not tend to heal normally, but continually to grow deeper. Their entirely pain- less course justifies the expression "panaris analgesique" used by Morvan for such cases. This can go on to severe mutilations as in leprosy, to the loss of the ends of, or of whole, fingers. Finally, in syringomyelia "'osteoarthropathies" in the extremities, also in the vertebral column, occur, and indeed, quite frequently (according to Schlesinger in 30 per cent, of the cases). These affect, usually, the shoulder or the elbow. Atrophic and hypertrophic changes in the head of the bone, erosion of the socket, loosening of the ligaments, great intracapsular effusions, etc., are found. We leave now the so-called "cardinal symptoms'' to turn our attention to the less frequent manifestations of syringomyelia. Most important among these last are the oculo-pupillary symptoms which have been united under the name of Horner's symptom-complex. The relative frequency of this syndrome depends upon the fact that gliosis and syringomyelia have a strong predilection for the level of the lower cervical cord. At the level of the 8th cervical seg- ment in the gray substance, however, is located the so-called "cilio-spinal center" from which fibers proceed by way of the anterior roots of the 8th cervical and two upper dorsal nerves into the lower ganglion of the cervical sympathetic, from which by another set of neurones there is further conduction to the superior SPINAL GLIOSIS AND SYRINGOMYELIA 147 tarsal, the orbital and the dilator pupillae muscles. The first mentioned is made up of the involuntary muscular fibers of the levator palpebrae; the second bridges over the lower portion of the orbital fissure and so prevents the contents of the oibit from sinking downward and backward; the third is the antagonist of the sphincter pupillae, innervated from the motor oculi. The cutting off of influ- ences from the cilio-spinal center is manifested, 1, by a paralytic mvosis in which the pupil narrowed on account of paralysis of the dilator fibers, does not enlarge when the eye is shaded; 2, by a narrowing of the palpebral fissure, the so-called "sympathetic ptosis"; 3, by a sinking of the eyeball back into the orbit, the so-called "enophthalmus.'' This last component of Horner's symp- tom-complex is, indeed, not always definitely to be found. Obviously this symptom-complex can also occur in lesions of the cervical sympathetic, of the anterior roots of the lowest cervical and two upper thoracic nerves and their rami comniunieantes ; we have met it as an accompaniment of the "lower armplexus paralysis" ("Klumpke's paralysis") already in Lecture I. (See also Lecture XXV.) Further, spastic symptoms which owe their origin to the involvement of the lateral columns by the pathological process, are not at all infrequent, par- ticularly in the lower extremities, as in the arms, atrophy goes hand in hand with loss of reflexes; exaggeration of the patellar and Achilles reflexes are found ; often, also, Babinski's phenomenon and ankle clonus; the hypertonia of the muscles only seldom reaches such a degree that the characteristic gait of spastic spinal paralysis occurs. Both the oculo-pupillary and the spastic phenomena usually are limited to one side. Of the atypical forms of syringomyelia we will give a short clinical outline of "syringobulbia" already mentioned; in this, there are found (often only on une side) dissociated sensory paralysis in the distribution of the trigeminus, atrophy of the tongue or masticatory muscles, recurrens paralysis, formation of ulcers on the posterior wall of the pharynx, paralysis of the eye muscl s, ageusia, etc. Differential Diagnosis The- differentiation of syringomyelia from progressive spinal muscular atrophy, since as far as the muscle symptoms go, they can greatly resemble one another, depends upon the demonstration of the typical dissociation of sensation. Also the trophic disturbances found eventually in the AranrDuchenne disi .use are of much les> intensity than those df syringomyelia. In spastic s. i mgomvehi the determination--^! I lie condition of sensibility likewise furnishes tin- means of deciding between it and amyotrophic lateral sclerosis. The differ- ential diagnosis between syringomyelia and lepra anesthetics can sunn limes be m tv difficult, a question which in our neighborhood, fortunately, scarcely ever conns up. I It does in the United States. Translator.] Decisive as In lepra is the recognition of nodular thickenings of the peripheral nerve trunks, par ticularly, however, the finding of the Armauer Hansen bacillus. Trophic disturbances aboul the face occur frequently in lepra, very rarely in syringomyelia; for lepra speaks further the finding of skin areas with in- 148 LECTURE X creased or decreased pigment, in which sensibility is diminished (insular anes- thesia or hypesthesia) as well as the occasional occurrence of febrile paroxysms. A rare disease isolated by Charcot and Joffroy, hypertrophic cervical pachymeningitis can produce clinical pictures very similar to syringomyelia. Pathologico-anatomically it consists in a great proliferation of the meninges in the cervical region which finally surround the cord like a thick cicatricial band which cannot be separated from the cord proper. Contractions within this great connective tissue envelope lead to solutions of continuity within the gray matter of the spinal cord, which evidence themselves in dissociated anesthesia, and to lesions of the pyramids recognizable by spastic-paretic phenomena. Almost regularly the "preacher's hand" already mentioned, which may depend partly upon secondary alterations in the anterior horn and partly upon changes in the motor roots, is found. The regular involvement of the sensory roots in the meningeal thickening leads without exception to a neuralgiform initial stage of the disease, upon which in its differential diagnosis from syringomyelia, most importance is to be placed. In the late stages of the disease paralysis of sphincters, formation of bed-sores and marked spastic paraplegia of the legs appear and the picture loses its resemblance to syringomyelia, to pass over more and more into that of compression of the spinal cord. Course and Prognosis As a rule, the first beginning of the disease is, as already said, so little marked, that the patients 011I3' late — that is, when they become frightened by the occurrence of muscular atrophy or trophic skin and bone disturbances — come to a physician, usually between the ages of 20 and 25 years. Nevertheless, the presence of the disease in childhood can be affirmed, not only retrospectively from a history of analgesia, etc., but is sometimes directly determined by a physician, the last indeed seldom enough. The further development of the dis- ease proceeds extremely slowly ; exacerbations of rapid progress and long sta- tionary periods alternate with one another. As to life, the prognosis is in general good ; the patients can live to be old, in any case they only rarely succumb to the syringomyelic paralysis of the muscles of deglutition or of those of respiration, sepsis, etc., but die usually of some intercurrent disease. As to recovery, the outlook is naturally poor; nevertheless, trophic lesions can heal up not to return again. Treatment In the treatment of syringomyelics, prophylaxis on the one hand against overexertion, injurious climatic effects, etc., on the other against wounds and burns, plays the chief role. Against the disease itself occasional arsenic cures or galvanization of the spinal cord can be tried. For the technique of this last, see Lecture VII, page 116. The results which have been described from the subjection of the cervical cord to the X-ray are not very convincing, on account of the well-known tendency of the disease to come spontaneously to a standstill; nevertheless, further experiences must be awaited before forming a definite opinion as to this new method. HEMATOMYELIA 119 B. Hematomyelia Hematomyelia, or hemorrhage into the spinal cord occurs rarely spon- taneously, relatively frequently as the result of trauma. In the first case it is either the result of rupture of a vessel on account of pathological brittleness of the arterial wall (arteriosclerosis, syphilis, pernicious anemia) in which usually we find given as causes factors which raise the blood pressure, like sneez- ing, straining, coitus, etc., or the hemorrhage follows secondarily in an already diseased tissue. I saw one case (published by Gerhardt) in which the hemorrhage occurred in an intramedullary glioma, which up to this time had remained entirely latent. Also in acute and chronic myelitis secondary spontaneous hemorrhages have been described. Traumatic hematomyelia occurs in severe contusions of the spinal column, injuries by lightning or electrocution by powerful currents; also, as a result of certain operative measures in pathological labors (difficult forceps extractions, the Veit-Smellie manipulation), or in Scliultzc's method of artificial respiration in infants born asphyxiated. Further, by forced bending forward or backward of the cervical spine (prize-fighters, ''jiu-jitsu," etc.). In typical cases the patient experiences a sudden and severe pain in the region of the vertebral column corresponding to the hemorrhagic focus and falls to the ground with more or less marked paralysis of the muscles distal to this point. Less frequently there is a somewhat slower development of the motor symptoms to which also disturbance of sensibility and of the bladder and rectum are usually added. A great part of these symptoms do not depend upon the direct destruction of the elements of the spinal cord by the extravasated blood, but usually represent remote effects of the hemorrhage which are alluded to as "shock," "inhibition," "'diaschisis phenomena." These are in their nature temporary, so usually in the days, sometimes in the hours, next succeeding the hemorrhage, partial subsidence of the symptoms occurs. Since now the hemor- rhage is usually in the central region the predominating syndrome of hema- tomyelia often presents the greatest analogies to the syringomyelic symptom- complex, particularly in the specially frequent cases in which the location of the hemorrhage is in the cervical region; then .along with spastic phenomena in the lower limbs, atrophy of the small muscles of the hand, with the "ape-hand" or "claw-hand" deformity, a typical dissociated anesthesia and the Horner oculo-pupillary symptom-complex can lie found (see page 1 Hi). Making of a prognosis is naturally only possible after the remote symptoms have cleared up. It depends mainly upon the location of the lesion; if the hemorrhage has occurred high up in the cervical region the prognosis is very gloomy, since in consequence of its nearness to the medulla, paralysis of the heart and respira- tion with high fever is apt to occur. Also location low in the spinal cord has \'r\ evil results, since permanent paralysis of the bladder carries with it the Sanger of ascending infection of tit*- urinary apparatus. Many cases of hema- tomyelia high up in the cord perish rapidly in shock. On the other hand, small hemorrhages in the dorsal and lumbar regions can recover almost without residual symptoms. 150 LECTURE X Therapeutically, in the early stages absolute rest is the chief thing. Care must be taken that through the use of purgatives movement of the bowels takes place without straining, that is without any considerable elevation of the blood pressure. Bleeding, or a series of leeches or wet cups along the spine can be of use under some circumstances. The patients must not get up before the end of four weeks ! The residual parescs, sphincter disturbances, etc., often need symptomatic treatment; we need not go into these things here, since they are considered elsewhere. C. Transverse Lesions of the Spinal Cord Considered from a clinical point of view, discussion of a rather hetero- geneous array of disease conditions under this unique designation, is en- tirely justified; since disease or injury of the cross-section of the spinal cord, whether it owes its origin to a myelitis, to a traumatic solution of continuity, to compression by extra-medullary tumors or tuberculous spondylitis, or to the development of a neoplasm in the spinal cord itself, produces symptom-com- plexes agreeing to the fullest extent.* On this account I intend deviating from the usual method, first to present to you the facts common to the group of disease conditions indicated, then, in order to avoid repetition, I will expose the individual pathogenetic sub-varie- p IG 48 ties according to their particular Extent of the Anesthesia in Transverse pathologico-anatomical and clinical as- Lesions at Different Levels. pects. 1. General Symptomatology of "Transverse Lesions" A lesion of the spinal cord which so severely injures the total cross section of this cylindrical organ at a definite level, that the conduction of impulses by * The same remarks apply to the so-called "concussion of the spinal cord," which though anatomically still little investigated, can give clinically very suggestive pictures. -I. Visscher has communicated to me from the Balkan War some interesting observations of typical "trans- verse lesions" which had occurred without any direct traumatism in soldiers in whose neigh- borhood a grenade had burst or even had passed close by them. TRANSVERSE LESIONS OF THE SPINAL CORD 151 all the fiber tracts is here interrupted, causes, of course, entire loss of sensi- bility and motility in the portions of the body distal to this. Fig. 48 shows the extent of anesthesia in total and symmetrica] transverse lesions at different levels. The extent of motor paralysis is shown in the fol- lowing tables after E. ViUiger. For every assumed transverse lesion, the com- plex of muscles paralyzed is here exposed (those in the column representing the affected segment and all those to the right of this column). In injuries of the spinal cord (dislocation or fracture of the vertebral column) besides this paral- ysis, irritative symptoms from the region immediately above the lesion come into consideration in the clinical estimation of the case. So, for example, as shown in Fig. 49 in destruction of the 7th cervical segment, as a rule, the forearms are Complete Severing of the Spinal Cord at the Level of the Vllth Cervical Segment. (Vertebral Fracture.) held in forced flexion by- contracture of the biceps and the brachialis anticus muscles. In total solutions of continuity, anesthesia extends, of course, to all quali- ties of sensation; indeed, all centripetal conduction is suspended; the motor paralysis, too, is absolute (no paresis, but paralysis), since there is severing not only of the chief motor tracts, that is the pyramids, but of each and everj Connection between the organ of the will and the spinal centers of movement. Now as to the character of the paralysis: according to what we have said in Lecture VII (page 112), a spastic paralysis would be expected, as below tin- level of the separation the reflex arcs are intact and freed by the lesion from inhibiting cortico-spinal influences. In complete transverse separation of con- tinuity in the upper parts of the spinal cord, however, an absolute atonia and arcfli \ia in the paralyzed region is found almost without exception, at any rale, in the early stages. It has been attempted to explain this as due to shock ; tin-, however, is not satisfactory since, first, in many cases atonia and areflexia 152 LECTURE X s 3 j= o c eg > a c a a la a, 3 O C o H -5- CO '3 S 1 « O 3 E « O V OS X c~ •"# 72 CO 3 T3 i a, 5 CO P 'pi a a C>] Short deep neck muscles. - TRANSVERSE LESIONS OF THE SPINAL CORD 153 Segmental innervation of the arm muscles. Cervical segments. Thorac ic segm. 5 6 7 8 1 S o CO Supraspinal Teres min. 1 Deltoideus Infraspinatus Subscapularis 1 Teres majoi a < Biceps Brachialis Coracobraehialis 1 Triceps brach. Anconaeus a « u o Supinator long. Supinator brevis Extensor carpi radial. Pronator teres Flexor carpi radial. Flexor pollic. long. Abduct, poll. long. Extens. poll. brev. Extens. poll. long. Extens. digit, coram. Extens. indie, prop. Extens. carpi uln. Extens. digit. V prop. Flex, digitor. sublimis. Flex, digitor. profund. Pronator quadrat. Flexor carpi uln. Palniaris long. •6 c a X Abduct, poll. brev. Flex. poll. brev. Opponens poll. Flexor digit. V (Ipimnens dig.V Adduct. poll. Palmaris brev. Abductor dig. - ? Lumbricales [nteroBsei 154 LECTURE X Segmental innervation of the leg muscles. Th„ | L, | L, L 3 L t | L 5 S, S 2 a, X H to 1 fa Ileopsoas Tensor fasciae | Glutaeus medius Glutaeus minim. Quadratus femoris Gemellus inferior Gemellus super. Glutaeus maxim. Obturator intern. Pvriioriuis Sartorias Pectineus Adduct. Ions. Quadriceps Gracilis Adductor brevis Obturator ext. ! Adduct. magn. Adduct. minim. Articularis gen. Semitendinosus •Semimembranosus Biceps femoris Tibialis ant. | Extensor halluc. long. | Popliteus Plantaris Extensor digit, long. Soleus Gastrocnemius Peroneus longus Peroneus brevis Tibialis postic. Flexor dig. long. Flexor halluc. long. Extensor halluc. brev. | Extensor digit brevis. Flex. dig. brev. Abduct, hall. Flex, halluc. brev. Lumbricales Abduct hall. Abduct, dig. V Flexor dig. V br. ( Ipponens dig. V Quadrat, plant. Interossei TRANSVERSE LESIONS OF THE SPINAL CORD 155 remain permanently; second, they may occur also in non-traumatic cases (for example, in transverse myelitis). Probably it is the result of the disturbances of lymph and blood circulation occurring in complete transverse affections high up in the cord which lead to serious functional injury of the posterior roots and anterior horn cells in the lower levels. Complete transverse lesions of the spinal cord always lead to bladder and rectal disturbances; there is retention of stools; in the bladder, retention of urine is usually the most prominent symptom; when, however, the distention of the bladder has reached a certain degree, there can be a reflex involuntary dis- FlO, 50. The Brown Signard Symptom-Complex. charge of urine ("intermittent incontinence"). Only in lesions which are sit- uated very low down and destroy the vesico-spinal and ano-spinal centers of the gacral region, other symptoms occur; either a continued dribbling of the urine ("permanent incontinence") or discharge of the urine in drops from the moment the filling of the bladder has reached a certain degree ("'ischuria paradoxa"). Further, incontinence of the bowels. Of vasomotor and trophic disturbances are to be mentioned the dilatation of vessels to be observed in the paralyzed region in fresh cases, which later gives wav to conditions of cold- 156 LECTURE X ness and cyanosis and the marked tendency to deep and rapidly spreading bed-sores. Of great prognostic importance is the differentiation between partial and total transverse lesions. In making this, the following points are to be considered: In in- complete transverse lesions the paralysis is not symmetrical as in the complete ones and has, further, the tendency to partial recovery (naturally, provided the cause ceases) ; also when the lesion is located high, the patellar reflexes are never permanently lost, but, as a rule, exaggerated. Frequently there is a differ- ence between them on the right and on the left. Vasomotor and sphincter disturbances are only present to a slight degree. Further, irritative symptoms may be evident distal from the lesion (pains, twitching of the muscles in injuries to the cord, also permanent erection of the penis, the so-called "priapism"). The phenomena which occur in an only half- sided transverse lesion of the spinal cord, most usually from trauma or in tumor formation, make up what has been called the "Broun- Sequard symptom complex." This is composed of the following symptoms which present them- selves in the parts of the body distal to the seat of the lesion : On the side of the lesion motor paralysis, disturbance of the deep sensibility and hyperesthesia for touch ; on the opposite side disturbance of the superficial sensibility, namely for pain and temperature stimuli. The motor paralysis on the side of the lesion has naturally a spastic character ; its method of production is readily understandable from Fig. 50. The disturbance of the deep sensibility (marked involvement of the sense of position, ataxia) on the same side, also con- sidered in that diagram, is explained by the almost exclusively homolateral ascent of the paths for "bathvesthesia," that is, of the pos- terior columns and the direct cerebellar tracts. Also vibration sense is, as I have been able to recognize, disturbed or lost on the side of the lesion in Broum-Sequard's paralysis. The crossed superficial anesthesia affects always pain and temperature sense, since conduction of these qualities of sensa- tion is almost exclusively carried out through fibers passing over to the other half of the spinal cord. In typical cases touch sense is altered, too, but much Broirn-Stqiicird's Symptom-Complex. Lesion of the Left Half of the Spinal Cord. Disturbances of Sensibility. MB ro ithesia. = Deep Anesthesia. = Superficial Hyperesthesia. TRANSVERSE LESIONS OF THE SPINAL CORD 157 less intensely. This, also, is explainable from the anatomico-physiological con- ditions. Tactile impressions are conducted toward the brain partly uncrossed (in the posterior columns) partly crossed (in the lateral columns). Sometimes there is neither tactile anesthesia nor hypoesthesia. The explanation of super- ficial hyperesthesia on the same side (which is only a temporary symptom) is difficult. Probably after a one-sided lesion, the cells of the posterior horn, which are intercalated in the crossed sensory tracts are loaded down with too much work (since now only these crossed tracts are open to tactile stimuli, part of which normally pass up in the uncrossed tracts) and this overwork mani- fests itself, until the organ has become accustomed to the new relations, by touch stimuli being perceived as painful. If the unilateral lesion is located in the cervical region we have a "spinal hemiplegia," if it is in the dorsal region, a "hemiparaplegia." Fig. 51 illustrates such a case. The narrow anesthetic zone above the region of motor paralysis must be attributed to destruction of the posterior roots entering the affected region of the cord. Typical "classic" forms of the Brown-Sequard symptom- complex are, however, much rarer than atypical ones in which only an incom- plete half-sided lesion or, on the contrary, one also affecting the other half of the spinal cord, is present. 2. DIFFUSE MYELITIS We begin our consideration of the individual affections of the spinal cord leading to transverse lesions with Diffuse Myelitis, that is, with that inflamma- tory process, which in its diffusion in the diseased organ is not limited to definite anatomical elements (in contradistinction, for example, to "poliomyelitis an- terior," which, as we will see, does not, as a rule, pass the boundaries of the anferior horns, and which also does not occur in diffused foci (like disseminated myelitis already mentioned in Lecture IX). The myelitides present the following pathologico-anatomical characters: In very fresh cases, macroscopically the spinal cord in the affected regions ap- pears swollen and somewhat red in color; in the advanced disease it may be softened to a discolored pulp. Microscopically the early stage of the disease is characterized by cloudy swelling and vacuolization of the ganglion cells, with loss of their finer structure as well as of the nerve fibers arising from them; further, by great dilatation of the vessels, minute extravasations of blood, in- filtration of round cells, crowding of the perivascular lymph sheaths with granu- lar cells, etc. In the later stages of the disease the infiltrative processes pro- gressively decrease and "reparatory" proliferative processes in the neuroglia occur. Above and below the inflammatory foci the so-called "secondary de- generations" take place in thcdong fiber tracts of the spinal cord ; following "Waller's law" (see page 10), the degeneration in the pyramidal tracts is de- scending, that in the posterior columns and in the spino-cerebellar tract, ascend- ing (see Fig. 5"2). Purulent myelitis, in which there is breaking down of the spinal cord with pus formation and sometimes an abscess membrane, is exces sively rare. Very infrequent also are the subchronic and chronic diffuse myeli- 158 LECTURE X tides in which parenchymatous destruction is combined from the start with pro- liferative alterations of the neuroglia and of the vessel walls. From an etiological point of view the diffuse myelitides are quite hetero- genous. The acute forms can, as a whole, be considered infectious ; usually they are connected with general diseases (influenza, typhoid fever, acute rheu- matism, variola, erysipelas, malaria, pneumonia, diphtheria, dysentery, gono- coccemia, measles, syphilis). In the so-called "primary or idiopathic acute myelitis," for which sometimes exposure to cold, getting wet, and overexertion have been held responsible, most probably some obscure infection is always re- Tracts which Degenerate Upward. I. Long fibers from the posterior roots. II. Tractus spinocerebellars (a, poster direct cerebellar tract; b, anter Gowcrs 1 column). III. Tractus spino-tha! Tracts which Degenerate Downward. 1. Tractus cortico-spinalis, pyramidal tract, chief motor path (a, lateral pyramidal tract; b, anterior pyramidal tract). 2-5. Further motor tracts, subcortico-spinal tracts i -. tractus rubrospinalis and thalanm spinalis; 3, tractus vestibulo-spinalis; 4. tractus tectospinalis). 5. Descending fibers of the posterior column. dorsal sponsible, and the factors mentioned have only acted as exciting causes. In fact, streptococci, staphylococci, and colon bacilli have occasionally been recog- nized in the diseased spinal cord. On the other hand, it seems certain that infectious myelitides can occur also without microbic invasion of the organ, usually from the action of toxines. For the subacute and chronic forms, syphilis comes particularly into question as a causal factor; occasionally, also, exogenic, chronic intoxications, as saturnism and alcoholism. Abscess of the spinal cord occurs almost only from metastasis, particularly after ulcerative endocarditis. A special position belongs to those forms of myelitis which occur in connection with multiple gas embolisms in the vessels of the spinal cord ; these have been observed in consequence of the development of bubbles of air TRANSVERSE LESIONS OF THE SPINAL CORD 159 in the blood upon abruptly passing from a high to a low atmospheric pressure. Workers on bridge building and >n tunnels are particularly exposed to this "caisson paralysis" or "diver's paralysis" if special arrangements for a gradual "decompression" have not been provided. The development and course of transverse myelitides may be summed up as follows: usually a prodromal stage which is characterized by pain in the back, paresthesias in girdle form, sensa- tion of drawing and formication in the extremities along with general malaise, shivering and rise of temperature, precedes the development of paraplegia. Only very rarely is the beginning abrupt, "apoplectiform." A very slow be- ginning is not frequent and is to be considered prognostically unfavorable as to restoration of function (provided that, syphilis, against which we can act energetically, is not at fault). On the other hand the acute forms sometimes recover in astounding fashion. The longer the fever lasts the more unfavorable is the prognosis; it is further mainly dependent upon the seat of the transverse lesion. Myelitides of the cervical region, on account of their proximity to the medulla and its centers so important to life, is always very dangerous. The occurrence of broncho-pneumonia, large bed-sores, pyelo-nephritis, and the tendency of the disease process to extend further (ascending paralysis) are to be considered as prognostically unfavorable criteria. Interesting in a case of lumbo-sacral myelitis observed by me was the circumstance that the patient afterward, in spite of complete motor and sensory paraplegia, went through two labors, normal except for complete painlessness. According to the symptomatology we can distinguish a number of varieties. First, according to the level of the spinal cord in which the transverse lesion has occurred, a lumbo-sacral, dorsal and cervical form, individual forms which in clinical importance are inferior to ascending myelitis, in which after beginning in the lumbo-sacral region continually higher levels are involved in the motor and sensory paralysis. In this, the inflammation extends by way of the peri- vascular lymph-sheaths upward, as numerous animal experiments conducted by V. Salic, under my direction, have shown. The central canal plays a very subordinate role. Cases in which the ascent occurs in exceedingly violent and Steady manner, so that within a few days from their commencement deatli fol- lows from paralysis of the muscles of deglutition and of respiration, are classed with the so-called ''La/ndry's paralysis," or "paralysis ascendens acutissima." They belong properly, however, with affections which pathogenetieally must be Separated from transverse myelitis, namely with the ascending cases of polio- myelitis anterior which come under the head of the Hcinc-McJiii disease ( see Lecture XVI) and particularly, also, with those of polyneuritis acutissima. As "myelitis migrans" I have described a peculiar case in which a lumbo-sacral transverse paralysis, which had arisen acutely and was to he attributed to chill- ing and infection, recovered. Immediately afterward, however, paretic symp- toms and disturbances of sensibility occurred in the trunk. These symptoms now ascended, in a step-like manner to a certain extent, upward, with recrudes- cence of the fever, while at the same time from below, a return to the normal Occurred, likewise by steps. After the arms had been affected and already had begun to get better, the situation became extraordinarily dangerous on account of the occurrence of par stliesia in the region of the trigeminus and violent hie- 160 LECTURE X cough. At this critical moment the process came finally to a stop, and (in spite of the duration of the progressive period for weeks) at length recovered. This conduct can well be compared with that of '"migrating erysipelas." From a therapeutic point of view, active attack upon the cause is only possible in those forms depending upon malaria and syphilis. In a typical case of very severe syphilitic lumbo-sacral myelitis I saw a most startling effect from an intramuscular injection of 0.6 grin, of salvarsan. Six days after the injection the patient began to move the previously totally paralyzed legs again; on the thirteenth day he could stand, and in the end (after several, this time intravenous, injections of salvarsan) there was complete recovery. It may be attempted to treat the other infections by injections of colloidal silver (col- largol, elektrargol). Whether these really are of value, or if in the cases which have recovered under this treatment it was merely an accidental coincidence, can- not yet be decided. For the rest, we must content ourselves with symptomatic treatment and care. Prevention of bed-sores by frequent change of position, protecting dressings, bathing with alcohol, with subsequent application of ointment of balsam of Peru, rubber rings, water-cushions, etc., prevention of cystitis by the most careful asepsis on catheterizing (which is only to be done when it cannot be avoided), giving urotropin, etc. Complications which have already arisen (bed-sores, cystitis, broncho-pneumonia) are to be treated with all care. To the treatment of the paralyses, what has already been said in the discussion of spastic spinal paralysis, syringomyelia, etc.. applies ; I would refer you also to my later remarks in Lectures XVI and XXII. 3. TUMORS OF THE SPINAL CORD Under tumors of the spinal cord, in a clinical sense, we include both new formations developing in the spinal cord itself and extra medullary tumors which compress the spinal cord; the last are again divided into extradural and intradural neoplasms. Of growths within the cord itself, glioma, sarcoma, tubercle and gumma are particularly found ; the intradural growths are chiefly fibroma and fibro-sarcoma (usually pedunculated), attached to a vessel. Less frequently myxoma, psammoma, teratoma, lymphangioma ; while the extra- dural formations are chiefly lipoma and echinococcus cysts ; still Bircher and I have described an extradural pedunculated fibrosarcoma of the cervical region, which had passed out through an intervertebral foramen and had led to the formation of a second tumor in the fossa supraclaviculars. The two tumors, joined somewhat like an hour glass, could be extirpated and the patient cured. Etiologically, we have naturally definite information only about the in- fectious granulomata and the echinococcus cysts. Among exciting causes trauma must sometimes be considered; so, in my case of extramedullary sar- coma in the upper lumbar region (Fig. 53), the patient had been struck on the back with a cudgel. The development of most cases of spinal cord tumor is marked by a pro- dromal stage with neuralgiform pains, lasting for months or sometimes for years, to be attributed to the pressure which the tumor (in the great majority of cases located extramedullarily and on the dorso-lateral surface of the spinal TRANSVERSE LESIONS OF THE SPINAL CORD 161 cord and growing very slowly) exerts upon the posterior roots. Later the symptom-complex of transverse lesion usually develops and increases very slowly, the spastic paresis occurring early, the disturbances of sensibility later. These last are first manifested as hypesthesia of the feet, and then gradually ascend until an upper limit corresponding to the seat of the tumor is reached. Then, while the upper boundary remains fixed, the intensity of the sensory dis- turbances increases, eventually to complete anesthesia. Finally, disturbances of the sphincters are added; the legs can be drawn up in contracture as Fig. 53 shows. Almost always the Broicn-Sequard symptom-complex is present for some time in typical, or more or less modified development. Earlier or later, there are also atrophic paralyses of certain groups of muscles ; for example, of the small muscles of the hand, in tumors of the lower cervical region. Since the treatment (except in the case of the gummata which can be cured by antisyphilitic measures) can only be surgical, exact localization of the tumor has, above everything else, great importance. Into the points of view important Fig. 53. Paraplegia, with Extreme Contracture in Flexion, in Extra-medullary Fibro-sarcoma of the Lumbar Region. to this end we cannot enter here; I would refer you to my "Compendium of Topical Brain and Spinal Cord Diagnosis,'' where this matter, which is too extensive for the scope of these lectures, receives a treatment sufficiently ade- quate for practical purposes. With very rare exceptions the intramedullary tumors present no indication for surgical intervention; not so the extramedul- l.irv, whose extirpation presents no great difficulties to modern surgical tech- nique. It is noteworthy that from a clinical point of view all extramedullary tumors of the spinal cord, even the sarcomata, are to be considered benign, since after total extirpation no recurrence has so far been observed. The opera- tion is to be considered as dangerous, both in the upper part of the cervical region and (on account of the danger of an infection of the hematoma) in the lower part of the spinal cord. The best outlook is furnished by tumors in the lower cervical and upper dorsal regions. Prom the point of view of differential diagnosis, firsl "pseudo-tumor" is 162 LECTURE X to be considered, which, however, is only to be diagnosed by "autopsia in vivo," that is, by opening the vertebral canal, exploratory laminectomy. These cases are due to circumscribed cyst formations upon the basis of a localized serous meningitis which leads to local compression of the spinal cord in exactly the same manner as true tumor formations ; further, must be mentioned, multiple sclerosis, a failure to recognize which, has led to many unnecessary operations. In order to protect yourselves as far as possible from such a serious error, take to heart the following rules: Multiple sclerosis develops, as a rule, slower than a neoplasm and often shows decided remissions in its course, which, in the clinical picture of tumor are either absent or are of very short duration. By closer analysis of the pain phenomena of which patients with multiple sclerosis complain, it is usually found that there is only very annoying paresthesia (a feeling of burning, of tension, of weight, of twitching). However, actual pains of severe, lancinating or pseudo-neuralgic character which are often plainly dependent upon the weather, do also occur. Pain in the joints speaks for multiple sclerosis. Disturbances of sensibility, which are sharply limited above, occur also in multiple sclerosis occasionally, but there is never complete an- esthesia, as is frequently the case in the advanced stages of a tumor. The Broum-Scquarcl topography of disturbances is very unusual in multiple sclerosis. Of the greatest prognostic importance is also the (often excessively diffi-» cult) distinction between extra- and intra-medullary tumors in which some- times exploratory laminectomy alone can decide. In general, however, we have the following criteria (of which, observe, no one is pathognomonic): In favor of extramedullary location: 1. Slow development of the motor and sen- sory symptoms. 2. Tendency of the motor and sensory disturbances in spite of increasing intensity to remain stationary for a long time with regard to their extent upward. 3. Long duration of the Brozon-Sequard syndrome. 4. The pseudo-neuralgic prodromal stage. 5. Considerable intensity of the spastic phenomena, which persists even after the occurrence of complete paraplegia. 6. The presence of motor irritative phenomena, spasms, twitching. 7. Slight development of degenerative-atrophic paralyses, striking lack of relation be- tween the muscular atrophy and the only slight changes of electric irritability. 8. Sensitiveness to pressure over the spinal column. 9. Yellow color (xantho- chromia) and increase of albumin in the cerebro-spinal fluid. On the other hand a typical and marked dissociated anesthesia speaks strongly for the as- sumption of an intramedullary tumor, while a simple prevalence of disturbance of the pain and temperature sense as compared to that of the other senses also is not rare in extramedullary tumors (great sensitiveness of the pain and temperature fibers to pressure). 4. LESIONS OF THE SPINAL CORD DUE TO AFFECTIONS OF THE AERTEBRAL COLUMN We will only consider this subject quite cursorily, selecting what is im- portant neurologically ; the surgical side of the matter does not come within my province. Luxations and fractures of the vertebral column can lead to sudden "pres- TRANSVERSE LESIONS OF THE SPINAL CORD 163 sure paralysis" of the spinal cord or to complete compression or rupture of this latter. Luxations are more frequent in the cervical region and occur par- ticularly between the 1st and 2d or between the 5th and 6th cervical vertebrae. Fractures, on the other hand, are strikingly more frequent in the lowest dorsal and upper lumbar region. The prognosis depends particularly upon the to- pography of the injur}-; if this has affected the upper cervical region death is usually instantaneous ("broken neck"), but even in the less fatal location of the lesion, bed-sores, paralysis of the bladder, cystitis, etc., present such great dangers that death can occur even weeks later. Tumors of the vertebral column (above everything the dreaded cancer metastases after carcinoma of the breast, the uterus, the prostate, etc.), as well as Potts' disease (spondylitis tuberculosa) have, as a result, slow com- pression of the spinal cord. The clinical, neurological symptoms agree quite well in both processes. The picture is usually ushered in by shooting and tear- ing "root-pains," partly in girdle form, partly radiating into the legs, and of great intensity. Here the neuralgiform prodromal stage is, as a rule, of much shorter duration than in tumors of the spinal cord. Later, band-like hypes- thetic zones usually develop, often, also (as the result of compression of the anterior spinal roots), atrophic paralyses of certain groups of muscles corre-. sponding to the location of the pressure. An increasing exaggeration of the tendon reflexes in the leg, the appearance of the Babinski, Oppenheim, and Mendel-Bechterew reflexes, ankle clonus, etc., are the threatening forerunners of paraplegia, which, ushered in with increasing weakness of the legs, occurs at tin- start without hypertonia of the muscles, but later, however, can develop into marked spastic paresis. The tuberculous caries of the upper cervical vertebrw and of the atlanto-occipital joint which occurs both in children and in adults, presents a particularly typical picture, with which it is necessary to be acquainted, since here the "gibbus" which occurs in other locations is absent. The prodromal symptoms consist in stiffness of the neck and bilateral neuralgia of the occipital nerve, also in "Rust's phenomenon" in which the patient on changing his position supports his head with his hand. Oppenheim has also observed unilateral atrophy of the tongue and spinal accessory paralysis. Later, paralytic symptoms of motor and sensory nature appear, both reaching up to the neck. As to the treatment of diseases and injuries of the vertebral column I would tefer to the surgical text-books. As a palliative, "posterior radicotomy" (par- ticularly dangerous lure, however) in the affected region conies into question. To the treatment of the nervous symptoms remaining after cure of the causal lesions, the principles already repeatedly laid down apply. LECTURE XI The Syphilogenic Diseases of the Central Nervous System As syphilogenic we denominate those lesions of the brain and spinal cord, for whose origin syphilitic disease in the affected individual is a prerequisite. Among' these syphilogenic organopathics we must separate two categories from one another: 1, the syphilitic, and 2, the meta- or para-syphilitic affections. In the first, there is development of the pathologico-anatomical changes in general characteristic for syphilis, in the region of the nerve centers ; in the last, however, there are degenerative lesions of definite portions of the cerebro- spinal apparatus, which histologically have nothing in common with the ter- tiary manifestations as we find them in other organs. Since now we will save true "lues cerebro-spinalis" for a later lecture, in the description of the meta- syphilitic nervous diseases, we will start with the so common tabes dorsalis. The study of this will give us the opportunity of touching upon many points, which apply also to the other metasyphilitic affection of the central nervous system, to progressive paralysis, in the description of which we will hence be in a position to express ourselves somewhat more briefly. A. Tabes Dorsalis By this name, after Romberg's example, is quite generally denominated a disease for which Duchennc, of Boulogne, to whom we owe the first satisfactory description and nosological separation of the disease picture in question, in 1858, had proposed the name "progressive locomotor ataxia"; only in France is this last expression still used occasionally. The syphilogenic nature of tabes is, on the other hand, a discovery of the last two decades which we owe particularly to the investigations of Fournicr and of Erb. In his latest comprehensive statistics from the year 1901, Fournicr was able to demonstrate, positively, a former syphilitic infection in 93 per cent, of tabetics ; since then we have learned that also in tabetics who neither give the history of lues nor upon the most exact physical examination present residua of such a disease (scars, leucoplakia, glandular enlargements, etc.), serological recognition of syphilis is not rarely to be obtained (through the "Wassermann reaction" to be mentioned later). Even where this reaction is not positive, the possibility of a latent or hereditary syphilis is in no way to be excluded. Mendel and Tobias, in their cases of tabes in virgins, have been able with all certainty to demonstrate either a direct tabetic heredity or an ac- quired extragenital infection. To the very rare cases in which a tabetic has 164 SYPHILOGEXIC DISEASES OF CENTRAL NERVOUS SYSTEM 165 recognizably acquired sy T philis, any value as disproving the statement formulated by Mobhis, "Nulla tabes sine syphili," is to be denied. Hence I would not hesitate to denominate a case presenting the clinical picture of tabes dorsalis, in which, however, acquired or hereditary syphilis is with certainty to be ex- cluded as a "pseudo-tabes" (such a case, in fact, I never yet observed). To the conception of tabes belongs, according to our present view, along with the symptomatological criteria, the causal connection with syphilitic disease. Now, however, arises the following question : "Why is tabes such a rare result of syphilis"? {llcumont has, for example, shown that among 3,600 syphilitica only 40, that is, 1.1 per cent., later became tabetic.) May not other factors be determining for the development of the disease (usually 5 to 15 years after the infection) ? In fact, the opinion is wide-spread that here neuropathic heredity plays a part. Erb has found in 28 per cent, of his tabetics hereditary neuropathic factors; in 42 per cent, individual nervous disturbances — not belonging to tabes. If tabetic patients are systematically investigated as to the so-called '"stigmata of degeneration" (malformations of the skull, of the palate, of the ears "h'ssural angiomata," etc.), these are found more frequently and in the average in richer combination than in healthy persons. For the view that an originally defective structure of the spinal cord can predispose this organ to the development of tabes in case of syphilitic infection, the circumstance that it is in tabetics that most of the cases of heterotopia and abnormal course of the fibers have been described, is of some importance. Along with reduced power of resistance (which in the origin of progressive paralysis appears to play the same role as in that of tabes) differences in the luetic virus also are probably determining factors in the later involvement of the central nervous system. There are apparently definite strains of spiro- chietae whose toxines possess particularly strong neutropic properties — a "Lues nervosa," a "Syphilis a virus nerveux." We are forced to take this point of view by the numerous observations, in which, after the luetic infection of groups of people from a recognizable common source, syphilogenic diseases have later occurred in all or in a large number of those infected. Erb, for example, mowed that 4 men infected from the same prostitute later all became tabetic or paralytic. Broslus saw, 12 years later, 5 of 7 victims of an epidemic of syphilis among glass-blowers; 4 of these were tabetic or paralytic. In an entirely analogous manner must be interpreted the considerable number of recognized cases of family tabes or general paresis, in which father or mother, or both parents, along with one or several children, are afflicted with tabes. Family predisposition cannot always be invoked here, since, as a rule, the parents are not consanguine. As group diseases in a small way, the not at nil infrequent eases of conjugal Jabes or paresis can be subjected to the same method of consideration. An important question is whether the insufficiently treated syphilitic runs a greater risk of later metaluetic disease than one subjected to adequate treatment. Many authors, particularly syphilologists, have decided this ques- tion in the affirmative, while, on the other hand, there are those who would make mercurial intoxication in part responsible for the origin of tabes and 166 LECTURE XI paresis. According to my idea, both views are incorrect. What could be more conclusive than conjugal cases of tabes in which we hear that one of the pair has had extended anti-syphilitic treatment, the other none at all, and where still both have equally acquired tabes? We will occupy ourselves more ex- tensively with this question in the next lecture. Edinger has taken a totally different standpoint, in that he attributes to functional use (that is, to the measure of work required from the metaluetic spinal cord) a considerable role in the origin of tabes. In many cases this view has much that is illuminating, namely, in those in which tabes begins quite acutely after severe bodily excesses* or the first symptoms are noted in an extremity continually used. Not rarely, however, Edinger's "overuse theory" fails us, and we know tabetics enough whose disease remains particularly mild and stationary, although they, in spite of all medical advice, continually over- exert themselves (for example, as hunters and riders). Probably race predisposition comes also into question in the tendency of syphilitics to develop metaluetic nervous diseases. It has been scientifically demonstrated that in many races saturated with syphilis, tabes either does not occur or is extraordinarily rare (for example, among the Kurghise of Central Asia, the negroes and mulattoes of North America, the Arabs of Algeria, etc.f Finally, as occasional accessory factors, getting chilled or wet and trauma should be mentioned, which, along with overexertion, explains the fact that after campaigns an increase in the number of cases of tabes has been repeatedly observed among the combatants. Pathological Anatomy Tabes is pathologico-anatomically a systematic degeneration in the region of the posterior roots of the spinal cord. We call systematic, as already indicated (page 111), those diseases of the spinal cord whose lesions are limited to definite fiber systems (as, for example, amyotrophic lateral sclerosis and tabes), while "asystematic" or "diffuse" affections are those whose anatomical substratum is not limited to definite neurons (as multiple sclerosis and tumors of the spinal cord). A knowledge of the structure of the posterior spinal roots and of the further course of their fibers is of great importance for the understanding of the pathological anatomy of tabes dorsalis. The fibers of the posterior roots have their cells of origin in the spinal ganglia; these cells of origin, through one of their processes which runs in the course of a nerve trunk, are in relation with the different peripheral appa- ratus, which we find partly as free nerve endings, partly as terminal bodies (touch cells and bulbs, the Vater-Pacini corpuscles, etc.) in the integument, the mucous membranes, the mesentery, the joint surfaces, etc. The other or central process of the spinal ganglion cell, however, enters the spinal cord through a posterior root. * Corresponding to a certain extent to the generally recognized predisposing role which mental overexertion, excitement, etc., play in the causation of progressive paralysis. f This statement is incorrect in so far as it relates to the American negro. — Translator. SYPHILOGEMC DISEASES OF CENTRAL NERVOUS SYSTEM 167 The posterior root fibers, however, we separate into different categories, according to their further course. The "short fibers" pass directly through the rim zone of the posterior horn into the gray matter and break up either about the cells of the anterior horn, or about those of the posterior horn, on the same side of the cord. In the first instance they serve to convey reflex = Short fibers. Kl ) M = Fibers of medium length. L = Long fibers. The Different Categories of Fibers in the Posterior Roots and Their Continuation in the Spinal Cord. (i. It. —. I Join r'.i tract. Kh. S. St. Direct cerebellar tract. II. St. Posterior column. I'v. S. St. = Lateral pyramidal tract, l'y. V. St. = Anterior pyramidaj tract. stimuli to the anterior roots, in the second to conduct pain, temperature and touch stimuli to the so-called "Tractus spino thalamicus," which, after cross- ing to the other side of the cord, conducts these sensations toward the brain. The "middle fibers" of the posterior roots pass through the posterior roots on into the posterior columns, and from there into the base of the posterior 168 LECTURE XI horn on the same side, and end about the cells of the column of Clarke, from which the stimuli transmitted to the cord through this set of neurones are carried in the direct cerebellar tract to the cerebellum. The third variety, the "long fibers" of the posterior roots also enter the posterior columns through Fie;. 55. Structure of the Posterior Columns. G — Column of Goll (Funiculus gracilis). B — Column of Burdnch (Funiculus cuneatus). a — Long fillers of the posterior roots from the upper half of the body, b — Long fibers of the posterior roots from the lower half of the body. the posterior root zone, then run in these columns upward, and end in the "nuclei of the posterior columns" in the medulla, from whence the conduction of stimuli is transmitted via the optic thalamus to the opposite side of the cerebrum. The fibers of the posterior columns as well as those of the direct cerebellar SYPHILOGEXIC DISEASES OF CENTRAL NERVOUS SYSTEM 169 tracts conduct "deep sensibility," with the difference, however, that the spino- cerebellar tracts serve to transmit subconscious perceptions (of tactile and coordinatory nature), while the posterior column, along with the same func- tion, also serves to carry the conscious perceptions of position sense and movement sense to the cerebrum. The following anatomical peculiarities of the fibers of the posterior columns should also be noted: 1. While the long fibers entering the posterior column FlO. 56. Lumbar Tabes. (Cervical Region.) Weigert-Pal Stain. through a definite posterior root run upward, they are continually forced more and more toward the median line by fibers entering higher segments through the posterior root zone. On account of this, for example, in a cross Bection through the cervical region, the fibers of sacral origin lie next to the septum, those from the lumbar region farther out, then the dorsal fibers, and finally, next to the posterior horn, those of the cervical neurones. In the cervical cord, however, a macroscopical separation has occurred in this way between flic long fibers of the posterior roots which come from the tower half of the body (thai is, from the 4th thoracic segment downward), which are united in the columns of (loll, and those from the upper half of the body (that is, from the 4th thoracic segment upward) which make up the column of Burdach (sec Fig. 55). 2. Each one of tin' long fibers of the posterior roots, before it turns upward in the posterior columns, gives "If a branch (a collateral) which de- scends some distance, also in the posterior column. (In Figs. 54 and 55 tin- 170 LECTURE XI collaterals are omitted for the sake of simplicity.) These descending elements of the posterior root system lie together in a definite bundle, the so-called "Schultze's comma" (see above Fig. 52, page 158); this last, for example, in the upper lumbar segments contains no lumbar fibers, but those which have come in at a higher level of the spinal cord, that is, in the instance selected, belong to the dorsal segments. 3. The long fibers of the posterior root system are not the only nervous structures of the posterior column area. Rather are there mixed among them the so-called "endogenic" fibers of the posterior column, the so-called inter- segmental or association tracts, which connect with one another different levels of the posterior horns. At certain places in the posterior column area the endogenic fibers lie so close together that on cross sections the long fibers of the posterior roots appear quite thinned out. These "endogenic fields of the Fi„. 57. Lumbar Tabes. (Dorsal Region.) Weigert-Pal Stain. posterior columns" are, in the neighborhood of the commissure, the "ventral field of the posterior column" (Zona cornu-commissuralis), at the periphery of the spinal cord, along the septum, the "median peripheral field of the posterior column" (Zona septo-marginalis). Since now tabes, in the great majority of cases, begins as "tabes inferior," that is, as degeneration of the posterior roots in the lumbar region ; after the above remarks it is understandable that in the early stages of the disease, in sections through the higher levels of the spinal cord, it is only the mesially placed long tracts of the posterior column, that is, those which have come up from the lumbar region; in other words, the columns of Goll, which are degenerated (see Figs. 56 and 57). In sections through the lumbar segments, however, a degeneration involving also the lateral portions of the posterior column is found, indeed, it is most marked in the region of the posterior root SYPHILOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 171 zone (see Fig. 58). Upon careful microscopical examination it is found fur- ther, that "Schultze's comma" is not affected by the degeneration. This is not remarkable, since its fibers come from the higher roots not yet dis- eased. The "endogenic fields of the posterior column" are in contrast with the others by being relatively intact, since they carry a large number of fibers which have nothing to do with the posterior root system. In advanced cases of tabes on account of the involvement of higher segments of the cord in the disease process, "Schultze's comma" degenerates also, and finally even in the cervical region all the posterior area is involved. The central point in tabetic disease of the spinal cord is the primary degeneration of peripheral sensory neurones. In this there remain longest '/ Fig. 5S. (Lumbar Region.) Weigert-Pal Stain. intact (at least, according to external appearance) the cells themselves, that is, those of the spina] ganglia, while degeneration shows itself first in their central processes (posterior roots and posterior columns). Their peripheral processes (which are to be sought in the sensory nerves) usually are only found altered in advanced stages of the disease. That the radicular and medullary part of this neurone is more intensely affected than the peripheral bar! is partially explained by the following discovery of Obersteiner and Redlich. These authors showed, namely, that at the point of entrance of the posterior roots into the spinal cord there is normally a sort of strangula- tion of the root bundle, since at this point the pia is thickened and is covered with a particularly close layer of glia. This circumscribed part of the pos- terior root forms evidently a "Locus minoris rcsistentia?." On this account, 172 LECTURE XI even in incipient tabes, it is in the lumbar region where these constrictions are most marked that the roots are earliest and most intensely affected. The whole sensory neurone of the first order seems on account of the method of development peculiar to it (the sensory nerves grow in ontogenesis only sec- ondarily into the spinal cord) to have become the specially vulnerable part of the central nervous system. A certain specific affinity of metasyphilitic toxines for this system of fibers (also for the others affected by tabes, e.g., the optic nerve) seems probable, indeed. Symptomatology As we now proceed to the consideration of the manifold and interesting symptomatology of tabes dorsalis, regardless of the different clinical pictures which it can present to us, we will next pass in review all its disease symptoms in as rational grouping as possible. We will begin, of course, with those symptoms which can be directly attributed to the characteristic anatomical findings in the posterior root affection. This posterior root syndrome is made up of the following components: 1. ATAXIA We have already learned in the first lecture that from disturbance of such centripetal impulses as inform us at any given movement of the position of our limbs, the clinical phenomena of ataxia or disturbance of coordination results. The ataxia of the tabetic produces an exceedingly typical disturbance of gait, hence the name "progressive locomotor ataxia" which Diichenne, of Boulogne, applied to this whole nosological unit. There is a "dysmetria" in the movements necessary for progression, in that the legs are swung out and shoot forward beyond the measure of a normal step; further, the steps are of unequal length. We notice also abnormal positions of the joints during locomotion, so that, for example, the swung leg remains strongly extended at the knee, the point of the foot is directed too much outward and the foot strikes the ground not with the ball, but with the heel. In the most extreme degrees of tabetic ataxia walking becomes impossible, even when the patient is supported on both sides, as every attempt to make steps degenerates into a series of contrary movements in the lower extremities. On the other hand, in the early stages of tabes, the incoordination of the locomotor mechanisms is not to be demonstrated without special tests. Some easily applicable tests adapted to bring out an ataxia not evident in ordinary locomotion are walking on a straight line (in which each foot is alternately to be placed exactly in front of the other), quick turning about at command, walking on the tips of the toes or with the knees bent, walking backward, walking up and especially downstairs, finally walking with closed eyes. This last test makes evident to a certain extent the compensating influence which the optical control of movements can exercise upon the coordination of these last in tabetic patients ; also walking in the dark is considerably SYPHILOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 173 more difficult for them. This influence, however, makes itself felt not only in relation to locomotor or dynamic, but also in static ataxia. Upon this de- pends the well-known Romberg's symptom; if a tabetic is asked to stand with his feet pressed close together (that is, his base of support is made as small as possible), and then to close his eyes,- he immediately begins to stagger and would fall without support. Where in light or incipient cases Romberg's phenomenon is not present or is imperfect, it is well to test coordination by having the patient stand on one leg. It is then noticed that where this last is disturbed, the patient is unable to balance himself on one leg when he closes his eyes, often even with open eyes. In advanced stages of the disease static ataxia often appears spontaneously in that the body of the patient, when he is sitting upright, sways continually from side to side. Tests for demonstrating ataxia of the legs when the patient is lying down are the following: He is asked to describe a circle with each foot; to touch an object held before him with his toe; to touch one knee with the opposite heel (first with open and then with closed eyes). For testing for ataxia in the upper extremities the finger-finger and finger-nose tests, in which the patient first with open and then with closed eyes brings his index fingers to- gether in front of him, or touches the point of his nose, are customary. 2. HYPOTONIA Since for the preservation of tonus a continual passage of stimuli from the posterior roots* to the motor cells of the anterior horn is necessary, it is not remarkable that the prevention of this passage by the tabetic degen- eration of the posterior roots leads to an abnormal relaxation of the muscles. In the region of the pelvis and of the lower limbs the hypotonia is shown by the patient being able to spread his legs abnormally wide apart, or in that we can bring the leg, extended at the knee, into an acute angle with the trunk, and under certain conditions, indeed, we can in this manner, without trouble, •"make a shoulder of the leg" — things which the healthy person can do only al'ti ■]• long training (the "grand ecart" of the ballet dancers, the "art" of tli>- "snake man"). In the knee, on account of the relaxation of the biceps, (temitendinosus and semimembranosus a hypotonic subluxation backward, the so-called "Genu recurvatum" occurs. Fig. 59 shows this deformity, which, however, is not pathognomonic for talus dorsalis, hut can occur in muscular flystrophy, neuritis, etc. On the other hand, hypo tonus of the quadriceps some- times permits bringing the heel up against the buttock. :i. AREFLEXIA While in llic mtv first stages of tabes increase of the tendon reflexes is not tare (evidently as a symptom of irritation of the posterior roots), in the pourse of the disease these reflexes almosl always gradually disappear. On ■ This is by way of the short posterior runt fillers which enter Hie anterior horn directly and hre;ik up about its motor cells. 174- LECTURE XI account of the intimate relation between tonus and reflexes the pathogenesis of this hyporeflexia and areflexia corresponds to that of tabetic muscular relaxation (see also Lecture I, page 7). Most important among the reflex anomalies of tabes is the so-called WestphaVs phenomenon, the loss of the patellar reflex. Complete loss can only be diagnosed when striking upon the patellar tendon produces no contraction in the quadriceps muscle, all tension of the thigh muscles, voluntary or involuntary, being avoided. An excellent means of attaining the best conditions for this experiment is furnished by the so-called "Jcndrassik's maneuver"; in this the patient sits with loosely crossed legs on a comfortable chair, his head thrown back, looking at the ceiling, and the fingers of each hand hooked with those of the other ; after the patient has been previously in- structed to pull hard with his hands as the last number is given, 1 — 2 — 3 is counted, and immediately, while the patient's atten- tion is directed away from his legs, a firm stroke is made witli the percussion hammer upon the ligamentum patella 1 . The patellar reflex can also be tested while the patient is lying down ; the leg to be tested, the knee bent, is lifted from the bed by an aide, the patient being entirely passive and relaxing his muscles. [The limb is best rotated slightly inward. — Translator.] Testing the Achilles tendon reflex is almost as important as that of the patella tendon; it is most easilj' accomplished if the patient kneels upon a well-cushioned chair and turns his back to the examiner, the feet hanging over the edge of the chair. The different tendon and bone reflexes in the upper limbs ( see table, page 8), usually after exaggeration at the start, are lost later than those of the lower extremities (except in the so-called tabes superior). Before reduction or loss of the tendon reflexes are interpreted as indicating tabes dorsalis, it is necessary to find out by questioning if the anomaly can be brought into connection with any former local disease; so, for example, a sciatica very often leaves behind a hypo- or areflexia of the Achilles tendon on the same side. Loss of reflex on one side, or difference between the reflexes on the right and on the left, are frequent symptoms in the course of tabes dorsalis. In con- tradiction to the tendon reflexes, the skin reflexes are almost always retained ; often, indeed, they are abnormally lively. Fig. 59. Genu Recurvatum. SYPHILOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 173 L DISTURBANCES OF SENSIBILITY Even in the carl}' stages of tabes, disturbances in the power of appre- ciating different qualities of sensation, which in advance stages of the dis- ease may increase to anesthesia proper, make themselves felt. Both skin and deep sensibility may be affected (see page 6). a. Disturbances of Superficial Sensibility As to the location of the hyperesthesias and anesthesias to be found on the skin, as a rule they plainly follow the so-called "radicular type" in that the areas of reduced or lost sensation do not agree with the distribution of peripheral nerves, but on the extremities involve more or less longitudinal areas; on the trunk, circular zones. The difference between "peripheral" and "radicular" topography of dis- turbances of sensibility is explainable through the following relations. Each spinal ganglion sends its peripheral processes into different sensory nerves, each sensory nerve contains fibers which originate in different spinal ganglia. In- dependent of the often complicated paths which they have followed in the peripheral nerve, at the extreme periphery, that is, on the surface of the body, the sensory fibers so arrange themselves that those arising from a definite spinal root supply a definite region. This region is called a "radicular zone" or "root field." In it the sensory fibers originally united in one posterior root come together again, even when they are transmitted to the skin through different peripheral nerves. Figs. 60 and 61 expose the difference between "root fields" and peripheral nerve areas. In them the radicular zones are repre- sented as occupying the area on each side of the line which bears their root number. The root fields of the individual posterior roots overlap one an- other somewhat like the tiles on a roof. The special arrangement of the radicular zones in the extremities, in which in contradistinction to the circular arrangement on the trunk a longitudinally directed distribution prevails, is explainable from their ontogenetic relations. In the embryo, the first begin- nings of the limbs, growing out from the trunk, take with them the "dcrma- totomes" (fetal skin segments) lying in their way and corresponding to the same segments of the cord, and since the limbs grow out more or less vertically from the axis of the trunk, in them the principle of circular arrangement is not impaired by the extension of the segmental distribution in the direction of their axes (longitudinally). Tin- radicular hypesthetic or anesthetic zones are found particularly fre- quently on the inside of tin [eg and foot and on the inside of the upper ex- tremities; often, also, in the form of a girdle or a half girdle (since asymmetry is not at all rare) they surround the thorax or the epigastrium (see Fig. 62). In the early stages we find sometimes instead of the more or less bandlike hypesthetic or anesthetic zones, insulated areas of hvpesthesia and anes thesia, usually asymmetrically distributed. So much for the topography of the tabetic sensory disturbances in the integument. As to their quality, they can affect equally the tactile, the pain or the temperature senses. Usually one 176 LECTURE XI variety of sensation is more affected than the others, and this is usually the pain sense. Apart from diminution of sense perception we often find delay in conduction in which the patient first experiences pain some seconds after a pin prick. If in such cases tactile sense is undisturbed, one prick can produce two sensations, in that it is experienced first as a touch, and some- what later as pain. Further, the quite frequent faulty localization of touch, pain and temperature stimuli must be mentioned; if this is so marked that the patient, his eyes closed, when solicited to indicate the spot irritated, mis- takes the side of the body, that is, locates a prick on the right hand on the left side, we speak of "Allocheiria." Hypesthesia or anesthesia of the soles SYPHILOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 177 of the feet, a very common tabetic symptom, is usually experienced as very disagreeable by the patient and impairs his power of locomotion. b. Disturbances of Deep Sensibility Strictly considered, ataxia, already studied, belongs among the disturbances of deep sensibility, as shown in Lecture I. Besides this, interference with "bathyesthesia" is evident in most cases of tabes after they have reached a certain degree of intensity. One can convince himself of the loss of "position sense" without difficulty if, having the patient close his eyes, one extremity is put in a certain position and the subject is requested to put the opposite one in an exactly similar position. The gross errors which he makes give evidence of the fact that he has lost control over the relative attitude of his limbs without the assistance of the visual sense. In analogous manner loss of ''movement sense" may be recognized. Disturbances of the position and movement sense in the fingers lead to "stereo-anesthesia," that is, inability to recognize the form of objects by handling them. The testing of bone sensibility with a tuning-fork can render valuable service even in the early stage of tabes. The loss of vibration sense, or its manifest reduction, is, as I have recognized, the most delicate reagent for detect- ing beginning lesions of the posterior columns. To the early disturbances of deep sensibility usually belong also anesthesias of certain nerve trunks and viscera. The severe pain which firm compression of the ulnar or peroneal nerves normally produces is no longer to be elicited (Biernacki's symptom). The same remark applies to the exceedingly unpleasant sensation which energetic pinching of the Achilles tendon produces in healthy people; this analgesia of the Achilles tendon is called Abadie's tabes symptom. Of the frequent visceral analgesias to pressure I would mention those of the eyeball, the larynx, the trachea, the breast, the ovary and the testicle. Typical "Radicular" Disturbances of Sensibility in a Case of Tabes Dorsalis. 5. TROPHIC DISTl'KHANCES We must assume that, normally, the trophic influence which the cells of i In anterior horn of the spinal cord exert upon the bony skeleton and the integument are stimulated to a certain extent reflexlv through the elements of the posterior root system. In this manner can be explained the fact that 178 LECTURE XI tabetic disease of the posterior roots can sometimes lead to trophic disturb- ances in this apparatus. In the skeleton the so-called "tabetic spontaneous fractures" which depend on pathological brittleness of the bones deserve special consideration. They occur from the slightest causes ; for example, I saw fracture of the neck of the femur from stepping down from the pavement ; fracture of the radius from striking the swinging arm against the back of a chair. Characteristic of these fractures, whose occurrence is sometimes the first symptom calling attention to the presence of tabes, is their painlessness, an important evidence of the already mentioned deep anesthesia. Repair of these fractures some- times occurs normally ; occasionally, however, the callus formation is de- fective, so that pseudo-arthroses oc- cur; on the other hand, an excessive callus formation (leading to severe disturbances of function) has re- peatedly been observed. The X-ray examination of the bones of tabetics, which tend to undergo spontaneous fracture, shows thinning of their compact layer ; besides this, a dilatation of the Haversian canals and a poorness in lime salts of the bony tissue is found histo- logically. Still more interesting are the "tabetic arthropathies" first studied by Charcot. In the pathogenesis of these remarkable joint conditions, different factors combine. Small fractures in the region of the ends of the bones, and particularly 'tear- ing off of tendons, can act as a starting-point, or the hypotonic anomalies of position, for example, the genu recurvation, lead to stretch- ing of the capsular apparatus and to abnormal friction in the joint; to this is added, that in consequence of "deep anesthesia," the patient in such cases does not in the least spare his joint, but rather maltreats it freely; further, abnormal secretory conditions in the synovia, wearing away of the cartilage and the bone, or, on the contrary, pathological proliferation in them, etc., come into play. In this manner exceedingly striking disease pictures occur, which affect far most frequently the knee, then in diminishing frequency the ankle, hip, and shoulder joints. The greatly distended globular joint region, covered with tortuous veins, can justify the comparison with the abdomen of a dropsical child used by Brissaud. At other times marked flail joints, due to the relaxation of the capsular and ligamentous apparatus, occur; or thi' epiphysis is destroyed, in consequence of which the condyles of the femur. Fig. fi3. Tabetic Foot. SYPHILOGEXIC DISEASES OF CENTRAL NERVOUS SYSTEM 179 the head of the femur, or of the humerus, disappear, and the shaft of the diaphysis ends free in the empty capsule of the joint. Besides these atrophic arthropathies, hypertrophic forms occur, in which the proliferative processes gain the upper hand and portions of new-formed bone grow into the joint cavities and throughout the periarticular tissues leading to rapid ankylosis. Through osteoarthropathies in the region of the root and middle bones of the feet, a particular kind of flat foot, the so-called "pied tabetique" (tabetic foot) occurs (Fig. 63). The most important trophic disturbance of the integument is perforating ulcer ("mal perforant"), which in the great majority of cases is located on the sole of the foot (particularly on the ball of the great or of the little toe, or on the heel). This is a round, painless ulcer, which, first superficial, grad- ually spreads deeper and deeper, and finally may expose a joint of the foot. As "mal perforant palatin," Letulle has described an analogous lesion of the palate which can lead to perforation of this through into the nasal cavity. While these ulcerations are usually observed in the early stages of tabes, in the late stages there is a tendency to extended malignant bed-sores, which are located chiefly over the sacrum, but also in other places (heels, trochanters, elbows, etc.). A rare form of tabes, the "marantic tabes" of Oppenheim, is characterized by rapid and complete disappearance of the subcutaneous fatty tissue. As farther trophic skin symptoms of little practical importance, erup- tions of herpes, hemorrhages into the skin, vitiligo, ichthytosis and circum- scribed loss of hair, are observed occasionally in tabetics. Of the other epi- dermal structures, the nails (deformity, brittleness, falling off) and the teeth (painless and spontaneous separation) are sometimes affected. 6. RADICULAR IRRITATIVE PHENOMENA The five previously considered categories of posterior root symptoms — ataxia, hypertonia, arerlexia, sensory disturbances and trophic disturbances — are, as we have- seen, to be interpreted as due to impairment of function. We must now pass on to other radicular phenomena which plainly present the character of irritative symptoms. These are tabetic hyperesthesias, pares- thesias, pains and crises. Already in the early stages of the disease many tabetics complain of prick- ling, formication, burning, or cold feeling, of various distribution, now located in I lie trunk, again in the extremities. On the trunk the circular distribution of these paresthesias sometimes bring it about, that the patients sutler con- tinuously from a feeling of constriction, as if they were wearing a belt or a tightly [accd corset around thi' body. Hyperesthesias also occur in spots or iii girdle form, so that, for example, the rubbing of the shirt, the pressure of the shoe, etc., may become almost unbearable. Particularly frequent is a /one of marked hyperesthesia for cold, just below the ribs. All these paresthesias and hyperesthesias are usually quite constant, and last through long periods of the disease. It is otherwise usually with tabetic Bains, which almost always have a much more paroxysmal character than the attacks of neuralgic pain; we speak hence of lightning or shooting pains, 180 LECTURE XI "douleurs fulgurantes et lancinantes." Their intensity is usually very great, sometimes terrible, so that the patients at every "stroke" or "stab" writhe and scream aloud. On the trunk the radicular topography of the radiations is plainest ("girdle pain") ; in the extremities less so. So, many patients express themselves as having the feeling as if a knife or a red-hot iron was suddenly thrust through their extremities (by far most frequently into the legs) ; others have the sensation of being struck on the shin with a club; still others of the flesh being torn from their bones, etc. On the foot the type of pressure pain is particularly frequent. On this account the French, with a play upon the "Spanish boots" of the torture chamber, speak of "dou- leurs en brodequin." Only rarely are the pains more continuous, and of com- paratively steady, often very moderate, intensity, so that such patients are for a long time considered as "rheumatics." Radicular irritative phenomena in the region of the internal organs we call "crises." They are always accompanied with motor or secretory phe- nomena reflexly produced. Gastric crises are by far the most frequent. At the start they commence with severe pain in the abdomen and in the back, and intense retching and vomiting. In the X-ray picture after a bismuth or barium meal, the spasmodic contraction of the stomach to hour-glass form has been observed. Chemical examination of the gastric juice usually shows marked hyperchlorhydria ; however, this sometimes is absent; indeed, there is occa- sionally subacidity in gastric crises. These excessively painful attacks, which occur with great disturbance of the general condition, can last hours, or even days. In particularly stubborn cases we speak of a "status criticus," after the analogy of the "status epilepticus." In them, also, in consequence of parenchymatous ecchymoses of the mucous membrane of the stomach, there can be vomiting of blood ("crises noires"). The cessation of gastric crises is just as abrupt as their beginning, and even greatly reduced patients usually recover quite promptly from them. Other crises observed in tabes are : Larynx crises, in which the patient is suddenly attacked by a feeling of suffocation and cough, the air is drawn into the spasmodically narrowed glottis, with difficulty and with a whistling sound, the face is cyanotic, and the patient breaks out in a sweat. In severe cases there may be syncope ; the patient even may die ; in rudimentary cases the attack is usually limited to a moderate "suffocating cough." As in all other crises, the return to normal is almost immediate. Pharynx crises consist in painful swallowing spasms, oesophagus crises in painful spasms of the gullet, heart crises resemble angina pectoris. Before these last are diagnosed it is naturally necessary to exclude the (quite fre- quent) coincidence of tabes with luetic heart and vascular disease. Sympathetic crises can produce fleeting pictures which resemble those of Basedow's disease (see Lecture XXIII). Vagus crises lead to paroxysmal alterations of the heart beat and of the breathing; indeed, to apncea and syn- cope. I have interpreted as medullary crises attacks of hypcrpyretic tem- perature with Cheyne-Stohes breathing. Irritative phenomena on the part of the olfactory nerve with spasms of SYPHILOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 181 sneezing and rhinorrhcea, show nasal crises, colicky pains with profuse diar- rhoea, intestinal crises. The rectal and vesical crises are characterized by very severe tenesmus. We have already alluded to testicular crises in speaking of the differential diagnosis of neuralgia of the testicles (see page 53). Renal crises imitate renal colic, liver crises that due to gallstones. The clitoris crises of female tabetics are characterized by spontaneous paroxysms of sensuous feeling, which gradually pass over into painful sensations ; also by hyper- secretion of the vaginal mucus and emptying of the glands of Bartholin. Painful attacks of singultus, finally, are considered as diaphragmatic crises. LECTURE XII The Syphilogenic Diseases of the Central Nervous System A. Tabes Dorsalis (Continued) Gentlemen : In the last lecture we became acquainted with those com- ponents of the tabes symptomatology which may be classed together as "the posterior root symptom-complex" and for whose physio-pathological explana- tion we can point to the pathologico-anatomical considerations with which we preceded our remarks. You have noted in the description of the "crises," that tabes dorsalis passes beyond the bounds of the spinal innervation, and now the description of its important ocular symptoms will bring still more clearly before you that it is not strictly to be included among diseases of the spinal cord, but is rather to be considered as a cerebro-spinal affection. Fortunately, our clinical knowledge of this subject is more satisfactory than what we know of its pathologico-anatomical and physiological basis, hence, since so much in- formation is still lacking, especially as to the explanation of the pupillary symptoms, I prefer not to enter into the matter in order to avoid becoming involved in a discussion of theoretical and disputed questions. OCULAR SYMPTOMS 1. The Pupillary Symptoms. — Since the anomalies presented on the part of the pupillary innervation are common to tabes and progressive paralysis, our description of these will be applicable to what may be found in all the metasyphilitic diseases of the nervous system. In a great many cases of lues cerebro-spinalis we meet with the same conditions, and it is above everything a question if we should not consider them as pathognomonic of the syphilogenic nature of an existing nervous affection. Personally, I am, in common with many other neurologists, of this last opinion ; indeed, I have never found the most important of the clinical manifestations now to be described, Robertson's symptom, in typical development in a patient, in whom a non-syphilogenic dis- ease had to be diagnosed as was shown on autopsy. It is hence just as much a criterion of previous syphilis as a positive Wasserinann reaction. Testing the Pupillary Functions. — Normally, contraction of the pupils ("myosis," action of the sphincter pupilla' innervated from the oculo-motorius) occurs under the following conditions: 1. By the action of light upon the eye being examined or upon the other eye, "direct and consensual light reaction." 2. As a regular accompaniment of movements of the other eye muscles, among 182 SYPHILOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 183 which, clinically, only movements of accommodation and convergence come into consideration ( "synergic pupillary reaction"). 3. In certain individuals upon the basis of the psychical conception "bright" ("ideomotor pupillary re- action"). A pupillary dilatation ("mydriasis" through the dilator pupilla' innervated from the sympathetic), on the other hand, occurs normally in many individuals when a painful stimulus is applied, particularly to the face or neck, further, in fright, anxiety, orgasm, etc. The specific action of many alkaloids, some dilating, others contracting the pupils, is well known. Mydriatics: among others, are atropin, cocain, scopolamin, duboisin. Myotics: physostigmin, morphine, pilocarpin. In every patient, determining the morphological relations of the pupils should precede testing their reaction, whether they are round, irregular, of medium width, large or small, if they are of the same size on both sides or if there is difference between them (anisocoria), finally, if they are centrally or eccentrically located. We must also consider further whether they have been acted upon by any of the above-mentioned drugs and whether there has been, or is. any local disease {e.g., iritis) which could alter the shape and function of the pupils. Next, we examine the direct reaction to light, either to day- light (before a window) or to artificial light (electric lamp, candle, match, etc.). The patient should not be frightened, however, by suddenly throwing a strong light into his eyes, since the psychical mydriasis due to fright may inhibit the light myosis and cause an imperfect reaction. The patient must further look into the distance in this test, in order to eliminate synergic convergence and accommodation reactions. Further, the eye not being tested must be covered to prevent consensual narrowing. On the other hand we test the last-men- tioned reaction by observing the iris of the shaded eye. Usually on sudden illumination, the light reaction after an appreciable period of latence appears very plainly. Following the initial contraction, after several oscillations (physiological hippus) the pupil assumes a medium width. We test reaction for a convergence and accommodation, by having the patient fix his eyes on an object about 1 ' ^ meters in front of him, and then approaching it slowly toward his nose. The isolated reflex pupillary rigidity, or the Argyll-Robertson symptom of tabes and general paresis, manifests itself, in that the pupil dots not contract igither when exposed directly to light, or when the other eye is illuminated, but does react in accommodation and convergence. The Argyll-Robertson symp- tom can occur unilaterally; if it is only partial, a slight direct and consensual light reaction can still be obtained on strong illumination, and we speak then of reflex sluggishness of the pupils. Reflex rigidity is often accompanied by inyosis and irregularity of the pupil,* while abnormal width of one or both pupils is not rare. In a considerable portion of cases, anisocoria is present at tome stage of the disease. Where this is variable, so that sometimes the right fend sometimes the left eye presents a wide pupil, we speak of "bounding * It is not In In- forgotten that in later life, and especially in old age, the pupils arc nor- mally narrower than in young people; such narrow pupils, however, as is shown by man] tabetics ("pinpoint" pupils) (In nut ciinic within the physiological limit. Slow reaction to light is also proper to old age (on account of rigidity of the iris). 184 LECTURE XII pupils." Absolute rigidity, that is, inability of the pupils to react either to light, to accommodation or to convergence, can occur in tabes and paresis, but here it is to be considered an atypical finding. How fundamentally im- portant the exact examination of the pupils in tabetics is, is shown by the statistics of Faure and Dcsvaulx who, in 200 cases found pupillary anomalies of some sort 193 times. 2. Tabetic Diseases of the Optic Nerve. — Upon the basis of tabes (as well as upon that of progressive paralysis and indeed, as an independent mono- svmptomatic, metaluetic affection of the optic nerve) a simple noninflammatory atrophy of the optic nerve can develop. There is a primary degeneration of the optic neurones with reparatory proliferation of the supporting tissue, that is, the pathologico-anatomical analogue of the tabetic degeneration of the posterior roots and posterior columns, which is more to be dreaded in the early stages than in advanced tabes. According to Uhthoff, optic atrophy occurs in 10 per cent, to 15 per cent, of tabetics; according to my personal experience this percentage seems to me too high. As the study of the visual field shows, the so-called "papillomacular bundle" of the optic nerve remains longest intact, so that there is concentric narrowing of the visual field; less frequent, are irregular losses of vision, entirely atypical, central scotoma. Color perceptions (particularly for green) are usually lost before those for white. Ophthalmoscopically the general pallor of the nerve head is next ob- served; little by little it becomes chalk-white, on which account its outlines remain very sharp. Finally, the vessels are also affected and appear much thinned. Where, on the other hand, the atrophy develops from a neuritic process (that is, the papilla appears cloudy, washed out) there is a complicating syphilitic disease of the optic nerve and not a metaluetic, purely degenerative atrophy. This last is therapeutically little to be influenced and of very bad prognosis; it leads in a short time (1 to 3 years) to complete blindness. Only very exceptionally does the degenerative process come to a standstill. 3. Paralyses of the Eye Muscles. — In contradistinction to most of the tabes symptoms previously considered, which are characterized by their ten- dency to progress, or at least to remain constant, the tabetic eye-muscle paraly- ses are, in the majority of cases, of temporary, even fleeting nature. These phe- nomena, also, usually belong to the early stage of tabes, in which the paralysis affects b} r preference a single muscle ("paralysies parcellaire" of Fournier), most frequently the external rectus, but little more rarely the internal rectus or the levator palpebral. Where, on the other hand, the eye muscle paralyses appear in the late stages of the disease, they are usually of greater extent. Strabismus, double vision, ptosis, are their clinical results. We are not entirely clear either as to the location or as to the nature of the pathological substratum of these paralyses, whether there are changes in the nuclei or in the nerve trunks. The circumstance that these disturbances often last only a few days, further, can vary from one day to another, seems to justify the suspicion that they are due to circulatory disturbances, probably to vascular spasm. Permanent or even progressive eye muscle paralyses in tabetics we consider rather as tertiary luetic complications. SYPHELOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 185 OTHER CRANIAL NERVE SYMPTOMS Along with the so important ocular phenomena of tabes dorsalis, its mani- festations in the other cranial nerves play only a subordinate role. The involve- ment of the auditory nerve we have not rarely observed in the form of a progres- sive nerve deafness, with diminution of hearing through bone conduction (to be demonstrated by the Schwabach, Rhine or Weber tests — see Lecture II, page 34), partial loss of perception for the musical scale, tinnitus and occasionally, also, attacks of labyrinthine vertigo. Oppenhelm and Siemerling among others have recognized, anatomically, the degeneration of the auditory nerve. Re- duction of the olfactory and gustatory perceptions, which may increase to anosmia and ageusia, and which Klippell has attributed to destruction of fibers in the olfactory and glosso-pharyngeal nerves, are rare. In the trigeminus, paresthesias, hypesthesias and anesthesias occur; in the facial, unilateral or bilateral, usually temporary paralyses (exceedingly rare). In the hypoglossus, Raymond, Cassirer-Schiff and others have observed, as an excessive rarity, hemiatrophy of the tongue. Symptoms of disturbance in the innervation of the larynx arouse great in- terest. They are far more frequent than the already mentioned larynx crisis with which they are classed by the French under the name "laryngisme tabet- ique." According to Dcjerine, paralyses of the laryngeal muscles, or ataxia of the vocal cords, occur in nearly -i.5 per cent, of tabetics, the former affect by preference the muscles opening the glottis, that is, the posterior cricoaryte- noids. Pareses of the posterior muscles, of slight degree can, particularly when they are unilateral, cause no clinical symptoms, and may only be discovered on laryngoscopy. In severe cases the air is drawn in with a whistling sound, and slight bodily exertion leads to severe dyspnoea; on the other hand, phonation is uninfluenced. More rare, are disturbances in the closers of the glottis which, when they are present bilaterally, show themselves through bitonality of the voice, "breaking," etc., when unilateral, however, remain latent. In ataxia of the vocal cords the voice is tremulous, sensory disturbances of the larynx (hyperesthesia or anesthesia) are also occasionally observed. All these mani- festations belong to no particular stage of the disease, but occur now as early again as late symptoms. They may be inconstant, and in the same patient are sometimes present, sometimes absent. DISTURBANCES IN THE SKELETAL MUSCLES In general the muscles of the tabetic remain normal until the disease is very far advanced and has led to cachexia and great loss of strength, then we almost always see a wasting of the musvles without fibrillary contractions or reaction of degeneration, in which the feet of the permanently bedridden patient, partly on account of the hypotonia of the peroneal muscles which are no longer able to hold up the weight of the feet, partly from pressure of the bedclothes, etc., may take an equinovarus position. Sometimes, however, circumscribed muscular atrophies occur, even in the early stages of the disease, usually in the upper extremities (shoulder-girdle, small muscles of the hand), in which sometimes 186 LECTURE XII partial reaction of degeneration, sometimes only quantitative reduction of irritability, is to be found. Corresponding to tbe degree of atrophy, muscular power is naturally reduced, so that finally marked parescs or even paralyses may result. These atrophic phenomena are, as far. as their pathogenesis is con- cerned, scarcely to be considered as anything unique. In many cases they may present the analogue of the already mentioned trophic disturbances of the skeleton, integument, etc. ; in others, however, which have been distinguished as "amyotrophic tabes," there may be a combination of tabes dorsalis with "syphi- litic spinal amyotrophy" (Raymond, Lcri, Nonne, etc.), which depends upon disease of the anterior horns of the spinal cord. GENITAL AND SPHINCTER DISTURBANCES One of the first symptoms of tabes in men is usually impotence ; there are, however, cases in which potence is very long preserved ; usually a period of sexual excitement precedes the development of sexual weakness ; this may be responsible for the erroneous view of the older authors and which is still in existence among the laity, that sexual excesses may have tabes as a result. Anesthesia of the glans is often to be found along with disappearance of the power of erection. The genital disturbances of female tabetics present themselves apart from the clitoris crises mentioned, above everything in anaphrodisia or indeed, as total anesthesia of the genital organs ; labor sometimes runs its course entirely without pain and may be accompanied by insufficient expulsive power and atonic hemor- rhages. The bladder function very frequently suffers impairment and, indeed, in various forms. Sometimes the patient, in spite of great desire to urinate, must strain a long time before he can pass water; sometimes the desire is immediately followed by emptying the bladder and the patient wets himself, or, on the other hand, the feeling of desire to urinate is lost entirely and the patient only urinates "out of consideration," as Fournier expresses it. All these disturbances are often already present in the early stages of tabes, but are in no way constant ; rather are frequent intermissions, with entire return to the normal, as well as great variations in the intensity of the anomalies, quite frequently observed. Even severe symptoms, as, for example, complete retention which necessitates catheterism, can completely disappear after lasting for months. There occurs, further, ischuria paradoxa, in which discharge of urine (in drops) only occurs when filling of the bladder has reached a certain degree, incomplete retention, in which the bladder can never be completely emptied and a certain quantity of residual urine remains in it ; finally, true incontinence. This last, characterized by continual dribbling of urine and a consequence of atony of the sphincter and of the detrusor vesica?, belongs to the late stages of tabes. It may be only nocturnal, or can manifest itself also in the waking state. Only exceptionally, and then only in the terminal stages, incontinence of the bowels occurs in tabetics, while obstinate constipation, on account of intestinal atony, is quite frequent. SYPHILOGEXIC DISEASES OF CENTRAL NERVOUS SYSTEM 187 THE CONDITION OF THE CEREBROSPINAL FLUID When I now pass over to the consideration of this, of late so freely discussed questions of the anomalies in the cerebro-spinal fluid in tabes and their deter- mination — we will consider here also paresis and cerebro-spinal syphilis — I would warn you against overestimation of results obtained by these methods and the too frequent making of lumbar punctures in all patients with syphi- litic nervous diseases. While this procedure, carried out with proper technique, is scarcely dangerous (see Lecture XVI), it is best reserved for a specialist who is also skilled in the necessary laboratory investigations. He, also, should only use it where an extended clinical investigation still leaves the diagnosis in doubt. Within the limits of these lectures I cannot go into the technique of the Wassermann complement-fixation reaction which is to be applied in the first place to any spinal fluid removed on account of suspicion of a syphilogenic affection. (The amount of fluid necessary for making all the necessary investi- gations is about 5 or 6 cc.) The second test to be applied to the fluid is the globulin reaction of Xonne-Apelt. To the fluid, equal parts of a hot saturated and then cooled solution of ammonium sulphate is added; if in three minutes ( "Phase I" ) there is clouding, this speaks for abnormal globulin or nucleoal- bumin content of the specimen under examination. The third test consists in the cvtological examination of the centrifugated sediment (Widal, Ravaut, ffissl ) : while normally only occasional lymphocytes are found, marked lympho- cytosis is to be considered as a certainly pathological finding.* The indications of these reactions to which, as a fourth, the Wassermann test applied to the blood serum is to be added, in the diagnosis of syphilitic diseases of the central nervous system, is best shown in the following synopsis of Nonne: I. Blood Examination Wassermann Reaction. a. Positive. — Is characteristic for syphilis, with a few exceptions coming little or not at all into practical consideration. A positive reaction is given also by a few cases of scarlatina (only at certain very limited stages of the di-ease), by malaria, by framboesia, by lepra, by the plague, etc. A positive Wassermann reaction from the blood scrum tells nothing further than that the individual under consideration has at some time had syphilis, hereditary or acquired, not that the disease now present must be of luetic nature. I). Negative. — Is to be considered as speaking against paresis since with exceedingly rare exceptions the blood of parities gives a positive Wassermann reaction. II. Examination ok THE Spinal Fi.ru> a. Normal Fluid.— Pressure !'() to 180 mm water (Manometer). Phase 1 reaction negative; at most 5 to cells to the cu mm (Fnehs-Rosenthal counting Apparatus). " By many laboratory workers, in the United States at any rate, the Noguchi butyric acid test for globulin is considered more delicate than thai of Nonne-Apelt. Also it is customary to eounl the cells in (lie fluid willum! centrifugating it.— Translator. 188 LECTURE XII Wassermann reaction, applied according to the original method, using 0.2 cc of the fluid to be examined and also with larger quantities (0.3-1.0 cc fluid) negative. b. Pathological Fluid. — 1. Discharged under increased pressure (over 150 mm water). 2. Positive phase, 1 reaction. 3. Increased cell content. These three symptoms, in combination or alone, show that there is an organic disease of the central nervous system (specified or non-specific). 4. Whether the disease of the central nervous system is of syphilitic nature is decided by the Wassermann reaction applied to the spinal fluid. If the Wassermann reaction, applied according to the original method (0.2 cc fluid being used), is positive there is the greatest probability that the case under investigation is paresis or tabo-paralysis, much more rarely is it cerebro- spinal syphilis, and only in exceptional cases true tabes. In the great majority of cases of paresis the Wassermann reaction is posi- tive with 0.2 cc of spinal fluid. In a few cases of paresis, in almost all cases of cerebro-spinal syphilis and tabes, the Wassermann reaction is only positive when larger quantities of fluid (0.3, 0.4 to 1.0 cc) are used. TYPICAL FINDINGS /. Paresis or Taboparalysis. 1. Wassermann reaction in the blood positive (in almost 100 per cent, of cases). Lumbar pressure frequently increased. 2. Phase 1, reaction positive (in about 95 to 100 per cent, of cases). 3. Lymphocytosis (in about 95 per cent, of cases). Wassermann on the spinal fluid: a. Positive in about 85 to 90 per cent, when the original method is used; (0.2 cc fluid). b. Positive in 100 per cent, when larger quantities of fluid are used. //. Tabes without Combination with Paresis. 1. Wassermann reaction in the blood serum, positive in 60 to 70 per cent, of cases ; lumbar pressure frequently increased. 2. Phase 1, reaction positive in about 90 to 95 per cent, of cases. 3. Lymphocytosis positive in about 90 per cent, of cases. 4. Wassermann on the spinal fluid: a. Original method (0.2 cc), positive in 5 to 10 per cent. b. Larger amounts of fluid, positive in almost 100 per cent. SYPHILOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 189 III. Cerebrospinal Syphilis. 1. Wassermann reaction in blood serum positive in about 80 to 90 per cent. ; lumbar pressure frequently increased. 2. Phase 1, reaction negative only in exceptional cases, otherwise positive. 3. Lymphocytosis like Phase 1, almost always positive. 4. Wassermann on the spinal fluid: a. Original method (0.2 cc) positive in about 10 per cent. b. With larger quantities of fluid, almost always positive (particularly valuable in differential diagnosis from multiple sclerosis, brain tumor and spinal cord tumor). Course and Prognosis The average duration of tabes can be placed at from 10 to 20 years. On the one hand, however, malignant cases which lead to death in a few years occur, and on the other there are those which characterized by special mild- ness, slow progression, complete standstill, or even partial regression of the symptoms, permit reaching an advanced age. For the prognostic estimation of the individual case the following prin- ciples are applicable in general, though, like all rules, they of course have their exceptions : Mildness of course is the more to be expected, the later after luetic infection the disease becomes apparent, the longer also the latent or incubation stage of tabes has lasted ; with this well agrees the fact that cases of tabes in the mature period (beginning about the 5th decade) are usually characterized by their mildness. A tendency to attacks of pain persisting for a long time, al- though this peculiarity greatly influences the comfort of the patient, is usually associated with favorable prognosis as regards preservation of ability to walk and duration of life. On the other hand the richness in symptoms of a con- crete case of tabes, is to be considered unfavorable prognosticallv witli the single qualification that complication with paresis in typical forms of tabes with many symptoms, is scarcely to be feared. The same remark applies in greater measure to Hie cases of tabes characterized by location of the lesion high up (Tabes superior) in which variety the prognosis is clouded by fear of complica- tion witli bulbar symptoms. The estimation of the general condition is very im- portant : Syphilogenic heart and vascular lesions aii' not at all rarely found in tabetics on close investigation and where present alter, markedly tor the Worse, the outlook. Finally, the striking fact is to be considered that the Occurrence id' blindness from tabetic optic atrophy ("amaurotic tabes") very Often puts a stop to the further development of the disease, so that among l'ri neh authors the expression ''Tabes arrested by blindness" is in common use. In a somewhat schematic but practically quite useful manner, three stages in I lie development of tabes are distinguished from one another; the preatactic, ill which tin' gait is not yet altered; the atactic, in which it is characteristically altered on account of the disturbances of coordination; and the paralytic, in which a paralysis is combined with advanced ataxia, so that the patient is per- manently bedridden. Tabes is the cause of death only in very rare cases (par- alysis of the larynx, paralysis of Hie bladder, cystitis and uremia; bed-sores. 190 LECTURE XII and sepsis; inanition and exhaustion after protracted gastric crises; status criticus, vagus crises, etc.) ; death usually occurs from intercurrent diseases (tuberculosis, pneumonia, influenza, etc.), against which the power of resist- ance is greatly reduced, not at all infrequently from syphilogenic diseases of the vessels (angina pectoris, aneurism of the aorta, cerebral hemorrhage, etc.). We will now enumerate the courses which tabes dorsalis may take. Accord- ing to its rudimentary mildness or malignancy there have been separated on the one side rudimentary or abortive tabes, in which the patients never get beyond the preatactic stage (I know of cases which have been in existence 30 years) ; on the other hand, tabes acutissima, "galloping consumption of the spinal cord," which in one of my cases in 9 months ran through the atactic and paralytic stages and led to death in cachexia. Through the time of its beginning the very rare juvenile tabes dorsalis is characterized, commencing in youth or even in late childhood, it arises upon a hereditary luetic basis. According to its loca- tion, special positions are taken by tabes superior or cervical tabes, in which the anomalies att'ect the arms, and the lower limbs present loss of patellar reflex as their sole tabetic symptom ; and the opposite condition, tabes of the conus terminalis. A case of this last variety affecting a 43-year-old woman, care- fully investigated by Thomas and myself, presented the following symptoms: Rectal crises, incontinence of urine and stools, total anesthesia of the genitals (so that coitus was not at all noticed), total peri-ano-genital anesthesia (of the riding trouser form), sometimes pain in the legs, myosis, reflex rigidity of the pupils, exaggeration of the patellar reflexes. A special topographic variety is also tabes amaurotica, of whose frequent inhibitory influence upon the further extension of the spinal lesions we have already spoken ; simple metasyphilitic optic atrophy can be considered indeed, as a "tabes without spinal cord symp- toms." Finally, according to the special preponderance of individual symptoms or symptom-groups, we speak of a tabes dolorosa, visceralis, amyotrophia, marantica. Diagnosis and Differential Diagnosis The diagnosis of fully developed tabes is one of the easiest tasks in clinical neurology and can often be made at a glance. In the early stages the dis- turbances are less striking, so that, unfortunately, disastrous mistakes are frequent enough; so, for example, gastric crises have been mistaken for ulcer of the stomach and laparotomized, also patients with lancinating pains are continually sent into the hospitals with diagnoses of rheumatism, neuralgia, sciatica, etc. Nevertheless, even in the early stages, tabes can be recognized nearly always with certainty, after a thorough general examination. In tak- ing the history and estimating the present condition the following "cardinal symptoms" should be specially sought after: 1. Pupillary disturbances (reflex rigidity or slowness, anisocoria, irregu- larity, myosis). 2. Localized or radicular ( that is on the extremities in longitudinal bands, on the trunk as zones) hvpesthesias or hyperesthesias (the last parti- cularly for cold), anesthesias of the nerve trunks or viscera. SYPHILOGENIC DISEASES OE CENTRAL NERVOUS SYSTEM 191 3. Reduction or loss of tendon reflexes (their exaggeration m no way ex- eludes tabes incipiens). 4. Romberg's symptom, sometimes only to be recognized when standing on one leg. 5. Lancinating pains or crises. 6. Bladder or genital disturbances. 7. Optic atrophy. If four of these symptoms are found the diagnosis, tabes dorsalis, is abso- lutely assured, if three, it is almost certain. A probable diagnosis can be made from two of these symptoms and eventually confirmed by the results of blood and spinal fluid examination. The laboratory methods are a diagnostic neces- sity, only in the quite rare cases of beginning tabes, where most exact clinical examination shows only one cardinal symptom. The following compilation after Oppenheim may give you a conception of the manifoldness of the symptom grouping in tabes incipiens: 1. Lancinating pains, WestphaVs symptom, pupillary rigidity. 2. Bladder weakness, West phuVs symptom, girdle symptom. .'J. Pupillary rigidity, anesthesia on the trunk. 4. Optic atrophy, WestphaVs symptom, analgesia. 5. Optic atrophy, girdle symptom with corresponding hypesthesia, analgesia. o to gr. Yn\ per day). Tinctura nucis vomica' is also a favorite remedy : ty Tinct. nuc. vom 10.0 Tinct. cincona; co 20.0 M. S. 30 drops t. i. d. An excellent combination has been given by Erb in the form of his "tonic- pills." I£ Ferri lactat, Extract, cinchon. aq aa 5.0 (gr. Ixxv) Extract, nucis vom 0.8 (gr. xii) Ext. gentians, qs. ut. Fiat pil. No. C. S. 2 pills t. i. d. p. c. I have had better results from the following: K^ Ferri lactat . 4.0 (gr. lx) Quinin sulphat 3.0 (gr. xlv) Extract, nucis vom 1.0 (gr. xv) Extract, valerian T.O (gr. c) Fiat pil. No. C. S. 2 pills t. i. d. SYPHILOGENTC DISEASES OF CENTRAL NERVOUS SYSTEM 195 Next to strychnine the arsenic preparations should be recommended: some- times arsenic cures produce a manifest improvement of the nutrition and of the general condition, which occasionally has a favorable effect upon the course of the disease. In cachectic symptoms intensive subcutaneous arsenic medication is especially to be recommended. In order to avoid repetition I would remind you of what was said under the therapy of multiple sclerosis. Specially suited for the treatment of tabes appears to be the. in England officinal, iodide of arsenic (Arsenii iodidum, B. P.) in the form of Donovan's solution. R- Arsenii iodid 0.1 Hydrarg. biniodid 0.2 Potass, iodid 2.0 Aq. dest 60.0 .M. S. From 5 to 100 drops gradually increased, in water 2 or 3 times a day. While different derivatives of phosphoric acid, for example lecithin, calcium glycerophosphate, nucleinic acid or nucleinate of sodium, phytin, etc., can be occasionally tried, I can express myself as to the whole opo- and organo-therapy of tabes unfavorably only, also as to the use of spermin, cerebin, etc. From fibrolysin, which has been recommended upon theoretical grounds, in tabes, I have equally failed to see any result. The attacks of pain in tabetics frequently call urgently for symptomatic medicinal treatment. What is to be said here agrees entirely with our previous remark as to the drug treatment of neuralgia, hence we will not again enumerate the. different anti-neuralgics and their combinations, but will refer to Lecture III (page 57). As peculiar to the tabetic pains, we will only refer to their occasional susceptibility to the action of two drugs to which ordinary neuralgias scarcely ever react, namely, methylene blue and sodium nitrite. The first acts, perhaps, through its histo-chemical affinity for the axis cylinders ; the last by relieving vascular spasm. Methylene blue is always to be combined with pow- dered nutmeg in order to prevent irritation of the bladder. Sodium nitrite only acts when used subcutaneous] y, and since it may act as a cardiac poison, is to be given in very carefully regulated doses. I£ Methylene blue 0.1 (gr. \V.,) Powdered nutmeg 0.5 (gr. T 1 /^) M. Divide into 10 capsules. S. 1 t. i. d. K Sodii nitritis 0.1-0.3 Aq. desi ' 10.0 Should be sterilized. S. 1.0 to 1.25 (15-20 minims) daily (carefully in- creasing the quantity of sodium nitrite). The too ready ordering of hypodermic injections of morphine is to be un- qualifiedly condemned, since many tabetics, by carelessness of the physician in tins respect, acquire the morphine habit. Further, it looks as if the use of mor- 196 LECTURE XII phine in tabetics favors the occurrence of gastric crises, probably because mor- phine excites the secretion of gastric juice. Where morphine injections must be used as a last resort, it is best combined with atropin. For the gastric crises Kodari has recommended the combination of pantopon and atropin. ly Pantopon, 0.2 ; atropin sulph., 0.01 ; aq. laurocerasi, 10.0 ; 10 to 15 drops 2 or 3 times a day. Many patients with gastric crises, however, do not tolerate the internal administration of drugs (for example, of cerium oxalate recommended in doses of grm. 0.1). Here suppositories often work well, containing, for example, codein phosph. and extract, belladonna, aa. 0.05 (gr. ^4). These last are to be used also in intestinal crises, while in laryngeal crises local application of 10 per cent, cocaine solution or inhalations of amyl nitrite are to be tried. (Pearls of nitrite of amyl containing each 5 drops are con- venient.) In disturbances of potence yohimbin can be tried. However, if in the dose of 5 to 10 drops of a 1 per cent, solution three times a day (or in tablets of 0.005 each — gr. Vvi), the effect is not satisfactory, it must be given up, as no larger doses should be used. It need not be attempted to excite the libido in such cases in which erection is no longer possible. We will now leave the subject of the medicinal treatment of tabes and pro- ceed to consider the exceedingly important physical methods of treatment. For the use of electricity there is quite an extended field. Of galvanic pro- cedures I would mention, first, the stabile application to the spine which is found beneficial by many tabetics. Two large, flat electrodes are applied, one to the neck and the other over the sacrum, and a current of 5 milleampercs gradually introduced; after 3 minutes the current is gradually reduced, the poles changed, and the procedure repeated. In lancinating pains stabile galvanization, the anode over the peripheral nerve trunks as described in Lecture III, can be tried. It helps only, however, in a very small number of cases. For this indication, more result is promised by energetic faradization with the wire brush acting as a derivative, which often is useful for the paresthesias also. The faradic cur- rent is further applied within the urethra in the treatment of weakness of the bladder ; a flat electrode is placed over the lumbar region while a catheter elec- trode is passed one cm into the urethra ; the individual applications should last 5 minutes, the current being regulated so that it is felt plainly but not pain- fully. Finally, the favorable effects occasionally obtained from the high fre- quency current in crises and lightning pains should be mentioned. As to hydro- and balneotherapy, I would warn you most decidedly against the application of the various cold water procedures. The rapid deterioration often observed in tabetics who have sought aid in "cold water institutions" speaks impressively against these measures. Tabetics are also to be warned against the other ex- treme, namely, hot baths, douches, etc., or mineral springs of high temperature. These remarks need to be modified only as regards a number of local methods of applying heat, which sometimes act favorably against some of the paroxysmal symptoms, for example, hot packs or application of the incandescent light to the limbs in lightning pains, application of the thermaphor or hot poultices to the epigastrium, or to the lower abdomen in gastric and intestinal crises. Of mineral springs the carbonated salt waters are to be considered principally SYPHILOGEXIC DISEASES OF CENTRAL NERVOUS SYSTEM 197 (Nauheim, Oeynhausen, Saratoga, Now York, Ute Springs, Col., Paso Robles, Cal.), also the ordinary salt baths, since arrangements for the artificial addi- tion of carl ic acid are available almost everywhere (Rheinfelden Ischl, etc.). Further, the indifferent thermal waters, as those of Wildbad in Wurtemburg, Badenweiler, Teplitz, Virginia Hot Springs, Byron Hot Springs, Cal. The temperature of the baths should not exceed 35° C. (95° F.), their duration should usually be limited to half an hour. It has become the fashion of late to bring forward the radium content of these baths in order to explain their favorable action ; this is quite possible, but as far as the artificial radium baths (radiogen, etc.) are concerned, they usually fail in lancinating pains, and in the rare cases in which they have been reputed to aid, the element of suggestion can- not be excluded. Good results are to be obtained also with less expensive in- gredients for the baths, for example, with the slightly stimulating pine needle "ozofluin" baths, etc. Prolonged baths at 35° C. without any addition are alleviating for many tabetics. As a powerful derivative application in the lan- cinating pains, I can recommend applications of warm salt. Of mechanical procedures the so-called "suspension treatment" formerly played a great role in tabes dorsalis. Hanging up the patient by means of Glisson's [or Say res* — Tr.] suspension apparatus, affects an "extension of the spinal cord," which in man}' cases exerts a favorable effect upon the crises and attacks of pain. It has shown itself, however, very dangerous, in that softening of the spinal cord has been observed after the suspension. Also the modified proceeding in which the feet of the patient remain on the floor or the extension is carried out, the patient lying on an inclined plane, is now abandoned. Where we wish to exercise a mechanical traction upon the spinal cord or the posterior roots, we can accomplish this by having the patient lie upon his back and, draw his knees up as near to his chin as possible, then keeping them in this position for some time by a band passing around the neck and the bend of the knee. In tain 'tic irritative symptoms, drawing off a few cc of the cerebro-spinal fluid by lumbar puncture sometimes acts favorably. The most important aid in the correction of tabetic ataxia is the compensa- torv exercise treatment developed by Frenkel, Leyden, Goldscheider, and others, in which the patient under the control of his eyes attempts gradually to learn again to accomplish the coordination of his movements. These exercises, how- ever, should never be pushed to the extent of fatigue (which Is best avoided by watching the pulse, since in many tabetics the sense of fatigue is absent), should be given up when the ataxia is rapidly increasing, and are best begun whin we have received the impression that the tabes is in a stationary C lit ion. It is entirely unnecessary to use the complicated apparatus often proposed; most of tin' requisites can lie improvised; for example, the atactic is placed upon a com- fortable chair and is requested t*o practice touching with the point of his foot feme numbers which have been written on the floor in front of him until finally lie can do tliis on command, or a small ladder is placed before him ( Fig. 64), the individual rounds of which he [earns to touch with his foot (practice of eumetria in vertical movements). Then exercises in walking are taken up. in which the IV.t of the patient are to be placed narked areas; for this, simple lines on the floor or a strip of linoleum as in Fig. <>."» and Fig. <>(! can be used. A further 198 LECTURE XII stage is that of making steps of different length, stepping over pieces of wood placed at regular or irregular distances apart, of like or unlike dimensions, etc. ; finally, exercises in climbing stairs. For the upper extremities a chessboard, whose individual squares the patient must touch with his finger, a board with holes into which a stopper is to be placed, or an old typewriter, etc., can be used. In treating the hypotonia and the joint troubles in tabes we may need the aid of the orthopedist to apply a supporting corset in relaxation of the trunk muscles, suitable splints in genu recurvatum and arthropathies. Massage should only be practiced by a skilled operator under the direct control of the physician, otherwise it can only increase the hypotonic and arthropathic deformities and do more harm than good. As a surgical procedure for the relief of the gastric crises, which, however, on account of its great danger, should only be considered when everything else Fig. 65. Exercise Treatment in Tabes Dorsalis. has failed and the symptoms are excessively intense and persistent, posterior radicotomy has of late been recommended by Mingazzini, Forster, and others. This is the intradural or extradural resection of the posterior roots D. 7 to L. 1 (in several stages). Franke has proposed as a less dangerous substitute opera- tion, neurexaresis of the corresponding intercostal nerves in which the posterior roots are evulsed also, Konig, the (in any case little dangerous) production of deep anesthesia by injecting the nerves in question with 0.5 per cent, novocain- suprarenin solution after Brawn. This last method can also be tried for the lightning pains, but only temporary l-esults can be expected. For perforating ulcer, finally, Jabovlay has proposed an operation which consists in dissecting out the femoral artery in Scarpa's triangle on the affected side and separating it for some distance from its vascular sheath. The resulting division of the vaso-constrictor nerve fibers accompanying this vessel, has as its result a periph- eral vaso-dilation which has repeatedly led to the healing of ulcers which had resisted all other treatment. In every tabetic the most minute regulation of his whole manner of life is SYPHILOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 199 of extraordinary importance. The patient should be warned against the damage which every physical overexertion may do him ; walks, should be broken by periods of rest, climbing stairs should be restricted as much as possible; moun- tain climbing, even the slightest, should be forbidden ; also long standing is dangerous. Excesses in every direction, particularly in regard to alcohol, must be prevented, sexual excitement avoided. Regular, full, but unstimulating diet, long night's rest, frequent passing urine at regular intervals, combating con- stipation (when possible by diet); upon all these things great stress must be laid if we wish to aid in fulfilling the conditions contributing to a mild course of the disease. Where there is a tendency to cachexia, but only after gastroin- testinal crises have passed off, full-feeding cures are to be undertaken (see Lec- ture XXVIII). Last, but not least, the psychotherapy necessary for every tabetic should be considered. Avoidance of the tendency to depressive fore- bodings and to exaggerated anxious self-observation present in almost all tabetics, may in itself relieve organically caused difficulties, since psychogenic factors often reinforce disease symptoms having a material substratum and very considerably increase their intensity. LECTURE XIII The Syphilogenic Diseases of the Central Nervous System B. The Progressive Paralysis of the Insane. (General Paresis) With this important disease which Bai/lc, in 1822, isolated as a clinical entity and an intimate knowledge of which has been furnished us by the works of Falret, Westphal, Krafft-Ebing and others, we will occupy ourselves in this lecture only in a very condensed manner. Namely, we cannot attempt to give an accurate picture of the fully developed disease with its different clinical varie- ties, as nearly every psychiatrist has an opportunity to observe it in institu- tions, hence these matters can only be considered very cursorily. We must, how- ever, consider quite thoroughly the particularities of the prodromal and early stages of progressive paralysis, the measures indicated in it, and the points important in differential diagnosis coming under consideration, since all this comes especially within the sphere of the neurologist, even also within that of the practical physician, and since here mistakes in diagnosis and other errors (unfortunately quite frequent) may have regrettable consequences for the pa- tient, his family and the community. The etiology of "paresis" (to use a designation sanctioned by custom) has already been discussed in connection with'that of tabes, and we have there learned that along with the primordial syphilitic damage, different injurious factors in- herent to civilization are to be considered as important contributing causes. The steady increase in morbidity since the recognition of the disease, which according to nearly all psychiatrists, has occurred, stands in close connection with these facts, though as far as I know, extended statistics proving this latter statement beyond a doubt have not so far been furnished. Also we should not forget that to-day progressive paralysis is much more surely and on this account, much more frequently recognized, than was the case some decades ago. Arnaud has shown that paresis occurs about 4 times more frequently in cities than in the country. Pathological Anatomy Progressive paralysis has nothing to do with "softening of the brain" as which it is usually designated by the laity (a peculiar persistence of the erroneous term which the psychiatrist Parchappe used in the year 1838 "softening of the cortical layer"). Rather is the disease character!"^! macroscopically by a progressive atrophy of the cerebrum, particularly s frontal lobes ; micro- 200 SYPHILOGEXIC DISEASES OF CENTRAL NERVOUS SYSTEM 201 scopically by the gradual loss of the tangential fiber layer and of the ganglion cells along with compensatory glia proliferation in the cortex cerebri (particu- larly in the frontal regions and in the island of lied) ; further, by alteration of the cortical vessels (ectasies with perivascular or adventitial infiltration with round cells and with the specially typical plasma cells). In many cases there is added degeneration of the pyramids, in others, the systematic degeneration of the posterior roots and the posterior columns of the spinal cord described for tabes. Only where the last mentioned lesion is already developed in the early stages, the clinical picture of "tabo-paralvsis" occurs ; in the terminal stage on the other hand, more or less marked changes in the posterior columns in the upper cervical region are an almost regular autopsy finding, but clinically ir- relevant. Without exception also there is found a fibrous lepto-meningitis, the "arachnitis chronica," which Bayle considered the basis of the affection. PRODROMAL STAGE The beginning of the disease in the great majority of cases (over 80 per cent.) falls in the 4th and 5th decades of life. According to Obersteiners obser- vations, in 56 per cent, the disease begins between the 36th and the 45th year; the "incubation period" which has elapsed since the luetic infection, he esti- mates upon the average as 121/2 years, the shortest period in these cases was 3, the longest 32, years. Men are about 8 to 10 times more frequently attacked than women. Infantile or juvenile paresis (that is, beginning before the 20th year) is excessively rare and occurs only upon a basis of hereditary syphilis. The first alterations are in the sphere of the intellect and are manifested by defects of memory (particularly for recent events) further, by reduction of mental capacity and by marked distraction and forgetfulness, so that the pa- tient, for example, loses himself on the streets of his own town, or, like one of my parities, who repeatedly, when fishing, walked into the stream without his rubber boots. In the ethical sphere also, there is often alteration. The family complain of formerly unknown acts of neglect, of brutal egotism, of continued irritability. The patient is careless in his business, slovenly in his dress, in- decorous, even obscene, in his conversation. Physical troubles also appear, how- ever. Attacks of hemicrania not rarely in the form of the so-called "ophthalmic migraine" occur. These we will study in a later lecture (Lecture XXX). If Inch paroxysms of headache occur in the thirties and forties in people who have not before suffered from migraine we should be awake- to the eventuality of a threatening paresis. The same' remark applies lei vertigo, feiling of pressure in the head, sleeplessness and other "neurasthenic" symptoms when they occur for the first time in former syphiHtics, in these ominous decades, and we can find no causal factors for acquired neurasthenia (see Lecture XXVIII). Par- ticular] v alarming, however, are the following, it is true not very frequent, phe- nomena: Temporary paresis of one hand, temporary eye muscle paralyses with diplopia; temporary pupillary rigidity or anisocoria, fleeting nocturnal enuresis. The patient usually feels ill ami has a sense of alteration of his psychical per- sonality. The fear of threatened "softening of the brain" sometimes drives him to the physician. ( p also lead to suicide or a suicidal attempt. How 202 LECTURE XIII valuable in confirming the diagnosis in this stage the "four reactions" of Nonne can be we have already mentioned. THE INITIAL STAGE In th> initial stage there occur in different groupings a large number of objective, mainly somatic anomalies which even without the aid of biological methods and the anamnesis throw a clear light upon the nature of the affection present. These are: 1. Pupillary Alterations.- — The extreme importance of these anomalies in the symptomatology of paresis is shown from the fact that according to the statistics of Mignot, Schrameck and Parisot only 6 per cent, of the cases present intact pupils. The anomalies of iris innervation in progressive paralysis are in the main the same as those in tabes dorsalis, and after the very careful in- vestigation of B untie, the Argyll-Robert son phenomenon (reflex rigidity or slowness) occupies by far the first position in frequency. It is interesting, too, that Wolff, Gaupp, Reichardt and Bumke, in altogether about 70 cases of paresis with the Argyll-Robert son symptom, found in each instance changes in the posterior columns in the cervical region, that is, a combination with tabes superior. Fiirstner and Naka have indeed occasionally found absolutely intact posterior columns with clinically surely recognized reflex pupillary rigidity, and we are hence of the opinion that even without tabetic complications the typical Argyll-Robertson symptom can occur in paresis, namely in the initial stage. The cases which come to autopsy, however, have almost without exception reached the terminal stage, and we have already remarked, that in this last, the pos- terior columns of the upper cervical region are almost never intact. Further pupillary symptoms of paresis are absolute immobility (more frequent than in tabetics), irregularity, abnormal wideness or narrowness (the first rarer than the latter), anisocoria (very frequent), and the so-called "bounding mydriasis," that is, a rapid variation between the width of the right and of the left pupil. The last phenomenon originally considered pathognomonic for paresis and tabes, occurs also in Basedow's disease and even in neurasthenia and hysteria. 2. Speech Disturbances. — Already in 1814 the genius Esquirol wrote in a medical lexicon in an article on "Dementia," "Speech difficulty is a fatal sign," and it is not to be doubted that he had in mind cases which since then have been classed with dementia paralytica. Even the public knows that "softening of the brain" begins with trouble with the tongue. The first disturbances usually present themselves in the form of the so-called "syllable stumbling" which Kuss- muul defined as follows: "A disturbance of the coordination of the whole word as a speech unit, with intact formation of sounds and syllables." The patient mis- places letters and syllables, leaves them out, or repeats them in the course of the word (perseveration), confuses them with similar sounding ones. So, for example, for artillery brigade, he says artrallery brigade; for abnormality, ab- normalty; for truly rural, tooly looral, etc. If he is required to repeat these somewhat complicated words several times it is noticed that each time the dis- turbance, which at the start was overcome by strained attention, becomes more marked and finally the word becomes unrecognizable. One must, however, be SYPHILOGEMC DISEASES OF CENTRAL NERVOUS SYSTEM 203 careful with this test since the more educated patients may so be bi-ought to make a diagnosis of their own cases. It is better on this account to have them read a newspaper article adapted to bring out the defect under the pretext of testing their vision. In this (as well as. in the course of conversation with the patient) the following ominous speecli disturbances are readily noticed: First, the "drawling" speech in which it is noticeable that the more important letters are weakened, p is pronounced like b; t, like v (the form of the mouth is natur- ally to be noticed here), particularly that r is pronounced badly, more or less like 1. Further, frequent mistakes, substitution of words, paraphasias, which the patient usually does not observe or correct (in contradistinction to neuras- thenics or mentally fatigued healthy persons) and the slow and hesitating tempo, as well as the monotony and dullness of the diction, often also a certain nasal quality. In conversation, finally, the patient must strikingly often stop and think of a word or phrase, makes many grammatical errors and mistakes of syntax, stalls in the middle of a sentence, etc. 3. Motor Irritative- Symptoms. — In the first place the exceedingly char- acteristic fibrillary twitching in the region of the lower facial should be men- tioned: this either plays over the countenance like summer lightning, now right, now left, now about the comers of the mouth, now over the chin, or spreads as an almost continuous fluttering and trembling over the orbicularis oris, levator anguli oris and quadratus menti : indeed, occasionally (on emotion, etc.), it ex- tends over the ala> nasi and cheeks up to the eyelids. Also the outstretched tongue naves and trembles; sometimes there are recognized in it, plainly, gross con- tractions of the larger muscle bundles. In the muscles of the extremities anything analogous is scarcely ever perceived. However, tremor of the fingers is very frequent. It is not very characteristic but is quick and fine, increasing upon excitement and exertion, and docs not cease even upon complete rest. •i. Alterations of the Handwriting. — Obersteiner gives the following in- b Uigent description of the handwriting of the paretic in the initial stage: "The individual type of the characters is altered, the handwriting is often smaller, sometimes larger, and pointed; it appears (analogue of the hesitation in speech) not as from a copy, often markedly artificial with unusual flourishes, etc. Be- ginning affection of the muscle sense, muscular insufficiencies manifest them- ■elves, by the improper use of light and heavy strokes, getting off the line, un- equal size of the letters, angular excursions, wavering and zigzag strokes; in addition, leaving out of words, or at hast of syllables, occasionally doubling ol the latter, mutilation of words, to paraphasic phenomena." 5. The "Paralytic Fades."— The facial expression of many paretics shows even in the initial stage so typical a picture that a diagnosis at first sight, even among the crowd in the reception-room, is not infrequently possible for the experienced physician. The patient looks like an "all nighter," sleepy, relaxed, expressionless, exhausted; bis face has about it something dull and empty, the action of his muscles of expression is particularly limited. Asymmetric inner- vation of the facia] muscles on the two sides (sometimes varying, so thai to day She right, a fev days later the left naso-labial fold appears more marked), is indeed by far not SO frequenl as in the later stages, but is not rarely observed at I his I ime. 204 LECTURE XIII The Further Clinical Picture Along with the 5 groups of symptoms which we have now considered and which can be called the stigmata proper of beginning paresis, the clinical picture otherwise unfolds itself in this early stage in the following manner: The psychical anomalies which impress their stamp already upon the pro- dromal stage appear now in much more definite manner, since the weakening of judgment and beginning disintegration of the mental personality become con- tinually more evident. The amnesia for recent events is to be observed in much higher degree. Along with this, there is from time to time, clouding of consciousness which leads to occurrences which can no longer be considered as the result of patho- logical forgetfulness and abstraction, but as true confusional conditions. In these, things may be done which bring the patient into conflict with the police and the law, for example, if he enters a strange house at night, begins to sing or to whistle in church during the sermon, urinates on the pavement of a crowded street, etc. On the other hand the defect in ethical inhibition takes the upper hand in a way which often leads to forensic consequences ; particularly often there are sexual assaults upon children, exhibitionism, swindling, defalcations, aggressive behavior in public places and on the street, cheating at cards, etc. A teacher treated by me ran amuck among his pupils with a pair of compasses. The increased feeling of self-importance which, in the fully developed disease as- sumes the classical form of "grandiose delusions," can show itself even in the initial stage in senseless expenditure, reckless making of debts, a craze for in- vention, tendency to risky undertakings, etc. Inability to criticise his own acts or those of his surrounders, testifies to the growing preponderance of intellect- ual defects. The physical troubles enumerated for the prodromal stage either diminish in the initial stage or, on the contrary, they manifest such obstinacy and intensity (particularly vertical headache and sleeplessness) that they are markedly differentiated from the same symptoms as observed in neurasthenia. Occasionally an unconquerable desire to sleep by day is in contrast with the agrypnia by night. Consciousness of illness is now still present only for the physical troubles, not for the mental abnormality. Not rarely there appear even in the initial stage those ominous paroxysms from which only a minority of paretics are spared, but which, as a rule, do not come on until later in the disease, and which are known under the name of "paretic seizures." We dif- ferentiate apoplectiform and epileptiform attacks. In the first the patient falls unconscious as in a true attack of apoplexy, and shows a hemiplegia after he comes out of the coma. This, however, entirely disappears within a few days (occasionally even after a few hours), and the attack is accompanied by a rise in temperature which is in contrast to the subnormal temperature of cerebral hemorrhage but parallel to many of the apoplectiform paroxysms of multiple sclerosis. Krafft-Elmig has pointed out a further differential point in their diagnosis from cerebral hemorrhage. In the latter, the tendon reflexes in the region pai'alyzed are regularly lost for about 2-4 hours after the attack ; in the paretic hemiplegias, they are exaggerated immediately after its onset. Kraepelin is of the opinion that many cases of death at middle age from "apo- SYPHILOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 205 plexv" are really examples of a fatal ending of apoplectiform attacks in the initial stage of paresis. More frequent are epileptiform attacks, -which also are often accompanied by hyperthermia. They have usually the character of the so-called "Jacksonian or cortical epilepsy" which we will study in a later lec- ture (XIX) more fully. At present I would only remark that they consist in tonic and clonic spasms which usually begin in one corner of the mouth and spread from there over the face and extremities of the same side and often to the other side. Sometimes, however, they exactly resemble genuine epilepsy: The patient falls with a cry, is unconscious, shows general tonic and clonic convulsions, his face is cyanotic, fa?ces and urine are involuntarily discharged, and he foams at the mouth. After the attack, which lasts only a few minutes, tin- patient is confused for a long time and regains entire consciousness only little by little. In connection with paretic seizures, both apoplectiform and epileptiform, there occasionally occur, usually very temporary, aphasic, or apractic conditions, symptom complexes, which we will subject to a thorough consideration in a special lecture (XVIII). Changes in the optic nerve corresponding to those in tabes can also occur in paresis, but this is, on the whole, quite rare. Joffroy, among 227 paretics, only saw more or less developed atrophy of the papilla 27 times, and these were usually alterations which developed only in the later stages. THE STAGE OF THE FULLY DEVELOPED PSYCHOSIS The paroxysms just described can, in that they leave behind a considerable degree of mental deterioration which further rapidly develops into dementia, mark in rather precise manner the passing of the initial stage into dementia paralytica proper; more frequently, however, this change is imperceptible. Where, however, the disease which has fully developed manifests itself in de- lusional ideas and delirious conditions, the beginning of a new and more severe stage of the affection is recognizable even to the laity, the usual consequence of which is the bringing of the patient under psychiatric care. In the usual manner this paralytic psychosis will now be subjected to a bather schematic classification in that we speak of a depressive, an expansive, an agitated, and a dement form. If it is always remembered that between these forms there are intermediate varieties, and that they even can pass over into one another, there is no objection to using didactically these divisions. We will hence, adhering closely to the descriptions of Qberstevnm and Kraepelin, present the clinical criteria of these' 4 chief forms. 1. The Depressive (Melaneholie) Farm. — Depressive delusional ideas which show lack of insight, loss of power to criticise and which cannot be corn cted. are manifested, often iii an absurd manner. These can be of hypo- chondriacal nature (the patient. Cor example, laments that his head is shrunken, that he has no stomach, that his body has turned to pus, etc.), or they can take the form of "micromania," having committed great sins, etc. (The patient complains thai he has stolen a million; that he has destroyed his family; affirms that he is dead and refuses food, since a corpse cannot eat.) Into the course of this depressive form, conditions of maniacal exaltation with grand delusions 206 LECTURE XIII may force their way, and when the alternation is regular, we speak of the "cir- cular form" of paresis. 2. The Expansive ("Classic'") Form. — In this variety, to which the first descriptions of the disease applied, grandiose ideas, increasing to the im- measurable, dominate the picture. While the patient first contents himself with declaring that he is the richest, the handsomest and the healthiest man in the country ; before long he takes himself for a king, an emperor, God, Supergod ; he is going to marry princesses ; to buy Australia ; he is building a railroad to the moon, etc. One of my patients, immediately after the epileptiform attack which ushered in his psychosis, wrote to the King of the Greeks to urge him "in the name of the Swiss people gathered on the .shores of the Lake of Zurich" to make a republic out of Crete. 3. The Agitated (Maniacal) Form. — This variety is characterized by conditions of maniacal and delirious excitement. The patient disturbs day and night, roars and sings, holds confused conversations marked by flight of ideas, sleeps hardly any, loses flesh rapidly; attacks of real frenzy and dangerous assaults upon his surrounders also occur. The most severe cases of agitated paresis have been called "Galloping Paralysis." In these the patient after a few weeks of the most extreme excitement, obstinate refusal of nourishment, complete filthiness and absolute sleeplessness, perishes from heart weakness or from dysenteric diarrhoea. 4. The Dement Form. — Under this name, we include all the cases in which from the start progressive dementia stands in the foreground of the psychical symptomatology. Amnesia stretches continually farther backward until finally his whole previous existence with all its impressions and experiences are oblit- erated from the memory of the patient. Orientation for time and for place suffers and is finally lost entirely. The power of speech is impaired more and more, not only on account of the increase of the syllable stumbling and the anomalies occurring even in the initial stage, but .also through the progressive extinction of the speech memories. "Amnesic aphasia" of the most extreme de- gree so occurs. Since also the "conceptions of movement" are defective an "ideational apraxia" goes hand in hand with this last. The gait also is slow, clumsy, uncertain ; there is incontinence of urine and fa»ces. Remissions and Intermissions The severe disease pictures just described can (sometimes even suddenly, almost from one day to another) so far disappear that the patient and his family get the impression of a cure, while we, in such cases, usually speak only of remissions, occasionally, however, of true intermissions. In the remissions a return to the psychical and somatic status of the initial stage usually occurs. Sometimes, however, there remain more or less marked speech disturbances, and regularly indeed the pupillary anomalies formerly present. During these remissions which last for weeks and months, seldom for years, the patients are often let out of institutions by psychiatrists and many times take up their call- ings again. It is of capital importance that they remain under permanent and careful medical observation ; only too frequently, however, they are with- SYPHILOGEMC DISEASES OF CENTRAL NERVOUS SYSTEM 207 drawn by their friends from such oversight until a paretic seizure or the sudden recurrence of their delusional ideas again bring to the front the great seriousness of the situation and dissipate the beautiful illusions as to cure. I have so far only seen paretics of the expansive and depressive type, who have been let out of the asylum on account of remissions, return to my care; the longest of these remissions lasted one and one-half years. Frequently I have been able from return of the agrypnia, slight speech disturbances, pathological irritability, headache, unreasoning desire to speculate, etc., to diagnose the threatened return of progression of the disease and to put the patient back in the asylum in time; in one case in which the wife of the patient could not be convinced of the necessity of this measure, there broke out on the next day a condition of extreme excitement with tendencies to violence and to suicide, in which dreadful results were only with great difficulty prevented. One should also exercise continued control over paretics during these remissions since it is not only one of the most thankless tasks, but also one of the most responsible positions in which the physician can be placed. The great frequency of remis- sions in the classical form of progressive paralysis is emphasized by most psy- chiatrists; in the agitated form they are rare, in the dement form they almost never occur. True intermissions of j-ear-long duration are not entirely rare. My teacher. L. WiUe, told me about one of his paralytic patients, who, during an inter- mission, was able to assume again his position as minister of a small German state. A patient of Kracpel'uis not only discharged his duties as employee in the telegraph office for 5 years, but rose to higher positions, stood examinations and married. Another one who presented grandiose delusions, speech disturb- ance, pupillary rigidity, WesiphaVs symptom, and attacks of vertigo, lost his delusional ideas and could again occupy his former position as janitor at a school for years. A patient of Tuczck, who in his grandiose delusions de- clared himself Pope, Emperor of Germany, King of Darmstadt and father of 187 boys born at the same time, who presented the characteristic somatic stig- mata of paresis, and after repeated paretic seizures, had already passed into a condition of advanced cachexia, little by little recovered completely both physi- cally and mentally, took up again his position as conductor, and 5 years after the beginning of his intermission was promoted "on account of exceptionally good work," then 2 years later had to give up his position finally because of '•talus." Only just before his death, 20 years after the beginning of the inter- mission, he presented again mental anomalies, which however, Knoblauch, under whose care he had come, considered only as "marantic confusion." The autopsy showed in the brain none of the alterations characteristic for progressive paraly- sis. It was otherwise in a case of Dobrschansky's, in which after an intermis- sion of 15 years, practically amounting to recovery, the autopsy showed typical paralytic alterations. THE TERMINAL STAGE However different the clinical pictures of florid paresis may be, the patients finally in the end stage all come into the same wretched condition. The de lusions have disappeared in the predominating dementia, the patient has lost 208 LECTURE XIII all mental relations with the external world, even, indeed, the consciousness of his own personality, his movements are now only rudimentary, his discharges are all passed in the bed, he utters only inarticulate sounds, must be fed, the heart's action grows feeble, the skin is anesthetic, livid, cedematous, trophic dis- turbances, bed-sores, abscesses, hypostatic and aspiration pneumonias develop, and put an end to a vegetative existence hardly any longer to be considered as "life," if death does not occur from paralysis of the heart in a paretic seizure. In the end stage, "status epilepticus" from an unbroken series of attacks is frequent. Prognostic Cases like those of TuczeTc and Knoblauch, are such rare exceptions that they cannot prevent us from making the general statement, that general paresis leads inexorably to death ; besides, the autopsy findings in these patients also do not exclude the fact that there are particularly complete and long intermis- sions, during which the patient may be taken away. It is certain further, that in many of the so-called "recovered pareses" the condition was really some other symptomatologically closely related affection (alcoholic pseudo-paralysis, cere- bral syphilis with psychosis, etc.). The proximate causes of death can be: in the early stages, and further, at the height of the agitated and depressive forms, suicide; in all stages, paretic seizures; in the terminal stage, aspiration or hypostatic pneumonias, ascending infection of the urinary tract, sepsis from bed-sores or injuries, asphyxia from "choking," finally, simple marantic heart failure. Between the individual clinical varieties there is a certain difference in malignancy. Relatively more favorable is the "Classical," the expansive form; in it remissions may most frequently be hoped for, the paralytic attacks which almost always hasten the progress of the disease are comparatively rare, and finally, long stationary periods occur. So, then, this form can here and there present quite a long duration of the disease (to about 15 years) ; in general, however, from the beginning of the grandiose ideas to death, a period of about 3 years can be predicted. The other forms usually lead in less than 2 years to death ; apart from the "galloping" variety of the agitated form, the dement type is most malignant, since in it, the paretic seizures are very frequent during the whole time of the disease and remissions are almost entirely excluded. Differential Diagnosis In the prodromal stage the differentiation of progressive paralysis from neurasthenia can be a matter of great difficulty when it concerns a patient who has had syphilis. Not a few former syphilitics become neurasthenic on account of fear of paresis. It has been asserted, however, often with too great positive- ness, that this nosophobia speaks against paresis, since in this, prodromal auto- diagnosis are not at all rare. It is notable on the other hand, that the neuras- thenic occupies himself much more with his troubles and observes them much more carefully and in detail, notes them down and describes them to the physi- cian more frequently, than the, even at the start, rather indolent paretic, who is SYPHILOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 209 generally indifferent and forgetful as to the changes in his own personality. Plain alterations of character for the worse, with transgressions against pro- priety and custom, speaks strongly for paresis. Further, the "weakness of memory" complained of by neurasthenics on careful testing, shows itself to be only the expression of the imperfect and abnormally fatigable, power of mental concentration, while that of the paretic represents a true dys- or amnesia for recent events which, indeed, is plainer objectively than subjectively. Loss of topographic memory pictures, getting lost, never occurs in neurasthenia. Very properly, on the other hand, Oppenhcim suggests that imperative conceptions speak decidedly against paresis. Very suggestive of the latter are temporary paresis or nocturnal enuresis. With entrance into the initial stage with its char- acteristic somatic anomalies and the increasingly more evident psychical dis- turbances, the differential diagnosis soon loses its difficulty, particularly when a paretic seizure roughly tears down the last veil. We must not forget here to allude again to the great value of the "4 reactions" of Nonne. Also when the diagnosis between hysteria and beginning paresis is difficult the chemical, cvtological and serological blood and spinal fluid examinations can greatly aid us. As an example I would mention to you a case described by Jusgen which also is important medico-legally since not rarely a head injury gives the signal for the outbreak of progressive paralysis. It was that of a workman who was struck upon the head by a piece of falling timber while in apparently good health and in whom, after about 4 weeks, mental alterations not rare in traumatic neuroses appeared ; now indifference, now labile "affects" and nervous complaints. Clinical examination showed "hysterical" convulsions which partially could be relieved by suggestion; on the other hand no positive signs of progressive paralysis. Nevertheless, blood examination showed a strongly positive W assermarm reaction, as did also that of the spinal fluid with increased globulin and pleocytosis. The further course of the disease justi- fied these reactions; during the first weeks of observation, indeed, the hysterical symptoms remained fully developed, namely, there was great suggestibility. Then, however, there was rapid, unmistakable change; negativism was sub- stituted for suggestibility, the patient became restless, spoke in monotone, mani- fested foolish, depressive ideas and passed into a marked paralytic stupor. The differential diagnosis from multiple sclerosis was already discussed in connection witli this last (page 139). That from the so-called "pseudo-paralv- .si>" (of luetic and arteriosclerotic nature) as well as from certain manifesta- tions of brain tumor, will be taken up in later lectures (XIV, XV, XIX). We must not neglect to consider at this point "sleeping sickness," all the more, since this affection, as Spielmeyer has recognized, not only has clinical analogies with progressive paralysis, but also etiological relations (similarity between fcrypanosome and spirocha'te infection) and a certain pathologico-anatomical agreement. Along with the somnolence, this endemic disease of the black por- tion of the earth's population, which is called after its most striking symptom, shows frequently disturbances of motility and of the reflexes, epileptiform at- tacks, speech disturbances, decrease in intelligence, weakness of memory, de- lusional ideas; besides this lymphocytosis of the spina] fluid. In Africa the differential diagnosis between these two conditions can sometimes be difficult, 210 LECTURE XIII namely when the "sleeping sickness" (which indeed is seldom the case) attacks a white person. Treatment On account of our inability to permanently arrest the disease process, as soon as the diagnosis is confirmed, we must endeavor to delay the development of pro- gressive paralysis as much as possible and to favor the occurrence of remissions. In the prodromal and initial stages the most important point is immediate re- moval from business activity and transference to as quiet and nonirritating surroundings as possible, for example, such as are found in well managed sani- taria. You cannot be too urgently warned against sending such patients to hydrotherapeutie institutions (there are unfortunately too many) where the patients are subjected to fixed cold water procedures and incipient paretics are thereby greatly injured. If in a neurasthenic condition there is even the slight- est suspicion of paresis in the prodromal stage, care should be taken that the patient is not given cold baths or douches. Great importance is to be attached to full but simple and nonconstipating diet, with avoidance of all stimulants (alcoholics, spices, tea, coffee). For sleeplessness drugs must often be resorted to (veronal, 0.5—1.0 (gr. 8—15); trional, 1.0—2.0 (gr. 15—30; scopolamin, 0.0005 (gr. Vioo); paraldehyd, 3.0-5.0 (oi-oii) (greatly diluted); amylen- hydrate, 2.0—4.0 (." ss.— 5i), (greatly diluted), may be alternated as necessary). No time should be wasted on baths, sponging, or psychotherapy; if the tendency to agrypnia is reduced, the hypnotics may be gradually limited or withdrawn, although in this case they represent the less of two evils, since we must unceasingly attempt to procure rest for the diseased brain during the night. The question as to whether antisyphilitic treatment should be instituted or not, cannot be answered in general terms. Many psychiatrists and neurolo- gists, as Krafft-Ebing, Kraepclin and Dejerlne, consider mercurial treatment as contraindicated when the diagnosis is certain, since it reduces the nutrition of the patients and in a metasyphilitic disease can accomplish nothing. Others, as Leredde and Ziehen, on the contrary, are of the opinion that under mer- curial treatment remissions oftcner occur, are more marked and last longer than without such treatment. Ziehen hence advises the trial in every incipient case of a course of inunctions with subsequent administration of iodides, and even in advanced cases he has an inunction made twice a week and gives 0.2 (gr. 3) iodide of sodium a day. I consider an intermediate standpoint correct, and limit the mercurial treatment to early cases which have not previously been sufficiently mercurialized, that is, when they give a decidedly positive Wasser- mann reaction ; further, I avoid too energetic treatment, and content myself with a series of 10 to 12 intramuscular injections of about 1 cc of the following solution: Hydrarg. biniodid, 0.2; sodii iodid, 0.2; sodii chlorid., 0.075; aq. dest. 10 cc. (The series can be repeated later.) I have certainly never clone any harm with these injections (given at intervals of from 1 to 2 days), but believe that I have occasionally aided in the establishment of remissions. As to salvarsan, with which, however, I have had but very limited experience, I can neither affirm the one thing nor the other. Since I have in two instances SYPHILOGEXIC DISEASES OF CENTRAL NERVOUS SYSTEM 211 gained the impression that it accelerated the disease process or provoked seiz- ures and have had similar experiences narrated to me by colleagues, I advise on principle against this otherwise so valuable remedy, in paresis. Only in one case was there improvement, and this was only in the subjective symptoms, while speech disturbances, facial tremor, sluggishness of the pupils, intellectual weakness, etc., were not influenced in their progress by the injections. Into the symptomatic therapy and care of the fully developed disease and of its terminal stage (sedative drugs, keeping clean those who suffer from in- continence, the prevention and treatment of bed-sores, tube feeding in refusal of food, when there is danger of choking, etc.) we need not enter; these things are mainly for the asylum psychiatrist. It may be said, however, that for getting a disturbed paretic into the asylum, a large dose of scopolamin hydro- brom. (0.001 =gr. 1/60) injected subcutaneously, exerts a strikingly sedative effect (''chemical camisole"). In conclusion, a few words upon some new and interesting, although yet incomplete, therapeutic experiments. These are based upon the familiar ex- perience that remissions in progressive paralysis occur, not very infrequently, after febrile, infectious and suppurative processes. Upon this was based the old methods of provoking suppuration by the inunction of Autenrieth's "Pus- tulating ointment" (ung. tartari-stihiati) or by vaccination. Of late the Vienna psychiatrist Wagner v. Jauregg has advised the systematic use of tuber- culin injections, upon the favorable effects of which with regard to the oc- currence of remissions and in prolongation of life in paretics, Piles and Dobr- schansky have reported. Beginning with a dose of from 0.01 to 0.1 of tuber- culin, the dose is gradually raised to 0.5; this last dose is given 7 to 12 times, each injection at the interval of about 2 days. The raising of the dose is dependent upon the degree of febrile reaction; temperatures of over 39° (103.1° F. ) are avoided as far as possible. Sometimes Wagner precedes the tuberculin cure by antiluetic treatment. Friedlander has reported favorable results from dead cultures of colon and typhoid bacilli. O. Fischer and Donath produce in paretics an artificial hyperleucocytosis by injections of nucleinic acid; the latter uses the following solution: 1} sodii nucleinat., sodii chlorid., Is '_'.(); aq. dest. steril., 100. M. S. use for one or two injections. The in- jections, carried out at Intervals of from 5 to 7 days, should produce a leu- coevtosis of from 10,000 to 25,000 lasting some time. Sometimes they produce decided local reaction. Donath found in 70 per cent, of the cases treated more nr [ess marked improvement. The recent discovery by Noguchi .-mil Mnin-r of spirocha'br in tlir brain substance in gen- eral paresis (confirmed by other observers) semis to settle the question of the relation between this disease and syphilis. Very recently Noiiiirhl has reporteil finding spirochaetae in the spinal cord in one ease of tabes out of twelve examined. In view of these discoveries, it has been urged by sonic writers that the terms "inrta-" and "para-syphilitic" be abandoned. In so far, how- ever, as they are used to imply certain pathological changes differing from those usually consid- ered characteristic of syphilis, they may well be retained. On account of the inaccessibility of the spirochetal in the central nervous system to sal- rersan introduced into the circulation, Bvrift and Elli» have recommended administering this remedy intravenously, one hour later, bleeding, cent ri filiating the blood, beating the scrum for one hour to 5fi° ('., letting stand on Ice overnight, making a t n per cent, dilution of this serum 212 LECTURE XIII with physiological salt solution, and after preliminary lumbar puncture and withdrawal of a corresponding amount of fluid, injecting into the spinal canal 15 to 330 cc of this "salvarsanized serum." It is thought that by this means the salvarsan in the blood may be brought directly into the ventricles and lymph spaces, and can attack the spirochaetae there harbored. While this method has been used to a considerable extent in the United States, it has not entirely fulfilled the hopes attached to it, and our experience with it is as yet insufficient to warrant a positive opinion as to its value. The direct injection of salvarsan into the spinal canal has been tried, but some serious results have followed this method of administration, and its tech- nique is as yet insufficiently developed, hence it can hardly be recommended at the present time. — Translator. LECTURE XIV The Syphilogenic Diseases of the Central Nervous System Gentlemen : Now that in the last three lectures we have studied the two most typical representatives of the metasyphilitic nervous diseases, we will take up to-day the consideration of syphilogenic affections, which in pathologico- anatomical and symptomatological relation present less uniformity. These are, 1, the so-called syphilogenic combined system diseases and 2, cerebro- spinal syphilis. C. Syphilogenic Combined System Diseases In Lecture VIII we have seen in Friedreich's disease a representative of the so-called combined system diseases, but put off the general consideration of these in no way frequent disease forms until later. It is now time to enter into a short discussion of this nosological conception. While in spinal hereditary ataxia there is the combination of a purely endogenically caused degeneration in the different sets of fiber systems of the posterior and lateral columns of the spinal cord, similar lesions can occur from other causes. First from the action of poisons or toxines which are elective, but which affect at the same time several categories of long tracts and which lead to the progressive degeneration of the neurones affected. Next, as par- ticularly Nonne and Friind have shown, disseminated focal diseases, starting from the vascular apparatus, which is situated within the areas of definite fiber systems (for example, the pyramidal tracts, or the columns of Goll) which in consequence of the descending or ascending degeneration of these tracts so caused, can furnish the anatomical substratum for combined system diseases. On microscopical examination this pathogenetic subvariety can usually be ili^ tinguished without greal difficulty from tin- other varieties by the lack of sharp limitation of the sclerosed areas, as well by the marked vascular alterations; Bymptomatologically, however^the result can he identical. Finally, it may he emphasized that the greater susceptibility of the pyramidal tracts and the pos- terior columns to ischemic damage makes an apparently elective affection of just these tracts, even in more diffuse vascular lesions, comprehensible. In these different ways the combined system diseases which have been observed in the following general diseases are explainable: Chronic alcoholism, chronic lead poisoning, diabetes, carcinomatosis, pellagra (chronic intoxication by spoiled corn), lathyrism (intoxication from chickpeas ) . chronic nephritis, pernicious 218 214 LECTURE XIV anemia, leucemia, sepsis. In practical importance these conditions are far in- ferior to the syphilogenic combined system diseases with which they agree en- tirely from a symptomatological point of view. The description of these last may hence serve as paradigm for the whole group. Let us add that in the production of syphilogenic combined system diseases, in part simple degenerative processes as in tabes, in part tertiary luetic en- (darteritic processes, are in action. The disease pictures under consideration are hence to be considered partly as metasyphilitic, partly as purely syphilitic; their description, thus, conducts us in a natural manner from tabes and paresis to cerebro-spinal syphilis. The combined destruction of centripetal systems (affection of the pos- terior column) and of the most important centrifugal fiber complexes (Tractus cortico-spinalis) has as a consequence, that clinically the combination of atactic and hypesthetic phenomena predominates. Now, however, as we know from - Fig. 67. Changes in the Spinal Cord in Syphilitic Spinal Paralysis. Weipert-Pa! Stain. our former descriptions, elimination of the pyramidal tracts has as its conse- quence hypertonia and hyperreflexia, that of the posterior fasciculi, hypotonia and hyporeflexia. Hence, in combined lesions the influence of diametrically opposed tendencies make themselves felt. The result depends upon the greater or less intensity of the process in the different parts affected. If affection of the pyramids predominates (see Fig. 67), there results the symptom-complex of spastic-atactic paraplegia with sensory disturbances and we call such cases, after Erb's recommendation, syphilitic spinal paralysis. The sensory disturbances are often very slight. As I have shown, they are, as a rule, most easily recognizable on testing vibration sense with a tuning-fork. Besides this, lancinating pains, bladder disturbances, reflex pupillary rigidity and optic atrophy are sometimes observed, just as in tabes dorsalis. If, on the other hand, the pyramidal affection is less than that of the sen- sory tracts (see Fig. 68) a syndrome characterized by ataxia and arcflexia with motor weakness, is produced, and besides this, phenomena pathognomonic for the pyramidal affection (chiefly Babin&ki's symptom, more rarely that of SYPHILOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 215 Oppenheim or of Mendel-Bechterew) usually arc present. Also, in this case, the occurrence of lancinating pains, bladder disturbances, the Argyll-Robertson pupil and optic atrophy are not rare, and we speak of "combined tabes." Pure cases of syphilitic spinal paralysis and of combined tabes are rarer than their accompaniment with symptoms that belong to cerebro-spinal syphilis. The course of both forms is slowly progressive, in combined tabes on the average more rapid than in syphilitic spinal paralysis. In it also, the prog- nosis is clouded by the not so rare development in addition of general paresis and also by the fact that specific treatment can accomplish much less than in syphilitic spinal paralysis. In this last, indeed, which begins often in the first years after luetic infection, the therapeutic outlook is relatively good. If Fig. <>8. The Combined Talus. Weigert-Pal Stain. the treatment is begun very early complete recovery is possible; if later, a definite standstill can at least be obtained, and to this the disease may spon- taneously tend. As an early symptom the so-called "spinal intermittent limp- ing" is of great importance; a description of this I will take up in the next lecture when speaking of arteriosclerotic diseases of the spinal cord. We will consider treatment at the end of this lecture. D. Cerebro-Spinal Syphilis In the great majority of cases in which tertiary syphilitic processes de- velop in tin- central nervous system they appear simultaneously in the brain and in the spinal cord. This is explained by the facts that the syphilitic lesions of the nerve centers do not arise from the parenchyma proper, the ganglion cells and nerve fibers, but are partly of meningeal, partly of vascular origin, 216 LECTURE XIV and that both the meninges and the vessels of the brain and spinal cord present a unique and connected whole. Let us enumerate now the chief alterations which present the anatomical substratum of cerebro-spinal syphilis. 1. The gumma or syphilitic granulation tumor occurs but rarely as a gross and solitary structure, and then on the convexity of the cerebrum; its place of origin is then usually the dura mater and the symptomatology agrees with that of true neoplasms of corresponding size and analogous location. Much more frequent is the formation of small and multiple gummatous nodules which prefer the basal portions of the brain and usually are situated in the pia- arachnoid or on its vessels. Where they are particularly numerous we speak of "gummatous basilar meningitis." Also the gummata developing on the spinal cord are almost always multiple and originate in the meninges ; they are fre- quently miliary, so that on microscopical examination they may be mistaken for tuberculous nodules. 2. Syphilitic endarteritis, a disease of the vessels whose chief characteristic consists in a progressive thickening of the intima by a new formed connective tissue rich in cells. It can lead to complete obliteration of the lumen of the vessel. As a rarity the deposit of minute gummata in the new formed endar- teritic tissue has been described. Syphilitic endarteritis is ubiquitous in the central nervous system; it can be found as well in the meninges or on the sur- face of the brain and spinal cord as in their interior. 3. Meningoencephalitis and meningo-mvelitis syphilitica. At the basis of these processes lies a diffuse infiltration of the pia-arachnoid with small cells, which in later stages, leads to considerable connective tissue thickening and infiltration of this with a gelatinous deposit. Gummatous deposits, on the other hand, are absent in typical cases. In the altered meningeal region, how- ever, the vessels without exception undergo endarteritic and periarteritic changes ; their passing into the superficial portions of the central nervous system furnishes to the infiltration process a port of entry for propagation in the cortex of the brain or in the white matter of the spinal cord. In the first instance the convexity of the hemispheres, in the last, the posterior part of the periphery of the spinal cord, are seats of £>redilection. Symptomatology a. Cerebral Symptoms. — A particularly constant symptom is headache which usually has a paroxysmal exacerbating character and occurs with the greatest severity, particularly at night or early in the morning. At the height of its intensity it assumes, usually, a boring and hammering character; during the remissions it is usually described as a dull and continuous feeling of pressure. At the height of the paroxysm there is not very rarely nausea or vomiting, on which account on superficial diagnosis it is occasionally mis- taken for migraine. The comparison of clinical observations with autopsy findings justifies us in considering this headache as an expression of basilar- meningitic processes. .Only when this last assumes a high degree of intensity is syphilitic headache accompanied by stiff neck and rise of temperature. Circumscribed sensitiveness to tapping or to pressure over the skull is scarcely SYPHILOGENTC DISEASES OF CENTRAL NERVOUS SYSTEM 217 ever to be found in syphilitic basal meningitis ; where it is found, a suspicion that the convexity of the cerebrum is affected is justified. An important role in the picture of cerebral syphilis is played, further, by the eye symptoms, which often involve the optic nerve, since the region of the chiasma presents a point of predilection for the localization of tertiary products. Ophthalmoscopic examination shows different pictures, among which papilledema and optic neuritis depending upon increased intracranial pressure are most important. We will consider the first when speaking of brain tumors (Lecture XIX) ; the last — whose end result is neuritic optic atrophy — has already been considered in Lecture II among diseases of the cranial nerves. Simple, degenerative optic atrophy is sometimes found in cerebral syphilis and in a form entirely agreeing with what is observed in tabes and paresis; its occurrence always points to the (quite frequent indeed) com- bination of syphilitic and mctasyphilitic processes. Optic neuritis can occur unilaterally, papilledema and degenerative atrophy are practically always bilateral, indeed, often with considerable difference in intensity between right and left. Considerably more frequently than the optic nerve, however, the oculomotor is affected and indeed, now unilaterally, now bilaterally, almost never totally, but, as a rule, with avoidance of one or several of the muscles supplied by it. Particularly regularly and particularly early, occurs paralysis of the levator palpebrae muscle, so that a large number of these patients are brought to the physician, usually to an ophthalmologist, on account of ptosis as the first symptom of their brain syphilis. Rapid change in the picture of the eye muscle paralysis (it occurs also in the trochlear and the abducens, though considerably less frequently) is very characteristic of brain syphilis.'" As to syphilitic affection of the internal branches of the oculomotor, paralysis of the pupils, as well as its combination with paralysis of accommodation, is found (ophthalmoplegia interna), also, finally, the reflex pupillary rigidity already described (Argyll-Robertson pupil, see page 183). The pupillary* paralysis naturally manifests itself by mydriasis and absence of reaction of the pupil for light and convergence. Of other luetic cranial nerve symptoms the affections of the trigeminus, facial, auditory, glosso-pharyngeal, vagus, and hypoglossus should he men- tioned. Tin 1 trigeminus manifests its involvement in the disease process usually in the form of neuralgic pains (I would recall to you, for example, the bilateral Seeligmiiller's neuralgia of the auriculo-temporal nerves mentioned in Lecture II (page 47), as characteristic of syphilis), rarely by sensory defect symptoms or loss of the corneal reflex, only very exceptionally in the form of trophic dis- turbances, as, for example, herpes zoster, corneal ulcer, etc. On the part of the facial, paralyses of peripheral type are sometimes observed and in luetic basilar meningitis, indeed, not so very rarely, as the otherwise excessively in- frequenl facial diplegia. The eighth pair of nerves are very frequently affected, particularly the cochlear (tinnitus auriinn, nerve deafness) here and there also the vest il nilar branches ( vevi igo, Meniere's attacks) ; as to t be symptomatology, .i page •'!•'! et seq. The vagus and glosso-pharyngeal roots are much more rarely affected, and then almost always both together; their involvemenl mani fests itself in attacks of cough, difficulty in swallowing, paralysis of I be vocal 218 LECTURE XIV cord, the (also rare) basal syphilitic lesion of the hypoglossus, in hemi-atrophy of the tongue. In the vast majority of cases the syphilitic affections of the cranial nerves are observed not isolated, but in varied combinations. The peculiarity of rapid change in the symptom picture applies not only to the syphilitic eye muscle paralyses as emphasized above, but also to the other basal phenomena. Brain syphilis can further lead to paralyses in the extremities, both from basal meningitis and particularly from endarteritic changes. This occurs almost always as one of the varieties of hemiplegia. We will postpone the considera- tion of the particularities of these important syndromes to a future lecture in which an adequate presentation of the subject of half-sided paralyses of cerebral origin will be given (Lecture XVII). It will only be remarked here that the paralytic symptoms produced by the pressure of meningitic exudates upon the crus cerebri, the pons, and the medulla are differentiated by their less intensity, and particularly by their fugacity, from the prognostic-ally decidedly less favorable paralyses of vascular origin. Endarteritic processes in the branches of the basal and vertebral arteries can lead to bulbar paralytic disease pictures (see Lecture ATI, page 120). As to gumma and to gummatous meningitis of the cerebral convexity, with them disease processes occur which greatly resemble those which we will study when speaking of brain tumors ; indeed, many gummata clinically should come under the classification of tumor. For syphilis speaks in general the slight dev< lopment of papilledema, which indeed, in gummata of considerable size, is sometimes entirely absent; further, a striking variation in the intensity of the symptoms, a rapid alternation of improvement and retrogression. In general, much depends upon the localization of the syphilitic process. For foci which are located in the motor area of the cortex, the combination of the so- called "Jacksonian" or "cortical" attacks (see Lecture XIX) with relatively limited unilateral paralyses (monoplegia facialis, faciobrachialis, etc.) is typi- cal; left-sided foci often lead also to aphasic disturbances (see Lecture XVIII). Psychical disturbances point nearly always to diffuse encephalitic processes, particularly to extensive disease of the brain arteries. They are not common in gummata, still more rare in syphilitic meningitis (apart from the general clouding of the sensorium in advanced cases). Upon the basis of diffuse syphi- litic endarteritis, disease pictures, which may deceptively resemble dementia paralytica yet are distinguished from it by decidedly better prognosis, often occur. As chief differential diagnostic criteria for distinguishing them from true progressive paralysis, Kraepelin and Obersteiner give the absence of general paretic symptoms, while on the other hand focal symptoms may occur very early and are much more obstinate, the absence, or much less marked development of disturbances of speech and handwriting ; further, disturbances of memory and attention keep more in the background, reduction of sensitiveness to pain is not present ; particularly striking is the great frequency of hallucinations of hearing. Against a true syphilitic brain disease, but only with caution, a great length of interval between infection and outbreak of the disease can be utilized. All these criteria I consider of very questionable value and I could refer to SYPHILOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 219 several cases in which by psychiatric authorities, upon the basis of the psychical syndrome, a luetic psychosis was diagnosed which later, however, showed them- selves to be true paresis. Wrong diagnoses can never be entirely avoided. We are most justified in assuming syphilitic pseudo-paralysis when the somatic symptoms of brain syphilis, particularly the cranial nerve paralyses, are very marked, and a decided alternation of symptoms is present, mainly, however, when antisyphilitic treatment works promptly and strikingly. b. Spinal Symptoms. — Syphilitic meningomyelitis and spinal endarteritis, the syphilitic granulomata of the spinal cord and its membranes, lead to clinical pictures in which the meningeal and the medullary components of the anatomical substratum find expression in the two already well known syndromes, of radicu- lar pains and partial transverse lesion ; in the last the paralytic symptoms appear now symmetrical, that is, in paraplegic form, again unilateral in the form of the Brown-Sequard symptom complex. As to the rest, however, the combination of flaccid and spastic, sensory and motor, irritative and defect phenomena, varies according to the longitudinal and transverse extent of the anatomical lesions, within quite wide boundaries, and besides this, in the course of the disease very frequently a tendency to variation in intensity, decided remissions and renewed progressive advances are noticeable. This symptomatological lability can render a differential diagnosis from multiple sclerosis considerably more difficult. In general the intensity and the frequency of bladder troubles is much greater in syphilitic processes than in multiple sclerosis. This applies even more to rectal troubles which are exces- sively rare in the latter. What aid we are justified in expecting from examina- tion of the spinal fluid in cerebro-spinal syphilis has been already thoroughly considered in Lecture XII. That the spinal symptoms of cerebro-spinal syphilis as we have just indi- cated them, cannot be sharply separated from those of the "pseudo-systematic" forms of "syphilitic spinal paralysis" should be clear to you without further remark; also as compared to tabes and progressive paralysis, the boundaries ■in frequently bridged over by the combination of tertiary and metasyphilitic processes. Prognosis and Treatment The prognosis of cerebro-spinal syphilis is, presuming timely and thorough specific treatment, decidedly better than that of tabes and paresis, indeed, it BOH he said, better than the prognosis of the other organic brain and spinal cord affections. The more, however, the cerebral symptoms occupy the foreground, the more serious the prognosis. A syphilis located principally in the brain, sometimes defies the most energetic therapy and leads in spite of all our efforts to death or to chronic invalidism. Often we must content ourselves with incomplete recovery, or "recovery with defect." The best outlook is given by the spinal syndrome, where some- times cures bordering on the marvelous can be recorded. I have already men- tioned the surprisingly rapid cure of a complete syphilitic paraplegia as a result of treatment by salvarsan. Energetic mercurial treatment can, though ■MB rapid, effeci just as striking results in cerebro-spinal syphilis. With 220 LECTURE XIV all recognition of the powerful agent which we have in salvarsan, this must be expressly emphasized, as Oppcnhtim, Nonne, Sanger and others have done. Personally, my position is that in great development of cerebral symptoms I still hesitate to recommend its use instead of that of mercury, since the aggra- vation of the symptoms as they have been observed in such cases, after the use of salvarsan by Mlngazzini and others (the risk of them is small, it is true) need not be feared in the employment of the less heroic but nevertheless actively efficient mercurial treatment. Where, however, salvarsan is to be given (often in response to the direct wish of the patient, in which event it is advisable after Oppenheim's recommendation, to insist that he assume responsibility for any untoward results before beginning the treatment), it should not be used in insufficient doses, since the "neurcrezidive" observed after arsenobenzol injec- tions (they are usually paralyses of the cranial nerves) are probably not due to direct toxic action of the remedy but are of true syphilitic nature and their occurrence is favored by the "neurotropy" of the salvarsan, but only with the provision that the spirocha'tae after the injection still have sufficient power to cause organic lesions. A good rule for salvarsan treatment is presented in the scheme of Bruno Block: a. Intravenous salvarsan injection (0.6 grm in adults. In delicate persons, in children, etc., correspondingly less), b. 5 to 7 days later the first intramuscular salvarsan injection of 0.3 grm. c. 14 to 20 days after the intravenous injection a seconr intramuscular injection of 0.3 grm. d. 4 to 5 weeks after the first intravenc 5 injection, finally, a second intravenous injection. With regard to the use of mercury many neurologists still give preference to the old inunction treatment. However true it is that this latter properly carried out and sufficiently frequently repeated at graduated intervals can accomplish most excellent results, it is certain that exactly the same thing can be accomplished with the much more convenient and cleanly intramuscular injections, on which account I use these almost exclusively. Where there is danger in delay and very rapid mercurialization is necessary I usually begin with daily injections of a soluble salt of mercury, for example, the biniodide according to the formula given on page 210; instead of the daily dose of 0.02, larger doses (0.03 to 0.04) can be given, indeed, with risk of acute mercurial intoxication. This we can arrest, however, when using the soluble salts of mercury, by stopping the injections, which we cannot do with the still more active but insoluble calomel injections (in 40 per cent, emulsion) on which account this last method is best left to the experienced syphilologist. After 10 days the injections of biniodide of mercury are intermitted for several days. A series of 20 to 30 injections constitute a "cure." In later treatment under less pressing indications, injections of salicylate or thymoloacctate of mercury (in 10 per cent, emulsion with paraffine oil) should be preferred to the soluble salts, since on account of its more protracted absorption the injections are at longer intervals, hence more convenient and less expensive. Twelve injections of 1 cc each at intervals of from 3 to 4 days constitute a normal "cure" for a strong adult ; it is well, however, to test the tolerance at first by giving smaller doses. That in all mercurial injections the most careful asepsis and proper technique is necessary goes without saying. SYPHIEOGENIC DISEASES OF CENTRAL NERVOUS SYSTEM 221 Place of injection: External upper quadrant of the gluteal region; with avoidance of vessels and regular examination of the urine for albumin should never be neglected, particularly when large doses are used. The erroneous idea that cerebro-spinal syphilis, since it is "tertiary," is to be cured with iodide of potassium alone, must be decidedly opposed. Iodide of potassium, however, is to be used and in not too small doses along with mercury and eventually, salvarsan. I increase it usually to i to 6 grms a day ; many syphilologists go considerabl}' higher (to 15 grm). After each 20 days it is intermitted for about 10 days. If iodism begins, a solution of 3 grms each of sulphanilic acid and bicarbonate of soda in 200 cc water is given. As substitutes for iodide of potassium (over which iodide of sodium has no advan- tages) when there are symptoms of intolerance, different organic iodine prep- arations come under consideration, for example, sajodin, iodgline, iodtropon, iodstarin, lipoiodin, etc. They are all, however, less active with the exception of intramuscular injections of from 10 to 20 cc of 25 per cent, sajodin, which repeated at intervals of from 3 to 4 days sometimes has striking effect. Internal administration of mercury is at most to be made use of for later mild "intercures" ; under no circumstances can it replace injection or inunction cures. I would mention in this connection mercurous tannate 0.05 (gr. %) in pill form t.i.d. In conclusion a word 8 to the relations between the Wassermann reaction and the treatment of cerebi rspinal syphilis; it occurs not infrequently that in cases where this reaction is positive, even the most energetic specific treatment cannot change it into negative. This, however, in no way clouds the prognosis, since in spite of this, recovery can be obtained and preserved. In order that such patients do not become unhappy syphilophobes, we must practice a "pious fraud" and not inform them of the continued anomaly of their blood serum, lest they believe themselves continually threatened with a recurrence and doomed to life-long use of mercury. LECTURE XV Arteriosclerosis of the Nerve Centers Gentlemen : In to-day's lecture we will consider neither the mechanical destruction which the nervous tissue suffers in consequence of the rupture of a vessel altered by arteriosclerosis, nor the extended foci of softening which the atheromatous closure of a large artery produces. These gross and striking results of arteriosclerosis of the central nervous system will be described at length in a later lecture. To-day I will take up those phenomena which are explainable on the ground of diffuse disturbances of nutrition in the brain and spinal cord, consequent upon alteration of their vessels, as well as certain paroxysmal or intermittent nervous disturbances, whose comparison with other arteriosclerotic symptom complexes (for example, angina pectoris or intermittent limping) imposes itself upon us. Further, I will unfold before you. different clinical pictures which, in spite of their relative frequency, are still too little considered and which stand in relationship with special peculiar modalities of arteriosclerotic tissue destruction. Symptomatology Of special practical importance is acquaintance with that category of dis- ease manifestations which we can unite under the name of arteriosclerotic pseudo-neurasthenia. In the proper estimation and management of just this condition, of which Windscheid, Erlenmeyer and others have given us good descriptions, we will find the central point of prophylaxis against all destruc- tive nerve lesions of arteriosclerotic origin, for example, also against the cere- bral hemorrhages and foci of softening already mentioned. It is plainly the very first beginning of vascular degeneration in the central nervous system. The patients — usually men between 40 and 55 years old — generally come under treatment on account of headache. This is usually a feeling of pres- sure and heaviness, particularly about the forehead which is present on rising and increases during the day to a considerable, often to a distressing, intensity. Often the headache increases particularly on mental effort, sometimes even on meditation, which the French author Josne characterizes as "le signe de la pensee douloureuse" (symptom of painful thinking). There are also cases in which headache is absent during complete rest but is produced by the slight- est mental or bodily effort. This headache can present a great similarity to that produced by anomalies of refraction, or by moderate astigmatism in people beyond 40 years old — when the power of accommodation decreases. It will be well in such patients not to neglect the eye condition. Further, there is frequent complaint of vertigo; sometimes this designa- 222 ARTERIOSCLEROSIS OF THE NERVE CENTERS 223 tion is applicable (namely for sensations which the patients experience in the morning upon sitting up in bed or upon rising) ; often, however, under this so frequently misused expression are understood only sudden feelings of weak- ness or momentary darkening of the visual field, or finally, feelings of oppres- sion or anxiety. Only rarely do we encounter true rotary vertigo to the ves- tibular nature of which an accompanying tinnitus can point, although the otological examination may disclose no middle ear or labyrinthine affection. A noteworthy variety of arteriosclerotic vertigo which as far as I know was first described by Homburger, I have heard occasionally sketched; when the patient lies in bed he suffers from the painful uncertainty of not knowing exactly how he is lying — whether upon his back or upon his side — and cannot describe the location of his bed; with this a distressing anxiety and oppression may come over him and may be accompanied with a sensation of a dull humming inside of his skull. Almost always, also, there are sleep disturbances, which, however, scarcely ever lead to a severe and obstinate agrypnia, as for example, in the prodromal stage of general paresis. Usually it is only considerable difficulty in going to sleep, restless sleep and early awakening with a sense of weight in the head. Rather rare and somewhat limited to those of more advanced age among the arteriosclerotics, is the combination of ability to sleep well by day with nocturnal sleeplessness and jactitation. This paradoxical phenomenon has been called "sleep inversion." You will often note, also, that in arterio- sclerotics the intensity of the sleep disturbances varies from day to day. Ability to work suffers usually in this manner, that the patient can follow his ordinary occupation, but contrary to what was formerly the case, finds it a great exertion and is no longer able to take up new tasks. Mental con- centration is particularly difficult, which is brought to the realization of the patient on reading a paper, in keeping his accounts, in hearing a lecture or sermon, etc. The mood is usually fretful, irritable, morose, and besides this there often makes itself felt a pessimism due to the feeling of reduced capacity which first concerns the health and economic future of the patient, but soon, however, disturbs his judgment of all relationships. Finally, many of these patients complain of abnormal sensations, usually in the form of formication, prickling, going to sleep of the extremities. These troubles present a great similarity to neurasthenic symptom-com- plexes (see below. Lecture XXVII). The differential diagnosis from neuras- thenia is sometimes made easier for us by going thoroughly into the history. No neurasthenia occurs without some definite etiological preceding conditions. In the constitutional form of this disease its congenital degenerative basis is clear from the fad that its first beginnings can be traced back into early youth; in the acquired, accidental form, on the other hand, «c can regularly make definite exogenic causal factors responsible, among which there is usually a combination of nervous fatigue in any direction with the chronic action of disagreeable emotions. A nervosity, however, which begins apparently without cause in a person of "arteriosclerotic age" of previously healthy nervous sys- tem, imperatively demands the close investigation of the vascular apparatus. And even in genuine inveterate neurasthenics it Ihis critical ace, arteriosclerosis 224 LECTURE XV should conscientiously be sought for ; it is indeed, a fact long known, that neurasthenics — mainly those with vasomotor cardiac symptoms — are predis- posed to arteriosclerosis. Mutual relations between both disease conditions has, a priori, much probability. I have pointed out that cases of neurasthenia with cardiac and vascular symptoms ( for example, tachycardia, dermograph- ism, etc.) are characterized very often by abnormally high blood pressure (see Lecture XXVII). Now, however, cardio-vascular hypertension, as has been experimentally recognized, plays a role in favoring the occurrence of atheroma- tous heart lesions. I cannot refrain from mentioning at this opportunity the recognition by the American physiologist Cannon that in animals psychical excitement (in the cat, for example, coming into contact with a dog) produces an adrenal- inema. This fact appears to me to throw a new light upon the pathogenetic role which in the etiology of arteriosclerosis may be attributed to the con- tinuous increased emotivity common to all neurasthenics. Now adrenaline acts injuriously upon the vessel walls, both chemically and by raising the blood pressure. Finally, a still more direct connection between the nervous system and vascular degeneration has been experimentally rendered probable ; for ex- ample, Manouclian has shown, that through the destruction of definite nerve twigs entering the vessel walls, sclerotic plaques may be produced in a corre- sponding situation. To return to the clinical side, if there is a suspicion of arteriosclerotic ner- vosity in a patient, we must naturally confirm the diagnosis by recognizing the vascular affection. Here I would warn you above everything against depending upon the palpation of the peripheral arteries as is so often done. The clinic and the autopsy table in agreement, teach us that between the condition of the peripheral arteries and that of the brain and spinal cord vessels, a paradoxical incongruence often exists. I could mention many cases of cerebral hemorrhage and encephalomalacia with severe sclerotic alterations of the brain arteries, in which in the radial, the dorsalis pedis, the carotid, the brachial, and even the temporal, there was no perceptible tortuosity or harden- ing of the vessels. Nevertheless, I would add that (when all arteries accessible to palpation are examined) negative findings in this respect are not so very frequent. As to the rest, in arteriosclerotic pseudo-neurasthenia we can pretty well count on registering further stigmata of vascular disease in these patients, sometimes indeed, in considerable number. So, for example, alteration of the size of the heart, especially hypertrophy of the left ventricle, accentuation of the second, impurity of the first aortic sound. Slight albuminuria is usually to be found if the urine is repeatedly examined ; and also in urine free from albumin, hyaline and granular casts are occasionally observed. Of importance, also, is the recognition of slight oedema over the ankles in the evening, provided that it is not explainable through heart weak- ness, nephritis, or varicose veins. Sometimes there is a tendency to nose bleed. As to stenocardiac attacks proper, or cardiac asthma, it is, as a rule, not necessary to inquire, as these conditions are usually spontaneously mentioned by the patient ; still they occur sometimes in slight or rudimentary form, ARTERIOSCLEROSIS OF THE NERVE CENTERS 225 scarcely observed by him; this applies, namely, to the "respiratory fatigue"' of the French, which occurs on moderate bodily exertion. It is well, also, to measure the blood pressure, although its increase which according to Broedel occurs in about 65 per cent, of cases of Arteriosclerosis, according to Romberg and others, is no symptom of the vascular disease itself, but of the interstitial nephritis which so often accompanies this last. Of late it has been endeavored by Kurt Mendel, in disease pictures which occur without any external cause, in previously healthy men between 45 and 50 years of age and which closely correspond to the description which I have given you of arteriosclerotic pseudo-neurasthenia, in which, however (if we neglect congestions of the head and paroxysmal palpitation), cardiac vascular anomalies are absent, to refer these cases as "molimina climacteria virilia" to alterations of the inner secretion of the genital glands and to separate them from incipient arteriosclerosis of the nerve centers. We will turn now to a group of disturbances of considerable intensity but of fleeting character which we sometimes see appear upon the basis of arterio- sclerotic pseudo-neurasthenia and which demand our close attention. They inform us, namely, of an already high degree of intensity of the vascular dis- ease and represent only too often (as the further course of such cases teaches us) the forerunners of irreparable organic lesions of the nervous system. I would mention first, severe pains which occur in attacks, are characterized by great intensity, and are located both on the trunk, in the limbs and in the region of the trigeminus. They are mainly considered as neuralgias, although I believe, incorrectly very often. The absence of pressure points along the nerves in question, the boring, lancinating, constricting character of these attacks, recalling tabes dorsalis, makes it appear probable to me that they are due to vascular spasms in the central nervous organs or in their posterior roots. Nothnagel and Huchard long ago emphasized the role in the clinical picture of arteriosclerosis, which vascular spasm occurring in attacks, played alongside of the permanent vascular lesions, and the similarity of these painful crises of arteriosclerotics to angina pectoris and to arteriosclerotic colic, de- cidedly impresses itself. Still further analogies, however, exist between certain intermittent disturbances in the motor functions, which are occasionally found in cerebro-spinal arteriosclerosis and the interesting phenomenon which is known as "intermittent limping." This complaint ("Claudicatio intermittens," "Dysbasia angiosclerotic in- termittens") often presents a prodromal stage of the so-called spontaneous gangrene of the lower extremities. It is caused by arteriosclerosis of the leg arteries, though functional causes also play a role. While namely, on walk- ing, the movements of such patients are first normal, painless and unhindered, after :i certain time — this can be in one case, a few minutes, in another a half or even three-quarters of an hour — there occurs an extreme weak- ness of the lower extremities with cramplike sensation, which soon makes further progression impossible f<>r the patient. At the same time it can be remarked how single toes, single portions of the skin of the feet, alter their color under the eyes of the observer, now deathly pale, now livid, again cyanotic — a plain evidence that it is not exclusively the expression of blood supply 226 LECTURE XV insufficient for the locomotor requirements, but that a vasomotor spasm is also in action — after a short rest, however, all the trouble has disappeared again and the patient can move in a normal manner ; indeed, only after about the same time as formerly, to be again attacked by the trouble and to be compelled to rest. And so the same thing is repeated upon all attempts at walking; with the total duration of the locomotor effort, the time of unhindered walk- ing usually becoming continually shorter. Dysbasia angiosclerotica inter- mittens, occurs very rarely with us ; in Poland and Russia, however, it is strik- ingly frequent, which may have a connection with the very cold climate. There is in that region an apparent predisposition among the Jewish race which is not the case in our neighborhood ( of 5 cases which I have seen, not one was a Jew). Excessive smoking certainly plays an important etiological role. The treatment — apart from the general treatment of arteriosclerosis — falls in with that of the vasomotor neurotic forms of intermittent limping (see Lecture XXV). There occurs now in arteriosclerosis of the central nervous system a dis- turbance which appears so like the classical picture of intermittent claudication introduced into the nosology by Charcot and Erb, that Dcjerine denominates it as the spinal variety of this disease, an "intermittent claudication of the spinal cord." I have seen two exceedingly instructive cases of this. In dis- tinction from the peripheral form, all vasomotor disturbance in the lower extremities is absent and both arteries on the feet (dorsalis pedis and tibialis posticus) are to be felt normal. With the beginning of the limping, there occur the following symptoms characteristic of it: an increase in the patellar and Achilles reflexes, often a decided ankle clonus, and sometimes also, the Babinsky reflex, as clinical corollaries of an ischemic condition of the pyramidal tracts. Somewhat more frequently than in arteriosclerosis of the spinal cord — it can be said in passing — spinal intermittent limping has been observed upon a basis of syphilitic endarteritis. Along with the just described paretic paroxysms of spinal origin we can observe those of plainly cerebral origin, although much less frequently, in which, however, the cooperation of muscular effort as an exciting cause is less plain, or indeed, cannot be recognized. I have in mind particularly the tem- porary paresis of an arm or leg or even of both extremities on one side of the body which usually greatly frighten the patient, but after a few hours, or even minutes, entirely disappear. Still more interesting is the temporary motor aphasia, of which I might mention three observations: in one, the disturbance lasted only a few minutes, in the others, from 1.5 to 20 minutes. One of these last cases seemed to illustrate the influence of functional use, in that the woman affected afterward explained that on the day on which it occurred she hail felt very much fatigued from long talking to visitors. All these transitory phenomena, however striking their method of occur- rence may be, always stand on the border between the functional and the organic. They can be considered as the original expression of Grassefs has it, "avertissenients sans frais" (free warnings). The disturbances, however, to which we will now turn, depend upon material alterations which, although not very extensive, are nevertheless of far-reaching importance. ARTERIOSCLEROSIS OF THE NERVE CENTERS 227 I cannot go any farther into arteriosclerotic diseases of the spinal cord (as a forerunner of which we have become acquainted with spinal intermittent claudication) but will only state that a degeneration in the region of the lateral columns due to arterial ischemia' in consequence of atheromatous oblit- eration of vessels can produce the symptom-complex of spastic spinal paralysis. These cases which have been isolated by Dcmange, Oppenheim and others as senile spastic paraplegia, affect usually only the lower extremities, very rarely the arms also. A more extended affection of the vascular apparatus of the .-pinal cord can also produce sensory disturbances and sphincter paralysis. These findings are much less frequent than the extensive symptoms which small, unapparent losses of substance due to arteriosclerosis in the brain can produce, thanks to the multiplicity which, as a rule, characterizes their occur- rence. These are the so-called "Lacuna 1 " ("lacunes de desintegration cere- brate," "etat lacunaire du cerveau") first thoroughly studied by Pierre Metric in Paris. From miliary, to the size of a pea, of irregular outline, these losses of substance pervade in greater or less number circumscribed areas of the cerebrum, the interbrain, ami the midbrain. In the middle of each one of these small spaces located close together, we notice upon microscopical exam- ination an arteriosclerotic but still pervious vessel; they cannot hence be con- sidered as small areas of softening due to the closure of vessels. Rather have the newest histological investigations shown that inflammatory processes of the adventitia and of the peri-arterial lymph spaces are responsible for the destruction of the surrounding brain tissue. The status lacunaris of the brain is properly to be distinguished from the so-called "status cribrosus" of Durand-Fardel, which results from dilatations of the peri-vascular lymph sheaths, while it is pathogcnieally more nearly re- lated to a lesion of the cerebral cortex described by Marie, Alzheimer and others, which gives to this last a worm-eaten appearance ("etat vermoulu") since it is dui' to the destruction of cortical substance about diseased arteries. On the other band, the "cerebral porosis" of certain pathological anatomists (also called "cystic degeneration" or "'etat de fromage de Gruyere") is nothing else but a post-mortem artefact produced by the bacillus serogenes capsulatus, by tin' aid of which these changes can be experimentally produced also. A special variety of I be hemiplegia of old age depends, too, upon arterio- sclerotic formation of lacuna'. This lacunar hemiplegia (to anticipate our later remarks, Lecture XVII) can usually be distinguished without difficulty from the hemorrhagic, thrombotic and embolic hemiplegias by a number of clinical criteria. These criteria are particularly in tin' character and course of the attack, which almost never presents the picture of a true apoplectic seizure, namely, it' it does not occur at nighl (which it often does) it usually runs its course with retained consciousness and at most, some vertigo and a temporary confusion are its accompaniments. In the rare cases, however, ill which the hemiplegic paralysis benins violently (this is the case when the lacuna' involve the internal capsule) the loss of consciousness is of very short duration— lasts less than an hour. The resulting hemiplegia is. further, usually of little intensity and markedly transitory character. Although it also leads to exaggeration of reflexes, it never causes contractures, but more or less 228 LECTURE XV completely disappears after hours, clays or weeks. (A longer duration of the hemiplegic symptoms again points to the seat in the capsule of the tissue de- fects.) The lower extremities are usually longest affected, since a certain regularity in the distribution of the paralytic symptoms is not to be mistaken. Among these, most important is the absence of aphasic symptoms, while there may be some disarthria; further, we miss regularly hemianopsia, conjugate Status Laeunaris Cerebri. Typical attitude and "demarche a. petit pas." deviation, and appreciable disturbances of sensibility, while quite frequently the facial nerve is unaffected. Ankle clonus and the Babmsky and Oppcnlieim phenomena can often be observed ; on the other hand the abdominal reflexes are scarcely ever disturbed. Now, however, after the hemiplegic symptoms have disappeared with remarkable rapidity and completeness, a change which gives to such patients a quite characteristic attitude progressively sets in. While, in spite of persistent anomalies of reflexes, the paretic symptoms at ARTERIOSCLEROSIS OF THE NERVE CENTERS 229 most manifest themselves in the fine movements, for example, in buttoning the clothes, the gait takes on an exceedingly typical character (Fig. 69). In the yards and gardens of asylums for old people and almshouses, such patients can be seen going about, their bodies bent forward, the knees in half-flexion, not spastic, indeed, but with inflexible joints, and slowly dragging one foot after the other, the soles scraping the ground. A certain similarity between this "demarche a petit pas" (gait of little steps) as Brissaud has called it, and the gait of patients with paralysis agitans (see Lecture V) is not to be denied, though the differential diagnosis presents no difficulty to those familiar with both conditions. We leave now the lacunar hemiplegias to turn to a disease picture which in a large percentage of cases also depends upon arteriosclerotic tissue defects, sometimes, indeed, from disseminated arteriosclerotic foci of another character, for example, miliary hemorrhages or fibro-hyaline obliteration of vessels. This is pseudo-bulbar paralysis, which depends upon the fact that small but multiple lesions in both hemispheres interrupt the course of the nerve fibers between the cortex and the bulbar nuclei of the muscles of mastication, deglutition and speech. Unilateral location of the foci alone is so rare that we need not consider here such cases described as curiosities only. This is explain- able from the fact that the lower facial, the hypo-glossal and the motor portion of the trigeminus are connected with both hemispheres, ami that as a rule one of these is sufficient to maintain the functions of the muscles sup- plied by these nerves. Bilateral multiple losses of substance are seldom located in the cortex, more frequently in the centrum ovale, the central ganglia (particularly in the putamen of the lenticular nucleus), in the internal capsule and in the crura cerebri. * In typical cases of this pseudo-bulbar paralysis- — we prefer this expression to the more cumbrous "cerebral glosso-pharyngo-labial paralysis" — the pa- tients have formerly suffered from the previously described lacunar hemiplegia. Now comes a repetition of the same attack, this time, however, on the op- posite side. This shows us that the arteriosclerosis lias led to the gradual production of small tissue defects in the second hemisphere. And now we notice that this second half-sided paresis is accompanied by definite disturb- ances of articulation and deglutition, which, however (in contradistinction to the first time), persist further, while the weakness of the extremities gradually passes off this time also. Besides this typical beginning, as rarer methods of occurrence of arteriosclerotic pseudo-bulbar paralysis are observed, its sudden and definite origin after a single attack; slow development in steps broken by remissions; finally, a transition form with abrupt commencement, then total disappearance and progressiva reappearance of the symptoms anew. Let us look more closely into this last. The speech becomes drawling, monotonous, there is sometimes aphonia. While the formation of vowels suiters less, the consonants are pronounced badly and with trouble, so that in severe cases l he speech becomes unintelligible. In speaking the breath often fails, and to complete the sentence the patient must begin over again several times; the enunciation takes on a chopped off or semi-explosive character. According to whether paresis of the lips or that of the palate dominates, disturbances in the 230 LECTURE XV formation of labials or nasal character predominate. The mobility of the tongue can be so greatly affected that, following the laws of gravity, it lies immovable upon the floor of the mouth. Usually, however, it can be protruded, at least to a certain extent, but there are disturbances in lateral movements or in forming a trough, or frequent repetition of its protrusion leads to rapid paralysis. Also the movements of the palate are either suspended or only slow and incomplete ; in the larynx, however, paresis of the vocal cords but rarely reaches a high degree. The muscles of mastication are usually again greatly affected, a close approximation of the teeth is impossible, the mouth often remains permanently half open. Eventual paralysis of the pterygoids manifests itself through the impossibility of pushing the lower jaw forward or laterally. Sometimes, also, the muscles opening the mouth are weakened and complete opening, or opening it against resistance is impossible. The taking of nutrition sometimes develops exceedingly marked disturbances. Here along with weakness of the masticatory muscles, that of the muscles of the tongue, lips and cheeks plays a role; food cannot be pushed between the teeth, falls out of the mouth, and must often be forced into the pharynx by the aid of a finger. If paralysis of the palate and of the pharynx are added, portions of food get into the nose or the larynx. Still, in mild cases, the act of eating, namely, when the patient eats slowly and selects solid and semi-liquid food, is relatively well accomplished. What constitutes the chief difference between the disturbances just related and those which we find in true bulbar paralysis, is the fact that the paralyzed muscles in pseudo-bulbar paralysis show no degenerative atrophy and no reac- tion of degeneration. Also, mutatis mutandis, the same difference which spastic spinal paralysis presents in contradistinction to spinal progressive muscular atrophy. True conditions of contracture can, indeed, little by little develop in the lips, the tongue and the palate, in patients with pseudo-bulbar paralysis. Increase of the masseter reflex is scarcely ever absent and is understandable. Paradoxical, on the other hand, is the very frequent disappearance of the palate and pharyngeal reflexes even when the voluntary movements of these parts are still intact. The expression of countenance has something very characteristic. The lower part of the face acquires little by little a mask-like stiffness through the combination of paralysis and contracture. Saliva flows from the half open mouth. The physiognomy takes on a lacrimose expression. With this con- trasts, indeed, the better mobility of the forehead, and the movable, often expressive eyes. Much rarer than pseudo-bulbar paralysis, is another disease condition pro- duced by bilateral disseminated tissue defects, the senile incontinence, type Homburger. One case observed by me corresponds anatomically exactly with the statements of this author, that is, there was a symmetrical status lacunaris, in the optic thalamus and in the corpus striatum. Clinically, such cases are characterized by the automatic character of the discharge of urine. At more or less regular intervals approximately the same quantity of urine, with constant retention of a residual portion, is discharged, and so suddenly that the patient wets himself. The ability to urinate spontaneously is preserved at the start but is lost later. ARTERIOSCLEROSIS OF THE NERVE CENTERS 231 After a longer or shorter time all the somatic consequences of multiple arteriosclerotic focus formation in the brain are usually combined with a psychical decadence, becoming ever plainer. We enter with this, the subject of the arteriosclerotic dementias. I intentionally use the plural, since quite different symptom-complexes occur. In patients who have suffered from lacunar hemiplegias, in pseudo-bulbar paralysis and in the incontinence just related, we are struck particularly by the triad: weakness of memory, morbid emotivity, and loss of interest. The whole mental habitus becomes something silly. The patient, for example, pours out hot tears for a dog that was run over, about which he read in the newspaper, while the fortune or misfortune of his family does not impress him. Also the hypochondriacal complaints which are brought forward in such numbers by these old people, give the impression of mental weakness. They do not, as a rule, relate to serious troubles, for example, to the loss of memory, power of attention, etc., but to trivialities, as to the hardness of their stools, etc. Upon this quite characteristic basis but in a rather manifold combination, a whole line of further psychical anomalies develop themselves, for example, ideas of unseen influence, suicidal impulses, even hallucinations, so as observed, very heterogenous individual pictures occur. Many of these patients must be placed under psychiatric care; I will not enter upon the further disease pictures well known to asylum physicians. Only one special arteriosclerotic psychosis would I mention here, since to know and to recognize its early stages is of importance for the non-psychiatrist also. I mean "arteriosclerotic pseudo-paralysis." (Again a "pseudo" — you see, arte- riosclerosis is a great simulator among diseases.) As a fact the similarity of this syndrome to mctasyphilitic dementia paralytica is quite marked (see Lec- tin' XIII, page 205). One of the newest descriptions of arteriosclerotic pseudo- paresis, whose recognition we owe to Klippel, Alzheimer and Binswanger, is by Charles Ladame. According to this author, the clinical symptoms of these patients (they are usually men between 50 and (50 years old) are in the somatic sphere, premature senility, bodily deterioration, sluggishness in the pupillary reaction for light, convergence and accommodation, exaggeration of the tendon reflexes, hardening of the arteries, increased blood pressure, sometimes also, arteriosclerosis of the kidneys. In the psychical sphere isolated defects of intelligence, good preservation of orientation in general and of consciousness of self and of illness, reduction of the power of fixing the attention, partial de fects of memory; sometimes very good judgment forming capacity in certain directions, demeanor externally well regulated (they present a "deceptive front"). Delusional ideas (mainly of a hypochondriacal nature), illusions and hallucinations, occur. The mood shows a mixture of anxiety, indifference, euphoria, great egotism, ideas of suicide. There is marked impulsive automa- tism. The differential diagnosis from progressive paralysis must usually be based upon the condition of the pupils, the defective menial condition with retained personality, appreciation of disease, the age of the person and his genera] habitus. The prognosis is in I he great majority of cases had. The anatomical substratum of this psychosis is not a simple one. It can he made up of the most varied results of the ci rebral vascular disease, including Marie's lacuna', puncl tte or larger hemorrhages of the brain, cortical or subcortical 232 LECTURE XV foci of softening, or of the "subcortical chronic, progressive encephalitis" described by Binswanger a diffuse degeneration of the medullary layer as a consequence of insufficient blood supply. Treatment In conclusion a few therapeutic indications. Among the different clinical pictures which we have passed in review, of course only those which are usually the expression either of imperfect nutrition or of temporary vascular spasm, present a favorable outlook as to the possibility of being influenced by treat- ment. In the other forms, the treatment can only seek the prophylactic end of preventing further progress of the lesion if possible, but this end is important enough. Let me emphasize that in spite of their organic basis many of the troubles of the arteriosclerotic pseudo-neurasthenics are to be favorably in- fluenced by ps3'cho-therapeutic reassurance, since along with the material basis the depressing feeling of threatened "breakdown," and anxiety about previously unexperienced disagreeable sensations exercise a morbid influence. Under all conditions, further, the greatest importance must be laid upon the dietetic regime ; absolute milk diet is to be considered only for a short time and in the rare cases of particularly obstinate sleeplessness or headache, though it has here sometimes a striking effect. In general a bland, predominantly ovo-lacto- vegetarian diet suffices. We must endeavor to so arrange it that the patient will take it for a long time without repugnance, and hence must proceed not too rigidly. Meat, for example, may be permitted at the mid-day meal, but only one dish, and the w r hite meats are to be preferred. Fresh water fish may be permitted, sea fish forbidden. Bouillon should be replaced by cereal and vegetable soup ; plenty of vegetables, easily digested farinaceous foods and potatoes, fruit raw or cooked, are permitted. Alcoholic beverages, strong coffee, and particularly the use of tobacco should be restricted as much as pos- sible, and all the stronger condiments avoided. The patient should take plenty of milk, eventually buttermilk, yoghurt, kefir. In general, as table beverages, alkaline mineral waters, as those of Vichy, Passugg, Neuenahr, Fachingen, etc., are to be recommended, particularly during iodide cures. Now, as far as these cures go you know that of late the indications for iodide of potassium in arteriosclerosis have been much narrowed by internists. We neurologists, however, consider this agent now, as formerly, so valuable a remedy that it should not remain untried in any form of nervous manifestations in arteriosclerosis. U/pon what the action of iodine depends is not yet definitely concluded; it appears (from the investigations of Miiller, Inada and others) determined that by reducing the viscosity of the blood it facilitates the flushing of the nervous centers; also a certain vasodilating action is attributable to it. According to Pouchet it has also a decidedly stimulating effect upon the lympho- and leuko-cj'tes and aids phagocytic processes, by which a modern explanation is furnished for its ancient reputation as a "summum resorbens." Very ques- tionable, on the other hand, is the specific curative effect upon parts of the vessel walls already affected with arteriosclerosis affirmed for it, while again, ARTERIOSCLEROSIS OF THE NERVE CENTERS 233 a prophylactic action in this direction is plausible from the fact that administra- tion of iodides often lowers the blood pressure. Since iodide of potassium or iodide of sodium must be prescribed always for a long time in arteriosclerotic nervous diseases, it is recommended that for the prevention of iodism daily doses of from 0.5 to 1.0 should not be exceeded, and that after each 20 days of use there should be a 10 day pause. The addi- tion of sodium bicarbonate usually causes the drug to be better tolerated by patients with sensitive stomachs. As a specially well tolerated combination I would mention that of potassium iodide and strychnine : besides the alkaline iodides, in the better class of practice a number of organic preparations of iodine, such as iodipin, sajodin, iodon, etc., may be used; also these prepara- tions are usually well tolerated and iodism is almost excluded, but in activity they do not compare with the alkaline iodides. While we are speaking of drugs we may consider the question of hypnotics, to use which we are often compelled by arteriosclerotic insomnia. They appear to me unobjectionable provided that we avoid such remedies as act unfavorably upon the vessel walls (chloral hydrate, amylenhydrate, paraldehyde, for ex- ample), and on the other hand follow the end of the reestablishing the ability to sleep spontaneously. The remedies of choice are the difficultly soluble hypnotics of the type of trional and veronal; we must resort to them when we cannot accomplish our ends with dietetic and hydriatic measures, or with nightly doses of bromides from 1.0 to 2.0 bromide of potassium (we refrain from giving larger doses of bromides since the arteriosclerotic seems to have a predisposition for bromism). 1.0 (grs. xv) of trional or 0.5 of veronal (gr. viii) dissolved in some hot liquid is given one hour before bedtime ; this medication can be given every evening if it is desired that the patient should get the full hypnotic effect of the normal dose, and also when necessary, should sleep on into the next day. Under these conditions, after a few days a certain sleepiness often appears and this gives us the signal for the reduction of the dose — of veronal, for example, to 0.3 (gr. 5). Later it is attempted from time to time to intermit a night, then to give the hypnotic every second, then every third night, and finally to withdraw it altogether. For senile "sleep inversion," Hamburger has proposed a method of "reinversion" of the type of sleep which I can thoroughly recom- iiiiiid : forcibly keeping the patient awake by day is carefully avoided; on the other hand, he is kept in bed, and we limit ourselves at first to procuring some sleep at night with hypnotics. If now the duration of the sleep by day and its depth diminishes, the evening dose of hypnotic can be increased and the pa- tient kept up longer and longer by day. Of hydrotherapeutic measures, hoi sail and mustard fool baths which should be used daily when there is a tendency to congestions about the head, vertigo, headache, etc., may be mentioned in the first place. Further tepid baths of iml too long duration and tepid rain douches uiili rubbing down afterward, work favorably. Carbonic acid baths should be used only with great caution, as they are poorly borne by many patients. Rath (aires proper, unless they are indicated on account of some other result of arteriosclerosis are not called for. The patient is best sent to some quid resorl of medium altitude (500 to ■ f \ £? The Blood Supply of the Spinal Cord. Injected preparation. (Carmine gelatine.) is replacement of the parts formerly affected by the inflammation by a more or less thick, gliotic scar tissue, more or less rich in vessels. The more extended the affection of an anterior horn has been, the more shrunken it appears, on which account sometimes, one-half of the spinal cord appears smaller than the other. Only a more or less considerable part of the cells of the anterior horn remain of characteristic appearance ; the others have either entirely dis- appeared or have been changed to inconsiderable clumps (see Fig. 75). Prognosis The prognosis as to life is in general, and if the sporadic cases are chiefly considered, favorable. Only rarely does exitus occur in the infectious stage under hyperpyrexia and heart weakness, also the bulbar or ascending forms INFECTIOUS DISEASES OF CENTRAL NERVOUS SYSTEM 245 of poliomyelitis as well as the meningitic varieties of the disease, all of which endanger life through their localization, belong to the exceptions. In the epidemic poliomyelitis, however, the prognosis is much more serious. So, ac- cording to Harbitz, Scheel and Wickman, in the epidemics in Sweden and Norway in 1905, among 2,078 cases there were 290 deaths, which is a mortality of about 1-1 per cent. As to the prognosis with regard to recovery, complete restoration is a great rarity ; much more frequently there is partial recovery, that is, extensive improvement. The more rapid the regression of the paralysis, Fig. 75. Acute Anterior Poliomyelitis in Childhood. Late stage, carmine preparation from the spinal cord of a seventy-year-old man. (The cells of the anterior horn, still recognizable microscopically, are brought out by retouching.) the better the outlook for a favorable result. Of the greatest importance, further, as already emphasized (page 2.'J7), is the result of the electrodiag- nostic testing. Permanent severe paralysis are absent in only about a third of the cases. In various instances the occurrence of another spinal cord disease in later life in patients who have had infantile spinal paralysis has been observed (com- bined system diseases, diffuse myelitis; particularly, however, spinal progres- sive muscular atrophy and chronic anterior poliomyelitis).' * Chronic or subacute anterior poliomyelitis, an exceedingly rare disease, which we have already mentioned under the differential diagnosis of spinal progressive muscular atrophy, can occur also without previous acute poliomyelitis, and usually in middle life. Its etiology is ob- scure. Without febrile s\ mptoins, pain, sensory or sphincter disturbances, but usually with fibrillary contractions, there appears an Increasing weakness in the lower extremities, which 246 LECTURE XVI Differential Diagnosis Most important from a prognostic point of view is the distinction between poliomyelitis and polyneuritis, since the last (which is not so rare in children as was formerly assumed) presents a good outlook as to complete recovery. In poliomyelitis, the paralysis reaches its maximum in a few hours, shows from then on a decided tendency to regression and is scarcely ever progressive. On the other hand, the paralysis in acute polyneuritis only reaches its maximum, as a rule, after days or weeks. Continuous extension or advance by stages in acute atrophic spinal paralysis is of the greatest rarity, on the other hand. One can count less upon the results of testing sensibility, since it should be remembered that the polyneuritis of childhood is not rarely purely motor (see page -tl). Decisive as to polyneuritis is palpable thickening of nerve trunks or the occurrence of ''glossy skin." The involvement of the cranial nerves speaks on the whole more for polyneuritis, optic neuritis almost certainly so. Affection of the muscles of the neck and trunk, in case there is no history of diphtheria, speaks for poliomyelitis and against polyneuritis. In the first the decided affection of the roots of the extremities is more frequent than in the last, which usually more markedly affects their ends. While after about one year poliomvelitic paralysis has almost always reached its stationary stage, the regression of polyneuritic paralysis can extend over a much longer time. In poliomvelitic paralysis, atrophy, and reaction of degeneration are usually de- veloped proportionately. In polyneuritis reaction of degeneration can be found even in muscles which can still be moved voluntarily, hence electroprog- nosis is little certain here. The reappearance of faradic irritability which has been lost, turns the balance in favor of the neural seat of the paralysis. Oedema speaks for polyneuritis, and, finally, in this last, the fever may keep up longer and reappear from time to time. Acute myelitis occurs exceedingly seldom in children; in differential diag- nosis it hence plays a role almost only as opposed to the acute poliomyelitis of adults ("acute atrophic spinal paralysis" of adults). This last affection is exceedingly inferior in frequency to infantile poliomyelitis, but nevertheless is not such a very great rarity and has occasionally been confirmed by autopsy {Striimpell, Schultse, Williamson and others). That occasionally during epi- demics of the Heine-Medin disease cases affecting adults have been observed justifies us in considering the poliomyelitis of adults in connection with in- fantile spinal paralysis. Many times, however, the acute atrophic spinal par- alysis of adults has been observed in connection with influenza, gonorrhoea, puerperium, typhoid, etc., so that perhaps the most varied agents come into question in connection with it. Clinically it differs from the infantile form in the course of several days or weeks increases to an atrophic paralysis. The arms, usually soon after, share the same fate; the condition remains stationary several months, then im- proves gradually; indeed, there may he complete recovery. Important in prognosis is the degree of reaction of degeneration (always to he found). Death under bulbar symptoms only rarely occurs. Anatomically there are inflammatory degenerative lesions in the anterior horns of the spinal cord. In contradistinction to spinal muscular atrophy, in chronic poliomyelitis the paralysis precedes the muscular wasting. Further, it always begins in the lower extremities and runs a more rapid course. Its therapy is mainly an electric one; this corresponds to the electrotherapy of infantile spinal paralysis. INFECTIOUS DISEASES OF CENTRAL NERVOUS SYSTEM 2+7 through longer duration ( 1 to 2 weeks) of the acute stage, through, as a rule, greater extent of the sudden flaccid paralyses and through slight tendency to regression. On the other hand, corresponding to its occurrence in com- pletely developed individuals (usually in the third decade) the trophic dis- turbances and the tendency to contractures are less marked. From acute mye- litis this acute poliomyelitis is distinguished by the absence of sensory and sphincter disturbances — ataxia, spastic symptoms, Babinski reflex, etc. (see Lecture IX). Treatment In the infectious stage of poliomyelitis acuta anterior, there is little to be done therapeutically, indeed, meddlesome interference is inadvisable. It ap- pears to me much more correct to secure for the affected children as com- plete bodily rest as possible, than to disturb them by frequent warm baths and hot applications, as is the custom in America, for example. These procedures are intended to relieve the congestion of the spinal cord; this can be accom- plish! d, however, by the application of several leeches along the vertebral column, by mustard paste to the calves, as well as by stimulating the function of the intestines. Among purgatives, calomel is preferred with the idea that it exerts an antiseptic action. Upon the same ground quinine salol, aspirin, etc., are recommended. I must, however, confess a great scepticism as to all these remedies. Only after the patient has entered the stage of paralysis can we interfere with somewhat more confidence in order to aid the reparative processes. Here strychnin is the medication of choice, best in the form of daily subcutaneous injections which, of course, must be carefully adapted to the age of the children. For example, in the second year of life, in which the disease occurs most frequently, 0.0005 (gr. K20)) given in one dose during the day. Further, favoring diaphoresis with hot infusions (for instance, lime Bower tea), hot packs, hot air apparatus ("Phoenix," etc.) are to be recom- mended. On the other hand, too early beginning electrical and massage treat- ment is quite properly opposed by both neurologists and pediatrists. It is hist to hold to Opperihevm's rule not to begin until after two or three weeks have elapsed, while at the start the patient is kept continuously in bed, which restriction is only to be relaxed later in the stage of regression. When we have once begun with electricity and massage, this treatment must be kept up for a long period, until definite entry upon a stationary condition is no longer dubious, on an average, for about, a year. The elect ro-therapeut ic methods here applicable have already been described in Lecture III and Lecture VII, namely, galvanization and faradization of the nerves and mus- cles in the paralyzed regions, «nd the galvanic current passed through the spinal cord. One must begin with very weak currents and gradually accustom the little patient to stronger ones. .Massage, also, must be carried out in a mild, careful manner, and it must he attempted to stretch the muscles which are exposed to contracture as well as to preserve the mobility of the different joints. Vibration massage of the articulations tends to counteract trophic disturbances of the capsule atid ligaments. The carrying out of active gym nastic exercises is also important: those movements which the children may 248 LECTURE XVI little by little become able to practice again, must (systematically with avoid- ance of all overexertion) be carried out, and against regulated increasing re- sistance. Massage and exercise therapy combats sluggishness of circulation, and so brings the muscles, integument and skeleton under better conditions of nutrition. The very popular salt-bath cures, and rubbing with liniments which stimulate the skin (linimentum ammonias, linimentum saponis, spiritus juniperi, etc.), accomplish the same thing. Of modern procedures, "diather- my," after Nagelschmidt, Bergonie, etc., appears to act favorably. In no case should it be neglected to reduce conditions favoring the production of deformities as much as possible, by the application of suitable splints, bed- rests, etc. If the patient has entered the stage of trophic disturbances and contractures, he should come under the care of the orthopedist. This is not the place to discuss at length the often very successful operations (arthrode- sis, transplantations, plastic operations on tendons, etc.) or the various ortho- pedic apparatus, as they have been contrived and introduced by Vulpius, Hoffa, Nicoladoni, Hiibscher, Schvlihess, Hessing, and others. B. Epidemic Cerebro-Spinal Meningitis In contradistinction to epidemic infantile paralysis, epidemic meningitis is a bacteriologically well characterized, acute infectious disease. It is to-day established beyond a doubt that the meningococcus intracellularis (discovered by Weichselbaum in 1887) is its specific cause. This is a micro-organism, in its biscuit shape and its position within the leucocytes resembling the gono- coccus which, inoculated into dogs, monkeys, or goats, produces a meningitis. Nevertheless, mixed infections with other pathogenic germs, particularly with the pneumonococcus and the diplococcus crassus, occur. Epidemics of the last sort have been observed particularly in barracks. The method of infection is connected with living in close association and occurs (since the meningo- coccus may remain viable in the naso-pharyngeal mucus even of healthy indi- viduals, for months) regularly by "drop infection" in coughing, hawking, sneezing. Westenhoffe% has recognized that its passage into the meninges takes place from the pharyngeal tonsil and the lymphatic tissue. Children with hyperplasia of these structures should be considered as predisposed. Ordinary anginas favor the infection, hence the preponderant occurrence of epidemics in winter, and in the cold, damp spring months. That traumatic influences which affect the skull (in children, sometimes boxes on the ear from the teacher or from the parent, have been held responsible for the outbreak of meningitis), are to be considered as exciting causes, is not certainly proved, but quite plausible. Besides the lymph channels the blood vessels are also open to invasion of the meningococcus, as culture experiments after punctur- ing the vein have shown. The first meningitis epidemic was observed in our country (Geneva) in 1805. The pathological anatomy of the affection, apart from inconstant myo- carditic changes and rather regular inflammatory lesions of the tonsils and the middle ear, consists predominantly of meningitic processes. The brain mem- branes, particularly their basal region, usually are much more decidedly affected INFECTIOUS DISEASES OF CENTRAL NERVOUS SYSTEM 2-19 than the membranes of the cord. There is dependent upon the intensity of the case, and upon the stage in which it comes to autopsy, formation of a serous, sero-fibrinous or fibrino-purulent exudate between the pia mater and the arachnoid. The inflammation, however, also extends along the vessels or the nerve roots to the central organ itself. In the brain, abscesses, porencephalic defects, inflammatory hydrocephalus, etc., can occur. Symptomatology and Course Epidemic cerebrospinal meningitis usually seizes its victims brutally with- out prodromal symptoms, with sudden rise of temperature and repeated chills. In young children convulsions are not very rarely observed as an initial symp- tom. Only in the minority of cases a more or less marked feeling of illness, eventually with stupor and vomiting, precedes the disease proper. This, as a rule, in from 2-i to -18 hours after the onset of the fever, has readied its full height, and then presents the following symptoms in more or less intense degree : Headache of great violence and persistence, which appears to have its maximum intensity in the occipital region ; very frequently, also, the vertebral column is the seat of spontaneous pains, particularly in the cervical and lum- bar regions. Both the occipital region and the spinous processes of the vertebral column show great sensitiveness to percussion and pressure. Besides this, there is general hyperesthesia, all handling of the patient elicits expres- sions of pain ; there is also great sensitiveness to optic and auditory stimuli. Leichenstern' s phenomenon seems to indicate hyperesthesia of the skeleton; the patient when tapped on the bones of one of his extremities draws himself up violently, often with a cry. The "hydrocephalic cry" of Trousseau, a shrill scream which some patients even in deep stupor emit from time to time, is perhaps also to be considered as an expression of pain. The "stiff neck" which has given the disease its popular name * imposes itself as pathognomonic even on the laity. Even on slight development of this dreaded symptom it is noticeable that the patient does not, as is generally the rule in other severe illnesses, let the chin sink more or less upon the chest, but that he deflects the head, and the occiput bores into the pillow. Active as well as passive movement of the neck is hindered in an increasing degree; our attempts to bend his head forward are answered by the patient with ex- pressions of severe pain. Later, the rest of the vertebral column becomes stiff as a board. This high degree of hypertonia extends farther to the mus- cles of the extremities: in the lower extremities it is accompanied by a draw- ing them up on the body (tendency to contracture in flexion) through which can be produced the anomaly "I' position which has been called by the French on account of its analogy to the shape of a gun-flint, "attitude en chien de fusil." Here belongs also Kermg's sign, which depends upon the impossibility of bringing down the extremities of the patient placed in a sitting posture to the plane of the bed from their position of flexion at the hip and knee, further * German "Gcnickstnrrc." 250 LECTURE XVI phenomena characteristic of meningitis arc masklike rigidity of the counte- nance, sometimes a moderate degree of trismus, with audible grinding of the teeth, particularly frequent, however, the "boatlike" drawing in of the abdom- inal walls. The tendon and skin reflexes are usually exaggerated, their loss is an ominous symptom. Vasomotor irritability is also increased, from which the particular form of dermographia (see Lectures XXIII and XXVII) can ap- pear, which Trousseau denominated "tache cerebrale." Stroking the integu- ment leaves behind Maine-red streaks which only pale after considerable time. A further cutaneous symptom of epidemic spinal meningitis is herpes, which in the majority of cases appears on the lips and face between the second and sixth day of the disease, while scarlatiniform erythemata, multiple hemor- rhages into the skin, or a roseola resembling that of typhoid fever are quite atypical findings. Further symptoms of the disease are vomiting, stupor, less frequently de- lirium, further, retention of urine and stools or ischuria paradoxa (dribbling of the urine when the filling of the bladder has reached a certain degree), often rapid emaciation, occasionally albuminuria and glycosuria. Character- istic of the fever is a continuous course at the start with temperatures between 39° and 40° C, which later a stage of alternating remissions and exacerbations follows ; its rise to a hyperpyretic degree is prognostically bad and often im- mediately precedes death. In cases which enter upon recovery, the fever disappears by lysis. As to the pulse-rate, it should be emphasized that it is relatively low compared to the temperature, as a rule (for example, 100 for 40°, 80 for 39°), and that in convalescence a slow pulse is the rule (.50 and lower). A rapid and considerable increase in frequency during the disease points to threatened exitus. As to whether the slow pulse should be considered a vagus symptom, views differ. On the other hand, other cranial nerves in many cases are affected in unmistakable manner, particularly the optic and the auditory. While the meningitic optic affection (which shows itself ophthahnoscopically as papil- litis), in cases which survive, seldom leaves behind amaurosis, auditory neuritis usually causes impaired hearing, sometimes, indeed, deafness. The pupils are usually narrow and react sluggishly for light and accommodation; on the other hand, they often dilate markedly when the integument is irritated. This last symptom, described by Goppert, can be obtained by stroking the skin with a finger-nail. Paralyses of the facial or of the external eye muscles are rarer. In a limited number of the cases, pus collections in the eye, in the middle ear, in the brain substance occur, partly by direct conduction, in part by metastasis. With the last, there are produced spasms of constant localization (for example, in one side of the face, in one arm, etc.), which leave behind a paralysis of the muscles affected. Also a meningococcic endocarditis occurs. In the blood there is regularly a hyperlcucocytosis ; according to Goppert and others, high lcucocytosis (over 24,000) is prognostically serious. It can reach 60,000. As long as the lcucocytosis is not diminishing new exacerba- tions may be expected, even when the disease picture otherwise has consid- INFECTIOUS DISEASES OF CENTRAL NERVOUS SYSTEM 251 erably improved. Finally, at the height of the disease a considerable poly- nucleosis shows itself in the spinal fluid (see Lecture XII, page 187). In the stage of convalescence (according to Acharil and others) the polynuclear leucocytes are replaced by numerous lymphocytes. Bacteriologicallv the me- ningococcus can be recognized in the cerebro-spinal fluid, as Heubner first showed. According to the course of epidemic cerebro-spinal meningitis, different forms of it have been distinguished. In the "foudroyant" form (meningitis eerebro-spinalis siderans) the patients die within a few hours after the begin- ning of the first symptoms; indeed, they can after relatively slight prodromes (slight fever, headache, nausea, etc.) with a loud scream fall dead to the ground, in which case we speak of apoplectiform meningitis. In a few cases of the "foudroyant" form there has been no rise of temperature. The rudi- mentary or abortive cases, in which the chief symptoms of the disease are only present in an undeveloped manner, form the other extreme. There is slight fever, some headache and nausea, feeling of tension in the neck and in the limbs, sensitiveness to touch, noises, etc. Such cases recover in from 8 to 14 days: when they appear sporadically, they are scarcely ever diagnosed, but their connection with cerebrospinal meningitis is only recognized during epidemics. In the acute forms, however, death in coma occurs in from 1 to 3 weeks after the outbreak of the disease. Finally, there is a prolonged, remit- tent; or even intermittent form, which with alternating improvement and re- lapse, can extend over weeks or even months. Termination in recovery is possible; usually, however, death in a so-called "status hydrocephalicus" finally occurs, after the most extreme emaciation and exhaustion, tonic contractures and flexion of the extremities, deep coma, frequent vomiting, etc. In conva- lescence after epidemic cerebrospinal meningitis, the danger of relapse is always great. Prognosis After what has been said above, the prognosis of cerebrospinal menin- gitis is naturally a very grave one. The mortality varies from epidemic to epidemic within comparatively wide limits (30 to 70 per cent.) ; as an average it is about 40 to 50 per cent. The more violent the commencement, the iess tin hope of survival. Even among the so-called "recovered" a large number remain severely injured for life (blindness, partial or complete deafness, mental reduction). Others escape with less impairment (tinnitus aurium, squint, tendency to headaches or neuralgias). Treatment From Hie point of view of prophylaxis, isolation of the patients, accord- ing to the general rules in infectious diseases, is to he ordered. The isolation ■{"carriers," that is, healthy pel-sons who have meningococci in their naso- pharyngeal secretions and who contribute to spreading the epidemic, is unfor- tunately only exceptionally attainable (for example, in epidemics in barracks). 252 LECTURE XVI In general one must content himself with having the relatives of the patient use disinfecting washes, irrigations, etc., for careful cleansing of the naso- pharyngeal space. If a serum prepared by Wassermawn and Kollc for this purpose is specially efficacious, we must wait and see; favorable results have been obtained from it by Kutscher. Also with regard to the result of specific treatment with meningococcus- sera (such have been prepared by Jochmann, Flexner, Kolle, Wasxermann, and others, positive statements are not yet possible, since in consequence of the very variable malignity of different epidemics (see above, page 251) the com- parison between the mortality in cases in which the sero-therapy was carried out and those in which it was not gives too great room for subjective impres- sions. Nevertheless, it is imperatively necessary to clear up the subject by further researches. According to Peritz the following rules should regulate such investigations: If on the first lumbar puncture in a suspicious case, the cerebrospinal fluid is tin bid. serum (it should not be older than three months) should be at once injected, and only when the fluid is clear should we wait for bacteriolog- ical examinations. When the puncture is made, as much fluid as possible is withdrawn, and then 30 cc of the serum is injected into the subarachnoid space, even when less fluid than this has been withdrawn. When a larger quantity of fluid has escaped, as much as 45 cc of the serum can be given. In very severe cases this last is particularly desirable. An appreciable resistance to-; the injection gives a signal for its cessation (Levi/ recommends limiting the first injection to 10 cc in small children and to 20 cc in adults). In malignant cases, if there is no improvement the injection should be repeated in twelve hours. Except in the very mildest cases the injections should be repeated daily for four days. If then diplococci are still found in the spinal fluid, the injections should be continued; when, however, after disappearance of diplo- cocci from the fluid and in spite of four injections, the subjective symptoms, including the fever and stupor, continue, Ave should wait four days, and then if there is no improvement repeat the four injections. Each exacerbation should naturally be treated on the same day. Each time the process flares up again, we should at once begin the four-day treatment and proceed otherwise as on the first occasion. This plan of treatment is to be continued until the patient is free from symptoms, the diplococci have disappeared from the spinal fluid, or the disease has entered upon the chronic stage. As you see, this procedure demands numerous lumbar punctures and that these last by themselves, that is, even without scrum therapy, present a rational method of treatment — corresponding to the rule "where there is pus, evacu- ate" — is evident without further discussion. They are undertaken then quite generally in epidemic meningitis, and usually as a routine procedure, that is, daily or every second day, 10 to 20 cc of fluid are removed. A few authors have, indeed, no hesitation in removing sometimes far greater quantities of fluid Zupnik, for example, 70 to 90 cc. The headache and stupor often markedly decrease after this procedure. INFECTIOUS DISEASES OF CENTRAL NERVOUS SYSTEM 253 TECHNIQUE OF LUMBAR PUNCTURE The puncture of the subarachnoid space, first done by the American neu- rologist Leonard Corning and soon after introduced into clinical practice by Quincke, is made in the lower portion of the lumbar region of the vertebral column. This localization suggests itself from the necessity of avoiding injury of the spinal cord with the puncture needle ; the cord, however, does not extend below the second lumbar vertebra. Further distallv, indeed, the "cauda equina" lies in the subarachnoidal space, but the nerves composing it are always pushed aside by the needle so that appreciable wounding of them does not occur. Slight pricking of them produces onty a short, lightninglike pain. We punc- Fio. 76. Anatomical Points, tor Guidance in Making a Lumbar Puncture. ture usually between tin- 4th and 5th vertebrae, though the puncture can be made between the 2d and .'id or between the 5th lumbar vertebra and the lacrum. Topographic orientation is very easy, .since a line drawn through the* highest points of the ilia passes over Hie point of the spinous process of the 4th lumbar vertebra (sec Fig. 70). We use a hollow needle, at least S cm ping and <>( 1 mm externa] caliber and <).(> nun lumen, shortly but sharply beveled al Hie point; a mandrill should be inserted during the puncture. This cm he made in the median line-in the space between the 4th and the 5th lumbar vertebra?; it is in general better, however, to make the puncture about 1 cm to the right of the middle line, since by so doing, on the one hand the lough interspinous ligament is avoided, and on the other the needle enters the somewhat more roomy lateral part of the intervertebral foramen. Of course, such a ••paramedian" puncture demands that the needle he directed ■Omewhat toward the median line, as shown in Fig. 77. for the rest, in children the needle should be introduced al Hie ideal cross sect ion of the 254 LECTURE XVI trunk; in adults it must usually be introduced somewhat more frontallv. The operation is further made easier by strong flexion of the vertebral column, since by this the space between the arches of the vertebrae is enlarged. Pa- tients who can sit up must hence bring their heads forward as low as possible; where this is not possible, as is almost always the case in meningitis, the patient is laid on his side and his knees bent up as far as possible. The skin over the point of entrance is disinfected by painting it with fresh tincture of iodine, and eventually is anesthetized with the chloride of ethvl spray. The hands are carefully disinfected (by soap, scrubbing and alcohol), and the previously boiled needle is introduced in the direction indicated. The ligamen- tum flavum, which is stretched between the arches of the vertebra?, gives an elastic resistance which, however, suddenly yields, and the point of the needle enters the subarachnoid space. If, however, the needle is wrongly directed ltroduction of the Needle in Lumbar I'tincture. and strikes the hone instead of the yellow ligament, it is somewhat withdrawn and directed either more frontally or more caudallv. If the instrument has been properly introduced, the mandrill is withdrawn and the fluid escapes in drops or, when the pressure is increased, in a stream. If the needle gets stopped up by coagulum, which is not rare, the obstruction is to be removed by reintroduction of the mandrin. If, instead of fluid, blood appears, the needle has probably entered a vessel of the venous plexus which covers the posterior aspect of the body of the vertebra ; in this case the needle is with- drawn several mm until no more blood escapes. That anomalies of the verte- bral column or adhesions of the arachnoidal sac render lumbar puncture impossible is a rare happening. The depth to which the needle must penetrate varies from 2 cm in small children and 6 or 7 cm in adults. After lumbar puncture the patient should remain in a horizontal position for at least 24 hours. Bier, Vorschiitz and Eckert have recommended the combination of lumbar puncture with "stasis." Two hours after the puncture an elastic band is placed INFECTIOUS DISEASES OF CENTRAL NERVOUS SYSTEM 255 about the neck, tight enough to produce a slight cyanosis of the face, and kept on about 20 hours. In general use are hot baths — which, according to Heubner's directions, should be given daily — in which beginning with 35° C, the temperature should be raised slowly to 40° and more. On the other hand, cold baths and affusions, as are customary in typhoid, are recommended. Application of the ice-bag or of the cold cap to the head and along the spine usually reduce the headache. Further, derivative procedures, as leeches or cupping over the mastoid and along the vertebral column, inunction of gray ointment over the neck, blisters, frequent purgation (calomel), mustard paste to the calves, etc., can be used. Of drugs, antipyretics and sedatives come into consideration (salicylate of sodium, antipvrin, pyramidon, chloral, morphin, etc.). More important, how- ever, is the carrying out of the difficult and responsible nursing; namely, by the choice of concentrated food, its frequent introduction (when it is necessary with the oesophageal tube), the rapid loss of strength is to be combated as much as possible. In convalescents the most careful oversight and tonic treatment is in place (fresh air, sea and mineral baths, arsenic, strychnine, etc.). Xote. — On account of the discovery that when hexamethylamin (urotropin) is given hy the month it appears in the spinal fluid, this drug is quite generally administered in infectious dis- eases of the central nervous system, particularly in poliomyelitis and cerebrospinal meningitis, its value is, however, uncertain.— 7Y««W<(/ui\ LECTURE XVII Encephalorrhagia and Encephalomalacia (Cerebral Hemorrhage and Cerebral Softening) Gentlemen : We turn now to the exceedingly important disease pictures which arise from more or less extended, circumscribed destructions of the brain substance upon a basis of vascular lesions. Now it is rupture of an artery, again occlusion of an artery (from endarteritis, thrombosis or em- bolism). In the first case a pouring out of blood destroys the cerebral pa- renchyma, in the last this undergoes ischemic softening. Now, before we consider separately encephalorrhagia (cerebral hemorrhage) and encephaloma- lacia, we will take up the most typical of the symptom complexes through which they manifest themselves clinically — cerebral hemiplegia. Since now, however, of these cerebral hemiplegias by far the greatest number have their focus in the internal capsule, our description of the semiology will be based upon this form. Capsular Hemiplegia This is, as a rule, the result of a cerebral hemorrhage. That the internal capsule is the favorite seat of this last is due to the anatomical relations of the vascular distribution. The middle cerebral artery, the most important branch of the internal carotid, gives off on the base of the brain, branches mounting vertically to the optic thalamus, the corpus striatum, and the internal capsule, the lenticulo-optic artery, and the lenticulo-striatc arteries. One of these last which passes along the surface of the lenticular nucleus to the internal capsule, perforates this and finally ends in the corpus striatum, has been given by Charcot the name "artery of cerebral hemorrhage" (see Fig. 78). These perforating basal vessels (in contradistinction to the arteries of the cortex which anastomose with one another) are so-called "end-arteries"; since, be- sides this, they come off at nearly right angles from the largest branch of the carotid, there is in them a pressure nearly equal to that in the carotid, and every increase of this pressure is transferred directly to them, but not to other cerebral vessels of similar caliber. Mendel has been able to bring these rela- tions to demonstration manometrically in a model of the cerebral vascular system made out of rubber tubes. Variations of pressure are now, as we will later see, of decisive importance in the production of cerebral hemorrhages. There lies, however, in the posterior limb of the internal capsule nearly the whole of the motor tract for the opposite side of the body together in a 256 ENCEPHALORRHAGIA AND ENCEPHALOMALACIA 25't relatively very small space (see Fig. 79). Complete hemiplegia, hence, is in by far the majority of cases the consequence of a lesion of this posterior limb of the internal capsule, that is, there is a cross paralysis of the lower facial (the upper escapes in consequence of its bilateral representation in the cortex) of the hypoglossus, of the arm and of the leg. If the disease focus, however, extends also to the posterior third of the posterior limb of the internal capsule, Fig. 78. Arterial Supply of the Cerebrum and the Basal Ganglia. H =Distribution of the Anterior Cerebral Arterv. HD Distribution of t lie- Middle Cerebral Arte II = Distribution of the Posterior Cerebral Artery. C.i. = Internal ('anil id. A.e.ni. Middle Cerebral Arterj . ] = I-enticulo Optic Artery. .' and :'. Lentieulo Striate Arteries. 8= So-called "Artery of Cerebral Hemorrhage." where the sensory tracts lie together immediately after their exit from the pptic thalamus, I here results besides a crossed hemianesthesia of the whole body. Sometimes the destructive focus extends even to the posterior end of the internal capsule to the so called "Carrefour sensitif" (sensitive crossway), where two important sensory tracts, the optic and the auditory, branch off, from the total contingent of sensory tracts to proceed to the visual and audi fcory centers of the brain cortex (see Fig. ?!>)• In such cases, now, there occur also hemianopsia and unilateral deafness, 258 LECTURE XVII both on the opposite side; since each visual area receives the visual impressions from the opposite halves of the visual field of both eyes, as is shown in Fig. 80, and each auditory cortical area the end neurons of the auditory tract from the opposite ear. Apart from the eventual sensible and sensory accompanying symptoms, capsular hemiplegias present also the following peculiarities: Y t Fig. 79. The Internal Capsule and Corona Radiata. T = Optic Thalamus. L = Lenticular Nucleus. C = Caudate Nucleus. F = Supranuclear tract for the Facial. H = Supranuclear tract for the Hypo- glossal. A = Supranuclear tract for the Arm Muscles. B = Supranuclear tract for the Leg Muscles. S = Sensory tract (Tractus Thalamo- cortical ). a = Auditory tract to the Temporal Lobe. v = Visual (Oratiolet's) tract to the Oc- cipital Lobe. 1 = Fronto-Pontine tract and fibers to Thalamus. •2 = Occipito-Temporo- Pontine tract and fibers to Thalamus. Balken = Corpus Callosum. Insel = Island of Reil. Frontal-Lappen = Frontal Lobe. Temporal-Lappen = Temporal Lobe. Occipital-Lappen = Occipital Lobe. Besides the upper facial muscles and on account of analogous relations of innervation (connection of the corresponding nuclei of the brain axis or spinal cord with both halves of the cerebrum) the masticatory, deglutitory, eye and EXCEPHALORRHAGIA AND EXCEPHALOMALACIA 259 trunk muscles remain unaffected. (For the rest, as to the upper facial, it Is, however, to be stated that often there is slight diminution in the contractility of the frontalis and the orbicularis palpebrarum on the side opposite to the lesion; the eyebrows hang perhaps somewhat deeper, or the eye can be kept closed for a shorter time than upon the homolateral half of the face.) Also, Fio. 80. Visual Trait am! Pupillary Keflex Paths. Opt. Erinnerps. Centr. — Center fur Optic Memories. Sehrinde = Cortical Visual (enter. • Gratiolet'sche Strahlung= Optic Radiations. Primare Sehcentren Primary Visual Centej Gesichtsfeld Visual Field. in complete hemiplegia different groups of muscles in the extremities are regu- larly unequally affected. While power of movement, little by little, returns in a great number of muscles, others do not usually recover again (peroneal muscles, flexors of the knee, extensors of tin- elbow, extensors of the hand and fingers, external rotators of tin- arm, supinators of tin- forearm). The arm, as a rule, is more affected than the leg. The positions, however, which the 260 LECTURE XVII extremities assume in consequence of the preponderating action of those mus- cles which regain their motility are often later fixed by contractures. On this account the foot is held in equinovarus position and the knee extended so that in walking, the leg dragging, must be swung forward in a lateral arc ("circum- duction," "helicopodia"). The arm is fixed in adduction, the elbow, hand and fingers in flexion. These contractures occur from the fact that the anterior horn cells, liber- ated from the inhibiting influence of the pyramidal tract, are stimulated in a Cerebral Hemiplegia from Capsular Hemorrhage on the Right Side. Circumduction of the Left Leg in Walking ami Typical Contracture Position of the Left Ann. tonic manner by the impulses entering through the posterior roots, and a summation of these stimuli is produced. Since, however, there are certain definite muscles which regain their motility and therewith (thanks to the supranuclear hypertonic nature of cerebral hemiplegia) are predisposed to contracture, this striking regularity must depend upon relations of innervation not yet sufficiently explained anatomically. Probably motor paths from the subcortical centers (for example, the roof of the mid-brain, the tegmentum and Dciters' nucleus) pass in a preponderating manner directly into contact with the anterior horn cells of these groups of muscles, so that in the repara- tory effort of the organism it is not difficult for them to obtain again, in a roundabout way, a share of the cortical innervation. Cases of cerebral hemi- ENCEPHALORRHAGIA AND ENCEPHALOMALACIA 261 plegia in which other muscle groups retain preponderance and develop con- tractures (so that, for example, the leg is fixed in flexion) form very rare exceptions. The hypertonia of capsular hemiplegia is accompanied by increase of the tendon reflexes. To this hyperreflexia and the pathological reflex phe- nomena (Babinski's, Oppcnhcims and the Mendel-Becliterew reflexes), clonus, certain associated movements, and so forth, which so often accompany them, all that we said in Lecture All in connection with spastic paraplegia applies. In a great number of capsular hemiplegias besides, one or another of the following phenomena can be demonstrated: The patient is laid upon a firm surface, his arms crossed and his head partially supported ; if he is now re- quested to raise himself up, he bends his leg on the paralyzed side at the hip-joint so that the heel is raised from the supporting plane. The same asso- ciated movement (the "hip flexion phenomenon") appears when the patient, seated, attempts to lie down again. As "platysma phenomenon" ("signe de peaucier") is denominated the energetic contraction of the platysma of the healthy side, which becomes visible when the cerebral hemiplegic opens his mouth. Finally, an interesting modification of the radius reflex, which con- sists in the occurrence of a flexion of the fingers instead of the normal flexion of the forearm at the elbow, when the radius on the paralyzed side is tapped at its distal extremity, may be mentioned. The skin reflexes (namely, the abdominal and cremaster reflexes), on the contrary, are almost always reduced or absent on the paralyzed side; this symptom can be used while the patient is lying unconscious after an apoplectic stroke, to determine on which side of the body the hemiplegic paralysis will be found after he comes out of the coma. This phenomenon is explained in (mite plausible manner as follows: The centripetal fibers concerned in the abdominal and cremaster reflexes enter the cord through the posterior roots of the lower dorsal and upper lumbar regions and their centrifugal fibers come through the anterior roots from the same part of the spinal cord. But the stimulating process is not transmitted here through the direct spinal reflex arc, but through the intermediary of in- terposed neurones which first proceed in a frontal direction into the cerebrum and then again run caudallv. We cannot propose any further hypotheses concerning this, in any case, complicated mechanism. The conjunctival reflex is also often absent on the paralyzed side. Capsular hemiplegia is a nondegenerative paralysis. Nevertheless, there are certain exceptional cases in which iii the paralyzed or even in Hie recovered muscles, the so-called "cerebral atrophy" which cannot be referred to inactivity and is accompanied by reduction of the electric irritability, although without reaction of degeneration, develops. With this, arthropathies like those in tabes have been observed. Still more isolated are the observations of atrophy of bone upon the paralyzed side. .More frequent, on the contrary, than all these trophic disturbances, are anomalies of the vascular innervation: the paralyzed parts are cyanotic and feel cold; sometimes there develops quite decided localized (edema. Tin- clinical picture jusl sketched you need not expect immediately after the occurrence of a cerebral hemorrhage or of a sudden plugging of a vessel, u I have already indicated in speaking of the condition of the skin reflexes 262 LECTURE XVII Much more do these happenings accompany a serious, though also usually transitory injury of the whole brain, which, since Hippocrates, has been termed "apoplexy" ( aTtoizXyjxrsiv — to strike down). We will proceed to the clinical description of this condition. The Apoplectic Attack The most striking symptom of the apoplectic attack is the sudden loss of consciousness, which is, indeed, sometimes preceded by certain warnings, as feeling of dizziness, paresthesias in the limbs, headache, nausea, darkening of the field of vision; the patient is restless, begins to totter, and then sud- denly falls into a lifeless heap. He lies there unconscious, usually with con- gested and swollen face and stertorous breathing. There is incontinence of urine and stools, only exceptionally retention. The limbs are all flaccid, the tendon reflexes are lost. Nevertheless, we are enabled now and then to recog- nize, even at this time, which side of the brain has been affected. For example, if when raised up and then suddenly let loose, the extremity on one side falls more heavily to the bed than the other and remains stretched out straight upon it, this is the one in which hemiplegia will later be manifested. On this side, also, sometimes the hand brought into supination falls back into pronation much quicker than on the other side. Further, the limbs on the side opposite to the lesion, when the patient is exposed, chill more rapidly than the others. Also, observation of the face may permit localization of the disease focus, since the sail-like flapping of the cheeks in expiration in the comatose patient (so-called "blowing smoke") is more marked upon one side (the paralyzed one). Occasionally it is remarked also that the head and eyes of the apoplectio patient are directed toward one side, and when the face is brought to the front again it always returns to this forced position. This is Vul plan's "con- jugate deviation" ("deviation conjuguee de la tete et des yeux"). This, also, can well be applied in the localization of the disease focus, since the prostrate and relaxed apoplectic turns his head and eyes to the side of the body un- affected by the hemiplegia.* We have already spoken of the absence of the abdominal and cremaster reflexes on the hemiplegic side and its importance as an early symptom. The temperature of the body usually falls below the normal in the apo- plectic attack, to rise later to a febrile degree. When the course is favorable it returns to normal after some variation ; a continued mounting of the fever is very unfavorable prognosticallv. It depends so very frequently upon broncho-pneumonic processes, that many neurologists would make trophic dis- turbances in consequ'ence of the cerebral attack responsible for the rapid occurrence of these last. "Acute decubitus," developing occasionally imme- diately after the ictus (in the region of the sacrum), an ominous sign, encour- ages a similar hypothesis. The apoplectic coma usually lasts some hours, but unconsciousness for * In contradistinction to this, patients who show unilateral convulsions from irritative cere- bral lesions, in the ease of conjugate deviation, look toward the convulsed extremities. ENCEPHALORRHAGIA AND ENCEPHALOMALACIA 263 several days is nothing' unusual. The longer this condition, which must be considered as a sort of concussion of the brain, lasts the worse in general is the prognosis. It is particularly unfavorable in cases in which even dining the coma the tendon reflexes are exaggerated, the muscles spastic; previous experience justifies the diagnosis of the excessively dangerous breaking through of the blood into the lateral ventricle, under these circumstances. Sometimes a short, so-called "stage of reaction" pi'ecedes coming out of the coma; during this the apoplectic becomes restless, sweats profusely, is somewhat delirious, complains of headache, etc. When these general symp- toms have passed away and the patient is again conscious, there follows, by degrees, the change of flaccid into spastic hemiplegia introduced by the return of the tendon reflexes and their exaggeration upon the paralyzed side. The Babinski phenomenon appears o\\ this last, not at all infrequently, already in the coma; in traumatic unilateral cerebral hemorrhages I have been able many times to elicit it, even in the hours oust after the accident. With the develop- ment of spasticity there is often a rimiUiJjon of the extent of the paralysis. Indeed, not so very rarely, the disturbance of motility may disappear in relatively short time. This is the case when the hemorrhage does not directly sever the cortico-spinal tracts, but has occurred in their neighborhood, for example, in the lenticular nucleus. The compression to which the neighbor- hood of the hemorrhagic focus is exposed for some time, the collateral (edema, etc., are to be held responsible in such cases for the indirect symptoms of inter- ference witli the motor tracts. Only the pareses which persist 6 to 8 months after the stroke are to be considered as direct focal symptoms, and prognosti- cally to be estimated accordingly. This restitution may depend upon the disappearance of the so-called "Diaschisis." By this expression — since the fundamental work of v. Monakort — we denominate as sort of passive shock: the absence of stimuli which are connected with the destroyed nerve tracts paralyze, also, anatomically intact regions, which in their activity have adapted themselves to these stimuli. The action of this diaschisis is in its nature fleeting, temporary, not residual. Pathogenesis and Etiology of Cerebral Hemorrhagic Foci and Areas of Softening Since now we have studied, from an exclusively symptomatologieal point of view Hie typical clinical pictures of capsular hemiplegia and of the apoplectic attack which accompanies it, as a rule, il is now our task to subject to a systematic description Hit' different pathological conditions which lead to en- cephalorrhagia and encephalomalacia. 1. Arteriosclerotic changes hi the brain vessels take the first place in the anatomical substratum of cerebral hemorrhage. We can here refer to the general remarks which we made in Lecture W (page 224), in speaking "I cerebrospinal arteriosclerosis. We would emphasize again that between ar- teriosclerotic changes of the peripheral arteries and those of the cerebral ves gels there is no necessary parallelism, and that a high degree of alteration of the one gel iii no way excludes the good condition of the other, and vice versa. 264 LECTURE XVII Particular interest, however, from the point of view of the pathogenesis of cerebral hemorrhages is presented by the characteristic lesions whose exceed- ingly frequent occurrence in the small and medium-sized cerebral arteries (par- ticularly, however, in the lenticulo-striate artery) Charcot and Bouchard pointed out in 1868; the "miliary aneurisms'" which usually are a "sacciform" subvariety of vascular dilatations, have at most a caliber of 1 mm and are often found in very great numbers. Having been produced by the influence of permanently increased blood pressure (here contracted kidney and hypertrophy of the heart, frequent ac- companiments of arteriosclerosis play a very great role), they are exposed to the danger of bursting upon any sudden further increase of the blood pressure. In this sense act all influences tending to produce congestion, which either increase the arterial blood supply to the brain or hinder its venous return. On this account the following physiological conditions furnish the most fre- quent exciting causes of cerebral hemorrhages: Digestion, defecation, coitus, psychical excitement, overexertion of any sort. As to traumatic apoplexies, which occur, for example, after a fall on the head, it is to be remarked that they usually affect arteriosclerotic individuals, that also rupture of a brain vessel from the action of external force, as a rule, presupposes a morbid brittle- ness of the cerebral arteries. The apoplexies depending upon arteriosclerosis usually occur beyond the fortieth year. Many apoplectics, long before the occurrence of cerebral hemorrhage, are distinguished by the so-called "Hab- itus apoplecticus." They are thick-set, somewhat corpulent individuals, with short necks, red faces, and visibly swollen and tortuous temporal arteries. The pathologico-anatomical picture of encephalorrhagia is, according to the stage in which the patient comes to autopsy, a varied one. In fresh hemorrhages a more or less extended (usually walnut-size) black-red coagulum is found. About this the brain substance is broken up and infiltrated with blood. In somewhat later stages the saturation of the neighborhood with cedematous fluid, colored citron-yellow from admixture of blood-coloring mat- ter, is characteristic. Later, the following changes occur: Contraction of the blood clot, liquefaction and absorption of the destroyed brain substance, reactive proliferation of the glia at the periphery of the focus. If the last has become small, there results finally, as a residuum of the hemorrhage, an ochre-colored "apoplectic scar." If it is a large extravasation, an "apo- plectic cyst'' filled with serous fluid is left behind. The most important sec- ondary lesion of capsular hemorrhagic foci is descending degeneration of the pyramidal tracts. Much more rarely than to rupture of a vessel, arteriosclerosis leads to thrombotic closure of one of the brain arteries. In such cases the obliteration is introduced by a specially intense fibrocellular proliferation of the intima. 2. Si/philitic Endarteritis Obliterans. — In this vascular disease, studied by Heubncr, Fricdl/indcr. and others, there is inflammatory infiltration of the intima with round cells, as well as considerable increase of the endothelium. By the intrusion of newly formed vessels from the vasa vasorum, there is pro- duced within the diseased brain artery an organized granulation tissue, which (occasionally with the addition of thrombotic blood coagulation) finally com- EXCEPHALORRHAGIA AND EXCEPHALOMALACIA 265 pletely occludes the vessel lumen and interrupts the blood stream. Now, if no collateral circulation occurs (and in the "end arteries" of the brain this last is excluded), the brain area, cut off from its nourishment, undergoes ischemic necrosis and softening. Fresh areas of softening are white or, in consequence of infiltration by blood from the vessels of the. healthy neighbor- ing tissue, red in color. Older foci take on a yellow or brown color, on account of alterations in the blood pigment. Syphilitic endarteritis affects on the average younger individuals than arteriosclerosis. It occurs (upon a hereditary basis) even in children; most of the cases, however, are in the third and fourth decades of life. Sometimes it leads to rupture of the dis- eased vessel instead of to obliteration. All hemiplegias in young individuals are directly suggestive of syphilis. Once in a way other infectious diseases also lead to affections of the intima of the brain arteries, in which there is sometimes formation of a thrombus in the diseased vessels; the very rare encephalomalacias in typhoid fever, diph- theria, etc., are thus explained. 3. Cerebral Embolism. — This variety of vascular occlusion depends upon the lodgment of a coagulum originating somewhere else in the organism in an artery of the brain. The place of origin of the emboli brought to the brain is usually the left heart; the diseases responsible for their production are vegetating or ulcerous endocarditis of the aortic and mitral valves, further heart aneurisms and certain cases of heart weakness in which thrombi are formed in the recess or in the auricles of the left heart, and later portions broken off and thrown into the circulation. Also arteriosclerotic disease and aneurisms of the aorta and of the carotids occasionally furnish the material for brain emboli.* These last, in by far the majority of cases, are caught in the Syl- vian artery, occlude it, and effect destructive softening of the basal ganglia and the internal capsule — while in a remarkable manner the cortical regions supplied by this artery often escape destruction through collateral circulation. The lift Sylvian artery is more frequently affected than the right. f It is usually young individuals in whom brain emboli occur. As exciting factors those producing elevation of blood pressure, like in encephalorrhagia, play an unmistakable role: defecation, coughing, vomiting, overexertion, psychical ( xcitement, coitus, etc. Differential Diagnosis Between Hemorrhage mid Softening It is sometimes an exceedingly difficult task to form the decision as to whether an apoplectic attack or a cerebral hemiplegia depends upon a hemor- rhage or is the consequence of an embolic, thrombotic, or endarteritic vascular occlusion. As guiding principles in this differentia] diagnosis, flu- following points may be of use : * As to septic emboli from il»- pulmonary vein (in gangrene of Hit- lung, etc.), see Brain Misccss, i.ecturc MX. t As to embolism and thrombosis of the basilar artery ("acute apoplectic bulbar, paralysis"), see Lecture V 1 1. 266 LECTURE XVII 1. Embolism and syphilitic endarteritis usually affect younger, arterio- sclerotic thromboses as well as cerebral hemorrhages, older individuals. 2. Heart murmurs speak for embolism; it is to be remarked, however, that quite frequently with the appearance of the apoplectic attack, heart murmurs previously present, disappear, and do not reappear for some time. Hyper- trophy ox the left ventricle turns the balance in favor of hemorrhage. 3. The urinary findings characteristic for chronic interstitial nephritis (increased quantity, small amount of albumin, hyaline, and granular casts, a very few leucocytes) speak rather for hemorrhagic apoplexy. Contracted kidney occurs in about 30 per cent, of the cases of cerebral hemorrhage, while blood casts (an expression of a hemorrhagic kidney infarct) point to embolism. 4. In a small number of cases, examination of the eye grounds can furnish diagnostic aid, namely, when hemorrhages into the retina, albuminuric retinitis, or embolism of the central artery of the retina, can be found. The last speaks for an analogous process in the brain, the two first anomalies for cerebral hemorrhage. 5. The forerunners of the apoplectic attack (described on page 262) are of much longer duration in thrombotic or endarteritic vascular occlusions than in hemorrhage. In embolism they are but inconsiderable, or are absent entirely. 6. In the attack itself, congestion of the face makes probable a cerebral hemorrhage; its pallor, a closure of a vessel; nevertheless, these rules admit of exceptions which are in no way rare. The fall of the temperature at the start is nearly always absent in vascular occlusion. Clonic spasms speak decidedly in favor of embolism, particularly when they are unilateral. Con- jugate deviation with relaxed limbs is characteristic of hemorrhage. Coma sometimes is absent in autochthonous vascular occlusion, and when present is, as a rule, of shorter duration, but deeper than in embolism; very pro- found and long-continued coma, however, indicates bursting of an artery. The seizure clears up most slowly in this last lesion. 7. Accompaniment of a right-sided hemiplegia appearing after the ictus by aphasia (see Lecture XVIII) speaks in general for cerebral softening rather than cerebral hemorrhage. A complete, or almost complete, disap- pearance of the hemiplegia or an only partial development of this (for ex- ample, brachial monoplegia) speaks in the same sense. Apoplexies which leave behind a hemiplegia lasting only one or several days should always arouse a suspicion of general paresis. In young individuals there come also into question, as already said (see Lecture VIII, page 1-iO), the apoplectiform attacks of multiple sclerosis. Prognosis The clinical distinction between the several anatomical substrata of apo- plexies and hemiplegias is not only of scientific interest, but also of great importance prognostically. Now, the decision that an endarteritic obstruction has arisen upon a syphilitic basis, arouses the hope that by commencing an energetic anti-syphilitic treatment a recurrence will be prevented, while arterio- ENCEPHALORRHAGIA AND ENCEPHALOMALACIA 267 sclerotic thrombosis gives in this respect a very dubious outlook; repeated attacks are here the rule; in many cases there are, finally, bilateral hemiplegias, pseudo-bulbar paralytic phenomena, deep dementia, marasmus, etc. After passing through a cerebral hemorrhage the patient, while not safe from recur- rences, is much less threatened by them. Also, in contradistinction to arterio- sclerotics with brain softening, his intelligence remains intact after the seiz- ures. After embolism there are no further attacks, as a rule. Decisive for the prognosis as to life is here the underlying disease. Further, the paralytic symptoms resulting from cerebral hemorrhages, according to experience, show a much greater tendency to disappear little by little than those from foci of softening; this is connected with the fact that there the indirect and distant symptoms play an important role, here a very minor one. The defect symp- toms which persist about a month after the occurrence of embolism or throm- bosis can usually be considered as final, while in hemorrhage a permanent con- dition can only be assumed after 6 or 8 months. If there is decided regression of the paralysis immediately after recovering from a sanguineous apoplexy, this is prognostically very satisfactory. The appearance of contracture, on the other hand, excludes finally the hope of complete restoration. A very important prognostic question is as to whether the patient will survive the apoplectic attack or not, in case he does not die at once ("apo- plexie foudroyante"). That a spastic condition of the muscles from the start clouds the prognosis, we have already said; also, we have emphasized the un- favorable indication of acute decubitus. Further, very bad symptoms are, long duration of the coma (over 24 hours') ; its increasing depth (ingravescent ftpoplexy); the occurrence of a pneumonia; finally, "CheyTie-Stokes respira- tion" (a periodical increase, then decrease, of the depth of inspiration which occasionally can lead to complete intermissions in the breathing). Treatment In fresh apoplexy, after we have taken care that the patient is put to bed, avoiding all jarring (with careful supporting of the head), and all tighl clothing has been loosened, in our further proceedings the differential diag 1 - ttosis between hemorrhage and vascular occlusion, into which we have already entered quite thoroughly, are of importance; besides, the condition of the heart and the vessels must always be taken into consideration. The time when every one affected by an apoplectic stroke was bled schematically has gone by. We consider blood letting as indicated only when it must be assumed that there is cerebral hemorrhage, the face being re d, the pulse tense. In many eases the application of leeches- behind the mastoid processes suffices; when the carotids are very full of blood and there is great congestion of the face, however, withdrawing from 125 to 250 CC of blood (venesection or puncture of the cubital vein) is entirely in place. On the other hand, if the clinical picture, in agreement «ith the previous history, speaks for embolism or throm- bosis, if the face is pale, the pulse is weak or intermittent, we should refrain from bleeding, and must make Use of stimulants. As such, there come into Consideration subcutaneous injections of ether, acetic ether, camphorated oil. 268 LECTURE XVII or caffein, as well as oxygen inhalations. Tutting an ice-bag upon the head of the apoplectic is a measure sanctioned by tradition ; the possibility that in cerebral hemorrhage it acts through the skull in promoting hemostasis is, indeed, very problematic, though it is not entirely excluded; in embolism and thrombosis, however, it can in any case do no harm, so that we need not object to this ordinary measure, which gives a certain satisfaction to the friends. Adrenalin injections are to be avoided in cerebral hemorrhage; the disadvan- tage of the considerable increase of blood pressure which they cause outweighs the possible advantages of a vasoconstriction (which, if we draw conclusions from animal experiments, is least to be expected in the brain arteries). That the injection of ergot preparations is able to stop the cerebral hemorrhage is very questionable; nevertheless, there is no objection to trying them. From the start we should exert our efforts toward the avoidance of the dangers of acute decubitus and pneumonia. From the point of view of both of these it is to be recommended when the coma lasts longer than 2 or 3 hours, to turn the patient on his side (naturally very carefully and with proper assistance), and when it is necessary, after a few more hours, to change him to the other side. Eventually pneumatic or water cushions. The skin exposed to bed-sores is bathed from time to time with alcohol. Further, it is well, with a wad of cotton on the end of an applicator, to mop out the mucus which tends to collect in the pharynx, from time to time, or to promote its being swallowed by encouraging the swallowing reflex. The retention of this reflex almost without exception, as a rule, permits giving the comatose apoplectic some nourishment without having to use the oesophageal tube; naturally only small portions must be given at one time (beef-tea, egg-nog, "hvgiama," meat-juice, olive oil, etc., a tablespoonful at a time). Also analeptics and expectorants; for example, strong coffee with aromatic spirits of ammonia, can be given as needed from time to time, by the mouth. When there is retention of urine, we should not forget to cathe- terize at proper intervals; in the more frequent incontinence, the utmost cleanliness should be observed. Enemata are needed in most cases ; for "de- pletion by the intestine," instead of the usual injections (soapsuds, glycerin, etc.), enemata of sulphate of sodium or sulphate of magnesium solutions can be tried. (After emptying the bowel, a nutrient enema, consisting of gruel, yolk of egg and peptone may be given.) If it is desired to act depletingly not only through the intestine, but also by the skin, hot packs to the lower extremities are simplest; more energetic effect may be obtained, however, by the application of mustard plasters to the calves. In apoplexies of traumatic origin, with persistent or ingravescent coma, surgical interference (puncture, or even incision into the hemorrhagic focus) has been practiced occasionally with fortunate outcome. In the stage of reaction we should not fear to combat the often great jactitation by injections of morphine. If the patient regains consciousness, it is our first task to explain to him the situation, and to instill into him hope and equanimity .by pointing out the improvement that is to be expected. To obtain for him good nights he should be given the alkaline bromides, chloral, codein, veronal, trional, etc. For the rest, however, with the excep- ENCEPHALORRHAGIA AND ENCEPHALOMALACIA 269 tion of cases of syphilitic etiology (in which we usually give iodipin injections of 10 to 20 cc of a 25 per cent, solution in the first days after the attack, and begin soon after with injections of biniodide of mercury — see Lecture XIII, page 210), it is better to avoid further medication. Only after about 1-i days the arteriosclerotic apoplectic is allowed to begin a course of iodide of potassium, as we have described it in Lecture XV. Naturally, the therapeutic and special dietetic indications given for arteriosclerosis cerebro-spinalis apply to the later treatment of these cases in the fullest degree. During the earlier days after recovery from an apoplectic attack, the patient is kept on a liquid and semi-liquid diet (farinaceous soups, purees, compotes, pap, etc.), and it is sought to procure regular stools without straining, by mild purgatives (cas- eara, purgen, pil. rhei, comp., pulv. glycyrrhiz., etc.). A few days after the patient has regained consciousness very careful pas- sive movements of the paralyzed limbs, with gentle massage of their muscles, is begun. Above everything, it is necessary to oppose, as far as possible, the formation of contractures and faulty positions of the extremities; on this account we should see to it that the foot is kept continuously in a position at right angles to the leg, and that the arm and the fingers lie as far as possible stretched out (see Lecture III, page 54). Only after 2 or .'5 weeks should electric treatment be begun, also (and apart from faradization of the anesthetic skin regions with the wire brush), exclusively, galvanism. Stimulating the muscles by the faradic current is contraindicated. By the comparison of otherwise analogous cases of cerebral paralyses which have been treated b}' faradism, by galvanism, and without any electro-therapy, one may at any time convince himself that the faradization of the muscles can favor the development of hemiplegic contractures. The constant current appears, on the other hand, when it is used particularly to stimulate the extensor muscles in the upper, the peronei and flexors of the knee in the lower extremity, not only to encourage voluntary motor innervation (to open up new paths — "balmen"), but directly to oppose the occurrence of contractions. When motion has returned again in the leg, the patient can be gotten out of bed, at first for a very short period. For a number of weeks, however, In- should spend the greater part of the day ill bed, as too early and too frequent attempts at walking usually favor the development of contractures. Only very gradually should long remaining up and going about be permitted. The arm should, at the start, be kept in a sling in order to prevent swelling and cyanosis of the dependent hand as well as dragging upon the joint, which last can favor the development of arthropathies. Now, salt baths of gradually {DCreasing concentration may he given, provided thai they are neither too fold nor too hot 34 to :i'r, *'. (93 to !>.-. F.). During the bath, cold Compresses are applied to the patient's head to avoid congestion. In the bath, systematic exercises, never pushed to fatigue, are undertaken; soon, also, outside the bath. In these less stress is to be laid upon the development of itrength than upon the practicing familiar acts (eating, buttoning and mi buttoning the clothing, writing, etc.). Bui rarely orthopedic after-treatment of hcmiplcgic paralyses and eon fractures comes into question. At most, it is a matter of supportive apparatus 270 LECTURE XVII with arrangements for extension ; there is scarcely ever any indication for bloody interventions, as tenotomy, tendon transplantation, etc. (Supplementary.) Atypical and Extracapsular Hemiplegias Gentlemen: It is necessary now to call your attention to some varieties of cerebral hemiplegia, which are separated more or less widely, pathologico- anatomically and semiologically from the typical capsular hemiplegia with whose consideration we have commenced this lecture. If in this task I do not strictly confine myself to cerebral hemorrhages and cerebral softening, never- theless this bringing in of other pathologico-anatomical conditions is amply justified from the point of view of differential diagnosis. 1. Cortical Hemiplegia. — This depends chiefly upon embolic or thrombotic processes, though it can also occur from tumors, diseases of the meninges and the bones of the skull. It is, on account of the great extent of the cortical motor zone, usually incomplete, that is, sparing some portions of the limbs. When it begins in an apoplectiform manner it usually causes conjugate devia- tion (see page 262). The combination with cortical aphasia (see Lecture XVIII) is very frequent. The so-called "intra-cortical" hemiplegia presents a variety of cortical hemiplegia. Spielmeyer has shown, namely, that chronic diseases and resulting atrophies of the cerebral cortex (for instance, in epi- leptics) entirely isolate the (for the rest intact) pyramidal tracts from the other cells and cell associations of the cortex, and can hence paralyze them. Intra-cortical hemiplegias arise naturally, never in an apoplectiform manner, but are always chronic and progressive. 2. Peduncular Hemiplegia. — Hemorrhage into one crus cerebri (into the crusta or ventral portion) produces (as do also tumors or aneurisms of the same region) the so-called Weber's symptom-complex, or alternating oculo- motor hemiplegia. In this, there is found on the side of the lesion, a paralysis of the oculo-motor; on the opposite side, however, a paralysis of the face and the extremities. This occurs from the fact that the root fibers of the third nerve pass close to the pyramidal bundles which are proceeding distally in the ventral portion of the crus, but do not cross until lower down, and are destroyed in common with these latter. 3. Pontine Hemiplegia. — If the lesion is situated a little farther distally, namely in the lower third of the pons, there results from this another alternat- ing hemiplegia, the so-called "Millard-Gubler symptom-complex" (alternating facial hemiplegia). On the side of the lesion the facial, on the opposite side the limbs, are paralyzed. The explanation is furnished by the following anatomical relations : the pyramidal fibers intended for the facial undergo decussation in the middle third of the pons, while the rest of the pyramidal fibers only cross the middle line distally from the pons. A lesion in the lower third of the pons varolii hence affects, along with fibers for the contralateral extremities, those intended for the facial muscles of the same side. 4. Hemiplegia Cruciata, an excessive rarity, can be produced by hemor- rhages into the medulla oblongata, when these are located laterally at the pyramidal crossing through which the tracts for the arm are affected before, EXCEPHALORRHAGIA AXD ENCEPHALOMALACIA 271 those for the leg after, passing over to the opposite side. The paralysis then affects the contralateral arm and the homolateral leg. 5. "Lacunar Hemiplegia" has already been mentioned in Lecture XV, when discussing arteriosclerotic brain diseases. This form of hemiplegia is charac- terized, as was there emphasized, first by great capacity for rapid restitution and slight tendency to the formation of contractures, on the other hand, how- ever, by a manifest tendency to new attacks in the originally unaffected hemi- sphere. In the last instance paretic spastic disturbances occur then, also in the bilaterally innervated muscles (see above, page 258), which manifest them- selves in pseudo-bulbar phenomena. Sensibility and skin reflexes are scarcely disturbed in this form ; also, when it begins suddenly, the ictus is not severe, consciousness is usually preserved or but slightly clouded, eventually loss of consciousness, but of only very short duration. 6. The So-called "Hemiplegia Sine Materia." — Cerebral hemiplegias have now and then occurred without any lesion in the central nervous system being discoverable at the autopsy. Many times it has been in nephritics who died in uremia. The uremic poison seems here for some reason entirely obscure to us, to have acted in producing one-sided paralysis. The older authors thought of one-sided vascular spasm and spoke of "serous apoplexy." Other cases of so-called hemiplegia sine materia exposed in the older literature, however, are probably to be considered as unrecognized lacunar hemiplegias. 7. The So-called "Homolateral Hemiplegia." — There are found in the literature some observations in which the disease focus has been found, not on the opposite side, but upon the same side as the hemiplegic paralysis. Usually there have of course been mistakes in clinical or pathologico-anatomical ob- servation: so, namely after embolism, as already said, spasmodic movements mi v occur in the paralyzed extremities immediately after the ictus; these may be wrongly interpreted as signs of voluntary muscular power, while the con- tralateral extremities, in consequence of the coma, lie motionless and arc considered to be paralyzed. Further, on autopsy a gross lesion of one hemi- iphere, which, however, does not involve the motor tracts, may so fix the at- tention of the examiner that he overlooks inconspicuous foci located in the pons or medulla of the other side. However, after eliminating these cases erroneously designated as homolateral hemiplegia, there still remain a few unassailable observations: on several occasions an absence of the pyramidal decussation, a relatively rare fiber anomaly, has been found in such cases. LECTURE XVIII Aphasia, Apraxia and Agnosia Gentlemen : In our lecture to-day we will occupy ourselves with the ex- ceedingly instructive and interesting disturbances, which can appear in dif- ferent brain diseases in consequence of the more or less elective destruction of a definite "memory." Where it is a "memory" necessary for the speech ar- rangement, we see an aphasia result from its elimination; if there are dis- turbances of associated memories, affecting movements adapted to an end but not in the service of speech, the clinical picture of apraxia occurs; if, finally, the recognition of objects of any sort is interfered with or rendered impossible, we speak of agnosia. In each of these phenomena, however, we have to con- struct a large number of symptomatological sub-groups, which, for the deter- mination of the seat and the nature of the underlying pathological processes are in part of decisive importance. A. Aphasia Already, in 1825, the French clinician Bouillaud emphasized the difference which existed between a paralysis of the speech mechanisms and another con- dition in which these muscles had suffered loss, not in their power to act in itself, but only in their applicability to the service of forming words. Eleven years later Marc Dax, based upon the frequent coincidence of such a loss of speech with right hemiplegia, placed the seat of "word memory" ("memoire verbale") in the left hemisphere. The anatomical proof of the general correct- ness of this view was furnished first by P. Broca ( 1801-1865), in that he showed that the motor speech center was to be sought in the foot of the third lower frontal convolution on the left side. Further, he recognized exceptional cases with right-sided location of this center. Soon after, observations on a different sort of aphasia, in which the lesions occurred in the left temporal lobe and which were characterized not by the impossibility of forming words, but by substitu- tions, leaving out and mutilation of words ("paraphasia") and jargon, were made known. The English neurologist Charlton Bastian, laid stress on the fact that these patients also presented disturbances in the understanding of words, in that they perceived what was spoken to them only as noise, but could not understand it as speech. Finally, in 1874, Wernicke proved that this last phenomenon, which he called "sensory aphasia," occurred through the de- struction of a "center for the memory of sounds" in the superior left temporal convolution, and that the accompanying paraphasic phenomena are explain- able from the loss of the regulating influence which this "Wernicke's zone (as we call it to-day), exercises upon Broca's convolution. •272 APHASIA, APRAXIA AND AGNOSIA 273 Newer investigations make it exceedingly probable that Broca's center is not 1 mited to the foot of the third frontal convolution, but extends to the neighboring parts of the Island of Rcil, the second frontal convolution and the precentral gyrus. Further, v. Monakow has shown that Broca's center cannot be considered simply as a depot for "kinesthetic" memory pictures* for the synergies necessary for speech. The Zurich neurologist demands with ju,sti( . a greater consideration of the dynamic factor along with the anatomi- cal one, in the valuation of facts in the aphasia question. In this he stands upon the ground of the so-called "Diaschisis theory." This last assumes that when any part of the cortex of the brain is eliminated, not only this itself is inhibited in its function, but also a sort of shock-like inhibition is exerted upon other cortical areas connected with it. This shock is, however, not to be con- sidered as active; rather does the absence of stimuli from the destroyed center simp] affect a passive paralysis of those cortical regions which have regulated them lv»8 in their activity by these stimuli, but which, through the new con- ditions, have suddenly been put out of setting. This "Diaschisis" action is in its i nee of temporary nature.- In the extended Broca's region lies now a place of production of motor aphasia, in that, this part of the cortex acts as director over other cortical regions, which probably distributed through large portions of both hemispheres affect the proper carrying out of the acts of speech. Hence, lesions of Broca's zone alone (chiefly traumatic) produce motor aphasia only temporarily and in the restitution of the most important speech functions in such cases, the passing away of diaschisis is to be per- ceived — the cortical apparatus coming into question learns in time to work even withou' the regulation of Broca's center. Before v. Monakow, the disap- pearan ■ of aphasic phenomena was explained in general by the vicarious action of symmetrica] parts of the other hemisphere (to a certain extent, "reserve fcenters" iews appear to me, however, to indicate an important progress. This last can in no way be said of the attempt of P. Marie to deny to Broca's zone any significance in the production of motor aphasia. In the r i cases of Broca's aphasia in which the autopsy disclosed no disease of tin lot of the third frontal convolution, there may have been very fine alterations, for example, senile cortical atrophies, only to be discovered microscopicall if not purely functional aphasias; there is, for example, a hysterical aphi ia studied by Marinesco among others. As to the still rarer observations in which clinically there was no aphasia but Broca's cortical area on autopsy is found destroyed, we by no means need to overturn the whole mroca-Wernicke localization theory on this account. The following reflection suffices: The left-handed person has his speech sphere as Broca emphasized, on the right side. Nevertheless* he has seen left-handed people also become botor-aphasic when the left lowest frontal convolution was destroyed. Even so, is the left-sided cortical localization of the psychical speech mechanism in light-handed people a rule, which does not exclude certain exceptions, and on this account it is no wonder thai right-handed people sometimes have not be- * Thai is, memory pictures, which render j>< i---.il i]»- the repetition of formerlj carried oui analogous movements. 274. LECTURE XVIII come aphasic after destruction of the left lowest frontal convolution: — they had had, probably, their speech regulating centers exceptionally developed on the right, since in general an originally bilateral plan for this cortical sphere- can be assumed with the greatest probability. In destruction of Broca , s con- volution there may have been aphasia, which later, however, disappeared. , that the person making the autopsy if he has no accurate information as to the past history, registers a lesion in the third frontal convolution in a | not aphasic. THE PATHOLOGICAL PHYSIOLOGY OF THE APHASIC PHENOMENA I beg you now to carefully observe Fig. 82. You will note in it the fol- lowing: The center for motor aphasia is connected not only with the cortical centers for the lip, tongue, and larynx muscles ; that for sensory aphasia not only Fig. S2. The Cortical Speech Centers and Their Connections. B = Brora's Center. W = Wernicke's Center. L = Reading Center. S = Writing Center. a = Third (lower) Frontal Convolution, li = First (upper) Temporal Convolution. c = Anterior Central Convolution, d = Angular Gyrus. with the auditory cortical zone, but these two cortical regions communicate by association fibers, 1, with one another, 2, with the higher psychical centers of the frontal region (the so-called "conceptual center"), 3, with the cortical apparatus which render writing and reading possible. The left angular gyrus APHASIA, APRAXIA AND AGNOSIA 27.5 presents a reading center proper, the memory field for the recognition of letters, whose destruction produces alexia, word blindness. The writing center, on the other hand, contrary to the former view, coincides with the "hand and finger" center of the anterior central gyrus. The network of association fibers indicated schematically in our figure comes into consideration for those psychical functions which we can designate shortly as "internal speech." Under this designation we understand everything which must go on beneath the threshold of consciousness in our brains, before we translate a thought into words and project these words outward by way of mouth or writing, or before we can take other cognizance of spoken or written expressions. This internal speech develops ontogenetically, in that the BROCA'S CENTER. SPEECH MUSCLES CENTER. Seat of a Cortical Motor Aphasia ( \, Blocks 1. -. ■). and 1) and of a Sub-Cortical Motor Aphasia (B, Blocks only t). child first repeats words heard, then connects a concept ion with them, then upon the emergence of tin's conception reproduces tin- words himself, later, on learning to write and read, 'connects definite symbols with the individual sounds, etc. Of decisive importance for the understanding of the aphasia question, is now the fact, that all the association fibers in tin service of internal speech run through the brain cortex, while those neurones serving external speech make their way through the medullary substance. In Fig. 88 you see this schematically indicated, and learn from it that a cortical lesion of Broca's tenter (A) musi produci more symptoms than a subcortical one (B). By 276 LECTURE XVIII the first, the association net-work for internal speech is broken, by the last, however, in no way touched, so that subcortical motor aphasia indicates a pure aphasia an exclusive suppression of external speech, a simple "word dumb- ness." Mutatis mutandis in the same manner cortical sensory aphasia differ- entiates itself from the subcortical pure "word deafness." When now, we turn to the study of the individual aphasic symptom-com- plexes we will proceed according to the following pkui : 1. The four chief forms of Aphasia. (a) Cortical motor aphasia, Broca's aphasia. (b) Subcortical motor aphasia, pure word dumbness. (c) Cortical sensory aphasia, Wernicke's aphasia. (d) Subcortical sensory aphasia, pure word deafness. 2. Other forms of Aphasia. (a) Total aphasia. (b) Conduction aphasia. (c) Transcortical aphasias. (d) Aphasias of "single senses." 3. Other symptom-complexes resembling the aphasic phenomena. (a) Alexia. (b) Agraphia. (c) Amusia. (d) Amimia. CORTICAL MOTOR APHASIA, BROCA'S APHASIA Through the destruction of the motor cortical zone of Broca, it becomes impossible for the patient to translate conceptions into words. Indeed, such a patient can emit sounds and his speech muscles are not paralyzed like those of one with anarthria as a result of bulbar paralysis ; still their regulated co- operation necessary for speech has become impossible. The French language can express the difference between this expressive speech disturbance of the motor aphasic and the articulators speech disturbance of the anarthric in much shorter and more complete fashion than the German : "1'aphasique ne sait plus parler, 1'anarthrique ne peut plus parler" (the aphasic does not know how to speak any longer, the anarthric cannot speak any longer). There are graduated differences, hence, a quite considerable variety of clinical pic- tures occurs. Either the patient has entirely lost the power of speech, has re- tained a few stereotyped expressions, or, in slight cases, there has remained to him a rudimentary ability of emitting speech, so that, for example, he uses all verbs in the infinitive ("negro fashion") or even leaves them out ("dispatch style"). Along with the loss or the disturbance of speech, writing is also disturbed to a corresponding degree. Abi'ity to copy can, however, be retained, as a tracing of the letters which is accomplished without the assistance of memory pictures. Reading, on the other hand, is always more or less impaired and often APHASIA, APRAXIA AND AGNOSIA 277 special methods are needed to make evident this disturbance. So, for example, printed words «in be understood, written ones, on the other hand, cannot ; or the understanding of words may be suspended if (with the help of the well- known child's block alphabet) the syllables are separated and arranged at horizontal or vertical intervals. The understanding of speech appears in a general manner undisturbed ; when, however, one speaks very quickly to the patient, or uses a complicated sentence in the conversation, ofie can make out an increased difficulty of understanding. Though, however, the disturbances of speaking and writing stand out most prominently, nevertheless the de- struction of a nodal point in the cortical association net has also not left reading and understanding entirely unmolested. To have indicated this last point was namely, the service of my teacher, Dejerine. SUBCORTICAL MOTOR-APHASIA, PURE WORD DUMBNESS Here, as is visible in Fig. 82, the path between Broca's center and that for the speech muscles has been destroyed. Internal speech, reading, writing, understanding of speech, are intact. Though the patient cannot bring out the words in contradistinction to patients with Broca's aphasia, he has preserved the corresponding motor memory pictures; he can even with his fingers give tin- number of syllables in the expression which he endeavors in vain to emit. ( Dcjcrinc-IJchthrim phenomenon.) Pure subcortical motor aphasia has now certain relationships with those conditions with which we have become acquainted as anarthria, or dysarthria (Lectures VII and XV). Common to both symptom-complexes is the reten- tion of interna] speech; while, however, in anarthria the formation of sounds is disturbed, we find in pure motor aphasia that the formation of the word from the individual sounds is impaired. Of these sounds, often a large number can be emitted explosively as the so-called "word debris." For the inex- perienced, confusion may easily arise between these two conditions. CORTICAL SENSORY APHASIA, WERNICKE'S APHASIA As already said, the sound pictures rendering possible the understanding of speech, are preserved in the posterior sensory or Wernicke's speech center of the left superior temporal convolution. Destruction of this center causes, hence, no loss of the power of speaking, but of the understanding of speech, no expressive, but a perceptive disturbance. In this, the patient, indeed, hears the words spoken to him, but cannot comprehend their sense, since they no longer rouse into consciousness the conceptions corresponding to them. His native language sounds to him as a foreign language, of which he knows noth- ing, or lias learned but little, does to the healthy person. That sensory aphasia is often accompanied bv paraphasia, that such patients continually make mis- takes in speaking and instead of the words which are proper, emit other, often similarly sounding words, is connected with the following: We unconsciously first pronounce internally the word which we wish to speak by means of Wer- nicke's sound picture center. When, now, this no longer functionates, there 278 LECTURE XVIII is easily the emission of wrong words, which the patient again cannot notice, since in consequence of his sensory aphasia, lie does not understand his own words. Where the understanding of speech is not entirely lost but considerably affected, it is particularly the familiar expressions (such as: "How are you?" "What is your name?" etc.) whose sense the patient is still able to comprehend. Sometimes the patient also guesses the sense of the sentence from one char- acteristic word which he understands. If now, we vary the sense of the sen- tence while retaining this characteristic word, a corresponding modification of the answers of the patient does not occur. For example, "Have you chil- dren?" "Yes." "How many children?" "Yes." "Where are your children?" "Yes." In contradistinction to the motor aphasic who speaks little or not at all, the patient with Wernicke's aphasia usually talks a great deal, but badly. We denominate his paraphasia verbal when he confuses words (for instance, says "dog" instead of "bed") ; as literal when he confuses letters (for example, "winnow" instead of "window"), while we speak of "Jargon aphasia" when he brings out a succession of senseless syllables. In this last case there is some- times a clinging to one syllable which is repeated over and over — "persever- ation." The understanding of writing is also suspended in cortical sensory aphasia or at least impaired. In complete "word blindness" the patient can no longer read, and the letters are for him only senseless black figures upon a white ground, which, however, he may trace without understanding. Writing voluntarily or after dictation is, on the other hand, lost or impaired (this last in the form of literal or verbal paragraphia). Other symbols besides letters such patients may still understand, for example, very often numbers, and further, they can play cards and dominoes. A patient of Dejerine's who could not read the letters R. F., said instantly "Republique Francaise," as soon as the coat of arms was drawn about the two letters. SUBCORTICAL SENSORY APHASIA— PURE WORD DEAFNESS In this very rare form, of which I have so far been able to observe no case, and a knowledge of which we owe, among others, to Dejerine, Wernicke, Liep- mann and Sachs, internal speech is intact, reading and writing may be possible without disturbance and only the understanding of speech is suspended. Be- sides this, paraphasia is absent in pure word deafness since the sensory corti 7 cal center is still able to exert its stimulating and controlling action upon the motor center. This form occurs only through the cutting off of the other- wise intact Wernicke's zone from the stimuli coming from the general audi- tory center. I might here introduce an anatomical point not unimportant for localiza- tion. It has indeed been proved by autopsy that a subcortical lesion of the left temporal lobe can be the substratum of pure word deafness. Still, in- complete destruction of the sensory speech center can probably also produce pure word deafness, just as occasionally incomplete disturbances of the motor APHASIA, APRAXIA AND AGNOSIA 279 speech center can cause pure word dumbness. I assume that in such cases the association fibers between the cortical field of the cochlear nerves and Wernicke's center, or respectively between Broca's convolution and the speech muscle center, are interrupted intracortically, without on this account the function of the speech centers under consideration having been destroyed. TOTAL APHASIA We speak of total aphasia when a pathological process has destroyed the anterior as well as the posterior speech center and on this account ability to speak as well understanding of speech, and also ability to read and to write are destroyed. CONDUCTION APHASIA This designation Wernicke has introduced for certain speech disturbances which are characterized by paraphasia, paragraphia, and impairment of speech repetition. He assumes that here there is interruption of the direct communi- cation between the sensory and the motor speech centers, in which particularly a lesion in the Island of Keil would come into consideration. This clinico- anatomical hypothesis, however, has been proven untenable by v. Monakow. TRANSCORTICAL APHASIAS According to Lichtheim these arise by the blocking of the paths between the conceptional center on the one hand and Broca's or Wernicke's center on the other. Hence, a motor and a sensory variety are differentiated. In the first only voluntary speech and writing are suspended or impaired; speech repetition, reading aloud, writing from dictation are, on the other hand, re- tained. In the latter there is more or less pronounced word deafness and alexia along with paraphasia and paragraphia, but speech repetition, reading aloud and writing from dictation, may be possible, although without under- standing. With Dejerine, I consider these forms only as intermediate stages of cortical aphasias which are improving. As an ''attenuated form of transcor- tical motor aphasia" Liepmann has designated the so-called amnestic aphasia or verbal amnesia, in which finding the word has become very difficult, the suggested word, however, is at once recognized as correct and is repeated Bmoothly and perfectly. Further, chiefly substantives and verbs for bhe con- crete are not found, while the forms of speech and further inflection and declension are preserved. APHASIAS OF SINGLE SENSES Best known is the optic aphasia of Freud; for objects simply held before the patient he cannot find a name, which, however, promptly occurs to him when he is given an opportunity to observe the object through sonic other tense than that of sight, for example, to feel a spoon, to hear a bell, to taste a piece of sugar. At the basis of this disturbance lies an interruption of the 280 LECTURE XVIII connection between the centers for the recognition of objects in the occipital lobe and Wernicke's sound memory center in the temporal lobe. The existence of an optic aphasia in the strict sense of the word is, however, according to Gustav Wolff, not definitely proved — neither are the other analogous single sense aphasias (auditory, tactile aphasia). As a matter of fact, many of these observations have entirely lacked certain proof that the patients act- ually had recognized the seen, touched, heard things, and only could not find their names, also that they were free from mind blindness, mind deafness and touch agnosia. (See below.) ALEXIA This disturbance leads in slight cases to blindness for letters, in severe ones to word blindness, and can, apart from accompanying cortical aphasias, appear more or less independently. If a focus is seated in the reading center of the left angular gyrus, the alexia is accompanied by agraphia, since the association fibers to the center for writing movements in the precentral con- volution are interrupted. We speak then of "cortical alexia." If, on the other hand, the focus is subcortical, beneath the angular gyrus, so that only to the optic word pictures, which come from the visual cortex in the cuneus, the entrance is closed, there occurs "pure alexia." Also, conditions which mutatis mutandis correspond to those which are present in cortical and sub- cortical aphasias. AGRAPHIA Disturbance of the writing center, shown in Fig. 82, causes no isolated agraphia, but a paralysis of the right hand. With the left, however, such a patient may be able to write or to learn quickly to do so, provided that his Broca's, Wernicke's and reading centers have remained intact. Isolated agraphia we will indeed become acquainted with later as a component of apraxia. AMUSIA As motor amusia the inability to sing, as sensory, the inability to appre- ciate a melody, is designated. Also musical alexia or blindness for notes is usually herein included. The amusic disturbances are rare accompaniments of the corresponding forms of aphasia. The musical centers appear to be situated very close to the corresponding speech centers, but do not entirely coincide with them. AMIMIA The condition that in the patient also expression and gesture speech is lost (motor amimia) and even understanding for this has disappeared is found in total aphasia. If in this manner all possibility for comprehending one's fellow r s is excluded, we speak of asemia or asymbolia. APHASIA, APKAXIA AND AGNOSIA 281 EXAMINATION FOR APHASIA AND DIAGNOSIS OF THE INDIVIDUAL FORMS When we have before us an aphasic patient we must bend our endeavors toward making as rapid a diagnosis as possible of the form of aphasia pres- ent. After that, we will take up its symptomatological study. I would recom- mend that you proceed according to the following scheme which I have pre- pared after the manner of the botanical determinative books.* Understanding of < Speech. Retained : Mot. Aphasia - Lost: Sens. Aphasia Writing retained : Subcortical Writing lost: Understanding of writing retained : Subcortical Understanding of writing lost : Ability to repeat words retained : Transcorti- cal Ability to repeat words lost: Cortical Ability to repeat words retained : Transcorti- cal Ability to repeat words lost: Cortical In general it is, especially for the beginner, of advantage in the thorough ex- amination of an aphasic to stick to one of the schemata proposed by different investigators and to carefully register the result of the examination. I give you here for this purpose the Stewart examination schema somewhat modified by myself, which you will find useful in all cases: 1. Can the patient spontaneously give utterance to understandable words? How extensive is his vocabulary? Can he utter all words, or only some? The patient is allowed to talk spontaneously and it is observed whether lie beaks fluently, whether he misplaces words or syllables, whether he speaks in connected sentences, or talks unintelligible stuff. 2. Does he understand Hie words which he hears? He is asked to touch alternately his nose, his car, his eye, his chin, etc., in order to test his under- standing of substantives. He is then requested to whistle, to smile, to close his eves, etc., by which his understanding for verbs is determined. It should in observed if he carries out the first order correctly, then, however, continu- ally repeats the same act. even when requested to do something else. If there is no trouble in carrying out these simple orders, he is given more compli cated sentences, for example, "I beg you, when I have come around to the' other side of the bed, to touch my arm twice with your left hand." 1 .'5. Does he understand written questions or orders which are placed lie in this I ignore the problematical "conduction aphasia," and on account of it-- small prac- tical importance I have put in parentheses what is said about tin- differentia] diagnosis of the M-called "transcortical" aphasia. 282 LECTURE XVIII fore him? In testing this, simple sentences are first used (for example, "How- old are you?" "Show me your tongue." "Give me your left hand") and only after this, more complicated constructions. Further, not only hand- writing, but also print, is used and it is noticed whether separation of the individual syllables or their unusual arrangement disturbs this understanding of them (see page 277). 4. Can he write spontaneously? If his right hand is paralyzed, he may try with his left hand. It is observed whether he scribbles senseless signs. 5. Can he transcribe printed letters into written ones, and vice versa? For this test one of the so-called "letter games" with which short words like "Monday," or short sentences as "Where is the tree," are put together, or allowed to be put together, is used. 6. Can he write from dictation? 7. Can he find objects whose names he hears? A heap of objects, like a key, a piece of money, a lead pencil, a match, etc., are placed before him and he is solicited to pick up one at a time. 8. Can he repeat words which he hears? He is first tried with simple words and sentences, for example, "Cat," "Dog," "Sister," "Good morning," etc. If he has lost the power to repeat words spoken before him, it is ascertained whether the patient can sing, what is sung for him, which is sometimes the case. 9. Can he name objects which he sees? Different objects are pointed to (see under 7) and he is asked "What is that?" 10. Can he read aloud? 11. Can the patient, in case it is impossible for him to pronounce a word, give the number of its syllables on his fingers? 12. Does he understand gestures and mimic movements? Without speaking to him, he is solicited to imitate touching the nose spreading the fingers, sticking out the tongue, etc. In order not to fatigue the patient these different tests are to be under- taken not all at one sitting, but with extensive periods of rest between. In polyglot patients it is well to test the different languages separately when possible. The same thing applies to dialect and to written speech. PROGNOSIS OF APHASIA The estimation of the etiological factors is very important in the first place (whether hemorrhage, embolism, tumor, trauma, etc.). Of decisive im- portance is the intellectual condition. The onset of dementia makes the hope of disappearance of the aphasic disturbances illusory. Particular emphasis, however, is to be attached to the prognostic differences as to restoration of function which appear to exist according to the form of the aphasia or accord- ing to the localization of its anatomieal substratum. The best outlook is pre- sented by pure w r ord dumbness, while the severe cortical aphasias with com- plete loss of spoken mid written speech present little prospect of improvement. APHASIA, APRAXIA AND AGNOSIA 2«:i In sensory aphasias the prognosis is the worse the more intensely marked paraphasia and jargon aphasia are. Alexia and agraphia in improvement of Wernicke's aphasia, show themselves much more obstinate than word deafness. Treat nn nt The exercise treatment of these conditions furnishes the greatest test of patience which comes to the physician. Gutzmann properly warns impera- tively against beginning the exercises too early. We should wait at least half a year after the cessation of the stormy symptoms and the entire disappearance of all other disturbances. If exercise is commenced too early in apoplectics, there is the danger that during the exercises, in consequence of the exertion, a new hemorrhage may occur. Gutzmann usually first prescribes the exer- cises when, after one or two years, there is no further spontaneous improve- ment of speech. If they are applied earlier, it is very difficult to decide how much is owing to the exercise treatment. Gutzmann further lays the great- est stress upon slow progress, as only by this can the patient be kept in good spirits, which is absolutely necessary for the result of the exercise treatment. Sensory aphasics with speech impulsion must first practice holding their tongues, endeavoring to regain again to some extent the normal inhibition by the will. Even in the severe, prognostically unfavorable, Broca's aphasias, treatment should not be given up, since it is of great importance for the pa- tient to give expression to his will, at least by a few words, even when they are used ungrammatically. By this, the lability of mood which not rarely be- comes exaggerated to outbreaks of rage, is combated. There occurs, from the fact that the aphasic is not in a condition to give expression to his thoughts, wishes and conceptions, an internal tension, for which even a rudi- mentary ability to speak, gives an outlet. Also to this same end. such pa- tients must systematically practice gesture speech. A picture book with sim- ple representations of all the conceptions and wishes lying within the circle of thought »f the patient, should always accompany him, so that he can make known his wishes to those surrounding him by indicative gestures, though when apraxia is present, the descriptive gestures cannot be learned. B. Apraxia As apraxia* we denominate, since the monumental work of the Berlin psychiatrist Ijcjiniaini which first appeared in 1900, a disturbance in which the extremities, especially the hands, are capable of executing correct single movements, but not those movements directed to a definite end. A patienl effected with this disturbance has lost the memory of the synergies and com- hiii.il inns of single movements necessary for the proper use, for example, of a pair of scissors, I'm' beckoning, for shaking hands, etc. lie sticks a tooth- brush like a cigar into his mouth, and so on. There are hence great analogies with motor aphasia, or to better express it. aphasia from a physiological point Other names, .'is Asymbolia, Parektropia, Parekinesia, .ire applied only by ;i tew authors, ami have nut come into general use. 284 LECTURE XVIII of view can be considered a special form of apraxia, as apraxia of the speech apparatus. We distinguish three varieties of apractic disturbances : 1. IDEATORY APRAXIA The idea-plan for the composite act to be carried out is disturbed, so that the resulting unsuitable action gives the impression of extreme mental dis- traction. Liepmann cities the following typical examples: The patient sticks a match along with his cigar into his mouth instead of striking it ; in sealing a letter, puts the seal in the flame and then presses it on the stick of sealing wax; attempts to cut off the end of his cigar by wedging it between the match case and its cover. The single acts which make up these perverted maneuvers are in general correctly performed. This ideatory apraxia usually appears only in complicated movements and in proportion to their complexity. The limbs are, as a rule, equally affected ; it is not that they respond incorrectly to the will, but they receive perverted impulses from it. 2. IDEO-MOTOR APRAXIA In this form, both the plan of movement and also the limb center with its content of kinesthetic memory pictures are intact, but their connections are severed. Simple acts for whose accomplishment memories preserved in the limb centers suffice, are correctly executed ; only those requiring a great num- ber of successive movements are incorrectly accomplished, since the proper di- rections and commands are no longer carried from the ideation centers to the limb center which, to a certain extent, has become autonomous with regard to the whole brain. Liepmann calls these forms of apraxia the "ideo-kinetic," Heilbronner, the "transcortical." They can affect single limbs or one half of the body. According to Liepmann the following kinds of faulty reactions occur: a. Movements which do not resemble any purposive movements, flouri>h- ing the hand, spreading the fingers, so-called amorphous movements. b. Executing the wrong movements.* (Beckoning instead of threatening, taking hold of the ear instead of the nose, etc.) c. The movement occurs in another set of muscles ; standing stiff instead of giving the hand, which may simulate leaving out of movements. will show you the distribution of these centers. As cerebral monospasm we designate tonic-clonic spasms which affect a muscular center up to this time normal or already paretic (face, arm, hand, etc.), and leave behind them permanent paresis, or a permanent increase of an already present paresis. Jacksonian or cortical epileptic at lacks begin usually as monospasm, and. 294 LECTURE XIX as a rule, tonically. The contractions, however, do not remain limited to their starting-point, as does an ordinary cortical monospasm (to the muscles about the mouth, for instance), but they extend to the arm, and finally, also, to the leg of the same side of the body (naturally the seat of the tumor is on the side of the brain opposite to the spasm). When the attack begins in the arm, after the whole arm is affected, the face, and then the leg, become involved. As the crural type is designated the succession, lower limb, upper limb, face. The tumor sets the pyramidal cells in its neighborhood into a condition of irritability which spreads itself like waves over the surface of the water, gradually to the neighboring cortical regions. The muscles, indeed, are attacked in an order which corresponds to the arrangement next to one another of their cortical centers. In severe cases, Jacksoniun convulsions pass over also to the extremities on the other side and can be accompanied by loss of consciousness. A very intense irritation can also propagate itself through the commissural tracts of the corpus callosum, etc., to the cortical motor field of the other hemisphere, and can finally benumb the centers for the higher psychical functions (in the first place the frontal cortex). Cortical mono- plegia usually develops as a gradually increasing spastic paresis ; according to its extent, we speak of monoplegia cruralis, brachialis, facialis, facio- brachialis, facio-lingualis. Facial paralysis is limited to the lower division of the muscles, since the upper is innervated from both hemispheres so that the loss of one-sided innervation is comparatively unimportant clinically. While on account of their surgical interest the tumors of the cortical motor region a short time ago stood so much in the foreground of all brain tumors that v. Bcrgmann defined brain surgery as the "surgery of the central convolutions, 1 ' to-day the tumors of the posterior fossa of the skull are also relatively frequently operated upon. The description of their symptomatology we will reserve for the next lecture devoted to cerebellar affections. Also the most important facts with regard to the not very rare tumors of the hypo- physis of late equally attacked by the surgeons, we will put off until a later lecture (XXIV). The remaining localizations of intracranial growths indicate usually in- operability ; the knowledge of their special clinical symptom-complexes is hence somewhat less important, and we can content ourselves here with a comprehensive synopsis. Tumors of the frontal lobe often produce a marked defective intelligence, somewhat like that of progressive paralysis ; again, the so-called "Witzelsucht" ("moria") — a tendency to silly jests with loss of ethical feelings and pleasure in offensive demeanor. In affection of the lowest frontal convolution on the left side, that is, of Broca's center, motor aphasia occurs, as already men- tioned in Lecture XVIII. Occasionally paralysis or contracture of the trunk and neck muscles can be observed. Tumors on the lower surface of the frontal lobe lead, as a rule, to protrusion of the eyeball, to anosmia and to an atrophy of the optic nerve which often progresses so rapidly that a papil- ledema does not develop. Where, however, the last occurs, it is found not rarely only upon one side (that of the tumor). Tumors of the region of the optic thalamus set up lateral hemianopsia BRAIN' TUMOR 295 and (by their effect upon the internal capsule) a progressive spastic paresis of the extremities (both on the opposite side). Further, the suspension of certain '"psycho reflexes" have been described as a thalamus symptom. On unintentional laughing and crying the lower facial muscles of the opposite side remain masklike, stiff and immovable, while voluntarily they can be put in action in every manner. Finally are to be mentioned: A crossed, persistent hemianesthesia (which is usually much less marked for touch, pain and tem- perature than for deep sensibility) and exceedingly severe continual pain, which is refractory to analgesics and exacerbates from time to time ("central pain"), which is projected into the contralateral half of the body. Tumors of the temporal lobe are sometimes accompanied by sensorv apha- sia (see above, page 277), those of the occipital lobe by hemianopsia (see above, page 2.59). Also tumors of parietal situation can produce half-sided blindness by destruction of the optic radiations of Gratiolet. With the last localization the symptom-complex of alexia, already mentioned, is not very rarely observed, very frequently, however, the so-called astereognosis, that is, the inability to recognize objects by touch when the eyes are closed, although with this the elementary sense perceptions are tolerably preserved, and in the "pure" cases, which have been called "'touch paralysis," are found entirely intact. Characteristic of tumors of the corpus callosum are: Apraxia (see above, page 283), bilateral hemiparesis without increase of reflexes, and without Babinski's symptom. According to the more or less median situation of the lesion, the intensity of the paresis on the two sides is even or uneven; also, unilateral hemiparesis can be combined with contralateral motor irritative symptoms (for example, convulsions or hemichorea). In corpus callosum lesions, sensibility, as a rule, remains intact, also the functions of the cranial nerves; only in involvement of the most anterior portion of the corpus callosum the facial is found paretic. Tumor formation in the internal capsule pro- duces :i chronic progressive hemiplegia whose clinical end result closely approx- imates that of the acute form. In tumors of the region of the corpora quad- rigemina, pupillary paralysis, paralyses of the external eye muscles, ataxia, impaired hearing, visual disturbances, nystagmus directed vertically anil out- ward, are found. That the origin of a tumor pressing upon the lamina quad- rigemina is in the pineal gl^gd (epiphysis cerebri) is indicated by the occur- rence of conditions of sexual excitement, abnormal development of hair and adiposity, in young individuals, also, occurrence of a hyperplasia of the genitals and an abnormal increase of the length of the body (the so-called "dyspinealism," sec Lecture XXIV). The tumors of the base of the brain manifest themselves in general by early and marked involvement in the clinical picture of the cranial nerve toots, of irritative symptoms, hyperesthesia and neuralgiform "root pains" in the region of the trigeminus are to be specially mentioned. The irritative symptom of the motor trigeminus is trismus, the cramp of the masseters leading to grinding the teeth, that of the facial the twitching of tin face muscles. Also sensory irritative phenomena {e.g., tinnitus aurium) are not rare. The paralytic symptoms can affeel every brain nerve. The} are, as a 296 LECTURE XIX rule, multiple, but, on the other hand, usually develop only on one side. Most frequently affected are the facial, abducens, and oculo-motorius whose altera- tion, as a rule, first manifests itself by ptosis. Destruction of the pyramids is quite rare; slighter affection comes only little by little into plain view — through the Babinski reflex, spasticity, etc. Only lesions in the neighbor- hood of the crus cerebri have a great tendency to destroy the cortico-spinal tracts, so that in this location of the tumor the Weber symptom-complex hemiplegia alternans oculomotoria (see page 270) comes under observation. Papilloedema usually occurs early. If the tumor is located in the caudal divi- sion of the base of the brain, bulbar paralytic symptoms arise (see page 120). Prognosis and Treatment The prognosis of brain tumor is in general a very gloomy one. Sponta- neous improvement and recovery has been observed in aneurisms (obliteration), in cysts and serous meningitis (resorption), in tubercle, cysticercus, echino- coccus (calcification), in gumma (regression) ; the process of cure may be hastened in syphilitic new formations by anti-syphilitic treatment, also mercury and iodide cures have been of benefit in serous meningitis. In true neoplasms there are cases in which in benign tumors recovery up to a certain point has occurred through these growths undergoing calcification or ossification, very great rarities, so that death in coma or from rupture of a vessel is the nearly regular ending of the frightful disease picture (although sometimes it is only slowly progressive, and eventually interrupted by stationary periods). In most cases, hence, operative interference, in spite of the very great dangers which it presents in many instances, offers to us the only therapeutic way standing open. In principle, curable by operation are, properly, only tumors on the cerebral convexity and upon the cerebellum (provided that they are cystic, or, if solid, are well limited and can be extirpated in toto*); perhaps, also, hypophysis tumor. Very many cases can, however, derive great benefit from a pidliative operation ; this is the so-called "decompressive craniectomy." With regard to the indications for surgical interference, I would impress upon you the following: In too many cases decision as to an operation is put off for a long time to try combined mercui'y and iodide treatment (which usually has also in glioma and sarcoma a decided but unfortunately only temporary effect — probably on account of resorption of the secondary ven- tricular dropsy) until it finally proves itself necessary. Such treatment is usually ordered even in the absence of a history of syphilis, while on the other hand it should not be forgotten that former syphilitics also can have non-luetic brain affections. It is always to be remembered that direct danger to life, unbearable intensity of the headache, threatened loss of vision or de- mentia, present pressing indications for decompressive craniectomy. Babinski has properly emphasized that this indication should be accepted much more * Gliomata show such indefinite limitation from the healthy surrounding tissue and such a gradual passage over into it, that their operative prognosis, in spite of their pathologico-ana- tomical benignness, is unfavorable. BRAIN ABSCESS 297 frequently than it generally is. Even in malignant neoplasms it can act pal- liatively ; in benign tumors inaccessible to extirpation, which, however, are capable of coming to a standstill in growth, it may prevent the occurrence of irreparable destruction; in serous meningitis it may be directly curative. Where the brain pressure is immediately threatening it may be necessary to advise first operative decompression, and then a mercury cure. In tumors not exactly localizable, temporal craniectomy recommends itself, since the muscles of mastication oppose in suitable manner the occurrence of brain prolapse. If the disease is located in the posterior fossa, the occipital region is, as a rule, the place of choice. The neck muscles also affect a good closure. Less favorable conditions in this respect is afforded by parietal craniectomy which is undertaken for tumors of the motor region. B. Brain Abscess The infection lying at the base of brain abscess usually enters the organ by continuity, more rarely by metastasis. The first applies to traumatic and otitic brain abscesses which are observed after septic wounds of the skull and suppuration in the ear; in these the germs pass by the lymph channels, along the nerve sheaths, or by means of septic venous thromboses into the brain substance. Metastatic infections reaching the brain by way of the blood vessels, namely, after lung abscesses, empyemata of the pleura and putrid bronchitis, lead to cerebral pus collections. Brain abscesses can be multiple or solitary; in the last case they sometimes reach the size of an apple. Their pus contains staphylococci, streptococci, colon bacteria, different anae- robic germs, etc. Otitic abscesses locate by preference in the temporal lobe or in the cerebellum ; traumatic or metastatic, naturally show a very variable localization. The clinical symptoms of brain abscess, like those of brain tumor, may be divided into general symptoms and focal symptoms. In regard to the last, there is entire agreement between the two diseases, and also the general Symptoms show very great analogy. Only papilloedema is much rarer in abscesses than in brain tumors; in them fever, which sometimes has a septic character (with rapid rise of temperature and chills), sometimes, however, expresses itself only in moderate evening rises of temperature, plays a de- cidedly greater role That docs not mean indeed, that eases with entirely afebrile course are great rarities; on the contrary, they represent a very considerable minority which may well depend upon a relatively frequent for mation of thick "abscess membrane" which prevents the resorption of the septic substances. The encapsulation can go so far thai the abscess he Comes "latent," and either causes no longer any difficulties or manifests itself Only through the focal symptoms which have become stationary. Such a be- coming latent, however, cannot he considered equivalenl to a cure, as even after latency for years acute lighting up again of the process -till threatens. On tliis account the treatment of brain abscess (in SO far as it can lie diag- BOSed and an operatively accessible point can he localized) in general can he tuitablv and conclusively summed up in the old surgical axiom, "Where there 298 LECTURE XIX is pus, evacuate." The diagnosis, however, in those cases in which a skull trauma, suppuration in the ear or in the mastoid, a lung abscess, etc., cannot be recognized, and where fever and chills are absent, is mostly exceedingly difficult with regard to its distinction from brain tumor; indeed, sometimes an impossibility even for the experienced neurologist, or it must be made by exploratory trephining and puncture of the brain. Occasionally the differ- ential diagnosis from purulent meningitis, upon the description of which we will immediately enter, is difficult. C. Purulent Cerebral Meningitis What we have said as to the etiological factors in brain abscess applies also to purulent meningitis. It can also arise from traumatic, otitic or metas- tatic causes. The last method of origin, however, is observed here much more frequently than in brain abscess ; along with the affections of the lung and pleura mentioned, in purulent meningitis, ulcerous endocarditis, puerperal in- fection, further typhoid, pneumonia, influenza, etc., play a not inconsiderable role. A relatively frequent manner of meningeal infection by continuity is further the progressive infection of the so-called emissary veins of the skull- cap and the lymph vessels in erysipelas. Other important etiological factors are, finally, the septic diseases of the orbit, the nose, the frontal sinuses, and the ethmoid cavities, as well as the rupture of a brain abscess into the sub- arachnoid space. The often exceedingly voluminous pus collection in cerebral meningitis usually affects the convexity of the brain in such preponderant fashion that it is often directly denominated '•meningitis of the convexity.'' The clinical picture of this affection shows so great a similarity to that of epidemic meningitis that, referring to Lecture XVI, we can dispense with a thorough description of the individual symptoms and content ourselves with a summary sketch of its symptomatology. The initial symptoms of purulent meningitis are very often veiled by those of the underlying disease, for instance, of erysipelas or of purulent otitis. Where, however, they have not developed from such a primary infection, they are usually characterized by quite masked and sudden beginning. Fever is present in by far the majority of cases and of rather continued character. As a rule, the temperature varies between.38° and -K)° C. (100.4 and 104 J F.) ; apart from the initial chill sometimes occurring, many patients in the further course of the disease have marked shivering from time to time. Headaches of terrible severity and diffuse distribution are present from the start, and show themselves even in half coma by the patient frequently put- ting his hand to his head. The pulse is rapid, respiration hurried. Cerebral vomiting is only frequent in the initial stages. Stiffness of the neck is some- times developed quite early, also other conditions of muscular tension ; boat- shaped retraction of the abdomen, trismus, grimacing, contractures in flexion of the extremities, Kernig's symptom. On the other hand, general convul- sions coming on in attacks, are rare, more frequent, localized twitching of one extremity, one-half of the face, etc. The pupils are at the start narrowed CEREBRAL MENINGITIS 299 and frequently unequal; in otitic meningitis the myosis is more marked on the side of the affected ear; in the terminal stage they are wide. Of other ocular symptoms, there occur occasionally convergent and divergent strabis- mus, as well as tonic spasm of the orbicularis palpebrarum. Further disease signs are, hyperesthesia of the skin, the so-called "taches cerebrales" of Trousseau (see Lecture XVI, page 250), retention of urine and faces, par- ticularly regularly, however, deliria, which manifest themselves sometimes by the murmuring of incoherent words as well as by disorientation for time and place, sometimes, however, by screaming, motor jactitation, even furibund con- ditions. IKic and there papilloedema, usually of moderate grade, occurs. The disease results fatally in the great majority of cases. In the terminal stage the clinical picture changes, in that, in the place of contractures, par- alvses (monoplegia, hemiplegia, paraplegia, even tetraplegia), in the place of delirium, a torpor deepening to coma; instead of myosis, mydriasis: instead of retention, incontinence of stools and urine, instead of quickening of the pulse and respiration, their slowing occur, and finally death from respiratory and cardiac paralysis follows. Occasionally at the last, Cheyne-Stokcs breath- ing is observed. Cases going on to "recovery" almost always leave behind serious defects, as deafness, mental weakness, idiocy, etc. The treatment agrees in general with that of epidemic meningitis. Natu- rally, upon the beginning of meningeal symptoms, the underlying disease must be searched for, and energetically combated; for example, the mastoid tre- phined, the frontal sinus opened, furuncle of the face incised, etc. Repeated lumbar {junctures are always in place, although they only rarely produce tasting effect; nevertheless, they quite frequently affect considerable alleviation, especially as to headache and delirium. When the puncture fluid, at first cloudy, later distinctly purulent, becomes by degrees again free from leucocytes and clearer, this is naturally favorable prognostically. Definite surgical treat- ment (trephining, opening the dura, washing out, drainage) has of late been recommended by Girard and others; the results are, however, unfortunately very uncertain, as a rule. For the rest, see Lecture XVI, page 25L D. Tuberculous Meningitis A special clinical and anatomical consideration is due. to the by far must frequent variety of metastatic inflammation of the pia-arachnoid, tuberculous meningitis, which occurs, as a rule, in young individuals affected by lung, bone or joint tuberculosis. It is particularly often, also, a symptom in acute miliary tuberculosis. Tuberculous meningitis arising by continuity is much behind Hi. metastatic form in importance and frequency; tuberculous caries <>( the skull bones, ear tuberculosis, solitary tubercle of the brain, etc., usually fur- or. I: a starting point far it. As exciting cause-,, injuries to the head (evc;i bloodless ones), insolation, taking cold, overexertion, as well as psychical exciti in. hi. come into consideration. So. I have observed in a girl with slighl affee Bon of the lung apices, a tuberculous meningitis terminating fatally and con- 300 LECTURE XIX firmed at the autopsy, which occurred in immediate connection with a violent quarrel with her unfaithful lover, as acute delirium, and which was at first quite pardonably interpreted as hysteria by the family physician. In con- tradistinction to purulent meningitis, tuberculous meningitis in the great ma- jority of cases spreads itself over the region of the base of the brain. It is hence designated as basilar meningitis. The extension of the inflammatory process into the fissure of Sylvius is almost the rule, while the convexity of the brain is usually but inconsiderably affected. In the region of the disease, an opalescent or cloudy "gelatinous" infiltration of the pia and arachnoid is found ; the fluid is seropurulent only in places. At most varied points, how- ever, particularly in the neighborhood of the vessels, a more or less abundant deposit of miliary, whitish, tubercular nodules is noted, which anatomically present the well-known structure of tuberculous granulations (giant cells, etc.). Also Koch's bacillus can be recognized in most cases. The disease picture of tuberculous meningitis has so great similarity to that of purulent meningitis that in order to avoid repetition, I will limit my- self more or less to emphasizing the differences in its course and symptoma- tology. Only exceptionally does tuberculous meningitis begin suddenly. In the great majority of cases there is a definite prodromal stage of from one to several weeks. The patient is peevish and irritable, sometimes also apathetic and soporous. In many cases the alternating redness and pallor of his face is striking to those surrounding him. He loses all appetite and emaciates rapidly ; usually there is obstinate constipation, somewhat less frequently, nausea or vomiting. Soon there are irregular rises of temperature with slight shivering, further, headache, stiffness of the neck, confusion at night — and so it proceeds finally to the completely developed meningitic symptom-complex, in which headache, cerebral vomiting, stiffness of the neck, and tension in other muscles, vasomotor disturbances, hyperesthesia of skin and muscles, Kernlg's and Leichcnstern's signs (see Lecture XVI, page 24-9), reach a very high degree. In children opisthotonus is so excessively developed that the occiput almost rests upon the back ; there is frequently also very great contracture in flexion of the lower extremities. The delirium manifests itself usually in rest- less grasping movements of the hands, muttering, etc. ("muttering delirium"). In contradistinction to purulent meningitis, the relatively slight elevation of temperature (usually between 38° and 39° C), is striking; frequent, also, is the absence of quickening of the pulse, if not its slowing. Lumbar puncture shows in the initial stages almost always a lymphocytosis of the spinal fluid, further on in its course, however, the leucocytes may preponderate just as much as in purulent meningitis. Naturally, a positive finding of tubercle bacilli in the spinal fluid is decisive. As a criterion of the basal localization is, further, the much greater frequency of cranial nerve symptoms important : paralyses of the abducens, oculomotorius, facialis, deafness, optic neuritis. Symptoms from the side of the pyramidal tracts (Babinski and Oppenheim's reflexes, ankle clonus) not rarely occur. The ophthalmoscopic finding of tubercles in the choroid is, on the other hand, a very great rarity and scarcely ever available for confirming the etiological diagnosis (for which in children the v. Pirqua CEREBRAL MENINGITIS 301 cutaneous reaction may be called into aid, in case the primary tuberculous focus is latent). The prognosis is a very bad one. Of the bacteriologically confirmed ob- servations of tuberculous meningitis, only very exceptionally have cases sur- vived the disease, and here even the recovery was usually with defect ; also some of these patients perished later with recurrence. The treatment agrees in general with that of other meningitides. E. Internal Hemorrhagic Pachymeningitis In the etiology of this disease, chronic alcoholism occupies the first place in frequency and practical importance; besides, it occurs in different disease conditions accompanied by the "hemorrhagic diathesis" (scurvy, leucemia, hemophilia, pernicious anemia, etc.), as well as after the most varied ex- hausting infectious diseases. Exciting causes for this disease, which affects liv preference those of advanced age and the male sex, are, above everything else, contusions and wounds of the head. For the rest, many cases remain etiologically entirely unexplained. According to Obersteincr, hemorrhagic pachymeningitis occurs in about 20 per cent, of paretics as a complication of the end stage. Anatomically, internal hemorrhagic pachymeningitis presents itself as fol- lows: There arise upon the inside of the dura mater, flat, membrane-like de- posits, extraordinarily rich in vessels, of inflammatory granulation tissue, which later changes into connective tissue. The walls of these vessels show more or less marked evidence of degeneration and a great tendency to rupture, wliich have as a result the formation of blood coagula upon the membrane cov- ering the dura. New granulation tissue develops in these coagula, and so there arises from hemorrhages which later show themselves only as brown pigment, Strata layer built upon layer. This hemorrhagic pachymeningitis develops (usually bi-laterally, although, as a rule, asymmetric) particularly over the convexity of the hemispheres, namely over the parietal lobes; il is more rarely found at the base of the skull. The disease iii slight cases can run its course without symptoms and is then counted among the so-called "accidental autopsy findings."' This applies, for example, to most of the eases found in paretics. The more severe eases, on the contrary, produce grave symptoms, which indeed have in themselves little typical, and are subjected to such great variations in their character and grouping that confusion with leptomeningitis, brain tumor, hemorrhages, etc., is very frequent. In the early stages severe headache is the chief symptom. Since the patient besides this usually complains of vertigo, shows different speech disturbances and defects»of memory and in his movements can give evidence to a certain slowness, weakness and cl siness, we are greatly reminded of arteriosclerosis of the brain, cerebral syphilis or dementia paralytica. Also Conditions of psychical excitement and epileptiform attacks are described. Later the condition is complicated by suddenly occurring (probably corre- sponding to further hemorrhage) focal symptoms which sometimes disappear again, sometimes persist: Monoplegias, hemiplegias, also tetraplegias, con- 302 LECTURE XIX jugate deviation of the eyes and head, sometimes also spasms in the individual extremities or in the face. Stiff neck is rare, Kernig's symptom somewhat more frequent. The pupils are usually narrow, sometimes unequal, and react slowly ; the tendon reflexes are usually exaggerated, ankle clonus and Babinski, on the other hand, but rarely present. There can be also a weakening and diminution of the reflexes. The intelligence diminishes rapidly. In cases run- ning a fatal course, the symptom complex of brain pressure is gradually es- tablished (slowing of the pulse, papilledema, etc.) and the patient dies in coma. A standstill or partial regression of the disease picture can occur in cases which have not progressed too far. Along with treatment of the underlying disease, in all cases an energetic iodide cure should be tried. To be recom- mended further, are free purgation, bland diet, abstinence from alcohol and from smoking. Apoplectiform incidents are to be treated after the principles laid down in Lecture XVII. F. Thrombosis of the Brain Sinuses The thrombotic occlusion of the different brain sinuses (particularly of the transverse, superior longitudinal and cavernous sinuses) can arise autoch- thonously or secondarily, that is by the extension of infection or through metastasis. In the first instance there is the so-called "marantic" thrombosis which is observed in deci'epit individuals, in chlorosis, pernicious anemia, fol- lowing exhausting infectious diseases, particularly, however, in the "atrophy of nurslings." Secondary or infectious sinus thrombosis arises in consequence of erysipelas, suppurations in the petrous bone, in general sepsis, pyemia, etc. The thrombotic occlusion of a large sinus manifests itself clinically by severe headache beginning suddenly, by confusion and by spasms in the extremities; in infectious thromboses these symptoms are accompanied by chills and fever. Characteristic, however, are the local signs of stasis ; in thrombosis of the cavernous sinus the lids are swollen, the eyeballs protrude ; in thrombosis of the transverse sinus the neighborhood of the mastoids is cedematous and marked by dilated veins ; in thrombosis of the longitudinal sinus, the temporal veins are swollen, and there is also venous stasis in the nasal mucous membrane and epistaxis. Papilloedema is frequent only in cavernous thrombosis, rarest in transverse thrombosis. Extension of the thrombosis into the jugular vein is accessible to direct palpation. The prognosis of these conditions is very serious, as even mild sinus throm- boses, if they reach a higher degree, produce oedema of and hemorrhages into the brain. Onlv clotting of limited extent is susceptible of recovery, a result which we seek to assist by the application of leeches to the neck, iodide cures, etc. Infectious thromboses, on the other hand, demand operative removal, which, however, only in the minority of cases is able to prevent death from diffuse meningitis. G. Non-Suppurative Encephalitis The non-purulent inflammations of the brain, if we ignore the cerebral localization of the Hcine-Medin disease, and the inflammatory forms of in- ENCEPHALITIS 303 fantile cerebral paralysis, are great rarities ; their knowledge we owe to Striim- pell, Wernicke, Oppenheim, and Cassirer. Anatomically, the diseased parts present the histological criteria of inflammation (infiltration with small cells, dilatation of the vessels, oedematous swelling, degeneration of the parenchy- matous elements, etc.) and manifest themselves macroscopically by redness and swelling. Usually there are also numerous punctiform or miliary ex- travasations of blood upon cross section (the so-called "Flea-bite Encepha- litis"). Clinically, different forms may be separated, of which the two following are the most important : 1. POLIOENCEPHALITIS SUPERIOR HEMORRHAGICA (WERNICKE) The patients are taken ill suddenly, with headache, vertigo, vomiting, con- fusion, and somnolence, sometimes also with conditions of delirious excitement, and with this there develops a rapidly progressive, finally almost total ophthal- moplegia. The gait is extremely atactic. Tremor is almost the rule, choreic unrest is sometimes to be observed. The disease, which mainly affects con- firmed alcoholics of middle age, but can arise upon the basis of meat poisoning, usually leads to death under increasing stupor and heart weakness in one or two weeks. Recoveries are rare. Upon autopsy, in the neighborhood of the third ventricle and of the aque- duct of Sylvius, particularly in the region of the eye muscle nuclei, a severe hemorrhagic inflammation is found.* 2. THE ACUTE HEMORRHAGIC ENCEPHALITIS OF ADULTS (STRUMPELL) This is in the majority of cases an accompaniment or the result of acute infectious diseases, namely, of influenza. It can, however, represent a primary acute infection. Klieneberger saw it arise after salvarsan injection. Rather suddenly there arise with high fever, headache, unconsciousness and hemiplegic Disturbances, which end in a few days in death. Only a few cases pass on to Recovery, leaving behind more or less marked hemi-paresis. Pathologico- knatomically a hemorrhagic inflammatory process forms the basis of the dis- use. This, as a rule, is limited to one hemisphere, but is rather diffusely spread throughout this and affects both the white ami the gray matter. Therapeutically we can, though with justified pessimism, in both forms, hold fasl to the recommendations of Oppenheim: Icebag to the head, leeches to the temples or to the mastoid region, free purgation (calomel) inunctions of col- lurgol or a mercury cure, iodide of potassium, antipyretics, wet packs to the body, hot foot baths, sinapisms to the extremities, etc. ' "Polioencephalitis inferior hemorrhagica," which is located in the gray matter of the medulla, occurs in infectious diseases and gives the clinical picture of "acute bulbar paralysis." ■ lure VII.) 304 LECTURE XIX H. The Circulatory Disturbances of the Brain We will take into consideration here only those circulatory disturbances of the brain which occur in the form of attacks and rather rapidly disappear again ; hyperemia of the brain, anemia of the brain, and the so-called concussion of the brain. Determination of blood to the brain occurring in attacks forms the basis of the so-called "congestion of the brain" which occurs under the influence of heat, psychical and especially sexual excitement, etc., in predisposed individuals (vasomotor neurasthenics, arteriosclerotics, inveterate smokers, drinkers, peo- ple with "true plethora") and which can be experimentally produced by the inhalation of amyl nitrite. In it, the face becomes intensely red, there is a feeling of burning in the conjunctiva 3 , the carotids and the temporal arteries pulsate appreciably ; the patient usually experiences tinnitus aurium, a sense of pressure in the head, vertigo, spots before the eyes, sometimes, also, nausea, becomes excited, sometimes, slightly dull and confused. After a few min- utes, or only after a half hour to an hour, the hyperemia ceases and the symp- toms disappear. Its danger lies in arteriosclerotics, naturally, in the condition that it favors the occurrence of cerebral hemorrhage. Paroxysmal diminution of the blood in the brain finds its clinical expression in the temporary reduction of the brain functions to the minimum necessary for maintaining the vital functions; there is fainting, syncope. After indefinite prodromal symptoms and a general feeling of illness, there occurs, with small- ness and rapidity of the pulse as well as great pallor of the face and outbreak of a cold sweat, often also, with repeated yawning and usually decided nausea (rarely actual vomiting), a condition of complete muscular relaxation and marked clouding of consciousness or even complete unconsciousness, which lasts for a half hour or more, then to make way for the normal condition. The prognosis of these conditions which occur almost exclusively in anemic, weak or cachectic persons, is in general good; a fatal ending is only to be feared from syncope after severe hemorrhage. Commotio cerebri represents a special variety of circulatory disturbance in the brain, which occurs after violent concussions of the head in accidents of the most various sorts, and indeed also without direct traumatization of the skull ( for example in persons who have been in the neighborhood of a violent explosion). This "concussion of the brain" occurs at once and disappears after a short time (some minutes to a few hours). Only very exceptionally in such cases in which the autopsy has shown an absence of any organic injury of the brain (for instance, compression or hemorrhage) does death occur from cardiac and respiratory paralysis; the patient in commotio cerebri suddenly becomes unconscious, breathes, as a rule, shallowly (more rarely deep and stertorously), has a small, generally slow pulse, is pale and presents coldness of the surface. He shows complete muscular relaxation and does not react to painful stimuli (pinching, stabbing, etc.). The pupils are now narrow, again dilated, but react to strong light. Already during unconsciousness, particu- larly, however, when the victim comes to again, there is frequently retching or vomiting, and afterward there persists for a long time, headache, staggering DISTURBANCES OF CIRCULATION" IN THE BRAIN 305 gait, and slight clouding of consciousness. More severe symptoms, for ex- ample, convulsions, do not belong within the limits of cerebral concussion, but indicate organic injuries of the brain. As a characteristic result cerebral com- motion almost always leaves behind the so-called "retrograde amnesia," that is, the memory not only of the accident itself, but even the period (of about a quarter of an hour) preceding it, is completely obliterated from the recollec- tion of the patient. Sometimes, however, these memories return after a time, in which case, on retrospective diagnosis, we must think of concussion of the brain, when required to give an opinion on the case. Treatment of hyperemia of the brain consists in loosening the clothing about the neck, raising the head, giving hot foot baths, hot applications to the calves, mustard plasters to the feet, cold applications to the forehead and neck ; when necessary, also, in bleeding (best by puncture of a vein). In syncope and concussion of the brain, the patient is placed in a horizontal position, is al- lowed to inhale ammonia, the face is sprinkled with cold water, the temples rubbed with alcohol, the respiratory muscles are faradized, and, when necessary, the heart's action is stimulated by injections of ether and camphor. LECTURE XX Diseases of the Cerebellum Gentlemen: The progress which brain surgery has made during recent years has rebounded to the advantage of the cerebellum in special degree. This, however, places not only upon the neurologist, but also upon the general physi- cian, the duty of being alive to the earliest possible recognition of cerebellar af- fections, since the result of an operative procedure depends chiefly upon the time at which we entrust the patient to the hands of the surgeon. On the other hand, it should not be overlooked, that every operation in the posterior fossa of the skull is accompanied by dangers to which no patient should be lightly exposed. On this account the differential diagnosis of cerebellar diseases is of great prac- tical importance. From a clinical standpoint we are justified in including certain extra- cerebellar affections of the posterior fossa of the skull under "cerebellar affec- tions," since they manifest themselves preponderatingly through cerebellar symptoms, I mean: 1. Tumors of the cerebello-pontine angle. 2. Serous menin- gitis in the posterior fossa of the skull. Both disease conditions, as well as abscesses and tumors of intra-cerebellar location, come within the province of surgery, while softenings, hemorrhages, atrophies and ageneses of the cere- bellum, as well as cerebellar encephalitides and cerebellar syndromes in malaria, are to be cared for by the physician. We will take uj:> these disease processes in the order of their practical im- portance. A. Tumors The cerebellum is, during childhood and youth, the location of predi- lection for tumor formation in the nervous system. Along with true neo- plasms — gliomata, sarcomata, fibromata — cystic tumors occur, and as an infectious granuloma, solitary tubercle is to be considered. Besides, there mu->t be mentioned as a rarity, a parasitic tumor, echinococcus of the cerebellum. Symptomatologically, the brain pressure syndrome which we have already described under tumors of the cerebrum, makes itself first apparent ; still, with this, some important criteria for the localization of the pathological process, whether it is in the cerebellum or in its immediate neighborhood, usually must be considered. So, headache in cerebellar affections is characterized by ex- traordinary violence and persistence probably on account of the richness of the tentorium in sensory fibers. Further, this pain presents its maximum severity in the occipital region and the back of the neck, from whence it radiates some- times into the upper part of the back. Many times, also, the patients complain 306 DISEASES OF THE CEREBELLUM 307 of frontal headache; still, this is diffuse, while the occipito-nucheal pain usually predominates on the affected side. It can be accompanied by more or less marked stiffness of the neck. Very frequently the back of the head is sensitive to percussion and pressure ; still, I place particular value upon finding the characteristic pain which a pressure upward upon the mastoid process of the affected side produces. Papilloedema appears particularly quickly and mark- edly in cerebellar tumors, and even when they are unilateral it is generally bilateral. Finally, a "spinal fluid phenomenon" has been described in cere- bellar tumors ; this consists in the excessively rapid fall of pressure after a lumbar puncture and the abrupt cessation of the flow of the cerebrospinal fluid. Evidently the tumor presses the medulla suddenly into the foramen magnum and in this way interrupts the communication between the intracranial and the spinal fluid. In spite of all the assertedly infallible precautions which a few specialists on lumbar puncture have recommended, we had better avoid this dangerous diagnostic method just as soon as we have become convinced that there is a suspicion of tumor of the cerebellum, since some cases of sudden death have occurred under its application. Among the local symptoms of cerebellar tumors, cerebellar ataxia stands in the foreground, as it presents the cerebellar symptom par excellence. By this I mean only, that without ataxia no cerebellar affection can be diagnosed with certainty; in no way. however, that typical cerebellar ataxia is foreign to extra-cerebellar lesions. The interruption of one system of cerebcllifugal or cerebellipetal tracts suffices to deflect the regulating activity of the cerebellum, whether this lesion has its location in the midbrain, in the pons, in the bulb, or even in the spinal cord. It is all the same to a reflex apparatus, whether the interruption has destroyed the reflex center, the afferent or the efferent fiber complex. We have already, under the description of Friedreich's disease in Lecture VIII, mentioned the clinical peculiarities of cerebellar ataxia and its physio-pathological basis (page 12Ji). While incoordination in consequence of a total lesion of the posterior roots of the spinal cord affects movements in their entirety, cerebellar ataxia — par- ticularly on the trunk and in the lower extremities — shows a decided pi'edilec- tion for the composite movements, that is. for those movements which require the cooperation of extended groups of muscles. In such patients it is observed that the simple muscular movements, for example, flexing or extending the foot, the knee, the hip, adduction or abduction of the thigh, can be executed correctly; that, however, their dynamic and static combination is disturbed. s.i .He produced a zigzag gait, reeling, more or less marked swaying, as a con- sequence of an interruption of flic synergies which are necessary for the im- mobilization and steadying of the body and its members in walking and al rest. Here, also, must be considered that phenomenon to which attention has been called by Babinski under the name "cerebellar asynergy." Winn the patient attempts to raise himself while [ying*on his hack, he elevates his lower, extremi ties instead of his trunk; when going forward he allows his trunk to lag behind to a certain extent, and so is in danger of falling backward. As a rule, cerebellar ataxia affects the upper extremities to a mucn less Hegree; sometimes, indeed, it appears to avoid them entirely. It must be con- 308 LECTURE XX sidered, however, that in man the arms are subjected to the coordinating in- fluence of the cerebellum to a very limited extent on account of their little importance in the maintenance of the equilibrium. Nevertheless, I have nearly always been able to detect certain atactic disturbances in the movements of grasping with the hand. As to this, we owe to Bablnskl an ingenious method of bringing out latent disturbances of coordination in the arms. The patient is ordered to carry out in rapid succession muscular actions opposed to one another, for example, pronation and supination, and it is often noticed then that the individual with cerebellar disease can no longer effect so subtile co- operation of the antagonistic muscles. This phenomenon is called "adia- dochokinesis" (a=privative ; dtaoi^rj ^succession). In clinical importance cerebellar hypotonia is inferior to cerebellar ataxia, since the first may be entirely obscured by simultaneous lesion of the pyramidal tracts; such a lesion, however, is one of the most frequent indirect effects of tumors of the cerebellum, while, on the other hand, marked spastic conditions are in no way uncombinable with typical cerebellar ataxia. Where, however, cerebellar hypotonia is present, it manifests itself like spinal hypotonia (see above, page 173) as well in a relaxed condition of the muscles as in the possi- bility of bringing the extremities into very abnormal positions on account of the relaxation of the antagonists, for example, overextension or overflexion. A method of examination recommended by Stuart and Holmes, and of which I regularly make use, deserves to be mentioned. If a movement of flexion which the patient is attempting to carry out is opposed and the resistance is suddenly withdrawn, the flexion takes place in an extreme degree and the re- action, which normally always occurs, is either absent entirely or there is only a trace of it. This is a symptom which is capable of demonstrating to us the hypotonia of the extensor muscles. I would lay particular emphasis upon the different conditions of the re- flexes in spinal and in cerebellar hypotonia. The first is accompanied by areflexia or hyporeflcxia, as we saw in Lecture XI, the last is entirely inde- pendent of the intensity of the tendon phenomena which can be found not rarely retained or even exaggerated. Where, however, in cerebellar tumors, a reduc- tion or suspension of the reflexes is found, it appears to be dependent upon mechanical indirect action. Indeed, the increased brain pressure by extension to the closed sac which the spinal dura mater forms about the posterior spinal nerve roots may produce radicular lesions which can lead to symptoms of their anatomical destruction (Hoche, Wollcnberg and others). In general, ataxia and hypotonia are most marked in tumors of the vermis. In tumors of the cerebellar hemispheres, they, as a rule, ai - e present in less intensity, and indeed, when the process is unilateral, in the form of hemiataxia and hemihypotonia of the same side. According to my personal experience, strict unilaterality of these symptoms is much rarer than their preponderance on one side. This last is the rule in all those cases in which the neoplasm in- deed affects one hemisphere to a greater degree, but oversteps the middle line to a greater or less extent ; in tin's class are probably the majority of cerebellar tumors — if we leave out of .consideration those of the cerebello-pontine angle. As a matter of course, the recognition of atactic or hypotonic disturbances DISEASES OF THE CEREBELLUM 309 on tlie relatively avoided side requires particularly careful methods of inves- tigation. Paradoxical are certain observations of tumors of the vermis without ataxia or hypotonia. It has been attempted to explain these rare cases in different ways. For example, Xotlinagel believed that here the tumors were of such slow growth that a compensatory function could be established by an entering into action of other brain parts. In the case of solitary tubercle the absence of atactic disturbances has been attributed to the intactness of the axis cylin- ders which pass through the focus. Though these attempts at explanation may he satisfactory for a certain number of cases of tumors of the vermis which have run their course without ataxia and hypotonia, they are not suitable for other cases which, on the contrary, were characterized bv an extremely rapid growth and plainly destructive nature of the process. If later pathologico- anatomical investigations do not solve the riddle, we must satisfy ourselves with the hypothesis that these exceptional cases affected individuals in whom the cerebellum, "ab ovo" possesses a particularly small functional importance. Vertiginous phenomena are exceedingly characteristic for cerebellar tumors, even in the initial stages, and indeed they are manifested as true rotary vertigo, "systematic vertigo," produced by irritation of the neurones from the nuclei of the vestibular nerve, which pass through the cerebellum. The patients have the distinct sensation of rotation both of their own bodies and of the surround- ing objects in a definite direction. These sensations set up nausea, often ac- companied by pallor, sweating, vomiting, etc. They depend upon the fact that by the irritation of the tumor the stimulation of the vestibulo-cerebellar apparatus (which is normally produced only by the hydrostatic relations in the semicircular canals of the labyrinth) is in contradiction to the actual position of the body and so falsifies the ocular, articular and muscular im- pressions. Particularly interesting an' the attacks of vertigo which, under the names of "cerebellar" or "vestibular" attacks, "cerebellar fits," "cerebello- pontile seizures" have been described by Ziehen, Dana, limit, and others. The Midden and extremely violent vertigo which characterizes them is often ac- companied by loss of consciousness; as a rule, however, by definite atactic disturbances, severe vomiting, headache, tinnitus aurium and nystagmus. This last symptom is particularly frequent in cerebellar tumors and can in general he considered as a symptom of irritation of "Deiters' nucleus" or of the "posterior longitudinal fasciculus." The one, indeed, lies close to the Cerebellum, the other, however, passes directly under the vermis in the most dorsal portion of the tegmentum, to the eye muscle nuclei. Via the nucleus of Deiters and the posterior longitudinal fasciculus, also, in the artificial provoking of a nystagmus by the so-called Barony's lest, the impulse is transmitted to the ahducens and OClllo-motoriuS. In healthy peo- ple, by Syringing the external auditory canal with cold water, a nystagmus toward tin opposite side is produced; with hot water there is one toward the side of the injection. In tumors of the cerehellum, however this "'caloric nystagmus" may lie absent. A spontaneous nystagmus of patients with cerebellar tumors can lie hut rarely noticed when looking to the front and must lie provoked by having the 310 LECTURE XX patient look far to the right or to the left. On this, a decided oscillation of the eyes is observed when the patient looks in the direction of his cerebellar lesion. I lay great stress upon this point. On the other hand, I have never been able to confirm another phenomenon mentioned by different authors, namely, the greater extent of the excursions in the eye corresponding to the diseased half of the cerebellum. As Oppenheim has emphasized, a nystagmus which is absent in a standing position can sometimes be elicited by having the patient lie upon his side. Upon further growth of the cerebellar tumor a number of indirect symp- toms usually manifest themselves in variable combination. Among the most frequent is abducens paralysis or, rather, a paralysis in looking toward the cerebellar focus. Of course the last can give rise to "conjugate deviation" toward the opposite side. Trochlear paralysis occurs rather regularly in neoplasms of the anterior end of the vermis, besides this, also, sometimes a paralysis of the superior, inferior, and internal recti muscles, on the other hand, almost never one of the internal eye muscles. Defect and irritative symptoms on the part of the cranial nerves from the 5th to the 12th develop more rapidly when the neoplasms are situated outside of the cerebellum than when they are intracerebellar ; they are, however, quite frequent here also. Disturbances of speech, swallowing, respiration and articulation can produce in such cases the picture of bulbar paralysis already considered in Lecture VII. Possibly from dysarthria produced in this manner, the scanning speech which has been described by Dreschfeld and Bruns in cerebellar tumors, and which perhaps represents nothing more than a cerebellar ataxia of the speech muscles, must be distinguished. The investigations of Hothmann and Katzenstein, who have found in animals a region in the cerebellum whose extirpation produces an ataxia of the vocal cords, indicate this. Frequently in cerebellar tumors sudden death from pressure upon the medulla has been observed; not rarely also in the course of the affection, a unilateral paralysis or paresis. According to whether the pressure has oc- curred anterior or posterior to the pyramidal decussation, the hemiplegia or liemiparesis affects the limbs of the same side or those of the opposite side. Certain forced attitudes and compulsory movements are particularly marked when the neoplasm acts upon the anterior or the middle cerebellar peduncle. They are, rolling about the long axis or bending the neck and trunk toward a definite side, as, for example, in the observations of Russell and Bruns. Nevertheless it is impossible to give generally applicable rules with regard to the direction in which these involuntary attitudes and movements take place, whether toward the healthy or toward the diseased side, and its physio-patho- logical substratum is still quite obscure and the subject of controversy. No more definite is our knowledge of the pathogenesis of the choreic-athetoid movements which according to Bonhuffcr, Pincles, and others, may occur on the same side upon destruction of one brachium conjunctivum. Also the con- ditions to which the vertical divergence of the eyeballs (of Magendie) owes its origin, are unknown to us. This symptom is supposed to be found along with conjugate deviation in affections of the middle peduncle of the cerebellum. DISEASES OF THE CEREBELLUM 311 A definite category of tumors of the posterior fossa of the skull manifests itself by a rather stereotyped clinical picture, namely : TUMORS OF THE CEREBELLO-PONTIXE ANGLE These are neoplasms (usually fibroma or fibrosarcoma) which take their origin from the connective-tissue sheaths of the auditory or the facial close to the exit of these nerves and present the following symptoms: Paresis in look- ing in one direction, nystagmus, absence of the corneal reflex, disturbances of sensibility in the distribution of the trigeminus, nervous hardness of hearing or deafness, adiadochinesis, all upon the side of the disease focus, and besides this, papilloedema, cerebellar ataxia and occipital headache. Of course the symptomatology of these tumors is not always such a complete one ; on the other hand, however, further phenomena ma}' be added to it, for example, con- vulsions, neuralgiform pains in the trigeminus region, anosmia, etc. Differential Diagnosis Confusion of cerebellar tumors with multiple sclerosis has frequently oc- curred. We know, indeed, that this last has in common with cerebellar neo- plasms, the nystagmus, cerebellar ataxia, vertigo occurring sometimes in at- tacks, that further, scanning speech also occurs in cerebellar tumors, and that their pressure upon the motor tracts of the brain axis may produce increase of reflexes and spasticity. We might add that upon a few occasions an in- tention tremor has been observed in cerebellar neoplasms (whether as a symp- tom of irritation of the pyramidal fibers or not?). Very important for dif- ferential diagnosis is examination of the eye grounds; though there is ex- ceptionally a swelling of the papilla in multiple sclerosis also (probably from complicating hydrocephalus). The headache in multiple sclerosis is scarcely ever as severe as in cerebellar tumors. Particularly difficult is sometimes the differentiation of cerebellar tumor from cerebellar abscess and serous meningitis of the posterior fossa, in case Hi'' latter affections do not come on acutely with fever after an otitis or a in nl trauma. The absence of papilloedema in doubtful case-, speaks always for abscess, while in serous meningitis this symptom occurs to almost the same extent as in cerebellar tumor. In favor of the diagnosis, serous meningitis, an Outspoken remitting then exacerbating course may be in any event decisive. In the great majority of cases indeed, it is unfortunately impossible with tumor symptoms to exclude a '•pseudo-tumor meningiticus." This, however, matters little, since therapeutically after failure of medical treatment directed against serous meningitis, an operation for this is indicated, and its finding after opening the skull may prove a pleasing surprise for us. We might mention the also difficult differentiation from aneurisms of the basal arteries. Characteristic of the last is a vascular murmur within the skull synchronous with the pulse. A suspicion in this direction might be justi tied when there is advanced arteriosclerosis and increased blond pressure, also in alcoholism or syphilis, finally, after injuries to the skull (fractures of the base). 312 LECTURE XX Treatment We will proceed here according to the principles laid down already when speaking of tumors of the cerebrum, that is. at first we will try the resorption- favoring action of mercury and iodide against the scarcely ever to be ex- cluded eventuality of serous meningitis. But not for too long a time. Where, after 3 or 4 weeks, definite reduction of the objective and subjective symptoms is not apparent, the continuance of internal treatment should not be persisted in, hut trephining into the posterior fossa of the skull should be advised. Even if the tumor then proves to be irremovable, nevertheless a long period of improvement may be the result of the decompression, in any case we can hope to relieve the patient of his unbearable headache and to prevent threat- ened blindness. Now there is in any event danger connected with simple de- compressive craniectomy; so, a boy with glio-sarcoma of the vermis died sud- denly in collapse, 24 hours after making an opening in the occipital bone, although he apparently recovered well from the operation, and the section showed, besides the tumor, marked hyperemia of the choroid plexuses with great hydrops ventriculorum — perhaps to be considered as a reaction to the operative removal of pressure. Many neurologists and surgeons have reported similar experiences and many on this account prefer, even in cerebellar tumors, to make the decompression in the temporal region of the skull ; indeed, I have seen also in a case of sarcoma of the left cerebellar hemisphere subjected to temporal craniectomy, after a few days exitus from vagus paralysis in conse- quence of hydrops of the 4th ventricle. Naturally it may be objected to temporal craniectomy, that in it we either in advance renounce a curative operation in favor of a palliative one, or postpone the first by addition of the last and subject the jDatient to two operations instead of one. In so unfavor- able a disease, in any case some courage is needed. Now as to the curative effects of the radical procedure, they are, as already expressly emphasized, dependent upon the time of the intervention, and many bad results would perhaps be avoided by early diagnosis. The pathologico-anatomical nature of the tumor is also important ; cysts and fibroma give the best prognosis ; after them, solitary tubercle (which can spontaneously cease growing), gli- omata which pass over into normal tissue without sharp limitation, the worst; again, the location of the tumor plays a great role — so the operation for cere- bellopontine tumors in spite of their usually good limitation and often easy separation is, on account of the neighborhood of the medulla and the difficulty of access to the tumor, always a severe procedure, which is usually followed by death from heart failure. This happened in two cases observed by me, though the operation was undertaken with local anesthesia, and F. Krause in 50 operative cases, has lost 46 (that is, 92 per cent.). The operation in two stages is preferred by most surgeons upon intervention in the posterior fossa of the skull. B. Abscesses of the Cerebellum They occur like cerebral abscesses, either by metastasis (particularly after lung abscess and gangrene) or after injuries to the skull, which sometimes. DISEASES OF THE CEREBELLUM 313 have occurred years or decades before. Most frequently, however, cerebellar abscess is the result of purulent middle ear inflammation extending through the petrous bone and so presents the counterpart of otitic abscess of the temporal lobe. The development can be very acute, or the symptoms may arise gradually in the course of several weeks; finally there are very gradual forms which stretch over years. Through encapsulation such abscesses may indeed become latent ; in these, however, sudden lighting up of the acute process which then usually leads to perforation of the abscess wall and to death from purulent meningitis, is frequent. The symptoms are headache, stiff neck, ver- tigo, vomiting, dysarthria, disturbances in swallowing, paralysis in looking toward the affected side (important for localization) ataxia. Rarer are nystagmus, hemiataxia upon the side of the abscess, papilledema and loss of the patellar reflexes. Fever is important diagnostically, but no regular phe- nomenon; indeed, according to Macewen, subnormal temperatures occur. Against brain puncture which naturally is capable of confirming the diag- nosis in the first place, Fcdor Krause wains on account of the danger of an infection of the meninges. Operation is, of course, strictly indicated, but un- fortunately offers only slight probability of cure. C. Serous Meningitis of the Posterior Fossa of the Skull The sacculations of the pia arachnoid in the neighborhood of the cere- bellum — first, the "cisterna acustico-facialis" at the cerebello-pontine angle, second, the "cisterna cerebello-medullaris" between the medulla and the cere- bellum — present a peculiar seat of predilection for retention cysts. These last are of inflammatory nature, depend upon the closing off of these preformed arachnoidal spaces along with corresponding inhibition of the resorptive pro- Cesses and are caused by traumatic, otitic and syphilitic infection. Fig. 87 shows a case of such cystic serous meningitis formerly published by me. In an ideal sagittal cross-section in which trephining, with separation of the fiat adhesions which stretched over from the region of the pyramis and the lobuli bivc litres cerebelli to the posterior limit of the medulla, had as a result dis- appearance of all the symptoms pointing to a space-narrowing process in the posterior fossa of the skull. When, six months later, attacks of vertigo and headache. (I vsdiadochokincsis, and slight cerebellar gait occurred again, an iodide cure caused rapid disappearance of these symptoms. There was plainly onlv a slight accumulation of exudate within partial adhesions in the operative li( Id. Slight cases of serous meningitis can also, from the beginning, he brought to recovery by purely interna] treatment (in which, even in nonsyphilitic cases, along with iodide of potassium, mercury certainly works favorably); however, such treatment if it gives no result should not he loo long continued (especially when there is definite papilledema). Also, ;i decided tendency to relapse may justify a decision to operate even in such cases as promptly read to rcsorhcnl s, 111 the more, since the results of these operations ;ire very good. This is shown not only by mv ease, hut also by those of Placzek-Krause, Opperiheim, lion liimll. and others. Svmpt atologically a differentiation from tumor of the cerebellum or of the cerebello-pontine angle is usually not possible. The 314 LECTURE XX diagnosis can nevertheless be made from the anamnesis, the remittent-exacer- bating course, also ex juvantibus. In my case, besides this, the relatively slight degree of cerebellar ataxia after one year's duration of the disease, the absence of spontaneous nystagmus, the exceedingly marked facial paresis ; further, the comparatively equal distribution of the symptoms upon the right and upon the left, had so impressed upon me the idea of an extracerebellar and median location of the expansive process that I could make a probable diag- nosis of serous meningitis and hydrops of the 4th ventricle. This last con- _Sin occ. Cyste Serous Meningitis of the Posterior Fossa of the Skull. Ideal Sagittal Section. Kh. = Cerebellum. Cyste = Cyst. dition, as Fig. 87 shows, was in fact present and is a usual accompaniment of cysts in the region of the cisterna cerebello-medullaris. D. Hemorrhages and Softenings Hemorrhages into the cerebellum occur under the same conditions as those into the cerebrum (miliary aneurisms of the artery of the dentate nucleus play of hn an important role), but are, however, much rarer. The lamellar structure of the cerebellum brings it about that the blood breaks through outward or into the 4th ventricle. Softenings in the cerebellum are even rarer. This depends on the one hand upon the fact that the cerebellar arteries are given off from the basilar artery at an angle which docs not favor the entrance into them of embolisms ; on the other, there are numerous anastomoses between the individual arteries of the cerebellum. Slight hemorrhages and softenings in the cere- bellum may manifest themselves clinically only by a short period of uncon- sciousness or an attack of vertigo and leave behind no residua, so that such lesions occasionally are noticed only as accidental autopsy findings. As pro- dromal symptoms of cerebellar hemorrhage, Mingazzini has mentioned occipital headache in two-thirds of his cases lasting for months, even for years. Remafc has observed occasionally repeated severe vomiting. Lussuna considers severe and repeated attacks of vertigo a sign of arteriosclerosis of the cerebellum. In very severe cerebellar hemorrhage, death may occur suddenly ; Mingazzini DISEASES OF THE CEREBELLUM 315 describes a case in which an old woman suddenly turned around several times, and then fell dead. In other cases of cerebellar hemorrhage, as well as in many instances of cerebellar softening, definite functional disturbances follow an ictus, for example, staggering gait, conjugate deviation of the eyes, nys- tagmus, forced turning of the head and neck. These phenomena can dis- appear by degrees. E. Agenesis and Atrophies Congenital cerebellar defects may remain latent even when they affect an entire half of the organ. If they extend, however, to both halves, they usually manifest themselves by cerebellar ataxia. A few cases, however, do not show this even. Sclerotic atrophies of the cerebellum can equally, although more rarely, remain latent clinically, especially when they occur at an advanced age and may be considered as degenerative processes without inflammatory basis. In general, however, they cause more severe atactic disturbances than the congenital ageneses, and even when they are only unilateral. Sometimes, be- sides this, other symptoms are also noticed, for example, speech disturbances and choreiform movements. Often such patients have epileptic attacks which indicates involvement also of the cerebral cortex — even when this escapes recog- nition by the histological methods at our disposal. In any event, combination of atrophy of the cerebellum with sclerotic atrophy in the cerebrum and spinal cord is not at all rare. Here the clinical picture is richer in symptoms, in that tremor, nystagmus, weakmindedness, paresis, etc., are also present. To enter into the different disease pictures which it has been attempted to isolate as cerebello-spinal, olivo-ponto-cerebellar, cerebro-cerebellar atrophies, etc., does not correspond to the practical end held in view in these lectures. It is a difficult and imperfectly investigated subject which is connected at many points with the hereditary forms of ataxia which we have discussed in Lecture VIII. That even total defects of the cerebellum can remain latent, in case the rest of the central nervous system presents no defect, is explainable from the slight functional dignity of the cerebellum. This is taken strictly, neither motor nor sensory in function; neither is it possible, at least in man, to sepa- rate limited centers of specific activity. In animals there is, nevertheless, ac cording to Hoik, van Rynberle, Rothmann, Lourie, and others, a certain cor- tical localization possible, which, however, with ascent in the animal kingdom, diminishes in definiteness. The results of the best experimenters, however much tiny differ from one another in point of detail, agree in showing that the cerebellum is only able to act in„a modifying manner upon the cerebrospinal unction. Luciani first attempted to determine precisely the influence of the Cerebellum upon the rest of the nervous system and distinguished a sthenic, tonic- and static action. The cerebellum, according to fhis author, reinforces the potential energy of the brain-spinal cord innervation, increases the neuro muscular tonus, and aids the continual blending of the motor impulses. The elimination of the cerebellum has on this account asthenic-, atonic and astasic disturbances as a result. Then Thomas has defended the view that the triad of Luciani depends only indirectly on the suspension of cerebellar function. 316 LECTURE XX He has shown that the cerebellum is a reflex center in the service of preserving the equilibrium, that it receives peripheral and central impulses and reacts to both, that it is not the seat of a special sense, but that of a special reaction, that this last, finally, serves equilibration in the different positions, both on reflex, automatic and voluntary movements. The animal without a cerebellum owes, according to Thomas, to his cerebellar ataxia, the weakness, incomplete- ness and disharmony of his muscular contractions also ; must, indeed, to a certain extent, try his muscles out anew. Later, Hermann Murik has laid stress upon the fact that the ataxia of the animal without a cerebellum, in whom injury to neighboring structures has been avoided, is limited to the muscles of the vertebral column and the limbs. It affects the "composite move- ments" in the service of maintaining the equilibrium on standing and walking. This finding agrees best with the clinical studies on cerebellar ataxia. The preponderant importance of this last in the circle of all the cerebellar affections, even the agencses and atrophies, stands in agreement with the result of my experiments in cutting the spino-cerebellar tracts in animals. As these tracts represent the afferent portion of the reflex for the cerebellar coordination of the trunk, so the cerebellar influence in maintaining the equilibrium of the head and neck is regulated through the vestibulo-cerebellar fibers. This, the experiments of Ewald and others have made clear. F. Infectious Diseases of the Cerebellum There is a cerebellar form of acute infantile paralysis into which we have already gone when mentioning the Hcine-Medin disease. An encephalitis can also localize itself in the cerebellum; its differentiation from abscess of this region can be very difficult, even almost impossible, as is sometimes even that from cerebellar serous meningitis, although in such encephalitis a papilledema occasionally occurs (Op.penheim and others). Italian neurologists (Puii.tini, Forli, etc.) have pointed out, finally, an acute cerebellar symptom-complex in consequence of malaria, in which after a sudden febrile beginning (with Plasmodium in the blood) there appear cerebellar disturbance of gait, muscu- lar atony, nystagmus, tremor, dysarthria, and swaying of the head. This syn- drome reacts well to quinine, speedily disappears, but has a great tendency to recurrence. LECTURE XXI Malformations, Congenital and Early Acquired Defective Conditions A. Hydrocephalus In considering the disease condition known as hydrocephalus character- ized by an excessive collection of fluid within the skull, we will in the first place confine ourselves to the congenital form. It is by far the most frequent, but is not always separable from the early acquired forms. The pathological anatomist distinguishes a meningeal or external hydro- cephalus from a ventricular or internal hydrocephalus. The first is rare, reaches on an average only moderate intensity, and is clinically not to be differentiated with certainty from internal hydrocephalus. As a rule, external hydrocephalus, also called "intra-meningeal hygroma," is a consequence of malformations of the brain, for example, microcephalus ; we speak then of a "hydrocephalus ex vacuo." The possibilities for the production of a ven- tricular hydrocephalus are manifold. So, it can represent a standstill of brain development at the stage of membranous vesicle; on the other hand, however, it may be the result of a foetal or early infantile ependymitis or menin- gitis ( the choroid plexuses of the ventricle are morphologically the result of the invagination of the delicate membranes of the brain). Further, it can be a hydrops by stasia through interruption of the venous discharge channel from tin choroid plexus and the ventricle walls; finally, it is the result of the ob- struction of those orifices through which the infra-cerebral cavities commu- nicate with the subarachnoid space. In the different directions hereditary syphilis appeal's to play the most Important etiological role; v. Barensprv/ng found, among !)!) hereditary luetics, 4 congenita] cases of hydrocephalus; Eisner, among IS hydrocephalics, •'{ children with manifest signs of syphilis; Dean, in It hydrocephalics, obtained 1 times a positive Wassermann reaction. In the second place, parental alco- holism must he mentioned. Rarely can trauma or acute infection which have acted upon the pregnant mothe> he made responsible. The connection with mental shocks during pregnancy is scientifically not proven. A few authors have called attention to hereditary family eases of the disease. The ventricle walls can he so thinned in internal hydrocephalus that the brain has become a fluctuating vesicle. The amount of fluid varies between 50 to 100 (a- and 10 liters -the average, according to Oppenheim, is about 1 liter. \"o wonder that the skull can reach enormous dimensions. The eircuin- :il7 318 LECTURE XXI ference of the head of the normal new-born infant, taken about the glabella and the external occipital protuberance, measures 34 cm, and in the course of the first year reaches 45 cm. In hydrocephalus circumferences of 60 and 70 cm are not rare. A 16-months-old patient of Frank had, indeed, a measure of 154 cm. Since only the skull proper enlarges while the face remains small, the head assumes a characteristic pear-shape; the thin bones of the skullcap can become parchment-like, the whole cranium is translucent by transmitted light. The sutures and the fontanelles gape open, the skin of the forehead and the scalp becomes thin and atrophic, and all the more prominent appear the dilated veins. Since in young children the neck muscles are not able to support the head of adult size, it wobbles helplessly about. Sometimes,, Figs. 88 and 89. Congenital Hydrocephalus. on palpation, fluctuation is perceived, while on auscultation a vascular mur- mur is heard. The skull deformity exerts peculiar morphological reactions upon the eye and ear. The eyeball is forced down and forward, the upper lid can only incompletely cover it. A large portion of the sclerotic is always visible and the upper part of the cornea rising over the border of the lower lid gives a picture of the "rising sun." The (often much deformed) ears are placed strikingly far back and low on the head (see Figs. 88 and 89). These great morphological anomalies correspond to marked functional dis- turbances. As to the intellectual capabilities, it is indeed a fact that after recovered slight hydrocephalus, striking mental capacity has been observed (which is known to have been the case in Cuvier and Hclmlioltz), but perma- nent more or less gross defects are the rule and feeble-mindedncss, imbecility or idiocy is the usual lot of hydrocephalics who do not die in early childhood. Of 41 hydrocephalics, according to Wyss, only 5 could go to school. Dis- turbances of the cranial nerves are often observed, for example, dilatation or contraction of the pupils or lack of all reaction, nystagmus, strabismus, HYDROCEPHALUS 319 grimacing (irritative facial symptoms?). In a remarkable manner hearing is almost always retained and only exceedingly rarely is there change in the optic nerve (papilledema, atrophy). Spastic conditions of the muscles of the trunk and extremities are frequent; they correspond to the clinical picture which we will describe in the next lecture on infantile cerebral paralyses and Little's disease. Epileptiform attacks, paroxysmal vomiting and the occur- rence of severe headaches should indicate sudden increase of brain pressure. Young children give evidence of the last by whimpering and putting the hand to the head. Unusual symptoms are disturbances of coordination, tremor, paralyses, and (apart from the not infrequent hyperesthesia of the integument) disturbances of sensibility. Frequently, on the contrary, there is incontinence of faces and urine, as well as a decided tendency to bed-sores ; the last occur upon the skull also (parietal bosses, occiput). A word on the hydrocephalic fluid which is obtained on lumbar or brain puncture. It is, as a rule, colorless to a slight amber tinge, without clouding, only rarely it contains a few flocculi. The content of albumen (globulin) is practically none, or very little (not over 1 per cent.). The specific gravity lies between 1,001 and 1,009. The fluid contains further glucose and milk sugar. The cytological findings are limited to the occasional presence of a few leucocytes. How relatively frequent hydrocephalus is at birth is shown best by the statements of the obstetrician Rwnge, according to which a disturbance in d< livery in consequence of hydrocephalus comes under observation about once in 300 deliveries. In this connection it should be remembered that a great many congenital hydrocephali are at birth of still too moderate dimensions to cause any disturbance. In any event, the post-natal growth of congenital liuli ocephali plays a great role: this can amount to 1 cm a week and corre- sponds to an extra-uterine duration of the original disease process. The mortality of hydrocephalics is a very considerable one. At birth there may be bursting of the head if the child is not sacrificed by the obstetrician by p< rforation and cranioclasis to prevent rupture of the uterus. Hydrocephalics born alive often die during the first days, weeks or months; the remainder of the more severe eases are taken away before the third year of life; only slight eases can recover; usually, however, it is a "recovery with defect." Not to be underestimated is the danger of relapse, renewed progressive ad- vance which threatens during all of childhood. As immediate causes of death should be mentioned: increasing brain pressure and coma, bed-sores and hjfection, laryngismus stridulus; status epilepticus, attempts at operative treatment, particularly, however, intercurrent affections, as gastro-enteritis and bronchopneumonia. In one case there was bursting of the head. Hare but interesting are the so-called '•spontaneous recoveries" in which the hydro- cephalic fluid, by wearing away of its enclosing case, makes a path out either throu»h the nose, through the orbit, or through the coronal or sagittal suture. From the point of view of differentia] diagnosis, there come into consid (•ration before everything else the rachitic enlargement of the skull, in which, However, the cranium assumes the characteristic "box form," the open fon- fcnelles are not bulging, and the hydrocephalic position of the eyes is absent ; 320 LECTURE XXI also the so-called "steeple-skull" (oxycephalies) should not lead to confusion with hydrocephalus, although it is affirmed that this deformity represents the reaction of the rachitic bones of the skull to a moderate hydrocephalic press- ure (Meltzer). It is to be emphasized that oxycephalus is almost never ac- companied by idiocy, but, on the contrary, very frequently by optic atrophy. We would mention further the peculiar skull-form of hereditarily syphilitic children, which Fournier, on account of the abnormally high and bulging forehead, has called the "Olympic" forehead ("front olympien"), and would allude further to the idiots with sclerotic hyperplasia of the brain and great skull dimensions denominated by Virchoic "cephalones," and finally, the so- called "cleido-cranial dysostosis," a heredo-family anomaly in formation de- scribed by Marie and Sainton, in which persistence of the fontanelles and excessive width of the skull is accompanied by congenital defects of the clavicles. The non-congenital hydrocephalus of childhood is most frequently of men- ingitic origin, which can manifest itself clinically by acute onset with fever, stiff neck and convulsions. After the passing off of this infectious stage, dur- ing which the spinal fluid is usually characterized by richness in albumen, and has also been found to contain bacteria (for example, meningococci), there occurs a progressive increase of fluid within the skull which, by degrees, may produce the same symptom-complex as the congenital form. Only in children beyond the second year of life the firm closure of the sutures and fontanelles which has already occurred, causes a plainly different disease picture. Only very exceptionally are the sutures forced open ; as a rule, indeed, the skull responds to increase of pressure in its interior by abnormally rapid growth ; nevertheless, such monstrous dimensions as those in hydrocephalus of the first period of life are not nearly reached, neither does the typical eye position occur. All the more intensely, however, does the increase of the intracranial hypertension make itself felt ; severe headaches, tinnitus aurium, vertigo, vom- iting, confusion, optic atrophy, spastic rigidity of the extremities, name!}', the lower ones, epileptiform attacks, etc., make the differential diagnosis from brain tumor uncommonly difficult. Here and there, indeed, the hydrocephalus distinguishes itself from tumor formation by considerable variation in the intensity of the disease manifestations. Into the treatment of hydrocephalus we can enter with but very faint hope. In every case we begin witli iodide and mercury, which even in non- syphilitic cases appear to exercise a favorable influence. From the start large doses are called for (0.15 to 0.2 KI per diem for children in the first year, 0.2 to 0.25 in the second year, ungt. hydrarg., 1.0 a day by inunction). Sub- limate baths (1.0 HgCk per bath) have but slight activity, internal mercurial treatment is not to be recommended on account of the tendency of such chil- dren to gastro-enteritis. Derivative measures whose activity cannot be entirely denied are penciling the scalp with tinct. iodi, and the exposure of the occipital region to the sun for 15 to 20 minutes a day as recommended by Somma. More rational, however, is repeated lumbar puncture (25 to 50 cc spinal fluid drawn off at intervals of 3 weeks), while the incision of the dural sac MALFORMATIONS, ACQUIRED DEFECTIVE CONDITIONS 321 to produce permanent drainage, as recommended by Quincke, does not appear to promise much result. Puncture of the ventricles is reserved for those cases in which increasing symptoms of brain pressure or papilledema appear to call for relief, but in which on account of imperfect communication between the ventricles and the spinal dural sac this cannot be accomplished by lumbar puncture. Ventricular puncture is, indeed, a dangerous procedure, which frequently leads to fatal collapse or to status epilepticus ; in any case the following precautions should be observed: the most rigid asepsis, puncture 3 to 4 cm laterally from the large fontanelle, that is, below the longitudinal sinus; slow withdrawal of at most 100 cc fluid; accurate watching of pulse and respiration ; in threatening collapse a partial replacement of the fluid by physiological salt solution. After the operation an elastic, yielding bandage should be placed about the skull, but the dangerous compression by adhesive plaster, after Trousseau, should be avoided (brain pressure, bed-sores). In conclusion, the methods of "permanent drainage of the ventricle," recom- mended by surgeons, may be mentioned; these are: draining into the subdural space (Kocher), under the scalp (Mikulicz), into the longitudinal sinus (Pa/jr), and finally, the so-called "Balkenstich" (puncture through the corpus callo- sum) (Anton and Bramann). Since in marked hydrocephalus the existing defects in the psychical sphere, even though the disease process may cease, can only to the slightest extent be removed by any method of treatment, an ethically thinking physician will enter into the task of making possible the further vegetative existence of a paralyzed and idiotic being only with repugnance. B. Cranial and Spinal Ectopies Upon the basis of preformed defects of the skull partial escape of its con- tents can occur intra-uterine and be present at birth. We speak of a cranial meningocele when only the brain membranes air displaced outward (in this the dura mater i> usually absent), of hydrencephalo- c le when tin/ hydropic ventricle also pro- trudes. By enccphalocele is meant, on the contrary, an ectopy of solid brain substance which dors not include any part of the ven- tricle. These malformations can he located on the most varied parts of the skull. They are most frequently found in tlit occipital region, sometimes above and sometimes be- low the squama occipitalis (see Fig. 90); again liny appear between the nose and I he orbit or between the nose and the frontal hone Ectopies into the nasal cavity, tlir mouth and in tin temporal region Ire rarer. Cystic protrusions of the brain and brain membranes arc greatly mcreased in dimensions after birth. Pedunculated are the meningoceles, which occur particularly in the occipital region. This form i-- the mildest, since it Fin. no. Superior Occipital Hydrocephalocele 322 LECTURE XXI is compatible with normal brain activit}'. Often enough, indeed, it is combined with hydro- or microcephaly which, along with other developmental disturb- ances, are almost regular accompaniments of the ectopies containing brain substance. The worst prognosis is given by the hydrencephaloceles ; their post-natal growth provokes brain pressure symptoms which lead to death within a few months. Good results can be expected only from the operative removal of simple meningoceles ; these should be early transferred to the surgeon, as otherwise Fig. 91. Occult Spina Bifida with Local Hypertrichosis. there is a risk of rupture of the cyst with unavoidable meningeal infection. Spontaneous cures are excessive rarities. As clinical criteria of meningoceles it is noticed that they fluctuate always in contradistinction to the encephalo- celes, and they are distinguished from hydrencephaloceles by being usually pedunculated and often translucent; also they decrease in size on pressure, and can even be replaced. In the last method of examination there is, indeed, need for great caution on account of the danger of provoking brain pressure. We include the spinal ectopies under the common designation of "spina MALFORMATION'S, ACQUIRED DEFECTIVE CONDITIONS 323 bifida." If the skin over the hernia of the spinal cord or of the meninges is present, even though of abnormal appearance (e.g., showing hypertrichosis or drawn in like a stellate cicatrix), we speak of occult spina bifida (see Fig. 91 ) ; if the hernia is exposed, however, of open spina bifida, or "Rachis- chisis" (Fig. 92). For the rest, we also distinguish here the simple menin- goceles from the forms which contain nervous tissue. The spinal analogue of hydrencephalocele is called myelocystocele. The most pronounced anom- aly, however, is presented by myelomeningocele, in which the spinal cord, fissured and drawn apart, is exposed externally and closes dorsally the summit of the meningocele sac as the so-called "Zona medullo-vasculosa." Spinal ectopics can reach the size of a child's head. They are usually situated in the lumbo-sacral region which corresponds with the embryological fact that closure of the spinal canal takes place from above downward. Clinically, in so far as there is not occult spina bifida, the tumor naturally first strikes the eye. It is characterized usually by definite fluctuation and partial replaceability. In the last test great caution is necessary on account of the danger of brain pressure; on this the fontanelles are often noticed to protrude. On the other hand, when the child cries or strains, the tumor sac becomes more tightly stretched. Myelomeningocele, which is almost always accompanied by serious paralytic symptoms, is easily to be recognized by the deep red granu- lating "zona medullo-vasculosa"; the differen- tiation of the other types, even with the as- sistance of the X-ray, is scarcely possible before the operation. Of the symptoms of motor involvement, in consequence of spina bifida, symmetrica] paralyses in the legs are next to he mentioned. Clubfeet are practically always present. Many times not only are the feel and legs paralyzed, but there is complete paraplegia — with the usually deep situation of the tumor, of flaccid character. The elec- trical reactions may he lost, the patellar reflexes are usually markedly reduced, the Achilles reflexes are generally absent. In cervical spina bifida I have also geen spastic paraplegia — a very ran' occurrence. Even when the process is located high up, the alius usually remain free; on the other hand, bladder and rectal paralyses belong to the typical symptom-complex of spina bifida. In tin ir most severe form, these symptoms are found in the spinal hernias situated low. Disturbances of sensibility air usually but slight, anil only exceptionally is total anesthesia found in the paralyzed limbs. Finally, we may mention Certain trophic disturbances (ulcers on tin- genitals, on tin heels, over the tumor itself), etc., here and there also perforating ulcers on the feet. Occult spina bifida takes a special position, in that its symptoms develop only in late childhood, even after the truth year, There occurs, then, in Open Spina Bifida (Cervical Meningocele). 324 LECTURE XXI previously healthy individuals, little by little, either bilateral pes equinovarus, weakness of the sphincters appears, which can increase to total incontinence, or, and this is the usual thing, both these morbid conditions develop spon- taneously, accompanied by more or less severe pain in the legs. This is prob- ably a result of the traction which the spinal cord fixed at the point of the hernia must suffer from displacement by growth. In many such cases lumbar hypertrichosis (see Fig. 91), a scarlike depression or a slight bulging of the skin in the lower part of the back would point to the diagnosis of occult spina bifida, which naturally must be confirmed by X-ray examination. The "myelodysplasia" of Fuchs can be considered as a rudimentary form of occult spina bifida under which is understood the following symptom- complex: 1. Weakness of the sphincters which leads to continued nocturnal enuresis. 2. Syndactylism, or membrane formation between the individual toes. 3. Disturbances of pain sense in the toes. 4. Anomalies of the skin and ten- don reflexes on the abdomen and in the legs. 5. Occasional deformities of the bones of the foot, eventually combined with weakening of the peroneal mus- cles. 6. Roentgenological evidence of imperfect closure of the sacral canal, defects of the vertebral arches, etc. 7. Exceptionally, tropho-vasomotor dis- turbances on the toes. Spina bifida is often complicated by other malformations and defect con- ditions, for example, by hydrocephalus, ectopy of the bladder, defects of the abdominal walls, etc., which naturally unfavorably influence its prognosis. For the rest, one can say, the greater the involvement of the spinal cord, the worse the prognosis, even after operative procedures. Without operation the outlook is very bad ; Wernitz, in 90 patients with spina bifida, saw only 20 live to be over 5 years old; the majority died during the first month of life. That any one reaches middle age is excessively rare. Spontaneous dis- appearance of the tumor by contraction is so extraordinary that this even- tuality need not be reckoned with ; the same thing applies to spontaneous re- covery by rupture of the cyst. The last accident is usually, indeed, fatal. If the cyst ruptures before birth, the children may be born with a fistula which, left to itself, is usually sooner or later the port of entry for a menin- geal infection. Every case which is not accompanied by other serious mal- formations should be brought under surgical treatment. C. Congenital Nuclear and Muscle Defects In the cranial nerves, and the muscles supplied by them, congenital ageneses are not so very rarely observed. Most frequent is congenital ptosis, which may be uni- or bilateral. Rarer are the following congenital paralyses: total external ophthalmoplegia, paralyses of looking in one direction, abducens paralysis, paralysis of the superior rectus ; facial paralysis, paralysis of the muscles of mastication and of the tongue ; single or combined, unilateral or bilateral, symmetrical or asymmetrical. There is also a so-called "congenital bulbar paralysis." For these absences of function, as anatomical investigations have shown, there may be, as a basis, either defects of the muscles concerned or those of the nuclei of the cranial nerves supplying them. Now, there is MALFORMATIONS, ACQUIRED DEFECTIVE CONDITIONS 325 in these last, however, an "infantile nuclear atrophy" which is to be referred not to agenesis, but to an early atrophy of these structures; these two forms, indeed, are in principle not to be separated from one another, since for the early infantile atrophy a congenital defect should be a prerequisite also. In the paralyzed muscles the electric irritability is reduced or lost, still in con- genital defects reaction of degeneration is not found. The prognosis of all these conditions is, that they will remain stationary. The relatively slight functional disturbance which results from defects of the eye muscles is noteworthy: double vision scarcely ever occurs, and in con- genital abducens paralysis, contrary to what is observed in the acquired form, there is usualty no contracture of the antagonists ; the eye, on account of this, remains in the right position as long as the glance is not turned toward the paralyzed side. Therapeutically,, only plastic operations, as, for example, transplantation of the frontal muscle into the levator palpebral superioris, come into consideration. The congenital muscular defects on the trunk and extremities are, as far as anatomical investigations up to this time permit a decision, always of peripheral nature, that is, not dependent upon ageneses of the nuclei in the spinal cord. These conditions, which Erb, Damsch, and I have recognized, attract clinical interest less by their symptomatology than through their rela- tions to progressive muscular dystrophy. It is striking that the muscle defects are observed in the first place in sucli muscles as usually atrophy frequently and early in progressive muscular dystrophy. By far the most frequent is defect of the pectoralis, which Schlesmger, among 54,000 patients of one Vienna clinic, found 5 times; I have seen 6 cases in the course of 10 years; over 200 are described in the literature. The fact that, like in the Bcapulo-humeral type of dystrophy, the clavicular portion of the pectoralis major usually is preserved, is remarkable. Although rarer than isolated muscle defects, defects of whole groups of muscles also occur; also, these affect predominantly such muscle complexes as arc known as typical localiza- tions of the dystrophies; in a boy observed by me, for example, the left pec- toralis major was absent (exceptionally, in toto), also the triceps brachii, a pari of the trapezius and the rhomboids. I do not go so far as Erb, and particularly Damsch, who raise the question as to whether congeiiital muscle defects may not he the result of an intra-uterine variety of dystrophy. It appears to me, however, that between the total inhibition of formation, on the one hand, and the congenital predisposition to later dystrophic destruc- tion, there is only a difference in intensity. Repeatedly it has been observed for the rest, that people with epngenital muscle defects in the course of their later lives develop progressive muscular atrophy. Remarkable is the excess- ively frequent a cconipanimciit of congenital muscle defects with other mal- formations of all sorts, which in defects of the pectoralis are usually located about the shoulder girdle, thorax, or arm. It is also striking here how small the disturbances of function are; so, one patient with left-sided pectoralis defect, fenced with the left hand, another shone as a rider and swimmer. These are, of course, examples of the forma- tion of vicarious synergies by the retained muscles or portions of muscles. 326 LECTURE XXI Here, also, treatment has to take hold. In the boy above mentioned, by fara- dization of the retained portions of the shoulder girdle and by suitable exer- cises I brought him to the point of being able to execute the previously im- possible, for him, "dip" on the parallel bars. LECTURE XXII Infantile Spastic Hemiplegia and Diplegia; Little's Disease ; Idiocy Gentlemen: We will to-day review a number of disease conditions which etiologically and pathogenetically quite heterogeneous, are distinguished by common clinical features. These are always the result of injurious factors which have affected the nerve centers either prior to birth, during this, or in the course of earliest childhood, and have led to spastic-paretic symptoms of hemi- or diplegic type ; the syndromes which have so arisen are not progressive, but represent either a stationary residual condition or even manifest tenden- cies to spontaneous improvement. Within these rather wide nosological limits we include as "Little's" disease the quite frequent cases which are character- ized, 1, by diplegic type, and, 2, by the so-called Little's etiology. As early as 1846 the English obstetrician Little had pointed out the predisposition which premature births, multiple births, and difficult deliveries seem to exert toward the development in the children in question of bilateral "spasticity of the limbs." In order to introduce some system into this rather complicated matter, I must first make you acquainted with the different pathologico-anatomical bases of these symptom-complexes. I will divide them according to B. Sachs, into three categories: prenatal, natal, and post-natal lesions. It is to be remarked, however, that in individual cases, usually not the autopsy, but a sufficiently clear and extended anamnesis renders possible its inclusion in one of these groups. 1, Prenatal Lesions a. Porencephaly. — As "true" or "primary porencephaly" we denominate a craterlike depression of the brain surface which sinks in toward the ven- tricular cavity to communicate* with this latter. This defect arises in the intra-uterine period through the abnormal depth or infolding of the primary fissures at an early embryonic period, or from total encephalitis and menin- gitis. These last sometimes lead also to "secondary" or "pseudo-porenceph- jtlies," cystlike defects of substance, in consequence of the shrinking of cica- trices on the convexity of the brain. Porencephalies may have a unilateral or a bilateral symmetrical location. b. Lobar Sclerosis. — In this condition, described by Virchow as "congen- ital encephalitis," there is a gliotic contraction and induration of the totality 827 328 LECTURE XXII or of the greatest part of one or, indeed, of both hemispheres. It seems to present a parallel process to the encephalomalacia of the developed brain. In its early stage, the glia tissue remains more readily preserved after processes leading to ischemia and hence, after destruction of the nervous parenchyma, can proliferate reactively, and after that contract. The diffuse fetal scleroses, hence are very closely related to the prenatal porencephalies, but owe their origin to a less degree of intra-uterine disturbance of the blood supply. Lobar sclerosis can also affect only one or both halves of the brain. c. Tuberous Sclerosis. — This anomaly was formerly considered as the hy- pertrophic contrast of atrophic lobar sclerosis. According to the newest investigations of H. Vogt, however, it is a malformation resembling that in tumors. There are found in the cortical regions, particularly in the sensori- motor zone, superficial knotty prominences which may reach the size of a nut and consist of exceedingly proliferated glia. Vogt has pointed out also the fre- quent finding of congenital heart and kidney tumors (rhabdomyoma, hyper- nephroma, liposarcoma), as well as congenital adenoma sebaceum of the skin, in patients with tuberous sclerosis. These last render possible the diagnosis of this lesion intra vitam. (1. Cysts and -foci of softening in the brain arise from the causes mentioned when speaking of lobar sclerosis, rarely before birth; where this is the case, obstruction of arteries, usually upon a syphilitic basis, are to be held respon- sible as causes for the circumscribed destruction of brain substance. These are usually unilateral lesions. e. Spinal foci of disease w-hich lead to descending degeneration of the pyramidal tracts ; these are usually, as was recognized in two cases anatom- ically examined by Dcjcrine, also of syphilitic origin. 2. Natal Lesions These furnish, when bilateral, the anatomical substratum of Little's disease. a. Development of the corticospinal tracts interrupted by premature birth. This defect appears in many cases which later strikingly improve spontane- ously, capable of being compensated to a certain degree through supplemen- tary development of these tracts. b. Hemorrhages into the brain substance, or upon its surface, or their residues in the shape of cysts and areas of softening, on the one hand, of adhesions, with the meninges on the other. The hemorrhages occur ( often bilaterally) by tearing of the blood vessels in a difficult labor or through too sudden passage of the still soft skull through the superior strait of the pelvis in premature delivery. Factors favoring them are asphyxia, twisting of the umbilical cord, brittleness of the vessels on account of fetal or maternal diseases. c. Cerebral meningitis and encephalitis post partum, which may occur from, the infection of hematomata, in case the abrasions, bruises, and circulatory disturbances which the skull and its contents suffer in difficult labor, do not suffice to open the way for the invasion of microorganisms. The results of such meningeal infections can be, among other things, the abnormal folding INFANTILE SPASTIC HEMIPLEGIA AND DIPLEGIA 329 of the cerebral cortex known as "microgyria," external, or even internal, hydrocephalus, as well as pseudo-porencephalv. d. Hemorrhages into the spinal cord or its meningeal envelopes. These have been observed after breech delivery and extraction, after turning, etc. 3. Post Natal Lesions 1. Pseudo-porenccphalies arising from embolic, encephalitic, and meningo- encephalitic processes, also through trauma (falls on the head, striking it on sharp edges, etc.). 2. Lobar sclerosis; 3. tuberous sclerosis, rarer than that of prenatal ori- gin, but still agreeing with it pathogeneticallv. ■i. Cysts and Foci of Softening. — These changes arc relatively frequentlv the bases of spastic diplegias and hemiplegias arising in the course of earliest childhood. They depend upon vascular lesions (rupture or thrombosis of pathologically altered vessels, embolism). 5. Acute Polioencephalitis of Childhood (Striimpcll). — The relatively rare localization of the infectious process also lying at the base of acute polio- myelitis, in the cerebral cortex already considered by us (see Lecture XVI, page 242), occurs unilaterally and leaves behind infantile spastic hemiplegia. Etiology Apart from the last-mentioned infectious disease, as well as from the already mentioned "Little's etiology" (premature delivery, multiple births, pathological delivery, with the injuries resulting from them) specified in speak- ing of the natal lesions, congenital syphilis plays the chief role* among the causes of infantile spastic hemi- and diplegia. Most of the just sketched pathologico-anatomical anomalies can even at the autopsy be brought info connection with existing heredo-syphilis, which, on account of the well-known (i ratogenic action of the luetic virus, on the one hand, and its injurious effect upon the vessels on the other (endarteritis syphilitica), is not astonishing. That luetic patients do not bring into the world hemiplegic or diplegic children much more frequently than is actually the case, Sachs explains by the frequency of abortion in such families. For the rest, enough cases are found in which there have been a number of abortions, while the child finally carried to term presents one of the above-mentioned prenatal lesions. In the prenatal, as in the postnatal forms, Box has recognized that they frequently give a positive result with tin "four reactions" (sec above, page 187). Also tuberculosis of the parents is not rarely found, as is saturnism and alcoholism of the Ascendants. In congenital cases exhausting diseases of the mother during pregnancy can often lie held responsible. There is often a history of physical oi- psychical trauma which has been suffered by the pregnant woman. Value is to lie placed upon these anamnestic statements mainly when it is recognizable that ;i ^ro-s trauma has affected the uterus. Sachs mentions also uremia of •Indeed, it is In In- held responsible for many cases of Little's disease (predisposition to premature delivery, brittleness of tin' vessels in luetic foetuses). 330 LECTURE XXII the mother. First-born children are predisposed to injuries fit birth and to Little's disease. On the other hand, as Ganghofncr and Freund have shown, intra-uterine injurious factors frequently affect the last children in a large family (exhaustion of the maternal organism through overexercise of the gen- erative function). We will now proceed to the description of the individual forms of infantile hemi- and diplegia. A. HEMIPLEGIA SPASTICA INFANTILIS (Unilateral Form of Infantile Cerebral Palsy) The great majority of cases arise postnatally during the first year of life, and usually acutely febrile with hebetude, headache, often also vomiting and convulsions. After these symptoms pass away a spastic half-sided paresis, usually with inclusion of the face, becomes evident. Generally the leg is less affected than the arm; the patients learn to walk again with the leg slightly bent at the knee, adducted, and rotated inward with the foot fixed in equino- varus position; the latter drags and is "circumducted"; the arm, however, and particularly the hand, remains incapacitated. In general, the same tendency to contracture is evident in it, as in the cerebral hemiplegia of adults, the arm is pressed against the side, flexed at the elbow-joint, and there is pro- nation and flexion of the hand and fingers; only a fixation of the hand in a position of hypcrextension is sometimes found. Hypertonia, exaggeration of the tendon reflexes, BabinskVs phenomenon, associated movements, etc., are naturally present. In contradistinction to what is the case in adults, marked trophic disturbances usually manifest themselves in the paralyzed extremities: they are delayed in development, the X-ray picture shows a more or less marked osteoporosis; along with the skeleton, the muscles also appear atrophic, which is not only to be considered as a result of inactivity, but as a "cerebral muscular atrophy." Reaction of degeneration is never present. Fingers and toes are often the seat of athetoid, more rarely of choreic movements (see Lecture V, pages 80 and 85) ; unilateral tremor also appears. Sensibility is, apart from atactic disturbances of the paralyzed limbs, usually intact. Apha- sia is, even with paralysis of the extremities on the right side, very rare, which is connected with the early age of the patient. The patient, early deprived of his cerebral cortex on the left side, becomes not only left-handed, but also learns to talk with his right hemisphere. Accompaniment of hemiplegia spas- tica infantilis by disturbances of intelligence and epilepsy is an exceedingly common occurrence. B. DIPLEGIA SPASTICA INFANTILIS 1. Bilateral Forms of Infantile Cerebral Palsy Among these forms, according to Freud's example, 3 types may be dis- tinguished : a. The Bilateral Hemiplegia Type. — This presents a doubling of the in- fantile hemiplegic symptom-complex (in which, however, differences in intensity INFANTILE SPASTIC HEMIPLEGIA AND DIPLEGIA 331 between right and left usually exist), and is by far the most severe variety of infantile cerebral diplegia. Also bilaterally innervated cranial nerves can be paralyzed, on which account pseudo-bulbar paralytic phenomena (see above, page 229) occur. The arms are more severely affected than the legs and the psyche intensely injured; athetosis is frequent (see Fig. 93). b. Paraplegic Type. — The majority of these cases are congenital, whether they owe their origin to prenatal lesions or are to be referred to injuries at Fio. !>:!. Diplegia Spastica infantilis ("Bilateral Hemiplegic Type"; Idiocy, Athetosis). birth. The lasl represenl ;i particularly large contingenl of the cerebral form of Little's disease. Many cases an' noticed even in nurslings, since when dressing them, bathing them, etc., the legs are held abnormally --till' and immovable. Usually, how- ever, striking disturbances firs! become apparenl when the child should begin to take his firs! steps. It is then noticed that the thighs are held in forced 332 LECTURE XXII adduction pressed together, or indeed crossed like a pair of scissors, the knees are moderately flexed, the feet in equinovarus position with the toes turned in ; upon attempts at walking, the knees and the toes rub together. The legs are decidedly hypertonic, and oppose great resistance to passive movements. If the child is placed upon a chair they do not hang down, but remain more or less stretched out straight. As the child grows older, these disease manifestations not rarely improve, so that, indeed, occasionally in the 6th or 8th year of life locomotion may be nearly normal. Still the children usually long retain walking on the toes and a slowed and laborious rhythm in progression. The legs are delayed in development not at all, or only inconsiderably, in contradistinction to the hemiplegic form. Indeed, muscular hypertrophy, as a result of the hyper- tonia, has been observed (Ibrahim and others). Exaggeration of reflexes, Babinski's, often also Oppenheim's, and the Mendel-Bechterew symptoms, are to be found in the legs ; on the other hand, the arms usually remain free from every anomaly, or they are only slightly affected (somewhat slow movement, exaggeration of reflexes). Sensibility is, as a rule, intact; on the other hand, strabismus and weakmindedness are present. You will note that in the syndrome just sketched the paretic symptoms are much less manifest than the hypertonic ones. Freud sustains with good arguments the view that the paralytic symptoms are the more marked the deeper subcortical the hypertonic phenomena, the more decided the more superficially cortical the lesion lies. No wonder that the "paraplegic rigidity" is so preponderantly frequent in Little's birth-palsy, which usually presents the correlate of meningeal hemorrhages. Where, however, in cerebral spastic diplegia in childhood, paresis preponderates over hypertonia (also a "para- plegic paralysis" exists), there are usually deep-seated prenatal lesions. c. "General Rigidity." — This also usually falls under the "cerebral cases of Little's disease." All four extremities are affected; the arms, however, much less than the legs ; also there are never in them fixed contractures, as in infantile spastic hemiplegias. Hypertonia considerably preponderating over the paretic phenomena stands in the foreground of the clinical picture. Al- most always disturbances of speech and intelligence as well as defect symp- toms upon the part of different cranial nerves, for example, strabismus, are present. 2. Spastic Spinal Infantile Paraplegia Here belongs chiefly the "spinal type of Little's disease," in which along with the characteristic paraplegic rigidity of the legs, no other anomaly can be found, namely, disturbances of intelligence, strabismus, speech disturbances, choreic or athetoid phenomena, epilepsy, etc., are without exception absent. In the spastic paraplegias arising prenatally the paresis of the legs stands far in the foreground as compared to their rigidity. Freud assigns to infantile palsy also cases of persistent uni- or bilateral chorea as well as "double athetosis" in early infancy (see Lecture V, page 86). The clinical elements of hypertonia and paresis are here replaced by INFANTILE SPASTIC HEMIPLEGIA AND DIPLEGIA 333 spontaneous movements. The recommendation to record these forms as '"infantile cerebral palsy without paralysis" I cannot accept. Prognosis In the prognostic estimation of the spastic paralyses of childhood, it is well to draw a distinction between the motor symptoms and their eventually accompanying symptoms (among which the psychical anomalies and epilepsy Fig. 94. Diplegia Spastica Infantilis ("Paraplegic Kinin the contrary, are never progressive, fre- quently, indeed, regressive. Intact intelligence improves the prognosis very Considerably, since treatment, as we «ill see, cannot dispense with the coop- eration of conscious impulses of the will and its consequent exercise on the part of the patient. Choreic and athetoid phenomena usually persist, but 334 LECTURE XXII the patients often know how to reckon with them in a remarkable manner, and, in spite of them, use their limbs for all sorts of acts. Little's and the postnatal forms, particularly, however, "paraplegic rigidity" and the spinal form, are to be estimated as entirely more favorable than the results of intra- uterine disease; where in the last there are serious defects, death usually occurs during the first months of life. I have also repeatedly seen children with congenital cerebral palsy develop later (at the time of puberty) severe organic nervous diseases or die (for example, with fibro-sarcoma of the lum- bar cord, or purulent meningitis of the convexity of obscure pathogenesis). However, the prognosis of spastic hemiplegia and diplegia of infancy, as to life, is favorable. Treatment A causal treatment comes under consideration in a large number of cases of uni- and bilateral spastic paralysis in children, in which heredo-syphilis has played a part. Anti-syphilitic treatment should be begun as soon as possible and should not be carried out, as is so often the case, with inade- quate methods. The sublimate baths (0.5 to 1.0 hydrarg. bichloride per bath) preferred by many pediatrists, for example, are at most of use as an adjuvant. Of a 1 per cent, solution of iodide of mercury (hydrarg. biniodid, 0.01; sodii iodid, 0.01; sodii chlorid, 0.08; aq. dest., 10.00),0.2 to 0.5 cc, is injected every second, third or fourth day in very young infants; in older in- fants 0.5 to 0.1 cc is injected every second day; a course of treatment lasts 6 weeks. Internally, hydrarg. protiodid or calomel can be given in milk; the doses are for the first three months: 0.001 (gr. %o)» t. i. d. ; for the rest of the first year, 0.003 to 0.006 t. i. d. (gr. %o to gr. V w ). For the second year 0.0075, for the third 0.01, t. i. d. As a substitute for the inunction treat- ment Bruno Block recommends the "plaster treatment" as very simple: one extremity or a correspondingly large portion of the trunk is covered with mercurial plaster. The plaster is left on for a week. In cases beginning with fever during the early years of life antiphlogistic treatment is in place as long as the acute stage lasts ; ice bag to the head, free purgation by several doses of calomel, eventually the application of leeches behind the ears. For the convulsions, enemata of the following composition may be given with advantage: Chloral, hydrat., 0.4 (gr. vii) ; pulv. camphorae, 0.1 (gr. ll/ 2 ); vitell. ovi I; aq. dest., q. s. ad 200.0 (5 vi). As to the causal therapy of the initial stage of Little's disease, the operative removal of meningeal hematomata after protracted labors has been undertaken by Cushing and other surgeons: the children did not develop Little's disease (perhaps they would not have done so anyhow). The treatment of the later stages in all spastic hemiplegias and diplegias of childhood is above everything else by curative gymnastics and exercise therapy. All the measures to be applied by neurologists, orthopedists and sur- geons have the one end of shaping the conditions for the regression of the spastic resistance and the compensation of the paresis as favorably as possible. As already said, what may be expected from our therapeutic endeavors, apart from the seriousness of the lesion depends, above everything else, upon the INFANTILE SPASTIC HEMIPLEGIA AND DIPLEGIA 335 mental level of the patient. At the start we have to place our chief emphasis upon the application of physical agents (electricity, massage, hot baths) ; as far as electricity is concerned, the rubbing the hypertonic muscles with the anode of the galvanic current often acts quite favorably. For this application a rather large, flat electrode is selected and all sudden variations of current strength which may act as irritants are to be avoided by careful use of the rheostat; intensity 3 to 5 milleamperes. Those muscles, on the other hand, which are not greatly hypertonic but paretic, can be treated by the labile ap- plication of the cathode; only one should limit himself to such strength of current as suffices to produce a definite contraction on cathodal closing. On the other hand, I avoid in general the faradic current, since with it not only the muscles which it is desired to influence, but also those tending to con- tracture are affected by diffusion of the current. As to bathing, according to Heubncr, hot baths are much to be recommended: 3 to 4 times a year during a period of 4 to 6 weeks, a bath lasting for 10 to 15 minutes is given every dav; beginning with a temperature of 37 C. (98.6° F.) and gradually raising it to 40 C. ( 104" F.). Also the use of natural hot baths, especially those of higher temperature, for example, Baden-Baden, Aix-les-Bains, Teplitz, Hot Springs of Virginia, Arkansas and California are indicated. Massage can be carried on in the bath; it consists in careful (not jerky) stretching of the spastically shortened muscles whose hypertonia, as Hoffti has shown, can be diminished by tapotement of the ends of the tendons; their antagonists, on the other hand, art' stroked and kneaded. It quite frequently occurs that the systematic exercise of active movements, which must be begun as soon as the intelligence of the child permits it, must be preceded by orthopedic measures; above everything tenotomy, in its modern modifications (oblique, stair-like incisions), with application afterward of suit- able bandages, splints, etc., also shortening of tendons, tendon transplanta- tions, etc. On the other hand osteotomy of the thigh below the trochanter, which has been recommended in Little's disease with the view of moving the center of gravity of tin body farther backward, appears to have little justification. Fiirxtcr'x operation ( rhizotomia posterior) has met with considerably more encouragement. In it, a number of posterior spinal roots corresponding to tin spastic muscles are cut in order (according to the mechanism explained in Lecture I, page 7) to obtain relaxation of the hypertonia. Like all other operations, however, this last also promises success only with exceedingly care- ful after-treatment, long and continuous gymnastics and exercise therapy, in Connection with which appliances like the resistence apparatus of Zander & Herz, etc., can find frequent application. (Supplementary.) The Idiocies. We have seen how often infantile cerebral paralyses are accompanied by idiocy. This last can also occur without any, or witli such slight paralytic manifestations that it entirely dominates the picture. The accurate presen- tation of its semiological peculiarities belongs to the domain of psychiatry; ;33G LECTURE XXII I would, however, sketch broadly the psychic relations common to all forms of idiocy and give you also some insight into their most important clinical varieties. Under idiocy in the broad sense are included the conditions of psychical arrest of development characterized by want or defectiveness of the intellectual functions ; the term "idiocy" in the narrower sense is reserved for those severe forms in which the individual is unable to direct his own life within the bounds of society. In the most extreme cases of this sort all ability to receive im- pressions and to form conceptions is absent ; there is "mind blindness" and "mind deafness." The speech does not even reach rudimentary development, in short, the mental level is con- siderably below that of the higher mammals. On the other hand, how- ever, the defective condition may be slight, so that a smaller or larger number of concepts develop, a cer- tain amount of education is possible by a rational way of bringing up, ability to speak is developed to a greater or less extent, etc. There are the most manifold gradations until the slighter grades which are denominated "imbecility" are reached (in this latter the mental condition, in contradistinction to idiocy, per- mits the exercise of some calling, the individual can still be designated as "intra-social"), and to the very slightest "debility" or feeble-minded- ness, whose separation from physio- logical stupidity is quite indefinite. Frequent accompaniments of idiocy are: Epilepsy (in about one- third of the cases) ; genital infantil- ism ; physical stigmata of degenera- tion (Gothic palate, asymmetry of the skull = oblique skill, plagiocephaly, "pithccoid"=ape-like formation of the face (see Fig. 95), anomalies of the teeth, hare-lip, cleft palate, prognathism, hyperdactylism, syndactylism, malformations of the ears, etc.) ; ambidextrism (in about one-sixth of the cases); backwardness in development of the body, automatisms of movement (showing the teeth, boring the fist into the mouth), grimacing, rocking the body, etc.) ; reduction of pain, temperature and muscle sense. According to their conduct with regard to the external world idiots are divided into apathetic (anergetic or torpid) and erethristic (energetic, agile or versatile). All the pathologico-anatomical lesions mentioned on speaking of infantile cerebral palsy when they affect the mechanisms of the mental functions, furnish Pithecoid Idiot. IDIOCY 337 the substratum for more or less profound idiocies as do also malformations of the brain, like true poreneephali, or focal diseases in their earlier period of development, cysts, foci of softening, pseudo-porencephaly, further, micro- gyria depending upon meningitis, hypertrophic tuberosis and atropine lobar sclerosis. Other malformations and arrests of development of the brain found in idiots are, among other things, defect of the corpus callosum and Sachs' "Agenesis corticalis," in which the cortex may appear macroscopically normal, the cortical ganglion cells, however, prove to be rudimentary. "Hydrocephalic idiocy" we have already considered in the preceding lecture. There remains to us now the task of going over a few forms of idiocy particularly marked by their physical accompanying symptoms. Amaurotic Idiocy This is a marked family disease which was recognized in its clinical peculi- arities in 188T by the New York neurologist, B. Sachs, although six years earlier the English ophthalmologist, Warren Tay, first saw and described the alteration of the eye grounds pathognomonic for this affection. On this ac- count it is usually spoken of as the "Tay-Sach's" disease. In typical cases at an age of from 3 to 6 months an infant up to this time normal, is affected by a torpor increasing until at length he lies almost continuous] v in a condition of complete apathy, relaxation and immobility, in which however, breathing, the heart beat, and the taking of nourishment is .still undisturbed. All the muscles are hypotonic, if the child is set upright the head rolls, without support, in all directions. Now, however, spastic phenomena become more and more mixed with this picture of hypotonic akinesia and fi- nally occupy the foreground. At first they are intermittent, tonic extension Spasms, finally a continual spastic condition in the place of the former relaxa- tion. Now the nutrition suffers through involvement of the swallowing and sucking mechanisms and death occurs after skeleton-like emaciation. It can be stated, as a rule, that this occurs before the completion of the second year. Only once did such a child live to be 8 years "Id. Parallel with the progressive psychic and motor disturbances proceeds the lo^s of vision with a peculiar alteration of the eye grounds; this is a clouding of the retina distributed symmetrically in both fundi surrounding the yellow spot, of whitish color hut with a cherry-red point in the middle (sec Fig. 96). Finally, optic atrophy is added to it. It is noteworthy that the typical cases practically always occurred in Jewish families originating in Poland, which raises the suspicion of descent from a far-removed common ancestor. According to Apert's collection, only two of the 166 cases which he recognized as undoubted instances of the disease formed an exception to this rule. It is paradoxical, however, that the over- whelming majority of cases of amaurotic family idiocy have not come under observation in Poland, bul among Polish dew emigrants, and for the most part in America, though also in England, Germany, Austria. France, and even in Australia. Perhaps the "transplantation" into other conditions of lit',' is an ■zciting cause. The atypical cases In which the characteristic macular altera- 338 LECTURE XXII tions are absent (here belong also the so-called "late form" described by Spiel- meyer and Vogt), do not manifest this ethnological predilection, or only to a very small degree. . The pathological anatomy of the Tay-Sach's disease is very accurately known. All the gray matter of brain and spinal cord shows marked cytological alterations (swelling of the ganglion cells, disappearance of the Nissl granules and of the fibers passing through the cell body, vacuolation, etc.). Also the retinal cells of the macula and its neighborhood are affected by the degenerative Eye-ground in Fig. 96. Amaurotic Idiocy. (After Sachs.) process, through which they become opaque. Hence the white circle about the fovea centralis. Only in the last (which has no ganglion cells) the choroid is visible afterward as before, and by contrast imposes itself as Tay's "cherry- red spot." As chemical correlative of the disappearance of the tigroid sub- stance, that of nucleo-proteid in the whole nervous system has been shown, so it is probably a constitutional disease of metabolism of the ganglion cells. Microcephalous Idiocy This form, on account of the abnormal smallness of the skull, takes on a particularly characteristic picture. We must distinguish true microcephaly (simple pure microcephaly, micro-encephaly) from pseudo-microcephaly. At the base of the former there lies a genuine hypoplasia of the brain ; in the second, the inhibition of growth and development of the brain is caused by gross intra-uterinc brain diseases. In any case, the anomaly of the skull forms IDIOCY 339 the secondary correlative of an abnormal condition of the brain. Virchow's hypothesis, according to which premature synostosis of the sutures of the skull is responsible for the arrested development of the brain, has long been aban- doned. With it also, fortunately, the "Lane-Lannelongue" operation in which by removal of segments of bone from the roof of the skull or even the separa- tion of the calvarium (craniamphitomie) the compressed brain could be fur- nished relief. Fig. 97 represents a plaster model of one of the most celebrated Fig. 97. Microcephalous Idiot. (Modeled after Life.) cases of family true microcephaly, which is preserved in the Pathological In- stitute at Basle. The peculiar form of lace which is shown in our picture has led to the designation "Bird head" or "A/tec Type." The lowest brain weights observed were 15.9 grin, in a 7 wicks' old boy; 288 gin. in a -Hi years' old woman. Microcephaly must not be confused with the "nanocephaly" which Occurs in small individuals. ' Mongoloid Idiocy As the most important clinical peculiarities <>f this form of Idiocy dis covered by Down in lH(>f>, the following group of symptoms may be men- tioned. First, the peculiar physiognomy which lias given the disease its name, tin' "Tartar" or "Kalmuck" type (see Fig. 98); flal face, wide-bridged nose. prominent cheek bones, almond eyes (often with epicanthus), with reddened 340 LECTURE XXII lids and margins, and without lashes, a grayish-brown complexion, with red cheeks which produce the impression of being painted like a clown, round skull, flattened at the back. Then the hypertrophy of the tongue with enlargement of the circumvallate papilla* and remarkable wrinkling of the surface ("scrotal tongue"). Abnormal flaccidity and softness of the muscles, ability to bring the joints into abnormal positions, like a "snake man." Often there are con- genital anomalies of the internal organs (atresias, umbilical hernia, congenital defects of the heart, arrests of development of the teeth, indications of dwarf- ism with normal ossification shown by the X-ray, but with atrophy of the end and shortening of the mid- dle phalanx of the little finger, more rarely, a tendency to partial giantism). The growth of hair is not affected, the eyebrows, indeed, are abnormally heavy. The defect in intelligence in "Mongoloids" is, as a rule, combined with quite considerable ability to fix the at- tention and reactibility, with happy mood and a great ten- dency to imitation. This exceed- ingly characteristic form of idiocy is not very rare in our neighbor- hood; Konrad Fret/ found in the Aargau Idiot Asylum at Biber- stein, among 60 inmates, 3 Mon- goloids ; in England and Scan- dinavia also 5 per cent, are found, in Germany, only 2 per cent., on the other hand, according to Kowalewsky, in the Government of Petersburg, the percentage among the inmates of asylums is 10, in the Government of Kazan, indeed, 25. Whether this last is connected with the decided Mongol admixture in the population cannot be certainly stated. According to H. Yogt, Mongolism is connected with retardation in the later stages of development of the brain ; according to Buschan, Weygandt, Fret) and others, it could be attributed primarily to disturbances of internal secretion (thymus?). The brain is often small, sometimes it presents defects in development (for example, partial defect of the corpus callosum). Often the convolutional type renders recognizable an abnormally simple development with very coarse and wide convolutions ; the cortical cells are imperfectly differen- tiated, the cortex abnormally rich in vessels. Fig. 98. Mongoloid Idiot. CRETINISM 341 Cretinistic Idiocy Those forms of idiocy which occur in connection with alteration of the thyroid gland or of its "internal secretion" and are accompanied by peculiar changes in the skeleton, demand particular attention. We include them under the common designation of "Cretinism." It may be remarked here that in the earlier, and unfortunately also in the newer literature, much confusion has been produced by bringing together under this designation different diseases of other sorts (running their course partly with and partly without idiocy) on account of superficial resemblances in their external appearance. Above everything the just described "Mongolism,'' further, fatal chondrodystrophy or achondroplasia, a form of disproportioned dwarfism (micromelia) depend- ent upon a congenital defect of the zones of direction of the cartilages of the skeleton, which does not imply any disturbance of the intelligence or any al- teration of the thyroid. We distinguish 1, sporadic, and 2, endemic cretinism. The first, again, occurs in two forms: a, infantile myxcedema, and b, thyreoaplasia congenita. We will become acquainted in the lecture after the next with the disease picture of myxoedema which occurs from the destruction or the serious inter- ference with the function of the thyroid gland and in which the mental functions are more or less seriously affected. If this myxoedema occurs in childhood there results besides this, a retardation in the growth of the skeleton, a pro- portioned dwarfism on account of injury of the cartilage, the bone marrow and the periosteum, through which infantile myxoedema takes on the picture of a sporadic cretinism. Similar, only much more marked, are the anomalies in congenital defect of the thyroid gland (thyreoaplasia). Here also the hypothyreotic pathogenesis of idiocy is very plain. More complicated relations exist in endemic cretinism which is at home in such regions in which goiter and deaf-mutism occur in great frequency. The Alpine and sub-Alpine valleys are afflicted with this severe scourge. In Switzerland, in Tyrol, Styria, Savoy, Piedmont, in the Veltlin. For Swit- zerland II chinch and Kikjcii liirchcr have established the striking fact that there is a connection between cretinism and the geological formation of the soil, that above everything the marine formations of the 1'aleozoic, the Triassic and tin 1 Tertiary, are affected, while all the fresh-water formations, also the eruptive and the crystalline rucks and the sediments of the Jura and the lower chalk remain i'rvv from endemic goiter, cretinism and deaf mutism ( /■;. liirchcr ela-ses these .'} conditions together as cretinistic degeneration). That the cause is to lie sought iii the drinking-water is certain: for instance, it has been possible to check the endemic cretinistic degeneration in the Canton Aargau by supplying the villages of the endemic district with water from healthy localities. Further, in the rare endemics in lowlands (for example, on the island of Seliutt, or along the course of the Mur in Hungary) it has been found that the affected regions get their water from rivers which rise iii cretin regions. The nature of the disease agenf and its method of action, which for the rest presupposes a pergonal predisposition, on the other hand are nol \el 342 LECTURE XXII explained, in spite of recent hypotheses. In no case can endemic cretinism in its whole clinical picture be referred to a pure thyreoaplasia or hypothyreosis. The injurious agent must primarily have other points of attack than the thy- roid gland, since the symptom-complex is a much more complex one than in both the varieties of sporadic cretinism, and the reaction to thyroid adminis- tration, as we will soon see, is scarcely ever appreciable. The weakmindedness can reach an .excessive degree: there are cretins who can never be taught to take nourishment for themselves, but must even be fed with the tube. Along with these, however, there are often slighter forms of idiocy, imbecility, and debility (feeblemindedness). The apathetic torpid form of idiocy predominates. There are, however, very disturbed, obstinate and troublesome cretins who, as the accomplished describer of Alpine cretinism, Peter Rosegger, expresses it, "are capable of the 7 deadly sins." Where they can learn to talk it is imperfect, stammering. Smell, taste, sensibility, and especially hearing, are more or less seriously affected; vision, on the contrary, is usually good. The head is usually abnormally large, more rarely, micro- cephalous, frequently asymmetrical. The nose is wide, deeply drawn in at the loot, the eyes are widely separated from one another, small, and slit-like; especially is the whole face widened. A low forehead, a dry, wrinkled skin of an unclean color, sparse or absent growth of beard, a wide mouth with irreg- ular teeth, a short neck on which a goiter is often prominent, complete the CRETINISM 343 grotesque physiognomy (see Fig. 99). The body structure is characterized in general by disproportioned dwarfism, the lack of development in length affects chiefly the lower extremities, but, however, never reaches the excessive degree observed in achondroplasia; the trunk is not only too long in propor- tion to the limbs, but it is usually very massive and plump, with the exception of the sometimes deformed pelvis. Very many cretins are the bearers of great inguinal hernias. In the X-ray picture an irregular delay in the formation of bone nuclei and persistence for a long time of the epiphyseal cartilages (until the middle of the third decade) is noted. Along with these typical forms there are in cretin countries a great many cases in whom there are only a small number of the anomalies mentioned: they -are called "cretinoids," or "half cretins.'' The Treatment of Idiocy A causal treatment is only possible in idiots where there is either congenital svphilis or where thyreogenic causes prevail. In the last there is a great difference between endemic cretinism anil the sporadic forms, however. In- fantile myxcedema reacts even in the psychical condition, often with surpris- ing improvement, even recovery, to the administration of thyroid gland prepa- rations, for example, the thyroid gland tabloids of Burroughs, Wellcome & Co., or the Thyraden tablets of Knoll (each containing 0.3 grm of the thyroid gland substance of the sheep or of the hog, per tablet). The children are given one-half to one tablet (that is, 0.15 to 0.3 gland substance) per day. In thvreoaplasia improvement is the rule under organo-therapeutic treatment, but it is usually less marked than in infantile myxcedema and there is practi- cally never recovery. Endemic cretinism is still less suitable for thyroid treat- ment. In slight cases, indeed, Magnus-Levy and Wagner v. Jauregg have Obtained improvement, but this almost always fails to appear. The transplan- tation of viable thyroid into the spleen of a cretin as I'nj/r has undertaken, has only a certain influence in encouraging growth, none upon the psyche. The pedagogic treatment of psychical defect conditions of every pathogenesis is only hopeless in those of the highest degree and accomplishes indeed, in the majority of cases, as among others, Boumeville, has shown upon the enormous NKiti rial of the Bicetre Asylum at Paris, satisfactory and encouraging results. The instruction of idiots has become a scientific-ally well founded specialty. In order to develop the muscular sense in these children we usually begin by pstructing them In the use of their extremities (by the aid of their faculty of imitation) for all actions of daily life (also eating, etc.). At 4 or 5 years the education proper begins, which firs! has to render of use those capabilities Much have remained least rudimentary, also the instincts present (lor e\ ample, the desire for lid hits), are utilized for the accomplishment of results. for fixing the attention, etc. All this can only be ace plished iii a way promising results In an institution. On this account idiots should in all cases Ee removed from home in the 5th year. With tireless and intelligent manage- ment a large part of them learn in time to read and write and many of them can in any case learn to employ themselve8 as helpers in healthy manual occu- 344 LECTURE XXII nations (farming, gardening, etc.). In Basle the endemic cretins have to such an extent a monopoly of the peddling of sand that the terms "Sand-mannli" and "Sandwybli" (little sand men, little sand women) in the popular speech are equivalent to "cretin," and they are able to carry out this modest but useful occupation with entire satisfaction. Further mental development of idiots is terminated only at about 20 years. LECTURE XXIII Dysglandular Symptom-Complexes Gentlemen : We will occupy ourselves in this lecture with several disease pictures for whose clinical symptoms acting particularly upon the nervous system, as in certain forms of the idiocies just considered, anomalies of "in- ternal secretion" of different glands, play the most important pathological role, and which on this account I hence bring together as "'dysglandular." As is known, specific substances which are secreted by the definite glandular structures into the blood represent an important physiological factor of our organism in which they have to stimulate in a chemical way, certain functions. From op/idto= I stimulate, is derived the name "hormone" proposed by Starling for such substances. A particular affinity of a great number of hormones for the nervous system or certain parts of it, is just as important from a patho- genetic point of view, as the fact that the disturbance of function of a gland with internal secretion (they are also called "endocrine glands") can involve also one or more of the other glands, so that it is sometimes difficult to decide certainly as to which organ was primarily affected. I will confine myself chiefly to the discussion of such disease conditions in which the nature of the endocrine disturbances are relatively plain and generally recognized, namely! 1, Basedow's disease; 2, myxoedema, — both of thyreogenic origin; 3, Addison's diseas< — a consequence of disease of the adrenals, and, 4, different syndromes connected with disturbed function of the hypophysis. The future will show bow many of the dvskinetic conditions with which we have already occupied ourselves can definitely be arrayed among the dysglandular affections. For tetany, as we have seen, this is very probable, but also in relation to paroxys- mal paralysis, paralysis agitans, etc., the view that they are dependent upon disturbance in the action of hormones is coming more and more to the front. I. Basedow's Disease Tin's affection was comprehended in its clinical individuality, and described simultaneously (about 1K40) by tin' English clinician Graves, and the Rlerser- burg physician Basedow. There are. however, exact descriptions of a few casts from an earlier time, among which those of the Italian Flujiini (1802) deserve Bpecial mention. "Morbo di Flajani" IS also a name for the disease used ill Italy, while the English designation is "Graves' disease." Besides "maladie de Basedow," the disease i- also culled in French after two of its most striking symptoms, "goitre exophtalmique," while the German term, "Glotzaugen- krankheit" has become almost obsoleti . .-5 15 3iG LECTURE XXIII Symptomatology The fully developed cases of Basedow's disease are characterized by the unmistakable and characteristic combination of four cardinal symptoms about which -again are grouped a number of less striking phenomena. Recognizing that opposed to these classical cases there are a great number of "Formes frustes" with rudimentary symptomatology, we will begin with the considera- tion of these cardinal symptoms (goiter, exophthalmus, tachycardia, tremor). 1. The Goiter. — This is in general not an excessive struma formation, but only a moderate hypertrophy to about double the size of the healthy thyroid. Usually at once perceptible upon near inspection of the neck, it is often only plain upon palpation. Either both lobes are equally enlarged, or the in- crease in volume is especially of one lobe, remarkably more frequent on the right. The goiter is usually soft and as auscultation for murmurs shows, is very vascular. Not rarely an arterial thrill may be detected upon palpation, and besides this, a definite pulsation of the gland. This richness in vessels well ex- plains the great variations in the size of the struma, which can sometimes be noted when it is measured regularly. For the rest, it swells also upon exer- tion, excitement, etc., sometimes quite plainly. Within the soft tissue a few more resistant portions are to be felt in some places. In general the Basedow goiter, mainly in consequence of its gen- eral softness, produces much fewer sub- jective symptoms than an ordinary struma of the same size. Pressure symptoms are usually absent entirely; asphyxic difficulties proper, from com- pression of the trachea or the recurrent nerves only very rarely occur, while a feeling of fullness and tension in the region of the thyroid gland is somewhat more frequently complained of. 2. The Exophthalmus. — The protruding eyes ("Glotzauge") of Basedow patients when typically developed, give an uncommonlv characteristic expres- sion of countenance; this has been called the "tragic look," and as a fact, the physiognomy in advanced cases reminds one of certain masks of ancient trage- dies (see Fig. 100). In general, however, I should characterize the expression which the exophthalmus gives to the features of Basedow' patients rather as that of anger, to which, also, the swollen neck contributes its part (see Fig. 101). In less intense development, the exophthalmus, produces rather the im- pression of a glistening eye ("Glanzauge") than of a protruding eye, while Fig. 100. "Tragic Look" in Basedow's Disease. DYSGLANDTLAR SYMPTOM-COMPLEXES 347 on the other hand, particularly marked protrusion of the eyeball can cause inability to close the lid and has even been responsible for luxation of the eye from its socket. The exophthalmus is usually symmetrically developed, though in a considerable proportion of cases there is a distinct difference between right and left. That the intensity of the phenomenon shows great variation in the course of the disease is quite usual, so that even a marked exophthalmus can disappear again. Three phenomena are to be emphasized as characteristic accompanying symptoms of the protruding eyes in Basedow's disease: 1. "Stellwag's sign": winking is abnormally infrequent in such patients and the palpe- bral fissure is unusually wide, so that below and above the cornea si more or less wide strip of the sclerotic is visible. 2. Graefe's sign: in looking down a disturb- ance in the physiological syner- gy between the movements of the lid and those of the eyeball is apparent, so that the upper lid lags behind instead of sinking synchronously with the sagittal axis of the eye. 3. Mobiiis* sign: the movement of con- vergence of the eyes occurs im- perfectly or is quickly impaired. This last-mentioned phenomenon is less constant than the symp- t s of Stellwag and Graefe. 3. The Tachycardia. — The pulse is permanently quickened, so that even when lying quietly on lb,- back figures of 100. 120, and even 100 beats to the minute are observed. Changes of position, exertion of any sort, but particu- larly psychical excitement, increase the pulse frequency excessively, so thai e\in 200 beats a minute may occur (usually with a feeling of severe palpita tion of the heart). The rhythm of the pulse, however, remains nearly always " gular. Cardio-vascular symptoms accompanying this permanent tachycardia are quite frequent and manifold. I would mention above everything the in- creased vasomotor irritability of the integument which, among other things, Manifests itself in so-called dermographism: stroking the skin with a blunt ob- ject produces, after a few seconds, wide vn\ stripes in which the drawing or writing produced in this manner only pales and disappears after several hours. Often the dermographism is combined with a serous infiltration of the skin which leads to the production of wheals: we speak then of factitious urticaria. Congestion of blood about the bead, temporary redness which can alternate 348 LECTURE XXIII with marked pallor, intense feeling of heat in extended regions of the surface of the body, are further phenomena belonging here. The blood pressure I have sometimes found abnormally high; for example, from 140 to 185 (meas- ured with Gartner's tonometer on the patient's sitting quiet), and abnormally labile ; still, these conditions vary from day to day within wide limits. Over the heart systolic murmurs are sometimes to be heard, namely with increased tachycardia. Their maximum intensity is usually at the base; these are (as the inconstancy and not rarely quick disappearance of these phenomena show), as a rule, functional murmurs due to relaxation of the atrioventricular orifices and a resulting insufficiency of the mitral and tricuspid valves ; the character of these murmurs is soft and blowing. Also, by percussion and orthodiagraphy a certain, usually inconsiderable, degree of dilatation of the heart can be demon- strated in some cases. 4. The Tremor. — This is an exceedingly rapid and fine tremor (8 to 10 oscillations a second), which is to be perceived in the outstretched fingers of the patient, but which we can often plainly appreciate when we place a hand upon the head or upon the shoulder of the standing or sitting patient. Upon movement, intensity of this tremoi usually increases, while it may cease upon complete rest. When now the four cardinal symptoms are present, along with which, be- sides, a number of the ocular and cardio-vasomotor accompanying phenomena already mentioned are always to be found, we speak, as already said, of the classical form of the disease. Frequently, however, we meet with '"formes frustes" in which either the exophthalmus or the enlargement of the thyroid, or even both, are absent, and only the heart and vascular anomalies and the tremor can be found. If, now, for a diagnosis of Basedow's disease, the presence of these two "obligatory" cardinal symptoms would suffice, they must present, of course, those clinical peculiarities which I have pointed out to you. In spite of this, however, the inclusion of rudimentary cases of this kind under exophthalmic goiter is not justified if they do not present besides some of the rather rich array of the so-called "secondary Basedow symptoms" with which we will now become acquainted. The following disease manifestations of subjective and objective nature play, along with the cardinal symptoms in the clinical picture of Basedow's disease, a role varying from case to case and presenting manifold combinations, but yet quite important : a. Muscular Weakness in the lower extremities, often in the form of a peculiar, paroxysmal paraparesis, so that the patient's legs, while walking or standing, suddenly give way at the knees. Rarely, however, does this weakness increase to a degree causing the patient to be bedridden, and is even then tem- porary, since this symptom in particular is subject to great variations during the course of the disease. Infrequently do these transitory pareses assume an- other localization, for instance, hemiplegic or monoplegic, or affect the neck muscles or the regions innervated from the brain. In the last case there may be not only temporary pareses, but, as Stellwag has shown, actual paralyses of' the external eye muscles, while the muscles of accommodation and of the iris are always spared (except as an extreme rarity). Here belongs also the im- DYSGLANDULAR SYMPTOM-COMPLEXES 349 possibility of drawing deep inspirations sometimes observed ("Louise Bryson's sign").' b. Disturbances of Menstruation. — Dysmenorrhcea is very frequent; ainen- prrhoea not exactly rare. c. Diarrhoeas. — Charcot and Mobius have pointed out the great tendency of Basedow patients to profuse diarrhoeas occurring in attacks sometimes last- ing for days and weeks, which begin without any apparent reason, defy opium, bismuth, tannin, etc., and then suddenly and for no apparent reason, cease. The evacuations (up to 10 and more per day) are very thin and often entirely painless. d. Hyperidrosis. — Excessive sweat secretion may he general or it may be limited to a definite portion of the body (face, hands, feet): it may be con- tinuous, or there may be profuse outbreaks of sweat occurring in attacks usually coincident with congestions and palpitation of the heart. Also in such patients who do not complain of excessive sweat, even when the tem- perature is low, the skin is found in the condition for which I would borrow the French expression "peau moite" (moist skin). Yigouroux's phenomenon is to be attributed to this abnormal moisture of the integument: this is re- duction of the electrical resistance. If this last is in healthy persons about 4.000 ohm-, in Basedow patients it often sinks to 1,000 ohms and below. To obtain a certain number of milleamperes in galvanization, we need in these pases to switch in only one-quarter as many elements as for a normal person, corresponding to Ohm's law: _ . _,, ,, Electromotive Force Current strength = — . , Resistance c. Psychic Changes. — Most Basedow patients show a marked hasty and restless manner, a '"feverish motor impulsion," which, however, is accompanied bv great defecfin staying power when the task is once begun. They find it difficult to remain long seated or standing in the same place; their mood is extremely labile: their expressions of joyful, of sad emotions are boundless, and in conversation they are wordy, precipitate and inclined to deviation from the subject under discussion. Their surrounders often complain of the ex- aggerated sensitiveness, the irascibility, the capriciousness of such patients. Like the other disease symptoms these psychic symptoms are subject to the i variations in their intensity, can disappear in the course of the affec-" tion and reappear. .More severe psychic disturbances, psychoses proper, de- velop in some, fortunately rare cases. Homburger has furnished proof thai a specific "Basedow insanity," as was asserted by former authors, does not exist. Rather doc s Basedow's disease occur in connection with almosl all forms of insanity, a coincidence' which probably is to be explained through the st, mi t lines severe iieuro-ps vchopat hie heredity of Basedow patients, /. Neuralgic Phenomena. Eye neuralgias, intercostal neuralgias, pain in the trigeminus distribution, particularly in the upper branches, are the most frequent manifestations of these troubles, not rare in Basedow patients. The pains are. as a rule, of only moderate intensity; paresthesias may occur also, g. Signs of over irritability in the neuro-muscular apparatus. Light tap bing of the muscles usually calls forth a definite contraction, and by firmer 350 LECTURE XXIII striking, one can bring out sometimes the phenomenon of "idio-museular con- traction" with which we became acquainted when speaking of tetany. On the other hand the appearance of either mechanical or electric overirritability on percussion or on galvanic stimulation of the nerve trunks is very rare, while only as a very unusual occurrence has the appearance of tetanoid spasms been described. As to the "choreiform movements" to which, among others, Ray- mond and Dieidafoy have alluded, I believe that they may have been only a specially striking degree of intensity, of the already mentioned psycho-motor restlessness of Basedow patients. In a great majority of cases you will find the tendon reflexes, very often also the skin reflexes, abnormally lively. h. Trophic Disturbances. — Falling out of the hair, combined with dryness, brittleness and lack of lustre has been considered by Curschmann as a very important Basedow symptom and actually occurs in my experience in about 60 per cent, of the cases, however, usually only to a moderate extent, so that the patient's attention must be called to it. Less frequent are pigment anom- alies, usually in the form <>f a hyper-pigmentation, about the face, neck, ab- domen, etc. (similar but less intense than those which we will soon meet when describing Addison s disease), here and there, however, on the contrary, in the form of a pathological loss of pigment, vitiligo. As rarities may be men- tioned, also, a firm but fleeting oedema on the body and the lower extremities, further, abnormal softness of the bone and atrophic changes in the breasts, while the observations of sclerodermic phenomena (see below, Lecture XXV) in Basedow patients (Lathe, Stahelvn and others) practically do not indicate anything more than a combination of two distinct disease conditions. i. Anomalies of Metabolism. — The investigations of Fr. Mutter, Magnus Levi/, Scholz, and others have proved that in Bascdozc's disease the excretion of nitrogen, carbonic acid, phosphoric acid can experience a pathological in-, crease, which probably depends upon increased destruction of albumin in con- sequence of an elevation of the total metabolism. Xo wonder, that, as a rule, even when taking plenty of nourishment there is often a tendency to extreme and rapid emaciation (in one case of Mannheim's the weight fell 45 kilos, in 10 months). Huchard lias referred to "crises of emaciation" occurring parox- ysmally. On the other hand, there arc Basedow patients who are corpulent and remain so. Polyuria, eventually with polydipsia, is not entirely rare, also alimentary glycosuria, while true diabetes mellitus has only been de- scribed in a few cases (mostly as a complication in the advanced stages). Rises of temperature during shorter or longer periods are also an unusual symptom, while transitory albuminuria of slight degree (without casts) is somewhat more frequently found. A\ Blood Changes. — As characteristic for Basedozc's disease, Kochcr con- siders the following hematological finding: actual increase of the number of lymphocytes, causing relative diminution of the polynuclear leucocytes, with red corpuscles normal in number and in hemoglobin content. Course and Prognosis The disease, which affects by preference the female sex in the second and third decade, but occurs even in children, begins usually, but by no means DYSGLANDULAR SYMPTOM-COMPLEXES 351 always, by the generally gradual, only exceptionally sudden, appearance of a "cardinal symptom," and, indeed, in almost two-thirds of the cases with the heart troubles, more rarely with the struma or the tremor, most rarely with ocular symptoms. The further course is only in the mildest cases subchronic (of several months' duration), in cases of moderate severity chronic, remittent and intermittent, extending over years. Malignant cases with great intensity of the symptoms characterize themselves sometimes by acute or hyperacute course (in cases of Trousseau and Fr. M tiller, after about 2 months, in one case of Mackenzie, in 3 days, after the abrupt beginning of the first symptom, death occurred). Usually, however, severe disease pictures develop through the chronic, progressive increase of originally slight disturbances. The prog- nosis depends in the first place upon the intensity of the clinical manifestations and upon the possibility of appropriate care and manner of life; if this last condition can be obtained, even apparently hopeless cases, though not cured, may again be brought into a tolerable permanent condition. Along with the duration of the disease, for the prognostic estimation of the individual ease, the condition of the heart comes into consideration above everything else, since heart weakness, with its consequences (which we naturally do not include under the symptoms proper of Basedow's disease), also anasarca, ascites, oedema of the lungs, congestion of the liver, congestion of the kidneys, asystole, form along with general marasmus the chief proximate causes of death in the lethally ending cases. A specially wretched complication of the most severe Basedow pases may he mentioned here: the keratitis due to the excessive exophthalmos, the inability of the eyelids to close and the drying of the cornea, which may lead to the perforation of the eyeball and panophthalmitis with all its frightful consequences. Pathogenesis and Etiology How is this peculiar disease picture, so rich in symptoms, produced? This question (in consequence of the inconstancy and ambiguity of the sparse dis closures which pathological anatomy has furnished us) it has been attempted to answer in many different ways. Many of these theories have long ago fallen into oblivion after their all too apparent conflict with the data of actual observation has been shown: for example, it was suggested that the symptom-complex was due to the compression of the vessels and nerves of the neck by the goiter. There have remained as a subject of discussion only those views supported upon facts which either have asserted a primary nervous basis for Basedow's disease, or whieh have spoken Jfor the thyreogenic autotoxic nature of its symptoms. Today lioth views have justification; both are sanctioned by the result of clinical and experimental observation. It is not understandable why it is attempted to bring these two views into antithesis witll one another. That since Charcot. Basedow's disease ha- been included among nerVGUS diseases has a good clinical hasis. First, the hereditary and family rela- tions to the psychoses and psychoneuroses which are recognizable in most cases; second, its frequent coexistence with almost all forms of psychoneuroses and insanity which either precede the onscl of Basedow's disease begin 352 LECTURE XXIII simultaneously with this last, or develop in its later stages ; third, its occur- rence as a complication in organic diseases of the nervous system (tabes, mul- tiple sclerosis, paresis) ; fourth, finally, its beginning in countless cases in connection with a fright or some other psychical shock. But why cannot this neurosis be a secretory neurosis of the thyroid? Do we not know numerous cases of the quantitative influencing of the most varied secretory processes by functional as well as organic affections of the nervous system (nervous gastroxynsis, sialorrhoea, colica mucosa, etc.)? On the other hand, it cannot be denied that there are cases enough in which the functional anomaly of the thyroid gland appears to play the role of a "primum movens," the remaining nervous and vascular components of the syndrome that of secondary symp- toms. These cases have been denominated as "Basedowified goiter" and sepa- rated from true goiter. We can easily class both categories together and assume that the anomalies of the thyroid gland function coming into question arise not exclusively upon the basis of the neurosis, but sometimes also upon that of the local affection. Indeed, for both varieties, according to the results of clinical experience and experimental investigation, the two following points may be regarded as proven : 1. In the center of the disease picture stands the altered thyroid gland function. 2. The point of attack of the toxic action proceeding from this lies in definite portions of the nervous system. Recognition of the first forms the basis of the "thyreogenous theory" of Basedow's disease. This is supported by a number of experiences which I will shortly indicate. Cure of Basedow's disease by thyroidectomy; appearance of Basedow's symptoms in inflammation of the thyroid or carcinoma, after excessive use of thyroid tablets for therapeutic purposes ; identity of the anomalies of metabolism on thyroid feeding with those of Basedow's disease (Magnus Lei//) ; acute appearance of Basedow symptoms on the overwhelm- ing of the organism with expressed thyroid gland products, as sometimes occurs in goiter operations, the so-called "acute post-operative thyroidism"; more or less extensive analogy of the experimental hvperthyreosis (transplan- tation, feeding, injection experiments by Ballet, Enriquez, Lanz, and others) with the picture of human Basedow's disease.* As to the point of attack of the thyreogenous noxious agent, it is to be placed either in the sympathetic or in the medulla oblongata. The sympa- thetic theories (Bencdiht, Friedreich, Etderiburg, Abadie) have much that is seductive, as the tachycardia, the vaso-dilatation, the ocular symptoms, the hyperidrosis, etc., can well be brought into connection with disease of the sympathetic, as you will soon recognize in the lecture on sympathetic affec- tions. If we do not find cither the assumption of simple irritation or that * The value of this last demonstration has experienced a considerable limitation through the discovery of E. Bircher, that after transplantation of viable thymus into the abdominal cavity of dogs, Basedow's symptoms also appear. However, the relations between Basedow's disease and the most varied glands with internal secretion is an exceedingly interesting, though still insufficiently investigated subject. Whether the extirpation of remains of the thymus proposed by Garri will establish itself in the treatment of Basedow's disease remains to be seen. DYSGLAXDULAR SYMPTOM-COMPLEXES 353 of paralysis alone satisfactory, this is not in contradiction with the experi- ences of neuro-pathology, in which the most varied examples of combined irritative and defect symptoms in the same nerve territory are found. Rather must the absence of pupillary symptoms arouse consideration. Anatomical changes in the gangliated cord have been described in isolated cases, and the physiological proof has been furnished that the tachycardia produced by thyroid juice depends upon irritation of the accelerator nerves. Of late, however, those who hold that the point of attack of the noxious agent in Basedow's disease is in the bulb have become more and more numer- ous. More important than the heterogeneous and not very convincing autopsy findings (into which I will not enter) are the experiments of Filehrie, Durdufi, and Bienfait, who in animals set up a part of the Basedow syndrome by lesions of the restiform bodies, and especially those of Tedeschi, in which the experi- ment only succeeded when the animals were in possession of their thyroids. Personally, I am of the conviction that still other parts of the nervous system come into consideration, since how can we otherwise explain the psychic and paraplegic disturbances? Allow me to anticipate somewhat, and to remark that myxoedema, with which we will occupy ourselves after Basedow's disease, and which depends upon secretory insufficiency of the thyroid gland (whether on account of its opera- tive removal or its pathological elimination), may be considered the exact clinical antithesis of the latter. In myxoedema metabolism is reduced, in Basedow increased; in the former the temperature is reduced, in the latter increased; the skin in the first is dry and thickened, in the second hyperidrotic and thin; on this account, in the first instance there is elevation, in the second reduction of the electrical resistance; in myxoedema there is corpulence, in Basedow almost always emaciation; in the one there is small and slow pulse, in the other bounding and rapid pulse; here mental slowness, there excitation; in the former the movements are stiff and slow, in the latter hasty and tremu- lous, etc. Mobius first expressed the idea that in Basedow's disease there is overproduction of a secretion which, produced in normal quantity, is intended to neutralize the toxic product of metabolism, which one can designate briefly as "myxedema poison." Also in Basedozc's disease there is a pathogenic action of this excess of hormone, its symptoms are of a hyperthyreotic nature. The substance at fault is probably in the first instance iodothyrin, discov- ered by Banmann, for which also speaks the tact that iodin medication exerts an unfavorable influence upon Basedow's disease; indeed, as Kocher and others have shown, forced iodin cures lead to a syniptoniatologically similar disease picture, the so-called "iodin-B asedow." Indeed, iodothyrin is not the sole active constituent of the thyroid gland, but still other substances (organic- bases, thyreoproteid, etc.) come into consideration. Just to this multiplicity of active substances, which in pari seem to show antagonistic action, I havi ascribed the fact that certain symptoms of Basedow's disease and of myxoe- dema are the sam< — irregularity of menstruation, pigmentation of the skin, digestive disturbances, falling out of hair. Hyperthyroidism may well be accompanied by dyst hyroiclism (faulty composition of the secretion). Xow as to the "formes frustes" without enlargement of the thyroid gland, 354 LECTURE XXIII it must be pointed out that a secreting parenchyma may be found in chronic functional hyperactivity without hyperplasia. (I would remind you, for ex-* ample, of gastrorrhcea acida.) As Oppenheim suspects, however, the obstruc- tion of the intralobular lymph channels — a regular lesion of the Basedow thyroid can cause an inundation of the venous blood with the products of secretion of the thyroid. It is, however, also conceivable, that exceptionally the sympathetic or bulbar lesion-complex characteristic for Basedow's disease may be produced by other factors than thyroidism. Treatment The treatment of Basedow's disease in most cases makes great demands upon the persistence of the physician and of the patient. Though it is not rarely permitted to us, even after a short time, to note decided improvement, we should not be too ready to fold our hands and let the patient get out of sight. Only by therapeutic effort, extending over months if not years, will it be permitted to us, first to render durable the improvement obtained, then to increase it, and finally, often enough, to procure permanent recovery. Of capital importance in this connection is careful regulation of all the conditions of life of the patient. Except in very mild forms, one will do well to begin the treatment with several w T eeks' rest in bed, which also, in the further course of the disease, may be occasionally introduced with advantage. The excitability and the tachycardia are usually quite plainly influenced by this simple measure. As an ideal regime is to be regarded, the ovo-lacto- vegetarian diet which permits the preparation of quite tasty and varied bills of fare ; besides this, we forbid the stimulating spices. The avoidance of irritants which the spices and the extracts of meat prove themselves, acts not only sedatively upon the nervous system in toto, but specially plainly upon the cardiac and vasomotor innervation ; also tea, coffee, alcoholics and tobacco, are best forbidden upon the same grounds. Some concessions, especially with regard to the use of meat, can be made according to the individuality of the case. Where possible, however, it should always be brought about that the extractives are only taken in small quantities, in that, for example, the portions of fish, poultry, veal or beef allowed daily should be taken after the juice is expressed. The tendency to diarrhoea is not to be too much considered in the choice of food, since the character of the latter appears to be of slight influence upon the intestinal troubles of Basedow patients. Where milk is unpleasantly laxative, I rub up 10 grms of pure powdered gum arable in some cold milk, then add it to a 14 liter of milk, boil it, and finally add 1 bitter and 1 sweet almond, each blanched and ground. Next to rest in bed, it is well to prescribe freely sojourn in an elevated region. Besides moderate elevation, 2,500 to 4,000 ft., in most cases residence in the high valleys, from 4.000 to 6,000 ft., can be recommended with advan- tage. The Engadine furnishes all the degrees of elevation coming into consid- eration. But also the Black Forest and other wooded hill regions give satis- factory results, while residence by the sea is usually disadvantageous. A cer- DYSGLANDULAR SYMPTOM-COMPLEXES 355 1 measure of bodily exercise in the open air, increased little by little, should united in climatic cures with several hours daily reclining in the open air. >rts proper, as well as dancing, should be strictly forbidden; automobile ng also appears to me to act disadvantageously ; excitement is to be avoided much as possible, especially that in the sexual sphere. Under hydrotherapy there come into consideration carbonic acid baths, cool frictions, tepid fan or rain douches of short duration, slowly cooled from 20 to 18° C. I thoroughly disapprove of "cold water cures" proper. Electric treatment in different forms has been applied. In my experience, however, good results are obtained only from the stabile galvanization of the sympathetic in the neck (anode over the top of the sternum, cathode at the ungle of the jaw, the current is slowly raised from 3 to 5 milleamperes, 5 minutes' application to each side), provided that daily treatment can be car- ried out for several weeks successively. Our list of drugs furnishes some agents of valuable efficiency in the appli- cation of which you will do well to vary from time to time. In the first place, [ would mention sodium phosphate, which, given in adequate doses (6, 8, 10 ^rm per day, dissolved in water, soup or milk), appears to me to act almost is a specific on most .of the Basedow symptoms. Whether it exercises this iction through its property of being an antidote to iodin, as Kocher thinks, >r in some other pharmacodynamic manner, I will not attempt to decide, and mlv affirm the imperative fact; sodium phosphate must be given in relatively arge quantities, since it is only to a small extent absorbed. More absorbable md assimilable are the organic phosphoric combinations, for example, calcium flycero-phosphate (0.25 grm 4 times daily). I only give it preference over odium phosphate when this last sets up diarrhoea. We give bromin and valerian preparations symptomatically for jactita- ion. for which the List is in general to be preferred, since besides this it s the most efficient internal medication which we have at our disposal for he cardiac and vasomotor disturbances of innervation. The result is, how- ver, only to be expected with some certainty when the valerian preparation s given continuously and in large doses which I have denominated "valerian aturation." By preference I order valerian tea. It is most efficient when t is taken according to the following directions: In the morning the patient (Ids a heaping tablcspoonful of valerian roof to a large cup of cold water nd lets it stand all day, occasionally stirring it ; before bedtime, then, it is iltered through linen, expressed, and the concentrated infusion so obtained is ucen. The next cup of tea is immediately prepared in the same manner, is >ut on the night table, and taken immediately after awakening. Boiled \alc ian tea, or that made with hot water, acts much more weakly, since many of he ethereal components and volatile acids are thereby driven off. The wretched aste of valerian tea has created a need for a number of new valerian specialties Valyl, Bornyval, Gynoval, etc.) which, given in proper doses, also act quite ■'II. but are rather expensive and only applicable to elegant practice. I would warn against the use of digitalis and strophanthus except in such tages of the disease in which heart insufficiency and asystole dominate the ucture. Apart from the above-mentioned indications in which they fill a 356 LECTURE XXIII decided indication, these cardiac remedies accomplish nothing in Basedow'^, disease, if they do not, indeed, injure. Belladonna preparations and atropin, which are adapted to the symptomatic treatment of the hyperidrosis, have in certain cases also a favorable influence upon the disease picture as a whole, as Gowers and Grasset have shown. The use of ergot preparations and quinine appear to me to rest more upon theoretical grounds — attempting to act upon the vessels — than upon favorable and empirical results. Arsenic medication, on the contrary, properly enjoys the greatest popularity in the treatment of Basedow's disease; beginning with small doses as customary. we gradually increase to larger ones ; for instance, acid, arseniosi, 0.002 tc 0.005 (gr. 1/30 to gr. 1/12) twice a day, best in pill form, as Asiatic pill or sol. Fowleri, 3 to 7 drops t.i.d., or sodium cacodylate, daily 0.05 to Q.\l (gr. % to gr. 2) subcutaneously. Also courses of Levico-, Roncegno-, Va Sinestra or Durkheimer arsenic waters are in place. Most interesting are the attempts at opotherapy based upon the thyroge nous theory of Basedow's disease. So it has been attempted to introduce int< the patient the antagonistic "myxoedema poison." Already, in 1895, Bala and Enriquez had injected the serum of dogs in whom the thyroid had beei removed, with encouraging results; only since 1901, however, lias a serun preparation more simple in application the "antithyreodin" of Mobius-MerM which is obtained from the serum of sheep deprived of their thyroids and ordere. in doses of from 1.5 to 6.0 a day, has come into commerce. In practice th use of the flesh of thyroidectomized animals (Sorgo) and the serum of myxce dema patients (Burghardt-Blumenthal) have fallen entirely out of use, stil more so the anthropophagous methods of Lanz and Mobius (the admimstra tion of thyroid substance from cretins). On the other hand, the milk o thyroidectomized goats (introduced into commerce in a pulverized conditio as "Rodagen") has come into use after the advice of Lanz. Finally, in Franc the glycerinized blood of thyroidless animals has been given as "Hemato-ethj roidine." Most recently it has been endeavored to prepare a serum containing th specific cytolysines directed against the thyroid gland; the interesting result of various experimenters (Marikowslcy, MacCallum, Lepine, and others) ha\ not, however, proved of practical value. The pathogenetically so rational opotherapeutic measures have not | become established as a part of the therapy of Basedow's disease, and it questionable if this will ever be the case. According to their nature, thj substances exert only a temporary action, and are able to influence the sevei cases, which, as has been recognized, are insusceptible to recovery— only du ing the time that they are being administered, and here, even, on account < the high price of these anti-thyroid remedies (a course of antithyreoidin ma cost five hundred francs), they can rarely be chosen. Their indication found above everything in acute intermediate cases where there is danger delay, for example, from attacks of suffocation. A protracted use of tl antithyroid remedies will only take precedence over the other methods alreac mentioned in. those fortunately rare cases of very severe Basedow's disease. A formidable rival to them, besides the of late recommended X-ray trea DYSGLAXDULAR SYMPTOM-COMPLEXES 357 ment of goiter, has been developed in surgical treatment. With a description of this last we will close this lecture. Practically abandoned is resection of the sympathetic in the neck which Jaboulai/ attempted to establish. This operation can, indeed, favorably in- fluence goiter, exophthalmus and tachycardia, but only temporarily, and has also acted unfavorably. The same author has recommended "exothyropexia," in which the thyroid is drawn out through an incision, covered with a dressing, and left to the spontaneous atrophy which occurs in favorable cases ; this operation also has been abandoned (on account of the dangerous results which have repeatedly followed it). Tli. Kocher favors partial strumectomy in com- bination with ligature of the arteries, and with him the majority of surgeons agree. One must not conceive, however, that the results which this operation often produces in an astonishingly short time must be permanent, and only too often one sees rapid return of the troubles in their former, or even in greater intensity, which has often led to a new partial resection of the thyroid. The mortality of thyroidectomy in Basedow patients is about 5 per cent., mainly on account of the vascularity of the goiter and the labile condition of heart and vasomotor nerves. It will be well to reserve operative inter- ference for severe cases which do not react to other methods, but here, how- ever, not to put off this last resort until the disease has reached a stage at which life is directly threatened. Although there are surgeons who on prin- ciple advise against any strumectomv in Basedow's disease (for example, the well-known student of goiter, Heinrich Bircher), on the other hand, even in some cases, which from their nature could be very well cured bloodlessly, in practice, on economic and social grounds we cannot oppose operation, since these patients on the one hand are doomed to invalidism through the severity of their trouble, on the other are not in a position to afford a long rest cure, change of climate, etc. LECTURE XXIV Dysglandular Symptom-Complexes II. Myxoedema The pathological condition to whose description we must now proceed was first studied clinically by the Englishman Gull, in the year 1873, but received its present name 5 years later from Ord, since which time the name proposed by Charcot, "cachexie pachydermique," has become almost entirely obsolete. The most important step in the recognition of this disease was furnished by the discovery by the Geneva surgeons, J. and A. Reverdin, that after total extirpations of the thyroid gland, a condition entirely analogous to spon- taneous myxoedema appeared ("cachexia strumi-priva," after Kocher). On this account the conception that spontaneous myxoedema also is a hypo- or athyreosis, a more or less complete insufficiency of the internal secretion of the thyroid gland imposes itself. Eicald has found pathologico-anatomical support for this view: He showed that the thyroid in almost all cases of myxoedema which had come to autopsy was atrophic, cirrhotic, showed a de- struction of the parenchyma of the gland, with fibrous proliferation of the interstitial tissue, and also that in the macroscopically enlarged thyroid glands of myxoedema patients, the increase in volume is caused by connective tissue increase at the cost of the secreting epithelium. It is noteworthy that some- times enlargement of the hypophysis has been found in myxoedematous con- ditions (perhaps to be considered as an attempt of the organism to effect compensation). The chief symptoms of myxoedema are a peculiar alteration of the integu- ment — bv infiltration of the meshes of its connective tissue with a mucoid semi-fluid mass — and intellectual disintegration. Along with this there is also, in congenital myxoedema ( thyreoaplasia) and infantile myxoedema, re- tardation of the growth of the skeleton; since, however, we have already become acquainted with both these forms as sporadic cretinism, we will con- sider to-day only the myxoedema of adults, and first in its spontaneously appearing form. SPONTANEOUS MYXOEDEMA OF ADULTS As exciting causes of this form, like in its pathogenetic antithesis, Base- dow's disease, psychic traumata (fright, excitement, etc.) appear to play a certain role. Also infectious diseases and loss of blood (particularly in conse- quence of repeated difficult labors) have been accused. Certain relations to 358 DYSGLANDULAR SYMPTOM-COMPLEXES 359 the function of the female genital organs are made probable in that women are -i times as frequently affected as men; virgins, however, very rarely. Also, a local predisposition without doubt exists. In England and France, for example, spontaneous myxoedema occurs more frequently than in Ger- many, Austria and Switzerland (although the two last countries show numer- ous endemics of goiter and cretinism). In England hereditary-family occur- rence also does not appear to be very infrequent. A rapid development of the symptoms is entirely unusual, rather do these generally establish themselves gradually and little noticed in the course of years, until in typical cases the following exceedingly characteristic disease picture is present: The skin takes on a pale yellowish coloration and an (Edematous appearance, though pressure of the finger does not leave behind the pitting so characteristic of renal and cardiac anasarca; this is a hard and elastic infiltration, a pachydermia; the face upon whose waxlike pallor two rose-red spots appear over the cheeks, is swollen like a full moon; the thickened, deformed eyelids make the palpebral fissure appear very narrow ; the clucks appear puffed out, the lips form thick swellings, and are slightly cyanotic in color; the forehead lies in coarse, immovable folds, the physiognomy is expressionless, often dull. The fingers are like blocks, sausage-shaped, tin wide, plump hands remind one of fencing gloves; the feet are deformed like paws. The infiltration of the skin can render the use of the extremities quite difficult. The mucous membranes undergo similar alterations to those in the skin ; on this account the volume of the tongue increases considerably and the nryxcedematous alteration of the laryngeal mucous membrane makes the voice hoarse, deep and monotonous. The sweat secretion ceases entirely, or at least becomes markedly limited; the integument often becomes cracked and falls off in scales. Its electrical resistance is considerably increased, its sensi- bility dulled. The hair of the head and body, the eyebrows and lashes, fall out or become very sparse; often, also, the finger nails and toe nails become brittle and are lost. The movements grow continually slower and weaker, although no paralysis occurs. Locomotion is carried out at a snail's pace. Nearly always (there are exceptions) mental impoverishment goes hand in hand with this physical decay. The patients lose every interest, become weak in memory and judgment. If one talks with them, they must think a long time for an answer, and make the impression that they are continually going to sleep (luring the conversation; in fact, they show abnormal sleepiness, torpor. They complain 8 ureal deal of filling cold; the temperature of the skin and the centra] temperature an' lowered, the las! sometimes below 36 < '. (96. H V.). Headaches and tinnitus aurium occur in a portion of the cases, sometimes there is also a hemorrhagic diathesis (metrorrhagias, bleeding I'r the gums, etc.) ; further, occasional accompaniments of the disease are: chronic synovitis of the knee-joint, albuminuria with hyaline easts, and certain blood alterations, which Vaquez has studied; numerical decrease of the red blood corpuscles, with increase of their size and appearance of nucleated erythro- cytes (infantile peculiarities of the morphological blood picture). The sexual sphere is almost always affected; besides iiieiiorrlia^ia . amen orrhoea is also observed, libido disappears, frigidity appears; in men then Ie 3(50 LECTURE XXIV also impotence. The heart action is usually weak, the pulse small, occasion- ally irregular; the blood pressure is often lowered. Particularly interesting, since they stand in direct contradiction to those of Basedow's disease, are the anomalies of metabolism in myxedema patients, studied by Magnus-Levy; gas interchange is reduced to about half the normal, bodily weight decreases con- siderably; the appetite is sometimes reduced, but even where this is not the case a decided repugnance to meat is usually apparent. Palpation of the thyroid gland is made mort difficult by the infiltration of the integument ; usually no glandular body is to be felt ; sometimes, however, an ab- normallv hard one which is then, as a rule, very small, rarely enlarged. The tendon reflexes are sometimes normal, sometimes weakened. If the disease is uninfluenced therapeuti- cally j it is slowly progressive, though often with remissions, which have been observed under the influence of summer temperature, sometimes also upon the occurrence of preg- nancy. The patients usually die of inter- current diseases (particularly phthisis), more rarely from the severe cachexia, which the terminal stage of mvxeedema presents. As in Basedow's disease, the "Formes ■frustes" of this disease also demand our special interest, since on the one hand they appear much less rarely than the severe form, and on the other they can be much more easily overlooked. They have been designated as "benign hypo-thyroidism." Sometimes they represent, like the "formes frustes" in Basedow's disease, the prelimi- nary stage of the typical symptom-com- plex. The skin change can be entirely ab- sent, or may be present only as a suggestion in the form of a slight, nonoedematous swilling of the face. There is a marked feeling of general weakness and relaxation in spite of very good appetite and digestive function. The urine often contains some albumin and a few hyaline casts, so that one might think of a chronic rudimentary uremia. In the psychical pic- ture, in only a part of the cases a certain apathy and loss of interest makes itself apparent; more frequently, however, so great a sopor comes over the patient upon all occasions that he has the greatest trouble to keep awake, and usually, just on account of this trouble, seeks professional aid. In part of the cases there is falling out of the hair, which is often limited to the lateral parts of DYSGLANDULAR SYMPTOM-COMPLEXES 361 the eyebrows. Sometimes there is a feeling of cold, weak pulse, sexual indiffer- ence. The thyroid gland is either not palpable, or very small and hard. OPERATIVE MYXEDEMA, CACHEXIA STRUMIPRIVA This form of myxoedema, since the serious results of total thyroidectomy have been recognized and this procedure has been carefully avoided in goiter patients (this would be in any case in malignant goiters), is scarcely ever observed any more. Still, as K^clier has shown, it can arise exceptionally, also after partial thyroidectomy, in consequence of a subsequent atrophy of the portion of the gland parenchyma left behind. On the other hand, cases of total strumectomy have been seen which remained free from myxoedema or acquired only a "forme fruste"; they possess, evidently, which is not so rarely the case, accessory thyroid glands. Post-operative myxoedema usually develops 3 to -A months after the extirpation, in a somewhat more rapid manner than the spontaneous variety. The first symptoms are feeling of weakness, shivering, then the skin becomes altered in typical manner, the movements are slow, the hair falls out, intelligence decreases; in short, the clinical picture corresponds completely with that of the spontaneous eases. Formerly the combination with tetany was frequent; the importance of avoiding not only the parenchyma of the thyroid glands, but also that of the parathyroids, in goiter operations, was not yet known. The younger the individual the more severe the cachexia strumipriva; in children it leads to the picture of sporadic cretinism with complete idiocy and dwarfism. Treatment of Myxoedema There is only one, but fortunately a quite efficient, remedy for myxoedema of every variety; the thyroid gland substance of animals, which can be re- placed by certain substances isolated from it (iodothyrin, thyreoglobulin), but without therapeutic gain and with the disadvantage of increased cost. Without doubt, the fresh thyroid substance from sheep, cattle or hogs works most actively, in doses of 1.5 to 3.0 gnu per day, which doses, however, are only reached by degrees (beginning with 0.5 grin). One lobe of the thyroid gland of a sheep corresponds to 1.0 to 1.5 gnu. The thyroid must he eaten raw ("a la tartare," spread on bread); however, the patient soon resists this method of administration, and il is often accompanied by difficulties in ob- taining the very easily decomposable "land always fresh. On this account the compressed tablets of dried thyroid substance presenl Hie most convenienl and useful manner of administration. If they are nol prepared from entirely fresh material under aseptic precautions, they can ad injuriously on account of their content id' ptomaines; hence, one should restricl himself to tablet, of known efficacy (as, for example, Hie "Tabloids of Burroughs, Wellcome \ Co.," or Knoll's "Thyradine") . Both these products contain 0.3 grm glandular substance per tablet. The daily dose is raised slowly and cautiously from 1 to 5 nr even 1 tabli I s a day. The dosage given here is for adults; thai for chitdri m page 34 I ! of sporadic cretinism. 362 LECTURE XXIV While using this remedy, continued control of the patient is necessary, since under no circumstances should the symptoms of intoxication known as "thyroidism" be allowed to occur; these manifest themselves in tachycardia, palpitation, jactitation, rapid loss of weight, reduction in strength, attacks of vertigo, vomiting, diarrhoeas and exanthems. The patient is best protected from these unpleasant, often dangerous, incidents by discontinuous adminis- tration ; 5 to 6 days organo-therapy, 4 days pause, etc. Decided improve- ments are obtained, often cures, which verge on the marvelous. The infiltra- tion of the integument gives way to normal conditions, the temperature rises, the hair grows again, movability, activity of mind return, etc. If normal conditions have been reattained, a prophylactic permanent treatment with small doses of thyroid must be ordered. On the average it is correct to give one lobe of sheep's thyroid or 3 to 5 tabloids or thyraden tablets a week. The best criterion for determining the efficient dose is, as Combe has shown, the body temperature, which should remain permanently between 361/2 and 37^ ° C. As a regime, this author recommends chiefly lacto-vegetarian diet. Meat is best permitted only with the juice squeezed out, without the bouillon. III. Adrenal Insufficiency and Addison's Disease Our knowledge of the functions of the adrenals (glandular suprarenales), whose role in the organism has long been enveloped in obscurity, has been much advanced during the last few years by many experimental investigations. Indeed, it has been known since 1855-56 that the adrenals are absolutely neces- sary for life. This was proven by the pathologist Addison in agreement with the physiologist Broicn-Si'quard. To-day, however, we know many new and interesting details as to the functions of these peculiar glands. Among other things that their seci-etion acts as an antitoxic both against exogenous poisons (as for example, certain alkaloids), and also against the toxines produced by muscular exertion, and that they produce lecithin and pigments in their cortical layer, in their medullary substance, on the other hand, adrenaline and angiotonic substance, which slows and strengthens the heart beat, causes the circular muscles of the blood vessels to contract, and decidedly raises the blood pressure. Through its special ability to produce adrenaline, however, the medullary substance of the adrenal shows that it belongs to the "chromaf- fin system" (called after its histo-chemical relations). To this last we also assign besides this a number of small structures, the paraganglia, which lie in the retroperitoneal tissue near the abdominal aorta, further, diffused cells within the sympathetic nerves and ganglia, and. finally, the so-called "carotid gland" in the neck. The medullary substance of the adrenal and the rest of the chromaffine system show close anatomical and physiological relations to the sympathetic nervous system and have, indeed, been denominated directly as the "accessory apparatus of the sympathetic." On the other hand, there appears to be reciprocal action between the adrenals (cortex and medullary substance) and the chromaffine system on the one hand, and the thymus, the lymphatic apparatus and the sexual glands on the other; for this speak among other things, the hypoplasia of the paraganglia and the suprarenal medullary DYSGLAXDULAR SYMrTOM-COMPLEXES 363 substance which is found in status thymicolymphaticus, and the hypertrophy of the whole lymphatic apparatus which is found in adrenal tuberculosis; further, the occurrence of marked hyperplasia of the adrenal cortex ("struma suprarenalis") in pseudo-hermaphroditism, in inversion of the sexual charac- ters, in precocious puberty, etc. For the neurologist, however, very special interest is presented by the exceedingly frequent coincidence of defective de- velopment of the adrenals with anencephaly, microcephaly, encephalocele, hemi- oephaly and other congenital brain defects, a correlation whose nature is still disputed but which is none the less striking; possibly the function of the suprarenal gland as the locality of production for the lecithin, necessary for the upbuilding of the brain, plays a role here. All these things have mainly theoretic interest. Clinical importance is to be attached above everything to the symptom-complexes arising from insuffi- ciency of the adrenal function, the most striking of which is that known to us since 1885 as "Addison's disease." About 90 per cent, of the cases of this disease show on autopsy destructive lesions of the adrenals (usually this is tuberculosis, more rarely malignant tumors, syphilis, interstitial inflammatory atrophies). In such cases, however, in which, after Addison's disease, the suprarenal glands have been found intact, their function has been plainly sus- pended by disease of the chromaffine elements of the paraganglia and of the sympathetic plexus of the abdominal cavity so closely related to them physi- ologically. Von Neusser and Bittorf, indeed, take the not yet generally ac- cepted position that an affection of the splanchnic nerves, which, as Biedl has shown, contain the secretory nerves for the adrenals, can cause Addison's disease. On the other hand, the occasional cases of tuberculous destruction of the suprarenal glands without "Addison's" are brought nearer an explana- tion by an observation of Wiesel, who in a case of this character found vicari- ous hypertrophy of the paraganglia and other portions of the chromaffine Bystem. Addison's disease begins gradually; it may be in a manifestly tuberculous, or again in a previously healthy individual; usually abdominal pains or rapid loss of strength arc the first symptoms; sometimes, however, the peculiar coloration of the skin, which Addison denominated "bronzed skin,** and which also bears the name of "melanodermia," occurs at the start. At first appear brown-black spots on such regions as are normally rich in pigment, or are Usually expo d to the sun; for example, the groins, about. the genitals, the axilla, about the nipples, on the face, the neck, the backs ol the hand-. These spots become colli iuua II y darker, and are finally confluent, so that the patients assume a mulatto-like appearance; as, however, in mu- latto., in Addison's disease, the matrix of the nails, as well as the palms and soles, usually remain free from pigment. On the face sometimes, upon a dark ground, still darker points are found, also the hair of the head and the beard can occasionally become darker. Very frequently the melanodermia extends also to the mucous membranes, that is, to the inner surface of the cheeks and the palate; here, however, the single dark spots do not usually run together. The second cardinal symptom of Addison's disease is adynamia, an ex haustion ocean-ring Hi'"" the slightest use of the muscles. The patients on this 864 LECTURE XXIV account become continually less able to get about ; they assume a "drooping attitude," finally become bedridden. With this, examination of the function of the muscles shows that there never is true paresis or paralysis. There is always, too, a decided relaxation and loss of energy in the mental sphere. All intellectual exertion is avoided or fails, the patients become in- creasingly apathetic; the duration and depth of sleep are increased. Only rarely psychic irritative symptoms appear; for example, deliria, hallucina- tions, delusions. As an anatomical basis of these anomalies, a diffuse enceph-/^ * a lit is in the frontal region has been accused by Klippel (cncephalopathia\^ Addisonia). Sensory disturbances are present in only part of the cases. Usually these :ui' pains which are located in the epigastrium, in the loins, in the hypochondria, in the joints, in the head, and sometimes show an irritative, radiating, neuralgi- form character. More rarely, there is general hyperesthesia of the integument, never anesthesia or hypesthesia. The reflexes usually show no anomalies. Without exception there are gastro-intestinal disturbances, great loss of appe- tite (rarely and only episodically, excessive hunger), frequent vomiting, con- stipation, in the later stages diarrhoea. The pulse is small and weak. In women the menses usually stop. The blood pressure falls off decidedly ; in the ad- vanced stages the body temperature also (in this, subjective feeling of cold). There occur attacks of vertigo, tinnitus aurium, temporary amblyopia, syn- cope. The patients become continually more and more anemic and thin, finally cachectic. In the preponderant majority of cases, Addison's disease ends fatally, though its course is usually rather protracted and may extend over from 2 to 4, sometimes even over 10 or more years. Epileptiform convulsions with subsequent coma may precede death. Acute cases in which exitus takes place after a few weeks or months, are quite rare; still rarer, cases terminating in recovery, which is chiefly to be expected in adrenal syphilis. On the other hand, extended remissions occasionally occur in the course of the disease. The most striking symptom of Addison's disease, the bronzed skin, greatly facilitates the diagnosis of this variety of adrenal insufficiency (one should in- deed, never forget to exclude other diseases in which also a melanodermia may develop: pigmentary syphilides, melanodermia from pediculosis ("morbus vaga- bundus") malarial cachexia, cirrhosis of the liver with pigmentation, bronze diabetes, pellagra, Basedow's disease, chronic silver and arsenic intoxication. There is also an adrenal insufficiency ("hypocpinephria") without pigment anomaly, which most probably depends upon the fact, that in such cases the cortical layer is more or less spared by the destructive lesion (Bittorf). The symptomatology agrees otherwise so closely with that of Addison's disease that these rather rare observations may be considered as "formes frustes" of this. As the opposite of adrenal insufficiency may be considered certain nervous symptom-complexes which, for example, occurring after hemorrhages into the adrenal (Materna and others) have been included under the name "Hyper- epinephria" and considered as the result of an oversaturation of the organism with the hormones of the suprarenal glands. These observations are still too controverted to be described here. ;:: DYSGLAXDULAR SYMPTOM-COMPLEXES 365 It still remains for us to describe the treatment of adrenal insufficiency. Great hopes have been placed upon the use of extracts or organs, hopes which have proved to be much exaggerated, however. Both the chopped up fresh substance of the suprarenal glands of the sheep (2 to 5 grm a day) and dry adrenal extract (about 1 grm a day) have been administered; further, sub- cutaneous injections of adrenalin (1 ce of adrenalin solution (1 to 1000) in 250 cc physiological salt solution) have been tried, in this, on account of the vessel narrowing effect, the absorption is very slow. In many, but not in all ciMs, these remedies have effected definite, though temporary, remissions; a comparison with the stupendous results of thyroid medication in myxoedema is, however, not in the slightest degree possible. Adrenalin has, indeed, repeatedly harmed Addison patients. Hence, one must satisfy himself with increas- ing as far as possible the nutritive value of the diet with the avoidance of all overexertion, and with the administration of tonic remedies (iron, quinine, arsenic). Considering the antitoxic function of the adrenals, their insufficiency demands the strictest avoidance of alcohol and tobacco. Constipation should be combated with medicines only with the greatest caution, since otherwise the outbreak of uncontrollable diarrhoea is risked. Syphilitic patients are to be treated specifically; in adrenal tuberculosis Strumpell recommends a cautious trial of Koch's tuberculin. IV. Acromegaly In the year 1885 Pierre Marie called attention to a disease whose chief svmptom he defined in the following manner: "A singular non-congenital hypertrophy of the extremities, superior, inferior, and cephalic," and to which he applied the appropriate name of "acromegaly." As a constant finding in the autopsies on such cases, disease of the hypophysis cerebri, the pituitary body, was found. The disease begins, as a rule, between the age of 25 and 30 years and de- velops verv slowly. The anomalies of growth accompanying it are often noticed indirectly by the patients from the fact that from year to year larger hats, gloves, thimbles, rings and shoes must be used. Also those surrounding the patient become aware of a gradual increase in volume of his nose, his chin, the distal portions of his limbs, and finally the picture becomes so characteristic that any experienced person can make the diagnosis at the first glance. In the fully developed disease, the acromegalic presents coarse features with prominent brows and cheek-bones, great, prominent lower jaw, hang- inn- lower lip, thick, knobby, nose ; the external occipital prqtuberance is ix- cessively prominent and sometimes the ears lake part in the hypertrophy. The tongue is enlarged, while a thickening of the mucous membrane <>f the palate, the tonsils and uvula as well as enlargemenl of the teeth, an- rarities. Arm and forearm, thigh and leg are normal: SO much the more striking are the disproportioned paws in which the extremities terminate. Usually this hypertrophy of the hands and feet extends chiefly in a transverse, only rarely in an axial direction ("type en large," "type en long"). The fingers and toes arc- wide, quadrangular, the nails on the other hand, which have not taken part 366 LECTURE XXIV in the increase of volume, appear small. The hypertrophy affects both the skeleton and the soft parts (the skin, subcutaneous tissues, muscles). Less constantly than the limbs, is the thorax altered; in this case it increases par- ticularly in antero-posterior diameter, so that a kyphosis in the upper dorsal region along with knob-like bulging of the sternum forward occurs; also the collar-bones become very prominent. The neck appears shortened, the head drawn down between the shoulders. The larynx is often abnormally enlarged, the voice is rough and deep, which is particularly striking in women. The skin is discolored, dry, often covered with warts, feels spongy on the hands and feet. Falling out of hair is rare. Of other anomalies there have been found occasionally, hypertrophies of the viscera, heart, liver, spleen, etc.; almost regularly, on the other hand, disturbances in the sexual organs; in women there is early amenorrhea and sterility with atrophy of the breasts; in men impo- tence, sometimes also, atrophy of the genitals. In about one-third of the cases there is glycosuria with polyuria, polydipsia, polyphagia, sometimes with the criteria of diabetes mellitus. Abnormal fatigability and great feeling of weak- ness, are almost always present ; sometimes there are neuralgic pains in differ- ent groups of muscles which has been designated the "hyper-algesic form" of acromegaly. Psychically the patient is apathetic and permanently out of sorts. The tendon reflexes are, as a rule, normal, sometimes reduced. Acromegaly always has as its basis, disease of the hypophysis and indeed, as w 7 e know to-day (thanks to the most recent very thorough investigations, and contrary to the original opinion of Marie), in an alteration leading to overfunction of its glandular portion, a hyperpituitarism.* These are chiefly simple hyperplastic tumors, the so-called strumas of the hypophysis, further, adenomata with all transition forms to adenocarcinoma and carcinoma. That tumors of the connective tissue series have also occasionally been found (sar- coma) was for a long time brought forward as an argument against the con- sideration of acromegaly as hyperpituitarism. Still, Hanau and Benda have furnished the pathologico-anatomical evidence that these neoplasms which ap- pear sarcomatous are nevertheless to be classed among the malignantly de- generated glandular tissue tumors and indeed, in 4116 locations appearing like sarcoma, are infiltrated with glandular epithelium. Only rarely in acromegaly is a hypophysis tumor not present in such cases, in part, hypophyseal adeno- mata have been found in other locations (for example, in the cavities of the sphenoid bone), in part there has been an increase of the chromaffine cells of the pituitary body, which are to be considered as its functional element, without macroscopical enlargement of this latter. In general, however, by means of the X-ray the hypophyseal tumor can be recognized intra vitam; the sella turcica shows abnormal dimensions and is usually enlarged in a sagittal direction. Large neoplasms can, besides this, manifest themselves clinically by pressure and neighborhood symptoms ; severe headache, cerebral vomiting, vertigo, slowing of the pulse, only very rarely papilledema; on the other hand (from chiasma lesions) bitemporal hemianopsia, or even bilateral amaurosis. Further, by paralyses of the eye muscle nerves * From "glandula pituitaria," the old, to-day obsolete, designation of the hypophysis; DYSGLAXDULAR SYMPTOM-COMPLEXES 3(57 which pass by the hypophysis on their way to the orbit, as well as of the first branch of the trigeminus. The more or less great prominence of these special tumor symptoms is naturally of prime importance in relation to the course and prognosis of the individual case. Sternberg has differentiated three varieties of course, perhaps somewhat too schematically; the usual form, with a duration of from 10 to 30, the malignant, with one of from 3 to i years, and finally, the benign form which may extend over even 5 decades. In general the disease proceeds but very slowly and often remains a long time, sometimes even definitely, stationary. It does not seem susceptible of recovery. The proximal causes of death are intercurrent diseases, brain pressure, diabetes, cachexia. Therapeutically we are rather helpless. Extirpation of the hypophysis by the nasal or temporal routes (r. Eiselberg, Cashing, and others) is so exces- sively dangerous and offers so little hope of radical removal that it must be reserved. for cases with marked symptoms of brain pressure, unbearable head- ache, threatened blindness. As to the X-ray treatment of acromegaly (acting upon the hypophysis from the naso-pharynx) no decisive opinion can vet he formulated. On the other hand, repeated courses of arsenic, raising it to large daily doses, appears to favorably influence the course in most cases (Brissatid). The neuralgiform pains and the diabetes demand symptomatic and dietetic treatment. In differential diagnostic relations, as opposed to acromegaly, there come before everything else into consideration, the different varieties of giantism produced by a delay of the ossification in the region of the epiphyseal svn- fcfaondroses, in consequence of which growth comes to a standstill not at all, or only very late, and Hie skeleton assumes excessive dimensions. There are distinguished partial giantism, which usually affects only one or two extremi- ties (or indeed, only parts of them; for example, in one case of Wieland's, Only the anterior half of one foot), and general giantism. The last stands patho- genetically plainly wry mar to acromegaly, in a great number of cases; in mis, indeed, enlargement of the hypophysis cerebri has been confirmed both Roentgenological! y and on autopsy. If hyperpituitarism occurs in children, it also leads, as Brissaud has shown, to giantism, if it arises, however, after ossification of the epiphyseal lines of the skeleton, it makes itself evident by acromegalic changes. In hypophyseal giantism, the genitals usually remain rudimentary, also the secondary sexual characteristics (heard, puhes. etc.). do not develop. With acromegaly, further. Marie's "pulmonary osteoarthro- pathy" (Osteoarthropathie hypertrophiante pneumique") should not he con- Osed. In this last there is an enormous drumstick-like thickening of Hie end phalanges with marked curvation of the nails and ankylosis of the finger and toe joints changes which in patients with chronic lironehiectasies (more rarely with other diseases of the bronchi and lungs) occasionally develop. OTHER DYSGLANDULAR SYNDROMES If we now should go over all the dysglandular symptom-complexes no! ye\ described, we would exceed the limits of a neurological text hook in ;l manner 368 LECTURE XXIV which would be as little justified by the slight occurrence of nervous disease pictures in agenitalism and hypogenitalism (castrated and eunuchoid persons), in infantilism and its different varieties, and in the "pluriglandular insuffi- ciency" resulting from the secretory disturbances of numerous glandular ap- paratus, as by the slight practical importance of these conditions. Hence we will content ourselves with a short consideration of two clinical pictures which indeed are very rare, but as the newest 'acquisitions to the subject which has occupied us for the last two lectures, attract actual interest. Adiposo Genital Degeneration (Frohlich's Disease, Hypophyseal Eunuchism) This rare disease is due to hypophyseal tumors which cause no hyper- function of the pituitary gland, but rather act destructively, also to traumatic lesions, as, for example, the entrance of a rifle bullet into the sella turcica in an observation of Modelling. It is hence a clinical expression of hypopitui- tarism, of hypophyseal insufficiency which is confirmed by the improvement of thr symptoms upon the administration of hypophyseal substance. Sometimes disease processes which are located, not in the hypophysis itself, but in other parts of the base of the skull, by "neighborhood action" can lead to the cardinal symptoms of adiposal genital degeneration : excessive develop- ment of fat, along with arrest of development of the genitals and of the second- ary sexual characteristics (respectively disturbance of function and atrophy of the sexual organs in case the disease occurs after puberty). Sometimes this hypophyseal eunuchism is combined with dwarfism. That the genital disturb- ance is common to hyper- and hypopituitarism, may appear paradoxical ; still, Basedow's disease and myxcedema have traits in common, for example, dis- turbances of digestion, irregularity of the menses, falling out of the hair. Dyspinealism Only most recently has it been made known through Marburg in Vienna that the epiphysis, the pineal gland, is a blood gland, whose hormone during childhood has important influence upon the mental and physical development of the individual. On this account, in children, certain tumors in the region of the corpora quadrigemina, along with the correlates of disturbances directed into neighboring neurones (paralyses of the pupil and the external eye mus- cles, ataxia, hardness of hearing, visual disturbances) produce the following symptom-complex: Hyperplasia of the genitals, abnormal growth of the body in length, unusual growth of hair, sexual and mental precocity. Marburg as- sumes in these cases an insufficiency of the pineal secretion ; the last acts, in that it normally delays the sexual development, antagonistic to the hypophy- seal secretion. LECTURE XXV Diseases of the Sympathetic, Angio- and Tropho- Neuroses Gentlemen: That portion of our nervous system of whose disturbances we will speak to-day, lias other attributes than the brain, spinal cord, motor, sensible and sensory nerves. Separated from our consciousness and our will, to a certain extent independent of the cerebrospinal apparatus, it appears not by far to reach the importance of the latter. Indeed, it plays — in the lan- guage of A. v. Kolliker — while the brain like a mighty ruler is enthroned high up in the state chambers of the skull, only the role of a servant, who exercises in the lower rooms of the body his appointed functions. And still, this is not without importance, and the brain itself is in the last instance dependent on it. Indeed, for the whole life of the individual as well as for that of the race, a system which regulates the heart's action, the whole circulation, the secretion and the processes of nutrition as well as reproduction, has a fundamental im- portance. This nervous system has, as the sympathetic or autonomic, been contrasted with the cerebrospinal, as the visceral with the somatic and as the vegetative with the animal nervous systems. Of late, however, it has become customary no longer to use promiscuously the words "sympathetic" and '•autonomous," but to apply the term "sympathetic" to the system of the gangliated cords, and to reserve the word "autonomous"' for the visceral nervous apparatus in the head and the pelvic organs (cranial and sacral-autonomous systems). A separation lulu these two subvarities is justified among other things by differences in their susceptibility to toxicological influences which have become known to us, particularly through the brilliant work of the English physiologist Langley, to discuss which at length, however, would lead us too far afield. We cannot, however, neglect to sketch briefly the principles which control tin- anatomical structure and the physiological differentiation of the visceral nervous system. Its peculiar eriterion as compared to the somatic nervous system is the fact that numerous nerve cells are interpolated in its course far into the periphery. This interpolation occurs in two ways in that, on the one hand sympathetic cells are grouped in compact ganglia which, connected with '■in another by sets of fibers, form t he so-called "gangliated cord"; on t he other hand, however, both in the neighborhood of the viscera and also in the substance of these themselves, still further collections of cells are to he found. As you know, the gangliated cords extend along in front of the vertebral column on cither side from the neck to the coccyx and are in communication through 369 370 LECTURE XXV the "rami communicantes albi" (white communicating brandies) with the spinal roots and the ganglion cells of the spinal cord ; on the other hand, they give off the "rami communicantes grisei" (gray communicating branches) which enter the spinal nerves and intimately mixing with their fibers pass farther, finally to reach the blood vessels, and to undertake their innervation. The sympathetic ganglia not belonging to the gangliated cord (the latter is called by Gaskell "the lateral," the former, the "collateral" ganglion system) are connected with it through the visceral plexuses. These last are distinguished Fig. 103. Plan of the Sympathetic Nervous System. Rm = Spinal cord; Hw=Posterior root; Vw= Anterior root ; Spg = Spinal ganglion; Spn = Spinal nerve; Gst = Gangliated cord (Patera! ganglion system); pGg 1 = Peripheral ganglion in the neighborhood; pGg 2 = Peripheral ganglion in the wall of the innervated organ (Collateral ganglion system); PI = Sympathetic plexus; = Innervated organ (hollow organ). from the plexuses of the somatic nerves, on the one hand by their net-like structure, on the other by their gray color (without medullary sheath). Col- lateral ganglia arc, for example, the coeliac, solar, mesenteric ganglia in the abdominal cavity, Ludwig's, Bidder's, Remak's ganglion in the heart wall. It is important that a different and to a certain extent antagonistic, functional significance is attributed to the lateral and to the collateral sympathetic gan- glion system. The neurones arising from the cells of the lateral system of ganglia, that is, from the gangliated cord, subserve vaso-constriction, con- traction of the circular muscles of the hollow organs and acceleration of the heart. On the other hand, those from the collateral collections of cells, effect SYMPATHETIC, AXGIO- AND TROPHONEUROSES 371 vasodilatation, contraction of the longitudinal muscles and heart inhibition. Specially in regard to this last it must be emphasized that the cardiac plexus receives an important addition from the vagus nerve (which, however, also anastomoses with the sympathetic plexuses of the lungs and stomach, as well as with the cceliac plexus) and we already learned in Lecture II that irritation of this nerve slows the movements of the heart. Is there in this an encroachment of the cerebrospinal system upon the function of the visceral? Are there in this way vegetative functions exercised directly by the brain without the inter- mediary' of the sympathetic? Not at all. The vagus has in its role as a heart nerve, only to be considered in principle a "ramus communicans albus." The inhibitory apparatus proper of the heart are intracardiac ganglia — belonging to the collateral system — which give off their "post-ganglionic" inhibiting fibers to the heart muscle. A. Diseases of the Sympathetic The pathology of the sympathetic must, up to the present, be designated as that department of nervous diseases in which there is the least actual knowl- edge, and it has been attempted to supply its place by more or less well-founded hypotheses. So of late, some authors of the Vienna School (Eppinger, Hess and others) sketch a symptom-complex which may owe its origin to a hyper- function of the vagus or of the autonomous system, under the name of "vagotony." Such patients manifest narrowness of the pupils (irritation of the ciliary ganglion), salivation and lacrimation (irritation of the chorda tympani and of the lacrimal nerve), byperidrosis (irritation of the sweat nerves), bradycardia, irregular respiratory pulse, respiratory arhythmia (irri- tation of the heart and lung vagus), bronchial asthma (irritation of the smooth muscle fibers of the bronchial tubes), hyperacidity and increased .stomach peri- stalsis (irritation of the stomach vagus), and still other symptoms. The above-mentioned authors support their pathogenetic conception of these conditions upon a certain pharmaco-dynamic reaction of the patient in question: all the symptoms of "vagotony," according to them, disappear after large doses of atropin, on the contrary, undergo considerable exaggeration after the administration of pilocarpin, while the so-called adrenalin glycosuria is ab- sent after adrenaline injections. Further investigations must show in how far such a localization of a clinical syndrome can lay claim to proof by means of experimental pharmacology. Best known and most simply explainable physio-pathologically, is that -Miiploiu which is produced by a lesion of the sympathetic in the neck and which we call "Ilonur'x symptom-complex." We have already pointed out ( s(c page 1 Mi ) that through the third ganglion of tin- cervical gangliated con I, the fibers from the ciliospinal center of the lowest cervical segments of the cord run to the eve: they innervate 1, the superior tarsal muscle : "J, the orbital muscle; :i. the dilator pupilhe; loss of their function hence causes enophthal urns (sinking in of the eyeball ), narrowing of the palpebral fissure (sympathetic ptosis) and paralytic myosis (see Fig. 104). Since now, however, the cervi- cal sympathetic also contains vasomotor and sweat secretory fibers lor the 372 LECTURE XXV corresponding half of the face, there results further from their elimination a vasomotor paralysis in this region ; this manifests itself in fresh cases by heat and reddening, later, however, usually by cyanosis and coldness (see Lecture I, page 9), and anidrosis of the same region. Still, in Horner's symptom-com- plex there is quite frequently hvperidrosis of one side of the face, hence irrita- tive and defect symptoms seem combined. As causes of Horner's syndrome the following factors acting upon the cervical sympathetic come into considera- tion: Goiters, wounds (also operative) glandular tumors, for example malig- nant lymphoma, '"cervical ribs," etc. Not rarely, however, the most exact examination does not permit the discovery of such causal factors and we have Fig. 104. Horner's Symptom-Complex. (Enophthalmus, narrowing: of the palpebral fissure myosis, in lesion of the cervical sympathetic on the left side.) then before us a pathological manifestation, harmless in itself, which we, lack- ing a bitter explanation, must designate "neurotic." A unilateral symptom of irritation of the cervical sympathetic occurs relatively frequently in tuber- culosis of the lung apex, in that upon the side of the pulmonary lesion the pupil, eventually, also, the palpebral fissure, is widened. As "neuroses" ami, indeed, as "vasomotor-trophic neuroses," are described a number of interesting disturbances etiologically unfortunately quite obscure but in any case acting upon the sympathetic distribution, of which we will now consider a few. These are acroparesthesia, angiospastic dysbasia, angiospastic SYMPATHETIC, AXGIO- AND TROPHONEUROSES 373 gangrene, scleroderma, neurotic dropsy, erythromelagia, facial hemiatrophy and hemihypertrophy. B. Acroparesthesia Acroparesthesia (the name originated with Schultze), readily the most fre- quent of the vasomotor neuroses, affects in the great majority of cases women, and appears most frequently between the ages of 40 and 55 years. The climac- teric appears to create a special predisposition, as does also general nervosity (neurasthenia or hysteria) ; the frequency of the disease in individuals whose extremities are particularly exposed to wet anil cold, for example, washwomen, points to the influence of exogenic factors. The disease manifests itself in very unpleasant paresthesias, occurring in attacks, sometimes, indeed, increasing to actual pains, which are located mainly in the hands, less frequently in the feet. The causes of these unpleasant sen- sations (formication, prickling, tickling, stabbing, "going to sleep," etc.) are spasmodic conditions of the peripheral arteries, which are shown by the livid coloration resulting from pallor and cyanosis, which can be perceived in severe cases during the acroparesthetic attack, in the affected parts (while slight at- tacks present no visible alteration). The attacks occur by preference at night or in the early morning (when the blood pressure is lowest), and, as a rule, bilaterally. After the attack passes away, sometimes reactive reddening of the fingers or toes occurs. In the great majority of cases there arc, during the attack, occasionally also intcrparox vsmall v, slight disturbances of sensibility in the affected extremities; tiny present their maximum in the pulps of the fingers (or toes) and rapidly diminish proximallv. Dejerine, Trombert, and others, have occasionally observed along with this typical condition ('•ischemic disturbance of sensation" of Schlemiger), radicularly arranged hypesthesias in acromegaly; in spite of many reexaminations I have not so far been able to confirm these observations. The diagnosis of the disease is usually exceedingly easy, only one must remember that a number of other affections commence with paresthesias of the fingers or toes, or can do so. So, ergotism, "intermit tint limping," "dys- basia angiosclerotica intermittens" (see Lecture XV, page 225), respectively its analogue located in tin' upper extremities ("dyspraxia angiosclerotica in- termittens"), tabes dorsalis, tetany, acromegaly, and finally, as we will soon, sec, angiospastic gangrene {Raynaud's disease which is in so far related to acroparesthesia that it presents a malignant increased degree of intensity of the "vaso-constrictor neurosis of the extremities"). In any case, also tie re- lations between acroparesthesia and vasomotor angina pectoris (Nothnagel, Curshmann) in which also by preference in the night and morning hours, the same subjective and objective anomalies appear on the fingers, but also with palpitations, precordial anxiety, pain in the region of the heart with radiation into the left arm, also the sympt oms of Inir angina pectoris, are close. In contradistinction to the latter, however, there are absent all symptoms of sclerotic or luetic diseases of the aorta nr the coronary vessels; rather does this evidently benign disease affect generally, physically quite healthy per- 374 LECTURE XXV sons, usually hysterical young women. Psychical traumata are nearly always discovered in the etiology; Curshmann accuses among other things coitus in- terruptus. The prognosis of acroparesthesia cannot be laid down generally. There are cases which resist all treatment, though they usually present an alternation of improvements and relapses (such remissions usually occur during the warm part of the year). On the other hand I have seen not a small number of cases pass on to recovery and many permanently improve to an entirely tolerable stage. As to life, the disease is entirely harmless ; as to function, not all too disturbing, since it scarcely ever causes actual disability. From a therapeutic point of view electrical applications are to be recom- mended in the first place: The faradic brush, faradic hand baths, "four cell baths." Further, a course of quinine from time to time procures for many such patients a permanent or lengthy improvement. At bedtime a capsule of 0.5 quinine sulphate or "chinin-phytin" (combination with phosphorus) is given every night for from 2 to 4 weeks. Between these quinine cures proper, quinine in small doses can be given further, very well in the form of the fol- lowing pills : R^ Quinin sulph 3.0 (grs. 45) Ext. nucis vom 1.5 (grs. 22) Ext. hyoscyami 2.5 (grs. 38) Ext. valerian 7.0 (grs. 105) M. Fiat Pil. No. C. S. 2 pills t. i. d. after meals. For washing, only warm water must be used. At night the patient should wear fur-lined slippers or socks. C. Angiospastic Dysbasia The "intermittent limping," occurring from organic disease of the periph- eral, also of the spinal vessels, we have already become acquainted with in Lecture XV. There is now, however, a purely vasomotor form of this syn- drome to which Oppenheim first called attention and which of late has been accurately studied by Curschmann. The first author observed a case of in- termittent claudication for 15 years, without the severe results of vascular obstruction having developed. On this account there presented itself to him the possibility that permanent spastic conditions of the muscles of the artery wall, though varying in their intensity, can occur, and can produce the intermittent limping. He also expressed the suspicion that a congenital narrowness of the vascular system prepared the way for the occurrence of this disturbance. Curschvuinn saw in people from 18 to 22 years old intermittent limping with all subjective symptoms, also with the absence of pulse in some of the arteries of the foot, and could certainly exclude arteriosclerosis and arthritis. By plethysmographic investigations he was led to assume the possibility of a per- manent spasmodic condition of the arteries. I myself have classed as vaso- motor neurotic dyspraxia of the upper extremity, forms of writer's cramp which were accompanied by coldness and lividity of the hand, but the cases men- SYMPATHETIC, AXGIO- AND TROPHONEUROSES 375 tioned are on the whole quite infrequent and I would impress it upon you that the diagnosis of a functional, that is, a benign form of intermittent limping or similar functional disturbances of the upper extremities, should in any case only be made after long and thorough observation, and in case of doubt al- ways to suspect an organic vascular lesion and to make this supposition the basis of your treatment. This last, however, will be in general the same in angiospastic and in angiosclerotic intermittent dysbasia. The use of alcohol is to be limited, tobacco forbidden. Not less important is the absolute prohibition of those perverted therapeutic measures to which such patients only too often turn on their own account or from unwise counsel ; cold wate.r procedures, hot foot- baths, excessive gymnastics and massage, forced marches, etc. The same thing applies to the use of rubber stockings and bandages. To be avoided are all substances having decided vasomotor action, strong tea, coffee, spices. Further, care should be taken that the feet and legs are kept evenly warm (proper choice of residence and work-room, foot and leg coverings, bedclothes, occasionally mild rubbing with tepid water). Hot foot-baths are to be avoided; warm foot-baths, on the other hand, to be recommended, as also ap- plications of Fango, peat, Priessnitz compresses, etc. Erb has quite warmly recommended the galvanic foot-bath ; either in the fashion that each foot is placed in a separate tub containing salt water at from 34 to 36° C. on a pole plate and a stabile current of from 12 to 20 milleamperes is conducted through them, first in one direction and then in the other for from 3 to 6 min- utes, or so that both feet are placed in a tub containing the cathode while the anode rests over the sciatic in the bend of the knee, or over the plexus on the sacrum. Of drugs vaso-dilator substances come into consideration in the first place (courses of quinine as in acroparesthesia, also nitrites, nitro-glycerin and diuretin have been recommended) ; in the second place heart tonics, particularly those from which a better blood supply and favoring of the collateral circula- tion are to be expected, without action upon the vasomotors; also particularly the strophanthus preparations in contradistinction to digitalis. Simple or carbonated salt hat lis are to be recommended on account of their vaso-dilator effect. Of great importance, finally, is the regulation of the movements and of tin- use of the legs. At first, when possible, entire' rest ami long reclining are to he prescribed. In any event, however, all violent exertion is to he for- bidden and walking is to be permitted only to such an extent as does not pro- duce any difficulty. Only when decided improvement has been obtained, the patient, '"watch in hand," is allowed to undertake more prolonged exercise, D. Angiospastic Symmetrical Gangrene, Raynaud's Disease ("Asphyxie Locale Symmetrique") This very rare disease- I have only seen three cases is to he considered as the highest stage of intensity of the vaso-constrictor neurosis of the ex- tremities. The occasional, hut by no means regularly observed pathologico- anatomieal findings ( endartcrit ie ami endophlebitic changes) are readily I" be 376 LECTURE XXV considered as secondary. Raynaud's disease, in contradiction to acropares- thesia, shows no special predilection for either sex, and usually occurs in the com- paratively young, at between 25 and 35 years of age. Cassircr, indeed, saw a nursling affected with symmetrical gangrene. Etiologically, neuropathic pre- disposition plays in any event the chief role. Many cases affect hysterical, psychasthenic, psychotic patients. In one of my cases there was a history of recent, but thoroughly treated syphilis ( Wassermann negative), and lues is noted in a number of cases of Raynaud's disease in the literature. Nekam and Curshmann observed its family occurrence. As exciting causes, acute infec- tious diseases, trauma, severe emotions and chilling have been specially men- tioned. The disease begins usually with an attack of "local syncope"; one or several fingers or toes, through spasm of their arterial and venous vessels, become cold, waxy-pale, and so ischemic that deep needle stabs do not draw any blood. Now, however, I must warn you against considering every local syncope of this kind as a forerunner of Raynaud's disease. Much more frequently this phenomenon ("dead fingers") is a harmless symptom in neurasthenics and other psycho- neurotics, as we will see in Lecture XXATI, also in Bright' s disease. Char- acteristic for the Raynaud variety of this symptom is, on the one hand, its appearance in symmetrical positions in the upper or lower extremities, on the other, the (often very severe) pains which introduce or accompany it. With continuation, or even increase of these pains, there is added to the stage of local syncope which is of short duration, from minutes to hours, that of "local asphyxia," which lasts considerably longer and can extend over many hours, even several days. On the fingers or toes, and indeed, almost exclusively on the end phalanges, there appears now in spots, at first a bluish, then a con- tinually darker, discoloration, reaching finally slate-gray in color; sometimes, besides this, there are extravasations of blood under the skin and bullous ele- vations of the epidermis. Now, in the favorable cases, the circulation is es- tablished again, when a decided reddening of the fingers appears, in place of the regionary cyanosis ("stage of local reddening") ; in bad cases, however, the local asphyxia of the affected parts passes over into gangrene. This can be total, so that entire phalanges become mummified and cast off, or only partial, in which necrosis of circumscribed parts of the skin occurs. Moist gangrene is quite rare. I saw it in the case with luetic previous history men- tioned above. In another still rarer atypical form of Raynaud's disease, not only the ends of the extremities, but larger or smaller portions of the leg, the buttock, and even the tip of the nose, the lobe of the ear, etc., are affected by the disease. The relatively mild, that is, the Raynaud's paroxysms not coming to the gangrenous stage, can be repeated over years or decades, then at length to lead to mutilations. Without exception the general condition of the patient suffers considerably from the exceedingly painful attack. As a regular accom- paniment of the peripheral vascular spasm there is to be mentioned marked hyperesthesia, which makes itself evident during this spasm in the parts affected; of occasional complications, are of interest, the paralysis of the sympathetic of the neck (Horner's symptom-complex), further, paroxysmal disturbances of the SYMPATHETIC, AXGIO- AND TROPHONEUROSES 377 sense organs, which may well depend upon vasomotor spasm (transitory deaf- ness, blindness, ageusia). Here belong also the observations of transitory aphasia, hemiglobinuria, etc. The course can be quite acute, in that, after one or a few attacks the disease comes to an end through casting off of the affected phalanges. In other cases, however, the ever returning paroxysms constitute a life-long scourge. Life itself (if we leave out the rare cases in which sepsis has occurred) is not threatened by angiospastic gangrene. In differential diagnosis, as I already emphasized, the harmless form of "dead finger" is to be thought of. A benign disease is also presented in the "acrocyanosis chronica anaesthetica" of Cassirer. In this affection, an asphyxia of the ends of the extremities develops itself little by little and is accompanied by paresthesias, considerable local dulling of sensibility; sometimes also by trophic disturbances (increase of volume). The distinction of this from arteriosclerotic "spontaneous gangrene" can be difficult when attacks of inter- mittent limping or intermittent dyskinesia of the upper extremities have pre- ceded this last by some time. Still, the angio-sclerotic dyskinetic paroxysms distinguish themselves from those of Raynaud's disease, in that they are de- pendent upon the use of the affected extremity and disappear upon rest, the accompanying vasomotor skin changes are never very great (a spotted, marbled condition of the integument, but no acro-cyanosis), and the pulse in the peripheral arteries permanently disappears or is greatly reduced. The separation of Raynaud's disease from "multiple neurotic gangrene of the skin" in which on different portions of the skin after a painful prodromal stage, or with only burning and prickling paresthesias, superficial necrosis takes place, is not sharp. In making this diagnosis, however, the greatest caution is neces- sary. The affection attacks very frequently hysterical patients, who as we will see, have an uncommon tendency to self-mutilation; in one of mv cases by chemical recognition of silver in the sloughs, I was able to prove that the pa tient had produced the necrosis with nitrate of silver. Mutilations of syringo- myelia and lepra are separated most sharply from Raynaud's disease by their painlessness. The treatment of Raynaud's disease is to be carried out after the analogy of that for acroparesthesia and for intermittent limping (see above, pages 874-375), only one will do well to avoid all faradic applications, which here act unfavorably. Tin- always presenl general nervosity must lie combated with tlii' greatest care, according to the principles to he mentioned in Lecture XXVIII; they must, indeed, he considered as the peculiar substratum of the vasomotor neurosis. The severe pains call for resort to the most active aliti- neuralgics. Sometimes morphine injections cannot lie avoided. E. Scleroderma This disease, which is usually observed in women of middle age and was described for the first time by Thirial, is much more frequenl than Raynaud's disease, hut still must he denominated a rare affection. It is characterized bj the chronic development of a severe trophic alteration of the skin which only 378 LECTURE XXV rarely affects in a diffuse manner the whole integument, usually is limited to definite parts of the face, neck, thorax and upper extremities. The lower half of the trunk and the legs usually escape the disease. Etiologically we know nothing certain ; the same points which we brought out in speaking of Ray- naud's disease, could be repeated here. The sclerodermatically altered parts of the skin in the fully developed dis- ease, present an exceedingly characteristic appearance: they arc thinned, smooth, of a dull, bacon-like polish, tense, cool, hardened and firmly fixed to the subcutaneous tissue. Their color is usually pale, sometimes, however, of a yellowish or brownish pigmentation, also vitiliginous and pigmented spots occur alongside of one another. Also the deeper layers of tissue (subcutaneous fat, muscles, tendons, skeleton of the hand) undergo an increasing atrophy. Move- k S*> > PI k L J > i v P Fie. 105. Sclerodaetylism, with Formation of Necroses. ments of the limbs, opening and closing of the mouth, are more and more hindered by the skin becoming "too tight.'' True contractures can indeed occur. So, diffuse scleroderma can give to the patient a mummy-like appearance. As to the circumscribed forms, they furnish a great variety of striking clinical pictures. The alterations usually occur more or less bilaterally and symmetri- cally. They are found particularly frequently on the fingers; we then speak of "sclerodaetylism"; with this there are also atrophic and degenerative pro- cesses in the nails, occasionally, indeed, such intense disturbances of nutrition in the end phalanges that actual necroses occur (see Fig. 105). (Such cases have, in my opinion incorrectly, been termed a combination of scleroderma and Raynaud's disease ; as a diagnostic criterion of the last affection, in any case, the painful, paroxysmal attacks of regionary syncope and asphyxia are indis- pensable). Exceedingly rare is the "annular sclerodaetylism" of During which, distributed in a circular manner can lead to the strangulation of the phalanges. SYMPATHETIC, AXGIO- AND TROPHONEUROSES 379 The diffuse scleroderma of the trunk and of the face seldom leads to round, but usually to streak-like foci ("sclerodermic en bandes"). Particularly typical are isolated streaks extending from the line of the hair to the eyebrows, remind- ing one of the scar of a sabre wound ("sclerodermic en coup de sabre," see Fig. 106). Also the mucous membranes can be affected (mouth, nose, pharynx). Particularly interesting, though also very rare, are the observa- tions of certain circumscribed sclerodermas which were exactly limited to the area of a peripheral nerve or to a spinal root zone, or were localized on one side of the body (Leu-ht, Heller, Bonn, Brums, Curschmann, and others). As preliminary stages of the atrophic changes, a stage of firm oedema and an in- durative stage have been described; still, it appears as if this was an artificial general- ization of clinical pictures which by no means occur in all cases. No objective dis- turbances of sensibility worth mentioning occur in scleroderma, on the contrary the intact appreciation of sensation of the greatly altered skin (anatomically it is a "cirrhosis" of the chorium with thickening of the vessel walls) often appears to us paradoxical. Pains, on the other hand, are not rare, particularly in the hands. Though they also sometimes exacerbate, still they have neither the intensity nor the paroxys- mal character of those which are peculiar to Raynaud's disease. Scleroderma can be combined with different nervous diseases. Particularly frequently does lliis appear to be the case with regard to Basedow's disease (observations of Leube, Stahelim, and others) which all the more attracts at- tention to its eventual connection with internal secretory processes, as Bruno Block and lieitmann in severe scleroderma, have shown, a striking variation of the nitrogen balance hit ween positive and negative values; pathological in- crease of albumin destruction is, however, as we saw in Lecture XXIII, a typi- cal occurrence in Basedow's disease. Rarer is the association with Addison's disease or tetany. The combination with Homer's symptom complex i, also quite interesting; the patient shpwn in Fig. 106 showed this last on the side of the scleroderma (in the picture unfortunately obscure). In the most marked case of diffuse scleroderma which I have seen (it affected a 13 year old girl) besides this, in the course of yens, a general atrophy of all the muscles of the skeleton developed; also during the progress of the disease ul ceis and fistulas, from which carbonate of lime was discharged, broke out in different locations. The patient died of marasmus. The examination by Dietschg showed that there was a combination of scleroderma with interstitial polymyositis and calcareous tendinitis. Scleroderma — "En coup de sabre," with Horner's Symptom-Complex on the same side-. 380 LECTURE XXV From a prognostic point of view it is to be remarked that a cure of the changes is only to be thought of in the earliest stages, when no atrophy of the skin has yet occurred ; later lesions, however, are to be considered as irrepar- able. Circumscribed forms in a location which does not cause any disturbance of function and without painful phenomena, usually cause no difficulty worth mentioning and can become stationary. To sclerodactylism, however, all this does not apply and it constitutes a severe disease, sooner or later leading to invalidism. Exceedingly extensive forms of scleroderma are always to be con- sidered as very serious ; respiration is impeded, the act of eating is imperfectly performed and the patients die in cachexia, if they are not taken off by inter- current disease, against which they show themselves little resistant. Many of these patients die of degeneration of the heart, muscle; diffuse scleroderma considerably impairs the circulation and so injures the heart. In differential diagnosis it may be remarked that "glossy skin," as it occurs after a peripheral nerve lesion, should not be confused with scleroderma. The other skin atrophies (the stria 1 of pregnancy, senile atrophy of the skin, simple and pigmented xeroderma) have no resemblance to the picture of scleroderma, since in them all, the hardening is absent ; a similar aspect, however, may be presented by scars (after leg ulcers, burns, lupus, etc.) ; still here the history will naturally exclude wrong diagnoses. In treatment, above everything, frequent warm baths (eventually sulphur baths, mud baths, Fango packs) are to be recommended; also the steam bath and the hot air bath ("Bier's box'') can be tried. Mild massage is nearly al- ways found beneficial. Of internal medication the salicylic preparations ap- pear to exercise a decided effect, (sodium salicylate, acetylo-salicylic acid, salol, etc.) ; also ichthyol capsules (ail 0.25, 1 to 3 capsules t. i. d.) have been recom- mended. Ichthyol, by the way, comes also into external application, as does thiosinamine plaster. Hcbra has introduced thiosinamine into the therapy also in the form of injections (every second day 0.5 cc of a 15 per cent, alcoholic solution). These injections are very painful; better tolerated is the nearly related fibrolysin, which Cursch/mann has warmly recommended upon the ground of favorable experiences. Also courses of iodide appear to me often to act favorably. For the sclerodermic pains I recommend the following mix- ture, which must be shaken up : R< Sodii iodid, Lactophenin aa 5.0 (gr. 75) Codein phosph 0.2 (gr. 3) Spirit vmi 20.0 (5 5) Infus. radic. valerian (10 per cent.) ad 150.0 (§ 5) M. S. Three tablespoon fills a day until the pains are relieved, then one or two as needed. Shake before using. SYMPATHETIC, AXGIO- AXU TROPHONEUROSES 381 F. Neurotic Dropsy Exudations of scrum occurring from angio- or tropho-ncurotic cause we meet with in two different forms, on the one hand as neurotic oedema of the skin, on the other as neurotic dropsy of the joints. 1. Circumscribed (Edema, of the Skin (Quincke). — This affection is rela- tively rare and occurs in young individuals of nervous predisposition. (Pre- dominance in the male sex is plainly evident.) Partly in connection with ex- citing emotions, trauma, exposure to cold, partly without recognizable ex- ternal cause, sometimes hereditarily, circumscribed swellings of the skin which have a doughy feeling and retain the impression of the finger, occur in attacks. Their color is pale or, on the contrary, somewhat reddened, in size they vary from that of a dollar to that of the palm of the hand. Multiplicity of these usually quite indolent, but sometimes itching urticaria-like disease foci, is fre- quent. They can occur at any point on the surface of the skin, also may ex- tend to the mucous membrane of the mouth, the pharynx and the conjunctiva. The seat of predilection is the face, after this, the backs of the hands. The appearance of the oedema is sometimes accompanied by vomiting and diar- rhoea which has been considered as an expression of neurotic-cedematous changes in the intestinal tract; in a case of my observation (which occurred in a pre- viously healthy girl after the railroad accident at Miillheim in Baden) the attacks were at the start accompanied by bloody diarrhoea. Joseph has ob- served hemoglobinuria. After a few hours the circumscribed oedema disap- pears again, but has a great tendency to recur. Periodic (also menstrual) appearance is sometimes observed. Many patients suffer during their whole Eves from this, as a rule, harmless affection, which can only be dangerous when it is localized at the entrance to the larynx. Still, permanent recoveries are not entirely rare. There are certainly close nosological relations between Quincke's (edema and chronic recurrent giant urticaria. Many patients suffer simultaneously from Quincke's oedema and urticaria; in one case of Bircher's an urticarial eruption regularly preceded the attack of (edema and indeed, so that the two affections always appeared at different locations on the skin. Also Combination with asthma and migraine attacks occurs. The pathogenesis of the disease is obscure; perhaps it is (he result of a regional venous spasm. Umrjlcr as physician to the Basle house of correction, has observed a regular guesl of international penal institutions, who (in order to avoid punishment) could produce at will circumscribed (edema of the face, but who would not dis- close his method of procedure-;, Hv/nziker suspected that it was by manual Compression of a venous trunk. On the other hand Stahelin has shown that. eastro intestinal autointoxication may play a role as exciting cause for the appearance of Quincke's oedema, as is so often the c:r~>- In urticaria. Still more obscure is the pathogenesis of the so called "chronic form of neurotic oedema." By this is understood hydropic swellings of the skin, usually located on the leg or on the forearm, which appear without any apparent cause, also an- observed as a family complaint ("trophoedeme familial" of Mcii/i ) and last for years or for life, without any organic (renal Or cardio- 382 LECTURE XXV vascular) changes being present. You are warned against confusing traumatic forms with the so-called "hard traumatic oedema." This last, when it appears upon the backs of the hands, is often the result of fraudulent manipulations of skilled simulators, as Secretan and Haegler have recognized. Therapeutically, regular saline purgation, bland and lacto-vegetarian diet, daily cold spongings, rain douches or river baths, are to be advised. Of medi- cations I would recommend long-continued administration of salicylate of quinine (0.25 twice a day) to be tried in every case. Threatened obstruction of the entrance of the larynx, danger of suffocation has on various occasions rendered tracheotomy necessary. After this the application of a permanent tracheotomy tube is recommended. 2. INTERMITTENT DROPSY OF THE JOINTS Still more rare than Quincke's oedema is intermitting dropsy of the joints (Moore). This is a periodic swelling of the joints, sometimes recurring with striking chronological regularity, which usually affects one, somewhat more rarely, both knee-joints. In one of my patients the articular dropsy occurred, for example, every 13 days. The patient was, however, during these intervals free, but in them he suffered from cyclic melancholia (also in cases of Reismger and Morris, there was a 13-day rhythm). Intermittent dropsy of the joints, apart from a troublesome feeling of tension in the joints, runs its course with- out important subjective symptoms. Still, sometimes disagreeable accompanying symptoms have been mentioned (slight fever, palpitation of the heart, vertigo, vomiting, hyperidrosis, poly- uria, diarrhoeas, migraine, etc.). After from 1 to 3 days the swelling dis- appears again. The treatment coincides with that of Quincke's (edema ; also we can repeat the little that we have said about the etiology of this last here.* A difference consists in the fact that neurotic joint dropsy attacks by preference the female sex, from which it can be concluded that physical overexertion plays no special etiological role. The prognosis is worse than in circumscribed oedema of the skin, permanent recovery still rarer. G. Erythromelalgia Erythromelalgia. which was first described by Weir Mitchell, represents the type of a vaso-dilator neurosis of the extremities. Still, I have seen the disease in a case running a favorable course and of otherwise typical char- acter, appear in the face, which must be denominated "erythroprosopalgia." The very rare disease occurs almost exclusively in grown persons and mani- fests a certain predilection for the male sex. Those affected are generally persons of neuropathic heredity and as exciting causes, exposure to cold and overexertion have been accused. The upper extremities are much more rarely attacked than the lower, in which, in typical cases, the "erythromelalgic at- tacks" first manifest themselves by severe neuralgiform pains. Soon there fol- * No surgical treatment. It cannot aid, but will injure. SYMPATHETIC, ANGIO- AND TROPHONEUROSES 383 lows an intense reddening of the toes, of the feet, sometimes also of a part of the leg, in rare eases indeed, of the whole lower extremity; this reddening, which is usually accompanied by swelling, is sharply separated from the normal parts of the skin. With the appearance of this local dilatation of the vessels, the pain usually somewhat diminishes in intensity. The affected parts of the skin feel hot and sometimes sweat profusely; they are the seat of a decided hyperesthesia. The erythromelalgic attack can last hours, days, or even weeks ; in the last case the flaming redness more and more gives way to a cyanotic coloration and the local hyperthermia subsides. In many, perhaps most cases of erythromelalgia, between the individual attacks the integument of the af- fected parts does not completely return to normal, but there remains a certain degree of vaso-dilatation along with different trophic disturbances which per- sist interparoxysmally ; this is called "'chronic erythromelalgia." These chronic cases are prognostically very unfavorable, while true paroxysmal forms can recover. Erythromelalgia, indeed, does not constitute a danger to life. The differential diagnosis from erysipelas, acute gout, inflammatory flat-foot, etc.} is often quite difficult. As erythromelia is denominated a painless, vaso-dilator neurosis, which has been observed on the extensor side of the limbs. As treat- ment during the attack, elevation of the affected extremity witli cold com- presses, is to be recommended (while a hanging position and warmth consider- ably increase the distress. Further, naturally, the use of different anti- neuralgics. Electro-therapy is of little or no use. In hopeless cases nerve resection, and, indeed, amputation, have been carried out. H. Facial Hemi-atrophy and Hemi-hypertrophy Both these peculiar trophic neuroses, especially the second mentioned, are very rare, and for this reason, particularly, however, on account of their thera- peutic intractability, are practically without importance. To complete our discussion though, we will sketch I hem in their outlines. Progressive facial hemi-atrophy, or Romberg's disease, affects chiefly fe- males in early life and is usually introduced by neuralgiform pains in the affected half of the face which sometimes are also present in the fully developed stage of the affection. Very gradually there develops a strictly unilateral atrophy of the skin and of the subcutaneous connective and fatty tissue, often also of the bones of the face and of a few muscles (the temporals, masseters, tongue muscles). One-half of the face hence sinks in, in a striking manner. As rarities, cases have been described in which the atrophy has extended also to the neck, the thorax and the arm. In the majority of cases the circumscribed Atrophy of the face affects the left side. Sensibility remains unaffected in tin' atrophic region; on the other hand, in it vitiligo, abnormal pigmentation, falling nut of the hair, decolori/a I ion of the- hair, etc., are occasionally ob- served. Therapeutically, the disease is not to be influenced; it can, however, spontaneously come to a standstill before the deformity has reached a very high degree. In these last cases cosmetic paraffin prosthesis hy Gersu/ny'a method has sometimes given good results. Pathogenetically Romberg's disease is one of tin- most obscure of the tropho-neuroses. A near relationship t'» 384 LECTURE XXV scleroderma is the more probable, since combinations of both pathological con- ditions occur. The contrary condition to facial hemi-atrophy, facial hemi-hypertrophy was first described by Friedreich in the year 1862. It is such a rare disease that the last reviewer of the subject. Wanner, up to 1908, could find only 29 cases in the literature. It consists in a considerable increase in volume of the soft parts, sometimes also of the bones, on one side of the face, including the forehead and parietal region ; the tongue, the ear, the tonsil can take part in the hypertrophy. Anomalies of pigmentation, vasomotor disturbances, uni- lateral exophthalmus, and other things can complicate the picture. Men and women are found with equal frequency among those affected. Contrary to facial hemi-atrophy, the disease most frequently affects the right side. Neural- giform symptoms are rarer than in Romberg's disease. I. Herpes Zoster Herpes zoster ("Gurtelrose," "Zona" of the French authors) is a typical vesicular eruption which in its topographical distribution appears connected Fig. 107. Herpes Zoster of the Trunk. with the segmentary areas, that is, with the regions of distribution of the pos- terior spinal roots or their cranial homologues. Fig. 107 represents a herpes zoster on the trunk, Fig. 108, one on the face. In the last case I beg you to note that the herpetic area does not correspond exactly with the distribution SYMPATHETIC, ANGIO- AND TROPHONEUROSES 38.5 of a single branch of the trigeminus, but surrounds the nose more or less con- centrically and, hence affects the region of all three divisions. It is indeed, a radicular, not a peripheral-nervous tropho-neurosis. Barensprung's investiga- tions, which have been confirmed by numerous later authors, have shown that the seat of the disease is to be sought in the spinal ganglia, respectively in the homologous structures of the sensory brain nerves ( particularly in the Gasserian ganglion). It may be an acute inflammation of these (according to Head and Fig. 108. Herpes Zoster of the Face (with Homer's Symptom-Complex). ( a in pbell, usually so), but also may In- due to tumors, trauma, etc. The physio-pathological hasis of tin- vesicular eruption i^ indeed obscure to us in spite of this topical diagnostic recognition. In the mechanism concerned, how- ever, connection with the sympathetic comes into question. Of importance in this relation is. for example, the condition that in the patient shown in Fig. 108 a mvosis and enophthahnus and the narrowing of the palpebral fissure oc- curred on the same side as the herpes eruption. Etiological!; in the so-called secondary cases, as already said, then' have been injuries to the ganglia (frac- ture of the vertebral column, etc.) or tumors (carcinoma metastases, for ex- ampli ): further, poisoning with carbonic oxide and arsenic should be men- 386 LECTURE XXV tioncd. With regard to the last, there has been a controversy as to whether the herpes occasionally observed after salvarsan injections is to be considered as a toxic manifestation or as the so-called "Neurorecidiv" of syphilis. In primary or idiopathic herpes zoster, which we will now exclusively consider, there appears to be an acute infectious disease sui generis, in which exposure to cold and similar things can only be considered as exciting causes; the actual cause, however, is indeed still unknown to us. For this speak the above-men- tioned pathological findings of Head and Campbell, further the facts that the disease appears to leave behind it an immunity, runs its course with fever, swelling of the glands, and pleocytosis of the cerebrospinal fluid, and last, but not least, its occasionally observed appearance in epidemics. In typical cases of idiopathic zoster a moderate rise of temperature (to about 39° C. (102.2° F.) with general discomfort, disturbances of digestion, etc., precedes the eruption. With this there is usually very severe burning and boring, sometimes continued, sometimes exacerbating pains in the affected radi- cular area ; these pains can appear simultaneously with the vesicular eruption^ or indeed, only after its occurrence. They usually get worse toward evening- The herpes eruption itself is usually introduced by the appearance of red spots upon a normal appearing ground. Soon, however, the epidermis over these spots raises itself to serous vesicles whose contents gradually become cloudy and purulent. If the sensibility of the skin in the neighborhood of the vesicles is tested, sometimes hypesthesia and anesthesia, more rarely hyper- esthesia, is found. Sometimes atypical forms of eruption appear: bullous, hemorrhagic, gangrenous, ulcerative herpes ; the last two may leave behind scars. Regional glandular swellings are not rare, also hyperidrosis or anidrosis occur, as do also obstinate neuralgias, which may persist after the herpes has recovered, which takes usually from 2 to 3 weeks, but sometimes considerably longer. Such persistent root neuralgias cloud the prognosis of the in general entirely mild disease, especially in decrepit old people, who form a large contin- gent of the zoster patients. Serious results may also follow an extension of the herpes zoster to the cornea, when it may lead to ulceration and panophthal- mitis. Still, this localization is, fortunately, not frequent. Therapeutically, we limit ourselves to the administration of antineuralgics (see Lecture III, page 57), and the application of a 2 to 5 per cent, anes- thesin ointment, or of a powder of talc, bismuth subnitrate and starch, equal parts. Ulcerated herpes vesicles heal best under 5 to 10 per cent, ointment of balsam of Peru. Ophthalmic herpes should be turned over to the ophthal- mologist without delay. Gentlemen: We will here break off our sketching of the most typical tropho-vasomotor syndromes. I must remark to you, however, that it is just in this class of cases that mixed and transition forms are specially fre- quent ; for instance, not only can the different vaso-constrictor conditions be combined, but also vaso-constrictor and vaso-dilator neuroses have been ob- served in one and the same patient, for instance, erythromelalgia and Raynaud's disease. It may be also related that many skin diseases by their topographic limita- tion to definite regions of innervation, symmetric distribution, etc., proclaim SYMPATHETIC, ANGIO- AND TROPHONEUROSES 387 themselves of tropho-neurotic or vaso-neurotic nature. I would recall many vascular n;evi, cases of vitiligo, partial albinism, pigmented moles, etc. Also, symmetric lipomatosis or Madelwng's disease may will belong here (see Fig. 109). As a variety of this last is to be considered the adipositas dolorosa or Dcrcum's disease, which also shows its connection with nervous diseases, particularly by the element of pain. It affects mainly women (often alcoholics) Fib. 109. ,\l,iih hunt's Disease. who. also in general very corpulent, present tumor-like lumps of fat in the sub cutaneous tissue of the arm, feg and trunk, but with regular escape of the hands and feet. These lumps of fat are sensitive to pressure, as are also the regional nerve trunks. Also, spontaneous pains may arise in these [ipomata. Anesthesia or hyperesthesia of the affected skin zones are occasionally noted. The attempt of a few authors to prove the relationship between Den urn's dis ease and mv xcedema iniisi be considered as a failure. Finally, progressive lipo dystrophy in which the upper half of the body emaciates to complete loss of the panniculis adiposus while the lower hull', on the contrary, shows rather an increased deposition of fat. i^ to be mentioned. LECTURE XXVI Epilepsy Gentlemen : The disease to which to-day's lecture is devoted, must be considered as the first nervous disease of which literary information has been transmitted to us. That it since most remote times has laid claim to the phantasy of physicians and laymen to a high degree and that it, as no other disease, has had the reputation of being of supernatural origin, we need not wonder. Indeed, the "falling sickness" which causes a man apparently in perfect health, to fall unconscious and to go into convulsions, must make upon the masses of the people a particularly alarming, indeed, uncanny impression. Even the "scientific" name epilepsy, which we have inherited from ancient times, indicates nothing but "being seized" or "possessed." You know, indeed, that the disease was considered as a punishment inflicted by the gods, hence the name customary to the Hippocratic body, "hpij voudden causeless appearance of a d< finite memory, often » ith the plainness of a hallucination, or an abrupt change of i I. for instance, an outbreak of mirth, or feelings of anxiety and imperative idea-. Often lb'' 390 LECTURE XXVI aura is a complex one made up of psychic, sensor}', sensible and motor com- ponents in manifold combination, but it usually, in one and the same patient, repeats itself in a thoroughly stereotyped manner, so that he is entirely aware of the threatening attack. Indeed, the aura is so short that in but very few cases the epileptic finds time to take adequate precautions, for ex- ample, to prevent falling by lying down. Whether an aura precedes it or not, the epileptic attack proper begins in the most abrupt and "brutal" manner. In typical cases the patient emits a penetrating cry and falls unconscious, while wounds from striking the head or the limbs or from falling down stairs, etc., upon which the patient is at the time, are nothing rare. Biting the tongue occurs from the sudden bring- ing together of the teeth, and in one of my cases this led to almost complete hemisection of that organ. Now a general tonic spasm of the muscles appears, the jaws are pressed together, the fists clenched, arms and legs are rigidly extended, the eyes are directed fixedly forward or are drawn up under the fast-closed lids, the pupils are usually narrowed and reactionless ; also the inspiratory muscles usually take on a tonic contraction and respiration is suspended. Hence, the sudden pallor of the face, which is usually observed in epileptics at the beginning of the attack passes rather quickly over into a livid, indeed cyanotic color. This "tonic stage" of the severe epileptic attack is of very short duration (about one-half minute) and passes over into the "clonic stage," which usually lasts from several minutes to one-quarter hour. The limbs undergo violent contractions, the body writhes, the eyes roll wildly, the countenance is drawn up into frightful grimaces, the head is thrown in all directions. Also in this stage biting the tongue and other wounds can be pro- duced. There occurs also an excessive secretion of saliva which appears upon the lips as a blood-tinged foam. The pupils are still rigid, but now, however, maximally dilated. The respiration is stertorous and can be heard at a dis- tance ; outbreak of sweat and discharge of urine are frequent, rarer, discharge of faeces and ejaculation. The face appears dark red, swollen; the veins of the neck are congested ; sometimes there are small extravasations of blood into the conjunctiva, on the neck, on the chest, behind the ears, etc. Now follows, with gradual cessation of the convulsions, passage into a quiet coma, in which the stertorous breathing little by little changes into the normal type of respira- tion and the cyanosis again gives place to pallor. This last stage of the attack can be of very short duration, but may last from one to two hours; then the patient slowly regains his senses. As a rule, for hours he feels weak, exhausted, depressed, suffers from severe headache, sometimes also from nausea and vomiting. There is complete amnesia for the attack; indeed, the memory of the aura can at times be absent. Sometimes the temperature is somewhat elevated, often there is slight albuminuria which soon disappears. Very rarely transitory paralyses are to be observed in this post-paroxysmal stage, most frequently convergent strabismus. After one or two days they usually dis- appear. Also there are occasionally temporary pareses of one arm or leg, or of the two extremities on the same side: on two occasions I could elicit BabinskVs toe phenomenon (once also, ankle clonus). Further, post-parox- ysmal transitory aphasia as well as fleeting sensory paralyses (post epileptic EPILEPSY 391 deafness and blindness) need to be mentioned. Mushens has found after epi- leptic attacks cutaneous hyperesthesias of segmental type. That a true epileptic attack occurs unilaterally ("genuine hemi-epilepsy" ) . that is. like the Jacksonian attacks sketched in Lecture XIX (page 293), is exceedingly rare. In order to include such cases within the limits of the mor- bus sacer, in spite of the atypical nature of the convulsive attack, one must be able to base his opinion upon a very accurate observation of the course of the disease, the absence of symptoms of a cerebral disease focus and upon a careful study of the history. The occurrence even of a unilateral status epi- lepticus has been pointed out by Midler. As to the frequency of fits in epileptics, no general statements can be made. They may occur very rarely (once or twice a year), or very fre- quently (many times a day), and between these two extremes there lie all possible degrees of frequency. In women the menstrual type, in which the paroxyms always fall in the menstrual or in the pre- or post-menstrual period, are relatively frequent. When in the most severe cases of epilepsy, one attack immediately follows another without the patient regaining consciousness be- tween times, this constitutes the so-called "status epilepticus," a form of seizure which may last for hours and is always very dangerous to life. The body temperature usually increases with each successive attack, so that finally hyperpyretic temperatures — over 41° C. (106° F.) can be reached. Also the frequency of the pulse increases in a threatening manner and the action of the heart becomes weaker. When status epilepticus is going to terminate favorably there is gradually a longer interval between the attacks, the con- vulsions decrease in severity, the pupils begin to react again, the temperature falls, and finally, the patient regains consciousness. In cases terminating fatally on the other hand, there is added to the convulsions a condition of profound collapse in which the pupils remain mydriatic and rigid, all reflexes disappear, breathing becomes continually more superficial, the pulse indeed, slower again but steadily smaller, and finally cessation of heart action and respiration occurs. In the clinical estimation of epileptic attacks, besides their frequency, the time of day at which the paroxysms occurs is important. There are, besides patients who are' only attacked by their convulsions while awake' or indiffer entlv by dav or by night, alse) those in whom exclusively nocturnal attacks occur. Such attacks can be' easily overlooked when the' patient sleeps alone; Wetting tin' heel iii grown people, contusions from striking the' head against the' wall or on the' bedstead, alse> awakening with a feeling of being exhausted and very weak, should always arouse' a suspicion e>f nocturnal epilepsy. The differential diagnosis between 'the epileptic and the hysterical seizure we' will defer until Lecture XXIX. 392 LECTURE XXVI 2. Minor Epileptic Attacks ("Petit mal," Epilepsia Minor, Non-Convulsiva) At the side of the classical epileptic attack just sketched, to whose most striking criteria the convulsions belong, there are now to be placed other forms of seizure which with equal certainty declare themselves manifestations of the morbus sacer, in which, however, the convulsive factor is either entirely absent or only indicated. Common to all, is the paroxysmally appearing, more or less marked clouding of consciousness, which can increase to complete loss of consciousness. In general, however, their symptomatology varies within rather wide limits. We combine them under the name of "minor" or "non- convulsive epilepsy" and place them as "petit mal" in opposition to "haut mal." Many epileptics suffer from both major and minor seizures, while in others all the paroxysms belong to the same type. Let us now enumerate the most important varieties of non-convulsive epilepsy, proceeding from the more frequent to the less frequent. A. THE "MOMENTARY ARSENCE" This peculiar phenomenon may be defined briefly as a sudden and tran- sitory elimination of the higher psychical mechanisms. In the simplest cases the patient ceases suddenly the work which is occupying him, or in the mid- dle of a sentence which he is just speaking, remains quiet a few seconds with fixed look ("as if absent"), then resumes again his occupation or his con- versation as if nothing special had happened, and with complete amnesia for the interruption which has occurred, exactly at the point where the "absence" began. Usually, however, this time is filled out with peculiar automatisms varying exceedingly from case to case. The patient, for example, emits grunting or snoring noises, makes movements of chewing, a few twitching movements of the face or of the extremities, claps his hands, bends himself forward as in "salaam spasm" (see above, page 69), speaks senseless words ("epilepsie marmottante"), etc. Rarely the automatisms assume a more complicated form, as was the case in the epileptic President of the Court described by Trousseau, who in the middle of a session, rose, muttered some- thing unintelligible, went from the Tribunal into the robing-room, there urinated on the floor, then returned to his post and took up again the pro- ceedings where he had broken off. Occasionally during the absence, the occu- pation is not really interrupted, but is carried on in a senseless, purely me- chanical manner, so, one of my patients, a dactylographer, became aware of her petit mal attacks, by finding that in the middle of the text which she was copying, there was a half line of letters placed together indiscriminately. R. EPILEPTIC VERTIGO In epileptic vertigo a feeling of turning or of loss of equilibrium suddenly comes over the patient ; he totters, but, as a rule, before falling, regains his equilibrium. These attacks are usually accompanied by pallor, sometimes also EPILEPSY 393 by the escape of a few drops of urine ; consciousness is practically never com- pletely lost, although it is markedly clouded (a transitory feeling' of con- fusion, of defective psychic orientation). Epileptic vertigo is hence consid- ered as an "attenuated attack" of the morbus sacer. C. EPILEPTIC SYNCOPE Epileptic syncope can be considered as a higher degree of intensity of epileptic vertigo, since it begins, as a rule, with the feeling of apparent move- ment, tottering, etc., still, here the patient has loss of consciousness and ac- tually falls, hut regains consciousness in a short time. Spasms are absent or only slight. Such attacks have been also denominated "apoplectiform." D. THE "NARCOLEPTIC" ATTACK In this there is sudden falling asleep in the daytime and the patient cannot be awakened by shaking, etc. After spontaneous awakening the patient often shows a delirious condition; in any case, however, he has no consciousness of having been asleep. E. THE "PROCURSIVE EPILEPTIC" ATTACK In the paroxysms which have been denominated "procursive epilepsy" (Boufneville and Ladame) there is a sudden running forward in which the patient either avoids objects which stand in his way or forcibly pushes them aside. Here also there is lack of memory for the attack, sometimes also for the period immediately preceding it. A still more rare variety is the "retro- pulsive epilepsy" of Launois, in which the patient in his attacks runs backward. 3. Epileptic Equivalents As equivalents in the broad sense we denominate manifold transitory phe- nomena, which in the concrete ease we consider as of epileptic nature, since they appear to occur in epileptic individuals, or members of epileptic families, in the place of the major or minor attacks propel-, run their course usually with more or less clouded consciousness, are generally followed by a condition of decided prostration, and often react in a striking way to bromide treat- ment (see below). For example, outbreaks of sweat, trigeminus neuralgia, profuse di.i rrhcea ^, Quincke's oedema (see Lecture XXV), sialorrhoea, vomiting, attacks of migraine, stenocardic attacks, paroxysmal tachycardia, spasm of Hie glottis, .tc. .Much more important, however, bhan these sensory, vaso motor and viscera] equivalents (whose inclusion within the limits of epilepsy is through quite a wide extension of hypothesis), .ire the psychical, for which in general w< reserve Hie term "equivalent" in tin- narrower sense. Falret lias divided them according to the intensity of their symptoms into "intellectual grand mal" and "intellectual petit mal," a distinction which i> ton arbitrary to have been generally received. There occur, for example, in attacks, hallucinations with conditions of 394 LECTURE XXVI great anxiety, maniacal deliria, which under the influence of delusions and hallucinations can increase to frenzy and destructive rage, particularly, how- ever, impulsive acts of sometimes quite complicated content. In these differ- ent manifestations the patients are in a condition of abnormal, dreamy con- sciousness ("dammerzustand" "etat second") for which after the paroxysm has passed off, memory is by no means always obliterated (forensically im- portant), almost always, however, markedly cloudy. The impulsive equiva- lents of epileptics as well as epileptic mania, are responsible for many mis- demeanors and crimes. Here belong cases of incendiarism, motiveless homi- cides, thefts in part miscellaneous, in part confined to certain things, often openly carried out, public exposure of the genitals (exhibitionism), etc. Upon an epileptic basis, dipsomanic attacks may also rest, — sudden impulsion to senseless drinking themselves drunk, in previously abstemious persons. These equivalents are, on the average, of considerably longer duration than epileptic attacks proper; the abnormal condition can last for hours, even days. The last is the rule in the interesting phenomenon which has been denominated "ambulatory automatism," "poriamania," or epileptic "wandertrieb." The patient suddenly leaves without motive his place of residence, and wanders blindly about, during which time as far as buying tickets, paying his way, etc., is concerned, he may conduct himself in an entirely unobtrusive and normal manner. When the equivalent passes away, either there is no recollec- tion of the clouding of consciousness, or the patient has the feeling of waking from a dream whose content he but dimly remembers. A patient of Legrand du Soulle gained fame from having been overcome by his poriomania in Havre, and having to his boundless astonishment come to himself again in Bombay. The epileptic clouding of consciousness ("dammerzustand") as it serves as the basis of different psychical equivalents, can appear as the forerunner of a convulsive attack, or in connection with such an attack. In the cases in which it does not occur alone in the place of a seizure, we speak of pre- epileptic or post-epileptic clouding of consciousness (delirium), ("prepar- oxysmal" and "postparoxysmal" or "preconvulsive" and "postconvulsive" would be more correct). 4. The Interparoxysmal Anomalies When we, as is usually the case in the ambulatory treatment, examine epi- leptics outside of their attacks, we find occasionally an entirely normal status and must base our estimation of the case entirely upon the history. Neverthe- less, a thorough investigation in the majority of cases discloses definite anom- alies either in the bodily or in the mental sphere. Under the first we may mention at the start the so-called stigmata of degeneration which many epileptics (as many constitutional neuropaths in general) present. Most frequent are anomalies of the skull of the most varied sort, for example, marked asymmetry, abnormal shape of the palate (Gothic palate), abnormalities of the teeth, microcephaly, etc. Further, malforma-' tions of the ear (adhesion of the lobe, faun-like pointed or protruding, the so-called "jug handle" ears, etc.), and congenital anomalies of the eye (colo- EPILEPSY 395 boma iridis, marked astigmatism, etc.)- Much rarer are signs of degenera- tion on the trunk and on the extremities, as, for example, funnel chest, syn- dactylism, melanodermia. In the second place I would relate the muscular anomalies, above everything the muscular weakness appearing in contrast to the good morphological development of the muscles, which relatively often appears upon one side. Less frequent are muscle disturbances of atrophic character to which Onufrowicz has called attention. So, the occasionally ob- served scapula alata is to be referred to a paresis of the serratus magnus which may be accompanied by that of the trapezius, the rhomboids and the levator scapula. That infantile cerebral palsy is often combined with epilepsy we have already emphasized in Lecture XXII. Also myoclonus epilepsy has al- ready been considered (see page 73). Almost all epileptics show an unusual activity of the tendon reflexes, many are left-handed or ambidextrous. We find further, occasionally, general reduction of the superficial sensibility as well as the most varied speech defects. Finally, the scars of tongue-bites and other wounds occurring in the attacks should be considered. Practically much more important, are, however, the (often progressively appearing) permanent psychic and character alterations of epileptics. We will leave out of consideration here those cases in which the combination of congenital idiocy with epilepsy is present (see Lecture XXII, page 336) and confine ourselves to the consideration of such anomalies which must be inter- preted as the result cf epilepsy, not as a pathological condition coordinate with this last. Many epileptics are excessively quick-tempered and inclined to violence and sudden changes of mood, malignant, cruel, sexually perverse, of brutal egotism. In severe cases, on the other hand, there appears, little by little, a continually plainer reduction of the judgment-forming capacity which increases to actual dementia. In the severest cases the dementia may be com- plete with loss of all mental capacity and even of speech, uncleanness, refusal of food, etc. "Epileptic paranoia," in which ideas of unseen Influence and de- lusions of persecution, on account of the violence of character of epileptics, may be particularly dangerous, i> rare. To these psychical disturbances peculiar to epilepsy are opposed the numerous milder cases in which character and intelligence remain normal during life. It stands beyond question that many eminent personages have been epileptics. I myself know undoubted cases of this sort from my own practice. It must, indeed, be said that in the so- called "pathographies" with retrospective diagnosis, epilepsy has often been diagnosed with unbelievable carelessness, for example, in relation to Napoleon I. That he at the end of the battle of Wagram at a moment when the pursuit of the enemy still imposed upon him important duties, suddenly lay down on the floor and fell into a deep sleep, need not be interpreted as narcolepsy, win n one remembers that for over bS hours in spite of the greatest mental tension and bodily exertion, he had not slept one second. Etiology and Pathological Anatomy Gentlemen: That heredity plays > particularly greal rfile in the patho- genesis of the disease wluhr symptomatology I have now brought before you, 390 LECTURE XXVI there can be no doubt. Binswanger has, for example, been able to recognize mental and nervous diseases among the ascendants of from 35 to 40 per cent, of his epileptics, and the heredity can be equally like or unlike. Just as gen- erally recognized is the frequency of epilepsy among the descendants of drinkers, which we can interpret in the sense of injury to the germ ("blastoph- thoria") through parental alcoholism (compare Lecture VIII, page 127). Further, numerous observations speak for the fact that the changes in the brain which form the basis for genuine epilepsy can be produced, or at least favored, by exogenic factors. This applies particularly in childhood ; that "late epilepsy" is so rare depends upon the fact that after its development is completed, the brain has much less tendency to react to pathological altera- tions by paroxysmal discharges. Among the acute infectious diseases in direct sequence to which, or during whose course the outbreak of epilepsy has often been observed, scarlatina takes readily the first place; next to it are to be mentioned particularly small-pox, influenza, measles and typhoid fever. Among the chronic infectious diseases hereditary syphilis occupies the first place. For late epilepsy, acquired syphilis comes into consideration. Here, however, certain intoxications appear very frequently in the etiology: Saturn- ism, cocainism, alcoholism (when we speak of the latter we must make it clear to ourselves whether or not it is a dipsomanic form which is to be considered as a symptom of epilepsy, not its cause). Circulatory disturbances also may play a role in the etiology of late epilepsy- Often its beginning coincides with tlie development of severe arteriosclerosis; here, also, belong the not rare observations of epileptic attacks in diseases of the heart and aorta. Finally, it is to be considered as proved that injuries to the head can lead to genuine epilepsy (not only to the Jacksonian form depending upon a circumscribed lesion). A special position in the discussion of epilepsy is assumed by the so-called "reflex epilepsy," whose origin still remains a riddle. Sometimes in previously healthy persons after wounds of the extremities, of the trunk, or of the head, there appear typical epileptic attacks which are introduced by a sensory aura starting in the scar left behind by the trauma. Sometimes indeed, an attack may be produced by pressure upon such a scar (which then is denominated an "epileptogenic zone"). Occasionally diseases of the uterus, of the nose and its accessory cavities, etc., can act in a similar manner, while the convulsions of children having intestinal parasites do not usually appear to be of epileptic character. Further, many cases of hysterical attacks have been incorrectly considered as reflex epilepsy. Nevertheless, there remains a respectable num- ber of these cases which have been regarded as truly epileptic by recognized authorities, as for example, Oppcnlu'im. On account of the great rarity of such true reflex epilepsy in spite of the frequency of wounds, we must assume that in the individuals affected, the latent predisposition to epilepsy is anatom- ically predetermined and the irritation from the scar, etc., only furnishes the exciting cause. Excision of the scar or removal of any other pathological condition frequentty does away with the reflex epileptic attacks. Let us now pass on to the pathological anatomy of the morbus sacer. You have remarked that I have repeatedly spoken of a brain disease, of anatom- EPILEPSY 397 ical alterations, etc. There is for me to-day, as for the great majority of neurologists, no longer the slightest doubt that material alterations of the cerebral cortex lie at the basis of epilepsy, though they are here and there too little intense to be discovered by our histological methods. Epilepsy is no "neurosis," and Hippocrates has rightly remarked that its etiology and pathology are in the brain. Chaslin, in 1889, published the first findings, but in late years these have been extended and systematized by Alzheimer through finer methods. In many cases of genuine epilepsy he has shown a "marginal gliosis" of the cere- bral cortex with numerical reduction of the medullated fibres and ganglion, cells ; the latter, besides this, appear atrophic. The vessels are proliferated, show thickened walls, sometimes also lymph sheaths filled with mast cells. In patients who have died in attacks (or in status epilepticus) there are, besides severe acute changes in the ganglion cells and their axis cylinders, also ame- boid glia cells loaded with products of disintegration. The last mentioned anomalies indeed are connected with the epileptic attacks, while the marginal gliosis and its accompanying changes can be held responsible for the inter- paroxysmal clinical pictures, particularly for the dementia and change of character. On the other hand, Alzheimer attributes no great importance to th'- sclerosis of the cornu Ammonis present in 50 to 60 per cent, of these cases, considers it rather only as an occurrence associated with the epileptic degeneration. What, however, causes the intermittent, sometimes even explosive, onset of convulsive or other paroxysmal phenomena? Where peripheral irritative conditions cannot come into question as in reflex epilepsy, it is natural to consider autotoxic factors and many authors have instituted investigations in this direction, into which, since they concern things still quite hypothetical, «!■ cannot inter further. We need only mention as an example, the work of Donath, who asked himself what substances introduced into the circulation of animals are able to set off epileptic paroxysms. lie has come to the conclu- sion that the organic bases resembling ammonia ( triincthvlamin, cholin, ere atinin. guanidin ) act as eonvulsants. He hence believes that in epileptics the occasional accumulation of substances of this sort in the blood irritate the morbidly constituted brain to react by epileptic attacks. That the blood of epileptics during the attack possesses toxic properties appears probable from the investigations of Cent and others, without our knowing how to make much use of this pathogenetic fact. , Prognosis Epileptic attacks usually only indirectly endanger the life of the patient, that is, in consequence of the injuries which he can sustain by falling: as already said, the status epilepticus, whose prognosis in the individual case I have already discussed, furnishes an except ion to this. Though epilepsy in itself does not directly put life into question, still, patients with the severe form seldom live to he old. since they USUallj have, in general, a reduced in- sistence to all possible injurious factors. To definite recovery at mo-t 10 398 LECTURE XXVI to 15 per cent, of the cases are to be brought by proper treatment, still there are along with these, cases in which a very great improvement is to be ob- tained, so that, for example, the attacks only show themselves occasionally, at intervals of a year or more. The earlier the disease begins and the more frequent the attacks, the worse the prognosis as to recovery and also as to mental integrity. On the other hand, the occurrence of major attacks does not at all cloud the prognosis, in contradistinction to the forms manifesting them- selves in minor attacks and equivalents. On the contrary, the last are much more difficult to influence therapeutically and in the long run never leave the psyche intact. Patients with "procursive epilepsy" usually become demented particularly rapidly. T rentmcnt In the foreground of the treatment of epilepsy in spite of the manifold other methods of medication continually appearing (most of which fall again into oblivion), stands always the intelligent application of the alkaline bro- mides which we will hence discuss with a thoroughness corresponding to its importance. In the following I will not consider the severe cases of epilepsy or those combined with psychoses or idiocy, needing asylum care, but will keep in view those suitable for ambulant treatment or management at home. Patients entering upon treatment are particularly to be impressed with the fact, that under all circumstances the cure must extend long after the cessation of at- tacks, also that they must not interrupt it even for a few days of their own accord ; women, also that bromide medication is in no way incompatible with menstruation and pregnancy. Some person connected with the patient must assume responsibility for the strict carrying out of orders and must keep an accurate account of the number, duration and severity of the attacks. Among the alkaline bromides, potassium bromide is undoubtedly the most efficient preparation, probably because it is more slowly excreted than sodium bromide and ammonium bromide (the last is in spite of its high content of bromine, of exceedingly low activity; strontium bromide has almost only his- torical interest). Unfortunately the disagreeable associated effects of bromide medication are much more frequent and intense with potassium bromide than with the other bromine preparations (bromide acne, gastro-intestinal disturb- ances, general prostration, etc.), which sometimes moves us to abandon the potassium for the sodium salt or for "Erlenmeyer's Mixture" — 2 parts each of KBr and NaBr, 1 part of AmBr. The "injurious action of potassium upon the heart muscle" of which we still hear occasionally, need not be brought up against potassium bromide, since it is never observed in practice. To handle a case of genuine epilepsy with organic bromine preparations is an attempt which you will always regret. Next to the choice of a pi'eparation, its dosage is of great importance. Often there is the tendency to make it too low at the start. In general, one should not begin with less than 2.0 KBr a day. Giving a full day's dose at one time (before retiring) is the rule in nocturnal epilepsy; in diurnal forms, however, or in those which do not occur at any definite time of day, you will generally do better (though not always) EPILEPSY 399 if you divide the day's supply into 2 doses of 1.5 each. The patient must take one at bed-time and one at breakfast-time, or — when he tends to have attacks immediately after rising — half an hour before leaving his bed. If this dose proves insufficient we add 1.0 to 1.5 KBr at mid-day. Casus in which daily doses of from -1.0 to 5.0 show no effect, for further increase of the dose (to 6.0 or 8.0 and more) are best brought under hospital treatment or into special institutions for epileptics. Epileptics are almost always more tolerant of bromides than other individuals; particularly so are epileptic children. Since my remarks up to this time — as also the recipes which I will give you later — are adapted to adults, you may note the following figures as represent- ing the usual doses of potassium bromide for young epileptics of slight degree: Single Dose. Dose per Day. Two first years of life 0.1 (gr. 1%) 0.3 (gr. 4>V 2 ) 3d to 5th year 0.2 (gr. 3)" 0.5 (gr. Tl/o) 6th to 11th year 0.5 (gr. 7V 2 ) 1.0 (gr. 15) 12th to 16th year 0.5 (gr. 7 1 2 ) 2.0 (gr. 30) Still, when necessary, children can bear much higher doses very well, for example, 2.5 per day in the fourth year. After the patient has become accustomed to a plainly efficacious initial dose (for this several weeks are needed), for his further management, there is a choice between the following methods: («) The method of increasing and decreasing doses (Charcot). This is especially suited for cases with paroxysms appearing more or less periodically, for example, for the menstrual or pre-menstrual attacks. During the week in which attacks of the last kind are to be expected the patient receives the day's dose proved to be efficient (let us say 1.0 ); during the following week, 3.0 a day: the next week, 2.0; then for one week again 3.0, and finally at the critical period, again 1.0. After several months we try if 3.5 or 3.0 is not a sufficient maximum dose, and if the result is obtained, in the course of several years continually raising and lowering the dose, we endeavor to dispense witli the bromide allogetlii r. (?;) The method of gradual disuse. This is particularly suited for cases with irregular, entirely unaccountable attacks and those in which the seizures rapidly follow one another. The ascertained efficient dose is continued for months, then it is attempted every few weeks t,, reduce it about 0.5 a day. If the case gels worse we return to the next higher dose and endeavor to reduce it again some months later. Even when a gradual withdrawal is carried out without disturbance, we are very cautious about reducing the last dose and wait until no attack has occurred for at least a year. Then we proceed best after the method of Legrand du Saulle: for 3 months, Potassi bromid 200.0 Infus. radic valer. (20 per cent.) ad. 1000.0 M. S. One teaspoonful=1.0 grin. KBr. Ifs Potassi bromide 40.0 ( 10 drachms) Sodii bromide, Amnion, bromide, Sodium benzoat aa 12.0 (3 drachms) Aq. mentli. pip 1000.0 (1 quart) M. S. One tablespoonful=1.0 grm. bromides. (15 grains) 3^ Potassi bromide, Sodium bromide aa. 100.0 (3 oz.) Amnion, bromide 50.0 (l*--. oz -) M. S. Keep in a well-stoppered bottle. X grammes dissolved in water 2 to 3 times a da v.* 1$ Potassi bromide 20.0 (5 drachms) C'odeini phosphor 0.2 (3 grains) 1% Inf. Adonidis vernalis q. s. . . .ad. 300.0 (10 oz.) M. S. One tablespoonful=l gnu. KBr. (15 grains) The individual epileptic attack furnishes, as a rule, no indication for thera- peutic interference. Only in threatening asphyxia must we proceed to artificial respiration, injections of ether, camphor, etc. In general we content ourselves, however, with placing the patient so that he cannot injure himself during the convulsions, loosening his clothing, and placing a rolled-up handkerchief be- tween his teeth to prevent him biting his tongue. A cutting short of the attack through certain manipulations which they have come upon empirically occasionally appears possible in certain patients. One can mainly reckon upon this possibility in the so-called "reflex epilepsy" in which, tying a band tightly about the limb wherein the sensory aura appears can prevent the threatening attack. One of my patients whose attacks are. preceded by an acoustic aura, can prevent it by inflation of the Eustachian tubes; in a patient of Heilbron/ner singing has had the same effect ; two epilep- tics of fipt iilui m's have, indeed, found it possible to effect this end by a sim- ple effort of the will. In status epilepticus active intervention is always in place. Subcutaneous aseptic injections of sterile sodium bromide solution can lie recommended. Dosage (according to Morgan and Hodskins) 100 to 180 cc of a (i per cent, solution: place of injection, thigh or back. The painful induration which these injections produce, must lie treated feu- several days with cataplasms. Large Bromides are very heavy. A teaspoonful equals about 8.0 grm. In order l" spare the patient weighing nut liis dose every li lie can !"■ given :> test tube <»n which is filed a line corresponding In the point I" which ii must be filled in get ■•< definite quantity of (he mixed bromides. 402 LECTURE XXVI doses of bromide of sodium (15.0) can also be given in a clysma; however, the action when given in this way is naturally slower. For rectal application hence, chloral is better suited (4.0 grm in very dilute solution). A further, drug which sometimes is useful in status epilepticus is scopolamin hydrobromide in large, even overmaximum doses (0.001 to 0.002). Also chloroform nar- cosis is sometimes applied as a last resort. The question of the operative treatment of epilepsy, in so far as one has in view genuine epilepsy, and does not consider the results in such epileptiform attacks as are produced by local circumscribed disease processes (cysts, menin- geal adhesions, exostoses of the skull, etc.), is still under discussion. Kocher has expressed himself in favor of the undertaking of a craniectomy through which beneficial relief of pressure is to be effected. Against this theory of re- lief of pressure is. however, to be opposed the fact that Tissot for years has alternately left epileptic patients alone or has subjected them to regular lum- bar puncture. He found that lumbar puncture, regularly repeated, even for a long time, exerted a modifying influence neither upon the course nor upon, the character of their paroxysms. Also he could find no correlation between the pressure and the quantity of spinal fluid on the one hand, and the con- vulsive attacks on the other. In favor of partial excisions of the cortex. .S'. Auerbach has expressed himself of late: for "brain massage," E. Bircher. I must acknowledge that I have seen so many cases of genuine epilepsy operated upon without result, that I am inclined to refer the favorable changes in the character of the disease which have here and thei'e been observed after opera- tions, to accidental coincidence. On account of the dangerous character of epileptics who have undergone psychic alteration in any considerable degree, care should of course be taken that they are committed in time to an insane, or epileptic asylum. LECTURE XXVII The Psychoneuroses Gentlemen : When in the following lectures I describe neurasthenia and hysteria in particular detail, it is. in the first instance, from thoroughly practi- cal points of view. There can be no doubt that the importance which these two most wide-spread nervous diseases have in the work of the physician, stands in striking contradiction to the neglect under which thev suffer in it more brought to our con- sciousness that our specialty is not only a technical one. but to-day still, not deny a direct descent from ^philosophy. The study of hysteria and neuras- thenia often brings US into contact with very interesting persons, and when tiny, in free confession, present us an insight into their psyche, we learn to know mechanisms of the mental processes which are of course pathol but in general bring to us a rich harvest in knowledge of mankind and ps logical understanding. The most satisfactory gain remains, however, I re- peat it again, to therapeutic effort when it is granted to us to remodel un- fortunate individualities, a burden to themselves and others, into normally feeling human 1m ings. K)3 404. LECTURE XXVII A. Psychoneuroses and Neuropathic Diathesis Neurasthenia and hysteria are still much classed in the great group of "neuroses," as which, according to the example of Cvllen, the Scotch physician (1776), are brought together those nervous diseases which depend, not upon organic alterations of the nerve cells and nerve fibers, but usually upon func- tional abnormalities, upon an unruly activity of parts of the nervous system. Now, however, of late years it shows itself almost from day to day more plainly how greatly the composition of this group does violence to the facts, and how entirely heterogeneous diseases are brought in such manner under a common classification. The group of the neuroses is shrinking more and more, and this is indeed natural, since their definition is based upon a negative peculiarity, namely the want of an anatomical basis. We should better say, perhaps, upon our lack of knowledge of such. This grouping carries in itself the stamp of the provisory, since with the refinement of our histological tech- nique, and the progress of our etiological investigations, alterations up to this time unsuspected must come to our knowledge and also toxic and infectious factors will be disclosed. As some decades ago, even tabes dorsalis passed for a neurosis, so later tetanus and hydrophobia still figured under this designation, until finally their microbic nature became evident. That acromegaly, Base- dow's disease, tetany, etc., have as a basis, anomalies of the glandular func- tions, is a recognition of recent date, which must result in their exclusion from the nosological group "neuroses." As far as epilepsy is concerned, I have laid down to you in the last lecture, that to-day a doubt as to its nature as an organic affection of the brain-cortex is no longer possible, and that we must assume, where no histological changes could be recognized, that it was the fault of the insufficiency of technique; the same conclusion is permitted ii} chorea. That on the other hand in neurasthenia and hysteria, as a matter of fact, no anatomical alteration has ever been found, would not suffice in my opinion to prove them the definite survivors from the family of the neuroses; this still could depend upon the weakness of our microscopes and the imperfection of our staining methods. But there are also positive signs common to both affections. To be emphasized above every thing are the transitoriness and changeability, the proteus-like manifoldness in the succession and in the combination of the symptoms, which can make themselves apparent not only from case to case, but also in one and the same patient from day to day, often from hour to hour, while in other nervous diseases, the clinical pictures can repeat themselves from case to case with a stereotyped similarity ; for the troubles of neurasthenics and hysterics, if a paradox is permitted to me, the sole rule which prevails is that of irregularity. Still more important, however, is a peculiarity taking these disease con- ditions out of the limits of other nervous diseases, I mean the greatly pre- ponderant role which in their origin, in the method of appearance of their symptoms, and on account of this also in the curative procedures adapted to combating them, is played by psychic injuries, psychic phenomena, and psychic THE PSYCHONEUROSES 405 influences. On this account we accept the denomination "psychoneuro'ses" which Paul Dubois has introduced for these disease conditions. This word, indeed, expresses in appropriate manner that the whole multiplicity of "'ner- vous symptoms," even where they manifest themselves in the bodily sphere, is most closely connected with mental factors ; in their occurrence, emotions, hypochondriac fears, exaggerated self-observation, play an important role, as do also the suggestive effects of surroundings or the abnormal products of a phantasy directed into false paths. Welcome to us further is the term psychoneurosis as a common designation for the two disease conditions in question. In these latter, the sentence "natura non facit saltum" (nature does not take a leap), is especially applicable. The cases in which we are in doubt as to whether we shall diagnose hysteria or neurasthenia, in which we must assist ourselves with the designation "hystero- neurasthenia" and where transition and intermediate forms of these diseases are present, are excessively frequent. The boundaries in any case are not to be drawn sharply, and if we do draw them, this is chiefly upon didactic grounds, since without boundary marks to some extent definite, we could not locate ourselves in the confusion of symptomatology. In tiresome monotony there meets us, over and over again, the common- place, that the psychoneuroses (or as the popular equivalent has it, "nervos- ity") are diseases of modern civilization. This is applicable only with limita- tions, since hysteria, and indeed in its most severe and striking forms, is as old as humanity. This must be plainly apparent to every one who knows how to estimate in their proper nature the hysterical epidemics of ancient times and of the Middle Ages, and Hippocrates, even, has furnished good descriptions of hysterical symptoms, which he also recognized as such. It is somewhat differ- ent, indeed, with neurasthenia; though there are neurologists who speak of the '"discovery" of this disease by the American George Beard in the year 1887. Of course this author did not discover neurasthenia, hut studied and described it in masterly fashion, recognized it in its nature, and furnished it with a fortunately chosen name. When, however, we look over the medical literature of preceding decades, we find under other designations (such as "nervosism," "nervous erythism," "general hyperesthesia," "cerebro-cardial neuropathy," "spinal irritation") disease pictures which present typical neurasthenic symp- tom-complexes; particularly, however, is it the diagnosis hypochondria with which since ancient times, neurasthenia lias been saddled. With the old authors hypochondria played a preponderant role; to-day we make this diagnosis only in rare cases in which there is a mental disease proper, with thoroughly sys- tematized delusional conceptions built up upon fears of disease. The picture, however, which Moliere in his "Malade Imaginaire" draws of a presumable hypochondriac, is that of nothing else hut an excellently observed and. in spite of caricature, unmistakable neurasthenic. The disease which in the seven teenth century under the popular name "Vapors," scourged the Court ami noble circles of France, was scarcely anything else; in the letters of Mme. de SSvignS and of the Abbi de Brosse then are proofs of this. It would lead me too far afield to enter into the documents bearing upon the history id' neuras- thenia furnished by the ancient writers; for example, the Roman satirists, but 406 LECTURE XXVII I cannot fail to cite a clinical history taken from the fifth Hippocratic book, "On Epidemics." This was of a patient "Who suffered from bodily relaxa- tion. He could not pass by any pit, neither could he go on a bridge, nor yet was he in a condition to cross over even the slightest trench, although he could walk in the trench itself. Such things happened to him for a very long time." You see here the unmistakable sketch of obsessive ideas which are so frequent in severe neurasthenia. The description of "Gephyrophobia," as we term it to-day, is indeed, classical. Taken as individual diseases the psychoneuroses need not be designated as the regrettable privilege of our present epoch of culture. Now at present, however, that is for about the last three decades, we have not alone to deal with single cases or with epidemics bounded by time and place, but with a definite nervosity of peoples and races which although not dangerous to life, is nevertheless of great importance, since it injures intensely the capacity for work and productivity of extended strata, of all classes of society. Into the causes of this threatening increase of the psychoneuroses I will not enter at the start. We will reserve the pathogenetic considerations for the next lecture. In order to give you, however, a comparative numerical conception as to the extent of the psychoneuroses, I would refer to the investigations of Cramer among the Gottingen student body. By comparison of the whole number of students with the number of those who sought professional advice on account of nervosity, he found the figures of 30 to 40 per cent., and thinks that these figures are certainly not too high, since the students are, with few exceptions, individuals who have as yet been little shaken up by the storms and experiences of life. On the other hand, however, I might point out that among students, other nerve-injuring factors come into action in a relatively high percentage of cases ; fear as to examinations, venereal diseases, excesses in drinking, etc. In any case the comparison drawn by Cramer, with the frequency of psychoses, is interesting; among 1,000 people there are 2 insane persons needing asylum care, and 2 who can more or less get along in the world. Shall we class the psychoneuroses with the congenital or with the acquired diseases? This question I can answer you neither in the affirmative nor in the negative. It is true that on the one hand external injurious factors can pro- duce neurasthenia and hysteria; on the other, however, that for many cases the manifestation of an inferiority present in the organism from the start is re- sponsible. Between the congenital, endogenic, and the acquired exogenic fac- tors, there is only a relation of reciprocity. External injurious factors of considerable intensity can make even an individual who possesses from the start a robust nervous system neurasthenic or hysterical. In others, however, witli the best will and through the most exact anamnesis, we cannot elicit any ade- quate external injurious factors; here it is to be assumed (and often to be recognized) that such individuals have brought into the world a nervous sys- tem having abnormal lack of resistance, so that they are unable to withstand the demands of life, even on the school-bench, or still earlier, and fall ill under conditions which to one healthy nervously, "ab ovo," would be entirely harmless and irrelevant. We come here, also, as in so many branches of pathology, upon the con- THE PSYCHONEUROSES 407 ception of "predisposition." In this special case this is denominated "neuro- pathic diathesis." This conception has been much ridiculed, since it contains in itself much that is vague and unsatisfactory, but even the most exact branch of medicine, bacteriology, has had to recognize that it cannot get along with- out the conception of predisposition. Only in this way can we understand all the remarkable leaps which an epidemic makes, in that it affects one individual, spares another, although it is known that this last one has been exposed in a high degree to the danger of infection. Close relationships join the question of neuropathic diathesis with that of "hereditary predisposition." One can well say to-day that the importance of this last is greatly overestimated in the occurrence of the psychoneuroses. It has been implicitly assumed that the healthy person must be free from "hereditary predisposition." This is, however, not so. J. Roller has found that among the relations of 370 healthy persons, different neuroses and psy- choses were to be found 218 times, which corresponds to a percentage of 59. These figures, high beyond expectation, make for the critical observer the as- sumption of a sure causal nexus between the psychoneurotic disease of an individual and the neuro- and psycho-pathies occurring in his family, a matter of opinion which must in every individual case be particularly weighed and considered. Also it must continually be emphasized that from an entirely healthy stem neuropathic-ally inclined offspring may be produced. Here, now, in relatively numerous cases the "blastophthoric" injurious factors of which we spoke in Lecture VIII are to be found among the ascendants. In severe cases of neuropathic diathesis somatic signs of congenital inferiority appear to us in the form of the so-called stigmata of degeneration to whose importance Lombroso, in his studies on "The Congenital Criminal," lias referred. We consider as such stigmata: Marked asymmetry of the skull not to be explained by exogenic factors (for example injury at birth), anomalies of the teeth, greal prominence of the upper jaw or of the lower jaw (prognathism, caput progeneum, Gothic palate, cleft palate, harelip), anomalies of the external ear (jughandle ear, adherent lobe, pointed ear), hypospadias, genital infantil- ism, syndactylism, web fingers, gampsodactylism (inability to extend the little finger), etc. B. Neurasthenia Since we have touched upon the most important points which should pre- pare the way for the understanding of psychoneurotic manifestations in gen- eral, we will pass over to the description of those disease pictures which we bring together as neurasthenic; those of hysteria we will reserve until later, since tiny are mainly more complicated and more difficult to understand. Mid for the present in the consideration of the neuropathic phenomena we will put aside all theoretical and all pathogenetical considerations and make our de- scription purely clinical. 408 LECTURE XXVII Symptomatology The disease manifestations of neurasthenia separate into objective and sub- jective, of which the last play a specially important role. On account of this, the result of the anamnesis is just as instructive as that of the status, and we have to devote particular attention to hearing what the patient has to say. In this, however, a certain routine is an absolute requirement. It indeed is one of the peculiarities of the neurasthenic to enter with the utmost detail into each single symptom of his disease, and he usually makes no difference, or no sufficient difference, between the important and the unimportant. Noth- ing is more unwise than to cut him off shortly, when his conversation is super- fluously long. The neurasthenic takes this very ill, and a physician who is im- patient, or more correctly, who gives evidence of his impatience, will rapidly lose confidence and authority with such patients. One must hence learn to cur- tail the prolixity of his description of his disease, by properly interpolated questions, turning the narrator away from the unimportant and guiding him to the symptoms diagnostically important. A considerable fraction of neuras- thenics frequently aid us in taking the anamnesis and compiling the clinical history, in that they hand us written sketches of their disease (in which no symptom is unconsidered or forgotten) ; "l'homme aux petits papiers" (the man with the little papers) Charcot hence has called the neurasthenic. Let me place before you two examples of such auto-nosographies with the remark that we cannot expect always such short and expressive descriptions: 1. '"Symptoms. — With ever so slow increase of severe pains in the chest and in the back from the neck to the anus. Burning over the whole body, par- ticularly severe in the legs, a biting feeling over the whole head. Three and one-half to four hours rest at night, then sudden awakening, trembling over the whole body and no more sleep ; sweating at night, by day very frequent nervous tremor, anxiety, uncertainty in walking. Attacks of vertigo very frequent." 2. "Remarks for the Doctor. — Often headaches and sleepless nights. Short dizziness, a feeling as if the head was drawn backward, pain when there is noise, starting at every noise, for example, the fall of a tablespoon. One morning I felt a stiffness on the left side, felt myself strange in my own house, the people and everything appeared to me different. Little by little the old impressions returned to me, only memory remained poor. Visually the stiff- ness on the left side repeats itself, indeed it is noticeable also on the right. Sometimes stabbing headache and on strenuous work, sense of nausea. An ex- ceedingly painful uncertainty pursues me the whole day, then I question myself, must I do that, is it I or only a dream, and the interpretation is now obscure- in my head. I cannot any longer collect my thoughts." These two typical personal observations respectively of a patient with principally somatic symptoms and of one with predominantly psychic symp- toms ("psychasthenia") show how extremely manifold the symptoms of the psychoneuroses now occupying us can be. It is hence well for us to proceed quite systematically to bring the greatest possible order out of this confusion and to subject the chief phenomena of neurasthenia to a separate considera- THE PSYCHOXEUROSES 409 tion. We will suppose a fully developed case of acquired neurasthenia, that is, in which the special congenital degenerative characteristics do not occupy the foreground. The degenerative forms will later be discussed as well as a few semiologically characteristic varieties, such as the so-called '"sexual neuras- thenia." Since in the course of talcing the anamnesis we become acquainted with the subjective complaints while only examination into the present condition brings the objective symptoms before us, it is justifiable to begin our description with the first group of disease manifestations. 1. PSYCHIC ANOMALIES a. State of Feeling The basal symptom in the state of feeling of the neurasthenic is what George 'Beard has characterized as irritable weakness, an abnormal irritability and exhaustibility. The patient is irritable; trifles ("the flies on the wall") disturb him. To disagreeable occurrences, or to actual mental pain he reacts in im- moderate manner, after the emotional discharge to sink back into deep apathy. Even agreeable happenings, pleasurable stimuli, produce excessive reaction, and then weakness and exhaustion often with psychical depression. To "sky- high exaltation" succeeds "'mortal depression." Still the basal state of feeling upon which such episodes arise is usually a comparatively depressed one. rather morose than sad. This ill humor is in the main to be considered as a second- ary symptom corresponding to the uncomfortable general condition; and re- flections about the diseased ego play in this a considerable role. In any case, it is a quite different symptom from the depression of the melancholiac, since this last in no way arises from reflection, but without any thought as to its cause, it impresses itself upon the patient with overpowering might. Anxiety mainly of a hypochondriacal, sometimes also of a superstitious coloring, as well as defect of energy and inability to form conclusions, are also quite char- acteristic. b. Intellectual Capabilities In tlir intellectual sphere on the other hand, no important disturbances arc noticeable except for the abnormally rapidly occurring and tin- abnormally disagreeably perceived fatigability, which gives to the mental work of the highly endowed and talented persons, who form no small proportion of pa tiente with acquired neurasthenia, an aphoristic ami broken-off character. In any event the patients often' complain of "reduction of their mental force," that flu' ability to receive and combine impressions and the memory have suf fered. A more exact investigation, however, makes it plain to us that these functions are in fad uninfluenced, and that only the continued occupation of the patient with the observation and explanation of his troubles prevents him from turning his attention sufficiently to things of the surrounding world. This "'narrowing of the- field of consciousness," brings it about that a neuras- thenic must read the same sentence several times before he understands it, that 410 LECTURE XXVII nanus, words, dates, easily escape him (since he has imperfectly noted them), that he is unable to concentrate his thoughts strictly upon an intellectual task. 2. HEADACHES More than half of all neurasthenic patients complain of headache. If we require them, however, which we should not neglect in any case, to give an accurate description of this trouble, we will usually find that there are not actual pains, but only abnormal, more or less unpleasant sensations. Mainly we have to do with what Charcot called "le casque neurasthenique" (neuras- thenic helmet), a feeling of a weight pressing upon the whole skull. Frequently the pressure is located only in the frontal region, more rarely at the back of the neck. Other patients complain of a feeling that the brain is threatening to burst the skull, or, on the contrary, that it is too small, that the head is empty. Often it is only a "dullness in the head." Of true severe headaches only such neurasthenics who live in terror of suffering from a severe organic brain disease, as brain tumor or brain syphilis, complain usually. Neverthe- less, such head troubles increased in consequence of intense fixation of the at- tention and autosuggestive factors to considerable intensity, are to be recog- nized as to their neurasthenic nature before exact investigation, by the fact that upon the mental abstraction of the patient they disappear. 3. SLEEPLESSNESS This, also, constitutes an exceedingly frequent complaint of neurasthenics; still, their expression "sleeplessness" is never to be taken literally, even al- though the patients expressly insist that they cannot sleep at all, "hear every quarter hour strike." In such cases one has to do, as a rule, only with an insufficient depth of sleep, with a condition of half sleep, which we, with the Hungarian author Lechner, who has studied the disturbances of sleep particu- larly thoroughly, will designate "Dysnystaxis." Other patients suffer from difficulty in falling asleep ("Dyskoimesis"), or from too early awakening ("Dysphylaxia") although sleep is deep enough. Distressing dreams, night- mare, are not rare. The patients very often, indeed, complain of a sudden starting which frightens them just as they are going to sleep. Even when the duration and the depth of sleep apparently present no particular anomalies, the refreshing action of sleep is absent and we hear then that the patients "arise more tired than they were when they went to bed." Especially trying is a drowsiness during the day which is in great contrast with the abnor- mal wakefulness at night. "Night is the enemy of the nervous," correctly says Hermann Oppenheim, who has subjected the genesis of the neurasthenic disturbances of sleep to a keen analysis. With the abandonment of activity and all external interests, the attention of the neurasthenic may be turned in a higher degree to his bodily processes, so that there occurs a hyperesthesia toward sensations which the healthy person does not perceive at all, for in- stance, his own heart-beat, the peristalsis of his intestines. These continued sense perceptions naturally oppose going to sleep, for which the elimination THE PSYCHONEUROSES 411 as far as possible of the irritants reaching the sensorium furnishes the best conditions. Through a specially exaggerated "self-perception" every organic feeling can take on an actually painful coloring, on account of which we speak of " hypnalgias" and "nyctalgias." The healthy person also for all that, can provoke such phenomena in himself by way of experiment. Attempt during the stillness and darkness of the night when your attention cannot be dis- tracted by external perceptions to fix this in the greatest concentration pos- sible upon a definite part of your body, for example, upon one heel; you will in a few minutes perceive an unpleasant sensation in this region. The contact with the sheet will be first found noticed as an unpleasant tickling, then as itching and burning, and in case you do not allow your attention to be dis- tracted in some other direction, finally as actual pain. Upon the basis of this personal experiment which can be repeated without difficulty, I cannot agree with Oppenheim when he writes that the interpretation of hypnalgias and nyctalgias gives particular difficulty. 4. IRRITATIVE SYMPTOMS ON THE PART OF THE SENSE ORGANS Exaggerated feeling of self furnishes us the key to the understanding of a number of subjective ear and eye symptoms which occur in a large number of neurasthenics and in which we are justified in assuming a morbid irritability of the sense nerves in question, a reduction of their threshold of irritability. The following irritative symptoms are to be observed: Hyperesthesia to light, blinded feeling, spots before the eyes, hyperesthesia for noise, so that such patients, for example, perceive the whistle of a locomotive as actual pain and anxiously avoid one. Further, tinnitus auriurn, sometimes rhythmic, synchro- nous with the pulse, and in this case dependent upon the perception of the noise of the circulation within the skull, to which the cochlear nerve with normal threshold of irritability would not react. The entoptic perception of neuras- thenics, denominated "musci volitantes," has nothing to do with similar phe- nomena in opacities of the vitreous body (indeed, ophthalmoscopic examination Shows hen- tli.it all the media of the eye are clear), and is probably produced by the circulation in the retina or by the corpuscular elements of the tear secretion. Acuteness of vision and hearing remains uninfluenced in all the phenomena mentioned. The single defect symptom which neurasthenia may pro- duce in the visual apparatus is usually to be referred to the abnormal fatig- ability of the internal recti and of the ciliary muscle; these are the SO-Called "asthenopic" troubles which make themselves apparent after a more or less .short time, upon fixation and accommodation for near objects. 5. VERTIGO Only in quite rare cases do Inn attacks of vertigo with disturbances of equilibrium occur (for example having a rotatory vertiginous character), which can be considered as an irritative symptom on Hie part of a pathologi- cally irritable vestibular apparatus, wliieli answers in this manner to Midden 412 LECTURE XXVII variations in the blood distribution in the internal car. When, in spite of this, our neurasthenic patients complain of vertigo with excessive frequency, this comes from the fact that under this term, the public understands very different things. A momentary fit of weakness, the feeling that it is suddenly black before the eyes, as if the legs were sinking into the floor, or as if they were taken away from the body, all these abnormal sensations generally come under this designation. If a neurasthenic has once experienced a subjective difficulty of this sort the fear of "vertigo" usually makes itself felt in very marked form and the reoccurrence of such symptoms naturally increases considerably this fear, a painful, vicious circle. Fortunately in very few cases does an actual "jaermanent vertigo" appear. 6. PARESTHESIAS Abnormal sensations occur in neurasthenics not only in the regions of the skull and the nerves of special sense, but also in those of the skin, the mucous membranes, muscles, bones, tendons and joints, in varied and manifold forms. If they are also of unpleasant character and indeed actually painful, so is there here also, as we emphasized under the "Headache" and "Sleeplessness" of neurasthenics, very frequently an unconscious tendency to exaggeration. Not at all infrequently such patients complain of their "terrible" pains not ceasing a moment, but their healthy appearance, their expression, their abstractibilit y, the fact that they can exercise their calling undisturbed, etc., form such a con- trast to the patients who suffer from the lancinating pains of tabes or trige- minal neuralgia, that we are in a position without difficulty to reduce their description to its proper proportion. The most frequent seat of such pain- ful paresthesias is the back; we speak then of spinal irritation, of rachialgia. But also in the muscles of the limbs are located various sensations which probably correspond to those which the normal person perceives after intense exertion as the well-known "fatigue pain." In rare cases "dysbasia neuras- thenica intermittens," which should not be confounded with the angiogenic, forms of intermittent limping, can so occur (see above, pages 225 and 374). Here, probably, the two components of "irritative weakness" (morbid increase of motor fatigability on the one hand and of sensory irritability on the other) play an important role. As paresthesia of the bones is to be interpreted the so-called "anxietas tibiarum," a dull, difficultly definable feeling in the bones of the leg — whose parallel occurring in healthy people is the well-known sensation designated by the expression, "the fright has got into my legs" — which can be experienced continuously by the neurasthenic. Neurasthenic arthralgias have led to confusion with rheumatic joint diseases. In contradistinction to these last, however, they increase in intensity during rest, diminish on movement, a criterion which applies to many neurasthenic pains, as Kollorits has expressly emphasized. Pruritus, itching in the skin and mucous membranes forms, finally, a frequent complaint of neurasthenics. It is almost never general, as are often the parasitic, the senile, and the forms occurring in diabetics and arteriosclero- tics ; it is rather, almost always, localized upon definite areas. Itching of the pharyngeal mucous membrane leads to dry, nervous "irritative cough," pruri- THE PSYCHONEUROSES 413 tus of the urethral or rectal mucous membrane to distressing tenesmus of these excretory passages; pruritus of the genitals may be the starting-point for masturbatory practices. It is self-evident that the diagnosis of nervous pruri- tus should only be made, when an exact investigation can exclude the other disease conditions above mentioned. When we turn our attention to the subjective symptoms of neurasthenia. we are first expressly reminded of the polymorphism of the neurasthenic disease picture. This brings it about, that we need not expect in every neurasthenic to find represented each of the four groups of symptoms into which, upon didactic grounds, it is well to arrange these anomalies. Still, almost never will a neurasthenic fail to show any objective symptoms at all, so that their diagnostic importance is not to be underestimated. 7. INCREASED IRRITABILITY OF THE NEUROMUSCULAR APPARATUS This anomaly expresses itself particularly in a general increase of the bone and tendon reflexes, which indeed in general remains below that which we find in organic affections, for example, in the spastic symptom-complex, but still is occasionally so intense that a doubt may arise in this direction. In such cases, the fact that functional increase of reflexes is never accom- panied by Babinski's, Oppenheim's, or the Mendel-Bechterew phenomenon, asso- ciated movements, etc., is of great importance. Further, permanent hyper- tonia of the muscles is absent. The so-called ''functional" or "pseudo" ankle clonus could at most give occasion for confusion. Still, there are never here the thoroughly rhythmic, even contractions which can lie provoked as long as desired, which characterize true organic ankle clonus, but irregular move- ments of varying amplitude, which, besides, are soon exhausted. This is shown most plainly in graphic reproductions of true and false clonus upon Up- rotating drum ( clonograph ) , still, an examiner of any experience can al- ways dispense with this complicated method of investigation. A reduction of the tendon reflexes can some! inns he found in such neurasthenics who, in consequence of marked nervous dyspepsia, are in a condition of considerable muscular emaciation. The absence of a reflex phenomenon normally constant, particularly also of the patellar reflex, in a neurasthenic, indicates without exception a com- plicating organic anomaly. Also the skin reflexes, namely the plantar reflex, are in neurasthenics sometimes exaggeratedly lively. Frequently an increased mechanical muscle irritability is to hi' found, so thai even slight blows with the percussion hammer upon the extensor side of the forearm set up a powerful motor ell'ect at the wrist and in the indi- vidual fingers. Also in the quadriceps and in the pectoralis major is this mechanical hyper-excitability of tin- muscles often very clearly to In- recog- nized. The "idiomuscular contraction" is sometimes very plainly visible. On the other hand, mechanical o\ erirril aliilit y of the nerve trunks is iniieh rarer. It is most frequently demonstrable upon the ulnar nerve In hind the elbow, •114. LECTURE XXVII in that by rolling this nerve under the palpating finger a definite contraction of all the muscles supplied by it can be produced. 8. TREMOR AND SIMILAR PHENOMENA In the great majority of neurasthenics, when we request the patient to close the eyes firmly we can notice a marked tremor in the orbicularis palpe- brarum. The slightest degrees of this phenomenon are, as you should note, presented sometimes by an entirely healthy person, for instance, in a condition of fatigue, after long reading; in the neurasthenic, however, this may increase to a blinking, which is increased upon attempt to keep the eyelids as still as possible. Less frequent are tremor of the outstretched tongue, and that of the fingers which is usually very fine and vibrating. Quinquaud's symptom, already mentioned in Lecture IV, page 63, is by no means infrequent in neurasthenics, while the phenomena of fibrillary contractions and myokymia described in the same lecture are only very exceptionally observed here, and hence should naturally only be considered as neurasthenic, after exclusion of an organic substratum. In functional cases they occur most frequently in the first inter- osseus of the hand. 9. CARDIOVASCULAR AND RESPIRATORY DISTURBANCES The cardiac disturbances in many neurasthenics to such an extent occupy the foreground in the symptomatology that these patients frequently turn at first, not to the neurologist, but, thinking that they have heart disease, to the internist. So, Gerhard t affirmed that over half of the patients who came to consult him about heart disease actually suffered from "neurasthenia cordis." On the other hand, you are expressly warned against making this diagnosis too precipitately, and I greatly recommend to you to carefully examine the heart, the vessels, the urine, etc., even in "plain neurasthenias," in order not later to have to reproach yourself for having overlooked a myodegeneration of the heart, a contracted kidney, a coronary sclerosis, etc. The most im- portant objective symptom of the nervous heart is the quickening of the pulse, which may be permanent — analogous to that which we have learned to know in Basedow's disease, — but which shows itself usually only on occasion, by preference at night, but also during the day particularly with psychic dis- turbances. In exaggerated cases there is true paroxysmal tachycardia, with abrupt rise in the number of heart beats to 160 to 200 per minute. A diagnostically valuable symptom is, further, the increase of lability and variability of the pulse-beat. By lability I understand the difference between the number of beats under different conditions, for instance on change of position (lying, sitting, standing), on effort (for example, mounting upon a chair 3 to 6 times, rapidly one after another, which corresponds to a work of 100 to 200 meter-kilogrammes) — as variability, the difference between the pulse-rate on different days (measured as near as possible under identical conditions). In healthy people pulse lability and variability are slight; in cardio-vasomotor neurasthenia, on the contrary, exceedingly great. The same THE PSYCHONEUROSES 415 tiling applies to the lability and variability of the blood-pressure, as I have been able to determine by measurements with Gartner's tonometer, which for the rest in such forms is on the average abnormally high. While, for example, I found in healthy persons an average of 95 mm with the Gartner apparatus, among 27 neurasthenics with heart and vascular symptoms I found the blood- pressure only 7 times under 100, 8 times between 100 and 115, over this 12 times. The maximum was 160. Only in two cases were the figures abnormally low, 75 and 60 mm. To be placed parallel to this is the great rarity of a functional nervous bradycardia. Where this is present in neurasthenics, it can often be found that there is a combination with nicotinism. Irregularities of the heart action also belong to the rare objective symp- toms of neurasthenia. Among them, true "pulsus inasqualis" (allorhythmia) upon a purely functional basis, that is, if an affection of the heart muscle can be certainly excluded, may be counted a rarity ; somewhat more frequently the heart neurosis is accompanied by the missing of individual contractions, extra- systoles or arhythmia. In no way unusual, however, is the abnormal response of the heart to the respiratory phases, in that on inspiration an alteration of the pulse in the sense of "pulsus respiratione intermittens" makes itself more or less apparent. That upon a purely' nervous basis, variations in the size of the heart, the so-called "acute dilatation of the heart" can occur, I do not consider proved; also, in spite of the favorite diagnosis "nervous heart weakness," I have al- ways considered the finding of symptoms of stasis (engorged liver, congestion of the kidney, cyanosis of the lips, oedema, etc.), even when these were only of slight degree, as proof of an organic heart disease, anil this has always proved correct. On the other hand, in neurasthenics "cardioptosis" is a strik- ingly frequent symptom; if such patients are placed upon the left side the apex-beat moves across the mammillary line outward, and this "movable heart" i- also to be recognized by percussion. Further the second sound over the aorta and the pulmonary valves is often abnormally loud. According to Oppenhebm, upon excitement, besides increased rapidity of the pulse, a tem- porary systolic blowing may appear. I have never been able to confirm this. The objective heart anomalies just enumerated are practically always accompanied by subjective troubles, among which palpitation, the feeling of painful thumping of the heart, occupies the first place. Of other sensations, precordial pressure, which may increase to actual "pseudo-angina pectoris," comes into consideration. Between these last, in which psychogenic compo- nents in the sense of "exaggerated perception" which we have considered in the preceding lecture must play the chief role, and which also has properly been designated "precordial anxiety," and the already mentioned "angina pectoris vaso-motoria," the boundaries are not always to be sharply drawn. An original symptom of cardiac neurasthenia, which Trommer his described, you can hen' ami tin re confirm. This is a soiled area on the shirt-bosom oi male patients, corresponding to the position of the heart, which has been produced by their constantly placing the hand upon the heart region on account of the disagreeable sensations there localized. Of vasomotor disturbances there an most frequently to he found in neuras- 416 LECTURE XXVII thcnics "rushes of blood," that is, congestions of the head with great local feeling of heat ; the ears and cheeks feel hot and sometimes the cervical vessels heat with exaggerated intensity. With the hyperemia of the head is contrasted often the coldness and lividity of the hands and feet, also a rapid alternation of reddening and pallor is peculiar to many patients. A cooperation of the psyche manifests itself in many cases in characteristic manner; his tendency to frequent and motiveless blushing is well known to the patient; he fears it ("ereuthophobia") and this preoccupation is to blame that blushing actually occurs regularly upon meeting unknown persons, in any sort of situation not entirely common. Also circumscribed and migrating erythematous spots ("fliegende roten") are a frequent symptom in neurasthenia. Dermographism and factitious urticaria occur in the same manner, as has been described for Buscdoio's disease. But many neurasthenics also suffer from spontaneous urti- caria, and are further, as we already emphasized in the beginning lecture, predisposed to different vasomotor neuroses. Much more rare than the vasomotor-cardiac, are respiratory symptoms in neurasthenics. In spite of the certain relations of bronchial asthma "to nervous influences there are actually among the great horde of neurasthenics strikingly few typical asthmatics. What is designated by such patients as asthma is generally only a "nervous tachypnoea" sometimes running parallel with the tachycardia. 10. ANOMALIES OF SECRETION The secretory anomalies of neurasthenia are somewhat less frequent than the cardio-vasomotor. Most widespread is readily the tendency to excessive outbreaks of sweat, general or localized ( forehead, hands, feet) ; in the second place are to be mentioned anomalies of the gastric secretion, among which hyperacidity is the rule, only exceptionally hypacidity, or even anacidity. The occurrence of an acid gastrorrhoea, in attacks, is called "paroxysmal gastroxynsis." Increased salivation and polyuria are rare, exceedingly fre- quent, on the contrary, the symptom denominated, little justifiably, "phos- phaturia," a precipitate of the earthy phosphates (calcium and magnesium) soluble in acids, present in freshly passed urine, or occurring when it is warmed. Without a trace of proof this precipitate has been improperly interpreted as a sign of increased decomposition of phosphorus containing nerve substance (lecithin, protagon). This view, however, may be considered as aside from the subject, so long as it is not supported by exact experiments on metabolism carried out under all precautions upon such phosphaturic neu- rasthenics, and there are no such at present. With the simple determination of phosphates in the urine nothing can be decided; satisfactory conclusions can only be expected from the exact determination of the phosphorus intake and its excretion by the neurasthenic during a given time. At the present time I consider it probable that simple alteration of the conditions of solu- bility in the urine are to be held responsible for the so-called neurasthenic phosphaturia. These are perhaps due to abnormalities in the production of acids in the body. Finally, is to be mentioned the "mucous colic" or "pseudo- THE PSYCHONEUROSES 417 membranous colitis," in which, in attacks with severe abdominal pain, tube- like formations of mucus and fibrin are discharged. This disease, a secretory neurosis of the colon, is observed particularly in psychoneurotic patients (mainly females) ; the popular designation, "nerve mucus," customary in many places, shows sharp observation on the part of the laity. LECTURE XXVIII The Psychoneuroses B. Neurasthenia (Continued) Gentlemen: We have now brought to an end the analytical consideration of the manifold subjective and objective semiology of acquired neurasthenia, begun in the last lecture, and from now on we will direct our attention more to the grouping of symptoms than to single symptoms. It is self-evident, and I have sufficiently emphasized it, that all these disease symptoms cannot be present in every neurasthenic ; rather must an exceedingly great variety of clinical pic- tures arise, according to the special prominence of this or that anomaly and the absence or recession of other disturbances. The necessity for systematiza- tion has given rise to the separation of the disease according to such so-called "■localizations" of the neurasthenias into special varieties. So, many neurologists speak of cerebral neurasthenia or encephalasthenia when the troubles of the patient consist chiefly in pressure in the head, tin- nitus aurium, vertigo, disturbances of sleep, irritability, etc. As hyperalgetic neurasthenia has been denominated, that form in which pain in the back and disagreeable and painful sensations in the limbs constitute the chief complaint. Such disease pictures, however, in which palpitation of the heart, congestions, tachycardia, fleeting erythema, dermographism, etc., predominate, are classed as cardio-vasomotor neurasthenia. These three varieties, however, show so many transition forms and combinations that their separation must be con- sidered quite an artificial one. A much more independent position must, on the contrary, be assigned to those varieties of neurasthenia which are denom- inated nervous dyspepsia and sexual neurasthenia, hence you shall have a connected exposition of these before we turn to the etiology and pathogenesis of neurasthenic conditions. NERVOUS DYSPEPSIA Here disturbances on the part of the digestive organs to such an extent occupy the foreground that it is the stomach specialists, and not the neurolo- gists, who have contributed mainly to our knowledge of this exceedingly widespread form of disease. The average picture of nervous dyspepsia, which naturally presents the most varied degrees of intensity, is about the following: The patients com- plain of a frequent burdensome feeling of pressure that either comes on after meals or is independent of taking food. The qualitative character of the food 418 THE PSYCHONEUROSES 419 is in remarkable manner often a matter of entire indifference, or, indeed, there is the paradoxical phenomenon that heavy, fat. acid foods, etc., are best borne; hence, sometimes the peculiar "regimes" which many such patients lay down for themselves, and to which they hold fast with great pedantry and obstinacy. Precipitate change of the disease picture is a frequent occurrence; often we seek in vain for an adequate cause for the beginning of the dyspeptic trouble, very frequently, however, the influence of psychic factors is un- mistakable; an anger, an excitement, sometimes a ridiculously slight annoy- ance, has '"fallen on the stomach" of the patient, and only with the passing away of the mental annoyance the troublesome symptoms disappear. Fre- quent accompanying symptoms of stomach pressure arc sour or stale eruc- tations, anomalies of the stomach chemism (usually in the sense of a hyper- acidity (see above, page 416), heartburn, regurgitation). The appetite is usually capricious, anorexia and buliminia can alternate with one another. Quite characteristic is a painfully perceived feeling of hunger which usually occurs on an empty stomach (particularly at night or upon awakening in the morning), and has been called by Boas "gastralgokenosis." The tongue often looks quite normal; usually, however, it is slightly coated; also the intestinal functions are in very many cases disturbed; there is most frequently atonic constipation {KwssmauTs "torpor peristalticus"), sometimes spastic constipation; that for the rest the combination of atony in the proximal and spasm in the distal divisions of the colon occurs, Stierlin has of late recog- nized roentgenological^ - . Among the spastically constipated dyspeptics, the disposition to occasional paroxysms of mucous colic is most widespread. More frequent, indeed, than the last so exceedingly characteristic disturbance, there are in nervous dyspepsia, in consequence of peristaltic intestinal unrest, diar- rhoeas. Such "nervous diarrhoeas" are often abruptly set up by an emotion, a fright, etc. Very frequent is distressing pressure to empty the bowels, which usually occurs at an inconvenient time (in the theater, for example). A by result of the intestinal atony, found particularly distressing by tin' patient, is, finally, flatulence; distention making itself apparent during the night, is often designated as the direct cause' of sleep disturbances. In spite of these manifold digestive disturbances the condition of nutrition of the patient in nervous dyspepsia does not usually sutler to an appreciable de- gree. There are found among them, indeed, people of blooming appearance to whom may be applicable the exclamation of the visitor of Beard's, who, after a look at his waiting-room filled with neurasthenics, cried out, "Your patients are, indeed, regular giants." It is otherwise, however, with the (in general, rare) severe eases of nervous dyspepsia, since here there may hi' extreme emaciation, indeed, real marasmus. In such cases the gastric troubles have taken on a chronic character,' no! remit ting in anv appreciable extent. Loss of appetite predominates. Along with the slight desire for nourishment the continued anxiety as to supposed dietary errors leads the patient to limit his hill of fare more and more, and to undertake actual fast cures in which In- is often not .aware of the insufficiency of his nutrition. In one of my patients, for example, who. although reduced 420 LECTURE XXVIII to a skeleton, affirmed that he abundantly nourished himself, it appeared from the investigation of his metabolism by Jaquet that he, in his hypochondriacal anxiety about abdominal distress, had actually reduced his ration to 1,850 calories a day (an average of 3 days). (As a comparison it may be recalled that in a woman lying in hysterical sleep, the daily minimum needed was reck- oned by Sonden and Tigerstedt at 1,680 calories, while Voit gives the normal food value for adults at moderate labor at about 2,750 calories.) The loss of strength in severe cases of nervous dyspepsia can be increased in that there is not only eructation and regurgitation, as in the slighter forms, but there may be actual vomiting. Since, further, the feeling of pressure in the stomach sometimes increases to more or less severe pain, the differential diagnosis of such cases from ulcer or carcinoma of the stomach is often only possible after long observation with the aid of Roentgenological, chemical and micro- scopical examination of the stomach motility and stomach contents. SEXUAL NEURASTHENIA To the preponderant role which sexuality plays in the life of the individual and its manifold relationships to the most varied expressions of the psyche, corresponds the great extent of those forms of neurasthenia in which the whole disease picture revolves about disturbances of the sexual function. If the symptoms here are exclusively those to which organic basis is to be denied, nevertheless their appearance in material diseases of the male or female sexual organs (in the sense of a simple combination) is a very frequent occurrence. This is explainable, as we will assume in advance of our discussion of the etiology and pathogenesis of neurasthenia, in unconstrained manner from the fact that diseases of the genital organs, particularly venereal affections, to a special degree favor hypochondriacal self-observation and brooding. When discussing the treatment of neurasthenia I will shortly impress upon you the necessity of educating the patients to look upon their troubles with as much equanimity as possible, even to disregard them. Allow me to warn you against too close inquiry as to sexual anomalies which may furnish still more encour- agement to reflection on things which without this already dominate too much the thoughts of the patient. Here the necessity of finding out the cause, with the conscientious physician, must give way to the principle "non nocere" (do no harm). For the rest, the patients almost always acquire such confidence in a tactful, reserved medical adviser that they spread out before him voluntarily a sufficiently detailed sketch of the disturbances in question. These last can be quite satisfactorily arranged under Beard's definition of "irritable weak- ness." Indeed, abnormal sexual irritability and exhaustability can sometimes be differentiated as a first and second disease stage; more frequently, how- ever, symptoms of both categories occur actually along with one another. In our consideration we will for clearness hold fast to this division. The increased irritability first manifests itself in that the sexuality makes itself evident in a manner inadequate to the constitution of the patient. Erotic thoughts press upon ,the psyche in such predominant fashion as can often THE PSYCHOXEUROSES 421 find no satisfaction within the sphere of the attainable, and develop into sexual phantasies and waking dreams. Upon the same basis arise, also, the most varied sexual perversions. As physical expressions of the hyperaphrodisia are to be mentioned, in men excessive and continued erections which, particu- larly at night, reach distressing intensity and may lead to an agrypnia which, on account of the ever-present danger of masturbation, is particularly exhaust- ing. Still more serious than this last is psychic "onanism," orgasm intention- ally provoked by means of erotic phantasies. To be mentioned further are the excessively frequent (sometimes, indeed, nightly or several times in a night) nocturnal pollutions. If these are to be regarded as a morbid manifestation, when they occur in periods of sexual abstinence, only from excessive fre- quency and from the psychic and physical depression which follows them, diurnal pollutions are to be considered absolutely and without exception as a pathological occurrence. They occur, nevertheless, only in the most severe forms of sexual neurasthenia. From the increased sexual irritability, the carrying out of the sexual act is usually interfered with, often impossible; namely there is premature ejaculation. x\s irritative symptoms in sexual neurasthenia in females are to be mentioned: vaginismus (intercourse pre- vented), pruritus of the genitals (a starting-point for masturbatory practices) and pollutions, discharges of mucus from the glands about the vaginal en- trance (Bartholin's glands, etc.) occurring with orgasm, in half-sleeping con- ditions. As a symptom of weakness is to be mentioned the great exhaustion which even with active sexual desire follows coitus as well as pollutions. Sexual weakness in the male occurs most markedly, however, in the form of impotence which here, in contradistinction to the organic form (about which we learned in connection with tabes, for example), is to be denominated psychic. If here, insufficient response of the nerves of the sexual organs to erotic stimuli is often at fault (anaphrodisia), as a rule, the chief blame is to be ascribed to fear of failure, lack of self-confidence, etc., for this chief complaint of male sexual neurasthenics. Still higher grades of sexual weakness are characterized by the substitu- tion for pollutions of the discharge of semen while awake and without orgasm. This spermatorrhoea occurs upon urination and defecation (micturition and defecation spermatorrhoea). In women sexual weakness manifests itself ill absolute frigidity. The near relationships between the genital and the uropoetic apparatus, renders it easily understandable thai the majority of sexual neurasthenics also complain of urinary troubles, namely of desire to urinate at inconvenient times. pollakiuria, dysuria and strangury. Particularly characteristic is the symptom which has been called '•urinary stuttering." Tin patienl during micturition must repeatedly stop, and so empties his bladder in small quantities at a time. Finally, without exception, genera] neurasthenic symptoms manifest them selves in disturbing fashion, often favored l>\ the fear of threatened disease of the spinal cord. The belief on the part of the public that sexual neiiras- thenia is the precursor of "consumption of the spinal cord" ( tabes) maintains itself with ineradicable obstinacy, since it is intentionally supported by the 422 LECTURE XXVIII quacks and charlatans. The patients show a more or less severe depression, occasionally increasing to suicidal impulsion, are misanthropic, unable to ap- ply themselves to any work, lose all interest, complain of pain in the back, headache, etc. Pathogenesis and Etiology Since now we have obtained a sufficient insight into the semiology of neu- rasthenic conditions, having used as examples the ordinary acquired neuras- thenia as well as two of its varieties practically important, nervous dyspepsia and sexual neurasthenia, we must enter more fully into the question of the nature and the occurrence of such and similar symptom-complexes. Here I would refer to my remarks already made in Lecture XXVII, on the question as to whether the psychoneuroses are to be counted among the heredi- tary or the acquired diseases. We saw there, that between the endogenic and the exogenic factors forming the basis of the individual case there exists a reciprocal relation, so that on the one hand under the influence of powerful external factors, even a normally constituted nervous system is affected, on the other, a neuropathic disposition brought into the world can lead to the develop- ment of pathological conditions even from the ordinary stresses of life. For proper estimation of the individual neurasthenic disease picture, it is now of prime importance to take into consideration whether an endogenic or degenerative factor is present or absent. It is not the least service of Charcot that he first perceived the necessity for separating what he called true, and what hereditary, neurasthenia. In order to assign to both etiological varieties the same rank within the limits of Beard's disease, indeed, we had better strike out the epithet "true" and use for it "acquired" or "accidental" neurasthenia, in contradistinction to "congenital" or "constitutional neurasthenia." That these two varieties prognostically are to be estimated quite dissimilarly, and therapeutically are to be managed in an entirely different way, does not need to be specially emphasized. That, however, this principle of classification is a correct one, appears from the circumstances that in their symptomatology, also, as you will see directly, the accidental and the constitutional cases show differences, whose importance leaves far behind all that formerly justified the, attempt at a topographico-symptoniatological classification (see above, page 418). Let us turn next to the etiology of the accidental form, which includes al- ways recognizable and well-characterized influences, often affects, acutely, a previously entirely normal organism, and in which neuropathic heredity plays no role worth speaking of (see page 407). When we ask the patient himself what causes he holds responsible for his nervosity, we will hear him complain either of strokes of fate suffered, of agitations, distress and care, mental over- exertion, business troubles, — he accuses himself of sexual excesses, or finally, he dates his nervous weakness back to a physical disease of acute or chronic course, from which he has suffered. When we now search through this rather varied list of causal factors, after a common criterion, it may be seductive to regard the factor of exhaustion usually as such. So Mbbius would consider acquired neurasthenia as a variety of chronic exhaustion, its individual symp- THE PSYCHOXEUROSES 423 toms as potentiated exaggerated symptoms of the physiological fatigue process, developed into a permanent condition. This pathogenetic conception, however, does decided violence to the facts and hence, has even in neurological circles, been able to establish no support. Of course, there is a chronic fatigue, an exhaustion become a permanent condition ("defatigatio") ; we observe it after long lying in bed with fevers, in the undernourished, among the women of the proletariat affected by hard work and numerous labors, after exhausting ma- neuvers, after excessive feats of strength in various sports, but we miss in this the most important physical and psychic stigmata of Beard's psychoneurosis, and the picture of simple fatigue is rather that of a very general relaxation and prostration, which usually disappears leaving no symptoms, upon rest and full nourishment, without medical aid. For the development of an acquired neurasthenia, something more is needed than for that of a chronic exhaustion, not the excess of stimuli that press upon the nervous system, but their qualitative nature gives the result its marked emotional coloring. An example: A business man attributes his severe neurasthenia to the heaping up of the duties of his calling; and still he admits that some years back he passed over a period of much more severe, even excessive exertion, almost like child's play. The explanation is furnished us by the fact that he at that time was in a position of less responsihility, but in the meantime had moved up to a post of heavy responsibility. The robust nervous system over which the affect-free exertion passed without trace, gave way under that marked by strong affect. Also in the wide-spread "examination neurasthenia" the anxious tension, not the forced work, is chiefly to be blamed. Now there are indeed persons enough who under such circumstances do not fall victims to neurasthenia, and then' is no doubt that certain "tempera- ments" are more exposed to the acquisition of a neurasthenia than others. The person conscientious from the start, strict with himself, may be considered as predisposed; the one of sentimental nature and soft heart, not less so; none more so than the ambitious person whose mental work can never develop with- out affect, but through stimulated expectation, or painful undeception ex- periences a continuous emotional coloring. So we understand also, why neurasthenia grew first in North America to be a disease of the masses; in the last decades, however, it has overflown into the civilized countries of the Old World: parallel with the haste and unrest which modern methods of communication and the acquisitions of technology in all branches of human activity have liberated, in all nations the tendency to continually more complicated methods of life has developed. The movements of trade, the relations between 'production and consumption, have become eon tinuallv more complicated, so that to speak with the national economist Biicher, '•The existence and work of every individual is interlaced more and more with the existence and work of many others.'* In all departments of business life, we gee more and more dependence upon numerous unaccountable factors enter, a feeling of uncertainty which is continually increased by tin- constantly more acute competition. Along with these specifically increased cares of occupa- tion, those conducting different enterprises hear more and more the burden ol 424 LECTURE XXVIII crushing responsibility. The measure of responsibility has, however, also in- creased for the subordinates in industrial and commercial enterprises. The middle class is engaged in a hard and desperate struggle upon two fronts. The psyche of the working man is in continued tension through the sharpening of the social contrasts, through class struggle, through the danger to life of the mechanical occupations. Also for him the danger of crises is a continually threatening one; the hours of labor are shorter, but the work has become more intensive and intellectual. In short, if the "American nervousness*' of Beard has also mastered modern Europe, this has its basis in those phenomena which have been designated as the "Americanization" of our social conditions. Also in the cases where, following bodily diseases, a symptomatolcgicallv typical neurasthenia (not as more frequently a simple exhaustion) is acquired, the affective factor is never absent. No wonder that among these diseases, just those which give occasion for distressing self-reproaches, or to anxious fears for the future, far excell all others in importance and frequency; namely, the venereal affections. Also in the sexual neurasthenia caused through excesses or errors, there manifest themselves, as the most important etiological agents, remorse, shame, but particularly the fear of evil results. That this fear is encouraged by quacks and medical persons fallen into devious ways, by bro- chures, prospectuses, etc., which have as their end to draw into their nets as many victims as possible, I have already emphasized. One of the most favored tricks of these people consists in representing such normal things as the oc- casional occurrence of pollution in sexual abstinence, and one testicle hanging lower than the other, as the consequences of youthful sins, and the precursors of incurable marasmus, and in suggesting in this manner to the readers of their literature, sexual neurasthenic troubles, for which then they assert that they possess the only cure. For the origin of severe dyspepsia sometimes improper medical treatment is at fault, as Dejcrine has forcibly emphasized quite re- cently. As a prophylactic, one should refrain in neurasthenics from bringing into action for slight digestive disturbances, which, however, are greatly ex- aggerated by the patient, the imposing apparatus of complicated bills of fare, frequent washing out of the stomach, water cures, etc., but should on the contrary seek to represent to the patient his troubles as unimportant and fleet- ing symptoms and should avoid overtreatment. Special importance in this era of social legislation has been acquired by "accident neurasthenia," which often in little satisfactory manner has been brought together with accident hysteria, the fright neuroses, commotion psy- choses, etc., as "traumatic neurosis." Accident neurasthenia develops from the abnormal mental condition into which the injured person falls as a con- sequence of the accident, especially when this has affected the "noble parts" (the head, spine, region of the heart). The fear of permanent injury to health or economically, especially about the effect upon his ability to work and earn money, puts the patient into a state of chronic emotional unrest which in spite of undisturbed recovery from the material results of the accident, may develop into the severest neurasthenia. A particularly injurious influence is exercised heri' by preoccupation with regard to the question of damages, which the compensation and accident insurance laws of the different civilized states cause THE PSYCHONEUROSES 425 the injured person. Fear that he will receive too little indemnity keeps him in continued tension : it more and more impresses itself upon his mind that high damages are due him, and there is anxiety lest this be cut down or withheld from him, either carelessly or malignantly, or that his troubles will be estimated too lightly. The system of paying a monthly allowance customary in Germany, for in- stance, greatly encourages the formation of such "avaricious concepts," and indeed the continued "struggle for pension'' furnishes ever fresh material for accident neurasthenia. Much more rational is our Swiss system of cash settle- ment, in which the injured person after being paid the sum awarded by agree- ment or through a decision of Court, is as greatly interested on the ground of health, as financially in regaining as soon as possible his full capacity to work. In what manner the estimation of what compensation should be paid to the traumatic neurasthenic is to be estimated, cannot be rigidly laid down. A first principle is, however, that a permanent impairment of working capacity must not be assumed upon the basis of a traumatic neurasthenia (naturally the most exact observation must have permitted the exclusion of symptoms organi- cally caused). As an expert, I take the stand that according to the severity of the neurasthenia present (as to which the objective symptoms should be decisive), it must be considered that the disabilities should be estimated as, likely to extend over from six months to at most three years. My after- histories, in agreement with those of Nageli and other observers, prove the correctness of this view. The closing portion of one of my reports, for example, reads as follows : "The cure of the troubles is dependent upon the earliest removal possible of the question of compensation. As long as tins is pending, in spite of all the efforts of the physician, the subject will be exposed to emotional dis- turbances which will furnish conditions favoring the continuation of the trau- matic neurosis present. If, however. Mr. X realize-, that the question of com- pensation is definitely settled, his troubles will gradually disappear anil he will regain his ability to work. "In consideration 1, of the kind and manner and of the intensity of the present troubles of the subject (vertigo, headache, hypochondriacal depres- sion); '2, of his occupation as a carpenter ( which requires frequent bending over and in which vertigo is very disturbing) ; and .'5, of experiences acquired in similar cases, I would recommend to the Court a compensation upon the basis of the assumption of a diminution of earning capacity from •_'•"> to 33 per cent, (luring 2 years. Though the reduction of working capacity is greater at present, it is nevertheless to he assumed that after definite adjudi cation, this will diminish progressively and at latest will have disappeared at the end of the second year." Quite otherwise than in the acquired forms are the etiologico-pathogenetic relationships in constitutional neurasthenias. Tin- degenerative factor mani feats itself equally from the anamnesis and from the semiology, now in unmis takable intensity, now in more obscure forms, sometimes indeed, only as a sug- gestion. For neuropathic heredity, in contradistinction to the acquired case., we seldom seek in vain, also it i. usually furnished by numerous and seven 426 LECTURE XXVIII forms of disease in the ascendants or in the collateral branches of the family. These disease forms belong only in the minority of the cases, under the division of similar heredity. More frequently (and in the more severe cases) there are in the parents, brothers and sisters, not neurasthenic symptom-complexes, but more severe neuropathies, hysteria, psychoses, epilepsy, so that the "de- generative neurasthenic" in contradistinction to the victims of a reinforced pathological heredity (see Lecture VII, page 197) often appears to us as the relatively least injured member of an in general much more severely degenerated family. Often, also, separated still further from homomorphous heredity, we find common among the progenitors, affections injurious to the germ in general, as alcoholism, syphilis, tuberculosis, more rarely diabetes, perhaps also the uric acid diathesis, if we follow the statements of French and English authors, since with us the rarity of true gout does not permit the formation of a definite opinion in this direction. Finally, consanguinity of the parents is to be considered here. There are now, however, also neurasthenias of the most severe degenerative character in which we can find no hereditary factor. In general we must ex- pect to form a decisive conclusion as to the constitutional factors from the study of the patient himself. And hence we will take up now the previously unconsidered clinical peculiarities of these degenerative cases. CONSTITUTIONAL NEURASTHENIAS The three chief criteria of the cases falling under this head are: 1, the occasional presence of somatic stigmata of degeneration; 2, beginning of the disease manifestations in early or earliest childhood ; 3, certain anomalies to be considered as psychic stigmata of degeneration. The first point we have considered at length in our introduction to psy- choneuroses (Lecture XXVII), and only need to refer to it to-day to refresh your memory. Only it must be expressly emphasized that such morphological anomalies, which are entirely foreign to acquired neurasthenia, are not very frequent also in the constitutional form. Much more frequently, however, in any event in the majority of the cases, there is a history of the so-called congenital neurasthenia or that of child- hood. We find that already in school, or even before this, in the nursery, the patient is characterized by abnormal irritability, motiveless outbursts of anger; that he now misanthropically and discontentedly isolates himself from contemporaries, again manifests a restless motor impulsion, that he suffers from stuttering, or for years, often until puberty, from nocturnal enuresis ; that he has masturbated long before the age of puberty. Important, also, is the history that the patient as a child suffered from night terrors, those pe- culiar attacks of frightened waking out of sleep with momentary confusion and loud screaming, which even then, when they are referred to nasal polypi, adenoid vegetations of the pharynx or to intestinal worms, indicate an ab- normal reflex excitability of the brain. Milder fright conditions are more frequent; fear of darkness, of the whistle of a locomotive, of being alone: THE PSYCHONEUROSES 427 further peculiar habits, eating paper, forming senseless words, crying out on certain occasions, grimacing during serious situations, etc. Over these manifold expressions, which it is often not at all easy to dis- cover anamnestically, the psychic stigmata which so often as phobias and im- perative conceptions dominate the clinical picture of constitutional neuras- thenia in adults, cast their shadow. As best known and perhaps most frequent paradigm of the phobias, West- phal's "agoraphobia" (fear of open spaces) is to be mentioned. This is an overpowering feeling of anxiety which always affects the patient when he has to traverse an open space. A feeling of anxiety, which expresses itself in the countenance, is accompanied by pallor and palpitation of the heart, the break- ing out of a cold sweat, and the arrest of the salivary secretion. A feeling of anxiety in which the person affected has the sensation that he is about to sink through the ground, that his legs are paralyzed. A feeling of anxiety that in severe cases actually compels the individual to turn about, to creep along the houses, or to solicit the attendance of a passer-by, although he is entirely aware of the lack of motive and absurdity of his phobia. Endless is the list of other phobias which we meet as a frequent or occasional occurrence, in the picture of constitutional neurasthenia, and to which an endless number of foreign words have been applied; the gephyrophobia already described in the works of Hippocrates, that is the fear of passing over a bridge; siderodromo- phobia, the fear of traveling on a railroad train; claustrophobia, the fear of being pent up in a narrow space. Excessively variable are the special mani- festations of the so-called "situation anxiety," which, for example, affects the barber when he is about to shave, the preacher when he is about to mount the pulpit, etc. ; all manifestations which are strictly separable from the vague anxiety of the accidental neurasthenic. Closely related to the conditions of anxiety are imperative conceptions (obsessions), from which the constitutional neurasthenics so often suffer. "Conceptions which do not arise by way of associations," so Oppcnheim de- fines them, "but appear spontaneously and forcibly press into the circle of ideas, so that they cannot be banished from them although the individual con- siders them something foreign, not belonging to the mental ego." The most frequent paradigm of these disturbances is the "Folic du doute" ("doubting mania") studied by Griesinger, Legrand da Saulle, and Faint long before Beard. This consists in questions which continually and without motive press upon the mind, as "Why have I said this and not that?" or doubts which cause even the mosi indifferent acts to take on a painful character and are often com- bined with anxious concepts, ''Would it lie better to lake this journey or not?" ".May I not be the victim of a railroad accident?" or "Maj mil some body break into the house (hiring my absence?" "Did I really put a stamp on the letter just mailed?" "Have I not forgotten to shut tin- door of my house 1 ** "Have I not forgotten to turn out the gas?" Such doubts appear compul BOrily upon the most indifferent occasions (they can occur as episodes even within physiological limits) and are, besides, firsi disproportionately strong, and, second, so obstinate that they do not allow the patient to rest, although 428 LEGTURE XXVIII (and partly because) there is complete insight for the senselessness and patho- logical character of these manifestations, a phenomenon which is easily differ- entiated from the general lack of decision of the accidentally neurasthenic. More severe symptoms are arithmomania, the compulsion to count the win- dows of a house, the lanterns in a street, the flowers in the pattern of a carpet, onomatomania, the compulsion to call to mind certain forgotten proper names, the "deli re du toucher," the obsession that a defilement or infection must be connected with every touching of a strange person or object, etc. As to the psychical mechanism of the peculiar phenomena of the phobias and obsessions, we can with good conscience repeat "Ignoramus," since the attempt of Sigmund Freud to explain imperative conceptions as in general symbolical from certain "displaced" psychic complexes of erotic nature can- not be brought into accord with the facts ; also I consider the sharp separation between an anxiety neurosis and a compulsion neurosis undertaken by him as impracticable. Much more does it recommend itself to denominate both forms with their transitions just as frequently found, with Janet and Raymond, as "Psychas- thenia." Now "psychic powerlessness" (and this is indeed caused by the giv- ing way without resistance under hyper-quantivalent ideas in spite of com- plete insight), impresses a characteristic stamp upon the whole clinical picture. This does not indeed indicate that somatic signs of disease are absent — still they play a much smaller role than in acquired neurasthenia. It seems to me deserving of emphasis that pressure in the head and sleeplessness never reach a very high degree, indeed, can be entirely absent. Among the objective symp- toms which we mentioned for the acquired form, only the increase of reflexes and the cardio-vasculur lability usually manifest themselves with marked in- tensity, sometimes also vibrating tremor. Of other somatic phenomena the not altogether rare occurrence of "tics" (see Lecture IV, pages 67 and 72), indeed deserves mention. The conceptions psychasthenia and constitutional neurasthenia would agree with one another if it were not that a considerable contingent of cases which in their history prove themselves certainly constitutional, are entirely wanting in the phobic and obsessive element, and approach the picture of accidental neurasthenia. Indeed, they then usually bear a chronic character, the time of their development cannot be precisely determined and they have a less stormy symptomatology. In spite of this, just these cases are to be con- sidered much more serious and therapeutically much less infruenceable — which of course applies also to the psychasthenias. Diffcren Hal Diagnosis Now before we pass over to the so important treatment of neurasthenic conditions, it must not be neglected to point out the, unfortunately all too fre- quent, serious diagnostic and practical misconceptions which arise from the extension of the conception neurasthenia, to conditions which have with it nothing in common but a more or less extended semiological analogy. We should hold to the rule, only to make the diagnosis neurasthenia, by exclusion. THE PSYCHONEUROSES 429 At first it must be considered that incipient phthisis, diabetes mellitus, chronic alcoholism, nicotinism, morphinism and cocainism may produce similar symp- toms. The same remark applies to certain organic brain diseases whose "pseudo-neurasthenic" initial stage we have already described at length : de- mentia paralytica, and cerebral arteriosclerosis. Dementia praecox should also be thought of here : indeed, for example, it has been shown by Mile. Pascal that of 75 patients sent to the Insane Asylum, Ville-Evrard, with dementia praecox (of both the simple as well of the paranoid, hebephrenic and catatonic varie- ties) not less than 32 had first been wrongly diagnosed and treated for neuras- thenia, an error which may have serious results for the patient, his family and society. Against such serious errors only careful psychological investi- gation into the mental condition of the patient will protect us. It will be noticed that in the neurastheniform prodromal stage of dementia praecox — in contradistinction to true neurasthenia — emotional indifference is the basal element of the psychic syndrome, — the hypochondriac ideas are characterized liv their variability and their trifling character, concern themselves prepon- derantly about the physical condition of the health and are emitted with little affective accompaniment (emotional dulling). Notice also, peculiarities, odd demeanor, motiveless laughing; negativism in the form of systematic opposi- tion, and a hard-headedness alternating with childish docility and suggestibil- ity; "psychographic disturbances," an excess of pretentious word-forms used b}' preference, bombastic and inflated style further appear. Quite as frequently the confusion of neurasthenia with cyclothymia, that is, witli the "formes frustes" of circular melancholia occurs. This happens in patients usually of the female sex, who at more or less regular periods — for example, every Spring or every Fall, become relaxed, without energy, and inclined to tears, complain of dyspeptic troubles, pressure in the head and similar things, and usually suffer from marked agrypnia. As definite indications of their relationship to melan- cholia, besides the periodicity to be mentioned are, the regular occurrence of self-reproach and depressive ideas ("I cannot preside over my house, am a burden to my family! Oh! what a lazy ami unenergetic person I am!" etc.), and that in the great majority of cases there is an alternation of morning exacerbations and evening remissions. That the formes frustes of Basedow's disease usually sail under the colors of neurasthenia cannot be denied; also one must he on his guard againsl confusing this lasl with beginning Addison's disease. Treatment The interest which of late has been specially directed to the |is ychoneiiroses in general and to neurasthenia in particular, has fortunately redounded also to the advantage of the therapeutic side of the problem. Proceeding to discuss these matters I shall chiefly keep in mind those methods which can be carried out outside of a hospital or sanitarium; since mosl of you will meet your neurasthenics in private practice. In every rational treatment of neurasthenic conditions the Leading posi tion must be assigned to those measures for which the word "psychotherapy" has been coined. That this expression has become quite a commonplace, must 430 LECTURE XXVIII be admitted. Nevertheless, it has one great advantage in that it shows us in striking manner, how great a change our therapeutic measures for psycho- neurotic affections have experienced of late. Here the service of the path- finding work of Paul Dubois cannot be enough emphasized. Although even before him, consciously or unconsciously, every really good physician practiced mental treatment, and Ottomar liosenbach is to be considered as his prede- cessor in this method of cure (the so-called "dialectic psychotherapy"), it is only through the efforts of Dubois that the recognition of how predominant a position is to be attributed to psychic influences in the circle of therapeutic agents has become generally realized, and how astonishing results it is per- mitted to obtain by its systematic application. Further, he has made it clear to us that psychotherapy has its technique and its rules, like all other methods, and that to be applied intelligently and systematically it presumes in the physician a certain makeup besides practice and experience. These require- ments can never be replaced by theoretical study, also you should not expect from me more than some suggestions and indications toward developing your own psychotherapeutic system. According to the personality of the thera- peutist his methods will have to bring into play the factors of educational influence, explanation of facts and authoritative intervention in quite varied combinations, to estimate whose relative value would be a sterile pedantry. Rather are they to be judged by the effects produced. That, however, the personality of the patient must have a decisive influence upon the choice of the psychotherapeutic method, is self-apparent; every word that the physician addresses to a neurasthenic exercises upon him a psychic action, and hence must be adapted to his peculiarities. So, there are individuals who are entirely competent to realize that they must suffer throughout life from certain troubles, if it is only permitted to the physician to convince them that these symptoms do not place in question either their lives or their working capacity ; other neurasthenics, on the con- trary, cannot bring themselves into such a condition of stoicism, and by the (direct communication of such a prognosis are reduced to despair and driven deeper into their psychoneurosis. It is not given to every one to react to the appeal to duty, altruistic feeling, etc., in the sense of correcting the egocentric view of the world peculiar to many neurasthenics. Occasionally we appeal directly to self-love — so doing must of course be carefully considered in ad- vance — "If you do not carry out my recommendations and put aside the in- jurious factors pointed out to you, you are liable to become still w T orse." In short, a great measure of diplomatic skill is necessary, in properly estimating how the patient is best to be readied, corresponding to his psychological in- dividuality. This insight into the nature of the patient we must endeavor to obtain, particularly from the manner in which he describes his troubles to us. Since this is usually done with extraordinary detail and verbosity, we have, as a rule, only too much time to become acquainted with the mentality of the individual. Naturally, as I have already emphasized in the last lecture, it must be our endeavor not to allow the history to be extended indefinitely, since we cannot devote all our time to a single patient. But we need not cut off a neurasthenic by saying to him, "What you wish to tell me further is unim- THE PSYCHONEUROSES 431 portant ; I know enough," since by so doing we would irreparably lose his con- fidence. On this account one must learn, by skillful introduction of questions, to lead the patient away from unimportant matters and to keep his descrip- tions within reasonable bounds. The neurasthenic must, however, gain the im- pression from the conduct of the physician while taking the history, that his complaints are sufficiently considered and are not taken "with a shrug of the shoulders." Also the physical examination must be careful and thorough, since in this way we procure a basis for convincing strength in the reassuring advice which is to be imparted to the patient. Removal of the hypochondriacal ideas is important above everything else. To this end I do not hesitate, in the case of intelligent and educated patients, to support my word by clinical proof and explain to them, in a simple but scientific way. why their heart troubles are to be considered functional and cannot be organic, in what way their abnormal sensations are produced; with the hemoglobinometer I demonstrate that the suspected anemia is not present; by reading selected paragraphs from Band, Dubois, etc., I show that phe- nomena which greatly frighten the patient do not represent anything but typical neurasthenic symptoms; that phobias and obsessions are not at all precursors of mental darkening, that many views spread through inferior sexual literature which hold the sexual neurasthenic under their ban, are fabulous, etc. A wide and thankful field for explanation and reassurance. In this short discussion of the nature and symptoms of neurasthenia, I would only advise you to carefully avoid one thing, the word "imagination," which almost always makes such patients restive, while "autosuggestion" can perhaps be risked. With explanation to the patient of the nature and genesis of his troubles, the matter is indeed finished in only very few cases. Usually teaching self- control, consideration of the subjective disease symptoms as a "negligible quantity," training into a rational method of occupation, correction of ex- aggerated sensitiveness to the annoyances of life, etc., requires quite high qualities and psychotherapeutic skill on the part of the physician. It is usually specially difficult to talk a psychically impotent person back into his lost self-confidence, by far most troublesome, however (unfortunately often aKo most thankless), to influence anxious and imperative conditions through logic. Here, as there, it is ill general to be recommended, at first to advise I lie patient as far as possible to avoid for a long time the "critical situations" (for example, cohabitation, crossing bridges, etc. Through this, the memory of former ill results and attacks of anxiety becomes gradually less marked, and the inhibitions which oppose themselves to rational psychotherapy are grad ually reduced. Further, a great number of phobias and obsessions prove them selves accessible to psychic influence only after there has been a change ol surroundings as radical as possible; hence, treatmenl in sanitariums and asy- lums comes frequently into question in such cases. For the rest, however, I certainly agree with Edinger, when he warns against being too ready to order institution treatment. The slighter cases of neurasthenia are really often better suited when the changes in surroundings are procured in a [ess radical manner, as in the form of a sojourn iii the country, or a sea voyage, provided that, while so doing, enough new impressions are furnished fin the uiiiiil to keep 432 LECTURE XXVIII it from the observation of self. Even the prescription of such a cure acts often encouragingly upon patients who had feared to be sent to a sanitarium. A disadvantage of a sanitarium is often that the neurasthenic patients mutually lament about their troubles to one another, which hardly acts in the sense of an "abstraction." In neurasthenic conditions shall psychotherapy push en- tirely into the background other methods of treatment (formerly incorrectly occupying the foreground), or has each of these its justification along with the other? In this question I would occupy an eclectic stand-point and would epitomize the matter as follows : That the psychic abnormalities of the neurasthenic cannot be directly in- fiuenced by dietetic rules or by prescriptions is self-evident, and here psycho- therapy in the direction which was sketched above has uncontested results. Nevertheless, in the state of mind of the accidental neurasthenic the primary is not always easily to be differentiated from the secondary, namely what is irritable weakness of the brain functions from what is the natural result of a frequently quite painful feeling of general loss of strength anel prostration. The last, however, can be influenced by somatic measures of treatment. To the subjective disturbances in whose estimation the seconelary ideogenic element is difficult to differentiate from the results of primary injury, belong headache and pain in the back, loss of sleep, loss of appetite, vertigo, etc. Here, also, psychotherapy belongs in the foreground; as further curative factors, how- ever, physical and dietetic methods can be applied, and properly applied, they give excellent results. Only in the second place, however, should treatment of these subjective disturbances by drugs come into application, since in no other instance is there greater danger of the establishment by the super- ficial therapeutist of a '"therapy of little symptoms." The following principles should prevail: "Limitation of the indications as much as possible — the even- tual application of drugs is permissible only as a therapeutic compromise and by reason of opportunity, and in any case as rarely and as temporarily as possible." This applies namely, to the different "headache remedies" from the group of the antipyretics and for hypnotics proper. The better one understands how to carry on the general treatment of neurasthenia, the more rarely will he have to resort to remedies of this class, if only temporarily, it should always be considered as a forced position. Not only does the objection to this convenient method of treatment with sympto- matic drugs rest upon the danger of habit formation, but also in that it directly opposes a rational psychotherapy. Everything is in bringing the patient primarily to the conviction that all his individual complaints are not diseases in themselves, but represent the outcome of a unique abnormalization of the whole nervous system. If, however, we force these by too active thera- peutic consideration into the foreground of the treatment, we make him doubt- ful as to our assurances that his "everlasting pain and misery so thousand- fold" is to be cured from one point. It is somewhat different, however, with the manifold somatic visceral disturbances, which we have enumerated as ob- jective symptoms of neurasthenia. These disease manifestations (for ex- ample, abnormal variability of the pulse and of the blood pressure, factitious urticaria, hvperidrosis, fine tremor, tachycardia, hyperacidity, etc.) do not THE PSYCHONEUROSES 433 permit themselves to be absolutely subjected to a primordial mental alteration as in hysteria, in which the symptoms manifesting themselves in the territory of somatic or sympathetic n,erves are brought to pass in a roundabout way through the conception. Rather is it here plainly the coordination of irritable weakness both in the psychic and in the physical sphere, so that in neurasthenia we can recognize a general neurosis in the fullest sense of the word. The somatic visceral disturbances of neurasthenia have hence a claim to a treat- ment to a certain degree autonomous, to be begun along with psvchotherapy and based upon a rational empirical basis. The disappearance of these symp- toms will be an indication of its efficacy, that is, a reduction of the reflex and mechanical irritability, and return to normal secretory conditions, the quieting of the heart, etc., and since we can accomplish these actually by physical, dietetic and medicinal agents, I cannot decline these methods. Presuming that with the removal of the causal factors, an accidental neurasthenia can be cured, a constitutional, decidedly improved, by psychotherapy alone, nevertheless, in my experience our task is greatly lightened in most cases by a combined pro- cedure. No drug is so often given to the neurasthenic as the alkaline bromides ; to many physicians inclined to routine, ordering this drug follows almost as a matter of course the diagnosis neurasthenia. There is, however, no point in administering bromides to relaxed neurasthenics without any marked symptoms of excitation. At most a short course of bromide (with daily doses up to about 3 grins of KBr) can be occasionally recommended for such patients in whom irritative symptoms dominate the clinical picture, while weakness occu- pies the background; for example, in threatened predominance of conditions of anxiety, in disturbance of the always labile psychic equilibrium from some annoyance small or great. Very dilute solutions are best borne (10 grammes (3iiss.) of the salt in 150 cc (§v) of aq. menth. pip., of which 8 tablespoonful can be taken in a glass of water). If to this mixture 0.3 to O.-i (gr iv to vi) codein phosphate is added, the sedative action is increased. A further quite useful sedative is Indian hemp, in the prolonged adminis- tration of which very small doses suffice. It can also very well be combined with tonic remedies; for example, 1 have recommended the following combination under the name of pilulffi cannahin;e composite: I J Quinini sulphat 1.0 (gr. xv) Acid, arseniosi 0.00 — 0.1 (gr. %o to 1/4) Extract, cannabis ind 0.45 (gr. <> :, i) Extr. et pulv. rad. valerian q. s. ut f. pil. xxx. S. One pill every evening. A course of arsenic is sometimes above everything a curative factor in thin, chronically fatigued neurasthenics having little appetite. Large doses are practically never desirable; as a rule, in long administration a daily dose of ().()()•_' to 0.003 (gr ':■.,, to Yzo) of arsenious acid or 0.2 to 0.8 ( nyiii to ITRv) of Fowler's solution, is sufficient. The last, combined with tine, nucis vom., is usually well borne. .More convenient are the Asiastic pills, of which one can 434 LECTURE XXVIII be given daily after the evening meal (Acid, arsenics, 0.2; pip. nigri, 5.0; sach. alb., rad. alth. aa. .3.0, M. f. pil. No. C). Where exceptionally a very sensitive stomach refuses the arsenic preparations, I administer them sub- cutaneously in the form of sodium cacodylate in (sterile ampoules of 0.05). Where there is definite anemia, iron is called for. Very popular are Erb's ''Tonic Pills": Ferr. lactat., extract, cinchon., aquos, aa 4.0 (3i). Extract. nucis. vom., 1.0 (gr. xv) ; extract, gentian, q. s. ut f. pil. No. C. S. 2 pills t. i. d. after meals. Where iron does not appear indicated, cinchona and nux vomica are ordered in the form of drops (tinct. nuc. vom., 5.0; tinct. cinchon. comp., 10.0; M. S. 30 drops t. i. d. before meals, in water). This prescription is useful in in- testinal atony and other dyspeptic troubles. A drug in which tonic and sedative properties are united is valerian, an indication for which is furnished particularly by the cardio-vascular disturb- ances. A systematic course of the infusion, prepared in the cold, has proved most efficient. Directions for its use will be found under the treatment of Basedow's disease (see Lecture XXIII, page 353). Where the valerian tea is refused, the extract can be substituted for it, for example: Ty . Extract. valerian. 10.0 (oiiss) ; extract, hyoscyam., zinc. oxid. pur., aii 5.0 (gr lxxv). M. Fiat Pil. No. C. S. 1 pill three or four times a day. Phosphoric medica- tion, when it is indicated, namely in thin but full blooded neurasthenics, is to be carried out as recommended under Basedow's disease (under treatment with sodium phosphate, or calcium glycero-phosphate, there is often satisfactory gain in weight and improvement in the general condition), further, in neuroses of the stomach and, finally, in nervous irritative conditions of the sense organs, particularly in tinnitus aurium. Also with regard to one further point we can refer to what was said under Basedow's disease in Lecture XXIII. The ovo-lacto-vegetarian regime can act exceedingly favorably in neurasthenics with marked circulatory lability; quite as satisfactory is sometimes the effect of such change of diet in nervous dyspepsia, in which the gastric anomalies of secretion are favorably influenced by the relative lack of irritation of the mild meatless, or meat-poor diet. Finally, this is the most harmless method of combating the chronic intestinal sluggishness so frequent in nervous people, particularly when an increase in the content of cellulose is provided for by the free use of fruit, Graham bread, etc., and in obstinate cases, % liter of warm physiological salt solution is given in the morning on an empty stomach. The intestinal fermentation and the distention by which the atonic intestine of the neurasthenic is so often troubled, are decidedly reduced. Where there is anemia, naturally in the regula- tion of diet its content in iron should be taken into consideration (spinach, carrots, yolk of egg, oats, asparagus, strawberries, Graham bread). In cer- tain cases, particularly in cardio-vasomotor neurasthenia, alcohol is best entirely forbidden ; under all conditions, however, it is only to be permitted in small quantities and well diluted. Strong coffee is to be forbidden, at most, that well diluted with milk is permitted. Tea appears to. be less injurious, but also is to be permitted only in small amounts. With regard to tobacco, it is to be forbidden in the cardiac and vasomotor forms of neurasthenia; otherwise, however, we THE PSYCHONEUROSES 435 should, as a rule, be satisfied with restricting its use within moderate bounds. Indeed, we should consider the agreeable feeling which it procures for the pa- tient as an ally, from a psychotherapeutic standpoint, since it raises his spirits. A simple dietetic prescription which well suits most patients with sense of pressure in the head and dizziness, is to allow them to take some slight refreshment every hour in the day so that the stomach is almost never quite empty.* It is astonishing how rapidly almost every one becomes accus- tomed to this regime and how advantageous it is found. In cases in which the head troubles mentioned above, together with weakness and prostration, make themselves apparent immediately after rising, breakfast had better be taken in bed about one hour before getting up. This simple dietary measure can be denominated "fractional overnutrition" ; in ambulatory treatment and in slight cases, it can accomplish what is sought in severe cases by the "Weir Mitchell rest cure," which in any case can only rarely be carried out at home, and for which in general a sojourn in a hospital is necessary. f The marked taking on of flesh, which is attained in such cures by the gradual training to continually more frequent and continually more nutritious meals and aided by the large amount of physical rest, often acts as a marked sedative to the whole nervous system. A certain comfortable laziness gradually comes over those subjected to this "cure," and the proverb "Plenus venter non studet libenter" gradually finds application in the matter of constantly studying himself and his woes by such a patient. Besides this, the isolation with which the Weir Mitchell cure is united, gradually prepares the way fur psychotherapy. The physical curative measures which may he of value to the neurasthenic can be only briefly mentioned. With warm full baths and half-baths, air- baths, hot foot-baths, alternating foot-baths and cool spongings, we can succeed win rever baths are found advantageous and we do not need complicated hvdro- therapeutic apparatus. The foot-baths are particularly appropriate in com- bating the congestions and tinnitus aurium, the full-baths and half-baths for quieting the patient at night, the rubbings and the air-baths Cor general tonic treatment. With cold-Water procedures ami sun baths for which tile laity have such a great fondness we are more likely to do harm than to aid. Also the wide-spread idea that the practice of different athletic sports is a panacea for nervosity must he decidedly opposed. It is well to lay down for the pa- tients, who seek recovery in the country, rational directions with regard to walks, periods of rest, reclining in the open air, etc., as otherwise we risk hav- ing them comi hack in a seriously exhausted condition. Finally, I would recom- mend to von to take to In-art, the reminder of Heard: "Every ease of neuras- thenia is a study in itself; no two eases are just alike If two eases arc treated alike from start to finish it is probable thai one of them has hern wi treated." * For example, milk, cakes, figs, prunes, chocolate tabli I . 1 te. . Doctor Mitchell always preferred carrying ou1 the "real cure" in special privuti away from the hospital. — Translator. LECTURE XXIX The Psychoneuroses C. Hysteria In contradistinction to neurasthenia, which, as we saw, has only been iso- lated nosologically and named in recent times, in its sister neurosis hysteria, we have to do with a very ancient disease conception. That the striking symp- toms of the disease were brought into connection with supposed disturbances of the functions of the uterus, has, as is known, found expression in the name for the affection ( baripa = the womb). Hippocrates regarded the hysterical phenomena to a certain extent as abstinence symptoms on the part of the organ withdrawn from its natural function ; the disease appeared most fre- quently in old maids or in women early widowed, to whom the advice to have relations with men as soon as possible must be given; "since if they become pregnant, they will be cured." Particularly fantastic is the pathogenetic con- ception attributed to Timaus, who imagined that the unsatisfied uterus wan- dered restlessly about, like a rutting animal ("animal liberorum procreandorum appetens"="an animal desirous of begetting children"), throughout the body, and in this way set up the hysterical symptoms. That this grotesque view has been retained to this day among the country people of our neighboring Alsace, deserves mention : we occasionally hear from a peasant woman troubled about her daughter, the expression "her womb is trying to get out of her throat." Further, you know indeed, that among the laity of all classes of society (naturally also in romance literature), to-day still, the conceptions "hysteri- cal" and "man-crazy" are nearly synonymous. No wonder then, that even from the chain of thought of physicians, the teachings of Hippocrates only disappeared in the course of the eighteenth century. For these the equally incorrect view that hysteria and hypochondria (this last term we must to-day replace by "neurasthenia") were the same disease was substituted; only the first was the special privilege of the female, the last, that of the male sex. It is not the least service of Charcot that he made clear that hysteria and neuras- thenia are different conditions and that each may affect both sexes. As to hysteria indeed, its great preponderance in the female sex was recognized by Charcot, and statistics of later authors give this as 85 to 90 per cent., but the determining factor for this predisposition of women is, as our later dis- cussion of the nature and causes of hysteria will show, not the female genital organs, but the female psyche, in which, in comparison to the male, a prepon- derance of phantasy and conceptional life, as well as diminution in the power of judgment and of critical inspection, are evident Also, men who become hysterical are throughout of the so-called "feminine natures" (which, as is 436 THE PSYCHOXEUROSES 437 understood, is meant only in the psychic sense and may be combined with the most virile physical make-up). In analogous manner the predisposition in childhood as well as that of certain peoples (South Europeans, Slavs, Jews) is to be explained. Only as a curiosity does the theory of W. A. Freund introduced not so long ago, according to which a causal connection between hysterical phe- nomena and gjmecological diseases — for example, contraction of the broad liga- ments — exists (in the sense of a reflex neurosis) need mention. This develop- ment of a one-sided specialistic view has unfortunately led to the removal of the pelvic organs in hysterical women, by which the psychoneurosis was natu- rally not extirpated. To-day, fortunately, the operative era in the treatment of hysteria can be considered as definitely closed. Symptomatology As we did in the case of neurasthenia, in hysteria we will first describe the different disease manifestations, mainly in their most important clinical fea- tures, only after this to enter upon the more difficult task of an etiological pathogenetic consideration. The symptomatological description of hysteria is made easier for us from the fact that a po'-tion of the symptoms is common to both psychoneuroses ; this concerns namely, such phenomena in which ex- aggerated self-observation plays a mediating role as we have sketched it in Lecture XXVII. Still, the intensity of these disturbances, corresponding to the incomparably greater auto-suggestibility of hysterics, is usually much more marked; and further from their increased auto-suggestibility there are also qualitative differences: since only upon a basis of hysteria, never upon that of neurasthenia, can paralyses, contractures and anesthesias (of sensible or sensory nature) occur. Special diagnostic importance is finally to be at- tached to the "hysterical character'' and to the special psychic disturbances of hysterics. These anomalies manifest, however, such intimate connections) with the nature of the disease that we will first study them along with the pathogenetic relations. To bring some order into the striking multiplicity of the hysterical phenomena, it is advisable to subject the permanent symptoms, (stigmata) the hysterical attacks, paralyses and contractures, to a separate description. 1. PERMANEXT SYMPTOMS ("HYSTERICAL STIGMATA") a. Sensible axi> Sknsoky Symptoms The majority of hysterics complain of pain of one sort or another. In- dividually very variable in localization, they usually differentiate themselves from pains as they occur in "hyper-algesic neurasthenia" through their greater intensity, or let us say rather, through the greater activity of the expressions of pain (complaining, crying, etc.). Particularly frequent are hysterica] pains in the hack, also hysterical headaches, which last, as a rule, are not described as pressure in the head only, but as a throbbing, burning or boring pain, which is felt by the majority of patients superficially, that is, in the scalp, and not 438 LECTURE XXIX inside the skull. Usually there is with this, marked hyperesthesia of the hairy region, pressure or even touching the scalp, a slight pulling on the hair, pro- duce lively expressions of pain ; percussion is described as "actual torture." Very frequently headache and hyperesthesia center themselves in circumscribed regions over the top of the skull (see Fig. 110) ; the simile of a nail driven into one of these areas, occasionally used by hysterical patients, has led to the denomination of this symptom "Clavus hystericus." There is also a hysterical pain in the face which is differentiated from neuralgic prosopalgia, above everything by the continuity and diffuse nature of the pain and in contradistinction to that, is very often bi- lateral. The greatest intensity of pain is usu- ally located in the temporal region ; in many cases the pain occurs day after day at some definite time — habit pains (Brissaud). Ana- logues of the clavus hystericus, that is cir- cumscribed sensitiveness to pressure, often also spontaneously painful areas, the so-called "topalgias," occur on certain portions of the trunk (Fig. 110) with a certain predilection. By the Charcot school so much industry was expended upon the study of these "mammary," "sternal," "ovarian" points, etc., that one can- not escape the impression that just this search- ing for hyperesthesia over these "classical" areas, then as to-day, frequently unintention- ally suggested to the patient the phenomena sought. Nevertheless, just the possibility of conjuring up in this fashion hyperesthesias and pains, is a clinically very useful symptom of hysteria; further, the examination of healthy persons shows that the apex of the skull, as well as the other areas of predilection for topal- gias often are somewhat more sensitive to pres- sure than the rest of the body surface, so that perhaps the physiologically caused local pre- disposition may also come into play. That the so-called "ovarian point" has nothing to do with the ovaries is shown by the fact that this symptom also occurs in male hysterics. Since occasionally by the production of pain on pressure over the spots of circumscribed hyperes- thesia, hysterical attacks may be set up, on the other hand, however, those already in progress may be cut short; such areas have been denominated "hysterogenic" and "hysterofrenatory" zones. Diagnostic difficulties are presented sometimes by hysterical arthralgias, as they may localize themselves in joints to which the attention of the pa- tient has been directed by some sort of material anomaly, for instance, genu Typical Localizations of Hysterical Topalgias (Shaded) and Anes- thesias (Black). THE PSYCHONEUROSES 439 valgum, crackling of the joint surfaces, etc. Often, indeed, the surgical or roentgenological findings are entirely normal, or the hysterical nature of the pains is clear from their localization at an anatomically indifferent point of the joint region. For the rest, however, the diagnosis can only be made after long observation. Of important indication is particularly the disappearance of joint pains when the attention is distracted — which also applies to the other hysterical "psychalgias." These last are, however, not always of such circumscribed nature as in the examples already mentioned. They can affect the whole body (pantalgia), or more rarely, one entire half of the body (hemialgia). When the patient on account of hysterical pains which occur or are increased by every movement, is condemned to the most absolute help- lessness, we speak with Mobius of "Akinesia algera"; where every touch of the skin calls forth pain, with Pitres, of "Haphalgcsia." Of greater diagnostic importance than pains and hyperesthesias are the hypesthesias and anesthesias of the hysterical. Indeed, as already emphasized, just through the occurrence of such disturbances which are foreign to neuras- thenia, hysteria assumes a special clinical position in the circle of psychoneurotic conditions. What I remarked when speaking of hysterogenic zones, about the sug- gestive production of hysterical symptoms by the physician applies here also and there is no doubt that hysterical anesthesias can be provoked by the examiner in the patient intentionally or not, that further, by repeated examina- tions (which hence are to be avoided as far as possible as soon as tin- hysteri- cal nature of the disease is certain) they usually increase in intensity and extent. On this account never test the sensibility in a patient suspected to be hysterical in the way of asking him, "Do you feel the prick equally plainly upon both sides?" or "Is there any place where you do not feel the prick?" This is as if you had in mind directing his suggestibility toward the test. If you wish to rule this out as far as possible I would advise you to avoid all questioning during the examination, since the patient (not before examined) usually spontaneously remarks: "In this location I have not felt the prick," or when upon application of a strong faradic current to the right hand he ex- presses pain, to the left, however, hi' remains passive^ then you know at least" that you have not exercised any foreign suggestion and only autosuggestion remains in question. That suggestion in genera] often plays the most important role in the production of hysterical anesthesias is proved by the fact that such disturb- ances of sensibility can be cured by suggestion, and indeed, may be changed topographically thereby (which is called transference). Why, however, the suggestibility of Hie hysteric manifests it-elf in circumscribed loss, or reduc lion of sensation (and besides this Iii quite characteristic distribution) remains a puzzle. While the generally diffused conception of motor paralysis permits us, in almost every hysterically paralyzed patient, the presumption that he has already seen paralyzed persons, or at least has heard of them, with regard to the anesthesias, similar relations are not to be assumed, hence observations of suddenly appearing hysterica] hemianesthesias as have been published by 440 LECTURE XXIX Stierlin, myself and other authors, in previously healthy individuals, as a result of severe fright (railroad accidents, earthquakes, etc.), compel us to assume that some organic regularities in the apparatus of apperception supply a ready-made pattern through which the psychogenic disturbance receives its clinical stamp. The hysterical disturbances of sensibility vary greatly in intensity from case to case and there are all possible transitions from slight hypesthesia to complete anesthesia. Still, in contradistinction to the anesthesias caused by organic nervous diseases, the paradoxical fact is noticeable that the movements of extremities which upon testing sensibility show themselves totally insensible, occur in entirely normal, coordinated and undisturbed fashion, that with an anesthetic hand the patient can write, play the piano, etc. Also the tendon reflexes of the region are usually not diminished, indeed, they are often in- creased as in neurasthenia, only the mucous membrane reflexes- — pharyngeal reflex, conjunctival reflex — are in hysterics relatively often very weak, or in- deed, absent. Sometimes upon pinching or pricking the anesthetic cheek, dila- tation of the pupil occurs. Sometimes the different qualities of sensation are affected to a different degree, only pain sense may be noticeably disturbed. An important characteristic of the hysterical disturbances of sensibility, their suggestive influenceability ("Pithiatism" according to Babinski) naturally can only be tested with considerable reserve. Attempts at "transference" for example, in which through any hocus-pocus, an anesthesia is transferred from one side to the other, should be directly forbidden, since they distinctly in- crease the morbid suggestibility. As to the topography of hysterical disturb- ances of sensibility, we can distinguish 1, universal anesthesia (excessively rare) ; 2, hemianesthesia ; 3, circular or geometrical anesthesias ; 4, insular or disseminated anesthesias. Typical for hysterical hemianesthesia, which usually affects also the mucous membranes, is in contradistinction to the or- ganic form, its sharp limitation to the middle line. Also, circular anesthesias are separated from the normally feeling skin areas, by very sharp lines which usually run at right angles to the long axis of the extremity — so-called "ampu- tation lines" (see Fig. 110); we find instead of peripheral or radicular areas, cap-like, sleeve-like, sock-like, glove-like, finger-like, anesthesias. Of organic diseases only lepra sometimes gives similar pictures. That between the dif- ferent topographical types of hysterical anesthesia there are transitions I need not specially emphasize. Experience teaches that hysterical anesthesias more frequently occur upon the left side. Perhaps this coincides with that, as van Bieri'liet has shown, in healthy people there is usually a slight difference in the sensibility of the skin unfavorable to the left side, when tested by delicate methods. Very frequent is the combination of hysterical disturbances of general sensibility, with those of the sense organs in which there are naturally phe- nomena analogous in principal. Among these stands first in importance, con- centric limitation of the visual field, in confirming which with the perimeter, we notice in many cases that the visual field, in the course of the investigation, becomes constantly smaller; also that the mutual relations of the color fields have experienced a change from the normal. Namely, in healthy people, the THE PSYCHOXEUROSES 441 visual field for green is smallest, somewhat larger is that for red, still larger the area for yellow, particularly, however, that for blue. In hysterical dvschroma- topsia (we speak of Forster's type of alteration) on the other hand, the limits for red more or less overlap those for blue. In spite of the most pronounced hysterical narrowing f the visual field the patients can usually move about with entire safety without striking against anything and have no suspicion of the anomaly appearing upon perimetric examination. Occasionally, however, there is a hysterical amblyopia proper in which, besides the visual field, acuteness of vision, color sense and light sense are found diminished. Hysterical blindness is, on the other hand, quite rare; like hysterical amblyopia, it occurs with in- tact light reflex of the pupils. A very great rarity is monocular polyopia. The hysterical disturbances of vision occur, as a rule, upon both sides, still, they are generally most marked upon the side on which general sensibility is most affected. Xext to vision, taste is most frequently affected in hysteria ; now it is perversions of taste in which bitter or otherwise disagreeable tasting sub- stances are found agreeable, and eaten by preference (hysterical '"longings," '"pica") again, ageusia for single or for all qualities of taste. This ageusia can occur uni- or bi-laterally. Less frequent is hysterical anosmia, indiffer- ently unilateral or bilateral, still rarer hysterical dullness of hearing which, as a rule, occurring unilaterally, can sometimes increase to complete deafness. The different tests for hearing (see Lecture II) give the same results as in nerve deafness ('"Rhine"), positive, etc., but the subjective ear noises, so fre- quent in organic diseases of the auditory nerve apparatus, are almost always absent; further, the patient, deaf on one side, usually in conversation conducts himself like a healthy person, while in unilateral organic hypacusis of any con- siderable degree, we nearly always notice that the patient attempts to turn his better ear toward us. Sometimes hysterical deafness is an elective one. in that, for example, whispers are heard while there is complete deafness for the tick- ing of a watch, etc. b. Vasomotor Symptoms If we disregard the not infrequent combination of hysteria with different vasomotor neuroses (see Lecture XXV), as well as the disturbances of vas- cular innervation frequent also in neurasthenia (dermographism,* factitious urticaria, fleeting erythema, "dead finger," etc.), the vasomotor anomalies oc- curring in the region of hysterical anesthesias are especially to be mentioned. The absence of bleeding on deep stabbing with a needle has a peculiar im- portance in the history of civilization. The famous "Malleus maleficarum" was accounted one of tin- surest "witch signs" and it has hence contributed to the delivery of innumerable victims to the slake Sometimes there appears also about the stall a circumscribed oedema, Further, in hysteria there is a not entirely rare, firm swelling, not pitting * In hysterica soim-limcs more complicated forms of dermographism occur: for example, in one ut my patients, upon stroking tin- Bkin with tin- finger, there appeared one red be- tween two white streaks. 442 LECTURE XXIX upon pressure, combined with intense cyanosis and coldness of the skin over the anesthetic or paralyzed hand which has been called "blue oedema." The circumstance that in persons especially susceptible to hypnosis it has been possible to suggest the appearance of skin vesicles and sugillations, suggest the possibility that trophic skin disturbances and extravasations of blood may occur upon the basis of hysteria. Still, it is certain that the impressive ma- jority (if not indeed all) the cases described as "hysterical pemphigus," "hysterical ulceration," "hysterical hemorrhages," etc., have been produced by self-injury, a point to which, when speaking of the hysterical psyche, we will come back again. Also most cases of the so-called "hysterical fever" are to be referred to simulation (warming or shaking the thermometer), further, diagnostic errors — failure to recognize a latent tuberculous focus, etc. — often occur. On the other hand there are known a number of undoubted cases, in which the temperatures were taken by the physician himself in the rectum or vagina and the eventuality of an organic cause could be excluded. These are chiefly of temperature disturbances which occurred in hysterical seizures or at the time of the menses, some reaching quite high degrees (up to 42° C.= 106° F.), but were not accompanied either by corresponding alterations of pulse and respiration, or by the urinary changes characteristic for fever. On this account the name "hysterical hyperthermia" is decidedly to be preferred. Palpitation of the heart and rapidity of the pulse occur in hysteria much less frequently than in neurasthenia. c. Motor Symptoms Hysterical tremor can be unilateral or bilateral, and may affect the upper or the lower limbs. In Basle as the "shaking disease" it excited great wonder some 3'ears ago : A hysterica] school-girl had in this way exerted so striking an effect upon the phantasy of her school-mates that the tremor gradually spread from child to child. In this manner two hysterical school epidemics occurred, which were only stamped out by strictly enforced isolation. For the rest, we observe hysterical tremor with special frequency among "traumatic hysterics." It is usually in a great measure dependent upon attention, much more so than is the case in the tremor of neurasthenics or even in tremor organi- cally caused. Only in a minority of cases is it the fine vibrating tremor men- tioned in Lecture XXVII, usually it is of greater amplitude and moderate rapidity (5 to 7 oscillations a second) : Its character can, however, be subject to frequent change ("polymorphous tremor"). Further, occasionally the most varied dyskinesias can be "imitated" by hysteria, in which long observa- tion, careful taking of the history and accurate study of the suggestive influenceability is needed to determine the hysterical nature of the disease. Here belong among other things the disease pictures which we have already mentioned in Lecture V as "Chorea Major" and "Bergerons' Electric Chorea." The frequent coincidence of hysteria and local spasms as well as tics has been alluded to when speaking of the dyskinesias; I would mention particularly, THE PSYCHONEUROSES 443 oesophagism, pharyngism,* yawning, sneezing, snoring spasms, facial tic, etc. (see pages 67 and 72). Anesthetic limbs upon being tested with the dynamometer usually show a diminution of the gross strength with very rapid fatigability, more rarely, what has been described by Charcot as the "contracture diathesis." Massage, faradization or binding the affected extremity has as its result a contracture which only ceases again after some seconds or minutes. J. A iscerai> Disturbances On the part of the digestive organs practically the most important, since in severe cases they lead to marked inanition and cachexia, are hysterica] anorexia and hysterical vomiting. In the last, the food is usually vomited immediately after taking; also independent of taking food, there is bringing up of masses of mucus. Very often in hysteria, in contradistinction to neurasthenia, vom- iting is the only anomaly on the part of the stomach and the feeling of pres- sure in the epigastrium, eructations and the other symptoms of nervous dyspepsia are absent. That only an exact examination of the stomach with exclusion of an organic affection permits us to diagnose the vomiting as hysteri- cal is self-evident. Aery typical of hysteria are the cases in which patients regularly react with vomiting to some definite situation {e.g., coitus), upon seeing certain colors, hearing certain noises, etc., or where they are able to vomit "at command." Meteorism, the distention of the intestines by gas, reaches on an average much higher degrees than in neurasthenics; in hysterics, as a rule, the swallowing of air (aerophagia) plaj's a part in this. As curiosi- ties nia\ hi' mentioned here the cases of "hysterical pregnancy," in which women not pregnant but filled with longing for a child, have shown along with a meteoristic distention of the abdomen, cessation of the menses, swelling of the breasts, the appearance of colostrum, morning sickness, etc., in short, the most varied signs of pregnancy, and only upon the supposed beginning of labor, was the actual nature of the case maele evident. Obstinate constipation is particularly frequent in hysterics; also the mucous colic already mentioned is often a symptom of hysteria. On the part of the uropoetic apparatus the mosl importanl hysterica] anomalies arc oliguria which usually (to a certain extent vicariously) , ,H"e> hand in hand with hysterica] VOmiting,f and polyuria (up to 12 liters a day and more) ; dm- to polydipsia. Particularly frequent are genital disturbances; almost all female hysterics suffer from nervous symp- toms at menstruation; sexual frigidity i-- frequent, with which are contrasted an exalted psychic eroticism and a resulting "desire for excitement." On the other hand sexual hyperesthesia occurs frequently. It leads often to a vaginis- mus hindering cohabitation, or the introitus directly represents a hysterogenic Globus hystericus, with which we will become acquainted as .-in initial symptom of hysterical convulsive attacks, which, however, <><, etc.). We hence speak also of "Pension hysteria." Hysterical paralyses are usually flaccid, more rarely spastic, and belong iij\v to the mono- plegic, again to the heniiplegic or paraplegic type. They may represent transi- tory, fleeting phenomena (this applies particularly to post-paroxysmal paraly- ses which Gendrin hence considers as exhaustion paralyses), on the other hand, however, are marked by great obstinacy. The combination with hypesthesias or anesthesias of corresponding topography is very frequent. Hysterical paralyses when they persist very long can lead to a definite reduction in size of the affected limbs, still, this is only an atrophy from disuse which is never accompanied by reaction of degeneration or change of the superficial outlines. The reflexes are never lost in the flaccid form; in the spastic form they are usually somewhat exaggerated, but not greatly so. The phenomena of Babinski, Oppenheim and Mendel-Bechterew are always absent as is also true ankle clonus, while we occasionally find the "pseudo-ankle clonus" already de- scribed. Oppenheim has further pointed out that the examiner who holds a hysterically paralyzed extremity for some time in his hand sometimes plainly perceives occasional innervation impulses in its muscles and that such a limb, when passively held in a certain position, then suddenly let loose, is able to maintain for some time a position, which is only explainable by the activity of the otherwise paralyzed muscles. Hysterical hemiplegia almost always avoids the facial and hypo-glossus. The gait differs markedly from that of cerebral hemiplegia, in that the paralyzed leg is not circumducted, but is simply dragged along, the sole or the heel scraping the ground. If the patient uses a crutch, he particularly does not bring his leg into contact with the floor. In hysterical paraplegias the functions of bladder and rectum are almost always intact; in all hysterical paralyses tropho-vasoniotor disturbances are much rarer and in every case less intense than in the organic. Finally, it is pathogenetically as will as diagnostically important that only the conscious voluntary movements are suspended. In alcoholic and chloroform intoxications, on the contrary, there is lively gesticulation. A very characteristic syndrome is the so-called "astasia-abasia," in which the patienl in bed can execute every movement with normal strength ami com pleteness, but, on the contrary, upon attempting to walk or to stand, simply sinks down (different from this symptom-complex first described by Jaccoud, is Mingazzini's "Stasobasophobia," an emotionally caused psychasthenic dis- turbance of gait marly related lo agoraphobia). A frequent form of hysterical paralysis is hysterical aphonia, usually the immediate effeel of a fright, a particularly obstinate symptom, tending to re- cur. The patients speak only in whispers; in it. as laryngoscopic examination Bhows, the vocal chords are not at all, or only imperfectly approximated. Coughing and clearing the throat usually occur noisily, however. Rare, but almost pathognomonic of hysteria is suddenly appearing mutism, in which, in contradistinction to aphasia, no sound or portion of a word can be produced, while mimic movements remain quite active. Excessively rare is hysterical ptosis, which, according to Oppenheim, is to be distinguished from thai organ- ically caused, by the fact thai the contraction of the frontal muscle observed in this last i^ absent; il should no! be confused with hysterical pscudo ptosis 448 LECTURE XXIX which occurs from spasmodic closure of the eyes, also introduces us to the hysterical contractures. 4. HYSTERICAL CONTRACTURES In these contractures there is permanent fixation of a limb in a definite position by the tonic contraction of certain muscles. They develop in contra- distinction to the hysterical paralyses by degrees in the majority of cases, though usually in connection with the exciting causes mentioned under those paralyses. Upon distraction of the attention, the contracture diminishes (in sleep it usually disappears), while, on the contrary, the manipulation by the examiner of the contractured limb plainly increases the muscular tension. In very long duration of the hysterical contracture there appear, however, as in mechanically caused fixation in a definite position, material alterations in the muscles, the ligaments, etc., which are responsible for the fact that some- mes even upon cure of the psychogenic anomaly a complete restitutio ad integrum is not attainable. In contradistinction to the contracture after organic diseases of the pyramidal tracts, in hysterical contractures (they also assume by preference in the arm, the type of "flexion," in the leg that of "ex- tension"), the reflexes are not exaggerated and the other accompanying phe- nomena of the spastic symptom-complex are absent. Also upon passive short- ening of the distance between the origin and the insertion of the contracted muscles a relaxation does not occur. Along with the contractures of the ex- tremities and the pscudo-ptosis already mentioned as relatively frequent in hysteria, there are to be named contractures of the neck muscles (see in Lec- ture IV, under Tonic Local Spasms, Torticollis, Retrocollis, etc.) and glosso- labial hemispasm which draws the lips and tongue laterally, in a grotesque fashion. Psychology and Pathogenesis Gentlemen ! Many physicians, when asked their position upon the ques- tion as to the nature of this peculiar psychoneurosis whose semiological study we have now finished, confess still to-day the standpoint of Lasegue, "The definition of hysteria has never been given and never will be." This convenient axiom we can only accept in the sense that it is not possible to include the varied symptomatology of this "protean neurosis," the munifoldness of its special etiological factors and the numerous variations in its course, within a short formula. It is, however, possible to arrive at a pathogenetic conception of what authenticates itself as hysterical if one attempts to view the tangle of clinical details from a psychological point of view. If we start out with the description of the psychic stigmata of hysteria, whose discussion we have intentionally put off until this time, we can distinguish with Cramer four groups of phenomena which naturally, like the somatic stig- mata, need not all be present in every individual case, of which, however, one or more occur in definite or discrete manner in most hysterics. These are: 1, Excitability under affect; 2, ideas of being wronged; 3, inability to accurately reproduce impressions ; 4, striking variations of mood. In general the hys- THE PSYCHONEUROSES 449 fcerica] individual is moved with extraordinary intensity by affects of agreeable or disagreeable tone, and reacts to these emotions in a specially unrestrained manner. In general a morbid egocentrism manifests itself in the tendency to interpret occurrences in their relation to his own person and in so doing bj preference to assume the role of a martyr; in him in general the retouching of the objective facts through subjective factors, as well as the description of external happenings, particularly in that of his own sufferings, reaches. the highest degree, even "pathological untruthfulness ;*' finally, in general, the hysterical are entirely irresponsible in their disposition. It is a question now if this "hysterical character" furnishes a comprehensive and satisfactory explanation for the fact that just in such individuals auto- suggestibility (whose importance for the occurrence of hysterical phenomena can be experimentally recognized) is increased to an actual disease condition. This question is to be answered in the negative upon the ground that many healthy people, but particularly many psychasthenics and neurasthenics, pre- sent one or another of these stigmata, or indeed, all of them, in marked devel- opment, and even then, do not show specific hysterical symptoms if they experi- ence severe psychic traumata. Further, Dubois has pointed out in a brilliant manner, that autosugges- tibility, in conjunction with the constitutional inferiorities expressing them- selves in the hysterical character, may, indeed, explain the appearance of inadequate somatic reactions to emotions, not, however, the following impor- tant criteria: "The persistence during weeks, months, years, indeed during I he whole life, of an array of functional disturbances which appear in the absence of every primary material injury and which in consequence must he referred to psychic causes." This characteristic fixation of the somatic phenomena arising from emotions (actual or suggested), Dubois attributes to a psychic anomaly which is to be considered the most important factor in the specific hysterical diathesis, "sense impressionability." Now the Bern neurologist defines "sense impressionability," "The ability to impress upon the sensations arising from affects, the stamp of reality:" you see in how satisfactory an agreement what I remarked to you about the role of phantasy as a predisposing factor for hysterical affects, stands with the dedue tions of Dubois. That, also, in the stigma the inability to accurately repro duce impressions— which can increase to "mythomania," "phantasy," reaches clinical expression, has scarcely escaped you. Though I confess myself without reserve an adherenl <>. the teachings of Dubois as to the pathogenesis of hysterical conditions, I cannot leave unmen- tioned the much-discussed views of the Vienna neurologist Freud. He assumes that an affect when it does not had to an adequate reaction, is transposed into somatic disturbances. The hysterical disease manifestations are based upon such a "conversion." And, indeed, the disease-producing "complex" is regU larlv to he sought in the sexual experiences of the earliest childhood, which had exercised a powerful impression upon the childish psyche, hut from natural reasons i- concealed and not "reacted out." In that, tittle by little this com plex disappears from consciousness, that is. i- "supplanted" bj hysterical phenomena (which, all the -ana. ar>' Bymptomatologically in accord »ilh the- 450 LECTURE XXIX sexual trauma in childhood, for example, a hysterical attack with a parental coitus witnessed), from a therapeutic point of view it is necessary through "psycho-analysis" to bring up the "pent in affect" from the subconsciousness into consciousness and rid the patient of it. That in spite of the very great exaggeration of the frequency of an infantile sexual etiology, the extensive lack of criticism in the development of the doctrine of conversion, and the regrettable growth which the thera- peutic application of psychoanalysis has attained, Freud's views contain a useful principle, is to be acknowledged without dispute. So he has pointed out effectively, not indeed for the first time, but most clearly, that every hysteria is a traumatic one, and that it is regularly psychic factors which bring the dormant psychoneurosis to an outbreak. These are mainly acute and brutal psychic traumata, and not, as a rule (as is generally the case in acquired neurasthenia) a more chronic emotional unrest, the storm of affective irritations, small in themselves, but always protracted in action, which sup- porting themselves upon former ones not yet run out, undergo summation and accumulation. It is also true, that not rarely the symptomatology of the concrete hysterical attack is influenced by the initial trauma. I have already mentioned this for accident hysteria. As to sexual traumatic hysteria, a like relationship is sometimes found, so three cases of contracture of the adductors of the thigh which I observed in females arose from attempts at criminal assault, on which occasion these muscles were put into a position to do honor to their ancient name "custodes virginitatis." Let it be re- marked in passing, no "psycho-analysis" is needed to uncover sexual etiological' factors (or to suggest them) ; they are naturally not subconscious and "dis- placed," but usually they are intentionally concealed from the physician until he has inspired a sufficient confidence by tactful demeanor. That then talking it over with the psychotherapeutist and his soothing reassurance can only act advantageously and can assist recovery, is evident to any one without his having to confess adherence to the dogma of the "hemming in of affects." The Psalmist wrote about "sin," "Should I conceal it, my limbs would give way," and also the ordinance of auricular confession bespeaks eminent psycho- logical intuition. Still one thing more : it is shown that many cases of hysteria can be dated back to initial traumata of sexual or erotic nature; this corresponds to the powerful role which the sexual factor plays in the life of the individual. Only one must not think that the sexual traumata must always occur in early childhood, and must be connected with actual irritation of the genitals, as Freud has apodictically affirmed. Rather do sentimental-erotic misfortunes, as, for example, a broken engagement in a young girl, play a very great role. Also in the frequent hysteria of childless women, the insufficient potence of the husband is but rarely to be held respon- sible ; much more frequent is the collapse of a long-cherished hope of offspring; the same thing applies to the hysteria of old maids, which often breaks out at the time when the last illusion in regard to the longed-for "settlement" is dissipated. Dubois has said of the female hysteric, that she is a comedienne, but does not know it, that she is acting, and honestly believes in the truth of her THE PSYCHONEUROSES 451 roles. This may in general be entirely correct, but does not apply to certain cases in which the desire for commiseration, the "pose." the coquetting with illness, lead to refined "pathomimicry" which sometimes does not draw back from self-mutilation. Fig. Ill represents the hand of an hysterical woman who was detected as she was producing her "hysterical pemphigus" with hot sealing wax. Still more monstrous was the case of a young girl who had produced in herself a "puzzling tropho- neurosis" by about 150 cuts reaching down to the fascia on the left arm and hot Ii legs and in whom my diag- nosis of self-injury nearly brought me into an unpleasant situation for sus- pecting the "brave daughter" of such a tiling. Fortunately the discovery of a pair of bloody scissors concealed be- tween the mattresses rehabilitated me, and the patient then furnished an inter- esting written account, from which it appeared that after an exciting family scene, while in her bath, she accidentally noticed the anesthesia of her left arm, and then, partly in order to make her- self interesting and partly to mystify the family doctor, had begun the auto- mutilation. In another girl, when at different points in her skin needles ap- peared, I received the confession that she had swallowed them in order to pro- voke a hemorrhage from the stomach. In a case of Dietilafoy's the patient allowed it to go so far that the ex- tremities which he had intentionally cauterized with lye were amputated. Opposed to these rare extremes, forc- ing up the thermometer, the production of blood-spitting by wounding the gums, putting albumen in the urine, etc., represent the frequent "formes frustes" Hysterlca j Self-Injur of pathomimicry. From the fact that self-injury, swallowing foreign bodies, etc., occasionally result fatally, tin- otherwise good prognosis of hysteria as regards life becomes more serious in such cases. The same remark applies to the sometimes theatrically staged suicidal attempts, which occasionally succeed better than was intended. True, intentionally carried out suicide is exceedingly rare in hysterics. But one word on the so-called "hysterical psychoses." I am not willing to accept without reserve the view of Aschaffenburg, according t<> which there is no definite clinical form of hysterical insanity, bul rather that usually a Fig. in. Pseudo Pemphigus. 452 LECTURE XXIX mixture of more or less numerous and striking hysterical features with the most varied psychoses (for example, with manic depressive insanity) can occur. Treatment That from what has been said above, the rational treatment of hysteria can only be a psychic one is apparent without further remark. While here, in general, the same underlying principles apply as were laid down for the psychic treatment of neurasthenia, in the previous lecture, on account of the preponderant role which in the clinical picture of hysteria is to be assigned to autosuggestive factors, some modifications are desirable. While, for example, the neurasthenic is favorably influenced psychically by frequent and accurate examination into his condition, since he thereby becomes assured that the physician is carrying out his task particularly thoroughly, and on this account is inclined to put more and more faith in the assurance that there is no organic disease, and his troubles are bagatelles, in hysterics it is well to avoid frequent examinations. For example, if by an exact examination carried out under all precautions, an anesthesia is recog- nized as certainly hysterical, it is wisest to completely ignore it thereafter until taking the condition of the patient upon discharge. In severe cases of hysteria, isolation from the home surroundings and bed treatment, not ambula- tory, is absolutely required. In hysterics much reduced in nutrition this can be combined with the rest cure after Weir Mitchell. Anorexic hysterics must have the psychical genesis of their repugnance to taking food explained to them and after this must, without severity but with unyielding persistence, be trained to take an increasing quantity of nourishment. One should refrain as far as possible from using the oesophageal tube. While a few patients have a wholesome horror of this instrument and so may be persuaded to take a suffi- cient quantity of food, on the other hand the patient finds pleasure in his role of martyr, and there is difficult}' in laying the ghost that has been called up. Also astasia-abasia, aphonia, contractures, etc., must be cured by irresistible reassurance and patient explanation of their psychogenic nature by methodical re-education of the will. Seizures are disregarded as far as possible; also the family and surrounders must learn to not make much of these, but to content themselves, after sprinkling the patient with some cold water, to leave him alone as much as possible until he comes to himself. In paralyses, aphonia, etc., in fresh still untreated cases, the so-called "surprise method" is often found a success. In this, by the application of some striking procedure previously un- known to the patient (the application of static electricity, endo-laryngeal fara- dization, hypnosis, etc.), a sudden cure of the symptom (naturally not of the disease) bordering on the marvelous may be sometimes obtained. Against this method no objection can be made, only if we explain afterward to the patient the psychotherapeutic nature of the cure, that the procedure is only active sug- gestively, so proving to him directly the autosuggestive nature of his disease. For the rest, the object of our treatment must be not only the explanation to the patient of the nature of his troubles, but also of their cause; he must learn to combat his "sense impressionability," his phantasy, his egocentrism, THE PSYCHOXEUKOSES 453 his impulsiveness, his exaggerated emotivity, as well as to oppose the psychic traumata which has set up his hysteria, with objectivity and stoicism, after he liberates himself from the pressure of painful memories by their communi- cation to a sympathetic counselor. All these things allow themselves to be put into words in a very condensed and plausible manner, but in practice are the most difficult and trying tasks which come to the psychotherapeutist. LECTURE XXX Migraine In the course of these lectures we have become acquainted with different varieties of headache as a symptom occurring in a large number of nervous diseases: so in arteriosclerosis and syphilis of the brain, in internal hemor- rhagic pachymeningitis, in the different leptomeningitides, in brain tumors, brain abscesses, hydrocephalus, in progressive paralysis, after epileptic at- tacks, hysteria, etc. The occurrence of headache (cephalalgia, cephak-ea) ex- tends, however, far beyond the boundaries of neurology. I would remind you of those forms which are known to you as an accompaniment of fevers, as the expression of slight uremia, in consequence of chronic nephritis, as an initial symptom of syphilitic infection; further, of the locally caused headache in diseases of the ear, the eye, the frontal sinus and antrum of Highmore, of the bones of the skull (gummata, tumors, caries, periostitis), of the neck and frontal muscles, and the cephahea of chronic lead, alcohol, nicotine and many other intoxications, of those which are complained of during the incubation stage of infectious diseases, of the transitory headache with which even the healthy person becomes acquainted, after mental or physical overexertion, excesses in drinking, sleepless nights, sojourn in hot and poorly ventilated rooms, etc. To-day, however, there remains for us the description of a form of headache which even in the second century after Christ was described as a disease sui generis (by Galen and Aretwus, of Cappadocia), and still pre- serves its nosological autonomy. This is hemicrania or migraine. Just as in Lecture XXVI, we kept in mind only genuine, not symptomatic epilepsy, here also we will disregard those cases in which migraine-like attacks occur during the development of a brain tumor, as symptoms of multiple sclerosis, or pro- gressive paralysis; also those observations very interesting in themselves, where in epileptics migraine attacks alternated with convulsive seizures, so that their conception as "epileptic equivalents" has impressed itself. Such cases, like the not very rare occurrence of members of one and the same family, afflicted part with epilepsy and part with hemicrania, suggests the thought of a certain nosological relationship between the two affections (to which besides the oc- currence in paroxysms is common), while on the other hand, opposed to such a conception, is the fact that epilepsy usually manifests progressive tendencies ; migraine, on the contrary, shows practically always with the advance of age, a reduction in the frequency and intensity of the attacks and indeed, in many cases finally spontaneously disappears. Also in migraine patients degenerative symptoms are, as a rule, absent. It is an exceedingly frequent nervous disease, particularly among the upper 454 MIGRAINE 453 classes of societ}-, which affects women in a quite preponderant manner; in my material at the most 20 per cent, of the hemicranics were of the male sex. Like heredity, it is often recognizable. That the arthritic diathesis, which is much supported by the French and English, plays any role worth speaking of in the etiology of migraine, is very questionable; in any case, the notorious rarity of true gout in our neighborhood stands in striking contrast to the extent of migraine. The combination with psychoses, neurasthenia and hysteria is quite frequent; that with vasomotor neuroses not rare. Very questionable is the frequently affirmed causal relationship of migraine to gynecological, rhino- logical, or stomach diseases. The disease appears usually at the time of puberty, still, its beginning at an even earlier age, for example, ax 7 years, is in no way rare. Quite 90 per cent, of cases set in before the 20th 3'ear of life; an occurrence of at- tacks of migraine for the first time in persons between 40 and 50 years of age is always suggestive of the symptomatic form. Relatively frequently the at- tacks cease during the 6th decade, after it has decreased in severity and fre- quency from the 40th year; in women the recovery sometimes coincides exactly with the menopause. On the other hand, there are persons who are still sub- ject to migraine attacks even at an advanced age. Sy mp t o m a tology In the preponderant majority of hemicranics — if we disregard the ac- companying psychoneurotic conditions — the migraine attacks occurring at more or less regular intervals (it can show a regular or an irregular type), represent the whole clinical picture and in the intervals there are no anomalies to be observed. A minority of patients, on the other hand, show also inter- paroxysmal symptoms. We will now, to gain a comprehensive view, firs! sketch the usual clinical picture of migraine attacks, then consider the differenl varieties of the paroxysms, and finally the interparoxysmal phenomena, a. The Regular Migraine Attack, Hemicrania Simplex For the occurrence of a migraine attack, there are either no provocative agents to be found, or certain exciting causes can witli more or less proba- bility be held responsible; this most certainly applies to the menstruation, since many patients are exclusively and regularly affected by hemicrania at the time of their catamenia; for the rest, Hie patients frequently give as ex- citing factors overexertion, psychical excitement, coitus, excessive use of al- cohol, too short or too long sleep, indigestion, hunger, the occurrence of sultry weather, etc. The time of predilection for the outbreak of the attack is the early forenoon, still it can occur at any hour. Occasionally it strikes the pa- tient like lightning out of a clear sky, though he has gone lo lied with a clear head and feeling particularly well, lie wakes in the morning with severe mi- graine; usual] v, however, characteristic premonitory symptoms precede its ap pearance. Grasset, Rauzier, and others have differentiated two types of these prodromi, the "excited type" in which there conns oxer the patient a peculiar 456 LECTURE XXX motor unrest along with psychic irritability, and sometimes also sexual excite^ ment, and the "depressed type" which is characterized by the preponderance of a sad or morose mood with a feeling of depression, by drowsiness and yawning. When the attack is coming on, the patients usually look pale and prostrated; often they complain of pressure in the epigastrium, precordial pressure, urinary irritability, shivering, dullness in the head ; when the mi- graine comes on in the morning, during the preceding night, sleep is often restless and accompanied by bad, in part stereotyped, dreams. An actual "aura" occurs only in a few severe forms of migraine, for example, in the form of paresthesia of the tongue, or of one hand, tinnitus aurium, spots before the eyes, etc. The headache is, as the name hemicrania expresses, at the start one-sided or predominating upon one side, but not at all infrequently in the course of the attack affects both sides of the head with equal intensity. It is usually frontal or temporal, more rarely occipital, but can also radiate into the neck, shoulder and arm. Its severity usually increases little by little to remain at its maxi- mum several hours, and then just as gradually to disappear again; when the migraine has so far decreased that the patient can go to sleep he usually awakes with his head quite free again, rarer is a "critical" discontinuance of the pain while the patient is awake. Its severity is quite different from case to case, sometimes also in the same patient from attack to attack. With the mildest migraine the patient can still go about his business, the more severe forms compel him to lie down in a darkened room as free from noise as possible (since light and noise increase his trouble) ; the most severe cases, however, increase to actual torture which forces from the patient groans and lamenta- tions. The character of the pain is varied ; usually it is described as hammer- ing or boring, sometimes as cutting or burning. As to its topography, it is by no means always stereotyped ; even in the unilateral type, sometimes the right, again the left, now more the forehead, now more the temporal region can be affected, etc. The migraine headache is always accompanied by nausea, which usually in- creases to retching, often to actual vomiting; this last is independent of taking nourishment. It can occur upon an empty stomach (for example, immediately after awakening), with the bringing up of gastric juice or bile, or there are indeed, abortive vomiting movements, a particularly painful condition. The loss of appetite is complete during the attack. The face is usually pale and sunken in (the so-called "white" or "angio- spastic migraine"), more rarely reddened and swollen ("red" or "angio-para- lytic migraine"). Sometimes these disturbances of vascular innervation are accompanied also by other sympathetic phenomena ; so the physiologist Du Bois-Reymond noticed in his own migraine attacks, besides pallor of the face, redness and heat of the ear, hyperemia of the conjunctiva and narrowing of the palpebral fissure. Flatau found, at the height of the hemicranic paroxysm, hardening of the temporal arteries, myosis or mydriasis, swelling of the upper lids, etc. Oppenheim and others have described increased discharge of mucus from the nose, Tissot, Labarraque and others, small extravasations of blood in the face, in the nose, in the retina. Curschmann noticed "vasomotor angina MIGRAINE 457 pectoris." Berger, diarrhoeas, Calmeil, polyuria and pollakiuria, I myself cir- cumscribed oedema of the face, "dead fingers," and flow of tears, as accom- paniment of the hemicranic attacks. The pulse is sometimes slowed, indeed to 40 a minute. Mobius lias pointed out the relatively frequent occurrence of sensitiveness to pressure of the Vallcix's points of the trigeminus and occipitalis (see Lee hire III, pages 48-49. Also a hyperesthesia of the seal]) and the skin of the face appears during the migraine attacks, further, paresthesias of the lips, the tongue and the hand. The attacks just sketched, which in a few patients show themselves only at long intervals (about two or three times in the year), in others once or twice a week, upon the average, however, have a tendency to appear about every three or four weeks, can in severe cases succeed one another in such a manner that there is a status hemicranicus (or. according to Oppenheim, a "permanent hemicrania"). In this after the migraine is apparently on the de- crease, the headache increases again to maximum height, and this repeats itself for several days. From this condition Flat ail with propriety wishes to see separated "continued hemicrania." that is, cases in which the migraine instead of the average duration of the attack of about 12 hours, persists for days in unaltered intensity, and then slowly disappears. b. Particular Varieties of Migraine Among tin' varieties of migraine, on account of their frequency, the abortive or rudimentary attacks deserve to be mentioned in the first place; in these, after more or less definite development of the prodromal symptoms, there is a. slighl pressure in the head with some nausea, but after a short time the patient feels entirely well again. Of greater symptomatic interest are the migraine forms which occur with cerebral irritative or debet symptoms; so, at the height of the attack, there are not so very rarely more or less outspoken aphasic disturbances (usually of Brora's, more ranly of Wernicke's type); further FcrS and others have pointed out fleeting hemipareses on the 6pposite side to the maximum of pain; Liveing, to object ive disturbances of sensibility of cerebral topography : Oppen- heim, to transitory typical cerebellar ataxia, with severe vertigo (cerebellar hemicrania); Flatau, to clonic contractions on one side of the face, etc. A.s "ophthalmic migraine" Charcot and Frrr have isolated a variety of hemicrania, in whose clinical picture certain visual disturbances appear par- ticularly prominently. One of the most frequent tonus of course of this eye migraine, is the following: The patient has suddenly a peculiar light per- ception, in that either flames. >parks or lightning, move before his eyes, or with darkening of the central part of his visual field, in its periphery bright serrations, which now separate from, again approach one another, nou rotate like a cog w In,!, disappear, again glitter with all colors of the rainbow, but do nut prevent the perception of surrounding objects. We speak of "scintil lating scotoma" or also of "teichopsia" | rampart) since the serrated figures remind n> of the plan of a citadel aft, r Vauban. 458 LECTURE XXX The light phenomena, which usually occupy only one-half of the visual field, disappear after some minutes, usually to make place for a transitory hemianopsia, eventually, also, for a temporary amaurosis, to which then, the usually specially severe unilateral pain, the nausea, the prostration, in short, the ordinary migraine symptoms, succeed. The psychiatrist Jolly and the astronomer Airy have furnished very good descriptions and pictures of their own attacks of ophthalmic migraine and scintillating scotoma. Ophthalmic migraine can also begin without the entoptic phenomena described, with simple temporary hemianopsia or amaurosis ;* further, the ocular symptoms instead of affecting as is usual both eyes, exceptionally affect only one; finally, there is sometimes an intense sensitiveness to pressure of the one eye ("iritic mi- graine" of Piorry). Combination of ophthalmic hemicrania with the focal symptoms already enumerated (transitory aphasia, facial hemispasm, etc.), is relatively frequent. Antonelli and Siegrist have been able to recognize oph- thalmoscopically during the attack, a spasm of the retinal vessels. As ophthalmic migraine affects the visual organ itself, ophthalmoplegic migraine (denominated by Mobius "periodic oculomotor paralysis") involves its muscles. The attack begins like simple hemicrania, which, however, as a rule, is characterized by great severity and long duration (up to 14 days) ; then, however, it passes over into an oculomotor paralysis (usually total, but irregularly distributed among the different muscles). This last is homolateral with the headache and usually lasts for several weeks, while the pain generally disappears with the appearance of the ophthalmoplegia. The trochlearis and abducens are only rarely affected. As analogue of ophthalmoplegic migraine, Flatau has isolated "facio- plegic" (better, "prosoplegic") migraine, a form unknown to me personally, in any event very rare. It leaves behind a facial paralysis of peripheral type which recovers only after several weeks. We will close the enumeration of the atypical forms of migraine with the mention of "olfactory migraine," "gustatory migraine," and "otic migraine." These are attacks which are accompanied by disturbances of smell, taste or hearing, or are followed by such. Such observations are also to be considered as great rarities. Entirely outside of the limits of hemicrania are to be placed forms of headache which have been described by Hartcnbcrg, Peritz, and others, as "mi- graine of the arthritic," myalgic migraine, etc. As a matter of fact, these cases belong to an interesting rheumatic affection which has been isolated by Hcnschcn, Norstrom, S. Auerbach, Edinger, and others, as "nodular" or "in- durative" headache. In this, the pain radiates from the neck and forehead muscles unilaterally or bilaterally over the scalp, and in the trapezius, rhom- boideus, splenii, frontalis and other muscles there are to be recognized upon palpation, partly diffuse, partly circumscribed swellings. Now the swelling is yielding and elastic, again firm and hard. The first corresponds more to the * One of ray patients, a Polytechnic student, while in school, noticed the onset of hemianopsia, in that suddenly, instead of two blackboards, he only saw that to the left; immediately afterward bis right hand and right side of his tongue "went to sleep," and pain in the left side of the forehead appeared. MIGRAINE 459 subacute, the last more to the chronic cases. Almost always there are re- frigeratory influences, sometimes of local nature (in women washing the hair in winter) to be discovered. His has also pointed out the presence of uricemia. Under the influence of fatigue and dragging upon the affected muscles, of local refrigeration, and getting wet, this nodular or rheumatic cephalaea usually exacerbates. Besides muscular nodules and indurations, enlarged lymph glands, and infiltration of the skin at the back of the neck may sometimes be found. Reflexly (perhaps also by irritation of the sympathetic in the neck), the severity of the pain can lead to nausea, rarely to vomiting. In pieces removed from such "headache nodules" I have not been able to find anything abnormal microscopically; there is also no infiltration or true induration, but as A. Mutter thinks, the result of a localized hypertonia. Local applications of heat combined with massage and percutaneous or internal salicylic treat- ment, is if persisted in long enough, of sovereign efficacy in this "nodular headache." c. Interparoxysmal Symptoms Expressly to have pointed out the greatly neglected interparoxysmal symptoms in migraine patients, is a special service of E. Flatau. Among these are on the one hand interesting, those syndromes which occur themselves in the form of attacks and so justify their consideration as "migraine equiva- lents"; on the other those which are characterized by their constancy. Of migraine equivalents there may be mentioned as examples: Attacks of vaso- motor angina pectoris, Quincke's oedema, bronchial asthma, neuralgias (for example, Morton's metatarsalgia), rotary vertigo, tinnitus aurium, psychic depression, gastralgia, yawning and sneezing spasms. Of the permanent symp- toms which usually affect older persons, most frequently described is thCper- sistence of hemianopsias, or minor defects in the visual field after repeated' occurrence of ophthalmic migraine. Meige saw develop in an aged migraine patient a hemiparesis of the face with unilateral oedema. Exceptionally oph- thalmoplegic migraine can leave behind permanent paralysis of the oculo- motorius or of some of its branches. Finally, I would mention that two of inv migraine patients had permanent bradycardia. Pathogenesis As to the basis of migraine, a number of theories have been proposed, into all of which I naturally cannot go. The most modern, supported by S. Auer- bach, is based upon Reichardt's theory of "swelling of the brain" already touched upon in Lecture XIX. Auerbach thinks that the symptom-complex migraine is explained with least constraint, if we assume that the "hemicranic predisposition" depends upon a lack of relation between the space within the skull and the volume of the brain, and that the attacks are set up by exciting causes which are calculated to increase this lack of correspondence by waj of the vasomotor system. In my view, this theory, seductive from many points of contact between the semiology of the hemicranic paroxysm and that of the 460 LECTURE XXX brain pressure syndrome,* is not sufficiently well founded for my acceptance of it. Rather do I confess myself, with the great majority of neurologists, of the opinion that the migraine attacks depend upon vascular spasms in the brain, that is, the hemicrania represents a vaso-constrictor neurosis; no other theory can explain so satisfactorily the fleeting focal symptoms (henupareses, aphasia, hemianopsia, etc.), the accompanying paresthesias; the combination with vasomotor angina pectoris, "dead fingers," etc. Also, the ophthalmo- scopic findings of Sicgrist and Antonelli may be referred to. Further there are observations in which thromboses of the brain vessels and foci of softening, occurred after unusually severe attacks of migraine. The attempt of Du Bois- Reymond and Mollcndorf to differentiate a vaso-constriction and a vaso-dilata- tion migraine is, on the other hand, to be considered a failure ; from the investiga- tion of vasomotor neuroses we know so much about sharply localized vascular spasms, that it does not do to deduce from the different condition of the arteries of the integument in "white migraine" and "red migraine," that the condition of the brain vessels must coincide with that of the superficial vessels. That autointoxications, hormonological anomalies, etc., are to be held respon- sible for the periodic setting up of migraine attacks, is often assumed ; but the nature of the substances coming into question is still entirely obscure. Prognosis and Treatment In genuine migraine, fatal complications, as we have indicated, are most extreme rarities, so that the prognosis as to life is in general quite favorable. As to recovery, a somewhat reserved standpoint is proper, since not all casea recover in later life, as is the rule. Namely, ophthalmic and ophthalmoplegic migraine may be characterized by great obstinacy. Fortunately, however, from the point of view of treatment, we do not stand helpless against migraine, rather in the great majority of cases are we able to favorably influence its course. As a general rule applicable to all migraine patients, it may be stated that a bland, predominantly lacto-vegetarian diet, with frequent taking of small portions of nourishment between the principal meals (see Lecture XXVIII, page 434) and abstention from alcoholic beverages, exercises a favorable in- fluence upon the number and intensity of the paroxysms. A distribution of work and rest as rational as possible, avoidance of all excesses, treatment of constipation, regular exercise in the open air, generous vacations spent when possible in the mountains, are further important prophylactic measures. Under medicinal treatment are to be mentioned in the first place, occa- sional courses of bromides recommended by Charcot, Grilles de la Tourette, Mobius, S. Auerbacli and others, in which several times a year for several weeks at a time, potassium bromide raised to a dose of from 3.0 to 6.0 grammes a day and then gradually reduced, is given. More efficient than the simple alkaline bromide appears to me the following combination recommended by Mendel: for 20 days at a time the patient takes every morning in a large * Headache, vomitinj;, slow pulse. MIGRAINE 461 cup of hot orange flower tea, a powder of the following composition: I! Sodii bromide, 2.5 (gr. 35); sodii salicylat, 0.25 (gr. 4); aconitini, 0.0001 (',,, mg. = Vfiiio grain). For prolonged treatment, the extract of cannabis indica is especially suitable; of this 0.015 (gr. ]-,) is given, best every evening for several months. The pil. cannabime comp. which I have recommended, has also been found useful by Curschmann and others. When necessary, the dose of hashish can be doubled or tripled without hesitation. The administration of ext. cannabis ind. in a maximum dose to cut short the attack ( 1J Pastae guaranae, 0.25 (gr. iv) ; caffein. citrat, 0.05 to 0.1 (gr. % to 1%) ; extract cannabis ind., 0.1 (gr. 1% in capsule), however, sometimes produces un- pleasant symptoms. Here the different anti-neuralgics (see pages 57 and 59) are to be preferred, particularly pyramidon, migranin, trigemin, aspirin, or the so-called ''mixed powders"; among the last I would mention specially the standard recipe of Kraift-Ebing : IJ Past, guaran., 0.2 (gr. 3) ; caffein citrat., 0.06 (gr. %o) ; codein phosph., 0.02 (gr. %) ; phenacetin, 0.5 (gr. 7/4); further, the equally efficient Edingcr's formula: Past, guaran., 0.3 (gr. 4%) : antipyrin, 0.5 (gr. 7%) ; caffein. citrat. 0.02 (gr. Yz) ; 2 powders at an in- terval of an hour as needed. Since the patients are only too much inclined to misuse "headache powders," the taking of them except for actual severe pain must be firmly opposed. Alternation between the several drugs, in order to prevent habit formation and the patient's raising the dose on his own respon- sibility, is greatly to be recommended. Only in very rare cases is it necessary to use the certainly acting morphine, for particularly severe attacks (status hemicranicus). Of course the patient should never be given the syringe and the solution; do not be afraid, however, to give a large dose; from less than 0.015 (gr, 74) a prompt action is not to be expected. We need not order the pa- tient during the attack to lie quiet with loosened clothing in a darkened room from which all noises are excluded ; he does this himself when it is necessary. Also, the patients know better than the physician whether cold or warm com presses to the head suit them best. As a prophylactic against the paroxysms, however, I recommend daily application of very hot compresses to the fore- head for a quarter of an hour after going to bed. This is an adequate sub stitute for the hot forehead douches recommended by Carron dc la Carridre (which are in practice quite complicated). From electrotherapy (only trans- verse galvanization through the head comes into question), on the other hand, not much is to In' expected. In order to prevent the "vomiting on an empty stomach," the patient should be urged to take some food during the attack; milk, to which strong black tea has been added, is usually besl tolerated. THE COPYRIGHTS 01 THIS BOOK, IN Ml. ENGLISH SPJ IKING COl NTRIES, \i;l OWNED BY REBMAN COMPANY, MEW YORK. Index to Authors Abadie, 177, 352 Ac-hard. 257 Addison, 34.5, 362 Airy, 458 Alzheimer, 85, 221, 231, 397 Anton, 321 Antonelli, 158, 460 Apelt, 187. 337 Aran. 107 Aran-Duchenne, 1 I 5 Aretaeus, 454 Argyll-Robertson, 183, 191, 202 Annauer, 1 17 Aschaffenburg, 451 Auerbach, 242, 102, 158, 159, 460 Babinski, 6, in. 134, 136, 147, 163, 214, 307, 308, 140 Ballet, 89. 352, 356 Baranv. 36 Bardenheuer, 1 5 Barensprung, 317, 385 Basedow, 315 Bastian, 272 Baudoin, 61 Baumann, 353 Bayle, 2t)<), 201 Beard, K)5, H9, 120, 131, 435 Bechterew, 52. 136, 163 Bell, 7-' Benda, 366 Benedikt, 72, 352 Berger, 157 Bergeron, so. 442 Bergman n, 295 Bergonie, 248 Bernhardt, I-'. 112 Biedl, 363 Bienfait, 353 Bier, 254 Biernacki, 177 Biervliet, W0 Binswanger, 231, 232, 396 Blrcher, !■'... 160, 341, 381, 402 Bircher, H., 341, 35? Blttorf, 363. 361 Bloch, 220, 379 Blocq, 89 Blumenthal, 356 , Boas, H9 Bolk, 315 Bonhoffer, 310 Bonn, 379 Bonnet, 52 Borchardt, 313 Bonrneville, 343, 393 Bramann, 3_'i Bramwell, 125 Braun, 198 Brissaud, 52, 6T, 178, ' 89, S67, I '■* Broadhurst, B2 Broca, 272 Broedel, 225 Brosius, 165 Brosse, 405 Bronardel, 13 Brown-Sequard, 65, 139, 156, 161, 362 Bouchard, 264 Brims. 138, 111 310, 379 Bryson, 349 Bumke, 202 Burghardt, 356 Buschan, 340 Calmeil, 457 Campbell, 385, 386 Carron de la Carriere, 461 Cassirer, 185, 303. 376, 377 Charcot, 2.3. 88. 105, 111, 117. 132, 135, 138, 139, 148, 178. 194, 226, 256, 264, 358, 399, ins. 410, 422. 436, 443. 414, 157, 160 Cheyne-Stokes, 180, 267 Chvostek. 76 C'lnnct, 89, 90 Coester, 3 Cooper, Astley, 501 Corning, 253 " Corti, 33 Corvisart, 71 Cramer. 106. 118 Cullen, 104 Curschmann, 95, :^>K 373. 371. 376. 379. 380, 156, 16 1 Cushing, 334, 367 Cuvier, 318 Damsch, 325 Dana. 309 Dax, 273 Dean, 317 Dejerine, 3, 113, 115, 124, 131, 136, 137. Is;,, 210, 226, 878, 279, 328, 373, 124 Demange, .'.'7 Desvault, L84 Dietschy, 379 Dieulafoy, .ir.ii, i.-,i Dinkier, 193 Dobrschansky, 208, 211 Don.illi. .'I I, 397 Down, 339 Dreschfeld, 310 Dubief, 89 Dubini, so Dubois, 67. 105, 130, 131, 1 1» I)n Bois- Fteymond, 166, 160 Duchenne, 73, 107, lis. [64, 17.'. .'37 Durdufl, 353 DUring, 378 Durkheimer, 89 Durand-Fardel, .'.'7 468 404 IXDEX TO AUTHORS Eckert, -'.".1 Edinger, 4, 44, 128, 160, 431, 458, 461 Ehrlich, 44 Eiehhorst, 12, 134 Eiselberg, 367 Elischer, 82 Ellis, 211 Eisner, 317 Enada, 193 Enriquez, 352, 356 Eppinger, 371 Erb, 9, 50, 76, 94, 97, 98, 104, 111, 164, 165, 214, 226, 325, 375 Erdheim, 75 Erlenmayer, 222 Esquirol, 202 Eulenburg, 91, 193, 352 Ewald, 358 Falret, 200, 427 Faure, 184 Feindel, 67 Fere, 457 Filelme, 353 Fischer, 211 Flajani, 345 Fla'tau, 456, 457, 458, 459 Flexner, 242, 243, 252 Forster, 116, 198, 335, 441 Forli, 316 Fothergill, 17 Fournier, 164, 184, 186, 320 Frank, 318 Franke, 198 Frankl-Hochwart, 77 Frenkel, 128, 197 Freund, 137, 330, 437 Freud, 279, 331, 332, 428, 419, 450 Frey, 127, 340 Friedlander, 211, 264 Friedreich, 73, 123, 213, 352, 384 Frund, 213 Fuchs, 187 FUrstner, 202 Gartner, 348 Galen, 38!), 154 Galton, 35 Ganghofner, 330 Ganser, 445 Garre, 352 Gaupp, 202 Gauthier, 89 Gendrin, 447 Generali, 75 Gerhardt, 149, 414 Gerlier, 93 Gersuny, 383 Gilles de la Tourette, 400, 460 Goppert, 250 Goldflam, 94 Goldscheider, 197 Gowers, 126, 128, 356 Graef'e, 317 Grasset, 226, 356, 455 Gratiolet, 295 Graves, 315 Griesinger, 427 Gull, 358 Gutzmann, 283 Haegler, 382 Hagenbach, 75, 92 Hanau, 366 Hansen, 147 Harbitz, 245 Hartenberg, 458 Head, 7, 385, 386 Hebra, 380 Heilbronner, 284, 285, 401 Heine, 159, 235 Heller, 37S Helmholtz, 318 Henoch, 80 Hess, 371 Hessing, 248 Heubner, 251, 255, 264, 335 Heuschen, 458 Hippocrates, 397, 405, 427, 436 His, 459 Hnche, 308 Hndskins, 401 Hoffa, 248, 335 Hoffman, 77, 105, 107 Holmes, 308 Homburger, 223, 230, 233, 349 Horner, 146, 149, 371 Huchard, 225, 350 Hiibscher, 248 Hughes, 73 Hunt, 37, 309 Huntington, 80, 84, 128 Hunziker, 381 Ibrahim, 332 Inada, 232 Iselin, 75, 95, 288 Jaboulay, 198, 357 Jaccoud, 417 Jacquet, 91, 420 Jakowenko, 82 .la net, 428 Jauregg, 211, 343 Jendrassik, 174 Jochmann, 252 Joffroy, 148, 205 Jolly, 91, 458 Joseph, 381 Josue, 222 Jusgen, 209 Kassowitz, 69, 79 Katzenstein, 310 Kleist, 285 Klieneberger, 303 Klipell, 185 Klippel, 231, 364 Klumpke, 17, 147 Knapp. 193 Knoblauch, 91, 207, 208 Koch, 300, 365 Kocher-de-Quervain, 70 Kocher, 321, 350, 353, 357, 358, 361, 402 K.'inig, 198 Kohler, 145 Kolle, 252 Roller, 407 Kolliker, 369 Kollovits, 128, 412 Kownlewsky, 340 INDEX TO AUTHORS 465 Kraepelin, 201, 205, 207, 210, 218 Krafft-Ebing, 200, 204, 210, 461 Krause (Fedor), 61, 288, 312, 313 Kron, 193 Kunn, 13ii Kussmaul, 202, 419 Kutscher, 252 Laliarraque, 156 Laborde, 71 Ladame, 231, 393 Laniy, 243 Landry, 42, 159 Landsteiner, 243 Lane, 339 Lange, 60, 61 Lannelong, 339 Lanz, 352, 3S6 Lasegue, 51, 53, 60, 448 Launois, 393 Lechner, 410 Legrand du Saulle, 394, 399, 427 Lupine, 356 Leredde, 210 L6ri, 186 Lctulle, 179 Leube, 350, 379 Levaditi, 241, 2i2, 243 Levico, 89 Levy, 61, 252, 343, 350, 352, 360 I.ewin, 37!) i .ru is, .' I .' Leyden, 128, 197 Lichtheim, 279 Liepman, 27K, 279, 283, 281, 285 Little, 116, 327 I.ivcing, 457 In,- 1,. 7S Long, 133 Louril, 315 Luciani, 315 Lundborg, 95 Lussona, :il 1 \l.i allnm, 78, 356 Macewen, 313 Mackenzie, 351 Madelung, 368 Magendic, 310 Mankowsky, 356 Mann, 77 Mannheim, 350 Man/, ."III Marburg, 368 Marie, 105, 128, 133, 327, 231, 342, .'73. 320, 366, 367 Marina, 104 Marinesco, 95, 98, I 16, 27:! Mil. ma, 3ii I Maxquelle, 89 ' Med in. 159, -'35 Meige, 67. 381, 159 Meltzer, 320 Mendel, 136. 163, 161, 193, 225. 256, WO \l rv. 138, 217 Mignot, 202 Mikulicz. 321 Mingazz.ini. 198, -'.'II. 311 Minkowski, :t Minkowsky, 1 I -' Miura, 93 Mobius, 165, 317, 439, 353, 356. 457, 458, 469 Mollendorf, 460 Moliere, 405 Monakow, 263, 273, 279 Moore, 211. 382 Morgan, 401 Morris, 382 Morse, 73 Morton, 53, 459 Morvan, 146 Moussu, 75 Miiller, 126, 136, 193. 232, 236, 391 Miiller, A., 459 Miiller, Fr., 350, 351 Munk, 316 Muskens, 391 Kageli, 425 Nagelschmidt, 248 Kagy, (i I Naka, 202 Neisser, 293 Xe Kam, 376 Netter, 241 Nensser, 363 Nicoladoni, 248 Nissl, 1S7, 244. 338 N'ognehi, 187, 211. 243 Nonne, 1 11, 186, 187, 202, 209, 213, 220 NorstrSm, 458 Nothnagel, 225, 309, 373 Nns.sliauin, 73 Obersteiner, 171, 203, 205, 207, 218, 301 Ollivier d' Angers, 143 Oppenheim, 4, 43, 45, 47, 52, 66, 72, 86, 89, 96, 121. 126, 133. 136, 139, 112. 163, 179. IS5. 191, 2(19. 2211, 227, 212, 247. 303. 3111. 313. 317, 351, 374, 396, 401, 410, 411, 115. 127, 417, 156, 157 Ord, 358 Ostwaldt, 61 Pal, 126 Pansini, 316 Parchappe, 200 Parinaud, 136. 138 Parisot, 202 Parkinson, 86, 116 Paxal, IBS I'avr. 321, 313 I'critz, .'.-..'. 158 I'ilcz. 211 Pineles, 75. 76, 77, 78, 310 Piorry, 158 Pitres, i!' 1 Placzek, 31:'. Pollack, .'93 Pool-Schlesinger, 76 Pouchet, -':'•-' Qudnu, 51 Quervain, 75, 293 (Quincke, 253, 321. 381, 382, 159 Qulnquaud, 63 lianzier. I'.'. Ravaut, 181 Ravmond, 198, l-'.. I"'.. 350, I >. 160 466 INDEX TO AUTHORS Raynaud, 373 Recklinghausen, 5 Redlich, 89, 171 Regnaiilt, 64 Reiehardt, 202, 288, 459 Reisinger, 383 Reitmann, 379 Remak, 3, 40, 314 Rethi, 136 Reumdht, 165, 193 Reverdin, 358 Richet, 399, 444 Rinne, 34, 185, 441 Robertson, 183 Rodari, 196 Roemer, 241, 243 Romberg, 164, 173, 191, 235, 383 Rosegger, 343 Rosenbach, 430 Rosenthal, 187 Rossolymo, 134 Roth, 42 Rothmann, 243, 310, 315 Roussy, 89, 90 Roux," 129 Runge, 319 Russell, 310 Rust, 163 Rynberk, 315 Sachs, 278, 339, 337 Sanger, 220 Sainton, 320 Salle, 159 Sander, 89 Scarpa, 198 Sehachnowiez, 93 Scheel, 245 Schiff, 185 Schleich, 60 Schlesinger, 146, 325, 373 Sehloesser, 61, 67 Schmidt, 290 Scholz, 350 Schrameck, 202 Schuller, 290 Schulthess, 248 Schultze, 112, 141, 145, 149, 246, 373 Schuster, 193 Schwabach, 185 Schwann, 6 Secretan, 382 Seeligmueller, 47, 217 Senator, 128 Srvigne, 405 Siegrist, 458, 460 Siemerling, 185 Smith, Frank, 3 Soea, 124 Somma, 320 Sonden, 420 Splelmeyer, 209, 370. 338 Spillmann, 192 Stahelin, 350, 379, 381 Stellwag, 347, 348 Sternberg, 367 Stewart, 281 Stierlin, 419, 440 Stintzing, 10 Stolting, 138 Strumpell, 78, 112, 113, 115, 133, 242, 246, 303, 329, 365 Stuart, 308 Swift. 211 Sydenham, 82 Talma, 91 Tay, 337 Tedeschi, 353 Theohari, 3 Thiemich, 77 Thiersch-Witzell, 61 Thirial, 377 Thomas, 124, 129, 133, 190, 315, 316 Thomsen, 90 Tigerstedt, 420 Timaeus, 436 Tissot, 403, 456 Tobias, 164, 193 Tooth, 105 Toulouse, 399 Trombert, 373 Trommer, 415 Trousseau, 48, 75, 76, 349, 350, 399, 331, 351, 393 Tuczek, 307, 208 Uhthoff, 138, 184 Ulrich, 400 Valleix, 46, 47, 49, 59, 60, 67 Yal Sinestra, 89 Vassale, 75 Vauban, 457 Yigouroux, 349 Villiger, 151 Virchow, 320, 339 Vogt, 329, 338, 340 Volt, 420 Vorschiitz, 254 Yulpian, 132, 262 Waller, 5, 157 Wanner, 384 Wassermann. 140, 164, 182, 187, 209, 252 Weber, 35, 185 Weigert, 95, 126 Weir-Mitchell, 40, 382, 435. 1 53 Werding, 107 Wernicke, 273, 378, 379, 280, 303 Wernitz, 324 AVertheim-Salomonson, 16 Westenhoffer, 248 Westphal, 141, 174, 191, 200, 207 Weygandt, 340 Wickman, 242, 245 Widal, 187 Wiesel, 363 Wille, 207 Williamson, 246 Willis, 36 •Windscheid, 222 Wolff, 201, 280 Wollenberg, 308 Wunderlich, 194 Wyss, 318 Zabludowski, 73 Ziehen, 86, 210, 309 Zupnik, 252 General Index A bad ins tabes, 177 Abdominal reflex, 8 Abducens, 31, 39 paralysis, 324 Abiotrophy, 1 28 Abiinisin, 3s7 Abscess of the cerebellum, 31 2 Abscess of the spinal cord, 158 Accessory nerve, 37 Accident' hysteria, 424, 446, 450 Accident neurasthenia, 424 Accidental autopsy findings, 301 Accoucheur's hand, 75 Achilles reflex, S Achillodynia, S3 Achondroplasia. 341 Acrocyanosis chronica anoesthetica, 377 Acromegaly, 3 Acute bulbar paralysis, 303 Acute decubitus, 262 Acute dilatation of the heart, 115 Acute hemorrhagic encephalitis, 303 Acute infectious disease of the central ner- vous system, 235 Acute poliomyelitis of adults, _> Hi Acute post-operative thyroidism, 352 Adenoma, 289 Addison's disease, 362 Adiadochokinesis, 308 Adipositas dolorosa, 387 Adipo.so genital degeneration, 3fi8 Adrenal insufficiency, 262 Adrenalin glj cosuria, 371 Aerophagia, 143 Affect spasms. I 15 Agenesis eorticalis, 337 Agenitalism, 368 Ageusia, 30, 37 Agnosia, 272, 286, 321 Agoraphobia, 127 Agraphia, 280, 285 Akinesia algera, 139 Ucoholic polyneuritis, 12 Alcoholism, :'.'. 39, 13, IS, 63, 7.'. 75, 128, 158, ■I :. :io::. 31 I. 317. :;."i. :iss. I'm,, i.'i;, | »i Alexia. 275, -"-'I Allocheiria, 170 Allorhythmia, 115 Alterations of handwriting, Alternating facial hemiplegia. .'70 Alternating oculomotor hemiplegia, 270 Amaurosis. 26, 158 Amaurotic idiocy, 3.37 Ambidextrism, 336 Amblj opia, 26 Ambulatory automatism, 394 American nervousness, l_'| Americanization. 421 Amimi i. 280 Amnesic aphasia, 206, 279 Amputation lines, I lo Amusia, 280 Amyotrophic lateral sclerosis, 116, 147 Anacusis, 33 Anadrosis, 9 Analgesia, li Anal reflex, S Anaphrodisia, 1J| Ancle clonus. 1 1 I. 1 17, 163 Anemia of the brain, 304 Anesthesia, (i, 28 circular, 140 disseminated. 1 10 geometrical, 110 insular. 11(1 universal, 410 Angina pectoris, 373, 415 Angio neuroses, 369 Angio-paralvtic migraine, 456 Angiospastic dysbasia, 37 1 Angio-spastic migraine. 156 Angio-spastic symmetrical gangrene. 37. Anidrosis, 372 Anisocoria, 183, 202 Anomalies, of metabolism, 350 of the external ear, 394, 107 Anorexia, I 13 Anosmia, 26 Anospinal center. 155 Ansa hj poglossi, 3s Anterior polj neuritis. 235 Anticipatory heredity, si Antineuralgics, 57, 59, Iff", Antispasi lica, <>T Anxietas tibiarum, 1 1 J Anxiety neurosis, t.s Ape hand, 108, 1 18, l ID Aphasia. 205, -'7.'. 279, 158 i oi Heal motor. 276 Cortical sensory. .'77 of single senses. 27i» subcortical motor, 277 Wernicke's, .'77 Apoplectic attack, \ [loplectic ci st, 16 I Apoplectic scar, 264 Apoplectiform alcoholic paralysis, \2 Apoplexie f Iroj anti Apoplexy, 162 Ingravescent, -' Arteria sulco commissuralis, 213 Arteriosclerosis of the nerve centers, 222 Arteriosclerosis of the spinal cord, 286 Arteriosclerotic age. 22S Arteriosclerotic changes in the brain vessels, 263 Arteriosclerotic neurasthenia, 222 Arteriosclerotic neuritides, 42 Arteriosclerotic paresis, 232 Arteriosclerotic pseudo-paralysis, 232 Artery of cerebral hemorrhage, 25(i Arthogryposis, 78 Arthritic diathesis, 455 Arthrodosis, 107, .Ms Arthropathies, 178 Ascending degeneration. 213 Asphyxie locale symmetrique, 375 Associated movements, 111, 261, 330 Astasia-abasia, 447 Astereognosis, 295 Asthenopic troubles, 411 Asthma, 416 Vstigmatism, 395 Asymbolia, 280, 283 Asynergy, 7 Asystematic affections, 166 Ataxia, 7, 124, 172, 307 cerebellar, 242 Athetoses, 85, 331 double, 85, 332 Athvreosis, 358 Aloiiia, 8 Atrophic excavation, 27 Atrophies from overuse, 3 Atrophy, individual, 108 from disuse, 1 13 of nurslings, 302 of the papilla, 205 Atypical forms of multiple sclerosis, 139 Atypical hemiplegias, 270 Auditory, 33 agnosia, 286 aphasia, 280 Aura cursatoria, 389 sensory, 75 Auriculo temporal point. 48 Automobilist cramps, 72 Autonomic nervous system, 369 Autosuggestion, 431 Avaricious concepts, 425 Avertissements sans frais, 226 Aztec type, 339 Babinski phenomenon. 111. 124, 163, 226 Babinski reflex, 212, 261. 300, 330 Babinski toe phenomenon, 390 Balkenstich, 321 Ballet-dancer's cramps, 72 Balneotherapy, 136, 147, 196 Barany's test", 36, 309 Basedow's disease, 345, 364 Basedow's goiter, 346 Basedoi'jified goiter, 352 Bathyanesthesia, 10 Bathyesthesia, 7, 156 Bathyhypoesthesia, 10 Bechterew's sciatic phenomenon, 52 Bed-sore, 321 Bell's paralysis, 31 Being possessed, 388 Being seized, 388 Benign hypo-thyroidism, 360 Beri-beri,' 5, 13' Biceps reflex, 8 Bier's boxes, 57, 380 liii nmrk's symptom, 177 Bird head, 339 Blahals, 445 Blastophthoria, 128, 396, 407 Blepharoclonics, (Hi Blepharospasms, 66 Blindness for letters, 280 Blood changes, 350 Blood pressure, 276 Bloodletting, 267 Bloody nerve stretching, 60 Blowing smoke, 262 Blue oedema, 443 Bone reflex, 8 Bonnet's phenomenon, 52 Bounding mydriasis, 202 Bounding pupils. 183 Bouton diaphragmatique, 49 Brachialgias, 50 Brachial neuralgias, 50 Brachial plexus paralysis, 16 Braehioradialis, 14 Brachium conjunctivum, destruction of, 310 Bradycardia, 415 Brain, abscess, 297 disturbances of circulation in, 288 inflammations, 288 massage, 402 pressure, 289, 321 swelling, 288 tumor, 288 Broca's aphasia, 273, 276 Broca's center, 273 Broca-W ernicke localization theory, 273 Bromide therapy, 399, 460 Bronze diabetes, 364 Bronzed skin, 363, 364 Brown-Siquard paralysis, 156 Brown-Sequard symptom-complex, 139, 155, 161, 219 Bulbar paralysis, 122 Burdach's column, 168 Cachexie pachydermique, 358 Cachexia strumi-priva, 358, 361 Caisson paralysis, 159 Caloric nystagmus, 309 Capsular hemiplegia, 256 Caput obstipum spasticum, 68 Caput progeneum, 407 GENERAL INDEX 469 Carcinoma, 289 Carioptosis, 415 Cardio-vaseular disturbances, 111 Cardio-vasomotor neurasthenia, 418 Carrefour sensitif, 257 Catalepsy, 1 15 Cellist's cramps, 72 Center for the memory of sounds, 272 Central gliosis of the spinal cord, 143 Central pain, 295 Cephalaea, 454 nodular, 459 rheumatie, 459 Cephalagia, 454 Cephalones, 320 Cerehellar affections, 306 Cerebellar asynergy, 307 Cerebellar attacks,' 309 Cerebellar ataxia, 307 Cerebellar fits. 309 Cerebellar heretio ataxia, 128 Cerebellar hypotonia, 308 Cerebello-pontine seizure, 309 Cerebellum, agenesis of, 315 atrophies of, 315 diseases of, 306 hemorrhages of, 314 infectious diseases of, 316 softenings of, 314 tumors. 506 Cerebral atrophy, 261 Cerebral embolism, 265 Cerebral glosso-pharyngo-labial paralysis, 229 CVn-bral hemiplegia, 256 Cerebral hemorrhage, 255 Cerebral meningitis, 328 Cerebral monospasm, 293 Cerebral muscular atrophy, 33:) ( lerebral neurasthenia, 1 1^ Cerebral porosis, 227 Cerebral softening, 156 Cerebral vomiting, 290 Cerebro-cardial neuropathy, to.'. Cerebro-cerebellar atrophy, 315 Cerebrospinal atrophy, 315 fluid, 187 syphilis, .'15 Charcot's disease, 117 extension type, 88 Charcot-Marie type of progressive muscular atrophy, 105 Chemical chamisole, -'I I Cheyne-Stokes' respiration, 267, !92 Chloride of gold, [94 Cholesteal a. 289 Chondrodystrophy, foetal, 341 Chondroma, 289 Chordoma, 289 Chorea, 310. XU bodies, 82 electric, I I .' gravidarum, s -'. 85 hereditary . s ' Huntington' i, 84 hysterical, s " major, 80, L4S minor. 80 Mnllr's. 83 paral] tica, 82 sancti viti. 80 Chorea. Sydenham's, 80 Choreiform diseases, 80 movements, 350 Chromaffin system, M2 Chronic anterior poliomyelitis, 2\:t form of neurotic oedema, 381 ( 'hvostt k'.t sign, 77 Cigarmaker's cramps, 72 Ciliary neuralgia, 17 Cilio-spinal center, 146 Circulatory disturbances of the brain. 304 Circumduction, 113, 260 Circumflex nerve paralysis, 19 Cirrhosis of the chorium. 379 Clarinetist's cramps, 72 Claudicatio intermittens, 125 Claustrophobia, 427 Clavus hystericus, 138 Clawfoot. 23, 116 Claw hand, 20, 108, 118, 145, 149, 239 Cleft palate, 407 Cleido-cranial dysostosis, 320 Clitoris crises, 1st Clonograph, 413 Clonus, 111 Clouding of consciousness, 394 postconvulsive, 394 postepileptic, 394 postparoxysmal, 394 preconvulsive, 394 preepileptic, 391 preparoxysuial, 394 Clownism, 1 1 1 C'ocainism, 396 Coccygodynia, 53 Cochlear nerve, 33 Coin counting, 81 Cold water cures, 355 Collateral ganglion system, 370 Color scotoma, 26 Colored vision. 389 Combined tabes, 215 ( Composite movements, 316 Compression paralysis, -' Compulsion neurosis. 138 Compulsory crying, 13S laughing, 13S movements, 310 Concentric limitation of the visual Held, WO Concentric narrowing of the visual field, -'i> Conci pi center. J86 Conceptual center, 273. .'71 Conception of movement, J06 Concussion of the brain, 263, 304 of the spinal curd, 150 Conduction aphasia, 279 Conduction, disturbances of, .' symptomatology . >' Congenital, acquired defective conditions, 317 bulbar paralysis, ::.'i criminal. In; muscle defects, 324 muscular atony, 92 nucle tr defects, 334 ptosis. 39 i syphilis. 339 Congestion of the brain. 304 Conjugate Ae\ iation, 310 Conjunctival reflex, 29 Consensual light reaction, 183 470 GENERAL INDEX Constitutional neurasthenia, -125, 4J(i Contracture diathesis, 443 Contracture rheumatismale des nourrices, 75 Conversion, 4+9 Cooperation movements, 307, 310 Coordinatory occupation neuroses, 72 Corneal reflex, 29 Corpus callosum, defect of, 337 Corpus callosum, puncture through the, 321 tumors of, 295 Cortical alexia, 280 Cortical apraxia, 285 Cortical attacks, 218 Cortical epilepsy, 205, 218, 293 Cortical hemiplegia. 270 Cortical paralysis, 293 Cotugno's disease, 51 Coups de hache, 102 Cracked pot sound, 290 Craemer four groups of phenomena, 448 Cranial ectopies, 321 Cranial nerves, 26 Cranial paralysis, 38 Cranianiphitomie, 339 Cremasteric reflex, 8 Cretinism, 341 Cretinistic degeneration, 341 Cretinistic idiocy, 341 Cretinoids, 343 ' Crises comitiales, 388 Crises noires, 180 Crises of emaciation, 350 Critical situations, 431 Cyclothymia, 429 Cystic degeneration, 227 Cysticercus, 289 Cysts of softening of the brain, 328 Dammerzustand, 394 Daktvlographer's cramps, 72 Danse de Saint Guv, 80 Dead finger, 376, 377, 441 Deafness, 33 Deceptive front, 231 Decompressive craniectomy, 296, 312 Defatigatio, 423 Defective conditions, 317 Degenerative neurasthenia, 426 Degenerative processes, 9 Dejjerine-Lichiheim phenomenon, 277 Delire du toucher, 428 Demarche a petit pas, 229 Dementia myoclonica, 74 Dementia paralytica, 121, 205 Dementia praecox, 429 Demoniacal attacks, 445 Dental points, 48 lii ream's disease, 387 Derivatives, 70 Dermatotomes, 175 Dermographia, 250 Dermographism, 347, 416, 441 Dermoid cysts, 289 Descending degeneration, 213 Desire for excitement, 1 13 Development of the eortico-spinal tracts, 328 Deviation conjuquee. 262 Diaphragm crises, 180 Diaphragm spasms, 71 Diarrhoeas, 349 Diaschisis, 149, 263, 273 Diaschisis, theory, 273 Diathermy, 248 Diffuse diseases, 111 Diplegia, 7 facialis, 31, 94, 217 masticatoria, 30 spastica infantilis, 330 superior, 236 Dij) on the parallel bars, 326 Dipsomania, 394 Direct light reaction, 182 Disseminated neuritides, 41 Disseminated sclerosis, 132 Dissociated anasthesia, 145 Dissociated disturbances of potence, 137 Disturbances of menstruation, 349 Doubting mania, 427 Divergence of the eyeball of Magendie, 310 Diver's paralysis, 159 Dorsal foot reflex, 114 Douleurs en brodequin, 180 Douleurs fulgurantes, 180 Douleurs lancinantes, 180 Drawling speech, 203 Drop foot, 23 Drummer's cramps, 72 Duchenne-Erb type of paralysis, 17 Duchenne-Griesinger type of dystrophy, 102 Dwarfism, 341 Dysbasia angiosclerotica intermittens, 225, 373 Dysbasia lordotica progressiva, so Dysbasia neurasthenica intermittens, 412 Dyschromatopsia, 441 Dysglandular symptom-complex, 345 Dysglandular syndromes, 367 Dyskinesias, 63, 80 Dyskoimesis, 410 Dysmetria, 172 Dysnystaxis, 410 Dysphylaxia, 410 Dyspinealism, 295, 368 Dyspraxia angiosclerotica intermittens, 373 Dystrophia musculorum progressiva, 97 Dystrophy, progressive muscular, 325 Dysthyroidism, 353 Early acquired defective conditions, 317 Echinococcus, 289 Echolalia, 71 Echopraxia, 71 Eclampsia in infants, 77 Eclampsia infantium, 388 Ecmnesia, 445 Elbow clonus, 114 Electivity, 42 Electric chorea, 80 Electric hand, 105 Electrodiagnosis, 10, 77, 91, 95 Electro physiology, 10 Electrotherapy, 55, 60, 67, 196. 217, 269, 335, 355, 375 Embolism of basilar artery, 121, 147 Encephalasthenia, 418 Encephalitis, congenital, 327 non-suppurative, 302 post partum, 328 subcortical chronic progressive, 232 Encephalocele, 321 Encephalomalacia, 256 Encephalopathia addisonia, 364 Encephalorrhagia, 256 GENERAL INDEX 471 End arteries, 256 Endarteritis, syphilitic, 217, 329 Endarteritis syphilitica obliterans, 364 Endocrine glands, 315 Endogenic fibers, 170 Endogenic fields of the posterior columns, 170 Endothelioma, 289 Endotbeliome en nappe, 289 Enophthalmus, 30, :S71 Enuresis, 324, 426 Ependymitis, 317 Epidemic cerebro-spinal meningitis, 218 Epigastric reflex, 8 Epilepsia convulsiva, 389 major, 389 non-convulsiva, 392 Epilepsie marmottante, 392 Epilepsy, 242, 388 Epileptic, aura, 3S9 equivalents, 393, 454 paranoia, 395 syncope, 393 vertigo, 392, 393 Epileptogenic zone, 396 Epithelioma, 289 Erl> type of dystrophy, 102 Erb-Goldflam disease, 94 Ergotism, 373 Erlenmeyer's mixture, 398 "Ersatz" theory, 44 Erythromelalgia, 382 Erythromelalgic attacks, 382 Erythromelia, 383 Ereuthophobia, 416 Erythroprosopalgia, 382 Ettnarch bandage, 2 Essentia! infantile paralysis, 235 Etat de fromage de Gruyfere, 227 lacunaire du cerveau, 227 vermoulu, 22'i tftat second, 391 Exaggerated perception, H5 Examination neurasthenia, 123 Exhibitionism. 394 Exophthalmus, 346 Exploratory laminectomy, 162 Extracapsular hemiplegias, 270 Extramedullary tumors, 161 Kxtra systoles.' 415 Extremities, spasms of, 71 I ye muscle, paralysis in Basedow's disease, 348 Facial cramps, 66 Facial hemiatrophy, 38 I Facial hemihypertrophy, 383 facial hemispasm, tS8 Facial nerve, 31 Facial paralysis, 3. 3t, 324 Facies myopathica, 10] Factitious urticaria, H6, in Falling sickness, 388 Fatigue pain, 1 1 - Fetichism, ill Fibers in the posterior r Fibrillary contractions, I Hi. ill tremor, 106 twitchings, 203 Fibroma, 289 Finger clonus, 11 1- 167 His, I1H. 119, Finger-finger test, 173 Finger-nose test, 173 Flail joint, 2:19 Flea-bite encephalitis, 303 Flechsig's bromide opium cure, 400 Fliegende Roeten. 116 Flutist's cramps, 72 Foci of softening of the brain, 328 Forster's operation, 335 Forster's type of alteration, 441 Fcetal chondrodystrophy, 341 F'olie du doute, 427 Folie musculaire, 81 Forced attitudes, 310 Formes frustes, 346, 360 Fothergill's face pain, 47 Four cell baths, 374 Four reactions, 329 Four reactions of Nonne, 209 F'ree warnings, 226 Friedri ich's disease, 123 Friedreich's foot. 125 Frohlich's disease, 368 Front olympien, 320 Frontal headache, 290 Full feeding cure, 199 Fully developed psychoses, 205 Funiculus cuneatus, 212 Funiculus gracilis, 212 Functional ankle clonus, ll:( Funnel chest, 395 Qalton's whistle, 35 Galvanic irritability, 13 Galvanic vertigo. 36 Gampsodactvlism, 107 i langliated cord, 369 Oanser's symptom, 115 Gasserian ganglion, extirpation of, 61 Gastralgokenosis, 1 19 Gastric crises, iso, 196 Gastrorrhea, acid. 1 16 General hyperesthesia, 405 General rigidity, :i'M Genickstarre, 249 Genital disturbances, 186 Genu recurvatum, 173 Gephyrophobia, 106, 127 t ;> rating's method, \\*:\ Giantism, 367 Girdle pain, 180 Glandula pituitaria, 366 Glanzauge, 346 Glassworkers, 2 Glioma, J88 Gliosarcoma, 288 Gliosis, spinal. ISO Glistening eye. 316 Globulin reaction. 1S7 ( llobus hj stericus, 1 13, 1 1 1 Glossolabial hemispasm, lis Glossoplegia, 37 ( llossopharyngeus, 36 ( llossospagms, 67 Glossj skin. 13, I 16. 216, tso Glotzauge, 346 ( tlotzaugenkrankheit, 315 Gluteal point. 51 Gluteal reflex, 8 1 1 ■ . • r \ c . mi Goiter, 346 472 GENERAL INDEX Goitre exophthalmique, 3-15 Goll, column of, 168 Gordon's symptom, 82 Gothic palate, 407 Graefe'a sign, 347 Grand ecart, 173 Grand hysteria, 444 Grandiose delusions, 204 Graphospasms, 72 Grave's disease, 345 Gray communicating branches, 370 Gray rami communicantes, 13 Gumma, 289 Gummatous meningitis, 216, 218 Giirtelrose, 384 Gymnastic exercises, 55, 67, 197, 247 Habit pains, 438 Habitus apoplecticus, 264 Half cretins, 343 Hammer palsy, 3 Hand and finger centers, 275 Hand walker, 241 Haphalgesia, 439 Hard traumatic oedema, 382 Hardness of hearing, 33, 35 Harelip, 407 Haut mal, 389, 392 Headache, 216, 223, 410, 454 Headache in cerebellar affections, 306 in purulent cerebral meningitis, 298 Headache, hysterical, 437 neurasthenic, 410 Headache nodules, 459 Heart crises, 180 Hederotopia, 165 Heine-Media disease, 235 Helicopodia, 113, 260 Hemato-ethyroidine, 356 Hemialgia, 439 Hemianesthesia, 440 Hernia nopsia, 26, 258, 458 Hemiataxia, 308 Hemiathetosis, 242 Hemiballism, 80 Hemichorea, 80, 242 Hemicrania, 456 cerebellar, 457 continued, 457 permanent, 457 simple, 458 simplex, 455 Hemicranic predisposition, 459 Hemi-epilepsy, 391 Hemiglossoplegia, 38 Hemihypotonia, 308 Hemiparaplegia, 157 Hemiplegia, 7, 218 atypical, .'TO cortical, 270 cruciata, 270 extra capsular, 270 homolateral, 271 lacunar, 271 peduncular, 270 pontine, 270 sine materia, 271 spastica infantilis, 330 spinal, 157 Hemming in of affects, 450 Hemorrhages into the brain substance, 328 into the spinal cord, 32(1 Hemotomyelia, 149 Hereditary family ataxia, 123 Hereditary predisposition, 407 Heredosvphilis, 329, 333, 396 Herpes, 250 ophthalmic, 386 zoster, 384 Hip flexion phenomenon, 261 Hippus, 81, 136, 183 Hoffmann's phenomena, 77 Homochronous heredity, 127 Homolateral hemiplegia, 271 Homologous heredity, 127 Homosexuality, 444 Hormone, 345 Homer's symptom complex, 146, 149, 371, 372, 376, 379 Huntington's disease, 85 Hydatid cyst, 289 Hydrencephalocele, 321 Hydrocephalic cry, 249 Hydrocephalic idiocy, 337 Hydrocephalus, 317," 329 external, 317 ex vacuo, 317 internal, 317 meningeal, 317 Hydrocephaly, 317 Hydrophobia, 68 Hydrotherapy, 233, 355, 375, 435 Hygiama, 268 Hypacusis, 33 Hypalgesia, 6 Hypalgesia dolorosa, 40 Hyperacusis, 32 Hyperalgesia, 6 neurasthenia, 437 Hyperaphrodisia, 421 Hyperemisis gravidarum, 4 Hyperemia of the brain, 304 Hyperepinephria, 364 Hyperesthesia, 6 of the hairy region, 438 Hyperfunction of the pituitary gland, 368 Hyperidrosis, 9, 349, 372 Hyperkinesias, 65 Hyperparathyroidosis, 89 Hyperpituitarism, 366 Hyperreflexion, 8, 113 Hyperthyroidism, 353 Hypertonia, 8, 113 Hyperesthesia dolorosa, 40 Hypnalgias, 411 Hypnosis, 452 Hypoacusis, 34 Hypochondria, 405 Hypoesthesia, 6 Hypoepinephria, 364 Hypogenitalism, 368 Hypogeusia, 30 Hypoglossal nerve, 38 Hypoglossus, 38 bilateral paralysis, 38 Hypoidrosis, 9 Hypophyseal eunuchism, 368 Hypophyseal insufficiency, 368 Hypophysis, 367 tumor, 366 GENERAL INDEX 473 Hyporeflexia, 8 Hyposmia, 26 Hypotaxia, 7 1 l\ pothyreosis, 358 Hypotonia, 8, 173, 308 Hysteria, 137, 209, 43.; Hysterical, anorexia, 4 1- 1 aphonia, 447 arthralgia, 438 attacks, 4 13 aura, 444 harking cough, 445 contractures, 448 fever, 443 hemorrhages, 443 hyperthermia, 443 longings, 411 mutism, 447 swollen neck, 445 paralyses, 446 pemphigus, 443, 451 pregnancy, 443 psychoses, 451 stigmata, 437 ulceration, 443 Hysterofrenatory zones, 438 Hysterogenic zones, 439 Hysteroneurasthenia, 405 Ideational apraxia, 206 [deatory apraxia, 284 Ideo-kinetic, 285 ataxia, 284 Idco-motor apraxia, 284 [deomotor pupillary reaction, 183 Idiocy, 327, 335, 336 Idio-muscular contraction, 350, U3 Idiopathic facial paralysis, 39 Idiopathic forms of spasms, 65 Ileosacral point, 51 Imbecility, 336 Impairment of speech, 279 Imperativi conceptions, 127 Impotence, 286, t-'l Incontinence, intermittent, 155 of bowels, I Mi Of urine. ISfi Incontinence, permanent, l.jj Incoordination, 7, 123 Increased irritability of the neuro-mnsenlar apparal us. 413 Indurative headache, 158 Infantile. Cerebral palsy, 330 cerebral pals} without paralysis. 333 cerebral paralj sis, ji j multiple sclerosis, 134 muscular at rophy, 325 Invxtcdcloa. 341, 359 pareses, 201 ' progressive hypertrophic neuritis, l:',l spastic diplegia, 327 spastic hemiplegia, 327 Infantilism. 337. 368, 107 Inflai atory atrophy, 27 Inline,, /a, 303 I nfraoi bital point, is Inhibition. I I!) Injection therapy in facial cramps. 117 in neuralgias. 60 Innervation apraxia, 885 Instrumental amusia, 2S3 Intellectual grand mal, 393 petit mal, 393 Intention spasm, 76 Intention tremor, 134, 135, 311 Intercostal neuralgia, 50 Intermittent claudication of the spinal cord. 226 Intermittent dropsy of the joints, 382 Intermittent limping, 225, 373, 371 Internal secretion, 341, 345 Internal speech, 275 Interparoxysmal anomalies, 394 Intestinal crises, 181, 196 Intoxication tetany, 75 Intracortical hemiplegia, 270 Intramedullary tumors, 16] Intra-meningeal hygroma, 317 Intra-social imbecility, 336 Iodglidine, 193 Iodide, 193 intolerance, 221 Iodine, 193 Iodine Basedow, 353 Iodipin, 193 Iodism, 221 Iodon, 193 Iodostarin, 193 Iodotherapy, 193, 210. 321, -'33 Iodtropin, 193 I ri tic migraine, 458 Irritable breast, 50 Irritable testis, 53 Irritable weakness, 420 Irritative symptoms on the part of the sense organs, 411 Irritative weakness, 412 Ischemic disturbance of sensation, 373 Ischuria paradoxa, 137, 155 Jacksonian epilepsy, 218 Jacksonian epileptic attacks, 293 Jargon aphasia, 278 •law spasms, 65 Jendrdsoik'a maneuver, 171 Jiu-jitsu, 1 19 Jughandle cars, 394 .1 umping jack, si .lumping jack limbs, 237 Juvenile paresis, .'01 Juvenile scapulo humeral type, 98 Kakke, 5 Kalmuck type 339 ECeral ii is, neuroparalytic, 5(> Kt mig'a sign, 249, 300 ft i rnig't sympi 198, 30 ' Kinesthetic memorj picture, -'"3 K i, a, therapeutic baths. 55 Klumpke's paralysis. 17, 117 Koprolalia. 71 Knbisagari, 93 I aciinte, -'-'7 lacunar hemiplegia, .'-'7. jt i I agopbthalmus. 32 I .aneinaling pains. ISO Landow y lh j, i i,u type of dystrophy, 109 I, mill ni's paralysis, I-'. 159 /,„„, lannelongue operation. 339 I,iiiiiii 's method, 60 474 GENERAL INDEX Laryngism, 68 tabetique, 185 Larynx crises, 180, 196 Larynx spasms, 68 Lasagne's sciatic phenomenon, 51 Late epilepsy, 396 Latent heredity, 127 Latent tetany, 76 Lateral ganglion system, 370 Latero-pulsion, 88 I.athyrism, 112, 213 I.athyrus cicera, 112 Lathyrus sativa, 112 Laughing spasm, 145 Ivead neuritis, 43 Lead paralysis, 43 Lead poisoning, 4, 39, 42, 72, 75, 112, 133, 158 Leg phenomenon, 77 Leichens tern's phenomenon, 249 Leichens tern's sign, 300 Lepra, 4, 147, 410 Lethargic attacks, 445 Letter games, 282 Lii/ihn-M a bins type of dystrophy, 102 Limb kinetic apraxia, 285 Liniments, 58 I.ipiodin, 193 Lipodystrophy, progressive, 387 Lipoma, 289 Literal aphasia, 278 Little's birth palsy, 332 Little's disease, 327 Liver crises, 181 Lobar sclerosis, 327, 329 Local asphyxia, 376 Local poisoning, 4 Local spasms, 65 Local syncope, 376 Localized muscular spasm, 70 Lockjaw, 65 Loose shoulder, 101 Lordosis, 101 Louise Bryson's sign, 349 Lower complexus paralysis, 147 Lues cerebrospinalis, 131, 164 Lues nervosa, 165 Lumbar neuralgia, 50 Lumbar point, 51 Lumbar puncture, 293, 320 technique of, 253 Macropsia, 389 M ml el nun's disease, 387 Mai comitial, 388 Mai perforant, 179 Malade imaginaire, 405 Maladie des tics, 71 Malar point, 48 Malformations, 317 Malleolar point, 51 Malleus maleficarum, 441 Malum coxae senilis, 53 Mammary point, 438 Man-crazy, 436 Mandibular branch, neuralgia of, 47 Mandibular reflex, 28 Marantic confusion, 207 Marantic tabes, 179 Marantic thrombosis, 302 Marginal gliosis, 397 Marinesco's succulent hand, 146 Massage, 55, 60, 247 Mast cure, 71 Masticatory spasms, 65 Mastodynia, 50 Masturbation, 421 Maternal metrapectic inheritance, 98 Maxillary neuralgia, 47 Median paralysis, 3, 19 Median peripheral field of the posterior col- umn, 170 Medullary crises, 181 Melancholia, 429 Melanodermia, 363, 395 Membres de polichinelle, 237 Memoire verbale, 272 Mi iiilil-Heehtereir phenomenon, 114 Mi nilel-Bechterew reflex, 136, 163, 261 Miniiri's attacks, 217 M entire's disease, 138 Meningitis, 251 cerebro-spinalis siderans, 251 of the convexity, 298 purulent cerebral, 298 serous, 162 Meningocele, cranial, 321 pedunculated, 321 spinal, 322 Meningococcic endocarditis, 250 Meningococcus intracellularis, 248 Meningococcus sera, 252 Meningo-encephalitis, 217 Meningo-myelitis, 217 Mental point, 48 Meralgia, 42 Mercurial tremor, 140 Mercury, 193, 220, 334 Metatarsal point, 51 Metatarsalgia, 53 Micromania, 205 Microcephalus, 317 Microcephaly, 338 Micro-encephaly, 338 Microgyria, 329 Micromelia, 341 Micropsia, 389 Migraine, 454 equivalents, 459 facioplegic, 458 gustatory, 458 of the arthritic, 458 olfactory, 458 ophthalmic, 458 otic, 458 prosoplegic, 458 red, 456 white, 456 Miliary aneurism, 264, 314 Migrating erysipelas, 160 Millnril-Onbler symptom-complex, 270 Mimic cramp, 66 Mind blindness, 286, 336 Mind deafness, 286, 336 Minor epileptic attacks, 392 Mixed nerves, 24 Maebius sign, 347 Moist skin, 349 Molimina climacteria virilia, 225 Momentary absence, 393 Mongolism, 341 GENERAL INDEX 475 Mongoloid idiocy, 339 Monkey's poliomyelitis, 2+3 Monkey's spinal paralysis, 243 Mononeuritides, 41 Monoplegia, 7, 294 brachialis, 236 cruralis, 236 • facialis, 31 facio brachialis, 21S masticatoria, 30 Morbo di Fiajani, 34.5 Merlins comitialis, 388 Mori ms sacer, 388, 393 Morbus vagabundus, 364 Moria, 294 Morning paralysis, 241 Morphinism, 388 Mortal depression, 409 Morton's disease, 53 Morton's metatarsalgia, 459 Motility, 7 Mntur ataxia, 284 Motor points, 16 Movable heart, 41.5 Mucous colic, 116, H3 Multiple necrotic gangrene of the skin, 377 Multiple sclerosis, 132, 142, 162, 2(19, 311 Musei volantes, 411 Muscle wave, 91 Muscular ankylosis, 113 Muscular atrophy, 97, 21.5 Muscular defects, 101 Must ular madness, 61 Muscular spasms, 65 Muscular weakness in the lower extremities, 3*8 Muttering delirium, 300 Myalgic migraine, 158 Myasthenia, 93, l JO Myasthenic reaction, 95 Mydriasis, is:; Myelitis, 1.59. 246 Myelocystocele, 32:: .Myc.l,, dysplasia, 321 Myeloencephalitis, disseminated, 140 Myelomeningocele, 323 Myoclonias, 73 Myoclonus, 395 epileps} . '!'■'• Myokymia, l<>. II t Myopathia, primitive progressive, 97 rachitica, 9.' Myopathic forms of muscular atrophy, 93 Myosis. IS2. 371 Mj ospasia com ulsiva, 71 Mythomania, 1 19 Myotics, is.' Myotonia, 90 acquired, 91 atrophic, 91 congenital, 90 Myotonic reaction, 91 Myotomv, 70 Myxoede'ma, 353, 358 congenital, 358 infantile, ; .- operative, 36 1 spontaneous, 358 Nanocephaly , 339 Narcoleptic attack, 393 Nasal crises, 181 point, 48 Natal lesions, 32S Neck, spasm of muscle of, 68 Neighborhood action. 368 Nerve crural paralysis, 31 deafness, 185, 441 mucus, 417 suprascapular paralysis, 17 thoracic paralysis, 17 Nervi nervorum, 41 Nervosism, 105 Nervosity, 10.5 Nervous erythism, 405 Nervous diarrhoea, 419 Nervous dyspepsia, 418 Nervous heart weakness, 11.5 Nervous tachypnoea, 116 Neural form of progressive muscular atrophy, 105 Neuralgia, 45 major, 48 minor, 49 nocturnal, 47 of the feet, 53 spermatic, 53 Neuralgic phenomena, 349 Neurasthenia, 208, 107 accidental, 422 acquired, 122 congenital, 422 constitutional, 122 cordis, 414 sexual, 420 true, 122 Neurasthenic arthralgia, 112 Neurasthenic helmet, I lo Neurectomy, 70 Neurexaresis, 61, 198 Neuritic processes, 3 Neuritides, 3 Neuritis, 3, 40 optic, 27, 217 sciatic. .52 Neurogenic degenerative atrophy, 9 Neurolytic injections, lil Neuromata, 5 Neuromatosis. .5 Neuropathic diathesis, 104, 407 Neuropathic heredity, 165 Neurorecidiv, 386 Neurorezidive, 220 Neurotabes peripherica, 193 Neurotic drops} . 3SI Neurotomy, * » I Neurol rophy, II. 320 Nevralgie des cdentcs, t" Newspaper folder's cramps, 72 Nicotinism, I-'. 115 Nictitation, 66 Nighl terrors, I J6 Nocturnal epilepsy, 391 Nodular headache, 168 Nonne-Apelt reaction, is; Normal elect mile 10 Nuclei of the posterior column, 168 Nj ctalgias. I I I \\ itagmus, 36, I 'i. 135, 309 Nvstagmiis-niyiicloiii.i. I ' 47(3 GENERAL INDEX Object agnosia, 286 Obsessions, 427 Occipital neuralgia, 49 Occupational spasms, 72 Oculo motor nerve, 27 paralysis, 28 (Edema of the skin, 381 CBsophagism, (i8, 443 (Esophagus crises, 180 (Esophagus spasm, 68 Olfactory nerve, 2(i Olivo-ponto-cerebellar atrophy, 315 Olympic forehead, 320 Onanism, 421 Onomatomania, 428 Ophthalmic migraine, 201, 457 Ophthalmoplegia, 303 chronica, progressive, 121 externa, 95, :i-'l interna, 29, 218 totalis, 31 Oppenheim reflex. 114, 134, 136, 163, 261, 300 Optic agnosia, 286 Optic aphasia, 280 Optic nerve, 26 atrophy, 27, 184, 193 Organotrophy, 44 Osteoarthropathies, 146, 179 Osteoma, 289 Ovarian point, 438 Overuse theory, 166 Oxyakoia, 32 Oxycephalus, 320 Pachymeningitis, hypertrophic cervical, 148 internal hemorrhagic, 301 Palanesthesia, 7 Palate reflex, 29 Pallesthesia, 7 Palmar reflex, 8 Palpebral point, 48 Palpitation, 415 Panaris analgt^siquc, 146 Pantalgia, 439 Papilledema, 290, 307 Papillomacular bundle, 184 Paracusis Willisii, 36 Paradoxical foot phenomenon, 88 Paragraphia, 279 Parakinesia, 283 Paralvsies pareellaire, 184 Paralysing vertigo, 93 Paralysis, 7, 184, 200 agitans, 86 ascendens acutissima, 159 ascending, 242 bulbo-pontine, 242 compression, 3 diphtheritic, 43 galloping, 206 glosso-labio-pharyngeal, 118 individual, 7 Landry's, 242 median, 3 musculo-cuta neons, 19 muscular spinal, 3, 20 musculo spiral, 2 obstetrical, 3 of circumflex nerve, 19 of long thoracic nerve, 17 Paralysis of muscles of mastication, 324 of musculo-cutaneous nerve, 19 of the tongue, 324 of tibialis posticus, 21 paroxysmal, 93 periodic, 93 perineal, 2, 23 pressure, 3 professional, 2 progressive, 184, 200 progressive bulbar, 116 rheumatic facial, 3 serratus, 3 sleep, 2 spastic spinal, 111 spinal, 214, 219 superior bulbar, 121 the most important types, 16 toxico-professional, 4, 43 ulnar, 3 Paralytic face, 255 Paralytic pes cavus, 239 Paralytic pulse, 292 Paramyoclonus multiplex, 73 Paramyotonia, 91 Paraphasia, 272, 279 Paraplegia, 7 Paraplegic paralysis, 332 Paraplegic rigidity, 334 Parapraxia, 284 Parasitic cysts, 289 Parasvphilis, 164 Parectropia, 283 Paresis. 7, 200 circular form, 206 professional, 3 Paresthesias, 412 Paresthetic neuralgia, 42 Paretic seizures, 204 Parietal point, 48 Parkinson's disease, 86 without agitation, 88 Paroxysmal gastroxynsis, 416 Partial pseudo-hypertrophy; 99 Partial R. D., 11 Passionate attitudes, 445 Patellar, clonus, 114 reflex, 8 Pathformation, 55 Pathographies, 395 Pathomimicry, 451 Peau moite, 349 Peetoralis, defect of, 325 Peduncular hemiplegia, 270 Pellagra, 112, 213, 364 Pension hysteria, 447 Periodic oculomotor paralysis, 458 Peripheral nerves, 1, 13 diseases of, 26, 145 neuritides, 41 Peripheral pseudo-tabes, 191 Permanent drainage of the ventricle, 321 Peroneal point, 51 Perseveration, 284 Pes calcaneus, 146, 239 Pes equino-varus, 23, 146 Pes valgus, 239 Petit mal, 392 Phantasy, 449 Pharyngism, 68, 443 GENERAL INDEX 477 Pharynx, crises, 180 spasms of muscle of, 68 Phoenix, 57 Phosphate, 355 Phosphaturia, 416 Phrenic nerve neuralgia, 49 Pianoplaver's cramps, 72 Pica, 44l" Pied tabetique, 179 Pill rolling, 87 Pinpoint pupils, 183 Pithiatism, 440 Plain neurasthenias, 414 Plantar reflex, 8 Platysma phenomenon, 261 Plegias. ? Plagiocephaly, 336 Plexiform neuroma, 5 Plexus neuritides, 10, 41 Pluriglandular insufficiency, 368 Polioencephalitis, 242 inferior, 121 inferior, hemorrhagica, 303 superior, 121 superior hamorrhagiea, 303 Poliomyelitis, anterior, 157 anterior acuta infantum, 235 chronic, 109 Pollutions, 121 Polydipsia, 443 Polymorphous tremor, 442. 443 Polymyositis, interstitial, 379 Polyneuritic psychosis, 10 1 Polyneuritides, 11 Polyneuritis, 10, 13, 40, 41, 246 Polyopia, monocular, 441 Pontine hemiplegia, 270 PooISchlesingi r sign, 77 Popliteal point, 51 Porencephaly, 327 prenatal. 328 primary. 327 Poriamania, 394 Positive Rinnr. 31 Postdiphtheritic ataxia, 43 Posthemiplegic chorea, 80 Posticus paralysis, 37 Posterior horn type- of sensory anomaly, 145 Posterior perforating point, 50 Posterior radicotomy, 163 Posterior root,. 166 syndrome, 172 Poir.t disease, 163 Preacher's hand, 1 16, 148 precordial anxiety, 415 Prehemiplegic chorea, 80 Prenatal lesions, 327 Premal ure senescence, I -' s Pressure, paralysis, 162 , pulse, - M| i Priapism, 156 Procursive epilepsy, 398 Procursive epileptic attack, 393 Prognathism, kit Progressive locomotor ataxia, 87, 164, 182, 200 Progressive muscular atrophy, 97 Progressive muscular dj trophy. 97 Progressive primitive myopathy, 97 Progressive spinal muscular atrophy. 120, 117 Pronation phenomenon, 11a Propulsion, 88 Prosoplegia. 31 Protargol, 194 Protean neurosis. US Provocative agents, 47 Pruritus, nervous, 112 Psammoma, 289 Pseudo-ankle clonus, 413, 447 Pseudo, bulbar paralysis, 120, 229 hypertrophy, 98 membranous colitis, 417 microcephaly, :i:(s paralysis. 209 parencephaly, 327, 329, 337 tabes. 165 tumor, 161 tumor cerebri, 288 Psychalgia, 439 Psychasthenic HIS, 428 Psychic anomalies, 409 Psychic aura, 389 Psychic changes, 349 Psychic equivalents. 11.5 Psychic powerlessness, 128 Psycho-analysis, 150 Psychographic disturbances, 429 Psychoneuroses, 103, 105 Psychopathia sexualis, ill Psychotherapy, 70, 73, 199, 232, 429, 452 Ptosis, 28, 94 hysterical, II? Pulmonary osteoarthropathy, 367 Pulsus inaequalis, tl."> Pulsus respiratione intermittens, 415 Pupillary alterations. 202 Pupillary functions, is." Pupillary rigidity. 183, -'02. 217 Pupillary symptoms, 182 Pupils, sluggishness of, 183 Pure alexia, 280 Pure word deafness, 878 Pure word dumbness, 277 Puzzling trophoneurosis, 451 Pyelo-nephritis, 1S9 Pyknosis, -'I I Pyramidal tracts, physiology of, 112 Pyramids, decussation of, 112 Quinine therapy, 3? I Quinke'i oedema, 381, 382 Quinquaud't phenomenon, <>'i Quinquaud's symptom, ill Rachialgia, II.' Rachischisis, 32! Rachitic skull. 319 Radial phenomenon, 163 Radicotomy, 163 Radiculalgia, l"> Radicular neuralgia. I '. Radicular type of disturbances of sensibility, 17.) Radiculitides, II Radius, reflex, s Kami communicantes, alia. 370 irrisei. 370 Raynaud'* disease, 375, 377 Ri ich f (l. gener itlon, 10, 1 1, .'37 Hecklinghaun n't disea • . I Recovery « iih defect, 219 478 GENERAL INDEX Rectal crises, 181 . Red migraine, 460 Reflex epilepsy, 396, 401 Reflex neuralgias, 45 Reflexes, 7 Regeneration, 6, 9 Regionary cyanosis, 376 Reinversion, 233 Renal crises, 181 Reserve centers, 273 Residence in high valleys, 354 Respiration fatigue, 225 Respiratory disturbances, 414 Respiratory spasms, 445 Retention of urine, 186 Retrobulbar neuritis, 27 Retrocollis, 69, 448 Retrograde amnesia, 305 Hetropulsion, 88 Retropulsive epilepsy, 393 Revulsives, 60 Rhizotomia, posterior, 116, 335 Rhomboid spasm, 69 Rinne's positive, 34 Rhine's test, 34 Risus sardonicus, 66 Robertson's symptom, 182 Rodagen, 356 Romberg's disease, 383 Romberg's phenomenon, 173 Root, neuralgia, 45 neuritides, 41, 45 pains, 163, 295 Rotary spasms, 69 Rotary vertigo, 309 Rushes of blood, 416 Rust's phenomenon, 163 Sadism, 444 Sajodin, 193 Salaam spasm, 69, 392 Saltatory reflex spasm, 71 Salt free diet, 399 poor diet, 399 Salvarsan, 160, 193, 211, 220 Salvarsanized serum, 212 Sandmannli, 344 Sandwybli, 344 Sarcoma, 288 Saturnism, 158, 329, 388, 396 Sawyer's cramps, 72 Scapula alata, 17, 395 Scapular reflex, 8 Scapulo-humeral reflex, 8 Scapulopesia, 105 Schleich's infiltration anesthesia, 60 Srhloesser's injection, 61 Schultze's comma, 170 Schurbach'a test, 34 Schwann's sheath, 5 Sciatica, 51 varicose, 51 varicose, spasmodic, 52 Sciatic scoliosis, 52 Scintillating scotoma, 458 Sclerodactylism, 378 annular, 378 Scleroderma, 377 Sclerodermic en bandes, 379 Sclerodermic en coup de sabre, 379 Sclerose en plaques, 132 Sclerosis, 139, 239 amytrophic, 140 bulbar, 139 hemiparetic, 139 multiple, 121, 132, 142, 162 paraplegic, 139 Scotoma, 26 Screaming spasm, 71, 445 Scrotal tongue, 340 Seamstress's cramps, 72 Secondary degeneration, 157 Secondary porencephalies, 327 Seelig-Mueller's neuralgia, 47, 127 Segment innervation, 152 Self-perception, 411 Senile incontinence, 230 Senile spastic paraplegia, 227 Sensation, disturbances of, 25 Sense impressionability, 449, 453 Sense of cold, 6 of heat, 6 of movement, 6 of pain, 6 of position, 6, 7 of pressure, 6 of taste (testing), 32 of temperature, 6 of touch, 6 Sensibility, 6 disturbances of, 175 of deep, 175 of superficial, 175 Sensitive crossway, 257 Sensory aphasia, 272 Serous apoplexy, 271 Serous meningitis, 288 Serous meningitis of the posterior fossa of the skull, 313 Severe epileptic convulsive attack, 389 Sexual neurasthenia, 409 Shaking disease, 442 Shaving cramp, 72 Shock, 149 Shoemaker's cramp, 72 Shoulder, spasms of muscle of, 68 Siderodromophobia, 427 Signe de peaucier, Jul Signs of overirritabilitv, 349 Silver nitrate, 193 Simple atrophy, 27 Singultus, 71 Situation anxiety, 427 Skeletal muscles, disturbances of, 185 Skeleton hand, 108, 118 Skin reflexes, 8 Sky-high exaltation, 409 Sleep inversion, 223, 233 Sleeping sickness, 209 Sleeplessness, 410 Sleeplessness drugs, 210, 233 Smith's cramps, 72 Snake man, 173 Sneezing reflex, 29 Sneezing spasm, 71 Snoring reflex, 71 Softening of the brain, 200 Softening of the cortical layer, 200 Solitary tubercle, 289 Somnambulism, 445 GENERAL INDEX 479 Spanish boots, 180 Spasm nodding, 69 Spasm of glottis, 76 of splenitis, 69 Spasmodic epileptiform neuralgia, -18 Spasmophilia, 78 Spastic spinal infantile paraplegia, 332 spinal paralysis, 111, 115, 226 symptom-complex, 112, lit Spasticity of the limbs, 337 Speech disturbance, 202 Spermatorrhoea, 1.' 1 Sphincter disturbances, 186 Sphinx countenance, 101 Spina bifida, 322 occult, 323 Spinal ectopies, 321 Spinal epilepsy, 65 Spinal fluid phenomenon, 307 Spinal foci of disease, 32H Spinal form of muscular atrophy, 107 Spinal ganglia, 166 Spinal gliosis, 143 Spinal heredo-ataxia, 123 Spinal intermittent limping, 115, 215 Spinal irritations, 405, 412 Spinal progressive muscular atrophy, 245 Spirochete, 21 1 Spontaneous fractures, 178, 239 Spontaneous gangrene, 377 Spontaneous recoveries, 319 Sporadic cretinism, 358 Stage of local reddening, 376 Stage of reaction, 263 Stasobasophobia, 117 Status, choreicus, 81 criticus, isit crihrosus, 227 epileptieus, 180, 208,391 hemicranicus, 457 hydroceplialicus, 251 lacunaris, 227 St, cple skull. 320 Stellwag's sign, 317 Steppage, 23 Stereo-anesthesia, 7, 177 Sternal point, 138 Stiff neck. 249 Stiffness of the neck, ?98 Stigmata of degeneration, 165, 336, 394, 107, m Stork legs, 106 StrHmpelPs sign, 115 Struggle for pension, 1 !5 Struma suprarenalis, 363 Strychnine, 194 Stuttering, \S6 Subacute anterior poliomyelitis, 245 Subarteria] spinal tracts, 112 , Subcortical sensory aphasia, !78 Supiti itor longus, 1 1 Superficial sensibilities, 7 Supporting corset, ins Supportive apparatus, 116, 198 Supraorbital neuralgia, 11 Supraorbital point, is Suspension treatment, 197 Surgery of tlie central convolutions. ."I I Surprise method, 152 Sweat secretion, 9 Sydenham's chorea, 80 Syllable tumbling, 202 Symmetric lipomatosis, 387 Sympathetic, crises, 180 diseases of the, 369 nervous system, 369 ptosis, 147, 371 Symptom of approximate answers, 445 Symptom of painful thinking, 222 Symptomatological triad, 139 Syncopal attacks, 115 Syncope, 304 Syndactylism, 395 Synergic pupillary reaction, 183 Syphilis, 158, 164, 311, 317, 425 a virus nerveux, 165, 192 Syphilitic, endarteritis obliterans, 361 spinal amyotrophy, 186 Syphilogenic diseases, nil. tsj, 200, 213 Syphilogenic combined system diseases, 213 Syringo bnlbia. 143, 147 Syringomyelia, 4, 120, 143 System diseases, 111, 166, 213 Systematic vertigo, 309 Tabes, acuticima, 190 arrested by blindness, 189 cervical, 190 dorsalis, 164, 182 dorsaiis spasmodique, 111, 121 inferior, 190 paralysis, 189 rudimentary, 190 superior, KM) visceral, 190 without spinal cord symptoms, 190 Tabetic foot, 179 Taboparah sis. 201 Tache cerebrate, 209, 250 Tachycardia, 37, 317 paroxysmal, 41 t Tachynoic attacks, 445 '1'actilc- agnosia. 287 Tactile aphasia. .'SO Tailor's cramps, 12 Talalgia, 53 Talipes calcaneus, 21 Tapir snout, 101 Tarsalgia, 53 Tartar type, 339 Tay'i cherry red spot, :t:ts 7,ii/ Sack's disease, Teichopsia, 157 Telegrapher's cramps, 72 Telephone operators. 2 T, mperat ure sense. 6 Temporal point. Is Temporary infantile spinal paralysis. 241 Tendinitis, calcareous. 379 Tendon reflex, s (ran plantation, 107 Tenotomy, 105, 107, 116 Teratoma, 189 Terminal delirium, 115 Testicular crises. 1S1 Tetany, ?i Tetraplegia. 7, !Mi The man with (be litlle paper . ins Therapy of litil,- symptoms, ft." 480 GENERAL INDEX Thermoanesthesia, 6 Thermohypoesthesia, 6 Thermotherapy, 58 Thomsen's disease, 90 Throat, spasm of muscles of, 68 Thrombosis of basilar artery, 121 of the brain sinuses, 302 Thyradine, 361 Thyreoaplasia, 341, 358 Thyrogenous theory of Basedow's disease, 322 Thyroidism, 362 Tibial phenomenon, 115 Tic, 73, 428, 443 Tigroid scales, 244 Tigrolysis, 244 Tinnitus aurium, 309 Toe phenomena, 114 Tongue, spasm of, 67 Tonic reflex, 82 Tooth spasms, 388 Topalgias, 438 Torpor peristalticus, 419 Torsion neurosis, 86 Torticollis, 448 Touch paralysis, 295 Touch sense, 6 Tachycardia, 347 Tracts of spinal cord, 158 Tracts which degenerate downward, 158 Tracts which degenerate upward, 158 Tractus, spino-cerebellar, 169 spino-thalmicus, 167 Tragic look, 346 Transcortical aphasia, 279 Transference, 439 Transplantation, 248 Transposition of motor points, 16 Transverse, laughing, 102, 119 lesions of the spinal cord, 150 Traumatic hysterics, 442 Traumatic neuritis, 6 Traumatic neurosis, 424 Treatment in sanitariums, 431 Tremor, 63, 203, 414 hysterical, 414 in Basedow's disease, 348 neurasthenic, 414 of. the degenerate, 64 vibrating, 63 Trepidation, epileptoide, 114 Triceps reflex, 8 Trigeminal neuralgia, 47 Trigeminus, 29 Triplegia, 7, 236 Trismus, 65 Troehleares, 29 Trophic disturbances, 350 Trophic functions, 9 Trophoedeme, familial, 381 Trophoneuroses, 369 Trousseau sign, 77 True plethora. 304 Trumpeter's cramps, 72 Tubercula dolorosa, 5 Tubercles in the chorioid, 300 Tuberculous meningitis, 299 Tuberous sclerosis, 328, 329 Tumors in the region of the corpora quad- rigemina, 368 Tumors of basis of brain, 294 of cerebello-pontine angle, 288, 239, 311 of frontal lobe, 294 of occipital lobe, 295 of region of the optic thalamus, 294 of spinal cord, 160 of temporal lobe, 295 of vermis, 309 Urinary stuttering, 421 Urticaria, 381 Vaginismus, 421, 443 Vagotony, 371 Vagus crises, 180 Vagus nerve, 37 Vagus paralysis, 37 Valerian saturation, 355 Valleix points, 46, 457 Vapors, 405 Vasa corona, 243 Vasoconstriction, 9 Vasoconstrictor neuroses of the extremities, 373 "Vasodilators, 9 Vasodilator neurosis, 382 Vasomotor, angina pectoris, 456 functions, 9 trophic neuroses, 372 Vegetative nervous system, 369 Veitstanz, 80 Ventral field of the posterior column, 170 A'entricles, puncture of. 321 Verbal amnesia, 279 Verbal paragraphia, 278 Verbal paraphasia, 277 Vermicular response, 11 Vertebral column, affections of, 162 Vertebral point, 50 Vertical divergence of the eyeballs (of Magen- die), 310 Vertigo, 411 permanent, 412 Vesical crises, 181 Vesico spinal center, 155 Vestibular attacks, 309 Vestibular nerve, 33 Vigouroux's phenomenon, 349 Visceral nervous apparatus, 369 Vitiligo, 387 Vltlpian's conjugate deviation, 262 Waddling gait, 103 Waller's law, 5, 8, 157 Wandertrieb, 394 Was]) waste, 102 Wassermann reaction, 164, 221 Watchmaker's cramps, 72 Weakness of memory, 209 Weber's symptom-complex, 270 Weber's test, 35 Weeping spasm, 445 Weir-Mitchell rest cure, 435 Werding-Hoffmann type of progressive muscu- lar atrophy, 107 Wernicke's aphasia, 276 Wernicke's zone, 272 West/thai'.* phenomenon, 174 Westphal's pseudo-sclerosis, 141, 187 GENERAL INDEX ■481 White communicating; branches, 870 White migraine, 460 Witch signs, 441 Witzelsucht, 394 Word deafness, 276 Word debris, 2~1 Word dumbness, 276 Word memory. 272 Wrist clonus, 114 Wri^t drop, 20, 54 Writer's cramps, ~2 Xantho chromia, \t>2 Xylographers, 2 ' Xyrospasms, 72 Yawning spasms, 71 Zither player's cramps, 72 Zona, 384* Zona cornu-commissularis, 170 Zona meduUo-vasculosa, 8S3 Zona septo-marginalis, 170 i -0/*"^ COLUMBIA UNIVERSITY This book is due on the date indicated below, or at the expiration of a definite period after the date of borrowing, as provided by the rules of the Library or by special ar- rangement with the Librarian in charge. DATE BORROWED DATE DUE DATE BORROWED DATE DUE njC MAY 2 \ 1945 " tfjUV r Mil f WW 9 ?. A** 1 . \&h ]AY3i194^ «a,e3a, M =o