Clinical aspects of syphilitic nervous affections

Preface

THe object sought to be attained in the publication of this work is to describe simply and concisely the clinical characters which the author has found of most value in marking the syphilitic nature of certain nervous disorders. To aid this description he has added a series of cases, having selected for the purpose from those which have come under his personal observation in hospital and private practice such as seemed best fitted to illustrate the numerous phases of these affections. Many of the cases, as well as a small portion of the commentary, have already appeared in the ‘ Lancet,” but it is hoped that the subject will be found

1 Dec. 3rd, 1870; March 11th, 1871; February to May, 1873.

v1 PREFACE.

much more amply treated in the following pages than was possible in the limited space afforded by the columns of a weekly journal.

For obvious reasons, the social status, and other circumstances which might possibly serve to identify the patients whose cases are described, have been for the most part omitted.

In the chapter on Pathology and Morbid Anatomy the author has largely availed himself of the writings of several continental observers; and in case the frequent references to their works in the footnotes are not sufficient to indicate how much he owes to them, he desires here to acknowledge expressly his great obligations to the scientific labours of Virchow, Zeissl, and Lancereaux. If he has referred more frequently to the writings of German and French authorities than to those of his own countrymen, it is not that he fails to appreciate the importance of the contributions which have been made to our knowledge of the subject by the latter, for the value of the work done, especially by Wilks and Hughlings Jackson has not been surpassed, but because it seemed to him

PREFACE. vil that the usefulness of his book would be increased by its containing the views of authors whose writings are not so generally available to the English reader.

56, Grosvenor STREET; March, 1874.

CHAPTER I. On tHE Diagnosis oF Sypuiniric Nervous AFFec

TIONS.

PAGE General observations.—The syphilitic element frequently overlooked—Analogy with anasarca from scarlatina—Individual symptoms not diagnostic, but the mode in which they are grouped—Case of paraplegia with hemiplegia and aphasia—Treatment—Recovery—Important points in diagnosis —Age—Simultaneous lesions in distant parts of cerebro-spinal axis—Cachexia—History of infection—Curative influence of iodide of potassium— Characters of syphilitic convulsions—Hughlings Jackson’s views on association of convulsion with double optic neuritis—Characters of hemiplegia — Causes — Paralysis of cranial nerves— Zeiss! on hemiplegia from osseous changes— Sudden hemiplegia—Arterial changes—Paraplegia — Affections of spinal cord— Period at which nervous affections may occur—Vertigo —Mental disorders—Blandford on mental disturbance of syphilitic origin—Senile degeneration of tissues— Softening of the brain—Indications from use of ophthalmoscope—Evidence afforded by electrical - examination . ‘ ‘ . . I—41 x CONTENTS.

CHAPTER II. On THE PatHotogy anD Morzsip ANATOMY OF SYPHI

Litic Nervous AFFECTIONS.

PAGE

At what stage of syphilis do they occur ?—Difficulties belonging to this question—Lancereaux, Vidal, Kuh, Knorre, on nervous affections in early syphilis—Anstie on osteocopic pains—Virchow’s views—Two series of developments—Hyperplasia and gummatous formation—The gumma as described by Virchow—Syphilitic dyscrasia—Degeneration of the gumma—Seats of cerebral lesions may be the membranes, interstitial neuroglia, or blood-vessels — Matting together of membranes very characteristic—External and internal pachymeningitis—Arterial changes—Partial arachnitis —Gummatous encephalitis—Gummy tumours of brain — How distinguished from tubercle by Virchow—Gummata of cranial nerves—Changes in the sympathetic—Tendency to recurrence of gummata— Wilks on albumino-fibroid deposit 42—73

CHAPTER III. Cases InLustRatTive or Sypuninitic Nervous ; AFFECTIONS

Multiple character of the affections precludes satisfactory classification—Syphilitic fever—Osteocopic pains—Functional disorder of digestive and circulatory organs—Faintness—Epileptic seizures— Insomnia—Cases illustrating paroxysmal aphasia —The muddy pallor in the syphilitic dyscrasia—

CONTENTS. x1

PAGE Spasmodic seizures in extremities, with and without loss of consciousness—Cases illustrating the large and early mortality in offspring of patients with syphilitic nervous affections—Cases illustrating association of old choroiditis or optic neuritis with convulsion and paralysis—Cases of general paralysis cured by iodide and mercury — Local affections of muscle, bone, or joint simulating results of central lesion—Paralysis and atrophy in the district of the sciatic nerve—Cases illustrating the tendency to relapse—Cases of mental dis-

Chapter IV

On THE Prognosis AND TREATMENT OF SYPHILITIC Nervous AFFECTIONS.

General remarks on prognosis—Special reasons for a favourable or a guarded prognosis—Treatment— Ricord’s views—Iodide of potassium in increasing doses—Mercury—Very large doses of iodide sometimes necessary —lIodism—Influence of iodide upon nutrition—Mode of administration—Employment of mercury by the mouth, calomel bath, or subcutaneous injection—Staub’s solution of the chloro-albuminate for injection—Author’s experience of this—Use of electricity in aiding the nutrition of paralysed muscles—Mode of its employment—Other therapeutical measures . 128—140

{ 4 Sa ay

CLINICAL ASPECTS

OF

SYPHILITIC NERVOUS AFFECTIONS.

Chapter I. On The Diagnosis Of Syphilitic Nervous Affections

Kvery medical practitioner, it may be assumed, is acquainted with. the fact that persons who have, at one time or other in their lives, become affected with constitutional syphilis may — some day exhibit symptoms of nervous disorder which are referable to this infection and may prove to be curable by specific treatment. Notwithstanding this, it is certam that this pathological association (and consequently its important bearing upon therapeutics) is very far from being generally remembered. The result is seen in the fact that patients frequently present themselves for advice who are suffering from some form of nervous affection dependent upon syphilis, who have already received all kinds of treatment except one directed to the basis of their disorder, and who recover, or are more or less benefited when specific remedies are adopted. In the treatment of these disorders more, perhaps, than in any others, an early application of appropriate remedies is of the greatest importance, and frequently, it is certain, makes all the difference between cure and the continuance of irreparable injury, or even death. Hven when from some circumstance or other, often from the sufferer’s own folly in attempting deception, a correct diagnosis has not been made, and the patient has long continued to derive no relief from the treatment adopted, but has been getting gradually worse, a timely recognition of the specific origin of his disorder, and the application of suitable remedies, are very frequently found to stay the downward progress of his disease, and bring about an amount of improvement far beyond what might have been anticipated. No doubt, in such cases, the amelioration is often only partial, a disorganisation of nerve substance has taken place, which is irreparable so far as this has gone, but further extension is prevented, and time is given for the natural process of repair to effect a great change in the

DIAGNOSIS. af patient’s condition. It becomes, then, of great consequence to consider what are the points which may aid us to the formation of a correct judgment in this matter.

When we see a child who is anasarcous, and has albuminuria, our thoughts immediately turn _ to scarlatina, and we inquire whether the patient has lately had a sore throat. We do not expect necessarily to find at that time any external mark or indication of the antecedent fever. We know that the anasarca is a common sequel of scarlatina, and ninety-nine times out of a hundred we are right in supposing that the child has suffered from that disorder. Here is an association of ideas which is always present to every medical man, and in this case constantly leads him to a right conclusion. Now, it seems to me that the possibility of a similar association in respect to syphilis ought to find its place in the investigation of every case of nerve disorder. I need scarcely say that the analogy will not hold good as regards the frequency of the connexion between nerve disorder and syphilis. That is manifestly impossible. It is evident that all kinds of circumstances may place the probability of such an association out of the question, and I need not dwell upon these. But we should especially

A, ' SYPHILITIC NERVOUS AFFECTIONS.

remember that, just as in the case of the anasarcous child we are content with the history (often brought out only by our own questioning), and require to see no mark of scarlatina, so in cases of syphilitic nervous affection, we must not expect to find in the patient any definite sien which would necessarily prove the syphilitic nature of his malady. I cannot help thinking that an error in this respect is at the bottom of the frequent defects of diagnosis which occur in the examination of these cases. The medical man has read of syphilitic diseases of the nervous system, and expects, for some reason or other, that when such a case presents itself to him it will bear unmistakable marks of its peculiar origin. Called to a patient who has lost the use of his limbs on one side of the body, or who is in convulsions, he sees what is clearly to his mind a case of paralysis or epilepsy, differing in no respect, so far as he can observe, from other cases of these disorders which he has watched. There is nothing to suggest syphilis, and, unlike what happens by reason of the well-known bond of connexion between anasarca and scarlatina, his experience in this. instance has not taught him how frequent is the association of loss of power, or convulsion, with antecedent syphilis. I think it cannot be too distinctly affirmed that, although the concurrence of various symptoms and circumstances renders easy now and then a correct diagnosis of the syphilitic origin of a — nerve disorder even without the help of the patient’s history, yet there are no pathog- _nomonic signs as yet definitely ascertained which will point conclusively, without the aid of the history, to the specific nature of the case. The lesions which result from syphilis affect the machinery of the nervous system like the lesions which are produced by many other causes. The wheels of a watch may be stopped as well by the intrusion of a hair as of a piece of grit, and it is only the knowledge of the , conditions to which the instrument has been exposed which will enable us to decide which of these substances is the probable source of the resulting breakdown.

But although the individual symptoms carry with them no conviction as to the specific origin of the disorder (because they are but symptoms of some lesion or other affecting portions of the motor or sensory tracts), the pecuhar grouping of such symptoms may lead of itself to a probability but little short of certainty. The following case, which occurred. in hospital practice in 1870, affords a good illustration of this, as well as of other points upon which I have touched, or shall notice in other parts of this work.

Cast I.—A man, aged thirty-nine, was wheeled into the consulting-room on a chair, being unable, except in the slightest possible degree, to move either leg. Neither could he lift his right arm, and his face also was partially paralysed on the right side. He had great trouble besides in expressing himself, being constantly at a loss for the right word. A touch was felt very imperfectly on the right side of the face and arm, and on both legs. There was incontinence of urine and of feces, as well as absolute impotency. He was very cachectic in appearance. There was no evidence of disease of the heart, kidneys, or blood vessels, and there was no history of any injury. During a voyage to England five months previously he had complained of extreme weakness, which gradually merged into a definite loss of power in the right leg, and was accompanied by incontinence of urine. Two months after his arrival in this state, he suddenly became paralysed in the right arm and face, without any attack of loss of consciousness, and a week after this his left leg began to lose power, until it became as much paralysed as the right. The left arm was the only limb left unaffected. He had been under constant medical treatment, which included, as I ascertained, strychnia, iron, and quinine, but had uninterruptedly grown worse. Finding, on inquiry, that seven years previously he had suffered from a chancre for which he took mercury till his teeth were loose, and which was followed by sore throat, but by no skin eruption, I at once gave him iodide of potassium. In a month he could walk a mile with the help of two sticks, and could hold his urine forashort time. Jn another fortnight he could walk a little way without sticks, ‘and a month after this he could manage four miles by the help of a stick. His reproductive powers had returned, and he had much more command over his bladder and sphincter ani. Six months after his first visit to the hospital he had almost entirely recovered his usual health, could walk six miles “at a spell,” and more than that in the day. His grasp was equal and perfect with either hand. There was still a little hesitation in speech, but he had ceased to employ the wrong word. There was also some slight remaining weakness of the sphincter ani. He told me he could feel nothing the matter with him, and was about to start for one of the colonies. I advised him to resort to the iodide (of which he had taken thirty grains daily for six months) in case he felt any tendency to a return of his ailment.

This man must, probably, have had a double lesion—one situated in the district of the left middle cerebral artery, and the other in the sheath of the spinal cord, most likely in the lower dorsal portion. Leaving the previous history of the patient and the results of treatment out of the question, this pathological condition alone pointed to interrupted communication in various parts of the sensory and motor tracts. Now, as regards the intracranial lesion, the resulting symptoms were consistent with softening consequent upon embolism of the middle cerebral artery, or with cerebral hamorrhage in the region supplied by this vessel, or with pressure from some growth. But the lesion of the cord, if it were not dependent upon a partial myelitis, must be caused by some form of tumour, cancerous or otherwise, and the improbability of the almost coincident appearance, in a man of this age, of tumours which were not syphilitic in two parts of the cerebro-spinal axis, would practically decide the question. On the other hand, there would be nothing at all inconsistent in the occurrence of gummatous tumours about the same time in various portions of the cerebro-spinal membranes. The history, however, and the excellent recovery from an exceedingly grave state under iodide, left the nature of the disease in no manner of doubt.

This case will serve very well for a text upon which to found some general observations upon the diagnosis of syphilitic nervous affections. The points specially noticeable are as follows :—Ist. The age of the patient. He was too young for the hemiplegia to be reasonably explained by the occurrence of non-inflammatory softening of the brain, such as is so common in advanced life from degeneration of the cerebral arteries, and the condition of his circulatory apparatus and kidneys was equally sufficient to exclude the probability of embolism, thrombosis, or cerebral hemorrhage. ‘The absence of unconsciousness at the time of his hemiplegic seizure would also practically exclude the last. 2nd. The occurrence of lesion - contemporaneously in two portions of the cerebro-spinal axis. This is a feature peculiarly noteworthy. It is exceedingly uncommon except as a result of syphilis, and very common in the disorders of the nervous system which are consequent upon that disease. 3rd. The existence of marked cachexia unexplained by evident disease of any of the viscera. 4th. The history of a venereal sore, which had been contracted seven years previously, and which, as it was treated with mercury, and was followed by sore throat, was doubtless of true syphilitic character. 5th. The immediate improvement and rapid restoration to health under the employment of iodide of potassium.

It will be remarked, if this case be analysed, that the individual symptoms taken singly presented nothing conclusive of a syphilitic origin of his affection. The man was paralysed in one half of his body just as occurs in ordinary hemiplegia. He lost the use of both legs, the power of one failing somewhat _ before that of the other, which is not uncommon in ordinary paraplegia. The aphasia from which he suffered is constantly seen as an accompaniment of hemiplegia from various causes in- _ fluencing the integrity of the motor tract, from the anterior ganglionic centre of the left side. His impotence, incontinence of feces and of urine, are all everyday accompaniments of paraplegia, from various lesions of the cord. The cachexia might have been due to an unrecognisable disease of blood - making viscera. Lastly, he might have recovered health whilst taking iodide without that drug having anything to do with the restoration. Many cases of hysterical paralysis do so recover health under that treatment, although we are tolerably sure that the drug performs no part whatever in the cure. It is, however, in the combination of all these symptoms that we find, without any doubt, the key to their causation. So strong indeed is the evidence that we could even afford to do without the clinching proofs brought by the syphilitic history, and the results which followed a specific treatment of his paralytic condition.

Of the various points referred to, I would lay by far the most weight upon the age. It is this which first attracts the attention in a case of serious lesion of the nervous system, and in many cases almost immediately determines the diagnosis. I have little hesitation in stating my conviction that, putting aside cases of injury, hemiplegia or paraplegia occurring in a person between twenty and forty-five years of age, which is not associated with Bright’s disease, nor due to embolism (from disease of the cardiac valves) is, in at least nineteen cases out of twenty, the result of syphilis.

I need scarcely say that such a completeness of testimony as characterised the example quoted is not what we most commonly meet with. Were it so, there would be but little difficulty in our diagnosis of conditions which, on the contrary, are often extremely obscure, and require the most careful investigation and weighing of evidence in order that we may come to a right conclusion.

In noting various points, the consideration of which may help us in formmeg a diagnosis, I have placed last the evidence gained by the employment of iodide of potassium. Practically, for many years past, Im common with others who see a good many examples of nervous disorder, I have been in the habit of prescribing this drug very early in every case which did not from its symptoms offer a ready solution of the cause of the disease without the necessity of considering whether it was possibly of syphilitic origin. In very many instances this practice affords a convenient means of arriving © at an opinion which more elaborate investigation may then confirm. It would be rather like reasoning in a circle to say that “ because iodide of potassium immediately removes certain symptoms, therefore the case is of syphilitic nature, and because it is syphilitic therefore iodide of potassium will cure it.” But practically the statement is generally correct as far as it goes, though, of course, it is not logically an argument. We see a similar association of ideas every day in the case of intermittent fever and the employment of quinine, as well as in scabies and the local application of sulphur. Unfortunately a too great dependence upon the apparent results of treatment may lead us to very erroneous conclusions. As one example, let us take the case of peripheral paralysis of the portio dura. This is sometimes the result of syphilitic thickening of the fibrous membrane lining the aqueductus Fallopu, but it is still more frequently caused by the action of cold, and under these circumstances is supposed by some to be of rheumatic character. Now it is notorious that cases belonging to this last category will recover, sometimes rapidly, without any treatment, and if in such an instance

DIAGNOSIS. ee)

we give iodide, and the patient gets well, we shall wander very much astray in ascribing the lesion to a syphilitic origin. On the other hand many cases of specific character are unaffected. by moderate quantities of iodide, some indeed not responding at all to such treatment even in large doses, but requiring the employment of mercury ; and should a small and tentative employment only of iodide be made without good results, we may easily jump to the conclusion that there is no syphilitic basis, and be led to neglect the only means of curing our patient. Whilst, therefore, the beneficial effects of iodide will often give us a strong primd facie ground for concluding that the case is syphilitic, we must not rest too confidently upon this kind of evidence, nor consider that the non-specific character of the lesion is at once proved by the negative results which seem to follow treatment addressed to such a cause. Amongst the cases of epileptic character which I have met with, and some of which are narrated further on, there have been several in which, after bromide of potassium had failed to be of service, the iodide was immediately successful. I think that under such circumstances the results of treatment lend very strong testimony to the specific nature of the disorder. With a view of testing this point I have frequently given iodide of potassium in large doses to epileptics in whom from circumstances I could feel sure there was no syphilitic taint, and I have failed to find any well-marked influence exerted upon the number or severity of the attacks which, on the other hand, were speedily checked when the bromide was substituted. Convulsive attacks, on the contrary, dependent upon syphilis, are usually influenced to a remarkable extent by the iodide of potassium.

And here it may be well to refer to certain characters which point to the syphilitic origin of many cases of convulsive disorder as distinguished from what is called essential epilepsy. If pain in the head be associated with the convulsive attacks, it generally precedes the outbreak in syphilitic convulsion, and is often localised in one particular spot. There is frequently a history of antecedent pain for months before the first fit. In simple epilepsy it almost always follows the fit, is diffused over the forehead, and is at no time a strongly marked symptom. In the simple form the attacks most often commence about the period of puberty ; in the syphilitic, they do not make their appearance till a later period, generally perhaps when the patient has past twenty or twenty-five years of age. In syphilis there occur very often attacks not characterised by loss of consciousness ; the patient feels a cramp in the fingers of one hand, which draw up, then the - hand is contracted upon the forearm, and this upon the upper arm, and clonic convulsions ensue for several minutes. Or the tongue may be twisted to one side, and the cheek be then spasmodically drawn up. Sometimes the same phenomena happen in one of the lower extremities. These attacks may cease without the patient losing his senses or, beginning thus, the seizure may proceed to insensibility and general clonic convulsions, as in ordinary epilepsy. In syphilitic epilepsy there is frequently a rapid recurrence of seizures, with intervals of more or less complete coma, and often loss of power in the limbs of one side following the attacks, and lasting for a short time only.

The epileptic cry is often absent. My colleague, Dr Hughlings Jackson, to whom we are so much indebted for his valuable observations in syphilitic nervous affections, has described a condition which, according to his experience, is highly characteristic of syphilis. He finds that double optic neuritis (or amaurosis from its frequent sequela—atrophy of the optic disc), along with convulsions beginning unilaterally, most frequently in the hand, and especially in the two first fingers, form an association of symptoms which usually implies syphilitic disease of the brain. The convulsion no doubt points, he says, simply to disease of some kind, not to any particular pathological change of, or in the side of, the brain opposite to that in which the spasm sets in, The combination with optic neuritis merely helps us to say that the change is a gross one—a lump of something. But experience shows that the gross disease of the brain in these cases is usually syphilitic. Simce Dr Jackson called my attention to this combination, I have observed it sufficiently often to feel that he is right in regarding it as an important diagnostic feature. As regards the occurrence of hemiplegia this may be associated with a sudden attack of apoplectic character, or it may be slowly and gradually developed. If the attack be sudden there is this peculiarity, that the patient seldom loses consciousness; he feels giddy, perhaps, and on attempting to rise finds that he is paralysed. ‘This is the mode of onset described in several of the cases narrated further on. It is not, however, peculiar to syphilis, and cannot, by itself, be relied upon as diagnostic; and equally the slow development of hemiplegia only points to the growth of a tumour, or progressive increase of some formation, which is not, however, necessarily of syphilitic character. Other . circumstances have to be considered, such as the age, history, condition of other organs, especially the heart, blood-vessels, and kidneys, ere any approach to the probably syphilitic character of the lesion can be made.

And, supposing the circumstances should point to its being of this nature, there is still the difficulty,—not, it is true, a very important one in practice,—that the lesion may be either due to the existence of a gummatous tumour connected with the cerebral substance, or the symptoms may arise from morbid changes in the blood-vessels, the skull, or the membranes. If the lesion be connected with bone alteration, it is usually found to have been preceded by fixed, intolerable pains in the head, which may have lasted some considerable time, and which are often, but by no means always, increased at night. In addition, an important aid to diagnosis in such cases exists in the circumstance that the seat of pain may be actually tender on pressure, and that in its neighbourhood some periosteal thickening may be discovered.

Paralyses of individual cranial nerves are often due to morbid change existing on the

Ts SYPHILITIC NERVOUS AFFECTIONS.

same side of the skull in the bone or the membranes. According to Professor Zeissl* hemiplegias, dependent upon morbid change in the vitreous table of the skull, are, with the exception of those which proceed from an alteration in the base, never so marked and complete as those which are brought about by disease in. the brain substance itself, especially if the corpora striata and optic thalami are affected. | When hemiplegia is sudden, it can hardly depend upon exostosis, which would be more likely to bring about a gradual loss of power by its growth. So, also, those phenomena which are marked by intermissions, loss of power enduring only for a short time, aphasia of transient character, and frequently recurring, can hardly depend upon exostoses, the growth of which is gradual, and the pressure of which cannot be suddenly taken off and restored. Cases of this kind will be found described later in this work.

Huebner; divides the symptoms of brain disease from syphilis into those which are persistent, and those which are intercurrent. The symptoms may occur in any period of syphilis, sometimes, indeed, from the first appearance of

* ‘Lehrbuch der Syphilis.,’ 2 Th., p. 290, Erlangen, 1872. + §S. Canst. Jahresb. 1870.

the exanthem. In the beginning only trifling troubles are to be noted, and these either continually increase to a high degree, or constantly persist and show paroxysmal accessions. These apoplectiform paroxysms quickly lead, either by increase of the symptoms, to death, or diminish quickly or slowly, and only trifling disturbances remain behind; or the symptoms vanish completely. Huebner quotes two cases observed by him in which the symptoms of brain disease showed the above-named characters, and which ended fatally. Both patients evinced unmistakable signs of general syphilis. In one of the cases, a man, zt. 34, in whom the disturbances in the form of apoplexy quickly led to death, the autopsy showed alterations of different degree in the soft membranes, rigid spots in the left superior artery of the cerebellum, and the left sylvian artery; clinging loosely to these rigidities were soft coagula. In the other case, which was that of a woman et. 25, in whom the fluctuation and disappearance of the symptoms of brain disease had occasioned an erroneous diagnosis of hysteria, section showed softening of the brain, clouding of the soft membranes, thickening of a portion of the right sylvian artery, a partly solid, partly soft coagulum firmly sticking to this point, with © a brown-red, soft, recent coagulum behind it. The right artery of the corpus callosum showed likewise a thickening, to which an older thrombus clung, as also did the basilar artery— a gradual strata-like thrombosing of the blood-vessels (it was not a question of embolism), which was very characteristic of a syphilitic brain disease. In the chapter on morbid anatomy I shall have more to say about these arterial changes. They are especially interesting, in that it seems likely that iodide of potassium exerts little or no influence upon them. In a case in which I found such a condition unusually well-marked on post-mortem examination, the patient had been taking considerable doses of this drug for some time previous to his death.

In the diagnosis of paralysis of the extremities, without involvement of the cranial nerves, the question whether the cord or its membranes is the seat of a syphilitic affection must be considered with reference to the same points as those which guide us in examining cases of hemiplegia. The age, history, condition of the organs generally, the occurrence of injury and exposure to cold, the effect of remedies, must all bear their part in enabling us to arrive at a probable solution of the nature