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TEXT-BOOK 
 
 ON 
 
 NERVOUS DISEASES 
 BURR 
 
miS 
 
Base Hospital No. 20, 
 
 TEXT-BOOK 
 
 ON 
 
 NERVOUS DISEASES 
 
 BY 
 G. ASCHAFEENBURG, COLOGNE; H. CURSCHMANN, MAYENCE; R. FlNKELN- 
 
 burg, Bonn; R. Gaupp, Tubingen; C. Hirsch, Gottingen; Fr. Jamin, 
 Erlangen; J. Ibrahim, Munich; Fedor Krause, Berlin; M. Lewan- 
 dowsky, Berlin; Ff. Liepmann, Berlin; L. R. Muller, Augsburg; 
 H. Schlesinger, Vienna; S. Schoenborn, Heidelberg; Ff. Starck, 
 Karlsruhe; Ff. Steinert, Leipsic 
 
 AUTHORIZED ENGLISH EDITION 
 EDITED by 
 
 CHARLES W. BURR, B. S., M. D. 
 
 PROFESSOR OF MENTAL DISEASES IN THE UNIVERSITY OF PENNSYLVANIA; 
 NEUROLOGIST TO THE PHILADELPHIA GENERAL HOSPITAL 
 
 WITH 156 TEXT ILLUSTRATIONS 
 
 Base Hospital No. 20 ; 
 VOL. I 
 
 PHILADELPHIA 
 
 P. BLAKISTON'S SON & CO. 
 
 1012 WALNUT STREET 
 
Copyright, 1915, by P. Blakiston's Son & Co. 
 
 v. | 
 
 THE. MAPLE. PRESS. YORK. PA 
 
PREFACE TO THE AMERICAN EDITION 
 
 The German contributors to this book should need no introduction 
 to American physicians as they are all men of established reputation. The 
 articles are based on personal knowledge and experience and each writer 
 presents his own well thought out and mature opinions. While the work 
 is purely scientific in tone and conservative in attitude, a matter of much 
 importance in these days when much wild doctrine, especially as to mental 
 therapeutics, is being offered the public, it has never been forgotten by the 
 writers that the only useful knowledge is that which leads to sane therapeu- 
 tics. There is no need to discuss the advantages offered by a book 
 written by many authors over a one-man work as it has been done by the 
 editor of the German edition. The translators have endeavored to do 
 their work faithfully and to give an accurate rendering of the writers' 
 statements. The American editor has not cut out the many references to 
 European health resorts, not because he thinks Americans need go abroad 
 for such treatment, but in order to encourage Americans to develop and 
 encourage their own natural cure places. 
 
 Editor of the American Edition. 
 
PREFACE TO THE GERMAN EDITION 
 
 The marvellous advance made by neuro-therapeutics during the second 
 half of the last century has, as a natural consequence, induced specialization 
 within the confines of this domain. On the one hand this tendency was 
 furthered by the unlike scientific descent of the leading investigators, among 
 whom from the very first, there was a clear cut distinction between the 
 psychiatrical- and the interno-neurologists; on the other hand, neurology, 
 in itself, was a field to which not a few borderland fields naturally attached 
 themselves. 
 
 Thus it has come to pass that the neuro-therapeutics of to-day stands 
 in intimate relation not only with psychiatry and internal medicine, but 
 also with ophthalmology and otology, with dermatology, bacterio-serology, 
 and above all with surgery, making use more and more of the aid afforded 
 by these special sciences. 
 
 If therefore we venture to submit to physicians and students a newly 
 compiled text-book of neuro-therapeutics, we are not blindly following the 
 fashion — a fashion which in recent years has given extraordinary precedence 
 to this cooperative method of text-book production — but are conforming in 
 no slight degree to that very peculiarity which characterizes, as we have 
 said, our field. Our intention was to produce for students and practitioners 
 — that is essentially for non-specialists — a text-book comprising old material, 
 that has long been established, as well as the new, nay the very latest ac- 
 quisitions of neuro-therapeutics. It aimed to give those who have but 
 little time for specialization a book containing what is essential, concise, 
 and vitally instructive, though naturally devoid of that fullness to be found 
 in the special monographs. How could these requirements be better met 
 than by having the separate chapters written by authors whose work and 
 original successful researches prove that they stand in the very midst of the 
 events and the literature of each field concerned ? Within the narrow con- 
 fines of a text-book, they are able to unite, first of all, brevity with clearness, 
 and completeness with the necessary conciseness. A few examples may 
 serve to illustrate: Clinical treatment of disturbances of the circulation of 
 the brain, however much it concerns therapeutically the special neurologist, 
 considering its close relation to the pathology of the heart and the circula- 
 tion, doubtless demands presentation by an internist whose special subject 
 is the pathologic physiology of the circulation. The discussion of menin- 
 gitis, also, will be less adapted to the special neurologist than to the internist 
 skilled in bacteriology and cyto-diagnosis. On the other hand, the exposi- 
 
Vlll 
 
 PREFACE 
 
 tion of the present condition of the pathologic physiology of the brain necessi- 
 tates, in view of its complicated structure, and the rapid progress made in 
 recent times, a specialist's treatment. It is no accident that this difficult 
 realm is almost or quite a terra incognita to many interno-neurologists of 
 the well known schools and that it is to a small degree only cultivated by 
 many representatives of clinical psychiatry. And finally: of all the neurol- 
 ogists, internists and psychiatrists, who should undertake to present briefly 
 and critically the modern status as to the operative possibilities in neuro- 
 therapeutics ? Every one will here give precedence to the surgeon of this 
 special field, and we are glad and proud to have found as collaborator for 
 this special field the most noted German brain surgeon. 
 
 I think that I have shown by these few examples that the compiling of a 
 text-book that recognizes the newly-acquired results, by several authors, 
 may well be justified. That disadvantages may, to some extent, offset the 
 advantages of this method, can not be denied, and corresponds to the experi- 
 ence with compiled text-books in other fields of medicine. On account of 
 the multiplicity of authors, the uniformity of presentation necessarily suffers, 
 nor can repetitions be altogether avoided. We ask the reader, therefore, 
 to judge these faults leniently, and hope to obviate them to some extent in 
 later editions. 
 
 Should we succeed in arousing by this text-book, in wider classes of non- 
 specializing physicians, interest for and understanding of neurology as an 
 equally justifiable and important special field among those other special 
 fields long officially recognized as being of signal importance, we shall see 
 in this success at once the attainment of our purpose and our reward. 
 
 The Editor. 
 
TABLE OF CONTENTS 
 
 i 
 
 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 By Privatdocent Dr. S. Schoenborn, Heidelberg 
 
 Pagb 
 
 i. Taking the anamnesia i 
 
 2. The objective findings 3 
 
 A. General remarks 3 
 
 B. Testing the sense organs, voice and speech 5 
 
 1. The eye 5 
 
 2. The ear 14 
 
 3. Taste and smell 16 
 
 4. Disturbances in voice and speech 17 
 
 5. Disturbances in the realm of the other cerebral nerves 20 
 
 C. Motility 21 
 
 D. Sensibility 54 
 
 E. Reflexes 64 
 
 F. Vasomotor, trophic, and secretory disturbances 72 
 
 G. Examination of the cerebro-spinal fluid 75 
 
 H. Examination with Rontgen rays 77 
 
 II 
 
 DISEASES OF THE PERIPHERAL NERVES 
 By Privatdocent Dr. H. Steinert, Leipsic 
 
 Introductory anatomical remarks and definition 78 
 
 Classification of peripheral nerve diseases 80 
 
 I. Destructive diseases of the peripheral nerves 80 
 
 A. General part 80 
 
 1. General pathological anatomy 80 
 
 2. General symptomatology and pathological physiology ..... 83 
 
 3. General diagnostics and prognostics 89 
 
 4. Division of the destructive diseases 93 
 
 B. Special part 94 
 
 Diseases of single peripheral nerves 94 
 
 General aetiology 94 
 
 Pathological anatomy 95 
 
 General therapeutics 98 
 
 Paralyses of single peripheral nerves 106 
 
 A. The cranial nerves 106 
 
 1. The olfactory nerve .' 106 
 
 2. The optic nerve 107 
 
 ix 
 
x COX TEXTS 
 
 Page 
 
 3. Nerves of the eye muscles. Oculomotor, trochlear and abducens 
 
 nerves • II2 
 
 4. The trigeminal nerve 119 
 
 5. The facial nerve 123 
 
 6. The auditory nerve 133 
 
 7. The glossopharyngeal nerve 137 
 
 8. The vagus (pneumogastric) nerve 137 
 
 9. The accessory nerve 14° 
 
 10. The hypoglossal nerve 142 
 
 B. The spinal nerves 143 
 
 1. The phrenic nerve 144 
 
 2. The brachial plexus and its individual nerves 145 
 
 3. The other thoracic nerves 157 
 
 4. Xerves of the lumbar and sacral plexuses 157 
 
 5. Xerves of the pudendal and coccygeal plexuses 164 
 
 Supplement: Occupational paralysis and occupational neuritis 164 
 
 Polyneuritis 165 
 
 Particular clinical forms 169 
 
 Supplement: A. Landry's paralysis, paralysis ascendens acuta 174 
 
 B. Leprosy of the nervous system 175 
 
 II. The neuralgias 176 
 
 Supplement: Herpes zoster '186 
 
 III. The tumors of the peripheral nervous system 188 
 
 III 
 DISEASES OF THE SPIXAL CORD 
 
 1. Normal and pathological physiology of the spinal cord. By Privatdocent Dr. 
 
 Max Rothmann, Berlin 190 
 
 A. Anatomy of the spinal cord 190 
 
 B. Functions of the spinal cord - . . 205 
 
 1. The reflex activity 205 
 
 2. Conduction of motility 221 
 
 3. Conducting tracts for sensibility 229 
 
 Brown Sequard symptom-complex 235 
 
 2. Systemic diseases of the spinal cord 239 
 
 (a) Tabes dorsalis. By Privatdocent Dr. S. Schoenborn, Heidelberg . . . 239 
 
 (b) Friedreich's disease (hereditary spinal ataxia). By Privatdocent Dr. S. 
 Schoenborn, Heidelberg 276 
 
 (c) Spastic spinal paralysis. By Professor Dr. F. Janim, Erlangen . . . 282 
 
 (d) Amyotrophic lateral sclerosis. By Professor Dr. F. Jamin, Erlangen . 292 
 
 (e) Progressive spinal muscular atrophy (Duchenne-Aran) . By Professor 
 
 Dr. F. Jamin, Erlangen 297 
 
 (f) Xeurotic progressive muscular atrophy. By Professor F. Jamin, 
 Erlangen 306 
 
 (g) Subacute and chronic poliomyelitis. By Professor Dr. F. Jamin, 
 
 Erlangen 310 
 
 (h) The combined columnar diseases of the spinal cord. By Professor Dr. 
 
 F. Jamin, Erlangen 313 
 
 (i) Bulbar paralytic diseases. By Professor Dr. F. Jamin, Erlangen . . . 318 
 
CONTENTS xi 
 
 Page 
 
 i. Progressive bulbar paralysis 318 
 
 2. Acute (apoplectic) bulbar paralysis 324 
 
 3. Pseudo'-bulbar paralysis. By Professor Dr. F. Jamin, Erlangen . 333 
 
 3. Multiple insular sclerosis. By Professor Dr. Hermann Schlesinger, Vienna. . 340 
 
 4. Syringomyelia. Spinal gliosis. By Professor Dr. Hermann Schlesinger, Vienna 350 
 
 5. Haematomyelia. By Professor Dr. Hermann Schlesinger, Vienna 364 
 
 6. Focal diseases of the spinal cord due to extra- and intra-medullary affections. 
 
 By Professor Dr. R. Finkelnburg, Bonn 367 
 
 I. Traumatic diseases of the spinal cord 367 
 
 II. The pressure paralyses of the spinal cord 373 
 
 (a) Caries of the spinal column, spondylitis tuberculosa 374 
 
 (b) Carcinoma and other tumors of the spinal cord 379 
 
 (c) Neoplasms of the spinal cord and its membranes 382 
 
 Tumors of the membranes of the spinal cord 382 
 
 Intramedullary tumors 385 
 
 Determination of the location of the tumor ....... 388 
 
 (d) Pachymeningitis cervicalis hypertrophica (Charcot) 393 
 
 III. The syphilitic diseases of the spinal cord 394 
 
 Meningomyelitis chronica syphilitica 394 
 
 Syphilitic spinal paralysis 397 
 
 IV. Myelitis 400 
 
 Supplement: Abscess of the spinal cord 410 
 
 7. Diseases of the conus terminalis and the cauda equina. By Dr. L. R. Muller, 
 
 Augsburg 410 
 
 IV 
 
 THE MYOPATHIES WITHOUT DEMONSTRABLE CHANGES IN THE 
 
 NERVOUS SYSTEM 
 
 By Oberarzt Dr. Hans Curschmann, Mayence 
 
 1. Progressive muscular dystrophy 424 
 
 2. Myotonia congenita, Thomsen's disease 443 
 
 3. Myasthenia pseudo-paralytica 437 
 
 4. Myatonia congenita 443 
 
 V 
 
 DISEASES OF THE BRAIN 
 
 1. Normal and pathological physiology of the brain. By Professor Dr. Hugo 
 
 Liepmann, Berlin 445 
 
 I. Introductory remarks on the anatomy, physiology and pathology of the brain . . 445 
 
 1. The membranes of the brain 445 
 
 2. Circulation in the brain 446 
 
 Veins of the brain 450 
 
 3. Morphology of the brain 450 
 
 (a) Convolutions and fissures of the convexity 451 
 
 (b) The medial surface and base of the brain 453 
 
 4. Histological structure of the cortex 455 
 
 5. Nuclei of the cerebral nerves 458 
 
 6. General relation between structure and function of the brain 462 
 
 (a) Theory of projection, projection paths and projection fields . . 462 
 
xii CONTENTS 
 
 Page 
 
 (b) Mnemic cortical fields and the theory of association fields . . . . 467 
 
 (c) Association paths .. 47 1 
 
 (d) The commissural fibres, especially the corpus callosum .... 472 
 
 7. Clinical localization in the left hemisphere 474 
 
 8. Differences between the right and left hemispheres 479 
 
 9. The cerebellum 480 
 
 10. Brain and reflexes 482 
 
 11. Secretory functions of the brain 485 
 
 II. Symptomatology dependent upon location 486 
 
 1. General symptoms 486 
 
 2. Projection disturbances: Disturbance in movement and sensation. . . 488 
 
 (a) Motor symptoms of irritation 488 
 
 (/3) Sensory symptoms of irritation 490 
 
 (A) Motor symptoms of loss of function 491 
 
 1. Paralyses 491 
 
 2. Contractures and hypertonia 493 
 
 3. Differences in cerebral paralyses according to the location of 
 
 the injury . ■ 496 
 
 (a) Cortical foci 496 
 
 (/?) Capsular foci 498 
 
 (T) Focal injuries in the peduncle and pons, alternating 
 
 paralyses 498 
 
 Volume of muscles 499 
 
 (d) Sensory symptoms of lost function 500 
 
 (e) Cerebral and cerebellar ataxia 506 
 
 (f) Symptoms of lost sensory function (disturbances of the special 
 
 senses) 507 
 
 1. Visual disturbances, visual paths 507 
 
 2. Auditory disturbances 515 
 
 3. Olfactory and gustatory disturbances 518 
 
 3. Mnemic-associative disturbances 518 
 
 (a) Aphasia disturbances 518 
 
 1. Introduction 518 
 
 2. Disturbances of the mnemic-associative speech apparatus: 
 
 Aphasia. Internal speech 520 
 
 3. The speech regions in the brain 524 
 
 4. Complete motor aphasia 526 
 
 5. Complete sensory aphasia 527 
 
 6. Total aphasia 529 
 
 7. Insular aphasias ' 530 
 
 8. Pure word dumbness 531 
 
 9. Pure word deafness 533 
 
 10. Transcortical aphasias . . 534 
 
 Disturbances in written speech 539 
 
 (b) Agnostic disturbances 541 
 
 (c) Apraxic disturbances 544 
 
 1. General remarks 544 
 
 2. Motor apraxia 546 
 
 3. Ideational apraxia 548 
 
 4. The predominance of the left hemisphere in actions and 
 
 localization of apraxic disturbances 549 
 
GENERAL DIAGNOSTICS 
 
 OF 
 
 NERVOUS DISEASES 
 VOL. I 
 
I 
 
 GENERAL DIAGNOSTICS OF NERVOUS 
 DISEASES 
 
 BY 
 
 S. SCHOENBORN (Heidelberg) 
 
 i. TAKING THE HISTORY 
 
 The dictum that medical art in its clinical aspect is deprived of its best 
 foundation when lacking a good history of the patient, sounds very old- 
 fashioned to-day, in this age of orthodiagraphy and precipitin reactions. 
 Should I assert, however, that this dictum is nevertheless correct and for no 
 special field more correct than for nervous diseases, I should not like to be 
 summoned, on this ground, as star witness for a supposed backwardness 
 in neurology. On the contrary, I would state, that, rather it is an advan- 
 tage that within the confines of our field that endless splitting up into extreme 
 specialism, recent and very recent, has not as yet taken place, as we see it to- 
 day in several branches of internal medicine. A summary of our knowl- 
 edge of neurology, in spite of the wealth of new observations and facts 
 increasing, too, year by year, is still possible for the individual physician; 
 he need not yet, because of the confusing mass of separate sciences, lose a 
 clear view of the case as a whole- — that great inheritance of the first neurolo- 
 gists and internists of the last century. But for this he needs, over and 
 above the modern equipment of diagnosis, before all, the crude tools of 
 those masters: the history of the patient. 
 
 This history of the patient consists according to the traditional scheme 
 of anamnesia, of status, diagnosis, prognosis and therapy. I find no reason 
 for making any changes in this scheme. I know of no more comprehen- 
 sive one. The method of "genius diagnoses" ("snap" diagnoses) at 
 first sight imposes only on the novice. However certainly the experienced 
 may recognize from a distance a tabetic by his staggering gait and the easily 
 visible pupillary differences and a case of Basedow's disease by the staring 
 eyes and the goiter, he must never rely on his empiricism. Very often exact 
 anamnesia alone suffices to correct the would be cock-sureness of diagnosis 
 at first sight. 
 
 I said that the value of an exact history of the patient is especially high, 
 precisely in nervous diseases. This holds true, to begin with, of the anam- 
 nesis. It is more indispensable here than in most internal diseases, though 
 
2 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 on the other hand, it is less often possible to make the diagnosis from the 
 anamnesia alone, than in these diseases. A sufferer from kidney trouble 
 may by an exact account of the development of the disease, and of his troubles 
 alone, assist the physician to a correct diagnosis, and, on the other hand, in 
 this case, the physician can usually make the diagnosis by the examination 
 alone, even without any anamnesia. Both are but rarely possible with the 
 nervous patient. He can not off-hand with sufficient exactitude so state 
 his troubles with respect to time and place as to make the disease recogniz- 
 able by them alone, especially since, in nervous diseases, a slowly progressing 
 course with a large number of slighter and more severe symptoms is the 
 rule. But almost without exception, not even the most minute examination, 
 alone, can enlighten as to the genesis of the disease and the troubles of 
 the patient, which are of such great therapeutic importance. 
 
 In taking up the previous history we inquire first about the family 
 history. Are there among the ancestors or descendants, mental diseases, 
 characteristic neuroses, alcoholism? Were the parents consanguineous? 
 D'id the parents suffer from syphilis probably or possibly? (Mercurial 
 treatment, premature births.) 
 
 Nor can the unfortunately all too indefinite question about "nervousness 
 in the family" be avoided, and it often brings to light important facts. 
 
 All these factors may be comprised under the designation "neuropathic 
 taint." 
 
 Then follow the questions as to the previous diseases of the patient. 
 Here, one must consider before all: normal birth; children's diseases, espe- 
 cially of the nervous kind (spasms, infantile convulsions, migraine, pavor 
 nocturnus, enuresis, etc.), and the thereto predisposing disturbances in de- 
 velopment (rachitis); infectious diseases (measles, scarlet fever, typhoid, 
 diphtheria, influenza); chronic use or exposure to certain poisons (alcohol, 
 nicotine, lead, morphine); over-exertions of every kind (mental, physical, 
 especially too, sexual); masturbation. Particular attention, furthermore, 
 is to be given to two points: first, the possibility of preceding syphilis, for 
 which one should watch with the greatest care and the greatest objectivity 
 (chancre, eruptions, mercurial treatment, symptoms of visceral syphilis, 
 miscarriages and abortions in women; the vocation is to be considered 
 also) secondly, accidents or injuries experienced, whereby, of course, in 
 the interest of the patient, too clear a reference to the traumatism is, at first, 
 to be especially avoided by the questioner. Furthermore, one must inves- 
 tigate the mental development (schooling) and note the vocation of the 
 patient. 
 
 The previous history and the history of the present trouble conclude the 
 anamnesis. While, in his former questions, the investigator may proceed 
 according to a scheme, here, with minute consideration of all nervous 
 regions, he must often deviate from the scheme and follow a definite idea, 
 
THE OBJECTIVE FINDINGS 3 
 
 which may lead him in the direction of fixed disease pictures; for, as has 
 already been mentioned, the nervous patients scarcely ever succeed in sen- 
 sibly arranging their symptoms in proper order and even the schematic 
 questioner has before him, at the end, usually a chaos of individual symp- 
 toms, a disease history "without head or tail." In particular, the physician 
 should attempt to ascertain the very beginning of the disease, its time and 
 place; the correct diagnosis often depends on the temporal sequence of the 
 symptoms (for instance spinal atrophies). Therefore, in suspicion of tabes 
 one will proceed from possible eye symptoms, lancinating pains, in a myeli- 
 tis, from paraesthesias in the feet, bladder disturbances, etc. 
 
 Naturally the symptoms in the internal organs (stomach, heart), must 
 be considered also. 
 
 It should, as a rule, be possible to ascertain from the history all symptoms 
 of a nervous disease, that can at all be subjectively perceived. 
 
 2. THE OBJECTIVE FINDINGS 
 
 A. General Remarks 
 
 In the examination following the history the mere observation of the 
 nervous patient may, in itself, be of great value. Facial expression and the 
 attitude of the body, especially in bed-ridden patients, may give much infor- 
 mation. A rigid attitude or carriage of the body may be dependent upon 
 spastic pareses (myelitis, system-diseases, central affections), paralysis 
 agitans, upon meningitis, tetanus, and other affections. Pains in certain 
 nervous regions often induce anomalies in the posture of the body, which 
 mostly have in view a relaxation of the nerves concerned (sciatica), or cause 
 involuntary twitchings of the musculature in these regions (tic douloureux). 
 Asymmetries, too, strike the attention here at once, not only those that affect 
 the musculature (cf. below), but also especially those which affect the 
 structure of the bones. Scolioses and kypho-scolioses cause suspicion of 
 the presence of sciatica, compression myelitis, syringomyelia. Asymmetries 
 of the face may rest upon progressive facial hemiatrophy, sympathetic 
 affections, acromegaly. In the latter case, we rind similar asymmetries 
 in the extremities. Cranial asymmetry may be due to tumors or hydro- 
 cephalus. Nor must we overlook congenital malformations (cranial malfor- 
 mation, abnormal ear formation, hare-lip, polydactylia, etc.), but one may 
 say that this branch of neuropathic taint has more real importance in 
 psychoses than in nervous diseases (probably most in the so-called neuroses) . 
 There are rarely found in nervous troubles noticeable changes in the skin 
 and the visible parts of the mucous membranes. Here, again, traces of 
 syphilis must be most carefully followed (Hutchinson's triad, scars from 
 tertiary processes, swollen glands). Circumscribed, more rarely diffused 
 reddening or cyanosis, also anaemic pallor, may be interpreted as a sympathetic 
 
4 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 disease, occurring alone, or with spinal or cerebral troubles, even with 
 neuroses. Neurogenous edemas are found in hysteria, more rarely in 
 spinal diseases. Ulcerative changes we see also in hysteria (mostly through 
 self-inflicted injuries), likewise as severe trophic disturbances, usually com- 
 bined with synchronous disturbances in sensibility, especially in tabes and 
 syringomyelia, finally as decubital sores in every long continued loss of local 
 sensation (also in'the mucous membranes — keratitis neuroparaly tica) . Rare 
 trophic neuroses sui generis (Raynaud's disease) may lead to ulceration and 
 gangrenous destructive processes. The blister-like eruptions of herpes 
 zoster belong to a dermatologic-neurologic borderland; they are combined 
 nearly always with neuralgias in the nervous region concerned. We shall 
 have to discuss all these points in more detail later. 
 
 The physical methods of internal medicine in general use are of but 
 small service in the examination of nervous patients. Percussion may 
 occasionally be employed on the skull, in internal affections of the cranium; 
 a peculiar flat tinny percussion note (reminding of the bruit de pot fele), 
 the so-called " 'cracked pot note'" is occasionally found on the diseased side 
 in cerebral tumors. Palpation sometimes serves for the recognition of an- 
 atomic changes in the peripheral nerves and nerve sheaths. It may also be 
 important in discerning the causes of a dysbasia arteriosclerotica by the 
 absence or the smallness or the hardness of the peripheral arterial pulse. 
 By auscultation, we recognize in rare cases the presence of vascular tumors 
 and aneurysms of the arteries inside the cranium. As an aside we may re- 
 mark that auscultation and percussion naturally may be of use to us indirectly 
 also in neurology, in the recognition of the causes of peripheral paralyses 
 (recurrens paralysis in aortic aneurysms), etc., and that the establishment of 
 sensitiveness to pressure in bones and nerve trunks (caries, neuralgias) may 
 be of decisive importance. 
 
 Great weight must from the first be laid upon the correct interpretation 
 of disturbances of consciousness and the psyche. Dimming of conscious- 
 ness is, on the whole, easily recognized; the lightest form is usually desig- 
 nated somnolence, a condition of sleepiness from which the patient may how- 
 ever be awakened by a mere call, in which correct answers can be obtained, 
 the next higher degree as sopor, with retained reflexes, in which awakening 
 is produced only by means of violence (by stimulation of the skin, etc.), 
 the highest degree as coma, with the reflexes lost. Here the patient can not 
 be awakened even though force is used. All three forms occur in most 
 cerebral diseases as well as in all those internal diseases that affect the cen- 
 tral nervous system. 
 
 Knowledge of psychic disturbances in detail belongs to the realm 
 ■of psychiatry, with the fundamentals of which, however, every neurologist 
 must be acquainted. How important are even the lightest forms of these 
 disturbances, often how typical ! The patient's mood is occasionally charac- 
 
THE PSYCHE, THE SENSE ORGANS, VOICE AND SPEECH 5 
 
 teristic of his disease; the euphoric mood is seen mostly in multiple sclero- 
 sis, in Friedreich'' s disease; gloomy morose psychic states in paralysis agitans; 
 exalted (maniacal) moods in progressive paralysis (which, as a fact, all 
 but belongs to the purely psychic realm). Stupor, the almost reactionless 
 depressive state rarely occurs in pure nervous diseases; more frequently 
 the talkativeness of most neurasthenics which must not be at once assigned 
 to the sphere of maniacal conditions. Especially typical is it in traumatic 
 neuroses, in which, of course, the exclusion of the patient's desire for lucre 
 and of conscious or unconscious simulation is one of the most difficult tasks 
 of the neurologist. 
 
 In order to have a clear picture of the psyche of nervous sufferers, it is 
 important to win their confidence, to allow them to talk. 
 
 Then gradually, by clever inquiries, the investigator may discover the 
 severe psychic symptoms: delusions, illusions, hallucinations. Disturb- 
 ances in memory, especially for more recent events, are common and impor- 
 tant. Intelligence is often considerably clouded (dementia, imbecility, 
 idiocy), sometimes congenital, with especial frequency the result of diseases 
 during infancy (hydrocephalus, encephalitic processes) somewhat less 
 frequently acquired at an advanced age (multiple sclerosis, epilepsy, pro- 
 gressive paralysis of the insane, etc.). For many disturbances in memory 
 and intelligence, the ability to count is of peculiar importance; therefore one 
 should set the patient problems with this in view. For all psychic condi- 
 tions, finally, the writing of the patient may be significant, which further- 
 more discloses numerous disturbances in motility (ataxia, all forms of 
 tremor, writer's cramp, etc.). 
 
 All of the lighter and some of the more severe psychic disturbances are 
 found in the restricted field of neurology, if we except progressive paralysis 
 with the enormous variety of its psychic symptomatic picture, in all the 
 inflammatory processes inside the cranium, in cerebral tumors, hydro- 
 cephalus, traumatisms of all sorts, in multiple sclerosis, in some "system" 
 diseases (Friedreich's ataxia), in that strange combination of a multiple 
 neuritis with psychic disease, known as Korsakow's psychosis, and in numer- 
 ous neuroses (neurasthenia, hysteria, epilepsy, chorea, paralysis agitans, etc.) . 
 
 The general examination of nervous patients in respect to visible external 
 changes, the results of physical examination in the narrower sense and of 
 that of the psyche, are to be followed by the examination of the organs of 
 sense. 
 
 B. Testing the Sense Organs, Voice and Speech 
 
 1. The Eye 
 
 Every test of the function of the organ of vision should begin with an 
 examination of the pupils. 
 
6 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 In the normal state, both pupils are of equal width; congenital inequality 
 (anisocoria) occurs rarely and in contradistinction to other authors we do 
 not interpret it as a sign of a neuropathic disposition. In rare cases, this 
 inequality alternates between the two eyes; each eye in alternation with the 
 other shows now a miosis (contraction) now a mydriasis (dilatation). We 
 know as little of the origin of these "rapidly alternating" pupils as we do 
 of the so-called hippiis, the rapidly changing width of the pupil of one eye 
 without external stimulation. 
 
 Usually, however, pupillary differences signify a disease of the tracts 
 subserving this contraction and expansion, at least in one eye. Here, the 
 wider as well as the narrower pupil may be the one diseased (no absolute 
 values exist for pupillary width in any form that can be used by neurologists). 
 To decide whether both eyes or which of the two is the one affected, we test 
 the pupillary movements. 
 
 They consist in a contraction of the pupils controlled by the m. sphincter 
 pupillas (oculomotorius), which appears reflexly, when light falls upon the 
 eye and upon accommodation, as voluntary concomitant movement, with 
 convergence and energetic contraction of the orbicularis oculi, and in a 
 dilatation of the pupils controlled by the m. dilator pupillae (sympathetic), 
 which appears reflexly in the dark, and upon pain stimulation (for theory 
 and localization of pupillary movements see special section in this text-book) . 
 
 Testing of the reaction to light is best carried out in a darkened room, by 
 means of a reflector (forehead-, eye-mirror) throwing concentrated light, 
 coming from a source behind the patient, into one eye. This is the best 
 way of establishing the normal, rapid and extensive contraction of the 
 pupil under examination, the absence of reaction, the reflex-pupillary rigidity 
 to light {Argyll-Robertson's phenomenon) as well as all the intermediate 
 steps, the most important of which is the "sluggish" reaction, a slow con- 
 traction of the pupil, comparable in its course to several other pathological 
 reflexes (Babinski toe reflex) . 
 
 Almost simultaneously with the process in the illuminated eye there 
 appears in the non-illuminated, the so-called "consensual" contraction; 
 observation of it may be important for the determination whether reflex 
 disturbances are to be localized in the centripetal (opticus) or the centrif- 
 ugal (oculomotorius) reflex tract, since in the latter case, the non-illuminated 
 pupil contracts consensually in spite of the areflexia of the illuminated 
 pupil, and not in the former. 
 
 Theoretically, sluggish reaction and loss of the reaction to light of the 
 pupil may of course appear in every disease that interrupts the reflex tract 
 at any point. Practically, however, this is the case only in relatively few 
 well characterized diseases of the central nervous system, of which the 
 metasyphilitic (tabes, progressive paralysis), meningitis and tumor cerebri 
 may be mentioned as the most frequent. The rigidity to light may be uni- 
 
THE EYE, VISION, PUPILS 7 
 
 lateral or bilateral; pupils with total rigidity to light are somewhat more 
 frequently miotic, contracted (often to the size of a "pin head"), than 
 mydriatic, dilated. 
 
 Naturally, testing the effect of light falling upon the eye may — and this 
 will be the rule in investigations as to orientation — take place in daylight; 
 one then has the patient (this is the best way) look through a well lighted 
 window at a distant object and fixate it, and covers — this is to be recom- 
 mended in all cases — both eyes at the same time, and then by taking away 
 the covering (hand, etc.), tests separately the direct and consensual light 
 reaction of each eye. But this test may be made exceedingly difficult by 
 poor daylight, very dark iris, or by the light reflected from the cornea. 
 
 Independently of the pupillary contraction upon entrance of light, con- 
 traction upon accommodation or convergence may take place. Normally, 
 both pupils energetically contract upon accommodation of the eye for near 
 objects (if the mm. interni are normal, with simultaneous convergence). 
 This contraction may be retained in the absence of the light reaction (fre- 
 quently in tabes) or it may be missing at the same time as the light reflex 
 (tabes, progressive paralysis, cerebral tumors) constituting total pupillary 
 rigidity (ophthalmoplegia interna); a case of isolated accommodation pa- 
 ralysis occurs very rarely and is practically negligible. 
 
 The voluntary contraction of the pupil upon innervation of the orbicu- 
 laris oculi is easily tested, but has not yet attained pathological importance. 
 
 Reflex dilatation of the pupils is far less important. We test it by 
 shading the eye in day light, as well as by pain stimulations (pinching or 
 pricking the skin of the cheeks). The undubitably justifiable conclusion 
 that an absence of the dilatation reflex, must, in general, point to an affection 
 of the sympathetic, can be used but rarely neurologically with our slight 
 knowledge of the processes in the sympathetic region. On the other hand, 
 we find this absence frequently with the synchronous absence of contrac- 
 tions to light in the pupils, in tabes, progressive paralysis of the insane, 
 severe affections of the substance of the brain; the pupils in this case are 
 neither contracted nor dilated to a high degree, but are of medium width. 
 
 Abnormal miosis may depend upon absence of the reaction to light as 
 well as upon paralysis of the dilator pupillae, and also (rarely, almost only 
 in hysteria) upon a sphincter spasm. In analogous manner, we see mydri- 
 asis in sphincter paralysis, spasm in the dilator pupillae and not infrequently 
 as a purely psychic reaction in terror, great mental exertions, etc. Neu- 
 rasthenics often have permanently very dilated pupils. 
 
 Finally it must be mentioned that in old age all the pupillary reflexes 
 usually decrease, and that certain poisons tend to dilate the pupil (a tropin, 
 hyoscin), others to contract it (eserin, morphine). In chloroform narcosis, 
 the pupils, as a rule, are immobile to light and dilated. 
 
 Next to the test of pupillary reaction, comes that of the external muscles 
 
8 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 of the eye. Here are concerned the m. rectus ext. (n. abducens), obliquus 
 sup. (n. trochlearis), obliquus inf., rectus int., levator palpebrae {all the 
 n. oculomotorius), while the orbicularis oculi, supplied by the facialis, is to 
 be dealt with in the test of musculature of facial expression. 
 
 Besides the action of the m. levator palp., which raises the upper eyelid, 
 only the m. rectus ext. and the rectus int. are to be tested in simple manner, 
 by asking the patient to look energetically towards the right or the left, or 
 better, with head fixed, to follow with the eyes, as long as possible, an object 
 that is moved in the direction to the right or to the left. If one of these two 
 muscles is hindered from acting, the eye concerned does not follow in the 
 direction of its normal contraction. A second necessary consequence is 
 the appearance of double images (with retained vision), which, however, are 
 frequently consciously or unconsciously suppressed by the patient, since he 
 fixates with one eye only. In slight pareses of one muscle, the test for 
 double images is often the more delicate as contrasted with that for eye 
 movement; for this purpose one asks the patient to say when the object 
 which is being slowly moved sidewards is seen by him double or confused, 
 and in the former case, in what relation the double images stand to each 
 other. To facilitate the recognition of both images (of which one is usually 
 seen more brightly, that is, more clearly), one may place a red glass before 
 one eye; the red and the white image can then, even though partly overlap- 
 ping, be easily distinguished from each other. In paresis of a rectus int, 
 the double images are at the same height and are "crossed," i. e., the image 
 that "belongs" to the right eye lies on the left side; in paresis of the rectus 
 ext. the double images are at the same height and "uncrossed." 
 
 Examination of the obliqui and the rectus sup. and inf. is more difficult, 
 since their effects are always somewhat combined so as to move the eye 
 upwards, downwards, or in a circle. The rectus inf. moves the eyeball down- 
 wards and inwards, the obliquus sup. downwards and outwards and rotates it 
 somewhat; the obliquus inf. upwards and outwards, the rectus sup. upwards 
 and inwards, with synchronous rotation. In these muscles, the loss of 
 movement is, as a rule, very slight and difficult to test; it is best to examine 
 the double images, for which the following scheme holds good : 
 
 In paralysis of the rectus inferior, the double images appear only on 
 looking down, the images are crossed, inclined to each other, and one below 
 the other, the false image is the lower, and upon lowering of the object 
 concerned, separates from the true image, which is higher. 
 
 In paralysis of the obliquus superior, double images appear upon looking 
 up; the pictures are uncrossed, inclined, and one below the other. 
 
 In paralysis of the obliquus inferior, double images appear upon looking 
 up; they are inclined, one below the other, but uncrossed. 
 
 In paralysis of the rectus superior, double images appear upon looking 
 up; they are inclined, one above the other, and are crossed; the false upper 
 
THE OCULAR MUSCLES 9 
 
 image leaves the true lower image upon raising of the object used in the 
 test. 
 
 Weakness of the eye muscles causes still other phenomena of functional 
 loss, which, however, are of less diagnostic service to the neurologist. These 
 are for every single muscle the secondary contracture of its antagonist, which, 
 when vision is turned directly forwards, conditions an abnormal position, a 
 strabismus (most clearly in paresis of the rectus int. — strabismus divergens, 
 and in paresis of the rectus ext. — strabismus convergens); furthermore, 
 occasionally, the secondary deviation of the unaffected eye, the abnormal 
 position of the head, and finally, upon an attempt to exert the paralyzed 
 muscle, some twitchings of the bulbus in the sense of the working of this 
 muscle (so-called paresis-nystagmus, especially in conditions of weakness 
 in convalescents). 
 
 Naturally besides the paralysis of individual, and the paralyses of all 
 the external muscles (ophthalmoplegia externa), or of all the eye muscles 
 (total ophthalmoplegia), there occur also variously grouped paralytic con- 
 ditions in several muscles. Of these should be mentioned the so-called 
 conjugate paralysis of the eye muscles, with loss of the power to turn both 
 eyes laterally in the same direction (affecting, therefore, only one rectus 
 int. and ext. of each eye) ; here the convergence of bulbi is usually retained 
 in spite of the necessary co-operation of the paralyzed internus concerned. 
 Then there is convergence paralysis, the counterpart of the disturbance just 
 mentioned, in which both bulbi retain their ability to turn laterally, and 
 then the rare divergence paralysis, which is difficult to recognize (the patient 
 can not bring back to their normal position the bulbi which have been con- 
 verged to look at an object near by) . 
 
 Heretofore we have spoken only of the paralyses of the individual 
 muscles. The muscular groups supplied by one of the cerebral nerves men- 
 tioned, may also be paralyzed on one or both sides. When the n. abducens 
 and the n. trochlearis are paralyzed, the disturbances may readily be deduced 
 from what has already been said. In paralysis of the oculomotorius, the eye 
 affected is, as a rule, fixated towards the outside downwards; and of active 
 movements, there is possible only a slight outward rotation of the bulbus. 
 Furthermore, in total oculomotorius paralysis, the pupil concerned is dilated, 
 and, as a result of paralysis of the levator palpebrae, the upper eyelid droops 
 {ptosis). This ptosis may appear also as an isolated phenomenon (in 
 hysteria, occasionally likewise in organic diseases as for instance tabes). 
 For the rest, there occur isolated and grouped paralyses of the eye muscles 
 in a great many central and peripheral (eye-muscle-nerve) affections of the 
 central nervous system, among which must be mentioned above all, the 
 syphilitic and metasyphilitic diseases (tabes, progressive paralysis) as well 
 as some toxic-infectious processes (diphtheria). 
 
 As we shall see later, in all disturbances of motility in the human body, 
 
io GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 a second group of disturbances besides paralysis may be thought of, i. e., 
 hyperkinesis, spasms, in which we distinguish tonic (continuous tension of 
 the diseased muscle) and clonic spasms (change between tension and relaxa- 
 tion, twitching movement). This holds also for the eye muscles, in which, 
 however, spasms are very much rarer than paralyses. Thus a strabismus 
 based upon genuine spasm (usually convergent) is very rare, observed almost 
 exclusively in hysteria. Let us mention here that by far the greatest number 
 of all cases of strabismus (so-called muscular or concomitant squint) are 
 not based upon a muscular paralysis, but, so to say, only upon conditions 
 of weakness, a so-called disturbance in the equilibrium of the muscles; that 
 in spite of the changed position of the bulbi, it proceeds usually without 
 double vision and disturbances in motility, and that as a result, the typical 
 test for the individual eye muscles gives a negative result. Cf . on this point 
 text-books on ophthalmology. We must sometimes regard as tonic spasm, 
 the remarkable phenomenon of conjugate deviation, in which both eyes are 
 held fixed in a position of rotation towards the right or the left (especially 
 in cerebral tumor in certain regions, often accompanied with rotation of 
 the head to the same side). In rarer cases, it may rest also upon a paralysis 
 of the antagonists. 
 
 The various forms of nystagmus may be interpreted as clonic spasms 
 of the eye muscle: short twitchings following one another in slow or rapid 
 succession, of one or both bulbi in a lateral direction (nystagmus horizon- 
 talis), in a vertical direction (N. verticalis), or in rotation (N. rotatorius). 
 We find it (cf. above ) frequently in pareses of individual muscles in respect 
 to and in the direction of their activity (especially N. horizontalis, which, 
 moreover, in "end positions" of the bulbi, in the extreme lateral direction, 
 are not infrequently found in the healthy, too), furthermore, congenital 
 (here often very intensive, when vision is directed straight forward), as 
 vocational disease (miners), in blindness and finally — this is of especial 
 importance for the neurologist — in a series of affections of the brain, above 
 all in multiple (cerebro-spinal) sclerosis. 
 
 While testing the individual eye muscles is one of the most difficult 
 chapters in the diagnostics of nervous diseases and now and then makes 
 .assistance from an ophthalmologist desirable, examination of the back- 
 ground of the eye should, in its main points, be well mastered by the neurolo- 
 gist. We may exclude here the real affections of the retina and chorioidea, 
 which less frequently occupy the attention of the neurologist (at most occa- 
 sionally as sign of neuropathic disposition, as in retinitis pigmentosa and 
 other congenital diseases), but must consider that sometimes the diagnosis 
 of a cerebral disease seems to be erroneously confirmed by an affection of the 
 retina, as in a retinitis albuminurica confined to the disc and simulating a 
 neuritis optica. 
 
 Two ophthalmoscopic findings especially are of interest to us : 
 
OPTIC NEURITIS AND ATROPHY n 
 
 Neuritis optica, or choked disc and atrophy of the optic nerve. 
 
 In neuritis optica the disc appears clouded, reddened, or grayish red in 
 color, the veins are dilated, the arteries of the disc contracted, the edge of the 
 disc not clearly marked. The diameter may be considerably increased. 
 In pronounced choked disc, there is, besides, an ophthalmoscopic, usually 
 easily recognizable protrusion of the disc; the blood vessels appear to be 
 snapped off at the edge of the disc, and disappear in the diffuse redness of 
 the enlarged disc. 
 
 Concerning differentiation between neuritis optica (papillitis) and choked 
 disc, it is all the more difficult, considering what has been said, inasmuch 
 as there is by no means a consensus of opinion as to the cause of the disease. 
 A mechanical congestion (cerebral tumor) through increase in the intra- 
 cranial pressure (accumulation of large quantities of cerebro-spinal fluid 
 inside the cranium) and a gathering of fluid in the sheath of the optic nerve 
 may give rise to ''papillitis" as well as choked disc; on the other hand, toxic 
 products in infectious and other diseases may evoke choked disc also in 
 addition to the neuritis optica, or papillitis which usually results. In general, 
 the ophthalmologist will incline rather to a differentiation of the two condi- 
 tions, the neurologist to the identification of them, with the restriction that 
 they both represent different degrees of the same affection. 
 
 The acuteness of vision in neuritis optica is nearly always normal in the 
 beginning of the disease, and very frequently later also, at least central 
 vision, whereas contractions of the visual field (cf . below) frequently appear 
 later; but the beginner must never forget that there may exist even a high 
 degree of choked disc without the knowledge of the patient. Thus the 
 ophthalmoscopic picture here by no means corresponds to the degree of 
 the visual disturbance. 
 
 The recognition of choked disc is in nearly all cases easy; only the 
 lightest degrees of incipient papillitis may cause difficulties; of course one 
 must not be deceived by the apparent indistinctness of the edges of the 
 disc in refraction anomalies. 
 
 Naturally we find choked disc most frequently in processes which cause 
 congestion, especially in tumor of the brain. Here it is usually (but by no 
 means always) bilateral. The situation and size of the tumor are not always 
 decisive as to the origin and intensity of choked disc, still, very large tumors 
 can scarcely run their course without accompanying papillitis, while, on 
 the other hand, we must remember that frequently in recent times there 
 have been observed cases of typical tumor symptoms with choked disc, 
 though in the operation or the autopsy, no tumor was found (Nonne). 
 Meningitis of any kind may cause papillitis; it is found with special frequency 
 in meningitis serosa and in hydrocephalus. In all forms of cerebro-spinal 
 syphilis, in lead poisoning, in acute infectious diseases, in chlorosis, it occa- 
 sionally occurs; a rheumatic inflammation of the opticus, comparable to 
 
12 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 "rheumatic" paralysis of the eye muscles is also observed. On the other 
 hand, it is rarely or never found in encephalitis, nor in apoplexies of any 
 kind, and it is lacking almost without exception in two diseases which are 
 accompanied by very typical changes in the background of the eye, that is, 
 in tabes and in multiple sclerosis. In both cases, as a rule, we find in its 
 stead atrophy of the optic nerves. 
 
 Atrophy of the optic nerve is ophthalmoscopically recognizable by 
 the abnormal pallor of the disc, which may lead to a porcelain white colora- 
 tion or discoloration, but which may also present only a slightly paler color 
 in one-half of the disc. In the latter case, diagnosis is nearly always difficult, 
 since, as is well known, there are present in normal conditions differences in 
 clearness in the separate parts of the disc, and especially the part first affected, 
 the temporal half of the disc is mostly subject to these fluctuations. In 
 these incipient cases, there is lacking, too, that sharp almost hard outline of 
 the white disc against the red retina, that is so characteristic of the final 
 stage. The width of the vessels is usually not changed to any considerable 
 extent. 
 
 Atrophy of the optic nerve may appear as a primary phenomenon, and it 
 may also represent the final stage of a neuritis optica, that is, be a secondary 
 phenomenon. The latter, which may occur in all the diseases men- 
 tioned above, is usually accompanied by considerable disturbance in vision 
 (especially loss in the visual field) , whereas primary atrophy may exist for a 
 long time without any subjective disturbance, and is therefore often over- 
 looked (sometimes, especially in multiple sclerosis, there occur also sudden 
 transitory visual disturbances of high degree, though the background of the 
 eye shows nothing else but the always equally slight "temporal paling" of 
 the disc). Except when it follows traumatism, opticus atrophy is nearly 
 always bilateral, though often developed in different degree in the two eyes. 
 Primary atrophy of the optic nerves is found almost only in tabes, progress- 
 ive paralysis of the insane, and multiple sclerosis; in the former case it is 
 usually total and progressive; in the latter partial and more or less stationary. 
 Whether as a matter of fact, it is not frequently secondary in multiple scle- 
 rosis, arising after a neuritis belonging to the prodromal stage so often mis- 
 taken for a "hysteria," must, owing to the scarce findings from examination 
 at this prodromal stage, remain undecided. 
 
 Cases of so-called neuritis retrobulbaris occur rarely in the practice of the 
 neurologist, but more frequently in that of the ophthalmologist. Ophthal- 
 moscopically, they are recognizable not at all, or by an (secondary) atrophy 
 of the papilla of moderate degree; on the other hand, they show the typical 
 visual disturbance of a central loss in the visual field for colors (red and 
 green). Retrobulbar neuritis is usually toxic, or toxic-infectious, and 
 occurs as an isolated phenomenon or with a polyneuritis which owes its 
 origin to the same causes. The most common causes are chronic poisoning 
 
THE VISUAL FIELDS 13 
 
 with alcohol or nicotine, also, diphtheria. A medicinal neuritis retrobul- 
 baris occurs also. Frequently retrobulbar neuritis optica has been observed 
 as a very early prodromal symptom of multiple sclerosis {Uhthoff, and others). 
 
 The real examination of visual disturbances in nervous diseases — test of 
 acuteness of vision — is, in the main, the task of the oculist. But neverthe- 
 less, it is evident that no neurologist will fail to attempt an approximate 
 determination with the assistance of Snellen's types, of counting fingers at a 
 certain distance, etc. An examination of the visual field, which is generally 
 easily feasible, is earnestly recommended. While one covers one of the 
 patient's eyes, and asks him to fixate a point with the other, at the distance 
 of from one-half to one meter, small pieces of white or colored paper are 
 introduced into the visual field area, and one moves the pieces of paper from 
 the outer edge of the visual field (that is, behind or above or near the patient's 
 head) slowly in the direction of the point fixed by the patient up to that point 
 itself, and then tells the patient to say when he catches sight of them first 
 and whether they disappear again on the way (central scotoma). For 
 practical reasons, one chooses as the main direction of these movements, 
 first the main meridians, above and below, outside and inside, then even- 
 tually an intermediate meridian. All this can be done far more easily with 
 the various perimeters, some of which are quite cheap, on which one can 
 read the degrees directly (degrees in width of the approximately half spheri- 
 cal field of vision) . One must consider that the visual field in normal con- 
 dition is variously extended (for white above, 50 to 6o°; below, 6o° to 70 ; 
 outside, 90 ; inside, 6o°; for colors, considerably less) and that individual 
 differences occur also. One must never, especially when using a perimeter, 
 fail to enter the results upon printed diagrams of the visual field, as this is 
 the only way to get an accurate picture of the visual field. 
 
 Disturbances in the visual field occur with and without other visual 
 disturbances, and with or without changes in the background of the eye. 
 We differentiate the concentric contraction, the peripheral scotoma, the 
 central scotoma and hemianopsia. The concentric contraction, a diminution 
 on all sides (for white and colors, or only for colors) of the visual field, as 
 well as scotoma, the loss in spots, which, according to the location of the spot 
 in the visual field, is designated central (near the point of fixation) or periph- 
 eral, we find, as a rule, in neuritis optica (papillitis or neuritis retrobulbaris) 
 or in atrophy of the optic nerve. In contraction, the disease of the nerves is 
 more frequently diffuse, in scotoma it strikes only some bundles of fibres. 
 Therefore we find both disturbances in tabes, multiple sclerosis, polyneuritis, 
 in the processes above mentioned which lead to choked disc, and, finally, 
 isolated also; the concentric contraction is frequent too in hysteria. 
 
 Hemianopsia, the loss of an entire half of the visual field is less frequent. 
 Here, it is a question nearly always of the loss of the outer or inner (not the 
 upper or lower) half, and, moreover, preponderantly in both eyes at once, 
 
i 4 GEXERAL DIAGXOSTICS OF NERVOUS DISEASES 
 
 corresponding to the partial crossing of the fibres of the optic nerves. One 
 speaks of homonymous (bilateral) hemianopsia, if both right or both left 
 halves are lost; on the other hand, of bitemporal hemianopsia {heteronymous 
 hemianopsia), if both external halves of the visual field are missing. A 
 binasal hemianopsia occurs virtually never. Hemianopsia is easily recog- 
 nized by the test suggested for the visual field. 
 
 Hemianopsia is found in lesions of the chiasma and of the tractus opticus; 
 and, moreover, the latter produces homonymous hemianopsia, and, on the 
 other hand, the lesion of the chiasma (at least of a middle portion containing 
 the crossing of the fibres of the optic nerve) leads to bitemporal hemianopsia. 
 Diseases of the chiasma are, as a rule, evoked by small tumors at the base 
 of the brain, while the tractus may naturally be affected by every agent that 
 injures the substance of the brain. Especially important are the cases in 
 which a homonymous bilateral hemianopsia presents the only sign of a 
 lesion (hemorrhage, tumor) of the occipital lobe, in which the visual center 
 is situated. 
 
 Hemianopsia, in contrast to concentric contraction, is very clearly 
 present in the consciousness of the patient, since it is frequently combined 
 with other disturbances, concentric contraction of the field of vision, visual 
 hallucinations, etc. Theoretically, moreover, every hemianopsia belonging 
 to the tractus opticus should exhibit an objective symptom; there should be 
 no pupillary reflex to light thrown upon the eye, if one illuminates only the 
 "blind" half of the retina (hemianopic pupillary rigidity). This does 
 occur occasionally, but is often difficult to demonstrate. 
 
 Monocular diplopia, double vision in one eye alone, an abnormality 
 occurring almost exclusively in hysteria, does not belong to the real organ- 
 ically conditioned disturbances of the apparatus of vision; nor does exoph- 
 thalmus, the protrusion of one or both eyes. We find this phenomenon 
 unilaterally in tumors of the orbit or — through congestion — also in tumors 
 of the interior of the cranium; bilaterally, above all, in Basedow's disease. 
 In Basedow's disease the origin of the symptom is not altogether clear; but 
 it probably depends upon a swelling of the retrobulbar tissue, such as occurs, 
 and sometimes periodically under other conditions. 
 
 2. Hearing 
 
 While examination of the eyes has proved to be very useful, strangely 
 enough, the value of the ear findings has so far proved to be comparatively 
 slight for neurological diagnosis. In part this is due to the fact that genuine 
 nervous diseases of the ear are really rare; in part also to the difficulty of 
 diagnosis in these affections. Only very recently have clinicians (Witt- 
 maack) begun to look with more interest upon the diseases of the inner ear. 
 
 Three groups of symptoms are of use in neurologic — otiatric diagnostics; 
 
HEARING 15 
 
 subjective auditory disturbances (difficulty in hearing, hyperacusis, tinnitus 
 aurium, etc.), objective changes in the ability to hear (Weber's and Rinne's 
 experiments) vertigo, nystagmus, disturbances in equilibrium. All the 
 other resources of the otologist, especially the whole of otoscopy, may at 
 most be used indirectly by the neurologist, for the entire group of disturbances 
 in the sound conducting apparatus does not allow any conclusions to be 
 drawn, as to the presence of a nervous disease, excepting only anomalies, 
 like the occasional occurrence of laxness of the ear drum through atrophy 
 of the m. tensor tympani in advanced dystrophy. 
 
 Of the subjective auditory disturbances , it is most difficult to use tinnitus 
 aurium, ringing or buzzing of the ears, etc., for local diagnosis. This 
 occurs, it is true, especially in affections of the inner ear, which, however, 
 may be of most varying types: arterio-sclerosis, neuritis acustica, transitory 
 disturbances, usually of unknown origin, such as tinnitus aurium of 
 chlorotics, the ringing in the ears which appears paroxysmally in Meniere's 
 disease, etc. Hyperacusis (oxyecoia) is found in paralysis of the facial nerve 
 (cf . above) , and in some neuroses, especially in hysteria. Every degree of dif- 
 ficulty in hearing occurs in diseases of the inner ear, in those that are really 
 "nervous" (labyrinth and cochlea) as well as in those of the sound conduct- 
 ing apparatus (tube, tympanic cavity, drum, auditory meatus), and does not, 
 upon superficial examination permit of differential diagnosis between these 
 two groups. This first test of the ability to hear is made as follows: after 
 telling the patient to close one of his ears, words or numbers are whispered 
 or he is allowed to hear the ticking of a watch, and the limit of audition is 
 measured in meters (from the ear). Rinne's and Weber's experiments 
 afford a more delicate test, in as much as both are concerned with the con- 
 ductivity of the bones of the head. 
 
 (a) Rinne's Experiment. — The handle of a tuning fork pitched at low 
 c, after being set in vibration is placed upon the mastoid process of the 
 affected ear. In normal cases, when the sound is no longer audible to the 
 patient by this sort of "bone conduction" perception is still possible 
 through "air conduction," if one holds the fork close to the ear (Rinne's 
 positive test"). The case is the same — usually with diminished intensity 
 of perception — if there be present difficulty in hearing of nervous, labyrin- 
 thine type. If, on the other hand, Rinne's test is "negative," that is, if 
 the sound be heard longer by means of the bones than through conduction 
 by air, which occurs only in difficulty in hearing — this defect must be 
 traced back to the sound conducting apparatus. 
 
 ib) Weber's Experiment. — A vibrating tuning fork is placed upon the 
 middle of the frontal or occipital aspect of the head. In normal conditions 
 the sound is heard by each ear with equal intensity. Should, however, a 
 unilateral disturbance in hearing obtain — and the experiment is valuable 
 only in such conditions — and the sound be more perceptible by the better 
 
16 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 hearing ear, then the disturbance lies in the reception (nervous) apparatus 
 of the other, namely the affected ear. But should the patient localize the 
 sound in the less perceptive ear then the disturbance lies in the sound con- 
 duction apparatus of the affected ear. 
 
 We must mention, furthermore, that in diseases of the inner ear, as a 
 rule, the distinctness of audition is impaired especially in regard to the 
 higher tones. 
 
 Disturbances in equilibrium, nystagmus and vertigo, in so far as 
 they proceed from the ear, are probably always to be traced back to the inner 
 ear, the nervous apparatus (also indirectly in diseases of the tympanic cavity) . 
 The former occur spontaneously especially in the peculiar attacks that were 
 formerly supposed to be characteristic of the general concept: "Meniere's 
 disease," and besides this, may easily be evoked in pathological cases 
 (galvanization with weak currents). A feeling of dizziness (ear vertigo), 
 occurring in attacks as well as continuously, is very rarely lacking in disease 
 of the inner ear. A real nystagmus is, as a rule, evoked in labyrinthine 
 affections, upon turning the axis of the body. According to the most recent 
 views, these symptoms also make possible a differential diagnosis between 
 disease of the n. vestibularis, or of the labyrinth, and of the n. cochlearis, 
 or of the cochlea and the organ of Corti; to the n. vestibularis belong dis- 
 turbance in equilibrium and the feeling of dizziness, to the cochlearis, the 
 nervous auditory disturbance, in a stricter sense. 
 
 "Nervous" auditory disturbances are found in fracture of the base of 
 the skull, tumors of the base, arterio-sclerosis, in the changes due to old 
 age, and known as "otosclerosis," in multiple sclerosis, in tabes dorsalis 
 (as genuine degeneration of the nerves, comparable to atrophy of the optic 
 nerve), in isolated neuritis, or in neuritis complicated with polyneuritis 
 (Frankl-Hochwart) , hysteria, neurasthenia, and especially in traumatic 
 neurasthenia (the favorite hypothesis of "labyrinthine concussion"). 
 
 3. Taste and Smell 
 
 The sense of smell is of least neurological usefulness, in spite of its so fre- 
 quent disturbances in daily life; disorders are mostly dependent upon catarrhs 
 of the mucous membranes, not upon diseases of the olfactory nerves them- 
 selves. Above all, therefore, one must establish in neurological investiga- 
 tion whether the olfactory pathways are clear, and no catarrh is present. 
 If everything is well in both these respects, one must test the acuteness of 
 the sense of smell (naturally for each nostril separately) by holding odorous 
 substances in front of the nose, avoiding of course those which stimulate 
 the sensory end organs of the trigeminus (ammonia). As a rule, I use 
 asafoetida, tincture of valerian, oil of cloves and oil of roses (or eau de Co- 
 logne). A gradation of the acuteness of the sense of smell is all the less 
 
TASTE AXD SMELL 17 
 
 possible for the various odors, since the delicacy of the sense of smell, the 
 "good nose," varies exceedingly in normal conditions and in a large number 
 of healthy persons is developed very scantily. The odorous substances 
 mentioned, should, as a rule, be recognized. 
 
 In hysteria there occur hyperosmia (hyper-sensitiveness) and anosmia 
 (lack in sensitiveness to olfactory stimuli). In tumors of the base of the 
 cranium, of the ethmoid bone, etc., we find occasionally unilateral lowering 
 of the ability to smell, likewise, in meningitis and fractures. 
 
 Somewhat more useful is the test of the sense of taste. A prior condition, 
 however, is that the sense of smell be not simultaneously disturbed (catarrh 
 in the head) . We conduct the test as follows : with a little glass rod we place 
 in succession a drop each of solutions representing the four main qualities 
 of taste, sweet, sour, salt, bitter — sugar, vinegar, salt, quinine — on the out- 
 stretched tongue in this manner: 
 
 (a) on the anterior half of the tongue, separately on the right and left 
 sides ; 
 
 (b) on the posterior half of the tongue, separately on the right and left 
 sides. 
 
 After applying each drop of the solution one waits a few seconds, then 
 wipes off the tongue energetically with a dry cloth, has the patient withdraw 
 his tongue and state the taste concerned. We do this for the following 
 reasons : In respect to the sense of taste, the anterior half of the tongue is 
 under the dominion of the chorda tympani, that is, of the n. lingualis; the 
 posterior half and the palate under that of the n. glossopharyngeus. There- 
 fore the test is made separately. The tongue is drawn back because many 
 patients have no taste sensations upon the extended tongue. In general sour 
 is better recognized on the tip of the tongue, bitter on the posterior half of 
 the tongue. 
 
 Disturbances in taste come to be diagnostically important almost only 
 in diseases of the n. facialis for clearer localization (chorda tympani), also, 
 however, in disturbances of the n. trigeminus III. The diseases of the 
 glossopharyngeus which are rare in themselves, are concerned only excep- 
 tionally. Progressive paralysis and neurasthenia (especially their depress- 
 ive forms) are frequently accompanied by disturbances in taste, which in 
 that case, however, affect the entire tongue. 
 
 4. Disturbances in Voice and Speech 
 
 These processes belonging to the realm of the cerebral nerves, if not to 
 that of the actual nerves of sense, may be briefly discussed here. 
 
 Disturbances in voice occur mainly in two forms: as voicelessness (aphonia) 
 and as hoarseness; abnormally high pitch of the voice (squeaking voice, 
 etc.), is rare, occurring almost exclusively in hysteria. Aphonia also, in the 
 
i8 
 
 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 great majority of cases in which it comes to the notice of the neurologist, is 
 of hysteric, that is, of psychogenous, origin, but even detection of other 
 stigmata, etc., must not, as occasionally happens, deter the investigator from 
 Iaryngoscopic examination. For even if it occurs rather frequently that a 
 hysteric aphonia is treated for weeks as chronic laryngitis, still, a patient 
 suffering from catarrh has sometimes been branded as hysteric — the worse 
 
 Fig. i — Bilateral paralysis of the 
 posticus in the moment of inspiration. 
 {After Ziemssen.) 
 
 Fig. 2. — Paralysis of both N. thyreoary- 
 taenoidei interni. {After Ziemssen.) 
 
 Fig. 3. — Paralysis of the recurrens at the left side (position during inspiration). {After Ziemssen.) 
 
 Fig. 4. — Paralysis of the arytaenoi- 
 deus. {After Ziemssen.) 
 
 Fig. 5. — Bilateral paralysis of the thy- 
 reoarytaenoidei with synchronous paresis 
 of the arytaenoideus. {After Ziemssen.) 
 
 fate! The Iaryngoscopic examination must therefore be as familiar to the 
 neurologist as the ophthalmoscopic. The technique and the details of the 
 Iaryngoscopic findings do not belong here, but the disturbances first men- 
 tioned (aphonia, hoarseness) are nearly always the consequences of a lesion 
 of the n. laryngeus inf. (recurrens), which according to its severity, presents 
 very different Iaryngoscopic pictures. Of these we shall mention the three 
 most important. 
 
DISTURBANCES IN VOICE AND SPEECH 19 
 
 1. In total recurrens paralysis, the vocal cord concerned (or in bilateral 
 paralysis, both vocal cords) remains during phonation and inspiration in 
 the same immovable position between the ad- and abduction (cadaver 
 position) . 
 
 2. In so-called internus paralysis (paralysis of the thyreo-arytaenoidei 
 interni) the vocal cords are not altogether closed in phonation, but leave an 
 oval slit open; they remain lax, and consequently hoarseness results. 
 
 3. In so-called posticus paralysis (paralysis of the cricoarytenoids 
 postici) phonation is as a rule normal, but there usually appears inspiratory 
 dyspnoea, since the muscles mentioned are dilators of the glottis, and there- 
 fore upon their loss, the vocal cords are brought pathologically nearer to 
 each other, are drawn in. In unilateral paralysis there is only inspiratory 
 stridor; bilateral posticus paresis may, especially upon exertion and the 
 resulting forced breathing, lead to severe dyspnoea (Figs. 1 to 5). 
 
 All these conditions — there are in addition a number of rarer laryngeal 
 pareses, all of which cannot be mentioned here — are found in recurrens, 
 or in vagus affections, which localized peripherally more frequently than 
 centrally, may be evoked through tumors, traumatisms, aortic aneurysms, 
 etc. Hysteria may simulate each of these pictures, so that it may be difficult 
 to distinguish an hysterical from an organically conditioned (nervous) 
 aphonia. Organic recurrens paralyses are more frequently unilateral, 
 hysterical usually bilateral; but one must remember that also in chronic 
 catarrhs slight pareses of the type described may occur. 
 
 Of the disturbances in speech, the most important group, the aphonic 
 symptom complex belongs to a special chapter (cf . its discussion in a special 
 part of the volume) . We are concerned here only with the disturbances in 
 articulation ("speech utterance disturbance" " Sprachstoerung," Schuster) 
 which are usually designated as "anarthria" or "dysarthria." It is a ques- 
 tion here exclusively of innervation disturbances of the muscles needed in 
 speech (lips, tongue, palate, also larynx and respiration) or of the nerves 
 subserving them (facialis, hypoglossus, vago-accessorius, phrenicus) and 
 in many dysarthric disturbances one can recognize at once from the act of 
 speech which group of muscles is attacked when the patient is speaking. 
 When all the muscles of the tongue, palate and lips are affected, there results 
 only an unintelligible babbling, total anarthria, as we usually find it in 
 the final stage of bulbar paralysis. But this disease usually shows an affec- 
 tion of the separate muscles progressing gradually in groups, and these too 
 are not easily differentiated. In paralysis of the tongue, e and a, also 
 d, 1, r, s, t, are spoken indistinctly; paralysis of the lips makes the utterance 
 of, 00, o, b, f, p, v, difficult or impossible. Paralysis of the muscles of the 
 soft palate makes speech nasal (since part of the air expired passes through 
 the nose in phonation) , and separate letters (g, k, German ch) also become 
 indistinct. 
 
2o GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 Stuttering is dependent upon another disturbance. Here it is a matter, 
 partly of a tonic, partly of a clonic spasm in the muscles used in speech, and, 
 moreover, not only the lips and tongue, but also the muscles of respiration 
 are affected at the same time. Frequently it is not the spasmodic tension 
 of a certain muscular group that takes away from the patient the possibility 
 of getting beyond the initial letter (which most often causes the trouble) 
 but the synchronous tension of various muscles, working partly as antagonists. 
 In contrast to anarthria, stuttering must be looked upon as a functional 
 neurosis, and is scarcely ever dependent upon an organic disease. 
 
 By "scanning" we designate a speech disturbance in which the single 
 syllables follow one another slowly, rhythmically, at clearly defined inter- 
 vals. It is (with rare exceptions in hysteria) an organically conditioned 
 central innervation disturbance, which is characteristic of multiple sclerosis, 
 and finds its parallel in the spastic rigidity of the muscles of the extremities 
 which occurs in this disease. 
 
 The stumbling over syllables in patients suffering from progressive paraly- 
 sis, represents a mixture of anarthria on a motor and on a psychic basis. 
 In the utterance of long words — -favorite words for the test are "Massachu- 
 setts artillery," "truly rural" — the first syllables are spoken correctly, the 
 following ones stumblingly, or are mutilated or partly omitted. Obviously 
 there is concerned here in addition to the mere disturbance in articulation, a 
 forgetting of the separate syllables during speech, or failure to understand 
 the word. Frequently the paralytic does not notice his speech disturbance, 
 in contrast to other persons suffering from dysarthria, who are keenly con- 
 scious of their defect. 
 
 Besides the disease forms already mentioned, we find anarthria or 
 dysarthria, in peripheral nerve affections (hypoglossus), occasionally con- 
 genital, and commonly in all sorts of diseases of the cortex of the cerebrum 
 (tumors, apoplexies); in these latter, frequently with a genuine aphasia, 
 especially in recent apoplexies, which must not be overlooked when testing. 
 
 Mutism, the total inability to speak, is congenital, except for its appear- 
 ance in hysteria and will be discussed in that chapter. 
 
 5. Disturbances of the other Cerebral Nerves 
 
 belong here only in the minority of cases, since the motor nerves (facialis, 
 trigeminus III, accessorius, hypoglossus) will be mentioned when discussing 
 motility, the sensory trigeminus, when discussing the sensibility test, with 
 the symptoms of which the forms of disease, in the main, coincide. But we 
 recommend to the physician who is making a systematic examination always 
 to institute an investigation of all the cerebral nerves preceding that of the 
 peripheral nervous system. In addition, we shall mention here only the 
 disturbances affecting the soft palate, and the complicated mechanism 
 representing deglutition. 
 
DISTURBANCES IN SWALLOWING. MOTILITY 21 
 
 The soft palate (mainly innervated by the vago-accessorius group) par- 
 ticipates in speaking, swallowing (and in opening of the Eustachian tube). 
 We find corresponding symptoms of disturbance: 1. In phonation (one has 
 the patient pronounce ah) the diseased half of the palate is not raised, but 
 hangs down laxly, the soft palate and the uvula are in a position slanting 
 towards the sound side (the uvula even in normal persons is not always quite 
 straight). 2. Upon swallowing, food (especially liquid food) passes to the 
 nose because of - the insufficient closure of the pharynx. 3. Occasionally, 
 slight auditory disturbance. Paralyses of the palate are found most fre- 
 quently in polyneuritis (diphtheria), then in central affections (bulbar 
 paralysis) . 
 
 In deglutition the muscles of the lips, tongue, palate, pharynx and 
 oesophagus are involved. Consequently, different disturbances in swallowing 
 appear, according as to whether only one and which one of these groups is 
 affected. In weakness of the lips, liquid flows out of the mouth, in weakness 
 of the tongue, it cannot be transmitted to the pharynx, or the food remains 
 in the cavities of the cheeks. 
 
 Since all swallowing of liquids is effected by means of pressure of the 
 tongue against the hard palate, patients with paralysis of the tongue are 
 often unable to take any liquid nourishment at all; paralysis of the palate 
 causes, as has been said, part of the food to pass into the nose. Finally, a 
 paralysis of the muscles of the pharynx and oesophagus causes only a dis- 
 turbance in the swallowing of solid food, which, in such cases, is pushed 
 down only with great trouble, that is, sinks down of itself. Unilateral 
 deglutition paralysis gives rise to almost the same disturbances and produces 
 the feeling of " food going down the wrong way," which is caused by entrance 
 of part of the food into the larynx and its reflex reaction. 
 
 Disturbances in deglutition are found most frequently in toxic and infec- 
 tious polyneuritis and in central nervous diseases (bulbar paralysis, etc.). 
 
 Other symptoms of the glosso-pharyngeal-vagus group will be mentioned 
 in the special sections devoted to neuritis of the cerebral nerves. 
 
 We may remark here in anticipation that electric changes in the cerebral 
 nerves may be established with certainty only in the facialis, trigeminus III, 
 accessorius, hypoglossus, and the soft palate. 
 
 C. Motility 
 
 The condition and activity of the musculature represents the first of the 
 three main groups in the examination of the nervous system (especially of 
 the peripheral). 
 
 Inspection of the musculature can distinguish, above all, three things — 
 hypertrophy, atrophy, and, to a certain degree, the tonus of the muscles. To 
 ascertain the presence of hypertrophy or atrophy, especially the pathological 
 
22 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 degrees of both, one naturally needs an exact knowledge of the normal 
 forms of the body, that is, of the normal (or nearly normal) forms of the mus- 
 cles. Now there is scarcely any other tissue of the body which, through 
 exercise or lack of exercise or through weakening diseases, etc., undergoes 
 greater changes than the musculature. The great muscles of the pugilists 
 may bear the most striking external similarity to the pseudo-hypertrophic 
 muscles of the sufferer from dystrophy, and the patient with immobilized 
 joints may, in respect to the appearance of his muscles very easily be con- 
 fused at first sight with a case of severe polyneuritis. A less practised eye 
 than is concerned with the above changes is, however, somewhat more 
 useful, if it be a question of recognition of differences in the musculature 
 between the right and the left side, between peripheral and central parts. 
 But here, too, the eye alone must not decide, though doubtless it best recog- 
 nizes slight differences. One must employ an exact measurement likewise, 
 which, in so far as musculature alone is concerned, usually signifies only 
 measuring the circumferences of the extremities. Naturally, for measuring, 
 exact symmetrical points must be chosen (measuring the distance from 
 fixed points upon the bones in centimeters), and it must be considered 
 furthermore that in right handed persons the right arm and the right leg 
 have a circumference of about one to two cm. more than the corresponding 
 extremities upon the left side. The trained eye can naturally detect easily 
 symmetrical atrophies, especially those in the extremities (hands), but to 
 establish these changes definitely, one needs also an exact motility and 
 electricity test. For tonus of the muscles cf. below. 
 
 Palpation of the muscles except for the rare determination of nodular 
 thickenings (tumors, pseudo-hypertrophy) or a general softening of the 
 muscular substance, affords no special conclusions: 
 
 All the more important is the motility test. In order to control this, 
 one naturally needs an exact knowledge of the function of the individual 
 muscles, which shall be here recapitulated briefly, in so far as it is concerned 
 with testing the active movements. (I follow in the main StrilmpelVs 
 division.) 
 
 i. Mimetic musculature (nervus facialis). 
 
 M. frontalis. 
 
 Function: Wrinkles the forehead into transverse folds. 
 
 Test: To look directly upwards. To draw up the eyebrows. 
 
 Loss in paralysis: After the order to draw up the eyebrows, absence of 
 wrinkling on the diseased side. 
 
 M. occipitalis: Involved in the wrinkling of the forehead and the scalp. 
 
 Condition in loss and test the same as above. 
 
 M. corrugator supercilii. 
 
 Function: Wrinkles the forehead perpendicularly. 
 
 Test: Wrinkle your forehead! 
 
FUNCTIONS OF SINGLE MUSCLES 23 
 
 Loss in paralysis: In unilateral paralysis, an often indistinct absence 
 of wrinkles during the test. 
 
 M. orbicularis oculi. 
 
 Function: Eye closure. 
 
 Test: Close your eyes! 
 
 Loss in paralysis: The palpebral fissure can not be completely closed. 
 Lagophthalmos. 
 
 M . compressor nasi, levator alee nasi, zy gomaticus , risorius, levator labii 
 sup. 
 
 Function: Elevating the alas nasi and the corner of the mouth. 
 
 Test: Turn up your nose! 
 
 Functional loss in paralysis: Corresponding; above all, loss of the naso- 
 labial fold. 
 
 M. orbicularis oris. 
 
 Function: Pursing the lips, whistling. 
 
 Test: Corresponding command. 
 
 Functional loss in paralysis: Disability or inability to move the lips. 
 
 Atrophy of the mimetic muscles leads to sinking in of the parts involved. 
 
 2. Chewing (masticatory) musculature. 
 
 Mm. masseter and temporalis (N. Tringeminus III) . 
 Function: Movements of chewing. 
 Test: Close your teeth tightly ! 
 
 Functional loss in paralysis: Noticeable gap in the place of both muscles 
 on the diseased side. Especially distinct in simultaneous atrophy. 
 
 3. Movement of the soft palate; deglutition: cf. above, under "other 
 cerebral nerves." 
 
 4. Muscles of the tongue (N. hypoglossus) . 
 Function: All independent movements of the tongue. 
 
 Test: Stick out your tongue, move it to the right, to the left, roll it up- 
 wards, downwards! 
 
 Functional loss in paralysis: In unilateral paralysis, the tip of the out- 
 stretched tongue deviates towards the paralyzed side (as a result of the 
 peculiar radial distribution of the m. genioglossus) . The movements of the 
 tongue are all more or less hindered, but an isolated test of the other muscles 
 of the tongue (m. lingualis, m. transversus linguae) is of no great importance. 
 In bilateral paralysis (bulbar paralysis, etc.), the tongue lies motionless upon 
 the floor of the buccal cavity. Atrophy of the musculature of the tongue 
 leads to the quite characteristic picture of the furrowed tongue, which shows 
 uni- or bilateral transverse folds, irregular elevations and depressions and 
 feels abnormally soft. 
 
 5. M. sterno-cleido-mastoideus (N. accessorius) . 
 
 Function: Rotates and partially moves the head forward. 
 
 Test: Press the chin hard upon the fist of the examiner. 
 
24 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 Functional loss in paralysis: The head is incompletely rotated, and bent 
 crookedly forwards (the chin turns towards the paralyzed side) . In the test 
 described the characteristic cord of the muscle appears only on the sound 
 side. 
 
 6. Muscles of the Back. 
 
 Mm. splenii, biventer, recti capitis postici (cervical nerves 1-4) . 
 Function: Backward movement of the head and the cervical vertebras. 
 Test: Lay the head backwards upon the supporting hand of the examiner. 
 Functional loss in paralysis: Oblique and incomplete performance of 
 this movement. 
 
 7. Mm. sacrolumbalis, longissimus dorsi, spinalis dorsi (spinal 
 nerves) . 
 
 Function: Erects the spinal column. 
 
 Test: Rise from a stooping position against the resisting hand of the 
 examiner, without help from the hands. (But cf. under 34.) 
 
 Functional loss in paralysis: Hindrance or inability to rise in the test 
 described. In unilateral paralysis dorsolumbar scoliosis (often with lordo- 
 sis) with convexity towards the paralyzed side. In bilateral paralysis: 
 severe lumbar lordosis in walking (gait of dystrophics); in sitting often 
 (not always) kyphosis. 
 
 8. Abdominal muscles (recti, obliqui, transversus abd. ; dorsal 
 nerves from the eighth on) . 
 
 Function: Abdominal pressure; raising the body from a supine position. 
 Flexing the spinal column forwards. 
 
 Test: Exert pressure as if evacuating the bowels. Rise from a supine 
 position without help from the hands. 
 
 Functional loss in paralysis: Impossibility of rising as described. Diffi- 
 culty in evacuation of the feces and urine. Lumbar lordosis,, pot-belly. 
 In unilateral paralysis, occasionally the linea alba and navel are drawn 
 towards the sound side. 
 
 9. M. quadratus lumborum (plexus cruralis). 
 Function: Lateral movement of the spinal column. 
 Test: Corresponding to the function. 
 
 Functional loss in paralysis: Corresponding, generally unimportant. 
 
 10. Diaphragm (N. phrenicus from cervic. 4). 
 Function: Lengthening of the thoracic cavity in inspiration. 
 Test: Breathe deeply. 
 
 Functional loss in paralysis: Absence of the epigastric protuberance, 
 retraction of the intestines and the diaphragm phenomenon (visible descend- 
 ing shadow upon lateral illumination) in inspiration; dyspnoea. 
 
 11. Muscles of the shoulder girdle and arm. 
 M. cucullaris or trapezius (N. accessorius) . 
 
 Function: Elevates the shoulder blade and brings it nearer to the middle'line. 
 
FUNCTIONS OF SINGLE MUSCLES 25 
 
 Test: Raise your shoulder blades (" shrug your shoulders ") ! 
 
 Functional loss in paralysis: Since frequently only one of the three por- 
 tions of the muscle is paralyzed, the picture may vary. The characteristic 
 of cucullaris paralysis, restriction in raising the shoulders, belongs actually 
 to the middle portions alone, whereas the upper (clavicular) only when the 
 shoulder is fixated, draws the head somewhat towards the back, and the 
 lowest causes the shoulder to move towards the spinal column. The typical 
 picture of total paralysis of the muscles is : sinking of the acromion, lowering 
 and forward position of the shoulder (and the arm), more or less pronounced 
 horizontal position of the collar bone, and a moving away of the scapula 
 from the spinal column. The atrophy of the muscle, which is usually present, 
 leads to a flattening of the line between the shoulder and the neck and to a 
 clearer prominence of the contours of the scapula (above all of the spine of 
 that bone). This position of the shoulder blade is designated as "angel- 
 winged." 
 
 12. M. levator anguli scapulae ( 1 to 3 cervicalis). 
 Function: Elevates the inner superior angle of the scapula. 
 
 Test: Raise your shoulders. (Usually, if the cucullaris is retained, 
 not easily recognizable) ; when the cucullaris is paralyzed, partly replaces 
 it functionally. 
 
 Functional loss in paralysis: Slight (in so far as it is isolated) . In com- 
 bination with cucullaris paralysis; inability to raise the scapula. 
 
 13. Mm. rhomboidei (4 to 5 cervicalis) . 
 
 Function: To bring the scapula nearer to the spinal column (especially 
 the inferior angle). 
 
 Test: Draw your shoulders together in the back! 
 
 Functional loss in paralysis: The inner border of the shoulder blade 
 stands out from the thorax (visible almost only when simultaneous cucullaris 
 paralysis exists). 
 
 14. M. serratus anticus major (X. thoracicus longus, from cervical 5). 
 Function: Rotates the scapula about the sagittal axis, and fixates it 
 
 when the upper arm is raised vertically; serves for fixation of the scapula 
 to the thorax, even in a state of rest. 
 
 Test: Raise your arm above the horizontal position. Press your arm 
 forwards against some resistance (pushing). 
 
 Functional loss in paralysis: In rest, frequently, a deviated position of the 
 inner margin of the scapula, which, below, draws nearer the spinal column, 
 at the same time rising a little from the thorax. The arm can no longer be 
 raised above the horizontal line because of the insufficient fixation of the 
 scapula. In pushing the arm forwards, the inner margin of the scapula 
 moves forward like a wing from the thorax: "winged shoulder." The 
 atrophy that is usually present at the same time allows the winged position 
 
26 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 to become more clearly visible, and brings about a recognition of the usually 
 easily seen serratus digitations in the lateral wall of the thorax. 
 
 15. M. deltoideus (N. axillaris). 
 
 Function: Elevates the arm to somewhat above the horizontal line. 
 
 Test: Raise your arm to a horizontal position (eventually against 
 resistance). 
 
 Functional loss in paralysis: The arm can not be raised. The usually 
 simultaneous atrophy is characterized by loss of the shoulder curve and by 
 prominence of acromion and caput humeri. 
 
 16. M. pectoralis major and minor (N. thoracici ant. from cervical 5 
 and 6). 
 
 Function: Draws the arm up to the thorax. 
 
 Test: Press your outstretched arm against some resistance (hands of 
 the examiner). 
 
 Functional loss in paralysis: Usually only weakness in the before men- 
 tioned adduction movement, since deltoideus and teres major afford partial 
 compensation. In severe atrophy, the upper ribs may stand out prominently. 
 
 17. M. latissimus dorsi (N. subscapularis from cervicalis 5 and 6). 
 Function: Draws the upper arm backward and downward. 
 
 Test: With horizontally raised upper arm, press downward and back- 
 ward upon the supporting hand of the examiner. 
 
 Functional loss in paralysis: Weakness in the test described. 
 
 18. Mm. supraspinatus, infraspinatus, teres minor (N. supra- 
 scapular) . 
 
 Function: Rotates the arm outward. 
 
 Test: Rotate the outstretched arm outward (against resistance). 
 
 Functional loss in paralysis: Weakness in the movement described. 
 Sometimes disturbance in writing. The frequently simultaneous atrophy 
 may be recognized by the flattening of the posterior arch of the shoulder 
 blade. 
 
 19. M. subscapularis, teres major (N. subscapularis). 
 Function: Rotates the arm inwards. 
 
 Test: Rotate the extended arm inwards (against resistance). 
 Functional loss in paralysis: Weakness of the movement described. 
 
 20. Mm. biceps, brachialis internus (N. musculocutaneous). 
 Function: Flexes the forearm towards the upper arm without any rota- 
 tion of the lower arm (slight supination). 
 
 Test: Flex your forearm when in a supine position (against resistance). 
 
 Functional loss in paralysis: Impossibility of flexing in this position; 
 there appears at once pronation of the forearm. Atrophy of these muscles 
 is easily recognized by the loss of the biceps curvature; the circumference 
 of the upper arm is decreased perceptibly. 
 
 21. M. supinator longus (N. fadialis). 
 
FUNCTIONS OF SINGLE MUSCLES 27 
 
 Function: Flexes the forearm towards the upper arm in half pronated 
 position (the middle position between pronation and supination). 
 
 Test: Flex your forearm against resistance with half pronated hand 
 (palms towards the inside). 
 
 Functional loss in paralysis: In attempts at movement the hand is at 
 once supinated. Atrophy can be easily recognized by a conspicuous gap 
 in the musculature on the radial side of the bend of the elbow. 
 
 22. M. triceps (N. radialis). 
 Function: Extends the forearm. 
 
 Test: Extend your flexed arm against resistance. 
 
 Functional loss in paralysis: Impossibility of performing this movement 
 against resistance. 
 
 23. Mm. extensores carpi (rad. and uln., N. radialis). 
 
 Function: Extends (that is, overextends) , the hand towards the forearm. 
 Test: Extend your hand against resistance (at the metacarpus). 
 Functional loss in paralysis: Impossibility of performing the movement 
 described; the hand hangs down limply from the extended, pronated, forearm. 
 
 24. Mm. ext. digit, comm., indicator, ext. digiti v (N. radialis). 
 
 Function: Extends the basal phalanges of the second to fifth fingers. 
 
 Test: Extend your fingers against resistance (at the basal phalanges). 
 
 Functional loss in paralysis: Impossibility of performing the move- 
 ment described. The atrophy of all the muscles mentioned under 23 
 and 24 causes usually a moderate degree of wasting in the dorsal aspect of 
 the forearm. 
 
 25. Mm. interossei ext., and int., Mm. lumbricales (N. ulnaris, also 
 medianus) . 
 
 Function: These groups of muscles abduct and adduct the fingers (only 
 the interossei) at the same time, flex the basal phalanges, and extend the 
 end phalanges of the fingers. 
 
 Test: Spread and press together against resistance the fingers that have 
 been extended (it is best to do this against the fingers of the examiner which 
 are inserted as in "folding the hands"). Flex the fingers against resistance 
 (at the basal phalanges) and extend them against resistance (at the end 
 phalanges). 
 
 Functional loss in paralysis: Impossibility of performing the movements 
 described; above all, inability to hold on to thin objects (needle, coins) in 
 the fingers. In a condition of rest, the 4th and 5th fingers deviate towards 
 the ulnar side. There is no strength in pressure of the hand. Atrophy is 
 made very typical by the sinking in of the spatia interossea and by the 
 hyperextension of the basal phalanges resulting from the preponderance 
 of the antagonists (the long extensors and flexors) with simultaneous flexion 
 of the end phalanges. Wasting of the palm. The type of hand known as 
 "claw hand" (paralysis of the ulnaris). 
 
28 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 26. M. palmaris, Mm. flexores carpi (rad. and uln., N. medianus and 
 n. ulnaris). 
 
 Function: Flexes the hand towards the forearm. 
 Test: Flex your hand against resistance (at the metacarpus). 
 Functional loss in paralysis: Usually slight; weakness of the movement 
 mentioned. 
 
 27. Mm. flexores digit, long. subl. and profund (N. medianus). 
 Function: Flex the middle (sublimis) and end phalanges (profundus). 
 Test: Pressure of the hand with co-operation of the lumbricales and 
 
 interossei. Eventually, isolated test of the profundus: pressing the tips 
 of the fingers against tips of the flexed fingers of the inverted hand of the 
 examiner, with attempts to flex the fingers (interlocking). 
 
 Functional loss in paralysis: Weakness of hand pressure. One must 
 observe that simultaneous weakness of the extensores carpi also simulates 
 weak hand pressure, since when the hand hangs down, the flexors mentioned 
 are unable to functionate. Therefore with simultaneous radialis paralysis, 
 the test can be made only with passively fixed wrist. Atrophy of 26 and 27 
 combined, cause wasting of the forearm (usually of moderate degree). 
 
 28. M. flexor brevis and abductor digiti minimi (N. ulnaris). 
 Function: Flexes the basal phalanx of the little finger and abducts the 
 
 little finger. 
 
 Test: Flex your little finger against resistance (hooked in the finger of 
 the examiner). 
 
 Functional loss in paralysis: Weakness of the movement mentioned. 
 Atrophy of these muscles mainly causes the characteristic flattening of the 
 ball of the little finger in paralysis of the ulnaris. 
 
 29. Mm. extensor pollicis brevis and longus, M. abductor pollicis 
 (N. radialis). 
 
 Function: Extension, that is, abduction of the thumb and the metacarpus. 
 Test: Extend and abduct the flexed thumb against resistance. 
 Functional loss in paralysis: Weakness of the movement described. The 
 thumb falls into the palm of the hand. 
 
 30. M. adductor pollicis (N. ulnaris). 
 
 Function: Adducts the thumb (metacarpus) towards the forefinger. 
 Test: Press your thumb against resistance towards the fixed forefinger. 
 Functional loss in paralysis: Weakness of the movement mentioned. 
 
 31. M. opponens, abductor brevis and flexor brevis pollicis (N. 
 medianus). 
 
 Function: Flexion and opposition of the metacarpus 1 and the thumb 
 (1 phalanx). 
 
 Test: Press your hand. 
 
 Functional loss in paralysis: Weakness in hand pressure. Often dis- 
 turbances in writing. The atrophy, which is usually present at the same time, 
 
FUNCTIONS OF SINGLE MUSCLES 29 
 
 flattens the ball of the thumb in characteristic fashion, and gradually -brings 
 the thumb into the direction of the other fingers, in the plane of which it 
 finally lies: "ape hand" of the medianus paralysis. 
 
 32. Muscles of the leg. 
 
 M. ileopsoas (N. cruralis), M. tensor fascia lata (1 and 2 lumbalis). 
 Function: Elevates the leg at the hip joint. 
 
 Test: Raise your extended leg from a supine position (against resistance). 
 
 Functional loss in paralysis: Disturbance in walking; walking is usually 
 
 quite impossible, as is also raising the extended leg from a supine position. 
 
 33. M. sartorius (N. cruralis). 
 
 Function: Test like that under ^2. Not infrequently, in paralysis of 
 the flexors of the thigh and of the extensors of the leg proper the sartorius 
 alone is spared. 
 
 34. Mm. glutaei (sacral nerves). 
 
 Function: Extension and slight abduction of the leg at the hip joint. 
 
 Test: In a supine position, press down against resistance the raised 
 thigh. When standing, rise from a stooping position against resistance. 
 Press the thigh outwards against resistance. 
 
 Functional loss in paralysis: Difficulty in rising from a sitting posture, 
 in mounting stairs, in rising from a bent position (in common with 7), wad- 
 dling gait. 
 
 35. Mm. adductor brevis, longus, magnus, pectineus, gracilis (N. 
 obturatorius) . 
 
 Function: Adduction of the thigh at the hip joint. 
 Test: Press the thighs together against resistance. 
 
 Functional loss in paralysis: Loss of the movement mentioned. Inse- 
 cure seat when riding horseback. 
 
 36. M. extensor cruris quadriceps (N. cruralis). 
 Function: Extends the leg proper at the knee joint. 
 
 Test: In a supine position, extend the leg against resistance, while the 
 arm of the examiner placed below the knee fixates the thigh in a slightly 
 flexed position. 
 
 Functional loss in paralysis: Impossibility of extending the leg proper in 
 the manner described. Severe disturbance in walking, though walking is 
 possible with the leg in extension, as long as the patient seemingly supports 
 himself upon the surface of the knee joint and does not innervate the flexors 
 of the leg proper. Mounting stairs is, as a rule, impossible. 
 
 37. M. biceps, semitendinosus, semimembranosus (N. ischiadicus) . 
 Function: Flexion of the leg proper and extension of the hip joint. 
 Test: In a supine position, draw up the leg (against resistance) towards 
 
 the thigh. 
 
 Functional loss in paralysis: Inability to perform the movement men- 
 tioned, moderate disturbance in gait, inability to jump and run. 
 
30 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 38. M. tibialis anticus (N. peroneus). 
 
 Function: Elevates the inner margin of the foot and extends the foot 
 (subjects it to dorsal flexion). 
 
 Test: Scarcely possible as an isolated movement. 
 
 Functional loss in paralysis: Inconsiderable in isolated paralysis. The 
 atrophy effects a characteristic sinking in of the muscular layer directly 
 next to the margin of the tibia. Frequently the tendon of the overexerted 
 ext. hallucis stands out prominently; the large toe is flexed dorsally. 
 
 39. M. peroneus longus and brevis (N. peroneus). 
 
 Function: Elevates the outer margin of the foot and subjects the foot to 
 slight flexion (plantar flexion). 
 
 Test: As an isolated movement only possible electrically. 
 
 Functional loss in paralysis: In attempting extension, adduction of the 
 foot appears; the great toe drags slightly upon the ground in walking; flat- 
 foot develops. 
 
 40. Mm. extensor digit, comm. longus, ext. hallucis longus (N. 
 peroneus). 
 
 Function: Elevates the toes and thereby the tip of the foot. 
 
 Test: Draw up the toes against resistance. 
 
 Functional loss in paralysis: The toes hang down limply. The par- 
 ticipation of these muscles effects, mainly, however, in combination with 38 
 and 39, the picture of the peroneus paralysis: the tip of the foot hangs limply 
 down, usually its inner margin is the lowest; as a result, it always drags 
 upon the ground in walking. In order to avoid this the patient raises his 
 thigh excessively when walking, the characteristic "steppage gait" results. 
 Through secondary contracture of the antagonists (calf) there frequently 
 develops pes equinus, or pes equino-varus. Atrophy of the anterior aspect 
 of the leg proper. 
 
 41. M. triceps surae (M. gastrocnemius, plantaris, soleus — N. tibialis). 
 Function: Plantar flexion of the foot. 
 
 Test: Press the foot downwards against the hand of the examiner; 
 stand upon the tips of the toes. 
 
 Functional loss in paralysis: Loss of flexion of the foot. Inability to 
 stand upon the tips of the toes or to dance. There frequently develops a pes 
 calcaneus. Atrophy of this muscular group causes the most severe atrophy 
 possible in the leg proper. 
 
 42. M. flexor dig. comm. longus, brevis, flexor hallucis longus 
 and brevis (N. tibialis). 
 
 Function: Plantar flexion of the toes. 
 
 Test: Flex the toes downwards against resistance. 
 
 Functional loss in paralysis: Inconsiderable. 
 
 43. Mm. interossei (N. tibialis). 
 Function: Flexion of the basal phalanges. 
 
TYPES OF PARALYSIS 31 
 
 Test: Scarcely possible in isolation. 
 
 Functional loss in paralysis: Inconsiderable. But through the atrophy 
 of these muscles and secondary contractures of the antagonists, atrophy 
 of the planta pedis and a "claw-foot" may develop. 
 
 Functional testing conducted in this way will always give a complete 
 picture of the power of the individual muscles. For separate groups of 
 muscles, functional power may be measured directly by means of the so- 
 called dynamometer (Charriere, Duchenne, Sternberg), which, however, has 
 been extensively used only for pressure of the hands. Here it gives good 
 comparative values in respect to the strength of both hands and affords a 
 comparison of the strength in various stages of the same disease. 
 
 The degree of the paralyses is important for their recognition. While 
 total paralyses even of individual muscles alone will scarcely escape the notice 
 of the examiner, mere conditions of weakness, pareses, are often very difficult 
 to recognize; they are most easily recognized in unilateral paralyses through 
 comparison with the same muscle on the other side. Naturally disturbances 
 of the sensorium cause special difficulties in the recognition of palsies; here 
 sometimes the tonus (cf. below) is of decisive importance; in recent apoplex- 
 ies, the raised arm, even in stuporous patients, falls down far more laxly on 
 the paralyzed side, as if dead, in contrast to that on the sound side. In small 
 children, the determination even of complete paralyses is often difficult. 
 Where it is not a matter of the more easily recognized disturbances in walking, 
 the reaction of the child to pain stimulations often leads to the goal; one 
 pinches or pricks the skin approximately in the region of the antagonists of 
 those groups of muscles that one wishes to test; the degree of reaction to 
 escape the irritation will usually determine whether or not weakness is 
 present. 
 
 As to the type of paralysis, naturally nothing has been ascertained by the 
 mere determination of its presence. We must emphasize here, that almost 
 every muscular paralysis may be of organic or "functional" (psychogenous, 
 usually hysterical) origin; but even among the organic paralyses, there is 
 scarcely any point in the entire nerve-muscular apparatus which may not 
 give rise to muscular paralyses: disease of the muscle itself (myositis, 
 possibly dystrophy and myotonia) , of the peripheral nerve (neuritides) , of the 
 spinal cord (diseases of the columns of the spinal cord, poliomyelitis, etc.), 
 of the medulla, oblongata and brain (bulbar paralyses, apoplexies, etc.). 
 The paralysis may furthermore, as we shall see below, he flaccid or spastic 
 (with diminution or increase of the tonus), may be accompanied by atrophy 
 or hypertrophy, etc. As to what form is concerned in the individual 
 case, frequently electrical testing, sometimes only observation of the 
 entire disease picture may decide. 
 
 In our discussion of the individual muscles, we have always noted when 
 the atrophy of a muscle produces characteristic changes inform. Now this 
 
32 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 happens more frequently in groups of muscles than in individual muscles; 
 their decrease in volume produces characteristic pictures, which, for the experi- 
 enced physician, make possible diagnosis at first sight. The pictures are 
 often typical of certain well-determined diseases. We mentioned the "claw- 
 hand" of ulnar paralysis, the "ape-hand" of median paralysis (of high degree), 
 dorsal flexion of the large toe in paralysis of the tibialis anticus, etc. These 
 pictures are found most frequently, especially in isolated forms, in peripheral 
 neuritis, somewhat less frequently (and scarcely ever alone) in poliomyelitis, 
 syringomyelia, bulbar paralysis. For progressive neurotic muscular atrophy, 
 the peripheral beginning of the atrophy is characteristic; the extremities 
 acquire that peculiar attenuation towards the periphery, which is known as 
 "bird-legs." On the other hand, progressive spinal muscular atrophy also 
 attacks the periphery first, but the atrophy here is of much higher degree. 
 In syringomyelia we see that, as a rule, only the upper extremities show mus- 
 cular atrophy; for poliomyelitis the apparently total participation of all pos- 
 sible muscular groups here and there, all over the entire body is characteristic. 
 The most various and most easily remembered pictures are presented prob- 
 ably by dystrophy; the thin upper arms with the strong lower arm, the winged 
 shoulders, lumbar lordosis, if the face is affected the tapir-lip, lagophthalmos. 
 The number of such characteristic pictures may be easily increased, especially 
 if one counts in all the changes in form dependent upon contractures (cf. 
 below) with or without synchronous atrophies. For the sake of simplicity, 
 we may here mention at once some other pictures of disturbances in gait, 
 not directly connected with muscular paralysis and atrophy. We have just 
 mentioned the "steppage" gait, the waddling gait, posture and gait in 
 dystrophy. These kinds of gait belong to the simple paretic variety. All 
 gait disturbances accompanied by lesion of the pyramidal tracts are to be 
 designated as spastic, or spastic paretic. Common to them are spasms or 
 tensions (cf. under hypertonia) ; in walking the legs are pushed forwards as if 
 they were made of wood, flexed but slightly at the hip and not at all at the 
 knee, the tips of the feet graze the ground, the soles of the shoes are always 
 worn through at a definite place. Such is the gait in spastic spinal paralysis, 
 lateral sclerosis, myelitis, mostly too in syringomyelia and multiple sclerosis. 
 The ataxic, "swinging" or long-striding gait of the tabetics, the "tottering" . 
 gait of cerebellar ataxia, are mentioned below under ataxia. Persons suffer- 
 ing from paralysis agitans walk stiffly, mostly with bent back and bowed 
 head, taking small steps, also "stick" to the ground, and still the gait is not 
 really spastic; nay, rather upon occasion very quick tripping steps are made 
 forwards and backwards, irresistibly, especially when with a light push, one 
 sends the patient forward or backward or tells him to run. Then he cannot 
 arrest his movements (symptom of pro- and retropulsion), which never occurs 
 in spastics. Total inability to walk, known as "abasia" ("astasia"), which is 
 not rare in hysteria, will be mentioned in the special part of the book. 
 
DISORDERS OF WRITING. MUSCULAR TONUS 33 
 
 The manifold picture of writing disturbances of a motor nature furnish a 
 parallel to a certain extent to the disturbances in gait. Mere paretic disturb- 
 ances, such as appear in isolated median or ulnar paralysis are but little 
 characteristic; the patients write slowly, with trouble, but the writing is not 
 characteristically changed, and occasionally retains wholly its previous 
 character. Clumsiness naturally increases in complicated pareses (spinal 
 muscular atrophy, multiple neuritis) . There may appear total motor inabil- 
 ity to write (motor agraphia). In spastic conditions the writing as a rule 
 becomes smaller, labored, the separate letters run into one another, and the 
 words often are illegible. Ataxic script (tabes superior) furnishes the type of 
 deviating movements on paper; the letters are of varying length, single strokes 
 pass across the letters already written, the lines are crooked and vary in 
 level. Very similar is the script of most sufferers from writer's cramp. 
 In multiple sclerosis the letters do not run into one another, but the script is 
 shaky, and there are very regularly found deviations corresponding to the 
 tremor in the stroke, the general direction of which is correctly maintained. 
 Of the genuine forms of tremor, paralysis agitans has the most typical change 
 in script, the so-called flower script : the easily legible letters in all their parts 
 show, instead of straight lines, small zig-zag lines, consisting of deviations of 
 the length of a millimeter. For the writing of sufferers from progressive 
 paralysis, etc., cf. the special chapters concerned. 
 
 Pathological hypertrophies are much rarer; only acromegaly and pseudo- 
 hypertrophy in dystrophy are really concerned here. Both likewise give 
 typical pictures. 
 
 A symptom of great importance or rather the nucleus of several large 
 groups of symptoms is muscular tonus. By this one designates the con- 
 dition of a certain permanent irritability, so to speak, in the sound 
 muscle (also in condition of rest), which we may interpret as a reflex pro- 
 cess localized in the ganglion cells of the anterior horn of the spinal cord. 
 In this process, the anterior horns represent to a certain extent an electrical 
 power station (Schuster), which is influenced by the higher centers of the 
 cerebrum as well as by the periphery. Peripheral sensory stimulations may 
 increase temporarily the tonus of the muscles (precisely by way of this power 
 station) , as well as may the impulse of the will emanating from the cerebrum, 
 evoking the voluntary movement; on the other hand, there doubtless exist also 
 between the cerebrum and anterior horns inhibiting influences, since in 
 interruption of this conductivity the tonus may increase. 
 
 As to the tonus of the musculature, we can get some data from simply 
 testing the musculature, partly also from mere observation of the patient. 
 If his limbs lie flaccid, touching throughout their entire extent whatever they 
 are resting upon, possibly exhibiting also overextended joints, there is pres- 
 ent in general, a decrease in the tonus of the muscles; if they lie rigid in 
 more or less forced positions, and no joint fixations can be determined, it is 
 3 
 
34 
 
 GEXERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 usually a matter of increase in tonus. The former condition is known as 
 hypotonia, the latter as hypertonia, of the musculature. Still more obvious 
 is the difference in testing the voluntary, active, and especially in testing the 
 passive movements. One instructs the patient to relax his limbs as much as 
 possible and then places the different parts of the extremities now with 
 quicker, now with slower, now with brusque, now with gentle, passive 
 motions into all physiologically possible positions. Nearly always one feels 
 resistance at first, which must be interpreted in part as reflex increase in the 
 
 normal tonus, and in part is 
 actively, though uncon- 
 sciously evoked by the pa- 
 tient. But, as a rule, this 
 resistance soon ceases in the 
 healthy individual. Xow 
 sometimes, precisely in cases 
 of hypertonia, this decrease 
 does not take place; nay 
 rather, at every attempt at a 
 brusque motion, the antag- 
 onists, that is, those muscles 
 of the patient, the activity 
 of which is to be suppressed 
 for the execution of the in- 
 tended passive motion, easily 
 become very tense and afford 
 an almost insuperable resist- 
 ance to it. On the other 
 hand, the resistance may fre- 
 quently be overcome by 
 slower, more careful motions, 
 but now frequently a second 
 pathological condition ap- 
 pears: an involuntary spas- 
 modic working of the antagonists (in the sense of the voluntary motion), which 
 now suddenly, apparently spontaneously, sluggishly indeed, but extensively, 
 sets in motion the extremity which before lay in immobile rigidity. These 
 symptoms of hypertonia belong to the large group of spastic conditions, to 
 which we have to reckon also the increase of the tendon reflexes, of which we 
 shall speak in our discussion of reflexes. In spite of the often, as has been 
 said, almost invincible, tension of the musculature in most spastic conditions, 
 its active strength is loitered; the so-called spastic paresis occurs. As a conse- 
 quence of lasting spastic paresis, we find permanent tonic conditions of the 
 musculature, contractures, nearly always accompanied by stiffening in the 
 
 Fig. 6. — Spastic paralysis (Little's disease) . 
 
HYPOTONIA 35 
 
 regional joints. These contractures of the tense, active (though occasionally 
 also actively paretic) musculature are theoretically (this is more difficult in 
 practice) to be distinguished from paralytic contracture, in which it is a 
 question of mere preponderance of the strength of the antagonists as op- 
 posed to the paralyzed agonists that have lost their functional ability (Fig. 6). 
 
 Decrease of muscular tonus, hypotonia, is found especially where 
 the stimulations from the periphery to the "power station" (cf. above) of the 
 anterior horn are more or less completely lost, that is in lesion of the sensory 
 tracts above all. Here passive motions may be performed with abnormal 
 ease; the limbs as a result of the relaxation of the muscles (usually also of the 
 articular ligaments) may be brought into the most exaggerated, distorted posi- 
 tions. The knee joints are overextended in walking (genu curvatum), etc. 
 We see the most severe forms of hypotonia in tabes dorsalis. At the same 
 time, the motor strength may be altogether normal. The tendon reflexes are 
 often simultaneously (but by no means always) weakened or absent. Here 
 belongs the remarkable affection, first described by Oppenheim, later also by 
 Bernhardt and others under the name of myatonia: in early infancy, there 
 appears an abnormal flaccidity of the entire musculature, a high degree of 
 hypotonia, while at the same time, direct and indirect faradic excitability is 
 absent. The condition probably represents a retarded development of the 
 central nervous system and can be cured. 
 
 If we return again to the test for active motility, we see frequently in 
 nervous patients a disturbance which is not evinced in a decrease of strength 
 or volume, but in the incorrectness of the voluntary movement: the move- 
 ment becomes inco-ordinated; we have a disturbance of co-ordination before us. 
 
 The concept of the co-ordination disturbance is to be defined as follows : 
 In every movement of our body (with very few exceptions) several muscles, 
 not one alone, are synchronously set in action. The performance of a 
 direct movement is, therefore, an extraordinarily delicate co-operation of these 
 muscles, known as synergists; if only one muscle misses the cue, so to say 
 through too slight or excessive contraction, the entire movement is disturbed. 
 Still more complicated is the process, if (with the majority of authors) one 
 believes that the antagonists co-operate in every voluntary movement of the 
 agonists, whether it be through a particularly delicate gradation of their 
 relaxation or in some other way. If now for some reason the function of 
 one muscle is lost, the others naturally preponderate; the part of the body 
 concerned is then — possibly only for the duration of a fractional part of the 
 entire movement— moved in a direction other than the one desired, until 
 a new impulse sent to a muscle, serving as a corrective, pulls it back again 
 into the old direction. Thus there arises a zig-zag movement, a deviation 
 or throwing about of the extremity concerned; we describe this movement 
 as inco-ordinated or ataxic. This disturbance is more or less independent 
 of the will of the patient, but may be 'somewhat set aside through practice, 
 
36 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 and often occurs but slightly in a patient who controls with his eye all his 
 movements, and at every deviation "at once" calls into play corrective 
 muscles, but always increases, if one tells the patient to close his eyes, and 
 thereby removes the "eye-control." 
 
 The test of ataxia is therefore made as follows in the individual case. 
 First the patient in a position of supination is asked to make complicated 
 movements with his hands: put his forefinger to his nose; thread a needle; 
 perform the so-called "finger-tip" experiment (the arms which have been 
 drawn back far laterally, are brought together slowly with extended fore- 
 fingers until they touch). When doing these acts there appear very distinct 
 deviating movements, which produce quite irregular varying twitching 
 excursions, in various planes about the axis of the movement desired (in 
 contrast to the regulated movements of most kinds of tremor), but the 
 patient, with the assistance of the controlling eye, is usually still able to 
 reach the point aimed at. If no clear ataxia has been shown in this, one 
 has the patient perform the same movement after closing his eyes, whereby 
 the ataxia usually increases considerably. To test the legs in ataxia one 
 makes use of the so-called "knee-heel experiment" : the patient lying down 
 is asked to touch with the heel of one foot the knee of the other leg, by 
 moving the leg through the air (not by drawing it up along the recumbent 
 leg). Then one should have him form letters, figures (three) or draw 
 circles in the air with the extended leg. All these movements should be 
 repeated with closed eyes. An ataxia of the facial muscles, too, may 
 occasionally be established by corresponding tests. Then the patient is 
 to sit up. Here there frequently appears in a quiet sitting posture swaying 
 of the upper part of the body (which still increases upon standing), com- 
 parable possibly to a tree shaken by the wind, which also increases when 
 the eyes are closed (static ataxia). Now one should have the patient rise 
 and place his heels and toes close together. Very many ataxics fail even 
 in this movement, they begin to totter and have to put one foot on the side, 
 so as not to fall; the disturbance becomes clearer if one has the patient 
 stand on one foot — a thing that a normal person can do easily after some 
 slight preliminary swaying — or specially, if, when standing with his feet 
 close together, he is asked to close his eyes. This uncommonly characteristic 
 symptom (Romberg's phenomenon) is one of the most delicate and most 
 easily performed tests of co-ordination, and the manner of the response 
 should be remarked, as it is somewhat different in the various forms of ataxia. 
 
 In the ataxia of tabetics (the most common variety), the patient first 
 attempts to struggle against the swaying by means of slight movements 
 with his feet; then he places his feet somewhat apart, and finally opens his 
 eyes. In cerebellar ataxia (cf. below) the swaying, already in itself of high 
 degree, does not increase when the eyes are closed. In the ataxia of hysterics 
 (especially in traumatic hysteria) the swaying becomes noticeable only when 
 
ATAXIA, TREMOR 37 
 
 the eyes are closed, but then immediately becomes excessive; the patients 
 make no attempt to arrest the interesting disturbance and fall with apparent 
 carelessness (but in reality with great caution) to the ground. 
 
 In walking, ataxia, as a rule, does not become higher in degree, than in 
 standing, but it assumes peculiar forms. The ataxic tabetic walks (in the 
 most severe cases) with legs far apart, protects himself, so to speak, by 
 planting his feet as widely apart as possible (to overcome the swaying that 
 seizes him) like a sailor on ship-board. He likes to use a cane, which he 
 puts down as far as possible from him, in order to gain as large a triangle 
 as possible to serve as a firm support. Other tabetics maintain the 
 direction of their movements, but raise their legs (usually in extension) 
 unnecessarily high, and replace them with stamping, a kind of walk, recal- 
 ling the older forms of the German military goose-step, usually called 
 "swinging" gait. The "cerebellar ataxic" walks quite differently. He 
 totters like a drunken man; the working of the synergisms necessary for 
 walking is normal, but the movements are wrongly connected with one 
 another; the gait looks as if the patient had been suddenly seized with 
 vertigo (which, as a matter of fact, is often connected with this form of 
 ataxia). To the group of cerebellar ataxia belongs also the disturbance 
 described by Babinski as " asynergia," which occasionally appears unilater- 
 ally. x\taxic tabetics walk, as a rule, far more poorly in the dark than by 
 day; this statement also is often characteristic, that their ataxia had been 
 noticed first by them in the morning when they had closed their eyes to 
 wash the face. 
 
 The regulation essential for co-ordination of movements is mediated by 
 centripetal — in the main, therefore- — sensory stimulations, which become 
 transformed in the centers of the cerebrum and the cerebellum; it is not yet 
 known how far the system of semicircular canals in the labyrinth, which 
 unquestionably serves to maintain equilibrium, subserves co-ordination. 
 Disturbances in co-ordination, ataxia, will appear, therefore, as soon as the 
 paths or centers of regulation are interrupted: tabes, Friedreich' s disease and 
 possibly ataxic polyneuritis on the one hand, affections of the cerebellum and 
 (very rarely) those of the cerebrum, on the other, lead to ataxia, but, strange 
 to say, never mere affections of the centrifugal path — that is, pure motor 
 paralyses with exception of the rare polyneuritic ataxia. We must, more- 
 over, here repeat again that ataxia has nothing to do with disturbances in 
 coarse strength, though occasionally, motor conditions of weakness other 
 than ataxia may occur (ataxic polyneuritis). 
 
 Ataxia, to a certain extent, represents an " insufficiency " of irritation 
 phenomena of the motor sphere, combined with unregulated motor in- 
 nervation. We come now to several symptomatic groups, all of which are 
 characterized by an "excess" of motor irritation phenomena. 
 
 As tremor are designated regular involuntary movements following 
 
38 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 one another rapidly, taking place in smaller or larger excursions about an 
 axis and always in one plane; they are evoked by involuntary muscular con- 
 tractions, in which agonists and antagonists alternately participate. One 
 distinguishes between a fine and a coarse tremor, according to the size of the 
 excursions, eventually also between a rapidly and a slowly oscillating tremor. 
 Furthermore there must be differentiated, principally, a tremor which appears 
 only upon motion, and one that appears only in rest. The two extremes are 
 represented by the tremor of multiple sclerosis and that of paralysis agitans. 
 The sufferer from multiple sclerosis in a state of rest, is, as a rule, abso- 
 lutely free from tremor. Also in simple tonic innervation, if, for instance, he 
 holds his hands quietly extended, no tremor appears. As soon, however, 
 as he intends to perform complicated movements, as in the test in which an 
 
 
 I egivving 
 
 Beginning ..ill/! J ' l\ /! 'J / I i 
 
 JlJlJ/fli"' Left Hand Right Hand ■• 
 
 Fig. 7. — Tracing of the intention tremor in multiple sclerosis. 
 
 attempt is made to bring together the tips of his fingers, tremor appears. 
 This tremor is first characterized by small excursions, but the more the 
 movement comes "to a point," the more delicate it becomes, the greater 
 the demands to reach the desired point, just as in the finger-tip experiment, 
 at the moment the finger tips meet, the larger the excursions become, 
 which can be represented by a diagram as follows (Fig. 7) : 
 
 If one has an ataxic perform the same movement, the diagram will be 
 something like the following (Fig. 8) — in which one must consider, more- 
 over, that the deviating excursions of ataxia may lie in altogether different 
 planes. 
 
 vV - A/^ 
 
 Fig. 8. — Tracing of intention ataxia in tabes. The individual strokes of the line frequently lie in 
 
 quite different planes. 
 
 It is, therefore, certainly not permissible to confound the "intention 
 tremor" of multiple sclerosis, with the "intention ataxia'''' say, of tabetics, 
 though there may be some points of similarity. Even the innervation dis- 
 turbances upon which they depend may be different. 
 
 Quite different is the tremor of paralysis, agitans {Parkinson s disease). 
 Here in intended, voluntary movements, there is little or no tremor; but if 
 the patient allows his extremities to lie or hang quietly, if he places his 
 hands in his lap, etc., there appears usually a very fine tremor with rapid 
 oscillations, which, in severe cases, persists in sleep and in the beginning 
 often attacks only one extremity, then, perhaps, half of the body, finally, 
 
NYSTAGMUS, ASSOCIATED MOVEMENTS 39 
 
 usually the entire body. It also appears in the hands in a peculiar type of 
 tremor, designated as "coin-counting" (pill-rolling), which consists of move- 
 ments of the thumb in front of the other fingers — that recall the movement 
 from which it derives its name. 
 
 Most other forms of tremor are of the type of fine oscillations, usually 
 only slight but nevertheless present in the state of rest, increasing upon 
 motion, such as neurasthenic and hysteric tremor, alcoholic and senile tremor 
 and the tremor in Basedow's disease. Slight tremor can be made plainly 
 visible by laying a piece of paper on the outstretched fingers of the patient. 
 
 Nystagmus represents, as has been mentioned above, an "intention 
 tremor" of the eye muscles. Some forms of tonic spasm, as, for instance, 
 blepharospasm (eye-lid spasm) of hysterics, are also associated with slight, 
 finely oscillating, tremor of the muscles concerned. 
 
 The so-called "fibrillary" or u fascicular'" twitchings of parts of one 
 muscle represent a totally different process. In this, we see at various places 
 on the surface of the body, small twitchings of individual bundles of muscles, 
 usually taking place without any effect on movement; they are occasionally 
 rhythmical, recalling, then, especially shivering from cold, but are frequently 
 arythmical, attacking always different muscular bundles in the same region 
 and may lead to a total "muscular wave" {myokymia). The genuine 
 fibrillary, that is, smallest, muscular twitchings of this kind are found only 
 in organic diseases of the central nervous system. All other muscular groups 
 can, after sufficient practice, be distinguished from these. We must add 
 here, moreover, that in affections of the anterior horn, etc., which are asso- 
 ciated with muscular atrophy, these twitchings are restricted, in the main, 
 to the atrophic areas, and occasionally also indicate the spread of the 
 process to other muscular parts preceding the atrophy. 
 
 In the realm of involuntary movements, there belong the associated or 
 co -movements. Small children scarcely ever make movements of grasping, etc., 
 with one hand alone, but innervate both hands and arms synchronously; 
 complicated processes, such as crying and laughing, are also very frequently 
 found together with associated movements on the part of the extremities. 
 These co-movements, with the progress of development disappear, leaving 
 behind only traces, but are occasionally found even at a more advanced age, 
 and return in pronounced fashion in organic paralyses, especially in those of 
 cerebral origin. Most hemiplegics, for instance, can not make energetic 
 movements of the sound arm entirely without associated movements of the 
 paralyzed arm, and this symptom is an important differential diagnostic 
 criterion between organic and psychogenous, hysteric paralyses, in which 
 latter it is always lacking {Hans Curschmann). 
 
 Finally the last three groups of. involuntary hyperkinesis, those which we 
 designate choreic disturbance in movement, tic and athetosis, show a con- 
 siderable similarity to one another. Each is well characterized in itself, 
 
4Q 
 
 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 presents a peculiar form of disease, may often be classed among the neuroses, 
 and may therefore occur without any " organic symptoms." Therefore their 
 knowledge and recognition are particularly important. Chorea is character- 
 ized by sudden violent twitching excursions, without rhythm and regularity, 
 appearing in rest, and not totally disappearing upon motion. In these 
 movements single muscles as well as muscular groups and entire extremities 
 are involved; in the general impression the "restlessness of the limbs" 
 predominates, since some of the violent movements may seem absolutely 
 intentional. The patient puts his hand up to his nose, purses his lips for 
 whistling, reaches for an object, etc. Frequently the disease is first uni- 
 lateral, but usually it appears over 
 the entire body affecting especially, 
 inmost cases, the face (making 
 grimaces, rolling the eyes, etc.) — 
 and besides this, very frequently 
 the psyche also is affected (Fig. 9) . 
 In the most severe cases, the 
 twitchings follow one another in 
 uninterrupted succession, the 
 patient throws himself about in 
 bed, tears his hair, rends the 
 sheets and his clothes, can scarcely 
 be quieted with hypnotics, and fre- 
 quently dies in this condition. 
 Less severe are the comparatively 
 rare choreic movements in organic 
 diseases of the brain. In contrast 
 to this, tic represents a regulated 
 movement, in so far that it always 
 attacks only definite groups of 
 muscles, usually, however, in the 
 same brief twitching way as in 
 chorea. The movement may, at 
 first sight, appear voluntary, in- 
 tentional; the patient shrugs his 
 shoulders, throws his head backwards, strikes the table with his hand. The 
 same movement is repeated, not rhythmically, but at all times, upon all 
 occasions, increases especially during excitement, and can not be suppressed 
 in spite of the most earnest desire of the patient to put an end to it. If the 
 motility disturbance affects muscular groups over the entire body, one speaks 
 of "maladie des tics," which, however, can not, in its nature, be totally identi- 
 fied with simple tic (Gilles de la Tourette). Chorea and tic are frequently 
 accompanied by psychic disturbances. Athetosis also occasionally occurs as 
 
 FlG.'o 
 
 -Clonic spasms in chorea hereditaria. 
 
 {After Huntington.) 
 
SPASMS, CONVULSIONS 41 
 
 an independent neurosis, sometimes, however, after hemiplegia in the para- 
 lyzed limbs, is frequently combined with contractures and nearly always 
 affects only fingers or toes. In this case, the movements are sluggish, 
 vermicular, always with considerable effect upon motility and often of very 
 strange form. The fingers are extended, stretched, overstretched and bent 
 in the most remarkable manner, assuming the most unlikely positions. 
 Whereas in chorea and especially in tic there are usually longer or shorter 
 intervals of rest between the separate movements, athetosis is an almost un- 
 ceasing hyperkinesis, suppressed, as a rule, only during sleep. 
 
 Spasms (cramps) have often been designated as hyperkinesis in the 
 narrower sense, among which we can with some justification reckon the 
 motility disturbances we have just mentioned (especially tic). We dis- 
 tinguish as main groups, tonic and clonic spasms. By tonic spasm we mean 
 permanent conditions of contraction in one muscle group; the most severe 
 forms are called tetanic spasm, tetany. Clonic spasms are brief, rapid 
 twitchings, usually repeating themselves with a certain rhythm, which may, 
 in the highest degree, attack the entire body (convulsions) . We have already 
 found an example of isolated clonic spasm in tic. It appears frequently as a 
 reflex spasm (tic convulsif of the facial area) . The majority of spasm forms 
 belonging to this chapter, however, are to be classed among the neuroses or 
 psycho-neuroses, and may all probably be localized in the cerebral cortex. 
 This is certainly true of the spasms in Jacksonian epilepsy, where, as the 
 result of irritation of a definite part of the cortex (scar, tumor) tonic, or more 
 frequently, clonic spasms, always of definite muscular group, usually only 
 unilateral in the opposite half of the body, are evoked, with or without loss 
 of consciousness. Analogously, we may assume that convulsions of common 
 epilepsy may originate in the cerebral cortex. The question of localization 
 with respect to the major hysteric attacks of spasms is more difficult to answer 
 (Fig. 10). In appearance, epileptic and hysteric attacks are but little dif- 
 ferent and may pass over into each other. As to the differential diagnosis, 
 more will be said in the special chapters. In general, the tonic conditions 
 predominate in epilepsy; usually consciousness is entirely lost. In hysteria, 
 even the "major attack" is usually composed of periods of tonic rigidity and 
 clonic twitchings and consciousness is not totally lost, at least a psychic re- 
 action can usually be evoked by means of external influence, especially if 
 painful. The epileptic patient falls to the ground, usually without noticeable 
 prodromes (aura) of the attack, his arms and feet twitch a little, his teeth 
 gnash spasmodically, and there soon appears a high degree of tonic tension 
 throughout his entire body with finely oscillating clonic vibrations of the 
 muscles in tension; respiration becomes superficial, the patient wheezes, 
 becomes cyanotic, foams at the mouth, frequently urine is voided involuntar- 
 ily, until after a few minutes (rarely more) , tension is relaxed and conscious- 
 ness as a rule, soon returns with the restoration of the reflexes that had dis- 
 
42 
 
 GEXERAL DIAGNOSTICS OF XERVOUS DISEASES, 
 
 appeared (also of the pupils). The entire event creates an impression of a 
 real, genuine attack; not rarely the patients are injured by falling, or bite 
 their tongues. In the hysteric attack half is usually "histrionic." The 
 attack rarely appears when the patients are wholly unobserved; frequently it 
 crowns the demonstration of a passionate emotional outbreak. The hyster- 
 ical patient produces his effects with far coarser means than the epileptic; 
 the entire body is convulsed with twitchings, assumes "attitudes passionelles," 
 like the position of a crucified man, erotic positions, etc., lies in bed in the 
 form of an arch, so that only the back of the head and the heels touch the 
 
 Fig. io. — Hysteria (major hysteric attack). Attention is called to the opened and fixed eyes. 
 (After Schoenborn-Krieger , Klinischer Atlas der Nervenkrankheiten) 
 
 sheet. Any suggestive influence, pressure in " hysterogenous " zones, 
 faradization, in lighter cases, sprinkling with cold water and addressing the 
 patient may check the attack, which otherwise may last for hours. If the 
 patient falls to the ground, he usually falls carefully so as not to injure him- 
 self. In spite of all these signs of recognition, the differentiation of these two 
 forms of spasm may, as has been said, be very difficult. For a discussion of 
 separate rarer forms of spasms, such as the tonic tetanic spasms which are 
 localized especially in the extremities, myoclonus, occupational cramps, as 
 well as for traumatic tetanus, cf. the particular divisions devoted to these 
 subjects. 
 
 In the test of motility or of coarse strength we have still two phenomena to 
 mention. In some cases, upon first testing the muscles, as in warmly 
 shaking the patient's hand to see how strong his grip is, etc., his strength is 
 
MECHANICAL MUSCULAR EXCITABILITY 43 
 
 very good, but he is unable to relax the muscle that has been contracted. 
 But if he repeats the same movement several times in succession, "for 
 practice," the contraction and relaxation take place more and more rapidly, 
 finally, at the normal rate of speed; the grasp of the hand, which, like the 
 clutch of a vise, would not, in spite of all the endeavors on the part of the 
 patient, release the examiner's hand, can now no longer be distinguished from 
 the grasp of a healthy man, until after some period of rest, the first condition 
 reappears. In this disease, myotonia (Thomsen's disease), which, as a rule, 
 attacks the entire body and may be accompanied by very good coarse 
 strength, even with hypertrophic musculature (cases of atrophy are rare), 
 abnormal anatomic conditions and transformations in the muscle itself 
 probably play the main serological role. In a certain sense, myasthenia or 
 myasthenic paralysis forms a contrast to this — the picture of an abnormal 
 tendency to fatigue of the otherwise apparently normal musculature. Here, 
 it is true, the patient at first, for a few times, may perform the prescribed 
 movement rapidly and powerfully; after the fifth or tenth time his strength 
 decreases, and soon, in spite of all his efforts, he is unable to execute the 
 movement, until, after a resting period, the muscles have again recovered. 
 For this disease picture, which has not yet been consistently interpreted, 
 there exist, moreover, as for myotonia, analogous electrical changes (cf. 
 below). 
 
 An absolutely essential part of every test of the motor sphere is the 
 electrical examination. But it should be preceded by the test of the mechani- 
 cal excitability of the muscles and nerves, comparable with it in many respects. 
 We understand by this, naturally, not the pain stimulations that affect the 
 skin, but the movements due to pressure, tapping or striking, which affect 
 the skin as little as possible, and strike the underlying portions, as far as 
 possible, in isolation. For this test, almost without exception, only the finger 
 of the examiner or the tap of the percussion hammer is suitable. 
 
 Changes in mechanical muscular excitability are demonstrable almost 
 exclusively in the form of an increase in excitability, or of qualitative change 
 in it. For there is no norm in respect to the effect of tapping a sound muscle. 
 The local muscular prominence may form an " idiomuscular contraction" 
 but it may be very slight and, as happens in the majority of cases, may 
 disappear again immediately; it may also be very pronounced and persist 
 for some time. This increase in mechanical muscular excitability is found in 
 tuberculosis, typhoid, in most cachexias, here and there, in sciatica and 
 affections of the anterior horn. Very frequent, and occasionally classical, on 
 the other hand, is the qualitative change in excitability, the mechanical proof 
 of the reaction of degeneration (cf. below), or more correctly the "sluggish 
 mechanical contraction" as it is often found in the degenerative atrophies, 
 especially at an early stage. In ulnaris neuritis, bulbar paralysis, etc., 
 tapping the ball of the little finger, or the thumb, evoke the most beautiful 
 
44 
 
 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 "sluggish contractions." In Thomsen's disease, also, the mechanical tonic 
 twitching corresponding to the myotonic reaction (cf. below), the "arrest" 
 of contraction may be typically demonstrated. The theoretically postulated 
 lowering of mechanical excitability for severe old paralyses, for myasthenia, 
 etc., can not, on the other hand, as a rule, be shown. For the mechanical 
 excitability of the nerves also — which, in our sense, can be used only for motor 
 nerves — only an increase in excitability can be demonstrated; decrease and 
 qualitative change can not be proved. The mechanical increase in the 
 excitability of the nerves is best shown in some nerves lying near the surface : 
 
 Frontalis. 
 Facial {upper). 
 
 Corrugator super- 
 cilii. 
 
 Orbic. palpe- 
 brarum. 
 
 Nasal muscles. -J 
 
 Zygomatici. 
 
 Orbic. oris, -j 
 
 Facial (middle) . 
 Masseter. 
 
 Levator menti. 
 
 Quadratus. 
 
 Triangularis. 
 
 Hyfoglossus. 
 
 Facial (lower). 
 
 Platysma myoides. 
 
 Hyoid muscles. ] 
 
 Omohyoid. 
 
 Ext. anterior, 
 thoracic (pectoralis 
 major). 
 
 Ascending frontal and 
 parietal convolutions 
 (motor area). 
 
 Third frontal convolu- 
 tion and insula (cen- 
 ter of speech). 
 
 Temporalis. 
 Facial (upper branch). 
 Facial (trunk). 
 Posterior auricular. 
 Facial (middle branch). 
 Facial (lower branch). 
 Splenius. 
 
 Sternomastoideus. 
 
 Spinal accessory. 
 Levator anguli 
 
 scapulae. 
 Trapezius. 
 
 Dorsalis scapula 
 (rhomboidei). 
 
 Circumflex. 
 
 Long- thoracic (ser- 
 ratus magnus). 
 
 Phrenic. Fifth and sixth cervical 
 (deltoid, biceps, bra- 
 chialis anticus, supi- 
 nator longus). 
 
 Brachial plexus. 
 
 Fig. ii. — Motor points of face and neck. {After Erb and de Watterville.) 
 
 ulnaris (at the elbow), peroneus (capit. fibulae), facialis (most of its branches 
 as well as the trunk) . This increase, as a matter of fact, is found only in a 
 single disease, tetany, in which tapping upon the ulnaris at once evokes 
 twitchings in the muscles of the ball of the little finger, tapping or stroking 
 of the facial branches, unilateral twitching of the mimetic muscles (Chvostek's 
 symptom) . 
 
 The conclusions reached by testing the electrical reaction of the muscles 
 and nerves are very much more comprehensive. But in this field, which in 
 modern medicine, even in neurology, is somewhat unkindly treated and 
 
ELECTRICAL EXCITABILITY 
 
 45 
 
 rather neglected, there are necessary, more than in others, careful observa- 
 tion, experience and detailed knowledge of the technique (above all of the 
 electrical apparatus used by the examiner) . Therefore, if possible, one should 
 make an electrical examination of patients, only with an apparatus one owns 
 
 Triceps (long head). 
 
 Triceps (inner head). 
 Ulnar. 
 
 Flexor carpi ulnaris. 
 
 Flexor profundus 
 digitorum. 
 
 Flexor sublimis digitor 
 um (II and III). 
 
 Flexor sublimis digitor- 
 um (index and little 
 fingers). 
 
 Ulna?-. 
 
 Palmaris brevis. 
 
 Abductor min. digit. 
 
 Flexor min. digit. 
 
 Opponens min. digit. 
 
 I/umbricales. - 
 
 Deltoid (anterior 
 portion). 
 
 sublimis digitorum. 
 Flexor longus pollicis. 
 Median. 
 
 Abductor pollicis. 
 Opponens pollicis. 
 Flexor brevis pollicis. 
 Adductor pollicis. 
 
 Fig. 12. — Motor points on upper limb, flexor surface. {After Erb and de Watterville.) 
 
 oneself, or, at least, always with the same kind, which should comprise a 
 faradic (with the greatest possible variation in the relation between tube and 
 core) and a galvanic battery with a good galvanometer, as well as several 
 ordinary electrodes, a button electrode and several plate electrodes (surface 
 of 10 to 60 sq. cm.). 
 
46 
 
 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 We work, as a rule, with two electrodes of different size, a large so-called 
 indifferent one, about 60 sq. cm. and a small active one, as like as possible 
 Erb's normal electrode of 10 sq. cm. (round or square). The large one is 
 placed upon an indifferent spot (the sternum is best) and may be held there by 
 the patient himself; the small one is placed upon the muscles and nerves to 
 be tested. For the necessary opening and closing of the current, one makes 
 
 Deltoid (poste- 
 rior part). 
 
 Muscidospi7-al. 
 Brachialis anticus. 
 
 Supinator longus. 
 Ext. carpi radial, longior. 
 Ext. carpi radial, brevior. 
 
 Extensor communis ( 
 digitorum. \ 
 
 Extensor indicis 
 
 Ext. ossis metacarpi pollicis. 
 Ext. primi internodii pollicis. 
 
 t T f fl » 
 
 Dorsal interossei. < tj ' 
 
 :-* Triceps (long head). 
 
 Triceps (outer head)- 
 
 Extensor carpi ulnaris. 
 Supinator brevis. 
 
 Extensor minimi digiti. 
 Extensor indicis. 
 
 Extensor secundi internodii 
 pollicis. 
 
 Abductor minimi digiti. 
 Dorsal interossei (III and IV). 
 
 Fig. 13. — Motor points on upper limb, extensor surface. (After Erb and de Watterville.) 
 
 use of the interrupting electrode, upon the handle of which, pressure on a 
 button or a smiliar device, causes a break in the current. 
 
 Now, which points does one examine? Where does one set the active 
 electrode? In general, upon the empirically determined points of entry of 
 the nerves into the muscles — for the electric test of the musculature — and 
 upon the places in the nerve trunks where these approach the surface. These 
 "muscle and nerve points" were determined by Erb upon a foundation of 
 
ELECTRICAL EXCITABILITY 
 
 47 
 
 extensive and detailed investigation (Figs, n to 16). The significance of the 
 individual points is clear, without further discussion ; they show that if one 
 places the large electrode upon the sternum and the small one upon one of 
 the points upon the surface of the body here designated and employs (farad- 
 ically) a sufficiently strong current or (galvanically) opens and closes the 
 current, then, the muscle named in the tables (at the nerve points, the muscles 
 subserved by this nerve) twitches, that is, momentarily (galvanically) or 
 tonically, lastingly (faradically) contracts. If it be a matter of a diffuse 
 affection, all these muscular and nerve points must be examined in this way, 
 
 Anterior crural. 
 
 Obturator. 
 
 Pectineus, 
 
 Adductor magnus. 
 Adductor longus, 
 
 Crureus 
 
 Vastus internus 
 
 \ Tensor fasciae femoris 
 
 Sartorius. 
 
 Quadriceps femoris. 
 Rectus femoris. 
 
 Vastus externus 
 
 Fig. 14. — Motor points on thigh, anterior surface. {After Erb and de W.attervitte.) 
 
 while in local affections only the regionary muscular and nerve points are to 
 be thus examined. First the faradic and then the galvanic examination 
 should be made. 
 
 In testing the faradic excitability, one determines what minimal strength 
 of current barely suffices to evoke a contraction of the muscles, and (theo- 
 retically) in the indirect test — stimulation of the nerves — as well as in the direct 
 stimulation of the muscles. This minimal power of the current is shown in 
 millimeters upon the instrument, but since all apparatus for measuring the 
 faradic current are unreliable, and not absolutely equal in any two sets of 
 
48 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 apparatus, the examination is of direct value only where one can compare in a 
 patient the diseased muscle and nerve with the sound one on the other side, 
 when one can, to a certain extent, estimate the capacity of the skin to resist 
 the current and works with an apparatus one has already tried. Certain 
 normal medium figures for these "threshold" values have been determined 
 by Erb, Stintzing and others and may serve as points of reference; usually 
 Stintzing's figures are used. But the unskilled examiner (especially when the 
 nerve points are, as is sometimes the case, hard to find) often meets consider- 
 able variations from these mean values. Of these mean values, we shall 
 reproduce here some from Stintzing's instrument. But we must emphasize 
 the fact, that in direct muscular stimulation, the excitability fluctuates within 
 such wide bounds, that usually one is contented with reproducing the most 
 manageable values of the limit of indirect (nerve-) excitability. 
 
 Distance table for 
 Nerve or muscle minimal twitching 
 
 N. facialis 132-110 
 
 N. medianus 135-110 
 
 N. ulnaris 130-107 
 
 N. peroneus 127-103 
 
 N. cruralis 120-103 
 
 N. radialis 120-90 
 
 N. accessorius 145-130 
 
 N. musculocutaneus 145-125 
 
 N. axillaris 125—93 
 
 N. thoracicus ant 145-110 
 
 The direction of the current is of no importance in this test. In faradic 
 stimulation, upon interruption of the current ("opening shocks") there 
 appears a momentary contraction, otherwise, a tonic contraction lasting as 
 long as the current is applied. 
 
 The procedure in testing the galvanic excitability of the muscles and 
 nerves is somewhat different. Here, too, the large indifferent electrode is 
 placed upon the sternum (possibly upon the nape, or the lumbar portion of 
 the spinal column), the small, active one upon the points to be tested. Only 
 opening and closing of the current evokes muscular twitching, not the dura- 
 tion of the current. The contraction in the normal state, is brief, lightning- 
 like; tetanus appears only upon the use of very strong currents — but never 
 "sluggish contraction" (cf. below). But here the direction of the current 
 is of importance. We always begin by taking as the " active" electrode, 
 the cathode or negative pole. 
 
 Now we determine at what power of the current appears the minimal 
 
ELECTRICAL EXAMINATION 
 
 49 
 
 contraction for direct and for indirect stimulation. The measure of the 
 strength of the current is here given exclusively by the galvanometer, which 
 directly indicates in milliamperes the strength of the current. It is best, 
 in every examination to insert the galvanometer from the first into the 
 circuit of the current, since the resistance that it always offers to the current, 
 would, if it were put in and taken out later, destroy the result of the test; 
 if the galvanometer is excluded, a smaller number of elements will always 
 be necessary to secure the same strength of current and the minimal con- 
 
 ) 
 
 r Gluteus maximus, 
 
 Sciatic. 
 
 Biceps (long head). 
 Biceps (short head). 
 
 Peroneal. 
 
 Gastrocnemius 
 (outer head). 
 
 Soleus. 
 
 Flexor longus hallucis. 
 
 ) 
 
 Adductor magnus. 
 
 Semitendinosus. 
 
 Semimembranosus. 
 
 Posterior tibial. 
 
 Gastrocnemius 
 (inner head). 
 
 Soleus. 
 
 Flexor longus digitorum. 
 Posterior tibial. 
 
 Fig. 15. — Motor points on lower limb, posterior surface. {After Erb and de Watterville.) 
 
 traction. Furthermore, one must see to it that the electrodes, as always 
 should be done in electrical examinations, are thoroughly moistened with 
 warm water (salt water is unnecessary), and remember that in the gal- 
 vanic test, the resistance of the skin to the current decreases with the length 
 of application. 
 
 For galvanic minimal contractions, too, a series of average values have 
 been given; some of the most important, according to Stintzing, are: 
 4 
 
50 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 Minimal strength of 
 the current in Milli- 
 Nerve amperes (mean wave) 
 
 N. facialis 1.0-2.5 
 
 N. accessorius o .01-0 . 44 
 
 N. medianus 0.3 -1.5 
 
 N. ulnaris 0.6-2.6 
 
 N. radialis 0.9 -2.7 
 
 N. cruralis 0.4-1.7 
 
 N. peroneus 0.2 -2.0 
 
 N. tibialis post 0.4-2.5 
 
 The values, as shown above, vary considerably even in sound persons. 
 But the following must be remarked: these values, and especially the 
 
 Tibialis anticus. _, 
 Extensor longus digitorum 
 
 Peroneus brevis. 
 
 Extensor longus hallucis. 
 
 Interosse 
 
 _ Peioneal. 
 
 Gastrocnemius. 
 Peroneus longus. 
 
 •Soleus. 
 
 
 Flexor longus hallucis. 
 
 Extensor brevis digitorum, 
 Abductor minimi digiti. 
 
 Fig. 16. — Motor points on leg, external surface. {After Erb and de Watterville.) 
 
 "threshold" values of the galvanic stimulation of the sound nerves, hold 
 good for the cathodal closure contraction (C C C) that is, for the twitching, 
 which appears when the cathode is placed as active electrode upon the point 
 to be tested, and the current is passed in, then closed off. According to 
 the law of contraction this is the twitching that first appears in normal nerves. 
 
ELECTRICAL EXAMINATION 51 
 
 Now, if we open the current, and then by means of the commutator which 
 is necessary in every galvanic battery, reverse it, and then alternately 
 interrupt it (opening and closing), whereby the active pole which was 
 formerly the cathode has now become the anode, we find, if the power of 
 the current is kept as strong as before, no further twitching. But if we 
 slowly increase the current, we see first that, besides the twitching that 
 results from cathodal closure and remains the strongest, there appears a 
 contraction upon opening (interrupting) the current, at the pole that has 
 become the anode — the anodal opening contraction; at the same time, too 
 (sometimes a little earlier), there occurs a contraction at closing the anode 
 (A O C and A C C) . If we further increase the current, there results at the 
 cathode, upon closing the current, instead of the former brief twitching, 
 tetanus (C C Te), as long as the current is kept closed, upon still further 
 increase, contraction at the opening of the cathode (C O C), and an anodal- 
 closure tetany (A C Te). Naturally, it is theoretically possible and occurs 
 also in practice, that at the place where the indifferent electrode is used, 
 twitchings are also evoked simultaneously with those at the active pole 
 (occasionally especially disturbing in facialis paralysis) . This disturbance, 
 which may make the picture confusing, should be avoided by taking a large 
 electrode at the place which one wishes to be indifferent (at which if possible 
 no contractions are to be made manifest), which possesses slighter density 
 of current, and by placing this electrode precisely upon indifferent places 
 (sternum, spinal column) , where no nerves are located directly below the 
 surface of the skin. This "loop of current" from the indifferent electrode 
 may really become disturbing — if it be a question of determining precisely 
 the place of lesion of a nerve by electricity, and one follows the trunk of the 
 nerve with the indifferent electrode while the active one is placed upon 
 the muscle, so that under certain circumstances the two electrodes approach 
 each other closely. 
 
 It should be briefly mentioned that an electrical (as a rule only galvanic) 
 test of the sense nerves may be performed. The examination of the eye 
 is made as follows : the indifferent large electrode is placed upon the sternum 
 or nape, the active, small (normal) electrode, best covered with a sponge, 
 is placed on the closed eyelid, and now the current closed, naturally with 
 weak current values. The law of contracture remains as above, the reaction 
 of the nerves is an intensive light (sometimes also color) sensation; the 
 threshold of stimulation is usually very low. For the ear, it is best to use 
 as electrode, a normal or a button electrode, placing this upon the tragus; 
 there appear buzzing or whistling auditory sensations (the method is much 
 used nowadays for otological tests in labyrinthine vertigo, so-called voltaic 
 vertigo). Finally the sense of taste may be tested (small active button 
 electrode on the tongue or cheek), upon which the stronger sour taste 
 appears at the cathode, the weaker salty taste at the anode. This fact, 
 
52 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 moreover, may be used to ascertain the position of the pole, if one does not 
 know it; one places an electrode on each cheek, and passes a weak current 
 through; the side upon which a usually distinct sour sensation appears, 
 corresponds to the positive pole, to the anode. 
 
 Quantitative changes in electrical excitability of the muscles and the nerves 
 in unhealthy conditions are alone shown when the faradic current is used; 
 nerves and muscles are less or more excitable than in the normal con- 
 dition; with the galvanic current, on the other hand, quantitative and 
 qualitative changes are shown. We shall now take up the former. 
 
 Diminution and even cessation of faradic excitability is found for the 
 muscles as well as for the nerves in all possible diseases of the peripheral 
 neurons. Its combination with qualitative changes in galvanic excitability, 
 as in the reaction of degeneration, will be mentioned below. Mere diminu- 
 tion is found in all simple (that is not degenerative, cf. below) atrophies, in 
 some cerebral and spinal paralyses, in dystrophy, occasionally too, in other 
 diseases . that lead to degenerative atrophy (neuritis, poliomyelitis, etc.), 
 and then, as a rule, as the first or last stage of the electrical change; finally also 
 in the rare myotonia (cf . above) . An increase in faradic excitability is found 
 almost exclusively in tetany and even there not constantly. Increase or 
 decrease in faradic excitability is recognized simply by the increase or de- 
 crease of the gradations for the minimal twitching, as compared with the 
 mean values. 
 
 Increase in galvanic excitability is found (except in the beginning of R. D., 
 cf. below) also only in tetany, and here constantly for the excitation of the 
 motor, frequently for that of the sensory and sense nerves (especially of the 
 acusticus); it is recognized by the small power of the current necessary to 
 produce the minimal twitching. Decrease in galvanic excitability is found 
 simultaneously with that of faradic excitability in the diseases mentioned 
 above (at least for the indirect stimulation — that of the nerves). 
 
 More important, and in certain diseases far more common, is the appear- 
 ance of the reaction of degeneration (R.D.). In this we must distinguish 
 clearly between the electric condition of the nerves and that of the muscles. 
 In the "total" reaction of degeneration: 
 
 (a) The nerve is excitable neither faradically, nor galvanically. 
 
 (b) The muscle is not excitable faradically, on the other hand its galvanic 
 excitability is increased upon direct stimulation (only at the beginning of the 
 disease) ; besides this the law of contraction is so changed for the muscle that 
 there appears at first reaction (to the weakest stimulation), a twitching upon 
 anodal closing or even upon its opening ("reversal of the law of contraction"). 
 Besides this, the twitching becomes sluggish, that is, the contraction rises 
 gradually to its height, and then sinks again gradually, slowly; the difference 
 in the twitching curve in direct stimulation of the muscles is about that seen 
 in Fig. 17. 
 
DEGENERATION, MYOTONIC AND MYASTHENIC 53 
 
 There is also a partial reaction of degeneration, generally in less severe 
 lesions, or as preceding or following a stage of total lesions; here the only 
 f aradic muscular excitability is decreased or even lost, and sluggish twitching 
 is demonstrable; on the other hand, the excitability of the nerves may be re- 
 tained for both kinds of currents. The distinction of the total from the par- 
 tial R. D. may be important diagnostically as well as prognostically, since 
 the latter trouble is cured considerably more quickly. 
 
 The reaction of degeneration occurs everywhere, where degenerative 
 processes exist in the anterior horns, nerves, or muscles. Here belong 
 
 poliomyelitis ant., syringomyelia, tumors, 
 
 fl / \^ myelitis, bulbar paralysis, spinal muscular 
 
 / / / N. atrophy, amyotrophic lateral sclerosis; all 
 
 Normal sluggish contraction. peripheral nerve lesions; finally in rare 
 
 contraction cases, some muscular diseases, as to the 
 
 Fig. 17. — Diagram of the normal and ' ' 
 
 the sluggish contraction in closing the genesis of which we have not yet full knowl- 
 
 galvanic current. i /j i \ 
 
 edge (dystrophy). 
 
 We must mention, that, as excellent investigations of Grund have con- 
 firmed, the influence of cold may evoke the picture of partial reaction of 
 degeneration (sluggish twitching, equalization and even preponderance of 
 the anode over the cathode) {"cooling off reaction"). The curves of these 
 twitchings are not identical with those of the R. D., but even exceed them in 
 sluggishness. This "cooling off reaction" may appear spontaneously in the 
 small muscles of the hand of the healthy, and persist for a very long time 
 (for hours even in a very warm room) , evince itself also in the larger muscles 
 of the body upon a particular cooling off, and it may appear precisely in 
 diagnostically important cases, in which, then, warming of the muscles is 
 necessary to ascertain whether a partial R. D. is present. 
 
 In spite of this possibility of a confusion, the diagnostic value of R. D. is 
 very great; we must emphasize again the special importance of recognizing 
 the sluggish twitching. 
 
 The myotonic reaction of Thomsen's disease (myotonia congenita) pre- 
 sents a peculiarly abnormal disturbance in electrical excitability. Here, 
 upon direct galvanic and faradic stimulation of the muscles, there appears 
 even with weak currents, a "persistent" tonic contraction of very consider- 
 able duration; the My. R. is especially striking upon weak galvanic stimula- 
 tion. Stronger faradic currents effect also, on the part of the nerves, a tonic, 
 permanent contraction. The phenomenon is demonstrable in the (usually) 
 hypertrophic, as well as (in the rare cases of this type) in the atrophic, muscles 
 of such patients, occasionally very similar to R. D., but always without rever- 
 sal of the law of contraction. 
 
 As " myasthenic reaction" (Mya.R.), one designates an abnormal tend- 
 ency to fatigue, for the faradic current, on the part of the nerves as well as of 
 the muscles, appearing in myasthenia gravis pseudo paralytica. If one 
 
54 
 
 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 repeats at short intervals, faradic stimulations of equal strength, after some 
 (possibly 8 or 10) stimulations, a rapid decrease, even a disappearance of 
 excitability, will be observed. In rest, the muscle and nerve soon "recover" 
 again. Upon long lasting faradization, the contraction decreases from 
 second to second and finally disappears (Jolly). 
 
 My. R. and Mya. R. correspond throughout to the analogous changes 
 in active and mechanical excitablity in myotonia or myasthenia. Some 
 rare electric changes will be discussed in the separate chapters. 
 
 D. Sensibility 
 
 The disturbances in sensibility in their various forms may be subjective 
 and objective, but in no process in the human body is there a greater lack of 
 harmony between the subjective troubles of the patients and the objective 
 
 N. cutan. brachii lateralis (N. 
 axillaris) . 
 
 Nn. supraclavicularis. 
 
 Rami cutanei laterales Nn. 
 intercostalium. 
 
 N. cutaneus brachii medial. 
 
 Rami cut. brachii anterioris des N. 
 cut. antibrachii medialis. 
 
 N. cut. brachii posterior (N. radialis). 
 
 N. cutan. antibrachii lateralis 
 (N. musculo-cutaneus) . 
 
 . . Ram. volaris des N. cutan. antibrachii 
 medialis. 
 
 Ram. cut. palmaris des N. medianus. 
 Ram. superficial, des N. radialis. 
 
 Nn. digitalis volaris des N. medianus. ^--r.-jj-l 
 
 Ram. cutan. palmaris des N. ulnaris. 
 
 Ram. superficialis des N. ulnaris. 
 
 Fig. 18. — Distribution of the cutaneous nerves in the upper extremities. (After Toldt.) 
 
 findings, than in disturbances in sensibility; nowhere do the deficiencies in 
 our technique appear more sharply than here (Figs. 18 to 24). 
 
 A glance at the way sensibility is tested will confirm this. We distinguish 
 first, quite roughly, the methods of testing the separate qualities of sensation 
 (the designation " quality, " however, has by no means been proved justifiable 
 
TA C TILE SEXSIBILI T Y 
 
 55 
 
 in every direction) ; common to all, is naturally the great defect, that in making 
 the test we are (with a few exceptions) absolutely dependent upon the 
 patient's statement. 
 
 We test first the sense of touch. We have the patient close his eyes, or 
 cover them, and touch in succession with our finger tip the various portions 
 of the surface of the body. I consider this simplest of all methods the best, 
 because it alone affords the examiner possibility of certain control of the 
 pressure in the experiment, and alone permits keeping touch at the same 
 pressure. In the otherwise much used test by means of a small brush, the 
 
 X. supraclavicular • 
 
 Ram. cut. lateralis N. intercostalium. 
 
 N. cut. brachii lateralis (X. axillaris). 
 
 N. cutan. branchii medialis. -• -- 
 
 N. cut. brachii posterior (X. radialis). 
 
 Ram. ulnaris of the X. cutan. antibra- 
 chii medialis. 
 
 X T . cut. antibrachii lateralis (X. 
 musculocutaneus) . 
 
 X. cut. antibrachii dorsalis (X. 
 radialis) . 
 
 Ram. dorsalis manus. N. ulnaris. 
 
 Nn. digital, volares proprii. of -the N- ^_ 
 ulnaris. 
 
 Ram. superficial of the X. radialis. 
 
 "-"•Si Xn. digitatis volares proprii of the X. 
 medianus. 
 
 Fig. 19. — Distribution of the cutaneous nerves in the upper extremities. {After Toldt.) 
 
 touch in itself is always very light, but a fine gradation of the pressure of the 
 brush touch is almost an impossibility, and yet there occurs often the neces- 
 sity for ascertaining even very fine distinctions in touch sensibility. The test 
 of the " touch circles " of the skin by simultaneously placing upon it the points 
 of a compass at varying distances — which answers at the same time for a test 
 of "orientation" — the patient to state whether he feels one or two points of 
 contact, when the points of the compass are at a definite distance from 
 each other, may also be dispensed with, since even upon the normal skin it 
 gives widely varying results. 
 
56 
 
 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 It is best to carefully touch first, parts of the body which have a good 
 sense of touch, only then those that have less sensibility, and let the patient 
 respond to every touch he feels with the word "now." But here it is nec- 
 essary occasionally to touch also distant parts of the skin, in order to avoid a 
 sequence of symmetrical points on extremities (which the patient himself for 
 the most part unconsciously compares) and a definite rhythm of the touches, 
 since through all these procedures the task of the patient is made too easy, his 
 attention too little occupied, and I recommend also asking from time to time 
 "Do you feel this? this? this?" — occasionally asking the question without 
 really touching the skin. Nothing else so sharpens the attention. If one has 
 
 Skin distribution fifth nerve 
 
 Skin distribution 
 fifth nerve 
 
 Skin distribution 
 brachial plexus 
 
 Lateral cutane- 
 ous intercostal 
 branches 
 
 Skin distribution 
 sacral plexus 
 
 Skin distribution 
 cervical plexus 
 
 Skin distribution 
 brachial plexus 
 
 Lateral cutane- 
 ous branches (in- 
 tercostal) 
 
 N. dorsalis penis 
 
 Skin distribution lumbar plexus 
 Figs. 20 and 21. — Distribution of the cutaneous nerves in the trunk. {After Toldt.) 
 
 found parts with lessened sensibility to touch, one should try to make a 
 boundary between these and those that have the normal sensibility and im- 
 mediately observe whether this boundary is of a segmentary nature, follow- 
 ing the distribution of the individual cutaneous nerves, or of a hysteric (glove- 
 and sleeve-like, unilateral, etc.) character. One should note also whether 
 the touch which is felt everywhere may not be in some regions (affected with 
 paresthesia) felt "different" than in those with normal sensations, which is 
 not rarely the case. For these cases and for the lightest objective disturb- 
 ances in sensibility, it is also recommended to let the patient decide whether 
 he is being touched with the "point" or the "eye" (sharply or bluntly) of 
 a needle which is not too sharp; with some practice, the disturbing factors 
 of pain and pressure that are easily produced here may be avoided. 
 
 The mucous membranes which, on the whole, show somewhat slighter 
 sensibility than the skin, may be tested in like manner, but the results are then 
 less reliable (cf. below). 
 
THERMAL SENSIBILITY 
 
 57 
 
 The normal sensibility of the skin varies in delicacy in the different parts 
 of the body, being finest at the ends of the fingers and the tip of the tongue, 
 and dullest (even to anaesthesia) upon the callous portions of the soles of the 
 feet. 
 
 If we have examined the touch sensibility and noted the disturbance that 
 we may have found upon a diagram of the body (which is earnestly recom- 
 mended), we proceed to test the temperature sense or more correctly the sensa- 
 tions of heat and cold. For this we use test tubes, filled with warm and 
 
 Ram. cutaneus lateralis des N. 
 iliohypogastricus. 
 
 N. cutan. femoris lateralis. 
 
 N. lumboinguinalis. 
 
 N. ilioinguinal. N. spermaticus 
 extemus. 
 
 Ram. cutan. femoris arteriores. 
 
 Ram. cutan. N. obturatorii. 
 
 N. cutan. surae lateralis {a, d, N. 
 peronaeus). 
 
 \J/ 
 
 Ram. cutanei cruris mediales des N. 
 saphenus. 
 
 N. tibialis. 
 
 N. peron. superficial. 
 
 ~-~" N. peron. profundus. 
 Fig. 22.— Distribution of the cutaneous nerves in the lower extremities. {After Toldt.) 
 
 with cold water (no extreme degrees of heat and cold, through which pain 
 sensations are evoked), possibly also for a quick test the warm hand and the 
 cold metal of the percussion hammer, and proceed in the same manner as 
 described above. As a rule, the disturbances thus established will coincide 
 more or less with those of the touch sense, though not exactly at the bound- 
 aries. This is partly due to the fact that, as Goldscheider has recently 
 established on the basis of careful investigations, the skin has special points 
 of pressure, cold and warmth, that is, places (and especially nerve ends), 
 where touch, cold or warmth, may be best felt. But as Oppenkeim pointedly 
 remarks, it would be very difficult clinically to proceed to investigate the 
 
58 
 
 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 surface of the skin, as laid out by Goldscheider according to this scheme, as to 
 areas possessing varying degrees of sensibility, especially as this procedure 
 scarcely ever gives more important results than are obtained by the usual test. 
 In another respect also, I recommend a practical deviation from Gold- 
 scheider' 's proposals, namely, in the test of pain sensations. Granted, that 
 special points of pain and, therefore, pain-nerves too, can not be found in the 
 skin, and that, on the other hand, by increasing almost every stimulation of 
 any sensory quality, the sensation of pain may be evoked, still, clinically, we 
 can not dispense with the concept of pain sensation — first because of the 
 patient's psyche, usually very susceptible to the thought of pain (though, 
 
 N. clunium medii. ~T~ 
 
 • Nn. clunium superiores. 
 
 --» Nn. clunium inferiores. 
 
 Cutan. branches femor. anteriores. 
 
 .__ N. cutaneus femor. lateralis (Plex. 
 lumbal). 
 
 Cutaneus branches N. obturatorii. 
 
 1 N. cutaneus femor. posterior. 
 
 Cutan. branches cruris mediales. 
 
 Branches calcan. mediale (N. 
 tibialis) . 
 Branches calcan. lateralis (N. suralis) . 
 
 N. cutan. suras lateralis (from N. 
 peronaeus). 
 
 N. tibialis. 
 
 N. peronaeus superficialis. 
 
 N. plantaris lateralis. 
 -— (N. tibialis). 
 
 N. plantar mebialis (N. tidialis). —-"■"" 
 Fig. 23. — Distribution of the cutaneous nerves in the lower'extremities. (After Toldt.) 
 
 doubtless loss of the pain sensation is often observed only late), secondly, 
 because frequently in sensibility disturbances of functional and even of 
 organic nature, only these more intensive, pain-producing stimulations show 
 functional changes. It is best to test the pain sensation simply with a sharp 
 needle and by pinching folds of raised skin, or, further, by the electric 
 current, making use of fine electrodes. The needle point is also the principle 
 made use of in the numerously used apparatus for measuring pain (alges- 
 imeters) , in which a sharp needle may be screwed out for a greater or lesser 
 distance from a flat surface upon the instrument; when the needle protrudes 
 only slightly, so as not to pierce the epidermis, the application of the instru- 
 
PAIN SENSE, SENSE OF LOCALITY 59 
 
 merit is felt only as a touch, gradually, with the lengthening of the needle, as 
 pain. All algesimeters may be regarded as useless for clinical service. 
 
 Whether the psycho -galvanic reflex phenomenon described by Veraguth 
 (a galvanometer inserted into the circuit of bodily conduction shows deflec- 
 tions in psychic processes, therefore also in pains, no beat is visible in the 
 stimulation of analgesic zones) will be able to fill the gap, seems for the 
 present, very doubtful; but, not having peisonal experience, I shall, for the 
 time being, refrain from pronouncing judgment. For certain zones of the 
 body there are moreover useful reflex phenomena for judging the pain 
 sensation, as, for instance, dilatation of the pupils which occurs after painful 
 stimulation of the skin of the cheek, which is non-existent when the cheek is 
 analgesic. 
 
 The sensitiveness of the skin to pain is not constant over the entire body, 
 being greatest in the skin of the forehead, least in the region of the buttocks. 
 At every test of the pain sense, one must watch, besides the intensity of the 
 sensation, also the period of latency between the touch and the sensation, 
 since in no other sensory quality does such considerable "retardation" 
 occur (cf. below). The test may, in general (except particular cases, cf. 
 below), in order to spare the patient, be confined to those parts of the body 
 in which one of the previous examinations demonstrated the presence of 
 a disturbance. 
 
 The test of the sense of locality, that is, the keenness in the recognition 
 of the places upon the skin that are touched, may without trouble be com- 
 bined with the testing of the sense of touch, by having the patient, in the 
 more delicate tests, say where he was touched. As a rule, the results do 
 not differ essentially from those obtained by testing the sense of touch. 
 (The test by means of the compass is in a strict sense — cf. above — also a test 
 of the localization sense.) To the examinations of the actual sensibility 
 of the skin there belongs, furthermore, according to some authors, the hair 
 sensibility, that is, the sensitiveness to a slight touch upon the hair alone, not 
 upon the skin beneath it. The areas of most delicate hair sensation are by no 
 means identical with those of the most delicate skin sensation (but the sup- 
 position that the skin underlying the hairy parts of the body is always less 
 sensitive, is not justifiable). The method has not led as yet to practically 
 important results. The examination of far ado-cutaneous sensibility, occasion- 
 ally used in testing the sense of touch, that is, the feeling of tickling (formi- 
 cation) to weak faradic currents, seems to give results identical with those of 
 touch and pain sensation, so that this finely graded and measurable method 
 (determination of the stimulation threshold of sensation by means of the 
 gradation of the current) offers no special advantages. The assertion that 
 certain poisons (bisulphide of carbon) mainly disturb farado-cutaneous 
 sensibility, has not been established. 
 
 Far more important are investigations of the deep sensibility (bones, 
 
60 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 joints, muscles). According to Goldscheider , instead of the expression 
 formerly used " muscular sense" which comprises the main mass of deep 
 sensibility, one should use its separate components: the sensation of active 
 and passive movements, the sensation of weight and resistance (also called 
 strength sensation) and perception of situation. Stereo gnosis (recognition of 
 the form and other external qualities of objects by means of touch) again 
 embraces several of these qualities in another grouping, as well as the most 
 delicate psychic processes (mnemic images) . As an independent sensation, 
 bone sensibility would have to be added to this. All these forms of sensibility 
 may be disturbed individually, and in groups, with and without participation 
 of the skin sensation. 
 
 The sensation of active movements may be tested in the patient only 
 with great difficulty, if it occurs in isolation. When he is told to perform 
 certain movements and then to state the effect of movement, the situation 
 into which the limbs were brought, the sensation of position is virtually tested 
 (cf . below) . Unquestionably, there exist in a sound person sensations of active 
 movement, the loss of which is felt as a disturbance (cf . below) ; but an actual 
 test can scarcely be performed. The test of the sensation for passive move- 
 ments is, on the other hand, very easy. With one hand, the examiner fixes 
 one of the patient's extremities, and with the other, he performs as slight 
 excursions as are possible in small joints in the peripheral parts of the ex- 
 tremities, small movements of the toes, excursions of the fingers, etc. The 
 patient, who naturally must have his eyes closed or bandaged, states the 
 direction of the movement. Tests with considerable excursion in the joints 
 concerned should not be made, because through tension of the skin, tendons, 
 etc., the patient is supplied with other possible sources of knowledge. For 
 the same reason, the test for the large joints (knee, hip) can not be used 
 extensively because of the large areas of skin concerned. The sensation of 
 weight and resistance, the weight sense, may be tested in intelligent patients, 
 by allowing them to raise, with their eyes closed, loads of various weight (with 
 the entire extremity, or with individual parts of it, forearm, finger, etc.), and 
 to tell the difference in weight. As a rule, this sensation is disturbed only in 
 combination with others; moreover, the poor valuation ability of most 
 patients destroys its usefulness, which otherwise, for instance, might easily 
 be shaded more minutely by the dynamometer, upon which one lets the 
 patient exert certain predetermined degrees of pressure. 
 
 The feeling of posture, on the other hand, permits of more exact ex- 
 amination. One places the extremity (or part of it) in a position contrary 
 to that of the natural posture in rest, and has the patient state what the new 
 position is like, about how far it is removed from the position of rest, etc. 
 
 The stereo gnostic sensation may also be very easily tested; since this is 
 but very slightly developed without actual touch, it is best to put the object 
 to be tested directly in the hand of the patient, and to let him describe its 
 
STEROGNOSIS, BONE SENSIBILITY 61 
 
 form, size and substance. For this we choose as varied objects as possible, 
 which are, however, known by their form (coins, keys, dice, balls, fabrics 
 like velvet, silk, etc.). As has been observed above, several kinds of sen- 
 sation are involved in this recognition. 
 
 Dejerine and Egger designated as bone sensation, the sensation of the 
 vibration produced by a vibrating tuning fork when placed upon a part of 
 the body where the bone is covered only by the skin. They consider it an 
 independent sensation, because it may be disturbed without the tactile 
 disturbance of the skin, and vice versa. This fact is probably quite correct, 
 as German authors were able to prove also in the subsequent test (partly, 
 however, it is designated pallaesthesia, and ascribed not only to the bones 
 but also to all other subcutaneous tissue layers); but since this vibration 
 feeling is especially common with simultaneous disturbances of the " muscu- 
 lar sense" (tabes), a particularly far reaching result could scarcely be 
 expected, and has not yet been attained. 
 
 Our knowledge as to the sensibility of the internal organs in the narrower 
 sense, is by no means good. The mucous membranes of the cavities com- 
 municating with the air (mouth, nose, cornea) show the same, though in 
 general somewhat lowered sensibility, as that of the external skin, as we 
 have mentioned before. This is not true of the pleural and peritoneal 
 cavities, the lungs, stomach and intestines, the bladder and kidneys. Among 
 all these formations, the pleura, peritoneum and the mucous membrane 
 of the bladder are those only that are certainly sensitive to pain and touch. 
 The meninges, substance of the brain, lungs, stomach, intestines, on the 
 other hand, are not sensitive to touch, but may very easily be the seat of 
 spontaneous pain sensation, which is probably induced by the sympathetic 
 (L. R. Mutter). But for a test of these functions, we have as yet no sufficient 
 experience. 
 
 Disturbances in sensibility are divided first into subjective — paresthe- 
 sias and pains — and objective; namely, hyperesthesia, hypaesthesia, and 
 anaesthesia. 
 
 Paraesthesia signifies the abnormal sensations of the various qualities 
 which are present only subjectively. They belong most frequently to the 
 realm of the touch sensation, more rarely to the temperature sensations; 
 the abnormal subjective sensations from the group of the "muscular sense" 
 (cf. above) are rare; but frequently a tabetic may feel disturbances in the 
 sensations of position, movement, etc., that can not be objectively demon- 
 strated (he does not know how he holds the foot in walking, etc.; but here, 
 as a rule, paraesthesia and objectively demonstrable disturbances coincide), 
 also in psycho-neuroses, there occur occasionally disturbances of feeling 
 of posture, of stereognostic recognition, etc., in purely subjective form. 
 The most common paraesthesia is tickling (feeling of deadness, of numbness, 
 formication, etc.), which very frequently represents an early symptom of 
 
62 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 severe organic diseases, and moreover is often in so far objectively demon- 
 strable that the patient feels exactly a touch in the paresthetic area, but 
 "differently" than in the sound parts of the body. Disturbances in heat 
 sensation are usually described in very characteristic manner: "I feel as if 
 my legs were packed in ice" (myelitis). A common paresthesia, which 
 is often combined with pains, is the "girdle feeling"; as if a hoop were 
 placed around the body, a cord about the knee (usually corresponding to an 
 affection of the posterior roots). In many paresthesias, their purely 
 psychogenous origin may be recognized at once (for instance a feeling of a 
 living animal in the stomach, globus). 1 
 
 Pains represent the most difficult subject matter of neurologic (often, 
 too, of internal) diagnostics. Only in extremely rare cases can the spon- 
 taneous pain be recognized by the examiner (occasionally through vaso 
 motor symptoms, reddening of the face, pallor, fluctuations in blood pressure, 
 local coolness or warmth) — most often in pains evoked through the sympa- 
 thetic paths (vascular crises, etc.), in which we may find conditions of 
 collapse, superinduced by the pain, which, however, occur also in purely 
 "sensory" pains. Unfortunately, we have no means for measuring the 
 spontaneous pain (cf. above). In every case, we let the patient describe 
 his pains as accurately as possible; their localization (following nerve 
 trunks, diffuse, corresponding to an internal organ — cf. below — confined 
 to one vascular realm), their character ("nagging," "boring," "pressing," 
 the lightning-like "lancinating pains," "colic-like" pains); their duration 
 may often be of great significance. 
 
 Of the objective disturbances in sensibility, the hyperesthesias are com- 
 paratively the rarest. Tactile hyperesthesia, the feeling of an unpleasant 
 sensation of pain upon the slightest touch, is found occasionally in neu- 
 ralgias, but also in pure neuroses and in diseases of the internal organs in 
 the so-called "zones of Head" (cf. below). Hyperesthesia to cold and 
 (far more rarely) to heat is, though not a common, still a characteristic 
 symptom of organic diseases (tabes). Hyperesthesia to pain or hyper- 
 algesia, that is, feeling of a violent pain upon a touch that usually causes 
 but a disagreeable sensation or a slight pain, is, on the other hand, found 
 rather frequently in all sorts of organic (pressure points in neuralgias of 
 this kind) and psychogenous (pvaralgia in hysteria) diseases. 
 
 The group of hypcesthesias or anaesthesias, the partial or total loss of 
 the various qualities of sensation (the single forms are known also as thermo- 
 anesthesia, hypalgesia, asterognosia, pallanesthesia), is the largest. This 
 is the point in which the test of the separate sensory qualities has the most 
 valuable results. We must first, as has been stated above, consider the 
 distribution of the zones of such sensory disturbance (Fig. 24). Here we 
 
 1 On the other hand, the parses thesias of the sense nerves — entoptic and entotic phenomena — 
 are also frequently found in non-psychogenous diseases. 
 
TYPES OF AXMSTHESIA 
 
 63 
 
 find four main forms of distribution of these disturbances: types of total 
 break in conduction in the central organ (sensory hemiplegia, paraplegia — 
 occurring from organic, as well as from psychic causes) ; disturbances in the 
 area subserving the individual segments and roots of the spinal cord (cf . the 
 segment schemata in the special pathology of the spinal cord) ; disturbances 
 in the distribution area of the peripheral sensory nerves, for which the sche- 
 mata subjoined will serve as points of reference; finally disturbances of psy- 
 chogenous kinds ("central type"), which are restricted to none of the forms 
 described (glove, sleeve-stocking-like anaesthesias, usually in hysteria). 
 
 Fig. 24. — The three types of disturbances in sensibility at the extensor side of the right upper 
 extremity. (After Obersteiner and Redlich.) A. Peripheral type. 1, X. supraclavicularis- 2 N. 
 axillaris; 3, X. cutan. post. sup. radialis; 4, lateral branches n. spinal; 5, X. cutan. medial radialis; 
 6, X. cutan. post. inf. radialis; 7, X. cutan. med.; 8, X. cutan. lateralis; 9, X. radialis; 10, X. 
 ulnaris; n, X. medianus. (After Bernhardt.) B. Segmental type, 4, 5, 6, 7, 8. 4-8, Cervical 
 segment; I, 1. Dorsal segment. (After Allen Starr .) C. Central type. 
 
 To which of the groups mentioned, the hypaesthesia or anaesthesia under 
 consideration belongs — a question often decisive for diagnosis — is a matter 
 that can be settled only by determining accurately the boundaries of the 
 disturbance. Inasmuch as an anaesthetic area but rarely touches directly 
 one that has normal sensibility, this determination demands much patience 
 and skill. 
 
 We must also mention here, that Head succeeded in finding for diseases 
 of the internal organs (lungs, stomach, intestines, etc.), cutaneous nerve 
 areas, which synchronously display abnormal forms of sensibility, according 
 
64 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 to what has been determined so far, almost exclusively hyperesthesias 
 ("reflex hyperesthesias " best known in gastric affections, as band-like or 
 singularly bounded zones of half the abdomen, of the back, etc.). We can 
 not here enter upon a detailed discussion of this subject. 
 
 As has already been said, each quality of sensibility may be individually 
 disordered, or they may all be affected at the same time. We find, rather 
 frequently, also, an affection by groups; only the cutaneous, or only the deep 
 sensibility. The most common of these forms of a partial or "dissociated 
 sensibility disturbance'''' is probably that of simultaneous disturbance of the 
 pain sensation and of the cold and heat sensation, while the tactile sensibility 
 remains normal, as in syringomyelia. 
 
 Some rather rare disturbances shall be briefly mentioned. The pain 
 sensation is not rarely disturbed in such a way, that the pain stimulation is 
 felt only late, after a period of latency of one or more seconds, that is, is felt 
 as pain; as a rule, the touch is at once registered as such, and only the pain 
 appears later (delay or retardation of the pain sensation; double sensation) . 
 In other cases, a single, brief, though intense pain stimulation (prick) 
 produces no pain; on the other hand, pain is felt, if one allows it to persist 
 for some time with the same degree of severity {summation). Disturbances 
 in the temperature sense are most frequently the foundation of "perverse 
 sensations" which are as such, rare; warm is felt as cold, cold as touch or 
 pain, etc. Allocheiria, the localization of touch upon the corresponding 
 place in the extremity not touched, is, at any rate, as rare an occurrence as 
 polyasthesia, in which a single touch is felt as if multiple. 
 
 The characteristic disturbance of deep sensibility, frequently met in central 
 organic nervous diseases, affects most frequently the sense of position and the 
 stereognostic sensation. The patient in bed "no longer knows where his 
 legs are, whether he has any or not"; he no longer recognizes objects placed 
 in his hands. Disturbances in the sensation of vibration are decidedly less 
 common. 
 
 E. Reflexes 
 
 We distinguish tendon reflexes, cutaneous (and mucous membrane) 
 reflexes and internal reflexes. 
 
 Without entering with more detail into the concept of "reflex" we shall 
 mention here only that we shall designate as "reflex" in the sense of the 
 phenomena now under consideration, only muscular twitchings, that' is, 
 contractions, which follow a sensory stimulation, with the exception of the 
 idiomuscular contractions (cf. above). All these reflexes, then, have a 
 local motor effect. We regard in general, also, the tendon phenomena 
 discovered by Erb and Westphal, as genuine reflexes, not (as Westphal held) 
 as direct stimulation of the muscle concerned. 
 
PERIOSTEAL AND TENDON REFLEXES 65 
 
 There are an immense number of tendon reflexes in the human body, 
 many of which can be proved present, even in the normally healthy individ- 
 ual, though not all constantly, and some only upon increase of the reflex. 
 We shall mention here only the most important, the knowledge of which 
 quite suffices for the recognition of all nervous diseases involved. 
 
 In the head we know only the lower jaw or masseter reflex. It is most 
 easily evoked as follows : When the mouth of the patient has been slightly 
 opened the examiner places a finger upon his lower jaw and with the percus- 
 sion hammer, gives the finger a light blow from above; there results a raising 
 of the lower jaw, effected mainly by the masseters, the mouth closing. The 
 same reflex may be evoked by the periosteum of the lower jaw near the point 
 of insertion of the masseters; this reflex is virtually one of the so-called perios- 
 teal reflexes, which, however, genetically and nosologically, may be counted 
 among the tendon reflexes. The lower jaw reflex is comparatively constant 
 in the healthy. 
 
 The somewhat less constant forearm reflexes are also genuine periosteal 
 reflexes and may be evoked by tapping the lower end of the radius (radial 
 periosteal reflex) and the ulna (ulnar periosteal reflex) which causes, in the 
 (more common) radial reflex, a reflex contraction of the supinator, that is, a 
 flexion of the forearm, in the ulnar reflex, usually a slight retardation. 
 Both reflexes are tested with the forearm flexed into a right angle, ad- 
 ducted and slightly pronated, in order to relax the m. supinator as far as 
 possible. 
 
 The triceps reflex is scarcely ever lacking in the healthy, but is often diffi- 
 cult to test. The arm of the patient is held somewhat in the same flexed 
 position as mentioned before, and a short, quick blow with the percussion 
 hammer, delivered upon the tendon of the m. triceps, right above the ole- 
 cranon. There results a movement of extension, often only slight, in the 
 forearm. The danger consists in the possibility of striking the muscle itself 
 instead of the tendon, thus producing direct muscular stimulation, which, as 
 a rule, has a slighter motor effect, but may otherwise closely resemble the 
 reflex. Since the triceps reflex — a genuine tendon reflex — may be of diag- 
 nostic importance (especially its absence in tabes, etc.), this point must not 
 be overlooked. 
 
 The tendon reflexes in the trunk are of no great importance. We must 
 mention the scapulo-humeral reflex described by Bechterew, a periosteal reflex 
 produced by tapping the scapula, which, however, I have not found suffi- 
 ciently constant to attach great weight to its absence. 
 
 The most important tendon reflexes belong to the lower extremities. 
 Important above all is the patellar (kneecap) reflex, the contraction of the. 
 m. quadriceps upon tapping the patellar tendon below the patella. It is the 
 most constant of all reflexes in the healthy (lacking only in 0.04 % of the 
 cases) and can be tested with comparative ease. For its test, still more than 
 5 
 
66 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 for that of all other reflexes the principle that to evoke the reflex the muscle 
 concerned — here the quadriceps — must be totally relaxed, holds good. 
 
 Passive relaxation is usually attained by a certain position of the leg; but 
 often even in intelligent, especially in timorous, patients an active relaxation 
 is accomplished with the greatest difficulty. We recommend the following 
 ways and means : 
 
 i. The patient sits on a chair, crosses the leg to be tested over the other; 
 the examiner delivers a horizontal blow with the inner edge of his hand or 
 with the percussion hammer upon the quadriceps tendon below the patella, 
 where it bridges over the space between the patella and tuberositas tibiae and 
 lies therefore in a more or less hollow space (this "hollow space" is probably 
 the reason for its remarkable constancy) . This method is not safe because 
 total relaxation is rarely attained ; it has the advantage of the largest motor 
 effect (of the sudden lifting of the tip of the foot) which, however, is of com- 
 paratively less importance because in excitation of the patellar reflex one 
 should always pay more attention to the contracting muscle than to the motor 
 effect; in decreased reflexes, it often happens that the former only is visible. 
 
 2. The patient sits on a chair which is to be several centimeters lower 
 than the leg proper of the patient and places the foot of the leg to be 
 tested so far forward that leg and thigh form an angle of about ioo de- 
 grees, and the thigh rests easily upon the chair. A great many patients 
 are best able to relax the muscle when in this position; the motor effect is 
 slight, but the contraction of the muscle is usually very distinct. I recom- 
 mend this method, as well as the following : 
 
 3. The patient lies in bed, as flat as possible, with the upper part of his 
 body but slightly raised; the leg is somewhat flexed so that leg and thigh form 
 an angle of about 140 degrees and at the same time the thigh is abducted 
 (the knee of the flexed leg inclines to the outside). The blow on the 
 patellar tendon usually evokes an evident extension of the leg proper. The 
 relaxation of the quadriceps usually succeeds, though not always easily. 
 Crossing of the legs in a recumbent position is not so good. 
 
 4. The leg proper of the recumbent patient is raised by means of a 
 handkerchief or some similar device placed beneath it, so that the leg lies 
 horizontally in its support and the thigh is flexed into obtuse angles with it 
 and the trunk. Here, it is supposed, the relaxation is very nearly complete. 
 Almost every year, however, new modifications are recommended for testing 
 this reflex, that is, for relaxing the muscle. 
 
 I should call the Achilles tendon reflex, considering its work and signifi- 
 cance, the second of the most important tendon reflexes of the body. It con- 
 sists in a contraction of the calf-muscles from which the tendon of Achilles 
 courses, of the triceps suras, and thereby evokes plantar flexion of the foot. 
 It is elicited by striking the tendon of Achilles with the percussion hammer; 
 here, too, the relaxation of the calf is the main thing. For this, the position 
 
DISORDERS IN TENDON REFLEXES 67 
 
 described above under 3 usually suffices; the examiner then stands at the foot 
 of the bed and from the inner side of the leg delivers a brief blow upon the 
 tendon. Under certain circumstances one must test the tendon at several 
 places. The absence of this reflex, as that of the patellar reflex is, in every 
 case, pathological (unless contractures, etc., prevent their appearance). 
 Other methods for testing it are the striking of the tendon while the patient 
 kneels on a chair, or by striking the tendon while the patient lies on his 
 stomach with his leg flexed at the knee. 
 
 All other tendon reflexes are inconstant, that is, demonstrable only in 
 pathological increase of the reflex, even the dorsal foot reflex described by 
 Bechterew and Mendel (which with Spier I would count as a genuine tendon 
 phenomenon.) 
 
 We know two kinds of pathological changes in the tendon reflexes: 
 increase and decrease, or absence. The former condition is accompanied by 
 increase of the muscular tonus (cf. above), being its most regular concomitant 
 phenomenon, the latter with decrease of the tonus (but here the connection is 
 less constant). 
 
 We can say with certainty when a reflex is to be called increased only in 
 the case of the patellar and the Achilles tendon reflexes. Increase in the reflex, 
 as a rule, effects a more extensive muscular contraction with considerable 
 motor effect and also the appearance of tendon reflexes that are not demon- 
 strable at other times. In spastic conditions of high degree (i. e., naturally 
 only so long as the spastic musculature is not so tightly contracted as to make 
 further contraction impossible) by striking almost any tendon in the body 
 that is to some extent exposed, one may produce twitchings in the muscle 
 concerned; among the most common in this case are twitchings in the adduc- 
 tor tendons of the thigh (adductor reflex) and in the tibialis posticus. 
 
 Increase in the patellar reflex is present if the reflex can be evoked not only 
 at the tendon above and below the patella and from the patella itself, but 
 also by striking the periosteum of the tibia in a more or less extended area. 
 In some cases where the increased patellar reflex is evoked, the quadriceps 
 of the other leg contracts also (crossed reflex) . The highest degree of increase 
 is called patellar clonus; here the quadriceps is affected by more or less rapid 
 contractions, which last for some seconds, when, after the muscle has been 
 relaxed as far as possible, one seizes the patella firmly with two fingers and 
 moves it suddenly towards the tibia. 
 
 Foot-clonus (ankle clonus) as an expression of the increase of the Achilles 
 tendon reflex can be more constantly evoked. When the legs are totally 
 relaxed (position as above under 3) the examiner with his right hand placed 
 under the sole of the patient's foot gives a sudden jerk upward towards the 
 sole of the foot, while simultaneously the left hand protects and slightly 
 fixates the leg proper at the knee joint. The calf shows slow or more rapid 
 clonic contractions accompanied every time by twitching movement of the 
 
68 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 foot downwards. It is important here that the pressure of the hand upon the 
 sole of the foot must be relaxed, as soon as the blow at the sole of the foot 
 has been delivered; the hand then remains but lightly resting against it (but 
 may not let the sole go) . As a rule, 10 to 20 such twitchings appear, decreasing 
 gradually; in severe cases, however, the foot-clonus may persist for minutes. 
 From this must be differentiated the pseudodonus of hysterics, in which 
 either the calf comes more nearly into a state of tremor or is even actively 
 contracted, or some— about 3 to 4— clonic twitchings appear as expression 
 of a slight "reflex hyperesthesia. " Hand-clonus, clonic flexion movements 
 in the flexors of the hand and fingers, which may be evoked as the expression 
 of a high degree of increase in the reflexes in the upper extremities by a 
 sudden jerk against the relaxed metacarpus, from below upwards, with 
 fixated forearm, is rarer. 
 
 Increase of the tendon reflexes is found, as has been stated, almost con- 
 stantly in increase of the muscular tonus (cf. this) therefore in all so-called 
 spastic diseases (the highest degrees of it in diseases of the pyramidal tracts), 
 besides this, also in neurasthenia and hysteria; but here, as a rule, the 
 criterion of a genuine "clonus" is lacking. 
 
 The decrease of the tendon reflexes is difficult to judge. We may say that 
 we can speak certainly of a decrease in a (otherwise constant) tendon reflex 
 when it is evoked but feebly even with the assistance of Jendrassik's manoeu- 
 vre (reinforcement method), or also, when there is an obvious difference be- 
 tween the similar reflexes of each half of the body and the stronger reflex 
 must be regarded as normal, not increased. By Jendrassik's reinforce- 
 ment method we designate the strong active tension on the part of the patient 
 of a muscular area, during the test of the tendon reflexes of another area. 
 Usually the patellar reflex is concerned. If presence of the reflex seems 
 questionable one has the patient exert tension upon the musculature of his 
 arms, by pulling apart with all his might his hands tightly interlocked, 
 without letting them go (or by having the patient heartily grip his own hand). 
 At this moment the exciting blow upon the tendon is delivered. Whether in 
 this process it is a matter of "paving the way" for the reflex, and not of 
 merely distracting the attention of the patient and thereby securing better 
 relaxation of the muscle, is not yet known. 
 
 If even with this help the reflex can not be evoked, we describe it as 
 absent. The absence of the patellar reflex (and almost as certainly that of 
 the Achilles tendon and triceps reflex) are found in diseases with lowering of 
 the muscular tonus (tabes), in neuritis, in narcosis and coma, in muscular 
 atrophies of high degree, in rare cases also, apparently congenital. Oc- 
 casionally there occurs " exhaustion " of the tendon reflexes; after long 
 testing they become weaker and more difficult to evoke. 
 
 Among the cutaneous reflexes only three are of great importance; the plan- 
 tar, the cremasteric, and the .abdominal. 
 
CUTANEOUS REFLEXES 69 
 
 We designate by plantar reflex a movement of the toes, which occasionally 
 extends to the metatarsus, upon irritation of the skin of the sole of the foot 
 (done best with the handle of the percussion hammer, also with pieces of ice, 
 by pinpricks, etc.). The stimulation should be performed quickly and 
 energetically; if the patients are anxious it is best to fixate the leg proper. 
 Sometimes the reflex of certain parts of the planta pedis can be evoked with 
 special ease (usually this is done best from the inside edge). 
 
 The normal plantar reflex consists in an energetic plantar flexion of all 
 the toes, occasionally with simultaneous dorsal flexion of the metatarsus, or 
 of the entire foot (flight reflex) . 
 
 The reflex is not absolutely constant in the healthy. It is absent in 
 every severe hypsesthesia of the planta, also in severe cornification, edema, 
 cold; furthermore in sleep and in narcosis — besides in some organic diseases, 
 neuritis, cerebral and spinal paralyses. Its mere absence — -as well as its 
 increase — is but rarely of great diagnostic importance. 
 
 Under certain conditions we find, however, instead of the normal plantar 
 reflex, an abnormal reflex named after Babinski: the so-called Babinski 
 toe reflex. Here there appears upon irritation of the skin of the planta a 
 slow dorsal flexion of the large toe, occasionally, too, of the other toes and 
 the middle part of the foot instead of the normal, rapid plantar flexion. 
 Here we must emphasize the fact, that one uses at first but weak stimuli, 
 slight strokes of the percussion hammer, etc., and stimulates various parts 
 of the planta, one after the other. If this is done too energetically, there 
 is often a rapid withdrawal of the foot and an undecided wavering of the 
 toes, composed of extension and flexion, which can be interpreted only as 
 flight reflex. The Babinski phenomenon is found normally only in suck- 
 lings. In later life it may be regarded as a pathognomonic sign of disease 
 of the pyramidal tracts. As to its origin, whether it is only a modification 
 of the normal plantar reflex or is an independent reflex, opinions are still 
 divided; it is probably a matter of a purely spinal reflex, which can appear 
 only after suppression of the normal plantar reflex which goes through the 
 cortex of the brain. 
 
 Oppenheim's phenomenon may be described as to a certain degree 
 analogous to the Babinski phenomenon. Upon energetic stroking of the 
 skin of the leg at the inner edge of the tibia, there is, in the healthy person, 
 no reflex movement of plantar flexion of the toes ; in spastic conditions, on 
 the other hand, there appears a dorsal flexion of the foot and the toes. I 
 found the phenomenon less constant than the Babinski, with which it 
 generally appears, and its excitation frequently somewhat irritating to the 
 patient. In the same group belongs also Remak's femoral reflex: upon 
 stroking the inner side of the skin of the thigh, there results reflex raising 
 of the entire leg and dorsal flexion of the foot — usually only in pyramidal 
 tract lesions of the spinal cord. Probably in all these "pyramidal tract" 
 
7 o GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 reflexes, a synergism of certain groups of muscles (extensors of the foot and 
 toes, flexors of the leg proper, ileopsoas) appearing only in pathological 
 cases, plays the main role; it may be tested in another way, too {StrumpeW s 
 tibialis phenomenon) : the patient is asked to draw his thigh energetically 
 up to his trunk while the examiner exerts strong resisting pressure upon the 
 thigh; in certain diseases (according to Strumpell especially in multiple 
 sclerosis) the synergism mentioned appears especially in an energetic 
 contraction of the m. tibialis anticus. 
 
 Furthermore there occur occasionally, also in mere increase of the usual 
 plantar reflex, co-movements in the musculature of the thigh of the same, 
 sometimes, too, of the other extremity and even plantar reflex on the side 
 that has not been stimulated. 
 
 The cremasteric reflex consists in a prompt contraction of the cremaster 
 muscle, that is, an elevation of the testicle on the side in question, occasionally 
 too on the opposite side, upon stroking (prickj cold) the inner aspect of 
 the thigh near the scrotum. The reflex which in the female is replaced by 
 the analogous inguinal reflex and is rather constant, is important only when 
 there is some difference between the two sides and in total absence, especially 
 in transverse diseases of the spinal cord, in which the knowledge of the loca- 
 tion of a reflex arc is of importance for exact local diagnosis. 
 
 The same is true of the abdominal reflexes. We know of three such on 
 each side — the upper or epigastric, the middle and the lower (hypogastric) 
 reflex. They are evoked by transverse stroking of the upper, middle, or 
 lower third of the skin of the abdomen and consist in a sudden contraction 
 of the obliqui and the transversus abd. In most cases they can be evoked 
 separately; but frequently they are altogether lacking when the abdominal 
 walls are very flaccid or very thick (fatty, edematous). Their absence, 
 moreover, may be considered an early symptom of multiple sclerosis. The 
 absence of separate abdominal reflexes may attain great importance for 
 localization in myelitic processes. 
 
 We must mention briefly the almost constant anal reflex (contraction 
 of the sphincter ext. upon pricking or a similar irritation of the skin of 
 the anus) and the scrotal reflex, the peculiar slow contraction of the tunica 
 dartos of the scrotum, which upon stroking or refrigeration (uncover- 
 ing) passes in curious waves over the scrotum. The latter is remarkable 
 because at least one of these reflex paths might pass through sympathetic 
 fibres. These two reflexes have not yet attained any clinical importance 
 worth mentioning. 
 
 In respect to the mucous membrane reflexes we can be very brief. Only 
 the corneal or eyelid and the vomiting or retching reflex are important. In the 
 eyelid reflex there appears upon touching the conjunctiva or cornea immediate 
 contraction of the orbicularis (to be distinguished from the reflex closure of 
 ,the orbicularis upon touching the lashes, which is probably to be considered 
 
INTERNAL REFLEXES 71 
 
 a cutaneous reflex), and the eye closes. The vomiting reflex consists of a 
 retching movement produced reflexly by touching the posterior wall of the 
 pharynx. Both reflexes are occasionally found to be changed (lacking) in 
 organic processes in the brain and the medulla oblongata. But besides 
 this, they are frequently lacking in psychogenous affections (hysteria); but 
 since they are not constant even in the normal individual and (especially 
 the vomiting reflex) can be suppressed to a great extent by will-power, the 
 title of hysterical stigma has been unjustly assigned to them. 
 
 Of the internal reflexes we have already discussed one, the pupillary. 
 But we must mention here, furthermore, the reflex processes subserving the 
 evacuation of feces and urine as well as those of the sexual sphere. It is true 
 that we have no real test for these reflexes; but the pertinent statements of 
 the patients and the clinical observation of the physiological course of these 
 processes permit of a rather accurate representation and the leading 
 sphincter disturbances ought not to be neglected in any general nerve ex- 
 amination. 
 
 Evacuation of urine and feces is indeed subserved by both active and 
 reflex processes. The strong desire to evacuate, which occasionally even in 
 the normal individual overcomes the resistances of the sphincters, is certainly 
 for the most part reflex, caused by the degree of fulness in the intestines or 
 the bladder or by the manner of filling (irritation of the walls by pathologically 
 changed contents). The disturbances of these functions are called incon- 
 tinentia alvi (urinae), that is, the inability to hold back the contents, and 
 retentio urinae (retentio alvi), the impossibility of active evacuation. Natu- 
 rally these names are also used promiscuously for the disturbances of the 
 active powers concerned for voluntary evacuation. Still we may regard the 
 retention phenomena, such as are found in cerebral and high lesions of the 
 spinal cord, as well as incontinence in destructions in the lower segments of 
 the spinal cord, with some justification, as a disturbance of the reflex of 
 evacuation of urine and feces. 
 
 The reflexes of the sexual sphere are only of slight importance for the diag- 
 nosis of organic diseases, with the exception possibly of the phenomenon of 
 impotence (cceundi) in tabes and possibly as a rare symptom of the phenom- 
 ena of functional loss in diseases of the lowest segments of the spinal cord, 
 analogous with the animal experiments of L. R. Mutter (loss of ejaculation in 
 disturbances of the sacral portion of the cord). All the greater is the im- 
 portance of the numerous psychogenous disturbances which belong to this 
 realm, such as psychic impotence, priapism, too frequent pollutions, etc. 
 Especially in the various forms of neurasthenia (cf. in the special part) do 
 we meet those reflex disturbances, to which strictly speaking, many dis- 
 turbances in menstruation also belong. It is common to all internal reflexes, 
 with the exception of the pupillary alone, that the paths of the sympathetic 
 nervous system play an important role in producing them. 
 
72 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 F. Vasomotor, Trophic, and Secretory Disturbances 
 
 To this chapter belong the least investigated disturbances of the entire 
 neurological field. This is true especially of vasomotor phenomena of the 
 origin of which we know only that they possess centers in the medulla oblon- 
 gata and their paths for the most part or altogether are the sympathetic 
 tracts; disturbances of this kind are found in neuroses (partly independent) 
 and in diseases of the cervical sympathetic, in the latter case, in a definite 
 arrangement. As concomitant symptoms of organic diseases they are rarer. 
 
 Abnormal redness or pallor of the skin, abnormal warmth or cold are 
 naturally to be considered disturbances only when they follow inadequate 
 stimuli. Occasionally such a condition of abnormal redness, pallor, coolness, 
 persists for days and weeks, apparently caused by a paralysis or a spasm of 
 the vaso-constrictors. The most typical form of such local vascular dis- 
 turbances is Raynaud's disease (symmetrical gangrene), the incipient 
 stages of which are mostly characterized by pallor and coldness of the end 
 phalanges which later passes into gangrene. Similar conditions of tempo- 
 rary nature play the main part in intermittent claudication (dysbasia or 
 claudicatio intermittens arteriosclerotica), where through vascular spasm, 
 pallor and coldness of the skin, pains and disturbances in movement appear. 
 From this one must differentiate the akinesia algera (Mobius), general 
 motility disturbance upon a foundation of psychogenous pains. Also in 
 abnormal redness due to vascular paralysis (or dilator spasm) pains may 
 appear in rare cases (erythromelalgia). Occasionally these vascular dis- 
 turbances are unilateral, for instance, in the face in disease of the cervical 
 sympathetic. 
 
 Dermographism (urticaria factitia) is to be understood as abnormal 
 irritability of the vasomotors, as it is found in some neuroses and especially 
 in meningitis; upon rapid stroking of the skin (finger nail, percussion ham- 
 mer) there appears upon the spot stroked a temporary reddening, then an 
 anaemic white swelling which persists for minutes and even hours, possibly 
 caused by exudation into the tissue. 
 
 These serous exudations often appear in the form of local or general 
 edemas which are distinguished from those in renal disease only by the 
 normal findings in the circulatory apparatus, as well as by their transitory 
 nature; in older cases also by their great obstinacy. There has been de- 
 scribed as intermittent edema an independent "vasomotor" neurosis in which 
 a local edema appears now here, now there, in the skin or mucous mem- 
 branes, lasting some hours or even days. The joints also may be affected 
 by similar disturbances (hydrops articular, intermittens). In hysterics we 
 distinguish the so-called blue edema (with vascular congestion) and white 
 edema (with anaemia) which mostly appear locally, unilaterally, and may be 
 very obstinate. 
 
TROPHIC DISTURBANCES 73 
 
 The trophic disturbances appear in more striking form than the vasomotor. 
 Besides the muscular atrophy already discussed, the skin and skeleton fre- 
 quently exhibit trophic disturbances dependent upon nervous diseases. 
 Some form a borderland between neurology and dermatology. Among 
 these I reckon scleroderma, in which circumscribed or diffuse parts of the 
 skin (especially forehead, nose, fingers) through loss of elasticity and shrink- 
 ing of the skin become smooth, glossy, usually brownish in color; the skin 
 can no longer be raised from the parts beneath and considerable restrictions 
 in movement result. Furthermore, herpes zoster, a blister-like eruption 
 upon certain segments of the skin, accompanied with neuralgiform pains, 
 ichthyosis, usually a congenital disease characterized by abnormal dryness 
 and desquamation of the skin, the cutaneous atrophy known as "glossy 
 skin," in spinal cord and nerve trunk disease, myxedema, in which 
 besides a diffuse swelling and pallor of the skin (though upon pitting 
 the depressions do not persist), trophic disturbances in the nails, hair, etc., 
 may also appear. Trophic disturbances of the hair are by no means in- 
 frequent; loss of hair in spots (alopecia) and sudden diffuse or circumscribed 
 canities are the main types, both of which frequently appear after or because 
 of nervous diseases. 
 
 The most severe forms of atrophic skin affections are the ulcers, among 
 which we cannot, of course, include the artefacts of hysterics who occasion- 
 ally produce ulceration themselves. But there does belong here the mat 
 perforant of syringomyelia and tabes, the spontaneous occurrence of deep, 
 sharp-edged ulcers, especially on the planta pedis, and, furthermore, the 
 already mentioned Raynaud's disease in its last stages. In a certain sense 
 we must include here also the extensive decubital sores (due to pressure upon 
 anaesthetic places) of myelitics and the mutilation of the phalanges in the 
 so-called "Morvan's type" of syringomyelia. 
 
 Trophic disturbances in the bones and joints are seen in the form of 
 atrophies as well as of hypertrophies. The former appear especially in 
 spinal diseases accompanied by muscular atrophy, as in infantile spinal 
 paralysis, where usually the bone of the extremity affected remains behind 
 in growth as the musculature disappears; hypertrophies form the charac- 
 teristic of acromegaly, where the thickening of the bones (especially in the 
 forehead, chin, fingers and toes) besides the thickening of the other tissues 
 is the substratum of the disproportionate condition of the "acra," the 
 ends of the extremities. Destruction of bones is found in spontaneous 
 fractures and especially in arthropathies as in severe spinal diseases (tabes, 
 syringomyelia). Here there appear painless inflammations of the joints 
 accompanied by hydrops and pronounced thickening, later destruction of 
 the capsule and the articular ends of the bones, which in their turn may 
 lead to fractures, luxations and subluxations (Fig. 25). 
 
 The secretory disturbances form the smallest group of this division. If 
 
74 
 
 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 we do not include among them the above described disturbances in evacua- 
 tion of the feces and urine, only abnormal conditions in secretion of sweat 
 and saliva are concerned. We observe occasionally an abnormal dryness 
 of the mouth in tabetics, abnormal increase in saliva (salivation, ptyalism) 
 in epileptics, affections of the sympathetic, etc. The sweat secretion may 
 
 Fig. 25, 
 
 -Arthropathy and hypotonia (genu recurvatum in tabes dorsalis). 
 Krieger, Klinischer Atlas der Nervenkrankheiten.) 
 
 {After Schoenborn- 
 
 occasionally be present in the form of a defect {anhidrosis) in the above-men- 
 tioned cutaneous affections (ichthyosis), and may occur also in some forms of 
 neuritis and in spinal diseases. More common is hyperhidrosis in all possi- 
 ble organic (syringomyelia, neuritis) and especially in "functional" nervous 
 diseases (neurasthenia, Basedow's disease); frequently it is unilateral, 
 corresponding to the localization of the process and not rarely very easily 
 influenced by the psyche. 
 
EXAMINATION OF THE CEREBROSPINAL FLUID 75 
 
 Of all the above-mentioned disturbances of vascular innervation and 
 of the trophic centers we can control the occurrence only of a very few. For 
 one single group of these symptoms there are more favorable conditions, 
 i.e., disease of the cervical sympathetic. We know clinically, in addition to 
 the experimental results of stimulation and total division of the cervical 
 sympathetic, that in paralysis of the cervical sympathetic, contraction of the 
 palpebral fissure and the pupil on the same side, occasionally, too, constric- 
 tion of the cutaneous vessels and anhidrosis on the side concerned may 
 appear. Frankly, the findings are not altogether constant, but the opposite 
 symptoms which would naturally be expected from irritation of the cervical 
 sympathetic are still less constant. 
 
 G. Examination of the Cerebro-spinal Fluid 
 
 This examination, especially since the increase in epidemic meningitis, 
 should be familiar to the practising physician and to the neurologist. It is 
 connected with the performance of lumbar puncture, the inventor of which 
 was Quincke. 
 
 For lumbar puncture we need as instruments a sharp-pointed canula, 
 10 cm. in length, of a diameter at most of 1 mm. and a suction tube con- 
 nected with it by means of a rubber attachment. During its performance 
 the patient lies on his side or sits on a chair, in each case with spinal column 
 flexed far forward. The canula supplied with its mandril is inserted, with 
 all antiseptic precautions, between the 2 and 3, 3 and 4, or 4 and 5 lumbar 
 vertebrae, about 1/2 cm. laterally from the median line and in direction 
 inward and upward (in children in the median line sagitally upward). 
 After piercing the muscles, the instrument meets in the ligaments before 
 and in the foramen intervertebrale a slight, then in the dura, a still slighter 
 hindrance. Now the point of the canula is in the arachnoidal sac which is 
 filled with liquor; the mandril is withdrawn and the liquor drips or spurts 
 from the canula. Now the suction tube and the rubber attachment may be 
 connected and the pressure of the fluid be measured at once, the pres- 
 sure normally amounting to 40 to 130 mm. of water and pathologically may 
 rise to several hundred millimeters. The experienced may judge the pres- 
 sure as normal, decreased, or increased from the manner in which the liquor 
 leaves the canula, whether it be a rapid or slow dripping, or a sudden spurt- 
 ing. In this case we take for diagnosis only 4 to 6 c. cm., though for thera- 
 peutic reasons, it is true, often far larger quantities (up to 40 to 60 c. cm. at 
 one sitting, especially in various forms of meningitis). Then the needle is 
 rapidly withdrawn, the small wound closed with adhesive plaster. The 
 operation (unless, from the restlessness of the patient, the needle swerves 
 into the periosteum) is almost painless. On the other hand, there appear 
 frequently, especially in patients with normal fluid, a few hours after the 
 
76 GENERAL DIAGNOSTICS OF NERVOUS DISEASES 
 
 puncture, pains in the nuchal region and the head, which may be very 
 violent and last for several days; they never have serious consequences and 
 are easily overcome if the patient will lie quietly on his back. Only in 
 tumors of the posterior cranial cavity are there occasionally after the punc- 
 ture sudden displacements within the skull due to change in the pressure 
 (closure of the aqueduct) and now and then such a patient dies; in tumors 
 of this region, therefore, puncture should not be performed. 
 
 The fluid in its normal condition is as clear as water and contains only 
 minimal traces of albumen and very few cells. 
 
 Pathological changes may affect the pressure, the chemical and 
 microscopic condition of the cerebro-spinal fluid. 
 
 The pressure is rarely diminished, on the other hand, often increased 
 (in hydrocephalus, tumors and often in meningitis) as has been stated above. 
 
 Concerning its chemical condition, the albuminous content is of special 
 importance. Increase in the quantity of albumen (apparently, in particular 
 of the serumglobulin) is found in purulent or hemorrhagic processes of the 
 meninges and of the surface of the brain and the spinal cord, furthermore 
 in tubercular meningitis and above all in progressive paralysis of the insane, 
 where it may prove to be an important differential diagnostic factor; pure 
 globulin increase seems to occur besides these also in other metasyphilitic 
 diseases of the cerebro-spinal system. 
 
 The microscopy of the fluid concerns its cellular content {cytology) and 
 the micro-organisms it contains. We speak of an increase of contents, 
 especially of small uninuclear cells ("lymphocytes") if the number of these 
 formations in the visual field, in an enlargement of 400, amounts to more 
 than 4 to 5 in the average. This holds good especially for all metasyphilitic 
 diseases (gummatous processes, tabes, progressive paralysis of the insane) 
 and for meningitis of all kinds. Occasionally, too, one finds large quantities 
 of polynuclear cells (fresh meningitis and progressive paralysis). For the 
 purpose of this examination, a drop of the strongly centrifuged fluid is taken 
 from the bottom of the centrifugation glass, carefully dried on the object 
 slide, eventually fixed and stained with methylene blue or May-Griinwald's 
 staining solution, avoiding all rough measures (rough washing). It is 
 important not to delay the examination. Of micro-organisms there are 
 found in meningitis forms always corresponding to the aetiology, most 
 frequently tubercular bacilli, then meningococcus intracellularis, pneumo- 
 coccus, influenza bacilli, etc. The test (in the centrifuged fluid or in fluid 
 which has been set aside, same staining as usual) may be very troublesome. 
 The examination of the fluid may be of decisive importance especially for 
 meningitis and for cases of doubtful postsyphilitic diseases. In all pure 
 psychoneuroses and neuroses (at least when no syphilis appears in the history) 
 the fluid is normal. 
 
 Cerebral puncture described by Neisser, Pollack, Pfeiffer and others may 
 
EXAMINATION WITH RONTGEN RAYS 77 
 
 be spoken of as an extension of lumbar puncture. In this a fine drill driven 
 by electricity is used to pierce the cranium and then fine canulas may be 
 driven deep into the substance of the brain through the punctured hole. 
 
 For the diagnosis of cysts and (by removal of bits of substance) of tumors, 
 the method is unusually valuable, presenting no danger worth mentioning 
 to the patient; for the present, however, its use should still be restricted to 
 hospitals and clinics. 
 
 H. Examination with Rontgen Rays 
 
 Unfortunately the Rontgen process that has come to be so invaluable 
 for internal medicine, has as yet been of little use and help in neurology. 
 Indirectly, of course, it may be valuable, if, for instance, it proves the pres- 
 ence of an aortic aneurism as causation of a recurrens paralysis — of a bone 
 fracture in a neuralgia. But for actual neurological diagnostics, one should 
 try to learn clearly what the method can be expected to do; of course, it is 
 out of question to speak of proof of columnar diseases in the spinal cord, 
 neuritis, diffuse cerebral diseases and all neuroses. The chances in trophic 
 disturbances are somewhat better. In arthropathy, mal perforant, Ray- 
 naud's disease, acromegaly, we can well establish the hypertrophies and 
 destruction of the bones upon the radiograph. This may be possible also 
 for muscular atrophies. Tumors of the substance of the brain or even of the 
 spinal cord are unfortunately only very rarely or not at all to be distinguished 
 from parts covered with osseous tissue, which give quite diffuse shadows. 
 Only processes in the bone itself, that is, tumors of the base of the skull 
 or the cranial cap with pressure upon or extension to the brain, as well as 
 vertebral affections (tumors, caries) can easily be made visible — the latter 
 alas — not nearly so frequently as is to be desired (especially in incipient 
 cases) and only in favorable locations (lumbar vertebrae, sacrum, cervical 
 vertebras). Proof of the presence of foreign bodies (bullets) is always an 
 easy and a well-repaid task of radiography, whose results deserve attention 
 even with regard to neuro-therapeutics (Fiirnrohr). 
 
II 
 
 DISEASES OF THE PERIPHERAL NERVES 
 
 BY 
 
 H. STEINERT (Leipsic) 
 
 INTRODUCTORY ANATOMICAL REMARKS AND 
 DEFINITION 
 
 The peripheral cerebro-spinal nerves consist principally of fibres of the 
 peripheral motor and sensory neurons (teleneurons). Morphologically 
 and genetically the nerves of smell and sight occupy a special position. Of 
 the other cerebral and the spinal nerves it may be said that their motor 
 fibres emerge from the large motor cells located within the central organs, 
 from the anterior columns of the spinal cord and from the nuclei of the 
 brain stem, which are comparable to the anterior horns of the spinal cord. 
 The sensory fibres, on the other hand, emerge from the spinal ganglion cells, 
 and in the domain of the cerebral nerves, from the cells of the ganglia of 
 the head, which, likewise located outside the central organ, are closely 
 related to the spinal ganglia in structure and development. These are 
 the Gasserian ganglion; the spiral and vestibular ganglia of the eighth 
 nerve; the superior and petrous ganglia of the glossopharyngeal, and the 
 jugular and nodosum ganglia of the pneumogastric nerve. 
 
 Each spinal nerve originates from the spinal cord by two roots, the ante- 
 rior and the posterior. Through the anterior roots the centrifugal, chiefly 
 motor conduction, fibres emerge from the spinal cord; through the posterior 
 roots the sensory fibres enter into the spinal cord. If the roots themselves 
 or the spinal nerve be injured immediately after the roots coalesce, the 
 most important clinical symptoms — those of the loss of function — will be 
 almost identical with those resulting from an affection of the peripheral 
 neuron within the spinal cord segment to which it belongs. These clinical 
 symptoms are therefore treated in connection with the diseases of the spinal 
 cord, in whose topical diagnosis they play an important part. They also 
 play an important part in dealing with the diseases of the membranes of 
 the spinal cord, in which the roots of the spinal nerves are imbedded, so that 
 in the main we look upon these diseases as partial symptoms of diseases of 
 the spinal cord membranes. 
 
 Shortly after leaving the spinal cord the most important spinal nerves 
 undergo complicated anastomoses and interlacings of their fibres. Those 
 
 . 78 
 
PERIPHERAL NERVES 79 
 
 fibres belonging to the innervation areas, to the cutaneous areas and to the 
 single muscles, no longer are to be found grouped in bundles, in which shape 
 they were related to the central organ. Fibres of the same spinal nerve 
 roots separate into different branches of the nerve plexus; fibres of different 
 roots unite. In this way there arises an anatomical basis for the appearance 
 of new and distinct clinical pictures. The symptoms after lesion of certain 
 trunks of the plexus are grouped in a characteristic way, suited to the new 
 anatomical conditions, and differing from those of the diseases of the 
 spinal cord roots or of those of the primary trunks resulting through their 
 junction. 
 
 From these plexuses spring longer and shorter branches, by which the 
 nerve fibres, now finally divided, are led to their innervation areas, without 
 undergoing any more anastomoses. These branches, in the narrower sense 
 of the word, may be called peripheral nerves. The clinical disturbances 
 which result from their lesion are again characterized by distinct groupings 
 of their symptoms and by local diffusions. The diseases of these nerve 
 trunks and of those of the first named plexus form the principal object in 
 dealing with the diseases of the peripheral spinal nerves. 
 
 In the domain of the cerebral nerves, anastomotic formations do not 
 play such an important part as they do. with the spinal nerves. The trunks 
 proceeding from the brain retain, for the most part, their essential composi- 
 tion until their peripheral distribution. Nevertheless, even some of these 
 nerves possess anastomoses in certain places in their course, through which 
 functionally important species of fibres leave or are joined to them. In this 
 way particular varieties of clinical pictures are produced, depending on 
 whether the nerve trunk be injured in one place or another, above or below 
 an anastomosis. These symptom complexes afford us important aid for 
 the finer diagnosis of the seat of the lesion. 
 
 A description of the more intimate structure of the peripheral nerves is 
 unnecessary. It is assumed that the histology of the nervous fibres, their 
 construction from axons, medullary sheath and neurilemma, is known. All 
 fibres of peripheral nerves are constructed after one type. Only the olfactory 
 nerve consists of non-medullated fibres. It is further assumed as known, 
 that the nerve fibres are everywhere bound together to the nerve trunk or 
 branch by connective tissue, which contains also the nutritive blood-vessels. 
 According to experimental and pathological observations, the lymph spaces 
 of the nerve trunk communicate with those of the central organ. It is a 
 widely accepted belief that the connective-tissue capsule possesses its own 
 sensory nerves, the so-called nervi nervorum. 
 
 Lately the question has been discussed, how the functionally different 
 species of fibres are distributed in the peripheral nerves, whether they are 
 all mingled together, or whether certain fibres subserving special functions, 
 occupy special places in the nerve trunk. The adherents of this latter theory 
 
8o DISEASES OF THE PERIPHERAL NERVES 
 
 have spoken of a sy sterna tization of the peripheral nerves. No conclusive 
 results have been obtained so far and our answers to clinical pathological 
 questions have not been enriched from this source. 
 
 What has preceded makes it clear that the diseases of the peripheral 
 nerves have been defined as partial diseases of both peripheral neurons. 
 From what follows, it will be seen that a rather different definition is somewhat 
 more fitting, especially since the histological examination of some cases 
 shows, that, indeed, not only certain parts of the neurons are affected, but 
 that these in their total outspreading are anatomically changed. We there- 
 fore define the diseases of the peripheral nerves, as such diseases of the per- 
 ipheral neurons as definitely start from certain primary points within the 
 domain of the peripheral nerves. 
 
 Classification of Peripheral Nerve Diseases 
 
 We divide these diseases into two large groups: the destructive and the 
 neuralgic diseases. The first group is marked by a decided underlying 
 anatomical condition consisting of serious disturbances in the structure of 
 the tissues and clinically through the preponderance of symptoms of lost 
 function (Ausfallerschemungen) . In the neuralgic, on the other hand, we 
 find no regular underlying anatomical condition, while peculiar pains char- 
 acterize the clinical picture. 
 
 (I) THE DESTRUCTIVE DISEASES OF THE PERIPHERAL NERVES 
 
 A. General Part 
 
 (i) General Morbid Anatomy 
 
 The histological changes in destructive diseases of the peripheral nerve 
 concern the nerve fibres and the nerve connective tissue. The change 
 of the nerve fibres may be divided according to two primary types. One 
 form may be designated as Wallerian degeneration. Since the investigations 
 of Waller (1856) we know that after complete division of a nerve fibre, its 
 peripheral part rapidly — in fact, within a very few days — undergoes a 
 peculiar degenerative process. After some finer changes in the structure 
 of the axon have taken place, the nerve fibre degenerates by a kind of seg- 
 mentation into a number of fragments, which, at first larger, gradually dimin- 
 ish in size. An increase in the mass of their protoplasm and the number 
 of their nuclei is produced in the cells of the myelin sheath. The fragments 
 of the ruined fibres are gradually absorbed; nevertheless medullary detritus 
 is found, even after the expiration of some months, in the myelin sheath 
 (Fig. 26). 
 
 Investigation of the other important form of fibre disease begins with 
 
PERIPHERAL NERVES 
 
 81 
 
 
 1-0:1 
 
 * 
 
 ■'-J 
 
 Fig. 26. — Wallerian degeneration. (After 
 Lugaro.) Bundle of nerve fibres from the ischi- 
 adicus of a rabbit which was constricted four 
 days. Osmium preparation. A , place of con- 
 striction; B, central section; C, peripheral 
 section. 
 
 Fig. 27. — Degeneration of fibres of the type 
 of periaxial medullary disintegration. (After 
 Stransky.) Experimental neuritis through lead- 
 poisoning. The disintegrated parts of the med- 
 ullary sheath blackened by osmium. 
 
 bra 
 
 4 
 
 Fig. 27. 
 
82 DISEASES OF THE PERIPHERAL NERVES 
 
 Gombault's work in 1880. We designate this form with reference to its 
 most pronounced trait as the type of periaxial medullary disintegration. The 
 normal medullary sheath of the axon shows, at regular short intervals, inter- 
 ruptions in its course. At these points the nerve fibres have waist-like con- 
 strictions, known as the nodes of Ranvier. Here the periaxial medullary 
 disintegration frequently originates, first in the immediately adjacent 
 sections ; the outer layer of the neurilemma is disintegrated into more or less 
 fine grains. This fine-grained disintegration then seizes upon larger sections 
 of the fibres and pushes through the entire thickness of the medullary 
 sheath. Here, too, by a process of proliferation, the cells of the neurilemma 
 participate in the morbid process. Histologically, the axon undergoes 
 changes only later. Whether the total destruction of the axon, and through 
 this the local degeneration of the peripheral part and secondary Wallerian 
 degeneration, is possible, is still under debate, but there is much in favor of 
 this assumption. Repeatedly normal fibre sections between diseased sec- 
 tions have been observed in this form of diseased nerves (segmentary 
 neuritis) (Fig. 27). 
 
 While Wallerian degeneration dooms the fibre to destruction, the changes 
 in periaxial disintegration permit of a regeneration. In contradistinction 
 to these two most important forms of fibre disease, the simple atrophying 
 processes play only a subordinate part in the morbid anatomy of the clinical 
 types of these diseases. 
 
 The alterations of the nerve connective tissues are either secondary or 
 independent. In the first case, we are concerned with a mere increase, a 
 sclerosis, such as always accompanies the degenerative diseases of the paren- 
 chymatous tissues. Less often we find primary interstitial disease, which 
 usually affect the nervous elements also. Especially those pure inflamma- 
 tory processes have to be considered here, which may arise in cases of 
 hyperemia, exudation, cellular infiltration and even with hemorrhagic and 
 purulent changes. 
 
 In the most diverse diseases of the peripheral nerves we frequently find, 
 moreover, peculiar changes in the areas of the central sections of the affected 
 neurons. In the first place, in those diseases of the peripheral nerves which 
 arise from toxic substances in the blood, pathological processes occur in the 
 central organs, especially in the spinal cord, which, according to their kind 
 and extent, must be regarded as independent complications. As a rule, no 
 clinical interest attaches to them. Secondly, peculiar changes, which are 
 evidently restricted to the central parts of the peripherally diseased neuron, 
 may be observed in many cases. Above all, the root cells of the anterior 
 horn and the spinal ganglia, are implicated in the disease. It has been 
 ascertained through experimental investigations, that usually a histological 
 change sets in after complete division of a nerve fibre in the appertaining 
 ganglia cell, which consists chiefly in the destruction of the central Nissl 
 
DEGENERATION OF PERIPHERAL NERVES 83 
 
 bodies, situated in the vicinity of the nucleus, and in an outspoken eccentric 
 displacement of the nucleus. Analogous changes may frequently be noticed 
 in clinical diseases of the peripheral nerves. While in most cases these 
 adjust themselves again, very different, undoubtedly degenerative changes, 
 and even total destruction of the cell may take place, if the nerve injury is 
 very serious and permanent. Very instructive observations have been made 
 on the spinal cords of persons who have suffered amputations. The cells 
 of the anterior horn, corresponding to the amputated extremity, may 
 atrophy or may remain intact for years. It is impossible to state with any 
 degree of certainty, whether the one or the other will occur. 
 
 The proximal sections of the neurons, as well as the cells, may suffer 
 trivial, or in case of the destruction of the cells, even considerable degenera- 
 tive changes. Thus, for instance, dystrophic processes are observed in 
 precisely those areas of the posterior tract of the spinal cord which are 
 occupied by the central continuations of the peripherally diseased sensory 
 neurons. Consequently the anatomic process in many cases presents itself 
 as a general disease of the peripheral neurons. Merely histologically, it is 
 then not always possible to recognize with certainty the primary attacking 
 point of the injury. To decide in such cases whether a primary peripheral 
 disease exists, it is necessary to refer to the clinical picture. 
 
 Even if the nerve fibres are completely destroyed, an anatomical and 
 functional regeneration is possible, so long as the cellular centers of the 
 affected neurons are preserved with the central region. Whether the new 
 axon emanates from the proximal part and therefore, from the central cell, 
 or whether it is autochthonously regenerated in the periphery, is at present 
 the subject of an animated controversy. Perhaps the peripheral rem- 
 nant of the destroyed nerve does more than merely serve as a guide for those 
 fibres which newly originate from the center. At any rate the doctrine of 
 the neurons, the theory of the unity of the ganglia cells and their continua- 
 tions, can not be regarded as refuted in the chief points of its embryological 
 and pathological foundation, and this theory alone is able, at present, to 
 explain satisfactorily a great many incidents in the pathology of the diseases 
 of the peripheral nerve. 
 
 (2) General Symptomatology and Pathological Physiology 
 
 The disturbances which are common to the diseases of the peripheral 
 nerves, are determined by the nature of the fibres composing the nerve. 
 Definitely known are motor, sensible and sensory, vasomotor and secretory 
 fibres. 
 
 The most important symptom of a lesion of motor fibres, is the loss of 
 voluntary motion, which may range through all stages from slight paresis 
 to the total loss of motion, paralysis. Motor irritation phenomena are com- 
 
84 DISEASES OF THE PERIPHERAL NERVES 
 
 paratively rare and have no special practical interest. Besides, it is exceed- 
 ingly difficult to explain or to define them. A disease of the motor fibres 
 should be assumed only if clinical symptoms of loss of function are present. 
 In a few rare cases, even if the course of a single motor nerve, especially 
 the ulnaris, is interrupted entirely, there need not be any paralysis in its 
 innervation area, although as yet no experimental interpretation or satis- 
 factory explanations have been offered for this occurrence. 
 
 Passive muscular contractures are observed to result from peripheral 
 paralyses. In time the antagonists of the paralyzed muscles fall into a more 
 or less extensive and permanent process of shortening and shrinking, which 
 impedes also, to a corresponding degree, passive motion of the paralyzed 
 group, and which may lead to considerable deformity. Another form of 
 contracture, which affects the paralyzed muscles themselves, will be con- 
 sidered when discussing facial paralysis. 
 
 A detailed description of the disturbances of electric irritability, appearing 
 in peripheral paralysis, can be found in this work under the heading "Gen- 
 eral Diagnostics." Only a few points, essential to an understanding of 
 peripheral nerve diseases, are emphasized below. 
 
 In the lightest cases all disturbances of electric irritability may be lack- 
 ing. Often there is a mere decrease — this happens more frequently than 
 an increase for any length of time — in the electric irritability of the nerve- 
 muscle area. In the majority of cases a reaction of degeneration (R. D.), 
 in one of its numerous forms, may be shown. The sluggish, worm-like con- 
 traction of the muscle irritated by a direct galvanic current, is common to 
 all these forms. These contractions, according to a generally accepted 
 theory, are the deciding symptoms of R. D. It must be borne in mind, 
 however, that sluggish contractions also occur in supranuclear paralyses 
 (at least in a certain stage of the condition) occasionally in some of the 
 affected muscles, and that the myotonic reaction may also produce deceptive 
 sluggish contractions, which bear a striking resemblance to those of R. D. 
 
 About the relation of R. D. to peripheral paralysis, the following should 
 be noted. 
 
 In some diseases, as, for instance, in lead poisoning, R. D. has been 
 found even in muscles not affected by paralysis. This fact may occasionally 
 acquire significance as an objective symptom of organic disease of the nerve- 
 muscle apparatus (which otherwise could not be proven definitely) or, 
 perhaps, as an early symptom of such disease. 
 
 As a rule, if paralysis is occasioned by lesion of the nerve, the point of 
 lesion is rendered incapable both for the transmission of volitional impulses 
 and of the electric stimulus. If the nerve trunk is excited electrically above 
 the point of lesion, only those nerves can contract, whose motor fibres leave 
 the nerve trunk between the point of lesion and the point of irritation. The 
 nerve section located below the point of lesion, is at first both conductive and 
 
ELECTRICAL IRRITABILITY OF NERVES 85 
 
 irritable. After a short period of increase (which, however, can not always 
 be noted), its excitability decreases rapidly. In the case of a complete 
 division of the nerve it disappears entirely, approximately, on the twelfth 
 day. The direct faradic irritability of the muscle is wont to decrease just 
 as rapidly. It, too, disappears entirely in severe cases. The galvanic 
 muscle irritability exhibits, in the majority of cases, an increase at the end 
 of the second, or during the third, week, and this increase continues from 
 three to nine weeks, after which it gives place to a decrease. The sluggish 
 contractions occur during the period of increase as well as the period of 
 decrease. 
 
 The conditions of the regenerative period are particularly interesting in 
 those cases in which the irritability of the diseased nerve branch is entirely 
 lost. Voluntary motion returns to the paralyzed area before its excitability 
 to an electric current. Evidently the nerve becomes able to conduct voli- 
 tional impulses before it is able to respond to an electric stimulus. In proper 
 cases, observations may be made which show that conductivity for an electric 
 stimulus returns before electric irritability. It is found, for instance, that 
 when a nerve has been completely divided, an electric stimulus applied at 
 the time of the return of active motility above the point of lesion, causes 
 the muscles to contract, while a stimulation below that point is without 
 effect. The R. D. outlasts the paralysis for a considerable time. 
 
 If it were possible to place the various symptoms of the disturbances of 
 electric irritability, especially of R. D., in relation to certain physical or 
 chemical changes in the nerves and muscles, it would mean a great step 
 forward towards the establishment of a satisfactory theory for these symp- 
 toms. Of course, such knowledge would be of inestimable benefit for estab- 
 lishing a diagnosis, and, above all, a prognosis. Concerning the changes which 
 affect the nerve, it may be said with a tolerable degree of certainty, that the 
 irritability depends on the integrity of the medullary sheath, while the conduc- 
 tivity is dependent only upon the axon. The facts adduced above are in 
 complete accordance with this view. The conditions during regeneration 
 especially are thereby made comprehensible. 
 
 If a nerve trunk is completely divided, the axon is the first part of the 
 destroyed section to regenerate, by, it may be assumed, an outgrowth from 
 the central stump. The new growth of the axon is accompanied by the 
 return of conductivity for volitional impulses, which was discussed above, 
 and of electric irritability. It is only after a long time, however, that the 
 newly formed nerve is clothed with a sufficient medullary sheath, and it is 
 in accordance with this that the response of the regenerated section to electric 
 irritability, its electric stimulation, returns much later than its conductivity. 
 
 It must be assumed, that the altered irritability of the muscular substance 
 also depends on variations in its more intimate structure, although no gen- 
 erally recognized opinions concerning the nature of these variations exist. 
 
86 DISEASES OF THE PERIPHERAL NERVES 
 
 It is certain, however, that the older theory, according to which the coarse 
 degenerative changes correspond to R. D., is untenable. 
 
 The muscle that responds with R. D. to an electric stimulus, responds 
 frequently to a mechanical stimulus — such as a slight tap with a percussion 
 hammer — with a sluggish contraction, the so-called mechanical R. D. In 
 this case, too, quantitative changes of irritability are found. 
 
 In a few rare cases, it has been noted that the paralysis continues, not- 
 withstanding the return of electric irritability. In many such cases it must 
 be assumed that the cause for the continuing deficiency in voluntary motion 
 is not to be sought in the area of the peripheral neuron. If, despite the fact 
 that the neuro-muscular apparatus is evidently restored, the patient does 
 not regain control over the muscles concerned, it is possible that the cause 
 may be found in the domain of psychology. Oppenheim has spoken of a 
 habit paralysis. In other cases this symptom is to be explained differently. 
 (See section dealing with the course of facial paralysis.) 
 
 The symptoms of loss of function in the sensory area are decidedly less 
 important than those in the motor area. When a mixed nerve becomes 
 diseased, the motor manifestations of lost function appear in the foreground, 
 while the sensory occur later and are less pronounced. They are restricted 
 to parts of the innervation area, much more frequently than the disturbances 
 of motility (these partial disturbances of motility will be discussed later in 
 the chapter on diagnosis). Occasionally they are missing entirely, but this 
 is very rare; especially rare during an entire course of a serious disease. 
 Sensory function frequently returns long before the motor. 
 
 Numerous attempts have been made to explain these peculiar facts, 
 but none of them is quite satisfactory. The theory of the less liability to 
 lesion of the sensory fibres is really no more than begging the question. It 
 may be taken for granted that the double support rendered the same sensory 
 area by different nerves, and anastomotic formations between branches of 
 different nerves, play a very important part, varying in different individuals. 
 
 The different cutaneous sensation qualities, such as the sensory percep- 
 tions of touch, pain and temperature, are, in most cases, equally impaired; 
 but exceptions to this rule may occur, in which cases the perceptions of pain 
 or temperature may suffer chiefly or exclusively. 
 
 Slight as is today the basis on which to found an explanation of these 
 occurrences, it is nevertheless safe to say, that they appear not alone in dis- 
 eases of the spinal cord, but also, though much more rarely, in peripheral 
 affections. 
 
 Comparatively frequently, besides a considerable lowering or disap- 
 pearance of tactile and thermic excitability, hyperalgesia is found. A slight 
 pricking with a needle, light pressing or stroking of the skin, or stimulation 
 with feeble electric currents, even the pressure of the clothes, gives rise to 
 violent pains, while mere contacts are not perceived. Spontaneous pains 
 
SENSORY DISTURBANCES IN NERVES 87 
 
 may also occur in these cases. This condition has been designated as 
 anaesthesia dolorosa. It is found often in diseases resulting from alcoholic 
 or arsenical intoxication and in herpes zoster, more rarely after injuries of a 
 nerve. 
 
 Retardation in perception of pain, which is well known in tabes, also occurs 
 in peripheral diseases. A definite explanation of this phenomenon is want- 
 ing in both cases. 
 
 We have just touched upon the irritation symptoms. They play a role 
 of far greater importance in the sensory than in the motor areas. An 
 actual hyperesthesia, it is true, has not been noted with certainty aside 
 from the previously mentioned hyperalgesia. On the other hand, par- 
 esthesia and pains play a rather important part. The first manifests itself 
 as formication, pricklings, itchings, chilliness, and other similar unpleasant 
 sensations. The pains are felt partly in the course of the nerve, partly in 
 the area it supplies. Besides spontaneous pains, tenderness to pressure in 
 the nerve trunks plays an important, though formerly greatly overestimated, 
 part. The pains, which during disease of the nerve trunk are projected 
 into the area of peripheral distribution, may be traced to irritation of the 
 long sensory fibres which course in the nerve. The nervi nervorum are 
 probably largely responsible for the production of the other symptoms 
 spoken of. We know scarcely anything concerning the disturbances in sen- 
 sation of the deeper parts (ability to distinguish differences, of pressure; 
 susceptibility to passive joint-movement) in peripheral diseases. Especially 
 little is known about the deeper innervation areas of the single nerves. 
 
 There occur cases of disease of mixed nerves, in which, contrary to the 
 above stated rule, the sensory loss of function is greater than the motor loss. 
 Nothing definite is known about the conditions under which this occurs. 
 Sometimes the disturbances in motility show the character of those present 
 in locomotor ataxia, which according to the theory established by Leyden is 
 caused by the loss of centripetal impulses (neurotabes peripherica). 
 
 Since in disease of the peripheral nerves the motor as well as the sensory 
 limb of the reflex arc of the tendinous, periosteal, and cutaneous reflexes are 
 affected, it is self-evident that disorders of the reflexes (decrease and loss) will 
 occur. In general, one may say that the reflexes cease very early. The 
 loss of reflexes may often be the first objective indication of a destructive 
 nerve disease, just as it commonly outlasts all the other disturbances, and 
 may remain as a last and frequently permanent residuum. Cases occur, on 
 the other hand, in which the reflex, in spite of an indubitable disease of the 
 area of its reflex arc, persists remarkably long. Aside from its occurrence 
 as a transient initial symptom in some cases, a reflex increase is an exceed- 
 ingly rare occurrence, and is always a strong argument against a peripheral 
 nervous disease. Of course only those areas are meant which are actually 
 affected by the disease. An increase, for example, of the Achilles tendon or 
 
88 DISEASES OF THE PERIPHERAL NERVES 
 
 patellar tendon reflex in a pure peroneal paralysis is a common occurrence, 
 as the peroneus nerve is not at all essential for the formation of these 
 reflexes. 
 
 In a number of infectious fevers it frequently happens that during the 
 febrile stage, the tendon reflexes of the limbs disappear, at least temporarily. 
 This can be observed in lobar pneumonia, in typhoid, in diphtheria, and 
 even in ephemeral fever. The same thing occurs also in a number of toxic 
 conditions (alcoholism, diabetes mellitus), though in such cases the loss 
 frequently is permanent. It is not yet known whether in these cases the 
 anatomic lesion is to be sought in the area of the peripheral nerves. 
 
 We know little definite about the vas"o-motor disturbances. It is 
 assumed that the peripheral nerves carry both vaso-constrictor and vaso- 
 dilator fibres, thereby making it possible to understand many clinical symp- 
 toms which accompany peripheral nervous diseases. Here and there 
 redness and heat are observed, in other cases the diseased parts are cold, 
 pale and cyanosed, which can, in part, be definitely ascribed to the disturb- 
 ances of the innervation of nerves. No considerable practical interest 
 attaches to these disturbances. 
 
 Secretory disturbances will be treated minutely in the discussion of 
 the paralysis of the seventh and ninth cerebral nerves. In passing, only a 
 word regarding the production of sweat. With the motor peripheral 
 nerves course the secretory nerves for the sweat glands. Destruction of 
 the peripheral nerves may lead to the complete loss of sweat secretion. In 
 the early stage of peripheral diseases and in partial lesions hyperhidrosis, 
 which may be classed as an irritation symptom, sometimes occurs. 
 
 Of the trophic disturbances those of the musculature are by far the 
 most important. Atrophy of the muscle in diseases having an acute onset 
 develops some time after the paralysis, while in diseases with a more chronic 
 course, it may develop at the same time as the paralysis. As stated above, 
 definite histological characteristics of atrophy dependent upon disease of 
 the peripheral nerves are up to the present, unknown. The usual micro- 
 scopic methods merely show the ordinary characteristics of "simple atrophy." 
 It may be assumed as likely, that the trophic influence of the peripheral 
 motor neurons coincides exactly with its functional significance. All irrita- 
 tions which are transmitted to the muscle through a nerve tract, the psycho- 
 motor and reflex impulses, ultimately take their course over the cell of the 
 anterior horn and the peripheral motor nerves. If this course is interrupted 
 entirely, the muscle is cut off from all nervous irritation, while in supra 
 nuclear paralysis or interruptions of the pyramidal tract, irritations are 
 still transmitted to the peripheral nerves through other supervening neurons. 
 In this way the ceteris paribus, very severe, even complete, atrophy of the 
 muscles in many peripheral paralyses is explained, in contradistinction to 
 the usually far less pronounced atrophy in supra nuclear paralysis. 
 
TROPHIC DISORDERS 89 
 
 As to the contractures of the musculature some preliminary remarks 
 have already been made. 
 
 Of further trophic disturbances, those of the skin are particularly note- 
 worthy. Not infrequently the skin becomes peculiarly glossy, thin and 
 smooth, while the nails, on the other hand, lose their lustre, become cracked, 
 of irregular growth and sometimes crooked, so that a kind of onychogry- 
 phosis results. Occasionally inclination to serious cutaneous diseases, 
 eruptions, formation of ulcers and anomalies in hairgrowth are observed. 
 For an explanation of edema, which is sometimes observed, it is necessary 
 to take into consideration the immobility of the parts concerned. 
 
 As a peculiar cutaneous affection depending upon an affection of the 
 peripheral sensory neurons, herpes zoster is to be mentioned. It is chiefly 
 found accompanied by neuralgia and will be described briefly with it. 
 
 If the peripheral nerves of an entire extremity suffer a serious and ex- 
 tended lesion in earlier years, the affected limb is retarded in growth in all its 
 parts. But even in similar diseases of adults, acquired later, a slight degree 
 of bone atrophy will often be revealed by the X rays. The joints suffer 
 especially under the influence of immobility. 
 
 Besides the above there are a number of trophic disturbances which 
 have been designated as casuistic rarities. They are numerous and hetero- 
 geneous but no practical interest is attached to them. In general, great 
 deviations from the above types are very uncommon. 
 
 The essentials concerning the neuro-physiological origin of muscle 
 atrophy have been stated above. Our opinion concerning the manner in 
 which the other trophic disturbances originate, is still more pronouncedly 
 provisional. At all events, nothing forces us to the conclusion that there 
 are, besides the other fibres, also trophic fibres in peripheral nerves whose 
 only task would consist in influencing the nutritive condition of the tissues. 
 It is much more likely that these disturbances are explained part by the 
 injurious effect of the inactivity of the parts, more precisely by the loss of 
 centrifugal impulses, partly by the impairment of certain regulatory an- 
 tagonists in regard to external injuries on which the integrity of centripetal 
 tracts depends. In the first place the cessation of sensation must be consid- 
 ered. In fact many trophic disturbances appear especially in those diseases 
 resulting in grave losses of sensory functions. True trophic changes in 
 joints occur only in diseases in which there are sensor} 7 disorders. Muscle 
 atrophy occurs also where sensibility is normal. 
 
 (3) General Diagnosis and Prognosis 
 
 Destructive processes of the peripheral nerves can, as a rule, be diag- 
 nosed only in those cases in which clinical symptoms of lost functions exist. 
 Where no paresis or paralysis, no hypaesthesia or anaesthesia exists, it will 
 
oo DISEASES OF THE PERIPHERAL NERVES 
 
 be very difficult to reach even a partly certain diagnosis. One should be 
 especially cautious in not overrating the diagnostic significance of pains and 
 tenderness of the nerve trunks on pressure. The latter is an equivocal sign 
 and is found under manifold circumstances. In many nervous persons all 
 the nerve trunks react to pressure with pain sensations. In various affec- 
 tions of the joints the same symptom is found, more or less pronounced. 
 Very rarely pains appear in such characteristic ways and places as to deserve 
 any diagnestic consideration. The typically neuralgic pain, which will be 
 considered in another chapter, does, indeed, unquestionably indicate a 
 disease of the peripheral nerves, but points to so-called neuralgic changes, 
 not to destructive affections. The relation between neuralgia and destruc- 
 tive nerve diseases will be treated in the chapter on neuralgia. In most 
 cases the existing pains are not very characteristic. 
 
 The correct diagnosis depends on the accurate interpretation of the 
 symptoms of loss of motor and sensory power. 
 
 In the first place, paralyses possess certain common traits, peculiar to 
 the diseases of the entire peripheral neurons, and secondly they are charac- 
 terized by certain extensions of the disturbances which correspond to the 
 innervation area of the peripheral nerves. Taking these facts as a criterion 
 will in many cases facilitate the diagnosis considerably. 
 
 The following may be mentioned as general neuron symptoms. The 
 peripheral paralysis is in so far absolute as the muscles not only fail for cer- 
 tain functions, as in many supra nuclear paralyses, but are also in the same 
 degree cut off from all nervous stimulating impulses. Consequently the 
 so-called associated movements, which are frequently seen in central paraly- 
 ses, are lacking. A peripherally crippled hand will not reveal a contracting 
 movement, even after strong exertion of the healthy hand. 
 
 In certain cases, a sort of associated contraction sets in sometimes in a 
 peripherally crippled muscle, if intact nerves in its immediate vicinity are 
 innervated. This constitutes a rare, almost indefinable symptom, which 
 has been noted in the area of the ophthalmic muscles. Another very 
 singular form of associated contraction in paretic and paralyzed muscles 
 will be spoken of in the chapter on the course of facial paralysis. These 
 facts, however, do not destroy the value of the above cited rule. 
 
 Peripheral paralysis is flaccid. In parts not wholly paralyzed, a certain 
 amount of resistance is sometimes noted in passive motions, if these awaken 
 pain, though a real spasm without impairment of reflexes is never produced. 
 The tendinous, periosteal and cutaneous reflexes depending on the diseased 
 nerves are weakened or abolished. The genuine Babinski reflex does not 
 occur. (Concerning a pseudo-Babinski the reader is referred to tibial 
 paralysis and sciatica.) The muscles, at least in the serious cases, become 
 very atrophic. Electrical examination as a rule reveals R. D. But not in 
 all cases will the above mentioned criteria permit of a neuron diagnosis 
 
DIAGNOSIS OF NERVE TRUNK DISEASE 91 
 
 with certainty. In the chapters of this book dealing with the subject, it 
 will be shown that central paralysis may also be flaccid, and be accom- 
 panied by reflex losses, and that it, too, may cause muscle atrophy to a great 
 extent. On the other hand, the muscle atrophy in peripheral paralysis may 
 be remarkably slight, as it is generally in the lighter cases. But even in 
 severe peripheral paralysis, in multiple neuritis for instance, the muscles are 
 frequently, from the clinical standpoint, not at all atrophic, sometimes even 
 hypertrophic. In a few cases it was possible to trace the cause of this con- 
 dition anatomically to a lipomatosis of the musculature. 
 
 In contradistinction to these difficulties, the diagnostic significance of 
 the extension of the disturbances to the motor and sensory innervation areas 
 of certain peripheral nerves, in the more restricted sense, or of certain branches 
 of the nerv e plexus, by which for one familiar with these areas the diagnosis 
 at once suggests itself, is all the more important. More about this may be 
 found in the description of the various paralyses. 
 
 A few special difficulties which may obstruct a proper diagnosis, and are 
 particularly liable to mislead a novice, should be indicated here. We shall 
 not attempt, however, a discussion of the rather rare complication of central 
 by peripheral disturbances. 
 
 If very many peripheral nerves are simultaneously affected, the symptoms 
 of lost function may be so extensive as to preclude the possibility of recogniz- 
 ing a typical distribution by peripheral areas. If entire extremities, even all 
 extremities of the body are affected, it is yet possible, that by reason of the 
 disproportionate affection of the various peripheral nerves the peripheral 
 distribution type may be clearly marked in a few places, although this 
 sometimes is not the case. Then the symptoms we have described as 
 neuron symptoms, are of predominant importance in determining the di- 
 agnosis. For the diagnosis of a peripheral disease, in contradistinction to a 
 spinal affection of the same neuron, besides the certain features of its entire 
 clinical picture, astiological factors, condition of its development and course, 
 which are characteristic of certain types of diseases, the following is, above 
 all, important. Almost invariably motor and sensory disturbances co-exist 
 in peripheral diseases, just as in the most important form of dissemi- 
 nated spinal affections of the peripheral motor neuron, sensory disturb- 
 ances are, almost without exception, lacking. In other spinal diseases, 
 besides symptoms of the peripheral neuron disease, there appear typical, 
 specifically central or spinal disturbances, foreign to peripheral diseases; 
 symptoms of a lesion of the pyramidal tract, bladder disturbances, etc., 
 which unite with those into characteristic clinical pictures of the destruction 
 of certain spinal areas. (See differential diagnosis of multiple neuritis.) 
 
 But even in slightly disseminated processes difficulties may arise, since in 
 diseases of the peripheral nerves the symptoms of lost functions may be 
 confined to certain districts of the innervation area. First the paralysis may 
 
92 DISEASES OF THE PERIPHERAL NERVES 
 
 exhibit itself in different branches of a nerve consecutively, or it may continue 
 to be restricted to single branches. The preference which the disease 
 manifests for certain parts of the nerve area, is in many cases dependent not 
 on anatomic, but on functional conditions, so that under the influence of any 
 injuries, those parts will be affected primarily or exclusively, from which 
 especially strenuous and continued service is exacted. Furthermore, one 
 sometimes sees a predilective or exclusive disturbance in the remotest part 
 of the motor and sensory innervation area of a nerve, while the nearer parts 
 remain intact. This is said to occur also in cases where the lesion has doubt- 
 less affected the main trunk. It has already been noted that the sensory 
 disturbances are frequently, almost regularly, less pronounced than the motor. 
 It is very seldom that sensory disturbances are lacking completely, though it, 
 too, occurs, and mostly in light cases. Many diseases of the nerve trunks 
 exhibit only functional disturbances of certain fibres functionally belonging 
 together and terminating in the nerve, whereby a "systematizing tendency" 
 is revealed. A classical example is afforded by many cases of oculomotor 
 trunk affections, in which only the external, not the internal, eyemuscles are 
 paralyzed. The explanation of such occurrences is still a matter of contro- 
 versy. After what has been said, it is obvious that it is not always possible 
 to localize definitely intra vitam a disease of the peripheral nerves. However, 
 those are comparatively rare exceptions. 
 
 The prognosis of a peripheral nerve disease must be viewed from 
 two standpoints. First with relation to existing causative factors. If one 
 is certain that the injurious influences are at an end, that no new noxious 
 factors of any kind can influence the diseased parts, it is possible to make a 
 prognosis, not conclusively, it is true, but with a reasonable amount of 
 certainty, according to the degree of the existing disturbances. A test of 
 electric irritability is of great value in such cases. Though one may fre- 
 quently hit upon a correct diagnosis without such a test yet, upon the 
 whole, it will be practically impossible to make a fairly definite prognosis 
 without careful electro-diagnosis. As stated above, an electric test reveals 
 the extent of anatomic destruction. It must be borne in mind, however, 
 that usually the disturbances of electric irritability are only sufficiently 
 developed to permit of definite judgment, two, or even three, weeks after 
 the injury has begun its work. Slight paralyses without any pronounced 
 disturbances of the electric irritability may recover in a few weeks or even 
 days. If R. D. be present, however, it will always require many weeks, 
 sometimes even several months, for complete restoration. The more the 
 electric irritability of the nerve trunk has decreased, the less favorable the 
 prognosis becomes. When the nerve is mechanically divided, the prospect 
 of reunion between the diseased and the peripheral part affects the prog- 
 nosis considerably. Concerning its importance more will be said in another 
 chapter. 
 
CLASSIFICATION OF THE DESTRUCTIVE DISEASES 93 
 
 (4) Classification of the Destructive Diseases 
 
 The destructive diseases may be divided into two large groups. One 
 group comprises those cases, in which single peripheral nerves are injured, 
 and those rarer cases of multiple disease, in which the multiplicity bears 
 the character of being more of less accidental, so that no definite system of 
 the extension of the disturbances may be recognized. In a great majority 
 of these cases a definite local cause of the disease may be proven; in the 
 remaining cases, such a local cause is to be assumed, if the existing localiza- 
 tion is taken into consideration. 
 
 In the second of the main groups an absolutely certain, orderly, more 
 or less strictly bilateral, symmetrical extension of the disturbances occurs 
 and, as a rule, the resulting typical course of the disease is a very pronounced 
 feature. These clinical pictures are the result of internal injurious influ- 
 ences of a general character which reach the nerves through the bodily fluids. 
 
 It has been repeatedly attempted to make, besides this classification, 
 still another main division, namely, according to pathologic anatomic view- 
 points, so as to separate under the name "neuritis" the pure inflammatory 
 processes from the rest. This attempt has proven a failure, and precisely 
 there, where we possess accurate pathologic anatomic knowledge, which 
 is not always the case in our field. We shall not here point out the diffi- 
 culty of defining the "pure inflammatory," but the following must be 
 emphasized. 
 
 In the second main group of our division, the typical and regular altera- 
 tion in the nerves, bears, as we now definitely know, a purely degenerative, 
 but certainly not an inflammatory, character. Yet we' find in some cases 
 which neither clinically nor serologically are to be distinguished in any way 
 from the others, besides the usual processes, purely inflammatory foci in 
 several places. But surely no subdivision ought to be based upon this. 
 
 From the standpoint of nomenclature, therefore, there will scarcely 
 be any objection to the retention of the term "polyneuritis" to denote all 
 cases in this group, and from the standpoint of language, too, the word 
 is absolutely correct, if one recalls the purely adjectival meaning of the 
 word neuritis. As one no longer thinks only of purely inflammatory proc- 
 esses when hearing the word "nephritis," so one will also have to deprive 
 the word "neuritis" of the inflammatory characteristics, which have fre- 
 quently been assigned to it. 
 
 Not many attempts have been made to subdivide our first group since 
 it was realized that such attempts would be impracticable from the view- 
 point of morbid anatomy and would show all the deficiencies of an artificial 
 system, though it has been divided in such a way as to separate diseases 
 resulting from acute mechanical trauma as a specific group from the others 
 classified as "neuritic" diseases. But even in this, some artificiality may be 
 
94 DISEASES OF THE PERIPHERAL NERVES 
 
 clearly detected, as will be evident from the following description, which 
 disregards such division entirely. 
 
 In the first place, therefore, we shall deal with the diseases affecting 
 single peripheral nerves, peripheral paralysis in the more restricted sense, 
 mononeuritis in the broadest sense of the word, including also mono- 
 neuritis multiplex, and in the second place we shall deal with polyneuritis. 
 
 B. Special Part 
 i. Diseases of Single Peripheral Nerves 
 
 General JEtiology 
 
 Of greatest serological interest are the local injurious influences. 
 The relations to mechanical trauma are remarkably diversified. Mechanical 
 injuries act directly or indirectly on the nerves. The direct traumatic 
 effects are of the most varied degrees from a comparatively light pressure 
 to complete division, tearing asunder, cutting or crushing of the nerve. 
 The indirect traumatic effects are produced by such causes, for instance, 
 as an excessive motion of the body, producing an undue stretching of the 
 nerve. This will be gone into more minutely when discussing paralysis of 
 single nerves. 
 
 Indirectly, trauma may be responsible for peripheral paralysis in three 
 ways. In the first place, long continued mechanical irritation, such as the 
 pressure of crutches, may in time lead to degenerative processes and nerve 
 paralysis (so-called traumatic neuritis). But even a single mechanical 
 insult seems to be able to give rise to similar diseases. The trauma is at 
 once followed by pain. Only after some days — or weeks — the symptom of 
 lost function develops. Secondly, conditions resulting from an originally 
 non-nervous lesion may cause mechanical nerve injuries. After fracture of a 
 bone it is sometimes observed that nerve trunks not primarily affected may be 
 injured secondarily by bone fragments. Scar tissue or the outgrowth of 
 callus may lead to direct-pressure injuries by growing around and con- 
 stricting a nerve, or nerve trunks may be forced into such unfavorable posi- 
 tions that they are exposed to injuries whenever a member is moved. Nerve 
 dislocations, by which the nerve is displaced from its normal position, through 
 traumatic influences, act in the same way. Under such conditions, an old 
 trauma may lead to paralysis even after several years (late traumatic lesions) . 
 In the third place, finally, so-called ascending neuritis must be considered. 
 As a result of small infected wounds, splinter injuries, etc., especially of the 
 hand", grave neuritis of the nerve, in whose innervation area the wound was 
 located, develops in rare cases. 
 
 Other affections in addition to trauma may cause mechanical injuries to 
 nerves. Tumors, in the broadest sense of the word, and bone disease, may 
 all lead to paralysis of neighboring nerves through pressure. 
 
CA USES OF LOCAL NEURITIS 95 
 
 Malignant tumors may also affect the nerve directly; inflammation in its 
 proximity may spread to the nerve itself and either cause an inflammation, or 
 its degeneration through toxic influences. Ulcers and other cutaneous dis- 
 eases lead now and then to degeneration of the sensory terminal fibres in 
 their own area and that in their immediate vicinity — a most interesting fact 
 theoretically, but one which has been but little studied. 
 
 In order to explain a few. peculiarities of the clinical picture, a separate 
 short discussion will be devoted to tumors arising from the different an- 
 atomical elements of the nerve trunk itself. 
 
 A peripheral paralysis occasioned by local chemical injury is sometimes 
 seen after a bungling injection of ether or camphorated oil containing ether, 
 made in the immediate vicinity of a nerve trunk. Some observations would 
 seem to make it probable that absorption by the skin of bisulphide of carbon 
 — an injury to which workers in rubber are often exposed — may cause local 
 neuritis. 
 
 Local thermic injuries play only a subordinate part. When discussing 
 facial paralysis we shall meet with conditions which may be regarded as 
 arising from local colds. 
 
 It is said that arterio-sclerosis of the vasa nervorum may lead to degen- 
 eration of the nerves. 
 
 As a final local cause overexertion of certain parts of the body in 
 particular occupations must be considered. It will be necessary, however, 
 to discuss briefly the peculiar so-called occupation paralyses in a separate 
 paragraph. 
 
 Besides the local influences, general injuries acquire some significance, 
 even in the aetiology of the diseases of single peripheral nerves, though it can 
 not be said they they play more than a predisposing role. The same things 
 will have to be named here as are enumerated in the aetiology of polyneuritis : 
 infections and toxic influences, disorders of metabolism, pregnancy and the 
 puerperium, and debilitated conditions. Persons affected with tabes reveal 
 a peculiar inclination to peripheral paralysis. In all such cases it is advis- 
 able, however, to assume a locally acting disturbance besides the general pre- 
 disposing causes. If, in a few cases, it is not possible to prove it, it should be 
 remembered that with a strong predisposition under certain conditions, 
 trivial local influences which may be easily overlooked suffice to excite dis- 
 ease. Sometimes, at least, conjectures may be formed concerning their 
 nature. If, for example, an alcoholic after the passing of the delirium has 
 a radial paralysis, it can scarcely be amiss to assume a traumatic cause in 
 addition to the toxic predisposition. 
 
 Pathological Anatomy 
 
 Simple disintegrative processes of the nerves are much more frequent 
 than purely inflammatory changes. The latter are found especially in those 
 
96 DISEASES OF THE PERIPHERAL NERVES 
 
 cases in which the vicinal inflammation has extended to the nerve (extended 
 lymphogenous neuritis) , in neuritis emanating from infected wounds and in 
 local influences of chemical poisons. The nerve in such a case is turgid, red- 
 dish, while the microscope reveals hyperaemic and exudative changes, and 
 cellular, even suppurative infiltrations. Soon also, naturally, the paren- 
 chyma participates in this process. Occasionally bacteria may be found in 
 the nerve trunk. To distinguish between perineuritic, interstitial neuritic, 
 or nodular forms according to the degree to which the perineurium or the 
 endoneurium participates, or according to the form of the nerve swelling, 
 would be useless. 
 
 The parenchymatous degeneration which, as was said, in most cases 
 exists without actual inflammatory complications, bears in most of the lighter 
 cases the character of periaxial decay. This has been shown to be true 
 especially of the lighter traumatic influences. Though the axon is surely not 
 anatomically destroyed, its conductivity for the will impulses, at least, may be 
 affected temporarily. In such cases the mobility returns very soon after 
 the causative disturbance has been removed. Even if the duration of the 
 injury be longer (as in pressure due to callus and the like), the disease may 
 remain in this lighter stage, and under proper treatment permit of a very 
 favorable prognosis. 
 
 If the local injury is greater, and the axon is entirely destroyed, the 
 peripheral nerve section is doomed to Wallerian degeneration. The 
 regeneration will take the longer the more centrally the interruption of the 
 nerve has taken place. The reunion of the central and peripheral parts, 
 even if produced only by interposition of granular tissue which paves the way 
 for the newly formed fibres to the peripheral part, is an important factor 
 in the process of regeneration. Should the reunion not take place, the re- 
 generative efforts of the central end will lead to the formation of what is 
 known as amputation neuromata; knoblike swellings that form clinically 
 most irritating structures, which may cause violent pains. 
 
 Those very rare cases in which functional restoration has been reported 
 without the reunion of the two nerve ends are perhaps best classed with 
 those equally rare cases, already mentioned, of the complete division of large 
 nerve trunks without clinical symptoms of lost function. 
 
 The clinical picture will be considered here only very briefly. Those 
 symptoms which occur in common in destructive diseases of the peripheral 
 nerves have already been discussed in the general part and the clinical 
 picture in diseases of different single nerves will be shown in the next 
 section. It may suffice to state summarily once more here, that in paralyses 
 of mixed nerves, the characteristically grouped motor symptoms of lost 
 function together with the accompanying disturbances of the reflex function 
 and of the electric irritability, stand almost without exception in the fore- 
 ground. As a rule, the sensory symptoms of lost function, according to the 
 
DIAGNOSIS OF LOCAL NEURITIS 97 
 
 principle cited before, yield to the motor ones; still they belong to the cardinal 
 symptoms, even though they must often be sought for. More often 
 symptoms of sensory irritation are lacking. No general rule can be laid down 
 regarding their occurrence or non-occurrence. It is to be remarked, however, 
 that their absence is the rarer, the greater and more continuous the irrita- 
 tion of the nerve trunk dependent upon anatomic processes is. It would 
 be presumptuous, nevertheless, to draw from the sensory irritation symptoms 
 definite conclusions concerning the finer anatomic nature of the nerve disease. 
 Least of all is it possible to establish a rule about the pressure sensitiveness, 
 which sometimes extends to the musculature of the diseased region. In the 
 pure inflammatory processes, cord-like or knotty constrictions of the nerve 
 trunk may sometimes be felt. Whenever fever occurs it indicates that an 
 infectious process is producing the paralysis. 
 
 For a discussion of the diagnosis of the paralyses of single nerves, which 
 is usually very easy, the reader is referred to the general part, and for some 
 specific peculiarities, to the following section. Only a few remarks regarding 
 differential diagnosis are made here. We shall only mention here the great 
 demand that complications blurring the clinical picture of the peripheral 
 paralyses, because of central organic and hysteric disturbances, make on 
 diagnostic acumen. 
 
 Real difficulties arise now and then, especially in lighter cases, when the 
 symptoms of the paralysis are not very grave, its boundary not clearly defined 
 and when it affects only a few of the muscles subserved by one peripheral 
 nerve. Differentiation of spinal affections of the peripheral motor neuron 
 may then be rather difficult, and all the more so, because the innervation 
 areas of some of the spinal segments are very similar to those of some of the 
 peripheral nerves. Even when the disturbances of sensation do not cor- 
 respond to a whole peripheral nerve area, but are confined, say, to a distal 
 part of one, it is not always possible to base a decision on this, as in spinal 
 diseases also the disturbances of sensation may be restricted to distal parts 
 of the innervation area. In cases in which the knowledge of certain setiolog- 
 ical factors, or the course of the disease, or the finding of decisive symptoms 
 in other areas, does not enable a sure diagnosis, it will have to remain in 
 abeyance. 
 
 Experience shows that certain non-neurogenic contractures, atrophic 
 and paralytic conditions, sometimes lead to an incorrect diagnosis, which, 
 however, might almost always be avoided in these cases. Tendon and 
 fascia contractions, joint anomalies and cicatricial processes, may produce 
 deformations of the limbs, which superficially resemble the contracture pro- 
 duced by peripheral paralyses. A careful examination will enable one to 
 decide whether the situation of the contracture is conditioned upon a loss of 
 function in a definite nerve area. Of course, extensive fixation of the part 
 may make the investigation more difficult, especially in inveterate cases, and 
 7 
 
98 DISEASES OF THE PERIPHERAL NERVES 
 
 it will then become necessary, besides considering the contractibility of the 
 muscles, the reflexes, the electric reaction and the sensibility, to consider 
 whether the existing deformities may at all be traced, according to their form, 
 to peripheral paralysis (for the form of contractures in peripheral paralysis 
 is usually very characteristic) , and whether other causes for the deformity are 
 to be found. 
 
 It is usually very easy to determine correctly arthrogenous or arthritic 
 atrophy, because of its characteristic distribution in certain definite muscles 
 in the vicinity of a diseased joint. Usually the extensors of the joint are 
 affected. Atrophy of the quadriceps femoris in diseases of the knee and the 
 predominant atrophy of the deltoid and supra- and infraspinatus in arthritis 
 of the shoulder joint is especially important. The functional impairment 
 may attain to actual paralysis. The tendon and periosteal reflexes are in 
 such cases always exaggerated. 
 
 Ischaemic paralyses and contractures result when the blood supply to a 
 certain muscle area is seriously obstructed by too tight bandages or, as occurs 
 more rarely, through lesion of blood-vessels. If relief is not had soon, the 
 muscles fall into a paralytic and degenerative condition resulting in a rigid 
 contracture, which is not dependent upon nervous lesions. It has been espe- 
 cially noted in the upper extremities and is minutely described in surgical 
 text-books. 
 
 These and other myopathic paralyses and contractures are distinguished 
 from similar peripheral neuritic affections, not only by the aetiological 
 peculiarities, but also by the absence of pronounced sensory symptoms of 
 lost function, by the regular absence of R. D., and by the peculiar distribution, 
 which is not confined to the innervation area of peripheral nerves. 
 
 If careful consideration be given to the nature of the symptoms of lost 
 function, ' and if further, the anatomic extent of the symptoms which are 
 dependent upon the extent of the innervation areas be as carefully determined, 
 the diagnosis and differential diagnosis will fail one only in very rare cases. 
 
 Prognosis. — What was stated above concerning the different aetiological 
 factors will serve as an ampler illustration of the fundamental principle 
 already referred to in the general part, namely, that the prognosis is affected 
 decisively by the nature of the primary injuries. 
 
 A few viewpoints not here discussed, will be dealt with in the following 
 therapeutic section. 
 
 General Therapeutics 
 
 The prevention of peripheral paralysis not infrequently occupies the 
 attention of the physician. Both the surgeon and the obstetrician very 
 frequently are compelled to endanger the peripheral nerves in various ways. 
 Even if it is now and then, unavoidable to injure a nerve for the sake of 
 
THERAPEUTICS OF NEURITIS 
 
 99 
 
 attaining more important ends, it might more frequently be avoided, if only 
 the danger were considered. In making hypodermic injections, one ought not 
 to select a place near a large nerve-trunk. In prescribing medicines, the 
 possibility of producing hazardous conditions (as from the too long continued 
 use of arsenic) must be considered. Of great import is the prophylaxis, 
 depending upon the hygiene of the trades or occupations in which poisons 
 are used. As to predispositions it is necessary to remember the special 
 liability to lesion of the nerves of these individuals with regard to local in- 
 fluences (pressure paralysis in cases of tabes and in alcoholics). These 
 suggestions will suffice here. The preceding aetiological section furnishes 
 some further points, and in the following chapter special cases will be 
 mentioned, in which the especial attention of the physician is essential. 
 
 The actual treatment begins with an endeavor to remove the original 
 noxious factors as far as possible, or to prevent their further spread. Further 
 procedure depends on the nature of the injury. If a nerve is mechanically 
 divided, surgical aid, if at all possible, should be rendered at once. In other 
 cases, one should, in the early stages, confine one's self to preserving in its 
 broadest meaning the diseased part, avoiding all injurious influences, and to 
 symptomatic alleviation of the most severe troubles. Later, one's efforts 
 may be more directly aimed at the preservation and restoration of function. 
 Generally speaking, it may be said that this more active method of proce- 
 dure is ''n order only when the regressive changes will positively not progress 
 any further. In paralyses caused by a single acute insult this method may 
 be started about the third week. 
 
 General Measures. — In the early stage the diseased part should be 
 kept absolutely quiet, be put in slings, splints, bandaged in cotton, and pre- 
 served from shocks (especially of the nerve trunks) by proper padding. 
 The warmth generated thereby, is usually most beneficial. 
 
 Anaesthetic areas should be particularly guarded. Mechanical and 
 thermic injuries, which the patient does not notice because of the anaesthesia, 
 may lead to serious injuries, difficult to heal (burns caused by hot-water 
 bottles). 
 
 That the general health must be carefully observed and taken care of, is 
 not at all merely a schematic phrase, but in our field, particularly, is of the 
 greatest importance. Experience has shown that the prospects for the 
 regeneration of destroyed nerves depend considerably on the general condition 
 of the patient. Because of this, general medical principles have to be fol- 
 lowed. Anaemia, disturbances in metabolism, enfeebling diseases of any 
 kind, should be treated carefully ; the expenditure of strength, as far as possible, 
 reduced, and the diet regulated. 
 
 Toxic injuries, whether occasioned by one's trade or occupation or by 
 one's manner of living, should be strictly avoided, even if they were not a 
 factor in producing the disease. Patients who imagine that they may in- 
 
Hospital No. 20, 
 
 ioo DISEASES OF THE PERIPHERAL NERVES 
 
 dulge their taste for alcohol during the treatment, have little prospect of 
 recovery, and had better be discharged from treatment. 
 
 Medicines are to be considered first for the causal treatment. It is 
 usual to point out, that in combating infectious diseases and conditions due 
 to intoxicative processes, it is only necessary to proceed with suitable reme- 
 dies, such as iodides and mercury, for syphilis; quinine for malaria; iodide 
 of potassium for lead poisoning. But it is very seldom that these factors 
 enter into the question at all— more frequently they lead, not to paralysis of 
 single nerves, but to polyneuritis. Much more important with us is the use 
 of medicines with regard to the individual symptoms. To reduce the pains 
 we give the numerous well-known antipyretic and antineuralgic agents, 
 concerning the prescription of which the reader is referred to the neuralgias. 
 Morphine should be used very seldom. 
 
 Besides various other reasons against the use of vesicants and derivants 
 in painful conditions, it may be urged that the injured skin may afterwards 
 interfere with the electric treatment. If it is desired to administer a mild 
 cutaneous irritant beneath the bandage, mistura oleoso-balsamica or a 
 10% menthol oil should be applied to the aching parts. If an intense in- 
 flammatory swelling occurs near a nerve trunk, an attempt at relief may be 
 made by applying a few leeches. After the acute stage has ended, alcoholic 
 and other embrocations may often be recommended in combating the pares- 
 thesia. (Spirit, camphorat, 5% menthol in alcohol et al.) 
 
 Of those remedies to which a tonic action upon the nervous system is 
 ascribed and from which for that reason a direct action upon the regenerative 
 processes is to be expected, preparations of strychnine may be tried first. 
 They are best given in the shape of so-called tonic pills (Erb). 
 
 T}. Yen:, lactic, 
 
 Extr. chin, spirit aa 6 .o (gr. 90) 
 
 Extr. nucis vom 0.6 (gr. 9) 
 
 F. pill. LX S. 3 times daily a pill. 
 
 Or strychnin, nitrat. may be given by hypodermic injection, at first three 
 times a week, after that once daily i mg. The dose may gradually be some- 
 what increased. 
 
 Hydro- and thermotherapy have an extensive field of usefulness. In 
 the acute stage Priessnitz's compresses, which will usually have to be applied 
 warm, may be tried alternating with dry dressings. Other forms of local 
 devices which retain the heat may be tried. Poultices, cataplasms, thermo- 
 phores will very often have a favorable action upon the pains. Cold appli- 
 cations are not so often beneficial. Should this be the case, water or ice bags, 
 ice cataplasms and especially compress bandages may be applied, in which 
 the limb is bandaged with a moist linen bandage, which is kept wet by fre- 
 quent moistenings. Protracted cold applications are in general to be re- 
 
THERAPEUTICS OF LOCAL NEURITIS 101 
 
 garded merely as a transiently admissible symptomatic treatment in the 
 acute stage. 
 
 Baths of the entire body or of the diseased part may be used advan- 
 tageously in any stage of the disease. Baths of so-called "indifferent tem- 
 perature" (96 F., 35 C.) have a quieting effect upon sensory irritative 
 conditions, and probably act favorably besides in stimulating and regulating 
 the peripheral circulation. Additions of pine-needle extract (1/4 lb. for a 
 full, a few tablespoonfuls for a partial, bath) will often increase the agreeable 
 effect. Baths may be taken every day, or later 2 to 3 times a week; the 
 patientshould stay in at first 15 minutes, afterwards up to an hour. Pro- 
 tracted baths may possibly produce an enfeebling effect upon the general 
 condition, which should, of course, be avoided. For insomnia, the baths 
 will be most effective if taken in the late afternoon. 
 
 No indication will usually be found for the use of hot- water baths (ioo° F.) 
 and other general hot procedures which are, as a rule, more fatiguing. If a 
 cold seems to have produced the palsy — a rather rare occurrence, by the way 
 — diaphoretic treatment may be tried at the outset. In cases of poisoning, 
 too, especially those due to certain metals, one may try to help along the 
 treatment by occasional sweatbaths and packs. Concerning the practical 
 application hydrotherapeutic text-books should be consulted. 
 
 Local bog, peat or mud packings (122 F., duration from 1/2 to 2 hours) 
 most likely affect convalescence advantageously during the regenerative 
 stage; at any rate they will have a favorable effect upon symptoms of sensory 
 irritation that still exist. 
 
 Douches to produce irritation in sensory nerves, in disturbances of sensi- 
 bility, may be employed as well as other means which will be considered 
 somewhat later. 
 
 Electro-therapeutics is of much importance. It is true that some per- 
 ipheral paralyses recover without electric treatment, but the omission of an 
 indicated electric treatment in serious cases is always a grave error in judg- 
 ment. Aside from certain surgical procedures and some necessary measures 
 of general hygiene, all other methods rather than the electro-therapeutic 
 could be omitted. It is, however, not merely a question of employing 
 "electricity" but of employing it skilfully and by the proper method. One 
 who can not fulfill this demand should, as a matter of principle, abstain from 
 personally treating peripheral nerve diseases. This may even today be said 
 with a full recognition of the fact that the critical, even the sceptical reaction 
 against the former overrating of electro-therapeutics is justified. Of the 
 many methods recommended for our purpose, we shall especially emphasize 
 the following, approved by long experience. In the present state of our skill 
 and knowledge they will ordinarily suffice. 
 
 In case of severe pains in the early stage, stabile anodal galvanization, a 
 classic method of treating neuralgia, which will be described later on, may be 
 
102 DISEASES OF THE PERIPHERAL NERVES 
 
 used. The anode is applied at the point of greatest tenderness or in more 
 serious local lesion severing the nerve, in the region of the central stump. 
 
 The method just mentioned is a symptomatic one, to be tried occasionally, 
 while to the following two considerable influence on convalescence and the 
 prospect of recovery is justly ascribed. 
 
 The stabile cathodal galvanization is applied when the nerve trunk has 
 suffered a lesion, which may be definitely localized, particularly in pressure 
 paralysis, but in other traumatic affections also. The cathode (electrode 
 of about 20 qcm.) is placed over the point of lesion, the inactive (dispersing) 
 anode (about 100 qcm.) on the breast or back. The current is increased 
 and diminished gradually. Current strength: 5-6 (4-8) milliamperes. 
 Duration of treatment: 5-6-10 minutes. Seances to be repeated daily, at 
 least every other day. Treatment should be begun a few days after the 
 paralysis has set in. Reliable experience proves that it is apt to hasten the 
 convalescence (E. Remak). Instead of leaving both electrodes stationary, 
 the active electrode, the cathode, may be moved up and down the course of 
 the nerve trunk to be treated, without, however, removing it from the skin. 
 
 The actual galvanic stimulation of the diseased region is the most 
 important treatment in the later stages of the paralysis, and may sometimes 
 be combined with the preceding method, but should never be applied 
 before the third week. The endeavor is to produce muscular contractions 
 through direct stimulation of single muscles. Button-shaped electrodes, 
 whose surface is about 10 qcm. and, if desired, having a current interrupter 
 attachment, are used for this purpose. Generally speaking, the muscle is 
 stimulated with a current of just sufficient strength, afterwards with a 
 somewhat stronger current, applying the cathode as the irritation electrode, 
 or in cases of D. R. when the anode excites greater contractions, applying 
 it preferably. Stimulation is produced by abrupt closing of the current 
 by the interrupter, or by moving the electrode over the muscle to be stimu- 
 lated. In cases of considerable diminution of the irritability, alternating 
 currents with attached electrodes, so-called voltaic alternating currents, 
 are employed. It is best to let the muscles contract one after the other, 
 and then to begin again with the first. In this way each muscle of the 
 paralyzed area should be stimulated at first, say, 10 times, afterwards from 
 20 to 40 times. Cylindrical electrodes are also in use with which the 
 diseased area is traversed in a centripetal direction. The inactive large 
 electrode is again placed on the breast or back. Some recommend that it 
 be placed on a proximate part of the member to be treated, or on the nerve 
 trunk whose muscle area is to be treated. If the irritability is not too 
 greatly decreased, some stimulations of the nerve trunk may be added to the 
 galvanization of the muscles. Daily sittings should be held so far as 
 possible. 
 
 This method may or must be modified in cases where great sensory 
 
THERAPEUTICS OF LOCAL NEURITIS 103 
 
 irritability, hyperalgesia, to the electric current exists. All painful fluctua- 
 tions in the current are to be avoided by a very gradual increase of the 
 current, after the electrode has been attached, and moving the active elec- 
 trode up and down over the area to be treated, without, however, removing 
 it from the skin. The electrode is taken off only at the termination of the 
 treatment after the current has been gradually diminished. 
 
 Further, another method which does not favorably compare with the 
 above and which is based on the so-called refreshing influence of the flow 
 of the galvanic current in penetrating the muscle, may be applied. Two 
 nearly similar, medium sized, flat electrodes are applied in such a way on 
 suitable places of the paralyzed musculature that after closing the current 
 it will penetrate the muscles ; the anode on the more tender parts of the skin. 
 The current of 4 to 6 milliamperes is gradually increased and diminished. 
 The duration of the seance, during which the position of the electrodes may 
 be changed by sliding them over the skin, should vary from 5 to 10 minutes. 
 
 Just how these methods influence recovery in paralyses, is still prob- 
 lematic; but clinical experience has clearly shown that they prevent, to a 
 certain extent, muscular atrophy and that they promote recovery. Some 
 experimental investigations also give support to this statement. 
 
 The faradic current may be used in those cases of paralysis in which 
 the irritability has decreased but little and medium faradic currents suffice 
 to cause contraction of the muscles. They should be stimulated by stroking 
 or rolling of the musculature, or better still by sudden closing of the current. 
 Energetic prolonged tetanization of the muscles can not be advised. How- 
 ever in motor paralysis we decidedly prefer the galvanic treatment. Gal- 
 vano-faradization is recommended by a number of authorities. Both 
 currents are brought simultaneously to the electrodes, and the currents are 
 graduated in such a way, that each current just suffices by itself to produce 
 contractions. It is always presupposed, of course, that the faradic irritabil- 
 ity of the parts concerned has not suffered severely. The faradic current 
 is usually contra-indicated in motor and serious sensory conditions of 
 irritation. In the first stage of paralysis in which genuine irritants are to 
 be avoided, the faradic current should not be employed under any conditions. 
 
 The faradic current is particularly to be recommended for the treatment 
 of anaesthesia, if there is no contra-indication for other reasons. A dry, 
 brush-shaped electrode is used, with which the anaesthetic area is stroked 
 or, moving it from place to place, tapped with a current just strong enough 
 to excite the sensibility. By the use of a cylindrical or movable button 
 electrode in galvano-faradization it is possible to do justice at one and the 
 same time to the indication of the muscle irritability and the irritation in 
 sensibility. 
 
 The electric treatment should generally be continued until convalescence 
 is established. Treatment of a few weeks in grave paralysis will not be effec- 
 
104 DISEASES OF THE PERIPHERAL NERVES 
 
 tive. It will be clearly seen in many cases that in every stage of the conva- 
 lescence electric treatment acts beneficially, while the omission of it will 
 retard the progress of recovery. Sometimes, in inveterate cases, improvement 
 sets in only after the electric treatment has been begun. The ameliorating 
 effect which it frequently and directly produces on function, is especially 
 adapted to animate the spirits of the patient with all its consequences. 
 Experience proves that patients hardly follow as faithfully any other method 
 for such a long time as they will electro-therapeutics, even if the progress of 
 recovery be but slow. 
 
 This treatment should be tried even in quite inveterate cases. As long 
 as the musculature is still capable of being electrically irritated and the nerv e 
 conductivity is not wholly destroyed, even if only a prospect for the regenera- 
 tion of the nerve exists, there is still hope for success. 
 
 In the stage in which absolute rest does not seem necessary any longer, 
 massage materially assists electric treatment of the muscles. Exact pro- 
 cedure based upon anatomy and performed best according to Hoffa's ex- 
 cellent instructions 1 is indispensable. 
 
 Besides efSeurage, gentle, later vigorous kneading and careful percussion 
 may be instituted; the more energetic manipulations should be used only in 
 the stage of convalescence. Even if massage of the muscles may in a few 
 exceptional cases be entrusted to a specially trustworthy unprofessional 
 masseur, massage of the nerve trunk should be attempted only by an experi- 
 enced professional hand and then only in a few exceptional cases. The very 
 great liability of injuring this delicate structure should always be kept in 
 mind. 
 
 Medical gymnastics in its widest sense, is absolutely necessary in a cure 
 of peripheral motor paralysis. As soon as mobility returns, each injured 
 muscle should be exercised systematically. One should never depend on the 
 patient's using his muscles of his own volition because he fails to do so only 
 too often, or if he really uses them, he does so usually to an insufficient degree. 
 
 But even in the stage of complete paralysis, as early as the fundamental 
 principles already laid down will permit, gymnastics, at first only carefully 
 executed passive movements, should be instituted. By this means the devel- 
 opment of contractures is prevented. This danger should be thought of in 
 the beginning also, particularly in reference to the position of the diseased 
 parts. In a peroneus paralysis a pes equinus position is not to be tolerated; 
 in a radialis paralysis the hand is not to be left flexed. Proper support and 
 bandages should keep the member in a medial position. Above all, passive 
 movements promote the return of active motility. The patient should from 
 the beginning endeavor actively to assist the passive motion. The moving 
 hand perceives immediately when these endeavors begin to be successful. 
 Then the passiye motion is replaced by passive-active gymnastics. The dis- 
 
 1 His "technic of massage" (Stuttgart, 1903) is, in its technical suggestions, authoritative. 
 
THERAPEUTICS OF LOCAL NEURITIS 105 
 
 eased member requires aid for some time yet, if it is to utilize the existing 
 minimum of strength gymnastically. This support is rendered by the hand 
 of the physician or his assistant, who have so far moved the member only 
 passively. In many cases an unaffected hand of the patient may render the 
 assistance, in suitable cases, by means of a skilfully attached strap. Thus 
 if a part is to be dorsally flexed a strap may be attached to it and worked 
 by the patient. An excellent means of aiding the first attempts in active 
 gymnastics is afforded by warm baths. The laws of physics enable the 
 patient to execute motions independently in the bath which he is unable to 
 make otherwise (kinetotherapeutic baths) and this is a particularly important 
 point indicating the bath treatment (Goldscheider) . 
 
 If apparatus for so-called semi-active gymnastics is at one's disposal, 
 apparatus which mechanically assists the performance of the patient and 
 enables him to make movements with the expenditure of but little strength, 
 like the Krukenberg pendulum apparatus and similar contrivances, it may 
 be used. 
 
 With further progress toward recovery the real movement exercises and 
 finally the various forms of resistance gymnastics are reached. 
 
 As a general rule for gymnastic therapy it may be stated, that here as 
 everywhere, the patients should exercise frequently, but not long at a time; 
 that they should never be tired out or too much exhausted by it. 
 
 Surgical treatment, in many cases the conditio sine qua non of recovery, 
 is discussed separately in this work. We shall therefore simply suggest here 
 the general possibility of its application. 
 
 When a nerve is functionally injured by surrounding scar tissue, by pres- 
 sure of masses of callus, or, in a similar manner, neurolysis, notwithstanding 
 the fact that in some cases a spontaneous recovery may obtain, should, as a 
 rule, be used promptly as it sometimes restores the nerve conductivity directly. 
 A completely divided nerve should be sewn, if possible, primarily, as 
 restoration without the reunion of the nerve-ends, despite some sporadic, 
 anomalous cases, not as yet explained, can not be expected. If any doubt 
 exists as to the necessity of a suture, it is in most cases advisable to look for 
 the nerve operatively, and to decide then whether or not to suture it. In 
 inveterate cases, where the course has demonstrated that an amelioration is 
 not to be expected without surgical intervention, the different methods of 
 even secondary nerve suture promise success. If this is impracticable the 
 advisability of nerve grafting will have to be considered. 
 
 Of course in all these cases, with but few exceptions which are difficult to 
 explain, a long time will elapse before functional restoration takes place. 
 
 If the morbid process has entirely ceased, and it is quite obvious that a 
 change, either for better or for worse, will not set in, and if the procedures for 
 the restoration of innervation are no longer a point of issue, orthopedics are 
 in order. Attempts should be made to give the paralyzed member support 
 
106 DISEASES OF THE PERIPHERAL NERVES 
 
 by means of immobilization apparatus, by arthrodesis, by operative short- 
 ening of relaxed tendons. Contractures are relaxed by tenotomy or by 
 plastic operations on tendons; unimpaired muscles, by transplantation of 
 tendons, are made to serve as substitutes for muscles whose functions have 
 been lost. 
 
 In many cases such measures will produce extraordinary results. But 
 one must not expect too much from orthopedic operations. If the patient is 
 used to an old defect, and endures it without considerable impairment of 
 his well-being and his capabilities, it would mostly be well to omit surgical 
 operations, the result of which is never quite certain. 
 
 Finally let it be particularly emphasized that besides operative procedures 
 the other methods of treatment should never be neglected. 
 
 PARALYSIS OF INDIVIDUAL PERIPHERAL NERVES 
 
 Although in the following the clinical picture of diseases of individual 
 peripheral nerves is to be discussed, it will not be done with exclusive re- 
 gard for the clinical group of single diseases. We shall keep in mind that a 
 great part of the paralyses to be treated here, appear as partial symptoms also 
 in polyneuritis. 
 
 A. The Cranial Nerves 
 
 i. The Olfactory Nerve 
 
 It is obvious that the olfactory nerve may, in a number of intra-cranial 
 diseases, be affected unilaterally or bilaterally. In tabes it may atrophy. 
 
 Independent isolated affections of the olfactory nerve are rare. Only 
 one type is of some practical interest. After trauma of the skull, anosmia, 
 which can be ascribed to the mechanical destruction of the olfactory filaments, 
 may occur. The loss of the sense of smell may mean an impairment in the 
 ability to make a living, especially in those occupations in which the tasting 
 and "smelling" of food or drink is essential, and in others in which the organ 
 of smell is necessary to protect one's self from dangers, such as poisonous 
 gases (chemical factories, etc.). 
 
 Destruction of the olfactory nerves results in loss of smell. At the same 
 time, however, there is involved great impairment of the ability for what is 
 colloquially termed "tasting." The ability to taste is in organic anosmia 
 restricted to the elementary taste qualities (sweet, sour, etc.). On this the 
 differential diagnosis in regard to hysteric anosmia may be based. One 
 affected with organic anosmia, despite the preservation of the organs of 
 taste, lacks the ability to distinguish all those articles by the "taste," for 
 whose recognition, unknown to most people, the co-operation of the sense of 
 smell is necessary. If this ability is preserved in spite of anosmia, it 
 points unquestionably against an organic foundation. 
 
THE OLFACTORY AXD OPTIC NERVES 107 
 
 It must be remembered that by far the most frequent cause of anosmia is 
 nasal disease, and that it must be carefully excluded before nervous anosmia 
 is diagnosed. In obstruction of the posterior nares the sense of smell, in 
 subserving taste, is much diminished. Patients complain about disturbances 
 of taste, even though the sensitiveness of the organ of taste for the elemen- 
 tary qualities is normal and though odors are recognized by the nose 
 (gustatory anosmia). 
 
 Congenital defects of the olfactory nerve also occur. The fundamental 
 factors of senile anosmia, and of that which sometimes remains after nasal 
 disease, are still unknown. 
 
 2. The Optic Nerve 
 
 The diseases of the optic nerve of interest to the neurologist are those 
 which occur most often as part of the symptomatology of certain cerebral and 
 spinal diseases. Isolated diseases of the optic nerve, as a rule, fall to the lot 
 of the eye specialist. Nevertheless, especially in the interest of differential 
 diagnosis, a survey of the general field, which we shall give in the following 
 paragraphs, is necessary to the neurologist. 
 
 The optic nerve, in the morphologic embryological sense, is not a per- 
 ipheral nerve, but a projected portion of the brain. Of especial significance 
 for an understanding of its diseases is the fact that the fibres serving to give 
 the keenest central vision, belonging to the yellow spot (macula lutea), form 
 in the nerve trunk a large separate bundle. This papillo-macular bundle 
 first occupies, near the bulbus, a peripheral position corresponding to the 
 temporal border of the disc, while further on it runs in the axial part of the 
 transverse section. 
 
 The chief symptom of disease of the optic nerve is clearly disturbance 
 of vision of various forms, which, however, differ from those occurring in 
 affections of the chiasm, the optic tract, and the more central parts of 
 the visual tract, which are distinguished by their peculiar hemianopic 
 character, and which disease of the optic nerve does not produce. (Con- 
 cerning hemianopsia, cf. the chapter on brain diseases.) 
 
 In serious disease of an optic nerve the eye concerned, almost without 
 exception, does not react to light by contraction of the pupil. Of more 
 theoretical interest than practical importance is the fact that in very rare 
 cases, where total blindness resulted from atrophy of the optic nerve, the 
 optic nerve concerned was said to have transmitted pupillary light reflexes. 
 According to this it would seem that special fibres subserving the reaction to 
 light could withstand, on occasion, the morbid processes longer than the 
 tracts destined for vision, though even in these cases the pupillary light 
 reaction never was totally undisturbed. 
 
 In most diseases of the optic nerve ophthalmoscopic changes of a charac- 
 teristic nature are known to take place in the disc of the optic nerve. 
 
108 DISEASES' OF THE PERIPHERAL NERVES 
 
 For various reasons a classification of optic-nerve diseases is very difficult. 
 Concerning the pathological anatomy there is no unanimity among the most 
 experienced investigators, and still less clear in many respects is the patho- 
 genesis. The same visible changes in the fundus are occasioned by various 
 conditions, and, on the other hand, the same injury may occasion quite 
 different anatomic and ophthalmoscopic pictures. Finally, the ophthal- 
 moscopic findings change considerably in various stages of the same process. 
 
 The following classification will suffice for all practical purposes, though 
 it is necessarily lacking in unity. 
 
 {a) Primary {Idiopathic) Atrophy 
 
 Idiopathic atrophy is diagnostically a very important symptom, because 
 it often appears very early, of tabetic and paralytic diseases. It will be 
 treated more minutely in its relation to those diseases in the proper place. 
 Clinically it is characterized, besides the ophthalmoscopic findings of an 
 always bilateral, simple diffuse atrophy, by a more or less severe, usually 
 irregular, but concentric contraction of the field of vision. The sense of 
 color suffers likewise (at first for red and green), and so does central vision. 
 The fibres of the optic nerve disappear, and likewise very early their cells 
 of origin in the retina. Simultaneously with the atrophy, there gradually 
 develops a disturbance in sight, which usually progresses till total blindness 
 results. 
 
 The atrophy of the optic nerve in glaucoma, which is of purely oculistic 
 interest, may always be distinguished definitely from tabo-paralytic atrophy 
 by ophthalmoscopic examination. 
 
 The atrophy of the optic nerve in the Tay-Sachs amaurotic family idiocy 
 is probably the result of a serious disease of the retina. The ophthalmo- 
 scopic picture, the circumstances and the symptoms accompanying its 
 appearance are so characteristic that it never presents any difficulty in 
 differential diagnosis. 
 
 The secondary atrophy after papillitis (neuritic atrophy) will be discussed 
 in the following paragraphs. It, too, is differentiated from "simple" 
 atrophy by the peculiar ophthalmoscopic findings, which will in most 
 cases enable its recognition and differentiation, especially in the early 
 stages in which the differential diagnosis of the affection of the optic 
 nerve is of prime importance for a decision as to the fundamental morbid 
 condition. 
 
 The primary tabetic and paralytic atrophy may most easily be con- 
 founded with certain secondary simple atrophies, which appear most fre- 
 quently in connection with retrobulbar processes of the most varied nature. 
 They are treated of under (c) and (d), where reference is made to the peculiar 
 characteristics of the different forms in this group. 
 
DISORDERS OF THE OPTIC NERVE 109 
 
 (b) The Forms Occurring under the Aspect of Papillitis, Especially Choked 
 Disc and Primary N euro-papillitis , Neuritis Optica Intraocularis 
 
 In its incipient stage the prognosis of a papillitis may present considerable 
 difficulties. When the physiological tortuosity of the vessels of the retina 
 is strongly marked, and the disc is hyperemic, as is habitually the case in 
 hypermetropia, it is really a case of pseudo-neuritis optica. The concentric 
 wrinkling of the retina in the neighborhood of the disc, which is occasionally 
 observed in the beginning of true papillitis, may make a differential diagnosis 
 possible. 
 
 1. Choked disc occurs as a result of space contracting intra-cranial 
 processes, or, to be more precise, of such conditions as prevent the free flow 
 of the venous and lymphatic circulations in the cavity of the skull. This 
 causes trouble in the area of one or usually of both optic nerves, which is 
 the immediate cause of the development of choked disc. Disease processes 
 in the orbit, too, may in rare cases lead to analogous, though, of course, 
 unilateral, alterations. Choked disc will be treated in more detail with the 
 different intra-cranial diseases especially with brain tumor. From choked 
 disc it is not always easy by means of an ophthalmoscope clearly to dis- 
 tinguish the next affection. 
 
 2. Primary Neuritis Optica Intraocularis. — The histological and 
 pathogenic comprehension of choked disc in its more important manifesta- 
 tions is not yet very definite, and this holds true, perhaps even to a higher 
 degree, in the case of neuritis optica. In the latter, it is usually a case of 
 inflammatory processes, while choked disc, at least in the early stage, 
 shows only congestive symptoms, especially edema. As a primary inde- 
 pendent disease, neuritis optica has been traced back to all sorts of sources, 
 frequently on very questionable grounds. Its occurrence in connection 
 with various infectious diseases, intoxications (lead), and with intra-cranial 
 infectious inflammations, is certain. It is supposed that it may be directly 
 produced by anemic conditions and severe hemorrhage, Worthy of mention 
 is a hereditary form, usually appearing at puberty in males, and being 
 transmitted through the female line. 
 
 3. Papillitis in inflammation of the retina (albuminuric retinitis et al.), 
 changes of the disc in thrombosis of the central vein and in embolism of the 
 central artery, have a decided differential diagnostic interest for the 
 neurologist. 
 
 All forms of papillitis are accompanied by more or less serious dis- 
 turbances in vision. These may recover or result in so-called secondary 
 neuritic atrophy, which ophthalmoscopically is characterized by certain 
 peculiarities. 
 
 In choked disc, vision more frequently remains normal for a considerable 
 time. 
 
no DISEASES OF THE PERIPHERAL NERVES 
 
 (c) Diseases Beginning in the Retrobulbar Region (Retrobulbar Neuritis) 
 
 In cases of this group, the disorder begins with a destructive influence 
 acting upon that section of the optic nerve which is retrobulbar. Hence 
 the first clinical symptoms are disturbances in vision, sometimes total 
 blindness, sometimes peripheral defects in the field of vision, sometimes 
 disturbance of central vision without impairment of the peripheral parts of 
 the field of vision, so-called central scotoma, which indicate an isolated dis- 
 ease of the papillo-macular bundle, for which, according to the latest investi- 
 gations, an anatomic predisposition to peculiarities in the supply of vessels 
 of this area, is apparently to be held responsible (Birch Hirschfeld). 
 
 According to the place, nature and mode of attack of the influencing 
 injuries, ophthalmoscopic changes occur in a few cases relatively early, as 
 neuritis optica, which may be caused by a descending inflammation, or 
 choked disc. In such cases the disorder may terminate in atrophy of the 
 disc. 
 
 In many cases, however, the visible changes of the papilla of the optic 
 nerve occur only later, when, according to the extent and gravity of the dis- 
 ease, it appears as a more or less marked, usually only partial, especially often 
 temporary, simple atrophic discoloration. 
 
 Retrobulbar optic-nerve disease is as a rule unilateral. 
 
 From the facts stated, the necessary general basis for differential diagnosis 
 becomes apparent. 
 
 Not considering the rare focal diseases, such inflammatory processes as 
 occur in myelitis and meningitis, and the metastatic abscesses of the optic 
 nerve which are sometimes observed, the following diseases must be con- 
 sidered chiefly. 
 
 (i) Diseases due to pressure. The effect of long continued pressure on 
 the nerve trunk, occurring in tumors in the immediate vicinity, in aneurisms 
 of the internal carotid or ophthalmic artery, in old age perhaps even in 
 simple arterio-sclerosis of these vessels, usually is simple atrophy. 
 
 Constant pressure on the chiasm (tumors of the hypophysis) or the optic 
 tract may also lead to descending atrophy. Sight disturbances in these cases 
 generally are hemianopic; many diseases of the chiasm even lead to total 
 blindnesss. 
 
 In cases of very slowly increasing pressure, it may occur that disturbances 
 in sight will not be noticed until visible atrophy has set in, which is often 
 easily confounded with simple atrophy. 
 
 (2) The tumors of the optic nerve itself, of which many primary and 
 metastatic forms occur. As a rule they lead to papillitic processes. 
 
 (3) Other orbital diseases of all kinds may encroach upon and affect the 
 optic nerve. 
 
 (4) The affections of the optic nerve in diseases of the posterior accessory 
 
CAUSES OF OPTIC NEURITIS, TOXIC AMBLYOPIA m 
 
 cavities of the nose, of the sphenoid and ethmoid cavities, which stand in 
 close anatomic relation to the optic trunk. 
 
 (5) Malformations .of the skull, especially the so-called tower-shaped 
 head, frequently, even in infancy, lead to disease of the optic nerve. 
 
 (6) Injuries of the optic nerve caused directly by gunshot or knife wounds, 
 and more frequently by fracture of the skull, show naturally all degrees of 
 lesion up to the complete rupture of the nerve. In the beginning the disc may 
 be hyperasmic or edematous; in ruptures of the central vessels it presents the 
 appearance of grave anaemia. Very often the ophthalmoscopical picture is 
 at first quite normal. The outcome is always an atrophy, usually simple, 
 corresponding in degree to the injury. The atrophy of the optic nerve 
 which is sometimes observed in small children after delivery by means of 
 forceps, may also be included here. 
 
 In some rare instances, after cranial trauma, bilateral atrophy of the optic 
 nerves has gradually developed, which clinically has usually taken the same 
 course as primary tabo-paralytic atrophy. 
 
 (7) Diseases of the optic nerve in multiple sclerosis. 
 
 Multiple sclerosis very frequently leads to the development of focal lesions 
 in one or both optic nerves. The peculiarity of this disease to leave the axon 
 intact for a comparatively long time, explains some singularities in the clinical 
 picture of this disease of the optic nerve. Disturbances of vision may be 
 entirely wanting, even in cases in which an atrophy ophthalmoscopically 
 proven, most commonly with its characteristic picture of temporal pallor, 
 exists. In the course of the further development of the malady, slight dis- 
 turbances in vision may still set in. They rarely, if ever, develop to such a 
 degree as to result in marked diminution of vision, let alone blindness. 
 
 Not infrequently acute disturbances in vision are observed in the early 
 stage of the disease, mostly of one eye, as a rule in the form of central sco- 
 toma, occasionally with transient papillitic changes, and in exceptional cases 
 even choked disc. These sight disturbances are generally more or less com- 
 pletely recovered from. They are probably coincident with the early stage 
 of a sclerotic focus in the optic nerve. 
 
 (d) Toxic Amblyopias and a Few Related Forms of Acute Blindness 
 Originating Peripherally 
 
 That many different intoxications may lead to the development of papil- 
 litis has already been mentioned. The toxic amblyopias occupy a unique 
 position with respect to these affections. Under this name acute and chronic 
 diseases are grouped together. 
 
 The toxic amblyopia of chronic development, observed most frequently 
 in chronic tobacco and alcohol poisoning, may be considered as one of the 
 retrobulbar diseases which we have just discussed, though it is as a rule 
 
ii2 DISEASES OF THE PERIPHERAL NERVES 
 
 bilateral. Concerning its pathological anatomy and pathogenesis, nothing 
 conclusive is known, but the clinical picture indicates that the papillo-macular 
 bundle is chiefly affected. The disturbance in vision is a characteristic 
 scotoma, first for red and green, later on for white, while the field of vision 
 otherwise remains normal. In the later stages, a slight pallor, especially of 
 the temporal part of the optic nerve head may set in. Mistaking it for in- 
 cipient tabetic atrophy can be easily avoided by observing carefully the 
 characteristic functional disturbances. The prognosis is relatively favorable; 
 that of the tabetic atrophy, unfavorable. 
 
 Bilateral toxic amblyopias and amauroses of peripheral origin setting in 
 acutely occur in many acute poisonings, among which, those due to quinine, 1 
 male fern, and methyl alcohol should be mentioned. They have been also 
 observed to result from prolonged use of atoxyl. Very similar clinical 
 pictures may be produced by severe hemorrhage, and, after employment of 
 X-rays or other rays of somewhat similar character. They are occasioned 
 by grave, acute diseases of the ganglionic retinal nerve cells, and often of the 
 fibres themselves. A partial restoration is possible but the usual termi- 
 nation is a more or less complete atrophy of the optic nerve. 
 
 What produces the grave disease in the optic apparatus in these and other 
 cases in which general injurious influences are at work, is not known. 
 
 The therapeutics of the diseases of the optic nerves must proceed 
 according to fundamental principles. In cases of atrophy, in which amelio- 
 ration, or at least a cessation of the process, does not seem impossible, galvanic 
 treatment should be tried, though the recent optimistic reports concerning 
 the success of such treatment need to be more fully verified. 2 The anode is 
 placed upon the closed eyelid, the cathode, which should be larger, upon 
 the neck. Strong currents, carefully increased and diminished, and pro- 
 tracted seances (15 minutes and longer) are recommended. Sometimes long 
 continued treatment with strychnine appears to be useful. 
 
 3. Nerves of the Eye Muscles. Oculomotor, Trochlear and 
 
 Abducens Nerves 
 
 The palsies resulting from disease of the motor nerves of the eye, in a great 
 number of cases can, practically not, be distinguished with certainty from those 
 resulting from intra-cerebral injury of the same peripheral motor neuron. 
 Even theoretically, in a large group of cases, it can not be authoritatively 
 stated whether they may be considered as peripheral nerve disease or not. 
 
 Palsies due to progressive nuclear (spino-bulbar) muscular atrophy, 
 cases of polioencephalitis haemorrhagica superior, probably most cases of 
 congenital palsies caused by imperfect development of the nuclei which 
 often assume the character of supranuclear paralysis, and finally the paraly- 
 
 1 The minimum dose that has occasioned disturbances in vision, is 5 grm. in 30 hours. 
 
 2 Cf . Mann, Zeitschrif t fur diatet. und physic. Therapie, volume VIIL 
 
OCULAR PALSIES 113 
 
 ses occurring in focal disease of the crusta, either injuring the nuclei them- 
 selves or affecting the intra-cerebral root-bundle sympathetically (fascicular 
 paralyses), all these can be traced back to nuclear or at least to intra-cere- 
 bral injuries or diseases. 
 
 In contrast to these forms, which are mentioned here only for the purpose 
 of differential diagnosis, stand others which most certainly are peripheral 
 nerve diseases, i. e., the large group of basal and orbital paralyses. Among the 
 diseases which cause paralysis by lesion of the nerves, acute and chronic men- 
 ingitis from whatever cause, and especially syphilitic chronic basal meningitis, 
 must be first mentioned. Injury to the nerves caused by the pressure of tumors 
 must also be considered. These tumors may be situated in the meninges 
 themselves, though more frequently those of the base of the skull (sarcomata), 
 and above all, tumors of the brain are the cause of the paralyses through 
 pressure. It is very important to notice, that tumors of each brain region 
 may occasionally cause ocular paralysis. Tumors in the region of the pos- 
 terior cranial fossae most frequently injure the abducens; those of the middle 
 cranial fossae, especially of the temporal lobes, may lead to grave and extensive 
 ophthalmoplegia through pressure on the nerve trunks, which are closely 
 interwoven at the base of the brain, as is the case with the tumors of the 
 frontal region when they spread towards the superior orbital fissure. In 
 the cases named, the mechanism of the pressure effect is clearly understood. 
 But sometimes there are cases where it is very difficult to understand the 
 relation between a brain tumor located at some distance from the base, and 
 some coincident forms of slighter paralyses in the eye muscles. Neoplasms 
 very rarely directly involve the nerves. It is exceedingly seldom that tumors 
 arise on the nerves themselves (primary and metastatic). 
 
 Besides morbid growths, arterio-sclerotic changes in vessels, aneurisms 
 of the internal carotid and other arteries, can injure the nerve trunks through 
 pressure. 
 
 Like tumors of the temporal lobes, abscesses of the same region affect the 
 nerves of the base. 
 
 Thrombosis of the cerebral sinuses, particularly of the sinus cavernosus, 
 frequently lead to paralysis of the eye muscles. 
 
 Of diseases of the orbit, it is mainly inflammation and tumors or tumor- 
 like structures that play an aetiological role. 
 
 Of the traumatic palsies, those resulting from fracture of the base of the 
 skull are of the greatest interest. 
 
 Trauma and syphilis may likewise in rare cases lead to paralyses from 
 intra-cerebral injury or disease. The trauma may cause hemorrhages in the 
 nuclear region, while syphilis is likely to induce chronic progressive 
 disease of the nuclei. 
 
 Those cases that now and then are observed in connection with ophthalmic 
 herpes zoster are probably also of neuritic origin. 
 
ii 4 DISEASES OF THE PERIPHERAL NERVES 
 
 For an exceedingly large group of paralyses, as has been said before, no 
 definite classification which would serve for all cases can be made, since the 
 known pathologic-anatomical findings vary, at least enough to make differ- 
 ent explanations possible. This is the case with paralysis of the eye muscles 
 in tabes and paresis, in multiple sclerosis, and in the cases connected with 
 infectious and toxic diseases. Among the infectious diseases, diphtheria and 
 influenza stand in the front rank (cf . also chapter on polyneuritis) . Among 
 the toxic processes, sausage poisoning (botulismus) and lead poisoning deserve 
 to be mentioned. 'The cases observed in Basedow's disease and diabetes 
 most likely depend on autointoxication. How in all these cases there 
 results a localized affection of special eye muscles is entirely unknown. 
 Very uncertain is the astiological significance of catching cold or being chilled, 
 so often affirmed. In many cases all discoverable causes are lacking. 
 
 Clinical Conditions and the Course of the Disease. 
 
 Paralyses of the eye muscles appear, as is evident from the aetiological 
 discussion, as component symptoms of the most varied conditions. How- 
 ever, they appear also as an independent disease. 
 
 In either case the affection may be restricted to one of the cranial nerves 
 or to a nerve pair. Isolated oculomotor and abducens palsies are frequent; 
 isolated trochlear palsies rare. The trochlear is more frequently affected in 
 combination with other ocular nerves. 
 
 In oculomotor affections, total paralysis and paresis affecting all branches 
 of the nerve must be distinguished from partial functional disturbances, 
 restricted to a few single muscles of its innervation area. 
 
 In total oculomotor paralysis the mobility of the eye is practically gone. 
 As the abducens and trochlearis are functionally unimpaired, the eye de- 
 viates considerably outward and a trifle downward. Every attempt to 
 innervate motorially the ball is apt to increase this deviation. The eye is 
 diverted still further into the outer canthus, while, because the trochlearis 
 still acts, the upper pole of the vertical meridian of the cornea is at the same 
 time turned slightly inward. The increase in deviation due to an attempt at 
 innervation, no matter how directed, must be looked on as a so-called com- 
 pensation movement and is explained thus : that an innervation current flows 
 off into the open channels, since its own are closed. The upper eyelid is 
 dropped and can be raised only passively. The pupil is more than medium 
 wide, the contraction reactions are lacking, the accommodation is paralyzed. 
 Often there co-exists a slight degree of exophthalmus. The eye, since its 
 muscular fixedness is lacking, usually almost protrudes from the orbit. 
 
 Partial oculomotor paralysis may affect each muscle separately or an 
 irregular group of muscles. To the most frequent occurrences of this nature 
 belongs isolated paralysis of the levator palpebral (ptosis). Important 
 
OCULOMOTOR PARALYSIS 115 
 
 typical combinations are the so-called external oculomotor paralyses, in 
 which only the external branches of the nerve are paralyzed, 1 and the con- 
 trasting ophthalmoplegia interna, in which the paralysis is confined to the 
 internal muscles of the eye. It is of special importance to know the condi- 
 tions in convalescence of the latter, and of great practical interest, too. As a 
 rule, first the palsy of the ciliary muscle decreases, and the accommodation 
 naturally returns, while the pupil does not contract either to light rays or by 
 convergence. (A condition known as pupillary immobility, "Pupillenstarre.") 
 In the further course the convergence reaction returns before the light reac- 
 tion, so that in this stage it is easy to confuse this condition with that of 
 purely reflex pupillary immobility, which, diagnostically, is so extremely 
 significant. For the differential diagnosis it is important first to note that 
 ophthalmoplegia interna frequently occurs unilaterally, while the true 
 Argyll-Robertson phenomenon, on the other hand, occurs unilaterally only 
 in exceptional cases. In residual ophthalmoplegia interna, the pupil as a rule 
 is of an average or slightly larger width, while the reflex pupillary immobility 
 often combines with miosis. The most important point is, that in reflex 
 pupillary immobility the convergence reaction always remains absolutely 
 normal. In the spurious form slight residua of a disturbance can always be 
 found, even in regard to the convergence reaction. 
 
 Besides thus grouping these cases according to the extension of the para- 
 lytic symptoms in the different eye muscles, a further classification according 
 to their varying courses is necessary. We know of cases setting in acutely, 
 frequently comparatively mild, even transient forms; further of chronic pro- 
 gressive, and finally of relapsing and periodically reappearing cases. 
 
 Whether a paralysis starts acutely, whether its course is mild and regress- 
 ive or continuing, is, of course, dependent upon the nature of the influence, 
 and the reparability of the causal anatomic process. Those ("rheumatic") 
 paralyses which some writers have traced to colds, are famed as mild cases, 
 and the paralyses associated with herpes zoster ophthalmicus ought to per- 
 mit of a favorable prognosis. The palsies occurring in tabes and multiple 
 sclerosis, after lasting a short time, may disappear completely and forever. 
 
 On the other hand, however, these very ophthalmoplegias of tabes, 
 general paralysis and multiple sclerosis may take a chronic progressive 
 course. Several others, certainly the nuclear forms of the disease taking a 
 like course, need not be specially discussed here. 
 
 The recurring form of paralysis of the eye muscles occurs, too, in tabes 
 and paresis. A second group of recurring paralyses, which actually take a 
 periodic course, and which are almost invariably restricted to an oculomoto- 
 rius, may, according to anatomic findings, have their origin in pressure of 
 tumors on the nerve trunk, or in chronic meningitic and arterio-sclerotic 
 
 1 Strictly speaking, ophthalmoplegia externa totalis should be assumed only when all external 
 eye muscles including, of course, those innervated by the abducens and trochlearis, are affected. 
 
n6 DISEASES OF THE PERIPHERAL NERVES 
 
 processes taking place in the immediate vicinity of the nerve and injuring it. 
 The duration of the exacerbations, and the periods of remission and inter- 
 mission will naturally be very different in the various cases. At the time of 
 aggravation, a headache on the side affected manifests itself, sometimes 
 accompanied by vomiting, in consequence of which, it has become common 
 to speak of it as "migraine ophthalmoplegique " from a certain superficial 
 resemblance in clinical pictures. These conditions, nevertheless, have noth- 
 ing in common with genuine migraine. However, it is said that even in 
 genuine migraine some transient attacks of eye-muscle palsy occur. 
 
 Diagnosis. — The first problem, though by no means always the easiest 
 one, is to find out definitely whether a paralysis of the eye muscles exists at 
 all, and what muscles it affects. For this the reader is referred to the dis- 
 cussion in the general section dealing with diagnosis. 
 
 In the second place we must try to discover whether it is a supranuclear 
 or a peripheral paralysis proper, using that term in its widest sense. Finally, 
 and of most significance to us here, we are confronted by the most difficult, 
 only too often insoluble, question: which portion of the peripheral neuron is 
 the primarily diseased part — is it the nucleus, the intra-cerebral root-fibres, 
 the intra-cranial or the orbital part of the peripheral nerve ? 
 
 At first we must say a few words concerning the differentiation of dis- 
 ease of the peripheral neurons from the supranuclear or looking-paralysis 
 (Blicklahmiing), a problem the solution of which needs special tests. 
 
 Generally speaking, in diseases of the peripheral neuron only, it is a 
 matter of a true paralysis of the muscles, while in supranuclear lesions only 
 certain functions of the muscles disappear. Only in peripheral paralysis 
 is the muscle equally cut off from all inflowing nervous impulses. A supra- 
 nuclear focus, on the other hand, interrupts only those pathways which 
 convey to a certain group of synergists the impulses for a certain common 
 function, so that the latter is suspended, while the same muscles may remain 
 normal for other functions, and open to inflowing innervation from other 
 sources. Thus the "looking motion" (Blickbewegung) towards one side 
 may be palsied, while the co-affected internus is active in convergent motion. 
 A supranuclear lesion, however, may also be restricted to the fibres which 
 convey the impulse to one of the synergists. In that case, only this one 
 muscle is affected, but only for the function concerned, without therefore 
 being incapacitated for other functions. In such cases, just as in peripheral 
 paralysis, disturbances in equilibrium and double images manifest them- 
 selves with each innervation of the synergism, which in one of its parts is 
 injured. Decisive for the differential diagnosis as to the supranuclear seat 
 is, therefore, the proof that the paralytic symptoms appear only in certain 
 functions, while the same muscles for other functions remain normal. One 
 must consequently make different tests. First, the patient is commanded 
 to look in this or that direction, or to fix with his eye an object located 
 
FOCAL DIAGNOSIS OF OCULAR PARALYSIS 117 
 
 within the peripheral part of his field of vision. Besides these "com- 
 manded" movements, one should test the so-called reflex eye movements. 
 In the first place, the patient is made to follow with his eyes a moving 
 object — which he has previously fixed — in all directions; and secondly, he 
 is made to hold with his eyes a fixed object located in the primary direction 
 of his vision, while his head is passively turned in all directions. In the 
 particular case of a paralysis of an internus for the lateral looking move- 
 ments, his convergence function should also be examined, and vice versa. 
 In the majority of cases, these methods will suffice to determine the above 
 mentioned characteristics of a supranuclear paralysis. In other cases 
 different criteria are required. It is conceivable that because of complicated 
 lesions the reflex as well as the "commanded" movements may be syn- 
 chronously suspended, while the peripheral neuron is not diseased. In 
 that case, the strictly symmetrical character of a paralysis may serve as 
 evidence of its supranuclear origin. An absolutely symmetrical paralysis 
 of two or more synergists, in which no disturbance in the muscular equi- 
 librium could be detected, does not occur, aside from cases of total paralysis, 
 in peripheral diseases. It is evident, however, from the above, that the 
 converse need not be necessarily true, i. e., every paralysis that affects only 
 one synergist, or several unevenly, is not by reason of this a peripheral 
 palsy. 
 
 If these criterions be heeded, the neuron diagnosis will in most cases be 
 successful. To decide more exactly in what part of the peripheral neuron 
 the seat of the lesion is to be looked for, and particularly to decide whether 
 it is a question of a disease of the peripheral nerve, the following points 
 deserve consideration. In an isolated disease of the abducens or trochlear, 
 this decision can naturally be made only in those cases, where accompanying 
 symptoms render possible the diagnosis of the fundamental anatomic 
 process and its seat. Even for oculomotor paralysis this is frequently the 
 surest and often the only practicable way. 
 
 It was assumed formerly, that, excluding cases due to orbital disease, 
 the paralyses of single muscles supplied by the third nerve, were all caused 
 by nuclear disease. At present we know that disease of the nerve trunk 
 may lead only to paralysis of one or a few muscles, and, indeed, that this 
 occurs not infrequently. Isolated ptosis can, in numerous cases, be traced 
 to disease of the nerve trunk. Partial disease of the trunk, for example 
 scattered foci of disease, have been proved anatomically in a number of 
 such cases. The picture of external oculomotor paralysis may surely be 
 brought about in this way. There is no accurate explanation for it. It is 
 doubtful whether a unilateral external oculomotor paralysis may result 
 from nuclear disease. The fibres proceeding from the nuclei of both 
 oculomotor nerves are subjected to a partial crossing, so that each nerve is 
 composed of crossed and uncrossed fibres. Just how both are distributed 
 
n8 DISEASES OF THE PERIPHERAL NERVES 
 
 to the various muscles, is a question which has not yet been finally settled. 
 The clinical symptoms . which follow the destruction of one oculomotor 
 nucleus are not as yet known in detail. This is responsible in part for the 
 great difficulties confronting us in the task with which we are here concerned. 
 Only this may be said with certainty, that a total paralysis of all branches 
 of the oculomotorius will have to be localized distally from the partial 
 crossing of the fibres, in the majority of cases, basally. 
 
 Our knowledge concerning the localization of ophthalmoplegia interna 
 is also very incomplete. It is pointed out that a connection of an ophthal- 
 moplegia interna with a paralysis of the inferior oblique muscle indicates 
 orbital disease, since the short root of the ciliary ganglion which contains 
 the oculomotor fibres of the inner eye muscle originates from the nerve 
 branch destined for the inferior oblique. 
 
 Unfortunately we know of no other pathognomonic classification of the 
 symptoms, which would be even partially accurate for certain places in the 
 peripheral pathway. When several different eye muscles are affected 
 simultaneously, it denotes with certainty a basal or orbital seat. The cases 
 taking a chronic progressive course most frequently are the result of nuclear 
 disease; those taking an acute and mild course, and the relapsing and 
 periodic cases, are generally due to peripheral nerve disturbance. 
 
 The differentiation of certain myopathic ophthalmoplegias from neuro- 
 pathic forms is frequently possible only by taking into consideration the dis- 
 ease picture as a whole. (Cf. especially the chapter on myasthenia.) 
 Ophthalmoplegia interna, occasioned by the effect of a blow on the eye- 
 ball, is probably myopathic. 
 
 The prognosis of an ocular paralysis should always be made with great 
 reserve. Above everything else the fundamental disease must be considered. 
 Even in those cases in which it is possible to diagnose the pathologic-anatomic 
 process and to recognize it as remediable, it will not always be certain whether 
 the changes this has occasioned in the nerve are likewise adjustable. Con- 
 cerning their nature and gravity a decision is frequently impossible. But 
 only the inveterate cases and those taking a chronic progressive course are 
 hopeless from the beginning. A favorable prognosis is possible in the acute 
 cases, occurring in connection with infectious diseases and with a number of 
 toxic diseases (botulism). Some other important points for the prognosis 
 have been mentioned in preceding paragraphs. 
 
 Therapy. — There can really be no question of special therapeutics for 
 paralyses of the eye muscles. Whenever possible the treatment should be 
 aimed at the fundamental disease. In all other respects one should proceed 
 according to principles laid down for the treatment of paralyses in general. 
 The use of the stabile cathode on the closed eyelids of the diseased eye should 
 be tried. The inactive electrode is placed on the neck. From an authori- 
 tative source comes the suggestion that a few sweat baths be prescribed in 
 
THE TRIGEMINAL NERVE id 
 
 those acute cases, which presumably are due to an infectious cause or ex- 
 posure to cold. 
 
 Later, exercises to improve vision, and in the residual stage oculistic and 
 especially operative measures for the adjustment of the disturbances of 
 vision, will have to be considered. 
 
 4. The Trigeminal Nerve 
 
 The trigeminal nerve arises, like a spinal nerve, from the central organ 
 with an anterior motor and a posterior sensory root. The posterior root 
 enters the Gasserian ganglion. Distally from it the motor root unites with 
 the third of the sensory nerve branches proceeding from the ganglion ; the 
 first two of these nerve branches consequently remain purely sensory. 
 Besides combined disease of both roots, there may be disease of the sensory 
 part alone. Cases of purely motor trigeminal paralysis (masticatory facial 
 paralysis) are also on record. Besides the affections of the chief trunk, 
 paralyses of single branches have been observed. 
 
 Trigeminal paralysis usually makes its appearance as a partial symptom 
 of other disease pictures; it very rarely occurs isolated. 
 
 Its most frequent cause is basal disease. In basal syphilis, in tumors of 
 the base of the brain or skull, and after fracture of the skull, one may occa- 
 sionally observe isolated trigeminal paralyses, though even in these cases it is 
 seen more frequently in conjunction with palsies of other cranial nerves. 
 
 Of diseases of single branches, that of the ramus ophthalmicus, due to 
 morbid changes in the superior orbital fissure or inside the orbit, is observed 
 most commonly. 
 
 In a later chapter it will be shown that neuralgia may be followed by 
 symptoms of lost function in single trigeminal branches. 
 
 During the last few years, extirpation of the Gasserian ganglion in grave 
 cases of neuralgia has furnished repeatedly an opportunity for studying the 
 symptoms of trigeminal paralysis. 
 
 A lesion of the motor part gives rise to paralysis of the muscles of masti- 
 cation. It may be both seen and felt how the temporal and masseter mus- 
 cles on the affected side fail to contract, either wholly or partially, when 
 chewing and biting, and how they gradually atrophy. The lower jaw reflex 
 is also abolished on that side. The lower jaw, as a result of the paralysis of 
 the pterygoids, can not be moved towards the healthy side, it tends rather, 
 during chewing, to deviate towards the affected side. In bilateral paralysis 
 the jaw hangs down loose; it can be lifted only with the hand and an habitual 
 dislocation may develop. 
 
 Paralyses of the other muscles supplied by the motor trigeminus (mylo- 
 hyoid, anterior belly of the digastric, tensor tympani and tensor veli palati) 
 do not occasion very significant symptoms. 
 
 The disturbances of sensation affect, according to the seat of the lesion, 
 the whole innervation area or that of single branches. For a better under- 
 
120 DISEASES OF THE PERIPHERAL NERVES 
 
 standing of the clinical pictures and particularly of the rather far reaching 
 adjustability or possibility of compensation, it is exceedingly important to 
 know, that the innervation areas of the right and left nerves, the areas of 
 the single branches among themselves, and finally the areas of the trigeminus 
 and that of the neighboring cervical nerves may overlap one another 
 extensively. 
 
 It should further be remembered, that the innervation area of the 
 trigeminus is not confined to the skin of the face. Of those parts that can be 
 examined, the first branch supplies the cornea and conjunctiva as well as the 
 anterior and upper part of the nasal cavity; the second, the remaining part 
 of the nasal cavity, the upper jaw and the mucous membrane of the palate 
 and upper lip ; the third, the lower jaw and the mucous membrane of the 
 cheek, the tongue and the lower lip. 
 
 Besides the sensory symptoms of loss of function, pains frequently occur, 
 which, especially in compression of the nerve trunk, may be neuralgic and 
 be projected into the peripheral extension area. 
 
 Together with the conduction of sensibility, the conjunctival and corneal 
 as well as the sneezing and palatal reflexes, which are mediated by the sensory 
 trigeminus path, are interfered with. The functions of the lachrymal and 
 salivary glands, especially those of the former, suffer from the loss of a great 
 part of the reflex irritations, that stimulate their secretion. As a result of 
 this and of other disturbances in the region of the smaller mucous glands, 
 there occurs a certain dryness of the conjunctiva and the mucous membrane of 
 the nose, which may even in some cases, it is said, impair the sense of smell. 
 The real secretory nerves of the glands mentioned are not, however, generally 
 affected. A glance at the diagram (Fig. 28) will show that these fibres do 
 indeed mingle with branches of the trigeminus that are directed towards the 
 periphery and that in the last part of their course they take the same course as 
 these branches. Lesion of them in the trigeminus area would therefore be 
 possible only if those terminal branches should be subjected to an injury. 
 As a rule, then, a true paralysis of glandular secretion does not occur, and it 
 may be expected that the psychic emotional secretion especially will go on 
 quite undisturbed. 
 
 Fig. 28 gives information also concerning the relation of the peripheral 
 pathway of taste to the trigeminus. 
 
 It must even be emphasized here, and we will be confronted by it again 
 when studying facial paralysis, that without doubt individual variations in the 
 course of the taste fibres occur and, indeed, it seems that their relations to the 
 trigeminus are less regular than those comparatively constant ones to the 
 facial nerve. The fibres emanating from the anterior two-thirds of the 
 tongue enter almost regularly the lingual nerve, which they soon leave, 
 however, in order to enter the facial trunk with the chorda. They leave 
 it in the vicinity of the geniculate ganglion, mostly via the large superficial 
 
THE TRIGEMINAL NERVE 121 
 
 petrosal, enter thereafter into the second trigeminal branch and with the 
 latter reach the chief trunk. But after careful observation it must be 
 admitted that there is a possibility of the small petrosal taking over, in a few 
 cases, the gustatory fibres of the chorda from the facial trunk and taking 
 them to the trigeminal trunk by means of the third branch. This covers 
 the most important possibilities of a taste disturbance in trigeminal disease. 
 On the other hand it would seem certain that a lesion of the trigeminal trunk 
 need not necessarily lead to disturbances of taste. In such cases it is 
 assumed that the fibres of the chorda after their usual course reach the 
 
 I. trigeminus 
 
 lingualis u pubmaxittarip 
 
 Fig. 28. — Scheme of the relations between the trigeminal, the facial and the glosso-pharyngeal. 
 
 Taste fibres; Secretory fibres for sublingual and submaxillary glands; + + + + 
 
 Secretory fibres for tear glands. {Simplified from Koster.) 
 
 glosso-pharyngeal trunk by anastomosis through the facial and with it enter the 
 brain. That the chorda should not contain any taste fibres at all, may 
 occur as a great rarity. In that case they either reach the trigeminal trunk 
 directly through the lingual nerve or the lingual contains no taste fibres and 
 the glosso-pharyngeal innervates the entire half of the tongue directly. 
 So far we have spoken only of the gustatory innervation of the anterior 
 half of the tongue, usually effected through the chorda. The taste nerves of 
 the posterior part of the tongue, which usually belong to the lingual branches 
 of the glosso-pharyngeal, do not, perhaps, always take their further course to 
 the brain through the glosso-pharyngeal trunk, but sometimes, by means of 
 the Jacobson anastomosis (the tympanic, resp. the small superficial petrosal) 
 
122 DISEASES OF THE PERIPHERAL NERVES 
 
 also enter the trigeminal trunk. This is corroborated by the experience of 
 aurists in cases of lesion of the tympanic nerve, in connection with the 
 hemiageusia occurring occasionally in diseases of the trigeminal trunk. 
 
 Finally, it would seem that occasionally the chorda supplies the whole 
 half of the tongue with taste fibres. 
 
 In view of the great variability of the clinical pictures, the discussion of all 
 these probabilities could not be omitted. The careful study of each new 
 individual case is urged for the sake of acquiring a better and clearer insight 
 into these complicated conditions. 
 
 Of greatest interest among the trophic disturbances are herpes zoster, 
 to be discussed in the chapter on neuralgia, and the so-called neuroparalytic 
 keratitis. The more or less serious inflammations in corneal anaesthesia, 
 which are borne, however, with remarkable ease, must certainly be closely 
 related to external injuries, from which the cornea, on account of its insen- 
 sibility, is not sufficiently protected. Nevertheless the foremost authorities 
 agree that the pathogenesis of this keratitis is still a very complicated prob- 
 lem. Furthermore not every corneal anaesthesia leads to keratitis. 
 
 Diagnosis. — Only the differential diagnosis needs to be discussed 
 particularly. 
 
 The motor disturbances, when occurring isolated and without accom- 
 panying defects of sensibility, may make the diagnosis difficult. 
 
 The myopathic conditions of weakness and paralysis of the muscles 
 of mastication — such as are found in myasthenia, in very rare cases in the 
 common forms of muscle dystrophy, but occur more regularly in the advanced 
 dystrophies of those affected with Thomsen's disease — manifest themselves 
 bilaterally. The characteristic general aspects of the affections in question 
 will always facilitate the diagnosis. 
 
 The central, supranuclear palsies of the masticatory muscles affect both 
 sides. They are accompanied by exaggerated reflexes. R. D. is, of course, 
 always absent. The disturbance of mastication is a partial symptom of 
 the typical general picture of pseudo-bulbar paralysis. Therefore the 
 diagnosis is comparatively easy in these cases also. 
 
 Difficulties in differentiation may arise in cases of nuclear and intra- 
 cerebral radicular paralyses, which bear the character of peripheral paralysis, 
 without having any features of their own, to distinguish them from diseases 
 of the peripheral nerves. Such cases occur in focal diseases of the brain- 
 stem, in polioencephalitis, in bulbar paralysis and in tabes. In practice, 
 consideration of all the symptoms together will show in most of even these 
 cases, that a peripheral nerve disease is out of the question. 
 
 The disturbances of sensation, which in their distribution correspond 
 exactly to the innervation area of the trigeminus or one of its main branches, 
 indicate at once a disease of the peripheral nerve. The fibres entering 
 the pons are distributed over the long cell column of the sensory nucleus 
 
TRIGEMINAL PARALYSIS. THE FACIAL NERVE 123 
 
 of the trigeminal which extends down to the cervical spinal cor4- If a 
 focal lesion within the central organ affects part of this extended root area, 
 there result disturbances in sensibility of a peculiar, segmental character; 
 namely, stripe-like anaesthesias, which are arranged concentrically like the 
 layers of an onion about the middle of the face. Such a zone always belongs 
 to the areas of several peripheral nerve branches. An anaesthesia of an 
 entire trigeminal area through intra-cerebral lesion can be thought of only 
 in those cases in which a focus has affected the fibres already united in a 
 bundle, immediately before their exit from the pons. It is furthermore of 
 value to know, that in a few cases of pontile focal trouble, anaesthesias have 
 been observed which corresponded almost exactly to those of an area of 
 an ophthalmic nerve. 
 
 The anaesthesias in diseases of the cerebrum and in hysteria are never 
 confined very strictly to a trigeminal area. 
 
 There can be no question of a special prognosis or therapeutics in 
 trigeminal paralysis. 
 
 5. The Facial Nerve 
 
 The facial nerve perhaps more often than any other peripheral nerve is 
 affected alone. 
 
 Of eminent importance in the aetiology of facial palsy is exposure to 
 cold. Many authors attribute almost three-quarters of all cases to it. To 
 be sure, patients remarkably often declare that, for instance, they looked 
 out of the window in a heated condition, thus exposing the face to cold 
 draughts, and that on the next day the facial palsy appeared, etc. But 
 it is questionable, to say the least, in how many of these cases a cold 
 is the direct cause of the disease. Recent investigations seem to favor the 
 assumption that in not a few cases an acute, if slight, catarrh of the middle 
 ear may be the connecting link between cause and effect. 
 
 Be that as it may, in any case the diseases of the middle and inner ear 
 and of the petrous portion of the temporal bone are among the most frequent 
 and most important causes of facial palsy. The thin bone, which divides 
 the nerve canal from the middle ear, in many cases is defective in one or 
 more places, so that the nerve is in contact with the mucous membrane of 
 the tympanum. It is obvious from this, that even very slight disease of the 
 tympanum, which both the patient and the physician are very apt to over- 
 look, may lead to paralysis — a fact which in every case should prompt a 
 careful expert examination of the ear. It must also be remembered that 
 the palsy may continue even after the inflammation of the ear has entirely 
 ceased. Serious and particularly purulent inflammations, and carious 
 processes on the petrous portion of the temporal bone may injure the nerve 
 in various ways. 
 
i2 4 DISEASES OF THE PERIPHERAL NERVES 
 
 In the third place, traumatic causes must be mentioned. At times, 
 the facial is injured in an operation upon the ear, or the nerve has to be 
 sacrificed in some indispensable radical operation. Other typical cases of 
 traumatic facial palsy are those due to fracture of the skull, and the obstetric 
 palsies of newly born infants, in which cases, it is usually an injury of extra- 
 cranial branches occasioned either by pressure of the mother's pelvis, or by 
 the forceps of the physician. The peripheral terminal arborizations of the 
 nerve over the face suffer lesions very frequently from cuts and blows. 
 
 The last setiological group of importance contains those palsies occasioned 
 by basal diseases, among which meningitis of all kinds and new growths 
 in this region play the chief role. 
 
 Of less frequent occurrence are lesions of the nerves in cases of adjoining 
 inflammatory diseases located outside the cranium, such as parotitis. 
 
 In many cases facial palsy has been ascribed to some preceding infectious 
 disease, such as diphtheria and influenza, to toxic processes, and to metabolic 
 diseases, especially diabetes. A recent syphilis is said to predispose to 
 facial palsy. (Tertiary syphilis causes basal palsies by meningitis.) Fre- 
 quently, however, investigation does not reveal such predisposing factors. 
 Herpes zoster of the trigeminal area may be accompanied by a facial palsy. 
 
 It is questionable, whether hereditary neuropathic affections have any 
 significance. Some rare observations seem to support the theory of predis- 
 position in an entire family to facial palsy. Of the relapsing forms we shall 
 speak later. 
 
 Facial palsy appears as a congenital malformation, usually affecting both 
 sides, frequently associated with palsy of the eye muscles and with other 
 malformations. In the majority of cases it is doubtless due to intra-cerebral 
 disease. The accompanying disturbances in the oculomotor apparatus may 
 bear the character of a supranuclear or a "looking" paralysis. Like 
 myopathic palsy, nuclear facial palsy, in which very likely must be included 
 that occurring in cranial tetanus and tabes, is here of interest only for the 
 purposes of differential diagnosis. 
 
 Clinical Picture 
 
 In a recent facial palsy, all mobility in the affected half of the face is more 
 or less abolished. As a result of the flaccidity and paralysis of the muscula- 
 ture, the wrinkles on the affected side are smoothed out, the forehead is 
 smooth, and the nasolabial crease not very marked. The corner of the mouth 
 and the lower eyelid hang down a little. The mouth, as a whole, is slightly 
 drawn toward the opposite side. The palpebral fissure is wider than on the 
 sound side. In every expressive movement, wrinkling the brows, showing 
 the teeth, pursing the lips, laughing, etc., only the sound side is innervated. 
 When attempting to close the eyelids, the upper lid is lowered a trifle on 
 
PERIPHERAL FACIAL PALSY 
 
 125 
 
 account of the flabbiness of the levator palpebral, but it is impossible com _ 
 pletely to close the eyelids, and the palpebral fissure remains half open 
 (lagophthalmos). In this attempt, the upward rotation of the ball, physio- 
 logically associated with the impulse to close the lid, becomes plainly 
 visible. In healthy individuals this can be observed only by mechanically 
 preventing the closing of the eyelids, when the subject is strongly trying to 
 close them (Bell's phenomenon). It may be remarked in passing, that 
 sometimes the closing of the lids will be more complete during sleep than the 
 patient could ever produce while awake; and, on the other hand, that some- 
 times the fissure is larger during sleep than during a strong volitional attempt 
 
 Fig. 29. — Paralysis of the facial at the right 
 side during rest. (Leipsic Medical Clinic.) 
 
 Fig. 30. — Paralysis of the facial at the right 
 side when trying to whistle. (Leipsic Medical 
 Clinic.) 
 
 to close them. One may usually convince one's self of a unilateral palsy of the 
 platysma by asking the patient to draw his under lip as far as possible down- 
 ward. The paralysis of the posterior auricular branch coursing to the ear 
 muscles and to the occipital muscle, and of the branches destined for the 
 stylohyoid and the posterior belly of the digastric, manifest, as a rule, no 
 symptoms worthy of mention. 
 
 The direct consequences of the disturbance of the mobility in unilateral 
 facial palsy are of little importance from a practical standpoint. The patient 
 can not whistle; when he attempts to blow out a candle, the air escapes, with 
 but feeble pressure, through the corner of the mouth on the affected side. 
 If there is an abundant secretion of saliva, the imperfect closing of the mouth 
 
126 DISEASES OF THE PERIPHERAL NERVES 
 
 may become noticeably annoying, as the saliva may flow out of the mouth 
 on the affected side. 
 
 Of much greater importance is a lasting lagophthalmos, which regularly 
 causes the eye to weep considerably, quite often results in an obstinate con- 
 junctivitis and once in a while even leads to graver inflammations of the eye. 
 
 In the not very rare bilateral paralysis (diplegia facialis), the motor 
 loss is immediately and very painfully perceived. Speech is very much 
 impaired by the loss of the labial sounds. The patient cannot expectorate 
 and can blow only feebly. The flabbiness of the cheeks, which in chewing do 
 not touch the teeth, makes eating difficult. The saliva cannot be properly 
 retained in the mouth, but flows out over the lower lip. The entire face 
 is rigid like a mask, and because of the drooping of the lower eyelids and lips, 
 exceedingly distorted (Fig. 31). 
 
 Coincident with the symptoms of facial palsy, disturbances in secretion of 
 perspiration frequently manifest themselves, in which case, both hyperhi- 
 drosis and anhidrosis may occur. This alone makes it evident that a strict 
 parallel does not exist between the injury of motor fibres and those taking the 
 same course which subserve the production of perspiration. No diagnostic 
 interest attaches to these manifestations. The hyperhidrosis will usually 
 be noticed when chewing, especially when chewing foods containing acids. 
 
 We now have to consider some accompanying symptoms, which mani- 
 fest themselves only if the facial is injured in certain places in its course. 
 (Cf. diagram on p. 121, Fig. 28.) 
 
 From the point where the facial nerve leaves the brain, secretory fibres 
 subserving the lachrymal glands take their course through the nerve trunk 
 to the geniculate ganglion, where they leave it through the large superficial 
 petrosal nerve. A lesion of the facial nerve within the limits just outlined 
 regularly leads to disturbance in the secretion of tears, causing either a 
 decrease or cessation, or even a morbid increase of the secretion (Koster). 
 
 As this symptom is diagnostically important a short account of the technic 
 for examination follows. Strips of about 20 cm. in length and 1 cm. in 
 width are cut from blotting paper and one end of each turned down a trifle 
 making a hook. By means of these hooks one strip is hung into the con- 
 junctival sac of each of the two lower eyelids, which should first be somewhat 
 dried in order to remove the too ample secretions of the mucous membrane, 
 and their gradual permeation is then awaited. The lachrymal glands may 
 be stimulated to action by properly inserting a small brush well up into each 
 nostril. This test is continued, until the strips, which when fully per- 
 meated should be exchanged for fresh ones, are no longer permeated, in other 
 words, until the lachrymal glands are "pumped out." The functional 
 capacity of both sides is then judged according to the length of the per- 
 meated space on the strips. Minor differences (up to 3 or 4 cm. of the blot- 
 ting paper) occur even in normal conditions. Now, in facial palsy we find 
 
■PERIPHERAL FACIAL PALSY 
 
 127 
 
 in the corresponding localized cases on the affected side a cessation or a 
 large decrease, or an excessive increase, in the secretion of tears. If a marked 
 difference between both sides is at once obvious, say if the secretion should 
 be wholly lacking on one side, the test need not be so protracted, but in 
 doubtful cases the "pumping out" which may take an hour or more, must 
 be patiently awaited. Worthy of mention is the fact that the eye on the side 
 affected with paralysis may weep, even when the secretion of the lachrymal 
 gland has ceased. The comparatively tenacious secretion of the palpebral 
 conjunctiva, however, is sufficient 
 only for permeating at the most about 
 1 cm. of the strip of blotting paper. 
 
 The older investigators assumed 
 that motor fibres, too, left the facial 
 nerve by way of the large superficial 
 petrosus, to enter the palate. Today 
 we know that the facial does not in- 
 nervate the palate. Consequently a 
 paralysis of the palate is not a part of 
 the clinical picture of facial paralysis. 
 The former erroneous notion can 
 partially be accounted for by the fact 
 that the oblique position of the uvula, 
 which is frequent even in healthy 
 persons, was falsely regarded as a 
 sign of a unilateral palsy of the 
 palate. 
 
 Taste fibres which have been in- 
 troduced into the facial more distally, 
 through the chorda tympani, leave it 
 through the large superficial petrosal, 
 coursing to the second trigeminal branch. In an affection of the facial nerve 
 in its course between the geniculate ganglion and the junction of the chorda, 
 disturbances of taste are evidenced, corresponding to the innervation area of 
 the chorda, in the anterior two-thirds of the half of the tongue on the same 
 side. 
 
 It must be mentioned, though it does not materially affect the practical 
 worth of the above statements, that in very rare cases taste innervation 
 takes a somewhat different course. It occasionally happens that the 
 chorda fibres innervate gustatorily the entire half of the tongue; but it has 
 also been noted, that in spite of the destruction of the chorda, taste disturb- 
 ances were wholly lacking. 
 
 In the area in which the chorda fibres course in the nerve trunk a very 
 fine small branch, whose function is not known and which anastomoses with 
 
 Fig. 31. — Bilateral congenital paralysis of 
 the facial. Attempt to close the eyes. Mal- 
 formation of the ears. (Leipsic Medical Clinic) . 
 
128 DISEASES OF THE PERIPHERAL NERVES 
 
 the small superficial petrosal nerve and a motor twig, taking its course to- 
 wards the stapedius muscle, are separated. It would therefore have to be 
 assumed, that the symptoms of paralysis of this muscle exist in every facial 
 paralysis situated above the point of separation of its twig. Indeed, in- 
 creased auditory sensibility, keenness of hearing (hyperakusia), has been 
 sometimes regarded as a symptom of stapedius palsy. But this symptom is 
 so inconstant, so difficult to determine correctly, and so questionable in its 
 meaning, that one has to relinquish it for all purposes of determining the 
 diagnosis. 
 
 Fibres for the submaxillary and sublingual glands take their course 
 down the nerve trunk to where the chorda tympani leaves,. In conformity 
 with this, in many instances, disturbances of the secretion of these glands 
 are observed, manifesting themselves chiefly in a decrease, though some- 
 times in an increase in the secretion of saliva. For determining these things, 
 we can only avail ourselves of the very inadequate method of asking the 
 patient to raise his tongue, to dry the floor of his mouth and then to examine 
 and compare the two sublingual carunculae. 
 
 More pronounced trophic and vasomotor disturbances are very rare. 
 Sensory disturbances do not, as a rule, enter into the clinical picture of 
 facial paralysis. 
 
 According to recent investigations the chorda is supposed to contain, 
 besides the fibres of taste, a few sensory fibres, whose further course to 
 the central organ is unknown. It is true that occasionally a very slight 
 deadening of the sensibility is detected in the innervation area of the chorda dur- 
 ing facial paralysis. Perhaps slight disturbances of the sensibility in the area 
 of the skin of the face would, with proper attention, be detected more fre- 
 quently than, according to general belief, they occur. In such instances 
 it is questionable whether it is always a true complication with trigeminal 
 disease, or whether sensory fibres may not also enter into the facial trunk. 
 Peripheral anastomoses between the sensory and motor facial nerves are 
 known to exist and central relations between the facial roots and the bulbar 
 terminals of the trigeminal fibres have been reported. 
 
 Besides disease of the chief trunk, paralyses of single branches also occur. 
 Cuts and blows in the face are their most frequent cause. In its course 
 through the middle ear, the chorda is sometimes affected alone as a result 
 of an otitis media. It is occasionally destroyed in the course of an opera- 
 tion. As a result, corresponding disturbances of taste and of the secretion 
 of saliva set in. Isolated paralyses of the large superficial petrosal have 
 been observed after operative lesions in which the Gasserian ganglion has 
 been extirpated; the scars following such procedure may also injure the 
 nerve. We have already noted in basal syphilis the characteristic symptoms 
 of its isolated affection — taste disturbances in the chordal area and cessa- 
 tion of lachrymal secretion. 
 
THE COURSE OF PERIPHERAL FACIAL PALSY 129 
 
 The Course of the Disease 
 
 The prodromal symptoms which are sometimes observed, may be 
 traced back in part to the original disease, such as fever, headache and ear- 
 ache; some of them, such as taste paresthesia, slight clonic spasms, some- 
 times perhaps even pains in the face, point to a lesion of the nerve as already 
 present. 
 
 Usually the palsy sets in acutely. A gradual onset may sometimes occur, 
 most frequently in basal compression of the nerve. 
 
 The course of the disease, in the majority of cases, is favorable; the 
 mobility of the palsied area is restored more or less completely. In many 
 cases the restoration of the palsied area is not at all uniform. Some muscles 
 are more completely and sooner able to perform their functions than others. 
 The course of the other functional disturbances does not always correspond 
 precisely to that of the palsy. The disturbances in the secretion of perspi- 
 ration accompanying facial paralysis, are, as stated above, inconstant from 
 the very first; they may be either paralytic or irritative and are frequently re- 
 stored rather quickly. Similar conditions prevail as regards disturbances 
 of the salivary secretion — though, it must be added, only in so far as we are 
 able to judge. They, too, are inconstant, even when the lesion takes place 
 in the area through which the fibres that excite the glands take their course, 
 and they also may be restored before the paralysis. Taste disturbances 
 are often only partial, appearing to affect only a part of the taste qualities, 
 and they too may disappear before the palsy. One gains the impression, 
 that possibly the taste function quite like that of sensibility in general is, 
 in diseases of peripheral nerves, less subject to injury. 
 
 Recovery in the lighter cases is usually complete, in grave cases usually 
 defective. The contractures and associated movements manifesting them- 
 selves in cases in which the restoration has been only partial are more trouble- 
 some than the residuum of the palsy. 
 
 The facial contracture is conditioned upon a peculiar shrinking process 
 of the paretic muscles, thus distinguishing itself from other contractures in 
 peripheral palsy, which are produced by the shortening of antagonists not 
 affected by the paralysis. 
 
 The anatomic-physiological peculiarities of the muscles innervated by 
 the facial, which to a great extent lack even a somewhat fixed point of inser- 
 tion and true antagonists, may explain the exceptional position they occupy 
 in this relation. 
 
 The contracture precisely reverses, in the later stages, the clinical picture 
 of grave facial palsy. The wrinkles and creases on the affected half of the 
 face, which were formerly smoothed out, become more and more clearly 
 defined, and become even deeper than those on the healthy side of the face. 
 The palpebral fissure becomes narrower, the corner of the mouth is lifted 
 9 
 
i 3 o DISEASES OF THE PERIPHERAL NERVES 
 
 up, the mouth as a whole is distorted towards the affected side. Seeing a 
 patient in this stage of the disease, one is inclined to regard the healthy side 
 as the paretic one. Of course, the functional test at once enlightens one. 
 
 The contracture is quite regularly attended by associated movement 
 symptoms, which, however, also occur in the regenerative stage of cases 
 recovering without contractures, associated movements in different muscles 
 of the diseased half of the face, which accompany the involuntary droop- 
 ing of the eyelid, and by apparently spontaneous twitchings, that pass over 
 the face with lightning rapidity and which in reality are probably no more 
 than associated movements due to this paralytic drooping of the eyelid, 
 which may very easily be overlooked. Quite similar twitchings in the 
 morbid area have been observed when bringing a hand close to the face 
 quickly, when tapping it and when irritating electrically various places on 
 both the sound and the affected sides: the so-called reflex twitchings. It 
 certainly is not a matter of a genuine exaggerated reflex. The newer theory, 
 according to which it is, in all these cases, a question of associated movement 
 in closure of the eyelid, has many points in its favor. Also in voluntary 
 innervation of the different parts of the facialis area, associated movements 
 appear in other parts. In explaining all these associated movements 
 it is very plausibly held, that in the regeneration of the degenerated nerve 
 the fibres newly arising from every part of the nucleus, did not all find their 
 old muscle area, but distributed themselves among various muscle areas, 
 in consequence of which in innervations of the former, the latter also contract. 
 This would also explain a peculiar failure in voluntary motility despite the 
 restoration of the electric irritability, since a muscle area might have again 
 received conductive and excitable nerve fibres, but not from its own nucleus. 
 Therefore this area can be irritated electrically through the nerve and 
 associated movements may also occur in this area, when innervating 
 other parts, but every attempt at purposive appropriate innervation will 
 fail. 
 
 The statement that in the regenerative stage of grave facial paralyses 
 an exceedingly great confusion in the innervation takes place, very aptly 
 expresses the actual conditions (Lipschitz). 
 
 In facial paralysis not infrequently relapses occur once or several times. 
 The same side, or even the one which formerly remained sound, is then 
 affected. 
 
 In deciding upon the prognosis, which in the majority of cases will be 
 favorable, besides the fundamental disease the condition of the electric 
 irritability must be carefully considered. This, though not invariably, 
 indicates even in the second and third week with certainty whether recovery 
 may be expected in a few weeks or only after several months. The prognosis 
 of grave paralyses will under all circumstances be ominously affected by 
 the associated movements, which will seldom be lacking, and by contracture. 
 
DIAGNOSIS OF FACIAL PALSY 131 
 
 It is often well to acquaint the patient with this prospect at any early date. 
 The danger of a relapse is, on the other hand, of less importance. 
 
 The diagnosis of facial paralysis is very easy. No careful investigator 
 will be deceived by natural differences in the two sides of the face, nor, if he 
 note the functional disturbances properly, will he incur the danger of mis- 
 taking the side affected with paresis for the sound one, if contractures exist. 
 Often the associated movements at once reveal the diseased side. 
 
 The real task of one who diagnoses such cases lies, first, in the distinc- 
 tion of peripheral nerve disease from nuclear, supranuclear and myopathic 
 paralyses, and secondly, in determining in what part of the course of the 
 nerve the lesion is situated. 
 
 The facial paralyses in myasthenia and in the different forms of muscle 
 dystrophy have quite characteristic peculiarities, to which reference is made 
 in the chapters on these diseases. As a rule they are strictly bilateral. The 
 reaction of degeneration (R. D.) is never present. The myasthenic or 
 dystrophic symptoms in other areas facilitate the diagnosis. 
 
 As a rule, though not always, in the supra-nuclear facial paralysis 
 which, by the way, is usually connected with hemiplegia, the upper facial 
 region, as a result of its bilateral cerebral innervation, remains more or less 
 unaffected. In cases where the paralysed side takes part in a normal way 
 in the emotionally released involuntary expression, as well as in those, where, 
 on the other hand, the facialis is paralysed only for emotional impulses, 
 there is clear evidence of a supranuclear seat of the lesion. Testing the re- 
 flex action in diagnosing a facial palsy, plays a subordinate part. Of all 
 the more significance may be the electric examination for distinguishing 
 supranuclear from peripheral paralyses. It is said that Bell's phenomenon 
 may be absent in a central facialis paralysis. Cases are on record, in which 
 — e. g., in diseases of the pons — a supranuclear palsy manifested itself on 
 one side, and a palsy occasioned by lesion of the peripheral neuron, on the 
 other. A peripheral paralysis may follow after a central one on the same 
 side, if a focal lesion extends secondarily to the nucleus or the nerve roots. 
 
 It is sometimes very difficult or even impossible to distinguish nuclear 
 and fascicular cases by themselves from basal-nerve diseases. The distur- 
 bances in the secretion of tears, peculiar to basal palsies, appears to be lacking 
 in cases due to central disease. Usually, nuclear palsies are accompanied 
 by other pathognomonic signs of disease of the central organ, or they appear 
 in such characteristic forms, that there can be no doubt about a nuclear 
 disease, as in the case of a bulbar paralysis, which takes a highly chronic, 
 slowly progressing course, affecting at first, only a few muscles fibre by fibre. 
 But even the congenital palsies, which probably depend on aplasia of the 
 nucleus (infantile nuclear atrophy) may be confined to parts of the facialis 
 area. 
 
 For a finer diagnosis of the place of the lesion in the area of a peripheral 
 
i 3 2 DISEASES OF TEE PERIPHERAL NERVES 
 
 nerve, the disturbances of taste and the secretion of tears are decisive. If 
 the lachrymal secretion alone is affected, the lesion is situated basally, at all 
 events above the geniculate ganglion. Whenever the focus is situated in 
 the area from which the large superficial petrosal takes its origin, disturbances 
 of lachrymal secretion and of taste will co-exist. Disease situated below 
 the geniculate ganglion as far as the point of origin of the chorda occasions 
 only taste disturbances. Distally from the point of origin of the chorda, 
 disturbances both of taste and of the lachrymal secretion are lacking. In 
 this area the paralysis frequently affects only single nerve-branches. 
 
 It should be remembered that the auditory nerve down to the geniculate 
 ganglion takes its course in close proximity to the facial. Synchronous 
 disease of the auditory nerve may therefore be significant for the topical 
 diagnosis of the facial paralysis. 
 
 It is evident from the above that in inveterate cases the diagnosis will be 
 rendered more difficult by the early disappearance of the taste disturbance. 
 The same is true of disturbances in the lachrymal secretion. 
 
 Therapeutics. — In palsy of the facial nerve the possibility of treating 
 the cause of the disease has to be considered first of all. In basal syphilitic 
 meningitis, treatment by inunction promises success. In ear diseases an 
 aurist should be consulted. In very recent "rheumatic" paralyses, some 
 experienced physicians recommend that one or more diaphoretic procedures 
 be instituted. 
 
 Furthermore, in the very first rank stands electro-therapeutics, applied 
 according to the general rules for all such treatments, but especially the 
 stabile cathodic galvanization of the nerve, and afterwards the irritant appli- 
 cations must be considered. Systematic exercises aided by the hand are to 
 be recommended. 
 
 The peculiar disturbances in the regenerative stage of grave cases, as 
 contracture and associated movements, place on the physician a difficult 
 and peculiar task. It is an altogether erroneous impression that the con- 
 tract are is caused by the electric treatment. Nor, to be frank, does it pre- 
 vent its occurrence. As soon as the first symptoms appear, careful massage, 
 with special regard to suitable manipulations for extension and flexibility 
 should be instituted. Needless to say, this treatment should be undertaken 
 only by a professional. To stretch the cheek and keep it extended, older 
 doctors gave the patient a wooden ball to carry under it. The use of elec- 
 tricity on the sound half of the face is quite futile. 
 
 To counteract the associated movements, the patient should be taught 
 to practise innervational exercises in front of a mirror. 
 , The lagophthalmos of the more serious kind should be kept in mind 
 from the start. If necessary, a protective eye bandage should be prescribed. 
 
 If an absolute paralysis is persisting in a case, and there is no longer 
 any hope of amelioration — this will not be the case after a few months — 
 
TREATMENT OF FACIAL PALSY. THE AUDITORY NERVE 133 
 
 grafting of the nerve should be resorted to. According to the rather scanty 
 and not particularly encouraging reports, joining the peripheral part of 
 the facial nerve with the central end of the hypoglossus, which has been 
 entirely divided for this purpose, can be recommended in preference to any 
 other method. The unilateral palsy of the hypoglossus which is naturally 
 produced thereby is less troublesome to the patient, and by a complete divi- 
 sion, instead of a mere freshening, the vexatious associated movements 
 between the two areas, which would otherwise set in after regeneration, are 
 avoided. One should not hope for too much from this operation. For more 
 detailed information cf. Lipschitz, Monatsschrift f. Psychiatrie and Neur., 
 vol. xx, Ergaenzungsheft, page 84, and Bernhardt, Mitteil aus den Grenz- 
 gebieten, vol. xvi, page 476. 
 
 In the residual stage, plastic operations have now and then brought about 
 a cosmetic and functional betterment. Lagophthalmos may necessitate a 
 tarsorrhaphy. 
 
 6. The Auditory Xerve 
 
 The greater part of what we know concerning diseases of the auditory 
 nerve, we owe to aurists. The following exposition will show how impor- 
 tant it is that this nerve should not be neglected, as is often the case, in neuro- 
 logical examinations. 
 
 The auditory nerve becomes diseased either independently, primarily, 
 or is affected secondarily in diseases of the structures that surround it. 
 
 The primary diseases are tumors, simple atrophic or degenerative-neu- 
 ritic processes. Among the so-called tumors of the cerebello -pontile angle a 
 great number emanate from the auditory nerve itself. The auditory nerve 
 atrophies in tabes, where it is even an early symptom, and in senility 
 (presbyacusia) . Primary neuritis, comparable to neuritis optica, is observed 
 especially in infectious and toxic processes. Among poisonings those from 
 quinine and salicylic acid, alcohol and tobacco deserve to be mentioned. 
 Practically more important are perhaps those cases of neuritis acustica which 
 are caused, as has been experimentally demonstrated, by acoustic trauma 
 or by the long-continued effect of loud noises (professional deafness of 
 smiths, gunners). Other causes are very rare (thunderclap), or entirely 
 problematic ("rheumatic" origin). Among the paralyses of the cranial 
 nerves accompanying herpes zoster, a supposedly neuritic disease of the 
 auditory nerve also occurs. 
 
 Secondary disease of the acoustic nerve we find in affections at the base 
 of the brain, particularly in basal syphilitic meningitis, in disease of the 
 temporal bone (caries) and especially in that of the labyrinth. Slight 
 labyrinthine irritation accompanies various diseases of the middle and even 
 of the outer ear. Suppurative infections of the labyrinth occur in connection 
 with meningitis (during cerebro-spinal meningitis, after measles and scarlet 
 
i 3 4 DISEASES OF THE PERIPHERAL NERVES 
 
 fever). Frequently they occur during infancy and later become the cause 
 of deaf-mutism. Besides this, the labyrinth may be purulently infected 
 from the middle ear. Traumatic injuries of the labyrinth are usually the 
 result of indirect traumas (fracture of the skull) . When caisson workers 
 suddenly leave the place in which there is an increased air pressure, hemor- 
 rhage and gas-embolisms may result. Spontaneous hemorrhages have been 
 observed in persons of hemorrhagic diathesis. In leucaemia, hemorrhage 
 and leucocytic infiltrations occur. In affections of the heart the possibility 
 of an embolus in the labyrinthine artery will have to be borne in mind. 
 Frequently syphilis, especially late hereditary syphilis, is the cause of 
 labyrinthine disease. Concerning the so-called choked labyrinth (corres- 
 ponding to the choked disk) scarcely anything is known. 
 
 The exact situation and anatomic nature of the pathologic changes 
 which occur sometimes in the auditory nerve after mumps are still in doubt. 
 
 Clinical Picture. — The auditory nerve is affected alone or in associa- 
 tion with other cranial nerves. The lesion affects either both branches or 
 only one, either the vestibular or cochlear nerve. Symptoms of irritation 
 and of paralysis often appear simultaneously. 
 
 Diseases of the cochlear nerve lead to nerve deafness, the diagnostic sepa- 
 ration of which from sound conduction deafness, occasioned by diseases 
 of the middle ear, particularly if both forms be combined, is not always easy 
 and frequently requires the aurist's utmost skill. The following symptoms 
 are characteristic of disease of the auditory nerve. Complete deafness of 
 one ear never occurs without disease of the nerve itself. It is a peculiar 
 fact that partial disturbances of hearing affect especially the high tones, 
 that the limit of acoustic perception is "narrowed from above," while the 
 deepest tones may be perceived normally — an occurrence which never takes 
 place in disturbances of sound conduction. In other cases peculiar "tone- 
 gaps" are produced, certain parts of the tone range are lacking, which is 
 also important in regard to differential diagnosis. The symptoms of acous- 
 tic irritation are manifold; somewhat characteristic are those taking place 
 in the area of high tones, as the distressingly high ringing and buzzing in the 
 ear. From tests with the tuning fork, which is held now in front of the ear, 
 now on the skull, the following results are obtained: The duration of the 
 perception in conduction by means of the skull is shorter when contrasted 
 with the normal, but never, except in a preponderating complicated disease 
 of the middle ear, lengthened (Schwabach's test). The relation between 
 the length of perception by air and by bone conduction remains normal 
 and the tuning fork, which is heard no longer by bone conduction, is still 
 able to excite an auditory impulse if quickly held in front of the ear (positive 
 effect of Rinne's test) . The tuning fork applied to the crown will be heard 
 especially in the sound ear (lateralization towards the healthy side in the 
 otherwise rather unreliable Weber's test). 
 
DIAGNOSIS OF DISEASES OF THE AUDITORY NERVE 135 
 
 Diseases of the vestibular nerve lead particularly to disturbances of the 
 equilibration of the body. 
 
 When one vestibular apparatus is acutely destroyed, say by hemorrhage 
 into the labyrinth, a sudden attack of vertigo supervenes, in which the 
 patient is prostrated, vomits, sometimes faints, usually is deafened more or 
 less completely in one ear at the same time, in consequence of the participa- 
 tion of the cochlear nerve, and is tormented by violent buzzings (Meniere's 
 symptom complex). The patient has a sense of being twirled in a swing 
 or turned round and round. He often complains about apparent movements 
 of the objects he sees. Nystagmus towards the healthy side exists, caused 
 or increased by directing the look that way. When, after some time, the 
 patient tries to stand up he falls towards the affected side. 
 
 After a few days his condition is improved. The complaint usually 
 disappears entirely after a longer time. The defect becomes latent, even a 
 bilateral loss in the vestibular apparatus is gradually adjusted through func- 
 tional compensation, and thereby becomes latent. 
 
 In chronic diseases repeated brief attacks of Meniere's symptom complex 
 may occur; the attacks are usually to be considered a symptom of irritation. 
 The nystagmus is generally directed towards the affected side. A pro- 
 nounced inclination to fall towards a certain side is rarely present. In 
 other cases a more or less continuous feeling of dizziness is experienced, 
 which only at times leads to a real attack of vertigo and in still others the 
 symptoms may vary considerably. The attacks are then rudimentary and 
 perhaps only slight, rather subjective than objective, fits of giddiness may 
 manifest themselves. 
 
 When chronic disease has led to the gradual atrophy of the vestibular 
 nerve apparatus, the complaints will disappear (stage of latent vestibular 
 defect) . 
 
 The diagnosis of disease of the vestibular nerve may, as is evident from 
 the above, be difficult not only in the latent stage but also in some chronic 
 cases. Though the fully developed seizure is typical, nevertheless it is 
 sometimes extremely difficult to define the attacks and complaints in the 
 rudimentary stage. Recently different methods for systematically testing 
 the functional capacity of the vestibular nerve have been devised. The 
 test for caloric nystagmus appears to be especially valuable. When syring- 
 ing an ear with warm or cold water, in normal functioning of the vestibular 
 nerve, nystagmus in the same or in the opposite direction takes place. By 
 means of this procedure and other similar ones, it is apparently possible to 
 diagnose both destructive and irritative conditions of the nerve with much 
 more certainty now than heretofore. The test depends upon the absence 
 of, or the ease with which, caloric nystagmus can be produced. For more 
 information concerning these methods, a fuller study of which is recom- 
 mended, see Barany, Physiologie und Pathologie des Bogengangapparats, 
 
136 DISEASES OF THE PERIPHERAL NERVES 
 
 Leipsic and Vienna, 1907, and Politzer, Lehrbuch des Ohrenheilkunde, 5 
 edition, Stuttgart, 1908. 
 
 In disease of the auditory nerve the cochlear and vestibular nerves are not 
 always affected in the same degree. The primary diseases, with the excep- 
 tion of tumors, frequently confine themselves particularly to the cochlear 
 nerve and usually appear bilaterally. The majority of the secondary diseases, 
 especially the labyrinthine affections, affect, as a rule, both the cochlear 
 and vestibular nerves, but generally appear only unilaterally. What is 
 known as mumps deafness, is, for the most part, unilateral and is unaccom- 
 panied by any symptoms from the vestibular area. 
 
 The prognosis in most forms of neuritis acustica is not unfavorable, 
 but in almost all other conditions rather gloomy quoad restitutionem. 
 Many diseases dependent upon acquired syphilis can be cured with specific 
 treatment. 
 
 When disturbances of the apparatus for the perception of sound and of 
 the vestibular nervous apparatus are found, one must consider with refer- 
 ence to differential diagnosis, besides the diseases of the peripheral nerves, 
 also those of the intra-cerebral continuations of the auditor}'- and vestibular 
 pathways. An uncomplicated and complete clinical picture of an attack of 
 Meniere's disease does indeed indicate unconditionally a peripheral affection. 
 Otherwise, for a more minute topical diagnosis one must ask oneself, whether 
 the accompanying symptoms point to the petrous portion of the temporal 
 bone, to the base, the pons, or the cerebellum, as the seat of disease. 
 
 It is to be hoped that new and definite symptoms will be found in the 
 future, by means of the new diagnostic method presented above, for the 
 differentiation of peripheral and central lesions of the vestibular pathways. 
 
 The cochlear nerve of each side is, in the brain, immediately connected 
 with the bilateral central auditory pathway, so that, excepting pontile focal 
 lesions, which affect the zone of the root entrance directly, central auditory 
 disturbances are always bilateral in origin. Experience would seem to 
 show that only bilateral focal lesions can cause any serious auditory 
 disturbances. 
 
 In closing, let us once more emphasize how desirable it is, that in future 
 the disturbances caused by the eighth cranial nerve be carefully regarded 
 even in neurological examinations. Even today they play a decisive r61e 
 in the diagnosis of the tumors of the cerebello-pontile angle. They may be 
 of value in an early diagnosis of tabes, and in examinations of the victims 
 of accidents one will sometimes by this routine be enabled to decide, that 
 besides any functional nervous consequences, organic injuries as well may 
 have been caused by the trauma. 
 
 As to the treatment, the neurologist should avail himself, as far as 
 possible, of the services of an aurist; after that he should proceed according 
 to general principles. Especially to be mentioned is the frequent beneficial 
 
THE GLOSSO-PHARYNGEAL AND VAGUS NERVES 137 
 
 effect of the stabile anodic galvanization in cases of sensory irritation. The 
 button-shaped, active electrode (10 qcm.) is placed on the tragus. 
 
 7. The Glosso -pharyngeal Nerve 
 
 Concerning the diseases of the glosso-pharyngeal, but little definite is 
 known. Isolated paralyses of this nerve have been scarcely ever observed. 
 Up to the present time it has only been found affected in multiple basal- 
 cranial nerve disease. 
 
 Almost invariably the lingual branches contain the taste fibres for the 
 posterior third of the tongue. But not in every case do these fibres reach 
 the brain in the glosso-pharyngeal trunk, for they may leave it near the 
 petrous ganglion together with Jacobson's nerve and course to the trigeminal 
 with which they then take their further course to the brain. On the other 
 hand, however, it must be assumed that in some instances the taste fibres 
 of the chorda for the anterior two-thirds of the tongue are conveyed from the 
 facial trunk, not, as is usual, to the trigeminal, but to the glosso-pharyngeal 
 by means of an anastomosis. Finally the possibility of the glosso-pharyngeal 
 innervating, in exceptional cases, one whole side of the tongue can not be 
 lightly disregarded. (Cf. Fig. 28, and the statements made concerning 
 taste innervation in connection with the discussion of trigeminal and facial 
 palsy.) The relation of this nerve to the function of taste is therefore 
 undoubtedly inconstant. 
 
 For the parotid gland the glosso-pharyngeal possesses secretory fibres. 
 They come into the gland by way of Jacobson's nerve, the otic ganglion and 
 its anastomosis with the third trigeminal branch. Observations of aurists 
 have revealed disturbances in the functioning of the parotid gland in lesion 
 of Jacobson's nerve inside the tympanic cavity. 
 
 Unquestionably the glosso-pharyngeal participates in the sensory inner- 
 vation of the pharynx and supplies also a part of the pharyngeal and palatal 
 musculature with motor fibres. We have no definite knowledge with regard 
 to the degree of this participation. 
 
 8. The Vagus (Pneumo-gastric) Nerve 
 
 ^Etiology of paralysis of the vagus and its branches. Basal diseases 
 usually injure the vagus together with its immediate neighbors, especially 
 the spinal accessory, glosso-pharyngeal and hypo-glossal nerves. In the 
 area of the jugular foramen, its gate of exit from inside the skull, it anas- 
 tomoses with the accessory which lies closest to it. It takes up the internal 
 branch of this nerve, and thereby receives a supply of motor fibres, which 
 may be vagus fibres in reality since, according to several investigators, they 
 emanate from the vagus nucleus. We have no certain knowledge regarding 
 the function of these fibres. 
 
138 DISEASES OF THE PERIPHERAL NERVES 
 
 Isolated affections of the vagus always indicate a lesion outside the 
 cranium. 
 
 In the neck, gunshot wounds or those due to sharp instruments, opera- 
 tive injuries, lesions through pressure by glands, malignant tumors and 
 carotid aneurisms, may all lead to palsies of the nerve trunk. But also the 
 single branches, especially the important recurrent nerve, may become 
 diseased in this area either uni- or bi-laterally, e. g., through tumors of the 
 thyroid gland and new growths in the esophagus. 
 
 There are, besides, intrathoracic affections which cause paralysis of the 
 vagus, especially of the recurrent nerve. The left recurrent winds itself 
 around the arch of the aorta; the right, from the front backwards, around 
 the subclavian artery. Hence the frequent injury particularly of the left 
 nerve in aortic aneurisms will be clear. Left sided recurrent palsy, in steno- 
 sis of the mitral valve, can usually be explained by the effect of pressure 
 of the greatly distended left auricle. 
 
 Mediastinal tumors, both primary and metastatic, pleuritic and peri- 
 carditic conditions and chronic-pneumonic affections may injure the vagus 
 or recurrent by direct destruction, or by pressure or cicatricial contractions. 
 
 Lastly, there are cases of affection of the vagus for which no local cause 
 can definitely be assigned. Most of these cases depend upon toxic or infec- 
 tious processes. Most frequently we find palsy in the innervation area of 
 the vagus after diphtheria, at times as a partial symptom of polyneuritis, 
 and at times isolated. Nor can it be denied that sometimes direct injury 
 of the nerve through the diphtheritic inflammation, perhaps a kind of ascend- 
 ing neuritis, occurs (Cf. diphtheritic paralysis in chapter on polyneuritis). 
 
 To what extent vagus symptoms occurring in tabes depend upon per- 
 ipheral nerve disease, is doubtful. 
 
 The symptomatology is studied best from the point of view of disease 
 of the single branches of the nerve which are most frequently affected alone. 
 Symptoms of lost function are the most important and in disease of the main 
 trunk usually stand in the foreground. Paralysis of one recurrent nerve 
 produces motor paralysis of one vocal cord. In cases of slight lesions the 
 paralysis is at first almost always restricted to the dilator of the glottis, the 
 crico-arytenoideus posticus muscle (posticus paralysis). The vocal cord is 
 adducted into a position of phonation. Inspiratory abduction is lacking, 
 while the closure of the glottis on phonation remains unimpaired. In total 
 paralysis the vocal cord stands half abducted, in a medial position, the so- 
 called cadaveric-position, and is entirely immobile. The arytenoid cartilage 
 seems shifted somewhat to the front; the vocal cord somewhat shortened and 
 flaccid. In phonation the sound vocal cord may approach the paralyzed 
 One across the middle line, so that a fairly normal sound can be produced. 
 These are the two clinical pictures occurring in paralysis of the recurrent. 
 Other paralyses of single vocal-cord muscles, such as internus paralysis, 
 
SYMPTOMATOLOGY OF DISEASES OF THE VAGUS 139 
 
 paralysis of the interarytenoidei, are not a part of the picture of vagus paraly- 
 sis, but occur chiefly in grave catarrhs of the larynx. The participation of 
 the recurrent in the sensory innervation of the larynx is a question which 
 has not yet been definitely decided. 
 
 Vocal-cord paralysis in disease of the vagus trunk above the point where 
 the recurrent becomes separated, is always, as has been said, an important 
 partial symptom of the clinical picture. If, in cases in which the seat of the 
 lesion is higher, the fibres which leave the vagus in the superior laryngeal, 
 enter the area of the injury, an anaesthesia of the larynx, and a paralysis of 
 the crico-thyroid muscle, which is implicated in the tension of the cord, will 
 result, though the loss does not produce any clinical symptoms of impor- 
 tance. It may be that the motions of the epiglottis depend mainly on this 
 branch of the vagus. In very high seated paralyses of the vagus, paralysis 
 of the soft palate and the pharynx, whose innervation is accomplished 
 chiefly by the vagus, may be expected on the corresponding side. The 
 sensory fibres of the pharynx also take their course with the branches of 
 the vagus that leave the nerve trunk at about the level of the first cervical 
 vertebra. The trouble to the patient in unilateral paralysis is comparatively 
 slight. 
 
 The vagus also participates in the innervation of the heart. The cardiac 
 branches partly leave the nerve trunk in the neck between the superior laryn- 
 geal and recurrent, and partly are branches of the latter. Finally, the rela- 
 tion of the vagus to the trachea, lungs, esophagus, stomach, liver and 
 other abdominal organs must be mentioned. 
 
 The symptoms of the whole intestinal innervation area are, as a rule, 
 particularly ill defined and very difficult to interpret in diseases of one nerve 
 alone. Only when other and certain symptoms of a vagus paralysis manifest 
 themselves, will it be possible to connect such symptoms as bradycardia, 
 tachycardia, nausea, etc., with any degree of probability with disturbances 
 of the vagus. 
 
 Bilateral vagus paralysis under any condition is most dangerous. Very 
 threatening symptoms are exhibited even in bilateral posticus paralysis. 
 Severe dyspnoea, particularly inspiratory dyspnoea, is the result of the bi- 
 lateral functional loss of the abductors of the vocal cord. In grave cases, 
 tracheotomy is necessary. In bilateral simultaneous paralysis of the sup- 
 erior laryngeal and recurrent nerves, the complete immobility of the larynx 
 musculature, particularly that of the epiglottis, the inability to cough, 
 thereby resulting, and the anaesthesia of the larynx, deprive the lungs of 
 one of its most important protective mechanisms, and it will rarely be 
 long before deadly aspiration pneumonia, the so-called vagus pneumonia, 
 sets in. 
 
 Lastly, bilateral paralysis of the pharyngeal branches, whose one-sided 
 functional loss gives rise to comparatively trifling complaints, leads to the 
 
i 4 o DISEASES OF THE PERIPHERAL NERVES 
 
 unfortunate condition of total paralysis of deglutition, which necessitates 
 artificial nourishment. 
 
 Only a few words have to be added concerning diagnosis. A one sided 
 recurrent, or still more, a posticus paralysis may to the inexperienced be 
 simulated by tubercular or carcinomatous infiltrations of the larynx, that 
 mechanically impair the mobility of the vocal cord. In the differential 
 diagnosis of bilateral posticus paralysis, spasm of the glottis must be con- 
 sidered, which, however, occurs only in transitory attacks, almost exclu- 
 sively in a very characteristic way in rhachitic children, and in rare 
 instances in hysteria of adults. 
 
 A particularly important phase of the diagnosis of vagus paralysis is, 
 as is evident from the astiological discussion, the search for the fundamental 
 cause of the affection. 
 
 It should be remembered that a number of diseases of the central nervous 
 system, of which we shall name only bulbar paralysis and focal affections 
 of the medulla oblongata, may, by destruction of the root and nucleus areas, 
 even by bilateral destruction of supranuclear paths (pseudo-bulbar paraly- 
 sis), lead to paralytic symptoms in the innervation area of the vagus. 
 
 9. The Accessory Nerve 
 
 Isolated paralyses of the accessory result only, as in the case of the 
 vagus, through lesions outside the cranium. The lesion therefore is always 
 distal to the anastomosis of the two nerves. The physiological significance 
 of the anastomosis can not be estimated with certainty. In lesions common 
 to the last cranial nerves, carious and other processes in the region of the 
 upper cervical vertebrae must be borne in mind above all others. 
 
 In isolated accessory paralysis then, it is a matter of paralysis of the so- 
 called external branch, which supplies the trapezius and sterno-cleido- 
 mastoid muscles with motor fibres. The usual causes are injuries of the 
 nerve, particularly those resulting from extirpation of tubercular glands 
 of the neck, and inflammation and tumor formation in the immediate 
 neighborhood. 
 
 In paralysis of the sterno-cleido-mastoid muscle, the head can be turned 
 but feebly towards the sound side. When an attempt is made to effect this 
 movement against resistance, it will be plainly seen that the muscle does 
 not contract. It does not stand out sharply from the neck as in healthy 
 persons. If a one sided paralysis exists for some time permanent shortening 
 of the normal muscle occurs, and a caput obstipum (torticollis) usually 
 develops. 
 
 Corresponding to the physiological function of the trapezius, which 
 both lifts the shoulder girdle and draws it backwards at the same time, the 
 shoulder, in total paralysis of this muscle, sinks forwards and downwards. 
 
THE ACCESSORY NERVE 
 
 141 
 
 The medial edge of the scapula is moved outwards from the spine and runs 
 obliquely from the inside below to the outside above (the angel wing position 
 of the scapula). The clavicle springs abnormally forwards. Lifting 
 (shrugging) of the shoulders, and adduction of the scapula to the spine, is 
 possible only to a very limited extent. Through the lack of sufficient 
 fixation of the shoulders, the functional value of the arm-movements is 
 partially impaired. The shoulder girdle is abnormally motile on passive 
 movement (Figs. 32 and 33). 
 
 The changes of the external form are still more strikingly evident in the 
 atrophic stage. The neck-shoulder line, which is lengthened on the affected 
 side, becomes angular. The acromion and clavicle, in the neighborhood 
 of their articulation, appear to the touch to lie under the skin, as if they were 
 part of a skeleton. The relief normal to the neck is, on the side of the 
 atrophic sterno-cleido-mastoid muscle, flattened, even depressed. 
 
 Fig. 32. — Right-side paralysis of 
 the accessory. Posterior view. 
 {Leipsic Medical Clinic.) 
 
 Fig. 33. — Left-side paralysis 
 of the accessory. Lateral view. 
 {Leipsic Medical Clinic?) 
 
 Paralysis of the trapezius is very often incomplete, and usually some 
 of its parts remain more or less capable of discharging their functions. 
 Under such conditions not only should incomplete lesions of the accessory 
 trunk be thought of, but also the fact that branches of a few spinal nerves 
 participate in the motor innervation of the muscle and probably in an indi- 
 vidually varying degree. On the other hand, in total paralysis, the pos- 
 sibility of spinal nerve branches destined for the trapezius muscle becoming 
 paralyzed together with the accessory, must be considered. This occurs 
 perhaps chiefly in cases in which the seat of the lesion is rather deep. 
 
142 DISEASES OF THE PERIPHERAL NERVES 
 
 The lesion may be confined to the terminal branch destined for the 
 trapezius, so that the sterno-cleido-mastoid remains intact. 
 
 The complication of disturbances in the innervation of the palate, phar- 
 ynx and larynx, indicates intracranial disease in the region in which the 
 accessory takes its course in close proximity to the vagus, with which it anas- 
 tomoses through its internal branch. To what extent this branch partici- 
 pates in causing the disturbances mentioned, cannot yet, as we have before 
 stated, be determined with certainty. 
 
 In the differential diagnosis a supranuclear paralysis will never occasion 
 any doubt, as it invariably appears only as a partial symptom of a cerebral 
 hemiplegia. 
 
 Nuclear paralyses are found in progressive spinal muscle atrophy and in 
 other diseases of the spinal cord and the medulla oblongata. The rare cases 
 occurring as symptoms or complications of tabes are probably partly nuclear, 
 partly neuritic. 
 
 In myopathies, particularly in the various forms of muscle dystrophy, 
 the trapezius is especially often involved; while the sterno-cleido-mastoid 
 atrophies regularly only in advanced cases of myotonic dystrophy. 
 
 Congenital muscle defects also occur in the area of the accessory. 
 In considering treatment it should be borne in mind that disturbances 
 of function may be diminished by means of orthopaedic apparatus which 
 gives support to the lax shoulder girdle. 
 
 10. The Hypoglossal Nerve 
 
 What has been said of the preceding cranial nerves, applies likewise to 
 the hypoglossal nerve: basal intra-cranial processes and affections in the 
 area of the upper cervical vertebras paralyze the hypoglossal nerve together 
 with its neighbors, while the cause of isolated paralyses must be sought out- 
 side the cranium. Injuries in the neck are more likely to lead to a paralysis 
 of the hypoglossal nerve, but palsy now and then is occasioned by tumors 
 or inflammation in the vicinity of the nerve trunk. 
 
 Unilateral paralysis of the tongue which is the most important result 
 of disease of this nerve, causes the patient but slight trouble and disturbance. 
 The tongue can be moved only imperfectly towards the sound side. As a 
 rule it deviates towards the affected side when extended, which is usually 
 the case when the genio-glossus muscle is involved in the paralysis — a con- 
 dition which will hardly be absent in peripheral nerve diseases. When 
 resting in the mouth, the tongue does not usually show any considerable 
 deviation from the normal position. 
 
 On the other hand, the signs of atrophy will soon be distinctly visible, 
 namely, the decrease in its volume, the wrinkling, the flaccid, spongy consis- 
 tency of half of the tongue and further the reaction of degeneration. Remark- 
 
THE HYPOGLOSSAL NERVE 143 
 
 ably often fascicular and fibrillary spasmodic twitchings of the diseased 
 half and even of the whole tongue have been reported in cases of paralysis 
 of the tongue which were surely not nuclear. 
 
 The hypoglossal nerve innervates also the so-called infra-hyoid muscles 
 (sterno-hyoid, thyro-hyoid, sterno-thyroid, omo-hyoid). It receives the 
 fibres for these muscles in part through several anastomoses with the 
 superior cervical nerves. When the lesion is comparatively deep seated, 
 those fibres, which reach the nerve only peripherally, may in part be affected 
 also. In such cases the symptoms of the paralysis of the muscles concerned 
 are said to be more pronounced and the hyoid bone and larynx deviate 
 when swallowing towards the sound side; it is further claimed that a flat- 
 tening of the neck in the muscle area is observable and that R. D. may be 
 detected. 
 
 Bilateral paralysis of the tongue which, it is true, hardly ever results 
 from peripheral causes, is of course much more serious. Eating and 
 speaking are much hindered in such cases. 
 
 In the differential diagnosis, in addition to disease of the motor nerve 
 of the tongue, supranuclear glossoplegia must be first considered. It occurs 
 unilaterally as a partial symptom of cerebral hemiplegia and only in ex- 
 tremely rare cases as an isolated monoplegia. The symptoms indicative of 
 the peripheral neuron are of course lacking. Bilateral supranuclear glos- 
 soplegias belong to the picture of pseudobulbar paralysis. Nuclear paral- 
 yses are found in bulbar paralysis and in all sorts of other diseases of the 
 medulla oblongata. The paralysis is then very frequently a partial one; 
 single muscles, as the important genioglossus, may remain unaffected; 
 functional disturbances may be almost wholly wanting, and the disease may 
 be disclosed only through circumscribed atrophy accompanied by fibrillary 
 tremor. Tabetic hemiatrophy of the tongue is probably nuclear in some 
 cases, neuritic in others. The radicular paralyses, too, may be partial, in 
 explanation of which it is necessary only to consider the relatively great 
 extension of the root-area. 
 
 Except in myasthenia, myopathic paralyses are very rare. Congenital 
 unilateral defects have been described and also the participation of the 
 tongue in hemiatrophia faciei. 
 
 Glossospasm, which occurs unilaterally, will not be mistaken by any 
 careful investigator for a unilateral glossoplegia. The rigid fixation of 
 the tongue in the spasm prevents such an error. 
 
 B. The Spinal Nerves 
 
 In the following discussion of the diseases of the spinal nerves, only 
 those cases — selected from an abundance of possibilities — will be treated in 
 detail which are typical and of practical importance. Those who are 
 
144 DISEASES OF THE PERIPHERAL NERVES 
 
 familiar with the fundaments of the science of peripheral paralyses and 
 know the innervation areas of the various nerves, will be able to judge cor- 
 rectly even rarer clinical pictures. 
 
 i. The Phrenic Nerve 
 
 The phrenic nerve may be injured in the neck, or in the interior of the 
 thorax by injuries, tumors and inflammations. 
 
 The paralyses of the diaphragm resulting from the influence of toxic 
 and infectious factors without apparent local cause, are as a rule bilateral 
 and probably appear most frequently in polyneuritis. Diseases of this 
 nerve are rare. For the purposes of differential diagnosis it may be well, 
 however, to enumerate the other causes of paralysis of the diaphragm: the 
 most varied diseases of the spinal cord in the area of the third and fourth 
 cervical roots and the disease of these roots themselves in morbid processes 
 of the meninges or of the vertebrae. Tabes in a few rare cases leads to 
 diaphragmatic paralysis, which sometimes at least is of an undoubtedly 
 spinal nuclear nature. Further there are myopathic diaphragmatic paral- 
 yses; the progressive muscular dystrophy may seize upon the diaphragm. 
 Inflammations of the serous membrane, which covers the diaphragm, may 
 spread to the muscle and occasion a myositic paralysis. In hysteria a picture 
 of a complete suspension of the diaphragmatic functions may be observed. 
 Finally, for the sake of completeness, the congenital defects which occur in 
 the diaphragm in the peculiar form of hernia diaphragmatica should be 
 mentioned. This latter needs no consideration in differential diagnosis. 
 
 Symptoms. — In inspiration the paralyzed diaphragm either does not 
 descend at all or does so very incompletely. For this reason the inspiratory 
 arching of the upper abdomen is lacking and it is even possible that during 
 deep inspiration these regions may be drawn in. In paresis, the diaphragm 
 is capable of a moderate though sufficient bulging; but its descent may be 
 prevented through light pressure with the hands against the abdominal 
 walls. Litten's diaphragm phenomenon is absent in the paralyses. The 
 edge of the lung may be shifted somewhat upwards. An X-ray examination 
 will show that the diaphragm stands high, that it does not bulge normally 
 during inspiration and that, during deep inspirations, it will sometimes 
 even be drawn upwards. 
 
 In unilateral paralysis the symptoms are well defined only on one side, 
 while the subjective troubles and functional disturbances may be relatively 
 trifling. Bilateral paralysis, on the other hand, must be regarded as quite 
 a serious occurrence. Even when at rest the patient finds breathing 
 somewhat difficult. The slightest extra demands on him occasion great 
 anxiety. Much will depend upon the functional capacity and practice in 
 costal breathing. A supervening disease of the lungs produces grave 
 dangers. 
 
THE PHRENIC NERVE. THE BRACHIAL PLEXUS 
 
 145 
 
 The abdominal pressure is very much impaired in its influence on the 
 viscera in the abdominal cavity through the lack of resistance from above. 
 The abdomen, when pressed, does not arch. The force of coughing and 
 sneezing is also diminished on account of impairment of the functional 
 capacity of the abdominal muscles involved in paralysis of the diaphragm. 
 
 Nothing special need be added concerning the prognosis and 
 therapeutics. 
 
 The physician should be familiar with electric irritation of the phrenic 
 nerve because by this means in some cases of asphyxia, for instance in 
 narcotic poisoning, the respiration and consequently the life of the patient 
 can be kept up for several hours and time thus be gained for saving the 
 patient. 
 
 2. The Brachial Plexus and its Nerves 
 
 The network of the arm nerves consists chiefly of the four lower cervical 
 nerves and the first dorsal nerve. The scheme in Fig. 34 shows, how from 
 these five roots emanate first three plexus cords and from these again, 
 through renewed reunion and the splitting off of fibres, three secondary 
 
 Plexus roots 
 
 Primary 
 
 ^jLtiZsT Plexus cord 
 
 Secondary 
 Plexus cord 
 
 Ntnusculocu taneus 
 
 N.inedianup 
 
 M radial, u. axillar 
 
 N. uhiaris 
 
 > tftfwracicuf LonffUS 
 Fig. 34. — Schematic presentation of the plexus brachialis. 
 
 cords, one lateral, one medial, and one posterior. From the portion of it 
 situated above the clavicle, the plexus furnishes quite a number of its "short" 
 branches to the musculature of the shoulder. Below the clavicle, in the 
 axilla, the long nerves of the arm emanate from the secondary cords, the 
 posterior of which sends out the short axillary nerve also (Fig. 34). 
 
 As a supplement to the schematic representation, which, as far as pos- 
 sible, should be kept handy for reference, the following must be added. 
 Of the short supraclavicular branches only a few have been represented. 
 The subscapular nerve should be mentioned, which usually emanates 
 together with several roots, from the branches of the primary bundle, 
 that unite in the secondary posterior cord. From the medial (lower) 
 plexus cord the medial cutaneous branches of the arm and forearm 
 usually originate. 
 
146 DISEASES OF THE PERIPHERAL XERVES 
 
 Although in the main the type described is regularly found, yet the 
 structure of the plexus occasionally shows quite considerable individual 
 variations. Thus, the fasciculus secondarius posterior, instead of arising 
 from the primary cords, may originate from the spinal nerves themselves, 
 of which it is constituted. The manner and place of separation of the various 
 short branches are likewise liable to numerous variations. On that account 
 it would be somewhat hazardous to go into minute details in a topical diag- 
 nosis of paralysis of this area. 
 
 We distinguish root paralyses, true plexus paralyses, and paralyses of 
 the peripheral nerres in the more restricted sense of the term. 
 
 It is a case of root paralysis if the spinal nerves, designated above as 
 roots of the plexus, suffer a lesion themselves, before forming the primary 
 plexus bundle. A distinction can be made between intravertebral and 
 extravertebral root paralyses. The most frequent causes are morbid pro- 
 cesses in the area of the meninges or the vertebrae, traumas influencing the 
 spinal cord directly or its immediate vicinity, such as knife cuts and localized 
 tumor-like structures. In unilateral paralysis the symptoms of lost function 
 will correspond to the innervation area of the affected root. This will be 
 treated of with the spinal cord diseases. 
 
 Pluriradicular paralyses cannot, for the reasons just stated, be dis- 
 tinguished so easily from the corresponding plexus paralyses, unless some 
 astiological factors or some concomitant symptoms of the disease enable a 
 more definite local diagnosis. 
 
 Broadly speaking three main forms may be distinguished among the 
 plexus paralyses: first, the superior plexus or Duchenne-Erb paralysis; 
 secondly, the inferior plexus or Klumpke's paralysis; and thirdly, total plexus 
 paralysis. 
 
 ^Etiology. — Mechanical injury to the plexus stands pre-eminent among 
 all the original causes. It is produced by either direct or indirect influences. 
 
 The direct lesions usually occur in the supraclavicular fossa or the 
 axilla. Contusions, stabs or gunshot injuries may affect the plexus; in 
 serious trauma of the shoulder it may be affected by bone fragments or 
 hemorrhage. The plexus may also be endangered by tumors, or even be 
 destroyed by them; in the armpits it may be paralyzed by the constant 
 pressure of crutches. 
 
 In indirect mechanical influences it is usually a case of traction, some- 
 times perhaps of a contusion occasioned by the clavicle. We shall not 
 enter into a detailed discussion of the much disputed mechanism of the 
 latter occurrence. It becomes particularly dangerous when the arm is 
 drawn well down, outwards, while the head is at the same time inclined in 
 the opposite direction. It is easy to see that in this way traction is made 
 upon the plexus. But it is sometimes the result of lifting the arm strongly 
 up and backwards and letting it remain some time in this position. In 
 
THE SPINAL NERVES 147 
 
 both these ways plexus paralyses result, as for instance, in some exercises 
 on a horizontal bar and in breaking in wild horses. 
 
 Dislocations of the humerus as well as brusque attempts to replace it 
 are also significant, since especially in the latter case a direct lesion of the 
 plexus may occur. 
 
 It is probable that in plexus paralysis of porters or longshoremen the 
 direct pressure of the load exerts an influence besides the traction upon the 
 plexus. 
 
 The cervical ribs are mentioned as playing a predisposing role. 
 
 A special position is occupied by the so-called obstetric paralysis. With 
 infantile obstetric paralysis we have already become acquainted when 
 discussing the facial form. Much more important and frequent are the 
 plexus paralyses of the newly born, which occur intrapartum, and almost 
 exclusively in deliveries by artificial means. The specific reasons are the 
 same as in those cases of paralysis acquired in later life: direct pressure 
 on the plexus, whether produced by fingers or instruments, more serious 
 complicating lesions of the plexus, when a fracture of the clavicle or humerus 
 has taken place, traction in unfortunate positions of the arms, manual 
 traction upon an arm or awkardly releasing an uplifted arm. 
 
 Lastly, the so-called narcotic paralyses must be mentioned which may 
 affect either single arm nerves or the plexus itself. And in these cases, too, 
 there is no special mechanism through which they originate. The foremost 
 cause of these unpleasant occurrences is an unsuitable position of the arms 
 for some time during a lengthy narcosis. The feeble or non-resistance of the 
 patient by giving rise to a favorable opportunity for a traumatic attack upon 
 the plexus, at all events plays a greater role than the acute toxic process 
 which has been assumed by some to be the principal cause of the paralysis. 
 
 Plexus paralyses founded on generally injurious, toxic-infectious influ- 
 ences, without any visible local cause, are extremely rare. In many cases 
 of so-called plexus neuritis which have been observed in infectious diseases 
 of the lungs it may be. that the main cause was a neighboring injury from 
 an upper lobe and not a general toxic process. 
 
 By far the most frequent form of plexus paralysis is the Duchenne-Erb 
 "upper" plexus paralysis. It is usually brought on by direct mechanical 
 causes, attacking the supra-clavicular fossa and by the indirect mechanical 
 influences enumerated before. To this group also belong most of the 
 obstetric paralyses. The uppermost primary plexus trunk is affected. 
 The auxiliary branch of the secondary posterior bundle emerging from the 
 two uppermost roots is regularly affected. The deltoid, biceps, brachialis, 
 brachio-radialis (supinator longus) muscles and frequently also the supinator 
 (brevis) 1 muscle are paralyzed. The suprascapular nerve is very often 
 
 1 Hence the muscles regularly affected are those that in a normal condition may be irritated 
 together electrically from Erb's point in the supraclavicular fossa. 
 
148 DISEASES OF THE PERIPHERAL NERVES 
 
 caught in the area of the lesion, in which case the supra- and infraspinatus 
 are likewise paralyzed. The subscapularis is less often affected. It can 
 be no surprise to any one who is conversant with the topographic anatomy 
 to find that other complications may arise. But in such instances it is no 
 longer a typical occurrence, but a more or less rare variation of the disease. 
 The symptoms of lost function are as follows: The ability in the shoulder 
 joint to lift the arm is partially or wholly destroyed. Unless the mus- 
 culature of the forearm, particularly that flexor group emanating from the 
 condylus internus humeri, in time vicariously assumes, as it frequently does, 
 a part of the function, it is impossible to bend the elbow. The defect of the 
 brachio-radialis muscle will become most obvious when the patient tries to 
 bend the forearm, with it occupying a median position between pronation 
 and supination, against resistance. The muscle does not project in such 
 cases, as it does under normal conditions, from the forearm on the side of the 
 radius, forming a characteristic edge, but on the contrary remains flat or 
 perhaps it even sinks in, while the entire forearm seems "cylindrical." 
 Supination of the flexed forearm (biceps) is impossible; supination of the 
 forearm in extension becomes possible only in case of aid from the supinator 
 brevis. Quite frequently the patient is unable, in the shoulder joint, to 
 rotate the arm outwards (when the suprascapularis is affected). The 
 position alone of a small child's arm may often lead to a conjecture of the 
 nature of the disturbance; for the arm is then adducted, the elbow in 
 extension, the forearm in pronation and perhaps the entire arm turned 
 inwards. 
 
 Disturbances of sensibility occur in the innervation area of the fifth and 
 sixth cervical roots which forms a broad stripe, descending on the outer side 
 of the arm and forearm. 
 
 Klumpke's "inferior" plexus paralysis is much less frequent. It 
 affects the plexus cord which originates through union of the eighth cervical 
 with the first thoracic nerve. The most common causes of such paralysis 
 are direct mechanical disturbances, tumors, and trauma. It would be 
 difficult to state how many of such cases are, strictly speaking, plexus 
 paralyses and how many radicular paralyses. 
 
 The paralysis may be restricted to the small muscles of the hand, the 
 thenar and hypothenar eminences (the balls of the thumb and little finger), 
 and the interossei. Occasionally a few muscles of the flexor group of the 
 forearm are affected. The clinical picture becomes especially characteristic 
 when the ramus communicans of the first thoracic nerve is drawn into the 
 area of the disturbance. In that case paralysis of the dilator pupillae of 
 the side concerned results. The disturbances of sensation occupy a stripe- 
 like area descending along the inside of the arm and along the ulnar side 
 of the forearm. This area is innervated by the two spinal nerves mentioned 
 above. 
 
ROOT AND PLEXUS PARALYSIS 149 
 
 Total plexus paralysis affects all branches of the plexus more or less 
 completely. As the disturbances retrogress, the paralysis may confine 
 itself to one or another part. In some cases the area of the radial and 
 circumflex (secondary posterior plexus cord) appears to be most seriously 
 and lastingly injured. If the lesion has started in the axilla, the clinical 
 picture may resemble a complete paralysis of the long branches of the arm 
 nerves, which results, for instance, from encircling the arm too tightly. 
 
 Even when otherwise very grave and extensive, the disturbances of sensi- 
 bility often leave the most proximal part of the inside of the upper arm 
 unimpaired. This part receives sensory fibres from the intercosto-humeral 
 nerve, which does not belong to the brachial plexus. 
 
 The diagnosis of root paralysis and of plexus paralysis in recent 
 cases is not difficult as a rule, if a careful investigation is made and the 
 aetiological circumstances are duly considered. Mistaking such cases for 
 those impediments to motion resulting solely from bone lesions can always 
 be avoided. Of course, spinal diseases situated in the primary segments 
 of the plexus roots may lead to symptoms of lost function, which correspond 
 more or less closely to those of the plexus paralysis. But often other signs 
 of an intraspinal affection will manifest themselves in such cases. Serious, 
 even insurmountable, difficulties may be found in distinguishing it from a 
 poliomyelitis that is restricted to an arm area, though probably only in such 
 inveterate cases in which no information can be secured, concerning the 
 conditions under which the disease developed. The absence of disturbances 
 of sensation, which latter almost without exception in poliomyelitis remains 
 normal from the very beginning, does not afford a differentiating criterion 
 in inveterate cases. Judging residual conditions is made particularly 
 difficult by the fact that after partial recovery the defects may confine 
 themselves irregularly to some single muscles. 
 
 It must not be forgotten, besides, that both intra- and antepartum cerebral 
 and spinal diseases and paralyses of single peripheral nerves may be acquired 
 in some rare cases. Not every paralysis affecting the arm of a newly born 
 child is a plexus paralysis. 
 
 Statistically considered the prognosis of a plexus paralysis is decidedly 
 less favorable than that of a single peripheral nerve paralysis. Perhaps 
 this is due to the fact that the anatomic injuries are much more serious than 
 is usually supposed. For this reason an early, careful and active treatment, 
 and a vigilant observance of the course of the disease are urgently demanded 
 in every case. Especially in these cases is any negligence, and particularly 
 a "laisser aller" particularly reprehensible. It is self evident that compli- 
 cating bone lesions require surgical treatment. Even the nerve injury itself 
 may necessitate an operation. If there was no occasion in the early stages 
 for suturing the nerve, nevertheless, if the neurological treatment remains 
 without results for some time, one should consider the feasibility of finding 
 
150 DISEASES OF THE PERIPHERAL NERVES 
 
 the plexus secondarily, and if needed, paving the way for the restoration of 
 function by excision of scars and by suture. The results obtained by such 
 treatment have been encouraging. 
 
 It is well to state, that especially on account of the peculiar irritation 
 conditions obtaining in the first few weeks after birth, electric prognosis in 
 affections of the newly born does not as a rule give reliable results. 
 
 A knowledge of the origin of plexus paralyses should induce the surgeon 
 and the obstetrician to use whatever prophylactic care may be necessary. 
 
 The Single Peripheral Nerves That Emerge from the Brachial 
 
 Plexus 
 
 a. Short Branches Originating Above the Clavicle 
 
 The only paralysis in this group of any practical significance is that of the 
 long thoracic nerve, because it alone appears more frequently independently. 
 Its causes are similar to those of the plexus paralysis : mechanical influences 
 in the supraclavicular region and in the axilla, certain large, straining, long 
 continued movements, in connection with traction upon the arm, especially 
 raising the shoulder joint. In some cases generally injurious, toxic infectious 
 influences occupy a prominent place. 
 
 The clinical symptom of this paralysis is the paralysis of the large 
 serratus anticus muscle. As a result of the preponderant pull of its antago- 
 nist, the shoulder blade, when at rest, usually stands somewhat higher and its 
 inner edge is nearer to the spinal column than on the sound side. The in- 
 ferior angle of the shoulder blade usually stands off a little from the thorax, 
 and thereby slips out from the covering formed by the edge of the latissimus 
 dorsi. The ability to raise the arm sideways and especially forwards is 
 seriously impaired; in fact, the arm as a rule cannot be lifted higher than a 
 horizontal position. In time, however, a far reaching compensation may 
 take place, in which numerous muscles, among which the supraspinatus and 
 trapezius muscles stand first, participate. In all cases, however, the serratus 
 does not contract when the arm is raised, which is revealed by inspection and 
 palpation of its indentations. The characteristic turning of the shoulder 
 blade which moves its inferior angle outwards and upwards, and which, 
 occasioned by the serratus, sets in as a rule not when the arm is beginning to 
 be lifted, but shortly before it reaches a horizontal position, is likewise 
 lacking. Insteadjof this, the above mentioned anomalies in position, which 
 even when at rest are usually visible, become more prominent when the arm 
 is raised sideways ; in particular the medial edge of the scapula moves close to 
 the spinal column. In addition to this, in raising the arm forwards, the 
 medial edge stands off considerably backwards from the thorax, so that it is 
 possible to feel 'the under surface of the scapula or the subscapular muscle. 
 This position has been designated as the "winged" position of the scapula 
 (Fig- 35)- 
 
PARALYSIS OF THE LONG THORACIC NERVE 
 
 151 
 
 In diagnosing the paralysis, particular dependence must be placed upon 
 the absence of visible and palpable contractions of the muscle and also upon 
 the absence of the characteristic movement of the scapula when it is attempted 
 to raise the arm. An over-estimation of the anomalies in position has led to 
 the adoption of a pseudo-serratus paralysis. In some very thin persons a 
 kind of "wing" position of the scapula may be observed, which in asymmet- 
 rically built people may appear even unilaterally, when no serratus paralysis 
 exists. In such cases it is very easy to prove that the muscle is capable of 
 performing its functions. 
 
 Fig. 35. — Right-side paralysis of the serratus. Elevation of both arms laterally and 
 slightly forwards. {After Curschmann-Schiiffner,Leipsic Medical Clinic!) 
 
 For persons engaged in manual labor a serratus paralysis is a serious de- 
 fect, but the prognosis, in which one should consider the possibility of com- 
 pensation, is not on the whole unfavorable. 
 
 The other supraclavicular branches of the plexus are, indeed, seen in 
 plexus paralyses some more and some less often affected, but their isolated 
 paralyses are neurological rarities, which are observed now and then espe- 
 cially after mechanical injuries of the primary area of these nerves. Defects 
 in the area of the muscles innervated by them are usually caused not by a 
 disease of the nerves, but by myopathic-dystrophic, and above all, by arthritic 
 processes (arthritic atrophy of the supra- and infraspinatus), and central 
 
152 DISEASES OF THE PERIPHERAL NERVES 
 
 affections. Congenital defects, particularly in the pectoral area, may also 
 occur. In the latter cases the compensation is often remarkable for its 
 completeness. 
 
 b. Branches Developing From the Infraclavicular Plexus Section 
 
 Circumflex Paralysis. — ^Etiological factors are traumatic influences in 
 the shoulder and supraclavicular areas, pressure acting in the axilla, traction 
 upon the nerve occasioned by excessive raising of the arm and by dislocation 
 of the shoulder-joint, and in some cases by noxious factors of a toxic and in- 
 fectious nature. In those cases in which the paralysis results from sleeping 
 upon the uplifted arm, as well as in infantile obstetric paralysis and that 
 produced during narcosis, pulling or stretching the nerve is the chief cause. 
 
 The clinical symptoms are paralysis of the deltoid muscle and anaesthesia 
 in the cutaneous area supplied by the nerve. The muscle cannot be made to 
 contract; neither can the arm be raised from the trunk. The occasional 
 preservation from injury of an anterior muscle segment which derives its 
 fibres from the anterior thoracic nerve, is of no special functional value. 
 On the other hand, in the later stage, a partial functional compensation may 
 be possible by means of numerous auxiliary muscles, among which the supra- 
 spinatus, which is specially important in the earlier stages of the movements, 
 stands foremost. 
 
 The paralysis of the teres minor, which is likewise supplied by the circum- 
 flex, cannot, as a rule, be shown. 
 
 In regard to differential diagnosis, the fact is very important that the 
 deltoid muscle exceedingly often undergoes an arthritic atrophy, while its 
 nerve remains unimpaired. To determine such cases, the affection of the 
 joint should be proved first of all. In arthrogenous atrophy, both the supra- 
 and the infraspinatus are regularly affected, R. D. is absent, and, of course, 
 the disturbances of sensation in the circumflex area are always lacking. 
 
 Further, direct myopathic paralyses of the deltoid muscle resulting from 
 blows or pressure upon the muscle (as from lying upon the shoulder) have 
 been described, and these, too, might lead to a confusion between the two. 
 
 Because of its rarity, we shall do no more than simply mention the par- 
 alysis of the musculo -cutaneous. 
 
 Musculo -spiral or radial paralysis is among the most frequent 
 peripheral paralyses. It owes its special predilection to the extremely ex- 
 posed situation of the nerve. During the greater part of its course along the 
 arm, the musculo-spinal nerve lies directly on the periosteum of the humerus, 
 and particularly where it entwines itself around the bone on the external side 
 of the arm is it exposed to all kinds of injuries. In the first place, bone 
 fractures in the most diverse places may affect the nerve at once, as well as 
 lead later to so-called secondary traumatic paralysis. Further, the nerve may 
 
MUSCULO-SPIRAL PARALYSIS 153 
 
 suffer lesions from pressure in its course along the arm, in the armpit from 
 crutches, and in different places through an unfortunate position of the arm 
 during sleep or narcosis. Sometimes stretching plays a part, as when the 
 arm has lain in an extremely raised position. Dislocation of the shoulder 
 joint may also cause a radial paralysis. It is superfluous to enumerate the 
 many possibilities of direct lesion by blows, shots, etc. Paralysis by con- 
 stricting cords through encircling the upper arm, which affects the radial in 
 particular, has already been mentioned. Now and then, paralyses are 
 occasioned by bungling injections of ether or other substances, or by in- 
 jections in unsuitable places. In some cases, the paralysis has been due to a 
 sudden violent contraction of the triceps muscle. 
 
 In individual predispositions, such as exist, e.g., in toxic conditions and in 
 tabetic individuals, frequently very slight, external local influences suffice to 
 occasion the paralysis. 
 
 The so-called lead paralysis of the musculo-spiral nerve will be discussed 
 with polyneuritis. 
 
 Clinical Picture. — In the vast majority of cases, the paralysis affects 
 only the group of extensors of the forearm, and the disturbance of sensation 
 is limited to the skin area of the hand, which is supplied by the musculo- 
 spiral nerve. The motor symptoms of lost function are as follows: Exten- 
 sion of the wrist and of the fingers — except where rendered possible by 
 means of the interossei and lumbricales muscles — the supination of the 
 extended arm and abduction of the thumb, in so far as not enabled by the 
 abductor brevis, become impossible. An important synergist for bending 
 the elbow was lost in the brachio-radial muscle (supinator longus), whose 
 defect can be ascertained best, if, as has already been mentioned when 
 discussing Erb's paralysis, the patient is made to flex the forearm, when 
 in a semipronated position, forcefully against resistance. In a normal 
 condition, the muscle on the radial side of the forearm will then project 
 sharply, while in a radial paralysis no contraction whatever occurs. 
 
 This loss, however, impairs also the functional capability of a number of 
 nonparalyzed muscles. Doubling the fingers into a fist can be accomplished 
 but feebly, if the synchronous extension of the wrist be wanting. The 
 ability of the patient to spread his fingers out normally, and to execute 
 lateral movements of the wrist, can be demonstrated only when his fingers 
 and hand have been assisted to retain that semi-extended position favorable 
 to this function, but which they cannot assume by themselves. 
 
 When the hand is left to itself, it hangs down limp from the wrist, the 
 fingers are somewhat flexed and the thumb is slightly opposed (wrist drop) . 
 
 Only comparatively rarely, when the seat of the lesion is very high, does 
 the triceps muscle share in the paralysis. In such cases the disturbance of 
 sensation may also affect the cutaneous innervation area of the musculo- 
 spiral on the forearm (N. cutan. antibrach. dorsalis) and even on the arm 
 
154 DISEASES OF THE PERIPHERAL NERVES 
 
 ' (N. cutan. brach. posterior). The participation of the musculo-spiral nerve 
 in the innervation of the brachialis anticus muscle (outer part) may under 
 certain circumstances be shown conclusively by electric examination. 
 
 That the motor paralysis from the beginning is restricted to part of the 
 muscles of the extensor group, is likewise observed only comparatively 
 seldom. This form is most likely to occur when the lesion is situated on 
 the forearm. 
 
 The disturbances of sensation in musculo-spiral paralysis are usually 
 trifling. 
 
 A peculiar form of trophic disturbance, manifesting itself as a thickening 
 of the tendons and which is designated as Gubler's tendon swelling, has been 
 observed particularly in musculo-spiral paralysis, in the region of the back 
 of the hand. It does not seem to be of any great importance. 
 
 The prognosis of musculo-spiral paralysis, statistically considered, 
 is exceedingly favorable. Because of the exposed position of the nerve, a 
 particularly large number of cases of slight paralysis are observed. It has 
 been shown convincingly, that especially in musculo-spiral paralysis, early 
 and proper electric treatment affects very favorably the prognosis in regard 
 to the time required for recovery (E. Remak). 
 
 As a supplement to the general rule for the treatment we add that in 
 incurable musculo-spiral paralysis, orthopedics has at its disposal, besides 
 operative treatment, suitable corrective apparatus constructed as cuffs 
 with attached gloves, serving to replace by rubber traction the loss of the 
 extensors, which is so troublesome in using the hand. 
 
 Median Paralysis. — The various kinds of mechanical injuries which, 
 as we have seen, threaten the musculo-spiral especially in the area of the 
 arm, may all (with the exception of a paralysis caused by sudden muscular 
 contraction) affect the median also, though on account of the protected posi- 
 tion of the nerve this occurs much less frequently. Among the most fre- 
 quent causes of median paralysis is complete division of the nerve by wounds 
 resulting from cuts or stabs on the anterior side of the forearm. Ascending 
 neuritis in consequence of infected wounds in the area of the terminal 
 ramifications has been observed particularly in the median. 
 
 If the nerve sustains a lesion in its course down to the elbow joint, there 
 ensues a paralysis of the entire flexor group in the forearm with the exception 
 of the muscles supplied by the ulnar, of the flexor carpi ulnaris, and of that 
 part of the flexor profundus digitorum destined for the two last fingers; 
 further, a paralysis of the ball of the thumb (excepting the adductor pollicis 
 muscle) and a disturbance of sensation in the greater part of the palm of 
 the hand belonging to the median nerve. 
 
 The alteration of motor functions is therefore as follows : Pronation of the 
 forearm becomes impossible. The wrist can be bent feebly only when aided 
 by a simultaneous abduction toward the ulnar side. Flexing the second and 
 
ULNAR PARALYSIS 155 
 
 third phalanges is possible only in the last two fingers and even there only to 
 a limited extent. By means of the interossei and lumbricales, flexing the 
 proximal phalanges from the second to the fourth finger, under synchronous 
 extension of the other joints, remains intact. The apposition of the thumb 
 disappears. 
 
 Especially as a result of this disappearance the hand assumes that peculiar 
 position, which has caused it to be designated as ape-hand. 
 
 Generally speaking the disturbances of sensation are much more strongly 
 pronounced in median paralysis than in other peripheral paralyses of the 
 extremities. 
 
 If the median suffers an injury not far from the wrist, which happens not 
 infrequently, the clinical symptoms are restricted to the disturbance of 
 sensation and to the paralysis of the ball of the thumb — still, however, with 
 the exception of the adductor. 
 
 Ulnar Paralysis. — What has been said concerning the mechanical 
 aetiology of median paralysis is true also of the ulnar. It must be added, 
 however, that besides being exposed to mechanical injuries on the flexor side 
 of the forearm, where it is not seldom injured, e. g., by cuts, the ulnar is 
 especially exposed to them also in the region of the internal condyle of the 
 humerus. Above all pressure paralyses occasionally result from lesions at 
 this point, for instance when during sleep the head is propped up by the 
 elbow. A particular predisposing condition for this arises, when the nerve 
 is not securely seated in a deep sulcus, but, being in a position of habitual 
 dislocation, easily slips from its resting place. This condition appears either 
 congenitally or, in rare cases, is due to traumatic influences. Paralyses of 
 the ulnar, apparently originating spontaneously, especially in persons predis- 
 posed to them through toxic influences or infectious processes are described 
 likewise (Fig. 36, A and B). 
 
 In injuries located in the lower part of the forearm, the paralysis is 
 restricted to the interossei and lumbricales, the muscles of the ball of the little 
 finger and to the adductor pollicis. The pure adduction of the thumb, 
 both the abduction and adduction movements of the other fingers, and, 
 moreover particularly the flexion of the proximal phalanges of the second to 
 the fifth fingers with synchronous extension of their second and third joints 
 are suspended. The last named function is usually impaired less in the 
 second and third fingers than in the other two, as the first two of the lum- 
 bricales, which just in this movement participate as synergists of the 
 interossei, as a rule, derive their motor fibres from the median. 
 
 In high seated lesions of the ulnar nerve, the flexor carpi ulnaris, which 
 bends the wrist towards the ulnar aspect of the arm, and the flexor profundus 
 digitorum for the last two fingers, which participates in the flexion of their 
 second and third phalanges, are also paralyzed. The sinking in of the 
 interosseous spaces and the atrophy of the ball of the little finger are 
 
156 
 
 DISEASES OF THE PERIPHERAL NERVES 
 
 accompanied then by a characteristic flattening of the ulnar aspect of the 
 forearm. 
 
 The typical position of the fingers, overextension of the first phalanges 
 and flexion of the others from the second to the fifth fingers, dependent 
 upon the preponderance of healthy antagonists, is designated as claw or talon 
 hand, main en griff e. The anomaly is most strongly pronounced in the fifth 
 finger, least in the second, as can be easily understood from the above 
 statements. 
 
 The disturbances of sensation occur in a greater or smaller part of the 
 innervation area of the ulnar nerve situated on the ulnar side of the hand. 
 The ulnar edge of the hand and the small finger are rather regularly 
 hypothetic. 
 
 A B 
 
 Fig. 36. — A, paralysis of the left ulnaris, caused by the division of the nerve in the forearm. 
 The normal hand alongside. B, the affected hand from the volar side. Atrophy of the hand- 
 muscles supplied by the ulnaris, claw or talon hand. The formation of the usually present 
 hyperextension of the basal phalanges of the ring and little fingers was prevented in this case by 
 traction from the scar located at the flexor side of the forearm. (Leipsic Medical Clinic.) 
 
 Ulnar paralysis seems to cause a certain predisposition to Dupuytren's 
 fascia contraction. 
 
 When, in the general part, the reader was cautioned against confusing a 
 peripheral paralysis with all kinds of malformations and impairments of 
 movements, mechanically produced by cicatricial, arthritic, and other 
 processes, which may be accompanied by muscle atrophy and even slight 
 numbness of sensation, it was really more particularly the ulnar paralysis 
 that was alluded to. Careful examinations will prevent such errors. 
 
 In the treatment of ulnar paralysis, the habitual dislocation of the nerve 
 must be considered in some cases. It may be remedied by various operative 
 methods. 
 
THORACIC NERVES. LUMBAR AND SACRAL PLEXUSES 157 
 
 3. The Other Thoracic Nerves 
 
 It is extremely rare that paralyses of this area come under clinical 
 observation. Now and then paralyses of the abdominal muscles result from 
 a disease of the lower thoracic nerves, chiefly occasioned by pathological 
 processes that injure the roots concerned in the area of the spinal meninges. 
 Sometimes, however, they may appear in connection with infectious factors 
 and herpes zoster. 
 
 Motor disturbance is most clearly shown in the attempt to exert abdom- 
 inal pressure. The entire abdomen, or the diseased side, is spherically 
 arched, but no contraction of the abdominal muscles can be seen or felt. 
 It becomes impossible to raise the trunk from a lying position without 
 assistance from the hands. In this attempt, too, the inability to exert the 
 abdominal muscles can be clearly seen and felt. In unilateral paralysis the 
 umbilicus, which even when at rest sometimes deviates somewhat, is drawn 
 considerably towards the sound side. The abdominal reflex is absent in 
 the area of the disease, and hypaesthesia may usually be shown to exist. 
 
 Complete paralysis of the abdominal muscles leads to the development 
 of an abnormally increased lordosis of the lumbar portion of the spine. 
 More serious is the impossibility of making strong expiratory movements. 
 
 Much more frequent than the peripheral paralysis of abdominal muscles 
 is their participation in progressive muscle dystrophy and in varied spinal 
 diseases. Congenital defects also occur. 
 
 4. Nerves of the Lumbar and Sacral Plexuses 
 
 The plexus of the leg (plexus lumbo-sacralis) arises from the lumbar 
 nerves and from the first, second and half of the third sacral nerve. These 
 roots of the plexus participate in the formation of the cauda equina, and 
 proceed by a more or less extensive course along the inside of the spinal 
 canal, where diverse pathological processes may injure them. Radicular 
 paralysis of this area resulting from such injury will be dealt with in the 
 chapter on spinal diseases. After their exit from the intervertebral foramina, 
 the first three lumbar nerves and half of the fourth unite to form the trunk 
 of the crural nerve, after they have sent out a number of secondary branches : 
 a few small ones, mostly sensory, and the more important obturator and 
 lateral femoral cutaneous nerves (external cutaneous). So much for the 
 area of the lumbar plexus. Now, the other half of the fourth lumbar nerve, 
 having united with the fifth to form the lumbo-sacral cord, coalesces with 
 the sacral nerve segments of the leg plexus into the sciatic nerve. The roots 
 of this sacral plexus area send out several secondary branches, namely, the 
 gluteal nerves, the posterior femoral cutaneous (perforating cutaneous) and 
 the motor nerves for the small outward rotators of the thigh, the piriformis, 
 obturator internus, two gemelli and the quadratus femoris muscles. The 
 
158 
 
 DISEASES OF THE PERIPHERAL NERVES 
 
 Jliohypogastricus 
 Jlioinguinalis 
 
 (jenitqfemoralis 
 
 Cutanea? lateralis 
 
 Cruralis 
 
 Odturatoriujs 
 
 (flutaeus superior 
 
 branch for the first mentioned rotator muscle always emanates from the 
 roots; the others may arise from the sciatic trunk (Fig. 37). 
 
 Concerning peculiar groupings of symptoms, which would correspond 
 to lesions of certain plexus segments and which might be clearly differentiated 
 from pictures of paralyses of peripheral branches, we do not possess the 
 definite knowledge that has been obtained for the brachial plexus. In 
 affections of the leg plexus, we are usually confronted with the same clinical 
 pictures as in cases of single nerve paralysis, such as crural paralysis, sciatic 
 paralysis, etc. Some few remarks, which will perhaps be of value for the 
 localization in the plexus area, will be given in the following paragraphs 
 dealing with paralyses of the more important single nerves. 
 
 Anterior Crural Paralysis. — Among the more important causes of 
 this rare paralysis are the following: Injury of the nerve by direct wounding, 
 through tumors, particularly those near the spinal column, in the pelvis, 
 
 also by means of the pressure which 
 the nerve may sustain from the 
 body of the child during protracted 
 delivery (maternal obstetric paral- 
 ysis); pressure injuries caused by 
 protracted extreme flexion of the 
 hips, such as have been observed in 
 infantile obstetric paralysis in chil- 
 dren born with breech presentation, 
 or after narcoses during which the 
 leg was awkwardly placed; lesions 
 of the nerve in fracture of the pel- 
 vis and in fractures and dislocations 
 of the thigh. Lastly, a crural par- 
 alysis may in some cases be ob- 
 served without any visible local 
 cause in persons who were under 
 the influence of metabolic disturbances or of infectious and toxic processes. 
 The most important and the most regularly occurring consequence of a 
 crural paralysis is the paralysis of the quadriceps femoris muscle. Active 
 extension of the knee joint becomes an impossibility. The patient can 
 support himself on the leg only while he keeps the knee in a passively forced 
 extended position. As soon as it is flexed, he collapses. For this reason^he 
 swings the diseased leg forwards when walking, and after he has put down 
 the foot, passively presses the knee into extension until the other leg again 
 supports the body. The paralysis of the sartorius can be determined by 
 inspection and palpation; it does not, however, occasion any considerable 
 functional disturbances. The paralysis of the little branch, through which 
 the anterior crural participates in the innervation of the pectineus, appears 
 
 Jschiadicup 
 
 Glutaeus infer to r 
 Cutaneusjemorip post. 
 
 Fig. 37. — Scheme of the lumbosacral plexus 
 
PALSY OF THE LATERAL FEMORAL CUTANEOUS NERVE 159 
 
 to have no significance. In high seated lesions the ilio-psoas is likewise 
 paralyzed. This results in a loss of flexion in the hip and walking is rendered 
 extremely difficult. A bilateral quadriceps paralysis also renders walking 
 very uncertain, while in a bilateral, complete paralysis of the anterior crural 
 nerve walking becomes altogether impossible. 
 
 Besides motor disturbances, sensory ones in the extended area of the skin 
 supplied by the anterior crural nerve are part of the disease picture. The 
 patellar tendon reflex is destroyed. 
 
 It is hardly necessary to emphasize that a concomitant affection of the 
 secondary branches of the lumbar plexus may sometimes acquire a topical- 
 diagnostic significance. For parts of the innervation area of this peripheral 
 nerve to participate in the most diversified and different, myopathic and 
 nerve diseases occurs much more frequently than its rare paralysis. One 
 of the most frequent causes of a weakness resembling paralysis in the quad- 
 riceps area, is an arthrogenous atrophy of the muscle in diseases of the knee 
 joint. 
 
 Therapy. — It must be emphasized that an orthopaedic apparatus, which 
 replaces the functions of the lost muscles by elastic bands, may materially 
 improve the ability to use the leg. 
 
 Obturator paralysis is still more uncommon. It manifests itself alone 
 or accompanies anterior crural paralysis. iEtiologically affections in the 
 pelvic area must be mentioned. An obturator hernia may lead to paralysis 
 of the nerve; both maternal obstetric and narcosis paralyses have been 
 observed. 
 
 The adductors of the thigh, of which only the pectineus and magnus are 
 slightly innervated from other sources, are paralyzed, and so is the obturator 
 externus, which participates in the external rotation of the thigh. Besides 
 this, disturbances of sensation may manifest themselves on the inside of the 
 thigh. 
 
 The paralysis of the lateral femoral cutaneous nerve (external 
 cutaneous) is of greater practical interest. There is a peculiar clinical pic- 
 ture, known as meralgia ■paresthetica, Bernhardt-Roth's disturbance of 
 sensibility, caused by an isolated destructive disease of the lateral femoral 
 cutaneous nerve. Usually it appears unilaterally; patients complain of 
 various unpleasant sensations and pains on the outer side of the thigh, which 
 become particularly pronounced when walking. All mechanical irritations, 
 even the pressure of the clothes, become very unpleasant. Some patients 
 even complain of a feeling of heaviness in the calf, manifesting itself when 
 they walk and somewhat resembling intermittent claudication. Objectively, 
 a hypaesthesia or anaesthesia exists in a smaller or greater part of the innerva- 
 tion area of the nerve. The seat and extension of the anaesthesia may 
 fluctuate. Very often pressure will reveal particularly painful points along 
 the course of the nerve. 
 
160 DISEASES OF TEE PERIPHERAL NERVES 
 
 The aetiology of the disease is obscure. It develops in connection with 
 infectious diseases, such as typhoid and articular rheumatism. It has been 
 seen to follow injurious mechanical influences and overexertion, and in 
 patients suffering from disordered metabolism, and it has been ascribed, 
 more or less correctly, to several other factors. The affection is of a harmless 
 nature; nevertheless it may become very troublesome to quite a few of 
 those who suffer from it. The prognosis is very questionable, for the 
 disease may be very obstinate and even relapses may occur. 
 
 Of therapeutic measures, faradization may often be recommended, though 
 in cases with great irritation the stabile anode galvanization, in which the 
 inactive electrode can be placed on the calf, is usually to be preferred. It 
 has been reported that in some cases a resection of the nerve was successful. 
 
 Sciatic Paralysis. ^Etiology. — Even in their course through the 
 pelvis the sciatic fibres may sustain various injuries. One of the most im- 
 portant causes is maternal obstetric paralysis of the nerve, occasioned by the 
 pressure to which in protracted delivery the nerve is exposed. Besides 
 lesions may result from tumors, inflammatory processes of neighboring 
 organs, or pelvic exudates from fracture of the pelvis and of the lowest part 
 of the spinal column. Traction and stretching are produced especially by 
 dislocations of the hip; sometimes also by careless attempts to reduce them. 
 At the time when brusque stretchings of the nerve were still used as a treat- 
 ment of neuralgia, paralyses were seen to result from them as well as from 
 remedial injections into and below the gluteal musculature. 
 
 Further, peripherally the nerve is especially endangered by fractures 
 of the femur. 
 
 In the upper part of the popliteal space the two terminal branches, 
 the peroneus and tibialis nerves (external and internal popliteal), separate. 
 Along their individual course they are exposed to numerous dangers, which 
 require a special discussion. 
 
 The peroneal nerve (external popliteal nerve) is affected especially, fre- 
 quently by pressure influences, as for instance, in occupations which must be 
 carried on in a stooping, squatting position (e. g., workers in sugar beet fields), 
 by pressure during narcosis or sleep and in particularly predisposed persons, 
 even from keeping one leg over the other for a long time. Traction, for 
 instance from a sudden twist in the ankle, affects in particular the superficial 
 branch. Frequently fractures of the leg, especially of the fibula, affect the 
 nerve; but on the other hand, affections of the knee joint, in spite of their 
 proximity, only extremely seldom exert a harmful influence. The equally 
 rare case of an infantile obstetric paralysis of the peroneus nerve occasioned 
 by delivery of the child by the foot is mentioned on account of its prophylactic 
 interest. 
 
 The tibialis nerve (internal popliteal nerve) is much less endangered. 
 In its more protected position it suffers less frequently from pressure lesions; 
 
PERONEAL PALSY 161 
 
 probably the most frequent cause of its isolated paralysis is a direct 
 trauma. 
 
 The nerves of the sciatic area are also sometimes found to be affected 
 with an isolated paralysis, though no local cause can be determined. As a 
 rule, a toxic or infectious condition or a metabolic disturbance may be shown 
 to have been a strongly predisposing factor. Connected with protracted 
 neuralgia of the sciatic nerve are often symptoms of lost function, and even 
 more or less pronounced paralysis symptoms. 
 
 The discussion of symptomatology begins most appropriately with a 
 discussion of the symptoms of peroneal and tibial paralysis. 
 
 (a) Peroneal Paralysis. — In complete paralysis the extension (dorsal 
 flexion) of the ankle and of the proximal phalanges of the toes as well as the 
 pronation (abduction) of the foot are entirely lost. Extension of the second 
 and third phalanges is considerably impaired and is now possible only with 
 the aid of the interossei and lumbricales, which at the same time flex the 
 proximal joints. Supination (adduction) of the foot is seriously impaired 
 by the loss of the synchronous extension of the anterior tibial. The foot 
 assumes a pes equirius position, while the toes are flexed. To prevent the 
 toes from dragging when he walks, the patient has to lift his knee to an 
 abnormal extent (steppage gait 1 ). In protracted paralysis and with 
 insufficient medical aid, the abnormal position of the foot will become 
 permanently fixed by the contracture of antagonists. Since the pronation 
 of the foot is completely suspended and yet a few supinators are preserved 
 (those supplied by the tibial nerve), there is added to the pes equinus position 
 a slight raising of the medial border of the foot. Consequently a pes equinus 
 varus is formed. 
 
 Disturbance of sensation affects the dorsal surface of the foot and toes 
 and a short adjoining portion outside on the extensor surface of the leg. 
 The cutan. surge lateralis nerve (communicans peronei) which originates 
 in the popliteal space immediately after the division of the peroneal and the 
 tibial nerves, is not considered in this discussion of isolated peroneal paralysis. 
 
 It has already been mentioned, and it will be enlarged upon when 
 discussing sciatic paralysis, that the two main branches of the peroneal 
 nerve do not always participate to the same extent. Even direct injuries 
 may affect only one of them. The superficial branch (musculo-cutaneous) 
 innervates only the peronei muscles and but a very small part of the region 
 of the skin. Everything else devolves upon the deeper branch (anterior 
 tibial nerve). To understand the functional losses and anomalies of 
 position manifesting themselves in cases of partial paralyses of the peroneus 
 region, it must be remembered that in paralysis of the anterior tibial the 
 foot is disposed to assume a valgus position; in paralysis of the peronei 
 
 1 Charcot compared this gait to that of the grave step of noble horses; steppeur, Engl, stepper, 
 from to step, meaning particularly a dignified step . 
 
1 62 DISEASES OF THE PERIPHERAL NERVES 
 
 muscles, on the other hand, it tends to assume a varus position. The 
 peroneus longus muscle which is inserted at the base of the first metatarsal 
 bone, is also an antagonist of the tibialis anterior muscle in so far as it lowers 
 its point of insertion anteriorly and increases the arching of the foot. If 
 it is paralyzed, a flattening sets in; in case of its contracture an increase of 
 the arching of the ankle. The function of extension devolves solely upon 
 the ramus profundus. 
 
 (b) The tibial paralysis affects the musculature of the calf, the muscles 
 of the balls of the big and the little toe, the interossei and lumbricales. 
 Flexion of the foot and toes is lost — the slight flexor action of the peroneus 
 longus is hardly of any value in view of the grave defect — the patient is 
 unable to spread out his toes or to adduct them; neither can he extend the 
 second and the third toe joints with a synchronous flexion of the proximal 
 phalanges. Supination is possible only by means of the tibialis anterior, 
 therefore only with simultaneous extension. The foot assumes slight 
 extension and pronation (i. e., calcaneo-valgus) position; the toes in the 
 metatarso-phalangeal joint suffer over-extension (claw foot, pied en griff e). 
 
 The disturbance of sensation occurs in the plantar region of the foot, 
 including the flexor surface of the toes and the outer area of the ankle, 
 perhaps even a short contiguous portion outside on the posterior aspect of 
 the leg. 
 
 The Achilles tendon reflex is destroyed. The plantar reflex is either 
 destroyed or at any rate, if the sensory conduction is preserved, abnormal. 
 The characteristic flexion of the toes is suspended on account of an inter- 
 ruption in the motor path. The extension of the toes which takes its place, 
 especially in the case of the big toes, does not, of course, have the diagnostic 
 significance of the Babinski reflex (pseudo-Babinski). 
 
 (c) Sciatic Paralysis. — If the nerve suffers a complete interruption of 
 conductivity above the place where the separation of the peroneal and the 
 tibial takes place, it will result in an absolute paralysis of all the muscles in 
 the leg proper and in the foot. In high seated lesions, the flexor muscles 
 of the thigh (biceps, semimembranosus and semitendinosus) are also 
 paralyzed. The loss of the little branch by which the sciatic participates 
 in the innervation of the adductor magnus muscle, and the paralysis of the 
 small outer rotators, will hardly occasion any considerable clinical symptoms. 
 
 The disturbance of sensation extends also into the area of the cutaneus 
 surae lateralis nerve (external saphenous nerve). 
 
 Especially important is the fact that in high seated lesions in the sciatic 
 area, partial paralyses, particularly in the area of the peroneus, occur. It is 
 almost always the case in maternal obstetric paralysis, that any existing 
 more extended defects soon retrogress, but that a peroneal paralysis, which 
 shows a decided preference for the area of the ramus profundus, thus sparing 
 the peronei muscles, remains. A great many attempts have been made to 
 
PROGNOSIS AND THERAPY OF SCIATIC PALSY 163 
 
 explain this peculiar condition. The cause for the last mentioned specific 
 case might be traced to a certain part of the plexus (which was mentioned 
 in the introduction as the lumbosacral cord) which passes into the sciatic and 
 which perhaps carries fibres for the ramus profundus peronei, that is espe- 
 cially exposed to mechanical injuries in the pelvis. At any rate, we are really 
 confronted with a greater liability to lesion in the peroneal than in the tibial 
 nerve. Whether it is exposed more to mechanical injuries in other places 
 than in the one named, whether more unfavorable vascularisation conditions 
 obtain, or whether still other factors have any part in it, is unsettled. An 
 anatomic fact, which may some time acquire clinical interest, is the following : 
 even though the fibres destined for the peroneus and tibialis always take 
 their course as far as the upper part of the popliteal space in closest prox- 
 imity, yet they are frequently to be found far up from the point of their 
 separation, and even as far back as the plexus area, as separate, or easily 
 separated bundles. 
 
 In the prognosis, one should be very careful not to interpret too opti- 
 mistically those sciatic paralyses, whose point of lesion must be looked for 
 in the deeper part of the pelvis. Particularly the maternal obstetric paralysis 
 leaves, in a great percentage of cases, a lasting defect. 
 
 In separating numerous peripheral paralyses in the sciatic area with 
 regard to differential diagnosis from such as depend upon limited, spinal, 
 focal affections, peculiar difficulties will be encountered. Our knowledge 
 concerning the way in which the nuclei of the particular muscles are grouped 
 in the anterior horns of the spinal cord, is, in many respects, still incomplete. 
 At all events, there is a great similarity between these spinal groups and the 
 innervation area of the peripheral nerves. This hint should be sufficient 
 to warn every one to be careful. 
 
 In supranuclear paralysis of the leg produced by lesion of the pyramidal 
 tract, the extensors of the foot are usually injured very gravely. This 
 predilection is frequently so remarkable, that the thought of a peripheral 
 complication often suggests itself at once. 
 
 Bilateral peroneal paralyses are usually polyneuritic. 
 
 Therapy. — We call particular attention to prophylaxis with regard to 
 contractures, which is so very necessary in peroneal paralysis. The difficulty 
 in walking, due to elevation of the heel, may be remedied by means of suitable 
 apparatus, which partially replaces the function of the muscles by elastic 
 straps. For tibialis paralysis, similar apparatus has been invented. 
 
 Paralysis of the gluteal nerves is very rare. It has been observed, f. i., 
 after fracture of the os sacrum and in pelvic tumors. The same injury may 
 affect the posterior cutaneous nerve of the thigh. The motor paralysis 
 affects the gluteal muscles and the tensor fasciae latse. Extension and abduc- 
 tion in the hip joint are paralyzed; and the inner and outer rotation greatly 
 weakened. When walking, the lack of lateral fixation of the pelvis against 
 
1 64 • DISEASES OF THE PERIPHERAL NERVES 
 
 the thigh is especially striking. When stepping on the diseased leg, the 
 pelvis deviates towards the sound side; the movement becomes somewhat 
 waddling. 
 
 5. Nerves of the Pudendal and Coccygeal Plexuses 
 
 Radicular paralyses of this area will be discussed with the affections of 
 the cauda equina. Next to nothing is known concerning affections in the 
 area of the real plexuses and their peripheral branches. 
 
 Supplement 
 
 Occupation Paralysis and Occupation Neuritis 
 
 As we have seen, typical paralyses of single peripheral nerves may be 
 caused by occupational injuries. Thus, continual pressure produces paraly- 
 ses of the foot, especially of the peroneal nerve, in persons who are forced to 
 work in a squatting position, paralyses of the ulnar in persons who during 
 their work continually support their elbows on something, while pressure and 
 traction produces plexus paralyses in those who carry heavy loads. 
 
 One group of cases, however, requires a separate discussion, namely, that 
 in which peculiar clinical pictures are occasioned by the special conditions 
 of occupational aetiology, and cases of selective injury of a few known 
 muscles, which in the particular occupation are usually synchronously 
 strained and compressed or otherwise irritated mechanically. 
 
 The most important form practically, affects especially workers who 
 must handle continuously a certain tool, the handle of which they must clasp 
 tightly, so that the small muscles of the hand are both strained and exposed 
 to pressure, as ironers, locksmiths, borers, planers, cutters (who must use 
 scissors) and workers in many other occupations. Precisely the same dis- 
 turbance is seen in persons who are compelled to lean with one hand con- 
 tinuously and heavily on a crutch. In all these cases the muscles of the 
 ball of the thumb and of the interosseus primus are especially endangered. 
 
 Occupational paresis (which is similarly localized) of milkers, cigar- 
 makers, etc., may mostly be traced to strain. 
 
 We shall not enumerate the rarer analogous occurrences, except the 
 paralysis of a few muscles of the left thumb of drummers (drummer-paral- 
 ysis), in diagnosing which particular care should be taken not to confound it 
 with a surgical affection which manifests itself under the same circum- 
 stances (rupture of the tendon of the extensor longus pollicis, drummer's 
 tendon). 
 
 Besides those persons who by reason of toxic or infectious injuries are 
 predisposed to such disease, novices who do their work with an unnecessary 
 expenditure of force, are especially liable to it. 
 
OCCUPATION NEURITIS, POLYNEURITIS 165 
 
 The clinical picture which is frequently introduced and accompanied 
 by paresthesia and pains, is mainly composed of weakness of the affected 
 muscles or atrophy, which sets in either rapidly or stealthily. 
 
 R. D. and a slight decrease of sensibility in many cases indicate that the 
 affection is neuritic. Yet myopathic cases possibly occur also. 
 
 The diagnosis will be easy if proper regard be given to the aetiology. 
 Even disturbances of sensibility and pains, when present, may serve to dis- 
 tinguish several spinal affections having externally some similarity. Oc- 
 cupation paresis may combine with occupation neurosis. 
 
 The prognosis of the pure cases is usually in all respects favorable. 
 
 Of particular importance in the therapy is rest for the affected part, and 
 above all, of course, the patient must temporarily or permanently abandon 
 the injurious occupation. 
 
 Polyneuritis 
 
 Polyneuritis {Ley den, about 1880) is a more or less widespread primary 
 degenerative disease of the peripheral nervous system, distributed nearly 
 symmetrically and taking a regular course, and developed fundamentally 
 from a general injury, resulting from toxic matter in the very broadest sense of 
 the word, with which the bulk of the bodily fluids have become contaminated. 
 
 Hence not every multiple disease of peripheral nerves is a case of poly- 
 neuritis. Such diseases in which a multiple paralysis of the cranial nerves 
 or of the roots is occasioned by gummatous infiltration or sarcomatous 
 tumors in the area of the meninges, of the base of the skull or of the spinal 
 cord, are on the best of grounds not considered as polyneuritis. Nor would 
 multiple paralyses resulting from tumors or gummatous processes on the 
 peripheral nerves themselves come under such classification. Other cases 
 are much more akin to polyneuritis. We have observed before, that upon a sub- 
 stratum of general injuries, which are usually at the bottom of polyneuritis 
 there frequently develop isolated paralyses of peripheral nerves. Paralyses 
 of several single nerves, the irregular choice of which is presumably due to 
 specific local causes also occur rather often under such greatly predisposing 
 conditions. These cases must likewise be distinguished from polyneuritis. 
 
 On the other hand, however, local factors, such as heavy functional de- 
 mands on certain parts, may in some respects modify the distribution even in 
 polyneuritis proper. Nevertheless it will be necessary and almost always 
 possible to separate distinctly, both theoretically and practically, the poly- 
 neuritis, the symmetric polyneuritis, from other multiple nerve paralyses 
 (multiple mononeuritis or polyneuritis disseminata and multiple tumor 
 formations) . 
 
 Etiology. — The majority of cases of polyneuritis in Germany are occa- 
 sioned by chronic alcoholism. Next to this the form known as lead paralysis 
 
166 DISEASES OF THE PERIPHERAL NERVES 
 
 is probably of greatest practical importance. In the majority of cases chronic 
 poisonings sustained during work with lead occasion lead paralysis, but such 
 diseases have been seen, also, after all kinds of more casual poisonings. 
 Among the classes of workmen who are thus endangered are those who are 
 engaged in the metallurgical extraction of lead; those who cast the metal 
 into shot, pipes, type (type founders), or use it for chemical preparations, 
 such as colors; also painters, varnishers and dyers who use lead colors, potters 
 who apply glaze containing lead, compositors who set lead type; gold leaf 
 workers, filecutters and engravers who use lead plates as supports; workers 
 who must handle the lead plates used in storage batteries, plumbers and 
 others who solder pipes with lead alloys and tinners who use lead for the 
 same purpose. 
 
 Arsenical paralysis, after an improper medical use of arsenic, is the next 
 frequent toxic polyneuritis. Polyneuritis from bisulphide of carbon poison- 
 ing is seen in some rare cases in workers employed in the vulcanizing rooms 
 of rubber factories. Toxic polyneuritis after poisoning from carbonic oxide, 
 mercury and some other poisons is a great rarity. 
 
 Polyneuritis accompanying and following infectious diseases forms a 
 second important group. Of greatest practical interest is undoubtedly the 
 so-called post-diphtheritic paralysis, which most frequently develops about 
 the third week of convalescence. 1 In connection with typhoid, influenza, 
 malaria and other acute infections polyneuritis is much rarer. In these cases 
 the nerve affection also usually sets in during the convalescent stage of the 
 primary disease. In syphilis, polyneuritis manifests itself in the secondary 
 stage, generally not later than half a year after the infection. It also occurs 
 occasionally in the course of gonorrhea and in advanced pulmonary tuber- 
 culosis. We shall not enumerate here the rarer infectious causes. In a 
 supplement to this chapter we shall briefly consider the nerve affection in 
 leprosy. 
 
 Besides the main astiological groups of toxic polyneuritis and those 
 appearing in connection with infectious diseases, the so-called cachectic- 
 dyscrasic forms occupy practically only a subordinate place. Those cases 
 occasionally seen in various marasmic conditions and in metabolic diseases, 
 as diabetes, and polyneuritis manifesting itself during pregnancy or when 
 lying-in, unless it can be referred to an accompanying infectious disease 
 (puerperal fever), are classed among the above mentioned forms. 
 
 In quite a number of cases polyneuritis has to be designated as idiopathic 
 for want of detectable aetiological factors. It should not be forgotten that 
 even in the toxic, infectious and cachectic-dyscrasic forms the immediate 
 cause of the disease is entirely obscure, for it is only in a small percentage of 
 
 1 The prejudice of the laity that diphtheritic paralysis is a result of the serum treatment has 
 no foundation whatsoever. On the contrary, it seems as if serum treatment instituted very early 
 lessens the danger of an occurrence of this disease. (Heubner.) 
 
THE CLINICAL PICTURE OF POLYNEURITIS 167 
 
 those affected with such injuries that a polyneuritis really develops. It is 
 not. infrequent, therefore, that even such a predisposing factor can not be 
 found with certainty. Some of such cases give the impression of an inde- 
 pendent infectious disease. 
 
 A particular, probably infectious, form of disease, endemic in Eastern 
 Asia, Brazil and Africa, which sometimes spreads even into Germany, is 
 called beri-beri. 
 
 It remains only to be added, that in the aetiology of polyneuritis several 
 of the factors mentioned may concur and that a cold may in many cases be 
 an exciting factor. 
 
 The clinical picture of polyneuritis is composed of numerous peripheral 
 nerve paralyses and nerve pareses distributed more or less strictly sym- 
 metrically over both sides of the body. 
 
 In the following more minute description we begin with a fundamental 
 type, such as for instance in alcoholic polyneuritis and in the majority of 
 the other forms constitutes the rule, and then continue by discussing the 
 clinical peculiarities of some special cases. 
 
 In the great majority of cases the nerves of the leg, and among these, the 
 peroneal nerves, are affected first and most severely. Frequently only one 
 leg is affected at first but very soon the other succumbs. Next to the 
 peroneal the tibial nerves are affected, as a rule. Ascending, the disease 
 then seizes upon the nerves of the thigh and the pelvis, and usually together 
 with them, or even before them, also a part of the nerves of the upper extrem- 
 ities, in most cases first the musculo-spiral, then the median and ulnar nerves. 
 In the upper extremities too, a partiality for the distal parts manifests itself. 
 Less often are the shoulder girdle and the musculature of the upper arms 
 affected. The supinator longus muscles of the radial nerve's innervation 
 area are also not infrequently spared. Cases in which the paralysis of the 
 arms bears the character of a plexus paralysis are very uncommon. 
 
 As a rule, the paralysis does not develop with equal severity in the 
 various nerve areas. A complete paralysis occurs most frequently in the 
 musculature of the leg. In other nerve areas, it is often only a case of a 
 more or less serious paresis. In grave cases, almost or quite complete, 
 paralysis of all four extremities may indeed occur, and in such cases, the 
 muscular apparatus of the trunk, the abdominal musculature, and the 
 musculature of the back and neck will not often be spared. 
 
 Usually it soon becomes possible to recognize the atrophy of the affected 
 muscle-groups. Electric stimulation indicates what changes are to be 
 expected ; generally it will be R. D . Almost always the reflexes are very much 
 weakened or destroyed. Destruction of the tendon and periosteal reflexes 
 may be one of the earliest symptoms. Only rarely can a transient initial 
 exaggeration of the reflexes be noted. Even in those light cases in which the 
 tibial and crural nerves are comparatively slightly affected, it is unusual for 
 
1 68 DISEASES OF THE PERIPHERAL NERVES 
 
 the knee jerk and Achilles tendon reflexes to be preserved all through the 
 illness. More often an exaggeration of the cutaneous reflexes (plantar 
 reflexes), particularly in hyperalgesic cases, sets in. 
 
 By the paralyses the members are placed in anomalous positions. The 
 feet are in the pes equinus position; the hands usually show wrist-drop, or in 
 some cases, the ape hand or talon position, or a mixed form. In protracted 
 duration of the affection, the anomalous positions are fixed by contractures, 
 unless treatment prevents it. 
 
 "When the patient is still able to walk, or as soon as he has regained that 
 ability, a pronounced paretic gait, steppage gait or "waddling gait," accord- 
 ing to the extent and distribution of the affection, will be observed. Among 
 the earliest symptoms of many cases of disorders occurring in the sensory 
 area, are paraesthesia, formication, and a sense of numbness particularly in 
 the distal parts of the extremities, beginning in the feet. Frequently, but 
 by no means always, very irritating, drawing, burning pains manifest them- 
 selves in the parts affected, while the nerve trunks and the musculature 
 are sensitive to pressure. When the pains are severe, and the paralysis is 
 not total, the patient is wont to resist passive movements, which sometimes 
 misleads the inexperienced into thinking that he is dealing with spastic 
 conditions. 
 
 Objective disorders of sensation are of far less importance than motor 
 disorders, and even in grave cases sometimes confine themselves to a few 
 nerve areas of the distal parts of the extremity. However, it is very 7 unusual 
 for them to be lacking entirely. Especially in alcoholic and arsenical 
 paralyses a rather extensive hyperalgesia of the skin sometimes occurs, 
 besides the decrease of the other sense qualities commonly restricted within 
 narrower bounds. 
 
 Disorders in the secretion of sweat, hyperhidrosis or anhidrosis, reddening 
 of the skin, and a glossy skin, are among the trophic and vasomotor-secretory 
 disorders occurring more frequently. Considerable desquamation and now 
 and then edema are also seen. 
 
 The participation of the phrenic and of the cranial nerves is one of the 
 rarer symptoms of polyneuritis. Facial, eye muscle and vagus paralyses in 
 particular, less frequently optic, auditor} 7 and other forms of neuritis, are now 
 and then observed. 
 
 Very unusual are disorders of innervation affecting the bladder and 
 rectum. 
 
 Xot infrequently persons affected with polyneuritis show symptoms of a 
 psychosis. A certain symptom-complex of psychic disorders has in fact been 
 designated as polyneuritis psychosis (Korsakow). It is undoubtedly true 
 that it is found very frequently in persons affected with polyneuritis. It is 
 particularly characterized by a serious disorder of the ability to receive and 
 retain new impressions. Patients who often can still recollect events of the 
 
PARTICULAR CLINICAL FORMS OF POLYNEURITIS 169 
 
 more remote past, are entirely incapable of giving a correct account of the 
 events of the immediate past, or even the last few hours. The resulting void 
 in the memory is then filled with "fairytales" (confabulation). So, e. g., a 
 patient who for weeks had been confined to his bed with paralysis, told the 
 doctor who was making his morning round, that he had already taken a walk 
 in the morning, and had then eaten his dinner with much relish. 
 
 Particular Clinical Forms 
 
 1. Pseudotabes Peripherica. — This form, found especially in alcoholics, 
 but also after diphtheria, in those affected with diabetes and occasionally 
 from other causes, is characterized by the fact that its symptoms of lost 
 function manifest themselves preponderantly in the sensory area. Among 
 the earliest subjective complaints of the patient is a motor ataxia — which has 
 a strong resemblance to the tabetic one — of the lower, later perhaps also 
 of the upper, extremities. This ataxia, like all others, depends in particular 
 on extensive disorders of the deeper sensibility. In such cases, anaesthesias 
 of the skin occur more pronouncedly than in other cases, and are localized 
 corresponding to the ramifications of the cutaneous nerves. The Romberg 
 phenomenon is present; the reflexes are destroyed. Symptoms of motor 
 weakness, on the other hand, play only a secondary role, but at least in a few 
 nerve areas (e. g., the peroneal area) they will hardly ever be entirely absent. 
 
 2. Lead paralysis, in the great majority of cases, and especially as a 
 rule in adults, is confined to the upper extremities. It first affects the musculo- 
 spiral nerve, usually starting with the right, and going over to the left, in a very 
 peculiar selective fashion, first affecting the extensors of the fingers and those 
 of the wrist. The disease may be restricted to this narrow area. If it 
 progresses, it next seizes upon the long abductors of the thumb and the small 
 muscles of the hand supplied by the ulnar and median nerves. A further 
 extension to brachio-radial, supinator, and other arm muscles, is quite un- 
 common, while a generalization of the paralysis is a true rarity. A predi- 
 lection of the paralysis for the lower extremity, in particular, for parts of the 
 peroneal area, is most likely to be found in children. The sensibility almost 
 always remains unimpaired. 
 
 This most peculiar picture undoubtedly indicates a spinal rather than a 
 neuritic disease (Erb, E. Remak). But the numerous existing anatomical 
 reports do not permit any doubt as to its neuritic character. The peculiar 
 manner of localization is still a matter of controversy. 
 
 3. Postdiphtheritic paralysis is also distinguished by a peculiar 
 localization. Almost without exception, a motor paralysis, and sometimes 
 an anaesthesia of the soft palate (velum palatinum) is the first and often the 
 only symptom. The speech of the patients is openly nasal; liquids that they 
 are drinking partly flow back through the nose. Frequently a paralysis in 
 
170 DISEASES OF THE PERIPHERAL NERVES 
 
 accommodation accompanies the soft palate paralysis, to which it is interest- 
 ing to note, the hyperopics especially, who read much during convalescence, 
 are said to be predisposed. 
 
 In some cases, still other eye muscles, especially the external recti, the 
 muscles of the oesophagus, the vagus branches in the larynx, and other areas 
 of cranial nerves become affected. More frequent is a participation of the 
 extremities. Sometimes its only indication is the destruction of the tendon 
 reflexes of the legs. In other cases, a typical, polyneuritic paralysis of the 
 upper and the lower extremities sets in, and even further generalizations 
 occur. Instead of that, however, the picture of pseudotabes in some cases 
 combines with the palatal and accommodation paralysis. 
 
 One is inclined to regard the paralysis of the palate after diphtheria not 
 as a polyneuritic symptom, but as a kind of ascending neuritis, especially as 
 after diphtheria of the umbilicus, a paralysis of the abdominal muscles has 
 been observed. Yet on the other hand, paralysis of the soft palate and pa- 
 ralysis in accommodation have been seen to set in after diphtheritic infec- 
 tions in the arm. 
 
 The diagnosis of polyneuritis is in most cases easy. Leaving the 
 typical postdiphtheritic and lead paralysis, which, especially with a knowl- 
 edge of their aetiology, can hardly be mistaken, out of consideration, the follow- 
 ing signs of the clinical picture will serve especially to distinguish polyneuritis 
 from other extensive paralyses and paralytic conditions: its symmetrical 
 distribution; its incipiently progressive, as a rule ascending, course which 
 later has a tendency to recovery; the nature of the symptoms of lost function; 
 the paralyses connected with R. D. besides the disorders of sensation which 
 only rarely will be absent; and, lastly, the almost always obvious grouping 
 of disorders corresponding to the innervation area of peripheral nerves. Nor 
 is the psychosis of Korsakow found in those affections which are of any 
 differential diagnostic importance. 
 
 We shall now mention the most important special differentiating signs 
 of those diseases, which at one time or another, are most likely to be mis- 
 taken for polyneuritis. 
 
 In acute poliomyelitic paralysis the progressive course is lacking. It 
 sets in with all its gravity, and then retrogresses. Only in very exceptional 
 cases is its distribution symmetrical. Disorders of sensation are almost 
 always absent. Spinal progressive muscular atrophies are characterized by 
 their more creeping progress, by the lateness at which functional disorders 
 are observed, by fibrillary twitchings which are very unusual in peripheral 
 diseases, and also by the absence of disorders of sensation. Concerning 
 the differentiation (which sometimes becomes difficult) from the so-called 
 neuritic progressive muscular dystrophy, a disease which likewise sets in 
 very slowly and chronically, we refer to the chapter in this work dealing 
 with it. 
 
DIFFERENTIAL DIAGNOSIS OF POLYNEURITIS 171 
 
 Genuine tabes is distinguished from the ataxic form of polyneuritis by a 
 number of specific traits, foremost among which is reflex pupillary immobil- 
 ity; but also by other signs, some of which set in very early, such as gastric 
 crises, primary atrophy of the optic nerve, bladder disorders, which are very 
 rare in polyneuritis, and, of course, by the segmental arrangement of the 
 sensory losses. On the other hand, the paresis, which will hardly ever be 
 altogether lacking in typical tabes is absent in pseudotabes. In tabes there 
 often exists an analgesia of the nerve trunks to pressure; in pseudotabes, 
 there is an abnormal sensitiveness to pressure. 
 
 Myopathic diseases, which may lead to extensive, likewise symmetrically 
 distributed, disorders of motility (progressive dystrophies, polymyositis, 
 osteomalacial paralysis, myasthenia), lack the symptoms which indicate 
 directly a disease of the nerves, namely R. D., early decrease or destruction 
 of reflexes, and disorders of sensation. We need not mention here how each 
 individually is often revealed at a glance by certain positive signs to those 
 skilled in them. We refer only to the peculiar type of localization in the 
 various forms of dystrophy and to the inflammatory swelling of the muscles, 
 and in polymyositis, frequently of the skin also. 
 
 Chiefly, of course, in the interest of successful treatment, a complete 
 diagnosis of polyneuritis naturally requires that the aetiology, founded upon 
 objective criterions, be determined as accurately as possible. Hence it will 
 be necessary to look for objective signs of chronic alcoholism (psychic 
 changes, tremor); of lead poisoning (the blue line on the gums, basophilic 
 granulation of the erythrocytes, lead in the urine, which in some cases will 
 be revealed only after administering iodine); of an abuse of arsenic (cuta- 
 neous alterations, melanosis); of CS 2 poisoning (psychic alterations); 
 and for signs of a former or existing infectious disease or metabolic 
 affection. 
 
 In the treatment of a diphtheritic paralysis, the frequently occurring 
 myocarditic complication must be taken into consideration. In the disease 
 known as beri-beri, a myocarditis, taking the same course as the symptoms 
 of circulatory weakness, particularly edemas, is really part of the clinical 
 picture. 
 
 Course and Termination. Prognosis. — Polyneuritis may set in 
 acutely, even with fever, or may develop slowly. In some rare cases, the 
 disease progresses rapidly from day to day, and soon reaches its highest 
 point of development. More frequently, however, the symptoms require a 
 number of weeks, or even months for their full development. The more 
 chronic development progresses steadily, or, sometimes, by fits and starts. 
 Carelessness of the patient, such as excessive indulgence in alcohol, may at 
 times lead to an exacerbation of the symptoms. 
 
 It has already been pointed out in the discussion of symptomatology, 
 that different cases attain to varying degrees of extension. Some, in fact, 
 
172 DISEASES OF THE PERIPHERAL NERVES 
 
 remain rudimentary. In the form described as the fundamental type, the 
 affection sometimes does not spread beyond the lower extremities. 
 
 The prognosis in general is usually exceedingly favorable. 
 
 A fatal issue, caused by the affection alone, is a rare exception. Some 
 cases taking a highly acute and rapidly ascending course, do, indeed, 
 result in death in a short time. Paralyses of bulbar nerve areas, and of 
 respiration, which latter are often of such nature as to be difficult of explana- 
 tion, frequently cause death. Even in cases taking a less violent course, 
 bilateral phrenic and vagus paralyses may threaten life by their various 
 attendant dangers. It is hardly necessary to mention that for persons whose 
 general condition is wretched, the length of the disease may become serious, 
 or that the gravity of the primary disease may endanger life. 
 
 The great majority of cases, as we have already said, recover. Some- 
 times a complete restoration takes place after a few weeks. Even in wide- 
 spread cases this occurs, if the paralyses and the alterations of electric 
 irritability do not attain a very high degree. But in most cases, nevertheless, 
 it takes many months or even longer in grave cases, to recover. Lasting 
 defects may usually be avoided. 
 
 Naturally much depends on the possibility of completely eliminating 
 the causal injuries. Alcoholics who do not renounce alcohol, those affected 
 with lead poisoning who continue to receive the poison into their system, 
 cannot hope to recover. 
 
 Only a few words concerning the pathological anatomy are necessary 
 to supplement the discussion in the general part. It is a matter of a primary 
 degenerative process, a parenchymatous neuritis. The peripheral ramifica- 
 tions of the nerves are more likely to be seriously affected than the large 
 trunks and roots. It seems that the fibre disease, which is typical of poly- 
 neuritis, is the degenerative form beginning in the periaxial medullary 
 sheaths. Slight changes of a like nature are frequently found in nerve areas, 
 which have not given evidence of any symptoms of lost function (latent 
 neuritis). The unequal, intermittent extension of this destructive process 
 makes it possible that Wallerian degeneration sets in in a fibre segment which 
 at first remained normal, when the change in the neighboring proximal 
 parts has progressed to the destruction of the axon. One is inclined to 
 explain in this manner the discovery of fibres which show the Wallerian 
 degeneration type, in seriously diseased nerves. In cases of long standing 
 numerous fibres disappear wholly (Figs. 38 and 39). 
 
 The changes in the connective tissue of the nerve, as a rule, appear to 
 be secondary. Pure inflammatory focal affections of the nerve branches 
 and trunks are found now and then, but they are always quite circumscribed 
 in extent and are an inconstant accompanying symptom, showing nothing 
 typical. 
 
 In the spinal cord and spinal ganglia are often found those slight changes, 
 
THERAPY OF POLYNEURITIS 
 
 173 
 
 
 Fig. 38. — Fresh polyneuritic degenera- 
 tion. (After Balz and Miura.) Teased 
 preparation of the peroneal nerve in a case 
 of beri-beri. Osmium staining. In some 
 fibres pronounced periaxial medullary dis- 
 integration; in others apparently Wallerian 
 degeneration. 
 
 closely related to peripheral nerve diseases, which have been discussed in 
 the general part. In passing we may remark that besides these, complicating 
 focal affections of the brain and spinal cord may sometimes occur in some 
 forms of polyneuritis, as inflammatory and other cerebral focal affections in 
 diphtheria, and the so-called encephalo- 
 pathia saturnina in lead poisoning. 
 
 The therapy of polyneuritis requires 
 but a brief discussion, since the impor- 
 tant general rules which apply to poly- 
 neuritis as well, have been dealt with at 
 length in the discussion of the paralyses 
 of the single nerves. Because of this 
 we may pass over the requirements of 
 absolute rest in new cases, of a suitable 
 diet, of proper position, in connection 
 with which especially the prophylaxis of 
 passive contractures should be con- 
 sidered and of other requirements. 
 
 In order to treat the causal conditions properly, we should insist on total 
 abstinence in alcoholics, eliminate the poison in those affected with lead 
 poisoning by administering iodide of potassium (2 to 3 times daily 0.5 (5 grs.) 
 
 in solution) , and treat malaria with 
 quinine, diabetes with a proper 
 diet and syphilis energetically with 
 mercury. Any previous inunctions 
 should not be regarded as inter- 
 fering with this, unless, indeed, 
 symptoms of mercurial poisoning 
 should exist. Some cases which in 
 spite of the treatment continue to 
 progress in the beginning, will often 
 recover completely in a remarkably 
 short time if the treatment is con- 
 tinued longer. 
 
 No definite rule can at present 
 be established concerning the diffi- 
 cult question of interrupting preg- 
 nancy in pregnancy polyneuritis 
 (cf. von Hosslin, Arch. f. Psychia- 
 trie, vol. xl, and Munchner med. Wochenschr., 1905, page 636). 
 
 For the rest, the use of moist, warm packings of the whole body, or of the 
 diseased parts is to be recommended in the early stage. They may be 
 applied daily from 1 to 2 hours and will almost invariably prove beneficial, 
 
 Fig. 39. — Polyneuritis alcoholica in a later 
 stage. (After Jacob.) Cross-section of the n. 
 ischiadicus. In the proliferated connective tissue 
 only a few isolated (black colored) fibers are 
 preserved. 
 
174 DISEASES OF THE PERIPHERAL NERVES 
 
 particularly in painful cases. Suitable for every stage — in the earliest for 
 those patients, at least, who are not too sensitive — are thermal baths of 
 96 to 98 F., continued on the average for half an hour, taken 3 to 6 times a 
 week. Sometimes medicinal preparations (pine needle extract, salt, carbonic 
 acid, etc.) , may be added. Frequent diaphoretic procedures such as hot air 
 baths in the bed, are also urgently recommended for both the early and later 
 stages of the disease (Oppenheim) . 
 
 The tonic pills mentioned before should be used rather than other med- 
 icinal remedies, to aid the general treatment. 
 
 For the pains, antineuralgic agents or, if necessary, also the stabile anodic 
 galvanization are used besides warm local applications. 
 
 When the affection does not progress any further, and especially during 
 convalescence, the other electro-therapeutic means, massage, and passive 
 and active gymnastics should be used according to the rules mentioned above. 
 Careful passive movements may sometimes prove advantageous even in the 
 earlier stage. 
 
 As after cures, thermal bathing resorts furnished with good medico- 
 mechanical institutions are particularly to be recommended. 
 
 Careful treatment will obviate the necessity of having to remove any 
 residual defects by means of orthopedic surgery. 
 
 Naturally any grave special occurrences during the course of the disease 
 will require special treatment. In deglutition paralyses it is better to have 
 recourse to artificial feeding than to risk an aspiration pneumonia. In grave 
 acute disorders of respiration, the life of the patient may be saved by artificial 
 respiration (Ebstein). 
 
 Supplement 
 
 A. Landry's Paralysis, Paralysis Ascendens Acuta 
 
 As Landry's paralysis (Landry, 1859) all those cases are commonly 
 designated, in which a severe and flaccid paralysis begins acutely in the 
 feet, thence progresses rapidly and steadily to all remaining muscles of the 
 legs, trunk, arms, neck and the bulbar nerve areas. In sporadic cases the 
 onset of the disease is marked by a febrile attack. The reflexes are destroyed 
 and usually there are slight disorders of sensibility. As a rule, no real pains 
 are experienced. Disorders of bladder or rectum are found only exception- 
 ally. Death generally results in the first few days or the second week (rarely 
 later) from suffocation or respiratory failure. Some cases recover slowly. 
 
 A descending course of the disease has also been described. 
 
 It is undoubtedly true that a polyneuritis acutissima may occasion this 
 clinical picture. But it would be wrong, simply to identify Landry's paralysis 
 with polyneuritis. Some postmortem findings seem to indicate that it may 
 also be caused by an ascending myelitis. Besides there are some cases in 
 
LEPROSY OF THE XERVOCS SYSTEM 175 
 
 which a remarkable condition of electric irritability exists which is incom- 
 patible with either polyneuritis or myelitis. Autopsies very rarely afford a 
 satisfactory explanation of the clinical picture. 
 
 At any rate it is a matter of a toxic or infectious disease. It may 
 appear in connection with certain infectious diseases and toxic processes. 
 Swelling of the spleen and albuminuria may follow in its wake. In not a 
 few cases micro-organisms of various kinds have been found in the tissues 
 of the body, particularly in the central organs. 
 
 It is at present impossible to distinguish clearly from each other and to 
 classify the cases of Landry's paralysis. 
 
 B. Leprosy of the Nervous System 
 
 Leprosy of the nervous system is now and then very erroneously simply 
 designated as polyneuritis leprosa. 
 
 Grave disorders of cutaneous sensibility, in its earlier stages, as a rule, 
 extending irregularly in spots, or appearing in segmental radicular zones and 
 afterwards covering extensive areas, even the whole body, sometimes 
 affecting all qualities, sometimes only one or more, stand in the foreground 
 of the clinical picture (lepra ana?sthetica\ The sensibility of the deeper 
 parts usually remains unimpaired, for which reason ataxia hardly ever 
 occurs. The anaesthesias combine with muscle atrophies, usually only in 
 limited areas, among which there is a predilection for the small muscles of 
 the hand, and with grave ''trophic" disorders, which partly depend un- 
 doubtedly upon the nerve disease and partly constitute symptoms of local 
 leprous changes leading even to loss of entire ringer joints (lepra mutilans). 
 The state of the reflexes is always irregular. Typical paralyses of peripheral 
 nerves appear only in exceptional cases. 
 
 It is obvious that this clinical picture may be mistaken for syringomyelia 
 more easily than for polyneuritis, despite the fact that both halves of the 
 body may be affected almost symmetrically. It may be very difficult to 
 distinguish it from syringomyelia. But usually the characteristic spotted 
 and nodular leprous changes of the skin will be found in leprosy of the 
 nervous system, while in syringomyelia a few symptoms, like the spastic 
 palsy, will manifest themselves, which as a rule are not found in leprosy. 
 
 The pathological-anatomic explanation of the picture of "lepra ner- 
 vorum" is not at all conclusive as yet. It is certain that alterations of 
 minute nerve branches of the skin together with leprous cutaneous diseases 
 play a role in it. But besides these, there are also, perhaps ascending, 
 bacillary invasions of the larger trunks, the spinal ganglia and the spinal 
 cord, with or without consecutive, especially interstitial, changes (neuritis 
 interstitialis) . Occasionally lepra nodules on the larger nerve trunks destroy 
 them. Just how often primary polyneuritic degeneration of the nerves 
 
176 DISEASES OF THE PERIPHERAL NERVES 
 
 occurs in such cases as a result of a general toxaemic disposition, can not 
 be definitely ascertained. 
 
 II. THE NEURALGIAS 
 
 Neuralgia is used to denote a pain, appearing paroxysmally and spread- 
 ing along the course of a peripheral nerve. That it is a matter of an anatom- 
 ically limited disease of the peripheral nervous system, cannot be doubted, 
 since it appears in such a localized manner. 
 
 The pathologic-anatomic fundamental elements are by no means defi- 
 nitely known. It may be assumed that it is a matter of irritating in- 
 fluences affecting the nerve in some point or other of its course. In some 
 forms, indeed, as we shall see below, pathologic changes of the nerve or in its 
 vicinity are known, which may cause the irritation. How and when the 
 characteristic attacks take place after the nerve is affected by an apparently 
 permanent injury, is still undetermined. 
 
 The nerve trunks themselves, as seen under the microscope, are in many 
 cases entirely normal. The slight diffuse neuritic changes which occur in 
 some cases, are probably only in exceptional instances related directly to 
 neuralgia. As a rule, neuritis does not manifest any genuine neuralgic 
 symptoms. On the other hand, slight neuritic symptoms may often appear, 
 even clinically, in neuralgia. 
 
 ^Etiology 
 
 In many cases neuralgia follows infectious diseases. Probably the most 
 frequent one in Germany is that which appears in the area of the su- 
 praorbital nerve during or after influenza. In persons who have suffered 
 from malaria, attacks of neuralgia may take place at the same typical in- 
 tervals at which the attacks of fever appeared (masked malaria) . Second- 
 ary and tertiary syphilis may lead to neuralgia; in the tertiary form it is 
 usually produced by perineuritic or paraneuritic gummatous processes. 
 
 Now and then neuralgia, especially the form of it accompanying herpes 
 zoster, appears under a clinical picture of an independent infectious disease, 
 and even, according to some reports, as small endemics. 
 
 Toxic processes do not play an important part in neuralgia. Very 
 prominent, however, is the predisposing significance of metabolic diseases, 
 foremost among which is diabetes mellitus. 
 
 Colds are frequently given as causes of neuralgia. It is especially 
 difficult then not to recognize such connection between the two, when that 
 part of the body which was affected by the cold becomes the seat of neu- 
 ralgia (sciatica after sitting on the cold floor, etc.) . 
 
 Of special interest are those cases in which a chronic irritation of a certain 
 part of the nerve trunk may be directly proven. We mention tumors in the 
 
NEURALGIA 177 
 
 broadest sense of the word, osteitis and periostitis, bone fragments, scars and 
 inflammatory processes in the neighborhood of the nerve. Tumors of the 
 nerve itself may be disclosed by true neuralgia, especially in the early stage. 
 Diseases in the area of the nerve roots, the vertebrae and meninges must be 
 considered. 
 
 The pathogenic irritation may proceed also from the peripheral extension 
 area of the nerve. Various inflammatory processes, cicatrices and many 
 painful alterations of a chronic nature may occur. In amputation neuromata 
 the patient often imagines that he feels the pain they occasion in the part that 
 has been removed. 
 
 According to an old theory neuralgia may be excited reflexly from a 
 distance. Thus it is said that neuralgia may develop in a distant nerve area 
 from the intestines because of intestinal worms; from female genital organs 
 because of a reflexio uteri, etc. This, however, is very questionable. 
 
 Furthermore transient mechanical irritations of a nerve may result in a 
 neuralgic condition, though the pathologic connection can not be explained 
 in detail. To this class belong neuralgia after contusion of the affected 
 part of the body and postpartum sciatica. 
 
 Finally, severe functional strain upon some parts plays a certain role. So, 
 a supraorbital neuralgia is often seen after any work in which the eyes were 
 strained and a long continued one sided strain upon one leg will result in a 
 sciatica of that leg. 
 
 Lastly we mention that general debility seems to develop a tendency to 
 neuralgia. 
 
 Neuralgias are distinguished as symptomatic and idiopathic. All those 
 cases in which a local or general fundamental disease may be found are called 
 symptomatic. As long as it is impossible to find such disease, the neuralgia 
 is called idiopathic. It will be noted that this classification is not based on 
 any important internal difference, and is really worthless. Strictly speaking, 
 neuralgia is always only a symptom. 
 
 The practical result of this, as we shall point out also later, is the necessity 
 of looking carefully for a palpable cause in every case. 
 
 Such a conception also makes it unnecessary to separate from neuralgia 
 pains of a true neuralgic character manifesting themselves in other organic 
 nerve diseases, such as basal cerebral tumors, meningitic processes. 
 
 Clinical Picture 
 
 As a rule, the attack of pain is preceded by a brief premonitory parses- 
 thesia. It very soon attains its highest point. Pains have been described as 
 splitting, burning, boring, cutting and pricking, and very frequently they are 
 intolerably violent. Asked for the seat of the pain, the patient will often 
 indicate with his hand or finger the exact course of the diseased nerve. In 
 grave or protracted neuralgia, an irradiation frequently takes place, so that 
 
178 DISEASES OF THE PERIPHERAL NERVES 
 
 the whole vicinity of the nerve begins to pain also. Neuralgia of one nerve 
 may be accompanied by pains in its secondary branches and its neighboring 
 nerves. 
 
 The attack may pass away in a few minutes or last for hours. 
 
 The attack sets in either spontaneously or is excited by certain external 
 causes, among which are various sensory irritations, particularly those that 
 affect the diseased area, as touch, shock, draughts of air, thus movements and 
 exertions of the diseased area, for instance eating in trigeminal neuralgia, 
 walking in sciatica, and finally coughing, pressure, mental excitement and 
 changes in the weather. 
 
 Especially in chronic cases, and most frequently in sciatica, slight pains 
 are often either constant or nearly constant, and the attacks appear then as 
 temporary more or less pronounced exacerbations. 
 
 Of foremost diagnostic importance among other signs of the disease are 
 the so-called pressure points (Valleix, 1852). These limited points of exceed- 
 ing susceptibility to pressure are found in places where the nerve has been 
 slightly irritated mechanically, where it rests exposed upon a hard founda- 
 tion, where it emerges from bony canals or where it pierces a fascia. The 
 tenderness to pressure persists usually in the intervals between the attacks; 
 rarely during the latter alone. It is said that it may even be lacking entirely. 
 Slight pressure on the pressure point is liable to excite an attack, while strong 
 pressure is often soothing. Less often the entire course of the nerve is 
 tender to pressure. Trousseau regarded as very important the regular sus- 
 ceptibility to pressure of certain spinous processes in different neuralgias. 
 
 The disease is further accompanied by vasomotor, secretory and trophic 
 disorders. Reddening and a slight swelling of the skin in the area of the 
 pain is frequently seen during the attack and finally may become habitual. 
 More extensive cutaneous changes are often occasioned by mechanical 
 mistreatment, to which many patients expose the affected parts during the 
 attack. Occasionally hypersecretion of glands situated in the area of the 
 injured nerve, especially a surplus flow of tears and saliva in neuralgia of the 
 fifth nerve are seen. Among trophic disorders anomalies of hair-growth 
 occur. By far the most important is herpes zoster, which will be discussed 
 briefly in a supplement to this chapter. 
 
 In the motor area, tonic tension and clonic spasms of the muscles in the 
 affected parts are sometimes seen during the attack of neuralgia. A tic 
 convulsif may combine with the "tic douloureux," in trifacial neuralgia. 
 The great care which patients bestow upon the affected member, occasions 
 a kind of pseudo-paresis. The muscles of the affected area become more 
 or less wasted in all graver and more protracted cases. As a rule, however, 
 true pareses or paralyses do not occur, except in a minority of cases in which 
 a local progressive process forms the basis of neuralgia, which in time leads 
 to destruction of the motor nerve fibres. 
 
NEURALGIA 179 
 
 Sensory disorders are frequently represented by hyperesthesias of the 
 skin. Slight hyperesthesias in peripheral neuritic and sometimes in radicular 
 areas, also occur often. 
 
 Weakening or loss of a reflex, such as the Achilles tendon reflex in sciatica, 
 is in grave cases very common. 
 
 It is hardly necessary to mention that the general condition of the patient 
 may be greatly injured by the disease. 
 
 But little remains to be added concerning the special conditions of a 
 few quite important single neuralgias. 
 
 Trifacial {trigeminal) neuralgia, aside from sciatica, is by far the most 
 frequently occurring form. Usually only one branch, the first in most cases, 
 is affected. The most important pressure points are situated at the supra- 
 orbital foramen for the first branch, at the infraorbital and zygomatico- 
 facial foramina for the second, and at the mental foramen for the third. It 
 is probably only in syphilitics that a bilateral neuralgia of the auriculo- 
 temporal occurs, the pain area of which, in the form of a child's comb, 
 extends across the crown from one ear to the other, and whose pressure 
 point is situated on the zygomatic arch in front of the ear (Seeligmueller). 
 In neuralgia of the first branch, the pain usually extends along the ramus 
 supraorbitalis. In the area of the second branch a predilection for the rami 
 infraorbitalis and alveolaris, next to the zygomatic nerve is shown, in the 
 area of the third branch, for the ramus alveolaris inf. 
 
 Among the causes, the affections in the extension area of the nerve, such 
 as diseases of the nose and its cavities and especially of the teeth, play a 
 special part particularly in trifacial neuralgia. Even the sharp edge of the 
 shrunken alveolar processes in persons who have lost their teeth may excite 
 a neuralgia. 
 
 Hence the advice of a dentist rarely can be dispensed with. A random 
 extraction of sound teeth, such as has been observed occasionally, is of 
 course wholly inadvisable. 
 
 If in herpes zoster trigemini the cornea is affected, the aid of an oculist 
 is to be sought. 
 
 In occipital neuralgia, which chiefly affects the major occipital nerve, and 
 which belongs to the frequently bilaterally occurring neuralgias, tumors of 
 the posterior cranial fossa and affections of the uppermost vertebrae are 
 aetiologically to be considered. The main pressure point is situated on the 
 linea nuche sup. at the point where the nerve comes to the surface through a 
 hiatus in the muscle. 
 
 True brachial neuralgias are very rare. They are most likely to occur 
 (either uni- or bilaterally) in affections of the meninges or vertebras and in 
 tumors in the root or plexus area. In an etiological search cervical ribs 
 must also be sought for. 
 
 Intercostal Neuralgia. — Among special etiological factors are affections of 
 
180 DISEASES OF THE PERIPHERAL XERVES 
 
 the ribs, various intra-thoracic diseases, space contracting processes such as 
 aneurisms and severe scoliosis, although, of course, the pains in the chest 
 occasioned by all these diseases are only now and then of a truly neuralgic 
 character. The pressure points are situated in the intercostal space near 
 the vertebrae, in the mid-axillary line and near the sternum. 
 
 A neuralgic affection of the nerves of the mammary glands which appears 
 especially after trauma during pregnancy and lactation, is designated as 
 mastodynia (irritable breast, Astley Cooper). 
 
 Neuralgias of the lumbar plexus nerves, neuralgia lumbo-abdominalis, 
 cruralis, etc., are very rare. 
 
 Sciatic Neuralgia (Ischiadica). — Sciatica, the malum Cotunnii, 
 (Cotugno, 1764) is the most frequent form of neuralgia. Among the special 
 causes are diseases of the os sacrum and pelvic bones, tumors and exudations 
 of the pelvis, displacements of the uterus, sciatic hernia and chronic consti- 
 pation. A manual examination of the pelvic organs, through the rectum, 
 should never be omitted in any case of sciatica. 
 
 In bilateral sciatica there is always a suspicion of a meningeal or vertebral 
 affection. Next to that, it is probably most likely to occur in diabetes. 
 
 Sciatic neuralgia may be restricted to single branches, like the posterior 
 cutaneous. 
 
 The pressure points are situated at the great sacro-sciatic foramen, in the 
 gluteal crease, in the popliteal space, in the middle of the calf, at the head 
 of the fibula, behind the ankle, sometimes also laterally from the tubercles 
 of the os sacrum, somewhat below the middle of the crest of the ilium and 
 in other places. 
 
 Important for the diagnosis is the pain when stretching the nerve. 
 Flexion of the hip joint when the knee is in extension excites violent pains, 
 while no pains are occasioned if the same movement is executed with the 
 knee flexed (phenomenon of Lasegue). 
 
 The patient is apt to carefully avoid all movements which might produce 
 such stretching and he likewise avoids exposing the back part of the leg, 
 and through it the nerve, to any pressure. Most patients keep the leg 
 slightly flexed at hip and knee both when lying and walking and rotate it 
 somewhat outward. When standing or walking they throw the weight of 
 the body on the healthy leg. The trunk is slightly lowered towards the 
 healthy side (sciatic scoliosis). In some rare cases, however, a slight 
 deviation of the spinal column in the opposite direction takes place. The 
 origin of the scoliosis is still very doubtful (Figs. 40 and 41). 
 
 The reflexes of the affected leg are not infrequently slightly increased. 
 It has already been mentioned that sometimes they are also weakened. 
 Occasionally a peculiar kind of '''pseudo-Babinski" reflex is found, whose 
 nature has not yet been explained. 
 
 The diagnosis of a typical neuralgia is easy, if strict regard be given 
 
DIAGNOSIS OF NEURALGIA 
 
 181 
 
 to the main symptoms, which have been mentioned in the definition, the 
 anatomic extension and the paroxysmal character and course of the pain. 
 Difficulties ought to arise only in atypical forms. 
 
 When the course as to the paroxysms is not clearly pronounced, as 
 occurs in cases of long standing and especially also in sciatica, the other 
 symptoms, particularly the localization of pain and perhaps also the anam- 
 
 Fig. 40. Fig. 41. 
 
 Figs. 40 and 41. — Sciatica at the left side. Characteristic posture of the leg, scoliosis ischiadica. 
 
 (Leipsic Medical Clinic.) 
 
 nestic statement that characteristic attacks occurred before, will frequently 
 permit of a correct diagnosis. 
 
 Where the localization in the course of a certain peripheral nerve cannot 
 be authenticated, especially in neuralgoid conditions, in which the pain is 
 confined to a very narrow area, it will as a rule, be impossible to come to 
 any definite conclusion. This is true for instance of the so-called joint 
 neuralgia, achillodynia, mastodynia, coccygodinia and visceral neuralgias. 
 
 In all doubtful cases it should be ascertained whether other, especially 
 inflammatory affections, do not cause the existing pain. As we have seen, 
 
1 82 DISEASES OF THE PERIPHERAL NERVES 
 
 the existence of such a disease, of an inflammation, or of a tumor would not 
 necessarily exclude the existence of neuralgia. The latter may exist inde- 
 pendently of such affections, but, on the other hand, may not only be 
 unfavorably influenced by them, but even caused by them. It will therefore 
 always be a question of whether criteria of neuralgic pains are present or 
 whether the disorders correspond simply in nature and course to the other 
 disease which has been found. 
 
 We shall now mention a number of diseases, which as experience has 
 taught us, are frequently, with but a superficial examination, mistaken for 
 neuralgia. Thus neuralgic pains are easily confounded with the acute and 
 radiating pains in diseases of large joints of the extremities, especially of 
 the hip and shoulder joints, in hereditary deformities such as flat-foot, in 
 affections of the arteries and veins (intermittent claudication, varicose veins), 
 and in occupation neuroses. The so-called lymphangitis rheumatica 
 (Wilms 1 ) must also be considered. 
 
 Catarrh of the frontal sinus and even common attacks of migraine have 
 occasioned a wrong diagnosis by being mistaken for trifacial neuralgias and 
 beginning pleurisy has been confounded with intercostal neuralgia. 
 
 Of a more general, differential diagnostic significance are muscular 
 rheumatism, tabetic pains, spasms, neuritis, those reflex pains and hyper- 
 esthesias in visceral affections which have been investigated mainly by 
 Head, to which perhaps belong the pains in angina pectoris, and psychogenic 
 pains. 
 
 In all these painful diseases the characteristic signs of neuralgia, aside 
 from those rarer cases which are combined with a true neuralgia, are of 
 course lacking. We can not here deal with their positive symptoms. 
 
 The differential diagnostic significance of psychogenic pains is con- 
 siderably enhanced by the fact that in some cases, particularly in individuals 
 with a nervous predisposition and in those who because of injuries claim 
 insurance money, they may add themselves to true neuralgia and strongly 
 resemble it. At any rate, it may be seen in such cases how the type of the 
 neuralgic picture becomes more and more obliterated, while the signs of 
 the "psychalgia" (Oppenheim), the determination of the form and course 
 of the disorders by certain ideas, the dependence upon attention and expecta- 
 tion, becomes more and more pronounced and exclusive. Perhaps in those 
 cases of neuralgia, which ostensibly have been cured by hypnotism, it was a 
 matter of such psychogenic residual images. 
 
 Once the presence of neuralgia has been conclusively determined, the 
 investigation should continue as to the aetiology. The general condition of 
 the body should be carefully considered; one should search for infectious 
 and toxic conditions and for metabolic disturbances. Next, those parts 
 through which the diseased nerve takes its course, should be closely scruti- 
 
 1 Miinchener med. Wochenschr., 1906, page 1595. 
 
PROGNOSIS AND THERAPY OF NEURALGIA 183 
 
 nized, to see whether any alterations exist which might sustain or produce 
 the irritated condition of the nerve. Above all it should be discovered 
 whether some grave, progressive, local disease is not hidden behind the 
 neuralgia, such as a malignant neoplasm or something of the sort. Thus, 
 for example, a tubercular meningitis may begin under the innoxious picture 
 of a sciatica. Bilaterality, which is not infrequent, rise in temperature, the 
 proof of other tubercular diseases or of a tubercular diathesis, may early 
 confirm one's suspicion. 
 
 The prognosis should always be made with caution, even in those 
 cases where it is believed that a graver fundamental disease does not exist. 
 The prospects are comparatively favorable in many post-infectious neu- 
 ralgias. A neuralgia following influenza may recover in a few days. Dia- 
 betic neuralgia often disappears after one succeeds in removing or at least 
 considerably diminishing the glycosuria. In the majority of cases a neu- 
 ralgic affection lasts a number of weeks or a few months. Even its con- 
 tinuation for several years is by no means uncommon. 
 
 Old age, a poor general condition of the patient, a long course and 
 particularly the severity of the disease all darken the prospects for recovery. 
 In many cases the tendency to relapse is quite marked. 
 
 Therapy. — On account of the many sided setiological relations of 
 neuralgia great care is required in its treatment. The general condition 
 should be improved as far as possible. Any existing morbid disorders, 
 particularly those, of course, that from experience are known to have been 
 factors in the origin of neuralgia, should be taken up for treatment. After 
 that one should endeavor to remove the local causes. 
 
 Abstinence from alcohol is almost invariably to be advised; the use or 
 non-use of coffee or tea must be decided upon individual grounds in each 
 case. Experienced authors warn against a one sided diet, unless it is very 
 definitely indicated. Regulating the bowels is of great importance in all 
 cases; in a few rare cases it has actually direct curative value. 
 
 Particularly in recent cases it is advisable to keep the affected parts in 
 a condition of absolute rest for some time. Recent cases of sciatica neces- 
 sitate confinement to bed for two or three weeks, longer if the condition of 
 the patient requires it. It will sometimes be advisable, to secure rest for the 
 diseased parts in a way most comfortable to the patient by means of splints 
 or by other fixation methods. 
 
 From the abundance of various remedies we have selected the following 
 important ones, which have stood the test of time. 
 
 Among the medicinal agents, quinine, iron, mercury and the iodides play 
 their well known role in the specific setiological treatment of malaria, anemia, 
 syphilis and lead poisoning. 
 
 A general treatment with tonics, particularly arsenic and strychnine, and 
 with the ubiquitously tried iodides is believed to be useful in many cases. 
 
1 84 DISEASES OF THE PERIPHERAL NERVES 
 
 The antineuralgics occupy a foremost position. In recent cases, a 
 regular use of several (say about 3) doses daily is advised, afterwards they 
 may be kept in reserve to be used when necessary. Of the great number of 
 these agents we mention antipyrine (0.5 to 1.0 per dose); phenacetine (0.5); 
 antifebrin (0.25); migrainine or antipyreticum compos, Riedel (1.0); pyr- 
 amidon (0.3) ; lactophenin (0.5); aspirin (0.5 to 1); quinine sulfat. or muriat. 
 (0.5). When the single preparations do not prove successful, mixtures may 
 often prove of value, such as antipyrine 0.5, phenacetine antifebrin aa 0.25, 
 1/2 powder for one dose. Combinations with bromine and with codeine 
 are to be recommended. As another agent adapted to alleviate pain, we 
 mention butylchloral. We caution against the use of morphine, in view 
 of its uncertain action, and the danger of morphinism, since the duration of 
 the disease can not be foretold. In extreme cases single doses of mor- 
 phine or its derivatives (heroin, etc.), will have to be given, just as one 
 will sometimes have to resort to a soporific (chloral hydrate). In some 
 exceptional cases, atropine is found to be soothing where morphine has 
 failed. 
 
 Hydro- and Thermo -therapy. — Of the general applications in recent, 
 not very sensitive, cases warm baths of half an hour's duration, or hot baths 
 for a shorter time, taken several times a week should be considered, also the 
 more energetic, diaphoretic hot air baths in bed (they may be taken even by 
 the most seriously affected patients), by means of such apparatus as that of 
 Hilzinger, of 120 to 140 F., and about 1/4 to 1/2 hour length. Later, espe- 
 cially as after-treatment, thermal baths, carbonic acid and brine baths may 
 be recommended. 
 
 Local thermic procedures are usually applied upon the painful places 
 and preference will almost always have to be given to warm or hot applica- 
 tions. Moist or dry, warm or hot bandages, cataplasms, thermophores or 
 similar applications, packing in peat or mud, sand baths, hot air baths, most 
 conveniently in Bier's apparatus, radiations from photo-therapeutic appara- 
 tus are successfully prescribed, those most convenient to the patient in the 
 beginning, the others in the more advanced stages of the affection. 
 
 From the group of the so-called derivative agents which have stood the 
 test of experience, we recommend especially dry cupping glasses, to be used 
 even in recent cases and applied daily along the course of the affected nerve 
 in number corresponding to the extension of the painful area. Embrocations 
 which irritate the skin, tincture of iodine applied with a brush, plasters which 
 produce cutaneous irritations and which may be left on for several days 
 (emplastr. cantharid. perpet. and empl. oxycroceum, etc.), applications of 
 menthol spirits and menthol oil under covering compresses, perhaps some- 
 times even real vesicants (emplastr. cantharid. ordin.) may all be used to 
 alleviate the pain. Blood letting, applications of moxae, etc., should be 
 resorted to but seldom. 
 
THERAPY OF NEURALGIA 185 
 
 The chief method of electro-therapy is the stabile anodic galvanization 
 of the main points of the pain. 
 
 A button shaped electrode (surface 5 to 10 qcm.) is placed on the pressure 
 point; a larger one, the cathode, is placed as the dispersing electrode on any 
 point in the healthy area, as for instance, in sciatica in the region where the 
 nerve originates (os sacrum region). In order to attain success it will be 
 necessary, when beginning and ending the treatment, to increase and 
 diminish the current very carefully and to avoid all irritating influences. 
 Weak currents of about 2 ma., when the nerve is situated deeper, 3 to 4 ma., 
 should be used. Seances to last from 2 to 5 minutes. Occasionally more 
 protracted applications may be tried. At the same sitting various pressure 
 points, each for about 2 minutes, may be treated successively. Galvanic 
 treatment is given from 3 to 7 times a week; in exceptional cases even twice 
 daily. If applied correctly the result will not infrequently prove a direct 
 palliation of the troubles. 
 
 Besides this preeminently successful method, a great number of others 
 have been recommended. Particularly in inveterate cases, for instance of 
 sciatica, it may be expedient to place both electrodes (about 10 qcm. in size 
 each) proximally and distally on the affected nerve and to let somewhat 
 stronger currents (about 5 to 6 ma.) flow through it for about 5 minutes. 
 During this treatment the position of the electrodes may be changed. It 
 seems to make no difference whether the "ascending" (cathode proximal) 
 or the "descending" current is used. 
 
 Faradism is not as valuable as galvanization. In the lighter cases. of 
 long standing faradization of the skin and muscles of the affected area is 
 sometimes beneficial. The treatment of painful places by means of the 
 faradic brush should be tried only in exceptional cases. 
 
 Mechano-therapy. — In inveterate cases, particularly of sciatica, mas- 
 sage of the affected parts is exceedingly serviceable. Rubbing and knead- 
 ing may be followed by vibratory massage of the affected nerve along its 
 entire exposed course. In recent cases massage almost invariably proves 
 a failure. 
 
 Stretching the nerve, which today is probably used only in the form of so- 
 called bloodless stretching, may be appropriately combined with massage. 
 For example, in sciatica after the leg has been massaged and is in extension 
 at the knee it should be lifted from its support very slowly, so as to occasion as 
 little pain as possible and then be flexed in the hip joint more and more. 
 Avoiding all rough manipulations one should proceed as far as the patient 
 can bear it, then keep the leg in that position for 1 to 2 minutes, and then 
 perhaps after a final, stronger stretching, let the leg down slowly. Manual 
 stretchings of aching cutaneous areas are recommended also, and have 
 undoubtedly been used in some cases, in which they afforded an instantaneous 
 palliation of pain (Naegeli). 
 
1 86 DISEASES OF THE PERIPHERAL NERVES 
 
 Climatic therapy is of no considerable importance. In general, very 
 changeable, irritating, cold or moist climates should be avoided. 
 
 Of the many methods of injection of different liquids into the nerve trunk 
 or its immediate vicinity we recommend chiefly a trial of the method given by 
 Lange, which is almost entirely painless. It consists in a paraneuritic 
 injection, repeated when necessary, of rather large quantities (about ioo ccm. 
 in sciatica) of a solution of eucaine-/? in a physiological solution of cooking 
 salt (cf. Munchener med. Wochenschrift, 1904, No. 52, page 2325). This 
 causes a rapid palliation and even stopping of the pain, and in some cases 
 leads to a rapid and permanent recovery. 
 
 Among the surgical methods, which are discussed more fully elsewhere 
 in this volume, neurolysis, releasing the nerve from its irritating surroundings 
 and neurinsarcoclesis may be ranked among the treatments removing the 
 cause. The mutilating operations of neurotomy and neurectomy, of neur- 
 exairesis and of extirpation of ganglia, can be advised only in those cases 
 in which the resources of internal medicine have been exhausted. 
 An operation should not be performed before careful galvanic treatment, 
 which even in cases of old standing often rewards us with surprising results, 
 has been applied by an expert. Even surgical methods do not insure a 
 permanent cure. 
 
 SUPPLEMENT 
 
 Herpes Zoster 
 
 Herpes zoster consists of an eruption accompanied by pain. Quite 
 rapidly there develop one after another groups of vesicles on a reddening 
 surface. These localized groups have a highly characteristic lengthwise 
 formation in a stripe-like area, corresponding to the innervation area of a 
 certain nerve root, or sometimes of a peripheral branch. The affection which 
 is almost invariably unilateral, most frequently occupies the area of an inter- 
 costal nerve, a somewhat beltlike region on one side of the thorax, whence 
 its name. Next to this, the trigeminal area is most often affected. Some- 
 times the vesicles disappear after a few days, leaving either brown spots, 
 which later disappear, or, if the efflorescences were hemorrhagic or gangren- 
 ous, white scars typically grouped. 
 
 Anatomically, with the greatest regularity a disease of the peripheral 
 sensory neuron, especially of the spinal ganglia, but not infrequently also of 
 the peripheral nerves, and frequently true inflammatory changes, are found. 
 The real relation between the cutaneous affections and the nerve alterations 
 is entirely obscure. 
 
 Frequently, though by no means invariably, signs of a greater suspension 
 of function and anaesthesia occur during herpes zoster. Motor paralyses, like 
 
HERPES ZOSTER 
 
 187 
 
 facial and eye-muscle paralyses in herpes zoster trigemini, are symptoms that 
 rarely occur. 
 
 Many traits of which we mention only the frequent initial fever, the occur- 
 rence of epidemics, and the immunity which from the rareness of relapses 
 may be supposed to exist, all indicate that in numerous cases a peculiar 
 infectious process forms the foundation for this clinical picture. Among 
 the causes of herpes zoster, there are a number of factors which we have also 
 found in the aetiology of neuralgia, namely : other infectious diseases, affections 
 of the vertebrae, trauma, etc. 
 
 Fig. 42. Fig. 43. 
 
 Figs. 42 and 43. — Herpes zoster. Fig. 42, in the region of one of the middle thoracic nerves. 
 Fig. 43, in the region of the second thoracic nerve. On the shoulder at the right a birthmark, 
 (Leipsic Medical Clinic.) 
 
 Herpes zoster is closely related to carbon oxide and arsenical poisoning. 
 Occasionally herpes zoster manifests itself in connection with diseases of 
 internal organs, by appearing in a neighboring cutaneous zone, for instance 
 in inflammations of the costal pleura. The connection between the two has 
 not yet been explained. In many cases of herpes zoster, no definite aetiolog- 
 ical factors can be found. 
 
 The relation of herpes zoster to neuralgia can probably be summed up 
 thus: that they stand in a sort of co-ordinated position to each other. The 
 anatomic changes of the nerve which form the basis of the cutaneous affec- 
 tion, in many, but not all cases, give rise to neuralgia. It may be that the 
 
1 88 DISEASES OF THE PERIPHERAL NERVES 
 
 disorder occasionally is not accompanied by a cutaneous affection, and that 
 it becomes apparent only as a result of a neuralgia. 
 
 Zoster neuralgias form but a comparatively small percentage of all the 
 cases of neuralgia. 
 
 Neuralgia may precede herpes zoster for some time, may begin in 
 association with it, or may follow it. 
 
 No difficulty is, as a rule, experienced in distinguishing zoster from other 
 forms of herpes. In treating the eruption one may generally confine oneself 
 to a light protective bandage, some dusting powder, etc. 
 
 III. THE TUMORS OF THE PERIPHERAL NERVOUS SYSTEM 
 
 As we have seen, tumors seizing a nerve metastatically or by spreading 
 thence from a neighboring organ, may lead to its neuralgic affection or 
 paralysis. 
 
 Neuromata, the tumors emanating primarily from the peripheral nerve, 
 may occasion the same clinical picture. The special symptoms of these 
 tumors require a separate discussion. 
 
 It is relatively rare that neuromata emanate from the nervous tissue 
 proper. It is usually a matter of so-called spurious neuromata, tumors that 
 emanate from the connective tissue of the nerve and which histologically 
 appear as fibromata, lipomata, or even as angiomata. Some tumors may 
 degenerate and become malignant. 
 
 We shall consider first those forms appearing singly or at any rate 
 confined to a certain, small area of the body. They develop either on the larger 
 trunks or on the fine terminal ramifications, particularly in the subcutis. 
 The latter group includes the so-called plexiform (Ranken) neuromata, 
 which are almost exclusively of merely cosmetic surgical interest, and the 
 small, innoxious nodules, designated as tubercula dolorosa. These are 
 often very dense, sometimes angiomatously livid and painful. They appear 
 singly or in small groups and may be easily removed by an operation. 
 
 The neuromata of the trunk not infrequently lead to neuralgia and 
 paralysis. Intra-cranial neuromata of the cranial nerves, as they appear, 
 especially on the auditory and trigeminal, give the same clinical picture as 
 the basal brain tumors. 
 
 Neuromata on the intra-vertebral spinal cord roots produce the symptoms 
 of extra-medullary spinal tumors. 
 
 Besides this form of solitary neuromata there exists one of multiple 
 neuromatosis. The most frequent form, of the latter is the general one of the 
 skin and subcutis. Fibromata or lipomata, softer or harder nodules, are 
 generally found, sometimes in enormous numbers, distributed over the whole 
 surface of the body. The neurofibromatosis of the skin is designated also 
 as Recklinghausen's disease (after Recklinghausen 1882). In some cases, 
 
NEUROFIBROMATOSIS 189 
 
 however, the tumors of the large nerve trunks, of the cranial nerves and 
 spinal roots appear in large numbers. Occasionally numerous neuromata 
 are found in the skin besides the single or several centrally situated tumors of 
 this nature, and the determining of these may become very valuable for a 
 correct diagnosis of any central tumors appearing under the disease picture 
 of a spinal or brain tumor. At any rate, the most varied forms and local- 
 ization of neuromata may be found together in one and the same individual : 
 besides nodules on the nerve trunk, appearing in a wreath like formation, 
 root and skin neuromata, multiple tumors in the deeper part of the body, in 
 the muscles and the intestines, so that one may indeed speak of a general 
 neuromatosis. 
 
 The disease is evidently based upon a congenital predisposition. It 
 may appear even very early or, on the other hand, only late in life. It 
 occurs in whole families. The persons affected with it often show other 
 signs of a morbid disposition and are affected with deformities, numerous 
 naevi, pigment anomalies, etc., and show a neuropathic diathesis. 
 
 The clinical importance of the disease varies with the number, size and 
 location of the tumors. Especially the neurofibromatosis of the skin may 
 be almost devoid of troublesome features. In other cases they may become 
 very annoying through acute pains. Some neuromata situated compara- 
 tively indifferently, may become dangerous through excessive growth. The 
 danger of sarcomatous degeneration in multiple neuromatosis and in soli- 
 tary neuroma of the trunk is rather great. 
 
 Aside then from the entirely harmless, or nearly so, local affection of the 
 surface of the body, the prognosis is very uncertain. There are cases of a 
 mild nature, entirely lacking in any propensity to propagate, but there are 
 others also with a strong tendency to growth on the part of the existing tumors, 
 and a disposition to form more and more new ones. Strange to say, a spon- 
 taneous retrogression of single tumors has also been reported. 
 
 Surgical treatment should be used if seen to be absolutely necessary. 
 When advising patients one should always remember, however, that in such 
 cases as well as in all others, mechanical irritation may cause the trans- 
 formation of harmless tumors into malignant ones. 
 
Ill 
 
 DISEASES OF THE SPINAL CORD 
 
 i. NORMAL AND PATHOLOGICAL PHYSIOLOGY OF THE SPINAL 
 
 CORD 
 
 BY 
 
 MAX ROTHMANN (Berlin) 
 
 The spinal cord represents phylogenetically the oldest apparatus of the 
 central nervous system. In the lowest vertebrate, the amphioxus, it repre- 
 sents therefore, since the brain is quite rudimentary, a virtually independent 
 central apparatus, which shows normal functions, even after the removal of 
 the head-end. In the ascending scale of vertebrates, the dependence of 
 the spinal cord on the various higher centers of the brain increases continu- 
 ously, and special investigation is needed in the highest mammals, to still 
 find individual independent functions of the spinal cord. It is therefore 
 readily understood, that the spinal cord in building up its own centers, and 
 its connections with the brain, furnishes us means to recognize anatomical 
 and physiological arrangements peculiar to itself in each and every species 
 of animal. Comparative anatomy as well as physiology, therefore, may 
 transfer only with the greatest caution, the facts gleaned from one species of 
 animal to another however nearly related. This should be especially 
 considered in making use of the result of animal investigation for the pur- 
 poses of human pathology. 
 
 The spinal cord lies in the vertebral canal. At the upper end, it is 
 rather arbitrarily bounded by the exit of the first cervical nerve and the lower 
 boundary of the crossing of the most important cerebro-spinal tract, the 
 pyramidal crossing. At the lower end, in adult man, it is far from reaching 
 the bottom of the vertebral canal, but as a rule reaches only the lower part of 
 the first lumbar vertebra. In lower mammals and in the early foetal life of 
 man, on the other hand, it occupies the entire cavity of the lumbar and sacral 
 canal. While the spinal cord in its development thus remains behind the 
 growth of the vertebral column, it is replaced in the lumbar and sacral 
 vertebrae by a delicate thread, the filum terminate. 
 
 By this absence of the spinal cord in the lower part of the vertebral canal 
 of man, lumbar puncture invented by Quincke, at the level of the lower lumbar 
 vertebrae, has been made possible without danger of lesion to the spinal cord. 
 
 The spinal cord is enclosed by the spinal membranes. On the outside 
 lies the dura mater, a comparatively wide sac, which at the lower end fuses 
 
 190 
 
ANATOMY OF THE SPINAL CORD 
 
 191 
 
 with the filum terminale. On the inside of the dura lies the arachnoid 
 
 which, as a fine network, fills the space between the dura and the pia, and 
 
 contains the cerebro-spinal fluid, and the pia mater, lying directly upon the 
 
 surface of the spinal cord and sending branches into its fissures. The spinal 
 
 cord is usually divided into cervical, dorsal, 
 
 lumbar and sacral portions with the end 
 
 in the conus terminalis; this latter, by 
 
 gradual attenuation, goes over into the 
 
 filum terminale. To this corresponds 
 
 the division of the spinal cord roots into 
 
 8 cervical roots, the uppermost of which 
 
 emerges from the vertebral canal between 
 
 the occipital bone and the atlas, whereas 
 
 the lowest emerges between the 7 cervical 
 
 and the first dorsal vertebrae, into 12 dorsal, 
 
 5 lumbar and 5 sacral roots, the lowest 
 
 of which leaves the vertebral canal between 
 
 the os sacrum and the os coccygis, and 
 
 the coccygeal nerve. 
 
 The above mentioned gradual short- 
 ening of the spinal cord in relation to 
 the vertebral canal, leads to a displace- 
 ment of the spinal cord segments upwards, 
 so that the individual spinal cord roots, 
 in order to reach the foramina of exit des- 
 tined for them between the vertebrae, have 
 to course some distance downwards. The 
 length of the lumbar and especially of the 
 sacral roots, is considerable; the downward 
 coursing lumbar and sacral roots form, 
 around and below the conus terminalis, 
 the cauda equina. The relation between 
 the spinal origin of the individual roots 
 and their places of exit from the inter- 
 vertebral foramina, which is of the 
 greatest importance for spinal cord locali- 
 
 r r ^ Fig. 44. — Scheme of location of the 
 
 zation, appears clearly in Reid's diagram individual spinal-cord segments in the 
 /-,-,. N T ,, j , ,. j. ,, vertebral canal. (After Reid.) 
 
 (.big. 44). In the dorsal portion of the 
 
 cord, the entry of the root into the cord lies about 2 to 3 vertebrae above 
 the corresponding intervertebral foramen of the vertebral canal, but in the 
 lumbar and sacral roots this difference increases with extraordinary rapid- 
 ity; so that, for instance, the sacral roots all have their spinal origin at the 
 height of the twelfth dorsal and first lumbar vertebrae. 
 
192 DISEASES OF THE SPINAL CORD 
 
 In the spinal cord, there are two rather pronounced enlargements, corre- 
 sponding to the origin of the roots of the extremities, the cervical enlargement 
 from about the third cervical to the first dorsal vertebra and the lumhar 
 enlargement from about the tenth to the twelfth dorsal vertebrae. Both 
 appear in fcetal life synchronously with the development of the extremities. 
 
 In the spinal cord the gray substance is everywhere surrounded by white 
 substance. Only the tips of the posterior horns touch the periphery at the 
 place of entry of the posterior roots. The changing formation of the white 
 and the gray substance decides the different forms seen in transverse section 
 of the spinal cord in the various segments. Immediately below the pyramidal 
 crossing, the gray substance, by development of the anterior and posterior 
 horns, assumes the characteristic butterfly figure. The anterior horns, 
 the seat of the great motor cells, are, naturally, developed especially well in 
 the cervical and lumbar enlargements. But the development of the gray 
 substance in the entire lumbo-sacral portion of the cord, absolutely as well 
 as in relation to the quickly diminishing white substance is a particularly 
 strong one. In the dorsal portion of the cord a process, which may also be 
 observed between the anterior and posterior horn at other points of the cord, 
 the reticular process is developed into a lateral horn. The posterior horn 
 shows considerable thickening where it passes into the medulla oblongata, 
 as well as in the lower segments of the sacral portion of the cord. At its 
 base lies Clarke's column, developed especially well in the dorsal portion of 
 the cord; the head of the posterior horn follows, then the peculiarly trans- 
 parent gelatinous substance of Rolando, the marginal zone and the apex 
 of the posterior horn, which is to be regarded as belonging to the glial 
 covering of the cord. Both symmetrical halves of the gray substance are 
 bound together by an anterior and a posterior gray commissure, which 
 embrace, both in the front and the rear, the central canal with its surrounding 
 central gelatinous substance. In front of the anterior gray commissure lies a 
 broad bridge of white substance, the anterior white commissure; but number- 
 less medullated fibres pass also through the gray commissure. 
 
 The white substance of the spinal cord is divided into posterior, lateral, 
 and anterior columns. Whereas the separation between the posterior and 
 lateral columns is rather clearly indicated by the apex of the posterior horn 
 and the entering bundles of the posterior roots, no definite anatomic bound- 
 ary can be established between the lateral and anterior columns; the ventral 
 border of the roots which is, however, frequently interrupted may be con- 
 sidered as the boundary. Between the two anterior columns lies the deep 
 anterior fissure, which reaches the anterior white commissure that connects 
 the anterior columns. Between the two posterior columns is the septum 
 posterius, usually not quite opened into a fissure; it reaches the posterior 
 commissure. Between the septum posterius and the dorsal line of roots, 
 which deepens into the sulcus lateralis posterior, there is mostly a sulcus 
 
ANATOMY OF THE SPINAL CORD 193 
 
 intermedius posterior, which permits us to divide the posterior column into 
 a lateral part, the column of Burdach, and a medial part, the column of Goll. 
 
 The spinal cord roots are divided into anterior and posterior. The 
 anterior roots which spring from the anterior horn cells of the spinal cord, 
 pass, separated into several fibres, through the antero-lateral column to 
 the periphery, where they unite into a compact bundle. The posterior 
 roots spring from the spinal ganglia, which are situated in the intervertebral 
 foramina, outside of the dural sac. Only the lowest spinal ganglia, and, 
 exceptionally, those of the uppermost cervical roots, lie intradurally in the 
 vertebral canal. Hence the posterior roots pass to the sulcus lateralis 
 posterior, in order to penetrate the so-called entry zones of the roots of the 
 posterior column. While the anterior roots are thus placed ventrally to the 
 spinal ganglia, the anterior and posterior roots unite laterally from the gang- 
 lion into a bundle, the peripheral nerve. 
 
 Concerning the distribution of the blood in the spinal cord, every vertebral 
 artery, before union with the basilar artery, sends a branch backwards, and, 
 higher up, one to the front. Both anterior arteries, A. vertebrospinales 
 anteriores, unite, running downwards, in the top part of the spinal cord, 
 into an azygos artery, which, in the cervical enlargement, terminates in the 
 tractus arteriosus spinalis anterior. The latter is formed of an anastomotic 
 chain of the anterior short branches of the intercostal, lumbral and sacral 
 arteries, down to the filum terminale. From this, the central arteries pass 
 through the anterior fissure to the anterior commissure and send each a 
 branch into the gray substance of either side; only the peripheral divisions 
 of the latter are also supplied with blood from other sources. Furthermore, 
 from the tractus arteriosus spinalis anterior, lateral branches reach the 
 anterior roots, the anterior and antero-lateral column region. 
 
 The posterior vertebro-spinal artery unites with the posterior anasto- 
 motic chain of the intercostal, lumbar and sacral arteries to form the trac- 
 tus arteriosus posterolateralis, one on each side. From its medially passing 
 rami penetrantes, a second tractus arteriosus posterior is formed medially 
 from the posterior roots. From all these vessels, some arteries pass into 
 the white substance, the vasocorona, and supply this as well as the per- 
 ipheral parts of the gray substance. All arteries entering the spinal cord 
 are terminal arteries. 
 
 The veins of the spinal cord, upon whose course we can not enter here 
 with greater minuteness, are more developed than the arteries; they are all 
 characterized by the absence of valves. Though we know very little 
 of the course of the lymphatic vessels, their product, the cerebrospinal 
 fluid, has, in recent years, attracted attention in a constantly increasing 
 degree. This clear fluid of about 1003 specific gravity fills the subarach- 
 noid space as well as the cavities of the central nervous system; it shows a 
 slightly alkaline reaction, contains small quantities of globulin, and about 
 13 
 
i 9 4 DISEASES OF THE SPINAL CORD 
 
 i % of solid substance. Quincke's lumbar puncture, the cocainizing of the 
 spinal cord, introduced by Bier, and in most recent times, the serodiagnostic 
 syphilis reaction of Wassermann-Neisser-Bruck have more and more clearly 
 shown the importance of this fluid, which continually moistens the central 
 nervous system. 
 
 So far as the microscopically recognizable structure of the spinal cord is 
 concerned, it consists, like that of the entire central nervous system, of 
 ganglion cells, nerve fibres and neuroglia. Among the ganglion cells the large 
 cells of the anterior horn (anterior root cells) appear as the type of the motor 
 ganglion cell. They are multipolar and have an axis cylinder process, 
 and possess a centrally situated nucleus with nucleoli. The axis cylinder 
 process becomes continuous with a medullated nerve fibre, which passes 
 ventrad into the anterior column. In the cell protoplasm are found NissVs 
 granules (tigroid bodies), among which an achromatic substance is found, 
 the main components of which are the neurofibrils; these traverse the body 
 of the cell, and appear in all processes, even in the axis cylinder. The 
 neurofibrils probably represent that part of the body of the cell, which con- 
 ducts the stimulation, though nothing has been definitely ascertained as to 
 the relations of the ganglion cells to the extracellular fibril structures. One 
 thing, however, seems to be proved by the most recent investigations into the 
 coursing of the fibril, namely, that the theory of the neuron — that is, the entity 
 of the ganglion cell with its axis cylinder, which is said to have no connection 
 with other neurons except by contact, a theory which has been formulated 
 under the influence of Golgi's methods of metallic impregnation of the cell — 
 is not tenable from the purely anatomic point of view. On the other hand, we 
 must consider the neuron theory for the present as especially valuable for 
 physiological and clinical investigation, and adhere to it because of its great 
 importance for further researches. 
 
 In the ganglion cells, especially of the anterior horns, there is found also a 
 peculiar yellow pigment, which in adults, as a rule, is found collected in clusters 
 on one side of the protoplasm. This pigment, described as lipochrome 
 because of its behavior to osmium and the fatty extractive substances, is 
 entirely lacking in the newly born. It appears in the child at the age of from 
 6 to 8, and increases in quantity with advancing years, until in old people of 
 from 8o to 90 it often occupies almost the entire protoplasm of the cell. 
 Since this phenomenon appears with increasing years also in animals (dogs, 
 horses, monkeys) it might be regarded as a product of age, which inhibits 
 the activity of the ganglion cells. 
 
 The anterior horn cells are found at the various levels of the spinal cord, 
 in varying numbers, sizes and arrangements, but as a general rule, four 
 groups of ganglion cells may be distinguished by cross-section, a ventro- 
 medial and a dorsomedial, a ventrolateral and a dorsolateral. The latter 
 two are very strongly developed, especially in the cervical and lumbo-sacral 
 
ANATOMY OF THE SPINAL CORD 195 
 
 portions of the cord. Of other groups of cells, those in the lateral horn and 
 in the intermediate zone between the anterior and the posterior horns are to 
 be mentioned — the sympathetic nuclei. According to the most recent 
 investigations, there are three such nuclear columns, the nucleus sympathicus 
 lateralis superior from the eighth cervical to the third lumbar segment, the 
 nucleus sympathicus lateralis inferior from the second sacral segment to the 
 coccygeal portion of the canal and the nucleus sympathicus medialis inferior, 
 from the fourth lumbar segment to the coccygeal portion of the canal. 
 
 Besides these, Clarke's columns stand out preeminently. This group of 
 cells, situated at the base of the posterior horn, dorsolateral from the central 
 canal, is most strongly developed in the dorsal and lower cervical portions 
 of the cord, but is well marked also in all the other portions of the cord. 
 Clarke's columns are characterized by rather large ganglion cells with large 
 nuclei and nucleoli, relatively few and coarse-grained tigroid bodies, and 
 strikingly numerous protoplasmic processes. We cannot here enter into 
 the structure of the rest of the ganglion cells, which in great variety are 
 distributed in the spinal cord. 
 
 The gray matter, as it appears especially in Weigert's hasmotoxylin 
 preparations, is rilled by great masses of coarse and fine medullary sheath 
 nerve fibres. Among them we find a relatively small number of fibres 
 running from above downwards; they run for but a short distance. With 
 the exception of the accessorius roots in the upper portion of the cervical 
 segment, which run through the lateral column, only the anterior root fibres 
 leave the spinal cord from the gray matter. Besides these, there arise in 
 the gray matter of the spinal cord fibres which pass up and down; they 
 course in the lateral and anterior columns, and also, but fewer in number, 
 in the posterior column. Into the gray matter pass fibres from the various 
 columns of the white matter and fibres of the posterior roots; they radiate, 
 in part directly, and partly as collaterals of the posterior column fibres, 
 into the posterior horn and Clarke's column, but, a portion of them also may 
 be traced to the region of the anterior horn cells. Besides these there are 
 numberless commissural fibres, which, passing through the gray commis- 
 sures and especially also through the anterior white commissure, connect 
 the two halves of the gray matter; they also make possible a connection of 
 the white spinal cord tracts with the gray matter of the other half of the 
 spinal cord. 
 
 Finally relative to the neuroglia, which shares with the ganglion cells an 
 ectodermal origin, one must distinguish the ependymal cells, lining the 
 cavities of the central nervous system and therefore also the central canal, 
 and the neuroglia cells distributed throughout the gray and white matter, 
 which, however, seem to have the same embryonic origin. Intermingled 
 with the neuroglia cells, which we must be careful not to confuse with gang- 
 lion cells, especially in pathological relations, there exists an extraordinary 
 
196 DISEASES OF THE SPINAL CORD 
 
 fine meshwork of glia fibres. These glia fibres, however, are not to be 
 regarded as independent formations, but as processes of the glia cells. In 
 every loss of the actual nerve substance in the spinal cord, there is a pro- 
 liferation of the glious elements which leads to the formation of sclerosis. 
 
 If we now consider the white matter of the spinal cord, it consists, besides 
 the supporting substances, exclusively of nerve fibres, which serve to carry 
 intraspinal and cerebro-spinal impulses. The separate bundles of nerve 
 fibres bound together for the conduction of special functions, are therefore 
 called conducting paths. Since observation of the normal sections of the 
 spinal cord could give but little definite knowledge as to the course of such 
 tracts, the secondary degenerations, which appear in consequence of an inter- 
 ruption of certain tracts in focal affections of the central nervous system, 
 were early used to investigate this matter. After a medullated nerve fibre 
 is completely divided, there appears in the peripheral part, separated from 
 the parent cell, swelling, degeneration and finally absorption of the medullary 
 sheath. The fresh degeneration of medullary sheaths may be proved with 
 great accuracy by means of Marchi's method, in which osmic acid stains 
 newly degenerating medulla black; the absence of medullary sheaths in 
 older processes can be readily demonstrated by Weigert's medullary sheath 
 method. Only in later stages of the processes, and under peculiar circum- 
 stances, is there, besides this cellulifugal degeneration of the peripheral 
 nerve stump, also a retrograde degeneration of the central nerve stump in 
 addition to characteristic changes in the nerve cells themselves. 
 
 Another successful means of acquiring knowledge as to the individual 
 conducting tracts of the spinal cord, is Flechsig's method for the study of the 
 development of the medullary sheath, which is based upon the disparity in 
 time of the medullary development of the individual tracts. Then comes 
 Gudden , s method of developmental inhibition, which rests upon the fact that 
 after destruction of a center or a conducting path in a newly born animal, 
 i. e., in incomplete development, all the centers and tracts that belong to the 
 same system fail of complete development. 
 
 Finally the experimental and physiological methods of irritation and 
 destruction of certain centers or conducting tracts in the grown animal are 
 important for the establishment of the conducting tracts. 
 
 Of the conducting tracts of the spinal cord, the longest known is the 
 pyramidal tract (cortico-spinal tract). It has its origin in the region of the 
 central convolution of the cortex of the cerebrum, and, according to the most 
 recent investigations, predominantly in the anterior central convolution. 
 But the region of its origin must in any case extend over the area of the Betz 
 giant pyramid cells. After its known course through the internal capsule, 
 the basis of the cerebral peduncle, and the pons, it reaches as the pyramid, 
 the ventral surface of the medulla oblongata, and, where this makes its 
 transition into the spinal cord, enters the pyramidal crossing, in such a way 
 
THE PYRAMIDAL TRACTS 197 
 
 that the fibres from every pyramid pass backwards and cross one another at 
 an acute angle, in the form of bundles, like the fingers of folded hands, ven- 
 tral from the central canal. From the crossing the pyramidal fibres pass 
 
 Fig. 45, a. — Degeneration of both pyramidal tracts of the lateral columns in? the first cervical 
 segment, directly below the destroyed pyramidal crossing in the macacus rhesus. Descending 
 degeneration in the medial parts of the anterior columns. Marchi preparation. 
 
 Fig. 45, b. — The same degeneration in the seventh cervical segment. 
 
 to the base of the posterior horn, and then, with a sharp bend outwards, into 
 the lateral column. While this crossing in the dog and even in the lower 
 monkeys is complete, so that the pyramidal tract connects each hemisphere 
 of the cerebrum directly with the opposite lateral column, there is, in the 
 
198 
 
 DISEASES OF THE SPINAL CORD 
 
 anthropoid ape and especially in man, a considerable part of the pyramidal 
 tract, that takes no part in the crossing, but passes uncrossed downwards 
 along the anterior fissure into the anterior column. The relation of the 
 crossed part of the pyramidal tract (lateral pyramidal tract) to the uncrossed 
 (anterior pyramidal tract) is very changeable in man, so that cases in which 
 the anterior tract is almost lacking are found, as well as isolated cases, in 
 which the pyramidal crossing is utterly lacking and the entire pyramidal 
 tract reaches the anterior column uncrossed. Besides these two kinds of 
 fibres, there are a small number of pyramidal fibres, which, uncrossed, reach 
 the lateral column on the same side; these appear to be somewhat more 
 numerous in man than in monkeys and dogs, in which they may be occa- 
 sionally altogether absent (Fig. 45, a and b). 
 
 The great variability of the pyramidal tract in man coincides with the 
 ever changing situation and size of this tract in animals, and points to the 
 
 Fig. 46. — Pyramidal tract and rubrospinal bundle in the spinal cord of a dog. a, Cervical 
 enlargement; b, dorsal region. Py, pyramidal tract. R, rubro-spinal bundle. 
 
 comparative youthful age of this tract in the animal kingdom. Birds do 
 not have a pyramidal tract at all; it is only indicated in the upper part of 
 the cervical portion of the spinal column in the hedgehog, the sheep and 
 goat; in mice, rats and squirrels it does not pass into the lateral column, 
 but into the top of the posterior column, and we can recognize in passing 
 from the rabbit, by way of the cat and dog, to the monkey, a gradual growth 
 and increase of calibre in the fibres of the tract, which in that species is 
 situated exclusively in the lateral column. Only in anthropoids does a 
 clearly defined anterior pyramidal tract exist. 
 
 The development of the pyramidal tract is also relatively late; its axis 
 cylinders begin to appear in man in the sixth to seventh fcetal month. The 
 medullary envelopment does not take place until after birth. The lateral 
 pyramidal tract lies in the postero-lateral column; in the cervical portion 
 of the cord it is separated from the periphery by other masses of fibres; in 
 the lumbo-sacral portions, it is adjacent to the periphery. From it fine col- 
 
LATERAL PYRAMIDAL, RUBROSPINAL, DEITER'S TRACTS 199 
 
 Fig. 47. — Pyramidal tract and rubro-spinal 
 bundle in the ape. Third cervical segment. 
 
 laterals pass to the base of the anterior horns, though no one has, as yet 
 succeeded definitely in establishing the exact place where these fibres 
 terminate in the gray matter. At any rate, they do not seem to connect 
 directly with the cells of the anterior horn. The lateral pyramidal tract 
 may be traced downwards into the lowest part of the sacral portion of the 
 cord by staining the tissues by Marchi's degeneration method; it gradually 
 decreases in volume as it proceeds downwards. The anterior pyramidal 
 tract, as a rule, does not extend beyond the lowest part of the dorsal cord. 
 
 In about the same area as the lateral pyramidal tract, there runs a second 
 tract descending from the brain to the spinal cord, the rubrospinal tract 
 (Monakow's bundle) . Its main mass 
 springs from the red nucleus of the 
 corpora quadrigemina region, crosses 
 immediately after its origin in Forel's 
 tegmental crossing, and reaches, 
 after receiving some pontile fibres, 
 the postero-lateral column, in which 
 it passes down to the sacral portion 
 of the cord; it sends out compara- 
 tively large collaterals to the gray 
 matter at the base of the anterior 
 horns (Figs. 46 and 47). 
 
 This tract stands in inverse relation to the pyramidal tract; in the ab- 
 sence of the latter it is well developed, and as the other increases in the animal 
 kingdom it gradually diminishes. In the dog, the pyramidal tract is still 
 surpassed by it in size, surrounding it laterally and ventrally; in the monkey 
 it may be correctly designated as a prepyramidal column, since the bulk of 
 its fibres is being forced out ventrad by the strong development of the pyra- 
 midal tract. In man, it is only a rudimentary prepyramidally situated 
 little bundle of fibres. In about the same area there are also some fibres, 
 which descend from Deiter's nucleus to the spinal cord. 
 
 In addition to the anterior pyramidal tract, which is situated alongside 
 the anterior fissure, the anterior column also contains other important tracts 
 passing from the brain to the spinal cord. The largest of these has its 
 origin in Deiter's nucleus, a large group of cells lying in front of the cere- 
 bellum at the level of the acusticus-nuclei, and reaches, uncrossed in the main, 
 the anterior and antero-lateral columns of the spinal cord, whence, adja- 
 cent to the periphery, it passes to the lowest part of the sacral portion. 
 This vestibulospinal or Deiter's tract is connected by large collaterals with 
 the anterior horn of the same side. 
 
 A second important connection between the midbrain and the anterior 
 column is the corpora quadrigemina anterior tract; it arises from the gray 
 matter of the anterior quadrigeminal body, crosses completely in Meynert's 
 
200 
 
 DISEASES OF THE SPINAL CORD 
 
 tegmental crossing, reaches the anterior column of the spinal cord by 
 way of the posterior longitudinal bundle, where, by a wealth of collaterals, 
 it radiates into the anterior horn. This tract is paralleled by a pontile 
 anterior tract, rising in the gray matter of the pons; it likewise passes down- 
 wards in the peripheral divisions of the medio-anterior column (Fig. 48) . 
 
 In the most lateral portion of the anterior column, and above all in the 
 ventrad antero-lateral column of the upper cervical portion of the cord, the 
 bundles of fibres course, which descend from the respiratory centers in the 
 medulla oblongata to the phrenic centers in the fourth cervical segment, 
 but their course is not exactly known. 
 
 ^ 
 
 E3 
 
 EZ3 
 
 esa 
 
 POQMCDOSI 
 
 I 
 
 2 
 
 3 
 
 4 
 
 5 
 
 Fig. 48. — Descending cerebro-spinal anterior column tracts in the dog. 1, Tract from Deiter's 
 nucleus; 2, fibres from the pons to the anterior column on the same side; 3, fibres from the pons 
 to the anterior column of the opposite side; 4, tract from the quadrigeminal bodies to the anterior 
 column ; 5, fibres of the posterior longitudinal bundle. 
 
 In addition to all the tracts here described, which pass from the brain 
 into the spinal cord and degenerate descendingly after they are interrupted, 
 and which occupy, as a whole, peripheral divisions of the spinal cord, there 
 are in all parts of the anterior and lateral columns other intra-spinal connec- 
 tion fibres, which pass through the white matter, for shorter or longer dis- 
 tances, in order to connect higher with lower spinal segments. The shorter 
 of these pass on the very margin of the gray matter, while the longer ones 
 approach the periphery and partly commingle with the cerebro-spinal 
 fibre systems. 
 
 Now as to the ascending systems of fibres, passing from the spinal cord 
 to the brain; there is no such system, which analogous to the pyramidal tract, 
 ascends directly to the cerebral cortex. From the posterior roots, springing 
 
ASCENDING TRACTS OF THE SPINAL CORD 201 
 
 from the spinal ganglion cells, only one tract passes in the spinal cord, with- 
 out interruption, up to the medulla oblongata: the tract of the long posterior 
 column fibres. But only a small part of the posterior root fibres that pass 
 upwards reaches the posterior column nuclei of the medulla oblongata, 
 whereas a large part of them partly in the segment of the root entry, partly 
 in higher spinal segments, unites with the cells of the posterior horns, and 
 Clarke's columns. As the bundle of fibres, entering one segment of the 
 posterior column, places itself along the medial margin of the posterior horn 
 and is then pushed mediad by the posterior root fibres of the next higher 
 segment, there arises an imbricated structure of the long posterior root fibres 
 in the posterior column, in such a way that finally in the upper cervical por- 
 tion of the cord the posterior root fibres, coming from the posterior roots 
 of the lumbo-sacral portion and almost the entire dorsal portion, are placed 
 in the medial part of the posterior column, the so-called GolVs column, and 
 end in the medio-posterior column nucleus of the medulla oblongata, the 
 nucleus funiculi gracilis, while the laterally situated Bur (lack's column is 
 supplied by the posterior root fibres of the cervical and upper dorsal portions 
 of the cord, and ends in the nucleus funiculi cuneati of the medulla oblon- 
 gata (Fig. 49). None of the fibres of the posterior column goes higher than 
 this; only by the help of the lemniscus tract, rising in the posterior column 
 nuclei of the medulla oblongata and passing to the optic thalamus, and by 
 the thalamo-cortex connections is an indirect connection with the cortex of 
 the cerebrum established. 
 
 This posterior column portion of the posterior roots passing directly 
 to the posterior column nuclei of the medulla oblongata, increases gradually 
 n the ascending scale of animals, and is most developed in man. 
 
 In addition to the large ascending posterior column tract, every posterior 
 root sends off, at its entry into the posterior column a small descending 
 branch. It consists of a strand of fibres, which may be traced downwards a 
 few segments from the root entry; at the same time it gradually moves away 
 from the posterior horn, and attenuates very quickly. 
 
 Besides these exogenous or root fibres of the posterior column, there exist 
 also endogenous fibres, originating in the gray matter of the spinal cord. 
 Our knowledge of these we owe partly to experimental destruction of the 
 gray matter in the lumbar portion of the cord, as it may be produced by 
 ansemization in consequence of temporary occlusion of the aorta abdom- 
 inalis or by embolization of the terminal arteries of the gray matter; partly 
 also to the study of fresh cases of poliomyelitis, in which only the gray sub- 
 stance is affected in the more or less extended process of inflammation. In 
 such lesions of the gray substance of the lumbo-sacral portion of the cord, 
 one sees at the level of the lesion the ventral top of the posterior column, the 
 cornu- commissural zone, filled by moderate degeneration. Hence the field 
 of degeneration extends as a dorsal endogenous posterior column field, in 
 
202 
 
 DISEASES OF THE SPINAL CORD 
 
 an upward direction, dorsad along the posterior fissure, and reaches, with 
 a small part of its fibres mixed with the posterior root fibres of the sacral 
 portion of the cord, Goll's nucleus of the medulla oblongata (Fig. 50). 
 
 Descending degenerating posterior column fibres, described as endoge- 
 nous systems, have become known, more especially through pathological 
 observations. These form Schultze's comma shaped tract at the border of 
 Goll's and Burdach's columns in the cervical and upper part of the dorsal 
 portion, Flechsig's oval field at the posterior fissure in the lumbar portion 
 and the medio-triangtdar tract in the dorsomedial angle of the lower sacral 
 portion of the cord. These fields in their course from above downwards 
 seem to merge into one another, but whether they are purely endogenous 
 descending posterior column fibres, or are mixed with posterior root fibres, 
 has not been definitelv ascertained. 
 
 Fig. 49, a and b. — Degeneration of the posterior column after total'destruction of the fifth 
 lumbar root, a, Upper part of the fifth lumbar segment; b, twelfth dorsal segment. {After 
 Schaffer.) 
 
 Direct posterior root fibres end only in the posterior columns. The 
 connection of the posterior roots with the ascending systems of lateral and 
 anterior columns results exclusively through the mediation of the ganglion 
 cells of the gray matter. Most important here, are the spino-cerebellar 
 tracts of the lateral column, which are divided into a dorsal or Flechsig's 
 tract, and a ventral tract or Gowefs bundle. The dorsolateral cerebellar 
 tract springs from the ganglion cells of Clarke's column, in the dog from the 
 sacral portion on throughout the entire spinal cord, in man only beginning 
 with the lumbar enlargement; it lies laterally from the pyramidal tract and 
 the rubro-spinal bundle, along the periphery of the dorso-lateral column 
 and extends to the apex of the posterior horn. In its course upward, it 
 sends off numerous little fibres into the gray matter, at points but reaches 
 the medulla oblongata as a compact bundle, enters the inferior cerebellar 
 peduncle, and terminates, mostly crossed, in the superior vermis of the 
 cerebellum. 
 
 While this tract was first demonstrated by Flechsig on the basis of medul- 
 
THE VENTROLATERAL CEREBELLAR TRACT 
 
 203 
 
 lary sheath development, the ventrolateral cerebellar tract, Gower's bundle, 
 owes its discovery to the degeneration method. Its origin in the spinal cord 
 is not quite definitely known; but the ventral cells of the posterior horn of 
 both sides seem to send fibres to it. It lies in the spinal cord in the ventral 
 half of the lateral column along the periphery, dorsad directly touching the 
 dorsolateral cerebellar tract, but mediad pressing farther into the white 
 matter of the lateral column in the form of a triangle. This tract, also, con- 
 stantly receives fibres in its course through the spinal cord, and sends other 
 fibres off to the gray matter. The main bulk of these fibres diverge in 
 the medulla oblongata from Flechsig's tract where this tract enters the in- 
 ferior cerebellar peduncle; it describes a peculiar curve over the trigeminal 
 root at its exit and over the superior peduncle of the cerebellum, thus 
 reaching the dorsal division of the vermis in which it ends, partly crossed 
 and partly uncrossed. 
 
 Fig. 50. — General view of the ascending endogenous degeneration of the right posterior column 
 after cutting off the blood supply to the gray matter of the sacral and lumbar cord through 
 lycopodium embolism in the dog. 1, Sacral cord; 2-5, lumbar cord; 6-8, dorsal cord; 0, 
 cervical cord, a, Endogenous ventral region of the posterior column; b, endogenous dorsal 
 region of the posterior column; c, tractus septo-marginalis. 
 
 The termination of part of the fibres of Gower's tract in the corpora 
 quadrigemina region, without connection with the cerebellum, though 
 stated by many authors, is not established with certainty. 
 
 Besides these long ascending tracts, there course in the lateral columns 
 a great number of short fibres; they run upwards for a few segments, passing 
 along in the remnants of the lateral column near the gray matter. 
 
 In the anterior column also, throughout the entire spinal cord, long bun- 
 dles of fibres course upwards along the anterior fissure and the ventral per- 
 iphery, to be gradually lost in its uppermost divisions. They come, in the 
 
2o 4 DISEASES OF THE SPINAL CORD 
 
 main, from the gray matter of the other side, from which they reach the 
 anterior column through the anterior commissure. From the upper cervical 
 portion of the cord, however, one can trace fibres, which, arising in the 
 gray matter, reach the crossed anterior column by way of the anterior com- 
 missure, through the lateral division of the medial fillet and up to the optic 
 thalamus, so that here a second spino-thalamic connection is present besides 
 the above mentioned one of the long posterior tracts. Still other fibres 
 from the anterior column reach the region of Deiter's nucleus by way of the 
 reticular formation of the medulla oblongata. Besides these long paths, 
 there are in the anterior column a large number of short ascending fibres, 
 which after a few segments end again in the gray matter. 
 
 We can, therefore, group the conducting tracts of the spinal cord, as 
 follows : 
 
 I. Descending tracts. 
 
 (a) Lateral column. 
 
 i. Lateral pyramidal tract (tractus cortico-spinalis cruciatus). 
 
 2. Rubro-spinal tract (Monakow's bundle). 
 
 3. Fibres from Deiter's nucleus. 
 
 4. Respiratory tract in the antero-lateral column. 
 
 5. Intraspinal fibres. 
 
 (b) Anterior column. 
 
 1. Anterior pyramidal tract (tractus cortico-spinalis ant.). 
 
 2. Vestibulo-spinal tract. 
 
 3. Corpora quadrigemina anterior tract. 
 
 4. Anterior pontile tract. 
 
 5. Respiratory tract. 
 
 6. Intraspinal fibres. 
 
 (c) Posterior column. 
 
 1. Descending posterior root fibres. 
 
 2. Descending endogenous fibres (Schultze's comma, Flechsig's 
 
 oval field, medio-triangular tract). 
 
 II. Ascending paths. 
 
 (a) Lateral column. 
 
 1. Dorsolateral cerebellar tract (Flechsig) (tractus cerebello- 
 
 spinalis dorsalis). 
 
 2. Ventrolateral cerebellar tract (Gowers) (tractus cerebello- 
 
 spinalis ventralis) . 
 
 3. Intraspinal fibres. 
 
 (b) Anterior column. 
 
 1. Ascending anterior column tract (faisceau sulco-marginal 
 ascendant Marie). 
 
REFLEX ACTIVITY OF THE SPINAL CORD 205 
 
 2. Spinothalamic tract from the upper cervical portion of the 
 
 spinal cord. 
 
 3. Tract to the region of Deiter's nucleus. 
 
 4. Intraspinal fibres. 
 (c) Posterior column. 
 
 1. Ascending posterior column tract to the posterior column 
 
 nuclei of the medulla oblongata: GolPs and Burdach's 
 columns. 
 
 2. Posterior root fibres to the higher spinal cord segments. 
 
 3. Endogenous fibres (cornu-commissural zone and dorsal en- 
 
 dogenous field). 
 
 Function of the Spinal Cord 
 
 If we now turn to the function of the spinal cord, we must first of all keep 
 before our eyes the peculiar position of this central organ which, with the 
 development of the brain, has sacrificed to it, more and more, the peculiar 
 independence possessed by the cord in lower forms of animals and, as a 
 whole, has become an intermediate organ between the centripetal stimuli 
 coursing into the brain from the periphery of the body by way of the pos- 
 terior roots, and the impulses sent from the brain centers to the peripheral 
 organs. The former independent function of the spinal cord finds expres- 
 sion now only in reflex activity, and even this has in great measure come 
 under the dominion of the brain centers. Only after the two have been 
 completely separated, does a series of reflex phenomena appear in the 
 isolated spinal cord, which must be attributed to its "isolation changes." 
 
 For the production of a reflex, there is needed, besides the afferent and 
 efferent nerve paths, a connecting link, the reflex center. From the afferent 
 nerve, the reflex center and the efferent nerve the reflex arc is formed, the 
 functional intactness of which is necessary to the reflex action. For the 
 spinal cord the Magendie-BelVs law is of fundamental importance; accord- 
 ing to this only the anterior roots assume motor conductivity, whereas to 
 the posterior alone is assigned the sensory conductivity, a law, which in its 
 classic form, was first demonstrated in the frog by J oh. Mutter. Even if in 
 more recent times some observations have appeared, according to which, 
 in the frog, some vasodilatation impulses leave the spinal cord by posterior 
 roots, the general applicability of the law for mammals has not, as yet, 
 been shaken. 
 
 By this law, the direction in which the reflex processes have to take 
 place in the spinal cord, is firmly established. There are reflexes, which 
 have their entire reflex arc in one segment of the spinal cord, whereas in 
 other reflexes, various segments of the spinal cord, indeed even cerebral 
 centers are concerned. In the entire conception of the reflex process it is 
 
206 DISEASES OF THE SPINAL CORD 
 
 unquestionably a matter of importance, whether we must assume, according 
 to the neuron theory, a leap of the centripetal stimulus over to the motor 
 ganglion cells, or whether we must assume that the conduction of the stimu- 
 lus takes place through a fibrillary network without spacial interruption. 
 However that may be we, at any rate, will ascribe to the ganglion cells the 
 main role in these reflex transitions, and will not consider the extracellular 
 fibrillary net as the central place.. 
 
 The well-known experiment of Bethe on the black-clawed crab, in which 
 a separation of the afferent and efferent fibres of an antenna from the brain 
 ganglion did not remove the possibility of reflex stimulation of the feeler, 
 seems to speak for the possibility of the summation of the stimuli and the 
 tonus without ganglion cells. But a simple transference of these relations 
 to the reflex processes in vertebrates is not feasible. 
 
 For the production of reflexes, Pfliiger has laid down a series of conduc- 
 tion laws, which we reproduce in abbreviated form (according to Leyden 
 and Goldscheider) : 
 
 i. The Law of Equilateral Conduction for Unilateral Reflexes 
 
 Whenever muscular movements strictly confined to one-half of the 
 body follow as reflexes upon a stimulus, which affects a peripheral sensory 
 nerve, these movements without exception and under all circumstances, 
 are upon the same half of the body to which the stimulated sensory nerve 
 belongs. 
 
 2 . The Law of the Symmetry of Reflexes 
 
 If the change in the central organ, which is produced by a stimulated 
 sensory fibre, has already evoked unilateral reflexes and, in its further 
 spread, motors of the opposite half of the spinal cord are also excited, that is, 
 bilateral reflexes are produced, always and under all circumstances only 
 such motors are affected, as are already excited on the primarily affected 
 side, so that bilateral reflexes are never produced in the opposite direction. 
 
 3. The Unequal Development of the Reflexes on Both Sides of the Body in 
 
 Bilateral Reflexes 
 
 If the irritation of a sensory fibre induces reflexes in both halves of the 
 body, and they are more intensive and violent on one side, than upon the 
 other, the stronger are always upon that side, to which the stimulated 
 centripetal fibre belongs. 
 
 4. The Law of Inter sensitive-motor Movement and Reflex-irradiation 
 
 The direction from the sensory to the motor nerve in the central organ, 
 is turned in the brain, from before backward, in the spinal cord from below 
 upward, that is in both cases towards the medulla oblongata. 
 
THE PATELLAR AND ACHILLES TENDON REFLEXES 207 
 
 Even though these laws of Pflilger are on the whole valid, there are 
 nevertheless exceptions. There are found exclusively crossed reflexes; 
 even in the spinal cord reflexes may spread from above downwards, etc. 
 
 The movements executed by reflexes, have, on the whole, the character 
 of purposeful reactions. As a rule there is a co-operation of several muscles. 
 Many of these reflexes may be designated as protective reflexes, as, for in- 
 stance, the decapitated frog's withdrawal of his foot from diluted acid, but 
 even the reflexes that serve vegetative life, which regulate the activity of the 
 bowels, the bladder, the act of birth, are quite suitable to the purpose. 
 
 Of the greatest importance for the entire spinal cord pathology was the 
 discovery of the patellar reflex in 1875; this presents the most typical example 
 of the tendon reflexes. These tendon reflexes consist in the contraction of a 
 muscle, or of a group of muscles, on striking the tendon of the muscle. 
 Even though Westphal at first would have it that these reactions are only 
 an expression of the direct muscular irritation evoked by the shock, the 
 view of Erb, that a spinal reflex is concerned, is the opinion, which at present 
 is the generally accepted theory. In the patellar reflex the reflex arc is made 
 up of the sensory branches of the crural nerve, the quadriceps center in the 
 second to the fourth lumbar segment, the corresponding motor roots and 
 motor fibres of the crural nerve. An injury in the realm of any of these 
 divisions, and even in the quadriceps muscle itself, can abolish the patellar 
 reflex, even though the reflex arc is not completely destroyed. This is 
 proven by the return of the reflex in a cerebral disease which increases the 
 excitability of the anterior horn cells even though it has been lost for a 
 considerable period of time (hemiplegia in tabetics). In affections of the 
 cerebro-spinal descending tracts, especially of the pyramidal tract, exaggera- 
 tion of the patellar reflex to a high degree is always to be found. Even by 
 irritation of the reflex center itself, such as is evoked by strychnine and 
 tetanus poison, such a reflex exaggeration may be caused. In animals the 
 complete separation of the spinal cord from the higher centers leads after 
 a brief stage, during which the reflex is diminished, to a considerable in- 
 crease of it. On the other hand, a series of pathological observations in 
 man, in contrast to the animal experiments, seemed to prove a total loss of 
 the tendon reflexes after complete transverse section of the spinal cord. 
 But since in most recent times a series of trustworthy observations of the 
 retention or even increase of the tendon reflexes with a totally divided spinal 
 cord have been made, peculiar complications must be involved in those 
 cases, in which the reflex is absent. Probably, in these cases, abnormal 
 changes exist in the realm of the reflex arc itself. 
 
 Completely analogous to the patellar reflex is the Achilles tendon reflex, the 
 center of which is situated in the upper sacral portion of the cord, and the 
 triceps-reflex in the arm, the reflex center of which corresponds to the sixth 
 to seventh cervical segment. In injury to the cerebro-spinal motor conduct- 
 
208 DISEASES OF THE SPINAL CORD 
 
 ing tracts, there occurs not only an increase of the tendon reflexes, but 
 there appear immediately in the legs peculiar clonic phenomena, the ankle 
 clonus and the patellar clonus, which are induced by stimulation of the sen- 
 sory nerves of the tendons and muscles, the tendons being in a constant 
 state of tension. 
 
 In addition to the tendon reflexes, the skin reflexes are of great impor- 
 tance. In man the plantar, the abdominal and the cremaster reflex have 
 attained clinical importance above all others. The reflex arc of the skin 
 reflexes is not limited to the spinal cord, but reaches up to the brain, partly 
 even up to the cortex of the cerebrum. The tactile reflex of the toes in the 
 dog, which is elicited by touching the back of the foot, and is analogous to 
 the human skin reflexes, is permanently lost after the removal of the center 
 for the extremities in the cerebral cortex. In hemiplegia, the skin reflexes 
 on the side of the paralysis are, at least temporarily, abolished. 
 
 Study of the tactile reflex of the dog in cases of disjunction of cerebro- 
 spinal tracts, has, as a matter of fact, proven that the construction of the 
 reflex arc for the skin reflexes is very complicated. Not only the afferent 
 limb, but also the efferent limb of the tactile reflex possesses two different 
 conducting tracts (ascending posterior and anterior column tracts, descend- 
 ing pyramidal tract and rubro-spinal bundle), so that it can be made to dis- 
 appear only after the destruction of either both afferent or both efferent 
 tracts. 
 
 In most recent times, the Babinski toe reflex has finally proven, that at 
 least one of the skin reflexes possesses, under pathological conditions, besides 
 the cortical reflex arc, a subcortical one. The toes show under normal 
 circumstances a distinct plantar flexion upon irritation of the sole of the 
 foot; but in injuries of the brain or spinal cord, which, as a rule, lead to an 
 increase of the tendon reflexes and to spasticity, a dorsal flexion of the toes 
 appears. This phenomenon is the normal reflex process in children during 
 the first year of life, and is only supplanted by plantar flexion, probably 
 connected with the assumption of erect walking by man. Correspondingly, 
 in the lower apes, such plantar flexion of the toes is lacking under normal 
 conditions. 
 
 The plantar reflexes have their portals of entrance and exit in the first 
 two sacral roots. The cremaster reflex, which consists in raising the tes- 
 ticles by contraction of the cremaster muscle, when the inner surface of the 
 thigh is irritated, is carried by the first and second lumbar roots. The 
 abdominal reflexes, which are not always constant, are divided into an 
 upper, middle and lower; they are connected with the seventh to the 
 eleventh dorsal roots. 
 
 Besides these skin and tendon reflexes, the spinal centers, that are con- 
 nected with the sympathetic nervous system, govern a series of functions. 
 The fibres of the spinal cord that pass to the sympathetic, begin only in 
 
REFLEX CENTERS IN THE SPINAL CORD 
 
 209 
 
 Mid-brain. 
 
 Cervical swell- 
 ing. 
 
 Dorsal I to 
 lumbar II or 
 III. 
 
 definite divisions, that is, from the first dorsal, occasionally, too, from the 
 eighth cervical segment to the second or third lumbar segment, and from the 
 second to the fourth sacral segment. The sympathetic fibres pass out from 
 the spinal cord with the anterior roots, and reach the 
 sympathetic ganglia (Fig. 51). 
 
 At the level of the first dorsal segment lies the cilio- 
 spinal center, the irritation of which causes dilatation; its 
 loss, contraction of the pupil. It is subject to the influence 
 of a higher center in the medulla oblongata, so that a uni- 
 lateral total division of the cervical spinal cord induces like- 
 wise miosis of the pupil on the same side; this, however, is 
 transitory. 
 
 The sympathetic fibres from the dorsal and lumbar 
 portions of the cord are of marked importance for the in- 
 nervation of the vessels. Vasoconstrictors and vasodilators 
 for the vessels of the skin, muscles, intestines, seem to 
 be influenced by the spinal cord. Especially important 
 are the centers, which, in the upper sacral portion, in- 
 fluence the bladder, rectum, and the genital apparatus. 
 Experiments in recent years have proved, that even after 
 disjunction of the gray substance of the lumbo-sacral por- 
 tion, or after removal of the entire lower divisions of the 
 spinal cord, the evacuation of the bowels and bladder is 
 still regulated after a fashion, so that under pathological 
 conditions, centers independent of the spinal cord step into 
 active work; but it is certain, that the normally regulated 
 function of bladder and rectum is under the control of 
 the spinal cord; even where a total division of the cord oc- 
 curs at a high level, there appears again, after a time, in- 
 voluntary evacuation of the bladder, but at regular inter- 
 vals. The centers for the detrusor and sphincter of the 
 bladder and for the unstriped muscles of the rectum, lie in 
 the third to fifth sacral segment, for the external sphincter 
 ani in the fourth and fifth sacral segment, for the anal 
 reflex in the fifth sacral segment. 
 
 In the upper sacral portion of the cord, there are also 
 the centers for erection and ejaculation. But here too the yous system in man. 
 
 . ,. {After Langley.) 
 
 sympathetic ganglia, irrespective of the spinal-cord centers, 
 seem to possess a certain independence of their own. Far more extensive 
 is the same for the female genital apparatus; in animals with shortened 
 spinal cords, as well as in human beings, after destruction of the lowest 
 portion of the spinal cord, normal births have been observed. 
 
 The independent function of the spinal cord strikes us most clearly 
 14 
 
 Sacral II-IV. 
 
 Fig. 51. 
 autonomous 
 
 -The 
 ner- 
 
2IO 
 
 DISEASES OF THE SPINAL CORD 
 
 when it is separated from the higher centers. In the decapitated frog, one 
 can observe a great series of purposeful movements; thus, when one of its 
 
 Fig. 52, a. — Scheme of cutaneous sensibility corresponding to the distribution of the 
 posterior spinal roots. (After Seiffer.) 
 
 legs is held fast, it makes attempts to extricate it with the other, reacts with 
 perfect appropriateness to abnormal skin stimulations, etc. But even in 
 
REFLEX CENTERS IN THE SPINAL CORD 
 
 211 
 
 the dog, peculiar reflexes appear after isolation of the spinal cord, as theiwell- 
 srudied scratch reflex, the rhythmical pawing of the hind legs, when the 
 
 Fig. 52, b. — Scheme of cutaneous sensibility corresponding to the distribution of the 
 posterior spinal roots. (After Seiffer) 
 
 animal is lifted high up (Freusberg's phenomenon). Indeed, with forward 
 motions of the front part of the body, which is connected with the brain, 
 
2 i2 DISEASES OF THE SPINAL CORD 
 
 there may be a lifting and a certain running-like movement of the hind 
 legs, as the result of the reflex activity of the isolated spinal cord. In man, 
 of course, such independence of the isolated spinal cord no longer exists. 
 But here, too, there may be a series of abnormal reflexes and muscle 
 twitchings. 
 
 While in many invertebrates the entire body is divided into numerous 
 segments, to which a similarly divided central organ corresponds, this 
 division in vertebrates is demonstrable only during embryonal life. In 
 later life this segmentation is externally recognizable only in the vertebral 
 column and in the origin of the spinal roots. In spite of this, every spinal- 
 cord segment, characterized by the entrance and exit of definite roots, is in 
 relation with definite parts of the surface of the body and definite skeletal 
 muscles. Thus one may speak of a segment innervation of the skin, as every 
 posterior root supplies a connected skin region. Animal experiments 
 have taught us that the realms of the single posterior roots overlap, so that 
 in order to produce total anaesthesia in a portion of skin, not only the main 
 root but also the one above and below must always be destroyed. In addi- 
 tion to the schemes offered by these experimental investigations, schemes of 
 the radicular innervation of the skin have been outlined relatively exact by 
 the results of pathologic studies; first of all, by the phenomena of loss of 
 function observed in tumors and compressions of the spinal cord, as well 
 as by the peculiar trophic disturbances, corresponding to the sensory zones 
 in herpes zoster, a disease based upon inflammation of individual spinal 
 ganglia. In recent times they have come to be of the greatest practical im- 
 portance for the exact localization of the height at which spinal-cord tumors 
 exist and without which successful operative treatment is impossible. 
 
 The sensibility scheme (Fig. 52, a and b) of Seiffer reproduced here 
 shows as the most important boundaries : the parietal-ear-chin line, which 
 separates the trigeminus area from the cervical region; of next importance, 
 the neck-trunk boundary, anteriorly it is in the second intercostal space, 
 posteriorly it corresponds to the spines of the fifth to seventh cervical vertebras 
 where the zones of the fourth cervical segment and the second dorsal seg- 
 ment meet. After the fcetal outgrowth of the front extremities, the skin 
 regions corresponding with the fifth cervical to the second dorsal segments, 
 are distributed at the front and back of the arms. Of importance also are 
 the intermamillary line (fourth to fifth dorsal segment), the xiphoid line 
 (sixth to seventh dorsal segment) , and the navel line (tenth dorsal segment) . 
 The trunk-leg line, formed in front by Poupart's ligament, behind by the 
 first to second sacral vertebra, separates the twelfth dorsal and first lumbar 
 segment. The divisions from the second lumbar segment to the second 
 sacral segment serve for the skin innervation of the legs, while the lowest 
 sacral segments subserve the genital and anal spheres. Thereby it is note- 
 worthy that a lesion of the upper sacral portion may produce anaesthetic 
 
SEGMENTARY DISTRIBUTION OF THE SPINAL ROOTS 213 
 
 disturbances at the inner edge of the foot and in the genital area, while the 
 intermediate skin regions remain undisturbed. 
 
 But not only the skin innervation is dependent on the segmentary dis- 
 tribution of the roots, but in the anterior roots also there is a peculiar 
 segmentary arrangement of the innervation of the muscles of the body, which 
 swerves far from the distribution in the peripheral nerves and the muscles 
 themselves. Thus every anterior root takes part in the innervation of 
 several muscles, which may be supplied by different peripheral nerves, and 
 again a series of anterior roots may take part in the innervation of one 
 muscle. 
 
 The motor effects accomplished by the stimulation of anterior roots, 
 have become known to us, as a whole, by experiments on monkeys. Con- 
 cerning stimulation of anterior roots in man, we have only isolated information 
 at our disposal (see following scheme). 
 
 The Motor Effects of the Electric Stimulation of the Anterior Roots 
 in the Ape. {From Sherrington.) 
 
 Cervicalis. 
 
 I. Lateral flexion of the neck without rotation of the head. 
 II. Lateral flexion and retraction of the neck, very slight rotation 
 of the head. 
 
 III. Lateral flexion with rotation and retraction of the neck, so that 
 
 the chin is correspondingly turned towards the opposite side. 
 
 IV. Elevation and adduction of the shoulder. Retroflexion and 
 
 slight lateral flexion of the neck, by which the head, especially 
 when the shoulder is "fixed," is drawn to the contra-lateral 
 side. 
 V. Elevation, abduction and slight outward rotation of the shoulder. 
 Flexion of the elbow, at the same time slight supination and 
 radial flexion of the hand. Only slight lateral and retro- 
 flexion of the neck. 
 VI. Moderate adduction of the shoulder. Strong flexion of the 
 elbow. Slight extension of the fingers and the hand, but in 
 some individuals flexion. Some supination. Neck and 
 head as in V. 
 VII. Retraction and pronounced adduction of the shoulder and 
 inward rotation of the upper arm. Extension of the lower 
 arm. Slight flexion and pronation of the hand. 
 Slight flexion of the fingers. The shoulder is drawn down. 
 Neck as in V. 
 VIII. The shoulder is drawn down (latissimus dorsi). The adduction 
 of the shoulder is not so strong as in VII. Inward rotation 
 
2i 4 DISEASES OF THE SPINAL CORD 
 
 of the arms. Flexion and pronation of the hand. Flexion 
 of the fingers and the thumb with apposition of the latter. 
 
 Thoracica. 
 
 I. Retraction of the shoulder. Slight lateral and retro-flexion of 
 the neck. Slight extension of the arm. Flexion and prona- 
 tion of the hand. Flexion of the fingers and the thumb with 
 opposition of the latter. Usually, also, slight ulnar flexion 
 of the hand. 
 II. Retraction of the shoulder. Slight flexion of the hand. Flexion of 
 the fingers and the thumb with opposition of the latter. In 
 some cases, slight pronation of the hand. Lateral flexion of 
 the vertebral column. 
 
 Postthoracica (lumbalis) . 
 
 I. Drawing in of the abdomen. 
 II. The same with slight flexion of the hip. 
 
 III. Drawing in of the lower part of the abdomen. Flexion of the hip. 
 TV. Drawing in of the lower part of the abdomen. Flexion and 
 
 adduction of the hip. Extension of the knee. 
 V. Adduction of the hip. Extension of the knee. Slight flexion 
 
 of the foot; slight extension of the great toe. 
 VI. Extension of the hip. Adduction of the thigh. Strong flexion 
 
 of the knee. Dorsal flexion of the foot. Extension of the 
 
 toes. Adduction of the hallux. 
 VII. Extension of the hip. Flexion in the knee. Extension of the 
 
 foot. Rotation of the sole. Strong flexion and adduction 
 
 of the hallux. Downward movement of the tail. 
 VIII. Lateral movement of the tail. Slight outward rotation of the 
 
 hip and flexion in the knee, with extension in the hip and foot. 
 
 Strong flexion of the toes with flexion and adduction of the 
 
 hallux. 
 TX. Lateral movement of the tail. Sometimes, slight outward rota- 
 tion of the thigh and adduction with flexion of the toes. 
 X. Lateral movement of the tail (towards the side of stimulation) . 
 
 These discoveries prove so much, that the assumption of a definite func- 
 tional relation of the motor fibres united in one anterior root, is not justified. 
 Furthermore, it is noteworthy that even for paralysis of one muscle, at least 
 three successive anterior roots must as a rule be destroyed. In man, fre- 
 quently, a still farther reaching anastomosis of the individual roots is as- 
 sumed, so that beside the middle root, two upper and two lower roots take 
 part in the innervation of a muscle. 
 
FUNCTIONS OF SEGMENTS OF THE SPINAL CORD 215 
 
 Observers have been largely occupied, in ascertaining the localization 
 of the various muscles in the individual spinal-cord segments, from the 
 results of localized spinal-cord lesions. From the wealth of schemes put 
 together for this purpose, we give the one recently published by Bruns, as it 
 takes into consideration in addition skin innervation and the reflexes. 
 
 LOCALIZATION OF THE FUNCTIONS OF THE VARIOUS SEGMENTS OF THE 
 SPINAL CORD. (From Bruns) 
 
 Segments 
 
 1. Cervicalis. 
 
 2. Cervicalis. 
 
 Muscles 
 
 Sensory innervation of the 
 skin 
 
 Musculus rectus capitis 
 
 posterior minor. 1 
 M. rectus capitis posterior 
 
 major. 
 M. obliquus cap. superior. 
 M. semispinalis capitis. 
 M. spinalis cap. (pars 
 
 cranialis). 
 M. rectus cap. anterior. 
 M. longus capitis. 
 M. rectus capitis lateralis. 
 M. geniohyoideus. 
 M. omohyoideus. 
 M. sternohyoideus. 
 M. thyreo-hyoideus. 
 M. sternothyreoideus. 
 One branch to the muscu- 
 
 1 u s intertransversarius 
 
 posterior cervicalis. 
 
 Reflexes 
 
 M. rectus cap. post, ma- 
 jor. 
 
 M. obliquus cap. inferior. 
 
 M. semispinalis capitis. 
 
 M. spinalis capitis (pars 
 cranialis). 
 
 M. longus atlantis. 
 
 M. longus colli. 
 
 M. longus capitis. 
 
 M. geniohyoideus. 
 
 M. omohyoideus. 
 
 M. sternohyoideus. 
 
 M. thyreohyoideus. 
 M. sternothyreoideus. 
 
 1 Cervicales, 1 to 4, from Risien Russel. 
 
2l6 
 
 DISEASES OF THE SPINAL CORD 
 
 Segments 
 
 Muscles 
 
 Sensory innervation of the 
 skin 
 
 Reflexes 
 
 3. Cervicalis. 
 
 4. Cervicalis. 
 
 M. splenius capitis et cer- 
 
 vicis. 
 M. sternocleidomas- 
 
 toideus. 1 
 M. trapezius. 
 Serrations of the musculus 
 
 longissimus and the in- 
 
 tertransversarii. 
 
 Platysma (Kocher). 
 
 M. longus atlantis. 
 
 M. longus colli. 
 
 M. longus capitis. 
 
 M. diaphragma. 
 
 M. scalenus medius. 
 
 M. geniohyoideus. 
 
 M. omohyoideus. 
 
 M. sternohyoideus. 
 
 M. sternothyreoideus. 
 
 M. splenius capitis et cer- 
 
 vicis. 
 M. sternocleidomas- 
 
 toideus. 
 M. trapezius. 
 M. levator scapulae. 
 
 M. longus atlantis. 
 M. longus colli. 
 M. longus capitis. 
 M. Diaphragma. 
 M. scalenus anterior. 
 M. scalenus medius. 
 M. sternothyreoideus. 
 M. splenius capitis et 
 
 cervicis. 
 M. trapezius. 
 M. levator scapulae. 
 M. rhomboidei. 
 M. supra- and infra-spi- 
 
 natus. 
 M. deltoideus. 
 M. biceps and coracobra- 
 
 chialis. 
 M. brachialis int. 
 M. supinator longus. 
 
 Skin on the back part of the 
 head to the vertex; on the 
 sides of the head forwards to 
 the anterior boundary of the 
 ear; skin over the ear; over 
 the lowest and most poste- 
 rior parts of the lower jaw, 
 on the neck and nape, at 
 the front downwards to the 
 second rib; at the back 
 down to the spina scapulae 
 (according to Wichmann, 
 the middle lower part of 
 this dorsal region is sup- 
 plied by the dorsal branches 
 of the fifth and sixth and 
 even seventh cervical 
 nerves). The second cer- 
 vical root reaches up- 
 wards directly to the tri- 
 geminus region; the third 
 innervates the neck and 
 nape down to the clavicle; 
 the fourth from there to the 
 second rib. 
 
 1 Through accessorii. 
 
FUNCTIONS OF SEGMENTS OF THE SPINAL CORD 217 
 
 Segments 
 
 Muscles 
 
 Sensory innervation of the 
 skin 
 
 Reflexes 
 
 5. Cervicalis. Museums levator scapula;. : Outer side of shoulder, arm 
 
 6. Cervicalis. 
 
 M. teres minor. 
 
 M. diaphragma. 
 
 M. rhomboidei. 
 
 M. deltoideus. 
 
 M. biceps and coracobra- 
 chialis. 
 
 M. brachialis internus. 
 
 M. supra- and infra-spi- 
 natus. 
 
 M. supinator longus and 
 brevis. 
 
 M. pectoralis (pars cla- 
 vicularis) . Possibly exten- 
 sors of hand. 
 
 M. serratus anticus major. 
 
 M. splenius. 
 
 and forearm. 
 
 Scapular reflex. 
 
 From the fifth cer- 
 vical to the first 
 dorsal root, tendon 
 reflexes of the mus- 
 cles represented in 
 them. 
 
 Outer parts of the forearm 
 on the flexor and extensor 
 surfaces. 
 
 M. deltoideus. 
 
 M. teres major and minor. 
 
 M. biceps and brachialis 
 
 internus. 
 M. supra- and infra-spi- 
 
 natus. 
 M. supinator longus and 
 
 brevis. 
 M. subscapularis. 
 M. pronator quadratus Radial portion of the hand 
 
 and teres. and the fingers. 
 
 Extensors and flexors of the 
 
 hand. 
 
 Serratus anticus major. 
 Possibly long extensors of 
 
 the fingers. 
 M. splenius. 
 M. scaleni. 
 
 7. Cervicalis. Pronators of the hand. Middle parts of the forearm 
 
 M. subscapularis. 
 
 M. teres major. 
 Extensors and flexors of 
 
 the hand. 
 M. triceps. 
 Pectoralis major (pars cos- 
 
 talis). 
 Latissimus dorsi. 
 
 on the flexor and extensor 
 surfaces. 
 Radial and median district 
 of the hand and the fingers. 
 
 Palmar reflex. 
 
 Blow upon the pal- 
 mar surface causes 
 the fingers to close. 
 
 1 Besides this, the individual cervical roots innervate also the long muscles, extending over 
 the entire vertebral column, at the corresponding level. 
 
2l8 
 
 DISEASES OF THE SPINAL CORD 
 
 Segments 
 
 Muscles 
 
 Sensory innervation of the 
 skin 
 
 Reflexes 
 
 
 Long extensors and flexors 
 
 
 
 
 of the fingers. 
 
 
 
 
 M. splenius. 
 
 
 
 
 M. scaleni. 
 
 
 
 
 Small muscles of the hand 
 
 
 
 
 (interossei and lumbri- 
 
 
 
 
 cales) ? 
 
 
 
 8. Cervicalis. 
 
 M. triceps. 
 
 
 
 Flexor carpi ulnaris. 
 
 
 
 
 Long extensors and flexors 
 
 
 
 
 of the hand. 
 
 
 
 
 Interossei and lumbricales. 
 
 
 
 
 Scaleni. 
 
 Ulnar side of the hand, the 
 forearm and arm on the 
 extensor and flexor sur- 
 faces. The uppermost 
 part of the arm region is 
 supplied in addition by 
 dorsalis 2. 
 
 
 i. Dorsalis. 
 
 Long flexors of the fingers. 
 
 Interossei and lumbri- 
 cales. 
 
 Balls of the thumb and 
 small finger. 
 
 Scaleni. 
 
 
 
 (In the eighth cervical and 
 
 
 
 
 first dorsal root the fibres 
 
 
 
 
 for the dilatator pupillae 
 
 
 
 
 pass to the gangliated 
 
 
 
 
 cord.) 
 
 
 
 2 to 12 dorsalis, 
 
 The corresponding long 
 
 Skin of the chest, the back, 
 
 Epigastric reflex, 
 
 resp. i lumba- 
 
 muscles of the vertebral 
 
 and the abdomen, above 
 
 4th~7th dorsal root. 
 
 lis. 
 
 column. 
 
 from the lower boundary of 
 
 
 
 M. serratus post. sup. 1-4. 
 
 the upper cervical area, be- 
 
 
 
 M. serratus post. inf. 
 
 low reaching not quite to the 
 
 Abdominal reflex, 
 
 
 9-12. 
 
 inguinal fold; skin of the 
 
 7th-nth dorsal 
 
 
 M. intercostales interni 
 
 upper gluteal region. The 
 
 root. 
 
 
 2-1 1. 
 
 6th root reaches in front, 
 
 
 
 M. intercostales externi 
 
 medially, the region of the 
 
 
 
 2-11. 
 
 epigastrium; the 8th and oth 
 
 
 
 M. levat. cost. breves 
 
 do not reach beyond the 
 
 
 
 2-1 1. 
 
 skin over the abdominal 
 
 
 
 M. infracostales 2-4 and 
 
 cavity (nipple 4th dorsal 
 
 
 
 7-9- 
 
 root; upper epigastrium 6th, 
 
 
 
 M. levat. cost, longi 8-10. 
 
 umbilicus 10th). The 
 
 
 
 M. transversus thoracis 
 
 boundaries of the individ- 
 
 
 
 3-6- 
 
 ual skin regions pass hori- 
 
 
 
 M. transversus abdominis 
 
 zontally about the j trunk; 
 
 
 
 7-12. 
 
 therefore, at the thorax, 
 over several i ntercostal 
 spaces. 
 
 
FUNCTIONS OF SEGMENTS OF THE SPINAL CORD 
 
 219 
 
 Segments 
 
 Muscles 
 
 Sensory innervation of the 
 skin 
 
 Reflexes 
 
 
 M. obliq. abd. int. 7-12. 
 
 
 
 
 M. obliq. abd. ext. 7-12. 
 
 
 
 
 M. rectus abdominis 8-12. 
 
 
 
 
 M. pyramidalis d. 12 
 
 . 
 
 
 
 lumb. 1. 
 
 Skin of the inguinal region, 
 
 
 Lumbalis i. 1 
 
 Muscles of the abdomen, 
 
 Patellar reflex 2 nd- 
 
 
 see above. 
 
 of the uppermost part of the 
 
 4th lumbaris, espe- 
 
 
 Iliopsoas. 
 
 front and outer parts of the 
 
 cially lumb., 4. 
 
 
 Sartorius. 
 
 thigh, skin of the mons ven- 
 
 
 
 Cremaster. 
 
 eris, the root of the penis on 
 
 
 
 Quadratus lumborum. 
 
 the dorsal side, sensibility 
 of the testicles, seminal cord 
 and the tunica dartos, by 
 means of the sympathetic. 
 
 Outer and front part of the 
 
 
 Lumbalis 2. 1 
 
 Iliopsoas. 
 
 
 
 Sartorius. 
 
 hip, to the lower third of the 
 
 
 Lumbal plexus. 
 
 Quadriceps femoris. 
 Cremaster. 
 
 thigh. 
 
 
 
 Quadratus lumborum. 
 
 Anterior portion of the hip; 
 
 
 Lumbalis 3. 
 
 Small muscles of the pel- 
 
 
 
 vis. 
 
 partly in common with 2, 
 
 
 
 M. iliopsoas. 
 
 innermost parts of the back 
 
 
 
 M. sartorius. 
 
 of the hip in its upper parts, 
 
 
 
 M. quadriceps femoris. 
 
 and the inner side of the 
 
 
 
 M. adductores femoris. 
 
 leg. 
 
 
 
 M. quadratus lumborum. 
 
 Lower parts of the inner and 
 
 
 Lumbalis 4. 
 
 Small muscles of the pelvis. 
 
 Gluteal reflex 4th 
 
 
 M. quadriceps femoris. 
 
 anterior surfaces of the hip, 
 
 and 5th lumbalis 
 
 
 M. adductores femoris. 
 
 inner side of the leg, partly 
 also the back inner margin 
 of the foot, excepting the 
 large toe. 
 
 ( ? probably lower) . 
 
 
 Tibialis anticus. 
 
 
 
 
 (Extensor digitorum com- 
 
 
 
 
 mun., extensor hallucis.) 
 
 Anterior outer parts of the 
 
 
 Lumbalis 5. 
 
 Tibialis anticus. 
 
 
 
 Extensor hallucis. 
 
 leg, outer and dorsal parts 
 
 
 
 Extensor digitorum com- 
 
 of the foot, with exception of 
 
 
 
 munis. 
 
 the tips of the toes, inner 
 
 
 
 Musculi peronei. 
 
 parts of the plantar surface 
 of the foot (with sacralis ) . 
 
 
 1 Perhaps the 1st and 2nd lumbar segments contain no muscular nuclei at all; then the muscles 
 mentioned here would draw their nerve supply from the 3d segment. 
 
220 
 
 DISEASES OF THE SPINAL CORD 
 
 Segments 
 
 Muscles 
 
 Sensory innervation of the 
 skin 
 
 Reflexes 
 
 Sacral plexus. 
 
 Sacralis i and 2. 
 
 Sacral plexus. 
 Sacralis 3-5. 
 
 M. flexores cruris. 1 
 Musculi glutaei (extensores) 
 
 and abductors of the hip. 
 Outward rotators of the 
 
 hip. 
 Plantar flexors of the foot 
 
 (musculature of the calf) 
 
 and long flexors of the 
 
 toes. 
 
 M. peronei. 
 
 Muse, flexoris cruris. 
 
 Muscul. glutaei (extensors) 
 
 and abductors of hip. 
 Outward rotators of the 
 
 hip. 
 Plantar flexors of the foot 
 Long flexors of the toes. 
 Second especially: small 
 
 foot muscles, interossei 
 
 and flexor digitorum 
 
 brevis. 
 
 Muscles of the perineum 
 and the transversely 
 striated musculature of 
 the urethra (compressor 
 urethras) and of the rec- 
 tum (sphincter ani exter- 
 nus) and of the sexual 
 organs. 
 
 First sacralis. Outer and 
 middle part of the sole, 
 heel, parts of the dorsum of 
 the foot (inner side) , tips of 
 the toes. Outer and back 
 part of the calf. 
 
 Second sacralis. Middle part 
 of the back of the thigh and 
 
 Third sacralis. Skin over 
 the os sacrum, inner and 
 upper part of the thigh. 
 
 Fourth and fifth sacral root. 
 Perineum, anus, and con- 
 centric parts of the skin 
 over the os sacrum close to 
 the middle line, dorsum of 
 penis, and anterior surface 
 with the exception of the 
 root of the penis, skin of 
 the scrotum. 
 
 (In the female, back parts 
 of the large labia.) 
 
 Plantar reflex. 
 
 Achilles tendon re- 
 flex. According to 
 Ziehen, sacralis 1; 
 according to Qp- 
 penheim, lumbalis 
 5, sacralis 1 and 2. 
 
 Center for erection 
 2 and 3 sacralis. 
 For ejaculation 3 
 sacralis. 2 
 
 Detrusor and sphinc- 
 ter vesicae, unstrip- 
 ed musculature of 
 the rectum 3, 4, 
 and 5 sacralis. 3 
 
 Sphincter ani ex- 
 ternus 4 and 5 
 sacralis. 
 
 Anal reflex 5 sa- 
 cralis. 
 
 1 The segments for the flexores cruris and the glutaei are not yet clearly determined. The 
 former lie certainly higher than the latter; they may even be below the muscles of the calf. 
 Both lie certainly below the peronei. 
 
 2 According to L. R. Muller, not situated in the conus. 
 
THE CONDUCTING TRACTS OF THE SPINAL CORD 221 
 
 In recent times, with the aid afforded by the methods of chromatolysis in 
 amputations and muscle atrophies, it has been attempted to establish more 
 exactly the relations of the individual columns of nuclei in the anterior horns 
 to the muscle groups of the extremities. According to these investigations, for 
 instance, every cell column of the lumbar enlargement seems to stand in a 
 certain relation to all the muscles of a limb segment. Each of these seg- 
 mentary groups of nuclei might be divided into individual nuclei, which are 
 connected either with the function of similar groups of muscles, or with 
 single nerves of the limb segment concerned. Finally, there might be sub- 
 divisions here again for individual muscles. With these investigations as a 
 basis, schemes of the various columns of nuclei in the cervical and lumbar 
 enlargements with their proper functions have already been drawn up, 
 though no decisive results could be gained here. 
 
 If we until now have mainly been treating of the functions localized in 
 the individual spinal cord segments, we must now turn to the functional 
 importance of the conducting tracts of the spinal cord. Their anatomical 
 distribution we have discussed above. 
 
 The investigation of the function of the spinal cord tracts has undergone 
 great changes with the advancing knowledge of their anatomical structure. 
 Only since we have been precisely informed as to the course of the individual 
 tracts, which are often mixed in the same area, has it become possible to 
 confine their experimental disjunction to one or a few of them. Besides, the 
 accurate microscopic observation of the point of incision and the secondary 
 degenerations, in which Marchi's method has been of the greatest assistance, 
 places in our hands the indispensable means of controlling exactly the pre- 
 arranged injuries as to extent, as well as the manifestations that follow. 
 
 In addition to the experimental investigations in higher mammals, the 
 experience afforded by human pathology has been of great importance. 
 In very truth, it is indispensable for the ascertainment of the function of the 
 conducting tracts in man, since not only in anatomic structure, but also in 
 the physiological value of the individual tracts, there are important differences 
 between the various classes of animals and man. But one must always 
 remember, that in the sense of physiological experiment pure disjunctions 
 of definite divisions of the spinal cord in human pathology are the rarest 
 of exceptions, since pressure phenomena, focal diseases due to other causes, 
 etc., are quite liable to confuse the pure symptoms of lost function. 
 
 Considering the gradual development of our knowledge of the cerebro- 
 spinal conducting tracts, it is easy to understand, how observers were imme- 
 diately inclined to assign the individual functions to the few conducting 
 tracts that were known. Thus the pyramidal tract, especially, was long 
 considered as the only pathway for transmitting voluntary motor impulses 
 from the cortex of the cerebrum to the spinal cord. This conception found 
 its actual support in the proof of descending degeneration of the pyramidal 
 
222 DISEASES OF THE SPINAL CORD 
 
 tract in man after focal injuries to the brain, especially to the inner capsule 
 in connection with a typical hemiplegia. But it must be borne in mind, 
 that in this case, though only the pyramidal tract degenerates down to the 
 spinal cord, it was not alone interrupted in the brain by the focal injury, 
 but in connection with the other cortico-fugal tracts subserving the region 
 of the extremities. But even these wide spread disjunctions of the tracts, 
 that carry the motor impulses, do not prevent a considerable involution of 
 the hemiplegia after some time. 
 
 An isolated disjunction of the pyramidal tract is experimentally feasible 
 only by complete division of the pyramids in the medulla oblongata, where 
 they appear on the ventral surface and are not mixed with other fibres, or by 
 section of the pyramidal crossing itself making a longitudinal incision, 
 which at least produces, in the dog and lower ape, complete disjunction of 
 the pyramidal conductivity, since in them the anterior pyramidal tract is 
 wanting. In the lateral column of the spinal cord, however, the pyramidal 
 tract can be severed only in conjunction with the rubro-spinal and dorso- 
 lateral cerebellar tract. 
 
 While it had been known for some time, that after severing the pyramidal 
 tracts the motor effects in electric stimulation of the cortex of the cerebrum 
 remain unchanged, it has recently been possible to keep dogs alive for a 
 long time after the pyramidal tracts had been completely cut through. 
 Thus it was seen that the animals, immediately after waking from the 
 narcosis after the operation, were able to run about freely and, in the use of 
 their extremities, could not be differentiated from normal dogs. In uni- 
 lateral disjunction of the pyramids, a certain awkwardness in the employ- 
 ment of the muscles of the opposite side of the body was noted in perform- 
 ing unusual movements (waddling); but nothing of functional loss was 
 observed. Correspondingly, the isolated disjunction of the pyramidal 
 tracts does not essentially injure either the tactile reflex, passing over the 
 region of the extremities of the cerebral cortex, nor the effects of electric 
 stimulation of the region of the extremities. 
 
 Now, if the second important motor tract in the lateral column of the 
 dog is disjoined, the rubro-spinal bundle in the medulla oblongata above the 
 pyramidal crossing, that is, before the pyramidal fibres enter the lateral 
 column, there appears immediately clearly defined paresis of the limbs on 
 the same side, which, however, is completely recovered from in the next 
 few weeks. In this, also, electric stimulation of the region of the extrem- 
 ities shows no deviation from the normal; the tactile reflex, lost at first, is 
 soon recovered again (Fig. 53). 
 
 Not until a disjunction of both the pyramidal and the rubrospinal tracts is 
 performed in the lateral column of the upper cervical portion of the cord of 
 the dog, are serious phenomena of loss of function observed. After a tem- 
 porary, rather serious paralysis of the extremities of the same side, a spastic 
 
THE PYRAMIDAL TRACTS IX DOGS AXD APES 
 
 223 
 
 paresis of the limbs remains. The tactile reflex is permanently lost; iso- 
 lated movements of the extremities are no longer observed. Above all, the 
 effect of the electric stimulation of the crossed region of the extremities is 
 absolutely lost. In the dog, therefore, the impulses of the cerebral cortex 
 are transmitted to the spinal cord exclusively by way of the direct cortico- 
 spinal (pyramidal) and the rubro-spinal tracts. 
 
 In the lower apes also the total destruction of the pyramidal crossing has 
 the surprising result, that no paralysis of the extremities appears, not even 
 immediately after the operation. But not only the coarser voluntary move- 
 ments, as they appear in the acts of running and climbing, are retained, but 
 also the delicate isolated movements of the fingers which are necessary, for 
 instance, in reaching for small pieces of food, are absolutely intact. On 
 the whole, however, the ape. in whom the pyramid has been cut, performs all 
 his movements more slowly than the normal ape, and thus makes altogether 
 a clumsier impression (Fig. 54). 
 
 Fig. 
 
 -Division of the right postero-lateral column including the rubro-spinal bundle at the 
 height of the pyramidal crossing in the dog. Marchi preparation. 
 
 But that the pyramidal tracts are of considerably greater importance 
 in the ape than in the dog, is proved by the result of cerebral cortex stimula- 
 tion; when this is performed several weeks after the disjunction of the py- 
 ramidal tracts, only two small fields of the region of the extremities, those 
 corresponding to the centers for finger and toe movements, are proven 
 electrically excitable, whereas all the rest of the field does not respond. 
 
 Nor does the isolated disjunction of the rubrospinal tract of the lateral 
 column, before it joins with the pyramidal tract, lead to any paresis, worth 
 mentioning, of the extremities on the same side. In unilateral disjunction 
 they are somewhat more awkward for a few days than the extremities of the 
 other side. But after a few days they are used quite normally for all 
 purposes, including the most delicate grasping movements, with the excep- 
 tion that the normal arm is somewhat preferred. To this corresponds also 
 
224 DISEASES OF THE SPINAL CORD 
 
 the completely normal electric excitability of the crossed region of the ex- 
 tremities in the cerebral cortex. 
 
 Now, if in the ape, both pyramidal and rubro-spinal tracts are interrupted 
 together, by complete division of the postero-lateral column in the third 
 cervical segment, the result differs materially from that obtained in dogs. 
 On the first two days after such an operation the extremities concerned hang 
 down limply, and are but slightly used in the general movements. But in 
 the next few days, the motor power of the extremities gradually increases; 
 arms and legs are used not only in general, but possibly even better in isolated 
 movements. Indeed, after a week the motor weakness is virtually wholly 
 adjusted. After special practice (the sound arm is bound down for some 
 
 Fig. 54. — Division of the pyramidal crossing in the ape (Macacus rhesus) . Marchi preparation. 
 
 time every day) the ape prefers this arm, even for grasping. In the ape, in 
 contradistinction to the dog, and still sharper contrast to man, no spastic 
 phenomena follow such lateral column disjunction. 
 
 It is in full accord with this result, that in the ape the electrical stimula- 
 tion of the region of the extremities several weeks after a unilateral disjunc- 
 tion in the postero-lateral column still transmits motor impulses to the spinal 
 cord, which, though they are virtually confined to the fields of the hand and 
 foot region, are still somewhat more marked than may be observed in bilat- 
 eral isolated pyramidal division. 
 
 Since, then, the combined disjunction of the cortico-spinal and the 
 rubro-spinal tracts in the ape leads only to a slightly greater disturbance of 
 the voluntary movements of the extremities, than the isolated disjunction of 
 each of these tracts, other tracts must be involved in their conduction to 
 the spinal-cord centers. In the first place it can easily be proven that it 
 is not only, as one might think, the corresponding conducting tracts of the 
 other half of the spinal cord which permit the impulses to cross over to the 
 injured side at the level of the corresponding anterior-horn centers. Even 
 in the dog, bilateral disjunction of the postero-lateral column leads to no 
 serious disturbance so far as the usual locomotion is concerned. In apes, 
 
SECTION OF THE AXTERIOR COLUMNS IN DOGS 
 
 also, to the complete division of one postero-lateral column, a complete 
 division of the other may be added after 2 to 3 weeks, and still the ape can 
 learn to run and climb again after a few days, and is able, though with 
 lessened vigor, to reach for food. Furthermore, even if in the ape first the 
 entire arm region of one hemisphere of the cerebrum is removed to the 
 extent marked out by Munk, and the isolated grasping movements of the 
 opposite arm are thereby lost by complete destruction of the corresponding 
 pyramidal tract, and to this is added, after a 
 few weeks, the disjunction of the postero-lateral 
 column of the same side in the third cervical 
 segment, which has been innervating the here- 
 tofore normal extremities, he can nevertheless 
 soon learn to use these extremities again for 
 general movements and isolated grasping mo- 
 tions. Indeed, the disjunction of the impulses 
 of the other side can be carried still farther, by 
 cutting through the same ape's other postero- 
 lateral column in the first cervical segment, 
 thus disjoining the one remaining rubro-spinal 
 bundle and the few uncrossed pyramidal fibres 
 which radiate into this column, only to find that 
 
 the restitution of the arm, which still has its FlG> 55 ._ Co mbined disjunc- 
 cerebral cortical centers, is not impeded, though tion of one arm region and both 
 
 . « postero-lateral columns. I, Ex- 
 the motor lateral column tracts On both Sides tirpation of the left arm region 
 
 are totally disjoined. These movements of the " £f /»«^. £SSaS 
 extremities concerned are present even though column and of parts of the anterior 
 
 , . . M J ,. . , . ,. column in the third cervical seg- 
 
 only at first to a very limited extent, lmmedi- ment ; in, complete division of 
 atelv after complete division of the postero- ^ e ^ ht postero-lateral column in 
 
 J *■ x the first cervical segment. 
 
 lateral columns, so that an absolute restitution 
 
 should not be spoken of, as part of the motor impulses must already be 
 passing normally outside of the motor lateral column tracts of the spinal 
 cord (Fig. 55). 
 
 Here, then, the anterior columns must be considered; in them we have 
 already met with (see above) a series of cerebro-spinal tracts passing to the 
 spinal cord. Older physiologists already ascribed to them the most impor- 
 tant share in the conduction of movements. But, nevertheless, they were 
 later neglected as compared with the pyramidal tract, inasmuch as even 
 human pathology furthered the knowledge of the motor functions of the 
 anterior columns but slightly. Isolated disjunction of the anterior columns 
 may be performed in dogs, from the front, along the lower margin of the atlas, 
 directly beneath the pyramidal crossing. Such an isolated disjunction of 
 the anterior columns leads to no paralysis of the extremities. On the other 
 hand, the anterior columns subserve a function especially important for the 
 
226 DISEASES OF THE SPINAL CORD 
 
 station of the animal, namely, the innervation of the musculature of the 
 back. The dog, deprived of his anterior columns, runs in a wobbly fashion, 
 with legs apart and with the back part of his body shaking from side to side. 
 He can not turn sideways in a normal way, and when he is put on the table 
 with the back part of his body hanging down over the edge, he can not raise 
 it as a normal dog can. Electric cerebral cortex irritation of the regions of 
 the extremities, however, does not show the slightest loss of function, after 
 the anterior column has been disjoined. 
 
 In the dog, therefore, neither the disjunction of the lateral, nor that of 
 the anterior columns, brings about paralysis of the extremities, though 
 under normal conditions the motor impulses, which leave the cerebrum, 
 according to the results of stimulation of the cerebral cortex, seem, on the 
 whole, to use the lateral column. Since, on the other hand, the disjunction 
 of the pyramidal tract together with the anterior column, stops neither the 
 motor activity of the extremities, nor the effect of stimulation of the cerebral 
 cortex, we reach the important conclusion, for the dog at least, that all these 
 motor lateral column and anterior column tracts can mutually, to a great extent, 
 replace each other, with the exception that the impulses for the isolated 
 movements, dependent exclusively on the cortex of the cerebrum, use the 
 lateral column tracts par preference. 
 
 In the monkey, an isolated disjunction of the anterior column has not yet 
 been attempted. It has, however, been ascertained, that in the ape also a 
 destruction of the anterior column together with the pyramidal tract does 
 not cause any loss of motor function of the extremities concerned, nor a 
 total abolition of the electric cortical excitability, so that the rubro-spinal 
 tract alone, might, even in the ape, suffice to conduct these impulses. But 
 there can be no doubt, according to the above quoted results of disjunction 
 of the lateral column, that the anterior column possesses in the ape a higher 
 physiological value than in the dog, since it is used in great measure also 
 for the conduction of the isolated movements dependent on the cerebral 
 cortex, even to the most delicate play of the fingers. 
 
 But now, if in the dog or monkey the entire motor conduction of half the 
 spinal cord is disjoined, either by induction of a complete hemilesion, or 
 by severing only the anterior and lateral columns together in the upper 
 cervical portion, it appears that even then motor impulses from the other 
 half of the spinal cord may. reach the spinal centers of the extremities con- 
 cerned. Thus, after transient serious paralysis a moderate degree of 
 movement is regained, even though the excitability of the opposite cerebral 
 hemisphere is lost, and in the monkey, at least, the grasping movement of 
 the affected arm remains seriously injured. 
 
 The remarkable power of restitution which, with respect to coarse 
 locomotion, is innate in the spinal cord, is best demonstrated by an experi- 
 ment on the cat, in which the motor tracts of the lateral column, the medial 
 
THE MOTOR SPINAL CORD TRACTS IN APE AND MAN 227 
 
 halves of the anterior columns, and in addition the posterior columns on 
 both sides, were destroyed. In spite of the fact that the cat was totally 
 paralyzed in all four extremities for 2 1/2 weeks, ordinary walking move- 
 ments gradually returned, which improved to such an extent in the course of 
 1 1/2 months, that the cat was able to reach by jumping, and to bring down 
 meat from the edge of a table of ordinary height. For this action, which 
 certainly demands a considerable amount of strength and agility, the 
 lateral remnants of the anterior columns sufficed in this case, though the 
 extensive destruction of sensory fibres had certainly increased the difficulty 
 of restitution. 
 
 In the anthropoid ape, experiments concerning the functions of the motor 
 conducting tracts, which, as is easily understood, would be of especial value 
 particularly because of the far reaching analogy of its anatomic structure 
 with that of man, have been carried out only in isolated cases. The destruc- 
 tion of the smaller medial half of one pyramid immediately above the pyram- 
 idal crossing, is, in the chimpanzee, followed by slight weakness in the arm 
 concerned, without decided phenomena of paralysis, without any indication 
 of spasms, and with retention of the delicate grasping movements of the 
 fingers. A destruction of the medial half of one anterior column, including 
 the anterior pyramidal tract, which in the chimpanzee already appears in 
 the second cervical segment, caused, likewise, but a slight tendency to 
 spare tfre extremities on the same side, especially the arm, without the least 
 disturbance even of the most delicate finger movements. 
 
 Concerning the functions of the motor spinal cord tracts in man, it was 
 supposed, up to most recent times, that the pyramidal tract represented the 
 path of voluntary movements and that its destruction was necessarily 
 accompanied by paralysis of the corresponding extremities, with spasms and 
 exaggeration of the tendon reflexes. If in hemiplegias, the result of lesions 
 in the internal capsule, a return of voluntary movement could, as a rule, 
 be noted after a shorter or longer period of time, this was supposed to be 
 due to the fact that part of the pyramidal fibres were preserved. But after 
 the possibility of almost complete functional replacement of the pyramidal 
 tracts had been proved by experiments on animals, especially by those on 
 such monkeys, which in the structure of their nervous system and in the 
 functions of their extremities, especially the arms, resembles man, a revision 
 of the former theory in man was also unavoidable. The exact study of 
 cerebral hemiplegias proved, that in spite of a totally destroyed pyramidal 
 tract, an extensive restitution of the motor function might occur, which, if 
 the defects be acquired in earliest childhood, may even approach the normal 
 conditions. Even in spinal hemiplegia, due to total destruction of one 
 lateral column in the spinal cord, there is a restitution of approximately the 
 same extent. In bilateral loss of the lateral pyramidal tract, as it has been 
 observed in approximately pure form in some cases of "spastic spinal pa- 
 
228 DISEASES OF THE SPINAL CORD 
 
 ralysis," there is no serious paralysis, but only a serious hindrance in walking 
 because of rather severe spasms in the legs. 
 
 As all these results prove with certainty that the loss of the pyramidal 
 tracts is not followed, in man, by an irreparable loss of voluntary movements, 
 there remain only as consequences of the loss of the pyramidal tracts, exag- 
 gerated tendon reflexes with clonus, as well as the Babinski toe reflex and 
 spasms. Whether the latter are an absolutely necessary result of the loss 
 of pyramidal functions is, to say the least, questionable. In human path- 
 ology cases without injury of the pyramidal tracts have been observed with 
 severe spasms, as well as other cases in which diseases of the pyramidal 
 tracts were present with scarcely a suggestion of spasms. But it must be 
 granted, that the combination of disease of the pyramidal tract and muscle 
 tonus increased to spasm represents the rule. 
 
 But even though the pyramidal tract is unquestionably capable of a con- 
 siderable measure of replacement in man too, still its importance is far 
 greater, than it is in all animals up to apes, and it seems practically certain, 
 that sudden isolated interruption in the conductivity of the pyramidal tracts, as 
 occurs in rare cases in the medulla oblongata, is immediately followed by 
 serious paresis. 
 
 Considering now the question of compensation tracts for the pyramidal 
 tracts in man, the rubrospinal bundle seems to have become so rudimentary 
 here, that it can scarcely be considered in the restitution of the motor func- 
 tion. So far as the anterior pyramidal tract is concerned, which, of course, 
 escapes in spinal diseases of the lateral column, and might possibly be of 
 importance for motor conductivity, nothing certain is yet known about its 
 function. We know of no difference between the symptoms of total destruc- 
 tion of the pyramidal tract above the crossing, and a lesion of the lateral 
 column, with the anterior pyramidal tract intact. The above mentioned 
 experiment, in which the anterior pyramidal tract of one side, was disjoined 
 in the chimpanzee, seems to make it apparent, that it is not connected in any 
 way with the other half of the spinal cord. This leaves the other anterior 
 motor tracts to compensate for the pyramidal tract, analogous to the well 
 established relations in the lower ape. Furthermore, pathology proves 
 definitely, that in extensive destruction of the anterior tracts, the conduc- 
 tivity through the lateral pyramidal tracts suffices for the retention of 
 motility. 
 
 And finally in man also, after destruction of one entire half of the spinal 
 cord, the motor conductivity of the other side may bring about considerable 
 recovery, especially for the leg of the injured side. In spite of initial serious 
 paralysis of the leg, such individuals can, months and years afterwards, 
 take long walks without assistance. Insufficient observations for satis- 
 factory conclusions relative to this matter have been made concerning the 
 arm. ■ 
 
FUNCTION OF ASCENDING TRACTS OF SPINAL CORD 229 
 
 Since, therefore, the pyramidal tract can be replaced almost completely 
 in higher mammals, and very largely in man, by means of the other motor 
 spinal cord tracts, so that it is not possible to ascribe to it a specific function, 
 this question arises : of what importance is the greater and greater develop- 
 ment, of such an uninterrupted cortico-spinal tract in the ascending animal 
 series? The supposition, that this direct connection between the cerebral 
 cortex and the spinal cord is necessary for the individual's learning of special 
 motions, is contradicted by the established fact, that the ape, even after 
 being deprived of his pyramidal tracts, can be taught new and complicated 
 arm movements. Since the cerebral cortex can influence the spinal centers 
 through the pyramidal tracts only without the assistance of the subcortical 
 brain centers, it is probable, on the contrary, that in the learning of new 
 movements, the influence of these latter centers upon the gray matter of the 
 spinal cord is necessary for clearing the path of the groups of ganglion cells 
 in the anterior horns, which are essential to this. But when the movement 
 is completely controlled, the direct transference of the impulse from the 
 cerebral cortex to the spinal cord, with its anterior horn cells already ar- 
 ranged for the definite combinations of muscles, suffices and the centers of 
 the mid- and hind-brains may be passed over. The pyramidal tract is, 
 accordingly, the real conducting tract for the immense number of movements 
 already acquired; the cerebro-spinal tracts, on the other hand, that are 
 interrupted in the mid-brain, with their relations to the cerebellum, are of 
 the utmost importance in learning movements. 
 
 In turning now, to the function of the ascending tracts of the spinal cord, 
 we shall first consider the disturbances, which are caused by complete eli- 
 sion of all the sensory impulses of an extremity passing through the posterior 
 roots to the spinal cord. For instance, if, in the ape, we cut away all the poste- 
 rior roots concerned with the arm, from the fourth cervical to the fourth 
 dorsal segments, there is not only, as is to be expected, total loss of 
 all sensory conductivity; but the grasping movements of the arm concerned 
 also disappear at first. It was therefore supposed, that through this injury 
 the isolated movements dependent on the region of the extremities in the 
 cortex were being seriously impaired, or even destroyed with rather slight 
 injury to the general movements, thereby proving the necessity of the impulses 
 carried from the skin and muscles to the central organ for the execution of 
 the most highly organized movements. But, in contradistinction to this, 
 the most recent investigations have shown, that in fact, all normal movements 
 of the extremities are injured by the loss of all the centripetal impulses. If 
 one avoids all collateral injuries of the spinal cord, the disturbances of the 
 general movements, which are dependent on the subcortical centers, remain 
 unchanged. The grasping movements, however, which are principally or 
 entirely dependent on the centers of the cerebral cortex, improve under the 
 influence of practice; the loss of the stimulations, that normally pass to the 
 
2 3 o DISEASES OF THE SPINAL CORD 
 
 region of the extremities through the sensory tracts, is extensively compen- 
 sated for by the other sensory impulses, especially those of the visual sense. 
 Concerning the function of the individual ascending conducting tracts of 
 the spinal cord, the conducting paths for the various forms of sensation must 
 be regarded separately, whether one assumes now that they are specifically 
 differentiated from the periphery, or that they acquire their peculiar charac- 
 teristics only with the aid of the spinal centers. We must therefore consider 
 separately : 
 
 i. The tract for pain sensations. 
 
 2. The tract for temperature sensations, in which sensations of cold 
 and those of heat must be separately regarded. 
 
 3. The tract for pressure sense. 
 
 4. The tract for tactile sensations. 
 
 5. The tract for deep sensibility (bathycesthesia) , among the com- 
 ponents of which muscle sense with position, feeling, sensations 
 of movement, etc., are most important. 
 
 Just as with motor spinal cord tracts, observers have endeavored to 
 assign to each one of the known centripetal conducting tracts a function 
 peculiar to itself alone. But here, too, it has been established with the 
 increase of anatomic knowledge and the refinement of physiological investi- 
 gation, more and more clearly, that several tracts may share the same func- 
 tion, and to a great extent, compensate for one another. Unfortunately, 
 however, animal experiments relative to the conduction of sensibility, suffer 
 from the difficulty in separating clearly the conscious sensations, rising in 
 the cerebral cortex, from the general feelings evoked in the deeper centers 
 of the brain. 
 
 The conduction of pain sensation in the dog and monkey is mostly given 
 over to the lateral columns; their disjunction lowers it considerably, though 
 it does not wholly destroy it. It is certain, at the same time, that the majority 
 of the fibres concerned in the sense of pain, after their entry into the gray 
 matter of the spinal cord, and especially of the posterior horn, crosses to 
 the other half of the spinal cord, without, however, in the higher mammals a 
 complete crossing of pain conductivity taking place. Disjunction of one lat- 
 eral column, therefore, removes the pain sensation on neither side, but lowers 
 it very markedly temporarily on the opposite half of the body. One can 
 prove, furthermore, that the conduction of pain in the lateral column is, 
 on the whole, confined to its ventral half. When the lateral columns are 
 conserved, on the other hand, disjunction of the balance of the spinal cord 
 columns leads to no disturbance of pain sensation. After destruction of the 
 lateral columns in the dog, the preserved remainder of pain conduction, with 
 which no exact localization is connected, and which probably represents 
 only the subcortical components of the sensations of pain, just as it is retained 
 
TRACTS FOR PAIN, TEMPERATURE AND PRESSURE 231 
 
 after the extirpation of the cerebrum, is in no way influenced by the dis- 
 junction of the posterior columns. On the other hand, the additional dis- 
 junction of the anterior columns in the upper cervical portion of the cord 
 leads to an almost total loss of the pain sensation. But even now, if all the 
 white spinal cord columns are severed at various levels of the cord, there 
 still remains a remnant of pain sensation, induced by strongest stimulations. 
 
 If, therefore, the antero-lateral columns must be considered the most 
 important tract in the conduction of pain sensations, it is, nevertheless true, 
 that the anterior columns are also concerned, in this function, even if but 
 slightly, or are at least able to assume this function by substitution. Finally, 
 there must be considered the possibility, even if it be but a very slight one, 
 of the sensations of pain being conducted through the short paths of the 
 gray matter. This conception corresponds to the older discoveries in the 
 rabbit, that the conduction of pain is not interrupted by a unilateral lesion 
 of the spinal cord in a definite segment, and a second lesion of the other 
 half of the spinal cord a few segments higher up. In the ape, on the other 
 hand, such a bilateral hemisection of the spinal cord leads to complete loss 
 of sensibility. 
 
 Since the discovery of Gower^s ventral spino-cerebellar tract in the antero- 
 lateral column, the chief conduction of the sensation of pain has been fre- 
 quently ascribed to this tract. But since we know that Gower , s tract ends 
 in the cerebellum and that total extirpation of the cerebellum does not result 
 in any disturbance of the pain sensation, this view must be rejected as irrecon- 
 cilable with the facts. In the conducting path for the sensation of pain in 
 the anterior column no continuous tract is concerned, but there is a chain 
 of shorter and longer intersegmental nerve tracts, always returning to the 
 gray substance of the spinal cord, the longest fibres of which, might, of course, 
 commingle with those of Gower's tract (Fig. 56). 
 
 Concerning the temperature sense, its conduction in the lateral column 
 corresponds in the main with that of the pain sensation. In the monkey 
 there seems to be a bilateral conduction, showing a preference for the crossed 
 side. But new experiments, carried out by Kalischer's training methods, 
 have proved, that in the dog the conscious temperature sensations are through- 
 out their course crossed. The training, connected with the temperature 
 sense, was lost after severing of the crossed half of the spinal cord. 
 
 The conduction of the pain sense is paralleled by that for the pressure 
 sense; when pressure is in any way increased, it is easily transformed into 
 a pain sensation. Here, too, the main conducting path lies in the lateral 
 column with preponderance of the crossed conduction in the spinal cord. 
 In destruction of a lateral column in the dog, an increase of the pressure 
 sense of the same side is often noted ; it is accompanied also by a hyperal- 
 gesia. On the other hand, disjunction of the posterior column seems in 
 no way to influence the pressure sense. 
 
232 
 
 DISEASES OF THE SPINAL CORD 
 
 So far as the tactile sensation is concerned, it is naturally dependent upon 
 a certain degree of pressure sense, and is lost, therefore, when the other is 
 markedly lowered, as after bilateral disjunction of the lateral column. But, 
 on the other hand, with retained or even increased pressure sense, the delicate 
 localized tactile sensations may be severely lowered, or altogether lost. The 
 
 Fig. 56.— Conduction of the pain Fig. 57. — Conduction of the touch sensation 
 
 sensation in the spinal cord, a, Crossed in the spinal cord. H, Posterior column; V, 
 
 antero-lateral column^ b, antero-lateral anterior column, 
 column at the same side; c, crossed 
 anterior column; p, posterior root. 
 
 conduction of the tactile sensations has been previously credited exclusively 
 to the long posterior column tracts, which, without interruption ascend 
 from the periphery and the spinal ganglia to the nuclei of the medulla oblon- 
 gata and possess a secure connection with the cerebral cortex by way of the 
 lemniscus tract and the optic thalamus. According to previous investiga- 
 tions, total division of the posterior column in the dog was said to destroy 
 the tactile sensation, whereas, on the other hand, in abnormal heightening 
 
TACTILE AND MUSCLE SENSE 233 
 
 of the sensibility, due to serious loss of blood, a rabbit, whose entire cord 
 was divided with exception of the posterior column, still reacted to slight 
 touch of the hind feet with movements of his head and ears. New experi- 
 ments, on a dog, in which the total division of the posterior column, without 
 additional injury, was established by exact microscopic investigation, have 
 proved, however, that the tactile sensations are retained intact after total dis- 
 junction of the posterior cord. Nor does an extension of the unilateral division 
 of the posterior column to the posterior horn and the extreme dorsal layer 
 of the lateral column destroy tactile sensations in the extremities of the side 
 concerned. If in the normal dog, one severs the anterior column in the 
 first cervical segment, the tactile sensation is at first lowered, but soon 
 returns to its normal degree. But if total division of the posterior column 
 is combined with that of the anterior column in the upper cervical portion of 
 the cord, the tactile sensations are completely lost, and the tactile reflexes 
 disappear in spite of the intactness of the lateral column. At the same time 
 the ordinary pressure sense and the pain sensations are in nowise disturbed. 
 
 There exists, therefore, a double conducting path for the tactile sensations, 
 that through the posterior column on the same side, and that through the 
 anterior column (probably only crossed). The anterior column tract of 
 the tactile sensation must be built up in the spinal cord from shorter inter- 
 segmental tracts, since long anterior column fibres, ascending to the medulla 
 oblongata from the lower spinal cord segments are not known. Both tracts 
 are able to immediately replace each other, and both might, therefore, be 
 normally used (Fig. 57). 
 
 For the muscle sense as well, the most important components of which 
 are the sense of position and the co-ordination of movements, the posterior 
 column was oftenest considered as the path of the conduction. But here too 
 the isolated posterior column section in the dog results in no disturbance 
 of the functions mentioned. In most recent times observers have given 
 special attention to the spino-cerebellar lateral column tracts, since unques- 
 tionably a large number of sensations stream to the cerebellum from the 
 domain of the deep sensibility, which are used up for the more delicate 
 regulation of the equilibrium. If now these spino-cerebellar tracts, which 
 occupy the margin of the lateral column, are completely divided in the dog, 
 on one or both sides hypotonia results in the extremities on the same side and 
 a disturbance in regulation of the principal movements in the roots of the 
 extremities, which manifest themselves in standing, as well as in walking, 
 as abnormal spreadings and overcrossings. These disturbances, which 
 are suggested already in total division of the dorsal spino-cerebellar tract, 
 appear in full severity only in total division of both spino-cerebellar tracts, 
 the dorsal and the ventral. But all these pathological phenomena retro- 
 gress quickly, so that after four weeks, even with bilateral disjunction of 
 these tracts, scarcely any disturbance is present. 
 
234 DISEASES OF THE SPINAL CORD 
 
 Quite similar phenomena of loss of function of similarly transitory 
 nature appear also after isolated anterior column disjunction in the upper 
 cervical portion of the cord, so that we must ascribe also to the anterior 
 column centripetal conduction for the deep sensibility, especially for the 
 muscle sense. Especially severe is the appearance of these disturbances of 
 the muscle sense in combined disjunction of the anterior and lateral columns; 
 on the other hand, the intact lateral columns can almost completely take 
 over all the innervations concerned here, after total disjunction of the anterior 
 and posterior columns, followed by temporary disturbances. 
 
 It is probable, therefore, that in the conduction of the impulses brought 
 together in the muscle sense, all columns of the spinal cord are concerned, 
 even though under normal conditions the main conduction may be a func- 
 tion of the lateral columns. The larger number of impulses concerned here 
 ascend in the same half of the spinal cord; but for the position sense, for 
 instance, a bilateral path of conduction seems to be present. 
 
 If we again consider the conducting paths of the centripetal impulses, 
 especially as they appear after the experiments on the dog, we must say, 
 that the posterior columns have no specific, irreplaceable function. They are 
 important factors in the conduction of the tactile sensations, and are prob- 
 ably able to take over a part, if only a small one, of the conduction for the 
 muscle sense. The anterior columns are the second pathway for the conduc- 
 tion of tactile sensations, are feebly active in the conduction for the pain 
 sensation the fibres probably ending subcortically, and take a not incon- 
 siderable part in the conduction of the deep sensibility. The lateral columns 
 finally are the main tracts for the pain and temperature sense, for the pres- 
 sure sense and also for deep sensibility; only for the delicate tactile sensations, 
 they possess no direct components, but can indirectly destroy them by very 
 severe disturbance of the pressure sense. • 
 
 Incidentally the curious fact must be mentioned here, that the posterior 
 columns, the disjunction of which does not at all interfere with pain con- 
 duction, represent the most sensitive place (to pain) of the entire spinal 
 cord — perhaps even of the entire central nervous system. Even the thor- 
 oughly anaesthetized animal starts up whimpering when the columns are 
 pricked, and this is not because of tearing or cutting the root, which in fact 
 is not nearly so painful, but there must be special endogenous pain tracts 
 of the spinal cord, which course in the posterior column. 
 
 Complete division of an entire half of the spinal cord, in both dog and 
 monkey is followed at once by disturbances of the movements of the extrem- 
 ities on the same side, which in the monkey are more serious and of longer 
 duration than in the dog, but which in both animals disappear to a great 
 extent in a few weeks, corresponding to the relations of the motor conducting 
 tracts, as discussed above. So far as disturbances of the various sensory 
 qualities, after such a division of the spinal cord are concerned, it is shown 
 
SENSORY TRACTS IN MAN 235 
 
 by numerous competent experiments, that the muscle sense is disturbed 
 much more on the side of the division and that the pain and temperature, 
 as well as the pressure sense, are especially affected on the opposite side. 
 But in the dog none of these forms of sensibility are destroyed by a half 
 sided lesion of the cord, in fact, as a rule, they are generally restored almost 
 to their normal condition in the course of time. Each of the two halves 
 of the spinal cord, then, is able, to a certain degree, to conduct sensory 
 impulses for both halves of the body. But even after two hemi-lesions of 
 the spinal cord, which at a distance of several spinal segments, affect,' now 
 the right, now the left half, the sensory conductivity is not abolished, nor is 
 it entirely destroyed by a third lesion a few segments higher, on the first side. 
 
 How do these results attained in the higher mammals, compare with 
 those reached in man? Here we have, it is true, the immeasurable advan- 
 tage, that by the help of speech much more exact information may be had 
 about the sensations brought to consciousness; but, on the other hand, it is 
 only seldom that definite focal injuries are observed with such clear phenom- 
 ena of loss of function, as animal experiments offer us. Thus, for instance, 
 in the posterior column affections of the tabetics, there is never mere loss of 
 the posterior column fibres, but a combination with diseases of other pos- 
 terior root fibres, which end in the gray substance, so that naturally no 
 conclusions can be drawn concerning the functions of the posterior column 
 from the phenomena of functional loss, observed in such cases. Also in 
 tumors, which develop in definite parts of the spinal cord, for instance in the 
 lateral column, one must be very careful in interpretation of the symptoms, 
 because of their distal effects. 
 
 The best observations in human pathology for establishing the conducting 
 tracts of sensation in the spinal cord, therefore, are presented by the punctured 
 wounds of the spinal cord. These occur, as a rule, in young healthy individ- 
 uals, and are comparable, in the simple division of definite portions of the 
 spinal cord, with the conditions of the animal experiment. Here, however, 
 there is an insufficiency of autopsy findings. 
 
 Punctured wounds, in which the knife enters the vertebral canal from 
 behind, are followed, as a rule, by the appearance of the Brown-Sequard 
 symptom-complex. Motility and muscle sense are lost on the side of the 
 injury, pain and temperature sense, and, also to a greater or less extent, the 
 pressure and tactile sense on the opposite side. On the injured side a slight 
 tactile hyperesthesia is noticed; also as a rule at the upper boundary of the 
 anaesthesia, a small hyperaesthetic zone may be detected. In addition to 
 punctured wounds with approximately clean division of half the spinal cord, 
 this symptom complex, more or less pronounced, appears in cases of tumor, 
 softening, hemorrhage, meningomyelitis of the spinal cord, which are local- 
 ized preeminently in one-half of the spinal cord (Fig. 58) . 
 
 We have already said above, that the loss of motility, appearing after 
 
236 
 
 DISEASES OF THE SPINAL CORD 
 
 a unilateral lesion of the spinal cord, is capable of marked retrogression, 
 especially in the lower extremity. So far as the individual qualities of 
 sensation are concerned, the crossed conduction for the pain sense is un- 
 doubtedly far more developed in man than in animals. First, in all com- 
 plete divisions of one-half the spinal cord and even of a lateral column, the 
 pain sense of the opposite half of the body is lost, and limited restitution is 
 
 Fig. 58. — Left hemisection of cord, a, Motor and vasomotor paralysis, b and d, anaesthesia of 
 the skin; c, hyperesthesia of the skin. {After Erb.) 
 
 observed only after the lapse of considerable time. But there are a long 
 line of observations on record, in which after a one-sided lesion of the spinal 
 cord, complete loss of the pain sense was observed to continue for many 
 years. But in all these cases the lesion seems to have extended to the other 
 half of the spinal cord also. From the few cases that lived for years after a 
 punctured wound of the cord, and whose spinal cord condition could then be 
 
SEXSORY TRACTS IN MAN 237 
 
 ascertained, it may be concluded with certainty that in man also, pain sen- 
 sations, may be conducted though very incompletely along spinal cord tracts 
 of the same side. Thus appears occasionally the symptom of allocheiria, in 
 which, as the pain stimulus is carried only to the centers of the cerebral 
 hemisphere of the same side and hence, as usual projected upon the oppo- 
 site side of the body, the pain is not felt at the point of injury, but at the 
 corresponding place in the other half of the body. 
 
 The temperature sense in man is conducted exclusively and normally, in 
 the opposite half of the body, and as a rule does not seem to be restored, even 
 after a unilateral lesion of the spinal cord has persisted for years. But we 
 have at least one trustworthy observation, in which the preservation of the 
 lateral column on the same side sufficed in spite of destruction of the entire 
 opposite half of the spinal cord, to make an incomplete restitution of the 
 heat sense possible after some years. Furthermore, several observations 
 of almost isolated destruction of the antero-lateral column teach, that in 
 man also, under normal conditions, the entire conduction for the pain and 
 the temperature sense passes through this column. But all the objections 
 that were quoted against the transference of this conduction to Gower's 
 tract in animal experimentation, must be renewed here. 
 
 A separation of the pressure sense from the more delicate tactile sensa- 
 tions, has been accurately observed in only a few instances in man. At 
 any rate, the pressure sense, far more than the pain and temperature senses, 
 employs in addition to the opposite tracts, a less important system of con- 
 duction on the same side. For this conduction also, the antero-lateral column 
 seems to take first rank. To what extent posterior and anterior columns 
 may serve as compensating tracts, has not yet been definitely determined. 
 
 For tactile sensations, there is definite proof, from exact analysis of 
 competent observations, that normally it must employ two tracts, one on 
 the same, the other, on the opposite side. In many cases of unilateral lesions 
 of the spinal cord, therefore, the tactile sensations are retained, while the 
 pain and the temperature senses are lost. There is no doubt that one of the 
 tracts for the tactile sensations courses in the posterior column of the same 
 side. On the contrary, the frequently assumed hypothesis, that the second 
 tract uses the opposite lateral column, is untenable in view of the observa- 
 tions presented; this second crossed tract must in accordance to the results 
 of the animal experiments, pass through the anterior column. Therefore, 
 in punctured wounds, in which the knife penetrates obliquely from behind, 
 and in which, as a rule, one lateral and both posterior columns are destroyed, 
 the tactile sensations are retained on both sides, since both anterior columns 
 remained uninjured. That, on the other hand, the long posterior column 
 fibres, that pass without interruption through the gray matter of the spinal 
 cord to the medulla oblongata, conduct tactile sensation is proven by the 
 retention of the latter in extensive cavity formations in the gray substance 
 
238 DISEASES OF THE SPINAL CORD 
 
 of the spinal cord, though the pain and the temperature senses are lost 
 (partial sensory paralysis). 
 
 If we turn, finally, to the muscle sense, we find that the position sense, 
 about which alone we have exact knowledge, is at first lost in hemilesions 
 of the cord, on the side of the lesion, but is, as a rule, considerably, even 
 though not entirely, recovered. Here too, in man, the anterior columns 
 are concerned in the condition as well as the spino-cerebellar lateral column 
 tracts while the degree of participation of the posterior column is as yet 
 doubtful. 
 
 The Brown-Sequard symptom complex, is, accordingly in man only in so 
 far present as at first after a half sided lesion, the motility and the muscle 
 sense are lost on the same side, the pain and temperature and mostly the 
 pressure senses on the opposite side, whereas the tactile sensations from the 
 very beginning have conduction upon both sides. But neither the conduc- 
 tion on the same side for motility, nor that on the opposite side for sensibility, 
 is absolute. An extensive restitution of the functions through the other 
 half of the spinal cord takes place, a phenomenon of the greatest importance 
 for therapeutic efforts in human pathology. 
 
 If now this one sided lesion of the spinal cord affects the upper cervical 
 portion, another series of phenomena of loss of function may be observed in 
 addition. First respiration ceases on the side of the lesion, as the descend- 
 ing tract from the centers of the medulla oblongata to the phrenicus-center 
 in the fourth cervical segment is interrupted. This cessation of the working 
 of half of the diaphragm is permanent in man as well as in the dog and the 
 ape. Yet, if, in the animal experiment, after a duration for weeks and 
 months of the inactivity of one side of the diaphragm, the phrenic nerve of 
 the other half of the diaphragm, that has continued its functions, be cut, and 
 thereby this half also be made inactive, then, the paralyzed half immedi- 
 ately begins to work again (Porter's experiment). The respiratory stim- 
 uli, descending from the medulla oblongata in the sound half of the spinal 
 cord, and which are now no longer able to reach the same half of the dia- 
 phragm, make a commissural path, at the level of the fourth cervical seg- 
 ment, to the phrenicus center on the other side, thus awaking it to renewed 
 activity; a peculiarly instructive example of possible processes of restitution 
 in the central nervous system. These spinal respiratory paths pass, as has 
 been proven, by attempts to sever them in the dog, for the most part in the 
 ventral part of the antero-lateral column, to a lesser degree in the lateral part 
 of the anterior column. 
 
 Furthermore, one observes in both animal experiments and in man, 
 after a high half sided cervical lesion, an increase in cutaneous temperature 
 on the side of the injury, which is said to be due to a paralysis of vasomotor 
 tracts as they pass from the medulla oblongata to the spinal cord, coursing 
 more than likely, also, in the antero-lateral column. 
 
TABES DORSALIS 239 
 
 Finally, let us recall once more the oculo-pupillary symptoms after a half 
 sided lesion of the cervical portion of the cord. They consist of narrowing 
 of the palpebral fissure, of contraction of the pupil and occasionally also, of 
 a retraction of the eyeball (enophthalmos) , and are due to a severing of the 
 sympathetic fibres, passing from the medulla oblongata to the oculo-pupil- 
 lary center in the first dorsal segment. Here, too, a definite restitution of 
 the nervous conduction is apparently possible, through the other half of the 
 spinal cord. 
 
 Concerning the many finer details of diagnostic importance, especially 
 with regard to the lowest segment of the spinal cord, we must refer the reader 
 to the separate chapters on the pathology of the spinal cord. 
 
 2. SYSTEMIC DISEASES OF THE SPINAL CORD 
 
 (a) Tabes Dorsalis 
 
 (Ataxie locomotrice progressive. — Locomotor ataxia. — Ruckenmarkschwindsucht) 
 
 BY 
 
 S. SCHOENBORN (Heidelberg) 
 
 We designate as tabes dorsalis, or tabes, the most frequent of the systemic 
 degenerations of the spinal cord, the so-called gray degeneration of the 
 posterior columns and the posterior roots. This degeneration in general, 
 must be considered as progressive and incurable. It offers a great mass of 
 classic clinical symptoms, among which those of the motor sphere, with the 
 exception of ataxia, occupy a very insignificant position. On the other hand, 
 the subjective and objective disturbances of sensation — and, indeed, of 
 almost every quality thereof — those of the reflexes, of the sensory and trophic- 
 functions, play the main role. 
 
 The (Etiology of tabes is — at least in the way the question is usually formu- 
 lated — to be regarded as sufficiently clear to us to-day. We do not know 
 the "excitant factor of tabes," if there is such a one, but we do know that 
 the disease almost always appears only in people who have had syphilis at 
 one time. In the great majority of cases it is a meta-syphilitic disease. 
 This states the main fact about its aetiology; we must, however, enter in 
 somewhat more detail upon the theme, that has been the subject of ardent 
 dispute for decades. 
 
 The ideal proof of the connection between syphilis and tabes would be 
 established, if we could only succeed in proving, the same excitant clinically 
 in both diseases. We may consider, to-day, as the cause of syphilis, the 
 Schaudinn-Hoffmann spirochaeta pallida; but it has not yet been possible to 
 find in the cerebro-spinal fluid withdrawn by lumbar puncture or in the 
 central nervous system of (pure) tabetics, spirochetes (this, of course, 
 because of the nature of tabes as a metasyphilitic disease, was a priori to be 
 
2 4 o DISEASES OF THE SPINAL CORD 
 
 supposed improbable). [Very recently the spirochsetes have rarely been 
 found.] The proof of the presence of syphilitic anti-bodies in tabetics 
 (Wassermann-Plaut reaction) has indeed recently been successfully shown 
 with comparative constancy (Citron), but, in the first place, there is as 
 yet but little clinical material and, secondly, we must lay emphasis on 
 the fact that the reaction (which strikingly appeared rather infrequently in 
 the cerebro-spinal fluid, and mostly in the serum) proved that the tabetic 
 very probably (even when he denies it) has at some time had syphilis, but 
 does not at all prove that tabes depends upon syphilis, since most non- 
 tabetics, who had been infected with syphilis, similarly showed anti-bodies. 
 Besides these direct methods, pathological anatomy also fails, as it, as we 
 shall see below, shows the lack of really specific changes (in so far as the 
 word "specific" may be used here) in tabes. 
 
 For the present, therefore, the statistical method of the proof of the exist- 
 ence of syphilis remains the most serviceable for our purposes. And this 
 teaches clearly and indubitably that 
 
 i. Almost all tabetics have at one time had syphilis, whereby of course 
 an exact anamnesis (or circumstantial evidence in certain cases) must 
 often suffice as proof. The largest existing table of statistics, collected by 
 Erb (1904: 1 100 cases) gives about 90% of syphilitically infected tabetics; 
 the French writers ( Fournier, Dejerine, Belugon and Fame) 77 to 97% of 
 former syphilitics, Striimpell 90%, Gowers 75 to 80%, Negro 89%, Homen 
 83 %, etc. The number of authors crediting only a small percentage to 
 syphilis (Storbeck and Gutmann), grows constantly smaller and smaller, and 
 we may accept the fact to-day as established, that approximately 90% of all 
 tabetics were formerly infected with syphilis. All "proofs to the contrary" 
 constructed partly at least artificially, serve in objective judgment only to 
 confirm these results. Of 10,000 non-tabetic patients, only 21.5% had 
 syphilis in their anamnesis (Erb). The fact that of all syphilitics only 
 comparatively few get tabes, proves that tabes is a rare, and not a frequent 
 sequela of syphilis. 
 
 For the anamnestic proof of syphilis, it is of course essential, that the 
 physician knows "how to ask questions." The unpleasant fact of a past 
 experience with syphilis is an admission that nobody cares to make; very 
 many patients (especially of the lower classes, women, etc.), are not even 
 acquainted with the fact of having had syphilis or have forgotten it, either 
 because it was but slightly treated or not at all (this appears with extra- 
 ordinary frequency in the case of those who become tabetics later or because 
 its course was brief). Then one must seek for indications: eruptions, swell- 
 ing of the glands, infections of members of the family, abortions in the wife 
 or sterility, relapsing cerebral paralyses, etc., in the history, leukoderma, 
 plaques in the mouth, scars of all sorts, in rare cases also for typical tertiary 
 diseases, gummata, etc., in the physical examination. In tabes of children 
 
CAUSATION OF TABES 241 
 
 (rare) one must be on the watch for extragenital infection or for congenital 
 syphilis. The search succeeds more easily with men than with women, and 
 more easily in the upper than in the lower classes. Certain occupations, 
 which seem peculiarly to increase this liability to the acquisition of syphilis, 
 exhibit the most tabetics (merchants, travellers, actors, officers), others, 
 correspondingly the fewest (clergymen). The history of the individual 
 peculiarities of the infection {only hard chancres, eruptions, condylomata, 
 etc.), varies considerably; in general, it seems, as has been remarked above, 
 that the majority of apparently slight and but little treated cases lead to future 
 tabes. The time of the appearance of tabes after syphilis is also somewhat 
 variable, but it appears scarcely ever earlier than 5 or later than 20 years after 
 the infection; in late infection, also, the outbreak of the spinal cord disease 
 can be established, occasionally, only close to old age. Tabetics are, more- 
 over, nearly always immune to syphilis. Tabetic couples, i. e., when husband 
 and wife, one after the other, are attacked by tabes, are syphilitic, almost 
 without exception {Fischler). Why in certain races which are at least in 
 part fairly saturated with syphilis, tabes appears but comparatively seldom, 
 has not yet been completely explained (cf. below). (Tabes was never seen 
 in the American Negro until recent years. To-day it, and paresis, are 
 becoming common. Burr.) 
 
 2. The remaining ^etiological possibilities worthy of any consideration — 
 compared with syphilis — are of but slight moment. In this matter, however, 
 the statistics are unfortunately but slightly satisfying, since only a few writers 
 have objectively considered all such possibilities with complete impartiality. 
 Erb has done this most thoroughly (cf. below). As to these remaining 
 factors, it appears that the poisons and infections that are worth mentioning, 
 outside of syphilis (mercury, alcohol, tobacco, influenza, gonorrhoea) with 
 the exception of gonorrhoea alone play an insignificant role; according to 
 Erb, 90% of the tabetics had gonorrhoea; but for the most part they were 
 those, who at some time also had suffered from syphilis. Sexual excesses, in 
 so far as can be ascertained, also play a small part; on the other hand, 
 traumatism may give rise to tabes under certain conditions. " Catching cold," 
 on the contrary, the "rheumatism" in the anamnesia, is, in a disease, that 
 begins so frequently with pulling and tearing pains, to be regarded skeptically 
 and actually can be but seldom demonstrated on closer investigation. The 
 neuropathic disposition, the "ectodermal weakness of the germinal layer" 
 {Bittorf) doubtless co-operates, though we must note, that genuine signs of 
 physical degeneration in the patients are, in our experience, of less frequent 
 and regular occurrence, than nervousness in the family. Finally, we must 
 mention the theory of tabes as the "exhaustion disease" (Aufbrauchkrank- 
 heit) of the nervous system {Edinger) . In physical overexertion, particularly 
 where the activity of the individual is limited to one side of the body, there 
 takes place according to Edinger an early using up of the nerve substance 
 16 
 
242 DISEASES OF THE SPINAL CORD 
 
 which may develop into systemic degeneration of the tracts concerned. This 
 holds good, incidentally, for nervous regions, weakened by other kinds of 
 diseases, poisons, etc., and thus especially for the spinal cord of tabetics, 
 which has been influenced by syphilis. The hypothesis is quite illuminating, 
 and holds good for many tabetic symptoms (thus, for instance, after extra- 
 ordinary exertion of the legs, as in the case of Marathon runners, it happens 
 not infrequently, that the patellar reflexes do disappear, but they remain 
 absent for a long time or even permanently, only in those individuals who 
 have had syphilis) , but the theory does not explain, why, among the countless 
 individuals who have had syphilis, and who have frequently undergone one- 
 sided physical over-exertion, tabes does not appear far more frequently than 
 actually happens. 
 
 Only Erb, as we have said, has made, exact investigations of the condi- 
 tions having a possible aetiological relation to tabes, which are worthy of con- 
 sideration and has put them together in tabulated form. It appears, natu- 
 rally, that very frequently several aetiological factors co-operate; but if one 
 counts only the cases, in which only one aetiological factor was proved to be 
 possible (with the high percentage of syphilis in anamnesia, this number 
 is naturally small) one finds in cases of 
 
 Syphilis alone 27.0% 
 
 Neuropathic heredity alone 0.7% 
 
 Cold alone i-4% 
 
 Over-exertion alone 0.3% 
 
 Sexual excesses alone ■. 1 .0% 
 
 Traumatism alone 0.3% 
 
 As to the manner of the working of all these evil influences, we have 
 unfortunately, up to now, only conjectures; the majority, it is probable, 
 furnish only the exciting factor for the development of the disease. But 
 how the spinal cord becomes, primarily, less resistant or more, receptive to 
 the exciting stimulus, we do not know. This question is most important 
 as to syphilis: Is it only the most frequent "agent provocateur," or the 
 real cause of tabes? Many observations speak for the latter view. I must 
 mention also the hypothesis of French authors of a special "nerve syphilis," 
 a "syphilis a virus nerveux," which causes but slight phenomena to appear 
 on the skin, mucous membranes, etc., outside of the primary chancre, but 
 lays the stress of its dire .work to the destruction of the nerve elements, 
 upon which is based the future tabes or paralysis. Many striking phenom- 
 ena, as the occurrence of tabes in married couples, the frequency of cases 
 of tabes among syphilitics whose infection came from the same source 
 (Brosius) might thus be explained. 
 
 Another, at first sight amazing hypothesis (Loewenthal) , assumes that 
 not syphilis itself or its toxins, but the protective substances (anti-bodies) 
 
THE HISTOLOGICAL ANATOMY OF TABES 243 
 
 produced years after the infection, cause tabes. This, in any case, ex- 
 tremely doubtful hypothesis is to be merely mentioned here; it proves only, 
 as does all that has been said above, that though we know that syphilis is the 
 main factor in the (Etiology of tabes, we do not know the manner of its 
 working. 
 
 If we turn to the pathological anatomy of tabes, things are simple enough 
 so far as regards histology, more complicated as to the distribution of the 
 process in the central nervous system, and extraordinarily complicated and 
 difficult to explain so far as pathogenesis is concerned. 
 
 If we summarize our present knowledge on these points, we may say: 
 
 Tabes is a degenerative disease, characterized by progressive destruction of the 
 nerve fibres and loss of the medullated sheaths as well as by a secondary increase of 
 the glia — -so-called gray degeneration. It is located in the posterior roots and 
 the posterior columns — -first in the column of Burdach, then in GolVs column — and 
 in a few less constant points. It begins probably with a specific syphilitic inflam- 
 mation {in the broadest sense) in the posterior roots and a chronic slight menin- 
 gitis, which — segment after segment being affected — gradually causes the tracts 
 of the central organ co-ncerned to degenerate. We shall try 7 to prove these 
 statements in the following. 
 
 The histological process may almost be described as banal, so typically 
 does it repeat itself in the majority of cases. "The nerve fibres degenerate, 
 lose their medullated sheaths in a more or less irregular way, are in part 
 thickened and swollen, later atrophy, together with the axis cylinders, and 
 finally disappear altogether. The proliferated glia appears as a delicate 
 fibrous network, in which, frequently, the gaps left by the destruction of the 
 nerve fibres are still visible; later it becomes transformed into a delicately 
 waved, striped, tough mesh, in which nuclei and neuroglia cells, as well as 
 corpora amylacea are present in great numbers. The vessels are but slightly 
 changed; have thickened and hyaline walls, narrowed lumen and here and 
 there a somewhat richer intercalation of nuclei. In quickly progressing 
 recent cases granular cells have also been found (Erb)." 
 
 Changes quite analogous to these are found in the posterior roots, and 
 especially in the part that lies between the entrance into the dura and the 
 spinal ganglion, which French writers call the "nerf radiculaire, " since 
 Nageotte wrote about it, and here and there, but not constantly in the spinal 
 ganglia themselves. This division of the roots shows interstitial neuritis, 
 and later, degeneration; according to the most recent investigations, there 
 appear, in the beginning, regenerative processes also (Nageotte, Marinesco), 
 especially in the form of "collateral regeneration, " but the process always 
 ends in total destruction of the fibres. Finally, the meninges are frequently 
 also — constantly according to French writers — affected by diffuse prolifera- 
 tion of the cells, especially in the walls of the veins and the arteries of the 
 meninges, which, according to Nageotte and others, is identical with the 
 
244 DISEASES OF TEE SPINAL CORD 
 
 changes in the vicinity of the vessels, observed in genuine syphilitic processes 
 (these cells are also found in large quantities in the cerebro-spinal fluid of 
 tabetics) . The slight meningitis — in contradistinction to which there exists a 
 rarer coarse, chronic meningitis, with thickening and adhesions— occurs in the 
 vicinity of the posterior columns and the points of entry 7 of the posterior roots. 
 It is seldom that the anterior roots show a similar degeneration; on the other 
 hand, we find frequently, in addition to the changes in the fibres, degeneration 
 in the ganglion cells, and especially in those of Clarke's columns, rarely, also 
 in those of the anterior horns (the latter only in isolated forms of tabes with 
 amyotrophy, arranged in nests or foci, Lapinsky). Occasionally, also, 
 the degeneration affects peripheral motor and sensory nerves, but almost only 
 in the cases, in which, clinically also, the simultaneous presence of neuritis, 
 on account of existing pareses, etc., may be recognized. 
 
 It must be emphasized, however, that the changes in the "nerf radicu- 
 laire" and the meninges, in the form described, are an outcome of French 
 investigations of recent years, and are still awaiting full confirmation; but 
 they have been found with such constancy by these investigators, that, 
 considering their importance for pathogenesis, we are justified in regarding 
 them as peculiarly significant. 
 
 Macroscopically a tabetic spinal cord can often be easily recognized 
 by the small volume, the "disappearance" of the posterior columns; further- 
 more the portion of the posterior columns affected is characterized by the 
 easily recognized gray discoloration, not only when the cord is intact but 
 more particularly on cross-section. 
 
 These are the real tabetic changes. Besides these, we find, not very 
 rarely pathological pictures which belong to other diseases of the central 
 nervous system: combined diseases of the columns (with participation of 
 the lateral cerebellar tracts, the pyramidal tracts, etc., cf. below), pro- 
 liferations in the molecular layer of the cerebellum, formation of cavities 
 (combination with syringomyelia), and genuine gummatous, syphilitic 
 processes of the meninges, of the substance of the brain and spinal cord; 
 finally the frequent combination with progressive paralysis, even from an 
 anatomic standpoint. 
 
 How, then, does the tabetic process spread? 
 
 In part the localization has been already mentioned: the membranes, 
 posterior roots, posterior columns. In detail, we can start from the degen- 
 eration of the posterior roots, even when they do not show the earliest and 
 most marked appearance of the process, as occasionally happens. But if 
 we assume that the process starts regularly from the posterior roots (cf. 
 below), we should have to see the degeneration of the fibres of the posterior 
 roots pass over into the posterior columns, and, according to the level of the 
 spinal cord, affecting more either the columns of Burdach or those of Goll; 
 it would have to rise, in part, to the nuclei of the posterior columns in the 
 
MORBID ANATOMY OF TABES 245 
 
 medulla oblongata, and, for the rest, pass over into Clarke's columns of the 
 posterior horn and the Lissauer marginal zone. Also the descending fibres 
 in the posterior column would have to undergo degeneration. Then, 
 surely, the process could often be traced up to the spinal ganglion on one 
 side, up to the cortex of the cerebellum, into the inner capsule and to the 
 sensory centers of the cerebral cortex. 
 
 This actually corresponds to the course of the disease in the majority 
 of typical cases, with the one restriction mentioned, that the first 
 clearly visible changes, appear, as a rule, not in the posterior roots, 
 but in the posterior columns. In fact the first change observable in the 
 lumbar segment is a symmetric field of degeneration in the columns of 
 Burdach, laterally next to the posterior horn, the very place of the "bande- 
 lettes externes," the entry zone of the roots. This is the typical condition 
 at the initial stage of the disease. Thence the process continues upwards. 
 Corresponding to the course of the columns of Burdach, with the degene- 
 ration of every higher segment, the disease moves more towards the midline, 
 finally into the columns of Goll. The higher we go in the spinal cord, 
 the more clearly are the columns of Goll affected (in regular sequential 
 succumbing of the individual segments), so that we can lay down this 
 rule: in the lumbar segment, first the columns of Burdach, then the 
 total cross-section of the posterior columns are attacked; in the dorsal and 
 cervical segments, first the columns of Goll, only later the entire cross- 
 section; in the medulla, the nuclei of the posterior columns in the vicinity 
 of the calamus scriptorius. As a rule, the Lissauer marginal zone and 
 Clarke's columns of the posterior horns, are affected at the same time, the 
 latter, occasionally, in such a way that the cells remain intact, whereas the 
 fibres degenerate. The posterior roots themselves are also always affected, 
 even if not always apparently to the same extent, as the posterior columns, 
 so that many authors assume an "elective disturbance" of the root fibres. 
 Very often we find cell degeneration in the spinal ganglia, even if, and this 
 must be emphasized, not absolutely constant. On the other hand, in the 
 lumbar segment, the dorso-medial bundle, and farther up, the so-called 
 ventral posterior column field, are almost invariably spared. 
 
 Concerning the participation of the meninges, which may be described 
 as rather constant, we have spoken above under histology. Frequently 
 we find also affected the reflex collaterals, radiating into the anterior horn, 
 in the rare cases of combination with spinal muscular atrophy, also the 
 ganglion cells in the anterior horn. Not rarely, the gray degeneration 
 attacks also Gower's bundle and the lateral cerebellar tracts, but seldom, 
 on the other hand, does it affect the pyramidal tracts. In the cortex of the 
 cerebrum and of the cerebellum we occasionally find similarly degenerated 
 fibres. The peripheral sensory fibres (rarely the motor ones) show, at times, 
 a degenerative process, which can not, however, compare in severity with 
 
246 DISEASES OF THE SPINAL CORD 
 
 the columnar degeneration. Very much more frequently, however, are the 
 cerebral nerves attacked, and especially, and almost regularly, the opticus, 
 in the sense of a primary parenchymatous degeneration, possibly omitting a 
 small bundle (Marie and Z,m), and this degeneration seems to be quanti- 
 tatively independent of the simultaneously occurring disappearance of 
 ganglion cells in the retina. Next in frequency the abducens, the oculomotor 
 and trochlear nerves are affected, and, far more rarely, the auditory, olfac- 
 tory, trigeminal. Positive findings in the sympathetic have been strikingly 
 rare (cf. below). 
 
 In contradistinction to these more or less regular systemic degenerations 
 in the normal course of tabes stand some apparently irregular cases, in which, 
 for instance, there are scarcely any changes in the lumbar segments, but seri- 
 ous ones in the posterior columns of the cervical region (for instance, in so- 
 called tabes superior, only in the columns of Burdach of the cervical segments) , 
 or where spots of degeneration are scattered arbitrarily, as it seems, over 
 the posterior columns. If we follow up these cases, we find almost invariably 
 that the cause is an irregular sporadic lesion of individual posterior roots, 
 individual segments, which can even, apparently, be confined to one segment 
 only. From all this it appears with considerable definiteness, that in tabes 
 we have a segmentary, progressive, normally ascending disease of the posterior 
 root regions, that is, a lesion of the posterior columns of a radicular type (Erb) . 
 Upon this pathologic-anatomic fact, Nageotte and other French authors 
 base what is, at present, the most plausible theory of the pathogenesis of tabes, 
 into which we shall enter briefly. 
 
 According to^Nageotte, the process is as follows : At first, there develops 
 a diffuse leptomeningitis, which, because of the small-celled infiltration of the 
 pia and the changes in the vessels, which are supposed to strongly resemble 
 syphilitic changes, he regards as specific-syphilitic. Serious local changes, 
 adhesions, etc., do not arise from this (cf. above); on the other hand, there 
 soon occurs a root neuritis (cf . above, a neuritis of the part between the dura 
 and the spinal ganglion). Hence the degeneration occasionally extends to 
 the spinal ganglion, but always the entry zone of the roots and the posterior 
 columns, and is even, at times, most clearly visible in the posterior columns, 
 since, according to Nageotte, the collaterals or the so-called short root fibres 
 may degenerate first at the distal end. That from the meninges, the nerf 
 radiculaire is affected with special ease, is explained by the indubitable 
 fact, that lymphatic channels pass constantly along the posterior roots, which 
 can bring the supply of syphilitic and other poisons to bear with peculiar 
 intensity upon the restricted space. 
 
 This theory, doubtlessly, meets most of the demands. It has only two 
 weak points. One, that the syphilitic nature of the meningitis has thus far 
 not been established, since we simply do not know any changes, which must 
 be regarded as certainly syphilitic and which can be proven such experiment- 
 
PATHOGENESIS OF TABES. SYMPTOMS 247 
 
 ally; second, that up to the present time, there have been too few special 
 anatomic investigations of the nerf radiculaire. 
 
 The assumption of a primary disease of the spinal ganglia {Marie and 
 others) is weakened by the fact that the changes demonstrated are too 
 inconstant; nor is the exceedingly different participation of the incoming and 
 outgoing nerves favorable to the theory. 
 
 Likewise the assumption of a diffuse toxic influence acting on the poste- 
 rior columns through the poison circulating in their lymphatic channels, 
 coming from a meningitis "posterior" luetica {Marie and Guillain) is a 
 mere hypothesis. 
 
 Ley den and Goldscheider believe the process to have its beginning in the 
 peripheral sensory nerves, but they are too rarely found affected to make 
 this supposition probable. 
 
 The possibility that a primary toxic disease of the intramedullary root 
 tracts exists at the outset, which is the theory of Striimpell and others, is, on 
 the other hand, not to be set aside lightly. 
 
 To what extent the results of the investigation of the cerebrospinal fluid 
 have been favorable to the one or the other hypothesis, we shall see below. 
 
 Now the question arises; are these pathologic-anatomic processes, which 
 have been described, of syphilitic origin or not? It is impossible for us, at this 
 time, to answer either in the affirmative or the negative. The reason for this 
 is, that the means for certain diagnosis of a specific syphilitic change, is as yet 
 denied us, so that it has been possible, in recent times, to doubt even the 
 specific character of the genuine gummatous changes. So long as we can not 
 isolate the excitant of syphilis, or its toxins from the regions affected, or 
 experimentally evoke a typical tabes, by the use of syphilitic matter, we shall 
 have no positive proof. To-day we can only say, that, according to the status 
 of our present knowledge, it is possible, but that it has thus far not been 
 proven, that the changes of the nervous system found in tabes are with regard 
 to the pathological anatomy of a specific syphilitic character. But the 
 observations, attesting the correctness of this supposition, are multiplying. 
 The discussion of the specific tabetic anatomic changes in the rest of the body 
 (arthropathies, etc.), will follow their mention under symptomatology. 
 
 The clinical symptoms of tabes are unusually varied. Their distribu- 
 tion into stages of the disease (initial or preataxic stage, ataxic stage, para- 
 lytic or final stage) is correct only in isolated cases, because of the differences 
 in the course of the disease, and is subject to the point of view of the observer. 
 Most easy to demarcate is the initial stage, the duration of which, however, 
 may vary from a few months, or even weeks to 22 years (mentioned by Erb). 
 But it is characterized by symptoms which belong regularly to this stage. 
 
 Since it is usually only by chance that the diagnosis of tabes is made in 
 this early stage, the possibility of its demarcation depends often upon the 
 statements of the patient, who has already reached the later " recognizable ' ' 
 
248 DISEASES OF TEE SPINAL CORD 
 
 stages of the disease. The most constant complaint of these patients to 
 the physician is, " I have had rheumatic pains for so many years" (also often, 
 alas, in the form "my doctor has been treating me for years for "rheu- 
 matism"). This "rheumatism" is with tabetics the almost invariable, decep- 
 tive designation of the lancinating pains, which as a matter of fact are most 
 commonly the first symptom of the disease. Almost all tabetics give the 
 same description of this disturbance: lightning-like shooting pains, lasting 
 from an instant to several seconds — very seldom of longer duration — which 
 are unusually violent, so that the patients often scream aloud. As a rule, 
 these pains do not appear regularly distributed over long periods of time, 
 but rather massed, after exertions, excesses, psychic irritations, exposure to 
 cold, change in the weather, occasionally, too, without any apparent cause. 
 They usually follow the single nerve trunks and the localization of the disease 
 in the spinal cord and are usually, therefore, observed first in the legs 
 (sciatic, crural), only later in the arms and the trunk, very seldom in the 
 head. By no means, however, do the pains always recur in the same nerve, 
 but occur within the same exacerbation period in all possible regions attacked; 
 but the contrary also takes place at times, and in these latter cases there is 
 often hyperesthesia to touch, pressure, etc., in the regions concerned. The 
 lancinating pains are present in 90% of all tabetics (Erb) and not infrequently 
 characterize the entire picture. There are patients, who, through these 
 terrible pains alone— having very few other symptoms — are completely 
 deprived of their ability to work and their joy in life and finally commit 
 suicide. Very many more, even when these pains finally disappear after 
 many years, have in the meantime become morphine victims. It is remark- 
 able that besides the crises (cf. below) and these peculiar lightning-like 
 pains, other pain is not frequent in tabes. 
 
 Paresthesias, the subjective sensory disturbances, are considered as the 
 next early symptom. Many tabetics complain of their feet "falling asleep," 
 "feeling prickly," of a sensation of "ants crawling over their feet," and, 
 moreover, this disturbance (not taking into consideration the rare cases of 
 tabes superior, cf. above) is frequently a typically ascending one. It begins 
 in the tips of the toes, separate toes become "dead," then the soles become 
 paresthetic, or the one margin of the foot, or the entire foot. The feet are 
 continually cold or warm. Then the paresthesias become stocking-like, 
 the feeling of a band about the knee arises, which develops later and higher, 
 as the " girdle feeling" in the trunk, to one of the classic tabetic paresthesias, 
 which often appears as an early symptom. The ascending type, which 
 appears also in myelitis, is occasionally lacking; the patients complain of 
 their forearms going to sleep (ulnaris sensation), of the same feeling in the 
 rectum, scrotum, etc. A feeling of heaviness in the legs is to be interpreted 
 partly as a pure paresthesia, partly as a genuine diminution of muscular 
 strength. This also is very frequent in the initial stage as subjective dis- 
 
SYMPTOMATOLOGY OF TABES 249 
 
 turbance. The patients do not like to take long walks; they tire quickly. 
 At this time also there usually appear disturbances in the sphincters and of 
 the sexual function. In the beginning, disturbances of the bowels, because 
 of the great frequency of bowel sluggishness, are rarely of much value 
 {incontinentia alvi can scarcely occur as an early symptom); on the other 
 hand, one often hears that the patients have to urinate more frequently, 
 that they can not properly hold their urine, or (more seldom) that they have 
 to exert strong pressure in urination (incontinentia vel retentio urinse). 
 Male patients complain almost invariably (80%), and usually even in the 
 early stage, of impotence in the widest sense; as a rule, at first, of insufficient 
 erections, premature or insufficient ejaculation; the loss of the libido, to the 
 torture of the patient, usually follows only later. In women, similar changes 
 seem to take place. Frequently the stage of impotence is preceded by one of 
 increased libido, occasionally, too, of increased sexual capacity. 
 
 As the last preataxic symptom of a subjective nature, we note disturbances 
 affecting the eyes; most frequent is diplopia which often appears first as 
 "seeing dimly," as a confused picture, etc. Occasionally it persists as a 
 constant symptom, but it usually disappears, yielding to other phenomena; 
 it frequently, however, returns. Other patients complain first of a decrease 
 in the vision, which affects usually one eye at the start, later becomes bi- 
 lateral, and may lead to complete amaurosis. There are tabetics, who have 
 absolutely no symptoms, as long as they live, except total blindness and 
 possibly disturbances in the tendon reflexes. 
 
 For these ocular phenomena, we find even in the early stage, objective 
 foundations; disturbances in the muscles of the eyes and in the opticus, as 
 well as pupillary rigidity are present. Corresponding to the diplopia just 
 mentioned pareses of the abducens (most frequent) , oculomotor (ptosis also 
 very frequent) and trochlear are often temporary in the beginning, their 
 intensity changing in the course of weeks, even of days; they may disappear 
 completely and never return. But often in the later stages there develop 
 permanent, usually total, paralyses; we find ophthalmoplegia interna (paral- 
 ysis of all the inner muscles of the eye), externa and total ophthalmoplegia, 
 in which, back of the sunken lid, the ball usually slightly turned inwards 
 and downwards is fixed immovably (Fig. 59). 
 
 Changes in the optic nerve are frequent in the ophthalmoscopic picture, 
 but the findings are fortunately not always parallel to the actual visual 
 disturbance. The disc is more or less grayish-white, in later stages often 
 discolored to a porcelain white, thus marking the presence of atrophy of the 
 optic nerve; the vessels of the disc are contracted, its margin marked with 
 unusual sharpness; the retina is, as a rule, unchanged. In cases, in which 
 the discoloration from gray to white proceeds very rapidly, there appears also 
 a progressive loss of vision, finally leading to blindness; on the other hand, 
 however, one sometimes finds in tabetics, who complain only of slight visual 
 
250 
 
 DISEASES OF THE SPINAL CORD 
 
 disturbances, pure white discs in the final stage. Characteristic is the in- 
 creasing contraction of the visual field, especially for colors, which may lead 
 to a partial or total color blindness long before the appearance of total 
 amaurosis. 
 
 By far the most frequent of all changes in the eyes, however, is pupillary 
 rigidity, the Argyll-Robertson phenomenon; it appears in almost all instances 
 even in the early stage and is associated with sluggishness of reflexes. 
 Each eye is tested separately by allowing daylight or concentrated light to 
 fall on the previously covered eye; the pupil contracts slowly, sometimes 
 irregularly, often not at all. Next we allow the patient to converge, or to 
 
 Fig. 59. — Total paralysis of the oculomotor on both sides. Attempt to raise the lids 
 (to knit the brows) prevented by ptosis. 
 
 accommodate for distance; in both cases a distinct contraction is visible. 
 Therefore, there is pupillary rigidity to the stimulus of light, whereas the 
 convergence and accommodation reactions are retained. This is the rule, 
 occasionally even the only early symptom in tabes. As a rule the pupils are 
 usually of unequal width in the beginning (anisocoria) , one usually partly 
 dilated or mydriatic, the other in many cases and at a very early date con- 
 siderably narrowed (to the size of a pinhead) ; not seldom they are no longer 
 round, but elliptic or angular (without the presence of posterior synechia). 
 Also the dilatation reflex of the pupil by means of sensory stimuli (pricking 
 the skin of the cheek), is absent usually in advanced cases. 
 
SYMPTOMATOLOGY OF TABES 251 
 
 We mentioned above the subjective sensory disturbances of the early stage. 
 One of them is, as a rule, even now the expression of an objective disturbance : 
 the insufficient feeling of the patients relative to what is beneath their feet. 
 They do not know whether they walk on carpets or boards, on clay or stone; 
 they have the feeling of "walking on velvet," etc. Then we find mostly 
 a dulling of tactile sensations, probably also of the pressure sense, whether it 
 be in the plantar surface, in the outer or inner margin of the foot, or in the 
 toes. Also hypalgesia and analgesia occur, and, likewise, we find early a 
 slight degree of hyperesthesia to cold in the trunk, which may be subjec- 
 tively represented by the statement, that washing with cold water, to which 
 the patient has been formerly accustomed, has become unpleasant or 
 even painful. 
 
 As the last of the actual early symptoms, I mention the changes in the 
 tendon reflexes. Only very seldom do we miss in the initial stage a weakening 
 or absence of one or more tendon reflexes. Of course, to establish this, it is 
 invariably necessary to carefully test all the more important tendon reflexes 
 of the body, above all — after the patellar reflex — the Achilles tendon and 
 the triceps reflexes. Absolute relaxation of the muscles and joints con- 
 cerned in the test is the necessary condition, before one can speak of a 
 reflex being absent (for the methods of testing the reflexes cf. under "gen- 
 eral diagnostics"); at every such examination the Jendrassik or other 
 similarly valuable method for the purpose of facilitating and increasing 
 the relaxation must be employed. If these precautionary measures are 
 employed the earliest symptom in tabes is found to be a difference between 
 two symmetric tendon reflexes, the one being usually normal (scarcely 
 ever exaggerated), the other diminished. And, moreover, this diminution, 
 which quickly increases to a "total absence" of the reflex, by no means 
 always affects the patellar reflexes first (Westphal's phenomenon), but very 
 often the Achilles tendon reflex, or in tabes superior the triceps reflex. 
 
 If we mention, furthermore, the occasional appearance in the beginning 
 stage of the Romberg phenomenon, to be discussed at greater length below, 
 we shall have mentioned in a nutshell the usual early symptoms of tabes, to 
 which other authors (among them, Erb) it is true append a long list of others. 
 Meanwhile, the borderline is always more or less arbitrary, unless importance 
 is attached to the appearance of ataxia, which is often absent at this early 
 stage. The initial stage often passes unnoticed into the fully developed 
 disease, which we must now discuss, connecting the ataxic with the so-called 
 final stage. 
 
 As mentioned above the pains may accompany the unfortunate patient 
 through the entire course of the disease. They may become less frequent at 
 the height of the fully developed tabes, but they scarcely ever disappear 
 entirely and frequently, as "tabes dolorosa" form, at least subjectively, the 
 most prominent symptom. But in additon to the lancinating pains, the radius 
 
252 DISEASES OF THE SPINAL CORD 
 
 of action of which naturally increases with the progress of the disease, and 
 which to-day attack one part of the body of the patient, tomorrow, another, 
 we meet also with an entirely different kind of pains — the crises. 
 
 The term crisis includes painful sensations of acertain "degree," mostly 
 also of a somewhat longer duration, which almost invariably proceed from 
 organs, or are at least localized in them, the innervation of which is derived 
 also from the sympathetic in addition to the cerebral and spinal nerves 
 (especially of the vagus group). This naturally does not mean that these 
 pains are transmitted through the sympathetic — we shall speak below at 
 greater length upon this point. But it is nevertheless striking, that the 
 organs most frequently affected (stomach, bowels, kidneys, etc.) according to 
 the prevailing views (Lenander, L. R. Mutter, and others) and investigations, 
 are susceptible in but a slight degree to sensory stimulations. The first place 
 is beyond a doubt to be assigned to the stomach, or gastric crises. 
 
 The typical gastric crisis begins almost without any warning, rarely with 
 slight disturbances of the stomach, loss of appetite, etc. This is followed by 
 unusually violent pains in the epigastrium, which resemble the pains of 
 gastric ulcer, but may become more colic-like; they are mostly described as 
 tearing, jumping, boring. Almost simultaneously, the patient suffers with 
 uncontrollable vomiting, first of food, then of mucus, which is mostly 
 stained with bile, and usually shows but slight acidity. The patients vomit 
 then throughout the duration of the crisis — usually for a few hours, but also 
 with interruptions for days and weeks — literally all the nourishment they 
 take, so that occasionally artificial feeding must be resorted to. In the crises 
 pain and vomiting coincide, but the attack of pain may persist in the intervals 
 between vomiting. The loss of strength of the patient is enormous during 
 this time, but as recovery from the crises, which always end suddenly, is just 
 as quick, the conditions are rarely so bad as to menace life. The stomach 
 crises are the predominate features in many cases of tabes and recur every 
 few weeks or months, but, as a rule, only during a period of the disease, which 
 may however last through several years. The majority of tabetics are fortu- 
 nately spared them, but the percentage of those affected is not inconsiderable. 
 
 The other forms of crises occur far less frequently. 
 
 In "intestinal crises" there appear at intervals profuse attacks of diar- 
 rhoea, usually with colic-like pains. 
 
 Attacks simulating renal colic have been described as kidney-crises. 
 Crises of the bladder, urethra, testicles, clitoris, are all analogous conditions, 
 which the patients localize in the organs mentioned. As a rule they are of 
 much shorter duration than the gastric crises; those affecting the genital 
 region are frequently accompanied with intense feelings of libido, as well as 
 with pain. 
 
 Somewhat more frequent are the laryngeal crises. The onset is character- 
 zed by an apoplectiform coughing fit, with spasm of the glottis, which may 
 
SYMPTOMATOLOGY OF TABES 253 
 
 lead to serious attacks of choking, and often to temporary loss of conscious- 
 ness. This form is of all those mentioned, the only one that is directly 
 dangerous to life. Attacks of tachycardia and pseudo-angina pectoris have 
 been described as pure vagus crises, but the interpretation is absolutely 
 uncertain. 
 
 Between the crises and the lancinating pains stand the neuralgias, which 
 are comparatively infrequent but obstinate. These neuralgias may attach 
 themselves to the cerebral and peripheral nerves, in which case they resemble 
 rheumatic neuralgias, or they may affect the internal organs. I observed 
 for years a tabetic, who was tortured by the most violent unceasing enteralgia, 
 which neither increased to the form of crises, nor ever assumed the type of 
 lancinating pains. 
 
 To the subjective sensory disturbances of the later stages correspond more 
 nearly, than in the early stage, objective changes of sensation, which we shall 
 therefore speak of now. Even before the hyperesthesia has completely 
 risen in the legs, there generally appear zones of sensory disturbances in the 
 trunk, which (Hitzig, Laehr, Dejerine) have segmentary arrangement (cf. 
 general diagnostics) , i. e., occupy the region of the skin, supplied by the indi- 
 vidual posterior roots, resp. the segments belonging to them. Sometimes 
 the existence of such zones is suggested by the girdle feeling of the patient 
 (even in the region of the girdle pain the skin may be hyperaesthetic!), but 
 often the girdle zones are absolutely independent, at the height of the breast 
 or the costal arch, the umbilicus, etc.; very often, they are broader on one- 
 half of the trunk than on the other, or are demonstrable only on one side, etc. 
 The form of disturbance is here, usually a tactile hyperaesthesia or anaesthesia 
 with a most evident hyperalgesia, also with diminution of the cold and heat 
 sensations. Very remarkable also is the disturbance of the temperature sense 
 in the trunk in the form of hyperesthesia to cold; it appears usually early in 
 the disease, often in the first stage, and the patient is also subjectively con- 
 scious of it. The tabetic becomes unduly susceptible to cold touches, 
 especially to cold water, but also to other cold objects, etc. (usually less 
 frequently to cool air!); such a touch almost causes him pain, he starts up, 
 screams, etc. This hyperaesthesia to cold does not coincide with the girdle 
 zones, which have just been mentioned, but generally affects the trunk from 
 the costal arch downwards, often also the upper part of the leg, especially 
 on its inner side. In these hyperaesthesias to cold a radicular (segmentary 
 type) cannot be recognized. This disturbance is unusually characteristic 
 for tabes, and is rarely absent even in the rudimentary forms. 
 
 Otherwise hyperaesthesias are scarcely ever met with in tabes. On the 
 contrary in the later stage the loss of sensation to touch ascends the legs, 
 passes over trunk and arms and may even attack face and head. The entire 
 surface of the skin then, or only the surface of the body up to the neck, the 
 breasts, etc., shows diminution of perception to a simple touch and usually 
 
254 DISEASES OF THE SPINAL CORD 
 
 also to cold (with the one exception named), and heat. Total anaesthesia, 
 even to coarser touch, appears only late, and confines itself mostly to isolated 
 parts of the legs. On the other hand, sensibility to pain may be completely 
 lost even at an early stage. Hypalgesia in feet and legs is found almost 
 invariably. In addition to this, there appears very soon a characteristic 
 retardation of the perception of pain stimuli. If the skin be pinched or 
 pricked, the patient feels at first merely a touch, and only after several seconds 
 does the feeling of pain follow, or the corresponding pain reflex, the with- 
 drawal of the foot, etc. To this is often added a disturbance, which peculiarly 
 supplements hypalgesia, the so-called summation of the pain stimulus. A 
 stimulus of only short duration even though it be powerful calls forth no 
 feeling of pain, but a longer pinching, or pressing the point of the needle 
 against the skin, for a definite length of time does. In addition the feeling of 
 pain often lasts for a considerable time afterwards. " Abadie's symptom," 
 insensibility to pain when the Achilles tendon is pressed upon violently, is a 
 very inconstant sign. 
 
 Concerning sensibility of the internal organs in tabes, we know but 
 little. The normally great sensitiveness of the testicles and breasts to 
 pressure disappears (testicular analgesia). The subjective feeling of the 
 need of emptying the bowels and bladder, defecation and micturition, is 
 disturbed early (cf . above) . 
 
 Deep sensibility proper is always more or less influenced. The best 
 known disturbance of this sort is the Romberg phenomenon, generally de- 
 scribed as a disturbance of the muscle sense, which may be demonstrated in 
 90% of all tabetics; when standing with feet close together, and eyes shut, 
 there is so pronounced a swaying of the entire body, that the patient may 
 even fall to the ground, a phenomenon that is scarcely, or not at all, per- 
 ceived, when the eyes are open, that is, when the patient can control his posi- 
 tion with his own eyes. This symptom often belongs to the initial stage of 
 tabes. But the disturbance is by no means sufficiently well explained by the 
 otherwise unfortunate term "disturbance of the muscle sense" (Muskel- 
 sinnstcerung) , for in it participates also the sense for passive movements, for 
 gravity, for resistance, as well as finally for the actual perception of position. 
 
 But these sensory functions may be tested in other ways, too, and show, 
 as has been said before, in tabes, as a rule, great changes in the advanced' 
 stages of the disease. The patients no longer feel how their limbs, toes, etc., 
 are placed, bent, etc., by the examining physician — disturbance of the 
 perception of passive movement; without the control of their eyes, they can 
 not move their feet a definite distance apart — disturbance in perception of 
 active movement; they do not know, where their legs are under the bed- 
 cover — loss of perception of position. In similar manner the senses for 
 weight and resistance can be tested. Naturally, most of these tests prove 
 the presence of mixed disturbances of the different sensory faculties. Pall- 
 
SYMPTOMATOLOGY OF TABES 255 
 
 (Bsihesia or the perception of vibration, which is connected with the sensitive- 
 ness of bones (Egger), is also, as a rule, much disturbed in tabes; according 
 to some authors this proceeds parallel with the ataxia (see below), but 
 according to Egger's latest report this does not seem to be the case. The 
 stereognostic perception, finally, is disturbed more rarely, but occasionally 
 in late stages it is very marked; the patients no longer recognize an object 
 held in the hand, are no longer able to take money from their purses, etc. 
 
 The rather frequent absence in tabes of the normal feeling of fatigue 
 after movements and exertions, is probably of other origin (Frenkel). 
 
 Partly, at least, as a disturbance of the sensory pathways, we may con- 
 ceive the symptom we have not yet discussed and which is responsible for 
 the name given tabes during its entire "second stage": ataxia. Even 
 if there are cases of tabes absolutely without ataxia, this complete freedom 
 from it is very rare; its presence in tabes is extraordinarily significant and 
 characteristic, as nowhere else in human pathology do we meet it in exactly 
 the same form and but rarely in a similar form. If the tabetic — in any 
 position desired— is asked to perform complicated movements, especially 
 with the extremities, there appears an uncertainty, a wavering; the move- 
 ments wander from their direct line and proceed in zig-zag fashion. If the 
 patient is to bring the tips of his index fingers together, by bringing the arms, 
 which have been stretched far to the side, towards the front, this movement 
 is attended by utterly unregulated waverings, extending on all sides about 
 the axis of the movement. He invariably spills water from a full glass 
 that he is to take to his lips; in writing, one letter becomes microscopic- 
 ally small while to form the next, the hand moves the pen all the way across 
 the page. He is not able, when lying on his back, to perform the more 
 delicate movements with his feet, to describe a circle with the points of his 
 foot, to write numbers in the air, to place the heel of one foot, moving it 
 through the air, upon the knee of the other leg. If he is asked to walk upon 
 a line, he finds it very difficult, because his feet continually stray to one side 
 or the other. On the whole the gait of the ataxic is peculiarly characteristic. 
 At first it is swinging and stamping probably with the knee raised unneces- 
 sarily high and toes pointing downwards (like a rooster's walk). Later the 
 legs spread apart, usually assisted by a cane, which is placed far in front, in 
 order thus to attain as broad a base as possible for the three points of support. 
 Even a slight degree of so-called static ataxia is occasionally present, in 
 which the patient finds it less possible to sit or stand perfectly erect, without 
 at the same time moving the muscles of the trunk, but far more frequent 
 is the ataxia, which has just been discussed, the locomotor (or movement) 
 ataxia, which in England and France has given the disease its name. All 
 these atactic movements, moreover, occur even more typically when the 
 eyes are closed, so that, to a certain extent, even the Romberg symptom, 
 just mentioned, may be considered a partial phenomenon of ataxia. 
 
256 DISEASES OF THE SPINAL CORD 
 
 Ataxia is to be understood as a disturbance of co-ordination, the main 
 cause of which must be sought in a confusion of the centripetal stimuli — in 
 the widest sense, therefore, of the sensory stimuli, though the disturbance, 
 at first sight, appears to be a motor one. 
 
 Genuine, purely motor symptoms, however, are very infrequent even 
 in advanced stages of tabes. In the greater number of grave cases of tabes, 
 it is true, the patients are forced to stay in bed, and there is to a high degree, 
 a feeling of general weakness, but this is altogether the result of sensory 
 disturbances and ataxia. To some extent also trophic disturbances prevent 
 motion. Paralyses are known almost only in the area supplied by the 
 cerebral nerves, if we leave out of consideration the rare combinations with 
 atrophic paralyses, described by Dejerine and others, which are probably 
 partly dependent upon implication of the peripheral motor nerves, partly 
 the result of disease of the gray anterior horns, and which, therefore, in 
 neither case, belong to the picture of tabes proper. In these cases, we find 
 conditions of weakness and atrophy, especially in the small muscles of the 
 feet and hands, in the area of the peroneal, as well as in those of the ulnar 
 and median nerves; secondary contractures, the pied bot tabetique (tabetic 
 club foot), claw and monkey hand are the consequences. The reaction of 
 degeneration is not demonstrable in the majority of the cases. But the 
 general emaciation, so frequently observed in the terminal stage of tabes, is 
 only rarely due to these changes, much more frequently it depends upon 
 disturbances in nutrition, and disuse of the muscles (marantic tabes). 
 More frequent are, as has been mentioned, the paralyses of individual 
 cerebral nerves. 
 
 The olfactory is affected only in very rare instances. 
 
 The disturbances of the optic nerve — amblyopia, diminution of the 
 visual field, white atrophy- — have been mentioned; they are not at all 
 common. 
 
 The oculomotor, trochlear and abducent nerves are more frequently and 
 also to a more considerable degree affected than the other nerves; this also 
 has been mentioned above. Outside of the paralyses of individual nerves 
 and the ophthalmoplegias, there occur also accommodation paralyses — 
 scarcely ever isolated — as well as, but rarely, irritation phenomena (con- 
 vergence spasm) which like the club foot in tabes may also be considered 
 as paralytic contractures — possibly with better reason. The appearance, 
 however, of " disturbing" double images, is by no means very frequent, in 
 even the serious forms of tabetic paralyses of the ocular muscles, since the 
 patients are often able to suppress them. 
 
 Disease of the trifacial causes frequently, in advanced cases, more or less 
 severe disturbances of the cutaneous sensibility in the face, particularly in 
 the conjunctival mucous membrane, which especially when associated with 
 lagophthalmos (this is, of course, rare) may lead to the formation of ulcers, 
 
THE SYMPTOMATOLOGY OF TABES 257 
 
 and ophthalmia. The radicular (segmental) type may be recognized here 
 also. Very rarely the motor trifacial is affected causing weakness in the 
 muscles of mastication. 
 
 In the facial, paralyses as well as spasms (tic convulsif) have curiously 
 enough been described very infrequently, but both occur. 
 
 The auditory, again, is more frequently affected and labyrinthine vertigo 
 as well as disturbances in hearing are described, alone and together, that is, 
 disturbances that include part, or the whole of the Meniere symptom com- 
 plex. In the largest statistical paper (Tumpowsky in 225 cases of tabes), a 
 proportion of 0.9% of affections of the auditory nerve was given, a figure 
 which probably is too small, and could certainly be raised by exact otological 
 examination. 
 
 Glosso-pharyngeal Vagus. — Of taste disturbances all varieties are reported, 
 though, on the whole, they are of rare occurrence. Absolute ageusia, loss 
 of the sense of taste, as well as confusion of the separate qualities of taste, 
 occurs. Severely affected tabetics frequently say that everything tastes to 
 them "like wood"; with closer investigation, however, there is proved to be 
 only a partial lowering of the separate qualities of taste. One observation 
 of Pfeifer mentions, on the analogy of the delayed pain sensation, a retarded 
 taste sensation. This group of phenomena, as a whole, belongs to the 
 domain of atypical forms of tabes, especially to those of the bulbar-paralytic 
 type. 
 
 A pure vagus symptom is the not infrequent tachycardia of tabetics; at 
 rest, the pulse has been observed as high as 120. Tachypncea may belong 
 here also. The disturbances of the laryngeus inferior — seldom permanent 
 paralyses, most frequently posticus paralysis, occasionally spasms, now and 
 then the paroxysmal pareses of the laryngeal crises — lead us to the crises 
 that belong almost essentially to the vagus group, and which were discussed 
 above. Here we must emphasize the fact, that, if we wish to consider the 
 gastric crises, etc., as vagus symptoms, we must assume in numerous cases 
 an early vagus affection almost parallel to that of the opticus. I believe, 
 therefore, as has been mentioned already, that part of the crises must cer- 
 tainly be due to affections of the sympathetic nerve. 
 
 Spinal Accessory Symptoms. — Pareses of the cucullaris and the 
 sterno-cleido-mastoid have recently been described by Seiffer; they must 
 belong, at any rate, to the rarities, as do also the affections of the hypo- 
 glossus, which occur occasionally accompanied by atrophy. 
 
 One unusually frequent motor symptom, the loss of muscle tone, we have 
 not as yet spoken of. The reflex inhibitions, normally present in every 
 active muscular innervation, which prevent the movement from going beyond 
 its goal are often lost early in tabes (nearly always in the final stages). There- 
 fore the purposive movements, when the strength is normal (even when 
 ataxia is not present), make an excessive impression, which is specially 
 17 
 
258 DISEASES OF THE SPINAL CORD 
 
 increased by every passive movement. Without any trouble and without 
 any sensation of pain the legs of the patient may be flexed to such a degree 
 as to touch the head; the hyperextensibility of the fingers and wrist often 
 makes it possible to bring the fingers passively into contact with the dorsal 
 surface of the forearm; the leg extended at the knee, with the patient 
 standing upright, is bent backward by the weight of the body (genu recurva- 
 tum). At the same time the muscles, ligaments, joints are anatomically, 
 as a rule, perfectly normal. (Of course trophic changes in the joints may 
 greatly increase the extent of passive movement.) 
 
 Electrical changes in the muscles are naturally to be expected only when 
 peripheral paralyses exist and have been so reported. 
 
 Of the motor irritation phenomena, athetosis is a not very infrequent symp- 
 tom of irritation in advanced tabes, in tabes superior, and in the bulbar- 
 paralytic form; it gives the complete, uni- or bilateral, clearly defined picture 
 of the movement disturbances just as they are observed in other cerebral dis- 
 eases : continuous, slow, worm-like movements, which may lead to apparent 
 dislocations, to overextensions and to quite grotesque positions, especially in 
 the fingers and arms. Contractures and their concomitant symptoms — ex- 
 aggeration of the reflexes, Babinski symptom, etc. — are observed only in the 
 combination of tabes with other spinal troubles. That the crises, especially 
 the laryngeal, represent partly paralyses, partly also spasmodic spastic con- 
 ditions, has been said above. 
 
 Genuine, pure sympathetic symptoms — widening of the palpebral fissure, 
 unilateral blushing and sweating, etc. — have been found only exceptionally 
 in tabes. 
 
 The change of the tendon reflexes, which characterizes even the initial 
 stage of tabes, is practically constant in the ataxic stage. The Achilles and 
 patellar tendon reflexes are those most frequently absent; the patellar reflex 
 can only very rarely be evoked even by artificial reenforcement (Jendrassik). 
 The triceps and the lower jaw reflex are constantly absent in tabes superior 
 but are not necessarily absent even in advanced cases of the usual type, 
 beginning with the legs, since the reflex arcs for the arms and the head may 
 remain undisturbed for a long time. Occasionally, however, the tendon 
 reflexes that have been lost, may return temporarily or permanently, without 
 a cure being effected, as in slight narcosis, after cerebral apoplexies, and 
 upon the advent of other systemic diseases. Occasionally the reappearance 
 seems, symptomatically, to show a certain improvement, especially when 
 there was originally only a difference in the reflexes on the two sides, and then, 
 one disappeared to return again. This, at any rate, does not seem to be 
 such a very rare occurrence. In patients, moreover, in whom the tabes, 
 from the beginning, appeared in the form of a combined systemic disease, a 
 tabo-paralysis, etc., the reflexes can be retained from the first, or even 
 increased. 
 
THE SYMPTOMATOLOGY OF TABES 
 
 2 59 
 
 The cutaneous reflexes are altered very much more rarely, most frequently 
 the plantar. This is lost in most tabetics, as soon as the sensory disturbances 
 in the feet have attained a certain degree; their decrease is parallel, on the 
 whole, with the loss of feeling (which by no means holds good for the tendon 
 reflexes) . The so-called Babinski dorsal toe reflex is never present in pure 
 tabes. The cremaster reflexes, and especially the abdominal reflexes are, on 
 the other hand, only exceptionally affected by the tabetic process, even 
 where zones of anaesthesia exist in the trunk. Whether the activity of the 
 abdominal reflexes, which is not rare, is connected with the hyperesthesia to 
 cold in that region, has not yet been ascertained. 
 
 Barring the pupillary reflex already mentioned, the so-called "internal 
 reflexes" (of swallowing, sneezing, etc.), are disturbed only exceptionally, 
 unless we are willing to accept some of the crises, as the laryngeal crises, etc., 
 as due to reflex overexcitability, a theory which certainly would not hold good 
 for the majority of cases. Formerly several forms of male impotence were 
 explained as due to reflex disturbance (faulty ejaculation). 
 
 The symptoms referable to the urogenital tract in tabes, on the whole, 
 present a mixture of motor, sensory, and reflex disturbances; they have 
 already been discussed in part with the early symptoms. 
 
 In the completely developed clinical picture of the disease, they are 
 rarely ever altogether absent, and the bladder disturbances especially, very 
 frequently are of great importance in the termination of the disease, causing 
 death by ascending pyelonephritis. 
 
 The bladder disturbances are also the most frequent disturbances in this 
 group. The patients must in many cases use a greater amount of strength 
 to empty the bladder, and there usually remains some residual urine — retentio 
 urinae; this is mostly caused by spasm of the sphincter, but occasionally 
 there is also simultaneous weakness of the detrusor. This, however, is not 
 constant; it may even happen in tabes, that, because of lasting spasm of the 
 sphincter, a genuine hypertrophy of the bladder, a so-called "board-bladder" 
 (Balkenblase) may develop with partial hypertrophy of the muscle of the 
 detrusor. The retention may lead to the clinical picture of ischuria-para- 
 doxa : the constantly filled bladder,, unable to empty itself, emits passively, so 
 to say, small quantities continuously, like an overflowing rain-barrel. Nat- 
 urally such patients very often take shelter in the last resort — the catheter; 
 usually the bladder becomes infected from this, sooner or later, so that 
 cystitis in grave cases of tabes is often found as a direct result of this. 
 This, however, is also caused by the opposite condition to retention; namely, 
 incontinence of urine — the relaxation of the sphincter with constant drip- 
 ping of urine. In this condition also, which is almost as frequent as reten- 
 tion, infectious micro-organisms easily find their way through the urethra 
 into the insufficiently closed off bladder. Disturbances of the intestinal 
 sphincters are found less frequently ;. futhermore all proof for the statement 
 
2 6o DISEASES OF THE SPINAL CORD 
 
 that the constipation, which is as frequent in tabes as in other diseases of 
 the central nervous system, is induced by a spasm of the sphincter is lack- 
 ing. It is easier to believe that it is occasionally caused by a chronic 
 paresis of the intestinal muscles, or a genuine ataxia, which, however, 
 frequently seems to affect the sphincters alone. Often patients are 
 troubled by the lack of control over the emission of urine or of evacuation 
 of feces, due to a deeply seated anaesthesia. 
 
 Impotence is one of the most frequent tabetic symptoms of this group, 
 known, as such, even to the layman. Almost all male patients — in women 
 disturbances of libido and orgasm are seldom even observed by the 
 patients themselves — show, in advanced stages of tabes, a decline in virile 
 power, which affects all the factors of this function; erection and ejaculation 
 in particular become insufficient, take place prematurely, or are even pain- 
 ful, whereas the libido, to the torture of the patient, may last for some time 
 after the loss of potency. In the final stage there is generally total im- 
 potence. In the beginning of the disease, on the other hand, the presence 
 of satyriasis may be noted. 
 
 Of the ordinary groups of tabetic symptoms, only the trophic dis- 
 turbances and the changes in the cerebro-spinal fluid remain to be 
 mentioned. 
 
 The trophic disturbances may affect all the tissues of the body, but most 
 characteristically, the epidermis and the skeletal system. The deficient 
 nourishment of the skin is betokened frequently by ulcers and bed-sores; 
 wherever a tight bandage, a set of false teeth, a urine bottle have pressed 
 for some time, ulcers appear, which occasionally become very deep, have but 
 a slight tendency to heal and are nearly always painless. Such an ulcer, 
 which appears in tabetics apparently spontaneously, is the u mal perforant" 
 usually coming on gradually on the under surface of the great toe, or of the 
 ball of the foot back of the great toe, which, as a rule, eats in very deeply, 
 even to the bone, and is difficult to heal. Also an (apparently) spontaneous 
 herpes zoster is often observed in tabetics. Still more striking are the 
 trophic disturbances in the bones and joints, more particularly the arthro- 
 pathies (Fig. 60). Suddenly, without any spontaneous pains, and within a 
 few days, any joint of the body may become swollen; the patient, as a rule, 
 does not notice it until it causes purely mechanical disturbances in motion. 
 Upon objective examination, however, one generally finds serious changes; 
 of^en an enormous distention at the ends of the bones and effusion into the 
 joint. Local heat and redness, as well as pain, are absent. An X-ray 
 examination shows an indistinct clearing up of the shadows of the bones, 
 which, however, retain their sharp contours; the anatomic examination often 
 shows detachment of the periosteum with softening of the parts of the bone 
 beneath, formation of osteophytes, disintegration of small particles of bone, 
 destruction of ligaments and joint cartilage. The exudate is, as a rule, 
 
SYMPTOMATOLOGY OF TABES 261 
 
 serous and but rarely contains pus. The consequences are loose joints, 
 subluxations, etc. ; because of the chronic course and the very slight tendency 
 to heal, permanent deformities often supervene, which, combined with other 
 tabetic symptoms, especially with hypotonia of muscles, may lead to almost 
 grotesque pictures of diseases, as, for instance, to the not infrequent genu 
 recurvatum with synchronous arthropathies. As a matter of fact, the 
 arthropathies are found most frequently in the knee-joints, then come the 
 
 Fig. 60. — Arthropathy of the right knee in tabes dorsalis. 
 
 hip-, the shoulder-, the ankle-joints, etc. In more recent times, their occur- 
 rence in the spinal column has been described with striking frequency; 
 probably the general employment of radioscopy may have contributed to this. 
 To a certain extent the abnormal fragility of the bones in tabes, runs parallel 
 with the arthropathies; even slight exertion leads to complete, though pain- 
 less transverse fractures of the long bones, which, however, show a greater 
 tendency to heal than the arthropathies. Dentists, moreover, frequently 
 observe a strikingly rapid deformation of the jaws, especially of the alveolar 
 processes, with falling out of the teeth; the process resembles absolutely the 
 
262 DISEASES OF THE SPINAL CORD 
 
 atrophy of the senile jaw, but is more rapid and appears earlier. The 
 change often takes place so quickly that sets of false teeth, made to fit the 
 mouth, no longer fit properly at the end of a few months. 
 
 The last symptom which must be included among those that are almost 
 constant in tabes, is the change in the cerebrospinal fluid. If a few cubic 
 centimeters of the liquid are withdrawn, in. the usual way, by lumbar punc- 
 ture, from the spinal canal of a tabetic, and subjected to centrifugalization, 
 there is regularly found in the sediment an increase of the normally rather 
 rare cellular elements, and especially of the lymphocytes, so called because 
 of their likeness to the lymphocytes of the blood; there is usually a very high 
 degree of "lymphocytosis" of the fluid (Widal, Sicard, Schoenborn, Nissl). 
 A similar change is found in pure meningitis, as well as in some other meta- 
 syphilitic diseases of the central nervous system- — progressive paralysis, 
 cerebro-spinal syphilis. The most likely interpretation of this symptom in 
 tabes is a chronic change in the meninges brought about by the former 
 attack of syphilis — a syphilosis of the meninges. The supposition, that the 
 former attack of syphilis in itself causes the lymphocytosis in tabetics — in a 
 certain number of cases similar findings have been made in secondary 
 syphilis — becomes improbable, since upon closer investigation of the 
 meninges in tabes, anatomic changes are now almost regularly found 
 {Nageotte, cf. above). Lymphocytosis is an unusually constant and early 
 symptom of tabes dorsalis. 
 
 There are several groups of symptoms, which combine now and then with 
 those of tabes dorsalis. Among these are the symptoms caused by the 
 participation of those systems of tracts or columns in the spinal cord which, 
 as a rule, are not affected in tabes, especially that of the pyramidal tracts. 
 Then we have before us the complete picture of "combined system disease," 
 usually accompanied by spasticity and exaggerated reflexes. The combina- 
 tion with peripheral neuritis, also, is not infrequent. The most frequent 
 concomitant disease of the nervous system is probably progressive paralysis. 
 But this, as Erb pertinently emphasizes, must not be interpreted, as if tabes, 
 when reaching a certain high level, very frequently passed over into progress- 
 ive paralysis; this, on the contrary, is a relatively rare occurrence. The 
 combination is, in the first place, rather an anatomical one — in autopsies of 
 paretics a gray degeneration of the posterior columns (Westphal) is often 
 found, and since then it has been learned that occasional tabetic symptoms 
 (Romberg, pupillary rigidity, slight disturbances of sensation) may very 
 often be found in paretics upon closer investigation. Whether, in such a 
 case, the tabes was superimposed on a paresis already present, or whether 
 the two developed simultaneously, we are generally unable to decide; either 
 might happen. But tabo-paresis, though a frequent disease picture, is not 
 nearly as common as simple paresis, and especially not nearly so fre- 
 quent as simple tabes. Otherwise, psychic symptoms in tabes are rare, but 
 
A NA TO MIC CHA NGES AND S YMP TOMS 2 63 
 
 combinations with so-called "neuroses," epilepsy or Basedow's disease 
 may occasionally be observed. 
 
 We have already stated that tertiary syphilis may occur with tabes. But 
 in particular we see certain late forms of visceral syphilis in tabes, especially 
 aneurism of the aorta, to such an extent, that Babinski has described 
 a peculiar symptom complex: aneurism of the aorta, with disturbances of 
 the tendon and pupillary reflexes. In the great majority of cases, the 
 aneurism, as well as the not infrequent insufficiency of the aortic valves, 
 might be caused by a syphilitic aortitis. 
 
 The occurrence of chronic gastro-intestinal disturbances and ascending 
 pyelonephritis in tabes, is explained by the foundation for these troubles by 
 the tabetic disturbances of the respective sphincters. 
 
 How far, then, can a pathogenesis of the tabetic symptoms be estab- 
 lished, how far can they be traced back to the anatomic process? This 
 question is easily answered in regard to some phenomena; for others up to 
 this time with great difficulty, or not at all. The objective, and to an extent, 
 the subjective sensory disturbances are founded, doubtless, upon a degenera- 
 tion of the posterior root fibres; this is proved in many cases by the complete 
 radicular distribution, and the ascent of the disturbances, etc. But even 
 here, much is left unexplained. Thus, for instance, the "sensory disturb- 
 ances of the internal organs," especially of the intestines and bladder, seem 
 to be directly or indirectly connected with degeneration of sympathetic 
 tracts. Also the reason why so frequently certain qualities of sensation 
 are disturbed more seriously or earlier than others, has not been definitely 
 established thus far. The explanation of the tabetic pains, especially of the 
 lancinating pains, and of the so-called crises, is most difficult. For the 
 former, which show a predilection to limit themselves to the area of a 
 peripheral nerve, it is possible to consider irritation or compression of a 
 circumscribed place of the posterior roots, even though the type of pains, 
 which certainly depends on such compression (in pachymeningitis, tumors, 
 herpes zoster) , resemble but slightly, as a rule, the lancinating pains. Finally 
 as to the crises, we know, at present, no anatomic basis. Their localization, 
 their character, their connection with considerable increases in blood pres- 
 sure, tempt us very much to refer them to the vasomotor nerves, i. e., to the 
 sympathetic nerve system. 
 
 The disturbance of the muscle sense, Romberg's phenomenon, probably 
 must be traced back also to a disturbance of the centripetal paths, most 
 likely of the fibres ascending in the posterior column; pallesthesia and its 
 disturbance belong here also, and for the stereognostic disturbances we 
 must assume, in contradistinction to the astereognosia of cerebral affections, 
 a mixture of centripetal and centrifugal, sensory and motor disturbances. 
 On the other hand, the frequent absence of the feeling of fatigue in tabetics, 
 
264 DISEASES OF THE SPINAL CORD 
 
 represents certainly a pure disturbance in the sensory rapport of the extrem- 
 ities, that is, in the posterior columns. 
 
 The understanding of tabetic ataxia has long caused great difficulties. 
 If we consider briefly its manifestations, we shall see immediately that a 
 motor disturbance is out of the question. The patient is able to properly 
 innervate every single muscle, at least with the aid of the control of the eye; 
 the impulse of the will to the periphery, i. e., the motor conduction is therefore 
 absolutely retained in the usual forms of tabetic ataxia. What is disturbed 
 is, first, the sensory rapport at the periphery, the "information" of the central 
 organ as to the position of the separate divisions of limbs, and skeletal mus- 
 cles, but then again, the synergism of the individual muscles, especially the 
 relation between agonist and antagonist. For the lack of sensory rapport, 
 we have the anatomic basis: the degeneration of the posterior columns (in 
 the broadest sense) ; for the co-ordinations, we should have to consider, the 
 so-called co-ordination centers in the cerebellum, pons, quadrigeminal bodies; 
 they are certainly not affected, for the most part, in tabetic ataxia. But 
 co-ordination, also, needs the centripetal, sensible (sensory) impulses; it is 
 natural, therefore, to assume here, too, a genuine disturbance of the 
 centripetal paths. Without entering here upon the moot points in detail 
 (which, in Erb's opinion, are not, as yet, fully cleared up), let us mention 
 only that hypotonia of the muscles (cf. below) as well as reflex processes 
 seem to play some part in tabetic ataxia. But in the main, it is doubtless 
 a question of a "sensory" ataxia (Ley den) and of a disturbance of the sensory 
 paths (Otfried Fbrster) . The last named author sees the anatomic basis of 
 tabetic ataxia in the degeneration of the reflex collaterals in the spinal 
 cord, the collaterals to Clarke's columns (cerebellum) and the long fibres of 
 the posterior column. I fully agree with his point of view. 
 
 The disturbance of the tendon reflexes is certainly founded upon the 
 degeneration of the reflex collaterals, of the root fibres passing from the 
 radicular zone into the gray posterior columns. (Level for the patellar re- 
 flexes: lumbar segments II to IV; Achilles tendon reflexes: sacral segments 
 III to V; triceps reflex: cervical segment VI-VII.) Hypotonia, also, per- 
 haps even the disturbances of the cutaneous reflexes (rare) are to be traced 
 back to the reflex collaterals. For the "internal reflexes" and the disturb-, 
 ances of the urogenital tract (the so-called sphincter disturbances, incon- 
 tinence and retention of feces and urine, impotence), the genesis, as we 
 mentioned above, is not absolutely clear. We know, to be sure, that the 
 lumbar segments are the seat of the corresponding centers. But animal 
 experiments seem to indicate that the path for the processes mentioned 
 passes at least in part through the sympathetic, for the co-affection of 
 which in tabes dorsalis unfortunately only a limited number of reports are 
 available. 
 
CLINICAL COURSE AND PROGNOSIS IN TABES 265 
 
 The motor centers and paths need occupy us less. In the rare atrophic 
 paralyses, corresponding lesions of the anterior horn have been found, and 
 here and there also neuritic changes. Of the irritation phenomena, athetosis, 
 according to the present state of our knowledge, is certainly a cerebral 
 symptom. In the phenomena of the cranial nerves (nerves of the eye muscles, 
 special sense nerves, trigeminus, vagus) there appear with about the same 
 frequency, neuritic and nuclear lesions; optic atrophy especially is, as stated 
 above, before all a sign of a peripheral affection. 
 
 The changes in the pupils, especially the reflex light rigidity, have always 
 given much concern to neurologists and ophthalmologists. Experimental 
 investigations (Bach and H. Meyer) seemed to prove that in the posterior 
 columns, near the fourth ventricle, that is in the uppermost cervical segment, 
 there is a center for the pupillary reflex (and for the origin of so-called "spinal 
 miosis"); but recently Bumke has found proof to the contrary, and now it 
 almosts seems, as if one should have to return to the older theories, according 
 to which, pupillary rigidity is to be viewed as a lesion in the pupillary fibres 
 of the optic nerve, in the nuclei of the oculomotor or in the ciliary ganglion. 
 But no certain results have been established for the localization of this 
 apparently simple symptom (i. e., for the anatomic proof). 
 
 For the trophic disturbances, to be brief, we have, at present, absolutely 
 no satisfactory localization in the central nervous system ; only this is certain, 
 that they are not to be understood only as local, idiopathic destructive proc- 
 esses of the bones, etc. The changes of the cerebrospinal fluid, finally, are 
 to be explained only by the assumption of a chronic spinal meningitis, 
 perhaps starting especially from the posterior roots. 
 
 The course and prognosis of tabes dorsalis, present on the whole, and in 
 spite of some individual differences, a very sorry spectacle. The preceding 
 observations show the usual course. The patients mostly suffer for a few 
 years from lancinating pains, perhaps also from slight disturbances of the 
 bladder. With sufficient self-observation, they recognize already a slight 
 disturbance of sensation in the feet, some uncertainty in walking in the dark; 
 the examination at this time usually discloses pupillary changes, and a dif- 
 ference in or absence of the Achilles and patellar reflexes, possibly also some 
 hyperesthesia. Diplopia, disturbances in the muscles of the eye, impotence 
 also appear. As a rule years, ten or more, have passed since the first "light- 
 ning pains." Then are added increased disturbances of sensation and 
 ataxia in the legs, gradually also in the arms, which robs the patients more 
 and more of the ability to move (the demarcation of a third stage in the 
 symptomatic picture of tabes, the paraplegic stage, mostly resting upon 
 this, I consider as arbitrary) . The further extension of the process to the 
 cortex of the brain, is usually less clear, since some phenomena of the cerebral 
 nerves appear much earlier; on the other hand, certain cerebral symptoms 
 never or very rarely (athetosis) supervene, and the transition to paresis, as 
 
266 DISEASES OF THE SPINAL CORD 
 
 has been mentioned, is not frequent in typical tabes. The final issue is 
 usually fatal, though death relatively infrequently is due to direct symptoms 
 of the disease (inanition in gastric crises, posticus paralysis) ; more frequently 
 it is due to intercurrent diseases (infective diseases) and especially to second- 
 ary consequence of tabetic processes: sepsis due to trophic disturbances, 
 bed sores or cystopyelonephritis, as the result of external infection due to 
 the sphincteric lesions. 
 
 There are several characteristic variations from this usual type. In the 
 first place, the course may be very much more rapid, and it is generally 
 the first stage which is very much shortened; in such instances the first 
 paroxysms of pain may be followed by ataxia in a few weeks, while the later 
 stages may extend over the usual long period of time. Other characteristic- 
 ally atypical forms are juvenile tabes, appearing in children, almost without 
 exception, upon a congenital syphilitic basis, and, as a rule, of a relatively 
 benign character (thus far only a single autopsy of an indubitable case has 
 been reported), and tabes superior, which, on the other hand, is a very 
 unfavorable form of the disease. In tabes superior the symptoms begin in 
 the upper extremities, with pain in the ulnar area and paresthesias, also 
 triceps reflex disturbance, whereas the tendon reflexes in the legs may be 
 retained; in such cases bulbar symptoms, dangerous to life, usually 
 supervene very early. 
 
 In addition to these types, authors who have studied a large number 
 of tabetic patients, like Erb and Dejerine, have distinguished additional 
 clinical types, according to the predominance of one or the other symptom: 
 tabes dolorosa with especially violent pains; tabes visceralis with violent 
 crises and without specially marked subjective concomitant symptoms. 
 Even though the value of this classification can be practical only in so far, 
 as such cases usually preserve their characteristic form for the entire and 
 usually protracted course of the disease, for one form the predominance of 
 one symptom is so significant, that a particular anatomic basis has long been 
 looked for: tabes with early optic atrophy. According to the investigations, 
 especially of the school of Pierre Marie (Leri and others) 25% of tabetics 
 lose their sight in this way; in these cases the rest of the nervous system, 
 frequently participates with cerebral symptoms, but rarely with spinal 
 symptoms of marked gravity (with the exception of a regular loss of the 
 tendon reflexes); the further course also, usually protracted, corresponds 
 to this picture so that a few authors interpret "tabetic amaurosis" and the 
 usual form of tabes as two different localizations of the same process. 
 Finally to the deviating forms of tabes, there belong also the cases of imper- 
 fectly developed, rudimentary tabes, of which much has been published in 
 recent years. The conception is not easily defined; as a matter of fact, one 
 can speak of rudimentary or "abortive" tabes (Mobius) only when the con- 
 dition, once discovered, persists stationary throughout or at least remains 
 
COURSE AND PROGNOSIS OF TABES 267 
 
 thus for a very long time, and, finally, shows only few symptoms of the 
 disease. The possible combinations of such individual symptoms are, as 
 Erb emphasizes, naturally very numerous and varied, but in almost all the 
 cases, there is found one of three symptoms: loss of tendon reflexes (if only 
 of one single Achilles reflex); pupillary changes (possibly only miosis 
 without loss of the reaction to light); lymphocytosis of the cerebro-spinal 
 fluid. Naturally in such a patient, whose affection is recognized acci- 
 dentally, possibly as he is being examined by the physician because of 
 "rheumatic pains," one cannot tell at once, whether he will not end by 
 becoming afflicted with a fully developed tabes, but not rarely this is not 
 the case, and we may then assume without further investigation, that it is a 
 question of fixed, quasi "checked" pathological changes, which have 
 possibly affected only the posterior roots and meninges and did not go any 
 further than the entrance into the spinal cord. In these cases, the addition 
 of even one new symptom during the period of observation is usually the 
 signal for a further development of the disease, which then generally 
 continues steadily to progress. Naturally, this rudimentary tabes, is an 
 especially interesting field for diagnosis. 
 
 The prognosis of tabes is apparent, from what has been already said. 
 It is, quoad sanationem, bad. Cases of genuine recovery, i. e., of complete 
 disappearance of the subjective, and a total or almost total disappearance 
 of the objective symptoms (areflexia of the pupils or some similar symptom 
 alone remaining), as shown in the pathological changes brought to light by 
 post-mortem investigations, are exceedingly rare, but do occur occasionally. 
 Even the disappearance of single symptoms is rare; in regard to this v. 
 Malaise (from Oppenheim's polyclinic) recently reported the following: 
 The lancinating pains improved in 37% of the patients (in forms that ran 
 a wholly favorable course), the bladder disturbances in 8 to 10%, the sexual 
 power never, when once disturbed (but here one must consider the physio- 
 logical decrease that normally appears with advancing years). Very fre- 
 quently, on the other hand, the crises that have once appeared, disappear, 
 that is, they do not return, whereas atrophy of the optic nerve nearly always 
 leads to total blindness, occasionally only after ten years, on an average 
 after 5 1/2 years. Ataxia, as we shall see, can be improved by therapeutic 
 measures, and perhaps even spontaneous improvement is possible; in regard 
 to the paralysis of the muscles of the eyes we have mentioned already, that 
 even in their intensity, they are usually subject to extraordinary fluctuations. 
 
 Cases, in which the so-called late symptoms appear early, are, as in 
 most diseases, prognostically unfavorable (ataxia, trophic disturbances); 
 the longest possible extension of the first stage is rightly "considered favorable. 
 Frequently, also, the disease remains stationary for years, and under favor- 
 able conditions of life, the patients may live to a very old age, so that accord- 
 ing to French writers (Bellugaud and Faure) about 59% of all tabetics 
 
268 DISEASES OF THE SPINAL CORD 
 
 experience an "evolution benigne," 36% an "evolution grave," and 5% 
 recover. I consider these statistics too favorable, and should prefer to 
 subscribe to the statement of v. Malaise, who out of 70 patients, found 2 
 completely freed of their troubles, 26 retaining their working capacity for a 
 long time, and in good general condition, 30 with constant but slow pro- 
 gression and 18 running a rapid and unfavorable course. Some factors 
 mentioned when discussing the aetiology, influence also the further course 
 of the disease. Especially unfavorable results come from alcohol, sexual 
 excesses, traumatisms and physical over-exertion, extreme degrees of tem- 
 perature, persisting cares and excitements. 
 
 Somewhat more favorable is the question of prognosis quoad vitam. 
 Pierre Marie assumes that the length of life is not shortened by tabes (51.5% 
 died after the sixtieth year) and even with the less favorable material of 
 Oppenheim, the statement that the majority of the patients die after the 
 sixtieth year, holds good. 
 
 The diagnosis of tabes, under ordinary circumstances, is easy for every- 
 one who understands how to make a neurological examination. The un- 
 fortunately still very frequently occurring errors in the diagnosis of the 
 disease are to be explained only by a lack of such knowledge. The above 
 mentioned abortive cases and occasional cases with peculiarly complicated 
 symptoms or course, are the only ones which may cause the neurologist dif- 
 ficulty. We have mentioned several times already the cardinal symptoms, 
 of which one, at least, is to be found also in the abortive tabes, the "tabes 
 fruste " : disturbance of the tendon reflex, pupillary disturbance, lymphocytosis 
 of the cerebro-spinal fluid. If all three are absent, we may, with a consid- 
 erable degree of certainty, exclude tabes; at the most, patients with per- 
 sistent pains, like the lancinating ones, should always arouse a suspicion 
 of tabes, which, however, would have to be fully confirmed sooner or later 
 by the appearance of one of the symptoms named. Each of these cardi- 
 nal symptoms, that may accidentally be found in a patient, must, in itself, 
 suggest the possibility of the existence of tabes; if, in the anamnesis syphilis 
 appears, or if, in the history of the existing disease, possibly a paralysis of 
 the muscles of the eye is spoken of, or if, in the objective examination, 
 hyperesthesia to cold in the trunk is found, the thought is so much more 
 reenforced. Naturally some of these phenomena appear also in other 
 diseases, with the exception, perhaps, of the hyperesthesia to cold; at any 
 rate, the combination of some such symptoms, as well as the further course 
 of the disease, usually justify the diagnosis of tabes. 
 
 Of the abortive cases we have already spoken. If we see, for instance, a 
 patient with slight pupillary differences, lancinating pains, and owning to a 
 history of syphilis, I recommend always, beside the most careful examination 
 as to sensation and tendon reflexes, above all to perform a lumbar puncture 
 for purposes of diagnosis; if this turns out negatively, I am inclined to exclude 
 
DIAGNOSIS OF TABES 269 
 
 tabes or at least consider it most improbable; a positive result in lumbar 
 puncture makes the diagnosis of a metasyphilitic disease of the central ner- 
 vous system (according to its frequency, probably a tabes) almost certain, in 
 so far as, and this is easy for the most part to determine, another meningeal 
 process can be excluded. I should arrange the diagnostic symptoms, in the 
 order of descending importance, as follows: 
 
 1. Loss of the tendon reflexes. 
 
 Miosis and reflex rigidity of the pupils to light. 
 Lymphocytosis of the cerebro-spinal fluid. 
 
 2. Hyperaesthesia to cold. 
 
 Other sensory disturbances of tabetic type, especially, however, 
 summation and retardation in conduction of pain. 
 Anamnesis: lancinating pains; former syphilis. 
 
 3. Paralyses of the muscles of the eye. 
 
 4. Romberg's symptom. 
 Ataxia. 
 
 Hypotonia of the musculature. 
 Sphincter disturbances, impotence. 
 
 5. Optic atrophy. 
 Crises. 
 
 Trophic disturbances. 
 
 For the great variety of symptoms to produce diagnostic difficulties, is a 
 relatively rare occurrence, and happens almost only when totally foreign 
 phenomena are present in addition to the complete picture of tabes; for 
 instance, hypertension of the muscles, exaggerated tendon reflexes, periph- 
 eral paralyses. As a rule, then, it will be a question of combination with 
 other diseases— sclerosis of the lateral columns, peripheral neuritis — only 
 exceptionally of a pure tabes with an atypical course. 
 
 Excluding the combinations we have just mentioned, especially the so- 
 called combined systemic diseases, which vary in their symptoms according 
 to the greater participation of the posterior or the lateral columns, only the 
 various forms of neuritis are of differential diagnostic importance, if we except 
 also the rare, usually epidemic ergotin poisoning (ergotin tabes), the symp- 
 toms of which correspond almost exactly with those of real tabes. Of 
 neuritic processes, alcoholic neuritis most frequently hampers our diagnosis, 
 more rarely the diphtheritic and other toxic forms of neuritis (diabetes melli- 
 tus with neuritis) . Whereas in these patients, the loss of the tendon reflexes 
 and the disturbances in sensation may remind one strongly of tabes, the motor 
 paralyses, and, above all, the promptness of the pupillary reaction, lead 
 usually to the correct diagnosis. Multiple sclerosis and syringomyelia may 
 also upon occasion, enter into the problem of the differential diagnosis. 
 
 In spite of the bad prognosis, the treatment of tabes, corresponding to the 
 chronic character of the disease, is even to-day uncommonly diverse, and, 
 
270 DISEASES OF THE SPINAL CORD 
 
 let us add, by no means devoid of results from the standpoint of alleviating 
 the suffering of the tabetic. 
 
 Treatment must be aimed at two points, inasmuch as only in the rarest 
 cases, is there hope for complete recovery. It must be adapted to the 
 existing stage of the disease, and to the degree of the result that probably 
 may be attained, and must not, by the violence of applications, or by 
 meddlesomeness physically or mentally over-exert and fatigue the patient. 
 
 Because of the meta-syphilitic nature of the disease the first thing to do, 
 is to try a course of anti-syphilitic treatment, to give first mercury or iodides. 
 Owing to the differing views of various physicians and "schools" this 
 plunges us at once into a region of dispute. There is comparative agree- 
 ment as to the fact, that in particular mercurial treatment does as a rule no 
 harm, and in some few cases produces a slight, but definite beneficial effect. 
 From this middle point, however, the positions of the extreme camps deviate 
 considerably in both directions. While some physicians reject the Hg. cure 
 as useless and occasionally injurious, Erb, especially, defends it earnestly; 
 in his experience, it does "absolutely no harm," is well tolerated, and has 
 often an excellent influence especially on the lighter symptoms of the disease 
 (disturbances in sensation, etc.). I, myself, should like to say that I never 
 saw mercurial treatment make the condition worse (I am a little doubtful only 
 in cases of patients with optic atrophy, though I can not confirm the harmful 
 influences of the treatment described by some authors). Direct favorable 
 consequences, which could be ascribed only to the Hg. cure, I have seen but 
 rarely; on the other hand, it has been quite the usual thing in my cases, that 
 with regular treatment by inunction, repeated annually or every other year, 
 the patients were subjectively in good condition, were able to perform work, 
 gained weight, and remained for years about the same. Some of the patients 
 ascribed considerable improvement to the inunctions and asked for them 
 again and again. Naturally, even mercury fails to produce results in a 
 number of cases, and I have not succeeded definitely, any more than have 
 most of the French authors, in discovering in the lymphocytic content of the 
 spinal fluid — which denotes the condition of the meninges— any difference 
 worthy of mention before and after the treatment. On the whole, I recom- 
 mend mercurial treatment in recent cases (it constitutes, in my opinion, the 
 only prophylactic method worthy of recommendation, after syphilis has been 
 acquired, especially for neuropathic individuals, though one must concede, 
 that even after syphilis has been treated with the most painstaking correct- 
 ness, a tabes may appear later), in syphilis still active in association with 
 tabes, and in general in those cases in which, after definitely or probably 
 diagnosed syphilis, no mercurial treatment has been used for some time. 
 The only condition is that of moderate vigor of the patient and the possibility 
 of control of the treatment by the physician. If syphilis may be excluded 
 with probability, or many courses of treatments have already proved ineffec- 
 
TREATMENT OF TABES 271 
 
 tualj I forego mercury as a rule. Concerning the method of application, I 
 have like Erb, had the best results from small, frequently repeated inunctions 
 (daily, for 30 days, 1 to 2 5 mercurial ointment) ; the usual care of the gums 
 for the prevention of stomatitis is especially important. Concerning the use 
 of iodides, there is less difference of opinion; in addition to or between the 
 Hg. treatments, I administer for a space of 3 to 6 weeks, iodide of potassium 
 (15 to 30 grs. pro die), also iodipin (subcutaneously) or saiodin (20 to 30 grs. 
 pro die), and have seen from this treatment only favorable results; special 
 consideration must be given the appetite and digestion of the patient, which 
 therefore often requires that internal medication be replaced by subcutane- 
 ous administration of iodipin, which otherwise is not agreeable to either 
 patient or physician. Serological syphilitic therapy unfortunately has, up to 
 the present, shown no effect on tabes. Now the entire host of nervina follows ; 
 in order to make a change here and there, the physician must have at his 
 disposal a sufficient supply of remedies. Ergotin (secale cornutum 0.5 pro 
 die) I have used but seldom; Charcot's school, however, formerly recom- 
 mended it warmly. Argentum nitr. I give occasionally, preferably in pills (for 
 instance: Arg. nitr. 15 grs. strychni grs. ii. in 100 pills, one daily three times), 
 for several weeks, in combination or in alternation with preparations of 
 strychnine, of which, especially in the form of Erb's "tonic pills" I have seen 
 excellent results (Ferr. lactic. 25 grs., extr. chin. aq. 25 grs., Extr. nuc. vom. 
 7 to 10 grs., extr. gentian, q. s. to 100 pills, take six daily). The arsenical 
 preparations in their pure form I do not like very much, on the other hand, 
 I can recommend warmly the cacodyl preparations (natr. cacodyl. 0.02-0.1 
 subcutaneously pro die) from my own experience, for continual treatment. 
 Of atoxyl, which has recently been used as an anti-syphilitic agent, I have 
 seen little benefit in tabes. Of general tonics, one must mention in addition, 
 and for occasional trials, glycerophosphate, lecithin, nucleogen, thiodine, re- 
 cently inaugurated by the French, phytin, etc. The same purpose, preserva- 
 tion of strength, beside the nourishment of the patient is served by the host of 
 medicines that rouse the appetite, and the artificial food preparations. (Sal- 
 varsan is of no benefit in tabes, in my experience, and may do harm. Burr.) 
 
 As an attempt at local treatment at the place of the disease, we may name 
 here the various methods of influencing the spinal cord and the posterior 
 roots. In the front rank stands the galvanic current, which may be used in 
 almost every stage and with almost every patient. 
 
 To be recommended is the stabile use of the constant current; two 
 equally large, medium sized electrodes are used, one on the nape of the neck 
 and the other over the lumbar enlargement, using a current of 8 to 10 milli- 
 amperes, alternately 3 to 4 minutes in each direction, without brusque inter- 
 ruptions. Frequently also I apply a larger electrode to the spine, a smaller to 
 an extremity (peroneus point, wrist) or use Erb's sympathetic galvanization; 
 the small cathode over the cervical sympathetic, the larger anode over the 
 
272 DISEASES OF THE SPINAL CORD 
 
 opposite half of the body, placed in three "stations" near the spinal column, 
 using a current of about equal strength. Symptomatically, also, especially 
 in enteralgias and weakness of the bladder, the galvanic current is valuable : 
 medium sized electrode over the lumbar segment of the cord, a large one 
 over the abdomen or bladder. I use the faradic current (medium strength) 
 far more rarely, actually only with paresthesias and disturbances in sen- 
 sation, therefore more symptomatically. 
 
 As local application, we consider also counter-irritation along the spinal 
 column, particularly painting it with tincture of iodine, mercurial plaster 
 (combined possibly with belladonna); also from the actual cautery (small 
 point-size thermo-cauterizations on and near the spinal column, 20 to 30 at 
 every sitting) one occasionally sees results, especially for the girdle feeling, 
 etc. Furthermore, I wish to mention here the various methods of stretching 
 the spinal cord, or the spinal column; either in the form of suspension (it is 
 best to fasten to the seated patient a sort of Sayres suspensory apparatus at 
 nape, chin, possibly also with shoulder straps, which by means of a weighted 
 rope passing over a pulley, is drawn upward) or as so-called bloodless stretch- 
 ing of the nerves, by which — in various ways — the knees or the extended 
 limbs of the patient, who is lying on his back, are passively brought as near 
 as possible to the upper part of the body and kept thus fixed for some time. 
 The hypotonia, which is usually present, facilitates this method, which, on 
 the other hand, just because of this is not quite devoid of risk. The stretch- 
 ing procedures are praised very highly by a large number of writers. The 
 so-called bloody nerve-stretching has been justly abandoned. 
 
 Massage is especially valuable for bed-ridden tabetics; it is particularly 
 indispensable in the ataxic stage, partly to keep the musculature in good 
 condition, and at the same time to prepare it for the exercise treatment, to 
 be mentioned below, and partly as an aid to metabolic processes as a whole. 
 To watch over the metabolism and the state of nutrition of the patient, is on 
 the whole a main factor in the therapy of tabes. By every means, moderate 
 forced feeding, nourishing foods, methods of stimulating the appetite, etc., 
 the physician must try to keep the body in such condition, that it is capable 
 of action and resistance; indubitably, thus alone, greater service is rendered 
 the individual, but especially the more advanced tabetic, than by the use of 
 complicated medicinal compounds. 
 
 The allotment of rest and exercise is of great importance. Above all, the 
 slightest over-exertion on the part of the patient must be avoided. Accepting 
 Edinger's "using up" (exhaustion) hypothesis, it would be a good thing to 
 force the patients to take absolute rest for some time; Edinger himself has 
 recently emphasized this again. But without considering the fact, that in 
 the very protracted course of most cases, such treatment would be tantamount 
 to an almost unendurable test of patience on the part of the sufferers, accord- 
 ing to the views of most authors, the result of the rest cure is uncertain, even 
 
TREATMENT OF TABES 273 
 
 in those cases, in which local over-exertion had certainly helped to bring 
 about the disease (isolated ataxia of the right arm in tabetic clerks). 
 
 On the other hand, among the general therapeutic methods, hydro- and 
 balneotherapy, rank next in importance after nourishment. 
 
 Almost all methods of procedure may be employed, especially in the 
 earlier stages of the disease; they may be used for a long time, but, on the 
 other hand, it should be a principle never to employ them in too energetic 
 a form — especially in respect to the temperature. Few things can injure a 
 tabetic more than using applications too extreme in cold or heat; I, myself, 
 have seen several tabetics, who, after energetic schematically applied Kneipp's 
 cures, showed a decided turn for the worse. Among the water "cures" 
 that can be used at home, I recommend mostly the hip baths (as a rule, 
 without shower) of 90 to 95 F., which are applicable in nearly all stages 
 of the disease; alternating with them, or as a separate "cure" one can give 
 artificial carbonic acid baths, pine-needle baths, to strong patients cold 
 friction baths, to the weaker ones, lukewarm douches. 
 
 Not only upon psychic grounds, but especially because of their almost 
 specific effectiveness, "bath cures" (watering places) are to be recommended, 
 among which must be named first, the baths of Nauheim and Oeynhausen 
 (carbonic acid thermo-baths) ; here too, however, baths of medium intensity 
 are most effective. All other baths are inferior to these (but we might 
 recommend in addition the sulphur baths — Aixla Chapelle, Neundorf — and 
 the indifferent hot baths — Baden-Baden, Gastein, Wildbad) ; many tabetics, 
 however, are much improved by simple fresh air treatment in the middle and 
 high altitudes, whereas, sea baths, as a rule, are less beneficial. 
 
 The great variety and all too frequent ineffectuality of our therapy, is 
 shown in the treatment of the separate tabetic symptoms, which we shall 
 briefly discuss. 
 
 The lancinating pains are especially difficult to combat; as the attacks 
 are usually short, the use of morphine is, as a rule, to be dispensed with, 
 more so since these pains generally react best to the simple analgesic " anti- 
 rheumatic" agents: aspirin, pyramidon, citrophen, lactophenin, especially 
 antipyrin and antifebrin in the form of Erb's "mixed powder" with codein 
 (antipyrin grs. 7, antifebrin grs. 5, codein gr. 1/3-gr. 1/4; instead of anti- 
 pyrin possibly also phenacetin), migrainin, etc. If these agents are ordered 
 in large doses, or in short intervals, the result is usually favorable. It is 
 more difficult to combat the permanent pains, enteralgias, girdle-pains, 
 etc., and most difficult of all the genuine tabetic crises, especially the 
 gastric crises, which can be influenced almost only by large subcutane- 
 ous doses of morphine. The internal medicines above mentioned (and in 
 addition, bismuth, ansesthesin, cerium oxalate, bromides, etc.), are usually 
 ineffectual; I have occasionally seen results from lumbar puncture and 
 from the injection of soda nitrate subcutaneously. If one does not 
 
274 DISEASES OF THE SPINAL CORD 
 
 wish to give morphine, there remains, usually, only one way, namely, 
 to let the crises spend their strength and to attempt to keep the patient, by 
 complete rest and artificial nourishment, on a high plane of strength. 
 Nor are the external applications of much avail here, whereas in lancina- 
 ting pains, hydropathic packs, painting with iodine, chloroform, oil, methyl- 
 salicylate, salves and balsams may occasionally prove useful. 1 
 
 The other sensory disturbances rarely become so painful or (except when 
 trophic changes arise, cf. below), so dangerous, that they need a special 
 therapy; labile faradic treatment, farado-massage may be used for par- and 
 anaesthesias. 
 
 The motor disturbances with the exception of ataxia, likewise need no 
 particular treatment; peripheral paralyses are rare; for the paralyses of the 
 muscles of the eye, which incline towards spontaneous improvement, 
 galvanization may be tried. 
 
 On the other hand, as is well known, the treatment of ataxia forms one of 
 the main problems of modern tabes therapy, in the form of what is generally 
 called the Fraenkel " exercise therapy," which was variously tested and com- 
 pleted by Fraenkel, Goldscheider , Foerster, and others. As has been eluci- 
 dated above, we understand by ataxia, speaking generally, and for practical 
 therapeutic purposes, a disturbance of co-ordination, of the co-operation of 
 the individual muscles and groups of muscles. To this corresponds the single 
 therapeutic purpose: restoration of the lost co-ordination by practising this 
 very process. Inasmuch as the disturbance evinces itself, according to our 
 experience, preponderantly in the dark, that is, betrays itself in movements 
 not controlled by the eye, the process, in practice, is very simple; the move- 
 ment that has been disturbed is gradually learned again (by practice) under 
 the control of the eye, until it eventually succeeds, even without observation. 
 In our experience, the help of the eyes is a mighty factor in the movements of 
 ataxic limbs, and allows, even without special practice, many movements to 
 be performed, that would be wholly impossible, if the eyes were closed. 
 
 The exercise therapy is to affect every muscular area stricken by ataxia; as 
 a rule, of course, first and most important, the legs. Since it must be a main 
 principle, that the patient is not to be tired out (exhaustion may lead to out- 
 spoken deterioration in the condition of the patient), the first leg practice 
 should take place in bed; from the most simple, there should be gradual 
 advance to the difficult. For exercise therapy there exists a long line of 
 useful directions {Fraenkel, Goldscheider and others) which can be used in 
 complicated cases, especially also, to make greater variety possible. They 
 describe also a great number of assisting apparatus (diagrams to be followed 
 out by hands or feet, parallel bars to walk between, etc.), which, however, 
 in the majority of cases can be dispensed with, especially if the physician, as 
 
 1 Whether the results obtained by internal use of adrenal preparations, reported by Rohmer to 
 the society of German neurologists, October, 1908, are confirmed, has not yet been ascertained. 
 
TREATMENT OF TABES 275 
 
 should always be the case, watches over the execution of the exercises. As a 
 rule every physician will be able himself to compose easily the most important 
 movements. Besides the gradual learning of all movements, attention must 
 be paid above all to the exact co-working of the single components of the 
 most essential and important movements: walking, dressing, undressing, 
 eating, writing, etc. Employing the necessary patience, the method leads to 
 improvement in nearly every case, sometimes to an almost total disappearance 
 of the ataxia. Of course, one must work with greatest consideration and care 
 for weeks, often for months, in the beginning several times daily, for a few 
 minutes. They are really contraindicated only during the first weeks of an 
 acute, rapidly appearing and increasing ataxia and if the patient's general 
 state of health is in too low a condition. During other intensive treatments 
 (energetic bath cures) it is better to cease the exercise therapy temporarily/ 
 
 The other symptomatic indications for treatment are more or less self- 
 evident. A warning must be sounded against medication, directed to in- 
 fluencing tabetic impotence; all the highly recommended remedies, especially 
 yohimbin, the effects of which are to be understood as a stimulation of the 
 sexual sphere, have proved to be unnecessary, and partly injurious; in 
 lighter cases, rest, a sparing therapy, together with. general tonic procedures, 
 is most likely to produce an improvement, which, to tell the truth, is usually 
 transient. We can not give sufficiently strong warning against all extreme 
 measures. 
 
 The bladder disturbances react relatively best to galvanization, occasionally 
 also (bladder weakness) to ergot and strychnine. In pronounced retention, 
 catheterization is, alas, anavoidabh; this, after longer or shorter time, as a 
 rule, causes an infection of the bladder, which, it is true, can be treated for 
 some time by washing out, etc., but is apt finally to lead to ascending 
 processes — pyelitis, pyelonephritis. In tabetic retentio alvi, medicines are 
 to be used only in cases of extreme necessity. 
 
 The trophic disturbances are treated according to general surgical rules; 
 the arthropathies can, as a rule, be treated only orthopsedically. 
 
 Because of the usual, slowly progressive course of tabes, a somewhat 
 typical plan of treatment may be outlined for many cases. To a large extent, 
 however, this plan depends upon the stage in which the disease is recognized. 
 If the recognition takes place, as it always should, at the beginning of the 
 lancinating pains, for which the majority of tabetics consult their physicians, 
 there should be introduced, under the above (cf. under therapy) conditions, 
 a mercurial treatment, best by inunction. Naturally, besides this, at least 
 during the recognizable advance of the disease, every active mental and 
 physical exertion, especially exciting occupations, sports, hot or cold baths, 
 sexual indulgence, should be immediately given up, or, at .least curtailed. 
 Mild hydrotherapy (hip baths) and galvanization can even now, but in any 
 case, as continuation of the mercurial treatment lasting possibly four weeks, 
 
2 76 DISEASES OF THE SPINAL CORD 
 
 be used for 2 to 3 months. In cases where the mercurial treatment is 
 excluded (cf . above) an iodide therapy (iodide of potassium, iodipin, saiodin) 
 is mostly to be recommended as a substitute for 2 to 3 months; it may 
 be used also along with the Hg. treatment, or as its continuation. In the 
 second place, after the course of the first 2 to 3 months, a generally tonic 
 method may be employed in all cases: arsenic, cacodyl, ErVs tonic pills, etc., 
 for 4 to 8 weeks. If circumstances permit, a "bath cure" should be 
 interpolated in the summer, at Nauheim or Oeynhausen, with temporary 
 galvanization. 
 
 Then in the normal course, a pause in treatment of some weeks or even 
 months is to be recommended. After the symptoms have come to a stand- 
 still, one may wait to the end of the first year, and then carry out a similar 
 program in the following years, with slight modifications (relative to hydro- 
 therapy, the use of tonics, derivation to the skin, suspension). If the 
 disease makes a marked advance, the therapy must naturally keep step 
 with it, but with avoidance of every, even therapeutic exertion, for the 
 patient; often absolute rest is now best for the patient. Symptomatic 
 measures (especially exercise for the ataxia) naturally are to be employed 
 at the same time. In the last stage of the disease, there is a question only 
 of care and palliative measures, not infrequently of extensive morphine 
 therapy. Even now, however, mild procedures, as galvanization, may be 
 tried with some success. In any case, so long as no serious cachectic com- 
 plications (serious pyelitis, bed sores) are present, there is need to beware 
 as well of therapeutic nihilism as of meddlesomness, since in spite of the 
 slight influence we are able to exert on the course of the malady, there are 
 few diseases, in which such excessive demands may be made upon therapeutic 
 resources as in tabes dorsalis. 
 
 (b) Friedreich's Disease (Hereditary Spinal Ataxia) 
 
 BY 
 
 S. SCHOENBORN (Heidelberg) 
 
 Friedreich? s disease represents only a subdivision, though the best 
 known and sharpest defined, of a larger group of more or less systematized 
 diseases of the central nervous system, which are of a degenerative nature, 
 usually show hereditary or familial propagation, and are founded in part at 
 least upon an anomaly of predisposition of the central nervous system, and 
 develop from this. Common to them all, is a more or less well developed 
 degree of ataxia, which has given the group its name. To this are' added, 
 according to the individual form, disturbances of reflexes, scoliosis, bulbar 
 symptoms, optic nerve changes, and disturbances in intellect. The best 
 method of dividing the entire group may be that recently proposed by 
 
FRIEDREICH'S ATAXIA 277 
 
 Raymond, which divides it into 4 separate types of disease, of which we 
 must recognize 2 as indubitably correct. 
 
 1. Spinal form or Friedreich's disease sensu strictiori, with ataxia, 
 nystagmus, speech disturbances, absence of tendon reflexes, scoliosis, 
 club foot. 
 
 2. Cerebellar form or heredoataxie cerebelleuse {Marie) with ataxia, 
 optic nerve changes, paralysis of the muscles of the eye, exaggeration of 
 the reflexes, vertigo, intellectual disturbances. 
 
 Between these, there exist doubtless transitional forms as well as the 
 combination of 1 and 2, which Raymond classifies as an independent fourth 
 group: "type generalise" of familial character. Whether an independent 
 bulbar form (with dyspnoea, vomiting, cardiac arythmia) exists, seems to 
 me doubtful from the literature; I myself have seen no such case. Raymond 
 defines such a form as the last subdivision. Let us now turn to Friedreich? s 
 disease, in its stricter sense. 
 
 Friedreich's disease is of rare occurrence. In 1901 I could collect from 
 the literature only a few more than' 200 cases; meanwhile the number may 
 at the most have doubled. Apparently the disease occurs disproportionately 
 less frequently in Germany than in England and North America. Of its 
 origin we know but little. It seems certain that it does not owe its develop- 
 ment to a definite poison, differing in this from the otherwise so similar 
 tabes, but that many factors may serve as "agents provocateurs." Very 
 often the disease is familial, but it is very seldom directly hereditary. This 
 may be due to the lack of possibility of transmitting the disease by heredity, 
 since it is already far advanced, when the patients arrive at marriageable age, 
 partly, as Bouche has it, the disease itself may not be transferable, but only 
 the neuropathic tendency and degeneration, which (because of alcoholism, 
 tuberculosis, syphilis) is actually found in the parents of the patients. It 
 is probable that a slowly progressing inhibition in development of the 
 spinal cord, which quite early is found very small, is the primary cause, 
 which gives rise to the disease picture; the disease may then be excited by 
 infectious diseases, possibly also by traumatism. 
 
 The disease begins very early, usually between the 6th and 14th years; 
 all cases developing after the 25th year are doubtful. 
 
 The pathologic-anatomical conditions are rather typical. The 
 spinal cord is noticeably attenuated, especially in the posterior portions 
 (occasionally, also, in toto). To this corresponds an ascending degeneration 
 of the posterior columns, always of the columns of Goll, occasionally 
 partly, of the columns of Burdach also. As a rule, the lateral columns are 
 affected also and most frequently the lateral cerebellar tracts, somewhat 
 less frequently, the lateral pyramidal columns. The columns of Clarke 
 and of Gower may be affected too, rather exceptionally the anterior pyramidal 
 columns also. By French authors (Dejerine) emphasis is laid on the fact 
 
278 DISEASES OF THE SPINAL CORD 
 
 that the posterior roots and the peripheral nerves degenerate as well (motor 
 and sensory), which, however, is described as inconstant and insignificant 
 by German authors (Oppenheim). The medulla oblongata and the cere- 
 bellum, however, do not show any changes in typical cases. The degenera- 
 tion affects axis cylinders as well as medullary sheaths; in severe cases the 
 axis cylinders in the columns of Goll in the cervical part of the cord disappear 
 entirely. 
 
 The similarity to the process in tabes dorsalis, is quite considerable, if 
 one regards, as do the French authors, the degeneration of the posterior roots 
 as a regular thing, and the difference concerns particularly the lateral 
 columns, which remain intact in tabes. But it is far more difficult to dis- 
 cover the pathogenesis of the disease. It is as yet quite doubtful whether 
 the posterior columns are affected primarily, and everything else secondarily, 
 or if the disease actually ascends from the periphery through the posterior 
 roots. The latter could be accepted only with the aid of the hypothesis 
 (Dejerine), that Friedreich's disease consists in a deficient development of 
 certain neurons, together with insufficient replacement of the fibres used 
 up in the course of time. This is contradicted, however, by the regular, 
 intactn'ess of the ganglion cells in the anterior horns and of the spinal ganglia, 
 whereas in the columns of Clarke the ganglion cells are often found destroyed. 
 Also the Scherbak hypothesis of primary degeneration exclusively of fibres 
 passing to or .from the cerebellum, is still lacking in pathologic-anatomical 
 proof, however significant it might be in regard to the above mentioned prin- 
 ciple of dividing this group of diseases. The pathogenesis of the individual 
 symptoms we shall have to discuss later. On the whole, then, we shall 
 have to consider Friedreich's disease in the pathologic-anatomical sense as a 
 combined system disease with preponderant participation of the posterior 
 columns. 
 
 The clinical picture of the symptoms generally corresponds to this. 
 Nearly always the children themselves, or members of the family first notice 
 the appearance of the ataxia. Their gait becomes uncertain, with legs 
 .spread wide apart; they stamp while walking; they spill the liquids they are 
 carrying, and begin to write illegibly. If the movements are tested individ- 
 ually, a decided ataxia is seen to exist in the intentional as well as the unin- 
 tentional movements, and it is usually equally pronounced in all the extremi- 
 ties at an, early date; as a rule, the head also is attacked in the form of the 
 so-called static ataxia: the patients sway even while at rest, i. e., in sitting or 
 standing, rock constantly to and fro with the head and trunk, like a tree 
 blown about by the wind. This instability affects all movements simultane- 
 ously, and reminds one of the ataxia of tabetics. But doubtless there is an 
 admixture of another component (especially perceptible in walking), which 
 reminds one of cerebellar unsteadiness. Very many patients exhibit besides 
 the ataxia of the single movements, a wholly unsteady gait, as if intoxicated; 
 
SYMPTOMATOLOGY OF FRIEDREICH'S ATAXIA 
 
 279 
 
 when walking they shove themselves forward, while they attempt, to a cer- 
 tain extent, to steer the body, which is usually held stiff. Occasionally there 
 is even a certain degree of propulsion. With eyes shut, the unsteadiness, as a 
 rule, increases, as in tabes, but the patients virtually never fall down. On 
 the whole, because of the extremely slow development of the disease, the 
 patients learn to control their movements in part, in spite of the ataxia. 
 Thus one of my patients, in spite of a high degree of ataxia in the hands, was 
 
 Fig. 61. — Form of foot in Friedreich's disease. 
 
 able to draw very beautifully and write very legibly, both, of course, very 
 slowly. 
 
 Beside this disturbance in movement, there appears frequently, especially 
 in the muscles of the head and face, also of the hands, a muscular restlessness, 
 which recalls chorea (grimaces) or perhaps more often athetosis. 
 
 At the same time the mere physical strength of the patient is not decreased 
 to any extent, though usually the arms and legs grow somewhat emaciated. 
 But local atrophies are very rare, though the frequent pes varus (varo- 
 equinus) seems to suggest them in Friedreich's disease. As a matter of fact, 
 the planta pedis is usually very thin and the prominence of the arch of the 
 
2 8o DISEASES OF THE SPINAL CORD 
 
 foot and of the tendon of the extensor hallucis longus makes the disease pic- 
 ture very peculiar, but I never found actual atrophy in it (which, however, 
 is claimed for it by other authors), but rather an increase in the size of the 
 extensor hallucis longus. There appears, moreover, corresponding with the 
 prominence of this tendon and with the dorsal flexion of the great toe, an 
 occasional phenomenon in the hand ("main bote") in the form of a hyperex- 
 tension of the last phalanges. In this condition, slight muscular hyperten- 
 sion may be seen to exist in the parts affected, which otherwise is, how- 
 ever, usually absent in this disease. But, on the other hand, a considerable 
 hypotonia of the muscles is scarcely ever found. To explain these otherwise 
 astonishing, local muscular hypertensions, one must assume that they are 
 the consequence of continuous cramp-like attempts at equilibration, in which 
 the great toe plays the preponderant part. (The explanation of Cestan, that 
 the Babinski reflex, usually present in the disease (cf . below) has, so to say, 
 become permanent, is not sound) (Fig. 61). 
 
 Of other deformities we must mention the extraordinarily frequent 
 kyphosis, or kyphoscoliosis, which is probably only a secondary phenomenon 
 resulting from the lack of muscular balance. 
 
 Cutaneous sensibility as well as deep sensibility, are, strange to say, 
 retained as a rule. Only isolated instances of disturbances have been re- 
 ported in the literature; thus one of my patients made the characteristic 
 statement, that when swimming, he did not know where his legs were; as a 
 matter of fact, it turned out in the examination that coarser disturbances of 
 the sense of position were not perceivable. Oppenheim mentions a similar 
 case. Usually, however, both objective and subjective disturbances of 
 sensation are wholly lacking. Pains also are scarcely ever complained of. 
 The tendon reflexes are absent'm all definitely marked cases. Of the cutaneous 
 reflexes, the abdominal and cremaster reflexes are, as a rule, active, and the 
 plantar reflexes show the form of the so-called Babinski reflex, slow dorsal 
 flexion especially of the great toe, upon irritation of the planta pedis. 
 
 In regard to the cerebral nerves, one need only say that usually nystagmus 
 is found, corresponding to intention ataxia, and that speech becomes uncer- 
 tain early, now scanning, now explosive and above all it is distinctly slower 
 than normal. The other cerebral nerves are normal in pure cases, especially 
 those of the muscles of the eyes and the optic nerve. Disturbances of the 
 sympathetic nervous system also (vasomotor disturbances, sphincter affec- 
 tions, crises) are in contradistinction to tabes, as rare as pronounced trophic 
 changes. 
 
 The psyche remains normal. In one of my cases epileptiform convul- 
 sions were noted. As to combinations with bulbar symptoms, cf. below. 
 Concerning the pathogenesis of the individual symptoms, it is to be remarked, 
 that ataxia in its spinal components, is to be traced back to the process in the 
 posterior columns; in its cerebellar components, to the degeneration of the 
 
THE DIAGNOSIS OF FRIEDREICH'S ATAXIA 281 
 
 lateral cerebellar tracts. Whether the nystagmus is to be assigned to a 
 cerebellar tract, remains doubtful; for the speech disturbance also, a clear 
 anatomical foundation is lacking. The Babinski reflex we must probably 
 ascribe to the (slight!) lesion of the pyramidal tracts. It is very striking, 
 however, that notwithstanding the serious lesions of the posterior columns, no 
 disturbances of sensation are present. It seems, therefore, that the columns 
 of Goll are not absolutely necessary for the localization of peripheral sensa- 
 tions, and can even be replaced for their transmission; perhaps there is some 
 connection here with the fact that in spinal ataxia the short fibres are re- 
 tained, whereas the long fibres, contained for the most part in the columns 
 of Goll are largely destroyed (Dejerine) . 
 
 The course of the disease is very chronic, and it may drag on for 20 to 40 
 years. The symptoms increase slowly, but none of them in itself is apt to 
 bring on death, which is usually caused only by an intercurrent disease. 
 
 Correspondingly, the prognosis quoad vitam is not unfavorable, quoad 
 sanationem is absolutely bad. 
 
 The diagnosis of pure cases is easy. It is in itself easily distinguished 
 from all pure system diseases; from tabes, additionally, by the lack of all 
 disturbances in sensation and the absence of eye symptoms. Somewhat 
 more difficult may be its separation from multiple sclerosis, the intention 
 tremor of which may resemble ataxia; nystagmus, speech disturbance, youth, 
 and often but slight spastic phenomena occur in it also. But, as a rule, here 
 too, the difficulties are solved by the exact recognition of ataxia. 
 
 As great rarities some combined system diseases have been described, 
 which, like Friedreich's disease, presented a combination of symptoms of 
 the posterior and lateral columns, and in their course, much resembled it; 
 but, as a rule, the spastic phenomena predominated in them, and the ataxia 
 was slighter. Naturally, a sharp' division is often impossible in these cases. 
 
 On the other hand, we have still to consider its demarcation from the 
 disease picture of heredoataxie cerebelleuse (of Pierre Marie) . 
 
 In this disease, an ataxia develops in middle age, which exhibits an 
 obvious but not always an exclusive cerebellar character. To this are added 
 paralyses of the muscles of the eye, and nearly always atrophy of the optic 
 nerve, frequently psychic disturbances (imbecility), exaggerated tendon 
 reflexes. On the other hand, we miss the results of defects in muscular 
 balance, which correspond more nearly to spinal ataxia — namely, talipes 
 cavus, and scoliosis. The autopsy disclosed in pure cases of this disease 
 an abnormally small cerebellum, occasionally also some diminution in the 
 entire central nervous system, degeneration of the spinal tracts which stand 
 in connection with the cerebellum and of the posterior columns. But these 
 pure cases (also described by Marie von Londe, Miura, Switalski, and others) 
 are still more rare than pure cases of hereditary spinal ataxia. On the other 
 hand, in recent years, observations have been accumulating, in which symp- 
 
282 DISEASES OF THE SPINAL CORD 
 
 toms of both diseases are combined: Friedreich' 's disease with psychic symp- 
 toms, optic atrophy, Marie's disease with spinal ataxia, kyphoscoliosis, 
 talipes cavus. Even in one and the same family Friedreich's disease has 
 been observed in pure and in mixed form {Raymond). Still rarer are the 
 symptoms, which (cf. above) may be classified under the special group of 
 the "bulbar form"; disturbances in respiration, in so far as they do not come 
 from ataxia of the respiratory muscles, usually in the form of dyspnoea; 
 cardiac disturbances, recalling myocarditis (which according to French 
 authors, Lannois and Porot, frequently occur in the pure Friedreich as well), 
 vomiting. That these symptoms occur when the disease process is continued 
 to the medulla oblongata, is indubitably true; the formulation, however, of a 
 "beredoataxiebulbaire," seems nevertheless artificial. 
 
 Therapy, unfortunately, offers little, but luckily, with one exception, 
 there is but little pressing need of it in Friedreich's disease. This exception 
 concerns the removal of the ataxia, which, if it can not be entirely cured, can 
 at least be vigorously combated by practice of the most important muscular 
 movements in the form of FraenkeVs exercises. Very many patients, because 
 of the gradual development of the disease, succeed by themselves, in exten- 
 sively exercising the groups of muscles most necessary to their life. (For 
 more details on FraenkeVs exercises, cf. under the article "tabes".) After 
 all, therapy, except for a certain prophylaxis especially in infectious diseases 
 in families of a neuropathic diathesis or in those already affected by Fried- 
 reich's disease, can proceed only symptomatically. In longer treatment, 
 the same rules hold good as for the "tonic procedure" in tabes. 
 
 (c) Spastic Spinal Paralysis. 1 (Primary Lateral Sclerosis) 
 
 BY 
 
 FR. JAMIN (Erlangen) 
 
 The disease picture known as spastic spinal paralysis, the clinical expres- 
 sion of a purely motor system disease, a primary degeneration of the py- 
 ramidal tracts, is but rarely observed. It seems, however, that functionally 
 the pyramidal tracts are very easily affected, like the posterior columns of the 
 spinal cord, so that corresponding to their functional or anatomic injury, 
 the symptom complex of spastic spinal paralysis, or of spastic paraparesis, 
 temporarily or preponderantly steps into the foreground in many localized 
 or diffuse diseased conditions of the spinal cord. But even though it seemed 
 for a long time as if, according to anatomic findings, the sharply drawn 
 clinical picture of the disease described by Erb in 1875, following up Charcot's 
 hint, could not be characterized as a pure system disease, nevertheless in more 
 recent times a series of clinically as well as anatomically thoroughly investi- 
 
 1 Infantile spastic paraparesis (Little's and related diseases) is treated in the^chapter entitled 
 "Organic nervous diseases of childhood" by J. Ibrahim. 
 
SYMPTOMATOLOGY OF SPASTIC SPINAL PARALYSIS 
 
 283 
 
 gated cases have proven that there is actually a primary motor system disease 
 of the spinal cord, confined to the pyramidal tracts, with the symptoms of 
 spastic paralysis, and thereby not only a symptomatological, but also a 
 nosological individual place is secured for spastic spinal paralysis. 
 
 Symptomatology. — The spastic spinal paralysis of adults is, in its 
 purely systemic form, a very protracted disease (extending for years and dec- 
 ades after the onset of the first phenomena), which usually occurs in the 2nd 
 to 4th decade of life, progressing very gradually, but most constantly, and 
 characterized by but few subjective disturbances. As a rule, the first phenom- 
 ena appear in the lower extremities. 
 The patients notice, when walking, 
 that they become tired easily, and 
 that a certain heaviness and stiffness 
 in the legs interferes with the more 
 active movements, especially such as 
 running and jumping. Should their 
 vocation make greater demands upon 
 them, unpleasant sensations of 
 weariness appear, occasionally, too, 
 a feeling of cold .in the legs (they 
 are more than ordinarily difficult to 
 move), but violent pains or pares- 
 thesias do not occur. As the dis- 
 ease advances, the patients become 
 more and more helpless; they can 
 move forward only by means of 
 small, dragging steps, and become 
 so stiff in the legs, that when they 
 meet obstacles, they are no longer 
 able to avoid them quickly, therefore 
 
 they stumble easily, and, occasionally, fall. A number of the patients can, 
 with the aid of canes, preserve for an extraordinary length of time a limited 
 walking ability; others, on the other hand, because of the increasing stiff- 
 ness and weakness of the legs, and the supervening contractures, finally 
 become bedridden. 
 
 Examination enables us to recognize quite early the spastic-par etic 
 symptom complex. 
 
 Particularly striking is the stiffness of the legs, the hypertonia of the 
 muscles, which is exhibited even in conditions of rest, but more in active and 
 more especially in passive attempts at movement. It leads to a functional 
 stiffening of both legs, in the joints of the hip, knee, and foot, which occasion- 
 ally is more marked at the beginning, on one side, than on the other. The 
 stiffness prevails in the extensors, the gluteal muscles, the adductors, the power- 
 
 FiG. 62. — Spastic spinal paralysis; posture in 
 sitting. {After Schoenborn and Krieger.) 
 
284 DISEASES OF TEE SPINAL CORD 
 
 ful mass of the quadriceps muscles, and in the musculature of the calves of the 
 legs. In advanced cases, therefore, the patients can not flex their legs 
 sufficiently at the hips and the knees, to be able to sit down (Fig. 62). When 
 trying to sit, the patient leans against the edge of the chair, almost as stiff as a 
 stick, while the legs, almost at full extension at the knee, remain floating in the 
 air. It is only with great effort and with the use of considerable strength, 
 that passive flexion in the hip- or knee-joint may be carried out. Quickly 
 executed attempts at passive movements merely increase the muscular 
 resistance. In the ankle-joint, the stiffness may progress to such an extent, 
 that, as a result of the "muscular joint ankylosis" (v. Strilmpell) , active and 
 passive movements of the foot become virtually impossible. Frequently the 
 extension of the leg as a whole is so forcefully produced, so fixed by muscu- 
 lar tension, that the patient can be lifted from a supine position, by one leg, 
 almost like a board. Even in passive lateral movements of one leg, the pel- 
 vis and the other leg follow, whereas with careful yet forceful overcoming of 
 the muscular tension, it may be demonstrated that the possibility of move- 
 ment is retained in the apparatus of the joints and ligaments. 
 
 The fact that the patient, in spite of such an increase of the functional 
 obstacles, is still able to walk for considerable distances in an upright posi- 
 tion, shows that no decided paralysis of the legs is present. As a matter of 
 fact, in movements of resistance, the muscles of the leg, and especially the 
 extensors, prove, very frequently, to be still powerful, and we must ascribe 
 preponderantly to the increased muscular tension, the fact that the active 
 movements are so troublesome, so hampered and slow. Nevertheless, in 
 many cases, a diminution of the actual strength of the legs is also demonstrable, 
 independent of the obstacle of tension, especially in the abductors and 
 flexors in the hip-joint, the flexors in the thigh (biceps, semimembranosus 
 and semitendinosus) and in the dorsal flexors of the foot. This paresis of the 
 flexors, i. e., the shorteners of the leg, combined with actually greater strength 
 in the extensors or lengtheners of the leg, and the hypertonia prevailing in 
 the latter, influences the posture and the gait in spastic paraparesis character- 
 istically: the patients in order not to fall backwards, because of the extension 
 of the hip, move the weight of the body far forwards, the outstretched hands 
 at the same time seeking new points of support with the aid of canes or by 
 grasping furniture (Fig. 63). Both legs remain in full extension, the feet in 
 plantar flexion; and frequently the patients step only on the balls of the toes, 
 until by standing longer, the weight of the body overcomes the tension of 
 the muscles of the calf of the leg. The thighs are adducted, the feet 
 turned slightly inward. In walking, the legs can scarcely be raised or 
 flexed at the knee; in almost full extension, one after the other is pulled 
 forward laboriously in a short circle, the tips of the toes are allowed to drag, 
 or trailed forward and thus a short step is taken. At the same time, 
 because of the overbalance of the adductors, the knees and the balls of 
 
SYMPTOMATOLOGY OF SPASTIC SPINAL PARALYSIS 285 
 
 the toes frequently rub against each other on the medial side. Because 
 of this the shoes are worn off, especially in the foremost part of the sole, 
 and on the inner side of the tips of the toes. On sandy ground, slightly 
 curved, almost parallel lines show the tracks of the short, dragging steps. 
 And since walking is accomplished rather by dragging the extended leg 
 along, the upper part of the body being bent forward, than by means of an 
 elastic lifting swing, and replacement of the leg, as is performed by healthy 
 individuals, it produces a characteristic rhythmic scraping noise in short 
 intervals. 
 
 Next to the phenomena of hypertonia and paresis in certain muscle 
 groups, the tendency to associated movements is characteristic of the spastic 
 changes. Their common foundation lies probably in the fact that the 
 movements of the legs, interfered with by weakness and increased muscular 
 tension, are synergetically possible only by the co-operation of larger groups 
 of muscles, trained to perform primitive movement complexes, whereas the 
 volitional suppression of these combinations of movements, in favor of finer, 
 isolated movements, which were learned during the course of ,life, though 
 possible to the normal man, is now impossible. Especially clear does this 
 disturbance appear in spastic individuals in the movements of the foot and 
 toes. Whereas, in many cases, an isolated voluntary movement of the 
 great toe, or of the entire foot, e. g., dorsal flexion can no longer be performed 
 by the patient, strong active flexion of the limb at the hip or the knee is seen 
 to be accompanied by a strong, insuppressible dorsal flexion .of the foot, 
 with conspicuous protrusion of the tendon of the tibialis anticus, {StriimpelV s 
 tibial phenomenon). In lifting the whole leg, often also during attemps at 
 walking, a very marked dorsal flexion of the great toe appears {StriimpelV s 
 toe phenomenon) , which can not be produced isolated to the same excessive 
 degree by active effort. Flexion of the leg at the hip, which is almost im- 
 possible when the leg is in extension, is also far more easily accomplished 
 against resistance if at the same time the leg proper be flexed and the foot 
 put in dorsal flexion, and, on the other hand, extension of the hip is difficult 
 without simultaneous extension of the knee and plantar flexion of the foot. 
 
 The permanent hypertension and excessive excitability in individual 
 muscles or groups of muscles, not only during voluntary functional activity 
 but also to reflex stimuli, leads usually to a slight change in the permanent 
 position of the lower extremities, to contractures. Usually the extended 
 position of hip and knee is accompanied by plantar flexion with slight inward 
 rotation and supination of the foot and dorsal flexion of the great toe, with 
 moderate adduction of the thigh. In other cases, there appear, especially 
 in long persisting affections, flexion contractures in the hip- and knee-joints 
 in spite of the continuation of the equino-varus position of the feet and the 
 prominence of the tendon of the long extensor of the great toe. 
 
 Without exception spastic spinal paralysis is accompanied by a marked 
 
286 DISEASES OF THE SPINAL CORD 
 
 exaggeration of the tendon and periosteal reflexes in the lower extremities. 
 Occasionally it is difficult to demonstrate this exaggeration of the reflex 
 because of the enormous muscle tension, but even then it is usually possible 
 with the limbs in a suitable position of rest; the patellar and a chilles tendon 
 reflexes may be elicited; marked patellar and ankle clonus is very frequently 
 
 Fig. 63. — Spastic spinal paralysis; posture in standing. (Erlangen Medical Clinic.) 
 
 also induced in this condition. The reflexogenous zones are considerably 
 enlarged; the reflex movements may occur on the other side as well, and in 
 muscles at some distance. Xot rarely, under the influence of weight alone, 
 when the legs are hanging down, or after trifling spontaneous and reflex 
 
SYMPTOMATOLOGY OF SPASTIC SPINAL PARALYSIS 287 
 
 movements, one leg, or even both, are thrown for some little time into clonic 
 tremulous reflex movements, which, like genuine reflex spasms, can be sup- 
 pressed only with considerable trouble by fixation of the limb for some time, 
 or by vigorous stimulation of the skin. These spasms become very annoying 
 to the patient. In standing and walking, also, there appears often, as a 
 consequence of the abnormally vigorous reflex excitability of the muscles of 
 the calf of the leg, and the peculiar position of the toes, a reflexly conditioned 
 quivering and trembling over the entire body. Upon tapping the side of the 
 dorsum of the foot, there frequently appears, instead of the normal dorsal 
 flexion, plantar flexion of the toes (K. Mendel). 
 
 Of the cutaneous reflexes, those of the abdomen and the cremaster-reflex 
 occasionally, appear to be diminished, but usually suffer no change worth 
 mentioning. The cutaneous reflexes in the legs, especially those of the 
 sole of the foot, are, as a rule, exaggerated and changed in a typical manner : 
 lively flexing movements of the entire limb, or manifold combined reflex 
 muscular contractions follow upon energetic stimulations of the skin. 
 Especially characteristic is the Babinski sign, slow dorsal flexion of the great 
 toe, upon irritation of the sole of the foot. This important evidence of 
 spastic disturbance after lesion of the pyramidal tracts has, however, been 
 absent in some cases of pure lateral sclerosis, or found in a less clearly defined 
 form. The appearance of a powerful reflex dorsal flexion of the foot and 
 toes upon irritation of the inner surface of the leg proper is also of great 
 diagnostic importance (Oppenheim's dorsal leg phenomenon). Occasion- 
 ally also, especially in younger patients, there appears, upon irritation of the 
 sole of the foot, a spreading of the toes with particularly energetic abduction 
 of the small toe (Babinski' s fan phenomenon), which may, moreover, be 
 combined with peculiar clonic twitchings of the toes. 
 
 In the ascending progress of the disease, the phenomena of hypertonia, 
 paresis, and exaggeration of the tendon and periosteal reflexes extend also 
 to the upper extremities. Then the arms, too, show changes in posture, with 
 abduction of the upper arm, flexion of the forearm and pronation of the 
 hand, while the muscular weakness is evinced preponderantly in lifting 
 and extending the arm and on dorsal flexion of the hand. The arm reflexes 
 are extraordinarily active; hand clonus may be brought about by brusque 
 dorsal flexion of the hand and flexion of the fingers by tapping the back of the 
 hand. The very rare skin reflexes in the arms, shown by abduction and 
 extension of the arm, and spreading of the extended fingers upon irritation 
 of the palm of the hand, have been but little studied. 
 
 Finally, the motor cerebral nerves may also be attacked; we may conclude 
 that they are spastically disturbed from the presence of a strangely tense 
 expression in the face, from difficulty in and inhibition of speech, from diffi- 
 culty in the movements of the muscles concerned with chewing and swallow- 
 ing, as well as from attacks of spasm of the glottis. In such cases, the loss 
 
288 DISEASES OF THE SPINAL CORD 
 
 of central inhibition of expressive and emotional movements, is evinced in 
 remarkable fashion by the appearance of compulsory laughing and crying, 
 induced by slight stimulation and sometimes lasting without cessation, as 
 long as one occupies one's self with the patient. When the disturbance of the 
 entire central motor system of the pyramidal tracts is extensive, the disease 
 usually shows a certain tendency to extend to the spinal motor centers. 
 This may be recognized by the localized muscular atrophy that appears in 
 places, especially in the hands (cf. also Fig. 64 a). These cases represent 
 transitions to amyotrophic lateral sclerosis. In pure cases of primary 
 lateral sclerosis, the conservation of the muscles and the absence of all 
 localized atrophy is characteristic. 
 
 In all cases of spastic spinal paralysis, the functions of superficial and 
 of the deep sensibility remain absolutely intact. No vasomotor nor trophic 
 disturbances are observed, the organs of sense suffer no sort of injury, 
 disturbances of the bladder and intestinal tract are absent. At most, when 
 the spasms are very severe, possibly because of the participation in the 
 general rigidity of the muscles of the floor of the pelvis, there may result 
 difficulty in micturition. 
 
 Pathological Anatomy. — The symptom complex of spastic spinal 
 paralysis is produced by a degeneration of the pyramidal tracts, which, in 
 the pure cases, may be traced through the entire spinal cord, in the lateral 
 columns, and even in the anterior columns (Fig. 64). The manner and 
 duration of the disease determine the spread of this degeneration to the 
 higher sections of the central motor-conducting tracts. Occasionally it 
 extends beyond the pyramidal crossing to the pons, the cerebral peduncles 
 and the internal capsule. The ganglion cells of the psychomotor fields of the 
 cerebrum, are, as a rule, found intact, nor does the lesion extend to the motor 
 ganglion cells in the spinal centers of the anterior columns, that is, their 
 peripheral branches in the anterior roots and the peripheral nerves. Even 
 the muscles themselves prove upon anatomic examination to be intact; in 
 fact the muscle fibres are especially voluminous and hypertrophic in the 
 regions most affected by the hypertonia and the exaggeration of the reflexes. 
 Transitions to combined column diseases of the spinal cord may be observed, 
 due to the presence of a slight systemic degeneration in the posterior columns, 
 especially affecting the funiculi graciles (Goll), but in these cases, owing to 
 the precedence in time and the permanent preponderance of the functional 
 disturbances of the pyramidal tracts, the spastic paretic symptom complex 
 comes so completely to the fore, that the phenomena of sensory loss of 
 function, ataxic disturbances, and diminution in 'the tendon reflexes seem 
 to be wholly concealed. 
 
 ^Etiology. — The familial and hereditary occurrence of a pure spastic 
 spinal paralysis, i. e., primary lateral sclerosis, observed by v. Strilmpell and 
 others, places the possibility of a definitely endogenous occurrence of this 
 
ETIOLOGY AND DIAGNOSIS OF SPASTIC SPINAL PARALYSIS 289 
 
 systemic disease beyond doubt. But also in the isolated cases, probably a 
 deficient development of the tract, which later is destroyed, gives the founda- 
 tion for the elective disease, which is frequently excited in more mature age 
 under exogenous influences, like intoxications, infections, nutritional dis- 
 turbances (anemia), exhausting over-exertions, and traumatisms with their 
 
 FlG. 64. — Primary pyramidal-lateral colu m n sclerosis in spastic spinal paralysis. Weigert- 
 Pal staining, a, Upper cervical cord. Besides the degeneration of the pyramidal lateral tracts a 
 slight atrophy of the anterior columns exists; b, lower cervical cord; c, upper dorsal cord; d, 
 lower dorsal cord; e, lumbar cord. (Erlangen Medical Clinic?) 
 
 consequent phenomena. The injuries produced as the after-effects of 
 syphilitic infection, not infrequently remain for a long time confined to the 
 pyramidal tracts; this is likewise reported of chronic lead poisoning, of 
 eating lathyrus seeds (lathyrus sativus, lathyrus cicera, chick pea), of 
 pellagra occurring in those living upon Indian corn, of phenomena conse- 
 quent to the puerperium and to other infectious diseases. 
 
 Differential Diagnosis. — The clinical picture of spastic spinal paralysis 
 is so characteristic in its combination of muscular rigidity, reflex exaggera- 
 19 
 
2 9 o DISEASES OF THE SPINAL CORD 
 
 tionj and typically distributed pareses and contractures, that it can scarcely 
 fail to be recognized symptomatologically. The disturbances that take place 
 in active and passive motility, the increase in the tendon reflexes and the 
 pseudoclonus which are met with in hysteric and psychopathic patients 
 (medico-legal accident patients) can not easily be mistaken for it if the 
 arrangement and localization of the disturbances in movement are carefully 
 studied, the cutaneous reflexes (BabinskV s and Oppenheim's signs) carefully 
 tested and the associated movements taken into consideration. In this 
 respect serious difliculties in diagnostic judgment arise only when the 
 organic disturbances are concealed or blurred by a hysteria that has super- 
 vened upon the organic disease. 
 
 More difficult and often answerable only after long and thorough observa- 
 tion is the question, whether the spastic-paretic symptom complex is pro- 
 duced by a primary pure lateral sclerosis, or whether we are dealing with a 
 symptom of some other cerebral or spinal disease. The diagnosis of a 
 primary lateral sclerosis can and ought to be made only, then, if after careful 
 consideration of the subjective disturbances, and after minute examination 
 of the sensibility, co-ordination, reflexes, the trophic and vasomotor relations, 
 the spinal column and the cerebro-spinal fluid, the cerebral nerves and the 
 organs of special sense (the ophthalmoscopic findings), the urogenital and 
 intestinal functions, especially, too, after complete clearing up of the previous 
 history, especially of the course of the disease, a symptomatic occurrence of 
 spastic paresis in myelitis, compression myelitis, cerebro-spinal syphilis, 
 hydrocephalus, spinal gliosis, progressive paralysis, multiple sclerosis, 
 encephalitis and other diseases, may be excluded. 
 
 In old age there develops occasionally a spastic paraparesis with contrac- 
 tures, typical reflex changes and a disturbance in walking, which causes the 
 gait to resemble that of the spastic because of its stiffness and weakness, 
 which is accompanied by an attenuation of the pyramidal tract areas in the 
 lateral columns and is probably brought about by nutritional disturbance 
 in the spinal cord induced by arterio-sclerotic changes, though it may also 
 have its seat in the cerebral cortex. 
 
 The prognosis of spastic spinal paralysis naturally depends upon the 
 causes of the symptom complex. In the cases of pure lateral sclerosis, it is 
 not unfavorable, in that while the course of the disease is progressive, it is 
 frequently very protracted. The disease sometimes, too, may come to a 
 standstill for years and, as a rule, does not affect the vital nervous 
 functions. 
 
 The therapy can, to a certain degree, at least, be causal, inasmuch as 
 it seeks to prevent, by rest and nerve economy, a further exhaustion of the 
 weakened motor system. Therefore the patient, even at the beginning 
 of the disease, must avoid all movements of the body, that stir him up to 
 overhaste or over-exertion, or which by the tendency to make him fall, might 
 
TREATMENT OF SPASTIC SPINAL PARALYSIS 291 
 
 cause traumatic injuries. He must also avoid long walks and mechanical 
 occupations requiring the frequent repeated use of certain muscles. On the 
 other hand, by careful practice in walking and standing, at first, with the 
 assistance of passive movement by stimulating the cutaneous reflexes, which 
 often materially aid the flexing movements of the legs, the faculty for active 
 movement can be improved methodically and by becoming accustomed to 
 the altered circumstances, some degree of freedom in movement may be 
 attained. It is desirable as well that the patient should become aware of the 
 possibility of substitution movements, which may almost always be used, and 
 perfect these gradually by use; for this, however, if he remains a long time 
 in bed, he will lose both the opportunity and energy. Since, with the weak- 
 ening of the cutaneous irritability and the lessening of the effect of weight 
 in the warm bath, the stiffness in the muscles and the spasticity gradually 
 give way, the persistent use of protracted warm baths is to be recommended. 
 At the same time, the patients may derive benefit from the vivifying influence 
 of carbonic acid baths at home, or at suitable health resorts. Careful 
 massage, and especially passive aid in attempts at active movement, even 
 in the bath itself, may further the motility, at least in the first stages of the 
 disease. With the lowering of the tonic excitability by medicines (morphine, 
 narcotizing injections into the dural sac) one will have to be most conserva- 
 tive, considering the duration of the disease. It is far more important to 
 develop by suitable means the active powers of the patient in overcoming 
 muscular resistance. For this purpose one may use moderate application 
 of the constant current, while the stimulation of faradization and great 
 changes in current are to be avoided. Spastic-paretic patients doubtless 
 often recover their ability to move to a signal extent, if they leave externally 
 unfavorable environments and go to a place where they can have good 
 physical care and better nourishment. 
 
 The results of orthopaedic surgery have not been, so far, very encourag- 
 ing in spastic pareses, and especially in spastic spinal paralysis, unless we 
 are concerned about getting rid of troublesome phenomena of secondary 
 importance, as contractures. Neither through apparatus for support and 
 posture, nor by cutting tendons and muscles, nor by plastic and grafting 
 operations, can one effectively combat the disproportion between the 
 increased reflex muscle tonus in some and the diminution of the voluntary 
 development of strength in other groups of muscles. Recently, it has been 
 attempted successfully, after laminectomy, to resect part of the posterior 
 roots in corresponding selection, and thereby, without causing phenomena 
 of serious functional loss, to weaken the reflex exaggerations and the hyper- 
 tonia to such an extent, that the active capacity for movement becomes 
 more free (Forster). In desperate cases, this method must be considered 
 as the ultima ratio, if there be a possibility of strict aseptic procedure and 
 most careful after-treatment. 
 
292 DISEASES OF THE SPINAL CORD 
 
 (d) Amyotrophic Lateral Sclerosis 
 
 BY 
 
 FR. JAMIN (Erlangen) 
 
 The relatively rare disease, amyotrophic lateral sclerosis, affecting men 
 more frequently than women, is a comparatively rapidly advancing destruc- 
 tion of almost the entire motor central nervous system, occurring in mature 
 age — between the thirtieth and fiftieth years. In this affection not only the 
 cortico-spinal motor conduction path perishes, especially in its most impor- 
 tant component, the pyramidal tracts, but also the spinal motor centers in 
 the gray anterior horns of the spinal cord, and the nuclei of the motor cerebral 
 nerves situated in the medulla oblongata. In the clinical picture the injury 
 of the first central motor neuron causes the spastic paretic symptom complex, 
 as is shown in a pure form in spastic spinal paralysis, with muscular rigidity, 
 exaggeration of reflexes and diminution in ability to perform voluntary 
 movements — while the loss of the second peripheral neuron is responsible 
 for the symptom complex of paralysis and wasting of the muscles with the 
 reaction of degeneration. 
 
 One would think that the latter should bring about a disappearance of 
 the spastic phenomena. But since to the extensive injury of the reflex 
 inhibiting cortico-fugal motor paths, even in the advanced cases, there is 
 opposed only a partial injury of the spinal and bulbar nuclei, which is 
 confined to the innervation sphere and the trophic influence of special 
 groups of muscles, even parts of muscles, there exists in amyotrophic lateral 
 sclerosis an intimate union of spastic symptoms with paralysis and so-called 
 "degenerative" muscular atrophy, which is the distinctive characteristic of 
 this disease. Nor does the lesion affect all parts of the motor system in the 
 same way from the beginning, but, on the contrary, according to the localiza- 
 tion of the injury, definite types may be distinguished, which, in the majority 
 of cases, denote successive stages of the disease: 
 
 i. The paralysis and muscular atrophy in the arms combined with 
 spastic disturbances. 
 
 2. The disturbances in the movements of the legs, corresponding more 
 particularly to the spastic symptom complex. 
 
 3. The atrophic paralysis in the area of the motor cerebral nerves appear- 
 ing also with symptoms of spasms, bulbar paralysis. 
 
 Some observations of its occurrence as a familial affection, permit us to 
 conclude that the cause of this exhaustion associated with degeneration of 
 the motor nervous system at the time of active life, may rest upon a con- 
 genital weakness of this system. Physical over-exertions and the influence 
 (interpreted with difficulty) of colds and traumatisms are stated as factors 
 in exciting the disease. It is not known whether a specific uniform cause of 
 the disease is predispositional to its development. 
 
AMYOTROPHIC LATERAL SCLEROSIS 
 
 2 93 
 
 Symptomatology and Course of the Disease. — The insidious develop- 
 ment of the disease makes itself in most instances first known by a functional 
 disturbance of the upper extremities. In occasional cases, however, the 
 disease, even at the beginning shows a unilateral type, and attacks the arm 
 and leg on one side at approximately the same time. Even when it begins 
 in the arms the disturbance on the one side (mostly the right) as a rule 
 precedes that on the other by some months. 
 
 The patients themselves notice, that their arms and hands become 
 weaker and less skilful at their usual work, that they are easily fatigued, 
 without the occurrence, however, of pains or paresthesias. In the further 
 course, after some months, or only after a 
 space of a year, there is noticeable a disturb- 
 ance in gait, an unusual fatigue after walk- 
 ing, with stiffness, clumsiness, and weakness 
 in the legs. 
 
 The objective findings exhibit early in the 
 arms increased muscular tension with a ten- 
 dency to active contractures, especially with 
 regard to an adduction of the upper arm, 
 flexion of the forearm, pronation of the hand 
 and flexion of the phalanges. Synchronously 
 the characteristic muscular atrophy is found, 
 at first, in the small muscles of the hand. 
 The balls of the thumbs and of the small 
 fingers become flatter. The wasting of the 
 interossei gives rise to significant deep hollows 
 on the back of the hand, the adductor pollicis 
 becomes thin (cf. Fig. 65). Next, the ex- 
 tensors in the forearm become atrophic 
 
 followed by the long muscles of the thumb, then, too, the rest of the mus- 
 cles of the radial region and, since the flexors of the hand and the fingers 
 are relatively better preserved and remain more effective, there results a 
 claw-like position or flexion contracture of the fingers. Afterwards, the 
 deltoid and the triceps become visibly atrophic, finally, the rest of the muscles 
 of the arms and the shoulder girdle. Associated with the muscular wasting, 
 there is a muscular weakness which ends in complete paralysis, and even the 
 muscles which have retained their volume relatively well, frequently evince a 
 considerable diminution in power. 
 
 Upon careful observation of the diseased muscular parts one sees, 
 especially plainly in connection with attempts at movement, a lively play of 
 fibrillary and fascicular twitchings. The electric excitability is lowered, but for 
 the most part only in quantitative proportion to the wasting of the muscle 
 fibres. In the more severely atrophied muscles, however, especially in 
 
 Fig. 65. — Hand in amyotrophic 
 lateral sclerosis. Flexing contracture 
 of the end phalanges, II-V. At the 
 place of the adductor pollicis and the 
 interossei visible hollows. ( Erlangen 
 Medical Clinic.) 
 
2Q4 DISEASES OF THE SPINAL CORD 
 
 those of the hand, a total or at times only a partial reaction of degeneration 
 may be observed. 
 
 The tendon and periosteal reflexes are always very markedly exaggerated 
 in the weakened and atrophied arms. From the radius and the ulna, and 
 from the tendons of the upper arm muscles, especially in the triceps, lively 
 reflex muscular twitchings can be evoked; even wrist clonus is observed. 
 
 Sensibility remains absolutely undisturbed in all its qualities. 
 
 While in the arms, the muscular wasting and the paralysis preponderate 
 early over the spastic phenomena, without wholly crowding them out, the 
 lower extremities, should they share in the further course of the affection, 
 exhibit the reverse. Here, the spastic-par etic phenomena step quite decidedly 
 to the fore, whereas the muscular atrophy, fibrillary twitchings and changes in 
 electrical excitability appear either not at all, or relatively late, and then 
 mostly in the muscles of the leg proper. The laborious, dragging, sliding 
 gait, the paresis of the flexors, group action of the extensors and flexors of the 
 limb, and associated movements (tibial and toe phenomenon) spontaneous 
 clonus and marked increase of all tendon reflexes, changes in the cutaneous 
 reflexes (Babinski's great toe reflex and Oppenheim's leg reflex), finally, too, 
 contractures unite with the general hypertonia of the muscles of the leg, 
 which is made still greater by skin stimuli and motion, to make the clinical 
 picture of spastic paraparesis. In the trunk, and the legs, also, disturbances 
 of sensation are altogether absent. With the exception of a slight difficulty 
 in urination and defecation (constipation) due to spasms in the musculature 
 of the pelvic floor, there appear no bladder or intestinal disturbances. 
 
 After the patient, by a combination of spastic paraparesis and a more or 
 less complete inability to use the upper extremities, has already reached a 
 very helpless condition and has become bedridden, the disease becomes 
 really tormenting and even dangerous, when, after one or two years, the last 
 group of symptoms, which, in many cases, however, has developed much earlier, 
 namely, that of bulbar paralysis, is added to the other symptoms. 
 
 Now, with the paralysis that appears in the area of the motor cerebral 
 nerves, and the muscular atrophy, speech disturbances develop; speech 
 becomes first nasal and difficult to understand, is articulated worse and 
 worse, finally becomes aphonic; there appear difficulty in swallowing, paralysis 
 of the soft palate, of the tongue with visible atrophy and fibrillary twitchings, of 
 the muscles of chewing, and of the facial muscles, especially in the lower half 
 of the face. The lips become thin and can no longer be raised and pursed at 
 will, whereas the expressive movements of the face are frequently retained. 
 In these regions also side by side with and often before the appearance of 
 atrophy and paralysis, the loss of cerebral inhibition makes itself felt in many 
 ways: the masseter reflexes are exaggerated; the muscles of the face are tense, 
 often spasmodically distorted; glottis spasms appear, sudden and apparently 
 causeless bursts of emotion (obsessional laughing and crying) with retention 
 
AMYOTROPHIC LATERAL SCLEROSIS 295 
 
 of the intellect indicate the increase in reflex excitability. In the wrinkled 
 tongue, which is moved with difficulty and in the lips and the muscles of the 
 pharynx the reaction of degeneration or at least slow contraction upon galvanic 
 stimulation may be demonstrated. 
 
 Only the muscles of the eye are, for the most part, spared. The mechan- 
 ism of respiration may be impaired by the co-affection of the diaphragm and 
 the muscles of the ribs, so that the patient finally succumbs to a respiratory 
 paralysis, unless the secondary phenomena of paralysis of the throat and the 
 swallowing apparatus (aspiration pneumonia) and the disturbances in nour- 
 ishment, necessarily connected with the bulbar disturbances, have already 
 caused death after a few years. 
 
 The course of the disease and the order of the appearance of the individual 
 symptoms differ in different cases, depending upon whether the lesion of the 
 pyramidal tract or the loss of the motor nuclei in the medulla oblongata and 
 in the spinal cord has temporal precedence, and according as to which retains 
 the preponderant importance for a time, or permanently. Thus, there are 
 found transitional forms to spastic spinal paralysis, in which the atrophic 
 paralysis remains altogether undeveloped, to progressive muscular atrophy, 
 in which, from the beginning, the paralysis with muscular wasting conceals 
 the spasticity, or stops it in the final stage of the disease, and to progressive 
 bulbar paralysis, in which, early, the paralysis of the cerebral nerves, 
 accompanied by slight spastic disturbances in the extremities, comes to the 
 foreground. 
 
 Pathological Anatomy. — Transverse section of the spinal cord (Fig. 
 66) shows in amyotrophic lateral sclerosis very distinctly, especially in the 
 cervical segment, the combination of atrophy of the pyramidal tracts with 
 atrophy of the anterior horns, corresponding to the spastic atrophic paralysis 
 which is most pronounced in the arms. The pyramidal tracts have wasted 
 in the lateral column and, occasionally, also in the anterior column, not 
 here only but in their entire spinal course, and in their ascending way 
 through the medulla oblongata, the pons up to the cerebral peduncle and 
 the inner capsule. Slight wasting of fibres, too, is noticeable in the rest of 
 the area of the antero-lateral column, in some cases, also in the medial 
 posterior column, without the appearance, however, of noticeable sensory 
 phenomena of functional loss. The gray matter of the anterior columns 
 becomes reduced, especially in the cervical segment, by the wasting of its 
 network of nerve-fibres with the exception of the reflex collaterals (Oppen- 
 heim) and by destruction of a large part of the large motor ganglion cells. 
 The anterior roots, too, usually become smaller. A corresponding nuclear 
 wasting may be observed also in the bulbar centers of several cerebral nerves, 
 in the nucleus of the hypoglossus, the vagus-accessorius, the motor part of the 
 trigeminus, and in a slighter degree also in the facial. The psychomotor 
 cortical fields and their nearest connection paths, also, have not always been 
 
296 DISEASES OF THE SPIXAL CORD} 
 
 found intact. Slight proliferation of the supporting connective tissue re- 
 places the nervous elements that are lost. 
 
 To the extensive loss of the spinal motor centers, correspond degenera- 
 tions in the peripheral motor nerves, which, however, are difficult to find in 
 the mixed nerve trunks, and also because of the mingling of fibres still 
 capable of conduction with those that are already incapable. In the atro- 
 phied muscles of the arm, the •wasting of muscular fibres, resulting in some 
 places in complete loss of the contractile substance with increase of the 
 muscle nuclei and the interstitial connective tissue, mav be demonstrated 
 
 
 FiG. 66. — Amyotrophic lateral sclerosis. Weigert staining. Uppermost cervical cord. The 
 pyramidal tracts show degeneration in the anterior and lateral columns. A slight degeneration is 
 to be found also in Goll's column. The gray anterior columns have almost disappeared. {After 
 Schmaus.) 
 
 easily, accompanied under certain circumstances by profuse infiltration 
 of fat. In some muscles, affected only by partial atrophy, more or 
 less atrophied muscular fibres are mingled with those that are well 
 preserved. 
 
 In the differential diagnosis of amyotrophic lateral sclerosis it is 
 especially important to note that this disease is strictly confined to motor 
 functional disturbances. Testing the sensation and consideration of the 
 subjective troubles, which are not to be explained by disturbances in move- 
 ment, like pains and paresthesias, make easily possible a distinction from 
 the other spinal troubles, which like syringomyelia, spinal tumors, cervioal 
 pachymeningitis, myelitis localized in the cervical segment, or syphilis dna 
 tuberculosis of the cord may be accompanied by similar atrophic paralyses 
 
SPINAL PROGRESSIVE MUSCULAR ATROPHY 297 
 
 in the arms with spasticity in the legs. Differentiation from the rest of the 
 system motor diseases is made possible by the subacute course and the 
 commingling of spastic phenomena with cervical and bulbar atrophic paralyses. 
 In regard to this the increase of the periosteal reflexes in the atrophied fore- 
 arms is especially significant for the myatrophy connected with lateral 
 sclerosis; it does not appear in like manner in spinal muscular atrophy, nor 
 in other lesions restricted to the anterior horns, due to spinal gliosis or 
 hgematomyelia. In multiple sclerosis, which not infrequently simulates 
 motor systemic diseases, localized muscular atrophies are scarcely to be 
 observed in similar arrangement and development; it may be recognized 
 frequently, besides, by proof of disturbances in co-ordination and due 
 consideration of the eye findings even in the atypical cases. 
 
 The prognosis of amyotrophic lateral sclerosis is in every respect un- 
 favorable. Irresistibly, and apparently indifferent to external influences, 
 the disease proceeds to its fatal end. 
 
 The treatment can therefore only be symptomatic. Consideration and 
 good care are obviously necessary because of the helpless condition of the 
 patient. In the final stages, the patients, under certain conditions, have to 
 be fed through a tube. The spasticity and contractures may be combated 
 by baths, careful massage, also by suitable bandages; because of the progres- 
 sive nature of the disturbances, it is better to abstain from operative treat- 
 ment. The employment of the constant current in the region of the nape 
 and along the spinal column, also in the area of the atrophied muscles, and 
 the administering of nourishing foods and strengthening medicines, etc., is 
 to be all the more recommended since only steadily continued therapeutic 
 procedures may spare the patient, who with unimpaired intellect is gradually 
 perishing, a clear insight into his condition. 
 
 (e) Spinal Progressive Muscular Atrophy (Duchenne-Aran) 
 
 BY 
 
 FR. JAMIN (Erlangen) 
 
 The spinal motor centers in the gray anterior horns of the spinal cord 
 with their thick network of nerve fibres and the groups of motor ganglion 
 cells are the last central terminal stations for the muscles, through which the 
 stimulations emerging from the superior centers of the brain, the cerebellum 
 and the subcortical ganglia, as well as the reflex impulses created in the 
 spinal cord itself must take their way. Their destruction causes a secondary 
 degeneration of the peripheral motor nerve fibres and a complete exclusion 
 of the attendant contractile muscular fibres from every voluntary and reflex 
 activity, that is, not only a cessation of the motor activity, which has visible 
 results, but the loss of every function, and therefore also of the tension that 
 
298 DISEASES OF THE SPINAL CORD 
 
 exists in times of rest. The consequence of this loss of all functional stimula- 
 tions, is, for the muscle, a considerable diminution of the processes necessary 
 to its existence, of circulation and nourishment. Since such a muscle now 
 lacks the power of an up-building restitution to the natural loss of its sub- 
 stance, a power afforded it by its normal specific function, it perishes after 
 the loss of its innervating and thereby trophically acting spinal center, in a 
 slow way and, if considered for short periods of time, scarcely perceptibly, 
 yet irresistibly, until the complete process of destruction, atrophy, is 
 reached. 
 
 An injury of the anterior columns of the spinal cord with the corresponding 
 secondary phenomena in the muscles — paralysis, loss of reflex excitability and 
 muscular wasting — occurs in numerous diseases of the spinal cord. Even 
 among the system motor diseases of the spinal cord it has already been 
 mentioned as a partial phenomenon of amyotrophic lateral sclerosis. Pure 
 primary degeneration of the spinal motor centers, the premature using-up and 
 gradually progressing destruction of these important motor central stations 
 is, however, observed in the very rare disease picture of spinal progressive 
 muscular atrophy. 
 
 An entirely sharp separation of the manifold forms, under which a con- 
 genital weakness of the nervous and muscular apparatus of motion appears 
 clinically, can certainly not be made. As we have seen that the injury of 
 the cortico-fugal motor tracts often does not halt when reaching the spinal 
 and bulbar nuclei, but changes these also functionally and morphologically, 
 just so the atrophic processes of the myopathic forms of progressive muscular 
 atrophies, which begin in the muscles, lead not infrequently to an impair- 
 ment of the function and the anatomic condition of the gray anterior horns. 
 Therefore, even in this respect, in a large number of transitional forms, 
 which frequently make the interpretation of an individual case more difficult, 
 the theory of the unity of the entire motor system, possibly also a unity of the 
 pathological processes holds good. Notwithstanding this, a considerable 
 number of observations during a protracted course of the disease and with 
 characteristic grouping of symptoms, the peculiar localization of the muscu- 
 lar atrophy, made sure by anatomic findings, justifies the demarcation of 
 progressive spinal muscular atrophy as a different group of diseases from the 
 related affections, amyotrophic lateral sclerosis and myopathic muscular 
 atrophy. 
 
 There is but little known about the causes of the disease. Its appearance 
 in several members of one family, that is, upon a common congenital predis- 
 position, has been observed. The disease occurs, however, more frequently 
 isolated, in adults, and more frequently also in men than in women. The 
 exciting influence of local or general traumatism is certainly of importance, 
 but very difficult to judge because of the unusually slow and insidious develop- 
 ment of the disease. This is likewise true of the influence of colds and in- 
 
SPINAL PROGRESSIVE MUSCULAR ATROPHY 
 
 299 
 
 fectious diseases. It seems that the disease may develop upon the foundation 
 of a syphilitic infection, just as in tabes the injury of the nervous system not 
 infrequently extends itself to the spinal motor nuclei. Severe physical work 
 and over-exertion certainly further the development of the disease, but one 
 must beware of a confusion with the muscular atrophies in occupational 
 neuroses {Oppenheim) which are more favorable, prognostically and much 
 less severe. Finally, the development of a progressive spinal muscular atro- 
 phy in more mature years, has been likewise observed being grafted 
 on a defect which developed in childhood as the result of an anterior 
 poliomyelitis. 
 
 Symptomatology and Course. — It is especially characteristic of pro- 
 gressive spinal muscular atrophy, that it does not at one fell swoop or with 
 giant strides seize upon large sections of the spinal motor centres, but begin- 
 ning very gradually in a restricted area, 
 proceeds to the disjunction of one cen- 
 tral element after another, and there- 
 fore gives time for the development of 
 the trinity resulting from wasting of the 
 ganglion cells, of the motor nerve fibres 
 and of the muscular fibres, before new 
 and further areas are attacked. There- 
 fore we see, that in most instances the 
 phenomena of paralysis and muscular 
 atrophy appear simultaneously, and that 
 years often pass before a larger part FlG . 67 ._ Effe minate hand in spinal pro- 
 of the muscles of the body participates gressive muscular atrophy. Atrophy of the 
 
 ball of the thumb, of the little finger, and of 
 in these disturbances. the lumbricales. (Erlangen Medical Clinic.) 
 
 Nearly always the symptoms of 
 functional loss appear in the region of the small muscles of the hands. 
 The patients themselves probably notice that they no longer possess their 
 former skill in finer movements, and that they can no longer develop 
 sufficient strength in grasping, reaching for, and holding objects. Upon 
 examination at this time, one finds even then an emaciation and flat- 
 tening of the ball of the thumb and of the little finger, often more markedly 
 developed on the right than on the left hand. Especially the muscles 
 of the thumb, first, the opponens and abductor brevis, then also the flexor 
 brevis and the adductor, lose their functional ability and become thin, 
 limp, and weak Early, the long muscles of the thumb in the forearm, the 
 extensor and abductor pollicis longus are added, so that then the thumb 
 begins to lie almost immovable on a plane with the metacarpus {ape's hand) 
 (Fig. 67). But the other small muscles of the hand are not spared: the atro- 
 phy of the interossei and the lumbricales causes a. prominence of the lines 
 of the bones on the back of the hand and in the palm, and together with the 
 
300 DISEASES OF THE SPINAL CORD 
 
 paralysis of the extensors of the terminal phalanges and the contracture of 
 the still more effective long flexors and extensors in the forearm, leads to 
 the form known as claw hand (main en griff e). In the period following, 
 the extensors of the forearm are affected, also the flexors, the atrophy ap- 
 pearing preferably in the ulnar division of the flexors, so that here, too, the 
 contours of the bones become more and more prominent. At the same 
 time, there often exists and persists a peculiar diminution of the extremity' 
 towards the distal end, which is, moreover, favored by the wasting of the 
 cushion of fat, which is mostly connected with the atrophy of the muscles 
 (bird arms). Associated with the wasting of the muscles which continues 
 for years, and the greater and greater immobility of the hands, the bones, 
 too, become thinner, and appear in a skiagraph poor in calcium bone- 
 substance, somewhat wasted, with extremely delicate structural design, 
 without usually changing their general essential configuration. 
 
 The further spread of the atrophy and the paralysis does not ascend 
 the arm gradually, but usually by leaps, as a rule, jumping next to the deltoid. 
 With the emaciation, at first partial, then total, the patients lose more and 
 more the ability to raise their arms (Fig. 68) . The arm hangs down from the 
 shoulder, adducted, the use of the hands is made possible with difficulty only 
 by the assistance of the muscles of the upper arm — which may for a long 
 time continue to act — and by swinging motions or by the combined employ- 
 ment of the forearms pressed against each other. Furthermore, the atrophy 
 spreads into the region of the muscles of the shoulder, early in the supra- 
 and infra-spinati, so that the contours of the bony shoulder girdle, especially 
 of -the scapula, become more and more prominent; then, too, in the cucullaris, 
 whereby the raising of the arm and the turning of the shoulder are made 
 still more difficult; in the rhomboidei, the latissimus dor si, the pectorales, 
 and the muscles of the back of the neck, so that the head sinks forward and 
 can not be voluntarily raised. If the atrophy of the muscles is very far 
 advanced, as a result of the functional loss of the muscular tonus, the 
 ligaments of the joints relax, and the joints become loose. Finally, a 
 participation of the muscles of the trunk may make the upright position 
 difficult, and lead to a compensating lordosis. 
 
 The lower extremities are but rarely affected by the paralysis and the 
 muscular wasting; but when the affection is of long standing, there appears, 
 here too, and particularly in those muscles of the leg that raise the foot, and 
 in the pelvic muscles, a, flaccid atrophic paralysis. Therefore the gait 
 becomes a waddle, due to the loss of fixation of the pelvis to the thigh. In 
 order to lift the foot, which is hanging limply down, from the ground, the 
 thigh must be raised abnormally high. The patients become fatigued 
 easily and become more and more helpless. Even in spite of the ad- 
 vanced degree of atrophy, the patients can help themselves to a certain 
 point and retain some mobility through the skillful use of the muscles 
 
SPINAL PROGRESSIVE MUSCULAR ATROPHY 
 
 3oi 
 
 that are retained and still act; finally, however, such a large part of the 
 musculature of the body is affected that the patients become bedridden, 
 and depend upon the help of others for all that has to be done. Participation 
 in the disease process of the muscles of respiration may finally lead to serious 
 difficulty in breathing, and thereby to a fatal end. The condition becomes 
 
 Fig. 68. — Spinal progressive muscular atrophy. Besides the little muscles of the hand 
 the muscles of the forearms and the upper arms have also atrophied. (After Schoenborn and 
 Krieger.) 
 
 equally dangerous when the muscular atrophy reaches the realm of the 
 motor cerebral nerves and leads to the development of progressive bulbar 
 paralysis (cf. p. 318). 
 
 All these stages of the disease develop in the course of years. The 
 reacquisition of the capacity for movement, when it has once been lost, is 
 impossible; an improvement is possible only in so far as the patients may learn 
 
302 DISEASES OF THE SPINAL CORD 
 
 by practice and habit, in adaptation to the changed circumstances, to put 
 the muscular parts that have been retained to better use. Quite often also, 
 the condition remains apparently stationary for a long time. But if, at 
 intervals of some time, an exact and detailed examination is made, one will 
 usually be able to note an advance of the disease. 
 
 The onset of the disease in the arms, i. e., in the small muscles of the hand, 
 and next to them in the deltoid, is of by far the most frequent occurrence. 
 But cases are observed also, in which the first signs appear in the shoulder 
 girdle or even in the legs, and they are then much more difficult to find, than 
 the characteristic change in the configuration of the hands, which immediately 
 attracts the notice of the skilled practitioner. Possibly in this selective 
 action, so far as localization of the disturbance is concerned, the kind of 
 occupation may play a certain role, so that the regions most employed fall 
 first and most completely under the sway of the atrophic process. 
 
 The paralysis remains, as a rule, confined to the atrophied muscles, and, 
 in its degree, is dependent upon the degree of muscular wasting which 
 frequently spares isolated bundles of fibres in the individual muscles, as 
 can occasionally be seen clearly in the contraction of the deltoid. 
 
 In addition to the muscular wasting, which is usually confined within 
 strict boundaries and in typical localization, and the parallel functional 
 disturbance of paresis, or paralysis in certain parts of the limbs, some further 
 changes are worthy of notice. 
 
 The tendon and periosteal reflexes are absent in the atrophied regions. 
 In the arms they are nearly always lost or diminished to a high degree, and 
 this is the case even when the musculature in a part of the fore- and upper 
 arm is relatively well retained and is functionally intact. In the lower 
 extremities they are usually retained for a correspondingly longer time. 
 The cutaneous reflexes are changed only in so far as muscles that have 
 become wholly atrophied, naturally can no longer be stimulated to 
 contract. 
 
 In the muscles attacked by the atrophic process, unless the atrophy 
 is complete, fibrillary and fasciculary twitchings very frequently appear. 
 These are occasionally so active in the small muscles of the hand at 
 the beginning of the disease, that they may lead in the quietly resting hand, 
 to small jerky movements of the fingers; for instance, of the thumb. They 
 are especially striking in the deltoid, in which the muscular restlessness may 
 be increased to a long and persistent wavering and oscillation of the individual 
 fibres and bundles of fibres, which may be still further increased by stimula- 
 tions of the skin, tapping the muscle, under the influence of cold and electric 
 stimuli, as well as by active attempts at movement. 
 
 The electrical excitability of the wasted muscles, which only in rare in- 
 stances exhibit a tendency to fatty infiltration, is quantitatively always lowered. 
 In complete atrophy, well localized tests in which powerful currents are 
 
SPINAL PROGRESSIVE MUSCULAR ATROPHY 303 
 
 employed and the often very disturbing influence of a communicating loop of 
 the currents in the muscles that are intact is carefully avoided, may demon- 
 strate the presence of total reactions of degeneration, especially in the small 
 muscles of the hand. More frequently, however, the muscles, which are 
 usually considerably reduced in size, present, corresponding to the merely 
 partial atrophy, only a -partial reaction of degeneration with sluggish contrac- 
 tion upon direct galvanic stimulation. 
 
 Sensibility, which should always be thoroughly tested, remains com- 
 pletely normal in all its qualities. The paralyzed arms and hands become 
 cyanotic, as a consequence of hanging down immovably, and feel cold; the 
 skin over them occasionally becomes thin and delicate, but decided vaso- 
 motor and trophic changes are never found. Bladder disturbances, disturb- 
 ances of the genital functions and of ability to empty the bowels, do not belong 
 to the picture of the disease. 
 
 The occurrence of myotonic phenomena is worthy of mention (muscular 
 rigidity after active movements, increased mechanical excitability of the 
 muscles and myotonic electrical reaction) in cases of disease which, at least, 
 according to the localization of the simultaneously occurring muscular atro- 
 phies seem, very similar to progressive spinal myatrophy. Probably, it is a 
 matter, however, of atrophies, which proceed from the preceding myotonic 
 change in the muscles and are not dependent upon a primary spinal localiza- 
 tion of the process. 
 
 Pathological Anatomy. — Anatomical examination in progressive mus- 
 cular atrophy reveals a wasting of the gray anterior horns in the spinal cord, 
 confined mainly to the cervical segment. The horns are reduced in size; 
 many ganglion cells and nerve fibres disappear. The ganglion cells still 
 present are noticeably changed in structure. The anterior roots are atrophied 
 and their medullated fibres considerably diminished in number. In pure 
 cases the medullary sheaths in the spinal cord are intact. On the other hand, 
 there is a loss of motor nerve fibres in the muscle-nerves that belong to the 
 injured segments, and distinct changes of structure may be seen to exist in the 
 muscles themselves. The muscle fibres are pale in muscles showing a high 
 degree of atrophy and show, in microscopic sections, great narrowing and 
 proliferation of the nuclei. A luxurious growth of connective tissue sur- 
 rounds the thin muscle fibres, which up to the most extreme degree of atrophy, 
 under otherwise normal conditions still permit us to recognize transverse 
 stripes, but finally become reduced to very thin, slightly granular, clouded 
 little fibres, which no longer show any obvious structure. Frequently fibres 
 of considerably better preserved size and sharply defined structure, lie among 
 the muscle fibres, which have wasted to a considerable extent or even so 
 completely as to leave the sarcolemmic sheath almost empty. The muscle 
 spindles, corresponding to their physiological import as organs of the deep 
 sensibility, suffer, in the pure spinal muscular atrophy, no change. 
 
3 o4 DISEASES OF THE SPINAL CORD 
 
 A real "degenerative" change oithe muscle fibres (fatty degeneration, wax- 
 like changes, fissures and cracks in the muscle fibres) does not occur in 
 spinal muscular atrophy, which, as contrasted with myopathic muscular 
 atrophy shows only gradual anatomical differences. Nor has the occurrence 
 of genuine hypertrophy of individual fibres within the muscle, which has be- 
 come seriously atrophic in consequence of loss of innervation, been deter- 
 mined with any degree of certainty. The anatomic-histological examination 
 of the muscles, and especially of little pieces of muscle, taken from the living 
 body or from the dead body before or during rigor mortis is extremely com- 
 plicated by the changes occurring in the surviving contractile substance 
 during the preparation, and by the variable changes in the condition of the 
 muscular protoplasm dependent on metabolism and toxic influences before 
 death. The functional changes observed in the atrophied muscles (electrical 
 reaction of degeneration) following disjunction of the spinal motor center are 
 dependent upon the destruction or degeneration of the end branches of the 
 peripheral motor nerves, not upon the morphological condition of the muscle 
 fibre. The term "degenerative muscular atrophy" which is useful, in contra- 
 distinction to muscular atrophy where nerve conduction is retained, is there- 
 fore not to be understood, in a pathologic-anatomical, but purely in afunctional 
 sense, or in relation to the condition of the nerves. It concerns an atrophy, 
 which goes hand in hand with those changes of mechanical and electrical 
 muscular excitability, which can be proved to exist in "muscles, the nerves of 
 • which have been destroyed." 
 
 The diagnosis of spinal progressive muscular atrophy is based upon the 
 protracted course of the disease, extending over years and decades, upon the 
 parallelism between paralysis and muscular wasting, upon the typical localiza- 
 tion and the leap-like advance of the symptoms, the absence of the tendon 
 reflexes and all spastic phenomena and the strict confinement of the disease 
 picture to the motor sphere. Especially does consideration of pains and of 
 sensory disturbances make it possible and, as a rule, quite early in the disease, 
 to distinguish it from spinal gliosis or cervical compression myelitis; it is in 
 respect to muscular changes alone that they may give rise to similar clinical 
 pictures. We may refer here to what was said under amyotrophic lateral 
 sclerosis. From this latter muscular atrophy is separated by the absence of 
 spasticity in the lower extremities and of all reflex exaggeration. The dis- 
 tinction from typical cases of myopathic dystrophy is made possible in most 
 cases by the localization in the small muscles of the hand, by the fibrillary 
 twitchings, and by reactions of degeneration, but, especially in respect to 
 localization, there are many transitional types. The course of the disease 
 and the succession of the symptoms allow us to easily avoid confusion with 
 multiple neuritis, and with the acute poliomyelitis of adults. Chronic polio- 
 myelitis, which is closely related to spinal muscular atrophy, might furnish 
 most of the differential-diagnostic difficulties, were it not for the fact that 
 
SPINAL PROGRESSIVE MUSCULAR ATROPHY 305 
 
 domination of the paralyses over the atrophic defects, and a broader exten- 
 sion of the process over larger masses of muscles, from the very first speak 
 clearly for poliomyelitis. 
 
 As an infantile hereditary and familial form of progressive muscular 
 atrophy, cases of quickly progressing muscular atrophy have been reported 
 Werdnig, Hoffmann) which appear in earliest childhood in brothers and 
 sisters, attack the muscles of the thigh, of the pelvic girdle and the trunk, then, 
 also, of the extremities and lead to deformity of the vertebral column. The 
 atrophied muscles show reactions of degeneration. The children, in the first 
 years of their life, yield to the progressive muscular wasting as a result of 
 their congenital liability to intercurrent diseases. The anatomic findings 
 show degeneration of the cells of the anterior horns and of the peripheral 
 nerves. 
 
 The prognosis of spinal progressive muscular atrophy is always un- 
 favorable to this extent, that recovery or a permanent checking of the disease 
 is impossible; the illness always shows a tendency to spread, even though 
 slowly. In this way the condition of the patients becomes really helpless, 
 even if they do not often suffer for a long time directly from the disease. A 
 dangerous turn through the appearance of bulbar disturbances, of paralysis 
 of respiration, or of intercurrent diseases, favored in their effect by the difficulty 
 of the patients to move, are, at all times to be feared. 
 
 Therapy is virtually powerless in face of the progressive tendency of the 
 disease. One must be satisfied with avoiding a furthering of the progress of 
 the disease, by reduction of bodily movements, and guarding against all 
 over-exertion, to which the patients, precisely because of their weakness, 
 are very easily exposed. Careful treatment by exercises, arranged to suit 
 the peculiarities of each case, massage and the use of the constant current 
 may serve to strengthen fibres still capable of functioning, in the muscles 
 that have already been attacked and to awaken new efficiency in them. 
 It is important to combat in this way a premature stiffening of the limbs by a 
 secondary contracture of the antagonists (which have become too powerful) 
 by strychnine (daily, subcutaneously up to gr. 1/60) or with injections in 
 the paralyzed muscles. An attempt at medicinal treatment by strychnine 
 preparations administered in other forms, possibly combined with iron or 
 arsenic, may be recommended. If some suspicious factors speak for a 
 previous case of syphilis (serum test, scars on the skin or on the mucous 
 membranes, lymphocytosis of the cerebro-spinal fluid), a long and energet- 
 ically conducted treatment with iodide of potassium, with or without careful 
 mercurial treatment must be attempted, since, though it cannot restore the 
 musculature that has been lost or cause it to regenerate, it checks, under 
 certain conditions, further development of the disease, or of other nervous 
 maniiestations thereof. 
 
306 DISEASES OF THE SPIXAL CORD 
 
 (f) Neuritic Progressive Muscular Atrophy 
 
 Peroneal, Forearm Type of Progressive Muscular Atrophy 
 {Char cot-Marie, Hoffmann) 
 
 BY 
 
 FR. JAMIN (Erlangen) 
 
 Among the many and various forms, in which systemic change of the nerv- 
 ous system may express itself preeminently by myoatrophic conditions, a 
 group of diseases, not very frequently observed, may be set aside from the 
 closely related pictures of spinal and pure myopathic atrophies, which usually, 
 if not always, appear familially and in childhood are characterized by the 
 localization of degenerative muscular atrophies in the distal parts of the 
 body, in the foot and leg, the hand and the forearm, and by slight disturb- 
 ances of sensation prove the participation of the sensory conducting paths; 
 in most cases these changes predominate in the peripheral nerves. 
 
 This neuritic progressive muscular atrophy begins very gradually in 
 later childhood, in some cases, however, not until adult life. It attacks, as 
 a rule, several members of a family, sometimes through several genera- 
 tions, sparing neither boys nor girls, but showing a distinct preference 
 for the former. Isolated cases occur as well. If the disease has attacked a 
 family, it may skip individuals or entire generations, only to reappear 
 among their descendants. 
 
 The disease, then, is decidedly endogenous, developing upon congenital 
 foundations. Accidental etiological factors — getting drenched, and catching 
 cold, traumatism, post-infectious cachexias and intoxications — may be said to 
 be only exciting causes of the disease, or to assist in its development. 
 
 The arrangement of the symptoms, in particular the localization of the 
 myoatrophy, is very characteristic. The patients themselves become aware 
 of their disease by the remarkable weariness and weakness of the feet in 
 walking, and also by the change in the contour of the feet, which interferes 
 with the use of shoes. 
 
 Paresis and circumscribed muscular atrophy, discovered in the examina- 
 tion, appear first in the small muscles of the foot; they cause the contours of 
 the bones upon the dorsum to stand out, the sole is deepened, and the medial 
 edge of the foot is flattened out. Early, too, appear paralysis and atrophy 
 of the muscles of the leg that are supplied by the nervus peroneus, above all, 
 of the extensor hallucis longus, then, too, of the extensor digitorum and of the 
 peronei, also of the tibialis anticus. The anterior and external sides of the leg, 
 therefore, appear emaciated and through this atrophy raising the foot, 
 especially its outer edge, becomes impossible, likewise dorsal flexion of the 
 toes, above all of the great toes. When the leg is raised, the tip of the foot, 
 therefore, as in peroneus paralysis, drops towards the floor with the claw-like 
 
NEURITIC PROGRESSIVE MUSCULAR ATROPHY 
 
 3°7 
 
 toes in plantar flexion. In the beginning, and occasionally for a considerable 
 period of time, the foot remains easily, even abnormally movable at the 
 ankle-joint; it swings loosely in the ankle-joint in passive movements. But 
 since, as time goes on, the contrast in efficiency of the atrophied extensors of 
 the foot and toes and the considerably better conserved muscles on the flexor 
 side of the leg, of the group of the calf muscles, becomes more and more 
 pronounced, contractures form (cf. Fig. 69). There develops, symmetrically 
 on both sides, a formation of pointed and club foot, that can no longer be 
 made normal, a pes equinus varus. Since in the course of the disease, the 
 triceps surae is usually also (gastroc- 
 nemius and soleus) attacked by the 
 atrophic process, so that the curve of 
 the calf is lost, the plantar flexion of 
 the foot may also be lost; the con- 
 tracture position is then controlled by 
 the long flexors of the toes and the 
 tibialis posticus, which often continues 
 powerful for a very long time; when 
 the action of the peroneal muscles is 
 completely lost, it causes the foot to 
 turn inward with the formation of 
 "hollow foot." Then the toes can no 
 longer be passively extended. The 
 patients now walk upon the outer 
 edge of the foot, even upon the outer 
 dorsal side of the foot, upon which 
 callus forms, and since the patients are 
 mostly young individuals, the tarsal 
 bones may be crowded out and moved 
 from their natural places. 
 
 The muscles of the thigh become atrophic late and to a lesser degree. 
 On the other hand, there appears earlier, in rare cases, even preceding the 
 paralysis in the feet, an atrophy and corresponding paralysis of the small 
 muscles of the hand and the extensors of the forearm with claw-like formation of 
 the hands and tapering of the distal division of the arm, as it is described 
 in spinal muscular atrophy. In later stages of the disease, the muscles of the 
 upper arm and shoulder participate also, to a moderate degree, in the mus- 
 cular wasting, whereas the muscles of the face and the trunk, also the bulbar 
 realms are mostly spared, and therefore the muscular atrophy in these cases 
 rarely reaches such proportions as to seriously threaten health and life. 
 
 The atrophied muscles show occasionally, but not regularly, fibrillary 
 twitchings. Their electrical excitability is, both upon indirect and direct 
 stimulation,^ lowered quantitatively, corresponding to thej_degree of the 
 
 fck^l 
 
 
 
 
 ^L ^r 
 
 
 
 
 wS\ 
 
 
 
 
 ^. 1 
 
 
 
 
 
 
 H 
 
 
 
 
 1 9 
 
 
 I J 
 
 
 ^A SI 
 
 mm £ 
 
 
 
 
 
 
 
 
 Fig. 69. — Posture of the feet in neuritic mus- 
 cular atrophy. (Erlangen Medical Clinic.) 
 
308 DISEASES OF THE SPINAL CORD 
 
 atrophy; under certain conditions it is entirely lost. In the regions that 
 are more severely affected, the peroneal muscles, the small muscles of the 
 hand, etc., the presence of partial or total reactions of degeneration may be 
 demonstrated. 
 
 The tendon and periosteal reflexes are, always, so far as the atrophy extends, 
 frequently, too, throughout the extremities, wholly lost or considerably weak- 
 ened. The cutaneous reflexes are not changed, in so far as they are not 
 necessarily restricted by the loss of muscular regions that have become atro- 
 phic, or by the contractures. 
 
 Sensibility is really not much disturbed, but in many cases it does not 
 remain perfectly normal; painful sensations and paresthesias are observed 
 in the atrophic limbs, which can not be explained by the changes of position 
 and movement alone, also dulling of the superficial sensibility to the point of 
 complete anaesthesia in the feet. The feet are cyanotic and feel cool. More 
 serious trophic disturbances do not develop. Bladder and rectal disturbances 
 are also absent. 
 
 The pathologic-anatomic investigations have shown that this disease is 
 dependent upon degenerative changes in the peripheral nerves, which supply 
 the atrophied muscles, especially in their distal intramuscular ramifications. 
 Atrophies in the corresponding segments of the gray anterior columns of the 
 spinal cord have also been found. Likewise the posterior columns (funiculi 
 graciles Goll) have been found to be the seat of chronic changes. The 
 changes in the atrophied muscles correspond to the picture outlined in spinal 
 muscular atrophy — serious muscular wasting with connective tissue changes of 
 the muscle parenchyma, while fatty infiltration is rarely present, and genuine 
 muscular hypertrophy occurs but seldom in the well-preserved muscles, which 
 in part are strengthened through their compensatory activity. 
 
 The diagnosis is based on the beginning of the disease in childhood, the 
 very protracted course, the peculiar localization of the symptomatic muscular 
 wasting, which resembles spinal muscular atrophy of the peroneal-forearm 
 type. An absolute strict differentiation from spinal muscular atrophy can 
 not always be made because of the anatomic changes which in many respects 
 are identical, and there are transitions even to myopathic dystrophy, which, 
 however, as a rule, begins from adjacent groups of muscles. Genuine 
 polyneuritis develops far more quickly into a paralysis, which has occasion- 
 ally similar localization, but is usually far more extended, displays more 
 striking sensory disturbances (pains, ataxia) and, after a stormy course, 
 permits the return of ability to move, while neuritic muscular atrophy, in its 
 symmetrical, gradually progressing extension, produces irreparable defects. 
 The continuous increase in the wasting of muscles, lasting for years, from 
 slight beginning in the periphery, distinguishes it from poliomyelitis also. 
 
 A disease similar to this, with similarly localized muscular wasting, the 
 same disturbances of electrical excitability, and slight sensory disturbances, 
 
NEURITIC PROGRESSIVE MUSCULAR ATROPHY 
 
 3°9 
 
 has been described under the name interstitial hypertrophic progressive 
 neuritis; this disease may also be familial. It is distinguished from the 
 peroneal form of muscular atrophy by the objectively demonstrable thicken- 
 ings of the peripheral nerves (peroneus, ulnaris) as well as by the complica- 
 tion with distinct disturbances in co-ordination. 
 
 Fig. 70. — Neuritic muscular atrophy. Pronounced atrophy of the peripheral parts of the 
 extremities, especially in the leg proper. (After Schoenborn and Krieger.) 
 
 The prognosis with regard to life is not really unfavorable. The 
 ability to move and the possibility of following one's vocation, are, however, 
 always to a great extent impaired, especially when the upper extremities also 
 are severely attacked. 
 
 Therapy cannot stop the wasting of the muscles. By massage and 
 
3 io DISEASES OF THE SPINAL CORD 
 
 electrical treatment, as well as by a diligent care of the entire body, the rate of 
 the progression may be somewhat influenced, and the condition of the patient 
 is made distinctly more endurable by a careful strengthening of the retained 
 muscles by exercise and use. The changes in form and posture of the feet, 
 which are most disturbing, cannot be removed permanently or checked by 
 supporting apparatus. The function of the preserved muscles, especially of 
 the tibialis posticus, always forces the foot, if it is to be used at all, into an 
 abnormal position again. As in all progressive muscular atrophies, plastic 
 operations also afford little prospect of permanent results. The gait and 
 posture of the patient may be best improved, by first loosening the tendons 
 shortened by contracture, and then anchylosing the ankle-joint into a correct 
 position permanently by an operation (arthrodesis) , in order that the action of 
 the better preserved muscles of the pelvis and the thigh may be given a safer 
 support in walking. 
 
 (g) Subacute and Chronic Poliomyelitis 
 
 BY 
 
 FR. JAMIN (Erlangen) 
 
 In rare cases, in adults, a disease is observed, strictly confined to the 
 peripheral motor system, associated with muscular wasting, which, in the 
 localization of the paralysis and the muscular atrophy, as in the developing 
 sequence of the phenomena, can be distinguished from spinal progressive 
 muscular atrophy, as well as from the acute inflammatory diseases of the 
 anterior columns and the peripheral motor nerves. 
 
 Probably subacute and chronic inflammatory processes are concerned, 
 together with degeneration and atrophy in the gray anterior columns of the 
 spinal cord, which owe their origin to the selective effect of chronic poisonings. 
 Lead poisonings, autointoxications, diabetes mellitus are considered causal 
 factors. It is possible that traumatic shocks favor the peculiar localization 
 of the process of the disease. In the pathologic-anatomical picture, chronic, 
 inflammatory changes in the vessels, as well as extensive degenerative- 
 atrophic changes in the ganglion cells and in the mass of fibres of the anterior 
 horns, have been demonstrated. 
 
 In the clinical picture the symptoms of paralysis are most promi- 
 nent. Immediately, without any signs of a general disease, there usually 
 appears first a weakness in the legs, which is increased in the course of a few 
 days to a widespread paralysis of the extremities, but which, nevertheless, 
 usually remains incomplete, attacking some groups of muscles more severely, 
 and sparing others. The extent of the paralysis is not confined within definite 
 bounds. In irregular succession one limb after another may be attacked, or 
 both legs may be paralyzed alone, or both arms. After weeks, and months 
 in some cases, all four extremities may have become paralytic, nor do the 
 
SUBACUTE AND CHRONIC POLIOMYELITIS 
 
 3" 
 
 muscles of the trunk remain intact; and in the decidedly progressive cases, 
 there finally appears a paralysis of a bulbar character, which together with 
 a paralysis of the muscles of respiration may lead to death. 
 
 Only after the serious motor functional disturbance has been present for 
 some time, does the "degenerative" muscular wasting become apparent in the 
 
 Fig. 71. — Poliomyelitis anterior chronica. Severe atrophic paralysis of both hands; at the left 
 side also atrophy of the deltoideus and the brachio-radialis. (After Schoenborn and Krieger.) 
 
 paralyzed regions. The muscles decrease in volume quickly and to a consid- 
 erable degree, become limp and weak. As a rule they show fibrillary twitchings. 
 The electric examination permits us to recognize a diminution of the electrical 
 excitability and partial or total reactions of degeneration, even in paralyzed, 
 but as yet slightly atrophied, muscles. The tendon reflexes are markedly 
 
3 i2 DISEASES OF THE SPINAL CORD 
 
 diminished or entirely abolished, and the cutaneous reflexes are lowered to a 
 degree corresponding to the paralysis. 
 
 The sensibility in the pure cases, remains intact in all its qualities, should 
 there be no more diffuse injury of the spinal cord. In particular, stronger 
 sensory phenomena of irritation, also trophic disturbances, and disturbances 
 in the urogenital and intestinal innervation are absent. 
 
 The disease does not always progress to paralysis of all four extremities, 
 the trunk and the motor cerebral nerves. After weeks, it may become sta- 
 tionary, with more or less extensive paralytic and atrophic defects remaining 
 non-retrogressive, or, after months, there may possibly be an extensive im- 
 provement of the condition, even recovery. 
 
 In regard to differential diagnosis, the common polyneuritis is of 
 importance, since it has caused confusion with chronic poliomyelitis. But 
 it is distinguished from this by the course, which is mostly more rapid, and 
 above all, by the appearance of sensor} 7 phenomena of irritation and of loss 
 of function, severe plains, sensitiveness of the limbs and the nerve trunks to 
 pressure, and disturbances of sensibility and ataxia. In spinal muscular 
 atrophy, and neuritic muscular atrophy, there develop the more sharply 
 circumscribed functional disturbances, corresponding directly to the degree 
 of the muscular wasting, and progressing simultaneously from fibre to 
 fibre with it. They progress far more slowly than chronic poliomyelitis, 
 which in quick succession, at once paralyzes entire groups of muscles. 
 
 The prognosis may be doubtful in the beginning of the paralysis. If 
 the functional disturbance soon remains confined to a smaller region, and, 
 after the development of the atrophy only partial reaction of degeneration 
 sets in (middle form) the chances for recovery are not unfavorable. But 
 if prolonged investigation shows that the flaccid atrophic paralysis marches 
 on and on and can not be checked, it is to be feared that the vital muscular 
 regions will be affected, and that the disease will be fatal. 
 
 The treatment in the advanced cases must be guided by the principles 
 that hold good for acute poliomyelitis and spinal muscular atrophy. At 
 the beginning of the paralysis, rest in bed and very little exercise of the 
 muscles is suggested; massage too is to be applied only very cautiously; 
 in any case galvanic treatment with the anode, whose action is not so ener- 
 getic, is to be preferred to the use of faradic currents. Only when the 
 phenomena retrogress noticeably, may the regeneration of the nerves and 
 muscles be furthered by persistent methodical treatment with the constant 
 current. Where there is some suspicion of a toxic aetiology, one should 
 endeavor to eradicate it by dietetic measures, sweating and baths, also by 
 medicines (iodide of potassium, preparation of salicylic acid) with exact 
 control over the excretions. The possibility that similar forms of disease 
 may develop after a syphilitic infection ought to be considered in the 
 treatment. 
 
\COMBINED SYSTEM DISEASES OF THE SPINAL CORD 313 
 (h) The Combined System Diseases of the Spinal Cord 
 
 BY 
 
 FR. JAMIN (Erlangen) 
 
 The detailed anatomic investigation of the endogenous, congenital, 
 primary column diseases of the spinal cord, which are characterized by 
 hereditary and familial occurrence (Friedreich's ataxia, spastic spinal 
 paralysis) showed that not infrequently, a combined lesion of several systems 
 in the lateral and posterior columns of the spinal cord proved to be present, 
 even though the clinical picture of the disease was distinguished by the 
 predominance of functional disturbance in one system group; in spastic 
 paralysis, of the pyramidal tracts; in Friedreich' s ataxia, of the posterior 
 columns and the cerebellar lateral columns. It was natural, therefore, to 
 assume that pictures of symptoms, which allow us to conjecture the presence 
 of an approximately similar functional disturbance in the various sensory 
 and motor tracts of the spinal cord, have also been caused by a primary 
 selective injury -in the fibre systems of the posterior and lateral columns, 
 whether this injury be based upon a peculiar inborn weakness of this system, 
 relative to its being used up easily, or to general noxse, or whether it is caused 
 by the specific effect of toxic influences on these systems of tracts. As a 
 matter of fact, there are a series of observations, in which the clinical picture 
 of the disease permits us to recognize almost exclusively a functional dis- 
 turbance of the long tracts of the spinal cord, especially of the pyramidal 
 anterior columns, the pyramidal lateral columns, the posterior columns, 
 and the cerebellar lateral columns; and the anatomical findings also seem to 
 establish the assumption of a pure primary combined columnar disease. 
 But if the undoubtedly primary endogenous columnar diseases, which are 
 discussed in the chapters on Friedreich's ataxia, and spastic spinal paralysis, 
 are left out of consideration and also the many varied combinations of tabetic 
 phenomena as being an exogenous disease, the pathogenetic relations for 
 the isolated cases of clinically demonstrable combined columnar diseases, 
 are found considerably more complicated. 
 
 Pathological Anatomy. — The more recent investigations by Nonne 
 and Frnend, Henneberg and other authors, have shown that in most of the 
 cases, the detectable degenerations in the spinal cord correspond, on the 
 whole, to the course of the long fibre systems of the pyramidal tracts, the 
 posterior columns, and the lateral cerebellar tracts, but that they are not 
 strictly confined to these systems, but spare, in part, definite sections, 
 especially those directly adjacent to the gray substance and partly extend 
 irregularly at various levels of the spinal cord in various ways, and often 
 asymmetrically, beyond the area of the well-known tracts. These variations 
 in the spreading of the degeneration over the cross-section of the individual 
 column systems, cannot be quite satisfactorily explained by the individual 
 
3 i4 DISEASES OF THE SPINAL CORD 
 
 differences in the development of the physiologically differing systems. 
 In addition, transitional types, from apparently purely systemic columnar 
 degeneration to decidedly focal disease of the spinal cord, occur. 
 
 In those places, in which the degeneration in the posterior and lateral 
 columns is most marked, in the dorsal or cervical segments, dense sclerotic 
 glia growths appear, in place of the degenerated posterior columns and in 
 the center of the lost pyramidal lateral column, whereas the similarly 
 destroyed lateral cerebellar tracts are replaced by a looser net-work of glia 
 fibres. In the midst of the scleroses, however, thickenings, hyaline degenera- 
 tions and perivascular infiltrations or scleroses are found on the vessels, which 
 seem to confirm the supposition that it is not a matter of primary degeneration 
 of the columnar fibres, but of the cicatricial end-result of focal diseases of the 
 white substance emanating from the vascular system. By the confluence of 
 small focal lesions in the white matter, there arises, associated with the 
 phenomena of ascending and descending degeneration, from the picture of a 
 disseminated myelitis the picture of a visible, but by no means strictly 
 confined columnar disease. For the peculiar arrangement of the focal 
 disease the white matter's scant supply of blood-vessels from the small 
 branches of the marginal arteries of the spinal cord is the cause, in the 
 area of which, naturally, with slighter disturbances in circulation, sclerosis 
 of the fines tramifications and similar injuries appears more easily than in 
 the far more richly supplied gray matter. Nevertheless here, too, as in 
 other injuries which affect the cross-section of the spinal cord over a con- 
 siderable space, the increased vulnerability of the long paths, especially 
 of the posterior columns, and the pyramidal tracts may influence the arrange- 
 ment of the degenerations, and especially the distributions of the functional 
 disturbances. It is to be considered also in comparing the anatomical 
 findings with the clinical picture of the disease, that the anatomic picture, 
 especially in so far as it is confined to the degeneration of the medullated 
 sheaths alone, and does not affect the make up of the axis cylinders, is not 
 able to fully explain the functional disturbances. This explains also why 
 under certain conditions, when there are extensive anatomic changes in the 
 spinal cord, only slight functional disturbances are observed, and vice versa. 
 The lateral cerebellar tracts only participate systemicly in the pathological 
 process, if the degeneration of the lateral columns in the upper dorsal portion 
 of 4 the cord is considerable, because, in that case, the fibres passing from 
 Clarke's columns to the lateral column are injured on their way through the 
 focal injury in the lateral column. Clarke's columns, themselves, are often 
 found intact; in other cases, they too have become atrophic, probably 
 through retrograde degeneration. 
 
 If, therefore, it is probable, that the combined columnar diseases are 
 caused, not by a primary selective injury of the tracts, but by disseminated 
 focal disease as a consequence of disturbances in circulation, ischasmia, or 
 
COMBINED SYSTEM DISEASES OF THE SPINAL CORD 315 
 
 by an injury in the white matter, the result of a blood-vessel dis- 
 turbance, yet in many cases, the preference for the posterior columns, the 
 pyramidal tracts, and the lateral cerebellar tracts gives these processes of 
 degeneration a peculiar impress, and justifies us in regarding these forms of 
 disease, which differ even in the clinical course from the acute myelitic 
 processes, as a separate group. 
 
 iEtiology. — The causes of the pathological changes described, which 
 are rarely examined anatomically until the stage of the secondary and the 
 resulting cicatricial conditions, are manifold. From a number of infectious 
 and toxic injuries; from septic, chronic nephritic and tubercular processes, 
 from pellagra, alcoholism, diabetes, carcinomatosis, lead poisoning, com- 
 bined columnar diseases may result. Syphilis, too, may evoke a similar 
 disease picture.. With special frequency, extensive lesions seem to develop, 
 in serious anaemic and cachectic conditions such as in pernicious anaemia, 
 and leucaemia. In many of these constitutional diseases, special importance 
 attaches probably to the disturbances of circulation, caused by arterioscle- 
 rotic changes in the smallest vessels, and it is probable, that trauma- 
 tisms, with general concussion to the spinal cord, can exercise an exciting 
 influence. 
 
 Symptomatology. — Depending upon the cause underlying the disease, 
 and the extent of the pathological process in the spinal cord, i. e., of the 
 functional injuries caused thereby, the disease picture of combined system 
 disease may develop in various ways. The course of the disease is not 
 rarely very acute, with rapid development of far-reaching spinal functional 
 disturbances; in other cases, the disease shows rather a subacute course, 
 changing in the form of its symptoms with the spread of the changes to 
 various levels of the white matter, and in the cross-section of the white fibre 
 system. A chronic, quite slowly progressing form of the symptom complex 
 has also been reported. In the beginning of the disease at times there is 
 nothing more noticeable than slight subjective disturbances of feeling, 
 paresthesias: probably, in other cases also, slight pains of the character of 
 irritation symptoms of the roots as well, and there are cases in which more 
 important objectively detectable spinal symptoms of functional loss are 
 never developed. But in most instances, the system degenerations lead to 
 clearly recognizable functional disturbances in the lateral columns, pre- 
 eminently in the pyramidal tracts on the one side, and the posterior columns, 
 probably with the inclusion of the radiation areas of the posterior roots on 
 the other side. To these are added eventually the phenomena of functional 
 loss, which are dependent upon a participation of the lateral cerebellar 
 tracts and the antero-lateral columns. 
 
 Preeminently two groups of symptoms appear concurrently as they appear 
 in tabes and Friedreich's disease for the posterior column affection, in spastic 
 spinal paralysis for the lateral column affection. By the union of both, or 
 
316 DISEASES OF THE SPINAL CORD 
 
 an overlapping of the groups of symptoms, there arise various types of com- 
 bined system disease (Oppenheim, E. Mutter) . Degeneration of the posterior 
 column alone leads to the loss of the deep sensibility, to ataxia, to hypotonia of 
 the limbs and to the loss of the tendon reflexes. The degeneration of the 
 ■lateral pyramidal tracts causes paresis of the extremities, especially in the legs 
 with predilection for the flexors, synergisms (tibialis phenomenon, etc.) , after 
 longer duration a tendency to contractures, changes of the cutaneous reflexes 
 (Babinski, Oppenheim), exaggeration of the tendon reflexes, and particularly 
 to hypertonia of the muscles. If both systemic diseases occur together, the 
 symptoms must, in part, mutually neutralize each other, according as the 
 affection of the posterior columns or that of the lateral columns gradually 
 and according to their special expansion preponderate. If, as is frequently 
 the case, the functional lesion of the pyramidal tracts predominates in the 
 clinical picture, the symptoms of hypertonia, of spastic paraparesis, usually, 
 since it is a matter of disturbance in the dorsal segment, in the legs alone, 
 with exaggeration of the tendon reflexes, synergisms and changes of the 
 cutaneous reflexes step into the foreground, while disease of the posterior 
 columns makes itself felt only by slight disturbances of the deep sensibility in 
 the, distal sections of the extremities, as well as by ataxic disturbances in 
 movement, which may be increased upon participation of the cerebellar 
 lateral tracts by the phenomena of static ataxia. Then we have the fre- 
 quently observed symptom complex of spastic ataxic paraparesis. 
 
 If, on the other hand, the injury to the posterior column predominates, 
 or gradually overshadows the lateral column lesion, the disease picture 
 assumes a more tabetic character. The ataxia makes itself felt in a more 
 considerable degree, the sensibility disturbances become more prominent, the 
 tendon reflexes disappear. At times, even the paresis of the lower extremities 
 becomes flaccid, the muscles become atonic. In most of these cases, however, 
 some degree of hypertonia (while tendon reflexes are lost) persists as a sign of 
 the simultaneously present lateral column lesion; this, beside the change of 
 the cutaneous reflexes (Babinski, etc.), beside a slight paresis of the predi- 
 lection type and characteristic changes in the posture of the legs, which finally 
 pass over into contractures (dorsal flexion of the great toe, supination of the 
 foot in the equinus position) permits us to conclude, that there is participation 
 of the pyramidal tracts in the process of the disease. 
 
 More extensive disturbances in sensation, even in the realm of superficial 
 sensation, suggest a participation of the tracts of the antero-lateral columns. 
 It is worthy of note that the sensory disturbances in combined system diseases 
 of the above-mentioned, more pseudosystemic form, are occasionally sharply 
 limited upward, and so can simulate a pure cross-section lesion (Nonne, 
 Frund) . To the realm of the affections of the posterior columns in the wider 
 sense belong the bladder disturbances, which are very frequently observed in 
 cases belonging to this group: retention of the urine, frequently insurmount- 
 
COMBINED SYSTEM DISEASES OF THE SPINAL CORD 317 
 
 able vesical tenesmus, finally loss of the voluntary regulation of the bladder 
 function. Rectal disturbances, retention of the feces, coprostasis occur also. 
 
 In rare. cases, the degenerative process does not stop, continuing even to 
 the higher bulbar divisions of the central nervous system and leads to the 
 phenomenon of obsessional crying and laughing appertaining to the spastic 
 symptom complex, to dysarthria, nystagmus, vertigo, and a high degree of 
 cerebellar ataxia. 
 
 The realms of the sensory cerebral nerves do not, as a rule, participate in 
 the process of the disease. Several times a neuritis optica has been observed 
 to exist in the combined system disease analogously to certain forms of 
 disseminated myelitis. The pupillary phenomena are not changed or 
 influenced in the former. 
 
 The diagnosis of combined column disease is difficult and only to be 
 made very cautiously. It can be based upon proof of the existence of the 
 combination of posterior column symptoms with lateral column symptoms, 
 when either the tabetic or the spastic symptom complex predominates, when 
 signs are wholly lacking of a focal injury in the gray matter of the spinal cord 
 (dissociated disturbances in sensation, degenerative paralyses and localized 
 muscular atrophies) and upon the absence of symptoms of irritation pertain- 
 ing to the posterior roots (relatively violent pains). The course of the dis- 
 ease, which usually is free from suddenly appearing exacerbations, is also, to 
 a certain extent, a characteristic feature. It is to be remembered that in 
 tabes likewise there often appears a combined system disease, a participation 
 of the lateral columns, and in progressive paralysis as well; the existence of 
 pupillary disturbances, lancinating pains, examination of the cerebro-spinal 
 fluid will permit us to recognize under certain conditions, the tabetic nature 
 of the changes. Furthermore, all those spinal diseases, which cause a func- 
 tional disturbance of the posterior columns and the lateral columns to ap- 
 pear through disseminated disturbances or a slighter cross-section lesion, as 
 cerebro-spinal syphilis, which is characterized by relatively violent pains and, 
 occasionally, by early appearing peripheral paralyses, compression of the 
 spinal cord, spinal gliosis, etc., are to be excluded before one is justified in 
 thinking of a combined system disease. Differentiation from the spastic 
 ataxic paraparesis of multiple sclerosis As made possible by demonstration of 
 the purely atrophic optic affection, which is rarely absent in this disease, of 
 the intention tremor, of the absence of the abdominal reflexes in the early 
 stages of multiple sclerosis (E. Midler'). 
 
 Prognosis. — The forms of system disease that have been discussed here, 
 permit no favorable prognosis in the majority of cases. The degenerative 
 processes, in most instances spread farther and farther and, together with the 
 more or less quickly progressing functional limitations and the complications 
 caused by the bladder disturbances, and eventually also by bed-sores, lead, 
 in the course of a few years, to death. At the same time the lessening of the 
 
318 DISEASES OF THE SPINAL CORD 
 
 general ability to resist, due to the constitutional changes which are the basis 
 of the disease, diseases of the blood, chronic intoxications and infections have 
 a pernicious influence. Nevertheless, the disease may possibly become 
 stationary, and occasionally with an improvement in the general condition, a 
 far reaching restitution, leaving only slight functional defects, may take place. 
 
 The therapy must, above all, take into consideration the general con- 
 dition of the patient and aim at getting rid of toxic substances, at the im- 
 provement of the conditions of nutrition, and in anaemics, so far as 
 possible, at stimulating the formation of blood. Therefore the dietetic and 
 medicinal treatment is guided preeminently by the type of the internal dis- 
 turbance. The possiblity of a syphilitic disease of the blood-vessels must not 
 be overlooked. 
 
 In respect to the spinal disturbances, all possible care and rest is necessary 
 for the purpose of avoiding all over-exertion of a system which is often still 
 capable of performing its functions within moderate limits. Therefore all 
 forced movement exercises and bath procedures are contra-indicated. On 
 the other hand solicitous care of the skin, with careful furthering of diaphore- 
 sis is recommended; the emptying of the bladder is to be supervised and care 
 must be taken to keep the bowels regular. After the functional disturbances 
 have become stationary for a long time, and when there is an obvious improve- 
 ment in the general strength of the patient, slowly increasing exercises may 
 gradually be begun. Galvanization of the back along the spinal column may 
 be recommended. In the grave progressive cases, great care, a position in 
 bed calculated to avoid bed-sores, etc., may help in sparing the patient, who 
 is kept in bed by ataxia and paralysis, painful suffering. 
 
 (i) Bulbar-paralytic Diseases 
 
 BY 
 
 FR. JAMIN (Erlangen) 
 i. Progressive Bulbar Paralysis 
 
 Paralysis Glosso-labio-pharyngea Progressiva 
 
 Progressive bulbar paralysis belongs to the systemic diseases of the motor 
 nervous system and is closely related to the spinal diseases, which are charac- 
 terized by primary destruction of the peripheral motor neuron and the clinical 
 phenomena of paralysis and degenerative muscular wasting. Even in spinal 
 progressive muscular atrophy frequently, and in amyotrophic lateral sclerosis 
 almost regularly, besides the gray anterior columns of the spinal cord, the 
 nuclei of the motor cerebral nerves which are of equal functional importance, 
 are gradually implicated by the disease process in the form of progressive 
 atrophy. The nuclei of the nerves of the eye muscles are an exception to this. 
 In rare cases, for reasons hitherto unknown, the primary disturbance of the 
 
PROGRESSIVE BULBAR-PARALYSIS 319 
 
 motor system is limited from the first to these bulbar motor nuclei of the 
 medulla oblongata and the region of the pons, and then there develops the 
 well characterized symptom complex of a progressive paralysis and muscular 
 atrophy in the areas of the musculature of the tongue, face, palate, pharynx 
 and larynx, the picture of progressive bulbar paralysis. 
 
 With the exception of the rather rare atypical cases in childhood, the 
 disease is found as a rule at a somewhat advanced age, generally near the 
 fiftieth year, scarcely before the thirty-fifth or fortieth. Familial appearance 
 has been very seldom reported — as a rule the cases are isolated. Yet the 
 assumption of congenital weakness of the bulbar nuclear centers as a foun- 
 dation for the disease is not unjustifiable, on the analogy of the other related 
 systemic motor diseases and in the absence of definitely proven exogenous 
 causes. How far one is justified in regarding colds, traumatisms, emotional 
 disturbances as causal factors, a critical analysis of each individual case must 
 decide. It is not improbable that the over-exertion of the injured muscular 
 regions, especially of the muscular apparatus of the buccal cavity in certain 
 occupations (blowing musical instruments, glass blowing, etc.), has a pecu- 
 liarly injurious influence, if a diminished power of resistance is present at the 
 same time. Possibly the diminished resistance to the demands made upon 
 the economy by daily life, which is a sequela of syphilis and other infections 
 or intoxications, may also be limited to these regions. 
 
 Course of the Disease and Symptomatology. — The phenomena of the 
 disease develop very slowly and insidiously, as in progressive muscular 
 atrophy. Step by step in the comparatively narrow field of action, one section 
 after another is deprived of its function, in irresistable succession. Since the 
 muscles here concerned represent, in many respects, the guardians and the 
 assisting preparatory forces for the orifices into the gastro-intestinal canal and 
 the respiratory passage, their functional disturbance soon causes a disturbance 
 of the vital processes, nutrition and respiration. Therefore in every bulbar 
 motor disturbance, the duration of the disease in comparison with that of the 
 other systemic diseases, which affect only the locomotor capacity of the 
 patient, is limited. Thus progressive bulbar paralysis, in spite of its usually 
 regular, slow development, which rarely proceeds by greater strides, following 
 one another more quickly, ends in death after a few (two to five) years by 
 disturbances in nutrition, or by diseases of the respiratory organs. 
 
 The first signs of the disease become noticeable to the patient himself 
 and to those surrounding him through difficulty in speech. The extraordi- 
 narily fine degrees of movements of the tongue, that are essential in manifold 
 combinations for the production of sound, are already influenced at a time 
 when the disjunction of separate contractile elements in the midst of the 
 mighty muscular mass of the tongue, could not have altered its simple 
 general movements and its general noticeable volume. In progressive 
 bulbar paralysis, likewise, muscular wasting and paralysis go hand in hand; 
 
3 2o DISEASES OF THE SPINAL CORD} 
 
 but the functional abilities, which have been acquired with exceptional 
 difficulty, and which are essential, especially for the complicated mechanism of 
 speech, so influence bulbar paralyses, that the functional disturbance must be 
 noticeable quite early, even in the slightest injury to the muscular pliability and 
 action, and before the myoatrophic loss is visible. The composition of the 
 sentences and the placing of words and sounds are altogether undisturbed, 
 only pronunciation becomes difficult, confused, unclear, and thick; it is a 
 matter of a pure articular or dysarthric disturbance of speech. At first, the 
 utterance of the lingual sounds suffers (D, T, L, R, N, S, Sh, I) in which the 
 participation of the soft palate soon makes itself felt by a nasal accessory 
 sound and hindrance in the formation of the sounds (C, K, Ch) produced 
 in the back part of the buccal cavity. Afterwards, the labial sounds are 
 also more and more affected (B, P, F, M, W, O, U, E). The weakness of 
 the muscles of the larynx which is added late, causes hoarseness, a loss of tone 
 in the voice, monotony in a deep range, finally aphonia, so that the patient 
 can make himself understood only with difficulty because of an inarticulate 
 thickness of speech and stammering. 
 
 Soon after the appearance of the first speech disturbances, the first diffi- 
 culties in swallowing become apparent. The awkward tongue is unable to 
 direct the food which is in the mouth correctly down into the throat; the 
 paralysis of the soft palate frequently causes a regurgitation of fluid food 
 from the nasal cavity; solid food, because of the insufficiently functioning 
 epiglottis and of imperfect occlusion of the glottis, reaches the larynx and 
 the trachea; the musculature of the throat loses its ability to force the bits 
 of food into the oesophagus. 
 
 If the patients are examined at this time of obvious functional disturb- 
 ances, the bulbar paralyses and atrophies are usually found present. 
 
 The facial muscles are wasted in the lower half of the face. Especially 
 the lips seem thin, wrinkled. They cannot be closed satisfactorily, nor can 
 the mouth be pursed; whistling is impossible. At the same time, the muscles 
 of the forehead and the eyelids, as well as of the eyes, as a rule remain intact 
 and functionate. The tongue can be moved only with difficulty and in- 
 sufficiently. It lies limp in the buccal cavity, its surface is wrinkled, crossed 
 by deep furrows, has thin edges (Fig. 72) and displays active fibrillary 
 tremors, which may also appear in the lips. The uvula hangs low, and is but 
 little raised in phonation, if at all. The immobility of the floor of the mouth 
 and of the base of the tongue is completed by the paralysis and atrophy of the 
 muscles inserted in the hyoid bone from the innervation region of the tri- 
 facial, the facial, and the hypoglossal nerves. A laryngoscopic examination 
 discloses the imperfect occlusion of the glottis by means of a phona- 
 tion test. Active swallowing movements and the raising of the larynx con- 
 nected with them are lost. A paralysis of the muscles of chewing also 
 appears in many cases with atrophy of the temporals and the masseters 
 
PROGRESSIVE BULBAR PARALYSIS 
 
 321 
 
 and an inability to shut the jaws properly and to make extensive lateral 
 movements of the lower jaw. 
 
 In the affected muscles, single fibres and bundles of fibres are retained, 
 and remain functional for some time, so that for a long time the change in 
 electrical excitability can not be demonstrated at all, or only with difficulty. 
 But in the atrophic tqngue, in the thin pliable lips, a partial reaction of 
 degeneration is frequently found, and the 
 sluggish character of the contraction in 
 direct galvanic stimulation is unmis- 
 takable. 
 
 The reflex excitability of the atro- 
 phied muscles is, as a rule, considerably 
 weakened, or even lost. In several 
 cases, however, analogous to the phe- 
 nomena of amyotrophic lateral sclero- 
 sis, obviously with simultaneous lesion 
 of the proper central (pyramidal) tracts, 
 an exaggeration of the reflexes in the 
 facial region and especially of the 
 masseter reflexes could be established. 
 On the other hand, the palatal and 
 throat reflexes are usually altogether 
 lost. In "swallowing the wrong way" 
 which is of frequent occurrence, re- 
 flexly excited coughing appears, but 
 since an expiratory increase of pressure 
 in the air passages is impossible because 
 of the insufficient closing of the glottis, 
 the hoarse coughing-up lacks effective 
 force. Therefore foreign bodies, food 
 
 and bronchial secretions can no longer be ejected, and there results the 
 very dangerous deglutition pneumonias, serious bronchitis, broncho-pneu- 
 monic processes and gangrenous local lesions in the lungs. 
 
 The sensibility even in the area of the trigeminus is never disturbed in 
 any respect, nor the function of the sense organs or of the sense of taste. 
 Slight vaso-motor disturbances, rush of blood to the head with a feeling of 
 heat, and hyperemia occur. Observations of a considerable increase in the 
 pulse rate seem to indicate that the vagus is affected. 
 
 Paralysis and muscular wasting appear first in the tongue, then in the lips 
 and in the muscles of the lower part of the face; finally in the soft palate, the 
 floor of the oral cavity, pharynx, larynx, and the muscles of chewing. Hence 
 the paralysis is restricted to the hypoglossus, facialis in its lower division, 
 motor trigeminus and vagus accessorius. Only rarely do the motor dis- 
 
 FiG. 72. — Atrophy of the tongue in in- 
 fantile progressive bulbar paralysis. Flac- 
 cid features. Because of the co-affection of 
 the upper region of the facial the lids are 
 not perfectly closed. (After Schoenborn and 
 Krieger.) 
 
322 
 
 DISEASES OF THE SPINAL CORD 
 
 turbances spread to the muscles of the neck, the shoulder girdle and 
 the extremities. The upper facialis area is only exceptionally affected 
 (Oppenheim) . 
 
 In the later stages of the disease, a -proinse flow of saliva, which is very 
 annoying to the patient, is nearly always observed. In part, this may be 
 actually due to a secretory disturbance with increased production of saliva. 
 But, on the other hand, salivation, the continuous out-flow of saliva from the 
 corners of the half -open mouth, is certainly evoked indirectly also by the dis. 
 
 Fig. 73. — Progressive bulbar paralysis. "Transverse laughing" and impossibility of closing 
 the lips because of atrophy of the facial muscles. The emaciated face shows folds because of the 
 " skin having become too wide." (After Schoenborn and Krieger.) 
 
 turbances in movement, so that the saliva produced can no longer be 
 swallowed. 
 
 The completely developed picture of progressive bulbar paralysis is very 
 characteristic (Fig. 73). The face is rigid, thin, and wrinkled, only the 
 movements of the forehead and eyes give expression to the intellectual life. 
 The mouth is somewhat open, the lower lip hangs down and permits the 
 saliva to escape, the corners of the mouth droop. In attempting facial 
 expression, in crying or laughing, the mouth is drawn slightly outwards, 
 which gives the face a distorted, suffering look. The inanition produced by 
 the disturbance in swallowing still further increases the prominence of the 
 morbid emaciation, since every particle of the cushion of fat is absorbed. 
 
PROGRESSIVE BULBAR PARALYSIS 323 
 
 Pathological Anatomy. — To the selective injury of the function and 
 nutrition of the muscles from a part of the region of the motor cerebral nerves, 
 corresponds in the anatomic picture of the paralysis glosso-labio-pharyngea, 
 an atrophy of the nuclei of the cerebral nerves concerned. In the nucleus of 
 the hypoglossus, wasting and atrophy of the ganglion cells are most clearly 
 defined, but they are found also in the nuclei of the facialis, the motor 
 trigeminus, and the motor parts of the glosso-pharyngeus and vagus-acces- 
 sorius. Instead of the wasted ganglion cells, and the network of nerve fibres, 
 which has been for the most part destroyed in the degenerated nuclei, there 
 appear proliferation of the glia fibres and thickening of the vessels, but with- 
 out any decided inflammatory changes. The motor roots of the nuclei that 
 have wasted, are degenerated, likewise the appertaining peripheral motor 
 nerve fibres down into the muscles, which themselves show advanced atrophy 
 according to the manner of the spinal muscular atrophy. In some cases, 
 besides this, a participation of the pyramidal tracts also, just as in amyo- 
 trophic lateral sclerosis, has been found to be present. The close relations 
 of progressive bulbar paralysis to this and to spinal muscular atrophy have 
 been mentioned at the beginning. They are of one type, as primary selective 
 atrophic processes of the motor nervous system, the clinical appearance of 
 which depends only upon whether the localization of the injury is to be found 
 in the central tracts, the bulbar or the spinal nuclei. 
 
 The diagnosis of progressive bulbar paralysis offers no great difficulties, 
 if the course is considered which usually advances very gradually in typical 
 succession from the musculature of the tongue to that of the face and 
 pharynx, as well as the nature of the disease as a purely degenerative atrophic 
 paralysis within the boundaries defined. Differentiation from the rest of the 
 motor systemic diseases is founded upon the condition of the musculature of 
 the body and the extremities, possibly upon the presence of spasms. The 
 similarly localized paralyses in acute bulbar diseases show a different course, 
 and scarcely ever remain so free from participation of the tracts for move- 
 ment in the extremities, and for sensory conduction-tracts as they are so 
 closely packed together in the medulla oblongata. In these, as in bulbar 
 gliosis, multiple sclerosis, and tumors, the lesion of the cerebral nerves, 
 especially, is more extended, not so peculiarly circumscribed, attacking also 
 the muscles of the eye and the organs of sense. Pseudo-bulbar paralysis (cf. 
 p. 333) is distinguished from the genuine paralysis by the swifter, frequently 
 quite sudden or jerky progress of the disease picture, by many phenomena 
 which suggest a diffuse participation of the brain (psychic disturbances, 
 paresis of the extremities) and by the preservation of a far-reaching reflex 
 ability in the paralyzed, but not limp atrophic bulbar muscles. 
 
 The prognosis of atrophic bulbar paralysis is absolutely unfavorable. 
 The affection is incurable in its phenomena of nervous functional loss, and 
 because of the disturbances in swallowing, and the insufficient protection 
 
3 2 4 DISEASES OF THE SPINAL CORD 
 
 of the air passages, these, in turn, soon cause, as has been mentioned 
 above, complications in the lungs and bronchial tubes; malnutrition is 
 another important factor. Then, too, the paralysis of the larynx may lead 
 directly to dangerous attacks of choking, and, under certain conditions, as 
 result of a disturbance in the central regulation of the heart's activity, asso- 
 ciated with exhaustion, sudden paralysis of the heart may appear. 
 
 Nevertheless, the pitiful condition of the patient, who with an entirely 
 clear intellect, suffers extremely, demands great care and treatment. Beside 
 the general strengthening by means of baths, nourishing foods and various 
 restorative procedures at the beginning of the disease, an attempt should be 
 made to strengthen the resistance of the muscles retained, in the slightly 
 disturbed parts, by galvanization of the nuchal region, also by tonics (strych- 
 nine, nitrate of silver). As long as the cause is not entirely clear, treatment 
 with iodide of potassium should not be omitted. At the beginning of the 
 difficulties in swallowing, nutrition finds serious tasks. Now it is a question 
 of reducing, so far as possible, the trouble of swallowing by a fluid diet, or 
 one of thin porridge, with corresponding assistance; the artificial food 
 preparations are here very useful and are still more necessary when, because 
 of too great danger of choking, the food must be taken by means of a tube 
 either through the nose or throat — the nasal tube being occasionally more 
 easily inserted. The slow contractions of the atrophic pharyngeal muscula- 
 ture, which can be evoked by galvanization in the neck further the swallowing 
 act, and have, at least, the good suggestive influence in that the patient can 
 then feel the swallowing movements which he can no longer actively perform. 
 The excessive flow of saliva can be combated by atropin. Even though the 
 affection in itself causes no pain, still, in the last stages, the restless condition 
 of the patient, who is tortured by difficulties in swallowing, by the violent 
 and ineffectual cough, by difficulty in breathing, and a feeling of oppression 
 in the heart, demands the administration of narcotics. 
 
 2. Acute (Apoplectic) Bulbar Paralysis 
 
 Phenomena of paralysis in the areas of the motor cerebral nerves after 
 the manner of bulbar paralysis may develop in acute form also, if a sudden 
 injury strikes the motor nuclei situated in the regions of the pons, and in 
 the medulla, the roots or the supranuclear tracts of the cerebral nerves. 
 These acute bulbar paralyses appear but rarely in so symmetrical an arrange- 
 ment as the movement disturbances in the slowly advancing atrophic bulbar 
 paralysis, and only in the rare cases, in which a disease corresponding to 
 acute poliomyelitis anterior attacks the bulbar nuclear fields alone, or as 
 bulbar infantile paralysis, together with the inflammation of the gray 
 anterior columns of the spinal cord, do they remain wholly restricted to the 
 functional disturbances of the motor cranial nerves. In the more frequent 
 
ACUTE BULBAR PARALYSIS 325 
 
 lesions of the nuclei of the motor cerebral nerves due to hemorrhages, 
 thrombotic or embolic softening, traumatic degeneration and acute inflam- 
 mation in the medulla oblongata and the pons, the neighboring sensory 
 centers and conduction tracts, the motor tracts for the extremities and the 
 rest of the central apparatus of the medulla oblongata do not escape. 
 According to the location and the extent of the central lesion, there are 
 formed, in manifold combinations, complicated disease pictures, which are 
 especially characterized by the appearance of alternating and crossed 
 paralyses and alternating phenomena of sensory functional loss. 
 
 Etiology and Pathological Anatomy. — Acute bulbar paralysis is 
 most frequently caused by diseases of the blood-vessels. Arterio-sclerotic 
 changes in the basilar and in the vertebral arteries, as well as in their end 
 twigs, lead, through narrowing of the lumen of the arteries to insufficient 
 blood supply of the bulbus, and sometimes even by complete thrombotic 
 occlusion to ischaemic necrosis in the neighborhood. The branches of these 
 arteries, especially the arteria cerebelli inferior posterior, which springs from 
 the vertebralis, can to this extent be regarded as end arteries in that usually, 
 when there is sudden closure and difficulty in the circulation, as a result of 
 general atheromatosis of the cerebral arteries, sufficient collateral circulation 
 fails to develop. The consequence of such occlusion of the blood-vessels, 
 which in rarer cases can also be produced by an embolus from the heart, 
 especially in the left vertebral artery, is the local death of tissue, with conse- 
 quent softening and formation of cysts. Similar changes appear, also, in 
 consequence of syphilitic endarteritis. In rarer cases, arteriosclerotic 
 changes and the formation of miliary aneurisms, especially under the 
 influence of factors that increase the pressure of the blood, cause larger 
 focal, or small multiple hemorrhages with destruction of tissue in the 
 substance of the pons and the medulla. Larger aneurismal dilatations 
 of the basilar can, just as other narrowing processes do, through compression 
 of the medulla, lead to bulbar disturbances; in their most destructive form, 
 they are seen in fractures and dislocations of the upper two cervical vertebrae. 
 Severe traumatisms upon the back of the head, can also give rise to hemor- 
 rhages in the region of the bulbar centers. 
 
 Besides these softenings or hemorrhagic disturbances caused by changes 
 or injuries in the arterial system, inflammatory encephalitic bulbar diseases 
 are observed, which, especially in youth, appear in connection with infectious 
 diseases, or even in the wake of toxic processes. Perivascular infiltrations, 
 small hemorrhages, degenerations of the nerve fibres and the ganglion cells, 
 granular cells, proliferation of the vessels then disclose on microscopic 
 examination a more diffuse injury to the nervous system. Lighter dis- 
 turbances of this kind may be recovered from before a more deeply rooted 
 injury of the nervous substance has occurred. Such cases exhibit but 
 temporarily the symptomatic picture of acute bulbar paralysis; in a similar 
 
326 DISEASES OF THE SPINAL CORD 
 
 way, but without participation of the long conducting tracts, it may be 
 simulated also by a multiple neuritis of the bulbar nerves. We must like- 
 wise mention here, as a very rare occurrence, abscess of the medulla oblon- 
 gata, which is usually metastatic. 
 
 Symptomatology. — The phenomena of acute bulbar paralysis appear in 
 those cases in which they are evoked by hemorrhages or thrombotic soften- 
 ing, stroke-like, after the manner of a stroke of apoplexy. As a rule, slighter 
 subjective complaints like headaches, especially in the back of the head, 
 and attacks of dizziness precede for some time, or, perhaps, for a few days 
 only, as signs of the disease of the blood-vessels, which frequently accom- 
 panies chronic nephritis, then the patient suddenly falls down with a violent 
 attack of vertigo, associated with headache. Consciousness is often retained, 
 but may be lost. Vomiting, tinnitus aurium, clonic twitchings, in rare cases 
 (especially in hemorrhages of the pons), a single or even repeatedly occur- 
 ring epileptoid convulsive seizures accompany the stormy outbreak of the 
 phenomena. As a rule, the symptoms of labio-glosso-pharyngeal paralysis 
 may then be immediately recognized. Occasionally, a few days may elapse 
 before the picture of the disease is fully developed. Although the paralysis 
 of the cerebral nerves and also of the extremities is at first unilateral it may, 
 as the occlusion of the vessels progresses, seize upon the other half of the 
 body, and an extension of the lesion upwards or downwards may be recog- 
 nized by the fact, that ever more and more bulbar functions are being 
 attacked. In cases of inflammatory bulbar affections, the development 
 of the disease may occur also in a subacute manner, and extend over a num- 
 ber of days or weeks. 
 
 The articulatory dysarthria disturbances in speech, which appear with 
 retained sensorium, or when consciousness returns immediately after the 
 apoplectic attack, as a result of the paralysis of the muscles of the tongue and 
 larynx, are specially characteristic. Speech becomes thick, difficult to 
 understand, hoarse and aphonic. To this is added the early appearing deglu- 
 tition paralysis, combined with paralysis of the soft palate, and the loss of the 
 pharyngeal and palatal reflexes. The patients can not swallow either solid 
 or liquid food. The latter regurgitates through the nose; frequently, also, the 
 food goes into the windpipe, causing a troublesome cough which, because 
 of the inefficient closure of the glottis, does not lead to sufficient expectora- 
 tion. The paralysis of the face is still more striking. This frequently 
 attacks all the branches of the facialis, causing a relaxation of the features, 
 insufficient closure of the lids and impossibility of active or reflex movements 
 of the facial muscles, occurring usually on one side but occasionally on both. 
 This facial paralysis is often followed by an abducens paralysis on the 
 same side, or the inability to direct vision to the side of the paralysis 
 (cf . below) . The muscles of chewing are not rarely attacked by the paralysis ; 
 in other cases, especially at the beginning of the disturbance, because of 
 
ACUTE BULBAR PARALYSIS 
 
 327 
 
 central irritation due to a focus in close proximity to one of the motor nuclei 
 of the trigeminus, trismus is observed. 
 
 According to the location and extent of the central lesion, the motor 
 disturbance in the cerebral nerves, and, when the pyramidal tracts participate, 
 in the extremities also, may assume various forms in various combinations 
 (cf. Figs. 74 and 75). 
 
 If the disturbance extends cranially up to the region of the cerebral 
 peduncles and the area of the corpora quadrigemina, the central tracts for the 
 extremities and for the seventh, tenth and twelfth cerebral nerves, in cases 
 of one-sided focal disease, are affected above their crossing, together with 
 the nuclei of the oculomotor, and there appears a hemiplegia alternans 
 superior (Weber) : a paralysis of the oculomotor on the side of the lesion, and 
 a supranuclear paralysis of the bulbar nerves and the extremities on the 
 opposite side (Fig. 75, 77). 
 
 Corpora quadrigemina 
 
 Nucleus offortinu 
 column 
 
 Pons 
 
 Fig. 74. — Schematic view of the location of the nuclei III to XII. Motor nuclei dotted, 
 sensory deep black. XIX means X and IX (common nucleus of Nr. IX and X). (After 
 Villiger) . 
 
 Hemiplegia alternans inferior (Gubler), in which the nucleus of the facialis, 
 or its already crossed supranuclear tract, is drawn into the focal injury, 
 whereas the pyramidal tract is destroyed above the crossing in the pons, is 
 more frequently found, so that a paralysis of the face, usually flaccid, is 
 combined with a spastic paralysis in the extremities on the opposite side. At 
 the same time there is often a paralysis of the abducens on the side of the 
 lesion, or, if the nucleus of the abducens and its surroundings, with the 
 connections with the nuclei of the muscles of the eye are injured, there is an 
 associated ocular paralysis; the ability of both bulbi to turn to the side of 
 the lesion is lost (Fig. 75, III and IV). 
 
 If the focal disease is confined to the deeper parts of the medulla oblongata, 
 the unilateral lesion of the nuclei of the vagus-accessorius and of the hypo- 
 glossus or its motor roots, may strike the pyramidal tracts above the crossing, 
 
328 DISEASES OF THE SPINAL CORD 
 
 and then there occurs a paralysis of the tongue and the muscles of swallowing, 
 as well as of the soft palate on the side of the lesion, which, moreover, leads 
 from the onset to severe disturbances in deglutition — and paralysis of the 
 extremities on the opposite side (hemiplegia alternans infima) (Fig. 75, V). 
 
 Should the central injury finally strike the pyramidal crossing also, it may 
 happen that the central tract for the upper extremity is interrupted above the 
 crossing, while that for the lower extremity may be affected below the cross- 
 ing; then the arm on the side of the lesion and the leg on the opposite side, 
 will be paralyzed, i. e., exhibit a spastic paresis {hemiplegia cruciata) or 
 paralysis of both legs may appear or, more rarely, of both arms. 
 
 Since, owing to the small size of the pons and the medulla, the softening 
 and inflammatory foci frequently pass beyond the middle line, and as the 
 boundaries of the foci are, as a rule, irregular, there occur also bilateral 
 disturbances, paralysis of all the extremities, combined with unilateral 
 bulbar nuclear paralysis in the facialis or of the oral or pharyngeal cavities, 
 or even bilateral glosso-labio-pharyngeal paralysis, connected with unilateral 
 paresis of the extremities. 
 
 The motor tracts for the extremities are, in bulbar focal lesions, always 
 affected above the spinal motor nuclei; the paralysis of the extremities, there- 
 fore, corresponds in its details to cerebral hemiplegia; if the disease con- 
 tinues long enough, there develops a spastic paresis of the extremities. Not 
 infrequently, however, the tendon reflexes are lost, especially in the upper 
 extremities, probably as a result of synchronous lesion of the spino-cerebellar 
 tracts; the Babinski reflex also does not appear regularly in bulbar paralysis. 
 
 The paralysis in the areas of the cranial nerves is in most cases of acute 
 bulbar paralysis, throughout, or in the greater part of the paralyzed region, 
 degenerative, flaccid, corresponding to the lesion of the peripheral motor 
 neuron in the nuclear area, or in the motor roots. This may be demonstrated 
 in the individual case by the absence of reflex excitability in the musculature of 
 the face, tongue, and throat. If the paralysis persists for several days, the 
 presence of a quickly progressing atrophy in the paralyzed facial muscles and 
 in the tongue, as well as fibrillary Switchings and electric reactions of degenera- 
 tion may be seen to develop in many cases. The latter may occasionally be 
 evoked in the musculature of the pharynx also. 
 
 Next to the motor disturbances which are mostly of an alternating 
 character, the condition of sensation in acute bulbar paralysis deserves 
 attention. Here, also it is to be remembered that the conduction of the 
 sensibility of the trunk and the extremities, undergoes, for the most part, a 
 decussation in the higher parts of the medulla oblongata, so that with a focus 
 that appears on one side in the pons, the sensory tracts of the trunk and the 
 extremities may be affected on the opposite side, in addition to the sensory 
 roots and centers of the trigeminus on the same side of the lesion; this would 
 result in a hemianesthesia cruciata, with paralysis of sensation in the trunk 
 
ACUTE BULBAR PARALYSIS 
 
 329 
 
 and the extremities on the opposite side and loss of sensation in the face 
 (especially in the second and third branch of the trigeminus) and in the oral 
 and pharyngeal cavities on the side of the focal injury. In the deeper divi- 
 sions of the medulla oblongata (olivary region) relations are complicated by 
 
 Wind. 
 
 Pyramidal -\-- Anterior pyramidal trad 
 
 crossing 
 
 Spiral mortar 
 
 roots to right 
 
 arm. 
 
 Fig. 75. — Scheme of explanation of hemiplegia alternans (for the seventh and twelfth nerves). 
 Red: pyramidal tract; N.VII s, left facial nerve; N.XII d, right hypoglossal nerve. I, focus in 
 the right inner capsule left half of the body, the left seventh and twelfth nerves affected; II, focus 
 in the right peduncle, the same result; III, focus in the right half of the pons, left part of the body 
 and the left twelfth nerve, the right seventh nerve (supranuclearly) affected; IV, the same caudad, 
 left part of the body, left half of the tongue and the right seventh nerve affected^(nuclearly resp. 
 radicularly) ; V, a focus in the medulla paralyzes the left half of the body and the right half of the 
 tongue (nuclearly or radicularly) . {After Liepman.) 
 
 the fact that in near vicinity of each other, three sensory systems of various 
 importance are situated: 1. In the medial division, near the raphe (inter- 
 olivary layer) the kinesthetic tracts (sensation of position, muscle sense, 
 pressure sense — deep sensibility), which are crossed here, by the aid of the 
 
33© DISEASES OF THE SPINAL CORD 
 
 fibrae arcutse internse. A focus situated higher up will therefore cause a 
 disturbance of the deep sensibility on the opposite side, a lower focus may be 
 followed by a destruction of the deep sensibility on the side of the lesion. 
 2. A lesion in the lateral division (tractus spinotectales and spinothalamicae) , 
 the tracts for the sensations of pain, of cold, of heat, which have crossed 
 already in the spinal cord, brings with it a dissociation disturbance of the 
 sensations of pain and temperature on the side of the body opposite to the 
 focal injury (trunk and extremities). 3. The spinal root of the trigeminus 
 with the substantia gelatinosa, which is the medium for conduction of sensi- 
 bility for the face, especially in the region of the first branch of the trigeminus, 
 in all its qualities. Since the fibres of the trigeminus, which serve for 
 pain and temperature sensation, are crossed already in the lower sections 
 of the medulla oblongata, a focal injury here may, according to its extent, 
 cause a disturbance in sensibility of the trigeminus in all its qualities 
 on the side of the lesion, or, it may produce an analgesia and therm- 
 anaesthesia on the opposite side of the face (especially in the region of the 
 first branch of the tri-facial) (Wallenberg, Oppenheim, Breuer-M arbur g, 
 L. R. Miiller, E. Mutter). 
 
 These topographical relations explain the fact, that in acute bulbar 
 paralysis, as a result of focal disease in the medulla oblongata, the hemi- 
 anaesthesia alternans or cruciata frequently appears in the form of a disso- 
 ciated paralysis of sensation. The sensation of touch alone, which can prob- 
 ably be conducted by various tracts, is rarely affected. Frequently there is a 
 loss of the sensations of pain and temperature in the trunk and the extrem- 
 ities on the side opposite the lesion, which may, however, as we said above, 
 extend also to the upper half of the face thus forming a total hemianalgesia, and 
 which, as a result of the lesion of the long trigeminus root, is connected with a 
 disturbance in sensation of the face on the side of the lesion. Since a hypass- 
 thesia of the sensation of touch, but more frequently, a loss of deep sensibility 
 on the side of the lesion, maybe connected with it, there result disease pictures, 
 which have a certain similarity to the Brown-Sequard type of unilateral spinal 
 lesion, so that, in connection with the motor disturbance of the cerebral 
 nerves, which appears on the side of the lesion, especially in the facialis, one 
 may speak in a similar sense, of a unilateral bulbar lesion. 
 
 A peculiar type in this respect may be recognized in acute bulbar pa- 
 ralysis, which has its origin in thrombotic occlusion of the arteria cerebelli 
 inferior, a branch of the vertebral. Here there is a circumscribed sharply 
 denned softening in the lateral section of the medulla oblongata between the 
 olive, the fibrae arcuatae internae and the restiform bodies, while the pyramids, 
 the lemniscus and the floor of the fourth ventricle are often spared; the tracts 
 already crossed here, for the conduction of the pain and temperature sensa- 
 tions, the (motor) nucleus ambiguus vagi, the root of the hypoglossus, the 
 spinal trigeminus root and the restiform bodies are affected. The symptoms 
 
ACUTE BULBAR PARALYSIS 331 
 
 noted correspond to such a lesion: contralateral hemianalgesia, unilateral 
 paralysis of the musculature concerned in swallowing and of that of the 
 palate, larynx and tongue, trigeminus anaesthesia on the side of the lesion 
 and ataxia of the extremities on the same side. 
 
 Sometimes, subjective sensory disturbances, such as paresthesias and 
 perverse temperature sensations (cold is felt as heat, heat as cold) make 
 themselves felt in the anaesthetic or hypalgetic extremities in acute bulbar 
 paralysis. Homolateral hyperaesthesia has been also observed with the con- 
 tralateral analgesia, just as in the unilateral spinal lesion. 
 
 Ataxic disturbances in the extremities may be founded upon a lesion of the 
 kinaesthetic tracts, with corresponding loss of deep sensibility. They appear 
 more frequently in the form of cerebellar ataxia, associated with phenomena 
 of vertigo and falling to one side, in lesions of the restiform bodies, or the 
 tractus spino-cerebellaris lateralis on the side of the focal injury, and then 
 they usually remain confined to a disturbance in co-ordination in the large 
 joints, or, say, the musculature of the trunk. But the ataxia, combined with 
 hypotonia, may be so severe as to simulate, at first, a hemiplegic paralysis. 
 Nystagmus (rotatorius) , too, is frequently observed as sign of a disturbance 
 in the co-ordination of the muscles of the eye. Disturbances in the special 
 sensory organs rarely occur. In focal injuries that are situated very high, 
 there sometimes appear also disturbances in sight and pupillary phenom- 
 ena, occasionally, likewise, disturbances in hearing, when the nuclei of the 
 auditory nerve are affected. Participation of the vestibular nuclei, or of 
 the appertaining root fibres, then also of the cerebellum and its connection 
 with the pons, bring about further disturbances in the harmonic co-opera- 
 tion of the muscles, conditions of vertigo, disturbances in equilibrium. The 
 disturbance is always more marked on the side of the lesion. 
 
 Important are the changes which point to a participation of the nervous 
 centers, which, located in the medulla oblongata, control the vegetative 
 functions, and which often quickly cause an unfavorable turn in the course 
 of the disease. As such, we frequently find disturbances in respiration, 
 dyspnoea and especially periodic breathing, which frequently continues for a 
 long time (Cheyne-Stokes) with occasional alarming pauses in respiration. 
 Furthermore, acceleration or irregularity of the pulse and an increase in the 
 temperature of the body, which, especially when the outcome is fatal, rises 
 considerably (even as high as 106 F.). Albuminuria and glycosuria occa- 
 sionally accompany the disease picture. More frequently one observes a, 
 flow of saliva. Vaso-motor disturbances and disturbances in the secretion of 
 perspiration occur also. 
 
 That acute bulbar disease is capable of destroying also the central 
 connections with the sympathetic, is shown by observation of the occurrence 
 of Horner's oculo-pupillary symptom complex (ptosis, enophthalmos and 
 miosis, as a consequence of paresis of the sympathetic) on the side of the 
 
332 DISEASES OF THE SPINAL CORD 
 
 central focus (E. Midler). Bladder and rectal disturbances (retention of 
 urine and feces) are, as a rule, present at the beginning of the disease. 
 Priapism, likewise, has been observed in connection with the first bulbar 
 attack. 
 
 Course of the Disease and Prognosis. — In many cases the patients die 
 soon after the first attack; large hemorrhages, many small multiple hemor- 
 rhages and occlusion of the basilar artery lead, by injury of the important 
 vital bulbar centers, inevitably to death. If the immediate consequences of 
 bulbar apoplexy are overcome, life is still threatened by the results of the 
 paralysis of deglutition, which appear as aspiration pneumonia and bron- 
 chitis and cannot bs overcome, because of the insufficient nutrition and the 
 lowered respiratory function. But in some cases, after some time, through 
 absorption of the hemorrhages and restitution of the circulation in the injured 
 parts, the phenomena disappear to a greater or less extent, and in time there 
 may be sufficient restriction of the anatomic and functional defect, so that 
 only slight motor disturbance remains. In slight inflammatory affections of 
 the bulb, even complete recovery may occur in young patients, in spite of the 
 phenomena of extensive functional loss in the cerebral nerves and the ex- 
 tremities at the beginning. If the paralyses persist, the phenomena of degen- 
 erative atrophy develop more and more distinctly in the regions of the nuclei 
 that have been attacked (e.g., unilateral wasting of the tongue) and also the 
 spastic phenomena in the hemiparetic extremities. Nevertheless, at the 
 beginning of the disease the prognosis is dependent on the extent of the focal 
 phenomena and on the severity of the general symptoms, especially the impair- 
 ment of the vegetative functions. 
 
 The diagnosis is based on the sudden beginning, or at least on the unex- 
 pected onset associated with a rapid increase of the symptoms and on the 
 arrangement of these symptoms, especially the combination of glosso- 
 labio -pharyngeal paralysis with alternating hemiplegia and alternating hemian- 
 algesia. From pseudo-bulbar paralysis, acute bulbar paralysis is distinguished 
 by the course and the behavior of the reflex excitability in the cerebral nerves. 
 Transitional forms, however, occur. 
 
 New growths in the medulla oblongata or in its vicinity often induce, 
 long before the focal symptoms can be recognized, symptoms of general 
 brain pressure; the same is true of cysticerci and chronic meningitic proc- 
 esses; thereby they show a quite different course, than acute bulbar pa- 
 ralysis, in which the distal effects are far less apparent than the focal phenom- 
 ena, which can be determined with great exactness. Bulbar gliosis also can 
 usually be excluded without any difficulty, by considering the development 
 of the symptoms; it must be remembered, however, that occasionally 
 through intercurrent hemorrhages in the medulla oblongata, an acute 
 bulbar paralysis may be directly induced in the course of it. In multiple 
 sclerosis, the pons and medulla oblongata seldom remain intact. In this 
 
PSEU DO-BULB AR PARALYSIS 333 
 
 disease, the total disjunction of the bulbar centers with the corresponding 
 striking and well-defined paralyses and disturbances in sensation, etc., 
 never develops as quickly as in bulbar paralysis; nay more, for a long time, 
 only the lighter bulbar functional disturbances appear, together with a 
 number of characteristic spinal and cerebral disturbances, so that practically 
 there can scarcely ever be a question of confusion of the two, if a thorough 
 examination is made. 
 
 Therapy. — Treatment of a bulbar apoplectic attack is to be directed 
 along similar fundamental principles, as that of a cerebral apoplexy. Abso- 
 lute physical and psychical rest is, of course, essential; in convulsive attacks, 
 it may be attained by narcotics. Local or general blood-letting is to be 
 considered only when hemorrhages or local inflammation are suspected. 
 The possibility of a syphilitic aetiology must be taken into consideration 
 early and treatment by iodide of potassium instituted; in case of disturbances 
 in swallowing, injections of iodipin are always advisable. Nutrition must 
 be carefully watched over and when the paralysis of swallowing is severe, 
 is most satisfactorily attained from the beginning by means of a stomach 
 tube, since in this way, too, the frequent faulty swallowing and therewith 
 the fear of aspiration pneumonia may be avoided. In unilateral vagus 
 paralysis the patients are able, after some time, to swallow more easily 
 again, if they incline their head towards the unparalyzed side. Cold food 
 is more easily swallowed than warm (crossed hemithermanaesthesia) . The 
 analgesic disturbances demand at an early date careful protection of the 
 patient in bed (water cushion) and marked care of the skin, in order to 
 avoid bed-sores. Protection of the conjunctival sclerae and the cornea in 
 total facial paralysis and trifacial anaesthesia must be especially considered. 
 The use of the constant current may assist in reviving the weakened area 
 of the motor cerebral nerves; galvanization at the nape of the neck is espe- 
 cially to be recommended with the anode and excitation of the movements 
 of deglutition. If the paralyses prove after longer observation to be constant 
 and irreparable, with unclouded consciousness, there is no sense in with- 
 holding from the severely suffering patient, the beneficent action of effective 
 narcotics. 
 
 3. Pseudo -bulbar Paralysis 
 
 The bulbar-peripheral centers of the motor cerebral nerves subserve 
 as do the spinal motor centers of the gray anterior columns, the conduction 
 of cortico-fugal motor fibre systems coming from the corresponding centers 
 of the psychomotor region of the central convolutions and in the greater part 
 conducted through the pyramidal tracts. They transmit the motor impulses 
 from the cerebral cortex to the bulbar nuclei and thereby, for the most part, 
 bring about the production of voluntary movements in the appertaining 
 
334 DISEASES OF THE SPINAL CORD 
 
 muscular regions and especially in the extraordinarily fine shadings in the 
 movements of the lips, pharynx and larynx that are needed, for instance, 
 in speech. Purely reflex movements, partly in rather complicated movement 
 complexes (sucking reflexes), can also be produced in the bulbar centers 
 alone, as the experience in the malformations caused by arrested develop- 
 ment in the cerebrum teaches us. Then, too, the involuntary movements 
 of expression and emotion, as in laughing and crying can be evoked, inde- 
 pendent of the influence of the cerebral cortex, by the aid of the subcortical 
 centers in the lenticular nucleus and the optic thalamus. All these reflex 
 movements, which in early childhood are, in part at least, of great physio- 
 logical importance, are in the older child and in the adult subordinated to 
 the restraining, or stimulating control and the inhibitory influence of the 
 cortex of the brain; in part, they are completely crowded out by the pre- 
 ponderance of cortical influence under normal conditions. A destruction 
 or far-reaching restriction of this cortico-bulbar influence by interruption 
 of the supranuclear, cortico-bulbar tracts, or injury of the corresponding 
 centers of the cortex, will therefore be followed in the region of the motor 
 cerebral nerves, especially in the musculature of swallowing, of the lips, 
 tongue and larynx, by a paralysis or paresis in the sense of the loss of the 
 ability for voluntary movement without marked atrophy, while reflex excita- 
 bility is retained and under certain conditions also involuntary emotional and 
 expressional movements. This is the type of pseudo-bulbar paralysis in 
 contra-distinction to acute bulbar paralysis and to progressive bulbar 
 paralysis, both of which, though differing in course, location and extent of 
 lesion, are characterized by the fact, that the motor nuclei in the bulb are 
 themselves injured and at the same time all the motor bulbar functions, 
 even the reflex and automatic ones, corresponding to the localization of the 
 disease, are destroyed, with the natural consequence of a degenerative 
 atrophy, if the duration of the disease is sufficiently long. 
 
 It is significant, for the pathological physiology of pseudo-bulbar paral- 
 ysis, that in the most common form of supranuclear motor disturbance, cere- 
 bral hemiplegia, there are, as a rule, no symptoms of bulbar paralysis; it is 
 true there frequently appears a contra-lateral one-sided paresis of the lower 
 (oral) part of the facial nerve and of the tongue, associated with the paralysis 
 of the extremities; but the musculature of the pharynx and larynx is spared. 
 The reason for this is that these muscles receive bilateral cortical innerva- 
 tion, and that the voluntary movements of these regions on both sides can be 
 sufficiently supplied from one hemisphere alone. The cortico-bulbar tracts 
 must therefore be injured or interrupted in both hemispheres, if there is to 
 be a clinically recognizable supranuclear paralysis of the pharyngeal and 
 laryngeal muscles. The conditions are similar as regards the masticatory 
 muscles, those of the forehead, and those that close the eyelids. 
 
 ^Etiology and Pathological Anatomy. — Pseudo-bulbar paralysis occurs 
 
PSEUDO-BULBAR PARALYSIS 335 
 
 with relative frequency in childhood, associated with more or less promi- 
 nent spastic-diplegic phenomena, in those cases in which an intrauterine or a 
 later acquired inflammatory or traumatic injury caused a bilateral extensive 
 lesion of the cortex of the brain, and thereby led to a functional disturbance 
 of the psycho-motor cortical fields of the bulbar nerves as they are situated 
 within a narrow radius. 
 
 In adult life, it is principally arteriosclerotic changes of the blood-ves- 
 sels of the brain, that lead to multiple hemorrhages from miliary aneurisms, 
 to numerous thrombotic softenings, and to extensive degenerative processes 
 in the hemispheres, and thereby to bilateral lesions of the cortico-bulbar 
 motor systems as well as to the other disturbances. More extensive 
 apoplexies appearing consecutively, in both halves of the brain, may 
 have the same effect. Multiple inflammatory foci, scleroses and embolic 
 softenings, occur less frequently in connection with this condition. Syphilis 
 with its changes in the blood-vessels and gummatous focal disturbances 
 is quite frequently a cause of extensive cerebral disease. 
 
 The location of the injury may vary. The focal disease is rarely restricted 
 to those cortical fields, which on both sides direct the psycho-motor stimu- 
 lation of the bulbar nerves in the operculum. More frequently, soften- 
 ing foci, cysts, etc., may be demonstrated in the medullary layer of the hemi- 
 spheres, especially in the posterior parts of the frontal brain or even lower 
 down in the radiation of medullary fibres, the inner capsule — in the region 
 of the great basal ganglia. The cortical lesion of one side may combine 
 with a focus in the subcortical medullary layer of the other side and it is 
 characteristic of the disease that is based mainly upon disturbances in 
 circulation, that the arrangement of the changes in the brain may vary 
 greatly. 
 
 In some cases, symptoms of pseudo-bulbar paralysis have been observed 
 also in merely one-sided disease of the brain. It is a matter, then, usually 
 of very severe lesions, extensive hemorrhages, etc., in which a functional 
 injury of the other hemisphere also seems understandable, considering 
 the disturbances in pressure and circulation. 
 
 If the atheromatosis of the cerebral arteries has progressed so far that 
 it leads to multiple or extensive focal changes in both hemispheres of the 
 cerebrum, it does not seem strange that the more delicate branches of the 
 vessels in the brain stem and in the medulla oblongata also fail to remain 
 intact for any length of time and themselves cause changes and functional 
 disturbances in the nuclei of the cerebral nerves, beside the cerebral lesion 
 of the supranuclear system. We have, then, a combination of pseudo- 
 bulbar paralysis with phenomena of acute genuine bulbar paralysis, a 
 disease picture, that may have some traits of both forms, cerebro-bulbar 
 glosso-pharyngo-labial paralysis (Oppenheim) . 
 
 Symptomatology. — The cerebral glosso-labio-pharyngeal paralysis is 
 
336 DISEASES OF THE SPINAL CORD 
 
 but rarely ushered in by a severe stroke. More frequently there is observed 
 a subacute progressive development of the disease in starts or jerks, or the 
 symptom complex is completely developed only after several successive apo- 
 plectic attacks. Thus it may happen that after a first attack, a hemiplegia 
 appears with slight disturbances in speech and swallowing, the latter 
 two disappearing, while the hemiplegia persists and that only after a second 
 stroke which now paralyzes the other half of the body which thus far had 
 remained intact, associated with the now bilateral injury to the brain, cere- 
 bral-bulbar paralysis becomes distinct. In other cases again, the disease 
 may develop gradually through the slow accumulation of small, multiple 
 foci of cerebral softening, possibly hastened occasionally by slight apoplecti- 
 form attacks, or even without noticeable phenomena. 
 
 It is characteristic of the anatomic processes, which, in connection with 
 diseases of the blood-vessels, underlie the functional disturbances, that the 
 phenomena of functional loss in pseudo-bulbar paralysis are not strictly 
 confined to a definite topographical arrangement; therefore, they do not 
 usually appear in strict symmetry and may be complicated by manifold 
 focal or diffuse cerebral disturbances. 
 
 Especially characteristic are the disturbances in the facial muscles and 
 in those concerned with chewing and swallowing, likewise in the musculature 
 of the tongue, palate, and larynx with the prominent phenomena of 
 dysarthria and dysphagia, which, as a rule, are combined with general motor 
 disturbances. 
 
 The exterior appearance of the patient is striking : the face is rigid, immo- 
 bile, the mouth half open, the expression lifeless and stupid. The continu- 
 ous flow of saliva has a still more disfiguring effect. The posture of the 
 body is stiff and awkward, the head bowed forward, the movements 
 slow, clumsy and spastic, the gait a laborious drag with small tripping 
 steps. 
 
 The voluntary movements of the facial muscles, of the muscles of the eye 
 and tongue and of the chewing muscles, are very markedly limited, or entirely 
 lost. Speech becomes difficult to understand, nasal, or thick, due to the dis- 
 turbance of articulation and of the formation of labial sounds and to the 
 paralysis of the soft palate. It is affected likewise in intonation, by paresis 
 of the laryngeal muscles and disturbances in the mechanism of breathing. 
 The difficulties in deglutition but seldom attain threatening proportions, but 
 there is frequently a regurgitation of liquid food through the nose and solid 
 food frequently gets into the windpipe. 
 
 The muscles, that have thus been withdrawn from the dominion of the 
 will do not suffer serious atrophy, as a rule, even after prolonged continuation 
 of the disease, the lips and tongue do not grow thin, show no fibrillary 
 twitchings and neither upon indirect or direct stimulation do they give 
 electric reactions of degeneration. 
 
SYMPTOMATOLOGY OF PSEUDO-BULBAR PALSY 337 
 
 The muscular areas that voluntarily can not be set in motion at all, or, 
 only to a very slight degree, are set into very lively, morbidly increased 
 activity under the influence of emotional disturbances, or rejiexly by cuta- 
 neous and sensory stimulations. 
 
 Thus the eyeballs, remaining ordinarily in rigid immobility, turn involun- 
 tarily in the direction of an acustic or strong optic stimulation, and the 
 eyeballs, which can scarcely be moved at all by the patient, are able to fol- 
 low a moving object by reflex adjustment (Wernicke, Oppenheim, Strum- 
 pell). Very slight causes evoke spasms of crying and laughing, in swift, 
 often alternating succession (Oppenheim and Siemerling) . At the same time 
 the muscles, which are almost entirely paralyzed for voluntary movements, 
 contract strongly; the face, formerly immobile, is now spasmodically dis- 
 torted; spasmodic movements of expiration and coughing, occasionally a 
 temporary asphyctic stoppage of breath with pronounced cyanosis of the 
 face, accompany these peculiar emotional explosions, in which the more 
 intelligent patients are quite conscious of the insufficient psychological 
 motivation of these more reflex emotional and expressional disturbances, 
 which really are a result of insufficient cerebral inhibition. 
 
 A permanent increase of tension in the paretic muscles of the face, the 
 tongue, and the jaws (trismus), is occasionally present, and likewise irrita- 
 tion phenomena, like spasms in the muscles of the jaws, gnashing of the 
 teeth, a spasmodic clearing of the throat and choking may be observed. 
 The insufficient cerebral restraint and inhibition is further made manifest 
 in an increase in the reflex excitability of the bulbar nerves and in the appear- 
 ance of combined reflex movements, which under normal conditions, early 
 in extrauterine life give way to the voluntary use of the labio-glosso-pharyn- 
 geal musculature. The masseter reflexes are frequently exaggerated, and 
 upon tapping the lower half of the face, reflex contractions appear in the 
 orbicularis oris, recalling sucking movements (Toulouse and Vurpas). The 
 palatal and pharyngeal reflexes are retained. Irritation of the hard palate 
 causes muscular contractions of the face in the cheeks or upper lip (Henne- 
 berg's hard palate reflex). Irritation of the lips or of the oral cavity evokes 
 rhythmic movements of the lips, tongue, lower jaw, and in the throat (eat- 
 ing reflex of Oppenhein) in peculiar, seemingly almost co-ordinated purpose- 
 ful combination. Movements of swallowing, even when there is disturbance 
 in consciousness, may, as a rule, be evoked by the introduction of liquid food. 
 These reflexes appear most actively in infantile pseudo-bulbar paralysis, 
 but they are frequently found in adults also. One must notice, here, that 
 just as in cerebral hemiplegia, the reflex increase develops to its greatest 
 height only when the lesion has persisted for some time, and the subcortical 
 or bulbar centers, after the interruption of the cerebral influence have, to 
 some extent, recovered, or again become accustomed to independent 
 activity. 
 
338 DISEASES OF THE SPINAL CORD 
 
 That the control of respiratory innervation may suffer also is proven, 
 not only by the cases of the disturbances in breathing in obsessional laughing 
 and crying, but by the frequently occurring conditions of dyspnoea and 
 periodic breathing (Cheyne-Stokes) and the difficulty of regulating breathing 
 during speaking and swallowing (Hartmann). Acceleration and irregu- 
 larities of the heart's action also occur. In greater impairment of the auto- 
 matic functions of the bulbar centers (increase in temperature, glycosuria, 
 etc.), however, one must consider that the arterio-sclerotic disease may 
 likewise occasion foci in the pons and the medulla oblongata, in addition 
 to the cerebral injuries, whereby a disease picture develops, which no longer 
 exactly fits the frame of supranuclear bulbar paresis. 
 
 As a rule, in pseudo-bulbar paralysis, there are also spastic motor dis- . 
 turbances in both arms and legs, corresponding to the bilateral cerebral 
 injury, or, according to the localization of the cerebral foci, only in the 
 form of a cerebral hemiplegia. In extensive arterio-sclerosis of the nervous 
 system, the spastic character of the paresis of the extremities may be more or 
 less obliterated or concealed by injury of the compensation tracts and of 
 the long and short reflex arcs. Occasionally, too, in a restriction of the 
 central disease to the centers and supranuclear tracts of the motor cere- 
 bral nerves, there appears a pseudo-bulbar paralysis with but very slight 
 movement disturbances in the limbs (senile abasia) (Naunyn) or without 
 any at all. 
 
 Of other complications of cerebral labio-glosso-pharyngeal paralysis, 
 we must lay stress upon the very frequent psychic disturbances, which make 
 themselves manifest especially as intellectual weakness, deterioration in the 
 ability to receive and retain new impressions, and in memory, and may be 
 increased to disturbances in orientation, clouded intellect, conditions of 
 delirious excitement. Of course one must beware of deciding on the pres- 
 ence of a psychic defect, from the external aspect alone — from the com- 
 bination of a staring expression in the face with an open mouth, dysarthric 
 speech disturbances and general difficulty in movement. Furthermore, 
 the disease picture may be variously enlarged by a series of cerebral dis- 
 turbances corresponding to the extension of the injury in the brain: motor 
 and sensory aphasia, hemianopsia and other central visual and auditory 
 disturbances, disturbances in equilibrium, as well as more or less extensive 
 disturbances in sensation, especially of the sense of touch and localization, 
 but also hemianaesthesia and disturbances in co-ordination. Among motor 
 symptoms, athetosis and chorea from central irritation either on one or 
 both sides are sometimes seen. Pupillary disturbances and optic atrophy 
 have been occasionally observed along with the bulbar symptoms of func- 
 tional loss. 
 
 More frequently and especially in the beginning of the disease, but 
 also as a permanent concomitant phenomenon, pseudo-bulbar paralysis 
 
PSEU DO-BULB AR PARALYSIS 339 
 
 causes a loss of the voluntary control over the functions of the bladder and 
 rectum, which is present chiefly in the form of retention of feces and urine, 
 but may lead also to enuresis and coprostasis. 
 
 The diagnosis of pseudo-bulbar paralysis will offer no great difficulties 
 if there is complete development of the supranuclear bulbar paralysis with 
 retained muscular nutrition and electrical excitability, with normal, even 
 increased reflex and emotional motor excitability and with the frequent 
 complications of psychic disturbances and cerebral paralysis of the extrem- 
 ities. A confusion with progressive bulbar paralysis is excluded by the 
 fact that this has a far more insidious course, and is distinguished by degen- 
 erative atrophy, fibrillar}' tremors and loss of electrical excitability, as well 
 as of the reflexes of the motor cerebral nerves and by the strict confinement 
 to these areas with the exception of the nerves of the eye muscles. Acute 
 apoplectic bulbar paralysis may occasionally be mistaken for the pseudo-bul- 
 bar paralysis, but can usually be distinguished from it by the presence of 
 genuine bulbar focal symptoms, especially if alternating hemiplegia and 
 alternating hemianalgesia exist. Amyotrophic lateral sclerosis with bulbar 
 localization can give rise to disturbances in movement, which resemble 
 closely those of pseudo-bulbar paralysis, but it will make itself known by the 
 steadily progressing course without apoplectic advances by fits and starts, 
 by the occurrence of degenerative, localized muscular atrophies and by 
 sparing of the psyche and all functions not purely motor. In myasthenic 
 bulbar paralysis, the phenomena of fatigue, weakness and exhaustion, 
 beside the electro-diagnostic signs, predominate in the disease picture, and 
 it wholly lacks the phenomena, so characteristic of pseudo-bulbar paralysis, 
 of involuntary motor processes excitable by reflexes, frequently increased 
 to veritable paroxysms in the paretic bulbar muscles. 
 
 The prognosis of pseudo-bulbar paralysis is unfavorable, even when 
 one considers only the excitant factor, the atheromatosis of the cerebral 
 arteries. The recurrence of apoplectic seizures and therewith, a dangerous 
 turn in the disease, is always to be feared, while thanks to the reflex activity 
 of the bulbar centers, there is usually no danger to life directly from disturb- 
 ance in deglutition and aspiration of foreign bodies. With careful treatment, 
 avoidance of all influences, that might suddenly increase the pressure of the 
 blood (excitement, alcohol, caffein) the patients may, under certain con- 
 ditions, be kept alive for a comparatively long time; with careful attention 
 even an improvement in the disturbances is possible. Treatment by iodide 
 of potassium is always to be recommended; if a syphilitic aetiology is suspected, 
 or proved by anamnestic or objective signs (scars, Wasserman's serum 
 reaction, etc.), a well-directed combined treatment of iodide of potassium 
 and mercury may cause a considerable improvement in the disturbance 
 and in any case effectively combat the further spread of the disease in the 
 cerebrum and possibly in the bulb as well. 
 
34o DISEASES OF THE SPINAL CORD 
 
 3. MULTIPLE INSULAR SCLEROSIS. (Disseminated Sclerosis) 
 
 BY 
 
 HERMANN SCHLESINGER (Vienna) 
 ^Etiological Factors 
 
 The disease is traced back by a number of authors to early (embryolog- 
 ical) disturbances and a disposition upon the part of the neurogliar tissue 
 to proliferate (Striimpell, Mueller) . I am inclined to agree with Oppenheim 
 that congenital anomalies or such developmental disturbances as begin very 
 early in life represent only a factor favoring the special disposition for the 
 disease. 
 
 Marie is of the opinion that acute infectious diseases represent probably 
 the most important setiological factor. The same view is held by many of the 
 later writers, as Kahler and Pick (even before Marie), Oppenheim, Maixner, 
 Redlich, Spiller and others. From my own experience I should regard this 
 as the cardinal, the causal factor. 
 
 The diseases concerned chiefly are typhoid, pneumonia, diphtheria, 
 influenza, scarlatina, variola. Malaria also has been observed rather 
 frequently as an setiological factor. Intoxications (mercury, carbonic 
 oxide, tin, possibly manganese also), may call forth the disease. 
 
 The onset of the disease has frequently been observed after severe 
 psychic disturbances and especially after colds ; furthermore also after severe 
 traumatisms (Schlagenhaufer, Mendel, Redlich). In a great many cases no 
 setiological factors can be discovered. The period of adolescence is particu- 
 larly predisposed to the disease. 
 
 Anatomic Changes 
 
 Disseminated sclerosis is characterized by the appearance of multiple 
 lesions in the brain and spinal cord. The number of foci may range from 
 a few up to several hundred. Their distribution is not regular; sometimes 
 with a relatively large number of foci in the spinal cord, there are only 
 a few in the brain. They may affect every section of the central nervous 
 system, are occasionally arranged with striking symmetry and show quite 
 considerable variations in size. The largest foci are found in the pons and 
 in thejbrain. The optic nerve and the chiasma, also, often have one or 
 more'.foci. 
 
 The foci themselves are, as a rule, very strictly circumscribed and even 
 macroscopically are clearly distinguished from their surroundings by their 
 gray color. They are recognizable, if situated near the surface, even before 
 cutting into the spinal cord, and may be clearly felt by the palpating finger 
 as harder spots. They may lie partly in the white and partly in the gray 
 matter. 
 
MULTIPLE INSULAR SCLEROSIS 341 
 
 In the foci, one finds upon microscopic examination no, or only a few 
 nerve fibres retaining their medullary sheath. But many well-preserved 
 axis cylinders pass through the foci. The neurogliar tissue is extraordinarily 
 increased, and forms a thick felt-like layer, which does not show any ten- 
 dency to softening anywhere. The ganglion cells, even in the center of the 
 foci, are not injured, but the vessels often show grave changes; their walls 
 are thickened and the lumen narrowed, or even entirely obliterated. Sec- 
 ondary degenerations are absent, even after long continuation of the disease, 
 and with numerous foci. Upon the advent of diffuse sclerosis, changes in 
 the form of the sections attacked, especially in the pons, occasionally occur. 
 
 Besides the old foci, one frequently sees diseased spots in which the proc- 
 ess is of more recent date; small-celled infiltration of the vessels (filling of 
 the adventitial lymphatic spaces with fatty granular cells) is exhibited; 
 processes of breaking down in the focal regions are still going on. Foci of 
 more recent date are found especially in cases with an acute and subacute 
 course. 
 
 The pathogenesis is by no means clear. The various views are, in part, 
 in direct contrast with one another. Some authors, like Schmaus and E. 
 Miiller speak of congenital conditions (hypoplastic conditions of the nervous 
 system). Other investigators consider proliferation of the neuroglia the 
 primary change (Rossolimo, Strilmpell, Probst) . Still others believe in a pri- 
 mary lesion of the parenchymatous parts of the nervous system (Redlich, 
 Koppen). The disturbance of the vascular apparatus is considered as a 
 causal factor especially by Taylor, Rindjleisch, Ribbert. The theory of an 
 inflammatory origin finds many supporters (Charcot, Erb, Leyden, Oppen- 
 heim). It is very probable that an acute disseminated encephalomyelitis, 
 and in particular that form accompanied by cellular infiltration (Oppenheim), 
 leaves in its wake, as a resulting condition, a multiple sclerosis. 
 
 Symptoms and Course 
 
 We are indebted to an excellent study by Charcot for our knowledge 
 of several cardinal symptoms of multiple sclerosis, without which for a long 
 time one could scarcely venture to make a strict diagnosis of this affection. 
 Not for some time afterwards were quite a number of other symptoms dis- 
 covered. In the early stages of the disease cerebral or spinal symptoms 
 may occupy the foreground. Very frequently, the disturbances begin with 
 visual disturbances as has been pointed out particularly by Oppenheim, 
 Bruns, Stoelting and Uhthof}. Ophthalmoscopic examination then discloses 
 a neuritis, or an optic atrophy. 
 
 These symptoms may be the first ("like vanguards") (Oppenheim), and 
 are therefore very often misinterpreted. Since our knowledge of multiple 
 sclerosis has been steadily increasing, it happens more and more frequently, 
 that a " retro-bulbar neuritis" afterwards turns out to have been a precursor 
 
342 DISEASES OF THE SPIXAL CORD 
 
 of multiple sclerosis. I have seen quite a few patients, in which this 
 was the first symptom of the disease; occasionally it precedes the other phe- 
 nomena by years. In other cases, a uni- or bilateral optic atrophy develops 
 very slowly or very quickly, with a peculiar gray discoloration of the disc: 
 this optic atrophy as a rule is partial (Uhthoff), more frequently temporal 
 than nasal, rarely ever total, and was found in almost half the cases. Since 
 the visual disturbances in the great majority of cases are transient and are 
 not associated with pupillary disturbances, they are frequently regarded as 
 hysterical precisely because of their instability, when there has been no ex- 
 amination of the fundus. Bruns-Stoelting places the frequency of cases 
 beginning with optic atrophy at 30%. Even very marked visual disturb- 
 ances may disappear. One of my patients, who, five years ago, was unable 
 to recognize fingers, now reads the finest print without difficulty. 
 
 Paralyses of the muscles of the eye, especially of those supplied by 
 the oculomotor and the abducens, are frequent in the early stage. Ophthal- 
 moplegia is very rare — -as a rule, only a few of the muscles are disturbed 
 and the internal muscles of the eye remain normal. Often the paralysis 
 affects the associated movements. Permanent paralyses are rare. After a 
 shorter or longer time, the paralysis of the muscles of the eye disappears. 
 Difference in the size of the pupils is common, reflex rigidity very rare. 
 Charcot' s cardinal symptom, nystagmus, is observed quite frequently. First 
 visible in movements, in which the already paralyzed muscles are used 
 ("nystagmoid twitchings") it becomes more distinct later on and is notice- 
 able even when the eyes are at rest. Nystagmus horizontalis, as well as 
 nystagmus verticalis and rotatorius may be observed; the nystagmus is 
 most frequent when the patient looks to one side. Redlich regards it as 
 completely analogous to intention tremor. 
 
 Paralytic weakness of one extremity, especially of one leg, has often 
 been observed as an initial symptom. I have observed at the same time 
 three cases with initial paresis of one extremity (twice of a leg, in one case 
 of an arm). In addition to the weakness, rigidity of the musculature is 
 usually also present. 
 
 The presence of a spastic paralysis in the lower extremities, is quite 
 frequently observed in multiple sclerosis. Charcot already pointed to 
 this and later observers have, for the most part, confirmed it. Occasion- 
 ally this condition is only suggested and recognized with difficulty — in other 
 cases, it is well developed. 
 
 Increase of the patellar reflexes and ankle clonus belong to the most fre- 
 quent and important early symptoms of disseminated sclerosis. The 
 increase in the reflexes may be demonstrated on both sides. In the early 
 stages also tremor in one extremity is not rare. 
 
 If the disease is fully developed, the cardinal symptoms, postulated by 
 Charcot, are present — nystagmus, intention tremor, scanning speech, to which 
 
MULTIPLE INSULAR SCLEROSIS 343 
 
 are further added reflex exaggeration in the lower extremities with loss of 
 the abdominal reflex. 
 
 The so-called intention tremor is a coarse trembling of the extremities 
 while performing voluntary movements; the trembling becomes more and 
 more pronounced, the nearer the extremity approaches the goal which it is 
 endeavoring to reach. At the same time, muscle groups lying at some dis- 
 tance are contracted. If in a relatively severe case, with the patient in 
 a sitting posture, he attempts a movement with one extremity, his entire 
 body begins to tremble (wave to and fro). If the patient takes hold of an 
 object, it is swung to and fro with the extremity, but usually held fast, in 
 as much as intention tremor is never associated with any considerable 
 weakness of the extremities. The number of the contractions is not very 
 large, much smaller than in Basedow's disease. 
 
 The head also takes part in the oscillating movements. In more severe 
 cases, the tremor in the trunk becomes very decided and during intended 
 walking the trembling of the legs is also very distinct in most of the cases. 
 The tremor does not stop, even if the movements are frequently performed 
 in succession, but the intention tremor disappears altogether, when the 
 patient is in repose. 
 
 Scanning speech is a very noticeable disturbance. The speech becomes 
 monotonous and its capacity for modulation is absent. It becomes drawn 
 out as each syllable is clearly separated from the one following. Even 
 though this speech disturbance sometimes appears early, in the majority 
 of cases, scanning speech is a symptom, that becomes pronounced only 
 at the height of the disease. Brady phasia is occasionally present without 
 synchronous scanning (Redlich). Dysarthria is no rare symptom. 
 
 The exaggeration of the tendon reflexes has already been mentioned. 
 An important symptom is the diminution of the cutaneous reflexes, especially 
 of the abdominal reflexes (Mueller) , which may appear early. 
 
 Besides the increase of the reflexes, especially in the lower extremities, 
 other symptoms as well point to lesions of the pyramidal tracts. Thus the 
 Babinski phenomenon is present, and dorsal flexion of the great toe appears 
 not only when the test is made in the typical way (stroking the inner margin 
 of the foot or the instep or above the metatarsus quintus), but sometimes 
 even in walking, if the patient sets his foot down firmly. OppenheinCs 
 leg phenomenon is clearly developed (dorsal flexion of the great toe upon 
 vigorous stroking of the edge of the tibia) . The testing for Mendel's 
 reflex upon the dorsum of the foot occasionally elicits plantar flexion of the 
 great toe by tapping the dorsum of the foot at the base of the third and 
 fourth metatarsal bones (whereas in healthy people, a dorsal flexion results). 
 
 Disturbances in sensation are more frequent in the course of the disease, 
 than was formerly thought. Disturbances in perception, however, are only 
 temporary, and severe only in exceptional cases. 
 
344 DISEASES OF THE SPINAL CORD 
 
 Phenomena appear due to sensory irritation, especially intercostal pains, 
 pains in the joints, paresthesias, and neuralgic troubles. In one of my cases 
 neuralgia of terrible severity persisted for weeks. Oppenheim reports the 
 case of a patient in whom "tic douloureux" was an initial and permanent 
 symptom of the multiple sclerosis. 
 
 Sensory phenomena of functional loss usually do not persist for any 
 length of time. They are not very serious and cause only a deadening of 
 sensibility, no complete loss of sensation. The sensory disturbance may be 
 uniform and affect the various qualities of (superficial as well as deep) sen- 
 sation in the same way, or there may develop a partial disturbance in sensa- 
 tion, which affects only the pain or temperature sense and disappears after 
 a shorter or longer duration (Freund). They may then return anew and 
 disappear again. Permanent disturbances of sensation are rare. 
 
 Oppenheim observed the Brown-Sequard symptom complex as a transient 
 phenomenon and also hemianesthesia. 
 
 Paresthesias, like formication, feeling of numbness, are not very rare. 
 
 Rectal disturbances are observed at the beginning of the disease only in 
 exceptional cases. In the late stage of the affection incontinentia alvi is 
 frequently seen. I have observed it in quite a large number of cases. The 
 patients, as a rule, perceive the passage of the feces, but can not prevent it. 
 I have often seen this as a transient symptom. 
 
 Bladder disturbances are frequent, v. Frankl-Hochwart and Zuckerkandl 
 found them in four-fifths of their cases, especially when paraplegia of the 
 lower extremities existed. They may be the very first signs of the disease, 
 which is followed by others only after months, or even years. The form 
 of the bladder disturbances is not always the same. At one time simple 
 incontinence with dribbling of the urine appears; at another, spasms of the 
 sphincter with intact sensibility of the bladder, which demands catheteriza- 
 tion, or there develops the "irritable bladder" with frequent tenesmus and 
 incomplete emptying and a sudden shifting between incontinence and 
 retention. 
 
 The bladder disturbances may disappear permanently or return after 
 a shorter or longer time. Persistence of the bladder disturbances is noted, 
 as a rule, only towards the end of the disease. 
 
 Bladder disturbances are more frequent than rectal disturbances accord- 
 ing to my observations, and affect many patients in the initial stage of the 
 disease. 
 
 Disturbances in the realm of the sexual sphere are much less frequent 
 than might be suspected, considering the frequency of the bladder disturb- 
 ances. Only patients with far advanced disseminated sclerosis complain 
 of frequent pollutions or unsuccessful cohabitation. One of my patients 
 married some years after the beginning of his disease, and begot a goodly 
 number of children. 
 
SYMPTOMATOLOGY OF MULTIPLE INSULAR SCLEROSIS 345 
 
 In some cases, bulbar disturbances develop. Sudden paralyses of the 
 soft palate, of the pharyngeal musculature of transitory character are thus 
 explained. Disturbances in deglutition and mastication, regurgitation 
 through the nose, faulty swallowing, may be present as initial symptoms, 
 but do not remain. Disturbances of phonation may be caused by paresis 
 of the laryngeal musculature, and it is especially pareses of the interni, 
 rarely paralyses of the vocal cords, that are observed. Trembling of the 
 vocal cords in phonation has likewise been described. Oppenheim describes 
 tremolo of the voice in uttering a (German E). 
 
 Like other motor paralyses in disseminated sclerosis, the bulbar paralyses 
 do not persist;- 1 myself have seen the most severe bulbar processes retro- 
 gress. Towards the end of the disease, these phenomena may become 
 permanent and present themselves in a form clinically resembling progress- 
 ive bulbar paralysis, but without muscular atrophy in the region of distri- 
 bution of the bulbar nerves. 
 
 Obsessional laughing and crying are sometimes observed very early. If 
 they exist, they sometimes persist permanently. One of my patients was 
 first attacked by obsessional laughing at a funeral, as he stepped up to the 
 open grave, and has now suffered for ten years, uninterruptedly, from this 
 very annoying symptom. The transition from compulsory laughing to 
 compulsory crying may take place very suddenly. 
 
 Apoplectiform attacks, which threaten the patient in every stage of the 
 disease, but luckily, too, spare many patients, play an important part. The 
 attacks are occasionally accompanied by complete loss of consciousness, 
 and are then accompanied in some cases by epileptiform seizures. Or, it 
 may be that only severe attacks of vertigo occur, which may be compared 
 to apoplectiform attacks, strike the patient suddenly, and if he is standing 
 or walking at the time of the seizure throw him to the ground. In direct 
 connection with these attacks, one sees more or less extended unilateral pa- 
 ralyses of the cerebral nerves. Or immediately after the attacks, combinations 
 of cranial-nerve palsies will be observed, which show extraordinary variations. 
 All the cerebral nerves, even the auditory, are affected occasionally. Hemi- 
 ataxia after an attack has been described by Oppenheim, who reports, 
 too, rarer, suddenly occurring paralyses (atrophic paralysis with ataxia 
 of one arm). I have seen the clinical picture of an acute extended 
 bulbar paralysis with paralysis of deglutition, of the soft palate and of the 
 larynx. 
 
 Ataxia is occasionally present. In such instances it affects either only 
 single extremities, or one-half of the body, or finally, only the lower extrem- 
 ities. It is of the nature of cerebellar ataxia (Redlich), and manifests itself 
 by a tottering gait. According to Oppenheim, ataxia must be assumed to 
 be present if the disturbance increases when the eyes are closed. Ataxia 
 does not by any means exclude the existence of spastic conditions in the 
 
346 DISEASES OF THE SPIXAL CORD 
 
 limbs. Its acute development in the upper limbs with or without bulbar 
 symptoms has been observed several times. 
 
 Disturbances of the intellect, especially failing memory, are common; 
 delirium, and dementia are present only in exceptional cases; if either 
 occurred, they were, as a rule, in most of my cases, transitory phenomena. 
 
 Complaints about headaches are common and they occasionally resemble 
 migraine. 
 
 Respiratory disturbances are unusual. Towards the end of life Cheyne- 
 Stokes respiration is observed. Oppenheim has described conditions of 
 asphyxia. 
 
 The pulse rate is accelerated, if a bulbar lesion is present; tachycardia 
 is often continuous, but remains within moderate bounds. 
 
 Muscular atrophies are very rare. Should they develop, however, the 
 electrical excitability is changed only in exceptional cases. Atrophy is seen 
 in individual groups of muscles, or possibly in the musculature of an entire 
 limb. 
 
 The paresis usually affects both legs, so that spastic spinal paralysis 
 results. As a matter of fact, in many spastic spinal paralyses, the presence 
 of a disseminated sclerosis is only established postmortem (as long as these 
 peculiarities were unknown). Unilateral forms with paralytic phenomena 
 and tremor in one-half of the body were observed by Bikeles, Oppenheim, 
 Edwards. Extensive atrophic pareses, concerning which Probst reports, are 
 verv rare. In his case, the symptom complex of amyotrophic lateral 
 sclerosis was present. 
 
 Course. — With respect to the course, three forms may be distinguished: 
 i. Forms with an insidious onset. 2. Cases with acute initial symptoms, but 
 slow course with remissions and exacerbations. Both forms appear pre- 
 ponderantly in young persons. 3. Acutely beginning and quickly ending 
 cases ("acute multiple sclerosis"). 
 
 The cases with an insidious onset seem to be the more frequent. Since 
 the symptoms develop very gradually and show a considerable tendency 
 to remissions, the disease in most cases is supposed to be functional. This 
 error is supported by the fact that the disease is nearly always combined 
 with hysteria. 
 
 In the discussion of the individual symptoms, the possibility of an acute 
 onset was mentioned several times. With this onset the initial picture 
 may be very 7 varied and paralyses of the most varied kinds may develop 
 (unilateral paralysis of the body, ataxia of single limbs, bladder and rectal 
 disturbances, sensory paralyses and those of the muscles of the eye, bulbar 
 paralyses, etc.). 
 
 Extraordinarily important for the recognition of the nature of dissem- 
 inated sclerosis is the peculiarity of the disease, in having long remissions 
 and in advancing intermittently. 
 
MULTIPLE INSULAR SCLEROSIS 347 
 
 The remissions may be surprising even in advanced cases, and in less 
 progressive ones create the impression of a cure. 
 
 Exacerbations follow especially under the influence of weakening diseases, 
 of the puerperium, great emotional excitement and physical over-exertion. 
 Especially injurious seems to be the accumulated emotional excitement of 
 the newly married. Within a short time, I have seen serious symptoms 
 appear in six young brides whose anamnesis proved that the beginning 
 of the disease dated far back into their girlhood. 
 
 With remissions and exacerbations the disease gradually grows worse. 
 The duration is quite long, amounting to 5 to 10 years, and even longer. 
 
 Death results from intercurrent affections, or because of the bulbar 
 changes, occasionally during an apoplectic attack. 
 
 One form of the disease terminates in death very quickly, at most within 
 a few months, or a year: then one speaks of acute multiple sclerosis. Mar- 
 burg, Schlagenhaufer, Gudden, Flatau and others have especially studied this 
 form. The development of some of these cases from an inflammatory proc- 
 ess, encephalomyelitis, is proved with considerable certainty by several 
 observers. I myself have observed such a case clinically and anatomically. 
 Oppenheim has repeatedly seen the development of a disseminated sclerosis 
 from an infectious myelo-encephalitis. 
 
 Differential Diagnosis. — Many affections are to be considered in making 
 a differential diagnosis. Of especial importance is the differentiation from 
 hysteria. For a number of years, I have been emphasizing in my lectures, 
 again and again, the frequency of these confusions; Redlich and Oppenheim 
 have had the same experience. In every case of hysteria with ankle-clonus 
 the possibility of a disseminated sclerosis must be considered; furthermore, 
 also in every case of hysteric amaurosis, in quickly disappearing disturb- 
 ances in gait and pareses in hysterical individuals, should signs of an affec- 
 tion of the pyramidal tracts be present. If the Babinski phenomenon is 
 positive with dorsal flexion of the great toe, likewise Oppenheim' 's leg phenom- 
 enon, if there be spastic paresis in the lower extremities, an organic affec- 
 tion must be assumed in addition to hysteria and then multiple sclerosis 
 stands in the front rank of probability. This probability is increased if the 
 patient attacked is a young girl or a young woman. The further course, in 
 particular transient bladder disturbances, enables the physician to make a 
 diagnosis. 
 
 Differentiation from encephalitis may become very difficult, especially 
 if the symptoms of an acute pontile or medullary affection appear in young 
 persons without affection of the vessels or heart and there is no kidney 
 disease or syphilis. Detection of an elevation of temperature from a directly 
 preceding infectious disease would speak rather in behalf of the diagnosis 
 of an encephalo-myelitis. 
 
 If in a young person, the acute lesion (in the form of an apoplectic attack) 
 
348 DISEASES OF THE SPINAL CORD 
 
 affects the brain, the further course of the disease must be awaited if the 
 other changes discussed above are absent. If the symptoms disappear, and 
 absence of fever and a directly preceding serological factor is noted, a pro- 
 gressive paralysis or multiple sclerosis is probable. If the intellect is not 
 seriously disturbed by the attack, disseminated sclerosis is the more probable 
 diagnosis. 
 
 But if a lesion in the vessels (atheroma) is present, even with multiple 
 foci, the decision must be in favor of assuming the presence of a softening, 
 since multiple sclerosis is more apt to be found in young people. 
 
 Cerebrospinal syphilis can give rise to a disease picture very similar to 
 that of multiple sclerosis. Besides the anamnesis, the effect of specific 
 treatment will be of differential diagnostic value (though such treatment 
 must be used with caution). 
 
 Cases of solitary as well as multiple tumors of the brain have been ob- 
 served, which clinically bore a close resemblance to disseminated sclerosis 
 (Bruns, Nonne); in the latter even choked disc has been observed. Of 
 course this is not a common occurrence. The absence of disturbed intellect 
 and of constant headache militates against a diagnosis of a tumor, whereas 
 signs of constantly increasing intra-cranial pressure make the existence of 
 a tumor more probable. 
 
 Many years ago, Charcot separated multiple sclerosis and paralysis 
 agitans diagnostically in their clinical phenomena. Paralysis agitans is a 
 disease occurring in older persons; the tremor of shaking palsy is unlike 
 intention tremor; nystagmus and scanning speech do not belong to 
 paralysis agitans, while the general rigidity is not peculiar to multiple 
 sclerosis. 
 
 Friedreich's disease, or really heredo-ataxie cerebelleuse (Marie) which is 
 distinguished from it by many authors, may closely resemble certain stages 
 of multiple sclerosis. In heredo-ataxia there is nystagmus and a disturb- 
 ance in speech, which recalls scanning speech; the tendon reflexes may be 
 increased. A choreiform tremor can affect almost the entire musculature 
 of the body and become intensified upon motion. The gait, however, is 
 ataxic, occasionally a peculiar club foot develops and an optic atrophy, which 
 does not improve. 
 
 Progressive paralysis quite often exhibits a certain similarity to multiple 
 sclerosis. But the stumbling over syllables, the reflex pupillary rigidity, 
 the trembling speech, early psychic disturbances, unequal innervation of 
 the facial musculature, and the trembling of the lips are peculiar characteris- 
 tics of the paralysis and not of multiple sclerosis. 
 
 Differentiation from the affection, which was described by Westphal as 
 pseudo-sclerosis and later was specially studied by Striimpell and v. Frankl- 
 Hochwart, may prove difficult. These are comparatively rare cases, pre- 
 senting many of the clinical symptoms of disseminated sclerosis, while no 
 
MULTIPLE INSULAR SCLEROSIS 349 
 
 anatomic change can be found postmortem. This affection is distinguished 
 from multiple sclerosis by the absence of nystagmus, of the optic affection 
 and also of the genuine spastic phenomena. The early development and 
 the severe psychic disturbances (dementia) are conspicuous; Strilmpell 
 emphasized, besides, the fact that the tremor appeared even during rest, 
 was slower, but of greater amplitude. The active movements also are decid- 
 edly retarded. Speech is scanning, but also clearly dysarthric. Epilepti- 
 form and apoplectiform attacks are present in almost every case. On the 
 basis of these phenomena, correct diagnoses were made in vita by Strilmpell 
 and v. Frankl-Hochwart. 
 
 At present, pseudo-sclerosis cannot be distinguished from genuine diffuse 
 cerebral sclerosis with degeneration of the pyramidal tracts (Striimpell, 
 Oppenheim) . The latter author is also of the opinion that the combination 
 of progressive spastic paralysis with progressive dementia in childhood 
 must arouse a suspicion of diffuse cerebral sclerosis. 
 
 Therapy. — One is, alas, comparatively helpless, face to face with the 
 development of the disease, and can only sometimes, guided by a knowledge 
 of the peculiarities of its course, obtain remissions. 
 
 I have seen but few benefits follow the use of medicines. With anaemic 
 persons, especially in cases of a low state of nutrition, preparations of iron 
 and arsenic may well be used. In such cases I prefer ferratin, ferrum cacody- 
 licum or ferrum lacticum in combination with preparations of quinine, or 
 prescribe Blaud's , pills; arsenic I prefer to administer subcutaneously, in- 
 tramuscularly, as" sodium cacodylate (every other day 0.02 in a watery 
 solution), or as atoxyl (every other day 0.05-0.10 in a watery solution). Op- 
 penheim has seen bad results from mercurial treatment. 
 
 Of great importance is the carrying out of a strict rest cure. In poorly 
 nourished patients synchronous forced feeding is of considerable value. 
 The use of (not prolonged) lukewarm baths or baths of carbonic acid (lasting 
 from 10 to 15 minutes, temperature between 88° and 94 F.) seems to me 
 advantageous since they often influence the rigidity very favorably. If the 
 treatment by forced feeding and rest is continued long enough, and the patient 
 takes very good care of himself afterwards, a long remission may appear. At 
 present I am treating a young woman, who was paraplegic four years ago, 
 had bladder trouble and had so far recovered by means of forced feeding 
 and rest, that she could manage the household for years, until over-exertion 
 and excitement paved the way for a return of the paraplegia. Such long 
 remissions I have repeatedly seen. 
 
 I have seen strikingly favorable results from treatment by baths in Bad 
 Gastein. 
 
 Massage seems to act favorably. Oppenheim recommends that passive 
 movements be used in the bath. 
 
 Steam baths, hot baths, affect unfavorably the course of the disease; 
 
35© DISEASES OF THE SPINAL CORD 
 
 deterioration may follow immediately upon the bath. The same holds 
 true of all exciting hydriatic and other procedures. 
 
 Physical over-exertion may induce rapid deterioration; likewise under- 
 nutrition and psychic excitement may cause rapid progress of the disease. 
 
 Strong spices, highly seasoned food and alcoholic drinks are to be avoided. 
 
 Sexual excesses in one of my cases, caused in a short time complete 
 development of the disease which had been up to then but slightly 
 troublesome. 
 
 The abuse of tobacco also seems to be followed by an advance in the 
 disease. 
 
 4. SYRINGOMYELIA, SPINAL GLIOSIS 
 
 BY 
 
 HERMANN SCHLESINGER (Vienna) 
 
 Pathological conditions of different aetiology are embraced by the term 
 syringomyelia. They lead to the formation of long, extensive cavities, 
 which with special frequency occupy the central sections of the spinal cord. 
 They also cause considerable tumor-like proliferations of the glia. We 
 are dealing altogether with progressive processes; the stationary or retrogres- 
 sive cystic degenerations of the spinal cord are to be separated from syrin- 
 gomyelia (Kienbock) . The clinical picture is very variable, but permits 
 common traits to be recognized in the individual cases. 
 
 Pathological Anatomy and Pathogenesis 
 
 The spinal cord is frequently flattened out for long stretches, and much 
 reduced in substance; it is true, it frequently appears swollen in smaller 
 sections. If the cavity extends to the medulla oblongata, the latter becomes 
 very asymmetrical. Only in a minority of cases does one find a diffuse tumor- 
 like swelling of the spinal cord with cavity formation. In cross-section, one 
 sees a cavity occupying the central portion or even a large part of the dorsal 
 portion of the spinal cord; the size of the cavity varies greatly at levels but 
 little distant from each other. Frequently there are no large cavities 
 at all and instead one finds a long extended tumor, which occupies the 
 central area of the spinal cord, appearing now like a hard narrow glia 
 tube, now presenting a decidedly tumorous appearance. Pure unilateral 
 localization of the disease is rare; even then the gray matter is preferably 
 attacked (posterior horn). Most frequently, the cervical portion of the cord, 
 the cervical enlargement and the upper dorsal segment are the seat of the 
 lesion. Often, the extent, lengthwise, is very considerable and the changes 
 go far down into the lumbar segment, indeed even into the sacral portion at 
 one end and into the bulbus medullas at the other. Cases in which the dis- 
 
MORBID ANATOMY OF SYRINGOMYELIA 
 
 35i 
 
 ease is limited to the lowest sections of the spinal cord or to the medulla 
 oblongata alone, are of rare occurrence. 
 
 Most frequently injured are — the region of the central canal, the posterior 
 horns and the posterior columns; the latter are sometimes, for long stretches, 
 entirely swallowed up in the formation of the tumor or cavity, but usually 
 suffer extensive destruction only in their ventral parts (Fig. 76). The lateral 
 pyramidal tracts are, as a rule, interrupted at several points, and show 
 descending degeneration on one or both sides. The other parts of the cross- 
 section, as the lateral cerebellar tracts and the anterior columns, are affected 
 less frequently, but at any rate occasionally, while the anterior horns are 
 affected quite frequently. ' | 
 
 In the medulla oblongata, the lesion very rarely (Spiller describes such a 
 case) extends beyond the lower margin of the pons in the direction of the 
 
 Fig. 76. — Syringomyelia. Cavity behind the central canal. {After Schmaus.) 
 
 cerebrum. The changes occupy, preferably, definite places of the cross- 
 section (H. Schlesinger) ; usually either lateral or medial fissures are present. 
 As a rule, the lemniscus, the nuclei of the vagus, the accessorius, glosso- 
 pharyngeus, hypoglossus are affected, often, also, the spinal root of the fifth 
 nerve is destroyed (Fig. 77) {Schlesinger, Hatschek, A. Westphal, Philipp- 
 Oberthiir). 
 
 The tumor is built up of glia cells and glia fibres ; likewise the connective- 
 tissue elements play an important role. The cavities frequently have an 
 ependymal lining for a shorter or longer part of their course. 
 
 The origin of syringomyelias through the breaking down of these tumor- 
 like glioses was pointed out first by Simon, Westphal, later by Schultze, 
 Hoffmann, Oppenheim, Schlesinger and others. 
 
 The condition is termed a hydromyelia, if the cavity is lined throughout 
 with ependyma, a syringomyelic gliosis (Schlesinger), if the cavities are 
 combined with gliosis or have originated in the latter. The fissures of the 
 
352 DISEASES OF THE SPINAL CORD 
 
 spinal cord are surrounded by indurated masses of glia connective tissue, 
 while the syringobulbia is, as a rule, bounded by degenerating nerve tissue. 
 In the cavities and also in their walls are often found residues of hemor- 
 rhages and changes in the vessels. The glioses do not extend to the men- 
 inges. If extensive meningeal changes with cavity formations do exist, the 
 condition is not one of glio-syringomyelia. 
 
 As predisposing to the development of syringomyelia, we might consider 
 anomalies in the development of the entire central nervous system or of the 
 central canal (Leyden) and its immediate vicinity. Most likely glioses 
 (tumor-like, degenerating proliferations of glia) come from a congenital or 
 early acquired disposition (Hoffmann, H. Schlesinger) . Traumatism seems 
 occasionally to lead to the development of glioses {Minor, Westphal), but a 
 certain predisposition is probably essential. 
 
 Fig. 77. — Syringobulbia with degeneration of the lemniscus. {After Schmaus.) 
 
 F. Schultze has made it appear probable that traumatisms during birth 
 form the starting point of the affection. In compression of the spinal cord, 
 a syringomyelia is found occasionally above the place of compression. 
 Infectious diseases bring a gliosis in their train relatively often. Syphilis 
 (Schwarz, Simon) and meningitis may be followed by syringomyelia; possibly 
 meningeal and spinal affections are co-ordinated (Midler, Meder and others) . 
 Changes in the vessels can also, especially in advanced age, cause formation 
 of fissures (without gliosis). The aetiological role of inflammatory spinal 
 processes, for instance, myelitis (Joffroy, Hallopeau), for the origin of syringo- 
 myelia is still debatable. 
 
 For syringobulbia, changes in the vessels are of importance, as well as 
 anomalies in. development. 
 
 The anatomic aetiology of syringomyelia is therefore not uniform. 
 
 Besides infections, intoxications might also be an aetiological factor here 
 and there. But this influence is occasionally exaggerated. For the pro- 
 duction of a syringomyelia by peripheral traumata there is, as yet, no proof. 
 
SYRINGOMYELIA 
 
 353 
 
 Symptoms. — The clinical picture of syringomyelia is extraordinarily 
 varied. The phenomena that come under observation belong preponder- 
 antly to the following groups of symptoms : i. Phenomena in the domain 
 of the motor nerves; 2. disturbances in sensation; 3. vasomotor trophic 
 disturbances; 4. bulbar symptoms. 
 
 The motor disturbances are mainly muscular atrophies in the upper and 
 frequently, also, spastic conditions in the lower extremities. 
 
 The muscular atrophy begins most frequently insidiously in the mus- 
 culature of the hand. No other spinal affection leads so frequently in young 
 persons to atrophy of the smaller muscles of the hand. Since the muscles 
 do not always become atrophied in the same order, various positions of the 
 hand can result. The interosseous spaces frequently sink in, the fingers 
 
 Fig. 78. — Cheiromegaly in syringomyelia (as compared with the normal hand). 
 
 are over-extended at the metacarpo-phalangeal joints, flexed at the inter- 
 phalangeal joints, the tendons of the flexors become prominent in the hollow 
 of the hand, thenar and antithenar muscles become atrophied, so that the 
 position known as " claw-hand" results, or through atrophy of the ball of 
 the thumb and the antithenar eminence, with a change in the position of 
 the thumb, there develops the "ape's hand." Or through early paralysis 
 of the flexors of the wrist-joint, the dorsal musculature of the forearm 
 becomes the stronger, thus causing the development of the so-called 
 "preacher's hand." 
 
 In many cases, however, the muscular atrophy begins in the muscles 
 about the shoulder-girdle and extends to the musculature of the arm or of 
 the hand only after a longer time. Scapular "angel's wings" are very 
 23 
 
354 DISEASES OF THE SPINAL CORD 
 
 frequent in these cases since the muscles, that hold the scapula in its position, 
 become atrophied. The cucullaris is frequently atrophied only in its- 
 lower portion. 
 
 It is much less common for atrophy to appear early in the lower extrem- 
 ities; it can then lead to the formation of a pes equinus. The atrophies are 
 mostly asymmetrical. 
 
 In the atrophic muscles fibrillary twitching is commonly observed. 
 Reactions of degeneration cannot always be demonstrated. 
 
 Rigidity is often present in one or both of the lower extremities. The 
 rigidity can spread to the larger part of the musculature of the body, so that 
 the patients find themselves unable to move (Raymond, Dej trine, Redlich). 
 
 The tendon reflexes in the lower extremities are increased. Ankle 
 clonus is regularly present. In the areas of the muscular atrophies, the 
 tendon reflexes are usually diminished. 
 
 Oppenheim's leg phenomenon and the Babinski toe reflex (dorsal flexion 
 of the great toe) are present. 
 
 Spontaneous movements are observable in all stages of the disease, 
 especially in the fingers and thumbs (Schwarz). 
 
 In muscles, not wholly able to perform their functions, there occasionally 
 appear symptoms peculiar to myotonia, a condition that I designated as 
 myotonia syringomyelica (observed by Rybalkin, Fuchs and others). 
 
 The disturbance in sensibility is only partial in the great majority of 
 cases. As a rule, analgesia and thermo-anaesthesia are present, while the 
 other sensations suffer qualitatively none or but slight disturbances (" syringo- 
 myelic dissociation of sensation," first established by Kahler and F. Schultze). 
 The partial paralysis of sensation is demonstrable over a larger or smaller 
 area of the surface of the body. It shows as a rule (Laehr, Hahn, Dtjerine, 
 v. Solder, H. Schlesinger and others), segmental arrangement and also an 
 extension corresponding to the segments of the spinal cord. The area of 
 its extension does not necessarily correspond with that of the localization of 
 the muscular atrophies. The disturbances in sensibility may also affect 
 the pain sense alone, even the sensation for cold and heat alone. 
 
 The defects in sensibility are also asymmetrical; a sensory hemiplegia 
 has been repeatedly observed. 
 
 The thermo-anaesthesia leads to the burns that are so frequent in syringo- 
 myelia; they occur in typical places (hands, fingers, shoulder blade, buttocks). 
 Tactile sensibility and the muscular sense are not infrequently disturbed, 
 but the syringomyelic dissociation dominates. 
 
 In the initial stage, sensory irritation' phenomena (paresthesias affecting 
 the temperature sense) are usually present. 
 
 The sensibility of the bones is often considerably lowered (Egger, 
 Neutra) . 
 
 The trophic disturbances affect the skin, bones and joints. 
 
SYMPTOMATOLOGY OF SYRINGOMYELIA 355 
 
 The affections of the skin are so manifold, that practically the entire 
 subject matter of dermatology would have to be discussed, if one wished to 
 enter upon this phase. 
 
 The urticaria-like eruptions (dermographism), the formation of bullae 
 which always return in the same places on the body, are of especial impor- 
 tance. Decided thickenings (up to one centimeter) in the skin of the palm 
 of the hand approach to the forms of Morvan's type. Deep rhagades with 
 but slight tendency to heal, complicate this change. Vitiligo, sclerodermic 
 changes, discolorations of the skin, especially in the hands, are common. 
 Peculiar is the "main succulente" described by Marinesco, in which through 
 a transformation of the tissue of the subcutis, the skin has a pasty appearance, 
 the atrophies being masked. But there is actually no edema of the skin. 
 The change takes place also in the skin of the fingers. Very frequently 
 there develop in the fingers painless whitlows, which, if repeated, cause 
 extraordinary malformations of the fingers and the hand. Not rarely, in 
 these painless inflammatory processes, phalanges are lost. The nails become 
 misshapen, claw-like, rudimentary. Perforating ulcer of the sole of the foot 
 is rarer and must always remind one of a process which clinically closely 
 resembles syringomyelia — namely, leprosy. 
 
 Extensive spontaneous gangrenes occur, but are not peculiar to syringo- 
 myelia. 
 
 The disturbances in perspiration are very interesting. The secretion of 
 sweat is increased, or anidrosis develops. I also have described "paradoxic 
 sweat secretion," perspiring under the influence of cold. The anomalies of 
 perspiration affect only individual sections of the surface of the body, the 
 demarcation of which is parallel to the spread of segmental disturbances in 
 sensibility. 
 
 The most striking affections of the bones are spontaneous fractures, which 
 occur preferably in the lower extremities; their whole course is unattended by 
 pain and they result mostly from slight traumatism. Union often takes 
 place slowly with formation of a large amount of callus. The bones of the 
 forearm are preferred. 
 
 The fracture is remarkably often a pure transverse fracture (Gnesda). 
 Tedesko found in the systematic X-ray testing for transparency in the bones 
 of syringomyelic patients many cases with changed structure. 
 
 Spontaneous bone necroses of varying extent have been described several 
 times, likewise formation of multiple exostoses. 
 
 Scoliosis is often present, possibly in a quarter of the cases (Bernhardt) ; 
 it can attain high degrees, is combined with kyphosis and predominant in the 
 thoracic spinal column. The kypho-scoliosis usually develops slowly, but 
 may be an early symptom (Charcot, Oppenheim, Roth). 
 
 The chest is often deformed, a trough-like sinking in at the upper sternal 
 
356 DISEASES OF THE SPINAL CORD 
 
 end and the neighboring ribs ("Thorax en bateau" — Marie, Astie) is con- 
 sidered characteristic for syringomyelia. 
 
 In the course of the disease, a hand occasionally grows to gigantic size, 
 but not regularly; cheiromegaly (Hofmann, Marie) (Fig. 78). The partial 
 gigantic growth may also affect one foot — podomegaly (H. Schlesinger). 
 The typical picture of acromegaly is, however, not simulated by these 
 conditions. 
 
 • ( Bladder and rectal disturbances belong to the early symptoms only in the 
 lumbo-sacral forms of syringomyelia; otherwise they develop, if at all, only 
 in the later stages of the disease. Sphincteric spasm, afterwards incontinen- 
 tia urinae are the most frequent anomalies. The cystitis may run a wholly 
 painless course. 
 
 Diabetes insipidus, glycosuria, were observed in a number of cases. I 
 have seen nephrolithiasis in several cases. 
 
 Disturbances in the realm of the sexual sphere are unusual. 
 Hemiatrophia faciei has been observed as a striking complication in 
 several cases. 
 
 Bulbar lesions are common clinically as well as anatomically. They are 
 either unilateral or asymmetric. 
 
 The tongue is often affected. Then there develops a hemiatrophy with 
 wrinkling of the mucous membrane, fibrillary twitchings and considerable 
 diminution in size of one-half of the tongue (Lamacq, Maixner, Hitzig, 
 Raymond, Schlesinger) . 
 
 Hemiatrophia linguse is often combined with unilateral paralysis of the 
 musculature of the palate and the larynx, so that the symptom-complex of 
 unilateral bulbar paralysis is present. 
 
 The disturbances in deglutition are occasionally considerable: faulty 
 swallowing, regurgitation of liquids through the nose, remaining of bits of 
 food upon the base of the tongue. They may be transitory. 
 
 The laryngeal paralyses are characterized by a total palsy of the recurrent 
 laryngeal nerve with simultaneous paresis of the soft palate and of the 
 muscles of deglutition (H. Schlesinger, Baurovicz) . Atrophy of a vocal cord 
 is common; the external branch of the recurrens need not be injured. 
 
 Paralyses in taste occur, sometimes in individual qualities of taste. 
 Speech may be decidedly bulbar or hoarse with slight change of voice 
 (consequence of the recurrens paralysis). 
 
 Often the bulbar symptoms appear with an intense attack of vertigo with- 
 out loss of consciousness. The feeling of dizziness is of extraordinary vio- 
 lence, so that the patients fall to the ground. Immediately after this feeling 
 of vertigo, the bulbar paralysis appears, or the symptoms of it already 
 present become more progressive. ' 
 
 In many cases, a unilateral lesion of the sensory trigeminus is the first 
 symptom of bulbar disease (the spinal trigeminal root is attacked). The 
 
SYMPTOMATOLOGY OF SYRINGOMYELIA 
 
 357 
 
 extension of the disturbances in sensation in the scalp, and in the face follows 
 with sharp boundaries that are not to be found in peripheral trigeminus 
 lesion. The disturbance in sensation, which in most of the cases is partial, 
 is bounded, as it advances, by lines which are packed closely into each 
 other, in the form of an onion, about the mouth or the nose, and which have 
 
 Fig. 79. — Paralysis of the sympathetic at the left side in a case of unilaterally localized 
 syringomyelia (gliosis unilateralis) . (After Heinr. Curschmann.) 
 
 been closely studied by v. Solder and myself. This type of disturbance in 
 sensibility corresponds to the segmental arrangement of the elemental con- 
 stituents of the trigeminus. 
 
 First, in regular order, the laterally situated parts of the skin of the face, 
 and only towards the end, the most medially situated sections become anaes- 
 thetic, or only analgetic and thermo -anaesthetic. 
 
358 DISEASES OF THE SPINAL CORD 
 
 Neuralgia-like pains in the face are present, especially in the beginning of 
 the bulbar affection. 
 
 Trophic disturbances (corneal affections)- which often accompany a tri- 
 geminus lesion, are rare. 
 
 Facial paralyses are rarer, may strike now only the buccal branch, now 
 the entire facialis. The paralysis is located on the side of the more pro- 
 nounced defects in sensibility in the trunk. 
 
 The ocular disturbances are of various kinds. Very common is nystagmus 
 or nystagmus-like twitchings in turning the eyes to either side. Of paralysis 
 of the muscles of the eye, the abducens paralyses are most frequent; the 
 Argyll-Robertson phenomenon, which has been observed several times, is, as 
 a rule, to be traced back to a complicating disease (tabes or progressive 
 paralysis) . 
 
 Very frequently, in about one-sixth of the cases, a paralysis of the sympa- 
 thetic with all its typical phenomena (sunken eyeball, moderate degree of 
 ptosis, contraction of pupils, which retain the ability to react) is present 
 (Fig. 79). Besides, there are vasomotor phenomena in the same half of 
 the face and unilateral disturbances in the secretion of sweat (anidrosis or 
 hyperidrosis) . 
 
 The visual field, in the great majority of cases, where there is no com- 
 plication with hysteria, is normal. Neuritis and choked disc have been 
 described several times. 
 
 The bulbar disturbances, therefore, affect mainly the fifth to the twelfth 
 cerebral nerves. 
 
 Common Clinical Types. — Very frequently one sees a syringomyelia 
 with the "classic symptoms" when the disease is confined to the cervical 
 segment of the cord. Then there are present, usually, muscular atrophy in 
 the hands of the Aran-Duchenne type, dissociated disturbances in sensibility 
 and trophic-secretory disturbances in the upper half of the trunk and the 
 upper extremities. Or the muscular atrophy begins in the musculature of 
 the shoulder-girdle and is accompanied in earlier or later stages by disturb- 
 ances in sensation (humero-scapula type). 
 
 Often in the early stages of the disease, the disturbances in sensibility 
 are but slightly developed and their existence demonstrated with difficulty. 
 
 Symptoms of spastic spinal paralysis, or of an amyotrophic lateral 
 sclerosis are not very rare. In both cases disturbances in sensation may 
 long be absent or be only slightly developed, and the diagnosis will have to 
 be made from trophic disturbances of the skin, the bones, or the joints. 
 
 In the lower extremities also, onset with the well-known triad of symp- 
 toms is possible. Bladder disturbances are more common in these forms 
 than in others, just as is perforating ulcer of the foot. The syringomyelic 
 pes varo-equinus is usually found with this form (lumbosacral syringomyelia) . 
 
 I have already mentioned the syringomyelia, that is confined to the 
 
TYPES OF SYRINGOMYELIA 
 
 359 
 
 lowest segments of the spinal cord {sacral form) . In one case observed by 
 me, there were constantly present recurring perforating ulcers in both heels, 
 disturbances in the sexual sphere, loss of libido and ability of erection, 
 
 Fig. 80. — Syringomyelia of the Morvan type. {After Heinr. Curschmann.) 
 
 partial paralysis of sensation in the perineum and about the anus, loss of 
 the feeling of a full bladder and loss of the Achilles tendon reflex. 
 
 Beginning of the disease in the medulla oblongata, and a later affection of 
 the medulla spinalis is far less frequent than secondary syringobulbia. 
 
 Sometimes the gliosis attacks a posterior horn to a considerable extent 
 
360 DISEASES OF THE SPINAL CORD 
 
 lengthwise (Rossolimo, Oppenheim) ; then there occur unilateral disturbances 
 in sensibility, which remind one very much of hysteric disturbance. Only 
 in rare isolated cases has a general anaesthesia been observed in syringomyelia. 
 
 Through the predominance of trophic phenomena, peculiar clinical forms 
 are created, as the osteo -arthritic form due to early and extensive changes in 
 bones and joints. Cases with serious malformations of the fingers and 
 hands, as a result of repeated whitlows and phlegmonous processes are 
 particularly frequently observed. These forms with unsightly thickenings 
 and distortions of distal parts of the extremities and severe disturbance of 
 tactile sensibility are designated as Morvan's symptom complex (Fig. 80). 
 
 The tabetic type may originate from a combination of tabes and syringo- 
 myelia (for instance tabetic phenomena in the legs, syringomyelic in the 
 arms) or by localization of a gliosis in the posterior columns. 
 
 Besides these types, there are rarer forms, caused by peculiar arrange- 
 ments of the gliosis or by combination with other diseases, as gliosis cruciata 
 {Oppenheim) with typical disturbances in the upper and lower limbs of oppo- 
 site sides. The pachymeningitic forms soon lead to the phenomena of 
 transverse section of the cord with motor and sensory paralysis of the lower 
 half of the body. 
 
 Combination with hydrocephalus is not unusual and has been observed 
 several times in association with disturbances in the development of the 
 bones and the soft parts. 
 
 Oppenheim and Marburg have repeatedly seen "cervical ribs" combined 
 with syringomyelia. Syringomyelia has repeatedly been observed (I have 
 seen it) with cerebral tumors. 
 
 The very common combination with hysteria is clinically important, as it 
 modifies many of the phenomena of syringomyelia. 
 
 Onset, Age, Course. — The disease, as a rule, begins stealthily, exhibiting 
 a preference for young persons. I have several times been able to recognize 
 it clinically in young children. An onset in very old age or even in a moder- 
 ately advanced age is less frequent, though the presence of the disease even in 
 old age is not rare. It is usual, however, to find that the phenomena began 
 a long time before. The disease not infrequently lasts from 20 to 40 years. 
 If the disturbances are not serious, it is usually the injuries that drive the 
 patient to the physician. That is why the surgeon is so frequently the 
 first to see the patient. It is comprehensible that some vocations lead to 
 an earlier discovery of the disease (thus cooks and stokers seek a doctor 
 because of their frequent burns) . 
 
 Syringomyelia is found everywhere, provided that its variable clinical 
 picture is known. In my opinion it stands third or possibly fourth among 
 all the diseases of the spinal cord with reference to the frequency of its 
 occurrence. 
 
 The affection has, it is true, a general progressive character, but long 
 
DIFFERENTIAL DIAGNOSIS OF SYRINGOMYELIA 361 
 
 stationary periods, lasting even for many years are seen in many cases. 
 Very long remissions have been repeatedly described (Hatschek, Bruce, and 
 others). Acute exacerbations are frequent, being observed especially with 
 localization of the disease in the medulla oblongata and may possibly be 
 the result of hemorrhages, softenings or edema. 
 
 Complications may cause death. Among them cystitis with ascending 
 inflammation is specially important, as well as purulent infections of the 
 subcutaneous connective tissue and of the joints. I have frequently seen 
 nephrolithiasis with syringomyelia. 
 
 In spite of the frequency of syringobulbia, the fatal outcome is but 
 very rarely caused by bulbar disturbances. Intercurrent diseases, especially 
 tuberculosis and typhoid, add a large contingent to the number of fatal cases. 
 
 Differential diagnosis must take into consideration an extraordinarily 
 large number of diseases. Among these the most important practically 
 is leprosy. A very large number of monographs are concerned with this 
 theme, which is of immense importance for the question of the spread of 
 leprosy in civilized countries. 
 
 Among the most important we may mention those of Laehr, Zambaco, 
 F. Schultze, v. During, Lie, Bergmann, Jeanselme and H. Schlesinger. 
 
 The diagnostic difficulties are increased by the fact that the combination 
 of leprosy with syringomyelia (Gerber-Matzenauer) has been observed with 
 certainty. It is true that such a connection was formerly assumed several 
 times, even postulated as causal (Zambaco, Marestang), but this assertion 
 has not been proven. It was supported by the clinical similarity which 
 especially Morvan's type of syringomyelia exhibits to a form of leprosy, 
 but in leprosy, a spinal gliosis is absent from the anatomic phenomena and 
 in syringomyelia, the bacilli of leprosy are absent. 
 
 According to the present status of the question, the following factors 
 are to be considered: the origin is now less decisive since new leprous foci 
 are continually being discovered; at any rate, a case originating from a 
 region which has always been free of leprosy, speaks against this disease. 
 Nerve thickenings, especially the spindle-shaped, are peculiar to leprosy; in 
 excised pieces the presence of leprosy bacilli may occasionally be demon- 
 strated. They are early and regularly found in the N. auricularis magnus. 
 Oval cicatricial patches of the skin devoid of pigment with anaesthesia, 
 which have come from pemphigus bullae, point to leprosy. Tubercular 
 affections of the skin, which are spread over a large part of the surface of 
 the body and also attack the face, are to be similarly interpreted, likewise 
 attacks of fever, especially during progression of such eruptions. A bacterio- 
 logical examination of the nasal secretions may often early reveal the specific 
 bacillus in leprosy (G. Sticker) . Peripheral facial paralysis is rare in syringo- . 
 myelia, frequent in leprosy, especially with marked participation of the 
 frontal branch of the facial. Even specific disturbances of the eyes are not 
 
362 DISEASES OF THE SPINAL CORD 
 
 exactly rare in the latter affection. For gliosis the following speak: bladder 
 and rectal disturbances, increase of the tendon reflexes, spastic phenomena 
 in the lower extremities, severe multiple painless joint affections, the humero- 
 scapular type of muscular atrophy, nystagmus, attacks of vertigo, bulbar 
 symptoms, disturbances in perspiration, segmental disturbances in sensibility, 
 whereas the sensory disturbances in leprosy occur more in discrete areas. 
 
 Since syringomyelia may present the symptoms of scleroderma as well 
 as of Raynaud's disease and also of pemphigus, differentiation will occasion- 
 ally be difficult, but can usually be decided on the basis, of concomitant 
 symptoms, since gliosis beside these symptoms, exhibits others more peculiar 
 to itself (for instance spastic symptoms, partial paralysis of sensation, 
 bulbar symptoms, etc.), in short, symptoms that do not appear in skin 
 affections. 
 
 Arthritis deformans is distinguished from atypical forms of central 
 gliosis by the absence of analgesia, and partial macrosomia by the absence 
 of disturbances in sensibility. 
 
 Hysteria is occasionally very difficult to distinguish from syringomyelia, 
 especially from the forms with disturbances in sensation without muscular 
 atrophy. The bulbar symptoms, the increase in the tendon reflexes, 
 sympathetic paralysis, trophic disturbances will, as a rule, make diagnosis 
 easy, though hysteria is often combined with syringomyelia. 
 
 Root and plexus neuritis is distinguished by absence of the cord phenomena 
 (reflexes in the lower extremities unchanged), by the violent irritation 
 phenomena, and the uniform disturbance of all qualities of sensation. 
 
 Polyneuritis only rarely causes confusion, since its onset is much more 
 quick and it is usually bilateral from the beginning. 
 
 A spondylitis tuberculosa can more easily simulate the picture of a syringo- 
 myelia. The muscular atrophies may be similar, also the dissociation of 
 sensation may be analogous to that in syringomyelia ; even the oculo-pupillary 
 symptoms (sympathetic paralysis) occur. But the affection of the bones is 
 recognizable as such by direct inspection or palpation or in the radiograph; 
 the spinal column is held rigidly, transverse section symptoms caused by the 
 cord disease develop rather quickly. 
 
 L ■* The separation from hcematomyelia will not be very difficult with a 
 knowledge of the history of the case (acute onset, especially after traumatism). 
 Furthermore the phenomena in haematomyelia have a tendency to become 
 less, in syringomyelia usually not. 
 
 Syringomyelia may be confused with tabes, since occasionally the clinical 
 picture of syringomyelia reminds one strongly of that of tabes. Tabetic 
 disturbances of the cerebral nerves (bilateral posticus paralysis, Argyll- 
 Robertson's phenomenon), loss of the tenderness to pressure in the ulnaris, 
 paralysis of the muscles of the eye, and especially oculomotor or bilateral 
 abducens paralyses will suggest tabes first, if the other symptoms are specially 
 
PROGNOSIS AND TREATMENT OF SYRINGOMYELIA 363 
 
 pronounced in the lower extremities. But if a partial paralysis of sensation 
 is permanently present in the lower limbs, if muscular atrophies exist, if the 
 disturbances in the muscular sense are not of a high degree, one will have to 
 think of syringomyelia, even if there be ataxia and loss of the patellar re- 
 flexes. Typical phenomena in the upper extremities will confirm with 
 certainty the diagnosis. 
 
 From the spinal amyotrophies (poliomyelitis chronica, progressive muscu- 
 lar atrophy of the Aran-Duchenne type, amyotrophic lateral sclerosis) 
 syringomyelia can be distinguished by the presence of dissociated disturb- 
 ances in sensation, trophic disturbances of the bones, joints and skin. Also 
 the one-sided and irregular bulbar affection in contradistinction to the last 
 named disease, the increase of the tendon reflexes in the lower extremities in 
 contradistinction to the former two diseases, present important signs in 
 regard to differential diagnosis. 
 
 Multiple insular sclerosis, upon longer observation, will nearly always act 
 clinically in a different way than syringomyelia. Especially the disturbances 
 in sensation are usually not permanent; trophic disturbances do not play an 
 important role, more extensive muscular atrophies are quite unusual. 
 
 Intramedullary tumors may give rise to considerable diagnostic difficulties. 
 The following factors, among others, are important: the course in tumor is 
 far more rapid than in syringomyelia. The Brown-Sequard symptom com- 
 plex is a frequent, if only transitory occurrence in tumors. The phenomena 
 of sensory irritation are usually more prominent and pareses or paralyses 
 develop far more quickly than in syringomyelia. Bladder and rectal dis- 
 turbances, also, are found early. Bulbar disturbances, if they be present, 
 are, as a rule, a very bad sign, whereas in syringomyelia, bulbar symptoms 
 may persist for many years. 
 
 The prognosis of the disease is quoad vitam not unfavorable, since even 
 when bulbar affections exist, life is usually prolonged for many years. Death 
 is caused, as a rule, by intercurrent diseases. 
 
 Treatment is more or less powerless to cope with the disease. It is 
 asserted that use of the X-rays on the spinal column, especially in cervical 
 syringomyelia, may check the progress of the disease. I have not as yet seen 
 any such effect on the progress of the affection. 
 
 Prophylactic measures are important. The patients must not expose 
 themselves too much to high or low temperature, since there is danger of 
 burning or freezing. Severe physical work should also be avoided as much as 
 possible, because of a tendency in the affection to the formation of haemato- 
 myelias and because of the possibility of spontaneous fractures. The use of 
 hot baths may bring about an acute exacerbation of the affection. 
 
 The muscular rigidity is influenced favorably by protracted lukewarm 
 baths. The addition of treatment by radium seems to me useful, likewise 
 the use of the baths at Gastein. 
 
364 DISEASES OF THE SPINAL CORD 
 
 If pains be present, the use of the modern antipyretics, especially of 
 pyramidon, citrophen or aspirin, is of service. 
 
 Arthropathies in case of suppuration or of too much effusion demand 
 surgical treatment. 
 
 5. ILEMATOMYELIA 
 
 BY 
 
 HERMANN SCHLESINGER (Vienna) 
 
 Hemorrhage into the spinal cord is a much rarer affection than hemorrhage 
 in the brain. We differentiate between primary and secondary hemorrhages 
 of the spinal cord, the latter of which takes place into a tissue that has already 
 been changed (for instance, tumors, inflammatory processes, etc.). Hsemato- 
 myelia preferably affects the gray matter; the posterior horns are more fre- 
 quently injured than the anterior horns. The hemorrhage spreads length- 
 wise in the spinal cord, may even occupy a large part of the medulla (tubular 
 hemorrhage), may occupy one or both sides of the cord, may injure the 
 anterior horn or the posterior horn alone, while the lateral columns are usually 
 spared (Minor). The localization and the spread of the hemorrhage, might, 
 as I assumed years ago, be dependent upon the loose, yielding structure of 
 the gray parts of the spinal cord. Later experimental investigations by 
 Goldscheider, Flatau support this view. The hemorrhages may take place in 
 any part of the spinal cord; they are most frequently found in the enlarge- 
 ments. The intrameningeal hemorrhages are mostly of less clinical im- 
 portance than the intracordal, as Thorbum and Stolper have shown. 
 
 Of the ^etiological factors traumatism is the most important (for about 
 9/10 of the cases, according to Oppenheim). According to Stolper, forced 
 flexion of the head forwards may cause a hsematomyelia (for instance, diving 
 head foremost) . In newly-born infants hsematomyelia was seen often after 
 Schultze's method of resuscitation of still-born children and difficult deliveries 
 (F. Schultze, Litzmanri). In children, after forced (Lorenz) reduction of 
 congenital dislocation of the hip-joint, hamiatomyelia of the conus was ob- 
 served (H. Schlesinger). After forcible muscular exertion, spinal hemor- 
 rhages have been observed, but a predisposition (for instance, haemophilia) 
 seems to be essential. In typhoid, hsematomyelia has been observed several 
 times, thus by Curschmann, A. Schiff. I saw the disease associated with 
 morbus maculosus Werlhoffii, other authors with pernicious anemia, etc. 
 Spontaneous hsematomyelias are exceedingly rare, but undoubtedly occur. 
 One of my patients developed it, while working on a farm, without 
 traumatism. 
 
 Symptoms. — They vary a great deal according to the spinal level and 
 to the extent of the hemorrhage. Common to all forms is the sudden onset 
 with the phenomena of an interruption in spinal conduction. The maximum 
 of the lesion, i. e., the greatest extent and intensity of the phenomena are 
 
SYMPTOMS OF HMMATOMYELIA 365 
 
 reached, usually, in the first few hours of the disease, far more rarely in the 
 first few days. Part of the symptoms, which are not at once visible (muscular 
 atrophies, trophic phenomena in the skin, etc.), are produced by the lesion, 
 once it is present, but need a certain amount of time for development. The 
 predilection (so striking anatomically) for the gray substance evokes very 
 peculiar symptom groupings, to which Minor especially has called attention. 
 Very often syringomyelic dissociation of sensibility , i. e., analgesia and thermo- 
 anesthesia, with other qualities of sensation retained or but slightly injured, 
 are demonstrable in smaller or larger portions of the surface of the body. 
 
 If the lesion is in the sacral cord, bladder and rectal disturbances and 
 occasionally anomalies in the sexual sphere, are added to the disturbances of 
 cutaneous sensibility in the perineum and about the anus. 
 
 With injury in the lumbar and lower dorsal segments of the cord, there 
 are usually added also paralyses of the musculature of the legs (severe atro- 
 phic paresis in one of my cases) and the tendon reflexes may show important 
 disturbances (loss or increase). Most frequently, the hasmatomyelia is 
 located in the cervical segment and evokes atrophic paralysis of the upper 
 extremities, whereas in the lower extremities, spastic paresis holds sway. 
 The extent and degree of muscular atrophy vary extraordinarily. When 
 the hasmatomyelia is deep-seated (lower cervical enlargement) the cervical 
 sympathetic of the same side is often affected (disturbances in sweat, oculo- 
 pupillary phenomena, vaso-motor disturbances in the face). 
 
 The Brown-Sequard symptom complex is developed with remarkable 
 frequency. As Minor, Oppenheim and others have shown, the disturbance 
 in sensation is partial; indeed, it may be for pain and temperature in one leg, 
 while the other is spastically paralyzed, and the upper extremity on the 
 opposite side is more or less atrophic. 
 
 Another form corresponds to the "classic" type of syringomyelia; there 
 is atrophic paralysis of an upper extremity and partial disturbance of 
 sensation in the same extremity. 
 
 Of irritation phenomena, initial pains in the back must be mentioned, 
 which, however, are rarely overpoweringly severe. 
 
 Course. — The disease attains its height, as I have already remarked, in 
 the first hours (Kienboeck). If serious complications exist (injuries to the 
 vertebral column, lesions of internal organs), these, as a rule, cause death. 
 Hsematomyelia, per se, even when situated high, is usually not fatal. If 
 observation be over a longer period of time, one sees a more or less rapid 
 retrogression of the symptoms, which, occasionally, is surprisingly complete. 
 In one of my cases (described by Labin) lesion of the cervical cord, paraplegia 
 of all four extremities was present, and disappeared completely; the disease 
 finally presented the clinical picture of a conus lesion. All phenomena, 
 which may be explained by blood compression, may retrogress, likewise 
 those symptoms that can be traced to an edema in the substance of the 
 
3 66 DISEASES OF THE SPINAL CORD 
 
 spinal cord. Finally, those symptoms persist which are caused by complete 
 destruction of spinal-cord substance. The permanent symptoms are 
 disturbances in sensibility, especially those of syringomyelic character, since 
 they are called forth by lesion of the gray axis of the spinal cord. To these 
 are to be added muscular atrophies, which owe their origin to destruction of 
 parts of the anterior horns; in the atrophic muscles, the electric excitability 
 is changed (reaction of degeneration). Real trophic disturbances seem to be 
 exceptional only, in haematomyelia. The muscular atrophies may be very 
 slight, and are therefore easily overlooked; this is a condition of special 
 importance in judging medico-legal accident cases (Oppenheim). 
 
 Diagnosis. Differential Diagnosis. — The diagnosis is, as a rule, made 
 easily because of the acute onset, the usually preceding traumatism, and the 
 beginning retrogression of the phenomena within a short time of the onset 
 of the disturbance. 
 
 From a differential diagnostic standpoint the diseases most concerned are 
 syringomyelia (cf. above), and acute myelitis. In the latter disease, I have 
 observed a most acute onset, with paralysis in several hours, and death within 
 a few days in three cases, and the literature furnishes similar observations. 
 In these cases, however, there is usually fever, and the spontaneous origin 
 also militates against haematomyelia. 
 
 The greater prominence of irritation symptoms (pains, rigidity of the 
 spinal column, sensitiveness of the muscles to pressure), particularly when the 
 paralytic phenomena are but slightly pronounced, points to hemorrhage in 
 the meninges (Haematorrhachis) . 
 
 In tumors accompanied by hemorrhage into the tumorous tissue, the 
 anamnesis, as a rule, points to a longer duration of the disease. 
 
 In poliomyelitis, the almost exclusive anterior horn symptoms and the 
 onset with fever, make the diagnosis possible. 
 
 The prognosis of the disease depends, as has been said before, on the 
 complications. It is better, when no bed-sores and no cystitis develop. 
 
 The duration of the disease may be considerable. I possess anatomic 
 preparations from a case that lasted 15 years (destruction of the sacral cord). 
 I know another case in which haematomyelia developed 17 years ago, and in 
 which I have been able to demonstrate the condition (Conus lesion) as 
 stationary for more than 10 years. 
 
 Treatment. — The most important therapeutic factor is absolute rest — 
 if possible, for several weeks. Oppenheim recommends lying on the stomach 
 or side (marked tendency to bed-sores). Internally, gelatin (2 drams daily 
 in watery solution), calcium chloride (20 grains daily in water) or adrenalin 
 (5 drops of the standard solution 4 times a day) are to be advised. Subcu- 
 taneous injections of ergotin may be beneficial. Especially in meningeal 
 conditions of irritation, and in full-blooded persons, leeches are indicated in 
 the region of the spinal column. 
 
FOCAL DISEASES OF THE SPINAL CORD 367 
 
 If atrophic paralyses develop, frequent lukewarm baths may be used; 
 hot baths are contraindicated. 
 
 Treatment by baths at Bad Gastein, Wildbad, Nauheim, Oeynhausen 
 are occasionally useful if the hemorrhage has existed some time (at least 
 5 to 6 weeks). But in treatment by baths, all the more strongly exciting 
 procedures, and especially hot baths are to be avoided. 
 
 6. FOCAL DISEASES OF THE SPINAL CORD DUE TO EXTRA- 
 AND INTRA -MEDULLARY AFFECTIONS 
 
 BY 
 
 R. FINKELNBURG (Bonn) 
 
 I. Traumatic Diseases of the Spinal Cord 
 
 (Changes Due to Crushing (Contusions) , Lacerations and Concussion) 
 Whenever the spinal column is injured either by direct or indirect violence, 
 that is, by a blow, contusion, by crushing or by traction upon it or by a fall 
 upon the head or the buttocks, the spinal cord may be injured also. This 
 will be particularly the case if, as a result of fracture of the body of a vertebra, 
 or of vertebral dislocation, there are permanent changes in the position of an 
 entire vertebra or of any of the parts thereof. The spinal cord may show 
 total or partial contusions and lacerations, root lesions with or without 
 hemorrhages into the membranes. But also after only momentary displace- 
 ment of one or more of the vertebral bodies, even if the vertebra has returned 
 to its natural position upon relaxation of the force influencing it, severe 
 pressure lesions of the cord may be caused. 
 
 The seat of a dislocation and distortion is most frequently the cervical 
 portion of the spinal column ; the former arises from forced flexion, with or 
 without simultaneous rotation of the neck, the latter not infrequently from 
 violent stretching of the spinal column. In isolated vertebral fractures, it is 
 a question either of so-called compression fractures, caused by destruction of 
 the body of a vertebra by forces exerted upon its axis, or of fractures in the 
 vertebral arches, which but rarely give rise to an injury of the cord and the 
 roots. Fractures in the body of a vertebra occur mainly in the region of 
 the lower dorsal and lumbar vertebrae. Isolated lesions of the inter-vertebral 
 discs are rare; they frequently accompany dislocations and fractures of the 
 vertebrae. 
 
 Apart from the serious injuries of the spinal cord resulting from perma- 
 nent or transitory compression, caused by vertebral injuries, even after simple 
 concussion to the spinal column, coarser or finer spinal-cord changes may arise. 
 The former consist in extra- or more frequently intra-medullary hemorrhages 
 and in softening of the substance of the spinal cord with possibly additional 
 chronic myelitis of the white and gray matter at a later period (cf. Fig. 81). 
 
3 68 
 
 DISEASES OF THE SPINAL CORD 
 
 Slight concussional injuries in the form of delicate changes in the gang- 
 lion cells and nerve fibres, as may also be evoked independently in experi- 
 menting on animals by concussion upon the spinal column, frequently dis- 
 appear entirely, after leading at first to serious phenomena of functional loss. 
 But occasionally in connection with such a commotio spinalis, even if the in- 
 cipient functional disturbances have disappeared, there develop insidious 
 
 degenerations of the spinal cord in the form 
 of a chronic myelitis. It is of special 
 practical importance, that after commotio 
 spinalis, in which the brain also is usually 
 co-affected, together with organic changes, 
 but also very frequently without them, 
 pure functional disturbances of the nervous 
 system may appear — traumatic neurasthe- 
 nias and hysterias. These commotion 
 neuroses were formerly spoken of as "rail- 
 way spine" since they were observed es- 
 pecially after railway collisions. 
 
 Anatomic Findings. — After severe 
 crushing there are found, in addition to 
 hemorrhages in the membranes and sub- 
 stance of the spinal cord, marked flatten- 
 ing, diminution, or even complete divi- 
 sion of the spinal cord. This is soon 
 followed by a hemorrhagic softening, in 
 the further course of which the destroyed 
 and liquefied remnants of the cord are 
 
 Fig. 81.— Fracture of the first lumbar- absorbed and partly taken up by granular 
 vertebra due to compression. {After Th. rf> ]i s 
 Kocher.) 
 
 In the vicinity of the lesion there ap- 
 pear inflammatory reaction phenomena; the glia cells and fibres swell and 
 multiply; the vessels are surrounded by granular cells, and occasionally 
 numerous round cells are to be seen in the area of destruction. The out- 
 come is a cicatricial focus, consisting of glia tissue with thickened vessels, 
 occasionally, also, with the formation of larger or smaller cysts. Ascend- 
 ing and descending degenerations are added to the picture. 
 
 Phenomena of the Disease 
 
 Immediately after every severe injury of the spinal cord, there appears, 
 during the first effects of shock, the symptomatic picture of a complete inter- 
 ruption in conduction, even when the spinal cord is but partly injured. It is 
 therefore only after the first shock phenomena have passed away, that we can 
 
FOCAL DISEASES OF THE SPINAL CORD 369 
 
 approach the therapeutically and prognostically important question : is there 
 a total or only a partial transverse lesion, is there present crushing or merely 
 compression of the spinal cord? 
 
 1. Complete Transverse Lesion 
 
 The most important sign of a complete transverse lesion is found in the 
 complete paralysis of the musculature of the body below the point of the lesion, 
 which appears immediately after the injury and remains permanently. At 
 the same time motor irritation phenomena, twitchings, spasms in the para- 
 lyzed region are wholly lacking, while they may appear near the upper 
 boundary. Even when the seat of the lesion is in the upper segments of 
 the spinal cord, the paralysis of the lower extremities, as a rule, is flaccid, 
 and not only during the period immediately following the injury, but 
 usually afterwards as well. 
 
 The tendon as well as the cutaneous reflexes are completely lost in total 
 lesions above the lumbar segment, not only immediately after the injury, 
 but often permanently. But permanent loss is by no means always the case. 
 The theory of Bastian, that every total transverse lesian of the spinal cord brings 
 with it a permanent loss of the tendon reflexes below the point of injury, is 
 disproven by a number of indisputable observations, in which, although 
 complete division of the spinal cord was anatomically demonstrated, the 
 tendon reflexes remained long after the injury and occasionally even showed 
 an increase. 
 
 The loss of sensation in all its qualities is complete, up to the level of that 
 region of the skin which is supplied by the destroyed segment of the spinal 
 cord. The sensory disturbances extend only then farther upwards than would 
 be expected, judging from the position of the injured segment, if by the crush- 
 ing the root-fibres originating in the higher segments and passing down 
 through the spinal canal are also destroyed. 
 
 This, however, is by no means always the case, since in an injury, which 
 strikes roots and cord simultaneously, the former are frequently less injured. 
 Sensory irritation phenomena in the form of radiating pains, hyper- and 
 paresthesias occur near the upper limit of the disturbance in sensation, never, 
 on the other hand, within the sensorily paralyzed region. 
 
 Disturbances of the bladder and bowels are always present and decided. 
 There is, at first, retention of urine, which later is followed by a periodic 
 reflex evacuation of the bladder. Bed-sores appear sooner or later. 
 
 2. Partial Lesions of the Cord 
 
 In these, the motor phenomena of paralysis appear, as a rule, in the 
 foreground of the disease picture in contradistinction to the usually less pro- 
 nounced sensory disturbances. In lesions, in which only a part of the 
 transverse section is affected, we see, a short time after the injury, the motor 
 24 
 
370 DISEASES OF THE SPINAL CORD 
 
 paralysis, which, at first is total, become transformed into one that is incom- 
 plete. At the same time, according to Kocher's observations, the parts of 
 the body lower down, that is, especially the lower extremities, are more 
 severely affected by the paralysis, than the upper parts, like the arms. An- 
 other important sign of a lesion of the cord that is only partial is the appear- 
 ance of motor irritation phenomena, of twitchings and early contractures in the 
 regions showing a motor paralysis. If the point of injury is above the lumbar 
 segment, there appears, as a rule, a spastic condition of the musculature and 
 a pathological increase of the tendon reflexes, also the Babinski phenomenon. 
 
 In partial destruction of the cord sensation even in pronounced paraplegia 
 may be but slightly disturbed; furthermore, the disturbance may at times be 
 confined to definite qualities in sensation only and does not extend nearly 
 so far upwards as the region of the motor paralysis. The appearance of 
 radiating pains and paresthesias in the' paralyzed limbs is, in a certain measure, 
 characteristic, in contradistinction to the total transverse lesions. If there are 
 only disturbances in sensation without motor paralysis, this indicates that 
 only a root injury is present. 
 
 Not very infrequently, the symptom complex of a Brown-Sequard uni- 
 lateral lesion develops in injuries due to stabbing, shooting, or crushing: 
 spinal hemiplegia on the side of the injury, with later appearing spastic phe- 
 nomena, and considerable exaggeration of the tendon reflexes; on the para- 
 lyzed side, there is found, in addition, a hyperesthesia and hyperalgesia of 
 the skin, and at first, paralysis of the blood-vessels. In the opposite side of 
 the body, which does not present motor disturbances, sensibility is impaired, 
 with the exception of the sense of position. Pain and temperature sensations 
 suffer most frequently and most severely. This typical picture of Brown- 
 Sequard paralysis is generally blurred because the injury does not as a rule 
 confine itself definitely to one-half of the spinal cord, or, because on account 
 of an added traumatic myelitis, the original semilesion becomes transformed 
 into one diffusely affecting the whole transverse area. 
 
 Disturbances of the bladder and bowels are frequent concomitants of an 
 incomplete transverse lesion: but they may be entirely absent in spite of 
 pronounced paralytic phenomena. In partial lesion of the cord, retention 
 of urine with completely preserved bladder sensation may be observed; 
 the sensation in many instances may even be morbidly increased, thus induc- 
 ing violent vesical and rectal tenesmus, and at the same time, an impossibility 
 of voluntarily evacuating the bladder and bowels. Priapism is no unusual 
 phenomenon in partial lesion; it may, however, be lacking even in total 
 transverse lesion. 
 
 For the general ascertainment, as to which of the main divisions of the 
 medulla spinalis is affected by complete or partial transverse lesion, the 
 following table gives sufficient data. The exact determination of the place 
 which is affected, in the absence of external injuries, depends above all, on 
 
FOCAL DISEASES OF THE SPINAL CORD 
 
 37i 
 
 the detection of motor and sensory phenomena of functional loss of definite 
 segments of the spinal cord. These are best shown in the tables of Edinger 
 and Starr-Brims, and the sensibility schemes of Edinger, Head, and Seiffer. 
 
 TABLE FOR THE GENERAL DETERMINATION OF THE PLACE OF TRANSVERSE 
 
 LESIONS {After Schultze) 
 
 Motor Sensory 
 
 Transverse injuries of the 
 cauda equina, cause: 
 
 Flaccid paralyses in the 
 sciatic region and the 
 sacral nerves only, or also 
 in the crural or obturator 
 
 Awzsthesias in the same 
 areas; in individual parts, 
 also parcesthesias, hyper- 
 cesthesias and pains. 
 
 Transverse injuries of the 
 lumbar enlargement, cause: 
 
 According to the place of 
 the injury flaccid paraly- 
 sis in the sacral nerves 
 only, or also in the lum- 
 bar (crural, obturat). 
 
 Like the above. 
 
 Transverse injuries of the 
 dorsal segment, cause: 
 
 Paralysis of the lower 
 extremities and individ- 
 ual parts of the trunk, 
 in lesions of the upper- 
 most parts also oculo- 
 pupillary symptoms. 
 Mostly spastic paralysis. 
 
 Anesthesias of the low- 
 er extremities and sym- 
 metrical parts of the 
 trunk. At the upper 
 boundary also hyperes- 
 thesias and pains. 
 
 Transverse injuries of the 
 cervical enlargement, cause: 
 
 Spastic paralysis of the 
 lower extremities; paraly- 
 sis of the muscles of the 
 trunk; partial or com- 
 plete flaccid or atrophic 
 paralysis of the muscles 
 of the upper extremity. 
 
 Anaesthesias of the low- 
 er extremities of the trunk 
 up to the height of the 
 shoulder, partly or entire- 
 ly of the upper extremities. 
 Hyperaesthesias and 
 pains. 
 
 Transverse injury of the 
 uppermost cervical segment, 
 causes: 
 
 Usually immediate death, or if the injury is at the 
 lower part and with paralysis of the phrenicus, fatal 
 termination after days or weeks. 
 
 Radiating pains espe- 
 cially in the n. occipit. 
 major. 
 
 In respect to bladder and 
 bowels and sexual function. 
 
 In respect to reflexes. 
 
 In respect to trophic dis- 
 turbances. 
 
 Severe paralysis, 
 cystitis. Impotence. 
 
 Later Loss of the cutaneous 
 
 and tendon reflexes in the 
 nerve areas concerned. 
 
 Reaction of degenera- 
 tion and wasting of the 
 muscles. Decubitus: tro- 
 phic disturbances of the 
 skin, joints and bones. 
 
372 
 
 DISEASES OF THE SPINAL CORD 
 
 Bladder, bowels, and sex- 
 ual function. 
 
 Reflexes. 
 
 Trophic disturbances. 
 
 The same as above. Im- 
 potence. 
 
 The same as above. 
 
 The same as above. 
 
 The same as above. Im- 
 potence. 
 
 Retention or exagger- 
 ation of the tendon and 
 cutaneous reflexes. Some- 
 times, also, absence of the 
 tendon reflexes. 
 
 No reaction of degen- 
 eration. Often marked 
 diminution of the electric 
 excitability. Decubitus. 
 
 The same as above. Im- 
 potence; sometimes pria- 
 pism. 
 
 The same as above. 
 
 Reaction of degenera- 
 tion in the muscles of the 
 upper extremities other- 
 wise as above. Often rise 
 in temperature. 
 
 Diagnosis. — After the first effect of the shock is over, differentiation 
 between a total and a partial transverse lesion is, as a rule, possible without 
 much difficulty. In the total cases, permanent, complete, motor and sensory 
 paralysis is present without any symptom of irritation in the paralyzed 
 region, while in the partial cases, incomplete paralysis, only partial sensory 
 disturbance, motor and sensory irritation phenomena and increased vesical 
 and rectal tenesmus are found. 
 
 On the other hand to determine whether a break in conduction depends 
 upon a complete or a partial destruction of the nerve substance, or whether 
 it is merely a matter of the effects of a simple compression, is more difficult 
 and under some circumstances impossible. It is certain that a total inter- 
 ruption of conduction may even be produced by a lesion causing pressure. 
 Likewise, concussion of the spinal cord, through hemorrhages into its 
 substance, may cause, even without greater anatomic changes in it/ severe 
 functional disturbances, which may simulate a contusion. In either case 
 rapid improvement frequently occurs. As a rule, definite differentiation 
 between a crushing of the cord and simple compression is made possible 
 only by long observation. The longer duration of the symptoms of func- 
 tional loss without signs of improvement makes probable the presence of 
 irreparable lesions of the cord. 
 
 Prognosis. — This is serious in all injuries of the spinal column, in which 
 the spinal cord is affected. Total transverse injuries are fatal without 
 exception, if not in direct connection with the traumatism, then later as a 
 result of the unavoidable complications of decubitus and cystitis. Nor 
 is the prognosis in partial lesions of the cord quoad vitam always favorable, 
 since a long time may elapse before there is functional restoration and 
 especially before adjustment of the disturbances with respect to the bladder 
 and bowels, while the patient may meanwhile die in consequence of a decu- 
 
FOCAL DISEASES OF THE SPINAL CORD 373 
 
 bitus or of a cystitis. The prognosis is made still worse by the fact, that 
 hand in hand with the improvement of the at first severe phenomena of 
 paralysis, obstinate irritation symptoms are caused by the sclerotic processes 
 following closely upon the destruction of tissue, which may disturb r con- 
 siderably the ability to use the limbs, which otherwise has been fortunately 
 regained. 
 
 Treatment. — This in the days immediately following an injury will 
 have to be restricted to preventing further displacement of the broken or 
 dislocated vertebras, by careful placing in a recumbent position and the 
 avoidance of all movements. Beside this all measures to prevent a decu- 
 bitus are of the greatest importance. Regarding operative results and 
 especially as to the proper time for operative interference, views differ con- 
 siderably. The majority reject an early operation (before the sixth week), 
 since in the incipient period the phenomena caused by the commotion and 
 intra-medullary hemorrhages can not be distinguished from those due to 
 crushing, contusion or compression. 
 
 If the symptoms of a total transverse lesion persist, operative interference 
 is useless. In cases of incomplete interruption the spontaneous improve- 
 ments are often very extensive. But if no progress is clearly discernible 
 after some weeks, this indicates rather that the spinal cord is injured not only 
 by a transitory lesion due to pressure but that a permanent compression from 
 detached bone splinters, or displaced vertebrae, is present. 
 
 Since in such cases there is a possibility of effecting an improvement 
 or a cure by removing the pressure, an operation is advisable. 
 
 II. The Pressure Paralyses of the Spinal Cord 
 
 As an important cause of either quickly or slowly appearing compression 
 paralyses of the medulla spinalis, are to be considered not only the tumors 
 of the spinal cord and its membranes, and the chronic thickenings of the 
 spinal coverings, which similarly to pachymeningitis cervicalis hypertrophica, 
 act locally like a tumor, but also and more especially the diseases of the 
 individual vertebra. 
 
 We observe suddenly developing pressure paralyses after vertebral 
 fractures and dislocations. Among the changes in the spinal column that 
 effect a slow gradual compression of the spinal cord, vertebral caries plays 
 the main role. In the great majority of cases it is tuberculous; but other 
 forms are known — spondylitis following traumatism, typhoid, pneumonia, 
 and an acute osteomyelitis of the vertebrae. 
 
 More rarely than by tuberculous caries, the spinal cord is injured by 
 neoplasms of the vertebrae of a primary or a metastatic nature or by malignant 
 tumors, which starting from the neighborhood of the spinal column, extend 
 to it and the spinal cord. Syphilis also can cause compression of the cord 
 by exostoses upon the body of the vertebra and its processes. 
 
374 DISEASES OF THE SPINAL CORD 
 
 (a) Caries of the Spinal Column, Spondylitis Tuberculosa 
 
 Tubercular vertebral disease occurs at all periods of life, but especially in 
 childhood and adolescence. It may appear as the only clinical symptom of 
 tuberculosis; but usually, other signs of an arrested tuberculosis, or of one 
 still active are demonstrable. Traumatisms of the spinal column may play 
 a role in the origin of caries, in that localization of tubercular processes in 
 the vertebrae is favored by a fall, blow or contusion in tuberculous individuals. 
 
 Pathological Findings. — The seat of the tubercular granular tissue, 
 which slowly consumes the substance of the bone, is particularly in the 
 body of the vertebra; less frequently the process begins in the vertebral 
 joints, intervertebral discs or arches. If the vertebral bone, destroyed by 
 caries, is gradually compressed by the weight of the body, the consequence 
 is a separation of the spinous processes, a sharp-angled projection or lateral 
 divergence of the spinous process of the diseased vertebra, a so-called Pott's 
 kyphosis. This may appear quite suddenly, if through a direct traumatism, 
 or through severe physical exertion, as in lifting loads, etc., the diseased 
 vertebra suddenly breaks. 
 
 The tuberculous process does not, as a rule, remain limited to the bone; 
 after a time there is an accumulation of cheesy purulent substance between 
 it and the dura and the latter itself undergoes inflammatory changes, a peri- 
 pachymeningitis develops; from the pressure of the dura mater, as a result of 
 the cheesy substance and its inflammatory thickening, the spinal cord and its 
 roots are, as a rule, considerably compressed. A direct transference of the 
 tubercular inflammation to the spinal cord itself through the dura or by 
 the way of the root bundles and blood-vessels, rarely takes place. 
 
 As a rule, a distinct constriction and diminutio-n in size of the spinal cord 
 is to be seen at the point of compression. A microscopic examination may 
 disclose comparatively slight changes in tissue, even though during life serious 
 disturbances in conductivity existed. In such cases groups of swollen axis 
 cylinders and medullated sheaths with incipient phenomena of degeneration 
 may be seen in the midst of well-preserved nerve fibres; the glia tissue also 
 shows infiltration phenomena and seems thickened. The absence of inflam- 
 matory changes proper is emphasized by the majority of authors. If the 
 process has gone farther, wide-meshed spaces have been formed through the 
 degeneration of nerve fibres, which are filled with granular products of 
 degeneration and granular cells. In the later stages after the pressure has 
 been exerted quite a long time, it comes through glia proliferation to the 
 formation of sclerotic focal lesions in place of the destroyed nervous tissue. 
 Secondary ascending and descending degenerations may follow in their wake. 
 
 Phenomena of the Disease 
 To the early symptoms of a developing caries, there belongs not infre- 
 quently a dull back-ache caused by the vertebral disease ; it is usually localized 
 
CARIES OF THE SPINAL COLUMN 375 
 
 to a definite place and is often felt only after motion, through concussions, 
 and by direct pressure. A symptom that results from the pains that appear 
 upon motion, is a certain stiffness of the spinal column, which is especially 
 apparent upon bending, as the patients will sink upon their knees to spare 
 the spinal column. But this local painfulness of certain vertebrae increasing 
 upon pressure, is by no means a regular phenomenon; it is occasionally 
 absent even when a sharp-angled kyphosis, or a lateral divergence of a spinous 
 process can already be demonstrated; they are the most important symptoms 
 of a caries. This change in form of the spinal column is most prominent in 
 the dorsal portion, whereas in a caries of the cervical and lumbar portions, 
 it may be recognized at times only as a flattening of the normal curvature. 
 Since the vertebral deformity as well as the pains may be absent during the 
 entire course of the disease, the possible proof of the burrowing of pus from 
 the diseased vertebrae is of great diagnostic importance. These so-called 
 burrowing abscesses in caries of the upper cervical vertebrae appear occasion- 
 ally in the space between the posterior pharyngeal wall and the spinal 
 column — in caries of the lower cervical and dorsal vertebrae, following the 
 course of the psoas muscle in the inguinal region; a rupture into the thoracic 
 cavity has also occasionally been observed. 
 
 Root and Spinal Cord Symptoms 
 
 These are not infrequently wholly absent, even in the presence of clin- 
 ically pronounced symptoms of caries — pains, stiffness, gibbosity — which is 
 not at all remarkable, since it is not the bending of the spinal column that 
 causes the appearance of compression phenomena, but only the narrowing 
 of the spinal canal, through the thickening of the dura and the accumulation 
 of cheesy tuberculous masses between the dura and the bones, which need 
 not be present in every case. But it happens also that even though the 
 vertebral phenomena are present only in slight degree, already pronounced 
 spinal disturbances are demonstrable. 
 
 As the first sign of compression root-irritation symptoms appear, which 
 are of a neuralgic nature and according to the seat of the injury, may appear 
 as uni- or bilateral occipital, branchial, intercostal, crural or sciatic neuralgia. 
 Not infrequently there exist, at the same time, paresthesias of great variety. 
 Sensory phenomena of functional loss, hypcesthesias and anesthesias appear 
 only later, when the nerve roots are destroyed to a greater extent. Atrophic 
 paralyses of definite muscular regions, through the destruction of the apper- 
 taining anterior roots, also develop, only later as a rule. 
 
 If in the progress of the disease, the cord itself is injured by the increasing 
 pressure, this is shown as a rule by the increased exaggeration of the tendon 
 reflexes lying below the place of the compression. Even before the appear- 
 ance of paralytic phenomena, patellar and ankle clonus, and the Babinski sign 
 
376 DISEASES OF THE SPINAL CORD 
 
 are present. The motor paralysis of the legs, which next appears either 
 quickly or slowly in a caries situated above the lumbar segment, is at first 
 flaccid and later spastic. 
 
 Disturbances in sensation may be absent for a strikingly long time in caries, 
 or be but slight, even though a pronounced motor paraplegia is already 
 present. An attempt has been made to explain this by the fact that the 
 indurated peripachymeningitis compresses especially the pyramidal tracts 
 from the front and the sides, while the posterior columns are spared more, and 
 that just as in lesions of the peripheral nerves, resulting from pressure, the 
 sensory fibres have greater ability to resist the pressure than the motor 
 fibres. But in a more severe injury of the cord from pressure, a decided 
 sensory disturbance, affecting all qualities, will gradually appear in caries 
 also — a disturbance that occasionally is bounded by a rather sharp upper 
 limit, and extends to the region of the roots, which originate from the 
 diseased segments of the spinal, cord. In rare cases, the Brown-Sequard 
 symptom complex has also been observed in caries (Oppenheim). 
 
 Disturbances of the bladder and bowels are occasionally seen early along 
 with exaggeration of the tendon reflexes. They are never absent, when 
 the motor and sensory phenomena of paralysis indicate a more extensive 
 transverse lesion. In such cases, bed-sores also may easily develop. 
 
 The general symptomatic picture of caries (which has just been discussed) 
 will exhibit certain characteristic peculiarities, according to the seat of the 
 carious vertebral change in the upper and lower cervical portion of the 
 vertebral column and in the lower dorsal and upper lumbar vertebrae. These 
 peculiarities we shall have to discuss briefly. 
 
 i. In caries of the upper cervical vertebrae and of the atlanto-occipital 
 joint, beside pains and stiffness in the back of the neck, a neuralgia of the occip- 
 ital nerves on one or both sides is the most important early symptom. In 
 all changes of position of the upper part of the body, the patient supports 
 his head with his hands (Rust's symptom). Oppenheim mentions also the 
 occurrence of paralysis of the accessory nerve and unilateral atrophy of the 
 tongue; upon compression of the medulla oblongata bulbar symptoms are 
 added; where pressure lesions are produced in the upper cervical segments, 
 phrenic phenomena will be noted in some cases. There are, besides, spastic 
 paresis of the arms and legs and paralyses of the musculature of the trunk, 
 as well as sensory disturbances extending to the neck. 
 
 2. If the cervical enlargement is affected in disease of the lower cervical 
 vertebrae, this may be recognized by the fact that beside spastic paresis of 
 the legs and weakness of the musculature of the trunk, a flaccid paralysis of 
 the muscles of the arm and hand develops with muscular atrophy and electrical 
 reaction of degeneration. 
 
 3. A caries of the eleventh and twelfth dorsal vertebrae and of the first 
 lumbar vertebra results in a pressure lesion of the lumbar enlargement. 
 
CARIES OF THE SPINAL COLUMN 377 
 
 The paralysis of the legs is, therefore, from the first, flaccid and atrophic 
 with signs of electric reaction of degeneration; at the same time, the tendon 
 reflexes are markedly diminished or even lost. The sensory disturbance re- 
 mains restricted to the legs; bladder and bowels usually show considerable 
 disturbances. 
 
 Diagnosis. — The diagnosis of a compression paralysis is easily made, 
 if besides the root and cord symptoms, which indicate a progressive transverse 
 lesion, a deformity of the spinal column with local sensitiveness to pressure, 
 may be demonstrated. Then only the further differential diagnostic 
 determination is necessary as to whether a tubercular process is the basis 
 of the vertebral affection, or if a tumor of the spinal column, especially a 
 carcinoma is to be assumed. The exclusion of the usually rare vertebral 
 disease following traumatism, typhoid, acute osteomyelitis, will, as a rule, 
 be easy by taking into consideration the aetiological factors. 
 
 The matter may be far more difficult if, as not rarely occurs, symptoms 
 that point to a disease of the spinal column are absent for a longer time and 
 only the signs of an increasing transverse disease of the cord are present. 
 Under such circumstances positive differentiation between tumors of the 
 spinal cord, the spinal membranes and myelitis having a syphilitic or a 
 tubercular basis on the one hand, and a caries or a vertebral tumor on the 
 other, is absolutely impossible at first and becomes possible only after long 
 observation. 
 
 For the diagnosis in favor of caries, in the first place, the proof of other 
 signs of recent or arrested tuberculosis {fever, enlarged glands, affection of bones) , 
 must be considered. Occasionally also it is possible to establish destruction 
 or displacement of the vertebrae by means of the X-rays. Furthermore the 
 ascertainment of burrowing abscesses may be decisive; value has also been 
 laid upon the greater stiffness of the spinal column in caries in contradistinc- 
 tion to tumors of the spinal cord. Even if it is certain, that larger tumors of 
 the spinal membranes may run their course -without special hindrance to 
 the motility of the spinal column, this holds good actually only for tumors 
 of the dorsal part, while those in the region of the cervical segment occasion- 
 ally cause a considerable stiffness in the neck. 
 
 The differential diagnosis in regard to a meningomyelitis syphilitica 
 must be especially based on the fact that in this disease, the symptoms show 
 quite a striking variety and that, as a rule, the picture of this disease points 
 to multiple, spinal and also cerebral foci. In a doubtful case the success of a 
 course of specific treatment may shed light on the nature of the disease. 
 
 For the distinction between a spondylitis tuberculosa and vertebral tumors, 
 one must consider that the latter usually attack and infiltrate several adjacent 
 vertebrae and therefore not infrequently cause a simple sinking into each 
 other of the vertebrae (changes easily seen on the Roentgen plates), and a 
 diminution in height of the patient; also, as a rule, the developing gibbosity is 
 
378 DISEASES OF THE SPINAL CORD 
 
 less angular than in caries, since several vertebrae participate in the deform- 
 ity. The age may afford a further important point, inasmuch as in the 
 young, the assumption of a vertebral carcinoma has in itself little probability, 
 while, on the other hand, in more advanced age with a more complete loss of 
 strength, a suspicion of vertebral cancer seems justified and makes it advis- 
 able to seek for a primary cancerous focus. 
 
 Prognosis and Course. — The tuberculous bone process, which may 
 extend over years without spinal phenomena necessarily occurring, may at 
 any time come to a standstill and recovery. The same holds true of the 
 spinal paralytic symptoms, which even after persistence for months and years 
 may completely disappear again. The course of the disease, however, is but 
 rarely so favorable. In the majority of cases of caries, with compression of 
 the spinal cord, death results, whether through progress of the tuberculosis 
 in other organs or by the unavoidable results of a decubitus or cystitis in 
 advanced paralysis. Sometimes the phenomena of the spinal cord disappear 
 only in part, as after healing of the bone disease and the inflammatory processes 
 in the membranes the sclerotic changes in the spinal cord have already ad- 
 vanced so far, that a compensation of the functional disturbances is no longer 
 possible. In such cases as permanent signs of the former compression, there 
 are more or less pronounced spastic pareses in the extremities, possibly com- 
 bined with disturbances in sensibility and of bladder function. A difficulty in 
 micturition was, in one of my cases, after the disappearance of all the other 
 spinal symptoms, for years, the only remaining really disturbing phenomenon. 
 The prognosis is the more favorable, the less the phenomena of 
 compression have advanced at the beginning of the treatment and the 
 younger and stronger the patient. It is remarkable that relapses, especially 
 after traumatism, may appear at any time and that even after persistence of a 
 simple gibbosity for decades, spinal symptoms may still appear occasionally 
 afterwards. 
 
 Treatment. — The most important thing in every caries with marked 
 phenomena of compression is a position assuring absolute rest for the diseased 
 spinal column. Even by simply lying on his back in bed for a long period of 
 time, the patient may occasionally bring about a complete cure from severe 
 compression paralysis. In addition the most careful general nutrition and 
 care of the skin is essential for the prevention of bed-sores (smooth mattress, 
 washing the places where pressure occurs several times daily (alcohol 
 and water), air or water cushions). 
 
 Very rapid improvement of the paralytic symptoms is observed after 
 careful extension of the spinal column. This is most easily performed in 
 caries cervicalis, as the pull upon the head is effectually exerted by Glisson's 
 suspensory apparatus with light loads (3 to 4 lbs. at the beginning, and never 
 exceeding 12 to 15 lbs.), the opposite pull being effected by the weight of the 
 body, the bed being elevated at the head. Occasionally, after using exten- 
 
CARIES OF THE SPINAL COLUMN 379 
 
 sion, a deterioration is observed, so that a return to simple rest upon the back 
 without further stretching becomes necessary. 
 
 If the pressure phenomena are but slight from the first, or if the paralysis 
 has disappeared by rest in bed, and the general health and strength of the 
 patient is excellent, ambulatory treatment with a well-fitted plaster -of -Par is 
 corset and the usual supporting apparatus by which the diseased vertebra is 
 relieved from the weight of the parts of the body lying above it may be 
 resorted to (Sayres Jury mast, the head supports of Nebel, Heusner, Schede). 
 Ambulatory treatment has the great advantage that through it the patient 
 may be enabled to live in the open, in the fresh air (seaside resort) and thus 
 through the movement and exercise of his musculature, a better circulation, 
 an increase in appetite, and a better general condition are attained. 
 
 Operative interference has been suggested and attempted in such cases, 
 in which the compression paralysis remains the same, or even increases under 
 the usual extension treatment. It is followed in almost every instance by 
 an immediate favorable result since the phenomena of paralysis improve 
 immediately after the tuberculous focus is removed. As a rule, however, 
 the improvement does not persist, and the resection of the vertebral arches — 
 in the case the tuberculosis is not restricted to these alone — has many more 
 failures than successes to its account. At any rate, Schede considers the 
 operative opening of the spinal canal, as a last resource, not only per- 
 missible, but under circumstances even necessary. 
 
 The course prescribed by Calot for recent and old cases, forcibly to press 
 back under anaesthesia the projecting vertebra and to fix the position so 
 attained by bandages, has been abandoned as being too dangerous. 
 
 Of local measures, rubbing with soft soap, and painting with iodine 
 have been frequently used. Oppenheim saw a favorable influence, several 
 times, from iodide-iron preparations. 
 
 For combating spastic conditions, but also in paralysis without severe 
 muscular spasms, if the patients can be moved, the use of salt and brine 
 baths and an eventual "bath cure" in Nauheim and Kreuznach are to be 
 recommended. 
 
 Electric treatment, at best, should be confined entirely to old cases that 
 have run their course, with flaccid paralysis and atrophic muscles. Direct 
 galvanic treatment applied to the back is productive at times, according to 
 Oppenheim, even in old cases, of favorable effects. 
 
 (b) Carcinoma and Other Tumors of the Vertebral Column 
 
 The not infrequent vertebral cancer is nearly always metastatic, and is 
 found relatively frequently in carcinoma of the breast, but the abdominal 
 organs must also be taken into consideration as the place of origin. The 
 connection between the vertebral spinal cord phenomena and cancer remains 
 quite often unsuspected for some time because the primary cancerous focus 
 
3 8o 
 
 DISEASES OF THE SPINAL CORD 
 
 at first causes few pronounced clinical symptoms, or, as in carcinoma of the 
 breast, under certain conditions, it has been removed by operation some time 
 before the appearance of the disease of the spinal cord. As a consequence, 
 doubts as to its nature may exist for some time. Vertebral cancer, like most 
 
 of the other tumors, which, beginning 
 either in the vertebrse themselves or 
 in their neighborhood, attack the 
 vertebras, effects, as a rule, several 
 vertebral bodies. Owing to the fact 
 that the tumors extend to the verte- 
 bral processes, and also infiltrate the 
 soft parts enveloping the spinal 
 column, there arise occasionally large, 
 easily palpable excrescences on the 
 spinal column. As a rule, however, 
 this is not the case, and as a direct 
 consequence of vertebral destruction, 
 g we find only a sinking of one body into 
 the other (Fig. 82). Besides carci- 
 noma, which is by far the most fre- 
 quent and therefore the most impor- 
 tant tumor of the spinal column, there 
 have been described sarcomata, osteo- 
 sarcomata, the rare enchondromata, 
 the multiple appearing myelomata, 
 also gummata and echinococcus cysts. 
 
 Syphilis also leads to the forma- 
 tion of exostosis on the body of the 
 vertebra and its processes. 
 
 Symptomatology. — The disease 
 
 Fig. 82— Carcinoma of the vertebral column picture shows great similarity to that 
 
 st" S-SSS'vSSta? jtS^K of f ^"ndylitis tuberculosa. In the 
 
 tumor and a narrowing of the vertebral canal typical cases, there are found besides 
 due to this. (After Schlesinger.) . . , ,, , . 
 
 obvious changes in the spinal column 
 with local vertebral pain, which is increased upon motion, root irritation 
 symptoms and phenomena of compression of the cord. Demonstrable changes 
 in the bones, however, may be absent in carcinoma at a time when root and 
 cord symptoms are already pronounced. As an important bone symptom, 
 there appears occasionally a gradual lessening of the length of the body 
 through the sinking together of several vertebras or a gibbosity, which be- 
 cause of the disease in several vertebras, is usually less pointed than in 
 caries. More rarely it comes to the development of large palpable thicken- 
 ngs of the vertebras. 
 
 £r 
 
 
 '■"■ ■ 
 
TUMORS OF THE VERTEBRAL COLUMN 381 
 
 The pains, not only the local pains, but the neuralgic pains produced by 
 root compression as well; are in vertebral tumors, as a rule, extraordinarily 
 violent in contrast to those due to caries. The spontaneous pain is not 
 rarely very severe, though the local tenderness to pressure, which even in 
 extended vertebral carcinoma may be restricted to several vertebral spinous 
 processes, is but slight. The cord symptoms, motor and sensory paralyses, 
 and disturbances in bladder and rectum, develop rather quickly upon occasion, 
 when a carcinoma after breaking through the dura, invades the cord. With 
 advanced changes in the bones, a slight traumatism may suffice to give rise 
 to a sudden collapse of the vertebras and an acute paraplegia. 
 
 Since in the paraplegic limbs, the pains usually persist with great violence 
 Cruveilhier long ago spoke of a paraplegia dolorosa specially in vertebral 
 carcinoma. 
 
 The course of the disease is rapid in the majority of cases; especially in 
 sarcomata and carcinomata the disease rarely lasts longer than nine months 
 to one and one-half years. 
 
 Diagnosis. — Even if a deformity of the spinal column points to a com- 
 pression paralysis, differentiation from a caries will not always be easy. 
 The diagnosis of vertebral cancer will have to be based on the age of the 
 patient, which justifies a suspicion of carcinoma, upon general loss of strength, 
 above all, on the demonstration, of a primary cancerous focus in other organs 
 and on a history of cancer, extirpation of the breast, etc. On the other 
 hand, youth and the proof of recent or healed tuberculosis speak for a 
 diagnosis of caries. It is important for differential diagnosis, that in carcino- 
 matoses also spinal phenomena may be observed, which depend upon a 
 disseminated myelitis. In the absence of definite changes in the bones, 
 and of stronger root irritation symptoms, with the demonstration of a primary 
 tumerous focus, we must reckon with this possibility. An X-ray investiga- 
 tion, which should always be tried as soon as possible, will be of further 
 assistance in these cases also. 
 
 Treatment. — If there be a suspicion of a syphilitic affection of the bones, 
 with the formation of an exostosis leading to compression, specific treatment 
 is to be begun, which in a series of cases (Oppenheim, Leyden, and others) 
 led to complete recovery. Operative interference is only to be advised when 
 the tumor is not a metastatic growth. In the primary tumors of the spinal 
 column, a favorable result can be attained by means of an operation, at least 
 temporarily, as it ameliorates the torturing pains. Recurrence occurs 
 quickly as a rule in consequence of the usually great extension of the 
 tumors over several vertebrae. But according to isolated observations in 
 osteoma and sarcoma (Oppenheim) a cure, which may last for years, seems to 
 lie within the bounds of possibility, so that one must not deprive the patient, 
 if conditions are favorable, of his chance by an operation, though the prog- 
 nosis is usually hopeless. In the majority of cases, we shall have to limit 
 
3 82 
 
 DISEASES OF THE SPINAL CORD 
 
 ourselves to combating the pains (morphine, aspirine, pyramidon) , and by a 
 careful recumbent position and avoidance of all unnecessary movements try to 
 attain a permanent relief for the diseased bones. 
 
 (c) Neoplasms of the Spinal Cord and its Membranes 
 Pathological Anatomy. — Tumors of extra-medullary development are 
 more common than neoplasms arising in the spinal 
 cord itself. Among the extra-medullary tumors 
 those situated outside of the dura mater, if we except 
 those of the vertebrae, are much rarer than the prac- 
 tically much more important intra-dural tumors. In 
 the rare extra-dural neoplasms, it is usually a matter of 
 lipomata and echinococci; among the tumors develop- 
 ing within the dura, fibromata, sarcomata and their 
 mixed forms play the most practically important part, 
 since they usually occur in circumscribed form about 
 the size of peas to hazel-nuts and are easily removed 
 by operation; besides these are found intra-dural 
 myxomata, psammomata, less frequently lipomata, 
 adenosarcomata, lymphangiomata, and teratomata. 
 Sarcoma occasionally appears in multiple form, or it 
 spreads diffusely over the membranes of the spinal 
 cord, the individual growths varying much in size. 
 
 The greater number of the growths in the spinal 
 cord itself are gliomata; they are preferably situated 
 in the cervical and upper dorsal cord and in the upper 
 part of the lumbar enlargement; other neoplasms oc- 
 curring are sarcomata, tubercles and gummata. Mul- 
 tiple neurofibromata emanating from the roots of the 
 spinal cord are frequently only a partial symptom of 
 an extensive general neurofibromatosis of the nervous 
 system. 
 
 Causes. — Excluding the parasitic and infectious 
 growths we know nothing of the aetiology of the neo- 
 plasms and must be satisfied with the assumption of 
 an abnormal congenital predisposition of the tissues. 
 It seems that traumatisms are to be considered as excit- 
 
 FiG. 83. — Intra-dural 
 
 tumor, situated opposite ing factors in the development of the growths or that 
 
 the dorsal cord between ,-, , r . f . .•■ . . 1 
 
 the 6th and 7th pair of tnev are at least factors favoring their quicker 
 
 dorsal roots, compressing 
 the spinal cord posteriorly 
 from the right side. 
 Drawing is natural size 
 after cutting open and 
 spreading the dura. 
 
 ( Author 's own observation) . emanating from the membranes are not malignant 
 
 progress. 
 
 Tumors in the Membranes of the Spinal Cord 
 Symptomatology. — Since the majority of tumors 
 
TUMORS OF THE SPINAL MEMBRANES 
 
 383 
 
 and grow but very slowly, the appearance of symptoms of compression 
 in the substance of the spinal cord is preceded by an earlier stage of 
 neuralgic pains, which may extend over months and even years and 
 which are caused by the irritation due to pressure upon the posterior 
 roots. These radiating pains are localized to one or both sides of a 
 definite nerve region and pre- 
 sent the picture of an inter- 
 costal or of a sciatic neuralgia, 
 or a neuralgia of the brachial 
 plexus. Patients frequently 
 complain of an increase in 
 the pain whenever the spinal 
 column is jarred, as in cough- 
 ing, sneezing, or by pressing 
 upon it. The prodromal 
 stage of pains need not always 
 be present in tumors of the 
 spinal cord membranes; oc- 
 casionally the pain is present 
 to only a slight degree, or lasts 
 only a short time. Irritation 
 symptoms in the anterior 
 motor roots, tremors, mus- 
 cular contractions, spasms oc- 
 cur but rarely compared to the 
 sensory irritation phenom- 
 ena, even where a gradually 
 growing tumor, as shown by 
 its location, must have pressed 
 almost equally on posterior 
 and anterior roots. As a rule, 
 in a compression of the an- 
 terior roots, the irritation FlG - 8 4— Gower's scheme (from the 7th cervical to the 
 . 2nd lumbar segment) shows the relation of the spinous 
 
 phenomena are quickly fol- processes and of the places of exit of the roots to the 
 
 lowed by atrophic paralysis in £*■* r^tSii" 1 t£ *h S^S 
 
 the corresponding muscular is regarded as diseased. 
 
 area. 
 
 In the usual location of a tumor in the dorsal portion of the cord, atrophic 
 muscular paralyses caused by pressure lesion of the motor roots fall entirely 
 into the background of the clinical picture, since weakness and wasting of 
 individual intercostal muscles escape detection. Only in cases in which 
 the lower dorsal roots (8-12 pair) are injured by the tumor, can iini- or 
 bilateral paralyses of the abdominal muscles appear and with the sensory 
 
384 DISEASES OF THE SPINAL CORD 
 
 phenomena of irritation, form an early local symptom of considerable diag- 
 nostic importance. 
 
 A less frequent symptom of irritation, which, however, must be sought 
 for, is a local tender point of the spinal column, usually at the height of those 
 vertebrae, opposite to which the tumor is located. This vertebral tenderness 
 is best sought for and tested in this manner: exert strong pressure with the 
 back of the flexed index or middle finger to one side of the spinous processes 
 of the vertebrae. This will be found painful especially on the side which 
 was the seat of the neuralgia at the onset. 
 
 As the tumor continues to grow, the signs of a compression of the cord 
 are added to the symptoms of root irritation, which persist in most of the 
 cases; a motor and sensory paralysis which, as a rule, advances slowly and 
 regularly in the part of the body lying below the place of pressure. The 
 motor paralysis is always spastic when the tumor is located, as is usually 
 the case, above the lumbar segment; in the beginning it usually appears 
 as slight paresis in one leg, and later extends to the other side, and even 
 when paraparesis is already present, the side of the body corresponding to 
 the seat of the tumor usually shows the more severe paralytic phenomena. 
 
 The tendon reflexes, the increase of which occasionally is the first symptom 
 of compression of the cord, are regularly and considerably exaggerated; 
 patellar and ankle clonus are present and frequently the Babinski sign, 
 especially in the more markedly paralyzed limb. The reflexes of the abdomi- 
 nal muscles are usually lost or weakened. 
 
 The disturbance in sensation may be limited at the beginning to one leg, 
 because the cord at first is compressed on one side, so that with a simul- 
 taneous paresis of the leg corresponding to the side of the tumor, there 
 comes to exist for some time the symptom complex of the Brown-Sequard 
 unilateral paralysis. Thus, for instance, Oppenheim saw a thermancesthesia 
 in the opposite leg precede the development of the homolateral paralysis 
 in two of his cases. As the transverse lesion increases in size, due to the 
 growing tumor, the unilateral sensory paralysis is soon transformed into a 
 bilateral disturbance; it may not come under observation until this stage 
 is reached. 
 
 In the typical cases, the upper boundary of the sensory disturbances, 
 which is usually a horizontal line on the trunk, may be established quite 
 accurately upon repeated tests. At this upper margin, sensibility is not 
 completely lost but only diminished. This upper hypasthetic cutaneous 
 area gradually passes, as we go downward, over into the region of the skin 
 that is more or less decidedly anasihetic. In some cases, it has even been 
 possible to demonstrate an abnormal sensitiveness in the skin area immedi- 
 ately above the hypaesthetic zone — a hyperesthesia. Especially characteristic 
 and important for the diagnosis of tumor is the determination that the 
 upper boundary of disturbed sensibility remains in the same place even 
 
INTRA-MEDULLARY TUMORS 385 
 
 after a longer period of observation, or, at any rate, undergoes only an 
 unimportant move upwards. This depends on the fact, that with a slowly 
 growing tumor, even a slight increase in its thickness must cause a marked 
 compression in the narrow spinal canal, whereas a slight increase in length 
 can cause no perceptible extension of the sensory disturbances upwards 
 by lesions of the roots and the cord. 
 
 Disturbances of bladder and bowels are always present as soon as the 
 phenomena of cord compression have developed to a somewhat considerable 
 degree; but even in the beginning of the disease bladder disturbances in the 
 form of increased urinary pressure or of slight difficulty in micturition may 
 make themselves felt. 
 
 If the tumor is not located, as we have assumed up to this point in the 
 discussion, in its favorite place in the dorsal portion of the spinal cord, but in 
 the region of the cervical or lumbar enlargement, or still farther down in the 
 conus terminalis or the cauda equina, the symptomatic picture, according to 
 the location in one of these regions will present certain peculiarities, impor- 
 tant for localization. In compression of the cervical enlargement, we shall 
 see, in addition to spastic paresis of the lower extremities after inceptive 
 neuralgic troubles in the course of the nerves of the arm, flaccid degenerative 
 paralyses of the arm-muscles develop caused partly by the destruction of the 
 anterior roots, but partly also by injury, through pressure, of the anterior horn 
 ganglion cells of the cervical enlargement. Through irritation or paralysis 
 of the sympathetic fibres, lying in the lower cervical and upper dorsal portion 
 of the cord, there may appear also so-called oculopupillary symptoms, dilata- 
 tion or contraction of the pupil and palpebral fissure on one or both sides. 
 Tumors of the lumbar region, because of the fact that the nerve roots are 
 placed close to one another, generally cause very violent and extensive 
 neuralgic pains; also the paralysis that appears later in the legs is from the 
 first flaccid and atrophic and is associated with loss of the tendon reflexes. 
 
 The symptomatic pictures evoked by compression of the conus and the 
 cauda will be discussed in a special chapter. 
 
 Intra-medullary Tumors 
 
 The intra-medullary tumors in their symptomatology and course fre- 
 quently differ essentially from the typical picture of the tumors of the 
 spinal cord membranes discussed above. In the first place, a pure neuralgic 
 stage lasting for any length of time is, as a rule, absent in tumors of the spinal 
 substance. Even if with the development of a new growth in the periphery 
 of the cord, in the neighborhood of the membranes and the posterior root 
 zone, pains form the first symptom, nevertheless, soon afterwards, or even 
 synchronously, appear the signs of a transverse lesion, during which a 
 transitory Brown-Sequard type of paralysis may be observed. Likewise 
 25 
 
3 86 
 
 DISEASES OF THE SPINAL CORD 
 
 the pains excited occasionally during the course of the disease, by tumors 
 of the cord, whether through irritation of tracts within the spinal cord 
 or an accompanying chronic meningitis, are usually only of slight degree, 
 and fall quite into the background of the disease picture. 
 
 If a tumor develops from the first in the central portion of the cord, dis- 
 ease pictures may develop, which do not at all resemble those of tumor of the 
 spinal cord membranes. In more diffuse extension over a transverse section 
 of the spinal cord, there appears for a long period of time, the picture of 
 a subacute or chronic myelitis transversa, without any demonstrable cause; 
 in gliomata with simultaneous cavity formation, the phenomena of a syringo- 
 myelia present themselves; it is precisely in cord tumors that dissociated 
 sensory paralysis is found most frequently; destruction of the anterior horns, 
 
 through tubercles or a sarcoma with 
 rapid extension in the longitudinal axis 
 produces the picture of a rapidly pro- 
 gressing spinal muscular atrophy. 
 
 For the course of tumors of the cord, 
 it is, moreover, characteristic to a cer- 
 tain degree, that a comparatively rapid 
 growth in the longitudinal direction up- 
 wards and downwards, takes place, 
 and thereby a swifter moving of the 
 boundary of the sensory paralysis up- 
 wards is observed than in the tumors of the membranes. 
 
 General Diagnosis. — Differentiation of a tumor of the spinal membranes 
 from other diseases of the spinal cord not dependent upon compression, 
 which run their course under the picture of a diffuse transverse disease, is 
 not as a rule difficult. Confusion with a pure myelitis transversa is prevented 
 by the usually acute onset of this disease without preceding neuralgic dis- 
 turbance, as well as by the simultaneous demonstration of causes and 
 assisting factors frequently to be considered in acute myelitis, as preceding 
 infectious diseases, anginas, colds, over-exertions, possibly also the existence 
 of fever. For the differential diagnosis from multiple sclerosis, which occa- 
 sionally, for some time presents the symptomatic picture of a chronic trans- 
 verse myelitis, the chief weight must be laid upon : i . The permanent absence 
 of pains, not only in the beginning of the disease but throughout its course; 
 2. upon the absence of a sensory disturbance noted evenly and constantly 
 at a certain level, and 3. upon the addition later of other symptoms charac- 
 teristic of sclerosis, like nystagmus, intention tremor, changes in the background 
 of the eye. At any rate, as some experiences have shown, a relatively long 
 period of observation may occasionally be necessary, to exclude a sclerosis 
 with certainty. 
 
 Differentiation from a meningo-myelitis luetica may not be easy at the 
 
 Fig. 85. — Intra-medullary spindle-cell sar- 
 coma. {After Gowers.) 
 
DIAGNOSIS OF TUMORS OF THE SPINAL CORD 387 
 
 beginning, if relatively violent pains are caused by root irritation. But 
 lues spinalis is characterized by the fact, that no regular, absolutely steady 
 advance of all symptoms can be observed as in the case of tumor, but that 
 in this disease there is frequently considerable alteration in the violence and 
 extent of the motor and sensory phenomena of paralysis; furthermore, the 
 syphilis is not as a rule confined to the spinal cord, so that the diagnosis is 
 made easier by simultaneous cerebral symptoms. In a doubtful case, the 
 employment of specific treatment will be necessary as a further means of 
 assistance. 
 
 We face a far more difficult problem, if it be a question of the practically 
 so important differentiation, whether we are dealing with a tumor of the 
 spinal membranes or with a compression paralysis from another cause; 
 through intra-medullary or vertebral tumors, spondylitis tuberculosa, pachy- 
 meningitis cervicalis hypertrophica or a circumscribed meningitis serosa 
 spinalis {Oppenheim and Krause). 
 
 1. The determination of a caries and a vertebral tumor is very frequently 
 made very difficult, or even quite impossible, by the fact that in the beginning 
 of the disease, even with the most minute examination of the spinal column, 
 no deformity or localized tenderness can be demonstrated and that examina- 
 tion with the X-rays has had a negative result. A greater degree of stiffness 
 of the spinal column and painfulness upon motion of the trunk will certainly, 
 even without externally noticeable change in form, count in favor of a devel- 
 oping vertebral affection, since even large tumors of the membranes, espe- 
 cially in their favorite location in the dorsal segment, exist, as a rule, without 
 considerable disturbances in movement. Besides this in doubtful cases, 
 the detection of signs of recent or arrested tuberculosis, of fever, or gravitation 
 abscesses and where a carcinoma is suspected, the discovery of a malignant 
 tumor in other organs, or the existence of a marked amount of cachexia, are 
 important points for diagnostic purposes. 
 
 2. Concerning differentiation from a pachymeningitis cervicalis, it is 
 to be remembered that this disease is located preferably in the cervical seg- 
 ment, while the majority of tumors are to be found in and opposite the dorsal 
 segment. Furthermore, the disease progresses far less rapidly than a tumor 
 of the medulla spinalis and develops preferably in individuals who formerly 
 had syphilis; in most instances it exists for years and decades until more 
 serious disturbances in cord conduction are developed. 
 
 3. The question, whether an intra-medullary tumor is present, or one 
 emanating from the meninges can, in many cases, not be answered with 
 certainty. A pronounced neuralgic prodromal stage presupposes in the first 
 instance an extra-medullary location. For even if the spinal cord tumors 
 may be accompanied by pains, due to concomitant chronic meningitis and 
 to irritation of intra-spinal tracts sensitive to pain, nevertheless it is usually 
 a matter of only transitory and slightly pronounced sensory symptoms of 
 
388 DISEASES OF THE SPINAL CORD 
 
 irritation. But unfortunately, in recent times, observations of meningeal 
 tumors with painless course have become more frequent, so that the absence 
 of a neuralgic stage by no means excludes an extra-medullary seat. The 
 experience mentioned above, is important, viz., that the neoplasms originating 
 in the cord, especially if a glioma is connected with formation of cavities, 
 often evoke in the beginning of the disease partial sensory paralysis. A 
 further diagnostically important sign of intra-medullary tumor consists, as 
 has already been mentioned, in the fact that they not infrequently display a 
 rapid growth upwards and downwards and thereby simulate the picture of a 
 subacute ascending myelitis, whereas for the meningeal tumors, it is the 
 slight displacement of the upper boundary of the sensory disturbance, in 
 spite of increase of the symptoms of paralysis, that is characteristic. 
 
 4. The syringomyelias may be distinguished from the meningeal tumors 
 mostly by the fact, that a real neuralgic stage is always absent in them, even 
 though slight pains and paraesthesias form no unusual concomitant phenom- 
 enon; that furthermore, the sensory disturbance bears a typically dissociated 
 character and is confined to a cutaneous region, supplied by a definite segment 
 of the spinal cord, while in a pressure paralysis of the cord, by interruption 
 in, the conductivity of the sensory tracts, sensory paralyses of one or both sides 
 appear below the point of compression. To this must be added the further 
 fact that syringomyelia progresses more quickly upwards, possibly even into 
 the medulla oblongata, and only after a much longer period and in long- 
 continuing stationary intervals gives rise to such severe paralyses of the 
 lower extremities as are usually developed in unbroken succession in 
 meningeal tumors as soon as the first signs of a compression of the cord 
 have appeared. 
 
 5. For the differential diagnosis between extra-medullary tumors and 
 that form of a localized serous meningitis, which has been described by 
 Oppenheim and Krause and which can imitate in a very deceptive manner the 
 disease picture of a tumor, we lack at present any secure foundation. 
 
 Determination of the Location of the Tumor. — The exact determina- 
 tion of the height at which the tumor is located in the spinal cord is of the 
 greatest practical importance, since upon it, in the first place, depends the 
 success of operative interference. 
 
 . As an important indication for determining the location, an occasionally 
 demonstrable locally circumscribed vertebral tenderness may be serviceable. 
 This will be especially important, if, at the same time, neuralgias appear in 
 a root area, the appertaining spinal-cord segment of which, according to the 
 knowledge we now possess, lies opposite to the vertebra that is sensitive to 
 pressure. Tenderness to pressure, symptoms of root irritation and of functional 
 loss, however, must be in harmony with one another, since the sensitiveness 
 of the spinal column as a result of chronic meningitis or for other unknown 
 reasons, is frequently far more extended than would correspond to the size 
 
LOCALIZATION OF TUMORS OF THE SPINAL CORD 389 
 
 of the tumor and an isolated area of pain on pressure can occasionally 
 develop on a purely nervous foundation. 
 
 In the majority of cases, local tenderness to pressure is not demonstrable, 
 and even pains may be entirely wanting, so that we are limited to the sensory 
 and motor symptoms of loss of function. 
 
 The possibility of establishing the location of a tumor in a certain segment 
 of the spinal cord, on the basis of sensory phenomena of functional loss, 
 rests upon our knowledge of the relations of cutaneous sensibility to the indi- 
 vidual segments of the spinal cord. Each of these, namely, receives the majority 
 of the sensory fibres of a definite rather sharply defined cutaneous region. 
 If, therefore, a single segment is injured by the presence of a tumor, a dis- 
 turbance in sensation makes itself felt, as a rule, in the corresponding region 
 of the skin. As may be seen from the scheme of Edinger presented herewith 
 (Fig. 86) the cutaneous regions belonging to the dorsal segments of the 
 spinal cord form stripes that course horizontally upon the skin of the trunk, so 
 that after the destruction of a dorsal segment a horizontal boundary of 
 sensory disturbances appears upon the trunk. 
 
 Clinical observation has taught us, furthermore, that after injury of a 
 single segment, no complete loss of sensation, but only a lowering, a hypces- 
 thesia, appears in the appertaining stripe upon the skin. This rests on the 
 fact that every individual cutaneous region sends its nerve fibres to two or 
 three segments placed one above the other, so that several of them must be injured 
 by a tumor, if a total anasthesia is to be found in a definite cutaneous area. 
 This condition, that the lesion of a single segment of the spinal cord causes in 
 the appertaining cutaneous area only a lowering, but not a total loss of sensi- 
 bility, is of special importance for the establishment of the upper limit of a 
 tumor. 
 
 If a tumor injures the spinal cord at a certain level by pressure, there 
 occurs in the place of strongest compression of the cord an interruption in the 
 conduction of the ascending sensory fibres of the spinal cord. As a result of this, 
 there is a general sensory paralysis in that entire part of the body which lies 
 below the point of compression. The tumor, however, causes also local seg- 
 mentary disturbances, since its upper end, which generally extends above the 
 region of the most severe transverse lesion, affects also the spinal cord seg- 
 ments lying above this place, and gives rise to disturbances in sensibility in 
 their appertaining cutaneous areas. 
 
 The scheme of Fig. 86 serves best for the explanation of these relations. 
 By the gross lines is meant that in the lower extremities and in the trunk up 
 to about a hand's breadth of the sternum, the ability of perceiving sensations 
 is completely lost, and that above this region with total anaesthesia there is a 
 cutaneous stripe (oblique lines, uncrossed), which shows only a diminution of 
 sensation. 
 
 This hypasthetic cutaneous area, which therefore forms the upper limit of 
 
39° 
 
 DISEASES OF THE SPINAL CORD 
 
 Fig. 86. — Disturbance of sensibility in myelitis due to compression (schematic). The dis- 
 turbance in sensibility reaches in the trunk the cutaneous region of the 7th dorsal segment. 
 The hypothetic cutaneous area is shown by simple cross lines. The double cross lines show 
 the anaesthetic area. (After Edinger.) - 
 
LOCALIZATION' OF TUMORS OF THE SPINAL CORD 391 
 
 the sensor} T disturbance, according to Edinger'' s scheme belongs to the 
 seventh dorsal segment. 
 
 According to the above explanation, we must interpret the origin of the 
 sensory paralysis thus schematically presented in this way, that through the 
 pressure of a tumor an interruption in conduction of the sensory spinal-cord 
 tracts lying below the seventh dorsal segment resulted, which caused a total 
 anaesthesia of that part of the body lying below the place of compression. 
 But we must assume in addition, that the upper end of the tumor extends 
 to the seventh dorsal segment, since the cutaneous area belonging to it still 
 exhibits a diminution in sensibility. 
 
 If we review the course of the investigation in determining the location of 
 a neoplasm, it is in the first place a matter of the most accurate determination 
 that is possible of the upper boundary, which shows barely demonstrable sensory 
 disturbances. With the aid of the well-known sensory area schemes of 
 Edinger, Seiffer, and others, we get our bearings to which spinal-cord segment 
 the cutaneous area belongs, which according to the result of the investigation 
 forms the upper limit of the sensory disturbance. If, e. g., the upper limit, as 
 drawn in the figure, reaches to the height of the seventh rib upon the trunk, 
 according to Edinger'' s scheme, the hypothetic cutaneous stripe corresponds 
 to the seventh dorsal segment; we may therefore expect the upper end of the 
 tumor to be operated on to be opposite Dy. As may also be seen from 
 Gower's scheme (Fig. 84 on p. 383), Dj (oblique lines) lies opposite to the 
 sixth dorsal vertebra and fifth dorsal spinal process. In order to expose the 
 tumor, therefore, the operator will make his incision at the level of the fifth 
 dorsal spinous process and remove the sixth and the seventh, possibly also 
 the fifth vertebral arch, since experience has shown that the seat of the tumor 
 is generally assumed too low rather than too high. In otherwise correct 
 diagnoses of the tumors in several cases the neoplasm was not found because 
 of a laminectomy that was performed too low. The longitudinal extent 
 downwards cannot, as a rule, be determined, especially when the location of 
 the tumor is in the dorsal segment, since this causes in addition to the local 
 phenomena of functional loss and irritation at the upper boundary, general 
 disturbances in conduction below the point of compression due to the pressure 
 on the entire transverse section of the spinal cord, which prevent us from 
 recognizing the segmentary arrangement of the sensor}* disturbances. 
 
 For the determination of the location of the neoplasms in the cervical and 
 lumbar segments, we must consider not only the sensor}*, but also the motor 
 phenomena of functional loss and the disturbances of the reflexes. The dis- 
 tribution of the muscular innervation among the various segments of the 
 spinal cord shows the same regularity as the sensory supply of the skin, in as 
 much as every muscle is subserved by several neighboring segments. Our 
 knowledge, which is as yet incomplete of the segments belonging to the 
 individual muscles is summarized, as it were, in Edinger s table. The 
 
392 DISEASES OF THE SPINAL CORD 
 
 localization diagnosis at the levels of the lumbar enlargement and the conus 
 terminalis is particularly difficult, because the roots of this region after their 
 exit from the spinal cord, descend for some distance before they leave the 
 spinal canal. As Schultze proved, in a corresponding localization of the 
 tumor in the conus terminalis, the same symptom complex may arise as in a 
 compression in the region of the last lumbar vertebra, or of the sacral vertebrce. 
 These conditions will be discussed in more detail in a separate chapter on 
 diseases of the conus. 
 
 Treatment. — As soon as a suspicion of spinal coral syphilis is aroused, 
 or of tubercular caries, which in so many cases cannot be excluded, our first 
 task is to order antisyphilitic treatment, or to try treatment by extension. 
 In tumor, after extension treatment of the spinal column, the patient grows 
 worse with unusual frequency; but this has occasionally been observed in 
 caries also, so that no decisive importance can be attributed to this phenom- 
 enon. If the treatment fails and the disease progresses, we face the question 
 of operative interference, which because of the unusually favorable results 
 that have heretofore been attained in meningo-spinal tumors, must always 
 be taken into consideration. 
 
 In every case, in which the typical symptomatic picture of a meningo- 
 spinal tumor is present, and a determination of the locality seems possible, 
 surgical interference is earnestly urged for even if a mistake in diagnosis 
 is occasionally unavoidable, since the typical disease picture of an operable 
 meningo-spinal tumor is simulated by vertebral disease or by a tumor of the 
 cord, nevertheless, the chances of complete cure by removal of the usually 
 benign meningo-spinal tumor, are quite favorable. 
 
 We are also absolutely justified in suggesting to the patient an explorative 
 laminectomy, even though the atypical disease picture indicates a progressive 
 compression of the spinal cord, and we are unable even after long observation 
 to make a certain diagnosis between intra- and extra-medullary tumors. 
 As long as in the progressive course of the disease, a meningo-spinal tumor 
 cannot be excluded, an exploratory opening of the spinal canal is advisable, 
 since without an operation the patient is steadily progressing to certain 
 death. An operation, if the symptoms point to multiple tumors, is futile. 
 
 Prognosis. — The chances of recovery are naturally most favorable, 
 if the operation is performed as soon as possible, before Jhe development of 
 the most severe disturbances in conduction. As a rule, a diagnosis of a 
 tumor and the more exact determination of its location, will be possible 
 only after the development of pronounced phenomena of compression on 
 the part of the cord. But even considerable disturbances in conduction, 
 complete paraplegias and bladder disturbances may absolutely disappear 
 in the course of weeks or months, if the pressure lesion has not lasted too 
 long. 
 
 The most favorable situation of tumors for an operation, is the dorsal 
 
PACHYMENINGITIS CERVICALIS HYPERTROPHICA 393 
 
 portion of the spinal cord, less favorable trie cervical and lumbar portions, 
 as well as the cauda equina region. 
 
 (d) Pachymeningitis Cervicalis Hypertrophica (Charcot) 
 
 That form of chronic inflammation of the membranes of the spinal cord, 
 described by Charcot under this name is a very rare disease. Anatomically 
 the most striking finding is a very considerable thickening of the dura mater, 
 which may possibly attain from five to ten times its normal size. The soft 
 membranes likewise undergo inflammatory changes, becoming adherent to 
 each other and to the spinal cord. The microscopic picture shows not only 
 dense, newly formed bands of connective tissue, but also regularly a consider- 
 able nuclear infiltration and a thickening of the vessels of the spinal cord as 
 well as of its membranes. The inflammatory process does not, in all prob- 
 ability, begin always in the dura, but originates also in the soft membranes; 
 it is usually most marked in the cervical portion; frequently, however, it 
 extends upwards to the meninges of the brain and downwards to the lumbar 
 segment of the spinal cord. 
 
 By mechanical compression and extension of the inflammatory process, 
 the roots and the cord itself, the latter at first especially in its marginal por- 
 tions, become injured. But also more extended degenerations of the nerve 
 fibres with chronic inflammatory changes of the cord and secondary ascend- 
 ing and descending degenerations may develop. 
 
 Of its cetiology we know only with certainty, that the disease is very 
 frequently preceded by a syphilitic infection, while the importance of the 
 other aetiological factors held responsible, like alcoholism, colds, over-exer- 
 tion, is quite uncertain. 
 
 Symptomatology. — The first phenomena of the disease, which may 
 constitute the whole clinical picture for several months, consist of pains 
 beginning in the back of the neck and radiating thence to the back of the head, 
 the shoulders and arms. These spontaneously appearing pains, increasing 
 upon pressure and motion, are referred preferably to the region of the ulnar 
 and the median nerves. To these phenomena of irritation, based on com- 
 pression of the posterior roots, there are added later objective sensory dis- 
 turbances, likewise paralysis and atrophy in the muscles of the arm and hand, 
 due to degeneration in the anterior motor roots. Through the fact, that the 
 radial region is frequently spared for some time, a peculiar position of the 
 hand and fingers develops, due to the overbalance of the extensors of the 
 hand and fingers: dorsal flexion of the entire hand and extension of the 
 basal phalanges, while the middle and terminal phalanges are flexed 
 (preacher's hand). During the advance of the process, there are added, 
 sooner or later, to the root symptoms, signs of a compression of the cord: 
 slowly increasing paresis of the legs with considerable exaggeration of the 
 
394 DISEASES OF THE SPINAL CORD 
 
 tendon reflexes, mostly slight sensory disturbances in the lower extremities, 
 disturbances in functions in the bladder and rectum. 
 
 Course and Prognosis. — The disease, as a rule, extends over many 
 (15 to 20) years. Death may result from decubitus or cystitis. Improve- 
 ment and standstill are observed in every stage. 
 
 Diagnosis. — Differentiation of the disease, is as a rule impossible at 
 the beginning, since the compression paralyses due to meningo-spinal tumors 
 and tumors of the spinal column as well as to tubercular caries, may, at first, 
 evoke similar symptoms of root irritation, without the possibility of always 
 demonstrating the early vertebral changes. Only the course that drags on 
 for years and the simultaneous absence of deformities in the spinal column 
 will point the way to the diagnosis. 
 
 Treatment. — Antisyphilitic treatment is of chief importance: iodide of 
 potassium for some time (10 gr. three times a day) and mercurial treatment. 
 As a derivative in more violent pains, the painting of the region of the neck 
 with iodine and ferrum candens may be recommended in particular, in 
 addition to medication with anti-neuralgics. 
 
 III. The Syphilitic Diseases of the Spinal Cord 
 
 Syphilis as an aetiological factor plays an extraordinarily important role 
 in the diseases of the spinal cord. We leave out of consideration, here, 
 such diseases of the spinal cord as in individuals formerly infected with 
 syphilis usually develop slowly, in the form of primary degenerations of definite 
 spinal-cord tracts and systems of fibres and are denominated metasyphilitic 
 diseases (tabes dorsalis). Here we are concerned only with those disease 
 pictures, in which the pathologic anatomic changes bear a specifically 
 syphilitic character, or in which, owing to a series of concomitant conditions, 
 the assumption, that the diffuse changes in the spinal cord, which are at the 
 basis of the disease, rest upon syphilis, is made highly probable, in spite of 
 the fact that the anatomic signs that characterize the disease are not, in 
 every case, demonstrable. The most frequent and important form of spinal- 
 cord syphilis is 
 
 Meningomyelitis Chronica Syphilitica 
 
 Anatomical Findings. — The process begins in the soft membranes of 
 the spinal cord, less frequently does it arise from the inner surface of the dura; 
 the membranes are thickened over a large area and permeated in a diffuse 
 manner with a dirty and jelly-like gummatous granulation-tissue. Circum- 
 scribed gummatous tumors are rare. Beginning from the gummatous new 
 growth, which surrounds the spinal cord like a rind, prong-like cellular 
 proliferations are seen advancing into the marginal portions and extend even 
 
CHRONIC SYPHILITIC MENINGOMYELITIS 
 
 395 
 
 deeper into the gray matter of the cord (Fig. 88) ; the roots of the spinal cord 
 likewise usually exhibit signs of a considerable infiltration with round cells. 
 Through the granulation tissue, which enters the substance of the cord like 
 a wedge and through the frequent and usually simultaneously appearing 
 end-arteritic changes of the vessels of the spinal 
 cord, the disease is spread to nerve tissue. Due to 
 the pressure of the disease foci advancing from the 
 periphery in the form of prongs an atrophy of the 
 nerve fibres results. To this is added as further dis- 
 turbance the deficient blood supply of the substance 
 of the spinal cord through the diseased vessels, the 
 thickening of whose walls may bring about a com- 
 plete closure of the lumen of the vessel ; in addition 
 to atrophy of nerve fibres, there are found ischemic softenings of the cord 
 substance, small hemorrhages and occasionally even cavity formations. The 
 gummatous meningitis in most cases is most strongly developed at the pos- 
 terior part of the spinal cord. If the process, as has been observed in some 
 cases, remains limited to a marked degree to the roots of the spinal cord, so 
 that many of them appear thickened like knots and swollen, the condition 
 
 Fig. 87. — Multiple root 
 neuritis in syphilis. (After 
 Butter sack.) 
 
 Fig. 88. — Meningomyelitis syphilitica. Penetration of the cord by the new growth. 
 «, New growths; w, roots of the spinal cord imbedded in the gummatous layers. (After 
 Boettiger.) 
 
 is spoken of as a multiple radicular neuritis (Fig. 87). All the changes here 
 described may be found also in congenital syphilis of the spinal cord. 
 
 Symptomatology.— It is easily understood, that with the variety of 
 anatomical changes caused by syphilis, the disease picture may be a very 
 variable one, differing according to the localization and the predominance 
 of lesions of the nerve tissue as caused either by arteritic processes, by 
 gummatous meningitis or by radicular neuritis. But Oppenheim has demon- 
 strated that a symptom complex may be mapped out for the syphilitic form 
 of meningomyelitis which, because of the simultaneous appearance of men- 
 
396 DISEASES OF THE SPINAL CORD 
 
 ingeal, radicular and cord symptoms as well as through certain peculiarities 
 of the course, bears an imprint so characteristic, that the diagnosis in many 
 cases may be made quite easily. 
 
 Besides local pains in the back and in the sacral region there appear 
 radiating pains due to root-irritation, which, according to the location of the 
 disease, are described as "girdle-feeling" or as neuralgias in the extremities. 
 Since first and predominantly the posterior half of the spinal cord is affected, 
 motor-root symptoms appear, as a rule, if at all, only later and then in the 
 form of partial atrophic paralyses in the arms and legs. To these meningeal 
 and radicular irritation phenomena are added the important symptoms of a 
 lesion of the cord: unilateral or bilateral spastic paresis of the legs caused by 
 the affection of the dorsal portion of the cord. If the cervical portion is 
 affected in its enlargement, the paralysis of the arms is flaccid and atrophic, 
 and is associated with spastic phenomena in the legs, while, if the lesion is 
 located in the lumbar portion, the paralysis — restricted to the lower extrem- 
 ities — is flaccid, from the first. The development of the disturbances in 
 motility may develop gradually or quite suddenly; likewise, at any time, a 
 slight paraparesis may quickly change into a total paraplegia. Objective 
 disturbances in sensation and in the functions of the bladder and rectum are 
 found regularly. Quite frequently the picture of a Brown-Sequard paralysis 
 appears temporarily as an effect of a more unilateral compression. If, at 
 one of the stages of the development of the disease, the posterior roots are 
 more extensively and more markedly affected by the gummatous changes, a 
 disease picture may occasionally appear showing absence of the patellar reflex, 
 lancinating pains, sensory disturbances, which closely resembles a tabes 
 dorsalis, especially if to the spinal phenomena, there are added cerebral 
 symptoms analogous to those of tabes — pupillary rigidity, paralysis of the 
 muscles of the eye — as a result of basal meningitis (Oppenheim) . Such cases 
 have been spoken of as a pseudotabes syphilitica. 
 
 The special peculiarity, which the course of a meningomyelitis syphilitica 
 is constantly showing, is, that frequently, a very striking change in the inten- 
 sity and extent of the symptoms just described may be observed. A repeated 
 appearance and disappearance, now sudden, now -gradual, of single paralytic 
 phenomena is no unusual occurrence. One sees abducens and oculomotor 
 pareses disappear again after existing but a few days, slight parapareses of 
 the legs quickly being transformed into severe paraplegias; pronounced 
 bladder disturbances may disappear again in a short time; in like manner 
 the neuralgic troubles caused by radicular irritation show considerable 
 fluctuations in severity and extent. Oppenheim has called attention in 
 particular also to the condition of the tendon reflexes, which may appear 
 diminished, abolished, or actively increased, all within a few days. It 
 has been attempted to explain this change in individual symptoms, which 
 is occasionally sudden and always characteristic of lues spinalis by the fact 
 
ERB'S SYPHILITIC SPINAL PARALYSIS 397 
 
 that, as a result of the change in the amount of blood the tissues, the granula- 
 tion tissue, the roots of the spinal cord and the cord are exposed to pressure 
 of varying force. Another important sign of lues spinalis depends upon 
 the fact that the syphilitic process does not, as a rule, confine itself to the 
 spinal cord, but attacks also the meninges and the vessels of the brain and 
 above all, basal cerebral phenomena appear at an early period. In the 
 majority of cases, therefore, there will appear, in addition to the spinal 
 symptoms, also some few cerebral paralytic symptoms — disturbances in the 
 muscles of the eye or in vision — which exhibit the same instability as do those 
 of the spinal cord. 
 
 In the year 1892 Erb separated another disease entity, under the desig- 
 nation of syphilitic spinal paralysis, from the large and varied group of 
 affections of the spinal cord, which are based upon syphilis. He had sus- 
 pected, that similar anatomic changes would be found at the basis of the 
 symptom complex sketched by him, as those of the meningomyelitis just 
 discussed, that is, in particular, specific myelitis, or arteritic and gummatous 
 processes. This hypothesis has not found support in the postmortem find- 
 ings that are available thus far. For it has been shown that various changes 
 in the spinal cord come under consideration as the pathologic-anatomical 
 foundation of ErVs symptomatic picture. In a number of cases, a sclerosis 
 of certain tracts of the spinal cord, of the pyramidal and lateral cerebellar 
 tracts and of the columns of Goll were found, without any participation of 
 the meninges, or any specific changes in the vessels being noted, so that 
 anatomically, the picture of a primary combined system disease was present. 
 In other cases, however, a chronic, disseminated or partial myelitis transversa 
 was found with ascending and descending secondary degenerations, or even 
 a combination of diffuse chronic myelitis with apparently primary columnar 
 degenerations. The thickening of the walls of the vessels, which was 
 demonstrated especially in the chronic myelitic foci, did not usually exhibit 
 the picture of specific end-arteritic changes, even though some observers, 
 like Nonne, proved the presence of apparently specific end-arteritis in some' 
 arteries, in addition to these ordinary thickenings in the walls of the vessels. 
 
 Erb's disease picture, in its pure form, is rather clearly defined. In 
 individuals, who previously have been infected with syphilis, there develops 
 gradually, and usually in an insidious manner, more commonly in the 
 earlier stages of syphilis, about two to six years after the infection, a spastic 
 paresis of the legs with considerable increase of the tendon reflexes and the 
 Babinski reflex. The gait is distinctly spastic, even if the muscular rigidity 
 is but slightly pronounced. Besides the weakness in the lower limbs, 
 there appears as a regular and early symptom, which sometimes precedes 
 all the other phenomena, a disturbance of the bladder. The sensory disturb- 
 ances and parcesthesias, which are demonstrable with equal frequency, are 
 usually of slight degree. Variations from this symptomatic picture may 
 
398 DISEASES OF TEE SPINAL CORD 
 
 occur as follows: the beginning of the disease may occasionally be rather 
 acute with quickly developing paraplegia and muscular contractures. In 
 the pure form of syphilitic spinal paralysis, according to Erb, we always 
 note the absence of more severe pains, of more severe sensory disturbances, as 
 well as of all phenomena which point to a disease of the brain or its membranes. 
 
 The course of the disease is, as a rule, slow, extending over many years, 
 with occasional improvements and standstills. But there is also a form 
 with a more rapid course and a fatal termination after a few years. 
 
 Diagnosis. — Considering the frequency of spinal diseases originating 
 upon a syphilitic foundation, and the ability of syphilis to appear through 
 its multiple distribution in all the different parts of the nervous system in 
 the most varied symptomatic pictures, it would be well always to think of 
 syphilis in all diffuse diseases of the spinal cord, unless we have before us 
 absolutely typical disease pictures. 
 
 In our inquiry into the previous history and our investigation of all the 
 other organs of the body, we must make it our chief aim to look up former 
 diseases, in which there was a suspicion of syphilis, and notice with special 
 care all signs of recent or past lues. According to experience, however, our 
 endeavors in this direction are not rarely entirely fruitless, even where the 
 disease of the spinal cord proves later to be surely syphilitic. 
 
 Differentiation of a meningomyelitis syphilitica from a simple acute or 
 subacute transverse myelitis and from a compression paralysis of the spinal 
 cord, will as a rule, cause no difficulties. The more severe pains and a 
 neuralgic prodromal stage do not belong to the disease picture of an acute 
 myelitis. As contrasted with a caries, which exists for a long time without 
 deformity of the spinal column, sufficient signs for distinction will be found 
 in the fact that the compression paralysis, as a rule, has a gradual steady 
 course, whereas the lues spinalis is characterized particularly by the coming 
 and going of phenomena and usually creates a disease picture pointing to 
 multiple foci. The distinction from multiple sclerosis, which likewise fre- 
 quently exhibits a striking change of sudden deteriorations and improve- 
 ments, can occasionally cause difficulties. The differential diagnosis will 
 mostly be based on the fact, that pronounced sensory phenomena of irrita- 
 tion are absent in sclerosis and that, on the other hand, the characteristic 
 intention tremor, the speech disturbance, the partial optic atrophy and nystag- 
 mus are foreign to lues spinalis. 
 
 Both multiple sarcomatosis and carcinomatosis of the membranes may give 
 rise to a confusion with lues spinalis. Here only the further course and the 
 result of specific treatment will make diagnosis possible. 
 
 ErVs syphilitic spinal paralysis, in its pure form, presents an easily defined 
 disease picture; it is distinguished from the simple spastic spinal paralysis 
 {Erb) by the presence of sensory and bladder disturbances, from meningo- 
 myelitis by the absence of sensory meningeal and radicular irritation symp- 
 
PROGNOSIS AND TREATMENT OF SPINAL SYPHILIS 399 
 
 toms and the absence of cerebral phenomena. In differentiation from 
 combined disease of the posterior and lateral columns, the course of the 
 disease must be specially considered. In combined columnar disease, there 
 is a steadily progressing process, which step by step, attacks one system after 
 the other. The course of Erb's disease is not, on the other hand, so regular; 
 standstills and improvements are not unusual; likewise even total disappear- 
 ance of single symptoms under specific treatment. 
 
 Erb himself emphasized the fact, that a large number of transitional 
 forms with their deviations, which are, in part, considerable, from the pure 
 picture of syphilitic spinal paralysis, may often exhibit far-reaching resem- 
 blance to the most varied forms of lues spinalis, so that certain differentia- 
 tion, particularly from meningomyelitis, is frequently impossible. Many 
 prominent neurologists advocate the point of view {Oppenheim and others) 
 that the symptom complex described by Erb represents only an initial stage, 
 or the picture of one phase of meningomyelitis chronica syphilitica. 
 
 Prognosis. — Complete recovery from lues spinalis is possible; it is to be 
 expected particularly when the disease is treated early, before the phenomena 
 of a lesion of the cord have appeared. But even after the development of 
 an incomplete transverse lesion, there is still, according to Oppenheim' s 
 experience, possibility of absolute restoration. In the great majority of 
 cases, however, the outcome of the disease, as soon as the cord itself has 
 suffered, is quoad restiturionem, unfavorable, as more or less considerable 
 functional disturbances remain in the form of spastic pareses, bladder dis- 
 turbances, etc. The prognosis even in the cases whose course is favorable 
 is, nevertheless, made less favorable by the fact that a relapse may occur at 
 any time. 
 
 Treatment. — The treatment consists in inunction which is continued 
 for several weeks (using 1-2 drams ung. hydrarg.) with simultaneous 
 administration of iodide of potassium (at the beginning 15 grs. ; later, 
 60 grs. daily) . Oppenheim recommends a rapid increase in the dosage of 
 iodide of potassium, or to give large doses at once (up to 200 and 300 grs. pro 
 die) ; he has also had results from internal or subcutaneous employment of 
 iodipin (10-20 g. of the 25% solution injected daily for 8-10 days), when 
 mercury and iodide of potassium remained ineffective. 
 
 The inunctions of 1 to 2 drams ung. hydrarg. pro die, had better be made 
 in the evening before going to bed, since the absorption of the mercury is 
 effected in the inunction treatment, mainly through inhalation of the mercu- 
 rial fumes. The most suitable places upon the body to be used alternately, 
 have been shown to be the calves, the outer and posterior surface of the thighs, 
 the region of the abdomen and the loins, the lateral parts of the thorax, the outer 
 and posterior surface of the fore- and upper arm. According to experience, 
 the following parts have proved sensitive to the salve, the flexor aspect of 
 
4 oo DISEASES OF THE SPINAL CORD 
 
 the joints, the axilla, the inner surface of the thighs and all places upon the 
 skin with but a small amount of underlying softer parts as, e. g., the skin over 
 the tibias. Between each group of five days, on which inunctions but no 
 baths are given, one sandwiches in a sixth day, in which the patient is 
 treated only to a warm cleansing bath. To avoid a mercurial disturbance 
 of the mucous membranes of the mouth, which in its initial stage, manifests 
 itself by an increase in the secretion of saliva, a metallic taste in the mouth 
 and a feeling as if the teeth are growing longer, persistent care of the mouth 
 is essential. The mouth should be cleansed after every meal. 
 
 A repetition of the inunction treatment is necessary, as soon as new 
 symptoms of disease appear. The question, how long shall the treatment 
 be continued, Gowers answers by stating that after a course of treatment 
 extending over 6 to 10 weeks without visible results, further continuation 
 for the time at least is useless. If there has been improvement in the dis- 
 ease, and a standstill has been reached, it seems advisable to order regularly 
 in the next few years, one or two courses of antisyphilitic treatment, even if 
 relapses have not occurred. The combination of the inunction treatment 
 with baths in various resorts (Aachen, Toelz, Nenndorf) is frequently recom- 
 mended. In addition to the specific treatment, it is important above all to 
 regulate the patient's entire manner of life : avoidance of all physical and 
 mental overe-xertion and of all sexual and alcoholic excesses. 
 
 IV. Myelitis 
 
 Definition. — We are not at present in a position to draw a sufficiently 
 sharp line from the clinical or pathologic-anatomical point of view between 
 disease pictures, which are based on acute and subacute changes in the 
 spinal cord with the signs regarded as characteristic of an inflammation, 
 and those, in which phenomena of acute degeneration and disintegration of 
 the cord substance are preponderantly demonstrable. Most clinicians, there- 
 fore, classify for the present as myelitis all diffuse and disseminated inflam- 
 matory and softening processes in the spinal cord, in so far as they are not 
 the result of a direct crushing or contusion of the cord. 
 
 Classification of the Forms of Myelitis and Anatomic Findings 
 
 One distinguishes a transverse myelitis, in which a focus, variable as to 
 the height of its location, takes up all or the greater part of the transverse 
 section of the spinal cord; also, a myelitis disseminata, with numerous smaller 
 and larger foci strewn over the entire length of the medulla spinalis, usually 
 associated with participation of the medulla oblongata and the brain; and 
 finally, poliomyelitis, which depends upon inflammatory changes, localized 
 mainly in the anterior gray horns. 
 
MORBID ANATOMY OF MYELITIS 401 
 
 The more recent investigations have shown that a separation of these 
 different forms of myelitis is justified only in so far as by the nomenclature 
 attention is called to the chief focus of the disease and the special localiza- 
 tion of the changes through which the disease picture attains its peculiar 
 stamp. As a rule, both in acute myelitis transversa and in poliomyelitis, 
 there are, in addition to the main focus, countless small and minute inflam- 
 matory foci, scattered over the gray and white matter, so that in both these 
 forms therefore, there is exhibited the anatomic picture of a disseminated 
 myelitis. 
 
 A diffuse myelitic focus may be recognized even by palpation and by 
 macroscopic inspection, because of its softer consistency and the more vivid 
 injection of the vessels of the soft membranes in contrast to the adjacent 
 sound parts of the spinal cord. When the softening is very pronounced, 
 the grayish-yellow discolored substance of the cord flows over the surface 
 of an incision, while in less marked development of the changes, only the 
 lines of separation between the gray and white matter seem blurred. The 
 microscopic appearance of the hardened preparation may vary considerably. 
 Occasionally the inflammatory changes of the vessels are comparatively 
 unimportant as compared with the acute phenomena of degeneration of 
 the nerve elements. The axis cylinders and medullated sheaths seem to be 
 much swollen and inflated like bubbles, either singly, or in groups, so that 
 the volume of a nerve fibre may often amount to from 10 to 20 times its 
 normal size. Through disintegration of the diseased fibres, there are 
 formed spaces in the tissue between the nerve fibres, which are still un- 
 changed; these spaces are filled with granular detritus, cord remnants and 
 drops of fat (Fig. 89). As the glia tissue also, at first by swelling, and 
 later by disintegration, participates in the process, softening foci develop. 
 Compensation takes place through the proliferation of the interstitial tissue 
 in the neighborhood of the focus of degeneration; a sclerotic scar tissue is 
 formed. During this process, the entire focus contains numerous, so-called 
 granular cells, which are loaded with disintegrated particles of tissue and 
 are to be found especially in the perivascular spaces of the vessels, which 
 are usually thickened. 
 
 In some cases, especially in myelitis disseminata and in poliomyelitis, 
 it is not only the phenomena of acute degeneration in the nerve parenchyma, 
 which occupy a prominent position in the anatomic picture, but also inflam- 
 matory changes in the vessels. They are present in the form of pronounced 
 dilatation of the arterial and venous pathways, possibly with an extravasa- 
 tion of blood into their surroundings and dense accumulation of round cells 
 in the adventitia of the vessels and the perivascular lymphatic spaces. After 
 all more extensive processes of inflammation and softening in the spinal 
 cord, there is a development of " secondary degenerations" of the ascending 
 and descending spinal-cord tracts, which have become separated from their 
 26 
 
402 
 
 DISEASES OF THE SPINAL CORD 
 
 ganglion cells. The soft membranes of the spinal cord are, as a rule, also 
 affected in myelitis. Even in acute poliomyelitis, in recent cases, changes 
 in the pia were found by Dauber, Schultze, and others in addition to the 
 changes in the vessels. 
 
 Bacterial Findings. — The presence of bacteria in the inflammatory foci, 
 the meninges and the cerebro-spinal fluid, has been demonstrated, hereto- 
 fore, only in rare cases, even when the disease appeared in direct connection 
 with infectious processes. In the diseased tissue of the spinal cord itself 
 
 f . l%vi'~4* 
 
 
 '■ : 
 
 ... 
 
 
 Fig. 89. — Myelitic focus in acute myelitis with numerous swollen axis cylinders (a) and lacuna 
 through, disintegration of nerve fibres (&). 
 
 have been found in addition to tubercle bacilli, strepto-staphylo- and pneumo- 
 cocci, also the bacilli of anthrax and typhoid; in the cerebro-spinal fluid, in 
 poliomyelitis, the presence of extra-cellular diplococci was demonstrated by 
 Schultze and others, the more exact determination of which as meningococci 
 by cultural proceedings was not possible; Strilmpell was able to isolate 
 staphylococci in the fluid of a patient with myelitis, which had supervened 
 in a case of whitlow. 
 
 According to the experiments of Hoche, Homen and others, the rarity of 
 bacterial findings may be due to the fact, that the bacteria after their pene- 
 tration into the substance of the spinal cord and into the cerebro-spinal 
 fluid, quickly disappear again. 
 
 iEtiology. — In the aetiology of acute myelitis, in all its different forms, 
 infections and intoxications play the most important role. There is probably 
 no acute infectious disease, in the wake of which the appearance of a mye- 
 litis has not been observed. I must mention especially smallpox, typhoid, 
 
ETIOLOGY OF MYELITIS 403 
 
 diphtheria, influenza, erysipelas, measles, scarlet fever, malaria, gonorrhea. 
 Even in connection with purulent processes in the various organs of the body : 
 in whitlow, perityphlitis, suppuration in the antrum of Highmore, an acute 
 myelitis is occasionally observed. Furthermore, in syphilis and tuberculosis, 
 acute myelitis occurs, which, anatomically, does not present the specific 
 changes characteristic of these diseases. For the myelitis, which occa- 
 sionally appears in pregnancy and in the puerperium, an infectious cause is 
 also assumed. Since investigators have succeeded experimentally, by the 
 introduction of toxic bacterial products, in producing inflammatory changes 
 of the spinal cord, which bear great similarity to those in acute myelitis, it is 
 very probable that myelitis is caused not only by the local effects of the bac- 
 teria themselves, but also by the toxins produced by them; they may reach 
 the spinal cord by means of the blood and the lymphatics. 
 
 Catching cold, over-exertion, and psychic excitement were formerly thought 
 by many to be the only sources of certain cases of acute myelitis ; this seems 
 doubtful in the light of more recent experience; however, one must assign 
 to them the importance of an influential assisting and exciting cause under 
 certain circumstances. 
 
 After poisonings with chemical substances of the most different varieties 
 — CO, illuminating gas, bisulphide of carbon, chloroform, lead — a larger 
 number of cases of myelitis having a more chronic and subacute course 
 rather than taking an acute form are observed; but acute cases do occur and 
 especially after poisoning by smoke in firemen, suddenly appearing phenom- 
 ena of spinal paralysis are not at all uncommon. 
 
 Those diffuse changes in the spinal cord, which with numerous small 
 acute foci of degeneration, develop upon the basis of a cancerous cachexia in 
 carcinoma, furthermore, in pernicious ancemia, leucaemia, chronic nephritis, 
 diabetes and gout, exhibit clinically, as a rule, also a more subacute ox chronic 
 character. In some of these cases morbid products of metabolism may be 
 considered to be the cause of the inflammation; in others it may be a ques- 
 tion of necroses and softenings resulting from the anaemia and general 
 cachexia, which so frequently accompany these diseases. 
 
 A peculiar form of myelitis, observed in workers in tunnels, and desig- 
 nated Caisson paralysis, needs special mention. As a result of a sudden 
 decrease in high atmospheric pressure, there is a development of gas bubbles 
 in the blood, and therewith gas embolism occurs in the blood-vessels includ- 
 ing the vessels of the spinal cord. Through these macroscopically and 
 microscopically recognizable embolizing gas bubbles there arise numerous 
 small ischaemic softening foci. Finally, we must mention the rare occur- 
 rence of softening as the consequence of an embolic closing of the aorta 
 abdominalis, also embolic and thrombotic occlusions of the arteries of the 
 spinal cord. 
 
 Symptomatology. — The phenomena of the disease set in very quickly 
 
4 o 4 DISEASES OF THE SPINAL CORD 
 
 in most cases of acute myelitis, so that after a brief prodromal period, during 
 which slight pains in the back and sacral region, weariness and paresthesias 
 in the legs are complained of, the symptoms of paralysis develop more or 
 less suddenly in a few hours or in the course of several days. The onset is 
 usually with fever; fever that lasts for weeks and occasional exacerbations 
 of fever with aggravation of the spinal symptoms frequently occur. 
 
 Even at the acme of the disease, the inflammatory focus does not, as a 
 rule, extend symmetrically over the entire transverse section, but leaves 
 larger or smaller areas of the gray and the white matter untouched. Accord- 
 ingly, the grouping of symptoms and the intensity of the phenomena of the 
 paralysis, are dependent, not only on the height of the localization, but also 
 and to a great degree on the manner of the diffusion to the individual tracts 
 of the spinal cord. In discussing the spinal phenomena, we shall start with 
 the most frequent form of myelitis dorsalis. 
 
 i. The disturbances of motility stand in the foreground of the disease 
 picture. A total paraplegia of the lower extremities develops if the lateral 
 pyramidal and anterior columnar tracts are destroyed by the great extent 
 of the myelitic focus. In an incomplete transverse myelitis, a series of 
 muscles will still be able to carry out their functions, or all movements of the 
 legs will still be possible, but with decreased strength. The paralysis is at 
 first flaccid; but very soon there appears a muscular stiffness, a spastic condi- 
 tion, which can lead first to a position of extension, later to severe flexion 
 contractures in the paralyzed legs. Not only in the beginning, but also in 
 the further course of the disease, motor irritation phenomena, twitchings, 
 appear in the paralyzed limbs, which partly, at least, depend on the increased 
 reflex excitability, since even slight irritations of the skin, such as moving 
 the bed cover, change of position, etc., suffice to evoke painful muscular 
 contractions; partly, also, they may be the result of a direct irritation of motor 
 fibres of the spinal cord. . The participation of the abdominal muscles is 
 recognizable even in its slight degrees by the fact that the patient is not 
 able to rise from a supine position, without aiding himself with his hands. 
 
 2. The tendon reflexes in the legs are usually increased. The morbid 
 increase manifests itself in that when the knee-cap or the tibia of the extended 
 leg is lightly tapped, a contraction of the quadriceps muscle results, and in 
 that patellar and ankle clonus may be excited. A permanent loss of the 
 tendon phenomena has been observed only in those cases in which the 
 disease approximated a total transverse interruption of the cord. It is 
 noteworthy, that as a result of high degrees of contractures, the excitation 
 of the tendon reflexes may be made difficult or impossible. 
 
 The cutaneous reflexes are usually retained and actively increased, so 
 that by pinching a fold of the skin in the paralyzed and anaesthetic regions 
 muscular contractions of that or even of the opposite extremity result. The 
 plantar reflex shows the alteration so frequently characteristic of a spastic 
 
SYMPTOMATOLOGY OF MYELITIS 405 
 
 paralysis, dorsal flexion of the great toe when the sole of the foot is stroked, 
 especially on its outer side (Babinski's sign) . In more extensive transverse 
 lesions the cutaneous reflexes like the tendon reflexes may disappear also. 
 The abdominal reflexes are partly or entirely lost, if the reflex arc subserved 
 by the posterior roots of the ninth to the twelfth intercostal nerves is destroyed 
 by the location of the myelitic focus in the lower dorsal cord. 
 
 3. The sensibility, as a rule, exhibits considerable disturbance, even to 
 the total loss of all qualities of sensation in the legs and trunk, up to a level 
 corresponding to the region subserved by the segment of the spinal cord 
 which is affected by the focus. If the process is restricted to a smaller part 
 of the cross-section, the disturbance in sensation extends only to one leg, 
 or parts of the trunk, or consists only in disturbances of certain qualities of 
 sensation, whereby the isolated diminution in muscle, tactile and pressure 
 sense points to a predominant disturbance in the posterior columns, impair- 
 ment of the temperature and pain sensations, to an affection of the posterior 
 horns. 
 
 4. Bladder disturbances are frequently an early symptom of acute mye- 
 litis, which may be discovered even in the prodromal stage. A participa- 
 tion of the bladder and rectum is never absent in any of the more extensive 
 transverse lesions. In the beginning, there is a slight difficulty in micturition, 
 which as a result of the paralysis of the detrusor muscle quickly increases 
 to a total retentio urines. Overdistension of the bladder is occasionally not 
 perceived by the patients at all, as, because of the lesion of the sensory 
 tracts, the conduction of the irritation impulses arising from the mucous 
 membrane of the bladder and going to the cerebrum no longer takes place. 
 As further consequence of the excessive filling and dilatation of the bladder, 
 there appears a slow dribbling of the urine, an ischuria paradoxa. As a 
 rule, after a certain time, the mechanism of the bladder works independ- 
 ently, in such a way that at comparatively regular periods, as soon as the 
 bladder has reached a certain degree of fulness, reflex evacuation takes place. 
 The disturbance in rectal activity shows itself usually as a high degree of 
 sluggishness of the bowels. 
 
 5. The trophic disturbances of the skin are of special importance. Far 
 more quickly than with other bed-ridden patients is an acute appearance of 
 decubitus observed in myelitis. As early as the first 24 to 48 hours after the 
 appearance of the severe phenomena of paralysis, bed-sores due to pressure 
 may develop, so that careful treatment of the skin, belongs to the most im- 
 portant duties of the physician, from the very beginning of the disease. 
 The favorite places for bed-sores are, beside the sacral bones, the region 
 of the trochanters, the heels and the shoulder-blades, and the inner surfaces 
 of the knees where they rub against each other. Frequently, also, edema 
 and sclerodermoid conditions develop during the longer continuation of the 
 disease. 
 
4 o6 DISEASES OF THE SPINAL CORD 
 
 6. Of cerebral complications of a focal myelitis only neuritis optica is 
 worth mentioning. Other disturbances in the realm of the cerebral nerves 
 are usually absent in the non-disseminated form. 
 
 If the seat of the focus is not, as is usual, in the dorsal portion of the spinal 
 cord, but in the cervical or lumbar portion, we must expect certain deviations 
 from the disease picture just described. 
 
 Myelitis cervicalis is the rarest and most dangerous form of myelitis. 
 If the focus lies above the cervical enlargement there is found, besides spastic 
 paralysis of the legs and the usual phenomena of a dorsal myelitis, also a 
 spastic paresis of the arms; a bilateral phrenicus paralysis is followed by 
 death through suffocation. If, however, the focus is in the region of the 
 cervical enlargement, there develops — because of the destruction of the gang- 
 lion cells of the anterior horn — & flaccid, atrophic paralysis of the arms with 
 electric reaction of degeneration. The synchronous appearance of oculo- 
 pupillary symptoms due to sympathetic paralysis — contraction of the pupils 
 and of the palpebral fissures — indicates that the eighth cervical and first 
 dorsal segments are also affected. 
 
 In a myelitis lumbalis, the upper extremities are quite free; there exists 
 & flaccid degenerative paralysis of the legs with weakening or absence of the 
 tendon reflexes. The paralysis of sensation is restricted to the lower extrem- 
 ities alone ; bladder and rectum are paralyzed. 
 
 If the lower sacral cord is principally affected, it is preeminently the sciatic 
 region which is paralyzed — in the motor aspect — and the sensory disturb- 
 ances extend only to the back of the thighs and their inner half to the lower 
 third on the buttocks and the genital organs. Besides, there exist disturb- 
 ances in bladder and rectum. 
 
 Myelitis disseminata requires special discussion, since the disease picture 
 may exhibit many different forms, especially when greater participation 
 of the medulla oblongata and the brain is shown to exist (Fig. 90). When 
 through numerous smaller foci a larger part of the transverse section of the 
 spinal cord is deprived of its function, the disease may present clinically so 
 completely the picture of an acute transverse myelitis, as to make it well- 
 nigh impossible during lifetime for a diagnosis of disseminated myelitis to 
 be made. Furthermore, a symptom complex may develop which bears the 
 greatest similarity to a multiple sclerosis running an acute course. In other 
 cases, an acute ataxia, associated with loss of tendon reflexes and bladder 
 disturbances, or even with spastic pareses, occupy the foreground of the 
 symptomatic picture. Occasionally, also, it may happen that in the begin- 
 ning of the disease, the cerebral phenomena are very much pronounced as 
 compared with the symptoms of spinal paralysis, so that the picture of a 
 pure encephalitis may be believed to exist. 
 
 Course. — As a rule, myelitis reaches the acme of its development within 
 a few days and weeks; death may occur rapidly or possibly an improvement 
 
CHRONIC MYELITIS 407 
 
 may ensue (with occasional sharp relapses, which are accompanied by 
 renewed fever), which may lead to complete recovery. This, however, is 
 rare indeed; usually the severe phenomena of paralysis are but partially 
 recovered from, and there comes to be a permanent condition of illness, in 
 the course of which, death may appear at any time, as a result of decubitus 
 or cystitis. 
 
 Fig. 90. — Myelitis disseminata. Cross-section through the pons with numerous small in- 
 flammatory foci grouped around the vessels (a). (Author's own observation.) 
 
 Chronic Myelitis 
 
 in which the disease picture of a progressive diffuse transverse myelitis 
 develops insidiously from the first, is an extremely rare disease. Only in 
 isolated cases, heretofore, could be demonstrated an anatomic basis for 
 a disease of the spinal cord, exhibiting the picture of chronic myelitis, the 
 presence of a diffuse chronic myelitis. In the great majority of cases, another 
 disease is hidden behind the symptom complex of a chronic transverse 
 myelitis, a multiple sclerosis, a lues spinalis, a combined column disease, or a 
 compression paralysis of the spinal cord. 
 
 In chronic dorsal myelitis, the initial symptoms are first of all a slight 
 tendency to fatigue in walking with increasing stiffness in the muscles and 
 abnormal sensations, paresthesias, in the legs. Actual pains are only tran- 
 sitory, and usually present only in slight degree. The tendon reflexes 
 frequently show a considerable increase with or without accompanying mus- 
 cular rigidity. The objective sensory disturbances usually remain slight, 
 
4 o8 DISEASES OF THE SPINAL CORD 
 
 as compared with the gradually appearing phenomena of. motor paralysis. 
 Bladder and rectal disturbances and impotence are regular concomitant 
 phenomena in advanced stages of the disease. The duration, as a rule, ex- 
 tends over years; there may be a standstill, and long periods of improvement. 
 
 Diagnosis. — For the diagnosis of acute myelitis transversa, it will fre- 
 quently be of importance to show that the beginning of the disease was con- 
 nected with certain diseases, infections, etc., which, as we know from experi- 
 ence, play an aetiological role in acute myelitis. Confusion with suddenly 
 occurring hemorrhage of the spinal cord is possible, since in this disease-, 
 also, prodromal phenomena, paresthesias, twitchings may appear, caused 
 by disturbances in circulation, as a result of diseases of the blood-vessels. 
 Differentiation from a pressure paralysis in caries or vertebral carcinoma, 
 both of which may occasionally develop rapidly, will, as a rule, cause no 
 difficulty, since the neuralgic prodromal stage and eventually, deformities 
 of the spinal column afford sufficient points for diagnostic differentiation. 
 Schultze observed in one instance that a tumor of the spinal meninges (pos- 
 sibly by a transitory damming back of the cerebro-spinal fluid) might rather 
 suddenly exhibit the phenomena of transverse lesion, and thereby simulate an 
 acute myelitis. In such cases, only further observation, the proof of the 
 steady and irresistible progress of all spinal symptoms will make diagnosis 
 possible. Separation from a polyneuritis, which may also appear in connec- 
 tion with infectious diseases, will be called into question only when, in lumbar 
 myelitis, the ganglion cells of the anterior horns are destroyed, and atrophic 
 degenerative muscular paralyses are present. The neuritic pains on the 
 one hand, and on the other the bladder disturbances, which are very rarely 
 found in polyneuritis, are points for differentiation between them. 
 
 Confusion with hysteric paralyses is possible especially when, associated 
 with the usual hysteric phenomena, there is present a real transverse myelitis. 
 The Babinski sign has proved to be a sure sign for distinguishing between 
 functional and organic paralysis. Beside this, the change in the severity 
 of the phenomena of paralysis, the occasionally striking contrast between 
 the ability to walk and the mobility of the legs when the patient is lying on 
 his back, as well as the effect of suggestive influences, make possible the 
 detection of the hysteric nature of the disease. 
 
 In the differential diagnosis of chronic myelitis, the most important dis- 
 eases are compression paralyses due to caries, vertebral tumors and tumors 
 of the spinal cord, sclerosis multiplex, lues cerebrospinalis and combined 
 column diseases. We would refer to the chapters concerning them. 
 
 Prognosis. — This is always serious in myelitis. The chances of pre- 
 serving life and of complete or partial functional recovery are the less favor- 
 able, the more quickly the phenomena of a total transverse lesion are devel- 
 oped. Especially dangerous is the disease of the upper cervical cord, 
 because of the possibility of the occurrence of paralysis of the diaphragm. 
 
TREATMENT OF MYELITIS 409 
 
 Even after the disappearance of the acute phenomena, if there is a stationary 
 condition with partial functional restoration, the disease may at any time 
 terminate fatally in consequence of decubitus and cystitis with their resulting 
 phenomena. Those cases of myelitis which appear after acute infectious 
 diseases offer the best chance of recovery. Syphilitic myelitis which develops 
 soon after the infection, frequently runs quite a rapid course. 
 
 Treatment. — In the acute stage our chief aim will have to be the pre- 
 vention of decubitus and cystitis. The early use of water or air beds and 
 frequent bathing with alcohol and water of those parts of the body which 
 are especially exposed to pressure, act as prophylactics with respect to 
 pressure bed-sores which so frequently develop with considerable acute- 
 ness precisely in myelitis. For the rest, absolute quiet in bed at the begin- 
 ning of the disease, and even a long time after the disappearance of 
 the symptoms of the spinal paralysis, is necessary. In the post-infectious 
 forms, diaphoretic procedures and the administration of preparations of 
 salicylates are recommended. If there be a suspicion of syphilis, iodide 
 of potassium (gr. x t.i.d.) should be tried; mercurial treatment (3j ung. 
 hydrarg.), in which there is always a possibility that the Hg. may have an 
 injurious effect upon the more severely diseased tissue, must be stopped 
 at once if an unfavorable result is perceived. In previous malarial in- 
 fection, quinine and arsenic may have favorable results. After lumbar 
 punctures we have observed frequently an objective improvement, return 
 of the reflexes, increase in mobility. 
 
 If frequent catheterization is necessary, besides very painstaking anti- 
 sepsis, a prophylactic administration of urotropin (gr. v t.i.d.) is advisable. 
 
 In the stationary stage with residuary phenomena of pareses and slight 
 spastic conditions, treatment by baths is to be tried first. There is need of 
 serious warning against hot baths. Simple warm baths {()2°-g6° F. and 
 lasting 15 minutes) are most useful, which in case of more severe spastic 
 phenomena may be extended over a long time (1-2 hours). Treatment at 
 thermal resorts where the hot springs contain carbonic acid, Oeynhausen, 
 Nauheim, and others like Ragaz, Teplitz, Wildbad are to be recommended. 
 
 As a means of comfort for the patients with stationary conditions of 
 paralysis, the galvanic current is recommended for direct treatment of the 
 spinal cord (3 to 5 minutes of not too strong, stabile galvanic current on 
 the spinal column) and for muscular nerve stimulation in the paralyzed 
 extremities. 
 
 We may expect from electricity an effect that will further recovery, only 
 when the phenomena of paralysis are themselves on the point of retrogres- 
 sion. If a bladder disturbance remains, galvanic treatment may be tried, 
 either both electrodes over the lumbar cord, or anode on the lumbar region, 
 cathode above the symphysis or upon the perineum. The strength of the 
 current, in a duration of 5 to 10 minutes, is to be 3 to 5 M. A. 
 
4 i o DISEASES OF THE SPINAL CORD 
 
 Supplement 
 
 Spinal-cord abscess, an extraordinarily rare disease, develops either 
 after an injury to the spinal cord, or is due to metastasis. The formation of 
 an abscess has been observed after putrid bronchitis, prostatic suppuration, 
 gonorrhoea, malignant endocarditis, appendicitis. Spinal-cord abscess is 
 usually of central location and may become very extensive; usually it is 
 connected with purulent meningitis, which occasionally appears only later, 
 so that lumbar puncture may give, at first, a negative result. 
 
 The disease picture, which is constructed from the phenomena of a 
 diffuse transverse disease with an acute onset, affords nothing character- 
 istic. Usually it will be concealed behind the symptomatic picture of an 
 acute myelitis and only by the detection of other purulent foci, synchronous 
 general pycemic phenomena and a purulent exudate found by lumbar puncture, 
 is the diagnosis made possible. 
 
 (7). DISEASES OF THE CONUS TERMINALIS AND THE CAUDA 
 
 EQUINA 
 
 BY 
 
 L. R. MULLER (Augsburg) 
 
 The lowest section of the spinal cord and the nerve roots going out from 
 it, are anatomically so different from the other parts of the spinal cord, that 
 the diseases there have a peculiar character. 
 
 The sacral cord with the conus terminalis is, for the most part, situated 
 behind the body of the first lumbar vertebra and extends downwards only to 
 the middle of the second lumbar vertebra. After opening of the spinal 
 canal and of the dura mater the lowest division of the spinal cord is not, 
 itself, visible. From the lumbar enlargement, spring in continuous series 
 thick bundles of nerves, which all running downwards, soon become so 
 numerous that they entirely conceal the lowest part of the sacral cord, 
 the conus. A separation between the sacral and lumbar cord, such 
 as is easily to be seen between the cervical and dorsal portions, or the 
 dorsal and lumbar portions, is to be determined only with chfficulty. Only 
 when the bundles of the cauda equina are traced to their exit from the 
 dural sac and from the spinal canal, that is 10 to 14 cm. downwards, can the 
 artificial boundary between the lumbar and sacral cord be determined by 
 counting the separate roots. But even if the boundary between the lumbar 
 and sacral cord can not be recognized morphologically, theoretically, at 
 least, a distinction must be maintained between the nuclear region of the 
 plexus lumbalis and that of the plexus sacralis or ischiadicus. In its lower 
 half, the sacral cord tapers off quickly; that part of the spinal cord which 
 embraces the third, fourth and fifth sacral segment and both coccygeal 
 
THE CONUS TERMINALIS AND THE CAUDA 411 
 
 segments, is called conus medullaris. In this lowest division of the spinal 
 cord, the relation between the anterior and the posterior roots is not so 
 uniform as in the rest of the spinal cord. If more than double the number 
 of posterior roots enter the cord here, as anterior roots spring from it, this 
 must be explained by the fact that no large group of muscles are to be 
 innervated from the third sacral segment downward. 
 
 Macroscopically, then, the upper boundary of the conus terminalis 
 can be determined only arbitrarily, or not at all. But it can be accurately 
 determined by histological examination. In the third sacral segment, the 
 character of the transverse section is considerably altered. The gray 
 substance, in comparison with the white, is excessively developed. As 
 Fig. 91 shows, the plump, broad anterior horns are abruptly rounded 
 
 * 5 * S> *lmm 
 
 ; \ -■ 
 
 'vft-i' ' W 
 
 Fig. 91. — Cross-section through the conus terminalis. 
 
 anteriorly, and no longer have any lateral protuberance, as they still have 
 in the upper sacral cord; the posterior horns fairly bulge, due to the luxuriant 
 development of the gelatinous substance. The ganglion cells in the anterior 
 horns are very scanty here, but they are all the more numerous in the tran- 
 sitional zone between anterior and posterior horns; from here, fibres radiate 
 directly into the postero-lateral columns, and into the roots that are there 
 situated. The lower the point in the conus from which the sections are 
 taken, the larger and more powerful are the ganglion cells in the "inter- 
 mediary zone." In the arrangement of the white substance also, essential 
 differences may be established in the conus from the other parts of the 
 spinal cord. At the same level of the cord at which the great ganglion cells 
 disappear from the anterior horn and new groups of them appear in the 
 intermediary zone, one finds that a bundle of medullated fibres is seen to- 
 pass directly from the posterior columns towards the front, that is, ventrally 
 towards the central canal and on both sides, increases the fibres in the 
 intermediary zone. Here, then, and this characterizes the beginning of 
 the conus, no posterior gray commissure of the gray substance can be found. 
 
4 i2 DISEASES OF THE SPINAL CORD 
 
 Transverse section above the conus shows the degeneration in the 
 lateral pyramidal columns does not extend downwards beyond the third 
 sacral segment. On the other hand, in the posterior columns of the sacral 
 cord and in its lowest part, in the conus alongside the septum medianum 
 posticum course very many descending conducting paths, whereas in the 
 cervical, dorsal, and lumbar segments, these tracts contain almost only 
 centripetal and ascendingly degenerating fibres, and it seems to be these 
 descending fibres, which then radiate towards the front into the gray sub- 
 stance, and are lost in the intermediary zone. 
 
 In the conus medullaris the centers for the sexual functions and for the 
 evacuation of bladder and rectum were always supposed to have been 
 situated, but more recent investigations cast a great deal of doubt upon 
 the correctness of this hypothesis. Thus, it can be demonstrated, that the 
 act of parturition may proceed normally, even after disjunction of the lowest 
 section of the spinal cord. Likewise it is certain that after the lower sacral 
 cord is completely destroyed, erection and ejaculation of the semen into 
 the urethra may still take place; as a result of the paralysis of the ischio- 
 and bulbo-cavernosi, however, the semen can no longer be ejaculated from 
 the urethra, but dribbles slowly away. 
 
 Neither can it be doubted any longer that the last nervous centers 
 subserving the bladder and the rectum are situated outside of the spinal 
 cord in the sympathetic nervous system; it must be remembered that this 
 theory concerns organs with involuntary musculature and such are not 
 innervated directly from the cerebro-spinal system anywhere in the body. 
 It "is true that the reflex, which subserves evacuation of urine and feces, 
 can be carried out voluntarily to a certain degree. But the formerly gen- 
 erally accepted hypothesis, that one center for the detrusor and another for 
 the sphincter vesicae are situated in the conus medullaris, is probably incor- 
 rect. On the contrary, we must conceive of the automatic process, which 
 leads to relaxation of the sphincter and to contraction of the walls of the 
 bladder, as being entirely excited in the sympathetic system. We cannot 
 decide, as yet, by what paths this reflex is evoked, whether by way of the 
 rami communicantes over the ganglion mesenter inferius and ganglion 
 hypogastricum, or whether possibly this process might be aroused by a 
 contraction of the striped musculature situated in the pelvis. 
 
 The conditions underlying the evacuation of the bowel-contents are 
 much clearer. By abdominal pressure, the column of feces is pushed 
 downward somewhat; then the peristaltic movement of the ampulla recti 
 takes hold and this leads to the ejection of the excrement. It is very 
 improbable, that in this process, a center for defecation in the conus is 
 actively concerned. True it is that the transversely striated sphincter ani 
 externus has its ganglion cells in the lower division of the spinal cord, just 
 as has the compressor urethras, which is also under the influence of the will- 
 
THE CON US TERM I NAZIS AND CAUDA 413 
 
 power; spontaneous peristaltic movements of the rectum and the musculature 
 of the bladder may therefore be repressed to a certain degree, i. e., it is 
 possible to retain urine and feces, even when desire to evacuate exists. 
 In like manner the centripetal paths, over which we are informed as to the 
 degree of fulness in the bladder and the ampulla recti, as well as about the 
 processes of motility taking place there, probably pass through the conus 
 terminalis. Thus is explained, that when the lower sacral cord is diseased 
 or the nerve bundles in the cauda equina passing thither are interrupted, 
 the patient is not aware of the fulness of the bladder and rectum, and is not 
 able to perform the automatic process necessary for the ejection of the 
 excrement (retentio urinae et fsecium). Nor is he able to inhibit the reflex, 
 once it has started, as in diarrhoea or in spontaneous evacuation of urine 
 by contraction of the voluntary musculature of the sphincters of the anus 
 and the bladder (incontinentia urinae et alvi). The disturbances in mictu- 
 rition and defecation in affections of the conus and the cauda equina do not 
 differ from those which develop in connection with transverse affections 
 in the rest of the spinal cord. First there is always retention of urine and 
 feces, later there appear involuntary automatic evacuations. 
 
 The diseases of the conus medullaris are often accompanied by those 
 of the upper sacral and the lumbar portion of the cord, or they attack the 
 bundles of roots which spring from these parts and surround the conus; 
 it is therefore advisable to give a short summary of the situation of the nuclear 
 areas of the individual muscle groups in the lumbar enlargement of the spinal 
 cord. But we must remark, that here only the center of a nuclear realm is 
 indicated, the nuclear realms of the larger muscle groups being mostly 
 extensive and reaching over several segments. 
 
 1. Lumbar segment: lower part of the abdominal muscles, quadratus 
 lumborum. 
 
 2. Lumbar segment: iliopsoas, cremaster, sartorius. 
 
 3. Lumbar segment: adductors of the thigh. 
 
 4. Lumbar segment: quadriceps femoris, tibialis anticus. 
 
 5. Lumbar segment: abductors of the thigh (glutaeus medius and 
 minimus), flexors of the knee (semimembranosus and semitendonosus 
 and biceps). 
 
 1. Sacral segment : glutaeus maximus, outward rotator of the hip, extensors 
 of the toes. 
 
 2. Sacral segment: gastrocnemius, soleus, peronei, flexors of the toes. 
 
 3. Sacral segment: ischio- and bulbocavernosus, perineal muscles 
 (compressor urethrae). 
 
 4. and 5. Sacral segment: sphincter ani externus, levator ani. 
 Figures 92-98 1 show what disturbances in sensibility correspond to 
 
 1 Figs. 92-98 are taken from a previous work of the author's (L. R. Mutter, Investigations 
 into the anatomy and pathology of the lowest segment of the spinal cord. Deutsche Zeitschrift 
 fuer Nervenheilkunde, vol. xiv). 
 
414 
 
 DISEASES OF THE SPINAL CORD 
 
 the diseases in the various segments of the lumbar enlargement. As one 
 may see from Fig. 98, the nerves from a small oval zone about the anus 
 reach the fourth sacral segment. In a lesion of the third sacral segment, 
 the anaesthesia extends also, to the perineum, the dorsum of the penis and 
 to the middle parts of the scrotum; in a lesion of the second sacral segment 
 the whole external genital organs are anaesthetic, the insensitive zone in the 
 buttocks has become considerably larger, extending to the upper portion of 
 the posterior aspect of the thigh; there is also a large insensitive area on the 
 outer portion of the foot. In lesions of the first sacral segment, this spreads 
 to the outer and posterior portion of the leg proper, which is supplied by the 
 peroneus nerve. Transverse diseases of the fifth lumbar segment lead to 
 
 Fig. 9: 
 
 -Lesion at the level of the 
 lumbar segment. 
 
 2d 
 
 Fig. 93. — Lesion at the level of the 3d lumbar 
 segment. 
 
 the typical "riding breeches" anaesthesia (Reithosenanaesthesie) . In all 
 these disturbances of sensibility, the testicles in the anaesthetic scrotum, since 
 they are innervated by the spermatic nerve from the second lumbar segment, 
 are still very sensitive to pressure. 
 
 Stroking the skin in the immediate vicinity of the anus causes contraction 
 of the sphincter ani and the levator ani. This reflex, the anal reflex, has its 
 center in the conus terminalis, hence may still be elicited in diseases of the 
 upper sacral cord. But the Achilles tendon reflex is lost in a disturbance at 
 this level since it has its seat in the region of the epiconus, in the second sacral 
 segment. The patellar reflex must be localized much higher still, in the 
 fourth lumbar segment. 
 
 As has been mentioned before, the lower end of the conus terminalis 
 reaches only to the upper margin, or at most, to the middle of the second 
 lumbar vertebra; from that point on the fibre bundle of the cauda equina, 
 
THE CON US TERM I NAZIS AND CAUDA 
 
 4*5 
 
 which is about as thick as a finger, contains only the filum terminale. The 
 parallel strands of the cauda equina must pass for a long way inside the 
 spinal canal, in order to reach their foramina of exit. Thus the first sacral 
 nerve arises at the level of the first lumbar vertebra and leaves the dural sac 
 
 Fig. 94. — Lesion at the level of the 5th lumbar Fig. 95. — Lesion in the 1st sacral segment, 
 segment. 
 
 Fig. 96. — Lesion in the 2d sacral segment. 
 
 Fig. 97. — Lesion in the 3d sacral segment. 
 
 only in the sacrum, in order to enter the pelvis from the first sacral foramen. 
 Therefore, it must course a distance of 14 cm. within the dural sac of an 
 adult. A cross-section through the cauda equina, at the level of the conus 
 terminalis strikes besides all the sacral nerves, all the lumbar roots also with 
 the exception of the first. Naturally, the lumbar roots which escape 
 
4i6 
 
 DISEASES OF THE SPINAL CORD 
 
 higher up, are situated at the outer side in the bundle of the cauda equina, 
 while the sacral fibres which escape farther down, are medially situated. 
 
 The sensory roots form their spinal ganglion only after their escape from 
 the dural sac; it reaches a quite considerable size in the lumbar nerves, but 
 especially so in the sacral nerves. Now, finally, after the sensory root has 
 passed through the spinal ganglion, it unites with the anterior roots to form 
 the peripheral nerve. The fibres of the cauda equina, therefore, are not, as 
 often happens, to be regarded as peripheral nerves, but as root fibres, i. e., 
 in the cauda, the motor and sensory bundles course separated from each 
 other; we may easily convince ourselves in processes in which only the 
 sensory tracts degenerate (for instance in tabes) that these sensory bundles 
 are situated all together, in one posterior field, while the motor roots are 
 
 VW 1 
 
 Fig. 98. — Lesion in the 4th sacral segment. 
 
 situated farther front, also joined in groups. It is clear that this fact is of 
 great importance for the diagnosis and localization of the disturbances with 
 which we are here concerned (caudal diseases). 
 
 After the escape of the caudal fibres from the spinal canal one distinguishes 
 five pairs of lumbar nerves, the first of which pass out between the first and 
 second lumbar vertebrae, the last between the fifth lumbar vertebra and the 
 sacrum; five pairs of sacral nerves which radiate outwards through the foramina 
 sacralia anteriora and posteriora and finally one coccygeal nerve, which after 
 passing the hiatus sacralis courses downwards. Now, while the intercostal 
 nerves form no plexus, the outgoing lumbar and sacral nerves are mingled, 
 each in a plexus, and now for the first time they separate into the real periph- 
 eral nerves. Thus, the distribution of fibres in the cauda equina and the 
 order of their origin from the spinal cord is different from the arrangement in 
 thejDeripheral nervous system. The plexus lumbalis is formed by the four 
 
ETIOLOGY OF DISEASES OF THE CONUS TERMINALIS 417 
 
 upper lumbar nerves, the sacral plexus, the large plexus situated on the 
 anterior surface of the pyriformis muscle, is made up from parts of the fourth, 
 from the fifth lumbar nerve and from the upper four sacral nerves. The 
 last sacral nerve and the coccygeal nerve unite to form the plexus coccygeus. 
 The nervus ano-coccygeus thus formed supplies the skin in the neighborhood 
 of the tip of the coccyx. 
 
 To summarize from the innervation of the pelvis, what is important for 
 diagnosis, we must point out that the anterior and external portions of the 
 pelvis and thigh get all their sensory supply from the lumbar plexus; only the 
 penis and the scrotum are supplied by the pudendal nerve, which springs 
 from the sacral plexus. The posterior portion of the pelvis, also, or, better, 
 of the buttocks, is innervated by the ramus iliacus of the nervus ilio-hypo- 
 gastricus and by the nervus cutaneus femoris lateralis from the lumbar plexus; 
 on the other hand, an oval zone, about the size of a saucer, lying around the 
 anus, and the perineum gets its sensory supply from the sacral plexus and 
 the coccygeal plexus. 
 
 The conditions within the pelvis are still more complicated. Of the 
 muscles there situated, the psoas, the iliacus internus, the obturator externus, 
 the cremasier and the tunica dartos are innervated from the lumbar plexus, 
 all the other small pelvic muscles (M. pyriformis, M. obturator internus, 
 Mm. gemelli), the musculature of the perineum, the anus, and the large 
 muscles of the buttocks receive their nerves from the sacral plexus. 
 
 The diseases of the conus terminalis are brought about by various causes. 
 Since the conus terminalis is part of the substance of the spinal cord, all 
 pathologic-anatomical processes, which attack the rest of the medulla spinalis, 
 may be localized occasionally in this, its lowest part, as well. As a matter of 
 fact, isolated cases of myelitis, haematomyelia, multiple sclerosis, and various 
 sorts of tumors have been described in the conus medullaris. In the great 
 majority of cases, however, traumatisms are responsible for the affections of 
 the conus. The first lumbar vertebra, behind which the conus is situated — 
 as a result of the static relations of the spinal column, in its capacity as a 
 transitional vertebra from the rigid, backwardly curved dorsal spinal column 
 to the movable lordotic lumbar spinal column — suffers with especial fre- 
 quency compression fracture from violent jolts, i. e., falls upon the legs or the 
 buttocks. By such an injury, the spinal canal is narrowed and the lowest 
 section of the spinal cord crushed. The fibres of the cauda equina, which 
 surround the conus, being far tougher and more capable of resistance, usually 
 do not surfer, when the first lumbar vertebra is broken, since they are able 
 to get out of the way in time, so that pure cord lesions without participation of 
 the roots have been observed. Fig. 99 shows the cross-section of a dural 
 sac, broadly flattened out by pressure from a compression fracture of the first 
 lumbar vertebra; the cauda bundles are very well preserved, the destroyed 
 conus is replaced by gliar and fibrous scar tissue. The preparation is taken 
 27 
 
4 i8 DISEASES OF THE SPINAL CORD. 
 
 from a patient, who by falling from a height of 10 m. sustained a fracture of 
 his first lumbar vertebra. 
 
 Occasionally, after a concussion, the typical symptoms of a conus affection 
 appear, though it is impossible to discover the presence of a deformity in the 
 spinal column. A possible explanation for such cases might lie in the fact 
 that there had been no fracture, but merely a momentary elastic displacement 
 of the upper lumbar spinal column, which, however, sufficed to crush the 
 conus. On the other hand, the following hypothesis has been advanced by 
 Fischler: The fall is followed by excessive forward flexion of the trunk, which 
 in turn, causes violent bending in the spinal cord, so that, at the place where 
 the caudal roots enter the conus, there may be lacerations and secondary 
 hemorrhages into the cord. 
 
 Cicatricial tissue. 
 
 Fibre of the cauda 
 equina. 
 
 Fibre of the 
 cauda equina. 
 
 Dura mater. 
 
 Glial substance of the 4th sacral. 
 Fig. 99. — -Cross-section through the dural sac after crushing of the 1st lumbar vertebra. 
 
 Besides traumatism, the other causes of disease play but a slight part. 
 Now and again the symptoms of an affection of the conus develop spontane- 
 ously, and with comparative rapidity, so that the existence of a myelitis 
 must be suspected. Only rarely do tumors develop in the conus itself; on 
 the other hand, it occurs more frequently that the conus is compressed by 
 tumors starting in the cauda equina or the vertebrae, by pachymeningitis, or . 
 by tuberculous processes. The literature does not as yet furnish cases of 
 syringomyelia, poliomyelitis, sclerosis multiplex of the conus medullaris, 
 which were positively identified as such by postmortem examinations. 
 
 The clinical phenomena of conus diseases are limited to an anaesthesia 
 over the sacrum, anus, perineum, in the genitalia, and to a disturbance in 
 the functions Of the rectum, the bladder and the sexual organs; nearly always 
 there appears, as has been already stated, retention of urine and feces. 
 After ischuria paradoxa has persisted for some weeks, or has been combated 
 by catheterization, frequent spontaneous evacuations of urine occur. At a 
 certain degree of fulness in the bladder, automatic evacuation is induced, 
 Shortly before, the patients usually have a certain indefinite sensation in the 
 
SYMPTOMATOLOGY OF DISEASE OF THE CONUS 419 
 
 abdominal region, so that, if a receptacle is at hand, they may avoid getting 
 soiled by the urine. But voluntary excitation or suppression of micturition 
 is and remains impossible. Closure of the sphincter is, however, not so 
 secure as in the healthy, so that during coughing, sneezing, or more violent 
 movements, urine generally escapes. Retention of feces, on the other hand, 
 remains permanently; usually, there is evacuation only after the administra- 
 tion of a purgative. After taking the purgatives, the patients must remain 
 for hours on the toilet, because they have no sensation of the oncoming 
 defecation, and they can not hold it back even for a moment because of the 
 paralysis of the external sphincter ani. Since the patients are not able to 
 retain rectal injections, the constipation can not be combated by enemata. 
 The paralysis of the sphincter ani externus is also evinced by the fact that 
 the anal reflex is always lost in affections of the conus. The sexual functions, 
 immediately after an injury to the conus, and as long as severe general 
 phenomena like ischuria or acute cystitis persist, are entirely absent. 
 Later on, in spite of the disjunction of the lower section of the spinal cord, 
 penile erection may occur and evacuation of the semen into the urethra, 
 though, as a rule, the sensory impressions and orgasm are absent. From the 
 urethra, the semen dribbles but slowly; hence it is always possible that such 
 patients may have fructifying cohabitation. 
 
 In pure affections of the conus, i. e., if the pathologic-anatomical process 
 is restricted to the lowest three sacral and the coccygeal segment, there are 
 no disturbances in sensation or phenomena of paralysis in the lower extrem- 
 ities. Locomotion therefore is in no way interfered with. Very frequently, 
 however, the upper sacral cord, or even the lowest lumbar cord, are simul- 
 taneously affected, and then there are added to the bladder and rectal dis- 
 turbances, paralyses of the peronei and flattening and functional loss of the 
 glutaeus maximus and of the flexors of the knee; the loss of sensation about 
 the anus, of the perineum and the genitalia, are supplemented by the anaes- 
 thetic zones, represented in Figs. 92-98. 
 
 After traumatic lesions of the conus, the morbid changes in the cord, 
 such as hemorrhage, serous infiltration, edema, or reactive inflammation 
 extend nearly always to the middle of the lumbar enlargement. In time, 
 however, the reparable disturbances, and together with them, the phenomena 
 of paralysis and the loss of sensation disappear in the lower extremities, and 
 only those symptoms remain which were excited by crushing the substance 
 of the cord. 
 
 Minor denominates as the epiconus that part of the lumbar enlargement 
 which is situated immediately above the conus and corresponds to the 
 5th lumbar and the 1st and 2nd sacral segments. Disease pictures are 
 described by this author, in which the paralysis is confined to the realms of 
 the peronei and glutaei, so that even the knee reflexes, on the one hand, and 
 the control over the sphincters of the bladder and rectum, on the other, are 
 
4 2o DISEASES OF THE SPINAL CORD 
 
 retained. Such a symptom-complex is nearly always caused by acute 
 poliomyelitis. 
 
 In transverse diseases in the lumbar cord, caused, as they may be, by 
 inflammatory or traumatic myelitis, or by tumorous formations, there results, 
 from the interruption in conduction, also paralysis of the muscular groups 
 innervated by the sacral plexus. But here the type of paralysis will be spastic 
 (ankle clonus, retention of the Achilles reflex) whereas in the realm of the 
 lumbar plexus, degenerative, i. e., atrophic paralyses may be demonstrated 
 (absence of the patellar tendon reflex) . 
 
 If the motor disturbances predominate in the diseases of the conus, the 
 sensory irritation phenomena are characteristic of the caudal diseases. 
 Scarcely any other disease is associated with such painful conditions, as the 
 gradual compression of the bundle of the cauda equina, as it results from 
 tumors. The pains are referred to the anus, the perineum, and the 
 genitalia, and frequently radiate to the legs proper and the peroneal realm. 
 In advanced cases, the corresponding cutaneous areas are insensitive, as a 
 result of the break in conduction in the roots of the cauda, but nevertheless 
 there are still pains in this region, and the condition is then spoken of as 
 "anaesthesia dolorosa." 
 
 The disturbances caused by caudal diseases appear gradually, especially 
 when they are caused by tumors. At first, there are pains only with certain 
 movements of the body, or by pressure on a vertebra; later they appear 
 spontaneously, become almost continuous, and are increased to unbearable 
 violence. 
 
 Although the cauda equina contains, in its upper part, roots which come 
 from the lumbar cord, nevertheless, the pains in compression of the cauda 
 equina are nearly always restricted to the realm of the sacral roots. This 
 fact, taught us by experience, is not easily explained, since it is only natural 
 to suppose, that in a space-contracting process in the canal of the lumbar 
 spinal column the roots, situated at the outer side would be affected 
 likewise. 
 
 In the cauda equina, as was stated above, the sensory and motor tracts are 
 still separated. Thus it is comprehensible, that sensory irritation phenom- 
 ena may exist without motor disturbances. Pareses and paralyses develop 
 much later, if at all. The paralyses are flaccid and usually not strictly 
 symmetrical, i. e., more pronounced on one side than on the other. The 
 disturbances in bladder and rectum caused by caudal affections are in no 
 wise different from those which appear in connection with conus diseases, 
 except, possibly, that their onset is more gradual; before ischuria supervenes 
 the evacuation of the urine is made very difficult for a long time. 
 
 As cause of their origin, traumatism in lesions of the cauda equina plays 
 not nearly so important a part as in those of the conus. In the first place, 
 the lower lumbar vertebras and the os sacrum, are not so frequently exposed to 
 
DIAGNOSIS OF DISEASE OF THE CON US AND CAUDA 421 
 
 fractures as the first lumbar vertebra, which is especially endangered in falls 
 on the legs, or on the buttocks; then the tougher nerve roots, which are more 
 capable of resistance, evade injury in compression of the spinal cord more 
 easily than can the tender substance of the sacral cord. Very often, however, 
 tumors cause injuries of the caudal roots. All sorts of growths, like neuroma, 
 lymphangioma, fibromyxoma, enchondroma, exostoses, cysticerci, etc., may 
 develop within the lower spinal canal, inside and outside of the dural sac. 
 But especially frequently metastases lead to compression of the caudal fibres 
 and above all carcinomata and sarcomata show an inclination to second- 
 ary tumor formation in the lumbar spinal column, and in the os sacrum. 
 On the other hand, tubercular and gummatous processes occur far more 
 rarely in the lower part of the vertebral column than in the dorsal or cervical 
 parts. 
 
 It is to be remembered that tumors outside of the spinal canal may also lead 
 to injury of the nerve tracts here concerned. A tumor located in the pelvis, 
 and attached to the sacrum will, as it grows, attack the fibres which pass to 
 the bladder, the genitalia, and the rectum. Such disturbances assume the 
 character of plexus paralyses. For the nerve tracts are not usually affected 
 by the injury, at a place where the bundles are already leaving the plexus to 
 separate into single nerves, but are injured immediately after they leave the 
 spinal column, that is, in the plexus, or before they have joined to form it. 
 
 Finally it must be mentioned that diseases of the peripheral nerves present 
 similar clinical phenomena as affections of the plexus or cauda. Frequently, 
 cases of sciatica, or of coccygodynia can scarcely be distinguished from the 
 initial difficulties caused by compression of the fibres of the cauda equina, 
 or of the sacral and coccygeal plexuses. 
 
 From the facts here presented, one may appreciate what difficulties 
 differential diagnosis may present, in cases in which one must decide 
 whether the lower portion of the spinal cord itself, or its surrounding and 
 outgoing roots, in their long course, or, finally the plexus and its peripheral 
 processes are injured. But differential diagnosis has not only theoretical 
 interest, but is of great practical importance, also. Thus, the prognosis 
 changes considerably, as the location of the seat of the disease varies. In a 
 sciatica, the prognosis is usually favorable; if the same subjective disturbances 
 are caused by the formation of a tumor in the lower spinal canal, one must 
 count upon an aggravation of the disease. Diseases of the lower spinal cord 
 itself usually run their course rapidly, but their resulting phenomena remain 
 stationary, after the reactive phenomena have disappeared, i. e., improvement 
 and cure are excluded, but neither is an aggravation of the disease to be 
 feared, unless complications appear through decubitus, or through cystitis 
 and pyelitis. In our discussion of differential diagnosis, we must emphasize 
 again the fact, that in diseases of the cord itself, the motor phenomena of 
 functional loss predominate, that these are usually symmetrical and exhibit a 
 
422 DISEASES OF THE SPINAL CORD 
 
 segmentary arrangement. The sensory disturbances, in diseases of the 
 conus, are restricted to loss of sensation; the anesthesia is frequently dissoci- 
 ated, i. e., includes only certain qualities of sensation, such as pain and 
 temperature. The Brown-Sequard symptom complex points definitely to a 
 localization of the disease in the lumbar enlargement. Furthermore, 
 the sensory irritation symptoms are lacking in conus diseases. These, 
 however, predominate in the clinical picture, when a caudal affection is con- 
 cerned. If the pains are permanently unilateral, one must consider whether 
 a plexus disease, or a neuralgia of a peripheral nerve, f. i., ischialgia, is not 
 present. Sensitiveness of the musculature to pressure speaks for neuritis; 
 in the latter case, the paralyses are usually definitely located in the area of 
 one or more peripheral nerves. The disturbances in the bladder and rectum 
 can not be used in a differential diagnosis between "conus" and "cauda." 
 Only this one fact points to a caudal affection, if together with ischuria, 
 and retentio faecium, violent pains radiate to the perineum. In diseases of 
 the peripheral nerves, as in coccygodynia, in sciatica, or polyneuritis, dis- 
 turbances in evacuation of urine and feces are always lacking. 
 
 In typical cases, the differential diagnosis between diseases of the cauda 
 and conus presents no difficulties too great to be overcome. But if a caudal 
 lesion had been diagnosed, then the difficult decision must be made, at 
 what height of the cauda the lesion is to be sought. The rule may be estab- 
 lished here, as in diseases of the spinal cord, to look for the disturbance 
 at as high a point as possible. The nerves, which subserve the bladder and 
 rectum, may be injured throughout their entire course through the cauda, 
 and it has been shown several times, that in diseases of the cauda located 
 high, these nerves situated in the middle and passing out farthest down, 
 usually suffer -first. . The lesion of the cauda must at least be localized at a 
 height, which corresponds to the exit of the uppermost roots, in which 
 irritation phenomena are still demonstrable. 
 
 If a disease of the cauda equina and the conus medullaris are synchro- 
 nously present, a more exact diagnosis will usually be impossible. 
 
 Treatment is absolutely impotent when facing myelitic processes in the 
 lower division of the spinal cord, whether they be of inflammatory or trau- 
 matic character, whether they be recent or partly healed. Likewise, when 
 a tumor has caused pressure atrophy of the cord, no result is attained by 
 its removal. 
 
 Conditions in the cauda equina are different. There, by extirpation 
 of the compressing tumor, an amelioration of troubles, indeed, under 
 certain conditions, complete recovery may be effected. The motor roots 
 of the cauda have their trophic centre in the spinal cord, and the sensory, 
 in the spinal ganglia, and thence, a restoration of fibres, and at the same 
 time, of the functions may result. The operative technique also is not excep- 
 tionally difficult. But permanent results are opposed by the fact discussed 
 
TREATMENT OF DISEASES OF THE CON US AND CAUDA 423 
 
 above, that in the lower spinal canal, we rarely have to deal with isolated 
 tumors; on the contrary, it is usually a matter of metastases. Operation is 
 absolutely hopeless, if tumor masses penetrate the vertebral bones. Natu- 
 rally, with the first symptoms of a caudal compression, treatment with 
 iodide of potassium and mercury will always be instituted at once, assuming 
 the possibility of a gummatous change or a pachymeningitis specifica of the 
 lower dural sac being present. 
 
IV 
 THE MYOPATHIES 
 
 WITHOUT DEMONSTRABLE CHANGE IN THE NERVOUS SYSTEM 
 
 BY 
 
 HANS CURSCHMANN (Mainz) 
 
 Under the term myopathies, we can group together a series of disease 
 pictures, all of which are of rather rare occurrence : dystrophia musculorum 
 progressiva (Erb), myotonia congenita (Thomsen), myasthenia pseudo- 
 paralytica and lastly a disease, that has been known only for a few years — ■ 
 congenital myatonia (Oppenheim). All four are severe afflictions of the 
 function of movement; some forms are accompanied by trophic disturbances 
 of the musculature (dystrophy, amyotrophic myotonia), others run their 
 course without any macroscopic changes in it (myasthenia, myotonia con- 
 genita, myatonia (Oppenheim). Their disturbances in movement vary con- 
 siderably ; in part they are directly antagonistic (myotonia and myasthenia) . 
 In one respect, however, all four diseases are alike: in the macroscopic 
 and microscopic intactness of the nervous system, on the one hand, and in the 
 well-characterized macroscopic, or, as is mostly the case, only microscopic 
 changes in the musculature. It has been attempted, also, to include the first 
 three diseases under one pathogenetic hypothesis (Lundborg) without, how- 
 ever, being able to prove their common parathyreogenic origin. 
 
 i. DYSTROPHIA MUSCULORUM PROGRESSIVA (ERB) 
 
 From the confusion of the various forms of muscular wasting that still 
 reigned in the time of Friedreich, Duchenne, Charcot and others, ErVs search- 
 ing hand has extricated, under the above name, a definite group of pure 
 myopathic atrophies and pseudo-hypertrophies. Even though the usual 
 division into various sub-forms (infantile, pseudo-hypertrophic form, 
 juvenile form, etc.), is to be retained in these diseases, the fact must never- 
 theless be emphasized, that transitional forms occur quite as frequently, as 
 the pure and separate forms, and this always brings the unity of Erb's 
 disease picture to clear recognition. One may therefore well dispense with 
 distinctions between still more separate forms (Leyden-Moebius, Duchenne- 
 Dejerine, etc.). 
 
 Definition. — Erb's dystrophy embraces forms with the following peculi- 
 arities: i. Beginning of the disease in childhood and youth (from earliest 
 
 424 
 
PROGRESSIVE MUSCULAR DYSTROPHY 
 
 425 
 
 infancy to the end of the second decade, very rarely in the thirties and forties) ; 
 2. very frequent (but by no means constant) familial appearance in such a 
 manner that the disease is usually found in several brothers and sisters, but 
 there is but rarely transmission from ancestors to descendants; 3. atrophies 
 and pseudo-hypertrophies regularly attack definite groups of muscles, the 
 atrophies usually definite muscles of the chest, shoulder-girdle, back, arms, 
 buttocks and thighs, often, too, 
 those of the face, while the distal 
 parts of the limbs are nearly always 
 spared to the elbow and to the 
 knee (in striking contrast to spinal 
 muscular atrophy) ; the pseudo-hy- 
 pertrophy prefers definite muscles 
 of the arm and above all, the mus- 
 cles of the calf of the leg; 4. the 
 clinical signs of degenerative atrophy 
 (fibrillary tremors, electrical and 
 mechanical reactions of degenera- 
 tion) are nearly always absent; 5. 
 the following other symptoms of 
 functional loss of the central and per- 
 ipheral neuron are always absent, 
 also: participation of the sensory 
 cerebral nerves, reflex augmenta- 
 tion (Babinski) with spastic con- 
 tractures, changes in sensibility 
 and in the activities of the sphinc- 
 ters; 6. anatomical changes are found 
 regularly in the muscular apparatus, 
 but in pure forms of the disease 
 never in the nervous system. 
 
 The infantile atrophic form 
 of dystrophy is relatively the most 
 frequent; its course may run with 
 and more rarely without involve- 
 ment of the face; pseudo-hyper- 
 trophies are scarcely ever absent. 
 The disease begins (it is more fre- 
 quent in boys than in girls) insidiously and very slowly in early childhood 
 between the third and fifth years, after the patients have already learned 
 to walk in the normal way. The first things the parents notice are: a 
 somewhat slower, waddling and rocking gait, difficulty in running, jump- 
 ing and especially in rising from a recumbent or bent-over position. Simul- 
 
 FiG. 100. — Infantile atrophic form of dys- 
 trophy with participation of the face. {After 
 Heinr. Curschmann.) 
 
426 THE MYOPATHIES 
 
 taneously, a lordosis of the spinal column develops. As a rule, disturbances 
 in the motility of the upper extremities (in raising the arms and shoulders) 
 are not perceived till later. At this time paralytic phenomena in certain 
 facial muscles also appear quite frequently. After having existed several 
 years the disease reaches its climax. 
 
 The findings are now usually as follows (Fig. ioo) : The most serious 
 disturbances nearly always affect the musculature of the trunk, pelvis and 
 thigh. The gait is noticeably uncertain, rocking, waddling (because of 
 the weakness of the glutaei medii, Strumpell), the thighs are raised labori- 
 ously and relatively high, the feet fall stamping to the ground (weakness of 
 the muscles of the thigh). In walking and standing a pronounced lordosis 
 of the lumbar spinal column appears, which may attain grotesque degrees, 
 a result of the atrophy of the muscles of the back and pelvis; the abdomen 
 protrudes considerably. The atrophies constantly single out, besides the 
 long muscles of the back, the glutaei, the extensors of the leg proper (quad- 
 riceps), somewhat less frequently, its flexors. The paralysis of the two 
 former causes the well-known phenomenon in rising from a recumbent 
 position (cf . Fig. 102) : first the children by means of their arms raise them- 
 selves high enough to stand on "all fours," then they climb up on themselves, 
 as it were, resting their arms first upon their knees and then upon their thighs. 
 
 Associated with the atrophies, pseudo-hypertrophies are nearly always 
 found in the lower extremities, most constantly in the musculis gastrocnemius; 
 less frequently traces of pseudo-hypertrophy are found in the glutsei and 
 the deltoid. The hypei trophic muscles usually feel more flaccid than 
 normal muscles, often peculiarly doughy, sometimes, however, dense and 
 solid. Their strength is often noimal, somewhat less frequently diminished, 
 very rarely increased. 
 
 In the infantile form the muscles of the shoulder-girdle and the arms are 
 for a long time less severely affected than those of the trunk and the legs. 
 But atrophies of the m. pectoralis, the m. cucullares, the m. serrati and 
 others are quite constant. The resulting disturbances in motility, which 
 especially affect the raising and use of the arms, when they are lifted above a 
 horizontal position, are discussed in greater detail under the juvenile form 
 of the disease, in which they play a far greater part. The forearm is nearly 
 always spared in the infantile form. 
 
 The face is attacked by the atrophy and paresis at an early period. 
 First is noticed the weakness of the orbicular muscles of the eyes and the 
 mouth; the closure of the eyes is performed incompletely and lacks power; 
 the mouth can not be pursed. As a result of atrophy of its muscles, the 
 upper lip protrudes abnormally; there develops a suggestion of " tapir-snout." 
 Later the atrophy extends — not always symmetrically — over the musculature 
 of the cheeks and forehead, so that finally the myopathic mask-face appears. 
 The tongue and pharynx, as well as the muscles of mastication — in contrast 
 
PSEUDOMUSCULAR HYPERTROPHY 
 
 427 
 
 to bulbar paralyses of other genesis — nearly always remain intact; likewise 
 the external and internal muscles of the eye. 
 
 It has already been stated that the sensory cerebral nerves, sensibility 
 and sphincters remain normal. Likewise, psychic anomalies belong to 
 the exceptions; on the contrary, the children, as a rule, are intellectually 
 alert and clever. 
 
 A rarer modification of infantile dystrophy is pseudo-hypertrophy 
 of the muscles, described first by Griesinger, later by Duchenne and others. 
 It also begins in earliest childhood, between the third and sixth years; its 
 
 Fig. ioi. — Four children of the same parents with infantile muscular dystrophy. The two 
 older children show the atrophic, the two younger boys, the almost pure hypertrophic form. 
 {After Heinr. Curschmann.) 
 
 appearance is very insidious, since the general stoutness of the body does 
 not, at first, impress the parents, as the sign of a disease. 
 
 In typical cases of this disease the pseudo-hypertrophies overshadow 
 the atrophies decidedly. The muscles of the calves and thighs, often also 
 of the buttocks, stand out with enormous volume; in like manner, the muscles 
 of the shoulder and arm (especially the m. triceps) become hypertrophic. 
 At the same time the abdomen is protruded, because of the existing lordosis, 
 and seems abnormally developed. The external picture might resemble 
 that of a caricature of a youthful Hercules. 
 
428 THE MYOPATHIES 
 
 At the same time, the consistency of the hypertrophied muscles is often 
 very soft and spongy, but occasionally, however, as in genuine hypertrophies, 
 solid. Correspondingly, the functioning power of these muscles is, for the 
 most part, relatively reduced; but we have also seen cases in which hyper- 
 trophied muscles were abnormally strong. This is true especially of the 
 musculature of the calf, the endurance and strength of which made one of 
 my little patients, a virtuoso in "toe-running." 
 
 Beside the pseudo-hypertrophies, atrophies also are nearly always 
 discovered upon closer examination; they attack preferentially the long 
 muscles of the back, and not infrequently also the glutaei. The weakness 
 of the pseudo-hypertrophic and atrophic muscles of the trunk and the legs 
 now cause disturbances in posture (especially in standing and walking) and 
 in the gait itself, which closely resemble those of the atrophic dystrophies; 
 pronounced lumbar lordosis and waddling, rocking outward walking. 
 The ability of the patient to raise himself from the ground is always dis- 
 turbed. The weakness of the arm and shoulder muscles appears less 
 distinctly in the pure pseudo-hypertrophic forms, though it is never alto- 
 gether absent. Atrophy and paresis of the musculature of the face are 
 said not to occur in these forms. 
 
 Concerning the occurrence and course of pseudo-hypertrophy the fact 
 must be emphasized, that not infrequently it is found in families, in which the 
 older children suffer from atrophic dystrophy, as the reproduced dystrophic 
 brothers from the Leipsic clinic show in a classical way (Fig. 101). In 
 such cases the pseudo-hypertrophy usually passes over into the atrophic 
 form with involvement of the face. In other instances, also, transitions 
 between the two forms frequently occur. 
 
 The juvenile form of dystrophy, which is almost as common as the 
 infantile atrophic form, does not show familial appearance so frequently 
 as the forms just described. Sporadic cases are certainly no rarities. The 
 disease attacks, as Erb, Strumpell and I have observed, the female sex 
 relatively more frequently than do the infantile forms. 
 
 The onset in the juvenile form occurs about the middle of the second 
 decade and is usually most insidious, even though more rapid progress 
 has supposedly been observed after traumatism. 
 
 As characteristic distinctive signs from the forms discussed heretofore, 
 we must mention first and foremost: the prevalence and the early beginning 
 of the disturbances in the shoulder- girdle and the upper extremities and the 
 falling into the background of the pseudo -hyper trophy in the juvenile form. 
 It is not improbable that the former characteristic, lacking in the infantile 
 form, is to be explained by the considerably increased demands that are 
 being made upon the muscles of the arms and shoulders (in the sense of the 
 "using up" theory of L. Edinger). 
 
 The entire type of the patients differs from that of the infantile form by 
 
PROGRESSIVE MUSCULAR DYSTROPHY 
 
 429 
 
 FlG. 102. — a-e. Rising from the ground in dystro- 
 phia musculorum. {After Heinr. Curschmann.) 
 
 Fig. 102, b. 
 
 Fig. 102, c. 
 
 Fig. 102, d. 
 
 Fig. 102, e. 
 
43° THE MYOPATHIES 
 
 the pronounced changes in the form of the shoulders, thorax and arms 
 (Fig. 103). Owing to the paralyses of the m. cucullares, of the muscles that 
 fix the scapula and in part of the m. pectorales, the shoulders sink far down- 
 wards and frequently not only downwards but forwards as well. From these 
 "fallen" shoulders, arms hang often thin as broom-sticks, and at their top 
 lies a deltoideus of almost normal volume which has also slipped downwards 
 and forearms, which, by atrophy of the supinator longus and occasionally 
 of other muscles, have lost their oval contour and acquired an approximately 
 round one. Through the overhanging of the shoulders and the atrophy 
 of the pectoral muscles, which causes the breast-arm furrow to run upwards 
 instead of downwards, there appears a sunken breast form, the "thorax en 
 bateau," occasionally an actual funnel-shaped thorax. Especially striking 
 are the changes in form and disturbances in function, which are produced 
 through the defects of the scapular muscles. Through paralysis of the ser- 
 ratus. the scapulae protrude like wings, not only while at rest, but especially 
 when lifting; in addition, through the disappearance of the m. cucullares, 
 the shoulder-blades are in an abnormally low position and far from the mid- 
 dle line. If the attempt is made to raise the patient by or under the upper 
 arms, this fails, as the shoulders are "loose, " cannot be fixed, and during the 
 attempt are pulled limply upwards above the ears. If the patient is ordered 
 to press the arm, that has been raised to a horizontal position, downwards 
 against a resistance, there also appears a characteristic phenomenon: 
 because of the disappearance of the m. latissimus dorsi the attempt men- 
 tioned fails and the unfixed shoulder-blades are now partly actively, partly 
 passively and indirectly pushed back and down (by the resistance). 
 
 Beside this the appearance of the juvenile dystrophic patient is charac- 
 terized by dorso-lumbar lordosis and the secondarily protuberant abdomen 
 (Fig. 104). The gait also, because of the atrophy of the glutaei and some 
 of the muscles of the thigh, is waddling and rocking, even though fre- 
 quently less so than in the infantile forms. 
 
 To recapitulate briefly the following muscles are usually atrophic: 
 cucullaris, pectoralis major and minor, serratus anticus, latissimus dorsi, the 
 long muscles of the back and loins, somewhat less regularly and later, rhom- 
 boidei and triceps, as well as the supinator longus. The following are, as a 
 rule, spared: sternocleido-mastoideus, levator anguli scapulas, coracobrachia- 
 lis, teres major and minor, supra- and infra-spinatus, the flexors and exten- 
 sors of the forearm and the small muscles of the hand. In the lower extrem- 
 ities the following are nearly always atrophic: m. quadriceps and the glutaei, 
 somewhat less frequently the flexors of the thigh (m. biceps, semitendinosus, 
 etc.), and the adductors, very rarely the m. tibialis anticus, the peronei and 
 the extensors of the toes. The calf musculature (in respect to function) 
 mostly remains normal, but very frequently it shows signs of pseudo-hyper- 
 
MORBID ANATOMY OF MUSCULAR DYSTROPHY 
 
 431 
 
 trophy. In the upper half of the body the m. supra- and infra-spinati and 
 the m. triceps are not infrequently hypertrophic. 
 
 The mechanical excitability of the atrophic muscles (general and idio- 
 muscular) shows a simple diminution, or it may be lost. Likewise, electrical 
 tests show only a quantitative change up to the loss of the reaction; in a few 
 cases, a suggestion of R.D. is said to have been observed. The tendon 
 reflexes usually disappear in the regions that are affected; frequently, also, 
 I have found general abolishment of 
 the tendon and periosteal reflexes. 
 The cutaneous reflexes remain intact. 
 
 Anatomy. — Brain, spinal cord, 
 and peripheral nervous system have 
 (in pure cases) been found normal 
 in all cases examined thus far, even 
 by the most delicate histological 
 methods. 
 
 The muscles, on the other hand, 
 always show striking changes: in 
 the atrophic muscles numerous mus- 
 cular fasciculi are completely de- 
 stroyed and their spaces filled up by 
 the infiltration of fat; the greater 
 number of the individual muscular 
 fibres are markedly atrophic, but a 
 part of them are also of abnormally 
 large circumference; the muscle nu- 
 clei are increased, the muscular fibres 
 themselves usually show fissures and 
 vacuoles, beside proliferation of the 
 interstitial connective tissue. While 
 in pseudo-hypertrophic muscles, al- 
 
 Fig. 103. — Juvenile form of dystrophy. 
 Atrophy of the face (lips), pronounced atrophy 
 of the m. cucullares, serrati (-wing position of 
 the shoulder-blades) "sunk in shoulders" and 
 atrophy of the muscles of the upper arm. 
 (Heidelberg Medical Clinic.) 
 
 though the atrophy, the fatty degeneration and proliferation predominate, 
 still there are muscles which, owing to an extraordinary increase in volume in 
 the original fibres, are to be considered genuinely hypertrophic. 
 
 ^Etiology. — An actual "exciting" cause of dystrophy is not known. 
 That traumatism could fill this position I consider very doubtful. The 
 disease is to be interpreted rather, as the expression and consequence of a 
 specifically weak congenital predisposition of the muscular system, which 
 succumbs to use, sooner or later, in this typical way. 
 
 Course and Prognosis. — The course is always very chronic; whereas 
 the infantile patients rarely reach adult life, dying, as a rule early, of inter- 
 current diseases; the juvenile form lasts a long time. There are not a few 
 cases in which, dystrophy notwithstanding, the 50th and 60th years was 
 
432 THE MYOPATHIES 
 
 reached. The progress is usually very slow; especially juvenile cases fre- 
 quently show a standstill of the disease for ten years and more. The prog- 
 nosis quoad valetudinem is always bad. I know of only one case through 
 (verbal) communication of Erb, in which dystrophy, so diagnosed by him, in 
 a little girl, resulted in recovery. 
 
 Treatment is correspondingly almost always powerless, even though 
 mild hydriatic, gymnastic and electric procedures are occasionally supposed 
 to have caused improvement (in connection with the rest of the hospital 
 treatment). The organ- therapeutic preparations have proved to be ineffec- 
 tual. In some cases (especially in the infrequent complications with con- 
 tractures) orthopedic surgical interference may be indicated. Supporting 
 corsets, on the whole, have not proved very serviceable. 
 
 Differential diagnosis must consider in the infantile form, the similarity 
 — which, however, is quite superficial — to diplegia infantum; this, however, 
 may be immediately distinguished by its pyramidal tract symptoms. Polio- 
 myelitis anterior could but rarely be confused with dystrophy. In the pseudo- 
 hypertrophic form a possible confusion with myotonia congenita must be 
 thought of; however, the characteristic disturbance in motility and muscular 
 reaction of the latter should at any rate easily establish the diagnosis. Dif- 
 ferentiation from Thomsen's disease with muscular wasting may be 
 more difficult, since the myoatrophy here occasionally shows the typical 
 distribution of dystrophy. But even in such cases the existence of 
 myotonic reaction of individual muscles will decide the differential diagnosis. 
 Furthermore, the rare cases of actual genuine muscular hypertrophy (either 
 congenital or acquired) may appear in differential diagnostic competition, 
 and also the very rare cases of hypertrophy following thrombosis, neuritis 
 and in spastic spinal paralysis. A consideration of the manner of the dis- 
 tribution of the muscular dystrophy will make the diagnosis in all these cases 
 easy. The not uncommon cases of congenital defects of single muscles 
 (especially of the trapezius and pectoral muscles) may also arouse a slight 
 suspicion of dystrophy. 
 
 Most important in differential diagnosis are, naturally, the other forms of 
 muscular wasting: the spinal, the neural (Marie- Hoffmann' 's) amyotrophy, 
 syringomyelia, the muscular atrophies in polyneuritis and the already men- 
 tioned myotonia amyotrophica. The first two forms mentioned are imme- 
 diately distinguished from dystrophy, by the fact that they show an opposite 
 arrangement of atrophies : the preference for the distal ends of the extremities, 
 whereas dystrophy shows preference for the proximal parts. In rare cases, 
 of course, one must reckon with the beginning of the spinal affection in the 
 muscles of the back. The same thing holds true of the polyneuritic forms of 
 muscular wasting. Syringomyelia may indeed, even if rarely, exhibit its 
 earliest atrophy in the proximal arm muscles and the shoulder- girdle; but 
 in this case the disturbance in sensation will immediately decide the differ- 
 
RARE FORMS OF MUSCULAR DYSTROPHY 
 
 433 
 
 ential diagnosis. But above all, in all the before- mentioned conditions, the 
 appearance of fibrillary tremors and of the electrical reaction of degeneration 
 make the diagnosis considerably easier. 
 
 One must mention, finally, that occasionally inflammatory diseases of 
 
 Fig. 
 
 104, a and b. — Lordosis and typical gait in juvenile dystrophy. {After Heinr. 
 
 Curschmann.) 
 
 the muscles with atrophy (Oppenheim) and certain diseases of the bones 
 (e. g., rachitis tarda (author)) may remind superficially of dystrophy. 
 
 Rare Forms and Complications. — Here, first and foremost, the form 
 observed by /. Hoffmann, with bulbar paralytic localization of dystrophy, 
 must be mentioned. Also by Oppenheim and others, the (very rare) par- 
 28 
 
434 THE MYOPATHIES 
 
 ticipation of the muscles of the tongue and pharynx has been established; 
 likewise the spread of the disturbance to the muscles of the eye. That the 
 dystrophy may begin, quite exceptionally, in the legs proper, in the hands 
 and forearms, was proved by the reports of /. Hoffmann, Schultze and Erb. 
 Furthermore those cases are remarkable, in which the trophic changes are 
 not confined to the musculature, but extend also to the bones {Friedreich, E. 
 Schultze, Schlippe and others) ; some of these cases show in addition extensive 
 early contractures in the muscles. Anomalies in the formation of the skull 
 and thorax have also been observed. 
 
 We must mention, furthermore, that dystrophy has been observed in 
 combination with all sorts of organic and functional nervous disease (for 
 instance, with tabes, epilepsy, hysteria or psychoses). It is probable from 
 late observations, that there are forms of dystrophy, which never attain 
 complete development (formes frustes). 
 
 2. MYOTONIA CONGENITA, THOMSEN'S DISEASE] 
 
 This rare disease, described first by Ch. Bell and Leyden, but in the year 
 1876, actually "discovered" by the Silesian physician Thomsen and classic- 
 ally worked up by Erb must still be regarded — like progressive dystrophia 
 musculorum — so long as no stringent proof to the contrary be adduced, as a 
 primary myopathy, as a severe intention neurosis of the muscles. 
 
 The disease, which in many cases, especially in the family of the dis- 
 coverer, appears mostly familial, with a preference for males, begins in the 
 typical cases usually in early youth, and not seldom, too, about the time of 
 puberty. It usually progresses very slowly, rapidly only upon the occurrence 
 of unusually strong physical exertion (military service) and is then occasion- 
 ally heightened to an acute onset. The first symptoms are nearly always a 
 certain stiffness and difficulty in walking, rising, lifting, etc., after a period 
 of rest, especially in the morning. This stiffness diminishes upon the repeti- 
 tion of the movement concerned and finally disappears altogether. 
 
 The typical picture of the completely developed myotonia, is as follows: 
 the young men, who are usually well-nourished and not seldom full-blooded, 
 show an almost athletic development of the muscles of the trunk and the 
 extremities, which contrasts strikingly with the functional weakness of 
 which they complain. At every first attempt at movement in any group of 
 muscles, after they have been rested (especially in the extremities), there 
 is a peculiar tension and stiffness, a veritable spasm of the muscles concerned, 
 which causes a marked slowing of the movement; and this "myotonus" 
 usually reaches its height at the second movement (not the first) ; for instance, 
 if the first adduction of the thumb succeeds with relative speed, the follow- 
 ing abduction may meet the strongest resistance. This difference between 
 the first and second movement is exhibited very distinctly in rapidly 
 executed powerful movements. 
 
MYOTONIA CONGENITA 435 
 
 Upon further repetition of the movement concerned, it becomes freer 
 and freer, the tension is soon relaxed and the movement becomes absolutely 
 normal. The disturbance in the gait appears in a most characteristic form: 
 at the beginning slow, short, dragging steps (veritably spastic) are taken 
 with great exertion- — after 8-10 m. the walk is freer and becomes completely 
 normal, quick and elastic at the end of a walk of about 15 m. (for instance, 
 a walk through a hospital ward). The disturbance becomes very serious, 
 if the patient is suddenly forced to make a brusque and quick movement; 
 for instance, to avoid an obstacle that appears in his path, or the like; the 
 legs may then become as stiff as sticks, the patient falls down like a log, 
 and can only rise with difficulty. The myotonic disturbance also, is usually 
 increased by excitement and by cold. 
 
 This disturbance may now — in rarer cases — be restricted to definite 
 muscle areas; for instance, to the legs, hands, tongue, etc.; but mostly, it 
 appears generalized, attacking all the muscles of the trunk and limbs, even 
 if not with the same severity. It often disturbs even the motility of the 
 muscles of the face and of mastication, of the muscles of the tongue and of 
 those that close the eyelid, very rarely those of the throat and oesophagus 
 and the external muscles of the eye; it is doubtful whether the musculature 
 of respiration and the heart ever participate. 
 
 The affected muscles do not usually show an increase in tonicity while 
 at rest and during passive movements; the passive movements are completely 
 free, in contrast to the active ones; the same is true of the reflex movements 
 (for instance the plantar reflex). On the other hand, the mechanical and 
 electrical excitability, as Erb first showed, are changed in a most striking way. 
 
 The mechanical excitability is usually perceptibly augmented; every 
 tapping of the muscle, often mere pressure or kneading, produces an abnormal 
 continuation of the local muscular contraction for from 5 to 30 seconds; 
 there arise, according to the location and form of the muscles, either weals 
 or deep grooves and furrows (with special distinctness in the tongue, the 
 m. gastrocnemius, etc.). At the same time, the idiomuscular excitability 
 is very slight; Schiff's waves are also absent. 
 
 The electrical excitability is usually absolutely increased for both kinds 
 of currents. With the usual faradic (direct) stimulation, employing some- 
 what stronger currents, there is occasionally a somewhat slow contraction 
 with long prolongation (2 to 20 seconds or more) ; single opening shocks of 
 any strength whatever cause only normal, quick contraction; with strong 
 stabile faradization, there appear, occasionally, oscillating muscular waves. 
 The contractions caused by (direct) galvanization also are slow, tonic, and 
 show abnormal prolongations; usually only closing contractions (ACC = 
 CCC) appear; in stabile galvanization, occasionally (not always and not in 
 all muscles) rhythmical wave-like contractions from the C to the A. This 
 electric symptom complex is named, according to Erb, " myotonic reaction." 
 
436 THE MYOPATHIES 
 
 The nerve trunks usually do not show any peculiar change in excitability 
 upon mechanical and electrical stimulation; with indirect electric stimulation, 
 therefore, there is no myotonus. 
 
 The sensory functions, the sphincters, the sensibility and the cutaneous 
 reflexes have always been found normal ; the tendon reflexes were occasionally 
 weakened, or abnormally easily wearied, but, as a rule, active. Trophic 
 and vaso-motor disturbances are nearly always absent. The psyche is 
 usually normal — which must be emphasized as against the classification 
 of the disease under the psychoses by Thomsen himself — even though a 
 combination with hysteria and epilepsy has been observed. 
 
 Varieties of Myotonia. — The most important variety is the one accom- 
 panied by different forms of muscular wasting, amyotrophic myotonia, 
 which has been observed rather frequently in recent times — in about 20 
 to 25 cases. In contrast to typical myotonia, it does not appear to begin 
 in early youth, but not until the second to the third decade, to run its course 
 simultaneously with atrophy and pareses and myotonic disturbances. 
 The myotonia may remain more or less generalized, but may also limit 
 itself to only a few muscles (especially to the hands and tongue). The 
 atrophies may be of the type of ErVs dystrophy, now but more rarely, of 
 that of the Duchenne-Aran amyotrophy (forearm-peroneal type); atrophy 
 of the muscles of the face and mastication is relatively frequent (spontaneous 
 dislocation of the mandibula), as well as speech disturbances, but ptosis 
 (Fiirnrohr) is very rare. Once inceptive atrophies were proved present in a 
 previously typical case of congenital myotonia (Hoffmann). The myotonic 
 reaction is, of course, modified by the atrophies; occasionally one finds the 
 myasthenic reaction. The tendon reflexes are not rarely entirely absent 
 in the amyotrophic myotonia; sometimes there is mechanical over-excitability 
 of the nerves. The course, because of the atrophies, is more severe, but 
 improvements are possible. 
 
 The combination of syringomyelia with myotonia, which has been 
 observed in some cases, is also to be placed in the domain of amyotrophic 
 myotonia. 
 
 Myotonia acquisita has been described by Talma in cases in connection 
 with traumatisms and acute infections. The prognosis in it seems more 
 favorable than in idiopathic cases. 
 
 Furthermore, in very rare cases, there have been observed instances of 
 intermittent myotonia; myotonia-like neuroses, accompanied by intermittent 
 paralyses (paramyotonia, Eulenburg), of myotonia combined with tetany 
 (Bettmann, v. Voss), of myotonia of nursing infants and of those suffering 
 with gastric dilatation. 
 
 The differential diagnosis of generalized myotonia scarcely ever pre- 
 sents difficulties; at most some cases of pseudo-hypertrophic dystrophia mus- 
 culorum or rare cases of spastic spinal paralysis may display a superficial 
 
MYASTHENIA PSEUDOPARALYTIC A 437 
 
 similarity to it; the correct diagnosis, however, is easily recognized by the 
 demonstration of the typical disappearance of the myotonus after repetition 
 of the movement and by the myotonic reaction. The partial myotonia, for 
 instance, of the forearms, may bear a slight resemblance to the chronic tonic 
 form of tetany and to occupational cramps, without, however, causing 
 serious differential diagnostic difficulties. The pseudo-spastic paresis of 
 hysteria may also— very rarely and only superficially — resemble myotonia. 
 In the amyotrophic form, however, the separation from other forms of 
 muscular wasting, sometimes succeeds only after an exact electrical investi- 
 gation, especially of the non-atrophic musculature. 
 
 Morbid Anatomy. — So far, the central nervous system has been found to 
 be free from all changes. On the other hand, the muscles, according to Erb, 
 show the following changes: enormous hypertrophy of all the fibres, with 
 most profuse proliferation of the nuclei of the sarcolemma, changes in the 
 finer structure (indistinct striation, formation of vacuoles, etc.), besides a 
 slight increase of the interstitial connective tissue and deposit of a granular 
 substance. Schieffer decker recently proved that the nuclear increase is only 
 relative and that in the sarcoplasma, peculiar grains are found, when a 
 certain fixing agent is employed. 
 
 The actual nature of the disease is not yet clear; the heretofore assumed 
 purely myogenous theory is not wholly satisfactory, in spite of Schieffer- 
 decker's findings. Several symptoms point perhaps to a central (cerebral?) 
 source of the disease. 
 
 Correspondingly, the aetiology of myotonia congenita has not been at 
 all explained; the thought of an auto-intoxication (analogy with veratrin 
 poisoning) has only hypothetical interest. The important role of heredity 
 speaks for a congenital failure in development of some kind. 
 
 The prognosis of typical Thomserts disease is quoad valetudinem 
 always bad, quoad vitam always good. The patients are mostly unsuited to 
 vocations which are physically exhausting. The varieties, especially amyo- 
 trophic myotonia, the myotonia of gastric dilatation and of babies at the 
 breast have a more serious prognosis. 
 
 Treatment is — in typical cases — always fruitless; nerve-stretchings 
 have not proved to be of any value. Careful gymnastics and massage are 
 supposed to be able to induce functional improvements. The main point 
 seems to be prophylaxis with regard to accidents. 
 
 3. MYASTHENIA PSEUDOPARALYTICA 
 
 {Myasthenic Bulbar Paralysis, ErVs Disease) 
 The first communications concerning this peculiar disease, we owe to 
 Erb; later the investigations of Oppenheim, Hoppe, Goldflam, Strumpell, 
 Eisenlohr, Jolly and others have taught us more of the nature of the disease 
 and clinically, at least, they have cleared the subject fully. 
 
438 THE MYOPATHIES 
 
 We must regard the peculiarity of myasthenic paralysis to lie in the fact, 
 that it leads to serious paralyses of the bulbar nervous realms, first and foremost 
 of the muscles of the eyes and the pharynx, furthermore, to paralytic-like weakness 
 of the musculature of the trunk and the extremities, without ever attaining degen- 
 erative atrophies or hypertonic muscular changes, while the macroscopic or 
 microscopic findings in the central nervous system do not show sufficient changes 
 to explain these disturbances. The paralyses in all the regions have, as 
 Gold-flam first showed, especially in the beginning, a clear remittent character; 
 they are the product of a morbidly increased capacity for fatigue. The 
 prognosis is mostly unfavorable; recovery is, on the whole, rare. 
 
 Course and Symptomatology. — The disease attacks individuals who 
 have been well heretofore, between the 20th and the 40th years; women are 
 affected somewhat more frequently than men. Usually very gradually, more 
 rarely rapidly after an acute disease, after traumatism or over-exertion, the 
 patients become afflicted with paralytic phenomena of the muscles of the eye, 
 very often with ptosis and diplopia; at the same time one or the other bulbar 
 function, phonation or deglutition suffers. These phenomena may also be 
 associated with a feeling of weakness in the nape of the neck, the trunk and 
 extremities, even though in the beginning the bulbar symptoms — they are 
 often the only symptoms for years — predominate. All disturbances may 
 then disappear for weeks, months or years, without leaving a trace, so that 
 the disease at the beginning may consist of short exacerbations between long 
 remissions. This relatively harmless initial stage may persist for a long 
 time, up to 22 years {author). Occasionally, however, the course of the 
 disease is more rapid and approaches its climax in weeks and months 
 gradually, without longer periods of improvement. 
 
 It is precisely in the first stage, that this disease shows its characteristic 
 trait, one that distinguishes it totally from all other bulbar symptom-com- 
 plexes, namely, the symptom of paralysis, through pathologically increased 
 fatigue: the muscles of the eye, the muscles of deglutition, which in the morn- 
 ing, after the strengthening rest of the night, perform their functions well or 
 fairly well, fail after a shorter or longer activity, more and more, in the course 
 of the day, until they become paralytic (or more exactly, pseudo-paralytic). 
 A night's rest or some hours of repose during the day suffice to remove the 
 paralysis and the next day the drama of myasthenic fatigue paralysis begins 
 anew. Once the disease reaches its climax or more particularly, if it reaches 
 the final stage, part of the paralyses, however, persist, or show only slight 
 changes according to the condition of rest or fatigue. 
 
 Gradually, often too, suddenly, the climax of the disease is reached: the 
 eye muscles, the muscles of mastication and of deglutition, more rarely 
 those of the facial region, frequently the muscles of the nape of the neck and 
 of the back become almost permanently paretic; the fatigue paralysis of the 
 extremities reaches so high a degree, that the patients become permanently 
 
MYASTHENIA PSEUDOPARALYTICA 
 
 439 
 
 bedridden. Nutrition suffers through the deglutition paralysis; faulty- 
 swallowing appears; finally, too, the musculature of respiration fails (pos- 
 sibly also the muscle of the heart) and the end, prepared by inanition, appears 
 quite spontaneously as the result of respiratory or cardiac paralysis, or 
 suddenly after, an action that strains the heart (stomach pump, different 
 baths) or is more slowly brought about by an aspiration pneumonia. 
 
 Rarely the myasthenia runs its course without actual bulbar paralysis, 
 as pure paresis of the extremities and the trunk (Grund, Rautenberg and 
 others). 
 
 Of the objective symptoms of the completely developed disease the 
 disturbances of the muscles of the eye appear most prominently in the fore- 
 
 FiG. 105. — Myasthenia' with ophthalmoplegia ex- 
 terna on the right side. {After Knoblauch.) 
 
 Fig. 106. — The same patient trying to 
 look upwards. {After Knoblauch.) 
 
 ground; the ptosis, which the patients attempt in vain to counteract by auxil- 
 iary contraction of the muscles of the forehead, the paralyses of the n. 
 abducentes, of the oculomotor muscles and the rest, so that a total ophthal- 
 moplegia externa results. The defects in mobility frequently seem to be of 
 strictly associated nature (Bielschowsky) ; it is characteristic that the sphincter 
 iridis only rarely is involved in fatigue paralysis. Frequently there exists — 
 in incomplete paralysis of the muscles of the eye — diplopia in a degree that 
 varies from morning to night. 
 
 The muscles of mastication become paretic markedly and very early in 
 the disturbance; after a few bites the masseters and temporals give out; 
 swallowing is disturbed by the increasing paralysis of the soft palate, of the 
 muscles of the pharynx and oesophagus; liquid nourishment flows out through 
 
44© 
 
 THE MYOPATHIES 
 
 the nose. The laryngeal muscles, mostly the phonatores, rarely then, 
 postici, fail, the voice becomes hoarse, aphonic. 
 
 The muscles of the lips, the naso-labial muscles (most rarely the m. 
 frontales), finally almost the entire facial region is paralyzed; the patient has 
 a worried, tired, "mask" face. 
 
 At the height of the disease, as we have said, the muscles of the nape of 
 the neck and all the muscles of the trunk show signs of weakness and fatigue; 
 arms and legs, especially in their proximal muscular regions, become tired 
 and paretic after a few movements, after a little lifting and bending, after a 
 few steps. 
 
 Fig. 107. — Forty-five-year-old patient with myasthenia, bilateral ptosis, myopathic mask-face. 
 
 {Author's own observation.) 
 
 At the same time, the paralyzed muscles (in pure cases of the disease) 
 never present atrophies, fibrillary twitchings, electric or mechanical reaction of 
 degeneration. 
 
 On the other hand, one finds in many muscles (but by no means always 
 in those that are permanently paretic and most seriously affected) besides the 
 active overcapacity for fatigue, also the same thing for the faradic current, the 
 myasthenic reaction discovered by Jolly (Mya. R.) (Fig. 108) ; if such a muscle 
 is stimulated by direct tetanization, it reacts at first with normal contractions, 
 gradually the closing contractions lose their intensity and finally fail alto- 
 gether; after the tetanization has been stopped for a short while (minimum 
 ca. 2 sec.) the muscle is again capable of renewed contraction. The myas- 
 thenic reaction does not need to be complete, but it is at least suggested, in 
 
MYASTHENIA PSEUDOPARALYTIC A 
 
 441 
 
 most of the cases. The degree and rapidity with which the Mya. R. is 
 elicited, seems to stand in direct proportion to the former voluntary motor 
 or even electrical fatigue of the muscle. ^ 
 
 It must be mentioned that the Mya. R. 
 has been found also in the atrophic muscles 
 in other nervous diseases, in focal injuries 
 of the brain, in "cerebral muscular atro- 
 phies" (H. Steinert), in amyotrophic myo- 
 tonia, etc. These findings, however, can 
 not cast doubt on the specific nature of the 
 reaction in the myasthenia, which runs its 
 course without atrophies. 
 
 The sensory cerebral nerves (optic, ol- 
 factory, glosso-pharyngeal, auditory, etc.) 
 are always spared by the disease. The sen- 
 sory functions of every other sort also 
 always remain intact, usually, too, the 
 bladder and rectum. 
 
 The tendon reflexes remain mostly nor- 
 mal; in rare cases they are weakened; often, 
 if frequently elicited, they become over- 
 fatigued and disappear. Rarely they may 
 be permanently lost during the period of 
 total paralysis (author's observation). The 
 cutaneous reflexes always remain normal. 
 
 The psyche of the patient is mostly 
 intact, always, too, the higher functions 
 of speech. Compulsory emotions, the crux 
 of the atrophic and pseudo-bulbar para- 
 lytics, seem to be absent. 
 
 Morbid Anatomy. — The brain, spinal 
 cord and efferent nerves have been found 
 altogether intact, in most typical cases, ex- 
 cluding a few in which the changes were 
 insufficient to explain the serious dis- 
 turbances. On the other hand, in numer- 
 ous cases, accumulations of lymphoid cells fe 
 have been found in the affected muscles (Weigert, Flatau, Link, Goldflam, 
 Laqueur, Boldt, Burr, author and others) , which, because of their frequency in 
 the more recent postmortem examinations, possibly have a pathognomonic 
 importance. In one case fatty transformation of the sarcoplasm was found 
 (Marburg). In several cases persistent thymus or thymus tumors and 
 muscle metastases (Weigert) were found. In one case Pel found leucocytosis 
 
 o *- 
 
 bD ^> 
 
 o.S 
 "35 'a 
 o « 
 
 O 
 §| 
 
 tn •« 
 
 «*- 
 
 ta o 
 
 IB 
 
 a. a 
 
 o „ 
 
 §5 
 •BM 
 
 'c3 xi 
 
 "- 1 to 
 
 > o 
 u 
 
 3 
 
442 THE MYOPATHIES 
 
 intra vitam and leucocytic accumulations in the parenchymatous organs. 
 Taking all in all, however, the anatomic findings thus far do not explain fully the 
 severe functional disturbances of myasthenia. Whether the morbid preponder- 
 ance of the "light muscular fibres," recently found by Knoblauch, at the 
 expense of the "red dark ones," in myasthenia and the comparative biolog- 
 ical reasons of the author for the pathogenesis of myasthenia, will acquire 
 importance, is as yet by no means sure. 
 
 Etiology. — The real cause is unknown. Acute infectious diseases, 
 however (influenza, traumata, intoxications, over-exertions, etc.), are 
 credited as excitant factors. The latter factor seems especially plausible 
 in an unpublished case of Erb, that of an old Boer, who was chased on 
 horseback with Dewet by the English for weeks with minimal resting 
 periods. 
 
 Often, however, there is no obvious causal factor to be found. 
 The assumption that a secretory disturbance of the glandulse parathy- 
 reoideae is the cause of myasthenia (Lundborg, Chvostek) has not yet been 
 proven in any way. 
 
 Of great importance, however, is the proof first adduced by Oppenheim, 
 that many myasthenics exhibit a striking congenital inhibition in development 
 and deformities (anomalies in the nervous apparatus, polydactylia in feet and 
 hands — Oppenheim — total aplasia of the genital organs and hyperplasia of 
 the lobes of the lungs — observation of the author) . These findings stamp the 
 patient doubtless as belonging to the congenital hypoplasts, in regard to 
 which Edinger's theory of abnormal "using up" and insufficient reparation 
 in the nervous system, must seem very probable to us, and explicative of 
 many things. 
 
 The complications, by which the disease is rarely accompanied — 
 hysteria, Basedow's disease, dystrophia musculorum, Banti's disease 
 (Mohr) , can be of little assistance in the aetiological research. 
 
 The differential diagnosis may be difficult in the beginning, especially 
 relative to hysteria, but is cleared up by the type of the paralysis of the eye 
 muscles, the disturbance in deglutition, the strictly remittent character of 
 the paralyses and finally the My a. R. Against the diagnosis of atrophic 
 and pseudo-bulbar paralysis we are protected, on the one hand, by observa- 
 tion of the degenerative atrophies, on the other by the disturbances of the 
 lateral pyramidal tracts (spasms, increased reflexes) in those cases. Differ- 
 entiation from a polyneuritic bulbar paralysis is more difficult in the begin- 
 ning; the same difficulty is met with in cerebral syphilis, Landry's paralysis 
 and especially poliencephalomyelitis {Oppenheim). The absence of the 
 quantitative and qualitative electrical changes and the appearance of the 
 Mya. R. will, however, decide the differential diagnosis, even in these cases. 
 The prognosis is usually unfavorable. Although Erb, Goldflam, H. 
 Steinert and others observed recoveries in light cases, most cases after shorter, 
 
MYOTONIA CONGENITA 443 
 
 or, as usually happens, longer duration, end, as has been described above, 
 fatally. 
 
 Treatment can consist only of great conservation of effort, rest and suit- 
 able nourishment; besides tonics may be useful. All severe electric or 
 active and passive gymnastic procedures, which could only increase the 
 exaggerated tendency of the muscles to become fatigued, are contra-indicated. 
 Treatment by baths is to be employed only with attentive control of respira- 
 tion and pulse; better, not at all (Steinert). The stomach-tube, which is 
 occasionally unavoidable, when the swallowing apparatus is totally paralyzed, 
 is to be used only with the greatest caution, since cases of death during its 
 passage have been observed (Oppenheim). 
 
 4. MYOTONIA CONGENITA (Oppenheim) 
 
 This disease, first described by Oppenheim in 1894, since then described 
 in only about ten cases, seems to be nearly always present at birth, though 
 occasionally — in milder degrees — it was noticed first in the 2nd or 3rd year 
 of life. The children thus affected are conspicuous in that they cannot move 
 their limbs, especially the lower extremities, spontaneously: the limbs are 
 completely paralyzed and flaccid, the muscular tonus is markedly reduced, 
 the limbs hang loosely from the joints. In the arms, the disturbance is 
 usually slighter and here the weakness of the movements is more prominent. 
 The motility of the trunk and neck, on the other hand, is scarcely ever dis- 
 turbed. Involvement of the muscles of the head, face, tongue, eyes, larynx 
 and diaphragm has not yet been observed. The sensory cerebral nerves, 
 the sensory functions, the sphincters and the intellectual development, also, 
 seem not to suffer in the disease. 
 
 The muscles, though they feel limp and withered, show no real atrophies 
 and no fibrillary twitchings. The direct and indirect electrical excitability 
 in the paralyzed as well as in the apparently sound muscles is usually 
 reduced, quantitatively to a large degree, frequently even to the point of 
 entire disappearance of the excitability, and a suggestion of the reaction of 
 degeneration has been observed. 
 
 The tendon reflexes are nearly always abolished, not only in the extrem- 
 ities affected, but also in those that are normally movable; the cutaneous 
 reflexes are mostly described as normal. 
 
 Anatomic findings are known so far, only in one case (Spiller): the 
 entire nervous system proved to be intact; the muscles, on the other hand, 
 showed hyaloid changes, looked cloudy and had only indistinct striation; 
 the individual fibrils were abnormally small, the fat containing connective 
 tissue increased. Signs of inflammation and breaking down were lacking. 
 Other authors corroborated these findings in sections of muscle taken from 
 the living body (Reyher and Helmholtz), whereas Bing found normal structure. 
 
4 44 THE MYOPATHIES 
 
 Oppenheim sees the very essence of myatonia in an inborn retarded 
 development of the musculature; the anatomical findings for the most part, 
 seem to speak for this. Bernhardt is rather inclined to assume an injury to 
 the peripheral nerves, somewhat like a generalized polyneuritis of autotoxic 
 or infectious origin (partly on the basis of cases, which were notcongenitally 
 diseased). At any rate, the pathogenesis of the disease is greatly in need 
 of classification by further clinical and anatomical findings, which first and 
 foremost must prove if there is really such a disease entity. 
 
 The differential diagnosis must consider especially, poliomyelitis 
 anterior and the peripherally and spinally induced paralyses intra-partum. 
 The absence of atrophies and disturbances in sensation, the generalized, 
 almost always symmetrical affection of the muscles and the change in 
 electrical excitability, which mostly affects all the muscles, distinguishes, 
 however, myatonia from those diseases. From a — probably exceedingly 
 rare — general polyneuritis of nursing infants, the disease, it seems to me, 
 can not be distinguished. Finally, I desire to earnestly point out, that 
 nervously normal, but somewhat atrophic or rachitic children may show a 
 high degree of hypotonia of the muscles and joints (which, to a certain 
 degree, is physiologically peculiar to early childhood). This likewise 
 furnishes a differential diagnostic source of error. 
 
 The course is always extremely slow. The prognosis, however, is not 
 bad, since in some of the cases, in the course of time, a more or less complete 
 disappearance of the disturbances has been observed. 
 
 Treatment must consist in the use of general tonic means, of careful 
 strychnine medication, massage and the electric current; possibly ortho- 
 pedic procedures may also become necessary. 
 
V 
 DISEASES OF THE BRAIN 
 
 NORMAL AND PATHOLOGICAL PHYSIOLOGY OF 1HE BRAIN x 
 
 BY 
 
 HUGO LIEPMANN (Berlin) 
 
 I. INTRODUCTORY REMARKS ON THE ANATOMY, PHYSIOLOGY, 
 AND PATHOLOGY OF THE BRAIN 
 
 i. The Membranes of the Brain 
 
 The brain, covered by two membranes, the dura mater and pia-arach- 
 noid, lies enclosed within the cranium. 
 
 The topographical relations of the different parts of the brain to the 
 skull are shown in Fig. 109. 
 
 The dura mater is the periosteum of the bones of the skull. In the 
 healthy adult it is attached firmly only to the base of the skull, and can be 
 easily detached from the convexity. Extensions of it project sagitally 
 into the spaces between the hemispheres of the cerebrum (proc. falcif. 
 major.), the hemispheres of the cerebellum (proc. falcif. minor.), and trans- 
 versely between the cerebellum and the base of the occipital lobe (tentorium). 
 
 It forms, in places, by splitting into two layers, the so-called sinuses. 
 They contain blood. Its fibrous layer forms sheaths for the cranial nerves 
 as they pass through the foramina. 
 
 Its nervous supply arises partly from the sympathetic and partly from 
 the trigeminal. 
 
 It is impossible to give the varying views of the leading specialists on 
 the many mooted points. 
 
 The names of authors are purposely not quoted, except in a few cases, 
 in which a fundamental theory is connected with a name. The soft 
 membrane is divisible into two, an outer layer, the arachnoid, and an inner, 
 the pia mater. 
 
 The pia lines all fissures, following closely the surface of the brain and 
 enters the ventricles as the tela chorioidea, while the arachnoid membrane 
 is stretched across the fissures. The two are closely connected by numerous 
 trabecular of connective tissue, so that pathologically, they may be treated 
 
 1 The need of brevity prevents minute discussion and compels us to present a very intricate 
 matter in as simple a form as possible. 
 
 445 
 
446 
 
 DISEASES OF THE BRAIN 
 
 as one. Between the dura and the pia arachnoid lies the subdural space, 
 which contains scarcely a trace of fluid. Even in disease it is not a place 
 for the collection of fluids. 
 
 On the other hand in the subarachnoid space, between the arachnoid 
 membrane and the pia, because of its communication with the ventricles, is 
 found normally some cerebro-spinal fluid, which, in progressive paralysis, 
 senile atrophy, etc., is often much increased (hydrocephalus externus). 
 
 Fig. 109. — Relation between the cranium and the' cerebral surface. (After Bardeleben- 
 
 Haeckel.) 
 
 2. Circulation in the Brain 
 
 The brain receives blood from 
 
 1. Both vertebral arteries. 
 
 2. Both internal carotid arteries. 
 
 Both vertebral arteries unite in passing from the medulla oblongata to 
 the pons and form the basilar artery, which before reaching the pons divides 
 into the two posterior cerebral arteries coursing backward and laterally. 
 — Art. cerebr. post. 
 
 The internal carotid branches as follows on both sides: 
 
 (a) Anterior cerebral artery (Art. cerebr. ant.) . 
 These two are connected by the anterior communicating artery. 
 
CEREBRAL CIRCULATION 
 
 (b) The middle cerebral artery (Art. foss. Sylvii). 
 
 (c) The posterior communicating artery (Art. commun. post.). 
 
 (d) The chorioidal artery (Art. chorioidea). 
 
 447 
 
 p ,'OpRL. 
 
 Gyv temp- prof 
 
 Fig. i 10. — Areas of the convexity as supplied by the cerebral arteries. White, area of the 
 art. fossae Sylvii. Red, of the art. cerebri anterior. Blue, of the art. cerebri posterior. The 
 other abbreviations as in Fig. 112. 
 
 Fig. hi. — Areas of the median surface as supplied by the cerebral arteries. 
 Colors as in Fig. no. 
 
 In the posterior communicating artery is mixed the blood from the carotid 
 with that from the vertebral, as it joins the posterior cerebral artery. 'By 
 
448 
 
 DISEASES OF THE BRAIN 
 
 means of the anterior and posterior communicating arteries the circle of 
 Willis is completed. Variations in it are frequent. 
 The three main cerebral arteries are therefore: 
 
 i. The anterior cerebral artery. ] — . , -_■ - ., N 
 
 „,, . , „ , t (Both from the carotid.) 
 
 2. The middle cerebral artery. J 
 
 3. The posterior cerebral artery (from the basilar artery). 
 
 These main arteries send short branches into the inner part of the brain 
 (central arteries), especially to the ganglia, and long branches to the cortex, 
 and the adjacent white matter (cortical arteries). 
 
 The short and long (cortical) branches form, in themselves, two complete 
 systems of circulation, not interdependent but independent of each other, 
 as there are no anastomoses between them. 
 
 F.tr: 
 
 P / Op.RL 
 
 1. ope re. v- 
 
 Gyr temp. prof. 
 
 Fig. 112. — Convolutions and fissures of the convexity. (Using a design of Flechsig.) S.h., 
 S.a., S.f, Ramus horizontalis, ramus ascendens or verticalis and ramus posterior of trie Sylvian 
 fissure; Of.R., operculum Rolandi; f.tr., pars triangularis of the third frontal convolution; 
 P. of ere, pars opercularis of the third frontal convolution; P.O., pars orbitalis of the third frontal 
 convolution. The designation Gyr. suframargin ought to begin more in the front immediately 
 behind the Gyr. centr. fost. 
 
 The short branches (central arteries) have among themselves as well as 
 with other arteries few anastomoses, so that they are designated terminal 
 arteries. 
 
 The long branches course along on the surface of the brain, supply the 
 cortex and adjacent white matter, and have, among themselves, anastomoses. 
 
 Of the central branches, those that supply the inner capsule, the lenticular 
 nucleus and the optic thalamus, are of especial importance. (Lenticulo- 
 striate and lenticulo-optic arteries). The region supplied by these non- 
 anastomosing vessels, is the place of preference, for the bursting of blood- 
 vessels and cerebral softening;. 
 
CEREBRAL CIRCULATION 
 
 449 
 
 The anterior cerebral artery (main branch: the corpus callosum artery: 
 art. corp. callosi) supplies both superior frontal convolutions, the base of the 
 frontal lobes and the medial parts of the central hemisphere (gyrus f ornicatus, 
 the paracentral lobule including the precuneus, and the corpus callosum). 
 
 Since the paracentral lobule is the seat of the leg-center, an obstruction in 
 this branch of the anterior cerebral artery produces an isolated paralysis of 
 the leg. 
 
 Disease of the middle cerebral artery or the artery of the fissure of Sylvius 
 has especial importance in disturbances of speech. Its short central branches 
 have already been mentioned, as the important lenticulo-striate, etc. 
 
 Fig. 113. — Convolutions and fissures of the medial surface. (Using a design of Flechsig.) Sulc. 
 calhsom., Sulcus callosomarginalis; Lobus parac., lobus paracentralis. 
 
 Its 5 to 7 main cortical branches supply the third frontal convolution 
 the island of Reil, the central convolutions, the parietal lobes, and the 
 superior convolutions of both temporal lobes. 
 
 The posterior cerebral artery supplies the walls of the ventricles. Its 
 short branches go to the optic thalamus, the cms cerebri, the geniculate 
 body, the chorioid tela, etc. By short branches it supplies also the splenium 
 of the corpus callosum. 
 
 It divides into two main cortical branches : 
 
 1. The temporal artery which supplies the undersurface of the temporal 
 lobe. 
 
 2. The occipital artery which supplies the occipital lobe. 
 
 Of the branches of the latter the calcarine artery is especially important, 
 because it supplies the vicinity of the calcarine fissure — that is the visual 
 center — and the optic radiation. 
 29 
 
450 DISEASES OF THE BRAIN 
 
 The cerebellum and the pons are supplied by branches of the basilar artery 
 (the cerebellar artery). 
 
 ,The different parts of the brain are, therefore, supplied as follows: 
 
 The cortex of the cerebrum and its white matter; by the cortical branches of 
 the three main arteries, thus: 
 
 The frontal brain: basal, medial and upper convexity, by the anterior 
 cerebral artery; the rest of its convexity, especially F. m. and F. i. by the 
 middle cerebral artery. 
 
 The central convolutions, the island of Rett and the convexity of the parietal 
 lobes by the middle cerebral artery. 
 
 The medial parietal lobes (paracentral lobule and prsecuneus) gyrus 
 fornicatus, by the anterior cerebral artery. (Prsecuneus partly also by the 
 posterior cerebral artery.) 
 
 The temporal lobes, the two upper convolutions by the middle cerebral 
 artery; the rest, especially the base, by the posterior cerebral artery. 
 
 The occipital lobes, by the posterior cerebral arteries. 
 
 The large ganglia and inner capsule, by the central branches of the three 
 main arteries. 
 
 The corpus callosum by the anterior cerebral artery; the splenium by the 
 posterior cerebral artery. 
 
 The middle basal region, by the posterior communicating artery. 
 
 The cerebral peduncles, by the posterior cerebral and the posterior com- 
 municating arteries. 
 
 The quadrigeminal bodies, by the posterior cerebral artery. 
 
 The pons, cerebellum and medulla oblongata, by the superior cerebellar 
 artery, the basilar artery and the inferior cerebellar artery, the vertebral 
 artery. 
 
 Veins of the Brain. 
 
 The veins of the brain have no valves and are connected by innumerable 
 anastomoses. They drain into the dural sinuses (sinus longitudinalis, etc.) , 
 which finally empty their blood into the internal jugular vein. The veins 
 from the corpus striatum and the choroid plexuses of the ventricles, unite in 
 the azygos great cerebral vein {vena magna Galeni) below the corpus cal- 
 losum. This empties its blood into the straight sinus (sinus rectus). 
 
 3. Morphology of the Brain 
 
 The weight of the brain in man averages about 49 ounces, in woman 
 
 4 ounces less. 
 
 On the grounds of embryological development one distinguishes: 
 1. The end brain (telencephalon); both hemispheres with the anterior 
 
 ganglia; caudate nucleus, lenticular nucleus, amygdaloid nucleus and claus- 
 
 trum. Its cavity: the lateral ventricles. 
 
MORPHOLOGY OF THE BRAIN 451 
 
 2. The interbrain (diencephalon) : the optic thalamus with its appendages, 
 especially the important geniculate body and, at the base, the chiasma and 
 optic tract. Its cavity: the third ventricle. 
 
 These together constitute the forebrain (prosencephalon). 
 
 3. The midbrain (mesencephalon) : the quadrigeminal bodies, inferior 
 lemniscus, red nucleus and the nuclei of the third and fourth nerves, cerebral 
 peduncles (tegmentum and base) with the substantia nigra. Of the cerebral 
 cavities the aqueduct of Sylvius corresponds to the midbrain. 
 
 4. The hind-brain (metencephalon or rhombencephalon) : the pons and 
 cerebellum. 
 
 5. The after-brain (myelencephalon) : the medulla oblongata. 
 
 The cavity of the hind and after brain is the fourth ventricle. The end 
 towards the spinal cord forms the lowest part of the pyramidal crossing. 
 
 The forebrain, together with the midbrain is frequently spoken of as 
 the greater brain (cerebrum) as against the cerebellum, the medulla and the 
 pons. 
 
 The "brain stem" is the name for the medulla, pons, midbrain, inter- 
 brain and the cerebral ganglia in contrast to the cortex and the white matter 
 of the hemispheres. 
 
 Each hemisphere is divided into frontal, parietal, temporal and occipital 
 lobes. 
 
 The largest fissure, the fissure of Sylvius, separates the temporal lobe 
 from the frontal and parietal lobes. Many claim that the frontal lobe is 
 separated from the parietal lobe by the fissure of Rolando or central fissure, 
 so that the anterior central convolution is counted as belonging to the frontal, 
 the posterior central convolution as belonging to the parietal lobe. But both 
 central convolutions are often spoken of as a whole: the central region or 
 region of Rolando, and in that case it counts as an independent part lying 
 between the frontal and parietal lobes. 
 
 Between the frontal, parietal, and temporal lobes, in the depths of the 
 fissure of Sylvius lies the island of Reil (insula). The parts of the frontal 
 brain and of the central convolutions covering it are, hence, called the oper- 
 culum, and that part of the operculum that belongs to the central convolutions, 
 the Rolandic operculum, that, which belongs to the inferior frontal cortex, 
 the frontal operculum (Broca's area). 
 
 The occipital lobe is divided sharply from the parietal lobe, only on the 
 medial surface, by the parieto-occipital fissure; a distinct boundary is lacking 
 at its convexity. 
 
 (a) Convolutions and Fissures of the Convexity. 
 
 The surface of the frontal lobe is divided by two fissures into three con- 
 volutions, the superior or first, F s, the middle or second, F m, and the inferior 
 or third, F i, frontal convolution. 
 
452 DISEASES OF THE BRAIN 
 
 The inferior frontal convolution is divided by the anterior (horizontal) 
 and the ascending (vertical) branch, (ramus ascendens) of the fissure of 
 Sylvius into three parts (Fig. no): 
 
 i. In front, pars orbitalis, 
 
 2. Between the horizontal and the ascending branch, the pars triangularis 
 and 
 
 3. Behind the ascending branch, the before mentioned frontal operculum 
 which posteriorly gradually disappears in the Rolandic operculum. 
 
 The parietal lobe extends anteriorly, if we consider both central con- 
 volutions as the Rolandic region per se, up to the fissure that marks the back 
 boundary of the posterior central convolution and is divided by the inter- 
 parietal fissure into a superior and an inferior lobule. 
 
 The inferior parietal lobule is divided into 
 
 1. The anterior part or the supramarginal gyrus which surrounds the 
 posterior end of the fissure of Sylvius and, below, forms the extension of the 
 superior temporal convolution posteriorly, and which extends anteriorly to 
 the posterior central 'convolution (the French designate only that part of 
 the convolution that touches the posterior end of the fissure of Sylvius, as the 
 gyrus supramarginalis) . 
 
 2. The posterior part, or the angular gyrus (pli courbe) which surrounds 
 the posterior end of the temporal fissure. Between the supramarginal and 
 angular gyri, the inconstant fissure of Jensen. 
 
 This angular gyrus, often interrupted by a fissure, continues into the 
 occipital lobe. 
 
 To simplify orientation one must remember that the supramarginal 
 gyrus is placed at the posterior end of the fissure of Sylvius, and the angular 
 gyrus at the posterior end of the superior temporal fissure. 
 
 The convexity of the temporal lobe is divided, by the above-mentioned 
 superior temporal fissure (sulcus temp, sup.) and the middle temporal fissure, 
 into three convolutions, the superior, middle, and inferior temporal convolu- 
 tions. The superior temporal convolution continues posteriorly into the 
 parietal lobe, especially the supramarginal gyrus corresponding to the end of 
 the fissure of Sylvius, the middle temporal convolution, especially into the 
 angular gyrus and the inferior, into the corresponding occipital convolutions. 
 
 Coursing along the surface of that part of the temporal lobe, that is 
 turned toward the island of Reil — concealed in the fissure of Sylvius — taking 
 their origin in the surface of the superior temporal convolution, 1 or 2 cross 
 convolutions (gyri temporalis profundi or transversi, also called the convolutions 
 of Heschl), pass backwards and inwards to the parietal region behind the 
 island of Reil. 
 
 The occipital lobe has anteriorly no sharp boundary. As an artificial 
 boundary one assumes a line that unites the upper end of the parieto- 
 occipital fissure with the incisura praeoccipitalis. 
 
MORPHOLOGY OF THE BRAIN 453 
 
 (b) The Median Surface and Base of the Brain 
 
 The median surface of the hemispheres shows first of all the entrance of 
 the corpus callosum, that great commissural system, which connects both the 
 symmetrical and the asymmetrical parts of the two hemispheres. 
 
 Below the corpus callosum, and bent from before backwards in a similar 
 curve, we see the fornix (the thickened ends of the hemispheres), which 
 continues towards the back in the fimbria of the inferior horn of the lateral 
 ventricle (Fig. 113). 
 
 Parallel to, and above the corpus callosum, lies one of the main furrows 
 of the median surface, the sulcus cinguli or callosomarginal sulcus, which 
 turns posteriorly upwards to the edge of the hemisphere and ends in a small 
 notch behind the end of the posterior central convolution. 
 
 The concentric convolution between this furrow and the corpus callosum, 
 is called the gyrus fornicatus. What lies in front of, and above it, is classed 
 with the frontal brain (gyrus rectus and medial part of the superior frontal 
 convolution); only the hindmost part of this concentric convolution, which 
 corresponds to the central convolutions of the convex surface, is considered 
 as a small lobe, per se, called the paracentral lobule (lobus par acentr alls). 
 
 The posterior part of the gyrus fornicatus (back of the ascending branch 
 of the sulcus cinguli) , spreads into the precuneus, which is part of the parietal 
 lobe. Its posterior boundary is the second main fissure of the median sur- 
 face: the parieto-occipital fissure, mentioned before as the boundary between 
 the parietal and the occipital lobes. Into this fissure, anteriorly, passes a 
 third very important main fissure, the calcarine fissure (fissura calcarina), run- 
 ning sagitally postero-anteriorly. 
 
 The wedge-shaped part of the convolution, lying between the fissures, 
 is the cuneus. 
 
 Under the calcarine fissure lies the gyrus lingualis, and under this, sepa- 
 rated from it by the occipito-temporal sulcus (collateralis) the gyrus fusiformis, 
 which continues anteriorly through the temporal lobe. Both, the gyrus 
 lingualis and fusiformis belong to the basal surface. The gyrus fornicatus 
 continues backwards and upwards into the precuneus. Towards the lower 
 and back part, it winds about the splenium of the corpus callosum, and here 
 continues into the gyrus hippocampi, which extends to the anterior end of the 
 median temporal lobe. The hook-like bend, which the concentric convolu- 
 tions (gyrus fornicatus and gyrus hippocampi) show at their anterior extrem- 
 ity, is called the uncus. 
 
 In the gyrus hippocampus, the gyrus lingualis continues from the back, 
 so that in the gyrus hippocampus the gyrus fornicatus and the gyrus lingualis 
 are united. 
 
 As is evident, the median surface, posteriorly, gradually passes into the 
 basal surface. 
 
454 
 
 DISEASES OF THE BRAIN 
 
 On the basal surface, between the temporal and the occipital lobes, no 
 sharp line can be drawn. The inferior temporal convolution helps to form 
 the base. 
 
 Medially from it, separated by the inferior temporal fissure, lies the 
 before mentioned fusiform gyrus, and medially in front of this, lies the 
 hippocampal gyrus, in back of it the lingual gyrus. The fusiform gyrus is 
 separated from them by the occipito-temporal fissure (collateral fissure) 
 
 Cornmis. 
 ant. 
 
 Fig. 114. — Lateral view of inter- and midbrain. The connections between corp. geniculat. 
 laterale {Corp. g. lat.) and the occipital lobe, not indicated. {After Edinger.) 
 
 which extends through the entire undersurface, almost to the temporal pole. 
 In front the base shows between the central peduncles, the mammillary 
 bodies, the pituitary body, the optic tracts, the anterior and posterior 
 perforated spaces. 
 
 At the base of the frontal lobe the gyrus rectus, i. e., the basal continuation 
 of the superior frontal convolution is cut off towards the inner side, by the 
 olfactory sulcus in which the olfactory lobe is situated. Outwards from it, 
 lies the basal part of the middle frontal convolution, and separated from it 
 by the sulcus orbitalis, the basal (also called orbital) part of the inferior 
 
HISTOLOGICAL STRUCTURE OF THE CORTEX 
 
 455 
 
 frontal convolution (pars orbitalis of F. i.) . To understand the form relations 
 of the inter-, mid-, after-, and hind-brain, one needs only to look at Figs. 
 114 and 115. 
 
 VentrLculus tertius 
 
 Interbrain 
 
 Midbrain 
 
 Hind-brain 
 
 After-brain 
 
 Peduncle 
 
 Corp. restif. 
 Aia cine re a 
 
 Cerebelum 
 
 Spinal cord 
 Fig. 115. — View of inter- mid- and after-brain. {After Edinger.) 
 
 4. Histological Structure of the Cortex 
 
 The cellular cortical structure, as proven by embryology and comparative 
 anatomy, may be divided into six layers: 
 
 1. Lamina zonalis (molecular layer). 
 
 2. Lamina granulans externa (outer granular layer). 
 
 3. Lamina pyramidalis (pyramid layer). 
 
 4. Lamina granulans interna (inner granular layer). 
 
456 
 
 DISEASES OF THE BRAIN 
 
 5. Lamina ganglionaris (ganglia cells layer). 
 
 6. Lamina multiformis (polymorphous or spindle celled layer). 
 
 This six-layered type, established on embryologic grounds, in the whole 
 cortex of the cerebrum, is, besides, in Fig. 116 divided into subdivisions 
 (III a and b, VI a and b), and is subject to numerous, local modifications 
 which frequently are sharply limited; they consist in increase, diminution, or 
 
 
 Fig. 116. — Cyto- and myelo-architectonic structure of the cortex. The Arabic figures at the 
 right designate the myelo-architectonic layers, xo, Pars afibrosa laminae tangentalis; ia, pars 
 externa laminae tangentalis; ib, pars intermedia laminae tangentalis; ic, pars interna la min a?, 
 tangentalis; 2, lamina dysfibrosa; ^aa, stria Kaesi-Bechterewi; 3a/?, regio typica partis superficialis 
 laminae suprastriatae; 36, pars profunda laminae suprastriatae; 4, stria Baillargeri externa; 5a, 
 lamina intrastriata; $b, stria Baillargeri interna; 6a(3, lamina substriata; 6a/3, lamina limitans 
 externa; 66a, lamina limitans interna; 6fy3 substantia alba. The Roman figures at the left 
 designate the cyto-architectonic layers. I to VI see text. (After Vogt and Brodmann.) 
 
 transposition of single layers, and the inner granular layer may disappear, 
 or by fission, double, and other cellular formations, i. e., giant pyramid cells 
 may appear and finally variations in the thickness and size of the cells, in 
 their density, and the breadth of the layers, are found. Accordingly, in the 
 entire cortex about 50 such modifications of the layers can be distinguished, and 
 corresponding regions marked off. In general, their boundaries do not 
 correspond to the fissures and convolutions (Fig. 117). 
 
 Of special importance are two extreme variations of cell layers : 
 1. The giant pyramidal or motor type in the anterior central convolution 
 (Fig. 118). The fourth layer is wanting. 
 
HISTOLOGICAL STRUCTURE OF THE CORTEX 
 
 457 
 
 2. The calcarine type with duplication of the inner granular layer in the 
 immediate vicinity of the calcarine fissure (corresponding to the clinico- 
 pathologically defined visual sphere of Henschen) . 
 
 The regions as mapped off according to the cortical cell architecture 
 (cyto-architectonically), correspond only partly, and only approximately to 
 the regions mapped out by Flechsig, on the basis of myelin-development 
 (myelogenesis) . 
 
 Beside the cellular layer, differences in density, arrangement, and caliber 
 of the medullated nerve fibres of the cortex, condition a different cross-cut 
 
 Fig. 117. — The cyto-architectonic cortical regions of the convexity. (After Brodman.) 
 
 division of various places of the cortex, which gives boundaries of about the 
 same cortical fields, as cyto-architectonics (Fig. 116). 
 
 The relation of the horizontal fibrillation of the cortex, to the radial 
 medullated nerve fibres, if we overlook local peculiarities, presents the 
 following appearance: 
 
 1. Tangential layer. 
 
 2. Supraradial felt work (Edinger). 
 
 3. Intraradial felt work, containing the internal and external lines of 
 Baillarger, which form only a density in the fibres that runs horizontally 
 through the cortex. 
 
 More detailed investigation has led to a further separation into divisions 
 
458 
 
 DISEASES OF THE BRAIN 
 
 ** * • » ■• 4'- • '• J. ■' 
 
 , » 
 
 and subdivisions, which we, in the above scheme 
 of O. Vogt (Fig. 1 1 6) have brought into relation 
 with the cyto-architectonic fundamental type of 
 Brodmann. 
 
 The peculiarly strong marking of this hori- 
 zontal fibre layer in the calcarine region is known 
 as the Vicq d'Azyr stripe; it is easily seen in a 
 freshly cut brain, appearing as a white line in 
 the cortex. 
 
 5. Nuclei of the Cerebral Nerves 
 
 1. The olfactory nerve (Nerve I) cf. page 466. 
 
 2. The optic nerve (Nerve II) cf. visual 
 paths (Fig. 119). 
 
 3. Nerves of the eye muscles: the oculo- 
 motor nerve (Nerve III), the trochlear nerve 
 (Nerve IV), the abducens nerve (Nerve VI) are 
 all purely motor. 
 
 The nucleus of the oculomotor nerve lies in 
 the region of the anterior quadrigeminal bodies, 
 in the floor of the aqueduct of Sylvius. It has 
 a medial division, lying exactly in the middle 
 line, which sends fibres to both sides, and a 
 larger lateral one, on each side, which also sends 
 off fibres to the opposite side. The nucleus 
 consists of aggregations of cells, each of which, 
 probably, supplies a definite muscle. The roots 
 issue from the inner edge of the cerebral pe- 
 duncle, in front of the pons, the fibres pierce 
 the dura mater, run into the wall of the caver- 
 nous sinus, and, through the superior orbital 
 fissure, reach the eye. The nerve is divided 
 into a superior and an inferior division (ramus 
 superior and inferior). From this latter a short 
 branch, the radix brevis ganglii ciliaris, goes to 
 the ciliary ganglion (Fig. 120). 
 
 4. The nucleus of the trochlear nerve lies in the 
 caudal extension of the oculomotor nerve nucleus 
 in the region of the posterior quadrigeminal 
 
 Fig. 118. — The giant pyramidal type in the anterior central 
 ^convolution. (After Brodman.) 
 
NUCLEI OF THE CEREBRAL NERVES 
 
 459 
 
 bodies. The fibres pass from the nucleus dorsally and decussate completely 
 in the velum medullare anticum. They appear back of the quadrigeminal 
 bodies, pass around the cerebral peduncles towards the front and ventrally, 
 and reach, through the wall of the cavernous sinus and the superior orbital 
 fissure (Fissura orbitalis superior) , the superior oblique muscle. (Musculus 
 obliquus superior.) 
 
 5. The Abducens Nerve. — The nucleus lies in the region of the pons, in 
 the floor of the fourth ventricle. The fibres pass ventrally, emerging between 
 the pons and the pyramid. The nerve passes through the cavernous sinus 
 and the superior orbital fissure to the external rectus muscle. (Musculus 
 rectus externus.) (Fig. 121.) 
 
 Connections between the muscles of the eye, as well as with the Deiter 
 nucleus (of the vestibular nerve) and the cerebellum are made by the posterior 
 longitudinal bundle (Fascicul. longitud. post.), which can be traced from the 
 
 Corp. Quadrigemina 
 
 Pons 
 
 Fig. 119. — Schematic view of the location of the nuclei Nr. III-XII. Motor nuclei dotted, 
 sensory deep black. XI X means X and IX (common nucleus of Nr. IX and X). (After 
 Villiger.) 
 
 posterior commissure to the spinal cord, and which among other things 
 produces the relations existing between ocular movements and changes in 
 equilibrium. 
 
 6. The trigeminal nerve (Nerve V) , a mixed nerve, has a very large nucleus. 
 The main motor nucleus lies in the dorsolateral tegmental part of the pons. 
 In addition, there is a smaller motor nucleus in the quadrigeminal region at 
 the side of the aqueduct. The motor root passes directly to the third branch 
 of the mandibular nerve. 
 
 The thicker sensory root has its origin in the Gasserian ganglion, enters 
 the pons, and runs near the sensory end nucleus, where the fibres divide into 
 ascending and descending branches. 
 
 The ascending branch ends in a collection of cells, situated next to the 
 motor nucleus in the tegmental part of the pons (nucleus sensibilis N. V) ; 
 
460 
 
 DISEASES OF THE BRA IX 
 
 the descending branch ends in a cell column which is the caudal extension of 
 the before mentioned sensory nucleus. This collection of descending fibres 
 is called the descending or spinal trigeminal root; it can be traced to the 
 second cervical segment. The caudal part of this sensory nucleus is identical 
 with the gelatinous substance of Rolando in the posterior horn of the spinal 
 cord. 
 
 The facial nerve, a motor and secretory nerve (N. VII) . The nucleus lies 
 in the tegmental part of the pons, ventrolaterally from the abducens nucleus. 
 
 Fig. 120. — Schematic cut behind the anterior quadrigeminal bodies through the nucleus of 
 the oculomotor. Instead of nucleus Nr. IV read Nr. III. {After Edinger.) 
 
 The fibres pass dorsally around the nucleus of the abducens nerve, then 
 ventrally and appear at the posterior edge of the pons, at the side of the olive. 
 The intermediate nerve (X. intermed. Wrisbergii), classified as a minor 
 part of the facial nerve because so closely associated with it, has partly sen- 
 sory functions (fibres of taste) , partly secretory (through the chorda tympani) . 
 Its sensory fibres from the geniculate ganglion end probably in the sensory 
 end-organ of the glosso-pharyngeal nerve. 
 
NUCLEI OF THE CEREBRAL NERVES 
 
 461 
 (See 
 
 The auditory nerve (nervus acusticus, N. VIII), cf. auditory paths. 
 Fig. 122.) 
 
 The glosso-pharyngeal nerve (N. IX), a mixed nerve. The motor fibres 
 arise in two nuclei: 
 
 1. The dorsal nucleus in the floor of the fourth ventricle, and 
 
 2. The ventral nucleus (or ambiguus) , dorsal from the olive. 
 
 The sensory fibres arise in both ganglia of the nerve (G. superius and 
 petrosum) and pass as sensory roots into the medulla oblongata beneath the 
 facial and auditory nerves behind the olive. 
 
 Here the fibres divide into ascending and descending branches. The 
 ascending end in the nucleus ala cinerece, the descending (the so-called 
 tractus solitarius) in the adjoining nucleus tractus solitarii. 
 
 Fig. 121. — Cross-section in the region of the origin of the abducens. {After Edinger.) 
 
 The pneumo gastric nerve (Nervus vagus, N. X), a mixed nerve. The 
 motor fibres arise in the same nuclei as those of the glosso-pharyngeal nerve, 
 that is in the nucleus dorsalis and in the nucleus ambiguus. The sensory fibres 
 arise in both ganglia of the vagus nerve (G. jugulare and nodosum), pass 
 below the ninth nerve, back of the olive into the medulla, and end in the 
 same sensory end nuclei (nucleus alae cinerea? and nucleus tractus solitarii) 
 as those of the glosso-pharyngeal nerve. 
 
 The spinal accessory nerve {nervus accessorius, N. XI), a pure motor 
 nerve. It rises from a collection of cells that begins in the caudal extension 
 of the nucleus ambiguus (cerebral part of the accessory nucleus) and can be 
 traced to the seventh cervical segment (spinal part of the accessory nucleus). 
 
462 
 
 DISEASES OF THE BRAIN 
 
 The latter is situated at the base of the lateral horns, and in the dorsolateral 
 part of the anterior horns of the spinal cord. 
 
 From this nucleus arise 9-13 root-fibres, which leave the medulla oblon- 
 gata, resp. the cervical white matter (Fig. 124). 
 
 The hypoglossal nerve (nervus hypoglossus, N. XII), a pure motor nerve. 
 The nucleus lies in the floor of the fourth ventricle, next to the raphe. The 
 fibres pass ventrally and leave the medulla in 10-15 root-fibres, between the 
 olive and pyramid (Fig. 125). 
 
 S^p-^Ui-jn^f^- — -, 
 
 Fig. 122. — Motor nucleus and root of the trigeminal. (After Edingcr.) 
 
 6. General Relation between Structure and Function of the Brain 
 
 (a) Theory of Projection; Paths and Fields of Projection 
 
 The cortex receives stimuli from the sensitive parts of the surface of the 
 body (the entire skin, joints, muscles, so-called higher sensory surfaces, as 
 retina, organ of Corti, etc.), and sends out stimuli into parts that may be 
 moved volitionally, to the muscles, and to certain glands. 
 
 The paths which connect the cortex with the periphery are called pro- 
 jection paths. 
 
 There is no uninterrupted connection between the cortex and a sensory or 
 muscular end-organ. Between the cortex and periphery gray masses (e. g., of 
 the spinal cord) are interposed. In the cells of these interposed gray masses 
 the sensory nerves end and the motor arise. 
 
 The cells of these gray masses are connected with the cortex through ascend- 
 
THEORY OF PROJECTION: PATHS OF PROJECTION 463 
 
 ing paths. There are then in the projection paths, between periphery and cor- 
 tex, one or more gray-matter way-stations. So all sensory nerves of the trunk and 
 the limbs starting from the periphery have their first end in the gray matter 
 of the spinal cord or of the medulla oblongata (sensory end-nuclei). And 
 all motor nerves for trunk and limbs have their origin in the gray matter of the 
 spinal cord (anterior horn cells, motor origin cells). 
 
 But the cerebral nerves are wholly analogous to these. They, too, have 
 their sensory end-nuclei and their motor origin nuclei in the gray matter 
 lying next to the fourth ventricle and the aqueduct of Sylvius. These nuclei, 
 too, are aggregations of ganglia cells, which — in centripetal directions — 
 receive the impulses of the peripheral sensoiy neurons and transmit them to 
 their axons leading to the cortex, or — in centrifugal directions — their axons, 
 themselves, become motor nerves, passing to the periphery. 
 
 Just because this gray matter of the medulla and the quadrigeminal 
 region receives sensory roots in the end-nuclei, and sends out motor roots 
 from origin nuclei, one has named this cerebral region in consequence of its 
 far-reaching analogy with the function of the spinal cord, the spinal cerebral 
 region. This gray matter in the floor of the fourth ventricle and aqueduct, 
 we can, when taken together with the nuclei of the posterior column of the 
 medulla and the gray matter of the spinal cord (anterior and posterior horns), 
 look upon as the first station of combined sensory and motor processes, and 
 as primary centers of these functions. These primary centers represent 
 the first projection of the periphery in the central nervous system. 
 
 Any lesion of these primary centers interrupts the functional capacity 
 of some peripheral part, whether it be sensory or contractile. About the 
 term projection, see below. 
 
 For the further connection of these primary centers with the gray matter 
 of the cortex, we must consider separately, the motor and the sensory system. 
 
 For the main paths of motility (direct motor paths), there is no further 
 way-station between muscles and cortex, besides the subcortical gray matter 
 we have just mentioned, which contains the origin nuclei of the motor nerves 
 in the midbrain, medulla oblongata and spinal cord. For more detailed 
 information about these origin nuclei cf. page 458. 
 
 From the cortex to these nuclei there is an uninterrupted conduction 
 path, the central motor path. 
 
 Its region of origin in the cortex represents, for the second time, all vol- 
 untary motor processes (projection of the second class, cortical center), in a 
 circuit still to be described. For the voluntary movements, we have, then, 
 1. a cortical projection, 2. a subcortical projection (the latter for the cerebral 
 nerves in the gray matter of the quadrigeminal bodies, the pons, medulla 
 oblongata, and for the nerves of the trunk and limbs in the gray matter of 
 the anterior horn of the spinal cord). Both projection centers are con- 
 nected by motor projection paths. The main path for the voluntary move- 
 
464 
 
 DISEASES OF THE BRAIN 
 
 ments of the limbs and the trunk is also called the pyramidal tract (cortico- 
 spinal path). It rises in the upper two-thirds of the anterior central con- 
 volution, and its immediate vicinity, and in the paracentral lobule and 
 passes, without interruption, through the centrum semiovale, the inner 
 capsule (anterior two-thirds of the posterior limb), through the foot of the 
 cerebral peduncle and the pons into the medulla, at the lower end of which 
 its greater part undergoes decussation. The part that has decussated 
 passes into the lateral tracts of the spinal cord, and ends in the anterior 
 horn on the same side, from the cells of which the anterior roots, the motor 
 nerve rises. The non-decussating fibres pass away in the ventral bundle, 
 
 tfuc/eus ofUe 
 hferitt column 
 
 Fig. 123. — Exit of the vagus. {After Edinger.) 
 
 and reach the anterior horn on the same side. The pyramidal tract is the 
 longest of all conducting paths. 
 
 The corresponding motor path for the cerebral nerves, the cortico-bulbar 
 path, arises in the lower third of the anterior central convolution, passes 
 through the white matter, through the genu of the inner capsule, the foot of 
 the peduncles, and ends, with much decussation, in the contralateral nuclei 
 of the cerebral nerves. 
 
 Besides these important direct motor paths there are indirect paths, 
 interrupted in the red nucleus, and very important in the higher animals, but 
 less so in man. There is no unanimity of opinion as yet as to whether the 
 central path, that leads to the red nucleus, comes straight from the cortex, 
 or is interrupted in the thalamus, or whether it first makes a large detour 
 about the cerebellum, as do the pyramidal tracts which pass within the tract 
 of the crusta through the internal capsule and the cerebral peduncle to 
 
THEORY OF PROJECTION 
 
 465 
 
 the pontile nuclei (decussated) and into the cerebellum and through the 
 superior peduncles of the cerebellum to the red nucleus. 
 
 Things are more complicated for the centripetal paths, as well for general, 
 as for special sensation. Between the first endings in the gray matter 
 of the end-nuclei, and their final endings in the cortex, if we ignore 
 further interruptions, one way-station, at least, is always interposed; this is 
 formed by the gray nucleus of the optic thalamus with its appendages 
 (both geniculate bodies) and the gray matter of the quadrigeminal bodies. 
 
 fibr-< 
 
 Fig. 124. — Nucleus and root of the hypoglossal nerve, nucleus ambiguus and root of the vagus. 
 
 (After Edinger.) 
 
 From the sensory end nuclei arises a second centripetal path, which, for 
 the most part, passes into the medial and lateral fillet and ends in the optic 
 thalamus with its appendages and in the quadrigeminal bodies. Therefore 
 the thalamus and the quadrigeminal bodies represent a second sensory 
 projection. From the thalamus and the quadrigeminal bodies arises, 
 then, a third path which ends in the cortex of the cerebrum, so that the 
 cortical sensory projection is at least the third projection. (Cf . also page 
 491.) 
 
 Although, according to its embryological development, the ganglia cells 
 of the retina do not correspond to the spinal ganglia, nor the optic nerve, to 
 a posterior root, but rather represents the connection of two cerebral parts 
 (the retina is a cerebral part that has been pressed forward) , physiologically 
 the same observation that we have just made with reference to the other 
 3° 
 
466 
 
 DISEASES OF THE BRAIN 
 
 centripetal nerves may be applied to the connection between retina and 
 cortex. Even the fibres of the optic nerve, which arise in the cells of the 
 retina, find their first ending in a subcortical nucleus, its primary center, 
 only this lies in the interbrain or midbrain (the second station of the rest of 
 the sensory paths), namely, in the external geniculate bodies of the optic 
 thalamus and the anterior quadrigeminal bodies. Hence the central path 
 goes through the optic radiation into the cortex of the occipital lobe (cf. 
 later visual pathways). Here also there is a primary subcortical and a 
 secondary cortical projection. 
 
 .tfudeueX 
 
 Ttbrat propria^ 
 nucUiXn 
 
 .Vucleuj XII 
 
 Fibrae afTerefitea 
 juLclei XD 
 
 -UusKniieTLtr. 
 
 Karros XII. 
 
 Fig. 125. — Nucleus of the N. hypoglossus (Weigert stain). 
 
 The olfactory path demands separate treatment. The axons of the 
 peripheral nerves end in the olfactory bulb; from here a second path goes, 
 especially to the gray matter of the olfactory trigone and the anterior per- 
 forated substance; from here a third path, which ends in the cortex of the 
 gyrus hippocampi and in the hippocampus major. Therefore for the optic 
 and olfactory nerves, between which and the other sensory nerves, in respect 
 to their embryological development, there is not the slightest analogy, the 
 
MNEMIC CORTICAL FIELDS: ASSOCIATION FIELDS 467 
 
 same law holds good, viz. — that between the periphery and the cortical center 
 gray matter intermediate stations are interposed. 
 
 Therefore we can say: All the sensitive surfaces of the body and all Us 
 organs that produce motion, are projected upon the cortex by the help of one or 
 more intermediate stations of gray matter. 
 
 The word projection is not to be understood literally, even in its applica- 
 tion to the first projection in the spinal cord, and certainly not when applied 
 to cortical projections — not in the sense, that for every point in the periphery, 
 there exists a co-ordinate point in the cortex, and that the arrangement of the 
 separate cortical elements is a replica of that of the peripheral sensory and 
 muscular elements. Such correspondence between periphery and cortex 
 does not exist. For instance, not each separate muscle has its own center in 
 the cortex, but certain groups of muscles for definite related movements are 
 represented there. [In other words there is a center for movements rather 
 than for muscles.] But in broad outline, the projection does exist, in so far as 
 various sense-organs, and various sensory and motor processes of the limbs 
 are co-ordinated with various regions of the cortex. And even if, within such 
 a cortical region, corresponding to the motor processes, say, of an upper 
 extremity, the arrangement does not repeat the muscular scheme in absolute 
 detail, on the other hand, the entire region is not as a mass co-ordinated to the 
 entire limb, but parts of it correspond to certain groups of muscles. In this 
 broader sense, the conception of a projection of the body, on the cortex, is 
 justified. 
 
 Those cortical regions, that receive projection paths, or from which they 
 emerge, are called fields of projection. Their destruction is marked, there- 
 fore, by the destruction of the motor processes or sensory processes of defi- 
 nite perceptive or effector organs; their injury without destruction by convul- 
 sions or other phenomena of motor irritation, or pains or other sensory 
 phenomena of irritation (elementary hallucinations, for instance). 
 
 (b) The Mnemic Cortical Fields and the Theory of Association 
 
 Fields 
 
 The reception of centripetal stimuli in the projection fields, when this 
 innervation has reached a certain level, 1 is the cause of perception. The excita- 
 tion of motor fields of projection, at a certain level leads to innervation and 
 subsequent movement. 
 
 The cerebrum has another broader function — that of retaining lasting 
 traces of sensory or motor innervations that once existed (residues or rem- 
 nants, the possession of the memory) . These remnants not only make 
 
 1 Not every stimulation of the cerebrum is connected with consciousness, though it is to be 
 assumed, that, in man, no consciousness is possible without corresponding stimulation of the 
 cerebrum. 
 
468 DISEASES OF THE BRAIN 
 
 possible conscious recollection of perceptions and movements, but also, even 
 without leading to conscious reproduction, as purely material possession make 
 it possible for the nervous system, to perform repeated operations with greater 
 ease, certainty and perfection; upon them therefore depend dexterity. 
 
 This is the mnemic function of the cerebrum. On the other hand, it 
 produces associations among these residues; this is the associative function 
 of the cerebrum. The smell of a rose, for instance, rouses in consequence 
 of such association, a complete image of a rose. 
 
 It has been long debated whether the mnemic-associative functions are 
 bound to the same cortical regions as perception and innervation, or if they 
 have their own peculiar cortical fields. 
 
 According to a widely held opinion all parts of the cortex receive pro- 
 jection paths, or send them forth, so that the entire cortex is divided into 
 projection fields. The elementary mnemic possession belonging to every 
 projection field, that is, the remnants in any one sense field, would, eventu- 
 ally be localized within the same cortical region, but may be in other layers 
 as perception and innervation, so that, for instance, the association among 
 elements of the same sense fields, say among the optic elements, would take 
 place in the same cortical regions, the association, on the other hand, among 
 the remnants of various sense fields (optic, tactile, etc.), would be brought 
 about by the long associative fibres. Then, the same cortical region that 
 receives the optic radiation would possess optic memories. In this view, 
 it is not necessary to assume that the same nervous elements serve for percep- 
 tion, innervation, and memory, as this is, indeed, very improbable; the rem- 
 nants might be registered in the same large cortical region, and yet be bound 
 to different cell-fibre complexes. 
 
 Opposed to this is Flechsig's theory, which accepts only a small part of 
 the cortex as projection fields. According to him, only these are in com- 
 munication with the periphery through in- and outgoing paths. The rest 
 of the cortex has no such connection with the periphery. It is occupied by the 
 association fields, that have connections only with the projection fields 
 (associative fibres) and with the opposite hemisphere (commissural fibres) 
 and carry on the associative and higher mnemic functions. Furthermore 
 Flechsig divided the entire cortex, according to the time when myelin develops 
 into 36 different fields, of which those first maturing (primordial regions), 
 12 in number, constitute the projection fields, the majority of those maturing 
 later (intermediary regions), 16 in number, and the 7 which mature last 
 (terminal regions) exclusively constitute association fields (Fig. 126). 
 
 Flechsig's projection fields include as most important, both central con- 
 volutions, beside the paracentral lobe, the adjacent part of the first frontal 
 convolution and the gyrus fornicatus, the transverse convolution of the first 
 temporal convolution, with a small part of the latter, the vicinity of the calca- 
 rine, also little fields of the surface of the occipital lobe, the uncus of the 
 
MNEMIC CORTICAL FIELDS: ASSOCIATION FIELDS 
 
 469 
 
 hippocampal gyrus, and the inner surface of the temporal poles. All the 
 rest of the surface of the brain, and especially, both inferior frontal convolu- 
 
 Fig. 126. 
 
 Fig. 127. 
 Figs. 126 and 127. — Projection and association fields. According to Flechsig corona radiata 
 fibres are certainly proved to exist only in the dotted regions, not in the regions not dotted. The 
 remainder of the first temporal convolution and of the Gyr. hippocampi are still under considera- 
 tion. The other undotted regions are, according to Flechsig, association centers. {After 
 Flechsig.) 
 
 tions, the insula, the rest of the temporal and occipital lobes and the entire 
 parietal lobe, are, according to Flechsig, association fields (Fig. 127). 
 
470 DISEASES OF THE BRAIN 
 
 In Flechsig's theory two theses are included that demand separate treat- 
 ment: first, the purely anatomical, that only part of the cortex receives and 
 sends out corona radiata fibres, that only this part is connected directly 
 with the periphery and that the remaining portions of the cortex, are not, 
 therefore, projection fields. 
 
 The second thesis is physiological, relating to the function of the fields in 
 which corona radiata fibres are absent. These fields serve for the association 
 between optic, tactile, etc., processes, and, at the same time, for the higher, 
 even the highest psychic processes. 
 
 So far as the anatomical question is concerned, it must be granted Flechsig, 
 that the theory of equal distribution of projection fibres on the cortex can not 
 be maintained. The majority of the most important motor and sensory tracts 
 actually crowd together into narrow regions, lying in and about Flechsig's 
 projection fields. Even if the other regions are not wholly free of projection 
 fibres, and this or that boundary of Flechsig is, as yet, disputable, we must, 
 at any rate, recognize the important separation of these regions which are 
 very rich and those which are very poor in corona radiata fibres, whereby 
 the fundamental division of Flechsig must be conceded, at least a relative 
 justification. 
 
 A second question is, whether these regions in which corona radiata 
 fibres are scanty, are really seats of the highest associations. Another pos- 
 sibility is, that they have elementary mnemic functions, and as Exner, Noth- 
 nagel and Ziehen thought, that they are to be regarded as memory fields. 
 
 Then, in the neighborhood of every projection field, the corresponding 
 remnants of any one kind of sense impressions would have their substratum, 
 i. e., in the neighborhood of the acoustic projection field, the acoustic rem- 
 nants, in the neighborhood of the optic projection field (medial side of the 
 occipital lobe), the optic remnants (convexity of the occipital lobe). The 
 regions poor in corona radiata fibres, would then be mostly memory fields, 
 nevertheless the intra-sensory associations would take place within them, 
 which, as all authors agree, take place within the cortex. But associations 
 among the various sensory and motor areas, and all higher associative com- 
 plexes, would, as Wernicke supposed, be carried on through the large asso- 
 ciation paths running in the white matter. 
 
 For this latter supposition much experimental evidence may be adduced. 
 
 In the entire question, which can by no means be, as yet, satisfactorily 
 decided, the following point of view seems to be indicated as a compromise : 
 
 Certain cortical regions richly supplied with corona radiata fibres are 
 connected with receptor and effector organs : the projection fields. 
 
 There are further cortical areas poorly supplied with corona radiata 
 fibres, without important connection with the periphery. 
 
 But the distribution of perception and innervation, of memory and 
 association is not such, that the fields of the former serve only for projection, 
 
ASSOCIA TION PA THS 
 
 471 
 
 and only those of the latter for memory, and the latter, moreover, for the 
 higher associations but the fields of the former kind, serve for retaining the 
 remnants as well as for projection. 
 
 The fields of the latter kind serve almost exclusively for remnants, hence 
 deserve the name, mnemic fields. The association between the remnants 
 of the same sense takes place all over the cortex. 
 
 The associations between remnants of various senses, and all higher com- 
 plexes are made possible only by the long association fibres. This higher 
 association is bound to the systems, which by association fibres, represent 
 projection and memory fields combined. 
 
 Hence, no part of the cortex is wholly free from remnants. Granted 
 only, that certain parts serve as well for projection, while others do not, we 
 rr ay distinguish the latter as -purely mnemic fields, from the projection fields. 
 And the higher associative processes are not carried on in sepaiate cortical 
 fields but demand the long association fibres, which unite various cortical 
 fields in a common activity. 
 
 (c) Association Paths 
 
 The short association fibres which run within the cortex (intracortical) 
 or unite neighboring gyri (arcuate fibres), vary greatly in arrangement and 
 direction in different parts of the cortex. 
 
 The long association fibres, which unite distant parts of the cortex, run, 
 the longer they are, the deeper into the white substance. The knowledge 
 we have of them is still very limited. 
 
 Certain conspicuous aggregations of long association fibres in compact 
 bundles have received individual names. But one must not forget that the 
 many, more scattered fibres with association functions, are not included 
 therein. 
 
 These bundles consist not only of long fibres, which run from beginning 
 to end in the bundle, but, in their course, many fibres from the cortex enter, 
 and many pass out (Fig. 128). 
 
 Only the most important pathways of the long association fibres will be 
 mentioned and named : 
 
 1. Uncinate fasciculus. Connects the orbital surface of the frontal brain 
 with the pole and anterior portion of the temporal lobe. 
 
 2. Superior longitudinal fasciculus (or arcuate). From the frontal brain 
 to the parietal-occipital lobes and the posterior portion of the temporal lobe. 
 
 3. Inferior longitudinal fasciculus, ending in the vicinity of the posterior 
 horns, as the outermost of the three sagittal medullary layers, that sur- 
 round the posterior horn (cf. Figs. 143 and 144). It was considered for- 
 merly a mere bundle of association fibres between temporal and occipital 
 lobes, but is a mixed pathway. It takes also many projection fibres to the 
 
472 
 
 DISEASES OF THE BRAIN' 
 
 visual sphere, contains optic radiations and even corpus callosum fibres, 
 besides association fibres. 
 
 4. Occipitofrontal fasciculus, adjacent to the caudate nucleus, a mass 
 of fibres also passing from front to back. 
 
 5. Cingulum. Runs in the white matter of the gyrus fornicatus, begin- 
 ning in the anterior part of the frontal brain, ending in the occipital lobe, 
 consisting of sagittal fibres, which, mostly, connect adjacent cortical areas, 
 but contain single fibres running through its entire length. 
 
 6. The external and extreme capsules also contain sagittal association 
 fibres. See still other bundles of association fibres in Monakow's scheme. 
 
 (d) The Commissures, especially the Corpus Callosum 
 
 The commissural fibres connect cortical parts of both hemispheres. 
 1. The most important commissure is the corpus callosum. The mass 
 of fibres included in the center is known as the body. 
 
 
 Fasc. fronto-centralis 
 
 Regia Ralandica 
 
 Fasc. centro-parietalis 
 
 Cingulum 
 
 Fasc. fronto-occipit. 
 
 Occipital 
 pole 
 
 Fasc. rencinat. 
 
 Cingulum 
 
 Fornix 
 Fasc. longitud. infer. 
 
 Fig. 128. — Scheme of the most important association paths.' (After v. Monakow.) 
 
 The anterior curved portion is called the genu (knee) ; the posterior thick- 
 ening, the pad or splenium; the middle part the body. 
 
 It connects, by means of intra-hemispherical corpus callosum fibres, 
 symmetrical and asymmetrical cortical areas. This fibrillation is mixed 
 with the projection and association fibres, and constitutes a great part of 
 the white matter (Fig. 1 29) . 
 
 The knee (genu) sends its fibres into the frontal brain, as the anterior 
 
THE COMMISSURES 
 
 473 
 
 forceps; the splenium into the occipital and temporal lobes as the posterior 
 forceps, the fibres of which, for the most part, pass over into the tapetum of 
 the posterior and inferior horns of the lateral ventricle. The body of the 
 corpus callosum connects the central parts of the hemispheres, including 
 both central convolutions. 
 
 If one considers, that in the centrum semiovale projection and commis- 
 sural fibres run side by side (see scheme), but separate at the lateral edge 
 of the lateral horn, that then the projection fibres pass into the inner capsule, 
 whereas the commissural fibres, forming the corpus callosum, pass over the 
 ventricle, to the other side, it is clear, that in focal injuries there is a distinc- 
 tion, but lately discovered: a focal injur)' (/, Fig. 129) in the centrum semiovale 
 
 Fig. 129. — Scheme of the fibres in the corpus callosum (red). In the frontal plane only 
 connections of symmetrical regions could be indicated. The projection fibres black. I, II, 
 Ha and b, III, foci. 7 in the white matter affects fibres in tha corpus callosum and projection 
 fibres. II, Ha and lib only fibres in the corpus callosum. Ill in the inner capsule projection 
 fibres only. Th, thalamus; N.I., lenticular nucleus; anterior central convolution. 
 
 strikes projection fibres and fibres of the corpus callosum, a focal injury in 
 the inner capsule {III) only projection fibres, a focal injury in the corpus 
 callosum (II, Ila, lib), only commissural fibres. Since (see under apraxia) 
 the left hemisphere, through the corpus callosum, affects the innervations 
 of the right hemisphere, a focal injury (I), will cause besides paralysis of 
 the right extremities, dyspraxia of the left. A focal injury in the inner cap- 
 sule, on the other hand, produces only paralysis of the right, in the corpus 
 callosum only dyspraxia of the left extremities. Extensive interruption 
 of the fibres of the corpus callosum, probably especially of the central third, 
 produces a local symptom: dyspraxia of the left upper extremity — it does 
 not matter whether the interruption is in the left hemisphere, the corpus 
 callosum itself, or in the right hemisphere. By the great crowding of fibres 
 in the corpus, a lesion therein will, of course, be most fateful for praxia. 
 Of great importance in the production of motor aphasia, is the interrup- 
 
474 DISEASES OF THE BRAIX 
 
 tion of the anterior fibres in the corpus, since the frontal speech center 
 influences the right brain centers of the 7th and 12th nerves through the 
 corpus fibres. 
 
 So, too, interruption in the splenium fibres either in it or within the 
 forceps, and the white matter of the temporal and occipital lobes can help 
 to cause alexia and mind blindness (see sections dealing with these subjects). 
 
 2. The anterior cerebral commissure connects the basal parts of the 
 temporal and frontal lobes. 
 
 3. The lyre of fornix (Lyra Davidis) connects the hippocampal horns. 
 
 7. Clinical Localization in the Left Hemisphere 
 
 Fortunately pathology, roughly speaking, is not seriously affected by 
 differences in the views discussed in pages 467-472. 
 
 For, even if the answer is left open, whether the association between 
 various qualities (optic, tactile, acoustic, etc.), is secured by means of spe- 
 cialized cortical fields, orby long associative pathways, on one point the adher- 
 ents of both views agree, that lesions of certain areas in the cerebrum, if 
 cortex and white matter be considered together, produce no massive, striking 
 symptoms of lost functions in the sensory and motor realms, but cause 
 serious mnemic disturbances and loss of associative processes, that, on the 
 other hand, lesions in other areas are first chiefly characterized by paralysis 
 or anaesthesia. Thus, destruction of the anterior central convolution causes 
 hemiplegia, of the calcarine region, hemianopia; on the other hand, a lesion 
 in the third frontal convolution, the posterior third of the first temporal 
 convolution, of the parietal lobe and its transition into the occipital lobe 
 (in so far as there is no injury of the pathways leading to the primary pro- 
 jection centers), causes no paralysis or anaesthesia, but disturbances in 
 speech, writing, reading, agnosia, apraxia, respectively (cf. page 518, ff). 
 "Whereas Flechsig and his school connect these mnemic-associative disturb- 
 ances mainly with cortical lesions, we trace them mainly to injury of the 
 associative paths that pass below the cortex. 
 
 So we can say with some degree of certainty what clinical symptoms of 
 lost function will follow upon the destruction of entire parts of the cerebrum, 
 including cortex and white matter, i. e., of entire lobes, or parts of lobes, 
 though the question as to how far the cortical elements themselves, or the 
 association fibres passing through the white matter, are implicated, is, as yet, 
 in many respects, debatable. If one combines such a purely clinico-patho- 
 logical localization with the known results of electrical stimuli used to experi- 
 ment upon motor areas, the following localization appears. 1 
 
 For the more detailed localization of motor functions, the evidence 
 
 1 Only those symptoms have been described, which may be regarded as symptoms resulting 
 directly from injuries, not conditioned by the effects of vicinity or distance. The entire survey, 
 because of its brevity, can naturally give only a summary orientation. 
 
LOCALIZA TION IN THE LEFT HEMISPHERE 
 
 475 
 
 obtained by means of electrical stimulation, is of the greatest importance. 
 It has been shown that, in man also, the easily stimulated points are situ- 
 ated, in overwhelming majority in the anterior central convolution (Ca), that 
 the lower third belongs to the muscles of the head, the middle third to those 
 of the upper extremity, the upper third, together with the paracentral lobe, 
 to those of the lower extremity. 
 
 The posterior two-thirds of the inferior frontal convolution (possibly 
 
 Ccl4 
 
 ■R&£ 
 
 "Raf 
 
 Cca. 
 
 Fig. 130. — The corpus callosum and its radiations. By breaking off with a forceps, the; 
 radiation of the posterior end of the corpus callosum is indicated. Cc/4, Splenium; Tho, thalamus; 
 Tap, tapetum; C, ca corpus candicans; B, place where the cerebral peduncle is cut; Fcp, forceps) 
 IV, optic nerve; Raf, fornix; Rdf, Vicq d'Azyr stripe; Fli, fasciculus longit. inf. {After Edinger. 
 
 also the lower third of the middle frontal convolution) besides the adjacent 
 part of the lower fourth of the central convolution, together with the anterior 
 part of the island of Reil form the frontal speech region, the destruction of 
 which causes motor aphasia. 
 
 Focal injuries at the base of the frontal brain cause, if they destroy the 
 olfactory bulb and tract, inability to smell on the same side. 
 
 Destruction of the lower fourth of the anterior central convolution, alone, 
 causes paresis of the contralateral muscles of the tongue, palate, lips, cheeks 
 
476 
 
 DISEASES OF THE BRAIN 
 
 and of those used in mastication, which, however, as all these muscles are 
 innervated from both hemispheres, is not very serious, and (except for the 
 paralysis of the contralateral genioglossal muscle) is not permanent. Con- 
 sequently, only a moderate degree of dysarthria remains. 
 
 Serious paralysis of the muscles mentioned, on both sides, as well as of 
 those of the throat, hence, also anarthria or lasting serious dysarthria only 
 appear, when, at the same time, the corresponding area of the right hemi- 
 sphere is destroyed (cortical pseudo-bulbar paralysis). 
 
 p / OpR 
 
 Lope- 
 
 , ope re. 
 
 Gyn temp, prof 
 
 Fig. 131. — Sketch to explain the effect of variously situated foci in the left hemisphere. The 
 blue lines indicate that subcortical foci corresponding to the demarcated region cause the loss of 
 function concerned. 1, Motor aphasia (because of the illustration showing the third frontal 
 convolution in direct upward view and not shortened, the frontal speech region appears unpro- 
 portionately large); 2, paresis of the lingual, facial, masseter, deglutition and laryngeal muscles 
 on the opposite side (transitory except for the tongue muscles) ; 3, paralysis of the arm and the 
 hand; 4, paralysis of the leg; 5, 6, 7, disturbances of sensibility in the face, arm, leg (in 6 also 
 paralysis of touch) ; 8, if destroyed bilaterally, deafness — if on the left side, pure word deafness (?) ; 
 9, sensory aphasia; 10, near to the convexity, alexia and agraphia — if deeper near the medial 
 region, pure alexia; n, near it, amnestic aphasia — deeper, apraxia; 12, amnestic aphasia; 13, if 
 bilateral, mental blindness (visual agnosia) which, however, may occur also because of various 
 other combinations of foci, besides amnestic (especially optic) aphasia; 14, deviation conjuguee; 
 15, insular aphasia (blue) . P.tr., pars triangulars of the third frontal convolution; P.O., pars orbit- 
 alis of the third frontal convolution; P.operc, pars opercularis of the third frontal convolution; 
 Op.R., operculum Rolandi; S.h., S.a., S.p., ramus horizontalis, ascendens, posterior fossas sylvii. 
 
 Lesions in the first and second frontal convolution may disturb, some 
 say, equilibrium in walking, as do lesions in the cerebellum (frontal brain 
 ataxia). 
 
 Regarding a center for eye movement, to be found in the frontal brain, 
 see page 478. 
 
 The central two-fourths of the anterior central convolution contain, 
 first of all, the motor center for the upper extremity on the other side. From 
 
LOCALIZATION IN THE LEFTJ HEMISPHERE 477 
 
 above downwards occur in natural sequence the centers for shoulder, arm, 
 hand, finger. 
 
 The upper fourth of the anterior central convolution, and the anterior 
 part of the paracentral lobule lying on the median surface contain the motor 
 center for the legs. The posterior central convolution, and a part of the 
 adjacent superior parietal lobe, is the seat of sensibility of the opposite half 
 of the body (arranged from above down, like those of motility) and the 
 posterior central convolution is preeminently the seat of perceptions of 
 movement and position, of the sense of place and space and the corresponding 
 concepts. These perceptions and representations reach over also into the 
 principally motor area of the anterior central convolution (Fig. 131). 
 
 In contradistinction to this, the area for touch and pain sensations 
 extends over the posterior central convolution, backwards, at least in the front 
 parts of the superior parietal lobe. Destruction of the middle third of the 
 posterior central convolution and the directly adjacent part of the parietal 
 lobe produces paralysis of touch. 
 
 The middle third of the superior temporal convolution, together with the 
 transverse convolution of the temporal lobe (gyrus transversus or profundus) 
 represent the auditory center, the destruction of which, because of the demi- 
 decussation of the acoustic fibres causes deafness, only when it is bilateral. 
 
 Destruction of the third of the superior temporal convolution, lying 
 behind it, and of the anterior adjacent parts of the supramarginal convolu- 
 tion causes sensory aphasia. Lesions in the angular gyrus cause disturbances 
 in reading and writing with difficulty in finding words. 
 
 Lesions in the depths of the angular gyrus near the median and basal 
 surface of the brain cause merely disturbances in reading and difficulty in 
 finding words. 
 
 Large lesions in the white matter of the supramarginal gyrus, of the angu- 
 lar gyrus, and possibly also in the white matter of the upper parietal lobe 
 cause, in addition to these, apraxic disturbances. See page 544. 
 
 Lesions at the base of the posterior half of the temporal lobe and the 
 adjacent parts of the occipital lobe cause particularly great difficulty in 
 finding words (amnestic aphasia) (Fig. 132). 
 
 Focal injuries in the cortex and white matter of the convexity of the occip- 
 ital lobe affect visual memories, the appreciation of forms and spatial rela- 
 tions and the assimilation of visual sensations. In order to cause mind 
 blindness, a left-sided focal injury will suffice in some people; in the majority, 
 however, a bilateral occipital focal injury is necessary to cause serious mind 
 blindness. 
 
 Focal injuries on the median surface of the occipital lobe, which destroy 
 the vicinity of the calcarine fissure, cuneus, lingual gyrus, and, at the base 
 the fusiform gyrus, cause half-blindness. 
 
 Focal injuries, which in the white matter of the occipital lobe or even of 
 
478 
 
 DISEASES OF THE BRAIN 
 
 the parietal lobe, include the optic radiation, cause half- or quarter- 
 blindness. 
 
 In the angular gyrus lies a projection center for the movements of both 
 eyes in the opposite direction. Irritation there causes a deviation of the 
 eyes towards the other side (the patient looks away from the side with the 
 focal injury), and frequently, simultaneous turning of the head. 
 
 Focal injuries, however, in the pons, between the oculomotor and abdu- 
 cens nuclei, cause paralysis of the " looking process" (Blicklaehmung), not 
 to be confused with deviation; the eyes can not be led across the middle line, 
 nor towards the side of the focal injury. 
 
 Fig. 132. — Foci on the median surface of the left hemisphere. 15 (red), Paralysis of the leg; 
 16/ disturbance of sensibility in the leg; 17, hemianopia towards the right side; 18 (in the white 
 matter) amnestic aphasia; 19, is said to produce disturbances in smelling. 
 
 Paralysis of the center of eye movement in the angular gyrus leads pos- 
 sibly, since from here simultaneously the inhibition of the antagonists takes 
 place, to preponderance of contralateral looks (the patient looks towards 
 the focally injured side). A second center of the eye muscles in the frontal 
 brain, which has been proved to exist in higher animals, is assumed by some 
 authors to exist in man, also, at the foot of the first and second frontal 
 convolution. 
 
 The hippocampal gyrus, uncus and hippocampus major are brought 
 into relation with the senses of smell and taste, with the latter, especially the 
 back part of the fornicatus gyrus. 
 
 Of the functions of the lenticular nucleus and caudate nucleus nothing 
 certain is known; injury of the optic thalamus with its appendages, as inter- 
 mediate station for all sensory stimulations, even for the higher perceptions, 
 
DIFFERENCES IN FUXCTIOX OF THE TWO HEMISPHERES 479 
 
 causes serious disturbances in sensibility. Destruction of the outer genicu- 
 late body, hemianopia. Destruction of one of the inner geniculate bodies 
 leads only to serious auditory disturbances, when the auditory path on the 
 opposite side is injured somewhere in its course. 
 
 8. Differences in Function of the Two Hemispheres 
 
 While for projection functions — movement, sensation, seeing, hearing, 
 tasting, smelling — the right hemisphere is naturally equal to the left in all 
 men, it takes a far smaller part in the higher mnemic and associative func- 
 tions. This holds good, at least, for right-handed people, ca. 90% of the 
 total. The opposite is true of the 4 to 5%, of left-handed people. Both 
 hemispheres are of equal importance in the ambidextrous, 5 to 6%. 
 
 The right hemisphere in the right-handed is so far from indispensable 
 for functions of speech, that destruction of the right frontal brain, the right 
 temporal lobe, the right angular gyrus, very seldom causes disturbances in 
 speech, writing or reading, so that these functions can be fully performed 
 without the right hemisphere. 
 
 On the other hand, intactness of the right hemisphere does not protect 
 persons who have suffered extensive lesions in the left speech area, from 
 lingering speech disturbances, that are often serious and permanent. In the 
 course of time, however, the right temporal lobe may vicariously represent 
 and take the place of the left, and, to a certain extent, obliterate the aphasic 
 disturbances. 
 
 Likewise the left hemisphere is more important for praxia; through lesions 
 in certain parts of the left hemisphere, not only the right, but also, to a cer- 
 tain extent, the left upper extremity, loses the power to perform movements 
 from memory, to imitate movements, etc. For the simpler manipulation 
 of objects, the memory movements of the right hemisphere suffice, as a rule. 
 Even for memories of form and color, precedence must be assigned to the 
 left hemisphere, and especially for higher association of all simpler memo- 
 ries, and the spontaneous awakening of memories. 
 
 "While lesions of the central convolutions and of the auditory, visual, 
 olfactory and gustatory areas in the right hemisphere cause identically the 
 same clinical symptoms of lost function on the left side, as those of the 
 corresponding parts of the left hemisphere cause on the right side, the right 
 frontal lobe and large parts of the right temporo-parietal lobe are marked 
 as "silent" parts of the brain (lesions of the occipital lobe are noticeable 
 through the interruption in the optic radiation usually resulting), by which 
 is meant, that mnemic-associative disturbances that are caused by focal 
 injuries in the corresponding areas on the left side, are only slightly notice- 
 able when the lesion is on the right side. Naturally with refinement of 
 methods, even the silent parts of the brain will begin to speak, and it must 
 
480 . DISEASES OF THE BRAIN 
 
 be assumed, that they, only in a far less degree, have a mechanism similar 
 to the corresponding parts of the left hemisphere. When added to left- 
 sided lesions, the corresponding right-sided lesions are clearly brought into 
 evidence even to-day. 
 
 9. Cerebellum 
 
 The cerebellum demands special attention. Cf. Figs. 114 and 115. 
 
 Of the morphologically important relations we emphasize only that the 
 cerebellum is connected with the rest of the central nervous system through 
 three pairs of crural connections : 
 
 1. Crura cerebelli ad cerebrum or ad corpora quadrigemina, anterior 
 crus cerebelli, called also superior cerebellar peduncle disappears, under the 
 quadrigeminal bodies. 
 
 2. Crura cerebelli ad pontem, middle cerebellar peduncles, leave the 
 cerebellum laterally, passing into the pons. 
 
 3. Crura cerebelli ad medullam oblongatam or restiform body. 
 Though the physiological relations are still obscure in many particulars, 
 
 the following statements may be assumed as probably true. 
 
 I. The cerebellum receives its own centripetal tracts, from the periphery 
 of the body, and sends out centrifugal stimulations to the spinal cord. 
 
 As centripetal must be named first of all : 
 
 1. The lateral cerebellar tract: arises in Clark's column of the spinal cord, 
 and passes through the restiform body into the cerebellum. 
 
 2. Gower's tract: arises in the lumbar portion of the spinal cord and 
 loses itself in the cerebellum. 
 
 3. Other centripetal stimulations come to the cerebellum from the 
 nucleus of the lateral tract and the crossed olive. 
 
 4. Stimulations from the vestibular nerve probably by the help of Deiter's 
 nucleus. 
 
 By the help of the centripetal tracts, the cerebellum receives messages 
 from the muscles of the trunk, neck, head, limbs (unconsciously persisting 
 analog of sensations of position and movement) , and from the labyrinth. 
 
 As centrifugal tracts the following must be first considered: 
 
 1. The cerebellar cortex tract (roof nucleus?), Deiter's nucleus, the 
 uncrossed anterior lateral funicular path. 
 
 2. The cerebellar cortex-dentate nucleus superior cerebellar peduncle — 
 red nucleus — Monakow's bundle. 
 
 3. Connections by means of Deiter's nucleus and posterior longitudinal 
 bundle with the nuclei for the eye muscles. 
 
 Through these centrifugal pathways muscles are influenced automatic- 
 ally, unconsciously. The cerebellum regulates automatically the holding 
 of the trunk, head, and eyes, especially in standing upright and walking. 
 This regulation of the cerebellum does not suffice, in man, to produce erect 
 
THE CEREBELLUM 481 
 
 walking; the action of the cerebrum is also essential, but it takes an impor- 
 tant part. 
 
 The cerebellum represents^/w^ an independent sense-motor organ, which 
 by its own centripetal tracts {different from the sensory tracts, that go to the 
 cerebrum, as the posterior columns, fillet, etc.), receives impressions from 
 the periphery, and by its own centrifugal tracts, makes use of these impres- 
 sions, by means of the muscles, particularly for the co-operation of the many 
 factors of equilibrium, that are concerned in standing upright and in walking. 
 
 II. But the cerebellum has (indirect) connections with the cortex of the 
 cerebrum, corticopetal as well as corticofugal. Through the middle cere- 
 bellar peduncle, the corticopetal tracts, after various interruptions, reach 
 the frontal cortex, from which the corticofugal tracts through the frontal 
 pontile tract and the pontile cerebellar tract reach the opposite hemisphere 
 of the cerebellum. 
 
 By means of this cortico-cerebello-cortical system, cerebellar sensations 
 come to be acted on in the cortex of the cerebrum which thus gains influence 
 over the cerebellum. When a frontal lobe is destroyed, the volume of the 
 opposite hemisphere of the cerebellum diminishes. 
 
 III. The cerebellum, through its superior peduncles and the red nucleus, 
 sends other stimuli to the cortex of the cerebrum, which this answers, in part 
 at least, through motor processes, without the assistance of the cerebellum, by 
 the descending motor tegmental tract, which arises from the red nucleus and 
 ends decussating in the lateral corona. 
 
 The main symptoms of disease of the cerebellum and its peduncles, or 
 of the tracts mentioned, are: 
 
 1. Cerebellar ataxia. Cf. page 506. 
 
 2. Subjective: vertigo. 
 
 3. Forced postures and forced movements, which are observed, either 
 as an effect of irritation, or as a symptom of loss of function, when the hemi- 
 spheres of the cerebellum and especially the middle cerebellar peduncle 
 are affected, as well as abnormal inability to move the eyes and nystagmus. 
 
 4. The muscles of the body of the same side are usually hypotonic (a 
 result of the loss of centripetal stimuli) . 
 
 5. Occasionally, in diseases of the cerebellum, the ability to carry out 
 antagonistic movements, in immediate succession (as pronation and supina- 
 tion) is lost (diadocokinesis). 
 
 6. In contrast to the tabetic the limbs of many patients suffering from 
 diseases of the cerebellum, after a little wavering, may persist for a remark- 
 ably long time in one position; at times even cataleptic symptoms appear. 
 
 7. A species of dysarthria that is occasionally observed (slow, "scan- 
 ning" speech), could, if not caused by pressure on the bulbar centers of 
 speech, arise from the loss of regulating influences from the cerebellum 
 (analogous to a diadocokinesis). 
 
 31 
 
482 DISEASES OF THE BRAIN 
 
 8. One-sided chorea appears in the case of focal injuries that irritate the 
 superior cerebellar peduncle and its connections. A host of other symp- 
 toms, observed in diseases of the cerebellum, especially diseases that com- 
 press the neighboring organs (tumors), arise from effects on the adjacent 
 pons, medulla oblongata, etc., as hemiplegia, spastic phenomena, vomiting 
 and looking palsy (Blicklaehmung). 
 
 Even the direct symptoms of cerebellar diseases are not always present 
 and are often transitory, because there may be compensation, in the course 
 of time, for the losses concerned. The cerebrum seems to be able to com- 
 pensate, to a great extent, for disturbances, that have arisen in the cere- 
 bellum. By far the most important and constant symptom is cerebellar 
 ataxia. 
 
 It is important to note that focal injuries in the cerebellum, as opposed 
 to those in the cerebrum, cause disturbances, in the majority of cases, on 
 the same side of the body. 
 
 10. Brain and Reflexes 
 
 In the first place, the brain has an inhibitory influence on a series of 
 reflexes of the other divisions of the central nervous system. This influ- 
 ence is evinced normally in the possibility of voluntarily checking certain 
 reflexes (coughing, breathing, closing the eyes at the approach of the hand, 
 etc.), furthermore, in the influence exerted on reflexes by mental disturbances 
 (breathing stopped by fear). In lesions of the brain, therefore, by loss of 
 inhibition, many reflexes are exaggerated, such as those of the tendons. 
 
 This, of course, is true only so long as a remnant of connection between 
 cerebrum and periphery is retained. When the connection is absolutely 
 broken — total division of the spinal cord — exactly those reflexes belonging to 
 the severed part of the spinal cord are abolished, but in a manner difficult, 
 as yet, to understand. A few authorities still maintain that in cases of a 
 total transverse lesion of the spinal cord above the lumbar swelling the knee- 
 jerks, which at first are abolished, return and are permanently exaggerated. 
 
 The brain not only influences spinal reflexes, but is itself the center for 
 certain reflexes. 
 
 1. Pupillary reflexes. 
 
 i. Light Reflex. — When light is thrown on the eye, the pupil of that eye 
 contracts (direct light reaction) . 
 
 At the same time, the pupil of the other eye contracts (consensual light 
 reaction) . 
 
 2. Convergence Reaction. — When looking at near objects (convergence and 
 accommodation reactions) both pupils contract, more than from light. This 
 must be conceived as a concomitant movement.- 
 
 3. Orbicular Phenomenon. — The contracting of the pupils that appears 
 
PUPILLARY REFLEXES 483 
 
 when the eyes are shut tightly, must also be regarded as a concomitant move- 
 ment. When this is checked — and a series of concurrent factors does 
 destroy the effect — this contracting becomes evident. 
 
 4. Pupillary Cortical Reflex. — Contraction of the pupil, from mere direc- 
 tion of the attention to a source of light that has not been fixed, appears only 
 in few individuals. 
 
 5. The asserted contracting of the pupil from a mere concept of a bright 
 object, has been proved doubtful by further investigation. 
 
 6. Psycho-reflex. — On the other hand, without doubt, a dilatation of 
 the pupils occurs, not only with all sorts of painful or vigorous stimulations 
 (especially of the skin, but also from noises) but with every psychic disturb- 
 ance (anxiety, terror) and energetic mental work or strongly concentrated 
 attention. 
 
 During the vivid conception of a dark object the dilatation of the pupil 
 appears as such a psycho-reflex, which, however, is independent of the 
 content of the concept (anything dark) and the result only of concentrated 
 attention. 
 
 In sleep maximum contraction of the pupils occurs. 
 
 Anatomical 
 
 The fibres of both optic nerves end, as we see, half crossed in the lateral 
 geniculate body and the pulvinar. Besides this, fibres reach the anterior 
 quadrigeminal bodies (pupillary fibres). Hence arises a connection with 
 the deeply situated nuclei of the oculomotor nerves, whence a stimulation 
 of the pupil fibres causes contraction of the pupil (Fig. 133). 
 
 The tracts of the pupillary light-reflex, as yet not wholly ascertained 
 anatomically (contraction due to light) are as follows : 
 
 1. Centripetal limb. Retina, optic nerve, optic tract, anterior quadri- 
 geminal bodies. 
 
 2. Central transmission. Anterior quadrigeminal bodies, nucleus of 
 the oculomotor nerve (probably not a direct connection) . 
 
 3. Centrifugal limb. Nucleus of the oculomotor nerve to the ciliary 
 ganglion and hence with the musculus sphincter pupillae. 
 
 Since in a strong light, both pupils contract (consensual contraction of 
 the unexposed eye), there must be a connection between each anterior 
 quadrigeminal body and both oculomotor nuclei. 
 
 The dilatation of the pupils may result from the innervation of the dila- 
 tator pupillae by the sympathetic nerve. 
 
 The center is Budge's centrum ciliospinale in the spinal cord, lying at 
 about the level of the origin of the first dorsal nerves. It receives its stimula- 
 tions from the skin through the posterior roots, also from other sources and 
 sends the innervation through rami communicantes to the superior cervical 
 
484 DISEASES OF THE BRAIN 
 
 ganglion of the sympathicus, hence to the Gasserian ganglion, and through 
 the ramus ophthalm. trigem. to the long ciliary branches and to the iris. 
 
 But dilatation of the pupils through the dilatator pupillae, that is through 
 the stimulation of the sympathetic nerve, plays a smaller part than was 
 formerly believed. The dilatation of the pupils in all vigorous sensory 
 irritations, in every psychic exertion and excitement, in concentration of 
 attention, energetic contraction of the muscles, etc., is caused especially by 
 inhibition of the sphincter center, i. e., by the oculomotor nerve. 
 
 The influence of psychic processes on the dilatation of the pupil — which 
 can easily be proved, for instance, in the case of simple intense recollection 
 by an apparatus for measuring differences — points to connections between 
 the cortex and the sphincter center (Cf), probably also with the ciliospinal 
 center. 
 
 Pathology of the Pupillary Reflexes 
 
 When a lesion destroys any part of the reflex arc, the pupillary reflex is 
 destroyed. The peculiar conditions, under which total loss of pupillary 
 contraction and dilatation, especially when exposed to light, slowness in 
 moving the pupils, hippus, hemianopic reaction of the pupils, myosis, my- 
 driasis, anisocoria (difference in the diameter) appear, are discussed in a 
 special chapter. (See also General Symptomalogy.) 
 
 Loss of the psychic-reflex alone, is found frequently in certain mental 
 diseases (juvenile idiocy). The assumption of a so-called "paradoxical" 
 pupillary reaction (dilatation in light), is founded in the majority of cases 
 upon a dilatation that quickly follows an unnoticed contraction. 
 
 II. Eyelid reflexes. 
 
 (a) Touch reflex: closure of the eye when the cornea or conjunctiva is 
 touched (trigeminal nerve, nucleus of the facial nerve and this nerve itself) . 
 It is absent in diseases of the pons, on both sides also in hysteria. 
 
 (b) Light reflex: closure of the lid on sudden exposure to strong light, 
 probably caused through the cortex (absent in cortical blindness) . 
 
 III. Palatal or choking reflex, as well as the reflex act of swallowing: 
 sensory divisions in the pneumogastric and glosso-phary ngeal nerves . Center : 
 the nucleus of the motor trigeminus, the pneumogastric, glosso-pharyngeal 
 and hypoglossal nerves. Centrifugal division: these nerves. 
 
 IV. Vomiting. — Sensory division: pneumogastric, glosso-pharyngeal, 
 splanchnic nerves. Center in the medulla. Motor division: pneumogas- 
 tric, splanchnic and phrenic nerves. 
 
 (a) Coughing and sneezing made possible through the fifth, ninth, tenth, 
 eleventh and twelfth nerves. 
 
 We may only suggest the importance of the medulla for the automatic 
 regulation of the heart and respiratory innervations, and of a vaso-con- 
 strictor center supposed to exist in the medulla oblongata. 
 
SECRETORY FUNCTIONS OF THE BRAIN 
 
 485 
 
 11. Secretory Functions of the Brain 
 
 1. For secreting the sweat, a center, including the spinal centers, is sup- 
 posed to be in the medulla oblongata. 
 
 2. Secretion of the Saliva.— There is in the medulla oblongata a reflex, 
 salivary center, which controls the chorda tympani, as well as the sympa- 
 thetic secretory nerves of the salivary glands (in the passing of the pons 
 into the medulla, in the tegmentum, the so-called nucleus salivatorius). In 
 bulbar paralysis, excessive flow of saliva occurs. 
 
 Dilatator gpAinctet- 
 
 Ggicitia 
 
 DitaTa-tor 
 
 Ggi- Suprem 
 $ymp. 
 
 'lc radiation 
 
 Fig. 133. — Simplified scheme of pupillary reflexes. Cf, Presumed centrifugal path from the 
 cortex to the center of the sphincter; Ggl. gen. lat., ganglion geniculatum laterale; N.N. III., 
 nucleus N. oculomot; N.III., nervus oculomot. 
 
 Even if the salivary secretion, caused by taste and sensory stimulations 
 of the oral cavity (trigeminous nerve) is a reflex of the medulla, the salivary 
 secretion that takes place at the sight, smell, and imagination of food (the 
 "mouth waters") must be called a cortical reflex. 
 
 3. Secretion of Tears.- — The subcortical reflex has its center in the medulla 
 oblongata. Centripetal fibres run, especially in the trigeminus, and other 
 sensory nerves, centrifugal in the facial nerve. The secretion of tears is 
 under cortical influence, as is proved by its dependence on psychic processes. 
 
 4. The medulla, through the splanchnic nerves, exerts also an influence 
 on the secretion of the liver (glycosuria in diabetic puncture). 
 
486 DISEASES OF THE BRAIN 
 
 II. LOCAL SYMPTOMATOLOGY 
 i. General Symptoms 
 
 i. Disturbances of consciousness. — In the first place there are various 
 degrees of a general diminution of consciousness. In coma—ior instance, 
 in an epileptic fit, or often, after an apoplectic attack — there is total loss of 
 consciousness. 
 
 The reflexes are altogether, or, for the most part, abolished. The limbs, 
 when held up, fall limply down, if left to themselves. The comatose patient 
 .can not be roused however strong the stimulus may be. 
 
 Slighter degrees of lessening of consciousness are called sopor and somno- 
 lence. The soporose patient is roused by strong stimuli but only for the 
 time being; the somnolent person, is rather easily aroused and capable of 
 reactions, but when left to himself, easily falls back into a drowsy condition, 
 in which most external occurrences have no existence for him. 
 
 Associated with these diminutions of consciousness, appear irritations 
 of and changes in the conscious processes. 
 
 These dimmings of consciousness, with their countless sense delusions, 
 and the ensuing motor restlessness, are designated as delirium. Disturb- 
 ance of consciousness together with sense delusions causes disorientation. 
 Delirium appears in meningitis, infectious diseases, in the course of alcohol- 
 ism, morphinism, and also after occlusion and other lesions of vessels. 
 
 Anxiousness, and torpidity (stupor), even with catatonic phenomena, 
 are observed in organic diseases of the brain. Other mental disturbances, 
 especially general weakness of memory (loss of or difficulty in rousing the 
 formerly associated mnemic possessions) , disturbance in the ability to notice 
 (inability to assimilate anything new into the memory), disturbances in 
 attention, judgment, feeling, volition, supersensitiveness, unnatural suspi- 
 cion, increased to prejudicial ideas, diminution of ethical motives, narrow- 
 ing of the circle of interest to the Ego, etc., general symptoms, which appear 
 frequently, as a result of chronic and diffuse disorders of the brain (arterio- 
 sclerosis, progressive paralysis, senile atrophy, also multiple sclerosis, tumors, 
 etc.), are described, in more detail, in the text-books of psychiatry. 
 
 2. Headache is a very ambiguous symptom, which, even when the pain 
 is clearly localized by the patient, does not at all warrant the conclusion 
 that the focal injury corresponds to the seat of pain. 
 
 3. Vertigo a word used by laymen in a very different sense — i. e., for a 
 mere fainting spell or a darkening of the field of vision — means: (1) That . 
 the patient's own body or even external objects seem to him to move, at 
 least there is disorientation regarding his posture, or the position of the 
 body itself, that is, subjective vertigo or (2), loss of equilibrium, that is, 
 objective vertigo. When severe the patient may fall. Vertigo, especially 
 
SYMPTOMATOLOGY OF CEREBRAL DISEASE 487 
 
 if slight or if subjective, is seen in several different conditions, i. e., anaemia 
 of the brain, general vascular changes, cerebral hemorrhage, etc. But the 
 higher degrees stand in particular relation to cerebellar and labyrinthine 
 diseases. (Cf. pages 481 and 15.) 
 
 4. Vomiting is* a symptom of local irritation in the medulla oblongata, 
 and is classed among the general symptoms only because this center is 
 easily stimulated to reaction by pressure and irritation in far distant parts of 
 the brain, or perhaps renexly, by disturbances of the sensory branches of 
 the dura. 
 
 Cerebral vomiting is distinctly characterized by its occurring without 
 nausea, the ease with which it occurs, and its occurrence when no nourish- 
 ment had been previously taken. It is often projectile. 
 
 5. Changes in the pulse, respiration, temperature. 
 
 Cerebral diseases can influence, in many ways, the action of the heart. 
 
 Slowing of the pulse, which frequently appears when there is a rapid in- 
 crease of pressure within the cavity of the cranium, by irritation of the 
 pneumogastric center in the medulla oblongata is of special importance. 
 
 Even in the absence of an infectious disease, a transient rise in tempera- 
 ture occurs after arterial occlusion, or cerebral hemorrhage or a paralytic 
 seizure, without any certainty whatever, as to what definite parts of the brain 
 are directly concerned. On the other hand, the temperature occasionally 
 falls to 96 degrees. 
 
 Of the many changes from normal respiration, which appear especially 
 in coma, the most important is the Cheyne-Stokes' respiration, which occurs 
 also in the final stages of heart and lung diseases and uraemia, and is a 
 "signum pessimum." It is marked by the following series of irregularly 
 interchanging phases: 1. A number (20-30) of increasingly superficial 
 breaths, following one another in quick succession. 2. Pause inbreathing. 
 3. Little by little breathing beginning again, at first slow and deep, but 
 gradually increasing in rapidity till phase 1 is reached. This sequence 
 recurs again and again. 
 
 6. Choked disc (merely a severe optic neuritis) presents ill-defined 
 boundaries of the turgid, reddish, and swollen papilla, with total dis- 
 appearance of its boundary, often with hemorrhage and white spots, 
 enlarged and tortuous veins, and constricted arteries; the vessels seem to 
 be broken off at the edge of the disc. Choked disc is a general symptom, 
 because it occurs in consequence of any space-restricting lesion anywhere 
 within the cranium (especially in tumors, internal hydrocephalus, abscesses) , 
 also in meningitis, less often in cerebral syphilis and lead poisoning and very 
 seldom in multiple sclerosis. It occurs most frequently with tumors of the 
 posterior cranial fossa. It is most frequently bilateral, even when appearing, 
 as it often does, on one side first. 
 
488 DISEASES OF THE BRAIN 
 
 2. Projection Disorders 
 
 Disorders in Motion and Sensation 
 
 We differentiate: 
 
 i . Projection Disorders. — They affect movement, sensation, and secretion. 
 Phenomena of irritation or loss of functions of receptor or effector organs. 
 
 2. Mnemic-associative Disturbances. — They affect those functions of the 
 brain which consist in storing up past stimulations, and in fixing their 
 connection. The associative work of the brain does not correspond to any 
 arrangements of the sense and muscle apparatus of the body, but to the 
 connections established by experience; so, for instance, the association between 
 the scent and the appearance of the rose, is not at all brought about by the 
 connection between the mucous membrane of the nose and the retina. 
 
 The projection disturbances are divided into motor, sensory and secre- 
 tory, and each of these groups, into symptoms of irritation and of loss. 
 
 (a) Motor Symptoms of Irritation 
 
 Cortical spasms. As the electric current, and all other irritants that 
 influence the motor cortical centers, cause contractions in the muscles of 
 the limbs on the other side of the body, so with pathological irritants (tumors, 
 hemorrhage, scars, etc.), spasms occur in the limbs. Usually the spasm 
 is first tonic, later clonic. 
 
 According to the place in the cortex that is affected by the irritation, 
 the muscles of the face, arm, or leg are the seat of the spasm. When the 
 irritation is lasting and extended, the spasm spreads from the region first 
 attacked to the other limb on the opposite side and it proceeds in such a 
 way, that the muscles are affected, in the order in which their cortical centers are 
 arranged, cf. page 475, so that the spasm that has started in the face, continues 
 first in the upper, then in the lower extremities. Finally, the spasm can pass 
 over to the same side of the body, in which the focal injury lies, whereby the 
 clinical picture of a general epileptic fit is produced. Even when the spasm 
 does not, universally, attack the other side, the usual bilateral muscle groups 
 (of the jaw, for breathing, etc. — viz., in what is otherwise one-sided spasm) 
 are also affected by twitching, even though they lie on the other side. 
 
 These cortical spasms, confined to one group of muscles, or to one side 
 of the body, or at least, beginning locally, are known as Jacksonian or cor- 
 tical epilepsy. They are contrasted with the convulsions of ordinary epilepsy, 
 which affect the whole body from the start. 
 
 After severe Jacksonian convulsions, paresis appears in the muscles 
 attacked, as a consequence of exhaustion, but retrogresses after a few hours, 
 at most. But if in the progress of the disease, as often happens, though the 
 effect of irritation is only temporary, the lesion causing the spasms, e. g., a 
 
MOTOR SYMPTOMS OF IRRITATION 489 
 
 neoplasm leads to destruction of the motor cortical center, permanent pa- 
 ralysis of those groups of muscles, which were formerly affected by the spasm, 
 appears. Together with the extremities, on the opposite sides, head and 
 eyes fall into a mostly tonic spasm. If the irritating focal injury lies towards 
 the left, both eyes and the head turn towards the right: conjugate deviation 
 (deviation conjugue). In this deviation, produced by an irritative cause, 
 the patient looks towards the side on which the limbs are spastic, contrary 
 to the deviation caused by paralysis. It is worthy of notice, that not only 
 the contralateral eye, but both eyes are affected by the effect of this irritation 
 which exists in one hemisphere alone. 
 
 This combination of tonic-clonic spasms in isolated groups of muscles 
 on one side, can occur, pathologically, only with cortical irritation. It 
 must be remembered, however, that focal injury not immediately in the center 
 concerned, hit adjacent to it, can cause the irritation. 
 
 Therefore spasm is of much less importance in the diagnosis of the 
 localization of disease than is palsy. Symptoms of lost function are of much 
 greater surety, for local diagnosis, than those of irritation. 
 
 General spasms, and especially, tonic spasms can also be discharged 
 from deeper lying subcortical centers. In irritation of cortical motor centers 
 (Jacksonian epilepsy), the characteristics are: spasmodic contractions, con- 
 fined to a group of muscles, or at most, to one side, the change from tonic to 
 clonic, and its spreading according to the location of the centers for indi- 
 vidual limbs, in the anterior central convolution. 
 
 Choreic Twitchings . Hemichorea 
 
 Movements of the limbs much resembling those of Saint Vitus dance in 
 children and parturient women, appear in focal injuries, on one side, in par- 
 etic limbs (often long after the paralysis), or sometimes precede, by a con- 
 siderable time a paresis or paralysis. {Hemichorea.) If, as is usual, they 
 appear in hemiplegic limbs, we have posthemiplegic chorea. The affected 
 limbs are often hypotonic. The hemichorea is sometimes accompanied by 
 pain. The kind of disturbance in movement varies in different cases, 
 sometimes resembling mere tremor, at others athetosis. 
 
 As to the anatomical basis of hemichorea, as a rule focal injuries in the 
 subthalamic region (red nucleus and its connections with the cerebellum 
 (superior cerebellar peduncle) and the optic thalamus) seem to be the cause. 
 
 Athetosis, a mostly unilateral, unceasing, involuntary movement, 
 appearing in paralyzed limbs, abating only in sleep. It consists in slow 
 spreading, adducting, flexing, and extending movements, especially of the 
 fingers and toes. (Athetosis of the facial muscles is of rare occurrence.) 
 Over-extension of the fingers is conspicuous. Athetosis appears most 
 frequently in cerebral infantile paralysis, but not until long after the onset 
 
49o DISEASES OF THE BRAIN 
 
 of the paralysis, and when it has, to a certain extent, retrogressed. Athetosis, 
 too, seems to be caused by focal injuries in the sensory tracts leading to the 
 thalamus. It may appear, exceptionally, with diplegic paralysis, in both 
 halves of the body. 
 
 The so-called double athetosis (Athetose double) is a disease sui generis, 
 which appears symmetrically on both sides without preceding paralysis 
 from an unknown cause and without anatomic concomitants. 
 
 Unilateral tremor is found occasionally in hemiparetic limbs. In 
 many cases it resembles perfectly the marked tremor of paralysis agitans. 
 
 Clonic-tonic twitchings of the palatal muscles, 150 to 200 in the minute, 
 have been observed, especially with pseudo-bulbar symptoms, in hemiplegic 
 patients, and seem to have been traced back to injuries in the vicinity of the 
 red nucleus. 
 
 Associated movements are involuntary movements appearing in other 
 limbs or in other muscles of the same limb while this limb is being moved 
 purposely or even moving reflexly. 
 
 They appear especially in the paralyzed limbs, when strong movements 
 occur in the sound limbs. But also in the sound limbs, when an attempt 
 is made to innervate the paretic ones. Thus, when the sound hand is 
 energetically closed, an impulse discharges itself into the paralyzed hand, 
 which can not voluntarily be closed, and it closes or an unsuccessful attempt 
 to close the paralyzed hand has the effect of making the sound hand into a 
 fist. With sneezing and yawning a paralyzed limb sometimes extends or 
 flexes. 
 
 StrumpelVs tibialis phenomenon; strong dorsal flexion of the foot while 
 the hemiplegic leg is being drawn toward the body, is an associated move- 
 ment of another group of muscles of the same limb. Even in passive move- 
 ments of the sound limb, the paretic limb may sometimes carry on similar 
 movements. 
 
 In paralytics, chronic alcoholics, idiots, and aphasics, we often find, 
 when they are speaking associated movements of the facial muscles, especially 
 of the forehead. 
 
 Contractures will be discussed later on with the paralyses. See page 
 493- 
 
 (b) Sensory Irritation Symptoms 
 
 In focal injuries, that irritate the sensory tracts (see below), constant, 
 or intermittent violent pains may appear, which, to distinguish them from 
 the usual pains produced by peripheral irritation, are called "central pains." 
 These pains, sometimes combined with a feeling of heat, are felt in the 
 opposite side of the body, or it may be, too, in a single limb, arm, or in the 
 face, tongue, etc. Besides pains, parassthesias of various kinds, formication, 
 
SENSORY IRRITATION, PARALYSIS 491 
 
 thermic sensations, etc., may appear. Unilateral sensations of cold of 
 sudden onset have been noticed, and sensations of movement, without 
 objective movement. 
 
 Sensibility may remain undisturbed in the presence of these central pains; 
 but pains may appear in regions insensitive to external irritation — anaesthesia 
 dolorosa. 
 
 Sometimes, hemichoreic twitchings, produced by focal injuries, are 
 combined with central pains. 
 
 As irritation phenomena, we may regard the hyperesthesias also: even a 
 slight touch, or a slight thermic stimulus is painful, as well as the paresthesias 
 due to touch alone: strange, "funny" sensations. 
 
 Central pains and parsesthesias may be caused by focal injuries in every 
 part of the sensory system, even in the cortex and corona radiata, more 
 frequently by those in the thalamus and subthalamic region. Therefore 
 they have no localizing value. 
 
 In the territory of the special senses (sight, hearing, etc.), stimulation 
 phenomena appear in the form of elementary sensations, the seeing of light, 
 flames, colors, the hearing of humming, ringing and rustling, and of complex 
 hallucinations. Thus disease in the occipital lobe, may cause visual hallu- 
 cinations in the hemianopic half of the field of vision. 
 
 (c) Motor Symptoms of Loss of Function 
 1. Paralyses 
 
 Soon after a grave apoplectic seizure, the so-called initial flaccid paralysis 
 appears; the passively raised limbs fall as if lifeless. 
 
 The reflexes are lost at first, but soon a certain tonus appears in the 
 muscles, the tendon reflexes return and are soon exaggerated, the Babinski 
 plantar reflex appears. The stage of flaccid paralysis usually lasts only a 
 few days, rarely, a few weeks. In some muscles a certain degree of motility 
 returns, e. g., in those which flex the arm and fingers, and now gradually the 
 flaccid paralysis passes over into the permanent condition of residuary 
 hemiplegia, to be described immediately. 
 
 In residuary hemiplegia, it is not, as after complete division of nerves, a 
 question of paralysis in the strictest sense. The muscles are not as in the 
 case of nerve division robbed of all tonicity, nor are they incapable of reflex 
 movements. On the contrary, in part of the muscles hypertonia appears 
 and the tendon reflexes are 'exaggerated. 
 
 The behavior of the reflexes, discussed on page 64, will be only touched 
 upon here. 
 
 In cerebral paralyses a few days after the attack we find exaggeration of 
 the tendon reflexes and mechanical muscular irritability. Often patellar 
 and ankle clonus, less frequently hand and masseter clonus are found. Tap- 
 ping on many other tendons (periosteal spots) and muscles, results in in- 
 
492 DISEASES OF THE BRAIN 
 
 creased contractions (supinator, triceps, radius-periosteal reflexes, etc.). 
 In a certain but not in absolute contrast to the tendon reflexes stand certain 
 skin reflexes. The abdominal and cremasteric reflexes are generally 
 abolished on the paralyzed side. But one must consider, that, as a matter of 
 fact, even the former can not always be obtained in healthy people, and that 
 therefore, only a one-sided loss is significant. (In long-standing hemiplegia 
 the knee-jerk may be greatly increased on the unparalyzed side.) 
 
 If exaggeration of the reflexes should, exceptionally, fail to appear, either 
 a concomitant disease of the posterior column (tabes) or neuritis is the cause. 
 
 A frequent symptom — in 70-80% of cerebral paralyses — is the Babinski 
 plantar phenomenon (see page 69). Oppenheim's dorsal leg phenomenon 
 (see page 69) and Kurt MendeVs reflex (plantar flexion of the toes instead of 
 the normal dorsal flexion, when the dorsum of the foot is tapped, near the 
 third and fourth metatarsal bones) are frequent signs of paralysis caused by 
 lesion in the cortico-spinal path. 
 
 In the hemiplegic limbs, all muscles, to a certain degree, are weakened, 
 but some groups of muscles, the so-called preferred muscles of hemiplegia 
 remain strongly affected while the others recover somewhat. These pre- 
 ferred muscles belong to definite mechanisms. The peculiarity of all cerebral 
 paralyses, is, that not single muscles, but entire muscular mechanisms, i. e., 
 many muscles working together, called synergies, are paralyzed. One must 
 consider, that a simple movement (such as extending the index finger) is not 
 the work of a single muscle. Antagonists are involved as well as collateral 
 and rotatory synergists. 
 
 The mechanisms particularly injured in cerebral paralysis are, in regard 
 to the leg, all the mechanisms which help to retract the leg, especially those 
 which bend the knee and the dorsal flexors of the foot. Because of the weak- 
 ness of the dorsal flexion, the tip of the foot often drags on the ground and 
 the leg is lengthened. The extensors of the leg are retained, especially those 
 of the leg below the knee and the plantar flexors of the foot. Therefore, the 
 leg may be used stilt-like in walking. In any case, the leg is less seriously 
 affected than the arm. The glutasus medius is often paralyzed, which fixes 
 the pelvis against the thigh. Consequently in walking, the pelvis falls towards 
 the unparalyzed side. In order to compensate for this, the hemiplegic 
 patient, every time he steps on the paralyzed side, throws his trunk towards 
 that side. Hence occurs the peculiar sideward movement of the trunk in 
 walking, found in many hemiplegics. In double paralysis of the gluteus 
 medius — a rare phenomenon of cerebral infantile paralysis — a waddling 
 walk results. The body, at every step, sinks to the side of the standing leg. 
 
 In the upper extremities, the muscles that open the hand, and rotate it 
 and the arm outward, are more seriously paralyzed, while those of opposite 
 action, closing the hand, and inward rotation of the arm, are less seriously 
 affected. 
 
PARALYSIS; CONTRACTURES; HYPERTONIA 493 
 
 The raising of the shoulder (M. cucullaris and levator scapulae) is seri- 
 ously affected. The sternocleido-mastoid muscle, on the contrary, is always 
 unaffected. For both upper and lower extremities, this rule holds good, that 
 the distal parts are always more seriously affected, i. e., hand and foot, and 
 especially the differentiated movements of fingers and toes, the isolated move- 
 ments in the small joints. In contrast to these, there is retained ability to 
 make a few gross changes in the position of the entire limb. 
 
 The frontal ocular branch of the facial nerve is spared (usually, however, 
 the eye on the paralyzed side can no longer be closed alone), whereas the 
 branch running to the mouth and cheek is clearly paretic. (An important dif- 
 ference from the peripheral paralysis of the seventh nerve.) The mouth is 
 drawn to the unaffected side. 
 
 The tongue, when stuck out, turns to the paralyzed side, because the 
 sound geniohyoglossus presses to the other side, but for the rest, is sufficiently 
 mobile. 
 
 Symmetrically working muscles, on the two sides of the body, which, as is 
 well known, may be innervated by each hemisphere individually (muscles of 
 the eye, back, larynx, of chewing and swallowing) are unaffected or almost 
 unaffected in hemiplegia. 
 
 The bilaterally working speech muscles, too (of the tongue, palate, face), 
 are not so deprived of function by one-sided focal injury, that speech is per- 
 manently lost. Serious dysarthria, for the most part, appears only as a transi- 
 tory phenomenon, after one-sided injuries and only when they are left-sided. 
 Lasting anarthria or serious lasting dysarthria, appear almost only in 
 bilateral focal injuries, in contrast to aphasia, which appears only after lesions 
 on the left side. 
 
 2. Contractures and Hypertonia 
 
 A further factor in restricting motion (myogenic contracture) is added to 
 actual muscular weakness in hemiplegia, after the initial flaccid palsy has 
 passed off. 
 
 By this one understands the fixation of limbs in a definite position by the 
 occurrence of involuntary permanent shortening of muscles. 
 
 These myogenic contractures are to be distinguished from the contrac- 
 tures due to cutaneous scars, ankylosis, or shortening of ligaments or 
 tendons. 
 
 The normal tension of the muscles, fixes in a certain measure, in healthy 
 conditions, the limbs in their positions at all times, so that in sudden and 
 quick passive movement of a limb, a moderate resistance of the stretched 
 muscle is felt. This normal resistance is lacking in some nervous diseases, 
 especially in tabes. Then we speak of increased passive motility or of 
 hypotonia. 
 
494 DISEASES OF THE BRAIN 
 
 On the contrary, the resistance to passive movement, in diseases of the 
 pyramidal tracts, is very much increased : hypertonia of certain muscles and, 
 therefore, fixation in definite positions appears. 
 
 In this fixation, there is a second factor; frequently there appears besides 
 hypertonia, connective-tissue contraction. Contraction of the connective tissue 
 of a muscle appears, when its points of insertion are not separated from one 
 another for a long time, therefore, in the antagonists of a paralyzed muscle, 
 even if 'it is not in the condition of hypertonia. Shrinking contracture, there- 
 fore, appears also in flaccid paralyses, as, for instance, in poliomyelitis. 
 
 In spastic -paralyses, which have existed for a long time, there is added 
 this shrinking contracture to the hypertonic or spastic contractures. 
 
 These shrinking contractures must, therefore, be differentiated from the 
 spastic, which are conditioned not by tissue changes, but by nervous hyper- 
 tonia. This hypertonia exhibits itself in hemiparetic limbs, even when it 
 does not go so far as contracture, by increased spring-like resistance on 
 sudden passive flexion or extension, and corresponds to the spasm, which 
 appears with the patient's own movements. The reflexes are exaggerated. 
 
 Sometimes one sees, either at once or soon after a cerebral injury occurs 
 (hematoma of the dura or trauma, meningitis), spasmodic, rigid, abnormal 
 attitudes and positions of the limbs, which, unfortunately, have been called 
 early contractures. They usually disappear again and alternate with tonic- 
 clonic spasms. They are conditions of irritation, better included under tonic 
 spasms. If one speaks broadly of contractures, one does not mean these 
 transient increases of tension caused by special irritation, but permanent 
 conditions of contracture, which appear as a permanent symptom in from 
 a few days to one or two weeks, after apoplexy, resulting from a lesion in any 
 part of the entire motor cortico-spinal system, that is, from the cortex of the 
 anterior cerebral convolution to, but not including, the anterior cornu cells. 
 
 Unless therapeutic preventative measures are taken in these cerebro- 
 spinal paralyses, a shrinking contracture in the course of time is added to 
 the spastic contracture. 
 
 Spastic contractures are relaxed in narcosis, in deep sleep, under the effect 
 of constriction in Esmarch's bandaging of the affected limb, and also partly 
 in a warm bath. 
 
 Naturally shrinking contracture can not, under these circumstances, be 
 relaxed, and thereby the part that hypotonia and shrinking play in contrac- 
 ture, can easily be determined. Mental excitement increases spastic con- 
 tracture. Spastic contracture shows a spring-like resistance against attempts 
 to cause relaxation. It is violent in repelling sudden brusque attempts; 
 on the other hand, many contractures can be easily relaxed by gradual 
 procedure. Attempts at movement both in the affected and the unaffected 
 limbs, increase the contracture. 
 
 The characteristic positions of the limbs of patients suffering from mono- 
 
CONTRACTURES 495 
 
 hemi-, and diplegia, appear only because of differences in the degree of 
 contracture in the various groups of muscles. 
 
 There is, indeed, absolutely no regular form of hemiplegic contracture, 
 but one type is the most frequent. On the whole, we find contracture in those 
 muscles that are relatively free from paralysis (cf. page 492), that is, the 
 antagonists of the "predilection" muscles. The typical position of the 
 hemiplegic arm corresponds, therefore, to the power retained by the flexors 
 of the hand, the inner rotators and flexors of the arm : the fingers are closed, 
 the thumb turned in or adducted, the arm lies close to the breast, bent at 
 the elbow, the leg in extension, the foot in the position of equino-varus. 
 Because of the lengthening of the leg by the plantar contracture or weakness 
 of dorsal flexion, the leg in walking is moved in a circle (circumducted). 
 This is the usual distribution of contractures, in which generally, the muscles 
 that may still be somewhat moved at will, are contracted. Sometimes, 
 but more seldom the opposite takes place: flexion and contraction of the 
 leg, extension and contraction of the arm and fingers. 
 
 The distribution of contractures and the corresponding permanent posi- 
 tion of the paralyzed limbs, certainly depend on the fact that certain muscles 
 are relatively spared. But only indirectly, through the fact, that the return 
 of a certain power in single groups of muscles is decisive for the position of 
 the limb in the first weeks after the paralysis occurs. In the final issue, the 
 position assumed by the limb during the time of relaxed and semi-relaxed 
 paralysis determines the distribution of contractures. Each group of 
 muscles is inclined in pyramidal disease to adapt itself to an approach of the 
 insertion points, brought about by any cause whatsoever, through in- 
 crease in tension and shortening. This is a subcortical reflex, which ap- 
 pears only after the "isolation" of the subcortical centers (and besides de- 
 pends on the intactness of centripetal pathways, hence is missing when 
 tabes exists). 
 
 That this assumption of the dependence of contractures on the position 
 taken by the limb after paralysis, is correct, is proved by the fact that 
 one is able, by artificial fixation of a limb, to change and transform an 
 already existing flexion contracture into an extension contracture and 
 vice versa. 
 
 The return of power to certain groups of muscles (the antagonists of the 
 preferred (predilection) muscles) seems, therefore, to lead to contracture 
 only by causing the choice of a certain position of the limbs in the first weeks 
 after the paralysis. Hence it happens that, if, by external conditions, the 
 limb is prevented from following this influence, the distribution of contrac- 
 ture is changed, and in this way mainly can we account for the various 
 modifications of the above described typical position of the limbs in 
 hemiplegia. 
 
496 
 
 DISEASES OF THE BRAIN 
 
 (3) Differences in Cerebral Paralysis According to the Situation of the Lesion 
 
 (a) Cortical Focal Lesions " 
 
 The fact that the motor paths in the inner capsule and the cerebral 
 peduncle are placed closely side by side, on the one hand, and the fact that 
 
 Sensory tract- — — \ — 
 
 Fig. 134. — The principal segments of the internal capsule. Bkn, Genu of the corpus callosum; 
 Vh, anterior horn; Knee ic, genu of the inner capsule; / optic, lenticulo-optical section of the 
 internal capsule; CA, cornu ammoni; Hh, posterior horn; calc, fissura calcarina; .F3, third frontal 
 convolution; sta, corona radiata; I sir ic, lenticulo-striate section of the internal capsule; c ext, 
 capsula externa; Z, island of Reil; Operc, operculum; CI, claustrum; Ti, first temporal convolu- 
 tion; r lie, retro-lenticular section of the internal capsule; Ss, optic radiations; T2, second temporal 
 convolution. (After v. Monakow.) 
 
 they are separated in the white matter, corresponding to the various parts 
 of the anterior central convolution, from which they arise, on the other hand, 
 
CORTICAL FOCAL LESIONS 
 
 497 
 
 cause a difference in the effects of focal injuries, that affect the cortex and 
 
 parts of the adjacent white matter from those focal injuries affecting the 
 
 inner capsule, or the cerebral 
 
 peduncle, and, of course, the 
 
 still deeper lying planes of 
 
 the motor path. The paths 
 
 for the leg come from the 
 
 paracentral lobule and the 
 
 upper fourth of the anterior 
 
 central convolution, those 
 
 for the arm and hand from 
 
 the middle two-fourths, 
 
 those for the face, tongue, 
 
 masticatory muscles, from 
 
 the lower fourth of the same 
 
 convolution. 
 
 Focal injuries in or near 
 the cortex, of moderate ex- 
 tent, paralyze only arm or 
 leg or face, that is, cause so- 
 called monoplegias, whereas 
 focal injuries more deeply 
 situated paralyze most fre- 
 quently the entire contralat- 
 eral side, causing hemi- 
 plegia. 
 
 Monoplegia of the face is 
 often combined with that of 
 the arm: monoplegia facio- 
 linguo-brachialis; that of the 
 leg, often with that of the 
 arm, monoplegia brachio- 
 cruralis. 
 
 Ant. pyramid Col. 
 
 Fig. 135. — Scheme for the explanation of hemiplegia 
 alternans (for the seventh and twelfth nerves). Red: 
 pyramidal tract. N .VIIs, Nerv. facialis sinister; N. 
 A lesion entirely in the XIId > right hypoglossal nerve. I, Focus in the right 
 
 internal capsule, left half of the body, left seventh and 
 paracentral lobule Causes twelfth nerves affected; II, in the right peduncle, the 
 merelv -bnrnU^^ nf ih? Ua same; HI > focus m the rf ght half of the pons, left half of 
 
 merely paralysis oj me leg. me body and left twelfth nerVG) right sev £ nth ' nerve (sup _ 
 
 As the paracentral lobule is ranuclearly), affected; IV, in the same place caudad, left 
 
 ,. , , . half of the body, left half of the tongue and right seventh 
 
 Supplied by another artery nerve affected (nuclearly resp. radicularly); V, focus in the 
 
 than the central convolution, Z^\lT lYZts ^\ M \ haH °i th ? W and the right 
 
 » nali of the tongue (nuclearly or radicularly) . 
 
 namely the corpus callosum 
 
 artery, this isolated paralysis, combined with softening of the corpus callosum 
 
 occurs when the above-named artery is occluded. 
 
 32 
 
498 DISEASES OF THE BRAIN 
 
 Therefore the characteristics of cortical focal injuries, or of focal injuries 
 lying near the cortex are : 
 
 i. The paralysis is confined to one or two members (arm, leg, face). 
 
 2. Localized Jacksonian convulsions. 
 
 In lesions of the central convolutions, inner capsule, etc., the muscles 
 of the eye are not affected. For conjugate deviation see page 478. . 
 
 (/?) Capsular Focal Lesions 
 
 In the internal capsule, all the motor paths are easily stricken together; 
 they lie back of the so-called knee in the front third of the posterior limb. 
 
 The order of fibre bundles, from the knee backwards, is as follows: 
 
 Facial fibres, tongue fibres, arm fibres, leg fibres (Fig. 134). The fibres 
 are so indistinctly separated that a monoplegic paralysis through capsular 
 focal injury is exceedingly rare. It has been observed that the leg is mostly 
 affected by an injury situated in the hind part, the face by one in the front. 
 
 Focal injuries in the cortex and the white matter differ in one respect, 
 that has long been disregarded, from those in the inner capsule and still 
 deeper lying parts of the pyramidal tract. The former affect the fibrillation 
 of the hemisphere, where it contains closely mixed, projection fibres and 
 corpus callosum fibres, destroy therefore besides these, countless connections 
 with the other hemisphere. On the contrary, focal injuries in the inner 
 capsule, in the peduncle, etc., destroy no commissural fibres and therefore 
 leave the harmonious co-operation of the two hemispheres undisturbed. A 
 consequence of this difference we shall learn later on under apraxia. 
 
 (f) Focal Lesions in the Peduncle and Pons. A Iternate Paralyses 
 
 If a focal injury affects the motor path in the peduncle, where it is situated 
 in the middle third of its foot, the result for the extremities is the same as if 
 there were a break in the inner capsule. 
 
 But for the cerebral nerves here and in the pons, certain peculiarities 
 occur. Whereas the pyramidal tracts for the extremities do not cross until 
 the medulla is reached, the corresponding paths for the cerebral nerves 
 pass already in the peduncular layers or pontile layers to the nuclei of the 
 nerves concerned on the other side (third, fourth, fifth, sixth and seventh 
 nerves). Such a focal injury in the left peduncle may strike the entire 
 cortico-spinal path for the right extremities, and also the nucleus or root 
 of the left oculomotor nerve. Consequently to the spastic paralysis of the 
 right limbs and the right seventh nerve, is added a nuclear or peripheral 
 paralysis of the left oculomotor nerve (ptosis, internus, paralysis, etc.). 
 This is called hemiplegia alternans (alternate hemiplegia) . 
 
 The same thing, moreover, can take place in the pons for the trigeminus, 
 abducens, and facial nerves. Then to the paralysis of the limbs of one side 
 is added paralysis of the trigeminus, abducens or facial nerve, on the other 
 
CORTICAL FOCAL LESIONS, VOLUME OF MUSCLES 
 
 499 
 
 side, and moreover, when nucleus or root is affected, the paralysis is of a 
 peripheral character. It may happen, however, that only the supranuclear 
 bundles of the central neuron are affected. 
 
 Because of the relation of the nuclei to the pyramidal tracts, many 
 combinations are possible. For instance, when the focal injury crosses the 
 middle line, both seventh nerves or both twelfth nerves may be stricken by 
 supranuclear paralysis, or one by supranuclear and the other by -tmclear 
 paralysis besides a hemiplegia (Fig. 135). 
 
 Ftllet 
 
 Red nucleus " m - 1 "^ w * aiB * e *^, 
 
 Fig. 136. — Hemiplegia alternans superior. The focus H interrupts the right pyramidal 
 tract 2 and the right oculomotor root N.III and produces therefore paralysis of the left extremities 
 and of the right eye. 
 
 The combination of hemiplegia with crossed oculomotor paralysis 
 (peduncular focal injury) is known as Weber's paralysis or hemiplegia 
 alternans superior. 
 
 Hemiplegia with crossed paralysis of the facial or abducens nerve must 
 result from a focal injury in the pons (Gubler's paralysis). Hemiplegia 
 alternans inferior. 
 
 In the medulla, a focal injury may strike the as yet uncrossed pyramidal 
 tract of the one side, and the root of the hypoglossal nerve of the same side; 
 then the limbs are spastically paralyzed on one side and the tongue atrophic- 
 ally paralyzed on the other (hemiplegia alternans infima). 
 
 Just as cerebral motor nerves crossed to the extremities, can be paralyzed 
 by focal injuries in the peduncle and pons, there can be disturbances in the 
 crossed sensory nerves, of such kind, that with the pyramidal tract of the right 
 half of the pons, the right root of the trigeminal nerve is affected, hence 
 with paralysis of the left side of the body, disturbances in sensibility in the 
 right side of the face may appear (Fig. 137). 
 
 These interchanging paralyses of the cerebral nerves are important local 
 symptoms of lesions in the peduncle and pons. 
 
 Volume of Muscles 
 
 In contradistinction to nuclear and peripheral paralyses, the muscles 
 in cerebral paralysis retain, at first, their volume and gradually atrophy at a 
 
5oo 
 
 DISEASES OF THE BRAIN 
 
 rate corresponding to their remaining unused. Atrophy is not so marked 
 as in nuclear and peripheral palsy. Likewise electric reaction is not quali- 
 tatively changed in muscles and nerves, and quantitatively is but slightly 
 lowered. Exceptions are rare (atrophy and electric changes). Their 
 cause is not known. 
 
 Vasomotor and trophic disturbances are observed in hemiplegic and 
 sometimes in merely hemiansesthetic limbs; changes in temperature, cyano- 
 sis, edema, joint diseases, skin diseases, etc., also occur. 
 
 NIV. HL 
 
 N.IV 
 Aq 
 
 LSchl 
 
 Vw 
 
 Fig. 137. — Relation of the trigeminal to the pyramidal tract in the pons. A focus, which 
 strikes the root of the fifth nerve and the pyramidal tract Py, produces synchronous paralysis of 
 sensibility in the face and crossed hemiplegia. Sch.m and Sch.l., middle and lateral parts of the 
 principal fillet; L.Schl, lateral or lower fillet; fr, forma no reticularis; BA, superior peduncle; HL, 
 posterior longitudinal bundle; N.IV, trochlearis, Vw, root of the trigeminal. (After v. Monakow.) 
 
 Bilateral focal injuries cause bilateral hemiplegia — diplegia. Focal 
 injuries that strike the paracentral lobules only, paralyze both legs (cerebral 
 paraplegia) . 
 
 (d) Sensory Symptoms of Lost Function 
 
 General sensation includes various qualities : 
 
 1. Tactile sensations. 1 
 
 2. Sensations of pain (loss: analgesia or 
 hypalgesia) . 
 
 3. Thermic sensations (loss: thermoan- 
 sesthesia). 
 
 4. Sensations of place (localization of the 
 place of stimulation). 
 
 1 Striimpell differentiates pressure sensations of the deeper parts. 
 
 Superficial sensibility. 
 
SENSORY SYMPTOMS OF LOST FUNCTION 501 
 
 Sensations of space. Differentiation of 
 two simultaneously stimulated points on 
 the skin as separate (Weber's circles of 
 touch) . 
 
 6. Sensations of situation and of movement, 
 the latter both passive and active. 
 
 7. Sensations of weight and resistance. 
 
 Deep sensibility of 
 joints, tendons, mus- 
 cles (also called mus- 
 cular sense). 
 
 A " stereognostic " sense, that is the ability to recognize the form of a 
 body from sensations of touch, position, movement, obtained by handling it, 
 may not be placed with the elementary qualities. 
 
 Aster eo gnosis, in so far as it is not the consequence of the loss of ele- 
 mentary sensations, belongs among agnostic symptoms. (See page 541.) 
 
 A loss of sensation may be partial, in so far as only certain qualities are 
 lost, for instance only sensations of position and movement {dissociation of 
 sensibility) . 
 
 It can also be partial in relation to its intensity (loss, mere diminution of 
 sensibility) . 
 
 Finally in relation to its extent. Only part of half of the body, or only 
 part of a member is anaesthetic or hypaesthetic. 
 
 In order to cause complete loss of all sensation in the distribution of any 
 nerve, either the nerve or its root must be completely cut off. Even then 
 partial compensation may occur in the course of time because of the partial 
 overlapping of the sensory areas by various roots. 
 
 Such total anaesthesia can be caused by cerebral focal injuries only in the 
 pons or medulla oblongata, since only in these places can the peripheral 
 neuron of a nerve which subserves general sensibility, namely, the root of the 
 trigeminus be affected. More centrally, a supranuclear central neuron is 
 always struck by the focal injury. Lesions of the central sensory pathways, 
 in whatever plane they may lie, usually do not totally destroy sensibility in any 
 of the three directions (quality, intensity, extent). 
 
 Anaesthesias of the entire half of the body, contralateral to the injury, 
 endure only for the first days or at most weeks after the attack. 
 
 In particular, sensibility to pain is nearly always to, a certain extent 
 restored, even if it frequently remain diminished. 
 
 Next thermic sensibility is most frequently restored, and after it sensibility 
 to touch. Sensations of place and space, position and movement, on the con- 
 trary, in cases of central focal injuries, are often permanently destroyed. 
 
 On the whole, even in large cerebral focal injuries, sensory processes are 
 better restored than motor processes. 
 
 Concerning the extent of sensorial disturbances, it must be noticed, that at 
 
502 DISEASES OF THE BRAIN 
 
 first they frequently exhibit an hemianaesthetic character (the anaesthesia 
 ceases precisely at the middle line) but that later the boundary recedes from 
 the middle line — the medial parts of the head and neck again become sensi- 
 tive — and the disturbance becomes less from the distal parts of the body, 
 hand, foot, etc. 
 
 Hemianaesthesia, as long as it is total, reaches the middle line and affects 
 not only the skin, but the mucous membranes, joints and muscles. It is 
 accompanied usually by hemiplegia, but there are also cases of isolated and 
 nearly isolated cerebral sensory disturbances. 
 
 Anaesthesia may also confine itself wholly or approximately to single 
 limbs or parts of limbs. (In that case, the distal parts are more seriously 
 affected.) 
 
 Generally the more seriously paralyzed limb, is also more seriously 
 impaired in respect to its sensory functions. 
 
 The dissociation of sensibility by cerebral lesions may in exceptional 
 cases go so far, that cold sensations are retained, while heat sensations are 
 lost. 
 
 Concerning ataxia of the injured side, in so far as it is connected with 
 disturbances in muscular sense, see next division. 
 
 The above general statements concerning the usual incomplete loss of 
 sensibility, or, at least, the probable restoration of part of the sensibility, holds 
 good first of all, for the cortical parts and those lying near the cortex, but to a 
 certain degree also for focal injuries in the thalamus, even for those in the 
 pons and medulla. 
 
 The sensory pathways obviously have manifold lateral branches — as is 
 proved by their frequent interruption by gray matter. One must consider 
 even the vicarious entrance of the other hemisphere, since in some cases 
 return of a considerable amount of sensibility has been observed, though all 
 the sensory paths of one hemisphere had been totally destroyed. Gradual 
 processes, like tumors, cause far less sensorial disturbances, than those 
 appearing suddenly. 
 
 Peculiarities of focal injuries at various levels must be discussed under a 
 review of the sensory paths. 
 
 Our knowledge of the sensory pathways is, as yet, very incomplete; 
 opinions vary greatly on the more delicate relations of parts; therefore the 
 following statement can give only a rough schematic sketch. 
 
 The centripetal paths, that course to the cerebellum, and do not cause 
 stimulation that leads to conscious perception (lateral cerebellar tract and 
 Gower's tract) have been mentioned in the discussion of the cerebellum (page 
 480) and are passed by here. 
 
 The major part of the sensory tracts, which, uncrossed, run through the 
 posterior columns of the spinal cord (paths for the muscular sense) and end in 
 the posterior column nuclei (spino-bulbar tract) takes from these its continua- 
 
THE SENSORY PATHWA YS 
 
 5o3 
 
 tion in the central (bulbothalamic) sensory path, which, above the pyramidal 
 decussation, also crosses as fibrse arcuatas internae (superior pyramidal decus- 
 sation) and forms the inter-olivary layer (see Figs. 123 and 124). These 
 fibres, after crossing reach the medial or principal fillet (Fig. 138), which ends 
 in the optic thalamus. For the situation of the main fillet in the pons see 
 Fig. 137. 
 
 That part of the sensory fibres that has crossed in the spinal cord 
 (tract, spino-thalamicus) , and passes through the lateral tract (pain and 
 temperature conductors), consisting possibly of a chain of short tracts, 
 
 Fig. 138. — General course of the medial or principal fillet. (After Edinger.) 
 
 reaches the reticular formation of the pons and joins the principal fillet, in 
 order to reach the optic thalamus together with it. 
 
 Touch sensations seem to pass through both, posterior column and lateral 
 tract. 
 
 The separate position of the paths for the muscular sense on the one hand 
 (inter-olivary layer, fillet), for pain and thermic sensations, on the other 
 (reticular formation — subst. reticularis), in the medulla and the more caudal 
 planes of the pons, makes it possible, that a focal injury here may cause such 
 a dissociation of sensibility that position and movement sensations are re- 
 tained, and pain and thermic sensations lost, or vice versa; since the paths 
 for the muscular sense cross only in the medulla, it depends on the plane, in 
 
5°4 
 
 DISEASES OF THE BRAIN 
 
 which the focal injury lies, whether the sensory disturbance is on the crossed 
 or uncrossed side. The fact that the paths of the lateral tract have already 
 crossed in the spinal cord, makes possible very complicated results (as loss 
 of muscular sense on the same side, and of the thermic and pain sensations 
 on the opposite side). 
 
 Focal injuries in the pons, which strike the trigeminus roots or nuclei 
 and the already crossed sensory paths, cause sensorial disturbances in the 
 face, on the same side as the injury, and in the body, on the opposite side 
 (hemianesthesia cruciata) (Fig. 140). 
 
 The central path that rises in the nuclei of the sensory cerebral nerves 
 (fifth nerve, etc.), and probably also, as has been said, on proximal planes 
 of the pons, the central continuation of the lateral tract, that has reached the 
 
 Ant. Quad, bodies 
 
 Right 
 
 Fig. 139. — Foci in the quadrigeminal region. Focus 1 produces hemianopia (at the right 
 side). Focus 2 diminishes the auditory acumen in both ears. If combined with a lesion of 
 the auditory tracts at the right side it produces bilateral deafness. Focus 3 produces disturbance 
 in sensibility at the right side (because of the lesion of the fillet) and paralysis of the oculomotor 
 at the left side. Focus 4 produces disturbance of motility in the right arm and leg and lesion 
 of the oculomotor at the left side (hemiplegia alternans superior) . 
 
 reticular formation, and is there interrupted by gray matter, join the prin- 
 cipal fillet. Thus all the sensory paths reach the thalamus. 
 
 On the entire way to the thalamus, the sensory paths are separated from 
 the motor paths and can, therefore, obviously be interrupted by injuries that 
 spare the motor paths. Thus, for instance in the frontal division, through- 
 out the hindmost part of the thalamus, the sensory paths lie in a position 
 clearly dorsal (in the tegmental region) from the pyramidal paths that go 
 through the foot of the cerebral peduncle. 
 
 From the thalamus, the thalamo-cortical path passes through the posterior 
 limb of the inner capsule, medially from the lenticular nucleus, and partly 
 through it to the cortex. 
 
 The former supposition that the sensory paths in the posterior limb of 
 the inner capsule are placed absolutely separate from the motor paths, and 
 lie behind the latter (Fig. 134), has been impossible to maintain. 
 
THE SENSORY PA THWA Y 5 o 5 
 
 The isolated sensorial losses often noticed in focal injuries situated here 
 (with motility intact) are caused, in the majority of cases by the participation 
 of the optic thalamus in the focal injury. But even if the sensory paths of 
 the inner capsule run partly together with the motor paths it is still possible 
 for them to be struck comparatively isolated and en masse, in the place, 
 where they pass from the ventral optic thalamus nucleus into the posterior 
 limb of the inner capsule, so that focal injuries in the hind third of the 
 posterior limb of the inner capsule cause more damage to sensibility than to 
 motility. 
 
 Because of the proximity of the central visual path passing through the 
 hindmost part of the inner capsule, the capsular sensory disturbances are 
 generally combined with hemianopia. 
 
 The ends of the sensory paths, lie mainly and most densely in the posterior 
 central convolution. The anterior one receives only few sensory fibres. 
 But besides the posterior central convolution, the anterior division of the pari- 
 etal lobe receives them. 
 
 Superior 
 peduncle 
 
 Fig. 140. — Effect of a focus in the middle of the pons. (Exit of the trigeminal nerve.) A, 
 Aqueduct; B, pyramidal tract. Focus 1, striking only the fillet, produces crossed disturbance 
 of sensibility in the body. Focus 2, striking besides the root of the trigeminal, produces dis- 
 turbance insensibility of the face on the same side, crossed disturbance of sensibility in the body 
 (Hemianaesthesia cruciata). Focus 3 produces crossed motor and sensory disturbance. 
 
 Focal injuries in the posterior central convolution, affect with special force, 
 sensations of place, position and movement. 
 
 Localization by the patient, in that case, is extremely poor, and the 
 consciousness of the position of the limb is often wholly lost. On the con- 
 trary, the other qualities, pain, temperature, and touch sensations, are 
 attached to extensive cortical fields (both central convolutions) and other 
 regions, especially of the upper parietal lobe, and are affected more or less 
 slightly in mere focal injuries of the posterior central convolution. Great 
 destruction of the posterior central convolution, besides that of the adjacent 
 parts of the parietal lobe can cause serious hemianassthesia, which, however, 
 as has been said before, may retrogress to a considerable degree, in the course 
 of time, except that the loss of muscular sense is more permanent. (Some 
 
5o6 DISEASES OF THE BRAIN 
 
 authorities hold that the anterior central conventions have no sensory- 
 functions.) 
 
 When sensations of place, position and movement are lost, and those of 
 touch injured, naturally the forms of objects are not recognized, by feeling 
 them, and the objects themselves can not be identified. This is a perceptive 
 astereognosia which is a natural consequence of the loss of the elementary 
 qualities needed for recognition of form, and must not be confused with 
 Wernicke touch-crippling (touch-paralysis, tactile agnosia). It is to the 
 latter what the blind man's inability to read is to alexia. 
 
 The above mentioned tactile agnosia, appearing in lesions of the middle 
 third of the posterior central convolution and its immediate vicinity towards 
 the rear, cortical touch-paralysis, is discussed hereafter. 
 
 Figs. 139 and 140 illustrate the effects of variously situated focal injuries 
 in the quadrigeminal region and the pons. 
 
 (e) Cerebral and Cerebellar Ataxia 
 
 As destruction of the posterior column of the spinal cord, through the 
 loss of centripetal signals, which regulate movement, especially of the 
 stimuli coming from the muscles, joints, ligaments, causes spinal ataxia, so 
 extensive breaks in these sensory paths, in the higher divisions — in the 
 medulla, in the cap of the pons, in the cerebral peduncle, in the thalamus, in 
 the inner capsule, centrum semiovale and cortex — cause cerebral ataxia. 
 Hence, especially, lesions of the fillet, optic thalamus, thalamo-cortical teg- 
 mental path, the cortex of the posterior central convolution and of the 
 adjacent parietal cortex, disturb the co-ordination of movements. 
 
 Cortical ataxia and subcortical ataxia, which is caused by a break in the 
 sensory paths and is with difficulty differentiated from it present a somewhat 
 different picture from that of the well known spinal ataxia of tabes. The 
 excessive, flail-like movements of the tabetic are not so prominent. Instead, 
 all movements become inexact, without consideration as to the shortest way; 
 now the movement comes near the goal, now it falls short of it. 
 
 The fine harmony in the co-operation of muscles is absent; neither choice, 
 nor measure, nor sequence in time of the innervations is correct. The 
 synergies (co-operation of muscles (agonists) and their antagonists and of 
 collateral and rotatory synergists) are disordered. Often the object falls 
 from the hand, the sensory disturbance directly co-operating with ill managed 
 muscular action. The writing of the ataxic is scribbling, shows evidences of 
 trembling and does not stay on the line; the letters and parts of letters are 
 uneven and contorted. All the assurance, exactness and finish, that practice 
 has given our movements, are lost. In what was formerly called cortical 
 ataxia, there was included, besides this awkwardness, which is conditioned 
 by the loss of centripetal control, something, now classed with apraxia: 
 
DISORDERS OF THE SPECIAL SENSES 507 
 
 kinetic memories for certain innervation complexes are lost, cf. apraxia. 
 (Probably mimic disturbances, at times observed in focal injuries of the thala- 
 mus are merely the consequence of loss in sensibility.) 
 
 Different from cortical and subcortical cerebral ataxia (focal injuries in 
 the medulla, pons, etc., belong here, in as much, as they act through lesions 
 which affect paths going to the cerebrum) is cerebellar ataxia. The cere- 
 bellum has less to do with the co-ordination of the movements of the extremi- 
 ties, than with the regulation of upright position, of the maintenance of equi- 
 librium in standing and walking. The sufferer from cerebellar disease totters 
 like a drunken man. The characteristic of cerebellar ataxia is, that a person 
 exhibiting it in its severer forms when walking or standing, shows compara- 
 tively little ataxia in arm and leg movements made while lying on his back. 
 
 Especially in walking interference is shown in the regular co-operation 
 of legs and trunk. This may be only unilateral and then the ataxia is on the 
 same side as the cerebellar focal injury (in contradistinction to cerebral 
 focal injuries). This control by the cerebellum occurs probably because of 
 the stimulations flowing towards it through the lateral cerebellar tract, which 
 do not become conscious, whereas the usual control of the movements of the 
 extremities comes from the cerebrum and is based for the most part on 
 conscious perceptions. 
 
 /. Symptoms of Lost Sensory Function (Disturbances of the 
 
 Special Senses) 
 
 1. Visual Disturbances, Visual Paths 
 
 (a) Choked disc has been discussed under general symptoms. See 
 page 487. 
 
 In long persisting choked disc, central keenness of vision is generally 
 impaired, and the field of vision irregularly restricted. The final result may 
 be blindness, which corresponds ophthalmoscopically to the clinical picture 
 of atrophy of the optic nerve. If an operation be had in time or perhaps in 
 case of cerebral syphilis, therapeutical methods be used, choked disc and 
 optic neuritis may disappear entirely. [It should never be forgotten that 
 a quite marked optic neuritis may be present and the patient not realize any 
 deficiency of vision.] 
 
 (b) Atrophy of the optic nerve, greenish whitish discoloration of the 
 well defined disc, usually bilateral, is a rather frequent symptom of degenera- 
 tive processes in tabes and tabo-paralysis, occurring less often in pure syph- 
 ilis. It often precedes the other symptoms by years. One must be very 
 cautious, therefore, in assuming the existence of isolated optic atrophy without 
 tabes, paralysis, or syphilis. It may happen, however, that optic atrophy 
 exists permanently without other nervous disease. Optic atrophy of slighter 
 degree is found, at times, in multiple sclerosis. 
 
5 o8 DISEASES OF THE BRAIN 
 
 There are, besides, other bilateral diseases of the optic nerve, which 
 cause slight or no changes in the fundus of the eye, that often lead to sudden 
 blindness or produce only central scotoma especially for red and green. 
 They are traced back to certain neuritic processes in the optic nerve 
 (neuritis retrobulbaris), toxic substances, as alcohol, nicotine, extract, filic. 
 mas., being usually the cause. A more detailed description of them belongs 
 in a text-book on diseases of the eye. 
 
 Of interest is "amaurotic idiocy," recently discovered, a combination of 
 optic nerve atrophy with peculiar changes in the macula lutea and idiocy, 
 which in certain families is hereditary and found chiefly in Jewish children, 
 (c) Injuries to the chiasm. 
 
 Since affections that attack the chiasm, generally proceed from the middle, 
 very frequently from the hypophysis, it frequently happens that both crossed 
 
 bundles of the optic nerves — which supply 
 
 \v\ //■' / ^ e nasa l retinal surface — are affected, there - 
 
 "\ \^^ -' fore the temporal halves of the field of vision 
 
 y'yy^Wd^S: -^ are blinded (bitemporal hemianopia) . If the 
 
 / /// Herd \V\ \ uncrossed bundle on one side is also affected, 
 
 / /// \vN tota * blindness of the eye on that side, and only 
 
 ' T . ^ N ' temporal blindness of the other results. A 
 
 Fig. 141. — Lesion m the chiasm . .. . 
 
 arising from disease in the hy- lesion arising without the chiasm that affects 
 Eet£opE d ^"S^dTSi it (««»w Fig. 141), may cause unilateral nasal 
 
 are indicated by dotted lines. The hemianopia. 
 
 crossed fibres affected by the lesion „, , . f , ., , , 
 
 subservebothnasalhalvesoftheret- The combination of bitemporal nemi- 
 
 ina therefore the temporal halves anop i a w i tn acromegaly IS well known as 
 of the -visual field. r b J 
 
 characteristic of a tumor of the hypophysis. 
 
 The Visual Paths. — Into the optic tract of each side, enter the fibres of 
 the same sided half of the retina of that side and the (crossed) fibres of the 
 same sided half of the retina of the opposite side. As to their main body, 
 the tractus fibres end in the lateral geniculate body; a smaller part ends in the 
 anterior quadrigeminal body and in the pulvinar. See Fig. 142. They are 
 the so-called primary optical centers. The fibres, that go to the quadrigeminal 
 body, come into communication with the oculomotor nucleus, and produce 
 the pupillary reflex. 
 
 From these primary centers the so-called optic radiation passes back into 
 the occipital lobe, and, surrounds, in its entire course from the outside, the 
 posterior horn. In this course it passes the deep white matter of the lower 
 parietal lobe (especially of the angular gyrus) and occipital lobe and the 
 greater part of it reaches the medial surface of the occipital lobe, in the vicinity 
 of the calcarine fissure, in the visual center marked by Vicq d'Azyr's stripe 
 and peculiar formation. This embraces mainly the cuneus (dorsal lip of the 
 calcarina) and the lobus lingualis, perhaps also the fusiform gyrus. The 
 
THE VISUAL PATHS 
 
 5°9 
 
 visual center encroaches but little upon the convexity, except perhaps at the 
 occipital pole and in the third occipital convolution. 
 
 From the visual center pass corticofugal paths, together with the cen- 
 
 Ant. quadrigem. 
 
 Fig. 142.— Diagram of the optic paths. The left halves of the retina correspond with the 
 
 right halves of the visual field. 
 
 tripetal ones, just described, into the same ganglia, from which the optic 
 radiation arises. 
 
 The posterior horn is surrounded, on the outside, by three medullary 
 layers, the fibres of which run sagitally. The innermost, lying directly 
 
5 io DISEASES OF THE BRAIN 
 
 adjacent to the ependyma of the ventricles, lining the ventricles to a certain 
 extent, is called the tapetum (Strat. sagittale mediale). 
 
 Towards the outside of it, therefore in the middle, lies the stratum 
 sagittale internum and outside of it, the stratum sagittale externum or inferior 
 longitudinal fasciculus (Fig. 143). 
 
 The latter was long considered a pure bundle of association fibres between 
 occipital and temporal lobes, whereas the real optic radiation (Gratiolet's) 
 was placed in the stratum sagittale internum. The tapetum was supposed 
 to consist of corpus callosum fibres exclusively. But it has been proved, 
 that in all three strata, especially in the stratum internum and externum, 
 optic radiations course, that the stratum sagittale externum also contains 
 association fibres, the tapetum, mostly corpus callosum fibres, by inter- 
 vention of the forceps. At any rate, the optic radiation can no longer be 
 
 Tapetum. \^ \_x/ \. J) 
 
 Strat. sag. med. ^-A . J y ^. S^~~^^ Fa.sc. long, inf. 
 
 / Strat. sag. ext. 
 
 Gratiolet's optic radiation 
 Strat. sag, int. 
 
 Fig. 143. — Diagram of the three sagittal medullary layers surrounding the posterior horn V 
 in a frontal cut through the gyrangularis. 
 
 confined to the middle layer, but to the inferior longitudinal fasciculus must 
 be assigned a large part of the optic projection fibres. 
 
 Lesions behind the chiasm throughout the visual path, that is in the 
 optic tract, in the ganglion geniculatum laterale, in the optic radiation and 
 the cortical center, are bound to cause bilateral homonymous-hemianopia, 
 that is loss of function in the halves of both retinas, on the same side as the 
 cerebral lesion and correspondingly, in the halves of the field of vision on the 
 opposite side. In the majority of cases, the whole half of the field (except 
 the so-called superfluous field) is lost, so that hemianopia, in the strict sense, 
 occurs. That is, when the focal injury is on the left, a right hemianopia of 
 both eyes corresponding to the blindness of both left halves of the retinas. 
 
THE VISUAL PATHS 
 
 5ii 
 
 Under certain conditions, only a quarter is lost for both eyes : quarter hemi- 
 anopia (Fig. 144). 
 
 It is important to notice, that the loss of exactly half of the field of vision 
 
 Fig. 144. — The location of the three sagittal medullary layers in a horizontal cut of the brain 
 (Drawn from a Weigert preparation (3/2 of natural size) from Dejerine.) C, cuneus. CA, 
 Amnion's horn. Cc, corpus callosum. Ce, external capsule. Cex, capsula extrema. Ci(y), 
 capsula interna (genu). Cip, capsula interna (posterior limb). CI, claustrum. K, calcarine 
 fissure (appears in two places). Lg, gyr. lingualis. Put, putamen (external layer of the lentic- 
 ular nucleus). O, occipital convolution. St. s. ext, stratum sagiltale externum or fasciculus 
 longitudinalis inferior. Str. s. i., stratum sagittale internum or Gratiolet's visual radiation. 
 Dtr. s. m., Stratum sagittale mediale or tapetum. T2, second temporal convolution. Th, 
 thalamus. 
 
 (total hemianopia) is very rare. This would mean, that the line of division 
 between the parts of the visual field that are retained and those that are lost, 
 would go as a vertical line directly through the point of fixation. It occurs 
 
512 
 
 DISEASES OF THE BRAIN 
 
 occasionally, with focal injuries in the optic tract, but not generally with 
 centrally situated focal injuries. Usually at least a small area about the 
 point of fixation, in the direction of the loss, belongs to the part retained. 
 This very area, namely, the part retained, situated beyond the vertical 
 
 2S& 
 
 Fig. 146. 
 Figs. 145 and 146.— Visual field in right-sided hemianopia with residual field of vision. 
 
 meridian passing through the point of fixation, is known as the "superfluous 
 field of vision." The line of division makes a curve about the point of 
 fixation, at least, which with its convexity extends into the blind half of the 
 field of vision (Figs. 145 and 146). 
 
THE VISUAL PATHS 513 
 
 The fact, that the direct vicinity of the point of fixation on both sides, 
 is almost always retained, proves that the region of clearest sight in the 
 retina (macula) is double, that is, represented in both hemispheres. The 
 size of the superfluous field of vision varies with the individual case (2 to 15 
 degrees) and may even be different in each eye. Moderate concentric 
 narrowing of the retained field of vision, also may occur, more obviously in 
 the eye that is on the opposite side from the focal injury. Certain other 
 irregularities in the boundaries of the visual field, in focal injuries of the 
 occipital lobe, are still more difficult to understand. 
 
 Bilateral hemianopia is called cortical blindness, an adopted, but not 
 wholly correct nomenclature (cerebral blindness is better), since it is often a 
 question, not of cortical focal injuries, but of bilateral interruption of the 
 optic radiation or cortical focal injury on one side and subcortical on the 
 other. 
 
 In this bilateral hemianopia a small central part of the field of vision 
 (sometimes 2 to 3 degrees) is generally retained, which, however, easily 
 escapes both observer and patient, and, therefore, demands skilful investi- 
 gation to prove its existence. 
 
 In rare cases, both halves of the visual field have been lost simultaneously, 
 by embolism of the occipital lobes. 
 
 Vision in the retained visual field is often, though not always, diminished 
 in hemianopia as well as in cortical blindness. 
 
 A case in which, on the contrary, macular vision is destroyed, but 
 peripheral retained — that is a field of vision with central scotoma — due to an 
 occipital focal injury— has not yet been observed to occur as a permanent 
 affection. 
 
 The fact that in uniocular as well as in binocular hemianopia, macular 
 vision is usually retained, has been attributed by some investigators to 
 double cortical representation of the macula, by others to the fact, that the 
 situation of the cortical representation of the macula, is protected especially 
 against circulatory disturbances, by others (who speak against an isolated 
 representation of the macula), to a representation of the macula, extending 
 over large areas of both occipital lobes. 
 
 From the last point of view, the macula has an extraordinarily extended 
 representation in the occipital lobe, so that only immensely extended focal 
 injuries could rob it of all connections with the cortex. 
 
 How far definite parts of the retina correspond to definite parts of the 
 cortical visual center, i. e., how far the retina is, strictly speaking, projected 
 on the cortex, has not been unanimously decided. There is much difference 
 of opinion about it. 
 
 As the termination of the optic radiation (visual sphere, visual center) 
 chiefly the vicinity of the calcarine fissure must be considered. Especially 
 its upper border — the cuneus— and its under border, the fusiform gyrus, 
 
 33 
 
5 i4 DISEASES OF THE BRAIN 
 
 probably the entire region marked out by the Vicq d'Azyr stripe, the cellular 
 structure of which is peculiar and uniform. 
 
 The fact that quarters and even sixths are lost by focal injuries in the 
 optic radiation and visual center proves, at any rate, that no complete min- 
 gling of fibres, which come from various parts of the retina, takes place, that 
 at least, on broad lines, projection takes place. And so to the upper quarters 
 of the retina in both eyes, corresponds the upper border of the calcarine fis- 
 sure, to the lower quarters, the lower border. 
 
 By important authorities, the pathological discoveries which led to this 
 proof, were used to show that not the cortical focal injuries concerned, by 
 themselves, but accompanying lesions of various parts of the optic radiation, 
 had the effect mentioned, so that a projection of the quarters would exist 
 in the optic radiation, but no longer in the cortex. 
 
 The last word has not yet been said about the projection of the 
 macula. 
 
 Instead of hemianopia, mere disturbances of color vision may affect half 
 the field of vision (hemiachromatopsia or hemidyschromatopsia). Now 
 general weakness for color vision appears, now color blindness for red and 
 green only, or less frequently for yellow and blue. It is remarkable that 
 often with right hemianopia, is found hemidyschromatopsia in the half of 
 the field of vision that has been retained. 
 
 Paralyses of the muscles of the eye are not, per se, connected with hemi- 
 anopia. But the loss of sensory-optic stimulations can produce an uneco- 
 nomic use of the muscles of the eye and hence lead to mistakes in estimating 
 distance. 
 
 With hemianopia, from whatever cause it arises, frequently disturbance 
 in measuring with the eye is connected, in such a way that by halving a 
 horizontal, the half corresponding to the loss in the field of vision is made 
 too small. (The opposite error is rare.) 
 
 Hemianopia caused by a focal injury in the optic tract must be distin- 
 guished from hemianopia caused by an interruption in the optic radiation or 
 injury in the visual center, by the fact that in the former case, throwing light 
 upon the blind half of the retina, causes no pupillary reflex, whereas there is 
 no cause for this in the latter case, since the reflex arc (connection of the 
 tract with the oculomotor nerve) is intact. The proving of pupillary rigidity 
 in hemianopia is, however, attended with great difficulties, because it is 
 impossible to succeed in confining the illumination exclusively to the retinal 
 halves concerned. But in bilateral hemianopia, caused by cortical or 
 subcortical focal injury, the fact that the pupillary reflex is retained, can be 
 easily proved. 
 
 Subcortical focal injuries may irritate the visual center, and occasionally 
 cause visual hallucinations in those parts of the field of vision that have been 
 lost. 
 
AUDITORY DISTURBANCES 
 
 5i5 
 
 It is still uncertain which part of the visual system is affected in uraemic 
 amaurosis. 
 
 In addition to the hemianopsia present in all focal diseases, which strike 
 the visual system from the tractus to the visual center, it is found frequently 
 as a transient affliction in progressive paralysis, after the attacks, and in 
 migraine. After focal injuries in the temporal and parietal lobes, it occurs 
 temporarily in the first weeks after the attack as an indirect symptom of the 
 nearby lesion. Hemianopia is frequently the first symptom of apoplexy. 
 Focal lesions in the parietal lobe, especially in the angular gyrus, which ex- 
 tend deeply below the surface, break through the optic radiation, and hence 
 cause permanent hemianopia (Fig. 147). 
 
 $ts.L 
 
 Fig. 147. — Case of softening in the gyr. angularis. The softening focus H passes through the 
 3 sagittal medullary layers. Drawing from a Pal preparation. Nat. size (frontal cut). At 
 the left, medial surface of the brain. V, posterior horn. P.O., fissura parieto-occipitalis. The 
 fissure without any designation below the latter is the calcarine. P, forceps, the preserved part 
 of the corpus callosum radiation. F.l.i., The preserved parts of the fasciculus longitudinalis 
 inferior. Str.s.i., Remains of the stratum sagittale interius. T, place of the absent tapetum. 
 
 2. Auditory Disturbances 
 
 Auditory Paths. — The cochlear nerve (which is the only branch of the 
 acoustic nerve that is concerned with hearing) , rises in the cells of the spiral 
 ganglion of Corti. It ends in the ventral nucleus of the cochlear nerve and 
 in the dorsal nucleus (acoustic tubercle-tuberculum acusticum). From 
 these spring other paths, which, excluding further interruptions in the nuclei 
 of the lateral fillet, for the most part pass in the trapezoid body (interruption 
 in the olive) , partly pass into the striae acusticae, on the other side, reach there 
 the opposite lateral fillet, and for the most part end, after going through the 
 arm of the posterior quadrigeminal body, in the medial geniculate ganglion 
 (the smaller part in the posterior quadrigeminal body) (Fig. 148). 
 
 Part of the fibres remain uncrossed, and there is, besides, a commissure 
 between the bilateral nuclei of the lateral fillet, so that each cochlear nerve 
 is connected with both medial geniculate bodies, hence with both hemispheres. 
 Here, too, we find se/mcrossing. 
 
5 l6 
 
 DISEASES OF THE BRAIN 
 
 From the medial geniculate ganglion the central auditory path goes to 
 the cortex of the temporal lobe and the greater number of the auditory fibres 
 reach the transverse convolutions of the temporal lobe (gyr. transversi), 
 Heschl's convolutions, and a neighboring tract in the middle third of the first 
 temporal convolution. 
 
 The further course of the fibres reaching the posterior quadrigeminal 
 body is a matter that has not as yet been cleared up. 
 
 Deafness in the opposite ear, after one-sided lesions in the temporal lobe, 
 has never been observed. In that case, we have only lessened hearing, in both 
 ears, corresponding to the connection of each ear with both temporal lobes. 
 
 Likewise, unilateral interruption of the medial geniculate ganglionic 
 paths, going to the temporal lobe, does not cause total one-sided deafness. 
 
 Gglgen.med VorptereVierhuyel 
 
 Hintere Vierfiiigel 
 
 Striae acusticae 
 dor,s AT cochL 
 
 Later ale /Schteift 
 
 Cortisckes Organ 
 McocAlearis 
 
 tfYL Corpus trapezoides 
 
 Fig. 148. — Scheme of the central auditory paths. Interruption in the nuclei of the lateral 
 fillet not considered. N.N. VI., nucleus of the abducens. O.d., right superior olive. In the cut: 
 Laterale Schleife = lateral fillet. Scblafenlappen =■ temporal lobe. Querwindung = transverse 
 convolution. Vordere Vierhigel = anterior quadrigeminal bodies. Hintere Vierhugel = posterior 
 quadrigeminal bodies. Cortisches Organ = Corti's organ. 
 
 Total deafness in both ears may be caused by bilateral disease in the 
 temporal lobes (so-called cortical deafness). Heretofore, this has been 
 observed only in very extended focal injuries, so that the above anatomical 
 boundaries of the auditory center (based upon the myelogenetic method) 
 could not be clinico-pathologically substantiated, and many authors ascribe 
 to the auditory center much more space in the temporal lobe, than in follow- 
 ing Flechsig, is given above. 
 
 More often than total deafness, great difficulty in hearing, in both ears, 
 results from bilateral lesions of the temporal lobes. This does not show the 
 well known tone-gaps of labyrinthine diseases (as the loss of the highest 
 tones), but a more regular diminution for the entire tonal sequences. 
 
 This does not decide against a projection of various tone-heights on the 
 cortex, since in the two-sidedness of the representation of the organs of Corti 
 
AUDITORY DISTURBANCES 
 
 5*7 
 
 in both temporal lobes, an insulated tone-gap could only appear, if by chance, 
 exactly the same parts were destroyed on both sides. At any rate the assump- 
 tion, that is to be discussed under sensory aphasia — that the cortical end- 
 
 Insul 
 
 ^Auditory 
 'tract to 
 cortex. 
 
 First 
 
 temporo 
 sphen- 
 oidal lobe 
 
 Optic plus 
 
 Organ of < 
 Corti 
 
 Nucl. vent 
 
 X. cochl. 
 Fibres of the 
 trapezoid body 
 
 long. int. 
 
 Stria? acustics 
 
 Fig. 149.— Topography of the auditor}' paths. (A connection is shown between the unmasked 
 posterior quadrigeminal body and the C. gen. int.) In the cut: Linsenk. = lenticular nucleus, tot. 
 
 Schl. = lower fillet. 
 
 ing of the fibres of the tones b' to g"— a very important part of the tonal 
 sequence— take up a definite spacial division within the entire auditory 
 sphere, is as yet, wholly hypothetical. 
 
 Auditory disturbances caused by focal injuries in the cap of the cerebral 
 
5 i8 DISEASES OF THE BRAIN 
 
 peduncle and the pons and in the region of the posterior quadrigeminal 
 body, occur, yet, because of the wide distribution of the paths concerned, 
 and their semi-crossing, are seen permanently in high degree, only when the 
 focal injury is very extensive — except when nucleus or root of the cochlear 
 nerve is destroyed, in which case, deafness in one ear results. 
 
 3. Olfactory and Gustatory Disturbances 
 
 In basal focal injuries that affect the olfactory nerve, nucleus, or tract, 
 the olfactory trigone, or the perforated substance, a diminution in the 
 sense of smell, on both sides, is noticed. 
 
 The olfactory centers lie in the hippocampal gyrus and the Ammon's 
 horn. 
 
 Olfactory hallucinations also appear in irritations of the olfactory regions. 
 
 The gustatory sphere, too, lies in Ammon's horn, the hippocampal 
 gyrus and the adjacent posterior end of the fornicatus gyrus. The peripheral 
 paths of taste course in the trigeminus and the glosso-pharyngeal nerves. 
 
 3. Mnemic-associative Derangements 
 
 (a) Aphasic Disturbances 
 
 1. Introduction 
 
 Disorders of the Projection System of the Apparatus of Speech: 
 Anarthria in Bulbar and Pseudo-bulbar Paralysis 
 
 Language is a system of symbols serving for the communication of 
 thought between men. 
 
 The symbols of spoken language are sounds and combinations of sounds 
 which are naturally produced by the action of the muscles of the lips, jaws, 
 tongue, palate, and larynx. In this aspect, speech is, then, a series of move- 
 ments, the effect of which is to give other people symbols of the psychic 
 processes, such as thoughts, feelings, desires, etc., of the person speaking. 
 {Speech-expression.) 
 
 But considered with reference to its effect as audible sound, it may be 
 included with the process of hearing, and evokes in the listener the corre- 
 sponding psychic processes. The role of the listener is understanding. 
 {Speech-perception.) 
 
 Not until late in the development of both the species and the individual, 
 do we find a second system of symbols: written speech which also has an 
 expressive (writing) and a receptive (reading) side. 
 
 Both written and spoken language employ certain muscles which serve 
 other purposes as well. Thus the muscles of the tongue, jaws, and lips, 
 serve for eating, chewing, sucking, swallowing, licking, kissing, etc., and 
 for various voluntary and involuntary imitative movements. 
 
SPEECH DISTURBANCES 
 
 5i9 
 
 If this system of muscles or its corresponding nervous centers is diseased 
 (paralysis, paresis, ataxia, tremor, etc.) , speech is affected secondarily. The 
 resulting disturbances of speech are called, according to their degree, dys- 
 or an-arthria. 
 
 To this nervous mechanism belong: 
 
 1. The cortical centers of the muscles concerned, therefore the centers 
 of the 12th, 7th, 10th, and nth Nerves in the lower fourth of the anterior 
 central convolution. (Operculum Rolandi.) 
 
 2. The motor (cortico-bulbar) tract from these centers to the bulbar 
 nuclei of the nerves. They pass through the white matter, through the 
 "genu" of the internal capsule, the base of 
 the cerebral peduncle and reach to the nu- 
 clei of both sides in the pons Varolii and the 
 medulla oblongata. 
 
 Because of the connection of each hemi- 
 sphere of the brain with the nuclei of both 
 sides, it results that supranuclear lesions of 
 one side do not produce permanent paraly- 
 sis of the nerves concerned, and therefore 
 the dysarthria resulting is neither severe 
 nor long-continued. A break in the con- 
 nection of the cortico-bulbar tracts and 
 their centers can indeed bring about tem- 
 porary dysarthria, especially when the left 
 hemisphere is affected. Slight dysarthric 
 disturbances often persist after a lesion of one side. 
 
 3. The peripheral tract from the nucleus of the 12th Nerve, etc., to the 
 muscle. 
 
 Disease in the medulla oblongata affects the peripheral tract represented 
 by 3. If the bulbar nuclei, or their roots, are destroyed, we have bulbar 
 paralysis, together with atrophy and degenerative reactions in the muscles 
 concerned (tongue, lips). It causes besides the dysarthria — which consists 
 of incorrect articulation of letters, and is conditioned by the centers affected, 
 whether they be more particularly labial, palatal, or lingual — other paralytic 
 phenomena: partial paralysis of the facial muscles and disturbances in eating, 
 chewing and swallowing. 
 
 If the cortical centers or the supranuclear nerve tract are affected on one 
 side alone, there results only, as we have said, a slight degree of disturbances 
 in the muscles concerned: slight paralysis of the ends of the facial nerve, 
 deviation of the tongue, when it is extended, slight difficulty in articulation 
 with no disturbance in swallowing, no atrophy and no reaction of degenera- 
 tion. But if the centers or nerve tracts are disturbed on both sides, or on one 
 side the center and on the other the tract, similar disturbances result as arise 
 
 Km ' — ■*- xjz 
 
 Fig. 150. — Seats of lesions causing 
 pseudo-bulbar paralysis, i and 2 foci 
 in the left and right cortical center of the 
 seventh, twelfth nerves, etc. 3 and 4 foci 
 in the paths to the pontine and bulbar 
 nuclei. The combinations: 1 + 2, 2 + 
 3, 1 + 4, 3 + 4 produce pseudo-bulbar 
 paralysis. 
 
5 2o DISEASES OF THE BRAIN 
 
 from the destruction of the bulbar nuclei themselves, only that atrophy and 
 degenerative reaction are absent and that there is a smaller degree of dis- 
 turbance. Since the supranuclear fibres above the pons disperse in all 
 directions, very great focal injury is necessary to interrupt them wholly. 
 Those localized in a supranuclear position in the pons are most likely to effect 
 this (Fig. 150). 
 
 Since such two-sided disturbances of the cortical centers and the cortico- 
 bulbar nerve tracts produce phenomena so like bulbar paralysis, they have 
 been named pseudo-bulbar paralyses. 
 
 The essential feature of pseudo-bulbar paralysis is therefore that on 
 both sides the cortico-bulbar nerve tracts (eventually at the place of their 
 cortical origin) are interrupted at some one place. To bring this about, 
 unless it be in the pons the nerve tracts must be stricken twice, once on the 
 left, and once on the right side. In this case, there are disturbances in articu- 
 lation, in swallowing, etc. 
 
 Disturbances in this efferent neuro-muscular apparatus belong to the 
 derangement of the projection system alone and have no connection with 
 aphasia, even when they are caused by cortical injuries. They cause corti- 
 cal, cortico-bulbar and bulbar anarthria or dysarthria. 
 
 Not only spoken but written language as well employs a neuro-muscular 
 apparatus, which serves other purposes as well. It is the apparatus which 
 innervates the more delicate movements of the hand. Paralysis, weakness, 
 ataxia of the hand, produce the same hindrances in writing, that pseudo- 
 bulbar or bulbar paralysis produces in uttered speech, except that only 
 rarely is the other hand affected also, and that the movements used in writing 
 can be performed, more or less, by any other movable portion of the body 
 (foot or tongue), while the production of sound is confined to a definite 
 muscular system. Just as utterance can be affected indirectly by injury to 
 the efferent neu.ro-muscular apparatus, the perceptive faculties of speech 
 suffer from lesions of the perceptive apparatus, i. e., of the organs of hearing 
 and seeing. By a difficulty in hearing verging on deafness, or as we shall see, 
 by a definite gap in the perception ofto-nes, the understanding of speech can be 
 destroyed, as well as the ability to read by a weakness of eyes, verging on 
 blindness. 
 
 Even then, we cannot speak of aphasic, but of perceptive disturbances. 
 
 2. Disturbances of the Mnemic-as so dative Speech Apparatus: Aphasia. 
 
 Internal Speech 
 
 When, however, the cortico-bulbar apparatus, on which sound-formation 
 depends, fulfills all its functions, and yet the patient can only speak badly or 
 not at all, aphasia exists. 1 
 
 1 Mutism from hysteria and mental disorders is not taken into consideration here. 
 
MECHANISM OF SPEECH 521 
 
 What has failed, is so-called internal speech, i. e., something that has been 
 learned, a -possession of memory. 1 
 
 We use internal speech to distinguish it from external speech. The 
 latter is the speech-musculature's creation, which produced by the one, the 
 other hears. The primary condition for external speech is internal speech, 
 but loss of internal speech entails the loss of external speech. 
 
 Internal speech is ever present while we are thinking. Most of our 
 thoughts present themselves in the form of speech. In the case of vivid 
 thought impulses discharge themselves into the neuro-muscular apparatus, 
 so that we have abortive innervations, which may rise into involuntary 
 utterance of thought. 
 
 The internal word consists originally of two parts, closely connected: 
 
 1. Memory of the word-sound, or the acoustic word (a), the residuum of 
 remembered words, that the child hears from his environment. It is the 
 oldest word-possession and the great majority of words are acoustically 
 acquired. 
 
 Moreover the meaning of the word, i. e., the corresponding concept, 
 attaches itself to the acoustic word — as the word Bow-wow to the concept 
 of the dog — so that for many words, before the child itself can speak, 
 there arises a close connection between the concept (c) and the acoustic 
 word. C — a is the first speech-association. 
 
 2. The memory of word-movements or the motor-word (m). The 
 memory of the process necessary for the utterance of the word. 
 
 Usually this process is called kinesthetic. It comprehends memory of 
 perceptions reaching all the parts moved by us in speech, lips, tongue, 
 palate, etc. The utterance of the word '"parrot" gives us other sensations 
 of place, movement, and contact than, say, the word "weasel." 
 
 In truth, we find in consciousness nothing besides memories of this 
 perception complex. But beneath the threshold of consciousness there 
 comes a richer, a more material memory possession. The frequently 
 repeated sequence of innervations, which correspond with a definite sound- 
 complex, leaves probably a lasting connection between the co-working 
 innervational elements, so that the motor-word 2 is composed of kinassthetic 
 and innervational memories, of which only the former have a psychic 
 equivalent. 
 
 The motor-word is greatly dependent on the acoustic word. The mouth 
 cannot produce what the ear has not heard. The child learns to repeat a 
 series of words he has heard. By this means a very close connection is 
 produced between a and m (a — m). This is the word as it exists in the child. 
 
 1 We have learned articulation, too. But it is not what we have learned that is lost in dysar- 
 thria, but the natural instrument, with which what has been learned is produced. 
 
 2 This innervational memory holds good in part for whole words, especially however, for parts 
 of words, for syllables and letters. 
 
522 DISEASES OF THE BRAIN 
 
 This combination of the acoustic with the motor-word represents internal 
 speech in its restricted sense. As we see, the concepts are predominantly 
 connected with a, and with m only through the mediation of a, so that the 
 relations of the motor-word to the concept is brought about mainly through a. 
 The following association is thus formed: 
 
 C 
 
 TI 
 
 (a — m) . 
 
 The recollection of word-movements can combine itself with the concept 
 only secondarily, inasmuch as every concept has, indeed, led to m only 
 through the mediation of a, yet the frequent simultaneous appearance of C 
 and m nevertheless produces a secondary direct association. This less 
 firm and comprehensive connection we shall represent by a dotted line 
 between C and m. 
 
 C 
 
 x\ 
 
 (a— >m) 
 
 When, between the ages of seven and eight years, the child learns the 
 written language, by the complex (a — m) the written word attaches itself to 
 the sounded word. But, unlike hieroglyphics, the written word-symbols 
 are not direct symbols for the apprehension of the objects, but are symbols 
 for sounds, i .e., for parts of the sounded word. In other terms, our writing is 
 phonetic. 
 
 Now only does the child learn to separate the words into syllables and 
 letters and to assign to each letter a written symbol. Hence it is, that as a 
 rule, the written language is connected with the concept only through the mediation 
 of the sounded language. To each sound an optical letter symbol is assigned 
 (o) and in writing this optical letter is produced by the movement of what- 
 ever member is guiding the pen. Thus the acoustic motor-word, aroused 
 by the concept, is separated into its component parts, each of which evokes 
 its corresponding optical image, from which impulses flow to the hand center. 
 
 Through practice, graphic-motor recollections are formed in the hand- 
 center. 
 
 The complex associations formed thus between the concept and the 
 acoustic, linguo-motor, optical and grapho-motor components, represents 
 internal speech in its wider sense. 
 
 Now impulses flow from m to the neuro-muscular apparatus of the 
 tongue muscles. The stimulations in the hand-center flow off to the 
 muscles of the hand, while to a and o arrive the centripetal stimulations of 
 the ear and eye. The sound of the words penetrates to the seat of the 
 acoustic memory images, arouses them, and thereby brings about the 
 release of the components associatively connected with a, that is of the 
 whole word. 
 
MECHANISM OF SPEECH 
 
 523 
 
 The theory once held of a direct speech pathway to and from the bulbar 
 nuclei had to be abandoned. On the contrary speech makes use of the 
 same projection pathways (although with a certain preference for those on 
 the left side) as the other movements of the tongue, lips, etc. Therefore 
 the stimulation from m must first reach the cortical center " p" of the 7th 
 and 1 2th Nerves, etc., in the Rolandic Operculum, where the real innervation 
 takes place. It is consequently necessary to insert " p" between m and 
 tongue. 
 
 In the same way the acoustic stimulations reach first the acoustic pro- 
 jection-field "alpha" before waking the remembrances in a. Even the 
 authors who do not share the view of Flechsig of the absolute separation of 
 the projection-field (acoustic-center) from the mnemic field (a), assign the 
 perceptive and the mnemic process to various processes of the same field, 
 so that perception and waking of the memory are regarded as two distinct 
 
 Fig. 151. — B, conception; a, acous- 
 tic component; m, speech motor com 
 ponent; 0, optic component; gr, grapho- 
 motor component. 
 
 Ear Eye Hand Tongue 
 
 Fig. 152 — a, acoustic perception; u, in- 
 nervation. The other letters as in scheme 
 Fig. 151. 
 
 parts of the process. (For o and gr the corresponding projection apparatus 
 are not shown separately.) 
 
 The complete scheme of speech is shown in Fig. 152. 
 
 This scheme which is not intended to represent anatomic relations, but 
 to make evident and comprehensible certain fundamental rules, plainly 
 teaches the following: the way by speaking and writing leads from C 1 
 principally over a. 
 
 Speaking: — :B — a — m — " jj, " — tongue 
 
 Writing: — :B — / a \ \ 
 
 ( I ) yo — gr — hand 
 
 In the same way not only in understanding speech (Ear — "alpha" — a — B), 
 but also in reading (Eye — o — a — B) the way leads over a. 
 
 V 
 
 m 
 
 Hence it is clear that an annihilation of a disturbs not only speech but 
 'B = Concept is represented in the above scheme only by a point, but in the brain, very 
 extensive cortical areas correspond to it. 
 
524 DISEASES OF THE BRAIN 
 
 also reading and writing. But, since in writing and reading a dismembering 
 of the entire word-sound (a — m) into its component parts (syllables and 
 letters) takes place, also the loss of m will be peculiarly damaging for reading 
 and writing. Writing and reading depend upon the condition that a and m 
 are intact! 
 
 These relations of dependence (especially that B be directly connected 
 only with a, further, that o enter into connection with B only over a — m) 
 are not fixed inevitably by the organization of the brain, but by the way the 
 function of speech is learned. 
 
 The deaf-mutes are the best illustration of this as they learn to write and 
 even to speak, though a does not exist in them at all. 
 
 The relations of dependence can therefore be modified by individual 
 predisposition, or peculiar development. For a time, it was believed even 
 that individual peculiarities, sometimes the visual, then the acoustic, and 
 again the motor predisposition gave grounds for questioning the regularity 
 of speech mechanism altogether, that a great number of men with motor 
 predispositions (Moteurs) could suffer the loss of acoustic speech com- 
 ponents without injury to their speaking, so that they found innervations to 
 speech directly from conception (i. e., in other words: the Concept evokes 
 the motor components). Or that a great number of optically predisposed 
 men (Visuels) are not disturbed in reading when a is injured, since they 
 would be in a condition to evoke directly from conception visual images of 
 letters. But it has been proved that the great fundamental relations, in con- 
 sequence of similar formation in the majority of men, correspond more 
 closely, than the exaggerated observations of individual differences have 
 caused some to think. One can not even count four approximately similar 
 types. There are only a few exceptions to the fundamental relations given 
 in the scheme. The predominating place of a is almost always preserved and 
 not overbalanced by individual predisposition, so that the motor word in the 
 majority of cases, is conditioned by the connection between a and B, and 
 written language usually also depends upon a. On the other hand, the rule 
 that written language depends also on m, has not a few exceptions. There 
 are people who suffer the loss of motor memories, without their written 
 language being affected. 
 
 For the quicker or slower restoration, for the finer differentiation, and 
 for the degree of the disturbances, individual differences must be considered. 
 So must the fluency in writing, which a man possessed before illness, be con- 
 sidered also. As a rule, however, we may assume an approximate similarity 
 in the organization of different human brains. 
 
 3. The Speech Regions in the Brain 
 
 Let us see, now, how the psychological scheme is carried out in the brain. 
 Here, before all, a fundamental fact is to be noted: that the speech-functions 
 
CEREBRAL SPEECH CENTERS 
 
 525 
 
 are performed chiefly by the left hemisphere. The opposite holds good 
 only in left-handed people, in whom the right hemisphere plays the greater 
 part. 
 
 Besides the left-handed people, 4 to 6% are ambidextrous — using both 
 hands equally well — and only in these do both hemispheres share equally 
 in the functions of speech. Now, it must not be thought that in right- 
 handed people — the majority of mankind — the right hemisphere not only 
 takes no part whatsoever in speech, but is also by nature unfitted for the 
 function of speech. This is not true. 
 
 The right hemisphere, may, in healthy people, share in speech to a 
 certain extent; but it is not able, by itself, to care for the functions of speech, 
 so that from disturbance in certain parts of the left hemisphere, speech in 
 
 Ft 
 
 Mot. A. ro - 
 
 p / Op.R 
 
 J. ope re. 
 
 Cfyr temp, prof 
 
 Al.+Agn 
 
 OeilS.Ji 
 
 Fig. 153. — -The speech-region (red). Mot.A , motor aphasia. Insel.A, insular aphasia. Sens. 
 A, sensory aphasia. A focus in X (in the temporal transverse convolution) may perhaps cause 
 pure word deafness. Al. Agr., Alexia and agraphia. 
 
 certain of its functions (expressive, perceptive), is destroyed or badly injured. 
 Were the right hemisphere as important to speech as the left, the same 
 results would follow a lesion in the right hemisphere, which is not the case. 
 Besides participating to a certain degree in the functions of speech in the 
 healthy individual, the right hemisphere may, after a lesion in the left, under- 
 take to some extent, after practice, the function of the injured half. There 
 is, however, never a complete assumption in the case of right-handed people, 
 and defects can always be proved by delicate tests. 
 
 All the rules to be stated hereafter hold good with only these limitations: 
 
 1. That ambidextrous people are not included. 
 
526 DISEASES OF THE BRAIN 
 
 2. That the right hemisphere (the left in the left handed) in the course 
 of time, can to a certain extent, to be discussed later, substitute for the left. 
 
 3. That no individual differences in predisposition or use exist. 
 
 In the left hemisphere there is an area that is principally concerned with 
 the functions of speech: the speech region, to which are to be added, those 
 regions in which lesions under the cortex result in serious losses of function. 
 But since the most varied combination of breaks in the fibres, so numerous 
 as not to be discussed in a short space, cause disturbances in speech, a 
 diagram of the speech region on the surface can give only a rough, approximate 
 representation (Fig. 153). Only the names of the most important 
 speech disorders can be marked upon it. If all the regions in which lesions 
 produce difficulty in finding words (amnesic aphasia) were included, the 
 region for speech would have to be drawn considerably larger. 
 
 The speech region, therefore, comprehends the lower, posterior part of the 
 convexity of the left frontal lobe, the island of Reil, the front portion of the 
 operculum of the anterior cerebral convolution {the Rolandic operculum) , the 
 posterior third of the temporal lobe, and one part of the lower occipital lobe. 
 
 These various parts of the speech region are not of equal importance. 
 
 4. Complete Motor Aphasia 
 {Cortical Motor Aphasia of Wernicke) 
 
 We must distinguish the Frontal speech region, the center nucleus 1 of 
 which is the posterior two-thirds of the inferior frontal convolution (Pars 
 triangularis and opercularis, the latter being Broca's region), but which, at 
 least in many people extends to the lower third of the anterior central gyrus 
 and the front part of the Island of Reil, probably even up to the middle 
 frontal convolution. And, moreover, the anterior and posterior boundaries 
 seem to differ somewhat in different individuals. Extensive lesions of this 
 region cause disorder, which, clinico-psychologically, corresponds to the 
 loss of motor word-components. The patients are still able to understand 
 what is being said to them — a is retained— but can not find the innervation- 
 complexes necessary to the utterance of words — that is, motor mnemic 
 images are lacking. They are therefore unable to utter words {word-dumb, 
 aphemic), or have left only a few fragments of speech. Because of the 
 dependence of written speech on m, writing is lost or badly disturbed in 
 the majority of people (see below) — except copying, which over o gr 
 
 eye hand 
 
 is possible without the use of (a — m). Even reading shows a gradual but 
 very distinct deterioration. Reading aloud is impossible because of the 
 inability to utter words, but the internal comprehension of reading is, for the 
 most part, not seriously affected, although seldom, intact. 
 
 1 Naturally, the word "nucleus" here, has nothing in common with the anatomical concept 
 of "nucleus." 
 
MOTOR AND SEXSORY APHASIA 527 
 
 The resulting clinical picture is known as complete motor aphasia, con- 
 sisting of: 
 
 Cessation of imitative speech. 
 
 Cessation of spontaneous speech. 
 
 Cessation of reading aloud. 
 
 Cessation of spontaneous writing. 
 
 Cessation of writing from dictation. 
 
 Difficulty in the comprehension of reading. 
 
 Comprehension of speech and copying are retained. 
 
 In mental excitement, the sufferer from motor aphasia (in contrast to the 
 anarthric) at times correctly utters a word or even a sentence which as a rule 
 he cannot bring to utterance. The speech of many people thus affected 
 shows improvement when singing. (Speech as an expression of the emotions 
 may be partly retained, when as an expression of thought it is lost. Thus 
 the motor-aphasic may swear roundly.) 
 
 For the retrogression of motor aphasia, and the peculiarities of returning 
 speech, cf . page 529. 
 
 For clinical pictures approximating complete motor aphasia, cf. under 
 insular aphasia, page 530. 
 
 5. Complete Sensory Aphasia 
 
 {Cortical Sensory Aphasia of Wernicke) 
 
 On the other hand, extensive focal injury of the temporal speech-region 
 (the posterior third of the superior temporal convolution with the temporal 
 transverse convolution and the adjoining anterior part of the supramarginal 
 gyrus) produces sensory aphasia (cf. also pages 531, 534, 536). 
 
 In sensory aphasia, the memory of word-sounds (a) is severely impaired. 
 Consequently, the main symptom is word-deafness. The patients hear, but 
 fail to understand what is said to them. And since the sound of the word is 
 not grasped, of course the meaning of the word is not understood. To grasp 
 the sound of the word, more is needed than to hear each separate tone. 
 Besides the formation of letters and syllables from the tones, and the ability 
 to grasp them, the co-operation of the memory of word-sounds is particularly 
 necessary. 
 
 Since, as we saw, acoustic components play a great part in speech, it is 
 disordered by injury to them. 
 
 There is not indeed a cessation of speech, as in motor aphasia, but there 
 are many derangements, and much confusion of words, syllables, and letters, 
 known as Paraphasia. 1 
 
 If the patient instead of knife says hammer, he has verbal paraphasia; if, 
 
 1 In the derangements of the sensory-aphasic patient, "getting stuck" plays an important 
 part. 
 
5 28 DISEASES OF THE BRAIN 
 
 instead of knife, he says life, he has literal paraphasia. If the latter is highly 
 developed, the result is an incomprehensible jargon: a jargon-par aphasia, 
 where one can understand, only by conjecture, what the patient means. 
 
 The degree of paraphasia varies greatly. 
 
 Many patients, suffering from sensory aphasia, can, by circumlocutions, 
 avoid the words they do not command, and utter a lot of phrases, which 
 while correct in themselves, are conspicuous by their lack of concreteness. 
 Then the serious disturbances in speech appear only when one lets them name 
 concrete objects (knife, chair, window, etc.). 
 
 The sensory aphasic in contradistinction to the motor aphasic, even in 
 an advanced degree of speech-disturbance, commands a great number of 
 letters and. syllables, the expression of which causes him no trouble; but he 
 does not always use the fitting ones, and often combines them so confusedly, 
 as to be understood only partially, or not at all. 
 
 Such a patient is even usually talkative (scarcely realizing that his speech 
 is unintelligible), while the sufferer from motor aphasia generally remains 
 silent, hesitating to attempt speech (noticing his errors because of the intact 
 acoustic word (a)). 
 
 Since written speech depends on a, it is disturbed even in sensory aphasia, 
 the writing being, for the most part, better than in the case of motor aphasia. 
 In sensory aphasia, the patient writes paragraphically, i. e., confusing the 
 letters, as he does in speech, whereas reading is more affected than in motor 
 aphasia. Alexia persists, more frequently only severe dyslexia. Reading 
 aloud is paralectic, as speaking is paraphasic, and even the internal compre- 
 hension of speech is, for the most part, seriously disturbed. 
 
 The clinical picture of sensory aphasia, is therefore, as follows: — Ces- 
 sation of speech understanding, or serious disturbance. 
 
 Spontaneous speech: paraphasic in varying degrees, with comparative 
 wealth of syllables, often indeed of words. 
 
 Imitative speech : cessation or serious paraphasia. 
 
 Writing: paragraphic (spontaneous and dictated). 
 
 Copying: retained. 
 
 Reading aloud: paralexia. 
 
 Understanding of reading: seriously affected. 
 
 The main differences, then, between motor and sensory aphasia are: 
 
 Motor Aphasia Sensory Aphasia 
 
 Speech: Lost. Abundant, but paraphasic. 
 
 Understanding: Retained. Lost. 
 
 Understanding Slightly Seriously disturbed, 
 
 of reading: disturbed. 
 
 Now, in sensory aphasia, word-deafness commonly departs after some months 
 (by substitution of the right hemisphere). By strict tests, however, defects 
 
TOTAL APHASIA 529 
 
 in the understanding of words may be found years later. Paraphasia, para- 
 graphia and paralexia are usually more lasting. Therefore one may not 
 always expect a high degree of word-deafness from an old injury in the left 
 temporal lobe. 
 
 While, from mere destruction of the left temporal speech region, not loss 
 of speech, but paraphasia results, an additional focal injury in the right 
 temporal lobe may cause word-deafness. This word-deafness happens in an 
 entirely different manner from that of motor aphasia: all stimuli coming 
 from the outside have been withdrawn from the motor- word center. In these 
 two-sided focal injuries in the temporal lobes, the word-deafness remains 
 stable, and should these focal injuries occur in certain places, complete 
 cerebral deafness persists. Practically, therefore, temporal word-dumbness 
 is differentiated from motor aphasia by its concomitant word-deafness, even 
 common deafness. (Cf. also pages 534 and 356.) 
 
 The word dumbness of motor aphasia resulting from extensive frontal 
 focal injury, is quite lasting. But even in this case, after considerable time — 
 though in many individuals never — a certain retrogression may take place; 
 after some months when the focal injuries are small, or when there are favor- 
 able compensating conditions. 
 
 First substantives and separate infinitives return, and adjectives without 
 particles, without inflection and declension, one after the other. (Agram- 
 matism or Telegram speech, i. e. Took walk, dizzy, sick, fall, hospital — as 
 the narration of the course of his illness.) 
 
 By this telegraphic style, the speech of motor aphasia, after a certain 
 retrogression, is distinguished from the speech of the sensory aphasic, which 
 commands the inflexions and forms of speech, uses many well-built circum- 
 locutory phrases, but can not find concrete nouns and verbs. 
 
 Furthermore, the former is distinguished by the continuing difficulty in 
 articulation. In improvement in motor aphasia, imitative speech returns 
 before spontaneous speech, and can become fairly good, though spontaneous 
 speech long remains inadequate. 
 
 6. Total Aphasia 
 
 More frequently than a lesion confined to the frontal or temporal speech 
 region, we find, as a result of the arterial distribution bringing all the blood, 
 in the whole region of speech, through the Art.foss. Sylvii, lesions, that affect 
 both regions, and therefore causing total or almost total (motor and sensory) 
 aphasia. Following the retrogression of the word-deafness, one sees, years 
 later, a clinical picture, in which the symptoms of motor aphasia predominate. 
 Word-dumbness conceals the paraphasia; the disturbance of speech under- 
 standing is no longer very serious. 
 
 Writing and reading, since a and m are affected, are very poor. Hence 
 
 34 
 
530 DISEASES OF THE BRAIN 
 
 it happens, that old cases, in which the lesions occur in both speech regions, 
 are often classed clinically only as motor aphasia. In these cases, disturb- 
 ances in speaking, writing and reading, are particularly stable. 
 
 This clinical picture in its entirety of motor and sensory aphasia was 
 named by Wernicke " cortical." He assumed that the cortex was the seat 
 of the mnemic images in question, and that only the loss of mnemic images 
 gives rise to the complete symptoms portrayed. Naturally, this must not be 
 interpreted so literally as to mean that the lesion must affect only the cortex. 
 This seldom happens; usually, not only the cortex but also the underlying 
 white matter is destroyed. 
 
 This, however, makes no change in the clinical picture, since the loss of 
 the projection and association fibres that run through the white matter, can 
 cause no additional disturbances, when once the center itself is destroyed. 
 
 7. Insular Aphasias 
 
 Between the frontal and temporal lobes lies the insular (Island of Reil) 
 speech region. Focal injuries in the Island of Reil give rise to very different 
 clinical pictures, which to this extent, resemble motor aphasia, in that they 
 disturb the expressive phase of speech. But on the other hand, they injure 
 the understanding of it only under circumstances to be more definitely 
 described. They are: 
 
 1. Possibly by injuring the cortex of the insula, since, as we have men- 
 tioned, the front part of the insular cortex may belong to the motor speech 
 center. 
 
 2. When they lie subcortically between the cortex and the lenticular 
 nucleus, by cutting through essential bundles of white fibres, which connect 
 a with m, namely, the external capsule and the extreme capsule. This 
 interruption of part of the paths a — m causes difficulty in finding words, 
 paraphasia, and injures imitative speech, but that less than spontaneous 
 speech, since this simplest function of speech is naturally capable of most 
 resistance. As long as some part of the connections a — m are retained — all 
 of which by no means pass through the insula — imitative speech is possible, 
 if not wholly intact. This is directly contrary to the older hypothesis, which 
 held that in cases of insular injuries, it is precisely imitative speech that is 
 the most seriously disturbed. Because of the dissociation of a and m, 
 written speech suffers more or less, since, in the majority of people, it is 
 dependent upon the internal word's being intact (a — m). 
 
 3. Frequently so-called focal insular injuries extend into the white 
 matter of the third frontal convolution and the Rolandic operculum. In 
 this case, we have besides the breaks (mentioned under 2) between a and m: 
 1. a break in the left centrifugal projection path to the bulbar cells; 2. inter- 
 ruptions in the fibres that pass from m to the Rolandic operculum; 3. of the 
 
PURE WORD-DUMBNESS 531 
 
 corpus callosum fibres; 4. destruction of the Fasc. arcuatus (cf. page 471), 
 another very important connective path between a and m. 
 
 Hence, a break between a and m, would, on the one hand, be more vital 
 than in two (2); on the other hand, a subcortical break of the pathways 
 leading from m to the periphery exists. Therefore one finds clinical pictures 
 closely approximating total motor aphasia: very poor, almost destroyed 
 spontaneous speech, almost no imitative speech, and disturbances in written 
 speech. Such insular focal injuries are of frequent occurrence. 
 
 4. Often the focal insular injury extends posteriorly down into the 
 white matter of the temporal lobe, and then it may injure the understanding 
 of speech. 
 
 From this it is clear, that focal insular injuries may have different effects, 
 according to their situation and extent; they all cause, however, greater or 
 less disturbance in the expressive phase of speech. 
 
 8. Pure Word-dumbness 
 
 Pure Motor Aphasia ("Subcortical" of Wernicke) 
 
 Besides the total motor aphasia (cortical of Wernicke), described above, 
 another form occurs, but more seldom, pure word-dumbness (subcortical 
 form of Wernicke) . In this form only expressive speech, that is, spontaneous 
 and imitative (by imitative is meant ability to repeat the words of others), 
 is lost, whereas written speech (writing and reading) is retained. In some 
 people, as has been mentioned before (page 524), written speech is more 
 independent of motor- word memories, and can be maintained by the acoustic 
 word alone. In this way, the rarer cases, in which the lesion is the same, 
 as in total motor-aphasia, and yet written speech is retained, are to be 
 explained. The majority of people, especially of the uneducated have to 
 spell the word internally in writing (and reading), others (especially those 
 well versed in writing and reading), need the motor word less frequently or 
 not at all. The latter, in case of cortical focal injury in the anterior speech- 
 region will show pure word-dumbness. 
 
 This would be pure word-dumbness by destruction of the motor speech 
 center (first form of pure word-dumbness). 
 
 In the majority of people the clinical picture of pure word-dumbness 
 is brought about by the fact that the motor center for speech is altogether 
 or largely retained, but cut off from the operative muscular apparatus of 
 speech. Under such conditions the motor images are spared, and remain 
 at the service of the acts of reading and writing, but the escape of stimuli 
 into the bulbar cells is prevented. In this case internal speech is retained, 
 the memory not impaired, so that we dare scarcely call it aphasia. But 
 since there is as yet no question of an injury in the outgoing neuro-muscular 
 
532 
 
 DISEASES OF THE BRAIN 
 
 speech apparatus, the disturbance belongs in no case to anarthria, but, as a 
 break between the memory apparatus and the projection apparatus, lies 
 between the two. Now, since the "purity" of this word-dumbness is 
 always only approximate, and as a slight admixture of disturbances of 
 internal speech can almost always be proved present, these cases are usually 
 and justly classified under the aphasias. 
 
 In the belief, that in pure word-dumbness, the cortical part of the center 
 for motor images is retained, and only the white matter and fibres running 
 to the speech centers are interrupted, Wernicke called this form "subcortical 
 aphasia." Since the older hypothesis of a direct path of projection (speech 
 path) from the motor speech center to the bulbar cells has become doubtful, 
 and it is far more probable, that from the motor speech center, the stimuli 
 reach first the cortical center of the hypoglossal and facial nerves ("/*" in 
 the scheme), on both sides in the inferior third of the frontal convolution 
 (Rolandic operculum), and from there only to the bulbar cells, one should 
 rather think of a break in these connections between the motor speech 
 center and both sides of the anterior central convolution, which can actually 
 be caused by subcortical focal injuries in the third frontal convolution and 
 the adjacent white matter. (Break in the pathways from m to the left side 
 of the Rolandic operculum and in the corpus callosum paths to the right side 
 of the Rolandic operculum.) 
 
 The old conception of subcortical aphasia — better replaced by pure word- 
 dumbness — may not find anatomically a strict application in words: the 
 meaning is that a break in the paths between the motor speech centers and 
 the muscular apparatus of speech is concerned. Naturally a subcortical 
 focal injury 7 outside of these connective paths, can interrupt so many associa- 
 tion fibres of the motor speech center with the whole cortex, that the motor 
 speech center is almost isolated, and therefore almost as funcrionless as if it 
 were destroyed. Such a subcortical lesion would give a clinical picture of 
 total motor aphasia. On the other hand, a subcortical lesion, according to 
 situation and extent, may be of such a nature, as to spare partly the fibres 
 passing from the speech center to the cortical centers of hypoglossal and 
 facial nerves on both sides, perhaps the commissural fibres on the right 
 passing through the corpus callosum, and then a subcortical focal injury 
 would not cause even pure word-dumbness. Finally a focal injury may 
 destroy part of the cortex without wholly incapacitating it, but with an exten- 
 sion into the white matter, break the connections with both sides of the 
 Rolandic operculum. Then this focal injury, partly cortical and partly 
 subcortical, would have a similar effect as a pure subcortical break of 
 the connections named. It is evident, that the conceptions of cortical and 
 subcortical aphasia are not anatomically to be taken literally, but mean as 
 much as incapacitation of the cortical speech center itself and a mere break 
 between this and its subordinate centers. In realitv, manv different combina- 
 
PURE WORD-DEAFNESS 533 
 
 tions of lesions may form one or the other clinical picture. But it has been 
 proved, that in the case of extensive destruction of the cortex the usual result 
 is the clinical picture of total motor aphasia, whereas pure word-dumbness 
 results, in the majority of cases from subcortical lesions. 
 
 Excluding that minority, whose written speech is independent of the 
 motor speech center, we may say: lesions, that spare wholly, or partly the 
 cortex of the frontal speech regions, cause only the loss of spoken speech, 
 and spare written speech. (Pure word-dumbness, second form of pure 
 word-dumbness.) Those lesions, however, that to a great extent destroy the 
 cortex of the frontal speech region, usually affect written as well as spoken 
 speech. A lesion, moreover, that breaks the left cortical bulbar tract, in 
 addition to a second lesion which breaks the commissural paths between 
 m and the right Rolandic operculum, likewise causes pure word-dumbness. 
 
 9. Pure Word-deafness 
 
 Likewise we have, beside total sensory aphasia, an isolated or pure 
 word-deafness {Lichiheim's Disease; subcortical sensory aphasia). 
 
 In this disease, only comprehension, and as a natural consequence, 
 imitative speech and dictated writing are lost, whereas speaking, writing, and 
 reading are retained. Paraphasia, paragraphia, and paralexia are wanting 
 to make this total sensory aphasia. That a focal injury, which destroys 
 the sensory speech center, causes pure word-deafness (because an individual 
 independence of spoken and written speech of auditory images is in question, 
 corresponding to the oft discussed relation when motor- word images are 
 disturbed) is necessarily a thing of rare occurrence. 
 
 Even the dependence of written speech on the acoustic word has fewer 
 exceptions than that on the motor-word. In a case of pure word-deafness, 
 one must think of the persistence of the substratum of auditory word images, 
 and of mere exclusion of these from acoustic stimuli. This, as Lichtheim 
 has taught, is caused in the first instance, by a subcortical focal injury in 
 the left temporal lobe, situated so as to break the incoming of all acoustic 
 stimuli to the left temporal lobe, and possibly, too, the corpus callosum 
 connection between the hearing center of the right and that of the left hemi- 
 sphere. Then the possession of auditory word images is established by the 
 fact that reading, speaking, and writing are intact, whereas the exclusion of 
 these auditory word images from all acoustic stimuli, causes the loss of 
 speech-comprehension. A second possibility as to the establishment of pure 
 word-deafness occurs, if with Flechsig, one separates the acoustic projection 
 center sharply from the mnemic acoustic center (cf. Fig. 131). Then, the 
 destruction of this center (i. e., particularly of the transverse convolution 
 in the temporal lobe) deprives the left hemisphere of acoustic stimuli and 
 hence cuts off auditory word images from the periphery, whereas they could 
 
534 DISEASES OF THE BRAIN 
 
 still develop their inter cortical effectiveness in speaking, writing, and reading. 
 This would be pure word-deafness due to cortical focal injury (in the trans- 
 verse convolution). It is doubtful whether pure word-deafness could arise 
 from a break in a path alpha — a, since it is doubtful, whether alpha and a are 
 connected by a complete set of association fibres. 
 
 Pure word-deafness persists, provided the right temporal lobe does not, 
 in the course of time, also take the place of the left, which, in the case of 
 subcortical focal injuries, often fails to happen. Here, too, as in total sensory 
 aphasia, the comprehension of the spoken word is already lost. Tones are 
 heard, but only as a strange confused noise. If the comprehension of the 
 spokm-wordis lost, naturally the meaning of the word is not grasped, i. e., the 
 comprehension of the meaning of the word is wholly lost. 
 
 To establish a case of pure word-deafness, it must be proved that hearing 
 is possible. If hearing is seriously impaired on both sides by disease of the 
 labyrinths or of the auditory tracts or centers, the comprehension of speech 
 may be lost as a result of defective hearing, alone. Then we have pseudo- 
 speech-deafness, a mere consequence of defective hearing. Since Bezold 
 proved, that total loss of the tone range b' to g", or serious weakening within 
 this range, eo ipso, destroys comprehension of speech, one must prove not 
 only the continuous range of tones but also that the patient hears with suf- 
 ficient clearness within the range of the tones concerned. It is not suf- 
 ficient to prove the existence of hearing, by received perception of noises, 
 whistling, clapping of hands, ringing, etc., since if the tone-range mentioned 
 above is lost, speech will not be understood, even if the hearing of the other 
 tones of the entire range of tones is intact. Furthermore, to distinguish 
 subcortical sensory aphasia from loss of comprehension of speech, due to gen- 
 eral deafness, this fact serves — that the former is never absolutely pure, but 
 that separate paraphasic or paragraphic symptoms prove that it is not a 
 question of mere defect in hearing. 
 
 There is besides a still rarer form of speech-deafness, in which letters, 
 syllables, and short words can be comprehended and repeated, but larger 
 words and sentences are not understood, not even in the sound of the words. 
 With this there is only very slight paraphasia, paragraphia and paralexia, so 
 that this form (because of the small proportion of disturbances in internal 
 speech) approximates pure word-deafness. A cortical focal injury in the 
 speech center, which injures auditory word images less, than their communi- 
 cation with the acoustic stimuli, might be the cause. 
 
 10. Transcortical Aphasias 
 
 (Including Amnesic and Visual Aphasia) 
 
 By transcortical aphasias, Wernicke, indorsing the theory and classifica- 
 tion of Lichtheim, understands those, in which the motor and sensory speech 
 
TRANSCORTICAL APHASIA 
 
 535 
 
 at 
 
 -■2 
 
 --o 
 
 Mouth 
 
 Wernicke's 
 scheme, i, Cortical sen- 
 
 Ear 
 Fig. 154.- 
 
 centers themselves, and their connection with each other and with the per- 
 iphery are retained, but one of the two is cut off from the perception area, 
 i. e., from all the rest of the cortex. Psychologically, the word (a — m) is 
 intact, but one of its components is cut off fromB. The complex ear (a — m) 
 mouth (Fig. 154) is therefore free. 
 
 W. thought this was caused by a break in the association fibres which 
 converge from the entire cortex to one of both speech centers. 
 
 In his scheme, disturbance in a and m means cortical aphasia, a break in 
 the pathway between m and a, subcortical aphasia, and a break in the paths 
 from B to m or to a, transcortical aphasia (correspondingly motor or sensory). 
 
 Clinically this is marked especially, by the ability to repeat words (the 
 comprehension of spoken speech is retained) , inasmuch 
 as the section ear — a — m — mouth is free, but that either 
 the word can not be found from the meaning (transcor- 
 tical motor aphasia, 6) or that the meaning of the word 
 heard cannot be found (loss of comprehension of 
 word meanings) transcortical sensory aphasia (3). 
 The disturbances in writing and reading ascribed to the 
 transcortical forms (spoken speech, dictated writing 
 retained) are realized only approximately. The clin- 
 ical reality of these transcortical forms, with this limi- 
 tation, is certain: we often have patients, who can re- sorv aphasi£i 
 peat words (sound comprehension retained), but who tical sensory aphasia. 3, 
 
 , . . n ... , , Transcortical sensory 
 
 do not understand them readily (meaning comprehen- ap hasia. 4, Cortical mo- 
 sion lost) or have difficultv in finding words from their tor ap^ia- 5, Subcor- 
 
 ' . tical motor aphasia, o, 
 
 meaning. Often these Pwo are associated : difficulty in Transcortical motor apha- 
 the comprehension of the meaning of words and very ^ (^^1^° n ot a con- 
 great difficulty in finding words, though imitative firmed). 
 speech is retained. 
 
 But anatomically, the relations assumed by Wernicke are not to be 
 realized in the simple manner, taken from the brain, as he supposed. It is 
 not merely the case that, when imitative speech is lost, the focal injury is 
 in the center, and when it is retained, that the focal injury lies in 
 the tracts converging to the center. The principle, in Wernicke's suppo- 
 sition, that when imitative speech is lost, the real conveyors of the motor or 
 sensory word are destroyed, and that when it is retained, they are retained, 
 but can not be reached from the superior cortical seat of perception, holds 
 good in part, but under very different anatomical conditions, and sometimes 
 the focal injuries are so situated that one does not, without further difficulty, 
 see this effect. The empirical conditions, and the knowledge that the direct 
 path B — m is of no great importance, that moreover, spontaneous speech 
 mainly uses the same pathway as imitative speech, namely the path a — m, 
 necessitate a modification of this part of Wernicke's view. Retention and 
 
536 DISEASES OF THE BRAIN 
 
 loss of imitative speech depend on other conditions beyond the situation of 
 the focal injuries, that is, on the degree of lesion. Imitative speech, naturally, 
 is,, on the whole, that function of speech, most capable of resistance, so that 
 slighter lesions of the same region, the destruction of which causes total 
 aphasia, permit of some imitative speech. This is true also of the paths that 
 lead to m (a-m). In partial lesions, the powerful stimuli, which come from 
 the periphery, are allowed to pass, while the weaker spontaneous stimuli 
 coming from B are not. 
 
 The word transcortical includes a clinico-symptomatological, an anatom- 
 ical' and a psychological set of conditions, all three of which are not always 
 fulfilled together. We shall use the word here only in its clinico-symptomato- 
 logical sense, i. e., consider as transcortical those forms only in which imita- 
 tive speech is retained. 
 
 This is not the place to discuss the value of the other meanings of the 
 word transcortical. 
 
 (A) Transcortical motor aphasia is here, the loss or serious disturbance of 
 spontaneous speech, associated with retention of imitative speech. 
 
 This is found in: 
 
 I. Slight injury of m itself, which destroys the possibility of stimulating 
 the center of perception from the periphery (via a), without injuring those 
 fibres in the center that convey the motor-word to such an extent that they 
 can not be innervated by the stronger stimulus of the spoken word. 
 
 II. Slight injury of the paths from a to m, a case already mentioned under 
 focal insular injuries (page 530) and of the accessory paths from B to m from 
 the same cause. 
 
 III. When one has to consider the possibility, that the return of imitative 
 speech has become a function of the right hemisphere. 
 
 In cases 1 to 3, imitative speech is better than spontaneous speech, but 
 nevertheless very much impaired and faulty. 
 
 IV. The requirement of the retention of imitative speech is more strictly 
 complied with in so-called amnesic aphasia or verbal amnesia, in which, it is 
 true, even spontaneous speech is not wholly lost. It is clinically, and prin- 
 cipally, a slighter form of transcortical motor aphasia, though the mode of 
 its origin be other than the one accepted by Wernicke for the latter case. 
 
 There is great difficulty in finding words, but if the word is suggested, 
 it is immediately recognized as the right one, and repeated with ease and 
 correctness. In this verbal amnesia, it is most difficult to find nouns and 
 verbs for concrete objects or actions, whereas in contrast to forms 1 and 2, 
 abstract nouns and inflected parts of speech, particles, prepositions, etc., as 
 well as inflections and declensions are retained. This form is found under 
 manifold conditions, always when one of the stations by which the process of 
 speech must pass, before reaching the motor center, is slightly injured. 
 Therefore : 
 
VISUAL APHASIA 537 
 
 1. When the sensory (temporal) speech center is but very slightly injured 
 (say, by moderate atrophy). This small injury does not destroy imitative 
 speech and comprehension, but makes difficult the wakening of auditory word 
 images from the perception of them (the function most easily disturbed) . 
 
 2. When the connections between the sensory speech center (a) and B are 
 slightly injured, because of which it is difficult to find words, while the 
 meaning of the word heard is still comprehended. 
 
 3. When the perceptive -region itself, that is, the entire cortex is injured, 
 even before the perception is so seriously disturbed, that verbal amnesia is 
 lost in imbecility, the pathways to the sensory speech center are, in their very 
 beginnings, injured. 
 
 In diffuse processes (paralysis, senile atrophy, arterio-sclerosis) frequently 
 several of the conditions named under 1 to 3 are fulfilled, so that in those . 
 cases, amnesic aphasia is quite usual. 
 
 But, moreover, focal injuries back of a, which are not sufficiently extended, 
 to produce a case of transcortical sensory aphasia (to be discussed later), 
 reduce spontaneous speech to the lowest degree, without in the least affecting 
 imitative speech. 
 
 The so-called visual (optic) aphasia is only a peculiar form of amnesic 
 aphasia. According to its definition, objects seen can not be named, and 
 those objects, that we know exceedingly well and mostly by sight, can not be 
 designated in free speech. On the other hand, objects perceived by any other 
 sense, for instance, that of touch, it should be possible to name. In reality, 
 the most frequent form is an optic-tactile aphasia, i. e., neither by sight, nor 
 by touch, is the name of an object found, though it may be easily found by 
 ear. A trumpet, for instance, that has been seen and touched, can not be 
 named, whereas as soon as it sounds, the name is immediately suggested. 
 In this visual aphasia, it is generally an extension of amnesic aphasia, that 
 we find, in which, the inability to name objects (in contrast to common parts 
 of speech and abstract nouns) is more than usually conspicuous, in which 
 the perception of the object creates less favorable conditions for naming it, 
 than exist in free speech, and naming by acoustic characteristics proves to be 
 easier. 
 
 This hall-mark of amnesic aphasia is found especially in cases of abscesses 
 in the temporal lobes, which mostly lie at the base of the temporal-occipital 
 lobes, furthermore in other injuries in the connecting pathways between the 
 temporal and occipital lobes, even in the occipital lobes themselves. They 
 have this in common, namely, that they interrupt connective paths between 
 a and the occipital lobe, the seat of the essential optic components of 
 perception. 
 
 Amnesic and especially optic aphasia is transcortical, even in that meaning 
 of the word that has been heretofore neglected: the hindrance in speech does 
 not lie in the word centers themselves (a or m), nor in their connections with 
 
538 DISEASES OF THE BRAIN 
 
 one another and the periphery, but beyond the centers, on the path that must 
 be transversed by the stimulus before it can reach the word centers from 
 preception. 
 
 Since a is for us an intermediate station for the stimulus from C to m, in 
 this sense every hindrance in expressive speech caused by a lesion in a is 
 transcortical, especially for instance, the word-dumbness due to bilateral in- 
 juries in the temporal lobes (see page 529). In this case, however, imitative 
 speech also is lost so that the distinctive feature that clinically and particularly 
 characterizes transcortical aphasias, is absent. This difficulty we may only 
 point out here. 
 
 (B) Transcortical Sensory Aphasia. — We have seen that an injury to 
 the connective pathway between a and B, that is, a partial interruption only, 
 causes transcortical motor aphasia, seriously hindering spontaneous speech 
 (that is the stimulus in the direction B to a), but does not destroy the pathway 
 in the opposite direction a to B : the connection of the percept with the word, 
 the comprehension of the meaning of the word. 
 
 If, however, a total interruption occurs in the paths from B to a, as op- 
 posed to partial interruption, or if B itself, is seriously injured (asymbolia in 
 the older sense of the word) we have transcortical sensory aphasia: imitative 
 speech is retained, often occurring irrepressibly (echolalia), but comprehen- 
 sion of what is spoken is lost. This happens, because no concept joins the 
 auditory word image evoked in a, but the stimulus, to which the path to B is 
 closed, flows out into the free path a to m of imitative speech. Anatomically 
 transcortical sensory aphasia is shown frequently by marked atrophy in the 
 neighborhood of the first temporal convolution, that is, in the second and third 
 temporal convolution and even further back (marked atrophy in the white 
 matter of the temporal lobe). It occurs often side by side with general 
 atrophy and corresponding injury in the perception centers. (Asymbolic 
 disturbances in the older sense.) Several focal injuries, moreover, in the 
 posterior half of the brain can bring about this isolation of a, which spares 
 only the connection with m. 
 
 Now, it must be remarked, that, if only a partial interruption in a — B, 
 brought about almost complete loss of expressive speech, a greater interrup- 
 tion would certainly alter expressive speech in a considerable degree. As a 
 matter of fact, we have almost always associated with transcortical sensory 
 aphasia a serious disturbance in spontaneous speech. Hence transcortical 
 sensory aphasia, as a rule, is associated with transcortical motor aphasia; 
 only the sound of the word is comprehended, and parrot-like imitated, but 
 neither is the meaning understood, nor spontaneous speech to any extent 
 possible. A complete restriction to the symptom-complex actually found in 
 transcortical sensory aphasia (i. e., imitative speech, but lack of compre- 
 hension in the meager spontaneous speech) is possible only in a person in 
 whom, exceptionally, the pathway B — m is particularly efficient. 
 
SENSORY APHASIA 539 
 
 Summary of Temporal Lesions. — To summarize what has been said 
 about sensory aphasic disturbances, an interruption of the path leading to 
 the left auditory center (including the corpus callosum connection), as well 
 as one in the left auditory center itself (middle third of the superior temporal 
 convolution and transverse convolution), creates pure word-deafness. A 
 destruction of the "sensory speech center" (posterior third of the superior 
 temporal convolution and that part of the supra marginal gyrus, lying 
 directly behind it) causes word deafness combined with paraphasia, para- 
 graphia and paralexia. Where there is deafness to the word-saund, we find, 
 in consequence deafness to the word-meaning. 
 
 A total isolation — with the exception of the connection with m — of the 
 sensory speech (serious atrophy of the whole temporal lobe, extensive focal 
 injuries in the depths back of and around the sensory speech center), destroys 
 only the comprehension of the meaning and not that of the sound of the word, 
 which depends on imitative speech, and at the same time produces the 
 greatest difficulty in finding words. But if the sensory speech center or its 
 connection with B is but slightly or partially disturbed, only amnesic aphasia 
 results (in the second case, especially, optic aphasia), whereas compre- 
 hension of word sound and meaning are retained. 
 
 It is now self-evident, and this is true of all cerebral lesions, that diseases 
 affecting the left temporal lobe, whether they be neoplasms or focal injuries 
 of vascular origin, only exceptionally, restrict their destructive and far reach- 
 ing consequences to the substratum of one element or an element connective, 
 as sharply differentiated in the scheme. According to their various situa- 
 tions and extent, and to the participation of many pathways, represented by a 
 single line in the scheme, various combinations of symptoms appear. Loss of 
 comprehension of word-sound and word-meaning, difficulty in finding words 
 and paraphasia, disturbances in writing and reading, combined in varying 
 degrees, form the typical clinical pictures of the diseases which can be classi- 
 fied only approximately according to theoretically developed forms. Pure 
 word-deafness, total sensory aphasia, and "transcortical" sensory aphasia, 
 are only three particularly striking types of sensory aphasia, the diagnosis 
 of which has great instructive value. 
 
 In order to comprehend all real differences, one would, in each case 
 have to take into account, the extent to which the cortex, the projection — 
 association — and commissural fibres are affected, and would moreover, have 
 to know clearly the function of each of them, a knowledge which we have 
 unfortunately by no means attained. 
 
 Disturbances in Written Speech 
 {a) Alexia and Agraphia; (b) Pure Alexia; (c) Pure Agraphia 
 We noticed that written speech was usually affected with spoken speech: 
 writing, seriously in both main forms of aphasia; reading, seriously in sensory, 
 
54° 
 
 DISEASES OF THE BRAIN 
 
 less seriously in motor aphasia. Only in the so-called pure forms was written 
 speech retained intact. 
 
 There are disturbances in written speech, that are almost isolated, i. e., 
 reading and writing are seriously impaired, while little harm is done to the 
 other functions of speech. 
 
 Focal injuries, situated directly behind the sensory speech center, that is, 
 in the angular gyrus and the upper layers of its white matter, cause agraphia 
 and alexia, associated with but slight symptoms of paraphasia, and difficulty 
 in finding words. Those focal injuries, however, which from the medial 
 
 surface of the center extend for- 
 wards into the deep white matter 
 of the angular gyrus, cause, 
 especially, pure alexia, i. e. writ- 
 ing is retained, reading lost. 
 With this right hemianopia is 
 almost always associated, 
 whereas superficial focal in- 
 juries in the angular gyrus, 
 which cause the loss of both 
 reading and writing, often do 
 not produce hemianopia. 
 
 The simplest explanation of 
 these statements, is as follows : 
 
 The optic radiation runs 
 through the deep white matter 
 of the angular gyrus, outside of 
 the posterior horn through the 
 white matter of the occipital 
 lobe, to the calcarine region, 
 the left and right visual center 
 (s and s'). From here pass connecting fibres (not shown in Fig. 155) to the con- 
 vexity of the occipital lobe, the seat of visual images (form memories) (s — o). 
 These are registered mostly in the left, but also in the right occipital lobe. The 
 connective fibres from the right occipital lobe pass through the splenium 
 to the convexity of the left occipital lobe (s' — o). From the sensory speech 
 center an association path passes to the convexity of the occipital lobe (a — 0) . 
 From o to the hand center (gr) passes the path by which visual directions 
 are sent to the hand (Fig. 155). 
 
 Now, if a focal injury destroys . (1) the angular gyrus, starting from the 
 convexity, it interrupts the path a — o. The consequence is, that the sound 
 pictures of the letters aroused in a, do not awaken the corresponding visual 
 images in o, which is necessary for writing; whence agraphia. In reading, 
 the opposite takes place; the stimuli of both fields of light are not carried 
 
 Fig. 155. — (Explanation in the text.) Focus 1 
 causes alexia and agraphia. Focus 2 pure alexia with 
 hemianopia. Focus 3 pure agraphia of the right hand 
 only. Focus 4 pure bilateral agraphia. 
 
ALEXIA: AGRAPHIA 541 
 
 over to a, so that images of letters fail to call up their sounds (for the sake 
 of simplicity, we pay no attention to the secondary connective path — m), 
 and what is seen, is not understood; whence alexia. Because of the inter- 
 ruption in an important path from o to a, there results difficulty in finding 
 words, and because of the proximity of the focal injury to a, there is fre- 
 quently slight paraphasia. But if the focal injury lies in 2, it interrupts, 
 on the one side, the optic radiation, hence right hemianopia, on the other, 
 the commissural paths from the right occipital lobe (s' — 0) so that no optic 
 stimulus reaches the left o. The visual stimuli, that reach only the right 
 occipital lobe, do not reach a, and therefore call up no auditory images, so 
 reading is lost. But focal injury in 2 does not break the connections of a 
 and m with o, nor those between o and gr, so that there is no reason for the 
 loss of writing, hence pure alexia. 
 
 If a focal injury (3 and 4) interrupts the path from the left occipital lobe 
 to the center for the right (or the left) hand, pure agraphia appears, usually 
 in both hands, but under certain conditions, only in the right. Pure agraphia 
 is also often a partial symptom of apraxia of the hand affected. 
 
 If one considers, that in the brain, it is not, as in the scheme, a matter 
 of linear connection, but of numerous and various connections of fibres, 
 that these pass to the other categories of fibres, on various levels and in 
 various situations, that the size, extent, direction, form, and number of the 
 focal injuries create the most varied combinations of interruptions, it is 
 then clear, that the above linear scheme is merely a simple chart, but that, 
 in reality, manifold combinations and varieties may appear. Suppose for 
 instance, that focal injury 2 extends an outshoot so far laterally, that o — gr, too, 
 is interrupted, then we shall have alexia associated with agraphia. 
 
 Pure alexia is caused by a focal injury in the region supplied by the 
 posterior cerebral artery. 
 
 (b) Agnostic Disturbances 
 
 Word-deafness and reading blindness are really special manifestations 
 of disturbances of cognition. The patient in reading blindness sees, but 
 fails to understand what is seen. Perception is retained, but comprehension 
 lost. Reading blindness is agnosia for written letters, word-deafness 
 agnosia for word-sounds. In these receptive aphasic disturbances, it is a 
 question of agnosia for conventional signs, for the symbols of speech. 
 Speaking of agnosia, in the stricter sense, one means corresponding dis- 
 turbances for the things of the outer world, namely, for all sense impressions, 
 that are not symbols. (Object agnosia.) If say, a comb is seen, but its 
 significance not recognized, because the complex of visual perceptions does 
 not awaken those associations that normally appear, for instance, that the 
 comb is the thing with which we comb our hair, that it belongs with the 
 brush, etc., an agnostic disturbance is present. It is not a matter of making 
 
542 DISEASES OF THE BRAIN 
 
 the connection of the symbol and its import, but of the object's real bearing, 
 origin, purpose, etc. According to the sense affected, one differentiates 
 between acoustic, visual, tactile, gustatory and olfactory agnosia. 
 
 i. Acoustic agnosia or mind deafness is characterized by the lack of 
 comprehension of speech sounds and all possible auditory stimuli, such as 
 cries of animals, musical instruments, cracking of the whip, clinking of 
 money, etc. It occurs associated with sensory aphasia, when the focal 
 injury is in the left temporal lobe. 
 
 2. Visual agnosia or mind blindness. The patient can discuss the 
 form and even the color of objects, but they are strange to him; he not only 
 can not name them, when shown, as in visual aphasia, but can really not 
 recognize them. 
 
 Mental blindness results from lesions in the occipital lobes and generally 
 occurs when the focal injuries are bilateral. Usually by a lesion in the visual 
 center, or optic radiation, half or at least a quarter of the field of vision is 
 excluded, i. e., for this part of the visual field the person is, perceptively 
 speaking, blind. The comprehension of the impressions received by the 
 unaffected parts of the field of vision, is lost because of an interruption in the 
 association fibres, which run from the visual center to the seat of visual form 
 memories in the convexity of the occipital lobe, or because of the destruction 
 of the center itself or its connections with the other cortical areas. This can 
 be caused by various combinations of injuries, which affect the white matter 
 and the convexity of the occipital lobe, and those parts of the parietal lobe 
 lying directly in front of it. One of both injuries must always be on the left; 
 focal injuries to the right occipital lobe do not, except in left-handed people, 
 cause mental blindness. This proves, that the stability and delicacy of 
 visual images in the left hemisphere are greater than those in the right. 
 Indeed, in the majority of people the left hemisphere is so predominantly 
 the seat of visual images, that, in them, an injury that is only left-sided effects 
 a considerable degree of mental blindness. Mental blindness caused only 
 by a left-sided injury, occurs, in such cases, in the following manner: destruc- 
 tion of the left visual center or of the optic radiation entirely cuts off optic 
 stimuli from the right hemisphere (right hemianopia). The focal injury 
 further breaks the corpus callosum connection between the right occipital 
 lobe and the left hemisphere (splenium or forceps), whereby the visual 
 stimuli reaching the right field of vision, cause perceptions, but are not 
 understood, since they do not reach the seat of memories stored up predomi 
 nantly on the left side. At the same time there naturally occurs pure alexia, 
 since the mechanism of mental blindness, due to one-sided focal injury, 
 operates as in pure alexia. Still other combinations of lesions are possible. 
 
 Usually perceptive and gnostic disturbances are found together, so that 
 for one part of the field of vision there is blindness, and for that part of the 
 field that has been retained, mental blindness. 
 
MIND BLINDNESS 543 
 
 As a rule, the acuity of vision is also impaired, often, too, there is some 
 color blindness. Before one diagnoses mental blindness, it must, of course, 
 be proved that these perceptive disturbances are not so weighty, that it is not 
 due to the presentation of data, insufficient for the recognition of the objects. 
 (Pseudo-mental blindness.) 
 
 In many cases of mental blindness visual memories are lost, in others, 
 they merely can not be aroused by centripetal stimuli but the patient can some- 
 how, by description, or drawing, indicate the possession of memory. 
 
 Or, although the visual memories are really aroused, the associated memo- 
 ries of the other senses, tactile, acoustic, can not connect with them. Often 
 the reason of failure to recognize is that the composition of the concept of the 
 object from the various sensory data is impossible. The conceptions, then, 
 remain a chaos of simple impressions, which do not flow together to form 
 concepts of concrete objects. 
 
 Usually, not only form memories, but also color memories are injured; 
 the patient can not imagine the color of the frog, of the carriage, of the blood. 
 Occasionally color memories are retained; but it is an exception when form 
 memories are retained and only color memories lost. Usually, in mental 
 blindness, memories of the spatial order of things suffer. The patient can not 
 see "in his mind's eye," roads that were formerly familiar, and therefore 
 easily loses his way. 
 
 Not to be confused with disturbance in color memories (loss of color 
 images) or with disturbances in the perception of colors (color blindnes — tested 
 by sorting colors), there is a disturbance in naming colors, occurring now 
 with, now without the other two (inappositely named amnesic colorblindness). 
 In this case the patient can not give the right name to a color, just as he can 
 not correctly name other things. (One of the phenomena in amnesic and so- 
 called visual aphasia, that shows itself especially in names of colors and 
 persons.) 
 
 Amnesic aphasia is usually associated with mental blindness and alexia, 
 eventually also, with agraphia. 
 
 3. Tactile Agnosia (" Touch Crippling"). — In tactile agnosia the patient 
 does not recognize the object touched in spite of the presence of various and 
 sufficient incoming sensations (perceptions of touch, location, movement, 
 etc.). For example, forms are not recognized (astereognosis). Nor do 
 perceptions of moisture, cold, velvety softness, etc., lead to correct conclusions. 
 
 There are three kinds of tactile agnosia: 
 
 1. Either separate impressions are not correctly correlated, though 
 tactile memories are retained, that is, they do not correctly evoke the latter, 
 or 
 
 2. Tactile memories are lost, or 
 
 3. The association between them and the visual, auditory, etc., memo- 
 ries is broken. 
 
544 DISEASES OF TEE BRAIN 
 
 Anatomically, in tactile agnosia, focal injuries exist in the middle third 
 of the posterior central convolution, or behind it, in the parietal lobe, the latter 
 being especially concerned in the third associative form. 
 
 Not to be confused with tactile agnosia, there is that failure to recognize 
 through touch, which is caused by severe disturbances in sensation and by 
 lesions of sensor}' paths. In this case we find not an agnostic, but a per- 
 ceptive disturbance, and one should distinguish also, similarly caused dis- 
 turbances in form recognitionas, as perceptive astereognosis, from tactile 
 agnosia (see page 506). 
 
 4. Agnosias in the spheres of smell and taste are distinguished with 
 difficulty from the corresponding perceptive disturbances and have been 
 little studied. 
 
 The agnostic (dissolutory) disturbances that have been heretofore dis- 
 cussed, rest on the fact, that the members of the series of ideas essential to 
 cognition, split into their separate sensory components, or are injured by the 
 destruction of such a component; for instance, the optic. Frequently, how- 
 ever, a disturbance in recognition is caused by the fact, that ideas, intact in 
 their sensory elements, are combined in the wrong order, that perhaps the 
 connection between the partial conceptions and the whole concept, or the 
 correct association of cause, purpose, or characteristics with an object is lost, 
 that is, those associations, necessary to recognition, are injured that do not 
 precisely concern the connection of the sensory elements {ideational agnosia, 
 counterpart, and frequently companion of ideational apraxia). 
 
 Various associative and attentional disturbances thus affect the train of 
 ideas. The object is, for example, not recognized, because a " persevering" 
 idea, or an accidental sensory impression, or an emerging immaterial side 
 issue interposes and turns aside the train of ideas. 
 
 These ideational agnosias occur especially with diffuse mental processes, 
 furthermore as general effects of focal injuries, hence often accompany the 
 focal symptoms, possibly occur even as direct focal symptoms. They present 
 those disturbances in cognition, that one generally characterizes as "psychic." 
 
 If optic, tactile and acoustic agnosia are associated, we have total agnosia, 
 used in the older sense of total asymbolia. This presupposes such extensive 
 injuries, large lesions in both temporal, parietal, and occipital lobes, that 
 memory pictures themselves and their connection with understanding are 
 virtually destroyed. Then there is entire loss of comprehension. 
 
 (c) Apraxic Disturbances 
 
 1. General Remarks 
 
 In brain diseases, there is often inability to move the limbs, so as to carry 
 out the movements intended by the patient, so that even very familiar and 
 
APRAXIA 545 
 
 thoroughly learned movements can not be made, though neither palsy nor 
 ataxia is a sufficient cause. 
 
 For the patient can occasionally contract all muscles; he raises, lowers, 
 bends, stretches arm and hand often vigorously and once in a while executes 
 complicated movements, and is thus differentiated from the paralytic; but 
 just when he wishes to make a certain movement he is unable to do it. 
 Other apraxics can carry out such simple purposive movements, but the still 
 relatively simple movement complexes, such as greeting, beckoning, threat- 
 ening, lighting cigars, sealing letters, pouring out water, etc., they can not 
 execute. Objects are wrongly used, or the patient stares at them, being at a 
 loss as to what to do with them. 
 
 The limbs then can not be made to serve the purposes of life. As in 
 aphasia, the unparalyzed muscles of tongue, lips, and palate, can not be so 
 directed as to produce the word in mind, the apraxic can not make his para- 
 lyzed hand execute certain movements. The expressive-aphasic disturb- 
 ances present only part of the symptoms of apraxia, just as the receptive- 
 aphasic present part of those of agnosia, and it is only from historical 
 considerations that aphasic disturbances are treated separately. 
 
 The inability to perform purposive movements, and purposive groups of 
 movements in the every-day affairs of life, arises from very different causes. 
 
 Partial recollections (i. e., kinetic or optic) for combined movements may 
 be lost, or difficult to rouse, or the connection among the partial components 
 may be lost, owing to an interruption in the fibres, or the regular outlet of the 
 mental process that prepares complicated movements, step by step, has been 
 affected by various disturbances (consisting not exactly in loss or splitting of 
 partial memories) of mnemic, associative, attentional kinds. 
 
 The apraxic suffers not from a disturbance of those lower co-ordinations of 
 muscles, known as ataxia, which is caused by severe sensory disturbances. 
 
 Taxia so regulates the working together of muscles, that the limbs, 
 without wavering, on the shortest path, traverse the road to the goal. But 
 which road is to be traversed, by which members or parts of members, in what 
 co-ordination or sequence, which object is to be the starting point, in order that 
 the desired form of motions, and that demanded by the purposes of life, 
 may result, and how, in the use of taxia, all necessary simultaneous and suc- 
 cessive innervations shall occur — in these questions, taxia says nothing, but 
 praxia rules. 
 
 For practical differentiation of the ataxic, there serves among other tests, 
 this — that the ataxic performs inexact movements, while the apraxic (for 
 exceptions see below) performs frequently movements — quite different from 
 those desired, that, on the other hand, while the movements of the apraxic 
 do not correspond to the intention, they appear perfectly co-ordinated, viewed 
 in the light of another purpose. He may write the wrong letter, that is, in 
 itself correct, or he places the comb behind his ear with the muscular move- 
 35 
 
546 DISEASES OF THE BRAIN 
 
 ments perfectly suited to this act. Those apraxics, who do not know how 
 to handle objects, were formerly usually classified with the agnostics. The 
 word apraxia was formerly employed when some one used an object incor- 
 rectly. But it was thought that he did so from mistaking the object or failing 
 to recognize its use. As one-sided apraxics prove, a man can, however, 
 recognize the object, know its use, and yet be unable to use it. 
 
 In order to diagnose apraxia, it must be proved that the patient recognizes 
 the object. At first sight, he seems to be mistaken, as when he makes motions 
 of smoking with a tooth-brush, as if it were a cigar. 
 
 The pre-essential condition of purposive movement, as, say, the lighting 
 of a cigar, is that the details of this movement arise internally, in correct 
 sequence, and in relation to the right object, so that the train of ideas corre- 
 sponds to the sequence of the necessary partial movements, and their relations 
 to objects (in very frequently performed acts, the subconscious cerebral 
 equivalent of this train of ideas). This is the ideational "plan" of move- 
 ment, which determines which paths, in what sequence, are to be run over 
 in relation to what object. In order that the corresponding movement may 
 be actually reached, directions corresponding to the ideational plan, must go 
 to the motor center of the executing limb, i. e., kinetic-innervational memories 
 must be awakened which release the real innervation. 
 
 If the ideational "plan" for movement is wrong, we speak of ideational 
 apraxia; if the disturbance is only in the transmission of ideational plans to 
 the special kinematic of the executing limb, we speak of motor apraxia (in 
 the broader sense). 
 
 We begin with the latter. 
 
 2. Motor Apraxia 
 
 Two subordinate forms are to be distinguished: 
 
 i. Motor apraxia in the narrower sense or ideo-kinetic apraxia. Here 
 the limb-center with its kinetic-innervational memories is itself retained, 
 but through breaks of countless connections with the other brain centers 
 the transmission of the "plan" of the movement to the limb-center is hin- 
 dered. The disease tears asunder ideation (plan of movement) and limb 
 kinematics. It must be assumed that in the sensory-motor limb center, not 
 only the seat of innervation, and the substratum of synergies is laid, but also 
 the substratum for certain much practised simple movements, the building 
 stones of complex purposive movements, as blowing, whistling, winking, 
 writing a letter of the alphabet, etc. 
 
 These simplest actions do not need directions from the brain as to every 
 step of their movement, but are a special possession of the limb centers, as 
 are the synergies. The retention of this special possession of the limb center 
 is shown clinically by the fact, that occasionally well-formed movements are 
 executed, i. e., a correct letter written, hands clasped, etc., but not at the 
 
MOTOR APRAXIA 547 
 
 proper point, not when the patient wishes and is determined to do it, because the 
 necessary co-operation of the limb-center with the brain is lacking. 
 
 2. If on the other hand, from a lesion in the limb-center itself that does 
 not go so far as to paralyze it, the special possession of the limb-center of ki- 
 netic memories, be injured, the seat of innervation can still obey, in part, the 
 orders of the brain; but the loss of this possession, given to the limb by long 
 practice, causes all movements to become rough, inexact, uneconomic, 
 like those of a man who is attempting them for the first time, with the result 
 that such movements that are not planned in the rest of the brain, but exist 
 only as kinetic memories of the limb-center, as blowing and whistling, can not 
 be performed at all. This subordinate form of motor apraxia may be 
 called limb-kinetic apraxia. 1 In so far, however, as co-operation between 
 limb-center and the whole brain exists, usually through the mediation of this 
 specialized possession, its injury will cause "derailments," which resemble 
 those caused by breaks in the paths running to the limb-center, namely, the 
 ideo-kinetic. 
 (a) Clinical Aspect of Ideo-kinetic Apraxia (Motor Apraxia Par Excellence) 
 
 It is confined to separate limbs, often half of the body. (For exceptions 
 see below.) 
 
 A train of simple movements is, on occasion, quite correctly performed: 
 the patient closes his hand correctly when he grasps an object, but is unable 
 to do the same thing when he wishes to make a fist; in writing he writes the 
 wrong letters, though each is correct in itself. Beyond this occasional 
 occurrence of correctly executed movements, however, even the very simplest 
 movements fail, when the stimulus comes from this part of the brain from 
 which the limb-center is cut off. The patient can not copy a vertical or 
 horizontal line that has been drawn for him, nor open his fist when he is told; 
 more complicated movements, pouring out water, striking matches, etc., 
 are out of question. The following kinds of false reactions occur: 
 
 1. Movements that resemble no purposive movements, such as beating 
 the hand, spreading the fingers (so-called amorphous movements). 
 
 2. Parapraxic movements — winking in place of threatening, touching the 
 ear instead of the nose, etc. 
 
 3. The movement is transferred to an entirely different musculature — 
 standing straight and rigid, in place of extending the hand. This simulates 
 real loss of movement. 
 
 4. Often motor indecision and real loss of movement take place. In 
 many miss-reactions, there is shown a strong tendency to fixation of ideas 
 (perseveration), namely, the intended movement is not executed, but the 
 preceding one repeated. By combination of such " persevering " movements 
 with components of the new purposive movements, very curious bastard 
 
 1 This conforms, at least so far as definition is concerned, with Meynert's motor asymboia. 
 
548 DISEASES OF THE BRAIN 
 
 formations arise. The fixation of movements is probably not the cause, but 
 the result of the right movement not being executed. Ideo-kinetic apraxia 
 is shown even in imitative movements. 
 
 5. Secondary disturbances of the nature of ideational apraxia, to be 
 described below, appear. 
 
 (/?) Clinical Aspect of Limb-kinetic Apraxia 
 
 Since very extensive focal lesions in the limb-center lead to paralysis 
 (which conceals the loss of limb-kinetic memories), it occurs mainly in 
 discrete processes (arterio-sclerosis, senile atrophy, progressive paralysis). 
 The coarse movements are awkwardly, inexactly performed and resemble 
 those in cerebral ataxia. They are distinguished from them in this, that 
 for many more delicate actions, sewing, blowing, whistling, not even a 
 tendency towards the action is to be distinguished. The movement shown 
 in these actions, is not only an ataxic distortion of the correct one, but is 
 wholly lost, or bears not a trace of resemblance to the original form. Here, 
 too, secondary ideational disturbances may occur. 
 
 3. Ideational Apraxia 
 
 Here manifold disturbances even in the ideational plan, are concerned — 
 due to flaws in memory, attention and association. The patient omits 
 parts of an action, performs them in the wrong order, or makes the right 
 movement towards the wrong object. Such a strange confusion of move- 
 ments results, that the patient, e. g., instead of striking the match, places it 
 in his mouth, next to a cigar, or attempts to cut off the end of the cigar, by 
 inserting it between the two parts of the match box. In sealing, he puts 
 the seal in the fire, and then impresses it on the wax. The errors look like 
 results of absent-mindedness; generally the association bond between 
 correct and incorrect movement can be proved. Single movements are 
 quite correct. 
 
 Ideational apraxia appears only in complicated movements, or, at least, 
 grows with the degree of complication. Imitation of short movements is 
 retained, since here the plan of movement is given the patient from without. 
 Only that the form of movement does not spontaneously occur to him. 
 (If the latter disturbance predominates, and other ideational derangements 
 appear in a small degree, or not at all, as is often the case, one may call it 
 amnesic apraxia.) It is, in general, not confined to separate limbs, but 
 affects all equally. It happens, however, that in a slightly motor-apraxic 
 limb, a general uncertainty of ideation is strongly marked. Ideational 
 apraxia is very often associated with agnostic disturbances. 
 
APRAXIA 549 
 
 4. The Predominance of the Left Hemisphere in Controlling Muscular Actions 
 and the Localization of Apractic Disorders 
 
 Heretofore, it has been assumed that the plan of movement rises in the 
 entire brain, that the visual images in both hemispheres and the kinetic 
 centers for all the limbs share it equally, and that this plan is transmitted 
 to the center of the acting limb. This, to a certain extent, is fiction. It 
 has been proved that the special kinetic function of the left arm center 
 and its connection with the rest of the perception centers has not only a 
 peculiar importance for the movement of the right arm, but also for move- 
 ment in other parts of the body and most surely for those of the left arm. 
 Probably the arm center in the left hemisphere is not only an intermediate 
 station for the stimulation of the arm center in the right hemisphere in 
 purposive movements, but the kinetic memories of the arm center in the 
 left hemisphere are an almost indispensable prop for the ideational plan 
 and for movements of the left hand. 
 
 As a matter of fact, we find, that in many lesions of the left hemisphere, 
 that paralyze the right hand or make it apraxic, the praxia of the left hand 
 is also affected, whence it is clear, that the direction of both halves of the 
 body in purposive movements is assumed, to a considerable degree, by the 
 left hemisphere. 
 
 The left hemisphere predominates, then, as in speech, so also in 
 action as well, even if not in similar degree. Focal injuries that strike, 
 
 1. The hand center in the left hemisphere, or 
 
 2. The underlying white matter, or 
 
 3. The connection of the hand center with other parts of the brain, 
 especially, the temporal, parietal and occipital lobes, cause in the majority 
 of people, besides the effect on the right side of the body (in 1 and 2 mostly 
 paralysis, in 3 apraxia of the right arm) the following dyspraxic symptoms 
 of slighter degrees of severity, in the left, unparalyzed, upper extremity: 
 
 1. Movements from memory can be performed only in a curtailed 
 fashion, or not at all : the patient can not longer differentiate how one catches 
 a fly, turns a hand-organ, or swings a cane, etc. Especially he can not 
 perform correctly expressive movements, as threatening, winking, giving a 
 military salute, throwing kisses, making a long face, etc. More or less 
 distorted movements result, and many perseveration miss-reactions occur. 
 If one can ascribe all this to mere amnesia (difiiculy in rousing the mem- 
 ory of the meaning and the word), it appears 
 
 2. That the patient can not imitate movements, so that not only the 
 spontaneous evoking of movement memories is assigned to the left hemi- 
 sphere, but a direction of the movements of the left hand by the left hemi- 
 sphere must be maintained. 
 
 3. That a few patients manipulate objects wrongly, even with the left 
 
55° 
 
 DISEASES OF THE BRAIN 
 
 hand. The majority, on the other hand, can when they see and feel the 
 objects, by the help of optic-tactile-kinesthetic signals coming from the 
 object, perform movements, that they could not accomplish from memory 
 alone. 
 
 fitirnpol 
 
 Fig. 156. — Horizontal scheme of the apraxic disturbances. L.H., left brain center of the right 
 hand. R.H., right brain center of the left hand. Co., C.p., C.t., cortical origin of the occipital, 
 parietal and temporal association fibres to the left brain hand center. The corresponding as- 
 sociation paths to the hand center in the right brain as well as those coursing from the right 
 himisphere to the left in red dashes, to point out their subordinate importance. The corpus 
 callosum connections between L.H. and R.H. aremarked by two full-drawn red lines. The blue 
 line which at the end of the arrow deviates from the plane of the figure, represents the projection 
 fibres of L.H. The pathway for purposive movements of the right hand is from Co., C.p., C.t., 
 over L.H. through the blue line into the anterior horn cells of the cervical cord. For purposive 
 movements of the left hand principally from Co., C.p., C.t., overL. H. through the corpus callosum 
 to R.H.; a less important path leads through the red dash lines to R.H. 
 
 1. The focus, which destroys completely L.H.: paralysis of the right and dyspraxia of the left 
 hand. 
 
 1a. Slighter lesion oiL.H. which does not lead to paralysis but destroys the mnemic possession 
 of L.H. only: kinetic apraxia of the right and dyspraxia of the left hand. 2. Paralysis of the 
 right and dyspraxia of the left hand. 3. (Focus in the corpus callosum) dyspraxia of the left hand. 
 4. (Focus behind the hand center in the parietal lobe) ideo-kinetic apraxia of the right and dys- 
 praxia of the left hand. Foci in the left hemisphere located farther backwards and diffuse processes 
 frequently cause ideatory apraxia. 5. Focus in the capsula causes paralysis of the right hand with- 
 out causing dyspraxia of the left hand. Above the cut: Stirnpol = frontal pole. 
 
 The weight of evidence and anatomic findings indicate that the right 
 hemisphere in purposive movements of the left arm innervated by it, receives 
 
APRAXIA 551 
 
 through the cor pis callosum, directions from the left hemisphere. Extensive 
 lesions in the corpus callosum have in a series of cases made the left hand 
 dyspraxic. 
 
 One may suppose, then, that memories of well-learned activities, the free 
 plan of movements, and, finally the supervision of their execution are pre- 
 dominantly the affairs of the left hemisphere and through the corpus callosum 
 are transmitted to the right. 
 
 The fact that apraxia of the left hand in focal injuries of the left hemi- 
 sphere is usually not of a very high degree, and especially often spares the 
 manipulation of objects, proves that the right hemisphere is not wholly 
 dependent on the left for praxia, but has a certain measure of kinetic power 
 peculiar to itself, as well as connections with the entire brain. 
 
 Eupraxia, then, is dependent on the soundness of a great apparatus, the 
 main part of which is the arm center in the left hemisphere. If this is wholly 
 destroyed (Fig. 156), paralysis of the right arm and dyspraxia of the left 
 appear. If it is but slightly injured (la) limb-kinetic apraxia of the right 
 arm and again dyspraxia of the left appear. If the white matter underneath 
 the hand center be injured (2), the right arm is again paralyzed, and the left, 
 because the corpus callosum fibres are broken in the white matter, is dys- 
 praxic. If in the parietal lobes, by an extensive focal injury, the connections 
 of the arm center with the temporal parietal, and occipital lobes and the right 
 hemisphere are interrupted, (4) ideo-kinetic apraxia of the right arm appears, 
 associated with slight dyspraxia of the left. 
 
 Focal injuries still farther back, in the hindmost part of the temporal, 
 and foremost part of the occipital lobes, as well as diffuse injuries of the brain 
 show ideational apraxia. 
 
 If only the corpus callosum is interrupted to a very considerable extent, 
 (3) the left hand is dyspraxic, the right, is neither paralyzed nor dyspraxic. 
 How focal injuries in the right hemisphere by robbing the arm center in the 
 right hemisphere of the nervous impulses, that carry the directions (and 
 eventually by destroying the memory), influence praxia of the left hand, has 
 not been definitely ascertained. 
 
 Focal injuries that destroy the projection fibres from the inner capsule, and 
 farther down (5), cause no apraxia of the hand on that side, because the 
 corpus callosal fibres are not affected. So that only supracapsular focal injuries 
 lead to apraxia. Hence apraxia is a characteristic that permits us to dis- 
 tinguish cortical injuries and those in the white matter, from those that are 
 more deeply laid, in the capsules, peduncles, pons, and bulbar region. 
 
 A phenomenon has been described under the name mind palsy which 
 stands between actual paralysis and apraxia : a limb can not be moved at will, 
 or only with great difficulty, but shows, through movements, that are per- 
 formed under peculiar circumstances that it is not really paralyzed, and by 
 their accuracy, that it is not apraxic. It is only a matter of great difficulty in 
 
552 DISEASES OF THE BRAIN 
 
 innervation. Since the words "mind palsy" (Seelenlaehmung) are used in 
 very different senses, one should call this phenomenon "volition palsy." 
 
 Whether, and to what extent, the left frontal brain lobe, participates in 
 praxia, is as yet uncertain. 
 
 The left parietal lobe is of the greatest importance for apraxia. Exten- 
 sive focal injuries in the left hand center, itself (central convolutions), and 
 in its white matter, paralyze mostly the right hand (la is a rare case) ; apraxia 
 is concealed in the right, and shows itself only in quantitatively slighter 
 disturbances of the left hand. The lesion of the parietal lobe, on the con- 
 trary, causes apraxia of the right hand to appear clearly. Lesions of the 
 corpus callosum have considerable influence only as regards the praxia of 
 the left hand. So the left parietal lobe is not at all the center for praxia in 
 the sense that there alone purposive movements are "made," but in the 
 sense that it is that place in the brain, in which lesions can interrupt in great 
 number, and with comparative isolation, connective paths most necessary 
 for praxia. 
 
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